Kobadwa nako adrenal hyperplasia

Congenital adrenal hyperplasia ndi dzina lomwe limaperekedwa ku gulu la zovuta zobadwa nazo za adrenal gland.

Anthu ali ndi zilonda zam'mimbazi ziwiri. Imodzi ili pamwamba pa impso zawo zonse. Izi zimatulutsa mahomoni, monga cortisol ndi aldosterone, omwe ndi ofunikira pamoyo. Anthu omwe amabadwa ndi adrenal hyperplasia alibe ma enzyme adrenal gland amafunika kupanga mahomoni.

Nthawi yomweyo, thupi limapanga androgen yambiri, mtundu wa mahomoni ogonana amuna. Izi zimapangitsa kuti mawonekedwe amphongo awonekere koyambirira (kapena mosayenera).

Congenital adrenal hyperplasia imatha kukhudza anyamata ndi atsikana. Pafupifupi mwana mmodzi mwa ana 10,000 mpaka 18,000 amabadwa ndi kobadwa nako adrenal hyperplasia.

Zizindikiro zimasiyanasiyana, kutengera mtundu wa kobadwa nako adrenal hyperplasia wina yemwe ali nawo, komanso msinkhu wawo matendawa akapezeka.

• Ana omwe ali ndi mitundu yosakhazikika sangakhale ndi zizindikilo zobadwa ndi adrenal hyperplasia ndipo sangapezeke mpaka atafika msinkhu.
• Atsikana omwe ali ndi mawonekedwe owopsa nthawi zambiri amakhala ndi maliseche pakubadwa ndipo amatha kupezeka matenda asanawonekere.
• Anyamata amawoneka achibadwa pobadwa, ngakhale atakhala ndi mawonekedwe owopsa.

Kwa ana omwe ali ndi vuto lalikulu kwambiri, matendawa amakhala mkati mwa milungu iwiri kapena itatu atabadwa.

• Kusadyetsa bwino kapena kusanza
• Kutaya madzi m'thupi
• Electrolyte amasintha (kuchuluka kwa sodium ndi potaziyamu m'magazi)
• Nyimbo yachilendo

Atsikana omwe ali ndi mawonekedwe ocheperako nthawi zambiri amakhala ndi ziwalo zoberekera zachikazi (mazira, chiberekero, ndi machubu oyambira). Atha kukhala ndi zosintha izi:

• Kusamba modabwitsa kapena kulephera kusamba
• Kutuluka koyambirira kwa tsitsi lapa pubic kapena laakhwapa
• Kukula kwambiri kwa tsitsi kapena nkhope
• Kukulitsa kwina kwa nkongo

Anyamata okhala ndi mawonekedwe ofatsa nthawi zambiri amawoneka achibadwa atabadwa. Komabe, angawoneke kuti akutha msinkhu msanga. Zizindikiro zimaphatikizapo:

• Kuzama mawu
• Kutuluka koyambirira kwa tsitsi lapa pubic kapena laakhwapa
• Kukula kwa mbolo koma mayeso abwinobwino
• Minofu yopangidwa bwino

Anyamata ndi atsikana adzakhala amtali ngati ana, koma amafupikitsa kuposa achikulire.

Wothandizira zaumoyo wa mwana wanu adzaitanitsa mayeso ena. Mayeso wamba amagazi ndi awa:

• Ma seramu ma electrolyte
• Aldosterone
• Renin
• Cortisol

X-ray ya kumanzere ndi dzanja lingasonyeze kuti mafupa a mwanayo amawoneka ngati a munthu wamkulu kuposa msinkhu wawo weniweni.

Kuyesedwa kwa majini kumatha kuthandizira kuzindikira kapena kutsimikizira vutoli, koma sikofunikira kwenikweni.

Cholinga cha mankhwalawa ndikubwezeretsa kuchuluka kwa mahomoni kukhala abwinobwino, kapena pafupi. Izi zimachitika potenga mawonekedwe a cortisol, nthawi zambiri hydrocortisone. Anthu angafunike mankhwala owonjezerawa panthawi yamavuto, monga matenda akulu kapena opaleshoni.

Woperekayo adzawona momwe mwana wakhanda amakhalira ndi maliseche abwinobwino poyang'ana ma chromosomes (karyotyping). Atsikana omwe ali ndi maliseche owoneka ngati abambo amatha kuchitidwa opaleshoni kumaliseche ali akhanda.

Steroids omwe amagwiritsidwa ntchito pochiza congenital adrenal hyperplasia samakonda kuyambitsa zovuta monga kunenepa kwambiri kapena mafupa ofooka, chifukwa milingo imalowa m'malo mwa mahomoni omwe thupi la mwanayo silingathe kupanga. Ndikofunika kuti makolo anene zakupezeka kwa matenda ndikupanikizika kwa omwe amapereka kwa mwana wawo chifukwa mwanayo angafunikire mankhwala ambiri. Steroid sangaimitsidwe mwadzidzidzi chifukwa kutero kumatha kubweretsa kusakwanira kwa adrenal.

Mabungwewa atha kukhala othandiza:

• Maziko a National Adrenal Diseases Foundation - www.nadf.us
• MAGIC Foundation - www.magicfoundation.org
• Maziko a CARES - www.caresfoundation.org
• Kuperewera kwa Adrenal United - aiunited.org

Anthu omwe ali ndi vutoli ayenera kumwa mankhwala moyo wawo wonse. Nthawi zambiri amakhala ndi thanzi labwino. Komabe, amatha kukhala achidule kuposa achikulire, ngakhale atalandira chithandizo.

Nthawi zina, kobadwa nako adrenal hyperplasia kungakhudze chonde.

Zovuta zingaphatikizepo:

• Kuthamanga kwa magazi
• Shuga wamagazi ochepa
• Sodium wocheperako

Makolo omwe ali ndi mbiri yabanja yobadwa ndi adrenal hyperplasia (yamtundu uliwonse) kapena mwana yemwe ali ndi vutoli ayenera kulingalira za upangiri wa majini.

Matendawa asanabadwe amapezeka m'mitundu ina yobadwa nayo ya adrenal hyperplasia. Kuzindikira kumapangidwa mu trimester yoyamba ndi zitsanzo za chorionic villus. Matendawa mu trimester yachiwiri amapangidwa poyesa mahomoni monga 17-hydroxyprogesterone mu amniotic fluid.

Kuyezetsa ana kakhanda kumapezeka pamtundu wodziwika bwino wobadwa nawo wa adrenal hyperplasia. Zitha kuchitidwa pachidendene magazi (monga gawo la zowunikira zomwe zachitika pa ana obadwa kumene). Kuyesaku kumachitika pano m'maiko ambiri.

Matenda a Adrenogenital; Kusowa kwa 21-hydroxylase; CAH

• Zilonda za adrenal

Donohoue PA. Zovuta zakukula kwakugonana. Mu: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 606.

Yau M, Khattab A, Pina C, Yuen T, Meyer-Bahlburg HFL, Watsopano MI. Zofooka za andrenal steroidsidogenesis. Mu: Jameson JL, De Groot LJ, de Kretser DM, et al, olemba. Endocrinology: Akuluakulu ndi Ana. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier Saunders; 2016: chap 104.