Alagille Syndrome Follow-up

Updated: Oct 01, 2021
  • Author: Ann Scheimann, MD, MBA; Chief Editor: Carmen Cuffari, MD  more...
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Follow-up

Further Outpatient Care

Further care may include the following:

  • Monitor patient's nutritional status. Screen patients for fat-soluble vitamin deficiencies.

  • Consultation with a pediatric cardiologist for management of structural cardiac or vascular disease, and hyperlipidemia is advised.

  • Manage chronic cholestatic liver disease, including pruritus, cirrhosis and portal hypertension, ascites, and screening for hepatocellular carcinoma, when appropriate.

  • Manage chronic renal disease.

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Further Inpatient Care

Patients may require inpatient treatment for nutritional support, cardiovascular disease, and chronic liver disease.

  • Patients with evidence of undernutrition and failure of conservative measures may benefit from gastrostomy placement for initiation of nocturnal drip feedings.

  • Patients may require cardiac catheterization and cardiac or vascular surgery/procedures for significant symptomatic lesions.

  • Complications of chronic liver disease, including variceal hemorrhage, refractory ascites, and spontaneous bacterial peritonitis (as well as development of hepatocellular carcinoma), occur in patients with Alagille syndrome and long-standing liver involvement. These patients merit strong consideration for liver transplantation.

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Transfer

Patients with Alagille syndrome and significant cardiovascular or hepatic disease merit consultation with a subspecialist.

Consider transferring patient if signs of decompensation are evident upon presentation or if they are likely to evolve during hospitalization; patient also must be stable for transfer.

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Deterrence/Prevention

Patients with significant intracardiac disease require subacute bacterial endocarditis (SBE) prophylaxis. Consider trials of bile acid-binding resins (eg, cholestyramine) to those with significant hyperlipidemia and pruritus.

Supplementation of fat-soluble vitamins, alteration in dietary intake (higher carbohydrate/medium chain triglyceride), and immunizations (hepatitis B, hepatitis A, Pneumovax) may minimize the development of complications of cholestatic chronic liver disease.

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