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At least 145 records · Page 8Linked to original sources
PubMed2000

[Female pseudohermaphroditism in congenital adrenogenital syndrome as an incidental intraoperative finding in a 68 year old patient].

HISTORY AND CLINICAL FINDINGS: A 68-year-old man of small stature, previously always healthy and with a grown-up daughter, was suspected of having carcinoma of the colon with metastasis to the right kidney. At laparotomy internal female genitalia with cancerous changes were unexpectedly discovered in the left adnexae. The colon carcinoma, the right adrenocortical tumour and left adnexae were resected. INVESTIGATIONS: Histological examination revealed adenocarcinoma of the colon, right adrenocortical adenoma and a Brenner tumour of the left female adnexae. Postoperative tests showed increased levels of 17-OH-progesterone (3192 ng/dl), 21-desoxycortisol (1856 ng/dl) and of adrostenedione (745 ng/dl), while the concentrations of 17-OH pregnenolone, testosterone and mineralocorticoids were within normal limits. Chromosome analysis demonstrated karyotype 46 XX. DIAGNOSIS, TREATMENT AND COURSE: As far as could be ascertained, this is the first documented case in the German-speaking region of female pseudohermaphroditism diagnosed in an elderly person with uncomplicated virilizing congenital adrenogrenital syndrome due to 21-hydroxylase deficiency (deletion of CYP21 gene). To avoid a cortisone deficiency crisis the patient was regularly given hydrocortisone and he quickly recovered. But he died 6 months later of sequelae of the carcinoma of the colon. CONCLUSION: An adrenogenital syndrome should be excluded in a case of bilateral adrenocortical tumour. As this is usually benign, conservative treatment should be attempted. This case demonstrates the necessity of thorough examination which could have given an early indication of the underlying condition.

Adrenal Cortex Neoplasms↗
PubMed1986

[Arrhythmia-hyperkalemia in a newborn infant with congenital adrenogenital syndrome. Usefulness of cardioversion].

Cardiac arrhythmias can be life-threatening situations requiring swift action. Disorders of heart beat in pediatric age and, mainly, in neonatal period are very rare out of those occurring after cardiac surgery. Because of this there can be no training in their management on the part of pediatricians. A case of hyperkalemic arrhythmia in a newborn with adrenogenital syndrome in which electrical cardioversion was successful is presented. Furthermore, sequential electrocardiographic changes with hyperkalemia are shown and comments on physiopathology, diagnosis, treatment and etiology are made.

Adrenal Hyperplasia, Congenital↗
PubMed1989

[Neonatal levels of 17-hydroxyprogesterone--early diagnosis of adrenogenital syndrome].

The authors submit the results of 17-hydroxyprogesterone estimations in serum of healthy neonates after birth and during the first days of life. The 17-hydroxyprogesterone level declines markedly after birth to the fourth day after birth in infants of both sexes. The levels recorded in infants above 4 days (8.4 +/- 3.4 nmol/l) will serve as normal values in the diagnosis of adrenogenital syndrome caused by the block of 21-hydroxylase steroids. Estimation of 17-hydroxyprogesterone in umbilical blood cannot be used for the diagnosis of AGS in neonates.

17-alpha-Hydroxyprogesterone↗
PubMed2001

Ovarian 'tumor' of the adrenogenital syndrome: the first reported case.

We report the case of a 36-year-old woman with congenital adrenal hyperplasia from 21-hydroxylase deficiency who had been receiving replacement therapy with corticosteroids since birth. At the age of 35 years, she developed abrupt aggravation of her virilizing symptoms and underwent an adrenalectomy and partial left oophorectomy. Persistent virilization and high testosterone levels led to right oophorectomy and completion left oophorectomy 6 months later. Each adnexa contained ovarian or paraovarian soft brown masses that on microscopic examination were identical to the testicular tumor of the adrenogenital syndrome. This represents the first reported case of this pathology (well known in the testis) in the ovary.

Adrenal Hyperplasia, Congenital↗