CAH natna enkawlna thar a ni! A chungchanga hriat tur i neihte (Congenital Adrenal Hyperplasia) .

I chhungkuaah hian tu emaw, a bik takin naupang, pianpui hormone disorder nei an awm em? Hmeichhia zingah hian thanlenna tlahniam, puitlin hma, leh thlasik hun mumal lo ang chi harsatna te i hre tawh mai thei. Hengte thlen thei genetic condition hmuh tur awm lo tak chu ‘Congenital Adrenal Hyperplasia’ an ti a. A tawi zawngin CAH kan ti ang. Vawiin hian he thil leh a enkawlna thar lo chhuak chungchang tawi te tein kan sawi dawn a ni.

A awlsam zawngin, CAH chu eng nge ni?

CAH hi genetic condition a ni a , adrenal glands, kal chunga awm gland te tak te pahnih te hian kan nunna atana pawimawh hormone cortisol an siam chhuak thei lo.

Kan taksa hi factory angin han ngaihtuah teh. He factory-a thil siam pakhat (cortisol) a tlakchham chuan thluaka control center (pituitary gland) chu a buai a, adrenal glands-ah signal (ACTH) a thawn a, a thawkrim zawk thin. Hetianga a tihchak lutuk avang hian cortisol siam chhuah chu a pung lo nain, chu ai chuan mipa hormone ‘androgens’ an tih tam zawk a siam tan ta zawk a ni.

He hormone inthlauhna hian harsatna tam tak a thlen thei a ni:

• Hmeichhia naute: Pian tirh hian pawn lam serh hi naute mipa serh ang mai a ni thei.
• Mi pahnih tan: harsatna, than chak lohna, puitlin hma, leh nau pai theih lohna te hi nakin lawkah a thleng thei.

Tin, CAH vei thenkhat chuan hormone pawimawh dang aldosterone an siam chhuah pawh a tlahniam thei bawk. He hormone tel lo chuan CAH hi a na hle thei a, shock, coma, thihna thlengin a thlen thei a ni.

Kum 70 chhung enkawl tawh leh beiseina thar a awm tawh

Kum sawm tam tak chhung chu CAH enkawlna awmchhun chu taksaa hormone cortisol level hniam tak chu steroid dose sang tak , hydrocortisone ang chi hmanga thlak a ni. Mahse, heng steroid tam tak hi rei tak ei hian side effect engemaw zat a nei thei a ni. Chung zinga a tam ber chu naupang taksa rihna sang, thisen sang, ruh chak lo, leh naupangte thanlenna tlahniam te hi a ni.

He damdawi thar (Crinecerfont) hian engtin nge hna a thawh?

Hei hi a pawimawh ber a ni. Damdawi hlui ang lo takin he damdawi thar hian kawng danglam takin hna a thawk a ni.

‘Crinesurfont’ hian pawn lam atanga hormone a rawn dah tel lo. Chu ai chuan he damdawi hi thluak control center (pituitary gland)-ah direct-in a kal a, adrenal glands tichaktu unwanted signal (ACTH) chhuah chu a tikhawlo zawk a ni .

A awlsam zawngin sawi ila, hei hi system tul lo tak ‘switch’ off ang mai a ni. Hei hian taksain a tul lova ‘androgen’ hormone a siam chhuah chu a thunun a ni.

Hei hi a thatna lian ber chu androgen level control a nih avangin hormonal balance vawng reng tura steroid dose mamawh chu nasa takin a tlahniam thei a ni Hei hian a awmzia chu steroid avanga side effects te pawh nasa takin a tihziaawm thei tihna a ni.

He damdawi hi tute tan nge a inhmeh ber?

Tunah hian CAH vei kum 4 chunglam tan 'Crinesurfont' hi pawm a ni tawh . Steroid thlakna ni lovin, steroid tihpunna a ni.A add-on treatment a ni.

• Naupang tan: Naupangte chu an la thang lian zel avangin steroid dose sang tak hian an thanlennaah nghawng lian tak a nei thei a ni. Chuvangin he damdawi, steroid dose tihhniam thei hi an tan a tangkai hle thei a ni. Hei hi tleirawl lai, hormone thunun a harsat lai hian a pawimawh hle.
• Puitling tan: He enkawlna hian puitling, hun rei tak chhung steroid ei avanga side effects tuar, emaw, hormone level nghet tak vawn that harsatna nei te tan pawh hahdamna a pe thei bawk.

Side effect leh himna chungchang zir rawh

Clinical trial-ah chuan he damdawi hian side effect tenau, hun eng emaw chen atana hman tur tlemte chauh a nei tih hmuhchhuah a ni.

• Puitling tan: hahna, lu na, luak chhuak, ruh na.
• Naupang tan: lu na, pum na, hah, hnar khar.

Pawimawh tak chu heng symptoms tam zawk hi a na lo hle a, damlo damdawi ei tawh loh ngai zat pawh a tlem hle.

Vaukhânna bik: Adrenal Crisis hi fimkhur rawh!

He damdawi ei hian natna khirh tak ‘adrenal crisis’ an tih hriat a pawimawh hle . A awlsam zawngin sawi ila, taksain stress nasa tak (e.g., infection na tak, surgery, accident) chhunga ‘cortisol’ mamawh zat a dawn loh chuan he natna hi a awm thei a ni.

Engtin nge he damdawi hi ka/ka fa tan a tha em tih ka hriat theih ang?

He thutlukna hi i doctor, a bik takin endocrinologist nen inrawnkhawmin siam tur a ni . I doctor chuan he damdawi hi i tan a tha em tih hriat nan thil tam tak a ngaihtuah ang a, chung zingah chuan i thisen test result, i than chak dan, i rihna, thisen sang, leh tuna hormone level te pawh a tel ang.

Hei hi CAH damdawi a ni lo a, mahse damlote nunphung tihchangtlunna tur, steroid side effects tihziaawmna tur leh hormone enkawlna awlsam zawka siam a nih theih nan beiseina lian tak a awm.