Study of Hepatobiliary Involvement in Children with Sickle Cell Disease

Bokade, Chandrakant M; Chauhan, Urmila; Dhole, Charushila

doi:10.21276/aimdr.2017.3.3.pe3

Cited by 3 publications

(8 citation statements)

References 8 publications

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“…Majority of these patients were in the age group of 6.1-12 years (53%), followed by the age group of 12.1-18 years (25%) and the least number of patients were under 6 (22%) years. Our results were similar to the study done by Bokade et al, 3 in Nagpur, where majority of their patients, i.e., 49.23%, also belonged to the age group of 6.1-12 years, followed by 27.18% between 3.1 and 6 years and 23.59% in 6 months and 3 years age group. Almost equal number of males and females (52.5% of females and 47.5% of males) were enrolled in our study.…”

Section: Discussionsupporting

confidence: 92%

“…Hepatitis B antigen status was negative in all these patients. Similar results were observed in studies done by Botezelli et al, 5 and Bokade et al, 3 where hepatobiliary involvement was observed in 47% and 44% of the patients, respectively. The spectrum of hepatobiliary involvement, as studied by Bokade et al, 3 was such that, Acute hepatic crisis was found in only in 1.17% of patients, hepatic sequestration in 2.35%, cirrhosis in 4.71%, cholelithiasis in 28.24%, cholecystitis in 27.06%, and viral hepatitis in 36.47% of patients.…”

Section: Discussionsupporting

confidence: 91%

“…The most common symptom at presentation was yellowish discoloration of the skin (39.10%), followed by fever seen in 36.10% of patients, abdominal pain in 20.80%, vomiting in 14.40% and 0.9% of patients had some form of bleeding manifestation. Similar to our study, Bokade et al, 3 in their study observed that yellowish discoloration was the most common presentation of patients, seen in 61.02% of patients, followed by abdominal pain in 37.4% of patients, fever in 28.71%, and vomiting in 23.58%. In another study, Mohanty et al, 4 studied 60 patients with SCD, and in their study, fever was the most common symptom at presentation seen in 83.3% of patients.…”

Section: Discussionsupporting

confidence: 91%

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Study of spectrum of hepatobiliary involvement in children with sickle cell disease

Dey,

Preeti Malpani,

Urvashi Channa

2023

Asian J Med Sci

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Background: Sickle cell disease (SCD) has various complications among which one of the main organs to be affected is the hepatobiliary system ranging from mild liver function test derangements to significant hepatic abnormalities with marked hyperbilirubinemia. Aims and Objectives: We studied the spectrum of hepatobiliary involvement in children with SCD and its association with various factors. Materials and Methods: This cross-sectional observational study was conducted at M.Y Hospital Indore, a tertiary centre in central India. All old and new SCD patients under age of 18 years were enrolled sequentially, their detailed history and thorough clinical examination based on a pre-structured proforma noted. Laboratory investigations and radiological evaluation were performed to assess hepatobiliary involvement and its association with various factors was studied. Results: Two hundred and two patients with SCD were enrolled during the study period and hepatobiliary involvement was seen in 84 (41.5%) subjects. The most common acute complication was acute hepatic crisis, (20; 9.9%) followed by hepatic sequestration and intrahepatic cholestasis (2; 0.9%). Cholelithiasis was the only chronic complication, seen in 60 (29.7%) patients. Maximum number of patients (53%) were in the age group of 6.1–12 years, least (22%) belonged to 6 months–6 years of age. In our study, 52.5% of patients were females. Symptoms with hepatobiliary involvement were yellowish discoloration of eyes (39.10%), fever (36.10%), abdominal pain (20.8%), vomiting (14.4%), and bleeding (0.9%). Indirect and direct bilirubin, liver enzymes were raised, coagulation profile deranged, and the synthetic function of liver reduced. The most common USG finding was hepatomegaly, seen in 111 (54.9%) patients. Significant association between hepatobiliary involvement with higher HbS concentration, increased frequency of vaso-occlusive crisis/year, and higher transfusion requirement was noted. Conclusion: There is significant hepatobiliary involvement in patients with SCD, accounting for great morbidity and mortality requiring high degree of clinical suspicion for timely diagnosis and early treatment.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 91%

See 1 more Smart Citation

Study of spectrum of hepatobiliary involvement in children with sickle cell disease

Dey,

Preeti Malpani,

Urvashi Channa

2023

Asian J Med Sci

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“…The study done by Bokade, fever was found in 28.71% of patients, yellowish discoloration of urine and eyes in 61%, and right abdominal pain in 37.4%. 6 In Johnson et al study, bone pain was present in 72% of patients and fever in 34%. 7 In the Mahebba study, bone pain was present in 100% of patients and abdominal pain in 1 % of patients.…”

Section: Discussionmentioning

confidence: 89%

“…Early detection of hepatic dysfunction is essential in reducing the morbidity and mortality in SCD. 4 Sickle cell hemoglobinopathy is common in Western Odisha. 5 A significant number of these patients have hepatobiliary manifestations.…”

mentioning

confidence: 99%

A study of hepatobiliary involvement in adult patients with sickle cell disease

et al. 2020

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Background: Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the ß-globin chain. Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the adult age group are limited. Aim of our study aims to find the prevalence of hepatic-biliary involvement in Sickle cell disease in adult patients admitted to our hospital.Methods: A prospective study was done for a period of two years from October 2017 to October 2019. Subjects of both sexes above the age of 18 years with SCD admitted to our hospital were enrolled. Thorough history taking, full clinical examination, hematological and biochemical parameters assessment, and abdominal ultrasonographic studies were performed to all patients.Results: The results obtained showed that 59% of patients had hepatobiliary involvement. The most common symptom among these patients was bone pain, and the pallor was the most common sign. Biochemical tests revealed reduced hemoglobin concentration, elevated bilirubin, and compromised liver function. The most common ultrasound finding in this study was hepatomegaly, hepatosplenomegaly, cholelithiasis, and gall bladder sludge. The incidence of viral hepatitis was low compared to previous studies. Other hepatobiliary complications were cholelithiasis (14%), benign hyperbilirubinemia (14%), cholecystitis (8%) hepatic crisis (9%), hepatic sequestration (1%) , liver cirrhosis (1%), choledocholithiasis (1%) and cholangitis (1%).Conclusions:Hepatobiliary complications, particularly gallbladder diseases, are frequent among SCD patients. The early detection of hepatobiliary complications by repeated ultrasound screening and liver function tests is significant as their frequency and intensity are related to the patient's age and the duration.

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