Table 1.
ALGS population: patient demographic and clinical characteristics reported by caregivers (for patients under 18 years of age) or patients
| Demographic and clinical characteristics | N = 25 |
|---|---|
| Sex, n (%) | |
| Male | 10 (40.0) |
| Female | 15 (60.0) |
| Age, years | |
| Mean | 8.3 |
| Median | 5.8 |
| Range | 0.44–34.9 |
| Race/ethnicitya, n (%) | |
| Asian | 1 (4.0) |
| Black/African–American | 5 (20.0) |
| Hispanic/Spanish American/Latino (of any race) | 4 (16.0) |
| White/Caucasian | 14 (56.0) |
| Severity of reported itching, n (%) | |
| Very mild | 7 (28.0) |
| Mild | 7 (28.0) |
| Moderate | 7 (28.0) |
| Severe | 4 (16.0) |
| Patient receiving treatment for any aspect of ALGS, n (%) | |
| Yes | 20 (80.0) |
| No | 2 (8.0) |
| Not answered | 3 (12.0) |
| Patient has undergone surgery for any aspect of ALGSb, n (%) | |
| Yes | 9 (36.0) |
| No | 14 (56.0) |
| Not answered | 2 (8.0) |
| Type of surgery for ALGS, n (%) | |
| Biliary diversionc | 3 (12.0) |
| Open biopsy and catheterization | 1 (4.0) |
| Tetralogy of Fallot repair | 1 (4.0) |
| Liver biopsy | 1 (4.0) |
| Liver transplant | 1 (4.0) |
| Bone graft/hardware | 1 (4.0) |
| Not answeredd | 17 (68.0) |
| Patient receiving treatment for itching, n (%) | |
| Yes | 18 (72.0) |
| No | 4 (16.0) |
| Not answered | 3 (12.0) |
| Current itching treatmentse, n (%) | |
| Rifampin | 12 (48.0) |
| Hydroxyzine (Atarax) | 10 (40.0) |
| Ursodeoxycholic acid | 6 (24.0) |
| Naltrexone | 3 (12.0) |
| Clonidine | 1 (4.0) |
| Unspecified medications | 3 (12.0) |
ALGS Alagille syndrome
aRace information was not provided for one participant
bWhile any surgical interruption of the enterohepatic circulation and liver transplant were exclusion criteria for this study, all patients who had undergone these surgery types continued to experience itching following surgery and were, therefore, included in the study
cIncluding partial external biliary diversion, ileal exclusion, biliary diversion and partial internal biliary diversion
dIncludes one patient for whom surgery type was not specified
eMore than one treatment was reported for some participants