A Case of Neonatal Severe Hyperparathyroidism: Challenges in Management

Neonatal severe hyperparathyroidism is a rare disorder arising from inherited defects in the calcium sensing receptor (CaSR) that presents early in life with severe hypercalcemia, failure to thrive, and developmental retardation. The authors describe an infant with neonatal severe hyperparathyroidism due to homozygous CaSR gene mutation presenting with recurrent episodes of severe hypercalcemia, growth retardation, and developmental delay. Medical management served as an efective bridge therapy to surgery. Total parathyroidectomy with right hemithyroidectomy was performed at 7 mo of age and resulted in successful cure and normalization of growth and developmental milestones. Timely medical and surgical management can help prevent mortality and morbidity in the form of neurodevelopmental sequelae. Life-long monitoring and treatment is mandatory for the resultant hypoparathyroidism.

Clinical Profile and Outcome of Neonates with Hypernatremic Dehydration - A Tertiary Care Hospital based Study

Introduction: Neonatal hypernatremic dehydration is amedical emergency. Early diagnosis and appropriate treatmentis crucial for survival and better prognosis. However, diagnosisis often difficult due to apparent well being and dehydration isunderestimated. The aim of our study was to see the outcomeof neonates admitted with hypernatremic dehydration in ourhospitalMaterial and Methods: The study was conducted inneonatology of Postgraduate Department of Pediatrics, inGB Pant Hospital, an associated hospital of GovernmentMedical College Srinagar. It was a hospital based prospectiveobservational study conducted for 1 year, from March 2016to February 2017. A total of 67 neonates were enrolled in ourstudy.Results: The results showed mean age of presentation 18days, females were 37(55.5%), mean birth order was 1.6,44(65.7%) cases were from rural background,62 (92.5%) ofour cases were term babies, an average weight loss of 14.6%was found. There was a significant statistical relation in thepercentage of weight loss, incidence of shock, incidence ofAKI and sodium levels(p value<0.001).Conclusion: Women should be educated about theproper technique of breast feeding during prenatal visitsand after the delivery of the newborn. Mothers should beinformed about ‘ten steps of successful breast feeding’.All breast-fed infant should be weighed at least onceseveral days after discharge