Abstract

IntroductionAutoimmune polyendocrine syndrome type 2 is a rare disorder. Its prevalence in western populations has been reported as 1.5 to 4.5/100,000. On the other hand, its prevalence in Egypt is unknown. It is characterized by the association of autoimmune Addison’s disease with thyroid autoimmune diseases and/or type I diabetes mellitus. Hepatitis C virus infection is an important public health issue worldwide. Egypt has the highest prevalence of hepatitis C virus infection of any country in the world. It is estimated to be 8% in urban and 25% in rural areas. We present the case of an Egyptian child with autoimmune polyendocrine syndrome type 2 associated with chronic hepatitis C infection.Case presentationA 14-year-old Egyptian boy with type 1 diabetes mellitus was referred to our institution for an evaluation of recurrent attacks of hypoglycemia of two months duration. The initial clinical examination revealed hypotension as well as vitiligo of the skin. He had high potassium, low sodium, low cortisol, high adrenocorticotropic hormone, slightly high thyroid stimulating levels with strong positivity of anti-thyroglobulin and anti-thyroid peroxidase antibodies. The hepatitis C antibody and hepatitis C virus–polymerase chain reaction were positive. Based on these findings, a diagnosis of autoimmune polyendocrine syndrome type 2 with chronic hepatitis C was made. He was started on hydrocortisone (10mg twice daily), fludrocortisone (0.1mg twice daily) and multiple daily doses of insulin. He showed great improvement of his symptoms on the prescribed treatment.ConclusionsThe importance of the early diagnosis of autoimmune polyendocrine syndrome type 2 and the possibility of its association with chronic hepatitis C infection should be considered in order to implement the proper management of such cases.

Highlights

  • Autoimmune polyendocrine syndrome type 2 is a rare disorder

  • The importance of the early diagnosis of autoimmune polyendocrine syndrome type 2 and the possibility of its association with chronic hepatitis C infection should be considered in order to implement the proper management of such cases

  • We present the case of an Egyptian child who presented with Autoimmune polyendocrine syndrome type 2 (APS2) associated chronic hepatitis C

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Summary

Introduction

Autoimmune polyendocrine syndrome (APS) is a rare syndrome caused by immune-mediated destruction and involves the failure of both endocrine and non-endocrine organs. Apart from post circumcision bleeding that was done at the age of two years by a barber, there was nothing significant in his past medical history On physical examination, his body temperature was 36.1C, pulse rate was 92/min, blood pressure was 80/45mmHg, his height was in the 10th percentile while his weight was in the 25th percentile. The presence of positivity for both anti-HCV and HCV-RNA with elevated ALT and AST levels even in the absence of hepatomegaly confirmed the diagnosis of chronic hepatitis C infection. His general condition improved gradually following the administration of hydrocortisone (10mg twice daily) and fludrocortisone (0.1mg twice daily). He was referred to the regional hepatology unit for further assessment including liver biopsy and consideration of hepatitis C eradication therapy but his father preferred to delay the treatment because he was worried about the side effects of the treatment

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