Mauriac Syndrome in a Nigerian child with Type 1 Diabetes Mellitus: A Case Report

Autor: Ezeani IU, Oyenusi EE
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Annals of Health Research, Vol 7, Iss 4, Pp 403-409 (2021)
Druh dokumentu: article
ISSN: 2476-8642
2536-6149
DOI: 10.30442/ahr.0704-08-150
Popis: A 14-year-old boy with Type 1 Diabetes mellitus (diagnosed at eight years of age) presented with complaints of fever, weight loss, growth failure, pubertal delay, abdominal swelling and discomfort. He was on Premixed insulin (70/30) with inadequate follow-up and poor diabetic control. Examination revealed cachexia, generalised lymphadenopathy, a protuberant abdomen and hepatosplenomegaly. Anthropometry showed a bodyweight of 19.6kg, a height of 116cm and a BMI of 14.1kg/m2, all markedly below the 3rd centile. He had no secondary sexual characteristics: axillary hair stage 1, pubic hair stage 1, penile length of 4.9cm and prepubertal testicular volumes of 3mls bilaterally. At presentation, his random blood glucose was 400mg/dl, and glycosylated haemoglobin was 11.6%. Screening for tuberculosis, human immunodeficiency virus, hepatitis and lymphoproliferative disorders were negative. Other blood investigation findings included leucocytosis, erythrocyte sedimentation rate of 30mm/hr, normal liver function tests, normal serum electrolytes, urea and creatinine. His haemoglobin genotype was AS. Chest radiograph showed features of bronchopneumonia. A presumptive diagnosis of Mauriac Syndrome was made. With the optimisation of glycaemic control, he improved clinically with a weight gain of 5.7kg over four months and resolution of hepatosplenomegaly.
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