Corazon Locsin Montelibano Memorial Hospital

Department of Pediatrics

A Clinical Case Presentation on

Kawasaki
Disease
JI HENARES | JI FAURA | PGI TORCITA | PGI
DUENAS
 CASE OVERVIEW

01 02 03
History of Approach to
The Patient
Present Illness Diagnosis

04 05
Course in the Wards Case Discussion
 01
The Patient
General Data
Age: 2 years & 6 months old
Gender: Male
Date of Birth: October 9, 2021

Address: Pahanocoy, Bacolod City,

Negros Occidental
Place of Birth: Bacolod, Philippines

Religion Roman Catholic

Date of Admission: April 16, 2024

Informant: Mother
M.E. Reliability: 90%
Chief Complaint
Fever
 02
History and
Physical Examination
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 History of Present Illness
(+) fever (Tmax 40.5)
(+) given Paracetamol, no
relief
5 days PTA
(+) persistence of fever (Tmax
39.5)
(+) upward rolling of
extremities and stiffening of
extremities ~1 minute (1st
episode)
no consult done
3 days PTA
(+) still with fever (39.1),
not relieved by paracetamol
(+) nonpruritic
maculopapular rash on the
abdomen
2 days PTA
(+) persistence of fever
(+) progression of rash to
the extremities
(+) conjunctival erythema
consult at LHC and was
advised to consult at our
institution
ODA
 REVIEW OF SYSTEMS

(+) decreased level of activity

GENERAL (-) weight loss
(-) chills

(-) pallor
(-) hematoma or bruises
(-) jaundice
SKIN (+) perianal desquamation
(+) edema hands and feet
(+) maculopapular rash trunk, back and
extremities

(-) bilateral conjunctivitis nonexudative

(+) sunken eyeballs
HEENT (-) naso-aural discharges
(+) dried, cracking, erythematous lips
(-) epistaxis

(-) difficulty of breathing

RESPIRATORY (-) nasal catarrh
 REVIEW OF SYSTEMS

(-) palpitations
CARDIOVASCULAR (-) cyanotic episodes
(-) fainting spells

(+) loss of appetite

(-) stomach pain
GASTROINTESTINAL (-) diarrhea
(-) hyperactive bowel sounds

(-) changes in urination

GENITOURINARY (-) hematuria

(-)changes in sensorium
(-) convulsions
NERVOUS/BEHAVIORAL (-) tremors
(-) loss of consciousness

(-) limited range of motion

MUSCULOSKELETAL (-) joint pain
 Past Medical Family
History History
No previous hospitalization (+) HPN - maternal
No previous surgeries (-) DM, TB
No known food/drug allergies
 Personal and
Social History
Lives with both parents and 2 other
siblings
Mother - 34 years old unemployed
Father - 23 years old tricycle driver
Environmental
History
(+) Exposure to cigarette smoke (father)
Distilled water for consumption
Garbage disposal every 3 days
Lives near a stagnant waterway
 Diet Immunization
Formula milk (Nido) BCG 1
Soft food Hepatitis B 3
DPT 3
OPV 3
PCV 3
MMR 2
 Milestones

Motor Adaptive
Able to run well, climbs furniture Able to draw circles and scribble
and open doors on paper

Language
Able to name objects and body
parts, name pictures,
communicates wants/needs with
one word
Social
Prefers to eat with a spoon
unassisted, helps to undress, listens
to others talk and plays with other
children
 PHYSICAL
EXAMINATIO
N
 Physical Examination

GENERAL VITAL SIGNS ANTHROPOMETRI

SURVEY CS
Temp: 39.4 C Weight: 9.6 kg
Awake, irritable but CR: 122 bpm Length: 80 cm
consolable, not in RR: 43 cpm BSA: 0.46
cardiopulmonary BP: 90/60 mmhg MUAC: 14cm
distress, ambulatory O2 sat: 98%
ANTHROPOMETRICS
 REGIONAL EXAMINATION
HEEN Pulmonary
T● Normocephalic ● Maculopapular rash
● No facial edema ● Symmetrical chest
● Conjunctival expansion
injection with limbal ● No lesions
sparing ● No retractions
● Nonsunken eyeballs, ● No masses palpated
Moist mucosa ● Bronchovesicular
● Erythematous and breath sounds
cracked lips
● Unilateral CLAD 2
cm
 REGIONAL EXAMINATION
GASTROINTESTI
CARDIOVASC NAL
● Maculopapular
ULAR
● Adynamic rash
precordium ● Flat abdomen
● PMI 4th ICS, ● No lesions
MCL ● Normoactive
● Distinct heart bowel sounds
sounds ● Nondistended
● Tachycardic nontender
● Regular cardiac abdomen
rhythm
● No murmurs
 REGIONAL EXAMINATION

Extremities Genitalia
● Maculopapular
rash ● Perianal
● Skin is warm to Desquamations
touch ● Shaft at midline
● Edema of ● Meatus at midline
Maculopapular ● Fully descended
rashhands and Testis
feet
● Full pulses
● CRT <2s
 SALIENT POINTS
HISTORY PE
● Remittent Fever not relieved with Bilateral conjunctivitis with limbal
paracetamol sparing
● Nonpruritic maculopapular rash and Erythema and cracking of lips
conjunctival erythema Maculopapular rash
Edema of hands and feet
Unilateral cervical LAD 2cm
03
Approach to
Diagnosis
 Algorithm
2 y.o/M

CC: Fever

Infectious Non-infectious

Acquired Inflammatory
Bacterial Viral Autoimmune Disease
Syndrome
Scarlet Fever Dengue Systemic Idiopathic Juvenile Arthritis
Kawasaki Disease
Rule in Rule out Rule in Rule out Rule in Rule out (+) Fever
(+) Fever (+) bilateral (+) Fever (+) perineal (+) bilateral conjunctival injection
Age conjunctival (+) desquamation (+) Fever (+) unilateral (+) non-tender unilateral
injection maculopapular (+) unilateral Age cervical lymphadenopathy
(+) non-tender rash cervical (+) redness of lymphadenopathy (+) maculopapular rash
unilateral (+) bilateral lymphadenopathy the white of (+) edema of (+) edema of hands and feet
lymphadenopathy conjunctival (+) edema of the eyes hands and feet (+) perineal desquamation
(+) perineal injection hands and feet (+) rash (+) Erythema and
desquamation (+) Cracked lips cracked lips
(+) edema of (-) joint pain and
hands and feet swelling
(+) maculopapular
rash
(-) strawberry
tongue
 04
Course in the
Wards
HOSPITAL DAY 0 4/16/24 PLANS LABS

Day 5 of fever 4/16/24

Wdx: Complete Kawasaki Disease
CBC
Hct 0.28, Hgb 91 , WBC 11.4 , plt 189 , S
79, L 16, M 4, E 1
Normocytic hypochromic anemia

UA
PS, HAZY, 6.0, 1.010, pus 3-6, rbc 2-4,
protein 2+, bilirubin 2+ ketones 5 mg/dl,
few squamous, few bacteria - Sterile
pyuria
>Admit at MISC 1
> TPR q4h and record CRP 695.4 mg/dL

> Diet for age and as tolerated ESR 121 mm/hr

> IVF: D5IMB to run at 48cc/hr (MR+10%)
> Labs:
CBC plt, ESR
CXR AP, CRP
UA 2d Echocardiogram
> Meds:
1. Aspirin 80mg/tab - 2 ½ tab - may mix with milk/water to
give every 6 hrs PO
S/O: 2. Omeprazole 10mg IVTT ODAC
(+) rashes on trunk and extremities > S/O:
(+) fever - Please secure 4 vials of IVIG
 05
Case Discussion
KAWASAKI
DISEASE
● Formerly known as Mucocutaneous lymph
node syndrome and Infantile polyarteritis
nodosa
● Acute febrile illness of childhood
● Vasculitis with a predilection for the
coronary arteries
● Leading cause of acquired heart disease in
children
 ETIOPATHOGENESIS
Etiology Pathology
● Unknown cause ● Predominantly affects the medium size
● Likely infectious in origin arteries
● Coronary arteries - most commonly
involved
Epidemiology ● Popliteal, Brachial, Axillary arteries can
also develop dilation
● Young age (80% of patients are <5
years old)
● Male gender (1.5:1)
● Asian, Pacific Islander race &
Hispanic ethnicity
 CLINICAL MANIFESTATIONS

5 Principal Clinical Criteria

fever for at least 1. Bilateral nonexudative, conjunctival injection with limbal

5 days (≥38.3°C), sparing
2. Erythema of the oral and pharyngeal mucosa with strawberry
remitting, &
tongue and red, cracked lips
unresponsive to 3. Edema and erythema of the hands and feet
antipyretics 4. Rash of various forms
5. Non-suppurative cervical lymphadenopathy, usually unilateral
with node size >1.5cm
 CLINICAL PHASES OF KD

Acute Febrile Phase Subacute Phase Convalescent Phase

1 2
● Fever and other acute signs of ● Fever and other acutes signs have
illness abated
● Last 1-2 weeks ● Desquamation, thrombocytosis,
development of CAA
● Last about 3 weeks
3
● All clinical signs have
disappeared
● Until ESR returns to normal
● 6-8 weeks after onset
 DIAGNOSTICS
Leukocytosis with neutrophilia and immature Hypoalbuminemia
forms Hyponatremia
Elevated erythrocyte sedimentation rate Thrombocytosis after week 1
Elevated C-reactive protein Sterile pyuria
Anemia Elevated serum glutamyl transpeptidase
Abnormal plasma lipids Pleocytosis of cerebrospinal fluid
Leukocytosis in synovial fluid
 2D
Echocardiogram
● Most commonly affected : LAD,
RCA; Circumflex L MCA may also be
involved
● Done at time of diagnosis, 2 weeks
and 6-8 weeks

https://pedecho.org/library/chd/kawasaki-disease
 2D
Echocardiogram
● Z score LAD >2.5mm
● Z score RCA >2.5mm
● Coronary artery diameter
○ > 3 mm in children <5 years
○ > 4 mm in children ≥ to 5 years
● Lumen diameter ≥1.5 times adjacent
segment
● Coronary lumen is clearly irregular

https://pedecho.org/library/chd/kawasaki-disease
KOBAYASHI RISK SCORING
ATYPICAL VS INCOMPLETE KD
Atypical Incomplete
5 or more days of fever unexpected fever for 5 or more days

2-3 of the principal clinical features 3 or less of the clinical criteria

compatible laboratory findings laboratory evidence of systemic inflammation

perineal erythema and desquamation

more common in young infants

higher risk to develop coronary artery lesions


Acute Phase
● Intravenous
immunoglobulin 2g/kg
over 10-13 hrs
● Aspirin 80-100
mg/kg/day divided every
6 hrs orally until patient
is afebrile for at least 48
hrs
TREATMENT
Long Term Therapy for Acute Coronary
Convalescent Phase
Coronary Abnormalities Thrombosis
● Aspirin 3-5 mg/kg ● Aspirin 3-5 mg/kg ● Prompt fibrinolytic
once daily orally once daily orally therapy with tissue
continued until the ● Clopidogrel 1 plasminogen activator
patient has no mg/kg/day (max: or other thrombolytic
evidence of coronary 75mg/day agent under
changes by 6-8 weeks supervision of
after illness onset pediatric cardiologist
 IVIG RESISTANT KD
● Persistent or recrudescent fever 36 hr after completion of the initial IVIG
infusion
● Increased risk for CAA
○ Another dose of IVIG at 2 g/kg is administered to patients with
IVIG resistance
○ Re-treatment of patients with refractory Kawasaki disease - another
IVIG 2g/kg
○ If there is a poor response to the 2nd IVIG, some patients have
responded to IV methylprednisolone 30 mg/kg/day for 3 days.
○ Patients with a small solitary aneurysm should continue aspirin
indefinitely.
 COMPLICATIONS
Small Solitary Aneurysm
continue aspirin indefinitely

Large or Numerous
require addition of other antiplatelet agents or
Aneurysm
anticoagulants

Acute Thrombosis
thrombolytic therapy

Patient undergoing long-term aspirin therapy should receive annual influenza vaccination to
reduce the risk of Reye syndrome
 PROGNOSIS
● Vast majority return to normal health, as timely treatment reduces risk of CAA
● Acute KD recurs in 1-3% cases
● Patients with CAA prognosis depends on severity
○ 50% of coronary artery aneurysm regress to normal lumen diameter by 1-2 yrs
after illness
○ Published fatality rates <1.0%
 Reference:

Thank you, Doctors!