PHOSPHATE MOB NTSHAV QAB ZIB HAUV COV MENYUAM YAUS: PATHOGENESIS, CHAW KHOMOB POM ZOO THIAB KHO MOB

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Ntshav qab zib phosphate yog ib qho ntawm cov kev mob uas tsis tshua muaj nyob hauv kev xyaum tsiaj txhu.

Qhov no yog cov kab mob tshwm sim los ntawm caj ces, hauv tsev kho mob zoo ib yam li rickets, uas tau tshwm sim tawm tsam keeb kwm ntawm qhov tsis txaus phosphate reabsorption hauv cov hlab hlaus.

Cov kev hloov pauv no ua rau kev txhim kho hypophosphatemia, kev poob siab tsis txaus ntawm calcium thiab phosphorus hauv txoj hnyuv thiab, vim li no, pob txha tsis zoo.

Raws li koj paub, cov tseem ceeb tshaj plaws ntawm cov pob txha pob txha yog calcium, uas tus neeg tau txais los ntawm ib puag ncig nrog rau cov zaub mov nqus. Qhov nqus ntawm cov khoom siv tshuaj lom neeg no yog yooj yim los ntawm phosphorus compounds.

Lub pathogenesis ntawm phosphate mob ntshav qab zib yog cuam tshuam nrog kev hloov caj ces ua rau muaj kev cuam tshuam hauv cov txheej txheem ntawm phosphate los ntawm cov hlab ntsha mus rau hauv cov hlab ntsha, uas tom qab ntawd ua rau muaj kev cuam tshuam ntawm pathological cuam tshuam nrog pob txha demineralization, lawv deformation thiab nce fragility.

Vim li cas tus kab mob thiaj li tshwm sim?

Cov mob ntshav qab zib phosphate hauv cov menyuam yaus yog qhov tshwm sim los ntawm kev hloov pauv hauv ib ntawm caj ces nyob ntawm X lub cev hluav taws xob.

Tus kab mob yog tau txais raws li hom tseem ceeb, uas yog, los ntawm leej txiv mob rau txhua tus ntxhais, thiab los ntawm leej niam mob mus txog 50% ntawm cov tub yug thiab 25% ntawm cov ntxhais (qhia tias tsuas yog tus poj niam muaj mob los ntawm tus kab mob).

Hauv kev xyaum ua paum, kev mob qog ntshav hypophosphatemic rickets, uas tshwm sim nrog mob qog ntshav ntawm lub raum, kuj tseem kuaj tau.

Hauv cov tshuaj, paraneoplastic phosphate mob ntshav qab zib kuj tseem txawv, uas tshwm sim vim qhov nce ntau ntawm parathyroid hormone yam los ntawm cov qog hlwb. Ntxiv rau, kev mob caj ceg no yog ib feem ntawm cov kab mob hauv lub nruab nrog cev, tshwj xeeb tshaj yog Fanconi syndrome.

Tus mob no li cas thiaj paub?

Hauv cov tshuaj kho mob niaj hnub no, nws yog ib txwm coj los cais plaub yam tseem ceeb ntawm hypophosphatemic rickets, uas tau muab tshuaj los ntawm thawj xyoo ntawm lub neej ntawm tus neeg mob me thiab muaj lawv tus kheej cov yam ntxwv.

Hom I phosphate mob ntshav qab zib lossis ntshav qab zib hypophosphatemia txuas nrog X chromosome yog kab mob muaj keeb, qhov tseem ceeb uas ua rau muaj kev cuam tshuam ntawm kev rov qab phosphorus hauv cov hlab ntsha rov qab, uas ua rau muaj kev txo qis ntawm cov phosphates hauv cov ntshav, kev txhim kho phosphaturia thiab qhov tshwm sim ntawm rickets-zoo li kev hloov ntawm cov pob txha mos uas tiv taus kev txais tos. tsim nyog cov tshuaj vitamin D

Tus kab mob kis tau pom tseeb thaum thawj ob xyoos ntawm tus menyuam lub neej thiab raug pom los ntawm cov tsos mob ntawm cov tsos mob hauv qab no:

kev loj hlob sai ntawm lub keeb kwm yav dhau ntawm cov nqaij pob txha muaj zog;
tab tom ua tiav lossis ua kom tiav qhov txha hniav laus ntawm cov hniav;
plaub hau poob
hais tau valgus deformity ntawm qhov qis dua, tshwm nrog rau thawj kauj ruam ntawm tus menyuam;
hluav taws xob xoo hluav taws xob ntawm cov rickets, uas hloov zuj zus nrog cov koob tshuaj vitamin D ib txwm muaj;
txo qis hauv theem ntawm phosphates hauv cov ntshav thiab lawv cov zis ntxiv ntxiv;
ncua kev tsim cov leeg ua haujlwm raws txoj cai hnub nyoog.

Ntshav Qab Zib Hom Ntshav Qab Zib II Hom Ntshav Qab Zib yog hloov pauv ntawm caj ces ntawm hypophosphatemia, uas yog autosomal tseem ceeb thiab tsis txuas nrog rau tus txiv neej X chromosome.

Mob Ntshav Qab Zib Hom II II yog cov tsos mob xws li:

thawj cov kabmob ntawm tus kabmob txij thaum muaj hnub nyoog 12 lub hlis;
qhov tseem ceeb curvature ntawm qis dua pob txha thiab deformation ntawm lub cev pob txha;
tsis muaj kev loj hlob qeeb thiab muaj zog ntawm lub cev;
hluav taws xob xoo ntawm lub rickets me me thiab osteomalacia ntawm cov leeg pob txha;
hauv kev kuaj sim, muaj kev txo qis ntawm phosphates hauv cov ntshav thiab nce ntxiv hauv cov zis tawm tsam cov calcium uas nyob ib puag ncig.

Phosphate mob ntshav qab zib hom III lossis hypocalcemic rickets yog ib hom kab mob caj ces uas yog tau los ntawm kev muaj lub cev ua kom tsis muaj zog thiab muaj kev vam khom rau cov vitamin d.

Thaum kuaj xyuas tus neeg mob me, nws yog qhov ua tau los txiav txim siab muaj ntau cov kab mob pathological, cov uas:

txob taus, kua muag, poob siab;
cov leeg tsis tshua muaj zog;
mob huam;
qis kev loj hlob, kev loj hlob sai ntawm deformations;
cov kaus hniav tsis zoo;
kev pib taug kev lig;
hypophosphatemia yog txiav txim siab hauv cov ntshav, thiab hyperphosphaturia hauv cov zis;
cov kev hloov pauv zoo ib yam li rickets hauv cov chaw loj hlob, nrog rau cov cim ntawm tag nrho cov leeg txha caj ntawm axial cov ntaub so ntswg tau sau cia radiologically.

Phosphate mob ntshav qab zib hom IV lossis vitamin D3 tsis muaj kab mob yog kab mob kis rau cov menyuam los ntawm niam txiv ntawm kev rov ua haujlwm tsis zoo thiab tshwm sim los ntawm lub hnub nyoog tseem me.Cov tsos mob hauv qab no yog cov yam ntxwv ntawm Mob Ntshav Qab Zib Hom IV:

kev hloov pauv ntawm qhov qis ntawm qhov qis qis, qhov nkhaus ntawm qhov sib txawv ntawm cov pob txha;
cramps
kev plhaw thiab txoj kev ua kom hniav laus;
Xoo hluav taws xob xoo nrhiav cov cim ntawm cov rickets.

Yuav ua li cas los txiav txim siab tus kabmob?

Cov lus pom zoo soj ntsuam rau phosphate ntshav qab zib hauv kev sib piv rau kev txheeb xyuas tus txheej txheem pathological muaj xws li yuav tsum ua tus cwj pwm ntawm ntau cov kev tshawb fawb tsom rau kev txiav txim siab cuam tshuam ntawm phosphate reabsorption hauv hlaus plab hlaus.

Ntawm cov kev kuaj mob tawm yog:

sau los ntawm cov ntaub ntawv tsis zoo los ntawm cov lus ntawm niam txiv ntawm tus menyuam uas cim qhov zoo li ntawm pob txha pob txha pob txha, curvature ntawm ob txhais ceg, stunted kev loj hlob thiab cov zoo li;
kev tshuaj xyuas lub hom phiaj ntawm tus neeg mob me me nrog xaiv cov syndromes ua;
kev tshawb nrhiav keeb kwm rau kev tsis tuaj yeem ntawm X lub cev thiab cov keeb kwm muaj keeb rau kev txhim kho hypophosphatemia;
urinalysis, nyob rau hauv uas loj npaum li cas ntawm phosphate sib txuas yog zais cia;
Kev ntsuam xyuas xoo hluav taws xob ntawm cov pob txha ntawm cov menyuam pob txha nrog cov lus txhais ntawm thaj chaw ntawm osteomalacia, txha txha pob txha, kev cuam tshuam tshwj xeeb hauv thaj chaw loj hlob;
kuaj ntshav kuaj ntsuas kom txo qis ntawm phosphate thaum muaj cov tshuaj calcium ntau.

Cov yam ntxwv khomob

Ob peb lub xyoos dhau los, phosphate mob ntshav qab zib yog qhov nyuaj rau kev kho mob, yog li nws yuav luag tsis tuaj yeem tiv thaiv pob txha pob txha tsis zoo. Niaj hnub no, ua tsaug rau kev kuaj mob niaj hnub no, nws muaj peev xwm txhim kho cov txheeb cais, raws li cov neeg mob hypophosphatemia muaj kev vam meej rau kev kho mob, pom tau tias tus kab mob tau kuaj tau thaum ntxov thiab muaj peev xwm mus rau kev kho tau pom.

Kev kho mob ntshav qab zib phosphate muaj:

lub sij hawm teem tseg ntawm cov qib siab ntawm cov vitamin D (los ntawm 25 txhiab rau 250 txhiab units / hnub);
kev siv cov calcium, phosphorus;
kev nqus ntawm cov vitamins A thiab E, citrate sib xyaw.

Kev qoj ib ce ntawm pob txha pob txha tsis zoo yuav tsum muaj ntau qhov kev kawm ntawm kev zaws, nrog rau kev hnav lub ntsej muag ntev ntev ntawm kev txhawb nqa corset, uas yuav ua rau cov pob txha txhim kho hauv qhov chaw raug.

Kev phais mob ntawm tus kabmob yog raug tshwj tseg qee yam ntawm pob txha caj dab, uas muaj kev hem thawj rau kev ua haujlwm ntawm tus menyuam lub cev.

Nws puas tuaj yeem tiv thaiv tus mob?

Txij li thaum tus kab mob no yog xeeb leej xeeb ntxwv, nws tsuas tuaj yeem tiv thaiv los ntawm kev saib xyuas tsev neeg hluas nyob rau hauv kev sib tham caj ces thaum tab tom npaj tswv yim.

Kev tham sab caj ces

Kev saib xyuas tshwj xeeb rau qhov teeb meem yuav tsum tau them rau cov khub niam txiv uas ib tug ntawm cov neeg koom nrog kev txom nyem los ntawm qhov mob no. Tus kws tshaj lij yuav piav qhia qhov muaj peev xwm ua kom tau tus menyuam muaj lub dag zog zoo thiab ceeb toom txog cov kev pheej hmoo ntawm kev xeeb tub menyuam uas yuav yog tus kabmob ntawm nws niam nws txiv.

Kev tiv thaiv kab mob thib ob muaj nyob rau hauv kev txheeb xyuas lub sijhawm ntev ntawm cov neeg mob muaj peev xwm thiab kev kho mob muaj peev xwm ntawm cov menyuam yaus uas twb muaj thawj cov tsos mob ntawm hypophosphatemia.Nrog rau lo lus teb tsis tseeb rau qhov teeb meem, tus menyuam muaj ntau yam teeb meem thiab tshwm sim, suav nrog:

poob qab ntawm cov phooj ywg hauv kev txhim kho lub cev thiab lub hlwb;
cov tsos pom muaj kev cuam tshuam loj ntawm cov nqaj qaum thiab qis dua;
tag nrho pathologies ntawm hniav txha hniav laus;
distortion ntawm pob txha mos, nqaim ntawm nws txoj kab uas hla;
ua txhaum ntawm kev txhim kho ntawm cov pob ntseg ntawm cov pob ntseg hauv pob ntseg hauv nruab nrab thiab pob ntseg;
urolithiasis thiab raum tsis ua haujlwm los ntawm nws.