Dubin-Johnson syndrome: whakaahuatanga, te tātaritanga, tohu me te maimoatanga

Ahakoa te meka i hanga e kua rongoā hou nui te ahunga whakamua, me te maha mate he kore te kupu mo nga tūroro, e kore e matau ano tino etahi o enei mate. Ko tētahi o ratou ko te syndrome Dubin-Johnson. Te whare hauora, te tātaritanga me te maimoatanga o tenei mate, notemea ki te ngaio hauora o etahi moni. Whakaaroa tenei pātai i roto i te taipitopito atu.

Rotor syndrome me Dubin-Johnson syndrome - he onge tuku mate ate rite jaundice tūhonohono.

take o

Dubin-Johnson syndrome hoki he ingoa tetahi: enzimopaticheskaya jaundice ate. whakatika te reira i roto i te hononga ki te ofatiraa o te tuku ki te ate i te pūtau ate o bilirubin.

puta tēnei rauemi i roto i te tinana mo te tango reira pakaru rawa ngaro ranei erythrocytes. puta Opaniraa bilirubin i roto i ara e rua:

• te whakanui ake i te nui o te taonga mai i roto i te ate;
• moni iti o te mimi ki te haere.

puta Dubin-Johnson syndrome ka fakahohaasi waka o bilirubin mā te ate. Ko te mate tino onge i haria ki atu taitamariki tangata (e pā ana ki 20-30 tau) tenei. take o te mate mai whanau ētahi wā puta. He iti te mōrea o te mate i muri i te tau o te 50 tau tangata pakeke ranei.

Ko te take ira o te syndrome, Dubin-Johnson he tika ki te irakētanga ira, whai wāhi i roto i te kaweake o bilirubin i roto i te ate, me te whakawaehere pūmua.

tohu

Dubin-Johnson syndrome, tohu o nei he āhuatanga o ētahi atu mate pērā i, mo te tauira, te rohirohi, a ka he tona ake āhuatanga motuhake:

• mamae i roto i te rara i te taha ki matau;
• he kore tino o te hiahia;
• reira kia kia minamina iti o te kiri;
• ate rahi;
• colic, e te tahi mau taime faarahi;
• ruaki;
• hua pāmahana.

koe'uhi ko Faaiteraa tika ki ngā mate hopuhopu, kikī nui tinana, me te aronganui. No te wahine, whakahōhā taea i muri i te horomanga o te whakawhānau.

rōpū mōrearea

Ko tēhea rōpū o te iwi kia tūpato o te syndrome?

He tuku te mate. Ki te i te whānau tamariki ki tenei mate, he tika te rua i te wā o te whakamahere o hapūtanga ki tika i te whakamātautau katoa, haere i te whakamātautau e tika ana, me te kōrero ki te mātanga. He hāngai hoki aua faaipoipo aua tūtohutanga, tae atu tetahi o nga hoa faaipoipo ratou syndrome Dubin-Johnson kua tukua whanaunga tata ranei.

Ko te tukanga tātaritanga

Fakasiokālafi, Ko te nuinga noa i roto i nga Hurai Iranian te mate. Ratou tau tae 70% o te katoa taimaha o nga tūroro turoro.

He mea nui kia tata kahore Dubin-Johnson syndrome ora-te whakawehi. tūroro TAUIRA fakapapau'i tenei. korenga i Doctors te tümanako ora o tūroro ki tenei mate, me mutu e kore e arahi i te reira ki te mate.

Mō 25% o te tūroro wheako mamae i roto i te hypochondrium tika. Ko te hanganga o te ate e kore pānga, i reira he kahore deformation i te syndrome. Otiia i te wa ano i roto i ona kopa puta wahi mangu, hua i roto i te tinana e taui tona tae ki te parauri matomato pouri ranei. huaina tēnei āhuatanga o te syndrome te pihikete tiakarete kongakonga. tutuki ana tēnei pānga e te secretion pakaru metabolites.

Ko te mate he waitohunga pai, ahakoa e taea tonu te reira puta noa te ora o te manawanui.

Ko te ripanga i raro e whakaata ana te raraunga i runga i te mana o nga whekau o tūroro.

tātaringa

Dubin-Johnson syndrome, te tātaritanga o e e wahi i roto i te maha o ngā wāhanga, whakaritea e te tätari.

• Ko reira whakahauanga ki ultrasound o te puku.
• toto General me mimi whakamātautau.
• Tātaritanga o toto me te mimi mo bilirubin. Ka rite ki te tikanga, he tiketike rawa ratou.
• Tauira fenobarbetalom.
• Tohu o te kakā.
• bromsulfaleinova Tauira.
• uara Coproporphyrin o tohu i roto i te mimi ia ra.
• Ko te ihirangi o te whākōkī i roto i te toto.

Hoatu etahi atu tikanga o te tātaritanga.

I roto i te aroaro o te tūroro syndrome Dubin-Johnson i ki te haere ki te kaihaumanu.

Treatment me te ārai

Dubin-Johnson syndrome, e kore e maimoatanga whai wāhi te fariiraa i te mau faaineineraa motuhake e whakahaeretia ana e āraihia:

• pipiki ki te kai tino, te whakamahi o te huaora B, kore-korotutu;
• whakaiti i roto i te āhuatanga ahotea;
• whakaiti o te mahi whakakori tinana;
• kore o inu 'olokaholó;
• aroturuki i ia te wā o ngā taumata bilirubin mo te ora o te ora o te manawanui.

Ko reira tika hoki ki te mahara kia exacerbate te mate e hapūtanga. Drugs ki te āwhina te whakaora i te syndrome Dubin-Johnson, tēnei wā e kore e whakawhanakehia.

Waihoki, ko te āhua o te mate, ko te rotor syndrome.

whakamārama poto o

Ko hoki rotor syndrome he mate tuku e pānga te ate. huringa whakamamaetia i roto i te tuku o te bilirubin, noho noa mahi whākōkī. Ka taea e tatou te mea e, te mate ko te ahua ono tekau o Dubin-Johnson syndrome whakaahuatia i runga. hoki faaite te iti bit ngawari tohumate.

Ka taea e te mate faaite mai rite i whanau, me te tamarikitanga. Bilirubin i roto i te toto o te manawanui e taea e noa, me te here. He he he rerekē o te gallbladder.

āhuatanga rotor syndrome e te meka e i roto i hepatocytes kahore ihirangi o ngā uhi parauri. Ehara i reira ko te pānga, i mohiotia te rite pihikete tiakarete kongakonga. Ko paku rerekē i Dubin-Johnson syndrome te take o te mate. I roto i tenei take, e kore e taea e te ate akakoromaki i te absorption o bilirubin. Ka rite ki te hua, kore pukupuku taonga hoki ki te toto. I tenei kaupapa, i roto i te manawanui whakawhanake giperbulemiya. waitohunga pai o te mate.

I te wa o te tātaritanga lidofreninom ate, au tōngāmimi me aerodynamics e kitea.

tohu syndrome

Ko te tino rite ki te tohu o te syndrome Dubin-Johnson Signs rotor syndrome:

• Kakato faaite jaundice.
• Ko te mana'o o te kawa i roto i te mangai.
• Mamae i roto i te hypochondrium tika.
• A, no te Dubin-Johnson syndrome, nui haere tūroro ate i roto i te rahi. I roto i te take o rotor syndrome nui haere te anake i roto i ētahi wā.
• taumata teitei o te bilirubin i te toto.
• He noa whakamātautau mahi ate.
• whakamātautau toto, kahore huringa.
• Ko te bioptome ate he kahore haaputuputuraa o uhi.

te tikanga pai waitohunga, puta te syndrome mo nga tau maha, e kore tä rawa hauora tangata.

tātaritanga o te syndrome

taea anake te whakaturia te mate i roto i te tātaritanga whānui o te rauropi.

Akoranga whakahaere i roto i te taiwhanga ki te āwhina i whakakitea he ihirangi nui ake o bilirubin i te toto. Hoki te tātaritanga he mea nui ki te mohio te maha o coproporphyrin roto i te mimi.

I roto i te nuinga o ngā tūroro, kauneke te mate, kahore tetahi tohumate.

I roto i te take o te tātaritanga o rotor syndrome noho whakamātautau ate: tona X-ray, me te iratuki. I roto i te tātari taua mamae au tōngāmimi me aerodynamics.

ārai mate

He āraihia General e whai ake:

• whakaiti o te mahi whakakori tinana;
• maimoatanga o mate e pā ana rotor syndrome;
• A ape i te ahotea me te ahotea aronganui.

Āhuatanga o te hua o te bilirubin i roto i te toto

piki Bilirubin mo nga take e whai ake nei:

• ahotea nui;
• kai e hawata ana, kai takahi;
• te whakamahi o te raau taero;
• whakamahi nui o te waipiro.

maimoatanga o te mate

tikanga noa ki te whakakore i te mate ki reira. Heoi, me ū te reira ki āraihia. Ka rite ki nga taero e hiahia ana koe ki te tango i te tikanga o taua putanga bilirubin i te tinana. Ko maha ate e whakamahia taero.

Kōwhiritia te maimoatanga tika ki te āwhina i te tākuta.