Diagnosis of rupture of the fetal membranes by glucose and fructose measurements.

A new method is presented for differentiating amniotic fluid from cervical mucus, based on differences in the respective carbohydrate concentrations present in these fluids. Glucose and fructose are present in high concentrations in cervical mucus during pregnancy, average values being 240 and 30.4 mg/100 ml, respectively. Their concentrations in amniotic fluid, however, are low, averaging 39 and 3.3 mg/ml, respectively. Examination of 215 women between 36 and 42 weeks' gestation, using either glucose or fructose values, revealed a high degree of discrimination between amniotic fluid and cervical mucus. The best results, however, were obtained by employing the calculated linear sum 3.32 log glucose plus log fructose for each sample. Inaccuracy rates were low in comparison to those of other diagnostic methods, such as pH values, ferning, and Nile blue sulfate staining. A further advantage of the method is that it remains fairly accurate even when the time interval between rupture of the membranes and the investigation exceeds 24 hours.


Steroids in human myometrium and maternal and umbilical cord plasma before and during labor.

Concentrations of estrone, estradiol, and progesterone in the myometrium, maternal peripheral vein, and mixed umbilical cord plasma were determined before and during labor by radioimmunoassay. Of the 29 patients studied, 4 underwent elective abortions by hysterotomy in the second trimester of pregnancy, and 25 underwent cesarean section (elective or during labor). The concentration of umbilical cord cortisol in the latter group of patients was also determined. Advanced labor was characterized by a high concentration of estrone in the maternal peripheral vein, the umbilical cord, and the myometrium. Concentrations of estradiol, progesterone, and cortisol in cord plasma also increased during labor. From the second trimester to term pregnancy, increases in the concentrations of estrone and estradiol in the myometrium were proportionately greater than those of progesterone. During labor, there were no significant changes in the myometrial concentrations of estrone, estradiol, or progesterone. During labor in term pregnancies, the myometrial concentration of estrone was higher than that of estradiol.


Serum unconjugated estriol after intravenous cortisol administration in late pregnancy.

Serum unconjugated estriol levels were measured in 44 pregnant women treated with corticoids for the prevention of respiratory distress syndrome (RDS) in premature infants. Intravenous administration of cortisol (1 g every 6 hours for 24 hours) decreased the level of circulating unconjugated estriol to 40% of the initial value after 6 hours and to 27% after 24 hours. During the 2 succeeding days, all the results remain below the pretreatment values. On the third day, the mean value is no longer different from the control value, but in some patients recovery takes more than 3 days.


Prolactin levels during labor.

To test the hypothesis that prolactin (PRL) plays a role in the hormonal events of labor, serum PRL levels in 15 normal secundigravidas were measured on 2 occasions 10-15 days before delivery, at the onset of labor, at cervical dilatation of 5 and 10 cm, at the time of delivery, and on the first, second, and fifth days postpartum. The mean level of PRL was 163 ng/ml +/- 26 ng/ml at the onset of cervical dilatation; it typically decreased with the progress of labor, reaching a value of 140 ng/ml +/- 21 ng/ml at the time of delivery. The differences during the various stages of labor, however, were not found to be statistically significant. Postpartum values were significantly lower (P less than 0.01) on the fifth day after parturition. It is therefore unlikely that PRL is involved in or influenced by the hormonal interplay that occurs during labor.


The effect of a perinatal center on perinatal mortality.

The evolution of a perinatal center over the past 12 years was reviewed. Factors in obstetric practices, maternal morbidity, and perinatal mortality were evaluated. The reduction in conditions leading to uteroplacental insufficiency (toxemia, hypertension, high parity) has been the most significant result. This in turn has led to a decrease in deaths from abruption, asphyxia, and respiratory distress syndrome (RDS). The practice of referring high-risk mothers to a perinatal center for delivery can continue to reduce perinatal mortality significantly.


Professional liability insurance in obstetrics-gynecology.

A geographically stratified sample of Fellows and Junior Fellows of The American College of Obstetricians and Gynecologists (ACOG) responded to a questionnaire, describing the source, coverage, and cost of their professional liability insurance, and the effects this had on patterns of practice. Most physicians carry primary professional liability coverage, although 6% do not. The majority pay less than $8000/year for a wide variety of dollar coverages. Less than half of the surveyed obstetrician-gynecologists carry excess/umbrella liability insurance, and a majority pay less than $2000/year for this coverage. The liability problem has not had a substantial effect on restricting the number of obstetrician-gynecologists, but it has had a major effect on patterns of practice.


Academic manpower for obstetrics and gynecology in the United States.

All 119 medical schools in the United States provided information for a study of academic manpower conducted by the Association of Professors of Gynecology and Obstetrics (APGO). The average number of full-time faculty members per medical school is 13.2, with 2 positions vacant per school. Subspecialists are concentrated in university medical school departments, which also have large residency programs. A perceived need for 46% more faculty over the next 5 years will probably aggravate the shortage.


Ultrasonography in the evaluation of intrauterine transfusion.

Numerous techniques have been described in the literature to aid the obstetrician in safely and easily performing an intrauterine transfusion. None, however, have addressed themselves to the question of whether the transfused cells actually enter the fetal peritoneal cavity. We have used gray-scale ultrasonography to evaluate this question in 4 patients who had undergone a total of 8 intrauterine transfusions. By scanning the fetal abdomen both before and after the transfusion, we were able to identify the transfused fluid within the fetal abdomen in all cases.


Early prenatal diagnosis of soft-tissue malformations.

This study presents 3 cases of severe soft-tissue malformations of the fetus. Prenatal diagnosis was established early in the second trimester, and the accuracy of the diagnosis was confirmed by pathological examination in each instance. A clinically oriented work-up plan for early prenatal diagnosis is proposed.


[Prevention of retinal detachment].

The decision to prophylactically treat retinal lesions which could eventually lead to retinal detachment is often a delicate one. The author attempts to outline the principles of an attitude as reasonable as possible to limit the risks inherent in such a prophylactic treatment and to avoid its excessive use.


Transconjunctival buckling for retinal detachment.

A specially designed ring indentator for transconjunctival buckling of retinal detachments is described. The experience on the first 15 patients treated is reported.


Simple glaucoma before the age of 30 years.

'Juvenile glaucoma' is a meaningless term which should not be used. Glaucoma in young subjects can be hydrophthalmus, secondary or simple glaucoma. In this paper, early simple glaucoma is described in 15 patients (8 males, 7 females) below the age of 30 years, starting in some subjects already at 8 years of age. Inheritance was dominant in 6 patients. Anticipation could be shown in 2 pedigrees through 4 and 5 generations, respectively. The course of the disease was severe in 8 patients, with intraocular pressures over 40 mm Hg and/or heavy functional loss. There were usually no subjective symptoms. The chamber angles were gonioscopically normal in all patients. Miotics were effective but did not normalize the intraocular pressure. Surgical treatment is discussed.


Adult rabbit eye and orbital volumes after periodic intrabulbar injections of silicone.

The intent of this experiment was to determine whether periodic injections of silicone into the adult rabbit eye would alter the volume of the eye and/or orbit. This procedure in growing rabbits had resulted in increased volumes of both the eye and orbit. Nineteen 13-month-old (adult) rabbits were used. In 12 animals silicone was injected into one eye but not the opposite one. No silicone was injected into the eyes of 7 untreated rabbits. In a 9-week period the total amount of ten silicone injections into each treated eye ranged from 5.7 to 7.6 ml. 1 week after the last injection each eye was enucleated, an impression made immediately, and a permanent model subsequently developed. From this the volume was determined. A t test was performed on the mean differences of the eye and orbital volumes of the injected and noninjected sides for each group. No significant differences was found. It was concluded within the limitations of this experiment that there was no evidence that periodic intrabulbar injection of silicone into the adult rabbit eye altered its volume. Thus, this procedure also did not alter orbital volume. In contrast, in the growing rabbit both eye and orbital volume had increased significantly after intrabulbar injection of silicone.


[Encephalic Doppler ultrasonography in everyday ophthalmology].

Encephalic circulation has been studied by Doppler ultrasonography in a number of persons who complained of discreet visual troubles, transient alterations of the uni- or bilateral visual acuity, apparently isolated alterations of the visual fields, or organic or functional alterations of the retina. In a significant number of cases, the examination has shown cerebral vascular alterations--sometimes very severe--which warrant very careful medical supervision and the institution of a sustained vascular treatment.


[Treatment of retinoblastomas, Clinique de Lausanne, 1944--1978].

Retinoblastoma remains a rare disease, but still one which has an uncertain life and functional prognosis. The diagnosis is often made late because the principal signs such as leukokoria and strabismus have not been recognized, thus a conservative treatment is no longer possible. However, when treated at an early stage, a child suffering from retinoblastoma has every chance of a complete cure and even normal vision may be preserved. Irradiation therapy plays an essential role, using local radiation by radioactive applicators or by external Betatron electron beams. Based on 52 cases, the methods of treatment and the results obtained at the Ophthalmology Department of Lausanne are reported, including one case of spontaneous cure of a bilateral retinoblastoma.


[Operation on a congenital cataract in a Siberian Tiger].

A cataract operation in a young Siberian tiger is reported. This 5-month-old tiger, presenting with blindness, showed bilateral residual pupillary membranes and anterior polar cataracts combined with nuclear cataracts. After unilateral intracapsular extraction of the lens, the tiger adapted well to the improvement in sight and behaved almost like an animal with normal vision. The animal died 3 months post-operatively from unknown cause. Histological examination of the aphakic eye showed, at the site of operation on the cornea, an intact epithelium with discrete infiltration of the stroma together with low-grade vascularisation. In the eye not treated operatively, both an anterior polar cataract with adherent pupillary membrane strands and a central cataract were seen. Of particular note, when comparing this large wild cat with the domestic cat, was the marked development of the tapetum lucidum (up to 30 cell layers).


[Dynamics of nitrogen-containing compounds in the hemolymph of 2d- and 3d-stage larvae of the reindeer warblefly (Hypodermatidae)].

The content of the total protein, residual and amine nitrogen, urea and ammoniac depends on the physiological state of the warble fly larvae of the reindeer. Metabolism of nitrogen-containing compounds carries out most intensively in the growing organism and during the moulting period. The moulting period is characterized by the fall in the total protein, residual and amine nitrogen and increase of the ammonias and urea.


[Circadian rhythm of the dropping of engorged larvae in 2 species of ixodid ticks (Ixodidae) and its photoperiodic entrainment].

Daily rhythm of detachment of engorged larvae of Ixodes ricinus L. and Hyalomma anatolicum Koch is regulated by photoperiod. In photoperiodic conditions (16--20 hours of light p. d.) the maximum of detachment in H. anatolicum occurs at the beginning of photophase (the first 4--8 hours), but in I. ricinus it occurs at the second part of photo-phase (12--16 hours after the light is on). The rhythm of detachment is regulated according to the processes of endogenous circadian nature which are maintained in the continuous light and are species-specific. The phase entrainment of detachment in continuous light is also influenced by biorhythms of hosts.


[Basic trends in the research for the control of infected blood-sucking ticks and insects, the vectors of human diseases].

Attention was given to the necessity of control of the infected part of the population of vectors of transmissible infections. For this purpose it was suggested to investigate the nature of parasitism of the agent, to look for synergists of their pathogenic effect and to study the ecology of vectors in order to use control measures against the infected part of the population in the sites of aggregation.


[Histopathological changes in the skin of the rabbit fed on by preimaginal phases of the tick, Alveonasus lahorensis (Argasidae)].

During the feeding of larvae and nymphs of A. lahorensis haemorrhagic inflammation develops in the rabbit's skin. Lymphocytes and macrophages are the dominant cell elements in the reaction while during the feeding of nymphs eosinophilous leucocytes are observed in great number in the infiltrate. Around the oral parts of the tick an infiltration of collagenous fibres of the connective tissue is formed, which serves for a more firm attachment of the parasite, while beneath the proboscis a light band is formed from which the tick sucks the food substratum. By the end of the feeding of nymphs III in the place of skin affection a crust can be formed on account of the host's atrophied tissues, and in the integuments there are formed necrotic foci due to the hystolitic and toxic effect of the saliva.


[Effect of the diapause in the Ixodes ricinus (Ixodidae) tick on the multiplication of the tick-borne encephalitis virus in its body].

The tick encephalitis virus was found to multiply more intensively in ticks developing without diapause. Thus, in larvae developing with diapause, in 4 and 8 weeks after they feed on infected animals, the virus titers were 3.0 and 3.5 lg LD50 respectively. At the development without diapause the titers were 4.3 and 5.3, respectively (P less than 0.05). The virus titers in infected nymphs developing without diapause were 2.5 to 3.9 lg LD50 higher than those of the diapausing individuals. In diapausing larvae and nymphs the tick encephalitis virus remained for a long time (8 to 10 weeks) without fall of titers. The fall of virus titers was not recorded either during the moulting of larvae for nymphs and nymphs for imago but in hungry ticks, which were maintained for a long time at 18 to 23 C, the amount of virus gradually diminished.


Where do the heaviest children come from? A prospective study of white children from birth to 5 years of age.

A prospective follow-up study, from birth to age 5, of height, weight, and weight/height indices in 582 white children was carried out in a suburban private pediatric practice. The purpose of the study was to examine trends in height and weight over time, to evaluate any differences in measures of ponderosity between breast-fed and bottle-fed infants, and to locate the heaviest children at age 5. There were significant correlations between height, weight, the ratio of height to weight, the ponderal index (height/weight 1/3), and the Quetelet index (weight/height2) achieved during the first year of life, and that attained at age 5 years. However, approximately 70% of the variance in weight and ponderosity indices at age 5 could not be accounted for by measurement of weight and ponderosity during the first year of life. Breast-fed and bottle-fed infants did not differ in weight and weight/height indices. There was a modest, but consistent, "tracking" pattern among children in the upper decile for weight and ponderosity at age 5 years in that 30% of them were also in the top decile for weight and ponderosity at age 6 months, and 30% to 40% were in the top decile at age 1 year. More than half of the variance in weight or indices of body proportion at age 5 is not accounted for by these variables in the first year of life, indicating limitations to the generalizability of the concept, that obese infants become obese children.


Further observations on noise levels in infant incubators.

The purpose of this study was to conduct an acoustic analysis of incubator noise under two conditions: when the incubator was associated with different types of life-support equipment; and when impulse noise was created by striking the side of the incubator or by opening and closing the doors of the storage unit. It was found that the life-support equipment increased the overall noise levels of incubators by as much as 15 to 20 dB. Much of this increased energy was in the high frequency region. Impulse signals created by striking the side of the incubator ranged from 130 to 140 dB. A representative impulse for opening the incubator was 92.8 dB, whereas closing the door produced a peak amplitude of 114 dB.


[Karyotypes of the stem eelworms from wild-growing plants].

Unlike the stem eelworms of cultivated plants, which have n = 12, the stem eelworms of wild plants (Picris sp., Taraxacum officinale, Hieracium pratense, H. pilosella, Cirsium setosum and Falcaria vulgaris) possess high chromosomal numbers (from n = 19 in the first to n = 28 in the latter). Due to this the stem eelworms of wild plants must be separated from the collective species Ditylenchus dipsaci. Apparently these forms of stem eelworms are distinct species polyploid in their origin.


[Role of Toxoplasma gondii in human and animal chromosomal pathology].

Toxoplasma gondii was found to cause an appearance of cells with a changed number of chromosomes in the bone marrow of infected white rats. Besides the increase of aneuploid and polyploid cells the infection with toxoplasms inhibits the mitotic activity in bone marrow of rats. The similar changes were observed in blood leucocytes of toxoplasmosis patients.


Treatment of isoimmune thrombocytopenia of the newborn with transfusion of maternal platelets.

Isoimmune neonatal thrombocytopenia is a rare condition with potentially serious complications and mortality of 10%. There are several ways of diagnosing and treating this potential neonatal emergency, but the most simple and effective method we have found is that of transfusing maternal platelets. Two cases are reported in which this type of management was successful.


[Systematic position of Hemiurus macrouri Gayevskaya, 1975 (Trematoda: Hemiuridae)].

On the basis of the structure of the terminal parts of the genital system Hemiurus macrouri Gayevskaya, 1975 is transferred to the genus Glomericirrus. G. ulmeri Campbell and Munroe, 1977 is considered as a synonym of G. macrouri (Gayevskaya, 1975). It is noted that G. macrouri occurs but rarely (1.1%) in Coryphaenoides rupestris from the northern part of the Middle Atlantic ridge.


Mitral valve prolapse syndrome in children with Duchenne's progressive muscular dystrophy.

Myocardial function was evaluated prospectively by noninvasive methods in 20 boys with clinical, biochemical, muscle biopsy, and electromyographic evidence of Duchenne's progressive muscular dystrophy. Auscultatory evidence of a nonejection systolic click suggested mitral valve prolapse (MVP) syndrome in seven patients. Phonocardiography disclosed that the click was mid-systolic in four patients and early in three. Echocardiographic features consistent with this diagnosis were identified in all seven patients and in an additional four. One of these had an apical pansystolic murmur, suggestive of mitral regurgitation, whereas in the other three, prolapse of the mitral valve was "silent". Echocardiographic findings included an abrupt midsystolic, posterior motion (greater than 3 mm beyond the CD line) in five patients, multiple sequence echoes in six, and posterior coaptation of the mitral valve near the left atrial wall in six. The features most characteristic of MVP syndrome was a smooth, pansystolic, anteriorly concave (hammock-like) posterior motion deviating more than 3 mm beyond the CD line. Among the remaining nine patients who did not have echocardiographic evidence of prolapsing mitral valve, none had an early, middle or late nonejection systolic click or a heart murmur, although four patients in this group had moderate to severe scoliosis. These observations document of occurrence of MVP syndrome in children with Duchenne's muscular dystrophy and indicate that its prevalence is high. We speculate that prolapse of the mitral valve in these patients is an expression of the underlying cardiomyopathy characteristic of Duchenne's muscular dystrophy rather than an isolated, dystrophic involvement of the mitral valve leaflets.


Ultrastructural abnormalities of the carotid body in sudden infant death syndrome.

The carotid bodies of four infants who died of sudden infant death syndrome (SIDS) were compared, using electron microscopic techniques, with the carotid bodies of various control subjects. In the SIDS patients, there was a marked reduction or absence of the dense cytoplasmic granules of the carotid chemorecptor cells, as well as a reduction in cell number and size. These ultrastructural abnormalities may be pathophysiologically related to SIDS. A defect in this respiratory control organ could block normal stimulation of respiration during the periods of hypoxia that occur during episodes of sleep apnea in infancy. Further studies by electron microscopy are required to confirm degranulation of the carotid body as a pathognomonic sign of SIDS. Screening of high-risk infants should be directed at studying the carotid body and its mediated responses to hypoxia.


Severe immobilization hypercalcemia, renal insufficiency, and calcification.

Severe hypercalcemia with decreased renal function, hypertension, and renal calcifications developed in a 14-year-old boy who required prolonged immobilization for multiple fractures. Parathyroid hormone was not detectable in the serum. Urinary calcium excretion was high. Initially, mobilization was impossible and the patient was treated with a high fluid intake, low calcium intake, acidification of the urine, furosemide, and a passive exercise program. Renal function improved and renal calcifications resolved but hypercalcemia did not resolve. After mobilization the serum calcium concentration became normal rapidly. This treatment regimen is suggested for use in patients with immobilization hypercalcemia when mobilization is impossible.


Increased muscularity of the pylmonary circulation in victims of sudden infant death syndrome.

It has recently become apparent that some victims of sudden infant death syndrome (SIDS) are chronically hypoxemic before death. In this study, the structural alterations in the pulmonary circulation of 15 victims of SIDS were examined using precise quantitative morphometric techniques applied to the injected and inflated lung. Increased muscularity of the pulmonary circulation has been demonstrated, as evidenced by the extension of muscle into arteries not usually muscularized during the first year of life, and by an increased thickness of the medial wall in some patients. This confirms the findings of Naeye and further justifies the shift of investigative efforts from the events immediately surrounding death to a search for chronic abnormalities.


The chest roentgenogram in cystic fibrosis: a new scoring system.

A roentgenogram scoring system is presented that is useful in the evaluation and follow-up of patients with cystic fibrosis. The system has been shown to be reproducible by and between observers and to correlate significantly with results of pulmonary function tests, Shwachman-Kulczycki scores, and, in a short-term evaluation, morbidity.


Roentgenographic follow-up of acute pneumonia in children.

The need for follow-up roentgenograms documenting complete clearing of pulmonary infiltrates in the pediatric patient with acute pneumonia was studied prospectively. Seventy of 129 children enrolled in the study had a repeat roentgenogram within three to four weeks after initial diagnosis. Twenty percent of this group had residual pulmonary infiltrates. Of the two thirds of those who returned for a second follow-up roetgenogram, the infiltratrates had cleared completely within three months. Routine repeat chest roentgenograms may not be necessary unless there is clinical evidence of persistent respiratory difficulty or failure to thrive.


Hypertension screening in schools: results of the Dallas study.

The purpose of this study was to determine the prevalence of persistent blood pressure elevations in an eighth-grade population composed of three ethnic groups, and to determine the feasibility of using school health facilities for hypertension screening. Blood pressure was recorded in 10,641 subjects (90% of the total eighth-grade population) in the Dallas Independent School District. Blacks made up 46% of the population; non-Latin whites, 40.1%; and Latin-Americans, 13.9%. On the first blood pressure screening, 8.9% had systolic or diastolic pressures or both at or above the 95th percentile. Of those whose blood pressures were elevated on the first examination, 98.3% were reexamined. After the third examination, 1.2% continued to have systolic hypertension, and 0.37% diastolic hypertension. No student had diastolic pressure above 90 mm Hg on all three examinations. The prevalence of persistent hypertension was similar for the three ethnic groups. Analysis of variation in blood pressure measurements revealed that the school nurses introduced a relatively small increase in variability. These data indicate that although school screening initially identifies large numbers of students as having inconstant pressure elevations, subsequent follow-up examinations show that less than 2% have persistent hypertension.


Appendiceal rupture: a continuing diagnostic problem.

The mortality rate for appendicitis in children has remained relatively unchanged since the 1940s, when antibiotics were introduced in the treatment of appendiceal peritonitis. However, since this time the incidence of appendiceal rupture has increased appreciably, presumably owing to a failure of early recognition and treatment. At Columbus Children's Hospital, one half of all patients undergoing appendectomy for ruptured appendix in 1975 had been seen by another physician before admission, but the correct diagnosis had not been made. The history obtained by the primary physician and that given on admission were similar, yet differed from the histories given by patients whose disease had been correctly diagnosed. Findings on in-hospital physical examination of incorrectly diagnosed patients differed from those recorded by the primary physician, but were similar to those of patients whose disease had been correctly diagnosed. Since it is unlikely that the natural history of the disease has changed, the increased incidence of rupture must result either from early misinterpretation of physical findings or from greater delay by parents in responding to the child's illness. Physicians and parents must share the responsibility equally for the increasing incidence of appendiceal rupture in children.


Recurrent pulmonary disease in children: a complication of gastroesophageal reflux.

To evaluate the role of gastroesophageal reflux (GER) as a possible cause of recurrent pulmonary disease, 30 children, aged 1 to 18 years, were studied prospectively with esophageal function tests. These included esophagram (30 patients), esophageal manometry (29 patients), pH probe (Tuttle) test (29 patients), and esophagoscopy with esophageal biopsy (23 patients). The patients studied had either chronic asthma or two or more documented pneumonias within a one-year period. Nineteen (63%) had GER based on two or more positive tests. Eighteen had positive Tuttle tests; 13 had abnormal manometry studies; nine had esophagitis on biopsy; six had esophagitis on esophagoscopy; and five had reflux on esophagram. Of those with GER, 17 had a history of nocturnal cough and eight vomited during infancy. Children with recurrent pulmonary disease should have esophageal function testing to exclude GER as the cause.


L-Glutaric acidemia: investigation of a patient and his family.

A 5-month-old infant had an unusual combination of clinical signs and symptoms. These consisted of irritability, dystonia, lack of head control, grimacing, opisthotonos, choreoathetoid movements, delayed development, and severe metabolic acidosis. Metabolic investigation by gas-liquid chromatography/mass spectrometry detected urinary organic acids. This confirmed the diagnosis of L-glutaric aciduria. The concentration of L-glutaric acid in the patient's plasma was 2.5 mg/dl (normal range, 0 to 0.1 mg/dl), and in the patient's urine was 4.6 mg/mg of creatinine (normal range, 0 to 0.05 mg/mg of creatinine), but the concentration was not elevated in the plasma and urine of the infant's parents nor of two other family members. No glutaryl-CoA dehydrogenase activity was found in leukocytes taken from the patient. Three of the four family members, including the parents, demonstrated 38%, 42%, and 42% activity, respectively, compared with the activity of normal controls. These findings are consistent with an autosomal recessive disorder involving the metabolism of glutaryl-CoA to crotonyl-Co-a. Dietary restriction was instituted on two separate occasions. First, a low protein diet of 1.6 gm/kg of body weight per day was given, then a low lysine intake of 50 mg/kg/day. These dietary manipulations caused a decrease in the plasma and urine concentrations of L-glutaric acid and beta-hydroxyglutaric acid. However, no effect on the clinical manifestations of the disease was noted.


Kawasaki disease: effect of treatment on coronary artery involvement.

Ninety-two patients with Kawasaki disease were treated with five different types of drug therapy: a steroid preparation (prednisolone), aspirin, an antibiotic, a combination of steroid plus aspirin, and a combination of steroid plus warfarin. One or two months after the onset of the disease, coronary angiography demonstrated coronary aneurysms in 20% of cases treated with an antibiotic alone, 64.7% of cases in the steroid-treated group, and 11% of those in the aspirin-treated group. These findings suggest that the steroid might act adversely to cause a progression of coronary lesions of the disease. The aspirin-treated group did not have a significantly lower incidence of coronary lesions compared with the group treated with an antibiotic alone. But in view of the fact that the direct cause of sudden death of the disease is thrombotic occlusion of a coronary artery, aspirin might act as the effective means for prevention of sudden death due to Kawasaki disease.


Clinical characteristics of the afebrile pneumonia associated with Chlamydia trachomatis infection in infants less than 6 months of age.

Respiratory tract colonization with Chlamydia trachomatis commonly occurs in natally acquired chlamydial infection and is sometimes associated with a chronic, afebrile pneumonia that has relatively distinctive clinical characteristics. To further define the frequency and clinical characteristics of lower respiratory tract disease associated with C trachomatis, we grouped 56 infants aged less than 6 months with afebrile pneumonia according to nasopharyngeal shedding of Chlamydia and viruses and compared their illnesses. Forty-one (73%) were positive for C trachomatis (23 had C trachomatis only, while 18 had C trachomatis plus a virus [cytomegalovirus, respiratory synctial virus, adenovirus, rhinovirus, or enterovirus]), and 15 were C trachomatis negative (nine had a virus only, and six had neither C trachomatis nor virus). The 41 infants with C trachomatis alone or C trachomatis plus a virus were similar clinically and differed significantly from other infants in several ways: (1) onset of symptoms before 8 weeks of age; (2) gradually worsening symptoms; (3) presentation for care at 4 to 11 weeks of age; (4) presence of conjunctivitis and ear abnormalities; (5) chest roentgenograms showing bilateral, symmetrical, interstitial infiltrates and hyperexpansion; (6) peripheral blood eosinophils greater than or equal to 300/cu mm; and (7) elevated values for serum immunoglobulins M, G, and A. Pediatrics 63:192--197, 1979, Chlamydia trachomatis, pneumonia, afebrile pneumonia, interstitial pneumonia.


Treatment of chlamydial pneumonia of infancy.

Infants with untreated chlamydial pneumonia shed Chlamydia trachomatis and are symptomatic for may weeks. We used sulfisoxazole, 150 mg/kg/day, or erythromycin ethyl succinate, 40 mg/kg/day, for approximately 14 days to treat 32 patients with chlamydial pneumonia of infancy, and observed them for nasopharyngeal shedding of C trachomatis and changing clinical status. All infants stopped shedding chlamydiae soon after treatment was started. After treatment, three of the 25 infants tested again became culture positive (but did not have clinical relapse). All infants improved clinically. In 24 (83%) of 29 infants, where the onset of improvement could be times, improvement began within seven days of starting treatment. Progression to complete recovery was observed in 27 of 28 infants examined between two weeks and two months of treatment completion. Neither the existence of concomitant viral infection nor the duration of illness or hospitalization before starting treatment influenced the interval between initiation of treatment and onset of clinical improvement. While these observations do not prove, they are at least compatible with the hypotheses that C trachomatis plays a central etiologic role in this illness and that termination of chlamydial infection is beneficial clinically. Pending the availibility of data from controlled studies, we believe that either of the treatment programs outlined warrant consideration in the clinical management of patients with chlamydial pneumonia of infancy.


Spontaneous closure of small ventricular septal defects: ten-year follow-up.

The natural history of small ventricular septal defect (VSD) was studied in 50 infants for up to 10.5 years. The VSD closed spontaneously in 34 patients. Life-table analysis of the data showed that by 10 years of age, 75% of small VSD will close spontaneously; the figure is higher for defects in the muscular septum (83%).


Gastroesophageal reflux in steroid-dependent asthmatic youths.

The aims of this study were to evaluate the incidence of gastroesophageal reflux (GER) in chronic allergic steroid-dependent asthmatic children and to assess whether a medical antireflux regimen might improve pulmonary status of asthmatics found to have reflux. Nineteen patients had a determination of lower esophageal sphincter (LES) pressure, pH assessment after acid instillation into the stomach (acid reflux test), and esophagram. After the reflux evaluation, an antireflux regimen was instituted for three weeks; patients were followed with asthma symptom diaries and weekly pulmonary function tests for this period and for another three weeks after finishing the regimen. Gastroesophageal reflux, diagnosed by positive acid reflux test, occurred in nine patients. Five patients had low LES pressure (less than or equal to 12 mm Hg), and two patients had an abnormal esophagram. There were no significant changes in asthma symptoms or pulmonary function tests with the medical antireflux regimen. Although GER does exist in a high percentage of this patient sample (47%), a short-term antacid and positional antireflux regimen does not improve the pulmonary status of these patients.


Amniotic fluid microviscosity determined by fluorescence polarization: methodology and relation to gestational age.

The fluorescence polarization of 116 amniotic fluid specimens obtained from 22 isoimmunized pregnant women was determined. The degree of fluorescence polarization of amniotic fluid provides an index of microvisocity in lipid aggregates that is dependent on the lecithin-to-sphingomyelin ratio and the degree of saturation of fatty acid side chains. We confirmed the reproducibility of the measurement of amniotic fluid microviscosity (coefficient of variation, 2.0%). The measurements are not effected by bilirubin concentration of amniotic fluid dilution. The pattern of change of amniotic fluid microviscosity during gestation parallels the expected development of the surfactant system. Amniotic fluid microviscosity is high during early gestation and abruptly and sequentially decreases between the 28th and 36th week of gestation. Since the measurements are an accurate reflection of the biochemical properties of amniotic fluid lipids and parallel the development of the surfactant system, we conclude that amniotic fluid microviscosity may well serve as an indicator of the process of fetal lung maturation.


Vaccination of children with human cell culture rabies vaccine.

Three children exposed to the bites of proved or probably rabid animals were immunized with human rabies immune globulin and human diploid cell culture vaccine. There were no significant clinical reactions, and all three developed high levels of rabies antibody. The patients have remained well from four to 18 months.


Early detection of adenomatous polyposis coli in Gardner's syndrome.

Gardner's syndrome, an autosomal dominant disorder, consists of multiple polyposis of the colon associated with various soft- and hard-tissue tumors. The appearance of adenomatous hyperplasia and polyposis in at-risk patients before adolescence has not been full appreciated. Four preadolescent children from a kindred with Gardner's syndrome were examined by use of colonoscopy and mucosal biopsy. In three children (18 months, 6 years, and 9 years old) adenomatous hyperplasia or polyposis was found. The colon of the fourth child (3 years old) was normal. The three affected children were asymptomatic. The youngest had a barium enema and results were normal. The oldest child had polyps. Biopsies revealed focal atypical hyperplasia of the glands with pseudostratification of the epithelial cells, frequent mitotic figures, and the absence of goblet cells. More severe manifestations were noted in the splenic flexure than in the sigmoid flexure or rectum. The youngest patient showed early adenomatous hyperplasia characterized by a marked reduction of the goblet cells, especially in the surface epithelium. Colonoscopy and mucosal biopsies are mandatory in at-risk children. By deferring colonic examination until adolescence, a patient is placed at risk for malignant degeneration of the adenomatous tumor.


Munchausen syndrome by proxy: definition of factitious bleeding in an infant by 51Cr labeling of erythrocytes.

The Munchausen syndrome by proxy is a phenomenon in which symptoms of a disease are fabricated by some person other than the patient. This report describes and 8-week-old infant with repetitive bleeding episodes, presumably originating from the upper respiratory tract. Extensive investigations, including angiography, several endoscopies under general anesthesia, and reinfusion of the infant's red cells labeled with 51Cr followed by pulmonary and upper airway scanning, failed to reveal the source of bleeding. Within two weeks after initiation of the 51Cr studies, radioactivity of facial blood from two separate bleeding episodes did not exceed background counts. Simultaneous examination of the infant's capillary blood, however, showed moderate to marked radioactivity. The Rh subtype of the facial blood was cc, whereas the infant's type was Cc. These findings indicated that the facial blood was factitious in origin. No further "bleeding" occurred after this information was presented to the parents. This case represents an unusual example of the Munchausen syndrome by proxy. Awareness of this entity can prevent potentially harmful investigations. Documenting its occurrence and sharing the information with parents in a nonaccusatory manner may prevent future harm to the patient.


Sickle cell anemia in two White American children: essential laboratory criteria for diagnosis.

A number of hematologic disorders share diagnostic and clinical features of sickle cell anemia but have significantly different genetic implications and prognosis. Because of these differences, the establishment of a precise diagnosis is essential for the child in whom any form of sickle cell disease is identified. To illustrate the requirements for a definitive laboratory diagnosis of sickle cell anemia, this report presents the approach to establishing this diagnosis in two white American patients. From a review of the literature, these patients appear to be the only white Americans with sickle cell anemia in whom the diagnosis has been unequivocally established.


Maternal oral herpes: isolation policy.

Current official recommendations are that parents and personnel with oral herpes lesions be isolated from newborn infants. A survey of 110 neonatal centers, 50% of them replying, showed that most centers do not isolate mothers with oral herpes from their newborn infants. A review of the literature suggest that exposure of infants to personnel or family with oral herpes lesions might occasionally result in disseminated neonatal herpes simplex virus disease. Since the separation of a mother from her newborn infant up to 1 week of ages is a decision that should not be taken lightly, more information is urgently needed to decide whether or not to isolate mothers and hospital personnel with oral herpes lesions from newborn infants. Recommendations to obtain more information concerning these infants are provided.


A new device for diagnosis and treatment of neonatal pneumothorax.

A new closed-system device for the diagnosis and treatment of pneumothorax was evaluated in ten New Zealand white rabbits and compared with and open-system needle. The closed-system device proved to be safe for diagnostic thoracentesis. There were no pneumothoraces as a result of the procedure with the closed-system device as confirmed by chest roentgenograms and pleural pressure measurements. In contrast, 70% of the diagnostic thoracenteses with the open-system needle were associated with pneumothorax documented by x-ray films and a significant increase in mean pleural pressure. The new apparatus was more efficacious for evacuation of pneumothoraces because complete air removal occurred in 90% of the rabbits as compared with 60% of trials with the open-system needle. If the efficacy of the new closed-system device proves to be good in human infants, the pediatrician encountering a tension pneumothorax in the newborn can use a completely assembled system that is safe for diagnosing and treating this acute life-threatening condition.


Diphtheria-pertussis-tetanus vaccine: reactogenicity of commercial products.

Parents from four practices were surveyed to ascertain reactions of children to diphtheria-pertussis-tetanus (DPT) vaccine in the 48 hours after immunization. Vaccines were administered according to current recommendations. Responses were scored in three categories: temperature, behavioral changes, and local reactions. Questionnaires were returned by 1,232 (84.9%) patients. Only 7.0% reported no reaction, while 336 (27.3%) reported mild, 722 (58.6%) moderate, and 88 (7.1%) severe reactions. Over 50% experienced temperatures of at least 100 F, and 80% noted behavioral changes; 72.2% had local reactions. No encephalitis, seizures, or hospitalizations were reported. Reactogenicity was similar for the five immunizations of the recommended series and the two manufacturers evaluated. Reported reactions in the control group were significantly lower than in the study group. These reaction rates underline the need to reevaluate present DPT vaccines.


The pharyngeal effect of partial nasal obstruction.

The case histroy and cinematoradiographic findings of a baby with partial nasal obstruction are presented. This infant's restriction to air entry at the nose led to severe airway obstruction during inspiration by a forward movement of the posterior pharyngeal wall and backward movement of the tongue and lower jaw. At the height of inspiration, there was total airway occlusion in the pharynx. These events can be explained by the pressure drop that takes place behind a restriction if air is sucked through it forcibly from an area of atmospheric pressure. Studies of postpalatal pressures in adults and infants demonstrate such a drop in pressure during nasal breathing if the nose is partly obstructed. If the adult or infant is able to respond to the diminished nasal airway by mouth breathing, there is no postpalatal pressure drop. It is suggested that partial nasal obstruction in a sleeping obligatory nasal-breathing infant could result in a sucking back of the tongue over the larynx in this "cafe coronary" type of situation. This could be the mechanism of the obstructive type of apnea recorded by Steinschneider, and of the asphyxial type of death that is suggested by autopsies on some "cot death" victims. This hypothesis is consistent with the frequency of infection of rhinitis and pharyngitis in victims of sudden infant death syndrome and with the seasonal incidence. Prevention of this obstructive type of apnea would depend on the recognition of infants showing inspiratory and expiratory changes in pharyngeal airway size as can be seen externally by the movements in the carotid triangle of the neck and confirmed by roentgenography or cinematoradiography.


A comparative study of the behavior of Greek neonates.

Three groups of 30 Greek neonates each (an adoptive group from the Metera orphanage, a lower-class group, and a middle-class group) were evaluated at days 1, 5, and 10 after birth using a behavioral scale of 21 items and a neurologic evaluation of 16 items. Behaviors were examined for group differences and group-by-age recovery curves were determined during the first ten days. Significant differences were found in the separate items and items grouped to reflect interactive, motor, and state behavioral dimensions. The adoptive babies at the Metera orphanage generally performed the most poorly. This difference seems not only to reflect intrauterine differences, especially in regard to nutrition, but to point to the likelihood of eliciting less than optimal responses from future environments. The middle-class group had the worst scores on physiologic items and were similar to the Metera babies in having initially depressed interactive, motor, and state behavior. Improvement in these areas over ten days suggested that temporary effects of maternal medication caused the poor scores. The recovery curves of the infants pointed to the important effects of such perinatal variables as maternal medication on early neonatal behavior.


Hydrops of the gallbladder in a child: diagnosis by ultrasonography.

Hydrops of the gallbladder is a rare cause of right upper quadrant mass in children. A case of acute hydrops of the gallbladder in a 5-year-old child is reported. A specific and accurate diagnosis can be made preoperatively only by sonography. Characteristic sonographic findings are described.


Tropical pyomyositis: a case report and review.

Tropical pyomyositis is a disease of skeletal muscle characterized by single or multiple abscesses. The infective organism is most often penicillin-resistant Staphylococcus aureus. Only recently have case reports appeared in the literature from temperate zones; however, this disease is common in the tropics. This report reviews the literature and describes a child from rural North Carolina in whom tropical pyomyositis developed after incision and drainage of a furuncle.


American Academy of Pediatrics Committee on Drugs: anticonvulsants and pregnancy.

There is an increased risk of congenital heart disease and cleft palate among the offspring of women with epilepsy, most of whom are on anticonvulsants. Part of this increase may be caused by phenytoin. The risk of all abnormalities in these infants appears to be about 4% to 5%, which is approximately double the rate of malformations in the general population. There appears to be an ill-defined "syndrome" associated with fetal exposure to hydantoin, although it has not been proven to be caused by the drug. The risk of this "syndrome" is of unknown magnitude, but it is probably less than 10%. The significance to a given child of the abnormalities in this "syndrome" is not predictable at this time.


Periodic breathing in infants with near-miss sudden infant death syndrome.

Twelve-hour nocturnal home recordings of respiration and heart rates were obtained during sleep in 32 infants with near-miss sudden infant death syndrome (SIDS) and in 32 control infants, and the recordings were analyzed for periodic breathing. An episode of periodic breathing was defined as three or more apneic pauses of three or more seconds. The duration of respirations interrupting the pauses was 20 seconds or less. Analysis revealed a statistically significant difference (P less than .001) between the two groups, using criteria of percent of periodic breathing episodes, number of periodic breathing episodes/100 min of recorded sleep time, average duration of all episodes, and duration of the longest episode of periodic breathing. It is concluded that periodic breathing is present in excessive amounts during sleep in infants with near-miss sudden infant death syndrome.


Multifocal myocardial necrosis and fibrosis in pancreatic diseases of children.

From a review of 2,000 autopsies of children, 16 cases of extensive necrosis and scarring fibrosis of the myocardium were found. These lesions involved mainly the left ventricle and spared the endocardium, the pericardium, and the coronary vessels. These necrotic of fibrotic heart lesions were found to be closely associated with various pancreatic diseases: cystic fibrosis (11 cases), pancreatic lipomatosis (2 cases), extensive small bowel resection (3 cases, 2 of which were associated with acute interstitial pancreatitis). To explain these unexpected associations, two hypotheses can be put forth: (1) The lack of absorption of some presently undetermined substances indispensable for the correct trophicity of the myocardium, and (2) the release in the blood of proteolytic enzymes with consecutive activation of phlogistic substances such as kinins.


Strychnine therapy in nonketotic hyperglycinemia.

Nonketotic hyperglycinemia is an inborn error of metabolism resulting from a defect in the glycine cleavage enzyme system. It is characterized biochemically by elevated concentrations of glycine in blood, spinal fluid, and urine. Previous therapies which have been directed toward reducing the glycine concentration in plasma and CSF have not been successful in preventing neurological deterioration, which may be the result of the role of glycine as an inhibitory neurotransmitter. Strychnine treatment was initiated because it is a specific antagonist of glycine at postsynaptic membranes. The patient reported here has shown clinical and EEG improvement while taking strychnine in conjunction with sodium benzoate.


Age, sex, and season of onset of juvenile diabetes in different geographic areas.

Age, sex, and estimated time of onset of insulin-dependent diabetes were determined for children in Pittsburgh (N = 673), Gainesville (N = 976), Galveston (n = 741), and Melbourne (N = 851). The US cities had a decrease in new cases during the summer and peak incidence in January through April. In Melbourne, monthly trends were reversed: there were more cases during May through August. In US cities, but not in Melbourne, children less than 6 years old showed a greater variation by season than children 6 years old and older. Observations of the same fall and winter onset (in different calendar months) of insulin-dependent diabetes in Australia and the United States, and exaggeration of seasonal differences in young US children, suggest that onset of insulin-dependent diabetes is associated with seasonally varying viral diseases. Mumps and rubella infections do not seem to be responsible for much of the seasonal variation. Seasonal peaks of mumps and rubella are later than those observed for insulin-dependent diabetes, and immunization with live mumps and rubella viruses has not been associated with changes in incidence of insulin-dependent diabetes. An increase in disease incidence in boys over girls below age 6 years and in girls over boys at ages 6 through 11 years was consistently observed but not explained.


Alterations in gastrointestinal microflora during antimicrobial therapy for necrotizing enterocolitis.

Changes in the gastrointestinal microflora were noted in 22 infants during combined topical and parenteral antimicrobial therapy for necrotizing enterocolitis (NEC). Gastric and fecal cultures obtained during therapy showed significantly decreased Gram-negative aerobic organisms, most of which were Enterobacteriaceae, when compared with pretreatment cultures. Members of this bacterial family have been implicated in the pathogenesis of NEC in many reports. The data presented here show that the number of organisms retrieved can be reduced with this method of antimicrobial therapy.


Aerobic and anaerobic bacterial flora of the maternal cervix and newborn gastric fluid and conjunctiva: a prospective study.

Aerobic and anaerobic cultures were performed on cervical swabs from 35 mothers and their newborn infants' gastric aspirates and conjunctival sacs. Four hundred seventy-nine isolates were obtained; 287 were aerobes and 192 were anaerobes. The cervical and gastric cultures overall yielded a similar flora, but this was not always the case when one compared mothers with their own newborn infants. The conjunctival cultures yielded about half of the number of bacteria per specimen that the gastric contents yielded, and a repeat conjunctival culture done 48 hours later showed a marked reduction in the number of potentially pathogenic bacteria isolated. The predominant aerobes isolated were Staphylococcus epidermidis, diphtheroids, viridans streptococci and Haemophilus vaginalis. The predominant anaerobes were the Bacteroides fragilis group, Propionibacterium acnes, Peptococcus, other Bacteroides and Peptostreptococcus organisms. Statistical analysis revealed significant positive correlation between the isolation of certain groups of organisms and increased duration of pregnancy, increased baby's weight, and prolonged duration of labor.


Neonatal traumatic proximal femoral epiphysiolysis.

Traumatic proximal femoral epiphysiolysis is a rare event. Early recognition and management are important to minimize sequelae. Evaluation should include plain roentgenograms and arthrography. Treatment should be conservative with open reduction reserved only for severe cases.


Postnatal acquisition of herpes simplex virus by the newborn infant: a review of the literature.

The literature relating to the hazards of herpes simplex virus acquired by newborn infants after birth is reviewed. A total of 24 infants has been reported who have acquired the infection in this manner; 66% of these infants expired. Transmission of virus from mothers, adults (including personnel) other than the mother, and other infants has been reported. In order to prevent this infrequent but life-threatening illness, separation of the newborn infant from any person with active lesions caused by herpes simplex virus is recommended.


A decongestant-antihistamine mixture in the prevention of otitis media in children with colds.

One hundred four preschool-age patients in a private pediatric practice were treated prospectively during colds with either decongestant-antihistamine or placebo to determine if the decongestant-antihistamine mixture was effective in the prevention of otitis media. Each child received a standard dosage of the study medicine according to a double-blind, crossover design. Otitis media was diagnosed on the basis of clinical criteria. Fifteen (6.4%) of 234 colds treated with placebo and 14 (5.8%) of 241 colds treated with the study drug were followed by otitis. The decongestant-antihistamine mixture was not useful in preventing development of otitis media in children with colds.


Emergency room utilization in the first 15 months of life: a randomized study.

The impact of a system of primary pediatric care on emergency room use in a municipal hospital was measured by comparing that use in two randomly selected populations. The population that was offered participation in a primary care program consistently used the emergency room less than did the control group. Analysis of the pattern of utilization revealed that the differences were limited to patients who actually participated in the program, and to weekday use of the emergency room. Unusually heavy (greater than ten visits per year) use of the emergency room was virtually eliminated among participants in the primary care program.


Attitudes and interpersonal skills during pediatric internship.

The initial phases of a program for analyzing interpersonal skills and attitudes and personal adaptation of pediatric interns at a high-pressure tertiary care hospital are described. Interns found internship stressful and less satisfying in many regards than they expected. They were often unable to cope adaptively with the stresses encountered. There were indications of worsening attitudes toward aspects of patient care. However, interns' confidence increased during the year. A videotape vignette test of interpersonal sensitivity showed no change in sensitivity from beginning to end of the year. Videotapes of interns' visits with clinic outpatients made at three points during the year also showed no improvement in interpersonal or communication skills or attention to psychosocial issues. While parents were generally satisfied with the visit, interns lacked skills in some of these areas. None of the data collected, including sex, marital status, and pre-admission rank by the selection committee, predicted end-of-year performance rating by the chief residents. Sex and marital status were also uncorrelated with measured skills and attitudes.


The Alice in Wonderland syndrome in juvenile migraine.

Two children are reported who had recurrent attacks of impairment of time sense, body image, and visual analysis of the environment. These occurred with a clear state of consciousness and in the absence of any evidence of an encephalitic process, seizures, drug ingestion, or psychiatric illness. Both children had recurrent headaches; one was clearly migrainous. There was a family history of migraine in both cases. These children represent examples of the Alice in Wonderland syndrome in juvenile migraine.


Jimson "loco" weed abuse in adolescents.

Over a three-year period, 29 adolescent patients were hospitalized because of intentional Jimson weed ingestion. Their records were reviewed for the presence of signs and symptoms of atropine/scopolamine toxicity clinical course, treatment, and outcome. Twenty-two were male and seven were female. All had mydriasis, hallucinations, and were disoriented. Tachycardia (heart rate greater than 95), dry mucous membranes, and flushed facies were often present. Urinary retention requiring catheterization was present in five patients. Sixty-five percent (17/26) had detectable atropine or scopolamine in their urine. The average length of hospitalization was 1.8 days. No serious complications were encountered during hospitalization and full recovery were noted in all patients. Gastric lavage and hospital admission for close monitoring and medical support are essential phases of management. Physicians who care for adolescents should be aware of this relatively new form of drug abuse and its management.


Ganglioneuroblastoma and fetal hydantoin-alcohol syndromes.

A ganglioneuroblastoma developed in a 35-month-old boy with both the fetal hydantoin and fetal alcohol syndromes. Our case, plus two recent reports in the literature, would very likely establish the relationship between fetal hydantoin syndrome and the development of neural crest tumors. Infants exposed in utero to hydantoins should be closely observed for the development of these tumors.


Effect of supine and prone positions on arterial oxygen tension in the preterm infant.

To determine the optimal position for the preterm infant, arterial oxygen tension (Pao2) was monitored in 16 preterm infants by the transcutaneous method with the infants in both supine and prone positions. When the infants were prone, Pao2 rose by a mean of 7.4 mm Hg (P less than .001), an increase of 15%. In those infants with residual cardiopulmonary disease a 25% increase was noted. The higher Pao2 in the prone position was accompanied by a significant decrease in the amount of time the chest wall moved asynchronously. This improved oxygenation in the prone position appears to be the result of enhanced ventilation/perfusion ratios and not merely secondary to an alteration in sleep state with positioning of the infant. These findings may have important implications in the management of preterm infants, requiring neonatal intensive care.


Effect of posture on the pattern of stomach emptying in the newborn.

To determine the effect of posture on the pattern of neonatal stomach emptying, 56 test meals of a 10% dextrose were given to 14 healthy infants over a period of two or three consecutive days. The infants, whose postnatal age ranged between 5 and 27 days, were studied in each of four positions. The emptying pattern was determined by the double-sampling dye-technique. The results indicate that there are no significant postural differences in the pattern of stomach emptying. They also indicate that neonatal and adult patterns are similar.


Resting oxygen consumption of premature infants covered with a plastic thermal blanket.

Premature infants in single-wall incubators covered with "thermal blankets" made of plastic packing material have large reductions in insensible water loss (IWL) compared with naked infants. We postulated that such reductions inevaporative heat loss would not result in decreases in caloric expenditure if body temperature were maintained by a servocontrolled heat source. Using an open-circuit technique, we measured oxygen consumption (VO2), carbon dioxide production (VCO2), heart rate (HR), respiratory rate (RR), and abdominal skin (Tabd), cheek, thigh, rectal, incubator air, wall, and room air temperatures in ten infants less than 37 weeks gestational age and from 2 to 24 days of age both naked and covered with a plastic thermal blanket. Tabd temperature was maintained between 36.2 and 36.8 C and rectal temperature between 36.8 and 37.2 C in each environment by manual or automatic servocontrol. A "resting state" was defined by using a combination of subjective and objective criteria. The mean values of VO2 during the "resting state" were 7.31 and 7.59 cc/kg of body weight per minute for naked and covered infants, respectively. There were no significant differences between mean values of VCO2, respiratory quotient, HR, RR, abdominal, cheek, thigh, or rectal temperatures in the two environments. Operant temperatures averaged 0.5 C lower when the infants were covered. These data support the hypothesis that decreases in insensible water loss do not necessarily imply reductions in caloric requirements in infants where Tabd is maintained by servocontrol.


Psychological management of acute paraplegia in adolescence.

Important management principles regarding emotional needs of the teenager with acute paraplegia during early hospitalization are discussed. One illustrative case is presented. Mourning reaction of the patient and family to this severe loss of bodily function is reviewed. Reaction of medical caretakers to this catastrophe is also discussed. It is emphasized that these concepts be applied by pediatricians in managing adolescent paraplegics.


School health experience before and after completion of pediatric training.

A questionnaire regarding school health training and current school-based activities was completed by 116 of 141 young pediatricians practicing in four southwestern states. Responses indicated that few (22.4%) were exposed to school health during their residencies, although most (65.5%) are now providing some school health services. Those who had had school health training during residency are much more likely to be providing services than those who did not. Likewise, during their residencies, few received instruction in methods of health education, though many, led again by those who had appropriate residency experience, now participate in school health education programs. Residency programs should provide training in school health to improve the physician's comfort and efficiency in the roles he is presently asked to play, and to increase his involvement in this critically important area of pediatrics.


Physiologic determinants of serum urate levels in adolescence.

During adolescence, serum urate increases and adult levels are achieved. Physiologic factors related to serum urate were investigated in a nationally representative population of 6,768 youths aged 12 to 17 years (the US Health Examination Survey). Serum urate concentration increases markedly from ages 12 to 14 years in males, and levels were related to sexual and skeletal maturation. Although similar relationships were observed in females, the association is less striking, probably because of earlier pubescence, which was not captured in this study, and a pronounced androgenic response. In the later stages of adolescence (ages 15 to 17 years for males and 13 to 17 years for females), body composition (body mass index and skinfold thickness), blood pressure, and hematocrit have stronger relationships than sexual and somatic maturation. These latter relationships are similar to those in adults. This survey affords a portrayal of physiologic interrelationships with serum uric acid during adolescence.


Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis.

The records of all children in the Tucson area diagnosed as having cystic fibrosis (CF) before the age of 12 months were reviewed to ascertain the prevalence of metabolic alkalosis as a major presenting manifestation of CF. Five of eleven infants (46%) in whom CF had been diagnosed between 1 and 12 months of age initially were seen with hypokalemia, hypochloremia, and metabolic alkalosis unassociated with marked dehydration, hyperpyrexia, or major pulmonary and/or gastrointestinal symptoms. Two infants had repeated episodes of metabolic alkalosis; for one of these infants, both episodes of metabolic alkalosis occurred before the diagnosis of CF. It is postulated that chronic loss of sweat electrolytes together with mild gastrointestinal or respiratory illness may predispose the infant with cystic fibrosis to a severe electrolyte and acid-base disturbance. The lack of shock and hyperpyrexia together with the apparent chronicity of electrolyte losses differentiates metabolic alkalosis from the heat prostration syndrome, a more acute complication of cystic fibrosis. Quantitative sweat testing should be part of the evaluation of any infant with unexplained metabolic alkalosis. Serum electrolytes should be assessed regularly in infants with cystic fibrosis during hot weather months.


Accidental childhood iron poisoning: a problem of marketing and labeling.

Accidental iron poisoning still represents a significant hazard in children less than 5 years of age. The problem is compounded by the attractiveness of dosage forms, their high availability, and ambiguities in product labeling. Manufacturers accentuate label ambiguity by not specifically designating the iron as being total elemental iron or iron compound. A list of products that contain iron has been prepared to help clarify this issue.


Transmission of hepatitis B virus infection by asymptomatic chronic HBsAg carrier mothers.

The courses of 18 children born to 13 chronic hepatitis B surface antigen (HBsAg) carrier mothers were followed prospectively for serological and biochemical evidence of type B hepatitis. Three children developed transient HBsAg positivity accompanied by the appearance of antibody to the hepatitis B core antigen. Two others had no detectable HBsAg but developed antibody to HBsAg. These serological manifestations of hepatitis B virus infection occurred late--6 to 24 months after birth. None of the children had clinical evidence of hepatitis and none became chronic HBsAg carriers. The infrequency of transmission of infection, the mild course of disease, and the lack of persistence of HBsAg in these children probably reflected the low level of infectivity of the chronic carrier mothers and perhaps the healthy immunologic status of the children.


Repeated naloxone administration for morphine overdose in a 1-month-old infant.

Repeated injections of naloxone by hydrochloride, a pure narcotic antagonist, were administered to a 3,320-gm 4-week-old boy for treatment of an accidental morphine overdose. The infant received frequent naloxone hydrochloride injections (0.2 mg per injection) during the first 12 hours of therapy to reverse morphine-induced apnea, hypothermia, and flaccidity. A cumulative dose of 2.73 mg of naloxone hydrochloride (0.822 mg/kg) was administered over 27 hours without apparent adverse effect or evidence of toxicity.


Dystonia associated with carbamazepine administration: experience in brain-damaged children.

Carbamazepine is an anticonvulsant most effective in treating complex partial and generalized tonic-clonic seizures. We have cared for three children in whom four episodes of dystonia proceeding to opisthotonus occurred in association with carbamazepine use. The patients, a 4-year-old with microcephaly and severe retardation, a 1-year-old with cerebral dysgenesis, and a 5-year-old with spastic quadriplegia and mild retardation, all had seizures unresponsive to multiple anticonvulsant combinations. In all three patients carbamazepine was introduced and gradually increased to a maximum dosage of 25 mg/kg of body weight per day. Dystonic symptoms began two to three weeks after introduction of therapy and subsided within three weeks after discontinuation. In one child, a second course of carbamazepine resulted in a return of the dystonia. The currently available clinical and neuropharmacologic data suggest that carbamazepine may be an antagonist of dopamine and that this property is responsible for the production of dystonia.


Psychologic adjustment of siblings of children with chronic illness.

Although numerous references are made to the stressful, deleterious effects of chronic or terminal illnesses and handicaps on the siblings of pediatric patients, very few studies have been conducted using comparison groups. The present study compared the adjustment of 3- to 13-year-old siblings of pediatric hematology (N = 62), cardiology (N = 57), and plastic surgery patients (N = 37) with healthy siblings (N = 46). The adjustment measure was an objective, paper-and-pencil measure of children's emotional and behavioral problems, the Louisville Behavior Checklist. On analyses of covariance, the patient groups were more likely to show symptoms of irritability and social withdrawal, and the differences between illness groups approached significance on measures of fear and inhibition. Among the younger children, ages 3 to 6, there were significant group differences, with the siblings of patients undergoing plastic surgery showing the highest level of general psychopathology. Among children ages 7 to 13, male siblings of patients with blood disorders were more likely to show signs of emotional disturbance than female siblings. No group differences were noted on measures of aggression or learning problems. Significant interactions between sex and age relationship to the child were noted on scales of social withdrawal, inhibition, immaturity, and irritability.


Purulent ocular discharge in neonates: significance of Chlamydia trachomatis.

We obtained cultures for bacteria and chlamydiae from 100 infants with conjunctivitis that began during the first month of life. Sixty-nine infants were evaluated during well-child visits (group A); 31 were seen specifically for the ocular discharge (group B). Potentially pathogenic bacteria, predominantly Staphylococcus aureus, were cultured from one third of the infants in each group. Chlamydia trachomatis was recovered from three infants (4%) in group A and from ten (32%) in group B. Three infants with chlamydial conjunctivitis (two in group A, one in group B) had only mild inflammation. Initial treatment with topical antibiotics was unsuccessful in eliminating the organism from seven of 11 infants.


Variations in utilization of health services by children.

In this longitudinal study in two prepaid group practices, many more children stayed at the same level of use of services over a six- to ten-year period than would be expected if use of services had distributed randomly. Overall, about 13% remained consistently in the highest third of the distribution of use, and another 13% remained consistently in the middle or lowest third. If use of services had distributed randomly, 4% and 7%, respectively, would have been in these groups. Conversely, many fewer children (25%) showed fluctuating patterns of use over time than would be expected by chance alone (37%). Although the reasons for this phenomenon are unknown (and may be multiple), the findings have implications both for clinical care and for development of policy regarding the organization and financing of health services for children.


Lactobezoar formation associated with high-density caloric formula.

Two cases of lactobezoar formation were seen in premature low-birth-weight infants in a 24 cal/oz formula fortified with medium-chain triglycerides and glucose polymers given by continuous nasogastric infusion. Medical management included cessation of oral feedings and increased hydration and led to a rapid dissolution of the problem. The use of this preparation in small low-birth-weight infants is not recommended as an initial formula, and it should only be used when oral feedings have been well tolerated in a growing premature infant.


Effect of safety packaging on aspirin ingestion by children.

The effectiveness of child-resistant closures, required under the Poison Prevention Packaging Act of 1970, in reducing the incidence of accidental ingestion of aspirin and aspirin-containing products among children less than 5 years of age has been investigated. Data from Poison Control Centers and the National Center for Health Statistics were analyzed to determine the ingestion level before and two to three years after safety closures were required. Baby aspirin and nonbaby aspirin products were analyzed separately. For baby aspirin. It is estimated that safety packaging has reduced the incidence of ingestions 45% to 55%. For nonbaby aspirin products, the reduction has been 40% to 45%.


Intellectual development and familial dysautonomia.

The study examined adaptive trends in cognitive development among individuals with familial dysautonomia and sought to establish new base rates of intelligence for the dysautonomic population. Fifty-two subjects, aged 6 to 28 years, were administered the Wechsler scales of intelligence. The results indicate that there is less cognitive impairment than previous research would suggest, and that more dysautonomic children are capable of adjusting to standard school programs than was heretofore thought possible. Specific deficits seen in this population are discussed along with a rationale for deleting the term retarded where most of these individuals are concerned.


Food intake and growth of children between 30 and 48 months of age in Jerusalem.

The food intake and body size of a sample of children from a western neighborhood of Jerusalem were determined at 30, 36, and 48 months of age. The mean caloric intake was below the 100% of the Recommended dietary allowance (RDA). The percentage of children receiving less than 100% of the RDA increased slightly with age. Less than 20% of the children had intakes below 60% of the RDA. The mean protein intake was 200% of the RDA. Iron intake met the World Health Organization recommendations. The heme component of the iron intake decreased with age. The mean protein and caloric intake increased as the mother's educational level increased. The weight and height distribution of this population does not differ significantly from the US reference population. At 48 months of age, the children are heavy for their height. Some difficulties of dietary intake studies and the association between food intake and growth are discussed.


Apnea recordings of healthy infants at 40, 44, and 52 weeks postconception.

A total of 129 recordings of the respiratory activity of 46 normal full-term infants were obtained continuously for 24 hours in the hospital nursery at 3 days of life and in the home environment at 4 weeks and 12 weeks after birth. The pediatric pneumogram (PPG) technique, an impedance method, was used. Pneumogram data over longer than 16 hours was obtained on 77% of infants monitored. No infants experienced apnea longer than 15 seconds in duration at 40 and 44 weeks postconception, or greater than 11 seconds at 52 weeks postconception. Twenty-four hour plots of hourly apnea frequency revealed a marked variability, with evidence of clustering of apneic episodes during periods of reported sleep. Longest apnea time and hourly frequency of apneic episodes were highly correlated.


Lung function in adolescents receiving high-dose methotrexate.

Methotrexate (MTX) has been implicated as a cause of interstitial pneumonitis and/or fibrosis, but the mechanism by which the drug causes these processes is not known. The purpose of this study was to determine whether patients receiving high-dose MTX developed a consistent decrease in pulmonary function, which would implicate a role for total dose of MTX received in the pathogenesis of the lung toxicity. Pulmonary function studies, including spirometry, plethysmography, and diffusing capacity at two levels of alveolar PO2, were performed in 38 adolescents treated for osteogenic sarcoma. The patients were divided into three groups including 12 patients (group 1) studied before and during therapy, 15 patients (group 2) studied during therapy, and 11 patients (group 3) studied after completion of treatment. While total dose received at the time of the study varied from 0 to 256 gm/sq m, pulmonary function showed no change, with one exception. A mild restrictive defect and decrease in diffusing capacity due to unilateral pleural and diaphragmatic disease, whose relationship to MTX therapy is uncertain, developed in one patient. MTX, when administered in high dose to young patients by the described protocol, causes no dose-related decrease in pulmonary function.


Cloprednol therapy in steroid-dependent asthma.

Cloprednol is a new oral corticosteroid with a short half-life that is presently under investigation for use in asthma. Seventeen steroid-dependent children and adolescents were switched from daily treatment with prednisone to cloprednol for a one-year study. Patients showed a statistically significant improvement in symptoms while receiving cloprednol therapy. Two patients had extraordinarily good responses. Growth trends, bone age, and chest roentgenograms were not remarkably changed by cloprednol. Pulmonary function was stable. There was a significant improvement in fasting morning cortisol levels and ability to respond to metyrapone and adrenocorticotropic hormone (ACTH) challenge. Cloprednol appears to be a promising drug for steroid-dependent patients who require oral corticosteroid therapy.


Long-term use of propranolol, ibuprofen, and spironolactone in the management of Bartter's syndrome.

This report concerns two patients with Bartter's syndrome who were treated with propranolol, spironolactone, and potassium supplements. When ibuprofen was added to this regimen, potassium supplements were no longer required. In both patients, plasma renin activity decreased, plasma volumes increased, and a "catch-up" in linear growth ensued. This report confirms others that indicate prostaglandin synthetase inhibitors are a useful adjunct in the therapy of Bartter's syndrome.


Ferrochelatase deficiency in an infant with anemia and growth delay.

A 5-month-old infant with hypochromic anemia and iron overload secondary to ferrochelatase (heme synthetase) deficiency is described. Decreased activity of iron-containing enzymes in the absence of any other proven cause is suggested as the main cause of the associated growth retardation.


Cerebral cysticercosis.

A case of cerebral cysticercosis in an American child is described. The patient was only 2 years old and had never left the United States. Her symptoms began with febrile seizures and progressed to focal motor seizures. Cerebrospinal fluid pleocytosis with eosinophilia, candle-guttering of the walls of the ventricles on pneumoencephalography, and a titer of 1:4,096 against cysticercosis antigen in her blood led to the diagnosis. Over a five-year follow-up period, the patient's course has been one of resolution of her symptoms, improvement in her electroencephalogram, and excellent seizure control with anticonvulsant therapy. Cysticercosis should be considered in the differential diagnosis of a child who shows CSF pleocytosis with eosinophilia, particularly if accompanied by focal seizures.


The frequency of pericardial effusions in bacterial meningitis.

Because of our experience with four cases of purulent pericarditis complicating bacterial meningitis during a 13-month period, we performed a prospective study to determine the frequency of this complication. Echocardiograms were done on 100 patients with bacterial meningitis. Small or moderate pericardial effusions were detected in 19 patients, but none had symptoms or signs related to the effusion. Pericardiocentesis was done in one infant; all the other effusions resolved spontaneously. Patients with effusion were significantly younger than those without this complication, but no other significant risk factors were identified. A literature survey indicated that symptomatic pericarditis occurs in fewer than 1% of patients with meningitis. Conversely, in series of cases of purulent pericarditis, associated meningitis was reported in 12% of patients. We concluded that pericardial infection is common in patients with meningitis but that it is usually of no clinical significance and resolves with antibiotic therapy.


Left atrial myxoma: association with acute coronary embolization in an 11-year-old boy.

An 11-year-old boy had clinical signs of an acute inferior wall myocardial infarction. On echocardiography, a left atrial myxoma was demonstrated. Coronary artery embolization from myxoma was confirmed by selective coronary arteriography. The tumor was removed 12 days after acute coronary embolization. The clinical value of echocardiography is demonstrated in this report of a successful surgical excision of left atrial myxoma in a child with a myocardial infarction due to tumor embolization.


Inheritance of uncomplicated hypospadias.

Isolated first-degree hypospadias in three generations of one family and in two generations of two families is reported and the literature reviewed. Pedigree information suggests autosomal-dominant, sex-limited inheritance in these cases although other inheritance patterns are possible. Mendelian transmission of hypospadias may be more frequent than commonly believed and implies that the abnormality is potentially definable in molecular terms.


The right not to read.

Skill in reading is desirable. However, the importance of reading may be overemphasized in schools. Reading skills are determined relatively and not absolutely. Thus, relatively poor readers will persist. Schools cannot eradicate individual differences. Biological makeup and societal pressures are the important factors in determining reading skill. Present methods of reading remediation are of questionable efficacy and are traumatic to some children. Time with its associated normal development succeeds in remediating the majority of children with dyslexia. Most poor readers eventually attain reading levels that enable them to comprehend the types of printed materials commonly encountered. If a child finds reading difficult or distasteful, that child should be encouraged to read but should have the right not to be forced to read.


[Thyroid hemiagenesis (single thyroid lobe). 8 cases (author's transl)].

Thyroid agenesis would appear to be a rare abnormality (50 cases, including 8 personal). In fact, it is a simple anatomical curiosity which may be found in the presence of any type of thyroid condition (simple goitre: 4 cases, thyrotoxicosis: 2 cases, cold nodule: 2 cases, as far as our own patients were concerned). Our 8 patients were all women, hemiagenesis being more commonly discovered in the female sex. In 2 patients the right lobe was absent, and the left in the other 6. In general, the abnormality affects the left lobe three times more commonly than the right. The differential diagnosis essentially lies with autonomic thyroid nodules, which are much more common, and isotope scanning provides the answer.


[Haemoperfusion on coated activited charcoal. Experience in French anti-poison centres based upon 60 cases (author's transl)].

Sixty three sessions of haemoperfusion on coated activated charcoal were carried out in 60 cases of poisoning. The indications concerned 2 clinical situations: - massive poisoning with coma (by hypnotics or chlorine-containing solvents) with high extracellular concentrations of the toxic agent, with the aim of reducing the duration of the coma; - intoxications with a high mortality rate, such as those due to paraquat, colchicine, digitoxine, tricyclic antidepressants, and heavy metals, even when plasma levels were low in relation to the dose ingested. Such discordance may be related not only to predominantly intracellular fixation, but also to low intestinal absorption. The effectiveness of haemoperfusion was estimated in terms of the amount of toxic substance extracted, measured either indirectly by repeated calculation of the arteriovenous difference multiplied by the flow rate through the column, or directly by elution of the column. This method for the removal of toxic substances would not appear to be life-saving, is too costly for the extraction obtained and is not free from specific complications. Thrombocytopaenia remains an epiphenomenon, but the frequency of infection is significantly higher than with haemodialysis. An effective and safe method for the vascular extraction of poisons, particularly one favourising removal of toxic substances from intracellular localisation, remains to be found.


[Malabsorption syndrome during the initial phase of a case of severe rheumatoid purpura].

We report a case of severe Henoch syndrome in which the first manifestation was digestive with a prolonged and important intestinal malabsorption syndrome. The clinical, radiological and histological data allow to consider a malabsorption due to lesions of the chorion and submucosa probably secondary to the allergic vasculitis. The malabsorption syndrome, confirmed by the intestinal absorption tests, the study of which must be more systematic, seems to belong to the severe and polyvisceral forms of the Schönlein-Henoch syndrome.


[Total iliectomy with preservation of the lower limb (author's transl)].

We treated a chondro-sarcoma of the ilium by total resection of the hemipelvis preserving the lower limb. We describe our technique and give the fonctionnel result of this operation which we believe could in certain cases replace a hemipelvectomy.


Regulation of hemopoiesis.

The development of in vitro cloning systems for populations of hemopoietic and lymphoid cells has enabled a family of specific regulatory macromolecules to be detected and characterized. These macromolecules control proliferation and differentiation in hemopoietic cell populations. Studies have demonstrated the complexity of the spectrum of molecules which are involved in the regulation of each group of hemopoietic cells. Heterogeneous subpopulations of molecules exist for each regulatory function and more than one molecular form exists for the various regulator molecules. While the cellular origin of many of these regulators has not been clearly demonstrated, it is apparent that hemopoietic populations themselves can be significant sources of both stimulatory and inhibitory regulators. In particular, there are now clear examples of regulatory interactions which occur in both directions between hemopoietic and lymphoid populations. While in no case a complete analysis of the control systems has been achieved, maintenance of homeostasis in hemopoietic systems is now better understood than for most other cell populations in the body. Further, with existing techniques, and in particular, the exploitation of the full potential of the semi-solid cloning systems, it is realistic to expect that a complete analysis of hemopoietic regulation can now be accomplished.


[Repeated sampling of rat polymorphonuclear leucocytes from the pleural cavity. Application for the study of leucocyte chemotaxis (author's transl)].

A method is described for allowing the sampling of a homogeneous population of polymorphonuclear cells (PMN) from the rat pleural cavity a well as a technique involving the use of a phase-contrast microscope and video-recording system to study the necrotaxis of rat PMN towards a single erythrocyte lysed by ruby-laser irradiation. This allows both the speed of the leucocytes and the degree of their chemotactic response to be determined. The advantages and disadvantages of the experimental technique are discussed along with its application to the study of in vitro and in vivo actions of various drugs on rat PMN chemotaxis.


Proliferation of human lymphocytes in culutre: determination by measurement of nuclear volume and cell number.

Proliferation of stimulated human lymphocytes was studied in cultures by determining cell counts and nuclear volume enlargement. DNA content and the nuclear volume increase before the cell divides. Volume-frequency curves were obtained of unstimulated and phytohemagglutinin (PHA) stimulated lymphocytes on 0, 3, 6 and 9 days of cultures with the Coulter Counter H4 system. As the number of lymphocytes in the cultures transformed and enlarged in volume, the area under the volume-frequency curve increased proportionately. The fraction of transforming cells was determined by comparing the area under the curve of the unstimulated and the stimulated lymphocytes. This method provides both the fraction of cells transformed and the increment of the number of cells in the culture and is therefore a better indicator of cell proliferation than the commonly used isotope-labeled thymidine uptake method which monitors the fraction of the cells in DNA synthesis phase only.


[Study of the electrophoretic and kinetic properties of erythrocyte glucose-6-phosphate dehydrogenase in hereditary enzyme deficiencies. Description of a new variant of glucose-6-phosphate dehydrogenase: the Gd Kremenchug].

A study of the activity of human erythrocyte G-6-PD has been completed on a great number of normal controls, coming from different regions of the USSR. This study has led to the discovery of a new variant of erythrocytic Gd, which was named "Kremenchug" from the origin of the patient. This new Gd, has a very low Km for G-6-P, and has a greater affinity for 2-deoxyglucose-6-phosphate. The maximal activity versus the pH of the medium is also characteristic of this new Gd.


[In vitro study of erythroblast precursors in Vasquez disease (polycythemia vera). Arguments in favor of a double population of erythroblastic stem cells in the bone marrow].

In vitro studies using the plasma clot culture system have been performed in order to compare the red cell pregenitors able to rise to erythrocytic colonies in 7 days (CFUE) in the bone marrow of polycythemia vera (PV), secondary polycythemias and normal subjects. In PV but never in normal individuals or secondary polycythemias, the bone marrow cells producing  erythroid colonies without addition of erythropoietin were found. The erythropoietin dode response curves in PV is biphasic with a plateau up to a concentration of erythropoietin of 0.02-0.05 i.U./ml followed by a near normal response to erythropoietin at higher doses. Thus our results demonstrate that two populations of erythroid stem cells coexist in PV, one being abnormally sensitive to (or independant of) erythropoietin, the other normally responding to erythropoietin. After remission induced by P32 treatment, the abnormal population can disappear but the prognostic significance of this disappearance is uncertain. On the whole these results are in agreement with those of others laboratories using the plasma clot culture system. The reasons of the disagreement with the data published using the methylcellulose technic of culture are discussed.


[Distribution of HLA antigens among the population of Finistère (France) (author's transl)].

We have studied by using HLA system, 200 people, without filiation but having the same geographic origine (Finistère). The results have been compared with those measured for Parisian and Basque population. A significantly decrease of antigen A29, B18 and BW16 frequency have been observed in the population from Finistere. But, antigen B8 and B27 seems to be more frequent than in population from Paris. A linkage desequilibrium is described for A1-B8 and A3-B7 haplotypes as in population from Paris; but the desequilibrium for A11-B5 seens to be characteristic of population from Finistere.


[Materno-foetal transfer of anti-HLA antibodies. Study by using LDA test (author's transl)].

The LDA test was used to study the materno-foetal transfer of anti-HLA antibodies. The results were compared with those obtained earlier by the complement dependent lymphocytotoxicity test (CdL). Although more sensitive than CdL, the LDA test did not demonstrate more maternal antibodies in cord blood when these antibodies corresponded to the child's paternal antigens. Nevertheless LDA demonstrated antibodies did not always behave similarly to those seen by CdL.


Hydergine and vincamine derivative LD 4298 exhibit no anti-sickling properties in vitro.

Amongst the treatments proposed for Sickle Cell disease Ergot derivatives and extracts from Pervinca have been proposed. We have studied in vitro the effect of Hydergine and LD 4298 on Sickle Cells and Sickle Hemoglobin according to the following four parameters: percentage of sickling, potassium leak, minimum gelation concentration and speed of gelation. In these studies no anti-sickling effect was detected. Moreover Hydergine at 50 microgram/ml increased spontaneous potassium loss without any anti-sickling effect. One can conclude that these two drugs have no effect on sickling.


The mechanism of action of tRNA methylases studied with immobilized tRNAs.

Each of the individual tRNAs immobilized on aminohydroxybutyl-cellulose (ABC) through their oxidized 3'-terminal binds affinitively all methylases present in the enzyme extract irrespective of whether this tRNA will be involved in the following step of methylation or not. These data allow to suggest that (a) the formation of a methylase-tRNA complex and the catalytic act of methylation are indeed autonomous processes and (b) the first step of interaction between tRNAs and tRNA methylases is rather unspecific and consists in the recognition of the whole class of tRNA molecules.


The DNA polymerases associated with the adenovirus type 2 replication complex: effect of 2'-3'-dideoxythymidine-5'-triphosphate on viral DNA synthesis.

An adenovirus (Ad) DNA replication complex extracted from infected HeLa nuclei could be purified free of the bulk of intracellular DNA polymerase activity by sedimetation in neutral sucrose gradients. However, the replication complex still retained some alpha and gamma DNA-polymerase activity. Since this complex is inhibited by 2', 3' dideoxythymidine-5'-triphosphate (ddTTP), an inhibitor of DNA polymerase gamma, a functional role for this enzyme in Ad DNA replication is suggested. Similar inhibition by ddTTP in intact Ad infected nuclei and comparable inhibition of Ad DNA synthesis in whole cells by dideoxythymidine (ddThy) are consistent with a role for DNA polymerase gamma. Uninfected HeLa nuclei or whole cells are not similarly inhibited by ddTTP or DDThy respectively. Such data does not rule out an additional functional role for other DNA polymerases, and recent experiments from this laboratory (1) suggest that DNA polymerase alpha is also involved in Ad DNA synthesis.


Raman spectral studies of poly(1-methylinosinic acid).

The synthesis and characterization of poly(1-methylinosinic acid) are described. Laser Raman spectra of poly (mII) were obtained as a function of temperature in D2O solution. Thermal melting profiles derived from the intensity variations of the 712, 795, 814, 986, 1333, 1509, 1550 and 1680 cm-1 bands indicate a cooperative melting temperature of 9 +/- 1 degree C. The low temperature form of poly(mII) exhibits a carbonyl frequency at 1710 cm-I which is decreased to 1680 cm-I upon melting. The Raman hypochromism in the bands reported are equal to or much larger than any reported for other nucleic acids. The data are consistent with the low temperature form of poly(mII) being an ordered single stranded unit with a high degree of basestacking. The melting profiles obtained from the uv and cd spectra are consistent with and support the Raman data. This single stranded RNA exhibits an uncharacteristic behavior in that it melts cooperatively.