Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis
Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis
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DOI：
10.1080/15513815.2017.1397071
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发表时间：
2018-01-01
影响因子：
1.1
通讯作者：
Sammut, Paul
中科院分区：
文献类型：
--
作者：
Still, Gordon Gray;Li, Shuo;Sammut, Paul
简介：肺间质性糖原累积症（PIG）是一种特发性肺部疾病，尽管有越来越多的文献报道，但临床上仍未得到充分认识。病例报告：我们提出了一个病例猪肺动脉高压没有潜在的心脏疾病。这位病人在6个月大时出现呼吸窘迫和自发性气胸。实验室和影像学检查显示非特异性的特点，但顽固性肺动脉高压证实心导管检查。肺组织病理学显示间质细胞糖原阳性染色，与PIG一致。结论：这一独特病例表明，肺动脉高压可以在没有相关心脏异常的猪的情况下观察到，并且尽管在其他自限性条件下进行治疗，但仍持续存在。
Introduction: Pulmonary interstitial glycogenosis (PIG) is an idiopathic lung condition that remains clinically underrecognized despite a growing body of literature. Case report: We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6months of age. Laboratory and imaging investigations demonstrated nonspecific features, but refractory pulmonary hypertension was confirmed on cardiac catheterization. Lung tissue histopathology showed glycogen positive staining of the interstitial cells, consistent with PIG. Conclusion: This unique case demonstrates that pulmonary hypertension can be seen in the setting of PIG without associated cardiac anomalies, and persists despite treatment in an otherwise self-limited condition.