I-Hemophilia - iyini, nokuthi ungakugwema kanjani izinkinga ezibucayi?

Qonda i-hemophilia-ukuthi lokhu kubaluleke kakhulu, ngoba lesi sifo siphutha kakhulu. Lezi zifo zithandwa kakhulu. Lesi sifo siyingozi kakhulu: kubalulekile ukuyibona ekuqaleni kwesigaba sokuthuthukiswa, ukuze kungabi nokuletha isimo esimweni esibucayi.

I-Hemophilia - iyini?

Ukuze uqonde ukuthi lesi sifo siyini, incazelo yegama elisetshenziswe izosiza. Kusukela olimini lwesiGreki, elithi "haima" lisho "igazi," nelithi "philia" lisho "ukulutha." Lesi sifo sibonakala nge-coagulability ephansi yegazi. Bahlushwa ngamadoda, kodwa kukhona okuhlukile (uhlobo C). Bafumana ukubola kwegazi okubi kumama: nguye othwala lesi sifo futhi uhambisa "ibhonasi" kumadodana akhe. Umlando uqukethe amagama abesifazane abakhulu abathwala isakhi se-hemophilia. Isibonelo, iNdlovukazi uVictoria noDuchess of Hesse.

I-haemophilia izuze kanjani ifa?

Isakhi se-pathology siyaphindaphinda. Itholakala ku-X chromosome. Ifa lomdlavuza we-pathological lihlotshaniswa nobulili. Ukuze uqale ukubonakalisa lesi sifo, udinga ama-X-chromosomes amabili ashintsho. Lena "isethi" yesifazane. Amadoda nawo anama-chromosomes e-X ne-Y. Nokho, ngokusebenza konke kwenzeka ngokuhlukile. Lapho owesifazane ekhulelwa intombazane enama-chromosome amabili aguquguqukayo, ukukhulelwa kwesisu kwenzeka emavikini amane. Lokhu kungenxa yokuthi i-fetus ekhulayo ayikwazi ukusebenza.

Intombazane ingazalwa ngokushintsha kwe-chromosome eyodwa ye-X. Kulesi simo, lesi sifo asizibonakali ngokwayo: isakhi sofuzo esiphezulu asikwenzanga ukuvuselela isifo lesi sifo. Ifa le-hemophilia lingabonwa kubafana. Emzimbeni wesilisa, ayikho isakhi esivelele ku-Y chromosome, futhi isethi se-X enokuguqulwa kwesimo sithuthuka ngamandla. Ngenxa yalesi sizathu, abafana bazuze lesi sifo, futhi i-hemophilia inezici ezidlulayo.

Yini engasongela ukubola kwegazi okubi?

Lesi simo se-pathological siyingozi kakhulu. Ukuvela kwezinkinga ezalandela kungenakuqhathaniswa nokuhlanganiswa nesigaba sesifo. Lokhu kuyingozi kangakanani i-coagulability embi yegazi:

1. Lokhu kubangela inqwaba ye-placenta ekukhulelweni kwesikhathi eside.
2. Kungabangela ukuphuma kwegazi ngesikhathi sokubeletha.
3. I-dental extraction, imisebenzi yokuhlinzwa nokulimala kanzima kubangela ukulahlekelwa igazi.

I-Hemophilia - Izinhlobo

Ukweqa ngegazi kuyindlela yokuzivikela yomzimba. Kule nqubo, i-fibrinogens, amaplatelets kanye nezici ze-plasma zithatha ingxenye esebenzayo. Ukuntuleka kwesinye salezi zinto kubangela ukuphazamiseka ekusebenzeni kwayo yonke uhlelo lwe-coagulation. Ngokuya ngesici esingekho, lezi zinhlobo ze-hemophilia ziyahlukaniswa:

• A;
• B;
• C.

Ngokwesilinganiso sobukhulu besifo lesi sifo, lezi zigaba ze-pathology ziyahlukaniswa:

1. Kulula - ngokuphuma kwegazi kwenzeka kaningi. Ngaphezu kwalokho, bancane kakhulu.
2. I-Medium-severe - ngoba iboniswa ubunzima obulinganiselayo bokubonakaliswa okukhulu.
3. Okukhulu-uma, isibonelo, uma i-coagulation encane yegazi enganeni isanda kuzalwa, lokhu kubonakala ngokuphuma ekhanda lomzimba, ukutholakala kwama-hematomas ekhanda, melena njalonjalo. Lapho usukhulile, isifo esinjalo asiyingozi nakakhulu kunasebuntwaneni.

I-Hemophilia A

Ngoba lolu hlobo lwesifo lubhekene nokulahlekelwa kwe-globulin ye-antihemophilic - isici VIII. Loluhlobo lwempilo lubhekwa njengesiqalo futhi oluvame kakhulu. Kutholakala ku-85% wamacala lapho isiguli sinesifo se-hemophilia. Lolu hlobo lwesifo luhambisana nokuphuma kwegazi okukhulu kakhulu. Ngalesi sizathu kubalulekile ukuqonda: i-hemophilia - iyini nokuthi yini ehambisana nayo.

Isici esihlukile salolu hlobo lokugula ukuhlukumeza isigaba se-plasma ye-hemostasis. Ngamazwi alula, ukuphuma kwegazi akuveli ngaso sonke isikhathi ngemuva kokulimala. Lokhu kungenxa yokuthi amapleletlet kanye nezigaba ze-vascular zisebenza kahle. Ngemuva kwalolo suku ngemuva kokuthola ukulimala, ukuphuma kwegazi okungase kuqale, kungase kube nzima ukuma. Le nkinga ayikwazi ukuxazululwa ngosuku olulodwa.

I-Hemophilia B

Igama lesibili lesi sifo yisifo se-Christmass. Lesi sifo sibonakala ngokuntuleka kwe-factor IX. Le nxenye ye-enzyme isebenza isakhiwo se-Stuart-Prower. Lolu hlobo lwe-hemophilia ezinganeni lwenzeka kuma-20% wamacala ahlolwe ukukhubazeka kwegazi. I-pathology enjalo ibonakala ku-1 kwabangu-30 000 abafana abasanda kuzalwa.

I-Hemophilia C

Lezi subspecies inezibonakaliso zayo zemitholampilo. Ihluke kakhulu kuhlobo lobuhlobo lwesifo. Lezi zinsizakalo ze-subspecies zikhishwe ngisho nokuhlukaniswa kwamanje. Ngenxa yalokhu kubalulekile ukuqonda i-hemophilia - kuyini. It isici ehlukile ukuthi akwenzeki kwezinye izinhlobo zezifo. I-hemophilia enjalo kulabo besifazane ibhekwa njalo njengamadoda. Lokhu kungenxa yokuthi ukudluliswa kwezakhi zofuzo kwenziwa ngento evelele. Kukhona nokutholakala kwezwe kuhulumeni wesifo sezempilo. Lesi sifo sithinta ama-Ashkenazi amaJuda kaningi.

Izimpawu ze-hemophilia

Ezifo zezinhlobo A no-B, isithombe esifanayo somtholampilo. Kulesi simo, izimpawu ezimbi ze-clotting igazi zilandelayo:

1. Ukuba khona kwamahematomasi ahlukahlukene emzimbeni. Zivela ngisho nangemva kokulimaza okuncane. I-hematomas enjalo ine-distribution ehlukahlukene. Uma ucindezela endaweni yokulimaza kukhona ubuhlungu obuqinile.
2. Ezimweni ezingu-80%, i-hemarthrosis yenzeka. Isihlanganisi siphenduka, sishisa, futhi isikhumba phezu kwalo siyanqamuka. Ngokuphefumula okuphindaphindiwe, i-osteoarthritis iqala ukuthuthukisa. Ngesikhathi esifanayo kungase kube nokuvinjelwa kwamathambo esifuba kanye nekholomu ye-vertebral, i-atrophy of muscle, ukuguqulwa kwezinyawo.
3. Ukwehla okwenzeka ngemva kokuhlinzwa, ukusika, ukuchithwa kwezinyo kanye nokulimala okunye.
4. Ezimweni ezingu-5% (ngokuvamile kaningi ezigulini ezisencane) kunesifo esibuhlungu .
5. Ukushona kwesimiso emasendeni - kuhambisana nobuhlungu obukhulu nezinye izibonakaliso ezifana nomtholampilo we-peritonitis.
6. Ezimweni ezingu-20%, i-hematuria ibonakala. Lesi simo sokugula singahambisana nokuhlaselwa kwe-renal colic futhi kubangele ukuthuthukiswa kwe-pyelonephritis.
7. Ukwakheka kwesibhamu , okwenziwe ama-hematoma amakhulu.
8. Ukuphuma kwamathumbu emathunjini, okubhekwa ngamacala angu-8% kulabo abahluphekayo nge-coagulation embi. Izinhlanzi ezimnyama zivame ukuhambisana nobuthakathaka kanye nosizi.

Izibonakaliso zokugwinya kwegazi emzimbeni we-hemophilia uhlobo C ezigulini eziningi zichazwe ngokweqile noma zingabonakali nhlobo. Abanye abantu baphepha, i-menorrhagia kanye nokubonakala kwemivimbo emzimbeni. Ngisho emndenini owodwa, amalungu awo ahlupheka ngenxa yalesi sifo, ubukhulu bokubonakaliswa kwemitholampilo kuyahluka kakhulu. Kodwa-ke, ngokungafani nezifo zezinhlobo A no-B, i-hemophilia C ngokweqile kwamathumbu emisipha akuvamile. Ama-hemarthroses ayenzeka kuphela lapho kunamahlumela okuhlukumeza amajoyina.

I-coagulability engalungile yegazi - yini okufanele uyenze?

Uma une okungenani isibonakaliso esisodwa sesifo, kufanele uthole ngokushesha usizo lwezokwelapha. Ukuqinisekisa ukuxilongwa, udokotela uzohlinzekela isiguli ukuhlolwa okubandakanya izindlela ezinjalo zokubhubhisa:

• ukuzimisela kwenani lama-coagulation izici ezikhona egazini;
• inkomba yeprothrombin;
• ukuzimisela isikhathi lapho i- clotting yegazi ithatha khona;
• i-mixt;
• izinga le-fibrogen egazini nokunye.

I-Hemophilia ibhekwa ingabi nalutho - ukwelashwa kwalesi sifo kuncishiswe ukwelashwa kwesondlo. Ngaphezu kwalokho, hhayi indima encane kunikezwa ukudla okunomsoco. Phakathi nenkathi yokwehla kwesifo, ukwelashwa kwayo kufanele kwenziwe esibhedlela esikhungweni sezokwelapha. Ochwepheshe bemitholampilo enjalo bayazi ukuthi i-hemophilia - yimuphi uhlobo lwe-pathology. Isiguli esiye saxhumana nesiguli kumele sibe ne "Incwadi yesiguli" naye. Le dokhumenti ehambisana nalokhu iqukethe ulwazi mayelana neqembu labantu legazi, i-Rh factor. Ibuye ibonise ubukhulu besifo.

Ungamisa kanjani igazi nge-coagulability engafanele yegazi?

Uma umuntu ene-hemophilia, udinga ukucabangela ngokukhethekile. Awukwazi ukuthatha kalula impilo yakho! Izimiso zokuziphatha kulokhu zilandelayo:

1. Uma amanxeba ajulile, udinga ukuguqula futhi uphathe ukwelashwa okunye esikhundleni. Ngaphezu kwalokho, i-ice kufanele isetshenziswe endaweni elonakalisiwe (akufanele ihlangene nesikhumba, ngakho kufanele ifakwe ithawula leshidi noma ithawula elincane ngaphambi kokusetshenziswa).
2. Esilonda nasezikhaleni kungenza ama-clots amakhulu. Kuzo, igazi liyavutha. Ngenxa yezindwangu ezinjalo, amanxeba ayanda. Ukuze ugweme lokhu, udinga ukususa ngokucophelela "izimpumputhe". Ngaphezu kwalokho, le ndawo kufanele ihlanjwe ngesisombululo se-penicillin. Ngemva kwalokho, ibhande elifakwe isakhi se-hemostatic lisetshenziswa esikhumbeni. Lokhu kuphuthumisa ukuphulukiswa kwesilonda.

Imithi ene-poor coagulability engafanele

Ukwelashwa kwe-hemophilia kubonakala kanje:

1. Isigulane sijojowe ngezinto zokulahleka ezingekho. Inani lezinqubo ezinjalo lingashintsha kusuka ku-4 kuya ku-8 ngosuku.
2. Nika ukujova kwamalungiselelo okwenziwa nge-plasma.
3. Ukuze ususe emagciwane omzimba wesiguli ukuze uthole izici zokugqoka, udokotela angakwazi ukubeka i- plasmapheresis .
4. I-hemophilia ye-Hereditary ihlinzekela ukukhipha isisombululo se-glucose, i-Reamberin noma i-Polyglucin.

Nge-hemarthroses, ukukhishwa kwesikhwama esivumelwaneni kwenziwa ngokwengeziwe. Ukuphefumula kokuqukethwe kwegazi kanye nokucebisa kwayo ngama-hormonal amalungiselelo kwenziwa. Ngokuqinisekile, isilwane esithintekile kufanele singabi nhlobo ngesikhathi sokwelapha. Esikhathini esizayo, emva kokuvuselelwa, ukuqeqeshwa komzimba kanye nokwelashwa kwe-physiotherapeutic kunqunywe.

Yini ongayidla ngegazi elibi?

Ukudla okunomthelela kunomthelela omkhulu esimweni seziguli. Ukuvimbela ukuvela kwegazi, iziguli ezine-hemophilia kufanele zicebise ukudla kwazo ngokudla okunjalo:

• amafutha wesilwane (ibhotela, isinqumelo, ukhilimu);
• isibindi (ubuchopho, izinso, isibindi);
• ibhuloho lokubhakana;
• amajikijolo ombala we-violet (ama-blueberry, ama-blueberry, ama-blackberry nabanye);
• ubhanana namaMango;
• pepper obomvu, isithombo, iklabishi ebomvu neminye imifino;
• ama-walnuts;
• ubhontshisi, uphizi, ubhontshisi kanye nezinye ubhontshisi;
• i-dill, i-parsley, i-basil;
• isinkwa esimhlophe.