Outcome of Vertical Expandable Prosthetic Titanium Rib (VEPTR) Instrumentation in Scoliosis Associated With 1p36 Deletion Syndrome: A Case Report.

Monosomy 1p36 deletion is a rare syndrome that consists of developmental delay, intellectual disability, seizures, hearing and vision defects, brain anomalies, orofacial clefting, congenital heart defects, cardiomyopathy, renal anomalies, and scoliosis. We report the case of an eight-year-old boy who presented to the orthopedic clinic with spinal deformity with a background of 1p36 deletion syndrome. The treatment modalities at this age include growing rods, vertical expandable prosthetic titanium rib (VEPTR), or posterior spinal fusion. Keeping in view the challenges in this case due to multi-organ involvement and severe intellectual disability, we decided to manage this patient with a VEPTR device to prevent the progression of scoliosis and allow spinal growth. Vertical expandable prosthetic titanium rib (VEPTR) instrumentation for progressive scoliosis in p36 deletion syndrome is an effective mode of treatment and leads to favorable outcomes.

Status of 25-hydroxy vitamin D level in simple febrile seizures and its correlation with recurrence of seizures.

BACKGROUND: Febrile seizures are associated with a lot of modifiable and nonmodifiable risk factors. Extensive research is currently going on to discover more and more risk factors of febrile seizures, so that they can be modified to decrease their incidence and recurrence. AIMS AND OBJECTIVES: The aim of this study was to determine the status of 25-hydroxy vitamin D in children presented with simple febrile seizures and to find its correlation with recurrence of seizures. MATERIALS AND METHODS: This prospective observational study was conducted on 223 children of age group 7-59 months who presented with simple febrile seizures. 25-hydroxy vitamin D were sent to laboratory for quantitative estimation. All data were recorded, status of vitamin D in these children was analyzed, and statistical significance of correlation of vitamin D with the number of recurrent seizure episodes was derived. STATISTICAL ANALYSIS: The comparison among groups was carried out by analysis of variance and correlation was conducted by Pearson's correlation analysis. A value of P < 0.05 was considered statistically significant. RESULTS: 25-hydroxy vitamin D insufficiency was present in 43.5% of the children, deficiency in 30.85 %, and normal level in 25.56% of children who had simple febrile seizures. Majority of the children presented with recurrent episodes of seizures had vitamin D deficiency followed by insufficiency and normal level. Comparison of Vitamin D showed significant negative correlation (As vitamin D level increases frequency of seizure febrile seizure recurrence decreases and vice versa) with recurrence of simple febrile seizures. CONCLUSION: Deficiency of vitamin D is associated significantly with simple febrile seizures and their recurrence is negatively correlated with it.