CASE REPORT

Niger J Paed 2013; 40 (4): 422 –425

Sani UM

Ahmed H

Kawasaki disease: an unusual

presentation in a 14-year old boy in

Sokoto, north western Nigeria

DOI:http://dx.doi.org/10.4314/njp.v40i4,15

Accepted: 31st March 2013

Abstract Kawasaki disease (KD)

is an acute systemic vasculitis that

mostly affects children less than

(8.3mmol/L and 1.9mg/dl respec-

tively). He was managed with high

dose aspirin at 80mg/kg/day. The

dose was reduced (5mg/Kg/day)

and subsequently stopped after

serial echocardiography showed

normal coronary arteries. Intrave-

nous immune globuline (IVIG)

could not be started due to non

availability. Nevertheless, clinical

signs resolved, renal function nor-

malised after 6 weeks and echocar-

diographic picture did not deterio-

rate. Patient is currently on follow

up at the paediatric cardiology

clinic of UDUTH, Sokoto, Nige-

ria..

(

)

Sani UM

Ahmed H

5

years. Occasionally, it may pre-

Department of Paediatrics,

Usmanu Danfodiyo University

Teaching Hospital (UDUTH)

Sokoto-Nigeria.

sents with renal involvement of

varying severity. In Nigeria and

most of Africa, only a few cases

of KD have been reported and

these were among children within

the typical age group.

We report an unusual case of Ka-

wasaki disease with renal mani-

festation in a 14 year old adoles-

cent. Apart from the principal

features of KD comprising of high

grade fever, non purulent con-

junctivitis, polymorphous rash,

right sided cervical lymphadenitis

and symmetrical desquamative

lesions of the digits of the hands

and feet; our patient also had renal

involvement. The renal manifesta-

tions included mild periorbital

edema, oliguria, hypertension

Email: usmansani2005@yahoo.com

Conclusion: Kawasaki disease can

occur even in older children and

renal manifestation may be self

limiting. This report highlights the

need for high index of suspicion in

all cases.

(

proteinuria(++) and elevated

serum urea and creatinine

140/90mmHg), hematuria(++),

Key words: Kawasaki disease,

renal involvement, Adolescent,

Sokoto, North-western Nigeria.

Introduction

tival injection, polymorphous exanthem, cervical

lymphadenopathy (>1.5cm), changes in extremities and

2

,4

Kawasaki disease (KD) is an acute febrile illn_,₂ess charac-

oral changes . The acute vasculitic process in KD can

also leads to disturbed haematological profile and abnor-

malities of the cardiovascular, renal and respiratory

1

terised by widespread systemic vasculitis. It occurs

3

,4

.

primarily in young children under the age of 5 yrs

The disease has a worldwide distribution, but is mo₂_,s₃t

prevalent in Asia and other developed nations

1

systems . Coronary artery lesions such as aneurysms and

thrombosis occur in up to 25% of patients and are the

most important cause of morbidity and mortality .

Renal involvement is rare, but has been reported . It

can manifest₁s_,₅w_-₇i_.th proteinuria, hematuria or even frank

renal failure

.

3

1,4

Though considered less common in our environment ,

the burden may actually be underestimated due to low

index of suspicion.

5

-7

As there is no specific laboratory parameter for confir-

matory diagnosis of KD, diagnosis is made using clini-

cal criteria which was first described by Tomasaku Ka-

wasaki and adopted by the American Heart Associa-

There are only few reports of KD in Africa including

Nigeria and almost all were among children within the

8

,9

typical age group . O₁c₀c_,₁u₁rrence in adult and older pedi-

2

tion . Principal features required for diagnosis are pres-

atric age group is rare . We report an unusual case of

ence of fever for at least 5days together with four of five

of the following signs: non exudative bilateral conjunc-

Kawasaki disease in a 14 year old boy who presents

with renal involvement

4

23

Case

Fig 3a: A Parasternal short

axis view (PSAX) showing

normal right coronary artery

A 14 year old boy presented with 2 weeks history of

fever, skin rashes and redness of the eyes. There was no

history of contact with children having skin rashes and

his immunisation status was complete. He was initially

treated with oral antibiotic, Paracetamol and antimalarial

at home. Chlorpeniramine (Piriton) tablets were also

given on suspicion of an allergic disorder. Two weeks

after the onset of symptoms, he noticed peeling of his

hands and feet which necessitated presentation to our

hospital. On examination, he was afebrile, but had dis-

crete right sided cervical lymph adenopathy (with the

largest measuring 4x3cm) and symmetric desquamation

of both hands and feet (See Fig. 1 and 2). He had mild

peri-orbital puffiness, with slightly elevated blood pres-

(RCA) in the patient at

presentation. Note the labelled

arrow pointing at the RCA.

Fig 3b: Parasternal

short axis view at aortic

level showing normal

Left coronary artery

(indicated by an arrow)

th

sure (140/90mmHg or >95 percentile for age). A diag-

nosis of Kawasaki disease was considered based on

2

clinical criteria . Complete blood count showed WBC

3

of 11.8 x 10 /µL with relative neutrophilia (8.2 x 10 /

3

µL) and platelet count of 277. X 10 /µL. Erythrocyte

sedimentation rate (ESR) was 20mm/hour. Urinalysis

showed hematuria (++) and proteinuria (++). Serum

Urea and Creatinine were slightly elevated (8.3mmol/L

and 1.9mg/dl respectively). Echocardiography showed

normal Coronary arteries (Fig 3). Blood and urine cul-

tures were negative. In view of strong suspicion of

Kawasaki disease with renal manifestation, patient was

commenced on high dose Aspirin at 80mg/Kg/day.

Intravenous immune globulin (IVIG) could not be

started due to non availability.

There was clinical improvement with significant regres-

sion of lymph adenopathy, resolution of oedema and

normalisation of renal function. However, his blood

pressure remained slightly elevated (140/85_t_hmmHg) and

returned to normal (100/70mmHg or <90 percentile)

th

only by the 6 week of illness. Serial urinalysis after

discharge showed persistence of proteinuria and_t_h hema-

Fig 1: Cervical

lymphadenitis.

Note the arrows

showing enlarged

cervical lymph

nodes in the patient

turia of ++ each, but complete resolution by 9 week.

th

Repeat Echocardiograhy by 6 week still showed nor-

mal coronary arteries. Aspirin was changed to low dose

(

5mg/Kg/day) and subsequently stopped. His clinical

Enlarged cervical

lymph nodes

condition has remained stable since discharge and is

currently on follow up.

Discussion

This is the first report of Kawasaki disease presenting at

an unusual age and with renal involvement from our

centre. Our case fulfilled 5 of the clinical criteria for

diagnosis of Kawasaki disease namely fever, exanthema,

bilateral non exudative conjunctivitis, cervical lympha-

denitis and desquamative changes of the hands and feet.

Initially, the diagnosis was not suspected as typical fea-

tures such as desquamation and lymphadenitis were

lacking in the patient, but these appeared subsequently.

It is well documented that clinical features of KD may

not appear all at the same time and diagnosis often

Fig 2: Symmetric des-

quamation of the hands

in the patient with

Kawasaki Disease

1

2

require sequential evaluation of patient .

A major challenge in the diagnosis of KD is the lack of a

specific diagnostic parameter to confirm diagnosis.

Some patients (up to 15%) with KD may not even fulfil

the clinical criteria and may have atypical or incomplete

KD characterised by presence of only two or three prin-

cipal features with no other diagnostic or laboratory fea-

4

24

tures supportive₁o_,₄f_,₁₂severe inflammation or coronary

artery aneurysms . This can result in misdiagnosis/

The observation of proteinuria, hematuria, elevated

blood urea nitrogen and mild hypertension in our patient

is a definite evidence of renal involvement. The patho-

genesis of renal ma₁n_,₄i_,₁f₂estation in KD is attributed to the

under diagnosis particularly if index of suspicion is low.

The paucity of reports on KD in Nigeria and most of

Africa may reflect the rarity of the disease in our envi-

ronment; but underestimation of the actual disease bur-

den due to non recognition is another possibility.

vasculitic process

, though other workers failed to

demonstrate histopathologic evidence for this in their

patients and suggested the possible role of immune me-

6

diated injury during the sub acute phase of the disease .

KD is generally a disease of the young, with 80% of

cases occurring in children less than 5 years . Unlike

We did not do renal biopsy in our patient as it was con-

sidered unnecessary. This is because the patient had

clinical and biochemical evidence of resolution of his

renal function. The complete recovery of renal function

in our case confirms earlier reports that renal₁_,₅i_,n₇ volve-

4

Sotimehin et al in Nigeria and Badoe et al in Ghana w₈h_,₉o

reported KD in 3 and 4 year old children respectively

,

our patient presented at an unusual age of 14years. A

few other reports across the w₀_,o₁₁rld have also reported

ment in KD is usually benign and self limiting

.

1

KD among atypical age group . Kara and Tezer et al

1

reported it in a 30-day-old neonate where as Rozo and

colleaques observed the typical features of the disease in

Though intravenous immune globulin (IVIG) has been

shown to reduce the risk of co₄r_,₁o₂nary artery lesion when

given early at a dose of 2g/kg , it was not used for our

patient due to non availability- a typical problem in

many developing countries. Despite this limitation, our

patient clinical condition remained normal and his echo-

cardiographic picture did not deteriorate. However, he

was given Acetyl Salicylic Acid (aspirin) which is also

an integral component of management of KD that is

recommended for use during the initial and convalescent

1

0

a 36-year-old man . It was also reported in a 2-wee₁₂k

old-neonate- the youngest age in the world so far

.

Hence irrespective of age, Kawasaki Disease should

always be considered as a possible differential.

The exact cause of the disease is still unknown though

an inciting agent, such as bacterial super antigen or a

viral agent in a susceptible host may trigge₂r_,₄a_,₁n₂ immune

2,5

vasculitis which is typically multi systemic

. Cardiac

stages of the disease .

complication is the most life threatening sequalae of the

dis₂_,e₄a_,₁s₂e, largely due to its effect on the coronary arteri-

es

. Fortunately, our patient had no echocardio-

graphic evidence of coronary artery involvement at pres-

entation and up to the time of last follow up echocardi-

ography by six month. Predictive factors for coronary

artery lesion (CAL) including marked leucocytosis

Conclusion

Kawasaki disease can occur even in older children and

may present with renal involvement which is self limit-

ing. Since early diagnosis with institution of appropriate

treatment can significantly reduce the risk of morbidity

and mortality, clinicians should have high index of sus-

picion for KD to prevent misdiagnosis.

(

>30,000/mm3), elevated ESR (>101mm/hr), low hemo-

globin (<10g/dl), prolong fever (>14days), hypoalbu-

2

,4

minaemia and male gender have been described . Ex-

cept for the latter, our patient had none of the above risk

factors. Hence, it is not surprising that serial echocardio-

graphic evaluation of his cardiac status remained nor-

mal. Some authors have observed the occurrence of se-

vere coronary artery abnormalities in their patients such

as giant aneurysms, thrombosis and₁ myocardial infarc-

Conflict of interest: None

Funding: None

1

tion leading to death in some cases.

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