Katecholaminabhängiges hereditäres Cushing-Syndrom-Verlaufsbeobachtung nach einseitiger adrenalektomie [Catecholamine-dependent hereditary Cushing's syndrome - follow-up after unilateral adrenalectomy]

Details

Serval ID
serval:BIB_64B15AFCC1A8
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Katecholaminabhängiges hereditäres Cushing-Syndrom-Verlaufsbeobachtung nach einseitiger adrenalektomie [Catecholamine-dependent hereditary Cushing's syndrome - follow-up after unilateral adrenalectomy]
Journal
Medizinische Klinik
Author(s)
Imöhl M., Köditz R., Stachon A., Müller K.M., Nicolas V., Pfeilschifter J., Krieg M.
ISSN
0723-5003[print], 0723-5003[linking]
Publication state
Published
Issued date
12/2002
Volume
97
Number
12
Pages
747-753
Language
german
Notes
Publication types: Case Reports ; English Abstract ; Journal Article
Publication Status: ppublish
Abstract
CASE REPORT: We report on the laboratory, clinical, radiologic and histological findings of a 44-year-old male patient who was hospitalized with the clinical signs of Cushing's syndrome. The laboratory findings were suggestive of an adrenal genesis. Abdominal computed tomography revealed macronodular hyperplasia of both adrenal glands. Specific endocrinological tests demonstrated an increased secretion of cortisol during adrenergic stimulation which, however, was absent following beta-blocker medication. This would seem to point to an aberrant, beta-receptor-mediated regulation of cortisol secretion as being the cause of increased cortisol production. ACTH-independent bilateral macronodular adrenocortical hyperplasia was also noted in the patient's mother. Neither the father nor the brother were affected by the disease. Apart from one case of familial appearance involving mother and daughter previously reported in the literature, this is the second description of a familial appearance and the first case involving mother and son. The patient underwent a left-sided adrenalectomy. Quick postoperative normalization of S-cortisol day profile and excretion of free dU-cortisol were noted under supportive temporary blockade of beta-receptors. In the further course, meanwhile spanning 2 1/4 years, a low basal cortisol secretion of the right adrenal gland has been observed, together with a lack of suppressibility in the dexamethasone suppression test. CONCLUSION: In the presence of mild clinical signs of adrenocortical insufficiency, matutinal administration of 5 mg hydrocortisone has proven sufficient for the past 6 months to reestablish a physiologic S-cortisol day profile with morning peak.
Keywords
Adrenal Cortex/pathology, Adrenal Cortex Function Tests, Adrenalectomy, Adrenocorticotropic Hormone/blood, Adult, Catecholamines/physiology, Cushing Syndrome/diagnosis, Cushing Syndrome/genetics, Dexamethasone/diagnostic use, Female, Follow-Up Studies, Humans, Hydrocortisone/blood, Hyperplasia, Male
Pubmed
Web of science
Create date
25/01/2008 13:52
Last modification date
20/08/2019 14:20
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