Uremic Leontiasis Ossea: Cov duab tshwj xeeb tso cai rau kev sib txawv ntawm lwm yam kev kho mob ua rau Leontiasis Ossea

Yog xav paub ntxiv. tiv taujtina.xiang@wecistanche.com

A B S T R A C T

Kev hloov ntawm pob txha yog ib qho teeb meem tshwm sim hauv cov neeg mob uas muaj kab mob raum ntev thiab yog ib txwm sau npe ualub raum osteodystrophy. Uremic leontiasis ossea yog ib hom tsis tshua muaj thiab hnyav ntawm lub raum osteodystrophy nrog cov yam ntxwv ntawm cov pob txha craniofacial. Kev kos duab, tshwj xeeb yog suav tomography, muaj txiaj ntsig zoo rau kev kuaj mob thiab kev tswj xyuas cov xwm txheej tsis tshua muaj. Uremic leontiasis ossea muaj cov duab tshwj xeeb nrog kev loj hlob ntawm lub puab tsaig thiab cov yam ntxwv sab hauv serpiginous tunneling. Kev lees paub ntawm nws cov duab hluav taws xob thiab kev tswj xyuas sai yog qhov tseem ceeb kom tsis txhob muaj kev puas tsuaj rau kev zoo nkauj thiab kev ua haujlwm tsis zoo.

Nyem qhov no kom paub txog cov txiaj ntsig herba cistanches

Taw qhia

Lo lus leontiasis tau siv los piav qhia txog lub ntsej muag zoo li tsov ntxhuav nyob rau hauv mob ruas vim nws qhov kev hloov pauv ntawm daim tawv nqaij. Tom qab xyoo 1869, lo lus ossea tau muab ntxiv rau leontiasis los ntawm Virchow los piav txog inflammatory hyperostosis ntawm lub ntsej muag pob txha. Tseeb tiag, leontiasis ossea yog ib lo lus piav qhia dav dav es tsis yog kab mob hauv nws tus kheej, uas feem ntau siv rau diffuse craniofacial overgrowth uas zoo li tsov ntxhuav lub ntsej muag [3]. Xws li deformity tau sau tseg nyob rau hauv ntau yam mob xws li Paget's kab mob, fibrous dysplasia, thiab lub raum osteodystrophy (ROD).In 1953, Cohen li al. thawj zaug piav qhia txog lub ntsej muag tshwm sim hauv theem nrab hyperparathyroidism (SHPT) thiabmob raum mob(CKD) raws li uremic leontiasis ossea (ULO).

Kev hloov ntawm pob txha yog ib qho teeb meem tshwm sim hauv cov neeg mob CKD thiab ib txwm sau npe ua ROD. Txawm li cas los xij, cov neeg mob cov pab pawg no kuj tau pom zoo rau lwm qhov kev tshwm sim ntawm lub cev pob txha uas lub raum Kab Mob Txhim Kho Kev Txhim Kho Ntiaj Teb (KDIGO) Foundation tau pom zoo rau cov lus dav dav, mob raum kab mob-mineral thiab pob txha tsis zoo (CKD-MBD). KDIGO kuj tau pom zoo tias lo lus ROD tsuas yog siv los piav qhia txog kev hloov pauv ntawm pob txha morphology cuam tshuam nrog CKD. Li no, CKD-MBD yog ib qho kev tsis sib haum xeeb uas muaj kev hloov pauv biochemical, hloov pob txha, cov nqaij mos, thiab vascular calcifications [8. Cov kev hloov pauv biochemical yooj yim hauv CKD-MBD yog hyperphosphatemia, hypocalcemia, thiab txo qis kev ua kom cov vitamin D uas nws thiaj li ua rau SHPT nrog nce cov tshuaj parathyroid hormone (PTH).

ULO yog hom tsis tshua muaj thiab hnyav ntawm ROD nrog cov yam ntxwv ntawm cov pob txha craniofacial. Ob peb kis ntawm ULO tau luam tawm hauv cov ntaub ntawv, uas tuaj yeem piav qhia los ntawm qhov muaj kev lim ntshav, thiab kev tswj hwm sai ntawm SHPT; raws li kev hloov pauv biochemical feem ntau tau kuaj pom ua ntej ua ntej kev txhim kho pob txha hloov. Tsis tas li ntawd, muaj cov spectrum ntawm cov pob txha hloov pauv los ntawm SHPT thiab sau nrog cov lus sib txawv, uas qhov tseeb ntawm cov ntaub ntawv luam tawm tsis tuaj yeem kwv yees. Cov kws kho mob yuav tsum paub txog kev kuaj mob thiab kev tswj hwm ntawm qhov tsis tshua muaj mob no, vim tias nws yuav ua rau muaj kev puas tsuaj rau kev zoo nkauj thiab kev ua haujlwm tsis zoo. Daim ntawv tshaj tawm no yog txhawm rau tshawb nrhiav lub luag haujlwm ntawm kev thaij duab hauv ULO thiab nws cov duab tshwj xeeb uas tso cai rau kev sib txawv ntawm lwm yam kev kho mob ntawm leontiasis ossea. Tsis tas li ntawd, cov teeb meem xav tau thiab cov tswv yim kho mob kuj tseem yuav tham txog.

Case report

Ib tug txiv neej muaj hnub nyoog 28- xyoo tau nthuav tawm nrog qhov mob tsis zoo ntawm lub ntsej muag o nyob rau xya lub hlis dhau los. Hloov hauv nws lub suab kuj tau sau tseg. Nws keeb kwm kho mob yav dhau los suav nrog theem kawgmob raum(ESRD) ntawm undetermined etiology rau cuaj lub xyoos dhau los, tam sim no nyob rau hauv hemodialysis 3 zaug ib lub lim tiam, hnyav un-controlled SHPT, thiab kub siab. Tus neeg mob tsis kam lees paub txog keeb kwm ntawm kev tsis pom kev, hnov ​​​​lus tsis zoo, ua tsis taus pa, lossis dysphagia.

Thaum tuaj txog, tus neeg mob ntshav siab tau nce siab (161/101 mmHg), thiab lwm yam cim tseem ceeb yog qhov qub. Ntawm kev kuaj lub cev, tus neeg mob tau raug sau tseg tias muaj lub cev tawv, lub cev craniofacial protrusion, feem ntau hais raws maxilla. Tsis muaj ntaub ntawv sau tseg. Labs tau txais cov nram qab no: 13.4 mmol/L urea (4.2-7.2), 920 umol/L creatinine (64-115), 6 mL/min/1.73m^2 e-GFR, 1.65 mmol/L phosphate (0.80-1.45), 1714.3 U/L alkaline phosphatase (50-116), 2.080 mmol/L calcium (2.{25}}.550), thiab 3012 ng /L PTH.

Kev suav tomography (CT) ntawm cov pob txha ntawm lub ntsej muag tau rov nug dua los txiav txim cov pob txha. Cov yeeb yaj kiab scout qhia txog kev loj hlob ntawm lub puab tsaig, tshwj xeeb tshaj yog cov maxilla, nrog ntau yam me me zoo-txhais lucencies hauv calvaria (Fig. 1). Cov duab axial, coronal, thiab sagittal CT qhia pom qhov loj ntawm lub puab tsaig nrog sab hauv serpiginous tunneling lossis channeling; pom raws li lwm cov nplhaib ntawm hypo- thiab hyperattenuating cuam tshuam nrog kev poob ntawm corticomedullary sib txawv (Fig. 2).

Daim duab sagittal CT qhia tau hais tias sclerosis ntawm vertebral kawg-plates (Fig. 2).

Keeb kwm ntawm ESRD, biochemical cov txiaj ntsig ntawm SHPT, thiab cov duab tshwj xeeb hauv cov pob txha craniofacial yog kuaj ntawm ULO. Tus neeg mob tam sim no tau tso npe rau hauv daim ntawv teev npe hloov chaw tos. SHPT ua tsis tau raws li kev tswj hwm los ntawm kev kho mob rau qhov kev sib tham txog kev phais endocrine tau thov kom muaj peev xwm parathyroidectomy.

Kev sib tham

Feem ntau, cov pob txha tshwm sim hauv ROD poob rau hauv peb lub ntsiab qauv: cov pob txha loj hauv SHPT, cov pob txha qis hauv rickets / osteomalacia, lossis sib xyaw [121. Txawm hais tias tsis zoo con-trolled SHPTcan tau sau tias yog ib qho tseem ceeb ntawm ULO, qhov tseeb pathogenesis tseem tsis paub [13]. Tsis tas li ntawd, qhov predilection ntawm ULO los koom nrog cov pob txha craniofacial tseem tsis paub meej [14]. Massicotte-Azamiouch et al. piav qhia txog kev hloov pauv ntawm morphological hauv ULO tsis txwv rau kev hloov pauv osseous tab sis suav nrog cov ntaub so ntswg hloov pauv uas thaum kawg muab cov tsov ntxhuav zoo li lub ntsej muag zoo li [15].In 2004, Sagliker et al.found 25 cov neeg mob nrog CKD, SHPT, mob loj craniofacial deformities, qog. cov ntaub so ntswg hauv qhov ncauj, cov ntiv tes hloov pauv, thiab teeb meem puas siab puas ntsws; thiab lawv tau sau nws tias Sagliker syndrome.

Txawm hais tias ULO tau kuaj pom hauv tsev kho mob, txawm li cas los xij, kev kuaj pom tso cai rau kev sib txawv ntawm lwm cov kab mob uas zoo sib xws rau kev loj hlob ntawm craniofacial overgrowth. Kev kos duab kuj tseem muaj txiaj ntsig ua ntej kev phais kho dua tshiab rau cov neeg mob uas muaj kev zoo nkauj lossis kev ua haujlwm tsis zoo. Tsis tas li ntawd, kev kos duab tuaj yeem siv los ua cov cuab yeej prognostic hauv ULO kom paub meej tias kev txhim kho hauv craniofacial deformities tom qab kev tswj hwm zoo [1]. Kev tshawb fawb hla ntu ntu, feem ntau CT, nrog lossis tsis muaj peb-dimensional (3D) rov tsim kho yog qhov kev xaiv ntawm kev xaiv. Sib nqus resonance imaging (MRI) tuaj yeem thov thaum CT kev tshawb pom tsis tiav. Lub tshuab hluav taws xob panoramic muaj txiaj ntsig zoo rau kev ntsuas ntawm lamina dura uas zoo li cuam tshuam los ntawm hyperparathyroidism. Raws li ib feem ntawm kev tswj hwm ntawm ULO, kev kos duab ua lub luag haujlwm tseem ceeb hauv qhov chaw ua ntej ntawm parathyroid adenoma. Ultrasound thiab 99mTc-sestamibi scintigraphy yog cov txheej txheem imaging ntawm kev xaiv rau cov xwm txheej zoo li no[18]. Contrast-enhanced CT thiab MRI tuaj yeem khaws cia rau kev ua tsis tiav parathyroidectomy lossis tsis sib haum xeeb ultrasound thiab kev tshawb pom scintigraphy [18]. Plaub-dimensional (4D) CT yog ib qho tseem ceeb txheej txheem uas qhia tau hais tias muaj kev kuaj mob siab rau parathyroid adenoma localization tshwj xeeb rau kev tshawb pom tsis pom ntawm ultrasound lossis scintigraphy.

Kev kuaj mob ntawm ULO ua rau muaj teeb meem hauv hluav taws xob thiab kab mob hauv qhov tshwj xeeb nrog tsis muaj keeb kwm ntawm ESRD thiab biochemical cov txiaj ntsig ntawm SHPT. Radiologically, cov yam ntxwv ntawm ULO yog complex raws li nyob rau hauv ROD uas muaj peev xwm sib tshooj ntawm rickets / osteomalacia thiab SHPT [20]. Txawm hais tias pathologically, ULO tuaj yeem txhais tau tias yog lwm yam ua rau kev loj hlob ntawm craniofacial xws li fibrous dysplasia [10]. Feem ntau, qhov kev tshawb pom ntawm osteomalacia ntawm kev kuaj pom tsis yog qhov tshwj xeeb thiab suav nrog txo cov pob txha ceev, poob ntawm cortical txhais, thiab coarsening ntawm tus qauv trabecular ntxiv rau Looser zones lossis pseudofractures; ib hom kev puas tsuaj tsis txaus [20,21].Ntawm qhov tod tes, hyperparathyroidism nthuav tawm nrog cov pob txha resorption raws li qhov tshwm sim ntau tshaj plaws. Cov pob txha resorption nyob rau hauv lub pob txha taub hau yog qhov zoo tshaj plaws piav raws li ib tug "ntsev thiab kua txob" tshwm sim los ntawm ntau yam lucencies nyob rau hauv lub keeb kwm yav dhau ntawm ib tug sclerotic av-iav tsos txuam nrog indistinct nyob rau hauv-ner thiab txheej rooj. Xws li cov qauv hauv av tuaj yeem ua yuam kev rau fibrous dysplasia, txawm li cas los xij, hauv ULO zoo nkaus li tau ploj mus ntawm kev sib txawv ntawm corticomedullary [10]. Subperiosteal resorption kuj tuaj yeem pom nyob rau hauv lamina dura, uas yog cov pob txha nyob ib puag ncig cov hniav. Osteitis fibrosis cystic yog lwm qhov tshwm sim ntawm hyperparathyroidism uas yog tshwm sim los ntawm kev ua kom cov osteoclasts thiab hloov cov pob txha pob txha los ntawm vascular thiab fibrous cov ntaub so ntswg. Brown qog yog ib hom mob hnyav ntawm osteitis fibrosa cystica uas tshwm sim raws li ib leeg lossis ntau qhov lytic lesions. Osteosclerosis tseem tuaj yeem pom tshwj xeeb thaum lub sijhawm kho nrog cov yam ntxwv nce pob txha ntom ntawm vertebral kawg-plates (rugger-jersey qaum). Qhov loj tshaj ntawm lub puab tsaig nrog rau sab hauv serpiginous tunneling los yog channeling thiab poob ntawm corticomedullary sib txawv yog qhov tshwj xeeb tshwm sim ntawm ULO. Qhov ua rau ntawm qhov zoo li no tseem tsis paub meej yam tsis muaj kev piav qhia histopathological [10]. Txawm li cas los xij, Wang et al. qhia txog serpiginous tunneling los yog channeling raws li ib tug zonal qauv nrog lwm rings ntawm hypo- thiab hyperattenuating raws li ib tug tshwm sim ntawm osteoclastic thiab osteoblastic kev ua si [23]. Lee thiab al.reported xws li ib tug zonal qauv yuav tsum muaj feem xyuam rau concentric txheej ntawm repetitive hlwb thiab trabeculae ntxiv rau cov ntshav cov khoom nyob ib ncig ntawm involuted ntom xim av qog.

Cov kev kuaj mob tseem ceeb ntawm ULO yog Paget's kab mob thiab fibrous dysplasia raws li kev tshawb fawb soj ntsuam ntawm craniofacial overgrowth. Txawm li cas los xij, qhov kev loj hlob ntawm cov xwm txheej zoo li no yog asymmetrical thiab multifocal raws li qhov sib txawv rau qhov sib txawv ntawm kev koom tes hauv ULO. Kev lees paub txog histopathological tsis tsim nyog li ULO, fibrous dysplasia, thiab Paget tus kab mob tuaj yeem muaj qhov kev tshawb pom zoo sib xws. Yog li, kev kuaj mob ntawm ULO hauv cov neeg mob uas muaj kev hloov pauv ntawm craniofacial nyob ntawm qhov kev tshawb pom ntawm ESRD, biochemical cov txiaj ntsig ntawm SHPT, thiab kev tshawb pom classical. ULO nthuav tawm kev kho mob nrog kev mob hnyav heev ntawm cov pob txha craniofacial tshwj xeeb hauv lub puab tsaig, nthuav dav ntawm nares, flattening ntawm qhov ntswg choj, thiab nce qhov sib txawv ntawm qhov sib txawv. Nyob rau sab ntawm nws cov teebmeem tshuaj pleev ib ce, kev koom tes ntawm cov pob txha craniofacial hauv ULO ua rau muaj kev cuam tshuam ntawm pob txha taub hau hauv paus foramina uas yuav ua rau muaj kev cuam tshuam ntawm cranial neuropathy. Sundaram et al. Cov ntaub ntawv ob tog compressive optic neuropathy tshwm sim los ntawm ULO nrog kev txhim kho spontaneous txhais tau tias pom cov tsos mob tom qab parathyroidectomy 9]. Tsis hnov ​​lus kuj tau tshaj tawm yav dhau los. Tsis tas li ntawd, ULO tuaj yeem ua rau lwm yam kev ua haujlwm tsis zoo xws li ua rau lub neej ua rau muaj kev cuam tshuam rau txoj hlab cua sab sauv, ua pa qhov ntswg, dysarthria, thiab dysphagia.

Muaj qee cov ntaub ntawv luam tawm ntawm cov tswv yim kho mob ntawm ULO uas tuaj yeem kho mob thiab phais. Feem ntau ntawm cov neeg mob yog resistant rau kev kho mob ib leeg [1]. Cov tswv yim phais mob txhawm rau kho qhov laj thawj tseem ceeb ntawm SHPT, ntxiv rau, txhawm rau sim ua kom rov zoo li qub thiab ua haujlwm. Lee et al. Cov ntaub ntawv kho mob me me los yog tsawg kawg stabilization ntawm lub ntsej muag deformities tom qab parathyroidectomy hauv lawv cov 5 cov neeg mob nrog ULO[13]. Yang et al. tau lees paub cov txiaj ntsig tau tshaj tawm ua ntej thaum lawv rov qab soj ntsuam 6 tus neeg mob nrog kev pab SHPT thiab txhim kho lub neej zoo tom qab tag nrho parathyroidectomy thiab autotransplantation [27]. Duan et al. Cov ntaub ntawv tseem ceeb hloov pauv ntawm lub cev pob txha ntawm cov duab tom qab parathyroidectomy thiab autotransplantation ntxiv rau kev kho mob[1. Txawm li cas los xij, ULO tej zaum yuav muaj qhov biochemical rov ua tau zoo (60 feem pua) hauv 1 xyoos tom qab kev phais [27]. Li no, lawv xaus lus, tias ULO tsis yog ib yam kab mob yooj yim nrog craniofacial malformations tab sis yog kab mob hnyav. Tsis tas li ntawd, qhov txawv txav ntawm PTH tom qab parathyroidectomy tuaj yeem piav qhia los ntawm cov ntaub so ntswg parathyroid residual uas tuaj yeem ua ectopic nyob rau hauv qhov chaw thiab xav tau tom qab kev ua haujlwm 99mTc-sestamibi scintigraphy txhawm rau txheeb xyuas qhov chaw raws li tau lees paub los ntawm cov ntaub ntawv ua ntej [24]. Kev ua tiav ntawm qhov kev cog lus zoo li no los ua kom cov qib PTH zoo li qub [24]. Raws li qhov tsis tshua muaj tshwm sim, tsis muaj cov tswv yim meej rau kev tsim kho maxillofacial txhawm rau txhim kho tus neeg mob lub neej zoo. Cov tswv yim zoo li no yuav tsum tau npaj raws sijhawm kom ntseeg tau tias feem pua ​​​​ntawm cov pob txha loj dua nyob rau hauv qhov txhab.

Xaus

ULO muaj cov duab tshwj xeeb nrog kev loj hlob ntawm lub puab tsaig thiab cov yam ntxwv sab hauv serpiginous tunneling. Kev lees paub ntawm nws cov duab hluav taws xob thiab kev tswj xyuas sai yog qhov tseem ceeb kom tsis txhob muaj kev puas tsuaj rau kev zoo nkauj thiab kev ua haujlwm tsis zoo. Kev soj ntsuam mus sij hawm ntev yuav tsum tau ua kom rov muaj dua tom qab kev tswj hwm zoo. Kev tshuaj xyuas biochemical thaum lub sij hawm hemodialysis yog qhov tseem ceeb vim tias qhov kuaj pom txawv txav tuaj yeem ua nrog cov kev hloov ntawm pob txha uas tuaj yeem siv los ua cov cuab yeej tiv thaiv.

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ROD, raum osteodystrophy; SHPT, theem nrab hyperparathyroidism; CKD, mob raum mob; ULO, uremic leontasis ossea; KDIGO, Kab mob raum-Txhim kho cov txiaj ntsig thoob ntiaj teb; CKD-MBD, mob raum kab mob-mineral, thiab mob pob txha: PTH. parathyroid hormone; ESRD, kab mob raum kawg; CT, xam tomography; 3D, peb-dimensional; MRI, Sib nqus resonance imaging; 4D, plaub-dimensional.