低鉀血癥臨床診斷思路培訓精選

1、會計學1低鉀血癥臨床診斷思路培訓精選低鉀血癥臨床診斷思路培訓精選23456789101112131415l原發(fā)性l繼發(fā)性l完全性l不完全性l嬰兒型l成人型l暫時性l持續(xù)性l失鉀型l軟骨型l混合型161718192021HCO3-排出分數(shù)=HCO3- （尿）/ HCO3-（血）肌酐（尿）/肌酐（血）15 型；5 型 2223242526272829303132預測值90%3330高度懷疑，50確診擴容試驗（0.9%NaCl500ml/h4-6h 或口服NaCl10g/d3d）原發(fā)性高血壓單側結節(jié)，對側正常高血壓，低血鉀25 立位PAC/PRA原醛？Ald不被抑制Ald被抑制原醛CT可疑雙側大或小

2、結節(jié)病變APA手術腎上腺V導管取血IHA內(nèi)科治療1、輸鹽水后PAC(Ald)280pmol/l(10mg/dl)(5-8)2、口服NaCl后PAC39nmol/d(14ug/d)(10)34353637383940膽固醇孕烯醇酮17-孕烯醇酮去氧表雄酮雄烯二醇孕酮17-去氧皮質(zhì)酮皮質(zhì)酮18-羥皮質(zhì)酮醛固酮17-羥孕酮雄烯二酮睪酮11-去氧皮質(zhì)醇皮質(zhì)醇雌酮雌二醇膽固醇側鏈裂解酶（P450scc）3-羥類固醇脫氫異構酶（3-HSD）17-羥化酶（P450c17，羥化）17，20裂解酶（P450c17，裂解）21-羥化酶（P450c21）11-羥化酶（P450c11）17-羥類固醇氧化還原酶（17-

3、HSD)芳香化酶（P450aro）41膽固醇孕烯醇酮17-孕烯醇酮去氧表雄酮雄烯二醇孕酮17-去氧皮質(zhì)酮皮質(zhì)酮18-羥皮質(zhì)酮醛固酮17-羥孕酮雄烯二酮睪酮11-去氧皮質(zhì)醇皮質(zhì)醇雌酮雌二醇膽固醇側鏈裂解酶（P450scc）3-羥類固醇脫氫異構酶（3-HSD）17-羥化酶（P450c17，羥化）17，20裂解酶（P450c17，裂解）21-羥化酶（P450c21）11-羥化酶（P450c11）17-羥類固醇氧化還原酶（17-HSD)芳香化酶（P450aro）ACTH42膽固醇孕烯醇酮17-孕烯醇酮去氧表雄酮雄烯二醇孕酮17-去氧皮質(zhì)酮皮質(zhì)酮18-羥皮質(zhì)酮醛固酮17-羥孕酮雄烯二酮睪酮11-去氧皮質(zhì)

4、醇皮質(zhì)醇雌酮雌二醇膽固醇側鏈裂解酶（P450scc）3-羥類固醇脫氫異構酶（3-HSD）17-羥化酶（P450c17，羥化）17，20裂解酶（P450c17，裂解）21-羥化酶（P450c21）11-羥化酶（P450c11）17-羥類固醇氧化還原酶（17-HSD)芳香化酶（P450aro）ACTH4344病例核型年齡(歲)血壓(mmHg)K+(mmol/l)ACTH(ng/l)24hUFC(ug)Ald(pmol/)PRA(ugL-3h-1)146,XY32180/1203.411516.02030.40.03246,XX19160/1203.44469.0908.60346,XX34180/

5、1202.83925.31273.60446,XX18120/803.713319.6表1 部分性聯(lián)合缺陷癥患者臨床特點45病例外生殖器月經(jīng)情況LH(U/L)FSH(U/L)E2(pmol/l)T(nmol/l)1Pnder-級-31.928.90.050.72女性幼稚型月經(jīng)稀發(fā)15.814.62530.43成年女性月經(jīng)規(guī)律17.48.1177.60.354女性幼稚型原發(fā)閉經(jīng)19.018.188.10.1表1 部分性聯(lián)合缺陷癥患者臨床特點注：1mmHg=0.133kPa46病例血漿總皮質(zhì)醇（nmol/l）17-OHP(ug/l)0min60min0min60min1173.9209.82.46

6、3.052-4.004.90349.760.71.701.37463.599.42.663.66表2 部分性聯(lián)合缺陷癥患者ACTH興奮試驗結果474817-去氧皮質(zhì)酮膽固醇孕烯醇酮17-孕烯醇酮去氧表雄酮雄烯二醇孕酮皮質(zhì)酮18-羥皮質(zhì)酮醛固酮17-羥孕酮雄烯二酮睪酮11-去氧皮質(zhì)醇皮質(zhì)醇雌酮雌二醇膽固醇側鏈裂解酶（P450scc）3-羥類固醇脫氫異構酶（3-HSD）17-羥化酶（P450c17，羥化）17，20裂解酶（P450c17，裂解）21-羥化酶（P450c21）11-羥化酶（P450c11）17-羥類固醇氧化還原酶（17-HSD)芳香化酶（P450aro）ACTH49505152535

7、45556K(3.5-5.5mmol/l)Na(130-150mmol/l)Ca(2.25-2.75mmol/l)P(0.97-1.62mmol/l)Mg(0.6-1.4mmol/l)Cl(94-110mmol/l)07-1-23 16:401.86140.72.090.620.82106.107-1-24 06:302.56143.92.150.820.76110.107-1-24 16:001.80140.12.100.640.74105.807-1-25 06:302.73144.62.140.900.74111.207-1-25 16:002.60139.72.100.610.7410

8、5.807-1-26 06:303.13149.32.121.020.82109.307-1-26 16:002.67139.72.160.800.78102.707-1-27 06:303.10142.52.100.970.72104.307-1-28 14:003.83139.72.381.360.84106.007-1-29 06:304.22142.72.141.520.86106.157CK(2-100U/L)CK-MB(0-16U/L)LDH(40-250U/L)AKP(0-130U/L)ALT(0-40U/L)AST(0-40U/L)07-1-23 16:4012430.0135

9、.8675.077.0155.0178.007-1-24 06:306048.1117.8611.482.0152.1178.207-1-24 16:0010765.0142.4973.0198.0244.0570.007-1-25 06:305420.5102.2601.7193.6241.2301.507-1-25 16:008330.0123.4534.0152.0173.0196.007-1-26 06:305009.594.7549.2156.0167.5147.707-1-26 16:003253.090.0438.0116.0121.0106.007-1-27 06:303372

10、.660.6514.1142.5115.390.007-1-28 14:001542.084.0397.0114.078.057.007-1-29 06:301478.768.0467.5127.480.262.75826,1600.51.01.52.02.53.03.54.04.51400012000100008000600040000200023,1624,0624,1625,0625,1626,0627,0628,1429,06CK(U/L)血鉀(mmol/l)時間59PH(7.35-7.45)PO2(69-116mmHg)PCO2(32-45mmHg)HCO3-(20-26mM)BE(

11、-3-3mM)SO2(95-99%)07-1-237.489120.034.425.62.4498.707-1-24 7.45096.439.726.92.898.007-1-287.44887.446.331.46.597.560K(mmol/l)Na(mmol/l)Cl(mmol/l)Pr(g)Cr(mmol/l)Ua(mmol/l)07-1-24（3400ml）54.4血鉀2.56266.251.00.517.31.0207-1-26（3300ml）89.1259.7359.7-肌酐清除率：82.1ml/min61PH比重滲透壓(mOsm/L)NAG酶(0-21U/g Cr)2-微球蛋白

12、(0-0.02mg/dl)Alb/Cr(0-37mg/g)微量白蛋白(mg/dl)07-1-2471.00507-1-26-27293.255.031633.656263ACTH(pM)皮質(zhì)醇(nM)0am17.325.7（0-165.7）8am29.3（0-15）25.7(0-797.5)24h尿UFC25.7nM(78.6-589.6)尿量2100ml6465LH(Miu/ml)FSH(IU/L)E2(pmol/l)睪酮(nmol/l)孕酮(nmol/l)0泌乳素(ug/l)07-1-2480.4574.6036.70.0521.2719.43（0-29.2）07-1-2742.5380.

13、6136.70.3531.0913.72（0-29.2）666768697071727374757677尿常規(guī)PH比重糖Mg/dl蛋白Mg/dl06-2-1071.005-06-2-11 51.020502506-2-1471.007-血氣PHHCO3-BE06-2-107.22112.2-14.206-2-137.29115.3-10.178時間NaKCaPMgCl尿酸AKPCO22-10143.92.632.030.741.26119.595.42-11143.33.662.180.851.15119.9100.752513.22-131433.522.160.661.0811917.77

14、98006-2-11ProKNaMgPCaClGlu尿酸血3.461431.150.852.18 119 100 24小時尿0.265575.22.886.821.941250.041.838182838485868788899091929394954/95/97/99/9(mmol/l)1.421.662.582.95血鉀日期96甲功五項實測值正常值TT31.60(1.01-2.95)TT498.3(55.34-160.88)FT34.95(2.76-6.3)FT418.38(10.42-24.32)TSH1.22(0.35-5.50)97臥立位試驗臥位實測值臥位正常值立位實測值立位正常值腎

15、素4.51（0.07-1.47）6（1.5-5.0）血管緊張素55.9（11.8-95.0）376.2（92.5-150.0）醛固酮542.1（163.4-481.9）995.9（180.1-819.9）98血生化KClMgCO28/113.02101.60.6128.39/112.7695.630.611/113.1098.70.5431.4(3.5-5.5)(94-110)(0.6-1.4)(20.2-30)99尿PH鉀糖蛋白8/1179/11780.7mmol11/11745.76mmol0.7566mmol0.1846g100血氣分析PHHCO3-BE9/117.40628.12.61

16、1/11 7.40626.41.4(7.35-7.45)(20-26)(-3-+3)101102103104105106病例血漿總皮質(zhì)醇（nmol/l）17-OHP(ug/l)0min60min0min60min1173.9209.82.463.052-4.004.90349.760.71.701.37463.599.42.663.66表2 部分性聯(lián)合缺陷癥患者ACTH興奮試驗結果10717-去氧皮質(zhì)酮膽固醇孕烯醇酮17-孕烯醇酮去氧表雄酮雄烯二醇孕酮皮質(zhì)酮18-羥皮質(zhì)酮醛固酮17-羥孕酮雄烯二酮睪酮11-去氧皮質(zhì)醇皮質(zhì)醇雌酮雌二醇膽固醇側鏈裂解酶（P450scc）3-羥類固醇脫氫異構酶（3-HSD）17-羥化酶