Name: Cardo Dalisay Date: 09/05/2023

Section: BSA 3A

Key Points: Epidemiology

 The cause is unknown.

 The estimated incidence in North America is ≈25 cases per 100

000 children <5 years of age per year.

 The highest relative risk is in Asian children, especially of

Japanese ancestry.

 The ratio of males to females is ≈1.5:1.

 KD affects predominantly, but not exclusively, young children.

 It is most common in winter and early spring in North America.

 Predisposing factors have been reported inconsistently.

 Nonspecific symptoms are common in the 10 days before

diagnosis.

 In Japan, the recurrence rate is ≈3%, and the relative risk in

siblings is 10-fold higher.

 The case fatality rate is <0.1% in Japan.

 Coronary artery aneurysms from KD account for 5% of acute

coronary syndromes (ACS) in adults <40 years of age.

Genetics
Evidence for a genetic component to KD susceptibility includes the
observation of an increased incidence among Japanese children and
among children of Japanese descent residing outside of Japan, the
increased incidence of a history of KD among the parents of a KD
patient, and the increased incidence among siblings and extended
family members of an index case.18,35,58–60 Family linkage studies and
genome-wide association studies with subsequent validation studies
have implicated single-nucleotide polymorphisms in 6 genes or gene
regions: FcγR2a, caspase 3 (CASP3), human leukocyte antigen class II,
B-cell lymphoid kinase (BLK), inositol 1,4,5-trisphosphate kinase-C
(ITPKC), and CD40 (Table 2). Variants in genes in the transforming
growth factor (TGF)-β signaling pathway (TGFβ2, TGFβR2, and SMAD3)
were associated with increased risk of aneurysm formation in patients
of European descent by use of a case-control study design and the
transmission disequilibrium test, which assesses transmission of
candidate risk alleles from heterozygous parents to their affected
offspring.