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近端肾小管性酸中毒
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<span> [[近端肾小管性酸中毒]]<span lang="EN-US">(proximal renal tubular acidosis</span>,<span lang="EN-US">PRTA)</span>又称<span lang="EN-US"> Ⅱ</span>型<span lang="EN-US">[[RTA]]</span>。本病是由于近端肾小管[[重吸收]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>功能有缺陷,肾<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>-</span>[[阈值]]降低,尿液过多丢失<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>使[[血浆]]中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度下降而产生的高氯性[[酸中毒]]。<span lang="EN-US"></span></span> ==近端肾小管性酸中毒的病因== <span> <span lang="EN-US">(</span>一<span lang="EN-US">)</span>发病原因<span lang="EN-US"></span></span> <span> <span lang="EN-US">1.</span>[[原发性]] 病因不明,一般认为与[[遗传]]有关。仅表现为<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>再吸收障碍,不伴有其他[[肾小管]]和[[肾小球]]功能障碍。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(1)</span>散发性<span lang="EN-US"><span> </span></span>[[婴儿]]为暂时性。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(2)</span>遗传性<span lang="EN-US"><span> </span></span>为持续性,呈[[常染色体]]显性遗传或[[常染色体隐性遗传]]。<span lang="EN-US"></span></span> <span> <span lang="EN-US">2.</span>[[继发性]] 常继发于全身性[[疾病]],可伴多种肾小管功能异常,以范可尼<span lang="EN-US">(Fanconi)</span>[[综合征]]最为多见。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(1)</span>伴其他[[遗传病]]:伴有其他近端肾小管[[功能障碍]]的[[遗传性疾病]],如特发性[[范可尼综合征]]、[[胱氨酸病]]、眼<span lang="EN-US">-</span>脑<span lang="EN-US">-</span>[[肾综合征]]<span lang="EN-US">(Lowe</span>综合征<span lang="EN-US">)</span>、[[遗传性果糖不耐受症]]、[[酪氨酸血症]]、[[半乳糖血症]]、[[糖原贮积病]]、[[线粒体肌病]]、[[异染性脑白质营养不良]]等。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(2)</span>药物和[[毒素]][[肾损害]]:如[[碳酸酐酶]]抑制物、过期[[四环素]]、甲基<span lang="EN-US">3-</span>[[色酮]]、[[马来酸]][[中毒]]、重金属<span lang="EN-US">(</span>钙、铅、铜、汞<span lang="EN-US">)</span>中毒等。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(3)</span>其他:如[[亚急性坏死性脑脊髓病]]<span lang="EN-US">(Leigh</span>综合征<span lang="EN-US">)</span>、[[法洛四联症]]、[[肠吸收不良]]、[[甲状旁腺功能亢进]]、[[肾囊肿]]病、[[遗传性肾炎]]、[[肾移植]][[慢性排斥反应]]、[[多发性骨髓瘤]]、<span lang="EN-US">Sjögren</span>综合征、[[淀粉样变性]]、[[慢性活动性肝炎]]、复发性[[肾结石]]、[[肾髓质囊性病]]、<span lang="EN-US">Wilson</span>病等。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(</span>二<span lang="EN-US">)</span>发病机制<span lang="EN-US"></span></span> <span> 在正常情况下,肾小球滤过的<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>99%</span>被[[重吸收]],其中近端小管重吸收<span lang="EN-US">80%</span>~<span lang="EN-US">90%</span>,其余<span lang="EN-US">2%</span>在髓襻,<span lang="EN-US">8%</span>在远端小管重吸收。而<span lang="EN-US">HCO3-</span>重吸收和小[[管细胞]]分泌<span lang="EN-US">H+</span>的功能密切相关。在小管中<span lang="EN-US">H<sup>+</sup>- [[Na]]<sup>+</sup></span>交换,<span lang="EN-US">Na<sup>+</sup></span>被重吸收入细胞内与<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>结合成<span lang="EN-US">NaHCO3</span>,再进入[[血液]]中,为身体保留了[[碱储备]]。依赖<span lang="EN-US">Na<sup>+</sup>- K<sup>+</sup>-[[ATP]]</span>酶泵的活性,近端小管重吸收[[肾小球滤液]]中大部分的钠,<span lang="EN-US">Cl-</span>和水随<span lang="EN-US">Na<sup>+</sup></span></span><span>被动重吸收。另外,近端小管主动重吸收全部<span lang="EN-US">K<sup>+</sup></span>、<span lang="EN-US">2/3</span>钙和部分[[磷酸盐]]。<span lang="EN-US">PRTA</span>为近端肾小管重吸收<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>不足,<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[肾阈]]降低,正常人为<span lang="EN-US">25</span>~<span lang="EN-US">26mmol/L</span>,婴儿为<span lang="EN-US">22mmol/L</span>,而<span lang="EN-US">PRTA</span>时为<span lang="EN-US">18</span>~<span lang="EN-US">20mmol/L</span>。当患者[[血浆]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度正常时,即有<span lang="EN-US">15%</span>以上的<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>排至尿中<span lang="EN-US">(</span>正常人仅为<span lang="EN-US">1%)</span>。即使在轻度[[酸中毒]]时,若患者血浆中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度仍高于肾阈,则<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>仍排至尿中。只有严重酸中毒时,患者可排出酸性尿。<span lang="EN-US"></span></span> <span> 由于近端肾小管对<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>重吸收减少,使<span lang="EN-US">Na<sup>+</sup>- H<sup>+</sup></span>交换减少,<span lang="EN-US">Na<sup>+</sup></span></span><span>从尿中大量丢失,引起低钠、[[脱水]]。失<span lang="EN-US">Na<sup>+</sup></span>导致继发性醛固酮增多,使<span lang="EN-US">Na<sup>+</sup></span></span><span>、<span lang="EN-US">C l<sup>-</sup></span>[[潴留]]。加之由于<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>丢失增多,为维持阴离子平衡,而保留<span lang="EN-US">C l<sup>-</sup></span>,因而出现[[高氯血症]]。在[[醛固酮]]作用下,以<span lang="EN-US">Na<sup>+</sup>- K<sup>+</sup></span>交换而保留<span lang="EN-US">Na<sup>+</sup></span>,可引起[[低钾血症]],长期[[代谢性酸中毒]]可能通过阻碍[[生长激素]]的分泌或应答而引起[[生长发育]]障碍。导致近端肾小管重吸收<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>障碍的原因尚不清楚,可能是由于肾小管功能发育不成熟。在继发性病因中,大都是由于内生[[代谢]]产物或外来物质损坏近端小管[[上皮]]引起。</span> ==近端肾小管性酸中毒的症状== <span> 本[[病症]]状通常较轻,表现为生长迟缓,[[营养不良]],易乏,软弱无力,[[厌食]]、[[多尿]]、烦渴或有[[低钾血症]]。典型病例有高氯酸[[血症]],但远端肾小管酸化功能正常,尿<span lang="EN-US">pH</span>能降至<span lang="EN-US">5.5</span>以下,或伴有骨损害<span lang="EN-US">(</span>[[骨软化]]、[[骨质疏松]]<span lang="EN-US">)</span>,[[糖尿]]、[[氨基酸尿]]等。<span lang="EN-US">Ⅱ</span>型<span lang="EN-US">PRTA</span>主要临床特点为:<span lang="EN-US"></span></span> <span> [[原发性]]<span lang="EN-US">PRTA</span>主要见于男性[[婴儿]],多伴其他近端肾小管[[重吸收]]功能缺陷如糖尿、磷尿等,在<span lang="EN-US">1</span>~<span lang="EN-US">2</span>岁可自发消失。<span lang="EN-US"></span></span> <span> 患者由于[[代谢性酸中毒]]与低钠、低钾血症可出现[[生长发育]]迟缓、[[恶心]]、[[呕吐]]等酸性[[中毒]]以及软弱、[[疲乏]]、[[肌无力]]、[[便秘]]等[[低钠血症]]和低钾血症表现。由于<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[肾阈]]在<span lang="EN-US">PRTA</span>时降至<span lang="EN-US">15</span>~<span lang="EN-US">18mmol/L</span>,低于<span lang="EN-US">15mmol/L</span>后可排酸性尿<span lang="EN-US">(pH&lt;5.5)</span>,严重[[酸中毒]]少见。<span lang="EN-US"></span></span> <span> 另外如不伴近端小管磷[[吸收障碍]]则无高磷尿症,并很少出现[[代谢性骨病]]、[[肾钙化]]、[[肾结石]]。而非选择性患者,则可出现[[尿磷]]增多,[[葡萄糖尿]]、氨基酸尿等。<span lang="EN-US"></span></span> <span> [[继发性]]<span lang="EN-US">PRTA</span>除上述表现外,还有原发病[[症状]]。且易被原发病的症状所掩盖,应提高警惕继发性<span lang="EN-US">PRTA</span>的发生。<span lang="EN-US"></span></span> <span> 本型多见于男性儿童,[[幼年期]]发病,有些随年龄增长而自行缓解。症状通常较轻,表现生长迟缓,营养不良,易乏,软弱无力,厌食、多尿、烦渴,或有低钾血症。典型病例有高氯酸血症,但远端肾小管酸化功能正常,尿<span lang="EN-US">pH</span>能降至<span lang="EN-US">5.5</span>以下。或有骨损害<span lang="EN-US">(</span>骨软化、骨质疏松<span lang="EN-US">)</span>,糖尿、氨基酸尿等。依据前述表现及[[实验室检查]]诊断可以成立。必要时作碳酸氢盐重吸收试验和肾<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[阈值]]测定,如尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[排泄]]率为滤过量的<span lang="EN-US">15%</span>以上部分可确诊。<span lang="EN-US"></span></span> <span> <span lang="EN-US">Ⅱ</span>型<span lang="EN-US">[[RTA]]</span>的诊断要点:<span lang="EN-US"></span></span> <span> <span lang="EN-US">1.</span>高氯性酸中毒 除外非肾原性[[疾病]]所致者。如代谢性酸中毒严重<span lang="EN-US">[</span>[[血浆]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>&lt;15</span>~<span lang="EN-US">18mmol/L</span>,而晨尿<span lang="EN-US">pH≤5.5</span>,<span lang="EN-US">NH<sub>4</sub><sup>+</sup></span>,排量<span lang="EN-US">&gt;40μmol/(min.1.73m2)</span>,且排除自[[胃肠道]]丢失<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>,可诊断本病。<span lang="EN-US"></span></span> <span> <span lang="EN-US">2.</span>不明原因的低钾血症、[[低磷血症]],[[尿糖]]阳性、尿钾升高、尿磷升高和高尿酸盐尿症。<span lang="EN-US"></span></span> <span> <span lang="EN-US">3.</span>尿<span lang="EN-US">pH&gt;6.0</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">4.</span>酸、碱负荷试验阳性。<span lang="EN-US"></span></span> ==近端肾小管性酸中毒的诊断== ===近端肾小管性酸中毒的检查化验=== <span> <span lang="EN-US">1.</span>[[血液]][[生化]]检查 [[血浆]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>和<span lang="EN-US">pH</span>降低、[[高氯血症]],钠、钾正常或下降。<span lang="EN-US"></span></span> <span> <span lang="EN-US">2.</span>尿液检查 尿<span lang="EN-US">pH</span>根据血<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>水平可呈碱性或酸性。<span lang="EN-US">24h</span>尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>仅可[[滴定]]酸正常,尿钙可增高或正常测尿<span lang="EN-US">P CO<sub>2</sub> </span>时可注入<span lang="EN-US">[[Na]] HCO<sub>3</sub></span>使尿液[[碱化]],当尿<span lang="EN-US">pH&gt;</span>血<span lang="EN-US">pH</span>时,尿<span lang="EN-US">P CO<sub>2</sub>&gt;</span>血<span lang="EN-US">P CO<sub>2</sub>2.66kPa</span>或更多则有诊断意义<span lang="EN-US">)</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">3.</span>尿[[胱氨酸]]检查 [[近曲小管]]疾患时常存在[[胱氨酸尿]],如阳性则有助于诊断<span lang="EN-US">(</span>氰化物硝基氢氰酸盐试验:取尿液<span lang="EN-US">5ml</span>加[[浓氨水]]<span lang="EN-US">1</span>滴,<span lang="EN-US">5%</span>的氰化钠<span lang="EN-US">3</span>滴,呈紫红色反应为阳性<span lang="EN-US">)</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">4.</span>[[酸负荷试验]] 方法见<span lang="EN-US">Ⅰ</span>型<span lang="EN-US">[[RTA]]</span>,在酸负荷试验中,如尿<span lang="EN-US">pH&lt;5.5</span>或更低,则诊断<span lang="EN-US">Ⅱ</span>型<span lang="EN-US">RTA</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">5.</span>碱负荷试验<span lang="EN-US"></span></span> <span> <span lang="EN-US">(1)</span>口服[[碳酸氢钠]]法:从<span lang="EN-US">1mmol/(kg.d)</span>开始,逐天加量至<span lang="EN-US">10mmol/(kg.d)</span>,[[酸中毒]]被纠正后,测血、尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度与[[肾小球滤过率]],计算尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>的百分率:尿中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>量<span lang="EN-US">=</span>尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>mmol/L)×</span>[[尿量]]<span lang="EN-US">(ml/min)/</span>血浆<span lang="EN-US">HCO<sub>3</sub><sup>-</sup> (mmol/L)×GFR</span>。正常人尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>为零;<span lang="EN-US">Ⅱ</span>型、混合型<span lang="EN-US">RTA&gt;15%</span>,<span lang="EN-US">I</span>型<span lang="EN-US">RTA&lt;3%</span>~<span lang="EN-US">5%</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(2)</span>[[静脉滴入]]碳酸氢钠法:以<span lang="EN-US">4ml/min</span>的速度滴入<span lang="EN-US">5% Na HCO<sub>3</sub></span>,持续<span lang="EN-US">2h</span>。注入前测患者血<span lang="EN-US">pH</span>、<span lang="EN-US">PCO<sub>2</sub></span>、<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度和尿<span lang="EN-US">pH</span>、<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度;以后分别在注入<span lang="EN-US">30</span>,<span lang="EN-US">90min</span>后测血<span lang="EN-US">pH</span>、<span lang="EN-US">PCO<sub>2</sub></span>、<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度;<span lang="EN-US">60</span>,<span lang="EN-US">120min</span>测尿<span lang="EN-US">pH</span>和<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度,在患者血<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度恢复正常时,尿中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[排泄]]量<span lang="EN-US">&gt;</span>[[肾小球]]滤过量的<span lang="EN-US">15%</span>,提示近曲小管<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>[[吸收障碍]]。尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>排泄分数<span lang="EN-US">=(</span>尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>/</span>血浆<span lang="EN-US">HCO<sub>3</sub><sup>-</sup>/(</span>尿[[肌酐]]<span lang="EN-US">/</span>血肌酐<span lang="EN-US">)</span>。血浆<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度正常时,<span lang="EN-US">Ⅱ</span>型<span lang="EN-US">RTA HCO<sub>3</sub><sup>-</sup></span>排泄分数<span lang="EN-US">&gt;15%</span>,<span lang="EN-US">Ⅰ</span>型<span lang="EN-US">RTA&lt;5%</span>。此法可鉴别<span lang="EN-US">Ⅰ</span>型、<span lang="EN-US">Ⅱ</span>型<span lang="EN-US">RTA</span>。<span lang="EN-US"></span></span> <span> 常规做[[心电图]]、[[影像学]]检查和<span lang="EN-US">B</span>超检查。<span lang="EN-US"></span></span> ===近端肾小管性酸中毒的鉴别诊断=== <span> <span lang="EN-US">1.</span>与氮质[[潴留]]所致[[酸中毒]]的其他[[疾病]]鉴别,如[[腹泻]]、[[酮症酸中毒]]等。<span lang="EN-US"></span></span> <span> <span lang="EN-US">2.</span>和其他类型[[肾小管性酸中毒]]鉴别。尤其应与<span lang="EN-US">Ⅰ</span>型相鉴别。<span lang="EN-US"></span></span> <span> 本病的主要[[临床表现]]为高氯[[血症]]性[[代谢性酸中毒]]。年幼儿童[[生长发育]]迟缓常为本病最主要、甚至是惟一表现,因此对[[发育迟缓]]患儿,应高度注意有无<span lang="EN-US">PRTA</span>。凡遇难以纠正的[[脱水]]和酸中毒时,应警惕本病可能,并作相应检查,应用碳酸氢盐或[[枸橼酸]][[缓冲液]]的量须在<span lang="EN-US">6mmol/(kg.d)</span>方可维持[[血浆]]<span lang="EN-US">CO<sub>2</sub></span>结合力于<span lang="EN-US">22mmol/L</span>,此点可与远端<span lang="EN-US">[[RTA]]</span>相鉴别。尿浓缩[[功能障碍]]比远端<span lang="EN-US">RTA</span>时为轻。<span lang="EN-US"></span></span> ==近端肾小管性酸中毒的并发症== [[营养障碍]]、[[代谢性酸中毒]]、[[低钾血症]]、软骨病,[[生长发育]]迟缓。 ==近端肾小管性酸中毒的预防和治疗方法== [[原发性]]者因病因不明,无可靠预防方法,临床上主要对继发于药物和[[毒素]][[肾损害]]和其他如[[肠吸收不良]]、[[甲状腺功能亢进]]等[[疾病]]者,给予积极防治,以防[[代谢性酸中毒]]迁延造成全身代谢紊乱及[[肾功能]]损害。 ===近端肾小管性酸中毒的西医治疗=== <span> <span lang="EN-US">(</span>一<span lang="EN-US">)</span>治疗<span lang="EN-US"></span></span> <span> 本病无特效[[疗法]],一般采用对症治疗,以补充丢失的<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>中和内生酸性物质。<span lang="EN-US"></span></span> <span> <span lang="EN-US">1.</span>病因治疗 如治疗药物或[[金属中毒]]、[[多发性骨髓瘤]]、[[肾病综合征]]、[[肾小管]]间质性[[疾病]]等。<span lang="EN-US"></span></span> <span> <span lang="EN-US">2.</span>纠正[[酸中毒]] 轻症者如[[症状]]很轻微可暂不服药,随访观察。症状明显时予以碱剂治疗,常用的[[碳酸氢钠]],一般开始剂量每天<span lang="EN-US">5</span>~<span lang="EN-US">10mmol/kg</span>。由于服药后血中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度增高,尿中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>排量也增加,故常需大剂量多次投药,有时可增至每天<span lang="EN-US">10</span>~<span lang="EN-US">25mmol/kg</span>以维持血中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>恒定浓度,以上剂量可分次口服。由于<span lang="EN-US">PRTA</span>对补碱有一定抵抗性,因此碱性药物多<span lang="EN-US">2</span>~<span lang="EN-US">3</span>倍于<span lang="EN-US">DRTA</span>时的剂量。但应用大剂量钠盐时<span lang="EN-US">,</span>肾小管<span lang="EN-US">[[Na]]<sup>+</sup> - K<sup>+</sup></span>交换增加,会加重失钾,甚至严重失钾。因此要同时补钾并要低盐<span lang="EN-US">(</span>[[氯化钠]]<span lang="EN-US">)</span>饮食,以减少高氯酸中毒和失钾,限钠入量也减少尿<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>的[[排泄]]。因此症多见于婴幼儿,儿童补<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>的量大约<span lang="EN-US">10mmol/(kg.d)</span>,此后以维持血中<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度于正常范围而调整剂量。也可应用[[枸橼酸盐]][[缓冲液]]:[[枸橼酸钠]]<span lang="EN-US">50g</span>,[[枸橼酸钾]]<span lang="EN-US">50g</span>,[[枸橼酸]]<span lang="EN-US">100g</span>,加水至<span lang="EN-US">1000ml</span>,口服<span lang="EN-US">3</span>次<span lang="EN-US">/d</span>,每次<span lang="EN-US">50ml</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">3.</span>补钾 轻症一般无须给钾盐,但重症或使用[[利尿药]]时必须给钾。因利尿药可使症状改善和[[尿量]]减少,而不能使[[血浆]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度恢复正常,失钾却见增加。补钾常用枸橼酸钾合剂。<span lang="EN-US"></span></span> <span> <span lang="EN-US">4.</span>利尿药应用 当不能耐受大剂量碳酸氢盐或重症时,单独用碱盐难以奏效,补给的碱盐迅速经尿排出,补的多丢失亦多,酸中毒不易纠正,即需合用利尿药。诸利尿药中[[氢氯噻嗪]]<span lang="EN-US">(</span>[[双氢克尿塞]]<span lang="EN-US">)</span>最有效,作用机制为引起[[利尿]]后产生轻度[[脱水]],[[细胞]]外液容量缩减,促使近端肾小管[[重吸收]]<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>增加,酸中毒得以纠正。另外可促使肾小管对钙重吸收使血钙上升,尿钙排出减少,[[甲状旁腺素]]分泌减低,解除抑制<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>再吸收,更进一步使血浆<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>增高,酸中毒得以纠正。髓襻利尿药如[[呋塞米]]、[[依他尼酸]]<span lang="EN-US">(</span>[[利尿酸]]<span lang="EN-US">)</span>、[[布美他尼]]<span lang="EN-US">(</span>[[丁尿胺]]<span lang="EN-US">)</span>等使尿钙排泄增加<span lang="EN-US">,</span>不能提高血浆<span lang="EN-US">HCO<sub>3</sub><sup>-</sup></span>浓度,<span lang="EN-US">Ⅱ</span>型<span lang="EN-US">[[RTA]]</span>禁忌使用。氢氯噻嗪<span lang="EN-US">(</span>双氢克尿塞<span lang="EN-US">)</span>剂量,每天<span lang="EN-US">1.5</span>~<span lang="EN-US">2mg/kg</span>,分<span lang="EN-US">2</span>次口服。治疗中应注意[[低血钾]]的发生。酸中毒纠正后减少至维持量。<span lang="EN-US"></span></span> <span> <span lang="EN-US">5.</span>[[补钙]]及[[维生素]]<span lang="EN-US">D </span>对有骨损害<span lang="EN-US">(</span>一般较轻<span lang="EN-US">)</span>,如[[骨质疏松]]、[[骨软化]]者应补充钙剂、维生素<span lang="EN-US">D</span>、[[蛋白合成]]剂等,治疗和注意事项同<span lang="EN-US">Ⅰ</span>型<span lang="EN-US">RTA</span>。<span lang="EN-US">Ⅱ</span>型近端小管磷重吸收有缺陷,[[尿磷]]丢失,若血磷低,每天补磷<span lang="EN-US">1</span>~<span lang="EN-US">3g</span>。[[磷酸盐]]合剂配方:[[磷酸二氢钠]]<span lang="EN-US">NaH<sub>2</sub>PO<sub>4</sub>18g</span>,[[磷酸氢二钠]]<span lang="EN-US">NaH<sub>2</sub>PO<sub>4</sub>145g</span>加水溶解至<span lang="EN-US">1000ml(</span>每毫升含<span lang="EN-US">20mg</span>磷<span lang="EN-US">)</span>,每次口服<span lang="EN-US">20ml</span>,<span lang="EN-US">4</span>~<span lang="EN-US">5</span>次<span lang="EN-US">/d</span>。注意剂量大时可引起[[呕吐]]、[[腹部不适]]、[[腹泻]]等,缓慢增加剂量多数患者多能耐受。若长期服用磷盐治疗可能会发生[[高磷血症]]、继发甲旁亢,应监测[[血清]]磷水平并维持在<span lang="EN-US">1</span>~<span lang="EN-US">1.3mmol/L</span>。<span lang="EN-US"></span></span> <span> <span lang="EN-US">(</span>二<span lang="EN-US">)</span>预后<span lang="EN-US"></span></span> <span> [[原发性]]<span lang="EN-US">PRTA</span>常为[[自限性疾病]],常随年龄增长而缓解,本型若能及早治疗,坚持用药数年,一般预后良好,部分轻症可自愈;若未能早期诊断,可因酸中毒或[[低钾血症]]死亡。[[继发性]]者预后取决于原发病。<span lang="EN-US"></span></span> ==近端肾小管性酸中毒的护理== [[原发性]]者因病因不明,无可靠预防方法,临床上主要对继发于药物和[[毒素]][[肾损害]]和其他如[[肠吸收不良]]、[[甲状腺功能亢进]]等[[疾病]]者,给予积极防治,以防[[代谢性酸中毒]]迁延造成全身代谢紊乱及[[肾功能]]损害。 ==参看== *[[肾内科疾病]] <seo title="近端肾小管性酸中毒,近端肾小管性酸中毒症状_什么是近端肾小管性酸中毒_近端肾小管性酸中毒的治疗方法_近端肾小管性酸中毒怎么办_医学百科" metak="近端肾小管性酸中毒,近端肾小管性酸中毒治疗方法,近端肾小管性酸中毒的原因,近端肾小管性酸中毒吃什么好,近端肾小管性酸中毒症状,近端肾小管性酸中毒诊断" metad="医学百科近端肾小管性酸中毒条目介绍什么是近端肾小管性酸中毒,近端肾小管性酸中毒有什么症状,近端肾小管性酸中毒吃什么好,如何治疗近端肾小管性酸中毒等。近端肾小管性酸中毒(proximal re..." /> [[分类:肾内科疾病]] {{导航板-中毒}}
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