Welcome to Cross-Canada Paediatric - Respiratory Residency Rounds

Page 2/Case 02/01

She was transferred to a tertiary care pediatric center with a working diagnosis of "atypical Kawasaki disease".

After transfer she was also noted to have a mild myocarditis (base upon subtle ECG abnormalities but a normal echocardiogram), an erythematous facial and trunkal rash, subcutaneous nodules (which were biopsied and showed a panniculitis histology consistent with erythema nodosum and not a vasculitic pattern), and mild elevation of liver enzymes (AST252, ALT73).

Her initial bloodwork also showed a microcytic anemia (Hemoglobin of 95and she was subsequently proven to have thalassemia minor trait), a white blood count of 9.2, and a platelet count of 206. Her erythrocyte sedimentation rate was markedly elevated at 118.

She was further treated with intravenous gammaglobulins with no clinical response.

She was then treated with high dose intravenous corticosteroids for 3 days followed by oral prednisone 2mg/kg/day. With steroid therapy she finally defervesced. An extensive infectious work-up (blood, urine, and stool cultures, nasopharyngeal swab, parvovirus PCR, EBV PCR, HBSAg, Brucellosis ab) and rheumatology work-up (ANA, antidouble stranded DNA, anti smooth muscle, ANCA, antiRo, antiLa and anticardiolipin antibodies) were negative.

She was discharged home after three weeks in hospital with her steroid dose tapered to prednisone 1 mg/kg/day and ambulating in room air. She did not have respiratory symptoms noted by either hospital staff or parents at the time of hospital discharge.

After one month at home she developed cold-like symptoms, with fever, cough and dyspnea. She was readmitted to hospital and a repeat CXR showed the new changes you saw on the previous CXR. The child was started on intravenous antibiotics, oxygen and a chest consult was called.

This child was previously healthy with no prior hospitalizations. Her birth history was unremarkable.

She is the first born and only child of parents born in Pakistan. She is cared for at home by her mother. The family traveled to Pakistan for a visit more than a year before her illness. There is no known tuberculosis exposure. There is no family history of autoimmune or chronic lung disease.

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