RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)

We described a case of an endogenous hypercorticism in 18-years old patient with Itsenko - Cushing syndrome which was caused by a hormonally active adrenal tumor of the left adrenal gland. In a clinical picture of a disease the following symptoms dominated: arterial hypertension, increase in body we...

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Autores principales:	V. F. Sobotovich, Y. A. Privalov, L. K. Kulikov
Formato:	article
Lenguaje:	RU
Publicado:	Scientific Сentre for Family Health and Human Reproduction Problems 2017
Materias:	carney complex pigmental nodular adrenal dysplasia itsenko - cushing syndrome hypercorticoidism true lentiginosis autosomal dominant inheritance Science Q
Acceso en línea:	https://doaj.org/article/46b4ce64c1304c9ba612bea73bff8c07
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spelling	oai:doaj.org-article:46b4ce64c1304c9ba612bea73bff8c072021-11-23T06:14:37ZRARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)2541-94202587-959610.12737/article_59a614fe3caca8.08555042https://doaj.org/article/46b4ce64c1304c9ba612bea73bff8c072017-04-01T00:00:00Zhttps://www.actabiomedica.ru/jour/article/view/631https://doaj.org/toc/2541-9420https://doaj.org/toc/2587-9596We described a case of an endogenous hypercorticism in 18-years old patient with Itsenko - Cushing syndrome which was caused by a hormonally active adrenal tumor of the left adrenal gland. In a clinical picture of a disease the following symptoms dominated: arterial hypertension, increase in body weight, adiposity of the trunk, hands, neck, face, strips of extensions of skin on femurs. We found multiple nevus pigmentosus of black, yellowy-brown color from 1.5 to 10 mm on lips and mucous of a mouth, skin of a face and a trunk. The hormonal research established violations of a circadian rhythm of a hydrocortisone, increase in the free hydrocortisone of blood to 749.0 nmol/24 h (reference range 123.0-626.0), urine to 1314.0 nmol/24 h (reference range 60.0-413.0). The ACTH level decreased to 1.7 pg/ml (reference range 7.0-66.0). The negative result of the night overwhelming test with Dexamethazonum is received. Muitislice CT of adrenal glands revealed signs of a nodular hyperplasia of the left-hand adrenal gland. The laparoscopic adrenalectomy is made at the left. Pathoanathomical conclusion: a nodular pigmental hyperplasia of a glomerular and fascicular zone of bark of an adrenal gland with inclusion of a miyelolipomatoz. In the nodular corticocytes inclusions of lipofuscin granules were found. In the postoperative period symptoms of a hypercorticoidism regressed, throughout two years of observation there is no recurrence. Monitoring of hormones of blood in six months: hydrocortisone 359.5 nmol/l, ACTH 7.02 pg/ml. Retrospectively, the patient displays an infrequent heritable disease - Carney complex. The patient's mother at the age of 32 years underwent left-side adrenalectomy concerning Itsenko - Cushing disease with morphological confirmation of a nodular pigmental hyperplasia in adrenal gland. In both cases clinical convalescence occurred. Interest of the presented observation consists in its exclusive rarity. The unilateral adrenalectomy was efficient, but the probability of a recurrence of a hypercorticoidism and other manifestations of Carney complex remains.V. F. SobotovichY. A. PrivalovL. K. KulikovScientific Сentre for Family Health and Human Reproduction Problemsarticlecarney complexpigmental nodular adrenal dysplasiaitsenko - cushing syndromehypercorticoidismtrue lentiginosisautosomal dominant inheritanceScienceQRUActa Biomedica Scientifica, Vol 2, Iss 2, Pp 120-124 (2017)
institution	DOAJ
collection	DOAJ
language	RU
topic	carney complex pigmental nodular adrenal dysplasia itsenko - cushing syndrome hypercorticoidism true lentiginosis autosomal dominant inheritance Science Q
spellingShingle	carney complex pigmental nodular adrenal dysplasia itsenko - cushing syndrome hypercorticoidism true lentiginosis autosomal dominant inheritance Science Q V. F. Sobotovich Y. A. Privalov L. K. Kulikov RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
description	We described a case of an endogenous hypercorticism in 18-years old patient with Itsenko - Cushing syndrome which was caused by a hormonally active adrenal tumor of the left adrenal gland. In a clinical picture of a disease the following symptoms dominated: arterial hypertension, increase in body weight, adiposity of the trunk, hands, neck, face, strips of extensions of skin on femurs. We found multiple nevus pigmentosus of black, yellowy-brown color from 1.5 to 10 mm on lips and mucous of a mouth, skin of a face and a trunk. The hormonal research established violations of a circadian rhythm of a hydrocortisone, increase in the free hydrocortisone of blood to 749.0 nmol/24 h (reference range 123.0-626.0), urine to 1314.0 nmol/24 h (reference range 60.0-413.0). The ACTH level decreased to 1.7 pg/ml (reference range 7.0-66.0). The negative result of the night overwhelming test with Dexamethazonum is received. Muitislice CT of adrenal glands revealed signs of a nodular hyperplasia of the left-hand adrenal gland. The laparoscopic adrenalectomy is made at the left. Pathoanathomical conclusion: a nodular pigmental hyperplasia of a glomerular and fascicular zone of bark of an adrenal gland with inclusion of a miyelolipomatoz. In the nodular corticocytes inclusions of lipofuscin granules were found. In the postoperative period symptoms of a hypercorticoidism regressed, throughout two years of observation there is no recurrence. Monitoring of hormones of blood in six months: hydrocortisone 359.5 nmol/l, ACTH 7.02 pg/ml. Retrospectively, the patient displays an infrequent heritable disease - Carney complex. The patient's mother at the age of 32 years underwent left-side adrenalectomy concerning Itsenko - Cushing disease with morphological confirmation of a nodular pigmental hyperplasia in adrenal gland. In both cases clinical convalescence occurred. Interest of the presented observation consists in its exclusive rarity. The unilateral adrenalectomy was efficient, but the probability of a recurrence of a hypercorticoidism and other manifestations of Carney complex remains.
format	article
author	V. F. Sobotovich Y. A. Privalov L. K. Kulikov
author_facet	V. F. Sobotovich Y. A. Privalov L. K. Kulikov
author_sort	V. F. Sobotovich
title	RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
title_short	RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
title_full	RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
title_fullStr	RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
title_full_unstemmed	RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)
title_sort	rare case of endogenous family hypercorticism (clinical case)
publisher	Scientific Сentre for Family Health and Human Reproduction Problems
publishDate	2017
url	https://doaj.org/article/46b4ce64c1304c9ba612bea73bff8c07
work_keys_str_mv	AT vfsobotovich rarecaseofendogenousfamilyhypercorticismclinicalcase AT yaprivalov rarecaseofendogenousfamilyhypercorticismclinicalcase AT lkkulikov rarecaseofendogenousfamilyhypercorticismclinicalcase
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RARE CASE OF ENDOGENOUS FAMILY HYPERCORTICISM (CLINICAL CASE)

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