Sengoli: Clyde Lopez
Letsatsi La Creation: 17 Phupu 2021
Ntlafatsa Letsatsi: 11 December 2024
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Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment
Video: Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment

Litaba

Teko ea 17-hydroxyprogesterone (17-OHP) ke eng?

Teko ena e lekanya palo ea 17-hydroxyprogesterone (17-OHP) maling. 17-OHP ke hormone e entsoeng ke litšoelesa tsa adrenal, litšoelesa tse peli tse ka holim'a liphio. Litšoelesa tsa adrenal li etsa lihormone tse 'maloa, ho kenyeletsoa le cortisol. Cortisol e bohlokoa bakeng sa ho boloka khatello ea mali, tsoekere maling le mesebetsi e meng ea sesole sa 'mele. 17-OHP e entsoe e le karolo ea ts'ebetso ea ho hlahisa cortisol.

Teko ea 17-OHP e thusa ho fumana lefu le sa tloaelehang la liphatsa tsa lefutso le bitsoang congenital adrenal hyperplasia (CAH). Ho CAH, phetoho ea liphatsa tsa lefutso, e tsejoang e le phetoho, e thibela tšoelesa ea adrenal ho etsa cortisol e lekaneng. Ha litšoelesa tsa adrenal li sebetsa ka thata ho etsa cortisol e ngata, li hlahisa 17-OHP e eketsehileng, hammoho le lihormone tse itseng tsa bong.

CAH e ka baka kholo e sa tloaelehang ea litho tsa botona le botšehali. Matšoao a lefu lena a fapana ho tloha ho o bonolo ho ea ho o matla. Haeba e sa phekoloe, mefuta e matla ea CAH e ka baka mathata a tebileng, ho kenyelletsa le ho omella, khatello ea mali e tlase le ho otla ha pelo ho sa tloaelehang (arrhythmia).


Mabitso a mang: 17-OH progesterone, 17-OHP

E sebelisetsoa eng?

Hangata ho sebelisoa tlhahlobo ea 17-OHP ho hlahloba CAH ho masea a sa tsoa tsoaloa. E kanna ea sebelisoa ho:

  • Hlahloba CAH ho bana ba baholo le ho batho ba baholo ba ka bang le mofuta o bobebe oa lefu lena. Ka CAH e bonolo, matšoao a ka hlaha hamorao bophelong, kapa ka linako tse ling ho hang.
  • Hlahloba kalafo ea CAH

Hobaneng ha ke hloka tlhahlobo ea 17-OHP?

Lesea la hau le tla hloka teko ea 17-OHP, hangata nakong ea matsatsi a 1-2 kamora ho hlaha. Ho etsa liteko tsa CAH tsa 17-OHP joale ho hlokahala ka molao e le karolo ea tlhahlobo ea masea a sa tsoa tsoaloa. Ho hlahloba lesea le sa tsoa tsoaloa ke tlhahlobo e bonolo ea mali e lekolang mafu a tebileng a fapaneng.

Bana ba baholo le batho ba baholo le bona ba ka hloka liteko haeba ba na le matšoao a CAH. Matšoao a tla fapana ho latela hore na lefu lena le matla hakae, lilemo tseo matšoao a hlahang ka tsona le hore na u monna kapa u mosali.

Matšoao a mofuta o matla haholo oa lefu lena hangata a hlaha nakong ea libeke tse 2-3 kamora ho hlaha.

Haeba ngoana oa hau a hlahetse kantle ho United States mme a sa hlahlojoe a sa tsoa tsoaloa, ba kanna ba hloka tlhahlobo haeba ba na le le leng la a mang a matšoao a latelang:


  • Likamano tsa botona le botšehali tse sa tsejoeng ka ho hlaka hore ke monna kapa mosali (genitalia e sa hlakang)
  • Ho felloa ke metsi 'meleng
  • Ho hlatsa le mathata a mang a phepo
  • Lipelo tse sa tloaelehang tsa pelo (arrhythmia)

Bana ba baholo ba kanna ba se be le matšoao ho fihlela ba kena bohlankaneng kapa boroetsaneng. Ho banana, matšoao a CAH a kenyelletsa:

  • Linako tse sa tloaelehang tsa ho ilela khoeli, kapa ho se na linako ho hang
  • Ponahalo ea pele ea pubic le / kapa moriri oa letsoho
  • Moriri o feteletseng sefahlehong le mmeleng
  • Lentsoe le tebileng
  • Clitoris e holisitsoeng

Ho bashanyana, matšoao a kenyelletsa:

  • Botoneng bo atolositsoeng
  • Ho kena bohlankaneng kapa boroetsaneng pele ho nako

Ho banna le basali ba baholo, matšoao a ka kenyelletsa:

  • Ho hloka thari (ho se khone ho ima kapa ho fumana molekane moimana)
  • Makhopho a maholo a 'mele

Ho etsahalang nakong ea teko ea 17-OHP?

Bakeng sa tlhahlobo ea lesea le sa tsoa tsoaloa, setsebi sa tlhokomelo ea bophelo bo botle se tla hloekisa serethe sa ngoana oa hau ka joala ebe se phunya serethe ka nale e nyane. Mofani oa thepa o tla bokella marotholi a 'maloa a mali ebe o beha bandage setšeng.


Nakong ea tlhahlobo ea mali bakeng sa bana ba baholo le ba baholo, setsebi sa tlhokomelo ea bophelo se tla nka sampole ea mali mothapong o letsohong la hao, a sebelisa nale e nyane. Kamora hore nalete e kenngoe, ho tla bokelloa mali a manyane ka har'a pompo ea liteko. U ka ikutloa u hlaba hanyane ha nale e kena kapa e tsoa. Hangata hona ho nka nako e ka tlase ho metsotso e mehlano.

Na ke tla hloka ho etsa eng kapa eng ho itokisetsa tlhahlobo?

Ha ho na litokisetso tse khethehileng tse hlokahalang bakeng sa tlhahlobo ea 17-OHP.

Na ho na le likotsi tsa tlhahlobo?

Ho na le kotsi e nyane haholo ho uena kapa ho lesea la hau ka tlhahlobo ea 17-OHP. U kanna oa ba le mahlaba a fokolang kapa ho longoa moo nale e kentsoeng teng, empa matšoao a mangata a fela kapele. Lesea la hau le ka 'na la ikutloa le phunya hanyane ha serethe se ts'oaroa,' me ho ka hlaha leqeba le lenyane setšeng. Sena se lokela ho tloha kapele.

Liphetho li bolelang?

Haeba liphetho li bonts'a maemo a phahameng a 17-OHP, mohlomong ke uena kapa ngoana oa hau ea nang le CAH. Ka tloaelo, maemo a phahameng haholo a bolela mofuta o boima oa boemo, ha maemo a holimo haholo a bolela mokhoa o bobebe.

Haeba uena kapa ngoana oa hau a ntse a alafshoa bakeng sa CAH, maemo a tlase a 17-OHP a ka bolela hore kalafo ea sebetsa. Kalafo e ka kenyelletsa meriana ea ho khutlisa cortisol e lahlehileng. Ka linako tse ling ho etsoa opereishene ho fetola ponahalo le tšebetso ea litho tsa botona kapa botšehali.

Haeba u na le lipotso mabapi le liphetho tsa hau kapa liphetho tsa ngoana oa hau, bua le mofani oa tlhokomelo ea bophelo.

Ithute haholoanyane ka liteko tsa laboratori, libaka tsa litšupiso, le liphetho tsa kutloisiso.

Na ho na le eng kapa eng eo ke hlokang ho e tseba ka tlhahlobo ea 17-OHP?

Haeba uena kapa ngoana oa hau ho fumanoe hore o na le CAH, o kanna oa batla ho buisana le moeletsi oa liphatsa tsa lefutso, setsebi se koetlisitsoeng ka ho khetheha liphatseng tsa lefutso. CAH ke lefu la lefutso leo ho lona batsoali ka bobeli ba tlamehang ho ba le phetoho ea lefutso e bakang CAH. Motsoali e kanna ea ba mojari oa lefutso, ho bolelang hore ba na le lefutso empa hangata ha ba na matšoao a lefu. Haeba batsoali ka bobeli e le bajari, ngoana e mong le e mong o na le monyetla oa 25% oa ho ba le boemo boo.

Litšupiso

  1. Cares Foundation [Inthanete]. Union (NJ): Motheo oa Cares; c2012. Congenital Adrenal Hyperplasia (CAH) ke eng ?; [e qotsitsoeng 2019 Aug 17]; [li-skrini tse ka bang 3]. E fumaneha ho tsoa ho: https://www.caresfoundation.org/what-is-cah
  2. Eunice Kennedy Shriver Setsi sa Naha sa Bophelo ba Bana le Nts'etsopele ea Botho [Marang-rang]. Rockville (MD): Lefapha la Bophelo le Litšebeletso tsa Botho la U.S. Congenital Adrenal Hyperplasia (CAH): Tlhahisoleseling ea Boemo; [e qotsitsoeng 2019 Aug 17]; [li-skrini tse ka bang 3]. E fumaneha ho tsoa ho: https://www.nichd.nih.gov/health/topics/cah/conditioninfo
  3. Hormone Health Network [Inthanete]. Mokhatlo oa Endocrine; c2019. Congenital Adrenal Hyperplasia; [e ntlafalitsoeng 2018 Sep; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://www.hormone.org/diseases-and-conditions/congenital-adrenal-hyperplasia
  4. Bophelo ba Bana ho tsoa Nemours [Marang-rang]. Jacksonville (FL): Motheo oa Nemours; c1995–2019. Congenital Adrenal Hyperplasia; [e qotsitsoeng 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://kidshealth.org/en/parents/congenital-adrenal-hyperplasia.html
  5. Bophelo ba Bana ho tsoa Nemours [Marang-rang]. Jacksonville (FL): Motheo oa Nemours; c1995–2019. Litlhahlobo tsa masea a sa tsoa tsoaloa; [e qotsitsoeng 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://kidshealth.org/en/parents/newborn-screening-tests.html
  6. Liteko tsa Lab Marang-rang [Inthaneteng]. Washington DC; Mokhatlo oa Amerika oa Clinical Chemistry; c2001–2019. 17-Hydroxyprogesterone; [e ntlafalitsoeng 2018 Dec 21; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://labtestsonline.org/tests/17-hydroxyprogesterone
  7. Liteko tsa Lab Marang-rang [Inthaneteng]. Washington DC; Mokhatlo oa Amerika oa Clinical Chemistry; c2001–2019. Ho hloka thari; [e ntlafalitsoeng 2017 Nov 27; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://labtestsonline.org/conditions/infertility
  8. Mokhatlo oa Naha oa Kankere [Inthanete]. Bethesda (MD): Lefapha la Bophelo le Litšebeletso tsa Botho la U.S. Bukantswe ya NCI ya Dipehelo tsa Kankere: moeletsi wa lefutso; [e qotsitsoeng ka la 9 Phato 17]; [li-skrini tse ka bang 3]. E fumaneha ho tsoa ho: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/794108
  9. Setsi sa Naha sa Tsoelo-pele ea Saense ea Phetolelo: Setsi sa Boitsebiso ba Mafu le Mafu a Rare [Internet]. Bethesda (MD): Lefapha la Bophelo le Litšebeletso tsa Botho la U.S. Khaello ea 21-hydroxolase; [e ntlafalitsoeng 2019 Apr 11; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://rarediseases.info.nih.gov/diseases/5757/21-hydroxylase-deficiency
  10. The Magic Foundation [Inthanete]. Warrenville (IL): Motheo oa Boselamose; c1989–2019. Congenital Adrenal Hyperplasia; [e qotsitsoeng 2019 Aug 17]; [hoo e ka bang li-skrini tse 4]. E fumaneha ho tsoa ho: https://www.magicfoundation.org/Growth-Disorders/Congenital-Adrenal-Hyperplasia
  11. Tlhakubele ea Dimes [Marang-rang]. Arlington (VA): Hlakubele ea Dimes; c2020. Litlhahlobo tsa masea a sa tsoa hlaha tsa liteko tsa ngoana oa hau; [e qotsitsoeng ka la 2020 Phato 8]; [li-skrini tse ka bang 3]. E fumaneha ho tsoa ho: https://www.marchofdimes.org/baby/newborn-screening-tests-for-your-baby.aspx
  12. Bophelo ba UF: Univesithi ea Florida Health [Marang-rang]. Gainesville (FL): Univesithi ea Florida Health; c2019. 17-OH progesterone: Kakaretso; [e ntlafalitsoeng 2019 Aug 17; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://ufhealth.org/17-oh-progesterone
  13. Bophelo ba UF: Univesithi ea Florida Health [Marang-rang]. Gainesville (FL): Univesithi ea Florida Health; c2019. Congenital adrenal hyperplasia: Kakaretso; [e ntlafalitsoeng 2019 Aug 17; ho qotsitsoe 2019 Aug 17]; [li-skrini tse ka bang 2]. E fumaneha ho tsoa ho: https://ufhealth.org/congenital-adrenal-hyperplasia

Tlhahisoleseling e fumanehang sebakeng sena sa marang-rang ha ea lokela ho sebelisoa ho nka sebaka sa tlhokomelo ea bongaka le likeletso. Ikopanye le mofani oa tlhokomelo ea bophelo haeba u na le lipotso ka bophelo ba hau.

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