scholarly journals Adrenal Insufficiency After Unilateral Adrenalectomy in Primary Aldosteronism: Long-Term Outcome and Clinical Impact

2019 ◽  
Vol 104 (11) ◽  
pp. 5658-5664 ◽  
Author(s):  
Daniel A Heinrich ◽  
Christian Adolf ◽  
Finn Holler ◽  
Benjamin Lechner ◽  
Holger Schneider ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Primary Aldosteronism ◽  
Serum Cortisol ◽  
Adrenal Crisis ◽  
Acth Stimulation ◽  
Term Outcome ◽  
Unilateral Adrenalectomy ◽  
Long Term Outcome ◽  
Cortisol Levels

Abstract Context Primary aldosteronism (PA) represents a secondary form of arterial hypertension that can be cured by surgery. Evidence of adrenal insufficiency (AI) was recently found in patients with PA who had undergone unilateral adrenalectomy (uADX). Objective To study the incidence and long-term outcome of postoperative AI after uADX for PA. Design Prospective registry study (August 2014 until the end of 2018). Setting Tertiary referral center. Patients One hundred consecutive patients undergoing uADX for PA were included. All patients underwent postoperative ACTH stimulation testing. Intervention Postoperative ACTH stimulation testing to identify patients with AI. Main Outcome Measures Incidence of patients with postoperative AI and definition of long-term outcome. Results Twenty-seven percent of patients developed postoperative AI. Of these, 48% had postoperative ACTH stimulation serum cortisol levels ≤13.5 µg/dL (severe AI); 52% were classified into the group with moderate AI (stimulated serum cortisol levels: 13.5 to 17 µg/dL). Patients with severe AI required significantly longer hydrocortisone replacement therapy than the moderate group (median [25th, 75th percentiles]: 353 [294, 476] days; 95% CI: 284 to 322 days; vs 74 [32, 293] days; 95% CI: 11 to 137 days; P = 0.016). One patient with severe AI was hospitalized for an acute adrenal crisis. With a cumulative follow-up of 14.5 years, this produced an incidence rate of 6.9 adrenal crises per 100 patient-years. Conclusion We suggest performing postoperative ACTH stimulation tests in all patients who undergo uADX for PA.

scholarly journals Long-term outcome from unilateral adrenalectomy in patients with primary aldosteronism

2015 ◽  
Author(s):  
Mark J Hannon ◽  
Candy Sze ◽  
Robert Carpenter ◽  
Laila Parvanta ◽  
Matthew Matson ◽  
...  
Keyword(s):  
Primary Aldosteronism ◽  
Term Outcome ◽  
Unilateral Adrenalectomy ◽  
Long Term Outcome

scholarly journals Assessment of Adrenal Function in Women Heterozygous for Adrenoleukodystrophy1

1997 ◽  
Vol 82 (3) ◽  
pp. 856-860
Author(s):  
Samer S. El-Deiry ◽  
Sakkubai Naidu ◽  
Lewis S. Blevins ◽  
Paul W. Ladenson
Keyword(s):  
Fatty Acid ◽  
Adrenal Insufficiency ◽  
Serum Cortisol ◽  
Chain Fatty Acid ◽  
Acth Stimulation ◽  
Long Chain Fatty Acid ◽  
Increased Risk ◽  
The Mean ◽  
Cortisol Levels ◽  
Long Chain

Abstract Adrenoleukodystrophy (ALD) is an X-linked recessive disorder that destroys the white matter of the brain and is associated with adrenal insufficiency. The prevalence of adrenal dysfunction in 71 women carriers of the X-linked ALD gene was studied. These subjects were identified initially on the basis of being obligate carriers of the X-linked trait by pedigree analysis and were confirmed by plasma very long chain fatty acid levels consistent with a heterozygote status. One subject had well documented overt adrenal insufficiency, diagnosed and treated since age 9 yr. Among the remaining women, the mean serum 0800 h and 1 h post-ACTH cortisol concentrations [16 ± 7 (±sd) and 34 ± 8 μg/dL, respectively] were normal. All subjects had normal ACTH-stimulated serum cortisol levels, i.e. more than 20 μg/dL. However, 4 subjects (6%) had subnormal ACTH-stimulated aldosterone concentrations (mean, 9 ± 6 vs. 42 ± 16 ng/dL for other subjects; P = 0.001, by Mann Whitney rank sum test). Three of these women (75%) were taking nonsteroidal antiinflammatory agents (NSAIDs), whereas only 4 of 67 (6%) subjects with normal aldosterone responsiveness were NSAIDs users (P < 0.01, by Fisher’s exact test). Thus, NSAIDs use was associated with increased risk of hypoaldosteronism (odds ratio, 50.2; 95% confidence interval, 3.3–266; P < 0.002). Three of these four women had symptoms consistent with mineralocorticoid deficiency. Serum sodium and potassium concentrations were normal in all subjects. Basal and metyrapone-stimulated plasma ACTH concentrations were also normal in adequately tested subjects with and without mineralocorticoid insufficiency. Five of eight subjects (63%) who underwent testing with synthetic ovine CRH (oCRH) had abnormalities. Three did not meet the criteria for adequate cortisol stimulation (i.e. >20 μg/dL) and had peak ACTH levels greater than 30 pg/mL. Two other subjects had exaggerated ACTH responses with normal cortisol levels. There were no significant differences in the mean or median levels of very long chain fatty acid, C26:0, C24/22 ratios, or C26/22 ratios among the entire subject group, the subgroup with blunted aldosterone responses to ACTH, and the subgroup with blunted responses to oCRH (P > 0.05, by ANOVA and Kruskall-Wallis test for C26, C24/22 ratio, and C26/22 ratio). We conclude that 1) adrenal cortical insufficiency rarely develops in ALD heterozygotes; 2) isolated mineralocorticoid insufficiency can occur in ALD heterozygotes, as has been previously reported to occur with autoimmune and acquired immunodeficiency syndrome-related adrenal dysfunction; 3) ALD heterozygosity may predispose these individuals to NSAID-related hypoaldosteronism; and 4) a subclinical decrease in glucocorticoid reserve, as measured by oCRH testing, may be present in a majority of these women. Aldosterone levels should be included in the ACTH stimulation testing when seeking evidence of adrenal insufficiency in affected women. NSAIDs should be considered a risk factor for the development of hypoaldosteronism in women heterozygous for ALD.

scholarly journals Dual-release Hydrocortisone in Addison’s Disease—A Review of the Literature

2013 ◽  
Vol 09 (02) ◽  
pp. 177
Author(s):  
Roberta Giordano ◽  
Federica Guaraldi ◽  
Rita Berardelli ◽  
Ioannis Karamouzis ◽  
Valentina D Angelo ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Pharmaceutical Preparations ◽  
Serum Cortisol ◽  
Immediate Release ◽  
Time Profile ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Related Quality ◽  
Health Related ◽  
Dual Release

In patients with adrenal insufficiency, glucocorticoids (GCs) are insufficiently secreted and GC replacement is essential for health and, indeed, life. Despite GC-replacement therapy, patients with adrenal insufficiency have a greater cardiovascular risk than the general population, and suffer from impaired health-related quality of life. Although the aim of the replacement GC therapy is to reproduce as much as possible the physiologic pattern of cortisol secretion by the normal adrenal gland, the pharmacokinetics of available oral immediate-release hydrocortisone or cortisone make it impossible to fully mimic the cortisol rhythm. Therefore, there is an unmet clinical need for the development of novel pharmaceutical preparations of hydrocortisone, in order to guarantee a more physiologic serum cortisol concentration time-profile, and to improve the long-term outcome in patients under GC substitution therapy.

scholarly journals Steroid-Induced Iatrogenic Adrenal Insufficiency in Children: A Literature Review

Endocrines ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 125-137
Author(s):  
Shogo Akahoshi ◽  
Yukihiro Hasegawa
Keyword(s):  
Adrenal Insufficiency ◽  
Serum Cortisol ◽  
Early Morning ◽  
Serum Cortisol Level ◽  
Adrenal Crisis ◽  
Acth Stimulation ◽  
Morning Serum Cortisol ◽  
High Doses ◽  
Loss Of Appetite ◽  
Cortisol Suppression

The present review focuses on steroid-induced adrenal insufficiency (SIAI) in children and discusses the latest findings by surveying recent studies. SIAI is a condition involving adrenocorticotropic hormone (ACTH) and cortisol suppression due to high doses or prolonged administration of glucocorticoids. While its chronic symptoms, such as fatigue and loss of appetite, are nonspecific, exposure to physical stressors, such as infection and surgery, increases the risk of adrenal crisis development accompanied by hypoglycemia, hypotension, or shock. The low-dose ACTH stimulation test is generally used for diagnosis, and the early morning serum cortisol level has also been shown to be useful in screening for the condition. Medical management includes gradually reducing the amount of steroid treatment, continuing administration of hydrocortisone corresponding to the physiological range, and increasing the dosage when physical stressors are present.

scholarly journals Clinical Characteristics and Long-Term Outcome of Primary Aldosteronism in a Norwegian Population

2017 ◽  
Vol 49 (11) ◽  
pp. 838-846 ◽  
Author(s):  
Marianne Grytaas ◽  
Siri Strømsøy ◽  
Jarle Rørvik ◽  
Jarle Arnes ◽  
Anette Heie ◽  
...  
Keyword(s):  
Primary Aldosteronism ◽  
Clinical Characteristics ◽  
Diagnostic Procedures ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Confirmatory Testing ◽  
Female Sex ◽  
Norwegian Population

AbstractPrimary aldosteronism (PA) is the most common cause of secondary hypertension (HT). We describe here clinical characteristics, diagnostic procedures, and long-term outcomes in a Norwegian population. All suspected PA patients investigated at a tertiary centre from 1998–2012 were retrospectively evaluated. Inclusion criteria were verified PA after confirmatory testing or otherwise considered highly likely PA. Clinical, biochemical, radiological, and adrenal vein sampling (AVS) findings were analysed. Surgically removed adrenals were re-evaluated histopathologically and tested for somatic mutations. All patients still alive by August 2014 were invited to a follow-up visit. One-hundred and eight patients were included, of whom 85% had a history of hypokalaemia. PA was verified by confirmatory testing in 83 (77%), and AVS performed in 95 (88%) patients. The proportion with AVS-confirmed bilateral PA increased during the study period. Sixty-eight patients (63%) underwent adrenalectomy. KCNJ5 mutations were found in 30% of the surgical specimens and were associated with female sex and a florid PA phenotype. Follow-up visits were undertaken in 73/108 (68%), of whom 52 adrenalectomised. After adrenalectomy, 83% were biochemically cured of PA, but only 21% were cured for HT. Female sex, a verified adenoma, and KCNJ5 mutations were associated with cure of HT. In conclusion, the majority of our patients had unilateral PA and hypokalaemia, indicating that patients with bilateral and milder PA may still be underdiagnosed. Female sex, a histopathological adenoma, and the presence of KCNJ5 mutations predicted cure of HT after adrenalectomy, but the overall cure rate of HT was low.

scholarly journals Primary aldosteronism: key characteristics at diagnosis: a trend toward milder forms

2018 ◽  
Vol 178 (6) ◽  
pp. 605-611 ◽  
Author(s):  
Daniel A Heinrich ◽  
Christian Adolf ◽  
Lars C Rump ◽  
Ivo Quack ◽  
Marcus Quinkler ◽  
...  
Keyword(s):  
Primary Aldosteronism ◽  
Newly Diagnosed ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Independent Variables ◽  
Key Characteristics ◽  
Drug Doses ◽  
Epidemiological Characteristics ◽  
Referral Practice

Objective Primary aldosteronism (PA) is the most common endocrine form of arterial hypertension. The German Conn’s Registry’s purpose is to improve treatment outcomes of PA. We assessed whether key clinical, biochemical and epidemiological characteristics of newly diagnosed PA cases have changed over time, potentially indicating a different screening and referral practice in Germany evolving from 2008 to 2016. Design The German Conn’s Registry is a multicenter database prospectively analyzing morbidity and long-term outcome of patients with PA. Methods Phenotypic changes between three year periods were calculated using Mann–Whitney U tests and Kruskal–Wallis tests for independent variables. Results Over three time periods from 2008 to 2016, we noted a relative decrease of unilateral PA cases (67 vs 43%). Significantly more females were diagnosed with PA (33 vs 43%). Median daily defined drug doses decreased (3.1 vs 2.0) in the presence of unchanged SBP (150 vs 150 mmHg), plasma aldosterone (199 vs 173 ng/L) and PRC (3.2 vs 3.2 U/L). Median ARR values decreased (70 vs 47 ng/U) and median potassium levels at diagnosis (3.5 vs 3.7 mmol/L) increased as the percentage of normokalemic patients (25 vs 41%), indicating milder forms of PA. Conclusions Our results are in accordance with an increased screening intensity for PA. We identified a trend toward diagnosing milder forms, increasingly more females and less unilateral cases of PA.

scholarly journals Dual-release Hydrocortisone in Addison’s Disease – A Review of the Literature

2010 ◽  
Vol 10 (1) ◽  
pp. 75 ◽  
Author(s):  
Roberta Giordano ◽  
Federica Guaraldi ◽  
Rita Berardelli ◽  
Ioannis Karamouzis ◽  
Valentina D Angelo ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Pharmaceutical Preparations ◽  
Serum Cortisol ◽  
Immediate Release ◽  
Time Profile ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Physiological Serum ◽  
Related Quality ◽  
Health Related

In patients with adrenal insufficiency, glucocorticoids (GCs) are insufficiently secreted and GC replacement is essential for health and, indeed, life. Despite GC-replacement therapy, patients with adrenal insufficiency have a greater cardiovascular risk than the general population, and suffer from impaired health-related quality of life. Although the aim of the replacement GC therapy is to reproduce as much as possible the physiological pattern of cortisol secretion by the normal adrenal gland, the pharmacokinetics of available oral immediate-release hydrocortisone or cortisone make it impossible to fully mimic the cortisol rhythm. Therefore, there is an unmet clinical need for the development of novel pharmaceutical preparations of hydrocortisone, in order to guarantee a more physiological serum cortisol concentration time-profile, and to improve the long-term outcome in patients under GC substitution therapy.

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