Klippel-Trenaunay syndrome: ifoto, iimpawu, unyango

izifo Okuninzi Ukuba kwisininzi kuyilwa ixesha osandul. Ngoko ke, impembelelo kuphuhliso lwabo abakwazi njalo. Ngapha koko, uninzi lwala makhwiniba abafunyenwe kuphela emva kokuzalwa komntwana. Kwaye abanye kamva. Ku sesinye sezifo genetic kufuneka ziquka isifo Klippel-Trenaunay. Esi sifo lwafumaneka kwinkulungwane yama-20. Oku kunganxulunyaniswa ukuvela nenzululwazi imizila yemfuza. Ngokufuthi sifo ento sifo. Nangona kunjalo, oku kwakhona kwafunyanwa phakathi kwabantu ababhinqileyo. Pathology zibangelwa ukuphazamiseka bekhubazekile nemithambo.

syndrome, Klippel-Trenaunay Inkcazelo

Klippel-Trenaunay syndrome kugqalwa siphene yemfuza ezinqabileyo. Qikelela uphuhliso le ngezifo phantse nzima, kuba isiphako inqabileyo kwaye akukho kwafunyaniswa ngexesha lokukhulelwa. Noko ke, kwezinye iimeko, esi sifo ingakrokreleka Klippel-Trenaunay. Esi sifo ibhekisa sisifo njengelifa. Ngoko ke, uhlalutyo yemfuza kumele kunyanzeleke ukuba abasetyhini abakhulelweyo kwintsapho apho kukho ukuqhambuka kwesi sifo kwenzeke. Elinye igama isifo - hemangiectasia hypertrophic. Lo mfanekiso lwezonyango Klippel-Trenaunay syndrome kuquka iimpawu ezintathu eziphambili:

1. mithambo Varicose.
2. Imbonakalo amabala ubudala apha esikhumbeni.
3. Ukutshintsha ububanzi nobude mlenze achaphazelekayo.

Kwezinye iimeko abantu skeletal kunye soft izihlunu hypertrophy uyalandelwa. Amaxesha amaninzi pathology luphawulwa ngekhondo progressive. I-ALS ixhomekeke ebukhulwini iinguqu nemithambo. Nangona unobangela sifo akayi lupheliswe, amanyathelo zonyango ukunceda ukuphucula umgangatho wobomi isigulane. Abantu abanengxaki kwesi sifo unikwe ukukhubazeka.

Oonobangela Klippel-Trenaunay syndrome

Ngesizathu esithile, kukho isifo Klippel-Trenaunay? Le syndrome ngenxa yomonakalo yemfuza. Le anomaly ezidluliselwa an autosomal uhlobo ilifa lubalaseleyo. Oku kuthetha ukuba xa kukho isiphako yemfuza hlobo omnye wabazali, kukho amathuba sifo ngo umntwana yi-50%. Kwakhona, esi sifo ezosulela isizukulwana mnye, oko kukuthi - nomkhulu. Kulo mzekelo, linokuba yi-25%.

Njengabo bonke makhwiniba yemfuza, iziphene nemithambo ukuphuhlisa ngexesha ibhukmakhi inyama Palsy. Kungenzeka kuchaphazela iziphumo umonakalo. Le ibandakanya imibandela efana nale:

1. kabi Psychological kwiiveki zokuqala ezimbalwa zokukhulelwa.
2. izifo ezosulelayo. Okubaluleke kakhulu ke amanxeba ezingapheliyo wentsholongwane egazini komzimba.
3. Ukusetyenziswa kweziyobisi kunye iipropati yaManzi naMahlathi.
4. iziyobisi Ukwamkelwa nje kokuba akhulelwe ngexesha lokukhulelwa.
5. Ukutshaya kunye nokusela utywala.
6. Ungcoliseko lomoya zizinto eziyityhefu, radiation.

Zonke ezi zinto kunokubangela utshintsho emzimbeni kwabasetyhini kunye nomntwana engekazalwa. Bona yingozi ingakumbi xa nisaqala yokukhulelwa. Emva mgaqweni ezahlukeneyo olusuka kweli xesha.

Indlela kwesifo

Kutheni kukho utshintsho nemithambo izifo Klippel-Trenaunay? Syndrome ubonisa iimpawu ukuphazamiseka lwemithambo. Indlela yophuhliso leengxaki ngokubhloka yayikukuphuphuma igazi kwiziphelo asezantsi. Oku kubangelwa yinto yokuba le mithambo lwemithambo na imithambo, kukho ucinezelo benqanawa yesebe esimbaxa, izihlunu hypertrophied, izicwili intsalela osandul. Ngenxa sanctions nencindi igazi kukho lwegazi. EVienna ebegqwese thinned, boniswa umonakalo. Ukongeza, ephazamisa ukusebenza izixhobo vana. Ngenxa stasis igazi lomzimba eqalisa hypertrophy. Ngenxa kokoma ubunzima umsebenzi trophic. Ngoko ke, ithishu boniswa ischemia. ukuphazamiseka elide igazi kukhokelela sclerosis. kwizihlunu ezithambileyo hypertrophy kwenzeka hyperemia kunye nokudumba. Kwezinye iimeko, kuxhomekeke ukutshintsha namathambo. Ngenxa yoko wothuka xa lomzimba akufunekanga ukwandisa kuphela ububanzi, kodwa ndoyolula.

Klippel Trenaunay Syndrome-: iimpawu

Ngu zokubonisa Klippel-Trenaunay syndrome ziquka utshintsho kungekuphela na inxalenye yenkqubo lwemithambo, kodwa ezinye iziphene. Nangona isibakala sokuba engeyoyesiqhelo enaso, iimpawu yokuqala zingavela elivisayo okanye ebudaleni. Ngenxa yoko, wabonakala lonyango ziquka iintshukumo zokwaphulwa kwemithetho zilandelayo:

1. Komlomo emithanjeni umlenze phezulu. Kubonisa kinking zophendlo lwezifo kunye nezazo isitya zibonakale ukuya ngeliso lenyama. Kwiimeko ezininzi imithambo varicose ibekwe phezu olungaphandle imilenze.
2. Ukwanda isixa lomzimba. Soloko ilandelwa lutshintsho lwemithetho iziganeko arhabaxa. Ngenxa stasis igazi umlenze ochaphazelekayo inokuba nomahluko kakhulu ukusuka mlenze esempilweni. Idla ngokuba hyperemic, isikhumba eqinileyo.
3. Lomzimba yokwandisa. Hypertrophy angakwazi zombini iinyawo nezandla.
4. Imbonakalo nevi ulwehlo apha esikhumbeni. Ngenye indlela, le ngokobuninzi zibizwa ngokuba "amabala iwayini".

Kwakhona, kunye ukuqhubela phambili kwesifo kuvela utshintsho trophic. iziphene sisilonda lunokwenzeka kwimimandla echaphazelekayo, yeyiphi na ukuphilisa ixesha elide. Phakathi kwezinye ukungaqheleki asakhasayo kulo ngezifo, kukho polydactyly, ubukho ngeminwe oqhagamshelwano phakathi edidekile, okanye iinzwane.

Indlela ukufumanisa isifo Klippel-Trenaunay?

Syndrome Uxilongo of Klippel-Trenaunay lusoloko lusekwe phezu ekubonakalisweni onyango. Iimpawu zesi sifo kuvela ngcembe. Uphawu lokuqala isenokuba ukuvela 'amabala zewayini "okanye mithambo. Ukuba ezi mpawu zidibene ne hypertrophy alo mlenze kwabenza kwesifo: Klippel-Trenaunay syndrome. Izithombe zabantu abanengxaki kwesi sifo iyafana omnye komnye. Baya kunokubonwa uncwadi eyayinikelwe ezimbi genetic ezinqabileyo. Ngoko ke, inkoliso uphethwe ayikho nzima.

Ukongeza data yezonyango, wenza venography, ultrasonography le nemilenze,-X-reyi.

Klippel Trenaunay Syndrome-: unyango lwesifo

umsebenzi uthathwa isiganeko ezinkulu zonyango. Umthamo longenelelo emiselwe iqondo sisilonda esidumbileyoesingaphakathi yegazi. Ukwandisa mithambo le stenting vein luyenziwa. Xa indawo enkulu intshabalalo kuvelisa isitya bypass - uze umisele indawo kweyodwa.

Ukuphelisa varicose skleroteropiya amisiweyo, laser coagulation le mithambo. Izigulana na kwesi sifo kufuneka banxibe ucinezelo sangaphantsi.

syndrome, Klippel-Trenaunay Prevention

Klippel-Trenaunay syndrome kwiintsana lunqabe sifo. Ngokufuthi oko kubonakele kwiinyanga zokuqala okanye iminyaka yobomi. Ukuthintela isifo, oko kuyimfuneko ukuze azikhusele neziphumo eziyingozi xa ukhulelwe. Enye umlinganiselo othintelo kuthathwa ukuba kube luviwo genetic Umbungu phambi kumatyala izifo entsatsheni.