Parenchymal dystrophy. Pathological anatomy

Qee lub sij hawm nyob rau hauv kev soj ntsuam xyaum muaj xws li ib tug phenomenon raws li parenchymal dystrophy. Pathological anatomy hais txog lawv cov teeb meem metabolic hauv hlwb. Hauv cov ntsiab lus yooj yooj yim, lub cev cuam tshuam los ntawm txoj kev noj haus thiab txuam ntawm cov as-ham, uas ua rau lub cev hloov (morphological). Tshaj tawm qhov kev soj ntsuam no nws ua tau rau hauv seem los yog tom qab muaj kev tshuaj xyuas tshwj xeeb heev. Parenchymal thiab stromal-vascular dystrophy underlie ntau cov kab mob ua tuag.

Txhais

Parenchymal dystrophies yog pathological kev ua uas ua rau kev hloov hauv cov qauv ntawm lub hlwb ntawm lub plab hnyuv siab raum. Ntawm cov mechanisms ntawm txoj kev loj hlob ntawm tus kab mob, kev mob ntawm kev tswj tus kheej ntawm hlwb nrog lub zog tsis muaj zog, fermentopathies, dyscirculatory mob (ntshav, lymph, kua qaub, intercellular kua), endocrine thiab cerebral dystrophies yog raug rho tawm.

Muaj ntau ntau txoj kev ntawm dystrophy:

- Infiltration, uas yog, kev thauj khoom ntawm cov khoom siv metabolic los ntawm cov ntshav mus rau sab hauv ntawm lub xovtooj ntawm tes lossis qhov chaw sib txuas ntawm lub cev los ntawm qhov ua rau lub cev tsis muaj zog hauv lub cev;

- Decomposition, los yog Phaneroz, yog lub disintegration ntawm cov txheej txheem intracellular, uas ua rau kev cuam tshuam ntawm cov metabolism hauv thiab tsub zuj zus ntawm cov khoom siv hauv cov khoom oxidized metabolic;

- Cov kab txaij ntawm cov tshuaj yeeb dej cawv, uas feem ntau ntawm tes tsis tuaj me me;

- Cov kev hloov ntawm cov as-ham rau hauv lub xovtooj rau kev tsim kho ntawm ib hom khoom kawg (cov nqaijrog, roj lossis carbohydrates).

Kev faib tawm

Pathomorphologists qhov txawv cov nram qab no hom parenchymal dystrophies:

1. Nyob ntawm seb cov kev hloov ntawm kev hloov ntawm morphological:

- purely parenchymal;

- stromal-vascular;

- tov.

2. Los ntawm hom tshuaj txuam nrog:

- Protein lossis dysproteinosis;

- rog lossis lipidoses;

- carbohydrate;

- ntxhia.

3. Los ntawm cov txheej txheem ntawm cov txheej txheem:

- qhov system;

- Hauv zos.

4. Sij hawm dhau los:

- Tau txais;

- kev xeebtub.

Cov no los yog lwm yam parenchymal dystrophies pathological anatomy pom tseeb tsis yog tus neeg ua kom puas, tab sis kuj yog qhov tseem ceeb ntawm cov hlwb cuam tshuam. Txoj kev hloov ntawm ib qho dystrophy mus rau lwm tus yog theoretically tau, tab sis tsuas yog ib tug ua ke pathology yog suab tau. Parenchymal dystrophies yog qhov essence ntawm cov txheej txheem ntawm cov txheej txheem ntawm tes, tab sis tsuas yog ib feem ntawm qhov kev soj ntsuam mob khomob uas yog siv lub cev tsis muaj zog thiab tsis muaj zog ntawm lub cev.

Dysproteinases

Tib neeg lub cev rau feem ntau yog cov nqaij thiab cov dej. Protein molecules yog ib feem ntawm cov phab ntsa ntawm tes, lub hli ntawm mitochondria thiab lwm yam organelles, ntxiv rau, lawv nyob hauv ib lub xeev dawb hauv cytoplasm. Raws li txoj cai, cov no yog cov enzymes.

Dysprotainosis yog txwv tsis pub hu ua pathology, xws li parenchymal protein dystrophy. Thiab nws cov essence yog tias cov protein proteins hloov lawv lub zog, nrog rau kev hloov hauv kev hloov, xws li denaturation los yog collimation. Rau protein parenchymal dystrophies xws li hyaline-droplet, hydropic, horny thiab granular dystrophy. Thawj peb daim ntawv yuav muab sau rau ntau yam, tab sis qhov tom kawg, ntau tshaj plaws, yog qhov khoom txuam nrog cov khoom noj protein hauv lub hlwb, uas ua rau lub hlwb ncab, thiab qhov hloov li cas, yuav ua rau luag, dim. Tias yog vim li cas dystrophy granular tseem hu ua ib qho kev dim dim. Tab sis cov kws tshawb fawb muaj kev tsis ntseeg tias qhov no yog parenchymal dystrophy. Tus kab mob pathanatomy ntawm tus txheej txheem no yog xws li tias cov nplej uas nws muaj peev xwm siv cov kab mob txuas ntxiv ntawm cov cellular raws li lo lus teb rau kev ntxhov siab.

Hyaline-nco dystrophy

Nrog rau hom dystrophy, cov dej khaus loj tshwm sim nyob rau hauv lub hlwb, uas nws thiaj li sib tshuam nrog ib leeg thiab sau tag nrho qhov chaw ntawm lub xov tooj ntawm tes, tshem tawm cov organelles los yog rhuav tshem lawv. Qhov no ua rau ploj thiab muaj kev tuag ntawm tes. Feem ntau, tus kab mob no tshwm sim hauv cov ntaub so ntswg, tsawg zaus hauv daim siab thiab lub plawv.

Thaum kuaj cytological tom qab lub raum biopsy, ntxiv rau qhov txuam nrog ntawm hyaline nyob rau hauv nephrocytes, kev puas tsuaj ntawm tag nrho cov cellular ntsiab yog kuaj. Qhov tshwm sim no tshwm sim yog hais tias tus neeg mob tsim muaj cov tshuaj vacuolar-lysosomal insufficiency, uas ua rau txo qis dua ntawm cov roj ntsha los ntawm cov zis thawj. Feem ntau, tus kab mob no tshwm sim hauv nephrotic syndrome. Qhov feem ntau heev mob ntawm cov neeg mob - glomerulonephritis thiab raum amyloidosis. Cov tsos ntawm cov khoom nrog hyaline-droplet dystrophy tsis hloov.

Hauv cages, txoj kev yog txawv me ntsis. Thaum lub sij hawm me me, Mallory lub cev, muaj hom fibrils thiab alcoholic hyaline, muaj nyob hauv lawv. Lawv zoo li yog txuam nrog Wilson tus kab mob, dej tus kab mob siab, raws li tau zoo raws li biliary mob npuas paug thiab Indian. Qhov tshwm sim ntawm txoj kev no yog unfavorable - necrosis ntawm daim siab hlwb, poob ntawm nws muaj nuj nqi.

Hydropic degeneration

Qhov dystrophy no txawv ntawm qhov so nyob rau hauv cov tshiab organelles uas muaj kua tshwm sim hauv lub hlwb. Feem ntau, qhov tshwm sim no tuaj yeem pom nyob rau ntawm daim tawv nqaij thiab tubules ntawm ob lub raum, nyob rau hauv lub hlwb ntawm daim siab, cov leeg thiab adrenals.

Me me, lub hlwb zoo dua, lawv cov cytoplasm yog ntim nrog vacuoles nrog pob tshab kua txheem. Cov tub ntxhais kawm tau tawm los yog xyuas, lwm yam kev tawm raug tshem tawm. Thaum kawg, lub xov tooj ntawm tes yog "balloon" muaj dej. Yog li, hydropic dystrophy tej zaum kuj hu ua balloon dystrophy.

Macroscopically, lub nruab nrog cev tsis hloov. Lub tswv yim ntawm txoj kev loj hlob ntawm tus kab mob no yog qhov ua txhaum ntawm colloid-osmotic siab nyob rau hauv lub xov tooj ntawm tes thiab nyob rau hauv qhov chaw sib txuas. Vim li no, cov kab mob ntawm cov qog muaj zog, lub sijhawm tawg thiab lub hlwb tuag. Qhov ua rau cov kev hloov pauv tshuaj no yuav yog glomerulonephritis, ntshav qab zib mellitus, raum amyloidosis. Nyob rau hauv daim siab, cov kev hloov ntawm lub cev yog ua los ntawm cov kab mob viral thiab qhov mob. Nyob rau hauv daim tawv nqaij hydropic degeneration yuav tshwm sim los ntawm ib tug kab mob ntawm smallpox.

Txoj kev siv tshuaj pathological no xaus nrog focal los sis tag nrho cov necrosis, yog li ntawd lub cev thiab kev ua haujlwm ntawm cov kabmob yuav ua rau kom sai sai.

Horn dystrophy

Kev noj nqaij keratinization ntawm cov plab hnyuv siab raum yog qhov ntau ntawm cov khoom pov thawj ntawm cov tawv nqaij, piv txwv li, hyperkeratosis lossis ichthyosis, thiab qhov muag ntawm horny tshuaj uas nws yuav tsum tsis nquag muaj - nyob rau hauv mucous membranes (leukoplakia, squamous cell carcinoma). Cov txheej txheem no yuav yog ob qho tib si hauv zos thiab tag nrho.

Qhov ua rau hom kab mob no tuaj yeem ua txhaum ntawm ectodermal rudiment thaum lub sij hawm embryogenesis, cov kev mob hloov ntawm cov nqaij mob, kis kab mob thiab tsis muaj cov vitamins.

Yog tias txoj kev kho mob pib tshwm sim sai tom qab qhov pom ntawm cov tsos mob thawj zaug, cov ntaub so ntswg tseem tuaj yeem rov qab tau zoo dua, tab sis nyob rau hauv cov rooj sib tham uas tsis muaj teeb meem, rov qab los tsis tau lawm. Cov cheeb tsam uas ntev ntev ntawm lub caj pas dystrophy tuaj yeem cuam tshuam mus rau hauv cov kabmob ntawm daim tawv nqaij, thiab tus mob ichthyosis yog qhov ua tsis taus nrog lub plab hauv lub neej.

Muaj kab dystrophy

Hereditary parenchymal dystrophies tshwm sim los ntawm congenital fermentopathy. Cov kab mob no kuj hu ua cov kab mob txuam nrog, vim metabolic disturbances, cov khoom metabolic nyob hauv lub hlwb thiab kua hauv lub cev, lom nws. Cov neeg sawv cev nto moo tshaj plaws ntawm pawg no yog phenylketonuria, tyrosinosis, thiab cystinosis.

Lub hauv paus plab hnyuv hu ua phenylketonuria yog lub hauv paus paj hlwb, leeg, tawv nqaij thiab kua (ntshav, zis). Cov metabolites ntawm tyrosinosis zais nyob rau hauv lub hlwb ntawm daim siab, ob lub raum thiab cov pob txha. Tus mob hu ua Cystinosis kuj tseem ua rau lub siab thiab lub raum, tab sis tsis pub lawv, tus po, pob txha, pob txha, cov kab mob hauv lub cev thiab tawv nqaij.

Lipidoses

Lipids muaj nyob hauv txhua lub cell, lawv tuaj yeem sib cais los yog ua ke nrog cov nqaijrog thiab ua tej yam hauv cov cell membrane, nrog rau lwm cov kab mob ultrastructures. Tsis tas li ntawd, cov glycerin thiab fatty acids uas muaj nyob hauv cytoplasm. Txhawm rau txhom lawv hauv cov ntaub so ntswg, kev kho tshwj xeeb thiab kev siv tau yog siv, piv txwv li, dej los sis liab liab, osmic acid, Nile xiav sulphate. Tom qab ib qho kev npaj, cov kev npaj tau ua tib zoo soj ntsuam hauv lub tshuab hluav taws xob.

Parenchymal fatty degeneration ua rau nws tus kheej nyob rau hauv daim ntawv ntawm ntau tsuj roj ntawm qhov chaw uas lawv yuav tsum tau, thiab cov tsos ntawm lipids qhov twg lawv tsis tsim nyog. Lawv yuav noog nruab nrab rog. Lub plab hnyuv siab raum ntawm lub hom phiaj yog tib yam li hauv protein dystrophy - lub plawv, lub raum thiab lub siab.

Cov roj parenchymal myocardial dystrophy pib nrog lub tsos nyob rau hauv myocytes ntawm heev me me mob ntawm cov rog, uas yog. N. Pulverized rog. Yog hais tias tus txheej txheem tsis nres nyob rau theem no, ces nws thiaj li dhaus ua ke thiab ua kom loj dua mus txog thaum lawv nyob tag nrho cytoplasm. Organelles nyob rau hauv cov ntaub ntawv no disintegrate, lub striation ntawm cov leeg fibers disappears. Tus kab mob no tshwm sim hauv zos ze ntawm lub vas sab vascular.

Macroscopically, parenchymal fatty degeneration tshwm sim nws tus kheej nyob rau hauv ntau txoj kev, nws tag nrho cov nyob ntawm theem ntawm tus txheej txheem. Thaum pib los, tus mob no tsuas yog ua rau hauv lub tshuab xwb, tab sis thaum kawg nws lub plawv loj hlob vim qhov ncab ntawm qhov chaw, nws cov phab ntsa ua nyias thiab ntuav, thaum txiav qhov myocardium qhia tau qias daj daj. Pathophysiologists tuaj nrog lub npe rau cov khoom no: "tiger plawv".

Rog dystrophy ntawm parenchymal plab hnyuv siab tuaj tsim tawm raws li peb lub tswv yim tseem ceeb.

1. Txo kom tsawg ntawm cov dawb fatty acids uas yog nyob hauv hlwb myocardial.
2. Kev puas tsuaj ntawm roj metabolism.
3. Disintegration ntawm lipoprotein cov kab hauv lub cell.

Feem ntau cov tswv yim no yog tshwm sim thaum hypoxia, kis kab mob (kab mob qog nqaij hlav, mob ntses, sepsis) thiab kev qaug hauv lub cev nrog chlorine, phosphorus lossis arsenic.

Raws li txoj cai, rog dystrophy reversible, thiab cov qauv ntawm tes tsim kho nrog lub sijhawm. Tab sis yog tias txheej txheem yog ua phem heev, ces txhua yam xaus nrog kev tuag ntawm cov ntaub so ntswg thiab lub cev. Cov kws kho mob paub cov kab mob nram qab no nrog cov txuam nrog cov rog hauv hlwb:

- Gaucher tus kab mob;

- Cov kab mob Thea-Sachs;

- Niemann-Pick kab mob thiab lwm tus.

Carbohydrate Dystrophies

Tag nrho cov carbohydrates uas nyob hauv lub cev tau muab faib ua cov mob polysaccharides (feem ntau yog cov glycogen), glycosaminoglycans (mucopolysaccharides: hyaluronic thiab chondroitin sulfuric acids, heparin) thiab glycoproteins (mucins, xws li mucus, thiab mucoids).

Yuav kom paub txog cov carbohydrates hauv lub hlwb ntawm lub cev, ib qho kev xeem twg yog kwv yees - kev sib tw SHIC. Tus essence ntawm nws yog tias cov ntaub yog kho nrog iodic acid, thiab ces nrog magenta. Thiab tag nrho cov aldehydes tig liab. Yog tias tsim nyog yuav tau cais tawm glycogen, ces amylase yog ntxiv rau cov tshuaj reagents. Glycosaminoglycans thiab glycoproteins raug xuas nrog methylene xiav. Parenchymal carbohydrate dystrophies yog txuam, raws li ib txoj cai, nrog impaired glycogen thiab glycoprotein metabolism.

Hloov kev hloov ntawm glycogen

Glycogen yog lub cev tiv thaiv rau "hnub tshaib plab dub." Feem ntau ntawm nws yog nyob rau hauv daim siab thiab cov leeg thiab siv zog ntawm no lub zog heev. Kev tswj ntawm cov metabolism hauv carbohydrate tshwm sim los ntawm cov kab mob neuroendocrine. Lub luag hauj lwm tseem ceeb tau muab tso rau, xws li niaj zaus, mus rau qhov hypothalamic-pituitary system. Nws ua cov tshuaj hormones tropic uas tswj tag nrho lwm cov qog ntawm kev tso kua mis.

Kev ua txhaum ntawm glycogen kev sib pauv yog nce lossis qis dua nyob rau hauv nws cov nquag hauv cov ntaub so ntswg, as Well as qhov tsos qhov twg nws tsis tsim nyog. Feem ntau meej, xws li cov kev hloov no tshwm sim hauv cov ntshav qab zib mellitus los yog myeditary glycogenoses. Tus kab mob pathogenesis ntawm ntshav qab zib yog to taub zoo: cov kab mob pancreatic nres ua insulin nyob rau hauv tus nqi uas yuav tsum tau, thiab lub zog reserves ntawm lub hlwb yog sai depleted, vim cov qabzib tsis nquag hauv cov nqaij, tab sis tawm ntawm lub cev nrog cov zis. Tus kab mob "nthuav qhia" nws cov reserves, thiab feem ntau qhov parenchymal dystrophy ntawm daim siab npaj. Nyob rau hauv lub nuclei ntawm hepatocytes tshwm kaj kaj, thiab lawv ua lub teeb. Yog li ntawd lawv kuj hu ua "empty nuclei".

Cov kis hniav glycogenoses yog tshwm sim los ntawm qhov tsis muaj los yog tsis muaj enzymes koom tes hauv qhov txuam nrog glycogen. Tam sim no, nws muaj 6 hom kab mob xws li:

- Gierke tus kab mob ;

- Pompe kab mob ;

McDurdle tus kab mob;

- mob kab mob;

- Forbes-Corey tus kab mob;

- Andersen tus kab mob.

Cov kab mob sib txawv yog qhov ua tau tom qab daim siab nqaij hlav thiab kev siv cov tshuaj histoenzymatic.

Kev puas tsuaj ntawm metabolism hauv glycoprotein

Cov no yog parenchymal dystrophies tshwm sim los ntawm lub tsub zuj zuj nyob rau hauv cov ntaub so ntswg ntawm mucins los yog mucoids. Txwv tsis pub, cov dystrophies no kuj hu ua mucous los yog mucoid, vim tias cov xeeb ceem ntawm cov inclusions. Qee lub sij hawm lawv xyuav cov mucins tseeb, tab sis tsuas tshuaj zoo li lawv, uas tuaj yeem ua denser. Hauv qhov no, nws yog ib nqe lus nug ntawm colloid dystrophy.

Kev tsom ntawm cov ntaub so ntswg pub rau peb los txiav txim tsis yog qhov tseeb ntawm qhov muaj cov hnoos qeev, tab sis kuj yog nws lub zog. Vim yog qhov tseeb tias cov khib nyiab ntawm tes, nrog rau kev zais khov tsis pub ua rau cov kua qaub ntawm cov qog, cov hlwv tsim, thiab lawv cov khoom yuav raug mob.

Qhov ua rau hom dystrophy tuaj yeem sib txawv heev, tiam sis feem ntau nws yog ib tug catarrhal o ntawm cov mucous membranes. Tsis tas li ntawd, yog hais tias tus kab mob caj ces, tus qauv pathogenetic uas haum zoo rau hauv lub ntsiab lus ntawm qhov dej degcosity. Qhov no yog cystic fibrosis. Nws cuam tshuam rau txiav, lub plab hnyuv, lub qhov tso zis, kab mob hauv lub cev, hws thiab cov kua qaub qaub.

Txoj kev daws teeb meem ntawm hom kab mob no nyob ntawm seb qhov hnoos qeev thiab qhov nws tso tawm ntev npaum li cas. Qhov tsawg dua lub sij hawm dhau los txij thaum pib ntawm qhov kev mob pathological, qhov ntau dua tias cov mucosa yuav zoo tag nrho. Tab sis qee qhov kev zuaj ntawm epithelium, sclerosis thiab impaired muaj nuj nqi ntawm lub cev raug mob.