A Case of Congenital Adrenal Hyperplasia due to 11beta-Hydroxylase Deficiency / 대한내분비학회지

Congenital adrenal hyperplasia refers to a group of autosomal recessive disorders that is defective in the synthesis of cortisol. The enzymes most often affected are 21-hydroxylase and 11beta hydroxylase. The low levels of cortisol stimulate the pituitary gland to release ACTH. Chronic elevation of the ACTH level causes bilateral adrenal hyperplasia and a secondary increase in androgen formation. We examined a 19 year-old woman presented with clitoral hypertrophy and vaginal spotting. The subjects basal level of serum cortisol was low, but the serum levels of ACTH, 17a-hydroxyprogesterone, deoxy-corticosterone were elevated. The urinary excretions of 17-ketosteroids and 17-hydroxycorticosteroids were also increased. The karyotyping study and transrectal ultrasonography showed normal findings. The patient underwent clitoris reduction surgery and received hydrocortisone. To the best of our knowledge, this is the first case of 11beta-Hydroxylase deficiency in Korea.

Impact of feeding ethanolic extracts of Achyranthes aspera Linn. on reproductive functions in male rats.

Feeding 50% ethanolic extract of A. aspera to male rats resulted in reduced sperm counts, weight of epididymis, serum level of testosterone and testicular activity of 3beta-hydroxysteroid dehydrogenase, while motility of the sperm and activity of the HMG CoA reductase were not affected. Cholesterol level in the testis, incorporation of labelled acetate into cholesterol, 17-ketosteroids in urine and hepatic and fecal bile acids were increased. The results suggest that ethanolic extract of A. aspera caused reproductive toxicity in male rats and the action may be by suppressing the synthesis of androgen.

Clinical Features of Adrenocortical Neoplasms

PURPOSE: Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of nonfunctioning tumors is not easy and the diagnosis may be delayed. Benign tumors can be cured by complete surgical excision, but malignant cases have poor response to treatment and worse prognosis. Early diagnosis and proper management are very important because of the large proportion of functioning malignant tumors in children. We report clinical features of adrenocortical tumors in children that may be of help in the early detection, proper management, and assessment of prognosis of patients. METHODS: We reviewed the clinical characteristics of 14 cases of adrenocortical tumors, among 85 children diagnosed with adrenal tumors, who visited the Severance Hospital, College of Medicine, Yonsei University, from January 1970 to July 1996. RESULTS: 85 Patients were diagnosed with adrenal tumors. Among them, 71 cases 83.5%) were tumors of the adrenal medulla, neuroblastoma and pheochromocytoma, and 14 cases (16.5%) were adrenocortical tumors, consisting of 5 cases of adenoma, 7 cases of carcinoma, and 2 cases unspecified. The age distribution ranged from 16 months to 14 years of age, and the mean was 5 years & 11 months (median 4 years & 2 months). Sex distribution revealed a male to female ratio of 1:1.33. The left to right ratio was 3.7:1, showing a left side predominance. 13 Cases (92.9%) were functioning tumors: 12 cases (92.3%) had clinical evidence of androgen excess, among which 6 cases (46.2%) were associated with Cushing's syndrome, and 1 case was compatible with primary aldosteronism. Serum cortisol, urinary 17-ketosteroids and 17-hydroxycorticosteroids concentrations were measured in 11 cases and urinary concentrations of 17-ketosteroids were elevated in all 11 cases (100%), while 17-hydroxycorticosteroids were elevated in 4 cases (36.4%). Abnormalities of serum cortisol were found in all cases except 1: serum cortisol concentrations were abnormally elevated in 5 cases (45.5%), and the remainder (5 cases, 45.5%) showed loss of diurnal variation. Dexamethasone suppression test was performed in 9 cases, and all (100%) showed no suppression. Preoperative radiologic studies included abdominal sonograms, CT or MRI scans, and angiography. Histology showed carcinomas to be bigger and heavier than adenomas, and microscopically carcinomas had necrosis, calcifications, and invasions of vessels. Distant metastases were found in 4 cases (12.7%). Adrenalectomy with complete surgical excision was performed in 12 cases. Long-term follow-up was possible in 10 patients after operation: 3 patients initially diagnosed with adenoma survived without tumor recurrence over a year, and among 6 carcinoma patients, 4 expired within a year, and 2 survived, with one patient currently undergoing postoperative chemotherapy. Of the 10 patients currently under going follow-up, one patient was initially diagnosed with a histologically unspecified tumor, and has survived 4 years after operation. CONCLUSIONS: When adrenocortical neoplasms are suspected by clinical symptoms and laboratory findings, abdominal ultrasonogram, CT or MRI scans must be performed immediately. Early detection and proper management are important for better prognosis, but are often delayed in the majority of cases. Ultimately, pediatricians need to be familiar with clinical characteristics and laboratory findings of adrenocortical tumors, bearing in mind the possibility of diagnosis in children.

Preliminary study of androgen, thyroid & adrenal status in alcoholic men during deaddiction.

To evaluate the status of the testes, thyroid, and adrenals in male alcoholics during the period of voluntary abstinence and therapy, chronic male drinkers undergoing a 4 wk inpatient deaddiction programme in a social hospital were recruited. Levels of a few serum and urinary hormones/metabolites viz., serum testosterone, total triiodothyronine (T3) and thyroxine (T4) and urinary total 17-ketosteroids (17-KS), estrone, estradiol, and 17-hydroxy corticosteroids (17-OHCS) were assessed in alcoholics thrice during the treatment programme at hospital i.e., on the zero (day of admission), 10th, and 20th day and compared to those of non-alcoholic controls. Alcoholics registered elevated serum total T3, and reduced total T4 and testosterone levels at admission, which persisted even after 20 days of the rehabilitative programme. Markedly high urinary levels of total 17-KS, estrone, and 17-OHCS were observed on zero day of admission. Urinary estrone and 17-OHCS, unlike total 17-KS, showed a trend to return to the normal range during the 20 days period. Urinary estradiol levels, however, recorded no significant alteration. The results of this preliminary study are suggestive of alcohol-induced perturbations on the functional integrity of the testes, thyroid, and adrenal in male alcohol addicts, wherein 20 days period of total alcohol abstinence and rehabilitative programme failed to reverse alcohol-induced hypoandrogenization and altered thyroidal status, but only partially restored certain biochemical events associated with the excretion of steroid metabolites.

Hirsutismo: experiencia en diagnóstico y tratamiento en el período 1970-1995 / Hirsutism: experience in diagnosis and treatment in the period 1970-1995

En la anamnesis de la hirsuta, destaca el inicio peripuberal del hirsutismo y en el examen físico la importancia del vello pubiano que aporta el 40 por ciento del score total. Del 74 por ciento de hirsutas hiperandrogénicas, el 34 por ciento poseen elevación exclusiva de la testosterona, el 24 por ciento de la dehidroepiandrosterona sulfato y el 42 por ciento un alza de ambas. El alto porcentaje de dehidroepiandrosterona sulfato aumentada, sola o junto a testosterona, sugiere un compromiso suprarrenal en la etiología del hirsutismo, respaldado por un 50 por ciento de prueba de estimulación suprarrenal con hiperrespuesta de este andrógeno, en hirsutas y en pacientes con síndrome de ovario poliquístico. El alza de la 17 hidroxiprogesterona basal, obliga a realizar prueba de estimulación cob ACTH para descartar su origen ov+arico y confirmar el déficit de la 21 hidroxilasa. Un 5 por ciento de las hirsutas tenían acantosis nigricans con resistencia insulínica y alteraciones lipídicas. Se analiza el tratamiento con antiandrógenos, ciproterona y espironolactona y la frenación con glucocorticoides

Male gonadal function in Hodgkin's disease before and after treatment.

Twenty eight histologically confirmed cases of Hodgkin's disease were evaluates with regard to libido, sperm count, FSH, LH and urinary ketosteroid levels, before and during different stages of chemotherapy along with testicular biopsy on 16 cases. Decrease in libido during therapy improved following treatment, 50% cases who were oligospermic before treatment became azospermic, Serum FSH levels increased significantly (p 0.001) during the course of treatment. There was no significant change in LH and urinary ketosteroid levels (p 0.05). Testicular biopsy, normal initially, showed germ cell aplasia and absence of spermatogenesis after therapy. Drug induced testicular change leads to sterility.

Effect of chronic toxicity of Kat [catha edulis] extract on some physiological parameters of male rats

The effects of chronic administration of kat [Calha edulis] on several physiological and biochemical parameters of the male rats [Rattus norvegicus] were studied. Cholesterol and ascorbic acid contents of the adrenals were found to be gradually declined as a result of kat-administration. Neurotransmitters in the brain appeared to be affected by kat-administration. Norepinephrine content was significantly decreased and acetylcholine significantly increased in kat-treated than in control rats. However, no significant changes in serotonin level were found. Kat administration also affected the plasma testosterone level; an initial rise occurred during the first four months probably as a result of the stimulating effect of kat, then a general decrease was observed until the termination of the experiment. On the other hand, kat-treated rats appeared to have no significant changes in the urinary 17-ketosteroid level throughout the experiment

A Case of Bilateral Adrenal Masses due to Tuberculosis / 대한비뇨기과학회지

Bilateral adrenal tuberculosis is a rare disease and often occurs bilaterally. We report a case of bilateral adrenal masses due to tuberculosis with adrenal insufficiency. The patient was a 39- year-old man who had complained of intermittent pain of right upper quadrant and general weakness. The plasma levels of cortisol and catecholamine were normal. The levels of 24-hour urinary catecholamine and VMA were also normal. But the levels of 24-hour urinary 17- hydroxycorticosteroids and 17-ketosteroids were reduced. Abdominal CT showed about 6.5 x4.8 x 5.4cm sized left adrenal mass and 4.0 x 2.8 x 3.6cm sized right adrenal mass with calcification. The result of sono-guided percutaneous needle biopsy was adrenal tuberculosis. The patient was treated with antituberculous chemotherapy and hormonal replacement. But the masses are unchanged during 5-months follow-up.

The Effects of Continuous Epidural Fentanyl / Bupivacaine Mixtures on Analgesia and Pulmonary Function after Thoracotomy / 대한마취과학회지

Patients undergoing thoracotomy experience severe postoperative pain and marked respiratory impairment. Analgesics(narcotics or loeal anesthetics) administered via epidural catheter in epidural space have been shown to provide postoperative analgesia and improve respiratory mechanics after thoracotomy. Several different methods have been utilized in an attempt to reduce pain and pulmonary mechanics after thoracotomy. These include epidural blocks using local anesthetics, epidural narcotics, ketamine, steroid, and clonidine. These methods have been shown to provide pain relief with relative preservation of lung volumes in the postoperative period, but have disadvantages. Especially epidural local anesthetics may cause hypotension and motor blockade of lower extremities, and epidural narcotics may cause pruritus, nausea and vomiting, urinary retension and respiratory depression. In an attempt to provide excellent analgesia and improve pulmonary mechanics after thoracotomy and to decrease the side effects associated with the intermittent bolus administration of epidural narcotics or local anesthetics, we performed a study of continuously administered epidural infusion of small concentration of fentanyl combined with low concentration of bupivacaine. Twenty eight patients undergoing thoracotomy were randomized into groups based upon a postoperative pain regimen as indicated: Group I: intermittent intramuscualr injection of nalbuphine 0.2 mg/kg(n=13), Group II: continuous epidural injection of mixtures of 0.2/ bupivacaine and fentanyl 3 ug/ml(n= 15). Two, 8, 24 and 48 hours postoperative, the following indices were measured: visual analogue pain scale, vital capacity, tidal volume, arterial blood gas analysis(pH, PaCo2, PaO2), side effects, and 24 hour urine 17-ketosteroids. The results were as follows: 1) Pain score was evaluated by visual analogue pain scale postoperatively and the pain scores significantly decresed in group II as compaired with those in group L 2) Vital capacity and tidal volume in group II were more improved than group I. 3) There was no difference in arterial blood gas analysis except for decreased PaO2 at 2 hour and 24 hour compared with preoperative value in group L 4) Major complications in group II were two cases of nausea and vomiting, one case of urinary retension, whereas only I patient in group I complained of nausea and vomiting. 5) No significant difference occurred in 24 hour urine l7-ketosteroid at 24 hour and 48 hour postoperatively in group I and group II, which were within normal limits.

Myopia and plasma cortisol.

Estimation of plasma cortisol by fluorometric technique desorbed by Mallingly's was carried out in 56 cases, including 38 cases of myopia (19 cases of simple myopia and 19 cases of degenerative myopia) and 18 normal individuals. Urinary 17-keto steroids/24 hours was also estimated by Zimmermann technique in 12 out of the 56 cases, which include 8 cases of myopia (4 cases of simple myopia and 4 cases of degenerative myopia) and 4 normal individuals as control. Plasma cortisol level is lower in simple and degenerative myopia than in normal subjects, but on statistical analysis the difference was not of much significance. Patients with simple myopia with positive family history showed lower value of plasma cortisol than patients with simple myopia with negative family history, the difference was also statistically insignificant (P-0.1). In degenerative myopia patients, with and without family history, there was very little difference in plasma cortisol level and statistically highly insignificant (P-0.8). Urinary 17 keto steroid/24 hour values are lower in simple and degenerative myopia than in normal subjects.

Atividade endócrina relacionada aos órgäos sexuais na pós-menopausa / Endocrine activity related to sexual organs during postmenopause

A atividade endócrina relacionada com os órgäos sexuais foi avaliada em mulheres menopausadas e pós-menopausadas, antes e depois da ressecçäo cirúrgica bilateral dos ovários. Comparando os valores urinários das gonadotrofinas dos 17-cetoesteróides e estrogênios, comprova-se uma persistente atividade endócrina nos ovários senis

Adreno-cortical function in leprosy.

Adreno cortical function was carried out in 43 cases of leprosy. These cases were further divided into tuberculoid, borderline, lepromatous and Lepra reaction. Serum and urinary electrolyte, urinary 17-Ketosteroid and 17-Ketogenic steroid and plasma cortisol levels were measured to assess the adrenocortical status in these different forms of leprosy. It was observed that these parameters were within normal limit in tuberculoid leprosy except low value of urinary 17-Ketogenic steroid. The borderline and Lepromatous leprosy cases revealed low values of urinary sodium, potassium and 17-Ketogenic steroid and high level of serum potassium. However, the cases of lepra reaction revealed low value of serum and urinary sodium and potassium, urinary 17-Ketogenic steroid. The basal plasma cortisol level was high in this group but it was statistically insignificant.

Microtécnica para la determinación de 17-cetoesteroides urinarios / Microtechnique for the determination of urinary 17-ketosteroids

Se desarrolló una microtécnica para la cuantificación de 17-cetoesteroides en orina de 24 horas. Se procesaron 75 muestras de orina con concentraciones bajas, medias y altas. Al analizar las muestras por la microtécnica se obtiene un ahorro de 66% en reactivos y por ende, en costo por prueba. Se encontró excelente correlación entre el micrométodo y macrotécnica, p <0.001, r = 0.99. Por lo tanto, esta técnica es recomendable por su excelente reproducibilidad y economía

Fraccionamiento de 17-cetoesteróides por cromatografía en capa delgada / Fractionation of 17-ketosteroids by thin layer chromatography

Se presenta un método de fraccionamiento de 17-Cetoesteroides (17-Ce) neutros urinarios, mediante dos cromatografías en capa delgada sucesivas: la primera, sobre sílica gel, separa en forma adecuada androstenodiona (delta 4) etiocoloanoloma (E) y derivados 11 oxigenados (C19 O3); la segunda, sobre óxido de aluminio y doble desarrollo, separa convenientemente dehidroepiandrosterona (DHEA) de adrosterona (A). Para ambas se utiliza el sistema de solventes compuesto por cloruro de metileno: etanol (97:3). Las determinaciones cuantitativas se realizan aplicaciones intra e interensayos por triplicado fueron: para C19 O3 ñ 3,62 y ñ 4,31; E, ñ 4,94 y ñ delta 4, ñ 2,08 y ñ 6,27; DHEA, ñ 6,66 y ñ 7,45; A, ñ 3,00 y ñ 13,26, respectivamente. La exactitud del método oscila entre 92,1 y 106,8%. En un cuadro comparativo se ordenan, además, los valores de referencia del método y los referidos por algunos autores en nuestro páis y en el extranjero. El método es relativamente sencillo y reproducible; requiere de instrumental accesible y logra un fraccionamiento satisfactorio de los 17-Ce más importantes en el manejo del laboratorio clínico

epidemiological study on virus hepatitis in Alexandria II- Liver and renal functions in virus hepatitis

This study was carried on 100 cases [50 males and 50 females] aged 20-40 years, clinically diagnosed as virus hepatitis, admitted to Alexandria Fever Hospital. A control group of 100 healthy persons were chosen. Blood samples were taken for estimation of the following parameters: Hepatitis B surface antigen, serum alanine aminotransferase [ALT or SGPT], serum aminotransferase [AST or SGOT], serum alkaline phosphatase and serum bilirubin. In addition a 24 hours urine specimens were collected for estimation of 17-ketosteroid. The obtained results showed that liver function tests were increased significantly among patients in both groups HBsAg positive and HBsAg negative, while no difference was detected between both groups. The level of urinary 17- ketosteroid was found to be increased in all types of hepatitis

Microbiological transformations of steroids VI Amortization of 19-hydroxyandrosta-4,7-diene-3,17-dione into equilin

Washed mycelia and nongerminating spores of Fusarium solani aromatized 19-hydroxyandrosta-4, 7-diene-3, 17-dione into equilin and equilenin. The transformation was more complex with the mycelia rather than with the other unidentified products were formed early in the transformation. Substrate transforming enzymes appear to be induced in the mycelium and constitutive in the spores

Funçäo pituitária e gravidez a termo na síndrome de Sheehan / Pituitary function and term pregnancy in Sheehan's syndrome

Uma paciente com síndrome de Sheehan (necrose pituitária pós-parto) desenvolveu gravidez a termo, quatro anos após a instalaçäo do hipopituitarismo, na vigência de reposiçäp hormonal tiroidiana e corticosteróide. Testes de estímulo da pituitária demonstraram claramente que o setor gonadotrófico era responsivo, sugerindo que possivelmente as células gonadotróficas teriam sido poupadas, pelo menos parcialmente, o suficiente para que houvesse ovulaçäo espontânea.

HCG test in Egyptian patients with cryptorchidism

Serum testosterone [T] concentration and urine 17 - Ketosteroid [17-KS] were determined under basal conditions and after stimulation by human chorionic gonado trophins [HCG] in thirty-six prepubertal males [10 normal children and 26 cryptorchid patients]. As a function of HCG stimulation, the increase in serum T level was evident in both groups, although the magnitude of rise was higher in control group than cryptorchid boys. No significant differences were obtained between unilateral and bilateral undesecended testis patients at basal level or after of the wide individual variations in testicular response in all subjects studied, yet the test is of value in assessing leydig cell function and in prognosis as regards virilization at puberty