PP on kawasaki disease

1. A CHILD WITH INCOMPLETE KAWASAKI DISEASE
N.SHRUTHI
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2. CHILD NILAANI SURESH
• Age: 11 months
• Sex: Female
• Address: Jafferkhanpet, chennai
• Date of admission: 04.11.2022
• Unit: First Unit
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3. Presenting Complaints
• Fever since 10 days
• Cough since 7 days
• Seizure on day 2 of illness
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4. Details of presenting illness
• Child had fever on and off since 10 days.
• Fever intensity is mild to moderate.
• It is intermittent in nature.
• No chills or delirium
• Cough is occasional, not brassy, not pertussaid, not productive.
• Seizure on day 2 of illness is generalized tonic clonic with loss of consciousness and
persisted for 2 to 3 minutes. Recovered spontantaneously. No post ictal palsy.
• No history of diarrhea, vomiting or abdominal pain.
• Child was treated in a nearby hospital and as the conditioning was worsening child got
admitted in our hospital.
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5. Treatment before admission
• Child was admitted in a hospital on 6th day of illness at Dharmapuri and
treated for 4 days before coming to our hospital
• Inj. Piptaz for 4 days
• Inj. Amikacin for 4 days
• Suspension Azithral for 100 mg 0rally for 2 days
• Syrup Paracetamol, Mefenamic acid, Oseltamivir, Syp. Prednisolone, and Syp.
Levosalbutamol for 5 days.
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6. Past history
• No similar illness in the past.
• No history of exposure to infections from the family members
• No history of seizures in the past.
• No history suggestive of any chronic illness like Asthma, cardiovascular
diseases or diseases suggestive of immune deficiency.
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7. Birth history
• Antenatal: mother was registered for AN Care in local health facility and
received TT vaccines. Her antenatal ultrasound studies were normal.
She had GDM and PIH and received appropriate treatment from the
primary care facility.
• Natal History: Elective LSCS, cried well after birth. Birth weight 2.98 kg
• Postnatal History: Child was with mother for rooming-in, breastfeeding
and there was no need for intensive care.
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8. Family and Developmental history
• Child was second born to nonconsanguineous parents.
• Received immunization as per national schedule for the age.
• Developmental history:
• It is essentially normal.
• Motor: Now child walks with support
• Fine motor: has immature grasp
• Speech: babbling
• Social: shows stranger anxiety
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9. Physical Examination
GE:
• Adequately nourished: 8 kg for 11 months; no evidence of vitamin deficiencies.
• Conscious, responds to stimulus, afebrile with adequate hydration
• Pallor+, no icterus, no cyanosis; no clubbing; no jaundice; no generalized lymphadenopathy; no
oedema.
• Pulse rate: 166/min;
• regular; equally palpable in both upper and lower limbs.
RS: Respiratory rate: 35/min; not dyspnoeic; equal movements on both sides, no chest retractions;
normal breath sound on both sides; no adventitious sounds.
CVS: S1 and S2 normal; no murmurs; no cardiomegaly.
GIT: oral thrush present; abdomen not distended; no significant organomegaly; normal bowel sounds;
normal genitals.
CNS: Conscious; no meningeal signs; no focal neurological deficits.
Musculoskeletal: Normal power and tone
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10. Anthropometry
• Weight: 8 kg; Z score -1 to -2
• Height: 73 cm; Z score -1 to -2
• Mid arm circumference: 13 cm; Z score 0 to -1
• Head circumference: 43 cm; Z score -1 to -2
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11. Initial Diagnosis
• Sepsis
• ? Kawasaki disease
• Febrile seizures
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12. Biochemistry
• Triglyceride: 434 mg/dL (High)
• Ferritin: 230 ng/mL (High)
• LDH: 872 U/L (High)
• BUN: 20 mg/dL (Normal)
• Creatinine: 0.3 mg/dL (Normal)
• Sodium: 133 mmol/L (near normal); Potassium: 6.1 mmol/L (High); Chloride: 97 mmol/L (Near
normal)
• Bicarbonate: 21 mmol/L (Normal)
• S.G.O.T: 64 IU/L (Raised); S.G.P.T: 17 IU/L (Normal)
• Alkaline Phosphatase: 170 (Raised)
• Total protein: 6.5 g/dL (Normal) Albumin: 3.5 (Normal); Globulin: 3.0 (Normal); AG Ratio: 1:1
(Normal)
• Bilirubin: Total: 0.24 m mg/dL; Direct: 0.031 (Normal); Indirect: 0.21 (Normal)
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13. Clinical Pathology
• ESR 1 hour: 93 (Raised)
• Routine urine exam: Essentially normal
• Peripheral smear examination:
• RBC: hypochromic; microcytic; anisopoikilocytosis
• WBC: lymphocytic leucocytosis; atypical lymphocytes present.
• Platelet: Thrombocytosis
• Hemoparasites: Nil
• HB: 8.9 gm/dL (Anaemia)
• TC: 42150 cells/cumm: High; metamyelocytes and atypical lymphocytes seen
• RBC count: 5.3 mill/cumm
• Platelet: 12.01 lakhs/cumm
• Blood culture: Streptococcus pneumoniae; repeat culture: No growth; Urine culture: No growth.
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14. Imaging
• Chest: evidence of pneumonia; consolidation with minimal fluid
• Usg Abdomen: Normal
• ECHO by Cardiologist: dilatation of LMCA; repeat ECHO showed
confirmed coronary dilatation.
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15. 15
Left lung patches +
Parenchymal infiltration in left lung
Pleural effusion in left lung
Left lung consolidation + visible in
5/11 and 8/11 xrays

16. Course in the hospital
• IV Piperacillin, Tazobactam and Amikacin for sepsis
• Oral Doxycycline for ? Scrub typhus
• In view of high TC, anaemia and high platelet count, raised CRP, LDH, ESR, persistent fever
spikes and ECHO findings, a diagnosis of incomplete Kawasaki disease was made.
• IV immunoglobulin 2 gm/kg was given.
• Repeat CBC showed reducing trend.
• Streptococcal Pneumoniae sepsis shown in 1st blood culture
• Xray : consolidation with minimal fluid.
• Hyperkalaemia and elevate BUN: showed reducing trend on treatment
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17. At discharge
• No symptoms
• Feeds well
• Afebrile for more than 24 hours
• Normal urine output
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18. Discharge advice
• Review in C2 OPD
• Iron supplement
• Diet advice
• Cardiologist review and ECHO at 2 weeks, 6 weeks, 12 weeks and at 6
month.
• To collect pending reports like AFB, CMC report
• To report if there is:
• Fever
• Vomiting
• Loose stools
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19. KAWASAKI DISEASE
1. Synonyms: mucocutaneous lymph node syndrome; infantile polyarteritis nodosa.
2. Highest incidence occurring in Asian children more in Japanese.
3. KD is a vasculitis with a predilection for the coronary arteries. Approximately 20-25% of
untreated children develop coronary artery abnormalities (CAA) including aneurysms, whereas
<5% of children treated with intravenous gammaglobulin (IVIG) develop CAA.
4. KD is the leading cause of acquired heart disease in children in most developed countries,
including the United States and Japan.
5. Age: < 5 year; mean age 3 year
6. Sex: More in boys
7. Risk factors: young age, male gender and Asian and Pacific Islander race and Hispanic ethnicity
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20. ETIOLOGY
1. The cause remains unknown
a. Infection: epidemiologic and following clinical features support an infectious origin
b. Infrequent occurrence of the illness in infants younger than 3 mo, likely due to maternal antibodies
c. Less common in adults likely due to prior exposures with subsequent immunity.
2. Points against infectious origin:
i. No multiple cases present at the same time within a family or daycare center
ii. No single infectious etiologic agent has been successfully identified so far.
3. Genetic role:
a. Asian children more affected
b. Siblings are affected
c. Significant associations between polymorphisms in the ITPKC gene, a T-cell regulator, with increased
susceptibility to KD
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21. CLINICAL MANIFESTATIONS
1. Fever high unremitting, and unresponsive to antibiotics. The duration of fever without
treatment is generally 1-2 wk, but may persist for 3-4 wk.
2. 5 clinical criteria of KD :
a. Fever at least for 5 days
b. Bilateral nonexudative conjunctival injection with limbal sparing;
c. Erythema of the oral and pharyngeal mucosa with strawberry tongue and red,
cracked lips;
d. Edema and erythema of the hands and feet; rash of various forms (maculopapular,
erythema multiforme, or scarlatiniform)
e. Nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm
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22. 3 PHASES OF ILLNESS
1. Febrile phase 1-2 week
2. Sub acute phase 3 weeks
a. Desquamation,
b. Thrombocytosis,
c. The development of CAA
d. Sudden death
3. The convalescent phase: (ESR) returns to normal 6-8 wks. after the
onset of illness.
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23. LABORATORY AND RADIOLOGY FINDINGS
a. The leukocyte count is elevated, with a predominance of neutrophils and
immature forms.
b. Normocytic, normochromic anemia
c. The platelet count is generally normal in the 1st wk of illness and rapidly
increases by 1,000,000/mm3.
d. An elevated ESR and/or C-reactive protein
e. Sterile pyuria, mild elevations of the hepatic transaminases, hyperbilirubinemia,
and cerebrospinal fluid pleocytosis may also be present.
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24. ECHOCARDIGRAPHY:
a. Two-dimensional echocardiography:
a.lack of normal tapering of the vessels is typical.
b.coronary artery dimensions may be increased in the 1st 5 wk after
presentation.
c.Aneurysms have been defined with use of absolute dimensions by the
Japanese Ministry of Health and are classified as small (<5 mm internal
diameter), medium (5-8 mm internal diameter), or giant (>8 mm
internal diameter).
b. Periodic evaluation for preventive cardiology counseling is warranted, and
cardiologic follow-up every 5 yr.
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25. DIAGNOSIS
a.Complete KD: presence of fever for at least 4 days and at least 4 of
5 of the other principal characteristics
b.Incomplete KD: patients have persistent fever but fewer than 4 of
the 5 characteristics plus laboratory and echocardiographic
findings
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26. DIFFERENTIAL DIAGNOSIS:
VIRAL INFECTIONS
a. Adenovirus
b. Enterovirus
c. Measles
d. Epstein-Barr virus
e. Cytomegalovirus
BACTERIAL INFECTIONS
a. Scarlet fever
b. Rocky Mountain spotted fever
c. Leptospirosis
d. Bacterial cervical lymphadenitis
e. Meningococcemia
RHEUMATOLOGIC DISEASE
a. Systemic-onset juvenile idiopathic arthritis
b. Behçet disease
OTHER
a. Toxic shock syndromes
b. Staphylococcal scalded skin syndrome
c. Drug hypersensitivity reactions
d. Stevens-Johnson syndrome
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27. Kawasaki disease. Strawberry tongue

28. Kawasaki disease. Congestion of bulbar conjunctiva

29. Kawasaki disease. Indurative edema of the hands

30. Kawasaki disease. Desquamation of the fingers

32. TREATMENT
a. ACUTE STAGE
a. Intravenous immunoglobulin 2 g/kg over 10-12 hr
b. Aspirin 80-100 mg/kg/day divided every 6 hr orally until patient is afebrile for at least 48 hr
b. CONVALESCENT STAGE
a. Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset if normal coronary findings
throughout course
c. LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES
a. Aspirin 3-5 mg/kg once daily orally
b. Clopidogrel 1 mg/kg/day (maximum: 75 mg/day)
c. Most experts add warfarin or low-molecular-weight heparin for those patients at particularly high risk
of thrombosis
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33. PROGNOSIS
a. Majority return to normal health, as timely treatment reduces the risk of
coronary aneurysms to less than 5%.
b. Giant aneurysms are less likely to regress to normal lumen diameter and are
most likely to lead to thrombosis or stenosis.
c. Coronary artery bypass grafting may be required if myocardial perfusion is
significantly impaired
d. Heart transplantation has been required in rare cases
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