Osteopetrosis, a rare congenital genetic disease characterized by increased bone density due to impaired bone resorption by osteoclasts. It is classified into three forms: Infantile malignant autosomal recessive (AR) osteopetrosis, intermediate (AR) osteopetrosis and autosomal dominant (AD) osteopetrosis. Incidence of infantile malignant AR is 1/2,00,000 and if untreated has a fatal outcome. The condition is commonly diagnosed in infancy with symptoms of significant hematologic abnormalities with bone marrow failure, hepatosplenomegaly, macrocephaly with frontal bossing and bone fractures. Because of rarity of this type of malignant infantile form of osteopretrosis, we like to report this case of malignant infantile osteopetrosis who presented with bronchopneumonia, anemia with melaena at 2 months 15 days of age.

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Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red cell enzymopathy affecting red cell metabolism. Its presentation can vary from drug induced haemolysis to neonatal jaundice with a prevalence ranging from 0-27% in India. The objective of this study was to find out the incidence of G6PD deficiency in babies delivered in our hospital and to assess its contribution in causing neonatal jaundice. 500 babies were screened for G6PD deficiency by Fluorescent spot test method. All the babies were found to have normal enzyme activity. Hence we concluded the burden of G6PD deficiency causing neonatal jaundice is less in our setup.J Nepal Paediatr Soc 2016;36(2):141-142.