Cardiac tamponade due to systemic lupus erythematosus in patient with Prader-Willi syndrome after growth hormone therapy.

We present a 16-year old girl with Prader-Willi syndrome who developed cardiac tamponade as an initial finding of systemic lupus erythematosus. Until one year prior to this episode, she had received growth hormone treatment for nine years. The association among Prader-Willi syndrome, growth hormone treatment and systemic lupus erythematosus is discussed.

Hepatic induction of mitochondrial and cytosolic acyl-coenzyme a hydrolases/thioesterases in rats under conditions of diabetes and fasting.

Acyl-coenzyme A (CoA) hydrolases/thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoA thioesters to free fatty acids and CoA-SH. The potency of these enzymes may serve to modulate intracellular concentrations of acyl-CoAs, free fatty acids, and CoA to affect various cellular functions, including lipid metabolism. In this study, we investigated the effect of diabetes and fasting on the protein levels of mitochondrial (MTE-I) and cytosolic acyl-CoA thioesterases (CTE-I), multigene family members of this class of enzymes, in adult rat liver. Rats were treated with alloxan to induce diabetes or fasted for 72 hours. Western blot analysis with the liver homogenates revealed 2.8-fold and 3.8-fold increases in MTE-I and 8.5-fold and 9.2-fold increases in CTE-I under the diabetic and fasting conditions, respectively, compared with the control in which the level of MTE-I was 4.3-fold higher than CTE-I. Serum level of free fatty acids was elevated 5-fold and 2.5-fold in diabetic and fasted rats, respectively. These results confirm the adaptive induction of MTE-I and CTE-I in response to fatty acid overload in the liver, being consistent with their auxiliary role in fatty acid degradation.

[Clinical characteristics of congenital heart disease diagnosed during neonatal period].

We have analyzed 43 newborn babies with congenital heart disease (CHD) over the last two years. The occurrence rate of CHD was 7.5 per 1,000 live births in our maternity hospital, and 4.5% of all babies admitted to our NICU. Low birth weight (<2,500g), and prematurity (<37 weeks of gestation) led to occurrence rates of 34% and 26%, respectively. Cardiac symptoms (heart murmur and cyanosis) accounted for 47% of all cases in which CHD was discovered, and extracardiac symptoms accounted for 40%. Prenatal diagnosis was made in two patients. Twenty babies were transferred to the pediatric cardiac care unit in the general hospital from our maternity hospital during their neonatal period. Dividing them into two categories by hemodynamic state, acyanotic type made up 72% of all patients; VSDs (Ventricular Septal Defects) were the most frequent anomaly. Cyanotic type made up only 28%, but included all 4 cases of neonatal death. Twelve babies underwent surgery during the study period, and 7 of them reached final anatomical correction. Early detection of the cardiac anomaly, including prenatal diagnosis, plays an important role in improving the prognosis of patients, and comprehensive therapeutic strategies are essential for neonatal CHD.

Feasibility of percutaneous transluminal coronary angioplasty to patients with Kawasaki Disease as an early management strategy.

We successfully performed percutaneous transluminal coronary angioplasty (PTCA) in three infants with Kawasaki disease ages 11 to 29 months. The time from the onset of disease to PTCA ranged from 6 to 21 months. On period reevaluation 12 to 40 months after PTCA, they had no evidence of myocardial ischemia or restenosis. As an early management strategy, PTCA could be a good palliation to control myocardial ischemia associated with Kawasaki disease. From our study and a literature review, we suggest that PTCA may be more effective if it is performed earlier, even in young patients.

Hemodynamic factors of thrombus formation in coronary aneurysms associated with Kawasaki disease.

BACKGROUND: We studied the mechanism of thrombus formation in coronary aneurysms based on rheological findings. METHODS: We studied 43 coronary aneurysms in 33 patients with Kawasaki disease (mean (+/- SD) age 6.1 +/- 4.3 years). These lesions were divided into three groups on the basis of maximum diameter: (i) small (group S); (ii) medium sized (group M); and (iii) large (group L) aneurysms. Using a Doppler flow guidewire and a pressure-monitoring guidewire, we measured coronary flow velocity and perfusion pressure inside aneurysms and in adjacent normal-looking vessels. We calculated the average peak velocity (APV) index, the mean coronary perfusion pressure (P) index and shear index. RESULTS: The APV index and shear index decreased significantly (p < 0.005) as the aneurysm size increased (APV index in groups S, M and L was 0.893 +/- 0.149, 0.573 +/- 0.242 and 0.128 +/- 0.131, respectively; shear index in groups S, M and L was 0.750 +/- 0.149, 0.328 +/- 0.153 and 0.020 +/- 0.028, respectively). However, coronary perfusion pressure showed no relationship to aneurysm size and was not significantly different from that in adjacent normal-looking vessels. CONCLUSIONS: The results of the present study suggest that the stagnation of flow and the reduction of shear stress in coronary aneurysms could initiate thrombus formation.

Modified Fontan operation. Considerations for the determination of the appropriate procedure.

BACKGROUND: Although the surgical results of the modified Fontan operation continues to improve, there are various advantages and disadvantages in terms of the post operative condition associated with the Fontan modifications. Late morbidity and mortality are mainly due to arrhythmias, thromboembolic complications, systemic venous hypertension and infective endocarditis. We reported our experience of the modified Fontan operation to determine an appropriate procedure for each patient. METHODS AND RESULTS: Seven patients (ranging from the age 1-14 years) underwent a modified Fontan operation including a lateral tunnel (n = 1), extracardiac conduit (n = 2) and autogenous atrial tunnel (n = 4). There was one hospital death due to sepsis in which the patient underwent lateral tunnel procedure. The mean follow up of another six patients was 20 months (ranging from 1-39 months) and all patients were classified as NYHA class I, and remained in normal sinus rhythm without any thromboembolic complications. CONCLUSIONS: When using the autogenous atrial tunnel, there are potential advantages; it is not associated with thromboembolism or endocarditis and has growth potential. However, in high-risk patients with increased pulmonary vascular resistance, impaired ventricular function and pre-operative atrial arrhythmias, it appears reasonable to use an extracardiac conduit.

Silent myocardial ischemia in Kawasaki disease: evaluation of percutaneous transluminal coronary angioplasty by dobutamine stress testing.

BACKGROUND: Myocardial ischemia and myocardial infarction are the most serious complications of coronary artery lesions in children with Kawasaki disease (KD). Therefore, early detection and treatment of myocardial ischemia in patients with KD is essential. We studied the effectiveness of percutaneous transluminal coronary angioplasty (PTCA) in patients with silent myocardial ischemia detected by dobutamine stress 99mTc myocardial scintigraphy (TMS), body surface mapping (BMS), and signal-averaged ECG late potentials (ELP). METHODS AND RESULTS: Eight of 76 asymptomatic patients with a coronary stenosis >25% and a positive dobutamine stress test were considered to have silent myocardial ischemia. All eight patients had >95% stenoses demonstrated by coronary angiography (CAG) just before PTCA. After PTCA, CAG showed that all of the coronary artery stenoses had been reduced to <50%. Additionally, intravascular ultrasonography (IVUS) performed in five patients before and after PTCA demonstrated adequate dilation of the coronary stenosis after PTCA. All eight patients underwent dobutamine stress TMS, BMS, and ELP 2 to 3 months after PTCA, which demonstrated no regions of myocardial ischemia. Approximately 6 months later, CAG was performed in all eight patients, and only one patient had developed restenosis. CONCLUSIONS: PTCA effectively dilates stenotic coronary arteries in children with KD. Moreover, dobutamine stress TMS, BMS, and ELP are useful for detecting silent myocardial ischemia and estimating the effectiveness of PTCA. Furthermore, IVUS is useful for evaluating the severity of coronary artery lesions before and after PTCA in patients with KD.

[Pathomorphological studies on arterial cushion. 2. Aminonucleoside nephrotic rat].

The present study is an attempt to solve the problem of pathogenesis of focal glomerular sclerosis (FGS), especially in juxtamedullary cortex, we were investigated by measurement of luminal diameters of afferent arterioles (Aff), efferent arterioles (Eff) of numerous glomeruli, arterial cushion (AC), afferent arterioles (AC-aff) at a region of branching arteries from the interlobular arteries, using scanning electron micrographs of methyl methacrylate casts of intrarenal arteries of aminonucleoside nephrotic rats. As regards luminal diameters, Eff were nearly equal Aff in minor glomerular abnormalities (Minor). Nevertheless Eff of glomeruli with segmental sclerosis (FSHS) were smaller than Aff (t-test). AC with FSHS were smaller than ones of Minor, statistically (t-test). The results of these examination, the cushion may be important factor for the regulation of blood flow in sclerotic glomeruli.