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[突尼斯儿童医院婴儿胆汁淤积症研究]

[Cholestasis in infants: a study of the Children's Hospital of Tunisia].

作者信息

Bouyahia Olfa, Khelifi Ibtissem, Mazigh Sonia Mrad, Gharsallah Lamia, Chaouachi Beji, Hamzaoui Mourad, Barsaoui Sihem, Ben Becher Sayda, Bousnina Souad, Boukthir Samir, El Gharbi Azza Sammoud

机构信息

Hôpital d'Enfants, Bab Saadoun, Tunis, Tunisie.

出版信息

Tunis Med. 2008 Feb;86(2):128-35.

Abstract

BACKGROUND

Cholestasis in infant constitutes an heterogeneous group of disease; diagnosis and management are often difficult. THE AIM of the study is to describe clinical, paraclinical characteristics and outcome of infants hospitalized for cholestasis in children's Hospital of Tunis.

METHODS

A retrospective study of 94 infants with cholestasis was conducted. Patients were hospitalized in four departments of paediatrics of our hospital between January 1995 and December 2005. Cholestasis complicating severe sepsis and visceral leishmaniasis were excluded.

RESULTS

Incidence of cholestasis was 8.5 cases/year which represented 0.72% of the hospitalizations. Sex ratio was 1.08 and mean age at diagnosis was 105 days (extremes: 1 day- 24 months). Biliary atresia was the most common cause of extra hepatic cholestasis (13.8%). Normal A GT cholestasis (11.7 %), benign neonatal cholestasis (11.7%) and bile duct hypoplasia (9.5%) represented the most common aetiologies of intra hepatic cholestasis. Aetiology remained unknown in 12.7% of cases. Only three infants with biliary atresia had Kasaï operation. After a mean follow-up of 6 years, 18% of patients had portal hypertension, 14.8% had hepatic failure and mortality rate was 14.8%.

CONCLUSION

Cholestasis of unknown aetiologies are frequent in our hospital. Poor prognosis, in our study, is due to delay to diagnosis and difficulties in medical and surgical management.

摘要

背景

婴儿胆汁淤积是一组异质性疾病;诊断和管理往往具有挑战性。本研究旨在描述在突尼斯儿童医院因胆汁淤积住院的婴儿的临床、辅助检查特征及预后情况。

方法

对94例胆汁淤积婴儿进行回顾性研究。患者于1995年1月至2005年12月期间在我院四个儿科病房住院。排除并发严重脓毒症和内脏利什曼病的胆汁淤积。

结果

胆汁淤积的发病率为8.5例/年,占住院患者的0.72%。男女比例为1.08,诊断时的平均年龄为105天(范围:1天至24个月)。胆道闭锁是肝外胆汁淤积最常见的原因(13.8%)。正常γ-谷氨酰转肽酶胆汁淤积(11.7%)、良性新生儿胆汁淤积(11.7%)和胆管发育不全(9.5%)是肝内胆汁淤积最常见的病因。12.7%的病例病因不明。仅3例胆道闭锁婴儿接受了Kasai手术。平均随访6年后,18%的患者出现门静脉高压,14.8%的患者出现肝衰竭,死亡率为14.8%。

结论

在我院,病因不明的胆汁淤积较为常见。在我们的研究中,预后不良是由于诊断延迟以及医疗和手术管理困难所致。

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