Pheochromocytoma - ntoni na? Pheochromocytoma: iimpawu, uxilongo, unyango, iifoto

Isibakala ingenzeka yamathumba emzimbeni womntu, nokuba ebubonisayo? Sele sizathu sivakalayo inkxalabo. Kwaye ukuba uphendule yinyaniso onjalo kufuneka ngokukhawuleza kakhulu, kungenjalo ukujamelana ingozi kubunzima ukunyuka kakhulu. Ngenxa yesi sizathu, iyavakala ukufunda imiqondiso engundoqo inkangeleko yamathumba, ingakumbi xa sithetha ngendlela kunqabile pheochromocytoma. Asimele ingqalelo iziphumo injalo kwi ingxaki amayeza esizwe kunye nezinye iindlela ezizezinye. Utyelelo kugqirha na isisombululo inokuthenjwa.

Pheochromocytoma - ntoni na?

Eli gama kufuneka iqondwe njengoko thumba olubulalayo okanye ingozi uhlobo, eyakhiwa iiseli chromaffin, ekuthi kwezo nto kuphume catecholamines. Inokuvelisa zombini amine biogenic kunye peptides eziquka norepinephrine kunye dopamine.

Kwiimeko ezininzi (90%) yale hlobo ithumba senzeka kwi Medulla adrenal, ngaphantsi kakhulu imbonakalo yayo ilungiswe emzimbeni aortic, isinqe, esiswini okanye kwisisu thoracic. Ukuvela pheochromocytoma entloko okanye emqaleni kummandla kunganxulunyaniswa kudidi lwe ngaphandle.

Ngowe-10% yazo zonke iimeko olu hlobo Ithumba a lizwe amabi extraadrenal. Ngenxa yoko kukho imveliso dopamine. Njengokuba inkqubo Ukudlulela kwi ingxaki enjalo, kuwa iiseli ithumba, ngokuqhelekileyo emiphungeni, isibindi, amathambo, izihlunu, neendawo nkovu lwengingqi.

Izizathu ukuvela kunye nophuhliso

Abaninzi baye beva ngale isifo pheochromocytoma. Ntoni na, ngelishwa, ukwazi oko akubantwini bonke. Kubalulekile ukuqonda ukuba kutheni kwenzeka ngxaki emzimbeni womntu. Kwaye ngoko xa kufuneka kuqatshelwe ukuba impendulo ecacileyo ebanzi malunga unobangela okwangoku. Kodwa ngenxa yokuba izifundo ziye kwenziwe izigqibo ethile malunga nendalo yemfuza pheochromocytoma.

Isikakhulu sifo kwenzeka abantu abaneminyaka eyi-25 ukuya ku-50. Ngokusisiseko, uphuhliso olu hlobo Ithumba sisigxina embindini-abaminyaka abafazi. Xa sincokola ngayo abantwana, uyayazi loo inkulu amakhwenkwe. Kodwa ngokubanzi, pheochromocytoma ithumba - a amila kakhulu.

Ngoko malunga ne-10% yenani lilonke iingxaki ezinjalo inxulumene kwifom kwiintsapho. Oku kuthetha ukuba olu hlobo ithumba wayekhona ubuncinane omnye wabazali yesigulana.

Ukuqhubeka ukuhlola oonobangela olu hlobo sifo, kubalulekile ukukhumbula ukuba yenye amacandelo esi sifo of ezidalwe neoplasia ezininzi (uhlobo 2A kunye 2B). Pheochromocytoma fixed-1% ezigulane une uxinzelelo lwegazi ukuzala.

symptomatology

Xa isifundo yamathumba ezifana pheochromocytoma, iimpawu, isifo kwaye afumane unyango, ezi zinto zizitena iinkcukacha ezingundoqo, nto leyo evumela ukuba siqwalasele isifundo kwiikona ezahlukeneyo. Kodwa kufuneka uqale ezibonisa sifo. Symptomatology kakhulu ezahlukahlukeneyo yaye ngokufuthi ikhangeleka ngathi kukho iingxaki zempilo kangako yingozi.

Kufuneka uqale nesibakala sokuba kukho enye uphawu ukuba uzinzile xa sisifo ezifana pheochromocytoma. Lwegazi - ezi ezinye iziphiwo ithumba kuthethwa. Inokuba kwenzeke ngendlela ezinzileyo krizovoe. Xa ithumba nesifo catecholamine ntlekele unesifo, ukunyuka koxinzelelo lwegazi kakhulu kabukhali. Kodwa kwisithuba mezhkrizovy BP ahlale ophakamileyo okanye ube uhla yesiqhelo. Ezishicilelweyo iimeko xa pheochromocytoma waqhubeka kunye noxinzelelo oluphezulu rhoqo, kodwa ngaphandle iingxaki.

Ukuze ufumane inkcazelo engakumbi malunga Stroke

Kwenza ingqondo ukuze sinikele ingqalelo into yokuba loo ngxaki pheochromocytoma ingxaki (hypertension) kudla kunye emathunjini, sentliziyo, yokucolwa kunye nezigulo neuro-engqondweni. Ethetha malunga nophuhliso ukwimeko enjalo, kufuneka kuqatshelwe ukuba luphawulwa mpawu zilandelayo: intloko ebuhlungu, lafa, ixhala, iveze, isikhumba lipholi, ukugabha, amahlaba kunye ukubila. Kusenokubakho ukuhlukunyezwa isingqisho, tachycardia nentlungu intliziyo.

Xa unesifo pheochromocytoma, nengxaki unesifo ukuze bandiqumbise ukugabha, isicaphucaphu kunye umlomo owomileyo. Kodwa ezi nguqu kwi imeko yomguli lezempilo akusoloko kuphela. Ngamathumba enjalo aphela rhoqo nokutshintsha imo egazini. Sithetha ngayo iingxaki ezifana lymphocytosis, hyperglycemia, leukocytosis, kunye eosinophilia.

Ndezela nako zingahlala ukusuka kwimizuzu embalwa iyure okanye ngaphezulu. Kule Uphawu karhulumente cima ikhompyutha yayo engalindelekanga. Ukuncipha kwii uxinzelelo lwegazi kwakhona kwenzeka ngokukhawuleza, kangangokuba kukho umngcipheko hypotension. Njengoko iimpawu evusa ezo zinto ukuze wenze umthambo, ekuqhubeni Ngokucofa zinzulu esiswini, ukusetyenziswa kotywala neziyobisi, emzimbeni okanye nobushushu, iintshukumo ezibukhali, ukuphazamiseka ngokweemvakalelo kunye nezinye izinto.

ikakhulu ukuhlaselwa

Ukuxhuzula bakholisa ukuba frequency eyahlukileyo. Bangakwazi obungqingqwa izihlandlo-10 ukuya kwi-15 ngosuku okanye kanye ngenyanga. Ukuba ukuqala lentlekele kakhulu kwi pheochromocytoma, ngoko kusengqiqweni ukuthetha malunga nengozi yokwenene nestroke, ukopha kwiretina, ukusilela kwezintso, iposi engabalulekanga, sokuhlaziya kwemithambo le aorta, nokudumba yemiphunga, kunye nabanye. Le bunzima kakhulu kule meko Njengokungeniswa catecholamine esizibonakalisa yi ongalawulekiyo hemodynamics. Sithetha ngokutshintsha esihanahanisa- kunye noxinzelelo lwegazi, ukuba ngokungakhethi ngokwendalo kwaye ayikwazi alungiswe.

Ngoko ke, pheochromocytoma - ntoni na? Ucinga obubonakala, kufuneka zihlawulwe ingqalelo abafazi abakhulelweyo, ngenxa yokuba ithumba onjalo bazimilise eclampsia, pre-eclampsia kunye toxaemia. Le ngenxa iimpawu ezinjalo abe iziphumo ukuzalwa ezibi.

imilo kunokwenzeka nezinzileyo le ithumba, apho uxinzelelo lwegazi lihlala phezulu. Kule meko, ngokufuthi abhalwe utshintsho nezintso, lo fundus, kunye myocardium. Kwakhona utshintsho kunokwenzeka ngesiquphe mood, intloko ebuhlungu, ukudinwa yaye akhawuleze ukucaphuka. Enye izigulane 10 uphuhliso seswekile.

Kukho nezinye izifo ukuba basoloko ezinxulumene ne pheochromocytoma. Kumalunga syndrome Raynaud kaThixo, isifo gallstone, syndrome Cushing, neurofibromatosis, kunye nabanye. Kwimeko apho ekuphuhliseni ithumba, iintlungu esiswini, kukho Ukudlulela zivamvo ezikude, kwaye ngokukhawuleza umzimba.

kubonakaliswa ezithile clinical

Kukho iinkalo ezithile zesi sifo, nto leyo inokukhokelela ezinye iingxaki. Oku ngale nkqubo zilandelayo:

• Abantwana unesifo pheochromocytoma kwiimeko ezininzi iphuhlisa lwegazi. Ikota izigulane esemncinane na polydipsia esingatshintshiyo, polyuria, nokuxutha izinto. Enye ingxaki ezinokubangela pheochromocytoma ebantwaneni - nokuthoba isantya sokukhula. Ngaphezu koko, kwaba esemncinane kunye uxilongo uzenza waziva enamandla ukwehla kobunzima, ukubila, isicaphucaphu nokugabha, ukuphazamiseka ezibonwayo, kunye nokuphazamiseka vasomotor.
• Abasetyhini ne pheochromocytoma sinokufumana ukonakala zempilo ngenxa olwandle. Oomama abakhulelweyo kuba Sixelisa eclampsia kunye pre-eclampsia.
• Kwiimeko apho nethumba sibekwe ecaleni eludongeni lwesinye, ukuchama esinxulunyaniswa nalo ukuxhuzula. Kukwakho nengozi hematuria yalula, ukwanda koxinzelelo lwegazi, intloko ebuhlungu, isicaphucaphu kunye iyangongoza.

iindlela isifo

Ukuze kuchongwe imeko yomguli kunye nesifo okusolwayo ezifana pheochromocytoma, uhlalutyo karhulumente umzimba kufuneka wanikel 'ingqalelo ezi mpawu zilandelayo: isiyezi ulusu kobuso kunye amabele, ukwanda koxinzelelo lwegazi, tachycardia, hypotension intolerance.

Ngoko kuyimfuneko ukuba Ngokucofa ngononophelo ukuzama kwimeko yamathumba entanyeni, okanye isisu. Eyona nto ibalulekileyo kukuba izenzo ezinjalo kunokubangela uphuhliso catecholamine ntlekele.

Kwakhona, ezikumgangatho ophezulu uphethwe in yamathumba ezinjalo kufuneka kuquka utyelelo kwi zamehlo. Le meko kunganxulunyaniswa yokuba phantse-40% abantu abanengxaki lwegazi, retinopathy unesifo wabonakala, yaye zaba ezahlukahlukeneyo.

Kukho imiqathango kwemichiza ukuba une pheochromocytoma, ifoto ezivumela ukuba siqonde ubunzulu le meko. Ngoko sithetha kumanqanaba of catecholamines igazi, umchamo, iswekile yegazi kunye chromogranin-A in serum egazini. Ngamanye amaxesha kusenokuba iinkcukacha ezifanelekileyo ukwanda uyondelelwano lwee- hormone parathyroid, cortisol, calcium, phosphorus, calcitonin, kunye nezinye izinto.

Phantsi kweemeko ezithile izigulane ikhutshwa phandle ezohlukileyo une yamathumba. Sithetha izifo ezifana ukugula ngengqondo, neurosis, lwegazi, hyperthyroidism, tachycardia paroxysmal, ukutyhefa urhwaphilizo CNS.

Ekumiseleni uhlobo thumba kunye nezinga yophuhliso lwayo zingasetyenziswa MRI kunye CT, kwakunye isifundo komsebenzi kwentliziyo yesigulana. Ukuba pheochromocytoma unesifo, iimpawu, isifo kwaye afumane unyango - ziinkalo ezihambelana kakhulu njengokuba yamathumba ngeentlobo ezahlukahlukeneyo ekufumanekeni kwangethuba iingxaki kunye nonyango olukhawulezileyo ukuba isitshixo ukuba zihambe kakuhle lo msebenzi isigulane.

Indlela yokuphatha

Kukho iindlela ezahlukeneyo azinampembelelo thumba njengoko okubulalayo hayi isoyikiso. Kwiimeko ezininzi, utyando iya kuba sisigqibo kakhulu ifana, ukuba kwafunyaniswa pheochromocytoma. Ukususwa iya kuba afanelekileyo ingakumbi xa kukho ithumba kwi le ndlala adrenal.

Kodwa ke kungakho ngaphandle ngenxa imeko abaziintsizana intliziyo, imithambo yegazi kunye noxinzelelo oluphezulu lwegazi. ugqirha nxaxheba xa imeko enjalo ayinqweneleki kakhulu okanye ayamkelekanga. Kubonakala ngakumbi ifuthe ithumba ezifana pheochromocytoma. Unyango kuyehla kuthomalaliso iziyobisi, ngokuqhelekileyo kuthatha iintsuku ezimbalwa.

Ukusetyenziswa kweziyobisi ezahlukeneyo ithumba neutralization kuthetha ukuba isigulana eso siya kuba ebhedini, uthabathe nakwisiqingatha behleli isikhundla. Ukuze eze ugqirha oqhelekileyo uxinzelelo lwegazi unakho ukumisela udibaniso lwe-blocker. Ngokucace kakhulu, ukuba iziyobisi ezifana "Tropafen" yaye "phenoxybenzamine." Ukuba isigulane Iqinisekile ntlekele unesifo, ulawulo mlonyeni wayeza kuba "phentolamine" "nitroprusside Isiqalelo setyuwa" kunye namanye amachiza afanayo. Into ebalulekileyo kukuba ngexesha pre-unyango kuyimfuneko ukugcina emikhuhlaneni emzimbeni.

ukusebenzisa utyando

Ukuba pheochromocytoma ibhaqwe, unyango usebenzisa kuphungula uyonyulwa malunga noko inkqubo ethile ithumba. Avamise kakhulu zidibene, transbryushinny, extraperitoneal kunye neendlela transthoracic.

Kwimeko ukuba thumba identity lwafumaneka emva kokuba uviwo, le ALS kunye unyango ngotyando iza kuba yingenelo. Kakade ke, kusenokubakho likakade, kodwa kuzibonakalisa kuphela 13-15% yamatyala.

Kodwa xa kukho nangezilonda ezininzi, ngokuphawulekayo nzima ngakumbi le meko. Isisombululo enengqondo kukususa zonke nangezilonda, kodwa ingxaki lilele yokuba amathuba isiphumo ngempumelelo luphantsi kakhulu. Kungenxa yesi sizathu ukuba kwimeko pheochromocytomas ezininzi kotyando oluqhutywa kwizigaba ezininzi. Kwezinye iimeko, kuphela nethumba lisusiwe ngokuyinxenye.

Njengoko kuba iimpawu ngokusebenza, ngethuba ukuphunyezwa kwayo ziya kusebenza ukufikelela kuphela laparotomy. Yayichaza le ndlela ophezulu umngcipheko ithumba extraadrenal zalapha. Kwakhona kubalulekile ukwazi ukuba ulawulo hemodynamic (uxinzelelo lwegazi kunye noxinzelelo central lwemithambo) ibanjwa kulo lonke ungenelelo ngoncedo.

Kwiimeko ezininzi emva kokuba pheochromocytoma lususiwe, uxinzelelo lwegazi kunciphisa. Ukuba ukusabela enjalo kumzimba akakho, ngoko kukho zonke isizathu sokukrokrela inyaniso ukuyilwa pregnancy ithumba kwithishyu.

Ukuba pheochromocytoma kwafunyanwa abasetyhini abakhulelweyo, kuqala wenza nokuzinziswa uxinzelelo lwegazi, waza wenza uqhaqho, okanye isisu, kwaye kuphela uze ususe nethumba ngokwayo.

Kwimeko zokufumanis a pheochromocytoma onobungozi, apho ukusasazwa metastases kufumana chemotherapy ( "Dacarbazine" "Vincristine", "Cyclophosphamide").

Ngokubanzi, ukuba ithumba ibhaqwe kusekwangoko, kwaye isigulana uzilungiselele kakuhle kunyango, amathuba ukoyisa le ngxaki kakhulu phezulu.

Ziziphi izinto inokuchazwa kusungula

Ngophuhliso yamathumba emzimbeni usoloko umngcipheko bamqumbise uhlaselo. Ngoko ke kubalulekile ukuba uyazi, nto leyo enokukhokelela ekubeni ukuqhubela karhulumente. It is a umbuzo malunga neentlobano zesini, utshintsho ukuma umzimba, hyperventilation, abahluza ngexesha ukuhamba kwesisu, umthwalo ngokwasemzimbeni, excitation ngengqondo kunye noxinzelelo ukuya kwindawo apho yaba ithumba. Ukuba unesifo pheochromocytoma, musa ukuzibandakanya utywala, ingakumbi iwayini, ibhiya, ngokunjalo adle sisi.

Kukho ziye zabhala iimeko xa ukuhlaselwa kwaqala emva kokufumana inicotine, lakhula tricyclic, avela phenothiazine beta-blocker.

iziphumo

Ngaloo ndlela siya kuba umxholo othi "Pheochromocytoma" kuvezwe. Yintoni na, saza iinkcukacha ezininzi. Kuhleli ukwenza le nto ibalulekileyo: ingxaki inkulu kakhulu ukuluphatha lula. Kwimiqondiso yokuqala efana iimpawu pheochromocytoma, kuyimfuneko ukuba udibane nogqirha, kuba unesifo kwaye uqale unyango.