ICD (International Classification of Diseases) nephrotic syndrome tsis yog kab mob raum ywj pheej, tab sis ib pawg ntawm cov tsos mob, tag nrho cov uas qhia tias ob lub raum tsis ua haujlwm zoo li lawv yuav tsum.
Cov hlab ntsha me (venules, arterioles thiab capillaries) hauv ob lub raum ua haujlwm ua microfilters, tshem tawm cov co toxins, cov khoom pov tseg thiab cov dej ntau dhau ntawm cov ntshav. Cov khib nyiab thiab dej nkag mus rau hauv lub zais zis thiab tawm hauv peb lub cev nrog cov zis. Feem ntau, yuav tsum tsis muaj protein ntau hauv cov zis.
Lub raum hlab ntsha yog ib feem ntawm glomerular network uas lim cov raum. Thaum lub filtration network puas lawm, protein ntau dhau los ntawm cov lim dej rau hauv cov zis. Qhov tshwm sim ntawm uas yog nephropathic syndrome, piv txwv li kev puas tsuaj loj ntawm cov nqaij ua haujlwm ntawm lub raum (nephrons).
kab mob raum no cuam tshuam rau cov neeg laus thiab menyuam yaus.
Kim mob raum
Ntau tus neeg uas tau kuaj pom tus kab mob no tsis paub txog nws mus txog thaum lawv tau mus kuaj mob niaj hnub ntawm kev kuaj mob niaj hnub.
Cov tsos mob ntawm nephropathology suav nrog:
- Ntau dhau ntawm cov protein ntau hauv cov zis (proteinuria).
- Low plasma protein. Cov ntaub ntawv kho mob yuav hais tias "hypoalbuminemia".
- Cov roj cholesterol siab. Lub sij hawm kho mob rau qhov no yog hyperlipidemia.
- Cov roj ntsha nruab nrab hauv cov ntshav, hu ua triglycerides.
- o ntawm lub ntsej muag, tes, ko taw thiab pob taws.
- Nyob qhov hnyav.
- Nyob ib leeg.
- Ntsuab zis.
- Txo kev tshaib kev nqhis.
Yog tias koj muaj mob nephrotic syndrome ntawm kev sim dav dav, koj tus kws kho mob yuav tsum paub qhov ua rau ntawm qhov teeb meem. Qhov no yuav xav tau kev sim ntxiv thiab cov txheej txheem kuaj mob txhawm rau txhawm rau pom qhov tshwm sim.
Kab mob raum feem ntau tsis muaj cov tsos mob tshwm sim kom txog thaum cov ntaub so ntswg ua haujlwm ntawm lub raum puas loj heev (70-80%).
Txhua tus xav tau cov protein
Muaj ntau hom proteins, peb lub cev siv cov protein ntau ntau, suav nrog kev tsim cov pob txha, cov leeg thiab lwm cov ntaub so ntswg uas ua rau lub cev, thiab tiv thaiv kab mob.
Thaum cov ntaub so ntswg raug mob, ob lub raum tsis ua haujlwm ib txwm, yog li tso cai rau cov protein hu ua albumin dhau los ntawm lawv.filtration system rau hauv cov zis.
Albumin pab lub cev tshem tawm cov kua dej ntau dhau. Nrog rau qhov tsis muaj albumin hauv cov ntshav, cov kua dej ntau hauv lub cev, ua rau o ntawm lub ntsej muag thiab qis ntawm lub cev.
Y Cov roj (cholesterol) yog ib feem tseem ceeb ntawm lub cev
Peb lub cev xav tau cov roj cholesterol, uas yog tsim los ntawm nws tus kheej. Tsis tas li ntawd, cov cholesterol kuj tau noj los ntawm cov zaub mov. Kev noj cov roj (cholesterol) ntau dhau rau hauv cov ntshav ua rau cov hlab ntsha, vim tias cov tee ntawm cov tshuaj no txuas ua ke rau ntawm phab ntsa ntawm cov hlab ntsha thiab cov hlab ntsha thiab tuaj yeem ua rau cov ntshav txhaws (ua tiav lossis ib feem ntawm cov lumen ntawm lub nkoj). Cov ntshav txhaws hauv cov hlab ntsha cuam tshuam kev ua haujlwm ntawm lub plawv thiab cov ntshav ntws mus rau hauv nruab nrog cev thiab cov ntaub so ntswg, uas tuaj yeem ua rau myocardial infarction lossis stroke.
Triglycerides yog hom "zog" rog hauv cov ntshav
Thaum peb noj zaub mov, peb lub cev hlawv calories los ntawm cov khoom noj los tsim kom muaj zog. Yog tias peb noj ntau calorie ntau dua li peb siv, cov calories ntxiv yuav hloov mus ua triglycerides.
Triglycerides khaws cia rau hauv cov ntaub so ntswg adipose, thaum muaj xwm txheej ceev lawv tau siv los ua lub zog los tswj kev ua haujlwm ntawm tes. Lub xub ntiag ntawm cov qib triglycerides hauv cov ntshav qhia tias muaj kev pheej hmoo siab rau kab mob plawv.
Leej twg yog tus mob nephrotic syndrome?
Txhua tus neeg ntawm txhua lub hnub nyoog, poj niam txiv neej thiab pab pawg neeg tuaj yeem ua rau muaj kab mob no, tab sis raws li Lavxias Ministry of He alth(MoH), ntau dua ntawm cov txiv neej dua poj niam.
Nephrotic syndrome hauv cov menyuam yaus feem ntau tshwm sim thaum muaj hnub nyoog 2 txog 6.
Qee yam ua rau muaj feem yuav kis tau tus kab mob raum, cov no suav nrog:
- Nephropathology (glomerulonephritis, nephrolithiasis, thiab lwm yam).
- YUrolithiasis - urolithiasis.
- siv sijhawm ntev ntawm cov tshuaj xws li cov tshuaj tsis-steroidal anti-inflammatory (NSAIDs) thiab tshuaj tua kab mob.
- Kab mob: HIV, kab mob siab viral, malaria.
- Y Ntshav Qab Zib, lupus thiab amyloidosis.
Etiological yam (ua rau)
Syndrome tuaj yeem tshwm sim los ntawm ntau yam kab mob raum thiab lwm yam.
Yog tias tus kab mob cuam tshuam rau lub raum nkaus xwb, ces lawv hu ua thawj qhov ua rau mob nephrotic. Lwm yam uas cuam tshuam rau tag nrho lub cev, suav nrog ob lub raum, yog hu ua qhov ua rau thib ob.
Cov neeg feem coob raug mob raum mob siab vim yog qhov ua rau theem nrab.
Qhov feem ntau tshwm sim hauv cov neeg laus yog ib yam mob hu ua focal segmental glomerulosclerosis (FSGS). FSGS ua rau microscopic caws pliav ntawm lub raum lim hu ua glomeruli.
Ntau yam kab mob autoimmune thiab kab mob tiv thaiv kab mob ntev tuaj yeem ua rau lub raum puas tsuaj loj.
Amyloidosis yog ib yam kab mob txiav txim siab caj ces uas muaj cov khoom sib txuam ntawm cov protein hu ua amyloid hauv cov ntshav. Nws yog tso rau ntawm phab ntsa ntawm cov hlab ntshantau lub cev, suav nrog raum.
Qhov thib ob feem ntau ntawm cov neeg laus yog ntshav qab zib. Cov kab mob pathology yog nrog los ntawm kab mob raum hu ua lub raum (lub raum) ntshav qab zib.
Qhov feem ntau ua rau lub raum mob rau cov menyuam yaus yog tus kab mob hloov me me (MCD). Tus kab mob hloov pauv tsawg kawg nkaus ua rau lub raum puas tsuaj uas tsuas pom tau nrog lub tshuab tsom iav uas muaj zog heev.
Qhov thib ob feem ntau ntawm cov menyuam yaus yog ntshav qab zib.
Nyob rau hauv txhua daim ntawv, lub ntsiab lus sib koom ua ke ntawm tus kab mob no yog qhov kev puas tsuaj loj ntawm glomeruli.
Cov kab mob ntawm lub raum uas cuam tshuam rau cov tubules thiab interstitium, xws li interstitial nephritis, tsis ua rau mob nephrotic.
Kev kuaj mob muaj peev xwm
- Qhov Kev Ntsuas Glomerular Filtration (eGFR) yog ib qho kev sim sai los ntsuas lub raum ua haujlwm. Koj tus eGFR yog tus lej raws li kev txheeb xyuas ntawm koj cov ntshav creatinine thiab qib urea. Thawj cov zis yog tsim los ntawm kev lim ntshav plasma hla lub glomerular barrier; Hauv tib neeg, glomerular filtration rate (GFR) yog 125 ml / min.
- Kev kuaj zis tso zis. Nrog rau lub raum mob hnyav, cov protein ntau nkag mus rau hauv cov zis. Qhov no tej zaum yuav yog ib qho ntawm cov tsos mob ntxov tshaj plaws ntawm lub raum nephrotic syndrome. Txhawm rau txheeb xyuas qhov muaj protein ntau hauv cov zis (lub npe hu ua proteinuria), nws yog qhov yuav tsum tau ua kom pom cov zis dav dav nrog cov zis.sediment microscopy. Tus nqi physiological ntawm plasma albumin yog 0.1%, uas ib txwm tuaj yeem dhau los ntawm glomerular filtration barrier.
- kuaj xyuas lub raum thiab zais zis rau kev kuaj mob nephrotic syndrome. Tso cai rau koj los ntsuas qhov morphological (structural) lub xeev ntawm lub raum thiab cov ntshav ncig. Ultrasound tseem yuav pab txheeb xyuas cov kab mob sib kis ntawm cov zis.
Koj tuaj yeem xav tias lub raum pathology tom qab kuaj cov zis nrog cov ntawv xeem. Nrog rau tus nqi siab proteinuria siv, daim kab xev kuaj yuav hloov xim.
Kev kuaj ntshav kuaj pom cov qib qis ntawm cov protein hauv cov ntshav hu ua albumin yuav lees paub qhov kev kuaj mob.
Ib txhia zaus, thaum txoj kev kho mob tsis zoo, lub raum yuav raug txiav txim. Txhawm rau ua qhov no, ib qho qauv me me ntawm lub raum cov ntaub so ntswg raug tshem tawm nrog rab koob thiab saib hauv qab lub tshuab ntsuas.
Lub raum proteinuria yog qhov poob ntawm peb lossis ntau dua grams ntawm cov protein nyob rau ib hnub los ntawm cov zis los yog, hauv ib qho kev tso zis, muaj 2 grams protein ib gram ntawm cov zis creatinine.
Nephrotic syndrome yog tus cwj pwm los ntawm kev sib xyaw ntawm nephrotic ntau yam proteinuria nrog cov ntshav hypoalbuminemia thiab edema ntawm lub ntsej muag qhov chaw thiab hauv qab ntawm lub cev.
Cov yam tseem ceeb ntawm lub raum syndrome
Proteins ua ntau yam haujlwm sib txawv. Thaum cov ntshav (ntshav) cov protein tsawg tsawg, lub cev ua rau muaj teeb meem nrog cov ntshav txhaws thiab txhim kho.kab mob (xav tias cov protein feem ntawm cov ntshav suav nrog immunoglobulins - lub ntsiab hlwb ntawm lub cev tiv thaiv kab mob).
Cov kab mob uas muaj kab mob ntau tshaj plaws yog mob sepsis, mob ntsws thiab mob peritonitis.
Venous thrombosis thiab pulmonary embolism (PE) yog qhov paub zoo ntawm cov kab mob nephrotic syndrome.
Lwm yam teeb meem suav nrog:
- Anemia (anemia).
- Cardiomyopathy, suav nrog ischemia.
- ntshav siab - systemic hypertension.
- Chronic edema.
- mob raum tsis ua haujlwm (ARF, CRF).
Kev xaiv kho mob raum
Tsis muaj kev kho mob tshwj xeeb rau nephrotic syndrome, txhua yam kev kho mob feem ntau tsuas yog cov tsos mob (tshwm sim cov tsos mob thiab teeb meem) thiab tiv thaiv (tiv thaiv ntxiv cov ntaub so ntswg puas tsuaj).
Nws yog ib qho tseem ceeb kom paub tias ua kom lub raum tsis ua haujlwm (mob raum kawg) yuav tsum tau lim ntshav thiab hloov lub raum ntxiv kom cawm tau txoj sia.
Tus kws kho mob tau sau tshuaj los txo cov tsos mob. Cov no yuav suav nrog cov tshuaj los tswj ntshav siab thiab cov roj cholesterol kom txo tau txoj kev pheej hmoo ntawm kab mob plawv.
Cov tshuaj txo qis ntshav siab hu ua ACE (angiotensin-hloov enzyme) inhibitors thiab ARBs (angiotensin II receptor blockers), uas txo qis capillary siab thiabtiv thaiv kev tso tawm cov protein rau hauv cov zis.
Diuretics tau muab tshuaj los pab lub cev tshem tawm cov dej ntau dhau, nrog rau tswj ntshav siab thiab txo qhov o.
cov tshuaj ntshav thinning (anticoagulants) raug pom zoo nyob rau hauv cov xwm txheej uas muaj kev pheej hmoo ntawm cov ntshav txhaws los tiv thaiv lub plawv nres (myocardial infarction) thiab mob stroke.
Kev hloov pauv hauv kev noj haus ua lub luag haujlwm tseem ceeb hauv kev kho mob; Kev noj zaub mov tsis muaj rog pab tswj cov ntshav cholesterol. Xaiv ntses los yog nqaij ntshiv.
txwv ntsev (sodium chloride) kom tsawg kom txo tau cov edema thiab ua kom ntshav siab nyob rau theem noj qab haus huv.
Cov tshuaj tiv thaiv kab mob tiv thaiv cov lus teb ntau dhau ntawm lub cev tiv thaiv kab mob, nrog rau glomerulonephritis thiab kab mob lupus erythematosus, xws li glucocorticosteroids (Prednisolone, Decortin, Medopred, thiab lwm yam).
Yuav ua li cas tiv thaiv lub raum puas tsuaj?
Txoj kev tiv thaiv tus mob no tsuas yog tiv thaiv kab mob uas tuaj yeem ua rau nws.
Yog tias koj muaj kab mob uas tuaj yeem ua rau koj lub raum tsis zoo, nrog koj tus kws kho mob los tsim cov txheej txheem kho mob nephrotic syndrome los tswj koj cov kab mob hauv qab thiab tiv thaiv lub raum puas.
Tseem nrog koj tus kws kho mob tham txog kev kuaj mob raum ua haujlwm.
Qhov no tseem ceeb heevrau cov neeg muaj ntshav qab zib, ntshav siab, lossis tsev neeg keeb kwm ntawm mob raum. Kev puas tsuaj rau lub raum yog ib txwm irreversible, lawv cov hlwb tsis zoo tom qab tuag. Tab sis yog tias koj muaj tus kab mob hauv qab tau kuaj pom nyob rau theem pib thiab raug kho raws sijhawm, ces muaj txoj hauv kev los tiv thaiv tus mob kom tsis zoo.
raum pathology hauv menyuam yaus
Txawm hais tias nephrotic syndrome tuaj yeem cuam tshuam rau tib neeg txhua lub hnub nyoog, feem ntau nws yog thawj zaug kuaj pom hauv cov menyuam yaus hnub nyoog 2 txog 5 xyoos.
Pathology cuam tshuam rau cov tub ntau dua li cov ntxhais. Txhua xyoo, kwv yees li 50,000 tus menyuam yaus raug kuaj pom tias muaj glomerulonephritis nrog nephrotic syndrome. Nws nyiam ntau dua hauv cov tsev neeg uas muaj keeb kwm ntawm lub raum lossis kab mob autoimmune, lossis hauv cov neeg Esxias diaspora, txawm hais tias nws tseem tsis tau paub meej vim li cas.
Cov tsos mob ntawm lub raum hauv cov menyuam yaus
Zoo li cov neeg laus, qhov o yog thawj zaug pom ntawm lub qhov muag, tom qab ntawd hauv ob txhais ceg thiab lub cev.
Immunoglobulins yog cov tshuaj tiv thaiv uas yog ib pawg tshwj xeeb ntawm cov proteins hauv cov ntshav uas tiv thaiv kab mob. Thaum lub cev poob cov protein ntau, cov menyuam yaus yuav kis tau tus kab mob ntau dua.
Muaj cov zis hloov - qee zaum muaj cov protein ntau hauv cov zis ua rau nws ua kom khov.
Feem ntau cov menyuam yaus uas muaj tus mob nephrotic muaj "mob hloov pauv tsawg kawg nkaus". Qhov no txhais tau hais tias lawv ob lub raum zoo li qub los yog ze li qub ntawm cov kev ntsuam xyuas kom txog thaum cov ntaub so ntswg tau txais los ntawm biopsytsis kuaj nyob rau hauv lub tshuab microscope. Qhov ua rau tus kab mob hloov me ntsis yog tsis paub.
In Finnish-type hereditary nephrotic syndrome, the gene for nephrin, a filter gap protein, mutates, ua rau mob raum thaum menyuam mos.
Nws kuj tshwm sim vim muaj teeb meem raum lossis lwm yam mob xws li:
- glomerulosclerosis - thaum cov qauv sab hauv ntawm lub raum ua puas;
- glomerulonephritis - mob hauv lub raum pom;
- kab mob xws li HIV lossis kab mob siab B thiab C;
- Ysystemic lupus erythematosus;
- mob ntshav qab zib;
- sickle cell anemia;
- Nyob tsis tshua muaj neeg, qee yam mob qog noj ntshav, xws li leukemia, ntau yam myeloma, lossis lymphoma.
Tab sis cov teeb meem no tshwm sim rau cov neeg laus dua li cov menyuam yaus.
Cov tsos mob ntawm nephrotic syndrome hauv cov menyuam yaus tuaj yeem tswj tau nrog cov tshuaj steroid.
Feem ntau cov menyuam yaus teb tau zoo rau cov tshuaj steroids thiab kev pheej hmoo ntawm lub raum tsis ua haujlwm tsawg. Txawm li cas los xij, ib tug me me ntawm cov me nyuam muaj (kwv yees) congenital nephrotic syndrome, thiab nws zoo li tsis tshua teb rau kev kho. Thaum kawg, lawv cov tsos mob yuav xaus rau lub raum tsis ua haujlwm, thiab cov menyuam yaus yuav tsum tau hloov lub raum.
Nyob rau hauv cov menyuam yaus feem ntau uas teb tau zoo rau kev kho mob, cov tsos mob tau tswj tau, muaj kev tso cai - kev ncua ib ntus ntawm kev loj hlob ntawm tus kab mob, tom qab ib ntus cov tsos mob rov qab los - rov tshwm sim.
BFeem ntau, kev rov ua dua tshiab tsis tshua muaj thaum cov menyuam loj hlob tuaj, thiab nephrotic syndrome feem ntau daws tau thaum hluas.
Pathological Condition Control
Cov niam txiv yuav tsum coj lawv tus menyuam mus rau tus kws kho mob tshwj xeeb (pediatric nephrologist) kom muaj lus qhia txog nephrotic syndrome, kuaj thiab kho tshwj xeeb.
Kev kho mob tseem ceeb yog steroids (glucocorticosteroids), tab sis kev kho mob ntxiv tuaj yeem siv tau yog tias tus menyuam muaj kev phiv loj heev.
Cov menyuam yaus feem ntau rov qab ua ntej thaum hluas thiab xav tau cov tshuaj steroids thaum lub sijhawm no.
Cov menyuam yaus uas muaj tus mob nephrotic feem ntau yog muab tsawg kawg 4 lub lis piam ntawm prednisolone ua raws li kev txo koob tshuaj txhua hnub rau plaub lub lis piam ntxiv. Qhov no tiv thaiv cov proteinuria.
Thaum Prednisolone tau muab rau lub sijhawm luv, feem ntau tsis muaj kev phiv loj lossis ntev mus ntev, txawm tias qee tus menyuam yaus ntsib:
- nce qab los;
- hnyav nce;
- ntsej muag liab;
- frequent mood swings.
Cov menyuam yaus feem ntau teb tau zoo rau kev kho mob nephrotic syndrome nrog Prednisolone, nrog cov protein feem ntau ploj ntawm lawv cov zis thiab o ploj hauv ob peb lub lis piam. Lub sijhawm no, kev zam txim tshwm sim.
Y Diuretics, lossis diuretics, kuj tuaj yeem siv los txo qhov tsimkua. Lawv ua hauj lwm los ntawm kev nce cov zis ntau lawm.
Penicillin yog tshuaj tua kab mob thiab tuaj yeem muab tau thaum lub sijhawm rov qab los kom txo tau txoj kev kis kab mob.
Khoom noj khoom haus tseem ceeb. Txo cov ntsev hauv koj tus menyuam cov zaub mov kom tsis txhob muaj dej ntxiv thiab edema.
