Cardiac sequelae of Kawasaki disease are an important cause of ischemic heart disease in young adults. The possibility of early progression of atherosclerosis following Kawasaki disease is therefore of great concern. We examined whether preclinical atherosclerotic changes are seen in patients with a history of Kawasaki disease, and whether these changes appear in all or in only a proportion of patients. Sixty-five patients with a history of Kawasaki disease, aged 13.1 +/- 2.1 years, and 20 aged-matched controls participated in the study. All subjects underwent flow-mediated dilatation (FMD) of the brachial artery and analysis of carotid artery size and pulse-wave transmission. Patients were classified into four groups depending on the severity of the maximum coronary artery lesion: group 0 (normal), group 1 (mild), group 2 (moderate), and group 3 (severe). There was no statistical difference in the carotid artery analyses between the four groups. FMD (mean +/- SD) was significantly lower in groups 2 and 3 than in groups 0 and 1 and the control group (group 0, 19.4 +/- 3.9%; group 1, 19.5 +/-4.1%; group 2, 8.9 +/- 2.8%; group 3, 4.2 +/- 1.5%; control group, 18.8 +/- 2.8%; p < 0.0001). There was a significantly negative correlation between the severity of the coronary artery lesion and FMD (p < 0.0001 for both). Endothelial dysfunction was revealed by FMD in patients with persistent coronary artery lesions subsequent to Kawasaki disease. Preclinical atherosclerosis may be present only in patients with coronary aneurysms.
Two cases of cecal volvulus in children with mental disability are described. Case 1: a 3-year-old girl with trisomy 18 was admitted with abdominal pain and vomiting. She had received left lateral segmentectomy 6 months earlier because of hepatoblastoma. Release of the cecal volvulus followed by the fixation of the cecum and ascending colon to the right retroperitonium was performed. Case 2: a 15-month-old boy with Cornelia de Lange syndrome who had undergone a standard Nissen's fundoplication. On the sixth postoperative day, progressive abdominal distention developed. Abdominal color Doppler ultrasonography from the right rear side revealed a clockwise-twisted ileocecal artery and vein and a dilated colon with tapering configuration. On the ninth postoperative day, emergent release of the cecal volvulus followed by ascending colostomy through a perforation site was performed. Approximately 40 children with cecal volvulus have so far been reported, of whom 13 are mentally disabled. We speculated that in the cases reported here, distention of the intestine accompanying the mental disability and the previous surgery contributed to the development of cecal volvulus in addition to the prerequisite of abnormal mobility of the cecum. Pediatric surgeons should consider the cecal volvulus as a cause of intestinal obstruction in mentally disabled children.
Patients with Kawasaki disease (KD) who did not respond to the initial IVIG are known to have higher risk for developing coronary arterial lesions (CALs). Our aim is to clarify whether patients with initial IVIG resistant KD may benefit from methylprednisolone pulse therapy (MPT) in comparison with re- treatment of IVIG (2nd IVIG). A total of 237 patients (median age: 2 years 2 months; range 1 months-10 years) with KD were initially treated with IVIG (2 g/kg). Among them, 41 patients (22 %) were assessed as IVIG resistance: these patients were allocated to either group A receiving MPT (n = 14) or group B receiving the 2nd IVIG (n = 27). Patients with resistant to the additional therapy (MPT or 2nd IVIG) were received second IVIG (group A) or MPT (group B). Changes in leukocyte count, C-reactive protein and albumin before and after an additional therapy were significantly greater in group A than those in group B. However, the prevalence of CALs did not differ between the groups (36 % in group A and 26 % in group B, p > 0.05). There was no significant difference in the medical cost between the groups (median cost: 92,032 JPY in group A and 97,331 JPY in group B). MPT does not reduce the risk of development to CAL and does not seem to be beneficial as single agent therapy for IVIG resistant KD.
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