UGT1A1-related disorders in Bahrain: A genetic and clinical overview of Crigler-Najjar and Gilbert syndromes

Table 4 Management of patients with UGT1A1-related disorders, n (%)

Patient management	Total, n = 35 (100)	CNS-I, n = 1 (2.9)	CNS-II, n = 7 (20.0)	GS, n = 27 (77.1)
Medications
Phenobarbital	20 (57.1)	1 (100)	6 (85.7)	13 (48.1)
Folic acid	20 (57.1)	1 (100)	3 (42.9)	16 (59.3)
Vitamin D supplementation	20 (57.1)	1 (100)	1 (14.3)	18 (66.7)
Hydroxyurea	16 (45.7)	0 (0.0)	2 (28.6)	14 (51.9)
Ursodeoxycholic acid	13 (37.1)	0 (0.0)	1 (14.3)	12 (44.4)
Other medications1	16 (45.7)	1 (100)	1 (14.3)	14 (51.9)
Medical/surgical procedures
Blood transfusion	15 (42.9)	1 (100)	2 (28.6)	12 (44.4)
Phototherapy	13/31 (41.9)	1 (100)	4/6 (66.7)	8/24 (33.3)
Cholecystectomy	8 (22.9)	0 (0.0)	2 (28.6)	6 (22.2)
Splenectomy	6 (17.1)	0 (0.0)	1 (14.3)	5 (18.5)
Liver transplantation	1 (2.9)	1 (100)	0 (0.0)	0 (0.0)

¹Crigler-Najjar syndrome-I: Tacrolimus, magnesium supplement, and iron supplement (n = 1); Crigler-Najjar syndrome-II: Prophylactic antibiotics (n = 1); Gilbert syndrome: Prophylactic antibiotics and aspirin (n = 5, each), iron chelation (n = 4), laxative (n = 4), calcium supplement (n = 3); omeprazole (n = 2), rifampicin, erythromycin, multivitamins, fat-soluble vitamins, pancreatic enzyme replacement therapy (Creon), L-thyroxin, growth hormone, testosterone, warfarin, antihypertensives, frusemide, inhaled steroids, and bronchodilators (n = 1, each).

CNS: Crigler-Najjar syndrome; GS: Gilbert syndrome.