Qee zaum hauv kev kho mob muaj qhov tshwm sim xws li parenchymal dystrophy. Pathological anatomy hais txog lawv rau cov kab mob metabolic hauv cov hlwb. Hauv cov ntsiab lus yooj yim, cov txheej txheem ntawm cov khoom noj khoom haus thiab sib sau ua ke ntawm cov khoom muaj txiaj ntsig yog cuam tshuam rau hauv lub cev, uas ua rau muaj kev hloov pauv ntawm morphological (visual). Koj tuaj yeem txheeb xyuas cov kab mob pathology ntawm ntu lossis tom qab cov kev sim tshwj xeeb. Parenchymal thiab stromal-vascular dystrophies ua rau muaj ntau yam kab mob tuag taus.
Definition
Parenchymal dystrophies yog cov txheej txheem pathological uas ua rau muaj kev hloov pauv hauv cov qauv ntawm lub cev. Ntawm cov txheej txheem ntawm txoj kev loj hlob ntawm tus kab mob, muaj teeb meem ntawm cell self-regulation nrog lub zog tsis muaj zog, fermentopathy, dyscirculatory ntshawv siab (ntshav, lymph, interstitium, intercellular kua), endocrine thiab cerebral dystrophies..
Muaj ntau lub tswv yim ntawm dystrophy:
- infiltration, uas yog, kev thauj mus los ntau dhau ntawm cov khoom lag luam metabolic los ntawm cov ntshav mus rau hauv lub cell lossis intercellular qhov chaw, vim muaj kev ua haujlwm tsis zoo hauv lub cev enzymes;
- decomposition, lossis phanerosis, sawv cevyog ib qho kev puas tsuaj ntawm cov txheej txheem intracellular, uas ua rau cov kab mob metabolic thiab tsub zuj zuj ntawm cov khoom hauv qab-oxidized metabolic;
- perverted synthesis ntawm cov tshuaj uas ib txwm lub cell tsis reproduce;
- kev hloov pauv ntawm cov as-ham nkag mus rau ntawm tes los tsim ib hom khoom kawg (proteins, rog lossis carbohydrates).
Kev faib tawm
Pathologists paub qhov txawv ntawm cov hauv qab no ntawm parenchymal dystrophies:
1. Nyob ntawm cov kev hloov morphological:
- ntshiab parenchymal;
- stromal-vascular;
- mix.
2. Los ntawm hom khoom xyaw:
- protein lossis dysproteinoses;
- rog lossis lipidoses;
- carbohydrate;
- ntxhia.
3. Los ntawm cov txheej txheem nthuav dav:
- system;
- zos.
4. Los ntawm lub sijhawm tshwm sim:
- muas;
- congenital.
Pathological anatomy txiav txim siab qee yam parenchymal dystrophies tsis yog los ntawm tus neeg saib xyuas kev puas tsuaj, tab sis kuj yog los ntawm cov ntsiab lus ntawm cov hlwb cuam tshuam. Kev hloov pauv ntawm ib qho dystrophy mus rau lwm qhov yog theoretically ua tau, tab sis tsuas yog ib qho kev sib koom ua ke ntawm pathology yog xyaum ua tau. Parenchymal dystrophies yog cov ntsiab lus ntawm cov txheej txheem tshwm sim hauv lub xovtooj, tab sis tsuas yog ib feem ntawm qhov chaw kho mob, uas suav nrog morphological thiab kev ua haujlwm tsis txaus ntawm ib lub cev.
YDysproteinoses
Tib neeg lub cev feem ntau yog tsim los ntawm cov protein thiab dej. Protein molecules yogtivthaiv ntawm cov phab ntsa ntawm tes, daim nyias nyias ntawm mitochondria thiab lwm yam organelles, nyob rau hauv tas li ntawd, lawv nyob rau hauv ib tug dawb lub xeev nyob rau hauv lub cytoplasm. Raws li txoj cai, cov no yog cov enzymes.
Dysproteinosis yog lwm yam hu ua pathology xws li parenchymal protein dystrophy. Thiab nws cov ntsiab lus nyob rau hauv qhov tseeb tias cellular proteins hloov lawv lub zog, nrog rau kev hloov pauv ntawm cov qauv, xws li denaturation lossis colliquation. Protein parenchymal dystrophies muaj xws li hyaline-poob, hydropic, horny thiab granular dystrophies. Thawj peb yuav tau sau kom ntxaws ntxiv, tab sis qhov kawg, granular, yog tus cwj pwm los ntawm qhov tseeb tias cov nplej protein ntau hauv cov hlwb, vim tias cov hlwb stretch, thiab lub cev nce, ua xoob, npub. Yog vim li cas granular dystrophy tseem hu ua npub o. Tab sis cov kws tshawb fawb tau tsis ntseeg tias qhov no yog parenchymal dystrophy. Lub pathanatomy ntawm tus txheej txheem no yog xws li hais tias compensatory enlarged cellular qauv yuav yuam kev rau nplej, raws li ib tug teb rau kev ua hauj lwm kev nyuaj siab.
YHyaline drip degeneration
Nrog rau hom dystrophies no, cov hlwv loj loj tshwm sim hauv cov hlwb, uas nws thiaj li sib koom ua ke thiab sau tag nrho qhov chaw sab hauv ntawm tes, tshem tawm cov organelles lossis rhuav tshem lawv. Qhov no ua rau poob ntawm kev ua haujlwm thiab txawm tias cell tuag. Feem ntau, tus kab mob no tshwm sim nyob rau hauv lub raum cov ntaub so ntswg, tsawg zaus nyob rau hauv daim siab thiab lub plawv.
Thaum kuaj cytological tom qab kuaj pom lub raum, ntxiv rau qhov sib xyaw ntawm hyaline hauv nephrocytes, kev puas tsuaj tag nrho.cellular ntsiab. Qhov tshwm sim no tshwm sim yog tias tus neeg mob tsim vacuolar-lysosomal insufficiency, uas ua rau txo qis ntawm cov protein rov qab los ntawm cov zis thawj. Feem ntau, qhov no pathology tshwm sim nrog nephrotic syndrome. Feem ntau cov kev kuaj mob ntawm cov neeg mob no yog glomerulonephritis thiab amyloidosis ntawm lub raum. Cov tsos mob ntawm lub cev nrog hyaline poob dystrophy tsis hloov.
Qhov xwm txheej txawv me ntsis hauv cov kua nplaum nplaum. Thaum lub sij hawm microscopy, Mallory lub cev muaj nyob rau hauv lawv, muaj fibrils thiab cawv hyaline. Lawv cov tsos mob yog txuam nrog Wilson-Konovalov tus kab mob, cawv kab mob siab, nrog rau biliary thiab Indian cirrhosis. Qhov tshwm sim ntawm cov txheej txheem no tsis zoo - necrosis ntawm daim siab hlwb, poob ntawm nws txoj haujlwm.
YHydropic dystrophy
Hom kab mob dystrophy no txawv ntawm qhov seem ntawm cov organelles tshiab uas muaj cov kua dej tshwm sim hauv cov hlwb cuam tshuam. Feem ntau, qhov tshwm sim no tuaj yeem pom hauv daim tawv nqaij thiab tubules ntawm lub raum, hauv cov hlwb ntawm daim siab, cov leeg thiab cov qog adrenal.
Microscopically cov hlwb tau nthuav dav, lawv cov cytoplasm tau ntim nrog cov vacuoles nrog cov kua dej ntshiab. Lub nucleus raug tshem tawm lossis lysed, cov qauv seem raug tshem tawm. Thaum kawg, lub xovtooj ntawm tes yog "pob pam" ntim dej. Yog li ntawd, hydropic dystrophy qee zaum hu ua ballooning.
Macroscopically, lub cev xyaum tsis hloov. Lub mechanism ntawm kev loj hlob ntawm tus kab mob no yog ua txhaum ntawm colloid osmotic siab nyob rau hauv lub cell thiab nyob rau hauv lub intercellular qhov chaw. Vim li no, lub permeability ntawm cov cell nce, lawv cov membranes disintegrate thiab cov hlwb tuag. Qhov ua rau ntawm cov tshuaj hloov pauv tuaj yeem yog glomerulonephritis, ntshav qab zib mellitus, raum amyloidosis. Cov kab mob kab mob siab thiab tshuaj lom kab mob ua rau lub cev hloov pauv hauv daim siab. Ntawm daim tawv nqaij, hydropic dystrophy tuaj yeem tshwm sim los ntawm tus kab mob variola.
Cov txheej txheem pathological no xaus nrog focal lossis tag nrho necrosis, yog li morphology thiab kev ua haujlwm ntawm lub cev ua haujlwm tsis zoo sai.
Pathological keratinization ntawm lub cev yog ib qho kev sib txuam ntau dhau ntawm cov khoom horny nyob rau hauv cov txheej txheem ntawm daim tawv nqaij, piv txwv li, hyperkeratosis lossis ichthyosis, nrog rau cov tsos ntawm cov khoom horny qhov twg, raws li txoj cai, nws yuav tsum tsis txhob yuav - nyob rau hauv lub mucous daim nyias nyias (leukoplakia, squamous cell carcinoma). Cov txheej txheem no tuaj yeem yog ob qho tib si hauv zos thiab tag nrho.
Qhov ua rau hom kab mob no tuaj yeem ua rau muaj kev cuam tshuam ntawm ectodermal bud thaum lub sij hawm embryogenesis, hloov cov ntaub so ntswg ntev, kis kab mob thiab vitamin deficiency.
Yog tias kev kho mob tau pib tam sim tom qab thawj cov tsos mob tshwm sim, cov ntaub so ntswg tseem tuaj yeem rov zoo, tab sis nyob rau hauv cov xwm txheej siab heev, kev rov qab tsis tuaj yeem ua tau. Cov cheeb tsam ntev ntawm horny degeneration tuaj yeem ua rau mob qog noj ntshav ntawm daim tawv nqaij, thiab congenital ichthyosis tsis sib haum nrog fetal lub neej.
Hereditary dystrophies
Hereditary parenchymal dystrophy tshwm sim vim congenital fermentopathy. Cov kab mob no yog hu ua cov kab mob khaws cia, vim tias vim muaj cov kab mob metabolic, cov khoom lag luam metabolic khaws cia hauv cov hlwb thiablub cev kua, lom nws. Cov tswv cuab zoo tshaj plaws ntawm pab pawg no yog phenylketonuria, tyrosinosis, thiab cystinosis.
Lub hom phiaj ntawm lub cev rau PKU yog lub hauv paus paj hlwb, cov leeg, tawv nqaij thiab cov kua dej (ntshav, zis). Cov khoom lag luam metabolic hauv tyrosinosis khaws cia hauv cov hlwb ntawm daim siab, ob lub raum thiab pob txha. Cystinosis tseem cuam tshuam rau daim siab thiab lub raum, tab sis kuj cuam tshuam rau tus po, pob qhov muag, pob txha pob txha, lymphatic system, thiab tawv nqaij.
Lipidoses
Lipids muaj nyob rau hauv txhua lub cell, lawv tuaj yeem yog ob qho tib si sib cais thiab ua ke nrog cov proteins thiab ua cov txheej txheem ntawm cov cell membrane, nrog rau lwm cov txheej txheem ultrastructures. Tsis tas li ntawd, glycerol thiab fatty acids muaj nyob rau hauv cytoplasm. Txhawm rau txhawm rau txheeb xyuas lawv hauv cov ntaub so ntswg, cov txheej txheem tshwj xeeb ntawm kev kho thiab staining yog siv, piv txwv li, sudan dub lossis liab, osmic acid, Nile xiav sulfate. Tom qab kev npaj tshwj xeeb, cov kev npaj tau ua tib zoo tshuaj xyuas raws li lub tshuab ntsuas hluav taws xob.
Parenchymal fatty degeneration manifests nws tus kheej nyob rau hauv daim ntawv ntawm ntau tsub zuj zuj ntawm cov rog nyob qhov twg lawv yuav tsum tau, thiab cov tsos ntawm lipids qhov twg lawv yuav tsum tsis txhob yuav. Raws li txoj cai, cov rog nruab nrab nce. Lub hom phiaj ntawm lub cev yog tib yam li hauv cov protein dystrophy - lub plawv, ob lub raum thiab siab.
Fatty parenchymal degeneration ntawm myocardium pib nrog cov tsos ntawm cov me me ntawm cov roj hauv myocytes, lub npe hu ua. plua plav rog. Yog tias cov txheej txheem tsis nres ntawm theem no, tom qab lub sijhawm cov tee sib koom ua ke thiab loj dua,mus txog thaum lawv nyob tag nrho cytoplasm. Nyob rau tib lub sij hawm, organelles disintegrate, lub striation ntawm cov nqaij fibers disappears. Tus kab mob no tshwm sim hauv zos ze ntawm lub txaj vascular.
Macroscopically, parenchymal fatty degeneration manifests nws tus kheej nyob rau hauv ntau txoj kev, nws tag nrho yog nyob ntawm theem ntawm cov txheej txheem. Thaum pib, kev kuaj mob tsuas yog ua tau nyob rau hauv lub tshuab ntsuas, tab sis lub sijhawm dhau los, lub plawv loj tuaj vim qhov nthuav dav ntawm cov chav, nws cov phab ntsa ua nyias thiab flabby, qias neeg daj kab txaij pom thaum lub myocardium raug txiav. Pathophysiologists tuaj nrog lub npe rau lub cev: "tus tsov lub siab."
Fatty degeneration ntawm parenchymal kab mob txhim kho raws li peb lub ntsiab mechanisms.
- Ntau ntxiv ntawm cov fatty acids dawb rau cov hlwb myocardial.
- Impaired fat metabolism.
- Decomposition ntawm lipoprotein qauv hauv cell.
Feem ntau cov txheej txheem no tau tshwm sim thaum lub sijhawm hypoxia, kab mob (diphtheria, tuberculosis, sepsis) thiab intoxication ntawm lub cev nrog chlorine, phosphorus lossis arsenic.
Raws li txoj cai, fatty degeneration yog thim rov qab, thiab kev ua txhaum ntawm cov qauv ntawm tes tau rov qab los rau lub sijhawm. Tab sis yog hais tias tus txheej txheem yog heev launched, ces txhua yam xaus nrog kev tuag ntawm cov ntaub so ntswg thiab lub cev. Cov kws kho mob paub qhov txawv ntawm cov kab mob hauv qab no cuam tshuam nrog kev sib sau ntawm cov rog hauv cov hlwb:
- Kab mob Gaucher;
- Tus kab mob Tay-Sachs;
- Niemann-Pick kab mob thiab lwm tus.
YCarbohydrate dystrophies
Txhua cov carbohydrates uas muaj nyob hauv lub cev tuaj yeem muab faib ua polysaccharides (feem ntau ntawm covyog glycogen), glycosaminoglycans (mucopolysaccharides: hyaluronic thiab chondroitinsulfuric acids, heparin) thiab glycoproteins (mucins, i.e. mucus, thiab mucoids).
txhawm rau txhawm rau txheeb xyuas cov carbohydrates hauv cov hlwb ntawm lub cev, ib qho kev sim tshwj xeeb yog ua tiav - CHIC cov tshuaj tiv thaiv. Nws essence yog tias cov ntaub yog kho nrog iodic acid, thiab ces fuchsin. Thiab tag nrho cov aldehydes tig liab. Yog tias tsim nyog cais glycogen, tom qab ntawd amylase ntxiv rau cov tshuaj reagents. Glycosaminoglycans thiab glycoproteins yog stained nrog methylene xiav. Parenchymal carbohydrate dystrophies feem ntau cuam tshuam nrog glycogen thiab glycoprotein metabolism.
YKev cuam tshuam ntawm glycogen metabolism
Glycogen yog lub cev khaws cia rau "hnub tshaib plab dub". Nws khaws cov khoom tseem ceeb ntawm lawv hauv daim siab thiab cov leeg thiab siv lub zog no heev. Kev tswj hwm ntawm carbohydrate metabolism tshwm sim los ntawm neuroendocrine system. Lub luag haujlwm tseem ceeb yog muab, raws li niaj zaus, rau lub cev hypothalamic-pituitary. Nws tsim cov tshuaj hormones tropic uas tswj tag nrho lwm cov qog endocrine.
Kev ua txhaum ntawm glycogen metabolism yog nce lossis txo nws cov nyiaj hauv cov ntaub so ntswg, nrog rau qhov zoo li qhov nws yuav tsum tsis txhob ua. Feem ntau kom meej meej, cov kev hloov pauv no tshwm sim hauv cov ntshav qab zib mellitus lossis keeb kwm ntawm glycogenoses. Lub pathogenesis ntawm cov ntshav qab zib mellitus tau nkag siab zoo: cov hlwb ntawm lub txiav txiav tsis tsim cov tshuaj insulin hauv qhov xav tau, thiab lub zog reserves ntawm cov hlwb tau ploj mus sai, vim tias cov piam thaj tsis sib xyaw hauv cov ntaub so ntswg, tab sis raug tso tawm los ntawm lub cev.cev nrog zis. Lub cev "qhia" nws cov reserves, thiab ua ntej ntawm tag nrho cov, parenchymal dystrophy ntawm daim siab tsim. Lub teeb khoob tshwm sim hauv cov nuclei ntawm hepatocytes, thiab lawv dhau los ua lub teeb. Yog li ntawd, lawv tseem hu ua "cov cores tsis muaj."
Hereditary glycogenoses yog tshwm sim los ntawm qhov tsis muaj lossis tsis muaj enzymes koom nrog hauv kev sib sau ntawm glycogen. Tam sim no, 6 yam kab mob no paub:
- Gierke's disease;
- Pompe kab mob;
- kab mob McArdle;
- Nws tus kab mob;
- Forbes-Corey kab mob;
- Andersen's disease.
Lawv qhov kev kuaj mob sib txawv tuaj yeem ua tau tom qab lub siab biopsy thiab siv cov tshuaj histoenzyme.
cuam tshuam ntawm glycoprotein metabolism
Cov no yog parenchymal dystrophies tshwm sim los ntawm kev sib sau ntawm mucins lossis mucoids hauv cov ntaub so ntswg. Txwv tsis pub, cov dystrophies no kuj hu ua mucous los yog mucus-zoo li, vim hais tias ntawm cov yam ntxwv sib xws ntawm cov inclusions. Qee lub sij hawm lawv khaws cov mucins tiag tiag, tab sis tsuas yog cov tshuaj zoo ib yam li lawv, uas tuaj yeem ua kom nruj. Hauv qhov no, peb tab tom tham txog colloid dystrophy.
Tissue microscopy tso cai rau koj los txiav txim siab tsis yog tsuas yog muaj cov hnoos qeev, tab sis kuj nws cov khoom. Vim lub fact tias cov seem ntawm cov hlwb, raws li zoo raws li ib tug viscous zais cia, tiv thaiv qhov ib txwm outflow ntawm cov kua los ntawm cov qog, cysts yog tsim, thiab lawv cov ntsiab lus yuav ua rau mob.
Qhov ua rau ntawm hom dystrophy no tuaj yeem sib txawv, tab sis feem ntau nws yog catarrhal o ntawm cov mucous membranes. Tsis tas li ntawd, yog hais tias muaj kab mob hereditary, daim duab pathogeneticuas haum zoo rau hauv lub ntsiab lus ntawm mucosal degeneration. Qhov no yog cystic fibrosis. Cov kab mob pancreas, txoj hnyuv, txoj hnyuv, kab mob, cov kua tsib, hws thiab qog qaub ncaug raug cuam tshuam.
Qhov kev daws teeb meem ntawm hom kab mob no nyob ntawm seb cov hnoos qeev thiab lub sijhawm tso tawm. Lub sijhawm tsawg dhau los txij li qhov pib ntawm cov txheej txheem pathological, qhov ntau dua nws yog tias cov mucosa yuav zoo tag nrho. Tab sis qee zaum, muaj desquamation ntawm epithelium, sclerosis thiab ua tsis taus haujlwm ntawm lub cev cuam tshuam.
