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Liver transplantation for fulminant Wilson's disease in children
Małgorzata Markiewicz-Kijewska1, Marek Szymczak, Hor Ismail
1Department of Pediatric Surgery and Organ Transplantation, Childrens Memorial Health Institute, Warsaw, Poland. gonia.mark@wp.pl
Insights
Fulminant Wilson's disease (FWD) in children is often fatal without liver transplantation. However, new therapies like albumin dialysis and chelating treatment show promise for survival and can extend transplant waiting times.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Transplant Surgery
Background:
- Fulminant Wilson's disease (FWD) presents a rare but fatal condition in children, with liver transplantation being the primary life-saving intervention.
- Advancements in medical technologies offer potential to improve the prognosis for pediatric patients diagnosed with FWD.
Purpose of the Study:
- To conduct a retrospective analysis of the clinical course, treatment strategies, and outcomes of pediatric patients with FWD treated at the institution.
- To reevaluate the eligibility for liver transplantation based on pathological findings of explanted livers.
Main Methods:
- Retrospective analysis of 13 pediatric patients (mean age 15.5 years) with FWD treated between 1999-2007.
- Evaluation of clinical data, biochemical parameters, MELD/PELD scores, Wilson score, and King's College criteria for liver transplantation.
- Assessment of treatment types, outcomes, and transplant qualification in relation to explanted liver pathology.
Main Results:
- Initial FWD symptoms included weakness, abdominal pain, and jaundice developing within 5-60 days (mean 20 days).
- Eleven patients experienced neurological symptoms and coma preceding transplantation or death; maximal serum bilirubin ranged from 4.5-71.6 mg% (mean 42.24 mg%), INR 2.9-10.0 (mean 5.4).
- MELD/PELD scores ranged from 21-58 (mean 38), with 10 patients meeting King's College criteria; Wilson's index averaged 13 points. Urgent liver transplantation (LTx) was performed in 11 children, one recovered with albumin dialysis and chelating treatment, and one died due to late referral. Survivors (n=12) show good liver function with a mean follow-up of 2.57 years.
Conclusions:
- FWD is nearly universally fatal in pediatric patients without timely liver transplantation.
- Early implementation of albumin dialysis (MARS) and chelating therapy facilitated survival in one patient and may prolong waiting times for LTx.
- The Wilson's index demonstrated a slightly superior predictive value for LTx necessity compared to the King's College criteria in this cohort.
Background:
Fulminant Wilson's disease (FWD) is rare and fatal condition in children unless liver transplantation is performed, however introduction of new technologies could change this poor prognosis. The aim of our study was retrospective analysis of clinical course, treatment and outcome of children with FWD treated in our institution.
Material/Methods:
Between 1999-2007 we've treated in our hospital 13 patients with mean age of 15.5 yrs with FWD. We performed retrospective analysis of clinical course, biochemical parameters, MELD/PELD score, Wilson score and Kings'-College criteria for LTx in acute liver failure in all these patients. Type of treatment and final outcome were analyzed, as well as qualification for transplantation was reevaluated in each case in accordance to pathological examination of explanted during transplantation livers.
Results:
The initial symptoms of FWD were typically weakness, abdominal pain and developing later after 5-60 days (mean 20 days), jaundice. Eleven patients developed neurological symptoms with coma lasting for 2-11 days before transplantation or death. Maximal serum bilirubin concentration ranged between 4.5-71.6 mg% (mean 42.24 mg%), INR 2.9-10.0 (mean 5.4). MELD/PELD score was between 21-58 (mean 38), 10 patients fulfilled general King's-College criteria for transplantation in acute liver failure. Wilson's index ranged between 11 and 17 points (mean 13 points). In 11 children urgent liver transplantation (LTx) was performed, 1 child recovered on albumin dialysis and chelating treatment, 1 child died shortly after very late referral to our center. Actual follow-up of living patients is 0.36-7.43 years (mean 2.57 yrs), all are doing well with good liver function.
Conclusions:
FWD lead to death in almost all pediatric patients if LTx can not be performed, however early introduction of albumin dialysis (MARS) and chelating therapy allowed for survival without transplantation in single patient. It seems also that MARS therapy allows for at least prolongation of waiting time for LTx. Wilson's was slightly better predictor of need for LTx in our patients than classical King's-College criteria.
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