Multiple Primary Choledocholithiasis in Sickle Cell Disease

Vicari, Perla; Gil, Marco Vinicio Fanucchi; Cavalheiro, Rita de Cassia Rosário; Figueiredo, Maria Stella

doi:10.2169/internalmedicine.47.1326

Cited by 12 publications

(10 citation statements)

References 6 publications

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“…Another relatively common complication of sickle cell anaemia is the development of choledocholithiasis which is usually secondary to cholelithiasis, but there is also primary choledocholithiasis [29]. In this study, none of the patients had choledocholithiasis.…”

Section: Discussionmentioning

confidence: 68%

Hepatobiliary Ultrasonographic Abnormalities in Adult Patients with Sickle Cell Anaemia in Steady State in Ile-Ife, Nigeria

et al. 2018

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SummaryBackgroundSickle cell anaemia (SCA) is associated with structural manifestations in the hepatobiliary axis. This study aimed to investigate the hepatobiliary ultrasonographic abnormalities in adult patients with sickle cell anaemia in steady state attending the Haematology clinic of a federal tertiary health institution in Ile-Ife, Nigeria.Material/MethodsBasic demographic data as well as right upper abdominal quadrant ultrasonography of 50 consecutive sickle cell anaemia patients were compared with those of 50 age- and sex-matched subjects with HbAA as controls.ResultsEach of the study groups (patients and controls) comprised of 21 (42%) males and 29 (58%) females. The age range of the patients was 18–45 years with a mean (±SD) of 27.6±7.607 years, while that of the controls was 21–43 years with a mean (±SD) of 28.0±5.079 years (p=0.746). Amongst the patients, 32 (64%) had hepatomegaly, 15 (30%) cholelithiasis and 3 (6%) biliary sludge. Fourteen (28%) of the patients had normal hepatobiliary ultrasound findings. In the control group, one (2%) person had cholelithiasis, one (2%) biliary sludge, one (2%) fatty liver and none hepatomegaly. Forty-seven (94%) of the controls had normal hepatobiliary ultrasound findings. There was a statistically significant difference in the prevalence of hepatomegaly and cholelithiasis between the patients and controls (p value <0.001 for both comparisons).ConclusionsIn this study, hepatomegaly, cholelithiasis and biliary sludge were the most common hepatobiliary ultrasound findings in patients with sickle cell anaemia. Ultrasonography is a useful tool for assessing hepatobiliary abnormalities in patients with sickle cell anaemia.

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Section: Discussionmentioning

confidence: 68%

Hepatobiliary Ultrasonographic Abnormalities in Adult Patients with Sickle Cell Anaemia in Steady State in Ile-Ife, Nigeria

et al. 2018

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“…The gallbladder is affected by hemoglobin (pigmented) stones [47], biliary sludge [48–50], and obstruction [51–53]. The liver is affected by vasoocclusive changes (right upper quadrant syndrome) of recurrent ischemia and reperfusion injuries [46, 54], iron overload from transfusions that are used to treat both symptomatic anemia and the complications of sickle cell disease [55–59], vascular endothelial dysfunction [60], and the liver consequences of the hypercoagulation of sickle cell [61–63].…”

Section: Gastrointestinal/hepatobiliary Complicationsmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

158

144

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The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

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“…In the general population with cholelithiasis, the incidence of common bile duct stones has been reported as 10%-15% [23]. In patients with SCA, the frequency of common bile duct stones is 18%-30% [23, 25, 26]. We found a 30% incidence of common bile duct stones in children with SCA undergoing cholecystectomy [26].…”

Section: Choledocholithiasismentioning

confidence: 80%

“…Another relatively common complication of SCA is the development of choledocholithiasis which is usually secondary to cholelithiasis but there are also primary choledocholithiasis [25] (Fig. 3).…”

Section: Choledocholithiasismentioning

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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Multiple Primary Choledocholithiasis in Sickle Cell Disease

Abstract: Abstract

Cited by 12 publications

References 6 publications

Hepatobiliary Ultrasonographic Abnormalities in Adult Patients with Sickle Cell Anaemia in Steady State in Ile-Ife, Nigeria

Hepatobiliary Ultrasonographic Abnormalities in Adult Patients with Sickle Cell Anaemia in Steady State in Ile-Ife, Nigeria

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Hepatobiliary Manifestations of Sickle Cell Anemia

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