Hepatobiliary Manifestations of Sickle Cell Anemia

Issa, Hussain; Al-Salem, Ahmed H.

doi:10.4021/gr2010.01.1332

Cited by 18 publications

(36 citation statements)

References 55 publications

(62 reference statements)

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“…Clinically, it resembles acute hepatic crisis but the main differentiating points is the associated extreme hyperbilirubinemia, coagulopathy and acute hepatic failure and renal impairment. [2,4] Plasma ALT, AST and alkaline phosphatase levels are very high. Prothrombin time and partial thromboplastin time are prolonged; blood urea, creatinine, and ammonia are elevated.…”

Section: Hepatomegalymentioning

confidence: 99%

“…It can be successfully treated with a regular exchange blood transfusion program with or without hydroxyurea. [2] Acute sickle cell hepatic crisis It occurs in approximately 10% of patients with sickle cell anaemia. Patients present with acute right upper quadrant pain, nausea, low grade fever, tender hepatomegaly, and jaundice.…”

Section: Hepatomegalymentioning

confidence: 99%

“…Acute viral hepatitis has the same clinical course in the sickling disorders as in the general population, other than a higher peak bilirubin level reflecting baseline haemolysis. [2,3] Intrahepatic cholestasis Acute sickle cell intrahepatic cholestasis is a rare but a serious and sometimes fatal complication of SCA. It results from severe obstruction of the liver sinusoids leading to stasis, hypoxia and intracanalicular cholestasis secondary to ballooning of the hepatocytes.…”

Section: Hepatomegalymentioning

confidence: 99%

“…[4] Early diagnosis, intensive supportive care including exchange blood transfusion, fresh frozen plasma, platelet transfusion and plasmapheresis may reverse the process of intrahepatic sickling and cholestasis resulting in a favourable outcome. [2] Chronic intrahepatic cholestasis is a mild benign condition characterised by prolonged hyperbilirubinemia in the absence of right upper guardant abdominal pain and evidence of haemolysis. It can be successfully treated with a regular exchange blood transfusion program with or without hydroxyurea.…”

Section: Hepatomegalymentioning

confidence: 99%

“…[1] Sickle cell disease is a multisystem disorder can affect any part of the body .One of the main organ to be affected is hepatobiliary system. [2] Liver and biliary tract abnormalities are common in sickle cell disorder. The liver can be affected by a number of complications due to the disease itself and its treatment.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Bokade¹,

Chauhan²,

Dhole³

2017

Ann. Int. Med. Den.Res.

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Section: Hepatomegalymentioning

confidence: 99%

Section: Hepatomegalymentioning

confidence: 99%

Section: Hepatomegalymentioning

confidence: 99%

Section: Hepatomegalymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Bokade¹,

Chauhan²,

Dhole³

2017

Ann. Int. Med. Den.Res.

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Hydroxyurea treatment for adult sickle cell anemia patients in Kinshasa

Kabuyi

Mbayabo

Ngole

et al. 2023

eJHaem

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Background: Despite a high incidence of sickle cell anemia, hydroxyurea (HU) treatment is rarely used in the DR Congo. This study aims to assess the efficacy of HU, the incidence of side effects that may limit its use in adults and to determine the dose needed for clinical improvement in patients.Methods: In a prospective study, patients received an initial dose of 15 mg/kg/day which was increased by 5 mg/kg every 6 months, up to a maximum of 30 mg/kg/day. The response and side effects to HU were evaluated biologically and clinically every 3 months during a 2‐year period.Results: Seventy adult patients with a moderate or severe clinical phenotype initiated treatment. Only minor side effects were reported. At the end of the 2‐year treatment phase, 45 (64.3%) had dropped out, of whom 33 were without a clear reason. Clinical and biological improvement was more marked during the first year. There was a reduction in severe vaso‐occlusive crises (p < 0.001), need for transfusion (p < 0.001), and hospitalization days (p = 0.038). Fetal hemoglobin (HbF) levels increased on average 2.9 times after 12 months (p < 0.001). The increase in mean corpuscular volume was greater in the first year (p < 0.001) than in the second year (p = 0.041). The decrease in leukocytes (p < 0.001) was significant during the first year. In 70% of patients, the 20 mg/kg/day dose was needed to reach the 20% HbF threshold.Conclusion: HU is effective and well tolerated. The magnitude of the response varies from one patient to another. Improvement of clinical manifestations is achieved in most patients with a relatively low dose. Effective implementation of HU treatment will require improved adherence to treatment.

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Cholelithiasis in patients with paediatric sickle cell anaemia in a Saudi hospital

Al-Hawsawi

Alshenqeti

Alqarafi

et al. 2019

Journal of Taibah University Medical Sciences

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Objective Sickle cell disease is one of the most common inherited hemoglobinopathies in the world. Chronic haemolysis predisposes individuals to the development of bilirubinate cholelithiasis, which can be asymptomatic or can result in cholecystitis, choledocholithiasis, cholangitis, and gallstone pancreatitis. We aimed to determine the prevalence of cholelithiasis and associated gallstone disease among patients with paediatric sickle cell disease in a Saudi hospital. Methods This retrospective study was conducted among all patients aged between 2 and 18 years. We reviewed the medical records of patients diagnosed with sickle cell anaemia. Mean and standard deviation were calculated for quantitative variables, and the Student t-test was used to compare means. The chi-square test was used to assess those risk factors possibly associated with cholelithiasis. A P-value of ≤0.05 was considered statistically significant. Results Approximately 75% of participants developed cholelithiasis (27.5%) at a mean age of 6.9 ± 3.4 years. The frequency of cholelithiasis was significantly higher with increasing age (40.8% in participants 12 years and older) and among those with high levels of haemoglobin S (Hb S) and mean corpuscular volume (MCV). Moreover, cholelithiasis was more frequent among males than females, Saudis than non-Saudis, and in those with sickle cell disease than in those with sickle thalassemia. However, these differences were not statistically significant. Conclusion In this study, the prevalence of cholelithiasis among children with sickle cell anaemia was found to be high. This association was significantly increased with age and high levels of MCV and Hb S.

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Hepatobiliary Manifestations of Sickle Cell Anemia

Cited by 18 publications

References 55 publications

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Hydroxyurea treatment for adult sickle cell anemia patients in Kinshasa

Cholelithiasis in patients with paediatric sickle cell anaemia in a Saudi hospital

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