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Addison's Disease Associated with Hypokalemia

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Addison's Disease Associated with Hypokalemia Abdalla et al. J Med Case Reports (2021) 15:131 https://doi.org/10.1186/s13256-021-02724-6 CASE REPORT Open Access Addison’s disease associated with hypokalemia: a case report M. Abdalla, J. A. Dave and I. L. Ross* Abstract Background: Primary adrenal insufciency (Addison’s disease) is a rare medical condition usually associated with hyperkalemia or normokalemia. We report a rare case of Addison’s disease, coexisting with hypokalemia, requiring treatment. Case presentation: In this case, a 42-year-old man was admitted to the intensive care unit with a history of loss of consciousness and severe hypoglycemia. His blood tests showed metabolic acidosis, low concentrations of cor- tisol 6 nmol/L (normal 68–327 nmol/L), and high plasma adrenocorticotropic hormone 253 pmol/L (normal 1.6– 13.9 pmol/L), and he was diagnosed with primary adrenal insufciency. Surprisingly, his serum potassium was low, 2.3 mmol/L (normal 3.5–5.1 mmol/L), requiring replacement over the course of his admission. Computed tomography scan of the adrenal glands showed features suggestive of unilateral adrenal tuberculosis. Investigations confrmed renal tubulopathy. The patient responded favorably to cortisol replacement, but never required fudrocortisone. Conclusions: Coexistence of hypokalemia with Addison’s disease is unusual. We recommend investigation of the cause of hypokalemia in its own right, if it occurs with primary adrenal insufciency. Keywords: Addison’s disease, Hypokalemia, Tubulopathy Background is variable. It occurs as a result of a high concentration Addison’s disease may manifest with electrolyte abnor- of proopiomelanocortin, a prohormone of adrenocorti- malities, metabolic derangements, and associated endo- cotropic hormone (ACTH) and melanocyte-stimulating crinopathies, some of which are reversible by replacement hormone (MSH) [2]. Vitiligo may be seen in association with glucocorticoids and mineralocorticoids alone. Te with autoimmune adrenal disease [3]. Te most common clinical features of primary adrenal insufciency are non- laboratory fndings include: hyponatremia, hyperkalemia, specifc, including fatigue, loss of weight, nausea, vomit- and anemia [4]. Hydrocortisone is the mainstay of treat- ing, and salt craving, but shock is the preeminent feature ment, and fudrocortisone is added to replace mineralo- of adrenal crisis [1]. Most patients have hypotension and corticoid defciency. Usually, hydrocortisone doses are postural dizziness, as a result of volume depletion and divided into two or three doses, with the highest dose in loss of vascular reactivity, due to defciency of glucocor- the morning to mimic the circadian rhythm. Monitoring ticoids and mineralocorticoids. A cardinal sign of Addi- of replacement relies on clinical assessment in combina- son’s disease is hyperpigmentation, which presents in tion with normal sodium and potassium concentrations, nearly all patients, but the degree of hyperpigmentation along with plasma renin [5]. We encountered a classical presentation of Addison’s *Correspondence: [email protected] disease, apart from severe hypokalemia, which was an Division of Endocrinology, Department of Medicine, Faculty of Health unexpected fnding. We present a case of a 42-year-old Sciences, Groote Schuur Hospital and University of Cape Town, Private man, whose workup included causes of Addison’s disease Bag X3, J47-85 Old Main Building, Observatory, Cape Town 7935, South Africa and hypokalemia. © The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Abdalla et al. J Med Case Reports (2021) 15:131 Page 2 of 5 Case presentation hydrocortisone 100 mg six hourly and thiamine. He was A 42-year-old man, previously healthy apart from sub- extubated after 24 hours of ventilation. stantial alcohol use, presented with weight loss and On normalization of his serum potassium to fatigue, and no recent history of either nausea, vomit- 3.9 mmol/L (normal 3.5–5.1 mmol/L), his serum ing, or diarrhea. He was seen at his local clinic with a aldosterone was less than 52.9 pmol/L (supine 49.0– profoundly decreased level of consciousness. Retro- 643.0 pmol/L). While convalescing on a general medi- spectively, we were able to elicit that he had experienced cal ward, taking 20 mg of hydrocortisone per day and loss of libido for 4 months prior to his presentation. His while normokalemic with potassium supplementa- Glasgow Coma Score (GCS) was 3/15, laboratory glu- tion, investigation for his hypokalemia revealed a uri- cose was 1.1 mmol/L (normal 4.0–5.4 mmol/L), and nary potassium of 17.4 mmol/L (normal less than insulin level 1.3 mIU/L (normal 2.6–24.9 mIU/L), but no 20 mmol/L), urinary sodium of 113 mmol/L (normal blood was taken to measure C-peptide, sulfonylurea, or less than 20 mmol/L), transtubular potassium gradient IGF-I levels. He received two doses of 50 mL 50% dex- (TTKG) of 8, and urine potassium-to-creatinine [K/Cr] trose intravenously, which restored his blood glucose to ratio of 3.9 mmol/mmol. 12.8 mmol/L, with a marginal improvement in his level of He was started on thyroxine replacement. As his consciousness. blood pressure and serum potassium were normal, a A computed tomography (CT) scan of his brain decision was made to withhold fudrocortisone and revealed no intracranial pathology. His initial biochemis- stop oral potassium replacement. Investigation for try revealed a serum sodium level of 131 mmol/L (nor- renal tubular acidosis, after extubation, showed a nor- mal 136–145 mmol/L), potassium 2.3 mmol/L (normal mal arterial blood pH of 7.39 and urine pH of 6. Moreo- 3.5–5.1 mmol/L), creatinine 81 µmol/L (normal 64.0– ver, Fanconi syndrome was negative, including absent 104.0 µmol/L), and ionized calcium of 1.22 mmol/L (nor- glycosuria, absent aminoaciduria, and urine phos- mal 1.05–1.30 mmol/L). At the time his glucose level was phate-to-creatinine ratio of 1.22 (normal 0.20–3.14) 12.8 mmol/L, a random cortisol level was 6 nmol/L (nor- mmol/mmol creatinine. Investigations for an under- mal 68 nmol/L–327 nmol/L) and simultaneous plasma lying cause of primary adrenal insufciency, includ- adrenocorticotropic hormone (ACTH) was 253 pmol/L ing measurement of adrenocortical antibodies and (normal 1.6–13.9 pmol/L), suggestive of primary hypoad- very-long-chain fatty acids, and an intensive screen renalism, and therefore an ACTH stimulation test was for tuberculosis, were negative. CT scan of the adre- not deemed necessary. His dehydroepiandrosterone sul- nal glands revealed a normal-size right adrenal gland, fate of 0.9 µmol/L (normal 2.4–11.6 µmol/L) was also cor- while the left adrenal gland revealed linear calcifcation roborative of primary adrenal insufciency. In addition, of 827 Hounsfeld units (HU), suggestive of previous thyroid functions revealed a TSH of 16.61 mIU/L (nor- tuberculosis infection. Two separate human immu- mal 0.27–4.20 mIU/L), and free T4 7.6 pmol/L (normal nodefciency virus (HIV) tests were conducted using 12.0–22.0 pmol/L) and antithyroid peroxidase antibod- enzyme-linked immunosorbent assay (ELISA), both ies were 10 U/mL (normal <34 U/mL). His arterial blood of which were negative. Considering his complaint of gas demonstrated a pH of 7.22 (acidotic), PO2 13.8 KPa, reduced libido, we investigated his early-morning tes- PCO2 4.82 KPa, standard HCO 3 15.1 mmol/L, and base tosterone concentrations, which were 7.7 nmol/L (nor- excess –12.3 mmol/L (normal –2 to +2 mmol/L), in mal 8.6–29.0 nmol/L), follicle-stimulating hormone keeping with metabolic acidosis. He was intubated for (FSH) 1.8 IU/L (normal 1.5–12.4 IU/L), and luteinizing airway protection and referred to a tertiary hospital for hormone (LH) 6.2 IU/L (normal 1.7–8.6 IU/L), indicat- intensive care unit management. ing hypogonadotropic hypogonadism. On examination, he was underweight but not emaci- Ten days after admission, the patient was discharged ated, blood pressure was 103/62 mmHg, and he had a from hospital well, on hydrocortisone 20 mg per day regular pulse rate of 98 beats per minute. He appeared and thyroxine, as the abnormal thyroid functions per- clinically dehydrated and was noted to have some mild sisted despite hydrocortisone. At follow-up, his oral increased skin pigmentation of his palmar creases and potassium was tapered and stopped after 6 weeks, but extensor surfaces of his elbows. Cardiovascular exami- he never required mineralocorticoid or testosterone nation was normal. Chest examination while intubated replacement as the latter improved. On follow-up, 3 revealed no wheezes or crepitations. Examination of the months after discharge, his urinary potassium level was abdomen was normal, and neurological review demon- 51.6 mmol/L (normal less than 20 mmol/L) and urinary strated no lateralizing signs.
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