Cudurka McCune-Albright

Cudurka McCune-Albright waa cudur ku dhaca hidde-sideyaasha oo ku dhaca lafaha, hormoonnada, iyo midabka (midabka midabka) ee maqaarka.

Cudurka loo yaqaan 'McCune-Albright syndrome' waxaa sababa isbeddellada ku dhaca GNAS hiddo-wadaha Tiro yar, laakiin aan ahayn dhammaan, unugyada qofka ayaa kujira hiddo-wadaha cilladaysan (mosaicism).

Cudurkan lama iska dhaxlo.

Astaamaha ugu weyn ee cudurka McCune-Albright syndrome waa qaangaadhnimada hore ee gabdhaha. Muddooyinka caadada ayaa bilaaban kara caruurnimada hore, waqti dheer kahor naasaha ama timaha xubinta taranka (oo caadiyan dhaca marka hore). Celcelis ahaan da'da calaamaduhu muuqdaan waa 3 sano jir. Si kastaba ha noqotee, qaan-gaarnimada iyo dhiig-baxa caadada ayaa ku dhacay ugu horreyn 4 ilaa 6 bilood gabdhaha.

Horumarka galmada hore waxay sidoo kale ku dhici kartaa wiilasha, laakiin maahan sida badanaa gabdhaha.

Calaamadaha kale waxaa ka mid ah:

• Jabka lafaha
• Ciladaha ku dhaca lafaha wajiga
• Gigantism
• Meel aan caadi ahayn, oo leh maqaaxiyo waaweyn oo baaxad weyn leh oo lakabyo ah

Baadhitaanka jirka ayaa laga yaabaa inuu muujiyo calaamadaha:

• Kordhinta lafaha aan caadiga ahayn ee madaxa
• Wadna garaac aan caadi ahayn (arrhythmias)
• Acromegaly
• Gigantism
• Meelo waaweyn oo cafe-au-lait ah oo maqaarka saaran
• Cudurka beerka, cagaarshow, beerka dufanka badan
• Nudaha nabarrada u eg ee lafta (fibrous dysplasia)

Tijaabooyinka waxaa laga yaabaa inay muujiyaan:

• Ciladaha adrenal
• Heerka sare ee hoormoonka parathyroid (hyperparathyroidism)
• Heerka sare ee hoormoonka tayroodhka (hyperthyroidism)
• Cilladaha hormoonka aan caadiga ahayn
• Heerka hoose ee fosfooraska ku jira dhiigga (hypophosphatemia)
• Finanka ugxaanta
• Burooyinka ama burooyinka tayroodhka
• Heerka prolactin ee dhiiga oo aan caadi ahayn
• Heerka hormoonka koritaanka aan caadiga ahayn

Tijaabooyinka kale ee laga yaabo in la sameeyo waxaa ka mid ah:

• MRI madaxa
• Raajooyinka lafaha

Baaritaanka hidda-sidaha ayaa la sameyn karaa si loo xaqiijiyo cudurka.

Ma jiro daaweyn gaar ah oo loo yaqaan 'McCune-Albright syndrome'. Daawooyinka xayira wax soo saarka estrogen, sida testolactone, ayaa lagu tijaabiyay xoogaa guul ah.

Cilladaha aan caadiga ahayn ee Adrenal (sida Cushing syndrome) ayaa lagu daaweyn karaa qalliin si looga saaro qanjidhada adrenal. Gigantism iyo pituitary adenoma waxay u baahan doonaan in lagu daaweeyo daawooyinka xannibaya soosaarka hoormoonka, ama qalliin.

Lafaha aan caadiga ahayn (fibrous dysplasia) ayaa mararka qaarkood qalliin looga saaraa.

Xaddid tirada raajada laga qaado meelaha jirku ku dhacay.

Cimriga waa caadi.

Dhibaatooyinka waxaa ka mid noqon kara:

• Indho la’aan
• Dhibaatooyinka qurxinta ee ka yimaada cilladaha lafaha
• Dhago la'aan
• Cudurka Osteitis fibrosa cystica
• Qaangaarka oo dhicis ah
• Lafo jaban oo soo noqnoqday
• Burooyinka lafaha (dhif)

Wac daryeel caafimaad bixiyahaaga haddii ilmahaagu bilowdo qaangaadhnimada goor hore, ama uu leeyahay astaamo kale oo ah cudurka McCune-Albright syndrome. La-talinta hidda-sidaha, iyo suurta-galnimada baaritaanka hidda-socodka, ayaa lagu talin karaa haddii cudurka la ogaado.

Cudurka 'Polyostotic fibre dysplasia'

• Anatomy qalfoofka hore
• Neurofibromatosis - rug cafe-au-lait ah

Garibaldi LR, Chemaitilly W. Dhibaatooyinka horumarka qaangaarka. Gudaha: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Buugga Buugga Caafimaadka Caruurta. 21aad ed. Philadelphia, PA: Elsevier; 2020: baab 578.

Styne DM. Jirka iyo cilladaha qaan-gaarnimada. Gudaha: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Buugga Buugga Endocrinology. 14aad. Philadelphia, PA: Elsevier; 2020: cutubka 26.