Albright ciwo. Syndrome Mc Kyung-Albright-Braitseva. Haddasawa, magani

Syndrome Mc Kyung-Albright aka mai suna a cikin girmamawa biyu mashawarta likitoci, wanda aka bayyana shi fiye da rabin karni da suka wuce. Suka faɗa wa jama'a game da yara, wanda mafi yawansu sun girls. Mutane da yawa daga cikinsu sun short jiki, zagaye fuska, short wuyansa, short IV kuma V yatsun kafa ko metacarpal ƙasusuwan sun tsoka spasms, canje-canje a cikin kasusuwan sa, jinkiri teething enamel hypoplasia. Akwai kuma wani shafi tunanin mutum retardation da endocrine cututtuka, ya bayyana farkon balaga da hailar zub da jini, ci gaban da ƙirãza, ci gaban da pubic gashi kuma ƙirãza, da karuwa a cikin kudi na girma na yara da kuma canje-canje a cikin fata.

A zamani magani, da kalmar "Albright ciwo" da ake amfani da marasa lafiya da suke da duk ko kawai wasu endocrine da kuma fata munanan. Akwai lokuta inda ganewar asali kafa a farkon yara. Duk da haka, a hankula lokuta, shi ne ya sa wa yara 5-10 years old, dangane da ãyõyinMu, waxanda suke da halayyar wannan cuta. Kullum shi ne a rare kuma gaji. Ya zauna ba a sani ba a matsayin etiology da pathogenesis da wannan cuta. Bari mu dubi a cikin ãyõyin cutar.

Keta a cikin tsarin endocrine

More sau da yawa fiye da 'yan mata, da Albright ciwo an kiyaye precocious balaga, wanda aka sa ta estrogens saki a cikin jini daga ovarian cysts. Cysts iya kara bayan karu a size a cikin 'yan makonni ko kwanaki. Da taimakon duban dan tayi hanyoyin suna da damar ganin da su domin auna girman siffofin maruran. Cysts iya girma zuwa quite mai kyau size. Akwai lokuta, a lõkacin da ta yi girma ya girman golf ball, da cewa shi ne fiye da 50 mm a diamita.

Ƙirjinka augmentation da hailar zub da jini ne kiyaye tare da ci gaban da cysts. Idan yarinya ta fara haila kafin shekaru 2, shi ne na farko alama na Albright ciwo. Duk da haka, da gaban sababbu haila kuma ovarian cysts za a iya lura biyu a matasa da kuma adult mata. Wannan duk bai hana zuwa da yara masu lafiya.

Jiyya na yara da precocious mafarki, quite wuya da kuma wanda ya kasa aiki. Ko da mafitsara surgically cire, shi zai iya sake fito fili. Lokacin da shan hormone progesterone za a iya tsaya haila, amma m kudi na kashi ci gaba da kuma ci gaban da aka ba inda hakan ya sauka. Zai yiwu korau abubuwan da aikin na adrenal gland. A lura da amfani formulations domin ingestion tarewa estrogen kira.

thyroid aiki

50 bisa dari na mutane da Albright ciwo, wahala daga cuta na thyroid gland shine yake. Wannan ake kira goiter, nodules da cysts. Akwai iya zama rare lokuta da dabara tsarin canje-canje. A cikin wadannan marasa lafiya, da matakin samar da pituitary TSH ne low, amma matakan da thyroid hormones ne na al'ada dabi'u ko da dan kadan dagagge. Bi, ta hanyar da rage-rage kira na thyroid ji ba gani. An nuna a lokuta inda matakin ne babban isa ga saki da hormone.

Wuce kima mugunya girma hormone

Lokacin da cutar pituitary fara asirce yawa girma hormone. A yara, wanda aka sanya wannan ganewar asali a matsayin Albright ciwo, acromegaly da aka gano. The samari suka fara bayyana m fuska fasali, da sauri girma hannunwansa da ƙafafunsa, suka iya sha amosanin gabbai. Jiyya na yara da irin bayyanar cututtuka an rage zuwa m kau da pituitary yankin da kuma yin amfani da roba hormone somatostatin analogs, damuarn samar da ci gaban hormone.

Sauran endocrine cuta

Akwai rare da kuma wuce kima mugunya adrenal kara girma. Irin wannan warwarewarsu iya haifar da kiba jiki da kuma fuska, da karuwa a jikin nauyi, cessation na ci gaba da kuma fata fragility. Duk wadannan cututtuka kira Cushing ta ciwo. A lokacin da irin canje-canje suna cire shafi adrenal ko shan magunguna da cewa rage kira na Cortisol.

Wani lokacin yara da Albright ciwo, lura a cikin jini sosai low matakan da phosphorus saboda manyan asarar phosphate a cikin fitsari. Wannan cuta na iya zama dalilin kashi canje-canje dangantawa da rickets. Kamar yadda magani da na baka phosphate da kuma bitamin D a Bugu da kari.

Cuta hade tare da fata

A fata a haihuwa ko kuma jim kaɗan bayan haka ya bayyana spots na launi "kofi tare da madara". Su mafi sau da yawa faruwa a kan sacrum, akwati, kafafu, gindi, baya daga cikin wuyansa, goshi, fatar kan mutum, baya na kai. Dukan su ne ma wata ãyã cewa yaro Albright ciwo. Photos of wadannan aibobi za a iya gani a kasa.

Ko da yake wannan cutar ne neurofibromatosis, kuma suna da tabo launi "kofi tare da madara". Duk da haka, Albright ciwo ne halin da ya fi girma spots tare da sababbu shaci, su ne ƙarancin adadi. Bã su da wani diamita na daga 1 zuwa 'yan santimita, ruwan kasa tint. Launi duk da wannan, su ne m, a siffar, suna halin da m surface. A histological jarrabawa sau da yawa ya nuna cewa epidermis ba ya canja a tsarinta, amma adadin melanin a keratinocytes ya karu da dan kadan.

Ware spots na da irin wannan za a iya samunsa a cikin in ba haka ba lafiya mutane. Idan ba su dame kuma ba su girma, sa'an nan da magani ba lallai ba ne. Idan akwai m girma, akwai irregularly siffa spots, shi ne shawarar gudanar da bincike su histologically. Kuma a sa'an nan cire surgically.

ƙarshe

Saboda haka, zamu iya cewa ga Albright ciwo ne halin da asarar kashi ko kwanyar, gaban fata pigment spots, farkon balaga. Ko da yake akwai lokuta inda akwai kawai biyu na farko bayyanar cututtuka. A general, babban alama na ciwo ne kashi raunuka (osteodysplasia). Duk da haka, wannan tsari yana tsayawa lokacin da mafarki. A manya, kashi canje-canje ba su ci gaba. Kullum, da ganewa da kuma dace da magani, da hangen nesa daga far da wannan cuta ne quite m.