Management of a Patient with Addison’s Disease, Chronic Malnutrition, and Irritable Bowel Syndrome Presenting with Neurological and Heart Failure: A Case Report

by kh-ima-admin | July 10, 2024 8:56 am

Abstract

This case report describes the presentation, diagnosis, and successful management of a patient with Addison’s disease complicated by chronic malnutrition and irritable bowel syndrome (IBS). The patient presented with neurological symptoms and heart failure, which were addressed through antifailure measures, hormonal therapy, intravenous (IV) supplementation of vitamins, and re-initiation of feeding.

Introduction

Addison’s disease is a rare endocrine disorder characterized by adrenal insufficiency. Chronic malnutrition and IBS can complicate the clinical picture, leading to a range of systemic issues, including neurological and cardiovascular complications.

The intersection of Addison’s disease and thiamine deficiency, although uncommon, poses significant diagnostic and therapeutic challenges. This article explores the link between Addison’s disease and thiamine deficiency, highlighting the importance of comprehensive nutritional assessment and management in these patients.

Case Presentation

Patient Information: 50 years old female with a background history of Addison’s and irritable bowel syndrome not on any medications, presented with Chief Complaints of chronic backache, with loose stools and later developed drop in GCS.

Investigations

Blood investigations– hypokalemia with leucopenia.

Initial echo on admission showed severe PAH (63 mm/hg) with normal LV function. Repeat echo done 5 days later showed global hypokinesia with regional wall motion abnormalities (EF – 45%). Echo at the time of discharge showed complete resolution of pulmonary artery hypertension (23 mm/hg) with normal LV.

Management and Outcome

Initial Management:

Stabilization with IV fluids and re initiation of enteral feeding with regular electrolytes (K+,Mg, PO) monitoring for the possiblity of refeeding syndrome. Intravenous hydrocortisone and fludrocortisone were started initially. Intravenous high dose thiamine with other multivitamins. Antifailure measures- Diuretics, beta-blockers, pulmonary vasodilators.

Fludrocortisone was stopped later and hydrocortisone was tapered and continued. Successful transition to oral intake with a balanced diet and continued nutritional supplements.

Outcome:

The patient showed significant clinical improvement in both neurological and complete resolution to normal cardiovascular status. Stabilized on long-term medication regimen for Addison’s disease.

Discussion:

This case illustrates the complexity of managing a patient with multiple interrelated  conditions. Thiamine plays a key role in several enzyme and metabolic pathways, including involvement with transketolase and glucose metabolism. Prolonged thiamine deficiency depletes the body stores within 2-3 weeks. Thiamine deficiency can affect the cardiovascular, nervous, and immune systems, as commonly seen in wet beriberi, dry beriberi, or Wernicke-Korsakoff syndrome.

Wernicke’s encephalopathy is an acute neuropsychiatric disorder that occurs as a result of thiamine (vitamin B1) deficiency. About 80-85% of patients who survive develop a chronic disorder of severe memory deficits with amnestic states that include learning defects and short term memory loss. This condition, known as Korsakoff’s psychosis, often follows Wernicke’s encephalopathy, which collectively is known as Wernicke-Korsakoff Syndrome (WKS). Treatment for WKS involves administration of thiamine, even though firm consensus on the optimal dose, frequency, route and duration has not yet been established. Typical suggested regimens include high-dose thiamine (≥500 mg) prescribed intravenously three times a day for two to three days initially with additional treatment doses based on clinical response.

Cardiovascular manifestations of thiamine deficiency include high-output cardiac failure, peripheral vasodilation, arteriovenous shunting of blood, activation of the reninangiotensin-aldosterone system, dyspnea, edema, and dysrhythmias (wet beriberi).12,26 The fulminant Shoshin form of wet beriberi is characterized by a low cardiac ejection fraction, peripheral vasoconstriction, cold periphery, severe lactic acidosis, profound hypotension, Torsades des Pointes, cyanosis, biventricular heart failure, refractory cardiovascular collapse, and death within hours if not treated immediately with parenteral thiamine. Despite the general belief that the output is high in beriberi heart, the cardiac output and ventricular filling pressure are extremely variable. The work of both ventricles is increased in thiamine deficiency, but that of the right rises more than that of the left. Only seven cases of thiamine-responsive pulmonary hypertension have been reported, including six from East Asia and one from Brazil.

Apart from dyselectrolytenemia adrenal crisis can cause varying from angina pectoris, myocardial stunning or hibernation, takotsubo heart disease to ST-segment elevation myocardial infarction with segmental dyskinesia of the ventricular wall in echocardiography. Overall, adrenal insufficiency can impair cardiovascular system in terms of low blood volume, low blood pressure, increased inflammatory factors, dyslipidemia, coronary calcification, arterial thrombosis, etc.

In summary, the present report revealed that thiamine deficiency can present with pulmonary hypertension along with cardiac failure and neurological effects aggravated by pre-existing Addison’s disease.

Successful outcome highlights the importance of a multidisciplinary approach, including prompt correction of electrolyte imbalances, appropriate use of antifailure measures, and careful nutritional support. The re-initiation of enteral feeding was crucial in addressing chronic malnutrition and supporting overall recovery.

Conclusion:

Patients with Addison’s disease and comorbid conditions like chronic malnutrition and IBS require comprehensive and coordinated care. Early recognition and management of complications such as neurological and heart failure are critical for improving patient outcomes. This case underscores the need for vigilant monitoring and tailored therapeutic strategies in managing complex medical conditions.

References:

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Dr Ramapriya
Critical Care
Kauvery Hospital, Chennai

Source URL: https://www.kauveryhospital.com/ima-journal/ima-journal-july-2024/management-of-a-patient-with-addisons-disease-chronic-malnutrition-and-irritable-bowel-syndrome-presenting-with-neurological-and-heart-failure-a-case-report/