Nephrocalcinosis in a 13-year-old girl with type 1 diabetes mellitus complicated by Mauriac syndrome

Abstract

Mauriac syndrome is a rare complication of poorly-controlled type 1 diabetes mellitus (T1DM) characterised by hepatomegaly, short stature, and pubertal delay. We report the case of a 13-year-old girl with T1DM who presented to our hospital with hyperglycaemia and hepatomegaly, and was also found to have bilateral medullary nephrocalcinosis. It is hypothesised that chronic hyperglycaemia resulting from long-term insulin under-dosage led to chronic acidosis which caused hypercalciuria and consequently, nephrocalcinosis. Poor glycaemic control, Mauriac syndrome and nephrocalcinosis, may be associated with socio-economic difficulties. Both medical optimisation and psychosocial support should be provided to reach optimal glycaemic targets, reverse the features of Mauriac syndrome, and prevent worsening of nephrocalcinosis that could potentially lead to chronic renal impairment.