Kawasaki disease at British Columbia's Children's Hospital.

OBJECTIVES: To describe the clinical features, diagnosis, treatment and outcome of children with Kawasaki disease (KD) treated at a large tertiary care Canadian paediatric hospital and to try to identify correlations between clinical features and the development of coronary artery abnormalities. METHODS: The charts of 176 patients diagnosed with typical, atypical or incomplete KD between 1992 and 2000 at British Columbia's Children's Hospital were reviewed. RESULTS: The male to female ratio was 1.8:1. The median age was 2.5 years (range two months to 14 years), with 8% nine years or older (42% Caucasian, 43% Asian). Cases occurred steadily throughout the year. One hundred two (58%) patients had typical, 18 (10%) patients had atypical and 56 (32%) patients had incomplete KD. The median time from fever onset to first intravenous immunoglobulin (IVIG) was seven days (range two to 49 days), and treatment began within 10 days of fever onset in 134 (76%) patients. All patients received one or more doses of 2 g/kg IVIG. Forty-two (24%) patients received a second dose for nonresponsiveness, of whom 10 (6%) remained nonresponsive. Eight (5%) patients received intravenous methylprednisolone. Forty-eight (27%) patients developed coronary artery abnormalities, with 10 (6%) echogenic abnormalities, 25 (14%) dilatations and 13 (7%) aneurysms (seven giant). No patient with a normal echocardiogram at four to eight weeks developed an abnormality on subsequent study. Fourteen (8%) patients had persistent abnormalities at last follow-up (median 447 days, range 62 to 3272 days): seven dilations and seven aneurysms (six giant). Five of 13 children (39%) who developed aneurysms failed to meet diagnostic criteria for typical KD, and three of those five aneurysms were present at less than one year after diagnosis. Four of eight (50%) patients receiving intravenous methyl-prednisolone for IVIG nonresponsiveness had or developed aneurysms. One patient died. CONCLUSION: Some children diagnosed with KD who fail to meet the diagnostic description develop coronary artery abnormalities. There is a need for a more accurate means of diagnosis to more appropriately use IVIG, an expensive and increasingly scarce resource. The role of corticosteroids remains unclear and a randomized controlled clinical trial to determine their role is needed.

Rhabdomyolysis, acute renal failure, and compartment syndrome in a child with parainfluenza type 1 infection.

We present a case of para-influenza type 1 induced rhabdomyolysis, acute renal failure, and extensive compartment syndrome in a 6-year-old previously well child. Fasciotomies and subsequent skin grafting of both lower extremities and a prolonged course of hemodialysis led to a complete clinical and biochemical recovery.

Corticosteroid treatment of refractory Kawasaki disease.

OBJECTIVE: To review the indications for corticosteroids in patients with Kawasaki disease (KD) treated by pediatric rheumatologists in Canada and to determine their efficacy on fever in patients with refractory KD. METHODS: All practicing pediatric rheumatologists in Canada identified KD patients treated with corticosteroids and completed a standard data form that included demographics, clinical and laboratory features, imaging studies, and therapeutic interventions, by chart review. RESULTS: Thirty-two patients with KD (14 female; 18 male: mean age 4.6 years) were treated with corticosteroids. Corticosteroids were used in 26 patients (81%) for persistent fever despite treatment with intravenous immunoglobulin (IVIG) (refractory KD), 5 patients (19%) for congestive heart failure, and 1 patient for persistent acute phase symptoms other than fever. The 26 patients with refractory KD are the primary subject of this report. Twenty-two patients (85%) had rapid, sustained resolution of fever after corticosteroids. There were no serious reported adverse effects. Eight patients (31%) treated with corticosteroids developed coronary artery (CA) aneurysms and 9 (35%) developed CA dilatations without aneurysms. Of those who developed CA aneurysm, 4 had aneurysms detected prior to IV methylprednisolone (MP) on echocardiograms performed on days 6-27 (mean day 13) of illness. The remaining 4 patients had CA aneurysm detected after IVMP therapy, on echocardiograms performed on days 13-49 (mean day 23) of illness, 1-25 days (mean 9 days) after IVMP. In patients with one year or more of followup, 46% had resolution of CA abnormalities. CONCLUSION: Corticosteroids are effective in the treatment of fever in most patients with IVIG-refractory KD. A multicenter prospective study is needed to determine the effect of corticosteroids on CA outcome in patients with refractory KD.

Diagnosing autoimmune hepatitis in children: is the International Autoimmune Hepatitis Group scoring system useful?

BACKGROUND & AIMS: In 1999, the International Autoimmune Hepatitis Group (IAIHG) modified a scoring system to differentiate adult patients with definite or probable autoimmune hepatitis (AIH) from those with other forms of chronic liver disease. We assessed the use of the scoring system in children. METHODS: Twenty-eight pediatric patients with AIH and/or sclerosing cholangitis were reviewed. Clinical, laboratory, and histologic data were collected to score patients both before and after standard treatment. RESULTS: There were 8 boys and 20 girls. The median age at diagnosis was 11 years (range, 2-16 years). Twenty-one of 28 children were diagnosed with AIH, 4 as isolated primary sclerosing cholangitis (PSC), and 3 as overlap syndrome. At presentation, 18 of 21 (86%) with AIH scored as definite AIH and 3 of 21 (14%) scored as probable. No patient clinically diagnosed as AIH scored as other. Seven of 28 patients had proven PSC. All patients with isolated PSC scored as other. The 3 with overlap syndrome scored as definite AIH. When the gamma-glutamyltranspeptidase (GGT) ratio was substituted for the alkaline phosphatase (ALP) ratio, 5 patients were reclassified from definite to probable AIH. Four of these 5 had an incomplete response to therapy, and 2 of 4 have confirmed overlap syndrome. CONCLUSIONS: The IAIHG scoring system has a use in children. Patients who fall into the other category should have cholangiographic imaging. Using the GGT ratio instead of the ALP ratio in the IAIHG score may improve the specificity for children, identifying those likely to have biliary disease. When GGT is used, patients classified as needing probable pretreatment should be considered for biliary imaging.