Characterization of Zona Glomerulosa Function in Patients with Classic and Non-classic Forms of Congenital Adrenal Hyperplasia due to 11ß-Hydroxylase Deficiency

Castro‐Magana, Mariano; Angulo, Moris; Canas, Jose A; Mazur, Bogdan; Sarrantonio, Mary J; Vitollo, Pam; Palekar, Anagha Vinayak; Fuentes, Billy; Lee, A.

doi:10.1515/jpem.1995.8.1.19

Cited by 5 publications

(1 citation statement)

References 19 publications

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“…However, diagnostic value of the LDT has not been investigated in detail so far in other primary adrenocortical disorders including the biosynthetic defects in steroidogenesis. NCAH due to 11-b OH deficiency is usually characterized by normal/increased ACTH level besides the normal cortisol and increased 11-deoxycortisol level (Castro-Magana et al, 1995;White, 2001). There is a subtle defect in steroid biosynthesis in 11-b OH deficient NCAH patients which is a good opportunity to assess the value of LDT in mild steroid biosynthetic defect in the adrenal cortex.…”

Section: Introductionmentioning

confidence: 99%

The Value of Low Dose (1 μg) ACTH Stimulation Test in the Investigation of Non-Classic Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

Unluhizarci¹,

Keleştimur²,

Güven³

et al. 2003

Exp Clin Endocrinol Diabetes

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Non-classic congenital adrenal hyperplasia (NCAH) is a rare cause of hirsutism and it results from a defect in the biosynthetic pathway of cortisol and/or aldosterone. 250 microg ACTH test (SDT) is used in the diagnosis of this disease. Our aim was to investigate the responses of 11-deoxycortisol to 1microg ACTH (LDT) test in women with NCAH due to 11-beta hydroxylase (11- beta OH) deficiency and to compare them with the values obtained after SDT in the patients and in the control subjects. Eleven patients with NCAH due to 11- beta OH deficiency and 15 control subjects were involved in the study. The main complaint of the patients with NCAH was hirsutism and the diagnosis was made if the adrenal 11-deoxycortisol response to SDT exceed threefold the 95th percentile of controls. ACTH stimulation tests were carried out consecutively by using 250 microg and 1 microg intravenous ACTH as a bolus injection after an overnight fast, and blood samples were drawn at 0,30 and 60 min. Peak cortisol, 17-hydroxyprogesterone (17-OHP) and DHEAS responses were similar in LDT and SDT while 11-deoxycortisol responses to LDT (15.7 +/- 1.8 nmol/L) were significantly (p < 0.005) lower than the results obtained after SDT (76.3 +/- 21.4 nmol/L) in women with 11- beta OH deficiency. Peak cortisol and 17-OHP responses to LDT in patients and control subjects were similar. Peak 11-deoxycortisol responses to LDT were significantly (p < 0.05) higher in NCAH patients (15.7 +/- 1.8 nmol/L) than in the control subjects (6.5 +/- 0.8 nmol/L). However, in LDT, all patients had peak 11-deoxycortisol level lower than threefold the 95th percentile (25.8 nmol/L) of controls. This study represents the first demonstration that LDT gives similar cortisol but not 11-deoxycortisol responses to SDT in patients with 11- beta OH deficiency. This study also showed that LDT can not replace SDT in every clinical situation.

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Section: Introductionmentioning

confidence: 99%

The Value of Low Dose (1 μg) ACTH Stimulation Test in the Investigation of Non-Classic Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

Unluhizarci¹,

Keleştimur²,

Güven³

et al. 2003

Exp Clin Endocrinol Diabetes

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Hypergonadism

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2023

Steroids in the Laboratory and Clinical Practice

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Clinical Pharmacology of Furosemide in Children: A Supplement

Prandota

2001

American Journal of Therapeutics

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Furosemide is one of the most effective and least toxic diuretics used in pediatric practice. Experimental and clinical data suggest that adrenocorticosteroids and/or endogenous ouabain-like substances may play an important role in its diuretic effect. Also, the drug appears to have anti-inflammatory properties. In children with different diseases who received orally or intravenously 1 to 2 mg/kg doses of furosemide, a statistically significant positive linear relationship was found between the drug urinary excretion rate and the urine flow rate, but log dose-response curves to the drug were found to vary depending on the disease and the route of the drug administration. No sigmoid-shaped log dose-response curve (ie, one approaching a zero response at very low furosemide urinary excretion rates and a maximum response at very high excretion rates) was attained, which may suggest that the capacity of the kidney tubules to respond diuretically to the aforementioned doses of furosemide was not exceeded in these patients. However, in infants with different diseases and reasonably normal renal function who required administration of this diuretic, a very steep log dose-response curve to a 1 mg/kg intravenous dose of furosemide was found, which may suggest that higher doses may not result in a significant increase in diuretic response. The lowest mean furosemide urinary excretion rate and its concentration in urine associated with a significant diuresis were found to be 0.58 +/- 0.33 microg/kg/min and 24.2 +/- 10.5 microg/ml, respectively. Also, a significant correlation was found between the amount (in milligrams) of furosemide excreted in the urine during the first 6 hours after administration and the urine volume collected during that time. Patients with cystic fibrosis appeared to have a markedly more pronounced diuretic response to the average oral dose of 0.835 +/- 0.18 mg/kg than that reported in control children given 2 mg/kg. In children with acute renal failure caused by acute gastroenterocolitis or glomerulonephritis, a broad relationship was observed between a single intravenous dose and diuretic response after administration of furosemide (1.2 to 30.8 mg/kg). It was suggested that the total daily dose of the drug should not exceed 100 mg in these patients. Furosemide was found to be effective in management of bronchoconstriction accompanying chronic lung disease and narrowing of the upper respiratory airways; in hydrocephalus in infancy to avoid cerebrospinal fluid shunts; in some diagnostic procedures, such as an assessment of fetal and neonatal hydronephrosis; and in evaluation of different types of renal tubular acidosis. Among side effects accompanying clinical use of this drug were cholelithiasis in premature infants receiving total parenteral nutrition concomitantly with the diuretic; secondary hyperparathyroidism and bone disease in infants obtaining long-term furosemide treatment; and drug-induced fever.

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Characterization of Zona Glomerulosa Function in Patients with Classic and Non-classic Forms of Congenital Adrenal Hyperplasia due to 11ß-Hydroxylase Deficiency

Cited by 5 publications

References 19 publications

The Value of Low Dose (1 μg) ACTH Stimulation Test in the Investigation of Non-Classic Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

The Value of Low Dose (1 μg) ACTH Stimulation Test in the Investigation of Non-Classic Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

Hypergonadism

Clinical Pharmacology of Furosemide in Children: A Supplement

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