DOCSLIB.ORG

Conduct Protocol in Emergency: Acute Adrenal Insufficiency

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Conduct Protocol in Emergency: Acute Adrenal Insufficiency ORIGINAL ARTICLE FARES AND SANTOS Conduct protocol in emergency: Acute adrenal insufficiency ADIL BACHIR FARES1*, RÔMULO AUGUSTO DOS SANTOS2 1Medical Student, 6th year, Faculdade de Medicina de São José do Rio Preto (Famerp), São José do Rio Preto, SP, Brazil 2Degree in Endocrinology and Metabology from Sociedade Brasileira de Endocrinologia e Metabologia (SBEM). Assistant Physician at the Internal Medicine Service of Hospital de Base. Researcher at Centro Integrado de Pesquisa (CIP), Hospital de Base, São José do Rio Preto. Endocrinology Coordinator of the Specialties Outpatient Clinic (AME), São José do Rio Preto, SP, Brazil SUMMARY Introduction: Acute adrenal insufficiency or addisonian crisis is a rare comor- bidity in emergency; however, if not properly diagnosed and treated, it may progress unfavorably. Objective: To alert all health professionals about the diagnosis and correct treatment of this complication. Method: We performed an extensive search of the medical literature using spe- cific search tools, retrieving 20 articles on the topic. Results: Addisonian crisis is a difficult diagnosis due to the unspecificity of its signs and symptoms. Nevertheless, it can be suspected in patients who enter the emergency room with complaints of abdominal pain, hypotension unresponsive to volume or vasopressor agents, clouding, and torpor. This situation may be associated with symptoms suggestive of chronic adrenal insufficiency such as hyperpigmentation, salt craving, and association with autoimmune diseases such as vitiligo and Hashimoto’s thyroiditis. Hemodynamically stable patients Study conducted at Faculdade may undergo more accurate diagnostic methods to confirm or rule out addiso- de Medicina de São José do nian crisis. Delay to perform diagnostic tests should be avoided, in any circum- Rio Preto (Famerp), São José do Rio Preto, SP, Brazil stances, and unstable patients should be immediately medicated with intravenous glucocorticoid, even before confirmatory tests. Article received: 9/3/2015 Accepted for publication: 9/28/2015 Conclusion: Acute adrenal insufficiency is a severe disease that is difficult to -di agnose. It should be part of the differential diagnosis in cases of hypotensive patient *Correspondence: Address: Av. Deputado Emílio Carlos, 586 who is unresponsive to vasoactive agents. Therefore, whenever this complication São Paulo, SP – Brazil is considered, health professionals should aim specifically at this pathology. Postal code: 02721-100 [email protected] Keywords: acute adrenal insufficiency, Addison’s disease, addisonian crisis, http://dx.doi.org/10.1590/1806-9282.62.08.728 adrenal crisis. INTRODUCTION eosinopenic effects. Among the many adrenal androgens, The adrenal are glands divided in two histological areas: the main one is dehydroepiandrosterone (DHEA), which cortex and medulla. The adrenal cortex is composed of has weaker effect in humans, contributing to the develop- three layers: zona fasciculata, zona glomerulosa and zona ment of secondary sexual characteristics.1,2,6 reticularis, producing mainly cortisol, aldosterone, and Given the importance of adrenal hormones, severity estrogens and androgens, respectively.1 of adrenal insufficiency (AI) or adrenocortical becomes Aldosterone, a potent mineralocorticoid, is very im- clearer. It is clinically manifested in two forms: acute and portant for the control of blood pressure and volume, by chronic. In addition, AI can be further divided into: pri- increasing sodium reabsorption and potassium secretion mary AI, known as Addison’s disease, a result of destruction in the distal tubules of the kidneys. Cortisol is a glucocor- of 90% or more of the adrenocortical gland or conditions ticoid of great relevance in body stress, acting on the in- that involve the decreased production of adrenal steroids, crease of blood glucose, on plasma concentrations of resulting in subnormal synthesis of aldosterone, cortisol, proteins and lipids, and as an anti-inflammatory agent. At and androgens; and secondary and tertiary AI, which have high concentrations, it has relevant lymphopenic and their pathophysiology linked to deficient secretion of cor- 728 Rev ASSOC Med BRAS 2016; 62(8):728-734 CONDUCT PROTOCOL IN EMERGENCY: ACUTE ADRENAL INSUFFICIENCY ticotropin (ACTH) and corticotropin releasing hormone and the diagnosis of possible acute AI in humans (some (CRH), respectively.2,17 ACTH is a hormone secreted by articles refer to this pathology in animals) were includ- corticotropic cells of pars distalis in the hypophysis. It is ed, while those that did not follow this logic were ex- upregulated by CRH, which is produced in the paraven- cluded from the analysis. 175 articles were found ini- tricular nucleus of the hypothalamus (Figure 1). tially, of which we selected 20. For better anatomical and pathophysiological understanding of the subject, METHOD important books in the academic circles were also used Between the months of April and June 2015, we performed as a source of knowledge. extensive literature search and review to find the most current articles on acute adrenal insufficiency (acute AI). ACUTE ADRENAL INSUFFICIENCY (ADDISONIAN Initially, we selected synonyms for acute AI, including: CRISIS OR ADRENAL CRISIS) addisonian crisis and adrenal crisis, which were used as The addisonian crisis is a relatively uncommon endocrine keywords in our search for articles and dissertations from emergency that translates to serious risk of death if not databases such as Uptodate, Pubmed, and Scielo. diagnosed and treated in time. It is usually seen in patients This study aimed to find and review the most recent with chronic AI exposed to acute stress such as trauma, articles in English or Portuguese on the subject studied, surgery, infections, and dehydration. Other causes are but without being oblivious to very important articles displayed in Chart 1. 3,7,8,11,16-18 that, though older, bring useful and still prevalent in- The physician should always be alert to any changes formation on the subject. Therefore, all relevant articles in the vital signs of a patient with known chronic AI. Be- that contributed with solid knowledge regarding conduct ing aware and knowing how to correlate certain signs and Normal axis Primary adrenal insufficiency Hypothalamus Hypothalamus CRH CRH Hypophysis Hypophysis ACTH ACTH Adrenal Adrenal Cortisol Secondary and tertiary adrenal insufficiency Hypothalamus Hypothalamus CRH CRH Hypophysis Hypophysis ACTH ACTH Adrenal Cortisol Adrenal Cortisol FIGURE 1 Schematic comparison of the adrenal physiology and pathophysiology of the adrenal insufficiency. CRH: corticotropin-releasing hormone; ACTH: adrenocorticotropic hormone. Rev ASSOC Med BRAS 2016; 62(8):728-734 729 FARES AND SANTOS The clinical picture that allows us to suspect a second- CHART 1 Triggering factors of adrenal insufficiency. ary AI is: pallor, but with no changes in red blood cells Infections that could suggest anemia, and symptoms of hypogonad- Surgeries ism (loss of axillary and pubic hair, loss of libido, and Dehydration amenorrhea in women). In secondary AI, usually there is Lack of early diagnosis no aldosterone deficiency because the renin-angiotensin Improper adjustment of glucocorticoid dose, or abrupt withdrawal system is intact; however, it may be associated with hy- in chronic users ponatremia due to reduced water clearance resulting from Isolated and hasty replacement of thyroid hormones in patients with hypocortisolism.3,4 The most common manifestations hypothyroidism and hypocortisolism of acute AI can be seen in Chart 2. Use of drugs that inhibit adrenal steroidogenesis (ketoconazole, aminoglutethimide, metyrapone, mitotane, etc.) CHART 2 Clinical manifestations of acute Bilateral adrenal hemorrhage adrenal insufficiency. Meningococcemia (Waterhouse-Friderichsen syndrome) High fever Abdominal pain Use of anticoagulants Tachycardia or bradycardia Apathy and tiredness Trauma or abdominal surgeries Dehydration Nausea and vomiting Sepsis due to other bacteria (P. aeruginosa) Hypotension and shock Anorexia Coagulopathies Cyanosis or paleness Malaise Leukemia Petechiae and ecchymosis (Waterhouse- Constipation Metastasis -Friderichsen syndrome) Sequel from bilateral venography Faintness, numbness Myalgia and arthralgia Primary antiphospholipid syndrome Coma Syncope Acute myocardial infarction Hypoglycemia LABORATORY TESTS Some immediate laboratory changes may be observed symptoms that suggest acute AI is also necessary, as over with plain doses of Na+1, Ca+2 , K+, blood glucose, and urea. 25% of patients with Addison’s disease are diagnosed at Sodium tends to fall (hyponatremia) and potassium tends the time of an adrenal crisis, probably triggered by a stress- to rise (hypercalcemia) due to decreased aldosterone. There ful event (surgery, infection or trauma).4,16,18 is a drop in blood glucose due to serum decrease of an Adrenal crisis should be included in the differential important hormone counter-regulator of insulin, cortisol. diagnosis when a patient enters the emergency room with Decreased levels of this hormone also lead to an increase hypotension that does not respond to the administration in lymphocytes and eosinophils, as a result of decreased of volume or vasopressors, worsening on standing posi- immune-modulatory action of hydrocortisone. In some tion, sometimes leading to shock, associated with non- cases, mild hypercalcemia (rarely) and uremia (55% of specific symptoms such as anorexia, nausea, vomiting, cases) are observed.3,9,12,18 pain unexplained abdominal
Load more

Recommended publications
  • Congenital Adrenal Hyperplasia in the Newborn
    The Leo Fung Center for CAH and Disorders of Sex Development Congenital Adrenal Hyperplasia in the Newborn Contents Introduction 1 What is congenital adrenal hyperplasia? 1 Types of CAH 3 Diagnosing CAH in newborns 4 Treating CAH 5 Untreated CAH 7 CAH in children and young adults 8 Frequently asked questions 9 Glossary 11 Resources 13 Acknowledgments 14 Congenital Adrenal Hyperplasia in the Newborn 1 Introduction This handbook will provide you and your family information about congenital adrenal hyperplasia (CAH). While this guide will not answer all of your questions, it will provide basic medical facts that will help you to talk to your doctors. It is important to know that CAH cannot be cured but it can be treated. Your child will need to take medicine for the rest of his or her life. If your child takes this medicine, he or she should have a completely normal life in every way. Successful treatment requires teamwork between you and your doctor. The doctor will monitor your child in order to know what dose of medicine is needed. We ask that you give your baby the medication on the schedule recommended by your doctor. Your family is not alone. The Leo Fung Center for CAH and Disorders of Sex Development (DSD) at University of Minnesota Amplatz Children’s Hospital, provides a large network of support, including medical specialists, therapists and counselors who all have expertise in caring for patients with CAH. What is congenital adrenal hyperplasia? Let’s begin by examining each word. • Congenital means existing at birth (inherited). • Adrenal means that the adrenal glands are involved.
    [Show full text]
  • DOI: 10.4274/Jcrpe.Galenos.2021.2020.0175
    DOI: 10.4274/jcrpe.galenos.2021.2020.0175 Case report A novel SCNN1A variation in a patient with autosomal-recessive pseudohypoaldosteronism type 1 Mohammed Ayed Huneif1*, Ziyad Hamad AlHazmy2, Anas M. Shoomi 3, Mohammed A. AlGhofely 3, Dr Humariya Heena 5, Aziza M. Mushiba 4, Abdulhamid AlSaheel3 1Pediatric Endocrinologist at Najran university hospital, Najran Saudi Arabia. 2 Pediatric Endocrinologist at Al yamammah hospital, , Riyadh, Saudi Arabia. 3 Pediatric Endocrinologist at Pediatric endocrine department,. Obesity, Endocrine, and Metabolism Center, , King Fahad Medical City, Riyadh, Saudi Arabia. 4Clinical Geneticist, Pediatric Subspecialties Department, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia. 5 Research Center, King Fahad Medical City, Riyadh , Saudi Arabia What is already known on this topic ? Autosomal-recessive pseudohypoaldosteronism type 1 (PHA1) is a rare genetic disorder caused by different variations in the ENaC subunit genes. Most of these variations appear in SCNN1A mainly in exon eight, which encodes for the alpha subunit of the epithelial sodium channel ENaC. Variations are nonsense, single-base deletions or insertions, or splice site variations, leading to mRNA and proteins of abnormal length. In addition, a few new missense variations have been reported. What this study adds ? We report a novel mutation [ c.729_730delAG (p.Val245Glyfs*65) ] in the exon 4 of the SCNN1A gene In case of autosomal recessive pseudohypoaldosteronism type 1. Patient with PHA1 requires early recognition, proper treatment, and close follow-up. Parents are advised to seek genetic counseling and plan future pregnancies. proof Abstract Pseudohypoaldosteronism type 1 (PHA1) is an autosomal-recessive disorder characterized by defective regulation of body sodium levels.
    [Show full text]
  • ACUTE ADRENAL INSUFFICIENCY by PAUL FOURMAN, M.D., M.R.C.P
    Postgrad Med J: first published as 10.1136/pgmj.29.330.215 on 1 April 1953. Downloaded from 215 ACUTE ADRENAL INSUFFICIENCY By PAUL FOURMAN, M.D., M.R.C.P. Nuffield Department of Clinical Medicine, University of Oxford In acute adrenal insufficiency we are faced with common in patients whose adrenal insufficiency is the interaction of many factors ; if in trying to due to hypopituitarism than in patients with disentangle them I have cut some knots it is for Addison's disease. the sake of brevity. Addison's disease is characterized by a failure to Acute adrenal insufficiency may result from conserve sodium and we usually think of the crisis sudden loss of adrenal function by haemorrhage, of acute adrenal insufficiency as a condition of thrombosis or ablation. More often it-occurs in a shock brought about by salt depletion. This patient with chronic adrenal insufficiency, either might be true in a patient with Addison's disease in the natural course of the illness or following who is in crisis when he first presents ; in him the an injury such as'infection, operation or exposure. crisis is the climax of a long illness during which Acute adrenal insufficiency is characterized by there has been time for the sodium stores to by copyright. prostration and collapse with low blood pressure become depleted. The sodium depletion may and rapid pulse. It is usually accompanied by represent a stress that released the crisis rather gastro-intestinal symptoms: anorexia, vomiting than the immediate cause of the shock-like state. and diarrhoea, and sometimes abdominal pain and Sodium depletion is not an essential feature of hiccup.
    [Show full text]
  • Lyase Deficiency Due to P.R96W Mutation in the CYP17 Gene in a Brazilian Patient
    clinical case report Combined 17α-hydroxylase/17,20- lyase deficiency due to p.R96W mutation in the CYP17 gene in a Brazilian patient Deficiência combinada de 17α-hidroxilase/17,20 liase devido à mutação p.R96W no gene CYP17 em um paciente brasileiro Fabíola Costenaro1, Ticiana C. Rodrigues1, Claudio E. Kater2, Richard J. Auchus3, Mahboubeh Papari-Zareei3, Mauro A. Czepielewski1 SUMMARY 1 Division of Endocrinology, Congenital adrenal hyperplasia (CAH) resulting from 17α-hydroxylase/17,20-lyase deficiency is Hospital de Clínicas de Porto a rare autosomal recessive disease and the second most common form of CAH in Brazil. We Alegre, Universidade Federal do Rio Grande do Sul (UFRGS), describe the case of a Brazilian patient with CYP17 deficiency (17α-hydroxylase/17,20-lyase de- Porto Alegre, RS, Brazil ficiency) caused by a homozygous p.R96W mutation on exon 1 of the CYP17 gene, an unusual 2 Division of Endocrinology genotype in Brazilian patients with this form of CAH. The patient, raised as a normal female, and Metabolism, Department of Medicine, Escola Paulista sought medical care for lack of pubertal signs and primary amenorrhea at the age of 16 years. At de Medicina, Universidade evaluation, the presence of a 46,XY karyotype, hypertension and hypokalemia were observed. Federal de São Paulo (Unifesp/ We emphasize the recognition of CYP17 deficiency in the differential diagnosis of cases of hy- EPM), São Paulo, SP, Brazil 3 Division of Endocrinology and pergonadotrophic hypogonadism and hypertension in young patients who need specific treat- Metabolism, Department of Internal ment for both situations. Arq Bras Endocrinol Metab. 2010;54(8):744-8 Medicine, University of Texas Southwestern Medical Center, USA SUMÁRIO Correspondence to: A hiperplasia adrenal congênita (HAC), em razão da deficiência de 17α-hidroxilase/17,20-liase, Mauro A.
    [Show full text]
  • Preventable Deaths: Panhypopituitarism and Adrenal Insufficiency
    Preventable Deaths: Panhypopituitarism and adrenal insufficiency. What you need to know What is panhypopituitarism? Your child has been diagnosed with a big scary sounding word, and all you can think is: 'What does this mean? and 'Why have I never heard of this?' Simply put, panhypopituitarism means that your child's pituitary gland does not function properly and as a result, your child is deficient in one or several hormones. Some children have congenital panhypopituitarism, meaning they are born with it. Others have acquired panhypopituitarism following an event such as head trauma, brain tumor surgery, or brain radiation. It is rare enough that is entirely possible, in fact, probable, that you will not initially know anyone else with this disorder. What will my child need? Although the diagnosis and condition can seem intimidating, it is very manageable once you understand what is needed. Unfortunately the condition cannot be cured or reversed, but again, it can be effectively managed. Your child will need to take medications to replace the missing hormones. These might include thyroid hormone, growth hormone, cortisol, and/or possibly others. Your doctor will go over these with you, as dosages vary from child to child. Most medications are taken orally, but growth hormone must be taken by daily injection. Your endocrinologist will work with you and your child to achieve the proper dosages and will guide you in how to administer any necessary medications. Why is it sometimes life threatening? What is adrenal insufficiency? Of the hormone deficiencies your child may have, the most critical is cortisol, also known as the 'stress hormone.' Cortisol is essential for life , and is therefore the central focus of this guide.
    [Show full text]
  • Disorders of the Adrenal Glands
    Disorders of the Adrenal Glands Presley Regional Trauma Center Department of Surgery University of Tennessee Health Science Center Memphis, Tennessee Physiology Cortex • Steroid hormones cortisol, aldosterone and androgens are synthesized • Derived from cholesterol • Three histologic zones – glomerulosa (aldosterone), fasiculata and reticularis Cortisol • Secretion is regulated by hypothalamus (CRH) and the pituitary (ACTH) • Exerts effects on intermediary metabolism through variety of cells and tissues • Stimulates hepatic gluconeogenesis and glycogen synthesis Cortisol • Inhibits protein synthesis, increases protein catabolism and fat lipolysis • Inhibits peripheral uptake of glucose in most tissues (liver, brain and RBCs) • Promote collagen loss and impairment of wound healing (inhibit fibroblast activity) Cortisol • Inhibit bone formation • Inhibit numerous anti-inflammatory actions • Critical for cardiovascular stability Androgens • Synthesized primarily in zona reticularis – DHEA • Peripheral conversion to testosterone and dihydrotestosterone • Account for less than 5% of normal male testosterone production Aldosterone • Regulation of ECF volume • Regulation of Na and K balance • Release is stimulated by renin through renin-angiotensin-aldosterone pathway Adrenal Medulla • Derived from neural crest cells • Principal site for catecholamine synthesis • Only site for the enzyme PNMT • Converts NE to EPI Disorders of the Adrenal Cortex Primary Hyperaldosteronism • Conn syndrome • Responsible for HTN in 8 to 12% of pts • Characterized
    [Show full text]
  • Adrenal Crisis: Still a Deadly Event in the 21St Century Troy H.K
    REVIEW Adrenal Crisis: Still a Deadly Event in the 21st Century Troy H.K. Puar, MBBS, MRCP (UK),a,b Nike M.M.L. Stikkelbroeck, MD, PhD,a Lisanne C.C.J. Smans, MD, PhD,c Pierre M.J. Zelissen, MD, PhD,c Ad. R.M.M. Hermus, MD, PhDa aDivision of Endocrinology, Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands; bDepartment of Endocrinology, Changi General Hospital, Singapore; cDepartment of Internal Medicine and Endocrinology, University Medical Center Utrecht, Utrecht, The Netherlands. ABSTRACT Adrenal crisis is a life-threatening medical emergency, associated with a high mortality unless it is appropriately recognized and early treatment is rendered. Despite it being a treatable condition for almost 70 years, failure of adequate preventive measures or delayed treatment has often led to unnecessary deaths. Gastrointestinal illness is the most common precipitant for an adrenal crisis. Although most patients are educated about “sick day rules,” patients, and physicians too, are often reluctant to increase their gluco- corticoid doses or switch to parenteral injections, and thereby fail to avert the rapid deterioration of the patients’ condition. Therefore, more can be done to prevent an adrenal crisis, as well as to ensure that adequate acute medical care is instituted after a crisis has occurred. There is generally a paucity of studies on adrenal crisis. Hence, we will review the current literature, while also focusing on the incidence, pre- sentation, treatment, prevention strategies, and latest recommendations in terms of steroid dosing in stress situations. Ó 2015 Elsevier Inc. All rights reserved. The American Journal of Medicine (2015) -, --- KEYWORDS: Adrenal crisis; Adrenal insufficiency; Crisis; Emergency DEFINITION AND EPIDEMIOLOGY only if metastatic disease is bilateral, with extensive damage 4 In 1855, Thomas Addison first described patients with to the adrenal glands.
    [Show full text]
  • Mechanisms and Clinical Consequences of Critical Illness Associated Adrenal Insufficiency Paul E
    Mechanisms and clinical consequences of critical illness associated adrenal insufficiency Paul E. Marik Purpose of review Abbreviations Adrenal insufficiency is being diagnosed with increasing ACTH adrenocorticotrophic hormone frequency in critically ill patients. There exists, however, ARDS acute respiratory distress syndrome CBG corticosteroid-binding globulin much controversy in the literature as to the nature of this CIRCI critical illness-related corticosteroid insufficiency entity, including its pathophysiology, epidemiology, CRH corticotrophin-releasing hormone HDL high-density lipoprotein diagnosis and treatment. The review summarizes our HPA hypothalamic–pituitary–adrenal current understanding of the causes and consequences of SAS sympatho-adrenal system TNF tumor necrosis factor adrenal insufficiency in critically ill patients. Relevant findings Activation of the hypothalamic–pituitary–adrenal axis with ß 2007 Lippincott Williams & Wilkins the production of cortisol is a fundamental component of 1070-5295 the stress response and is essential for survival of the host. Dysfunction of the hypothalamic–pituitary–adrenal axis with decreased glucocorticoid activity is being increasingly Introduction recognized in critically ill patients, particularly those with The stress system receives and integrates a diversity of sepsis. This condition is best referred to as ‘critical illness- cognitive, emotional, neurosensory and peripheral related corticosteroid insufficiency’. Critical illness-related somatic signals that arrive through distinct
    [Show full text]
  • Anemia LECTURE in INTERNAL MEDICINE for IV COURSE
    Essentials of Diagnosis, Treatment and Prevention of Major Endocrine Diseases: Diseases of the Adrenal Glands. Adrenal Insufficiency. Adrenal Hyperfunction. Hormonally Active Tumors. LECTURE IN INTERNAL MEDICINE FOR IV COURSE STUDENTS M. Yabluchansky, L. Bogun, L. Martymianova, O. Bychkova, N. Lysenko, N. Makienko V.N. Karazin National University Medical School’ Internal Medicine Dept. Plan of the Lecture • Definition • Epidemiology • Risk factors • Etiology • Mechanisms • Classification • Clinical presentation • Diagnosis • Treatment • Prognosis • Prophylaxis • Abbreviations • Diagnostic guidelines http://www.thelancet.com/pb/assets/raw/pb/assets/raw/Lancet/clinical/diseases/Cushing's.jpg http://peninsulamassage.com.au/wp-content/uploads/2012/08/Adrenal_Glands_1.jpg Definition Diseases of the Adrenal Glands • Diseases of the Adrenal Glands are conditions that interfere with the normal functioning of the adrenal glands and may cause hyperfunction (Overactive Adrenal Glands) or hypofunction (Underactive Adrenal Glands), and may be congenital or acquired. • There are two parts of the adrenal glands, the cortex, derived from mesenchymal cells, and the medulla, derived from neuroectodermal cells; first one produces mineralocorticoids, glucocorticoids, and androgens; and second one produces epinephrine (adrenaline) and norepinephrine(noradrenaline). https://en.wikipedia.org/wiki/Adrenal_gland_disorder Epidemiology Epidemiologic study of adrenal gland disorders in Japan • The total numbers of patients in Japan in 1997 were estimated as 1,450
    [Show full text]
  • GLOWM.414493 23/09/2021 This Chapter Should Be Cited As Follows: Lust K, Tellam J, Glob
    The Continuous Textbook of Women\'s Medicine Series ISSN: 1756-2228; DOI 10.3843/GLOWM.414493 23/09/2021 This chapter should be cited as follows: Lust K, Tellam J, Glob. libr. women's med., ISSN: 1756-2228; DOI 10.3843/GLOWM.414493 The Continuous Textbook of Women’s Medicine Series – Obstetrics Module Volume 8 MATERNAL MEDICAL HEALTH AND DISORDERS IN PREGNANCY Volume Editor: Clinical Associate Professor Sandra Lowe, University of New South Wales, Australia Chapter Adrenal Disorders in Pregnancy First published: February 2021 AUTHORS Karin Lust, MBBS, FRACP Director Women’s and Newborn Services, General & Obstetric Physician, Royal Brisbane and Women’s Hospital, Herston, Australia Jane Tellam, MBBS Endocrinology and Obstetric Medicine Advanced Trainee, Department of Endocrinology and Obstetric Medicine, Royal Brisbane and Women’s Hospital, Herston, Australia PHYSIOLOGY OF ADRENAL GLAND FUNCTION IN PREGNANCY There are two major components to the adrenal glands: the cortex and medulla. The adrenal cortex consists of the outermost zona glomerulosa (ZG) which produces mineralocorticoids (aldosterone), the middle zona fasciculata (ZF) which produces glucocorticoids (cortisol) and the zona reticularis which produces androgens (dehydroepiandrosterone, DHEA, and the sulfated version DHEA-S).1,2 The adrenal medulla produces adrenaline and noradrenaline. Cortisol Cortisol is essential for maintaining blood pressure, electrolyte physiology and glycemic control. Ten to fteen per cent circulates freely in non-pregnant women, with the remainder bound to cortisol binding globulin (CBG) and albumin.3 Both free and bound cortisol are elevated throughout pregnancy and spike at delivery. By the third trimester, cortisol levels have increased by 2–3 fold. Diurnal variation is preserved, characterized by high levels of cortisol in the morning and low levels at night.4 Multiple mechanisms are involved (Figure 1):5 1.
    [Show full text]
  • A Case of Waterhouse-Friderichsen Syndrome in a Patient with Streptococcus Pyogenes Bacteremia
    A Case of Waterhouse-Friderichsen Syndrome in a Patient with Streptococcus Pyogenes Bacteremia D. I. BACAL, B. E. CATALDO, P. M. LUCERI, G. VARALLO Rowan University School of Osteopathic Medicine, and Jefferson University Hospital System, NJ INTRODUCTION Waterhouse-Friderichsen Syndrome (WFS) is a rare condition of adrenal insufficiency (AI) due to adrenal hemorrhage after a severe infection. The incidence of WFS has been estimated at 0.14-1.8% based on post-mortem studies.1 It has been associated with a 55-60% mortality rate.2 Meningococcal disease comprises up to 80% of WFS, but additional causative agents continue to be identified.3 This is the case of a 52-year-old female with toxic shock syndrome from Group A Streptococcal (GAS) bacteremia who developed bilateral adrenal hemorrhage & subsequent AI. Septic shock occurred during an initial hospitalization & resolved. Four weeks after discharge she presented with evidence of an adrenal crisis. CT scans demonstrated bilateral adrenal enlargement concerning for adrenal hemorrhage. Primary AI was confirmed via ACTH stimulation testing. A literature search found fewer than ten cases of WFS described due to streptococcal bacteremia. BACKGROUND OF WATERHOUSE-FRIDERICHSEN SYNDROME CONCLUSIONS • Bilateral adrenal hemorrhage has multiple causes: coagulopathies, sepsis from infection, hypotension (i.e. MI), severe volume loss, or surgical WFS is a rare, often fatal, clinical condition that can develop after a intervention 4 severe infection & leads to adrenal hemorrhage. Many factors • Signs include pallor, weakness, fatigue, anorexia, nausea, vomiting, and lethargy 5,6 & labs often depict hyponatremia & hyperkalemia contribute to the pathogenicity of WFS, including coagulopathy, • Increased skin pigmentation develops due to an increase in proopiomelanocortin (POMC), Bacterial ischemia & bacterial toxins.
    [Show full text]
  • The Adrenal Glands
    The Adrenal Glands Thomas Jacobs, M.D. Diane Hamele-Bena, M.D. I. Normal adrenal gland A. Gross & microscopic B. Hormone synthesis, regulation & measurement II. Hypoadrenalism III. Hyperadrenalism; Adrenal cortical neoplasms IV. Adrenal medulla 1 Normal Adrenal Gland • Nldltdlld3545Normal adult adrenal gland: 3.5 - 4.5 grams Adrenal Cortex Morphology • Cortex: 3 zones: – Glomerulosa – Fasciculata – Reticularis 2 Hypoadrenalism 3 Hypoadrenalism • Primary Adrenocortical Insufficiency • Secondary Adrenocortical Insufficiency Hypoadrenalism Clinical Manifestations Primary adrenal insufficiency: Deficiency of glucocorticoids, mineralocorticoids, and androgens 4 Hypoadrenalism Clinical Manifestations Primary adrenal insufficiency: Concomitant hypersecretion of ACTH Hyperpigmentation Hypoadrenalism Clinical Manifestations Secondary adrenal insufficiency: Deficiency of ACTH NO hyperpigmentation 5 Pathology of Hypoadrenalism • PiPrimary Adrenocort ica l IffiiInsufficiency –Acute – Chronic = Addison Disease • Secondary Adrenocortical Insufficiency Pathology of Hypoadrenalism • PiPrimary Adrenocort ica l IffiiInsufficiency –Acute – Chronic = Addison Disease •Autoimmune adrenalitis 6 Addison Disease Clinical findings Mineralocorticoid deficiency Glucocorticoid deficiency •Hypotension •Weakness and fatigue •Hyponatremia •Weight loss •Hyperkalemia •Hyponatremia •Hypoglycemia •Pigmentation Androgenic deficiency •Abnormal H2O metabolism •Loss of pubic and axillary •Irritability and mental hair in women sluggishness Autoimmune Adrenalitis Three settings:
    [Show full text]