J Korean Med Sci.  2012 Jan;27(1):101-103. 10.3346/jkms.2012.27.1.101.

Severe Jaundice in Two Children with Kawasaki Disease: A Possible Association with Gilbert Syndrome

• Karpathios T
• Moustaki M
• Yiallouros P
• Sharifi F
• Attilakos
• Papadopoulou A
• Fretzayas

• KMID: 1792988
• DOI: http://doi.org/10.3346/jkms.2012.27.1.101

Abstract

Kawasaki disease is a systemic vasculitis, mainly encountered in children. It may affect any organ. Acute cholestasis and severe obstructive jaundice is an atypical manifestation of the disease. We herein present two children with Kawasaki disease and severe direct hypebilibirunemia who also were homozygous and heterozygous respectively for the (TA)7 promoter polymorphism of Gilbert syndrome. Intravenous immunoglobulin was administered to both patients at the acute phase of the disease and the fever remitted within 24 hr following the immunoglobulin administration. Furthermore oral aspirin at a dose of 80-100 mg/kg/24 hr was also given. The first child did not develop any coronary ectasia or aneurysm, whereas dilation of the right coronary artery was identified in the second child, one month after the disease onset. We discuss the possible contribution of Gilbert syndrome to the development of jaundice in our patients.

Keyword

Kawasaki Disease; Gilbert Syndrome; Jaundice; Acute Cholestasis

MeSH Terms

Administration, Oral
Aspirin/therapeutic use
Child
Child, Preschool
Echocardiography
Female
Gilbert Disease/*complications/*diagnosis/genetics
Humans
Immunoglobulins, Intravenous/therapeutic use
Jaundice/etiology
Male
Mucocutaneous Lymph Node Syndrome/*complications/*diagnosis/drug therapy
Polymorphism, Single Nucleotide
Promoter Regions, Genetic
Sequence Analysis, DNA

Reference

1. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics. 1974. 54:271–276.

2. Burns JC, Kushner HI, Bastian JF, Shike H, Shimizu C, Matsubara T, Turner CL. Kawasaki disease: a brief history. Pediatrics. 2000. 106:e27.

3. Scheinfeld NS. Pediatric Kawasaki disease. accessed on 26 May 2011. Available at http://emedicine.medscape.com/article/965367-overview.

4. Valentini P, Ausili E, Schiavino A, Angelone DF, Focarelli B, De Rosa G, Ranno O. Acute cholestasis: atypical onset of Kawasaki disease. Dig Liver Dis. 2008. 40:582–584.

5. Fukunishi M, Kikkawa M, Hamana K, Onodera T, Matsuzaki K, Matsumoto Y, Hara J. Prediction of non-responsiveness to intravenous high-dose γ-globulin therapy in patients with Kawasaki disease at onset. J Pediatr. 2000. 137:172–176.

6. Radu P, Atsmon J. Gilbert's syndrome: clinical and pharmacological implications. Isr Med Assoc J. 2001. 3:593–598.

7. Bosma PJ, Chowdhury JR, Bakker C, Gantla S, de Boer A, Oostra B, Lindhout D, Tytgat GN, Jansen PL, Oude Elferink RP, Chowdhury N. The genetic basis of the reduced expression of bilirubin UDP-glucuronosyltransferase 1 in Gilbert's syndrome. N Engl J Med. 1995. 333:1171–1175.

8. Berk PD, Blaschke TF, Waggoner JG. Defective bromosulfophtalein clearance in patients with constitutional hepatic dysfunction (Gilbert's syndrome). Gastroenterology. 1972. 63:472–481.

9. Tsezou A, Tzetis M, Giannatou E, Spanos I, Roma E, Fretzayas A, Kanavakis E, Kitsiou-Tzeli S. Gilbert syndrome as a predisposing factor for cholelithiasis risk in the Greek adult population. Genet Test Mol Biomarkers. 2009. 13:143–146.

10. Attilakos A, Prassouli A, Hadjigeorgiou G, Lagona E, Kitsiou-Tzeli S, Galla A, Stasinopoulou A, Karpathios T. Acute acalculous cholecystitis in children with Epstein-Barr virus infection: a role for Gilbert's syndrome? Int J Infect Dis. 2009. 13:e161–e164.