Potential hepatotoxicity of penicillamine treatment in three patients with Wilson's disease.
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Biomedical subjects
Publications and source records attributed to G Scheerschmidt.
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Highly differentiated processes relating to insulin-generating cells of the endocrine pancreas are covered by the term of nesidioblastosis. The disease is primarily characterised by persistent hypoglycaemia, and it affects newborns and young infants. Diffuse nesidioblastosis is predominant, as compared to focal processes. So called ductulo-insular complexes are characteristic immunohistochemical manifestations. While dietary and medicamentous therapies (diazoxide) usually failed to be effective, surgical removal so far has worked better than any other approach (subtotal and total pancreatectomy). Adequate early diagnosis should be established and pancreatectomy performed even before irreversible cerebral damage is caused by glucose deficit. Operations for subtotal or total pancreatectomy were performed on five children with nesidioblastosis at the Department of Paediatric Surgery in Erfurt, over the last two years. Epilepsy continued to be manifest in one of the five. Success eventually depends on close cooperation between paediatrics and paediatric surgery.
Hyperinsulinemia due to an excessive secretion of insulin independent on normal regulation is the most frequent cause of persistent neonatal hypoglycemia. We report on clinical course, diagnostic procedures and treatment of nesidioblastosis in three patients. Main symptoms observed in newborn period were hypoglycemia, respiratory embarrassment, cyanosis and convulsions. Primary treatment was started by continuous infusion of glucose, administration of diazoxide and prednisolone or glucagon. Most important investigations were performed simultaneously. In all three children subtotal resection of pancreas was necessary, because there was no constant blood glucose level. Histological specimens confirmed diagnosis. In two of three patients pancreatectomy followed. One suffers from diabetes mellitus, the other one fed normally, has stable blood glucose level possibly due to existence of extrapancreatic insulin producing cells.
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We report on two brothers with chronic congenital lymphoedema. Besides the oedemas of limbs we found an unusual facial appearance, abnormalities of external genitals as a deformation sequence resulting from intrauterine oedemas and intestinal lymphoedema. This X-linked or autosomal recessive trait may be a new entity, to be differentiated from other genetic lymphoedema syndromes, the so-called familial protein-losing enteropathy, and dominantly inherited intestinal lymphangiectasia. A prominent sign of the syndrome is chemosis and injection of conjunctiva.
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The digestion rates of lactose and the absorption rates of glucose, galactose, and fructose were studied by continuous perfusion of the jejunum in three patients aged 7 weeks to 9 months with congenital glucose-galactose malabsorption (infusion rate:1.0 ml min-1; concentration of each sugar: 200 mM; perfusion distance: 30 cm). The mean absorption rates of glucose and galactose were 26.5 and 43.8 mumol min-1 30 cm-1, respectively, and were significantly reduced (p less than 0.001) to 13 and 22%, respectively, of intake. On the other hand, the absorption of fructose was 133.3 mumol min-1 30 cm-1, i.e., as high as in the controls. The hydrolysis rate of lactose was also normal (134.0 mumol min-1 30 cm-1). However, the absorption rates of glucose and galactose released from the disaccharide were as low as the perfusion of free monosaccharides. In patients with glucose-galactose malabsorption the glucose absorption rate is as low as that of galactose. No additional glucose transport system seems to exist. A hydrolase related transport system is also of no importance in compensating for the primary defect of monosaccharide absorption.
On the basis of experience with 35 children suffering from short-gut-syndrome its pathogenesis and clinical symptoms were discussed. Not all of these cases can be treated surgically. This burdens the surgeon with high responsibility to calculate the extent of bowel resection. With a two stage operation--enterostomy followed by anastomosis the area of resorption and the ileocoecal valve can be preserved.
Pancreozymin-secretin tests were carried out in children aged from 0,5 till 12 years by means of a two lumen tube of the Salem-Sump-type and a three lumen perfusion tube. For stimulation we used 2 U/kg of the hormones, each from BOOTs-Corp. Amylase was determined with dinitrosalicylic acid, lipase by titration of acidic equivalents after half hour incubation and trypsin and chymotrypsin with TAME and BTEE respectively. We used PEG 4000 as marker and quantified enzymes secretion as well as liquid secretion by it. We got up to 50% lower results by testing with the two lumen tube. If we are laying the perfusion tube we measured an elevation of the two lumen tube. If we are laying the perfusion tube we measured an elevation of the II-hydroxycorticoides. The concentration of the enzymes in the specimines before perfusion on 3 consecutive days fell by 90% on the average. We discussed some problems of the determination of enzymes and performing of the tests by means of the results.
17 former patients with nutritional marasmus due to malabsorption syndromes of various etiology were followed-up at the age of 5 7/12 +/- 2 1/12 years for the assessment of their psychomotor development. As to the intelligence quotient there was no significant difference in comparison with a representative random sample of 200 preschool children. The mean IQ was 92 +/- 20 (n = 17). 2 children were moron (IQ 64 and 61, resp.), one child was imbecile (IQ 46). In two of them familial disposition of debility must be assumed. The deficit in the capability of the psychomotor functions was striking (fine motoricity, designing). 6 children showed mild forms of electroencephalographic deviations from normal (relative frequency 0.35).
The authors report on a case of sepsis due to E. coli in a newborn baby with galactose intolerance. The immature immunological state of the newborn child in combination with a disorder of galactose metabolism obviously favour the development of bacterial infections. Galactose-free formulas should be applied quickly together with an adequate antibiotic therapy in the case of newborn babies with suspected bacterial infection. The prognosis may be influenced favourably by early clarification of the cause of severe impairment including jaundice, vomiting, loss of weight and somnolence in the first three weeks of life.
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The authors report on 6 children with short-bowel-syndrome. The expectancy of life of the patients depends on the mucosal surface of the residual intestine and on the degree of adaption. After removal of larger proportions of the intestine, nutrition should be started parenterally and followed by an early but cautious oral feeding. The administration must be distributed continuously over the day. At first carbohydrates are given as monosaccharides, fats as M. C. T. and proteins as amino acid mixtures. Carrot soup has proved useful as a water-binding substance for the improvement of stool consistency. An anabolic condition of the metabolism should be maintained. Therapy with cholestyramin is indicated in chologenic diarrhoea. Substitution of vitamins, especially vitamin B12 is very important after resection of the ileum.
The authors describe their experiences with the treatment of 3 newborns with meningitis due to B-streptococci,, and give a review of the problems of etiology, clinical symptoms, diagnosis and therapy of infections with B-streptococci in newborns. Furthermore, the article deals withthe frequency of the ocurrence of these bacteria in mothers, children and in the medical personal staff as well as with the possible ways of transmission. The relations between infections with B-streptococci and respiratory distress syndrome in newborns are discussed with the problems of differential diagnosis and therapy.