Incidence of febrile seizure in patients with Down syndrome.

BACKGROUND: It is unclear whether the incidence of febrile seizure (FS) in children with Down syndrome (DS) is higher or lower than in the general population. In this study, we investigated the incidence of FS in DS patients using mailed questionnaires. METHODS: The questionnaires were distributed to parents or caregivers of DS patients attending Osaka Medical College Hospital and six other facilities. The questionnaires were returned by mail from February 2012 to September 2013 from 323 families of DS patients (176 male, 147 female; age range, 3 months-47 years; median age, 5.0 years). To assess the incidence of FS in DS, we performed the following two analyses: (i) we calculated the incidence of FS among DS patients between the ages of 4 and 20 years (n = 199; 113 male, 86 female), and (ii) we extracted families with both DS and healthy siblings between the ages of 4 and 20 years (n = 150; 77 male, 73 female) and compared the incidence of FS in these sibling groups. RESULTS: Five DS patients had a past history of FS. The incidence of FS in DS was 2.5%. The incidence of FS was significantly lower in DS patients compared with healthy siblings (P < 0.003; OR, 0.14). CONCLUSION: The incidence of FS is lower in DS patients than in the general population.

Spontaneous closure of muscular trabecular ventricular septal defect: comparison of defect positions.

AIM: To evaluate the timing and frequency of spontaneous closure of the muscular trabecular ventricular septal defect (VSD). METHODS: We performed a historical cohort study for which 150 patients <3 months of age (median age, 9 days) diagnosed as having a muscular trabecular VSD were selected. Median age at latest follow-up was 2.8 years. Another 32 patients diagnosed after 3 months of age were also reviewed. Using colour Doppler, defects were classified into three groups: anterior, apical and midventricular. RESULTS: Spontaneous closure occurred in 126 patients (84%): anterior, 36 of 47 (83%); apical, 26 of 31 (84%); and midventricular, 64 of 72 (89%). Multivariate analyses showed a lower frequency of spontaneous closure for patients of age of ≥ 20 days at initial echocardiography [hazard ratio 0.60, 95% confidence interval (CI) 0.39-0.89] and for anterior and apical muscular trabecular VSD (hazard ratio 0.66, 95% CI 0.47-0.95). The prevalence of the midventricular muscular trabecular VSD was significantly lower in patients ≥ 3 months of age at initial echocardiography than in those < 3 months (p = 0.010). CONCLUSION: We infer that midventricular muscular trabecular VSD tends to close spontaneously earlier and more frequently than either anterior or apical muscular trabecular VSD.

Spontaneous closure of perimembranous ventricular septal defect after school age.

BACKGROUND: The number of studies of long-term follow up to adolescence is very low on spontaneous closure (SC) of perimembranous ventricular septal defects (P-VSD) in children not undergoing surgical closure because of small left-to-right shunting. METHODS: Seventy patients with a P-VSD with pulmonary-to-systemic flow ratio (Qp/Qs) < 1.7 underwent cardiac catheterization at the age of 2-10 years (mean, 5.1 years). Excluding 22 patients who dropped out by 15 years, 48 were selected. Qp/Qs ranged from 1.00 to 1.68 (mean, 1.17). The average follow-up period of 37 patients excluding the SC patients was 17.8 years. They were classified according to Qp/Qs into three groups: group I, 1.4 or= 1.2 showed no tendency to close spontaneously. The factor most influencing SC of P-VSD after school age seemed to be the shunt ratio.

Does a correlation exist between tumor occupation rate and Dukes classification in rectal cancer?

This study was undertaken to evaluate the correlation between tumor occupation rate and stage in rectal cancer patients. 210 patients with histologically confirmed adenocarcinoma of the rectum were evaluated. Retrospectively, we divided the subjects' pathological specimens into four groups: group A, tumor occupied less than one fourth of the colic lumen; group B, tumor occupied one fourth to one half of the colic lumen; group C, tumor occupied one half to three quarters of the colic lumen; group D, tumor occupied more than three quarters of the colic lumen. As the tumor occupation rate increased, more invasive cases were observed. Patients of group A had significantly longer survival than those of the other groups. Tumor occupation rates correlated significantly with the Dukes classification (r = 0.546, P < 0.0001). Tumor occupation rate is a primitive method but an easy and inexpensive technique for judging tumor stage. This method can assist in preoperative evaluation, thereby reducing the need for other redundant methods.

Surgical treatment of infective endocarditis complicated by intracranial hemorrhage in a patient with hypertrophic obstructive cardiomyopathy.

A patient with hypertrophic obstructive cardiomyopathy developed mitral regurgitation due to infective endocarditis. The patient, a 29-year-old man with a 16-year history of a severe obstructive form of hypertrophic obstructive cardiomyopathy (left ventricular outflow gradient more than 100 mmHg), was admitted with bacteremia. During medical therapy with antibiotics for six months, the patient suffered an intracranial hemorrhage without a mycotic aneurysm and developed severe mitral regurgitation due to the infective endocarditis. One month after clinical stability of the cerebral damage, the patient underwent a combined mitral valve replacement and transaortic septal myectomy. Postoperative echocardiography revealed that the left ventricular outflow gradient had decreased to 15 mmHg. Ten months after the combined operation, the patient was well and asymptomatic.

Spontaneous closure of ventricular septal defects followed up from <3 months of age.

BACKGROUND: This study evaluates the incidence and timing of spontaneous closure (SC) of ventricular septal defect (VSD) using Doppler color flow mapping. METHODS: A total of 225 infants (mean age 30 days) were diagnosed with uncomplicated VSD: 31 (14%) subpulmonary VSD, 159 (70%) perimembranous, and 35 (16%) muscular. The patients were divided into two groups according to the presence or absence of congestive heart failure (CHF). SC was confirmed with color Doppler. RESULTS: Surgical closure was performed in 59 patients (26%). SC occurred in 107 patients (48%); three (10%) of 31 with subpulmonary VSD, 75 (47%) of 159 with perimembranous VSD, and 29 (83%) of 35 with a muscular VSD. Average age at SC was 19 months. In three SC patients with a subpulmonary VSD, there was no aortic valve prolapse and no aortic regurgitation. SC occurred in 96% of SC patients with a perimembranous VSD by the age of 6 years, and in 93% of those with a muscular VSD by the age of 3 years. In patients without CHF, the rate of SC was 72%; 23% in subpulmonary VSD, 74% in perimembranous, and 85% in muscular. SC occurred in only 23% of patients with a perimembranous VSD with CHF. Mean age at the final examination was 6.9 years in 59 patients with a VSD remaining open, and 63% of patients with a perimembranous VSD remaining open had an aneurysm of the ventricular membranous septum. CONCLUSIONS: The SC rate of VSD by mean age of 6.9 years was 48%, but it was 72% in patients without CHF. In patients with CHF, SC was seen only in patients with a perimembranous VSD. The rate of SC was 10% in subpulmonary VSD. The authors contend that SC probably occurred by growth of muscular septum surrounding VSD. Muscular VSD spontaneously closed earlier than perimembranous VSD.

Myocardial ischemia in Kawasaki disease: evaluation with dipyridamole stress technetium 99m tetrofosmin scintigraphy.

BACKGROUND: The coronary artery abnormalities in Kawasaki disease (KD) often cause myocardial ischemia. Previous publications have described the use of thallium 201 myocardial perfusion imaging to determine the extent of ischemia in patients with KD. The technetium perfusion agents offer better resolution and may offer additional information about ventricular function in these patients. This study was performed to evaluate myocardial perfusion in patients with KD through use of technetium 99m tetrofosmin (TF) in conjunction with dipyridamole vasodilator stress. METHODS AND RESULTS: Eighty-six patients with KD aged 11.5 +/- 6.4 years and 20 age-matched control patients without heart disease were studied. Among 86 patients with KD, significant coronary artery stenosis (>or=75%) was observed in 20, coronary aneurysm without stenosis in 37, and no coronary lesions in 29. After administration of high-dose dipyridamole infusion (0.70 mg/kg), 74 to 370 MBq TF was injected and 148 to 740 MBq TF was injected at rest 4 hours later. Single photon emission computed tomography images were obtained more than 30 minutes after TF injection. Regional myocardial hypoperfusion was observed in 18 patients who had coronary artery stenosis of 75% or greater (sensitivity, 90%, and specificity, 100%). Two patients did not demonstrate ischemic changes in coronary artery stenosis in group I; one had good collateral flow. There were no TF injection-related complications. CONCLUSIONS: Tetrofosmin myocardial perfusion imaging can detect regional hypoperfusion in children with KD.