Hemophilia - wannan shi ne abin da cuta? Yadda aka kuma abin da suke bayyanar cututtuka na hemophilia?

Domin mafi jahilan mutane hemophilia - abin da ake kira gidan sarauta cuta, san game da shi kawai a tarihi: suka ce, shi ya sha wahala Tsarevich Alexei. Saboda rashin ilmi, mutane sau da yawa sun gaskata cewa talakawa mutane ba zai iya samun rashin lafiya tare da hemophilia. An yi imani da cewa shi rinjayar kawai tsoho zamaninsu. A wannan hali ya dade da "aristocratic" gout. Duk da haka, idan gout - Power cuta, kuma shi yanzu zai iya zama batun da wani mutum, cewa hemophilia - wani hereditary cuta, kuma shi zai iya samun wani yaro, wanda kakanninsa suka yi da cutar.

Mene ne Hemophilia?

A mutane, cutar da ake kira "ruwa da jini." Lalle ne, tsarin pathological, sabili da haka damuwa ikon gudan jini. 'Yar alamar karce - da kuma zub da jini wuya a dakatar. Duk da haka, shi ne waje bayyana. Mafi tsanlnin ciki da ke faruwa a gidajen abinci, ciki, koda. Zubar jinni a cikinsu za a iya kira ko da ba tare da waje tasiri da kuma masu kawo hadari sakamakon.

Domin jini coagulation alhakin goma sha biyu musamman sunadarai cewa ya kamata ba a cikin jini a iyakancin taro. Hemophilia cutar da ake bincikar lafiya a yayin da daya daga wadannan sunadarai zai yi ba ya nan a duk ko da yake ba a kasa taro.

iri hemophilia

A magani, akwai iri uku cutar.

1. Hemophilia A. Ake rashi ko rashin coagulation factor Sabunta. Mafi na kowa tsari da cuta ne, bisa ga statistics, 85 bisa dari na duk lokuta. A kan talakawan, a baby na 10 000 ne kawai irin wannan haƙuri da hemophilia.
2. Hemophilia B. Lokacin da shi ya lura da matsaloli tare da factor karkashin yawan IX. Alama a matsayin mai yawa fiye da rare: shi m hadarin shi ne sau shida ƙananan fiye da a cikin hali na zabin A.
3. Hemophilia C aka rasa factor yawan XI. Wannan nau'in shi ne na musamman: shi ne halin da mata da maza. Haka kuma, sau da yawa m-Ashkenazi Yahudawa (wanda a general ba hali wani ailments da suka saba ne na kasa da kasa da kuma wannan "jijjiga" to duk jinsi, kasashe da kuma kabilu). Tare da bayyanuwar hemophilia kuma buga daga cikin sauran na asibiti hoto, don haka a 'yan shekarun nan, shi ne riƙi fita daga cikin jerin hemophilia.

Ya kamata a lura da cewa daya bisa uku na iyalan wannan cuta ya auku (ko kamu) karo na farko, shi ya zama mai buga wa unprepared iyaye.

Me cuta ya auku?

Its ƙãga zama nakasar hemophilia gene, wanda aka located a kan X chromosome. Jigilar shi ne mace, ta ce tana ba da haƙuri, fãce dõmin ya zama m nosebleeds, ma nauyi haila, ko fiye da sannu a hankali yana warkar da raunuka kananan (msl, bayan da ja daga hakora). A gene ne recessive, don haka rashin lafiya, ba duk wanda ya yi wata uwa - wani m da cutar. Yawanci, yiwuwar an rarraba 50:50. Yana qara idan iyali yana ma mahaifinsa. Girls ma suna dako na gene ne m.

Me hemophilia - wata cuta ga maza

Kamar yadda aka ambata riga, da hemophilia gene ne recessive da aka haɗe zuwa chromosome, kira a matsayin X. A mata, biyu, waɗannan, chromosomes. Idan wanda irin wannan gene buga, shi ne mafi rauni da na biyu mur, rinjaye, saboda haka cewa akwai kawai wata yarinya m, ta hanyar abin da aka ruwaito hemophilia, amma kanta zauna lafiya. Akwai tsammãnin tare da biyu ganewa X chromosome iya ƙunsar daidai gene. Duk da haka, a lokacin da samuwar 'ya'yan nasu jijiyoyi (da wannan ya auku a kwata ciki mako) shi ya zama unsustainable, kuma akwai wani yunkri zubar da ciki (ashara). Tun da irin wannan sabon abu zai iya zama saboda dalilai daban-daban, yawanci ba nazarin samoabortnogo ba za a za'ayi, don haka da cewa statistics a kan wannan batun ba.

Wani abu - maza. Bã su da wani biyu X chromosome ba yanzu, shi ya maye gurbin Y. Marinjãyi "xxx" ba, don haka idan wani recessive bayyana kanta, shi fara gudãna daga cutar maimakon ta latent jihar. Duk da haka, saboda chromosome ne har yanzu biyu, yiwuwar samun irin wannan ci gaban da mãkirci ne daidai da rabin dukan rashin daidaito.

bayyanar cututtuka na hemophilia

Suna iya bayyana kanta a haihuwa, idan dacewa factor a cikin jiki shi ne kusan ba ya nan, kuma zai iya yin kanta ji kawai a kan lokaci idan akwai wani rashin shi.

1. Zub da jini a cikin rashi na dalilai. Sau da yawa yaro an haifi tare da zane na jini daga hanci, idanu, cibiya, kuma yana da wuya a dakatar da zub da jini.
2. Hemophilia (hoto ne show), bayyana kanta a cikin ya fi girma kumbura bruises a kan cikakken bai isa a kula tasiri (misali, yatsa matsa lamba).
3. Maimaita zub da jini kamar riga ya warkar da ciwo.
4. Karuwan iyali zub da jini: hanci, gumis ko da brushing ka hakora.
5. Zub da jini a cikin gidajen abinci.
6. jini a cikin fitsari da kuma tumbi.

Duk da haka, irin wannan "ãyõyi" Ba dole ba ne nuna shi ne game da hemophilia. Alal misali, nosebleeds iya nuna wani rauni daga cikin ganuwar jini, jini a cikin fitsari - domin na koda cututtuka da kuma a cikin stool - annoba. Saboda haka shi ne tabbatar zama ƙarin bincike.

Gano hemophilia

Bugu da kari ga karatu da likita tarihi na haƙuri da jarrabawa na wani iri-iri na kwararru gudanar da dakin gwaje-gwaje gwaje-gwaje. A farko wuri ƙaddara da kasancewar a cikin jinin dukan coagulation dalilai da su maida hankali. Don saita lokaci da ta dauki ga coagulation na jini samfurin. Sau da yawa wadannan gwaje-gwaje suna tare da DNA bincike. kafiya iya zama dole domin karin m ganewar asali:

• thrombin lokaci.
• gauraye nunka;
• prothrombin index.
• da adadin fibrinogen.

Wani lokacin nema, kuma mafi musamman bayani. Hakika, sanye take da da ya dace kayan aiki, ba kowane asibiti, don haka tare da zargin hemophilia aka aika zuwa jini Lab.

Da cutar, wanda yana tare da hemophilia (photo)

A mafi halayyar hemophilia - shi ne articular zub da jini. A likita sunan - gemoartroz. Yana tasowa wajen sauri, ko da yake mafi peculiar ga marasa lafiya tare da m hemophilia. Sun zub da jini a cikin gidajen abinci faruwa ba tare da wani waje tasiri, spontaneously. M don su tsokane gemoartroza bukata rauni. Gidajen abinci da bala'in ya shafa da farko ta ga waɗanda ake fuskantar danniya, watau, gwiwa, hip da pristopnye. Na biyu a layi - kafada bayan su - da gwiwar hannu. A farko bayyanar cututtuka bayyananne gemoartroza riga da shekaru takwas yaro. Saboda articular raunuka na mafi yawan marasa lafiya saya da wani tawaya.

M sashin jiki: koda

Hemophilia cutar sau da yawa yana sa bayyanar jini da fitsari. Shi ne ake kira hematuria. iya ci gaba smoothly, ko da yake da alama har yanzu ya kasance gangami. Game da rabin da haematuria yana tare da shafe tsawon m zafi. M koda colic lalacewa ta hanyar jini clots turawa ureter. Da aka fi a marasa lafiya tare da hemophilia pyelonephritis, bi a hankali da aukuwar - hydronephrosis, da kuma kwanan nan ya dauki wani capillary sclerosis. Jiyya na koda cuta wuya da wasu hani a kan magani kwayoyi: wani abu da taimaka wa liquefaction na jini, ba za a iya amfani da su.

hemophilia magani

Abin baƙin ciki, hemophilia - wani m cutar da ke tafiya tare da dan adam rayuwa. Ba tunanin wani hanya da cewa ba za ka iya tilasta wa jiki, don samar da sunadarin da ake bukata, idan shi ne bai iya yi da shi daga haihuwa. Duk da haka, da ci gaba a zamani magani sa shi yiwuwa a kula da jiki a matakin a wanda mutane da hemophilia, musamman a ba mai tsanani, zai iya kai kusan al'ada zama. Don hana zub da jini da kuma hematoma bukatar yau da kullum jiko mafita bace clotting dalilai. Suna ware daga jini na mutum gudunmawa da girma ga mai kyauta dabbobi. Administration da kwayoyi yana da wani dundundun a matsayin yin rigakafi da warkewa idan mai zuwa tiyata ko rauni.

A a layi daya, hemophiliacs dole ne kullum sha jiki far don kula da hadin gwiwa kiwon lafiya. Idan ma manyan, shi ya zama m hematoma likita sa wani aiki ya cire su.

Kamar yadda ake bukata transfussion kwayoyi sanya a kan tushen da jini, hemophilia cuta qara hadarin don samun kwayar hepatitis, cytomegalovirus kamuwa da cuta, herpes, da kuma - m - cutar HIV. Ba tare da wani shakka, duk masu bada taimako ana gwada wa aminci su jini, amma a tabbatar babu wanda zai iya.

samu hemophilia

A mafi yawan lokuta, hemophilia ne hereditary. Duk da haka, akwai wasu statistics a lõkacin da ta bayyana kanta a manya, a baya ba tare da wani tarihi na shi. Abin farin, irin haka ne musamman rare - daya ko mutane biyu da miliyan. Mafi samun cutar, kasancewa girmi shekaru 60. A cikin dukkan lokuta na samu haemophilia - wani irin A. Abin lura shi ne cewa dalilai wanda shi zo, sami kasa da rabi daga cikin marasa lafiya. Daga cikin wadannan cutar daji, da shan wasu magunguna, autoimmune cuta, shi ne sosai rare - pathological, tare da abin da ya faru na tsanani, marigayi ciki. Me wasu zama da rashin lafiya, ba za a iya kafa likitoci.

Victoria cuta

A farko idan na samu cututtuka da aka bayyana a cikin Example Sarauniya Victoria. Na dogon lokaci da aka dauke shi kadai daya daga irinsa, tun da ba ta, kuma kusan rabin karni bayan da hemophilia a mata ba ya lura. Duk da haka, a cikin karni na ashirin, tare da zuwan statistics a kan saye da sarauta cuta, musamman sarauniya ba za a dauke: hemophilia, abin da ya bayyana bayan haihuwa, wadanda ba hereditary, ba ya dogara ne a kan jima'i rashin lafiya.