PEDIATRICS CASE PRESENTATION

KAWASAKI DISEASE

NALLATHAMBI, AISWARYA BHARATHI

Objectives

To establish the diagnosis of Kawasaki disease through Clinical History and P.E

To review the course in the ward with regards to the management of the disease

To discuss the pathophysiology, diagnostic criteria and treatment of Kawasaki

disease

GENERAL DATA

Name : U, M

Sex : Female

Age : 6y 2m 3d

Nationality : Filipino

Address : Davao city

Religion : Catholic

Informant : Mother and Father

Reliability : Good reliability

CHIEF COMPLAINT(S)

Fever with conjunctivitis and rashes

HISTORY OF PRESENT ILLNESS

6 days PTA, patient had onset of intermittent fever with Tmax of 39 deg Celsius.
Consult done with a doctor. Given Coamoxiclav 5 ml q 12 h for 3 days and
Paracetamol(Tempra) 5 ml q 4h given. Fever subsided temporarily.

3 days PTA, patient developed diffuse erythema of oral mucosa and tongue became
erythematous with prominent papillae.

No management done. No consult done.

1 day PTA, patient had non exudative conjunctivitis and diffuse maculopapular
rashes on trunk, chest and extremities.
Persistence of symptoms the day of admission prompted consult in the OPD and
subsequent admission

PAST MEDICAL HISTORY

No previous hospitalisations before. Did not undergo any surgery(maj/min)

Maintenance medication None.

Allergies No allergy to food and medication.

FAMILY HISTORY

(+) Diabetes Mellitus Paternal

(+) Hypertension Maternal

(-) Cancer

(-) Heart Disease (Con/Acq)

(-) Asthma

No family history of Autism or other developmental disorders.

BIRTH/PREGNANCY HISTORY

Prenatal history

No maternal illness during pregnancy

No intake of drugs during pregnancy other than Iron and Folic Acid

No history of smoking, alcohol or irradiation exposure

BIRTH/PREGNANCY HISTORY

Natal history

Born to a G2P2A0 mother, born Full Term, 38 weeks, BW2.9 kgs, AGA,
delivered via Normal Spontaneous Vaginal Delivery.

APGAR score unrecalled but with good cry and no cyanosis

No congenital anomalies observed.

No complications

BIRTH/PRGNANCY HISTORY
Neonatal history

Newborn screening revealed no abnormalities.

No admissions for neonatal sepsis or pneumonia.

GROWTH AND DEVELOPMENT HISTORY

Motor Started walking at 1 year. No motor deficiencies noted.

Language No developmental delays noted.

Social In first grade in school. Socially active. No aggressive behavior noted. Shes
a good pupil in academics

IMMUNISATIONS

Completed Primary vaccination at a local health center.

BCG at birth

DPT1 6 wks; DPT2 10 wks; DPT3 14 wks

OPV1 6 wks; OPV2 10 wks; OPV3 14 wks

Hepa B1 at birth; Hepa B2 10 wks; Hepa B3 14 wks

Hib1 6 wks; Hib2 10 wks; Hib 3 14 wks.

Measles 9 mo.

DIET/ NUTRITIONAL HISTORY

6 months exclusively breastfed, and shifted to mix feed(formula and breastmilk)

until 1 year.

Solid food was introduced at 1 year.

Currently on no special diet and everyday diet includes rice, meat and veggies.

Personal/Social History

Good eye contact since childhood.

Active in school.

Development is on par with age.

ENVIRONMENTAL HISTORY

Patient lives in a smoke free environment.

Not an endemic area.

No exposure to Pb/Hg or any hazardous/ occupational chemicals.

Patient drinks purified water.

REVIEW OF SYSTEMS

General : (-) Anorexia (-) Weight Loss (-) Fever.

HEENT : No visual or auditory complaints, No dysphagia or odynophagia. No neck

stiffness.

Gastro-intestinal : (-) Right Lower Quadrant Pain, (-) Hypogastric Pain (-) Diarrhea.

Pulmonary : No Dyspnea, No Cough.

Cardiac : No Palpitations, No Chest pain, No Orthopnea.

Vascular : No Phlebitis, No Varicocities.

Genito-Urinary : (-) No Dysuria, (-) No Flank Pain, No Discharges, No Urgency.

Breast : No breast hypertrophy. No discharges. No nipple changes or discharges.

Neurologic : No Memory Loss, No Seizures.

Musculoskeletal : (-) No Joint Pains, No Cramps.

Psychiatric : No Psychiatric Complaints.

PHYSICAL EXAMINATION

VITAL SIGNS

Temp : 37.6

Blood Pressure : 111/67

HR : 119

RR : 24

BMI : 16.64

General: Awake, Irritable, In tears, Not in Respiratory Distress

Skin: The Skin is warm mottled; skin turgor is slightly delayed; (+) diffuse
polymorphous maculopapular rash on trunk, abdomen and extremities
erythematous, but no vesicular eruptions
Lymph nodes: (+) left anterior cervical lymphadenopathy

Skull: Normocephalic, Atraumatic

Eyes: Symmetrical eyes, No exophthalmos. (+) Bulbar conjunctivitis on both

eyes with an avascular zone around the iris. No exudates. Pupils equal and
reactive to light. Accommodation reflex present

Ears: Symmetrical pinna, No discharges, Intact tympanic membrane.

Nose : Nasal septum midline. Nasal mucosa appears pink and without any abnormal
discharge. No nasal Polyps or other lesion noted. Frontal and maxillary sinuses are
nontender.

Mouth and Throat : Peeling/ cracked lips. Erythematous oral mucosa (+)
strawberry tongue.

Chest : Thorax is symmetric. Full expansion is noted bilaterally. Anterior/Posterior

diameter is within normal limits

Lungs : Fremitus is equal bilaterally. Lung fields are resonant throughout. Clear
breath sounds, vocal fremitus is equal bilaterally. There are no rales or ronchi or any
other such adventitious breath sounds.

Cardiovascular : Adynamic precordium. No heaves or thrills on palpation. No

murmurs noted.

Abdomen : Soft (+) Polymorphous rashes noted. Non tender. (-)

Hepatosplenomegaly

Extremities : Warm to touch extremities. No cyanosis. (+) Dorsal edema of hands

and feet. CRT = 2s. (-) periungual desquamation. Radial, brachial, popliteal
pulses are 2+ and equal bilaterally. No joint deformities noted.

Genitourinary and Digital Rectal Exam : Not performed.

NEUROLOGICAL EXAMINATION

Patient is awake, irritable periodically. GCS 15

Kernigs sign ve.

Brudzinski sign ve.

No deficits in memory.

Good insight and judgement.

Cranial nerve examination

I - Can smell ethyl alcohol

II - Fundoscopic exam shows normal cup to disc ratio. No papilledema noted. No

optic atrophy seen. Patient is able to assess all visual fields and exhibits normal
visual acuity.

III, IV, IV - No ptosis, pupils are equal measuring approximately 2mm and are
reactive to light and accommodation and also exhibit normal direct and consensual
light reflexes. Negative for vertical as well as horizontal nystagmus. Patient shows
adequate extraocular movements equally and bilaterally.

V - Equal Sensation to the face, masseter and buccinator muscle tone are adequate.

VII - Patient does not exhibit facial asymmetry.

VIII - Patient is able to hear equally on both sides

IX, X - Patient is able to swallow, shows adequate gag reflex, exhibits no hoarseness
of any sort, uvula is midline

XI - Shows good sternocleidomastoid muscle strength and trapezius muscle strength

XII - No tongue deviation seen, no fasciculations, tremors or atrophy observed

Gait and station Normal Gait and Station

Motor strength 5/5 in both upper and lower extremities

ADMITTING IMPRESSION

Complete/ Classical Kawasaki disease (Acute Phase)

DIFFERENTIAL DIAGNOSIS

Streptococcal Scarlet fever

Stevens Johnson Syndrome

MEASLES

KAWASAKI DISEASE(Acute phase)

Course in the Ward

DAY 1

DAY 2

DAY 3

DISCHARGE
Home meds:

Give Aspirin(Aspirin Bayer) 490mg/Paper TAB every 6 hours PO for three

days #13

>2 am 8 am 2pm 8 pm

Continue until follow up when dosage is completed.

DISCUSSION

KAWASAKI DISEASE

Mucocutaneous Lymph Node Syndrome

> Its an acute, self limited, multisystem vasculitis of infancy and childhood.

> In developed countries, KD has replaced Acute Rheumatic Fever as the

most common acquired heart disease in children.

> MI in young adults can be missed KD in childhood.

INCIDENCE AND ETIOLOGY

Peak onset 6-11 months

80% are under 4 years old

ETIOLOGY Clinical and epidemiological features suggest infectious cause

Probable immunologic response triggered by several different microbial agents

Recent researches suggest cytoplasmic inclusion bodies and viral particles in KD

tissues

PATHOPHYSIOLOGY

Generalized systemic vasculitis involving blood vessels throughout the body

Active inflammation progressive fibrosis scar formation

In coronary arteries arterial remodelling or revascularization

CLINICAL DIAGNOSTIC CRITERIA

Fever for atleast 5 days and 4 of the following

1. Conjunctival injection

2. Changes in the oral mucosa (mouth & lips)

 3. Changes in the peripheral extremities

4. Polymorphous rash

5. Cervical lymphadenopathy

AND illness process not explained by other unknown disease process

FEVER High, spiking, remittent with an avg duration of 11 days.

BULBAR CONJUNCTIVAE Sparing limbus, No exudates, No conjunctival edema, No

corneal ulceration, No pain

ERYTHEMA OF ORAL MUCOSAE Within 1-3 days after onset of fever, fissuring and
cracking/peeling of lips

Strawberry tongue with prominent papillae & erythema

*No oral ulcerations, pharyngeal exudates & Kopliks spots

PERIPHERAL EXTREMITIES

Acute phase

Induration (swelling) of hands & feet (dorsal edema); sometimes painful

Erythema of palms & soles; abrupt change to normal skin at wrist & ankle (stocking
& glove areas)

Subacute phase (2 wks after onset)

Periungual desquamation of fingers and toes.

Convalescent phase (1-2 months post onset)

Transverse grooves across lines (Beaus lines

PERIPHERAL EXTREMITIES

RASH

Polymorphous rash on trunk & extremities

Most common: non-specific diffuse maculopapular erythematous rash

No bullous & vesicular eruptions

CERVICAL LYMPHADENOPATHY

Least common feature (50-75%)

Usually unilateral & confined to the anterior cervical triangle

Classic criterion:

1 lymph node

> 1.5 cm in size

Nonfluctuant, nonpurulent

nontender; no marked erythema

CARDIAC COMPLICATIONS

ACUTE PHASE

Myocarditis

Pericarditis

CHF/Mitral insufficiency

SUBACUTE PHASE

Mitral insufficiency

Coronary aneurysm

Coronary thrombosis with infarction

CONVALESCENT PHASE

Coronary and peripheral aneurysms may persist

CHRONIC PHASE

Angina pectoris &/myocardial insufficiency may develop

TREATMENT

ASPIRIN

Anti-inflammatory dose in acute phase= 80-100 mg/kg/day given every 6 hours

Antiplatelet / anti-thrombotic dose: 3-5 mg/kg/day single dose 2-3 days after the
fever lyzes; given for 6 weeks & continued indefinitely if coronary abnormalities are
observed

IV Immunoglobulin

Acute phase:

2 g/kg given single infusion for 12 hours OR

400 mg/kg/day for 4 days leads to rapid defervescence of fever & more rapid
normalization of acute phase reactants compared to treatment with ASA alone
improves myocardial function

Heparin/ Warfarin

Indicated in large coronary aneurysms with or without thrombus

IV heparin or subcutaneous low molecular weight (LMWH) heparin

Warfarin dose titrated to target INR of 2 2.5; should be given with low dose aspirin

Disadvantage: interactions with Vit K rich food and many drugs

Corticosteroids

ONLY indicated for IVIG resistant patients

(15% of patients have resistance to 1st IVIG)

Varying responses to addition of steroids to IVIG

Treatment of patients who failed to respond to intial therapy

Retreatment with IVIG 2g/kg

Steroids: should be restricted to children in whom 2 infusions of IVIG have been

given

IV Methylprednisolone: 30 mg/kg for 2-3 hours once daily for 1 to 3 days

Infliximab (anti-TNF-alpha agent)- given with 2nd dose of IVIG in IVIG resistant
patients

Others: Plasma exchange Ulinastatin, Abciximab, Monoclonal antibodies

LONG TERM FOLLOW UP

1. After baseline 2D-echo, repeat study:

2-4 weeks after onset of illness

4-6 months after illness

9-12 months after illness

2. After baseline ESR & Platelet Count REPEAT

2-3 weeks after onset of illness

6-8 weeks following onset

guide in discontinuation of salicylates

3. ECG as necessary deemed clinically indicated

THANK YOU