SUPFAM: a database of sequence superfamilies of protein domains.

Background: SUPFAM database is a compilation of superfamily relationships between protein domain families of either known or unknown 3-D structure. In SUPFAM, sequence families from Pfam and structural families from SCOP are associated, using profile matching, to result in sequence superfamilies of known structure. Subsequently all-against-all family profile matches are made to deduce a list of new potential superfamilies of yet unknown structure.

Modified temporalis anchorage in craniomandibular reankylosis.

This study evaluates the long-term outcomes and clinical results of costochondral graft and temporalis muscle flap interpositioning with submandibular anchorage in the management of TMJ re-ankylosis. Thirty-one patients, 9 children and 22 adults, with recurrence of ankylosis after gap arthroplasty, with a mouth opening less than 5 mm were evaluated. The management protocol consisted of resection of the ankylotic mass through an Al-Kayat and Bramley incision; contralateral coronoidectomy in unilateral cases; replacement of the condyle in children by means of a costochondral graft through Risdon's approach and interpositional temporalis muscle flap and submandibular anchorage of the temporalis flap in children and adults.

Tolerance to the substitution of buried apolar residues by charged residues in the homologous protein structures.

Occurrence and accommodation of charged amino acid residues in proteins that are structurally equivalent to buried non-polar residues in homologues have been investigated. Using a dataset of 1,852 homologous pairs of crystal structures of proteins available at 2A or better resolution, 14,024 examples of apolar residues in the structurally conserved regions replaced by charged residues in homologues have been identified. Out of 2,530 cases of buried apolar residues, 1,677 of the equivalent charged residues in homologues are exposed and the rest of the charged residues are buried.

Apparent protective effect of increased left ventricular wall thickness in an ICD population.

Background: Expanding indications for the implantable cardioverter defibrillator (ICD) call for further enhancement of patient selection for optimization of use. Because a subgroup of patients who receive ICDs may not receive therapies, we sought to identify clinical predictors of therapy-free survival in ICD patients.

Methods: We performed an analysis of a single-center, 13-year ICD implantation experience (1990-2002).

Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology.

We describe a 5-year-old boy with Fontan physiology and a ventricular pacemaker who developed severe plastic bronchitis. Evaluation by cardiac catheterization revealed profoundly altered hemodynamics, which improved with atrial pacing. Following implantation of an atrial pacemaker, which restored atrioventricular (A-V) synchrony, the patient's hemodynamics greatly improved and his plastic bronchitis resolved.

Efficacy of implantable loop recorders in establishing symptom-rhythm correlation in young patients with syncope and palpitations.

Objective: To evaluate efficacy of the recently introduced implantable loop recorder (ILR) in establishing symptom-rhythm correlation in young patients with syncope, near syncope, palpitations, and acute life-threatening events (ALTEs).

Methods: A retrospective study was conducted with patients with ILR from 3 pediatric centers. Inclusion criteria were age View Article and Find Full Text PDF

Recent advances in pediatric cardiology--electrophysiology, transcatheter and surgical advances.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children.

Treatment of steal syndrome complicating a proximal arteriovenous bridge graft fistula by simple distal artery ligation without revascularization using intraoperative pressure measurements.

This case report describes the treatment of a patient with a steal syndrome complicating a brachioaxillary prosthetic bridge graft AV fistula by simple ligation of the brachial artery beyond the fistula. Intraoperative pressure measurements showed concomitant distal revascularization to be unnecessary.

Nonautomatic focal atrial tachycardia: characterization and ablation of a poorly understood arrhythmia in 38 patients.

Nonautomatic focal atrial tachycardia (NAFAT) is a rare and poorly understood arrhythmia either due to microreentry or triggered mechanism. NAFAT was defined as a focal atrial tachycardia which was inducible with pacing maneuvers in the electrophysiology lab. We reviewed the charts and EP study reports of all 38 patients with NAFAT, who underwent an EP study at our center between April 1994 and September 2000.

Unruptured sinus of Valsalva aneurysm with right ventricular outflow obstruction.

The recommended operative management of unruptured sinus of Valsalva aneurysm consists of closure of the mouth of the aneurysm with or without aortic valve surgery. We report a case of unruptured aneurysm producing right ventricular outflow tract obstruction. Closure of the mouth of the aneurysm failed to relieve the obstruction, which was subsequently achieved by excising the aneurysmal wall overlying the outflow tract.

Modeling and mutational analysis of the GAF domain of the cGMP-binding, cGMP-specific phosphodiesterase, PDE5.

The GAFa domain of the cGMP-binding, cGMP-specific phosphodiesterase (PDE5A) was modeled on the crystal structure of PDE2A GAF domain and residues involved in cGMP binding identified. Tandem GAFa and GAFb domains of PDE5A, expressed in Escherichia coli, bound cGMP (K(d) 27 nM). Mutation of aspartate-299 in GAFa, suggested earlier to be critical for cGMP binding, did not abrogate cGMP binding, but mutation of F205, which formed a stacking interaction with the guanine ring of cGMP, led to complete loss of cGMP binding.

Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures.

This study compares the effects of two techniques for the Fontan procedure-intraatrial lateral tunnel (IALT) and extracardiac conduit (ECC) -on sinus node dysfunction. Between January 1992 and December 1998, 54 patients underwent a total cavopulmonary connection Fontan procedure. Of these, 36 had follow-up 24-hour Holter recordings, and they constitute the population for this study.

A modified temporalis transfer in facial reanimation.

A modified surgical procedure for temporalis transfer in facial reanimation of five consecutive cases is presented. Instead of the traditional stripping of the temporalis from its origin, its attachment at the coronoid removed, and to its end, the harvested fascia lata graft was sutured to lengthen the muscle's action. These fibres were then passed to the Orbicularis Oculi and Oris to aid in reanimation and to improve their tone.

Integration of related sequences with protein three-dimensional structural families in an updated version of PALI database.

The database of Phylogeny and ALIgnment of homologous protein structures (PALI) contains three-dimensional (3-D) structure-dependent sequence alignments as well as structure-based phylogenetic trees of protein domains in various families. The latest updated version (Release 2.1) comprises of 844 families of homologous proteins involving 3863 protein domain structures with each of these families having at least two members.

Management of deficient anterior maxillary alveolus with mandibular parasymphyseal bone graft for implants.

This paper discusses the use of autogenous bone graft from the symphyseal region of the mandible to widen the deficient anterior maxilla. Compressing short or narrow implants into deficient ridges is a poor technique that often fails to correctly replace ridge anatomy or afford stable restorations. Patients who were missing a maxillary incisor, and exhibiting a loss of supporting bone in the area, were found suitable for this technique.

Atrial arrhythmias in congenital heart disease.

Surgical scars, volume overload and pressure overload are the three main determinants of atrial arrhythmias in patients with prior congenital heart surgery. The workup should include a thorough hemodynamic assessment. Treatment modalities available include drugs, pacemakers, catheter ablation and surgery.

Update on intensive care ECG and cardiac event monitoring.

This brief review is aimed primarily as a resource for the clinician and summarizes recent advancements in electrocardiographic monitoring in the intensive care unit. Emphasis is placed on recent advances in ICU ECG and cardiac event monitoring with particular attention to arrhythmia detection in patients following myocardial infarction. Specific topics addressed include: clinical indicators of impending arrhythmic events and sudden death, signal averaged ECG, QT dispersion, ST segment fluctuation, T-wave alternans, QT interval beat-to-beat variability, heart rate variability, and advances in automated arrhythmia detection.

Postoperative Cardiac Arrhythmias in Children.

The cardiac rhythm can be very labile in the early postoperative period, necessitating a high degree of vigilance. Bradycardia may be due to sinus node dysfunction or varying degrees of atrioventricular block, both of which are usually due to surgical trauma. Temporary pacing, using implanted temporary pacing wires, should be readily available.

Use of a database of structural alignments and phylogenetic trees in investigating the relationship between sequence and structural variability among homologous proteins.

The database PALI (Phylogeny and ALIgnment of homologous protein structures) consists of families of protein domains of known three-dimensional (3D) structure. In a PALI family, every member has been structurally aligned with every other member (pairwise) and also simultaneous superposition (multiple) of all the members has been performed. The database also contains 3D structure-based and structure-dependent sequence similarity-based phylogenetic dendrograms for all the families.

PALI: a database of alignments and phylogeny of homologous protein structures.

PALI is a database of structure-based sequence alignments and phylogenetic relationships derived on the basis of three-dimensional structures of homologous proteins. This database enables grouping of pairs of homologous protein structures on the basis of their sequence identity calculated from the structure-based alignment and PALI also enables association of a new sequence to a family and automatic generation of a dendrogram combining the query sequence and homologous protein structures.

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.

Objective: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing.

Background: Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects.

Adsorption Studies of Nonionic Surfactants onto Polyvinyltoluene Microlatexes in Aqueous Medium.

Negatively charged polyvinyltoluene (PVT) microlatexes of various dimensions were prepared by polymerization of the corresponding vinyltoluene-in-water microemulsion precursors containing sodium dodecyl sulfate and n-pentanol. PVT microlatexes were used as adsorbents for adsorption of nonionic surfactants such as Tween 80, Tween 20, and Triton X-100. The extents of adsorption were known from the differences in the diffusion coefficient of the PVT microlatexes containing a solubilized suitable electro active probe.

Supraventricular Tachycardia in Children.

A reasonably precise and mechanistic diagnosis of the cause of supraventricular tachycardia (SVT) can be made using noninvasive tests such as an electrocardiogram, Holter monitoring, or cardiac event recorder and by determining the response to vagal maneuvers (or intravenous adenosine). Therapy options include prophylactic drug therapy, catheter ablation, and doing nothing (for those with infrequent and non-life-threatening symptoms). Drug therapy attempts to suppress the arrhythmia rather than to cure it and could cause side effects.

A new framework for echocardiographic assessment of left ventricular mechanics: sensitivity to heart failure.

A recent report describes an approach to ventricular mechanics that employs mean end-systolic fiber stress and an exact mathematical strain index based on wall thickness referenced to myocardial mass. We used echocardiography and mean arterial pressures to determine the strain index and wall stress in (1) normal hearts from patients and swine, (2) swine with pacing-induced congestive heart failure, and (3) patients with dilated cardiomyopathy. Pigs were also studied under afterload variation with phenylephrine.