Coronary Artery Bypass Grafting and Percutaneous Coronary Intervention after Kawasaki Disease: The Pediatric Canadian Series.

Coronary artery (CA) aneurysms are serious complications of Kawasaki disease (KD) responsible for ischemic events. Percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) are reported with limited data on indications and comparative efficacy. Retrospective multicenter comparison of CA intervention following KD is performed in this study. Twenty two cases were available from 5 centers, of whom 11 underwent CABG, 10 PCI and 1 systemic thrombolysis. Age at intervention (8.3 ± 3.9 vs 11.3 ± 4.9 years, p = 0.14) and interval from diagnosis (5.6 ± 4.1 vs 6.5 ± 4.7 years, p = 0.64) were similar between CABG and PCI. Interventions were based on angiography in 15 patients or cardiac event in 7, with no difference between CABG and PCI (p = 0.24). Patients with CABG were more likely to undergo multivessel intervention (73 vs 10 %, p = 0.006). None of the patients needed reintervention after CABG, compared to 6 after PCI and 1 after systemic thrombolysis (p = 0.004). Signs of ischemia on stress testing or MIBI were present in 15 patients before intervention and persisted in 9 patients following last intervention, in a significantly higher proportion after CABG than PCI (80 vs 17 %, p = 0.01). In this series, CABG, which mostly involved multivessel intervention, was superior to PCI. Nevertheless, larger-scale studies may help define patient selection criteria for a beneficial PCI approach.

[The association of VAC® therapy, titanium plates osteosynthesis and bilateral pectoral muscle flaps in the management of postoperative mediastinitis in an obese and diabetic patient]. / Intérêt de l'association VAC®-thérapie, ostéosynthèse sternale et lambeaux pectoraux d'avancement dans la prise en charge d'une médiastinite postopératoire après chirurgie cardiaque chez une obèse diabétique.

Postoperative mediastinitis is one of the most worrisome complications after heart surgery. Until now there is no universally accepted strategy in the management of this infectious complication. Recently, various novel techniques like negative pressure therapy and titanium plates sternal reconstruction have allowed a dramatic decrease of mortality and morbidity after mediastinitis. We report the case of a diabetic patient suffering from morbid obesity who developed a severe postoperative mediastinitis after a coronary artery bypass; she was successfully treated by combining negative pressure therapy, titanium plates osteosynthesis and bilateral pectoral muscle flaps.

Maturation-dependent sensitivity of oligodendrocyte lineage cells to apoptosis: implications for normal development and disease.

Apoptosis plays a crucial role in brain development by ensuring that only appropriately growing, migrating, and synapse-forming neurons and their associated glial cells survive. This process involves an intimate relationship between cell-cell interactions and developmental cues and is further impacted by environmental stress during neurogenesis and disease. Oligodendrocytes (OLs), the major myelin-forming cells in the central nervous system, largely form after this wave of neurogenesis but also show a selective vulnerability to cell death stimuli depending on their stage of development. This can affect not only embryonic and early postnatal brain formation but also the response to demyelinating pathologies. In the present review, we discuss the stage-specific sensitivity of OL lineage cells to damage-induced death and how this might impact myelin survival and regeneration during injury or disease.

Non-invasive determination of the systolic peak-to-peak gradient in children with aortic stenosis: validation of a mathematical model.

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient = 6.02+/-1.49*(mean gradient)-0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y = 0.67 x +11.11, r = 0.87, SEE = 6 mm Hg, p = 0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y = 0.66 x +14.44, r = 0.84, SEE = 9 mm Hg, p = 0.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.

cis-Determinants in the cytoplasmic domain of CEACAM1 responsible for its tumor inhibitory function.

CEACAM1, also known as C-CAM, BGP and CD66a, is a member of the carcinoembryonic antigen (CEA) family which is itself part of the immunoglobulin supergene family. CEACAM1 is involved in intercellular adhesion, signal transduction and tumor cell growth regulation. CEACAM1 is down-regulated in colon and prostate carcinomas, as well as in endometrial, bladder and hepatic tumors, and 30% of breast cancers. We have shown in a mouse colon tumor model that CEACAM1 with a long cytoplasmic domain inhibited the development of tumors whereas a splice variant lacking the cytoplasmic domain did not. In this study, we define the subregions of the long cytoplasmic domain participating in the tumor inhibition phenotype of CEACAM1. We show that a single point mutation of Tyr488, conforming to an Immunoreceptor Tyrosine Inhibition Motif (ITIM), was sufficient to reverse the in vivo tumor cell growth inhibition. Substitution or deletion of residues in the C-terminal region of the CEACAM1 cytoplasmic domain also led to reversal of tumor cell growth inhibition. This result is in agreement with our previous studies demonstrating the C-terminal region of the cytoplasmic domain influences the levels of CEACAM1 Tyr phosphorylation and its association with the protein Tyr phosphatases SHP-1 and SHP-2. Furthermore, removal of the N-terminal domain of CEACAM1, essential for intercellular adhesion, did not impair the tumor inhibitory effect. These results suggest that Tyr phosphorylation or dephosphorylation of the CEACAM1 cytoplasmic domain represents a crucial step in the control of epithelial cell proliferation.

The carboxyl-terminal region of biliary glycoprotein controls its tyrosine phosphorylation and association with protein-tyrosine phosphatases SHP-1 and SHP-2 in epithelial cells.

Biliary glycoprotein (Bgp, C-CAM, or CD66a) is an immunoglobulin-like cell adhesion molecule and functions as a tumor suppressor protein. We have previously shown that the Bgp1 isoform responsible for inhibition of colonic, liver, prostate, and breast tumor cell growth contains within its cytoplasmic domain two tyrosine residues positioned in immunoreceptor tyrosine-based inhibition motif (ITIM) consensus sequences. Moreover, we determined that these residues, upon phosphorylation, associate with the protein-tyrosine phosphatase SHP-1. In this report, we have further evaluated the structural bases of the association of Bgp1 with Tyr phosphatases. First, we demonstrate that Bgp1 also associates with the SHP-2 Tyr phosphatase, but not with an unrelated Tyr phosphatase, PTP-PEST. Association of Bgp1 and SHP-2 involves the Tyr residues within the Bgp1 ITIM sequences, Val at position +3 relative to the second Tyr (Tyr-515), and the SHP-2 N-terminal SH2 domain. In addition, our results indicate that residues +4, +5, and +6 relative to Tyr-515 in the Bgp1 cytoplasmic domain play a significant role in these interactions, as their deletion reduced Bgp1 Tyr phosphorylation and association with SHP-1 and SHP-2 by as much as 80%. Together, these results indicate that both SHP-1 and SHP-2 interact with the Bgp1 cytoplasmic domain via ITIM-like sequences. Furthermore, they reveal that the C-terminal amino acids of Bgp1 are critical for these interactions.

Lobectomy in the treatment of the scimitar syndrome.

The scimitar syndrome is a congenital anomaly that consists mainly of total or partial anomalous venous drainage of the right lung to the inferior vena cava. We report the case of an 8-year-old girl diagnosed at birth as having a scimitar syndrome and who presented with recurrent right lower lung infections. She underwent resection of the lower half of her right lung without diversion of the anomalous venous drainage of the upper portion of the lung. She improved rapidly postoperatively and the shunt fraction diminished.

Profile of paediatric patients with pulmonary hypertension judged by responsiveness to vasodilators.

OBJECTIVE: To describe the demographic and haemodynamic variables of children presenting with primary pulmonary hypertension or pulmonary hypertension appearing or persisting after surgical correction of congenital heart defects and to assess the acute effect of vasodilator drugs on their pulmonary vascular bed. DESIGN: Retrospective review. SETTING: Paediatric cardiology department and intensive care unit of a large tertiary centre. PATIENTS: Fourteen consecutive patients presenting with primary pulmonary hypertension (group 1) or pulmonary hypertension persisting or appearing late after complete surgical repair (group 2). INTERVENTION: Baseline haemodynamic measurements were taken in room air at rest and repeated in 100% oxygen. With constant monitoring of heart rate and pulmonary and systemic arterial pressures, patients were given serial intravenous, sublingual, or oral incremental doses of vasodilators (mean 4.1 trials per patient). The maximum effect of the drug was charted. MAIN OUTCOME MEASURES: A positive response to acute vasodilator tests was defined as a decrease in mean pulmonary or mean systemic arterial pressure > 15% with the mean pulmonary artery pressure not reaching the systemic level and either no change or an increase in mean systemic arterial pressure. Haemodynamic variables between groups (1 v 2, responders v non-responders, patients experiencing or not experiencing adverse effects to vasodilators) were compared by a two tailed unpaired Student's t test, and their survival curves were compared by the log rank statistic. RESULTS: Groups are small and definitive conclusions are difficult to draw, but the baseline haemodynamic assessments were not significantly different between group 1 and 2 or between responders and non-responders to vasodilators. Patients experiencing adverse effects had a higher pulmonary vascular resistance (p = 0.04) and wedge pressure (p = 0.02) than those without adverse effects. Of the vasodilators used, tolazoline, hydralazine, salbutamol, phentolamine, and phenoxybenzamine were ineffective. A positive response was seen in five of 13 patients given oxygen, in one of eight given prostacyclin, four of 12 given nifedipine, four of eight given diltiazem, one of six given captopril, and two of seven given glyceryl trinitrate. Estimates of survival of the population with primary pulmonary hypertension were 37% at one year and 12% at 2.5 years. Survival was significantly shorter in the non-responders than in the responders (p = 0.005). CONCLUSION: Children with primary pulmonary hypertension present to the cardiologist at a young age (five of eight were younger than 7 years) but with advanced pulmonary vascular disease and have a poor prognosis. 64% of group 1 and group 2 patients had a positive response to acute treatment with at least one vasodilator. Calcium channel blockers were the most effective agents. There was a positive response to drugs despite a negative response to acute treatment with oxygen. The survival of non-responders was shorter than that of the responders.

A case report of double-orifice tricuspid valve.

A heart specimen with double-orifice tricuspid valve is described. The tricuspid valve was divided into the anterior and posterior orifices by a bridge of leaflet tissue. The valve was stenotic because of the arcade deformity of the anterior papillary muscle to which the bridging leaflet tissue had short chordal insertions. The anterior orifice was regurgitant as a result of a deficient septal leaflet. Clinical correlation was obtained by magnetic resonance imaging of the specimen. The functional significance of the malformed valve was assessed by three-dimensional reconstruction of the two-dimensional magnetic resonance images.

Transcatheter closure of Blalock-Taussig shunts with a modified Rashkind umbrella delivery system.

Transcatheter occlusion of Blalock-Taussig shunts was successfully performed in three children with the Rashkind double umbrella occluding device. Complete occlusion was confirmed angiographically in two patients and echocardiographically in the third. The delivery system was adapted for use through smaller sheaths making it more suitable for small children or arterial access.

Percutaneous pericardial catheter drainage in childhood.

The clinical course of 41 consecutive pediatric patients (mean age 7.6 +/- 5.8 years, weight 27 +/- 22 kg) who underwent percutaneous pericardial drain placement during a 3-year period were reviewed. The most common diagnoses were malignancy (20%), postpericardiotomy syndrome (17%), aseptic pericarditis (12%), and patients recovering from a Fontan type of operation (12%). Indications for drainage included increasing effusion size determined by 2-dimensional echocardiogram (48%), clinical deterioration (33%) and echocardiographic evidence of hemodynamic compromise (12%). Only 3 (7%) patients had clinical evidence of cardiac tamponade. Drainage catheter placement was accomplished percutaneously from the subxiphoid approach. Insertion was successful in all but 1 patient (98%) and successful evacuation of the pericardial space was achieved in 93% of patients. There was 1 death in a critically ill 2-week-old infant and 4 complications, 3 of which occurred in patients aged less than 2 years. Drainage catheters remained in position from 1 to 18 days (mean 3 +/- 3 days) with no late complications. There were 3 instances (7%) of drainage catheter occlusion. These data support the notion that placement of a percutaneous pericardial catheter is safe and effective in providing definitive drainage of the pericardial space in the pediatric age group. Children younger than age 2 years may be at increased risk for complications.

Balloon atrial septostomy under transesophageal echocardiographic guidance.

Balloon atrial septostomy is an established method of palliation for several forms of congenital heart disease. Previously performed under fluoroscopic x-ray control, recent reports have demonstrated the utility of transthoracic echocardiographic monitoring. We report the first application of uniplane transesophageal echocardiography (TEE) (6.7-mm probe) as an alternative imaging modality for control of balloon atrial septostomy on neonates in the intensive care unit.

Adolescent contraceptive counseling.

PIP: Adolescents need total counseling in the area of sexual matters. Th is counseling is a social, mental, and physical need. Adolescents today are more interested in satisfying sexual relationships and need more from a counseling situation than contraceptive advice and supplies. Medical and nursing school curricula do not provide adequate training in sex counseling. Doctors and nurses should make themselves comfortable and sophisticated in the field. A team approach, using male and female counselors, is recommended. Counseling could be provided in many different seetings: 1) student health services, 2) mental health centers, 3) periodic clinics, and 4) community health clinics.^ieng