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Index gauges liver disease in hemorrhagic telangiectasia

Index gauges liver disease in hemorrhagic telangiectasia

By David Douglas

NEW YORK (Reuters Health) - A four-item scoring index helps assess liver involvement in hereditary hemorrhagic telangiectasia (HHT), investigators say. The tool, which incorporates age, gender, hemoglobin and alkaline phosphatase at presentation, "can categorize patients with HHT into low, moderate or high risk" of significant liver disease, Dr. Siddharth Singh told Reuters Health by email.

"Since universal screening for liver involvement in HHT is not recommended, this simple scoring system may allow individualized and cost-effective screening in a selective subset of patients, and also help physicians in monitoring these patients," Dr. Singh said.

Although symptomatic liver disease is rare in HHT, it may be fatal without liver transplantation, Dr. Singh and colleagues wrote in a March 7th online paper in the Journal of Hepatology.

They noted that in a recent study of 154 unselected HHT patients with hepatic involvement, 5.2% died and 25.3% had complications due to liver vascular malformations over a median follow-up of just over 3.5 years.

Although these patients were identified on screening, factors associated with clinically significant liver disease in patients with HHT are unknown. To learn more, the researchers screened 316 patients with definite HHT according to a systematic protocol that included contrast-enhanced hepatic CT and abdominal ultrasound.

Roughly half the cohort (171 patients; 54.1%) had hepatic involvement on imaging; 101 of these were female.

Using data from their patients, the team developed a system using cardiac failure, portal hypertension, or biliary disease to identify the presence of symptomatic liver disease. Being at risk (having liver abnormalities without symptoms) was identified via hepatic bruit, abnormal liver biochemistry, or an elevated cardiac index.

Overall, 45 patients had symptomatic liver disease, including 22 with high-output heart failure, and 45 were deemed to be at risk.

Using multivariable logistic regression analysis, the investigators derived a four-item score using age, gender, hemoglobin and alkaline phosphatase at presentation. The score, they say, can accurately distinguish patients with clinically significant liver involvement from patients with no or incidental liver lesions.

Age at or below 47 years gave a score of 0, greater age scored 1. Being male had a score of 0 and being female rated as 1. Hemoglobin at presentation gave scores of 0 to 3; for alkaline phosphatase at presentation, the range was from 0 to 4.

A score below 3 indicated low risk (less than 5%) and one of greater than 6 indicated high risk (more than 80%) of harboring clinically significant liver disease.

The team stresses that "external validation in prospective cohort studies is warranted before widespread applicability of this clinical scoring index."

Should this be achieved, they conclude, "this index could be used for individualized and cost effective screening for hepatic vascular malformations in patients with HHT, and also help researchers in stratifying patients for inclusion in clinical trials of disease-modifying medications."


J Hepatol 2014.

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