Hormone lama harsatna a ni em? Congenital Adrenal Hyperplasia (CAH) chungchang hi a awlsam zawngin i zir ang u

I fanu piang thar taksa peng hrang hrangte lan danah hian inthlak danglamna emaw, ngaihtuahna emaw i hmu em? A nih loh leh i fapa naupang hian a pian hun hmain a puitlin tawh tih a lantir tan em? I fa chu a luak chhuak a, a puak a, a nih loh leh plain lethargic mai mai a ni mai thei em? Vawiin hian heng thilte thlen thei genetic condition kan sawi dawn a ni. Hei hi Congenital Adrenal Hyperplasia, a tawi zawngin CAH kan ti a. Ngaihtuah buai suh, a hming hi a buaithlak deuhin a lang thei a, mahse a awlsam zawngin i sawi ang u.

CAH hian eng nge a awmzia mai?

Okay, CAH chu eng nge a nih kan en hmasa phawt ang u. Kan kal chungah hian gland pahnih, lukhum te tak te ang mai kan nei vek a. Hengte hi adrenal glands kan tih te hi an ni. Heng gland te pahnih hian kan taksa hnathawhna atana pawimawh tak tak hormone pawimawh tak tak engemaw zat an siam chhuak thin.

• Cortisol: Hei hi kan taksaa stress hormone ber a ni. Natna te, hlauhawmna te, leh lungkhamna te hmachhawn turin taksa a buatsaih a ni. Thisen sang, chakna level, leh thisen sugar level tihreh nan a pui bawk.
• Aldosterone: He hormone hian kan taksaa chi (sodium) leh tui balance dik tak vawng reng turin a pui a, chu chuan thisen sang leh thisen volume a control thei a ni.
• Androgens: Hengte hi mipa sex hormone te an ni. Testosterone hi chutiang hormone pakhat a ni. Heng hormone te hi puitlin tanna atan te, an than dan pangngai leh thanlenna atan te an pawimawh hle a ni.

Tunah chuan CAH vei chunga thil thleng chu heng hormone siam chhuahna atana mamawh enzyme bik chu taksaah a bo emaw, a tlahniam emaw a ni. Dish-a spice a bo chuan dish thlum a inthlak ang bawkin, he enzyme hi a bo chuan adrenal glands hian heng hormone te hi a siam tha thei lo.

Chuti a nih chuan eng nge thleng?

• Hormone cortisol hi a tul angin siam chhuah a ni lo mai thei.
• Hormone aldosterone hi a tul angin siam chhuah a ni lo mai thei.
• Chu ai chuan mipa hormone androgens an tih chu a tam lutuk a siam tan ta zawk a ni .

He hormone imbalance hi CAH-a harsatna lo awm zawng zawng bulpui ber a ni.

CAH chi hrang hrang lian ber ber a awm em?

Ni e, CAH hi chi hnih lian tak a awm. Damlo hmuhchhuah zinga 95% chu heng chi hnih ah hian an tla a ni.

1. Classic CAH: Hei hi CAH natna khirh ber leh pawi ber a ni. Pian tirh atanga hriat chhuah a ni tlangpui. Enkawl that loh chuan shock, coma, thihna thlengin a thlen thei a ni. Chuvangin, he natna hi hriat hmasak leh enkawl a pawimawh hle .Hetiang chi hi sub-type pahnih a awm a.

• Salt-wasting CAH: Hei hi CAH chi khat na ber a ni. Chutiang a nih chuan hormone aldosterone hi a tlem hle a ni. Hei hian taksain a chi (sodium) level a tidanglam thei lo. Chuvang chuan chi tam lutuk chu zunah a chhuak thin. Chutih rual chuan cortisol hormone pawh a tlahniam a, hormone androgen pawh a tam lutuk a siam chhuak bawk.
• Simple-virilizing CAH (non-salt-excreting, common type): Hei hi a nasat loh deuh a ni. Hetah hian aldosterone hormone tlakchhamna hi chutiang chuan a na lo. Chuvangin nunna atana hlauhawm symptoms a awm lo. Mahse, cortisol hormone a la hniam a, androgen hormone a la sang hle. Hei hian mipat hmeichhiatna lama hmasawnna nena inzawm harsatna a thlen thin.

2. Nonclassic CAH: Hei hi CAH chi hrang hrang zinga mildest ber a ni. Naupan lai emaw, tleirawl lai emaw, puitling lai emawa hriat chhuah a ni tlangpui. Mi thenkhat chuan symptom engmah an nei lo mai thei. Hetah pawh hian mipat hmeichhiatna lama hmasawnna nena inzawm symptom thenkhat chu androgen hormone siam tam lutuk vang a ni thei.

CAH natna lan chhuahna awm thei te chu engte nge ni?

CAH symptoms hi a chi leh mipa leh hmeichhia a zirin a danglam thei. Hei hi chiang taka hrethiam turin a hnuaia table hi han en ila.

Engvangin nge CAH hi a lo thanglian? Eng nge a chhan?

A awlsam zawngin, CAH hi genetic disease a ni . Chumi awmzia chu kan nu leh pate hnen atanga kan rochun thil a ni tihna a ni. Natna hrik kai theih a ni lo.

Kan taksaa mizia tinrengah hian gene pahnih a awm a, pakhat chu kan nu atanga lo chhuak a ni a, pakhat chu kan pa atanga lo chhuak a ni. CAH a lo awm theih nan naupang chuan an nu leh pa hnen atangin gene chhia chu a rochun a ngai a ni . Hetiang dinhmun hi autosomal recessive disorder an ti a.

CAH hi enzyme 21-hydroxylase tlakchham vang a ni tlangpui. He enzyme code tu gene-a mutation awm vang a ni.

Engtin nge CAH i neih leh neih loh i hriat theih ang?

Classic CAH (a chi na tak) 1.1.

Hei hi chanchin tha ber a ni. Tunah chuan Sri Lanka-a damdawi in tam takah chuan nau piang lai test zingah Classic CAH screening test hi an dah tel tawh a ni. Hei hi naute pian hnu ni engemaw zat hnuah tih a ni a, naute kekawr atanga thisen tlemte lak chhuah hmangin . He test (newborn screening) hian naute hian Classic CAH a nei em tih chu rang takin a hre thei a ni. Chumi awmzia chu natna lan chhuah hmain enkawl tan theih a ni a, naute nunna a chhanhim thei tihna a ni.

Naupang CAH nei tawh chhungkaw pakhatin fa dang an beisei a nih chuan naupai laiin naute chu a dinhmun enfiah theih a ni. Hei hi test bik a awm a, chu chu amniocentesis te hi a ni. Hemi chungchang hi i doctor nen sawiho rawh.

Nonclassic CAH (mild type) a ni a, a rilru a hah lutuk a, a rilru a buai em em bawk a.

He mild form hi hriat a tlai deuh thei a, a chhan chu symptoms hi a hnuah a lo lang thin a ni. Nangmah emaw, i fa emaw chuan a chunga kan sawi tak symptoms te hi i neih tan chuan doctor i pan tur a ni. Doctor chuan:

• Taksa enfiahna (physical examination) neih a ni.
• Thisen test leh zun test hian hormone level a enfiah thin.
• A chang chuan genetic testing hmangin natna hi a finfiah thei a ni.

Hei hi eng enkawlna nge ni?

CAH hi a dam kim thei lo. Mahse, enkawl dan dik tak chuan a thunun tha thei a, nun pangngai tak, hrisel takin i nung thei bawk . Enkawlna chu natna chi hrang hrang leh a lan chhuah nasat danah a innghat a ni.

Classic CAH enkawl dan tur

Heng mite hian an dam chhung zawng nitin damdawi an ei a ngai a ni . Enkawlna tum ber chu taksaa hormone tlakchhamna pek hmanga hormone level inthlauhna siam a ni. Hei hian thanlenna pangngai leh mipat hmeichhiatna lama hmasawnna tur a pui a ni.

• Glucocorticoids: Heng damdawi te hian taksain a siam chhuah loh cortisol hormone a thlak a. Naupangin natna a neih chuan khawsik, natna hrik a neih emaw, operation ang chi thil buaithlak tak a neih hunah emaw heng damdawi te hi tihpun a ngai mai thei.
• Mineralocorticoids: Hengte hi taksaa siam chhuah loh hormone aldosterone thlakna atana pek a ni.
• Salt supplement: Nau piang thar, salt-wasting syndrome nei te chu taksa atanga chi bo tawh thlak nan sodium chloride pek an ni.

Enkawlna dang chu hmeichhe naupangte taksa peng hrang hrang (ambiguous genitalia) siamthatna tur surgery a ni. Hei hi naute pian hnu thla 2 leh thla 6 inkarah tih theih a ni tlangpui.

Nonclassic CAH enkawlna

Hetiang mild form nei te hian symptomatic an nih chuan enkawlna engmah an mamawh lo mai thei. Symptoms na lo tak tak an neih chuan glucocorticoids dose tlemte pek theih an ni. Heng mite hian dam chhung enkawlna an mamawh lo fo thin.

Heng dinhmun eng pawhah hian naupang leh nu leh pate’n an tawrh theih tur stress leh harsatna chungchang sawi turin rilru lam hriselna lama counselling zawng hi enkawlna atana pawimawh tak a ni.

Enkawl loh chuan eng harsatna nge thleng thei?

A bik takin Classic CAH chi tichhe thei chi hi tihfai loh a nih chuan a hlauhawm hle thei. Taksa atanga chi leh tui hloh tam lutuk hian harsatna a thlen thei a, nunna atana hlauhawm pawh a ni thei. Naupang chu a luak nasa hle a, a ke a chhe lo a, a hnute a hnek lo a nih chuan damdawi in emergency treatment unit (ETU)-ah hruai nghal tur a ni.

Enkawl loh chuan eng nge thleng thei:

• Thinlung thawk mumal lo (Arrhythmia) .
• Lung natna (Cardiac arrest) a awm
• Thihna pawh a thleng thei.

Noclassic CAH hian enkawl loh pawhin harsatna a thlen thei. Entirnan, nau pai theih lohna, thlasik hun mumal lo, leh hmeichhiaah mipa mizia awm reng.