Maturity-Onset Diabetes of the Young (MODY) is a monogenic form of diabetes, consisting of a heterogeneous group of autosomal dominant inherited disorders. Typical onset is in individuals prior to twenty five years, and presentation can mimic type 1 or 2 diabetes. Molecular genetic testing can allow precise identification of the different MODY sub-types. Making a specific diagnosis of MODY can have important implications for the guidance of appropriate treatment, prognosis and genetic counselling.We present the cases of three children and their families diagnosed with MODY over the past two years. These families highlight the features of three of the more common MODY subtypes, including two with novel mutations, one of which segregates in a kindred that is strongly affected by both MODY and classic autoimmune mediated diabetes. To date, we have identified a prevalence of MODY in the paediatric diabetes population of the lower South Island, New Zealand, of approximately 2.5%. This prevalence, along with increasing access to molecular genetic testing, highlights the importance of consideration of MODY in atypical diabetes presentations in the paediatric/adolescent population.

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<b><i>Background:</i></b> The objective of this study was to determine whether ventilator bias gas flow affects tracheal aspirate (TA) cytokine concentrations in ventilated extremely preterm infants. <b><i>Methods:</i></b> This is a randomized controlled trial in a tertiary neonatal unit in New Zealand. Preterm infants (&#x3c;28 weeks’ gestation/&#x3c;1,000 g) requiring intubation in the first 7 days after birth were randomized to bias gas flows of 4 or 10 L/min. Cytokine concentrations in TA and plasma were measured at 24, 72, and 120 h after the onset of ventilation. The primary outcome measure was concentration of interleukin (IL)-8 in TA 24 h after the onset of mechanical ventilation. <b><i>Results:</i></b> Baseline demographics were similar in babies randomized to 4 (<i>n</i> = 50) and 10 (<i>n</i> = 45) L/min bias gas flow. TA IL-8 concentrations were not different between groups. Plasma IL-8 concentrations decreased over time (<i>p</i> &#x3c; 0.05). Respiratory support and incidence of bronchopulmonary dysplasia at 36 weeks’ corrected gestational age were similar between groups. Fewer babies ventilated at 4 L/min developed necrotizing enterocolitis (NEC) ≥ stage 2 (<i>n</i> = 0 vs. <i>n</i> = 5; <i>p</i> = 0.02) and fewer died (<i>n</i> = 1 vs. <i>n</i> = 5, <i>p</i> = 0.06). <b><i>Conclusions:</i></b> Lower bias gas flow in ventilated extremely preterm infants did not alter TA cytokine concentrations but the lower incidence of NEC and mortality warrants further investigation.

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