Disaccharidase deficiency yog ib pab pawg ntawm cov kab mob hauv plab hnyuv uas tshwm sim los ntawm kev txo qis lossis ua tsis tiav ntawm ib qho enzymes. Tus neeg mob tau ntsib teeb meem ntawm kev nqus thiab zom cov carbohydrates: sucrose, m altase, trecolase, lactose.
Etiology thiab pathogenesis
Disaccharidase deficiency tuaj yeem kis tau los yog yug los.
Pib, lossis qhov tsis txaus ntseeg yog tsim tawm tsam keeb kwm ntawm caj ces tsis ua haujlwm thiab muaj txij li thaum tus menyuam yug los. Tus kab mob yog txuam nrog ib tug tsis xws luag nyob rau hauv lub mucous daim nyias nyias ntawm cov hnyuv. Feem ntau, cov kab mob tshwm sim hauv cov menyuam mos ntxov ntxov, tab sis thaum kawg ploj mus, vim qhov ua haujlwm enzymatic ntawm txoj hnyuv tau rov qab los.
Lactose intolerance yog qhov tshwm sim hauv cov menyuam yaus thiab tshwm sim nws tus kheej tom qab pub niam mis. Qhov tshwm sim ntawm tus kab mob nyob rau hauv txhua lub teb chaws yog nyob ntawm cov khoom noj ib txwm muaj. Yog tias cov mis nyuj yog cov khoom noj uas pheej yig thiab nyiam, ces qhov tshwm sim yog siab. Piv txwv li, hauv Asia, Africa thiab AsmeskasTus nqi ntawm cov neeg raug kev txom nyem los ntawm disaccharidase deficiency yog 70% ntawm tag nrho cov pejxeem thiab ntau dua, thiab nyob rau hauv lub Scandinavian lub teb chaws tsis pub tshaj 15%. Hauv tebchaws Russia, qhov xwm txheej ntawm tus kabmob tsis tshaj 18%.
Secondary lossis tau txais hom tsis txaus yog tsim los ntawm keeb kwm ntawm cov kab mob plab hnyuv. Feem ntau, cov teeb meem tshwm sim tom qab kis kab mob ntawm txoj hnyuv. Cov txheej txheem biochemical tshwm sim hauv cov hlwb ntawm lub cev, ua rau tsim cov disaccharidase deficiency.
Zaj caj ces
Nyob rau hauv ntau txoj hauv kev, cov kev xav ntawm cov kws kho mob txog qhov teeb meem ntawm disaccharidase deficiency txawv, tab sis lawv yog ib qho kev paub tseeb ntawm ib yam - tus kab mob muaj cov caj ces. Muaj ntau qhov kev tsis sib haum xeeb txog yuav ua li cas tsis muaj peev xwm tau txais txiaj ntsig. Qee cov kws tshawb fawb tau hais tias tus kab mob kis tau los ntawm txoj kev autosomal tseem ceeb, lwm tus - hauv txoj kev autosomal recessive. Tab sis kuj tseem muaj teeb meem uas txhua tus kws tshaj lij hauv cov tshuaj tshawb fawb pom zoo.
Ua ntej ntawm tag nrho cov, pathology yog sib npaug ntawm cov menyuam yaus hauv cov tub thiab ntxhais. Tsis tas li, cov kws kho mob pom zoo tias kev tsis haum rau isom altase thiab m altase feem ntau yog asymptomatic kiag li. Thiab qhov tsis txaus sucrose-isom altase tseem nyob rau lub neej thiab feem ntau tshwm sim tawm tsam keeb kwm ntawm lactose intolerance. Tom qab tag nrho, ob qho teeb meem yog qhov tshwm sim ntawm ib qho kev hloov pauv.
Symptomatics in Pediatrics
Disaccharidase deficiency hauv cov menyuam yaus yog tus cwj pwm los ntawm kev ua tsis tiavcarbohydrates thiab nce osmotic siab nyob rau hauv txoj hnyuv lumen. Hauv qhov no, dej thiab electrolytes nkag mus rau lumen, thiab cov txheej txheem fermentation pib. Raws li qhov tshwm sim ntawm fermentation ntawm carbohydrates, organic acids yog tsim. Vim tias muaj cov pa roj carbon dioxide thiab hydrogen ntau ntxiv hauv cov hnyuv, kev tsim roj ntau pib pib. Raws li qhov tshwm sim, tus menyuam muaj mob raws plab uas kav ntev tshaj peb lub lis piam. Cov kab mob lactose intolerance ntawm Durand hom tshwm sim nyob rau hauv keeb kwm yav dhau ntawm copious quav uas ua npuas ncauj thiab muaj ib tug muaj zog qaub tsw. Tus me nyuam muaj eructation, xav tias hnyav hauv plab. Qee zaum, muaj qhov txo qis hauv lub cev hnyav tiv thaiv keeb kwm ntawm lub cev qhuav dej. Qhov concentration ntawm lactose hauv cov ntshav nce ntxiv. Feem ntau, cov txiaj ntsig zoo tau pom, thiab thaum muaj hnub nyoog 2-3 xyoos, tag nrho cov tsos mob ploj mus, txoj hnyuv ua haujlwm rov qab los. Yog tias tus kab mob hnyav heev, ua rau mob ntev, tom qab ntawd cov tsos mob ntawm lub plab zom mov tsis zoo tuaj, uas yog nrog los ntawm cov kab mob metabolic.
Yog tias tus menyuam muaj sucrase lossis isom altase daim ntawv ntawm tus kabmob (yam tsawg tshaj plaws ntawm pathology), ces cov tsos mob tshwm sim los ntawm kev noj sucrose hauv lub cev thiab tshwm sim raws li cov quav dej. Thaum kuaj xyuas cov quav, pom muaj ntau cov lactic acid. Tus menyuam yuav pib ntuav. Yog hais tias tus me nyuam noj ntau ntau ntawm lactose nyob rau hauv ib pluas noj, ces nws muaj chills thiab tachycardia, hypovolemia, thiab nce hws. Feem ntau nyob rau hauv cov menyuam yaus nrog daim ntawv no tsis txaus, muajstarch intolerance.
Cov tsos mob hauv cov neeg laus
Disaccharidase deficiency hauv cov neeg laus yog mob me dua. Cov neeg mob muaj kev cuam tshuam los ntawm ntu ntu lossis raws plab. Pathology yog nrog los ntawm me ntsis mob nyob rau hauv lub plab mog thiab flatulence, rumbling. Qhov hnyav ntawm cov tsos mob feem ntau yog nyob ntawm tus nqi ntawm cov carbohydrates noj rau hauv lub cev. Qhov no lactose intolerance tseem hu ua Holrel.
ntsuas ntsuas
Tom qab sau cov anamnesis, kev tshawb fawb biochemical ntawm plasma thiab kev sim rau rhiab heev rau carbohydrates (cov hmoov txhuv nplej siab, lactose, thiab lwm yam) yog qhov yuav tsum tau ua. Kev kuaj X-ray ntawm lub plab zom mov lossis biopsy ntawm txoj hnyuv mucosa yuav raug sau tseg. Kev kuaj ntshav tom qab lactose nkag mus rau hauv lub cev tso cai rau koj los txiav txim siab qib ntawm cov piam thaj. Ib qho mucosal biopsy tsuas yog ua los txiav cov qog nqaij hlav cancer xwb.
Hauv kev kho menyuam yaus, kev tshuaj xyuas fecal yog qhov yuav tsum tau tsim kom muaj qib pH thiab tus nqi ntawm carbohydrates. Tsis tas li ntawd, kev tshuaj xyuas rau disaccharidase tsis muaj peev xwm tso cai rau koj los txiav txim seb qhov muaj pes tsawg leeg ntawm cov roj ntsha nruab nrab, cov qog nqaij hlav neoplasm, helminths, fatty acids thiab erythrocytes, uas yog, ua qhov kev kuaj mob sib txawv..
Kev ntsuas lactose kam rau ua feem ntau ua, uas tso cai rau koj los txiav txim siab qib glycemia tom qab haus cov lactose. Thiab txhawm rau tsim kom muaj kev ua haujlwm ntawm biotopes ntawm cov mucous membrane ntawm txoj hnyuv me, tus menyuam tau muab kefir.
Kho ntawm thawj daim ntawv ntawm pathology
Kev kho Disaccharidaseinsufficiency nyob rau hauv thaum yau yog nqa tawm tawm tsam keeb kwm ntawm ib tug tag nrho los yog ib feem ntawm kev cais tawm ntawm cov zaub mov provokes tus kab mob. Kev noj zaub mov kom zoo yog txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhawm rau txhaws. Yog tias kuaj tau alactosia, ces cov zaub mov yuav tsum tau ua raws li lub neej.
Yog tias kev noj zaub mov raug xaiv raug, ces qhov kev txhim kho ntawm tus menyuam tus mob tuaj yeem pom nyob rau hnub peb. Tus me nyuam txoj kev xav zoo, qab los noj mov zoo, mob plab nres, flatulence txo.
Cov tsos mob hnyav ntawm disaccharidase tsis muaj peev xwm qee zaum tau txo qis nrog kev pab ntawm kev npaj enzyme. Tab sis nyob rau hauv kev kho mob ntawm cov me nyuam, cov tshuaj tau sau nyob rau hauv ib tug luv luv chav kawm - los ntawm 5 mus rau 7 hnub. Ntawm cov tshuaj yuav yog Creon thiab Pancitrate. Hauv 30-45 hnub tom ntej no, probiotics yog siv, uas yuav normalize lub plab hnyuv microflora. Tus kws kho mob yuav muab "Hilak forte", "Linex", "Biovestin-lacto" rau cov kua mis thiab lwm yam tshuaj.
Hom me nyuam mos thiab lub sijhawm noj zaub mov
Rau cov menyuam yaus hauv thawj xyoo ntawm lub neej, ob hom kev sib xyaw ua ke:
- Nqaij. Cov protein hauv cov khoom no yog sawv cev los ntawm dextrin-m altose, casein. ("Bebilak", "L-110", "Eldolak-F");
- Soy lossis tsis muaj mis nyuj (Nutria-soy, Humana SL, Enfamil-soy thiab lwm yam).
Cov khoom noj uas muaj cov ntsiab lus siab ntawm lactose raug tshem tawm tag nrho los ntawm kev noj zaub mov: mis mis, mis nyuj, mis nyuj cheese thiab ib hnub kefir. Yog tias tus menyuam tau pub niam mis, ces tsum tsis txhob pub niam misme nyuam tsis pom zoo.
cov zaub mov rau cov menyuam uas tsis kam noj yog npaj tsis yog mis nyuj, tab sis nrog cov khoom sib xyaw. Twb tau nyob rau 4-4, 5 lub hlis ntawm lub neej, txiv hmab txiv ntoo purees, ci txiv apples yog qhia. Nws ntseeg tau tias thawj cov khoom noj tseem ceeb ntxiv yuav tsum muaj cov khoom lag luam nrog cov zaub ntxhib fiber ntau thiab cereals (buckwheat, mov). Los ntawm ib nrab ntawm lub neej, nws yog qhov ua tau kom maj mam qhia cov khoom lag luam nrog cov ntsiab lus tsis muaj lactose (butter, cheese nyuaj, tsev cheese whey).
Kev noj zaub mov rau disaccharidase tsis txaus yuav kav ntev li ntawm 2 mus rau 6 lub hlis, tab sis lub sijhawm yog nyob ntawm tus nqi ntawm kev rov ua haujlwm lactose.
Yog tus menyuam tsis kam sucrose thiab isom altase, ces cov hmoov txhuv nplej siab thiab dextrins hauv cov zaub mov yuav txo. Cov menyuam yaus no tau pom cov mis mis nrog lactose carbohydrate Cheebtsam. Cov zaub mov ntxiv tau pom zoo kom pib nrog qos yaj ywm mashed los ntawm zaub thiab txiv hmab txiv ntoo (peas, Brussels sprouts, cauliflower, broccoli, taum ntsuab, txiv lws suav, cranberries, cherries).
Khoom noj khoom haus hauv daim ntawv thib ob ntawm pathology
Kev noj zaub mov thaum kuaj pom qhov tsis txaus siab thib ob (lactose form) qhia txog kev cais tawm ib ntus los ntawm kev noj cov khoom noj siv mis thiab mis nyuj tag nrho. Tso cai siv butter thiab nyuaj cheese. Koj tuaj yeem noj zaub thiab txiv hmab txiv ntoo, nqaij, ntses, hmoov, qe, uas yog, txhua yam khoom noj uas tsis muaj lactose. Nws tsis pom zoo kom koom nrog hauv cov khoom bakery, vim lawv feem ntau muaj cov mis nyuj qab zib.
Nrog sucrase-isom altase daim ntawv ntawm tus kab mob los ntawm kev noj hausCov khoom noj uas muaj m altose thiab qab zib tsis suav nrog. Lawv tuaj yeem hloov nrog fructose, galactose lossis qabzib. Honey pub.
Yog kuaj pom qhov tsis txaus trecalase, koj yuav tsum tsis txhob noj nceb. Tab sis qhov tseem ceeb tshaj plaws, nyob rau hauv daim ntawv thib ob ntawm qhov tsis txaus, yuav tsum tau them nyiaj tshwj xeeb rau kev kho tus kab mob hauv qab uas ua rau cov tsos mob.
Cov lus qhia kho mob
Disaccharidase deficiency yog ib yam kab mob nyuaj, txawm tias qhov tseeb tias pathology yuav tsum tau kho cov zaub mov xwb. Tus kws kho mob, thaum sau cov khoom noj, yuav tsum coj mus rau hauv tus account ntau yam:
- ua haujlwm ntawm lub siab, txiav txiav thiab lwm yam kabmob sab hauv ntawm tus neeg mob;
- hnub;
- plab rhiab heev rau osmotic load;
- tus yam ntxwv ntawm tus kheej thiab kev nyiam noj zaub mov;
- qab zib.
Yog li ntawd, nws yog ib qho tseem ceeb heev tsis txhob noj tshuaj rau tus kheej thiab sab laj nrog tus kws kho mob tshwj xeeb hauv lub sijhawm, tshwj xeeb tshaj yog thaum nws los txog rau tus menyuam hnub nyoog qis dua ib xyoos. Hauv qhov no, nws feem ntau nyuaj rau kev noj zaub mov.
