1.A modified tricuspid annuloplasty for functional severe tricuspid regurgitation
Shengli JIANG ; Changqing GAO ; Chonglei REN ; Lin ZHANG ; Zhiyun GONG ; Tingting CHENG ; Tao ZHANG ; Yao WANG
Chinese Journal of Thoracic and Cardiovascular Surgery 2011;27(8):462-465
ObjectiveTo retrospectively analyze clinical data of patients who has left-side valvular disease combined with severe tricuspid regurgitation and evaluate the effect of our modified tricuspid annuloplasty with enforcement of artificial felt strip.Methods76 patients who had left-side valvular disease combined with severe tricuspid regurgitation received operations between Jan.2008 and Jun.2010.The average age of the patients was 53.5 years old (32 male and 44 female).Besides the severe tricuspid regurgitation, other combined cardiac impairments included mitral valvar disease (52 cases), aortic valvar disease(5 cases), double valvar disease(19 cases) and left atrial thrombosis(22 cases).6 patients had grade II cardiac function according to the NYHA criteria, while 47 and 23 were in grade III and IV, respectively.Other signs included cyanosis(5cases), jaundice(11 cases), neck vein engorgement(48 cases) , ascites(22 cases), hepatomegaly(41 cases) and pitting edema in the lower limbs(68 cases).The concomitant operative procedures included mitral valve replacement in 52 patients,aortic valve replacement in 5 patients, double valve replacement in 19 patients, removal of left atrial thrombus in 22 patients,left atrium folding in 21 patients and left atrium appendage suture in 68 patients.Left-sided valve disease were corrected first,TAP was performed on the beating heart after the heart had been defibrillated.The anteroseptal commissure was plicated first.A double-armed 3-0 pledgeted suture was taken through the base of the septal leaflet, 5-6 mm from the commissure, extending along the annulus, and out from the point in the anterior annulus 10-12 mm from the anteroseptal commissure.Both ends of the suture was tied until the two Teflon pledgets approximated each other near the commissure.Then a semicircular De Vega type of plicating with a 3-0 prolene was taken, starting just from the anterior annulus near the anteroposterior commissure, and extending clockwise to a point just cephalad to the posteroseptal commissure.The suture was tied with positioning a 27-29 mm valve siser across the tricuspid valve.At last, a 3-5 mm width felt strip was prepared and was sutured to the plicated posterior annulus region with interrupted mattress sutures of 2 to 3 2-0 prolene.A favorable result was considered when TR was not marked by saline injection.Echocardiography was routinely examined one week postoperatively and patients were followed up 6 month after discharge.ResultsThere is no death in all patients.The CVP diminished significantly from 16mmHg preoperatively to 8mmHg postoperatively (P = 0.0021).The systomic pulmonary pressure diminished from 59 mmHg preoperatively to 41 mmHg postoperatively (P = 0.038).Echo one week postoperative showed no tricuspid regurgitation in 56 patients and mild in 18 patients, while 2 had moderate tricuspid regurgitation.The diameter of right atrium diminished significantly postoperatively, too.The ejection fraction was improved even though there was not significant difference as compared with preoperative data.The cardiac function of all patients improved and the signs of right heart failure were alleviated or disappeared.Follow up 1 to 36 months showed no change of the regurgitation except for one become moderate from mild when discharged.No hepatic congestion or edema was observed in all patients.ConclusionThese new modifications make the technique more selective in the remodeling of the tricuspid annulus.It could achieve better coaptation of the anterior leaflet with the others, successful annular reduction, better maintenance of the contractile property of the tricuspid ring, better distribution of pursing force in the more dilated region.It could prevent the tear of the endocardium in the posteroseptal region in the long period of time postoperatively.
2.Findings of 256-slice CT angiography in atherosclerosis of the intracranial carotid artery
Jiao HUO ; Bin FENG ; Shaoyong GONG ; Xiaojun YANG ; Huan YANG ; Sanshan XING ; Yanni GAO ; Zhiwei YAN ; Xiaoqun YAO ; Guangfu YANG
Journal of Practical Radiology 2016;32(5):686-690
Objective To study the findings of 256‐slice CT angiography in atherosclerosis of the intracranial carotid arteary . Methods 402 cases of atherosclerotic lesions in the neck and brain were collected from 2013 to 2014 in our hospital ,in which there were 274 cases of male and 128 cases female .All patients underwent 256‐slice CT angiography of the head and neck .The incidence of atherosclerosis involvement in the intracranial carotid artery was analysed .The characteristics of carotid plaque on different sexes , age(≤60 years and >60 years) and location (right and left ) were observed .The length and property of plaque ,and stenostic rate of the artery were measured respectively .Results There were 164 cases (40 .80% ) of intracranial carotid artery athrosclerosis in 402 cases of atherosclerotic lesions of the neck and brain ,in which the incident rate of male was 38 .32% and female 46 .09% (χ2 =2 .182 ,P=0 .140) .The incident rate of >60 years group was more than that of ≤60 years group (χ2 <31 .105 ,P<0 .001)for both sexes . There were no differences on lesion location (right or left)for different sexes or age groups (χ2 <0 .035 ,P>0 .088) .And there were also no differences on lesion length (≤10 mm ,11-20 mm ,>20 mm) for different sexes or age groups (χ2 <0 .027 ,P>0 .091) . Calcified plaques were seen the most (79 .81% ) ,mixed plaques were seen more (15 .46% ) ,and lipid plaques (1 .58% ) and fibrous plaques (3 .15% ) were seen less .The mixed plaques were more seen on >60 years group than that on ≤60 years group for male pa‐tients (χ2 =12 .204 ,P=0 .001) ,but no other difference was found for plaque property in other different sexes or age groups .Mild stenosis of the corated artery was found in 54 .57% lesions ,moderate stenosis in 39 .75% and severe stenosis in 5 .68% ,and there were no any difference in different sexes or age groups (χ2 <2 .062 ,P>0 .151) .Conclusion CT angiography of the head and neck is of important significance for diagnosis of atherosclerosis in the intracranial carotid artery .
3.Surgery in patients of severe aortic stenosis with low transvalvular gradients and severe left ventricular dysfunction
Shengli JIANG ; Changqing GAO ; Bojun LI ; Lin ZHANG ; Mingyan WANG ; Zhiyun GONG ; Chonglei REN ; Dong LI ; Tingting CHEN ; Tao ZHANG ; Yao WANG ; Wei YU
Chinese Journal of Thoracic and Cardiovascular Surgery 2012;28(7):415-417
Objective To assess the result of aortic valve replacement(AVR) for patients of severe aortic stenosis(AS)with low transvalvular gradients(TVG) and severe left ventricular dysfunction,and try to identify the determinants of survival,functional status and change in left ventricular ejection fraction(LNEF) during follow-up.Methods From 2005 to 2011,35patients with aortic valve area(AVA) < 1 cm2,LN EF < 0.40 and mean TVG < 30 mm Hg underwent AVR in our hospital.The average age of the patients was 58 yeats old,and 88.6% of the patients were in New York Heart Association (NYHA) functional class Ⅲ/IV at admission to the hospital.The AVA was (0.70 ± 0.09) cm2,LVEF was 0.276 ± 0.020,TVG was (26.0 ± 2.3) mm Hg,and left ventricular end-diastolic diameter (LNEDD) was (6.3 ±0.4) cm respectively.35 prosthetic valves were implanted,including 20 mechanical prostheses and 15 biological prostheses with the mean sizs of (23 ± 1) mm.Concomitant procedures included mitral valvularplasty in 5.tricuspid valve repair in 3 and coronary artery bypass grafting in 4.Results The perioperative mortaiity was 8.6%.Follow-up period was 3 to 60 monthes.The survival rates were:1-year 78%,2-year 68%,5-year 60%.LVEF increased significantly to 0.358 ± 0.047 one week postoperatively (P =0.008) and 0.426 ± 0.031 six months later (P < 0.01)).LNEDD decreased to (5.7 ± 0.4) cm one week later(P =0.062) and (5.3 ±0.3)cm 6 months postoperatiwely (P < 0.01).NYHA functional class improved from 88.6% in class Ⅲ/Ⅳ to 35% (P <0.01).Compared with those who surviwd during follow-up,the patients who died during follow-up were older in the year of operation[(63 ± 10) vs (54 ± 11),P =0.017],their NYHA functional class was higher[(3.9 ±0.2) vs (2.9 ±0.3),P =0.003]and the LVEDD for them in one week postoperatively was larger[(6.0 ± 1.0) cm vs (5.5 ± 0.3) cm,P =0.031].Conclusion The left ventricle contractile reserve seems to play an essential role for surgery in patients of severe aortic stenosis with low transvalvular gradients and severe left ventricular dysfunction.AVR can be performed for them with acceptable results.
4.Chemical components of essential oils from Meconopsis oliverana and their antioxidant activity.
Ang GAO ; Bing ZHAO ; Jiang GONG ; Shi-Feng NI ; Chao CUI ; Mo YAO
China Journal of Chinese Materia Medica 2013;38(2):284-288
OBJECTIVETo study the chemical components of essential oils from Meconopsis oliverana and their antioxidant activity.
METHODThe essential oil was extracted by steam distillation, and GC-MS analysis was used to identify its constituents. The OH free radical scavenging activity of the essential oils was evaluated with an enzyme mark instrument by assay of the ability of DPPH free radical scavenging. BHT was used as positive control.
RESULTForty-seven compounds, account for 91.866% of the essential oils, were identified. The ability of scavenging OH and DPPH radicals of the essential oils is stronger than that of BHT.
CONCLUSIONThe main chemical constituents of the essential oils from M. oliverana are n-hexadecanoic acid (27.653%) and 6,10,14-trimethyl-2-pentadecanone (16.330%). And the essential oils showed strong antioxidant activity.
Antioxidants ; chemistry ; metabolism ; Biphenyl Compounds ; metabolism ; Butylated Hydroxytoluene ; metabolism ; China ; Fatty Alcohols ; chemistry ; isolation & purification ; Free Radical Scavengers ; chemistry ; metabolism ; Gas Chromatography-Mass Spectrometry ; Hydroxyl Radical ; metabolism ; Oils, Volatile ; chemistry ; isolation & purification ; Oxidation-Reduction ; Palmitic Acid ; chemistry ; isolation & purification ; Papaveraceae ; chemistry ; Picrates ; metabolism ; Plant Oils ; chemistry ; isolation & purification
5.Study the effects of Salvia miltiorrhiza monomer IH764-3 on the expression of matrix metalloproteinase in lungs of rats exposed to Paraquat (PQ).
Dong-qi YAO ; Ying-ping TIAN ; Heng-bo GAO ; Yu GONG ; Zhao-hua MENG ; Fei TONG ; Han-wen SHI
Chinese Journal of Industrial Hygiene and Occupational Diseases 2012;30(5):321-325
OBJECTIVETo observe the expression of the matrix metalloproteinase 2 (MMP-2) and membrane-type 1 metalloproteinase (MT1-MMP) in lung of rats exposed to paraquat (PQ) and the effects of Salvia miltiorrhiza monomer IH764-3 on above expression.
METHODSNinety adult healthy Sprague-Dawley (SD) rats were randomly divided into the control group (group A, 6 rats), the exposure group (group B, 42 rats) and the group treated by Salvia miltiorrhiza monomer IH764-3 (group C, 42 rats). The group B and C were treated intragastrically with 1ml of PQ (50 mg/kg), and the group A was treated intragastrically with normal saline. The group C was treated intraperitoneally with 1 ml Salvia miltiorrhiza monomer IH764-3 at the dose of 40 mg/kg a day. The group A and B were treated intraperitoneally with 1 ml normal saline day. The expression of MMP-2 and MT1-MMP was detected on the 1st, 3rd, 7th, 14th, 21st, 28th and 35th days after exposure for all groups.
RESULTSAs compared with the expression level (0.305 ± 0.045) of MMP-2 mRNA in group A, the expression levels of MMP-2 mRNA in Group B significantly increased, which were 0.654 ± 0.077, 0.623 ± 0.051, 0.637 ± 0.024, 0.533 ± 0.043 and 0.552 ± 0.050 on the 1st, 3rd, 7th, 14th, 21st days after exposure, respectively (P < 0.01). As compared with group A, the the expression levels of MMP-2 mRNA on the 1st, 3rd, 7th days in Group C slightly increased, but the expression levels of MMP-2 mRNA on the 1st, 3rd, 7th, 14th, 21st days in Group C were 0.523 ± 0.074, 0.567 ± 0.097, 0.514 ± 0.058, 0.359 ± 0.018 and 0.374 ± 0.020, respectively, which were significantly lower than those in group B (P < 0.01). As compared with the expression level (0.391 ± 0.058) of MT1-MMP mRNA in group A, the expression levels of MT1-MMP mRNA in Group B significantly increased, which were 0.796 ± 0.021, 0.762 ± 0.043, 0.590 ± 0.010, 0.803 ± 0.076 and 0.680 ± 0.034 on the 1st, 3rd, 7th, 14th and 21st days after exposure, respectively (P < 0.01). As compared with group A, the expression levels of MT1-MMP mRNA in Group C significantly increased, which were 0.594 ± 0.010, 0.653 ± 0.044 and 0.564 ± 0.009 on the 1st, 3rd and 21st days after exposure, respectively (P < 0.01). The expression levels of MT1-MMP mRNA in Group C were significantly lower than those in group B (P < 0.05 or P < 0.01).
CONCLUSIONThe expression changes of MMP-2 and MT1-MMP genes of lungs in rats intragastrically exposed to PQ could result in the unbalance the synthesis and degradation of ECM, which may be a cause of lung fibrosis. The Salvia miltiorrhiza monomer IH764-3 could affect the expression of MMP-2 and MT1-MMP genes to a certain extent, resulting in the reduction of lung fibrosis.
Animals ; Drugs, Chinese Herbal ; pharmacology ; Female ; Lung ; drug effects ; metabolism ; Male ; Matrix Metalloproteinase 14 ; metabolism ; Matrix Metalloproteinase 2 ; metabolism ; Paraquat ; toxicity ; RNA, Messenger ; genetics ; Rats ; Rats, Sprague-Dawley ; Salvia miltiorrhiza
6.Acute intestinal obstruction: etiology and treatment
Lu YAO ; Yuda GONG ; Bo ZHANG ; Weidong GAO ; Weizhong SHENG ; Yihong SUN
Chinese Journal of General Surgery 2019;34(3):196-199
Objective To explore the etiology and treatment of acute intestinal obstruction.Methods Clinical data of patients who underwent operation for acute intestinal obstruction in Zhongshan Hospital from May 2012 to May 2017 were collected and retrospectively analyzed.Results 721 patients were included and the ratio of males to females was 1.55 ∶ 1.There were 48.8% in old-aged group and 51.1% in young-middle-aged group.The most common causes of ileus included tumor in 376 cases (51.5%),adhesion in 168 cases (23.3%),hernia in 70 cases (9.7%),intraluminal obstruction in 42 cases (5.8%) and others in 79 cases.There was a significant difference between incarcerated hernia in elderly group and middle-young-aged group (4.6% vs.15.1%,x2 =22.4,P < 0.01).The length of hospitalized days in patients with tumor and incarcerated hernia in elderly group were significantly longer than young-middle-aged patients [(15.3±8.6)d vs.(13.4±6.3)d,t =-2.5,P<0.05;(10.1 ± 6.7) d vs.(6.4 ± 2.9) d,t =-2.2,P < 0.05].The length of hospitalized days by limited operation in patients with tumor were significantly shorter than those by emergency operation [(16 ± 12)d vs.(18 ± 24) d,t =-0.3,P > 0.05].Conclusion Tumor and adhesion are the main causes of acute intestinal obstruction.Neoplastic bowel obstruction from small intestine or proximal colon requires emergency surgery.
7.Virulence-associated gene detection and analysis of Staphylococcus aureus isolates from pediatric patients′ feces
Xiaolan AI ; Yan LONG ; Bingshao LIANG ; Shuwen YAO ; Yunfeng LIU ; Fei GAO ; Jialiang MAI ; Zhile XIONG ; Zhuwei LIANG ; Jielin WANG ; Xiantang CHEN ; Min YANG ; Sitang GONG ; Zhenwen ZHOU
Chinese Journal of Laboratory Medicine 2021;44(4):291-297
Objective:To investigate the multilocus sequence typing feature of the virulence-associated genes of Staphylococcus aureus(S. aureus) separated from the clinical specimens of a multi-center cohort children in Guangzhou area. Methods:A total number of 412 Staphylococcus aureus strains isolated from 2 059 non-repeated fecal specimens of children by three groups′ researchers in Guangzhou Women and Children′s Medical Center from August 2018 to November 2018. While collecting specimens, patient clinical information is also properly collected and preserved. After extracting the DNA of the strain, the virulence-associated genes were detected by polymerase chain reaction (PCR), including the staphylococcal enterotoxin (SE) genes ( sea, seb, sec, sed, see) and the Panton-Valentine leucocidin-encoding gene ( pvl).The multi-locus sequence typing (MLST) method was performed to reveal the MLST feature of these genes and the statistical difference were examined by the the χ 2 test. Results:Among the 412 isolates of S. aureus, 256 strains (256/412, 62.1%) contains at least one SE gene. Among the enterotoxin gens, the sec (125/412, 30.3%), seb(98/412, 23.8%)and sea (66/412, 16.0%)genes were the three most prevalent members of SEs. The frequency of pvl gene in Staphylococcus aureus was 18.7%(77/412).Among them, the frequency of Staphylococcus aureus sea gene isolated from patients with gastroenteritis (58/319, 18.2%) was significantly higher than that from the non-gastroenteritis group (8/93, 8.6%)(χ2=4.912, P=0.027). The frequency of Staphylococcus aureus pvl gene isolated from the patients with pneumonia (8/21, 38.1%) was greater than that from the non-pneumonia group (6/47, 12.8%)(χ2=4.252, P=0.039). In addition, the virulence-associated gene of S. aureus was closely related to the specific ST type, 82.4% (28/34) of ST6 carried sea gene, all ST338 and ST59 carried seb gene, 96% (48/50) ST45 carried sec gene, and the pvl gene carrying rate of ST338 was 5/5. Conclusions:The SEA toxin produced by ST6 Staphylococcus aureus may be closely related to the diagnosis of gastroenteritis in children. The frequency of pvl virulence gene in Staphylococcus aureus in children with community-acquired pneumonia was higher than that in the non-pneumonia group, and closely related to the CC59.
8.Effect of doxazosin and metoprolol on vascuIar remodeling in rats with hypertension induced by abdominal aorta coarctation
Lishuang HUANG ; Weili LLU ; Jingbo GONG ; Xiujie GAO ; Yun ZHAO ; Jing MA ; Fang XLE ; Tao ZHANG ; Kaiqi TLAN ; Lin YAO ; Lingjia QLAN
Chinese Journal of Pharmacology and Toxicology 2015;(2):208-212
OBJECTIVE To investigate the effect of doxazosin(DOX) and metoprolol( MET) on vascular remodeling in rats with abdominal aorta coarctation (AAC). METHODS An animal model was established by AAC. Two weeks later, the rats were treated with DOX (10 mg.kg-1 per day) or MET (20 mg.kg-1 per day) for six weeks. Blood pressure was measured using carotid artery intubation with a MP150 polygraph. The media thickness, wall cross-sectional area and thickness / internal diameter ratio were calculated by morphometry. Vascular fibrosis was evaluated by Masson′s trichrome staining. The collagen and fibronectin expression in vascules was measured by Western blotting. RESULTS Compared with the sham group 〔(17.6±0.5)kPa〕, the mean arterial blood pressure in the model group〔(23.3±0.7)kPa〕 was significantly increased(P<0.05), but was lowered by DOX 〔(20.5±0.7)kPa〕 and MET 〔(19.0±0.4) kPa〕 (P<0.05). Moreover, HE staining showed that tunica media thickness, artery vessel area and thickness / inner diameter in the model group were increased by 39.5%, 46.4% and 27.0%(P<0.05), respectively. The tunica media thickness was decreased by 16.0% and 26.1%( P<0.05), respectively, the artery vessel area by 22.8% and 26.1%(P<0.05), respectively, and the thick-ness / inner diameter by 17.0% and 26.0%( P<0.05) when the rats were treated with DOX and MET. Masson staining showed that the collagen accumulation in vascules increased, suggesting that AAC induced fibrosis. Meanwhile, vascular fibrosis induced by AAC was also reduced by MET or DOX. Western blotting also proved that the increase of collagen and fibronectin induced by AAC could be attenuated by DOX and MET(P<0.05). CONCLUSION DOX and MET are effective in suppressing the role of norepi-nephrine in vassels, which can attenuate AAC-induced vassels remodeling by preventing the binding between norepinephrine and adrenoceptors.
9.Study on the trend of nutritional biochemical indexes in patients with paraquat poisoning
Zhaohua MENG ; Lin YUAN ; Yu GONG ; Dongqi YAO ; Tieying ZHU ; Hengbo GAO ; Yingping TIAN
Chinese Journal of Emergency Medicine 2018;27(9):1035-1039
Objective To observe the nutritional biochemical indicators of paraquat poisoning patients, analyze and compare the nutritional status of patients and understand the changing trend of each indicator. Methods A total of 104 patients with acute paraquat poisoning who were admitted to the emergency department of the Second Hospital of Hebei Medical University from December 2015 to December 2017 were enrolled, and divided into the cure group (patients who survived >30 days) and the death group. Nutritional biochemical indicators including serum protein (ALB, PA, TP) and serum lipids (TCh, TG, LDL) were selected for dynamic observation. The observation time points were set as follows: immediate treatment of poisoned patients (day 1 on admission), on day 4, 7, 10, 13 and 16 after admission, and on day 30 after follow-up. The nutritional biochemical indicators of the two groups on day 1 and 4 were statistically analyzed and compared by t test. The nutritional status of the patients in the cure group was analyzed, and the Repeated Measures Anova was performed to understand the trend of each indicator over time. Results In the cure group, the TP level decreased from (73.34±5.75)g/L on day 1 to (51.95±6.05)g/L on day 4, t=20.34, P<0.01; and the TCh level decreased from (4.37±0.98) mmol/L on day 1 to (3.03±1.01)mmol/L on day 4, t=7.56, P<0.01. In the death group, the TP level decreased from (72.25±8.80)g/L on day 1 to (49.07±5.48)g/L on day 4, t=12.38, P<0.01, and the TCh level decreased from (4.38±0.88)mmol/L on day 1 to (2.51±1.07) mmol/L on day 4, t=7.94, P<0.01. Compared with the cure group, serum levels of ALB, TP and TCh in the death group decreased greater from day 1 to day 4 (all P<0.05). In addition, dynamic observation of the indicators in the cure group within 16 days after admission showed that, after treatment, the levels of ALB and TP recovered slowly and were still lower than normal . While the levels of PA and lipid rose rapidly after 10 days of admission. Conclusions Paraquat poisoning seriously affects the nutritional status of patients, and the serum protein levels decline significantly and can not be recovered easily. Therefore, sufficient attention should be paid to the treatment, and timely and appropriate nutritional support should be provided.
10.Fine mapping of Smith-Fineman-Myers syndrome and exclusion of GPC3, GPCR2 MST4 and GLUD2 as candidate genes.
Qi-ji LIU ; Yao-qin GONG ; Jiang-xia LI ; Xi-yu ZHANG ; Gui-min GAO ; Yi-shou GUO
Chinese Journal of Medical Genetics 2004;21(3):198-202
OBJECTIVESmith-Fineman-Myers syndrome (SFMS) is an X-linked mental retardation syndrome. The authors had ascertained a large Chinese family with SFMS from Shandong and had mapped the disease locus to an interval of 19.8 Mb on Xq25 flanked by markers DXS8064 and DXS8050. Further investigation suggested that SFMS exhibited locus heterogeneity. In this study for facilitating the identification of the gene responsible for SFMS, the additional markers were analyzed to narrow down the candidate region, and four candidate genes (GPC3, MST4,GPCR2 and GLUD2) were chosen and screened for disease-causing mutation.
METHODSPCR and denaturing polyacrylamide gel electrophoresis were used to genotype 13 new polymorphic markers distributed within the candidate region. Mutation detection was accomplished by sequencing the exons and intron-exon junctions of the candidate genes.
RESULTSBy analyzing 13 additional polymorphic markers, SFMS candidate region can be reduced to an interval of 10.18 Mb bounded by XSTR3 and XSTR4, and no disease-causing mutation was identified in the coding regions of four candidate genes.
CONCLUSIONGPCR2 GPC3, MST4 and GLUD2 were excluded as pathogenic genes for SFMS. The refined SFMS locus will assist in the identification and characterization of other candidate genes for SFMS.
Abnormalities, Multiple ; genetics ; Chromosome Mapping ; Chromosomes, Human, X ; Genetic Linkage ; Glutamate Dehydrogenase ; genetics ; Glypicans ; Humans ; Intellectual Disability ; genetics ; Male ; Membrane Proteins ; genetics ; Neoplasm Proteins ; genetics ; Protein-Serine-Threonine Kinases ; genetics ; Receptors, G-Protein-Coupled ; genetics ; Syndrome