Dr.Shobika,Dr.SajithaNair ,DrSreya Nair DrSindhu Dr.C. Jayakumar,
AIMS Kochi
Eleven months old male ba presented with high grade intermittent fever of 18 days duration –associated with occasional cough of 2 weeks duration.
No history of associated fast breathing/ chest indrawing/ grunting/ cyanosis/ seizures.
Labs in the local hospital showed neutrophilic leucocytosis with elevated inflammatory markers.
Xray chest showed hilar prominence .He was treated with nebulised bronchodilators and multiple IV antibiotics(Ceftriaxone,amikacin,piptaz,teicoplanin,linezolid,fluconazole)
As the fever persisted and repeat x ray didn’t show resolution,the child was referred here
PAST HISTORY:H/o hospital admissions at 2 months,5 months,7 months of age – for LRTI and treated with iv antibiotics,nebulizations for 7 days.
At admission,child was febrile, tired looking with stable vitals.
On examination,Pallor and Small, discrete left cervical, axillary,lymphadneopathy present.
Respiratory system exam showed occasional crackles only
On systemic examination,P/A:Soft,Liver palpable 2 cm below right costal margin, liver span – 6cm, spleen not palpable, Bowel sounds present.
Differentials considered were
LRTI
Tuberculosis
Hematological malignancy
Kawasaki disease
Labs done showed Neutrophilic leucocytosis,thrombocytosis and increased inflammatory markers CRP(44.5mg/dl).
Gene expert for Tb -negative
USG abdomen – Normal
CT thorax – Normal lung parenchyma Mediastinal and axillary lymphadenopathy
ECHO done showed Giant coronary artery aneurysm with diffuse dilatation of left and right coronary system and its branches,hyperechogenicity noted over the vessel walls.LMCA-3.9mm, Z score – (+5.12)RCA -8.8mm,Z score – (+23.59)LAD – 6.02mm, Z score – (+419.3)LCX – 5mm,
So
Incomplete Kawasaki disease was made as diagnosis.
The child was treated with IVIG at 2g/kg/dose,Infliximab – 5mg/kg/dose,Aspirin and enoxaparin
After the infusions,there were no fever spikesRepeat
ECHO after IVIg infusions showed no improvement in Z scores with persistent coronary dilatation in RCA with new dilatation in LMCA,so he was started on Cyclosporine i/v/o of refractory Kawasaki disease.
After 5 days repeat ECHO showed reduction in Z score of LMCA and RCA And adviced to continue cyclosporine,aspirin and enoxaparin.
Kawasaki disease:
Also known as mucocutaneous lymph node syndrome.It is an acute, self-limited medium vessel vasculitis. It is the leading cause of acquired heart disease in developed countries.Typically affects children between age 6 months to 5 years of age with male preponderance.
Clinical phases of KD:
1)Acute febrile phase : lasts 7to 14 days.
Ends with the resolution of fever, is characterized conjunctival congestion, mouth and lip changes, swelling and erythema of the hands and feet, rash and cervical lymphadenopathy
2.Subacute phase :End of the fever to about day25. Desquamation of the fingers and toes , arthritis and arthralgia, and thrombocytosis.
3.Convalescent phase begins when clinical signs disappear and continues until the erythrocyte sedimentation rate becomes normal.Usually, six to eight weeks after the onset of illness .
Atypical or Incomplete Kawasaki disease:
Fever for at least five days and two or three principal features; coronary artery abnormalities on transthoracic echocardiography.
Supplemental laboratory criteria Anemia Cerebrospinal fluid pleocytosis Elevated C-reactive protein level and erythrocyte sedimentation rate Elevated liver enzyme levels
Hypoalbuminemia ≤ 3.0 g per dL (30 g per L)
Hyponatremia
Platelets ≥ 450 × 103 per µL (450 × 109 per L) after first week
Sterile pyuria (Urine WBC > 10/high-power field )
White blood cell count ≥ 15,000 per µL (15.0 × 109 per L)
Other clinical findings Cardiovascular: coronary artery abnormalities, decreased left ventricular function, mitral regurgitation, pericardial effusion ,
Gastrointestinal: abdominal pain, diarrhea, nausea, vomiting Musculoskeletal: arthritis, arthralgia .
Primary Treatment:Intravenous Immunoglobulin:IVIG 2 g/kg as a single infusion, usually given over 10 to 12 hours
Complications:-Fever-Coombs positive hemolytic anemia-Aseptic meningitis.Corticosteroids
Take home messages:
Early Recognition for Preventing Complications
Atypical Presentations require High index of Suspicion:
Perianal excoriation and periungual desquamation, highlight the need for a high index of suspicion for Kawasaki disease, even when typical symptoms may not be present.
Comprehensive Treatment Strategies:
Timely administration of
IV immunoglobulin (IVIg),
Infliximab,
IV methylprednisolone,
Cyclosporin,
Aspirin , dual antiplatelet therapy, and Enoxaparin Promising Results with Proper Care : Effective treatment can lead to good results, like the shrinking of the aneurysm in this case. It’s crucial to keep following up and sticking to the treatment plan