Introduction: New-onset diabetes after transplantation (NODAT) is a clinically significant complication associated with adverse metabolic and graft outcomes and affects patients’ quality of life. Data in paediatric populations are limited. This case series describes the incidence, clinical characteristics, and early outcomes of NODAT in a paediatric kidney transplant cohort. Cases: Thirty patients (aged 5–17 years; 16 males, 14 females; 14 Malay, 15 Chinese, 1 Indian) underwent renal transplantation at Hospital Tunku Azizah between 2019 and 2025. NODAT was identified using standard diagnostic criteria and occurred in four patients (13.3%). Among patients who developed NODAT, three were Malay and one Chinese; all were aged 10–17 years, with a predominance of females (n = 3). All were non-obese (body mass index [BMI]: 11–21.4 kg/m²), and only one had a family history of type 2 diabetes mellitus. Underlying causes of ESRF in these patients included focal segmental glomerulosclerosis (FSGS, n = 2), CAKUT (n = 1), and unknown etiology (n = 1). Two patients received kidneys from living donors, and two from cadaveric donors. Two patients demonstrated reduced C-peptide levels, consistent with impaired insulin secretion. All patients received steroid induction and maintenance therapy alongside tacrolimus-based immunosuppression, with one patient also receiving everolimus. Two patients developed post-transplant CMV viremia, which has been associated with NODAT. The onset of NODAT was early, occurring within 2 weeks post-transplant in three patients and at 1 month in one patient. Insulin therapy achieved glycemic control in all cases. Two patients were able to discontinue insulin within 3–4 months, suggesting recovery of endogenous glycemic regulation, while the remaining two required ongoing insulin therapy. Conclusion NODAT affects a substantial proportion of paediatric kidney transplant recipients and may present despite the absence of conventional risk factors. Early surveillance is essential. The observed potential for insulin independence may indicate a reversible component of β-cell dysfunction, which warrants further investigation in larger cohorts.
Child ; Kidney Transplantation ; Hospitals

Introduction: RENI syndrome (renal, endocrine, neurologic, and immune syndrome), also known as sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS), is a rare autosomal recessive disorder caused by pathogenic variants in the SGPL1 gene. This gene encodes sphingosine-1- phosphate lyase, an enzyme responsible for the final step in sphingolipid degradation. Impaired enzyme activity results in the accumulation of sphingolipid intermediates, leading to multisystem involvement affecting the kidneys, adrenal glands, skin, immune system, and nervous system. Frequently reported manifestations include steroid-resistant nephrotic syndrome, primary adrenal insufficiency, ichthyosis, and neurological abnormalities. Case: We report a 15-year-old male with a history of primary adrenal insufficiency diagnosed at age three after presenting with recurrent vomiting and abdominal pain. He was started on steroid replacement therapy and remained stable without episodes of adrenal crisis. He also had ichthyosis requiring dermatological follow-up. During routine surveillance, persistent proteinuria was detected, and urinary protein excretion increased over time. Ultrasound showed renal parenchymal changes despite preserved renal function. Whole-exome sequencing identified a homozygous variant of uncertain significance in the SGPL1 gene, consistent with RENI syndrome. He was started on enalapril for his proteinuria and continues to undergo multidisciplinary follow-ups. Both parents are consanguineous, but were not screened. Mutations in SGPL1 have increasingly been recognized as a monogenic cause of syndromic steroid-resistant nephrotic syndrome, associated with endocrine and dermatological features. Renal histopathology in cases reported often shows focal segmental glomerulosclerosis, reflecting podocyte injury due to disruption of sphingolipid signaling pathways. Early recognition is crucial, as patients need multidisciplinary care. Conclusion This case highlights the importance of considering RENI syndrome in patients presenting with early-onset primary adrenal insufficiency accompanied by renal and dermatological symptoms. Increased clinical awareness and prompt genetic testing can enable earlier diagnosis, guide long-term monitoring, and support appropriate genetic counseling.
Adrenal Insufficiency ; Proteinuria ; Ichthyosis

Introduction: Growth hormone deficiency (GHD) is a recognized endocrine complication among childhood cancer survivors resulting from disruption of hypothalamic–pituitary axis due to tumors, neurosurgery, or cranial irradiation. While recombinant human growth hormone (rhGH) therapy improves growth and metabolic outcome, concerns remain regarding its long-term safety with risk of tumor recurrence and secondary neoplasm. We present a case series of five paediatric oncology survivors with confirmed GHD receiving rhGH in Hospital Tunku Azizah, highlighting our clinical experience in comparison with international practice. Case: Five paediatric oncology survivors (acute lymphoblastic leukemia, craniopharyngioma, medulloblastoma, and supratentorial PNET) with GHD were commenced on rhGH therapy 2.5–5.5 years after completion of cancer treatment. All patients had significant short stature with a mean height SDS of −3.22 prior to the initiation of therapy. GHD was confirmed biochemically with low IGF-1 level and dynamic testing (peak GH 0.35–4.61 ng/mL). Among the four patients with brain tumors, two had stable residual disease while the remaining were tumor-free. rhGH therapy was temporarily ceased in two patients due to minor increment in tumor size but was successfully resumed following stabilization without further complications to date. Two patients are concurrently on pubertal induction. Most patients demonstrated catch-up growth with increased height velocity, supporting the efficacy of rhGH in this population. No secondary malignancies or significant adverse events were observed during follow-up. Conclusion Paediatric oncology survivors with GHD in this series demonstrated a favorable response to rhGH therapy, evidenced by improvements in height SDS and growth velocity, while maintaining oncological stability in the majority of cases. Our findings are consistent with international data, supporting cautious but appropriate use of rhGH in this population. Careful patient selection and close multidisciplinary monitoring remain essential.
Child ; Neoplasms ; Growth Hormone ; Survivors