Volume 91  ·   Issue 42  ·  2011

Parodi's outstanding contribution in the field of endovascular repair of abdominal aortic aneurysm (EVAR) has been widely recognized by academic circles, which can be said to be of epoch-making significance [1]. Just as there is a saying in Zuo Zhuan, "The supreme has virtue, the second is meritorious service, and the second is established. Although it is not abandoned for a long time, this is immortal". In the mid-late 1990s, EVAR entered China and rapidly entered a stage of rapid development. At present, the literature we have searched shows that the first case of EVAR in China was reported in 1998 [2]. With the help of domestic and foreign experts, various large vascular surgery centers in China took the first "small steps". This "small steps" era basically completed the primitive accumulation around 2000, and the first 100 cases of EVAR were successively completed around 2005 [3-7]. It should be said that the development of EVAR in China is almost synchronized with the popularization and promotion of vascular surgery in China. With the rapid development of vascular surgery and the popularization of continuing education in China, EVAR technology, which could only be completed in large hospitals, has penetrated into more primary hospitals, further driving the development of vascular surgery in China.

Objective To explore the diagnosis and treatment of spontaneous isolated middle aortic dissection(SIMAD).Methods The clinical data of 7 SIMAD patients admitted into our hospital between January 2007 and June 2011 were retrospectively analyzed.Results There were 3 males and 4 females with an average age of 53 years old.Five patients had an acute onset.According to the original tear location and flow of celiac artery,6 patients received endovascular repair and another patient underwent abdominal aortabilateral-femoral artery bypass with artificial graft.All cases were successfully treated.Six patients recovered well from the procedures.One patient suffered acute coronary artery syndrome at Day 2 after endovascular treatment.After transferal into cardiology unit,emergency percutaneous transluminal coronary angioplasty was performed with a satisfactory outcome.The postoperative examinations of CTA (computed tomography angiography) of all patients demonstrated that the false lumen of dissection was basically thrombolized without endoleak or stent migration.Conclusion Vascular surgeons should pay more attention to SIMAD with the potentials of rupture and aneurysm.Endovascular graft exclusion may be a first-line therapy as long as the anatomical conditions allow.However,longer follow-up and further evaluations should be performed to determine the long-term benefits.

Objective To establish a patient-specific hemodynamics model of thoracic aortic aneurysm (TAA) based on computational fluid dynamics technique and investigate its role in the study of growth and rupture mechanism of TAA.Methods 3D realistic model of thoracic aortic aneurysm was reconstructed from DICOM format computed tomography angiography (CTA) images of a male patient.The geometry was reconstructed using medical image processing software Mimics.The blood flow in aorta was assumed to be laminar and incompressible and the blood Newtonian fluid.A time-dependent pulsatile boundary condition was deployed at inlet.Unsteady blood flow simulation was performed in real geometry of TAA with a finite volume method (FVM) code FLUENT.The hemodynamic parameters related with the growth and rupture of aneurysm were analyzed.Results Analysis of the distributions of hemodynamic variables during the cardiac cycle such as wall shear stress,streamlines and velocity profiles in TAA were carried out.The numerical simulation results demonstrated that the blood velocity of proximal neck was considerably faster than that of aneurysm body.The outerwall of proximal aneurysm body was hit by blood jet entering aneurysm.Instant streamlines at peak systole showed that the entering blood stream hit the aneurysm wall and right-hand helical vortex was observed in aneurysm body.The distributions of high wall shear stress were observed in the proximal and distal aneurysm neck and the area where the entering blood stream first hit the wall.Large regions of lower wall shear stress occurred in aneurysm body.Conclusion The growth and rupture mechanisms of TAA may be analyzed based on a constructed patient-specific model and hemodynamic simulation.

Objective To summarize the surgical experiences of treating 61 cases of non-traumatic aneurysms in ilio-femoral arterial region.Methods The clinical data of 61 consecutive patients with nontraumatic aneurysms in ilio-femoral arterial region between January 1985 and November 2010 were retrospectively reviewed.Results There were a total of 76 non-traumatic aneurysms in ilio-femoral arterial region,including solitary iliac aneurysms ( n =29) and femoral aneurysms ( n =32).Seventeen (27.9％)patients had multiple aneurysms,8 ( 13.1％ ) patients ruptured aneurysms and 4 ( 6.6％ ) patients coexistent peripheral vascular occlusive disease.Fifty patients underwent electively aneurysm excision and graft (or autogenous vein ) replacement.Seven patients with ruptured aneurysms received emergency treatment.And one with multiple aneurysms died intra-operatively from ruptured iliac aneurysm.One patient with common iliac aneurysm underwent endovascular repair without endoleak and 1 with intemal iliac aneurysm received embolization.There was no perioperative mortality.But one with femoral aneurysm underwent amputation due to acute thrombosis.Seven patients died during the follow-up period and the survivors remained stable and had a good graft patency without new aneurysm formation.Conclusion Early management of aneurysms in ilio-femoral arterial region is rather important and multiple aneurysms should be considered.Aneurysm excision and arterial reconstruction yield an excellent outcome.Close and long-term follow-up is mandatory for the detection of new aneurysm formation.

Objective To explore the treatment of type Ⅱ endoleak (TEE) after the endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm (AAA).Methods From January 2009 to July 2011,10 patients with type Ⅱ endoleak after AAA EVAR were recruited.Different treatment methods were employed for the criminal arteries,including transcatheter embolization with coils,percutaneous sac puncture,intrasac injection of fibrin glue and outcome follow-up.Results Among them,9 patients underwent treatment and 8 received a mean follow-up of (2.4 ± 1.6) months.The mean secondary time was (62 ±39) months.The criminal arteries were inferior mesenteric artery (n =3),internal iliac artery (IA)( n =1 ),IA with lumbar artery ( n =3 ) and lumbar artery ( n =3 ).Two patients died from cardiovascular and cerebrovascular events during the follow-up.The primary technical success was 44％,retreat rate 33％ and clinical success rate 89％.Conclusion Type Ⅱ endoleak has a relatively benign course.In the absence of sac expansion,it can be followed for a long period of time without the needs for intervention.Treatment is necessary when it persists beyond 12 months or when there is a significant increase in aneurysm sac diameter.

Objective To evaluate the effects of hemoglobin (Hb) levels on long-term prognosis in the patients with acute ST-segment elevation myocardial infarction undergoing primary percutaneous coronary intervention.Methods A total of 150 patients with acute ST-segment elevation myocardial infarction undergoing primary percutaneous coronary intervention between January 2005 to December 2007 were enrolled.They were divided into 2 groups:Hb ＜ 120 g/L group ( n =21 ) and Hb ≥ 120 g/L group ( n =129).The mean clinical follow-up period was 3 years (range:41.4 ± 16.1 months).The differences of major adverse cardiac events (MACE),including death,acute myocardial infarction,stent thrombosis & stent restenosis),were observed between two groups.Results The parameters of infarction site,infarction relative artery,2-vessel disease,3-vessel disease,Killip class ≥ Ⅱ,drug-eluting stent,TIMI grade 3 flow,hypertension,hyperlipidemia,smoking,obesity,aspirin and clopidogrel use were not different between two groups (all P ＞0.05).The rate of diabetes was significantly higher in Hb ＜ 120 g/L group than that in Hb≥ 120 g/L group (47.62 ％ vs 18.60％,P =0.0032).The mean age and symptom-onset-to balloon- time (SOTB) were significantly higher in Hb ＜ 120 g/L group than that in Hb≥ 120 g/L group (68.5 ±9.2 vs 61.2±12.2 years,P＜0.0001; 8.8±10.5 vs 6.3 ±5.0 h,P＜0.0001).The mean LVEF (left ventricular ejection fraction) ( ％ ) and rate of complete revascularization were significantly lower in Hb ＜ 120 g/L group than that in Hb ≥ 120 g/L group (51.25 ± 11.34 vs 58.79 ± 10.38,P ＜ 0.0001 ; 61.9％ vs 86.8％,P =0.0045 ).Logistic regression analysis showed that LVEF was an independent predictor of MACE during the follow-up period ( P =0.0140 ).During a 3-year follow-up,MACE occurred in 16 patients.The incidence of MACE was significantly higher in Hb ＜ 120 g/L group than that in Hb ≥ 120 g/L group (33.33％ vs 6.98％,P =0.0003 ) ; Moreover the all-cause mortality and cardiac mortality were significantly higher in Hb ＜ 120 g/L group than those in Hb≥ 120 g/L group (28.57％ vs 3.10％,P ＜ 0.0001 ; 23.81％ vs 2.33％,P ＜ 0.0001 ).Conclusion In the patients with acute ST-segment elevation myocardial infarction undergoing primary percutaneous coronary intervention,hemoglobin level ＜ 120 g/L at baseline is markedly associated with adverse outcomes and an elevated incidence of MACE and mortality during the follow-up period.

Objective To explore the clinical and genetic characteristics of two patients with 11β- hydroxylase deficiency (11β-OHD).Methods The clinical features and laboratory data were collected from the patients and their families.All exons of CYP11 B1 gene were amplified by PCR.And the PCR product sequences were identified by a DNA analyzer.Results Two patients presented with juvenile hypertension with bilateral adrenal hyperplasia and congenital hypospadias,hypertension for 17 years and periodic hematuria for 3 months after dexamethasone therapy respectively.Steroid analysis showed the typical pattern of 11β-OHD:elevated plasma levels of adrenocorticotropic hormone (ACTH),17-hydroxyprogesterone (17OHP),11-deoxycortisol,androstenedione and testosterone and lowered levels of potassium,aldosterone and plasma renin activity (PRA).CT scan revealed the presence of bilateral nodular hyperplasia of adrenal glands.Sequencing analysis showed compound heterozygous mutations of [ R453Q] + [ R454C ] at exon 8 in patient 1 and homozygous mutation of [ R454C ] at exon 8 in patient 2.Conclusion 11β-OHD is the second major cause of congenital adrenal hyperplasia.The classic characteristics are hypertension with low a level of PRA,hypokalemia,female pseudohermaphrodism and male sexual precocity.11β-OHD should be screened in the patients with juvenile onset hypertension accompanied by bilateral adrenal hyperplasia.

Objective To evaluate the efficacy and safety of intubation dilatation under flexible bronchoscopic guidance in the management of benign tracheal stenosis.Methods A retrospective analysis of the clinical data was performed for 12 patients with benign tracheal stenosis from March 2010 to August 2011.There were 5 males and 7 females with a mean age of 37 ± 11 years old (range:27 -65).They were treated by intubation dilatation with different sizes under bronchoscopic guidance.And balloon dilatation was also performed for left or right main stem bronchial stenosis.And metal stents were implanted if necessary.Airway diameter,dyspnea index,complications and blood gas analysis were evaluated in all patients.And the forced expiratory volume in one second ( FEV1 ) was tested in 9 cases before and after the treatments of intubation dilation,balloon dilation and other interventions.Results One to five attempts of intubation dilation were required to achieve satisfactory dilatation.There was immediate postoperative relief of dyspnea for all 12 cases.And PaO2 and SaO2 rose markedly,but PaCO2 declined after intervention.The effective rate of intubation dilation was 100％.The average airway diameter increased from (5.7 ± 1.2) to ( 12.2 ±2.1 )mm and FEV1 improved from (0.67 ± 0.13 ) to ( 1.73 ± 0.37 ) L ( P ＜ 0.01 ).Conclusion The minimally invasive management of benign tracheal stenosis with intubation dilatation is both safe and efficacious.

Objective To compare the reconstructing safety of pancreaticogastrostomy (PG) versus pancreaticojejunostomy (PJ) after pancreaticoduodenectomy (PD).Methods The articles of prospectively controlled trials published until late December 2010 comparing PJ and PG after PD were searched by the means of MEDLINE,EMBASE,Cochrane Controlled Trials Register databases and Chinese Biomedical Database.After quality assessment of all included prospective controlled trials,a meta-analysis was performed with Review Manager 5.0 for statistic analysis.Results A total of 6 prospective controlled trials were included.Among 867 patients analyzed,440 underwent PG and 426 PJ.A meta-analysis of 6 prospective controlled trials (including randomized control trial (RCT) and non-randomized prospective trial) revealed significant differences between PJ and PG regarding the overall postoperative complication rates [ OR 0.53,95％ CI(0.30,0.95 ),P =0.03 ],pancreatic fistula [ OR 0.47,95％ CI(0.22,0.97 ),P =0.04 ] and intra-abdominal fluid collection [ OR 0.42,95％ CI ( 0.25,0.72 ),P =0.001 ].The differences in biliary fistula,intra-abdominal (IAC) complications and mortality were of no significance.Meta-analysis of 4 RCTs revealed significant differences between PJ and PG regarding intra-abdominal fluid collection [ OR 0.46,95％ CI (0.26,0.79 ),P =0.005 ].The differences in pancreatic fistula,overall postoperative complications,biliary fistula,intra-abdominal complications and mortality were of no statistical significance.Conclusion Through a meta-analysis of 6 prospective controlled trials,there are significant differences between PJ and PG regarding overall postoperative complications,pancreatic fistula and intraabdominal fluid collection.Significant differences exist between PJ and PG regarding intra-abdominal fluid collection.The safety profiles of PG and PJ are comparable.

Objective To evaluate the relevance of cardiovascular disease (CVD) risk and abnormal ankle brachial index (ABI) in high-risk and very high-risk hypertensive patients and explore the strategies of prevention and treatment for peripheral arterial disease according to the CVD risk.Methods Between December 2008 and May 2009,the cross-sectional study investigated the prevalence of abnormal ABI (ABI ＜0.90) in 2674 community-dwelling,hypertensive patients,aged ≥40 years old,without coronary heart disease,stroke/transient ischemic attack or known arteriosclerosis,from 18 centers in China.The relevant data were acquired through history,physical examination,laboratory examinations and other diagnostic tests.Results The number of subjects available for the full-analysis set was 2615.There was a rising prevalence of abnormal ABI with the advancing age in high-risk hypertensive patients and very highrisk ones (P＜0.001 ).Compared with the high-risk group,the 60 years ( 1.3％ vs 5.1％ ) and 70 years (4.1％ vs 10.5％ ) age sub-groups of the very high-risk group had a significantly higher prevalence of abnormal ABI (P ＜0.05 ).After adjusting for such factors as investigational center,target organ damage,CVD risk and demographics,an unconditional Logistic regression model revealed that CVD risk was positively correlated with abnormal ABI ( odds ratio 1.874,95％ confidence interval 1.153 - 3.045,P =0.0112).A very high-risk hypertensive patient was 1.874 times more likely than a high-risk one to have an abnormal ABI.Conclusion A high-risk hypertensive patient with a higher CVD risk is more prone to have an abnormal ABI.Among the high-risk hypertensive patients in the 60 years and 70 years age sub-groups,such a positive correlation is especially pronounced.

Objective To explore the relationship between the sputum levels of high mobility group protein B1 ( HMGB1 ) and airway inflammation in bronchial asthma and chronic obstructive pulmonary disease (COPD) patients.Methods A total of 57 patients with persistent asthma [ per Global Initiative for Asthma (GINA) guidelines ],30 patients with stable COPD [ stratified by Global Initiative for COPD (GOLD) status] and 20 control subjects were recruited.After completing an asthma control questionnaire,spirometry was performed before sputum induction.The ratio of forced expiratory volume in the first second (FEV1)/predictive value (FEV1 ％ Pre) and neutrophil differential count in induced sputum were recorded.The concentrations of HMGB1 in the supernatant of sputum were measured by ELISA (enzyme-linked immunosorbent assay).Results The sputum concentrations of HMGB1 in the asthmatics and COPD patients were significantly higher than those of the control subjects [ (291 ±55) and (511 ±39) vs (61 ±5) ng/L,all P ＜0.01 ].And they were significantly negatively correlated with FEV1％Pre in all subjects.The levels of HMGB1 in induced sputum of COPD patients were significantly higher than those of asthmatics and healthy controls ( P ＜ 0.01 ).No significant difference existed in the levels of HMGB1 between patients with eosinophilic and noneosinophilic asthma [ (290 ± 55 ) vs (292 ± 54) ng/L,P ＞ 0.05 ].The HMGB1 levels with COPD stage Ⅱand stage Ⅲwere significantly higher than those with stage Ⅰ [ (526 ±29) and (541 ±29) vs (471 ± 18) ng/L].The differences of sputum neutrophil percentage were statistically significant in mild,moderate and severe asthma [ (27 ± 2) ％,( 36 ± 4) ％,(49 ± 4) ％ ].And the sputum levels of HMGB1 were significantly higher in the patients with moderate and severe asthma [ (312 + 14) vs (347 ±11 ) ng/L].And the levels of HMGB1 in asthmatic and COPD patients were positively correlated with neutrophil percentage.According to the multivariate analysis,neutrophil percentage and FEV1 ％ Pre were independent predictors of sputum HMGB1,but not smoking,age,gender and eosinophilic percentage.Conclusion HMGB1 may contribute to airway inflammation through its higher expression in bronchial asthma and COPD patients.

Objective To explore the resistance rate,risk factors and mortality of Escherichia coli bloodstream infections (BSI) after liver transplantation.Methods From January 1993 to May 2010,a retrospective analysis of Escherichia coli in liver transplants were conducted.Results A total of 88 BSI occurred in 83/695 patients and Escherichia coli ( n =23 ) was most commonly found.Carbapenem and piperacillin-tazobactam were the most consistently active against Escherichia coli while the resistance rate to enterococcus for ciprofloxacin,gentamycin,ampicillin-clavulanic acid was over 60％.Univariate analysis identified the following variables as risk factors for Escherichia coli bacteremia:cholangioenterostomy ( P ＜0.001 ) and ductal complications ( P ＜ 0.001 ).Escherichia coli bloodstream infection could increase the mortality at 15 days after bloodstream infection.No significant difference in mortality occurred at 30 days and 1 year after enterococcal bacteremia.Conclusion Escherichia coli after liver transplantation is resistant to agents but commonly active to carbapenem and piperacillin-tazobactam.The risk factor associated with Escherichia coli bloodstream infections are cholangioenterostomy and ductal complications.Escherichia coli bloodstream infection can increase the mortality at 15 days after bloodstream infection.

Objective To identify the pathogenic mutations of phenylalanine hydroxylase gene (PAH) in patients with phenylketonuria (PKU) from Hebei Province.Methods Genomic DNA was extracted from 55 unrelated PKU patients from September 2007 to July 2009.All PAH exons and exon-intron junctions were amplified by polymerase chain reaction (PCR) and sequenced.Multiplex ligation-dependent probe amplifications (MLPA) was performed to detect the deletions or duplications of PAH.Gap-PCR was used to determine the breakpoints of large deletions.Results Among them,108 mutant alleles (98.2％)were found.All PAH exons with the exceptions of exons 9 and 13 were affected.A total of 41 different mutations were detected,including missense ( n =24),nonsense ( n =7),splicing ( n =7),small deletion (n =1 ) and large deletion ( n =2).Among them,4 missense mutations ( p.Pro147Leu,p.Gly289Arg,p.Phe392Ser,p.11e421Thr) and 2 large deletions ( -4163_-406del and -1932_ + 3402del) were novel.The most common mutations were p.Arg243Gln ( 12.7％ ),c.611A ＞ G ( 11.8％ ) and c.1197A ＞ T (9.1％ ).Conclusion The mutations of PKU patients with from Hebei Province are scattered throughout the PAH gene.Most of them are of single nucleotide substitutions,but large deletions are not rare.

Objective To define the protective role of pericardial tissue and identify the subcellular population with potential contribution to cardiac protection after injury in a rat model.Methods Myocardial infarction (MI) was created by the ligation of left anterior descending artery (LAD) in rats while the pericardial sac was either completely removed ( - PC,n =10) or surgically closed after ligation ( + PC,n =8 ).The follow-up functional and structure benefits were analyzed by echocardiography and 2,3,5- triphenyl tetrazoliumch(TTC) and bromodeoxyuridine (BrdU)staining.The contributions of cardiac stem cells (sca-1,c-kit and KDR) were evaluated by immunohistochemistry.Results The integrity of poricardial sac showed significant benefits of cardiac contractile function (ejection fraction and short axis fractional shortening) in comparison to the controls (52 ± 12 vs 37 ± 12 and 36 ± 14 vs 25 + 13,both P ＜0.05).The observed functional amelioration after MI was obvious based on structural repair examined by TTC staining and BrdU incorporation,showing significantly more de novo cardiomyocytes in + PC rats.Interestingly,we have immunohistologically identified a sub-population of KDR + (flk-1 + ) cells in pericardial tissue which clonegenically distributed and mildly expressed cardiac specific proteins (cTnT).It suggested that the KDR + cells might act as cardiac progenitors and therefore play an important role in cardiac repair.Meanwhile,rare SCA-1 and c-kit positive cells were detected in pericardial tissue.Conclusion The intact pericardial tissue exerts protective effects after MI.It may be related with a specific cell population of KDR + cells from pericardial tissue.

Objective To compare the structures and functions of rabbit bladder after partial bladder outlet obstruction versus without ischemia so as to explore the effects of ischemia on bladder pathogenesis in rabbits with partial bladder outlet obstruction.Methods A total of 64 mature male rabbits were divided into 4 groups (n =16 each).Four of each group underwent operation to establish an ischemie animal model ( ischemia group ),another 4 underwent operation to establish a partial bladder outlet obstruction animal model ( obstruct group),the other 4 underwent operation to establish an ischemic and partial bladder outlet obstruction animal model (combination group) and the remaining 4 underwent a sham operation as control.The rabbits in 4 groups were evaluated at Week 1,2,4 and 8 post-operation respectively.The weight of bladder,the thickness of mucosal,submucosa,muscular layer and placenta percreta and the activities of sarco/endoplasmic reticulum Ca2+ ATPase citrate synthase of cystic smooth muscle were detected respectively.MASSON staining was used to observe the smooth muscle and collagen in stroma of bladder and S-100 staining for observing the neurons in bladder.Results In obstruct and combination groups,the weights of bladder at week 1 were ( 5.10 ± 0.29 ) g and ( 4.80 ± 0.37 ) g respectively.They were both significantly higher than control group[ ( 1.93 +0.17) g,all P ＜0.05].The weights of bladder in obstruct and combination groups peaked at Week 4 and they were (18.48 + 2.03 ) g and ( 12.35 ±0.39) g respectively.The weight of bladder in obstruct group was significantly heavier than combination group in the same terms.And they were both significantly heavier than control and ischemia groups(all P ＜ 0.05 ).Muscular tissue vicariously thickened during the first 4 weeks,and collagen and stroma increased at Week 4 in obstruct group.Muscular tissue,collagen and stroma all increased initially.But at Week 2 only collagen and stroma increased in combination group.Compare with control group,the other groups all have deletion of neurons,especially in combination group.The activities of sarco/endoplasmic reticulum Ca2 + ATPase and citrate synthase of cystic smooth muscle of obstruct group peaked at Week 4.In combination group,the activities of sarco/endoplasmic reticulum Ca2+ ATPase and citrate synthase of cystic smooth muscle were decreased over 2 -8 weeks.In the same terms,the activities of sarco/endoplasmic reticulum Ca2+ ATPase and citrate synthase of cystic smooth muscle in control group were significantly higher than those in obstruct and combination groups ( all P ＜ 0.05 ).Conclusion Ischemia can reduce the tolerance of bladder and aggravate the impairment of bladder to partial outlet obstruction.

The patient was a 27-year-old female who was admitted to the Pulmonary Vascular Center of Fuwai Cardiovascular Hospital on February 10, 2011 due to chest tightness and fatigue for half a year. In September 2010, the patient developed high fever of 39℃, accompanied by chest tightness, shortness of breath, fatigue and cough after upper respiratory tract infection. She was diagnosed as "acute myocarditis" in the local hospital. During hospitalization, she developed bilateral pleural effusion, obvious on the right side. The pleural effusion was extracted twice, which was pale yellow clear liquid. The laboratory results showed that it was leakage liquid. After symptomatic treatment, it improved and was discharged. In October, she had chest tightness and fatigue again, accompanied by mild edema of both lower limbs, pleural effusion, and could not lie down at night. It improved after diuresis, thoracentesis and fluid extraction. In mid-to-late November, she was hospitalized again due to the aggravation of the above symptoms. Echocardiography showed: left atrial diameter 34 mm, left ventricular end diastolic diameter 46 mm, left ventricular ejection fraction 36%, right atrial and right ventricular enlargement, right ventricular diameter 38 mm, diffuse motion amplitude of left and right ventricular walls decreased, tricuspid valve insufficiency, trace pericardial effusion, Mild pulmonary arterial hypertension. X-ray chest radiographs showed massive pleural effusion on both sides and incomplete dilation of both lower lungs. Pulmonary embolism was suspected because of pulmonary hypertension, and pulmonary CT angiography showed multiple pulmonary embolism, pleural effusion, peritoneal effusion and atelectasis due to external pressure on both lungs. The local hospital diagnosed cardiomyopathy, heart failure and pulmonary embolism. Cardiotonic, diuretic and warfarin anticoagulation were given. The patient had normal diet, bowel movement and sleep from the onset of the disease, and there was no obvious change in body weight. The history of hypertension and hyperlipidemia was denied. Skin pigmentation was found 8 years ago, and liver function was abnormal in laboratory, so he was given hepatoprotective treatment. He was admitted to hospital 3 years ago due to ketosis coma and was diagnosed as type 1 diabetes, and was treated with insulin. The parents were intimate relatives within 4 generations, and his first brother suffered from dilated cardiomyopathy, accompanied by skin pigmentation and liver function damage. No history of drug allergy and no history of surgical trauma.

Behcet's disease (BD) is a chronic inflammatory disease with multi-system involvement. It was first discovered by Hippocrates in the 15th century BC, and summarized as a syndrome by a Turkish physician Behcet in 1937. At present, it is believed that BD may be a systemic vascular inflammatory disease. Its etiology and pathophysiological mechanism are still unclear, but it is mainly considered to be related to genetic, environmental and autoimmune factors. BD tends to occur in young adults aged 30~40 years old, and there are certain regional differences in the main clinical manifestations, disease severity and sex ratio. BD patients are mainly distributed in countries around the ancient Silk Road (including China), with the highest incidence rate in the Middle East, while the lowest incidence rate in the Caucasus, and black people are rarely affected. And the incidence rate is also higher in the Far East [1]. The clinical manifestations of BD are various, mainly including: (1) skin and mucosa: oral ulcer (98%), genital ulcer (80%), erythema nodosum, folliculitis acneiform, acupuncture reaction; (2) arthritis/arthralgia (50%); (3) ocular involvement: panuveitis, retinal vasculitis (50%); (4) Vasculitis: thromboarteritis, phlebitis, aneurysm formation, vasculitis involves the whole system including peptic ulcer, epididymitis, central nervous system changes, lung changes, etc. [2]. The National Institutes of Health (NIH) put forward the diagnostic criteria of BD in 1990: (1) recurrent oral ulcer; (2) recurrent genital ulcer; (3) ocular involvement; (4) characteristic skin lesions; (5) Skin allergic reaction is positive. Any two of (1) + (2) ~ (5) can be diagnosed. Although this clinical diagnostic criterion has certain application value, there are some problems due to the lack of objective laboratory index support. For example, acupuncture reaction can also be seen in some neutrophilic skin diseases Sweet syndrome, gangrenous pyoderma and recurrent aphthous stomatitis (RAS) [3]. More importantly, this diagnostic criterion is of little significance to judge whether the disease is active or recurrent.

Donor: 33-year-old male, hospitalized in Guangdong Provincial Hospital of Traditional Chinese Medicine for more than 2 years due to severe brain trauma. Due to long-term ineffective treatment, his family members have repeatedly and repeatedly requested the competent departments at all levels and the media to donate his body and organs for free to treat his biological father and other similar patients suffering from chronic renal failure (renal failure). The patient died on the morning of July 27, 2005 due to complete arrest of heartbeat and spontaneous breathing, and was rescued by various means such as external cardiac compression, mechanical ventilation and stimulants. All indicators have been repeatedly checked to meet the international standards, indications and ethical requirements of organ donation in cardiac death (DCD); After heparinization, continuous pure oxygen-assisted respiration and adrenaline treatment, the kidney remains were urgently extracted. The time of hot ischemia was 40 min and the time of cold ischemia was 8 h. The pathological examination of the donated kidneys showed moderate hypoxic changes by zero-point puncture. Two adult kidney transplantations were continuously completed, one of which was a rare "kinship kidney transplantation" (son → father).

The patient, a 12-year-old female, came to the Ophthalmology Clinic of Peking Union Medical College Hospital on June 7, 2010 due to blurred vision in both eyes for 2 months. There was no obvious visual distortion. Past history: Occasional epileptic seizures before the age of 5, no seizures after school age, no drug treatment, and the intelligence and physical development were the same as those of peers. Physical examination: Binocular visual acuity 1 2, normal intraocular pressure, normal anterior segment, clear vitreous body, normal color boundary of optic disc, retinal edema lesions with clear local boundary above the temporal side of the optic disc, slightly gray-white color, no obvious bleeding and exudation, and foveal reflection (Figure 1). Fluorescein sodium fundus angiography (FFA): small vessels on the surface of the lesion were dilated in the early stage, and leakage gradually appeared with time. In the late stage, there was uniform high fluorescence with clear boundary, and there was no staining or leakage in the deep retinal vessels (Figure 2). Indocyanine green fundus angiography: low fluorescence from beginning to end (Figure 3). Optical coherence tomography (OCT): the inner retinal signal at the lesion was uniformly enhanced and raised (Figure 4 The foveal shape of the macula was normal. Dense cortical adenomas of the size of miliary to soybean were seen on the dorsum of the hands, cheeks and nasolabial folds on both sides. MRI of the head showed patchy long T1 and long T2 signal shadows in the cerebral cortex. Dermatology and neurology consultation: it was consistent with tuberous sclerosis (TS) changes. Diagnosis: TS complicated with retinal hamartoma in both eyes. No treatment.