KAWASAKI DISEASEDR MAHTAB

MBBS ,DCH,DNBGMSH-16 CHD

ALTERNATIVE NAMES

KAWASAKI SYNDROMELYMPH NODE SYNDROMEMUCO CUTANEOUS LYMPH NODE

SYNDROMEINFANTILE POLY ARTERITIS NODUSA

HISTORY1960 TOMISAKU KAWASAKI (JAPANESE

PEDIATRICIAN ) DESCRIBE A SERIES OF 50 CHIDREN MANIFESTING THE CLINICAL FEATURE OF SYNDROME IN JAPAN

1974 FIRST ENGLISH LANUAGE REPORT OF KAWASAKI SYNDROME BY KAWASAKI

1977 FIRST REPORTED CASE IN INDIA1988 AMERICAN ACADEMY OF PEDIATRIC

ENDORSE IVIG AND ASA AS RECOMMENDED THERAPY OF KAWASAKI DISAESE

DEFINITIONIDIOPATHIC MULTISYSTEM DISEASE

CHARECTERIZE BY VASCULITIS OF SMALL AND MEDIUM BLOOD VESSELS INCLUDING CORONARY ARTERIES

A SELF LIMITED MULTISYSTEM VASCULITIS OF UNKNOWN ETIOLOGY THAT PREDOMINANTLY AFFECT CHILDREN YOUNGER THAN 5 YEARS - JAMES BURNS

IT IS NOW THE MOST COMMON CAUSE OF ACQUIRED HEART DISEASE IN CHILDREN IN USA AND JAPAN(MOST DEVELOPED COUNTRY)

EPIDEMIOLOGYAGE - 75% PATIENT ARE YOUNGER THAN

<5YEARS -MEAN AGE IS 3 YEARM>F 1.6:1 CHILDREN OF ASIAN AND PACIFIC

ISLANDER DESCENT HAS HIGHEST HOSPITALISATION RATES - IT IS 30.3/100000 TO 17.5/100000 BLACK

JAPAN HAS HIGHEST RECORDED RATE 239.6/100000 CHILDREN 0-4 YEARS OF AGE

PREDICTOR OF POOR OUTCOME YOUNGER AGE MALE GENDER PERSISTENT FEVER POOR RESPONSE TO IVIGLABORATORY ABNORMALITIES(NEUTROPHILIA ,THROMBOCYTOPENIA ,H

YPONATREMIA,ELEVATED CRP,HYPOALBUMINEMIA)

ETIOLOGYREMAIN UNKNOWN ALTHOUGH CLINICAL

AND EPIDEMIOLOGICAL FEATURES STRONGLY SUGGEST INFECTIOUS CAUSE

GENETIC - HIGH INCIDENCE OF KD IN ASIAN CHILDREN HAVING POLYMORPHISM OF ITPKC GENE - A T CELL REGULATOR GENE

AUTOIMMUNE

PATHOLOGYGENERALISED SYSTEMIC VASCULITIS

INVOLVING BLOOD VESSELS THROUGHOUT THE BODY

PREDOMINANTLY AFFECT MEDIUM SIZE ARTERIES LIKE CORONARY ARTERIES MC INVOLVED

OTHER ARTERIES MAY BE POPLITEAL AND BRACHIAL ARTERIES

PHASES OF ETIOPATHOLOGY 1ST PHASE - NEUTROPHILIC NECROTIZING

ARTERITIS OCCURING IN FIRST 2 WEEKS OF ILLNESSS SACCULAR ANEURYSM MAY BE FORMED

2ND PHASE - SUBACUTE OR CHRONIC VASCULITIS DRIVEN BY LYMPHOCYTES PLASMA CELLA ND EOSINOPHILS LASTS WEEKS TO YEARS RESULTS IN FUSIFORM ANEURYSM

VESSELS AFEECTED DEVELOP SMOOTH MUSCLE CELL MYOFIBROBLAST WHICH CAUSE PROGRESSIVE STENOSIS THROMBI MAY FORM IN THE LUMEN AND OBSTRUCTED BLOOD FLOW

CLINICAL FEATURES PROLONGED FEVER -FUO/PUOB/L NON EXUDATIVE CONJUNTIVITISERYTHEMA OF LIPS AND ORAL MUCOSACHANGES IN EXTREMITIES -OEDEMA

PEELINGRASH - NON VESICULARU/L LYMPHADENOPATHY

FEVER IN KDHIGH GRADE >101F UNREMITING AND

UNRESPONSIVE TO ANTIBIOTICSFEVER OF >OR = 5 DAYS GENERALLY

DISTINGUISH KD FROM OTHER SELF LIMITING VIRAL INFECTIONS

DURATION OF FEVER WITHOUT TREATMENT IS GENERALLY 1-2 WEEKS BUT MAY PERSIST 3-4 WEEKS

DEFERVENCE WITHIN 1-2 DAYS OF TREATMENT WITH IVIG

CONJUNCTIVITIS BEGIN SHORTLY AFTER FEVERNON PURULENT CONJUNCTIVAL

INJECTIONBULBAR CONJUNCTIVITIS WITH LIMBIC

SPARINGANTERIOR UVEITIS MAY OCCUR UPTO 80%

OROPHARYNGEAL CHANGESERYTHEMA OF ORAL AND PHYRANGEAL

MUCOSASTRAWBERRY TONGUERED CRACKED LIPSLIP MAY BLEED

CHANGES IN EXTREMITIESERYTHEMA AND OEDEMA OF HANDS AND

FEETPEELING OF HAND AND FEET IN

SUBACUTE PHASE ( 1-2 WEEKS) NOT IN ACUTE PRESENTATION

BEAU LINE IN NAILNAIL IS LOST

POLYMORPHIC RASHRASH OF VARIOUS

FORMS(MACULOPAPULAR,ERYTHEMA MULTIFORME OR SCARLATIFORM

GENERALLY OCCUR WITH ONSET OF FEVER AND FADES WITHIN WEEKS

ERYTHEMA AND DESQUAMATION OF INGUINAL / PERINEAL AREA

DESQUAMATION OF HAND AND FEET IS LATER SIGN

LYMPHADENOPATHY IN KD50-80 % OF CASESNON SUPPURATIVE CERVICAL

LYMPHADENOPATHY USUALLY UNILATERAL NODE SIZE >1.5cm

OTHER CLINICAL FEATURES OF KDGASTROINTESTINAL SYMPTOMS( OCCURS IN 60%

OF PATIENTS - VOMITING ,DIARRHOEA,ABD PAIN)RESPIRATORY SYMPTOMS (35%) RHINORRHEA

AND COUGHIRRITABILITY - ASEPTIC MENINGITIS (25%) CSF INC

LYMPHOCYTES AND NORMAL GLUCOSE/PROTEINARTHRITIS- EARLY IN ILLNESS,SMALL AND

LARGE JOINTSSTERILE PYURIA, URETHRITIS AND DIARRHEAMILD HEPATITIS ,HYDROPS OF GALL BLADDER

INFLAMATION AT SITE OF BCG SCARCROSS REACTIVITY OF T CELLIN KD

PATIENTS BETWEEN SPECIFIC EPITOPE OF MYCOBACTELIAL AND HUMAN HEAT SHOCK PROTEIN.

DIAGNOSTIC CRITERIAFEVER OF >5 DAYS DURATION PLUS 4 OUT

OF 5 CRITERIA-OROPHYRANGEAL CHANGES (90% + OF

CASES)-CHANGES IN PERIPHERAL EXTREMITIES

(90% + OF CASES) CERVICAL LYMPHADENOPATHY (75% + OF

CASES)POLYMORPHOUS RASH (95% + OF CASES) BILATERAL NON PURULENT

CONJUNCTIVITS (90% + OF CASES)

ATYPICAL OR INCOMPLETE KDRECOGNITION IS DIFFICULTMORE FREQUENT IN INFANTPROLONGED FEVER + THEN 4 OF THE 5

CHARACTERLABORATORY + ECHOCARDIOGRAPHY

DATA CAN HELP IN DIAGNOSISINCOMPLETE CASES HAVE HIGHEST

LAIKELYHOOD OF DEVELOPMENT OF CAA

RECURRENT KAWASAKI DISEASE2% IN JAPANESE , <1% IN UK AND NORTH

AMERICA MUST FULFILL DIAGNOSTIC CRITERIA

AGAIN IN FULLINCREASED RATE OF HEART DAMAGE IN

SECOND EPISODE OF KD

D/D OF KDADENOVIRUSENTEROVIRUSMEASLESEPSTEIN BARR VIRUSCYTOMEGALOVIRUS

BACTERIALSTREPTOCOCCUS INFECTION( SCARLET

FEVER ,TOXIC SHOCK SYNDROME,SCALDED SKIN SYNDROME)

MENINGOCOCCEMIABACTERIAL CERVICAL LYMPHADENITISLEPTOSPIROSISROCKY MOUNTAIN SPOTTED FEVER

OTHERSDRUG HYPERSENSTIVITY REACTIONSYSTEMIC IDIOPATHIC JUVENILE

ARTHRITISBECHET DISEASE

LABORATORY AND RADIOLOGICAL FINDINGNO DIAGNOSTIC TEST FOR KD BUT USUALLY HAVE

CHARACTERICTIC LABORATORY FINDINGFULL BLOOD COUNT AND FILMS LEUKOCYTOSIS

WITH PREDOMINENTLY NEUTROPHILIA NORMOCYTIC NORMOCHROMIC ANEMIA PLATELETS COUNTS IS NORMAL IN FIRST WEEK

BUT MAY RAPIDLY INCREASED IN 2ND 3RD WEEK ELEVATED CRP AND ESR STERILE PYURIA CSF PLEOCYTOSIS MAY BE PRESENT MILD ELEVATION OF HEPATIC

TRANSAMINASES,HYPERBILIRUBINEMIA

TWO DIMENTIONAL ECHOCARDIOGRAPHY IS MOST USEFUL TEST TO MONITOR FOR DEVELOPMENT OF CAA

ANEURYSM MAY BE SMALL <5MM,MEDIUM 5-8MM,GIANTS> 8MM INTERNAL DIAMETER

ECHOCARDIOGRAPHY SHOULD BE PERFORMED AT DIAGNOSIS AND AGAIN AFTER 2-3 WK OF ILLNESS ,REPEAT STUDY SHOULD BE PERFORMED

6-8 WK OF ILLNESS IF NO ABNORMALITIES LIPID PROFILE AND ECHOCARDIOGRAPHY 1YR LATER THEN EVERY 5 YR

IF RESULTS OF EITHER OF INITIAL STUDIES ARE ABNORMAL MORE FREQUENT ECHOCARDIOGRAPHY OR OTHER STUDIES MAY BE NECESSARY

TREATMENTACUTE STAGE IVIG 2G/KG OVER 10-12 HRSASPIRIN 80-100MG /KG/DAY DIVIDED EVERY 6HRLY UNTIL PT IS

AFEBRILE ATLEAST FOR 48HRCONVALESCENT STAGEASPIRIN 3-5 MG /KG ONCE DAILY ORALLY UNTIL 6-8 WEEK AFTER

ILLNESS( ANTI INFLAMMETRY TO ANTI THROMBOTIC DOSE)LONG TERM THERAPY FOR CAAASPIRIN 3-5MG/KG ONCE DAILY FR INDEFINITELYCLOPODOGREL 1MG/KG/DAYWARFARIN AND LOW MOLECULAR WT HEPARIN FR THOSE PT AT

HIGHER RISK OF THROMBOSISCAA SHOULD BE T/T BY PEDIATRIC CARDIOLOGIST BY SPECIFIC

INTERVENTION LIKE POSITRON EMISSON TOMOGRAPHY ,CALCIUM CHANNEL BLOCKER,CORONARY ANGIOPLASTY

COMPLICATIONIRRITABILITY AND ASEPTIC MENINGITISGALL BLADDER HYDROPSHEPATITISOTITIS MEDIAPANCREATITISMYOSITISPERICARDITIS AND MYOCARDITISANEURYSM FORMATION CAN LED TO PERIPHERAL

GANGRENE ,CEREBRAL INFARCTION,CARDIAC ARTERY ANEURYSM (THIS LED TO THROMBOSIS ,MYOCARDIAL INFARCTION,DYS ARRYTHMIA

APP 20-25% OF UNTREATEDCHILDREN DEVELOP CAA

PROGNOSISMAJORITY OF PT WITH KD RETURN TO

NORMAL HEALTHTIMELY T/T REDUCE RISK OF CAA <5%ACUTE KD RECUR IN1-3%OF CASESFATALITY IS <1%OVER ALL 50% OF CAA REGRESS TO

NORMAL LUMAN DIAMETER BY 1-2 YRS

THANKS YOU