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Clinician-to-Clinician Update
To schedule a pediatric liver care or transplant consultation, referral or appointment:
For living-donor liver transplant consultation or referral:

Gastroenterology, Hepatology and Nutrition or Transplant, Solid Organ (Liver, Kidney and Intestine)
M Health Fairview Discovery Pediatric Specialty Clinic
2512 Building
2512 S 7th St., Floor 3
Minneapolis, MN 55454 

Solid Organ Transplant
M Health Fairview University of Minnesota Medical Center - East Bank
500 Harvard St. SE
Minneapolis, MN 55455 

For our pediatric liver failure care teams, go here.

Rapid Assessment and Diagnosis Enable Successful Emergent Liver Transplant

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Acute liver failure in the pediatric population is rare, and rarer still is Wilson’s disease, a genetic disorder that leads to liver dysfunction and ultimately liver failure. For pediatric patients with acute liver failure, access to rapid multidisciplinary evaluation, accurate diagnosis, and emergency transplantation are vital, and they were the driving factors that enabled the patient in this case to achieve the best possible outcome.

Patient

A 16-year-old female presented with 3-day history of nausea, vomiting, and diarrhea. She was noted to have acute renal failure along with elevated levels of the liver enzymes aspartate aminotransferase (AST) and alanine aminotransferase (ALT) but normal alkaline phosphatase. Her blood urea nitrogen-to-serum creatinine (BUN-to-Cr) ratio was less than 20, and she had possible signs of blood in her stool. Tests demonstrated hemolytic anemia. She quickly developed encephalopathy and became hypotensive.

Management

After taking steps to manage the acute renal failure, the pediatric critical care team provided the following differential diagnoses. Chronic kidney disease not previously discovered was possible, but normal renal size made this unlikely. Pre-renal acute kidney injury was not likely due to absence of elevated BUN-to-Cr ratio. Given the possibility of the patient’s exposure to animals, hemolytic anemia, possible blood in her stool, and the acute renal failure, hemolyticuremic syndrome (HUS) was possible. The patient’s elevated transaminases, splenomegaly, and hyperbilirubinemia, however, would not be explained by HUS. Physicians concluded that other primary gastrointestinal causes had to be considered, and a pediatric hepatology consult was requested along with a referral to ophthalmology.

Hepatology tests revealed low factor levels: Factor VII was 15, and Factor V was 24. They also indicated Coombs negative hemolytic anemia with mildly elevated transaminases and low alkaline phosphatase. The ophthalmologic examination identified Kayser-Fleischer rings encircling the iris, a sign indicating Wilson’s disease. The tests confirmed a diagnosis of acute liver failure due to Wilson’s disease.

Emergent liver transplantation is the definitive treatment for the condition, and physicians recommended this course of treatment to the patient and family at 1:00 p.m. on the day of diagnosis confirmation. By 5:00 p.m. that day, the patient was given the top priority status of 1A on the liver transplant waiting list.

A donor was identified, and the patient was readied for transplant at M Health Fairview University of Minnesota Masonic Children’s Hospital. After the 10-hour transplant procedure, the donor liver was functioning well. Subsequent surgical procedures addressed issues that arose from liver size mismatch and compartment syndrome in the patient’s leg. Two-and-a-half months post-transplant, the patient was off dialysis and back at school and work.

Discussion

In Wilson’s disease, excess copper is not filtered from the patient’s system, causing it to build up in the liver, brain, eyes, and other organs. Without treatment, high copper levels can cause life-threatening organ damage.

Wilson’s disease is rare but should be suspected when liver failure is associated with mild transaminitis, low alkaline phosphatase, and Coombs negative hemolytic anemia. Diagnosis is confirmed via urinary copper estimates and by the presence of Kayser-Fleischer rings. In this case, multidisciplinary assessment, team-based care, and access to specialty expertise in both transplant and the conditions that can indicate it together enabled a positive patient outcome.

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