Biomedical subjects
Publications and source records attributed to H Skopnik.
The case of a small girl is reported who after a head injury showed radiologically two fractures of the skull whereas the bone scintigram done eight days later with 99mTc-DPD was normal. The possible reasons for this discrepancy are discussed.
BACKGROUND: Glucose tolerance is often impaired in Cystic Fibrosis. The impact of this condition on enteral nutritional support in case of malnourishment remains to be studied. METHOD: In this study the glucose-, C-peptide- and insulin-responses to an OGTT and a standard enteral formula load were investigated in 19 non-diabetic CF-patients. Each test was performed with a carbohydrate load of 1.75 g/kg bodyweight. RESULTS: 11 patients had a normal OGTT (Group I) and 8 had an impaired glucose tolerance (Group II). After the formula load the glucose concentrations were significantly lower than after the OGTT at 30, 60, and 90 min in both groups and at 120 min in group II only. In group II the glucose values exceeded 6.7 mmol/l at 120 min after both OGTT and formula load and were significantly higher than in group I. The food glycaemic index, a parameter for comparison of glucose response to different diets was calculated. It ranged from 30 to 80% in both groups. An increased C-peptide- and insulin-secretion was found in patients with impaired glucose tolerance. CONCLUSIONS: The condition of impaired glucose tolerance must be considered for enteral hyperalimentation of Cystic Fibrosis-patients. The selection of formulas for nutritional support should be based on the calculation of the Food Glycemic Index because of its high interindividual variability.
The pharmacokinetics of theophylline and its metabolites, caffeine, 1,3-dimethyluric acid, 1-methyluric acid and 3-methylxanthine were studied in a 5-day old 1.3 kg premature neonate who accidentally received 180 mg theophylline in 26 h during treatment for bradycardia. Tachycardia, hyperventilation, increased diuresis, central nervous system excitation, an increase in blood glucose concentrations followed by a prolonged decrease and hypercalcaemia were the predominant clinical and laboratory manifestations. The patient responded to supportive care and survived without sequelae. The concentration time course of theophylline and its metabolites in plasma and the pattern of urinary and gastric elimination were determined over 95 h. Theophylline showed, in contrast to its metabolites, a log linear decline in plasma. Elimination of theophylline and caffeine, calculated from their urinary excretion rates, were both exponential during the entire observation period. Urine flow dependence of renal clearance was obvious for theophylline and caffeine. Implications of theophylline disposition in neonates are discussed with special regard to theophylline poisoning.
The chest X-ray film of a girl with cystic fibrosis (CF) showed slowly increasing mottled densities during the 6th and 7th year of her life. Pulmonary symptoms and distress proceeded fast in spite of intensive treatment with antibiotics, corticosteroids, and physiotherapy. Three different fungal organisms were repeatedly cultured from the sputum: Candida albicans, Aspergillus fumigatus, and Exophiala dermatitidis. Antibodies against C. albicans were in the normal range. Candida antigen in blood and antibodies against A. fumigatus were absent. Antibodies against E. dermatitidis were detected by a recently developed indirect immunofluorescence assay. It seems most probable that E. dermatitidis was the causal agent for fungal pneumonia in this case. Under therapy with amphotericin B and flucytosine the clinical course and radiological appearance improved but definitive eradication of E. dermatitidis was only achieved after treatment with itraconazole. The isolation of this fungus from the sputum of a CF patient is reported for the first time. The significance of fungal infections in CF is discussed.
Twenty full term neonates with suspected bacterial infection were randomly assigned to a once daily or a twice daily dosage regimen with gentamicin (4 mg/kg/day). Concomitantly all patients were treated with ampicillin (200 mg/kg/day). The gentamicin concentration time curves were analysed by an open two compartment model under steady state conditions on day 4 of treatment. The mean theoretical maximum serum concentration in the group taking gentamicin once daily (10.9 micrograms/ml) was significantly higher than in the group taking it twice daily (7.4 micrograms/ml). Potentially toxic serum concentrations were never reached. Mean trough concentrations were comparable in both groups (once daily 0.8 micrograms/ml; twice daily 1.0 micrograms/ml). Urinary alanine aminopeptidase excretion increased during and even two days after end of treatment in both groups without any significant differences. The results of the dynamic in vitro model revealed that both dosage schedules showed comparable bactericidal effects on pathogens inhibited by low concentrations of gentamicin like Escherichia coli and Staphylococcus aureus. However the once daily regimen was significantly superior in isolates with high minimal inhibitory concentrations.
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AIM OF STUDY: Shwachman-Kulczycki- and Chrispin-Norman-Scores are widely used scoring systems for CF-patients. Maximum bicycle exercise testing was performed in 15 patients (medium age 13.4 years) to investigate whether clinical and radiographic scores or pulmonary function testing could predict cardiorespiratory fitness. METHODS: A progressive exercise test was used to determine maximum working capacity (Wmax). Prior to exercise testing, lung function and blood gases were investigated. Chest radiographs were scored by an independent radiologist (G.B.) applying the Chrispin-Norman-Score. The Shwachman-Kulczycki-Score was determined by two observers (F.F., H.S.). RESULTS: Chrispin-Norman-Score, Schwachman-Kulczycki-Score, results of lung function testing and blood gas values were significantly correlated to each other. However no significant correlation was found to the degree of exercise limitation. CONCLUSION: Clinical, radiographic scores and lung function testing cannot predict exercise tolerance. Exercise testing is mandatory to evaluate cardio-respiratory fitness in CF-patients.
Pyloric biopsies obtained at pyloromyotomy from 46 infants were studied by light and electron microscopy and compared to 8 autopsy control cases without any evidence of infantile hypertrophic pyloric stenosis (IHPS). A positive family history of this disorder was recorded in 8 cases (2 girls and 6 boys). The most frequent changes in the myenteric plexus comprised axonal alterations. In glial cells, cytoplasmic vacuolisation or an increase of intermediate filaments occurred. In ganglion cells, vacuolisation of perikaryal cytoplasm or dense bodies were observed. No obvious differences were seen between sporadic and hereditary cases. In addition to these fine structural alterations, immunohistochemistry in 6 IHPS cases and 4 controls revealed differences in the distribution of substance P, bombesin, calcitonin gene related peptide and enkephalin-like immunoreactivity within the myenteric plexus between IHPS and control cases. The immunoreactivity, however, was unevenly distributed from case to case and even within individual cases. The reduction of immunoreactivity corresponded, at least in part, to an increase of neurofilaments or abnormal organelles within axons. Increased immunoreactivity was apparently related to focal accumulation of dense cored vesicles noted in the preceding study. It is suggested that these and other changes reported interfere with the normal gastrointestinal reflex mechanisms leading to intestinal obstruction.
The occurrence of increased serum concentrations of pancreatic tumor markers CA 19-9, CA 50 and elastase 1 in cystic fibrosis (CF) prompted us to investigate the pancreatic markers DU-PAN-2 and TATI. DU-PAN-2 was measured in serum samples of 48 CF patients, 42 pediatric control patients, and 51 parents of CF children by a competition radioimmunoassay (RIA). A commercially available RIA test kit was used to measure TATI serum levels of 38 CF patients and 40 control patients. Nineteen percent of CF patients, 0% of the control group, and 6% of the parents exceeded the DU-PAN-2 threshold value of 300 U/ml. TATI concentrations were increased (greater than 20 micrograms/L) in 24% of CF patients and in 17% of control patients. The sensitivity-specificity curve of DU-PAN-2 was similar to those of CA 50 and elastase 1 but less discriminating than that of CA 19-9. However, the sensitivity-specificity profile of CA 19-9 can only be marginally enhanced in a range of low specificity by simultaneously determining elastase 1 and/or DU-PAN-2 antigens. TATI does not provide any diagnostic use in CF.
Mycological examination of sputum from 121 patients with cystic fibrosis by means of long-term culture (4 weeks) revealed the occurrence of Candida albicans in low quantities in 70%, Aspergillus fumigatus in 30%, and Exophiala/Wangiella dermatitidis in 9% of the examined patients. A. fumigatus causes frequently the development of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. The predisposing factors for colonization with the otherwise seldom recovered fungus E. dermatitidis in these patients and the consequences of these findings are discussed. In conclusion, long-term fungal cultures are advocated for specimens from CF patients.
Symptoms of gastrointestinal disease are variable in Langerhans' cell histiocytosis (LCH). The incidence of gastrointestinal involvement is estimated to be approximately 5% in disseminated LCH. This report focuses on a 10.5 months old female infant who suffered from relapsing diarrhea and intermittent anal blood loss since the second week of life and from weight loss later in the course of disease. Definite diagnosis of LCH was not established before the patient developed additional characteristic symptoms. Analysis of large series of patients and a retrospective histopathological study reveals that an involvement of the gastrointestinal tract must be anticipated in about 50% of all cases with disseminated LCH.
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Smooth muscle cell biopsies obtained at pyloromyotomy from 37 children with infantile hypertrophic pyloric stenosis (IHPS) were studied by light and electron microscopy and compared with 6 autopsy control cases without any clinical evidence of this disorder. In cases with IHPS an apparently irregular increase in the number of smooth muscle cells by mitosis was accompanied by an increase of the endoplasmic reticulum, proliferation of mitochondria and regressive changes, such as shrinkage, swelling, necrosis and apoptosis of smooth muscle cells. Other alterations, seen in some but not all cases consisted of large numbers of unusual dense granules some of which were clearly associated with actin filaments and, therefore, regarded as derivatives of the normally occurring dense bodies. Furthermore, intermyofibrillar and subsarcolemmal glycogen accumulations, various nuclear abnormalities and pleomorphic membranous cytoplasmic or nuclear bodies occurred. While smooth muscle cell abnormalities predominated in some cases of IHPS, in others there were more severe axonal changes in the myenteric plexus. It is suggested, therefore, that a primarily myogenic type of IHPS can be distinguished from a predominantly neurogenic type.
The treatment of neonatal apnea and bradycardia with methylated xanthines--theophylline and caffeine--is generally accepted. Besides the desired effects of these drugs they induce a wide range of side effects including relaxation of smooth muscles and increased gastric secretion. The aims of this study were, at first to investigate the coincidence of periodic breathing (PA) and acid gastro-esophageal reflux (GER) in neonates (n = 15) without therapy; at second to examine the influence of the consecutive medication with theophylline and caffeine on these parameters in patients (n = 10) with recurrent episodes of bradycardia and apnea. A 24 h esophageal pH-monitoring and 24 h cardiorespirography were performed simultaneously under standarized conditions. In the 15 neonates studied a weak correlation was found between the time spent breathing periodically and the duration of GER; the overlap of PB and GER was minimal. Theophylline and caffeine medication resulted in a marked reduction of PB which was more pronounced than it could be expected from maturation. The total time of a 24 h esophageal pH-monitoring was subdivided in an early postprandial time (FPP: first two hours after the beginning of a meal) and a late postprandial time (SPP: remaining time until the following meal). An increased duration of acid GER was observed during the SPP under therapy with theophylline and even more distinct with caffeine treatment.
During an acute respiratory infection, a 4-year-old girl with propionic acidaemia deteriorated rapidly, becoming comatose with a marked acidosis. She did not respond to standard treatment but during haemodialysis her clinical state quickly improved.
Encephalocraniocutaneous lipomatosis is a rare neurocutaneous dysgenetic syndrome, which is characterized by unilateral lipomas in the subcutis of the face, skalp and skull, porencephalic cyst of the ipsilateral hemisphere, epilepsy, and severely delayed development. Furthermore a variety of skin lesions (choristomas) has been described. In the present case the skin lesions had the form of a linear nevus sebaceus. This condition is frequently combined with epilepsy and neurological disorders (Schimmelpenning-Feuerstein-Mims-syndrome). The latter seems not to be a very rare syndrome. The coincidence of characteristics of both syndromes in the same child supports the suggestion of a pathogenetical relationship.
The physicochemical properties of the blood-to-brain barrier, the cerebrospinal fluid (CSF) to brain barrier, and the blood-CSF barrier, including their specific transport mechanisms, are responsible for drug concentration time courses in the CSF, which increase slower and remain lower than in the serum. From the kinetic point of view the central nervous system (CNS) can be understood as a deep compartment in the organism. During the initial inflammatory phase of a bacterial meningitis, the penetration of antibiotics can be increased. Consequently, sufficient CSF concentrations should be achieved as soon as possible. To investigate the influence of the dosage regimen on the kinetic profile of antibiotics in the CSF, chloramphenicol and cefotaxim were measured using high pressure liquid chromatography (HPLC)-techniques. Five children had a bacterial meningitis and five an external ventricular drainage due to various diseases. Neither continuous infusion over a 12-24-hour-period nor four bolus-injections per day led to sufficient antimicrobial concentrations within the first hours after starting therapy. Using a loading dose of 40-50% of the daily dose administered over a 2-4-hour infusion period and followed by bolus injections, sufficient concentrations of chloramphenicol and cefotaxim could be achieved within a few hours. Besides an adequate choice of antibiotics, the special pharmacokinetic properties of the cerebrospinal membranes should not be neglected in the concept of the antibiotic therapy of meningitis.
Di-methyltin-dichloride (DMTC), di-butyltin-dichloride (DBTC), di-octyltin-dichloride (DOTC) and two commercial products (CP 1, CP 2) used as stabilizers in plastics induce a dose-related decrease of the thymus weight in young rats when given by gastric intubation for 10 or 14 days; in the chicken a decrease of the bursa Fabricii weight can also be observed. It is of interest, that there are distinct differences in response between the animal species. The sensitivity in acute toxicity tests does not correspond with the sensitivity of thymus and bursa Fabricii. The response is also dependent on the vehicle in which the agent is given.