Pseudohypoparathyroidism

Pseudohypoparathyroidism (PHP) waa cillad hidde ah oo jirku ku guuldareysto inuu ka jawaabo hoormoonka parathyroid.

Xaaladda la xiriirta waa hypoparathyroidism, taas oo jidhku aanu samayn hormoon parathyroid ku filan.

Qanjirrada 'parathyroid' waxay soo saaraan hormoon parathyroid (PTH). PTH waxay caawisaa xakamaynta kalsiyum, fosfooraska, iyo fitamiin D-ga dhiiga kujira waxayna muhiim u tahay caafimaadka lafaha.

Haddii aad leedahay PHP, jirkaaga wuxuu soo saaraa qaddarka saxda ah ee PTH, laakiin waa "iska caabbinta" saameynteeda. Tani waxay keentaa heerarka kaalshiyamka dhiigga oo hooseeya iyo heerarka fosfateerka dhiigga oo sarreeya.

PHP waxaa sababa hiddo-wadayaal aan caadi ahayn. Waxaa jira noocyo kala duwan oo PHP ah. Dhammaan noocyadu waa dhif iyo sida caadiga ah waxaa lagu ogaadaa carruurnimada.

• Nooca 1a waxaa lagu dhaxlaa hab ku dhisan autosomal. Taasi waxay ka dhigan tahay in waalid keliya uu u baahan yahay inuu kuu soo gudbiyo hiddo-wade cilladaysan si aad xaaladda u leedahay. Waxaa sidoo kale loo yaqaan Albright osteodystrophy. Xaaladda ayaa sababi dherer gaaban, weji wareega ah, buurnida, dib u dhaca koritaanka, iyo lafaha gacanta oo gaagaaban. Astaamuhu waxay kuxiran yihiin inaad hiddo-wadaha ka dhaxasho hooyadaa ama aabahaaga.
• Nooca 1b wuxuu ku lug leeyahay iska caabinta PTH oo keliya kelyaha. In yar ayaa laga ogyahay nooca 1b marka loo eego nooca 1a. Kaalshiyamka dhiigga ku jira wuu hooseeyaa, laakiin ma jiro mid ka mid ah astaamaha kale ee lagu garto Albright osteodystrophy.
• Nooca 2 sidoo kale wuxuu ku lug leeyahay kaalshiyamta dhiigga oo hooseysa iyo heerarka fosfateerka dhiigga oo sarreeya. Dadka foomkan leh ma laha astaamo jireed oo ay wadaagaan dadka qaba Nooca 1a. Cilladaha hidde ahaaneed ee sababa lama yaqaan. Way ka duwan tahay Nooca 1b sida kelyaha uga jawaabaan heerarka sare ee PTH.

Calaamaduhu waxay la xiriiraan heer hoose oo kaalshiyam ah waxaana ka mid ah:

• Indho-beelka indhaha
• Dhibaatooyinka ilkaha
• Kabuubyo
• Suuxdin
• Tetany (ururinta astaamaha oo ay kujiraan murqaha muruqyada iyo murqaha gacmaha iyo cagaha iyo murqaha oo iskulaabma)

Dadka qaba Albright osteodystrophy-ka dhaxalka ah waxaa laga yaabaa inay yeeshaan astaamaha soo socda:

• Kalshiyam-ka ayaa dhiga maqaarka hoostiisa
• Kala-baxyo beddeli kara jilbaha faraha la taabto
• Wejiga wareega iyo qoorta oo gaaban
• Lafaha gacanta oo gaagaaban, gaar ahaan lafta ka hooseysa farta 4aad
• Dherer Gaaban

Tijaabooyinka dhiigga ayaa loo sameyn doonaa si loo hubiyo kalsiyum, fosfooraska, iyo heerarka PTH. Waxaad sidoo kale u baahan kartaa baaritaanno kaadi ah.

Tijaabooyinka kale waxaa ka mid noqon kara:

• Tijaabada hidaha
• Madaxa MRI ama CT scan maskaxda

Bixiyaha xanaanada caafimaadkaaga ayaa kugula talin doona kaalshiyamka iyo fitamiin D-ka si aad uhesho heerka kalsiyum ee saxda ah. Haddii heerka fosfateetka dhiiggu sarreeyo, waxaad u baahan kartaa inaad raacdo cunto fosfoorasku ku yar yahay ama aad qaadato daawooyinka loo yaqaan 'fosfate binders' (sida kaalshiyamka kaarboonate ama kalsiyum cetate). Daaweyntu caadi ahaan waa mid cimri dheer.

Kaalshiyam dhiig oo hooseeya ee ku jira PHP ayaa caadi ahaan ka fudud marka loo eego noocyada kale ee hypoparathyroidism, laakiin darnaanta astaamuhu way ka duwanaan kartaa dadka kala duwan.

Dadka qaba nooca 1a PHP waxay u badan tahay inay yeeshaan dhibaatooyin kale ee nidaamka endocrine (sida hypothyroidism iyo hypogonadism).

PHP waxaa laga yaabaa inuu ku xirnaado dhibaatooyinka kale ee hoormoonka, taasoo keentay:

• Jinsiga oo hooseeya
• Horumarka galmada oo gaabis ah
• Heerarka tamarta oo hooseeya
• Miisaanka oo kordha

La xiriir daryeelahaaga haddii adiga ama ilmahaagu aad isku aragtaan wax calaamado ah oo ah heerka kaalshiyamka hooseeya ama 'pseudohypoparathyroidism'.

Albright osteodystrophy; Noocyada 1A iyo 1B pseudohypoparathyroidism; PHP

• Qanjirada 'Endocrine'
• Qanjirrada Parathyroid

Bastepe M, Juppner H. Pseudohypoparathyroidism, Albright’s osteodystrophy la iska dhaxlo, iyo heseroplasia osseous oo horumar leh: xanuuno ka dhasha firfircoonida isbedelada GNAS. Gudaha: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Dadka waaweyn iyo carruurta. 7aad ed. Philadelphia, PA: Elsevier Saunders; 2016: cutubka 66.

Doyle DA. Pseudohypoparathyroidism (Albright la iska dhaxlo osteodystrophy). 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 590.

Thakker RV. Qanjirka 'parathyroid', 'hypercalcemia' iyo 'hypocalcemia'. Gudaha: Goldman L, Schafer AI, eds. Daawada Goldman-Cecil. 26aad. Philadelphia, PA: Elsevier; 2020: cutubka 232.