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Biomedical subjects

D H Shmerling

Publications and source records attributed to D H Shmerling.

At least 19 recordsLinked to original sources
PubMed1992

Short-term changes in erythrocyte alpha-tocopherol content of vitamin E-deficient patients with cystic fibrosis.

Polyunsaturated fatty acids of biomembranes are a major target of lipid peroxidation. In vitamin E deficiency an efficient delivery of a high oral loading dose of all-rac-alpha-tocopheryl acetate to erythrocyte membranes could provide an early onset antioxidative effect. We investigated short-term changes in erythrocyte alpha-tocopherol after a single oral dose of 100 mg all-rac-alpha-tocopheryl acetate/kg in 10 vitamin E-deficient cystic fibrosis (CF) patients. Over 24 h, erythrocyte alpha-tocopherol increased 68% to 420% of preloading concentrations. With two exceptions, peak values were achieved 12 or 24 h after administration, which was 3-18 h later than peak plasma concentrations. Separate median-based curve estimates for the changes in erythrocyte alpha-tocopherol for five patients with and five without associated cholestatic liver disease were obtained. Cross-sectional test results revealed significantly lower erythrocyte alpha-tocopherol for the 9- and 24-h observations for patients with cholestatic liver disease compared with those without. Oral all-rac-alpha-tocopheryl acetate can be rapidly incorporated into erythrocyte membranes in vitamin E-deficient CF patients.

Adolescent↗
PubMed1992

Progressive idiopathic cholestasis presenting with profuse watery diarrhoea and recurrent infections (Byler's disease).

The second child of healthy unrelated parents presented with chronic diarrhoea since the age of two months, initially associated with non-characteristic liver involvement. Recurrent infections, severe failure to thrive and various metabolic deficiencies complicated the further course, as well as profuse watery diarrhoea with elevated regulatory gut peptides, responding only to somatostatin analog treatment. At 22 months of age, intermittent cholestasis with permanently normal serum gamma-glutamyltransferase was evident. The child died of fulminant purulent meningitis at the age of three years six months. Liver histology showed intrahepatic cholestasis, bile duct paucity with focal proliferation as well as slight portal and intralobular fibrosis. The clinical, biochemical and histopathological findings were indicative of Byler's disease.

Cholestasis, Intrahepatic↗
PubMed1990

[Diagnosis and treatment of celiac disease--what is the status in 1990?].

Summarizing the development of experiences and discussions of the last 20 years, a working group of the ESPGAN has recently updated the criteria of diagnosis of coeliac disease. The reliable finding of the initial typical mucosal lesion in the untreated patient is a hallmark of the disease and, if followed by an unequivocal clinical response to a gluten free diet, can be considered as final evidence for the diagnosis. Gluten challenges should be limited to cases without this evidence. High levels of antigliadin- and antireticulin- or or antiendomysium antibodies are supportive for the diagnosis but cannot substitute biopsy proof of the disease. The importance of a very long term strict gluten free diet in coeliac disease is established by recent evidence showing that in such cases there is no increased risk of malignancy.

Celiac Disease↗
PubMed1979

[Coeliac disease: an analysis of the clinical data in 176 patients (author's transl)].

A retrospective analysis of the case histories of 176 infants and children with documented coeliac disease born between 1953 and 1975 revealed the following data: Gluten was introduced into the diet of 49% of these patients at an age of 3 to 4 months. The interval between the introduction of gluten and the appearance of first symptoms was very variable and independent of age, occurring within 4 weeks in 32% and within 2 weeks in 20% of cases. In 13% this interval was 6 to 13 months. 91% of cases presented during the first year of life. Signs were also variable, the most frequent combination being failure to thrive, abnormal stools, anorexia vomiting and abdominal distension. In young infants symptoms tended to be more severe, whilst in children older than 2 years stunting of growth was the most frequent single clinical finding.

Celiac Disease↗
PubMed1979

The use of a programmable pocket calculator in clinical dietetics.

The application of programmable pocket calculators to clinical dietetics is described. The development of programs for the HP-67 and 97 for the evaluation of nutritional intakes of patients with obesity, renal disease, etc. and for the calculation and interpretation of food intakes in nutritional surveys is given in detail. The calculators simplify the practical work, shorten the calculation time substantially and allow direct incorporation of newly published data into analysis.

Diet↗
PubMed1979

The diagnosis of coeliac disease. A commentary on the current practices of members of the European Society for Paediatric Gastroenterology and Nutrition (ESPGAN).

In 1977, 53 members of ESPGAN completed a questionnaire on their current practice in diagnosing coeliac disease. The usefulness of the 'Interlaken' criteria enumerated 9 years previously was reassessed. Details were obtained about the initial diagnostic approach, the acceptable histological criteria of the initial jejunal biopsy, and the timing, technique, response, and interpretation of early and late rechallenges with gluten. Answers indicated that, although the initial mucosal lesion is usually 'flat' at the time of diagnosis, a few infants may present at a time when the mucosal lesion is less completely damaged. Furthermore, the degree of histological change after gluten challenge that is acceptable as a positive response may vary according to the state of the mucosa before challenge. It was noted that there are still no generally agreed criteria by which the histological lesions may be described, so that (after further discussions at the Third International Coeliac Conference in Galway) a European panel has been set up to make recommendations. In the experience of ESPGAN members, most coeliac children will have a histological relapse within 2 years of reintroduction of gluten. But a small number of unorthodox cases were reported that suggest that (a) histological relapse may take longer than 2 years to appear, or (b) the degree of sensitivity to gluten may vary at different ages. Very long-term follow-up will be needed to explain these anomalies. Meanwhile the search continues for 'the basic defect'.

Age Factors↗
PubMed1979

[Effect of previous breast feeding and early diagnosis on the course of cow's milk protein intolerance].

The clinical course of 21 children with cow's milk protein intolerance (CMPI) who were treated at the Children's Hospital of Zurich during the years of 1963-1977 was analysed in retrospect. Oral cow's milk challenges undertaken 3 weeks after the beginning of the treatment or later did not lengthen the apparent duration of the disease. Children who had initially been breast fed during at least 10 days, and those who were treated within 2 weeks after the appearance of the first symptoms with a cow's milk-free diet presented a milder and shorter course of CMPI. Severely undernourished or dehydrated patients did not differ from others in the course or duration of the disease, and patients with bloody stools showed a less serious but not shorter course of the CMPI.

Animals↗
PubMed1978

PABA screening test for exocrine pancreatic function in infants and children.

P-Amino-benzoic acid (PABA) is split specifically by pancreatic chymotrypsin from the synthetic tripeptide N-benzoyl-L-tyrosyl-PABA. The urinary excretion of absorbed PABA serves as an index for exocrine pancreatic function. The peptide (0.015 g/kg) was administered orally to 20 controls (aged between 5 months and 16 years), 6 patients with exocrine pancreatic insufficiency caused by cystic fibrosis (CF), and 9 newborn infants. In the controls the mean 6-hour PABA recovery was 58.5% (+/- 11.2 SD). Recovery in patients with CF was lower (P less than 0.001) with no overlap. In newborn infants the mean 6-hour PABA recovery was 23.4 (+/- 17.7 SD); overlapping in 3 instances with the results in CF patients. This simple, noninvasive test thus appears promising and merits further investigation in younger infants, especially newborns.

4-Aminobenzoic Acid↗
PubMed1978

Growth retardation and bone mineral status in children with coeliac disease recognized after the age of 3 years.

Growth data, clinical symptoms and bone mineral parameters were analyzed in 20 children with coeliac disease in whom the diagnosis was established by biopsy at age 3-13 years. Small stature and bone age retardation (greater than 2 SD) were present in 65% and 60%, respectively. Typical clinical symptoms of coeliac disease as found in the younger child were present in many cases, but 3 were completely asymptomatic except for severe growth retardation. Metacarpal diameters and cortical thickness were significantly decreased for chronological age but in most cases normal for bone age. Quantitative bone mineral analysis of the radius by computed tomography revealed normal values for height and weight in the 4 cases investigated. It is concluded that coeliac disease should always be considered in the differential diagnosis of retarded growth and bone age. "Osteoporosis" may occur in coeliac disease, but does not necessarily accompany growth failure. The analysis of metacarpal diameters and cortical thickness in the search of "osteoporosis" may result in false interpretation if not correlated to height and weight.

Adolescent↗
PubMed1978

Granulomatous ileocolitis (Crohn's disease).

In a clinical survey, the symptoms, associated diseases, and local and metabolic complications of Crohn's disease are discussed. Medical treatment (with SASP, corticosteroids, and immunosuppressives), as well as treatment for malnutrition, is of special importance.

Adrenal Cortex Hormones↗
PubMed1978

Ulcerative colitis.

The clinical symptoms of ulcerative colitis are described and three main points are emphasized: 1. The importance of associated diseases of ulcerative colitis (hepatic involvement, arthropathy, skin affections, uveitis and thyroiditis). 2. The importance of psychopathological conflicts in the pathogenesis and treatment of the disease. 3. The necessity to have a well-balanced team of pediatricians, pediatric surgeons, psychologists or psychotherapists, pathologists, social workers and stoma therapists for optimal therapy.

Child↗
PubMed1977

Long-term results following extensive small intestinal resection in the neonatal period.

At Alder Hey Children's Hospital in Liverpool there have been 8 children who survived long-segment intestinal resection in the neonatal period for more than 10 years, and there has been one further case at the University Children's Hospital in Zürich. The total of 9 children who were left with residual lengths of small intestine of between 26 and 75 cm form the basis of this report. Seven of these children have been personally followed up. One patient, although apparently perfectly well, is now an adult, has left home and refused to be re-examined. One child has been followed up by another paediatrician. In general it can be said that, provided these children are treated with care postoperatively over a period which may extend for many months or even a few years, the ultimate prognosis is extremely good. These children will grow up perfectly normally and will have no absorption difficulties. If the child is left with less than 30 cm of small intestine, the initial treatment becomes very difficult, but with adequate management even these infants can finally grow up into perfectly normal children with little or no absorption difficulties. The critical length of intestine lies in the neighbourhood of 20 cm. Correct initial treatment is all important and the one case in this series where this was not carried out is the only child who has still considerable difficulties. In some of the children under discussion, a hemicolectomy was performed as well as the small intestinal resection. This does not seem to have made much difference to the ultimate satisfactory outcome.

Adolescent↗
PubMed1977

[Catamnestic study in 82 patients with unspecified changes in the small intestinal mucosa].

A definitive diagnosis could not be established in 135 out of 713 (19%) infants and children admitted for chronic diarrhea and/or failure to thrive during the first admission. Their mucosal biopsy findings were either normal or showed unspecific changes. A retrospective inquiry, 1 1/2 to 8 years later, into the later course of 82 out of the initial 135 children revealed that 76.5% were free of symptoms without any specific therapy within a short time after discharge. The remaining 19 cases still complained of similar symptoms as initially. In 6 of these patients the indication for further investigations was established. These patients presented already at the time of their first admission with the most severe mucosal alterations (convoluted pattern) and clinical symptoms. Although the majority of patients with mild and non-specific mucosal abnormalities proved to have had a self-limited harmless intestinal disease, in 7,3% of them further investigations during follow-up remain indicated.

Biopsy↗