THE PRELAPAROTOMY DIAGNOSIS OF EXTRA-HEPATIC BILIARY ATRESIA
THE PRELAPAROTOMY DIAGNOSIS OF EXTRA-HEPATIC BILIARY ATRESIA
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DOI：
10.1136/adc.58.8.591
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发表时间：
1983-01-01
影响因子：
5.2
通讯作者：
HOWARD, ER
中科院分区：
文献类型：
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作者：
MANOLAKI, AG;LARCHER, VF;HOWARD, ER
在 86 名连续出现结合高胆红素血症的婴儿中，评估了剖腹手术前区分肝外胆道闭锁 (EHBA) 和肝内疾病 (IHD) 的实验室检查的诊断准确性。 45 名婴儿患有 EHBA，41 名婴儿患有 IHD。患有EHBA的婴儿的平均血清胆红素浓度、γ-谷氨酰转肽酶(GGT)活性和GGT/天冬氨酸转氨酶(AST)比率明显高于患有IHD的婴儿。然而，在患有 IHD 的婴儿中，有 19 名婴儿 (47%) 的血清胆红素浓度处于 EHBA 范围内，29 名婴儿 (71%) 的 GGT 值处于 EHBA 范围内，33 名婴儿 (80%) 的 GGT/AST 比率也处于 EHBA 范围内。在个别患者中，GGT 值的升高或降低均不具有诊断意义。 30 名 EHBA 患者中有 29 名 (97%) 出现 99mTc-p-Butyl-ida [亚氨基二乙酸] 胆汁排泄失败，23 名 IHD 患者中也有 22 名 (67%) 出现这种情况。在所有 5 名与 α1 抗胰蛋白酶缺乏相关的 IHD 患者中，这 4 项研究给出了 EHBA 范围内的结果。 42 名患有 EHBA 的婴儿中，肝活检标本的解读正确，其中 38 名的诊断总体准确率为 86%：另外 3 次活检的结果是模棱两可的。 40 名 IHD 婴儿中，有 33 名排除了胆管阻塞；其余 7 种，包括 4 和 α1 抗胰蛋白酶缺乏症，表现出模棱两可的变化。 37 名 EHBA 患者中的 36 名和 26 名 IHD 患者中的 9 名患者中，131I 玫瑰红粪便排泄率 < 10%，总体诊断准确率为 84%。在已排除遗传性疾病（例如 α1 抗胰蛋白酶缺乏症）的患者中，对肝活检标本和 131I 孟加拉粪便排泄物的解释仍然是识别需要 EHBA 手术的患者和避免 IHD 婴儿不必要的剖腹手术的最准确方法。
The diagnostic accuracy of laboratory investigations in the prelaparotomy differentiation between extrahepatic biliary atresia (EHBA) and intrahepatic disease (IHD) was assessed in 86 consecutive infants presenting with conjugated hyperbilirubinemia. Forty five infants had EHBA and 41 IHD. The mean serum bilirubin concentration, .gamma.-glutamyltranspeptidase (GGT) activity and the GGT/aspartate transaminase (AST) ratio were appreciably higher in infants with EHBA than in those with IHD. In infants with IHD, however, serum bilirubin concentrations were in the EHBA range in 19 (47%), as were GGT values in 29 (71%), and GGT/AST ratios in 33 (80%). In individual patients, neither increasing nor decreasing GGT values were of diagnostic importance. Failure of biliary excretion of 99mTc-p-Butyl-ida [iminodiacetic acid] occurred in 29 of 30 (97%) patients with EHBA but also in 22 of 23 (67%) with IHD. In all 5 patients with IHD associated with .alpha.1 antitrypsin deficiency, these 4 investigations gave results in the EHBA range. Liver biopsy specimen interpretation, correct in 38 of 42 infants with EHBA, gave an overall accuracy of diagnosis of 86%: the results of 3 further biopsies were equivocal. In 33 of 40 infants with IHD, bile duct obstruction was excluded; the remaining 7, including 4 and .alpha.1 antitrypsin deficiency, showed equivocal changes. Fecal excretion of 131I rose bengal fecal excretion was < 10% in 36 of 37 patients with EHBA and in 9 of 26 with IHD, giving an overall accuracy of diagnosis of 84%. In patients in whom genetic disorders, such as .alpha.1 antitrypsin deficiency had been excluded, interpretation of liver biopsy specimens together with 131I rose bengal fecal excretion remain the most accurate means of identifying those who need surgery for EHBA and of avoiding unnecessary laparotomy in infants with IHD.