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  • 简介:Hereditaryfructoseintolerance(HFI)isanunderrecognized,preventablelife-threateningcondition.ItisanautosomalrecessivedisorderwithsubnormalactivityofaldolaseBintheliver,kidneyandsmallbowel.Symptomsarepresentonlyaftertheingestionoffructose,whichleadstobriskhypoglycemia,andanindividualwithcontinuedingestionwillexhibitvomiting,abdominalpain,failuretothrive,andrenalandliverfailure.AdiagnosisofHFIwasmadeina50-year-oldwomanonthebasisofmedicalhistory,responsetofructoseintolerancetest,demonstrationofaldolaseBactivityreductioninduodenalbiopsy,andmolecularanalysisofleukocyteDNAbyPCRshowedhomozygosityfortwodosesofmutantgene.HFImayremainundiagnoseduntiladultlifeandmayleadtodisastrouscomplicationsfollowinginadvertentfructoseorsorbitolinfusion.SeverallethalepisodesofHFIfollowingsorbitolandfructoseinfusionhavebeenreported.Thediagnosiscanonlybesuspectedbytakingacarefuldietaryhistory,andthiscanpresentseriouscomplications.

  • 标签: 遗传性 果糖 常染色体隐性遗传疾病 肝功能衰竭 成人 生长迟滞
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  • 简介:AbstractImportance:Childhood and adolescence are critical periods for lifelong bone mineral accrual, but few studies have determined the impact of childhood adiposity on adult bone density.Objective:To determine the long-term impact of childhood adiposity on adult areal bone mineral density (aBMD) and the effect of adult adiposity on this relationship.Methods:We conducted a longitudinal study of 1156 adults (56.3% men), for whom skinfold thickness (SFT) had been measured during childhood (6-18 years) and fat mass percentage (FMP) and aBMD were measured during adulthood (29-43 years). Adult aBMD in the lumbar spine (LS), femoral neck (FN), arms, and legs was measured using dual-energy X-ray absorptiometry. The direct effect of childhood SFT and its indirect effect through adult FMP on adult aBMD were estimated using general linear regression and a causal steps approach.Results:Significant positive associations between childhood SFT and adult aBMD were found in the LS in men (β = 0.089, P = 0.044) and in all the skeletal sites in women. With respect to the adult fat-bone relationship, high adult FMP was associated with low aBMD in most of the sites in men, but with high FN aBMD in women (β = 0.144, P = 0.002). Moreover, suppressive effects of adult FMP on the associations between childhood SFT and adult aBMD in the LS (-34.8%) and legs (-67.1%) of men, and a positive effect on the FN aBMD in women (17.0%) were identified.Interpretation:Childhood adiposity appears to have a positive long-term effect on adult aBMD, which may be reduced by adiposity in adult men but reinforced by adiposity in adult women.

  • 标签: Child Adiposity Bone mass Longitudinal studies
  • 简介:ADULTMESENCHYMALHAMARTOMAOFTHELIVER:ACASEREPORTWangXianghui王湘辉ChaiFulu柴福录DongLiang董亮LanzhouGeneralHospitalofPLA,Lanzhou730050...

  • 标签: 王湘
  • 简介:AbstractBackground:Acute kidney injury (AKI) is a common and serious complication following lung transplantation (LTx), and it is associated with high mortality and morbidity. This study assessed the incidence of AKI after LTx and analyzed the associated perioperative factors and clinical outcomes.Methods:This retrospective study included all adult LTx recipients at the China-Japan Friendship Hospital in Beijing between March 2017 and December 2019. The outcomes were AKI incidence, risk factors, mortality, and kidney recovery. Multivariate analysis was performed to identify independent risk factors. Survival analysis was presented using the Kaplan-Meier curves.Results:AKI occurred in 137 of the 191 patients (71.7%), with transient AKI in 43 (22.5%) and persistent AKI in 94 (49.2%). AKI stage 1 occurred in 27/191 (14.1%), stage 2 in 46/191 (24.1%), and stage 3 in 64/191 (33.5%) of the AKI patients. Renal replacement therapy (RRT) was administered to 35/191 (18.3%) of the patients. Male sex, older age, mechanical ventilation (MV), severe hypotension, septic shock, multiple organ dysfunction (MODS), prolonged extracorporeal membrane oxygenation (ECMO), reintubation, and nephrotoxic agents were associated with AKI (P < 0.050). Persistent AKI was independently associated with preoperative pulmonary hypertension, severe hypotension, post-operative MODS, and nephrotoxic agents. Severe hypotension, septic shock, MODS, reintubation, prolonged MV, and ECMO during or after LTx were related to severe AKI (stage 3) (P < 0.050). Patients with persistent and severe AKI had a significantly longer duration of MV, longer duration in the intensive care unit (ICU), worse downstream kidney function, and reduced survival (P < 0.050).Conclusions:AKI is common after LTx, but the pathogenic mechanism of AKI is complicated, and prerenal causes are important. Persistent and severe AKI were associated with poor short- and long-term kidney function and reduced survival in LTx patients.

  • 标签: Acute kidney injury Adult lung transplantation Incidence Outcomes Risk factors
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  • 简介:第二与雄激素受体(AR)的敏感为出生前的睾丸激素和covaries作为通常认为的biomarker被建议了到第四位比率(2D:4D)。出生前的睾丸激素和AR在阴茎生长起一个中央作用。在这研究,我们调查了在位比率和阴茎长度之间的关系。在一个单个第三级的学术中心为urological外科被就医的朝鲜的人在这被检查给了知情同意的20岁以上的学习,和144个人有希望地被注册。右边的秒位和第四位的长度被一个单个调查者在阴茎长度的测量以前测量。在麻醉下面,松驰、拉长的阴茎长度被另一个调查者测量没测量也不有任何东西关于位长度的信息。用线性回归模型的Univariate和multivariate分析证明那唯一的高度是为松驰的阴茎长度的一个重要预兆的因素(univariate分析:r=0.185,P=0.026;multivariate分析:r=0.172,P=0.038)并且那唯一的位比率是为拉长的阴茎长度的一个重要预兆的因素(univariateanalysis:r=−;0.216,P=0.009;multivariate分析:r=−;0.201,P=0.024;拉长的阴茎length=−;9.201×;位比率+;20.577)。把证据基于这,我们建议位比率能预言成年阴茎尺寸并且出生前的睾丸激素的效果可以部分地在成年阴茎长度解释差别。

  • 标签: 预测因子 阴茎 成人 比例 单因素分析 长度测量
  • 简介:Theventricularseptumseparatestherightandleftventriclesandthusispartofbothventricles.Itisdirectedobliquelybackwardtotheright,andcurvedwiththeconvexitytowardtherightventricle;itmustbeemphasizedthatthetotalcardiacseptumhasacomplex,longitudinaltwistanddoesnotlieinanysingleplane.Itsupperandposteriorpart,isthinandfibrous,andistermedthemembranousventricularseptum.Thegreaterportionoftheseptumisthickandmuscularandconstitutesthemuscularventricularseptum.Theventricularseptumconsistsoftwolayers,athinlayerontheRVsideandathickerlayerontheLVside[1].Themajorseptalarteriestendtorunbetweenthesetwolayers.

  • 标签: ADULT VENTRICULAR SEPTUM RIGHT VENTRICLE
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  • 简介:ObjectiveTocharacterizemicroRNA(miRNA)expressionprofileinmicrodissectedauditoryepitheliafromtheCorti'sOrganinnewbornandadultrats.MethodsTheTaqManMicroRNAArrayswereusedtoidentifyexpressionofmicroRNAinthenewbornandadultgroups.GOanalysiswasappliedtoanalyzethemainfunctionofthedifferentialexpressiongenesaccordingtotheGeneOntologywhichisthekeyfunctionalclassificationofNCBI.Similarly,PathwayanalysiswasusedtofindoutthesignificantpathwayofthedifferentialgenesaccordingtoKEGG,BiocartaandReatome.ResultsIncreasedexpressionwasseenin16miRNAsinmatureratcomparedtonewbornrats,withincreasedfoldingrangingfrom17to600folds.Expressionlevelsin2miRNAswerereducedinmaturerats,namelyrno-miR-29candrno-miR-29a.Thehigh-enrichmentGOstargetedbyover-expressedmiRNAswerenegativeregulationofepithelialcelldifferentiation,common-partnerSMADproteinphosphorylation,mesenchymal-epithelialcellsignaling,regulationoftransforminggrowthfactorbeta2production,etc.FunctionalanalysisofmiRNAsbyKEGGrevealedthat19signaltransductionpathwayswereupregulatedand14weredownregulated.ConclusionsThedifferenceinmiRNAexpressionpatternsintheorganofCortibetweenneonatalandadultratsmaybecloselyrelatedtomaturationoftheorganofCortiandlossofproliferativecapacityofinnerearhaircells,andTGFβsignalingmayplayanimportantroleinhaircellsregeneration.

  • 标签: 成年大鼠 小分子RNA 表达分析 新生 microRNA MIRNA
  • 简介:Satellitecells(SCs)hasbeenshowntobethesourceofmyogenicprecursorcellsresponsibleformusclefiberrepairandregenerationduringatrophy,afterinjuryorneuromusculardisorders.RecentstudiesdemonstratedthefeasibilityofautologousSCstransplantationforrepairofmyocardialnecrosis.

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  • 简介:客观:调查成年钝脾的损害的非起作用的管理的指示。方法:回顾的评论在所有成年病人(年龄>15年)上被执行,钝脾的损害从1999~2003在法国承认了到Pellegrinhospital的脉管的外科的部门。我们不管年龄在所有适当病人non-operatively管理了脾的损害。结果:在4年期间,有钝脾的损害的54个连续成年病人在医院里被对待。有稳定的血液动力学的地位的27个病人的一个总数起初non-operatively被对待,哪个2个病人被辜负到non-operativetreatment。非起作用的管理的成功的百分比是92.6%。在54个病人,比55年老的7个of8病人与非起作用的管理被对待。二个案例developingpostoperatively代替膈的感染被合适的治疗愈合。在系列,没有死亡。结论:低档的非起作用的管理脾的损害能与可接受的低失败的率被完成。如果临床并且对外科医生到困难的实验室参数作决定,他们能取决于Resciniti“获得系统与脾的损伤选择成年人的一个子集的sCT(计算断层摄影术)是non-operativemanagement的试用的优秀候选人。比55年老的病人绝对没被禁止收到non-operativemanagement。

  • 标签: 手术治疗 脾脏损伤 成年 病理机制
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  • 简介:Objective:Toexplorechemicalcomponentchangesofdogboneatdifferentlengtheningtimeandindifferentboneregionsofinterest,andtoevaluatethemineralizationduringIiizarovlengtheningprocess.Methods:Theashweight,theconcentrationsofcalcium,phosphorusandthecalcium/phosphorusratioweremeasuredatdifferentintervals(2,4,6,8,12weeks)sincelengtheningandthelengthenedpartwascomparedwithacontrolareaateachinterval.Results:Theashweight,theconcentrationsofcalciumandphosphorusinthelengthenedareadifferedatalldevelopmenttime.Thecalcium/phosphorus(Ca/P)ratiointhelengthenedregionremainedsignificantlylowerthanthatinthecontrolregionupto12weeksafterthelengthening.Conclusions:Theseresultssuggestthatalsootherinorganicionsplayanimportantroleinthemineralizationprocessandthattheybecomerelativelymoreimportantsince8weeksafterthelengthening.

  • 标签: 成年犬 骨骼延长术 成骨作用 钙磷成分变化
  • 简介:Aim:Toisolateandtransplantgermcellsfromadultmousetestesfortransplantation.Methods:Inordertodistinguishtransplantedcellsfromendogenouscellsofrecipients,donortransgenicmiceexpressinggreenfluorescentprotein(GFP)wereused.Germcellswerecollectedfromthedonorsat10-12weeksofageandspermatogoniawereconcentratedbypercollfractionationandtransplantedintorecipientseminiferoustubulesthathadbeenpreviouslytreatedwithbusulfanat5weeksofagetoremovetheendogenousspermatogeniccells.Results:Twentyweeksafterthetransplantation,awidespreadGFPsignalwasobservedintherecipientseminiferoustubules.Thepresenceofspermatogenesisandspermatozoawasconfirmedinsectionsof12outof14testestransplanted(86%).However,whengermcellsweretransplantedwithoutconcentrationthesuccessratewaszero(0/9).Conclusion:Germcellsfromadultmousetestescanbesuccessfullytransplantedintorecipientseminiferoustubulesifthecellpopulationisrichinspermatogoniaandthepercollfractionationisusefulinobtainingsuchacellpopulation.

  • 标签: 精原细胞 微生物细胞 细胞移植 成年老鼠 硅石胶态悬浮液分馏法 内生细胞