Interventions for the prevention of adrenal crisis in adults with primary adrenal insufficiency: a systematic review.

Objective: The incidence of adrenal crisis (AC) remains high, particularly for people with primary adrenal insufficiency, despite the introduction of behavioural interventions. The present study aimed to identify and evaluate available evidence of interventions aiming to prevent AC in primary adrenal insufficiency. Design: This study is a systematic review of the literature and theoretical mapping. Methods: MEDLINE, MEDLINE in Process, EMBASE, ERIC, Cochrane CENTRAL, CINAHL, PsycINFO, the Health Management Information Consortium and trial registries were searched from inception to November 2021. Three reviewers independently selected studies and extracted data. Two reviewers appraised the studies for the risk of bias. Results: Seven observational or mixed methods studies were identified where interventions were designed to prevent AC in adrenal insufficiency. Patient education was the focus of all interventions and utilised the same two behaviour change techniques, 'instruction on how to perform a behaviour' and 'pharmacological support'. Barrier and facilitator themes aiding or hindering the intervention included knowledge, behaviour, emotions, skills, social influences and environmental context and resources. Most studies did not measure effectiveness, and assessment of knowledge varied across studies. The study quality was moderate. Conclusion: This is an emerging field with limited studies available. Further research is required in relation to the development and assessment of different behaviour change interventions to prevent AC.

Impact of glucocorticoid supplementation on reducing perioperative complications in patients on long-term glucocorticoid medication: A propensity score analysis using a nationwide inpatient database.

BACKGROUND: Perioperative glucocorticoid supplementation has been suggested as a potentially effective precaution against perioperative adrenal crisis in patients on long-term glucocorticoid medication. METHODS: This retrospective cohort study used a national inpatient database in Japan. We included patients who underwent general surgery and those who received long-term glucocorticoid medication before surgery. A one-to-one propensity score-matched analysis was performed to compare patients who received 100 mg hydrocortisone during surgery with those who received no supplementation. The primary outcome was use of vasopressor agents on the day of surgery. The secondary outcomes included bleeding, perioperative infection, wound dehiscence, postoperative length of stay, and in-hospital mortality. RESULTS: Among the 807 propensity score-matched pairs, there was no significant difference in use of vasopressor agents between patients with and without glucocorticoid supplementation (24.5% vs. 21.9%; P = 0.22) and no significant differences in any secondary outcomes. CONCLUSIONS: Perioperative glucocorticoid supplementation was not associated with decreased morbidity or mortality.

Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency.

Primary adrenal insufficiency (PAI), or Addison's disease, is a rare, potentially deadly, but treatable disease. Most cases of PAI are caused by autoimmune destruction of the adrenal cortex. Consequently, patients with PAI are at higher risk of developing other autoimmune diseases. The diagnosis of PAI is often delayed by many months, and most patients present with symptoms of acute adrenal insufficiency. Because PAI is rare, even medical specialists in this therapeutic area rarely manage more than a few patients. Currently, the procedures for diagnosis, treatment and follow-up of this rare disease vary greatly within Europe. The common autoimmune form of PAI is characterized by the presence of 21-hydroxylase autoantibodies; other causes should be sought if no autoantibodies are detected. Acute adrenal crisis is a life-threatening condition that requires immediate treatment. Standard replacement therapy consists of multiple daily doses of hydrocortisone or cortisone acetate combined with fludrocortisone. Annual follow-up by an endocrinologist is recommended with the focus on optimization of replacement therapy and detection of new autoimmune diseases. Patient education to enable self-adjustment of dosages of replacement therapy and crisis prevention is particularly important in this disease. The authors of this document have collaborated within an EU project (Euadrenal) to study the pathogenesis, describe the natural course and improve the treatment for Addison's disease. Based on a synthesis of this research, the available literature, and the views and experiences of the consortium's investigators and key experts, we now attempt to provide a European Expert Consensus Statement for diagnosis, treatment and follow-up.

[Treatment of adult men with congenital adrenal hyperplasia syndrome due to 21-hydroxylase deficiency]. / Behandlung des klassischen adrenogenitalen Syndroms mit 21-Hydroxylase-Defekt bei erwachsenen Männern.

Information about the treatment of males with congenital adrenal hyperplasia (CAH) is scarce and there are no therapeutical guidelines. The aim of this review is to provide a survey of the current data. An extensive literature research was performed in PubMed for relevant articles published in the last ten years. The aim in the treatment of adult male CAH patients is preservation of fertility, prevention of an addisonian crisis, blood pressure management, prevention of testicular adrenal rest tumors (TART), maintaining well-being and good quality of life, satisfactory sexual function and prevention of long-term side effects of gluco- and mineralocorticoid therapy. The change from paediatric to adult medicine should be handled in a transition outpatient clinic organized by paediatric and adult endocrinologists. Most studies have included only small numbers of patients. The steroid therapy is usually orientated on an individual basis; but, general guidelines are lacking. It is reported that fertility is often impaired and related to the occurrence of TART. Some of these tumors are responsive to altered glucocorticoid therapy. However, glucocorticoid-resistant TART have been described, and testis-sparing surgery seems to be a treatment option. A future system of regular follow-up visits and standardized therapy guidelines are essential to provide a better medical care and a higher quality of life for male patients with CAH.

Surgical management of hereditary pheochromocytoma.

BACKGROUND: Surgical treatment of hereditary pheochromocytoma remains controversial because of the need for lifelong corticosteroid therapy and the risk of Addisonian crisis associated with bilateral total adrenalectomy. We examined our large series of patients with hereditary pheochromocytoma to evaluate postsurgical outcomes, particularly in those who underwent cortical-sparing adrenalectomy. STUDY DESIGN: We retrospectively reviewed the outcomes of all patients with histopathologic diagnoses of hereditary pheochromocytoma treated at our institution from 1962 to 2003. Familial disease was initially determined by pedigree analysis, genetic testing, or both for multiple endocrine neoplasia (MEN) types 1, 2A, or 2B; von Hippel-Lindau disease (VHL); neurofibromatosis type 1 (NF-1); or familial paraganglioma syndrome (FP). RESULTS: Adrenal pheochromocytomas were present in 56 of 59 patients (95%): MEN2A (39), MEN2B (7), VHL (6), MEN1 (2), NF-1 (2). Paragangliomas (extraadrenal pheochromocytomas) were present in the remaining 3 of 59 patients (5%): FP (2) and NF-1 (1). Thirty-eight of 56 patients with pheochromocytomas had cumulative operations resulting in total or subtotal bilateral adrenalectomy. Acute adrenal insufficiency (Addisonian crisis) occurred in 4 of these 38 patients (11%). Cortical-sparing adrenalectomy was performed in 26 patients who underwent bilateral adrenal resection; 17 (65%) were corticosteroid independent at a median followup of 71 months. Recurrent pheochromocytoma developed in an adrenal remnant in 3 of 30 patients (10%) who underwent unilateral or bilateral cortical-sparing procedures. Metastatic disease did not develop in any patient with pheochromocytoma, but has occurred in two of three patients with paragangliomas. CONCLUSIONS: Our data suggest that a cortical-sparing adrenalectomy can successfully avoid the need for corticosteroid replacement in the majority of patients who undergo a bilateral adrenalectomy. Long-term followup should include monitoring of the remnant gland for recurrent pheochromocytoma with yearly biochemical screening studies.

Complications after bilateral adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2--a plea to conserve adrenal function.

OBJECTIVE: To evaluate the complications of the adrenocortical supplementation that is needed after bilateral adrenalectomy for phaeochromocytoma in patients with multiple endocrine neoplasia (MEN) type 2 syndrome. DESIGN: Retrospective study. SETTING: University hospital, The Netherlands. MATERIALS: 28 patients with MEN 2 who underwent total adrenalectomy for phaeochromocytoma between 1972 and 1996. MAIN OUTCOME MEASURES: Perioperative morbidity and mortality, histopathological findings, complications of adrenocortical supplementation therapy. RESULTS: 22 patients had bilateral phaeochromocytomas on histopathological examination (79%) and 6 patients had initially unilateral phaeochromocytomas There was no operative mortality or substantial morbidity except for one splenic injury that necessitated splenectomy. During a mean follow-up period of 14 years (range 1-26) nine patients (32%) had a total of 19 Addisonian crises that necessitated admission to hospital. One patient died of an unrecognised Addisonian crisis. CONCLUSION: Complications of adrenocortical supplementation therapy are considerable, but they can be reduced when unilateral adrenalectomy is done for a unilateral phaeochromocytoma in patients with MEN 2 syndrome, provided that they are carefully followed up.

Fetal adrenal transplantation: success of a laparoscopic technique in rats.

BACKGROUND/PURPOSE: The present study investigates a new laparoscopic technique for fetal adrenal transplantation in rats. RESULTS: The procedure was successful in 9 of 10 cases (one hole in the omentum) with no postoperative complications. On examination 4 weeks postoperatively, all but one graft showed macroscopic integrity, vascular supply, and histological maturation to normal zonal differentiation. When bilateral adrenalectomy was performed in the recipient to assess endocrine competence of the fetal adrenal grafts, survival was prolonged and Addison crisis was prevented in the animals that underwent transplantation. Levels of aldosterone dropped within the first week after adrenalectomy, but recovered steadily. Analysis of corticosterone demonstrated that levels fell to 25% of sham operated rats in the first week, but then steadily climbed to 70%. CONCLUSIONS: To the authors' knowledge this report presents the first study for laparoscopic transplantation of fetal tissues. Laparoscopic transplantation of fetal adrenal glands seemed feasible and successful in rats. The fetal adrenal transplants matured and served for a prolonged survival.

Metastatic tumors of the adrenals.

Adrenals are a common site of metastasis for many solid tumors. Adrenal metastases, and related symptoms of adrenal failure, are usually overlooked in clinical practice. This is probably due to the functional compensation of the adrenal glands and to the fact that signs and symptoms of adrenal insufficiency are aspecific, and often masked by symptoms of the neoplastic disease. In some tumors in which adrenal involvement is particularly frequent, adrenal evaluation should be an essential part of the preoperative diagnostic work-up. In case of demonstration of metastatic involvement the patient could be spared a useless resection of the primary tumor. However, in selected patients, even after the demonstration of an adrenal metastasis, radical surgery could still be considered for tumors with favorable biological behaviour. In patients with widespread disease, if clinical indicators of possible adrenal involvement are present an adequate palliative therapy should be started, thus ameliorating the quality of life of the patients.

[Acute endocrinology]. / Akutt endokrinologi.

The author presents a practical guide for diagnosis and management of some common emergencies. The most common are hypoglycaemia in persons with diabetes, diabetic ketoacidosis and non-ketotic hyperosmolar diabetic coma. Acute adrenocortical failure is also quite common. These three conditions can be presented in persons with diagnosed disease. Therefore prevention should be possible with proper education. In rare cases the emergency situation is the presenting symptom. Thyreotoxic crisis, myxoedema coma and hypercalcemic crisis are less common. A high level of suspicion is important for early diagnosis and successful treatment of these conditions.

Screening of patients with hereditary spastic paraparesis and Addison's disease for adrenoleukodystrophy/adrenomyeloneuropathy.

X-linked adrenoleukodystrophy/adrenomyeloneuropathy presents a wide variation of clinical manifestations and may mimic several diseases. A screening investigation by measuring plasma saturated very long-chain fatty acids was performed in two groups of patients. Among six patients with hereditary spastic paraparesis one woman was detected to be a heterozygous gene carrier. However, in a group of eleven adult men with idiopathic Addison's disease the plasma concentrations of very long-chain fatty acids were all within normal limits. We conclude that X-linked adrenoleukodystrophy and symptomatic heterozygous females should be considered in cases of progressive spastic paraparesis.