The incidence of pancreatitis in HIV-infected children is not well kno
wn. Medical records of 42 children with HIV infection followed at Chil
dren's Hospital during a 6-year period were reviewed. Pancreatitis (el
evated serum lipase levels) developed in 10 children (23.8%). Three ch
ildren acquired HIV infection from vertical transmission and seven fro
m contaminated blood products (hemophiliacs). Nine were severely immun
osuppressed (CD4(+) of <100 cells/mm(3)). Lipase values were more ofte
n elevated than amylase values. The clinical course was protracted and
severe in two children, one had four recurrences, and seven had only
a single episode of pancreatitis lasting a few weeks. Opportunistic in
fections were present in four children and seven were receiving medica
tions previously implicated as cause of pancreatitis. Discontinuation
of dideoxynosine (ddI) in one child led to rapid resolution of pancrea
titis, but continuation of medications in the other children did not a
lter the course. The etiology of pancreatitis may be multifactorial. S
evere and prolonged clinical course is associated with advanced HIV in
fection. Determination of serum lipase is more useful than serum amyla
se for identifying those with pancreatitis.