Effect of diazoxide on oxygen free radicals and cell apoptosis in brain tissue after deep hypothermia cerebral ischemia reperfusion injury in young rats / 中华外科杂志

<p><b>OBJECTIVE</b>To determine the effects of diazoxide on oxygen free radicals and cell apoptosis in brain tissue after deep hypothermia cerebral ischemia reperfusion injury in young rats.</p><p><b>METHODS</b>Fifty-four 3-week-old Sprague-Dawley rats were randomly and equitably divided into sham-operated group, model group and diazoxide group respectively (n = 18). The model of hypothermia cerebral ischemia reperfusion injury was made. After 24 hours of operation, the brains of rats were removed and preserved. The content of superoxide dismutase (SOD) and malonaldehyde (MDA) in brain tissue were detected. Cytosolic C release of cytochrome was confirmed by Western Blot. The protein expression of Caspase-3 was determined by immunohistochemistry.</p><p><b>RESULTS</b>In the model group, the content of SOD was (198 +/- 41) U/mg, lower than the sham-operated group's (321 +/- 36) U/mg (P < 0.01). The content of MDA was (212 +/- 21) nmol/mg, was higher than the sham-operated group's (100 +/- 23) nmol/mg (P < 0.01), and the expressions of cytochrome C (0.72 +/- 0.09) and Caspase-3 (83 +/- 10) were all significantly higher than those in the sham-operated group (0.17 +/- 0.02 and 115 +/- 9) (P < 0.01). Compared with the model group, the content of SOD in the diazoxide group [(264 +/- 34) U/mg] was markedly increased (P < 0.05). In addition, diazoxide provided significant reductions in the content of MDA [(174 +/- 19) nmol/mg] and the expressions of cytochrome C (0.41 +/- 0.05) and Caspase-3 (99 +/- 11) (P < 0.05).</p><p><b>CONCLUSIONS</b>The neuroprotective effects of diazoxide against brain injury induced by deep hypothermia cerebral ischemia reperfusion through inhibiting oxygen free radicals and cell apoptosis. Diazoxide may become a new neuroprotective drug after infant complicated congenital cardiac operation.</p>

The individualized surgical treatment of transposition of the great arteries / 中华外科杂志

<p><b>OBJECTIVE</b>To discuss the effectiveness of individualized strategy of surgical management on the great arteries (TGA).</p><p><b>METHODS</b>From March 1998 to October 2009, 127 cases (97 males and 30 females) with TGA were treated. There were 97 male and 30 female, aged from 4 hours old to 17 years old with a mean of (25 ± 37) months, weighted from 2.7 to 47.5 kg with a mean of (8 ± 8) kg. The palliative operations included Glenn operation in 14 cases (3 cases double Glenn procedure), Balalock-Taussing shunt in 14 cases, Banding operation in 8 cases, and atrial septal defect enlarge/Banding/Balalock-Taussing shunt in 15 cases. The end-stage operation included Senning procedure in 5 cases, Switch procedure in 32 cases, 2(nd)-stage Switch procedure in 11 cases, Switch procedure with VSD repairing in 20 cases, Switch procedure with Hybrid in 1 case, Nikaidoh procedure in 3 cases, Rastelli procedure in 13 cases, Fonton procedure in 18 cases, other procedure in 4 cases. Twenty-one cases underwent 2 operations, and 5 cases underwent 3 or more operations. Sixty-six cases underwent delayed sternal closure.</p><p><b>RESULTS</b>There were 12 cases of death operatively in 127 cases. The total operative mortality was 9.4%. There were 5 cases dying of low cardiac output during the operation, 2 of pulmonary hypertension crisis, 2 of hemorrhage, 1 of grafting problem of coronary artery deformation, 1 of renal failure after Fonton procedure and 1 case of newborn dying of spontaneous rupture of liver post-operatively. The patients were followed up for 1 month to 12 years. There were 10 patients with vary degrees complications such as pulmonary stenosis, residual shunt and narrow channel. Three cases underwent reoperation. The rest of survived cases had normal heart function, good growth and development state.</p><p><b>CONCLUSIONS</b>Individualized strategy of surgical management based on anatomical conditions of TGA can significantly improve the success rate of surgery and long-term survival.</p>

Diagnosis of 22q11 deletion and duplication in congenital heart disease by multiplex ligation dependent probe amplification / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical utility of multiplex ligation-dependent probe amplification (MLPA) for detecting 22q11 deletion and duplication in congenital heart disease (CHD) cases and to study the incidence of 22q11 deletion and duplicaton in different kinds of CHD.</p><p><b>METHODS</b>Forty eight probes of which 25 located in 22q11 low copy number region (LCR 22s A-H), 7 in 22q11 surrounding region (CES, 22q13) and 16 in chromosomes 4, 8, 10 and 17 were selected to detect 22q11 deletion and duplication in 181 preoperative children with CHD and 14 fetuses with serious CHD or CHD with multiple malformations. In these cases, karyotype analysis was also performed.</p><p><b>RESULTS</b>MLPA demonstrated that 7 cases had 22q11 deletion [6 cases from CLTCL1 to LZTR1(LCR A-D) and 1 case from CLTCL1 to PCQAP (LCR A-C)] and that 1 case had 22q11 duplication,spanning from ZNF74 to LZTR1(LCR B-D). The phenotypes of heart defect included ventricular septal defect, atrioventricular septal defect, pulmonary stenosis and tetralogy of Fallot. Karyotype analysis showed that 1 case had 21q deletion [46, XY, 21q], 1 case had mosaic trisomy 8 [47,XY, +8/46, XY(1:2)] and 4 cases had trisomy 21. One of the 4 cases with trisomy 21 had concurrent 22q11 duplication.</p><p><b>CONCLUSIONS</b>MLPA is a rapid, sensitive, site specific and relatively inexpensive method for diagnosis of 22q11 deletion and duplication in CHD. 22q11 deletion and duplication may cause various kinds of CHD, suggesting that genetic detection should be performed routinely in CHD patients.</p>

Detection and related analysis to chromosome 22q11 microdeletion in patients with congenital heart diseases / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To ascertain 5 short tandem repeat (STR) markers as qualified tools for detecting chromosome 22q11 deletion and to understand the prevalence and clinical importance of the deletions in patients with congenital heart diseases (CHD) from Chinese Han population.</p><p><b>METHODS</b>The authors selected 5 new tetranucleotide repeat markers, 22D_4_1, 22D_4_2, 22D_4_3, 22D_4_4 and D22S873 located in the proximal region of chromosome 22q11 deletion. One hundred and sixty-three unselected CHD patients and their unaffected parents were analyzed by genotyping of these new tetranucleotide STR markers to detect 22q11 deletion. With fluorescence in situ hybridization (FISH, LSI dual color DNA probe), the deletion status was confirmed in all patients with deletions and some patients without deletions.</p><p><b>RESULTS</b>The heterozygosity of these STR markers in normal population was more than 0.7, except for 22D_4_1 and 22D_4_2 that were 0.65 and 0.52 respectively. Twelve cases of 163 CHD patients (7.36%) had the deletions at chromosome 22q11. The deletions were confirmed in 9 of 12 patients by FISH, except for 2 cases who had unique nested deletion and 1 case who had nested distal deletion. One hundred and ten patients were associated with ventricular septal defect (VSD); and 9 (8.18%) of these cases had microdeletion. Twenty-one patients were associated with tetralogy of Fallot (TOF); and 3 (14.3%) of these cases had microdeletion.</p><p><b>CONCLUSION</b>This study demonstrated that genotyping of 5 STR markers was a useful mean of detecting 22q11 microdeletion in clinical diagnosis owing to its rapid experimental procedure, cost effectiveness and high resolution. 22q11 deletion was common in CHD patients, particularly in VSD and TOF patients, from Chinese Han population.</p>

Clinical application and experimental studies of the pulsed inhaled nitric oxide flow controlling instrument / 中国医疗器械杂志

A flow controlling system for pulsed inhaled nitric oxide has been developed and tested, and here its features and initial animal experiments and clinical applications are described. The physical characteristic test indicates that the practical released dose of NO gas is very close to the theoretical flow of NO gas at variant pressures. Animal experiments demonstrate that inhaled NO gas concentration is lower than the concentration of theoretical inhalation, but the variance is not remarkable (p>0.05). When sixteen cases with CHD and PH were chosen to inhale NO gas (15 ppm, 15 min) PAP and PVR of all cases were reduced after inhalation of NO gas from 617 +/-51.3 dyn x s x cm(-5), 54.4+/-13.1 mmHg to 417+/-36.9 dym x s x cm(-5), 33.8+/-12.3 mmHg (PVR, p<0.01; PAP, p<0.01) respectively. When gas inhalation was stopped, these values returned to their base lines after a short period of time. All these show that the pulsed inhaled NO flow controlling instrument in accordance with the requirements of the designing, can be widely used in clinical diagnoses and treatments and will be a new tool offered for the treatments of the patients with PH.