Pheochromocytoma - kuyini? Pheochromocytoma: izimpawu, uphethwe, ukuphathwa, izithombe

Kwakona nesenzakalo izimila emzimbeni womuntu, ngisho ingozi? Kuyinto kakade kwesizathu esiqinile sokukhathazeka. Futhi ukuze bayosingatha Eqinisweni okunjalo kumelwe ube fast kakhulu, kungenjalo babhekane nengozi yokuthola inyuka kakhulu. Ngenxa yalesi sizathu, kunengqondo ukuba bafunde izimpawu eziyisisekelo ukubukeka izimila, ikakhulukazi uma sikhuluma ifomu ezingavamile njengoba pheochromocytoma. Akufanele sicabange ukuthi imithelela umbono inkinga amakhambi abantu nezinye izindlela. I Ukuvakashela udokotela ikhambi enokwethenjelwa kakhulu.

Pheochromocytoma - kuyini?

Leli gama kumele aqondwe njengokufaka isimila sidalwe abulalayo noma kungavamile ukuba ingozi, akhiwa amangqamuzana chromaffin, okuyinto ukukhiqiza catecholamines. Kungaba ukukhiqiza kokubili ama biogenic futhi peptides okuyinto zihlanganisa norepinephrine futhi Dopamine.

Ezimweni eziningi (90%) asebenzisa lolu hlobo lwesimila ikhulela medulla adrenal, ingasaphathwa ukubukeka kwayo ilungiswe umzimba yomthambo i-aorta, ukhalo, lesisu noma umgodi thoracic. Ukuvela a pheochromocytoma ekhanda noma entanyeni esifundeni akunakubangelwa isigaba okuhlukile.

Ngo-10% kuzo zonke izimo lolu hlobo lwesimila kuyinto kwasendaweni abulalayo extraadrenal. Ngakho kukhona ukukhiqizwa Dopamine. Kepha inqubo Ukudlulela at inkinga enjalo, amaseli isimila iwe, ngokuvamile emaphashini, isibindi, amathambo, imisipha, futhi zesifunda zamanzi.

Izizathu ukuvela nokuthuthukiswa

Abaningi baye bezwa ngalesi sifo njengoba pheochromocytoma. Yini-ke, ngeshwa, akubona bonke uyazi. Kubalulekile ukuqonda ukuthi kungani inkinga enjalo kwenzeka emzimbeni womuntu. Kodwa-ke lapho kufanele kuphawulwe ukuthi impendulo ecacile esibanzi mayelana imbangela okwamanje. Kodwa ngenxa izifundo baye benziwa eziphethweni eziqinisekile mayelana nemvelo kwezakhi pheochromocytoma.

Ngokuvamile lesi sifo kwenzeka kubantu abaneminyaka engu-25 kuya ku-50. Ngokuyinhloko, ukuthuthukiswa lolu hlobo lwesimila iqinile e abesifazane asebeqinile abaneminyaka. Uma sikhuluma nezingane, kokutshelwa ngokukhubazeka kwengane mkhulu kubafana. Kodwa ngokuvamile, pheochromocytoma isimila - into abavamile.

Ngakho mayelana 10% inani eliphelele lezinkinga ezinjalo iphathelene ifomu kwamalungu omkhaya. Lokhu kusho ukuthi lolu hlobo lwesimila wayekhona okungenani oyedwa kubazali isiguli.

Eqhubeka sihlole izimbangela zalesi inhlobo yesifo, kuyafaneleka ukuthi kungaba kwenye yezingxenye yalesi sifo multiple endocrine neoplasia (uhlobo 2A kanye 2B). Pheochromocytoma ilungiswe 1% eziguli ukuthi nginesifo diastolic blood pressure.

symptomatology

Esifundweni izimila ezifana pheochromocytoma, izimpawu, ukuxilongwa kanye nokwelashwa osemqoka Imininingwane amabhlogo, okukuvumela ukuba ucabangele isihloko ngezindlela ezihlukahlukene. Kodwa kufanele uqale nge izenzo sifo. Symptomatology wehlukile ngempela futhi ngokuvamile ifana izinkinga zezempilo ngaphansi yingozi.

Kufanele uqale neqiniso lokuthi kukhona omunye uphawu okungukuthi esigculisayo isifo esinjengo pheochromocytoma. Kwegazi - lezi ezinye izenzo isimila embuzweni. Kungathatha indawo ifomu ozinzile futhi krizovoe. Lapho isimila eba at catecholamine crisis unesifo sikashukela, umfutho wegazi inyuka ngempela ngokucijile. Kodwa esikhathini mezhkrizovy BP angahlala njalo okusezingeni eliphezulu noma ibe uhla evamile. Eqoshiwe izimo lapho pheochromocytoma siqhutshwele phambili njalo umfutho ophakeme, kodwa ngaphandle ngesikhathi sezinhlekelele.

Ukuze uthole ukwaziswa okwengeziwe mayelana Stroke

Kunengqondo ukuba banake yokuthi nenkinga efana pheochromocytoma crisis (hypertension) ngokuvamile kuhambisana emathunjini, senhliziyo, umzimba kanye iziyaluyalu Neuro-zengqondo. Ukukhuluma mayelana nokuthuthukiswa esesimweni esinjalo, kufanele kuphawulwe ukuthi libhekene zimpawu ezilandelayo: ikhanda, liqhaqhazela, ukukhathazeka, ukungahlaliseki, isikhumba ugodo, ukuhlanza, amajaqamba futhi ukujuluka. Kungase kube ukuhlukumeza isigqi, tachycardia nobuhlungu benhliziyo.

Lapho ethola ukuthi unesifo pheochromocytoma, crisis unesifo sikashukela kungase kumkhathaze ukuhlanza, isicanucanu nomlomo owomile. Kodwa lezi zinguquko esimweni lesiguli zempilo akuyona entweni eyodwa njalo. Nge izimila enjalo ngokuvamile ekushintsheni isimo igazi. Sikhuluma yezinkinga ezifana lymphocytosis, Hyperglycemia, leukocytosis, futhi eosinophilia.

Crease akwazi ukuhlala isikhathi eside kusukela imizuzu embalwa ihora noma ngaphezulu. Ukuze lesi sici state ukuvala shaqa yayo ezingalindelekile. Nciphisa wegazi futhi kwenzeka ngokushesha, ukuze ngisho kukhona ingozi hypotension. Njengoba izimpawu avuse izinto ezifana angenza umsebenzi, enifeza palpation ezijulile esiswini, utshwala nezidakamizwa, hypothermia noma okweqile, ukunyakaza abukhali, ukuphazamiseka ngokomzwelo nezinye izinto.

ikakhulukazi ukuhlaselwa

Ukuquleka bavame ukuba imvamisa ehlukene. Bangakwazi wazizwa izikhathi 10 kuya ku-15 ngosuku noma kanye ngenyanga. Uma ukuqala esibucayi ezinzima e pheochromocytoma ke kunengqondo ukukhuluma mayelana ingozi yangempela yokushaywa unhlangothi, ukopha retina, ukwehluleka kwezinso, isilethikusebenza, sokuhlaziya useshonile le-aorta, edema yamaphaphu, nabanye. Uhlelo inkinga olunzima kunalo lonke ake kulesi simo kuyinto ukushaqeka catecholamine kuzibonakalisa ngu ongalawuleki hemodynamics. Sikhuluma ekushintsheni hypo- kanye nomfutho wegazi ophezulu, kokuba yokubulala okungakhethi nemvelo futhi ayikwazi ukulungiswa.

Ngakho, pheochromocytoma - kuyini? Uma ucabangela ubufakazi baso, kufanele alalelwa abesifazane abakhulelwe, ngoba isimila enjalo kungaba azenza eclampsia, pre-eclampsia futhi toxaemia. Umphumela izimpawu kanjalo ingaba enqabayo lokuzalwa emiphumeleni.

umumo Okungenzeka oqine yesimila, lapho ingcindezi igazi njalo okusezingeni eliphezulu. Kuleli zinga, ngokuvamile eqoshiwe izinguquko izinso, fundus, futhi myocardium. Kungenzeka futhi ngokuphazima kweso lapho isimo, ubuhlungu bekhanda, ukukhathala kanye nobuhlungu. Omunye iziguli 10 babe ukuthuthukiswa sikashukela.

Kukhona ezinye izifo zona zivame ezihlobene pheochromocytoma. Kumayelana Raynaud Syndrome, isifo gallstone, syndrome Cushing, neurofibromatosis, nabanye. Esimweni lapho asathuthuka isimila esibulalayo, ubuhlungu esiswini, kukhona Ukudlulela kwezitho ezikude, futhi ngokushesha kwesisindo.

ukubonakaliswa emitholampilo Specific

Kukhona izici ezithile zalesi sifo, okungaholela abanye izinkinga. Kumayelana izinqubo ezilandelayo:

• Abanezingane okwatholakala pheochromocytoma ezimweni eziningi eba nomfutho wegazi ophezulu. Ikota iziguli zisencane kuyinto polydipsia esinqunyiwe, polyuria, futhi nokuquleka. Enye inkinga ukuthi kungabangela pheochromocytoma ezinganeni - a Ukwehla ekukhuleni. Ngaphezu kwalokho, kwaba zisencane ne uphethwe izenze wazizwa oqinile kwesisindo, ukujuluka, isicanucanu nokuhlanza, ukuphazamiseka visual, futhi vasomotor thuthuva.
• Abesifazane kanye pheochromocytoma bangezwa Ukuwohloka impilo ngenxa namagagasi. Abesifazane abakhulelwe kungase kube silingisa eclampsia futhi pre-eclampsia.
• Ezimweni lapho isimila itholakala eduze odongeni isinye, ukuchama lingaphathelaniswa ne nokuquleka. Kukhona ingozi hematuria kobuhlungu, kukhuphuke blood pressure, ikhanda elibuhlungu, isicanucanu futhi palpitations.

izindlela ukuxilongwa

Ukuze ukukhomba ngezimo zesiguli nge isifo kusolwa ezifana pheochromocytoma, ukuhlaziywa isimo umzimba kufanele udzimelela zimpawu ezilandelayo: paleness kwesikhumba ebusweni amabele, kukhuphuke blood pressure, tachycardia, hypotension orthostatic.

Ngakho kubalulekile ukuba palpation ngokucophelela umzamo esimweni izimila entanyeni, noma isisu. Iphuzu eliyinhloko ukuthi izenzo ezinjalo kungabangela ukuthuthukiswa catecholamine crisis.

Futhi, eliphezulu uphethwe ngo izimila ezinjalo kufanele kufake evakashele ophthalmologist. Lesi simo singaba sithi iqiniso ukuthi cishe u-40% abantu abaphethwe umfutho wegazi ophakeme, retinopathy abanomfutho wegazi ophakeme lutholwa, futhi obuhlukahlukene zaba namandla kakhulu.

Kukhona isimo sokunquma kwamakhemikhali for sitholakele pheochromocytoma, isithombe okuyinto sikwazi ukuqondisisa kahle ukungathí sina kwaleso simo. Ngakho sikhuluma amazinga anda catecholamines egazini, umchamo, igazi glucose chromogranin-A egazini serum. Ngezinye izikhathi kungase kube idatha efanele on ukwanda lokuhlushwa i-hormone parathyroid, cortisol, calcium, phosphorus, calcitonin, nezinye izakhi.

Ngaphansi kwezimo ezithile, iziguli Kwenziwa umehluko ukuxilongwa izimila. Sikhuluma izifo ezifana sengqondo, neurosis, umfutho ophakeme wegazi, i-hyperthyroidism, tachycardia paroxysmal, ubuthi kanye ukushayisana kwe-CNS.

Ekunqumeni uhlobo isimila kanye degree sokuthuthuka ingasetshenziswa MRI nakuma-CT, kanye cwaningo womsebenzi lesiguli inhliziyo. Uma pheochromocytoma sitholakale, izimpawu, ukuxilongwa kanye nokwelashwa - izici ezihambelana kakhulu njengoba e izimila ezihlukahlukene is ukuthola esifike izinkinga nokwelashwa ngokushesha kuthiwa ukhiye kube nomphumela omuhle umsebenzi nesiguli.

Indlela esiphatha ngayo

Zikhona izindlela ezehlukene kwethonya isimila njengoba okubulalayo nokuhlala hhayi usongo. Ezimweni eziningi, ukuhlinzwa kuyoba isinqumo okunengqondo kunazo, uma kwatholakala pheochromocytoma. Ukususwa kuyoba efanele ikakhulukazi uma isimila kule ndlala adrenal.

Kodwa kungase kube okuhlukile ngenxa Isimo esibi inhliziyo, imithambo yegazi kanye nomfutho wegazi ophakeme. Ukubamba iqhaza udokotela ohlinzayo lapho isimo esinjalo kubaluleke kakhulu engathandeki noma engamukeleki. It kuvela okwengeziwe umthelela isimila ezifana pheochromocytoma. Ukwelashwa kuyehla kube yokwelapha izidakamizwa, ngokuvamile kuthatha izinsuku ezimbalwa.

Ukusetshenziswa kwezidakamizwa ezahlukene isimila neutralization kusho ukuthi isiguli kuyoba embhedeni, futhi uthathe isikhundla isigamu Ukugada. Ukuletha udokotela evamile blood pressure angabeka-alpha-blocker. Ngaphezu kwakho konke, kungcono izidakamizwa ezifana "Tropafen" futhi "phenoxybenzamine." Uma isiguli iqinile crisis abanomfutho wegazi ophakeme, abaphathi nezokusigcoba babeyoba "phentolamine" "Sodium nitroprusside" nezinye izidakamizwa ezifanayo. Okuqakathekileko yokuthi phakathi pre-ukwelashwa kuyadingeka ukugcina isiguli kusukela amanzi emzimbeni.

Ukusebenzisa ukuhlinzwa

Uma pheochromocytoma itholiwe, ukuphathwa besebenzisa nommese ikhethwa ngokuphathelene nalokho inqubo oluthile lwesimila. Okuvame kakhulu ehlangana, transbryushinny, extraperitoneal nezindlela transthoracic.

Esimweni ukuthi ungubani isimila kwatholakala ngemva luhlolo, onalesi sifo nokwelashwa kuhlinzwa kuzokuzuzisa. Yiqiniso, kungase kube umuntu uyahluleka uma elwa, kodwa azibonakali kuphela 13-15% of amacala.

Kodwa lapho kukhona izimila eziningi, isimo luyindida kakhulu ngaphezulu. Isixazululo enengqondo kunazo zonke iwukuthi ukususa zonke izimila, kodwa inkinga itholakala lokuthi sokuthi ngisho namathuba okuba nomphumela ophumelelayo kakhulu. Kungenxa yalesi sizathu ukuthi esimweni pheochromocytomas amaningi ukuhlinzwa wenziwa izigaba eziningana. Kwezinye izimo, kuphela isimila nendlala okuthiwa i sisuswa kancane.

Njengoba for izici zomsebenzi, ngokuhamba nokuqaliswa kwalo iyosebenza ukufinyelela kuphela laparotomy. Yayichaza le ndlela ephezulu isimila extraadrenal kwasendaweni. Kubalulekile ukwazi ukuthi ukulawula hemodynamic (blood pressure kanye central ingcindezi emithambo yegazi) is egujwa kokungenela.

Ezimweni eziningi ngemva pheochromocytoma isuswe, umfutho wegazi incipha. Uma ukusabela umzimba okunjalo engekho, khona-ke zonke isizathu sokusola yokuthi kwetinhlavu ethunjini isimila nezicubu.

Uma pheochromocytoma itholwe kwabesifazane abakhulelwe, okokuqala wenza kuqiniswa wegazi, bese wenza isigaba cesarean, noma isisu, futhi kuphela bese ususa isimila uqobo.

Endabeni ka ekuxilongweni a pheochromocytoma abulalayo, lapho ukusatshalaliswa metastases ukuthola imiphumela emibi yokwelashwa ngamakhemikhali ( "Dacarbazine" "Vincristine", "Cyclophosphamide").

Ngokuvamile, uma isimila itholiwe ngendlela efanele usaqala, kanti isiguli kulungiselela ukwelashwa, amathuba ukuba unqobe le nkinga abasakwazi okusezingeni eliphezulu.

Iziphi izici ingachazwa njenge-trigger

With ukuthuthukiswa izimila emzimbeni njalo ingozi ingqondo ukuhlaselwa. Ngakho-ke kubalulekile ukuba uziqaphele, okungase kuholele ekubeni uthunyelelwe okubi kakhulu isimo. Kuyinto umbuzo mayelana nobuhlobo bobulili, izinguquko emzimbeni ukuma, hyperventilation, ukunwebeka kwemisipha ngesikhathi wamathumbu ukunyakaza, umthwalo ngokomzimba, excitation engqondo futhi ingcindezi endaweni lapho yenziwe yaba yasendaweni lwesimila. Uma kutholakala ukuthi pheochromocytoma, musa ukuhileleka utshwala, ikakhulukazi iwayini ubhiya, kanye ukudla ushizi.

Kukhona futhi atloliwe izimo lapho ukuhlaselwa kwaqala ngemva kokwamukela i-nicotine, tricyclic antidepressant, phenothiazine nemikhiqizo ne-beta-blocker.

Imiphumela

Ngakho sinesizathu kwengqikithi ethi "Pheochromocytoma" wakusho. Kuyini, sabuka nomniningwane kakhulu. It uhlala ukwenza Isiphetho esisobala: inkinga sisikhulu kakhulu ukuba aphathe it kancane. Ngesikhathi izimpawu zokuqala abafana izimpawu pheochromocytoma, kubalulekile ukuba kudokotela uyofuna icebiso, kube ukuhlolelwa lesi sifo futhi uqale ukwelashwa.