Hamberger Eric, Yu Yolanda, Choi Hyo-Jung
Department of Pediatrics, Division of Pediatric Pulmonology and Sleep Medicine, University of California Los Angeles, Los Angeles, CA, USA.
Department of Pediatrics, Division of Neonatology and Developmental Biology, University of California Los Angeles, Los Angeles, CA, USA.
Respir Med Case Rep. 2024 Feb 1;48:101990. doi: 10.1016/j.rmcr.2024.101990. eCollection 2024.
Pulmonary interstitial glycogenosis (PIG) is known to be associated with a wide variety of congenital conditions, though the extent to which PIG contributes to clinical presentation and outcomes in infants remains controversial. We describe two cases of infants with congenital anomalies and respiratory distress at birth who were diagnosed with PIG with differing clinical courses and response to methylprednisolone therapy. These cases highlight the importance of improved recognition of PIG and uncertainties about which patients may benefit from treatment.
肺间质糖原沉积症（PIG）已知与多种先天性疾病相关，尽管PIG在婴儿临床表现和预后中的作用程度仍存在争议。我们描述了两例出生时患有先天性异常和呼吸窘迫的婴儿病例，他们被诊断为PIG，临床病程和对甲泼尼龙治疗的反应各不相同。这些病例凸显了提高对PIG认识的重要性以及哪些患者可能从治疗中获益的不确定性。
