Mutations in hepatocyte nuclear factor-1 beta ( HNF1B ) are the most commonly identified genetic cause of renal malformations. Heterozygous mutations are associated with renal cysts and diabetes syndrome. Various renal developmental abnormalities and maturity-onset diabetes of the young could be the presenting factors of these mutations. A 10-year-old boy who was evaluated for bilateral cystic kidneys and chronic kidney disease from the newborn period was diagnosed with HNF1B -related glomerulocystic disease by DNA sequencing. The differential diagnosis of autosomal dominant polycystic kidney disease was a diagnostic pitfall. The genetic screening of the family revealed his mother, sister, and brother to have the same mutation. Therefore, genetic diagnosis and counseling are important for cystic kidney diseases not only for formulating the diagnosis and early management plan but also for the diagnosis of potential asymptomatic cases in the family.

Background and aims: We aimed to evaluate the effects of maternal obesity or gestational diabetes on body composition, lipid, and glucose metabolism, arterial morphology, and functions in children, and to investigate these effects in terms of cardiometabolic diseases. Methods: The study group was composed of 48 children who had a history of gestational diabetes or maternal obesity, and the control group was composed of 33 children. Echocardiographic assessments were performed. Socio-economic status and education level of mothers were obtained. Results: In the study group, carotid intima-media thickness, epicardial adipose tissue thickness, and arterial stiffness values were found to be significantly higher compared to the control group (p < 0.001, p < 0.001, p = 0.003, respectively), while arterial distensibility and arterial strain values were found to be significantly lower (p = 0.003, p = 0.008, respectively). Among the children who had similar body mass index in both groups, children in the study group had higher carotid intima-media thickness and epicardial adipose tissue thickness values. Arterial stiffness values were significantly reduced (p = 0.028) and arterial distensibility and strain values were significantly increased (p = 0.039, p = 0.033, respectively) in the children whose mothers had gestational diabetes and high socio-economic status. Left ventricular mass and left ventricular end-diastolic internal thickness were found to be significantly increased in the children who had obese and unemployed mothers (p = 0.04, p = 0.03, respectively). Conclusion: Low socio-economic status was found to be associated with increased maternal obesity and gestational diabetes. Poor socio-economic status, poor glycaemic control and being overweight during pregnancy indicate negative cardiometabolic outcomes for children in the long term.