Aberrant thymus--MR assessment.

Aberrant thymus--MR assessment. Aberrant thymic tissue, particularly when it is located posteriorly, can present a diagnostic dilemma. Once a mediastinal "lesion" is defined on the plain film, MR is the most efficient and best diagnostic test for defining the nature of the mass and showing its relationship to other mediastinal structures. Two cases of aberrant thymic tissue, one on the right side and one on the left, are presented to elucidate the unique role of MRI.


[Rhodococcus equi infections in AIDS: personal cases]. We report on the occurrence of an uncommon pathogen, Rhodococcus equi (R.e.) in HIV infection. In the period february 1990-July 1991 we performed the diagnosis of R.e. infection (lung, lung-central nervous system) in two infected patients, aged 27 and 31 years, both males, drug addicts, classified as CDC IV. In the first of them an cavitating right bronchopneumonia (bpm) was diagnosed as of tuberculous origin, on the ground of the microscopic demonstration of an acid-fast, gram-positive strain in b.a.l.. A standard antituberculous regimen was uneffective and R.e. was eventually isolated in three blood culture taken shortly before the exitus and at post-mortem examination of the lungs. In the second patient a subclavian right bpm developed without microbiological evidence of any common pathogen. A third-generation cephalosporin course was only partially successful (clinical amelioration, incomplete clearing of radiological findings). After 3 months the patient relapsed and an aseptic meningitis developed. Cultures of blood and csf yielded a R.e. strain; post-mortem examination revealed a diffuse, cavitating bpm. Our personal experience underlines the difficulty in recognizing R.e. infections in compromised host, because of similarity to other entities (i.e. tuberculosis) and the absence of pathognomonic hints.


Neuroimaging in tuberous sclerosis: a clinicoradiological evaluation in pediatric patients. CT, MRI and neurological features of 27 children with tuberous sclerosis were prospectively compared. Imaging studies were positive in 92.5% of cases. CT was more useful in detecting subependymal nodules, while MRI showed the number and location of cerebral cortical and subcortical lesions more accurately. Cortical lesions in the cerebellar hemispheres were present in 26% of patients. Gadolinium-DTPA used in 10 patients showed slight enhancement of the subependymal lesions in 80% of cases, probably representing active lesions with alteration of the blood-brain barrier. None of the cortical and subcortical lesions enhanced. Giant-cell astrocytomas were detected in 5 patients, the postcontrast CT and MRI studies improving their assessment. Unlike subependymal nodules, all tumors showed marked enhancement regardless of their size. Seizures were present in 96% of patients, mostly beginning before 1 year of age. There was no clear relationship between the radiological features and the neurological evolution of these patients. Therefore, it is not possible to establish a clinical prognosis based on the radiological findings. MRI is the procedure of choice in the diagnosis of tuberous sclerosis. When pre and postcontrast MRI are negative, CT is used to exclude small calcified subependymal nodules. MRI follow-up is required only when tumoral development is clinically suspected.


[Glial heterotopy of the nose ("nasal glioma"). Description of a case]. A six months female infant was admitted in our hospital for congenital dysmorphism of face: a subcutaneous nodule in left nose region was present. An x-ray study showed relevant scoliosis of the nasal septum. On surgery a white firm nodule was incompletely excised; a post-operatory CT-scan excluded any communication of neoplasia with brain. No bone lacunae were seen. Clinically there was neither rhinorrhea nor meningitis. The baby was discharged on 7th day. Grossly the mass presented white surface, firm consistency with small hemorrhages on cut surface. Microscopically the nodule, encircled by a fibrous pseudo-capsule, was mostly composed of gemistocytic astrocytes, occasionally binucleated, interspersed within fibrillary neuroglial tissue. Strands of fibrous tissue, in continuity with the pseudo-capsule, separated the glial tissue. No neuronal cells were seen. Necrosis, mitotic figures and vascular proliferations were absent. GFAP immunohistochemical stain confirmed the glial nature of the cells. Our diagnosis was one of "heterotopic glial tissue of nose" (nasal glioma). The absence of connection between the nodule and endocranial contents (CSF-filled spaces, leptomeningeal or dural tissue), excluded the diagnosis of encephalocele. In our case, the tissue was only of embryonic neuroectodermal derivation: on this basis the diagnosis of teratoma, which is classically composed of two or three embryonic layers could be excluded. The pathogenesis of nasal glioma is briefly discussed by authors.


Intravascular papillary endothelial hyperplasia in the hand of a fencer. A case of intravascular papillary endothelial hyperplasia in the hypothenar eminence of a 23-year-old female fencer is described. Differential diagnosis with other similar benign and malignant lesions was made using histopathology and immunohistochemistry. The possible post-traumatic proliferative histogenesis is discussed. The non-malignant nature of the mass was confirmed after a three year follow-up.


Blue nevus of the uterine cervix.

Blue nevus of the uterine cervix. A case of blue naevus of the endocervix is reported with immunohistochemical and ultrastructural findings. The dendritic melanin-containing cells are analogous to the blue naevi cells of the skin. Electron microscopy showed melanosomes in dendritic cells but no cells with melanin-producing Schwann cells features were observed. Histogenetic hypotheses are discussed.


Rare malignant mid-femoral tumours in the first decade of life. Report of three cases with short literature review. The most common femoral shaft tumours in children are eosinophilic granuloma and Ewing's sarcoma. Three children in the first decade of life with rare femoral shaft tumours are reported. There was one osteosarcoma and two chondrosarcomas. Radiographic differential diagnosis of the femoral shaft tumours and microscopic diagnostic difficulties of chondrosarcoma are discussed.


Ultrasonography in Legg-Calvé-Perthes disease. Ultrasonography was found to be a valuable investigation in the assessment and management of Legg-Calvé-Perthes disease (LCPD). It was used to assess 23 patients with LCPD in 25 affected hips and was compared with radiographs obtained at the same time. A chronological five-part staging of LCPD is proposed, expressing the degree of flattening and fragmentation as well as reconstitution of the femoral head as seen on ultrasound examination. Thickening of articular cartilage was documented, and associated findings of synovitis and lateral extrusion of the femoral head were evaluated. An intraarticular hip effusion was present in 74% of cases in stages I-II. Lateral extrusion increased from stage II onwards until the healing stage. The phase of reconstitution (stage IV) demonstrated both resorption of the necrotic bone and formation of new immature osteoid tissue. Lateral extrusion and the start of the healing phase can be shown earlier by ultrasonography than by radiography.


Pancreatic malignant melanoma with occult primary lesion. A case report. A case of unusual location of a M.M. in the head of pancreas is described. Evidence of a primary lesion was not achieved, still we incline to retain this case as a metastatic malignant melanoma with occult primary location.


Scrotal ultrasonography in Henoch-Schönlein purpura. The vasculitis of Henoch-Schönlein purpura may involve the scrotum and clinically mimic diseases requiring surgical intervention, such as testicular torsion or an incarcerated inguinal hernia. The ultrasonographic features include marked edema of the scrotal skin and contents with intact vascular flow in the testicles. These findings may suggest the correct diagnosis and prevent unnecessary surgical exploration.


Congenital bladder rupture and urine ascites secondary to a sacrococcygeal teratoma. A case of congenital bladder rupture with urine ascites and bilateral hydronephrosis secondary to a sacrococcygeal teratoma obstructing the bladder outlet is presented. As no evidence of pulmonary hypoplasia and renal dysplasia was present, with rapid recovery of renal function after decompressive surgery, the infravesical obstruction probably occurred late in the fetal period. The bladder rupture resulting in internal decompression may have constituted an additional protective mechanism for the preservation of renal function.


The size and contractility of the gallbladder in infants. The size of the gallbladder and the common bile duct as well as the contractility of the gallbladder were measured in infants under the age of 1 year. A total of 43 healthy infants were studied at their routine visit to the well-baby clinic at the ages of 6 weeks, 4 months, 8 months or 12 months. Gallbladder size was measured before and after a test meal. Fasting gallbladder size increased with the age of the infants. However, when the size was corrected for the infant's weight, no age-related variation was found. The contraction index (CI) was determined as a percentage decrement of planimetric gallbladder size from the initial size. The mean CI declined with age from 75% (+/- 27%) (mean (+/- SD)) at the age of 6 weeks to 24% (+/- 28%) at the age of 12 months. Overall CI varied from -44% to 100%. An increase in gallbladder size after the test meal was observed in 4 infants. Gallbladder volumes were also calculated, but two-dimensional measurements were regarded as more accurate in infants. A large variation was found in the size as well as in the contractility of the gallbladder in infants less than 1 year of age.


Renal transplant artery stenosis in children: treatment with percutaneous transluminal angioplasty. Hypertension due to transplant renal artery stenosis was treated with percutaneous transluminal angioplasty (PTA) in 6 children. 8 angioplasties were performed and the follow-up period varied between 3 and 66 months (average 23 months). The stenoses were located at the site of anastomosis in 4 children and distal to the anastomosis in 2. PTA resulted in improvement of hypertension in all patients, although the need for antihypertensive medication remained. No grafts were lost. Our findings show that PTA of transplant renal artery stenosis in children is a good first choice of treatment.


Balloon dilatation of cricopharyngeal achalasia. A 6-month-old male infant with difficulty swallowing, recurrent aspiration pneumonia and failure to thrive was diagnosed with cricopharyngeal achalasia. Normal oral feeding and no further aspirations followed a single catheter balloon dilatation.


CT of gyriform calcification in tuberous sclerosis. This paper presents a patient with typical clinical and CT features of tuberous sclerosis associated with gyriform calcification of the most part of the left frontal lobe. This patient represents the third case of tuberous sclerosis in the literature presenting with gyriform calcification.


Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography. Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.


Cystic encephalomalacia and intrauterine herpes simplex virus infection. Cystic encephalomalacia occurred in two preterm infants who had proven intrauterine herpes simplex virus type 2 infection. Calcification was evident in the basal ganglia. Follow-up scans indicated that the cysts had resolved over a period of two months in one infant, while in the case of the other, the cysts became progressively larger with significant ventricular dilatation developing. While cystic changes in the brain of preterm infants are usually due to periventricular leukomalacia, intrauterine infection needs to be considered as a possible cause. Antiviral treatment may be of benefit to infants with herpes simplex virus infection.


MRI in poliomyelitis-like syndrome.

MRI in poliomyelitis-like syndrome. We report the MRI findings in a patient with poliomyelitis-like syndrome. MRI demonstrated a lesion extending from the dorsal region of the pons to the upper level of the thoracic spinal cord. The spinal cord was particularly affected in a region involving the bilateral anterior horns. Although its pathology is unknown, the lesion of the spinal cord seemingly reflects the pathological features of this disease.


Chronic recurrent multifocal osteomyelitis with MR correlation: a case report. Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disorder of unknown etiology. The main characteristics on plain X-rays are a lytic destruction in the metaphyseal region of the long bones followed by progressive sclerosis. The symmetrical pattern and the frequent involvement of the sternoclavicular joints and vertebrae are typical. An association with palmoplantar pustulosis has also been described. Laboratory findings are nonspecific. Although MRI is not necessary to make the diagnosis of chronic osteomyelitis, it is useful in assessing the extent and the evolution of the lesions. CRMO of the tibial and fibular bones is described in a 14-year-old girl, who suffered from pain and soft tissue swelling in both ankles. Initial plain X-rays and laboratory findings were normal. After relapsing clinically, progressive sclerosis of both fibular bones occurred. Lytic lesions in the left tibia and both fibular bones were visible. Scintigraphic examination showed pathologic tracer accumulation in both legs. The diagnosis of CRMO was suggested based on CT and MRI findings. CRMO was confirmed after curettage and bone biopsy. Histopathological findings were consistent with active chronic osteomyelitis.


Relapsing polychondritis in childhood: a rare observation studied by CT and MRI. Relapsing polychondritis is very rare in children. The diagnosis must be based on a combination of clinical and pathologic features. CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures. The role of MR imaging should be complementary to CT.


Hypertrophic osteoarthropathy in a young child with adult respiratory distress syndrome (ARDS) secondary to burns. A case of hypertrophic osteoarthropathy (HOA) is presented in a 14-month-old girl with adult respiratory distress syndrome (ARDS) secondary to severe burn injury. The child developed clubbing during a lengthy and tumultuous hospitalization for her underlying lung disease, and hypertrophic osteoarthropathy was demonstrated radiologically.


Wolman's disease: ultrasonographic and computed tomographic findings. Acid lipase deficiency which is an inborn error of lipid metabolism leads to an abnormal accumulation of cholesteryl esters and triglycerides in many tissues. It is manifested in two clinical forms: Wolman's disease (WD) which is fatal in infancy and cholesteryl ester storage disease (CESD) which is a milder form and usually presented in adulthood. An infant with a clinical diagnosis of WD was examined with CT and ultrasound. Where as CT showed an enlarged liver with decreased density and heavily calcified adrenal glands, ultrasound revealed an enlarged liver with normal echogenicity, adrenal calcification and thickening of bowel loops. Bowel wall thickening in WD was not demonstrated in the literature before with any imaging modality.


Congenital solitary nonparasitic cyst of the liver in a newborn. Congenital solitary nonparasitic cysts of the liver (CSNCL) are extremely rare causes of abdominal masses in newborns and infants. We describe a cystic abdominal mass virtually all extrahepatic in a newborn that proved to be a CSNCL.


Iatrogenic perforation of the lamina cribrosa by nasogastric tube in an infant. A case of iatrogenic perforation of the lamina cribrosa, followed by intracranial placement of a nasogastric tube in a preterm neonate is described. By routine ultrasound examination of the brain an echogenic structure was seen, which was radiographically diagnosed as a nasogastric tube. The tube was manually removed under antibiotic prophylaxis. No complications were observed. The false route disappeared and long-term follow-up showed no neurological side effects.


Pericardial effusion and its relationship to cardiac disease in children with acquired immunodeficiency syndrome. Over 200 children with acquired immunodeficiency syndrome (AIDS) have been followed at our institution. We retrospectively evaluated 45 children from the above group. 26 of the 45 children had a pericardial effusion documented at echocardiography and/or at post-mortem examination. This report describes the association of pericardial effusion, myocarditis, and pericarditis in children with AIDS and the implications for imaging. Half of the children with a pericardial effusion had a normal cardiac silhouette on chest radiography. 18 children with a pericardial effusion, had associated cardiac abnormalities. These abnormalities were ventricular dilatation and/or hypertrophy, myocarditis, or pericarditis. The presence of pericardial effusion also correlated highly with pleural effusion and ascites. The presence of a pleural effusion and a pericardial effusion was almost exclusively seen in the children with cardiac abnormalities. Pericardial effusion and cardiac disease should not only be suspected in any child with radiographic signs of cardiomegaly, but be strongly suspected in any child with pleural effusions or ascites, even with a normal cardiac silhouette, especially if they are not responding to conventional medical therapy and their respiratory condition is not improving.


Two smooth muscle tumors in the airway of an HIV-infected child. Tumors of smooth muscle origin are rare in childhood. We report a case of multiple bronchial leiomyomata in a seven year old girl with clinical HIV infection who presented with new onset of wheezing. Clinical details of this case have been published elsewhere, but without imaging studies [1].


Normal childhood developmental patterns in skull bone marrow by MR imaging. To establish the normal developmental pattern of skull bone marrow in children by MR imaging, sagittal T1-weighted MR skull images of 324 normal children (newborn to 18 years) were reviewed. Bone marrow intensity was assigned four gradations as compared with that of muscle and fat on the same image. Bone marrow became isointense with fat (yellow marrow) at a mean age +/- S.E.M. (in years) of 8.5 +/- 0.24 in sphenoid, 9.1 +/- 0.29 in mandible, 9.3 +/- 0.28 in hard palate, 9.7 +/- 0.26 in frontal, 11.0 +/- 0.26 in squamous occiput, 11.5 +/- 0.28 in parietal, and 11.9 +/- 0.24 in basiocciput. There is a strong correlation between age and marrow intensity by Spearman analysis (p < 0.001): hard palate 0.64, mandible 0.61, parietal 0.42, sphenoid 0.70, cervical spine 0.50, basi-occiput 0.58 and occiput 0.52. Two consistent overall patterns of red-yellow marrow conversion were observed. Bone marrow became isointense with fat prior to pneumatization of the paranasal sinuses. Marrow conversion in the bones of the face occurred before those of the calvarium in a specific pattern. There was no significant sex difference in the pattern or rate of marrow conversion. These normative data are necessary to evaluate the immature skull by MR imaging in disease states.


Dual-energy X-ray absorptiometry in the lumbar spine, proximal femur and distal radius in children. Dual-energy X-ray absorptiometry was used to measure bone mineral density (BMD) in the lumbar spine, proximal femur and distal radius in 48 Japanese children aged 3-18 years. In the normal children (n = 32), BMD increased with age in all locations, with a nearly twofold increase from preschool age to adolescence. Most of the children with chronic diseases known to affect bone metabolism (e.g., steroid osteoporosis) (n = 16) had low BMD in every region, indicating that these disease states probably affect multiple sites of the skeleton in children.


Coexistence of bronchial atresia and bronchogenic cyst: diagnostic criteria and embryologic considerations. We report a case in a neonate of concurrent bronchial atresia and bronchogenic cyst. An accurate, noninvasive, preoperative diagnosis of this unusual combination of anomalies was made by ultrafast computed tomography (UFCT). This case supports the hypothesis that bronchial atresia results from an event occurring in the 5th-6th week of embryological development, rather than after the 16th week as previously believed.


Contrast enemas after necrotising enterocolitis: a case for prophylaxis? During a 4-year period 9 out of 35 patients deteriorated following a contrast enema after necrotising enterocolitis (NEC). Two developed Klebsiella septicaemia with one subsequent death. Following the latter two cases the paediatric surgeons instituted intravenous prophylactic antibiotics (benzyl penicillin, metronidazole, gentamicin) prior to contrast enemas post-NEC. Of the factors examined only the presence a long line in-situ or history of previous perforation demonstrated any increased risk with regard to clinical deterioration post-examination.


The antegrade colonogram: extending the small bowel follow through for children suspected of having colonic disease. Fifty pediatric patients suspected of having colonic disease were evaluated by antegrade barium study ("antegrade colonogram") and by colonoscopy with multiple biopsy specimens. Radiographic patterns of mucosal irregularity, nodularity, or ulceration, haustral asymmetry or edema, and narrowing of the colonic lumen were easily recognized. Correlation between colonographic patterns, biopsy findings, and gross appearance at colonoscopy showed the colonogram to have an overall accuracy of 77-88%. The antegrade colonogram is easily performed and often provides information regarding areas of the colon not seen by the endoscopist. In some cases it may also be useful in the evaluation of response to therapy for inflammatory bowel disease.


Sonographic diagnosis of portal venous gas in two pediatric liver transplant patients with benign pneumatosis intestinalis. Case reports and literature review. Pneumatosis Intestinalis (gas in the bowel wall) is uncommon in children and is associated with a number of clinical conditions most notably necrotizing enterocolitis in sick neonates. We observed portal venous gas on sonography and benign pneumatosis intestinalis on abdominal radiography in two children who had recently undergone liver transplantation. Asymptomatic portal venous gas and pneumatosis intestinalis has not been described previously in pediatric liver transplant patients. It is important to recognize the sonographic appearance of portal venous gas since sonography is the imaging modality most frequently utilized for monitoring the allograft post liver transplantation.


Gas gangrene: an unusual cause of graft failure in an orthotopic pediatric liver transplant. Gas gangrene of the liver is a very unusual cause of graft failure, and gas gangrene caused by Enterobacter cloacae is also exceedingly rare. Although Clostridial infection could not be excluded in this case, the literature and our case show that Enterobacter cloacae can be present in gas gangrene tissue whether or not it is the only infecting organism.


Is cystic mesenchymal hamartoma of the liver similar to infantile hemangioendothelioma and cavernous hemangioma on dynamic computed tomography? Dynamic incremental contrast CT of a cystic mesenchymal hamartoma of the liver in a 10 year old boy demonstrated peripheral and septal enhancement with partial centripetal fill-in during the next sixty minutes, not unlike that which occurs in hemangioendothelioma and hemangioma. Histologic similarities among these mesenchymal lesions may explain the similar CT and angiographic characteristics of such malformations.


Spinal muscular atrophy: MR evaluation.

Spinal muscular atrophy: MR evaluation. The neurogenic myopathy of spinal muscular atrophy (SMA) is degeneration of anterior horn cells of the spinal cord and associated muscle weakness. In three patients with the severe type, according to Dubowitz's classification, magnetic resonance imaging (MRI) of the lower extremity showed severe atrophy of the entire muscle bundles of the thigh and the calf. Nine intermediate type patients had ragged atrophy of muscle bundles of the thigh and the calf with selective preservation of adductor longus muscle. Five patients with the mild type had fatty infiltration of muscle bundles and increased intermuscular fat planes. MRI was insufficient for the evaluation of cervical cord abnormalities. MRI of the lower extremity was a reliable complementary modality for the diagnosis and follow-up of SMA patients.


MRI of fibromatosis: with pathologic correlation. Fibromatosis refers to group of benign but sometimes locally aggressive proliferative lesions of myofibroblasts. These are characterized by infiltrative growth, and hence may mimic a malignant lesion. These lesions reveal a low signal intensity on T1-weighted pulse sequences, but may show low or high signal intensity on the T2-weighted sequences. Histologic correlation reveals that the lesion showing low signal intensity on T2-weighted sequences has a larger collagenous component, and reduced cellularity compared with the lesion showing high signal intensity on T2-weighted sequences.


The conus medullaris: time of ascendence to normal level. This study was intended to determine the level of the conus medullaris in normal babies. We examined 114 healthy infants using high resolution ultrasound which identified the spinal cord and the tip of the conus medullaris. This method provided a good analysis of the level of the conus medullaris so that we could assess the rate of ascent to L1/2. The range of the conus level for all children was at TH12/L1 interspace to L4.78% of babies aged between the 30th and 39th postmenstrual week had the tip of the conus medullaris between L2 and L4.84% of babies aged between the 40th and 63rd postmenstrual week had their conus level between TH12/L1 and L1/2 interspace. In one girl aged 53 weeks the tip was found at L4. Ultrasound is a reliable method to observe the development of the conus level in young infants and to identify a tethered cord.


Genochondromatosis II.

Genochondromatosis II. We report a new disorder which we have called genochondromatosis II. The disorder is similar in mode of inheritance and long bone changes to that named genochondromatosis but shows some distinctive features, namely involvement of short tubular bones and normal clavicles. The disorder has a benign clinical course and may be discovered incidentally. Accurate diagnosis is important for proper genetic counselling.


Chronic granulomatous disease with renal stones. A case of chronic granulomatous disease with hydronephrosis and renal calculi is presented. This is to our knowledge the first such case to be reported. The calculi were successfully ablated by extracorporeal shockwave lithotripsy.


An unusual thoracic duplication arising below the diaphragm. Gastrointestinal tract duplications are relatively uncommon in the paediatric age group. This is a report of an infant who had a tubular duplication of the jejunum which extended into the thoracic cavity via the aortic hiatus of the diaphragm.


Invasive pulmonary aspergillosis with pneumopericardium and pneumothorax. A case of an invasive pulmonary aspergillosis with pneumopericardium and pneumothorax in an immunocompromised child of 5 years is reported. He was treated with thoracic drainage, amphotericin B and itraconazole. The course was satisfactory.


Bronchiectasis in children with lymphocytic interstitial pneumonia and acquired immune deficiency syndrome. Plain film and CT observations. In a review of 77 HIV positive children seen between 1981 and 1990, 32 were diagnosed as having lymphocytic interstitial pneumonitis). Four of the LIP group developed bronchiectasis, a finding not previously reported. The precise factors leading to the bronchiectasis are unclear. All patients had chronically consolidated lung with volume loss. A history of recurrent bacterial superinfection was not noted in any of the cases. With more cases of HIV positive children living longer, bronchiectasis, long known to occur in primary immunologic disorders, will probably be more frequently noted.


The iron hand from Slovenia.

The iron hand from Slovenia. The iron hand prosthesis now in the custody of the Rehabilitation Institute Ljubljana, was excavated some 80 years ago in the ruins of the Vransko castle. The hand, its form and function are described. It was manufactured somewhere in Europe between the years 1500-1650. The owner, probably one of the local nobles, has remained unknown.


A standardised trans-tibial amputation method following chronic occlusive arterial disease. The histo- and biochemical parameters of leg muscles from patients with chronic occlusive arterial disease were examined. The outcome of these tests indicated that it is not possible visually to determine the amputation level accurately at the time of surgery. These test results therefore encouraged the authors to develop a standardised surgical procedure for trans-tibial amputations. With this standardised technique specific musculature is resected to assure that no pathological tissues remain. This surgical prophylaxis is meant to prevent the development of gas gangrene and thus to obtain primary healing. Eighty-six trans-tibial amputations were performed and in 93% of these the knee joint was preserved.


Level of lower limb amputation in relation to etiology: an epidemiological study. The Danish Amputation Register and the nationwide National Patient Register are presented. Based upon the code numbers in the WHO classification system (ICD), 4 etiology groups i.e. vascular insufficiency, diabetes mellitus, malignant neoplasma and trauma were extracted. The purpose was to analyse the relationship between level of amputation (i.e. foot, below-knee, through-knee, above-knee and hip) and etiology (cause of amputation). The material represents all such amputations in Denmark during the period 1978 to 1989 (n = 25.767). The number of amputations because of vascular insufficiency with and without diabetes mellitus decreased over the period studied. The number of tumour and trauma amputations seemed unchanged. There was a significant reduction in the number of amputations at proximal levels (above-knee) for vascular insufficiency with and without diabetes mellitus and in the trauma group. No such change was found regarding tumour amputations. There was a characteristic pattern in the distribution of level respectively of etiological factors for each etiology group and for each level of amputation.


Function after through-knee compared with below-knee and above-knee amputation. Fifty-nine amputees, 24 below-knee (BK), 17 through-knee (TK) and 18 above-knee (AK) who had prosthetic replacements, were evaluated using a questionnaire which provided a quantitative and qualitative assessment scale for the prosthetic function. The ability to apply or don the prosthesis was noted in 100% of the BK, 70% of the TK and 56% of the AK amputations (p < 0.001). Daily use of the prosthesis was recorded in 96% of the BK, 76% of the TK and 50% of the AK amputations (p < 0.001). A higher level of amputation resulted in a significantly lower degree of rehabilitation (p < 0.05). The qualitative evaluation shows that the higher the level of amputation, the lower the usefulness of the prosthesis. Four percent of the BK, 12% of the TK and 39% of the AK amputees had no use whatsoever of their prosthesis (p < 0.01). From a functional standpoint, TK amputation should always be considered as the primary alternative to AK amputation when a BK amputation is not feasible.


Functional evaluation by gait analysis of various ankle-foot assemblies used by below-knee amputees. Twelve different prosthetic feet were tested by 10 male subjects with right below-knee amputations. Level walking with each prosthetic foot was investigated using a pair of force plates. Five parameters were selected to compare the functional characteristics of the feet: 1) step length, 2) walking velocity on the sound side in relation to the prosthetic side, 3) depth of valley in the pattern of the vertical component of the floor reaction force, 4) efficiency of the deceleration and acceleration by the prosthetic foot, and 5) irregular patterns in the wave form of the fore and aft components of the floor reaction force. Each of the above parameters was rated numerically. The total score of the objective evaluation attained by analysing the five parameters showed some coincidence to the results of subjective evaluation. However, a good correlation existed between the objective negative score and the subjective negative rating (p < 0.05). Non-axial feet developed recently, such as the SAFE II and Seattle Light feet achieved higher scores in the older age group, while single-axis feet, such as the LAPOC and Otto Bock feet achieved higher scores in the younger age group (p < 0.05).


A CAD CAM method for custom below-knee sockets. The purpose of this investigation was to develop a numerical method for fabricating prosthetic sockets for below-knee amputees. An optical/laser digitiser scans an amputee's stump and collects three dimensional numerical data describing the surface of the limb and describing specific modification site locations. The numerical data from the laser camera representing the stump and modification sites are altered by the prosthetist using a custom computer aided design software system running on a personal computer. Using the altered numerical data a programme is created for a high resolution numerically controlled milling machine and a mould is made. The prosthetist then fabricates a socket. While the system has been tested with below-knee amputees it has been designed for application in most areas of prosthetics and orthotics. Utilising this method 15 patients were fitted. All patients subjectively stated that their "computer designed" socket fitted better than their conventionally made socket. As the research progressed and experience was gained with the system patients were normally fitted with the first socket iteration. The system overcomes five limitations existing with some of the other numerical systems: 1) accurate high resolution surface topography, 2) specific identification of subject modification sites, 3) flexible, user friendly software, 4) high resolution numerically controlled milling, and 5) integrated expansion to other prosthetic and orthotic areas.


CAD CAM trans-tibial temporary prosthesis: analysis and comparison with an established technique. The purpose of this study was to evaluate the application of CAD CAM in the production of temporary trans-tibial prostheses. The CAD CAM system was assessed based on the number of socket attempts, number of prosthetic appointments, and temporary prosthesis rehabilitation time. These parameters were considered to be related to the quality of socket fit and were influenced by the entire interdisciplinary team including the patient. A concurrent prospective comparison between the CAD CAM system and an established fiberglass/pelite liner technique was also performed. Patients (n = 30), were fitted with either a conventional or a CAD CAM socket. Records were kept before and after discharge until the interdisciplinary team considered the patient ready for definitive prosthesis casting. After approximately 90 postoperative days, patients were deemed fit to proceed from their initial plaster cast prostheses to their temporary prostheses. The group fitted with conventional sockets had an in-patient rehabilitation phase of 10.5 +/- 15.0 days and required 2.9 +/- 1.1 prosthetic appointments. In-patients fitted with CAD CAM sockets required 5.1 +/- 1.8 appointments and were hospitalised for 23.6 +/- 15.0 days. The significantly increased rehabilitation duration and number of appointments (p = 0.01), were generally due to incorrect socket volume and/or inadequately modified relief/loading areas. In this study 67% of the patients fitted with CAD CAM sockets required at least one additional attempt. The clinical evaluation and modification of the temporary prostheses, including the decision to remake a particular socket, were carried out by the same prosthetist who cast the patients.(ABSTRACT TRUNCATED AT 250 WORDS)


Psychological factors leading to amputations in adults. Psychological factors may lead to a small number of amputations in adults. They may be classified as being due to: 1. chronic pain syndrome; 2. artefactualists; 3. self-mutilation; 4. attempted murder. An understanding of these potential factors will make the amputee clinic team aware of this problem, and help them deal with the rehabilitation of the patient.


Weight distribution of below-knee amputee and able-bodied children during standing. The purpose of this investigation was to compare weight distributions of a relatively large number of below-knee (BK) amputee and able-bodied children during two different standing positions. Twenty-one BK amputees and 200 able-bodied children volunteered as subjects for this investigation. Each child stood on a pressure plate and three sets of trial data were collected. One set of trial data was collected with both feet together on the pressure plate and two were collected with feet placed 20cm apart. The total force applied by each foot to the pressure plate was normalised by dividing by subject weight to yield foot force to body weight ratios. Data were separated into forefoot and rearfoot areas, force for the forefoot area was then calculated and normalised by dividing by total foot force to yield forefoot to whole-foot force ratios. Ratios for the two foot placement conditions and for non-prosthetic, prosthetic, dominant, and non-dominant feet were compared using paired t-tests (p < 0.05). Results indicated that: 1) BK amputee children placed more weight on their non-prosthetic limb than their prosthetic limb, yet this was not different from able-bodied children in respect of weight distribution between dominant and non-dominant limbs; 2) approximately 90% of the load on the prosthetic foot was placed on the forefoot; and 3) the load on the non-prosthetic foot was evenly distributed between the forefoot and rearfoot like that of able-bodied children. It was concluded that except for substantially more weight on the forefoot of the prosthetic leg BK amputee children stood in the same way as able-bodied children.


[Follow-up evaluation of metastatic neuroblastoma using high dose [131I-meta]iodobenzylguanidine]. Between 1989 and 1990 nine children with neuroblastoma stage IV (according to Evans) have been treated with high-dose [131I-meta]Iodobenzylguanidine (HD-mIBG). The total HD-mIBG dose administered to each child was at mean 699.3 +/- 111 MBq/kg body weight. Prior to (median 28 days) and after (median 50 days) HD-mIBG treatment a diagnostic scan with [123I-meta]Iodobenzylguanidine ([123I-m]IBG) was performed. Scans performed with HD-mIBG were superior to diagnostic scans for the detection of bone lesions in 8/9 children, for the detection of soft tissue lesions in 4/9 children, and for a more precise diagnosis of the primary tumor in 1 child. In 4 children lesions which were primarily identified in the therapeutic scan could be further observed in posttherapeutical examinations.


[The potential significance of early effort myocardial scintigraphy following PTCA]. Exercise 201Tl scintigraphy before and after successful PTCA was evaluated in 69 patients with coronary stenosis > 60%. Mean luminal stenosis was found reduced from 74 +/- 10% to 31 +/- 11% (58% reduction). Scintitomographic examination within 3 weeks following intervention showed a reduction of the exercise perfusion defect from 17.00 +/- 14.6 to 5.8 +/- 7.8 arbitrary units (66% reduction). Angiographically, re-stenosis was identified in 22/41 patients (54%) after 6 months. Unexpectedly, re-stenosis could not be predicted by early 201Tl scintigraphy (positive and negative predictive value in patients with single-vessel disease: 61% and 41%, resp.). Nevertheless, evaluation of PTCA results was possible (sensitivity 83%). However, postinterventional transient defects, indicating ischemia, were often seen (all patients: 43/69; patients with single-vessel disease: 31/55) and not indicative of failure of the intervention. Thus, post-PTCA scintigrams should be compared with scintigrams recorded before the intervention to ensure reliable follow-up control.


[Reinjection of 201TL in postinfarct patients so as to improve the demarcation between cicatrix and ischemia]. In patients with earlier myocardial infarction the value of additional imaging after reinjection of 201Tl for better detection of ischemic myocardium was assessed. In 40 patients 40 MBq 201Tl were reinjected 24 h after stress and additional SPECT was performed 1 h later. Quantification of defect sizes showed a further reduction of 4 h redistribution defects by more than 30% in 18 patients. Comparison with clinical, electrocardiographic and scintigraphic parameters showed that none of these was sufficiently accurate for predicting additional thallium uptake after reinjection. In 22 patients with coronary angiography all 11 patients with collateralized occlusions of a coronary artery showed a reduction of the 4 h redistribution defect by > 20%, whereas all patients (5/5) with occlusions without distal collateralization had changes < 20%. This demonstrates that an improved thallium uptake after reinjection is typical of ischemic but vital myocardium.


[Combined bilateral idiopathic necrosis of the humerus and femur heads: bone scintigraphy, x-ray, CT and MRT findings]. Aseptic bone necrosis in adults is usually caused by circulation defects or metabolic diseases but may also occur without any obvious reason. In these "idiopathic" cases, alcoholism seems to play a prominent role. In addition to the frequently affected femoral heads other bones may be involved. Here a remarkable pattern of localizations is reported: the bilateral idiopathic necroses of the heads of both humerus and femur. Untreated aseptic bone necroses close to a joint commonly leads to severe secondary arthrosis and destruction of the joint within a short time. Therefore, only a diagnosis in an early stage of the disease offers the chance of a successful joint-preserving therapy. In cases of clinically suspected aseptic bone necrosis but still negative or doubtful X-ray findings, bone scans or MRI are reliable methods of verifying the diagnosis.


[The clinical course of oxyphilic carcinoma of the thyroid]. In a total of 1665 patients with malignant thyroid neoplasms 90 oxyphilic thyroid carcinomas (OTC) were found of whom 55 could be re-examined and newly classified. Morphological and clinical parameters influencing the clinical course were determined. During a mean follow-up period of 6.5 y metastases or local recurrent disease occurred in 12 patients (24%). Apart from 3 early manifestations of metastases, 9 patients developed recurrent disease within, on average, 4.7 y after thyroidectomy: local lymph node metastases and local recurrences occurred within an average of 5.4 y, distant metastases after only 2.7 y. Thyroglobulin proved to be reliable for follow-up with a sensitivity of 88% on levothyroxine and 75% on endogenous TSH-stimulation (specificity: 98%). The frequency of metastases and local recurrences correlated with age at the time of tumor diagnosis, the degree of invasiveness and the local tumor extension (pT4 vs. pT1-3), whereas other factors such as the absolute diameter of the tumor or patient's sex had no influence on the clinical course. The survival probability for 5 and 10 years was 95 and 75%, respectively. All OTC patients should be examined regularly at least once a year by cervical sonography and thyroglobulin measurement. Because 18% recurrences occurred within 4.7 y such examinations should be repeated beyond year 5 after thyroidectomy.


Survey of 1725 bone scans in patients with malignant disease with particular emphasis on carcinoma of the breast. 1725 bone scans, done for evaluation of metastatic involvement from malignant neoplasms, were reviewed. The fraction of positive scans (multiple lesions in the bone scan) was 34% of the patients with different malignancies. In 251 patients (12%) a single bone lesion was detected and 114 of these patients were followed-up and re-scanned once or twice within 1 to 4 years. 39 of the 93 patients with solitary bone lesion who on follow-up developed multiple lesions had carcinoma of the breast as the primary.


[The effect of Ca-diethylenetriamine pentaacetate on the bio-behavior of tumor-affine metal complexes]. The complexon CaDTPA was injected into tumour-bearing mice in concentrations of 0.05, 0.1, 0.3, and 0.6 mole/l (pH:6) 30 min after the 169Yb-injection. 100 microliters of a 0.3 M CaDTPA solution were injected at different time points (simultaneously, 2, 5, 10, 20, 30, 40 and 50 min, 1, 1.25, 1.5, 2.5 and 10 h) after 169Yb-citrate injection. The animals were killed 24 h after radionuclide administration. A strong radioactivity decrease was observable 24 h p.i. not only in blood, liver, spleen, muscle and bone but also in the tumour if CaDTPA was administered within the first 2 h after ytterbium injection. Thereafter no change in radioactivity could be achieved by DTPA. A time phase in which the Yb could be eliminated from the tissues by means of DTPA (time intervals < 2 h) was distinguishable from a time phase in which it was not attainable for DTPA (time intervals > 5 h). This indicates that the incorporation of Yb into the cells is completed after 5 h and that the metals are intracellularly bound, probably to the lysosomes. Improvements of the scintigraphic tumour detection cannot be expected from the use of complexons.


[A clinical evaluation of MAb BW 835/6 in breast and ovarian cancer]. Studies with the 99mTc-labeled murine monoclonal antibody BW 835/6 in patients suspected of breast (n = 7) or ovarian cancer (n = 8) showed insufficient depiction of primary tumors and metastases of breast cancer, but promising results in the detection of primary tumors in ovarian cancer without metastases. The small number of patients does not allow statistical analysis.


Comparative pharmacokinetics of zidovudine in healthy volunteers and in patients with AIDS with and without hepatic disease. To understand whether disease caused by the human immunodeficiency virus (HIV) affects zidovudine disposition, we compared the drug's pharmacokinetics in six healthy volunteers; six persons with the acquired immunodeficiency syndrome (AIDS) and no evidence of gastrointestinal (nausea, vomiting, diarrhea), renal (elevated blood urea nitrogen, serum creatinine), or hepatic (elevated liver function tests) disease; and three patients with AIDS and hepatic disease. After a single oral dose of zidovudine, serial blood samples were analyzed for drug concentration by radioimmunoassay. A one-compartment oral absorption model was fit to the concentration-time data. The absorption rate constant (4.05 vs 2.11 hr-1) and time to maximum concentration (0.61 vs 1.03 hr) were significantly different in healthy volunteers versus patients with AIDS without hepatic disease. Differences in half-life, oral clearance, and area under the curve were not statistically significant. In the three patients with AIDS plus hepatic disease, clearance was reduced an average of 63%, and area under the curve was increased by a factor of 2.3. These comparative pharmacokinetic data do not support profound differences between zidovudine's disposition in healthy volunteers and individuals with AIDS; however, the differences and trends that were observed may represent an effect of HIV disease. Although the presence of hepatic disease clearly indicates a need to modify individual dosages, these pharmacokinetic data may have more generalized implications for zidovudine dosing as the relationships between drug concentration and therapeutic or toxic effects are clarified.


Potential warfarin-ciprofloxacin interaction in patients receiving long-term anticoagulation. This study prospectively evaluated the potential interaction between the oral anticoagulant warfarin and the quinolone antimicrobial agent ciprofloxacin. After a 10-day placebo lead-in phase, 16 patients stabilized with long-term warfarin therapy were randomized to receive ciprofloxacin 500 mg or a matching placebo twice/day for 10 days. International normalized ratios (INRs) measured by both standard laboratory analysis and by Coumatrak (finger-stick) methods were evaluated at 3- to 5-day intervals. No patient experienced a significant increase in INR. No patient experienced a bleeding event. These data support the fact that a warfarin-ciprofloxacin interaction does not routinely occur at this dosage and duration of ciprofloxacin therapy.


Evaluation and comparison of the adverse effects of streptokinase and alteplase. The frequency and severity of adverse effects resulting from the administration of streptokinase and alteplase were determined in 126 consecutive patients who received standard dosages of these agents for the treatment of acute myocardial infarction. Evaluation was based on patient assessment by nursing staff, physicians, and the investigators before, during, and after thrombolytic administration. Overall, adverse effects occurred in 15 (41.7%) of 36 patients receiving streptokinase and 12 (13.3%) of 90 receiving alteplase (p = 0.001). No major bleeding or neurologic events were documented. Minor bleeding occurred in 13.9% and 7.8% of streptokinase and alteplase recipients, respectively (p = 0.47), and hypotension in 8 (22.2%) and 5 (5.6%), respectively (p = 0.01). The frequency of hypotension associated with streptokinase was significantly higher than that with alteplase. Thrombolytic-induced hypotension was easily managed and was not associated with sequelae.


A retrospective review of the use of lipid-lowering agents in combination, specifically, gemfibrozil and lovastatin. We conducted a retrospective review examining lipid profiles, creatine phosphokinase (CK) levels, and alanine aminotransferase levels (ALT) in patients receiving the combination of gemfibrozil and lovastatin. Serum lipid levels were significantly improved with therapy over those before therapy. Of the 70 patients receiving the combination, 5 experienced mild elevations in CK, 1 a mild elevation in ALT, and 1 mild elevations in both. No patient reported muscle weakness or muscle pain. The combination of these two medications appeared to be at least additive, highly effective, and well tolerated. The mean total cholesterol, triglyceride, and low-density lipoprotein cholesterol levels decreased from 278, 306, and 180 mg% to 200, 151, and 129 mg%, respectively, and the mean high-density lipoprotein cholesterol levels increased from 34 to 40 mg%. This retrospective data analysis suggests that the combination of gemfibrozil and lovastatin may be safe in patients with normal renal function when the dosage of lovastatin is limited and when CK and ALT levels are monitored carefully.


Comparison of frequencies of suicidal tendencies among patients receiving fluoxetine, lofepramine, mianserin, or trazodone. To evaluate whether fluoxetine causes an important increased risk of suicidal behavior, we compared the frequency of attempted suicide, suicidal ideation, and aggressive behavior in persons who received fluoxetine, lofepramine, mianserin, and trazodone, based on information available on general practitioners' computers provided by Value Added Medical Products, Ltd. The frequency of these events was higher in fluoxetine users in the year prior to first treatment than in users of the other three antidepressants. The frequency of these events in the 90 days after the study drug was started was similar for the users of all four drugs. These data indicate that fluoxetine does not directly cause suicidal behavior at a substantially higher frequency than do lofepramine, mianserin, and trazodone.


Granulocyte-macrophage colony-stimulating factor and zidovudine in the treatment of neutropenia and human immunodeficiency virus infection. Granulocyte-macrophage colony-stimulating factor (GMCSF) is a hematopoietic protein that has been studied both in vitro and in vivo in human immunodeficiency virus (HIV) infection. Since both HIV infection primarily and zidovudine (formerly AZT) treatment secondarily may result in neutropenia, administration of GMCSF to persons with HIV infection is generating considerable interest. Despite in vitro studies demonstrating that the agent may stimulate HIV replication, in the presence of zidovudine a synergistic inhibition of replication occurs. Early clinical studies in patients with the acquired immunodeficiency syndrome indicate that GMCSF can raise neutrophil counts with or without concurrent zidovudine treatment. The long-term safety and tolerance of the combination has to be established.


Review of drug-induced acute interstitial nephritis. Drug-induced acute interstitial nephritis (AIN) is a rare, potentially correctable cause of acute renal failure. Early recognition and appropriate therapy are essential to its management. Several drugs have been associated with the development of AIN, including antibiotics and nonsteroidal antiinflammatory, agents, all with characteristic mechanisms of toxicity.


Survey of ACCP members regarding use of computers and information processing. A questionnaire was sent to a random sample of 480 members of the American College of Clinical Pharmacy to determine their opinions on various issues relating to computer technology and the future role of computers in information processing in pharmacy. Results from the 335 evaluable responses revealed nearly universal use of computers. Word processing was the most common application and IBM or compatible computers were the dominant machines. Respondents used a wide variety of generalized and specialized programs, especially electronic communication products. Computer technology is expected to have a major impact on routine aspects of pharmacy practice, although, respondents were split on its impact on more cognitively intensive functions.


Statistical interpretation of body surface potential maps; aspects of adequate group representation and parameter selection. Nonparametric approaches for studying adequate group representation accuracy vs. sample size relationship and two discriminative parameter searching methods of statistical body surface map analysis are discussed. A clinical application of the selection methods supports that body surface potential mapping provides significant additional diagnostic information with respect to the conventional 12 lead ECG.


[Heart potential mapping from the thoracic surface in healthy persons]. We have examined 56 normal subjects, 24 females and 32 males. We have calculated the average maps on the basis of 30 patients of the average age of 24.3 years (SD = 4.91). The aim of our studies was to settle the normal heart potentials mapping. The studies were realized in the Laboratory of Electrophysiology in the Department of Pathophysiology of Wrocław Medical Academy. In order to record the heart potential mapping we have used the HPM 7100 system (Fukuda Denshi). We have analyzed isopotential maps, isointegral maps and ventricular activation time maps. We have calculated the average maps using the information system "Heart-Map" Normal heart potential mapping are necessary for statistic analyzing of heart potentials mapping in patients suffering from the heart diseases.


[Multi-electrode mapping from thoracic surface in patients with Wolff-Parkinson-White syndrome]. Body surface potentials mapping is a new method of heart electric phenomena visualisation. Attempts of accessory conduction pathways localisation in 9 patients with overt and in 1 with concealed WPW syndrome are presented. In 4 pts the preexcitation with QRS complex > 120 ms was clearly seen, in next 4 individuals that symptom was less evident and in one case the retrograde conduction through the accessory pathway was hardly noticed. The isopotential maps allowed precise localisation of the accessory pathways ventricular insertion in all patients with QRS > 120 ms and in one with QRS < 120 ms. After successful dissection of accessory pathways, isopotential maps became normal. In patients with slight signs of preexcitation, noninvasive (transesophageal) or invasive atrial stimulation could improve the visibility of accessory pathways localisation.


[Multipolar image of the heart in patients after myocardial infarction]. This paper regards 136 cases of cardiac ischaemia including 93 post-infarction cases and 43 with post-effort angina pectoris. The patients with history of infarction were divided according to its electrocardiographic++ location. The groups of subjects were submitted to multipolar electrocardiographic recording from chest surface by means of a multi-electrode network (diamentoid) introduced to vectorcardiography by the Kowarzyk method. The QRS complex divided into 8 equal sectors served as the basis for calculating, among other things of the non-ipolarity index (km) and its graphical representation. The ++non-dipolarity index tends to be especially high in patients with recurrent and large infarction, it was increased in angina pectoris.


[Analysis of the foci of ectopic stimuli based on body surface mapping of cardiac potentials during sinus rhythm]. This paper submits results of cardiac potentials mapping recorded from the body surface in a 87-electrodes Fukuda-Denshi system in 10 patients exhibiting documented ventricular cardiac rhythm disturbances. Isopotential and isointegral maps of depolarisation periods of the ventricles were analysed for sinus cycle and additional ventricular activations. The results were then compared to those of a 15-persons reference group of people not displaying any cardiac rhythm disturbances. Occurrence was pointed out of aberration at different cardiac activation time, manifested as additional potential extremes during QRS as well ventricular repolarization. The additional extremes during ventricular repolarization are of a persistent potential character, probably resulting in disturbed activation.


[Localization of infarction of the anterior and inferior myocardial wall by body surface mapping]. 11 patients after anterior myocardial infarction and 7 patients inferior myocardial infarction were subjected to potentials mapping from 87 body surface electrode system. The reference group was made up by 15 healthy individuals. The analysis referred to isopotential and isointegral maps during the 20 ms and 40 ms of the QRS onset and for the entire QRS. It was stated that the occurrence of abnormal potential minimum might be the essential diagnostic criterion. In the case of anterior infarction, the abnormal negative potentials is located in the vicinity of the sternum, whereas in inferior infarction in right and lower part of the chest. The diagnostics of inferior infarction requires additional criteria, ventricular activation time maps especially.


[Histocompatibility antigens (HLA) in children with lipoid nephrosis]. Actual knowledge on the HLA relationship with the primary glomerulopathies, with particular reference to steroid - sensitive nephrosis of childhood, is surveyed. Occurrence of HLA B-8 and B-35 in this nephropathy has been investigated. The studies involved 47 patients aged between 3 and 15 years and 117 healthy children from Lower Silesian region. It has been showed, that HLA B-8 is present more frequently in sick children, than in healthy controls. The situation is reverse in case of HLA B-35 antigen. However, the difference is statistically insignificant. A probability of the lipid nephrosis sensitivity to corticosteroids can not be predicted on the base of the presence of these HLA antigens.


[Clinical significance of urinary excretion of beta 2-microglobulin in patients with chronic glomerulonephritis]. Assuming, that deterioration of renal function in the primary glomerulonephritis may also result from the destructive lesions to parenchymal tissue of the renal cortex (with simultaneous lesions to renal tubuli), the assessment of the urinary excretion of beta 2-microglobulin could be approached as an indicator of renal function in this situation. The study involved 85 patients with chronic glomerulonephritis treated with immunosuppressors++. In these subjects beta 2-microglobulin concentration per one unit of the urine (UB2M) together with fractional excretion of this globulin (FEB2M) was studied. Statistically significant differences in mean UB2M and FEB2M values have been noted between patients with remission and with the lack of the remission following therapy. Gradual increase in UB2M values as well as FEB2M values have been noted with deterioration of excretory renal function. The results have shown, that the assays of UB2M, and particularly FEB2M, is a sensitive marker of proximal renal tubuli function and indirectly of the processes which take place in extraglomerular renal cortex structures. It might be there fore of prognostic value.


[Evaluation of selected indicators of the renal proximal tubule function in patients treated with gentamicin]. The study aimed at evaluating proximal renal tubule function in patients with nephrolithiasis and chronic pyelonephritis, and in patients with infectious diseases treated with gentamicin. The study involved 2 groups of patients: group A--17 patients with nephrolithiasis and chronic pyelonephritis and group B--30 patients with other infectious diseases (pneumonia, biliary tract infections) but with normal glomerular filtration rate. Patients from both groups were treated with gentamicin in a daily dose of 2-3 mg/kg for 7-10 days. Serum and urine creatinine levels were assayed in all patients prior to, 2-3, 7, 10 days, and after the treatment. Patients assigned to group B were divided into two subgroups: B1 included 15 patients with normal beta 2-microglobulinuria, and B2 15 patients with increased renal loss of beta 2-microglobulin and decreased tubular reabsorption of this protein. Significant increase in beta 2-microglobulinuria was seen on the third day of therapy, the decrease in the tubular reabsorption and glomerular filtration rate were noted in all patients on the seventh day of gentamicin administration. Beta 2-microglobulinuria was significantly higher in patients from groups A and B2 in comparison with group B1 in which no dysfunction of the proximal renal tubule was present before gentamicin therapy. A degree of beta 2-microglobulinuria is an early and sensitive indicator of gentamicin nephrotoxicity. The risk of nephrotixic symptoms is particularly obvious in patients with deteriorated function of renal proximal tubuli before the treatment with gentamicin.


[Zinc level in the blood of children with nephrotic syndrome and correction of its deficiency with animal blood preparation Livex]. Serum and erythrocyte zinc levels have been assayed in in 45 children with steroid-dependent nephrotic syndrome in both acute phase and remission. Out of these children a group of 22 has been distinguished. These patients have been treated with Livex--animal blood preparation containing amino acids and trace elements, including zinc. It was found, that serum zinc has been significantly lowered in children with the acute phase of nephrotic syndrome who were not treated with Livex. Erythrocyte zinc levels have been normal in these patients. A significant increase in both serum and erythrocyte zinc levels has been noted during remission, but they have still been lower that in healthy children. A three-month cure with Livex produced statistically significant increase in zinc levels in children during remission.


[Urinary tract infection caused by Chlamydia trachomatis in children]. In 1988-1989, 131 children aged between 1 and 18 years were examined for urinary infections with C. trachomatis at the Department of Pediatric Urology in Wrocław. Chlamydia trachomatis was diagnosed with the aid of McCoy's cellular cultures and immunoenzymatic test Chlamydiazyme in 25% of the examined children. A relationship between Chlamydia trachomatis and particular components of the clinical picture has been analysed. Effectiveness of the treatment with erythromycin (Davercin) and doxycycline (Vibramycin) has been also assessed.


Pediatric HIV-1 infection: a clinical overview. Several questions remain unanswered including the timing of perinatal transmission, maternal factors predisposing to perinatal transmission of HIV-1, the best methods for early diagnosis in the neonate, and means of preventing perinatal HIV-1 infection. Significant advances have been made in the early diagnosis of HIV-1 infection, and now it is possible to make a diagnosis in most infants by 6 months of age. Unfortunately, not all these techniques are commercially available, so this capability is limited to certain institutions and laboratories. The natural history of HIV-1 infection in children continues to evolve, particularly with increased prophylaxis of P. carinii pneumonia and the availability of antiretroviral therapy. Our challenges for the future are to prevent perinatal transmission, to develop new and better therapies for opportunistic infections and HIV-associated complications, and to improve outcome and prognosis.


Cutaneous manifestations of pediatric HIV infection. Children who are infected with human immunodeficiency virus may develop a wide variety of mucocutaneous manifestations, such as skin infections, tumors, and inflammatory skin disorders. The most significant infectious diseases are candidiasis, dermatophytosis, herpes simplex, herpes zoster, and pyoderma. Inflammatory disorders include seborrheic dermatitis, vasculitis, and pyoderma gangrenosum. Kaposi sarcoma is extremely rare in children with the acquired immunodeficiency syndrome.


[Arteriovenous hemofiltration as an adjuvant therapy in peritoneal dialysis and hemodialysis in a patient with terminal renal failure]. In the uraemic patient regularly treated with peritoneal dialyses occurring peritonitis caused a decrease of ultrafiltration and transfer abilities of the peritoneum. Other symptoms dangerous for life also appeared: uraemic pericarditis and significant overhydration. Peritoneal dialyses lost its effectiveness. Therefore they were supplemented by arterio-venous haemofiltration. Haemofiltration was also conducted at the beginning of haemodialysis treatment, which was initially unregular. Application of haemofiltration enabled the patient to survive during the time of waiting for regular haemodialyses. It may be useful to consider such a treatment, when the adequacy of proper renal substitutive management of uraemia by other methods is impossible to obtain.


Sexually transmitted diseases in children. Sexually transmitted diseases (STDs) in children may be transmitted by sexual abuse, by accidental contact, or perinatally. Although only 2% to 10% of abused children become infected, childhood syphilis, gonorrhea, condylomata acuminata, and Chlamydia trachomatis must always be considered. We reviewed data from our hospital regarding the frequency, prevalence, routes of transmission, and clinical features of these infections. Ninety-five percent of acquired syphilis in children is transmitted by sexual abuse. The perpetrator is usually someone the child knows or trusts. Of our 21 patients under 14 years of age with acquired syphilis, most were 4 to 8 years old. Girls were infected twice as often as boys. Sexual contact was confirmed in 71.4%. A chancre sore was infrequent in children; condylomata lata was the most frequent cutaneous lesion (80.9%). In the last 10 years, 102 cases of congenital syphilis were diagnosed in our hospital. The main clinical findings were bone involvement (78.7%), hepatosplenomegaly (68.8%), cutaneous lesions (50.8%), and jaundice (15.1%). Gonorrhea was detected in only nine children. Vulvovaginitis was the most common clinical manifestation. Sexual transmission was documented in three patients. Accidental contact with their infected mother occurred in two sisters. Three newborns acquired the disease during delivery. The STDs in children are a worrisome problem. Evaluation for sexual abuse should be done in all cases. Prevention and treatment of adults are the main steps to prevent these infections in children.


Epidermal nevi and the epidermal nevus syndromes: a review of 233 cases. It is likely that most if not all epidermal nevi can be explained on the basis of genetic mosaicism, with each of the many types of nevi representing the cutaneous manifestation of a different mosaic phenotype. Hence there are potentially many different epidermal nevus syndromes.


Investigation of the photosensitive child. Photosensitivity disorders in childhood frequently can be diagnosed and managed in the general dermatology clinic. Occasionally, when diagnostic doubt exists, referral to a specialist unit is required for diagnostic phototesting. Light testing equipment is fickle by nature, making such units uncommon. Phototesting using monochromator or provocation systems takes approximately 45 minutes. Immediate and delayed readings over the following 48 hours as appropriate are required to cover the diagnostic possibilities. Individual diseases are characterized by particular patterns of wavelength dependency and evolution of the abnormal response. Other investigations that may be required are autoantibodies to exclude lupus erythematosus, a porphyrin scan leading to full studies and, on occasion, cell mutation or survival, and chromosome studies for the rarer genophotodermatoses. Good investigative data frequently help clarify the common clinical variants.


Epidermolysis bullosa: to split and to clump. During the past few years various proteins have come to the forefront as underlying the structural abnormalities of different forms of epidermolysis bullosa. Only very recently has linkage been established between certain major forms of epidermolysis bullosa and the genes encoding some of these proteins, and mutations of these genes identified. The stage will soon be set for providing a new and rational basis for a better understanding of the pathogenesis of epidermolysis bullosa, for improving diagnosis, including prenatal testing, and for devising new approaches to treatment.


Ichthyosis: mechanisms of disease.

Ichthyosis: mechanisms of disease. The disorders of cornification (ichthyoses) comprise acquired and inherited disorders characterized clinically by generalized scaling and histologically by hyperkeratosis. They may arise through defects in the production or maintenance of a normal cornified cell compartment, or both. The stratum corneum is composed of protein-enriched and lipid-depleted corneocytes ("bricks") surrounded by an intercellular domain ("mortar") composed of hydrophobic, lipid-enriched membrane bilayers, and containing desmosomes and a limited array of hydrolytic enzymes. Mechanisms whereby a genetic defect involving either the bricks or the mortar may result in abnormal stratum corneum retention are discussed using ichthyosis vulgaris and recessive X-linked ichthyosis as examples. In addition, epidermal hyperproliferation, which floods the cornified cell compartment with incompletely formed units, results in hyperkeratosis. To date, no primary disorders of epidermal hyperproliferation have been defined. Recent work, however, demonstrates that stratum corneum barrier function regulates epidermal DNA synthesis. For example, in essential fatty acid deficiency, barrier dysfunction is responsible at least in part ror the epidermal hyperproliferation. Defective barrier function due to defective lamellar body secretion may also underlie the phenotypic changes after birth in harlequin ichthyosis; that is, from the massive, constrictive hyperkeratosis of the newborn to an exfoliative erythroderma in survivors. The mechanisms whereby specific defects in cornification result in generalized scaling disease are only beginning to be defined. Yet, even at this early stage, the view of the stratum corneum as a tightly organized structure whose function is highly regulated is emerging. Hence, the disorders of cornification should provide important insights into stratum corneum structure and function.


Food hypersensitivity and dietary management in atopic dermatitis. Although immune dysfunction is known to play an integral role in the development of atopic dermatitis, no clear delineation of the underlying pathogenic mechanism(s) responsible for this disorder has been proposed. Several factors are known to trigger flares of atopic dermatitis. In the extrinsic form of this disorder, food and airborne allergens may provoke flares of eczema. Research implicating food hypersensitivity in the pathogenesis of atopic dermatitis includes studies of food allergen avoidance in newborn infants at high risk for atopic disease, investigations of children with blinded food challenges, and therapeutic trials of allergen-elimination diets. Taken together, these studies demonstrate a significant pathogenic role for food hypersensitivity in about one-third of children with atopic dermatitis.


Atopic dermatitis: studies of skin permeability and effectiveness of topical PUVA treatment. Ultraviolet light is effective treatment for patients with atopic dermatitis that is resistant to conservative therapy, or complicated by adverse effects of extended steroid use. We designed a protocol using topical psoralen chemotherapy with ultraviolet A (PUVA) to treat atopic dermatitis in 114 patients. Clinical results were excellent, with complete clearing in 50% of patients receiving daily treatment. Histologic and immunologic values correlated with the clinical response, including reduced epidermal thickness, and decreased numbers of epidermal Langerhans cells and dermal mast and mononuclear cell infiltrates. The pattern of keratin 14-positive keratinocytes returned toward normal. In addition, the water-holding capacity of the stratum corneum increased to near normal levels. We also studied stratum corneum permeability in lesional and nonlesional skin using the dimethyl sulfoxide whealing test and theophylline absorption studies. Compared with controls, permeability was markedly increased in lesional skin and mildly increased in nonlesional skin in patients with atopic dermatitis. These results suggest that immune abnormalities and barrier dysfunction participate in the pathogenesis of atopic dermatitis.


Atopic dermatitis: recent therapeutic advances. New treatments were recently proposed for the management of severe atopic dermatitis (AD). They all act on some component of the immune reaction. Oral cyclosporine reduces the number of CD4+ cells, the secretion of interleukins, and the function of Langerhans cells. Although the action of oral cyclosporine at moderately high dosages is regular and rapid, the risk of serious side effects and the reappearance of progressive disease after stopping treatment limits the use of this drug in AD. Thymic hormone extracts in patients with severe AD affect the deficit of cellular immunity. gamma-Interferon inhibits IgE synthesis induced by interleukin-4, increases expression of Fc gamma receptors, and increases superoxide production by circulating monocytes. More recently, two other treatments were published: complexes of allergen and specific antibodies to Dermatophagoides pteronyssinus, and interleukin-2. All of these modalities have only a transitory effect, but they can help to modify a flare of severe AD.


Pigment cell refugia in homeotherms--the unique evolutionary position of the iris. Homeotherms are generally considered to lack classical active dermal pigment cells (chromatophores) in their integument, attributable to the development of an outer covering coat of hair or feathers. However, bright colored dermal pigment cells, comparable to chromatophores of lower vertebrates, are found in the irides of many birds. We propose that, because of its exposed location, the iris is an area in which color from pigment cells has sustained a selective advantage and appears to have evolved independently of the general integument. In birds, the iris appears to have retained the potential for the complete expression of all dermal chromatophore types. Differences in cell morphology and the presence of unusual pigments in birds are suggested to be the result of evolutionary changes that followed the divergence of birds from reptiles. By comparison, mammals appear to have lost the potential for producing iridophores, xanthophores, or erythrophores comparable to those of lower vertebrates, even though some species possess brightly colored irides. It is proposed that at least one species of mammal (the domestic cat) has recruited a novel iridial reflecting pigment organelle originally developed in the choroidal tapetum lucidum. The potential presence of classical chromatophores in mammals remains open, as few species with bright irides have been examined.


The effect of oxygen on melanin precursors released from retinal pigment epithelial cells in vitro. The autoxidation of dopa to melanin in culture media causes toxicity to retinal pigment epithelial (RPE) cells and endothelial cells. The damage is specific to cell type and to the ambient oxygen concentration. To determine whether RPE cells influence the oxidation of dopa to media, we compared light absorbing dopa derivatives in the media exposed to cells with those found in the media incubated without cells. Dopa was extensively oxidized in the presence of RPE cells, and more light absorbing substances were generated with higher dopa and oxygen concentrations. However, an increase in ambient oxygen concentration decreased the quantity of several dopa derivatives which had been formed. The data provided evidence that RPE modulated dopa metabolism. Quinolic derivatives produced from a tyrosinase reaction and dopa-melanin formation moved the peak absorbance wavelength of dopa into the visible range. The spectrum between the dopa-derived compounds in the media has an absorbance at 240-275 nm and a maximum around 300 nm with a shoulder near 375 nm. Gaussian analysis (peak separation) resolved these spectra into five components: a sharp band at 248 nm, a band at 295 nm, a large band at 359 nm, and two broad bands at 459 and 585 nm.


Proteinuria of B700, a 67 kD albumin-like melanoma-specific antigen. B700 is a murine melanoma antigen that is closely related to, but distinct from, serum albumin. The present study examined the metabolic fate and anatomic distribution of radioiodinated B700 and mouse serum albumin (MSA) administered s.c. to mice. In blood, both proteins were associated with the plasma fraction where the halflife of B700, a glycoprotein, was 0.5 days, compared to 2.7 days for MSA. Of particular interest was the observation that B700, a 67 kD anionic protein, was excreted primarily in urine. The selective B700-proteinuria did not alter urinary volumes or produce hematuria or edema. SDS-polyacrylamide gel electrophoresis and western blot analysis using the H-2-3-3 B700-specific monoclonal antibody revealed that B700 proteinuria occurred in B-16 murine melanoma bearing animals but not in control mice. These studies demonstrate that the tumor-bearing host readily distinguishes between very similar normal protein (MSA) and tumor-associated antigen (B700) molecules and processes them differently.


The effect of sodium iodate and melanin on the formation of glyoxylate. Sodium iodate damages retinal pigment epithelium specifically, but the reason for this specificity is not well understood. The work reported here describes an effect of sodium iodate on melanin, a major component of the retinal pigment epithelium. Sodium iodate increases the ability of melanin to convert glycine to glyoxylate. Almost ten times as much glyoxylate is formed when sodium iodate is present compared to the amount formed with melanin alone, although iodate alone does not convert glycine to glyoxylate. A chemical reaction between sodium iodate and melanin is suggested as a partial explanation of the specificity of iodate toxicity towards retinal pigment epithelium.


L-dopa inhibits in vitro phosphorylation of melanoma glycoproteins. L-DOPA had no effect on the endogenous phosphorylation of proteins after extraction with 1% Triton X-100 from hamster melanoma. When proteins were purified further by wheat germ-agglutinin chromatography, however, a dramatic and dose-dependent inhibitory effect of DOPA on glycoprotein phosphorylation was observed in the presence of Mn+2.


Effect of amphotericin B on dopachrome tautomerase activity and other melanogenic parameters in cultured B16/F10 melanoma cells. The antifungal reagent Fungizone (amphotericin B and deoxycholate) caused an activation in dopachrome tautomerase and dopa oxidase activities of B16/F10 melanoma cells at the routine concentration (2.5 micrograms/ml) used for preventing molds and yeast growth in cultures of animal cells. However, higher amphotericin B concentrations caused a significant cell death and the inhibition of enzymatic activities. At the optimal concentration of Fungizone, the enzymatic activities and melanin content were augmented as incubation time increased. The detergent sodium deoxycholate alone exerted no effect on these melanogenic parameters, eliminating the possibility that this detergent was partially responsible for melanogenic modifications produced by Fungizone. After withdrawal of Fungizone from the reaction medium, the recovery of melanogenic parameters to normal values was slower for DCT than for tyrosinase. The behavior of dopa oxidase was very similar to that reported by Johnson and Bagnara (Pigment Cell Res. 3, 173-175) for tyrosine hydroxylase.


Penile lentiginosis. An ultrastructural study. This study on five patients has revealed more extensive alterations to melanocytes than previously reported, and emphasizes the fact that depigmentation is an essential element of the condition. In hyperpigmented areas, melanocytes were increased in number along the basal layer of the epithelium, were hyperactive, and in some cases contained bizarre melanosomes. In two cases there was suggestion of a defect in melanosome transfer to keratinocytes. Lymphocytes were closely apposed to melanocytes, and, in hypopigmented areas, were clearly involved in their disintegration. In depigmented areas, there was complete absence of melanocytes and of melanosomes in keratinocytes, and lymphocytes were present in the basal layer. In general, the appearances did not resemble melanoma in situ with spontaneous regression, although a second biopsy of one patient after one year did reveal features of melanocytes suggestive of an early stage of this condition. The study has provided no clear information as to the initial cause of the condition, but the manner of destruction of melanocytes suggests an immune reaction. Neither has it been of assistance in suggesting a more precise name for it.


[Surgical treatment of inguinal hernia in short-term hospitalization. Prospective survey of 500 consecutive unselected cases. Associations Francaises de Recherche en Chirurgie]. Individual medical and social factors associated with the acceptance and success of short stay surgery for unilateral, uncomplicated inguinal hernia were looked for in 500 consecutive unselected patients. The mean duration of postoperative hospital stay was 3.4 days. Sixty percent of the patients were discharged within the first 48 hours following surgery, including 10 percent who left the hospital in the evening of the operation day. Twenty-two percent of the patients who could have left during these 48 hours refused to do so. The most predictive variable for acceptance and success was a profession requiring little physical activity. Local anaesthesia has been associated with success in the so-called ambulatory surgery, but the methodology of the present study did not permit to establish a cause-effect relationship.


[Unsatisfactory results of intradiscal injection of triamcinolone hexacetonide in the treatment of sciatica caused by intervertebral disk herniation]. Sciatica caused by intervertebral disc herniation can be treated with intradiscal injection of chymopapain. A search for a cheaper and less allergizing product led to triamcinolone hexacetonide, this procedure being known as "nucleorthesis". The first results at 6 months were encouraging. In 3 centres where triamcinolone hexacetonide was tested with a more than 2 years' follow-up 92 patients could be evaluated. The results obtained were considered satisfactory in 34 patients (36.9 percent), but they were poor in 19 patients (20.6 percent), and 39 patients (42 percent) had to be operated upon within 2 years. Return to surgery took place within the 6 months following nucleorthesis in 18 patients (19.56 percent) and beyond this period in 17 patients (22.8 percent) with degradation of the results. Moreover, calcifications were found in 19 out of 38 patients; they were of varying size, sometimes detected only at computerized tomography, and some of them appeared to produce symptoms. All considered, the failure rates, the number of patients who required surgery and the occurrence of large and sometimes symptomatic calcifications make triamcinolone nucleorthesis unacceptable compared with the recognized percentages of success with papain nucleolysis and surgical operations. For these reasons, we consider that this treatment should be abandoned.


[Parasitemia in Plasmodium falciparum malarial attacks in children]. In order to evaluate the predictive value of parasitaemia, this parameter was measured on admission in 69 Gabonese children aged from 3 to 13 years hospitalized for Plasmodium falciparum malarial attacks. Fourteen of these children had cerebral malaria, 5 had isolated convulsions and 50 had uncomplicated attacks. The parasitaemia values measured were compared with those found in asymptomatic children of the same age range carrying trophozoites. There were no significant differences in mean parasitaemia count between the 3 types of malarial attack, and only the asymptomatic carriers had significantly lower counts. However, the wide scattering of individual values within each group indicated that simple measurement of parasitaemia is not discriminative enough to predict the course of malarial attacks in children living in endemic regions. In particular, malarial attacks with very low or very high parasitaemia value are possible, but similar values are found in asymptomatic carriers.


[Role of antiphospholipid antibodies in fetal loss]. A review of the literature and our own experience have shown that the three types of antibodies to phospholipids (APL) usually looked for (i.e. dissociated treponema serology, antiprothrombinase and anticardiolipin) increase the risk of foetal loss, irrespective of whether the mothers have or do not have systemic lupus erythematosus. The prevalence of APL may exceed 40 percent in some series of women who suffered at least 3 foetal losses. Conversely, the incidence of foetal loss (often late in pregnancy) in women with APL has been estimated at 25 to 75 percent, depending on the studies. The presence of lupus seems to increase the risk. There is no consensus on the best factor predictive of foetal loss (antiprothrombinase or one of the anticardiolipin isotypes). Foetal loss seems to be caused by thrombosis of the placenta, the origin of which remains controverted. The therapeutic escalade consists of abstention (in the first pregnancy), aspirin (about 100 mg/day), aspirin-corticosteroid combination or subcutaneous heparin, high-dose intravenous immunoglobulins and plasmapheresis. With these various methods, the birth of a normal child can be expected in almost every case.


Canine pancreatic kallikrein: enzyme isolation and characterization. Two forms of canine pancreatic kallikrein, designated as canine pancreatic kallikrein A and B, were separately isolated by ion-exchange, affinity and hydrophobic chromatographies. These enzymes had similar apparent molecular masses, substrate specificities and pH optima. However, kallikrein B was inhibited by soybean trypsin inhibitor, while kallikrein A was not. Both kallikrein A and B were shown by sodium dodecyl sulfate-polyacryl amide gel electrophoresis to consist of two polypeptide chains, designated alpha and beta chains, and binding by disulfide bond(s). The N-terminal amino acid sequences of each alpha and beta chains of kallikrein A and B were determined.


Amino acid sequences of ferredoxins from Alocasia macrorrhiza Schott in Papua New Guinea. The amino acid sequences of ferredoxin isoproteins (Fd A and Fd B) from Alocasia macrorrhiza Schott in Papua New Guinea were determined. They consisted of single polypeptide chains of 97 and 98 residues, respectively, and both Fds had a molecular mass of 10,800 Da. There was an 88% identity between the sequences of the isoproteins (Fd A and Fd B). These sequences were compared with those of the closely related plant Fds and their phylogenetic relationships are discussed.


Complete amino acid sequence of ovine miniplasminogen. The complete amino acid sequence of ovine miniplasminogen (M(r) 37,662, 343 residues) was determined with the aid of fragments obtained by cleavage with 2-(2-nitrophenylsulfenyl)-3-methyl-3'-bromoindolenine and clostripain. The fragments were aligned with overlapping sequences and sequence comparison with miniplasminogens of other species. Sequence comparison with other species (human, bovine, porcine, equine and canine) gave an overall identity of 63% and a similarity of 83%. The dendrogram of the alignment indicates that ovine miniplasminogen has the closest relationship with the bovine (87% identity) and the most distant with the equine (77% identity) species. The close relationship is indicative for the presence of the same structural and functional domains as in the other species. Sequence comparison of different miniplasminogens showed that positions 49 (Arg), 83 (Arg) and 161 (Ser) in the light chain of the plasmin molecule may play a role in the interaction between plasminogen and streptokinase.


The amino acid sequences of two 13-kDa alpha-amylase inhibitors from the seeds of Sorghum bicolor (L.) Moench. Two inhibitors (SI alpha 4 and SI alpha 5) of the alpha-amylases from insect and mammalian sources were purified from seeds of Sorghum bicolor by saline extraction, precipitation with ammonium sulphate, affinity chromatography on Red Sepharose, and preparative and analytical reverse-phase HPLC on columns of Vydac C18. The complete primary structures of these two inhibitors were determined by automated degradation of the intact, reduced and S-alkylated proteins and by manual 4-N,N-dimethylaminoazobenzene-4-isothiocyanate/phenyl isothiocyanate microsequencing of peptides derived from them following enzyme digests. The amino acid sequences were as follows: SI alpha 4: TVDVTACAPGLAIPAPPLPTCRTFARPRTCGLGGPYGPVDPSPVLKQ- RCCRELAAVPSRCRCAALGFMMDGVDAPLQDFRGCTREMQRIYAVSRLTRAAECNLPTIPGGGCHLSNS PR; and SI alpha 5: ANWCEPGLVIPLNPLPSCRTYMVRRACGVSIGPVVPLPVLKERCCSELEKLV- PYCRCGALRTALDSMMTGYEMRPTCSWGGLLTFAPTIVCYRECNLRTLHGRPFCYALGAEGTTT. Comparisons of these sequences with one another and with those of other proteins in the US National Biomedical Research Foundation Databank indicated that the two Sorghum proteins had significant similarities (21%-42% identity) with the members of the cereal superfamily of enzyme inhibitors.


Hydrophobic zippers and hook-and-eye: evolutionarily conserved protein sequence motifs in eukaryotic acidic ribosomal proteins which are assumed to be involved in the association of the protein family. The acidic ribosomal protein family of eukaryotic cells is thought to form a complex on ribosomes mainly by hydrophobic forces. To investigate the structural basis of how they associate with one another, the primary sequences of the related proteins accumulated from various organisms were analyzed searching for evolutionarily conserved hydrophobic motifs. Initially it is shown that all the P1-type 13-kDa proteins contain a bilateral hydrophobic zipper on a putative alpha-helix, which consists of two periodic arrays of hydrophobic amino acid residues arranged on the opposite sides of an alpha-helix. The P2-type 13-kDa proteins, except for those from the yeast Saccharomyces cerevisiae, are shown to contain two kinds of hydrophobic areas on putative alpha-helices, which can sterically bind to each other in a hook-and-eye fashion. On the other hand, the 38-kDa proteins contain a hydrophobic zipper and a hydrophobic hook in different helical regions. Thus, it is proposed that the 13-kDa proteins associate with the 38-kDa proteins via the hydrophobic zipper or hydrophobic hook-and-eye, and associate with one another with these hydrophobic elements.


Improved detection of homology in distantly related proteins: similarity of adducin with actin-binding proteins. A novel and generally applicable method is described for the detection of homology in distantly related proteins using a new domain sequence database that contains over 20,000 protein sequence segments of known function. The use of the method is illustrated on distantly related domains shared by complement components C1S and C1R, calcium-dependent serine proteinase and bone morphogenetic protein 1. New homologies are shown between human adducin and the actin-binding domains of alfa-actinin and dystrophin.


The superfamily of UvrA-related ATPases includes three more subunits of putative ATP-dependent nucleases. It is demonstrated that the amino acid sequences of the products of E. coli genes sbcC and prrC, and bacteriophage P2 gene old encompass the four conserved motifs typical of the superfamily of UvrA-related ATPases. A more pronounced statistically significant similarity was revealed between SbcC protein, bacteriophage T4 endonuclease component gp46 and bacteriophage T5 protein D13. It is suggested that the newly identified members of the superfamily might all be ATPase components of the respective nucleases, and that the reactions catalyzed by these enzymes are probably ATP dependent.


Structural similarities in the repeat sequences of plasma apolipoproteins, A-I, A-IV, and E. The presence of 22-residue repeats, each with a preferential potential to form an amphipathic alpha-helix, is a unique feature of the plasma apolipoproteins. There are 27 such repeats in the three human apolipoproteins A-I, A-IV, and E. The extent of similarities and differences among these repeats have been estimated by computing correlation coefficients, Dayhoff scores, secondary structure difference profiles, and discrete Fourier transforms. The results reveal that there is a high level of similarity among the repeats of apo A-IV, and a low level of similarity in the repeats of apo E. Within each protein, similarity among some specified repeat pairs is distinctively higher than the others. A high order of similarity is also found among certain segments of each protein with those in the other two. The repeats prefer a mostly alpha-helical structure that is amphipathic in nature. Among the repeats of the three proteins, those of apo E show a high level of divergence among themselves. A consensus alignment of the residues of the 27 repeats into a hydrophobic versus hydrophilic pattern brings to focus the possible specific structure-stabilizing factors, such as the leucine zipper and the salt bridge. The recently reported crystal structures of the human apolipoprotein E and locust apolipophorin-III support many of the predictions made in this study.


A review of cytokine structures.

A review of cytokine structures. The expanding family of cytokines, interleukins and colony-stimulatory factors has made it difficult to readily access their structural and biological properties for comparative purposes. Here their aligned amino acid sequences, biological actions and some structural predictions are presented together for ready comparisons


Effect of vitamin C and vitamin E on prostaglandin synthesis by fibroblasts and squamous carcinoma cells. Dietary levels of vitamins C and E have been associated with cancer prevention and to a lesser extent with therapeutic enhancement of cancer treatment. Inhibition of prostaglandins (PGs) by pharmacological agents has been demonstrated to enhance immunocompetence, and to suppress growth of tumors in animals and humans. We report here on the effect of vitamins C and E on PGE2 production by human gingival fibroblasts and SCC-25 oral squamous carcinoma cells. The results indicate: 1. vitamins C and E exert a dose-dependent effect on arachidonic acid (AA) release and PGE2 synthesis; 2. vitamin E has a biphasic effect which is stimulatory at 1 and 10 microM and inhibitory at 100 microM; 3. vitamin E is considerably more potent than vitamin C in its inhibitory effect on AA and PGE2 in both cell types; 4. a combination of the two vitamins has a consistent dose-dependent inhibitory effect on AA and PGE2; 5. vitamin C stimulates PGE2 synthesis from exogenous AA in fibroblasts, and inhibits it in SCC-25 cells. The in vivo significance of these findings requires further investigation.