Kawasaki Disease – A Case Overview

: Kawasaki disease, a medium vessel vasculitis, is seen commonly in children below 5 years of age. In developing countries Kawasaki disease is being increasingly reported, and is one of the emerging leading causes of acquired heart disease in children. Diagnosis of this case is often missed in this age group as it is not considering an upfront clinical possibility in a febrile child which often leads to delay in diagnosis and initiation of therapy. A 2 years old male child got admitted on 24/08/2022 under PICU, AIIMS, Raipur with chief complaints of cough and cold for 7 days, fever for 5 days, redness of eyes for 2 days, 3 days back, rash for 3 days, vomiting after meals for 2 days, fast breathing for 1 day, redness of palm and soles for 1 day, neck swelling for 1 day. The patient underwent basic investigations to rule out the diagnosis and differential diagnosis and was treated with oxygen support, IVIg, aspirin, antibiotics and supportive management. There was significant improvement in the child’s condition and was discharged with follow up advise.


Introduction
Kawasaki disease, also called mucocutaneous lymph node syndrome is an acute systematic vasculitis which causes inflammation (i.e., swelling and redness) in the walls of small and medium sized blood vessels in the body; swelling in glands (lymph nodes), mucous membranes of mouth, nose, eyes and throat.This condition most often affects children of less than 5 years of age.Children with this disorder will show signs and symptoms of high fever, rash over body and extremities, redness and swelling of hands and feet, skin peeling in hands and foots in the second and third weeks, and red eyes and tongue.Kawasaki disease is usually treatable, and most children recover without serious problems if they receive treatment within 10 days of onset.Early treatment to prevent cardiovascular complication is important.
Kawasaki disease is the leading cause of acquired heart disease in children.In as many as 25 percent of children with Kawasaki disease, the heart becomes affected without proper treatment.Kawasaki disease commonly leads in inflammation of coronary arteries.

Case History Informant: Child's Mother
A 2 years old developmentally normal male child got admitted on 24/08/2022 under PICU, AIIMS, Raipur with chief complaints of cough and cold for 7 days, fever for 5 days, redness of eyes for 2 days, 3 days

General Examination
• Skull and spine normal.

Course of Illness and Management
2 years old developmentally normal, inadequately immunized third order male child born out of nonconsanguineous marriage diagnosed to have cyanotic congenital heart disease 1 month back presented with acute febrile illness associated with rash, significant lymphadenopathy and respiratory distress.

Course in PICU
Child was received in a state of irritability with tachypnea and cyanosis noted on initial assessment.Primary survey revealed tachypnea with increased efforts for which oxygen support via nasal prongs was provided initially and later shifted to HFNC.The child had following issues: 1. Acute Febrile Illness: Child had low grade fever for more than 5 days with red, cracking lips with erythematous palm and soles, cervical lymphadenopathy with maculopapular rash and congested and hypertrophied tonsils V (grade 3).Possibilities of diseases like Measles, EBV, Kaswasaki were ruled out.Since, age group was not appropriate for EBV; Measles and Kawasaki disease were kept as close differentials.Points against measles were appearance character of the rash, absence of enanthem and severe prodromal cough.criteria's in favour of Kawasaki were met along with raised acute phase reactants and ESR.So, IVIg was given on admission following which significant improvement was noted in form of disappearance of palm and sole erythema, rash and red, cracked lips.Fever resolved and child remained afebrile for 48 hours.• Recurrence of fever after 48 h occurred, so two possibilities were kept.Hospital acquired infection or IVIg resistant Kawasaki disease.So, repeat dose of IVIg @ 1 g/kg was given and empirically Inj.Piptaz had been started.• The blood culture on admission grew enterococcus faecalis sensitive to ampicillin so antibiotic was changed to inj.ampicillin.The child responded to the treatment and been afebrile for more than 72 hours in ICU.

Respiratory Distress:
Child had increased work of breathing for which nasal prongs support was provided and later shifted to HFNC and child was weaned off to nasal prongs in subsequent days.

Pulmonary Hypertension:
Child had been started on Sildenafil and Bosentan in view of severe PAH which was continued but on day 3 of admission child had hypotension episodes so sildenafil was withheld and was re-started after 7 days at low dose.There was no PAH crisis episodes during PICU stay.CTVS opinion was taken but operability was told be limited and advised to continue on medical management for PAH.

Therapeutic Interventions
The child received combination of therapeutic management in view of underlying condition, supplementation and antibiotics in view of blood culture report.The child has been treated with the following medications:

Discharge and Follow Up
Child was properly assessed every day for prognosis and review of treatment.Childs condition had a marked improvement and underlying symptoms reduced and need for oxygen reduced considerably over the course of management.Parents were satisfied with the management from the health care team.The patient was discharged after explaining the danger signs and to continue medication -Tab Aspirin 75 mg 1/2 tab Po BD, Tab Bosentan 8 mg PO BD, Tab Sildenafil (1 tab = 50 mg) 20 ml DW give 1 ml, Furoped drops (10 mg/ml) 0.8 ml, Zinc oxide ointment L/A TDS, Syrup Vit D (400 IU/ml) 1 ml PO OD, Syrup IFA (15 ml = 38 ml iron) 5 ml PO BD, Syrup PCM (125 mg / 5 ml) 5 ml PO SOS, Follow up in CTVS OPD, Review SOS in pediatric OPD.The parents were counselled about the need for nutritional supplementation as the child is malnourished.
The child was referred to dietician in view of malnourished and has underlying heart disease, and was advised about the steps to improve the intake of nutritious food suitable to the economic conditions and dietary patterns of the patient.The child was advised to come back after 10 days of discharge for a follow up on the improvement in-patient's health.

Discussion
After collecting history based on the inputs provided by the informants, presenting chief clinical signs and symptoms and conducting requisite tests on the child, diagnosis has been made as Kawasaki disease with blood stream infection (enterococcus).The child was given with appropriate treatment including oxygen therapy, IVIg, aspirin and supportive management and helped the child to get back health on track.Parents were advised and created the sense of awareness on need for long term follow up, medications, proper care of hygiene, healthy lifestyle, diet patterns etc. providing all the information and symptoms of Kawasaki disease so that, similar cases don't occur in their immediate family.A long term follow is important in such case as the child is having underlying congenital heart disease and Kawasaki disease will impose more complications on heart and heart vessels.

Conclusion
Kawasaki disease is clinically stated as systemic vasculitis of mainly medium sized arteries.While Kawasaki disease remains as poorly understood disorder, delayed and missed diagnosis can result in severe complications in children, while timely diagnosis and prompt treatment initiation and long term follow up could immensely result in good prognosis and also lowers the complications associated with Kawasaki disease.Awareness of the diseases should be created by educating the parents/public at large regarding the symptoms, major causes, importance of early diagnosis and treatment to prevent long term complications, follow up requisites and how these can be countered.Such an widespread awareness will help in early diagnosis/treatment, thereby bringing the situation under control without causing major damage to the children physically, mentally and economically; not just for the child but for their entire family and society.

Statement of Human and Animal Rights
All procedures performed in human participants were in accordance with the ethical standards of the institutional research committee.Rights and privacy of the patient maintained.This article does not contain any studies with animals performed by any of the authors.
RSInspection: B/L equal chest rise.Palpation: B/L chest expansion equal.Percussion: Resonant note.Auscultation: B/L equal air entry.CVS S1 normally heard.Loud P2.No murmur audible.P/A Soft and non-tender.No organomegaly.CNS E4V5M6, Cranial nerve normal, Tone normal in all 4 limbs, Power > 3/5 in all limbs, Reflexes normal in upper limb and lower limb, B/L plantar flexor.Meningeal signs absent, Cerebellar signs absent, Autonomic system normal, Skull/Spine normal

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No dysmorphism.•Genitalia:Normal•Skin: Maculopapular rashes over anterior and posterior aspect of trunk, bilateral upper and lower limbs sparing palm and sole.•Erythematouspalm and soles.•Perinealdesquamationpresent.•Bilateralsignificant cervical lymphadenopathy present.Throat congested with grade III tonsils.Tongue congested.Posterior pharyngeal wall could not be seen as child is irritable.• Lip cracking present.Angular stomatitis present.