Factitial synovitis.

This unusual case of trauma depicts a 17-year-old female who had a history of chronic synovitis of her left knee for several months. The patient demonstrated persistent knee effusion despite treatment of several physicians. Failure of the left knee to respond to several surgical procedures, as well as the finding of numerous foreign bodies not present previously, evoked a high index of suspicion leading to the diagnosis of factitial synovitis. The paper further defines the entity, describes the personalities involved and the numerous methods used to inflict trauma. Lastly, the treatment regime is given.


Orthopedic injuries in water-skiing: etiology and prevention.

The inherent danger of orthopedic injury is little recognized in the extremely popular sport of water-skiing. The water-skiing equipment, the speed at which the boat is driven and the experience of the skier play a major role in the severe injuries reported here.


Dialysis carpal tunnel syndrome.

Twelve carpal tunnel releases were performed in eight out of ten dialysis patients who developed progressive median and ulnar nerve compartment syndromes following side (artery)-to-end (vein) fistulae required for vascular access, out of a total population of between 200 and 250 patients (4 to 5%). It is our contention that etiology is directly related to the altered vascular hemodynamics produced by the fistulae. All the patients had rapid relief of their pain and dysesthesias, and were very satisfied despite some residual numbness in a few cases.


Superweak interactions and the biological time direction.

A hypothesis is proposed in qualitative terms, according to which left helical electrons in chiral molecules, and right helical electrons in oppositely chiral molecules have different internal timings. The basis of this hypothesis stems from two facts: (1) It has been observed that the decay processes caused by superweak interactions go slower if left helical electrons are produced compared to decay processes in which right helical positrons are produced. (2) The excited states of D and L molecules are not only parity transformed but time reversed pairs as well. Re-evaluation of many data makes the hypothesis likely. The significance of this hypothesis in the origin and evolution of life is apparent since life and evolution as a whole have a special time direction.


The orthopedic surgeon and rehabilitation engineering.

Rapid advancements in technology in the past 30 years have brought about a close relationship between medicine and engineering. Part of this has been the close association of orthopedics and the prosthetics and orthotics technologies. In recent years, advances in other aspects of rehabilitation engineering have taken place; these have been partly based on the technology established by research in prosthetics and orthotics. The rapid progress in rehabilitation engineering now demands the attention of orthopedists. The role of the rehabilitation engineer is defined, and some examples are given of the kinds of problems confronted by the rehabilitation engineering clinic team. Especially as further progress takes place, the orthopedic surgeon and the prosthetist-orthotist need to obtain more knowledge of the technical aids needed to restore independence to the disabled.


Temporary occlusion of the common iliac artery during hemipelvectomy.

The surgical technique for temporarily occluding the common iliac artery during hemipelvectomy has been described. Not only can this technique significantly reduce the blood loss and operative time, but it may also minimize postoperative complications and increase the chances of a complete eradication of the primary malignant disease.


Early evolution of cellular electron transport: molecular models for the ferredoxin-rubredoxin-flavodoxin region.

Using information from conformation-predicting algorithms and X-ray data, a possible mode of structural evolution from an ancestral molecule of about 28 residues to bacterial ferredoxins and rubredoxins is suggested. The possibility of a further evolutionary pathway leading to flavodoxin-like proteins is also indicated.


Traumatic fibular bowing with tibial fracture: report of two cases.

Two cases of children with traumatic fibular bowing associated with mid-shaft tibial fractures are presented. Healing of the mildly angulated tibial fractures without reduction of the bones was accepted. The case, followed 38 weeks, demonstrated corrective remodeling with growth and an excellent clinical result.


Malignant hyperthermia: a complication of orthopedic surgery.

Although malignant hyperthermia is seen most frequently as a complication of orthopedic surgery, most orthopedists have not encountered this commonly fatal problem. A case report and discussion of the inheritable metabolic defect is given. Immediate treatment concepts are discussed.


Arthrodesis for paralytic shoulder: review of ten patients.

Ten patients who had arthrodesis of their flail shoulders secondary to poliomyelitis were reviewed. The average age at the time of surgery was 15 years, 3 months, and follow-up range from one year eight months to seven years. Union occurred in each case, and no reoperations were required. Internal fixation was used for each patient, and this maintained the position of arthrodesis. All patients were significantly improved. Optimal position of fusion seems to be in 30 degrees of abduction, 30 degrees of forward flexion, and 45 degrees of internal rotation.


Aminoacyl-tRNA synthetase families and their significance to the origin of the genetic code.

A correlation of various aspects of the protein structures and substrate and mechanistic specificities of the aminoacyl-tRNA synthetases has led to the identification of at least one family of enzymes probably derived from a common ancestral synthetase. While strong correlations exist only in one part of the array of 64 codons comprising the Genetic Code, this itself may be interpreted as a meaningful pattern, most consistent with a development of the present code from earlier codes containing fewer amino acids and fewer available codons. Specifically, strong correlations in the enzymes whose cognate tRNAs respond to codons containing a central pyrimidine, including the enzyme family of Ile-, Phe-, Val-, Met-, and Leu-tRNA synthetases, suggests that these enzymes evolved last, and that, therefore, an earlier version of the Genetic Code was comprised solely of codons containing a central purine. It is suggested that further study of the historical interrelationships of these enzymes could lead to a fairly detailed picture of how the Genetic Code developed.


Infection after knee replacement surgery.

Vigorous treatment is not always necessary to eradicate late postoperative infection after total knee implantation. Aspiration of the pus and intravenous antibiotic administration was enough to eradicate the infection in the case reported.


Chirality and stereochemical recognition in DNA-phytohormone interactions: a model approach.

Space-filling molecular models of selected phytohormones and DNA, employed as described herein, illustrate possible in vivo stereochemical recognition between nucleic acids and intercalated phytohormones. In this regard, the absolute chirality of certain phytohormones, and that of DNA may be essential for the recognition process. It is speculated further that the specific interactions shown by molecular models have significance in the evolution of plant regulatory mechanisms.


Exercise testing in children.

Exercise testing has a definite role in pediatrics today. Different methods are presented, and the value of maximal exercise with determination of oxygen uptake and blood lactate is stressed. In children with heart disease, exercise testing with precordial electrocardiogram can be of both diagnostic and prognostic value. The cardiovascular function at different intensities of exercise is evaluated, serious dysrhythmias may be revealed, hypertension judged and the effect of drug therapy can be checked by exercise testing. It is an important way in assessing the child's functional capacity after heart surgery in the decision whether she or he should take part in physical education and sports activities and in the choice of profession. It is also of great psychological value to the parents and the patient himself. In children with other chronic diseases, e.g., diabetes, obesity, asthma, neurocirculatory dysfunctions--physical training together with exercise testing is of importance for therapy and rehabilitation.


Exercise electrocardiography in the evaluation of cardiac dysrhythmias in children.

Exercise electrocardiography is a useful means to evaluate dysrhythmias in children and young adults. Exercise may suppress a dysrhythmia present at rest and thus suggest a relatively benign problem. It may provoke a latent dysrhythmia which is absent at rest but suggested by a history of exercise intolerance or palpitation with exertion. Exercise may also modify a dysrhythmia. Maximum exercise testing has been used to evaluate each of these situations in children. The results of testing will be presented. Since no dysrhythmias were provoked in 170 healthy children, we think that any dysrhythmia initiated by exercise in the young should be considered abnormal.


Current status of open heart surgery in infancy.

The enthusiasm for primary intracardiac surgery in symptomatic infants with congenital cardiac disease generated by early successful reports continues. The results obtained to data indicate that when surgery is carried out in extremely ill symptomatic infants, the mortality is high but still superior to staged procedures. When surgery is performed relatively electively, to prevent complications related to the unnatural history of the disease, the results of surgery are excellent. Profound hypothermia and circulatory arrest have contributed greatly towards facilitating technical surgery and postoperative management. An increasing number of centers are utilizing conventional cardiopulmonary bypass with hypothermia to achieve the same result. One of the major objections to using hypothermia and cardiocirculatory arrest has been the fear of cerebral complications, both neurological and psychomotor. Our follow-up studies on an unselected group of patients have shown very clearly that when the technique was applied carefully with strict attention to detail, late neurological and psychomotor complications were virtually nil. The IQs of these children fall within the normal distribution curve of a comparable group of noncardiac children. In view of these late follow-up findings, we feel that hypothermia and cardiocirculatory arrest are safe modalities in handling infants undergoing open intracardiac surgery. There is probably still a place for two-stage procedures in selected lesions and these are complex transposition of the great arteries and perhaps as a clinical trial in a randomized group of infants with tetralogy of Fallot. With the exception of these lesions, early correction of all operable symptomatic heart defects in infants is recommended.


Problem of patent ductus arteriosus in premature infants.

Postanatal closure of the ductus arteriosus depends on the level of O2 to which it is exposed. Certain vasoactive substances may also play a role in this constriction. The response to O2 is not as well developed in immature fetal animals and this probably explains the high incidence of patent ductus arteriosus (PDA) in premature human infants. The magnitude of shunting through a PDA depends on its size and the relationship between the pulmonary and systemic vascular resistances. Factors affecting the pulmonary vascular resistance (such as hypoxia) will therefore affect the shunt. The ability of the infant to handle the volume overload also depends on maturity of the infant since myocardial development may not be complete even at term. The clinical manifestations and management of PDA with and without pulmonary disease are described. Recent attempts at pharmacological closure of the PDA are presented.


Surgical treatment of complex congenital heart defects: recent developments with valved external conduits.

The use of valved external conduits has majorly expanded the place of corrective operations for children with a variety of congenital cardiac defects. Initially they were employed to correct patients with congenital pulmonary atresia and ventricular septal defect and subsequently placed between the venous ventricle and pulmonary artery for a number of other complex malformations. More recently they have been placed between the right atrium and pulmonary artery and between the systemic (left) ventricle and aorta. Surgical techniques, early and late results of these procedures are discussed.


Pericardial disease in children.

This article discusses the etiology, clinical presentation, diagnosis and treatment of pericardial disease in the pediatric age group. Purulent pericarditis is a medical and surgical emergency. Prompt surgical drainage and intravenous antibiotic therapy may be lifesaving. The pathogenesis and hemodynamic consequences of pericardial effusion, cardiac tamponade, pericardial constriction and pneumopericardium are also discussed. The key to intelligent management of pericardial disease is an appreciation of the fact that the abnormal hemodynamics resulting from these disorders can frequently only be relieved by cardiac decompression.


Infective endocarditis: a review. I. Incidence, etiology, pathology and clinical features.

In this paper demographic characteristics, etiology, pathology and clinical features of infective endocarditis are reviewed simultaneous presentation of the data from our series of 50 cases with infective endocarditis. The peak incidence of infective endocarditis is between 11 and 15 years. Both sexes are equally affected. Patients with congenital or acquired heart disease tend to have hemodynamic trauma to the endocardium and vascular endothelium. These sites form the nidus for circulating bacteria of either spontaneous origin or the result of any oro-dental, genitourinary or other surgery or procedures and produce vegetations characteristic of infective endocarditis. The location of the vegetation is dependent upon the predisposing cardiac lesion. Embolic phenomenon is another cardinal feature of endocarditis and may occur in any organ system. Although a large variety of microbes have been known to cause endocarditis, streptococci and staphylococci remain the most frequent offenders. Clinical diagnosis of infective endocarditis is difficult because of the insidious onset and varied clinical features. A high degree of suspicion is essential for early diagnosis. Any patient with known heart disease and unexplained fever should be suspect for endocarditis. Splenomegaly, petechiae and embolic phenomena support this diagnosis. New or changing murmurs, splinter hemorrhages, Osler's nodes. Janeway's lesions and Roth's spots may be present. Elevated sedimentation rate, microscopic hematuria, leukocytosis with a shift-to-the-left and anemia may further support the diagnosis. Congenital or acquired heart disease and fever are all that will be present in many cases. Only isolation of the causative agent from the blood can confirm the diagnosis.


Infective endocarditis: a review. II. Diagnosis and treatment.

Early diagnosis of infective endocarditis is difficult because of the insidious onset and varied clinical presentation. High degree of suspicion and blood culturing for the causative agent are essential to confirm the diagnosis. General principles of management include selection of antibiotics based on the antibiotic sensitivities of the causative organism, use of bactericidal rather than bacteriostatic drugs, usage of a combination of two or more antibiotics to enhance the synergic bactericidal activity, and their administration by the intravenous route for prolonged periods (6 weeks). Monitoring the serum bactericidal activity to confirm the biological effectiveness of the antibiotics used and adjusting the level of 1:8 to 1:16 is recommended. Specific drug therapy for each type of endocarditis is discussed. Supportive measures and indications for surgical intervention are also discussed. A summary of preventive aspects of infective endocarditis is also presented.


The unconscious child.

Coma in children is an overwhelming process. The article discusses the probable etiological factors that may lead to coma condition, the physical examination, laboratory diagnosis and therapeutic management of the unconscious patient. Emphasis is being given to the emergency treatment of associated cardiac arrest and reference to the medicolegal problem arising from the prolonged use and discontinuance of mechanical life-supporting devices.


Liver failure.

Liver failure is a complication of viral hepatitis, as well as a consequence of acute mushroom or drug poisoning. The different therapies to be employed in a "pre emergency" state, or in case of "emergency" (resistant to medical treatment or starting as "fulminant" hepatic failure) are summarized. The percentage of survival, even by means of sophisticated treatment, does not overcome 30%.


Acute anaphylaxis in children.

Much progress in the knowledge of immunological and biochemical mechanisms involved in anaphylaxis has been achieved during the last years. The complexity of these mechanisms still makes many of the details enigmatic and difficult to study. The pathogenic mechanisms, symptoms and treatment of anaphylaxis are discussed in the light of our present understanding. Anaphylactic reactions should be rare in childhood as most of them are iatrogenic and thus possible to prevent by suitable precautions.


Renal emergencies in children.

Acute renal failure, severe hypertension, and some complications of the nephrotic syndrome and chronic renal failure are the renal emergencies most frequently seen in the pediatric age group. Diagnosis of acute renal failure is based mainly upon U/P ratios of osmolality and urea as well as a negative mannitol test. Conservative medical management is useful in uncomplicated cases, whereas dialytic procedures are more effective in severe or complicated cases. The most frequent reversible complications in chronic renal failure are infections, extracellular volume changes, electrolyte imbalance and cardiovascular alterations. Intercurrent infection and vein thromboses are the most frequent complication in minimal change nephrotic syndrome and they may be a cause of death. Early diagnosis and adequate treatment are mandatory in these complications.


Emergency surgery of the neonates.

A review of 1,666 neonatal surgical operations is given. According to the presenting symptoms the cases are divided into five major groups: skin defects and obvious malformations, tumours, acute abdominal conditions, respiratory emergencies, and cardiac conditions. The number of cases in each group are presented in tables and the surgical mortality of the most important conditions are similarly presented. Prematurity and associated malformations are pointed out as the most important factors causing death in neonatal surgery. While it is difficult to influence their existence, it is always possible to improve the diagnostic readiness and surgical skills.


Radiofrequency thermoneurolysis of peripheral nerves for control of trigeminal neuralgia.

Radiofrequency thermoneurolysis (RFTN) was performed in the peripheral infraorbital and inferior alveolar nerves of eight patients with paroxysmal trigeminal neuralgias. Pain severity as measured by tourniquet test and global estimate was significantly reduced in seven of eight patients. The sharp, paroxysmal component of neuralgia was controlled in all seven successes although significant pain was recurring in two patients one year post-operatively. Neurosensory threshold responses to tactile-discriminative stimuli were not permanently changed from pre-lesion levels but pain detection and pain tolerance threshold responses to mechanical pin-pressure and thermal stimuli were significantly raised, suggesting that RFTN is more selective for small myelinated and unmyelinated fibers. This procedure was found to be safe, effective, simple, and predictable on the basis of diagnostic blocks. Results suggest that it is a temporary control measure which nevertheless can be repeated. It may also prove useful as a test to identify those patients who may experience anesthesia dolorosa. It is suggested for patients with toxic responses to medical therapy and as an alternative to more invasive surgeries for the long-term management of trigeminal neuralgia.


Pain infirmity and psychotropic drugs in oncology.

The treatment of cancer pain by psychotropic drugs is a method which has been employed for a long time [8] and in which the results obtained have appeared very interesting from the beginning: there is a high percentage of success, rapid action, absence of addiction, and although there are sometimes unpleasant side-effects, they are reversible when the treatment is stopped. Even after several years of application, this therapy still sets some unsolved problems. Some consider that psychotropics are not real analgesics, but that they work on the emotional reaction rather than on the pain itself [3]. Still others consider that the results are obtained only at the price of a state of prostration of the patient similar to that obtained after lobectomy. Finally, this procedure is reproached as having unpredictable results and indications difficult to define. We think that what has, up to now, prevented these types of problems from being solved has been the absence of a really objective evaluation of the pain in the patients observed. We have wrestled with this problem for several years [1,2], and offer the following hypothesis: what is important in considering chronic pain is, above all, the infirmity conferred upon the patient. If "pain" in the broad sense of the term lends itself to objective evaluation with difficulty, it is not the same with respect to infirmity. A method of evaluation of the physical disability intended for routine practice in a cancer center has been used on a series of 100 patients. The results obtained in this series have been analyzed and give the answer to questions such as mechanism of action, indications of psychotropic drugs and prognosis of cancer pain.


Relationship of patient semantic pain descriptions to physician diagnostic judgments, activity level measures and MMPI.

A consecutive sample of chronic pain patients presenting themselves for evaluation was studied. A set of 25 bi-polar adjectives was developed from medical records of previous pain patients' descriptions of their pain. Using the Semantic Differential (S-D) method, each patient rated the extent to which his/her pain was best described by either of the adjective pairs. The S-D findings were then compared with three other data sets and those data sets were compared with each other. First, six physicians classified each patient along an "organic"--"non-organic" continuum based on ratings derived from the full set of medical diagnostic labels each patient had accrued. Secondly, each patient, prior to examination, had completed up to two weeks of diary forms at home on which were recorded amount and distribution of time among sitting, standing/walking, and reclining. Finally, each patient completed a Minnesota Multiphasic Personality Inventory (MMPI). Few and only marginally significant relationships between patient semantic descriptions of their pain and the other measures were found. Secondly, physician agreement ot the "organic"--"non-organic" criterion, using diagnostic labels as their data, was statistically significant but clinically modest. The most substantial findings were between walking hours per week recorded on diary forms and five MMPI scales. Patients who walked more were less depressed, had fewer diffuse somatic complaints, and described themselves as less frustrated or angry and as less hypersensitive in interpersonal situations. The major conclusion of the study is that chronic pain patients present sets of interrelated problems too complex to be discriminated reliably by a single set of measures; particularly, by simple word sets.


Structural changes of experimental glomerulonephritis in rats as revealed by microdissection.

Microdissection revealed striking alterations in the nephrons of animal models AICN and anti-GBMN. The most severely damaged AICN kidney in the series presented marked heterogeneity in the size and deformity of the proximal tubules, corresponding to the diverse and variegated nephrons described by Oliver in chronic Bright's disease. The severely damaged anti-GBMN kidneys revealed widespread alterations in the proximal tubules, which, however, tended to be fairly uniform among the affected nephrons. This uniformity, perhaps, reflected the shorter duration of disease in the anti-GBMN animals. The most characteristic proximal tubular alteration in either the AICN or anti-GBMN animals was that combining atrophy of the pars convoluta and hypertrophy of the pars recta in same tubule. The largest and smallest nephrons encountered in the entire study were found in the most severly damaged kidney in the AICN. This finding reflected the simultaneous existence of regressive and progressive changes in this kidney, as certain nephrons underwent hypertrophy to compensate for the atrophy and disappearance of others. The remarkable functional glomerulotubular balance of single nephrons known to exist in both AICN and anti-GBMN was matched by the evidence presented here of structural glomerulotubular balance among the severely altered nephrons of a representative kidney from each group. Microdissection studies directed toward the characterization of the nephronic alterations in both membranous glomerulonephritis and proliferative glomerulonephritis of man should be of interest in relation to the findings in these experimental rat models of human disease.


An association between the onset of rigor and loss of vascular competence in early myocardial infarcts.

Experimental myocardial ischaemia was induced in 12 anaesthetized mongrel dogs by ligation of the circumflex branch of the left coronary artery. Twenty minutes after ligation 1% sodium fluorescein injected into the artery distal to the ligature evenly perfused the left ventricular wall in the vicinity of the posterior papillary muscle (PPM) but, when injected 90 minutes after ligation, the PPM and adjacent subendocardial myocardium was not perfused by this tracer. Measurements with a linear variable transducer and standard load demonstrated that after 20 minutes ischaemia, the PPM had a similar percentage compressibility to the corresponding unaffected anterior papillary muscle but, after 90 minutes, the compressibility of the PPM was significantly reduced. Scanning electron microscopy of the marginal zone between the perfused and unperfused parts of the myocardium revealed many collapsed vessels which contained small groups of tightly packed erythrocytes indicating that the loss of vascular competence was probably due to the plugging of small vessels by erythrocytes.


The effect of rigor mortis on the passage of erythrocytes and fluid through the myocardium of isolated dog hearts.

The effect of normal and artificially induced rigor mortis on the vascular passage of erythrocytes and fluid through isolated dog hearts was studied. Increased rigidity of 6-mm thick transmural sections through the centre of the posterior papillary muscle was used as an indication of rigor. The perfusibility of the myocardium was tested by injecting 10 ml of 1% sodium fluorescein in Hanks solution into the circumflex branch of the left coronary artery. In prerigor hearts (20 minute incubation) fluorescein perfused the myocardium evenly whether or not it was preceded by an injection of 10 ml of heparinized dog blood. Rigor mortis developed in all hearts after 90 minutes incubation or within 20 minutes of perfusing the heart with 50 ml of 5 mM iodoacetate in Hanks solution. Fluorescein injected into hearts in rigor did not enter the posterior papillary muscle and adjacent subendocardium whether or not it was preceded by heparinized blood. Thus the vascular occlusion caused by rigor in the dog heart appears to be so effective that it prevents flow into the subendocardium of small soluble ions such as fluorescein.


Bilateral pulmonary agenesis.

Two cases of bilateral pulmonary agenesis in infants of 31 and 41 weeks gestation are reported. In addition to absence of both lungs, major abnormalities not previously reported were also found in other systems. It is concluded that these abnormalities are not due to chromosomal abnormality but no alternative can be offered.


Sporotrichosis: a review of 39 cases.

In a series of 39 cases of cutaneous sporotrichosis, 37 were of the localized cutaneous type and only 2 of the lymphangitic variety. Fungi were identified histologically in every case, most often as asteroid bodies which were found in all but 3 lesions. Cultures were occasionally negative even though asteroids or yeast-like forms were present in tissue sections. A histological diagnosis should be possible in all cases if a sufficient number of serial sections are examined by H & E and the PAS method.


Malignant haemangiopericytoma: case report and ultrastructural study.

A case of a rapidly fatal malignant haemangiopericytoma arising in the deep tissues of the face is recorded. The histology of the tumour was anaplastic but otherwise typical. Ultrastructural study confirmed the diagnosis by revealing tumour cells with delicate interdigitating cytoplasmic processes and basement membrane formation resembling pericytes. A feature of the tumour not previously recorded in haemangiopericytoma was ciliogenesis.


Chromosome analysis of bone marrow samples from country patients.

Co-operation by outside centres has made it possible to offer bone marrow chromosome analysis to patients living up to 1800 kilometres from the central cytogenetics laboratory. Diagnostic karyotypes have been obtained in 9 of 10 bone marrow specimens which were partially processed before being transported to the city laboratory for final analysis.


Respiratory suppression and swallowing from introduction of fluids into the laryngeal region of the lamb.

Introduction of 0.9% NaCl or undiluted fetal tracheal fluid into the laryngeal region produced no suppression of breathing in lambs during the perinatal period. As NaCl or tracheal fluid solutions were increasingly diluted with water, progressively greater respiratory suppression associated with rapid swallowing was observed. Introduction of amniotic fluid was associated with variable suppression of respiration. The swallowing induced by the dilute solutions was as rapid as two swallows per sec. Lambs 3 months of age swallowed when water was introduced into the laryngeal region, but were able to alternate swallows between breaths without suppression of breathing.


A new variant of mannosidosis with increased residual enzymatic activity and mild clinical manifestation.

A partial deficiency of alpha-mannosidase was found in cultured skin fibroblasts, serum, and extracts of leukoytes in two siblings with mild mental retardation, delayed speech, a suggestion of coarse or full facies, and limited mobility of the large joints. All other lysosomal enzymes tested were within the normal range. Their father demonstrated intermediate alpha-mannosidase activity. The addition of 2 mM Zn++ caused a 40% increase of the alpha-mannosidase activity in cell extracts of both patients and control subjects. pH profiles and Cellogel electrophoresis of the patients' cells indicated 20% residual activity of the acidic alpha-mannosidase isoenzyme (pH optimum at 4.0), whereas the activity of the isozyme with pH optimum of 6.0 was normal. Increasing substrate concentration (1--10 mM) demonstrated a 4 to 5-fold increase in the apparent Km of the acidic alpha-mannosidase in the patients' fibroblasts. This residual activity, however, was apparently not sufficient for the normal catabolism of mannose-containing molecules, since electron microscopic examination of the cultured fibroblasts demonstrated numerous lysosomal storage bodies.


Splenic sequestration and ineffective erythropoiesis in hemoglobin E-beta-thalassemia disease.

A 13-year-old Thai female with hemoglobin E-beta-thalassemia was evaluated for anemia and splenomegaly. Globin chain synthesis in a whole cell system revealed an absence of betaA chains and excessive alpha chains. The alpha/betaE + gamma ratio was 1.26 in bone marrow and 1.90 in peripheral blood. The average gamma/betaE ratio in bone marrow and peripheral blood was 0.36 compared to peripheral blood concentrations of 49% hemoglobin E and 51% hemoglobin F. Homologous red cell 51Cr half-life increased from 22.7 days to 32.8 days after splenectomy. Total circulating hemoglobin increased from 112.9 to 149.7 g. Endogenous carbon monoxide productive (Vco) as a measure of total heme catabolism decreased from 2.00 to 1.54 mumol/hr/kg. Ineffective erythropoiesis was manifested by an increased Vco/Vheme-c ratio of 7.52.


X-linked immunodeficiency with increased IgM: clinical, ethnic, and immunologic heterogeneity.

Two cases of immunodeficiency with increased IgM are reported. Patient 1 was a black male 3.5 years old who had recurrent pyogenic infections, failure to thrive, oral thrush, and systemic cryptococcal infection. Patient 2 was a 9-year-old white female who had recurrent cervical abscesses. Serum immunoglobulin determinations by radial immunodiffusion in both patients showed marked depression of IgG and IgA and marked elevation of IgM. A low molecular weight circulating monomeric IgM was demonstrated by immunoelectrophoresis and gel filtration in the second patient; this was not present in the first case. In vitro impairment of cellular immunity was observed in both patients. Administration of dialyzable leukocyte extracts (transfer factor) led to improvement of cell-mediated immunity in patient 1. The etiology of this syndrome apparently has several different genetic bases. These patients demonstrate heterogeneity in genetic, ethnic, immunologic, and other features of the syndrome.


Salivary amylase in cystic fibrosis: a marker of disordered autonomic function.

Total serum amylase was determined in 83 patients with cystic fibrosis (CF) (14 of whom had no steatorrhea), 21 control patients, and 6 patients with Shwachman's syndrome. Patients with CF who had steatorrhea and therefore lacked pancreatic function had the same serum levels pre- and postprandially as the control subjects, whereas patients without steatorrhea and, therefore, with evidence of pancreatic function, had significantly higher levels. The patients with Shwachman's syndrome had significantly lower total serum amylase than the three other patient groups. Only the patients with Shwachman's syndrome demonstrated a significant postprandial rise in activity. Only the salivary isoenzyme was detected in patients with Shwachman's syndrome or CF and apparently absent pancreatic function, whereas CF patients without steatorrhea had both salivary and pancreatic isoenzymes. These results demonstrate that patients with CF have an increase in circulating salivary amylase isoenzyme whereas patients with Shwachman's syndrome do not. The increase probably reflects an autonomic hyperstimulation of salivary secretion which is primary to CF, and unrelated to the state of pancreatic activity.


Fetomaternal potassium relations in the rat on the twentieth day of gestation. II. Effects of maternal hypokalemia.

Time-corrected measurements of transplacental and transamniotic electrical potentials were made in potassium-deficient rats on the 20th day of gestation. These averaged 6.4 mV and 2.0 mV, respectively, in the deficient group as compared with 14.3 and 18.4, respectively, in the controls. The measured maternal potassium concentrations were 2.8 mEq/liter in the K+-restricted animals and 4.8 mEq/liter in the controls. These figures were substituted into the Nernst equation in order to predict fetal concentrations in plasma. Similar predictions were made for amniotic fluid assuming equilibrium with fetal plasma. Predicted concentrations in fetal plasma were 2.3 mEq/liter in the deficient animals and 2.8 mEq/liter in the controls. By contrast, observed time-corrected potassium concentrations in fetal plasma were 4.0 mEq/liter in deficient animals and 2.9 mEq/liter in controls. Amniotic fluid values were predicted to be 4.7 mEq/liter in deficient and 2.5 mEq/liter in control animals. These values were compared with the observed averages in amniotic fluid of 4.7 and 4.6 mEq/liter in the deficient and control groups, respectively.


Pulmonary diffusing capacity in lambs during the early neonatal period.

A rebreathing method was used to make 53 measurements of pulmonary diffusing capacity (DLco) and functional residual capacity (FRC) in 17 newborn lambs during the first 2 days of life. DLco, FRC, and DLco/FRC all increased in studies made at 24--48 hr of age compared to those at 2--4 hr of age: DLco 0.86 +/- 0.18 to 1.52 +/- 0.09 ml/min/torr; FRC 37 +/- 10 to 60 +/- 8 ml and DLco/FRC 2.52 +/- 0.75 to 2.89 +/- 0.37 ml/min/torr/ml X 10(-2). DLco measured using 0.005% CO in the test gas was not different than that measured using 0.5% CO.


Distortions in fetal growth standards.

The study analyzed several errors incorporated in widely used fetal growth standards. Such standards contain measurements from many infants who died as neonates. The present study found many of these nonsurvivors to be growth retarded so new standards were developed which contain only measurements from survivors. The study also found that weights and other body measurements of prematurely born neonates cluster at intervals consistent with maternal vaginal bleeding at monthly intervals in early pregnancy. Such bleeding is often mistaken for the last menstrual period. To avoid this error, the probability plot method was used to separate the clusters of measurements at each gestational age. The cluster with the lowest values at each gestational age was used as the data base for the new growth standards. Standard deviations from most mean values in the new growth standards are much smaller than are such deviations in the older standards.


Maturation of p-aminohippuric acid transport in the developing rabbit kidney: interrelationships of the individual components.

The excretion of both endogenous organic (aryl) acids, such as benzoic acid, and exogenous ones, such as p-aminohippuric acid (PAH), penicillin, and furosemide, is reduced in the human neonate and other immature animals. The unique developmental pattern of aryl acid transport in immature rabbit kidney cortex slices is produced by the interrelationships of PAH uptake, efflux, and amount of intracellular binding protein, all of which reach mature levels at different ages.


Abnormal colonization of neonates in an intensive care unit: means of identifying neonates at risk of infection.

The current investigation was designed to select infants in the neonatal intensive care unit (ICU) at highest risk of infection. The data, derived from a prospective study of 223 neonates, indicate that neonates in an ICU develop their initial aerobic pharyngeal flora according to one of the three patterns shown in Table 1, one of which (low titer or no growth) is dependent on antibiotic therapy. Of particular importance is the finding that infants with normal flora (alpha-streptococci predominating) or cultures showing low titers (less than 10(4) colony-forming units (cfu)/ml) or no growth did not become infected. All infections occurred in the group of infants with abnormal pharyngeal colonization (18 infections in 115 abnormally colonized infants): with a single exception the infecting organism showed the cultural characteristics of the colonizing organism. Consequently one can pinpoint the relatively small number of infants at particular risk of infection and predict what the infecting organism will be if infection occurs.


An animal model simulating acute infective upper airway obstruction of childhood and its use in the investigation of croup therapy.

Twelve heartworm-free mongrel dogs (10 males, 2 females) mean weight 22.2 kg (range 17.7--28.2 kg) were sedated and placed in a supine position with the neck extended. A double tracheotomy was performed under sterile conditions. The first tracheotomy tube was inserted 2 cm above the sternum in the direction of the carina using a shortened Silastic American tracheotomy tube (id 10 mm); the second was inserted two cartilage rings proximal to the first and approximately 5 cm distal to the larynx. All animals were kept deeply anesthesized by repeated iv injections of 3--6 mg/kg pentobarbitone sodium so that all reflexes (in particular laryngeal and palatal) were abolished. After control translaryngeal pressure measurements were obtained, the vocal cords were visualized, and steam was introduced onto the through the cords for a mean of 10 sec from above. For 2 hr after this, repeat pressure measurements, as described above, were made. After an overnight stabilizing period, two to three of five randomly chosen air environments were passed over the larynx at 10 liters/min via the proximal tracheotomy, and four half-hourly pressure measurements taken with control periods of at least 2 hr, as described above, separating each experiment. Environments utilized were as follows: i) "cold dry" air, i.e., air at a mean temperature of 9 degrees, obtained by passing compressed air through a coil placed in a solution of alcohol/Dry Ice; ii) "cold moist" air, i.e., air as above, subsequently passed over cold water at a mean temperature of 11 degrees; iii) "warm dry" air, i.e., air at a mean temperature of 36.5 degrees, obtained by passing compressed air through a copper coil, immersed in hot water; iv) "warm moist" air at a mean temperature of 36 degrees, obtained by passing compressed air through a heated Puritan humidifier; v) "ultrasonically produced mist" at room temperature (24 degrees), produced by a De Vilbiss ultrasonic nebulizer 900, set at maximum output. Six 2-hr experimental periods were utilized for each regimen of treatment (i--v). The mean of the combined experimental resistance calculations for each environment were compared statistically with those of its similarly acquired control data, expressed as a positive or negative percentage change, and displayed as a histogram. In addition, the data from each experimental environment were compared with the others, and mean percentage changes from control against time were calculated. Results indicate no statistically significant change in resistance from control for ultrasonic mist or warm moist treatment regimens. Warm dry, cold dry, and cold moist treatments, however, all produced significant reductions in translaryngeal resistance (74%, 60%, and 54%, respectively), there being no difference between cold dry and cold moist and warm dry and cold dry. Significant differences between warm dry and cold moist treatments were obtained, the former decreasing translaryngeal resistance the greatest amount...


Apnea, hypoxemia, and aborted sudden infant death syndrome.

To find out whether there is any relationship between the ventilatory response to hypoxia and the sudden infant death syndrome (SIDS), we studied the effects of mild induced hypoxia (PIO2, 120 mm Hg = 17% oxygen) in 16 infants aged 2 weeks to 6 months. Eight had recurrent apneic spells (apnea group) (five had aborted SIDS and three had recurrent apnea in the intensive care nursery) and eight were "well" preterm infants about to fly in a pressurized airplane (PIO2, 120 mm Hg) (control group). Mean birth weights were 2,245 and 1,400 gm and mean gestational ages were 35 and 30 weeks. Postconceptual ages (41.8 and 41.3 weeks) were almost identical. Heart rate was obtained from an ECG, and respiratory rate and pattern were obtained from a pneumogram. In addition, end-tidal PCO2 and PN2 or PO2 were obtained with a nasal catheter and gas analyzers. In the apnea group with inhalation of 17% oxygen, we observed an increase in periodic breathing and an increase in both rate and total duration of respiratory pauses. In the control group there were no significant changes. Heart rate and PCO2 did not change in either group. Our findings suggest that infants prone to apnea may have unique respiratory responses to mild induced hypoxia.


Comparison of transcutaneous oxygen tension with arterial oxygen tension in newborn infants with severe respiratory illnesses.

Transcutaneous oxygen tension (tcPO2), measured by two skin electrodes of different design, and arterial oxygen tension (PaO2), measured by an intravascular oxygen electrode, were continuously recorded for periods of six hours in 15 newborn infants with serious respiratory illnesses. Ten of the infants needed mechanical ventilation and three needed continuous positive airway pressure. One skin electrode had three microcathodes surrounded by a heated ring-shaped anode, and the other had a large heated cathode. The temperature of both electrodes was set at 44 degrees C and they were calibrated in vitro. The tcPO2 recorded by the electrode with the microcathodes was found to estimate PaO2 reasonably accurately for the whole six-hour duration of the study. The tcPO2 recorded by the electrode with the large cathode gave a similar estimate of PaO2 for three hours, but then tcPO2 often fell relative to PaO2. This fall was probably caused by skin changes at the electrode site. For a variety of reasons, our results suggest that measurement of tcPO2 is unlikely to replace continuous intravascular measurement of PaO2 in infants with severe respiratory illnesses.


Transcutaneous oxygen monitoring in aminophylline-treated apneic infants.

Transcutaneous PO2 (tcPO2) monitoring offers a new approach to the evaluation of drug effects. We investigated the effect of theophylline on ten premature infants with apnea. Theophylline was administered as aminophylline, 8 mg/kg per rectum every 12 hours for two doses and 4 mg/kg every 12 hours for a total of two or five days (short and long courses). The tcPO2, heart rate (beat-to-beat), and thoracic impedance were continuously monitored during each of three 4-hour study periods: 12 hours before theophylline administration, 12 hours after initiation of theophylline therapy, and 24 to 48 hours after discontinuing the drug's use. Plasma levels were measured by a radioimmunoassay developed in our laboratory. Polygraphic recordings were analyzed without knowledge of treatment for frequency of apneic spells, mean duration of apneas, total duration of hypoxemia (tcPO2 less than or equal to 40 torr), total duration of hyperoxemia (tcPO2 greater than or equal to 100 torr), basal tcPO2, heart rate, and respiratory rate. In each case during theophylline use, cardiorespiratory patterns were altered, respirations were more regular, apneic spells were reduced, PO2 was stabilized with less hypoxia and hyperoxia, and bradycardic episodes were decreased. There was considerable variation in the response of the ten infants and a significant difference in the frequency of return of symptoms between those receiving short-term therapy and those receiving the longer course.


Surface morphology of polyvinyl chloride and silicone elastomer umbilical artery catheters by scanning electron microscopy.

The surface features of a new silicone elastomer umbilical artery catheter have been defined by scanning electron microscopy and compared with those of a standard polyvinyl chloride catheter. Little difference was noted in the surfaces of the catheter materials themselves, but the radiopaque material used to render the catheters visible by x-ray resulted in a considerable difference in the overall smoothness. The polyvinyl chloride catheter's marker was in the form of a wide, rough band extending the length of the tubing whereas the marker particles of the silicone elastomer were dispersed, more uniform in size, and resulted in less overall irregularity of the surface.


Age-dependent closure of the patent ductus arteriosus by indomethacin.

Indomethacin has been shown to be effective in closing a patient ductus arteriosus (PDA) in small premature infants. However, the age range over which this therapy is effective remains undetermined. Eighteen infants ranging in age from 4 to 45 days were studied. All had auscultatory and clinical findings indicative of PDA. Seventeen had roentgenographic evidence of cardiomegaly and/or increased pulmonary vascular markings, and eight had ECG evidence of ventricular hypertrophy. Indomethacin was administered to most patients in two doses of 0.1 to 0.3 mg/kg 24 hours apart. In eight of 12 patients, 3 week of age or younger, the PDA closed after indomethacin therapy. Two patients had a decrease in the intensity of murmur and improvement of congestive heart failure after treatment, but the PDA did not close completely. Only patients 33 weeks of age (actual age) or younger responded to indomethacin therapy with complete ductal closure. The condition of patients 34 to 36 weeks of age improved but there was not complete closure; in patients older than 36 weeks there was no response. The data suggest an age-related mechanism for PDA closure and that treatment with indomethacin before 33 weeks of age is probably required. A role for prostaglandin in ductal patency is postulated.


Chest physiotherapy in the newborn: effect on secretions removed.

The amount of upper airway secretions removed by suction alone was compared to that removed after chest physiotherapy in six neonates with a clinically suspected problem of increased respiratory tract secretions. More secretions were removed after physiotherapy.


Comparison of acute-phase reactants in pediatric patients with fever.

We compared WBC count greater than or equal to 15,000/cu mm (high WBC count). Wintrobe ESR greater than or equal to 30 mm/hr (high ESR), temperature greater than or equal to 40 degrees C, and positive slide tests for C-reactive protein (CRP) at a serum dilution of 1:50 in febrile, ambulatory children. The CRP test was performed with and without heat inactivation of serum. An excellent correlation was found between noninactivated and inactivated CRP test results. Since the noninactivated CRP test can be done quickly, its results would be readily available in an outpatient setting. High ESR demonstrated the best balance of specificity and sensitivity for bacteremia, pneumonia, and other possible or proved bacterial illnesses. A positive CRP test was highly specific for these diagnoses but less sensitive than an ERS greater than or equal to 30 mm/hr. Three combinations of acute-phase reactants, high WBC count and/or high ESR, high ESR and/or positive CRP test, and high WBC count and/or high ESR and/or positive CRP test performed as well as high ESR alone. Each was less specific but more sensitive than high ESR for possible or proved bacterial illnesses. The evaluation of an ambulatory, febrile child with acute-phase reactants should include at least determination of ESR.


Control of nosocomial respiratory syncytial viral infections.

We evaluated methods to control the spread of respiratory syncytial virus (RSV) on our infants' ward during a community outbreak of RSV infection. Methods included isolation and cohorting of infected infants, strict handwashing, use of gowns, and the cohorting of staff to the ill infants. Of 123 infants studied, 36 were admitted with RSV infections. Of the remaining 87 contact infants, eight (19%) acquired nosocomial RSV disease. Three of the eight developed pneumonia and one died. Of the 43 staff members, 24 (56%) became infected and 82% were symptomatic. Four acquired repeated infections within weeks of the initial infection. Studies a year previously had revealed that 45% of contact infants and 42% of the staff had acquired nosocomial RSV infections. Thus, the employed procedures appeared to have decreased the transmission of RSV to infants but not to the staff. Staff may continue to be infected by large droplets from close contact with ill infants or by self-inoculation of contaminated secretions.


Dietary fluoride supplementation for the prevention of caries.

Fluoride supplements are effective in preventing dental caries if conscientiously taken on a daily basis from birth until at least 12 to 14 years of age. The optimal dose depends on the child's age and the existing fluoride concentration in the water supply. A dosage schedule is recommended that decreases the dose of fluoride in infancy and that is inversely related to the concentration of fluoride in the drinking water.


Use of prothrombin complex concentrates in hemophiliacs with inhibitors: clinical and laboratory studies.

Nine patients with severe classic hemophilia and inhibitors against factor VIII were treated for 156 bleeding episodes with 503 infusions of Proplex, Konyne, or Auto-Factor IX, three preparations of prothrombin complex concentrates (PCCs). Approximately two thirds of the bleeding episodes were managed successfully. Although the prothrombin time (PT) and partial thromboplastin time (PTT) were shortened after most PCC infusions, there was no evidence of disseminated intravascular coagulation. The degree of shortening of PT or PTT was not related to the particular PCC preparation used, dose, or cessation of hemorrhage. All PCC preparations contained activated clotting factors, as manifested by their ability to shorten the PTT of normal plasma, factor-VIII-deficient plasma, and factor-IX-deficient plasma. Shortening, which was greater with Auto-Factor IX than with the other products, was inhibited partially by a factor IX antibody and blocked completely by prolonged incubation with plasma. Although the nature of the procoagulant material in PCCs is uncertain, these products are of proven benefit to hemophilic patients with high-titer inhibitors. Side effects have been minimal and inhibitor titers have not risen.


Improved student learning of infant growth and development.

All physicians who care for children require a knowledge of normal growth and development in order to detect and interpret abnormalities. Yet traditional instruction tends to focus on the ill child and rarely provides students the opportunity to follow up the cases of healthy children over a prolonged period of time. A new course was developed that enabled students to observe the longitudinal developmemt of a normal infant from birth to age 16 months. At the completion of the course, a multiple-choice examination covering developmental concepts from birth to age 6 years was administered to the students who participated in this course (experimental group) and to two comparison groups. The experimental group scored substantially higher than both comparison groups on items covering birth to age 18 months. The program offers an opportunity for medical students to observe the longitudinal development of a normal infant and appears complementary to other instructional methods.


Psychosocial functioning of mothers of malnourished children.

The relationship between infant malnutrition and maternal psychosocial behavior was explored by comparing mothers of malnourished children with mother whose children were matched for age and family income but were not malnourished. The mothers were interviewed and asked to describe their relationships with their children, their children's fathers, extended families, friends, and employers. The mothers of malnourished children described more chronically disrupted lives. Their housing conditions and employment records reflected disorganization. They had fewer social contacts except with extended families who supervised excessively. The fathers of their babies were either not present or unsupportive. Relationships were more stereotyped, transient, and focused on material aspects. The mothers narcissistic concerns took precedence over the needs of their children. Nearly all the mothers, including the controls, had suffered severe deprivation in childhood. Some mothers of malnourished children were apathetic and dependent, whereas others were manipulative and evasive. These findings were consistent with those previously described for mothers of children with "failure-to-thrive" in affluent countries.


Pulmonary function abnormalities in asymptomatic children after hydrocarbon pneumonitis.

To assess the effect of hydrocarbon pneumonitis on the developing lung, we studied the pulmonary function of 17 asymptomatic children, 8 to 14 years after the initial insult. Fourteen of the 17 subjects (82%) had one or more pulmonary function abnormalities, the most frequent being a high volume of isoflow. Volume of isoflow, ratio residual volume to total lung capacity, slope of phase III, flow rates at 50% and 25% of vital capacity and 60% of the total lung capacity, one-second forced expiratory volume, and maximum midexpiratory flow rate differed significantly (P less than .05) from values in control groups. Closing volume and closing capacity were not significantly different. Residual abnormalities present in children after hydrocarbon pneumonitis can be explained on the basis of small airway obstruction and/or loss of elastic recoil. These children may be at risk for the development of chronic lung disease as adults when they are exposed to exogenous factors such as air pollution or smoking.


Aminophylline therapy for idiopathic apnea in premature infants: effects on lung function.

The effect of aminophylline on lung function was studied in 14 premature infants with apnea (gestational age, 30.2 weeks; birth weight, 1.052 gm). The infants showed compromised pulmonary function with a large difference in alveolar-arterial PO2 gradient (83.9 mm Hg), a low normal lung compliance of 1.1 ml/cm H2O . kg, and a normal inspiratory resistance of 45.3 cm H2O/liter/sec. Aminophylline therapy did not change these functions significantly, but it decreased the incidence of apneic episodes from 29.7 to 4.4 per day. The effectiveness of aminophylline in treating apnea in premature infants must be related to its central stimulating effect and not to an improvement in lung function and oxygenation.


Manometric diagnosis of lower esophageal sphincter incompetence in infants: use of a small, single-lumen perfused catheter.

Lower esophageal sphincter (LES) pressure is frequently measured to diagnose LES incompetence in adults. The multilumen perfused catheters with large diameters that are used for ths purpose are not suitable for small infants. We measured LES pressure in ten normal newborns with a small, single-lumen perfused catheter and compared our values with those obtained with the standard adult apparatus. Higher pressures were recorded with the single-lumen catheter. Chloral hydrate sedation had no effect on LES pressure. Two-day-old infants had LES pressures comparable to those of adults and older children. The technique was applied to the diagnosis of LES incompetence in 23 infants. Infants with LES incompetence (chalasia) were correctly separated from infants with chronic vomiting secondary to all other causes. Single-lumen manometric studies provide a simple, reliable, and safe method of assessing LES incompetence in small infants.


The use of furosemide in the treatment of edema in infants and children.

One hundred thirty-seven courses of furosemide therapy were given to 106 hospitalized pediatric patients with salt and water retention associated with cardiac or renal disease. The diuretic was effective and safe in the pediatric age group when administered acutely as a parenteral medication and over a long-term course by the oral route in the doses and at the time intervals used in this study. On the basis of each kilogram of body weight, the infants with edema as a result of cardiac failure and the children with edema secondary to renal disease responded equally well to furosemide therapy.


Clinical pharmacokinetics of aspirin.

Aspirin is very rapidly absorbed from the gastrointestinal tract when administered as a solution, and somewhat more slowly when administered in tablets. It is rapidly hydrolyzed in the body to salicylic acid; the plasma concentration of the latter must be maintained within a relatively narrow range to obtain an adequate anti-inflammatory effect and to minimize systemic adverse effects. The two major pathways of salicylate elimination, i.e., formation of salicyluric acid and salicyl phenolic glucuronide, become saturated at relatively low body levels of the drug. Consequently, steady-state ("plateau") salicylate levels increase more than proportionately with increasing daily dose, and the time required to reach steady state increases with increasing daily dose. The renal clearance of salicylic acid increases markedly with increasing urine pH; antacids capable of increasing urine pH can therefore cause a pronounced lowering of steady-state salicylate concentrations under clinical conditions. There are pronounced intersubject differences in salicylate elimination kinetics; dosage must be individualized on the basis of plasma concentration and clinical response. The drug is readily transferred across the placenta and is only slowly eliminated by the newborn infant. The drug is also transferred from mother to nursing infant through the breast milk.


Pathophysiology of acetaminophen overdosage toxicity: implications for management.

Acute acetaminophen intoxication was studied in the dog to characterize pathogenesis and in the mouse as a model for antidotal research. In the dog, overt toxicity was manifested principally by cyanosis, facial and paw edema, gastrointestinal disturbance, and coma. Typical laboratory findings were methemoglobinemia, hemoconcentration, leukocytosis, and hepatic centrolobular necrosis. In the mouse, physical signs of acetaminophen overdose appeared to be central in origin; sequelae included anemia, leukopenia, thrombocytopenia, and hepatic centrolobular necrosis. The antidotal profile of acetylcysteine in mice was characterized. When acetylcysteine therapy was instituted early (one hour after acetaminophen overdose), it conferred dose-related protection from lethality coupled with hepatoprotection, as judged from transaminase activity. When acetylcysteine therapy was instituted relatively late (4 1/2 hours after acetaminophen overdose), its beneficial effect on survival persisted but was unaccompanied by distinct hepatoprotection, indicating that SGPT activity was an unreliable prognostic indicator. Acetylcysteine was well tolerated in mice even when administered in the presence of preexisting acetaminophen-induced liver damage.


Aspirin overdosage: incidence, diagnosis, and management.

The importance of aspirin as a cause of poisoning in children has declined dramatically with safety packaging and reductions in the dose of flavored children's aspirin per package. Although flavoring entices children to ingest more tablets, the increment is less than the dose differential between the children's and adults' preparations, and so the latter pose the greater hazard in the individual case. Chronic poisoning of children during therapy with aspirin is aggravated by the peculiar kinetics of the drug, but is preventable and constitutes no essential basis for the substitution of acetaminophen, which may not be devoid of risk factors in sick children. Salicylate levels are essential in the diagnosis and management of intoxication. In treatment, emphasis should be on trapping salicylate in the plasma and eventually the urine--through ionization to prevent its entry into the brain.


Acetaminophen overdose: incidence, diagnosis, and management in 416 patients.

Acetaminophen overdose was studied in 416 patients as part of a nationwide multiclinic open study. Twenty-seven percent of the patients were toxic by plasma acetaminophen level. The 12- to 21-year-old age group had the highest incidence of toxic blood levels (47%). Children under age 5 had the mildest toxicity of any age group; only two of 16 patients had toxic blood levels. No relationship to subsequent Reye's syndrome could be demonstrated. Acetylcysteine is a safe, effective treatment if administered within the first ten hours; it has some efficacy with no toxicity if used after ten hours. Acetylcysteine has advantages over both cysteamine and methionine. All patients in this study recovered with no sequelae.


Chronic salicylate administration in juvenile rheumatoid arthritis: aspirin "hepatitis" and its clinical significance.

Salicylates provide the backbone of therapy in juvenile rheumatoid arthritis. They are effective in controlling the disease approximately 75% of the time if they are properly used. Salicylate administration is relatively safe if carefully done. Serum salicylate levels should not exceed 30 mg/dl routinely. Patients, physicians, and parents should be alert to early clinical signs of toxicity. Chief hazards of chronic salicylate administration other than salicylism (which should be uniformly preventable) include gastric irritation with questionable relationship to peptic ulcer disease, and rare serious hepatotoxicity, bleeding diatheses, or hypersensitivity reactions.


Comparative effects of aspirin and acetaminophen on hemostasis.

Aspirin, even in small doses, has a profound and relatively long-lasting effect on platelet function and hemostasis. This usually produces few clinical problems. However, if a patient has an underlying hemostatic defect, undergoes surgery or sustains an injury, or is a newborn, severe hemorrhage is a potential risk. Aspirin is contraindicated in these clinical contexts. In contrast, acetaminophen has no effect on the hemostatic mechanism unless massive overdose results in hepatic necrosis with depression of synthesis of coagulation factors. Acetaminophen can be used when indicated in clinical situations where the use of aspirin may be potentially dangerous.


Estrogen treatment of teenage girls in the United States: description of the treated population.

It is estimated that each year approximately 20% of all 15- to 19-year-old American girls receive estrogens in the form of a pharmaceutical agent. The proportion of blacks in this age group who receive some form of estrogen therapy is estimated at 36%. The great majority of estrogen use is for the purposes of contraception and, to a lesser extent, suppression of lactation following childbirth. Less than 1% of the girls in this age group receive estrogen for treatment of medical conditions.


Postmenopausal carcinoma as a result of estrogen treatment: survey of evidence.

Recent studies concerning the relationship of exogenous estrogens and endometrial cancer have been reviewed and the objections to their conclusions discussed. Even considering the most serious of these reservations, it is difficult to avoid the tentative conclusion that estrogens are causally linked to endometrial cancer and that the attributable risk is numerically sizable. Whether the medical costs will be ultimately shown to outweigh the benefits of estrogen replacement therapy will depend largely on whether or not a relationship to breast cancer is shown and on the magnitude of the effect of estrogens in preventing serious complications of ovarian failure. For the time being, treatment of menopausal symptoms with estrogens should be brief as possible, should use the lowest dose possible, and should probably be given in cyclic fashion, particularly to women with intact uteruses. Every attempt should be made to give patients a quantitative understanding of both potential risks and benefits before they decide in favor of or against estrogen therapy.


Closure difficulty, figure-size expansion, and figure-size constriction on 240 Graham-Kendall Memory-for-Designs records.

To determine the frequency of occurrence of closure difficulty, figure-size expansion, and figure-size constriction, 240 university students were administered the Graham-Kendall Memory-For-Designs test. Subjects were volunteers enrolled in undergraduate psychology courses and were between 18 and 23 yr. of age, male or female, and U.S. citizens. The purpose was to define a sampling of drawing styles exhibited in a normal group on this test. Each drawing style, or factor, was treated as an independent variable, and the frequency of occurence of each level (dependent variable) was compared via chi square. It was noted that a significant number of subjects exhibited some degree of closure difficulty with the designs (X2 = 101.72, p less than .01). Figure-size expansion was noted as extremely rare for this sample (X2 = 444.78, p less than .01). Finally, extreme figure-size constriction occurred significantly more often than either moderate or no contriction of the designs (X2 = 62.58, p less than .01).


Spatial problem-solving strategies as functions of sex.

Sex differences in spatial ability may be related to different problem-solving methods. Two spatial tests, Choosing A Path and Witkin's Rod and Frame Test, and related strategy questionnaires were given to 50 male and 50 female adults. Multivariate analyses of variance on the strategy questionnaires were significant. Women were more likely to use concrete and unorganized methods and to give up or skip problems. Spearman correlations between the males' and females' rank orders of strategies were high and significant, so that both similarities and differences between the sexes were demonstrated.


Inter-judge agreement for identifying stuttered words.

Inter-judge (n = 5) agreement for identifying stuttered words of an 18-yr.-old college woman was studied. In contrast to earlier studies, the agreement (.91) was considerable. The high agreement seems to reflect the duration, complexity, and consistency of the subject's stutterings.


A multidisciplinary approach to the expression of pain in psychic depression.

The expression of cold pressor pain was measured by recording simultaneously verbal magnitude estimates, heart rates, and facial displays of 16 recently hospitalized depressed patients, and 16 nondepressed adults. Independence of the two groups for the depression factor was verified using the Hamilton Scale for Depression and the 100-mm line self-rating scale. Verbal responses and amount of time the ice bath was tolerated, as well as heart-rate measures, indicated that depressed individuals were significantly more sensitive to the pain stimulus. However, this elevated intolerance to pain was not reflected by marked changes of facial display. Reasons for this discrepancy are discussed. Nondepressed subjects, although clearly able to verbalize intensity of pain, were much less reactive to the pain along all dimensions.


Relationships between MMPI measures of psychopathology and WAIS subtest scores and intelligence quotients.

This study investigated relationships between MMPI scale elevations and WAIS subtest deviation scores and Full Scale intelligence quotients in 272 psychiatric patients. Analysis indicated few significant relationships between MMPI elevations and WAIS deviation scores, especially when compared with the high proportion of significant relationships found between demographic variables and WAIS measures. MMPI elevations were significantly related to Full Scale IQ. Although the results do not support the idea that specific WAIS subtest scores are related to psychopathology, they do support the idea that general intelligence is negatively related to psychopathology.


Aetiological factors in dyslexia: I saccadic eye movement control.

A study was performed to examine the character of saccadic eye movement in dyslexic and normal children and normal adults. No significant differences were noted between 20 normal and 20 dyslexic children, but significant differences were noted between both groups of children and the adult population. Results were explained as reflecting no measured difference in duration/amplitude and velocity/amplitude functions in dyslexic and normal children, thereby indicating no differences in their saccadic eye-movement control functions. Observed differences between normal adult and child populations are explained.


The WISC-R and WRAT as indicators of arithmetic achievement in juvenile delinquents.

The relationship of the WISC-R Arithmetic and WRAT Arithmetic scales to grades was determined for a sample of 8 female and 14 male juvenile delinquents. The WISC-R correlated .538 with grades and .302 with the WRAT, while the WRAT correlated .289 with grades. The WISC-R also correlated .508 with sex (females scoring higher). While WRAT Arithmetic is questioned as an indicator of academic achievement, WISC-R accounts for 29% of grades variance and may be a useful correlate.


Apparent reversals as a function of perceiver group, distance, and geometric shape.

3 groups of 3 men (high, medium, low perceivers) reported more reversals for trapezoids than rectangles. Responses were sensitive to distance, shadowing, and target, as Cahill suggested.


Strength of association between sex and field dependence.

An index of the strength of association between sex and each of two measures of field dependence, the Rod and Frame Test and Embedded Figures Test, was calculated based upon reported significant sex differences on these measures. Median omega2 values based upon 20 rod-and-frame results and 18 embedded-figures results are .11 for the former and .12 for the latter. It appears that gender accounts for less than 15% of the variance in field-dependence scores.


Effect of mothers' voices on auditory comprehension in children.

Normal-speaking/hearing 2-, 3-, and 4-yr.-old children (N = 15) were asked to point to pictorial representations of words in a forced-choice task. The auditory stimuli were randomized tape-recorded presentations of each subject's mother and a male stranger. An analysis of variance indicated that subjects, regardless of age, understood their mothers better.


Self-reported consistency of normal habitual sleep durations of college students.

A relationship between hours of sleep/night and the consistency of this normal daily sleep duration was observed for 763 college students who had rated themselves as good sleepers with stable and fairly well established patterns of sleep. Congruent with a limited literature, these data suggest that shorter sleep durations are likely, for college students, to be relatively recently acquired patterns of sleep.


Influence of set on perception.

To examine the role which preparatory set plays in visual perception three groups of undergraduates were presented 10 items displayed tachistoscopically in .125-sec exposures. The two experimental groups were each given a different preparatory set relevant to the content of the items. The third group, the control group, was given no preparatory set. Six of the 10 items displayed were ambiguous items which might be seen as belonging to either set. Although the two experimental groups perceived more items correctly than did the control group, an analysis of the responses indicated that preparatory set did influence perception. Each experimental group perceived three times as many items as belonging to its set as did the other group.


Perception of the etiology of illness: causal attributions in a heart patient population.

This study examined perceived causes of myocardial infarction in a patient population of 345 men previously free from significant medical problems. Investigation of their perceptions following the life-threatening illness crisis indicated that stress and tension factors were the causes most commonly cited. Possible social and psychological correlates are analyzed using an attribution theory framework, and their implications are discussed.


Tactile-perceptual functioning and academic performance in brain-impaired and unimpaired children.

The purpose of this study was to determine whether tactile perception is a useful predictor of academic performance. 50 control and 42 brain-impaired (epileptic) children were examined. Both groups showed a strong relationship between tactile perceptual integrity and academic achievement. Analyses of extreme groups indicated that academic performance is clearly ordered by tactile perceptual skill not only for children with documented brain impairment but also for a normal control group as well. The implications for additions to the traditional cognitively oriented psychological test battery were discussed.


Augmentation-reduction and pain experience.

The role of augmentation-reduction in pain tolerance and threshold was examined using Petrie's (1967) criteria for classification of subjects. 14 augmenters did not differ from 14 reducers on pain tolerance and threshold. Trait anxiety correlated with both pain tolerance and threshold, but state anxiety did not.


Human eye color and reaction time.

Response latencies to a visual stimulus were determined for 44 male and 82 female Caucasians. Those with dark eyes had significantly shorter simple (one stimulus) reaction times. Complex (one of four stimuli) reaction times showed a similar, but nonsignificant, trend. Data from an additional 206 male Caucasians  on a one-trial non-practiced reaction task followed the same nonsignificant trend. Darker iris pigmentation apparently correlates with shorter reaction times but more strongly on practiced tasks and on those which do not involve accuracy.


Different levels in processing simple geometrical figures.

An experiment has been conducted in order to extend to geometrical figures Posner's (1969) findings of various levels of processing for alphabetical material. Pairs of simple figures (triangles and trapezoids) were tachistoscopically presented to subjects asked to judge if they were "same" or "different". Subjects were instructed to respond "same" either if the two figures had the same shape or the same name, e.g., a scalene triangle paired with a right-angled one. The results support the theory of different levels of processing also for geometrical figures.


Role of task strategy and stimulus processing in reflective and impulsive tactual matching performance.

14 reflective and 14 impulsive boys performed tactual matching tasks in which frequency of observation of the standard was manipulated by successive or simultaneous presentation and in which stimulus features were controlled so that the amount of stimulus analysis carried out might be inferred. The results suggest that an impulsive performance was improved by simultaneous presentation of the stimuli but even in this condition the impulsive subjects did not carry out an adequate amount of stimulus analysis.


Word recall as a function of bisensory and unisensory split-span task presentation and modality preferences.

This study investigated the effects of visual and auditory modality preferences on work recall in bisensory and unisensory split-span tasks. Subjects tended to perform better in the bisensory rather than unisensory condition. Modality preferences (n = 72 subjects) were generally unrelated to performance in these experimental conditions. These findings suggest that preferences for a modality are better characterized as response biases rather than as increased processing capacity for preferred modality material.


Effect of perspective and slant on perception of apparent size and distance.

An experiement was performed to re-examine the role of linear perspective and floor slant on apparent size and distance scales. Misleading perspective cues did not alter the observers' apparent distance scales but did influence the absolute magnitude of distance judgments; floor slant exerted little influence on apparent distance scales or the absolute magnitude of distance judgments. Neither perspective nor slant manipulation reliably affected apparent size scales or exerted more than a minor influence on the absolute magnitude of size judgments.


Effects of diagnostic labels and subject dogmatism upon the perception of persons.

Slides of unfamiliar individuals were rated on six semantic differential scales by high-dogmatic and low-dogmatic subjects. Subjects were informed that the individuals depicted were either normal, neurotic, or psychotic. Results indicated that diagnostic labels significantly affected the ratings and that one factor (a general Emotional Stability factor) accounted for 77.9% of the rating variance. Level of dogmatism appeared to affect subjects' ratings, although not in the manner predicted.


Comment on "augmentation-reduction and pain experience" by Elton, et al.

Some of the troubling aspects in the work of Elton, et al. (1978) are detailed. Included are matters related to the measurement of pain tolerance, as well as that of reduction and augmentation.


Age, familiarity, and visual processing schemes.

In a partial-report task adults and 5-yr.-old children identified stimuli of two types (common objects and familiar common objects) in two representations (black-and-white line drawings or full color photographs). It was hypothesized that familiar items and photographic representation would enhance the children's accuracy. Although both children and adults were more accurate when the stimuli were from the familiar set, children performed more accurate when the stimuli were from the familiar set, children performed poorly in all stimulus conditions. Results suggest that the age difference in this task reflects the "concrete" nature of the perceptual process in children.


Influence of active encoding on tactile recognition memory for common objects.

Active tactile recognition memory for common objects is compared with passive touch. The recognition memory test occure after a delay between inspection and test of seven days. Three groups of five each have the following conditions and results: Group I manipulated objects at both inspection and test to obtain a recognition rate of 87.9%; Group II manipulated objects at inspection but not at test, i.e., passive touch, to produce a recognition rate of 80.7%; Group III manipulated objects at test but not at inspection to perform at a 72.5% accuracy rate. Object manipulation had a significant effect and the group means were all significantly different (p less than or equal to .05), with the largest performance decrement due to passive touch at encoding. Generally, the findings implicated the importance of object identity in tactile recognition memory which, in turn, is supportive of the depth-of-processing approach to memory.


Mental rotations, a group test of three-dimensional spatial visualization.

A new paper-and-pencil test of spatial visualization was constructed from the figures used in the chronometric study of Shepard and Metzler (1971). In large samples, the new test displayed substantial internal consistency (Kuder-Richardson 20 = .88), a test-retest reliability (.83), and consistent sex differences over the entire range of ages investigated. Correlations with other measures indicated strong association with tests of spatial visualization and virtually no association with tests of verbal ability.


Effects on superiority of ear of intonation of dichotically presented sentences.

A dichotic listening paradigm utilizing sentences of three monosyllabic words examined the effects of intonation on superiority of ear. Five types of intoned contours, i.e., declarative, interrogative, imperative, conditional, and monotone, were employed. Subjects consisted of 20 adults who had normal hearing bilaterally, were native English speakers, and were righthanded in 100% of their daily activities. The results indicated that, when three monosyllabic work sentences with four different intoned contours were dichotically presented, superiority of neither left nor right ear was evidenced. However, the declarative intoned contours, being linguistically less marked, were perceived with significantly less accuracy than the relatively more marked interrogative, imperative, and conditional intoned contours.


Classification of random forms and distortions presented to the left or right visual field.

Twenty right-handed undergraduate men were taught in free vision to categorize distortions of prototypic random forms and were then tested in a Go/No Go task with lateralized tachistoscopic presentations of previously sorted (old) distortions, new distortions, the prototypes, and unrelated forms. Accuracy of performance on positive items increased in the order New less than Old less than Prototype. More false alarms occurred to unrelated items in the right than in the left visual field, suggesting that the trace systems generated during training had different characteristics in the two hemispheres.


Exploratory behavior in rats following bilateral lesions of the anterodorsal caudate nuclei.

9 rats received bilateral lesions to the anterodorsal caudate nuclei and 9 rats underwent sham operation procedures. After recovery the subjects were observed in an apparatus allowing access to familiar or novel stimuli. The caudotomized rats spent a significantly greater proportion of time exploring the novel stimuli. The results were not consistent with the suggestions that following damage to the caudate nucleus animals were unresponsive to novelty, however, they did support earlier findings implicating the caudate nucleus in learning and memory processes.


Field dependence and hemispheric specialization.

It has been suggested that field dependence arises out of deficit in left hemisphere function. There are numerous difficulties encountered in the interpretation of the research leading to this proposition. Problems in the establishment of such cause-effect relationships are outlined, and some future avenues for research are suggested.


Handedness and mental rotation.

Handedness and mental rotation test scores were examined by sex and generation for 801 individuals in 200 families. An orderly relationship between bilateralization of function on the hand preference task and mental rotation test scores was found.


Attentional deficits and word attributes preferred by learning disabled children in grades 2, 4, and 6.

This study investigated the postulated effects of attentional deficits on the relative preferences for associative, acoustic, and orthographic attributes in word recognition by 15 learning disabled children each in Grades 2, 4, and 6. An analysis of errors in recall suggests that the children in the second grade did not encode and retrieve information as did their normal counterparts but rather used several stimulus attributes in retrieval. The children in the sixth grade, however, evidenced a significant preference for the orthographic attribute in recall as did normal second grade children. These findings are discussed as they relate to attention deficits, the capacity of short-term memory and achievement in learning disabled children.


Perceptual aura: not spirit but afterimage and border contrast effects.

This study demonstrates that auras can be produced under laboratory conditions and explained by the principles of afterimages and border contrast phenomena.


Procedural controls in group auditory perceptual listening tasks.

This article discusses experimental procedure controls that can assist in producing more reliable listening task experiments for investigators. Issues addressed relate to using appropriate equipment, tape-processing procedures, and listening sessions controls.


The process of habituation to treadmill walking: a kinematic analysis.

Initial work toward a more definitive kinematic analysis of perceptual-motor processes involved initially in accommodation of naive subjects to treadmill walking is described. The kinematic cyclograph technique holds promise of achieving this.


Aetiological factors in dyslexia: II. ocular-motor programming.

A study is presented in which the preprogramming of saccadic eye movements is examined in normal (16 boys, 4 girls) and dyslexic subjects (19 boys, 1 girl), as well as the patterning of occular-motor differences between subjects, which is consistent with the previous study in which no differences in saccadic control are demonstrated between groups of subjects.


Aetiological factors in dyslexia: III. Ocular-motor factors in visual perceptual response efficiency.

The study concerned the effects of induced interference employing a backward masking paradigm, on the processing of sensory information and on the formation of perceptual-motor responses in 20 dyslexic and 20 normal children. Results indicate that dyslexics do not exhibit impairment in perceptual localization.


Prostheses, pain and sequelae of amputation, as seen by the amputee.

Results of a survey of 19 organizations belonging to World Veterans Federation indicate that major complains of amputees include; poor fitting, poor dissemination of knowledge to doctors and amputees regarding new prostheses, lack of opportunity for "input" from amputees at research level and inadequate measures to deal with phantom and stump pain. Suggested improvements by amputees; decrease in weight of prostheses, reduction in maintenance for swing and stance-phase control units development of recreational prostheses, more frequent checks through use of X-ray and film techniques, particularly during the "break-in" of a new appliance. Older veterans showed increasing concern in regard to development of consequential disabilities arising from amputation; premature arthritic changes in spine and remaining limb, circulatory problems and gastro-intestinal problems due to ingestion of drugs to control pain.


A statewide amputee rehabilitation programme.

Patients are referred to the amputee clinic at the Medical College of Georgia from all areas of the State of Georgia. Most referrals are made a considerable time after amputation and most patients live from 50 to 250 miles from the amputee clinic. A special programme was designed to alleviate some of the problems incurred by the distance and the delayed referral. Physical therapists, prosthetists, social workers, the vocational counsellor and the orthopaedic surgeon co-operate to provide maximum care on the day of the amputee clinic. The cooperative efforts of all members of the team and the ability to plan ahead for optimum rehabilitation maximize the use of patients' time and his rehabilitation level.


Ideas on the suspension of the below-knee prosthesis.

Comparative studies with the PTB-suction prosthesis and the ordinary PTB prosthesis have provided opportunities to analyze and understand some functional differences in the suspension of below-knee prostheses. In a roentgenological study of the stump-socket contact signficantly less vertical displacement in the PTB-suction prosthesis has been shown. A study of the pressure variations in the suction socket verified the theory of the necessary pressure gradient in the PTB-suction socket. Added negative pressure by muscle action was also observed. An electromyographic study suggested a muscular activity pattern in the suction prosthesis similar to that of a normal leg, as a contrast to the ordinary PTB prosthesis where simultaneous contractions of antagonistic muscles seemed to be the usual pattern. The latter is interpreted as a defence reaction. An optimal prosthesis has been deduced. It shall have a soft prosthetic socket with a perfect fit, a suction, adhesion and friction fixation and a pressure gradient between the skin and the socket wall with minimum compression distally. The plaster casting shall be done in a downward modelling manner making available space for soft tissue without compression.


Prostheses for partial hand amputations.

Amputation of part of a hand may create significant functional limitations for the amputee which are difficult to ameliorate by either orthoses or prostheses. To help develop a logical approach to devices for partial hand amputations the patterns of losses have been studied and a division into four categories has been proposed. Transphalangeal, thumb spared; thenar, partial or complete; transmetacarpal, distal; transmetacarpal, proximal. By utilizing basic principles of orthotics and prosthetics and by exercising ingenuity, it is often possible to provide considerable improvement in function and/or cosmesis to individuals with partial hand amputations. Case illustrations and their prosthetic solutions are presented.


Measurement of prosthetic alignment.

An essential part of alignment description is the position and orientation of the socket relative to the rest of the limb. Repeatable measurements of these parameters is hindered by the non-geometrical shape of the socket. A unique axis system has already been defined to enable such measurements to be carried out. The method however, employs an iterative technique and is time consuming. A simple device to facilitate these measurements has been developed and is reported.


A mechanically-actuated wave mattress.

A polio victim who has been in a negative pressure ventilator ("iron lung") since 1950 was in need of an activated mattress to combat the hallucinatory effects of complete immobilization and to assist with body pressure distribution. A mechanical method was used to give the patient's mattress a moving wave form of vertical amplitude 50mm with a period variable from 50 seconds to infinity. His arms rested on separate supports which oscillated 180 degrees out of phase with the trunk support. The device is powered by a 12 volt electric motor operating through two mechanical reduction gear units. It differs significantly in design and purpose from pneumatic ripple mattresses.


Rehabilitation engineering as the crow flies. Part IV--Criteria and constraints.

When engineers function in a biomechanics clinic team, collecting information for the definition and solution of problems, and developing solutions in a logical pattern, then establishment of criteria by which to judge actions and results at various stages are essential. In our procedures, we make the most general statement we can which will indicate the goal we have for the patient or the type of patient being considered. Based on this, we proceed with a breakdown of the goal into increasingly explicit statements keeping the objective in focus. Eventually, with the criteria we need in order to decide "yes or no" to any aspect of the solution developing, we consider the constraints. These we see as imposed by the life-requirements of the patient, the effects of the physical environment, the limitations imposed by the social environment, and the limits of available technology including the skills of the designers, the manufacturing capabilities and the distribution system with which the designers must cope. When a "checklist" of requirements and limits has been established, the "critical eye" watches over the rehabilitation engineer as he in effect watches over himself!


Hand orthosis for various finger impairments--the K U finger splint.

The K U (Kumamoto University) finger splint has been manufactured for the purpose of developing an effective hand orthosis for various finger impairments using simple parts and materials. By combining the W type orthosis for which piano wire is mostly used, and the S type for which fishing line is mainly used, it has been used to good effect on patients with several kinds of finger impairments. The fabrication and adjustment of the orthosis is simple and if the material is in kit form, a doctor or an occupational therapist can at once construct one and give it to a patient. For this reason, the patient will not lose the chance of timely treatment.


[Treatment of hereditary angioneurotic edema with androgens].

Hereditary angioedema (HANE) is a rare, life-threatening disease due to the deficiency of C1 inhibitor (C1 Inh). Androgen therapy has been recently shown to be effective for prophylaxis of Hane attacks. Since life-long androgen therapy may be hazardous, this study was designed to define the minimal doses required for effectiveness. Ten patients from six different families were treated during cumulative 73 months by danazol and/for methandrostenolone. One tablet/day of either drug was the minimal requirement to prevent any attack in all patients. When 3 tablets/day were given, complement abnormalities were simultaneously rapidly reversed. When 1 tablet/day was given the biological effect was barely detectable, except for C2. Serum C2 levels may, therefore, represent the best criteria of androgen therapy effectiveness. Thus, an excellent clinical result can be obtained with much lower doses of androgens than previously stated. This result seemed important with respect to the serious dose-dependent risk of androgens.


[The molecular expression of C1 esterase inhibitor in hereditary angioneurotic oedema. Study of 32 families (author's transl)].

Seventy seven patients from 32 families suffereing from angioneurotic oedema were studied and a single theory of the disorder is suggested. The functional abnormality of alpha2 neuraminoglycoprotein (NGP) or of C1 esterase inhibitor is related involvement of the structure gene (s) controlling its synthesis. Particular immunochemical characteristics of the inhibitor seen in severals families and remaining constant through a number of generations would indicate the existence of a phenomenon of functional haploidism in these heterozygous patients. Treatment of the condition with androgens suggests the existence of two varieties of alpha2 NGP molecules which are differentiated on the basis of their complete or incomplete glycoconjugation in the hepatocyte.


[Ulcers of the neck in hiatal hernias. Perforation into the free peritoneum (author's transl)].

Perforation into the free peritoneum of ulcers of the neck of hiatal hernias is a rare complication. On the basis of one case, which would appear to be the third reported in the literature but the only one in which the special circumstances resulted in the diagnosis being made preoperatively, the authors propose a new therapeutic attitude based upon the physiopathology of ulcers of the neck, combining suturing and reduction of the hernia with anterior repositioning of the greater curvature of the stomach, mobilised according to Thal's technique, and thereby simultaneously producing an anterior hemi-valve.


[Clometacine hepatitis. 2 cases (author's transl)].

Two women (aged 72 and 78 years) developed hepatitis due to clometacine. The main laboratory abnormalities were raised transaminases and blood eosinophil count. In one of these cases, in which the initial histological lesions had the appearance of aggressive chronic hepatitis, there was progression to cirrhosis despite the interruption of treatment. The mechanism of this hepatotoxicity is probably a hypersensitivity reaction.


[Femoro-popliteal venous thrombectomy by a new balloon catheter (author's transl)].

The use of a modified Fogarty catheter has been found to considerably facilitate disobliteration of the femoro-popliteal segment. The absence of vascular trauma is felt to be an important factor in ensuring long-term venous permeability.