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滚动的石头:新生儿胆汁淤积症的一个启发性病例。

Rolling stones: an instructive case of neonatal cholestasis.

机构信息

Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children's Hospital, 6621 Fannin St., Mark Wallace Tower Suite 1010, Houston, TX, 77030, USA.

Department of Radiology, Baylor College of Medicine and Texas Children's Hospital, 6621 Fannin St., Mark Wallace Tower Suite 1010, Houston, TX, USA.

出版信息

BMC Pediatr. 2022 Sep 4;22(1):526. doi: 10.1186/s12887-022-03560-3.

DOI:10.1186/s12887-022-03560-3
PMID:36058901
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9441063/
Abstract

BACKGROUND

Jaundice within the first 1-2 weeks of a neonate's life will generally self-resolve; however, if it lasts longer than this time frame it warrants further work up. Direct or conjugated hyperbilirubinemia can suggest neonatal cholestasis, which in turn reflects marked reduction in bile secretion and flow. The differential diagnosis for neonatal cholestasis is broad. Neonatal choledocholithiasis is a rare cause of neonatal cholestasis, but should be considered on the differential diagnosis for patients presenting with elevated conjugated bilirubin.

CASE PRESENTATION

We describe an infant who presented with neonatal cholestasis. He subsequently underwent work up for biliary atresia, as this is one of the more time-sensitive diagnoses that must be made in neonates with conjugated hyperbilirubinemia. He was ultimately found to have choledocholithiasis on magnetic resonance cholangiopancreatography. He was managed conservatively with optimizing nutrition and ursodeoxycholic acid therapy.

CONCLUSIONS

We found that conservative management, specifically optimizing nutrition and treating with ursodeoxycholic acid, can be a sufficient approach to facilitating resolution of the choledocholithiasis and conjugated hyperbilirubinemia.

摘要

背景

新生儿生命的头 1-2 周内出现的黄疸通常会自行消退;然而,如果持续时间超过这个时间框架,则需要进一步检查。直接或结合胆红素升高提示新生儿胆汁淤积症,这反过来反映了胆汁分泌和流动的明显减少。新生儿胆汁淤积症的鉴别诊断范围很广。新生儿胆总管结石是新生儿胆汁淤积症的罕见病因,但对于出现结合胆红素升高的患者,应在鉴别诊断中考虑到这一点。

病例介绍

我们描述了一名患有新生儿胆汁淤积症的婴儿。他随后接受了胆道闭锁的检查,因为这是新生儿结合性高胆红素血症中必须做出的更具时间敏感性的诊断之一。他最终在磁共振胰胆管造影术上发现了胆总管结石。他通过优化营养和熊去氧胆酸治疗进行保守治疗。

结论

我们发现,保守治疗,特别是优化营养和用熊去氧胆酸治疗,可以是促进胆总管结石和结合性高胆红素血症消退的有效方法。

相似文献

1
Rolling stones: an instructive case of neonatal cholestasis.滚动的石头:新生儿胆汁淤积症的一个启发性病例。
BMC Pediatr. 2022 Sep 4;22(1):526. doi: 10.1186/s12887-022-03560-3.
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Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition.婴儿胆汁淤积性黄疸评估指南:北美小儿胃肠病、肝病和营养学会与欧洲小儿胃肠病、肝病和营养学会联合推荐
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Conjugated hyperbilirubinemia in children.儿童结合胆红素血症
Pediatr Rev. 2012 Jul;33(7):291-302. doi: 10.1542/pir.33-7-291.
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Neonatal jaundice.新生儿黄疸。
Clin Res Hepatol Gastroenterol. 2012 Jun;36(3):253-6. doi: 10.1016/j.clinre.2012.03.018. Epub 2012 May 18.
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Neonatal cholestasis.新生儿胆汁淤积症
Semin Perinatol. 2004 Oct;28(5):348-55. doi: 10.1053/j.semperi.2004.09.008.
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Treatment of severe cholestasis in neonatal Dubin-Johnson syndrome with ursodeoxycholic acid.熊去氧胆酸治疗新生儿杜宾-约翰逊综合征的严重胆汁淤积症
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Hyperbilirubinemia and Choledocholithiasis in an Extremely Premature Neonate.极低体重新生儿高胆红素血症与胆总管结石
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[Congenital extrahepatic biliary atresia as a cause of cholestasis in newborns and infants].[先天性肝外胆管闭锁作为新生儿和婴儿胆汁淤积的原因]
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Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency.α-1抗胰蛋白酶缺乏症成人的肝胆表型
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Genome-wide association meta-analysis yields 20 loci associated with gallstone disease.全基因组关联荟萃分析产生 20 个与胆石病相关的位点。
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Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome: A Multicenter Study.区分胆道闭锁与其他婴儿胆汁淤积原因的肝活检关键组织病理学特征及其与预后的相关性:一项多中心研究。
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Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition.婴儿胆汁淤积性黄疸评估指南:北美小儿胃肠病、肝病和营养学会与欧洲小儿胃肠病、肝病和营养学会联合推荐
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Neonatal jaundice.新生儿黄疸
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Clinical practice: management of biliary atresia.临床实践:先天性胆道闭锁的管理。
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