Syndrome "pebaldizm": abin da shi ne, yana sa, cututtuka, bincike da kuma magance

Daya iri-iri na pigment metabolism cuta - rare ciwo "pebaldizm". Gnozdnym halin da discoloration na gashi a kansa. Wannan ba wani m kwaskwarima aibi, amma wani alama na babban adadin tsanani cututtuka.

A wannan labarin, za mu duba a cikin daki-daki, "pebaldizm". Haddasawa, cututtuka, ganewar asali, magani, shi ma za a bayyana.

Sanadin gashi Bleach

Take hakkin pigment metabolism ne depigmentation - pigment hasara, hyperpigmentation - karawa batawa.

The gashi launi pigment da darajar 2 - eumelinin, pheomelanin. Su ne a cikin bawo na gashi. Eumelanin sa gashi baki da kuma ruwan kasa pheomelanin - yellow-ja. A hade da wadannan pigments a cikin mutum da lambar da hulda da iska bada gashi a wani launi. Gashi launi ne genetically kaddara.

ciwo "pebaldizm" Sau nawa kada faruwa, abin da yake da wannan cuta?

A take hakkin da melanocytes, da pigment-rairaya gashi, graying da shekaru tasowa. Idan yanayin fara a wani matasa shekaru - wannan shi ne shaidar mai kayyade cuta, ko tsanani cututtuka.

Cututtuka a wanda yake shan wahala pigmentary musayar

Me ya sa ake "pebaldizm" ciwo, abin da yake wannan sabawa?

A wadannan jihohi suna tasowa a take hakkin da melanocytes:

• pebaldizm a ware form.
• zabiya.
• pebaldizm hade tare ido cuta, kunne, fata, da juyayi tsarin, musculoskeletal tsarin.

asibiti hoto

Yadda shi bayyana kanta "pebaldizm" ciwo, abin da yake nufi?

A asarar gashi pigment a cikin nau'i na bayyanar wani farin curl a kan shugaban na iya faruwa daga haihuwa zuwa adulthood.

A jima akwai wani take hakkin pigment metabolism, da mafi tsanani cikin hanyar mutum sakamakon.

Ware discoloration curl a hade tare da wani yanki daga discoloration a kan goshi. Tare da bayyanar foci na hyperpigmentation, t. E. Gnozdnogo duhun fata a gaban farfajiya na ciki, cinyoyinsa, kafafu, kirji, makamai girma daga 1 mm zuwa 6 cm.

Cuta lurking bayan pebaldizmom

Akwai babban rukuni na cututtuka da kuma anomalies na ci gaba, da hada tare da bleaching gashi tsanani lalacewa gabobin da kuma tsarin. Undiagnosed Pathology za a adana rai na yaro, kuma iyãyensa ne ya sadar da wani matsala.

Waardenburg-Klein ciwo

A cuta ne autosomal rinjaye juna na gado, watau. E. A wannan sau da yawa yakan faru a cikin maza da mata. A kayyade lahani za a iya gada daga ko dai iyaye.

Bugu da kari ga bleaching na gashi strands ne ya lura janar da kuma ocular manifestations da cutar.

Ocular bayyanar cututtuka da wannan cuta:

• hypoplasia na gashin idanu.
• yankuna masu launin toka a ciki girare;
• kunkuntar ido tsaguwa.
• daban-daban masu launin idanu.
• hyperopia.
• yadu spaced idanu.

Common bayyanar cututtuka na iya zama sãɓãwar launukansa digiri na tsanani:

• m zabiya.
• m hanci gada.
• short jiki.
• anomalies hakora.
• kurãme-mutism ko ya zama ruwan dare ji hasara.
• anomalies na ciki gabobin.
• canza girman da kwanyar.

Ganin cewa a farko da tsananin cutar da kuma na faruwa ne kawai m pebaldizmom bukatar da za a yi kariya ga ware tsanani Pathology. Wannan abin da piebaldism.

Wolfe ta ciwo

Ware heterochromia, t. E. A daban-daban ido launi ne mafi yawa samu a dabbobi. A cikin mutane, na mitar tsakanin dukan jama'a ne 1%.

Famous mutane tare da launi daban-daban idanu: Mila Kunis ( "Oz The Great da qarfi", "Bloodlines", "Jupiter hawa"), British actress Jane Seymour, mafi sani ga film "Live kuma Bari Mutu", David Bowie - Burtaniya dutse singer, songwriter, m, sauti m, artist, actor.

Ware heterochrony yake akwai wata cuta, da kuma a hade tare da pebaldizmom ciwo kira Wolfe, da kuma kaiwa zuwa wani azumi-girma nauyi.

Don bambance wannan cuta ya kamata ya kasance tare da retinitis pigmentosa - pigment epithelium dystrophy ido. Sau da yawa rungumi mana da wannan cuta - retinitis pigmentosa ba tare da pigment.

Inna da kuma 'yar tare da ciwo na "pebaldizm"

A cuta ne recessive, chromosome-nasaba mata gado. A mace wuce da gene wa ɗansa, kuma yana da wata cuta. 'Ya'ya mata na wadannan maza ne da lafiya, idan sun yi lafiya uwar. Zai zama kawai bayyanuwar pebaldizm.

Idan uwar ne da lafiya asibiti, amma shi ne mai jigilar kaya na gene, yaron zai zama lafiya.

Don gane asali cutar ta amfani da ophthalmoscopy, wadda tayi da fundus pigmentation spots kamar asu ya cinye.

Gipomelanoz ITO

Haka kuma cutar tasowa daga haihuwa. Sha da yara na biyu mata da maza tare da daidaita mita. Ba tabbatar hereditary watsa daga iyaye zuwa zuriya, amma cutar da ake ko da yaushe a hade tare da nakasar lahani na ciki gabobin.

A ganewar asali da aka sanya asibiti a kan tushen da wadannan cututtuka:

• bayyana a kan fata amelanotic rabo m, a siffar curls, snowflakes.
• A cikin palmar, da tafin saman da mucous membranes irin canje-canje ba faru.
• a cikin boko yankin na bleached gashi tashar - pebaldizm.
• shekaru depigmentation yankunan zama kasa a bayyane.
• lalacewar da juyayi tsarin ya ko da yaushe bayyana shafi tunanin mutum retardation, convulsive cuta, rage tsoka sautin;
• Pathology na ido ne bayyananne strabismus sãɓãwar launukansa digiri na tsanani.
• tare scoliosis, kirji nakasawa extremities a 25% na lokuta.
• tare da wani mita na 10% a hade tare da zuciya lahani.

Don bambance wannan cuta daga zama dole yanayin da ake kira urinary pigment. Suna da kama da na asibiti bayyanar cututtuka, amma kwanan nan gaji.

pigment incontinence

An wani m ciwo yana daukar kwayar cutar "pebaldizm"?

Cutar da ake daukar kwayar cutar daga uwa zuwa 'yar. Dangane da tsananin daga cikin asibiti hoto da aka bayyana ta da wadannan cututtuka:

• Skin rash a cikin nau'i na dogon tube wanda, bayan bushewa da kuma kau da crusts bar rabo pigmentation. Eruptions da wani undulating hanya tare da lokaci-lokaci bayyanar sabon fata Kwayoyin. Daga wadannan kaidi maturation yaro rasa pigment da kuma zama kodadde.
• Dysplasia da hakori sanyi, kusoshi, gashi depigmentation kamar yadda alopecia da kuma baldness.
• Neurological cuta an bayyana jinkiri Psycho-magana ci gaba, ta ƙara intracranial matsa lamba, seizures, mãsu rauni a hannuwansu da ƙafãfunsu.
• Eye cuta ne m, kuma bayyananna strabismus, nystagmus, cataracts, rage hangen nesa, makanta.

Gristselli ciwo

Wannan shi ne wani rukuni na cututtuka da za su iya faruwa a uku siffofin, babban kama shi ne a gaban discolored faci na gashi a kansa. Wadannan nuni ne samuwa:

• Neurological cuta da hypopigmentation.
• A mafi hatsari irin cuta a cikin abin da "pebaldizm" ciwo guda biyu tare da mai illa hematopoiesis. Clinical bayyananen: matsakaici zazzabi, paranormal da kuma magani. Tsanani zazzabi hankali qara, shiga kara girman hanta, da baƙin ciki, da rage erythrocytes, leukocytes, platelets, fibrinogen a cikin jini. A karshen ne da hannu a cikin matakai na juyayi tsarin.
• hypopigmentation na fata da kuma gashi, yana mai sãɓãnin mai tsanani, wanda har ya ci gaba ba tare da lalata da maikacin jini, da juyayi tsarin. Domin dace ganewar asali na wannan hatsari cuta ya kamata shawarta a hematologist likitan fata, itatuwa aure. Tabbata ga la'akari da kasancewar wani iyali na daban-daban cuta na pigment metabolism, da bincike don ware gene maye gurbi.

Gaggawa da kuma hadarin da cutar saboda da high mita hada shi tare da Down ciwo, Edwards, Turner.

Gane depigmentation na fata da kuma gashi na iya zama a haihuwa, amma ya tsayar da gaban Turner ta ciwo zai iya zama a matsayin mutum girma ne kawai a lokacin da alama short jiki, lymphostasis, cututtukan zuciya, hawan jini, underdeveloped mammary gland, low matsayi na nono, canzawa daga ciki al'aura gabobin connective zane. Intelligence a wadannan marasa lafiya kusan ba ya sha wahala, bango yanayi ne kusan ko da yaushe dagagge, akwai wasu m asar, sai murna, wanda ya bayyana mai kyau zamantakewa karbuwa daga wadannan marasa lafiya.

ƙarshe

A cikin mutane, gene na al'ada pigmentation na fata ne ko da yaushe rinjaye a dangane da gene alhakin da take hakkin pigment metabolism.

Ainihin ma'anar daga cikin matsalolin da pigmentation ne cewa su ne m alamomi na chromosomal cuta, mahara nakasar malformations.

Duk wani take hakkin da fata pigment metabolism, mucous membranes, da gashi ya shawarci wani gwani. Mun gani "pebaldizm" ciwo. Mene ne shi, shi ya bayyana.