Phospholipid syndrome: Oonobangela kunye Diagnosis

syndrome Phospholipid - a ngezifo eqhelekileyo noko imvelaphi sokuhlasela. Ngokuchasene nentsukaphi sifo zidla wabona semithambo, zezintso, amathambo kunye namanye amaziko. Xa kungekho unyango sifo kungakhokelela kubunzima eziyingozi de kufe isigulane. Ngaphezu koko, esi sifo isoloko labonwa abasetyhini ngexesha lokukhulelwa, engozini impilo kamama nomntwana.

Kakade ke, abantu abaninzi bafuna ulwazi olongezelelweyo, ukubuza imibuzo malunga ezibangela izifo. Ziziphi iimpawu kufuneka ndikhangela? Ingaba kukho uhlalutyo syndrome phospholipid? Ngaba amayeza ukunika iindlela ezisebenzayo unyango?

syndrome Phospholipid: ntoni na?

Ngokuba lixesha lokuqala sifo iye yachazwa kutshanje. ulwazi esemthethweni ngaye zapapashwa 1980. Ekubeni ukusebenza wafunda wamathambo IsiNgesi Grem Hyuz, esi sifo udla ngokubizwa ngokuba Hughes syndrome. Kukho nezinye amagama - syndrome antiphospholipid, antiphospholipid lomzimba syndrome.

Syndrome Phospholipid - isifo samathambo apho amajoni omzimba iqala ukuvelisa antibodies ukuhlasela phospholipids umzimba ngokwakhe. Ekubeni le into zibandakanywa inwebu yeendonga ezininzi yeseli, kwaye sisilonda esidumbileyoesingaphakathi phantsi ukugula ezibalulekileyo ezifana:

• Izilwa-buhlungu ukuhlasela iiseli ezinempilo endothelial, ukunciphisa imiba yokukhula kunye Yindibanisela prostacyclin, nenoxanduva ukwanda emithanjeni. Ngokuchasene yangasemva sifo kukho kukwaphula zegazi udibaniso.
• Phospholipids nazo zifumaneka ezindongeni zegazi ngokwabo, nto leyo ekhokelela ekunyukeni udibaniso kunye intshabalalo ekhawulezileyo.
• Phambi omzimba kuba waphawula ukunyuka igazi ekunqandeni kunye buthathaka umsebenzi lweeHedfowuni.
• Inkqubo kantshabalalo ukuba siqo- kunye neeseli luvo.

Igazi iqala ukuba ihlwili imithambo yegazi, ukwakha egazi eziphazamisana igazi, yaye ngenxa yoko umsebenzi amasebe awohlukeneyo - ngoko ukuphuhlisa phospholipid syndrome. Oonobangela kunye iimpawu zesi sifo umdla kubantu abaninzi. Ngapha koko, kamsinyane esi sifo elibhaqiweyo, iingxaki ngaphantsi kubakho umguli.

Eyona engundoqo zokugula

Kutheni abantu ukuphuhlisa phospholipid syndrome? Izizathu inokuba nomahluko. Yinto eyaziwayo ukuba ngokufuthi kakhulu ogulayo yofuzo. Ukugula ikhula ukuba ukungasebenzi le amajoni omzimba, nto leyo ngenxa yesizathu esithile iqalisa ukuvelisa antibodies iiseli zomzimba ngokwakhe. Enoba kunjalo, esi sifo kufuneka into kwavusa. Ukuza kuthi ga ngoku, oosonzululwazi baye bakwazi ukuchonga ubungozi ezininzi:

• Phospholipid syndrome kudla senzeka phambi microangiopathy, ingakumbi trobotsitopenii, hemolytic-uremic syndrome.
• Imiba yomngcipheko ziquka ezinye izifo samathambo ezifana lupus zomzimba, vasculitis, scleroderma.
• Ukugula kudla senzeka phambi somdlavuza kumguli.
• Imiba yomngcipheko ziquka izifo ezosulelayo. A ingozi oluthile mononucleosis esosulelayo ne AIDS.
• Izilwa zinokuvela nakwesiphi DIC.
• Yinto eyaziwayo ukuba izifo ukuze aphuhlise kwizigulane abafumana amayeza athile, kuquka izicwangcisi hormone, psychotropics, "novokainamid" kunye t. D.

Ngokuqhelekileyo, kubalulekile ukwazi ukuba kutheni isigulane liqulunqe phospholipid syndrome. Afunyaniswe isifo kwaye afumane unyango kufuneka kuchongwa kwaye ukuba kunokwenzeka asuse unobangela sifo.

Ekubulaleni nemithambo yegazi xa phospholipid syndrome

Igazi kunye negazi izitya - ezi "ekujoliswe" yokuqala ochaphazela syndrome phospholipid. Iimpawu kuxhomekeke kwi ibakala esi sifo. egazi ngokuqhelekileyo uhlobo kwesebe yokuqala beempahla ezincinane. Batyumza umsinga igazi, nto leyo sikhatshwa kwezihlunu ischemia. Le nomlenze ochaphazelekayo kusoloko opholileyo ukuya ngaphandle kwebala, isikhumba ujika libunile kunye nezihlunu ngcembe yome. Imihla iyoluka ukungondleki inyama kukhokelela necrosis ukubola follow.

Chance zinzulu yalityatya vein thrombosis, nto leyo sikhatshwa ukudumba, iintlungu, ukuhamba kakuhle. Le syndrome phospholipid unako nzima thrombophlebitis (ukudumba udonga semithambo), ukuba ihamba ifiva, kuthibaza, isikhumba bomvu kwi sisilonda esidumbileyoesingaphakathi kunye ezibukhali, iintlungu elibukhali.

ukuyilwa egazi gazi imithambo yegazi enkulu kungakhokelela uphuhliso ezimbi zilandelayo:

• syndrome aortic (lukhatshwe kukunyuka okukhwankqisayo uxinzelelo kwi iimpahla nomzimba ongasentla);
• syndrome, superior Vena cava (ukuba imeko elinikiweyo luphawulwa nokudumba, cyanosis ulusu, wopha impumlo, noqhoqhoqho kunye kuvuleke iminxunya);
• syndrome le Vena cava obuphantsi (ikhatshwe isiphazamiso ejikeleza igazi inxalenye esezantsi lomzimba, yalityatya ngokwamathambo ukudumba, iintlungu emilenzeni, ngeempundu, emzimbeni kunye nasemiphakathweni).

Thrombosis kuchaphazela ukusebenza kwentliziyo. Ngokufuthi sifo ihamba kuphuhliso angina, lwegazi olungapheliyo, iposi engabalulekanga.

umonakalo izintso kunye iimpawu eziphambili

Thrombus Ukwakheka kukhokelela kwegazi, hayi nje kuphela kweli imilenze - kwaye eve enyama yangaphakathi, ingakumbi izintso. Uphuhliso lwexesha elide phospholipid syndrome kunokwenzeka ekuthiwa-iqhosha kwezintso. Le meko ihamba intlungu emva asezantsi, ukunciphisa ubungakanani umchamo kunye nobukho igazi kuyo of ukungcola.

Le ihlwili unokublokha kwemithambo kwezintso, nto leyo lukhatshwa iintlungu ezibukhali, isicaphucaphu nokugabha. Le yimeko eyingozi - ukuba ekhohlo lunganyangwa, ukuze ukuphuhlisa inkqubo necrotic. nemiphumo eyingozi phospholipid syndrome ziquka microangiopathy kwezintso apho amahlwili egazi ezincinane abunjwe ngqo kwi glomeruli kwezintso. Loo imeko kukhokelela kuphuhliso ukusilela ezingapheliyo kwezintso.

Ngamanye amaxesha kukho kwegazi kwi adrenal, nto leyo ekhokelela ukuba ukuphazamiseka kumanqanaba hormone.

Ziziphi ezinye izigqeba banokuchaphazeleka?

syndrome Phospholipid - sisifo esichaphazela amalungu amaninzi. Njengoko kukhankanyiwe, ii-antibodies kuchaphazela inwebu iseli neuronal, nto leyo Asinakuyenza ngaphandle imiphumo. Izigulane ezininzi ezikhala yintloko rhoqo olomeleleyo soloko ilandelwa lutshintsho lwemithetho nesiyezi, isicaphucaphu nokugabha. Kukho amathuba nophuhliso ukuphazamiseka kwengqondo ezahlukeneyo.

Ezinye izigulane kufuneka egazi sibuphethe ngezitya yegazi ezibonelela igazi kwemali ukuya analyzer optic. ukungabikho elide oksijini kunye nezakhi ikhokelela yoma le-luvo optic. thrombosis kunokwenzeka iinqanawa semehlo kunye legazi elandelayo. Ezinye ezimbi ocular, ngelishwa, ayibuyiseki: ezibonwayo sokunciphisa kuhlala sesigulane ubomi.

Kwinkqubo zophendlo lwezifo inokuba inxaxheba kunye namathambo. Abantu sivame ukuxilongwa kwamathambo ingathityazwa, nto leyo sikhatshwa deformation emathanjeni kunye waphuka rhoqo. Okunye esiyingozi necrosis aseptic ithambo.

Kuba ukugula kwaye zibandakanya kolusu. Ngokufuthi isikhumba kweqondo nomphezulu zikhabane kwasekwa spider emithanjeni. Ngamanye amaxesha uya kuqaphela nerhashalala uphawu kakhulu, nto leyo ufana, hemorrhages ezincinane ngokukhomba. Ezinye izigulane ukuphuhlisa erythema phezu kweentende zeenyawo kunye neentende. Kukho ongama hematoma Ukwakheka rhoqo (akubonakali unobangela) kunye nokopha phantsi isikhonkwane nembasa. ukuphazamiseka elide inyama trophic kuquka nezilonda elide okuphilisa nzima ukunyanga.

Siye safumanisa ukuba i phospholipid syndrome. Oonobangela kunye zesifo - imiba engundoqo. Emva kokuba zonke ezi zinto ziza kuxhomekeka kwirejimeni yonyango ugqirha ekhethiweyo.

syndrome Phospholipid: isifo

Kakade ke, kule meko lixesha kubaluleke kakhulu ukubona ubukho sifo. Umrhanelwa phospholipid syndrome, ugqirha wakho ingase ngexesha anamnesis. Tyhiliza kule mbono ukuba isigulane unako thrombosis kunye lwemithambo izilonda, isisu rhoqo, iimpawu kwegazi. Kakade ke, uvavanyo olunye olongezelelweyo.

Uhlalutyo onaso phospholipid iqulathe ekugqibeni umgangatho antibodies phospholipids egazini kwizigulane. Ngokubanzi, uhlalutyo igazi unokujonga ukwehliswa zegazi count, ukunyuka kweqondo neentlenge erythrocyte, ukwanda kwenani leukocytes. Amaxesha amaninzi syndrome ihamba igazi hemolytic, leyo lungabonakala kwizifundo elebhu.

Ngaphezu eyenziwa chemistry igazi. Isigulane sine-mali olongeziweyo globulin gamma. Ukuba ngezifo yangasemva yayonakele kwesibindi, kwandisa isixa yibilirubin egazini, kunye obrak phosphatase. Xa kukho isifo sezintso ibonakala ukwanda serum creatinine kunye urea.

Ezinye izigulane kunye nokucebisa ezithile iimvavanyo zegazi ngumzimba. Umzekelo, izifundo yemigaqo ukuze kubonwe into samathambo kunye lupus coagulant. Xa igazi syndrome phospholipid uyakwazi ukubona ubukho antibodies iiseli ezibomvu zegazi, ukwanda lymphocyte. Ukuba kukho urhano lomonakalo omkhulu ukuya isibindi, intso, ithambo, khona apho kuya kubakho iimviwo negalelo, kuqukwa X-ray, ultrasound.

Zeziphi iingxaki esi sifo esidityaniswe?

Ukuba lunganyangwa, syndrome phospholipid kungakhokelela kubunzima kakhulu yingozi. Ngokuchasene imvelaphi zesi sifo imithambo yegazi yabumba egazi, nto leyo kuyingozi. egazi kuthintela imithambo yegazi, ukuphazamisa kwegazi yesiqhelo - ancedisana amalungu abafumani izondlo ngokwaneleyo neoksijini.

Amaxesha amaninzi ngokuchasene nentsukaphi sifo, izigulane ukuphuhlisa stroke, kunye iposi engabalulekanga. omzimba Embolism ingakhokelela ekuboleni. Njengoko kukhankanyiwe ngasentla, izigulane waphawula ukuba ukungasebenzi izintso adrenal. Isiphumo ingozi a Embolism yemiphunga - le ngezifo luphuhlisa wayibona, kwaye hayi kuzo zonke iimeko, isigulane wasilela ukunikezela ngexesha esibhedlele.

Ukukhulelwa izigulane kunye phospholipid syndrome

Njengoko sele kukhankanyiwe, syndrome phospholipid banesifo ngexesha lokukhulelwa. Njani yingozi ukugula kwaye nomawukwenze kwimeko enjalo?

syndrome phospholipid Ngenxa sibuphethe ngezitya bakha egazi ukuba wenza ucingo imithambo ephethe igazi ngumkhaya. Umbungu akafumani ioksijini ngokwaneleyo kunye nezondlo, ngo-95% amatyala oku kukhokelela kwezisu. Nokuba ukukhulelwa aziphazamiseki, kukho umngcipheko abruption placental kwangethuba kunye nophuhliso kade yokukhulelwa, nto leyo eyingozi kakhulu kumama nomntwana.

Ngokusesikweni, umfazi kufuneka uye kuvavanyelwa ezingeni lokuhlela. Kunjalo phospholipid syndrome sivame ukuxilongwa ngexesha lokukhulelwa. Kwiimeko ezinjalo, kubaluleke kakhulu ukuba uqaphele ubukho sifo yaye sithathe amanyathelo ayimfuneko. Kuthintelo unina elizayo thrombosis linokukwenziwa ukuba anticoagulation ngeedosi ezincinane. Ukongeza, abasetyhini kufuneka rhoqo esweni ukuze ugqirha Ungaqala uqaphele abruption placental. Rhoqo emva kweenyanga ezimbalwa, oomama abakhulelweyo babacukucezela ekomelezeni unyango, ukuthatha amalungiselelo eziqulathe weevithamini, iiminerali kunye Antioxidants. Kunye nenqubo eyiyo, ukukhulelwa rhoqo iphela ngokukhuselekileyo.

Yintoni unyango?

Kuthekani ukuba umntu ufunyenwe phospholipid syndrome? Unyango kule entsonkothileyo mzekelo, kwaye oko kuxhomekeke kubukho xa isigulane yeengxaki ezithile. Njengoko imvelaphi sifo ifomu egazi, uthomalaliso lwajoliswa olindelwe igazi. Irejimeni yonyango kudla ngokuquka ukusetyenziswa amaqela ezininzi iziyobisi:

• anticoagulants amisiweyo Okokuqala amanyathelo ngqo, kunye arhente antiplatelet ( "aspirin", "Warfarin").
• Therapy ivame non-steroidal amachiza uyakhetha anti-yokukrala imvelaphi, ingakumbi i "Nimesulide" okanye "Celecoxib".
• Ukuba isifo lu dityanisiwe lupus zomzimba systemic kunye nezinye izifo samathambo, ugqirha unakho ukumisela unyango steroid (amachiza hormone anti-ukudumba). Kunye nale lungasetyenziswa, iziyobisi immunosuppressive ukuba gqiba umsebenzi amajoni omzimba kunye nokunciphisa imveliso omzimba ezinobungozi.
• maxa wambi abafazi abakhulelweyo yegazi alawulwa immunoglobulin.
• Izigulane rhoqo ngokuthatha amalungiselelo equlathe iivithamini kwiqela B.
• Impilo jikelele, ukukhusela inwebu zeselula kunye nempahla asebenzisa iziyobisi, antioxidants, kwaye kananjalo amalungiselelo leyo ibe oluntsonkothileyo fatty polyunsaturated ( "Omacor", "Mexicor").

Kuluncedo imeko yomguli kuchaphazela inkqubo electrophoresis. Xa kufikwa phospholipid eziziisekondari syndrome, kubalulekile ukulawula esi sifo zaseprayimari. Umzekelo, izigulane vasculitis kunye lupus kufuneka bafumane unyango olwaneleyo ezi ezimbi. Kwakhona ixesha kubalulekile ukubhaqa izifo ezosulelayo kwaye enze uthomalaliso ude recovery epheleleyo (ukuba kunokwenzeka).

Le abane ukuba izigulane

Ukuba phospholipid syndrome safunyanwa ixesha isigulane lafumana unonophelo kuyimfuneko, i-ALS nto intle kakhulu. Ukuphelisa esi sifo ngelishwa kunokwenzeka, kodwa amayeza uyakwazi ukulawula okubi yayo kunye nonyango lokuthintela of thrombosis. Ingqalelo imeko eyingozi apho sifo inxulunyaniswa thrombocytopenia kunye noxinzelelo oluphezulu lwegazi.

Kuyo nayiphi na imeko, phantsi kweliso wamathambo kufuneka kuzo zonke izigulane unesifo "phospholipid syndrome." Ngohlalutyo ngokuphindaphindiweyo njengoko kufuneka ulizalise rhoqo kanganani Kwathi lweemviwo abanye oogqirha ukuba amayeza kufuneka athathwe ukuze esweni imeko umzimba wayo siqu - konke oku kuya kukuxelela gqirha khona.