The Pediatric Pulmonology Division of the Department of Pediatrics at the prestigious All India Institute of Medical Sciences (AIIMS), Jodhpur, has attained a remarkable and historic medical milestone by successfully treating a critically ill 16-month-old female child diagnosed with the extremely rare and complex Niemann-Pick disease Type C1 with Pulmonary Alveolar Proteinosis. This exceptionally rare genetic disorder precipitates a catastrophic and harmful accumulation of lipids in vital organs, including the lungs, liver, brain, spleen, and bone marrow, leading to severe and potentially life-threatening complications. In this child, the destructive lipid buildup in the lungs caused an alarming deterioration of lung function, necessitating immediate and sustained mechanical ventilation for survival. 

The skilled and specialized medical team responded with a life-changing whole lung lavage procedure, a highly complex and delicate intervention. This cutting-edge treatment meticulously rinses the lungs with a saline solution to remove excess lipids and debris, gradually restoring lung function and significantly improving respiratory health. The intricate procedure was expertly performed over a span of 2 days, lasting a total of 9 hours. Following the procedure, the child was carefully and gradually weaned off oxygen support, marking a major turning point in their recovery. Subsequently, the child was discharged from the hospital, symbolizing a triumphant victory over this rare and formidable disease.

Notably, the whole lung lavage procedure in children has been successfully performed only in a select few, elite institutes across India, making AIIMS Jodhpur's achievement a groundbreaking and significant milestone. This pioneering procedure, a first in AIIMS Jodhpur and Rajasthan, further underscores the division's exceptional expertise, innovative spirit, and unwavering commitment to delivering exceptional patient care. The achievement showcases the remarkable expertise, dedication, and collaborative efforts of the Pediatric Pulmonology Division of the Department of Pediatrics at AIIMS, Jodhpur, cementing its reputation as a leading centre of excellence. 

This procedure was done by team of Pediatric Pulmonologist led by Dr Jagdish Goyal and Prawin Kumar. In this procedure resident doctors Dr Kalyan, Dr Sathya, Dr Sneh, Dr Mahesh Dr Vaishnavi,   Dr Rewathi, Dr Firnas, Dr Adrash and nursing staff Mr Reshma Ram, Mr Hariom helped during the procedure. Dr. Kuldeep Singh, Head of Department, Department of Pediatrics, said that the Pediatric Pulmonology team provides excellent care to rare pediatric pulmonary disorders like cystic fibrosis and other rare disorders. Whole lung lavage is a rare procedure and highlights the dedication of the department to providing excellence in patient care.

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