Xav tias qhov no yog nephrotic syndrome. Qhov no yog ib qho nyuaj ntawm cov tsos mob uas tshwm sim nrog rau lub raum puas thiab muaj xws li loj proteinuria, edema, thiab impaired protein thiab lipid metabolism. Cov txheej txheem pathological yog nrog los ntawm dysproteinemia, hyperlipidemia, hypoalbuminemia, edema ntawm qhov chaw sib txawv (txog rau cov kua dej ntawm cov kab noj hniav), dystrophic hloov hauv cov mucous daim nyias nyias thiab ntawm daim tawv nqaij. Hauv kev kuaj mob, lub luag haujlwm tseem ceeb tshaj plaws yog ua los ntawm kev soj ntsuam thiab kuaj daim duab: cov tsos mob ntawm lub raum thiab lub raum, kev hloov pauv hauv biochemical tsom xam ntawm cov ntshav thiab zis, cov ntaub ntawv rau lub raum biopsy. Kev kho rau nephrotic syndrome feem ntau yog kev saib xyuas. Nws suav nrog kev teem caij diuretics, noj zaub mov, kev kho mob infusion, corticosteroids, tshuaj tua kab mob, cytostatics.
Mob nephrotic syndrome feem ntau tshwm sim nrog ntau yamsystemic, urological, kis kab mob, mob metabolic, kab mob suppurative. Nyob rau hauv niaj hnub urology, xws li cov tsos mob complex ua rau nyuaj rau lub raum kab mob, uas yog pom nyob rau hauv kwv yees li 20% ntawm cov neeg mob. Tus kab mob no feem ntau tshwm sim hauv cov neeg laus tom qab 30 xyoo, tsawg dua hauv cov neeg laus thiab cov menyuam yaus. Nyob rau tib lub sijhawm, muaj ib qho classic tetrad ntawm kev kuaj pom: proteinuria (ntau dua 3.5 g / hnub), hypoproteinemia thiab hypoalbuminemia (tsawg dua 60-50 g / l), hyperlipidemia (cholesterol ntau dua 6.5 mmol / l), o. Thaum tsis muaj qee qhov kev tshwm sim no, cov kws tshaj lij hais txog qhov txo qis nephrotic syndrome (tsis tiav). Qhov ua rau nephrotic syndrome yuav raug txiav txim siab ntxiv.
Kev tshwm sim
Yuav kom nkag siab tias qhov no yog kab mob nephrotic, cia peb tshawb nrhiav qhov ua rau tus kab mob no. Los ntawm qhov xwm txheej ntawm nws keeb kwm, tus kab mob yog thawj (kev nyuaj siab ntawm lub raum pathologies) thiab theem nrab (raws li cov kab mob tshwm sim nrog kev koom tes ntawm ob lub raum ntawm qhov xwm txheej thib ob). Thawj kab mob tshwm sim nrog pyelonephritis, glomerulonephritis, thawj amyloidosis, mob raum (hypernephroma), nephropathy ntawm cov poj niam cev xeeb tub. Tsis tas li ntawd, nephrotic syndrome yog congenital, thiab nyob rau hauv cov ntaub ntawv no, lub pathology yog vim muaj cov yam ntxwv thiab cov yam ntxwv ntawm lub sij hawm cev xeeb tub.
Dab tsi ua rau cov tsos mob thib ob?
Cov tsos mob thib ob feem ntau tshwm sim los ntawm ntau yam kab mob:
- mob rheumatic thiab collagenoses (SLE, hemorrhagic vasculitis, nodularperiarteritis, rheumatism, scleroderma, mob caj dab rheumatoid);
- cov txheej txheem suppuration (lub ntsws abscesses, bronchiectasis, septic endocarditis);
- kab mob ntawm lymphatic system (lymphogranulomatosis, lymphoma);
- kab mob parasitic thiab kis kab mob (malaria, tuberculosis, syphilis).
Ntau zaus, nephrotic syndrome tshwm sim tawm tsam keeb kwm ntawm kev kho tshuaj, nrog rau kev ua xua hnyav, hnyav hlau lom (tshuaj txhuas, mercury), muv thiab nab stings. Hauv cov menyuam yaus, qhov ua rau kev loj hlob ntawm tus kab mob feem ntau tsis tuaj yeem txheeb xyuas, yog li cov kws kho mob kuj paub qhov txawv ntawm idiopathic variant ntawm cov txheej txheem pathological. nephrotic syndrome txawv ntawm lwm cov kab mob raum li cas?
Pathogenesis
Ntawm cov ntsiab lus ntawm pathogenesis, qhov tsim nyog tshaj plaws thiab nthuav dav yog txoj kev xav ntawm kev tiv thaiv kab mob. Hauv nws qhov kev pom zoo yog qhov zaus ntawm qhov tshwm sim ntawm tus mob no hauv cov kab mob autoimmune thiab kev tsis haum tshuaj thiab cov lus teb zoo ntawm lub cev rau kev tiv thaiv kab mob. Nyob rau hauv cov ntaub ntawv no, lub cev tsis muaj zog circulating complexes tsim nyob rau hauv cov ntshav yog tshwm sim los ntawm kev sib cuam tshuam ntawm cov tshuaj tiv thaiv thiab sab hauv (DNA, cryoglobulins, denatured nucleoproteins, proteins) los yog sab nraud (viral, kab mob, zaub mov, tshuaj) antigens. Cov no yog cov qauv tseem ceeb rau nephrotic syndrome.
Qee zaum, cov tshuaj tiv thaiv tau tsim ncaj qha rau hauv qab daus daim nyias nyias ntawm glomeruli ntawm ob lub raum. Lub deposition ntawm lub cev tiv thaiv complexes nyob rau hauv cov ntaub so ntswg ntawm lub raum provokes ib tug inflammatory txheej txheem, cuam tshuam.microcirculation nyob rau hauv glomerular capillaries, txhawb kev loj hlob ntawm ntau dhau intravascular coagulation. Kev ua txhaum ntawm cov permeability ntawm glomerular lim nyob rau hauv nephrotic syndrome ua rau impaired protein absorption thiab nws nkag mus rau hauv cov zis (proteinuria).
Muab qhov poob ntawm cov protein ntau hauv cov ntshav, hypoalbuminemia, hypoproteinemia thiab hyperlipidemia (nce qib ntawm triglycerides, cov roj cholesterol thiab phospholipids), zoo sib xws nrog cov teeb meem ntawm cov protein metabolism, txhim kho. Kev loj hlob ntawm edema yog vim hypoalbuminemia, hypovolemia, txo qis hauv osmotic siab, tsis zoo ntawm lub raum ntshav khiav, pronounced zus tau tej cov renin thiab aldosterone, thiab sodium reabsorption.
Lub raum hauv nephrotic syndrome yog macroscopically loj, tiaj tus thiab du nto. Nyob rau hauv seem, cortical txheej muaj ib tug daj ntseg grey tint, thiab medulla yog reddish. Kev kuaj xyuas microscopic ntawm daim duab ntawm lub raum tso cai rau koj pom cov kev hloov pauv uas tsis yog tsuas yog cov kab mob nephrotic xwb, tab sis kuj tseem muaj cov kab mob hauv qab (glomerulonephritis, amyloidosis, tuberculosis, collagenoses). Histologically, nephrotic syndrome yog tus cwj pwm los ntawm kev ua txhaum ntawm cov qauv hauv qab daus ntawm capillaries thiab podocytes (cov cell ntawm glomerular capsule).
Symptoms
Ntau tus neeg mob tseem tsis tau paub tias qhov no yog nephrotic syndrome. Cov tsos mob tseem ceeb ntawm tus kab mob, raws li txoj cai, yog tib yam, txawm tias muaj qhov sib txawv ntawm qhov ua rau ua rau nws. Cov tsos mob tseem ceeb yog proteinuria, uas nce mus txog 3.5-5 g / hnub. Kwv yees li 90% ntawm cov protein tawm los ntawmtso zis, tsim albumins. Kev poob qis ntawm cov protein ntau ua rau txo qis ntawm tag nrho cov ntsiab lus ntawm whey protein mus rau 60-40 g / l lossis ntau dua. Kev tuav cov kua dej yog tshwm sim los ntawm ascites, peripheral edema, anasarca (generalized o ntawm cov ntaub so ntswg subcutaneous), hydropericardium, hydrothorax. Cov tsos mob ntawm nephrotic syndrome yog qhov tsis kaj siab heev.
Kev nce qib ntawm cov txheej txheem pathological no suav nrog kev qaug zog hnyav, nqhis dej, qhov ncauj qhuav, tsis qab los noj mov, mob taub hau, hnyav hauv qab nraub qaum, tsam plab, ntuav, raws plab. Lwm cov yam ntxwv tshwj xeeb yog polyguria, uas niaj hnub diuresis tsawg dua 1 liter. Nws kuj tseem tuaj yeem tshwm sim ntawm qhov tshwm sim ntawm paresthesia, myalgia, convulsions. Kev loj hlob ntawm hydropericardium thiab hydrothorax thiab provokes ua pa luv ntawm so thiab thaum lub sijhawm txav mus los. Peripheral edema txo qis tus neeg mob lub cev muaj zog ua haujlwm. Nws muaj inactivity, pallor, nce dryness thiab tev ntawm daim tawv nqaij, nkig rau tes thiab plaub hau.
Kab mob nephrotic syndrome tshwm sim sai lossis maj mam, thiab nrog ntau lossis tsawg dua cov tsos mob, uas feem ntau yog nyob ntawm qhov xwm txheej ntawm cov kab mob hauv qab. Raws li kev kho mob chav kawm, ob qhov sib txawv ntawm tus kab mob yuav tsum tau paub qhov txawv - sib xyaw thiab ntshiab. Hauv thawj kis, nws tuaj yeem siv daim ntawv nephrotic-hypertonic lossis nephrotic-hematuric, thiab hauv qhov thib ob, tus mob tshwm sim tsis muaj ntshav siab thiab hematuria.
Nyob hauv hom
Congenital nephrotic syndrome yogloj pathology pom nyob rau hauv cov me nyuam. Nws yog tus cwj pwm los ntawm daim ntawv dav dav ntawm edema (tshwj xeeb thoob plaws lub cev), hyperlipidemia, thiab proteinuria. Nyob rau hauv lub plawv ntawm txoj kev loj hlob ntawm tus kab mob no yog lub pathology ntawm lub raum ntawm ib tug hereditary xwm.
Raws li qhov ua rau ua rau mob nephrotic syndrome, ntau yam ntawm nws qhov txawv, thiab ntau txoj kev kho mob muaj feem xyuam rau txhua daim ntawv. Hom kab mob no yog:
- Secondary Syndrome, uas yog qhov tshwm sim ntawm ntau yam kab mob hauv cov menyuam yaus. Piv txwv li, lupus erythematosus, ntshav qab zib mellitus, kev mob ntawm cov hlab plawv, mob qog noj ntshav, kab mob siab ua haujlwm. Kev kwv yees, cov kab mob thiab kev xaiv ntawm txoj kev kho rau daim ntawv no yog nyob ntawm qhov hnyav ntawm cov txheej txheem pathological thiab qhov mob hnyav ntawm cov tsos mob.
- Hereditary Syndrome. Daim ntawv no sai sai manifests nws tus kheej nyob rau hauv cov me nyuam tom qab lawv yug los. Qee zaum, qhov kev kuaj mob no tau txiav txim siab txawm tias thaum lub sij hawm kev loj hlob ntawm tus me nyuam hauv plab. Txawm li cas los xij, muaj cov xwm txheej thaum nephrotic syndrome tshwm sim nws tus kheej twb muaj hnub nyoog laus dua (piv txwv li, thaum lub sijhawm kawm ntawv). Nyob rau hauv txhua rooj plaub, xws li ib tug pathology yog heev nyuaj rau kho. Cov neeg mob feem ntau ua rau lub raum tsis ua haujlwm.
- Idiopathic Syndrome. Nws raug kuaj pom nyob rau hauv rooj plaub thaum nws tsis tuaj yeem txiav txim siab qhov tseeb ntawm tus kab mob.
- Tubulointerstitial syndrome. Nyob rau hauv daim ntawv no ntawm nephrotic syndrome, ob lub raum raug cuam tshuam nyob rau hauv xws li ib txoj kev uas lawv theem ntawm kev ua hauj lwm yog txo. Muaj ntse thiabhom mob ntev. Thawj yog feem ntau provoked los ntawm kev noj tshuaj los yog tsis haum rau lawv. Tsis tas li ntawd, tus kab mob kis tau yog ib qho ua rau. Hom kab mob no tshwm sim, raws li txoj cai, tawm tsam keeb kwm ntawm lwm yam kab mob.
Cov tsos mob ntawm daim ntawv interstitial ntawm tus mob hauv cov menyuam yaus, xws li lwm hom kab mob no, feem ntau pom pom tam sim ntawd. Ua tib zoo saib xyuas cov xwm txheej hauv qab no:
- Puffiness ntawm lub cev nce nrawm. Nws thawj zaug tshwm rau ntawm daim tawv muag, ces mus rau lub plab, ob txhais ceg, puab tsaig. Tom qab ntawd, ascites tsim.
- Kev faib dej hauv lub cev feem ntau nyob ntawm qhov chaw ntawm tus menyuam lub cev. Nws kuj cuam tshuam puffiness. Piv txwv li, yog tias tus menyuam tau sawv ib ntus, nws ob txhais ceg yuav o.
- Cov zis tso tawm maj mam txo qis. Qhov no cuam tshuam rau kev kuaj sim vim nws nce qib protein ntau hauv cov zis.
Thawj zaug, tus menyuam muaj ntshav siab. Nws ua qaug zog, chim siab, mob taub hau thiab lwm yam tsos mob ntawm tus mob no. Yog tias qhov mob ntawm kev noj qab haus huv no tsis quav ntsej rau ntev, tus menyuam yuav mob raum tsis ua haujlwm.
Kab mob sib kis yog qhov txaus ntshai heev rau cov menyuam yaus uas muaj tus mob nephrotic. Pneumococcus lossis streptococcus tuaj yeem ua rau hauv lub cev, yog li cov kab mob loj tuaj yeem tshwm sim los ntawm qhov tshwm sim. Nws tuaj yeem yog bronchitis, erysipelas, thiab peritonitis. Nrhiav seb yuav ua li cas glomerulonephritis thiabnephrotic syndrome. Puas yog tib yam?
YGlomerulonephritis ua rau muaj kab mob
Glomerulonephritis yog tus cwj pwm los ntawm kev tiv thaiv kab mob ntawm lub raum. Feem ntau, qhov pib ntawm tus kab mob no yog vim muaj zog tiv thaiv kab mob rau cov kab mob antigens. Tsis tas li ntawd, ib daim ntawv autoimmune ntawm pathology paub, thaum lub raum kev puas tsuaj tshwm sim los ntawm kev puas tsuaj ntawm autoantibodies (antibodies rau ib tus kheej hlwb).
Tus kab mob thib ob ntawm cov kab mob thib ob ntawm ob lub raum hauv cov menyuam yaus tom qab kis kab mob ntawm cov zis. Raws li kev txheeb cais, pathology yog feem ntau ua rau muaj kev tsis taus thaum ntxov ntawm cov neeg mob vim kev loj hlob ntawm lub raum tsis ua haujlwm. Kev loj hlob ntawm tus mob glomerulonephritis thiab nephrotic syndrome tau pom nyob rau txhua lub hnub nyoog, tab sis feem ntau tus kab mob no tshwm sim rau cov neeg hnub nyoog qis dua 40.
Qhov ua rau feem ntau yog kab mob streptococcal mob ntev lossis mob ntsws (pneumonia, tonsillitis, tonsillitis, streptoderma, scarlet fever). Tus kab mob tuaj yeem tshwm sim los ntawm tus kab mob pox, qhua pias lossis SARS. Qhov kev pheej hmoo ntawm kev tsim cov kab mob pathological nce ntxiv nrog rau lub sijhawm ntev rau qhov txias txias nrog cov av noo siab (muaj lub npe "trench" nephritis), vim tias kev sib xyaw ua ke ntawm cov khoom siv sab nraud hloov pauv cov tshuaj tiv thaiv kab mob thiab ua rau cov ntshav tsis txaus rau lub raum.
ntsuas ntsuas
Cov ntsiab lus tseem ceeb rau kev kuaj mob nephrotic syndrome yog cov ntaub ntawv kho mob thiab chaw kuaj mob. Lub hom phiaj kev tshuaj xyuas pab txheeb xyuasdaj ntseg ("pearl"), qhuav thiab txias rau qhov kov ntawm daim tawv nqaij, nce qhov loj ntawm lub plab, tus nplaig plaub, hepatomegaly, o. Nrog hydropericardium, muaj kev nthuav dav ntawm thaj tsam ntawm lub plawv, muffled tones. Nrog rau txoj kev loj hlob ntawm hydrothorax - shortening ntawm percussion suab, congestive nplua bubbling rales, tsis muaj zog ua pa. ECG qhia cov cim qhia ntawm myocardial dystrophy, bradycardia tau kaw.
Kev kho mob nephrotic syndrome
Kev kho ntawm cov kab mob no tau ua hauv tsev kho mob raws li kev saib xyuas ntawm tus kws kho mob nephrologist. Cov kev ntsuas uas tsis yog nyob ntawm qhov tshwm sim ntawm keeb kwm ntawm tus mob nephrotic yog kev teem caij noj zaub mov tsis muaj ntsev nrog cov kua dej tsawg, cov tshuaj kho cov tsos mob (poov tshuaj npaj, diuretics, tshuaj tua kab mob, tshuaj mob plawv, vitamins, heparin, tshuaj tua kab mob), pw so, infusion ntawm albumin, rheopolyglucin.
Kev kho mob nephrotic cuam tshuam dab tsi ntxiv?
Thaum lub hauv paus ntawm tus kab mob tsis meej, nyob rau hauv ib qho mob tshwm sim los ntawm autoimmune los yog lub raum toxic puas, steroid kev kho mob nrog methylprednisolone los yog prednisolone (qhov ncauj los yog intravenously los ntawm pulse therapy) yog qhia rau cov neeg mob. Immunosuppressive steroid kev kho mob pab txhawm rau tsim cov tshuaj tiv thaiv kab mob, CEC, normalizes glomerular filtration thiab raum ntshav khiav. Ib qho txiaj ntsig zoo ntawm kev kho rau cov tshuaj hormone-resistant variant ntawm tus kab mob tuaj yeem ua tiav los ntawm kev kho cytostatic nrog chlorambucil thiab cyclophosphamide, uas tau ua tiav hauv cov kev kawm mem tes. Thaum lub sij hawm kev zam tximKev kho mob ntawm climatic resorts yog pom zoo. Tag nrho cov lus pom zoo rau nephrotic syndrome yuav tsum tau ua raws li nruj me ntsis.
Kev noj haus
Nrog rau txoj kev loj hlob ntawm cov kab mob no, tus neeg mob yuav tsum ua raws li kev noj zaub mov tshwj xeeb. Nws raug sau tseg rau cov neeg mob uas tau hais tawm qhov o, nrog lub peev xwm ntawm lub raum lim. Kev noj zaub mov noj kuj tseem nyob ntawm qib protein ntau hauv cov zis.
Thaum kuaj pom muaj tus mob nephrotic, kev noj haus qhia tau tias ua raws li cov lus pom zoo hauv qab no:
- noj 5 mus rau 6 zaug hauv ib hnub ua ntu me;
- tsis ntau tshaj 3,000 calories;
- txwv tsis pub noj ntsim thiab rog;
- tsis txhob haus ntau tshaj 4 g ntsev ib hnub;
- haus yam tsawg 1 litre dej ib hnub twg.
Nws tseem pom zoo kom noj ntau cov txiv hmab txiv ntoo thiab zaub, nrog rau cov nplej zom, cereals, compotes, muaj roj tsawg ntau hom ntses thiab nqaij, bran bread.
Cov chav kawm thiab kev kwv yees muaj feem cuam tshuam rau qhov xwm txheej thiab ua rau kev loj hlob ntawm cov kab mob hauv qab. Feem ntau, kev tawm tsam ntawm etiological yam, kev kho kom raug thiab raws sijhawm tuaj yeem rov ua haujlwm rau lub raum thiab ua tiav kev tshem tawm ruaj khov. Nrog rau qhov tsis tau daws qhov teeb meem, nephrotic syndrome feem ntau siv sijhawm rov qab los lossis tsis tu ncua nrog kev hloov mus rau lub raum tsis ua haujlwm ntev.
Kev tiv thaiv tus kab mob no suav nrog thaum ntxov thiabTxhim kho kev kho mob ntawm extrarenal lossis lub raum pathology, uas tej zaum yuav nyuaj los ntawm qhov tshwm sim ntawm nephrotic syndrome, tswj thiab ceev faj siv cov tshuaj uas muaj kev tsis haum tshuaj thiab nephrotoxic. Txawm li cas los xij, yog tias cov tsos mob ntawm tus kab mob no tshwm sim, kev mus ntsib kws kho mob raws sijhawm.
Peb tau txiav txim siab tias qhov no yog nephrotic syndrome.
