Qoraa: Clyde Lopez
Taariikhda Abuurista: 17 Julay 2021
Taariikhda Cusboonaysiinta: 16 Noofeembar 2024
Anonim
Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment
Dareemoleyad: Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment

Qanacsan

Waa maxay baaritaanka 17-hydroxyprogesterone (17-OHP)?

Tijaabadani waxay cabireysaa qadarka 17-hydroxyprogesterone (17-OHP) ee dhiiga kujira. 17-OHP waa hormoon ay sameeyeen qanjidhada adrenal, laba qanjirrood oo ku yaal kilyaha dusheeda. Qanjirada 'adrenal gland' waxay sameysaa dhowr hoormoon, oo ay ku jiraan cortisol. Cortisol waxay muhiim u tahay ilaalinta dhiig karka, sonkorta dhiiga, iyo shaqooyinka qaar ee nidaamka difaaca jirka. 17-OHP waxaa loo sameeyay iyada oo qayb ka ah geeddi-socodka soo saarista cortisol.

Baadhitaanka 17-OHP wuxuu ka caawiyaa baaritaanka cillad hidde ah oo dhif ah oo loo yaqaan 'congenital adrenal hyperplasia' (CAH). CAH, isbeddel hidde ah, oo loo yaqaan 'mutation', ayaa ka hortagaya qanjirka 'adrenal gland' inuu sameeyo cortisol ku filan. Maaddaama qanjirada 'adrenal gland' ay si adag u shaqeeyaan si ay u sameeyaan cortisol badan, waxay soo saaraan 17-OHP dheeraad ah, oo ay weheliyaan hormoonno jinsi ah oo lab ah.

CAH wuxuu sababi karaa koritaan aan caadi ahayn oo ku saabsan xubnaha galmada iyo astaamaha galmada. Astaamaha cilladdu waxay u dhexeeyaan mid fudud illaa mid daran. Haddii aan la daweyn, qaababka aadka u daran ee CAH waxay sababi karaan dhibaatooyin daran, oo ay ku jiraan fuuqbax, dhiig kar hoose, iyo garaaca wadnaha oo aan caadi ahayn (arrhythmia).


Magacyada kale: 17-OH progesterone, 17-OHP

Maxaa loo adeegsadaa?

Baaritaanka 17-OHP waxaa badanaa loo isticmaalaa in lagu ogaado CAH-da dhallaanka cusub. Waxaa sidoo kale loo isticmaali karaa in:

  • Baadhitaanka CAH ee carruurta waaweyn iyo dadka waaweyn ee laga yaabo inay yeeshaan qaab fudud oo cilladda ah. CAH ka fudud, astaamaha waxaa laga yaabaa inay soo baxaan nolosha dambe, ama mararka qaarkood haba yaraatee.
  • La soco daaweynta CAH

Maxaan ugu baahanahay baaritaanka 17-OHP?

Ilmahaagu wuxuu u baahan doonaa baaritaan 17-OHP ah, badiyaa 1-2 maalmood dhalashada ka dib. 17-Baaritaanka OHP ee CAH hadda sharciga ayaa u baahan qeyb ka mid ah baaritaanka dhallaanka. Baadhitaanka ilmaha dhasha ahi waa baadhitaan dhiig oo fudud oo lagu hubiyo cuduro kala duwan oo halis ah.

Carruurta waaweyn iyo dadka waaweyn ayaa sidoo kale u baahan kara baaritaan haddii ay leeyihiin astaamaha CAH. Calaamaduhu way kala duwanaan doonaan iyadoo ku xiran hadba sida uu cilladdu u daran tahay, da'da ay calaamaduhu u muuqdaan, iyo haddii aad tahay lab ama dhadig.

Astaamaha nooca ugu daran ee cilladda ayaa caadi ahaan soo ifbaxa 2-3 toddobaad dhalashada ka dib.

Haddii ilmahaagu ku dhasho meel ka baxsan Mareykanka oo aanu helin baaritaanka dhallaanka cusub, waxay u baahan karaan baaritaan haddii ay leeyihiin mid ama in ka badan astaamaha soo socda:


  • Xubnaha taranka oo aan si cad u ahayn lab ama dhadig (madmadow ku jira xubinta taranka)
  • Fuuqbax
  • Matagga iyo dhibaatooyinka kale ee quudinta
  • Wadna garaac aan caadi ahayn (arrhythmia)

Carruurta waaweyni malaha astaamo illaa ay ka qaan gaadhayaan. Gabdhaha, astaamaha CAH waxaa ka mid ah:

  • Xilliga caadada oo aan caadi ahayn, ama aan lahayn caadooyin gebi ahaanba
  • Muuqaalka hore ee timaha burooyinka iyo / ama gacanta
  • Timo xad dhaaf ah oo wajiga iyo jidhka ah
  • Cod qoto dheer
  • Kintirka oo weynaaday

Wiilasha, astaamaha waxaa ka mid ah:

  • Guska balaartay
  • Qaangaarnimada hore (qaangaadhnimada hore)

Ragga iyo dumarka qaangaarka ah, astaamaha waxaa ka mid noqon kara:

  • Dhalmo la'aan (awood la'aanta inaad uur yeelato ama uur yeelato lamaane)
  • Finanka daran

Maxaa dhacaya inta lagu jiro baaritaanka 17-OHP?

Baadhitaanka dhallaanka cusub, xirfadlaha daryeelka caafimaad wuxuu cidhibta canuggaaga ku nadiifin doonaa aalkolo wuxuuna ciribta ciribta ku durayaa irbad yar. Bixiyuhu wuxuu ururin doonaa dhawr dhibcood oo dhiig ah oo uu faashad ku rido goobta.


Inta lagu jiro baaritaanka dhiigga ee carruurta waaweyn iyo dadka waaweyn, xirfadle daryeel caafimaad ayaa tijaabo dhiig ka qaadi doona xididka gacantaada, isagoo isticmaalaya cirbad yar. Kaddib cirbada la geliyo, qadar yar oo dhiig ah ayaa lagu ururin doonaa tuubada baaritaanka ama fijaan. Waxaa laga yaabaa inaad dareento xoog wax yar ah markii irbadda ay soo galayso ama ay ka baxeyso. Tani badanaa waxay qaadataa in ka yar shan daqiiqo.

Ma u baahanahay inaan sameeyo wax walba si aan ugu diyaargaroobo imtixaanka?

Ma jiraan diyaargarow gaar ah oo loo baahan yahay tijaabada 17-OHP.

Ma jiraan wax khatar ah oo imtaxaanku leeyahay?

Khatar aad u yar ayaa adiga ama cunuggaaga ugu jirta baaritaanka 17-OHP. Waxaa laga yaabaa inaad xanuun yar ama nabar ku yeelato barta irbadda lagu muday, laakiin astaamaha badankood si dhakhso leh ayey u baxaan. Ilmahaagu wuxuu dareemi karaa xoogaa yar oo qanjaruufo marka cidhibku xidhmayaan, oo nabar yar ayaa ka soo bixi kara goobta. Tani waa inay dhaqso u tagtaa.

Maxay natiijooyinka ka dhigan yihiin?

Haddii natiijooyinka ay muujiyaan heerar sare oo 17-OHP ah, waxay u badan tahay in adiga ama ilmahaagu aad qaadataan CAH. Badanaa, heerarka aadka u sarreeya macnaheedu waa qaab aad u daran oo xaaladda ah, halka heerarka dhexdhexaadka ah ee sarreeyaa caadi ahaan macnaheedu yahay qaab fudud.

Haddii adiga ama ilmahaaga laga daweynayo CAH, heerarka hoose ee 17-OHP waxay noqon karaan inay daaweyntu shaqeyneyso. Daaweynta waxaa ku jiri kara dawooyin lagu beddelo cortisol maqan. Mararka qaar qalliin ayaa loo sameeyaa si loo beddelo muuqaalka iyo shaqada xubnaha taranka.

Haddii aad su'aalo ka qabtid natiijooyinkaaga ama natiijooyinka ilmahaaga, la hadal daryeel caafimaad bixiyahaaga.

Wax badan ka baro baaritaanka shaybaarka, kala duwanaanshaha tixraaca, iyo fahamka natiijooyinka.

Ma jiraan wax kale oo aan u baahanahay inaan ka ogaado baaritaanka 17-OHP?

Haddii adiga ama ilmahaaga laga helay CAH, waxaad u baahan kartaa inaad la tashato la-taliye hidde ah, xirfadle si gaar ah ugu tababaran hidda-socodka. CAH waa cillad hidde ah oo ay tahay in labada waalidba ay yeeshaan isbadal hidde ah oo keena CAH. Waalidku waxaa laga yaabaa inuu yahay side hiddo-wadaha, taas oo macnaheedu yahay inay leeyihiin hidda-wadaha laakiin badiyaa ma laha astaamo cudur. Hadday labada waalidba yihiin side, ilmo kasta wuxuu 25% fursad u leeyahay inuu ku dhaco cudurka.

Tixraacyo

  1. Aasaaska Cares [Internet]. Isbahaysiga (NJ): Aasaaska Daryeelka; c2012. Muxuu Yahay Dhalashada Adrenal Hyperplasia (CAH) ;; [daliishaday 2019 Aug 17]; [ilaa 3 shaashad]. Waxaa laga heli karaa: https://www.caresfoundation.org/what-is-cah
  2. Eunice Kennedy Shriver Machadka Qaranka ee Caafimaadka Carruurta iyo Horumarinta Aadanaha [Internet]. Rockville (MD): Waaxda Caafimaadka iyo Adeegyada Aadanaha ee Mareykanka; Kala-bixinta Adrenal Hyperplasia (CAH): Macluumaadka Xaaladda; [daliishaday 2019 Aug 17]; [ilaa 3 shaashad]. Waxaa laga heli karaa: https://www.nichd.nih.gov/health/topics/cah/conditioninfo
  3. Shabakadda Caafimaadka Hormone [Internet]. Bulshada Endocrine; c2019. Dhalmada Adrenal Hyperplasia; [cusboonaysiiyay 2018 Sep; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://www.hormone.org/diseases-and-conditions/congenital-adrenal-hyperplasia
  4. Caafimaadka Carruurta Saacadaha (Internetka). Jacksonville (FL): Mu'asasada Nemours; c1995–2019. Dhalmada Adrenal Hyperplasia; [xigasho 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://kidshealth.org/en/parents/congenital-adrenal-hyperplasia.html
  5. Caafimaadka Carruurta Saacadaha (Internetka). Jacksonville (FL): Mu'asasada Nemours; c1995–2019. Imtixaannada Baarista ee Dhallaanka cusub; [xigasho 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://kidshealth.org/en/parents/newborn-screening-tests.html
  6. Tijaabooyinka Shaybaarka ee Internetka [Internetka] Washington D.C; Ururka Mareykanka ee Kiimikada Caafimaadka; c2001–2019. 17-Hydroxyprogesterone; [cusboonaysiiyay 2018 Dec 21; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://labtestsonline.org/tests/17-hydroxyprogesterone
  7. Tijaabooyinka Shaybaarka ee Internetka [Internetka] Washington D.C; Ururka Mareykanka ee Kiimikada Caafimaadka; c2001–2019. Madhalaysnimo; [cusboonaysiiyay 2017 Nofeembar 27; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://labtestsonline.org/conditions/infertility
  8. Machadka Kansarka Qaranka [Internet]. Bethesda (MD): Waaxda Caafimaadka iyo Adeegyada Aadanaha ee Mareykanka; Qaamuuska NCI ee Shuruudaha Kansarka: lataliyaha hiddaha; [daliishaday 2019 Aug 17]; [ilaa 3 shaashad]. Waxaa laga heli karaa: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/794108
  9. Xarunta Qaranka ee Hormarinta Cilmiga Tarjumaadda: Xarunta Macluumaadka Cudurada Hiddaha iyo Dhif [Internet]. Bethesda (MD): Waaxda Caafimaadka iyo Adeegyada Aadanaha ee Mareykanka; 21-hydroxolase yaraanta; [cusboonaysiiyay 2019 Apr 11; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://rarediseases.info.nih.gov/diseases/5757/21-hydroxylase-deficiency
  10. Aasaaska Sixirka [Internetka]. Warrenville (IL): Aasaaska Aasaasiga; c1989–2019. Dhalmada Adrenal Hyperplasia; [daliishaday 2019 Aug 17]; [ilaa 4 shaashadood]. Waxaa laga heli karaa: https://www.magicfoundation.org/Growth-Disorders/Congenital-Adrenal-Hyperplasia
  11. Maarso ee Dimes [Internet]. Arlington (VA): Maarso ee Dimes; c2020. Tijaabooyinka Baarista Ilmaha Dhallaanka ah ee Ilmahaaga; [waxaa daliishaday 2020 Aug 8]; [ilaa 3 shaashad]. Waxaa laga heli karaa: https://www.marchofdimes.org/baby/newborn-screening-tests-for-your-baby.aspx
  12. Caafimaadka UF: Jaamacadda Florida Health [Internet]. Gainesville (FL): Jaamacadda Florida Caafimaadka; c2019. 17-OH progesterone: Guudmarka; [cusboonaysiiyay 2019 Aug 17; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://ufhealth.org/17-oh-progesterone
  13. Caafimaadka UF: Jaamacadda Florida Health [Internet]. Gainesville (FL): Jaamacadda Florida Caafimaadka; c2019. Dhalmada 'adrenal hyperplasia': Dulmar guud; [cusboonaysiiyay 2019 Aug 17; daliishaday 2019 Aug 17]; [ilaa 2 shaashad]. Waxaa laga heli karaa: https://ufhealth.org/congenital-adrenal-hyperplasia

Macluumaadka ku yaal boggan waa in aan loo isticmaalin beddel daryeel caafimaad oo xirfad leh ama talo. La xiriir bixiye daryeel caafimaad haddii aad su'aalo ka qabtid caafimaadkaaga.

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