High Sensitivity C Reactive Protein (hs-CRP) in Adolescent and Young Adult Patients with History of Kawasaki Disease.

BACKGROUND: For children with a history of Kawasaki disease (KD), low grade inflammation was generally reported to be associated with persistent coronary artery lesions (CAL). However, this association has not been clearly demonstrated to hold true in KD adolescents and young adults (10-25 years of age). METHODS: We enrolled 104 subjects into our study, who were separated into the following 3 groups and controls: 1): 22 KD patients with angiography-confirmed CAL which persisted for an average of 12.5 years after onset of KD; 2) 38 KD patients with regressed aneurysms; 3) 44 KD patients without any coronary complications from the disease onset; and 4) 31 age-matched (18.7 ± 1.88 years old) healthy controls. Plasma levels of high-sensitivity C reactive protein (hs-CRP) were measured for all participants. RESULTS: Plasma levels of hs-CRP were significantly higher in KD patients than in the controls, regardless of their coronary severity. However, there was no significant difference in hs-CRP levels among KD patients with different severities of CAL. Of the candidate risk factors of elevated hs-CRP such as body mass index, gender, coronary severity, and levels of high-density lipoprotein-cholesterol, linear regression analysis showed the only independent predictor of hs-CRP levels was BMI (ß = 0.306, p = 0.01), rather than patient grouping (p = 0.091). CONCLUSIONS: Our study found that levels of hs-CRP are significantly higher in adolescent and young adult patients with a history of KD, compared with age-matched controls. Low grade inflammation may play a minor role when KD patients enter into adulthood. body mass index (BMI), rather than coronary severity, was independently associated with the elevation of hs-CRP levels, one of biomarkers for further cardiovascular event. Therefore, ongoing control and management of BMI may be one of beneficial strategies that can be employed to help avoid elevation of hs-CRP levels in KD patients. KEY WORDS: Adolescents; High sensitivity-C reactive protein; Kawasaki disease; Young adult.

Misalignment of lung vessels and alveolar capillary dysplasia: a case report with autopsy.

Misalignment of lung vessels (MLV) with or without alveolar capillary dysplasia (ACD) is a rare cause of idiopathic persistent pulmonary hypertension of the neonate. This report describes a full-term infant with severe and intractable pulmonary hypertension. The patient's condition progressively deteriorated despite high-frequency oscillatory ventilation, infusion of magnesium sulfate, dopamine, and dobutamine to control blood pressure, and nitric oxide inhalation therapy. The infant died at 5 days of age. The diagnosis of MLV with ACD was established by autopsy. Histopathologic analysis revealed a failure of formation and an ingrowth of alveolar capillaries, thickening of the alveolar walls, poor contact of capillaries with alveolar epithelium, small intra-acinar muscularized arterioles, and anomalous pulmonary veins within bronchovascular bundles. The low rate of diagnosis of MLV with or without ACD may be because of the early high mortality rate or patchy involvement in some cases. Increasing awareness of this clinical entity may prevent the use of costly, invasive, and probably ineffective procedures. Short-term improvement after inhalation of nitric oxide does not lead to long-term survival but merely provides time for potential lung transplantation.

Clinical Implication of the C Allele of the ITPKC Gene SNP rs28493229 in Kawasaki Disease: Association With Disease Susceptibility and BCG Scar Reactivation.

BACKGROUND: a functional single nucleotide polymorphism (SNP) (rs28493229) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene has been linked to the susceptibility to Kawasaki disease (KD). The implication remains unclear. SUBJECTS AND METHODS: genotyping for the ITPKC polymorphism was conducted on 280 unrelated Taiwanese children with KD and 492 healthy ethnically and gender-matched controls. The clinical manifestations and laboratory data were systemically collected. RESULTS: the GC and CC genotypes of ITPKC gene SNP rs28493229 were overrepresented in KD patients (GG:GC:CC was 236:43:1, C allele frequency: 8.04%) than those in the controls (GG:GC:CC was 454:37:1, C allele frequency: 3.96%; OR: 2.23, P = 0.001). In KD patients, those with GC or CC genotypes of SNP rs28493229 (19/44) were more likely to have reactivation at the Bacille Calmette-Guérin (BCG) inoculation site than those with GG genotypes (66/236; OR: 1.96, P = 0.044). Such association was particularly strong in patients aged <20 months (OR: 3.26, P = 0.017). The other clinical manifestations were not related to this SNP. There were 160 (57.1%) patients with coronary arterial lesions. The development and the severity of coronary arterial lesion were also not associated with this SNP. Comparison between patients with and without BCG reactivation revealed only one difference: patients with reactivation were younger. CONCLUSION: in a cohort from a population with the world's third highest incidence of KD, we demonstrated that the C-allele of ITPKC SNP rs28493229 is associated with KD susceptibility and BCG scar reactivation during the acute phase, although its frequency is lower than that in the Japanese cohort (22.6%), suggesting this SNP contributes to KD susceptibility through induced hyperimmune function reflected in the BCG reactivation.

A patient with PHACE syndrome with marked ipsilateral cerebral atrophy.

The association of posterior fossa malformation, facial cavernous hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities (PHACE syndrome) represents a rare congenital anomaly with a broad spectrum of clinical manifestations and female predominance. We herein report on a girl who manifested the typical clinical features of PHACE syndrome, unusually associated with severe ipsilateral cerebral atrophy and hemiplegia. She received surgical aortoplasty, local steroid injection and laser therapy for the hemangioma, and intense physical therapy soon after diagnosis. The etiology of PHACE syndrome remains unclear, and its clinical spectrum is broad. The current case suggests that the spectrum of PHACE syndrome should be further expanded to include other forms of cerebral disorder.

Results of echocardiographic screening in 10,000 newborns.

BACKGROUND: In response to increasing demand for a more sophisticated newborn baby health examination, we designed and performed echocardiographic screenings on a voluntary and self-paid basis. METHODS: From 1997 to 2005, 10,000 newborn babies had received the echocardiographic examination on the next day after birth. The echocardiographic examinations were performed by a single sonographer. RESULTS: As well as physically patent ductus arteriosus (PDA) (2420 cases), and patent foramen ovale (PFO) (5813 cases), we also found atrial septal defect (ASD) (161 cases), muscular-type ventricular septal defect (VSD) (123 cases), perimembranous type VSD (58 cases), coarctation of the aorta (COA) (1 case), pulmonic stenosis (PS) (1 case), peripheral pulmonic stenosis (PPS) (15 cases), common atrioventricular canal (CAVC) (1 case), transposition of the great arteries (TGA) (3 cases), double outlets of the right ventricle (DORV) (2 cases), tetralogy of Fallot (TF) (2 cases), hypertrophic cardiomyopathy (2 cases), dextrocardia (2 cases) and cardiac tumor (2 cases). CONCLUSIONS: Newborn echocardiographic screening enables pediatricians to detect abnormal cardiac findings early and accurately, especially heart diseases without murmur such as COA, ASD, CAVC, dextrocardia, small muscular VSD, small PDA, hypertrophic cardiomyopathy and cardiac tumor. In addition, cardiopulmonary information obtained from the echocardiographic examination can be useful for neonatal care providers.

Radiofrequency catheter ablation of idiopathic left ventricular tachycardia in a child.

Ventricular tachycardia occurring in apparently normal heart is rare in children. A 9-year-old boy presented with recurrent palpitations and syncope was found to have idiopathic ventricular tachycardia with a right bundle branch block morphology and left axis deviation. Pace mapping and activation mapping were used to localize the site of ventricular tachycardia origin. Radiofrequency catheter ablation successfully abolished this arrhythmia at a site of the midportion of the inferoseptal region of the left ventricle. This boy was free of tachycardia over follow-up of 2 years.

Coronary arteriovenous fistula in pediatric patients: a 17-year institutional experience.

BACKGROUND AND PURPOSE: Coronary arteriovenous fistula (CAVF) is a rare congenital anomaly in pediatric patients. Its clinical manifestations vary considerably and its long-term outcome is not fully understood. This study sought to determine the natural history and long-term outcome in CAVF patients treated over a 17-year period at Cathay General Hospital in Taipei. MATERIALS AND METHODS: The medical records of all 10 pediatric patients (five boys and five girls aged between 15 days and 15 years) with CAVF treated from 1983 through 2000 at our hospital were reviewed. Data collected included symptoms and signs, the findings of electrocardiography, echocardiography, catheterization, and angiography, and surgical results. RESULTS: CAVF was diagnosed on the basis of color Doppler echocardiography in eight patients and by cardiac catheterization and angiography in two. Congestive heart failure was found in four patients and both myocardial ischemia and infarction were found in two patients. Most of the affected coronary arteries were tortuous and dilated with a mean diameter of 12.6 mm (range 5-40 mm). Under cardiopulmonary bypass, fistulous terminations were sutured in seven patients, three of whom were found to have multiple fistulous openings. Postoperative follow-up examinations revealed that all of the affected coronary arteries and fistulas remained dilated and tortuous, except in one patient. Two patients who had distal CAVF developed coronary thrombus, calcification, and ventricular aneurysm at 2 and 10 years after operation, respectively. Another patient developed fistulous recanalization 7 years after operation, but this abnormal channel had disappeared again 3 years after recanalization. One patient developed an iatrogenic CAVF 8 years after surgical repair of tetralogy of Fallot. CONCLUSIONS: Unlike adults, pediatric patients with CAVF tend to be symptomatic. Ligation of the fistulous termination alone does not reduce the size of the fistula. Our findings indicate that long-term follow-up is essential due to the possibility of postoperative recanalization, persistent dilation of the coronary artery and ostium, thrombus formation, calcification, and myocardial infarction. In addition, postoperative antiplatelet therapy is recommended, especially in patients with distal CAVF and abnormally dilated coronary arteries.