Serial stent implantation to relieve in-stent stenosis in obstructed total anomalous pulmonary venous return.

A nine-year-old girl with complex cyanotic heart disease associated with supracardiac total anomalous venous return and obstruction at the drainage site of the vertical vein into the left innominate vein had stent placement at the age of seven, with immediate increase of oxygen saturation and improvement of physical activity. Significant in-stent stenosis occurred that was successfully treated by concentrical placement of a second stent. This case report demonstrates the transluminal approach to be effective in the treatment of stenoses in congenital heart defects that are not eligible for corrective surgery.

GAD65 and IA-2 autoantibodies are common in a subset of siblings of Sardinian Type 2 diabetes families.

Unlabelled: Type 1 diabetes in Sardinia is very common in children, and we hypothesized that Latent Autoimmune Diabetes of Adult (LADA) might constitute a significant proportion of diabetes in adult Sardinian subjects. Since Type 2 diabetes is a familial disorder, we tested this hypothesis by investigating the prevalence of GAD65 and IA-2 autoantibodies (Ab) in Type 2 diabetes multiplex families of Sardinian ancestry enrolled in the Study Group for the Genetics of Diabetes in Sardinia.

Methods: A total of 684 individuals were ascertained from 252 Sardinian Type 2 diabetes multiplex families with 2.

Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart.

Background: Outcome of patients with hypoplastic left heart (HLH) is mainly influenced by the successful first-step palliation according to the Norwood procedure. An alternative approach is heart transplantation (HTX). The feasibility of ductal stenting in newborns with duct-dependent systemic blood flow and bilateral pulmonary artery banding has been reported.

Heart transplantation in children and infants: short-term outcome and long-term follow-up.

Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients.

[Cardiac troponin I after heart surgery corrective operation in infancy and childhood].

Background: Perioperative myocardial damage is an important determinant for postoperative cardiac function and recovery. Cardiac troponin I (cTNI) is a specific marker for myocardial damage. The aim of our study was to evaluate pre- and postoperative cTNI levels, the pattern of elevation in the first four postoperative days and the prognostic value after pediatric cardiac operation.

Safety and efficacy of interventional occlusion of patent ductus arteriosus with detachable coils: a multicentre experience.

Unlabelled: In order to define the safety and efficacy of Cook detachable coils for interventional closure of patent ductus arteriosus, we performed a retrospective analysis of all patients who underwent cardiac catheterization in seven centres for intended interventional occlusion of patent arterial duct. From January 1995 until March 1998, cardiac catheterization for intended interventional occlusion of patent arterial duct was performed in 317 consecutive children. Successful placement of at least one coil was achieved in 282 children (89%).

Newly diagnosed latent autoimmune diabetes in adults (LADA) is associated with low level glutamate decarboxylase (GAD65) and IA-2 autoantibodies. Diabetes Incidence Study in Sweden (DISS).

A quantitative assay with microSepharose was used to determine GAD65Ab and IA-2Ab levels in 771 population-based patients diagnosed with diabetes mellitus at 15 to 34 years of age, and in 828 matched controls. Among the patients, 587 (76%) were classified with type I, 108 (14%) with type II, and 76 (10%) with unclassifiable diabetes. The levels above normal demonstrated a prevalence of GAD65Ab in 66% of type I diabetes, 50% of type II diabetes and 54% of unclassifiable patients and for IA-2Ab in 40%, 17% and 21%, respectively.

Mycotic pseudoaneurysm of the aorta in children.

Mycotic pseudoaneurysm of the aorta is a rare disease in childhood. We report on two cases which were diagnosed in an unselected general pediatric population within an 8-month period. The first case was a 16-month-old toddler with a normal cardiac history who presented with purulent pericarditis due to group A streptococcus and subsequent pseudoaneurysm formation of the ascending aorta while convalescing from varicella infection.

Glutamate decarboxylase (GAD) autoantibody epitope shift during the first year of type 1 diabetes.

Autoantibodies in Type 1 diabetes patients may differentiate between glutamate decarboxylase (GAD65) cloned from human, mouse and rat with a significant better binding to the human antigen. A subgroup of 15% (27/183) patients showed significantly better binding to rodent than to human GAD65. The aim of this study was to determine whether the autoantibody specificity would remain anti-rodent during longitudinal follow-up for one year.

[Fulminant diphtheria myocarditis in an unvaccinated preschool child].

We describe a case of a three year old unvaccinated child who developed a fulminant diphtheric myocarditis and nephritis four days after the onset of a tonsillar diphtheria. Despite of the administration of antitoxin on day three and four the child died within 48 hours from the beginning of rhythm disturbances. The cardiac involvement rapidly progressed from bradyarrhythmias with the necessity of a temporary pacemaker to ventricular rhythm disturbances with cardiac failure and final ventricular fibrillation.

[Reopening of a persistent left superior vena cava in the early postoperative period following bidirectional cavopulmonary anastomosis--treatment by coil embolization].

Diagnosis of systemic venous drainage is mandatory for patients with congenital heart disease planned for cavopulmonary anastomosis or any Fontan-type palliation. Preexisting venous channels as the persistent left superior vena cava are common in cardiac anomalies and may lead to postoperative deterioration and cyanosis. We describe a 6 month old boy with a complex imbalanced atrioventricular septal defect who developed cyanosis in the very early postoperative period, following bidirectional cavopulmonary anastomosis.

Reduced inotropic support after aprotinin therapy during pediatric cardiac operations.

Background: Several reports indicate that aprotinin treatment before and during cardiopulmonary bypass (CPB) might have a protective effect on the myocardium. We evaluated the hemodynamic effects of perioperative aprotinin treatment.

Methods: We conducted a randomized, double-blind, placebo-controlled trial in 34 infants (mean age, 2.

Species-specific autoantibodies in type 1 diabetes.

GAD65 autoantibodies (GAD65Ab) are important markers for type 1 (insulin-dependent) diabetes mellitus. Although most patients have GAD65Ab at the time of clinical diagnosis, there are also GAD65Ab-positive individuals in the population at low risk of developing type 1 diabetes. The aim of this study was to test the hypothesis that the GAD65Ab reactivity to GAD65 cloned from human, mouse, and rat in newly diagnosed type 1 diabetic patients differ from antibody-positive healthy individuals.

Percutaneous closure of secundum atrial septal defect with a new self centering device ("angel wings").

Objective: To investigate the safety, efficacy, and clinical application of a new self centering device ("angel wings") for closure of secundum atrial septal defects (ASD II) and persistent foramen ovale in all age groups.

Design: Multicentre, prospective, nonrandomised study.

Inclusion Criteria: defects with an occlusive diameter of < or = 20 mm and a surrounding rim of > 4 mm; body weight > 10 kg; and an indication for surgical closure of secundum atrial septal defect.

Superior caval venous syndrome after atrial switch procedure: relief of complete venous obstruction by gradual angioplasty and placement of stents.

Superior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective.

Clinical experience with heart transplantation in infants.

Objective: Orthotopic heart transplantation has become an accepted therapeutic concept for adult patients with endstage heart disease. In newborns and infants this procedure is still a matter of discussion because of unknown long-term results and the lack of donor organs.

Methods: Since March 1988 we have performed 40 orthotopic heart transplantation in 39 infants who were from 1 to 280 days of age.

A simple and rapid microSepharose assay for GAD65 and ICA512 autoantibodies in diabetes. Diabetes Incidence Study in Sweden (DISS).

GAD65Ab and ICA512Ab are strongly associated with insulin-dependent (Type 1) diabetes mellitus. A novel, simple radio-antigen binding assay with microSepharose conjugated with monoclonal antibodies specific for human immunoglobulin light chains was developed to provide diagnostic sensitivity and specificity of GAD65Ab and ICA512Ab for Type 1 diabetes. The Receiver Operating Characteristic (ROC) curve was used to determine the upper level of Normal in 583 new onset Type 1 diabetic patients and in 829 matched controls.

Immunology in diabetes: an update.

Type 1 (insulin-dependent) diabetes mellitus is strongly associated with autoimmune phenomena connected to the loss of beta-cells in the pancreatic islets. Despite considerable progress in our understanding of genetic susceptibility factors and islet autoimmunity preceding the clinical onset of Type 1 diabetes there are considerable gaps in our knowledge. First, the etiology is unclear.

[Heart transplantation in infancy: experiences at the Giessen Pediatric Heart Center].

From June 1988 to December 1996 heart transplantations were performed in 36 newborns and infants below one year of age. Diagnosis were hypoplastic left heart syndrome (n = 26), endocardial fibroelastosis (n = 4), cardiomyopathy (n = 3), and other complex congenital heart defects (n = 3). Mean waiting time for transplantation was 52 days, the mean donor-recipient bodyweight ratio was 1.

Evaluation of gastric intramucosal pH during and after pediatric cardiac surgery.

Objectives: In adult patients, intramucosal pH (pHi) has been advocated to detect postoperative complications. The purpose of our study was to evaluate this technique in pediatric patients during and after cardiac surgery.

Methods: Thirty-five infants (age: 5 days to 15 years, median 1.

[Oral gentic syndromes causing dental anomalies].

The author briefly described those genetic syndromes that are accompanied by tooth or dental arch anomalies. Some of these can be corrected by prosthetic means. These anomalies can be indications for prosthetic treatment.

Insulin internalization in the absence of the insulin receptor tyrosine kinase domain is insufficient for mediating intracellular biological effects.

The intracellular portion of the human insulin receptor (hIR) beta-subunit contains distinct functional domains including the exon 16-encoded juxtamembrane (JM) domain that mediates endocytosis, and the tyrosine kinase (TK) domain that mediates insulin's metabolic and mitogenic actions. To explore the functional relationship between these domains and to determine the role of insulin internalization in insulin action, we constructed and studied the endocytic and signaling properties of an hIR mutant truncated at Glu-1012. This truncation removes the carboxyl-terminal 343 amino acids containing essentially all of the TK domain but leaves behind the exon 16-encoded JM domain that is necessary for endocytosis, plus an additional 35 amino acids downstream.