Glucose control during Ramadan in a pediatric cohort with type 1 diabetes on MiniMed standard and advanced hybrid closed­loop systems: A pilot study.

BACKGROUND: Hybrid closed-loop (HCL) systems have revolutionized the treatment of diabetes, enabling doctors to cope with challenging conditions that were previously almost impossible to manage or were very risky and difficult. AIMS: To assess the efficacy and safety of a hybrid closed-loop (HCL) system during Ramadan fasting in a pediatric cohort with type 1 diabetes (T1D). RESEARCH DESIGN AND METHODS: Glucose control outcomes in older children and adolescents aged 8-16 years with automated insulin delivery for T1D were analyzed during Ramadan and 1 month before Ramadan. Participants on MiniMed standard HCL (670G) or advanced HCL (780G) systems of Medtronic were categorized as fasting or nonfasting. RESULTS: The average age of the 19 participants (8 and 11 were on standard and advanced HCL systems, respectively) was 11.35 ± 2 years. Eleven patients fasted during Ramadan. Pump setup and sensor statistics were the same during Ramadan and the month before; no significant difference was found between the two groups in terms of insulin and glucose control metrics, with practically the same coefficient of variation, time in range (TIR) and time spent in hypoglycemia, maintained within the international recommended targets. Total daily doses were paradoxically higher in patients who fasted during Ramadan (p = 0.01), without repercussions on glucose control metrics. CONCLUSIONS: Standard and advanced HCL use during Ramadan were safe and were associated with a maintained optimum TIR (>70 %) and no significant hypoglycemia in adolescents and older children with T1D.

Response to growth hormone therapy in ring chromosome 15: Review and evidence from a new case on possible beneficial effect in neurodevelopment.

Type 1 Insulin-like Growth Factor Receptor(IGF1R) plays a fundamental role in normal growth and development. Its disruption is usually characterized by severe intrauterine and postnatal growth retardation, microcephaly and neurodevelopmental delay.The efficacy of recombinant human growth hormone treatment remains a challenge for children with IGF1 resistance and pathogenic mutations of IGF1R, with limited data in patients carrying the most severe form of IGF1R defect, the ring chromosome 15. SUBJECT AND METHOD: We tested a high dose of rhGH in a new patient with ring chromosome 15, as confirmed by karyotype and CGH array. We performed a systematic review, and all published r(15) syndrome cases treated by growth hormone(GH) up to April 2023 were searched, and their response to GH therapy was recorded and summarized. RESULTS: Twelve patients with ring chromosome 15 received GH therapy according to a literature review. We expand the spectrum by the 13th case treated by GH, and we report an impressive improvement in intellectual performance and progressive catch-up growth after 5 and 20 months of follow-up. By introducing our new case in the analysis, the sex ratio was 3:10, and GH therapy was started at the age of 5.5 (3/9.4) (years) for an age of diagnosis of 4.75 (1.3/9.5) (years). The height before GH therapy was -5.1(-5.9/-4.1) SDS. The median duration of treatment was 1.7(0.9/2) (years), with a median height gain of 1(0.3/1.8) SDS and an improvement in growth velocity of 4.1(2.8/5.3) (cm/year). CONCLUSION: GH seems to be effective for r(15) syndrome patients with short stature.

Orbital complication of acute ethmoiditis: A Tunisian paediatric cross sectional study.

OBJECTIVE: The anatomical and developmental particularities of sinus cavities in paediatric population lead acute ethmoiditis to be the earliest form of sinusitis in children. Orbital complications are frequent and could lead to visual and neurological impairment. This study investigated the clinical, biological and radiological features of orbital complications. We identified the predictive factors of severe ophthalmological lesions and/or associated cerebral complications of acute ethmoiditis. DESIGN AND METHODS: This cross sectional study included all patients identified as having orbital extension of acute ethmoiditis in the database of a single academic paediatric care centre over a period of 14 years. All orbital and cerebral Scans of the included patients were reviewed and the cohort was classified using Chandler's classification as having less severe lesions (Chandler's 1 and 2) or more serious lesions (Chandler's 3, 4 and 5). RESULTS: In total, 16 patients (12 girls and 4 boys) were included among 39 consecutives cases of ethmoiditis recessed with a complication rate of 41%. Average consultation delay was 4.88 days. The mean age was 4.37 years. Fever was objectified in 13 cases (81%). Six patients (37.5%) had exophthalmos. Orbital extension spectrum was: stage I (n = 4, 25%), stage II (n = 4, 25%), stage III (n = 6, 37.5%), stage IV (n = 1, 6.5%), stage V (n = 1, 6.5%) and intra cranial extension was associated in two cases. Univariate analysis showed that fever, exophthalmos, ophthalmoplegia, positive CRP, age and white blood cells count were not associated with more severe lesions in the CT scan. Initially, all children received intravenous antibiotic treatment. Association of multiple antibiotics was prescribed in 75% of the cases. With 21.07 days ±â€¯5.51 days as a total treatment duration. Only Four patients underwent surgical treatment. CONCLUSION: Orbital complications of ethmoiditis are frequent. No clinical or biological criteria seem to predict the severity of orbital lesions. Both orbital and brain CT scan could help detect eventual complications on time to adapt antibiotic treatment and eventually bring forward surgical intervention.

High prevalence of syndromic disorders in patients with non-isolated central precocious puberty.

Objective Non-idiopathic CPP is caused by acquired or congenital hypothalamic lesions visible on MRI or is associated with various complex genetic and/or syndromic disorders. This study investigated the different types and prevalence of non-isolated CPP phenotypes. Design and Methods This observational cohort study included all patients identified as having non-idiopathic CPP in the database of a single academic pediatric care center over a period of 11.5 years. Patients were classified on the basis of MRI findings for the CNS as having either hypothalamic lesions or complex syndromic phenotypes without structural lesions of the hypothalamus. Results In total, 63 consecutive children (42 girls and 21 boys) with non-isolated CPP were identified. Diverse diseases were detected, and the hypothalamic lesions visible on MRI (n = 28, 45% of cases) included hamartomas (n = 17; either isolated or with an associated syndromic phenotype), optic gliomas (n = 8; with or without neurofibromatosis type 1), malformations (n = 3) with interhypothalamic adhesions (n = 2; isolated or associated with syndromic CNS midline abnormalities, such as optic nerve hypoplasia, ectopic posterior pituitary) or arachnoid cysts (n = 1). The patients with non-structural hypothalamic lesions (n = 35, 55% of cases) had narcolepsy (n = 9), RASopathies (n = 4), encephalopathy or autism spectrum disorders with or without chromosomal abnormalities (n = 15) and other complex syndromic disorders (n = 7). Conclusion Our findings suggest that a large proportion (55%) of patients with non-isolated probable non-idiopathic CPP may have complex disorders without structural hypothalamic lesions on MRI. Future studies should explore the pathophysiological relevance of the mechanisms underlying CPP in these disorders.