Peutz ciwo - Jeghers - Touraine: Description, Alamun kuma Jiyya

Peutz Syndrome - Jeghers - Touraine - rare kwayoyin cuta lalacewa ta hanyar maye gurbi na gene STK Nuwamba 19 th chromosome. watsa ne autosomal rinjaye.

labarin

A 1921, lokacin da aka fara bayar da rahoton yanayin da cutar, a yanzu aka fi sani da Peutz ciwo - Touraine - Jeghers. Description an sanya ta Dutch likita Johannes Peutz, karatu da ciwo Dutch iyali misali. A shekarar 1949, ya kimiyya bincike ya kara da cewa wani American likita Harold Jeghers.

symptomatology

Peutz - Jeghers ciwo ne halin da girma a cikin gastrointestinal fili gamartomnyh polyps (Saboda haka wani sunan wannan cuta - gamartomny polyposis). Suna mafi yawan samu a ko babba hanji, mafi wuya - a ciki da kuma dubura. Da farko, polyps bai sa kansu a san, amma kamar yadda suke girma akwai maƙarƙashiya, bloating, rectal na jini, latsa zafi a ciki. Game da rabi na marasa lafiya fuskanci rikitarwa kamar hanji mai shãmakacẽwa. Boye zub da jini mai hadarin gaske saboda ba za su iya kai wa ga anemia, saboda haka, janar wani rauni, juwa ko jiri, rage yadda ya dace. Marasa lafiya ba kawai kula da halayyar bayyanar cututtuka da cutar - m zawo da kuma ciwon mara, kamar yadda wannan na iya zama wata ãyã daga wasu narkewa kamar cututtuka. Amma da ciwo Peutz - Jeghers iya kai wa ga sosai tsanani sakamakon da in babu magani, saboda haka kada ku yi shakka a ziyarci wani gwani.

Brown spots a kan fata, mucous membranes (lebe, dabino, tafin kafa wa idanu) kuma yayi magana game da m gaban da ciwo Peutz-Egersa.

A spots bayyana a wani wuri shekaru, wani lokacin jariran. Su ne lebur, duhu launin ruwan kasa, jere a size daga 1 zuwa 5 mm. A kan lokaci lebe spots iya brighten da Fade, amma sun kasance har abada a cikin bakinka. Wannan shi ne wani muhimmin alama a cikin ganewar asali, tun 98% da marasa lafiya da ciwo na Peutz - Jeghers da pigmentation na mucous membranes.

Tun da ciwo Peutz - Jeghers - Touraine - a kayyade cuta a ganewar asali gwani dole ne ya yi la'akari da tarihi na duk 'yan uwa. Idan daya daga cikinsu ya sha wahala daga wannan cuta, to, duk dangi, suna da mutated gene.

bincikowa da

Gane asali ciwo Peutz - Jeghers via biopsy. Idan dauka domin bincike na polyp aka samu gamartomnaya bangaren, shi ne na hali alama da wannan cuta. Polyps jere a size daga 1 zuwa 5 mm kullum ba su tsoma baki tare da al'ada aiki na gastrointestinal fili. Amma kamar yadda suke girma, ba za su iya kai wa ga zub da jini, domin nodules cewa ne ya fi girma fiye da 1 cm, dole ne a cire. Domin polyps ne halin da matsakaici girma, za su iya zama duka biyu da mahara da kuma guda. Lokacin da mahara jiyya fiye da wuya. Su ba za a iya cire zarar amfani saboda wani haske da abinci da kuma magani far da nufin slowing da girma da siffofin maruran.

Sauran muhimmanci sharudda ga ganewar asali - gadar hali da kuma pigmentation na mucous. Saboda polyps ake samu a marasa lafiya girmi shekaru 10, yara a pigmentation na mucous ne babban alama a ganewar asali. Petz - Jeghers ciwo ne ma sau da yawa tare da wani ciwo na Mac-Kyung - Albright (farkon jima'i ci gaba). Idan yaro kafin balaga, da alama na bunkasa shi gamartomnogo polyposis ne quite high.

Kasadar ciwon daji

Marasa lafiya tare da ciwo na Peutz - Jeghers muhimmanci mafi girma hadarin tasowa ciki ciwon daji fiye da a da lafiya mutane. Mafi sau da yawa, Oncology rinjayar da manyan hanji, kananan hanji da kuma pancreas. Mata sau da yawa lura da nono Oncology (45% na lokuta). Yana da wani babban Yiwuwar tasowa da kuma ciwon daji ne da hatsarin cuta, Peutz - Jeghers ciwo kamata a sarrafawa via da dama na asibiti hanyoyin.

Ganin ƙãra hadarin ciwon daji, dukan marasa lafiya ya kamata sha X-ray na kananan hanji game da sau daya a kowace 2 shekaru.

Gastroscopy da colonoscopy zai taimake ku likita saka idanu da yanayin da ciki da kuma hanjinsu, kazalika da saka idanu da girman da polyps. Ko da tare da nasara tiyata don cire polyp alama na komawa ne high, domin wadannan dubawa ne cikakken zama dole.

Mata daga shekaru 25 da bukatar wani mammogram kowace shekara don tsayar Oncology a wani wuri mataki.

A ganewar asali da ciwon daji taka muhimmiyar rawa a cikin bincike na gaban da jini carcinoembryonic antigen. Wannan sinadaran abu samar lokacin da hanji da ciwon daji, da ciwon daji na nono, ko kuma huhu.

Har ila yau muhimmanci ga definition wasu ciwon daji antigens (kuma aka sani da ƙari antigens) - abubuwa samar a cikin kamuwa Kwayoyin na wani sashin jiki Oncology. Da hali ya dogara da wuri daga cikin ƙari, wanda yake shi ne dalilin da ya sa wadannan abubuwa suna taka muhimmiyar rawa a cikin ganewar asali.

Mafi yawan marasa lafiya Peutz Syndrome - Egersa ba su isa shekaru 60, mutuwa na pancreatic ciwon daji (11%), ciki (57%), ciwon (85%), nono (45%). Dan kadan ƙãra hadarin tasowa huhu ciwon daji, testicular, mahaifa da ovarian. Idan Oncology gano a wani marigayi mataki, shi ne m. Wannan shi ne dalilin da ya sa yana da muhimmanci dace ganewar asali.

magani

Duk da cewa baya a 1921, ya bayyana Peutz ciwo Peutz - Jeghers - Touraine, cututtuka da kuma lura da wannan cuta ne har yanzu ba kyau gane. A halin yanzu m magani da wannan cuta ba a ci gaba. Tun da ciwo a cikin hanjinsu girma daruruwan polyps, prophylactically cire duk su ne ba zai yiwu ba saboda babban hadarin da irin wannan aiki. Saboda marasa lafiya tare da ciwo na Peutz - Jeghers rayuwa kawo mai yawa tiyata don cire polyps a lokacin da suka isa wani m size.

sakamakon

Ko da yake gaba daya warke daga cutar ba a halin yanzu zai yiwu, miyagun ƙwayoyi far zai muhimmanci rage gudu da girma da polyps, normalize hanji aiki da kuma ciki. Ganewar asali a wani wuri mataki na ciwon daji kuma qara da dama na magani bayan ƙari kau.