Deutsch Gail H, Young Lisa R
Department of Laboratories, A6901, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA.
Pediatr Dev Pathol. 2009 Nov-Dec;12(6):475-80. doi: 10.2350/08-12-0575.1.
Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress. Despite its dramatic clinical presentation, the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of comorbid conditions. In this report, we describe changes in successive lung biopsies in a neonate who presented with respiratory failure and pulmonary hypertension. Diagnostic lung biopsy at 10 days of age exhibited classic histologic and ultrastructural findings of PIG with diffuse expansion of the alveolar interstitium by glycogenated mesenchymal cells. Subsequent to the patient's clinical improvement, a repeat biopsy at 49 days of age showed significant resolution of the disorder. Colocalization of vimentin-immunopositive cells with both phospho-histone H3 and cleaved caspase-3 demonstrated prominent attenuation of mesenchymal cell proliferation and apoptosis in the second biopsy. Although the self-limited nature of PIG has been described clinically, it has never been documented histologically. We present this case to illustrate the clinical and pathologic resolution of the disorder and speculate that the lesional mesenchymal cells may have transient proliferative capacity.
肺间质糖原贮积症（PIG）是一种病因不明的神秘肺部疾病，表现为新生儿呼吸窘迫。尽管其临床表现显著，但PIG的诊断预后良好，在无合并症的情况下死亡率极低。在本报告中，我们描述了一名出现呼吸衰竭和肺动脉高压的新生儿连续肺活检的变化。10日龄时的诊断性肺活检显示出PIG典型的组织学和超微结构特征，糖原化的间充质细胞使肺泡间质弥漫性扩张。患者临床症状改善后，49日龄时再次活检显示病情显著缓解。波形蛋白免疫阳性细胞与磷酸化组蛋白H3和裂解的半胱天冬酶-3共定位，表明第二次活检中间充质细胞增殖和凋亡明显减弱。虽然PIG的自限性在临床上已有描述，但从未有过组织学记录。我们展示此病例以说明该疾病的临床和病理缓解情况，并推测病变间充质细胞可能具有短暂的增殖能力。
