Alagille Syndrome, Biliary Atresia, Cholestasis
Conditions
Keywords
Alagille syndrome, biliary atresia, cholestasis, gastrointestinal disorders, rare disease
Brief summary
OBJECTIVES: I. Determine the role of magnesium deficiency in the pathogenesis of decreased serum vitamin D and reduced bone density in children with chronic cholestatic liver disease.
Detailed description
PROTOCOL OUTLINE: Patients receive magnesium sulfate IV over 1 hour on day 3. Patients then receive oral magnesium gluconate supplementation daily. Treatment with magnesium sulfate repeats once at 3-6 months.
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
PROTOCOL ENTRY CRITERIA: --Disease Characteristics-- * Diagnosis of liver disease with chronic cholestasis Nonsyndromic intrahepatic cholestasis Alagille's syndrome Extrahepatic biliary atresia * Direct bilirubin greater than 2 mg/dL OR Bile acids greater than 20 micromoles/L * No hepatic decompensation defined as one or more of the following: Ascites Peripheral edema PT at least 4 seconds longer than control Albumin less than 3 g/dL --Patient Characteristics-- * Renal: No significant renal disease * Cardiovascular: No significant cardiovascular disease * Pulmonary: No significant pulmonary disease
Countries
United States