11/20. Bilateral ovarian lipid cell hyperplasia in a young hirsute patient.Virilizing lipid cell ovarian hyperplasia occurred in a 24-year-old woman referred with secondary amenorrhea, hirsutism, and weight gain. Hormone analyses revealed abnormal androgen production shown in a suppression test to be autonomic and of ovarian origin. Ovarian vein catheterization revealed abnormally increased androgen levels in the left ovarian vein and in the caval vein where it was entered by the right ovarian vein. As the patient wanted to become pregnant, three-quarters of her ovarian tissue was resected. menstruation and ovulation returned. Two years after the operation the patient conceived. Histopathological and electron microscopic examinations revealed bilateral lipid cell hyperplasia.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
12/20. Adrenocortical 11 beta-hydroxylation defect in adult women with postmenarchial onset of symptoms.Four cases in adults of a deficiency in the 11 beta-hydroxylation of corticosteroids were investigated by both basal and dynamic biological studies. Symptoms varied from patient to patient; hirsutism, menstrual disturbance, acne, deepening of the voice, and arterial hypertension appeared post puberty. Basal testing demonstrated elevated levels of plasma androgens. These include delta 4-androstenedione (patients, 3.80-6.43 ng/ml; normal, 1.33 /- 0.33 ng/ml), urinary 17-ketosteroids (patients, 11.8-16.7 mg/24 h; normal, 5-10 mg/24 h), and urinary dehydroepiandrosterone. The basal tests were often insufficient to show the accumulation of the precursors (especially 17-hydroxyprogesterone) which are often given as evidence for an increase in ACTH stimulation. In studying the levels of the mineralocorticoids, there was shown to be an increased basal level of tetrahydrodeoxycorticosterone (patients, 142-317 microgram/24 h; normal, 60-80 microgram/24 h) which was raised by ACTH stimulation. These results, therefore, confirm the characteristic partial enzyme defect and give evidence for the heterogeneity of this syndrome. Based on the above observations, we believe it is appropriate to rename this condition adult adrenocortical 11 beta-hydroxylation defect rather than late-onset congenital adrenal hyperplasia.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
13/20. Detection of late-onset 21-hydroxylase deficiency congenital adrenal hyperplasia in adolescents.Because severe hirsutism is difficult to reverse, the evaluation of the adolescent girl with progressive hirsutism should aim at the pathophysiology of androgen excess in order to select appropriate therapies. A prospective study was undertaken to determine the occurrence of late-onset 21-hydroxylase deficiency among adolescents with androgen excess. Twenty-two young women (mean age 17.3 /- 2.6 years) with androgen excess had serum 17-hydroxyprogesterone measured before and after bolus intravenous infusion of synthetic ACTH (Cortrosyn), 0.25 mg. Two patients, aged 13 and 19 years old, had elevated base line 17-hydroxyprogesterone and 30- and 60-minute responses to Cortrosyn consistent with 21-hydroxylase deficiency. Chromosome 6p haplotypes provided supportive evidence of 21-hydroxylase deficiency. The base line androgen levels, clinical presentation, and a four-day dexamethasone test did not distinguish patients with 21-hydroxylase deficiency from other hirsute adolescents. The Cortrosyn test identifies a population of adolescents who need long-term corticosteroid therapy. The use of major histocompatibility complex haplotypes could be of help in identifying affected siblings prior to the development of significant hirsutism.- - - - - - - - - - ranking = 0.66666666666667keywords = hyperplasia (Clic here for more details about this article) |
14/20. adult-onset familial adrenal 21-hydroxylase deficiency.Two sisters (28 and 30 years) were investigated for primary infertility and milk hirsutism. Both had normal puberty, were having regular menses and had normal female sexual characteristics. Studies revealed elevated urinary 17-ketosteroid levels (15.8, 18.8 mg/24 hours) and increased serum levels of 17-OH-progesterone (2,756, 1,121 ng/dl), 21-desoxycortisol (1,882, 1,090 ng/dl), progesterone (300, 346 ng/dl), dehydroepiandrosterone (DHA) (1,600, 1,700 ng/dl), and androstenedione (402, 366 ng/dl) and testosterone (100, 104 ng/dl), together with a slight increase in serum 11-desoxycortisol (1,180, 1,560 ng/dl). blood pressure, serum sodium/potassium plasma renin and serum aldosterone, corticosterone, 11-desoxycorticosterone and cortisol levels were normal. The administration of ACTH caused a further increase in 21-hydroxylase precursors; the administration of dexamethasone normalized hormone levels and produced ovulatory cycles. Similar studies in two siblings were normal. The affected sisters were HLA identical and did not share any hla antigens with their healthy siblings. The data suggest that these patients have a mild form of 21-hydroxylase deficiency which was insufficient to cause prenatal virilization. The gene for this disorder may be allelic with that for typical congenital adrenal hyperplasia.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
15/20. Strumal carcinoid of the ovary: an analysis of 50 cases of a distinctive tumor composed of thyroid tissue and carcinoid.Strumal carcinoid of the ovary is a type of germ-cell tumor characterized by an intimate mixture of thyroid tissue and carcinoid. Fifty patients with this type of tumor ranged in age from 21 to 77 years. The tumors measured up to 26 cm in diameter and were always unilateral. In 10% of the cases, the contralateral ovary contained another type of neoplasm, usually a dermoid cyst. Three-fifths of the strumal carcinoids arose in dermoid cysts or in mature solid teratomas. Birefringent calcium oxalate monohydrate crystals were commonly identified in the thyroid colloid, and argentaffin granules, in the carcinoid cells, thus substantiating the identity of the neoplastic components. Although 31% of the tumors were accompanied by focal stromal luteinization, only 8% of the cases exhibited clinical signs of steroid hormone production (endometrial hyperplasia, hirsutism, or virilism). In 8% of the cases, there was evidence suggesting functioning of the thyroid component, but no patient had the carcinoid syndrome. Only one woman died of tumour; five others died of unrelated causes. The remaining patients remained alive and well; 23, for at least five years, and 15, for at least ten years postoperatively. Although the carcinoid component of the strumal carcinoid has been considered a malignant transformation of struma ovarii, it is almost always benign, and treatment with a simple oophorectomy or salpingo-oophorectomy is effective.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
16/20. A case of late-onset congenital adrenal hyperplasia due to partial 3 beta-hydroxysteroid dehydrogenase deficiency.Late-onset congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency has been reported with increasing frequency, but only a few adult women have been found to have this disorder in japan. We report a 26-year-old Japanese hirsute woman with partial 3 beta-hydroxysteroid dehydrogenase deficiency. The diagnosis was based on significantly increased ratios of 17-hydroxypregnenolone to 17-hydroxyprogesterone and of dehydroepiandrosterone to androstenedione after administration of ACTH. hirsutism improved with the administration of dexamethasone (0.5 mg) every evening. Since routine assay of delta 5-steroid metabolites has become available, the incidence of this disorder will increase. Diagnostic effort should be attempted since the disorder is treatable with low-dose dexamethasone.- - - - - - - - - - ranking = 0.83333333333333keywords = hyperplasia (Clic here for more details about this article) |
17/20. Oral changes associated with biliary atresia and liver transplantation.A child is reported with green hypoplastic teeth in both dentitions, resulting from hyperbilirubinemia biliary atresia. Following liver transplantation, labial hirsutism, gingival hyperplasia and herpes labial, were seen as a consequence of cyclosporin therapy and iatrogenic immunosuppression. The oral manifestations and management in biliary atresia and after liver transplantation are reviewed.- - - - - - - - - - ranking = 0.16666666666667keywords = hyperplasia (Clic here for more details about this article) |
18/20. testosterone-producing solitary ovarian cyst with luteinized stromal-cell hyperplasia.BACKGROUND: Hormone production is a characteristic associated with the presence of solid tumors in the ovary. We present a case of a solitary ovarian cyst producing large amounts of testosterone and presenting with rapid onset of hirsutism. CASE: A woman presented approximately 6 months postpartum with irregular menses and rapid onset of hirsutism. Laboratory and radiologic evaluation indicated an ovarian cyst as the source. On removal, the cyst was found to have high amounts of testosterone in its fluid and the surrounding stroma had nests of luteinized cells. CONCLUSION: The chronic anovulation in the postpartum period may have prolonged the lifespan of the luteinized stromal cells, resulting in high levels of circulating testosterone and causing rapid progression of hirsutism.- - - - - - - - - - ranking = 0.66666666666667keywords = hyperplasia (Clic here for more details about this article) |
19/20. Hormonal and genetic analysis of a patient with congenital adrenal hyperplasia.We describe a patient with signs and symptoms of virilization caused by 21-hydroxylase deficiency. The patient, a Hispanic woman, first sought medical attention at age 24, when she presented to a medical clinic with an uncomplicated urinary tract infection. At that time several signs of virilization were noted and she was referred to the endocrinology clinic. Evaluation revealed temporal balding, hyperpigmentation, acne, absent breast development, a muscular habitus, and clitoromegaly. Radiological studies revealed bilaterally enlarged adrenal glands and ovaries. Laboratory evaluation revealed markedly increased concentrations of 17-hydroxyprogesterone, androstenedione, and testosterone. The patient was diagnosed with congenital adrenal hyperplasia (CAH) and received hormone therapy. In her sister, encouraged to undergo testing for this autosomal recessive disorder, HLA testing demonstrated that certain haplotypes in this family were associated with CAH. The case highlights key steps in the laboratory diagnosis and genetics of CAH.- - - - - - - - - - ranking = 0.83333333333333keywords = hyperplasia (Clic here for more details about this article) |
20/20. Adrenal incidentalomas: adrenal hemangioma in a patient with congenital adrenal hyperplasia.We describe a case of an adrenal incidentalomas in the setting of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The adrenal mass was shown to be a cavernous hemangioma. Such neoplasms are rare but have the risk of retroperitoneal hemorrhage and may be difficult to differentiate from malignant adrenal tumors. The main consideration brought up by this case was that the simultaneous occurrence of an endocrinologically active disease such as CAH in association with a likely incidentalomas may lead to surgical intervention, due to the impossibility of being certain of its nonsecretory nature. Laparoscopic adrenalectomy allowed safe resection with no morbidity and a short hospitalization.- - - - - - - - - - ranking = 0.83333333333333keywords = hyperplasia (Clic here for more details about this article) |
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