BackgroundHepatic glycogenosis is characterized by excessive glycogen accumulation in hepatocytes and represents a complication of poor controlled type 1 diabetes. It can be caused by excessive insulin doses or recurrent ketoacidosis episodes. Mauriac’s syndrome is a rare disease, which includes short stature, growth maturation delay, dyslipidemia, moon facies, protuberant abdomen, hepatomegaly with transaminase elevation. It has become even less common after the emergence of advances on diabetes treatment, but still exists. Recent reports described glycogenosis without the full spectrum of Mauriac’s syndrome in both adults and children with brittle diabetes. Clinical, laboratory and histological abnormalities are reversible with appropriate glycemic control.Case presentationWe hereby report a case of 11-year-old male who presented with hepatic glycogenosis mimicking Mauriac’s syndrome. The patient was admitted at our Pediatric Diabetes Clinic for marked hepatomegaly, short stature and for the poor metabolic control. Blood investigations and liver tests excluded most of major causes of hepatopathy. A liver biopsy allowed us to make diagnosis of hepatic glycogenosis. To control hyperglycaemia, initially we titrated daily insulin dosage, and then intravenous insulin treatment was practiced with the consequent normalization of liver enzymes.ConclusionMauriac’s syndrome should be considered in subjects with brittle type 1 diabetes and hepatomegaly.

Partial clinical remission (PCR) is a transitory period characterized by the residual endogenous insulin secretion following type 1 diabetes (T1D) diagnosis and introducing the insulin therapy. Scientific interest in PCR has been recently increasing, as this phase could be crucial to preserve functional beta cells after T1D onset, also taking advantage of new therapeutic opportunities. The aim of this study was to assess the frequency, duration and associated factors of PCR in children newly diagnosed with T1D. Our cohort study included 167 pediatric patients aged 13.8 ± 4.1 years. The association of clinical and laboratory factors with the occurrence and duration of PCR was evaluated via logistic regression and multivariable generalized linear model, respectively. PCR occurred in 63.5% of the examined patients. Patients who achieved the remission phase were significantly older, and they had lower daily insulin requirement compared with non-remitters. PCR was positively associated to body mass index (OR = 1.11; p = 0.032), pH value (OR 49.02; p = 0.003) and c-peptide levels (OR 12.8; p = 0.002). The average duration of PCR was 13.4 months, and older age at diagnosis was the only predictor factor. Two years after diagnosis remitter patients had lower HbA1c and daily insulin requirement.