Lefu la McCune-Albright

McCune-Albright syndrome ke lefu la lefutso le amang masapo, lihormone le 'mala (letlalo).

Lefu la McCune-Albright le bakoa ke liphetoho tse etsahalang lefapheng la GNAS liphatsa tsa lefutso. Palo e nyane, empa eseng kaofela, ea lisele tsa motho e na le lefutso lena le fosahetseng (mosaicism).

Boloetse bona ha bo futsetsoe.

Letšoao le ka sehloohong la lefu la McCune-Albright ke bohlankana kapa boroetsaneng pele ho nako. Linako tsa ho ilela khoeli li ka qala bongoaneng, nako e telele pele matsoele kapa moriri o hlahang (oo hangata o hlahang pele). Karolelano ea lilemo tseo matšoao a hlahang ke lilemo tse 3. Leha ho le joalo, ho kena bohlankaneng kapa boroetsaneng le ho tsoa khoeli ho etsahetse likhoeling tsa 4 ho isa ho tse 6 ho banana.

Khōlo ea pele ea thobalano le eona e ka etsahala ho bashanyana, empa eseng hangata joalo ka banana.

Matšoao a mang a kenyelletsa:

• Ho robeha ha masapo
• Ho holofala ha masapo sefahlehong
• Bokheleke
• Lebenkele le lecha le sa tloaelehang le na le mabala a maholo

Tlhahlobo ea 'mele e ka bontša matšoao a:

• Khōlo e sa tloaelehang ea lesapo lehata
• Lipelo tse sa tloaelehang tsa pelo (arrhythmias)
• Bokheleke
• Bokheleke
• Libaka tse kholo tsa cafe-au-lait letlalong
• Lefu la sebete, jaundice, mafura a sebete
• Lisele tse kang lebali masapong (fibrous dysplasia)

Liteko li ka bonts'a:

• Mathata a Adrenal
• Boemo bo phahameng ba hormone ea parathyroid (hyperparathyroidism)
• Boemo bo phahameng ba hormone ea qoqotho (hyperthyroidism)
• Adrenal hormone e sa tloaelehang
• Boemo bo tlase ba phosphorus maling (hypophosphatemia)
• Li-cysts tsa mahe a bomme
• Lihlahala tsa pituitary kapa thyroid
• Tekanyo e sa tloaelehang ea prolactin ea mali
• Boemo bo sa tloaelehang ba kholo ea kholo

Liteko tse ling tse ka etsoang li kenyelletsa:

• MRI ea hlooho
• X-ray ea masapo

Ho ka etsoa liteko tsa liphatsa tsa lefutso ho netefatsa hore na o fumane eng.

Ha ho na kalafo e khethehileng bakeng sa lefu la McCune-Albright. Lithethefatsi tse thibelang tlhahiso ea estrogen, joalo ka testolactone, li lekile ka katleho e itseng.

Lintho tse sa tloaelehang tsa Adrenal (tse kang Cushing syndrome) li ka phekoloa ka ho buuoa ho tlosa litšoelesa tsa adrenal. Gigantism le pituitary adenoma li tla hloka ho phekoloa ka meriana e thibelang tlhahiso ea lihormone, kapa ka ho buuoa.

Ho sa tloaelehang ha masapo (fibrous dysplasia) ka linako tse ling ho tlosoa ka ho buuoa.

Fokotsa palo ea li-x-ray tse nkiloeng libakeng tse anngoeng ke 'mele.

Bophelo ba bophelo bo batla bo tloaelehile.

Mathata a ka kenyelletsa:

• Bofofu
• Mathata a litlolo a tsoang linthong tse sa tloaelehang tsa masapo
• Ho se utloe litsebeng
• Osteitis fibrosa cystica
• Ho kena bohlankaneng kapa boroetsaneng pele ho nako
• Ho pheta masapo a robehileng
• Lihlahala tsa masapo (tse sa tloaelehang)

Bitsa mofani oa tlhokomelo ea bophelo haeba ngoana oa hau a qala ho kena bohlankaneng pele ho nako kapa a na le matšoao a mang a lefu la McCune-Albright Ho ka fanoa ka tlhabollo ea lefutso, mme mohlomong le liteko tsa lefutso haeba lefu le ka fumanoa.

Polyostotic fibrous dysplasia

• Anterior skeletal anatomy
• Neurofibromatosis - sebaka se seholo sa cafe-au-lait

Garibaldi LR, Chemaitilly W. Mathata a kholo ea bokhachane. Ho: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 578.

DM ea setaele. Physiology le mathata a ho kena bohlankaneng kapa boroetsaneng. Ka: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, bahlophisi. Buka ea Williams ea Endocrinology. La bo14 la ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 26.