Parenchymal dystrophy. Pathological anatomy

Dzimwe nguva mumakiriniki maitiro kune chimwe chinhu chakadai separenchymal dystrophy. Kuongorora kwepathological kunorondedzera kune zvinetso zvemagetsi mumasero. Mumashoko akajeka, muviri unotambudzwa nekugadzirisa kwekudya uye kuwedzera kwezvibereko, zvinotungamirira ku morphological (kuona) kuchinja. Kuzarura zvirwere zvakadaro zvinogona kuitika muchikamu kana mushure mechidimbu chekuongorora kwakanyanya. Kuora mwoyo uye stromal-vascular dystrophy inonzera zvirwere zvakawanda zvinouraya.

Tsanangudzo

Kunyora dystrophies kune mararamiro anokonzera kushanduka muurongwa hwemasero ezvomuviri. Pakati pezvirongwa zvekuvandudzwa kwechirwere, kushungurudzwa kwekuzvidzivirira kwemasero ane simba rekushaya simba, fermentopathies, dyscirculatory disorders (ropa, lymph, interstitium, intercellular fluid), endocrine uye cerebral dystrophies inosarudzwa.

Kune nzira dzakasiyana-siyana dzekuita dystrophy:

- kupindira, ndiko kuti, kutakura kwakanyanya kwezvinhu zvinoshandiswa mumagetsi kubva muropa kusvika mukati memasero kana nzvimbo inoshandiswa inokonzerwa nekushaya kushanda mumagadzirirwo emuviri wemuviri;

- kukanganisa, kana Phaneroz, ndiko kuparadzaniswa kwezvivako zvemukati, izvo zvinotungamirira mukuvhiringidzwa kwemasabetism uye kuunganidza kwezvinhu zvisiri pasi-oxyidized zvigadzirwa zvemagetsi;

- iyo yakatsauka chikamu chezvinhu, izvo zvinowanzo sero risingabereki;

- kuchinja kwezvibereko zvinopinda musero kwekuvakwa kwechimwe rudzi rwezvigadzirwa zvekupedzisira (mapuroteni, mafuta kana makhahydrates).

Kurongwa

Pathomorphologists anosiyanisa mhando dzinotevera dze dystrophies dzinotyisa:

1. Zvichienderana ne morphological change:

- purely parenchymal;

- stromal-vascular;

- yakavhenganiswa.

2. Nechiratidzo chezvinhu zvakaunganidzwa:

- Protein kana dysproteinosis;

- mafuta kana mapidzi;

- carbohydrate;

- mineral.

3. Nokupararira kwekuita:

- system;

- Local.

4. Nguva yekuonekwa:

- Akawana;

- congenital.

Izvi kana zvimwe zvinokonzera dystrophies pathological anatomy hazvirevi chete zvinokonzera zvinokanganisa, asiwo izvo zvakasviba masero akabatwa. Kuchinja kweimwe dystrophy kune imwe ndeyekuti inogona kuitika, asi kungobatanidzwa chete kunokwanisika. Kuora mwoyo kwepystrophies ndiyo inonyanya kukosha iyo inowanikwa muchitokisi, asi inongova chikamu chechirwere chetachiona chinosanganisira morphological uye kushanda kusakwanisa kwechimwe chimiro.

Dysproteinases

Muviri wemunhu kune chikamu chikuru unoumba mapuroteni nemvura. Mapuroteni mamolekemu ndiwo anowanikwa masero masvingo, membrane ye mitochondria nedzimwe organelles, uyezve, vari mune rusununguko mu cytoplasm. Sezvo mutemo, idzi idzi dzine enzymes.

Dysprotainosis inonziwo chirwere chepachirwere, seprisystine protein dystrophy. Uye chimiro chayo chiri muchokwadi chokuti mapuroteni emagetsi anoshandura mararamiro avo, pamwe nekugadziriswa kwemugadziro, zvakadai sekuchenesa kana kuparadzanisa. Kuti mapuroteni parenchymal dystrophies anosanganisira hyaline-droplet, hydropic, horny uye granular dystrophy. Zvitatu zvekutanga zvichave zvakanyorwa zvakanyatsorondedzerwa, asi iyo yekupedzisira, granular, inoratidzwa nekuunganidza kweprotini mumasero, izvo zvinokonzera masero kutambanudza, uye nhengo inowedzera, inotyisa, inopera. Ndicho chikonzero granular dystrophy inonziwo kuputika kwemadiki. Asi masayendisiti havana chokwadi kuti uku ndiko kunetseka kwekuita dystrophy. Iyo pataniatomy yeiyo nzira ndeyokuti zviyo zvinogona kutora kubhadharwa zvakanyanya kuwedzera masimba emagetsi semhinduro kugadzirisa kushanda.

Hyaline-drop dystrophy

Nechirudzi urwu rwekushaya ropa, madonhwe makuru e hyaline anoonekwa mumasero, ayo anozopedzisira akabatanirana uye anozadza yose mukati memusero, achidzinga organelles kana kuaparadza. Izvi zvinotungamirira mukurasikirwa kwebasa uye kunyange kushanda kwesero. Kazhinji kacho, chirwere chinowanikwa mumakumbo emagunwe, kashoma muropa uye mwoyo.

Munguva yekuongorora maitiro e-kidney shure kwepuropi biopsy, pamwe nekuunganidza kwe hyaline mu nephrocytes, kuparadzwa kwezvinhu zvose zvemasero zvinowanikwa. Izvi zvinowanzoitika kana murwere achiwedzera vacuolar-lysosomal insufficiency, izvo zvinoita kuti kuderedzwa kwekugadziriswa kwemapurotini kubva mumuguta wekutanga. Kazhinji kacho, chirwere ichi chinoitika muNephrotic syndrome. Kunyanya kuongororwa kwevarwere vakadai ndeye glomerulonephritis uye amyloidosis yeitsvo. Kuonekwa kwechirwere ne hyaline-droplet dystrophy hakushanduki.

Mumakamuri, iyo nzira yakasiyana zvakasiyana. Munguva iyo microscopy, miviri yaMallory, yakagadzirwa nemagetsi uye doro rinodhaka, inowanikwa mavari. Kuonekwa kwavo kunobatanidza nechirwere chaWilson-Konovalov, chidhakwa chehepatitis, pamwe chete nebhaariary uye Indian cirrhosis. Mugumisiro wechirongwa ichi hauna kunaka - necrosis yemasero eropa, kurasikirwa kwebasa racho.

Hydropic degeneration

Rudzi urwu rwekushaya dystrophy rwakasiyana nemamwe ose mumagetsi matsva aya azere nemvura inowanikwa mumasero akabatwa. Kazhinji kacho, chiitiko ichi chinogona kuonekwa muganda uye tubules yeinso, mumasero echiropa, musimba uye adrenals.

Yakakura zvishoma, masero akawedzerwa, cytoplasm yavo yakazadzwa nemapuuoles ane zviri pachena mvura iri mukati. Nheyo yacho inodzingwa kana kuti yakabviswa, zvimwe zvivako zvinobviswa. Pakupedzisira, sero i "bhoron" yakazara nemvura. Saka, hydropic dystrophy dzimwe nguva inonzi bhaluni dystrophy.

Zvinyorwa zvakanyanya, nhengo dzinenge dzisingashanduki. Iyo nzira yekusimudzirwa kwechirwere ichi kukanganisa kwe-colloid-osmotic mhepo muchitokisi uye munzvimbo ye intercellular. Nemhaka yeizvi, kuperevedza kwemasero kunowedzera, membrane inoputika uye masero anofa. Zvimwe zvinokonzera makemikari akadaro kuchinja angave ari glomerulonephritis, chirwere cheshuga mellitus, iroyo amyloidosis. Muropa, kushandura masero kunoshandiswa nehutachiona uye chepfu yehupatitis. Pamusoro peganda, kusviba kwe hydropic kunogona kukonzerwa nehutachiona hwechibhokisi.

Izvi zvinoguma nehana kana kuti necrosis yakazara, saka morphology uye basa rezvikamu zvinowedzera nokukurumidza.

Horn dystrophy

Chirwere chinonzi pathological keratinization yezvipembenene ndeyekuwedzera kuwanda kwezvinhu zvinokonzerwa nehuni muhupamusoro pepakati peganda, somuenzaniso, hyperkeratosis kana ichthyosis, uyewo kuonekwa kwezvinhu zvinokonzera iyo inenge isingawanzovipo - pane mucous membrane (leukoplakia, squamous cell carcinoma). Izvi zvinogona kuva zvose zviri mukati uye zvese.

Zvimwe zvinokonzera rudzi urwu rwezvirwere zvinogona kukanganisa ectodermal rudiment munguva ye embryogenesis, kusingaperi kuora kwechirwere kuchinja mumasukisi, zvirwere zvehutachiona uye kushaya mavitamini.

Kana kurapwa kunotanga pakarepo mushure mekuonekwa kwechiratidzo chekutanga, maviri acho anogona kugonazve, asi mumatambudziko akareba, kupora hakugoneki. Nzvimbo dzenguva refu dziripo yehwamanda dystrophy dzinogona kukonzera mukenza weganda, uye congenital ichthyosis haina kufanana nehutachiona hupenyu.

Hereditary dystrophies

Hereditary parenchymal dystrophies inomuka kubva kuhutano hwekuzvarwa. Zvirwere izvi zvinonziwo kuunganidza zvirwere, nokuti kushungurudzika kwemagetsi, zvigadzirwa zvemagetsi zvinowedzera mumasero uye mvura inoshandiswa mumuviri, ichiipisa. Vamiririri vanozivikanwa zvikuru veboka iri ndezve phenylketonuria, tyrosinosis, uye cystinosis.

Nhengo dzakatarirwa dze phenylketonuria ndiyo inoshungurudza manzwiro, masimba, ganda uye mvura (ropa, urini). Iyo metabolites ye tyrosinosis inounganidza mumasero emuropa, itsvo nemapfupa. Cystinosis inobatawo chiropa netsvo, asi kunze kwavo, spleen, eyeballs, bone marrow, lymphatic system uye ganda zvinotambura.

Lipidoses

Lipidhi dziri muchitokisi chimwe nechimwe, dzinogona kunge dzakasiyana kana kuti dzakabatana nemapuroteni uye dzive zvivako zvemukati membrane, pamwe chete nedzimwe nzira. Mukuwedzera, glycerin nemafuta acids zvinowanika mu cytoplasm. Kuti vazvione mumasukisi, kugadziriswa kwakananga uye nzira dzinoshandiswa dzinoshandiswa, somuenzaniso, dema kana kuti tsvuku dzvuku, osmic acid, Nile bluu sulphate. Mushure mekugadzirira kwakanyatsogadziriswa, magadzirirwo anonyatsoongororwa mu electros microscope.

Kupera kwemafuta kunotyisa kunoratidzika muhuwandu hwekuwedzerwa kwemafuta kunzvimbo dzavanofanira kuva, uye kuonekwa kwe lipids kwavasingafaniri kuva. Sezvo mutemo, mafuta asingatsigiri anowedzera . Nhengo dzechinangwa chacho zvakafanana neprotheni dystrophy - moyo, itsvo uye chiropa.

Fat parenchymal dystrophy yemakatoriyamu inotanga nechitarisiko myocytes yemadonhwe maduku akawanda emafuta, anonzi. Kuwedzera kuneta. Kana nzira yacho isingamiri panguva iyi, pakupedzisira madonhwe anounganidza uye ave makuru kusvika pavanenge vaine cytoplasm yose. Organelles munyaya iyi akaparadzana, kupesana kwemishupa yemasisipo kunopera. Chirwere chacho chinoratidzwa munzvimbo iri pedo nemubhedha wemavhutu ane mhepo.

Zvinyoronyoro, kupera kwemafuta kunotyisa kunozviratidza nenzira dzakasiyana, zvose zvinoenderana nesiteji rekuita. Pakutanga, kuongororwa kunogona kuitwa chete pasi pekroscope, asi pakupedzisira mwoyo unokura nekuda kwekutenderera kwemakamuri, masvingo ayo anova akaonda uye aine simba, asi kudyiwa kwemyocardium kunoratidza tsvina tsvina yejena. Pathophysiologist yakauya nezita rechiri ichi: "tiger moyo".

Dystrophy yemafuta yezvipembenene zvinowedzera maererano nemitatu mikuru.

1. Kuwedzera kwekudya kwemafuta emafuta emasiyopu.
2. Kushungurudzwa kwemafuta emasabolism.
3. Kusiyana kwemapoprotein zvivako mukati memusero.

Kazhinji kacho nzira idzi dzinokonzerwa panguva ye hypoxia, utachiona (diphtheria, tuberculosis, sepsis) uye kudhakwa kwemuviri ane chlorine, phosphorus kana arsenic.

Sezvo mutemo, mafuta dystrophy anonzwisisika, uye kushungurudzika kwemasero masero kunodzorerwa nenguva. Asi kana chirongwa ichi chisina kutanga, ipapo zvose zvinopera nekufa kwezviviri uye nhengo. Vanachiremba vanosiyanisa zvirwere zvinotevera zvinosangana nekuunganidza kwemafuta mumasero:

- Gaucher's disease;

- Thea-Sachs chirwere;

- Niemann-Pick chirwere nevamwe.

Carbohydrates Dystrophies

Zvose gorocidhi zviri mumuviri zvinogona kugoverwa mu polysaccharides (inowanzozivikanwa ndeye glycogen), glycosaminoglycans (mucopolysaccharides: hyaluronic uye chondroitin sulfuric acid, heparin) uye glycoprotein (mucins, kureva mucus, uye mucoids).

Kuti uzive makhahydrates mukati memasero emuviri, kuedza kwakananga kunoitwa - kushandiswa kweSIC. Chinokosha chazvo ndechokuti machira anobatwa ne iodic acid, uye ipapo nemagenta. Uye ose aldehydes anoshanduka. Kana zvakakosha kudzivisa glycogen, ipapo amylase yakawedzerwa kune reagents. Glycosaminoglycans uye glycoprotein zvakasvibiswa nemethylene bluu. Parenchymal carbohydrate dystrophies inosanganiswa, sekutonga, isina simba glycogen uye glycoprotein metabolism.

Kushungurudzika kweglycogen kushandiswa

Glycogen ndiyo nzvimbo yemuviri ye "zuva rakati rava nenzara." Zvizhinji zvezvo zvakachengetwa muropa uye musimba uye zvinopedza simba iri zvishoma zvishoma. Mutemo wemagufihydrate metabolism unoitika kuburikidza neurouroendocrine system. Basa guru rinogoverwa, sezvinowanzoitwa, kune hypothalamic-pituitary system. Inobereka mahomoni anotonhora anotarisa mamwe mazamu evhari mukati.

Kupesana kweglycogen kushandiswa ndiko kuwedzera kana kuderera kwehuwandu hwayo mumatumbu, pamwe nekuonekwa kwaasingafaniri kuva. Zvinyatsojeka, kuchinja kwakadaro kunoratidzwa mune chirwere cheshuga mellitus kana kuti glycogenoses. Iyo pathogenesis yechirwere cheshuga inonyatsonzwisiswa: pancreatic masero anomira kubudisa insulini mumutengo unodiwa, uye simba rekuchengetedzwa kwemasero rinokurumidza kupera, sezvo glucose isinganamati mumatumbu, asi inobudiswa kubva mumuviri nemuyo. Izvo zvipenyu "zvinoratidzira" zvayo, uye kunyanya kuderedza dystrophy yechiropa inowedzera. Muchikamu che hepatocytes vanoita sezvakajeka, uye vanova chiedza. Saka ivo vanonziwo "isina chinhu".

Hereditary glycogenoses inokonzerwa nokushaiwa kana kusavapo kwemakemikari anobatanidzwa mukuunganidza glycogen. Parizvino, kune zvirwere zvakadaro:

- Girke's disease ;

- Pompe chirwere ;

McDurdle's disease;

- Her chirwere;

- Chirwere cheForbes-Corey;

- Chirwere chaAndersen.

Kusiyana kwavo kuongororwa kunogona kuitika mushure mekushaya chiropa chinoshandiswa uye kushandiswa kwestoenzenzymatic analysis.

Kushungurudzika kweglycoprotein metabolism

Idzi idzi dzakanyanyisa kunetseka dzinokonzerwa nekuunganidza mumatumbu e mucins kana mucoids. Kana zvisina kudaro, idzi dystrophies dzinonziwo mucous kana mucoid, zvichienderana nehutano hwekushambadzira. Dzimwe nguva vanozvibatanidza pamakungu echokwadi, asi zvinhu zvakangofanana nazvo, izvo zvinogona kuva zvakanyanyisa. Muchiitiko ichi, iri mubvunzo wekolloid dystrophy.

Microscopy yemaviri inotitendera kuti tisarudze chokwadi chekuvapo kwekamu, asiwo zvayo. Pamusana pekuti zviyero zvemasero, pamwe chete nechakavanzika chepiscous chinodzivirira kubuda kwechidimbu kwehutachiona kubva kune gland, majee anogadzirwa, uye zviri mukati mazvo zvinowanzove zvichisvibiswa.

Zvimwe zvinokonzera rudzi urwu dystrophy zvinogona kunge zvakasiyana kwazvo, asi kazhinji kacho chirwere checatarrha chekudzivirira kwemukati. Mukuwedzera, kana chirwere chinotora nhaka, chirwere chepathogenetic chinowirirana zvakakwana mukududzirwa kwemucosal degeneration. Icho cystic fibrosis. Iyo inokanganisa pancreas, mumatumbo chubhu, mumugwagwa wemuguta, bile ducts, swet uye maronda emvura.

Kugadziriswa kwechirwere ichi chinokonzerwa nehuwandu hwehutu uye nguva yekusunungurwa kwayo. Nguva shoma yapfuura kubva pakutanga kwehutachiona hwehutachiona, zvinowanzoitika kuti mucosa ichapora zvachose. Asi pane dzimwe nguva kuderera kwe epithelium, sclerosis uye kusina simba kwechikamu chakakanganiswa zvinowanikwa.