闪烁扫描在儿童肝肺综合征诊断中的应用

Teisseyre M, Szymczak M, Swiatek-Rawa E, Pawłowska J, Socha P, Kamiński A, Jankowska I, Kamińska A

Clinic of Gastroenterology, Hepatology and Nutrition, Memorial Child Health Centre Institute, Aleja Dzieci Polskich 20, 04-730 Warsaw, Poland.

Med Sci Monit. 2001 May;7 Suppl 1:255-61.

BACKGROUND

Arterial blood oxygenation disturbances accompanying liver cirrhosis are referred to as hepatopulmonary syndrome (HPS). HPS develops due to the formation of intrapulmonary arteriovenous shunts and dilatation of the vascular bed. The aim of the study was to assess the incidence of arteriovenous shunts in children with hepatic cirrhosis and HPS suspected on the basis of clinical signs, qualified for liver transplantation.

MATERIAL AND METHODS

The study was carried out in a group of 21 children aged from 1.2 to 17.7 years (mean age 8.4); 8 girls and 13 boys. The patients were diagnosed as follows: biliary cirrhosis due to extrahepatic bile duct impatency--12, post-inflammatory liver cirrhosis due to infection with HVB--2, autoimmune hepatitis--2, fibrosis of the liver--2, alpha 1-antitrypsin deficiency--1, progressive familial intrahepatic cholestasis--1, cystic fibrosis--1. The presence of arteriovenous anastomoses in the lungs was detected by scintiscanning utilizing microspheres (albumin macroaggregates). Under physiologic conditions, technetium-labeled microspheres injected i.v. are accumulated in pulmonary capillaries. Radioactivity detected in other organs (kidneys, brain) indicates the presence of pathologic shunts omitting the alveoli. A percentage ratio of radioactivity detected in the brain to that present in the lungs serves as the index of blood flow through the anastomosis (SI--shunt index). According to Grimon, the mean SI value for healthy children amounts to 0.43%, the values ranging from 1% to 2% are regarded as borderline ones, and those above 2% allow unequivocal diagnosis of HPS.

RESULTS

The SI values obtained in the study ranged from 0.06 to 51%. In 7 patients SI exceeded 1%, reaching 1.23% in one, and over 3% in the remaining 6 patients. No correlations were found either between SI value and the etiology of cirrhosis, or between the index and clinical condition of the patient assessed according to Child-Pugh scale.

CONCLUSIONS

Scintiscanning revealed the presence of arteriovenous shunts in the lungs of 6/21 (28.6%) investigated patients. 2. No correlation was found between the severity of HPS and the etiology and stage of the underlying disease.

背景

肝硬化伴发的动脉血氧合障碍被称为肝肺综合征（HPS）。HPS是由于肺内动静脉分流的形成和血管床扩张所致。本研究的目的是评估基于临床症状怀疑患有肝硬化和HPS且符合肝移植条件的儿童动静脉分流的发生率。

材料与方法

本研究纳入了21名年龄在1.2至17.7岁（平均年龄8.4岁）的儿童；其中8名女孩，13名男孩。患者的诊断如下：肝外胆管闭锁所致胆汁性肝硬化——12例，乙肝病毒感染所致炎症后肝硬化——2例，自身免疫性肝炎——2例，肝纤维化——2例，α1-抗胰蛋白酶缺乏症——1例，进行性家族性肝内胆汁淤积症——1例，囊性纤维化——1例。利用微球（白蛋白大聚合体）闪烁扫描检测肺部动静脉吻合的存在。在生理条件下，静脉注射的锝标记微球会积聚在肺毛细血管中。在其他器官（肾脏、大脑）检测到的放射性表明存在绕过肺泡的病理性分流。大脑中检测到的放射性与肺部放射性的百分比比值作为通过吻合口的血流指数（SI——分流指数）。根据格里蒙的研究，健康儿童的平均SI值为0.43%，1%至2%的值被视为临界值，而高于2%的值则可明确诊断为HPS。