Infantile Dilated Cardiomyopathy in Alström Syndrome.

We report two cases of end-stage dilated cardiomyopathy as the initial manifestation of Alström syndrome (ALMS), in infants aged two and five months. This rare monogenic, autosomal, and recessive genetic condition is a multisystem disorder characterized by visual and hearing impairment, cardiomyopathy childhood obesity, and other anomalies. These cases highlight the importance of genetic testing targeting the ALMS1 gene in the assessment of apparently isolated dilated cardiomyopathy.

Pediatric vascular ring outcomes for surgically repaired unoperated children: a single-center experience.

Background: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate.

Atrial fibrillation in a pediatric patient caused by an unusual malignant etiology: A case report.

This case report describes a 15-year-old patient with a known congenital malformation syndrome and immune deficiency, presenting with new-onset atrial fibrillation (AF) after a recent diagnosis of an intrathoracic mass. Transthoracic echocardiography showed a structurally and functionally normal heart and workup confirmed a primary diffuse large B-cell lymphoma, with pericardial and left atrial involvement on cardiac magnetic resonance imaging. Electrical cardioversion was successfully performed to convert the AF and chemotherapy was promptly started.

Leftward deviation of the primary septum or dividing left atrial shelf?

Isolated leftward prolapse or deviation of the primary atrial septum is a rare CHD that can mimic abnormal pulmonary venous return at first sight. We present a case of a newborn infant, referred for surgical correction of totally anomalous pulmonary venous return into the right atrium, with the peri-operative finding of a leftward deviation of the superior margin of the primary atrial septum. The distinction with a dividing atrial shelf could not be confirmed with certainty.

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair.

Lipiodol-based lymphangiography is not only a diagnostic tool for visualization of lymphatic disorders such as plastic bronchitis (PB), but also aims a therapeutic effect by embolizing lymph leakages. We performed such percutaneous lymphatic embolization for PB in a Fontan patient with proven absence of right-to-left shunt, and demonstrated important lymphatic abnormalities in the mediastinum. Shortly after the procedure, the patient developed severe convulsive seizures, revealing multiple cerebral embolisms of Lipiodol.

Direct EBUS-guided transtracheal lymphosclerosis for plastic bronchitis after Fontan.

We report on a new puncture technique with direct transtracheal mediastinal lymphatic access to treat plastic bronchitis after Fontan repair. High resolution contrast-enhanced spiral CT identified enlarged lymph nodes in the paratracheal region. Inguinal intranodal Gadolinium Dynamic Contrast-enhanced Magnetic Resonance lymphangiography (DCMRL) confirmed the pathologic centrifugal lymph flow passing through these lymph nodes before leaking into the bronchial tree.

Infective endocarditis in patients after percutaneous pulmonary valve implantation with the stent-mounted bovine jugular vein valve: Clinical experience and evaluation of the modified Duke criteria.

Aims: Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment.

Methods: Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers.

Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation.

Background: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB).

Methods: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae.

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

Background And Aims: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices.

Methods And Results: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.

Use of the polymerase chain reaction (PCR) for the detection of aacA genes encoding aminoglycoside-6'-N-acetyltransferases in reference strains and gram-negative clinical isolates from two Belgium hospitals.

Genes encoding aminoglycoside 6'-N-acetyltransferases, were identified using the polymerase chain reaction (PCR). Four sets of primers delineating DNA fragments of 209 bp, 250 bp, 260 bp and 347 bp, specific for the four known aacA genes, and probes within these fragments, were constructed based on the nucleotide sequences of the aacA genes. The specificity of the primers was evaluated using reference strains encoding various aminoglycoside-modifying enzymes.

Serotypes and extended spectrum beta-lactam resistance in aminoglycoside resistant Pseudomonas aeruginosa isolates from two Belgian general hospitals: a seven year study.

A total of 1896 isolates of Pseudomonas aeruginosa resistant to aminoglycosides and isolated during the period 1983-1989 in two Belgian general hospitals were included in this study. The most frequently encountered O serotypes were O4, O11, O12 and non-typable isolates. The majority of the isolates showed resistance to extended spectrum beta-lactam antibiotics (cefotaxime, ceftriaxone and cefepime).

Activity of cefotiam in combination with beta-lactam antibiotics on enterobacterial hospital strains.

By using checkerboard titrations the effect of cefotiam combined with different beta-lactam antibiotics on fifty strains of Enterobacteriaceae moderately susceptible (minimal inhibiting concentration greater than or equal to 8 mg/l) or resistant (minimal inhibiting concentration greater than or equal to 64 mg/l) to cefotiam was evaluated. The following compounds were tested: cefamandole, cefazolin, cefmenoxime, cefotaxime, cefotiam, ceftazidime, cefuroxime, mecillinam and piperacillin. The synergistic effect varied markedly.

[Antibacterial activity of carumonam and cefpirome on hospital strains resistant to gentamicin and cephalothin: comparison with other beta-lactam antibiotics, new fluoroquinolones, aminoglycosides and other antibiotics].

The antibacterial in vitro activity of carumonam, a new monobactam, and cefpirome, a new cephalosporin, was studied on 483 hospital strains resistant to gentamicin and cephalothin, in comparison with amikacin, azlocillin, aztreonam, cefmenoxim, cefoperazone, cefotaxim, cefsulodin (for Pseudomonas), ceftazidime, ceftriaxone, cefuroxim, chloramphenicol, ciprofloxacin, doxycycline, enoxacin, netilmicin, norfloxacin, pefloxacin, piperacillin, rifampicin, tobramycin and trimethoprim. In general the two compounds have a very good in vito activity on Enterobacteriaceae but are less active on non-fermenting microorganisms. For the Enterobacteriaceae the minimal inhibitory concentrations 90% for carumonam was less than or equal to 1.

Three-year survey of amikacin use and aminoglycoside resistance in a general hospital in Belgium.

The aim of the study was to evaluate the effect of intensive use of amikacin on the resistance levels to amikacin, gentamicin, tobramycin, netilmicin and dibekacin. The base-line resistance in the preamikacin phase (three months of amikacin use less than 1%; 676 isolates) was 1.0% for amikacin, 11.

[In vitro bacteriostatic and bactericidal effect of ciprofloxacin and others quinolone derivatives on Campylobacter jejuni].

The in vitro bacteriostatic (MIC) and bactericidal (MBC) activities of ciprofloxacin and seven other quinolone derivatives on Campylobacter jejuni from human origin were determined. Ciprofloxacin, pefloxacin and rosoxacin exhibited the best bacteriostatic and bactericidal activities. For the three compounds the MIC90 was less than or equal to 0.

The comparative activity of pefloxacin, enoxacin, ciprofloxacin and 13 other antimicrobial agents against enteropathogenic microorganisms.

In this study, we compared the activity of pefloxacin, enoxacin and ciprofloxacin against 269 enteropathogenic strains (Campylobacter jejuni, enteropathogenic Escherichia coli, Salmonella typhi, Shigella spp., Vibrio cholerae and Yersinia enterocolitica) with that of rosoxacin, flumequin, nifuroxazide, erythromycin, chloramphenicol, ampicillin, cefotaxime, tetracycline, amikacin, netilmicin, sulfamethoxazole, trimethoprim and co-trimoxazole. Pefloxacin, enoxacin and ciprofloxacin were always among the most active compounds.

Antibacterial activity of enoxacin: comparison with aminoglycosides, beta-lactams and other antimicrobial agents.

The activity of enoxacin, a new quinolone carboxylic acid, was evaluated against 3014 clinical isolates of Enterobacteriaceae, Pseudomonas and other non-fermenters and Staphylococcus aureus. Comparison was made with gentamicin, tobramycin, amikacin, netilmicin, ampicillin, piperacillin, carbenicillin, ticarcillin, ticarcillin plus clavulanic acid, trimethoprim, cotrimoxazole and erythromycin. In general enoxacin was the most active compound and resistance was only rarely encountered.

Susceptibility of Gardnerella vaginalis to thiamphenicol: clinical experience with nonspecific vaginitis.

We compared the in-vitro activity of thiamphenicol against 100 strains of Gardnerella vaginalis with the activity of 11 other antimicrobial agents. The MICs for thiamphenicol ranged from 0.39 micrograms/ml to 6.

[Cat-scratch disease].

A case of cat-scratch syndrome is described. The dermatological features and skin reactions reported in this illness (erythema nodosum, erythema multiforma) as well as the diagnostical criteria are reviewed. The hypothetical nature of the etiologic agent of this syndrome is discussed.