An hepatomegaly in a child with type 1 diabetes and celiac disease: what could it be?

Yasmine Fakhfekh; Chaima Jemai; Hmida Yosra Ben; Yosra Htira; Salem Dorsaf Ben; Takwa Moussa; Ali Zohra Hadj; Imen Hedfi; Olfa Lajili; Mami Faika Ben

doi:10.1530/endoabs.99.P297

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Endocrine Abstracts (2024) 99 P297 | DOI: 10.1530/endoabs.99.P297

ECE2024 Poster Presentations Diabetes, Obesity, Metabolism and Nutrition (130 abstracts)

An hepatomegaly in a child with type 1 diabetes and celiac disease: what could it be?

Yasmine Fakhfekh ¹ , Chaima Jemai ¹ , Yosra Ben Hmida ¹ , Yosra Htira ¹ , Dorsaf Ben Salem ¹ , Takwa Moussa ¹ , Zohra Hadj Ali ¹ , Imen Hedfi ¹ , Olfa Lajili ¹ & Faika Ben Mami ¹

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¹National institute of nutrition of Tunis, C

Introduction: Type 1 diabetes mellitus (T1DM) and celiac disease are two frequently associated autoimmune pathologies. The association with hepatomegaly raises discussion of many diagnosis.

Case presentation: We report the case of a 12-years-old T1DM girl admitted at department C of the national institute of nutrition of Tunis for diabetic ketosis. She has T1DM since 5 years, with poor glycemic control (HbA1C=10%) and poor therapeutic adherence. On examination, she had abdominal bloating with homogeneous hepatomegaly. Height was 146 cm (less than 2nd percentile), weight 38 kg (less than 2nd percentile) and body mass index of 17.8 Kg/m². Tanner stage was S1P1A1. Biological investigations showed, mixed dyslipidemia, normal liver and renal functions and normal TSH. AST/ALT ratio was 1.35. FSH and LH levels were low. Ultrasound abdomen revealed hepatomegaly with a liver span of 19 cm. Based on the clinical history and investigations, Mauriac syndrome seems to be the most likely diagnosis of our patient. A holistic multidisciplinary approach, in collaboration with the child psychiatrist, was opted, in order to optimize diabetes management and to reduce hepatic metabolic overload. Further investigations was conducted to rule out differential diagnoses, especially viral and autoimmune hepatitis.

Conclusion: Poor acceptance of type 1 diabetes leads to non-compliance with insulin therapy. Then, energy metabolism becomes defective with growth retardation and pubertal delay. Glucose accumulates in the liver leading to metabolic liver disease. Liver damage could be irreversible. Therapeutic education, good doctor-patient relationship and family support constitute the main cornerstones of the management of T1DM diabetes complicated by Mauriac syndrome.