Dhalmada adrenal hyperplasia

Cudurka qanjirada 'conrenal adrenal hyperplasia' waa magac loo bixiyo koox ka mid ah cudurada la iska dhaxlo ee qanjirka 'adrenal gland'.

Dadku waxay leeyihiin 2 qanjirro adrenal ah. Midkood wuxuu ku yaalaa dusheeda mid kasta oo kelyahooda ah. Qanjirradan ayaa sameeya hormoonno, sida cortisol iyo aldosterone, kuwaasoo muhiim u ah nolosha. Dadka qaba conrenital adrenal hyperplasia waxaa ka maqan enzyme qanjidhada adrenal u baahan yihiin inay sameeyaan hormoonnada.

Isla mar ahaantaana, jidhku wuxuu soo saaraa androgen badan, oo ah nooc ka mid ah hoormoonka ragga. Tani waxay keentaa astaamaha lab inay hore u soo baxaan (ama si aan habooneyn).

Cudurka 'hypertlasia adrenal hyperplasia' ayaa ku dhici kara wiilasha iyo gabdhaha labadaba. Qiyaastii mid ka mid ah 10,000 ilaa 18,000 oo carruur ah ayaa ku dhasha qanjirada 'adrenal hyperplasia' ee lagu dhasho.

Calaamaduhu way kala duwanaan doonaan, waxayna kuxirantahay nooca hyperplasia ee lagu dhasho adrenal hyperplasia ee qof ku dhaco, iyo da'dooda marka cudurka la ogaado.

• Carruurta qaabab fudud ayaa laga yaabaa inaanay lahayn calaamado ama astaamo lagu garto cudurka 'adrenal hyperplasia' lagu dhasho lagamana ogaan karo ilaa iyo inta ay ka gaadhayaan qaangaadhnimada.
• Gabdhaha leh qaab aad u daran badanaa waxay leeyihiin xubnaha taranka ee ragga markay dhashaan waxaana laga yaabaa in la ogaado kahor intaan astaamuhu muuqan.
• Wiilasha waxay umuuqandoonaan caadi dhalashada, xitaa hadii ay qabaan qaab daran.

Carruurta leh qaabka daran ee cilladda, calaamadaha badanaa waxay ku soo baxaan 2 ama 3 toddobaad kadib dhalashada.

• Quudin xumo ama matagid
• Fuuqbax
• Isbedelada elektroolka (heerarka aan caadiga ahayn ee sodium iyo potassium ee dhiigga ku jirta)
• Wadna garaac aan caadi ahayn

Gabdhaha qaab fudud ayaa badanaa yeelan doona xubnaha taranka haweenka ee caadiga ah (ugxan sidaha, ilmagaleenka, iyo tuubbooyinka ugxanta). Waxay sidoo kale yeelan karaan isbeddelada soo socda:

• Xilliga caadada oo aan caadi ahayn ama caadada oo ku fashilanto
• Muuqaalka hore ee timaha maqaarka ama kilkisha
• Timo siyaado ah ama timaha wajiga
• Kordhinta kintirka

Wiilasha qaab fudud ayaa inta badan umuuqda kuwo caadi ah markay dhalayaan. Si kastaba ha noqotee, waxay u muuqan karaan inay qaan-gaaraan goor hore. Calaamadaha waxaa ka mid noqon kara:

• Cod qoto dheer
• Muuqaalka hore ee timaha kilkilaha ama kilkilaha
• Guska weynaaday laakiin tijaabooyinka caadiga ah
• Muruqyada sifiican u horumarsan

Wiilasha iyo gabdhuhuba way dhereri doonaan sida carruurta, laakiin aad ayey uga gaabin doonaan sidii caadiga ahayd marka loo eego dadka waaweyn.

Bixiyaha xanaanada caafimaadka canugaaga ayaa amri doona baaritaano qaarkood. Tijaabooyinka dhiigga ee caadiga ah waxaa ka mid ah:

• Serum elektrolytes
• Aldosterone
• Renin
• Cortisol

Raajada gacanta bidix iyo curcurka ayaa laga yaabaa inay muujiso in lafaha ilmuhu u muuqdaan inay yihiin kuwa qof ka weyn da'dooda dhabta ah.

Tijaabooyinka hiddo-wadaha ayaa kaa caawin kara ogaanshaha ama xaqiijinta cilladda, laakiin marar dhif ah ayaa loo baahan yahay.

Ujeedada daaweynta waa in lagu soo celiyo heerarka hoormoonka si caadi ah, ama ku dhowaad caadi. Tan waxaa lagu sameeyaa iyadoo la qaato nooc ka mid ah cortisol, inta badan hydrocortisone. Dadku waxay ubaahan karaan qiyaaso daawo oo dheeri ah inta lagu jiro waqtiga walaaca, sida jirro daran ama qalliin.

Bixiyaha ayaa go'aamin doona jinsiga hidaha ee cunuga ee leh xubinta taranka ee aan caadiga aheyn asaga oo hubinaya koromosoomyada (karyotyping). Gabdhaha leh xubnaha taranka ee ragga ayaa laga yaabaa in lagu sameeyo qalliin xubintooda taranka ah inta ay yaryihiin.

Daawooyinka isteeroydhiska loo isticmaalo si loogu daaweeyo hyperplasia adrenal hyperplasia badanaa ma keenaan waxyeellooyin sida cayilka ama lafaha daciifka ah, sababtoo ah qiyaasaha ayaa beddelaya hormoonnada jirka cunuggu uusan sameyn karin. Waxaa muhiim u ah waalidiinta inay ku soo wargeliyaan astaamaha infekshinka iyo walbahaarka bixiyaha ilmahooda sababtoo ah ilmuhu wuxuu u baahan karaa daawo dheeraad ah. Steroids si lama filaan ah looma joojin karo maxaa yeelay sidaas yeelku wuxuu u horseedi karaa ku filnaanta adrenal.

Ururradan ayaa laga yaabaa inay ku caawiyaan:

• Hay'adda Qaranka ee Cudurrada Ilmo-galeenka - www.nadf.us
• Aasaaska MAGIC - www.magicfoundation.org
• Mu'asasada CARES - www.caresfoundation.org
• Adrenal Insufficiency United - aiunited.org

Dadka qaba cilladan waa inay qaataan daawada noloshooda oo dhan. Badanaa waxay leeyihiin caafimaad wanaagsan. Hase yeeshe, way ka gaabi karaan dadka waaweyn ee caadiga ah, xitaa haddii la daaweynayo.

Xaaladaha qaarkood, hyperplasia adrenal hypergenia oo lagu dhasho ayaa saameyn ku yeelan kara bacriminta.

Dhibaatooyinka waxaa ka mid noqon kara:

• Dhiig kar
• Sonkorta dhiigga oo hooseysa
• Sodium yar

Waalidiinta leh taariikh qoys oo ku dhasha 'adrenal hyperplasia' (nooc kasta ha ahaadee) ama cunug qaba xaaladan waa inay tixgeliyaan la-talinta hidda-socodka.

Baadhitaanka uurka ka hor ayaa loo heli karaa noocyo ka mid ah qanjirka 'adrenal hyperplasia' lagu dhasho. Cilad-sheegista waxaa lagu sameeyaa saddexda bilood ee ugu horreysa sampling chorionic villus. Baadhista saddexda bilood ee labaad waxaa lagu sameeyaa iyadoo lagu cabiro hormoonnada sida 17-hydroxyprogesterone ee ku jira dheecaanka amniotic.

Tijaabada baaritaanka dhallaanka cusub ayaa loo heli karaa nooca ugu caamsan ee ku dhasha 'adrenal hyperplasia'. Waxaa lagu samayn karaa dhiiga ciribta (sida qeyb ka mid ah baaritaanada joogtada ah ee lagu sameeyo dhallaanka). Tijaabadani waxaa hadda lagu sameeyaa gobollada badankood.

Cudurka 'Adrenogenital Syndrome'; 21-hydroxylase yaraanta; CAH

• Qanjirada 'Adrenal'

Donohoue PA. Cilladaha horumarinta galmada. 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: cutubka 606.

Yau M, Khattab A, Pina C, Yuen T, Meyer-Bahlburg HFL, New MI. Cilladaha steroidogenesis andrenal. 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 104.