Hematopoietic Stem Cell Transplantation rau cov menyuam yaus uas muaj qhov tsis zoo ntawm kev tiv thaiv kab mob
Apr 21, 2023
Qhov no yog ib qho kev soj ntsuam rov qab los ntawm kev soj ntsuam cov yam ntxwv ntawm cov menyuam yaus uas muaj qhov tsis zoo ntawm kev tiv thaiv kab mob uas tau txais kev hloov pauv ntawm cov qia hlwb (HSCT). Txawm hais tias lub hnub nyoog nruab nrab ntawm kev kuaj mob yog 24.4 lub hlis, nws yog 51.9 lub hlis ntawm HSCT. Nws yog qhov yuav tsum tau ceev nrooj los txhim kho kev paub, nthuav cov npe pub dawb thiab pib kuaj cov menyuam yug tshiab rau kev yug menyuam tsis raug lossis tiv thaiv kab mob.
Kev tiv thaiv kab mob yog hais txog lub cev lub peev xwm los tiv thaiv kev cuam tshuam ntawm ntau yam kab mob sab nraud, uas yog pom tau los ntawm kev ua haujlwm ntawm lub cev tiv thaiv kab mob. Nyob rau hauv ib txwm muaj xwm txheej, lub cev tiv thaiv kab mob yuav paub txog ntau yam kab mob kis rau tib neeg lub cev, thiab tom qab ntawd ua tiav txoj haujlwm ntawm kev tawm tsam cov kab mob los ntawm kev ua ntawm lub cev tiv thaiv kab mob thiab lub cev tiv thaiv kab mob.
Hauv peb lub neej txhua hnub, peb yuav tsum tau ua tib zoo saib xyuas kev txhim kho peb txoj kev tiv thaiv kab mob, xws li kev noj zaub mov zoo thiab kev tawm dag zog kom raug. Cistanche tuaj yeem txhim kho kev tiv thaiv hauv peb lub neej. Cistanche yog nplua nuj nyob rau hauv ntau yam tshuaj antioxidant, xws li vitamin C, Carotene, thiab lwm yam. Cov khoom xyaw no tuaj yeem tshem tawm cov dawb radicals, txo oxidative kev nyuaj siab, thiab txhim kho kev tiv thaiv ntawm lub cev.
Nyem qhov txiaj ntsig kev noj qab haus huv ntawm cistanche
Ntsiab lus:
Cov kab mob tseem ceeb ntawm lub cev tsis muaj zog.
Inborn yuam kev ntawm kev tiv thaiv kab mob los yog thawj lub cev tsis muaj zog tiv thaiv kab mob (PIDDs) tshwm sim nrog ntau zaus ntawm 1 ntawm 5000 mus rau 1 ntawm 1000 [1] thiab feem ntau misdiagnosed ua rau zam tsis tau morbidity thiab tuag [2]. Kev kuaj mob thiab kev hloov pauv ntawm cov kab mob hematopoietic qia cell (HSCT) tsis tuaj yeem nkag mus tau zoo ib yam [3].
Tsoom Fwv Tsev Kawm Ntawv Kho Mob, Kozhikode, lub tsev kho mob tertiary hauv Kerala, thiab CSIR Lub Tsev Kawm Ntawv ntawm Genomics thiab Integrative Biology, Delhi tau ua ib qho kev qhia txog kev tiv thaiv kab mob hauv lub cev tsis muaj zog tshaj tsib xyoos dhau los. Txawm hais tias HSCT feem ntau yog qhov kev xaiv kho mob nkaus xwb, peb nyob ntawm cov chaw nyob sab nraud lub xeev. Txoj kev tshawb no tau tsim los sau cov yam ntxwv kho mob ntawm cov menyuam yaus uas tau txais HSCT rau qhov tsis zoo hauv kev tiv thaiv kab mob.
Cov ntaub ntawv hauv tsev kho mob ntawm cov menyuam yaus uas muaj PIDDs uas tau mus koom lub tsev kho mob tiv thaiv kab mob tsis muaj zog txij lub Rau Hli 2015 txog Lub Tsib Hlis 2020 tau txais thiab cov ntaub ntawv los ntawm cov neeg tau txais HSCT raug tshuaj xyuas. Tsuas yog cov menyuam yaus uas tau ua tiav tsawg kawg 3 lub hlis tom qab HSCT suav nrog. Cov kev hloov pauv tau kawm suav nrog hnub nyoog ntawm qhov pib kuaj mob thiab ntawm HSCT, poj niam los txiv neej, kev sib raug zoo nrog cov qia cell pub, lub sijhawm txij li HSCT, thiab kuaj cov caj ces lossis cov cim phenotypic. Ntau qhov sib txawv tau nkag mus rau hauv daim ntawv Excel cov ntaub ntawv thiab zaus thiab cov koom haum tau suav nrog siv cov ntaub ntawv txheeb cais Epi Info (version 7.2.3.1).
HSCT tau ua tiav hauv 13/67 (19.4 feem pua, 11 tus tub). Cov kev qhia suav nrog Wiskott-Aldrich syndrome (4, 30.8 feem pua), thiab leukocyte adhesion deficiency, hnyav ua ke tiv thaiv kab mob, thiab X-linked agammaglobulinemia nyob rau hauv ob qho tib si (15.4 feem pua) congenital neutropenia Fanconi anemia, thiab hyper IgM syndrome tau kuaj pom tus menyuam. txhua. Lub hnub nyoog nruab nrab (IQR) ntawm kev kuaj mob ntawm cov menyuam yaus uas tau txais HSCT yog 14 lub hlis (thawj quartile, III quartiles). Qhov nruab nrab (IQR) hnub nyoog ntawm HSCT yog 27.5 (thawj quartile, III quartiles) lub hlis thiab qhov nruab nrab (IQR) lub sijhawm nruab nrab ntawm kev kuaj mob thiab HSCT yog 7 (thawj quartile, III quartiles) lub hlis.
Kev mob ntsws rov tshwm sim yog qhov tshwm sim ntau tshaj plaws hauv 7 (54 feem pua) cov menyuam yaus, ua raws li cov tawv nqaij thiab cov nqaij mos mos hauv 6 (46 feem pua) thiab rov tshwm sim otitis media hauv 4 (30.8 feem pua). Feem ntau cov abscesses, raws plab, thiab los ntshav tau tshwm sim nyob rau hauv 2 (15 feem pua) cov me nyuam txhua. HSCT tau ua tiav hauv tus menyuam asymptomatic nrog Fanconi anemia tom qab nws tus niam laus tuag rau tib tus kab mob.
Ntawm 13 cov menyuam yaus uas tau txais HSCT, 9 (69 feem pua) cov menyuam yaus muaj cov kwv tij sib koom pub dawb, thiab 2 tus menyuam txhua tus (15 feem pua) tau sib piv cov kev hloov pauv tsis muaj feem cuam tshuam (MUDs) [4] thiab haploidentical qia cell transplants. Kev txo qis kev siv zog (RIC) [5] nrog treosulfan thiab fludarabine tau siv thiab 12 tus menyuam tau txais kev txhawb nqa. Muaj ib qho kev tsis lees paub nrog kev hloov kho autologous, thiab HSCT thib ob tau ua rau muaj kev cuam tshuam. Cov teeb meem tom qab HSCT suav nrog cov kab mob sepsis, cytomegaloviral reactivation, steroid-induced hypertension, thiab graft tiv thaiv tus kab mob. Tsis muaj kev tuag thiab lub sijhawm nruab nrab ntawm kev hloov pauv tsis pub muaj sia nyob yog 25.1 lub hlis.
HSCT tau ua tiav rau 2 (15 feem pua) cov menyuam yaus nrog XLA. Txawm hais tias qhov no tsis yog tus qauv kev kho mob, tab sis nws yog ib qho kev xaiv uas muaj thiab tus nqi ntawm immunoglobulin hloov kho yog txwv tsis pub thiab cov niam txiv tsis xav hloov lub neej [6].
Lub sijhawm nruab nrab ntawm qhov pib ntawm cov tsos mob rau kev kuaj mob yog 9 lub hlis. Qhov no qhia txog qhov yuav tsum tau txhim kho kev paub ntawm cov kws kho mob [2]. Qhov nruab nrab nruab nrab ntawm kev kuaj mob thiab HSCT yog 40.9 lub hlis, suav nrog kev tuag siab. Cov txiaj ntsig tau zoo tau piav qhia nrog HSCT ua ntej 3.5 lub hlis ntawm hnub nyoog ua ntej pib muaj kev sib kis kab mob [7,8]. Tus menyuam yau tshaj plaws uas tau txais HSCT hauv cov koob no yog 5 lub hlis.
Qhov txiaj ntsig ntawm HSCT rau cov menyuam yaus uas tsis sib haum nrog cov neeg pub dawb (MUDs) thiab cov neeg pub nyiaj haploidentical tau txhim kho thoob plaws ntiaj teb [4,9] ob tus menyuam hauv cov koob no tau txais txiaj ntsig zoo. Cov kev txwv ntawm txoj kev tshawb no suav nrog cov qauv me me thiab lub sijhawm hloov pauv txij li HSCT nrog kev rov qab tsis zoo.
Lub ntsiab stumbling blocks rau kev siv dav ntawm HSCT tseem yog tus nqi thiab tsis muaj cov neeg pub dawb. Txoj cai tswjfwm kab mob tsawg hauv tebchaws hais txog kev txhawj xeeb loj ntawm cov tsev neeg cuam tshuam yuav yog txoj hauv kev mus rau tom ntej. Kev paub txog PIDDs yuav tsum tau nce sai, cov npe pub dawb tau nthuav dav thiab tsoomfwv cov peev nyiaj tau yooj yim. Ib qho kev kuaj xyuas menyuam mos yuav pab txo qis kev tuag.
Kev lees paub:
Dr. Dhanasooraj, Scientist, MRU, Govt. Medical College, Kozhikode; Dr. Ajith Kumar VT thiab Dr. MP Jayakrishnan, Department of Pediatrics, Government Medical College, Kozhikode; Athulya EP, Junior Research Fellow; thiab Abhinav Jain thiab Dr. Sridhar Sivasubbu ntawm CSIR - Lub Tsev Haujlwm ntawm Genomics thiab Integrative Biology, Delhi. Kev coj ncaj ncees: GMCKKD/RP2020/IEC/428; hnub tim 29 lub Tsib Hlis 2017.
Cov neeg koom nrog:
GMG: conceptualization ntawm txoj kev tshawb no, cov ntaub ntawv tsom xam, thiab sau ntawv. RR thiab RU saib xyuas kev ua haujlwm thiab txheej txheem rau HSCT; VS: tau ua haujlwm genetic workup rau tus neeg mob. Txhua tus kws sau ntawv pom zoo rau daim ntawv kawg ntawm daim ntawv. Cov Nyiaj Txiag: Pawg Saib Xyuas Kev Tshawb Fawb Txog Kev Tshawb Fawb thiab Engineering, Delhi, thiab Lub Tsev Haujlwm Saib Xyuas Kev Tiv Thaiv Kab Mob Thaum Ntxov (FPID); Cov txiaj ntsig sib tw: Tsis tau hais.
REFERENCES
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2. Kapoor N, Raj R. Hematopoietic stem cell transplantation for the first immune deficiency disorders. Indian J Pediatr. Xyoo 2016; 83:450- 54.
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7. Szabolcs, M. Cavazzana-Calvo, A. Fischer, Veys P. Bone marrow transplantation for the first immunodeficiency disease. Pediatr Clinics North Am. 2010; 57: 207-237.
8. Mitchell R, Nivison-Smith I, Anazodo A, et al. Cov txiaj ntsig ntawm Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency: Ib Daim Ntawv Qhia los ntawm Australian thiab New Zealand Children's Hematology Oncology Group thiab Australasian Bone Marrow Transplant Recipient Registry. Kev hloov pauv ntawm cov hlab ntsha hauv lub cev. Xyoo 2013; 19:338-43.
9. Yadav SP. Kev hloov cov pob txha pob txha rau thawj lub cev tsis muaj zog hauv Is Nrias teb: Yav dhau los, tam sim no thiab yav tom ntej. Indian Pediatr. 2018;55:657-58.
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