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Biomedical subjects

W Hoepffner

Publications and source records attributed to W Hoepffner.

At least 55 records · Page 3Linked to original sources
PubMed1978

[Therapeutic problems in the "short-bowel-syndrome" (author's transl)].

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.

Amino Acids↗
PubMedDate not supplied

[Long-term study of patients with coeliac disease in childhood and adolescence: latent and transient coeliac disease].

UNLABELLED: Coeliac disease (CD) is known as a lifelong condition of gluten intolerance. In case of some patients, the time after which gluten exposition leads to damage of the small intestinal mucosa may be very long. In addition to the florid form of CD, there are also silent (atypical mono- and oligosymptomatic with typical damage of the small intestinal mucosa) and latent forms (often asymptomatic without clear mucosal changes, antibody titres often normal). Occasionally the development of gluten tolerance is postulated (transient CD). We investigated 47 subjects diagnosed in childhood definitely as CD patients. In the age of 16.3 +/- 4.8 years, the patients started a gluten containing diet. After 6 to 9 months of gluten containing diet a small intestinal biopsy was performed in all patients. Surprisingly we found 11 patients with normal small intestinal mucosa (group 1). The other 36 patients showed a flattened mucosa as expected (group 2). The further development of group 1 was followed. In 9 patients a further biopsy was performed after more than 2 (at maximum after 8.1) years of gluten containing diet. In all patients the morphology of the mucosa was normal. In 7 of 11 cases normal numbers of intraepithelial lymphocytes were counted. Only in two patients raised titres of gliadin and endomysium antibodies were found after 4.5 and 15 years of gluten containing diet. In the other 9 patients no increase in antibody titers was found up to 10.3 years. However, in group 1, mucosal lactase activity was decreased as was also the case for group 2. CONCLUSION: A high number of adolescent coeliac patients does not respond or responds only minimally to reintroduction of gluten into the diet over a period longer than two years. These patients should regularly be further controlled serologically.

Adolescent↗
PubMedDate not supplied

[Acrodermatitis enteropathica (AE) is caused by mutations in the zinc transporter gene SLC39A4].

BACKGROUND: Acrodermatitis enteropathica (AE) is an autosomal recessively inherited disease caused by a decreased intestinal zinc resorption and characterized by severe dermatitis (preferably hands, feet, mouth, genital region), chronic diarrhoea, retardation of growth and development, alopecia and increased proneness to infections. In 2002 it was shown that mutations in the zinc transporter gene SLC39A4 is the cause of AE. CASE REPORT: Here we report 4 patients with typical clinical signs since early childhood. Under regular substitution with zinc all patients are more or less free of symptoms. The first patient revealed compound-heterozygous missense/nonsense mutations (P200L/ W401X), the three other patients were homozygous for a mutation in intron 1 (c.192 + 19G > A) of the SLC39A4 gene. CONCLUSION: The diagnosis of hereditary acrodermatitis enteropathica can now easily be confirmed by mutation analysis of the SLC39A4 gene.

Acrodermatitis↗
PubMedDate not supplied

Prediction of height velocity of prepubertal children with growth hormone deficiency in the first year of treatment with recombinant human growth hormone.

AIM: Several methods have been developed to predict the outcome of growth hormone (GH) therapy in children with growth hormone deficiency (GHD). METHODS: Over 50 factors for each of 92 prepubertal patients with GHD (26 patients with total and 45 patients with partial GHD, 21 patients with neurosecretory dysfunction) were collected and included in multiple regression analyses and other nonlinear models to predict height velocity (HV) (cm/yr) in the first year of treatment. Afterwards the model was validated by two other cohorts of patients from other universities, which followed the same treatment regime as our clinic. RESULTS: Twelve parameters had a significant correlation to HV (p<0.05) and a coefficient of determination >20%. Two parameters (In BA, In GHmax) showed a coefficient of determination >60% for children with GHD in multiple regression analysis. The validation of the mathematical model against another data set showed different results. GH was measured by the same method, but BA was scored at the first clinic as in our clinic by only one doctor and in the other university by several radiologists. The accuracy of prediction in the first clinic was significantly higher than in the other university. CONCLUSION: This model demonstrates that exact scoring of BA and precise measurement of GHmax in the stimulation test is necessary, and if carried out carefully leads to useful prediction values for determining height velocity.

Body Height↗