Gịnị bụ hemochromatosis ma olee otu esi agwọ ya?

Hemochromatosis, makwaara dị ka ọrịa shuga nchara, bụ ọrịa na-adịghị ahụkebe nke e jikọtara ya na nnukwu mmebi nke metabolism iron n’ahụ mmadụ.

Site na usoro ọmụmụ a, a na-etinye akụkụ ihe a na-emetụta oke na eriri afọ wee mejupụta akụkụ ahụ na akwara, na-ebute oke ibu.

N'ihi ya, imeju na-emebi, ihe mgbu na nkwonkwo na-apụta, ụcha akpụkpọ ahụ na-agbanwe, ọdịmma onye ọrịa na-akawanye njọ. Enweghi ike ịkpọ ọrịa a ka ọ bụrụ ihe a na-ahụkarị, yabụ, ndị bu ụzọ nụ nchoputa nke hemochromatosis adịghị aghọta ihe ọ bụ. Isiokwu ahụ kọwara n’ụzọ zuru ezu ihe na-akpata, mgbaàmà na ụzọ usoro a na-ejikarị nchọpụta, ọgwụgwọ nke ọrịa a.

Njirimara nke oria

Mara ọdịiche dị n'etiti hemochromatosis nke mbụ na nke abụọ. Ejikọtara nke mbụ na mmụpụta sitere na mkpụrụ ndụ ihe nketa nke dị na chromosome nke isii, ya bụ, ihe eketa eketa. Ọ na - aputa ihe dị ka mmadụ atọ n’ime otu puku, ugboro ugboro n’ime ụmụ nwoke. A na-egosipụta ọbara hemochromatosis n'oge okenye, ọ na-abụkarị afọ 40-60, na mgbe ụmụ nwanyị mechara.

Hemochromatosis nke imeju

Secondary hemochromatosis bụ ihe ndị dị na mpụta na - akpata ya ma enwere ike ịchọpụta ị ofụ oke n'ahụ anyị, dịka ọmụmaatụ, site na iji usoro ọgwụgwọ ígwè eme ihe ma ọ bụ n'ihi mmịnye ọbara ugboro ugboro. Mgbe ụfọdụ, ọ nwere ike ịmalite dị ka ihe mgbagwoju anya nke ọrịa ọbara nke na-eme ka metabolism iron dị na ya.

Dika odi, aru nwanyi nwere 300-1000 mg nke iron, ndi nwoke - ihe dika 500-1500 mg. Ọtụtụ n'ime ya bụ akụkụ nke haemoglobin, a na-ahụkwa ihe ntụgharị enzymes na pigments. N'ime ígwè a na-eweta nri, ọ bụ naanị otu ụzọ n'ụzọ iri, 1-1.5 mg, na-eburu ya ọnụ, yana hamochromatosis a na-abawanye ruo 3-4 mg kwa ụbọchị.

Ọ tụgharịrị na ihe karịrị otu afọ ka gram dum nke anụ ahụ na - agbakọ n’ahụ onye ọrịa, a na-edebe ya na imeju, obi, ntụrụndụ, pancreas, wdg.

N'oge na-adịghị anya, onye ọrịa ahụ butere ọrịa cirrhosis, na 30% rite ọrịa kansa imeju. Nri na - ata ahuhu, nke n’eduba na ebute uto nke oria shuga.

N'ihi malfunctions na pituitary gland, ọrịa na arụ ọrụ nke usoro omumu na-egosipụta: enweghi ike na-etolite n'ime ụmụ nwoke, testicles atrophy, ara nwere ike ịmalite itolite, n'ime ụmụ nwanyị, a na-agbagha okirikiri, libido na-ebelata. Mbibi nke obi na-eduga n'ọgba aghara, na, n'ụzọ ụfọdụ, nkụda obi.

Ọrịa

Ihe mgbaàmà kachasị nke hemochromatosis:

• ike ọgwụgwụ, adịghị ike;
• ifelata
• mgbali elu
• ụyọkọ akwara mucous, sclera;
• ọzịza nke nkwonkwo ahụ, na-akawanye njọ nke ije ha;
• mbelata libido;
• na agbaji akpukpo ahu (o na enweta ọla ma o bu isi awọ).

Ọzọkwa, mmalite nke ọrịa shuga, ọrịa imeju, mmepe nke obi na ọrịa imeju nwere ike ịpụta na ihe ịrịba ama nke ọrịa.

Ihe nrịba ama kachasị egosi, bụ ọdịdị nke ụda akpụkpọ ahụ. A na-enyochakarị pigmentị n'aka, ihu, ụkwụ, olu na akụkụ ahụ. Akpukpo anụ ahụ na aka aka ya ka dị ọkụ.

Ihe kpatara

Isi ihe kpatara ya bu ihe ketara eketa, ọ bụ ya na-akpata hemochromatosis.

Banyere nke sekọndrị, ndị ọkachamara na-achọpụta ihe ndị na-akpata ya:

• ọrịa ịba ọcha n'anya nke na-adịghị ala ala B, C;
• idobere anụ ahụ adipose na imeju - steatohepatitis na-anaghị egbu egbu;
• imeju afọ;
• mgbochi nke akwara ngwo;
• ịwa ahụ portocaval, nke mere mmebi nke metabolism iron;
• ị ofụ ọgwụ ọ -ụ ironụ na - ejideghi;
• leukemia.

Diagnostics

Onye na-ahụ maka eriri afọ ma ọ bụ endocrinologist nwere ike ikpebi hemochromatosis (koodu nchọpụta dabere na nhazi mba dị ka ọrịa E83.1) dabere na isi ihe ndị a:

• njiri mara nke oria a;
• akụkọ banyere ezi na ụlọ, ya bụ, ọnụnọ nke ezigbo ndị ikwu ụdị nsogbu a;
• Nsonaazụ nyochara nke ọma nke ihe igwe dị n’usoro ya na ụdị ya dị iche iche n’ime ọbara;
• data nke bioped anara nke mere na imeju, na-egosi idobe iron n’ahụ n’ime sel nke akụkụ a.

Ọzọkwa, iji chọpụta ọrịa haemochromatosis, enwere ike kpebie ọnụnọ nke mmụkọ ugboro ugboro na mkpụrụ ndụ ihe nketa HFE nke na-ebute ọrịa a eketa eketa site na nyocha nke ịkọ mkpụrụ ndụ ihe nketa.

Ọ bụrụ n’inwe obi ọnụnọ nke hemochromatosis, dọkịta ahụ ga-eduzi gị ka ị were nyocha ọbara, biochemical ọbara, yana nnwale ọbara maka ọdịnaya shuga, ị ga-achọ nyocha nke ultrasound nke akụkụ dị iche iche nke oghere afọ, scintigraphy imeju.

Na mgbakwunye, ọ dị mkpa ịchọpụta kpọmkwem ebe nkwanye ụrọ ahụ dị n'oge hemochromatosis na akụkụ ndị ọ kacha emetụta, yabụ ndị dọkịta na-atụ aro ka ime ihe ọmụmụ na electrocardiography, imeju biopsy, na nyocha redio nke nkwonkwo.

Ọgwụgwọ

A na-eme ọgwụgwọ nke ọrịa a. Nke mbu, a na-enye onye ọrịa ahụ ọgwụ ndị na - ejikọ iron (Deferoxamine, Desferal, Desferin), nke gụnyere ihe ndị na - arụ ọrụ na - arụpụta ihe mgbagwoju anya na iron nke ewepụrụ n'ahụ ahụ. Oge ọrụ nchịkwa dị ihe dịka ụbọchị 20-40.

Ọchịchọ

Ọ dịkwa mkpa ịgbaso nri pụrụ iche: belata oriri nke ngwaahịa niile nwere iron na nnukwu oke (anụ ehi, pọmigranet, imeju, buckwheat, wdg), kpochapụ mmanya, belata oriri nke ascorbic acid n'ime ahụ onye ọrịa.

Ọtụtụ mgbe, a na-edobe ọbara n'ọbara site na phlebotomy - mgbidi ọnya ma ọ bụ akwara ndị ọzọ kwesịrị ekwesị ka a na-ekesa obere oge iji wepu otu ọbara (200-400 ml), n'ihi nke ọdịnaya iron na-ebelata, ọnọdụ onye ọrịa ahụ na-akawanye mma, na ụcha akpụkpọ ahụ na-ebelata.

A na - eme usoro a 1-2 ugboro n'izu maka afọ 2-3, mgbe ahụ, ugboro ole na - agbada, a na - ebu ọbara naanị mgbe ụfọdụ iji nọgide na - enwe ezigbo ọnụọgụ nke anụ ahụ.

N'ime usoro ndị a, a na-enyocha ọkwa nke haemoglobin, ferritin, ọbara hematocrit, na ụba nke oke ngafe. O kwere omume ka a na-agba ọbara ruo mgbe onye ọrịa nwere ọrịa anaemia.

N'ọnọdụ ụfọdụ, a na-eji cytapheresis - wepu akụkụ cellulite nke ọbara na iweghachite autoplasma zuru ezu. N'otu oge ahụ, a na-ewepụ akụkụ nke mkpụrụ ndụ ọbara uhie, nke nwere ihe ka ukwuu n'ọbara ahụ, a na-ahụkwa nsonye ahụ, n'ihi nke a na - ebelata nbibi nke usoro dị iche iche na - akpata mbufụt.

Nsochi

Arrhythmia na-egosipụtakarị, mgbe ụfọdụ nkụda obi, ya bụ, nnukwu mmebi nke akwara obi, n'ihi oke ahụ na-egbochi ahụ, ọrịa na-efe efe na-abawanye ugboro ugboro.

Nsonaazụ kachasị njọ nke hemochromatosis bụ ọrịa myocardial infarction, ọbara ọgbụgba na mucous nke mkpụrụ afọ, akpịrị na-arịa ọnya afọ, yana akpụ.

Mgbochi nke Mkpakọ

Na ọnụnọ isi haemochromatosis, ọ dị mkpa ịgbaso nri nwere mgbochi nke ngwaahịa nwere iron, ascorbic acid, yana nri nwere protein. Na mgbakwunye, a ga-emerịrị ọgwụ ndị a na-ejikọ ígwè.

Vidiyo ndị metụtara

Ewepụghị n'ihe nkiri televishọn bụ́ “Ahụike Dị Ndụ!” banyere ihe hemochromatosis bụ na otu esi emeso ya:

Dịka ị siri hụ, ọrịa a enweghị ike ọfụma, ọgwụgwọ kwesịrị ịmalite ozugbo. Nnukwu ígwè dị n’ahụ nwere mmetụta na-emebi ihe n’arụ na akwara dị iche iche. Ọ bụrụ na ị na-enwe mgbaàmà nchekasị, kpọtụrụ onye ọkachamara endocrinologist ma ọ bụ ọkachamara banyere gastroenterologist ozugbo.