Dr Ira Shah
MD, DCH (Gold Medalist), FCPS, DNB

- A 23-months-old female child presented with high-grade fever since 4 days and cough since 3 days. On examination she had a strawberry tongue, conjunctival congestion, erythematous rash over the palms and soles and generalized lymphadenopathy (largest cervical gland = 2 x 2 cm) and tachycardia. A clinical diagnosis of Kawasaki's disease was considered.

On admission, her CRP was positive with normal WBC counts. Hemoglobin was 8.8 gm% and platelet count was 3,40,000/cumm, which subsequently gradually increased to 6,91,000/cumm in a span of 6 days. Her ESR at onset was 40 mm at the end of 1 hour that increased to 65 mm at the end of 1 hour by Day 6. Her 2D Echo was suggestive of dilatation or coronary arteries with right coronary artery of 2.8mm, left anterior descending artery of 2.5mm and left coronary artery of 2.8mm. No aneurysms with seen. She was immediately started on high dose aspirin and as soon as Intravenous immunoglobulin (IVIg) could be mobilized, she was treated with IVIg -2gm/kg on Day 6.

However, her platelet count increased upto 9,29,000/cumm and ESR to 115mm on Day 9 of illness. She was again retreated with IVIg (2gm/kg) and aspirin in high doses was continued. She was also treated with dipyridamole for the thrombocytosis to prevent thrombus formation. By Day17, her platelet count dropped to 4,61,000/cumm and her ESR also started showing a decreasing trend and was 95 mm at the end of one hour. Her repeat 2D Echo showed the same echo findings with no evidence of aneurysms. Patient was clinically asymptomatic and discharged on high dose aspirin.
,br> On follow up on Day30, her platelet counts were in the normal range (4,00,000/cumm) and her ESR was 15 mm at end of 1 hour on day. The patient was continued on low dose aspirin and is advised regular close follow up.

Thus, we wish to highlight the treatment failure of initial dose of IVIg and subsequent response to retreatment with IVIg in a child with Kawasaki’s disease.

Kawasaki's disease also known as mucocutaneous lymph node syndrome or Infantile polyarteritis was first reported in Japanese children after World War II. It is primarily seen in children younger than 5 years of age. The cause remains unknown but bacterial toxins and autoimmunity have been postulated in its etiogenesis. It is characterized by fever (lasting for at least 5 days), bilateral nonpurulent conjunctival injecton, strawberry tongue, erythema of hands or feet with desquamation usually beginning periungually, polymorphous truncal rash and cervical lymphadenopathy. Transient arthritis may also occur. Other manifestations may include diarrhea, vomiting, abdominal pain, hydrops of gall bladder, myositis, ulcerative stomatitis, aseptic meningitis, cranial or peripheral nerve palsies and hepatosplenomegaly.

Cardiac involvement is the most important manifestation of Kawasaki’s disease. 10-40 % of untreated children have evidence of coronary vasculitis within 2 weeks of illness as seen by coronary dilatation or aneurysm on 2D Echo that may lead to myocarditis and on long term early myocardial infarction.

Laboratory investigations show predominance of leukocytosis, thrombocytosis, elevated C-reactive proteins and high ESR.

Treatment consists of intravenous gamma globulin (IVIG) during the acute phase along with high dose aspirin (100mg/kg/day) to prevent coronary vascular damage. IVIg is given as a single dose (2gm/kg) over 10-12 hours. Low dose aspirin (5mg/kg/day) as single daily dose is advocated for continuation phase for 6-8 weeks after the active disease subsides for its anti-thrombotic effects and till coronary lesions resolve. For patients unresponsive to initial treatment with IVIg a retreatment with second dose of IVIg is recommended. It is found that patients with low Hb (Hb< 10gm/dl), leucocytosis and low albumin are at risk of failure of initial IVIg and may require retreatment with second dose of IVIg. For patients with active coronary thrombosis or peripheral artery ischemia, thrombolytic therapy with streptokinase may be required.