Cases Of Adrenocortical Insufficiency Research Articles

Adrenocortical hypertrophy: establishing cause and toxicological significance

The primary cause of adrenocortical hypertrophy is increased adrenocorticotrophic hormone (ACTH) stimulation. In toxicology studies, such a condition can arise as a result of the stress response, but it may also occur due to deficient glucocorticoid feedback regulation of ACTH due to toxicity to the adrenal cortex. This latter condition is defined as adrenocortical insufficiency and represents a serious adverse toxic effect on the function of the adrenal cortex. Adrenocortical hypertrophy may occur in the absence of other adrenocortical lesions such that a toxicopathological mechanism is not obvious, for example by pharmacological inhibition of steroidogenesis at the biochemical level. This review discusses the different aetiological factors and mechanisms producing adrenocortical hypertrophy. The need for further evidence in ascribing findings to stress is discussed, as is a protocol for establishing differential diagnoses between stress-induced and toxicity-induced adrenocortical hypertrophy, which is useful in cases where there are no other histopathological lesions in the adrenal cortex. It is concluded that all cases of adrenocortical hypertrophy require further investigation or evidence to ascribe such findings to either stress or adrenocortical inhibition/insufficiency, and that all cases of adrenocortical insufficiency (whether due to a histopathological lesion or reversible pharmacological enzyme inhibition) represent a serious adverse effect that must be properly considered in toxicological risk assessment.

Importance of family history in patients with adrenoleukodystrophy

Sir, X-linked adrenoleukodystrophy accounts for up to 10% cases of adrenocortical insufficiency and is now being increasingly identified in young males with idiopathic Addison’s disease. Widespread pathological damage is encountered in the nervous system due to abnormal accumulation of very long chain fatty acids (VLCFAs). The disease often demonstrates intrafamilial phenotypic variability, suggesting that non-genetic factors play a role in such expression. A detailed history and early estimation of VLCFAs in clinically suspected individuals make diagnosis possible in affected families. We present an interesting case of adrenoleukodystrophy where diagnosis was helped by a significant family history and also a brief review of the contemporary literature. A 33-year-old gentleman presented in December 2002 with complaints of giddiness, vomiting, abdominal pain, loose motions and pigmentation around the umbilicus. He had confirmed hyponatremia (Na-122 mEq/l) and a subnormal synacthen test (cortisol at 30 min was 287 nmol/l) and was diagnosed with Addison’s disease and was commenced on hydrocortisone and fludrocortisone replacement. He had a stable disease throughout except for two episodes of Addisonian crisis secondary to gastroenteritis requiring intravenous steroids. Six years later, he presented with complaints of bladder disturbance (polyuria, urgency and dysuria). He was hospitalized thrice in a very short span of time with confirmed urinary tract infections. One such episode led a complete retention of urine for which he had to be catheterized for 2 days. Digital examinations of …

Ultrasound screening of neonatal adrenal hemorrhage.

Neonatal adrenal hemorrhage (NAH) is frequently overlooked because the majority of cases are asymptomatic. In this study, we investigated the clinical symptoms, echographic characteristics and incidence of NAH from January 1998 to December 1999 at Cathay General Hospital, Taipei, Taiwan. Among 3273 newborns who had ultrasound screening, 18 cases were diagnosed as having NAH. The incidence was 0.55% which was compatible with other reports. But there was a female predominance (10:8) which was different from previous reports. Only one premature baby was diagnosed with NAH. No patients had perinatal asphyxia, and this was different from other reports. The mean birth body weight was 3530 grams which was more than the 75th percentile of normal term baby birth weight. Sixteen cases had right-sided NAH and two cases had left-sided NAH. Eleven cases (61%) within this study were asymptomatic. The incidence of pathologic jaundice was 11%. Four babies (22%) had poor oral feeding combined with vomiting. Only one had severe anemia for which blood transfusion was required. There were no cases of adrenocortical insufficiency in our study. Only one patient received adrenalectomy. In conclusion, NAH can be detected early by sonography and most cases require only supportive treatment. Thus, unnecessary surgery and severe complications due to delayed diagnosis can be avoided.

Adrenocortical insufficiency and diffuse cerebral sclerosis: Problems of presentation and diagnosis

Three cases of adrenocortical insufficiency with diffuse cerebral sclerosis are described. One, which was confirmed by brain biopsy, had a family history of the combined disorder affecting members of a previous generation; this has not been previously reported; however pathological confirmation of the diagnosis in the earlier cases was not available. Two cases presented as acute neurosurgical emergencies and attention is drawn to this mode of presentation. Problems of aetiology, presentation and diagnosis are discussed and the finding of normal basal steroid levels is stressed.

Value of an ACTH Test in Assessing Hypothalamic-Pituitary-Adrenocortical Function in Glucocorticoid-treated Patients

Forty-eight patients receiving glucocorticoid treatment were tested with ACTH stimulation using alpha(1, 24) ACTH (tetracosactrin). All patients subsequently underwent non-acute major surgery without any glucocorticoid administration, and their clinical course and plasma corticosteroids were followed closely. No case of adrenocortical insufficiency was observed. A highly significant correlation was found between the pre-operative adrenocortical response to ACTH and the hypothalmic-pituitary-adrenocortical (H.P.A.) response to surgery. A normal response to ACTH stimulation was never followed by a greatly impaired H.P.A. response to surgery. It seems that a simple ACTH stimulation test is reliable in predicting the integrated H.P.A. response to major stress in glucocorticoid-treated patients.

Case of adrenocortical insufficiency and congestive heart failure

A case of adreno-cortical insufficiency co-existing with congestive heart failure is described. The heart failure was unresponsive to therapy until cortisone was also given and maintained.

Chronic occult adrenocortical insufficiency: report of a case.

The classic picture of adrenal insufficiency as described by Thomas Addison in 1868 is well recognized. 1 Recently, Haydar and associates 2 have described seven cases of adrenocortical insufficiency with normal basal levels of urinary 17-hydroxycorticoids and only a few of the typical clinical manifestations of this disease. The patient in the case presented here has had various nonspecific manifestations for many years, which in retrospect may well have been due, at least in part, to borderline adrenocortical function and presented ultimately in a state of acute addisonian crisis. Report of a Case A 50-year-old childless housewife was admitted to the Bridgeport Hospital on Jan. 30,1958, because of recurrent spells of vomiting and syncope for one day. One week prior to admission she experienced transient nausea and diarrhea, which was relieved by administration of a mixture of kaolin and pectin suspended with methylcellulose (Kaopectate). Severe vomiting and watery diarrhea recurred