Phosphate mob ntshav qab zib hauv cov menyuam yaus: ua rau thiab cov tsos mob

Mob Ntshav Qab Zib - kev ua kom caj los ntawm ua txhaum ntawm cov pob zeb hauv av, uas kev nqus thiab assimilation ntawm phosphorus tebchaw hauv lub cev raug mob, uas ua rau pathology ntawm cov pob txha pob txha. Raws li cov ntaub ntawv zaum kawg, nws yog ib pawg ua tiav ntawm cov kabmob muaj kabmob. Nws yog pom los ntawm cov leeg mob hauv lub cev, rickets ntawm lub pob txha (varus deformities ntawm cov pob txha ntawm qhov qis qis, rickets thiab lwm tus), kev loj hlob qeeb. Kev kuaj mob ntshav qab zib phosphate yog raws li kev soj ntsuam hauv chaw kuaj ntshav thiab zis (qib ntawm alkaline phosphatase, calcium ions, active form of vitamin D) thiab cov tshuaj molecular ntsuam xyuas. Kev kho tus kab mob no yog nqa tawm los ntawm kev muaj peev xwm ntawm ntau cov vitamin D, phosphorus thiab calcium calcium, orthopedic lossis phais kho cov pob txha tsis zoo.

Cov ntaub ntawv dav dav

Phosphate mob ntshav qab zib (vitamin D-resistant rickets) yog lub npe sib koom ua ke rau ntau txoj kev ua tiav tubulopathies (pathological cuam tshuam ntawm kev thauj khoom hauv cov tubules ntawm lub raum), nyob rau hauv uas reabsorption ntawm phosphate ions raug cuam tshuam nrog kev loj hlob ntawm lawv qhov tsis txaus hauv lub cev. Ib qho ntawm tsev neeg cov ntaub ntawv tshaj plaws ntawm tus kabmob no, kis los ntawm cov tshuab tseem ceeb uas txuas nrog X tus qauv hluav taws xob, tau piav qhia rov qab xyoo 1937. Hauv xyoo tom ntej, cov kws kho mob caj ces tau qhia ob peb hom phosphate muaj ntshav qab zib sib txawv nrog cov tshuaj sib txawv, kev sib kis thiab cov duab hauv chaw kuaj mob. Txawm li cas los xij, txhua tus ntawm lawv muaj cov yam ntxwv zoo - lawv tshwm sim los ntawm kev tsis tuaj yeem nqus tau phosphorus hauv lub raum, muaj cov yam ntxwv zoo li rickets thiab muaj kev cuam tshuam me ntsis ntawm kev siv li niaj hnub noj tshuaj vitamin D. Txog tam sim no, cov tsev neeg cov ntshav qab zib phosphate tau txheeb xyuas, qhov kis tau tus mob uas txuas nrog X chromosome ( ob qho tag nrho thiab kev cuam tshuam), autosomal tseem ceeb thiab autosomal recessive. Qhov dav dav ntawm cov hom muaj ntau ntawm tus mob no yog 1:20 000 (X-txuas kev sib txuas hauv daim ntawv), lwm hom muaj ntau tsawg dua.

Cov tshwm sim thiab faib tawm phosphate muaj ntshav qab zib

Txawm hais tias cov lus tshaj tawm heterogeneity ntawm phosphate mob ntshav qab zib, qhov tshwm sim tam sim ntawd ntawm hypophosphatemia nyob rau hauv cov mob sib txawv ntawm cov kab mob yog tib yam - ib qho kev ua txhaum ntawm thim rov qab nqus (reabsorption) ntawm phosphates nyob rau hauv cov hlab ntaws ntawm lub raum. Qhov no tso cai rau koj tus cwj pwm tus mob no rau tubulopathies lossis pathologies ntawm lub chaw tso zis, txawm li cas los xij, thaum nws tshwm sim, tag nrho lub cev thiab tshwj xeeb tshaj yog cov leeg mus rau lub cev. Tsis tas li ntawd, qee cov qauv ntawm cov ntshav qab zib phosphate yog nrog los ntawm qhov tsis txaus siab ntawm calcium nyob rau hauv cov hnyuv thiab lub raum, kev txhim kho ntawm urolithiasis, kev ua haujlwm tsis zoo ntawm cov qog parathyroid. Muaj qhov tseeb kev sib txheeb ntawm cov caj ces thiab chaw soj ntsuam ntau yam ntawm tus kab mob, uas tso cai rau peb los ua kom pom tseeb, feem ntau lees paub kev faib tawm uas suav nrog 5 hom qauv ntawm pathology.

X-txuas rau cov kab mob ntshav qab zib zoo tshaj plaws - yog cov teeb meem tshwm sim feem ntau ntawm cov kab mob no, vim hloov los ntawm PHEX cov noob. Nws encodes ib qho enzyme hu ua endopeptidase, uas tswj cov kev ua ntawm cov ion txuas ntawm lub raum thiab cov hnyuv me. Raws li qhov tshwm sim caj ces, qhov enzyme uas tau txais tsis tuaj yeem ua nws lub luag haujlwm, yog li ntawd, kev thauj mus los ntawm phosphate ions los ntawm lub cell cell hauv cov kabmob sab saud yog ua rau qeeb qeeb. Qhov no ua rau muaj kev nce ntxiv hauv qhov kev poob ntawm phosphate ions hauv cov zis thiab qhov nyuaj ntawm lawv qhov nqus mus rau txoj hnyuv, vim qhov hypophosphatemia tshwm sim hauv cov ntshav, thiab rickets-xws li kev hloov tshwm sim hauv cov pob txha nqaij vim qhov tsis muaj cov ntxhia.

X-txuas tshuaj tiv thaiv kab mob ntshav qab zib - Tsis zoo li tus qauv dhau los, nws cuam tshuam rau cov txiv neej nkaus xwb, thaum poj niam tsuas tuaj yeem ua raws li tus nqa khoom ntawm cov noob pathological. Qhov ua rau tus mob hom mob no yog kev hloov pauv ntawm cov noob CLCN5, uas encodes protein-chlorine ion channel ib ntu zuj zus. Raws li qhov tshwm sim caj ces, kev thauj khoom ntawm txhua ions (suav nrog phosphates) los ntawm cov qog ntawm nephron epithelial hlwb chim siab, vim qhov twg phosphate ntshav qab zib loj hlob tuaj.

Autosomal Dominant Phosphate Mob Ntshav Qab Zib - ib daim ntawv ntawm cov kabmob los ntawm kev sib hloov ntawm FGF23 noob nyob rau ntawm 12 lub chromosome. Cov khoom ntawm nws qhov kev tshwm sim yog cov protein uas tau ua tsis ncaj ncees lawm hu ua fibroblast-23 qhov kev loj hlob, txawm hais tias nws yog ib qho tseem ceeb zais los ntawm osteoblasts thiab ua kom nrawm rau kev nthuav tawm ntawm phosphate ions hauv qhov zis. Cov ntshav qab zib phosphate muaj kev hloov pauv nrog FGF23 kev hloov pauv, qhov txiaj ntsig ntawm cov protein nws tsim tawm los ua cov tshuaj tiv thaiv cov ntshav, vim tias nws nthuav tawm thiab, raws li, cov nyhuv cuam tshuam nrog kev loj hlob ntawm hypophosphatemia. Hom kab mob no xam tias yog mob ntshav qab zib me ntsis ntawm phosphate.

Autosomal kho tsis zoo phosphate mob ntshav qab zib Yog ib hom kab mob uas tsis tshua muaj tshwm sim los ntawm kev hloov pauv hauv DMP1 gene nyob rau ntawm 4 lub chromosome. Lub gene encodes ib acidic matrix dentine phosphoprotein, feem ntau tsim nyob rau hauv dentin thiab pob txha nqaij, qhov uas nws tswj lawv txoj kev loj hlob. Lub pathogenesis ntawm phosphate mob ntshav qab zib hauv hom kev hloov pauv no tsis tau ua tib zoo kawm.

Autosomal recessive phosphate mob ntshav qab zib nrog hypercalciuria - kuj tsis tshua muaj sib txawv ntawm tus kab mob no, tshwm sim los ntawm kev hloov pauv ntawm SLC34A3 lub cev nyob rau ntawm 9 chromosome. Nws hloov cov kab ke ntawm cov sodium-dependent channel ntawm phosphate ions hauv ob lub raum thiab, nrog kev tsis sib haum xeeb hauv tus qauv, ua rau muaj qhov nce ntxiv ntawm qhov tsis pom kev ntawm calcium thiab phosphorus hauv cov zis nrog tib pliag ntshav plasma.

Nws tseem muaj cov qauv ntawm cov ntshav qab zib phosphate, nrog rau hyperparathyroidism, urolithiasis thiab lwm yam kev cuam tshuam. Qee yam kabmob ntawm cov kabmob no cuam tshuam nrog cov caj ces xws li ENPP1, SLC34A1 thiab qee yam. Kev tshawb xyuas ntawm txhua qhov tshwm sim ntawm phosphate mob ntshav qab zib tseem niaj hnub ua.

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 cuam tshuam txog kev mob loj heev. Qee qhov xwm txheej ntawm pathology (piv txwv li, vim kev hloov pauv hauv FGF23 noob neej) tuaj yeem pom tsuas yog los ntawm 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.

Kev mob ntshav siab thaum tseem yau yog ib qho pom thawj zaug ntawm phosphate mob ntshav qab zib, tab sis nws tsis pom nyob hauv txhua kis. Feem ntau, kev txhim kho kabmob pib nrog O-puab deformation ntawm ob txhais ceg, uas tuaj yeem ua rau lub cev tsis zoo. Nrog rau cov chav kawm txuas ntxiv ntawm phosphate mob ntshav qab zib, lwm qhov kev kho mob ntawm cov rickets tuaj yeem tshwm sim - kev loj hlob ntawm lub zog rov qab thiab kev txhim kho lub cev, ua kom cov hniav tsis muaj zog (tshwj xeeb tshaj yog nrog autosomal recessive daim ntawv ntawm tus kabmob), alopecia. Pathological fractures, cov tsos ntawm rickets "rosary", ib qho tuab ntawm metaphyses ntawm cov pob txha ntawm cov nqaj yog cov yam ntxwv. Tsis tas li, nrog phosphate mob ntshav qab zib, mob nraub qaum (feem ntau ntawm neurological) thiab cov pob txha tuaj yeem pom, muaj qee qhov xwm txheej, vim mob mob ntawm txhais ceg, tus me nyuam tsis muaj txoj hauv kev taug kev. Kev txawj ntse hauv lub cev ntawm tus kab mob no, raws li txoj cai, tsis muaj sau tseg.

Kev kuaj mob ntshav qab zib phosphate

Ib txoj hauv kev ntxov tshaj plaws rau kev kuaj ntshav qab zib phosphate yog kev tshuaj xyuas dav dav ntawm tus menyuam muaj mob thiab kawm txog kev hloov tshuaj tiv thaiv tus kab mob mus rau kev siv cov tshuaj vitamin d li niaj zaus, raws li txoj cai, nrog cov kab mob no muaj kev saib xyuas cov kab mob rickets nrog kev siv tshuaj ntawm cov tshuaj no (cov roj ntses, cov roj tov) Cov. Rau qhov kev txiav txim raug tseeb dua ntawm phosphate ntshav qab zib siv cov hau kev ntawm kev tshawb fawb biochemical ntawm cov ntshav thiab zis, kev tshawb nrhiav xoo hluav taws xob, tshuaj ntsuam cov roj ntsha molecular. Qhov kev mob tshwm sim tsis tu ncua ntawm tus kab mob no yog hypophosphatemia lossis txo qis ntawm phosphate ions hauv cov ntshav ntshav, uas tau txiav txim siab yog ib feem ntawm kev tshawb xyuas biochemical. Nyob rau tib lub sijhawm, qib calcium uas tuaj yeem yog ib txwm muaj zog lossis nce ntxiv, txawm li cas los xij, qee hom kev phosphate mob ntshav qab zib (vim kev hloov pauv hauv SLC34A3 noob) kuj tseem ua los ntawm hypocalcemia. Tsis tas li ntawd, nrog phosphate mob ntshav qab zib, kev nce ntxiv hauv theem ntawm alkaline phosphatase thiab qee zaum kev nce qib ntawm parathyroid cov tshuaj hormones tuaj yeem tshwm sim. Ib txoj kev kuaj zis biochemical tso tawm kom pom qhov muaj feem ntawm phosphorus (hyperphosphaturia) thiab, nyob rau qee kis, hypercalciuria.

Kev tshawb nrhiav radiological ntawm cov ntshav qab zib phosphate txiav txim cov tsos mob ntawm rickets - deformation ntawm cov pob txha ntawm ob txhais ceg, hauv caug thiab lub duav, qhov muaj osteoporosis (qee qhov teeb meem, osteosclerosis hauv zos yuav tshwm sim) thiab osteomalacia. Tus qauv ntawm cov pob txha tau hloov - cortical txheej tuab, cov qauv trabecular ua coarser, lub diaphysis nthuav dav. Feem ntau, pob txha xoo hluav taws xob hnub nyoog nrog phosphate ntshav qab zib yog qhov qab qab qhov tseeb, uas qhia txog kev ncua ntawm kev txhim kho pob txha. Cov noob caj noob nyoog niaj hnub tso cai rau koj los tshawb nrhiav yuav luag txhua hom kabmob no, raws li txoj cai, txoj hauv kev ntawm kev hloov ncaj qha ntawm cov noob muaj feem nrog pathology yog siv. Muaj qee kis, keeb kwm caj ces ntawm tus neeg mob tuaj yeem qhia txog caj ces ntawm phosphate mob ntshav qab zib.

Kev Kho Mob Ntshav Qab Zib Phosphate

Mob ntshav qab zib yog kho nrog kev sib xyaw ua ke ntawm kev kho vitamin, orthopedic thiab qee zaum kev phais mob. Txawm hais tias muaj lwm lub npe rau cov kab mob pathology no (vitamin D-resistant rickets), cov vitamin no tau siv nyob rau hauv kev kho mob ntawm qhov mob no, tab sis cov tshuaj noj yuav tsum tau nce ntau. Tsis tas li ntawd, cov neeg mob uas muaj phosphate ntshav qab zib tau tsim tshuaj calcium thiab phosphorus npaj, cov vitamins A, E thiab pab pawg B. Nws yog ib qho tseem ceeb tias kev kho mob nrog cov roj-soluble vitamins (tshwj xeeb tshaj yog D thiab A) yuav tsum tau ua tshwj xeeb nyob rau hauv kev saib xyuas ntawm tus kws kho mob thiab nrog ceev faj soj ntsuam ntawm cov tshuaj los tiv thaiv cov kev xav tsis zoo thiab lwm yam nyuaj. Txhawm rau soj ntsuam cov txiaj ntsig ntawm txoj kev kho thiab qhov tseeb ntawm cov koob tshuaj uas tau teev tseg, ib qho kev ntsuas ib txwm ntawm cov zis phosphate thiab cov calcium uas tau ua. Hauv cov ntawv tshwj xeeb tshaj plaws ntawm cov ntshav qab zib phosphate, kev siv cov vitamin D yuav tau ua pov thawj rau lub neej.

Hauv kev kuaj mob thaum ntxov ntawm tus kab mob no, nws txoj kev kho mob suav nrog kev tiv thaiv pob txha pob txha los ntawm feem ntau lees txais orthopedic cov tswv yim - hnav cov ntaub qhwv rau txha nraub qaum. Nrog rau kev kuaj mob tom qab ntawm phosphate mob ntshav qab zib nrog cov pob txha tsis zoo, kev kho mob kho tau qhia. Cov ntaub ntawv asymptomatic ntawm tus kab mob no, tsuas yog los ntawm hypophosphatemia thiab hyperphosphaturia, raws li cov kws tshaj lij, tsis xav tau kev kho mob hnyav. Txawm li cas los xij, ua tib zoo saib xyuas cov mob ntawm pob txha pob txha, mob pob txha, thiab lub raum (tiv thaiv urolithiasis) yog qhov yuav tsum tau ua, uas yog ua los ntawm kev kuaj mob niaj hnub los ntawm tus kws kho mob endocrinologist.

Kev kwv yees thiab kev tiv thaiv kab mob ntshav qab zib phosphate

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. Cim cov pob txha tsis zoo uas yog los ntawm kev kuaj mob lig lossis kho kho tsis zoo ntawm phosphate ntshav qab zib tuaj yeem ua rau tus neeg mob lub neej tsis zoo. Kev tiv thaiv tus kab mob no muaj peev xwm ua tau tsuas yog kev kho mob ntawm kev sib tham ntawm kev kho mob thiab caj ces ntawm cov niam txiv ua ntej xeeb menyuam, rau qee hom kev qhia ntawm kev kuaj mob ua ntej tau tsim.

Ua rau thiab muaj ntau yam phosphate mob ntshav qab zib

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.

X-txuas hypophosphatemic rickets. Qhov no yog ib qho ntawm cov kabmob uas nyiam rickets, nws qhov ntau zaus yog 1: 20,000 ntawm tus menyuam ntawm cov menyuam. Qhov ua rau cov kab mob pathology no suav tias yog kev hloov pauv hauv PHEX gene encoding kev ua ub no ntawm endopeptidase enzyme koom nrog kev ua kom thiab tshem tawm ntawm ntau yam tshuaj peptide. Hauv qhov no, qhov tsis txaus ntawm cov protein tshwm sim, thauj cov phosphorus sib txuas hauv cov tub ntxhais ntawm lub raum (cov qauv ntawm lub raum) thiab cov hnyuv, uas ua rau cov phosphorus ions poob rau hauv cov zis thiab qhov tsis haum hauv lub plab zom mov. Yog li, phosphorus-calcium metabolism cuam tshuam hauv lub cev, thiab ntau yam kab mob pathological cuam tshuam nrog qhov no tshwm sim. Cov chav kawm ntawm tus kab mob no raug kev txom nyem los ntawm kev ua tsis tau zoo ntawm cov metabolism hauv metabolism hauv hepatocytes (mob hlwb) thiab muaj kev zais ntau dhau los ntawm cov qog ntshav parathyroid los ntawm parathyroid hormone.
Autosomal tseem ceeb hypophosphatemic rickets. Daim ntawv ntawm tus kab mob no tsis tshua zoo li yav dhau los thiab tsis muaj txoj kev mob hnyav. Nws cuam tshuam nrog kev hloov pauv ntawm FGF-23 noob, karyotyped ntawm chromosome 12. Lub noob no yog qhov chaw ncig mus los uas yog tsim los ntawm osteocytes (pob txha hlwb) txhawm rau txhawm rau txhaws lub raum reabsorption (rov nqus los ntawm cov zis) ntawm phosphates. Nrog rau kev nce ntxiv hauv nws cov kev ua hauv cov ntshav, hypophosphatemia yog pom.
Autosomal recessive hypophosphatemic rickets. Qhov sib txawv ntawm phosphate mob ntshav qab zib no yog vim muaj kev hloov pauv hauv DMP1 gene, uas yog lub luag haujlwm rau cov synthesis ntawm cov pob txha protein tshwj xeeb uas tau koom nrog kev tswj hwm ntawm kev ua kom loj hlob ntawm undifferentiated osteoblasts (lub hlwb tsis paub qab hau). Nws kuj tseem ua rau kom plam ntawm phosphorus hauv cov zis ntawm qhov kev ib txwm xav ntawm parathyroid hormone thiab calcitriol.
Kab mob hypophosphatemic rickets nrog hypercalciuria. Qhov no yog qhov tsis tshua muaj pathology vim muaj kev hloov pauv ntawm SLC34A3 gene, uas sau cov kev ua si ntawm sodium phosphate cotransporters, uas muab cov khoom thauj transmembrane ntawm cov tshuaj hauv cov hlab hlaus thiab phosphate homeostasis. Nws yog tus cwj pwm los ntawm kev ploj ntawm phosphorus thiab calcium nyob hauv cov zis, nce ntxiv hauv kev ua ntawm calcitriol thiab kev txhim kho rickets.

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 deformities (varus deformity ntawm qis dua qhov kawg, "rickets" ntawm tus tav, thickening ntawm distal tubular pob txha ntawm forearm, deformation ntawm lub pob txha taub hau),
hloov pauv hauv tus me nyuam lub plab (nco qab duck)
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 tsis tuaj yeem nyob hauv phosphate mob ntshav qab zib.

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

Kev kuaj mob

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 mob lub cev thiab tshuaj xyuas. 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 pob txha hloov, txha pob txha).

Qee zaum hauv qhov pib ntawm tus kab mob, cov neeg mob no tau hais tias mob rickets thiab kev noj tshuaj yog muaj cov tshuaj 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 tshuaj thiab kev sim 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.

Tus kws kho mob twg yuav hu rau

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.