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."
SOURCE: http://bit.ly/1kDYxYO
J Hepatol 2014.
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