醫(yī)學(xué)遺傳學(xué)測(cè)試資料：Molecular and bioche

1.Afewweeksafterminorsurgery,a14-year-oldboy'sroutinelaboratorytestingreveals0.4mg/dlofdirectbilirubin(直接膽紅素)and1.6mg/dlofindirectbilirubin(間接膽紅素).Thesolephysicalfindingisscleralicterusandnootherabnormalitiescanbedetected.Whatisthe(10.0)A.Crigler-NajjarsyndromeTypeI先天性葡萄糖醛酸轉(zhuǎn)移酶缺乏癥B.Crigler-NajjarsyndromeTypeIIC.Gilbert'ssyndrome吉伯特氏癥候群D.Rotor'ssyndromeE.DubinJohnsonsyndrome吉伯特（Gibert）氏病是一種先天性、非溶血性黃疸，它是由于肝細(xì)胞竇側(cè)微絨毛對(duì)膽紅素的攝取障礙所致總膽紅素：間接膽紅素偏高，直接膽紅素偏高，說(shuō)明肝細(xì)胞性黃疸，肝細(xì)胞受到損害，肝功能減退，肝臟不能完全將間接膽紅素轉(zhuǎn)化為直接膽紅素，同時(shí)肝內(nèi)膽管受壓引起了排泄障礙，直接膽紅也不能完全排到膽道，同時(shí)有可能伴有急性黃疸型肝炎，慢性活動(dòng)性肝炎，肝硬化，肝癌等疾病。直接膽紅素：說(shuō)明是由阻塞性黃疸造成的。間接膽紅素：說(shuō)明可能是溶血性黃疸造成的，直接膽紅素升高也可能會(huì)有輸血時(shí)血型不合，貧血等原因在肝功能化驗(yàn)里，膽紅素正常值范圍如下：[總膽紅素]1.71～17.1μmol/L（0.1mg/dl～1.0mg/dl）[直接膽紅素]0～3.42μmol/L（0～0.2mg/dl）[間接膽紅素0～13.68μmol/L（0～0.8mg/dl）2.Whichofthefollowingclassesoflipoproteins(脂蛋白)resultsfromtheinteractionofVLDLandlipoproteinlipase?(10.0)A.VLDL極低密度脂蛋白B.LDL低密度脂蛋白C.IDLD.HDL高密度脂蛋白E.Chylomicronremnant3.Elevationofwhatsubstanceisthelikelycauseofyellowskininahypothyroidadolescent?(10.0)什么物質(zhì)的增多導(dǎo)致青少年黃皮膚由于患者缺乏甲狀腺激素，胡蘿卜素不能轉(zhuǎn)變?yōu)榫S生素A，所以有胡蘿卜素血癥，皮膚顯現(xiàn)黃白色A.Carnitine卡尼?。ㄈ舛緣A）B.Carotene胡蘿卜素C.Directbilirubin直接膽紅素D.Indirectbilirubin間接膽紅素E.Totalbilirubin總膽紅素4.Deficiencyoflysosomala-1-4-and1-6glucosidase(acidmaltase)ischaracteristicofwhichoneofthefollowingdiseases?(10.0)A.Forbesdisease 糖原沉積Ⅲ型1,6B.Pompe'sdiseaseⅡ型1,4C.AndersondiseaseⅣ1,4-1,6D.Tarui'sdiseaseⅦ肌磷酸果糖激酶5.Inwhatchildhooddiseasedoesammonia（氨氣）reachdangerouslevelsinuntreatedcases(10.0)A.DiabetesmellitusB.Mediumchainacyl-CoAdehydrogenasedeficiencyC.VonGierke'sdiseaseD.OTCDE.Phenylketonuria6.Inwhatchildhooddiseasedoketonebodies（酮體）reachdangerouslevelsinuntreatedcases?(10.0)A.Diabetesmellitus糖尿病丙酮含量的升高則顯示患有糖尿病B.Medium-chainacyl-CoAdehydrogenasedeficiency中鏈?；o酶A脫氫酶缺乏癥脂肪酸氧化失調(diào)C.VonGierke'sdisease（GSDIa型糖原累積癥）D.OTCD鳥氨酸氨甲?；D(zhuǎn)移酶缺陷癥該酶缺陷是人類尿素循環(huán)疾病中最常見的原因，可導(dǎo)致氨蓄積，引起嚴(yán)重的神經(jīng)功能紊亂。E.Phenylketonuria苯丙酮尿癥7.Inwhatchildhooddiseasedoesfreefattyacids（游離脂肪酸）reachdangerouslevelsinuntreatedcases?(10.0)A.DiabetesmellitusB.Mediumchainacyl-CoAdehydrogenasedeficiencyC.VonGierke'sdiseaseD.OTCDE.Phenylketonuria8.Inwhatchildhooddiseasedoesphenylalanine（苯丙氨酸）reachdangerouslevelsinuntreatedcases?(10.0)A.DiabetesmellitusB.Mediumchainacyl-CoAdehydrogenasedeficiencyC.VonGierke'sdiseaseD.OTCDE.Phenylketonuria9.Whathormonestimulateshypertrophyandhyperplasiawithanincreaseinorganfunction?(10.0)（過(guò)度肥大和器官功能的增生）A.GlucagonB.InsulinC.ThyronineD.SomatotropinE.Cortisol10.W