Disease relevance of Adrenal Insufficiency

• Our results suggest a basis for adrenal insufficiency and hypogonadotropic hypogonadism in males affected by adrenal hypoplasia congenita and are consistent with a role for DAX1 in gonadal sex determination [1].
• Adrenomyeloneuropathy is the name given to a biochemically identical but milder and more slowly progressive variant of adrenoleukodystrophy that affects young adults, in whom adrenal insufficiency may long precede nervous system dysfunction [2].
• CONCLUSION: In our trial, a 7-day treatment with low doses of hydrocortisone and fludrocortisone significantly reduced the risk of death in patients with septic shock and relative adrenal insufficiency without increasing adverse events [3].
• A 52-year-old woman experienced hypoadrenalism (mean 8 AM plasma cortisol level, 3.7 microgram/dL) after hypothalamic surgery and radiotherapy for craniopharyngioma [4].
• Using long-acting ovine CRF (1 microgram/kg body weight as an intravenous bolus), we tested patients with Cushing's syndrome, adrenal insufficiency, and psychiatric conditions with mild hypercortisolism [5].

Psychiatry related information on Adrenal Insufficiency

• The patients who may be distantly related appeared phenotypically similar with the classical triad of the triple A syndrome (adrenal insufficiency, alacrima and achalasia) with dermatological manifestations while lacking neurological features except for mild mental retardation [6].
• The mortality rates among patients with occult adrenal insufficiencies were 40% in the steroid therapy group and 55.6% in the standard therapy group [7].
• Additional physical and/or psychological stress prompts pituitary dysfunction in such patients, causing the manifestation of acute symptoms of adrenal insufficiency based on panhypopituitarism [8].

High impact information on Adrenal Insufficiency

• Dehydroepiandrosterone for adrenal insufficiency [9].
• Whereas a fetal/placental source of cortisol may mitigate crisis during gestation, unrecognized adrenal insufficiency may lead to maternal or fetal demise either during gestation or in the puerperium [10].
• Reversible adrenal insufficiency induced by ketoconazole [11].
• The UFC is a useful biochemical marker that correlated clinically with glucocorticoid deficiency or excess in patients with hypoadrenalism receiving maintenance steroid replacement [12].
• Metyrapone test with adrenocorticotrophic levels. Separating primary from secondary adrenal insufficiency [13].

Chemical compound and disease context of Adrenal Insufficiency

• In patients with secondary adrenal insufficiency, baseline plasma ACTH levels were normal, but neither 11-deoxycortisol nor ACTH levels increased in response to metyrapone [13].
• Neonatal subclinical adrenal insufficiency. Result of maternal steroid therapy [14].
• Five patients with adrenal insufficiency were placed on a diet of 60 meq potassium and 100-150 meq of sodium while receiving a constant amount of cortisone acetate and Florinef [15].
• Methadone-induced hypoadrenalism [16].
• The sodium retention associated with liquorice ingestion has been thought to be due to a direct mineralocorticoid effect, despite the fact that it does not seem to occur in patients or animals with severe adrenal insufficiency [17].

Biological context of Adrenal Insufficiency

• Thus, in adrenal insufficiency, loss of glucocorticoid feedback by itself can increase POMC gene expression in the pituitary; but CRH action is essential for this to result in increased secretion of ACTH [18].
• A mutation in the proximal (P)-box of the first zinc finger of SF-1 (G35E) has been reported to cause complete XY sex reversal and adrenal insufficiency [19].
• Duchenne muscular dystrophy with adrenal insufficiency and glycerol kinase deficiency: high resolution cytogenetic analysis with molecular, biochemical, and clinical studies [20].
• Sixteen patients developed persisting adrenal insufficiency after neurosurgery so that the total number of patients on permanent steroid substitution was 24 [21].
• Megestrol acetate induced hypoadrenalism with cushingoid phenotype in endometrial cancer [22].

Anatomical context of Adrenal Insufficiency

• To examine the effect of glucocorticoid deficiency on the glucocorticoid receptor, we examine the binding of [3H] dexamethasone to lymphocytes in normal subjects and patients with adrenal insufficiency before and after glucocorticoid replacement therapy [23].
• These findings suggest that chronic excessive ACTH levels resulting from adrenal insufficiency may stimulate Leydig cells and lead to gonadotropin-independent precocious puberty in some boys with DAX1 gene mutations [24].
• X-linked adrenoleukodystrophy (ALD) is an inborn error of peroxisomal metabolism characterised by progressive demyelination of the central nervous system and by hypoadrenalism [25].
• Preservation of the adrenal glomerulosa was found in all the patients with secondary adrenal insufficiency through the normal rise in salivary aldosterone after both LDT and SDT [26].
• Usual causes of intolerance to thyroxine sodium include coronary artery disease, advanced age, untreated adrenal insufficiency, and severe hypothyroidism [27].

Gene context of Adrenal Insufficiency

• Mutations of orphan nuclear receptors SF1 and DAX1 each cause adrenal insufficiency and gonadal dysgenesis in humans, although the pathological features are distinct [28].
• This CRH regimen was found previously to normalize plasma ACTH and cortisol patterns in patients with secondary adrenal insufficiency who had normal ACTH responses to a single injection of oCRH (hypothalamic adrenal insufficiency) [29].
• The effect of pulsatile human corticotropin-releasing hormone administration on the adrenal insufficiency that follows cure of Cushing's disease [29].
• Although the mechanisms leading to glandular autoimmune process may be at least in part shared by different endocrine organs, the role of CXCL10 in autoimmune adrenal insufficiency is unknown [30].
• PROP1 mutations cause progressive deterioration of anterior pituitary function including adrenal insufficiency: a longitudinal analysis [31].

Analytical, diagnostic and therapeutic context of Adrenal Insufficiency

• METHODS: In a double-blind study, 24 women with adrenal insufficiency received in random order 50 mg of dehydroepiandrosterone orally each morning for four months and placebo daily for four months, with a one-month washout period [32].
• Self-management of adrenal insufficiency by rectal hydrocortisone [33].
• All placebo and RU 486-replaced adrenalectomized monkeys expired within 33 days after adrenalectomy, presumably from adrenal insufficiency [34].
• In conclusion, these data suggest that CIR for a childhood brain tumor may affect the HPA axis, resulting in secondary adrenal insufficiency, whereas adjuvant chemotherapy does not seem to add to the deleterious effect of CIR [35].
• We found only one case of de novo adrenal insufficiency using admission ACTH injection in 70 selected intensive care unit (ICU) patients [36].

References