Transient Neonatal Diabetes Mellitus and Seizure with an Unknown Etiology.

Neonatal diabetes mellitus (NDM) is a monogenic form of diabetes, usually occurring in the first 6 months of life. Here, we present a newborn, which was admitted with epileptic seizure on the postnatal second day of life. Sepsis and meningitis were ruled out. Cranial imaging and electroencephalography revealed normal. She developed transient NDM on the follow-up and was diagnosed to carry an ABCC8 mutation. Although the neurological features are more common in patients with KCJN11 mutations, patients with ABCC8 mutations could also represent with subtle neurodevelopmental changes or even with epileptic seizures. The genetic testing and appropriate therapy is important in this patient group for predicting clinical course and possible additional features.

Evaluation of the nutritional status of children with type 1 diabetes and their healthy siblings.

BACKGROUND: Although there are studies evaluating the psychological adjustment of healthy children when their siblings have type 1 diabetes mellitus (T1DM), no study evaluating their nutritional status exists. Thus, this study aimed to determine the nutritional status of children with T1DM and their healthy siblings. MATERIAL AND METHODS: Data from a control group, healthy siblings, and patients who were followed and treated for T1D in the Paediatric Gastroenterology and Paediatric Endocrinology outpatient clinics of the Health Sciences University Gülhane Medical Faculty between November 2019 and November 2020 were analysed and compared. The groups were compared in terms of nutritional characteristics, daily macro and micronutrient intakes and classified according to their body mass index (BMI) Z-scores. RESULTS: The study population consisted of 29 children with T1DM (51.7% female, 48.3% male; age: 11.00 ±3.66 years), 36 healthy siblings of children with T1D (50% female, 50% male; age: 9.61 ±4.84 years), and a control group of 58 healthy children (51.7% female, 48.3% male; age: 10.68 ±3.01years).The BMI Z-score of 28.6%of healthy siblings and 25% of children with T1D was > 1 SD or overweight. All of the control group children were of normal weight. None of the children were obese; however, the overweight rate was significantly higher in the healthy siblings and diabetes groups compared to the control group (p = 0.012). Daily energy intake (%) was significantly higher in the control group than in the healthy siblings (p < 0.001). CONCLUSIONS: A quarter of the children with T1D over five years of age were overweight. In addition, healthy siblings were found to have higher BMI Z-scores than controls. This is the first study to evaluate the nutritional status of siblings of patients with T1D and will hopefully lead to more comprehensive studies that will also assess their daily exercise and physical activity.

Impact of presence of paediatric palliative care units in hospitals on the confidence, knowledge and attitudes of paediatricians: the case of Turkey.

Background: Paediatric palliative care (PPC) focuses on improving the quality of life of children dealing with life-threatening conditions, as well as their families. Aims: To evaluate the knowledge and attitudes of paediatricians regarding palliative care in Turkey and the impact of PPC units on their confidence and symptom management abilities. Methods: This was a multicentre descriptive study conducted in 2019. A questionnaire consisting of 24 questions and 4 parts on palliative care was prepared. Paediatricians in hospitals with or without PPC units completed the questionnaire. Analyses were performed using NCSS 10 (2015) software. Results: There were 199 participants in the study, out of which 55 (27.6%) received palliative care training. One hundred and sixty-seven (83.9%) paediatricians defined palliative care as improving the quality of life of patients in the terminal period, and 77 (38.7%) stated that palliative care can be started after diagnosis. The groups of patients who would benefit from palliative care were most frequently identified as those with diseases that could not be cured (e.g. cystic fibrosis). Paediatricians with a PPC unit in their work environment, compared with those without a PPC unit, were significantly more competent in pain management (36.8% vs 6.4%, P < 0.001), symptom management (42.1% vs 19.2%, P < 0.001), and coping with the psychosocial problems of end-stage paediatric patients (36.8% vs 8.4%, P < 0.001). Conclusion: PPC units in hospitals contributed to paediatricians' ability to manage symptoms and communicate with families. The number of PPC units should be increased, especially in developing countries such as Turkey.