Primary ọrịa sclerosing cholangitis: Mgbaàmà, Diagnosis na Ọgwụgwọ

Ọrịa sclerosing cholangitis - a ọrịa nke imeju opi mgbailu nke ha mgbidi-adịghị ala ala mbufụt amalite. The N'ihi nke ya si malite bụ Filiks sclerosis, m. E. Nnọchi na onya anụ ahụ. Nke a daa ọrịa nwere dịghị causal mmekọrịta ọzọ imeju ọrịa, ma ọtụtụ mgbe na-eduga ná mpụta nke nsogbu si ahụ a. Na taa esonụ, anyị ga-akọwa ihe mere na-emepe emepe ọrịa sclerosing cholangitis. Mgbaàmà na ọgwụgwọ nke ọrịa ga-ga-wetaara gị ntị.

anatomical akwụkwọ

Bile bụ ihe dị mkpa akụrụngwa nke digestive usoro. Ọ na-agụnye mmebi nke abụba, na-enwekwu ọrụ nke pancreatic enzymes na-akpali peristalsis. Bile nọgidere na-emepụta imeju mkpụrụ ndụ - hepatocytes. Kwa ụbọchị dị ka ụbọchị a n'ihi ha kpụ ọkụ n'ọnụ na-arụ ọrụ na-enwetara banyere 1 liter nke mmiri mmiri. Mgbe ahụ, bile abatakwa eriri afo na duodenum.

Outflow secretion haziri site pụrụ iche ducts. Ka ha na-intrahepatic na extrahepatic dabere na ebe. Dị ka a n'ihi nke agaghinihu Filiks, penetration nke pathogens ma ọ bụ ihe ndị ọzọ, na ducts nwere ike ịghọ nanị ire ọkụ. N'otu oge ahụ na-ekwu banyere mmepe nke ọrịa ndị dị ka cholangitis. Enweghị nchịkwa usoro mgbe niile nwere a dị iche iche etiology. Ya mere, ọ na-dịpụrụ adịpụ na-esonụ ụdị: nsí, nje, helminthic, ọrịa sclerosing. Ikpeazụ bụ nnọọ obere, ma, e ji oké.

Ọrịa sclerosing cholangitis kewara abụọ iche: isi na sekọndrị. Onye ọ bụla n'ime ha e ji a kpọmkwem set nke mgbaàmà na N'ezie. Ke akpa idaha ọ pụtara a-adịghị ala ala ọrịa na-na-esonyere mkpọchi nke bile na bụghị purulent mbufụt ducts, mbibi ha na-eji connective anụ ahụ. Secondary ụdị daa ọrịa amalite n'okpuru edinam of bekee. Na obere okwu, ya omume e mere site ezughi oke ọbara ọkọnọ. Ke ibuotikọ emi anyị ga-elekwasị anya na ihe nkowa na isi variant nke ọrịa.

Brief Description nke ọrịa

Primary ọrịa sclerosing cholangitis na nkewa dị ka obere pathologies nke biliary usoro. Dị ka mgbakọ na mwepụ data, ọ na-chọpụtara na otu onye n'ime mmadụ anọ kwa 100 puku bi. Usoro mmepe nke ọrịa na-ebelata ọdịdị mbufụt na obere hepatic ducts. N'ihi ya, ọ a hardening nke ha. Nke nta nke nta yitewere na oruru ọwa site na nke bile aga n'ime afụ. Agaghinihu Filiks emetụta na intercellular ohere nke imeju, na-akpata imeju.

Ọbụna n'oge ọgwụgwụ nke ikpeazụ nke narị afọ na ọ bụ ike ịchọpụta ọrịa nanị mgbe ịwa ahụ ma ọ bụ otopsii. Ekele mmepe nke nkà mmụta ọgwụ taa ọrịa nwere ike achọpụtara ọtụtụ mbụ. Ka ukwuu susceptible ya site ndị ikom dị afọ 25 ruo afọ 40. Ndị a ókè dị nnọọ adabere na ya, n'ihi na ọrịa nwere ike ịbụ asymptomatic maka a ogologo oge. Mgbe ụfọdụ, ihe mgbaàmà nke mbufụt chere maka autoimmune ọrịa, ọnyá afọ na cystic fibrosis.

Na-akpata cholangitis

The kpọmkwem akpata ọrịa ahụ bụ ndị na-amaghị ama. Dọkịta ìgwè dịpụrụ adịpụ nke ihe na-amụbakwa ohere nke ya na omume. Otu n'ime ha bụ:

• mkpụrụ ndụ ihe nketa pụrụ ịrịa;
• Virus ọrụ ke idem;
• propensity ka autoimmune ọrịa;
• n'ebe nsi.

N'ime ihe ndị a na-elebara mkpụrụ ndụ ihe nketa usoro. Gbara ọkpụrụkpụ gosiri na nke a bụ eziokwu bụ ọtụtụ nnyocha nke ọrịa n'etiti ndị òtù ezinụlọ.

adakarị picture

Na ọrịa ruo ọtụtụ afọ nwere ike asymptomatic ma ọ bụ na nke dị nwayọọ mgbaàmà. Ọrịa na-enweghị ike ikwu kpọmkwem mgbe ọ pụtara na-egosi ọrịa sclerosing cholangitis mgbaàmà. Pathology na-emekarị achọpụtara site ohere ịhụ a dọkịta banyere ahụ ike ndị ọzọ nsogbu. Mgbe oge nke nchoputa gosiri ihe àmà mbụ nke ọrịa - nhapụ hepatic enzymes.

Dị ka ọrịa na-aga n'ihu na-adakarị picture mgbanwe. N'etiti ya isi mgbaàmà nke na-esonụ ga-pụta ìhè:

• ike ọgwụgwụ, mgbe nile na-ehi ụra;
• ogbenye agụụ;
• yellowing nke mucous membranes na akpụkpọ;
• azụ okpomọkụ na subfebrile ụkpụrụ;
• igbu mgbu mgbu na nri subcostal ebe, inyịme n'olu ma ọ bụ n'ubu;
• itching;
• multiple xanthomas;
• erughị ala na-ekpe elu quadrant, n'ihi ịmụba ọnụ ọgụgụ nke splin;
• enwekwukwa pigmentation nke anụ.

Mgbe ụfọdụ, isi ọrịa sclerosing cholangitis eso mkpali bowel ọrịa. Ndị a gụnyere ndị ọnyá afọ, Crohn oria.

ụzọ nke nchoputa

Ọ bụrụ na ị na-enyo a ọrịa kwesịrị ozugbo-achọ enyemaka si a dọkịta. Nnyocha nke onye ọrịa na-amalite na-amụ akụkọ ihe mere eme, mkpesa na isi ihe mgbaàmà. Mgbe ahụ, n'ihu anụ ahụ ule. Onye ọrịa nwere ike scratches na akpụkpọ, oké jaundice. Palpation na-na-hụrụ onye na-abawanye nke imeju na-na splin.

Iji gosi na nke mbido nchoputa nke ọrịa zitere maka inwekwu ule. Ọ na-agụnye ndị na-esonụ na-eme:

• ọbara ule (na ngafe nke na-egosi nke leukocytes na ESR egosi mbufụt);
• Abdominal ultrasound;
• elastography imeju (na-enye ohere ka na-eme atụmatụ ahụ ekwedo);
• retrograde cholangiopancreatography (radiography iji iche);
• ọbara n'ihi mmiri ọgwụ (na isi ọrịa sclerosing cholangitis hụrụ oké imeju enzymes);
• MRI;
• Imeju biopsy (usoro a nke nnyocha na-enyere aka ịchọpụta ebe nke fibrosis).

Ndị a ụzọ ka ndị nnyocha e mere na-egosi bụ isi ọrịa sclerosing cholangitis. Diagnosis nke ọrịa na-enyere aka ikpebi ogo-enweghị nchịkwa na usoro. Na ngụkọta e nwere ebe anọ:

1. Portal. Ọ e ji ọdịdị puffiness na hepatic fibrosis ducts.
2. Periportal. Mgbaàmà nke mbụ ogbo na-complemented a ọzọ akpọ fibrosis na mbibi Filiks ducts.
3. Septal. Mgbe a na ogbo nke ọrịa na e nwere na mbụ ihe ịrịba ama nke imeju.
4. Cirrhotic. Ji a na-fledged mmepe nke biliary imeju.

Dabere na ihe mgbagwoju nnyocha dọkịta edepụta ọgwụ.

ọgwụgwọ Iwu

Agwọ na ọrịa a na-iji na-ejide ndị mkpali usoro, na mgbake nke bile eruba na detoxification nke ahu. Iji mezuo nke a, na-achọghị mgbanwe na ịkpa ụzọ nke ọgwụgwọ eji na nkà mmụta ọgwụ ọgbara. Ke akpa idaha pụtara ọgwụ, ma igbaso a siri nri. Surgery na-owụt ke oké ikpe, mgbe mgbanwe ọgwụgwọ ada ada. Nhọrọ nke a akpan akpan usoro ọgwụgwọ bụ ruo dọkịta.

The iji ọgwụ

Dabere na ogo nke ọrịa, o nwere ike e kenyere bed ọzọ na-ekwe ihe ọ bụla mmega. Ọ bụrụ na onye ọrịa na-nchegbu banyere n'oké ihe mgbu, o kenyere antispasmodics ( "No-spa", "Spasmobru").

Iji kwụsị mkpali usoro enyere ndị na-esonụ ọgwụ ọjọọ:

1. Immunosuppressants ( "Azathioprine"). Ha inhibit ọrụ nke dịghịzi usoro.
2. Antifibrogennye pụtara. Ha isi edinam iji na mkpochapu fibrosis, na mgbochi nke ha n'ihu mmepe.
3. Glucocorticosteroid hormone ( "Prednisolone"). Ha na-enyere belata mbufụt.

The ojiji nke ndị ọgwụ na-enye ohere iji merie mbụ ogbo nke mmepe nke bụ isi ọrịa sclerosing cholangitis. Mgbaàmà nke ọrịa na-gbochie ụzọ kwesịrị ndụ nke ndị ọrịa. Itchy anụ ahụ, digestive tract nsogbu na dyspepsia - niile ndị a imebi nwere adịghị mma mmetụta na ahụ ike ha. Ya mere Ọzọkwa kenyere symptomatic ọgwụ. Ọ na-agụnye na-anata hepatic ( "Essentiale"), gastric enzymes ( "Creon") na ọgwụ iji kpochapụ itching. Ọgwụ ọjọọ na-mgbe niile họrọ n'otu n'otu, na-ewere n'ime akaụntụ n'ozuzu adakarị picture na ọrịa na-arịa.

nri Atụmatụ

Ọrịa na-ekenyela ike "table nọmba 5". Mgbe a na-eri ga-amachi oriri nke ọdụdụ, e ghere eghe na-atọkwa ụtọ efere. Animal abụba bụ ịrụ dochie osisi. Ke adianade do, ọ dị mkpa ka kpamkpam kpochapụ si nri achịcha dị iche iche na ato uto, utoojoo mkpụrụ osisi na tomato, chocolate, na mmanya, meats na marinades.

Ikike e nyere ikike iji dabere anụ / azu, ụfọdụ ụdị nke achịcha, porridge na mmiri. I nwekwara ike na-eri mmiri ara ehi na ngwaahịa, mmanụ aṅụ, pasta ofe akwukwo nri efere.

Mgbe nchoputa nke "isi ọrịa sclerosing cholangitis" ọgwụgwọ na ọgwụ na nri na-enye a nti utịp naanị na mbụ ogbo. Ọ bụrụ na nke a oge na-Agbaghara, ọ ga-achọ ịwa ahụ.

ịwa aka

Conservative usoro ọgwụgwọ-eji na-adịghị mgbagwoju shapes nke enweghị nchịkwa usoro. Ọbụna a na-adọ akwụkwọ kwuru na dọkịta ọ bụghị mgbe nile ka a mma n'ihi na omume nke ụdi ọgwụ. Mgbe ọgwụgwọ na ọgwụ ọjọọ eme ihe adịghị eduga normalisation nke ọnọdụ ma ọ bụ ada ada iji weghachi nkịtị ugbu a nke bile, na-amalite ịwa ahụ aka.

Taa dọkịta na-ahọrọ endoscopic arụmọrụ. Ha na-atụ aro na-eme ka ihe niile manipulations site na obere mbepụ ke ikpa. Otú ọ dị, ndị dị otú ahụ na usoro ndị na ọtụtụ ikpe-enye a na-adịghị adịte mmetụta na ndị fraught na nsogbu. Ọzọkwa na-eduzi balloon dilatation na stenting ducts. N'oge usoro, ndị dibịa kọwakwuru na ọwa nke pụrụ iche cylinders na-esetịpụrụ ndị ntupu, na-egbochi ha si ibelata. Ọ bụrụ na e na-oru ọrịa sclerosing cholangitis, imeju transplant ọgwụgwọ pụtara.

kwere omume nsogbu

The ọrịa e ji nwayọọ na-eruba. Ọ na-eme anaghị eme nke ọma ka ọgwụ, na ụba nke systemic ngosipụta nanị ka usoro. Otu n'ime ihe ndị kasị nkịtị nsogbu ya bụ ndị na-esonụ:

1. Portal ọbara mgbali. Nke a daa ọrịa na-esonyere na-abawanye na mgbali na hepatic ọbara. Ya isi pụta ìhè a na-ewere ascites.
2. Cholestasis syndrome. Megide backdrop nke hardening nke opi mgbailu nwayọọ nwayọọ taper na ha cross-isi. Nke a na-akọwa ọdịdị nke jaundice na pruritus. Dị ka progression nke ọrịa lumen asụchikwa ọzọ na ndị ọzọ. Steatorrhea emee, nke na-esonyere osteoporosis.
3. Nje ọrịa sclerosing cholangitis imeju.
4. Adịghị ala ala pancreatitis.
5. Holangiokartsionoma (akpụ opi mgbailu).
6. Cholelithiasis.

Dị otú ahụ nsogbu ime na 3-4 ogbo nke-enweghị nchịkwa na usoro.

Prediction na mgbochi jikoro

Primary ọrịa sclerosing cholangitis na nkewa dị ka nwayọọ nwayọọ na-enwe ọganihu ọrịa. Ya n'ihi na ọtụtụ mgbe bụ ala ala imeju ọdịda. Budata njọ prognosis nká onye ọrịa, ọnụnọ nke concomitant pathologies nke eriri afọ, omume nke nsogbu. A, na ọdịdị nke mbụ ihe ịrịba ama nke ọrịa tupu ikpeazụ ogbo gbatịrị 7 na 12 afọ.

Ọ ga-ekwe omume iji gbochie isi ọrịa sclerosing cholangitis? Nyocha nke dọkịta na-ekwu na n'ihi na ezughi oke amụ kpọmkwem mgbochi nke ọrịa e mepụtara.