4-YEAR-OLD BOY WITH

FEVER

Sesión Clínica de Estudiantes Daniel Alonso Losada

Hospital Universitario Central de Asturias Isabel Vallejo Manjón
07/12/2018 Álvaro Villarejo Pérez
 CLINICAL CASE
# DAY 1:
• ER: 4-year-old boy • CT:
• Fever (2 days ago) → peak of 38.5°C • Blood:
• Left cervical lymphadenopathy • Leukocytes 20.76x10E3/μl (N 65.7%)
• Diarrhea • CRP 5.3mg/dL
• PCT 0.13
• Personal history: • EB-VCA IgG+ IgM- EB EBNA IgG+-
• Preterm (36 weeks): 2.630 Kg • CMV IgM-
• Physiological heart murmur
• NKA
• Rapid strep test (-)
• No domiciliary tx • Virus exudate: HHV-7 +
• Several URTI + Otitis
• Cx → Open trigger finger release surgery • Dx: Infectious lymphadenitis
• Vaccinations: ✓
• Treatment:
• Physical Examination: • Amoxi/clav 80mg/kg/day
• Fever • Ibuprofen
• Exanthema
• Left cervical lymphadenopathy 3-4 cm
 CLINICAL CASE
# DAY 2: # DAY 3:
• ER: Extremities exanthema • ER: Fever (4 days)
• PE: • PE:
• Left cervical lymphadenopathy • Low grade fever: 37.5°C
• Low-grade fever: 37.8°C (3 days) • Trunk and extremities exanthema
• Pharinx: slightly hyperemic • Conjuctival injection
-clav
• Exanthema (non-allergic related) • Left cervical lymphadenopathy
• Dx: exanthema (probably viral) • Cracked lips
• Strawberry tongue
• Plan: keep amoxi/clav + ibuprofen • Interfalangic edema
• Plan:
• Lab test→ ESR
• Keep amoxi/clav ( ↓50mg/kg/day)
• Admission
 PEDIATRIC FACILITY
# DAY 4
• PE: Fever (5 days)
• Dx: Kawasaki disease
• Plan → CT:
• Blood
• CRP 10.5mg/dl
• Leukocytes 21.57x10E3 (N 78.8%)
• Echocardio
• Urine systematic and sediment
• Treatment:
• Inmuneglobulin 2g/kg/day iv
• Salicylic acid 80mg/kg/day
• STOP amoxi/clav
 ECHOCARDIOGRAM

• Normal heart function and

structure
• No pericardial effusion
• Normal coronary arteries
(CA)
• Z-score:
• LCA: -0.27
• RCA: -0.5
• Trivial Mitral Regurgitation

Representative images of bilateral aneurysm in KD

 FOLLOW-UP
#DAY 5 #DAY 6
• Fever: peack of 39.1 ⁰C • Clinical improvement:
• No fever
• Urine cultive: (-) • Persistance strawberry tongue + cracked lips
• Systematic and sediment: • ↓ Cervical lymphadenopathy
• Leukocytes ++ • Plan: ↓SA dosage to 50mg/kg/day
• Cetonic bodies ++
• Erythrocites +
#DAY 7
• Plan: no changes
• Clinical improvement
• CT: CRP 3.2 mg/dL ( ↓ 30%)
#DAY 12: HOSPITAL DISCHARGE • Plan: No changes
• Clinical features:
• No adenopathy
• Still strawberry tongue + cracked lips #DAYS 8-12
• Treatment: SA 25mg/6h oral • Tx: ↓SA dosage to 5mg/kg/day (#day 8)
• Plan: Appointment with Paediatric • Follow up until discharge: no
Cardiology in 9 days complications
 KAWASAKI DISEASE
 Medium and small vessels vasculitis
 Self-limited condition
 Cardiological complications
ETIOLOGY  UNKNOWN
• Infectious agents
 EPIDEMIOLOGY: • Genetic factors
• Higher prevalence in asiatic countries • Environmental factors
(Japan)
• No studies abour incidence in Spain
• 85% of cases in <5 yo
• More frequent in men and
Winter/spring season
• Mortality 0.1-0.3%
 DIAGNOSTIC CRITERIA

SEVERAL CASES
REPORTING PATIENTS
WITH ALL CLINICAL
MANIFESTATIONS
APART FROM FEVER

o CPR – cutoff 30mg/L o Hyponatremia

LABORATORY FINDINGS o ESR – cutoff 40 mm/h o Hypoalbuminemia
MAY SUPPORT THE o PCT o Elevated liver enzymes
DIAGNOSIS o Leukocytosis with
neutrophilia
 DIAGNOSTIC CRITERIA
o CLINICAL EVALUATION + LABORATORY TESTS
CPR < 30 mg/L and
ESR < 40 mm/h
o ECHOCARDIO IF PERSISTANT FEVER OR
PERIUNGAL DESQUAMATION
FEVER ≥ 5 DAYS and
NO
2-3 clinical criteria
or o 3 or more of the following:
FEVER ≥ 7 DAYS  Anemia
without other  Platelets ≥ 450000 day 7 of FEVER
explanation  Albumin ≤ 3 g/Dl
CPR ≥ 30 mg/L and  High ALT
ESR ≥ 40 mm/h  Leukocytes ≥ 15000/mm
 Urine ≥ 10 leukocytes/high-power field

TREAT YES OR

o Positive Echocardio
DIAGNOSTIC CRITERIA
 DIFFERENTIAL DIAGNOSIS
MEASLES, ECHOVIRUS, ADENOVIRUS AND EBV:
 Less systemic inflammation
 Lack of extremity changes

 GROUP A STREPTOCCAL TOXIC-MEDIATED ILNESSES

(eg, scarlet fever TSS)
 Lack the ocular and articular involvement
 Oedema rarely confined to hands/feet

 Rocky Mountain spotted fever and Leptospirosis

 Headache and GI complaints prominent

 Stevens-Johnson Syndrome
 Subtle differences in oocular and mucosal
 Laboratory markers normal/midly elevated

 Systemic juvenile idiopathic arthritis

 Lack of conjunctival and oral findings
 Generalized lympadenopathy +/- splenomegaly
 CARDIOLOGICAL COMPLICATIONS

1.CORONARY ARTERIES ANEURYSMS

• Most important pronostic factor
• Incidence 25%
• IGIV dependent
• RISK STABLISHED for FOLLOW-UP
2.MIOCARDITIS
3.CARDIOGENIC SHOCK
4.MITRAL AND AORTIC INSUFFICIENCY
5.PERICARDITIS
 TREATMENT
FIRST LINE: SECOND LINE
 IVIG 2g/kg one single dose  Corticoids  useful in
 Mechanism still unknown Japanese pts ↑ risk
 Most effective within the first 7-10 days  Ifliximab
 CA aneurysms prevention only dependent upon IGIV  Anakinra
 ESR ↑  Cyclosporine
 Cyclophosphamide
 SA  Dipyridamole
 Anti-inflammatory effect 30-50 mg/kg/day  Clopidogrel
 Anti-platelets effect 3-5mg/kg/día one single dose oral  Abciximab
 Alteplase
>> Treatment with IVIG within the first 10 days of illness  Urokinase
reduces the prevalence of CA aneurysms fivefold compared  LMWH
with children not treated  Acenocoumarol
 FOLLOW-UP AFTER HOSPITAL DISCHARGE
 Pts are stratified during their time in hospital

Z SCORE RISK FOLLOW UP

<2 Normal coronary arteries Not required. Appointment after 12 months and discharge (optional).

2 to <2.5 Transient dilation Appointment after 12 months and discharge

≥2.5 to <5 Small aneurysm Persistent  6-12 months (1st year), once a year the following /// Decrease  1-3 years

Persistent  3-6-12 months (1st year) // Lowered to small  1 year // Lowered to

≥5 to <10 Medium aneurysm normal or dilation  2 years
Persistent 1-2-3-6-9-12 months (1st year) 3-6 months (the following) // Lowered to
10 Large or giant aneurysm small or médium  6-12 months // Lowered to normal or dilation  1-2 years

 Cardiovascular risk in pts w/out aneurysms = normal population

 CONCLUSIONS

• Guides recommend changing from antiinflammatory

doses to antiaggregant doses from SA when fever stops

• Patients do not always match 100% with clinical guides

• Doubts can be presented during the work-up when

findings does not correlate with what we are expecting
 SUMMARY
 The diagnosis of KD according to classical criteria requires the presence of fever
at least 5 days, combined with at least four of the other five signs of
mucocutaneos inflammation, without any other explanation.

 Patients who fulfill the criteria of KD require treatment because of the

cardiovascular complications that may result in significant morbidity and
mortality.

 In children with KD who are determined to be at increased risk of IVIG

resistance, we suggest adding glucocorticoids to initial IVIG therapy.

 The KD is pathology with certain prevalence so we have to keep in mind in

patient with elevated fever without any other explanation.
 BIBLIOGRAPHY
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