Hom dystrophies - kev kawm txog biology thiab tshuaj. Cov txheej txheem pathological cuam tshuam nrog kev ua tsis zoo ntawm cov metabolism hauv qib cellular, uas ua rau muaj kev ua txhaum ntawm kev ncaj ncees ntawm cov txheej txheem organic. Nyob rau hauv cov ntaub so ntswg, hlwb, tshuaj compounds yog generated uas tsis muaj nyob rau hauv cov localizations. Ib qho kev faib tawm ntau dua tau tsim, thiab rau txhua hom, cov kws kho mob tab tom kawm txog qhov ua rau tshwm sim thiab muaj peev xwm los daws nws.
Qeb thiab pawg
Kev faib tawm ntawm hom dystrophies yog nyob ntawm morphology ntawm qib cellular thiab cov yam ntxwv ntawm nws qhov kev hloov pauv. Faib cov parenchymal, mesenchymal thiab ua ke mob. Kev ntsuam xyuas cov nuances ntawm cov txheej txheem metabolic, pathology yog suav tias yog lipidosis, dysproteinosis, lossis vim kev ua txhaum ntawm cov tshuaj tiv thaiv nrog cov zaub mov thiab carbohydrates. Cov ntaub ntawv tuaj yeem yog hauv zos lossis dav dav - qhov thib ob yog qhov hnyav dua.
Xav txog ntau hom dystrophies, cov kws tshawb fawb tau qhia txog kev faib ua pawg raws li keeb kwm ntawm pathology. Tus kab mob tuaj yeem tauqub txeeg qub teg, tiam sis koj muaj peev xwm yuav nws tom qab yug.
Morphology thiab noob caj noob ces
Ntau hom dystrophies muaj cov yam ntxwv tshwj xeeb thiab qhov sib txawv. Pathology tau piav qhia los ntawm lub peev xwm ntawm cov khoom nkag mus rau lwm tus zoo li hauv cov qauv rau lawv. Ib qho piv txwv classic yog carbohydrates, uas, nyob rau hauv tej yam kev mob, hloov mus rau hauv fatty qauv. Cells, cov ntaub so ntswg tuaj yeem dhau los ua qhov chaw ntawm thaj chaw ntawm qee cov tshuaj sib xyaw. Cov txheej txheem no hu ua infiltration thiab tseem ceeb heev nyob rau hauv morphogenesis ntawm dystrophy. Muaj ob qhov sib txawv ntawm infiltrates. Hauv thawj kis, lub xovtooj ntawm tes uas koom nrog hauv cov txheej txheem biochemical tau txais qee qhov sib xyaw ua ke ntau dhau. Muaj los ntawm kev txwv, cov qauv tsis tuaj yeem tiv nrog kev ua haujlwm ntawm kev sib txuas. Qhov thib ob ntawm qhov tshwm sim yog qhov txo qis hauv kev ua haujlwm ntawm cov qauv ntawm tes, uas tsis tuaj yeem tiv nrog cov ntim ib txwm muaj ntawm cov tshuaj nkag mus rau nws.
Nyob rau hauv lub morphogenesis ntawm ntau hom dystrophies, ib qho tseem ceeb nam yog decomposition, uas yog, qhov tshwm sim ntawm lub cev qhuav dej ntawm cov qauv hauv cov ntaub so ntswg, hlwb. Lipid thiab protein complexes muaj nyob rau hauv organelle daim nyias nyias raug rhuav tshem. Feem ntau, cov rog, cov ntsiab lus ntawm cov protein tau khi rau hauv daim nyias nyias, thiab tsis pom nyob rau hauv kev tshuaj xyuas microscopic, tab sis tom qab kev tawg ntawm qhov nyuaj, lawv tuaj yeem pom raws li ib feem ntawm kev kawm tshwj xeeb.
Qhov kawg feature yog qhov ua tsis zoo ntws los ua ke, uas lub xovtooj tau dhau los ua qhov chaw hauv cheeb tsam rau kev tsim cov khoom sib txawv uas tsis tas yuav tsum tau los ntawm lub cev rau kev ua haujlwm kom raug. Yog li, amyloidosis yog nrog los ntawm kev tsim cov qauv ntawm cov protein, los ntawm qhov ntawdamyloid tshwm. Tawm tsam keeb kwm yav dhau los ntawm kev haus dej cawv ntev ntev, lub siab hlwb tsim cov ntsiab lus protein - lub hauv paus rau kev tsim cov cawv hyaline.
Pathology: Qhia los ntawm kev cuam tshuam
Ntau hom nqaij pob txha dystrophy, mesenchymal, carbohydrate thiab lwm yam, ua rau qee yam tsis ua haujlwm ntawm cov ntaub so ntswg organic. Cov kev tsis sib haum xeeb tuaj yeem tsim nyob rau hauv ib qho ntawm ob txoj hauv kev: kev ua haujlwm tsis muaj zog lossis cov yam ntxwv ntawm kev ua haujlwm hloov pauv, cov yam ntxwv zoo li no yog tsim los uas tsis muaj nyob hauv cov qauv ntawm tes.
Ib qho piv txwv classic ntawm qhov tshwm sim: nyob rau hauv cov ntaub ntawv ntawm lub raum pathologies, cov ntsiab lus protein raug kuaj pom nyob rau hauv cov zis, cov tiam uas muaj feem xyuam nrog cov txheej txheem dystrophic. Nrog rau lub siab dystrophy, cov txiaj ntsig ntawm kev sim hloov pauv, thiab nrog lub plawv dystrophy, lub suab ntawm lub cev ua haujlwm.
Parenchymal dystrophies
Nyob rau hauv qeb no, peb hom phenomena yog qhov txawv; rau kev faib ua pawg, cov qauv kev koom tes hauv cov txheej txheem raug soj ntsuam. Yog li, dystrophy tuaj yeem tsim hom carbohydrate, nrog kev koom tes ntawm cov protein lossis lipids. Txhua tus ntawm lawv muaj nws tus kheej tshwj xeeb.
YProtein dystrophies
lo lus no hais txog cov txheej txheem pathological cuam tshuam nrog cov metabolism hauv cov protein tsis raug. Cov no feem ntau yog nyob hauv cov hlwb. Muaj cov nram qab no hom protein parenchymal dystrophies: horny, hydropic, drip hyaline thiab granular.
Ntxim qab heev lis os. Qhov ntau yam no tau yooj yim txiav txim siab nyob rau hauv lub moj khaum ntawm kev soj ntsuam histological:cov nplej ntawm cov protein tau pom meej meej. Xws li ib tug pathology feem ntau nyob rau hauv lub raum, lub plawv, siab, hu ua dull o. Lub npe qhia txog qhov pom ntawm microscopic - lub cev zoo li loj me ntsis, tab sis nyob rau hauv cov ntsiab lus lawv dull, pos huab. Nws zoo li yog tias cov ntaub tau scalded nrog boiling dej. Hom kab mob dystrophy no nyob ntawm ntau yam, thiab nws yog txoj cai los faib cov ua rau kis tau thiab lom.
Granular raum dystrophy yog ib txwm muaj. Ib qho kev pom zoo thiab cov txiaj ntsig tau ua tau yog tias qhov ua rau raug txheeb xyuas thiab tshem tawm hauv lub sijhawm - maj mam cov tubular epithelium normalizes nws cov khoom zoo. Nrog rau kev cuam tshuam ntev ntev, cov txheej txheem ua tau zoo, nws tsis tuaj yeem thim rov qab lawv, dystrophic kev hloov pauv dhau los ua thaj chaw ntawm thaj chaw ntawm cov txheej txheem necrotic. Qhov no feem ntau pom yog tias tus neeg mob tau ntsib cov tshuaj lom rau lub raum.
Granular dystrophy: hauv plawv thiab siab
Yog tias hom kab mob dystrophy no tshwm sim hauv lub plawv, lub cev tau nthuav dav me ntsis, thiab myocardium ua flabby, pom, thaum txiav, nws zoo ib yam li cov nqaij boiled. Histological tsom xam qhia basophilia. Myocardial nthwv dej sib txawv rau eosin, hematoxylin. Qee lub ntsiab lus tau txais cov xim lilac vim yog hematoxylin, lwm tus ua xiav xiav vim eosin.
Lub siab nrog cov kab mob no me ntsis loj dua li qub. Thaum txiav los ntawm cov ntaub so ntswg organic, lub xim zoo li zoo li av nplaum. Kev soj ntsuam histological tuaj yeem tsim cov nplej protein, tab sis lawv lub xub ntiag yuav tsis tas mus li. Nws yog ib qho tseem ceeb rau kev ntsuam xyuas kev ncaj nceesnqaj qauv. Proteins feem ntau faib ua hepatocytes, vim qhov xwm txheej hu ua discomplexation ntawm daim siab kab teeb.
Hyaline drip
daim ntawv no feem ntau nyob hauv ob lub raum, siab. Pom pom, kev hloov pauv hauv lub cev tsis tuaj yeem pom; thaum kuaj xyuas hauv lub tshuab ntsuas, cov kua protein tuaj yeem pom hauv lub cell cytoplasm. Dystrophy ua rau cell tuag vim coagulative necrosis. Nyob rau hauv lub raum, pathology feem ntau yog tsim los ntawm nephrotic syndrome yam ntxwv ntawm ntau yam kab mob. Feem ntau cov syndrome nrog nephropathy, amyloidosis. Ib qho infiltration pronounced yog muaj nyob rau hauv nephrocytic dystrophy, txij li thaum lub glomerular lim yog tus yam ntxwv los ntawm ib tug ntau ntawm pores. Qhov no sai sai no ua rau decomposition, cuam tshuam ntawm nephrocyte apparatus lub luag hauj lwm rau protein reabsorption.
Hepatic dystrophy ntawm hom no yog tus yam ntxwv ntawm cov neeg mob uas haus cawv, kab mob siab vim li no. Tsawg tsawg zaus, dystrophy raug kuaj pom tom qab ntawm cholestasis, cirrhosis. Hyaline zoo li cov ntsiab lus tau hu ua cawv hyaline. Xws li cov qauv - mesh los yog nyob rau hauv daim ntawv ntawm cov blocks, nyob rau hauv perinuclearly. Dystrophy yog ntau vim qhov tshwm sim tsis zoo ntawm cov tshuaj synthesis dua li lwm cov txheej txheem. Alcoholic hyaline teeb tsa tag nrho cov tshuaj biochemical hauv lub siab thiab sab nraum lub cev. Nws muaj chemotactic zoo, uas txiav txim siab leukotaxis. Feem ntau dystrophic foci yog ncig los ntawm polymorphonuclear leukocytes. Lawv lub xub ntiag yog ib qho cim qhia ntawm tus kab mob siab vim kev quav dej quav cawv.
YHydropic dystrophy
Nrog rau daim ntawv no ntawm tus kab mob no, lub cev pom lub cev zoo li qub, tab sis kev soj ntsuam kom ntxaws hauv lub tshuab ntsuas pom tuaj yeem pom cov vacuoles hauv cov cell cytoplasm, uas maj mam dhau los ua qhov ua rau lub zais pa dystrophy, cell tuag. Cov kab mob no feem ntau nyob hauv daim siab, raum, tsis tshua muaj nyob hauv daim tawv nqaij.
o ntawm cytoplasm, tsim ntawm lub vacuole yog nrog los ntawm kev hloov pauv ntawm cov cell nucleus mus rau daim nyias nyias, xim tsis muaj zog. Qhov no pathology yog tus yam ntxwv ntawm cov neeg mob uas muaj nephrotic syndrome. Lub epithelium npog lub tubules ntawm ob lub raum ua rau thaj tsam ntawm thaj chaw ntawm cov txheej txheem pathological vim kev cuam tshuam ntawm lub cev ntawm cov enzymes thiab daim nyias nyias. Nquag, cov tshuab no yog lub luag haujlwm rau reabsorption ntawm kua, proteins.
Nephrocytic hydropic dystrophy tshwm sim nrog kev nkag mus rau hauv qhov hnyav, decomposition. Cov sodium-potassium basal labyrinth ntawm ATP raug kev txom nyem, dhau los ntawm qhov kev nqus ntawm cov kua dej, sodium, tshwm sim.
Cov kab mob siab feem ntau yog kuaj pom hauv cov neeg mob uas muaj kab mob siab viral. Qhov laj thawj yog kev ua kis kab mob, uas cuam tshuam cov txheej txheem synthesis hauv hlwb.
Lipids
Muaj ntau hom rog rog degeneration, thiab kev faib ua pawg muaj feem cuam tshuam rau kev lom neeg thiab tshuaj lom neeg ntawm cov txheej txheem. Nws yog kev cai rau kev sib txawv ntawm cov rog ua ob hom: labile, uas nws cov ntim tau hloov kho tas li thaum tib neeg lub neej, thiab ruaj khov, suav nrog hauv cell membranes. Txhawm rau txheeb xyuas cov qauv rog, lawv mus rau dyes (Sudan, scarlet, osmic acid,Nile xiav). Tshaj tawm cov ua rau dystrophy, nws tau pom tias feem ntau cov kab mob pathological tshwm sim vim muaj tshuaj lom los yog kis kab mob. Cov tshuaj lom neeg mob ntev yog feem ntau haus dej cawv, me ntsis tsawg dua - tshuaj, endocrine. Cov tom kawg yog cov yam ntxwv ntawm cov neeg mob ntshav qab zib. Qhov chaw ntawm qhov chaw ntawm cov txheej txheem dystrophic yog myocardium, ob lub raum, siab.
Feem ntau nyob rau hauv kev kho mob, ib tug yuav tsum tau nrog cov hom fatty degeneration localized nyob rau hauv daim siab. Nrog rau qhov no pathology, nruab nrab rog yog ib nrab tsim los ntawm hepatocytes. Muaj peb theem ntawm tus mob: fatty siab, mesenchymal teb lub cev, fibrosis, thiab cirrhosis. Yog vim li cas rau tsub zuj zuj ntawm fatty lug nyob rau hauv daim siab cheeb tsam yog ib tug ua txhaum ntawm enzymatic tshuaj tiv thaiv ntawm ib tug tej yam theem ntawm lipid metabolism. Qhov no yog ua tau yog hais tias lub cell tau txais ntau fatty compounds, lub hepatocyte active synthesizes xws li tshuaj, vim hais tias tsis muaj txaus enzymes rau lub cev ua hauj lwm. Qhov ua rau dystrophy tuaj yeem ua rau lub sijhawm ntev ntawm cov tshuaj lom neeg, vim tias cov oxidation ntawm cov tshuaj yog inhibited, nrog rau qhov tsis muaj cov amino acids, yam tsis muaj lipoproteins thiab phospholipids yog tsis yooj yim sua..
Pathology ntawm daim siab: nta
Hauv cov ntawv tshaj tawm tshwj xeeb, koj tuaj yeem pom ntau lub rooj mob siab rau hom dystrophy, nthuav tawm cov nuances ntawm kev txhim kho cov txheej txheem pathological, cov yam ntxwv ntawm lawv qhov pib, thiab cov xwm txheej uas ua rau muaj kev ua txhaum cai. Kev tsom xam ntawm cov peev txheej no tso cai rau peb txheeb xyuas cov xwm txheej tseem ceeb uas ua rau lub siab rog rog degeneration. Hauv ntau tus neeg mob, ntshav qab zibntshav qhia tau tias muaj cov ntsiab lus ntawm fatty acids. Qhov no yog ib qho zoo rau cov neeg mob ntshav qab zib, cov neeg haus cawv thiab cov neeg uas muaj qhov hnyav dhau. Ethanol, phosphorus thiab lwm yam tshuaj lom cuam tshuam rau hepatocytes, uas provokes dystrophy.
Ib tus mob pathological tuaj yeem txhim kho vim kev noj zaub mov tsis txaus, tsis muaj cov qauv protein hauv cov zaub mov, tawm tsam keeb kwm yav dhau los ntawm cov kab mob hauv plab hnyuv. Genetic enzyme tsis ua haujlwm uas tswj cov lipid metabolism tuaj yeem ua rau lawv txoj kev koom tes. Hauv qhov no, lipidosis tau raug kuaj pom.
cawv thiab ntshav qab zib
Ntawm tag nrho cov xwm txheej ntawm fatty degeneration, txog ib nrab ntawm kev txhim kho vim kev siv ethanol. Cov tshuaj no txhawb nqa cov rog rog uas muaj nyob rau hauv lub depot, ua kom cov synthesis hauv hepatocytes, esterification rau hauv triglycerides, txo qis oxidation, thiab tso tawm lipoproteins. Tawm tsam keeb kwm yav dhau los ntawm kev haus dej cawv, permeability ntawm cell membranes txo, raws li kev tsim cov roj cholesterol tau qhib. Nrog cawv intoxication, hepatic deposits yog ntau, focal. Nrog diffuse rog, daim siab ua loj loj, tau ib tug daj tint, sagging. Qhov tshwm sim tau yog hepatic steatosis. Nws qhov tshwm sim yog nyob ntawm lub luag haujlwm ntawm tus neeg mob: nrog kev tsis txaus siab, tom qab ob peb lub lis piam (qee zaum ob zaug), cov rog tag nrho ploj ntawm daim siab. Kev loj hlob ntawm steatosis ua rau cirrhosis.
Txog li 75% ntawm cov neeg mob ntshav qab zib raug kev txom nyem los ntawm lub siab rog rog degeneration. Qhov no tsis tshua muaj tshwm sim hauv cov menyuam yaus, cov hluas, thiab cov hluas. Tsis tas li ntawd, muaj kev vam khom ntawm qhov hnyav, qib ntawm ketoacidosis. Hepatic steatosis hauv ntshav qab zibvim muaj kev koom tes ntawm fatty lug ntawm lub depot nrog kev hloov mus rau lub siab. Nyob rau tib lub sijhawm, cov txheej txheem ntawm kev tsim phospholipids thiab oxidative kev cuam tshuam cuam tshuam. Lipolysis yog txhim kho los ntawm qhov tsis muaj insulin, ib qho tshuaj tiv thaiv lipolytic. Cov ntsiab lus ntawm fatty acids nyob rau hauv lub circulatory system nce, lub siab nquag tsim lipoproteins, tab sis tsis muaj apoprotein txaus, yog li cov khoom siv acids tsis absorbed, thiab ntau dhau tau hloov mus rau hauv triglycerides. Hepatic steatosis ntawm keeb kwm ntawm ntshav qab zib feem ntau cuam tshuam nrog kev rog dhau, thiab dystrophy tau qhib vim muaj ntau ntawm carbohydrates thiab cov rog uas tau muab los ntawm zaub mov.
YCarbohydrates thiab pathology
Ntau hom carbohydrate dystrophies paub. Muaj cov kab mob pathologies cuam tshuam nrog kev cuam tshuam tsis zoo uas cuam tshuam nrog glycogen. Nyob rau tib lub sijhawm, lub cev tsis muaj enzyme (lossis tsis muaj nyob rau hauv tag nrho) lub luag haujlwm rau glycogen deposition. Cov kab mob pathological yog suav tias yog kab mob khaws cia. Rau ntau yam cuam tshuam nrog rau qhov tsis muaj rau hom enzymes tau txheeb xyuas thiab kawm.
Qee zaus, kuaj pom cov carbohydrate dystrophies, tshwm sim los ntawm qhov tshwm sim tsis raug ntawm cov metabolism hauv glycoproteins. Mucoids thiab mucins sau nyob rau hauv lub hlwb, nyob rau hauv qhov chaw ntawm lawv, thiab mucous degeneration tshwm.
txhawm rau txheeb xyuas qhov muaj ntawm rooj plaub rau qee yam, txhawm rau txiav txim siab hom kev sib xyaw ua ke, yog tias cov txheej txheem nyuaj, lawv mus rau kev tshuaj xyuas cov hlwb hauv lub tshuab tsom. Kev soj ntsuam pab txheeb xyuas qhov tsim ntawm cov hnoos qeev thiab kev hloov hauv nws cov tshuaj, lub cevyam zoo. Cells muaj peev xwm ntawm secreting tshuaj tuag, desquamate, glandular ducts ua obstructed nrog mucus. Vim li ntawd, lub cyst raug tsim.
Mesenchymal txheej txheem
Muaj ntau hom stromal-vascular dystrophies, feem ntau tshwm sim los ntawm cov ntaub so ntswg sib txuas tsis raug. Feem ntau cov no muaj nyob rau hauv lub vascular phab ntsa, stroma nyob rau hauv lub histion, tsim los ntawm cov microvasculature thiab connective cov ntaub so ntswg ntsiab nyob ze nrog rau cov hlab ntsha fibers. Ntawm cov txheej txheem ntawm kev txhim kho, qhov tseem ceeb tshaj plaws yog qhov tsis raug trophism, kev cuam tshuam ntawm kev thauj mus los. Feem ntau, tus neeg mob tau kuaj pom muaj ntau hom mesenchymal dystrophy ib zaug, uas tuaj yeem dhau ntawm lawv tus kheej.
Cov yam ntxwv tshaj plaws ntawm dystrophy nyob rau hauv rooj plaub ntawm dysproteinosis. Kev tsis sib haum xeeb ntawm cov ntaub so ntswg yog tshwm sim los ntawm kev puas tsuaj, qee zaum los ntawm kev tsim cov protein ntau txawv txav. Txhua hom mesenchymal dystrophy tam sim no tab tom kawm, tab sis qee qhov kev tshawb pom hauv cheeb tsam no tseem tsis tau ua. Feem ntau, dystrophies raug kuaj tawm tsam keeb kwm ntawm autoimmune. Qhov hnyav dua, cov lus tshaj tawm ntau dua dystrophy.
Teeb meem paub rau txhua tus
Muaj ib hom kab mob dystrophy, lub npe uas tau hnov los ntawm txhua tus. Qhov teeb meem no ntshai tib neeg rau qhov laj thawj - nws yog qhov dav thiab tuaj yeem ua rau muaj kev cuam tshuam tsis zoo. Peb tab tom tham txog retinal dystrophy. Cov yam ntxwv ntawm cov kab mob pathological no tau txais thiab muaj keeb kwm yav dhau los. Thawj qhov kev xaiv nrog lub sijhawmqhov pib kho tuaj yeem kho tau, qhov thib ob kho tsis tau.
