Yachha S K, Khanduri A, Kumar M, Sikora S S, Saxena R, Gupta R K, Kishore J
Department of Gastroenterology (Pediatric GE), Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow.
Indian Pediatr. 1996 Sep;33(9):729-34.
To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS).
Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years.
Tertiary level referral gastroenterology center in North India.
Liver function tests, urine examination, serum antibodies against Cytomegalovirus (CMV), Rubella and Toxoplasma; abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy were done. In appropriate setting, laparotomy and surgical corrections were done for biliary tract disorders.
NCS constituted 19% of pediatric liver diseases. Considerable delay in presentation was observed [mean delay, extrahepatic biliary atresia (EHBA) = 4 +/- 2.0 months, neonatal hepatitis (NH) = 2.2 +/- 1.3 months]. Thirty three (55%) infants had EHBA, 14 (23%) NH (4 CMV, 2 galactosemia, 1 urinary tract infection and 7 idiopathic), 2 (3%) paucity of intralobular bile ducts and 11 (18%) were of indeterminate etiology. Liver biopsy was the most accurate (96.4%) investigation in discriminating between EHBA and NH. Of the 18 operated infants with EHBA (portoenterostomy-15 and hepatico-jejunostomy-3), 10 were alive (mean follow up = 22.8 +/- 8.6 months) of which 4 were completely asymptomatic.
(i) NCS is an important cause of liver disease in Indian children. (ii) It requires prompt referral, quick investigative approach and targeted management. (iii) Liver biopsy is highly accurate in differentiating EHBA and NH. (iv) infants with EHBA and compensated status of liver should undergo corrective surgery irrespective of age at presentation.
了解新生儿胆汁淤积综合征（NCS）患者的发病率、病因及临床特征、各项检查的疗效及预后。
对连续60例NCS婴儿（平均年龄3.9±1.9个月；49例男性）进行为期3.5年的前瞻性评估。
印度北部的三级转诊胃肠病学中心。
进行肝功能检查、尿液检查、抗巨细胞病毒（CMV）、风疹和弓形虫血清抗体检测；腹部超声、肝胆闪烁显像及肝活检。在适当情况下，对胆道疾病进行剖腹手术及手术矫正。
NCS占儿童肝病的19%。观察到就诊明显延迟[平均延迟时间，肝外胆道闭锁（EHBA）=4±2.0个月，新生儿肝炎（NH）=2.2±1.3个月]。33例（55%）婴儿患有EHBA，14例（23%）患有NH（4例CMV、2例半乳糖血症、1例尿路感染和7例特发性），2例（3%）小叶内胆管稀少，11例（18%）病因不明。肝活检是鉴别EHBA和NH最准确的检查（96.4%）。18例接受手术的EHBA婴儿（15例行门肠吻合术，3例行肝空肠吻合术）中，10例存活（平均随访时间=22.8±8.6个月），其中4例完全无症状。
（i）NCS是印度儿童肝病的重要病因。（ii） 需要及时转诊、快速的检查方法和针对性的治疗。（iii）肝活检在鉴别EHBA和NH方面高度准确。（iv）患有EHBA且肝脏功能代偿的婴儿无论就诊时年龄多大均应接受矫正手术。
