I-Hemochromatosis

I-Hemochromatosis yimeko apho kukho i-iron eninzi kakhulu emzimbeni. Ikwabizwa ngokuba kukugcwala kwentsimbi.

I-Hemochromatosis inokuba sisifo semfuza esidluliswa kwiintsapho.

• Abantu abanolu hlobo bathatha isinyithi esininzi ngendlela yabo yokwetyisa. Intsimbi iyakha emzimbeni. Isibindi, intliziyo kunye ne-pancreas zizitho eziqhelekileyo apho i-iron iyakha khona.
• Kukho ekuzalweni, kodwa akunakufunyaniswa iminyaka.

I-Hemochromatosis inokwenzeka kwakhona ngenxa:

• Ezinye iingxaki zegazi, ezinje nge thalassemia okanye ii-anemias ezithile. Utofelo-gazi oluninzi ngokuhamba kwexesha kunokubangela ukugcwala kwentsimbi.
• Ukusetyenziswa kotywala ixesha elide kunye nezinye iimeko zempilo.

Esi sifo sichaphazela amadoda ngakumbi kunabafazi. Kuqhelekile kubantu abamhlophe abavela kumantla eYurophu.

Iimpawu zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Intlungu zesisu
• Ukukhathala, ukungabikho kwamandla, ubuthathaka
• Ubumnyama ngokubanzi bombala wesikhumba (ohlala ubizwa ngokuba yi-bronzing)
• Intlungu edibeneyo
• Ukulahleka kweenwele zomzimba
• Ukuphulukana nomnqweno wesini
• Ukuhla ukusinda

Umboneleli wezempilo uya kwenza uvavanyo lomzimba. Oku kunokubonisa ukudumba kwesibindi kunye nokudakumba, kunye notshintsho kumbala wolusu.

Uvavanyo lwegazi lunokunceda ekwenzeni uxilongo. Uvavanyo lunokubandakanya:

• Inqanaba likaFerritin
• Inqanaba lesinyithi
• Ipesenti yokudluliselwa kwe-transferrin (phezulu)
• Uvavanyo lwemfuzo

Olunye uvavanyo lunokubandakanya:

• Iswekile yegazi (iswekile)
• I-alpha fetoprotein
• I-Echocardiogram yokuvavanya umsebenzi wentliziyo
• I-Electrocardiogram (ECG) ukujonga umsebenzi wombane wentliziyo
• Ukujonga iimvavanyo ezinje nge-CT scan, iMRI, kunye ne-ultrasound
• Uvavanyo lomsebenzi wesibindi

Imeko inokuqinisekiswa nge-biopsy yesibindi okanye kuvavanyo lwemfuza. Ukuba isiphene semfuza siqinisekisiwe, olunye uvavanyo lwegazi lunokusetyenziselwa ukufumanisa ukuba amanye amalungu osapho asemngciphekweni wokugcwala ngentsimbi.

Injongo yonyango kukususa i-iron engaphezulu komzimba kunye nokunyanga nawuphina umonakalo kumzimba.

Inkqubo ebizwa ngokuba yi-phlebotomy yeyona ndlela ilungileyo yokususa i-iron egqithisileyo emzimbeni:

• Isiqingatha selitha segazi siyasuswa emzimbeni veki nganye de iivenkile zentsimbi zomzimba ziphele. Oku kungathatha iinyanga ezininzi ukwenza.
• Emva koko, inkqubo inokwenziwa ngokufuthi ukugcina ukugcinwa kwentsimbi okuqhelekileyo.

Kutheni le nkqubo ifunekayo ixhomekeke kwiimpawu zakho kunye namanqanaba e-hemoglobin kunye ne-serum ferritin kunye nokuba ingakanani na intsimbi oyithathayo ekutyeni

Ezinye iingxaki zempilo ezinjengesifo seswekile, ukwehla kwamanqanaba e testosterone emadodeni, isifo samathambo, ukusilela kwesibindi kunye nokusilela kwentliziyo kuya kunyangwa.

Ukuba ufumene ukuba une-hemochromatosis, umboneleli wakho unokucebisa ukutya ukuze kuncitshiswe ukuba ingakanani na intsimbi efunxwe kwitroke yakho yokugaya ukutya. Umboneleli wakho unokucebisa oku kulandelayo:

• Musa ukusela utywala, ngakumbi ukuba unokonakala kwesibindi.
• Musa ukuthatha iipilisi zentsimbi okanye iivithamini ezinesinyithi.
• Sukusebenzisa izixhobo zokupheka zentsimbi.
• Ukunciphisa ukutya okuqiniswe ngetsimbi, njenge-100% yeesiriyali ezinqabileyo zentsimbi.

Ukunganyangwa, ukugcwala kwentsimbi kunokukhokelela kumonakalo wesibindi.

Isinyithi esongezelelekileyo sinokwakha nakwezinye iindawo zomzimba, kubandakanya idlala lengqula, amasende, ipancreas, idlala lengqula, intliziyo okanye amalungu. Unyango lwakwangoko lunokunceda ukuthintela iingxaki ezifana nesifo sesibindi, isifo sentliziyo, isifo samathambo okanye isifo seswekile.

Wenza kakuhle kangakanani kuxhomekeke kubungakanani bomonakalo womzimba. Olunye ulwaphulo lomzimba lunokuguqulwa xa i-hemochromatosis ifunyenwe kwangoko kwaye iphathwa gadalala nge-phlebotomy.

Iingxaki zibandakanya:

• Isibindi sokuqina
• Ukungaphumeleli kwesibindi
• Umhlaza wesibindi

Esi sifo singakhokelela kuphuhliso lwe:

• Isifo samathambo
• Seswekile
• Iingxaki zentliziyo
• Ukwanda komngcipheko kusulelo oluthile lwebhakteria
• Uvavanyo lwe-atrophy
• Utshintsho kumbala wolusu

Tsalela umnikezeli wakho ukuba iimpawu ze-hemochromatosis ziyaphuhliswa.

Tsalela umnxeba ukuze ubonane nomnikezeli wakho (ukuze kuhlolwe) ukuba ilungu losapho lifunyenwe line-hemochromatosis.

Ukuvavanywa kwamalungu osapho omntu ofunyenwe ene-hemochromatosis kunokubhaqa esi sifo kwangoko ukuze kuqalwe unyango ngaphambi kokuba kwenzeke umonakalo kwizalamane ezichaphazelekayo.

Ukugcwala kwentsimbi; Utofelo-gazi - hemochromatosis

• I-hepatomegaly

I-Bacon BR, iFleming RE. I-Hemochromatosis. Ku: UFeldman M, uFriedman LS, uBrandt LJ, ii-eds. Sleisenger kunye neFordtran's Amathumbu kunye nezifo zesibindi: iPathophysiology / Diagnosis / Management. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2021: isahluko 75.

IBrittenham GM. Ukuphazamiseka kwentsimbi ye-homeostasis: ukunqongophala kwesinyithi kunye nokugcwala kakhulu. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 36.