Mob plab hnyuv Pseudo-obstruction Los ntawm Pheochromocytoma: Cov Ntaub Ntawv Qhia Nrog Kev Tshawb Fawb Cov Ntawv

TSAB NTAWV

Taw qhia: Pheochromocytomas yog cov qog tsis tshua muaj ntawm cov qog adrenal. Txoj hnyuv pseudo-obstruction yog ib qho kev nthuav qhia tsawg heev ntawm kev ua haujlwm catecholamine-secreting qog. Peb nthuav tawm ib rooj plaub ntawm plab hnyuv pseudo-obstruction vim muaj kev ua haujlwm loj pheochromocytoma. Cov ntaub ntawv ceeb toom: Ib tug poj niam muaj hnub nyoog 29- xyoo muaj mob plab, mob, xeev siab, thiab ntuav nrog cem quav rau 3 lub lis piam. Nws mob siab thiab mob ntshav qab zib thiab tau noj ntau yam tshuaj. Nws tau qhia ntau zaus ntawm mob taub hau, palpitations, tawm hws hmo ntuj, thiab poob 17 kg ntawm 6 lub hlis. Nws muaj pallor, dyspnea, cim plab zom mov, thiab lub suab plob tsis so tswj. Nws cov ntshav siab siab ntawm 200/120 mmHg. Nws muaj tachycardia (mem tes 120 bpm) thiab tachypnea (35 teev tsaus ntuj). Cov qib metanephrine hauv cov ntshav tau nce siab, ntsuas 1203 pg / ml. Abdominal CT pom ib heterogeneous, hyper-vascular loj nyob ze ntawm tus ncej qaum ntawm lub raum sab laug, ntsuas 10.75 cm × 8.72 cm. Qhib sab laug adrenalectomy tau ua los ntawm txoj hauv kev anterior subcostal kom tshem tawm cov qog nrog cov qog adrenal sab laug. Kev kuaj mob histopathological tau zoo ib yam nrog pheochromocytoma. Kev Sib Tham: Qee tus kws sau ntawv tau sau txog qhov sib txheeb ntawm cov qog loj thiab cov haujlwm metabolic ntawm catecholamine-secreting qog nrog plab hnyuv pseudo-obstruction los ntawm tus tuag tes tuag taw ileus. Cov ntaub ntawv no sib raug nrog cov kev tshawb pom no, nrog rau cov qog loj ntawm 350 g thiab cov ntshav metanephrine qib ntawm 1203 pg / ml. Xaus: Txawm hais tias nws tsis tshua muaj heev, kev ua haujlwm pheochromocytoma tuaj yeem ua rau mob plab hnyuv lossis pseudo-obstruction.

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1. Taw qhia

Pheochromocytomas yog cov qog tsis tshua muaj ntawm cov qog adrenal uas tuaj yeem ua rau tsis sib xws lossis tsev neeg, nrog kev kwv yees txhua xyoo tshwm sim ntawm 0.8 rau 100,000 neeg / xyoo [1]. Txoj hnyuv pseudo-obstruction yog ib qho kev nthuav qhia tsawg heev ntawm kev ua haujlwm catecholamine-secreting qog [2]. Nws nthuav tawm nrog cov cim qhia thiab cov tsos mob ntawm lub plab zom mov me lossis loj yam tsis muaj kev cuam tshuam txog kev siv tshuab [2]. Lub triad classic ntawm kev nthuav qhia rau pheochromocytoma yog tawm hws, mob taub hau, thiab tachycardia. Kev ua haujlwm pheochromocytoma tuaj yeem muaj ntau yam tsos mob tshwm sim. Yuav tsum muaj qhov xav tsis thoob siab rau qhov kev kuaj mob kom raug [2]. Qee lub sij hawm kev tswj cov plab hnyuv pseudo-kev cuam tshuam rau cov neeg mob uas muaj pheochromocytoma tuaj yeem tsis meej thiab tsis ncaj, tab sis qee cov neeg mob teb tau zoo rau kev phais tshem tawm ntawm qhov loj thiab tuaj yeem rov ua tau lawv lub plab zom mov tom qab ntawd. Txawm li cas los xij, muaj lwm qhov xwm txheej uas xav tau kev phais decompression ntawm txoj hnyuv, lossis tsim kom muaj lub stoma, tshwj xeeb tshaj yog rau cov neeg mob metastases [3].

Rawa Bapir a,b,c, Shaho F. Ahmed b, Soran Mohammeed Gharib d, Deedar Qader a,b, Fahmi H. Kakamad b,e,f,*, Elenko Popov c,g, Noor Buchholz c, Abduwahid M. Salih b,ea Department of Urology, Sulaimani Teaching Hospital, Sulaimani, Kurdistan, Iraq b Smart Health Tower, Madam Mitterrand Street, Sulaimani, Kurdistan, Iraq c U-merge Ltd. (Urology in Emerging Countries), London, Athens, Dubai, United Kingdom of Great Britain thiab Northern Ireland d Shaheed Shawkat Haji Musheer Tsev Kho Mob, Said Sadiq, Sulaimani, Iraq e College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaimani, Kurdistan, Iraq f Kscien Organization, Hamdi Str, Azadi Mall, Sulaimani , Kurdistan, Iraq g Department of Urology, Poj huab tais Yoanna – ISUL, Sofia, Bulgaria

Peb nthuav tawm ib rooj plaub ntawm plab hnyuv pseudo-obstruction nyob rau hauv ib tug hluas poj niam tus neeg mob vim yog ib tug loj ua hauj lwm pheochromocytoma uas teb zoo rau phais tshem tawm cov qog. Daim ntawv tshaj tawm tau sau ua ke nrog SCARE 2020 cov lus qhia [4].

2. Case report

2.1. Cov ntaub ntawv tus neeg mob

Ib tug poj niam muaj hnub nyoog 29- xyoo muaj mob plab, mob, xeev siab, thiab ntuav nrog cem quav rau 3 lub lis piam. Nws tau mob ntshav siab thiab mob ntshav qab zib rau 3 xyoos dhau los. Nws noj 5 mg ntawm amlodipine, 80 mg ntawm valsartan, 2.5 mg ntawm bisoprolol, thiab 500 mg ntawm metformin txhua hnub. Nws tau qhia ntau zaus ntawm mob taub hau, palpitations, tawm hws hmo ntuj, thiab poob 17 kg ntawm 6 lub hlis.

2.2. Kev tshawb pom

Thaum kuaj, nws muaj pallor, dyspnea, cim plab zom mov, thiab txo qis plob tsis so tswj suab. Nws cov ntshav siab siab ntawm 200/120 mmHg. Nws muaj tachycardia (mem tes 120 bpm) thiab tachypnea (35 teev tsaus ntuj).

2.3. Diagnostic txoj kev

Cov qib metanephrine hauv cov ntshav tau nce siab, ntsuas 1203 pg / ml, cov ntshav suav tag nrho pom tias muaj cov kab mob normocytic anemia, ESR yog 140 mm / h, CRP 340 mg / L, HbA1C 6.72, ntshav cov ntshav hauv qib 3.9 meq / L, thiab cov thyroid ua haujlwm. nyob rau hauv ib txwm txwv. Ib qho ultrasound scan ntawm lub plab tau pom ib sab laug suprarenal loj. Abdominal CT pom ib heterogeneous, hyper-vascular loj nyob ze ntawm tus ncej qaum ntawm lub raum sab laug ntsuas 10.75 cm × 8.72 cm (Fig. 1).

2.4. Kev kho mob cuam tshuam

Tsis ntev tom qab tau txais kev tso npe, nws tau tsim muaj kev kub ntxhov siab thiab cov tsos mob ntawm pulmonary edema nrog kev poob ntawm oxygen saturation mus rau 60 feem pua ​​​​ntawm cov huab cua. Tom qab decompression ntawm nws lub plab mog nrog lub raj nasogastric, nws tau muab tso rau hauv siab txaus oxygen, furosemide txhaj, thiab xaiv alpha -1 thaiv tus neeg sawv cev doxazosin, vim tias tsis muaj kev xaiv alpha-blocking tus neeg sawv cev ntawm kev xaiv (phenoxybenzamine los yog phentolamamine) vim yog lub ntiaj teb kev kaw, tus neeg mob tau khaws cia nyob rau hauv kev soj ntsuam rau ib lub lis piam, thiab doxazocin tau nce mus rau 8 mg txhua hnub kom txog thaum nws cov ntshav txo qis mus rau 160/100 mmHg. Txawm li cas los xij, nws tseem muaj mob plab thiab cem quav hnyav heev. Yog li ntawd, nws tau teem sijhawm rau kev qhib sab laug adrenalectomy los ntawm txoj hauv kev anterior subcostal. Cov qog adrenal sab laug nrog cov qog tau raug tshem tawm hauv bloc. Qhov loj ntawm lub qog yog 10 cm × 8 cm. Postoperatively, nws tau raug saib xyuas zoo hauv chav saib xyuas mob hnyav rau thawj 24 teev. Tus neeg mob kho tau zoo. Nws lub plab zom mov rov pib dua tam sim ntawd, thiab nws muaj peev xwm dhau los ntawm ntau cov quav xoob sai tom qab kev phais. Kev kuaj mob histopathological thiab immunohistochemical ntawm tus qauv kuaj pom qhov kev kuaj mob ntawm pheochromocytoma. Muaj cov tshuaj tiv thaiv cytoplasmic diffused rau chromogranin thiab cov tshuaj tiv thaiv tsis zoo rau inhibin (Daim duab 2) nrog Pheochromocytoma ntawm Adrenal Gland Scaled Score (PASS) ntawm 7.

2.5. Kev ua raws thiab qhov tshwm sim

Nws lub sijhawm postoperative ntxiv yog qhov tsis zoo thiab nws raug tso tawm rau nws plaub hnub tom qab. Nws qhov kev kuaj mob qog nqaij hlav rau metastases yog qhov tsis zoo, thiab nws qhov kev ua haujlwm tom qab ua haujlwm tau pom tias ua tiav kev ua tiav ntawm nws cov ntshav metanephrines. Kaum rau lub hlis tom qab, nws tseem nyob rau hauv biochemical remission. Tag nrho cov tshuaj tiv thaiv hypertensives thiab qhov ncauj los tiv thaiv hyperglycemic cov neeg ua haujlwm raug tso tseg.

3. Kev sib tham

Cov txheej txheem rau cem quav thiab plab hnyuv pseudo-obstruction nyob rau hauv cov neeg mob nrog pheochromocytoma yog qhov ntau tshaj ntawm catecholamines uas ua rau cov alpha-adrenergic receptors inhibiting lub secretion ntawm acetylcholine los ntawm postganglionic paj terminals thiab tseem thaiv excitatory postsynaptic muaj peev xwm nyob rau hauv cov neurons. kev txiav txim ntawm intrinsic inhibitory mechanisms [5] Acetylcholine zoo li yog qhov tseem ceeb excitatory neurotransmitter nyob rau hauv lub paj hlwb enteric thiab tau pom tias yuav ua rau kom lub amplitude ntawm contractions ntawm txoj hnyuv ncig du nqaij nyob rau hauv cov hnyuv luav, yog li tag nrho cov nyhuv ntawm alpha-adrenergic stimulation yuav inhibition ntawm txoj hnyuv motility [6] . Gastrointestinal pseudo-obstruction, uas txawv ntawm txhua yam teeb meem nyob rau hauv uas tsis muaj ib tug demonstrable mechanical ua rau pom, yog ib tug tsis tshua muaj tab sis muaj feem xyuam rau lub neej-kev nyuaj siab ntawm pheochromocytoma. Hauv kev tshuaj xyuas ntawm 34 qhov xwm txheej ntawm pseudo-kev cuam tshuam vim yog catecholamine-secreting qog los ntawm Osinga li al., plab hnyuv perforation tshwm sim hauv 15 feem pua ​​​​ntawm cov neeg mob, thiab 47 feem pua ​​​​tau tuag hauv ib xyoos tom qab [7].

Noguchi et al. tau sau tseg qhov kev sib raug zoo ntawm qog loj thiab metabolic kev ua ntawm catecholamine-secreting qog nyob rau hauv 16 mob nrog plab hnyuv pseudo-obstruction los ntawm paralytic ileus [8]. Qhov no tau lees paub hauv lwm daim ntawv tshaj tawm [9]. Peb cov ntaub ntawv sib raug nrog cov kev tshawb pom no, nrog rau cov qog loj ntawm 350 g thiab cov ntshav metanephrine qib ntawm 1203 pg / ml. Kev kho mob ntawm pheochromocytomas yuav tsum tau ua tib zoo pib thiab nce-titration ntawm alpha-blocking cov neeg ua haujlwm kom ua tiav qhov kev pom zoo alpha-receptor blockade, ua raws li beta-blockers los tswj cov peev xwm tachyarrhythmia [10]. Txawm li cas los xij, peb tus neeg mob twb tau txais 2.5 mg ntawm bisoprolol, uas peb ntseeg tias yog ib qho ntawm cov ua rau nws tus mob tsis zoo thiab nws cov ntshav siab nce sai. Ntxiv mus, peb tsis tuaj yeem tau txais phenoxybenzamine lossis phentolamamine, uas tsis yog xaiv alpha-blocking tus neeg sawv cev thiab tau tawm tswv yim los ntawm ntau tus kws sau ntawv ua thawj kab ntawm kev tswj cov kab mob plab hnyuv ntawm pheochromocytoma [10], vim muaj kev kaw thoob ntiaj teb los tiv thaiv kev sib kis. Ntawm COVID 19. Hloov chaw, peb pib nrog koob tshuaj tsawg doxazocin nrog nce-titration mus txog 8 mg / hnub, uas pab tswj nws cov ntshav siab, txawm tias tsis muaj txiaj ntsig rau nws qhov mob. Cov tom kawg teb tau zoo rau kev phais tshem tawm ntawm cov qog. Ib rooj plaub zoo sib xws thiab cov txiaj ntsig tau tshaj tawm yav dhau los [11].

Kev kuaj mob histopathological tau nthuav tawm pheochromocytoma ntawm lub qog adrenal nrog cov qhab nia ntsuas (PASS) ntawm 7, uas nyiam pheochromocytoma malignant. Kev kuaj qog nqaij hlav tsis tau nthuav tawm cov kab mob metastases, yog li nws tuaj yeem ua rau muaj kev nyab xeeb tias lub hauv paus ntawm cov ntshav ntau dhau ntawm catecholamine raug tshem tawm. Tus neeg mob tau txais kev ua haujlwm ntawm lub plab zom mov sai sai. Ntshav siab thiab ntshav qab zib ploj lawm. Hauv kev xaus, kev ua haujlwm pheochromocytoma nrog cov cim tshwj xeeb thiab cov tsos mob tuaj yeem suav nrog hauv kev kuaj mob sib txawv ntawm cem quav lossis ua haujlwm pseudo-obstruction hauv cov neeg mob uas muaj tachycardia, diaphoresis, thiab mob taub hau. Kev lees paub ntxov thiab kev tswj hwm ntawm cov neeg mob no yog qhov tseem ceeb, vim tias kev ncua sij hawm tuaj yeem ua rau lub plab zom mov tsis zoo nrog cov teeb meem loj lossis kev tuag.

Cov ntaub ntawv

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