2010年 · 第123卷第19期

In recent years, great progress has been made in the field of pediatric cardiology in China, including the development of diagnostic techniques and patient management and the elucidation of mechanisms responsible for cardiovascular diseases.1 The standardization of key diagnostic skills and patient management pathways, for example, has been continually improved.At the same time, the pathogenesis of pediatric cardiovascular diseases has been studied in depth,resulting in an increasing body of novel research findings with regard to the mechanisms responsible for these diseases. We must be aware, however, that great effort still needs to be made to further explore new diagnostic and treatment strategies and reveal the mysteries of cardiovascular diseases。

Abstract：Background The epidemiologic pictures of Kawasaki disease (KD) in Shanghai from 1998 through 2002 were reported,while the current status of KD in the following five years remains unknown.Methods A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited.Results In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 (mean 46.32±6.51) per 100 000children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of (27.32±7.11) per 100 000, (t=4.406, P=0.002). Ages at onset ranged from 12 days to 13.6 years (median 1.8 years). It occurred more frequently in summer and spring. Coronary arterial lesions (CAL), defined as ectasia or aneurysm,accounted for 19.8% (232 cases). Flattened or inverted T wave was the most frequent finding (194 cases, 20.5%) by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases (86.6%). The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice.Conclusions The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary sequelae。

Abstract：Background Syncope is a common clinical problem with multiple causes. Vasovagal syncope (WS) is by far the most frequent cause of syncope in children and adolescents. The traditional diagnostic approach to VVS of children and adolescents is based on a series of tests to exclude all other causes, which is complex and time and medical resource consuming. Attempts have been made to develop a new cost-effective diagnostic approach to avoid these problems. This study aimed to compare the economic effectiveness and diagnostic value of the traditional diagnostic approach to VVS of children with a new diagnostic approach.Methods One hundred and eighteen children diagnosed as VVS were divided into two groups according to the different diagnostic approaches. The diagnostic value of the two diagnostic approaches was then analyzed. Meanwhile, the costs of hospitalization, diagnostic testing and hospital stay were determined. Data were evaluated by the cost-minimization analysis.Results The diagnostic value of the new diagnostic approach was similar to that of the traditional diagnostic approach (56.57% vs. 53.91%, P=0.697). However, the cost of hospitalization per patient by the new diagnostic approach was (1507.08±144.63) Yuan (RMB) which was less than that of the traditional diagnostic approach (2603.64±P08.19) Yuan.The costs of diagnostic tests per patient by the new diagnostic approach was (1256.04±109.14) Yuan and by the traditional approach (2175.22±153.32) Yuan.Conclusion Compared to the traditional diagnostic approach to diagnose VVS in children and adolescents, the new diagnostic approach is of a good economic value, and it should be popularized in clinical practice。

Abstract：Background Respiratory distress syndrome (RDS) is one of the most common causes of neonatal respiratory failure and neonatal death, however, its clinical characteristics are very different from premature RDS, and these characteristics have not been well documented as yet. This study was to investigate the pathogenesis, clinical characteristics and management strategies of RDS in full-term neonates, with the aim of developing a working protocol for improving the outcome in full-term neonates with RDS.Methods A total of 125 full-term infants with RDS were enrolled in this study. Their clinical and laboratory data were collected for analyzing the characteristics of full-term neonatal RDS.Results (1) The 125 cases included 94 male and 31 female infants, vaginal delivery occurred in 80 cases and cesarean section in 45 cases. (2) The onset time of RDS was (3.11±3.59) hours after birth. (3)The possible reasons included severe perinatal infections in 63 patients, elective cesarean section in 34 cases, severe birth asphyxia in 12 patients,meconium aspiration syndrome in 9 patients, pulmonary hemorrhage in 4 patients and matemal diabetes in 3 patients. (4)Complications included multiple organ system failure (MOSF) in 49 patients, persistent pulmonary hypertension of newborn (PPHN) in 25 patients, acute renal failure in 18 patients, severe hyperkalemia in 25 patients, severe metabolic acidosis in 6 cases, severe myocardial injury in 9 cases, pulmonary hemorrhage in 3 cases, disseminated intravascular coagulation in 14 patients and shock in 12 patients.(5) Four patients died, the mortality was therefore 3.2% with the main cause of septicemia complicating of MOSF, but their prognosis was improved while comprehensive treatment measures including early mechanical ventilation and broad spectrum antibiotics were taken into account.Conclusions RDS is not an uncommon disease in full-term infants and is associated with a higher mortality, its clinical characteristics are very different from premature RDS, and its onset is earlier and is more likely to develop into PPHN and/or MOSF. The main cause of death is severe infection complicating of MOSF and most patients require prolonged mechanical ventilation. Comprehensive management strategies will help to improve patient's prognosis。

Abstract：Background Pulmonary artery perfusion during cardiopulmonary bypass (CPB) is a novel adjunctive method, which can minimize the lung ischemic-reperfusion injury and inflammatory response. This study evaluated the protective effect of pulmonary perfusion with hypothermic HTK solution in corrections of congenital heart defects with pulmonary hypertension.Methods Between June 2009 and December 2009, 24 consecutive infants with congenital heart defects and pulmonary hypertension were randomly divided into perfused group (n=12) and control group (n=12). Oxygen index, alveolar-arterial O2gradient, serum levels of malondialchehyche (MDA), interleukin (IL)-6, -8, -10, soluble intercellular adhesion molecule-1(slCAM-1), and P-selectin were measured before commencement and serially for 48 hours after termination of bypass.Results Oxygenation values were better preserved in the perfused group than in the control group. The serum levels of IL-6 increased immediately after CPB in both groups and returned to baseline at 48 hours after CPB, but it was restored faster and earlier in the perfused group. The serum levels of IL-8, slCAM-1, and MDA remained at baseline at each point after CPB in the perfused group and elevated significantly immediately after CPB in the control group, except for sICAM-1.The serum level of IL-10 increased immediately after CPB and decreased to baseline at 48 hours after CPB in both groups, but the IL-10 level in the perfused group was significantly higher than in the control group at 12 hours after CPB.The serum P-selectin levels in the control group immediately after CPB were significantly higher than prebypass levels.Moreover, there were no significant differences in postoperative clinical characters, except for the intubated time.Conclusion In infants with congenital heart defects, pulmonary perfusion with hypothermic HTK solution during cardiopulmonary bypass could ameliorate lung function and reduce the inflammatory response。

Abstract：Background The first case of pandemic influenza A (H1N1) virus infection in Macau Special Administrative Region (SAR) of the People's Republic of China was documented on June 18, 2009. Subsequently, persons with suspected infection or of contact with suspected cases received screening. All the confirmed cases were hospitalized and treated with oseltamivir. Their clinical features were observed. This may help for better management for later patients and be of benefit to the government of Macau SAR to adjust its strategy to combat the pandemic influenza A (H1N1) virus infection more efficiently.Methods From June to July 2009, the initial 72 cases of influenza A (H1N1) in Macau were hospitalized in Common Hospital Centre S. Januario (CHCSJ). The infection was confirmed by real-time reverse-transcriptase polymerase chain reaction (RT-PCR). The clinical features of the disease were closely observed and documented. Oseltamivir was given to all patients within 48 hours after the onset of disease and maintained for 5 days.Results The mean age of the 72 patients was 21 years old. Forty of them were men and 32 were women. The median incubation of the virus was 2 days (1 to 7 days). The most common symptoms were fever (97.2%) and cough (77.8%).The rate of gastrointestinal symptoms including nausea, vomiting, and diarrhea was 2.8%. Fever typically lasted for 3days (1 to 9 days). The median time from the onset to positive results of real-time RT-PCR was 6 days (3 to 13 days).After treatment with oseltamivir, most patients became afebrile within 48 hours. Only one aged patient with a history of glaucoma and hypothyroidism was found to have lung infiltration on chest X-ray.Conclusions The initial cases of pandemic influenza A (H1N1) virus infection in Macau SAR showed that most of the infected persons had a mild course. The virus could be detected by real-time RT-PCR within a median of 6 days from the onset. Oseltamivir was effective。

Abstract：Background There are numerous articles on the endothelial progenitor cells (EPCs) in different disease conditions.However, the functional properties of EPCs in acute coronary syndrome (ACS) are still uncertain. Here we aimed to study the number and functions of EPCs in ACS patients.Methods Patients were enrolled with admitted ACS (n=25) and another 25 gender-, age-, atherosclerotic risk factors-matched stable coronary artery disease (CAD) controls. EPCs were defined as CD34+/CD133+/VEGFR-2+ and quantified by flow cytometry. Moreover, functional properties of EPCs including colony-forming unit (CFU), proliferation,migration as well as apoptosis were evaluated and compared between the two groups. Plasma matrix metalloproteinase-9 (MMP-9) was detected in all patients as well.Results The two groups had similar medication and clinical characteristics on admission. The EPCs in ACS patients were more than 2.6 times that in stable CAD subjects (15.6±2.7 vs. 6.0±0.8/100 000 events, P ＜0.01). CFU was not statistically different between the two groups (10.8±2.9 vs. 8.2±1.8, number/well, P ＞0.05). Furthermore, EPCs isolated from ACS patients were significantly impaired in their proliferation (0.498±0.035 vs. 0.895±0.067, OD value, P ＜0.01) and migration capacity (20.5±3.4 vs. 30.7±4.3, number/well, P ＜0.01) compared with controls. Moreover, the apoptosis cell in cultured EPCs was drastically increased in ACS group ((18.3 ±2.1 )% vs. (7.8±0.4)%, P ＜0.01 ).Conclusions Patients with ACS exhibited apparently increased circulating EPCs as well as cultured apoptosis percentage together with a remarkable impairment of proliferation and migration activities compared with stable CAD subjects。

Abstract：Background The blood supply to the eye comes from the retinal central vascular system of the ophthalmic artery and the ciliary vascular system. The ophthalmic artery stems from the ipsilateral internal carotid artery. If occlusion or stenosis occurs in the carotid artery, the blood perfusion to the ophthalmic artery becomes insufficient, leading to signs and symptoms of anterior and posterior ocular ischemia. The objective of this study was to evaluate the clinical characteristics and risk factors of ocular ischemic diseases caused by carotid artery stenosis.Methods This study was a retrospective review of 145 patients with carotid artery stenosis. Fifty-eight patients who had symptoms of ocular ischemic disease caused by carotid artery stenosis formed group A and the other 87 patients who only had carotid artery stenosis formed group B. We analyzed the causes and course of disease, and relative risk factors,by comparing the two groups.Results The degree of carotid artery stenosis in group A was higher than that in group B. And group A had a greater decrease of ophthalmic artery flow. Male, hypertension, hyperlipidemia, and smoking were significantly related to carotid artery stenosis. Amaurosis fugax was the most common ocular symptom in group A. The ocular ischemic diseases mainly included ischemic optic neuropathy, central/branch retinal artery occlusion, ophthalmoplegia externa, and ocular ischemic syndrome.Conclusions Carotid artery stenosis correlates with ocular ischemic diseases. Ophthalmologists must observe for ocular symptoms, which were the onset symptoms in some patients。

Abstract：Background Severe valgus deformity often has bone defect and laxity of the medial ligamentous, and total knee arthroplasty in severe valgus knee is, in most cases, more challenging for surgeons. The usefulness of a computer assisted navigation system in reestablishing the mechanical axis has been well established. Hence, the interest for surgeons is how the navigation system makes the procedure of total knee arthroplasty with severe valgus knee easier.Methods From June 2006 to March 2008 in Department of Joint Surgery, Shanghai Sixth People's Hospital, 6 patients (7 knees) with severe valgus knee underwent total knee arthroplasty using the Stryker Navigation system, which is an active wireless and imageless system. All the patients were followed up for 12 to 18 months after surgery. The X-ray radiographs for whole limbs were obtained on all patients to determine preoperative and postoperative alignments.Results A primary, posterior stabilized prosthesis was utilized in all cases. The average preoperative overall mechanical axis of the seven knees was 19.6°±4.6° of valgus (range 16° to 29°), and the average postoperative mechanical axis was 0.4°±0.7° (range 0.8° varus to 1.4° valgus ).Conclusions The navigation system is a very effective and useful tool for accurate intraoperative restoration of alignment in the face of significant deformity with valgus knee. To prevent component malposition, we did not reduce the knee before solidification of bone cement but controlled alignment using the navigation system up to implantation of the final component。

Abstract：Background Although the locking plate has become popular for treating displaced proximal humeral fractures in recent years, the complications of this method are still underestimated. We tried to evaluate the factors that have an influence on the complications experienced after proximal humeral fractures fixed by locking plates and compare the results from patients having complications with those having no complications.Methods From September 2004 to September 2007, 92 out of 111 displaced proximal humeral fractures treated by open reduction and internal fixation with a locking plate were available for follow-up, with an average time of 15.2months (12-36 months). The range of motion, Visual Analog Score (VAS) for pain, American Shoulder and Elbow Surgeons' Form (ASES), Constant-Murley, University of California-Los Angles scoring system (UCLA) score, and Simple Shoulder Test (SST) for function evaluation was all recorded at the latest follow-up. The results from patients with complications were evaluated according to the indices listed above and compared with those patients without any complications.Results There were 17 patients with complications, an 18.5% complication rate. Among them, the forward flexion,external rotation and internal rotation were 139.1°±24.3°, 24.1°±19.6°, and up to T10 level on average. The mean VAS score was 1.0±1.1, the ASES score was 82.9±13.8, the Constant 82.1±11.8, the UCLA 28.5±4.1 and the mean SST 9.5on average. There was no significant difference of complication rate among different age, sex, and injured side, fresh or delayed fracture, combined with other injury or not groups. Compared with the group without complications, patients with complications showed significantly less external rotation and lower Constant-Murley and UCLA functional scores (P ＜0.05). A significant difference in results was seen between patients with complications and those without complications.Conclusion The indication control and appropriate surgical technique were important while performing the locking plate fixation for proximal humeral fractures。

Abstract：Background Allergic asthma is associated with airway inflammation and hyperresponsiveness caused by dysregulated production of cytokines secreted by allergen-specific helper T-type 2 (Th2) cells. The linker for activation of T cells (LAT)is a membrane-associated adaptor protein, which has been shown to take part in regulating T cell receptor (TCR)signaling and T cell homeostasis. In this study, we established an asthmatic mouse model to examine the changes in LAT levels during allergic airway disease and the effects of LAT transgenic expression on airway inflammation.Methods T ceils from mouse lung tissues were isolated from allergen challenged (ovalbumin (OVA)) and control mice,and the purity of these isolated T cells was examined by fluorescence-activated cell sorter (FACS). Semi-quantitative RT-PCR and Western blotting were used to detect the expression of the LAT gene and LAT protein, respectively. After an intranasally administered mixture of pCMV-HA-LAT plasmid and Lipofectamine 2000, 24 hours before and 72 hours after allergen challenge, the BALF cell count and the differential cytologies were studied. In addition, IL-4 and IFN-γ levels in the BALF were determined by ELISA, and pathological changes in lung tissues were observed.Results LAT protein and mRNA expression were decreased in lung T cells in a mouse model of allergen-induced airway disease. After intranasal administration of pCMV-HA-LAT, histopathological examination of the lungs showed that intervention with LAT overexpression prevented mice from developing airway inflammation, and the number of total cells,eosinophils, neutrophils, and lymphocytes in the BALF was reduced significantly compared with the OVA sensitized and challenged group. In addition, the Th2 cytokine IL-4 decreased, while the Th1 cytokine IFN-Y increased compared to the OVA sensitized and challenged group or the OVA sensitized group plus pCMV-HA treatment.Conclusion This study demonstrates that LAT might effectively diminish Th2 cytokine responses, lung histopathological changes and lung inflammation to allergen challenge in a model of experimentally induced asthma。

Abstract：Objective A general review was made of studies involving: (1) the concept and mechanism of the cholinergic anti-inflammatory pathway (CAP), (2) the important role of inflammatory response in myocardial ischemia reperfusion (I/R)injury and (3) the evidence and mechanisms by which CAP may provide protection against myocardial I/R injury.Data sources The data used in this review were mainly from manuscripts listed in PubMed that were published in English from 1987 to 2009. The search terms were "vagal nerve stimulation", "myocardial ischemia reperfusion injury","nicotine acetylcholine receptor" and "inflammation".Study selection (1) Clinical and experimental evidence that the inflammatory response induced by reperfusion enhances myocardial I/R injury. (2) Clinical and laboratory evidence that the CAP inhibits the inflammation and provides protection against myocardial I/R injury.Results The myocardial I/R injury is really an inflammatory process characterized by recruitment of neutrophils into the ischemic myocardium and excessive production of pro-inflammatory cytokines. Because the CAP can modulate the inflammatory response by decreasing the production and release of pro-inflammatory cytokines, it can provide protection against myocardial I/R injury.Conclusions The CAP can inhibit the inflammatory response induced by reperfusion and protect against myocardial I/R injury. It represents an exciting opportunity to develop new and novel therapeutics to attenuate the myocardial I/R injury。

Spondyloepiphyseal dysplasia (SED) comprises a group of hereditary disorders caused by osteochondrodysplasia of the spine and long tubular bones owing to chromosome abnormalities,1 SED can be divided into two major types, termed SED congenita and SED tarda, according to the time of onset, and both types have some particular characteristics. SED is a rare genetic disorder with an incidence of 1-4 per million population,2 and is prone to be confused with other conditions of short stature. We collected and analyzed the clinical data for 6 patients with SED who were admitted to Peking Union Medical College Hospital for treatment between May 1995 and January 2006 in order to provide a relatively comprehensive recognition of this disease for doctors and to facilitate the definite diagnosis among patients with dwarfism。

In the western countries, circumcision has been performed for a long time. However, because of religious and cultural differences, circumcision was rare in China before the 20th century. As a result, penile cancer, accounting for 17% of all cancers, was more common.1 With the introduction of western medicine in the 1920s, especially after the establishment of urology as a separate department, gradually more circumcisions were carried out. The incidence of penile cancer was also reduced to 0.34/100 000, which was similar to Western developed countries.1 Recently, in China, circumcision has become one of the most basic operations in urological surgery. More people have realized the efficacy of circumcision in preventing acquisition and transmission of sexually transmitted infections.2,3

Symptomatic arteriovenous fistula (AVF) with pseudoaneurysm after percutaneous renal biopsy is an uncommon anomaly, occurring from 0.34% to 6.3%.1Most of these vascular lesions are of little clinical importance. However, severe bleeding,2 persistent hematuria, or acute urinary retention may occur, requiring treatment. Here we report a case of gross hematuria and acute urinary retention after renal biopsy in a male patient.An arteriovenous fistula with pseudoaneurysm was detected by renal ultrasound, confirmed by angiography and then successfully treated by transcatheter arterial embolization3 without damage to renal parenchyma。

Currently coronary artery bypass grafting (CABG) is the most commonly used procedure for revascularization of coronary heart disease. However it may not be suitable for the patients with diffuse coronary artery diseases. Under this circumstance, retrograde perfusion via cardiac venous system, namely retrograde coronary venous bypass graft (CVBG), may be the proper therapeutic selection.1 The idea of myocardial revascularization by means of grafting the coronary venous system is more than a century old. However, few clinical trials and long-term outcome data have been presented. The use of venous arterialization has not been widely used. We report the use of a sequential saphenous vein graft from the aorta to middle cardiac vein in a patient with diffuse lesions in right coronary artery during off-pump coronary artery bypass surgery (OPCAB)。

Coronary artery perforation is a life-threatening complication during percutaneous coronary intervention (PCI) although it occurs rarely in contemporary era with incidence ranging from 0.1% to 0.8%.1-8 The overall mortality remains quite high and varied with the type of perforation, with higher risk in type Ⅱ and Ⅲ.2-5 Previous studies have demonstrated that some therapeutic measures can effectively seal the perforation including prolonged balloon inflation,implantation of polytetrafluoroethylene (PTFE)-covered stents, embolization with microcoils, gelfoam or polyvinyl alcohol, and coronary artery bypass grafting (CABG)。

Macrodystrophia lipomatosa is a rare form of congenital localized gigantism, which is characterized by slowly progressive overgrowth of the mesenchymal elements, especially the fibroadipose tissue,in a limb. Frequently, multiple contiguous digits are involved on the lateral side of the hand or the medial side of the foot. Overabundance of the fibroadipose tissue and osseous overgrowth are most remarkable at the distal ends of the extremity. Macrodystrophia lipomatosa involving an entire limb has been previously reported for only one case in a 45-year-old adult male;1 however, no details of treatment were described. Here, we present the first report of successful limb salvage surgery of macrodystrophia lipomatosa in an adolescent involving an entire upper extremity。

Basal cell carcinoma (BCC) is the most common cancer of the skin. However, BCC occurring on non-sun-exposed sites, especially the genital regions such as scrotum and labia, are very rare. An estimated annual incidence of basal cell carcinoma of the scrotum is 1 per 1 000 000 population.1 Here, we report a man with scrotal BCC with the lesion for 51 years, the longest one in the documents. He was successfully treated by circumferential excision。

Papillary thyroid carcinoma (PTC) is the most common type of well-differentiated thyroid cancer and is considered to be a relatively indolent tumor in which distant metastasis and death are rare.1 The metastasis of PTC is usually to regional lymph nodes, especially the cervical and mediastinal nodes. Metastases to the brain are rare, and constitute 0.1%-5.0% of distant metastases of papillary carcinoma.2 Skull metastasis is uncommon,and is found in 2.5%-5.8% of cases of thyroid cancer.3 Skull base metastasis of PTC is extremely rare, with only eight reported cases in the literature involving the clivus,cavernous sinus, sella turcica, and the petrous apex and ridge.4 A metastatic lesion can arise from histologically benign and silent thyroid neoplasms,5 and differential diagnosis between ectopic and metastatic thyroid cancer can be difficult,6 therefore, the management of thyroid cancer is controversial after removal of metastatic disease.Here we reported a case of papillary thyroid microcarcinoma (PTMC) that was manifested as a solitary clivus metastasis and discussed the diagnostic and therapeutic management strategies. The 73-year-old patient presented with visual impairment in the right eye,and epistaxis. The patient subsequently underwent complete total thyroidectomy after a diagnosis of papillary microcarcinoma. This finding emphasizes that clinically significant metastases can arise from thyroid papillary microcarcinoma. Total thyroidectomy and careful review of the histology are necessary to prevent delay in proper diagnosis。