scholarly journals Acute Primary Adrenal Insufficiency after Hip Replacement in a Patient with Acute Intermittent Porphyria

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Adele Latina ◽  
Massimo Terzolo ◽  
Anna Pia ◽  
Giuseppe Reimondo ◽  
Elena Castellano ◽  
...  
Keyword(s):  
Postmenopausal Woman ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Genetic Disease ◽  
Hip Replacement ◽  
Acute Intermittent Porphyria ◽  
Adrenal Hemorrhage ◽  
Primary Adrenal Insufficiency ◽  
Threatening Condition ◽  
Life Threatening

Adrenal insufficiency is a potentially life-threatening condition when it occurs acutely, as in adrenal hemorrhage. Generally it is not reversible and requires chronic replacement therapy. Acute intermittent porphyria (AIP) is a rare genetic disease characterized by alterations in heme biosynthesis that result in accumulation of precursors in tissues. A crisis can be triggered by many conditions such as surgery and infections. Symptoms are similar to those of acute hypoadrenalism. Moreover, both conditions are characterized by hyponatremia. We describe the case of a postmenopausal woman known to be affected by AIP who developed after surgery a primary adrenal insufficiency associated with adrenal enlargement; the latter completely reverted in six months.

Assessment of autoantibodies against 21-hydroxylase in the diagnosis of primary autoimmune adrenal insufficiency

2020 ◽  
Author(s):  
Leila Sozaeva ◽  
Nadezhda Makazan ◽  
Larisa Nikankina ◽  
Natalya Malysheva ◽  
Ekaterina Kuvaldina ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Patient Survival ◽  
Adrenal Crisis ◽  
Primary Adrenal Insufficiency ◽  
Adrenal Steroidogenesis ◽  
Threatening Condition ◽  
Life Threatening ◽  
The One

Primary adrenal insufficiency is manifested by a deficiency of adrenal cortex hormones and can lead to a life-threatening condition. Early diagnosis is key to patient survival. Auto-antibodies to one of the adrenal steroidogenesis enzymes, 21-hydroxylase, are an immunological marker of autoimmune adrenal insufficiency. On the one hand, the study of antibodies to 21-hydroxylase is a method that helps establish the etiology of the disease the autoimmune genesis of adrenal gland damage. On the other hand, the determination of autoantibodies to 21-hydroxylase is the only prognostic factor of the risk of adrenal insufficiency, which makes it possible to prevent the development of acute adrenal crisis. The article provides a brief literature review on autoantibodies to 21-hydroxylase and the pathogenesis of autoimmune adrenal insufficiency, and a series of clinical cases that illustrates the significant role of autoantibodies to 21-hydroxylase in diagnosis of adrenal insufficiency.

scholarly journals Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group

2017 ◽  
Vol 6 (8) ◽  
pp. 935-942 ◽  
Author(s):  
Lia Ferreira ◽  
João Silva ◽  
Susana Garrido ◽  
Carlos Bello ◽  
Diana Oliveira ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Adult Population ◽  
Serum Cortisol ◽  
High Plasma ◽  
Adrenal Crisis ◽  
Primary Adrenal Insufficiency ◽  
Electrolyte Disturbances ◽  
Threatening Condition ◽  
Life Threatening ◽  
Portuguese Study

Introduction Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI. Aims To characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI. Methods This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals. Results We investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 µg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment, 17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances. Conclusion This is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.

scholarly journals A Rare Case of Autoimmune Polyglandular Syndrome Type 2 in a Child With Persistent Fatigue

2019 ◽  
Vol 6 ◽  
pp. 2333794X1984507 ◽  
Author(s):  
Ryan Kenneth Smith ◽  
Peter M. Gerrits
Keyword(s):  
Adrenal Insufficiency ◽  
Autoimmune Polyglandular Syndrome ◽  
Threatening Condition ◽  
Viral Illness ◽  
Persistent Fatigue ◽  
Life Threatening ◽  
Local Emergency Department ◽  
Autoimmune Polyglandular Syndrome Type ◽  
High Index Of Suspicion

Adrenal insufficiency is a rare, potentially life-threatening condition whose diagnosis requires a high index of suspicion. Adrenal insufficiency may be primary, secondary, or tertiary with varied etiologies. Primary insufficiency may be part of a cluster of autoimmune diseases, referred to as autoimmune polyglandular syndrome(s) (APS). We describe a case of a 15-year-old male who presents to a local emergency department complaining of fatigue, fever, abdominal pain, nausea, and vomiting for a few days with a preceding viral illness. The patient was hyponatremic and hyperkalemic with skin hyperpigmentation, raising concern for adrenal insufficiency. Laboratory workup confirmed autoimmune primary adrenal insufficiency, with subsequent laboratory studies revealing autoimmune thyroiditis and celiac disease. Concomitant Addison’s and Hashimoto’s diseases led to a diagnosis of APS type 2. The patient was started on steroid replacement with rapid clinical improvement.

scholarly journals Bilateral Adrenal Hemorrhage in Coronavirus Disease 2019 Patient: A Case Report

2020 ◽  
Vol 105 (12) ◽  
pp. 3745-3749 ◽  
Author(s):  
Meir Frankel ◽  
Itamar Feldman ◽  
Michal Levine ◽  
Yigal Frank ◽  
Naama R Bogot ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Rare Condition ◽  
Renal Vein Thrombosis ◽  
Adrenal Hemorrhage ◽  
Polymerase Chain Reaction Test ◽  
Primary Antiphospholipid Syndrome ◽  
Thromboembolic Events ◽  
Adrenal Axis ◽  
Bilateral Adrenal Hemorrhage ◽  
Life Threatening

Abstract Context Bilateral adrenal hemorrhage is a rare condition with potentially life-threatening consequences such as acute adrenal insufficiency. Early adrenal axis testing, as well as directed imaging, is crucial for immediate diagnosis and treatment. Coronavirus disease 2019 (COVID-19) has been associated with coagulopathy and thromboembolic events. Case description A 66-year-old woman presented with acute COVID-19 infection and primary adrenal insufficiency due to bilateral adrenal hemorrhage (BAH). She also had a renal vein thrombosis. Her past medical history revealed primary antiphospholipid syndrome (APLS). Four weeks after discharge she had no signs of COVID-19 infection and her polymerase chain reaction test for COVID-19 was negative, but she still needed glucocorticoid and mineralocorticoid replacement therapy. The combination of APLS and COVID-19 was probably responsible of the adrenal event as a “two-hit” mechanism. Conclusions COVID-19 infection is associated with coagulopathy and thromboembolic events, including BAH. Adrenal insufficiency is life threatening; therefore, we suggest that early adrenal axis testing for COVID-19 patients with clinical suspicion of adrenal insufficiency should be carried out.

scholarly journals Prednisolone is associated with a worse bone mineral density in primary adrenal insufficiency

2018 ◽  
Vol 7 (6) ◽  
pp. 811-818 ◽  
Author(s):  
Kathrin R Frey ◽  
Tina Kienitz ◽  
Julia Schulz ◽  
Manfred Ventz ◽  
Kathrin Zopf ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Bone Mineral ◽  
Immediate Release ◽  
Modified Release ◽  
Mineral Density ◽  
Primary Adrenal Insufficiency ◽  
Z Scores

Context Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) therapy. Daily GC doses are often above the physiological cortisol production rate and can cause long-term morbidities such as osteoporosis. No prospective trial has investigated the long-term effect of different GC therapies on bone mineral density (BMD) in those patients. Objectives To determine if patients on hydrocortisone (HC) or prednisolone show changes in BMD after follow-up of 5.5 years. To investigate if BMD is altered after switching from immediate- to modified-release HC. Design and patients Prospective, observational, longitudinal study with evaluation of BMD by DXA at visit1, after 2.2 ± 0.4 (visit2) and after 5.5 ± 0.8 years (visit3) included 36 PAI and 8 CAH patients. Thirteen patients received prednisolone (age 52.5 ± 14.8 years; 8 women) and 31 patients received immediate-release HC (age 48.9 ± 15.8 years; 22 women). Twelve patients on immediate-release switched to modified-release HC at visit2. Results Prednisolone showed significantly lower Z-scores compared to HC at femoral neck (−0.85 ± 0.80 vs −0.25 ± 1.16, P < 0.05), trochanter (−0.96 ± 0.62 vs 0.51 ± 1.07, P < 0.05) and total hip (−0.78 ± 0.55 vs 0.36 ± 1.04, P < 0.05), but not at lumbar spine, throughout the study. Prednisolone dose decreased by 8% over study time, but no significant effect was seen on BMD. BMD did not change significantly after switching from immediate- to modified-release HC. Conclusions The use of prednisolone as hormone replacement therapy results in significantly lower BMD compared to HC. Patients on low-dose HC replacement therapy showed unchanged Z-scores within the normal reference range during the study period.

scholarly journals Primary Adrenal Insufficiency During Lenvatinib or Vandetanib and Improvement of Fatigue After Cortisone Acetate Therapy

2018 ◽  
Vol 104 (3) ◽  
pp. 779-784 ◽  
Author(s):  
Carla Colombo ◽  
Simone De Leo ◽  
Marta Di Stefano ◽  
Guia Vannucchi ◽  
Luca Persani ◽  
...  
Keyword(s):  
Adverse Event ◽  
Thyroid Cancer ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Dosage Reduction ◽  
Adrenal Function ◽  
Cortisone Acetate ◽  
Drug Discontinuation ◽  
Primary Adrenal Insufficiency ◽  
Common Cause

Abstract Context Two tyrosine kinase inhibitors (TKIs), lenvatinib and vandetanib, are often used to treat advanced radioiodine-refractory differentiated thyroid cancer (RAI-R DTC) and medullary thyroid cancer (MTC), respectively. Fatigue is a common adverse event during treatment with these and other TKIs and a common cause of drug discontinuation or dosage reduction. Cases Description We evaluated the basal and stimulated adrenal function in 12 patients with advanced RAI-R DTC and MTC treated with lenvatinib or vandetanib, respectively. Ten patients complaining of fatigue showed a progressive ACTH increase with normal cortisol levels. Moreover, six of 10 patients had a blunted cortisol response after ACTH stimulation, thus confirming the diagnosis of primary adrenal insufficiency (PAI). The causal relationship between TKIs and PAI onset was also demonstrated by the repeated testing of adrenal function before and during treatment. Patients with PAI received cortisone acetate replacement therapy, with a substantial and prompt improvement in the degree of fatigue, as assessed by the Common Terminology Criteria for Adverse Events version 4.03, thus supporting the major impact of impaired adrenal function in the genesis of this adverse event. Conclusions We show that the occurrence of PAI may be a common cause of fatigue during lenvatinib and vandetanib treatment, and we therefore recommend testing adrenal function for a prompt start of replacement therapy to avoid treatment discontinuation, dosage reduction, and potentially severe PAI complications.

scholarly journals Obojestranska krvavitev v nadledvičnico: vzrok visoki zgodnji zlatenici in akutni primarni insuficienci nadledvičnic pri novorojenčku

2018 ◽  
Vol 87 (3-4) ◽  
Author(s):  
Petja Fister ◽  
Marta Žnidaršič Eržen ◽  
Primož Kotnik ◽  
Mojca Tomažič
Keyword(s):  
Gestational Diabetes ◽  
Adrenal Insufficiency ◽  
Clinical Case ◽  
Adrenal Glands ◽  
Clinical Signs ◽  
Term Neonate ◽  
Primary Adrenal Insufficiency ◽  
Acute Adrenal Insufficiency ◽  
Life Threatening ◽  
Threatening Situation

Adrenal bleeding in a newborn is rare. The cause of bleeding is unknown, most likely due to several factors. Bleeding may be minimal with no clinical signs or fulminant with acute adrenal insufficiency, which is a life-threatening situation that requires immediate detection and treatment.In this paper we represent a clinical case of a term neonate born to the mother with gestational diabetes, who was hospitalised due to high early hyperbilirubinemia. Significant bleeding in both adrenal glands was identified by ultrasound and primary adrenal insufficiency diagnosed. We discuss possible causes, the diagnostic clues, the treatment of disease and its prognosis.

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