Pulmonary maturational arrest and death in a patient with pulmonary interstitial glycogenosis.
Pulmonary maturational arrest and death in a patient with pulmonary interstitial glycogenosis.
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肺部间质糖原病患者的肺成熟和死亡。
DOI：
10.1002/ppul.21486
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发表时间：
2011-11
影响因子：
3.1
通讯作者：
Kingma, Paul S.
中科院分区：
文献类型：
--
作者：
King, Brooke A.;Boyd, J. Todd;Kingma, Paul S.
我们报告一例患有非典型新生儿肺部疾病的足月儿的临床表现和病理结果。一名足月高加索男性出生时出现呼吸窘迫的迹象。尽管进行了最大限度的干预，但呼吸状况继续恶化，患者被安排在ECMO上接受进一步的心肺辅助。开胸肺活检发现与严重的肺生长异常和不均匀的肺间质糖原沉积相一致。因此，患者出现肺出血，需要停止体外反搏。病人在拔管后不久死亡。大多数文献表明，猪是少数几种预后良好的儿童间质性肺病之一，在没有合并症的情况下死亡率很低。然而，目前的病例表明，预后可能更多地取决于潜在的诊断，而不是猪的组织学发现。此外，此病例可能为这种特发性疾病的发病机制和潜在的修饰物提供洞察力。
We present the clinical presentation and pathological findings from a term infant with atypical neonatal lung disease. A full term Caucasian male presented at birth with signs of respiratory distress. The respiratory condition continued to deteriorate despite maximum intervention and the patient was placed on ECMO for further cardiorespiratory assistance. An open lung biopsy demonstrated findings consistent with severe lung growth abnormality with non-uniform pulmonary interstitial glycogenosis. The patient consequently developed a pulmonary hemorrhage that required discontinuation of ECMO. The patient died shortly after decannulation. Most literature suggests that PIG is one of the few pediatric interstitial lung diseases that has a favorable prognosis with rare mortality in the absence of co-morbidities. However, the current case suggests prognosis may depend more on the underlying diagnosis than on the histological finding of PIG. In addition, this case may provide insight into the pathogenesis and potential modifiers of this idiopathic disorder.
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DOI：
10.1164/rccm.2105139
发表时间：
2002-06-01
影响因子：
24.7
作者：
Canakis, AM;Cutz, E;O'Brodovich, H
通讯作者：
O'Brodovich, H
DOI：
10.1164/rccm.200703-393oc
发表时间：
2007-12-01
影响因子：
24.7
作者：
Deutsch, Gail H.;Young, Lisa R.;White, Frances V.
通讯作者：
White, Frances V.
影响因子：
3.1
作者：
Onland, W;Molenaar, JJ;Geukers, VGM
通讯作者：
Geukers, VGM
影响因子：
9.6
作者：
SCHROEDER, SA;SHANNON, DC;MARK, EJ
通讯作者：
MARK, EJ
影响因子：
2.3
作者：
Kline-Fath, Beth M.;Calvo-Garcia, Maria A.;Racadio, Judy M.
通讯作者：
Racadio, Judy M.