The therapeutic application of the soft contact lenses are popular in ophthalmic use recently. Drug effects of greater magnitude and duration may be achieved by using a soft contact lens soaked with drugs. Using mycon-soft lenses, with different diameter (14.5mm, 12.0mm) and thickness (0.2mm, 0.1mm), soaked in 2.5% hydrocortisone acetate or 2.5% hydrocortisone succinate for 15 minutes and 30 minutes according to the protocol, and elution studies were performed. Elution rate were not influenced by the thickness of contact lens and soaked time in drugs, but it was influenced by the size of contact lens. It showed that increased elution rate in larger contact lens than smaller one. It was suggested that neither hydrocortisone acetate nor hydrocortisone succinate were absorbed by the soft contact lens but were adhered to the lens surface and released from it gradually.
17-Hydroxycorticosteroids* ; Contact Lenses, Hydrophilic* ; Hydrocortisone ; Succinic Acid

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.
17-Hydroxycorticosteroids ; 17-Ketosteroids ; Adrenal Hyperplasia, Congenital* ; Adrenocorticotropic Hormone ; Clitoris ; Female ; Humans ; Hydrocortisone ; Hyperplasia ; Hypertrophy ; Karyotyping ; Korea ; Metrorrhagia ; Pituitary Gland ; Steroid 21-Hydroxylase ; Ultrasonography ; Young Adult

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.
17-Ketosteroids ; Adrenal Glands ; Adrenal Insufficiency ; Biopsy, Needle ; Drug Therapy ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hydroxycorticosteroids ; Plasma ; Rare Diseases ; Tomography, X-Ray Computed ; Tuberculosis*

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.
17-Hydroxycorticosteroids ; 17-Ketosteroids ; Adenoma ; Adrenal Gland Neoplasms ; Adrenal Medulla ; Adrenalectomy ; Age Distribution ; Angiography ; Child ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Drug Therapy ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Necrosis ; Neoplasm Metastasis ; Neuroblastoma ; Pheochromocytoma ; Prognosis ; Recurrence ; Sex Distribution ; Ultrasonography

The adrenocartical carcinoma is a rare tumor with an estimated incidence of 1 case per 1,700,000 population. Despite its rarity, a large number of investigators have studied this neoplasm for the following two reasons. The First is the occasional difficulty of differentiation between careinoma and adenoma at the time of initial surgery, even by histopathologic examination. The other is its unique feature of corticosteroidogenesis. Steroid metabolism of adrenocortieal carcinoma is characterized by its low efficiency of steroid production due to deficiency of steroidogenic enzyme. The deficieney of 11 B-hydroxylase has been indieated in case of adrenoeortical carcinoma by several investigators. In this study, the serum level of cortisol was within normal range, but the serum level of 11-deoxycortisol was 50 times higher than normal. After the removal of tumor, the serum level of ll-deoxycortisol was markedly decm. In conclusion, the results from the this case suggest that measurement of serum 11-deoxy- cortisol may be a useful tool in the diagnosis and follow-up of adrenocortical carcinoma.
Adenoma ; Adrenocortical Carcinoma* ; Cortodoxone ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Incidence ; Metabolism ; Reference Values ; Research Personnel

As a process to study the mechanism of steroid hormones at the molecular level,the activities of lactic dehydrogenase (L.D.) and malic dehydrogenase (M.D.),NAD-linked transhydrogenases, were measured in the testis and the prostate. Ahundred male rabbits were divided into ten group as follows: Group 1: Control Group 2: Estrogen (6,000 units) injected Group 3: Androgen (1,200 unite)injected Group 4: Progesterone (1,200 units) injected Group 5: Hydrocortisone(30 mg) injected Group 6: DOCA (6 mg) injected Group 7: Castration control Group8: Castration and estrogen (6, OOO units) injected Group 9: Castration and androgen (1,200 units) injected Group 10: Castration and progesterone (1,200units) injected Changes in the activities of lactic dehydrogenase and malic dehydrogenase in theorganic tissues by exogenous steroid hormones were carefullyobserved. The lactic dehydrogenase activities were measured by the method of Wroblewski and La Due, and malic dehydrogenase activities by the Bodansky's modification of Porter's method. The results are as follows: 1) The control valueof L.D. activities in the testicular tissue of normal rabbits proved to be 89,400units per ram. The L.D. activities showed an increase up to 110.4 per cent in theestrogen injected group, 179.3 per cent in the androgen injected group and 147.0 per cent in the progesterone injected group, while the administration of hydrocortisone and DOCA decreased the value down to 85.2 per cent and 81.5 per cent, respectively. 2) The control value of M.D. activities in the testicular tissue of the normal rabbits was 23,600 units per gram. The M.D. activities showed an increase upto 111.4 per cent in the estrogen injected group. 191.1 per cent in the androgen injected group, and 156.8 per cent in the progesterone injected group, while the administration of hydrocortisone and DOCA decreased the value down to 85.2 per cent and 81.5 per cent, respectively. 3) In the prostate tissues of non-castrated rabbits, the L.D. activities were estimated normally to be 48,100 units per gram. The administration of sex hormone resulted in raising the activities upto 101.8 per cent in the estrogen injected group, 196.9 per cent in the androgen injected group and 153.9 per cent in the progesterone injected group. But the administration of hydrocortisone and DOCA decreased the value down to 92.5 per cent and 97.1 per cent, respectively. 4) In the prostate tissue of non-castrated rabbits, the control value of M.D. activities proved to be 14,600 unite per gram. The M.D. activities showed an increase upto 117.8 per cent in the estrogen injected group, 206.8 per cent in the androgen injected group and, 176.7 per cent in the progesterone injected group, while the administration of hydrocortisone and DOCA decreased the value down to 81.9 per cent and 95.2 per cent, respectively. 5) The prostatic L.D. activities were decreased to half the normal two weeks after castration. The administration of sex hormones (i.e., estrogen, androgen and progesterone) acted inclusively upon elevating the level f activities. Androgen, in general, was the most effective to restore the activity to the level of pre-castrated state. 6) The prostatic M.D. activities were also decreased to half the normal two weeks after castration. The administration of sex hormones acted inclusively upon elevating the level ofthe activities. Androgen had a remarkable effect in elevating the M.D. activities, which showed twice the precastration level. In this study, it is concluded that L.D. and M.D. activities are present in the testis and the prostate. They are induced and activated by the administration of sex hormones, of which androgen has the most remarkable effect, and estrogen and progesteronehave less effect, while hydrocortisone and DOCA are ineffective in some cases orinhibitory in others.
Castration* ; Desoxycorticosterone Acetate ; Estrogens ; Gonadal Steroid Hormones ; Humans ; Hydrocortisone ; Malate Dehydrogenase* ; Male* ; Oxidoreductases ; Progesterone ; Prostate* ; Rabbits* ; Testis*

Congenital lipoid adrenal hyperplasia is the rarest type among salt losing types of congenital adrenal hyperplasia. The defect of this disorder is in the cholesterol side chain cleavage enzyme(P450SCC)which converts cholesterol to pregnenolone. W experienced a case of 20,22 desmolse deficiency in a 21-day old phenotypically female who was admitted to our hospital due to lethargy and dark skin pigmentation. The characteristic findings were decreased serum cortisol, aldosterone, testosterone, increased ACTH. The ACTH and hCG stimulation test were performed and there were no response. The sex chromosomal analysis showed made XY. One year later after hormone therapy, growth and development are normal.
Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Aldosterone ; Cholesterol ; Female ; Growth and Development ; Humans ; Hydrocortisone ; Hyperplasia* ; Lethargy ; Pregnenolone ; Skin Pigmentation ; Testosterone

Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

Female pseudohermaphroditism due to adrenogenital syndrome is a condition in which individuals with a 46XX karyotype, negative H-Y antigen, normal mullerian duct derivatives, and a lack of development of w lffian duct structures differentiate partially as phenotypic males. They usually manifest masculinization of the external genitalia as a result of excess endogenous androgens. Most female pseudohermaphrodities have one of the types of congenital virilizing adrenal hyperplasia. Adrenogenital syndrome is inborn errors transmitted by autosomal recessive genes and may be due to defects in any of the enzymic steps in the biosynthesis of cortisol. Most affected individuals have a failure of 21-hydroxylation which prevents the conversion of 17 alpha-hydroxyprogesterone to 11-deoxycortisol. Such a defect in 21-hydroxylase leads to excessive production of adrenal androgens causing virilization. The treatment is early endocrinologic support and surgical reconstruction. There are some considerations in surgical repairs including normal sized clitoris with adequate erogenous sensation, sufficiently wide vaginal introitus and normal aesthetic appearance of the external genitalia for her normalized life as a female. We have experienced four cases of female pseudohermaphroditism due to adrenogenital syndrome. In two cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty with mons pubis augmentation, vaginoplasty with posterior perineal flap and urethral reconstruction. In the other cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty and vaginoplasty There was no specific operative complication and the result of the correction was satisfactory.
17-alpha-Hydroxyprogesterone ; 46, XX Disorders of Sex Development* ; Adrenogenital Syndrome* ; Androgens ; Clitoris ; Cortodoxone ; Female* ; Genes, Recessive ; Genitalia ; H-Y Antigen ; Humans ; Hydrocortisone ; Hyperplasia ; Karyotype ; Male ; Sensation ; Steroid 21-Hydroxylase ; Virilism

The single enzyme P-450c17 hydroxylase catalyzes the 17a-hydroxylation of both pregnenolone and progesterone and the side-chain cleavage of 17a-hydroxypregnenolone and 17a-hydroxypro- gesterone to dehydroepiandrosterone and androstenedione. This enzyme is located in the endoplasmic reticulum and consists of a P-450c17 and a specific flavoprotein NADPH-cytochrome P-450 reductase. The clinical picture and hormonal pattern in 17a-hydroxylase deficiency have been consistent in both genotypic sexes with hypergonadotropic hypogonadism in whom the virtual absence of gonadal steroids results in a female phenotype with primary amenorrhea and pseudohermaphro- ditism in the male and underdeveloped secondary sex characteristics and hypermineralocorticoidism with hypertension, hypokalemia, suppressed renin-angiotensin system and extremely reduced aldo-sterone production. A 17-year-old girl visited endocrine clinic because of amenorrhea, absence of pubic and axillary hair, and hypertension. she had elevated levels of serum corticosterone, deoxycorticosterone(DOC), 18-hydroxycorticosterone(18-OHB). Stumulation with ACTH effected minimal increase in the elevated steroids and the ACTH-stimulated 18-OHB to aldosterone ratio was more than 280. These hormonal patterns appear to be homozygote in 17a-hydroxylase deficiency.
Adolescent ; Adrenocorticotropic Hormone ; Aldosterone ; Amenorrhea ; Androstenedione ; Corticosterone ; Dehydroepiandrosterone ; Endoplasmic Reticulum ; Female ; Flavoproteins ; Gonads ; Hair ; Homozygote ; Humans ; Hypertension ; Hypogonadism ; Hypokalemia ; Male ; Oxidoreductases ; Phenotype ; Pregnenolone ; Progesterone ; Renin-Angiotensin System ; Sex Characteristics ; Steroids