Vana vanoda iron mukati mokudya kwavo, ndicho chikonzero vabereki vakawanda vanoedza kuve nechokwadi chokuti vana vavo vanodya zvokudya zvakawanda zvesimbi zvakasikwa zuva rega rega. Pasina simbi, vanova pangozi yekushaya simba kwesimbi .
Chii chinoitika kana iwe ukawana dare rakawandisa, zvakadaro?
Nenzira yakanaka, iyo haisi dambudziko kuvana vakawanda, sezvo muviri wavo unoronga kuti yakawanda yakawanda sei uye inochengetedza. Asi kana vane hemochromatosis, chirwere chemafu chinogona kupiwa kubva kuvabereki vemwana, mwana anokwanisa kuwana yakawanda iron, zvichiita kuti iron yakawedzerwa ichengeterwe muropa rechiropa, mwoyo, pancreas, nezvimwe nhengo.
Zviratidzo zveHemochromatosis
Zviratidzo, zviratidzo, uye zvinetso zvinosanganiswa ne hemochromatosis zvinogona kusanganisira:
- marwadzo (arthralgia) uye arthritis
- kuneta
- kuderedza simba
- kuonda
- marwadzo emudumbu
- bvudzi rinopera
- zvinetso zvemoyo, kusanganisira chirwere chemoyo chinokanganisa nemoyo kusagadzikana uye arrhythmias
- chiropa chechiropa, nechiropa chakawedzerwa (hepatomegaly), cirrhosis, uye chiropa kusakundikana
- grey kana bhandi ganda rinoputika
- hypothyroidism
- chirwere cheshuga
- kusava nesimba
- amenorrhea (kusavapo kwenguva)
Vana vazhinji vane hemochromatosis havana zviratidzo, kunyange zvakadaro, sezvo zviratidzo zvingasazoitika kusvikira pane imwe nguva muhupenyu, sezvo simbi yakawedzera inowedzera mumuviri wavo.
Kuongorora Hemochromatosis
Sezvo vana vane hemochromatosis kazhinji vasina zviratidzo zvekare, zvinogona kuva zvakaoma kuongororwa. Chiitiko chakajairika ndechokuti hama yepedyo yepedyo inorwara nehemochromatosis uye zvakare nokuti iri chirwere chemazwi, dzimwe nhengo dzemhuri dziri kuedzwa.
Saka mwana anogona kuongororwa nevana vake kana asati aine zviratidzo, nekuda kwemhuri yake yekare.
Kuedza kwe hemochromatosis kunogona kusanganisira kuongororwa ropa kuitira kuyera huwandu hwedare mumuviri wemwana, kusanganisira kutsvaga:
- yakakwirira transferrin kuwedzera
- yakakwirira ferritin
- yakakura serum iron
- kuderera kwesimbi yakasunga simba (TIBC)
Izvi zvinonzi mapurisa ematare pane mabhiza akawanda.
Genetic Testing for Hemochromatosis
Izvo zvinokwanisikawo kuedza mazita ekutsvaga mazita ekutsvaga chimiro chisina kukwana (HFE gene) inokonzera nhaka yehemochromatosis, kusanganisira C282Y, H63D, uye shanduko yeS65C. Vana vane makopi maviri emajini anonzi HFE akachinjwa achava ne hemochromatosis (ivo vari pangozi yezviratidzo kana vane makopi maviri emuviri iwoyo mutated), asi kana vakangova neimwe yemagenene akachinjwa, ivo vachave vatakuri uye hazvizovi nehumwe humwe zviratidzo zve hemochromatosis.
Basa rekuongorora mazita ekuberekwa kwevana rinopesana kunyange zvakadaro. Ramba uchiyeuka kuti American Academy of Pediatrics inotaura kuti "kuderedza kuderedza kana kufa kwepamusana pekuenzanisa kwemajini hazvina kuratidzirwa nemamiriro mazhinji ezvo kuongororwa kwakaitwa kare kunowanikwa," uye kuti "ruzivo rwehuwandu hwemamiriro ezvinhu hunogona kuunza zvakaipa zvinopindura mupfungwa uye, zvichida, kusarurwa nevatsigiri, vashandi, kana vamwe. "
Muzvizhinji, kune mamiriro mazhinji, kusanganisira hemochromatosis, vanorayira kubvisa maitiro ekuberekorora kusvikira mwana ari mukuru kana akakwanisa kuita sarudzo, ruzivo rwekuziva.
Chimwe chikonzero chakanaka chekudzivisa kuongorora maitiro evanhu ndeyekuti 50% chete yevanhu vanoedza kuti vawane hemochromatosis zvirokwazvo vanoita zviratidzo.
Kune rimwe divi, nyanzvi dzeNational Human Genome Research Institute dzinotaura kuti "kubva pakutanga kuongororwa kunoreva kushandiswa kwekutanga nekugadzirisa kurapa zvirwere, zvinogona kuva nezvikomborero mukuziva vana vari panjodzi." Vanowedzerawo kuti "kuongorora mazita ekuberekwa kunoonekwa sekubatsira kune vakaroorana vakaronga kuva nemhuri."
Muzvizhinji, vana vanongoda kuongororwa kuti vaongorore hemochromatosis kana:
- Vabereki vose vane hemochromatosis (mwana achava ne hemochromatosiswo)
- mumwe mubereki ane hemochromatosis uye imwe yacho inotakura hemochromatosis (50% mukana mwana achava ne hemochromatosis)
- Vabereki vose vari vatakuri ve hemochromatosis (25% mukana mwana achave ne hemochromatosis)
Kana mubereki asiripo nokuda kwekuongorora mazita, iwe unogona kufungidzira njodzi yemunhu yekuwana majeni ehemochromatosis zvichienderana neveupi wemhuri ane hemochromatosis:
- kana amainini vako kana vanun'una vako vane hemochromatosis, ikozvino mukana wako wekuva nejena maviri ye hemochromatosis inenge 1%
- kana mumwe wevabereki vako aine hemochromatosis, ipapo mukana wako wekuva nejena maviri ye hemochromatosis inenge 5%
- kana mukoma wako kana hanzvadzi yako ine hemochromatosis, ipapo mukana wako wekuva nejena mbiri ye hemochromatosis inenge 25%
Uye ramba uchiyeuka kuti nyanzvi dzakawanda dzinokurudzira kuti mazita ekuongorora maitiro anochengeterwa vanhu vakuru vemhuri yevanhu vane hemochromatosis, panzvimbo yekuita mazita ekuongorora mazita kune vana vari mumhuri.
Uyewo, kana mubereki mumwe ane hemochromatosis uye mumwe mubereki ane maitiro ekuongorora uye anoonekwa kuti haana kunaka kwemaitiro ehemochromatosis, ipapo mwana angangodaro haafaniri kuongororwa, sezvo achingova mutakuri. Kana mumwe mubereki ari kutakura uye mumwe mubereki haana kunaka, ipapo pane imwe nguva mwana angave akaedza kuti aone kana kwete iye mutakuri.
Mishonga yeHemochromatosis
Mushonga mukuru we hemochromatosis inorapwa phlebotomy, umo murwere ane rimwe ropa rayo (anenge 500 ml) rakabviswa vhiki imwe neimwe. Sezvo zvizhinji zvesimbi mukati momuviri wavo mune ropa ravo, iyi ndiyo nzira yakanaka yekuwana yakawanda yakawanda kunze kwemuviri wavo, iyo inoramba ichiwedzera ropa.
Mimwe mishonga inogona kusanganisira chelation therapy ne deferoxamine uye kugadzirisa yakakwirira-iron, vitamine ane iron, vitamini C (iyo inogona kuwedzera kushandiswa kwesimbi), doro, uye yakasviba shellfish (nemhaka yehutachiona hwehutachiona hwebhakitiriya muhove yakasvibirira) .
Chii Chakuziva NezveHereditary Hemochromatosis
Zvimwe zvinhu zvekuziva nezvehutano hemochromatosis zvinosanganisira izvo:
- Hereditary hemochromatosis inoshandiswa pakugadzirisa zvinokonzerwa nemararamiro evanhu uye inowanzoonekwa kune vanhu vane nhoroondo yekuchamhembe kweEurope.
- Mumwe kubva pa8 kusvika ku12 vanhu ndiye anotakura hemochromatosis, asi sezvo vabereki vose vachifanira kutakura, uye zvino mumwe nomumwe anofanira kupfuudza jeni re hemochromatosis kumwana wavo, ngozi yekuwedzera hemochromatosis inoderera kuvana vazhinji.
- Kana mwana ari pangozi ye hemochromatosis uye iwe unosarudza kuisa maitiro ekuedza kuongorora, saka iwe unogona kufunga nezvekuongororwa ropa nguva dzose (makore maviri kusvika mashanu), kusanganisira serum iron, TIBC, transferrin saturation, uye ferritin level, kuti ave nechokwadi chokuti haasi 't kukura zviratidzo zvesimbi zvakanyanya.
- Mamwe marudzi e hemochromatosis anosanganisira vana vechiHebheru hemochromatosis uye neonatal hemochromatosis.
- Chiropa chinotsvaga kuti chiyero chesimbi chinenge pachiropa chingada kuitwa kana mwana ane kana anofungidzirwa kuti ane hemochromatosis.
Imwe nyanzvi yepamusoro uye / kana gastroenterologist inogona kubatsira kana iwe uchifunga kuti mwana wako ari panjodzi yehemochromatosis.
Sources:
Heeney MM. Iron homeostasis uye zvinokonzerwa nehutachiwana hwehutano hwekunyanya kuwanda: Mhedziso yekuongorora. Hematol Oncol Clin North Am 01-DEC-2004; 18 (6): 1379-403
Hoffman: Hematology: Basic Principles and Practice, 4th ed.
National Human Genome Research Institute. Kudzidza NezveHereditary Hemochromatosis. Yakasvika muna May 2016.
Nelson et al, American Academy yePediatrics Komiti yeBioethics: maonero ehutano nemararamiro ekuongorora vana mukurapa. Pediatrics (2001) 107: pp 1451-1455.