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Biomedical subjects

D H Shmerling

Publications and source records attributed to D H Shmerling.

At least 37 records · Page 2Linked to original sources

[Thoughts on the diagnosis, treatment and long-term course of patients with gluten-induced celiac disease].

The criteria for the diagnosis of coeliac disease are the evidence of a well characterized and typical, albeit not pathognomonic lesion of the proximal small bowel mucosa in untreated patients as well as the unequivocal rapid response to a gluten free diet (GFD). The instruction and the motivation of the patients in GFD aims at an early and positive establishment of dietary habits, based on an adequate choice of gluten free foods. As coeliac disease is genetically determined, GFD should be maintained life long. The results of long term evaluations of relapses in coeliac patients confirm this postulate by demonstrating that only 6.5% of the patients show no mucosal alteration upon gluten ingestion, even after years on GFD, and that another group (12-18%) will deteriorate very slowly over years. Routine gluten challenges in patients, whose initial diagnosis was established according to the discussed criteria, seem therefore unjustified, as they represent a serious challenge of established dietary and life habits for the vast majority of patients without any positive issue for them.

Biopsy↗

Follow-up examinations of conservatively and surgically treated children with hiatus hernia.

Between 1966 and 1976, 65 infants and children were treated because of hiatus hernia. The diagnosis was made radiologically in all cases and in a number of patients endoscopically. 54 patients had a forme mineure (types 1 and 2), and 11 had a forme majeure (types 3 and 4). Of the 20 patients with type 1, 16 (80%) were successfully treated conservatively; two further patients needed a secondary operation. Of 34 children with type 2, 15 (44%) were treated successfully conservatively, and four needed a secondary operation. Of ten patients with type 3, four (40%) were treated primarily conservatively with success. Two children with type 1, 11 with type 2, six with type 3, and one with type 4 had to be operated on primarily because of severe esophagitis at the time of admission. From 14 mon to 10 years after discharge from hospital, follow-up examination of 33 patients showed that one child died from another cause. In 14 of the 21 conservatively treated patients and in 12 of the 18 surgically treated patients (66% in both cases), there were no symptoms or complaints. Eight had subjective complaints, but in only four (10.5%) was there a definite complaint. Three of these four patients had associated diseases (spastic tetraplegia, operated jejunal atresia). In these patients, esophagitis was present on follow-up examination and necessitated further treatment. The treatment of choice for the forme mineure without esophagitis should be conservative, and in most cases this will be sufficient. In only a few of these patients did it become necessary to carry out a secondary fundoplication. Operation is indicated primarily in cases with esophagitis and esophageal stenosis as well as in patients with a forme majeure of the hiatus hernia.

Body Weight↗

[Acquired immunodeficiency syndrome in a child of Swiss origin whose mother died from AIDS].

Report of a now 2 8/12-year-old girl, who presented at the age of 8 months with chronic progressive pneumonia, mucocutaneous candidiasis, diarrhea, failure to thrive and a non-progressive paraplegia. The child's mother presented AIDS with pneumocystis carinii pneumonia and progressive general paralysis one year after the beginning of the child's disease and died within a few months. Additional findings in the child include lymphopenia, hyperimmunoglobulinemia, cutaneous anergy and an abnormal T helper/T suppressor cell ratio. HTLV-III antibodies were positive (ELISA and Western blot virus strip RIA). Prophylactic treatment with Co-trimoxazole relieved pulmonary infections but failure to thrive remained unchanged in spite of a continuous nutritional support. A vertical mode of transmission of AIDS from mother to child seems very probable.

Acquired Immunodeficiency Syndrome↗

Immunological and biochemical studies of an unusual alpha heavy chain protein in a 9-year-old boy.

Protein electrophoresis in a 9-yr-old Libyan boy suffering from intestinal alpha chain disease showed a conspicuous peak in the gamma region. Examination in immunoelectrophoresis with monospecific antisera against IgA revealed a quantitatively important monoclonal gradient. The heavy chain protein in serum, urine and duodenal juice was further analysed immunologically in double diffusion, antigen-antibody crossed electrophoresis and biochemically in gel filtration experiments and SDS PAGE. By these techniques alpha 1 chain aggregates of different molecular weight could be found. Immunofluorescence examination of small intestine biopsies showed abundant plasma cells containing alpha heavy chains but no light chains.

Child↗

The use of sweat osmolality in the diagnosis of cystic fibrosis.

Sweat osmolality determination with 7-10 microliter of sweat for the establishment of the diagnosis of cystic fibrosis (CF) was evaluated in 297 probands. In 12 out of 26 infants below 2 months of age and in 3 out of 271 older probands not enough sweat could be collected at the first attempt. A final diagnosis was established before the age of 2 months in 21 out of 26 infants. In 27 CF patients sweat osmolality was 285.86 +/- 41.25 mmol/kg (mean +/- SD), the mean value - 2 SD being 203.36 mmol/kg. In 266 control subjects sweat osmolality was 107.38 +/- 29.49 mmol/kg (mean +/- SD), the mean value + 2 SD being 166.36 mmol/kg. There was no overlap between CF patients and controls in each age group. The method is simple, rapid and reliable, and improves diagnostic possibilities especially in young infants. Sweat osmolality values above 180 mmol/kg should be repeated and checked for final confirmation or exclusion of the diagnosis of cystic fibrosis.

Adolescent↗

The incidence of coeliac disease in children in Switzerland.

In the pediatric hospitals and clinics of Zurich, St. Gallen, Winterthur, Aarau and Lucerne, 488 cases of coeliac disease have been found in children born between 1960 and 1978. The diagnosis was established clinically and confirmed by intestinal biopsy in each case. The data of coeliac patients diagnosed before 1966 and after 1975 has proven to be unreliable for statistical evaluation and were omitted from our calculations. During the years 1966-1975, 354 patients with confirmed coeliac disease and 412733 live births were registered. This corresponds, for the symptomatic coeliac disease, to an incidence of 1:1165 in the Northeastern part of Switzerland. A statistical analysis of the data confirmed its validity and the lack of trends during the period of study.

Celiac Disease↗

[Pneumothorax as complication of cystic fibrosis of the pancreas].

During recent years, 14 episodes of pneumothorax in 4 children and adolescents with cystic fibrosis of the pancreas (CF) have been treated in the University Department of Pediatrics in Zurich. Treatment with pleural drainage, associated with instillations of sclerosing agents was usually tedious and did not prevent relapses. One patient died after the second episode, another was successfully treated by pleurectomy. The incidence of pneumothorax in adolescent CF patients increases rapidly mostly due to the longer life expectancy of CF patients. Both lungs are equally affected. In more than 50% one or more relapses occur. The prognosis is usually bad. No specific etiologic factors can be incriminated, pneumothorax occurring mostly during periods of physical inactivity. The pathogenesis seems, in the majority of cases, to be the bursting of a subpleural emphysematous bulla or bleb. Such bullae can mostly be recognised radiologically many years prior to the first pneumothorax episode, usually in severely affected lungs in children over 10 years of age. From the reviewed literature as well as from our own experience it seems that pleurectomy is the only efficient treatment, being well tolerated, curing the actual episode of pneumothorax, and preventing relapses.

Adolescent↗

[Cholelithiasis as the first manifestation of cystic fibrosis of the pancreas (mucoviscidosis)].

A hitherto healthy 6-year-old girl developed cholestatic jaundice, due to multiple extra- and intrahepatic cholesterol bile calculi. Cystic fibrosis of the pancreas was established as the cause of the disease and of the biliary cirrhosis. Operative treatment and the ensuing substitution with pancreatic extracts have improved the cholestasis. Pulmonary involvement became subsequently evident and is now under treatment. The pathophysiology of bile acids in cystic fibrosis as relevant to the occurrence of gall stones in these patients is discussed.

Child↗

[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↗

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↗