Ormond's disease yog ib qho mob aseptic mob ntawm cov kab mob sib txuas thiab adipose ntawm qhov chaw retroperitoneal, uas ua rau compression ntawm tubular anatomical qauv nyob rau ntawd (lymphatic thiab cov hlab ntsha, ureters).
Retroperitoneal fibrosis (lwm lub npe rau tus kab mob) yog ib hom kab mob idiopathic fibrosis, uas kuj suav nrog mediastinal fibrosis, Riedel's struma, sclerosing cholangitis, Peyronie's disease thiab lwm yam. Tag nrho cov saum toj no yog qhov tshwm sim hauv zos ntawm tib tus kab mob.
Nyob zoo
Ormond's disease yog, hmoov zoo, tsis tshua muaj: tsuas yog ib kis ntawm ob puas txhiab tus neeg. Raws li txoj cai, nws raug kuaj pom hauv cov txiv neej los ntawm peb caug mus rau rau caum xyoo. Cov poj niam uas muaj hnub nyoog tib yam mob ob zaug ntau zaus. Kev tuag tsawg tsawg, thiab feem ntau ntawm cov xwm txheej tau piav qhia, nws tshwm sim los ntawm cov teeb meem, thiab tsis yog los ntawm tus kab mob nws tus kheej.
Cov ureters, uas tau them zoo nrog cov roj subcutaneous, feem ntau cuam tshuam los ntawm compression. Lawv pom lawv tus kheej, zoo li nws tau, hauv qhov nqaim ntom ntomrooj plaub thiab, vim nws qhov nruj, tsis ua tiav nws txoj haujlwm.
Kev faib tawm
Cov kws kho mob sib txawv ntawm thawj thiab theem nrab Ormond tus kab mob. Thawj, lossis idiopathic fibrosis, tshwm sim ntawm nws tus kheej, vim tsis paub. Cov kws tshawb fawb muaj ntau yam lus piav qhia rau qhov no:
- tawg ntawm cov noob lub luag haujlwm rau cov qauv ntawm cov ntaub so ntswg;
- kev tshwm sim tshwj xeeb ntawm autoimmune aggression;
- kev hloov pauv.
Secondary retroperitoneal fibrosis yog txuam nrog kev hloov pauv ntawm cov ntaub so ntswg vim muaj kab mob mus ntev dhau los. Qhov no tej zaum yuav kis tau tus kab mob mus ntev (los yog nqa cov kab mob), kab mob sib txuas, thiab lwm yam.
Yog vim li cas
Raws li tau hais dhau los, qhov ua rau Ormond tus mob tsis paub meej. Muaj ntau ntau txoj kev xav los piav qhia txog cov kev hloov pauv uas tshwm sim hauv lub cev.
- mob. Raws li qhov tshwm sim ntawm qhov ntev ntawm cov ntaub so ntswg edema, impregnation nrog fibrin thiab lwm yam mob theem proteins, lawv ua nruj thiab tsis ua haujlwm.
- Immune. Vim muaj kev ua haujlwm tsis zoo ntawm cov txheej txheem tiv thaiv, lub cev pib tsim cov tshuaj tiv thaiv tawm tsam nws cov ntaub so ntswg, ua rau lawv raug mob thiab, teb rau kev puas tsuaj, hloov cov chaw no nrog fibrin.
- Gennaya. Kev tawg nyob rau hauv cheeb tsam ntawm cov noob lub luag haujlwm rau cov qauv ntawm cov ntaub so ntswg. Manifested nyob rau hauv ib tug hloov nyob rau hauv cov qauv ntawm cov ntaub so ntswg retroperitoneal.
Ntau tus kws tshawb fawb qhia tias retroperitoneal fibrosis tuaj yeem raug ntaus nqi los ntawm ntau qhov tseebcollagenosis. Muaj qee yam uas ua rau muaj qhov tshwm sim ntawm tus kab mob. Cov no muaj xws li mob qog noj ntshav, kab mob siab, mob pancreatitis, sclerotic hloov pauv hauv cov ntaub so ntswg adipose hauv cov kab mob ntawm lub plab zom mov thiab cov poj niam hauv nruab nrog cev, tuberculous puas rau cov pob txha ntawm tus txha caj qaum, hematomas loj heev, vasculitis. Tsis tas li ntawd, cov chav kawm kws khomob thiab cov tshuaj uas txwv tsis pub mob migraine tuaj yeem pab tsim cov fibrosis. Tab sis feem ntau, nws tsis tuaj yeem txiav txim siab qhov tshwm sim.
Pathogenesis
Feem ntau ntawm cov kab mob retroperitoneal fibrosis (Ormond's disease) pib nrog cov tsos ntawm cov chaw compacted ntawm qib plaub lossis thib tsib lumbar vertebra. Cov hlab ntsha iliac thiab ureters nyob ntawd.
Sijhawm dhau mus, thaj chaw cuam tshuam nthuav dav thiab, ntes ntau thiab ntau cov fiber ntau, nqis mus rau lub cape ntawm lub sacrum, thiab tseem mus rau sab mus rau lub qhov rooj ntawm lub raum. Hauv yuav luag ib nrab ntawm cov xwm txheej, cov txheej txheem no yog ob txoj kev. Rough connective cov ntaub so ntswg ua piv rau qhov ntom rau ntoo. Nws kuj tshwm sim nyob ib ncig ntawm lub aorta, retroperitoneal lymph nodes, hlab ntsha thiab cov hlab ntsha. Cov qauv anatomical no yog compressed thiab cog lus, lawv patency deteriorates lub sij hawm.
Impaired patency ntawm ureters ua rau stagnation ntawm cov kua hauv lub raum, hydronephrosis, mob o thiab, vim li ntawd, lub raum tsis ua haujlwm. Muaj tsawg zaus, qhov no yog nrog los ntawm plab hnyuv los yog kev cuam tshuam ntawm cov hlab ntsha loj.
Symptoms
Tsis muaj cov cim qhia uas yuav ua rau tus kab mob Ormond tsis meej. Nws tag nrho yog nyob ntawm theem ntawm tus txheej txheem, nws qhov kev nthuav dav, kev ua haujlwm thiab cov yam ntxwv tshwj xeeb ntawm tib neeg lub cev. Lub sijhawm ntawm cov kab mob latent tuaj yeem sib txawv ntawm ob lub hlis mus rau kaum ib xyoos.
Txhua cov tsos mob ntawm Ormond tus kab mob tuaj yeem muab faib ua peb lub sijhawm, raws li lub sijhawm ntawm lawv qhov tsos:
- pib mob;
- nce fibrous fibers;
- "tightening" ntawm fibrin thiab compression.
Ib tus neeg mob tuaj rau tus kws kho mob hauv zos nrog kev tsis txaus siab ntawm kev npub, mob ib ce tsis tu ncua ntawm nraub qaum lossis sab. Kev hnov mob tsis zoo tuaj yeem kis mus rau hauv qab plab, puab tais, qhov chaw mos thiab pem hauv ntej ntawm tus ncej puab. Qee lub sij hawm qhov mob tuaj yeem ua rau ib leeg, tab sis dhau sijhawm nws tseem tshwm sim ntawm sab nraud.
Qhov tseem ceeb ntawm tus kab mob yog qhov maj mam tshwm sim ntawm cov tsos mob thiab qhov nce hauv lawv qhov hnyav. Hauv cov theem tom qab, cov neeg mob yws yws ntawm qhov txo qis ntawm cov zis, qhov pom ntawm qhov mob hnyav hauv lub nraub qaum sab nraub qaum, cov kab mob urinary tsis tu ncua uas ua rau lub raum tsis ua haujlwm ntev.
Diagnosis
Ormond tus kab mob yog tus yam ntxwv los ntawm ntau lub chaw kuaj thiab cov cuab yeej cuab tam uas pab kom paub qhov txawv ntawm lwm cov kab mob ntawm pawg no.
Nyob rau hauv kev kuaj ntshav, koj tuaj yeem pom qhov nrawm ntawm lub sijhawm ntawm sedimentation ntawm cov qe ntshav liab, tshuaj xyuas biochemicalqhia tias muaj ntau ntxiv ntawm alpha-globulins thiab C-reactive protein, nce cov ntsiab lus ntawm urea thiab creatinine (cov cim qhia ntawm CRF).
Los ntawm cov kev tshawb fawb, X-rays raug coj mus saib cov contours ntawm ob lub raum thiab cov leeg nyob ib ncig ntawm lawv. Tom qab ntawd tus neeg sawv cev sib txawv yog txhaj rau hauv cov ntshav thiab pom tias nws tau lim sai npaum li cas. Hauv qhov no, koj tuaj yeem pom cov contours ntawm lub khob thiab lub plab ntawm lub raum, cov duab, qhov chaw ntawm cov ureters thiab lawv qhov sib txawv ntawm lub hauv paus dav hlau ntawm lub cev. Tsis tas li ntawd, radiolabeled scintigraphy tuaj yeem ua tau los ntsuas qhov ua haujlwm ntawm lub tso zis.
Tsis txhob hnov qab txog cov txheej txheem xws li ultrasound. Nws pom tsis tau tsuas yog cov qauv hollow, tab sis kuj cov ntshav ntws hauv cov hlab ntsha tseem ceeb ntawm qhov chaw retroperitoneal. Qee lub sij hawm, raws li ib txoj hauv kev ntxiv, CT kev kuaj mob ntawm retroperitoneal fibrosis yog siv los qhia meej txog qhov chaw ntawm tus neeg lub cev ua ntej kev phais. Yog tias tsim nyog, koj tuaj yeem mus rau venokavagrafiya. Txoj kev no tso cai rau koj pom qhov tsis zoo vena cava, nws cov ceg thiab cov khoom lag luam, pom lawv qhov chaw thiab patency thoob plaws hauv lub nkoj.
Nws yog ib qho tseem ceeb tsis yog tsuas yog tsom rau kev kuaj mob, tab sis kuj tseem yog kev tshawb nrhiav qhov etiological yam uas ua rau muaj kev cuam tshuam rau kev loj hlob ntawm tus kab mob. Yog li ntawd, nws raug nquahu kom kuaj xyuas lub plab thiab plab rau cov kab mob zais zais.
Differential Diagnosis
Retroperitoneal fibrosis (Ormond's disease lossis RPF) yuav tsum txawv ntawm lwm tus los ntawm kev kho mob tshwm simpathological nqaim ntawm cov ureters (strictures thiab achalasia), thiab los ntawm ob sab hydronephrosis. Qhov tom kawg no tsis tshua muaj, vim tias kev cuam tshuam ntawm cov zis tawm ntawm ob sab ib zaug yuav ua rau lub raum tsis ua haujlwm thiab yuav tsum tau kho mob xwm txheej ceev, tsis zoo li qeeb qeeb fibrosis.
Txhawm rau kom paub meej qhov kev kuaj mob thiab sib txawv ntawm cov kab mob oncological, ntau qhov kev kuaj ntshav tuaj yeem ua raws li kev kuaj mob histological. Muaj tsawg zaus, nws tuaj yeem tsim qhov tseeb ntawm tus kab mob tsuas yog tom qab kuaj mob laparoscopy nrog kev sau cov khoom siv rau kev kuaj mob tom qab tuag.
Tsis tas li ntawd, qhov sib txawv tseem ceeb ntawm Ormond tus kab mob yog qhov clamping ntawm cov ureters tshwm sim nyob rau theem ntawm lawv cov kev sib tshuam nrog cov hlab ntsha iliac, thiab tsis nyob rau hauv ib qho chaw arbitrary. Qee lub sij hawm nws yuav tsum paub qhov txawv ntawm RPF los ntawm cov kab mob pancreatic uas nyob ib puag ncig, neoplasms ntawm lub plab zom mov, tuberculosis ntawm ob lub raum thiab ureters.
Ntxiv rau tag nrho cov saum toj no, tus neeg mob yuav tsum tau mus ntsib kws kho mob pulmonologist, phthisiatrician, oncologist thiab kws kho plawv.
Kev kho mob
Tus kws kho mob lub tswv yim dab tsi rau cov neeg mob kuaj pom tias muaj kab mob retroperitoneal fibrosis? Kev kho mob feem ntau nyob ntawm qhov ua rau tus kab mob. Yog tias qhov no yog qhov thib ob RPF, tom qab ntawd ntxiv rau kev kho cov tsos mob, lawv sim tshem tawm cov kab mob hauv qab - tshem tawm cov tshuaj, ntxuav lub ntsej muag ntawm cov kab mob ntev, lossis ua haujlwm ntawm cov qog. Thaum cov etiology ntawm tus kab mob tsis meej, cov tshuaj steroid raug muab.los yog tshuaj tiv thaiv kab mob uas inhibit qhov kev loj hlob ntawm cov ntaub so ntswg tshiab.
Kev saib xyuas tsis zoo yog hais txog kev tswj xyuas cov patency ntawm cov ureters hauv txhua lub tuam tsev thiab zam kev cuam tshuam.
Qhov twg kho Ormond tus kab mob? Nws tag nrho yog nyob ntawm theem ntawm tus kab mob. Thaum pib, koj tuaj yeem npaj cov tshuaj hauv tsev lossis hauv tsev kho mob sab nraud, vim tias tsis muaj kev hloov pauv morphological loj. Hauv cov mob hnyav heev, tus neeg mob yuav tsum tau nyob hauv tsev kho mob urological, qee zaum txawm nyob hauv kev saib xyuas hnyav.
Kev saib xyuas kev noj qab haus huv tsuas yog tsim nyog yog tias cov ureters txaus patent thiab lub raum tsis ua haujlwm tsis zoo. Txhawm rau qeeb thiab nres cov txheej txheem ntawm kev siv fibrosis:
- glucocorticosteroids ("Prednisolone");
- tsis yog-steroidal tshuaj tiv thaiv kab mob (Ibuprofen, Paracetamol, Celecoxib));
- immunosuppressants ("Azathioprine", "Metronidazole");
- absorbable npaj, enzymes (hyaluronidase, kua txiv aloe).
Cov txheej txheem hauv qab no suav tias yog qhov ua tau zoo tshaj plaws:
- 25 mg "Prednisolone" hauv thawj lub hlis, nrog kev txo qis hauv cov koob tshuaj hauv 3 lub hlis tom ntej;
- Esomeprazole 20mg ib hnub thaum pw;
- ib nrab xyoo kawm "Wobenzym" 15 ntsiav tshuaj ib hnub;
- ib hlis tom qab pib Prednisolone, ntxiv Celecoxib ntawm ib koob ntawm 100 mg.
Yog tias tsim nyog, ntxiv rau cov kab mob pathogenetic, cov tshuaj kho mob kuj tau sau tseg. Thaum noj tshuajtus mob ntawm cov neeg mob yog kev txhim kho. Hmoov tsis zoo, tus kab mob no yog tus cwj pwm los ntawm kev rov ua dua tshiab. Yog li ntawd, tom qab txiav tawm cov tshuaj hauv cov neeg mob feem ntau, cov tsos mob rov qab los thiab hnyav dua.
Kev kho mob phais tsuas yog muab rau thaum muaj teeb meem xws li:
- pronounced dilatation ntawm lub ureters;
- ntxhib deformity ntawm cov qauv retroperitoneal;
- mob ntshav siab cuam tshuam nrog lub raum tsis ua haujlwm, thiab lwm yam.
Cov kws phais ua ureteroplasty, txhim kho nephrostomy, rov kho qhov patency ntawm inferior vena cava thiab nws cov tributaries.
Khoom noj khoom haus
Retroperitoneal fibrosis yog ib yam kab mob uas yuav tsum tau noj zaub mov nruj. Cov neeg mob yuav tsum tsis suav nrog kib, ntsev, ntsim thiab haus luam yeeb los ntawm kev noj zaub mov. Nws yog ib qho tseem ceeb tshwj xeeb los saib xyuas koj cov zaub mov thaum ntxov ntawm txoj kev loj hlob ntawm tus kab mob, vim tias cov teeb meem yooj yim dua los tiv thaiv dua li kho tom qab. Tsis tas li ntawd, kev noj haus pab qeeb txoj kev loj hlob ntawm Ormond's syndrome.
Yog tias thawj zaug ib tus neeg mus rau hauv tsev kho mob, qhov nqaim ntawm lub ureter twb muaj lawm, ces nws raug nquahu kom haus dej kom ntau li ntau tau. Qhov no yog qhov tsim nyog tsis yog los tswj lub suab ntawm cov zis, tab sis kuj tseem yuav tshem tawm cov metabolites hauv cov ntshav vim qhov txo qis hauv lub raum ua haujlwm.
Lub neej niaj hnub ntawm tus neeg mob tuaj yeem cuam tshuam los ntawm kev teeb tsa lub nephrostomy kom tshem tawm cov zis los ntawm catheter. Kev lim dej yuav tsum tau saib xyuas tshwj xeeb los tiv thaiv maceration thiab o ntawm cov ntaub so ntswg ncig lub raj.
Kev nyuaj siab
J. Ormond tus kab mob, zoo li lwm yam kab mob hauv lub cev, muaj nws cov teeb meem. Qhov txaus ntshai tshaj plaws ntawm lawv yog kev tso tawm ntawm cov zis los yog anuria. Qhov no ua rau kom qaug cawv sai thiab txhim kho lub raum tsis ua haujlwm, raws li cov zis stagnates nyob rau hauv lub pelvicalyceal apparatus thiab ua rau lub raum puas hlwb.
Arterial hypertension yog qhov thib ob ntawm qhov tshwm sim. Nws tshwm sim vim qhov txo qis hauv txoj kab uas hla ntawm lub raum hlab ntsha thiab, vim li ntawd, cov ntshav ntws hauv nws. Qhov no ua rau kom them nyiaj nce ntxiv hauv renin concentration thiab nce siab hauv lub cev.
Qhov thib peb qhov teeb meem yog varicose leeg vim compression ntawm inferior vena cava thiab impaired outflow los ntawm qis extremities. Hauv cov mob hnyav heev, qhov mob tsis zoo yuav tshwm sim.
Yog tias plab hnyuv muaj feem cuam tshuam rau hauv cov txheej txheem ntawm cov nqaij mos fibrosis, plab hnyuv txhaws tshwm sim. Nws yog manifested los ntawm cov quav tuav, tsam plab thiab intoxication.
Kev Tiv Thaiv
Raws li nrog ntau lwm yam kab mob, txoj hauv kev tseem ceeb los tiv thaiv qhov tshwm sim ntawm retroperitoneal fibrosis yog tshem tawm foci ntawm tus kab mob ntev, muaj peev xwm thiab kho raws sijhawm ntawm kab mob siab, kab mob lossis kab mob vasculitis.
Ntxiv rau, cov kws kho mob pom zoo kom koj ua tib zoo saib xyuas koj txoj kev noj qab haus huv, tshwj xeeb tshaj yog tias twb tau tsim muaj cov mob collagenopathy hauv tsev neeg. Qhov no yuav tso cai rau txheeb xyuas tus kab mob thaum ntxov thiab pib kho raws sijhawm ua ntej kev txhim kho ntawm cov teeb meem, uas yav tom ntej yuav txhim kho lub neej zoo thiab ua kom nws ntev.
Huab cua
Kev txhim kho ntawm Ormond's kab mob hauv txhua tus neeg mob nyob ntawm theem uas kuaj pom pathology, thiab ntawm tus nqi ntawm kev loj hlob ntawm fibrosis. Nws tseem yog ib qho tseem ceeb uas yuav tsum tau coj mus rau hauv tus account lub xeev ntawm cov zis, muaj cov teeb meem thiab kev yug me nyuam. Feem ntau, kev kho kom zoo yuav ua rau muaj txiaj ntsig zoo ib ntus. Qhov kev vam meej tshaj plaws tau lees paub tias yog txoj kev kho mob phais, uas muaj nyob rau hauv lub plasticity ntawm lub ureter thiab nws lub zog. Tom qab kev phais, nws raug nquahu kom noj cov tshuaj steroid mus sij hawm ntev los txhim kho qhov kev cia siab ntawm kev ciaj sia thiab lub neej zoo. Kev rov tshwm sim tuaj yeem ua tau, tab sis lawv tau qeeb qeeb hauv lub sijhawm piv rau kev kho tshuaj ib leeg.
Qhov tseem ceeb ntawm kev tuag yog lub raum tsis ua haujlwm ntev. Yog li ntawd, qhov prognosis tseem unfavorable. Qee zaum, thaum kuaj pom tus kab mob nyob rau theem kawg, qhov tshwm sim ntawm kev tuag yog ntau tshaj rau caum feem pua. Yog li ntawd, qhov sai dua cov kab mob tau pom, qhov kev kho mob yuav ua tiav ntau dua.
