Yãfẽwa hyperbilirubinemia: bincike da kuma magance

Yellowing na mucous membranes, ido sclera da kuma fata ya kamata a sanar da wani mutum. Kowa ya san cewa wadannan cututtuka nuna wasu tafka magudi a aikin na irin wannan muhimmanci jiki kamar hanta. Wadannan ailments kamata ko da yaushe a iya sarrafawa da wani likita. Ya zai sa a daidai ganewar asali da kuma rubũta da zama dole magani. A mafi girma matakan da bilirubin yawanci bayyana yellowness. Yãfẽwa hyperbilirubinemia ma yana da irin wannan bayyanar cututtuka. A labarin da bincika a more daki-daki abin da wannan cuta ne, abin da suke ta haddasawa da kuma magani.

Definition na kirki hyperbilirubinemia

A ainihi bilirubin - a bile pigment, shi yana da halayyar m-yellow launi. Samar da wannan abu a haemoglobin daga erythrocytes wanda rarrabu saboda wani involutive canje-canje na hanta Kwayoyin, saifa, connective nama, da kuma bargo.

Yãfẽwa hyperbilirubinemia ne m cuta ya hada da pigment steatosis, sauki Holem iyali, na yara intermittent jaundice, wadanda ba hemolytic jaundice iyali, tsarin mulki hanta tabarbarewa, aikin distention jaundice da hyperbilirubinemia. Cutar da ake bayyana promezhayuscheysya ko na kullum obstructive jaundice, bayyana mutum hanta da kuma tsarinta ba tare da gagarumin disturbances. Babu kuma a lokaci guda a bayyane yake bayyanar cututtuka na cholestasis kuma Ya ƙãra hemolysis.

Yãfẽwa hyperbilirubinemia (code ICD 10: E 80 - Janar cuta da kuma porphine bilirubin metabolism) kuma yana da wadannan lambobin E 80,4 E 80,5 E 80.6 E 80. Haka shigar wanda ke aiki: Gilbert ciwo, Crigler syndromes, sauran cuta - dubin-Johnson ciwo na'ura mai juyi da kuma syndromes, unadjusted take hakkin bilirubin metabolism.

Sanadin

Yãfẽwa hyperbilirubinemia a manya a mafi yawan lokuta - An cutar da iyali hali, suna daukar kwayar cutar ta hanyar rinjaye irin. Wannan ya tabbatar da likita yi.

Akwai postgepatitnaya hyperbilirubinemia - taso a matsayin sakamako na kwayar m hepatitis. Shi ne ma dalilin da cutar na iya zama da cututtuka mononucleosis, bayan dawo da marasa lafiya na iya fuskanci bayyanar cututtuka na hyperbilirubinemia.

Hanyar da cuta ne mai gajiyarwa, a musayar bilirubin. A magani daga abu ƙaruwa, akwai wani hargitsi ko kama ko canja wurin shi a cikin hanta daga jini Kwayoyin.

Irin wannan jiha ne zai yiwu a kuma waɗannan lokuta idan akwai wani hargitsi a cikin Daure bilirubin da glyukuropovoy acid, shi ne m m ko wucin gadi ƙaranci daga wani enzyme kamar glyukuroniltrapsferaza.
Wadannan hanyoyi kuwa sune halin da hyperbilirubinemia Gilbert ciwo, Crigler-Najjar, kuma postgepatituyu hyperbilirubinemia. Na'ura mai juyi syndromes da dubin-Johnson magani bilirubin ƙaruwa saboda disturbances ekskrektsii pigment a cikin bile canaliculi ta cikin hepatocyte membrane.

m dalilai

Yãfẽwa hyperbilirubinemia, ganewar asali aka tabbatar da cewa sau da yawa shi ne gano a cikin samartaka, za a iya bayyana su bayyanar cututtuka na shekaru masu yawa har ma a rayuwa. A maza, wannan cuta da aka gano yawa akai akai fiye da mata.

The classic bayyanuwar da cutar - yellowing na sclera, icteric launi daga cikin fata na iya faruwa a cikin mutum lokuta, ba ko da yaushe. Bayyanuwar hyperbilirubinemia ake intermittent sau da yawa, a rare lokuta, ne m, kada ka wuce.

Ƙarfafa jaundice iya tsokana da wadannan dalilai:

• furta a zahiri ko juyayi sha.
• exacerbation na kamuwa da cuta, biliary fili rauni.
• Rigakafi kwayoyi.
• colds.
• Daban-daban m hanyoyin.
• barasa ci.

cututtuka

Sai dai cewa na sclera da fata ne fentin a yellow launi, marasa lafiya ji nauyi a cikin dama babba quadrant. Akwai sau idan gaji da damuwa da dyspepsia - wani tashin zuciya da amai, illa stool, rashin ci, ta ƙara flatulence a cikin hanji.

Bayyananen hyperbilirubinemia iya kai ga bayyanar astenovegetativnogo cuta wadda bayyana a matsayin ciki, gajiya, m gajiya.
A jarrabawa, likita da fari yana biya da hankali ga yellowing na sclera da wani Matte-yellow tinge da fata na haƙuri. A wasu lokuta, da fata ba juya rawaya. Hanta palpated a gefen costal baka, kuma ba za a iya ji. Sai ya faru wani kadan karuwa a jiki size, da hanta zama taushi da kuma haƙuri da palpation mai raɗaɗi ne. The baƙin ciki ba girma a cikin size. Ware ne a lokacin da benign hyperbilirubinemia auku a matsayin sakamako na hepatitis cututtuka. Postgepatitnaya hyperbilirubinemia kuma iya faruwa bayan cututtuka - mononucleosis.

Syndrome benign hyperbilirubinemia

Domin benign hyperbilirubinemia hada da bakwai nakasar syndromes a cikin likita yi:

• Crigler-Najjar syndromes, irin 1 da 2.
• Dubin-Johnson syndromes.
• Gilbert ciwo.
• Na'ura mai juyi syndromes.
• Baylera cuta (m).
• Lucy-Driscoll ciwo (m).
• Family shekaru benign cholestasis - a benign hyperbilirubinemia (m).

Dukan waɗannan syndromes faruwa saboda da metabolism na bilirubin, idan matakin unconjugated bilirubin a cikin jini yana ta ƙara, wanda accumulates a cikin kyallen takarda. Tasrifu na bilirubin taka muhimmiyar rawa a cikin jiki, da wani sosai mai guba bilirubin ne tuba zuwa cikin low-mai guba a diglyukoronid - narkewa fili (conjugated bilirubin). Free form bilirubin sauƙi ratsa cikin na roba masana'anta wanda aka kiyaye shi a cikin mucous membranes, fata a kan garun jini, ta haka ya haddasa jaundice.

Crigler-Najjar ciwo

Amurka pediatricians Nayyar V. da J .. Kriegler a shekara ta 1952 ya gano wani sabon ciwo kuma aka bayyana shi. Ya aka kira Crigler-Najjar ciwo irin 1. Wannan nakasar cuta yana da wani autosomal retsensivny gādo. A ci gaba da ciwo ya auku nan da nan a cikin ta farko hours bayan haihuwa. Akwai irin wannan bayyanar cututtuka daidai sau da yawa biyu 'yan mata da yara maza.

A pathogenesis da cutar ne ya sa ta da cikakken rashin wani enzyme kamar UDFGT (enzyme urndin-5-difosfatglyukuroniltransferazy). Lokacin da wannan ciwo irin 1 UDFGT fakowa ba gaba daya, free bilirubin ƙaruwa sharply, Manuniya isa 200 mol / L, har ma fiye. Bayan haihuwar rana ta farko da wani babban permeability p. Blood-kwakwalwa shãmaki. A cikin kwakwalwa (m al'amari), akwai wani m jari na pigment rawaya tasowa kernicterus. Kordiamin gwajin tare da ganewar asali na kirki hyperbilirubinemia, phenobarbital - korau.

Bilirubin encephalopathy take kaiwa zuwa cin gaban nystagmus, tsoka hypertonia, athetosis, opisthotonos, clonic da tonic convulsions. A hangen nesa ne musamman talakawa. A rashin m magani a rana ta farko yiwu m. A hanta ne ba ya canja a autopsy.

Dubin-Johnson ciwo

Syndrome benign hyperbilirubinemia dubin-Johnson aka farko aka bayyana a cikin 1954. Mafi yawa da cutar ne na kowa daga mazauna yankin gabas ta tsakiya. Men karkashin yana da shekaru 25 shekaru da aka samu a 0.2-1% na lokuta. Gādo auku a wani autosomal rinjaye hanya. Wannan ciwo yana da pathogenesis na wanda ake dangantawa da disturbances a kai ayyuka na hepatocyte bilirubin kuma daga gare ta saboda savanin kai ATP-dogara membrane cell tsarin. Kamar yadda wani sakamako, bayarwa na bilirubin a bile kakkarye, akwai wani reflux na bilirubin a cikin jini na hepatocyte. Wannan ya tabbatar da ganiya na fenti maida hankali a cikin jini da sa'o'i biyu a lokacin da fitina ta amfani da bromsulfaleina.

Morphological halayyar alama - da cakulan-canza launin hanta, inda babban jari na m granular pigment. Bayyananen na ciwo: yellowness m ko intermittent itching, zafi a gefen dama a sama quadrant, asthenic bayyanar cututtuka, dyspepsia, kara girma daga cikin baƙin ciki, kuma hanta. Farko da cutar na iya zama a kowane zamani. Akwai hadarin bayan dogon lokaci da yin amfani da maganin hana haifuwa hormonal jamiái, kazalika a lokacin daukar ciki.

Diagnosing cutar tushen bromsulfaleinovoy samfurin a holetsistografii a lokacin jinkirin tukar tumbi cikin bile bambanci wakili a cikin rashi na batawa a cikin gallbladder. Kordiamin a diagnosing benign hyperbilirubinemia a cikin wannan hali ba a amfani da su.

Total bilirubin index ba ya wuce 100 mol / l, free kuma conjugated bilirubin rabo ne 50/50.

Jiyya na ciwo ba a ci gaba. Rayuwa Hasashen ciwo ne ba ya canzawa, amma ingancin zama a cikin wannan Pathology worsens.

Yãfẽwa hyperbilirubinemia - Gilbert ta ciwo

Wannan hereditary cuta ne ya fi kowa, gaya game da shi a more daki-daki. Cuta daukar kwayar cutar daga iyaye zuwa 'ya'ya da ake dangantawa da wani lahani na gene, wanda aka hannu a cikin metabolism na bilirubin. Yãfẽwa hyperbilirubinemia (ICD - 10 - E80.4) - bã kõme ba ne kamar yadda Gilbert ta ciwo.

Bilirubin - daya daga cikin mafi muhimmanci bile pigments matsakaici rugujewar haemoglobin wanda aka hannu a cikin harkokin sufuri na oxygen.

Karuwan bilirubin matakan (80-100 micromol / L), wani gagarumin preponderance na ba-jini gina jiki (kai tsaye ba) bilirubin take kaiwa zuwa lokaci-lokaci bayyananen jaundice (mucous membranes, sclera, fata). A wannan yanayin, hanta aiki gwaje-gwaje, da sauran Manuniya ne na al'ada. A maza, Gilbert ta ciwo ne 2-3 sau fiye da na kowa, fiye da mata. Domin da farko lokacin da zai iya bayyana tsakanin zamanai na uku zuwa goma sha uku shekaru. Sau da yawa cutar da ake tare da duk rayuwar dan Adam.
By Gilbert ciwo ne fermentopaticheskie benign hyperbilirubinemia (pigment gepatozy). Akwai yawanci saboda kai tsaye ba (unconjugated) sulusi da murabba'i bile pigments. Wannan shi ne saboda kwayoyin lahani na hanta. Da ya faru na kirki - babban canje-canje a cikin hanta, ya nuna hemolysis faruwa.

Bayyanar cututtuka na Gilbert ta ciwo

Gilbert ta ciwo ba shi da furta cututtuka, na faruwa tare da kadan bayyanar cututtuka. Wasu likitoci ba su yi la'akari da ciwo a matsayin wata cuta, da kuma mayar da shi zuwa ga physiological halaye da kwayoyin.

The kawai bayyanuwar a mafi yawan lokuta suna m jaundice batawa yi na mucous membranes, fata, ido sclera. Sauran cututtuka ko m, ko ba faruwa a duk.
Mafi qarancin zai yiwu neurological cututtuka:

• rauni.
• dizziness.
• gajiya.
• barci cuta.
• rashin barci.

Ko da more rare bayyanar cututtuka na Gilbert ta ciwo - a dyspepsia (bacin)

• rashi ko raguwa na ci;
• m belching bayan cin;
• ƙwannafi.
• m aftertaste a bakin. da wuya amai, tashin zuciya.
• a ji na nauyi, cikar a ciki.
• stool munanan (zawo ko maƙarƙashiya).
• zafi a dama babba quadrant maras ban sha'awa aching hali. Na iya faruwa a lokacin da kurakurai a cikin abinci, da zagi na m, kuma m abinci.
• akwai iya zama wani karuwa a cikin hanta.

Yãfẽwa hyperbilirubinemia: magani

Idan akwai wani Associated cututtuka na gastrointestinal fili a gafarta musu likita nada rage cin abinci №15. A cikin m lokaci, idan akwai wani concomitant gallbladder cuta, rage cin abinci №5 nada. Musamman jiyya ga marasa lafiya hanta da aka ba da ake bukata.

Amfani a cikin wadannan lokuta, bitamin far, da yin amfani da choleretic kudi. Kuma ba ya bukatar marasa lafiya rike musamman dima jiki magani.
Banana ko thermal jiyya a cikin hanta, ba kawai aikata ba mai kyau, amma kuma suna da cutarwa effects. A hangen nesa ne quite m. Marasa lafiya suna iya aiki, amma shi wajibi ne don rage jijiya, kazalika da jiki aiki.

Yãfẽwa hyperbilirubinemia da Gilbert ba ya bukatar musamman da magani. Marasa lafiya ya bi wasu shawarwari domin kada su sa m manifestations da cutar.

Table №5.

• Yarda a yi amfani da: rauni shayi, ruwan 'ya'yan itace, low-mai gida cuku, alkama burodi miya da kayan lambu broth, nama, low-mai, kintsattse hatsi, kaza, m' ya'yan itãcen marmari.
• An haramta ci: naman alade, sabo pastries, alayyafo, zobo, nama mai, mustard, m kifi, ice cream, barkono, barasa, black kofi.
• Riko - suna gaba daya shafe nauyi motsa jiki. A amfani da takardar sayen magani magunguna: anticonvulsants, maganin rigakafi, anabolic steroids, idan ya cancanta - analogues na jima'i hormones, wanda aka yi amfani da magani daga hormonal rushewa, kazalika da 'yan wasa - don bunkasa mai kira yi.
• Gaba daya daina shan taba, shan barasa.

Idan ka fuskanci bayyanar cututtuka na jaundice, likita zai iya rubũta a yawan magunguna.

• barbiturates Group - antiepileptic kwayoyi ne tasiri a rage matakin na bilirubin.
• Bile nufi.
• Hanyarmu, wanda tasiri a kan aiki na gallbladder kuma ta ducts. Hana da ci gaban cholelithiasis, cholecystitis.
• Hepatoprotectors (kare nufin kare hanta Kwayoyin da lalacewar).
• Chelators. Wajen da inganta tukar tumbi da bilirubin daga gut.
• Narkewa kamar enzymes aka nada tare da dyspeptic cuta (tashin zuciya, amai, flatulence) - taimakon narkewa.
• Phototherapy - daukan hotuna zuwa haske blue fitilu take kaiwa zuwa ga halaka nama gyarawa bilirubin. Don hana ido konewa bukatar da za a kare.

Da aiwatar da wadannan shawarwari, marasa lafiya da al'ada da matakan bilirubin, bayyanar cututtuka faruwa da yawa kasa akai-akai.