Chii hunhu Fanconi kuti chirwere? Zvinokonzera, Zviratidzo, kuongorora chirwere

chirwere ichi yakatanga yakarondedzerwa kwachiremba wevana Swiss Toni Debre Fanconi, uyo akaonekwa ari chirwere ari mwana muna 1931. In chirwere chiremba uyu akarondedzerwa ikakuruma, zvishoma nezvishoma anoratidza zvimwe zviratidzo uye chirwere okunze, mukuru izvo zvinofanira kufungwa glycosuria (shuga pamberi weti) uye albuminuria (pakuonekwa mapuroteni iri weti).

The zvinokonzera Fanconi urwere

Kakawanda chirwere ichi yechipiri, mberi pamwe nezvimwe zvirwere. Dzimwe nguva zvinoitika kuti Fanconi chirwere ndicho chirwere chikuru iyi inogona kuwanikwa uye nhaka. Zvikonzero chingava:

• tinozvarwa zvikanganiso muviri;
• chepfu nokuda rinorema simbi kana chepfu (mekuri, kutungamirira, kunonoka tetracycline);
• chitarisiko namamota uye malignancies (mhando dzakasiyana nekenza);
• kushayikwa vhitamini D.

Dzimwe kufanana chirwere chinogona kuitika sezvo pakava pashure kutsva kana zvimwe zvirwere renal. Fanconi chirwere vakadzi vanogona kukonzerwa hormone imbalances kuti achamuka shure yokusununguka.

Huru Zviratidzo

Zviratidzo zviri chirwere zvinosiyana zvichienderana nemakore murwere. Nokuti vakuru zviratidzo chirwere maronda yokutanga: kunyanya weti, tsandanyama simba uye kurwadziwa mapfupa uye mapfundo. Izvi rinotsanagurwa ndechokuti chirwere chiri chakasimba munhu yokushomeka zvinokurisa (sodium, potassium, calcium yakawanda).

Fanconi chirwere kune ngozi zvikuru vana, nokuti kunogona kukonzera kusingarapiki kwazvinoita nemamiriro rejaya muviri. Kazhinji muvarwere vaduku kuvandudza kusimba, stunting uye underdeveloped tsandanyama, inova migumisiro kushayikwa zvicherwa zvakakosha. The kunzwisisa chirwere anogona kuderedza padanho kutapata ari mwana, zvichiita zvikuru kubatwa nezvirwere zvinotapukira.

Zvisinei, vanachiremba tsika zvinosanganisira zviitiko apo varwere vemazera akasiyana, pakanga pasina kurapwa zviratidzo Fanconi syndrome. Muchiitiko chino, zvimwe ndezvipi zvinogona kuitwa bedzi migumisiro ropa uye weti nemiedzo.

Nzira nechirwere

Zadzisai pamberi chirwere ari murwere yakawanikwa tichibatsirwa wakananga rabhoritari kuferefeta mvura (weti uye ropa), uyewo tichibatsirwa X-ray pakuongorora mapfupa uye mapfundo. Kakawanda, inoongorora mepu inoratidza kuvapo zvimwe pathologies mu nemakemikari omuviri. For Fanconi kuti chirwere kunoratidzwa:

• dzikira padanho calcium phosphorus muropa;
• kukanganisa pakufamba enyika ari renal tubules (iyo inotungamirira kurasikirwa alanine, glycine uye proline);
• metabolic acidosis (kuderera ropa bicarbonate wevasungwa).

Fanconi chirwere chinogona chirwere uchibatsirwa anokosha mano, akadai X-ray. Muchiitiko ichi icho zvinetso uye mapfupa deformation uye migumo yose. Zvisinei, kwete varwere vose vane zviratidzo zvakafanana, saka sokuongorora kwomurabhoritari zvinofanira kufungwa nzira zvikuru yakavimbika Detective chirwere.

Nzira kurapwa zvirwere

Nekuti maperembudzi kunokonzera chikuru kuvadza asidhi-nechigadziko uye electrolyte mwero mumuviri, mishonga nzira inofanira zvainanga kururamisa zviratidzo izvi. Varwere vanokurudzirwa Inwa mvura, anokosha zvokudya, hunoita kuti adzorere ufanane zvisipo zvinovaka muviri (calcium phosphorus) uye vhitamini D.

Saka zvinokosha kuti kusiyanisa zvokurapa uye vasiri pharmacological mishonga Fanconi syndrome. Chokutanga mhando kunobva pakushandisa kugadzirira ine vhitamini D3. Kushandiswa boka iri zvinotungamirira nokuwedzera zviri phosphorus uye calcium yakawanda muropa. Kana zviratidzo izvi tisvike kuipa zvese, vhitamini zvinofanira vakarega.

Zvinodhaka kurapwa kunobva zvokudya kurapa, iyo ipfupi kushomeka kushandisa munyu uye zvokudya anokonzera kurasikirwa nesurferi amino acids. Kana kuputswa ph-pachiyero (acidosis), zviyero akadaro arege kuva zvakakwana, saka varwere ushandiswe okuwedzera mishonga mapoka.

Kufembera uye kudzivirira zvirwere

Forecast Fanconi chirwere kune chokuita panowanikwa yematambudziko chokuita itsvo ushande. Izvi runoratidzwa muchimiro kutyorwa renal parenchyma (dzakananga zvinyama kuti chinovafukidza muviri). kuchinja izvi zvinogona kukonzera chitarisiko pyelonephritis uye renal kukundikana.

Okudzivirira nzira iyi nyaya dzakafanana nzira kurapa asiri zvinodhaka: murwere anofanira kugara vakaramba vari zvokudya zviri zvimwe zvinovaka muviri uye zvicherwa.

Kuvapo chirwere ichi vari hama uye vomumhuri zvingaratidza Kupfuudzwa yayo kuchizvarwa chevechiduku, nekuti Fanconi chirwere - iri nhaka chirwere. Kana Links yakadaro zvechokwadi zvakakodzera kuita zvakadzama inoongorora ropa uye weti muvana.

Fanconi urwere mune imbwa

Sezvo chirwere ichi hachisi mugumisiro matambudziko rechitatu-party Micro-zvinenge uye nemavhairasi, kubata navo nhau hazvibviri. The chirwere kungangova mugumisiro pathological kwekuneta itsvo mune vamwe vanhu. Uye mhuka, zvinokosha kuziva, dzinenge dziri imbwa nechirwere chirwere ichi, zvakare.

Kuvapo chirwere chakadaro imbwa akaonekwa pashure kupfuura unoenderana zvirwere zvevanhu. Kunyange zvakadaro, havana zvikuru zvakafanana unhu hwavo.

Havasi dzinoita zvose dzembwa sei nechirwere ichi. Akanga kuonekwa beagles, whippets uye Norwegian Elk nembwa. Kakawanda chirwere ne Veterinarians mu basenji Fanconi chirwere, mumwe mukuru dzemarudzi imbwa.

Matambudziko ari chirwere ari mumuviri wembwa

Sezvakaita nomunhu, hosha anouraya itsvo remhuka. Anotambura pachiyero zvinovaka muviri muropa uye weti kuonekwa tyisa kune svikiro yeimwe uye zvicherwa. imbwa muviri unotanga kuona kuti amino acids uye mavhitamini, pamwe mutsvina yadzo, zvichiguma kusagadzikana tsandanyama uye zvomukati.

Kwenguva yakareba kunokuvadza chirwere kungaita angoerekana uremu, kupera mvura mumuviri hwemhuka, uye kunyange rufu. Imbwa Tsandanyama zvishoma nezvishoma vanotanga atrophy nokuda kwokushayiwa runodiwa nhamba zvinhu muropa, saka murwere mhuka hakuchina vanoshingaira.

Zviratidzo zviri chirwere chirwere mune imbwa

The chikuru Zviratidzo chirwere zvinofanira kufungwa kunyanya mvura yaunodya, uye sezvinoita mvura. muviri Animal anoedza kudzosa mvura nechiyero, iyo inotungamira kuti kunaka zvachose Exchange Urume.

Panguva imbwa anotanga uremu, kunyange kugara uye mafashafasha. Pane bvudzi kurasikirwa, simba kurasikirwa, uye kufamba. mhuka vanorwara vanogona kunzwa marwadzo ari mapfundo mapfupa. Zvinokosha kucherechedza kuti chirwere kazhinji anoratidza pacharo kwete tanga upenyu wembwa (kusiyana munhu), asi kumwe pakati, kuguma upenyu kutenderera (pakati chechi5 uye 7th gore). For basenji zera iri kunyanya kutsoropodza, nekuti vanopfuura 50% dzembwa dzinotanga kutambura yakafanana chirwere panguva kupiwa nguva. Naizvozvo, varidzi erudzi urwu vanofanira kungwarira chaizvo kuti kufanotaura pakuonekwa syndrome.

Test zvechinguvana nokuti imbwa urwere

vachiongorora ichi yambokurukurwa nemasayendisiti muna July 2007. Kunoitawo kuti kuziva Gene anokonzera panowanikwa Fanconi chirwere mune imbwa. In ichi bvunzo mhuka inogona kusununguka zvachose kubva ndingangokuvara hosha angava chirwere kana chaidzo keriya kuti kufukurirwa pairi.

kennels Most uyo dzinoberekera imbwa, vane zvombo zvavo unyanzvi zvokuti unobvumira vatengi kuti kuvimba kuzviwanira mbwanana utano. Zvinofadza kuti Internet rine Database yose, inomiririra vakanyorwa puppies pakati nemaruva siyana uye migumisiro bvunzo neimwe nyaya.

The Developers of murabhoritari kuongorora kunoratidza kuti bvunzo hakusi inonzwisisika chinoratidza puppy kusangana chirwere. Kunyange zvakadaro, 90% of mugumisiro wanika ichokwadi.

The eponymous chirwere Wissler-Fanconi

Wissler-Fanconi chirwere mune vana chirwere isina chokuita chirwere, munyaya ino. zvokurwara Izvi zvinogona kuonekwa mune mwana pakati pemakore matatu navanomwe makore, kunyange pane nezvayo nyaya yacho urwere, uye panguva imwe yapfuura zera (anenge ari vhiki yokutanga muupenyu).

chirwere ichi akaonekwa navanachiremba Wiesler uye Fanconi panguva dzakasiyana uye munzvimbo dzakasiyana-siyana pasi pose, ndokusaka inonzi, rine mazita vanachiremba vaviri, kwete Wissler kuti urwere kana Fanconi syndrome. Photos re munyaya ino zvakakwana inoratidza zviratidzo nehosha, kureva neutachiona. Uyezve, mwana murwere tembiricha inogona wakawedzera, uye mapfundo kazhinji vanotanga kurwadzisa uye kuzvimba. Complex kurapwa kunosanganisira kushandisa mishonga inorwisa mabhakitiriya uye salicylates.