Kawasaki Disease: Does It Affect Children in Lagos, Nigeria?-Juniper Publishers
Juniper Publishers-Journal of Cardiology
Kawasaki disease is a rare vasculitis of unknown
aetiology that occurs in childhood. It can have a fatal outcome when
there is involvement of the heart. Only few cases have been reported in
Nigeria with none reported from Lagos despite the high turnout of
children presenting in the various private, general and teaching
hospitals in Lagos. We therefore report the current case in a 23 month
old boy who presented with persistent fever, non exudative bilateral
conjunctivitis, erythema and fissuring of the lips, perinanal
excoriation and swelling of the hands and feet. At the referral
hospital, he was being managed as a case of sepsis. Echocardiography
done in LASUTH was essentially normal and he had IVIG and oral aspirin. A
remarkable recovery was made and he is currently stable.
Conclusion: Kawasaki disease
exists among children in Lagos. Diagnosis however requires a high index
of suspicion. Early identification, evaluation and treatment are
important determinants of the likely outcome.
Keywords: Kawasaki Disease; Children; Lagos; Nigeria
Case Report
Kawasaki disease is an acute febrile illness of
childhood. It is the leading cause of acquired heart disease in children
in most developed countries, including the USA and Japan [1]. The
highest annual incidence is observed in children in Japan with 210 cases
per 100.000 under-five children compared to 13 per 100,000 in white
children [2]. Kawasaki disease is a vasculitis of medium-sized arteries
with a striking predilection for coronary arteries, [3] resulting in
coronary arterial abnormalities in 20 - 25% of untreated patients [4].
The aetiologic factors involved in development of the disease are
largely unknown, however certain epidemiologic and clinical features
point to an infectious origin. The features suggestive of an infectious
origin are affectation of young age group, occurrence in epidemics with
wave-like geographic spread, the clinical manifestations of fever,
enathem, non suppurative conjuctivitis and cervical lymphadenopathy
among others [5,6].
The higher incidence of Kawasaki disease in Asian
children irrespective of country of residence and in siblings and
children of individuals with a history of Kawasaki disease is also
strongly suggestive of a genetic role in the pathogenesis of the disease
[7]. The incidence of Kawasaki disease in developing countries
(including Nigeria) is largely undefined. Kawasaki disease was
unreported in the West African country Ghana until 2011 [8].
A 5-year descriptive study in Northwestern Nigeria
showed Kawasaki disease accounting for only 1.8% of 110 patients
diagnosed as having acquired heart disease over the study period [9].
Bode Thomas et al. [10] in a 10-year retrospective study at a center in
central Nigeria identified Kawasaki disease as accounting for 0.6% (a
single subject) of cases of acquired heart disease. In all, there has no
case reported among children in Lagos.
The limited data available highlights the apparent
rarity of Kawasaki disease in the Nigerian populace. It is however not
known whether this rarity represents a true reflection of the low
incidence and prevalence of Kawasaki disease in Nigeria for diverse
reasons, for instance, Kawasaki disease demonstrates striking clinical
similarities to measles [11] which is not uncommon in Nigeria,
highlighting the possibility of missed diagnoses of Kawasaki disease. A
low index of suspicion among Physicians and other healthcare workers has
also been reported as being responsible for the apparent rarity of the
diagnosis in this environment [9].
Case Report
23 months old boy who presented with nine-day history
of persistent high grade fever. Other complaints by the caregiver were
bilateral redness of the eyes, redness and cracking of the lips, rash
around the groin region and swelling of both hands
and feet all of three to four days’ duration prior to presentation.
The high grade fever did not subside despite use of anti-malarial
and antibiotics with minimal and short response to antipyretic
given at the referral center. There was associated irritability as
the child cried inconsolably. Bilateral redness of eyes was not
associated with purulent discharge and no contact with anyone
with red eyes. Ulceration of the lips was preceded with redness;
later cracking of the lips with minimal bleeding was noticed
prior to admission. No bleeding from other parts of the body. An
erythematous rash limited to the groin was noticed and it was
not itching.
Examination findings at presentation in Children Emergency
Unit of Lagos State University Teaching Hospital, Ikeja, Nigeria
(LASUTH) were thus: A conscious but irritable child, febrile with
temperature of 39 degrees Celsius, mildly pale, had bilateral
non-purulent conjunctivitis, hyperemic ulceration of the lips
with minimal bleeding but no redness of the tongue (Figure
1a), significant cervical and submental lymphadenopathy
and bilateral pitting oedema of both hands and feet of which
tenderness could not be ascertained due to irritability. Perianal
maculopapular skin eruption with subsequent desquamation
was noticed (Figure 1b). Periungual desquamation of both hands
and feet were noticed while on admission (Figure 1c & 1d).
Abbreviations: WBC: White Blood Cell; ESR: Erythrocyte Sedimentation Rate.
No significant tachypnoea or tachycardia on admission with
respiratory rate and heart rate of 32 cycles/minute and 140
beats/minute respectively. Other systemic examination was
normal. Subsequently, haematocrit dropped further, respiratory
rate and heart rate increased to 42 cycles/minute and 160
beats/minute respectively which necessitated blood transfusion.
Investigation results showed moderate anaemia, leukocytosis
with relative neutrophilia and linearly elevated platelet counts
as shown on the (Table 1) below. Erythrocyte sedimentation rate
was as high as 90mm/hr. There was mild hypoalbuminaemia
with serum albumin of 3.3g/dl. Renal function test results were
essentially normal and there was no bacterial growth on blood
culture investigation. Electrocardiography with color Doppler
revealed a structurally normal heart.
Medications given in LASUTH were high dose oral aspirin
and intravenous immunoglobulin. The patient was continued
on low dose aspirin after the resolution of the fever. Clinical
condition improved within two weeks of admission in LASUTH
with resolution of fever, clearance of bilateral conjunctivitis
and cracks on the lips and minimal residual desquamation of
the digits at discharge. Patient is currently on follow up at the outpatient clinic and is clinically stable.
Discussion
Kawasaki disease has only been limitedly reported in
Nigerian children and there exist only a few documented cases
in the region [12-16]. Literature review indicates that it is a
rare disease with diagnostic criteria provided by the American
Heart Association requiring the presence of fever that has lasted
for more than five days as a constant criterium [12]. In the
case described, the subject had fever for 9 days which was not
responsive to anti-malarial and antibiotics, even before referral to
our centre. Other principal clinical criteria described by the AHA
criteria for diagnosis of Kawasaki disease (at least four of which
must be present in addition to the fever to make a diagnosis of
Classic Kawasaki disease) that were all met in the case described
were non exudative bilateral conjunctivitis, fissuring of the lips,
swelling of hands and feet, perianal erythema and subsequent
desquamation and cervical lymphadenopathy of at least 1.5cm
[12].
Kawasaki disease occurs more commonly in children less than
five years of age commonly between 18 and 24 months and is one and a half times commoner in boys than girls [17], parameters
which are demographically consistent with the subject described.
The few cases that have been reported in Nigeria were also less
than five years old except a case of a 14 year old boy in Sokoto
[15]. The presentation of the disease can be non-specific with
evolution of clinical manifestations over time [7]. In this current
case however, most likely due to late presentation at our facility,
most of the physical manifestations were seen at presentation.
However, periungal desquamation of the fingers and toes only
developed five days into admission, which is consistent with the
course of disease in which periungual desquamation if present is
expected to develop 1-3 weeks after onset of illness [7].
No laboratory test is specific for Kawasaki disease but
leukocytosis is a common finding in the acute phase of the
illness which was seen in the index case [12]. A linear increase
in platelet count was recorded in this index case. This pattern
of increasing platelet count has been documented as the
outstanding laboratory marker of Kawasaki disease [7]. Severe
anaemia requiring transfusion has been linked to IVIG [18] but
this level of anaemia occurred before transfusion in this reported
case. Other characteristic findings like elevation of acute phase
reactants like erythrocyte sedimentation rate (ESR) was also
noticed in the index case which is almost a universal finding in
Kawasaki disease [12].
Coronary artery aneurysm is the gravest and most important
complication of Kawasaki disease [19]. Numerous predictive
parameters of cardiac affectation in Kawasaki disease have
been documented [19-21]. Some of these parameters that were
present in this subject are male gender, persistent fever, high
White Blood Cell count, low haemoglobin, elevated ESR and low
albumin. Despite the presence of these parameters however,
the index patient did not have echocardiography diagnosis of
heart involvement. Echocardiography was done at the acute and
sub-acute stages of the illness but was normal. Since coronary
aneurysm and risk of sudden death is highest at the sub-acute
stage of the illness [22,23].
Although echocardiography findings Independent predictors
of coronary artery disease identified by McCrindle and coresearchers
[19] are age less than one year, low albumin over a
five-week period, low IgM at presentation and duration of days
from onset of disease to treatment with IVIG. In this present case,
a single albumin measurement was done and it was marginally
low (3.3mg/dl). Also, the duration from onset of disease till time
of administration of IVIG in the index case was over 10 days
which is a positive predictive factor for development of heart
disease, warranting repeat echocardiography at six weeks’ postonset
of illness and if no abnormalities are found, one year later
[12]. Treatment of subjects with intravenous immunoglobulin
was not done in the few cases of Kawasaki disease reported in
Nigeria [13,14]. The reasons given for this were inaccessibility
and financial constraint. The index patient had IVIG at 2g/kg with
remarkable improvement in the clinical condition.
Conclusion
Kawasaki disease exists in among children in Lagos. There is
need for high index of suspicion. Early diagnosis and treatment
of cases will reduce morbidity that can occur from the disease.
Acknowledgement
The authors acknowledge the subject, his parent, and other
healthcare practitioners who participated in his care.
Ethical DisclosuresInformed and written consent were obtained from the parent of this reported case. The necessary institutional protocols on the publication of the data were duly followed. No experiments were performed on the patients for this article.
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