SEARCH · PubMed Health

Results for “Adrenal Cortex Function Tests”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 325 records · Page 18Linked to original sources

PubMed1990

[Diagnosis of the heterozygote carrier state in 21-hydroxylase deficiency using steroids].

In an attempt to elaborate a method for screening heterozygous carriers of inborn adrenal hyperplasia caused by insufficiency of steroid 21-hydroxylase, the authors examined in 24 obligatory heterozygotes and in seven controls the concentrations of four steroids. Cortisol (F), 11 beta-hydroxyandrostendione (11-OH), 17 alpha-hydroxyprogesterone (17-OH) and androstendione (A) were estimated by the RIA method using non-commercial antisera. In the examined subjects an abbreviated ACTH test was made and the mentioned parameters were assessed at times 0, 30 and 60 minutes. The results (nmol/l) were expressed by the relationship R = F.11-OH/17-OH.A for all investigated time intervals. The coefficient R60 or difference of coefficients delta R = R60-R0 differed significantly and overlapped to a minimum extent in the group of heterozygotes and controls. The authors assume that the suggested method for the detection of carriership of 21-hydroxylase insufficiency could be used in genetic counselling.

17-alpha-Hydroxyprogesterone↗

PubMed1979

Adrenal suppression after short-term corticosteroid therapy.

Adrenal function was evaluated in fourteen cancer patients receiving chemotherapy which included short-term high-dose courses of prednisone. 90 min corticotropin stimulation tests were performed before therapy and 1, 2, 4, and 7 days after steroids were discontinued. Responses were evaluated by standard criteria of adrenal function and by measurement of the intergrated cortisol response to corticotropin over 90 min. Thirteen of fourteen patients had suppressed adrenal function for at least 24 h. Although in most patients adrenal function had returned to normal between day 2 and 4, in five patients it remained suppressed for 7 days or more. Suppression did not correlate with either steroid dose or duration of therapy. Four of five patients receiving only 5 days' therapy showed evidence of adrenal suppression. Although overt clinical adrenal insufficiency after steroid administration is rare, these results indicate that adrenal function is suppressed more regularly after short-term high-dose steroid therapy than has been appreciated.

Adolescent↗

PubMed1999

Tests of adrenal insufficiency.

AIM: In suspected adrenal insufficiency, the ideal test for assessing the hypothalamo-pituitary-adrenal axis is controversial. Therefore, three tests were compared in patients presenting with symptoms suggestive of adrenal insufficiency. METHOD: Responses to the standard short Synacthen test (SSST), the low dose Synacthen test (LDST), and the 08:00 hour serum cortisol concentration were measured in 32 patients. A normal response to the synacthen test was defined as a peak serum cortisol of >/= 500 nmol/l and/or incremental concentration of >/= 200 nmol/l. The sensitivity and specificity of the 08:00 hour serum cortisol concentration compared with other tests was calculated. RESULTS: Three patients had neither an adequate peak nor increment after the SSST and LDST. All had a serum 08:00 hour cortisol concentration of < 200 nmol/l. Eight patients had abnormal responses by both criteria to the LDST but had normal responses to the SSST. Three reported amelioration of their symptoms on hydrocortisone replacement. Twenty one patients had a normal response to both tests (of these, 14 achieved adequate peak and increment after both tests and seven did not have an adequate peak after the LDST but had a normal increment). The lowest 08:00 hour serum cortisol concentration above which patients achieved normal responses to both the LDST and SSST was 500 nmol/l. At this cut off value (compared with the LDST), the serum 08:00 hour cortisol concentration had a sensitivity of 100% but specificity was only 33%. CONCLUSION: The LDST revealed mild degrees of adrenal insufficiency not detected by the SSST. The value of a single 08:00 hour serum cortisol concentration is limited.

Adolescent↗

PubMed1999

Adrenal insufficiency in septic shock.

BACKGROUND: Functional adrenal insufficiency has been documented in critically ill adults. OBJECTIVE: To document the incidence of adrenal insufficiency in children with septic shock, and to evaluate its effect on catecholamine requirements, duration of intensive care, and mortality. SETTING: Sixteen-bed paediatric intensive care unit in a university hospital. METHODS: Thirty three children with septic shock were enrolled. Adrenal function was assessed by the maximum cortisol response after synthetic adrenocorticotropin stimulation (short Synacthen test). Insufficiency was defined as a post-Synacthen cortisol increment < 200 nmol/l. RESULTS: Overall mortality was 33%. The incidence of adrenal insufficiency was 52% and children with adrenal insufficiency were significantly older and tended to have higher paediatric risk of mortality scores. They also required higher dose vasopressors for haemodynamic stability. In the survivor group, those with adrenal insufficiency needed a longer period of inotropic support than those with normal function (median, 3 v 2 days), but there was no significant difference in duration of ventilation (median, 4 days for each group) or length of stay (median, 5 v 4 days). Mortality was not significantly greater in children with adrenal insufficiency than in those with adequate adrenal function (6 of 17 v 5 of 16, respectively). CONCLUSION: Adrenal insufficiency is common in children with septic shock. It is associated with an increased vasopressor requirement and duration of shock.

Adolescent↗

PubMed2006

Variability of cortisol assays can confound the diagnosis of adrenal insufficiency in the critically ill population.

OBJECTIVE: To compare the total plasma cortisol values obtained from three widely used immunoassays and a high pressure liquid chromatography (HPLC) technique on samples obtained from patients with sepsis. DESIGN AND SETTING: Observational interventional in the general intensive care unit of a metropolitan hospital PATIENTS AND PARTICIPANTS: Patients admitted to the intensive care unit with a diagnosis of sepsis and fulfilling criteria of systemic inflammatory response syndrome. INTERVENTIONS: Standard short synacthen test performed with 250microg cosyntropin. MEASUREMENTS AND RESULTS: Two of the three immunoassays returned results significantly higher than those obtained by HPLC: Immulite by 95% (95%CI 31-188%) and TDx by 79% (21-165%). The limits of agreement for all three immunoassays with HPLC ranged from -62% to 770%. In addition, by classifying the patients into responders and non-responders to ACTH by standard criteria there was concordance in all assays in only 44% of patients. CONCLUSIONS: Immunoassay estimation of total plasma cortisol in septic patients shows wide assay related variation that may have significant impact in the diagnosis of relative adrenal insufficiency.

Adrenal Cortex Function Tests↗

PubMed1995

Increased 17 alpha-hydroxyprogesterone response to ACTH in silent adrenal adenoma: cause or effect?

OBJECTIVE: Recent studies suggest a possible connection between silent adrenal nodules and mild forms of 21-hydroxylase deficiency. It remains unclear whether the enzymatic deficiency is generalized or intrinsic to the adrenal mass. To help to clarify this, we have studied 17 alpha-OH-progesterone (17OH-P) response to ACTH stimulation in a group of patients with adrenal 'incidentaloma' in comparison with normal subjects. In patients who underwent surgical treatment, the test was repeated to evaluate possible modifications in 17OH-P behaviour after resection of the adrenal mass. SUBJECTS AND METHODS: Fifteen subjects with incidentally discovered asymptomatic adrenal masses were studied. Basal hormone evaluations were normal, with normal cortisol suppression after low-dose dexamethasone. Iodocholesterol scanning, performed in 12 patients, showed normal bilateral adrenal uptake in 2 subjects and an increased uptake on the side of the lesion in 10 subjects. In every patient, ACTH stimulation was performed to evaluate the secretory response of cortisol, 17OH-P, progesterone and dehydroepiandrosterone sulphate. An identical test was performed in 10 control subjects with normal adrenal glands, matched for age and sex. In six patients with an adrenal lesion > 3.5 cm, the ACTH stimulation test was repeated one month after surgery. RESULTS: The 17OH-P response to ACTH stimulation was significantly higher in subjects with adrenal 'incidentaloma' than in controls (P < 0.01). In particular, 10 subjects out of 15 (66%) evidenced a 17OH-P peak > 18 nmol/l at 60 minutes. No differences were seen in baseline 17OH-P or cortisol levels or in cortisol response to ACTH between the two groups. Dehydroepiandrosterone sulphate concentrations were significantly lower in patients with adrenal 'incidentaloma' than in normals. In six patients who had an increased 17OH-P response to ACTH on initial evaluation, the ACTH test was repeated one month after surgery. In five of these patients, 17OH-P response to ACTH was clearly reduced, suggesting that in these cases the enzymatic defect was restricted to the adenoma. In the other patient, however, stimulated 17OH-P levels remained unchanged. In this case, therefore, all of the adrenal tissue seems to be involved, suggesting a late-onset 21-hydroxylase deficiency. No significant modifications in cortisol response to ACTH were observed. CONCLUSION: It seems therefore that in some cases of incidentaloma the steroidogenic enzymatic defect may be secondary to the adenomatous proliferation, while in others such defects may induce the development of silent adrenal nodules.

17-alpha-Hydroxyprogesterone↗

PubMed1995

The "4A" syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities.

The triad of adrenocortical insufficiency with alacrima and achalasia is an unusual disease entity in paediatrics. The association of autonomic and peripheral neuropathies has more commonly been reported in older individuals. We describe four children (two siblings) with this disorder, aged between 3 and 6 years at diagnosis, all of whom had clinical neurological abnormalities when examined between 6 and 8 years of age. In addition, we performed cardiovascular autonomic testing in three subjects: heart rate variation during deep breathing was abnormal in all three; Valsalva ratio was abnormal in two; and postural systolic blood pressure response was abnormal in one. Pupillary reflexes were abnormal in the only subject in which they could be measured. These results indicate that subtle neurological and, in particular, autonomic abnormalities can be detected at an early age. We propose that autonomic neuropathy be considered as an integral feature of this rare condition and suggest the term "4A" syndrome as a useful mnemonic for the association of adrenocortical insufficiency, achalasia and alacrima with autonomic and other neurological abnormalities.

Adrenal Cortex Function Tests↗

PubMed1975

Aspects of the adrenal function in the domestic fowl.

Chromatographic purification of extracts of hen peripheral plasma on Florisil columns before measurement by spectrofluorometry showed a basal level for corticosterone of 1-3 mug/100 ml, which is much lower than concentrations previously reported using acid fluorescence. Neither handling, restraint nor repeated bleeding affected the concentration of this hormone or of glucose. Adrenal function tests with two preparations of synthetic corticotrophin showed that they caused a rapid rise in blood corticosterone and eventually of glucose. Results of studies using insulin or tolbutamide i.v. suggest that there is a threshold concentration of plasma glucose (about 70 mg/100 ml) below which hypoglycaemia stimulates the hypothalamic-pituitary-adrenal system in fowls. Prolonged treatment of laying hens with protamine zinc insulin led to aphagia and cessation of egg-laying; increased concentrations of corticosterone were observed 2 days after the administration of insulin ceased, coinciding with the return to normal plasma levels of glucose. 'Chemical adrenalectomy' with metyrapone showed that the restoration of plasma glucose to a normal concentration after insulin treatment is dependent upon fully functional adrenal cortical tissue. It appears likely that the adrenal medulla is a target for corticosterone which probably regulates the tissue levels of phenylethanolamine-N-methyltransferase, one of the enzymes necessary for the biosynthesis of adrenaline.

Adrenal Cortex Function Tests↗

PubMed2002

Prospective examination of adrenocortical function in advanced AIDS patients.

In human immunodeficiency virus infected individuals, human cytomegalovirus (CMV) remains a significant pathogen. The adrenal gland is a preferential site of CMV disease in acquired immunodeficiency syndrome (AIDS) patients. However, glucocorticoid replacement is often not selected because of the risk of exacerbating underlying infection. To evaluate the need for glucocorticoid replacement in these patients, we performed a prospective study to investigate the adrenal function in 60 advanced AIDS patients. Their adrenal function including rapid ACTH test (RAT), basal plasma ACTH level, and daily urinary free cortisol level was evaluated. Approximately 25% of the patients turned out to be abnormal in this evaluation. Almost 60% of the patients could be followed up for one year or until their death. Using the follow-up data, we calculated sensitivity, specificity, positive predictive value and negative predictive value. Excretion of urinary free cortisol with normal RAT was comparable to normal controls, whereas patients with abnormal RAT excreted significantly lower urinary free cortisol. During hospitalization, 14 patients with normal RAT had febrile episode. During the febrile period the concentration of the urinary free cortisol level increased by 2.2 times. This study suggests that glucocorticoid replacement is necessary for AIDS patients suspected as clinically adrenal insufficiency, and that the dose of glucocorticoid replacement might be increased during sick days in AIDS patients with abnormal adrenal function.

Acquired Immunodeficiency Syndrome↗

PubMed1987

Adrenal and steroidal cell antibodies in patients with autoimmune polyglandular disease type I and risk of adrenocortical and ovarian failure.

Thirty-one patients with autoimmune polyglandular disease type I who initially had no adrenocortical and/or ovarian failure were followed for 1.2-12.1 yr (mean, 8.3) by determinations of adrenal (AA) and steroidal cell antibodies (SCA) and functional tests. Adrenocortical failure developed in 13 and ovarian failure in 11 patients. SCA or AA preceded adrenocortical failure in 12 of the 13 patients and were found in 2 of 9 patients (so far) who still have normal adrenal function (P = 0.001). SCA preceded ovarian failure in all 11 patients and were found in 6 of 11 patients who still have normal ovarian function (P = 0.02). The sensitivities/specificities/predictive values were 0.77/0.78/0.90 in all patients for SCA predicting adrenocortical failure, and 0.92/0.89/0.92 for adrenal-binding antibody (which includes all AA and most SCA) in predicting adrenocortical failure. The sensitivities/specificities/predictive values in females who initially had normal adrenocortical and ovarian function were 1.0/0.56/0.50 for SCA in predicting ovarian failure, 0.86/0.83/0.86 for SCA in predicting adrenocortical failure, and 1.0/1.0/1.0 for adrenal-binding antibody in predicting adrenocortical failure. Thus, the appearance of AA or SCA in a male patient without adrenocortical failure or a female patient without adrenocortical or ovarian failure signals a high risk of their development.

Adolescent↗

PubMed1996

Skin bruising in asthmatic subjects treated with high doses of inhaled steroids: frequency and association with adrenal function.

High doses of inhaled corticosteroids (ICS) (budesonide and beclo-methasone > or = 800 micrograms.day-1) are commonly used in the treatment of asthma. Some investigators have presented evidence for cutaneous effects (bruising), which suggests systemic absorption. This study aimed to assess the prevalence of skin bruising, relate the occurrence of skin bruising to adrenocortical function, and determine the risk factors for skin bruising. One hundred adult asthmatic subjects taking high doses (800-2,000 micrograms.day-1) of ICS for 3 months or more were recruited in an asthma clinic, and 100 control subjects paired for sex and age were recruited from an ophthalmology out-patient clinic. A detailed questionnaire on asthma, general habits and cutaneous lesions was administered. A cutaneous examination was performed. Urine cortisol levels were assessed on two consecutive days. Blood cortisol level and the response to Cortrosyn injection (60 min test) were evaluated. One hundred adult asthmatics (66 females and 34 males), 73 on beclomethasone and 27 on budesonide, were included. The prevalence of skin bruising was 71% based on the questionnaire (32% in controls) and 48% (39 out of 81 subjects) based on direct examination of the skin. We found a satisfactory association between the response to questionnaire and examination of the skin. Adrenocortical function testing showed that a minority of subjects (14 with at least one abnormal test) had lower urinary or blood cortisol levels. These low cortisol levels occurred in subjects who reported skin bruising. By multiple logistic regression, being a female (odds ratio (OR) = 20; 95% confidence interval (95% CI) = 13-33) and taking ICS for asthma (OR = 12; 95% CI = 8-18) were significantly (t = 5.4) related to the likelihood of developing skin bruising. In addition, among the asthmatic subjects, being older (OR = 1.6; 95% CI = 1.1-2.4/10 yrs interval) (t = 2.3) and being a female (OR = 22; 95% CI = 7-75) (t = 5.0) influenced the occurrence of skin bruising as documented by questionnaire. In asthmatic subjects, taking high doses of ICS is associated with: 1) increased occurrence of skin bruising by comparison with controls, particularly in older subjects; and 2) a generally normal adrenocortical function, although this function is significantly lower in subjects reporting skin bruising.

Administration, Inhalation↗

PubMed2002

[Diagnostic and therapeutic strategy for an incidental finding of an adrenal mass].

The incidental finding of an unsuspected adrenal mass ranges from 0.5% to 5% in abdominal CT series. The optimal diagnostic approach to such masses is to diagnose malignant or secretory tumors requiring excision and to otherwise avoid unnecessary surgery. Physical examination generally contributes little. A standard biochemical evaluation should include the measurement of 24 hour urinary catecholamines and metanephrine, urinary free cortisol and plasma cortisol levels at 8 a.m. and 8 p.m. combined with an overnight low-dose dexamethasone suppression test, serum potassium assay, and determination of upright plasma aldosterone to plasma renin activity. These tests will serve to screen for pheochromocytoma, subclinical Cushing's syndrome, and primary hyperaldosteronism respectively. Imaging characteristics suggestive of malignancy include: size greater than 4 cm., heterogeneous lesion and/or density greater than 20 Hounsfield Units on CT scan, slow enhancement with delayed washout after intravenous contrast injection on CT scan, and slightly decreased signal intensity in out of phase (fat suppressed) MR acquisition. Fine-needle aspiration biopsy should be performed only if metastatic disease is suspected. Adrenal scintigraphy with iodocholesterol may be useful where adenoma with subclinical Cushing's syndrome or solid tumor is suspected. In summary, the following strategy is recommended for the management of adrenal incidentalomas : mass lesions larger than 4 cm. and hormone-secreting tumors should be removed. All non-secreting adrenal incidentalomas smaller than 4 cm. in diameter should be followed by serial imaging at regular intervals (6, 12, and 36 months) and by endocrine reevaluation at one year.

Adrenal Cortex Function Tests↗

PubMed2006

Adrenal function testing in patients with septic shock.

INTRODUCTION: Adrenal failure (AF) is associated with increased mortality in septic patients. Nonetheless, there is no agreement regarding the best diagnostic criteria for AF. We compared the diagnosis of AF considering different baseline total cortisol cutoff values and Deltamax values after low (1 microg) and high (249 microg) doses of corticotropin, we analyzed the impact of serum albumin on AF identification and we correlated laboratorial AF with norepinephrine removal. METHODS: A prospective noninterventional study was performed in an intensive care unit from May 2002 to May 2005, including septic shock patients over 18 years old without previous steroid usage. After measurement of serum albumin and baseline total cortisol, the patients were sequentially submitted to 1 microg and 249 microg corticotropin tests with a 60-minute interval between doses. Post-stimuli cortisol levels were drawn 60 minutes after each test (cortisol 60 and cortisol 120). The cortisol 60 and cortisol 120 values minus baseline were called Deltamax1 and Deltamax249, respectively. Adrenal failure was defined as Deltamax249 < or = 9 microg/dl or baseline cortisol < or = 10 microg/dl. Other baseline cortisol cutoff values referred to as AF in other studies (< or =15, < or =20, < or =25 and < or =34 mug/dl) were compared with Deltamax249 < or = 9 microg/dl and serum albumin influence. Norepinephrine removal was compared with the baseline cortisol values and Deltamax249 values. RESULTS: We enrolled 102 patients (43 male). AF was diagnosed in 22.5% (23/102). Patients with albumin < or =2.5 g/dl presented a lower baseline total cortisol level (15.5 microg/dl vs 22.4 microg/dl, P = 0.04) and a higher frequency of baseline cortisol < or =25 microg/dl (84% vs 58.3%, P = 0.05) than those with albumin > 2.5 g/dl. The Deltamax249 levels and Deltamax249 < or = 9, however, were not affected by serum albumin (14.5 microg/dl vs 18.8 microg/dl, P = 0.48 and 24% vs 25%, P = 1.0). Baseline cortisol < or = 23.6 microg/dl was the most accurate diagnostic threshold to determine norepinephrine removal according to the receiver operating characteristic curve. CONCLUSION: AF was identified in 22.5% of the studied population. Since Deltamax249 < or = 9 microg/dl results were not affected by serum albumin and since the baseline serum total cortisol varied directly with albumin levels, we propose that Deltamax249 < or = 9 microg/dl, which means Deltamax after high corticotropin dose may be a better option for AF diagnosis whenever measurement of free cortisol is not available. Baseline cortisol < or =23.6 microg/dl was the best value for predicting norepinephrine removal in patients without corticosteroid treatment.

Adrenal Cortex↗

PubMed2004

The low-dose corticotropin stimulation test in acute traumatic and non-traumatic brain injury: incidence of hypo-responsiveness and relationship to outcome.

OBJECTIVE: To investigate adrenal responses to the low-dose corticotropin (ACTH) stimulation test in acute traumatic or non-traumatic brain injury (BI) and to assess its value in predicting outcome. DESIGN: Prospective study. SETTING. Intensive care unit (ICU) in a university hospital. PATIENTS AND PARTICIPANTS: Seventy-five patients with acute BI, with a median age of 45 years were investigated. BI was due to trauma ( n=51), ischemic stroke ( n=17), subarachnoid hemorrhage ( n=4) or intracerebral hemorrhage (n=3). INTERVENTIONS: Blood was taken on day 16 (median) after admission to the ICU to determine baseline cortisol and ACTH. Thereafter, a low-dose stimulation test (LDST) was performed: 1 microg of tetracosactrin was injected and 30 min later a second blood specimen was obtained to measure stimulated cortisol. Patients having a stimulated cortisol below 500 nmol/l were defined as non-responders to the LDST. MEASUREMENTS AND RESULTS: Median baseline and stimulated cortisol were 491 nmol/l and 690 nmol/l, respectively. The median increment in cortisol was 154 nmol/l (range 5-579 nmol/l). Mean ACTH was 46+/-21 pg/ml. Ten (13%) patients were non-responders to the LDST; these had a higher mortality rate compared to patients with adequate cortisol production (70 vs 32%, p=0.034). Logistic regression analysis revealed that APACHE II ( p<0.001), Glasgow Coma Scale (GCS) ( p=0.04) and age ( p=0.02) were independent outcome predictors. In contrast, the increment in cortisol ( p=0.26) did not add to outcome prediction. CONCLUSIONS: Adrenal hypo-responsiveness in the setting of acute traumatic or non-traumatic BI is not an independent outcome predictor in the presence of high APACHE II, low GCS and older age.

Adolescent↗

PubMed1993

Human corticotropin-releasing hormone test in patients with hypothalamo-pituitary-adrenocortical disorders.

To assess the diagnostic usefulness of a synthetic human corticotropin-releasing hormone (CRH) formulation (Code No. MCI-028), we administered 100 micrograms of the peptide intravenously to 183 patients with hypothalamo-pituitary-adrenocortical (HPA) disorders, and obtained the following findings. Among the 183 patients, data from 125 patients were suitable for analyzing the effects of the test. In patients with Cushing's disease, high plasma adrenocorticotropic hormone (ACTH) and cortisol levels increased further in response to MCI-028, while in patients with adrenal Cushing's syndrome, low ACTH and high cortisol values remained unchanged. In patients with pituitary-type hypopituitarism or isolated ACTH deficiency, low ACTH and cortisol levels responded poorly or insignificantly to MCI-028, whereas those with hypothalamic hypopituitarism showed delayed and considerable degree of responses of plasma ACTH and little increase in plasma cortisol levels. In Addison's disease, high plasma ACTH increased further in response to MCI-028, but low cortisol levels did not change. In patients with Cushing's syndrome soon after successful surgical treatment, plasma ACTH responsiveness was low or different depending on the clinical course of the patient. Patients treated with high doses of glucocorticoids for non-endocrine diseases tended to show impaired ACTH and cortisol responsiveness to MCI-028. Side effects, including the transient flushing which was observed most frequently in this study, did not cause any clinical problems.

Adolescent↗

PubMed1993

Evaluation of hypothalamo-pituitary-adrenocortical function in children by human corticotropin-releasing hormone (MCI-028) test.

A dose of 1.5 micrograms/kg of MCI-028, human corticotropin-releasing hormone (hCRH), was administered intravenously to 38 children with non-endocrine short stature with normal function in the hypothalamo-pituitary-adrenocortical axis and to 71 children with a disorder in the same axis. Blood levels of adrenocorticotropic hormone (ACTH) and cortisol were determined to evaluate the axis. The 95% confidence limits of peak responses of ACTH and cortisol in non-endocrine short stature were between 17.2 and 135.3 pg/ml, and between 13.1 and 35.6 micrograms/dl, respectively, and were used as standards for children. When compared with these standards, the hormonal responses in children with various disorders in the hypothalamo-pituitary-adrenocortical axis were as follows: in two children with Cushing's syndrome caused by adrenal tumor, ACTH values were decreased and were not responsive to hCRH, while cortisol values, though within the normal limit, were not responsive; in children with primary adrenal insufficiency or congenital adrenal hyperplasia, cortisol values were decreased and not responsive, whereas ACTH values tended to be increased and ACTH response high except for 21 alpha-hydroxylase deficiency of congenital adrenal hyperplasia. In two cases of pituitary dwarfism complicated with ACTH deficiency, both ACTH and cortisol values were decreased and poorly responsive; and in children who were receiving glucocorticoid, both ACTH and cortisol values tended to be decreased and to respond poorly to hCRH. As for side effects, hot flushing was observed among 8.0% of the subjects after administration of hCRH. But this symptom was not severe and no other side effects of clinical importance were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1999

Results of the ACTH stimulation test in hirsute women.

OBJECTIVE: To determine the incidence of late-onset congenital adrenal hyperplasia (LOCAH) due to 21-hydroxylase deficiency among hirsute women and to evaluate the results of the ACTH stimulation test with the clinical characteristics. STUDY DESIGN: Prospective, controlled study. One hundred women with hirsutism and 14 normally cycling women without hirsutism were included in this study at the Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, Cerrahpasa School of Medicine, Istanbul University. After basal serum progesterone (P) and 17 hydroxyprogesterone (17OHP) levels were determined, an ACTH stimulation test was performed on cycle day 3-5. The same parameters were checked 30 minutes later. We estimated the 21 hydroxylase activity by calculating the change in 17OHP (17OHP 30-0) and the summed rate of the change in P and 17OHP ([P30-0] + [17OHP30-01/30 minutes). The 95th percentile for these estimates in normal women were calculated, and values above three times the 95th percentile were considered to distinguish women with LOCAH due to 21-hydroxylase deficiency. RESULTS: The 95th percentile for 17OHP 30-0 and (P30-0) + (17OHP30-0)/30 minutes in normal women was 1.6 and 8.9 ng/dL/min, respectively. Regarding 17OHP 30-0 values, three women with hirsutism had levels above three times the 95th percentile of these estimates, and 28 women had estimates of more than the 95th percentile but less than threefold. Seventeen of 28 women had oligomenorrhea, and all had severe hirsutism. The women with severe hirsutism and oligomenorrhea had significantly higher ACTH-stimulated serum 17OHP levels and values for 17OHP 30-0 and (P30-0 + (17OHP30-0)/30 min) than did normally cycling women. CONCLUSION: The incidence of LOCAH due to 21-hydroxylase deficiency and mild 21-hydroxylase deficiency is 3% and 28%, respectively, in women with hirsutism. Clinical characteristics are not helpful in determining 21-hydroxylase deficiency. However, the incidence of 21-hydroxylase deficiency is more common among women with severe hirsutism and oligomenorrhea. The change in serum 17OHP 30-0 seems to be greater than the summed rate of change in serum 17OHP and P in the detection of 21-hydroxylase enzyme deficiency.

17-alpha-Hydroxyprogesterone↗

PubMed1975

Adrenoleukodystrophy. Electron microscopic findings.

Ultrastructural and neurochemical studies were done on three male patients with adrenoleukodystrophy. In each case, the affected white matter contained enlarged glial cells filled with pathognomic intracytoplasmic inclusions consisting of electron-lucent spicules bounded by 25-Angstrom wide membranes. Similar inclusions were present in adrenocortical cells. These findings and a review of 47 reported cases indicate that adrenoleukodystrophy is a storage disorder caused by a sex-linked recessive error of metabolism.

Adrenal Cortex↗