Kab mob ntshav qab zib mob ntshav qab zib (vitamin D-resistant, hypophosphatemic, rickets)

Txhua iLive cov ntsiab lus raug tshuaj xyuas los ntawm cov kws kho mob kom ntseeg tau qhov ua tau zoo tshaj plaws thiab xwm yeem nrog cov lus tseeb.

Peb muaj cov kev cai nruj rau xaiv qhov chaw ntawm cov ntaub ntawv thiab peb tsuas yog xa mus rau cov chaw muaj suab npe, cov chaw tshawb fawb ntawm kev kawm txuj ci thiab, yog tias ua tau, pov thawj kev tshawb fawb kho mob. Thov nco ntsoov tias cov lej hauv kev nkhaus (,, thiab lwm yam) yog cov kev sib txuas sib txuas rau cov kev tshawb fawb no.

Yog tias koj xav tias txhua yam ntawm peb cov ntaub ntawv tsis raug, tsis muaj dhau los lossis muaj lus nug tsis zoo, xaiv nws thiab nias Ctrl + Enter.

Hereditary phosphate mob ntshav qab zib yog ib pawg kab mob muaj keeb ntawm cov kab mob tsis haum nrog lub cev tsis muaj zog ntawm phosphates thiab vitamin D. Hypophosphatemic rickets yog ib hom kab mob los ntawm hypophosphatemia, tsis hnov qab cov calcium thiab rickets lossis osteomalacia, tsis hnov mob vitamin D Cov tsos mob muaj xws li mob pob txha, pob txha lov thiab mob kev loj hlob. Kev kuaj mob yog txiav txim siab los ntawm kev txiav txim siab ntshiab phosphate, alkaline phosphatase thiab 1,25-dihydroxyvitamin D3 qib. Kev kho mob suav nrog kev noj phosphates thiab calcitriol.

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Ua thiab pathogenesis ntawm phosphate mob ntshav qab zib

Tsev neeg hypophosphatemic rickets tau txais koob hmoov los ntawm X-qhov tseem ceeb hom. Cov kab mob kis tau los ntawm cov kab mob hypophosphatemic rickets muaj qee zaum cuam tshuam nrog benign mesenchymal hlav (oncogenic rickets).

Lub hauv paus ntawm tus kabmob yog qhov txo qis hauv kev rov kho dua ntawm phosphates nyob rau ntawm cov menyuam thaum muaj menyuam, uas ua rau cov ntshav hypophosphatemia. Qhov kev tsis zoo no nthuav dav vim yog qhov hloov ncig thiab cuam tshuam nrog thawj qhov kev tsis txaus siab hauv osteoblast muaj nuj nqi. Kuj tseem muaj qhov txo qis hauv plab hnyuv nqus ntawm calcium thiab phosphate. Cov pob txha tsis zoo muaj ntau dua vim muaj phosphate tsawg thiab ua haujlwm ntawm cov osteoblasts dua li vim tias cov calcium tsis tshua zoo thiab muaj kev nce qib ntawm parathyroid hormone hauv calcium-tsis muaj rickets. Txij li theem ntawm 1,25-dihydroxycholecalciferol (1,25-dihydroxyvitamin D) zoo li qub lossis qis dua me ntsis, ib qho kev tsis txaus ntseeg ntawm kev tsim cov ntaub ntawv nquag ntawm cov vitamin D tuaj yeem xav tias, ib txwm hypophosphatemia yuav tsum ua rau nce ntawm theem ntawm 1,25-dihydroxyvitamin D.

Hypophosphatemic rickets (mob ntshav qab zib phosphate) yuav tshwm sim vim txo phosphate reabsorption hauv cov tub ntxhais hluas. Qhov no cov tubular ua hauj lwm tsis zoo yog pom nyob rau hauv kev rho tawm, cov hom qub txeeg qub teg yog qhov tseem ceeb, txuas nrog X chromosome. Ntxiv rau, phosphate mob ntshav qab zib yog ib qho ntawm cov ua haujlwm ntawm Fanconi syndrome.

Paraneoplastic phosphate mob ntshav qab zib yog tshwm sim los ntawm kev tsim cov kab mob parathyroid hormone zoo li los ntawm qog cov hlav.

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Cov tsos mob ntawm ntshav qab zib Phosphate

Hypophosphatemic rickets yog qhov ua rau nws tus kheej muaj mob ntau yam, los ntawm kev tiv thaiv asymptomatic hypophosphatemia qeeb mus rau kev txhim kho lub cev thiab kev loj hlob qis mus txog lub tsev kho mob ntawm mob rickets lossis pob txha txha hnyav. Kev tshwm sim hauv cov menyuam yaus feem ntau txawv tom qab lawv pib taug kev, lawv txhim kho O-zoo li tus ceg ntawm qhov ceg thiab lwm yam pob txha pob txha, pob txha, pob txha, mob pob txha thiab qis qis. Kev loj hlob Bony ntawm cov chaw txuas nqaij ntshiv tuaj yeem txwv kev txav chaw. Nrog hypophosphatemic rickets, rickets ntawm tus txha nqaj lossis txha caj qaum, qhov tsis xws luag ntawm txha hniav laus thiab spasmophilia, uas txhim kho nrog cov vitamin D-tsis muaj rickets, tsis tshua pom.

Cov neeg mob yuav tsum txiav txim siab txog qib calcium, phosphates, alkaline phosphatase thiab 1,25-dihydroxyvitamin D thiab HPT hauv ntshav dej, nrog rau cov kuab phosphate tsis txaus. Nrog hypophosphatemic rickets, theem ntawm phosphates hauv cov ntshav cov ntshav tau txo qis, tab sis lawv qhov kev tso tawm hauv cov zis yog siab. Ntshav dej calcium thiab qib PTH yog qhov qub, thiab cov alkaline phosphatase feem ntau yog nce siab. Nrog rau cov calcium uas tsis muaj cov rickets, hypocalcemia yog sau tseg, tsis muaj ntshav qab zib hypophosphatemia los sis nws yog mob me, kev tshem tawm phosphates hauv cov zis tsis nce ntxiv.

Hypophosphatemia tau kuaj pom hauv cov menyuam mos. Hauv 1-2 lub xyoo ntawm lub neej, cov tsos mob ntawm tus kab mob tshwm sim: kev loj hlob qeeb, qhov tsis zoo ntawm qhov qis qis. Cov leeg tsis muaj zog yog mob me los yog qhaj dua. Kev tsis pom zoo luv luv txhais ceg yog tus yam ntxwv. Hauv cov neeg laus, osteomalacia maj mam loj hlob tuaj.

Txog rau hnub no, 4 yam kev pub muaj keeb ntawm kev tsis txaus siab hauv cov kab mob hypophosphatemic rickets tau piav qhia.

Hom I - X-txuas ntshav mob hypophosphatemia - ntshav qab zib D-tiv thaiv kab mob ntshav qab zib (hypophosphatemic tubulopathy, mob ntshav qab zib hypophosphatemia, kab mob ntshav qab zib muaj ntshav qab zib, ntshav qab zib phosphate, mob ntshav qab zib phosphate ntshav qab zib, mob rwj tub raum, Albert Blairt's mob) vim ib qho kev txo qis hauv phosphate reabsorption nyob rau hauv qhov ze ntawm lub raum hlaus thiab nthuav tawm los ntawm hyperphosphaturia, hypophosphatemia thiab kev txhim kho ntawm rickets-xws li kev hloov pauv uas tiv taus cov koob tshuaj zoo li qub ntawm vitamin D.

Nws tau xav tias nrog X-txuas rau hypophosphatemic rickets, txoj cai ntawm kev ua si ntawm 1-a-hydroxylase nrog phosphate yog qhov tsis taus, uas qhia qhov tsis txaus ntseeg ntawm cov synthesis ntawm vitamin D metabolite 1.25 (OH) 2D3. Qhov siab ntawm l, 25 (OH) 2D3 hauv cov neeg mob yog qhov tsis txaus rau kev txiav txim siab ntawm hypophosphatemia.

Tus kab mob kis tau nws tus kheej mus txog 2 xyoos ntawm lub neej. Tus yam ntxwv tshwj xeeb tshaj plaws:

stunted kev loj hlob, zaum-sawv cev, cov leeg nqaij ua kom muaj zog, tsis muaj qhov hypoplasia ntawm qhov txha hniav laus ntawm cov hniav mus tas li, tab sis muaj cov ntawv txuas ntxiv ntawm cov chaw mos, alopecia,
hypophosphatemia thiab hyperphosphaturia nrog cov ntshav calcium calcium thiab nce ntxiv phosphatase alkaline,
kev txiav ceg tawv hnyav (nrog rau kev pib taug kev),
Xoo hluav taws xob rickets-zoo li kev hloov ntawm cov pob txha - dav diaphysis nrog lub thooj ntawm cov cortical txheej, cov qauv ntxhib ntawm cov leeg pob txha, txha caj qaum, lub cev tsis ua haujlwm ntawm qhov qis qis, ncua tsim cov pob txha, tag nrho cov ntsiab lus calcium hauv cov pob txha tau nce.

Kev rov ua kom tau phosphates hauv cov raum tsawg dua li 20-30% lossis tsawg dua, qhov kev tso tawm ntawm phosphorus hauv cov zis yuav nce mus rau 5 g / hnub, qhov haujlwm ntawm alkaline phosphatase tau nce (2-4 zaug piv nrog cov qauv). Hyperaminoaciduria thiab glucosuria yog uncharacteristic. Kev tshem tawm calcium tsis tau hloov.

Muaj 4 qhov chaw kho mob thiab biochemical hloov pauv ntawm phosphate mob ntshav qab zib raws li cov tshuaj tiv thaiv kev qhia txog ntawm cov vitamin D. Hauv thawj qhov kev hloov pauv, kev nce hauv cov ntsiab lus ntawm inorganic phosphates hauv cov ntshav thaum kho yog cuam tshuam nrog kev nce ntxiv ntawm lawv cov reabsorption hauv cov hlaus rov qab, nyob hauv ob, phosphate reabsorption nyob rau hauv ob lub raum thiab cov hnyuv tau zoo tuaj, nrog rau cov thib peb - nce reabsorption tshwm sim tsuas yog nyob rau hauv txoj hnyuv, thiab plaub, kev nkag siab zoo rau cov vitamin D tau nce ntau, yog li txawm tias muaj cov tshuaj vitamin me me ua rau muaj kev qaug dab peg.

Hom II - ib daim ntawv ntawm hypophosphatemic rickets - yog ib qho autosomal tseem ceeb, tsis txuas nrog X kab mob xos. Tus kab mob yog tus cwj pwm los ntawm:

qhov pib ntawm tus kab mob thaum hnub nyoog 1-2 xyoos,
curvature ntawm ob txhais ceg nrog pib ntawm kev taug kev, tab sis tsis muaj hloov qhov siab, muaj zog lub cev, lub cev pob txha deformations,
hypophosphatemia thiab hyperphosphaturia nrog cov calcium uas ib txwm muaj thiab cov txiaj ntsig kev ua si ntawm alkaline phosphatase nce ntxiv,
Xoo hluav taws xob: cov tsos mob me me ntawm rickets, tab sis nrog osteomalacia loj heev.

Tsis muaj kev hloov pauv ntawm cov khoom siv roj ntsha, CBS, qhov kev nqus ntawm parathyroid hormone, muaj pes tsawg leeg cov ntshav amino acids, qib ntawm creatinine, thiab residual nitrogen hauv cov ntshav dej. Hloov zis yog uncharacteristic.

Hom III - autosomal recessive dependant rau vitamin D (hypocalcemic rickets, osteomalacia, hypophosphatemic vitamin D-depend rickets nrog aminoaciduria). Qhov ua rau muaj tus kabmob yog qhov ua txhaum ntawm kev tsim ntawm 1.25 (OH) 2D3 hauv lub raum, uas ua rau lub cev tsis muaj peev xwm nqus tau cov calcium nyob rau hauv txoj hnyuv thiab muaj kev cuam tshuam ncaj qha ntawm cov vitamin D rau cov pob txha tshwj xeeb receptors, hypocalcemia, hyperaminoaciduria, theem nrab hyperparathyroidism, tsis hnov qab phosphorus reabsorption thiab hypophosphatemia.

Qhov pib ntawm tus kab mob yog hais txog lub hnub nyoog ntawm 6 lub hlis. txog li 2 xyoos Cov cim feem ntau:

excitability, hypotension, convulsions,
hypocalcemia, hypophosphatemia, hyperphosphaturia thiab nce kev ua si ntawm alkaline phosphatase hauv cov ntshav. Nws yog nce los ntawm ntshav parathyroid hormone concentrates, thiab generalized aminoaciduria thiab ib qho kev tsis sib haum xeeb kuj tau pom, qee zaum kuj tso zis acidification tsis xws luag,
kev pib qeeb ntawm kev taug kev, tsis meej, kev loj hlob sai deformities, leeg tsis muaj zog, txha hniav laus ntshav dawb, txha hniav tsis taus,
Xoo hluav taws xob qhia rickets loj heev nyob rau thaj tsam ntawm kev loj hlob ntawm cov pob txha ntev tubular, ua rau nyias ntawm cov cortical txheej, ib qho nyiam pob txha. Tsis muaj kev hloov pauv hauv CBS, cov ntsiab lus ntawm cov pa nitrogen, tab sis cov ntsiab lus ntawm l, 25 (OH) 2D3 hauv cov ntshav tau txo qis.

Hom IV - vitamin D3 tsis muaj txaus - yog tau los ntawm ib qho kev pheej hmoo tsis haum lossis tshwm sim ib zaug, cov ntxhais feem ntau muaj mob. Qhov pib ntawm tus kab mob tau sau tseg nyob rau hauv thaum yau, nws yog tus cwj pwm los ntawm:

curvature ntawm ob txhais ceg, deformation ntawm lub pob txha, cramps,
nquag alopecia thiab qee zaum hniav anomaly,
Xoo hluav taws xob qhia rickets ntawm qhov sib txawv.

Kev kuaj mob ntshav qab zib phosphate

Ib qho ntawm cov cim uas xav tias phosphate mob ntshav qab zib yog qhov tsis muaj txiaj ntsig ntawm cov qauv tshuaj ntawm cov vitamin D (2000-5000 IU / hnub) hauv cov menyuam yaus raug mob los ntawm rickets. Txawm li cas los xij, lo lus "Vitamin D-resistant rickets", yav dhau los siv los hais txog cov ntshav qab zib phosphate, tsis tau muaj tseeb.

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Kev kuaj mob sib txawv ntawm phosphate mob ntshav qab zib

Nws yog qhov tsim nyog kom sib txawv raws caj ces phosphate mob ntshav qab zib nrog vitamin D-tsis txaus rickets, uas qiv nws tus kheej kom zoo rau kev kho mob, de Toni-Debre-Fanconi syndrome, osteopathy hauv kev mob raum tsis ua haujlwm.

Thaum cov tsos mob phosphate mob ntshav qab zib tshwm sim thawj zaug hauv cov neeg laus, oncogenic hypophosphatemic osteomalacia yuav tsum xav. Qhov txawv txav ntawm paraneoplastic syndrome tau pom nyob rau hauv ntau cov qog, nrog rau daim tawv nqaij (ntau yam dysplastic nevi).

Mob ntshav qab zib yog dab tsi

Ntshav qab zib phosphate yog lub npe rau cov kab mob caj ces, cov yam ntxwv ntawm uas:

tsis muaj phosphorus nyob rau hauv lub cev,
kev txhim kho ntawm pob txha mob,
cov tsos ntawm rickets-zoo li cov tsos mob.

Ntshav qab zib phosphate yog ib qho kev mob uas tsis tshua pom muaj ntau tshaj plaws nrog 1: 20,000 lub caij nyoog ntawm kev tsim nws.

Txog yim hom kab mob pathology tau txawv raws qhov kev hloov pauv ntawm ib lub noob. Qhov tshwj xeeb tshaj yog cov X-txuas rau cov kab mob hypophosphatemic rickets vim tawg ntawm REX noob.

Raws li kev tshawb fawb txog kev kho mob niaj hnub no, tib neeg cov keeb kwm muaj xws li ntau tshaj 28 txhiab noob.

Ntshav qab zib phosphate yog ib tus kab mob ua tsis taus mis; tsis muaj ib qho tsim muaj cov kab mob pathology.

Vim li cas cov ntsiab lus phosphorus hauv lub cev txo qis thaum mob?

Kev txiav "caj ces" ua rau kom muaj kev cuam tshuam ntawm phosphorus reabsorption hauv cov hlaus plab. Ntshav nkag mus rau hauv lub tubule ntawm lub raum thiab raug tso ua txheej txheem. Raws li qhov tshwm sim, cov zis thawj thiab theem nrab tau tsim los. Thawj cov zis muaj cov tshuaj uas tsim nyog rau lub cev: piam thaj, protein, sodium, phosphorus, calcium. Cov txiaj ntsig zoo los ntawm cov hlaus hlab tau rov qab nkag mus rau hauv cov ntshav. Xws li tus txheej txheem hu ua "reabsorption".

Hauv cov tubules ntawm lub raum, rov tso tshuaj kom zoo rau lub cev tshwm sim

Cov tshuaj tsis tsim nyog (co toxins, toxins) raug tshem tawm los ntawm lub raum nrog zis theem nrab: tso zis ntawm qhov tso zis, tso zis thiab tawm lub cev los ntawm qhov tso zis. Hauv cov kab mob ntshav qab zib, phosphorus tsis yog nqus rov qab rau hauv cov ntshav los ntawm ob lub raum, tab sis nws dhau ntawm cov mob txeeb zig. Ib qho tsis txaus ntawm cov tshuaj no yog tsim hauv lub cev, uas ua rau kev rhuav tshem cov pob txha nqaij, mob leeg nqaij, thiab qhov tshwm sim ntawm lub raum, lub paj hlwb, thiab cov leeg ntshav.

Phosphorus yog lub luag haujlwm rau lub xeev ntawm tib neeg cov pob txha, thiab tseem cuam tshuam rau kev ua haujlwm ntawm lub siab thiab lub raum.

Lwm yam teeb meem ntawm cov metabolism hauv phosphate cov ntshav qab zib

Thiab tseem nrog tus kabmob, lwm cov txheej txheem tsis zoo hauv lub cev tuaj yeem pom:

malfunction ntawm parathyroid hormone (parathyroid hormone), uas tswj cov calcium-phosphorus metabolism. Raws li qhov tshwm sim, calcium reabsorption kuj txo.
kev ua txhaum ntawm kev tsim nyob rau hauv lub cev ntawm cov vitamin D, uas yog sib xyaw nyob rau hauv daim tawv nqaij nyob rau hauv tus ntawm ultraviolet hluav taws xob, thiab tseem nkag mus rau hauv lub cev nrog cov zaub mov. Txawm li cas los xij, cov vitamin D tsuas tuaj yeem nquag ua haujlwm tom qab cov txheej txheem uas tshwm sim hauv cov hlab hlaus. Nrog phosphate ntshav qab zib, cov txheej txheem no tau cuam tshuam, yog li qhov tsis muaj vitamin D tshwm sim hauv lub cev.

Cov cim ntawm pathology

Tus kab mob kis tau nws tus kheej hauv cov menyuam yaus uas muaj hnub nyoog 1-2 xyoos thiab nrog cov tsos mob hauv qab no:

hauv siab kab noj hniav
protrusion ntawm frontal tubercles,
Lub log zoo li tus log ntawm tus ceg,

Nrog rau ntshav qab zib phosphate, tus menyuam cov ceg txhais lub ntsej muag tus tsiaj ntawv “o”

Yog hais tias kev kho mob ntawm tus kab mob tsis tau pib thaum yau, ces ntau tshaj tawm cov tsos mob phosphate mob ntshav qab zib raug pom hauv cov neeg laus:

mob hnyav hauv cov pob qij txha hauv qab ntawm qhov qis qis, caj npab, nraub qaum,
nruj ntawm taw. Nws nyuaj rau tus txiv neej taug kev, zaum, khoov duav,
nquag pob txha thiab txhais caj npab uas tshwm sim los ntawm pob txha pob txha,
pos hniav los ntshav
ib nrab lossis tag nrho cov hniav,
hnov lus
kiv taub hau thiab tsaus muag, tshwm sim tawm tsam keeb kwm ntawm atrophy ntawm cov hlab ntsha ntawm nqaj qaum.

Kev siv cov tshuaj

Hauv ntshav qab zib phosphate, cov tshuaj hauv qab no tau sau tseg:

phosphorus npaj. Kev txais tos yog ua tiav 4-6 zaug ib hnub. Qhov ntau npaum li cas ntawm cov tshuaj nyob ntawm lub hnub nyoog ntawm tus neeg mob thiab raug xaiv los ntawm tus kws kho mob tuaj koom. Hauv kev kho mob rau menyuam yaus, tshuaj tuaj yeem tov nrog kua mis, txiv hmab txiv ntoo thiab zaub qab zib, kua txiv,
Alfacacidol yog ib qho tshuaj noj ntawm cov vitamin D. Cov tshuaj tsis tsuas yog ua kom phosphorus-calcium metabolism, tab sis kuj pab txo qhov mob, txhim kho kev sib koom tes ntawm kev txav. Kev kho mob nyob ntawm qib PTH,

Alfacacidol - ib qho analogue ntawm vitamin D

Kev phais mob

Kev phais mob rau ntshav qab zib phosphate yog qhia nyob rau hauv cov xwm txheej zoo li no:

nrog ntev ntev ntawm ob txhais ceg. Qhov tshwm sim no tshwm sim thaum ib sab ceg qis dua tau hla qhov deformation ntau dua li lwm qhov,
nrog kev hloov pauv tseem ceeb hauv cov qauv ntawm lub hauv caug, pob luj taws lossis pob qij txha. Cov teeb meem zoo li no feem ntau tsim nyob rau hauv neeg laus nrog phosphate mob ntshav qab zib siab heev. Kev sib koom ua ke tsis pub ua rau ib tus neeg hloov mus rau txhua zaus thiab tuaj yeem ua rau muaj kev tsis taus.

Hom kev phais mob:

ib ntus epiphysiodesis. Nws yog nqa nrog ntau qhov ntev ntawm cov ceg rau cov menyuam yaus hnub nyoog qis dua 15 xyoos. Thaum muaj hnub nyoog no, ib tus neeg nyob hauv theem loj hlob, yog li nrog kev pabcuam ntawm cov cuab yeej siv kho mob, koj tuaj yeem kho qhov nce hauv qhov ntev ntawm nqua. Ntawm txhais ceg, uas yuav tsum tau ua kom luv, 8-phaj daim phiaj yog ntsia. Cov cuab yeej siv tau tsau rau ntawm cov nqaj saum toj saud thiab qis dua thaj chaw loj hlob. Cov aav ntawm cov ceg ntawm ob txhais ceg yog nyob rau sab saud sab saud thiab hauv qab ntsag, zoo li hauv ceg qis,
kho osteotomy. Thaum phais, cov pob txha lov. Tom ntej no, qhov sib koom tes yog muab cov qauv tseeb, uas tau tsau nrog cov paib thiab screws,
sib koom tes hloov. Thaum lub sijhawm ua haujlwm, kev phais me me yog tsim nyob rau thaj tsam ntawm qhov sib koom ua ke. Txoj kev sib koom tes yog hloov los ntawm ib qho chaw yug tsiaj, lub qhov txhab yog sutured.

Yog hais tias tus neeg mob nrog phosphate mob ntshav qab zib lub hauv caug sib koom tes tsis tiav, tom qab ntawd nws hloov los ntawm ib qho prosthesis

Yuav ua li cas kom tsis txhob muaj kab mob qhov ncauj

Cov neeg mob uas muaj ntshav qab zib phosphate muaj qhov pheej hmoo ntawm kev tsim kab mob hauv qhov ncauj. Txhawm rau kom tsis txhob ua rau cov hniav thiab pos hniav, yuav tsum ua raws li txoj cai hauv qab no:

txhuam koj cov hniav 2-3 zaug ib hnub twg. Hauv qhov no, siv txhuam nrog cov mos muag,
tom qab txhua pluas noj, yaug lub qhov ncauj kab noj hniav nrog cov tshuaj tua kab mob,
zam kev siv cov khoom qab zib (khoom qab zib, qab zib, khoom qab zib, marmalade),
mus ntsib kws kho hniav tsawg kawg 1 zaug nyob rau ib hlis,
tiv thaiv lub ntsej muag ntawm cov hniav los ntawm txheej cov txha hniav laus nrog tshwj xeeb gel txhuam. Cov kev tiv thaiv zoo li no yog nqa tawm hauv txhua lub chaw kho hniav.

Phosphate mob ntshav qab zib hauv cov menyuam yaus: ua rau, cov tsos mob, cov qauv kev kho mob

Lo lus "phosphate diabetes" koom ua ke nrog cov kab mob rickets-zoo li cov kab mob, uas yog los ntawm kev ua txhaum ntawm kev thauj khoom ntawm phosphates nyob rau ntawm qhov tsis sib luag ntawm cov leeg ntawm nephron, lawv nqus hauv cov hnyuv thiab, vim li ntawd, cov tsis muaj phosphoric acid ntsev rau hauv lub cev. Nws tseem muaj lwm lub npe rau lub pathology no thiab. Vim tias muaj cov tshuaj phosphorus nyob rau hauv cov ntshav tsawg thiab cov duab ua pov thawj ntawm cov rickets, nws tseem hu ua hypophosphatemic rickets, thiab coj mus rau hauv tus lej qis tsis zoo rau kev kho mob nrog vitamin D, nws yog hu ua vitamin D-resistant rickets.

Txoj kev kho kab mob no yog ib qho teeb meem chaw kuaj mob hauv chaw kho pob qij txha orthopedics thiab nephrology. Nws qhov tseeb yog vim qhov xav tau kev kho mob mus ntev, qee zaum kev kho lub neej ntev, qhov tsis muaj peev xwm ntawm txoj kev kho mob tam sim no kom tshem tawm cov kev soj ntsuam ntawm tus kab mob thiab pob txha deformities.

Thawj cov lus piav qhia ntawm tus kab mob tau tshwm sim nyob rau xyoo 20. Tus neeg mob tau sau npe nrog kev sib txawv ntawm cov neeg muaj kabmob hypophosphatemic rickets thiab lub luag haujlwm ntawm kev muaj keeb hauv nws qhov tshwm sim tau ua pov thawj. Tom qab ntawd, lwm hom phosphate mob ntshav qab zib kuj tseem tau txheeb xyuas, muaj ob qho tib si sib xws thiab lawv cov laj thawj, hom cuab yeej cuab tam thiab hom kawm. Hauv qab no peb nyob ntawm qhov tseem ceeb.

Cov chav kho mob ntawm phosphate mob ntshav qab zib yog polymorphic. Tus kab mob feem ntau ua rau nws qhov tshwm sim thaum yau, tab sis tuaj yeem tshwm sim nws tus kheej tom qab - ntawm 7-9 xyoo. Ntxiv mus, qhov mob ntawm cov tsos mob pathological kuj tseem yuav sib txawv. Qee qhov xwm txheej, tus kab mob no muaj kev kawm asymptomatic thiab tshwm sim los ntawm kev hloov me me hauv phosphorus-calcium metabolism. Txawm li cas los xij, feem ntau cov kev mob siab hypophosphatemic rickets muaj cov duab hais ncaj qha:

pauj kev loj hlob ntawm lub cev thiab kev loj hlob,
pob txha pob txha mos (txawv txav ntawm sab hauv qis, "rickets" ntawm tus tav, ua rau pob txha pob txha txawv txav ntawm sab caj npab, deformation ntawm pob txha taub hau),
hloov pauv hauv tus me nyuam lub plab (zoo li os)
ua txhaum ntawm tsim ntawm cov hniav,
pathological pob txha lov,
mob pob txha, thiab lwm yam.

Kev mob ntshav hauv lub cev, tus yam ntxwv ntawm qhov tseeb rickets, feem ntau yog qhaj ntawv phosphate mob ntshav qab zib.

Cov kev txawj ntse hauv tus kab mob no tsis raug kev txom nyem.

Kev kuaj mob ntshav qab zib phosphate nyob rau hauv cov menyuam yaus yog ua raws cov duab hauv chaw kuaj mob, cov ntaub ntawv los ntawm kev kuaj lub cev thiab kev kuaj mob. Kev kuaj mob yog lees paub cov txiaj ntsig ntawm kev sim thiab cuab yeej cuab tam:

cov kev hloov hauv cov ntshav ntsuas (hypophosphatemia, nce alkaline phosphatase, ib txwm los sis nce qib ntawm parathyroid hormone thiab calcitonin) thiab tso zis (hyperphosphaturia, txo phosphate reabsorption nyob rau hauv lub raum tubules, nce calcium excretion tsuas yog nrog hypophosphatemic rickets nrog calcuria),
Cov ntaub ntawv xoo hluav taws xob (cov cim ntawm cov pob txha kho pob txha, pob txha tsis zoo, hloov pauv hauv cov pob txha pob txha, txha pob txha).

Qee zaum hauv qhov pib ntawm tus kab mob, cov neeg mob no tau kuaj pom tias yog rickets thiab kev noj tshuaj yog muaj cov vitamin D, cov kev kho mob no tsis muab cov txiaj ntsig thiab tsis muaj laj thawj xav tias muaj phosphate ntshav qab zib hauv menyuam yaus. Yog tias tsim nyog, nyob rau hauv cov xwm txheej zoo li no, kev tshawb fawb txog molecular muaj cuab kav tau muab coj los txheeb xyuas qhov teeb meem caj ces.

Kev sib xyaw ntawm hypophosphatemia thiab rickets ntawm nqua kuj tseem pom nyob rau hauv qee lwm txoj kev mob ntawm kev mob uas kev kuaj mob txawv yuav tsum tau nqa tawm:

mob raum (mob raum tubular acidosis, mob raum ntev) thiab mob siab (mob ntsws),
endocrine pathology (kev ua haujlwm siab ntawm cov qog ntshav parathyroid),
malabsorption nyob rau hauv ulcerative colitis, celiac enteropathy,
alimentary (khoom noj) tsis txaus vitamin D thiab phosphorus,
noj qee yam tshuaj.

Kev kho mob zoo kawg ntawm kev mob hypophosphatemic rickets yuav tsum pib thaum ntxov ntawm tus kab mob. Ua ntej tshaj plaws, nws yog txhawm rau kho qhov teeb meem ntawm cov roj ntsha tsis sib haum thiab tiv thaiv cov pob txha deformities. Thaum nws raug tshuaj, kev ua haujlwm ntawm cov txheej txheem thiab cov tib neeg kev mob siab rau ntawm cov tshuaj tau raug coj los siv.

Lub hauv paus ntawm cov kev kho mob ntev yog kev kho mob mus ntev nrog cov koob tshuaj vitamin d ntau dhau los:

nrog cov rickets hauv cov nqaij pob txha,
poob phosphorus tebchaw nyob rau hauv cov zis,
nce qib ntawm alkaline phosphatase nyob rau hauv cov ntshav,
nyob rau theem ntawm kev npaj rau kev phais txhawm rau txhim kho pob txha pob txha deformities.

Thaum pib txhaj tshuaj vitamin D yog 10,000-20000 IU ib hnub. Lawv txoj kev nce ntxiv yog ua nyob rau hauv kev tswj hwm ntawm qhov taw qhia ntawm phosphorus-calcium metabolism hauv cov ntshav. Cov koob tshuaj txhua hnub ntau yuav ua tau siab thiab qee zaum ncav cuag 250,000-300,000 IU.

Nyob rau hauv lub xub ntiag ntawm ib tug neeg intolerance mus rau vitamin D, raws li tau zoo heev hypercalciuria, lub sij hawm teem ntawm kev kho mob yog suav tias yog tsis tsim nyog.

Ntxiv rau cov vitamin D, cov neeg mob zoo li no tau pom zoo kom ua:

phosphorus thiab calcium npaj,
citrate sib tov (hauv 6 lub hlis txhawm rau txhim kho kev nqus ntawm cov ntsiab lus no),
kev loj hlob lawm.

Thaum lub sijhawm ua haujlwm siab ntawm cov txheej txheem, cov neeg mob tau qhia rau lub txaj so, tom qab ua tiav kev tshem tawm - kev kho mob zaws, kev ua si ntawm lub cev thiab kev kho mob spa.

Cov qauv rau kev ua haujlwm ntawm kev txuag kho yog:

kev nyob zoo,
kev ua kom muaj kev nrawm nrawm,
normalization ntawm phosphorus metabolism hauv lub cev,
zoo kev thaij duab hluav taws xob (rov kho lub cev pob txha qub).

Nyob rau hauv muaj cov lus tshaj tawm los ntawm pob txha deformities tiv thaiv keeb kwm ntawm kev pheej hmoo thiab kev kho mob hauv chaw kuaj ntshav, lawv txoj kev kho kom zoo yog ua. Cov hau kev hauv qab no tuaj yeem siv rau qhov no:

osteotomy (dissection) ntawm cov leeg pob txha ntev nrog kev kho ntawm lub axis ntawm nqua,
limb kev immobilization los ntawm Ilizarov kev cuam tshuam thiab ntsuas lub tshuab.

Cov kev ua haujlwm zoo li no yuav tsum tau nqa tawm tsuas yog tom qab lub sijhawm muaj kev txuag ntau thiab kev soj ntsuam kom zoo.

Yog xav tias tus mob ntshav qab zib phosphate, nws yog qhov yuav tsum tau sab laj nrog kws kho mob menyuam yaus, tom qab kuaj thawj zaug, yuav xa tus menyuam mus sab laj nrog tus kws kho mob endocrinologist, orthopedist, thiab kws kho mob hlwb. Txoj kev kho yuav koom nrog tshuaj pleev ib ce, kws kho mob lub cev, kws paub hauv ce pab kho mob thiab kev noj zaub mov zoo noj. Yog tias tsim nyog, kev kho yog ua los ntawm tus kws phais neeg orthopedic.

Nrog kev kuaj mob kom sai thiab kev kho mob ntxov, kev soj ntsuam rau kev mob ntshav qab zib yog kev txaus siab. Txawm li cas los xij, qee tus neeg mob yuav tsum muaj kev kho lub neej nrog vitamin D. Qhov kev kuaj mob lig txiav txim siab tias yuav muaj kev phom sij ntau dua, vim tias cov pob txha tsis ua haujlwm pab txo cov neeg muaj lub neej zoo.

Ntshav qab zib phosphate hauv cov menyuam yaus: yuav ua li cas tus kab mob nthuav qhia nws tus kheej, kho thiab ua daim duab

Qhov tsim ntawm phosphate mob ntshav qab zib tsis cuam tshuam nrog kev puas tsuaj rau tus txiav thiab nce ntshav qabzib.

Tus kab mob no, uas zoo li mob ntshav qab zib, muaj qhov mob ua ntej. Nws cov qauv qhia tau yog vim qhov tseeb tias phosphorus tsis yog nqus tau rov qab rau hauv cov ntshav hauv cov hlab ntsha.

Hauv cov ntshav, nws cov concentration poob qis, cov qauv ntawm cov pob txha pob txha yog ua txhaum, uas ua rau muaj qhov tsis xws luag hauv kev tsim cov pob txha thiab anomalies hauv cov qauv ntawm lub cev.

Ua raws li cov laj thawj ntawm phosphate, mob ntshav qab zib tuaj yeem raug tshem tawm cov roj ntsha thiab kis los ntawm cov niam txiv muaj mob rau menyuam yaus lossis yog qhov tshwm sim ntawm cov qog benign (oncogenic rickets).

Hypophosphatemic rickets tshwm sim nrog txo kev nqus ntawm phosphorus los ntawm thawj cov zis, nrog rau qhov tsis txaus ntawm cov calcium thiab phosphates los ntawm txoj hnyuv, qhov txo qis hauv cov synthesis ntawm vitamin D thiab nws ua haujlwm hauv lub siab. Qhov ua kom tsawg dua hauv kev ua haujlwm ntawm cov hlwb uas tsim cov pob txha nqaij (osteoblasts) ua rau lub pob txha muaj qhov tsis nyiam.

Me nyuam yaus muaj mob nyob ntawm qhov muaj ib yam pathology hauv lawv niam thiab txiv. Tsis muaj cov hau kev tshwj xeeb los tiv thaiv tus kabmob. Yog tias leej txiv muaj mob, nws hloov pauv phosphate mob ntshav qab zib rau nws tus ntxhais, thiab cov tub thiab cov ntxhais nrog qhov tsis zoo ntawm kev loj hlob tuaj yeem yug los ntawm leej niam mob. Lub noob ua lub luag haujlwm rau osteoblasts thiab cov ntsiab lus phosphorus hauv cov ntshav tau txuas rau X chromosome.

Feem ntau cov mob nyhav, cov tub hluas raug kev txom nyem los ntawm hypickosphatemic rickets. Hauv cov laus laus, tus kab mob no yuav cuam tshuam nrog cov qog ntshav hauv cov pob txha lossis cov nqaij mos.

Nrog phosphate ntshav qab zib, cov kev tsis zoo xws li tsim:

Cov pob txha loj hlob
Cov pob txha mos
Kev puas tsuaj ntawm pob taws thiab pob qij txha
Kev vam meej sai zuj zus ntawm qhov nqaj deformity.

Hypophosphatemic rickets tuaj yeem pib thaum hnub nyoog me me, feem ntau los ntawm lub sijhawm tus menyuam pib taug kev nws tus kheej. Ua ntej qhov no, qhov xwm txheej dav dav tuaj yeem nyob twj ywm li qub thiab tsis ua rau kev poob siab nyob hauv cov kws kho mob.

Thawj qhov mob tshwm sim yog qhov me nyuam lub cev ntaj, ces mob hauv pob txha thaum taug kev. Cov menyuam yaus yuav tsis kam mus los ntawm tsis muaj kev pab. Nyob rau hauv lub neej yav tom ntej, ob txhais ceg yog khoov thiab tus qauv ntawm lub hauv caug thiab pob qij txha tau sib txuam, thiab cov pob txha nyob hauv thaj chaw ntawm pob dab teg.

Cov kev qhia pom no yog los ntawm kev ua txhaum ntawm kev ncaj ncees ntawm tus txha hniav laus thiab kev txhim kho ntawm ntau caries, curvature ntawm tus nqaj qaum thiab pob txha pelvic.

Kuj tseem yam ntxwv ntawm tus mob ntshav qab zib Fofat yog:

Kev txo qhov nqaij leeg.
Tshuaj Spasmophilia.
O-puab curvature ntawm ob txhais ceg.
Pseudo-pob txha tawg thiab pob txha deformities.
Ua kom luv luv ntawm cov nqua, feem ntau tsis sib luag

Kev xoo hluav taws xob xoo hluav taws xob pom dav dav diaphysis (nruab nrab ntawm tubular pob txha), lub cev pob txha tsis zoo, txha pob txha, txhav txhav qeeb, cov pob txha muaj cov calcium ntau nyob rau hauv.

Cov yam ntxwv kuaj pom muaj txiaj ntsig yog qhov tsis muaj lus teb rau kev noj cov tshuaj vitamin D, tsis zoo li cov rickets, uas muaj phosphate ntshav qab zib, nws txoj kev siv tsis txo cov tsos mob ntawm tus kab mob.

Tsis tas li, thaum ua kev kuaj mob, kuaj pom ntawm phosphates nyob hauv cov zis yog ntau zaug ntau dua li cov txiaj ntsig ib txwm muaj.

Qhov kuaj ntshav qhia cov ntsiab lus phosphorus tsawg. Txhawm rau tshem tawm cov kab mob ntawm parathyroid caj pas, theem ntawm parathyroid hormone raug kuaj xyuas. Nrog phosphate mob ntshav qab zib, nws yog nce lossis tsis pub dhau ib txwm muaj. Kev kuaj pom nrog cov kev qhia ntawm parathyroid hormone qhia ib tug tsis tshua rhiab ntawm lub raum leeg rau nws.

Tsis tas li ntawd, nyob rau hauv cov neeg mob, qee zaum nce siab txog kev ua si alkaline phosphatase thiab muaj cov calcium nyob hauv cov ntshav qis tuaj yeem paub yog tias kev kho mob nrog cov tshuaj phosphorus ntau dua tau teev tseg.

Ntshav qab zib phosphate hauv cov menyuam yaus raug kho nrog phosphoric acid ntsev ntawm calcium thiab sodium ntawm 10 mg ntawm phosphate rau 1 kg ntawm tus menyuam lub cev hnyav 4 zaug hauv ib hnub. Cov tshuaj raug coj los ua txoj hauv kev daws teebmeem lossis ntsiav tshuaj.

Txhawm rau tiv thaiv kev ua txhaum ntawm cov tshuaj calcium metabolism, cov tshuaj vitamin D tau sau tseg. Nws siv pib los ntawm 0.005 μg thiab nce ntxiv rau 0.03 μg rau 1 kg ntawm lub cev qhov hnyav. Nyob rau tib lub sijhawm, theem ntawm phosphorus hauv cov ntshav nce, thiab cov kev ua ntawm alkaline phosphatase tsawg dua.

Txhawm rau txhawm rau txhawm ntxiv ntawm calcium ntsev rau hauv daim ntawv ntawm lub raum pob zeb, theem ntawm calcium nyob hauv cov ntshav thiab zis yog tswj. Nrog rau cov nuj nqis ib txwm, koj tuaj yeem maj mam nce koob tshuaj vitamin d.

Tsis tas li, cov neeg mob zoo li no tau qhia txog kev teem caij calcium. Txhawm rau txhawm rau txhim kho kev nkag mus ntawm ntsev ntawm phosphorus thiab calcium los ntawm plab hnyuv lumen lawv tau sib xyaw nrog citric acid. Siv Tshuaj Calcium Gluconate, Fitin, Calcium Glycerophosphate, Sodium Citrate. Txoj kev kho yog nqa tawm rau lub sijhawm ntev - txog rau lub hlis.

Ib qho ntxiv, cov kev kho mob hauv qab no yog kho rau cov neeg mob:

Kev kho mob Vitamin nrog tocopherol (vitamin E) thiab vitamin A.
Xaiv cov orthopedic corset rau kev kho ntawm curvature ntawm tus nqaj qaum.
Thaum kawg ntawm kev loj hlob, kev kho mob phais mob tuaj yeem ua nrog pob txha deformation tseem ceeb.
Nrog oncogenic phosphate mob ntshav qab zib, ib qho qog yog tshem tawm.

Hauv cov theem nquag ntawm tus kab mob nrog mob hnyav hauv cov pob txha, txha nraub qaum thiab pob qij txha, cov neeg mob tau kho lub txaj pw. Feem ntau nws lub sijhawm yog li 15 hnub. Xav kom siv cov tshuaj pleev xim thiab tiv thaiv cov tshuaj uas tsis yog tshuaj steroid.

Thaum tus kab mob tau dhau theem ntawm cov chaw kho mob ruaj khov thiab chaw kuaj ntshav, tom qab ntawd cov neeg mob tau raug tso tawm kev ua lub cev nrog kev txwv tsis pub dhia thiab qoj ib ce.

Nws kuj tseem pom zoo kom ua ib chav kawm kev kho zaws, ntsev-coniferous thiab dej xau, tso tshuaj rau hauv cov mob sanatorium.

Plaub hom kabmob muaj qhov sib txawv raws li hom kev cuam tshuam ntawm metabolic. Thawj ob qho sib txawv ntawm tus kab mob yog qhov txaus siab (hais txog kev kwv yees ntev). Thawj qhov kev xaiv txhim kho nyob rau hauv thawj xyoo ntawm lub neej, cov pob txha me ntawm lub cev tsis meej, vitamin D tau pom zoo zoo. Nws cuam tshuam nrog kev tshem tawm ntawm phosphorus thiab calcium nrog zis thiab quav.

Hauv qhov pauv hloov thib ob, tus kab mob no txhim kho xyoo ob ntawm lub neej, tshaj tawm cov pob txha pauv, phosphorus hauv cov ntshav tau txo qis, ntau ntawm phosphorus yog ntaws hauv cov zis. Vitamin D qhia ua hauj. Poob phosphorus yog muaj feem cuam tshuam txij li lub raum puas tsuaj tshwm sim hauv ntshav qab zib mellitus. Kev loj hlob ntawm menyuam yaus yog nce siab me ntsis, lub cev nqaij tawv tseem muaj zog. Xoo hluav taws xob xoo ntawm lub rickets, pob txha mos.

Qhov kev xaiv thib peb pib thaum muaj hnub nyoog tsib xyoos, cov pob txha tau cuam tshuam loj heev, cov calcium nyob hauv cov ntshav txo qis, thiab calcium thiab phosphates tsis nqus rau hauv cov hnyuv. Vitamin D qhia tawm tsis kam.

Cov menyuam yaus muaj stunted, cov hniav uas muaj qhov tsis xws ntawm txha hniav laus, nyiam nyob rau hauv plab. Cov ntshav calcium qib qis yog qis dua, thiab ntau cov amino acids kuj nyob hauv cov zis. Muaj cov cim qhia ntawm kev ua haujlwm ntawm lub cev theem nrab ntawm cov roj ntsha parathyroid. Kev soj ntsuam xoo hluav taws xob qhia kev hloov pauv hauv thaj tsam ntawm kev pob txha loj, pob txha pob txha.

Qhov kev xaiv thib plaub yog pom los ntawm kev nkag siab ntau ntxiv rau cov vitamin D thiab ib qho kev nyiam ntawm hypervitaminosis, txawm tias thaum siv ua koob tshuaj me. Pib hauv menyuam yaus thaum ntxov, curvature ntawm ob txhais ceg, baldness thiab deformation ntawm cov hniav yog sau tseg.

Teeb meem ntawm phosphate mob ntshav qab zib muaj xws li:

Kev ua txhaum ntawm kev nyob ntawm lub cev thiab deformation ntawm qhov qis qis.
Lub cev thiab qee zaum kev xav qeeb.
Kev txhuam hniav thiab ua kom hniav sib xyaw uas cuam tshuam.
Calcium deposition hauv lub raum.
Kev nyuaj nyob hauv kev xa khoom (ntu kev phais yuav tsum tau).

Kev tiv thaiv tus kab mob muaj nyob rau hauv kev kuaj keeb kwm nyob rau theem ntawm kev npaj xeeb tub, tshwj xeeb yog tias muaj cov xwm txheej zoo li ntawd hauv tsev neeg lossis hauv cov neeg txheeb ze. Cov chaw pabcuam tawm tswvyim txog caj ces tuaj yeem tsim txoj kev pheej hmoo ntawm kev muaj mob ntshav qab zib phosphate.

Yog tias tus me nyuam muaj kev pheej hmoo, tom qab ntawd nws tau soj ntsuam txij thaum yug los, soj ntsuam qib ntawm phosphorus thiab calcium hauv cov ntshav thiab zis, thiab tseem saib xyuas cov pob txha pob txha, kev loj hlob ua raws li cov txheej txheem hnub nyoog, kuaj cov tshuaj tiv thaiv kev tiv thaiv kev tiv thaiv ntawm cov vitamin D. Hauv lub xub ntiag ntawm thawj cov cim qhia ntawm tus kab mob, cov menyuam tau raug tshuaj kev kho vitamin. Cov niam txiv yuav tsum thov cov txiaj ntsig rau tus menyuam uas muaj ntshav qab zib mellitus thiab tau txais tshuaj noj dawb thiab taug kev mus rau qhov chaw noj qab haus huv.

Hauv daim yeeb yaj kiab hauv tsab xov xwm no, Dr. Komarovsky tham txog kev tsis txaus cov vitamin D.

Ntshav qab zib phosphate: cov yam ntxwv ntawm tus kabmob thiab cov hau kev ntawm kev kho

Ntshav qab zib phosphate yog ib hom kab mob muaj keeb uas tsis muaj dab tsi ua rau ntshav qab zib. Pathology yog tsim vim kev mob ntshav siab ntawm lub cev tsis ua haujlwm hauv lub cev thiab nthuav tawm nws tus kheej thaum yau. Txhawm rau kom pom cov tsos mob thawj zaug ntawm kev mob hlwb hauv lub sijhawm thiab pib ua kev sib tw, nws yog ib qho tsim nyog yuav tsum muaj lub tswv yim ntawm cov tsos mob ntawm phosphate mob ntshav qab zib, nrog rau cov hau kev ntawm kev kuaj mob thiab kho mob.

Ntshav qab zib phosphate yog lub npe rau cov kab mob caj ces, cov yam ntxwv ntawm uas:

tsis muaj phosphorus nyob rau hauv lub cev,
kev txhim kho ntawm pob txha mob,
cov tsos ntawm rickets-zoo li cov tsos mob.

Ntshav qab zib phosphate yog ib qho kev mob uas tsis tshua pom muaj ntau tshaj plaws nrog 1: 20,000 lub caij nyoog ntawm kev tsim nws.

Txog yim hom kab mob pathology tau txawv raws qhov kev hloov pauv ntawm ib lub noob. Qhov tshwj xeeb tshaj yog cov X-txuas rau cov kab mob hypophosphatemic rickets vim tawg ntawm REX noob.

Raws li kev tshawb fawb txog kev kho mob niaj hnub no, tib neeg cov keeb kwm muaj xws li ntau tshaj 28 txhiab noob.

Ntshav qab zib phosphate yog ib tus kab mob ua tsis taus mis; tsis muaj ib qho tsim muaj cov kab mob pathology.

Tus kab mob no yog dab tsi?

Ntshav qab zib phosphate yog lub npe rau cov kab mob caj ces, cov yam ntxwv ntawm uas:

tsis muaj phosphorus nyob rau hauv lub cev,
kev txhim kho ntawm pob txha mob,
cov tsos ntawm rickets-zoo li cov tsos mob.

Ntshav qab zib phosphate yog qhov kev tshawb fawb caj ces tsis cuam tshuam nrog kev ua txhaum ntawm kev tsim tawm ntawm cov tshuaj insulin hauv cov neeg txiav ntshav thiab cov ntshav qabzib hauv cov ntshav. Tus kab mob no yog tshwm sim los ntawm kev siv cov metabolism hauv vitamin tsis raug, nrog rau phosphates. Nrog kev kuaj mob ntshav qab zib phosphate, tsis muaj qhov thim rov qab nqus cov tshuaj no rau hauv cov hlab hlaus, thiab cov pob txha pob txha yog tus cwj pwm tsis haum rau cov tshuaj lom neeg.

Nws suav hais tias yog kev ua txhaum tsis tshua muaj tshwm sim, qhov tshwm sim ntawm kev txhim kho uas yog 1: 20,000. Qhov tsim ntawm phosphate mob ntshav qab zib tsis cuam tshuam nrog kev puas tsuaj rau tus txiav thiab nce ntshav qabzib. Tus kab mob no, uas zoo li mob ntshav qab zib, muaj qhov mob ua ntej. Nws cov qauv qhia tau yog vim qhov tseeb tias phosphorus tsis yog nqus tau rov qab rau hauv cov ntshav hauv cov hlab ntsha. Hauv cov ntshav, nws cov concentration poob qis, cov qauv ntawm cov pob txha pob txha yog ua txhaum, uas ua rau muaj qhov tsis xws luag hauv kev tsim cov pob txha thiab anomalies hauv cov qauv ntawm lub cev.

N25.1 Cov Neeg Mob Ntshav Qab Zib ntshav insipidus
N25.8 Lwm yam cuam tshuam vim los ntawm raum txoj hlab ntsws ua haujlwm tsis zoo

Cov tseem ceeb ua thiab pathogenesis

Tus kab mob yog tus cwj pwm tsis txaus ntseeg los ntawm reabsorption (thim rov qab nqus) ntawm phosphates nyob rau qhov ze ntawm cov menyuam ob lub raum, uas ua rau txo qis hauv lawv qib hauv ntshav dej. Qhov tshwm sim no cuam tshuam ncaj qha rau kev txawv txav hauv kev ua haujlwm ntawm osteoblasts - cov pob txha hluas. Yog li ntawd, txoj hnyuv nqus ntawm phosphates, calcium, thiab vitamin D yuav raug txo kom tsawg.

Tus kab mob no yog qhov tshwm sim ntawm kev hloov pauv hauv X chromosome, uas cuam tshuam kev nqus ntawm phosphates hauv cov hlab ntsha, thiab tom qab ntawd pib txoj saw ntawm cov txheej txheem pathological uas cuam tshuam nrog kev nqus ntawm calcium los ntawm cov pob txha.

Tus txiv neej uas nqa lub cev tsis xws kis tau tus kab mob mus rau lawv cov ntxhais.
Cov poj niam uas muaj cov roj ntsha sib kis tau kis tus kabmob mus rau cov menyuam yaus ntawm ob hom txiv neej (thiab cov tub thiab cov ntxhais) sib npaug.
Feem ntau cov mob nyhav, cov tub hluas raug kev txom nyem los ntawm hypickosphatemic rickets. Hauv cov laus laus, tus kab mob no yuav cuam tshuam nrog cov qog ntshav hauv cov pob txha lossis cov nqaij mos.

Kev faib tawm

Hauv kev kho mob niaj hnub no, 5 yam kab mob tseem ceeb tau muab coj los sib txawv, nyob ntawm seb cov noob tau dhau los sib kis.

X-txuas yam tseem ceeb yog hom kabmob sib kis, uas kev pom sai ntawm phosphates nyob rau hauv cov zis tau pom, tiv thaiv cov vitamins D. Pathology qhia tau nws tus kheej thaum thawj ob xyoos ntawm tus menyuam lub neej. Nyeem txog Rickets Vitamin D-Resistant Rickets
Autosomal recessive type - tus kab mob no tsis tshua muaj, nkag siab tsis txaus, tshwm sim vim kev hloov pauv hauv cov gene, uas yog lub luag haujlwm rau kev tsim kho hniav li qub, cov nqaij pob txha.
X-txuas ntxiv rau cov kev ua tsis zoo - cov xeeb txawm muaj ntawm cov noob hloov tau yog cov poj niam, tab sis tus kab mob tsuas yog tsim rau cov txiv neej. Pathology yog tus cwj pwm los ntawm kev hloov pauv ntawm phosphate ions los ntawm epithelium ntawm nephrons.
Autosomal hom tseem ceeb - yog suav tias yog daim ntawv me tshaj plaws ntawm pathology, tus cwj pwm los ntawm kev muaj zog ntau ntxiv ntawm phosphate ions hauv cov zis, hypophosphatemia.
Autosomal recessive type nrog hypercalciuria yog ib hom muaj mob tsawg. Hauv cov zis, cov ntsiab lus ntawm phosphorus thiab calcium nce ntxiv, hauv cov ntshav ntshav cov ntshav theem ntawm cov ntsiab no tsawg dua.

Tseem Ceeb! Ntshav qab zib phosphate yog ib tus kab mob ua ntej yug los. Daim foos tau txais hauv cov neeg laus yog pom muaj qee tus benign neoplasms hauv cov pob txha thiab cov nqaij mos, hu ua oncogenic rickets.

Thawj qhov tshwm sim hauv cov menyuam yaus

Ntshav ntshav phosphate tau tshwm sim li cas hauv menyuam yaus?

Muaj kev loj hlob qeeb.
Ob txhais ceg yuav ntswg.
Lub hauv caug thiab pob taws pob qij txha tau deformed.
Cov pob txha nyob hauv thaj chaw ntawm pob taws pob qij txha yog cov tuab tuab.
Txo cov leeg nqaij leeg.
Thaum lub sij hawm palpation, hnov mob hauv qab thiab pob txha. Kev mob hnyav yuav ua rau tus menyuam nres ntawm nws tus kheej taw.
Muaj qee zaus, txha hniav laus nyob ntawm cov hniav, rickets hauv tus txha nqaj lossis txha caj dab rau pom.
Spasmophilia, tus yam ntxwv ntawm rickets nrog cov vitamin D tsis txaus, tuaj yeem tsim kho.
Cov ceg tawv ntawm cov menyuam mos tuaj yeem muaj luv luv (feem ntau tsis sib luag).
Nrog lub hnub nyoog, tus neeg mob nthuav dav osteomalacia.
Cov duab xoo hluav taws xob tau qhia txog kev txhim kho txha, lub cev pob txha tau tsim los lig.
Cov yam ntxwv zoo calcium hauv cov pob txha.
Cov sib xyaw ua ke ntawm electrolytes, ntshav amino acids, creatinine, CBS tsis hloov.

Ib tug menyuam yaus xav tau cov khoom muaj txiaj ntsig zoo thiab muaj txiaj ntsig zoo, nrog rau phosphorus thiab calcium, txhawm rau kev loj hlob thiab kev loj hlob. Qhov tsis muaj cov ntsiab lus no thaum thawj xyoo ntawm lub neej piav qhia qhov hnyav ntawm tus kab mob.

Cov tsos mob ntawm ntshav qab zib Phosphate

Kev tshwm sim ntawm phosphate mob ntshav qab zib vim qhov caj ces heterogeneity ntawm tus kab mob no yog tus cwj pwm los ntawm ntau qhov sib txawv ntawm qhov mob hnyav - los ntawm cov chav kawm yuav luag tsis muaj qhov tsis pom kev ua rau muaj mob loj. Qee qhov xwm txheej ntawm pathology (piv txwv li, vim kev hloov pauv hauv FGF23 cov noob) tuaj yeem ua rau lawv tus kheej tsuas yog hypophosphatemia thiab nce ntxiv hauv theem ntawm phosphorus hauv cov zis, thaum tsis muaj tsos mob tshwm sim. Txawm li cas los xij, feem ntau, feem ntau phosphate mob ntshav qab zib ua rau cov duab ntawm rickets ib txwm muaj thiab feem ntau muaj kev txhim kho thaum yau - 1-2 xyoo, tsis ntev tom qab tus menyuam pib taug kev.

Cov chav kho mob ntawm phosphate mob ntshav qab zib yog polymorphic. Tus kab mob feem ntau ua rau nws qhov tshwm sim thaum yau, tab sis tuaj yeem tshwm sim nws tus kheej tom qab - ntawm 7-9 xyoo. Ntxiv mus, qhov mob ntawm cov tsos mob pathological kuj tseem yuav sib txawv. Qee qhov xwm txheej, tus kab mob no muaj kev kawm asymptomatic thiab tshwm sim los ntawm kev hloov me me hauv phosphorus-calcium metabolism.

Cov kev qhia pom no yog los ntawm kev ua txhaum ntawm kev ncaj ncees ntawm tus txha hniav laus thiab kev txhim kho ntawm ntau caries, curvature ntawm tus nqaj qaum thiab pob txha pelvic.

Cov tsos mob uas qhia tias qhov tshwm sim ntawm cov teeb meem kev noj qab haus huv uas tau txhawb txoj kev loj hlob ntawm phosphate mob ntshav qab zib yog li hauv qab no:

Deterioration hauv kev mob siab rau menyuam.
Stunted kev loj hlob.
Kev nkhaus ntawm qis qis hauv ib qho o-puab.
Hloov pob taws lossis hauv caug.
Cov leeg leeg nruj dua.
Hauv thaj tsam ntawm lub nraub qaum thaum palpating, qhov mob tshwm sim.
Hloov tus me nyuam lub gait (zoo li os)
Ua txhaum ntawm kev tsim ntawm cov hniav,
Pathological pob txha lov,
Mob pob txha, thiab lwm yam.

Cov kev tshawb fawb soj ntsuam piav qhia txog tus tsos mob tseem ceeb ntawm tus kab mob rau qhov tias ib tus neeg muaj teeb meem nrog kev txhim kho ntawm cov leeg ua haujlwm thiab deformities ntawm qis qis tshwm sim. Tsis tas li ntawd, nyob rau hauv cov neeg muaj phosphate mob ntshav qab zib, lub cev luv, txawv txav thiab lwm yam kev teeb tsa ntawm pob txha pob txha dhau los, qhov hloov gait, qhov mob thaum lub sijhawm txav tuaj yeem raug taug.

Kev mob ntshav hauv lub cev, tus yam ntxwv ntawm qhov tseeb rickets, feem ntau yog qhaj ntawv phosphate mob ntshav qab zib. Cov kev txawj ntse hauv tus kab mob no tsis raug kev txom nyem.

Teeb meem

Vim tias tsis muaj sijhawm kho, cov neeg mob uas tau kuaj pom no tom qab tau txais cov kev mob tshwm sim tom qab no:

Kev ua txhaum ntawm tus nqaj qaum, raws li qhov tshwm sim - hwj tau sawv.
Lub hlwb lossis lub cev tsis meej nyob hauv kev loj hlob ntawm tus menyuam.
Cov pob txha lossis pob txha lov uas ua rau muaj kev tsis taus.
Pathology ntawm kev tsim kho hniav, ua txhaum ntawm lub sijhawm ntawm lawv txoj kev txhim kho.
Kev cuam tshuam ntawm kev txhim kho ntawm tus neeg mloog cov ossicle.
Tus mob Nephrocalcinosis, uas tau piav qhia los ntawm kev tso nyiaj ntawm calcium ntsev nyob rau lub raum.

Yog tias tus menyuam muaj lub siab xav mus rau qhov kev txhim kho ntawm tus kabmob no, tom qab ntawd nws qhov kev tshuaj xyuas pib yuav luag txij thaum yug los, txhawm rau tiv thaiv kev ntxhov siab ntau li ntau tau Txhawm rau ua qhov no, lawv tshawb xyuas qib ntawm calcium thiab phosphorus, saib xyuas seb pob txha pob txha loj hlob li cas, seb qhov kev loj hlob puas sib haum nrog cov qauv uas yuav tsum muaj nyob rau lub hnub nyoog no.

Thaum pom thawj cov tsos mob ntawm ntshav qab zib phosphate, pom cov menyuam yaus yog cov vitamins. Yog qhov xav tau, cov niam txiv tuaj yeem npaj cov txiaj ntsig rau tus menyuam kom tau txais tshuaj dawb, nrog rau kev npaj mus ncig dawb hauv cov tsev noj qab haus huv tshwj xeeb.

Kuv yuav mus ntsib tus kws kho mob twg?

Txhawm rau txhawm rau txhawm ntxiv ntawm calcium ntsev rau hauv daim ntawv ntawm lub raum pob zeb, theem ntawm calcium nyob hauv cov ntshav thiab zis yog tswj. Nrog rau cov nuj nqis ib txwm, koj tuaj yeem maj mam nce koob tshuaj vitamin d.

Ntxiv rau cov vitamin D, cov neeg mob zoo li no tau pom zoo kom ua:

phosphorus thiab calcium npaj,
citrate sib tov (hauv 6 lub hlis txhawm rau txhim kho kev nqus ntawm cov ntsiab lus no),
kev loj hlob lawm.

Thaum lub sijhawm ua haujlwm siab ntawm cov txheej txheem, cov neeg mob tau qhia rau lub txaj so, tom qab ua tiav kev tshem tawm - kev kho mob zaws, kev ua si ntawm lub cev thiab kev kho mob spa.

Cov qauv rau kev ua haujlwm ntawm kev txuag kho yog:

kev nyob zoo,
kev ua kom muaj kev nrawm nrawm,
normalization ntawm phosphorus metabolism hauv lub cev,
zoo kev thaij duab hluav taws xob (rov kho lub cev pob txha qub).

osteotomy (dissection) ntawm cov leeg pob txha ntev nrog kev kho ntawm lub axis ntawm nqua,
limb kev immobilization los ntawm Ilizarov kev cuam tshuam thiab ntsuas lub tshuab.

Cov kev ua haujlwm zoo li no yuav tsum tau nqa tawm tsuas yog tom qab lub sijhawm muaj kev txuag ntau thiab kev soj ntsuam kom zoo.

Kev siv dag zog hauv kev kho mob rau cov ntshav qab zib phosphate

Kev siv dag zog hauv lub cev uas tau siv los kho tus mob:

Laser Kho Txoj kev kho no ua rau kev sib koom tes sib koom tes thiab txo qhov sib zog ntawm qhov mob hauv lawv. Cov txheej txheem tshwj xeeb yog nyob ntawm cov nyhuv ntawm laser hluav taws xob rau ntawm cov nqaij. Hauv qhov no, cov txheej txheem inflammatory raug tshem tawm, cov txheej txheem tsim dua tshiab yuav ua kom nrawm dua. Lub teeb ci tau muab pub rau qhov chaw ua teeb meem siv lub pob taws tshwj xeeb.
Hlau nplaum kho. Txoj kev no tso cai rau koj ntxiv dag zog rau lub cev, txhim kho kev noj qab haus huv tag nrho thiab ua kom nrawm rov kho dua ntawm pob txha. Txoj kev kho no yog los ntawm cov txiaj ntsig ntawm qhov chaw sib nqus ntawm tib neeg lub cev.
Electrophoresis Txo qhov mob hauv pob qij txha thiab pob txha. Txoj kev no yog raws li kev muaj peev xwm ntawm hluav taws xob ntxias kom coj yeeb tshuaj hauv thaj chaw muaj kev puas tsuaj. Hauv qhov xwm txheej no, ua kom lub cev tuaj yeem ua rau thaj chaw muaj kev cuam tshuam nce. Cov paib tshwj xeeb tam sim no tau muab tso rau saum cov teeb meem sib koom ua ke, tau muaj yav tas los kho nrog tshuaj ntsuab.
Ultraphonophoresis. Nws zoo ib yam li cov qauv dhau los, txawm li cas los xij, cov tshuaj hauv qhov teeb meem no tau mus txog thaj chaw muaj teeb meem vim kev deeg ultrasonic. Cov txheej txheem yog kev nyab xeeb thiab tsis mob.

Kev kwv yees ntawm phosphate ntshav qab zib tuaj yeem sib txawv thiab nyob ntawm ntau yam - hom kab mob, qhov hnyav ntawm cov tsos mob, hnub nyoog txiav txim siab tus kab mob pathology thiab pib kho kom raug. Ntau zaus, qhov pom tias muaj txiaj ntsig zoo, tab sis kev xav tau ntev mus rau kev siv cov vitamin D, calcium thiab phosphorus npaj yuav nyob mus ntev. Kev mob pob txha uas tsis zoo uas yog los ntawm kev kuaj mob lig lossis kho tsis raug tuaj yeem cuam tshuam rau tus neeg mob lub neej.

Kev Tiv Thaiv

Ntshav qab zib phosphate yog ib hom mob uas kis tau los ntawm yuav luag txhua qhov mob los ntawm niam txiv mob rau menyuam. Yog tias nws qhov tsos tshwm sim vim caj ces, tus neeg lossis tus kws kho mob uas tsim nyog yuav tsis muaj peev xwm cuam tshuam nws txoj kev txhim kho thiab tsis suav qhov txaus ntshai ntawm tus kab mob.

Kev tiv thaiv ntawm cov kab mob no tsuas yog tswj hwm kev tiv thaiv qhov tshwm sim ntawm lub txim thiab txo kev pheej hmoo ntawm pob txha pob txha deformation hauv cov neeg mob uas muaj hnub nyoog txawv.

Nyob rau hauv muaj phosphate mob ntshav qab zib hauv cov neeg txheeb ze, nws yog qhov yuav tsum tau mus ntsib cov noob caj noob ces nyob rau theem ntawm kev npaj xeeb tub. Nws yog ib qho tseem ceeb kom paub qhov sib xws - tus neeg uas muaj keeb kwm ntawm tsev neeg muaj mob pib.

Lub cev qoj ib ce

Nrog phosphate ntshav qab zib, nws yog ib qho tseem ceeb los ua lub neej nquag. Cov neeg mob nrog tus kab mob pathology:

kev tawm dag zog thaum sawv ntxov, uas yuav tsum muaj kev tawm dag zog ntawm caj npab, ceg, qaum:
- taug kev hauv chaw
- zaum-sawv
- viav vias ob txhais ceg thiab caj npab,
kev caij tsheb kauj vab

ua luam dej, uas ua kom cov leeg muaj zog, txo qhov mob, muaj cov txiaj ntsig zoo ntawm cov hlab plawv.

Kev ua luam dej nce ntxiv cov leeg nruj, txhim kho txoj hlab ntshav

Kev ua neej nyob lub neej nrog phosphate muaj ntshav qab zib ua kom nrawm nrawm nrawm.

Kev tawm dag zog lub cev

Kev siv dag zog zoo rau kev mob ntshav qab zib ntshav qab zib muaj xws li:

zaws ntawm qhov qis dua thiab sab saud, uas cov ntshav ncig yog txhim kho, mob leeg nqaij yog li qub,
kev tawm dag zog lub cev.

Thaum pib, cov zaws yuav tsum tau nqa los ntawm tus kws kho mob hauv chaw kho mob. Yav tom ntej, cov neeg hauv tsev neeg ntawm tus neeg mob tuaj yeem kawm paub cov txheej txheem zaws thiab ua cov txheej txheem hauv tsev.

Cov nyom ntawm kev tawm dag zog tawm dag zog yog xaiv los ntawm tus kws kho mob tshwj xeeb rau txhua tus neeg mob. Thawj zaug yuav tsum yog nyob ntawm kev saib xyuas ntawm tus kws tshaj lij, tom qab kev tawm dag zog lub cev tuaj yeem ua nyob hauv tsev.

Noj zaub mov

Kev noj tshuaj kho mob yog ib qho tseem ceeb hauv kev kho mob ntshav qab zib Fofat, uas yuav pab tau:

normalize metabolism hauv
txhim kho kev mob ntawm pob txha thiab cov leeg.

Nrog phosphate mob ntshav qab zib, cov ntawv qhia zaub mov yuav tsum suav nrog cov khoom lag luam:

muaj ntau ntawm phosphorus,
enriched nrog calcium.

Rooj: cov zaub mov muaj txiaj ntsig rau cov ntshav qab zib phosphate

Khoom Noj Muaj Khoom Noj hauv Phosphorus	Cov Khoom Noj Khoom Noj Khoom Noj Khoom Haus Zoo calcium
ntses nqaij nruab deg taum pauv lentils taum cheese lub qe.	tsev cheese cheese taum noob hnav noob almonds cov kaus poom salmon thiab sardines, zaub qhwv zaub ntsuab zaub txhwb qaib rhubarb.

Kev cais tawm ntawm cov zaub mov noj yuav tsum yog cov zaub mov uas ua rau lub raum thiab lub plawv mob ua haujlwm:

ntsev, uas dehydrates lub cev thiab cuam tshuam tsis zoo rau tag nrho cov kab ke hauv lub cev,
cov khoom noj muaj roj. Kev muaj rog ntau dhau hauv cov zaub mov muaj ntau ntxiv yuav txo cov roj cholesterol hauv cov ntshav, uas tuaj yeem ua rau kev txhim kho vascular pathologies. Tsis tas li, cov rog ua zaub mov tsis zoo rau lub cev metabolism, ua kom lub nra ntawm lub raum,
ntsim, ntsim, noj zaub mov. Xws li cov khoom ua rau lub plab zom mov ntawm lub plab,
cawv Cov dej cawv muaj cawv ntxiv ua rau cov ntshav siab, pab txhawb kev txhim kho ntawm lub cev ntawm lub cev, lom lub cev nrog cov tshuaj lom neeg lom.

Cov kev kuaj mob thiab qhov tshwm sim tuaj yeem tshwm sim

Qhov ntsuas ntawm kev kho mob rau ntshav qab zib phosphate yuav ua raws li cov hauv qab no:

kev ncua sijhawm ntawm kev kuaj mob,
kev paub ntawm cov kev kho mob kws kho mob,
cov kev ua kom yog ntawm tus neeg mob lossis nws niam nws txiv (yog tias peb tab tom tham txog tus menyuam muaj phosphate ntshav qab zib).

Cov teeb meem mob ntshav qab zib ntau ntawm phosphate muaj xws li:

urolithiasis,
osteoporosis (txo pob txha ceev),
scoliosis (curvature ntawm tus txha caj qaum),
arrhythmia (lub siab tus cwj pwm txawv),
arterial hypertension (mob ntshav nce siab),
txhawm ntawm nqua.

Qhov kev tu siab tshaj plaws yog qhov kev ua tiav ntawm tus neeg mob lub cev.

Cov Phosphate mob ntshav qab zib

Nrog phosphate ntshav qab zib, cov kev tsis zoo xws li tsim:

Cov pob txha loj hlob
Cov pob txha mos
Kev puas tsuaj ntawm pob taws thiab pob qij txha
Kev vam meej sai zuj zus ntawm qhov nqaj deformity.

Cov cim qhia ntawm cov ntshav qab zib phosphate

Cov kev qhia pom no yog los ntawm kev ua txhaum ntawm kev ncaj ncees ntawm tus txha hniav laus thiab kev txhim kho ntawm ntau caries, curvature ntawm tus nqaj qaum thiab pob txha pelvic.

Kuj tseem yam ntxwv ntawm tus mob ntshav qab zib Fofat yog:

Kev txo qhov nqaij leeg.
Tshuaj Spasmophilia.
O-puab curvature ntawm ob txhais ceg.
Pseudo-pob txha tawg thiab pob txha deformities.
Ua kom luv luv ntawm cov nqua, feem ntau tsis sib luag

Kev kuaj mob ntawm tus kabmob

Tsis tas li, thaum ua kev kuaj mob, kuaj pom ntawm phosphates nyob hauv cov zis yog ntau zaug ntau dua li cov txiaj ntsig ib txwm muaj.

Tsis tas li ntawd, nyob rau hauv cov neeg mob, qee zaum nce siab txog kev ua si alkaline phosphatase thiab muaj cov calcium nyob hauv cov ntshav qis tuaj yeem paub yog tias kev kho mob nrog cov tshuaj phosphorus ntau dua tau teev tseg.

Mob ntshav qab zib Phosphate kho

Ib qho ntxiv, cov kev kho mob hauv qab no yog kho rau cov neeg mob:

Kev kho mob Vitamin nrog tocopherol (vitamin E) thiab vitamin A.
Xaiv cov orthopedic corset rau kev kho ntawm curvature ntawm tus nqaj qaum.
Thaum kawg ntawm kev loj hlob, kev kho mob phais mob tuaj yeem ua nrog pob txha deformation tseem ceeb.
Nrog oncogenic phosphate mob ntshav qab zib, ib qho qog yog tshem tawm.

Thaum tus kab mob tau dhau theem ntawm cov chaw kho mob ruaj khov thiab chaw kuaj ntshav, tom qab ntawd cov neeg mob tau raug tso tawm kev ua lub cev nrog kev txwv tsis pub dhia thiab qoj ib ce.

Nws kuj tseem pom zoo kom ua ib chav kawm kev kho zaws, ntsev-coniferous thiab dej xau, tso tshuaj rau hauv cov mob sanatorium.

Qhov tshwm sim thiab lwm yam mob

Qhov kev xaiv thib peb pib thaum muaj hnub nyoog tsib xyoos, cov pob txha tau cuam tshuam loj heev, cov calcium nyob hauv cov ntshav txo qis, thiab calcium thiab phosphates tsis nqus rau hauv cov hnyuv. Vitamin D qhia tawm tsis kam.

Teeb meem ntawm phosphate mob ntshav qab zib muaj xws li:

Kev ua txhaum ntawm kev nyob ntawm lub cev thiab deformation ntawm qhov qis qis.
Lub cev thiab qee zaum kev xav qeeb.
Kev txhuam hniav thiab ua kom hniav sib xyaw uas cuam tshuam.
Calcium deposition hauv lub raum.
Kev nyuaj nyob hauv kev xa khoom (ntu kev phais yuav tsum tau).

Hauv daim yeeb yaj kiab hauv tsab xov xwm no, Dr. Komarovsky tham txog kev tsis txaus cov vitamin D.

Vim li cas thiab kev loj hlob yam

Lub hauv paus tseem ceeb ntawm tus kab mob yog lub raum plab hlaus lim vim kev hloov caj ces. Ib txoj hauv kev tsis yog ntawm tus poj niam cev xeeb tub, nws quav cawv, thiab haus luam yeeb lossis siv tshuaj yeeb tuaj yeem ua rau cov txheej txheem sib luag. Txoj kev pheej hmoo ntawm cov kev tsis zoo no tau nce ntxiv hauv cov neeg uas lawv cov txheeb ze muaj ntshav qab zib phosphate. Nyob rau tib lub sijhawm, nws yuav luag tsis tuaj yeem zam qhov qub txeeg qub teg los ntawm cov kev mob nkeeg ntawd.

Lwm qhov ntxiv rau:

noj tshuaj muaj zog hauv thawj peb lub hlis thaum cev xeeb tub,
mob toxicosis,
rhesus tsis sib haum xeeb.

Raws li kev txheeb cais, nyob rau hauv feem ntau ntawm cov mob, tus kabmob kis tau los ntawm leej txiv mus rau tus ntxhais, txawm hais tias muaj hom kev ncaus thiab leej niam.

Soj ntsuam duab

Hauv thawj 1-2 xyoos ntawm lub neej, pathology yuav tsis tshwm sim li. Qhov tsuas yog qhov qhia tau tias yog tus kabmob yog qhov muaj cov ntsiab lus ntawm phosphorus ions hauv kev tso zis. Thaum tus me nyuam pib taug kev, tom qab ntawd cov txheej txheem pathological ua rau nws tus kheej xav. Hauv qhov no, ob txhais ceg yog deformed, uas tau txais O-puab. Tus menyuam yaus kuj ua rau lub cev tsis muaj zog thiab lub cev ua tsis tau txhua yam.

kev loj hlob lag
lub cev tsis muaj zog
plaub hau poob
tsis zoo ntawm cov hniav
mob nraub qaum
tas li lov.

Nrog phosphate mob ntshav qab zib, txhais ceg cov pob txha muag thiab ua tau tawg

Yog tias tsis kho, tus kab mob no mus zuj zus, kev mob hnyav hauv cov pob txha thiab pob qij txha tshwm sim, vim qhov ntawd ua rau tus menyuam ploj mus tsis taus.

Kev kuaj mob

Tshawb nrhiav cov hauv kev uas siv los txheeb xyuas cov kab mob pathology:

Kev tsom xam cov zis thiab ntshav. Nws tso cai rau cov qib phosphorus hauv plasma thiab zis. Hauv thawj kis, tus naj npawb ntawm ions txo tsawg, thiab hauv qhov thib ob, ntawm qhov tsis sib xws, nws nce. Daim duab no tso cai peb xav tias muaj cov mob ntshav qab zib phosphate.
CT thiab MRI. Kev suav tomography ua rau nws muaj peev xwm los soj ntsuam kev txawv txav hauv tus qauv ntawm pob txha. Hauv qhov no, koj tuaj yeem pom ib qho kev hloov pauv hauv daim duab. Magnetic resonance imaging yog zoo li xam xaj tomography, tsuas yog thaum nws tsis siv, xoo hluav taws xob xoo hluav taws xob, tab sis nuclear hluav taws xob resonance. Siv cov qauv no, koj tuaj yeem tau txais cov ncauj lus kom ntxaws ntxiv los ntawm cov kev kwv yees sib txawv ntawm cov pob txha thiab pob qij txha.
Kev xoo hluav taws xob Nws yog ib txoj kev qub hauv kev tshawb fawb hauv kev ua txhaum ntawm cov pob txha txhim kho. Hauv daim duab koj tuaj yeem pom tus cwj pwm curvature ntawm tus kab mob no. Hauv qhov no, cov pob txha ua kom nyias nyias thiab tsis tshua muaj siab los cuam tshuam.

MRI yog ib qho ntawm cov kim tshaj tab sis kev tshawb fawb cov ntaub ntawv rau phosphate mob ntshav qab zib.

Tsis tas li ntawd, kev kuaj mob ultrasound ntawm lub raum tuaj yeem siv, uas kev hloov me ntsis pathological hauv cov qauv ntawm cov kabmob tau kuaj pom. Hauv qhov no, koj tuaj yeem nrhiav cov xuab zeb thiab pob zeb, feem ntau cuam tshuam nrog tus kabmob no.

Kho kho kev tawm tsam

Qhov kab ke no yog qhov tsis tuaj yeem kho, tab sis nrog kev sib koom ua ke ntawm kev kho, nws muaj peev xwm ncua kev txhim kho ntawm tus kabmob. Rau cov laj thawj no, siv tshuaj kho mob, kho lub cev thiab zaub mov noj yog siv. Raws li txoj kev pabcuam lwm txoj kev, lwm txoj hauv kev tau siv. Hauv kev ua kom zoo, mus rau kev phais mob. Lub luag haujlwm yog hnav ntawm cov qauv orthopedic tshwj xeeb - cov ntaub qhwv uas tiv thaiv kev deformation thiab curvature ntawm nqaj qaum.

Phosphate Diabetes Bandage tiv thaiv pob txha caj qaum

Kev sib xyaw ua ke ntawm kev siv tshuaj thiab kev kho lub cev tuaj yeem txhim kho cov neeg mob lub cev kom zoo, nrog rau kev tshem tawm lossis txo kev mob.

Tshuaj kho mob nkeeg

Cov tshuaj hauv qab no yog siv los kho cov kab mob pathology:

Kev npaj uas muaj cov ntsev phosphate ntsev: Osteogenon, Tridine, thiab lwm yam. Cov tshuaj no txhim kho cov mob pob txha, tiv thaiv kev hloov pauv ntxiv thiab pathological kev hloov hauv cov qauv ntawm pob qij txha. Cov tshuaj kho tau muab tshuaj rau lub sijhawm ntev (txij li 6 lub hlis lossis ntau dua).
Vitamin D: Aquadetrim, Ultra-D, thiab lwm yam. Cov tshuaj hauv pawg no yog qhov tseem ceeb rau kev nqus ntawm phosphorus ions thiab kom txo tau lawv cov zis hauv cov zis. Cov koob tshuaj ntawm cov vitamins yog maj nce, chav kawm ntawm kev kho kuj ntev.
Kev npaj calcium uas muaj: Fitin, Calcium gluconate thiab lwm yam. Cov tshuaj noj los ntawm pab pawg no ntxiv dag zog rau cov pob txha, tiv thaiv kev hloov pauv pathological thiab txhim kho cov hniav. Cov tshuaj no ua rau cov calcium uas tsis txaus nrog cov nquag plias ntawm cov ntxhia no. Qhov no pab txhim kho txoj kev noj qab haus huv txhua yam muaj phosphate mob ntshav qab zib. Lub chav kawm ntawm kev kho mob yog los ntawm ntau lub hlis txog rau lub hlis.
Vitamin E: Tocopherol acetate, Aevit thiab lwm yam. Cov tshuaj los ntawm cov pab pawg no muaj cov nyhuv ua antioxidant, txhim kho kom zoo thiab muaj cov txiaj ntsig zoo rau cov mob ntawm tag nrho cov kab mob.

Kev siv tshuaj kho mob tuaj yeem tiv thaiv qhov kev vam meej ntawm tus kabmob thiab pab txhim kho cov pob txha pob txha, txo qis ntawm phosphorus thiab calcium hauv cov zis.

Kev phais mob

Kev phais mob yog kho nrog qhov mob me me rau qhov hnyav hnyav uas ua tsis tau ntawm ob txhais ceg. Qhov no yog qhov tsim nyog thaum lwm txoj kev ua tsis tiav. Koj tuaj yeem rov qab rau txoj kev kho no tsuas yog tom qab kawg ntawm pob txha loj hlob. Nrog rau kev phais mob, cov ceg tawv deformed raug kho siv cov koob tshwj xeeb knitting. Ua ntej tshaj, tus pob txha hla, uas yog, nws tau tawg thiab txav mus rau qhov chaw raug. Tom qab ntawd kho hauv thaj chaw uas xav tau siv cov qauv hlau (Ilizarov txujci).

Rov qab yog ntev. Lub lag luam yog ua nyob rau hauv kev ua kom loog. Cov koob txhaj tshuaj tsis tau txav los ntawm cov pob txha kom txog thaum nws tau pleev xim rau hauv qhov chaw raug. Nrog rau txoj hauv kev no, koj tuaj yeem tshem tawm qhov mob tas mus li. Hmoov tsis zoo, nrog kev txiav txim siab hnyav, txoj kev qhia no tsis tuaj yeem siv tas li.

Kev kho cov pob txha deformities tom qab kev phais mob raws li tus qauv Ilizarov

Kev nrawm nrawm ntawm kev ua haujlwm yog txiav txim los ntawm tus kws kho mob tom qab kev kuaj ntshav thiab mus kuaj duab xoo. Nrog kev rhuav tshem tseem ceeb ntawm cov pob txha pob txha, kev phais mob yog txhawm rau txhim kho rov qab thaj chaw muaj kev puas tsuaj.

Kev phais mob txhawm rau tso cai rau koj tau txais tshem ntawm curvature ntawm ob txhais ceg, uas feem ntau nrog cov neeg mob ntshav qab zib phosphate.

Cov tshuaj kho neeg mob

Txoj hau kev no yog tsom rau lub cev ua kom muaj zog thiab txhim kho kev zom zaub mov. Cov zaub mov noj no tsis tuaj yeem tshem ntawm phosphate ntshav qab zib thiab tsis cuam tshuam rau qhov nqus ntawm phosphorus. Lwm txoj hauv kev uas tuaj yeem siv los txhim kho lub raum thiab lub cev kom tag nrho:

Txiv ntoo sib tov. Rov ua kom tiav qhov tsis txaus ntawm cov txiaj ntsig cov txiaj ntsig, txhim kho lub cev tsis muaj zog. Koj yuav xav tau walnuts, almonds thiab hazelnuts nyob rau hauv daim ntawv qhuav. Tag nrho cov khoom xyaw yuav tsum tau muab sib xyaw nrog tus sib npaug (100 g txhua), tom qab ntawd ntxiv 2 tbsp. l zib ntab. Lub txiaj ntsig sib xyaw yog pom zoo kom zom rau hauv rab thiab siv 1 tbsp. l 3 zaug hauv ib hnub 1 teev tom qab noj mov. Lub chav kawm ntawm kev kho mob yog 1 hlis.
Kho tus Txoj kev lis ntshav. Ua kom lub cev tiv thaiv, txhim kho raum ua haujlwm. Yuav xav tau pob kws stigmas hauv qhov nyiaj ntawm 1 tbsp. l., uas yuav tsum tau muab nchuav 300 ml dej rhaub kom npau thiab cia nws ywg rau 2 teev. Tom qab ntawd lim cov kua thiab noj 100 ml 3 zaug hauv ib hnub 15 feeb ua ntej noj mov. Lub sij hawm ntawm kev kho mob yog 2 lub lis piam.
Anti-inflammatory broth. Txhim kho txoj haujlwm ntawm lub raum, ua kom muaj zog tiv thaiv kab mob. Koj yuav tsum muab cov khoom sib npaug ntawm cov paj chamomile thiab calendula paj. 1 tbsp. l hliv 400 ml ntawm dej npau thiab rhaub dua li cov cua sov li 15 feeb. Tom qab ntawd cia cov kua kom txias thiab lim. Npaj zaub los noj 100 ml 3 zaug nyob rau ib hnub ib teev tom qab noj mov. Lub chav kawm ntawm kev kho mob yog 10 hnub.
Kho da dej. Nws yuav pab tshem ntawm kev mob hauv cov pob txha thiab pob qij txha. Nws yog ib qho tsim nyog yuav tsum hu rau tus da dej, ntsuas dej kub hauv qhov uas yuav tsum tsis txhob siab tshaj 41 degrees. Hauv 2 tbsp. l hiav txwv ntsev koj xav tau ntxiv 3 tee ntawm ntoo thuv tseem ceeb roj. Qhov loj tshaj plaws yog kom tsis txhob overdo nws! Tom qab ntawd yaj ntsev rau hauv dej. Da dej li 15-20 feeb. Rov ua qhov txheej txheem raws li xav tau.

Cov tshuaj kho neeg mob tsis tuaj yeem siv los kho cov menyuam yaus hnub nyoog qis dua 12 xyoos. Cov hau kev no yog npaj rau cov laus xwb. Nws yuav tsum tsis txhob hnov qab tias cov menyuam yaus lub cev yog qhov nkag siab heev thiab tej zaum yuav tsis teb rau cov kev kho mob zoo tshaj plaws.

Raug kuaj thiab qhov teeb meem

Nrog rau txoj kev kho mob kom ncav sij hawm, kev sojntsuam tau pom zoo. Kev noj cov zaub mov calcium, vitamin D thiab phosphorus tas li yuav pab txhawb kev noj qab haus huv. Txawm li cas los xij, cov ntsiab lus no yuav tsum tau coj mus txog thaum kawg ntawm lub neej ua kev kho kom zoo. Yog tias pib muaj tus kabmob, tomqab ntawd koj tuaj yeem ntsib ntau tus mob, xws li:

qhov tseem ceeb ntawm lub cev deformities,
leeg nrob qaum curvature,
pob txha lov tas li txawm tias cov nqaij doog me thiab ntog,
teething tsis meej,
kev tsim ntawm kev suav hauv lub raum.

Kev kho mob thaum ntxov rau kev mob ntshav qab zib tau pib, qhov zoo dua rau tus menyuam. Yog hais tias pom muaj tus kab mob hauv thawj xyoo ntawm lub neej thiab nyob rau tib lub sijhawm tsim nyog kho, ces qhov kev pheej hmoo ntawm kev phom sij yuav tsawg.

Kev tiv thaiv kev ntsuas

Hmoov tsis zoo, nws tsis tuaj yeem tiv thaiv kev mob ntshav qab zib phosphate, txij li tus kab mob yog keeb kwm. Txawm li cas los xij, koj tuaj yeem txo qhov kev pheej hmoo ntawm kev phom sij rau lub cev, yog tias koj ua raws li txoj cai yooj yim ntawm kev tiv thaiv:

Noj kom zoo thiab haus dej kom ntau.
Noj cov vitamin complex uas muaj cov vitamin D, calcium thiab phosphorus.
Yuav tsum tau kuaj los ntawm kws kho mob tas li: kuaj ntshav thiab tso zis, kuaj ntshav ntawm lub raum.
Ntau zaus yuav tsum tau nyob hauv huab cua ntshiab.
Tsis txhob noj cov tshuaj muaj zog yam tsis tau ntawv kho mob los ntawm kws kho mob.

Kev kuaj ntshav tso cai rau koj los tswj qib uas phosphorus

Txhawm rau txo txoj kev pheej hmoo mob mob hauv menyuam yaus hauv plab, nws yog qhov yuav tsum tau sab laj nrog tus kws kho caj ces thaum npaj cev xeeb tub.

Lub luag haujlwm ntawm cov vitamin D hauv kev tiv thaiv phosphorus metabolism cuam tshuam - video

Ntshav qab zib phosphate yog ib qho mob loj heev uas tuaj yeem ua rau kom pob txha pob ntseg. Tib lub sijhawm, tus neeg hnov tsis tsuas yog physiological, tab sis kuj mob hlwb tsis zoo, yog li nws tseem ceeb heev kom tsis txhob quav ntsej thawj cov cim ntawm pathology. Kev noj cov tshuaj vitamin ntev ntev yuav pab txhim kho kev nyab xeeb.