Biomedical subjects
Publications and source records attributed to W Hoepffner.
The mean height reduction in 23 tall girls was 3.8 cm in regard of Bayley-Pinneau height prediction. The results were independent of the doses und were better in younger than in older girls. However, corrections of height predictions show only a minimal mean height reduction of 1.1 cm.
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Stress response during genital surgery was investigated in 18 female patients with CAH. Changes were studied in blood pH and base excess, in serum concentrations of sodium- and potassium ions, in concentrations of 17 OHP in serum and saliva. The following conclusions may be drawn: On the day of operation patients should receive 100 mg Prednisolut per square metre of body surface area i.v., divided in three doses. On postoperative days 1 and 2 the 4 to 6 fold, on postoperative days 3 and 4 the 2 to 3 fold amounts of supplementary doses of hydrocortisone should be administered. Fludrocortisone is substituted with 3-5 mg DCA i.m. only on the day of operation. It is convenient to use a half-isotonic solution of electrolytes with 5% glucose for the infusion therapy. A dose of 25-50 mg Prednisolut i.v. can be added to the substitution therapy before short diagnostic and therapeutic procedures with or without anaesthesia such as endoscopy or change of bandage. Radenarcon (etomidate), used for induction of anesthesia, is contraindicated in patients with CAH.
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Plasma testosterone (T, nmol/l) and dihydrotestosterone (DTH, nmol/l) were measured in 54 children and young adults with male pseudohermaphroditism (46XY, no defect of steroid biosynthesis) 4 h after im injection of testosterone propionate (25 mg/m2, group 1, N = 18), or before and 2, 4 and 6 days after hCG (5000 IU/m2 im, group 2, N = 36). The response to hCG was also studied in 5 control children (unilateral cryptorchidism, group 3) and that to testosterone propionate in a gonadectomized child with confirmed 5 alpha-reductase deficiency. Mean T (133.1 +/- 14.0, SEM) and DHT (17.1 +/- 2.6) in group 1 were higher than in group 2 (17.3 +/- 2.1 and 2.9 +/- 0.4), but there was not significant difference in the T/DHT ratios (group 1: 10.7 +/- 2.0; group 2: 7.2 +/- 0.6). Following testosterone-propionate, there was a negative correlation of T with age (r = -0.723). After hCG, T and DHT were lower in the prepubertal children than in those under 2 or over 10 years, and the T/DHT-ratio rose with age. Two children from group 1 had a T/DHT-ratio above 18, but urinary aetiocholanolone/androsterone (Ae/A) ratios were normal. In the child with 5 alpha-reductase deficiency, the T/DHT ratio was 60, and the urinary Ae/A ratio high. We concluded that the two tests are suitable for confirming or excluding 5 alpha-reductase deficiency in prepubertal children, in whom basal DHT is too low for evaluation, but that physiological age-related changes in 5 alpha-reductase activity have to be taken into consideration.
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.
The diurnal rhythm of 17-hydroxyprogesterone concentration in the saliva was determined in 11 patients with congenital adrenal hyperplasia ranging in age from 9 to 20 years. A total of 21 tests were performed using a 17-OHP-enzyme immunoassay. The investigations were carried out during normal school or working days. The tests were done 16 times in patients with optimal therapeutic control and 5 times in patients with undertreatment. The concentrations of saliva 17-OHP in the morning were significantly lower after a therapy interval during the night of 8 hours than of more than 10 hours. In patients with poor substitution all values of the days were higher than in patients with optimal therapy control. The morning values are appropriate for the evaluation of the therapy control. With regular 8 hours' therapy intervals the limit between normal and pathological values may be about 500 pmol/l saliva.
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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.
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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).
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