Umlobi: Clyde Lopez
Usuku Lokudalwa: 17 Ujulayi 2021
Ukuvuselela Usuku: 16 Unovemba 2024
Anonim
Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment
Ividiyo: Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment

-Delile

Kuyini ukuhlolwa kwe-17-hydroxyprogesterone (17-OHP)?

Lokhu kuhlolwa kukala inani le-17-hydroxyprogesterone (17-OHP) egazini. I-17-OHP iyi-hormone eyenziwe yizindlala ze-adrenal, izindlala ezimbili ezibekwe ngaphezulu kwezinso. Izindlala ze-adrenal zenza ama-hormone amaningi, kufaka phakathi i-cortisol. ICortisol ibalulekile ekugcineni umfutho wegazi, ushukela wegazi, neminye imisebenzi yamasosha omzimba. I-17-OHP yenziwa njengengxenye yenqubo yokukhiqiza i-cortisol.

Ukuhlolwa kwe-17-OHP kusiza ukuxilonga ukugula okungavamile kofuzo okubizwa nge-congenital adrenal hyperplasia (CAH). E-CAH, ushintsho lwezakhi zofuzo, olwaziwa njengokuguquka kwezakhi zofuzo, luvimbela indlala ye-adrenal ekwenzeni i-cortisol eyanele. Njengoba izindlala ze-adrenal zisebenza kanzima ukwenza i-cortisol eningi, zikhiqiza i-17-OHP eyengeziwe, kanye namahomoni athile owesilisa ocansini.

I-CAH ingadala ukukhula okungajwayelekile kwezitho zobulili nezici zocansi. Izimpawu zesifo sisuka kobumnene ziye kwesinzima. Uma ingalashwa, izinhlobo ezinzima kakhulu ze-CAH zingadala izinkinga ezinkulu, kufaka phakathi ukuphelelwa amandla emzimbeni, umfutho wegazi ophansi, nokushaya kwenhliziyo okungavamile (i-arrhythmia).


Amanye amagama: 17-OH progesterone, 17-OHP

Kusetshenziselwa ini?

Ukuhlolwa kwe-17-OHP kuvame ukusetshenziselwa ukuxilonga i-CAH ezinganeni ezisanda kuzalwa. Ingasetshenziswa futhi uku:

  • Xilonga i-CAH ezinganeni ezindala nakubantu abadala abangahle babe nefomu elibi lesi sifo. Ku-CAH emnene, izimpawu zingavela kamuva empilweni, noma kwesinye isikhathi zingabi khona nhlobo.
  • Bheka ukwelashwa kwe-CAH

Kungani ngidinga isivivinyo se-17-OHP?

Ingane yakho izodinga ukuhlolwa kwe-17-OHP, imvamisa kungakapheli izinsuku eziyi-1-2 ngemuva kokuzalwa. Ukuhlolwa kwe-17-OHP kwe-CAH manje kudingwa ngumthetho njengengxenye yokuhlolwa kwezingane ezisanda kuzalwa. Ukuhlolwa okusanda kuzalwa ukuhlolwa kwegazi okulula okuhlola izifo ezinhlobonhlobo ezibucayi.

Izingane ezindadlana nabantu abadala nabo bangadinga ukuhlolwa uma benezimpawu ze-CAH. Izimpawu zizohluka ngokuya ngesifo esinzima kangakanani, ubudala lapho kuvela khona izimpawu, nokuthi ungowesilisa noma ungowesifazane.

Izimpawu zesimo esibi kakhulu sesifo zivame ukuvela kungakapheli amasonto ama-2-3 ngemuva kokuzalwa.

Uma ingane yakho izalwe ngaphandle kwase-United States futhi ingakutholi ukuhlolwa okusanda kuzalwa, ingadinga ukuhlolwa uma inezimpawu ezilandelayo noma ngaphezulu:


  • Izitho zobulili ezingacaci kahle ukuthi ziyabesilisa noma abesifazane (izitho zangasese eziyindida)
  • Ukuphela kwamanzi emzimbeni
  • Ukuhlanza nezinye izinkinga zokudla
  • Isigqi senhliziyo esingajwayelekile (i-arrhythmia)

Izingane ezindala kungenzeka zingabi nazimpawu kuze kube ukuthomba. Emantombazaneni, izimpawu ze-CAH zifaka:

  • Izinkathi zokuya esikhathini ezingajwayelekile, noma ukungabikho nhlobo kwezikhathi
  • Ukuvela kwasekuqaleni kwezindawo zokushicilela kanye / noma izinwele zengalo
  • Izinwele ezeqile ebusweni nasemzimbeni
  • Izwi elijulile
  • I-clitoris ekhulisiwe

Kubafana, izimpawu zifaka:

  • Umthondo okhulisiwe
  • Ukuthomba kwasekuqaleni (ukuthomba ngaphambi kwesikhathi)

Kubesilisa nabesifazane abadala, izimpawu zingafaka:

  • Ukungazali (ukungakwazi ukukhulelwa noma ukukhulelwa umlingani)
  • Izinduna ezinzima

Kwenzekani ngesikhathi sokuhlolwa kwe-17-OHP?

Ukuze kuhlolwe usana olusanda kuzalwa, uchwepheshe wezokunakekelwa kwempilo uzohlanza isithende sengane yakho ngotshwala bese ephoqa isithende ngenaliti encane. Umhlinzeki uzoqoqa amaconsi ambalwa egazi bese efaka ibhandishi kusayithi.


Ngesikhathi sokuhlolwa kwegazi ezinganeni ezindala nakubantu abadala, uchwepheshe wezokunakekelwa kwezempilo uzothatha isampuli yegazi emthanjeni osengalweni yakho, esebenzisa inaliti encane. Ngemuva kokuthi kufakwe inaliti, kuzoqoqwa inani elincane legazi kufakwe ithubhu yokuhlola noma isitsha. Ungase uzizwe uluma kancane lapho inaliti ingena noma iphuma. Lokhu ngokuvamile kuthatha imizuzu engaphansi kwemihlanu.

Ngabe kukhona engizodinga ukukwenza ukulungiselela isivivinyo?

Awekho amalungiselelo akhethekile adingekayo esivivinyo se-17-OHP.

Ingabe zikhona izingozi esivivinyweni?

Kunengozi encane kakhulu kuwe noma enganeni yakho ngokuhlolwa kwe-17-OHP. Ungaba nobuhlungu obuncane noma ukulimaza lapho kufakwe khona inaliti, kepha izimpawu eziningi ziyaphela ngokushesha. Ingane yakho ingazizwa incanyana lapho isithende sibhakwa, futhi umhuzuko omncane ungakheka esizeni. Lokhu kufanele kudlule ngokushesha.

Isho ukuthini imiphumela?

Uma imiphumela ibonisa amazinga aphezulu we-17-OHP, kungenzeka ukuthi wena noma ingane yakho une-CAH. Imvamisa, amazinga aphakeme kakhulu asho isimo esibi kakhulu sesimo, kuyilapho amazinga aphezulu ngokulingene esho ifomu elithambile.

Uma wena noma ingane yakho welashelwa i-CAH, amazinga aphansi we-17-OHP kungasho ukuthi ukwelashwa kuyasebenza. Ukwelashwa kungafaka imithi ezothatha indawo ye-cortisol elahlekile. Kwesinye isikhathi kwenziwa ukuhlinzwa ukushintsha ukubukeka nokusebenza kwesitho sangasese.

Uma unemibuzo ngemiphumela yakho noma ngemiphumela yengane yakho, khuluma nomhlinzeki wakho wezokunakekelwa kwempilo.

Funda kabanzi mayelana nokuhlolwa kwelabhorethri, ububanzi bezinkomba, nemiphumela yokuqonda.

Ngabe kukhona okunye engidinga ukukwazi ngokuhlolwa kwe-17-OHP?

Uma wena noma ingane yakho utholwe une-CAH, ungahle uthande ukubonisana nomeluleki wezofuzo, uchwepheshe oqeqeshwe ngokukhethekile kwezofuzo. I-CAH yisifo sofuzo lapho bobabili abazali kufanele babe nokuguqulwa kwezakhi zofuzo okubangela i-CAH. Umzali angaba ngumuntu ophethe ufuzo, okusho ukuthi banofuzo kodwa imvamisa abanazo izimpawu zesifo. Uma bobabili abazali bephatha, ingane ngayinye inamathuba angama-25% okuba nalesi simo.

Izinkomba

  1. ICares Foundation [i-Intanethi]. I-Union (NJ): ICares Foundation; c2012. Yini iCongenital Adrenal Hyperplasia (CAH) ?; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-3]. Kutholakala kusuka: https://www.caresfoundation.org/what-is-cah
  2. U-Eunice Kennedy Shriver Isikhungo Sikazwelonke Sezempilo Yezingane Nokuthuthukiswa Kwabantu [i-Inthanethi]. I-Rockville (MD): UMnyango Wezempilo kanye Nezinsizakalo Zabantu e-U.S. I-Congenital Adrenal Hyperplasia (CAH): Imininingwane Yesimo; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-3]. Kutholakala kusuka: https://www.nichd.nih.gov/health/topics/cah/conditioninfo
  3. IHormone Health Network [i-Intanethi]. Inhlangano ye-Endocrine; c2019. I-Adrenal Hyperplasia ezelwe; [kubuyekezwe i-2018 Sep; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://www.hormone.org/diseases-and-conditions/congenital-adrenal-hyperplasia
  4. Izingane Health from Nemours [Internet]. UJacksonville (FL): INemours Foundation; c1995–2019. I-Adrenal Hyperplasia ezelwe; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://kidshealth.org/en/parents/congenital-adrenal-hyperplasia.html
  5. Izingane Health from Nemours [Internet]. UJacksonville (FL): INemours Foundation; c1995–2019. Izivivinyo Zokuhlolwa Kwezinsana ezisanda kuzalwa; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://kidshealth.org/en/parents/newborn-screening-tests.html
  6. Ukuhlolwa Kwelebhu Ku-inthanethi [i-Intanethi]. IWashington D.C .; I-American Association for Clinical Chemistry; c2001–2019. I-17-Hydroxyprogesterone; [kubuyekezwe i-2018 Dec 21; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://labtestsonline.org/tests/17-hydroxyprogesterone
  7. Ukuhlolwa Kwelebhu Ku-inthanethi [i-Intanethi]. IWashington D.C .; I-American Association for Clinical Chemistry; c2001–2019. Ukungabi nenzalo; [kubuyekezwe i-2017 Nov 27; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://labtestsonline.org/conditions/infertility
  8. Isikhungo Somdlavuza Kazwelonke [i-Inthanethi]. I-Bethesda (MD): UMnyango Wezempilo kanye Nezinsizakalo Zabantu e-U.S. Isichazamazwi seNCI Semigomo Yomdlavuza: umeluleki wofuzo; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-3]. Kutholakala kusuka: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/794108
  9. Isikhungo Sikazwelonke Sokuthuthukisa Isayensi Yokuhumusha: Isikhungo Sokwaziswa Sofuzo kanye Nezifo Ezingavamile [i-Inthanethi]. I-Bethesda (MD): UMnyango Wezempilo kanye Nezinsizakalo Zabantu e-U.S. Ukushoda kwe-21-hydroxolase; [kubuyekezwe i-2019 Apr 11; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://rarediseases.info.nih.gov/diseases/5757/21-hydroxylase-deficiency
  10. IMagic Foundation [i-Intanethi]. IWarrenville (IL): Isisekelo Semilingo; c1989–2019. I-Adrenal Hyperplasia ezelwe; [kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-4]. Kutholakala kusuka: https://www.magicfoundation.org/Growth-Disorders/Congenital-Adrenal-Hyperplasia
  11. UMashi weDimes [i-Intanethi]. I-Arlington (VA): Mashi wamaDimes; c2020. Izivivinyo Zokuhlolwa Kwezingane Ezisanda Kuzalwa Zengane Yakho; [kucashunwe ngo-2020 Aug 8]; [cishe izikrini ezi-3]. Kutholakala kusuka: https://www.marchofdimes.org/baby/newborn-screening-tests-for-your-baby.aspx
  12. Impilo ye-UF: University of Florida Health [Internet]. IGainesville (FL): Inyuvesi yaseFlorida Impilo; c2019. I-17-OH progesterone: Ukubuka konke; [kubuyekezwe i-2019 Aug 17; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://ufhealth.org/17-oh-progesterone
  13. Impilo ye-UF: University of Florida Health [Internet]. IGainesville (FL): Inyuvesi yaseFlorida Impilo; c2019. I-hyperplasia ye-adrenal ye-Congenital: Ukubuka konke; [kubuyekezwe i-2019 Aug 17; kucashunwe ngo-2019 Aug 17]; [cishe izikrini ezi-2]. Kutholakala kusuka: https://ufhealth.org/congenital-adrenal-hyperplasia

Imininingwane ekulesi siza akufanele isetshenziswe esikhundleni sezokunakekelwa kwezokwelapha noma iseluleko. Xhumana nomhlinzeki wezempilo uma unemibuzo ngempilo yakho.

-Conywayo

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