Primary adrenocortical failure masked by exogenous steroid administration

This report describes an asthmatic patient in whom Addison's disease was masked by exogenous steroid treatment.     

Feminizing adrenocortical tumor: steroid hormone response to ketoconazole

A 58-yr-old man presented with gynecomastia and elevated serum estrogens. The diagnosis of an estrogen-secreting adrenal tumor was made based upon the finding of a 4-cm left adrenal mass, elevated levels of estradiol in peripheral and left adrenal venous blood, and increased urinary 17-ketosteroids. In addition to marked elevations in estradiol and 17-ketosteroids there was an increased baseline level of 11-deoxycorticosterone and a slightly decreased level of 18-hydroxycorticosterone, suggesting the possibility of impaired P450c11 activity. The effect of ketoconazole administration (600 mg/day) for 4 weeks was studied. Urinary free cortisol and 17-ketosteroid excretion and serum testosterone levels fell acutely (1 week). Serum estradiol levels decreased gradually over the 4-week course. Plasma aldosterone levels were essentially unaltered and 18-hydroxcorticosterone levels fell gradually, but there were marked increases in 11-deoxycorticosterone and corticosterone. Coincident with the increase in 11-deoxycorticosterone there was an increase in blood pressure and a transient fall in serum potassium. We conclude that ketoconazole administration may result in a hypermineralocorticoid state. Therefore, the usefulness of ketoconazole therapy for steroid hormone-producing neoplasms will depend upon the individual tumor's steroidogenic profile.     

以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症的临诊应对

报道3例以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症(LCH)患者,总结其临床表现、实验室检查、影像学检查、病理结果、诊断过程和治疗反应。3例患者早期均以中枢性尿崩症起病,垂体磁共振成像(MRI)均表现为垂体柄增粗,垂体后叶正常高信号消失。2例患者表现为孤立性下丘脑-垂体病变,1例表现为垂体和甲状腺多系统受累。病理结果显示典型的朗格汉斯细胞,免疫组织化学示S-100、CD1a、Langerin阳性。LCH临床表现呈现明显的异质性,容易误诊和漏诊。确诊依赖病理结果,孤立性下丘脑-垂体病变活检难度较大,建议积极筛查其他器官增加活检概率。LCH导致的神经垂体损害通常需要终生激素替代治疗。     

Wegener''s granulomatosis complicated by diabetes insipidus.

We report a case of Wegener's granulomatosis complicated by cranial diabetes insipidus in which no evidence of local sinus erosion or intracranial granuloma was found. Unlike previously reported cases, the diabetes insipidus has not resolved despite successful treatment of the vasculitis. The patient is also unusual in that she had a prolonged 9-month prodromal period of seronegative polyarthritis before the appearance of typical systemic vasculitis and granuloma formation.     

Klinefelter's syndrome accompanied by diabetes mellitus and diabetes insipidus.

The first case of Klinefelter's syndrome accompanied by diabetes insipidus and diabetes mellitus is reported. A 41-year-old man admitted for hyperosmolar diabetic coma with a past history of diabetes insipidus was diagnosed as having Klinefelter's syndrome by endocrinological examination and sex chromosome analysis. In this case, glucose tolerance test was normalized half a year later and blood glucose was well controlled with diet therapy alone.     

Intranasal administration of adrenocorticotropin-(1-24) stimulates adrenocortical hormone secretion

To determine the efficiency of transmucosal absorption of ACTH, we measured serum cortisol, aldosterone, dehydroepiandrosterone (DHEA), and DHEA sulfate (DHEA-S) levels after intranasal (in) vs. iv administration of ACTH-(1-24) (250 microg) in 12 healthy adult men (mean age, 24.3 +/- 3.2 yr; range, 21-31 yr), who had received no prior medication and had no symptoms of rhinitis. Blood was collected at 0, 30, 60, 120, and 180 min after administration of ACTH-(1-24), and the levels of adrenocortical steroids were measured by specific RIAs. There were no side-effects associated with in or iv ACTH administration. After in administration, serum cortisol and aldosterone increased rapidly by 224.7 +/- 39.2% and 147.2 +/- 50.5%, respectively, peaking 30 min after ACTH-(1-24) administration, and decreasing to basal levels within 120 min. These increases in serum cortisol and aldosterone were lower than those obtained after iv administration. Thirty minutes after in or iv administration of ACTH-(1-24), DHEA increased by 49.1 +/- 27.2% and 81.6 +/- 17.1%, respectively, and remained elevated for 180 min. Serum DHEA-S levels did not change after in administration of ACTH-(1-24) and increased only slightly after iv injection. Adrenocortical steroid levels did not increase after in administration of saline. These data demonstrate that adrenocortical steroids are stimulated by in administration of ACTH-(1-24). We suggest that intranasal administration of ACTH offers both a diagnostic approach as an adrenal function test and a therapeutic approach as ACTH replacement therapy in patients with ACTH deficiency. The latter may be more physiological than glucocorticoid replacement.     

Nephrogenic diabetes insipidus induced by colchicine--a case report

In some cases diabetes insipidus may be induced by taking medications. In this article we report the case of a 58-year-old man with manifest polyuria induced by colchicine administered because of a gout attack. Interestingly, similar symptoms were observed 6 years earlier after treatment with gentamycin. In the described state of the examined patient early detection of the disease, colchicine withdrawal and temporary amiloride (and indometacin) administration led to a full normalisation of the patient's clinical status. A similar reaction to two different drugs responsible for only a small percentage of iatrogenic diabetes insipidus suggests that some patients may exhibit a general "hypersensitivity" to the development of a drug-induced vasopressin resistance and that patients with the history of iatrogenic diabetes insipidus should not be treated or should be treated cautiously with other drugs known to induce vasopressin-resistant polyuria.     

Treatment of neurogenic diabetes insipidus

Central or neurogenic diabetes insipidus results from a deficiency in antidiuretic hormone (ADH) or arginine-vasopressin (AVP). Treatment is based on replacement therapy with the hormone analog desmopressin (d-DAVP). d-DAVP can be administered subcutaneously to infants or patients with postoperative or posttraumatic brain injury being monitored for transient diabetes insipidus. Intranasal and oral forms are also available. The recently introduced lyophilisate, which melts under the tongue, has replaced the tablet form (recently withdrawn from the market in France) and provides better bioavailability. Irrespective of the mode of administration, it is usually the patient who finds the effective minimal dose necessary for a normal life, i.e. without excessive polyuria, particularly at night. Patient education is necessary to avoid the risk of water intoxication and hyponatremia.     

Characterization of a new rodent model of diabetes insipidus: the Roman high avoidance rat homozygous for diabetes insipidus

These experiments were designed to characterize the nature and extent of diabetes insipidus present in a new model of genetic vasopressin (VP) deficiency, the Roman high avoidance rat homozygous for diabetes insipidus (RHA: di/di strain). The new strain was developed from an initial cross between Long-Evans derived Brattleboro (LE:di/di) rats and normal Roman high avoidance (RHA: +/+) rats, and has been bred to be congenic with the parent RHA: +/+ strain. RHA: di/di rats exhibited polydipsia, excreted dilute urine, and exhibited elevated plasma osmolality. RHA: di/di rats shows a similar urinary response to dehydration as LE: di/di rats. VP was undetectable by radioimmunoassay in the serum, brain, and neurohypophysis of RHA: di/di rats. VP-neurophysin containing cells were not observed in the brains of RHA: di/di rats upon immunocytochemical analysis. Thus, the new RHA: di/di strain exhibits essentially the same profile of diabetes insipidus as the LE: di/di rat. The congenic relationship between RHA: di/di and RHA: +/+ rats makes the RHA: di/di rat a useful model under circumstances where genetic variables unrelated to VP deficiency may confound the interpretation of data.