Colorful Eyes: Kawasaki Disease Presenting with Jaundice

1Children's Hospital Los Angeles, Los Angeles, CA

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 327

Case Presentation:

A 3‐year‐old healthy Hispanic female presented with a 7‐day history of persistent fevers and 3 days of jaundice, abdominal pain, and emesis. She was noted to be irritable and had just developed a pruritic rash on her trunk and extremities. She was taking amoxicillin for pharyngitis and acetaminophen for fever, had been vaccinated against hepatitis A and B, and had an unremarkable travel history. On physical exam, she was febrile and ill‐appearing. Skin was jaundiced, with a diffuse macular rash and a few areas of desquamation on her abdomen. Head, eyes, ears, nose, and throat exam revealed icteric sclera, mild nonpurulent conjunctivitis, edematous tongue papillae, and no lymphadenopathy. Abdominal exam was significant for right upper quadrant (RUQ) tenderness and hepatomegaly. Laboratory investigation showed hepatic dysfunction, with increased conjugated bilirubin (5.2, total 7.8 mg/dL), ALT/AST (174/203 U/L, respectively), alkaline phosphatase (709 U/L), and GGT (157 U/L). PT was prolonged, at 14.5 seconds, and albumin low, at 3.1 g/dL. C‐reactive protein (CRP) was 2.9 mg/dL; a complete blood count showed mild leukocytosis and normal but rising platelets.

Given the fever, rash, mucosal changes, and conjunctivitis‐subtle and because of the presence of icterus in addition to the abnormal CRP and liver function tests, she was treated for incomplete Kawasaki disease (KD) with intravenous im‐munoglobulin (IVIG) 2 g/kg and aspirin. She also received vitamin K for her mild coagulopathy. Ultrasound of the RUQ showed mild liver enlargement and a normal biliary tree without hydrops. Echocardiogram was normal. She immediately defervesced and showed marked improvement with IVIG, but workup was initiated to look for other etiologies for her liver dysfunction. Autoimmune workup revealed negative ANA and antibodies to LKM, SLA, and SMA. Infectious causes were excluded, including hepatitis A, B, C, and E, Epstein–Barr virus, and cytomegalovirus. Ceruloplasmin and ferritin were unremarkable. Alpha‐1‐antitrypsin phenotype was MM. Acetaminophen level was negligible. By hospital day 4, her bilirubin had decreased to 3.5 mg/ dL, and she was discharged. At follow‐up, echo remained normal, and liver function had normalized.

Discussion:

Although hepatobiliary involvement is often seen in KD, it is considered a mild clinical manifestation, usually associated with gallbladder hydrops or mild transaminitis. A recent review of 239 KD patients showed 45% to have at least 1 liver panel abnormality; however, only 2 patients had an elevated bilirubin, and 11 had an ALT or AST of at least 5 times the normal values. Although jaundice as a presenting feature of KD has been described in a few case reports, none reported mild liver failure with significant hepatocellular damage and cholestatic jaundice in the absence of gallbladder findings.

Conclusions:

Cases of incomplete KD are being increasingly recognized. They tend to have delayed diagnosis and treatment and are associated with more complications. This case highlights a rare but significant hepatobiliary manifestation of KD and emphasizes the importance of a thorough exam beyond the presenting complaint.

Disclosures:

V. Lee ‐ none

To cite this abstract:

Lee V. Colorful Eyes: Kawasaki Disease Presenting with Jaundice. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 327. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/colorful-eyes-kawasaki-disease-presenting-with-jaundice/. Accessed September 18, 2019.

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