Chest X Ray of a male baby born at term with an uncomplicated pregnancy, labor and delivery who developed severe respiratory distress within few hours of bih revealed a normal hea shadow and fine reticulonodular infiltrates radiating from the hilum. ECHO findings reveal no abnormality and results of routine culture were also negative. Family history reveals similar clinical course and death of a male and female sibling at 1 month and 2 months of age respectively. The most likely diagnosis is:
High-Yield Explanation
The child in question is presenting with characteristic features of Neonatal Pulmonary Alveolar Proteinosis (PAP). Familial cases of Neonatal Pulmonary Alveolar proteinosis have been observed and a genetic basis has been suggested. None of the other options have a familial basis and hence neonatal Neonatal Pulmonary Alveolar Proteinosis (PAP) is the single best answer of choice. Ref: Robbin's pathology 7th Edition, Page 741; O P Ghai 6th Edition, Page 168, 413