Evaluating the Risk of Development of Autoimmune Liver Diseases in Children
after Liver Resection
Petrů O.1, Bartůňková J.2, Rygl M.1, Šnajdauf J.1
Klinika dětské chirurgie 2. LF UK v Praze, FN Motol, subkatedra dětské chirurgie IPVZ, Praha1přednosta prof. MUDr. J. Šnajdauf, DrSc. Ústav imunologie 2. LF UK , FN Motol, Praha2 přednostka prof. MUDr. J. Bartůňková, DrSc. |
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Summary:
Objective: Evaluation of the risk of development of autoimmune liver diseases in children,
having undergone liver resection for malignant and benign diseases.
Material: A group of 28 children having undergone liver resection at our workplace in the period
of 1991–2002.
Methods: Analysis of blood samples from children in various periods of time after liver resection
and evaluation of the presence of autoantibodies, associated with different types of autoimmune
diseases of liver (autoantibodies: ANA – antinuclear, AMA – antimitochondrial, ASMA –
against smooth muscles, LKM – antimicrosomes (endoplasmic reticulum) of liver and kidney, ANCA – anticytoplasm of neutrophiles). Dependence of the presence of autoantibodies on the time
after operation, age at the time of operation, type of the basal disease and the extent of resection
was evaluated. The correlation of positivity of autoantibodies and biochemical signs of liver
parenchyma damage was evaluated.
Results: In the period of 1991–2002, there were 56 liver resection interventions in children with
malignant or benign diseases performed at our workplace. At the present time 39 patients are alive
(23 boys and 16 girls). Twenty-seven children were operated on and treated with chemotherapy
for malignant tumors, 12 children underwent liver resection for benign affections (benign
tumors, liver trauma, focal nodular hyperplasia, cholangiogenous cyst). The age at the time of
operation was in the range of 0.5 to 14.5 years. The period of observation after the operation is in
the range of 1.5 to 13 years and the age of the patients is presently 2 to 23 years.
Twenty-eight patients (16 boys, 12 girls) were examined. The autoantibodies of the ANCA, AMA
and LKM were negative in all patients. Antinuclear antibodies (ANA) were positive in one patient
(3.6%) with the diagnosis of focal nodular hyperplasia. Autoantibodies against smooth muscles
(ASMA) were present in 5 children (17.9%), in the remaining 23 children (82.1%) being undetectable.
In all these five children (boys only) hepatoblastoma was the basal diagnosis and all of them
were examined more then 5 years (5–12.5) after liver resection. The positive finding correlated
with discrete biochemical symptoms of hepatic lesion (isolated marginal increase in AST, ALT,
GMT or bilirubin).
Conclusion: These results indicate that autoantibodies typical for autoimmune hepatitis of
type I (ASMA) occur in children, whose liver resection was made for hepatoblastoma in the time
lapse of more then 5 years after the operation. This kind of association excludes the possibility
that the formation of autoantibodies is induced only by damage of liver parenchyma during the
operation.
The long period of time after the operation and absence of tumor or infection disease at the
time of examination does not indicate any possibility of non-specific positivity associated with
mechanical damage, infectious or tumor disease. All five children with ASMA displayed discrete
symptoms of liver lesion. In view of the absence of symptoms of hepatolysis or marked cholestasis
it seems impossible to conclude, whether the presence of ASMA indicates a risk of origin of
autoimmune hepatitis at a later time. Based on our results we recommend to invite these patients
to regular control examinations.
Key words:
liver resection in children, autoimmune hepatitis, autoantibodies
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