Case Of Adrenogenital Syndrome Research Articles

A case of adrenogenital syndrome caused by giant adrenal cortical adenoma and literature review

目的 报道本院1例巨大肾上腺皮质腺瘤引起性征异常症病例，并探讨此类疾病的临床表现、诊断及治疗方法。 方法 巨大肾上腺皮质腺瘤伴性征异常症1例，结合国内外相关文献进行回顾性分析。 结果 本例手术成功，术后复查支持雄激素异常分泌来源于巨大肾上皮质腺瘤。 结论 肾上腺皮质腺瘤伴性征异常症以女性多见，多表现为女性患者男性化及男性患者性早熟；超声、CT等检查可协助诊断并评估肿瘤性质；早期诊断和手术为治疗本病的关键，术后应长期随访并定期复查。

POSSIBILITIES AND THERAPY LIMITATIONS IN A CASE OF ADRENOGENITAL SYNDROME

Introduction. The adrenogenital syndrome with neonatal onset can manifest by repeated vomiting, dehydration and severe hydro-electrolytic disequilibria. The diagnostic approach, the metabolic re-equilibration and the maintenance of the electrolytic balance in normal ranges by chronic treatment can be associated with difficulties. Objective. We present a case of adrenogenital syndrome with severe neonatal onset, evolution accompanied by different complications, but with a prognosis improved by the adequate chronic treatment. Material and method. A male child with small birth weight, was admitted in the Pediatrics Clinic I, Targu-Mures, at the age of 3 weeks, with a severe clinic-biological syndrome by chronic vomiting and severe dehydration, and apparent macrogenitosomia. We performed a wide spectrum of investigations in acute phase, but also afterwards for the elaboration of the differential diagnosis. Results. We identified persistent hyponatremia and hypopotassemia, an increased level of 17-OH progesterone (44.96 ng/ml) and the cortisol under the normal limit (3 ug/dl). We discovered significantly increased concentrations of ACTH (103 pg/ml in the morning) and total serum cholesterol (186.72 ng/dl). The macromolecular analysis pointed out the heterozygote genotype for the following mutations: 12G, R356W, P453S. The adequate chronic treatment with cortisone improved the prognosis; at the age of 1 years and 5 months, he presents an adequate height, weight and psychomotor development. Conclusions. We fulfilled the conditions of the diagnosis of adrenogenital syndrome with neonatal onset in a heterozygote child. The small age the uncharacteristic symptomatology can be a burden for establishing an early diagnosis of adrenogenital syndrome.

Syndrome of hyperandrogenism and thyroid status in women

The article presents a case of adrenogenital syndrome in women, accompanied by a rare manifestation – galactorrhea. A version of the pathogenesis of galactorrhea and a possibility of pathogenetic therapy are discussed.

Value of laboratory parameters in androgenization in the female with special reference to the "free androgen index"

The degree of androgynism (A) in women can be assessed via clinical data and by determining the levels of testosterone (T), dehydroepiandrosterone sulfate (DHEA-S) and testosterone binding globulin (TBG) in the blood. The determination of the "free androgen index" (FAI), the ratio of total T and TBG, helpful in estimating the level of free T in serum, conveys valuable additional information: in case of FAI below 1, a mild kind of A without elevation of free testosterone can be assumed. However, severe A with elevation of free testosterone and absolute hyperandrogynism is associated with FAI values above 1. In 63 women with signs of A the parameters mentioned above and the FAI were used to determine the degree of severity of A. These data were compared with the corresponding data of a control group. It could be shown that in women whose A was due to idiopathic hirsutism the increased level of bonded T played a causal role. The mean FAI in this group was 0.41. In contrast, in women with POC syndrome and androgynous cycle disturbances, the absolute hyperandrogynism with elevation of free T levels predominated. In these groups the mean FAI was above 1. However, women with male pattern bolding as single sign of A did not exhibit any significant difference in comparison to the control group. In this disease, the increased response of androgen receptors in the skin despite normal androgen levels seems to play a causal role. Two cases of androgen producing ovarial tumours and one case of adrenogenital syndrome were analysed separately.

A Successful Pregnancy and Delivery on the Case of Adrenogenital Syndrome with Primary Amenorrhea

A Successful Pregnancy and Delivery on the Case of Adrenogenital Syndrome with Primary Amenorrhea

The diagnostic value of adrenal venography

Adrenal venography has been performed in 19 cases including 4 cases of Cushing's syndrome, 5 cases of primary aldosteronism, 2 cases of adrenogenital syndrome, 2 cases of retroperitoneal tumor, 3 cases of hypertension, and others.Adrenal tumors were roentgenographically identified in 2 cases of Cushing's syndrome and 4 cases of primary aldosteronism and a case of adrenogenital syndrome, and hyperplasia in a case of Cushing's syndrome, a case of Cushing's disease and a case of adrenogenital syndrome. The diagnostic accuracy of adrenal venography was confirmed by surgical exploration in 10 cases except a case of primary aldosteronism in which venography was misleading due to a poor X-ray condition. The smallest tumor diagnosed was 1.8cm in diameter. There were no serious complications during and after this examination.In the conventional perirenal gas insufflation combined with tomography, the normal adrenal gland, adrenal hyperplasia and small tumor less than 2cm in diameter would only show their dim outline. In addition, that a soft tissue mass in suprarenal space sometimes assumes lesions of the adrenal gland on the retroperitoneal peumogram was confirmed by surgical scrutiny.Adrenal venography is, at present, the most accurate roentgenographic method for assessing the adrenal lesions except pheochromocytoma and differentiating a retroperitoneal tumor from an adrenal tumor.

Adreno-genital syndrome.

Two cases of adrenogenital syndrome are presented. Case 1 was a 2-month-old female infant with the salt-losing form of C21-hydroxy-lase deficiency. She died after three bouts of generalised sepsis. Postmortem was performed. Case 2 was a 5-year old girl with the nonsaltlosing from of C21/hydroxylase deficiency. The problems in diagnosis and treatment are discussed.

Clinical Endocrinology: Vol. IV. Adrenal Cortex and Medulla

This monograph was written to meet the needs of the medical student and the internist for both an understanding of the physiology of the adrenal cortex and medulla and an appreciation of the effects of their hormones at various levels of organization—from the cell to man. Since the price places it beyond the reach of any but the most affluent medical student, it is fair to ask if it is an adequate monograph for the internist. The author has exhaustively documented each clinical syndrome and has constructed tables with the pertinent features of each and extensive references. Unfortunately, these references are not critically evaluated, e.g., Table 22-1, which purports to present the cases of adrenogenital syndrome in adults with adrenal hyperplasia. Fourteen of the 17 patients were studied before 1950, and 17-ketosteroids were incorrect in four. The discussions of the physiological effects of the hormones and the clinical syndromes are

Clinical applications of urinary ACTH assay.

ACTH in urine, extracted by benzoic acid adsorption and assayed by adrenal ascorbic acid depletion in hypophysectomized rats, showed a definite elevation upon administration of an 11-β-hydroxylase inhibitor, SU-8874 (2-methyl-1,2-bispyridyl-1-propanone hydrochloride), in 3 normal subjects and in 3 patients with intact pituitaries, whereas 4 patients with hypopituitarism failed to show an elevation. Elevated urinary ACTH levels were found in 4 cases of adrenogenital syndrome and in 3 cases of Cushing's syndrome due to bilateral adrenal hyperplasia. While increased urinary ACTH was suppressed by oral administration of 20 mg or less of hydrocortisone in 3 cases of Addison's disease and in one case of carcinoma of the prostate after bilateral adrenalectomy, more than 30 mg was required to suppress the increased urinary ACTH in Cushing's syndrome owing to bilateral adrenal hyperplasia.

Study of adrenogenital syndrome. I. Clinical investigation of adrenogenital syndrome

We reported the two cases of adrenogenital syndrome with the congenital adrenal hyperplasia and discussed this disease.

A study on the metabolism of calcium in the hyperadrenocortical syndrome.

ABSTRACT An investigation of the calcium metabolism has been carried out in 16 patients with Cushing's syndrome of various types and degrees, before and after either uni- or bilateral adrenalectomy, or implantation of the pituitary gland with 90 Y, and in 2 cases of adrenogenital syndrome. In all the cases of Cushing's syndrome a disturbance of calcium metabolism was observed. A negative trend of calcium metabolism was frequently found, both in cases with and without radiographic signs of osteoporosis. This was revealed by an increase of the urinary calcium, both spontaneously and after a calcium tolerance test. However, an opposite metabolic condition was found in 3 cases. A rapid change of the calcium metabolism, consisting of a marked decrease of both spontaneous and induced calciuria to values below the normal range, together with a normalisation of the hormonal picture was always recorded after the surgical treatment. A normal or positive calcium metabolism was found in the 2 cases of adrenogenital syndrome. The relationship between the metabolic and hormonal picture is thoroughly discussed.

Recognition and treatment of adrenal crises in the newborn infant

Summary<ul><li>1.Four cases of adrenogenital syndrome, salt-losing type, are presented. They demonstrate the clinical picture of adrenal crisis in the neonatal infant.</li><li>2.The early diagnosis and treatment are of utmost importance for the survival of these infants. All females with the adrenogenital syndrome and males suspected of having adrenogenital syndrome should be observed in the hospital for the first two weeks of life in order to determine the presence or absence of electrolyte imbalance.</li><li>3.Typically, the initial crisis occurs on the seventh day of life. The crisis is characterized by a lethargic infant with a gray pallor, vomiting, weight loss, hypotension, hyperkalemia, hyponatremia, hypochloremia, and dehydration. The excretions of pregnanetriol and 17-ketosteroids in the urine are elevated.</li><li>4.In each patient, the clinical impression is much more important than the confirmatory laboratory results in making a presumptive diagnosis and initiating prompt lifesaving treatment.</li><li>5.Treatment early in life for infants with adrenal crisis includes supplementary salt administration, DOCA given intramuscularly, and cortisone given intramuscularly. Following the initial control of the patient, he should be referred for long-term care to a physician familiar with this particular problem.</li><li>6.The patients reported here took from 40 to 50 days to become stabilized on the medications after initial control.</li><li>7.Hypertension occurred with each patient who was given DOCA, but the hypertension was not severe and did not lead to any complications. When the DOCA was reduced, the hypertension disappeared.</li><li>8.Premature withdrawal of DOCA from two patients was followed by adrenal crisis and death. Therefore, it is advisable to continue the administration of DOCA in spite of apparent electrolyte control and clinical stabilization with salt and cortisone. The maintenance of a normal weight and serum sodium level does not preclude the occurrence of a fatal hyperkalemia in these patients.</li></ul>

A case of adrenogenital syndrome (adrenal pseudohermaphrodism) with subsequent pregnancy

A case of adrenogenital syndrome (adrenal pseudohermaphrodism) with subsequent pregnancy

ADRENOGENITAL SYNDROME WITH ASSOCIATED EPISODES OF HYPOGLYCEMIA

THE adrenogenital syndrome is a type of hyperadrenocorticism characterized by excess production of adrenal androgens. When it occurs congenitally it is due to hyperplasia of the adrenal glands, and is manifested as pseudohermaphrodism in females and macrogenitosomia praecox in males. Wilkins (1) has recently described the syndrome and collected 99 such cases from the literature and his own observations. A postnatal variety of adrenogenital syndrome also occurs, and is caused usually by adrenal tumors. Forty-one cases are mentioned by Wilkins (1). Wilkins, Fleischmann, and Howard (2) and Butler, Ross and Talbot (3) in 1939 first described cases of adrenogenital syndrome in which Addisonian symptoms of acute adrenal insufficiency occurred. Zuelzer and Blum (4) recently collected 17 such cases from the literature and added 4 of their own. Since their review, 2 cases have been reported by DeJong and Schaberg (5), 1 case by Chenoweth (6), and 1 case by the New York Hospital group (7). At least 3 and possibly m...