Ntshav qab zib phosphate: ua rau, cov tsos mob, kuaj mob, kho mob

Hauv lub ntiaj teb niaj hnub no, yuav luag txhua tus niam txiv paub txog kab mob xws li rickets. Txhua tus kws kho mob menyuam yaus los ntawm thawj lub hlis ntawm ib tug menyuam lub neej tau ceeb toom tias yuav tsum muaj kev tiv thaiv tus kab mob no. Cov rickets yog hais txog cov kabmob kis tau uas tsim los ntawm cholecalciferol tsis muaj peev xwm, ib yam khoom muaj npe hu ua vitamin D.

Txawm li cas los xij, nyob hauv qee kis, cov menyuam yaus me ntsib cov tsos mob ntawm pathology, txawm hais tias soj ntsuam kev tiv thaiv. Thaum zoo li no, tus kab mob xws li phosphate ntshav qab zib yuav tsum xav tias yog. Tsis zoo li rickets, qhov mob no belongs rau caj ces pathologies. Yog li, nws yog tus kabmob hnyav dua thiab xav tau kev kho mob ntev ntev.

Mob ntshav qab zib yog dab tsi?

Qhov no yog lub npe ntawm ib pawg ntawm cov noob caj noob ces cuam tshuam nrog cov roj ntsha hauv lub cev tsis ua haujlwm. Lwm txoj kev, lub pathology yog hu ua vitamin D-resistant rickets. Cov hom mob ntshav qab zib hom phosphate tshwm sim hauv kwv yees li ntawm 1 ntawm 20,000 tus menyuam mosliab nyuam qhuav yug los. Tus kab mob no tau piav qhia thawj zaug xyoo 1937. Tom qab ntawd nws tau muab tawm tias muaj lwm cov caj ces ntawm pathology. Mob ntshav qab zib phosphate yog feem ntau pom muaj thaum menyuam yaus. Kev saib xyuas ntawm niam txiv yog qhov ntxim nyiam los ntawm kev ua tsis taus thiab nkhaus ntawm cov pob txha.

Qee cov qauv ntawm pathology nrog lwm cov teeb meem metabolic. Qee zaum tus kab mob yog asymptomatic, thiab nws tsuas yog kuaj tau los ntawm kev kuaj pom. Tus kab mob yog txwv kom tau nrog rickets nrhiav, hypoparathyroidism thiab lwm yam kab mob endocrine. Cov kev kho mob yuav tsum tau ua kom tiav txij li lub hnub nyoog ntxov.

Ua rau muaj tus kabmob

Qhov laj thawj tseem ceeb rau kev loj hlob ntawm phosphate ntshav qab zib yog qhov ua txhaum ntawm tus qauv keeb. Kev sib faib yog pub muaj. Cov neeg coj ntawm lub pathological noob tuaj yeem yog ob tus poj niam thiab txiv neej. Qhov kev sib hloov tshwm sim ntawm X-txuas nrog cov chromosome, uas yog tus tseem ceeb. Muaj qee zaus, phosphate mob ntshav qab zib muaj peev xwm nrhiav tau. Nws mob tawm tsam keeb kwm ntawm cov qog ntawm cov qog nqaij hlav mesenchymal, uas ua rau lub cev xeeb tub. Hauv qhov no, lub pathology yog hu ua "oncogenic rickets."

Tus kab mob no zwm rau pab pawg ntawm tubulopathies - lub raum khiav tsis zoo. Vim lub fact tias reabsorption ntawm cov zaub mov nyob rau hauv qhov tsis ntev tubules raug txo, phosphorus tsis muaj kev loj hlob hauv lub cev. Tsis tas li ntawd, cov khoom siv hauv plab hnyuv muaj qhov cuam tshuam. Raws li qhov tshwm sim, kev hloov pauv hauv cov ntxhia pob zeb hauv cov pob txha pob txha raug pom. Feem ntau muaj kev ua txhaum ntawm kev ua haujlwm ntawm osteoblasts. Tus txiv neej dhau cov kab mob pathologically hloov rau cov ntxhais, thiab poj niam - rau menyuam ntawm ob tus poj niam. Raws li txoj cai, hauv cov tub hluas tus kabmob muaj mob ntau dua hauv cov menyuam ntxhais.

Kev faib Cov Mob Ntshav Qab Zib Rau Cov Me Nyuam

Muaj ntau ntau hom kabmob uas txawv raws keeb kwm thiab kev kuaj mob. Feem ntau, X-txuas hypophosphatemic rickets yog kuaj tau, uas muaj hom tseem ceeb ntawm cov khoom qub txeeg qub teg. Txawm li cas los xij, muaj lwm yam sib txawv ntawm pathology. Kev faib tawm muaj cov qauv hauv qab no ntawm tus kabmob:

X-txuas hom tseem ceeb ntawm phosphate mob ntshav qab zib. Nws yog qhov txawv txav los ntawm kev hloov pauv ntawm PHEX gene, uas encodes endopeptidase. Qhov enzyme no yog lub luag haujlwm rau kev thauj ntau yam zaub mov los ntawm cov ion raws ntawm lub raum thiab cov hnyuv me. Vim tias qhov muaj kev hloov pauv rau cov noob, cov txheej txheem no ua rau qis qis, uas ua rau lub cev tsis muaj phosphate ions hauv lub cev.
X-txuas yam tsis cuam tshuam hom. Nws yog qhov cim hloov los ntawm kev hloov pauv ntawm CLCN5 gene, uas hloov cov protein ua lub luag haujlwm rau kev ua haujlwm ntawm chloride ion channels. Raws li qhov tshwm sim ntawm qhov tsis xws luag, thauj tag nrho cov tshuaj los ntawm daim nyias nyias ntawm lub raum hlaus muaj kev cuam tshuam. Cov neeg coj ntawm hom pathology tuaj yeem yog tus poj niam thiab tus txiv neej. Txawm li cas los xij, tus kab mob no tshwm sim rau cov tub nkaus xwb.
Autosomal hom hom phosphate mob ntshav qab zib. Nws cuam tshuam nrog kev hloov caj ces ntawm cov keeb kwm chromosome 12. Vim tias qhov tsis xws luag, osteoblasts secrete protein pathological uas txhawb kev ua kom zoo dua ntawm phosphates tawm hauv lub cev. Pathology yog tus cwj pwm kawm me me, hauv kev sib piv rau X-txuas cov ntawv.
Autosomal yam kev ntaus tawm. Nws yog tsawg. Nws yog qhov cim los ntawm kev tsis xws luag hauv DMP1 gene nyob rau ntawm chromosome 4. Nws yob ib qho protein ua lub luag haujlwm rau kev tsim cov pob txha caj dab thiab txhab hniav.
Autosomal recessive type, nrog rau kev nthuav tawm ntawm cov calcium hauv cov zis. Nws tshwm sim vim yog qhov tsis xws luag hauv lub gene nyob rau ntawm chromosome 9 thiab lub luag haujlwm rau kev ua haujlwm ntawm sodium-dependant renal channels.

Ntxiv nrog rau daim ntawv teev npe phosphate mob ntshav qab zib, muaj lwm hom pathology. Qee hom ntawm tus kabmob no tseem tsis tau raug tshawb nrhiav.

Qhov sib txawv ntawm Rickets thiab Phosphate Diabetes

Kev mob rickets thiab phosphate mob ntshav qab zib hauv cov menyuam yaus tsis zoo ib yam, txawm hais tias cov duab hauv chaw kuaj mob yuav luag zoo ib yam. Qhov sib txawv ntawm cov pathologies hauv etiology thiab lub tshuab ntawm kev txhim kho. Cov kab mob rickets zoo tib yam yog ib qho kev mob uas muaj nrog rau qhov tsis muaj cholecalciferol. Qhov khoom siv no yog tsim nyob rau hauv daim tawv nqaij nyob rau hauv tus ntawm tshav ntuj. Nws yog kev coj noj coj ua kuaj mob kuaj pom cov vitamin D ib hnub rau txhua tus menyuam txij li 1 hlis txog 3 xyoos, tsis suav lub caij ntuj sov. Vim yog vim li cas cholecalciferol tsis txaus, cov txheej txheem ntawm cov calcium uas nqus tau cuam tshuam. Raws li qhov tshwm sim, cov pob txha deformation loj tuaj.

Qhov sib txawv ntawm phosphate mob ntshav qab zib yog tias nws yog hais txog cov kab mob ua ntej. Nrog cov kab mob pathology no, kev nqus ntawm cov pob zeb hauv av hauv ob lub raum, tshwj xeeb hauv phosphates, muaj qhov tsis taus. Hauv qee kis, pom tias calcium tsis muaj teebmeem. Vim tias kev ua txhaum ntawm cov ntxhia hauv cov pob txha pob txha, cov tsos mob ntawm pathologies zoo sib xws. Qhov sib txawv ntawm lawv tuaj yeem tsim tau tsuas yog tom qab kuaj sim.

Hypophosphatemic rickets hauv cov menyuam mos: cov tsos mob ntawm tus kab mob

Cov tsos mob ntawm tus kab mob feem ntau kuaj pom nyob rau xyoo ob ntawm lub neej. Qee qhov tsis tshua muaj, kev tiv thaiv hypophosphatemic rickets hauv cov menyuam mos tau pom muaj. Cov tsos mob uas tuaj yeem kuaj tau hauv cov menyuam thaum tseem yau yog mob ntshav qis thiab ua rau luv luv ntawm nqua. Lub ntsiab soj ntsuam cov tsos mob yog:

Deformation ntawm cov pob qij txha ntawm ob txhais ceg.
O-puab nkhaus ntawm qhov qis qis.
Kev loj hlob qeeb hauv cov menyuam yaus - dhau los ua tus pom tom qab 1 xyoo.
Thickening ntawm pob qij txha pob qij txha thiab nqi pob txha mos - "rickety saws tes thiab rosary."
Mob hauv cov pob txha hu ua thiab pob txha nraub qaum.
Kev puas tsuaj rau tus hniav txha hniav laus.

Feem ntau, cov lus tsis txaus siab ntawm cov niam txiv tseem ceeb tshaj plaws yog tias tus menyuam tau khoov duav. Thaum tus mob loj zuj zus, cov pob txha mos tau tshwm sim - osteomalacia.

Kev kuaj mob ntawm kev mob ntshav qab zib thiab cov mob rickets

Txheeb xyuas cov kab mob pathology, kev kawm txog keeb kwm biochemical cov ntshav tau nqa tawm. Hauv qhov xwm txheej no, kev qhia tshuaj hypophosphatemia yog tau sau tseg. Qeb calcium hauv ntau feem ntau tsis muaj dab tsi, qee zaum nws txo qis. Thaum lub sij hawm qhov siab ntawm rickets, qhov nce ntawm alkaline phosphatase yog sau tseg. Radiography qhia tawm cov txha caj qaum thiab ua txhaum ntawm pob txha loj hlob ib cheeb tsam. Zis muaj qhov ntau ntawm phosphate. Qee zaum kuj pom Calciuria.

Kev kho mob ntawm rickets thiab phosphate mob ntshav qab zib

Cov tshuaj cholecalciferol loj loj tau siv coj los kho kho mob ntshav qab zib. Hauv qee kis, kev npaj tshuaj calcium yog qhia. Nco ntsoov siv cov tshuaj uas muaj phosphorus. Txhawm rau txhawm rau txhim kho tus mob, cov vitamins thiab pob zeb hauv av ua ntau yog kho. Cov no suav nrog kev npaj "Vitrum", "Duovit", "Niam ntawv", thiab lwm yam.

Nrog kev txiav hnyav ntawm cov pob txha, kev kho lub cev, kho kev tawm dag zog thiab kev phais mob yog qhia. Sab laj nrog orthopedic kws phais neeg yuav tsum tau sab laj.

Kev tiv thaiv kev ntsuas thiab qhov kwv yees

Cov txheej txheem tiv thaiv suav nrog kev kuaj mob ntawm tus kws kho menyuam yaus thiab kws phais neeg, taug kev hauv huab cua ntshiab, kev teem caij noj tshuaj vitamin D los ntawm thawj lub hlis. Yog tias muaj phosphate-mob ntshav qab zib cov neeg mob hauv tsev neeg, kev tshawb fawb raws caj ces yuav tsum tau ua thaum cev xeeb tub. Qhov raug teeb meem feem ntau pom zoo nrog kev kho mob raws sij hawm.

Cov tsev neeg thiab cov neeg tsis muaj caj ces: zoo sib xws thiab sib txawv

Nrog ntshav qab zib, phosphate mob ntshav qab zib ua ke sib txuam rau cov tsos mob thiab qhov tseeb tias nws tawg cov metabolism hauv. Nrog rickets - qhov tseeb tias nws ua rau kev txhim kho pob txha tsis zoo, thiab qhov no yog vim muaj qhov cuam tshuam cov metabolism hauv phosphorus thiab calcium. Phosphate mob ntshav qab zib hauv cov menyuam yaus zoo li mob uas tshwm sim thaum tsis muaj vitamin D hauv zaub mov, hauv cov neeg laus nws tshwm sim ntau tsawg dua thiab ua rau pom los ntawm cov pob txha mos thiab khoov ntawm cov pob txha - osteomalacia. Nws lwm cov npe yog cov vitamin D-depend rickets, hypophosphopenic rickets, rhawv kev sib raug zoo ntawm lub cev, hom 2 rickets.

Dab tsi tshwm sim hauv lub cev nrog tus kab mob no? Txhawm rau kom phosphorus thiab calcium tsim nyog nqus kom raug, thiab los ntawm cov pob txha muaj zog hauv lub cev, tsim cov vitamin D ua ntej.Thaum nws nkag mus rau hauv lub cev nrog cov zaub mov, nws tau hloov mus rau hauv cov tshuaj hormones tshwj xeeb.

Ib yam li cov tshuaj insulin pab cov cell nqus cov piam thaj hauv cov ntshav, vitamin D cov lus qhia cia lawv nqus phosphorus thiab calcium. Thiab tib txoj kev ib yam li nyob rau hauv cov ntshav qab zib mellitus, insulin ntau lawm lossis cell rhiab rau nws yog cuam tshuam, hauv phosphate ntshav qab zib cov txheej txheem ntawm kev tsim cov tshuaj tsim nyog rau lub cev los ntawm cov vitamin D cuam tshuam los yog daim ntaub so ntswg rhiab rau cov tshuaj txo qis. Tsis cuam tshuam los ntawm cov pob txha, cov calcium nyob hauv cov ntshav, thiab phosphorus yog ntxuav tawm nrog cov zis.

Qhov cuam tshuam ntawm ntshav qab zib phosphate kuj zoo ib yam li cov uas tshwm sim yam tsis tau kho rickets. Tus menyuam loj hlob, cov pob txha ntawm nws lub cev pob txha txuas mus ntxiv, thiab hauv cov neeg mob hnyav, nws poob lub peev xwm txav ntawm nws tus kheej.

Kev xiam oob khab tseem ua rau cov neeg laus muaj kab mob, uas yog, tsis yog hom kab mob - ntshav qab zib mob ntshav qab zib ua rau cov kab mob uas ua rau nws mob.

Cov tsos mob thiab kuaj mob

Hauv cov menyuam yaus, qhov xav tau phosphorus thiab calcium muaj ntau dua li cov neeg laus, yog li qhov tshwm sim ntawm tus kabmob nyuab dua. Raws li cov tsos mob tseem ceeb, phosphate mob ntshav qab zib sib luag nrog rickets. Hauv particular, cov no yog:

tsis ruaj tsis khov gait,
curvature ntawm qis dua ceg nrog tsab ntawv O,
qis dua qub kev loj hlob
curvature ntawm nqaj qaum.

Thaum muaj hnub nyoog ntxov, phosphate mob ntshav qab zib feem ntau kuaj tau tom qab niam txiv pom tias tus me nyuam tsis txav mus ntau. Qee tus menyuam yaus quaj thiab ua thaum lawv raug yuam kom taug kev - lawv cov pob txha raug mob. Nrog rickets, cov pob txha los ua brittle, yog li txawm tias curvature tseem tsis tau pom, tus kab mob tuaj yeem ntseeg tau rau cov menyuam yaus uas feem ntau ntsib yam tsis muaj pob txha lov.

Tab sis muaj qhov sib txawv ntawm cov tsos mob ntawm cov rickets classic thiab caj ces. Cov rickets ib txwm tshwm sim nyob rau thawj lub hlis ntawm lub neej, kev mob ntshav qab zib muaj mob ntshav qab zib - pib thaum ib nrab xyoo, thiab qee zaum hauv 1.5-2 xyoo, tsuas yog tom qab tus menyuam pib taug kev. Hauv qhov no, cov ceg qis (ntev tubular pob txha), lub hauv caug thiab pob taws pob qij txha yog khoov siab feem ntau.

Kev kuaj ntshav kho kom haum rau kev tshawb nrhiav kab mob biochemistry thiab xoo hluav taws xob pab. Kev txiav txim siab biochemical ntawm cov ntshav thiab cov qauv ntawm cov pob txha nqaij hauv cov kab mob muaj keeb tiv thaiv kab mob sib txawv ntawm ob qho tib si ib txwm thiab cov kev cuam tshuam uas pom tau nrog cov rickets classical. Raws li txoj cai, thaum muaj phosphate xav tias yuav muaj ntshav qab zib hauv menyuam yaus, cov kws kho mob pom zoo kom kuaj ob leeg niam txiv thiab cov txheeb ze.

Puas yog nws tuaj yeem kho kho tsev neeg mob rickets?

Lub hauv paus ntsiab lus ntawm kev kho mob ntawm tsev neeg thiab tsev neeg hypophosphatemic rickets yog qhov qub - kev qhia ntawm cov vitamin D. Tab sis vim tias kev ua tiav ntawm tsev neeg ntawm rickets muaj qhov tsis taus, cov vitamins xav tau ntau zaus ntau dua li ntawm thawj kis. Tib lub sijhawm, cov kws kho mob yuav tsum soj ntsuam cov qib phosphorus thiab calcium hauv cov ntshav thiab nws lwm yam kev hloov biochemical, tus kheej xaiv cov tshuaj kom raug.

Kev kho mob Vitamin yog ntxiv nrog kev npaj phosphorus (calcium glycerophosphate) thiab kev noj haus, thiab nyob ntawm lub hnub nyoog, cov menyuam yaus tau sau cov tshuaj vitamin ntau, piv txwv li, Oxidevit, thiab phosphorus cov zaub mov muaj txiaj ntsig tau ntxiv rau lawv cov zaub mov noj.

Txij li cov mob ntshav qab zib phosphate zoo ib yam li rickets ntawm hom 1, thiab qee zaus nws tsis tuaj yeem tsim kev kuaj mob tam sim ntawd, cov kws kho mob qhia cov niam txiv ntawm cov menyuam hnub nyoog qis dua ob txog peb xyoos kom kuaj xyuas tag nrho ib lub sijhawm. Muaj qee kis, kev kho tshuaj tsis txaus, thiab tom qab ntawd cov pob txha ua kom ncaj yog ncaj los ntawm kev phais mob. Tab sis puas yog tus kabmob nws tus kheej ploj tom qab kev kho mob?

Ib yam li mob ntshav qab zib 1 nws tsis muaj peev xwm los rov qab tsim cov tshuaj koj tus kheej cov insulin, nws tsis yooj yim sua los kho "" cov teeb meem metabolic hauv ntshav qab zib mellitus. Tab sis rau cov neeg laus, cov nyiaj calcium thiab phosphorus uas tseem nqus lub cev yog qhov txaus.

Yog li, tom qab tiav hluas, tus kabmob no cuam tshuam tsuas yog nyob rau lub sijhawm ntau ntxiv ntawm kev xav tau calcium thiab phosphorus - hauv cov pojniam thaum cev xeeb tub thiab lactation.

Tab sis qhov "tsis paub" raug txim ntawm rickets - luv stature, curvature ntawm ob txhais ceg - ob leeg nyob rau hauv poj niam thiab txiv neej nyob twj ywm rau lub neej. Cov poj niam vim kev kho mob ntawm pob txha hu ua pelvic qee zaum yuav tsum tau ua kev phais plab.

Cov tsos mob ntawm ntshav qab zib Phosphate

Tus kab mob no yog qhov tshwm sim ntawm kev hloov pauv hauv X lub cev, uas cuam tshuam kev nqus ntawm phosphates hauv cov hlab ntsha, thiab tom qab ntawd ua rau cov txheej txheem 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 txivneej (thiab cov tub thiab cov ntxhais) sib npaug.

Tus kws kho mob pediatrician yuav pab kho tus mob

Kev Kho Mob Ntshav Qab Zib Phosphate

Qhia tshuaj kom ntau ntau ntawm cov vitamin D (qee zaum rau lub neej).
Phosphorus, calcium, vitamins A thiab E.
Yog tias muaj pob txha tsis muaj zog (lossis nruab nrab) kev hloov kho pob txha pob txha, kev kho pob txha kho yog raug kho (piv txwv, kev kho ntawm curvature ntawm tus nqaj qaum los ntawm hnav tshwj xeeb orthopedic corsets).
Kev phais mob yog qhov tsim nyog tsuas yog nrog kev ua haujlwm tsis zoo ntawm lub cev pob txha. Siv nws tom qab qhov kawg ntawm lub sijhawm loj hlob.

Teeb meem thiab txim

Ua txhaum ntawm kev nyob ntawm lub cev thiab deformation ntawm lub cev pob txha tom qab phosphate mob ntshav qab zib raug kev txom nyem hauv menyuam yaus yog khaws cia rau lub neej.
Menyuam yaus poob qis hauv kev xav thiab lub cev.
Kev nce qib ntawm pob txha thiab pob qij txha yaig hauv qhov tsis muaj kev kho kom zoo txaus tuaj yeem ua rau muaj qhov tsis taus.
Kev ua txhaum ntawm kev txhim kho hniav (pathology ntawm cov qauv ntawm txha hniav laus, ua txhaum ntawm lub sijhawm thiab kev txiav txim ntawm teething, thiab lwm yam).
Hnov tsis hnov lus (vim qhov tshwm sim ntawm kev txhim kho tsis hnov lus ntawm lub pob ntseg ntawm pob ntseg hauv nruab nrab).
Qhov tshwm sim ntawm tus kabmob tuaj yeem yog nephrocalcinosis (kev tso tshuaj calcium ntsev hauv lub raum), uas tuaj yeem ua rau lub raum tsis ua haujlwm.
Ib tug kab mob raug mob los ntawm ib tug ntxhais hauv thaum yau tuaj yeem ua rau mob lub pob txha pob txha, ua rau txoj hauv kev nyuaj ntawm kev tawm dag zog yog qhov yuav tshwm sim. Nyob rau theem ntawm kev npaj cev xeeb tub, cov poj niam uas tau ntsib phosphate ntshav qab zib hauv menyuam yaus yuav tsum sab laj nrog tus kws tshaj lij txog cov kev phais plab menyuam.

Kev Tiv Thaiv Mob Ntshav Qab Zib Phosphate

Kev kuaj pom tus kab mob thaum ntxov (xa mus rau tus kws kho mob tshwj xeeb ntawm thawj tus cim ntawm tus kab mob rau lub hom phiaj kev kuaj mob thaum ntxov thiab kev teem sijhawm kho mob kom raws sijhawm: lub hom phiaj tseem ceeb ntawm cov kev ntsuas no yog txo qis kev pheej hmoo thiab tshwm sim).
Kev tu ncua sijhawm thiab kev saib xyuas cov menyuam tas mus li uas muaj cov kab mob zoo ib yam los ntawm tus kws kho menyuam yaus thiab tus kws kho keeb.
Cov kev tawm tswv yim kho mob thiab caj ces rau cov tsev neeg muaj mob ntshav qab zib phosphate (qhov twg yog ib tus hauv tsev neeg raug tus kab mob no thaum tseem yau) nyob rau theem ntawm kev xeeb tub.Lub hom phiaj ntawm qhov kev tshwm sim no yog los piav qhia rau cov niam txiv txog kev muaj menyuam muaj mob thiab qhia txog cov kev pheej hmoo, muaj tshwm sim, cov kev mob nyuaj rau tus kabmob no hauv tus menyuam.

Yeem

Nws paub tias ib qho ntawm cov tshuaj lom neeg tseem ceeb ntawm cov pob txha pob txha yog calcium. Lub hauv paus ntawm cov pob txha nqaij nrog rau calcium yog tseem phosphorus. Tus neeg noj cov khoom noj nrog cov khoom noj.
Ib qho txaus ntawm phosphates (phosphorus compounds) hauv lub cev yog ib qho tsim nyog rau kev nqus ntawm calcium los ntawm cov pob txha nqaij.
Raws li kev hloov pauv ntawm ib qho ntawm X chromosome noob, phosphate nqus nyob rau hauv lub raum txoj hlab ntsha yog qhov tsis taus, thiab tom qab ntawd cov saw hlau ntawm cov txheej txheem pathological uas cuam tshuam ib txwm nqus ntawm calcium los ntawm cov pob txha nqaij tau pib.

COV LUS QHIA PIV TXWV

Kev sab laj nrog kws kho mob yog qhov yuav tsum tau

Qhuab qhia ntawm biochemistry ntawm KSMA, 2004
Volkov M.V., Dedova V.D. Cov Me Nyuam Orthopedics-Tshuaj, 1980

Pathogenesis ntawm tus kab mob

Tus kab mob tshwm sim los ntawm kev sib kis los ntawm hom hom, uas yog nyob ntawm poj niam txiv neej. Nrog rau tus kabmob, cov txheej txheem enzymatic cuam tshuam, uas hloov vitamin D rau hauv cov tshuaj yeeb dej caw.

Phosphate mob ntshav qab zib muaj kev loj hlob vim yog qhov mob puas tsuaj rau lub raum plab hlaus, uas tau koom nrog phosphate reabsorption. Nrog qhov siab dhau feem pua ntawm phosphorus excretion ua ke nrog zis, nws cov nyiaj hauv cov ntshav ntawm tus neeg txo qis, uas ua rau cov pob txha deformities.

Lub hauv paus tseem ceeb ntawm qhov pib ntawm tus kabmob yog hloov mus rau ntawm X chromosome, uas cuam tshuam kev nqus ntawm phosphates thiab ua rau muaj ntau yam ntawm cov txheej txheem pathological uas cuam tshuam cov txheej txheem ntawm calcium nqus.

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 nraub qaum, thaum palpating, mob tshwm sim.

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 zog txav tuaj yeem taug qab.

Cov kws kho mob tuaj yeem ua tus kab mob hauv thawj ob xyoos ntawm lub neej, txij li tus kab mob pathology pib thaum hnub nyoog me me vim yog qhov muaj kev xav ntawm caj ces. Raws li kev tsim kho kev txawj ntse, hauv qhov no phosphate ntshav qab zib tsis muaj kev cuam tshuam, txawm li cas los xij, nws tseem cuam tshuam rau lub hlwb kev xav thiab nthuav qhia nws tus kheej hauv qhov tseeb tias tus menyuam nkag siab nws qhov tsis sib xws nrog kev phooj ywg, kev txwv ntawm lub cev.

Hom mob hypophosphatemic rickets (mob ntshav qab zib phosphate)

Cov kws kho mob faib cov kabmob no ua 4 hom loj:

Hom 1 tshwm sim nyob rau xyoo 2 ntawm tib neeg lub neej. Cov tsos mob tseem ceeb: kev loj hlob stunted, tsis muaj hypoplasia ntawm txha hniav laus ntawm tus hniav mus tas li, ceg deformities, rickets-zoo li hloov hauv cov pob txha. Nrog hom kabmob no, phosphate reabsorption poob qis thiab rickets-xws li kev hloov pauv.
Hom 2 yog autosomal tseem ceeb, uas tsis txuas nrog X chromosome. Yuav tshwm sim rau thawj xyoo lossis xyoo ob ntawm lub neej. Nws ua tau nws tus kheej hauv daim ntawv ntawm curvature ntawm qis qis, hloov ntawm lub cev pob txha. Hauv qhov no, qhov kev loj hlob hloov pauv tsis hloov, ib tus neeg muaj lub cev ncaj ncees uas muaj zog. Muaj cov tsos mob me me ntawm rickets.
Hom 3 nthuav qhia nws tus kheej hauv kev ua tsis tau ntawm kev nqus ntawm calcium, uas ua rau lub fact tias twb nyob rau 6 lub hlis ntawm lub neej, tus menyuam muaj cramps, hypotension, luv stature, leeg tsis muaj zog thiab rickets hloov hauv kev loj hlob.
Hom 4 tshwm sim feem ntau yog hauv tus poj niam thaum menyuam yaus. Nws ua tau nws tus kheej hauv daim ntawv ntawm curvature ntawm qis qis, hniav txawv txav thiab rickets nyob rau hauv sib txawv qib.

Cov kev mob tshwm sim los ntawm tus kab mob

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 lus.
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.

Cov lus pom zoo soj ntsuam

Ntshav qab zib phosphate yog ib qho kab mob txaus ntshai uas tshwm sim vim kev mob caj ces, tshwm nyob rau thaum yau thiab tuaj yeem ua rau muaj kev phom sij txaus ntshai.

Raws li cov lus pom zoo hais txog kev tiv thaiv tus kabmob, nws muaj peev xwm cais tawm txoj kev txhim kho ntawm qhov mob no tsuas yog ua tib zoo saib xyuas cov tsev neeg hluas, yog tias muaj teeb meem zoo li no hauv tsev neeg thiab hauv kev tshuaj ntsuam genetic predisposition.

Ua ntej yuav xeeb tub, tus kws kho mob yuav sau ntawv kuaj tag nrho, tom qab ntawd nws tuaj yeem tsim kom muaj peev xwm ntawm tus menyuam muaj hnub nyoog noj qab haus huv. Koj tuaj yeem sim tshem tawm txoj kev loj hlob ntawm phosphate mob ntshav qab zib nrog lub sijhawm mus ntsib kws tshaj lij yog tias nws cov cim pib tshwm sim hauv menyuam yaus txij li hnub nyoog me.

Txhawm rau tiv thaiv txoj kev loj hlob ntawm tus kab mob txaus ntshai hauv menyuam yaus, nws yog ib qho tsim nyog yuav tsum ua raws nraim li cov lus pom zoo ntawm cov kws tshaj lij thiab tiv tauj lub chaw kho mob kom raws sijhawm kom paub thiab kho. Lub sijhawm thaum ib tug neeg laus them sai sai rau qhov hloov pauv ntawm tus menyuam txoj kev loj hlob ib txwm muaj, ntau yam muaj peev xwm ntawm qhov pom ntawm qhov ua tau ntawm kev tshem tawm ntau qhov teebmeem ntxiv.

Raws li cov ntaub ntawv raug cai, muaj tseeb tiag, 52% ntawm lub teb chaws cov neeg raug kuaj ntshav qab zib. Tab sis tsis ntev los no, ntau thiab ntau tus neeg tig mus rau kev kho plawv thiab endocrinologist nrog cov teeb meem no.

Mob ntshav qab zib kuj tseem tuaj yeem ua rau kev loj hlob ntawm qog ntshav. Ib txoj kev los sis lwm qhov, qhov tshwm sim ntawm txhua qhov teeb meem zoo ib yam - cov mob ntshav qab zib yog tuag, tawm tsam nrog ib tus mob hnyav, lossis hloov mus rau tus neeg tsis taus tiag tiag, tau txais kev txhawb nqa nrog kev kho mob nkaus xwb.

Kuv yuav teb cov lus nug nrog ib lo lus nug - dab tsi tuaj yeem ua haujlwm hauv qhov xwm txheej no? Peb tsis muaj kev qhia tshwj xeeb los tua tshwj xeeb cov ntshav qab zib, yog tias koj tham txog nws. Thiab hauv cov chaw kho mob tam sim no nws tsis yog tuaj yeem pom tus kws kho mob endocrinologist txhua lub sijhawm, tsis yog hais txog kev nrhiav tus kws kho mob endocrinologist uas tsim nyog lossis tus kws kho ntshav qab zib uas yuav muab kev pabcuam zoo rau koj.

Peb tau tso cai nkag mus rau thawj cov tshuaj tsim los ua ib feem ntawm txoj haujlwm hauv ntiaj teb no. Nws qhov tsis txawv txav tso cai rau koj maj mam nqa tawm cov tshuaj uas tsim nyog rau hauv cov ntshav hauv lub cev, nkag mus rau cov hlab ntshav ntawm daim tawv nqaij. Kev nqus ntshav mus rau hauv cov ntshav ncig muab cov tshuaj uas tsim nyog nyob rau hauv cov hlab ntshav, uas ua rau cov suab thaj txo qis.