3. HISTORICAL PERSPECTIVE
• 1967 - TOMISAKU KAWASAKI reported 50 cases and established
clinical criteria for diagnosis
• 1977 - LANDING AND LARSON established that KD and IPAN are
pathologically indistinguishable
• 1988 - AAP recommended high dose IVIG and ASA as therapy of
choice
4. IT IS AN ACUTE FEBRILE
ILLNESS OF EARLY
CHILDHOOD MANIFESTING
AS VASULITIS OF MEDIUM
SIZED ARTERIES
5. MC ACQUIRED HEART DISEASE IN
CHILDREN IN INDIA
• HIGHEST INCIDENCE IN
MC ACQUIRED HEART DISEASE IN
CHILDREN IN DEVELOPED COUNTRY
• MC CHILDHOOD VASCULITIS
• 2ND MC CHILDHOOD VASCULITIS
6. EPIDEMIOLOGY
• MALE > FEMALE
• Winter & early spring
• 6mth to 5 yr AGE group
• INCIDENCE -JAPAN 240-260/lakh
in children under 5 age - NORTH AMERICA 25/lakh
-INDIA 10-100/lakh
• ASIAN POPULATION
• RECURRENCE rate around 3%
• CASE FATALITY RATE <0.1% in japan
9. GENETICS
1. Polymorphism in ITPKC gene ( T cell regulator)
2.Polymorphism in FCGR2A gene (receptor for IgG)
3. SNPs in HLA class II region ( HLA DQB2 , HLA DOB )
4. CASPASE 3 gene (CASP 3)
5. B CELL LYMPHOID KINASE ( BLK)
6. CD 40
27. CNS GI TRACT MUSCULO
SKELETAL
GENITO
URINARY
Extreme irritability Diarrhea,vomiting arthritis urethritis
Aseptic meningitis pancreatitis arthralgia hydrocele
Facial nerve palsy Abdominal pain Desquamating rash
SN hearing loss jaundice Sterile pyuria
RESPIRATO
RY
Pulmonary nodule
Interstitial infiltrate
28.
29. DIAGNOSIS
CLASSIC KD
FEVER FOR ATLEAST 5 DAYS
PLUS
4 OUT OF 5 PRINCIPAL
CHARACTERSICS OF ILLNESS
ATYPICAL OR INCOMPLETE
KD
FEVER FOR 5 DAYS+ <4 OUT OF 5
DIAGNOSTIC CRITERIAS
WITH LAB FINDING + ECHO
30. LAB FINDINGS
1. TLC –neutrophilia and immature forms
2. ANEMIA
3. ESR, CRP
4. PLATELET- normal in 1st wk then rapidly
5. PLEOCYTOSIS IN CSF
6. LEUKOCYTOSIS IN SYNOVIAL FLUID
7. STERILE PYURIA
8. TRANSAMINASES ,GGT
9. HYPOALBUMINEMIA
10. HYPONATREMIA
31.
32. POSITIVE
ECHO
Z SCORE of LAD/RCA >=2.5
>=3 of following
-LVF
-MR
-PE
-Z SCORE 2-2.5 in LAD/RCA
CORONARY ARTERY
ANEURYSM
41. ? ROLE OF STEROID
SINGLE PULSE DOSE OF IV METHYLPREDNISOLONE
(30mg/kg)
• PREDNISOLONE
(2mg/kg)
42.
43. IVIG RESISTANT KD
Persistent or recrudescent FEVER
(36hr after completion of IVIG therapy)
Presented with cardiogenic shock
IN 15% of patients
increased risk of CAA
44. KOBAYASHI SCORE
>=5 severe
ALT->=100iu/l 2 point
Duration of disease 2 point
<=4 days
Sodium <=133 2 point
Neutrophil >=80% 2 point
CRP >=100mg/l 1 point
Age <=12 mths 1 point
Platelet <=3 lakh 1 point
EGAMI SCORE
>=3 severe
ALT >=80 2 point
Duration of disease 1 point
<=4days
CRP >=80mg/l 1 point
Age < 6 mth 1 point
Platelet <=3lakh 1 point
SANO SCORE
>=2 severe
ALT >=200 1 point
CRP >=70 mg/l 1 point
Bilirubin>=0.9 mg/dl 1 point
45. TREATMENT OF IVIG RESISTANT KD
2ND DOSE OF IVIG
IVIG + PREDNISOLONE (2mg/kg/day IV Divided
doses 8Hrly)
INFLIXIMAB (5mg/kg IV over 2Hr )
46. LONG TERM THERAPY
• Only ASPIRIN 3-5mg/kg/day OD orally
with
CAA
• ASPIRIN +
• LMWH / WARFARIN
Expanding
aneurysm & Z
score >= 10
• TRIPLE therapy
• ASPRIN + LMWH/WARFARIN + CLOPIDOGREL
H/O coronary
artery
THROMBOSIS
47. NO CORONARY CHANGES IN ANY STAGE OF ILLNESS
TRANSIENT CORONARY ARTERY ECTASIA
• SMALL /MEDIUM SOLITARY CORONARY ANEURYSM
GIANT/LARGE/MULTIPLE SMALL ANEURYSM
• CORONARY ARTERY OBSTRUCTION
48.
49.
50. PROGNOSIS
• Prognosis depends on severity of coronary artery disease.
• Recovery is complete and without long term effect for patients who
don’t develop CAA.
• 50% of CAA resolve by 1-2 yr of illness.
• Recurrent illness occurs in 1-3% of cases.
51. TAKE HOME MESSAGE
• Strong clinical suspicion is required for diagnosis and clinical outcome.
• Normal ECHO in first week of illness does not rule out diagnosis of KD.
• Lab investigations &/ ECHO can help in diagnosis of atypical
/incomplete KD.
• IVIG to be started without delay within 10 days of onset of illness to
prevent complications related to CAA.
• Incase of IVIG resistant ,we can repeat IVIG therapy .
• Steroid therapy should not be considered as routine primary
therapy,instead reserved for high risk patient with acute KD.
• Follow up & cardiologist consultation is must in patient who develop
CAA.
