Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease

Table 1 Demographics differences in sickle cell disease patients with and without severe extreme hyperbilirubinemia

	SCD with EH	SCD without EH	P values
Gender: Male (percent)	21 (50%)	35 (35%)	0.11
Age in yr (mean ± SD)	29.5± 11.20	32.6 ± 13.20	0.19
Race (African American)	41 (98%)	97 (98%)	0.89

SCD: Sickle cell disease; EH: Extreme hyperbilirubinemia.

Table 2 Lab values and outcomes in sickle cell disease patients with and without severe sickle cell hepatopathy

	SCD with severe SCH	SCD without SCH	P values
Alkaline Phosphatase (IU/L; mean ± SD)	218.0 ± 176.2	85.9 ± 68.4	< 0.001
Alanine Aminotransferase (IU/L; mean ± SD)	157.0 ± 266.2	19.8 ± 21.3	< 0.001
Aspartate Aminotransferase (U/L; mean ± SD)	256.5 ± 485.9	28.2 ± 14.7	< 0.001
Direct Bilirubin (mg/dL; mean ± SD)	11.73 ± 6.03	0.28 ± 0.07	< 0.001
Total Bilirubin (mg/dL; mean ± SD)	18.41 ± 6.74	1.29 ± 0.19	< 0.001
qSOFA (mean ± SD)	0.42 ± 0.68	0.01 ± 0.12	< 0.001
Need for blood transfusion (number, percent)	24 (63%)	4 (5%)	< 0.001
Need for Exchange Transfusion (number, percent)	4 (10.5%)	1 (1.3%)	0.022
Mortality within 1 yr (number, percent)	2 (4.7%)	0 (0%)	0.09

SCD: Sickle cell disease; SCH: Sickle cell hepatopathy; qSOFA: Quick Sequential Organ Failure Assessment.

Table 3 Hemoglobin genotype breakdown between the extreme hyperbilirubinemia and control groups

	Hemoglobin SS (n)	Hemoglobin S Beta+ Thalassemia (n)	Hemoglobin S Beta null Thalassemia (n)	Hemoglobin S Beta (unknown) Thalassemia (n)	Hemoglobin SC (n)
Extreme hyperbilirubinemia	40	2	0	0	0
Control	33	11	2	2	51

EH: Extreme hyperbilirubinemia.