Sickle cell cholangiopathy: An endoscopic retrogradecholangiopancreatography evaluation

Issa, Hussain; Al-Haddad, Ali; Al-Salem, Ahmed H.

doi:10.3748/wjg.15.5316

Cited by 14 publications

(9 citation statements)

References 14 publications

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“…Clinical feature: In early stages, most patients present with cholestatic jaundice. In a study done on 224 SCD patients with cholestatic jaundice receiving total of 242 ERCP, prevalence of dilated biliary ducts was 24.6%[ 45 ]. Common causes of biliary obstruction such as stones, mass have to be excluded prior to attributing these changes to cholangiopathy.…”

Section: Chronic Hepatobiliary Manifestations Of Sickle Cell Diseasementioning

confidence: 99%

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Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

Shah

Taborda

Chawla

2017

WJGP

102

117

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Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as “sickle cell hepatopathy”. This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis. In this review, we detail the pathophysiology, clinical presentation and biochemical features of various acute and chronic hepatobiliary manifestations of SCD and present and evaluate existing evidence with regards to management of this disease process. We also discuss recent advances and controversies such as the role of liver transplantation in sickle cell hepatopathy and highlight important questions in this field which would require further research. Our aim with this review is to help increase the understanding, aid in early diagnosis and improve management of this important disease process.

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Section: Chronic Hepatobiliary Manifestations Of Sickle Cell Diseasementioning

confidence: 99%

“…Treatment: Patient who are asymptomatic but are found to be having dilated biliary ducts, should be closely followed up since they are at a high risk of having bile duct stones[ 45 ]. Endoscopic therapy is the mainstay for patients with choledocholithiasis or biliary strictures.…”

Section: Chronic Hepatobiliary Manifestations Of Sickle Cell Diseasementioning

confidence: 99%

Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

Shah

Taborda

Chawla

2017

WJGP

102

117

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“…The benign pancreatic tumor found in another patient was not linked to SCD but required regular controls. The prevalence of biliary disease in SCD could increase with age, because dilation of bile ducts without obstruction was found via endoscopic retrograde cholangiopancreatography (ERCP) in 27% of young adults with SCD and cholestatic jaundice [23]. Also, PSC will probably be more frequently diagnosed in SCD patients because the global incidence of inflammatory bowel disease is increasing and PSC develops in 5% of patients with ulcerative colitis.…”

Section: Discussionmentioning

confidence: 99%

Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients

Allali

Montalembert

Brousse

et al. 2019

JCM

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Hepatobiliary complications in children with sickle cell disease (SCD) are rarely reported but can be life-threatening. We retrospectively assessed their prevalence in a cohort of 616 children followed in a French university-hospital SCD reference center. Eligibility criteria were the following: age <18 years, seen at least twice with an interval of more than 6 months from January 2008 to December 2017, with all genotypes of SCD. Patients with hepatobiliary complications were identified via the local data warehouse and medical files were thoroughly reviewed. At least one hepatobiliary complication was reported in 37% of the children. The most frequent was cholelithiasis, in 25% of cases, which led to systematic screening and elective cholecystectomy in the case of gallstones. Overall, 6% of the children experienced acute sickle cell hepatic crisis, sickle cell intra-hepatic cholestasis, or acute hepatic sequestration, with severity ranging from mild liver pain and increased jaundice to multiple organ failure and death. Emergency treatment was exchange transfusion, which led to normalization of liver tests in most cases. Five children had chronic cholangiopathy, associated with auto-immune hepatitis in two cases. One needed liver transplantation, having a good outcome but with many complications. Transfusion iron load and infectious hepatitis cases were mild. Hepatotoxicity of an iron chelator was suspected to contribute to abnormal liver test results in five patients. We propose recommendations to prevent, explore, and treat hepatobiliary complications in SCD children. We underline the need for emergency exchange transfusion when acute liver failure develops and warn against liver biopsy and transplantation in this condition.

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“…6). We have evaluated 224 SCA patients who had ERCP as part of their work up for cholestatic jaundice and 50 (24.6%) of them were found to have bile duct dilatation without an obstructive cause [36]. The reason for this dilation is not exactly known and none of them had previously cholelithiasis or choledocholithiasis.…”

Section: Sickle Cell Cholangiopathymentioning

confidence: 99%

“…Documenting this is of great importance as these patients need to be followed up regularly for the possibility of developing bile duct stones. Taking in consideration the high frequency of biliary sludge and the possibility of bile duct stones formation in these patients, endoscopic sphincterotomy may be beneficial as this may obviate the future development of bile duct stones [36, 37]. This as well as the value of endoscopic sphincterotomy in this group of patients needs however to be evaluated further.…”

Section: Sickle Cell Cholangiopathymentioning

confidence: 99%

Hepatobiliary Manifestations of Sickle Cell Anemia

Issa

Al-Salem

2010

Gastroenterol Res

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Sickle cell anemia is one of the common hemoglobinopathies around the world. It results from a single change of one amino acid valine instead of glutamic acid in the hemoglobin beta change. This change leads to polymerization of the hemoglobin when the oxygen saturation is lowered, resulting in deformity of the red blood cells and microvascular occlusion. Sickle cell anemia can affect any part of the body and one of the main organs to be affected is the hepatobiliary system either directly from the sicklening process or indirectly as a result of chronic hemolysis and multiple blood transfusions. This manifests in several clinical conditions which poses diagnostic and therapeutic dilemmas to the treating physicians. These hepatobiliary manifestations will be outlined in this review.

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Sickle cell cholangiopathy: An endoscopic retrogradecholangiopancreatography evaluation

Abstract: Considering the high frequency of biliary sludge and bile duct stones in SCD, endoscopic sphincterotomy might prove helpful in these patients.

Cited by 14 publications

References 14 publications

Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients

Hepatobiliary Manifestations of Sickle Cell Anemia

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