Background: The aim of this study was to identify risk factors for immunoglobulin resistance, including clinical symptoms such as arthritis and the pH of intravenous immunoglobulin. Methods: The data of children with Kawasaki disease who had received immunoglobulin were evaluated. Data regarding the brand of immunoglobulin administered were abstracted from the pharmacy records. Results: Eighty consecutive children with Kawasaki disease were evaluated (Mdn age = 28 months, 66% male). The prevalence of immunoglobulin resistance was 30%. Arthritis was a presenting symptom in the acute phase of Kawasaki disease in 8% (6/80, all male) and was seen in significant association with immunoglobulin resistance in comparison to those without arthritis (16.7% vs. 0.2%, p = 0.008). Next, the immunoglobulin brand types were divided into two groups: the relatively high pH group (n = 16), including Carimune (pH 6.6 -0.2), and the low pH group (n = 63), including Gamunex (pH 4-4.5) or Privigen (pH 4.6-5). Overall, no significant difference in immunoglobulin responsiveness was found between the low pH and the high pH groups (73% vs. 56%, p = 0.193), although the low pH group showed a trend toward a larger decrease in erythrocyte sedimentation rate ( p = 0.048), lower steroid use ( p = 0.054), and lower coronary involvement ( p = 0.08) than those in the high pH group. Conclusions: Children presenting with arthritis in the acute phase of Kawasaki disease may be at risk for immunoglobulin resistance.

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Presence of systemic venous anomalies including a persistent left superior vena cava to a dilated coronary and interrupted inferior vena cava with azygous continuation are extremely rare in absence of heterotaxia or cardiosplenic syndrome. For this reason, the diagnosis of extracardiac venous anomalies can often be missed in absence of a heterotaxia as was the case in our patient whose prior fetal echocardiogram and the first postnatal echocardiogram read by other pediatric cardiologists failed to detect these anomalies. We report these systemic venous anomalies in a fetus in the absence of heterotaxia and further describe the echocardiographic findings helpful in their detection in-utero.