Treatment of the causes of many chronic
liver diseases (
CLDs) may not be possible. In this case, complications must be anticipated, prevented or at least controlled by the best available therapeutic modalities. There are three main goals for the management of
portal hypertension: (i) prevention of the first episode of variceal
bleeding largely by non-selective β-
adrenoceptor antagonists, which is not generally recommended in children; (ii) control of
bleeding by using a stepwise approach from the least to most invasive strategies; (iii) and prevention of re-
bleeding using bypass operations, with particular enthusiasm for the use of meso-Rex bypass in the pediatric population.
Hepatic encephalopathy management also consists of three main aspects: (i) ruling out other causes of
encephalopathy; (ii) identifying and treating precipitating factors; and (iii) starting empiric treatment with drugs such as
lactulose,
rifaximin,
sodium benzoate, and
flumazenil. Treatment of mild
ascites and peripheral
edema should begin with the restriction of
sodium and water, followed by careful diuresis, then large-volume paracentesis associated with
colloid volume expansion in severe cases. Empiric broad spectrum antimicrobial
therapy should be used for the treatment of spontaneous bacterial
peritonitis, bacterial and fungal
sepsis, and
cholangitis, after taking appropriate cultures, with appropriate changes in
therapy after sensitivity testing. Empirical
therapies continue to be the standard practice for
pruritus; these consist of
bile acid binding agents,
phenobarbital (
phenobarbitone),
ursodeoxycholic acid,
antihistamines,
rifampin (
rifampicin), and
carbamazepine. Partial external biliary diversion can be used in refractory cases. Once
hepatorenal syndrome is suspected, treatment should be initiated early in order to prevent the progression of
renal failure; approaches consist of general supportive measures, management of concomitant complications, screening for
sepsis, treatment with
antibiotics, use of
vasopressin analogs (
terlipressin), and
renal replacement therapy if needed.
Hepatopulmonary syndrome and portopulmonary
hypertension are best managed by
liver transplantation. Provision of an adequate caloric supply, nutrition, and
vitamin/
mineral supplements for the management of growth failure, required vaccinations, and special care for ensuring psychologic well-being should be ensured. Anticoagulation might be attempted in acute portal vein
thrombosis. Some
CLDs, such as
extrahepatic biliary atresia (EHBA),
Crigler-Najjar syndrome, and
Indian childhood cirrhosis, require special considerations. For EHBA, Kasai
hepatoportoenterostomy is the current standard surgical approach in combination with nutritional
therapy and supplemental fat and water soluble
vitamins, minerals, and
trace elements. In type 1
Crigler-Najjar syndrome, extensive
phototherapy is the mainstay of treatment, in association with adjuvant
therapy to bind
photobilirubin such as
calcium phosphate,
cholestyramine, or
agar, until
liver transplantation can be carried out. Treating
Indian childhood cirrhosis with
penicillamine early in the course of the disease and at doses similar to those used to treat
Wilson disease decreases the mortality rate by half. New hopes for the future include extracorporeal liver support devices (the molecular adsorbent recirculating system [MARS®] and Prometheus®), hepatocyte
transplantation, liver-directed gene therapy, genetically engineered
enzymes, and therapeutic modalities targeting fibrogenesis. Hepapoietin, a naturally occurring
cytokine that promotes hepatocyte growth, is under extensive research.