MANAGEMENT OF ENDOCRINE DISEASE Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

被引：44

作者：

Bensing, Sophie ^{[1
,2
]}

Hulting, Anna-Lena ^{[1
]}

Husebye, Eystein S. ^{[3
,4
]}

Kampe, Olle ^{[2
,5
]}

Lovas, Kristian ^{[3
,4
]}

机构：

[1] Karolinska Inst, Dept Mol Med & Surg, Stockholm, Sweden

[2] Karolinska Univ Hosp, Dept Endocrinol Diabet & Metab, Stockholm, Sweden

[3] Univ Bergen, Dept Clin Sci, Bergen, Norway

[4] Haukeland Hosp, Dept Med, Bergen, Norway

[5] Karolinska Inst, Ctr Mol Med, Dept Med Solna, Stockholm, Sweden

来源：

EUROPEAN JOURNAL OF ENDOCRINOLOGY | 2016年 / 175卷 / 03期

基金：

瑞典研究理事会;

关键词：

BONE-MINERAL DENSITY; GLUCOCORTICOID REPLACEMENT THERAPY; SUBJECTIVE HEALTH-STATUS; SUBCUTANEOUS HYDROCORTISONE INFUSION; ADDISONS-DISEASE; DEHYDROEPIANDROSTERONE REPLACEMENT; RELEASE HYDROCORTISONE; INCREASING PREVALENCE; CANCER INCIDENCE; P-GLYCOPROTEIN;

D O I：

10.1530/EJE-15-1242

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

In this article, we review published studies covering epidemiology, natural course and mortality in primary adrenal insufficiency (PAI) or Addison's disease. Autoimmune PAI is a rare disease with a prevalence of 100-220 per million inhabitants. It occurs as part of an autoimmune polyendocrine syndrome in more than half of the cases. The patients experience impaired quality of life, reduced parity and increased risk of preterm delivery. Following a conventional glucocorticoid replacement regimen leads to a reduction in bone mineral density and an increase in the prevalence of fractures. Registry studies indicate increased mortality, especially evident in patients diagnosed with PAI at a young age and in patients with the rare disease autoimmune polyendocrine syndrome type-1. Most notably, unnecessary deaths still occur because of adrenal crises. All these data imply the need to improve the therapy and care of patients with PAI.

引用

页码：R107 / R116

页数：10

共 106 条

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