Successful combined intravenous and subcutaneous immunoglobulin treatment for intractable protein-losing enteropathy in a patient long after Fontan-type operation.

A 20-year-old patient, who had double outlet right ventricle, mitral atresia, pulmonary atresia, and bilateral superior vena cava and underwent successful lateral tunnel total cavo-pulmonary connection at 6 years old, presented with frequent watery diarrhea, general malaise, and tetany. He was known to have intractable protein-losing enteropathy (PLE) from 7 years of age that was resistant to various treatments. To keep hemodynamics stable, he required intravenous albumin infusion every day.

Transpulmonary echocardiography to guide stent implantation into coarctation of the aorta.

Although stent implantation into aortic coarctation has been performed solely under fluoroscopy, we successfully applied intracardiac echocardiography (ICE) to guide this procedure in a 13-year-old patient. Placing an intracardiac echocardiographic catheter in the left pulmonary artery facing upward, we readily visualized the precise anatomy of coarctation, measured the pressure gradient, and monitored the stent inflation process. This report suggests a new application of ICE for intervention with structural and vascular diseases other than interatrial septum.

Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome.

There are few reports on successful high-dose spironolactone treatment of refractory protein-losing enteropathy (PLE) caused by Fontan procedure. We report successful diuretics treatment with spironolactone and furosemide at standard dose, of refractory PLE in a patient with Noonan syndrome and repaired congenital heart disease. This is the first successful application of diuretics treatment in a patient with refractory PLE without Fontan procedure.

Trans-pulmonary echocardiography as a guide for device closure of patent ductus arteriosus.

Objectives: The aim of this study was to develop trans-pulmonary echocardiography (TPE) to guide device closure of patent ductus arteriosus (DC-PDA).

Background: Aortography requires a large amount of contrast yet may give us an inadequate image to evaluate anatomy or residual shunt in patients with large PDA or dilated vessels and is precluded in patients with renal dysfunction. Practically, there is no imaging modality to monitor the entire procedure except for trans-esophageal echocardiography that requires general anesthesia.

Intra-cardiac echocardiography-guided stent implantation into stenosed superior vena cava in a patient with a history of contrast anaphylaxis.

A 37-year-old patient, who suffered from a repeated superior vena cava (SVC) syndrome, was scheduled for stent implantation into SVC, but suffered from contrast anaphylaxis. To monitor the procedure, we used intra-cardiac echocardiography and successfully implanted a stent. Placing an intra-cardiac echocardiographic catheter in the main pulmonary artery and facing towards the right, we could readily visualize stenosis in the SVC and inflation of the stent.

Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience.

Background: Some patients with Kawasaki disease develop giant coronary aneurysms and coronary stenosis, leading to ischemic heart disease. The aim of this study was to determine the long-term prognosis of patients with Kawasaki disease with giant aneurysms.

Methods And Results: From our institutional database, 76 patients (57 men and 19 women) who developed giant aneurysms after January 1, 1972, were identified.

Increased plasma type B natriuretic peptide in the acute phase of Kawasaki disease.

Background: The aim of this study was to identify possible factors associated with type-B natriuretic peptide (BNP) production in the acute phase of Kawasaki disease (KD).

Methods: Subjects were 54 patients with KD (KD group [KDG]) and 18 age-matched controls (control group [CG]). We evaluated left ventricular function using multi-modal echocardiography and determined blood chemistry including BNP, white blood cell count, C-reactive protein (CRP), and interleukin (IL)-6 in the KDG.

Microembolic signals measured by transcranial Doppler during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.

Purpose: To determine the frequency and factors associated with increase in microembolic signals during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.

Methods: During the procedure in 16 patients, we measured microembolic signals using transcranial Doppler. Procedure time was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; left cardiac angiocardiography; sizing and long sheath placement; device placement and release.

Impact of intravenous immunoglobulin infusion on longitudinal left ventricular performance in patients with acute Kawasaki disease of usual course.

Purpose: To determine the acute change in cardiac performance after intravenous immunoglobulin infusion (IVIG) in patients with acute Kawasaki disease (KD).

Materials And Methods: Subjects were 33 patients with KD who were treated with IVIG 2 g/kg and recovered without coronary artery lesion and 27 controls. Subjects underwent combined two-dimensional, Doppler, and tissue Doppler echocardiographic (TDI) studies.

Multicenter and retrospective case study of warfarin and aspirin combination therapy in patients with giant coronary aneurysms caused by Kawasaki disease.

Background: To determine the prognosis of patients with giant coronary aneurysms (GA) caused by Kawasaki disease (KD) treated with combined oral warfarin and aspirin.

Methods And Results: A multicenter follow-up study of 83 patients (65 males, 18 females) with GA who had been treated for > or =3 months with warfarin. Most patients were placed on the combination therapy as soon as the GA was detected and remained on it for 6.

Improved antibody responses in infants less than 1 year old using intradermal influenza vaccination.

Background: Antibody response to influenza vaccine is limited in early. Infants have poorer hemagglutination-inhibiting antibody responses than 12-month-old. Intradermal administration reportedly elicited immune responses similar to or better than a standard intramuscular dose.

[Prevention of thrombosis of coronary aneurysms in patients with a history of Kawasaki disease].

Patients with coronary artery aneurysms caused by Kawasaki disease are at increased risk of coronary thrombosis and ischemia. To prevent coronary thrombosis, long-term anti-thrombosis using anti-platelet drugs, such as aspirin, dipyridamole, ticlopidine, clopidogrel, and abciximab, with or without warfarin is recommended by official guidelines. In fact, aspirin or aspirin with warfarin are the most frequently administered regimen in these patients with coronary aneurysms.

Glucose oxidation catalyzed by liposomal glucose oxidase in the presence of catalase-containing liposomes.

A catalase-containing liposome (CAL) was prepared and characterized in terms of stability during storage and catalysis of the decomposition of hydrogen peroxide (H2O2) that was initially added or produced in the oxidation of glucose catalyzed by the glucose oxidase-containing liposomes (GOL). The reactors used were a test tube and an external loop airlift bubble column as the static liquid and circulating liquid flow systems, respectively. The free catalase (CA) at low concentrations was unstable during storage at 4 degrees C as a result of dissociation of the tetrameric CA subunits.