A prospective study of the association between sickle cell disease and hepatobiliary effects in Bahrain

Figures & data

Table 1 Diagnostic tests and procedures performed

Demographic data	Values
Number of patients	154
Age, years (mean, min–max)	31 (18–57)
Females (N, %)	99 (64%)
Males (N, %)	55 (36%)
Length of hospital stay, days (mean, min–max)	11 (1–119)
Number of admissions (mean, min–max)	50 (2–259)
Hospitalization outcome – alive (N, %)	152 (99%)
Clinical presentation (hemoglobin structure)	S-thal (7, 5%)
	SS (147, 95%)
Other comorbidities (N)	Asthma (1)
	Diabetes (1)
	Diabetes and dyslipidemia (1)
	Hypertension and cardiomyopathy (2)
Symptoms (N, %)	Bone pain (123, 80%)
	Abdominal pain (1, 1%)
Diagnosis (N, %)	Bone crisis (154, 100%)

Abbreviations: S-thal, hemoglobin S/thalassemia; SS, hemoglobin S – sickle cell.

Table 2 Hematological and biochemical profiles of the study cohort

Biochemical tests	Results	Normal range	Unit
Hemoglobin (Hb) (mean, min–max)	9.3 (5.7–13)	12.0–14.5	g/dL
HbS level (mean, min–max)	73 (30–94)	N/A	g/dL
HbF level (mean, min–max)	16 (1.1–75)	N/A	g/dL
HbA level (mean, min–max)	4 (0–56)	N/A	g/dL
Bilirubin level (mean, min–max)	33 (6–211)	5–21	µmol/L
Direct bilirubin (mean, min–max)	10 (2–116)	N/A	µmol/L
Indirect bilirubin (mean, min–max)	25 (5–116)	N/A	µmol/L
Albumin (mean, min–max)	38 (21–48)	35–52	g/L
WBC (mean, min–max)	10 (3.6–22)	3.6–9.6	×10^9/L
Platelet (mean, min–max)	250 (65–653)	150–400	×10^9/L
G6PD deficiency	Yes (14, 9%)	N/A	N/A
	No (133, 86%)	N/A	N/A
Liver function (mean, min–max)	ALT (55, 14–240)	<33	U/L
	ALP (118, 50–589)	50–136	U/L
	GGT (108, 11–1028)	5–55	U/L
Hepatitis B (HbsAg) (N, %)	Negative (154, 100%)	N/A	N/A
Hepatitis B core (anti-HBc) (N, %)	No (154, 100%)	N/A	N/A
Hepatitis C (anti-HCV) (N, %)	Positive (14, 9%)	N/A	N/A
	Negative (140, 91%)	N/A	N/A

Abbreviations: WBC, white blood cell; G6PD, glucose-6-phosphate dehydrogenase; ALT, alanine aminotransferase; ALP, alanine phosphatase; GGT, γ-glutamyltransferase; HbS, hemoglobin type S; HbA, adult hemoglobin type A; HbF, fetal hemoglobin type F; HbsAg, hepatitis B surface antigen; N/A, not applicable.

Figure 1 Abdomen ultrasound scanning results: a pie chart showing the types and frequency of pathological findings.

Abbreviations: GB, gallbladder; infart, infarction; 5IHD, type 5 intrahepatic bile duct.

Table 3 Diagnostic tests and procedures performed

Procedures	Results
Abdominal US (N, %)	Yes (65, 42%)
	No (89, 58%)
ERCP (N, %)	Yes (12, 8%)
	No (142, 92%)
ERCP indication (N, %)	Dilated bile ducts (8, 67%)
	Dilated bile ducts and stones (3, 25%)
	Cholestatic jaundice (1, 8%)
ERCP results (N, %)	Dilated bile ducts without stones (7, 58%)
	Dilated bile ducts with stones (4, 33%)
	Normal (1, 8%)
Laparoscopic cholecystectomy surgery (N, %)	Yes (14, 9%)
	No (140, 91%)
Sphincterotomy (N, %)	Yes (12, 8%)
Splenectomy (N, %)	3 (2%)
Treatment (N, %)	Blood transfusion (17, 11%)
	Deferoxamine (1, 1%)
	None (136, 88%)
Number of PRBC units (mean, min–max)	2 (1–2)

Abbreviations: US, ultrasound scanning; ERCP, endoscopic retrograde cholangiopancreatography; PRBC, packed red blood cells.