Matenda a McCune-Albright

Matenda a McCune-Albright ndimatenda amtundu omwe amakhudza mafupa, mahomoni, ndi utoto (khungu).

Matenda a McCune-Albright amayamba chifukwa cha kusintha kwa ma GNAS jini. Chiwerengero chochepa, koma osati chonse, cha maselo amunthuyu chimakhala ndi cholakwika ichi (mosaicism).

Matendawa siobadwa nawo.

Chizindikiro chachikulu cha matenda a McCune-Albright ndikutha msinkhu kwa atsikana. Kusamba kumatha kuyamba adakali aang'ono, mabere kapena tsitsi lisanachitike (zomwe zimayamba kuchitika). Zaka zapakati pazizindikirozi ndi zaka zitatu. Komabe, kutha msinkhu ndi kutaya magazi kwachitika miyezi 4 mpaka 6 mwa atsikana.

Kukula msanga pogonana kumatha kukhalanso kwa anyamata, koma osati pafupipafupi ngati atsikana.

Zizindikiro zina ndizo:

• Mafupa amathyoka
• Zofooka za mafupa kumaso
• Zosangalatsa
• Café yaying'ono, yaying'ono kapena yaying'ono

Kuyezetsa thupi kumatha kuwonetsa:

• Kukula kwachilendo m'mfupa
• Nyimbo zosazolowereka (arrhythmias)
• Zosintha
• Zosangalatsa
• Mawanga akulu a cafe-au-lait pakhungu
• Matenda a chiwindi, jaundice, mafuta chiwindi
• Minofu yofanana ndi yotupa m'mafupa (fibrous dysplasia)

Mayeso atha kuwonetsa:

• Zovuta zadrenal
• Mlingo waukulu wa parathyroid hormone (hyperparathyroidism)
• Mlingo waukulu wa mahomoni a chithokomiro (hyperthyroidism)
• Zovuta za mahomoni a adrenal
• Mulingo wotsika wa phosphorous m'magazi (hypophosphatemia)
• Ziphuphu zamchiberekero
• Zotupa za pituitary kapena chithokomiro
• Mulingo wodabwitsa wa prolactin wamagazi
• Mlingo wosakula modabwitsa

Mayesero ena omwe angachitike ndi awa:

• MRI ya mutu
• X-ray ya mafupa

Kuyezetsa magazi kumachitika kuti mutsimikizire kuti ali ndi matendawa.

Palibe mankhwala enieni a McCune-Albright syndrome. Mankhwala omwe amaletsa kupanga estrogen, monga testolactone, adayesedwa bwino.

Zovuta za Adrenal (monga Cushing syndrome) zitha kuthandizidwa ndikuchitidwa opareshoni kuchotsa ma adrenal gland. Gigantism ndi pituitary adenoma iyenera kuthandizidwa ndi mankhwala omwe amaletsa kupanga mahomoni, kapena ndi opaleshoni.

Zovuta za mafupa (fibrous dysplasia) nthawi zina zimachotsedwa ndikuchitidwa opaleshoni.

Chepetsani kuchuluka kwa ma x-ray omwe atengedwa m'malo omwe akhudzidwa ndi thupi.

Nthawi yamoyo ndi yachilendo.

Zovuta zingaphatikizepo:

• Khungu
• Mavuto azodzikongoletsa ochokera kuzolowera mafupa
• Kugontha
• Osteitis fibrosa cystica
• Kutha msinkhu msanga
• Mobwerezabwereza mafupa osweka
• Zotupa za mafupa (zosowa)

Itanani woyang'anira zaumoyo wanu ngati mwana wanu akuyamba kutha msinkhu msanga, kapena ali ndi zizindikiro zina za matenda a McCune-Albright. Upangiri wa chibadwa, mwinanso kuyesa kwa majini, atha kuperekedwa ngati matenda atapezeka.

Polyostotic fibrous dysplasia

• Anterior mafupa anatomy
• Neurofibromatosis - chimphona cafe-au-lait malo

Garibaldi LR, Chemaitilly W. Zovuta zakukula kwaubereki. Mu: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 578.

Styne DM. Physiology ndi zovuta zakutha msinkhu. Mu: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, olemba. Buku la Williams la Endocrinology. Wolemba 14th. Philadelphia, PA: Elsevier; 2020: mutu 26.