Osteopetrosis (inouraya machena). Osteopetrosis - chii?

Osteopetrosis (machena kana chirwere) - zvakakomba vachiberekwa skeletal chirwere, chemafuta akawandisa Ossificação em (local kana generalized). Kazhinji anozivikanwa zviitiko, zvokurwara nhaka. Very Kashoma hosha kunoitika mumhuri ine utano.

Zvokurwara yakatanga kudzidza uye yakarondedzerwa chiremba wokuGermany Albers-Schoenberg muna 1904. In mabhuku machena osteopetrosis izvozvo inonziwo vachiberekwa systemic osteosclerosis. In vana, chirwere ndiye anenge pakarepo pashure pokuberekwa. Hosha rinowanikwa kwazvo chimiro, pane zviitiko kufa. Pane rimwe zvishoma zvikuru osteopetrosis akatsunga chete vakura uye kazhinji kurongwa kuwanikwa musi X-ray nokuda mumwe musoro pfupa.

zvinokonzera

Kwemakore akawanda, masayendisiti vasingakwanisi kusvika nekubvirana pasi etiology chirwere Albers-Schonberg. Zvinofungidzirwa kuti muna imwe nhengo pathological urongwa vari atanga, unogona kudzivisa Absorção de Água calcium phosphorus. Zvakakomba metabolic zvirwere zvicherwa kukonzera chirwere inonzi osteopetrosis. Chii uye nei kusashanda ari mashandiro nokuparadzwa remafupa rinenge hazvibviri kuziva.

Kazhinji zvinoita kuronda pakutsanangura nhaka chirwere. Osteopetrosis ( "dzinouraya dzemabhura") hunogona kutapukira ive autosomal rave uye autosomal recessive. Muchiitiko chokutanga kuti zvokurwara iri dzakaonekwa mune vakuru zera uye zviri nyore. Kana recessive unhu kuchinjirwa Albers-Schonberg chirwere chiri chirwere achangoberekwa uye anotora nzvimbo ine zvakawanda yematambudziko.

Sei zvokurwara?

The pathogenesis pamusoro osteopetrosis insufficiently anodzidzwa. Zvinofungirwa kuti kunyange utero kwakakanganiswa kwomuzvarirwo kusakarongeka mapfupa uye hematopoietic tishu. Osteoclasts - masero anokonzera pokuparadzwa marangwanda - kwete kuzadzikisa ayo ushande. Somugumisiro, kuchinja mapfupa mamiriro, vane akaremara uye vasisiri kukurira basa. Uyezve, yekudyidzana anetseka ropa wedzerai, innervation marangwanda. Kakawanda kunokanganisa refu tubular mapfupa, dehenya, chiuno nomuzongoza.

kupatsanura pakati osteopetrosis

• Autosomal recessive chimiro (yakatanga pakuzvarwa, kunoitika zvakaoma. yematambudziko uye kakawanda zvinouraya).
• Autosomal hune chimiro (pachena vakura, imwe benign kosi).

Osteopetrosis - chii? zviratidzo

Kana recessive modes ose Kupfuudzwa zviratidzo chirwere rokutanga kuonekwa pakarepo pashure pokuberekwa. Vakomana nevasikana vanoitirwawo kazhinji kurwara. The kwomwana chiso ane maitiro chimiro: dzakakwirira cheekbones, wakasimwa kure nakure meso. Mudzi mhino anogara mwoyo, mhino akatendeuka outwards, miromo paukobvu. Nokukurumidza anovandudza kupererwa neropa uye mberi hydrocephalus, pane anyatsoona kuwedzera chiropa uye chirwatata. Very zvakafanana Hemorrhagic diathesis. Zvisingadzivisiki kuguma inova vakawanda mapfupa maronda. With kuparadzirwa dehenya pane compression pamusoro optic uye auditory tsinga kuva upofu uye kusanzwa.

Osteopetrosis - kuuraya kwacho kukanganisa anenge zvose mapfupa refu. Vana vakawanda havana kurarama kusvikira makore 10. Anovandudza purulent osteomyelitis mapfupa rinenge zvichitungamirira kuremara. Chinokonzera rufu, muitiro kuva zvakasimba kupererwa neropa uye sepsis.

Kana chikuru shanduro zvokurwara ari kuona kuchikoro-zera vana uyewo nevakuru. Kazhinji, murwere haasi kutomboziva kuti chirwere ichi achinyengera akaita vakavatamo muviri wake. kazhinji Osteopetrosis iri netsaona kuwanikwa musi radiography kuti mapfupa kutsemuka. Muzviitiko zvizhinji, zviri zvokurwara inoitika zvachose asymptomatic. Vamwe varwere chokunyunyuta pamusoro idzokororo kurwadziwa mapfupa, apo vamwe vave kakawanda vatengi kunotevera, uye ipapo kuwana pamusoro uchishandisa tafura pathological mitswe. Kupererwa neropa kunowanzoratidzirwa zvishoma, chetsinga zvirwere zvichikonzerwa compression of etsinga fibers hadziwanzowaniki.

In munyoro marudzi chirwere Albers-Schonberg chirwere chepfungwa kukura mukasazviziva. Very chinoratidza zvokurwara iyi Papera teething, zvemamiriro kukura kwavo, susceptibility kune caries. Nomumhu pakuzvarwa, haana chairwo, chakakanganisika kwomuviri yakaratidza pedyo gore.

Osteopetrosis (inouraya machena). Diagnostics

Kana okutanga kukura zvokurwara shoma murwere chikawira mumaoko vanachiremba mumwedzi zvoupenyu rokutanga. Panyaya iyi, nechirwere Kazhinji hazvina kunyanya dambudziko. The maitiro pakuonekwa murwere runoita kufungidzira osteopetrosis. Pana mazera vakwegura, varwere kazhinji vakatobatana vakanyorwa panguva orthopedist nokuda kakawanda mitswe. In vakuru, kuti kurapwa mufananidzo avhiringidzwa, zviratidzo zvizhinji regai kuitika, iyo yakati zvinoomesa zviratidzo.

Muzviitiko zvose, pasinei nezera X-ray anokwanisa kusimbisa kana kuramba osteopetrosis. Chii uye chii chiri revo nzira? Mune hofisi zvikuru akashongedzerwa mufananidzo njodzi nzvimbo iri pamaberere maviri. The X-ray chinoratidza chisimbiso mapfupa zvakajeka, apo muganhu pakati chubhu uye chirambe chakapfava nefuma vasipo. Mapfupa akaremara, metaphyses (munharaunda pedyo majoini) vari zvakati kuwedzerwa. Kazhinji kwacho kunosanganisira nomuzongoza, dehenya uye chiuno. Curving chirwere anopa muchinjikwa striation pamusoro vertebrae uye mapfupa refu, riri pachena zvinoonekwa pamusoro x-ray.

osteopetrosis kurapwa

mushonga kukuru asina dzakabudirira kubva chirwere ichi rairwadza. Treatment ari kunyanya symptomatic, nechinangwa kuwedzera vakawanda mamiriro uye kunaka kwoupenyu. Kana Albers-Schonberg chirwere ari kuona mangwanani, murwere anogona kuitwa mwongo transplantation. Uyu yerudzi rutsva muitiro unokubvumira kuponesa upenyu mwana, kunyange panyaya zvikuru skeletal maronda. Kwokutanga akabata mwongo nokuisazve, nani mikana kuti yakanaka Zvaizobuda chirwere.

Nokuti kuita akavhiyiwa hunodiwa kugamuchira zvinhu kubva yepedyo nako murwere wanyatsonzwisisa kunonyanyisa kugarisana Hla-magene. Zvikasadaro, mumwongo asingadi rukabata, pachava zvakakomba yematambudziko kuti kukanganisa upenyu murwere. Kuona kugarisana miedzo chaiyo ropa yaitwa, vachibvumira kuona kufanana genome pamusoro anyatsoronga uye wacho.

Kana vabudirira Zvaizobuda oparesheni pfupa mamiriro zvishoma rakadzorerwa. General mamiriro kuvandudza kunoitika, zvakajairika kuona uye kunzwa. Transplantation - ichi ndicho chete mukana kune varwere maari mamwe marapiro havana kubereka chero zvaioneka zvabuda.

Transplantation kuti hunde masero ose mumwongo ndiyewo aizobudirira chaizvo nzira nokuda kurapwa kwezvirwere zvakadai osteopetrosis. Chii? Muchiitiko chino, hachisi rose nhengo transplantation, asi chete sero rimwe chete. Ivo midzi murwere mwongo nokudzosa zvakajairika hematopoiesis. nzira Iri nyore kutakura varwere vaduku, nekuti haatarisiri intravenous yokutindivadza.

Nokuti pakurapa vakuru vakashandisa calcitriol kana γ-interferon, izvo normalize nezvebasa osteoclasts, nokudaro zvichivhiringidza muitiro remafupa kuparadza uye chisimbiso. kurapa kwakadaro kunogona kuderedza ngozi mitswe itsva, iyo zvakanaka kunokanganisa mararamiro murwere.

yematambudziko osteopetrosis

Kakawanda mitswe mapfupa refu - dambudziko kuti pakupedzisira anotarisana ose murwere. Most sei hudyu, dziripowo kuzotadzira rezasi shaya, nomuzongoza, chipfuva. Muchirwisa colour pakati nedanho hematopoiesis kukura suppurative osteomyelitis, zvakaoma kurapa. Iziviswe muzvidimbu deformation - mugumisiro zvirwere zvikuru inonzi osteopetrosis. Photo migumisiro zvokurwara ichi dostotatochno kutarisa kutyisa.

mataridzikiro

Panguva yakawanda chirwere kunoenderana nemakore murwere. Mune zvimwe zviitiko, chirwere zvingamuka akangoerekana arega uye havana kuzviyeuchidza kwemakore anouya. Mune dzimwe embodiments, kuchiita chirwere kunoitika zvakaoma. kupererwa neropa uye kakawanda pyogenic zvirwere. Muduku mwana, zvikuru mukana wokuti zvakakomba yematambudziko uye rufu. Kusiyana neizvi, munhu mukuru chirwere iyo hworatidza pacharo somunhu sekwaserera, kunetsa chakawedzera fragility mapfupa.

Pasinei kwokuomerwa chirwere, varwere vose ane yasimbiswa chirwere upenyu inofanira kuchengetwa panguva orthopedist. chiremba ane ruzivo vachakwanisa nenguva kuona chero zvichiguma matambudziko uye, kana zvakakodzera, kugadza mamwe nzira bvunzo uye kurapwa.