Objective. We aim to study the prevalence and risk factors of hypovitaminosis D among healthy adolescents in Kota Bharu, Kelantan based on the most recent Paediatric Consensus guideline. Methodology. Ten public schools were selected from Kota Bharu, Kelantan. We analysed their demography (age, gender, ethnicity, income), measured their anthropometry (height, weight, BMI) and finally analysed their vitamin D and intact- Parathyroid hormone levels. Results. The prevalence of hypovitaminosis D was 16.9% among healthy teenagers with mean age of 15.9±1.39 years. Multivariate analysis showed female gender (adjusted OR, 95% CI): 23.7 (5.64, 100.3) and Chinese 0.24 (0.07, 0.84) were the significant predictors for hypovitaminosis D. Conclusion. The prevalence of healthy adolescents with hypovitaminosis D in Kota Bharu, Kelantan was 16.9% using the most recent cut off value of 30 nmol/L from the global consensus 2016. Female and Malay were the significant risk factors associated with hypovitaminosis D. Higher cut off value would result in overestimation of prevalence rate of hypovitaminosis D.
Vitamin D Deficiency ; Rickets ; adolescents

Purpose: There are limited data from Asian countries regarding retinal thickness in children with type 1 diabetes mellitus (T1DM). This study aimed to compare the macular and retinal nerve fiber layer (RNFL) parameters between diabetic children without retinopathy and non-diabetic healthy children. We also evaluated the factors associated with RNFL thickness in children with T1DM. Methods: A comparative cross-sectional study was conducted among children with T1DM and healthy children aged 7 to 17 years old in Hospital Universiti Sains Malaysia from 2017 to 2019. Children with retinal disease or glaucoma were excluded. Macular and RNFL thicknesses were measured using spectral-domain optical coherence tomography. Demographic information, duration of diabetes, blood pressure, body mass index, visual acuity, and retinal examination findings were documented. Glycosylated hemoglobin levels, renal function, and blood lipid levels were also collected. Results: Forty-one children with T1DM and 80 age- and sex-matched children were enrolled. Both sexes were affected. Mean duration of diabetes mellitus was 3.66 years. The mean glycated hemoglobin levels in the T1DM group was 9.99%. The mean macular and RNFL thicknesses in children with T1DM were 277.56 (15.82) µm and 98.85 (12.05) µm, respectively. Children with T1DM had a significantly thinner average macula, superior outer macula, nasal outer macula, mean RNFL, and inferior RNFL thickness compared to controls (p < 0.05). There was a significant association between nephropathy and the mean RNFL thickness. Conclusions Children with T1DM had significantly decreased mean macular and RNFL thicknesses. Nephropathy is associated with an increased RNFL thickness.

Purpose: There are limited data from Asian countries regarding retinal thickness in children with type 1 diabetes mellitus (T1DM). This study aimed to compare the macular and retinal nerve fiber layer (RNFL) parameters between diabetic children without retinopathy and non-diabetic healthy children. We also evaluated the factors associated with RNFL thickness in children with T1DM. Methods: A comparative cross-sectional study was conducted among children with T1DM and healthy children aged 7 to 17 years old in Hospital Universiti Sains Malaysia from 2017 to 2019. Children with retinal disease or glaucoma were excluded. Macular and RNFL thicknesses were measured using spectral-domain optical coherence tomography. Demographic information, duration of diabetes, blood pressure, body mass index, visual acuity, and retinal examination findings were documented. Glycosylated hemoglobin levels, renal function, and blood lipid levels were also collected. Results: Forty-one children with T1DM and 80 age- and sex-matched children were enrolled. Both sexes were affected. Mean duration of diabetes mellitus was 3.66 years. The mean glycated hemoglobin levels in the T1DM group was 9.99%. The mean macular and RNFL thicknesses in children with T1DM were 277.56 (15.82) µm and 98.85 (12.05) µm, respectively. Children with T1DM had a significantly thinner average macula, superior outer macula, nasal outer macula, mean RNFL, and inferior RNFL thickness compared to controls (p < 0.05). There was a significant association between nephropathy and the mean RNFL thickness. Conclusions Children with T1DM had significantly decreased mean macular and RNFL thicknesses. Nephropathy is associated with an increased RNFL thickness.

Developmental delay, Epilepsy and Neonatal Diabetes (DEND) syndrome is the most severe form of Permanent Neonatal Diabetes with KCNJ11 gene mutation which accounts for most of the cases. We report the first DEND syndrome in Malaysia with heterozygous missense mutation Q52R at KCNJ11 (Kir6.2) gene with delayed presentation beyond 6 months of age and failure to transition to glibenclamide. This report signifies the phenotypical variability among patients with the same genetic mutation and the different response to treatment.
Glyburide

Objectives: This study described and compared glycaemic changes with the use of the following Continuous Glucose Monitoring (CGM) metrics: time in range, time in hyperglycaemia and time in hypoglycaemia from retrospective CGM data among children and adolescents with Type 1 Diabetes Mellitus (T1DM), before and during Ramadan to better understand the impact of fasting during this season. Methodology: This study was conducted in 2 tertiary centres: Hospital Putrajaya (HPJ) and Hospital Universiti Sains Malaysia (HUSM) from February to May 2020. Muslim T1DM patients between ages 8 to18 who intended to fast during Ramadan were given Ramadan-focused education. CGM iPro2® (Medtronic) was used before and during Ramadan, complemented by finger-prick glucose monitoring or self-monitoring of blood glucose (SMBG). Results: Of the 32 patients, only 24 (12 female) were analysed. Mean age was 13.6 ± 3.1 years old, mean HbAlc was 9.6 ± 1.9% and mean duration of illness was 5.4 ± 3.4 years. Majority (91.7%) were on multiple dose injections (MDI) while only 8.3% were on continuous subcutaneous insulin infusion (CSII). All fasted in Ramadan without acute complications. Retrospective CGM analysis revealed similar results in time in range (TIR), time in hyperglycaemia and time in hypoglycaemia before and during Ramadan, indicating no increased hypoglycaemic or hyperglycaemic events related to fasting. Glycaemic variability before Ramadan as measured by the LBGI, HBGI and MAG, were similar to values during Ramadan. Conclusion Ramadan fasting among T1DM children and adolescents, by itself, is not associated with short-term glycaemic deterioration. T1DM youths can fast safely in Ramadan with the provision of focused education and regular SMBG.

Purpose: The aim of this study is to determine the mean central corneal thickness (CCT) and mean intraocular pressure (IOP) in children with type 1 diabetes mellitus (T1DM) and to determine the relationship between CCT and IOP on the one hand and age, sex, retinopathy hemoglobin A1c (HbA1c), and duration of diabetes on the other. Methods: This is a case-control, hospital-based study conducted at Hospital Universiti Sains Malaysia between January and November 2022. Thirty-eight children with T1DM were recruited as cases, and 38 healthy children were recruited as controls. The cases and controls then underwent ophthalmic examination, IOP measurement, and CCT measurement using optical coherence tomography (OCT) of the right eye. The IOP measurements were adjusted for CCT for further analysis. Results: The means of CCT and IOP values were significantly higher in the T1DM group than in the control group (all p = 0.02). The mean CCT was 542.18 ± 20.40 μm in the T1DM group, and 529.52 ± 26.17 μm in the control group. The mean IOP was 14.68 ± 1.98 mmHg in the T1DM group, and 13.52 ± 1.66 mmHg in the control group. The mean HbA1c was 10.68% ± 2.49% in the T1DM group. Age and duration of DM were found to have a significant association with CCT in children with T1DM. The duration of DM was also found to be significantly associated with the IOP. Sex and HbA1c levels were found to have no significant relationship with either CCT or IOP. Conclusions Children with T1DM have significantly higher CCT and IOP than the average child. The duration of DM is a significant factor that impacts both CCT and IOP. In addition, age is another factor that affects CCT in children with T1DM.

A 2.4 kg baby boy born via Caesarian section at 35 weeks had the first onset of hypoglycemia at 2 hours of life. The infant required a glucose load of 30 mg/kg/min. Insulin level was 19.6 pmol/L (normal value 17.8-173.0) in the absence of ketosis. He was resistant to oral diazoxide but responded to octreotide infusion. The boy was found to be heterozygous for an ABCC8 nonsense mutation, p.R934*. We present our experience on the use of subcutaneous octreotide for 2 years for the treatment of diazoxide resistant congenital hyperinsulinism (CHI).

Male ; Infant ; Codon, Nonsense ; Congenital Hyperinsulinism ; Diazoxide ; Glucose ; Infant ; Insulins ; Ketosis ; Octreotide ; Parturition ; Pregnancy ; Mutation

Objective: The study aims to determine the prevalence and risk factors for endocrine disorders in childhood brain tumour survivors. Methodology: 124 childhood brain tumour survivors aged 18 years old or younger with either stable disease or in remission, and had survived for at least 2 years after diagnosis were included in the study. Demographic data (age at diagnosis, gender, ethnicity, socioeconomic status), clinical clues for endocrine disorders, anthropometrics (weight, height, midparental height), pubertal staging, tumour-related characteristics, treatment modalities and endocrine laboratory measurements at diagnosis and during follow up were obtained. Logistic regression was applied to evaluate risk factors for endocrine disorders in childhood brain tumour survivors. Results: The prevalence of endocrine disorders in childhood brain tumour survivors was 62.1%. The risk factors were high BMI [adjusted odds ratio (OR) 1.29, 95% CI: 1.12 to 1.5], high-risk site [adjusted odds ratio (OR) 7.15, 95% CI: 1.41 to 36.3] and chemotherapy [adjusted odds ratio (OR) 0.18 , 95% CI: 0.05 to 0.62]. Conclusion The prevalence of endocrine disorders in childhood brain tumour survivors in our centre was 62.1%. The significant risk factors were high BMI, tumour location (suprasellar and intrasellar) and chemotherapy.
Risk Factors

A four-year-old female who was born term via spontaneous vaginal delivery with a birth weight of 3.4 kg had an onset of persistent hypoglycaemia at the 6th hour of life. She was diagnosed with congenital hyperinsulinism based on high glucose load, negative ketone and a good response to glucagon. Genetic workup revealed the presence of ATP Binding Cassette Subfamily C Member 8 (ABCC8 genes) mutation which indicated a focal form of congenital hyperinsulinism. She was resistant to the standard dose of oral diazoxide but responded to subcutaneous somatostatin. At the age of 3 years and 6 months, multiple daily injections of somatostatin were replaced with a long-acting monthly somatostatin analogue. With the present treatment, she had better glycaemic control, normal growth and was able to stop tube feeding.
Congenital Hyperinsulinism ; Somatostatin

Objective: We aimed to study the median time to gain weight from baseline and factors that were associated with rate of weight gain among obese children attending pediatric endocrine clinic Hospital USM. Methodology: We recruited 70 participants with the mean age of 10.1 ± 2.94 years with exogenous or simple form of obesity from June 2019 until September 2020. We analyzed their demography (age, gender, ethnicity, family background), measured their anthropometry (weight, height, BMI) and monitored monthly weight increment and finally analyzed their HOMA-IR at baseline and after 6 months of follow up. Results: The mean time to gain 5 kg from baseline was 16 weeks (95% CI): (15.2, 16.7). Multivariate analysis showed only HOMA-IR after 6 months was a significant predictor affecting time to gain 5 kg; Adjusted HR: (95% CI) 1.617 (1.232, 2.123), (p=0.001). Conclusion The time to gain 5 kg from baseline weight was increased 1.6 times in the presence of insulin resistance at 6 months follow up in patients with obesity. More intensive education and closed follow-up are recommended for children with obesity.
Prognosis ; Obesity ; Insulin Resistance