Department of Pathology, National Institute of Pediatrics, Mexico City, Mexico.
Arch Med Res. 2009 Oct;40(7):635-9. doi: 10.1016/j.arcmed.2009.08.006.
The relative frequency and risk factors of portopulmonary hypertension in children have not been defined with precision. The few published reports refer to isolated cases or small series from selected populations. The development of pulmonary hypertension in patients with portal hypertension is seldom suspected or documented during life. The examination of autopsy material can identify these cases because pulmonary hypertension produces characteristic histological changes in the pulmonary vasculature.
Autopsy cases with evidence of portal hypertension were retrieved. Slides of pulmonary tissue from these cases were examined in search of histopathological changes of hypertensive arteriopathy
Seventy six cases of portal hypertension were identified among 7060 autopsies collected between January 1971 and January 2008 (1.07%). Four cases with histopathological evidence of pulmonary hypertension were identified. This represents 5.2% of all cases with portal hypertension. These four patients were females in late childhood or adolescence with idiopathic portal hypertension. Pulmonary hypertension was not diagnosed during their lifetime.
Idiopathic portal hypertension in adolescent girls should be considered a risk factor for the development of portopulmonary hypertension, very likely as a consequence of the functional preservation of hepatic tissue that allows a prolonged survival. An oriented search for early signs of pulmonary hypertension in these patients would appear advisable in order to install timely treatment.
儿童门静脉高压症的相对频率和危险因素尚未明确界定。少数已发表的报告仅涉及来自特定人群的孤立病例或小系列。门静脉高压症患者的肺动脉高压很少在生前被怀疑或记录。尸检材料的检查可以识别这些病例，因为肺动脉高压会导致肺血管的特征性组织学变化。
检索了有门静脉高压证据的尸检病例。检查这些病例的肺组织切片，寻找高血压性小动脉病变的组织病理学变化。
在 1971 年 1 月至 2008 年 1 月期间收集的 7060 例尸检中，共发现 76 例门静脉高压（1.07%）。发现 4 例有肺动脉高压的组织病理学证据。这代表所有门静脉高压症患者的 5.2%。这 4 名患者均为青春期少女，患有特发性门静脉高压症。他们在生前没有被诊断出患有肺动脉高压。
青春期少女的特发性门静脉高压症应被视为发生门静脉高压症的一个危险因素，这很可能是由于肝组织的功能保留允许延长生存时间所致。对这些患者进行有针对性的早期肺动脉高压迹象搜索，以便及时进行治疗，似乎是明智的。
