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

2016 ◽  
Vol 175 (3) ◽  
pp. R107-R116 ◽  
Author(s):  
Sophie Bensing ◽  
Anna-Lena Hulting ◽  
Eystein S Husebye ◽  
Olle Kämpe ◽  
Kristian Løvås
Keyword(s):  
Quality Of Life ◽  
Adrenal Insufficiency ◽  
Rare Disease ◽  
Mineral Density ◽  
Primary Adrenal Insufficiency ◽  
Disease Epidemiology ◽  
Autoimmune Polyendocrine Syndrome ◽  
Increased Risk ◽  
Patients Experience

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.

Addison’s Disease Presenting with Abdominal Pain

2006 ◽  
Vol 5 (3) ◽  
pp. 102-103
Author(s):  
Tejal Desai ◽  
◽  
Tristan Richardson ◽  
Keyword(s):  
Quality Of Life ◽  
Abdominal Pain ◽  
Life Expectancy ◽  
Adrenal Insufficiency ◽  
Unusual Case ◽  
Primary Adrenal Insufficiency ◽  
Prompt Treatment ◽  
Bilateral Destruction ◽  
Important Disease

Primary Adrenal Insufficiency is due to bilateral destruction of the adrenal cortex and has a prevalence of approximately 40-60 per million adults. 1 The commonest cause is autoimmune. 1 Prompt treatment not only restores quality of life but also a normal life expectancy. 2 Here we present an unusual case of primary adrenal insufficiency, which illustrates how such an important disease often provides a diagnostic conundrum.

C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema

2011 ◽  
Vol 4 ◽  
pp. CMBD.S4090 ◽  
Author(s):  
Solange Oliveira Rodrigues Valle ◽  
Alfeu Tavares França ◽  
Regis A. Campos ◽  
Anete Sevciovic Grumach
Keyword(s):  
Quality Of Life ◽  
Rare Disease ◽  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
Genetic Deficiency ◽  
Life Threatening ◽  
Patients Experience ◽  
Tremendous Impact ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary suffering and dangerous situations. HAE can have a tremendous impact on the quality of life. The major genetic deficiency in this disorder is either an absent or nonfunctional C1INH which regulates the complement, fibrinolitic, kalikrein and plasmin pathways.

scholarly journals The relationship between glucocorticoid replacement and quality of life in 2737 hypopituitary patients

2014 ◽  
Vol 171 (5) ◽  
pp. 571-579 ◽  
Author(s):  
Oskar Ragnarsson ◽  
Anders F Mattsson ◽  
John P Monson ◽  
Helena Filipsson Nyström ◽  
Ann-Charlotte Åkerblad ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Primary Adrenal Insufficiency ◽  
Main Hypothesis ◽  
Gh Replacement ◽  
Significant Difference ◽  
Patients Experience ◽  
The Impact ◽  
The Relationship ◽  
Dose Dependent

ObjectiveQuality of life (QoL) is impaired in hypopituitary patients and patients with primary adrenal insufficiency. The aim of this study was to analyse the impact of glucocorticoid (GC) replacement on QoL. The main hypothesis was that ACTH-insufficient patients experience a dose-dependent deterioration in QoL.Design, patients and methodsThis was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). Data from 2737 adult GH-deficient (GHD) hypopituitary patients were eligible for analysis. Thirty-six per cent were ACTH sufficient and 64% ACTH insufficient receiving a mean±s.d. hydrocortisone equivalent (HCeq) dose of 22.3±8.7 mg (median 20.0). QoL at baseline and 1 year after commencement of GH replacement was assessed by the QoL-assessment of GHD in adults.ResultsAt baseline, no significant difference in QoL was observed between ACTH-sufficient and -insufficient patients. Increasing HCeq dose was associated with worse QoL. Patients on HCeq ≤10 mg had the best and patients receiving ≥25 mg demonstrated the poorest QoL. At 1 year of GH replacement, the improvement in QoL did not differ between ACTH-sufficient and -insufficient patients, and no association was observed between HCeq dose and QoL improvement.ConclusionAdult hypopituitary patients with untreated GHD receiving GC replacement have similar QoL as ACTH-sufficient patients. Among ACTH-insufficient patients, there is a dose-dependent association between increasing dose and impaired QoL. This association may be explained by supraphysiological GC exposure although it remains plausible that clinicians may have increased GC doses in order to address otherwise unexplained QoL deficits.

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