Hemolytic igazi-Chauffard Minkowski: isifo kwaye afumane unyango

Xa igazi, inqanaba haemoglobin igazi tsakisi kabukhali. Nesi sifo kubo bonke ubomi babo kufuneka nazo abantu abaninzi. Ngaphezu koko, akukho nto ukuphepha imilinganiso abantwana abancinane. iintlobo ezininzi sifo zibangelwa ukungatyi ukutya okunesondlo, ukungabikho kweevithamini, okanye imithi ezithile. Emva lokuphelisa nya zonke mpawu ingqondo factor isikiwe. Phakathi ezahlukeneyo iinkqubo zophendlo lwezifo eyabelwe enye nzima kwaye kuyingozi kakhulu. Le igazi-Chauffard hemolytic Minkowski. Yilaa nto iya kuphendulwa kwinqaku yanamhlanje.

Yintoni igazi?

Phantsi igazi ngokuqhelekileyo iqondwe njengoko isimo umzimba, ebonakala ngu indicators ukuhla kabukhali iiseli ezibomvu kunye mpilo. Ezinye iintlobo ze esi sifo kukhokelela kutshintsho ukuma iiseli ezibomvu zegazi. Balahlekelwa umsebenzi wabo wokuqala ngokuhamba kwexesha.

Igazi udla sikhatshwe iintlobo zezifo, kodwa zange zaseprayimari. Yiyo le nto kufuneka ukuba ushiye ngaphandle ingqalelo disorder. Kuyimfuneko ngokukhawuleza kangangoko ukufumana unobangela kwaye bazama ukuphelisa.

Features hemolytic anemia

Igama elithi "anemia hemolytic" uquka iqela ezininzi izifo. Bonke bephela luphawulwa a Pathogenesis eqhelekileyo. Ukwanda ukutshatyalaliswa iiseli ezibomvu kubangela ukwanda iimveliso zabo ukucazululwa ekwandeni erythropoiesis. Loop Ukwakheka iphazamisekile iiseli ezibomvu. Iinkqubo wentshabalalo okunene kubo kuxhaphaka ekuqaleni ukuya busa phezu ezisebenza kokuhluma ekukhuleni.

Lonke igazi haemolytic kwemiqathango yahlulwe ngamaqela amabini: sofuzo kwaye ziyafikelelwa. Kweli nqaku, senza jonga eneenkcukacha kwi kwinguqulelo yokuqala. Ukuze sibe ngqo, sicinga ukuba sofuzo anemia-Chauffard Minkowski.

Zonyango iincwadi reference uyakwazi ukufumana amagama ambalwa ezichaza inkqubo yezifo. Le igazi microspherocytic kunye spherocytosis ilifa, kunye nezifo-Chauffard Minkowski. Uninzi wayedla igama lokugqibela amagama oosonzululwazi, discoverers.

Isifo ithathwa ngokuba eqhelekileyo kakhulu (1 kuzo zonke 5 amawaka. Population). It unesifo ubukhulu becala abantu Northern Europe. Imiqondiso yokuqala iyabonakala abantwana besebancinane. Ukuba ngaba uqala ukunyanga izifo ngendlela elisexesheni ifuthe layo elibi ukusebenza yonke eziphilayo.

Oonobangela kunye oluqhuba sifo

Igazi Minkowski-Chauffard ekhatshwa ukuphazamiseka inwebu cellular izakhiwo erythrocytes kunye nemisebenzi. Isiphumo sezi nkqubo, bebenokutshintsha imilo wabo setyhula, zaqina. Iimpawu zokuqala hemolysis - ukutshatyalaliswa iiseli ezibomvu kunye ukukhululwa ngaxeshanye mpilo.

Xa umntu iiseli ezibomvu qete imilo lwabo embhoxo idiski biconcave, ukuze ukuhamba ngokukhululekileyo ngokusebenzisa iimpahla. Xa igazi, izinto inwebu leeprothini amagama egxobhekile. Oku kukhokelela kwii-ulwelo kungena iiseli. Ngenxa yesi sizathu, ukutshintsha imilo zabo. Watyhutyha ilizwe imithambo yegazi, iiseli ezibomvu igazi kakhulu likhubazeke, yaye emva kwethutyana ziqala ukuyeka. Ngokuchasene imvelaphi iinkqubo oluqhubekayo, umgangatho iiseli ezibomvu lwehla, ukuphuhlisa anemia hemolytic.

Ukuba omnye umzali owawuthe kwafunyaniswa nesi sifo, kuya kuthi ukuba yi lilifa umntwana. kunqabile kakhulu abantwana abagulayo bazalwa oomama uphilile kwaye abazali. Kulo mzekelo, igazi kuphuhliswa kwimvelaphi utshintsho DNA. I-guquko gene ephambili kwenzeka naxa Palsy. Kunikezo uphuhliso sifo ifuthe le miba ilandelayo umzimba sikanina:

• radiation,-X-reyi;
• ukunxila idibene neetyuwa heavy metal, Narcotics, nicotine;
• kuhlaselwa virus.

Phantsi yempembelelo le mibandela kuphela anemia-Chauffard Minkowski, kodwa kwakhona ngezifo ngaphezulu kakhulu. Ngoko ke, ngexa lokukhulelwa, umfazi kufuneka bazame ukukhusela umzimba wakho.

I Iimpawu zokuqala

Lo mfanekiso kwezonyango luqinisekiswa kakhulu ubungqongqo inkqubo zophendlo lwezifo kunye nesixa erythrocytes iguqulwe kancinane. Iimpawu zakhe yokuqala ibonakala abantwana kokuya esikolweni nokuba ubudala ngexesha esikolweni. Ngeli hlobo igazi ngokuqhelekileyo lihlangana. Ukuxhuzula hemolytic ingxaki xa kukho inani elikhulu intshabalalo ngaxeshanye iiseli ezibomvu, kulandele cwaka. Kulo mzekelo, iimpawu zisenokwahluka kancinane.

Umzekelo, ixesha interictal zesi sifo ibonakalisiwe iimpawu kwegazi. Phakathi kwazo kwakukho pallor ulusu, inwebu yangaphakathi okanye amehlo. Ukuba nengxaki hemolytic picture lwezonyango yi iguqulwe kwaye ihamba kunye mpawu zilandelayo:

1. Ukwandisa lobushushu ukuya degrees 38, intloko ebuhlungu, umkhuhlane.
2. Uphuhliso senyongo.
3. Ubuhlungu esiswini, ebonakala umlinganiswa yiSpasticity.
4. Kabi esibindini ngenxa indyebo yalo.
5. Ukudumba udakada.

Sofuzo hemolytic igazi-Chauffard Minkowski ezifumaneka abadala. Esona sizathu rhoqo ukuze afune unyango kulo mzekelo i yellowness kwesikhumba. Nangona kunjalo, kwiimeko ezininzi, isifo nazimpawu. Izigulane bafunda khona ngengozi ngokuqhelekileyo ngexesha kuhlolwa.

iimviwo Medical

Uxilongo lwe igazi-Chauffard Minkowski ilula. Ukuba urhanela isifo, kwaye kubonakala iimpawu zokuqala kufuneka bafune iingcebiso zonyango. Pathology kwenkqubo hematopoietic na phakathi kobuchule a segazi. Emva kokuhlolisisa izikhalazo yomguli kunye nembali yakhe specialist yosapho kufuneka ahlole ulusu kunye sclera, aqhube Ngokucofa esiswini. Ubudala obunyanzelekileyo bokuhamba ultrasonography kwabelwa wesibindi, udakada, njengoko iliphawu esi sifo ukunyuka ubungakanani kula maqumrhu.

Kwangaxeshanye segazi inika umkhombandlela eziliqela iimvavanyo elebhu. Hemolytic igazi-Chauffard Minkowski yaqinisekisa ubukho nguqu zilandelayo:

1. Umchamo: hemoglobinuria, ukwanda izalathisi iiproteins kunye urobilin.
2. igazi eziphilayo: ukunciphisa cholesterol, dehydrogenase zincancise, ukukhula, ukwanda yibilirubin ngqo.
3. Uhlolisiso iiseli ezibomvu: reticulocytosis ngokukhawuleza, ukuncitshiswa ubukhulu yeseli, ehlise azinze osmotic.
4. Uhlalutyo jikelele igazi: ESR ukukhawulezisa, ukuhla okungephi zegazi kunye nokunciphisa leukocyte Isalathisi umbala.

Okokugqibela ukuqinisekisa kwisigqibo lokuqala inceda yokuziphatha electrophoresis kweziyi le inwebu erythrocyte ngokusebenzisana quantitation zabo.

diagnosis umehluko

igazi Hemolytic Minkowski-Chauffard abantwana maxa wambi kubangela ubunzima ngethuba kokuxilongwa. Esi sifo kuyafana nezinye iimpawu ezimbi sokuhlasela. Ngoko ke, oogqirha kufuneka bazazi ezinye kokuhle kunye uphawu olu hlobo iimpawu anemia.

Okokuqala sithetha yofuzo. Kuphela kukwiimeko ezizodwa bobabini abazali basempilweni ngokupheleleyo. Kwelinye icala, ukuba umntwana ingalandwa inguqu awathethi amathambo yokakayi. Kwiimeko zentandabuzo eyabelwe Ukongezelela Coombs isampula. Ukuba analysis negative, isigulana lixhaswa anemia-Chauffard Minkowski. Uxilongo Ngokwenjenje lugqityiwe.

unyango Conservative

therapy igazi okhethiweyo ngenxa namandla aso. Ngexesha senze, njengoko umthetho, ungenelelo iyafuneka. Ngexesha uhlaselo elandelayo isigulane esibhedlele ngoko nangoko.

unyango Conservative of kwegazi-Chauffard Minkowski kuquka la manyathelo alandelayo:

1. therapy replacement erythrocyte ubunzima, ukuba inqanaba haemoglobin igazi kusithwa kwinqanaba 70 g / l.
2. Albumin iboniswe ukunyanga amazinga aphezulu yibilirubin.
3. Ubuthi kusetyenziswa unyango ukumnika.
4. Xa kungekho ntlekele hemolytic express ibonisa ulwamkelo amachiza choleretic.

Ubude unyango olunjalo, amachiza ezithile umlinganiselo yabo - zonke ezi zinto igqitywa igqirha qho ngamnye.

ukungenelela operative

Ukuba hemolytic microspherocytic anemia-Chauffard Minkowski kuba kakhulu, unyango elondoloza abakwazi ukumelana neenjongo yathi, isigulana luyacetyiswa uqhaqho ukususa udakada. Loo ndlela alunakunyanga ngokupheleleyo ukugula. Kwelinye icala, emva kokuba ungenelelo inani kutshatyalaliswa iiseli ezibomvu yehla kakhulu kwaye elide umjikelo wobomi babo.

nengxaki Hemolytic emva kokuba sebenziso alwenziwanga kuphindwa, kodwa iqela Izimo. Umzekelo, ekususweni udakada akukhuthazwa kubantwana abangaphantsi kweminyaka emi-5 ngenxa yokuba ukufa aphezulu kwithuba postoperative. Icala elisezantsi inkqubo uthathwa ukunciphisa ukuxhathisa komzimba malunga izifo ezosulelayo ezinzwaneni.

Isixhobo esisesinye ngobuwena kucingwa ukuba ukususa udakada occlusion endovascular. Le yenye indlela unyango ukunceda idla kucelwa lokufunyaniswa "anemia-Chauffard microspherocytic Minkowski." Ngexesha inkqubo, ugqirha ifaka kungena umzimba ichiza elo Uxhokonxwa inkantsi, yaye udakada sikhokelela ndamelwa yintliziyo. Ezinye ke igcina igazi ngokupheleleyo kwaye angalahlekelwa ukukwazi ukulwa nezifo.

iingxaki kunokwenzeka

badla Hemolytic enkulisa abantwana anemia-Chauffard Minkowski kukhokelela ekulibazisekeni kuphuhliso engqondweni nasemzimbeni. Ingakumbi ukuba abazali ixesha elide akazange afune uncedo lonyango okanye ingcebiso kugqirha ngoyaba yakhe.

Xa izigulane abadala, i ingxaki ixhaphakileyo kuthathwa ngokuchasene nentsukaphi ukuphazamiseka isifo gallstone of yibilirubin ukutya. Eyona nto kukuba ingxaki hemolytic kaninzi kubonwa ekuqaleni uphuhliso senyongo, ngoko unyango olufanelekileyo ilityazisiwe. Phambi yamatye senyongo Kucetyiswa ukuba cholecystectomy kunye splenectomy.

Sase- sokubuyisela

Xa isifo ngaphantsi kakhulu kunye nokuziphatha kwangethuba umsebenzi ukususa udakada abane intle. Nixolelwe ngokuqhelekileyo kwenzeka ngokukhawuleza emva kwentlekele hemolytic. ubude bayo abafani, kodwa ke kwiimeko kakhulu malunga neminyaka emibini.

iindlela zokunqanda

Igazi Minkowski-Chauffard yi ilifa. Ngoko ke kuthintela elivela kuzo sifo akwenzeki. Ukuze kuthintelwe iintlobo kakhulu zesi sifo izigulane kwafunyaniswa ukuba kukho uhlobo kwegazi kucetyiswa ukuba bangenele njalo kuhlolwa ngokupheleleyo a segazi.

Xa becwangcisa isifundo ukukhulelwa kufuneka kuqondwe ukuba nokwenzeka ekuphuhliseni esi sifo umntwana ongekazalwa-50%. Ngoko kuzalwa kwakhona ubonisa esweni rhoqo ngugqirha ukuchonga ezimbi kwibakala lokuqala.