Adrenal Insufficiency in Pregnancy

Author(s):  
Meenu Jain ◽  
Sarah Knapp ◽  
Florence M. Brown
2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Jenny Carolina Bello ◽  
Xiaolei Chen ◽  
Kenneth K Chen

Abstract Nausea and vomiting are common symptoms in pregnancy, ranging from occasional nausea to fulminant and intractable vomiting. Many underlying metabolic disorders can mimic this, primary adrenal insufficiency (PAI) being one of them. Here, we present a case of adrenal insufficiency early in pregnancy. A 28 year old lady G1P0 at 8 weeks of gestations, with a past medical history of Grave’s Disease, presented to our hospital on 3 occasions over one week with severe intractable nausea and vomiting. On prior visits, she had received intravenous fluids and discharged home. Laboratory work-up was ordered on the third visit and she was found to have severe hyponatremia with level of 111mMol/L. TSH and FT4 levels were both within the reference range. AM cortisol level was low at 2.3mcg/dL. ACTH and renin were both significantly elevated confirming diagnosis of PAI. Intravenous hydrocortisone was commenced immediately with rapid resolution of her symptoms and correction of her hyponatremia. She was followed at the endocrinology clinic, with appropriate up-titration of glucocorticoid and mineralocorticoid doses throughout her pregnancy. Diagnosis of PAI is usually established prior to pregnancy. Presentation during pregnancy is not common, but it should be considered as a differential diagnosis when symptoms are out of proportion to the gestational status. Normal pregnancy is accompanied by progressive increase in circulating CRH and ACTH, increasing the levels of free cortisol as early as 7 weeks of gestation, rising up to 20-fold by the end of pregnancy. These physiologic changes could explain early presentation of adrenal crisis given insufficient glucocorticoid production. A delay in diagnosis and treatment increases the risk of maternal and fetal morbidity and mortality significantly. Management of PAI during pregnancy can be challenging as there are no established guidelines and they have mainly been based on observational studies (1). The appropriate selection and dose of the glucocorticoid is important for the treatment of PAI to minimize adverse effects on mother and baby (2). At the time of active labor and delivery, stress doses of glucocorticoids need to be administered to prevent adrenal crisis (3). In conclusion, early diagnosis and appropriate management of PAI during pregnancy is necessary to sustain a healthy pregnancy. Bibliography 1 Husebye ES, Allolio B, Arlt W, et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104– 15. 2 Bandoli G, Palmsten K, Forbess C, et al. A review of systemic corticosteroid use in pregnancy and the risk of select pregnancy and birth outcomes. Rheum Dis Clin North Am. 2017; 43(3): 489–502 3 Chen K, Powrie R. Approach to the use of Glucocorticoids in Pregnancy for Nonobstetric Indications. de Swiet’s Medical Disorders in Obstetric Practice: Fifth Edition. 736-741.


Author(s):  
Kavya Jonnalagadda ◽  
Nisha Bhavani ◽  
Praveen V. Pavithran ◽  
Harish Kumar ◽  
Usha V. Menon ◽  
...  

Spontaneous adrenal hemorrhage of pregnancy is an acute hemorrhage into the adrenal gland in pregnancy in the absence of trauma, tumor or decoagulant therapy. This can have catastrophic consequences on the mother and the baby and if the hemorrhage involves both the adrenal glands the risk is aggravated because of the high incidence of resulting adrenal insufficiency. We report a case of spontaneous bilateral adrenal hemorrhage in pregnancy resulting in adrenal crisis. A 26 year old primigravida presented at 32 weeks of gestation initially with right sided infrascapular pain and one month later with similar pain in the left side associated with high blood pressure. Imaging with ultrasound and MRI was suggestive of bilateral adrenal mass probably hemorrhage; 2 days following the second episode of pain she developed drowsiness and hypotension and a diagnosis of primary adrenal insufficiency was confirmed by a low serum cortisol and high ACTH. She stabilized with hydrocortisone therapy and the fetus was closely monitored. At 37 weeks she had a normal vaginal delivery under steroid cover. Repeat MRI abdomen 3 months after delivery showed resolution of the hemorrhage but biochemically she continued to be cortisol insufficient at 1 year of follow up. Prompt diagnosis of adrenal hemorrhage in pregnancy and treatment of adrenal insufficiency along with close fetal monitoring usually results in good perinatal outcome in spontaneous adrenal hemorrhage of pregnancy.


Endocrine ◽  
2013 ◽  
Vol 44 (2) ◽  
pp. 283-292 ◽  
Author(s):  
Kevin C. J. Yuen ◽  
Lindsay E. Chong ◽  
Christian A. Koch

2019 ◽  
Vol 105 (3) ◽  
pp. e42-e52 ◽  
Author(s):  
Benjamin Dean ◽  
Georgina L Chrisp ◽  
Maria Quartararo ◽  
Ann M Maguire ◽  
Shihab Hameed ◽  
...  

Abstract Context P450 oxidoreductase deficiency (PORD) is a rare genetic disorder that is associated with significant morbidity. However there has been limited analysis of reported PORD cases. Objective To determine, based on the cohort of reported PORD cases, genotype-phenotype relationships for skeletal malformations, maternal virilisation in pregnancy, adrenal insufficiency, and disorders of sexual development (DSD). Data Sources PubMed and Web of Science from January 2004 to February 2018. Study Selection Published case reports/series of patients with PORD. Eligible patients were unique, had biallelic mutations, and their clinical features were reported. Data Extraction Patient data were manually extracted from the text of case reports/series. A malformation score, representing the severity of skeletal malformations, was calculated for each patient. Data Synthesis Of the 211 patients published in the literature, 90 were eligible for inclusion. More than 60 unique mutations were identified in this cohort. Four groups of mutations were identified, through regression modeling, as having significantly different skeletal malformation scores. Maternal virilization in pregnancy, reported for 21% of patients, was most common for R457H mutations. Adrenal insufficiency occurred for the majority of patients (78%) and was typically mild, with homozygous R457H mutations being the least deficient. DSD affected most patients (72%), but were less common for males (46XY) with homozygous R457H mutations. Conclusions PORD is a complex disorder with many possible mutations affecting a large number of enzymes. By analyzing the cohort of reported PORD cases, this study identified clear relationships between genotype and several important phenotypic features.


Author(s):  
Murat Sarikaya ◽  
Nesibe Taser ◽  
Zeynal Dogan ◽  
Bilal Ergul ◽  
F. Irsel Tezer ◽  
...  

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