Medical Care
Much medical care in patients with cholestasis is disease specific.
Cholestasis often does not respond to medical therapy of any sort. Some reports indicate success in children with chronic cholestatic diseases with the use of ursodeoxycholic acid (20-30 mg/kg/d), which acts to increase bile formation and antagonizes the effect of hydrophobic bile acids on biological membranes. Phenobarbital (5 mg/kg/d) may also be useful in some children with chronic cholestasis.
Treatment of fat malabsorption principally involves dietary substitution. In older patients, a diet that is rich in carbohydrates and proteins can be substituted for a diet containing long-chain triglycerides. In infants, that may not be possible, and substitution of a formula containing medium-chain triglycerides may improve fat absorption and nutrition. This, however, has not clearly been proven, and therapeutic formulas containing medium-chain triglycerides may not be worth their expense. Bile salt therapy to replace missing bile salts is not practical. Ursodeoxycholic acid, which is used to treat some cholestatic conditions, does not form mixed micelles and has no effect on fat absorption.
In chronic cholestasis, careful attention must be paid to prevent fat-soluble vitamin deficiencies, which are common complications in pediatric patients with chronic cholestasis. This is accomplished by administering fat-soluble vitamins and monitoring the response to therapy. Oral absorbable, fat-soluble vitamin formulation A, D, E, and K supplementation is safe and potentially effective in pediatric patients with cholestasis.
Surgical Care
Surgical care is disease specific; therefore, refer to articles about disease states (see Causes).
Consultations
Referral to a specialist in gastroenterology or hepatology is indicated for any patient with cholestatic liver disease, particularly if it is severe or prolonged.
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Eruptive xanthomas. Courtesy of Duke University Medical Center.
