Umbhali: Clyde Lopez
Umhla Wokudalwa: 17 Eyekhala 2021
Hlaziya Umhla: 16 Eyenkanga 2024
Anonim
Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment
Ividiyo: Congenital adrenal hyperplasia : Etiology ,Pathophysiology ,Clinical features ,Diagnosis ,Treatment

Umxholo

Yintoni uvavanyo lwe-17-hydroxyprogesterone (17-OHP)?

Olu vavanyo lilinganisa inani le-17-hydroxyprogesterone (17-OHP) egazini. I-17-OHP yincindi yedlala eyenziwe ngamadlala adrenal, amadlala amabini abekwe ngaphezulu kwezintso. Amadlala adrenal enza iihomoni ezininzi, kubandakanya i-cortisol. ICortisol ibalulekile ekugcineni uxinzelelo lwegazi, iswekile yegazi, kunye neminye imisebenzi yenkqubo yomzimba yokuzikhusela. I-17-OHP yenziwa njengenxalenye yenkqubo yokuvelisa i-cortisol.

Uvavanyo lwe-17-OHP lunceda ukufumanisa isifo esingaqhelekanga semfuza esibizwa ngokuba yi-congenital adrenal hyperplasia (CAH). Kwi-CAH, utshintsho lwemfuza, olwaziwa njengotshintsho, luthintela i-adrenal gland ekwenzeni i-cortisol eyaneleyo. Njengoko amadlala eadrenal esebenza nzima ukwenza i-cortisol engakumbi, avelisa i-17-OHP eyongezelelweyo, kunye neehomoni ezithile zesini esingamadoda.

I-CAH inokubangela ukukhula okungaqhelekanga kwamalungu esini kunye neempawu zesondo. Iimpawu zoluhlu lwesifo ukusuka kubumnene ukuya kubunzima. Ukuba ayinyangwa, ezona ndlela zinzima ze-CAH zinokubangela iingxaki ezinzulu, kubandakanya ukomisa amanzi emzimbeni, uxinzelelo lwegazi oluphantsi, kunye nentliziyo engaqhelekanga (arrhythmia).


Amanye amagama: 17-OH progesterone, 17-OHP

Isetyenziselwa ntoni?

Uvavanyo lwe-17-OHP luhlala lusetyenziselwa ukufumanisa i-CAH kwiintsana. Inokusetyenziselwa:

  • Xilonga i-CAH kubantwana abadala nakubantu abadala abanokuba nefomathi engalunganga yesifo. Kwi-CAH enamandla, iimpawu zingabonakalisa kamva ebomini, okanye ngamanye amaxesha akunjalo.
  • Ukubeka iliso kunyango lwe-CAH

Kutheni ndifuna uvavanyo lwe-17-OHP?

Usana lwakho luya kufuna uvavanyo lwe-17-OHP, ngesiqhelo kwiintsuku ezi-1-2 emva kokuzalwa. Uvavanyo lwe-17-OHP lwe-CAH ngoku luyafuneka ngokomthetho njengenxalenye yovavanyo olusandul 'ukuzalwa. Uvavanyo olusandul 'ukuzalwa luvavanyo lwegazi olulula olukhangela izifo ezahlukeneyo.

Abantwana abadala kunye nabantu abadala banokufuna ukuvavanywa ukuba baneempawu ze-CAH. Iimpawu ziya kwahluka ngokuxhomekeka ekubeni sibuhlungu kangakanani esi sifo, ubudala xa kuvela iimpawu, nokuba uyindoda okanye ulibhinqa.

Iimpawu zesona sifo siqatha sesi sifo sihlala sivela kwiiveki ezi-3 ukuya kwezi-3 emva kokuzalwa.

Ukuba umntwana wakho wazalelwa ngaphandle kwe-United States kwaye akazange afumane uhlolo olusanda kuzalwa, banokufuna ukuvavanywa ukuba banenye okanye nangaphezulu kwezi mpawu zilandelayo:


  • Amalungu esini angacacanga gca ukuba ngamadoda okanye abasetyhini (intsholongwane)
  • Ukuphelelwa ngamanzi emzimbeni
  • Ukugabha kunye nezinye iingxaki zokondla
  • Iingqungquthela zentliziyo engaqhelekanga (i-arrhythmia)

Abantwana abadala abanako ukuba neempawu de kube kwinqanaba lokufikisa. Kumantombazana, iimpawu ze-CAH zibandakanya:

  • Ixesha lokuya esikhathini, okanye ukungabikho kwaphela
  • Ukuvela kwangoko kwe-pubic kunye / okanye iinwele zengalo
  • Izinwele ezigqithisileyo ebusweni nasemzimbeni
  • Ilizwi elinzulu
  • I-clitoris eyandisiweyo

Kubafana, iimpawu zibandakanya:

  • Ukwandiswa kwepenisi
  • Ukufikisa ngaphambi kwexesha (ukufikisa kwangaphambili)

Kubantu abadala nabasetyhini, iimpawu zingabandakanya:

  • Ukungachumi (ukungakwazi ukukhulelwa okanye ukukhulelwa iqabane)
  • Amabala amakhulu

Kwenzeka ntoni ngexesha lovavanyo lwe-17-OHP?

Ukuhlolwa komntwana osandul 'ukuzalwa, ugqirha wezempilo uyakucoca isithende somntwana wakho ngotywala kwaye asibambe isithende ngenaliti encinci. Umboneleli uya kuqokelela amathontsi ambalwa egazi kwaye abeke i-bandage kwisiza.


Ngexesha lokuvavanywa kwegazi kubantwana abadala nakubantu abadala, ingcali yokhathalelo lwempilo iya kuthatha isampulu yegazi kumthambo engalweni yakho, usebenzisa inaliti encinci. Emva kokuba inaliti ifakiwe, inani elincinci legazi liya kuqokelelwa kwityhubhu yovavanyo okanye igutyana. Unokuziva uluma kancinci xa inaliti ingena okanye iphuma. Oku kuhlala kuthatha imizuzu engaphantsi kwesihlanu.

Ngaba kuya kufuneka ndenze nantoni na ukulungiselela uvavanyo?

Akukho lungiselelo lukhethekileyo lufunekayo kuvavanyo lwe-17-OHP.

Ngaba bukhona ubungozi kuvavanyo?

Kukho umngcipheko omncinci kuwe okanye kusana lwakho ngovavanyo lwe-17-OHP. Unokuba nentlungu encinci okanye ukrakra kwindawo apho inaliti yafakwa khona, kodwa uninzi lweempawu zihamba ngokukhawuleza. Usana lwakho lunokuziva lincinci kancinci xa isithende sikhutshiwe, kwaye ukubetha okuncinci kunokubakho kwindawo leyo. Oku kuya kuhamba ngokukhawuleza.

Zithetha ntoni iziphumo?

Ukuba iziphumo zibonisa amanqanaba aphezulu e-17-OHP, kunokwenzeka ukuba wena okanye umntwana wakho une-CAH. Ngokwesiqhelo, amanqanaba aphezulu kakhulu athetha uhlobo olungqongqo lwesi simo, ngelixa amanqanaba aphezulu ngokuphakathi edla ngokubhekisa kwifom ethe kratya.

Ukuba wena okanye umntwana wakho uphathwa nge-CAH, amanqanaba asezantsi e-17-OHP anokuthetha ukuba unyango lusebenza. Unyango lunokubandakanya amayeza endaweni yecortisol elahlekileyo. Ngamanye amaxesha utyando lwenziwa ukutshintsha inkangeleko nokusebenza kwamalungu esini.

Ukuba unemibuzo malunga neziphumo zakho okanye iziphumo zomntwana wakho, thetha nomboneleli wakho wezempilo.

Funda ngakumbi malunga novavanyo lwelabhoratri, uluhlu lweereferensi, kunye neziphumo zokuqonda.

Ngaba ikhona enye into endifuna ukuyazi malunga novavanyo lwe-17-OHP?

Ukuba wena okanye umntwana wakho ufumene ukuba une-CAH, unokufuna ukubonisana nomcebisi ngemfuzo, ingcali eqeqeshwe ngokukodwa kwimfuza. I-CAH kukuphazamiseka kwemfuza apho bobabini abazali kufuneka babe nokutshintsha kwemfuza okubangela i-CAH. Umzali unokuba ngumntu ophethe ufuzo, oko kuthetha ukuba banofuzo kodwa ngesiqhelo abanazo iimpawu zesifo. Ukuba bobabini abazali bangabathwali, umntwana ngamnye unamathuba angama-25% okuba abe nale meko.

Izalathiso

  1. Isiseko seCare [i-Intanethi]. Umanyano (NJ): Isiseko seCare; c2012. Yintoni iCongenital Adrenal Hyperplasia (CAH) ?; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-3]. Ifumaneka kwi: https://www.caresfoundation.org/what-is-cah
  2. U-Eunice Kennedy Shriver iZiko leLizwe lezeMpilo yaBantwana noPhuhliso lwaBantu [kwi-Intanethi]. I-Rockville (MD): Isebe lezeMpilo laseMelika kunye neeNkonzo zoLuntu; I-Congenital Adrenal Hyperplasia (CAH): Ulwazi lweMeko; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-3]. Ifumaneka kwi: https://www.nichd.nih.gov/health/topics/cah/conditioninfo
  3. Inethiwekhi yeHormone yezeMpilo [i-Intanethi]. Umbutho we-Endocrine; c2019. Ukuzalwa kwe-Adrenal Hyperplasia; [ihlaziywe ngo-2018 Sep; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://www.hormone.org/diseases-and-conditions/congenital-adrenal-hyperplasia
  4. Impilo yaBantwana kwiNemours [kwi-Intanethi]. UJacksonville (FL): Isiseko seNemours; c1995–2019. Ukuzalwa kwe-Adrenal Hyperplasia; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://kidshealth.org/en/parents/congenital-adrenal-hyperplasia.html
  5. Impilo yaBantwana kwiNemours [kwi-Intanethi]. UJacksonville (FL): Isiseko seNemours; c1995–2019. Uvavanyo lokuSanda kuzalwa; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://kidshealth.org/en/parents/newborn-screening-tests.html
  6. Uvavanyo lweLebhu kwi-Intanethi [kwi-Intanethi]. IWashington DC; Umbutho waseMelika weKhemesti yezonyango; c2001–2019. 17-Hydroxyprogesterone; [ihlaziywe ngo-2018 Dec 21; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://labtestsonline.org/tests/17-hydroxyprogesterone
  7. Uvavanyo lweLebhu kwi-Intanethi [kwi-Intanethi]. IWashington DC; Umbutho waseMelika weKhemesti yezonyango; c2001–2019. Ukungachumi; [ihlaziywe ngo-2017 Novemba 27; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://labtestsonline.org/conditions/infertility
  8. IZiko leSizwe loMhlaza [kwi-Intanethi]. I-Bethesda (MD): Isebe lezeMpilo laseMelika kunye neeNkonzo zoLuntu; Isichazi-magama seNCI seMiqathango yoMhlaza: umcebisi wemfuzo; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-3]. Ifumaneka kwi: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/794108
  9. IZiko leLizwe lokuqhubela phambili iiNzululwazi zoGuqulelo: iZiko loLwazi ngezeMfuza neziNqabileyo iZiko loLwazi [kwi-Intanethi]. I-Bethesda (MD): Isebe lezeMpilo laseMelika kunye neeNkonzo zoLuntu; Ukuswela kwe-21-hydroxolase; [ihlaziywe ngo-Epreli 2019; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://rarediseases.info.nih.gov/diseases/5757/21-hydroxylase-deficiency
  10. Isiseko soMlingo [kwi-Intanethi]. IWarrenville (IL): Isiseko soMlingo; c1989–2019. Ukuzalwa kwe-Adrenal Hyperplasia; [icatshulwe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-4]. Ifumaneka kwi: https://www.magicfoundation.org/Growth-Disorders/Congenital-Adrenal-Hyperplasia
  11. Ngomhla we-Dimes [kwi-Intanethi]. Arlington (VA): Matshi weeDimes; c2020. Uvavanyo lokuSanda kuzalwa koMntwana wakho; [icatshulwe ngo-2020 ka-Agasti 8]; [malunga nezikrini ezi-3]. Ifumaneka kwi: https://www.marchofdimes.org/baby/newborn-screening-tests-for-your-baby.aspx
  12. Impilo ye-UF: iYunivesithi yaseFlorida yezeMpilo [i-Intanethi]. IGainesville (FL): yeYunivesithi yaseFlorida yezeMpilo; c2019. I-17-OH progesterone: Ubume; [ihlaziywe ngo-2019 Agasti 17; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://ufhealth.org/17-oh-progesterone
  13. Impilo ye-UF: iYunivesithi yaseFlorida yezeMpilo [i-Intanethi]. IGainesville (FL): yeYunivesithi yaseFlorida yezeMpilo; c2019. I-hyperplasia ye-adrenal ye-Congenital: Ubume; [ihlaziywe ngo-2019 Agasti 17; ikhankanywe ngo-2019 ngo-Agasti 17]; [malunga nezikrini ezi-2]. Ifumaneka kwi: https://ufhealth.org/congenital-adrenal-hyperplasia

Ulwazi olukule ndawo akufuneki lusetyenziswe endaweni yonyango okanye iingcebiso. Nxibelelana nomboneleli wezempilo ukuba unemibuzo malunga nempilo yakho.

Izithuba Ezitsha

Iingcebiso emva kokutya ukuze unciphise ukutsha

Iingcebiso emva kokutya ukuze unciphise ukutsha

UKURhoxi wa KWE-RANITIDINENgo-Epreli 2020, uceliwe ukuba zonke iintlobo zokumi elwa kunye ne-over-the-counter (OTC) ranitidine (Zantac) zi u we kwimarike ya e-U . Olu ngcebi o lwenziwe kuba amanqanaba...
Ngaba ibhotolo yamandongomane ikwenza uzuze ubunzima?

Ngaba ibhotolo yamandongomane ikwenza uzuze ubunzima?

Ipeanut butter iyathandwa, iya a azeka. Igcwele izondlo eziyimfuneko, kubandakanya iivithamini, iiminerali, kunye namafutha a empilweni. Ngenxa yomxholo wamafutha aphezulu, ibhotolo yamandongomane yi-...