Dr Ira Shah
M.D, DNB, DCH (Gold Medalist), FCPS

A 4 years old female child presented with fever since 4 days, generalized edema with oliguria since 2 days and vomiting and rash since 1 day. She had no history of jaundice or similar episodes in the past. On examination, the child was febrile and in shock. (Peripheral pulses were weak, B.P. = 90/76 mm of Hg). She had tachycardia with a heart rate of 134 /min. Anasarca was present. There was a petechial rash present over trunk, hands and legs. Discrete cervical lymphadenopathy (1.5 x 1 cm - largest) was present and lymphnodes were mobile and non-tender. Oral cheilosis was present. There was no strawberry tongue or conjunctival congestion. On systemic examination, she had a tender hepatosplenomegaly. A differential diagnosis of sepsis, viral hemorrhagic fever and Kawasaki's disease was considered. Her CBC revealed hemoglobin of 8.7 gm/dl and WBC count of 10,100 cells/cu mm (72% polymorphs, 86% lymphocytes and 1% monocyte) with a normal platelet count of 4,30,000 cells/cu mm. Her peripheral smear was normal and ESR was 4 mm at end of 1 hour. Stool showed presence of occult blood though her prothrombin time and partial Thromboplastin time were normal. Her SGPT was deranged (155 IU/L) and she had hypoalbuminemia (Total Proteins = 4.8 gm%, S. Albumin = 2 gm %). Her renal profile, electrolytes were normal. Blood culture showed no growth. In view of the suspicion of viral hemorrhagic fever her Dengue IgM and leptospira. IgM & IgG by ELISA were done which were negative. Patient was treated with IV ceftriaxone and ionotropic support. In view of clinical suspicion of Kawasaki's disease with laboratory parameter suggestive of hypoalbuminemia though there was no thrombocytosis or elevated ESR, an echocardiography with color doppler of the heart was done which showed mild pericardial effusion (0.73 cm posteriorly) with dilated coronary arteries (Right main coronary artery = 2.8 mm, left anterior descending coronary artery = 2.2 mm). Thus the child was diagnosed as Kawasaki's disease with shock. She was treated with intravenous immunoglobulin (2 gm/kg) to which the patient responded and ionotropic supports were omitted after 5 days. Oliguria, hepatitis and edema also resolved. Her CBC was repeated on Day 5 of admission which showed a drop in the hemoglobin (6.7 gm/dl) with thrombocytosis (Platelet count = 4,77,000 cells/cumm) and high ESR (135 mm at end of 1 hour). She was started on high dose Aspirin for 14 days and subsequently shifted to low dose Aspirin and advised regular follow up.

Thus, we have a child who did not fulfill all the diagnostic criteria of Kawasaki's disease and was in shock with only hypoalbuminemia as one of the suggestive laboratory parameter who had evidence of coronary dilatation on echocardiography and thrombocytosis and raised ESR came much later in the disease process.

Kawasaki disease, formerly known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa is an acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967. Asians are at the highest risk. Cause of the illness remains unknown but an infectious origin is strongly suspected.

Kawasaki disease causes a severe vasculitis of all blood vessels but predominantly affects the medium-sized arteries with predilection for the coronary arteries. Elevated levels of all serum immunoglobulins are present suggestive of a vigorous antibody response. Patients present with high-spiking fever (up to 104 F or higher) that is remittent and unresponsive to antibiotics hasting for 1-4 weeks. Prolonged fever is a risk factor for development of coronary artery disease. Other features include bilateral bulbar conjunctival injection; erythema of the oral and pharyngeal mucosa with "strawberry" tongue and dry-cracked lips; erythema and swelling of hands and feet; rash that is primarily truncal and polymorphous with cervical adenopathy > 1.5 cm. Periungual desquamation of the fingers and toes begins 1-3 weeks after the onset of illness.

Other features include extreme irritability, aseptic meningitis, diarrhea, hepatitis, hydrops of gall bladder, urethritis, meatitis, otitis media and arthritis. Cardiac involvement in form of myocarditis, pericarditis, coronary artery aneurysm, valvular regurgitation and systemic artery aneurysms occur.

Poor prognostic markers are male gender, age younger than 1 year, prolonged fever, recurrent fever after an afebrile period, low hemoglobin or platelet levels, high neutrophil and band counts and low albumin and serum IgG levels.

Diagnosis is mainly clinical. No specific diagnostic test exists but certain laboratory findings are characteristic such as elevated white cell counts (predominantly neutrophils), elevated ESR & CRP and other acute phase reactants, thrombocytosis (in 2nd-3rd week of illness) and mild elevation of hepatic transaminases.

Treatment for acute stage consists of IVIg (2gm/kg) with high dose aspirin (100 mg/kg/d every 6 hourly x 14 days). IVIg reduces the prevalence of coronary disease by 22-23%. Aspirin is continued in anti-thrombotic doses (3-5 mg/kg/day) for 6-8 weeks till ESR has normalized and in patients in whom echocardiography is normal. Patients with aneurysm need to continue aspirin indefinitely. Patients with large aneurysms may require warfarin or dipyridamole in addition.

Prognosis is good for patients who do not develop coronary artery disease. Overall 50% of coronary artery aneurysm resolves echocardiographically by 1-2 years after illness.