Sáenz-Gómez Jessica, Karam Bechara José, Jamaica Balderas Lourdes
Servicio de Neumología, Hospital Infantil de México Federico Gómez, México D.F., México.
Servicio de Neumología, Hospital Infantil de México Federico Gómez, México D.F., México.
Bol Med Hosp Infant Mex. 2015 Mar-Apr;72(2):124-128. doi: 10.1016/j.bmhimx.2015.04.001. Epub 2015 Jun 19.
Hepatopulmonary syndrome is a rare complication characterized by liver disease associated with hypoxemia and intrapulmonary vascular dilatations. The prevalence reported in the few studies in children with cirrhosis is 3-8%. Although uncommon, it is important for physicians to recognize this condition because of its progressive course.
We report the case of an 8-year-old girl diagnosed with liver cirrhosis and portal hypertension with symptoms of dyspnea and cyanosis. On physical examination the patient was found malnourished with jaundice, telangiectasias in abdomen, severe clubbing, acrocyanosis, platypnea and orthodeoxia; arterial blood gas showed PaO of 59mmHg. Echocardiography with agitated saline test was positive and lung perfusion scan with albumin macroaggregates reported 15% right-to-left short circuit, thus demonstrating the existence of intrapulmonary shunt. Diagnosis of severe hepatopulmonary syndrome was made. Liver transplantation is recommended as the only effective treatment.
In children with liver disease presenting dyspnea and hypoxemia and those enrolled in a liver transplant protocol, hepatopulmonary syndrome must be intentionally searched because the prognosis will depend on timely diagnosis.
肝肺综合征是一种罕见的并发症，其特征为与低氧血症和肺内血管扩张相关的肝脏疾病。在少数针对肝硬化儿童的研究中报告的患病率为3% - 8%。尽管不常见，但由于其病程呈进行性，医生认识到这种情况很重要。
我们报告一例8岁女孩，诊断为肝硬化和门静脉高压，伴有呼吸困难和发绀症状。体格检查发现患者营养不良，伴有黄疸、腹部毛细血管扩张、重度杵状指、肢端发绀、平卧呼吸困难和直立性低氧血症；动脉血气显示动脉血氧分压为59mmHg。超声心动图盐水激发试验呈阳性，白蛋白微球肺灌注扫描显示有15%的右向左分流，从而证实存在肺内分流。诊断为重度肝肺综合征。推荐肝移植作为唯一有效的治疗方法。
对于出现呼吸困难和低氧血症的肝病儿童以及纳入肝移植方案的儿童，必须有意排查肝肺综合征，因为预后将取决于及时诊断。
