scholarly journals CLINICAL CASE OF 19-YEARS-OLD PATIENT WITH CONGENITAL ADRENAL CORTEX DYSFUNCTION

2016 ◽  
Vol 7 (1) ◽  
pp. 64-69
Author(s):  
S I Ascolskaya ◽  
R H Tàzitdinov ◽  
Yu V Popov ◽  
E L Soyustova ◽  
A B Okulov
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Steroid Hormones ◽  
Adrenal Cortex ◽  
Clinical Case ◽  
Clinical Observation ◽  
In Utero ◽  
Surgical Correction ◽  
Adrenal Hyperplasia ◽  
Hormonal Profile ◽  
Enzymatic Systems

Congenital adrenal hyperplasia is caused by partial or complete block of enzymatic systems involved in the synthesis of steroid hormones of the adrenal cortex and leading to reduced production of glucocorticoids. Surgical correction is subject to, developing in utero, as a result of changes in hormonal profile, virilization. The article describes the clinical observation of a girl of 19 years, which has not underwent surgery for this disease at the usual term.

scholarly journals Molecular Characterization of Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Lesions With Both Adrenocortical and Leydig Cell Features

2015 ◽  
Vol 100 (3) ◽  
pp. E524-E530 ◽  
Author(s):  
Evelien E. J. W. Smeets ◽  
Paul N. Span ◽  
Antonius E. van Herwaarden ◽  
Ron A. Wevers ◽  
Ad R. M. M. Hermus ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Cortex ◽  
Leydig Cell ◽  
Adrenal Hyperplasia ◽  
Cell Markers ◽  
Expression Levels ◽  
Adrenal Tissue ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

Context: Testicular adrenal rest tumors (TART) are one of the major long term complications in patients with congenital adrenal hyperplasia. Although several adrenal-like properties have been assigned to these benign lesions, the etiology has not been confirmed yet. Objective: The aim of this study was to describe TART in more detail by analyzing several (steroidogenic) characteristics that may be classified as adrenal cortex or Leydig cell specific. Methods: Gene expression analysis by qPCR was performed for 14 genes in TART tissue (n = 12) and compared with the expression in healthy control fibroblasts (nonsteroidogenic control). In addition, a comparison was made with the expression levels in testis tissue (n = 9) and adrenal tissue (n = 13). Results: Nearly all genes were highly expressed in TART tissue, including all genes that encode the key steroidogenic enzymes. TART expression levels are in the majority almost identical to those found in adrenal tissue. The expression of adrenal cortex specific genes (CYP11B1, CYP11B2, and MC2R) in both TART and adrenal tissue is approximately 1000–10 000 times higher compared to that in testes samples. In addition, the Leydig cell markers INSL3 and HSD17B3 were not only found in testes, but also in TART, both at significantly higher levels than in the adrenal (p < 0.01). Conclusion: Our study shows for the first time that TART have multiple steroidogenic properties, which include not only the expression of adrenal cortex but also of Leydig cell markers. Therefore, the origin of these tumors might be a more totipotent embryonic cell type.

Prenatal Therapy in Congenital Adrenal Hyperplasia Attempted Prevention of Abnormal External Genital Masculinization by Pharmacologic Suppression of the Fetal Adrenal Gland in Utero

1985 ◽  
Vol 458 (1 Congenital Ad) ◽  
pp. 156-164 ◽  
Author(s):  
GEORGE P. CHROUSOS ◽  
MARK I. EVANS ◽  
D. LYNN LORIAUX ◽  
JAMES McCLUSKEY ◽  
JOHN C. FLETCHER ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Congenital Adrenal Hyperplasia ◽  
In Utero ◽  
Adrenal Hyperplasia

Sonographic Appearance of Congenital Adrenal Hyperplasia In Utero

2002 ◽  
Vol 21 (1) ◽  
pp. 97-100 ◽  
Author(s):  
Evelyne Diane Chambrier ◽  
Claudine Heinrichs ◽  
Fred E. Avni
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
In Utero ◽  
Adrenal Hyperplasia ◽  
Sonographic Appearance

scholarly journals A CLINICAL OBSERVATION OF THE COMBINATION OF OMPHALOCELE WITH ATRESIA OF THE SMALL INTESTINE OF TYPE 3A IN A NEWBORN

2019 ◽  
Vol 23 (5) ◽  
pp. 285-287
Author(s):  
Kapan T. Tursunov ◽  
S. A. Myrzakhmet ◽  
U. B. Alseitov ◽  
E. G. Nasirova
Keyword(s):  
Small Intestine ◽  
Surgical Treatment ◽  
Abdominal Wall ◽  
Clinical Case ◽  
Clinical Observation ◽  
Congenital Defect ◽  
Surgical Correction ◽  
Type Iii ◽  
Rare Combination ◽  
Treitz Ligament

The authors present a clinical case of an extremely rare combination of the congenital defect of the front abdominal wall as omphalocele, congenital evolution defect of the small intestine - atresia of the small intestine type III, agenesis of the ileum with ileocecal angle and malrotation syndrome. Surgical treatment was as follows: T-shaped ileocolic «end-to-side» anastomosis with an unloading colostomy by the Bishop-Koop technique and intestinal intubaton till the Treitz ligament. Outcomes of such surgical correction are good.

The 21-Hydroxylase Activity in the Glomerulosa and Fasciculata of the Adrenal Cortex in Congenital Adrenal Hyperplasia*

1981 ◽  
Vol 52 (3) ◽  
pp. 534-544 ◽  
Author(s):  
URSULA KUHNLE ◽  
DIANE CHOW ◽  
ROBERT RAPAPORT ◽  
SONGJA PANG ◽  
LENORE S. LEVINE ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Cortex ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Activity
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