DK’s KDby: Wiston E. Ilagan

This is a case of D.K, 5 months old, male, Filipino, Roman Catholic, presently residing at 336 Quilo-Quilo North P. Garcia, Batangas, who was admitted due to fever of 21 days.

Chief complaint

21 days PTA • Initially presented with fever (38 – 39 C) for 2 days • Paracetamol (10 mkdose), • Temporary relief of fever.

19 days PTA• Fever escalated (40 C) • Not relieved by Paracetamol. • Prompt consult at NL Villa Memorial Medical Center in

Lipa City Batangas.• Urinary Tract Infection based on urinalysis.• started with Ceftriaxone IV (123 mkdose)

History of Present Illness (Day 1 – Day 3)

17 days PTA• Defervescence• Ceftriaxone IV was shifted to Cefexime (6

mkday)• Discharged • In the afternoon of the same day, there was

reoccurrence of fever.

History of Present Illness (Day 4)

14 days PTA• Readmitted• Febrile (39.5 ), remittent • Cefixime discontinued• Shifted to Azithromycin• Irritable • ESR ( 48 mmol/hr) and CRP were requested which

showed increased values.• Given Hydrocortisone (5mkdose),

Diphenhydramine (1mkdose) and Ampicillin-Sulbactam (100 mkday)

History of Present Illness (Day 7)

13 DAYS PTA• ( + ) dry fissured lips, strawberry tongue , redness

of the bilateral eye and brawny edema of hands and feet

• More irritable that he can’t sleep.• Febrile (39 C). • Started on aspirin 80 mkD

History of Present Illness (Day 9)

11 days PTA• Given IVIG 2 g/kg. • Apparently well and disappearance of signs and

symptoms of KD • Deferevescence were noted, 48 hours after

administration. • Still on Azithromycin and developed loose stools. • Subsequently sent home on Aspirin tablet (80

mkday) and Azithromycin.

History of present illness (Day 11)

9 days PTA• Initially asymptomatic but developed fever

(39C) in the afternoon • 3 episodes of loose stools upon discharge

which • Prompted re-admission.• Referred to Pedia Intensivist• Looked up for alternative focus of

infection. • Blood, urine and stool C/S was done that

all eventually turned negative .

History of Present Illness (Day 12)

8 days PTA• Started on piperacillin-tazobactam 89 mkd.• Referred to a pediatric cardiologist.

History of Present Illness (Day 13)

7 days PTA• 2D echo was done: Dilated right coronary artery and

left coronary at 0.3 cm proximal and distal, minimal pericardial effusion

• Febrile (39 C).• Desquamating lesion on chest, abdomen and

extremities.• Erythematous rashes in the groin and buttocks area. • Started on methylprednisolone 255 IV • Aspirin was increased to 170 mg/tab 1 tab every 6

hours. • Stool and blood culture and sensitivity showed no

growth.

History of Present Illness ( Day 14)

6 days PTA• IV methylprednisolone was shifted to oral

methylprednisolone 8 mg BID.4 days PTA• Started on metronidazole. • Febrile (~38C).

History of Present Illness ( Day 15- Day 17)

3 days PTA• Febrile (39 C), • HAMA• discharged with home medications:

Paracetamol drops (10 mkdose), Metronidazole 125/ml, Methylprednisolone 8 mg tab BID, and ASA 8 mg 1 tab ½ tab OD.

2 days PTA• Febrile (38 C). • Paracetamol was given and was relieved.

History of Present Illness (Day 18 – Day 19)

1 day PTA• Afebrile, active, not irritable with decreasing

signs and symptoms of Kawasaki Disease. • His parents decided to bring him to Manila

for further evaluation.Few hours PTA .• Upon examination in the clinic, febrile

(38.4 C) • Admission

History of present Illness (Day 20-21)

No previous hospitalizations or surgeries. No known allergies to food or medications.

Past Medical History

Birth and Maternal history• Born to a 30-year old G2P1. • Denied any illnesses or exposure to

smoking/alcohol intake/radiation during her pregnancy.

• Term via normal spontaneous delivery• With no fetomaternal complications.

Paternal

HypertensionDiabetes mellitusLung carcinoma

Maternal

HypertensionDiabetes mellitusBreast carcinoma

Family history

Social smile at 1 month. Good head control; laughs loudly; and reaches for

objects Follows moving objects with eyes and takes bottle in

mouth = 4 months

Developmental History (5 months)

Nutritional History Breastfeeding until age of 2 with formula of

S-26, 1:1 dilution Consuming approximately 4 cm.

Lives in a well-lit, well-ventilated house with 3 other household members.

Water source for consumption is distilled water, not boiled. Garbage is collected daily.

Active and cheerful child and smiles to everyone

Personal and Social

Immunizations BCG, Hepa B, 1 dose of 5-in-1 (HiB/DPT/IPV)

vaccine, and 1 dose of 6-in-1 (HiB/IPV/DPT/HepaB) vaccine

• no weight lossGeneral

• No jaundice, no cyanosisSkin

• No swelling, discharge, tenderness, discharge epistaxis, discharge, mass

HEENT

Review of Systems

• No cough, colds, difficulty of breathingRESPIRATORY

• No cyanosisCardiovasular

• no hematemesis, constipation, hematocheziaGsstrointestinal

• No polyuria, hematuria, discharge, (+) erythematous rash in inguinal area

Genitourinary • No loss of consciousness, stiffening

of muscles, blank stare, drooling of saliva, muscle twitching

Neurology

Review of Systems

Physical ExaminationGeneral •awake, alert, not in cardiorespiratory distress

•Height: 70 cm Weight: 7.5 kg Length/Ht for age = O • Weight for Age = + 1

Vital Signs •Temperature = 36 C RR = 36 cpm HR = 142 bpm

Skin •(+) brownish desquamation on the chest and trunk

HEENT •normocephalic, pink palpebral conjunctiva, anicteric sclera, non sunken eyeball, no nasal discharge, (+) erythematous, dry lips and buccal mucosa, nonhyperemic posterior pharyngeal wall, tonsils not enlarged, (+) right palpable cervical lymphadenopathies = 1 cm

Chest/Lungs •symmetrical chest expansion, no retractions, clear and equal breath sounds, no wheezes/crackles

Cardiovascular •normal rate and regular rhythm, no murmurs

Physical Examination

Abdomen •globular, no visible pulsations, normoactive bowel sounds, soft, nontender

Genitourinary •(+) perineal erythema

Extremities •(+) erythematous palms and soles, no cyanosis, (+) edema dorsa of the hands and foot, pulses full and equal

KAWASAKI DISEASE S/P INTRAVENOUS IMMUNOGLOBULIN

Admitting Diagnosis / Working Impression

COURSE IN THE WARD

May 15 (Day 1) Vital signs

Skin:HEENTLungsHeart

Genitourinary Extremities

Course in the Ward

May 15, 2012

Day 1 afebrile3-4 episodes of pasty loose stools

Improved activityGood suck

less irritable 2D echo was done with the

follewing results:Advised for second round of IVIG

Discontinue MethylprednisoloneStart Dyprimadole 25 mg / tablet, prepare 6.25 mg papertab twice

a dayGive second dose of IVIG (2g/kg)

6 vials

May 16, 2012 (Day 2)

Vital signs

Skin:

HEENT

Lungs

Heart

Abdomen

Genitourinary

Extremities

May 16, 2012

Day 2 afebrile good suck

not irritableDecided to transfer to other

hospital for IVIG

Take home medications: Aspirin 100 mg / tablet # 10

(prepare 150 mg papertablet and give 1 papertablet every 6);

Dipyridamole 25 mg /tablet # 12 (prepare 6-25 mg paper tablet

two doses a day)

Salient Features 5 month old (+)Bilateral bulbar conjunctival injection,

nonpurulent (resolved) (+) Changes in the mucosa, dry fissured lips,

strawberry tongue (resolving) (+) Changes of the peripheral extremities, such as

edema and/or erythema of the hands or feet and periungual desquamation (resolving)

(+) Rash, primarily truncal; polymorphous but nonvesicular (resolving)

(+) Cervical adenopathy, lymphadenopathy (1 cm), uniateral right

1 2 3 4 5 6 7 8 9 10111213141516171819202135.5

3636.5

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3838.5

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Se...

Fever for 21 days (high spiking, remittent, and unresponsive to antibiotics)

LABORATORY STUDIES

May 6 48 (<10 mmol/hr)

May 9 51 (<10 mmol/hr)

May 11 81 (<10 mmol/hr)

Erythrocyte Sedimentation Rate

Parameters MAY 6 MAY 9 MAY 11

MAY 14

NORMAL VALUES

WBC 19.8 23.8 22.3 5.6 5,000-10,000

Henoglobin 9 9.8 9.6 10 12-15

Hematocrit 28 30 30 30 37-45

Segmenters 54 47 50 53 55-65

Lymphocytes 32 36 31 43 26-35

Monocytes 8 17 14 --- 2-6

Eisonophiles 6 6 5 --- 2-4

Platelet 457,000

768,000

772,000

916,000

150,000-400,000

Complete Blood CountUNIT

Cu. Mm.

g/dl

Vol %

%

%

%

%

Cu. Mm.

Parameters MAY 12 MAY 14Color Light yellow YellowTurbidity Clear ClearGlucose Negative NegativeProtein Negative NegativepH 6.0 6.0Specific Gravity 1.020 1.020WBC 0-1 0-1RBC 0-1 0-1Casts None seen None seen

URINALYSIS and FECALYSIS

Parameters Result (May 5)Color yellowish brownConsistency Semi-formedWBC 0-1RBC 0-1Baxteria Few Parasites None

May 6 Blood C/S: no growth after 24 hours of

incubation May 8 Blood C/S: no growth after 2 days

May 7 There is haziness of the inner lung markings Impression: Consider Bronhitis. No consolidation

identified. 

Laboratory studies

Normal abdominal sites Apex on the left Dilated right coronary artery and left

coronary at 0.3 cm proximal and distal Normal chamber sizes Minimal pericardial effusion Good left ventricular systolic function Left sided aortic arch

2D Echocardiography May 5

Intact interarterial / interventricular septae Normal cardiac valves and cardiac dimensions Dilated Left and Right Coronary artery. Left coronary

artery approximately 2.5 to 3.4 mm. The right coronary artery measures approximately 2.3 to 2.9 mm

There is saccular aneurysm at proximal coronary artery measuring 3.8 – 4.3 mm

Good left ventricular systolic function Small pericardial effusion No thrombus / vegetation seen Color flow Doppler study reveal tricuspid regurgitation

and pulmonic regurgitation

May 15 2D echo

DIFFERANTIAL DIAGNOSIS

Steven-Johnson Syndrome

Age = 5 mos, Prolonged fever, conjunctivitis, keratitis, target

lesions, erythema of oral mucosa, pseudomembrane formation

normal extremities, (-) CLADS, exudative conjunctivitis, vesiculr rash with crusting. associated with Herpes

Virus infection, athralgia

Streptococcal Scarlet fever

Fever = variable usually 10 days; strawberry tongue, flaky

desquamation, sandpaper rash, (+) CL, irritable

Normal eyes, pharyngitis, positive throat culture for Group A strep

Rapid clinical response to appropriate antibiotic therapy

Measles

High fever, rash , non-exudative conjunctivitis, lymphadenopathy,

desquamation severe cases.

Solitary enlarged lymphadenopathy, fever = 5 days only, Koplik spots and

morbiliform rash

Diagnostic Guidelines of Kawasaki Disease (MCLS: Infantile Acute Febrile Mucocutaneous Lymph Node Syndrome)

Pathophysiology of DiseasePredisposing factors:

Age-5 mosr old, Sex-Male, Race-Asian

Precipitating Factors:

Unknown yet linked with unknown etiologic agent and environmental factors

Entry of

KD AGENT

Autoimmune Response

Release of Chemical Mediators

Vasodilation and Cellular Permeabilty

Attraction of Phagocytes and WBC

Entry of antigen on lymphatic capillaries

S/S:

Redness, Swelling, Heat

Phagocytosis by neutrophils and macrophages (antigens are localized and inflammation happens

Increase pressure due to inflammation and entry

of antibodies

Entry of pathogens in the systemic circulation↓Regulation of toxins in the body↓Release of pyrogen↓Stimulation of the hypothalamus↓Increase or alteration of thermoregulation↓Increase in body temperature↓

Hyperthermia

Swelling of tissues↓Disruption of skin surfaces↓Skin desquamation and Rash↓Impaired skin integrity

Systemic blood vessels involvement (inflammation of small & medium size vessels)

If treated:

IVIG

GOOD PROGNOSIS Refractory symptoms

Give another round of IVIG

If not treated:

Complications developed

Pericarditis

Myocarditis

Cardiomegaly

Myocardial infarction

Heart failure

Ruptured coronary aneurysym

DEATH

Formerly known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa

Acute febrile vasculitis of childhood

First described by Dr. Tomisaku Kawasaki in Japan in 1967

Occurs worldwide with Asians at higher risk

Leading cause of acquired heart disease in children

WHAT IS KAWASAKI DISEASE?

Acute, multisystem, self-limited vasculitis• Small-medium vessels: panvasculitis• Age1: 0.5-5 years (90% of cases)Peak 9-24 months• Male: female = 1.3-1.7:1

• Incidence2-4: (per 100,000/yr of < 5 yrs old)–

◦ 360 for Japanese◦ 95 for Chinese◦ 77 for Hawaiians◦ 56 for Filipino◦ 7 for Caucasians

• Recurrence: 1.3-3%

Classical KDIncomplete KD

Atypical KD

Kawasaki Disease (KD)

Classical KD Fever > 5 days +

4/5 criteria of

• Bilateral conjunctival injection

• Changes in lips and oral cavity

• Cervical lymphadenopathy

• Polymorphous exanthem

• Changes in extremities

Exclusion of other diseases

Incomplete KD

Incomplete KD 15-20% of KD1 Age: <1 yr ESR > 40 mm/hr CRP > 3 mg/dL > 3/6 Supplemental

Criteria2 •Alb < 3 g/dL •Elevate ALT •Anemia for age •Plt after 7d >

450,000/mm3 •WBC > 15,000/mm3 •UA > 10 WBC/HPF

Atypical KD

Phases of KD

Subacute phase

Convalescent Phase

Thank you!