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Biomedical subjects

W Zäh

Publications and source records attributed to W Zäh.

9 recordsLinked to original sources
PubMed1985

[Acute liver necrosis caused by valproate].

After treatment with valproic acid a 19-year-old female patient with Friedreich's ataxia and generalised epilepsy died following acute hepatic failure with massive lactacidosis. The clinical symptoms were characterised by hyperventilation, increasing loss of consciousness and shock, leading to treatment-resistant hepatic coma. Morbid anatomy showed extensive confluent lytic necroses of liver acini with accentuation of centrolobular and intermediary structures as well as small and medium-sized fatty degeneration increasing from the periphery towards the centre. The disease picture is quite characteristic for being caused by valproic acid. As a safety measure liver function tests should be done should prodromal symptoms such as anorexia, weakness and apathy arise. If necessary the dosage has to be reduced or medication stopped. Serum valproic acid levels should remain in the lower half of the therapeutic range.

Adult↗
PubMed1979

Immunoreactive somatomedin B in acromegaly and in Turner's syndrome.

Serum somatomedin B was measured by radioimmunoassay in forty-seven normal subjects, twenty-nine patients with acromegaly before and twenty-four after treatment, and eighteen patients with Turner's syndrome. Somatomedin B levels were significantly elevated in untreated acromegaly and in Turner's syndrome compared with the control group; they decreased following treatment of acromegaly. Because of the overlap between the groups, little information could be obtained from single somatomedin B estimations, which could, therefore, not replace dynamic tests of growth hormone secretion. No correlation between growth hormone and somatomedin B in acromegaly was detected; however, somatomedin B appeared to be related to the insulin response during the oral glucose tolerance test. In Turner's syndrome, no relationship between somatomedin B and insulin production, urinary oestrogen excretion, growth hormone secretion, gonadotrophin levels, age or height was found. The reason for the raised somatomedin B levels in Turner's syndrome remains at present unknown.

Acromegaly↗
PubMed1979

[Pure gonadal dysgenesis. Case report with unusual anatomical and endocrine findings].

The syndrome of pure gonadal dysgenesis (PGD) cannot always easily be distinguished from other disorders of gonadal development. Relations are evident with Turner's syndrome, females with hypoplastic ovaries, male pseudohermaphroditism, mixed gonadal dysgenesis and the vanishing testes syndrome. The case is reported of a 40 year old female with primary amenorrhea, alopecia, eunuchoid features, XY karyotype with normal breast development and sexual hair after estrogen therapy. On laparotomy streak ovaries were found at ovarian site. Pathohistological examination revealed on the left side wolffian duct remnants such as ductuli deferentes and epididymis besides sparse Leydig-(hilus-)cells and on the right side only a rudimentary fallopian tube with subendothelial accumulation of hyperplastic Leydig-(hilus-)cells. Serum-testosterone elevation above the normal female range (630 ng/dl) persisted following gonadectomy (151 ng/dl). Ectopic Leydig-(hilus-)cells were regarded responsible for the continuing testosterone production. The present case lies on borderline between PGD and mixed gonadal dysgenesis because remnants of wolffian duct derivatives suggest unilateral fetal testicular activity; classification as PGD however was justified in purely female body features and lacking evidence of testicular tissue.

Adult↗
PubMed1976

Long-term treatment of acromegaly with bromocryptine: postprandial HGH levels and response to TRH and glucose administration.

Fourteen patients with acromegaly were treated with bromocryptine (CB 154, Sandoz), 4 X 2.5 mg, for periods of up to eleven months. One patient did not tolerate the drug, ten of the remaining thirteen experienced considerable clinical improvement. There was a dose-dependent suppression of plasma growth hormone levels, but growth hormone response to TRH injection and to glucose administration was still present during therapy although reduced. TSH response to TRH was not significantly altered. The suppressive power of bromocryptine on growth hormone appears to be related to the mechanism by which TRH stimulates growth hormone secretion in acromegaly, but long-term administration of this drug may be successful in spite of an absent response to TRH in some cases. Bromocryptine appears to be a safe and effective drug for the treatment of acromegaly.

Acromegaly↗
PubMed1975

[Abnormal hypothalamic-hypophyseal regulation as a new endocrine sign in the testicular feminisation syndrome (author's transl)].

In a 38-year-old woman with the testicular feminisation syndrome (TFS) there were increased serum levels of LH and FSH, despite normal testosterone levels, ranging from 808 to 1330 ng/dl. The patient exhibited complete end-organ resistance to androgens, as well as decreased free testosterone fraction of 1.5 percent. On stimulation with LH-RH there was enhanced LH reaction to 15 ng/ml, while FSH levels remained unchanged. Fluoxymesterone did not suppress LH and testosterone, as it did in normal controls. But the functional Leydig-cell reserve could be normally stimulated with HCG. Our results apparently confirm the suspected reduction in sensitivity of the hypothalamic-pituitary axis to androgens and suggest that this plays an important part in the pathogenesis of TES.

Adult↗
PubMed1975

Mixed gonadal dysgenesis.

Mixed gonadal dysgenesis (MCG) is a rare intersexual disorder, characterized in most cases by the presence of a testis and a contralateral streak gonad; in some cases the contralateral gonad may be rudimentary not having differentiated into an ovary or into a testis and in other cases it may be absent. A personal case was recently studied by the authors in some detail: A 51-year-old individual reared as a female with primary amenorrhea, short stature, subnormal intelligence, male type habitus, hirsutism, moderate breast development, ambiguous external genitalia with a 5 x 2 cm phallus, labia majora with scrotal type skin, and a urogenital sinus. Internal genitalia consisted of a hypoplastic vagina and uterus, a streak gonad on the right and intraabdominal testis, fallopian tube, and epididymis on the left side. The dysgenetic testis contained a gonadoblastoma and the streak a gonadoblastoma and dysgerminoma. A buccal smear was chromatin negative and karyotype analysis revealed XO/XY mosaicism. Thyroid and adrenocortical function was normal. A plasma testosterone level of 0.15 mu-g% rose to 0.45 mu-g% with 3 days of HCG administration. With bilateral gonadectomy the plasma testosterone level fell to 0.06 mu-g% and there was no response following HCG administration. 109 cases of MGD and 2 cases of unilateral gonadal dysgenesis reported in the world literature between 1937 and 1973 were reviewed. On the basis of their clinical and pathological features, the latter two patients were excluded from the category of MGD. In 15 of the 109 cases of MGD, a gonadoblastoma obscuring the gonad of origin was opposed by a dysgenetic gonad with or without a gonadoblastoma or no gonad. In these cases the presence of a testis was not proven histologically and their clinical-pathological features were similar to those found in pure gonadal dysgenesis. Of the remaining 94 cases of MGD, 72 had testicular tissue on one side and a streak or embryonic gonad on the other while 22 had a testis and no contralateral gonad...

Amenorrhea↗