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新生儿胆汁淤积症:巴基斯坦儿童病因谱的变化

Neonatal Cholestasis: The Changing Etiological Spectrum in Pakistani Children.

作者信息

Bilal Hazrat, Irshad Muhammad, Shahzadi Nagina, Hashmi Almas, Ullah Hashmat

机构信息

Pediatric Gastroenterology, Lady Reading Hospital, Peshawar, PAK.

Pediatrics, Lady Reading Hospital, Peshawar, PAK.

出版信息

Cureus. 2022 Jun 12;14(6):e25882. doi: 10.7759/cureus.25882. eCollection 2022 Jun.

DOI:10.7759/cureus.25882
PMID:35844336
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9277006/
Abstract

OBJECTIVES

To determine the frequency of clinical presentation and laboratory profile in the diagnosis of the etiological spectrum of neonatal cholestasis.

MATERIAL AND METHODS

In this prospective cross-sectional study, we recruited children who presented with jaundice and direct hyperbilirubinemia with onset in the first three months of life. The study was conducted between April 2019 to March 2021 (24 months) at the Government Lady Reading Hospital of Khyber Pakhtunkhwa province in Pakistan. The diagnosis was based on history and clinical findings that included jaundice, stool color, itching, abdominal distention, and deranged liver function tests and confirmed on liver biopsy and specific diagnostic tests. Data was recorded and analyzed using SPSS version 20 (IBM Corp., Armonk, NY).

RESULTS

A total of 90 children were included in the study, out of which 65.6% were male. The average age was recorded as 118.01 days + 118.1 SD. Jaundice, dark urine, and hepatomegaly were found in 85.6% of children while ophthalmologic disorder, congenital heart disease, and itching were the least common symptoms. Laboratory findings of the cholestasis patients showed high bilirubin (mean: 8.88 mg/dL), alanine transaminase (ALT) (mean: 177.48 IU/mL), aspartate transaminase (AST) (mean: 187.11 IU/mL), gamma-glutamyl transpeptidase (GGT) (mean: 187.66 IU/mL) and prolonged international normalized ratio (INR) (mean: 2.20) in majority of patients. The genetic and metabolic disorder was the leading cause found in the majority of children, which was 43.8%.

CONCLUSION

The common causes of neonatal cholestasis in this study are galactosemia, idiopathic hepatitis, and biliary atresia. The common presentation includes jaundice, hepatomegaly, direct hyperbilirubinemia, raised liver enzymes, and INR.

摘要

目的

确定新生儿胆汁淤积病因谱诊断中临床表现和实验室检查结果的频率。

材料与方法

在这项前瞻性横断面研究中,我们招募了在出生后前三个月出现黄疸和直接胆红素血症的儿童。该研究于2019年4月至2021年3月(24个月)在巴基斯坦开伯尔-普赫图赫瓦省政府拉迪夫人医院进行。诊断基于病史和临床检查结果,包括黄疸、大便颜色、瘙痒、腹胀以及肝功能检查异常,并通过肝活检和特定诊断检查得以确诊。使用SPSS 20版(IBM公司,纽约州阿蒙克)记录和分析数据。

结果

共有90名儿童纳入研究,其中65.6%为男性。记录的平均年龄为118.01天±118.1标准差。85.6%的儿童出现黄疸、深色尿和肝肿大,而眼科疾病、先天性心脏病和瘙痒是最不常见的症状。胆汁淤积患者的实验室检查结果显示,大多数患者胆红素水平高(平均:8.88mg/dL)、丙氨酸转氨酶(ALT)(平均:177.48IU/mL)、天冬氨酸转氨酶(AST)(平均:187.11IU/mL)、γ-谷氨酰转肽酶(GGT)(平均:187.66IU/mL)以及国际标准化比值(INR)延长(平均:2.20)。大多数儿童中发现的主要病因是遗传和代谢紊乱,占43.8%。

结论

本研究中新生儿胆汁淤积的常见病因是半乳糖血症、特发性肝炎和胆道闭锁。常见表现包括黄疸、肝肿大、直接胆红素血症、肝酶升高和INR升高。

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Evaluation of cholestasis in Iranian infants less than three months of age.对伊朗三个月以下婴儿胆汁淤积症的评估。
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Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants.肝脏超声、肝活检及十二指肠管检查对塞尔维亚婴儿肝外胆道闭锁的诊断预测价值
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