Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
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In 1942, when retinopathy of prematurity (ROP) first manifested as retrolental fibroplasia, the technology to monitor or regulate oxygen did not exist, and a fundus examination of preterm infants was not routinely performed. Supplemental, uncontrolled oxygen at birth has since been found to cause retrolental fibroplasia. ⋯ Nevertheless, despite our better understanding of ROP and ongoing investigations of supplemental therapeutic oxygen, including recent clinical trials (Surfactant, Positive Airway Pressure, Pulse Oximetry Randomized Trial [SUPPORT] and Benefits of Oxygen Saturation Targeting [BOOST]), the best oxygen profiles to reduce ROP risk while optimizing preterm infant health and development remain unknown. This article reviews major studies on oxygen use in preterm infants and the effects on the development of ROP.
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Hermansky-Pudlak syndrome (HPS) is an autosomal-recessive disorder clinically characterized by oculocutaneous albinism, bleeding diatheses, and lysosomal accumulation of ceroid lipofuscin, which in some cases may cause granulomatous colitis and pulmonary fibrosis. Any of these complications could result in a shortened life span for patients with HPS. We report a 92-year-old man with HPS 5 who, to our knowledge, is the oldest patient with HPS documented in the literature. This report highlights the importance of typing HPS to counsel patients regarding disease prognosis.