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J Sólyom

Publications and source records attributed to J Sólyom.

At least 19 recordsLinked to original sources
PubMed1992

[Management of congenital adrenal hyperplasia].

This review deals with the current problems in the management of classical 21-hydroxylase deficiency from the fetal life to the puberty. The clinical consequences of 21-hydroxylase deficiency reflect the disordered physiology--impaired secretion of glucocorticoids and mineralocorticoids, and excessive secretion of androgens. Current therapy is intended to correct the disordered physiology by replacing mineralocorticoid and glucocorticoid hormones, thereby reducing the ACTH-driven increase in adrenal androgen secretion. Treated patients should expect a normal life span and reproductive potential. This can be achieved by careful attention to regular measurements of clinical parameters and biochemical indices of control.

Adrenal Cortex Hormones↗
PubMed1992

Urinary excretion of 17-hydroxypregnanolones in patients with different forms of congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency.

To improve diagnostic criteria in different (classical salt-wasting (SW), classical simple virilizing (SV) and non classical late onset (LO)) forms of congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency, we investigated the urinary excretion of 17-hydroxypregnanolones (17OH-PO(5 beta) and (5 alpha)), 15 beta-hydroxypregnanolone(15 beta OH-PO), pregnanetriol(PT) and 11-oxo-pregnanetriol (11-O-PT) compared to hydrocortisone metabolities. During the 1st month of life newborn infants with CAH-SW excreted from barely detectable to very large amounts of 17OH-PO(5 beta), 15 beta OH-PO and PT, and, in 12 of 14 cases, also 11-O-PT in their urines. From the 1st to the 28th day of life, cortisol metabolites were virtually absent in urines of CAH-SW infants. This was in contrast of 36 healthy newborn infants. We measured the excretion of 17OH-PO(5 alpha) in children with CAH of whom 19 patients with CAH-SV had a median 17OH-PO(5 alpha) excretion of 1110 micrograms/day (range: 152-5515). In 21 patients with CAH-LO, median excretion of 17OH-PO(5 alpha) was 294 micrograms/day (range: 66-1273). Besides the conventional metabolites of 17-hydroxyprogesterone (17OH-PO(5 beta), PT and 11-O-PT), no 17OH-PO(5 alpha) was detected in the urines of 14 patients with precocious pubarche, in 14 patients with virilization of unknown origin and in 94 healthy children of comparable age. The ratio of 17OH-PO(5 alpha) to tetrahydrocortisone (THE) discriminated between CAH-SV and CAH-LO from the 1st to the 18th year of age.(ABSTRACT TRUNCATED AT 250 WORDS)

17-alpha-Hydroxypregnenolone↗
PubMed1991

Severe hypoaldosteronism due to corticosterone methyl oxidase type II deficiency in two boys: metabolic and gas chromatography-mass spectrometry studies.

Infection-triggered, life-threatening salt-loss and hyperkalaemia developed in two male infants with wasting, inappropriately low plasma aldosterone concentrations and elevated plasma renin activity. The presumptive diagnosis of a defective terminal step in aldosterone biosynthesis was made by the presence of large amounts of 11-dehydrotetrahydrocorticosterone and its 18-hydroxylated metabolite (18-OH-THA), free 18-hydroxycorticosterone (18-OH-B) and 18-hydroxytetrahydrocorticosterone in the urine of both patients. The diagnosis of corticosterone methyl oxidase type II (CMO II) deficiency was confirmed by an elevated urinary 18-OH-THA to tetrahydroaldosterone ratio in one boy and by an elevated plasma 18-OH-B to aldosterone ratio in the other boy. Unknown steroids responsible for the salt-loss were not identified. Sodium supplementation but not short-term high dose oral 9 alpha-fluorcortisol (FF) normalized the hyponatraemia in one patient, in whom sodium (Na+)/potassium (K+) co-transport was decreased. Both patients eventually received long-term FF treatment to prevent impairment of longitudinal growth caused by chronic salt-loss. The diagnosis of CMO II deficiency should always be confirmed by elevated precursor-product ratios in urine or plasma, using radioimmunoassays with prior chromatographic separation. Metabolic studies as the short-term response of serum Na+ to high dose FF may not be helpful in differentiating aldosterone biosynthetic defects from end-organ resistance to mineralocorticoids.

Cortisone↗
PubMed1991

Age of appearance of circadian rhythm in blood 17-hydroxyprogesterone in 21-hydroxylase deficiency.

Sixty-one daily profiles of blood-spot 17-hydroxyprogesterone derived from patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency were analysed to determine the age of appearance of circadian rhythm in hypothalamo-pituitary-adrenal activity. The results revealed that typical circadian adrenocortical rhythm is established at approximately three months after birth. In conclusion, the maturation of supra-hypothalamic brain structure proceeds normally also in humans with constant low level of cortisol in blood.

17-alpha-Hydroxyprogesterone↗
PubMed1990

Blood-spot 17-hydroxyprogesterone daily profiles in infants with congenital adrenal hyperplasia.

To define the optimum blood levels of 17-hydroxyprogesterone, the hormonal effects of glucocorticoid treatment were studied during the neonatal period and infancy in 20 patients with 21-hydroxylase deficiency. Repeated daily profiles of blood spot 17-hydroxyprogesterone were used to monitor therapy and these data were related to serum concentrations of testosterone. A wide fluctuation of blood 17-hydroxyprogesterone levels was observed in patients with a mean daily value higher than 150 nmol/l indicating poor control. Serum testosterone levels decreased into normal range in female patients with blood-spot 17-hydroxy-progesterone levels between 50 and 150 nmol/l. Daily profiles with mean values less than 50 nmol/l showed low magnitude of circadian variation suggesting overtreatment. We conclude that the daily profile of blood-spot 17-hydroxyprogesterone is a practical alternative in hormonal monitoring of infants treated for congenital adrenal hyperplasia.

17-alpha-Hydroxyprogesterone↗
PubMed1989

[Screening for congenital adrenal hyperplasia in Hungary].

Measurement of blood-spot 17-hydroxyprogesterone (17-OHP) concentration was used to identify cases of congenital adrenal hyperplasia (CAH) among patients with inappropriate virilization and/or salt wasting. Between 1978 to 1986 61 cases with 21-hydroxylase deficiency among 707 patients (278 newborns, 204 infants and 225 children) were identified. The incidence of classical CAH was calculated for a seven year prospective trial period using the blood-spot 17-OHP method in selective screening. There were 38 salt-losers and 14 simple virilizers in 968,303 live births giving an incidence of 1 in 18,000 for CAH in the Hungarian population. The use of a central laboratory facility to measure the blood-spot 17-OHP concentrations is proposed as a valuable initial method to investigate patients at risk for CAH in countries where blood steroid assays are not readily available in hospitals.

17-Hydroxycorticosteroids↗
PubMed1989

Blood-spot 17-hydroxyprogesterone in nonclassical 21-hydroxylase deficiency.

The value of blood-spot 17-hydroxyprogesterone (17-OHP) measurements in selective screening for non-classical 21-hydroxylase deficiency (NC 21-OHD) among female patients with postnatal virilization was studied. Early morning basal blood-spot and serum samples, and post-ACTH serum samples for 17-OHP measurement were collected from twenty-seven patients with precocious pubarche, hirsutism and/or menstrual abnormalities (age: 3 to 17 years). Using a two-step extraction method for blood-spot 17-OHP measurements of all the nine patients with NC 21-OHD showed higher values than the eighteen patients without 21-OHD. Blood-spot 17-OHP values of patients with NC 21-OHD showed a diurnal rhythm with pathological high levels between 0500 and 0900 h, and normal or slightly elevated levels between 1700 and 2100 h. In conclusion, early morning blood-spot 17-OHP measurement is a useful method to detect NC 21-OHD in the peripubertal period.

Adolescent↗
PubMed1989

Value of selective screening for congenital adrenal hyperplasia in Hungary.

Measurement of the 17-hydroxyprogesterone concentration in blood spots was used to identify cases of congenital adrenal hyperplasia among patients with inappropriate virilisation, or salt wasting, or both. Between 1978 and 1986 61 were identified among 707 patients (278 neonates, 204 infants, and 225 children). The incidence of classic congenital adrenal hyperplasia was calculated for a seven year prospective trial period using the blood spot 17-hydroxyprogesterone method in selective screening. There were 38 salt losers and 14 simple virilisers in 968,303 live births, an incidence of congenital adrenal hyperplasia of 1:18,000 in the Hungarian population. Selective screening led to earlier diagnosis of congenital adrenal hyperplasia and a pronounced decrease in mortality. A central laboratory to measure the blood spot 17-hydroxyprogesterone concentrations is valuable for the investigation of patients at risk for congenital adrenal hyperplasia in countries where blood steroid assays are not readily available.

17-alpha-Hydroxyprogesterone↗
PubMed1987

Detection of late-onset adrenal hyperplasia in girls with peripubertal virilization.

We investigated the value of serum levels of adrenal steroids (dehydroepiandrosterone sulphate, testosterone, 17-hydroxyprogesterone, cortisol) in the identification in peripubertal females with late-onset congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. Among 68 females (age 3-18 years) with virilization in childhood, peripubertally or postpubertally, we selected 21 girls for an ACTH test by measurement of basal blood-spot or serum 17-hydroxyprogesterone (17-OHP) levels. Eight of 21 patients had supranormal post-ACTH serum 17-OHP concentration (57-153 nmol/l) with low normal cortisol concentration. All of them had supranormal basal and post-ACTH 17-OHP to cortisol ratios. These data show a relatively high incidence (about 12%) of mild 21-hydroxylase deficiency among prepubertal and adolescent girls with virilization. It is concluded that the first step in the investigation of peripubertally virilized girls should be the determination of serum 17-OHP and cortisol. Patients with basal morning 17-OHP concentration and 17-OHP to cortisol ratio above reference range should be given an ACTH test.

17-alpha-Hydroxyprogesterone↗
PubMed1979

A method for identification and follow-up of patients with a steroid-21-hydroxylase deficiency.

A method is described for the determination of 17 alpha-hydroxyprogesterone from blood samples obtained by heel prick and dried on filter paper. Discs (10 mm diameter) were cut from the filter paper and extracted in the assay tubes with 1 ml of a methanol/diethyl ether/ethyl acetate (50 :45 :5, v/v) solvent mixture. Antibody, tritium-labelled tracer and dextran-coated charcoal were added to assay tubes using a multichannel dispenser. The approach used permits one technician to analyze two series, each of 60 duplicate samples, within one working day. Thus the method is applicable for the centralized screening of suspected cases, while emergency samples may be analyzed at the same time within 4 h. Comparisons with a highly specific but more elaborate technique for the determination of blood 17 alpha-hydroxyprogesterone showed a correlation coefficient of 0.99, and the regression equation for the present method (y) against the established method (x) was y = 1.07x + 2.72. The calculated upper reference limit (mean +/- S.D.) for 17 alpha-hydroxyprogesterone in healthy infants from two days to eight years of age of 7.5 ng/ml of serum.

Adrenal Glands↗
PubMed1979

Plasma renin activity and mineralocorticoid replacement therapy in congenital adrenal hyperplasia.

Plasma renin activity and serum concentrations of sodium and potassium were repeatedly measured in seven children with congenital adrenal hyperplasia due to steroid-21-hydroxylase deficiency, to monitor the sodium balance during treatment. Infants with the salt-losing form had high plasma renin activity levels 5--11 months after subcutaneous implantation of a pellet containing 100 mg deoxycorticosterone acetate. These elevated plasma renin activity levels were suppressed to the normal range by repeated implantation of DOCA pellet. Moderately elevated values of plasma renin activity in older salt-losers normalized after increasing the dietary sodium intake. Plasma renin activity level has superiority over serum electrolyte concentrations as an index of mineralocorticoid deficiency.

Adolescent↗
PubMed1975

Prolonged oral administration of potassium upon aldosterone biosynthesis by rat glomerulosa tissue in vitro.

Steroid production rate of adrenals derived from rats drinking a 0.3 M KC1 + 5% glucose solution for 7 days was compared to that of control rats drinking a 5% glucose solution in order to investigate the effect of potassium loading upon the early and late step of aldosterone biosynthesis. Following potassium loading the quartered adrenals produced more aldosterone but less corticosterone as compared to the control. Potassium loading resulted in an increased aldosterone production rate by capsular adrenals (z. glomerulosa) provided that the corticosterone concentration in the incubation medium was elevated either by incubating it together with the decapsulated adrenal or adding exogenous corticosterone (4--16 mug/ml) to the medium. The corticosterone to aldosterone converting capacity of capsular adrenals is markedly higher in the potassium-loaded rats than in the controls. In the first 15 minutes of incubation the corticosterone production rate of the two groups was equal, aldosterone production rate by capsular adrenals of potassium-loaded rats, being higher than that of control animals. Corticosterone output of capsular adrenals from potassium-loaded rats decreased more rapidly in course of the incubation than it did in control tissue. These results suggest that the increase in aldosterone secretion in vivo following potassium loading is due to the stimulation of conversion of corticosterone to aldosterone in the glomerulosa cells. However, the endogenous corticosterone production during the incubation of glomerulosa cells from pottasium-loaded rats decreases so rapidly that the cells are not capable of producing more aldosterone than the control ones in spite of activated 18-hydroxylase.

Administration, Oral↗
PubMed1975

Steroid production by quartered and capsular rat adrenal gland in response to haemorrhage.

60 min after rapid bleeding (1.5--2.0 per cent of b. w.) both aldosterone and corticosterone production rate by quartered rat adrenals were found to be elevated. However, no difference was observed in the rate of aldosterone and corticosterone production by capsular adrenals of sham operated and hypovolaemic rats. Corticosterone production rate by decapsulated adrenals was much more higher after haemorrhage than in the control group. The same alterations could be observed incubating adrenal tissue with ACTH (0.3 mug per ml). Steroid production rate by quartered adrenals of sodium deficient rats was not affected by high in vitro concentration of angiotensin II (2.5 mug per ml). It is concluded that the effect of acute blood loss on corticosteroid biosynthesis of the rat is mediated by ACTH alone.

Adrenal Cortex Hormones↗