I fa vun chu a thim a, a khauh em? Juvenile Systemic Scleroderma chungchang hi han sawi ila!

I fate vun chu a danglam deuh tih i hmu tawh em? Vun chu a hrual deuh tawh a, a tight deuh tawh a, khawih pawhin a danglamin a lang mai thei em? Chutiang thil i hriat chuan tlem chuan i ngaihtuah tur a ni. A chhan chu, hei hi a chang chuan Juvenile Systemic Scleroderma , tunlaia kan sawi tur dinhmun chhinchhiahna a ni thei. Lungngai suh, hemi chungchang hi chipchiar takin kan sawi dawn a ni.

Juvenile Systemic Scleroderma chu eng nge ni?

A awlsam zawngin, Juvenile Systemic Scleroderma ( Systemic Sclerosis tia hriat bawk) hi i fa vun chu a tlangpui aia thim leh rigid zawka awm thin a ni. Rubber phek, zawi zawiin a thim a, plastic khauh ang maia lo awm angin han ngaihtuah teh. Vun chauh a ni lo. A châng chuan, naupang taksa chhûnga taksa peng dangte pawh a nghawng thei a ni. ‘Systemic’ tih thumal hian naupang taksa pum pui a nghawng thei tihna a ni.

Hei hi a takah chuan autoimmune disease a ni . Chu chu kan taksa invenna system, immune system hian a tihsualin a cell hrisel tak takte chu a rawn bei tan tihna a ni. Hetiang Scleroderma-ah hian immune system-in hetiang hian vun cell a beih chuan vun a inflammation emaw swelling emaw a awm thin. Hetianga a hring avang hian kan vun a elasticity leh chakna pe theitu protein collagen an tih chu a tam lutuk tan a ni. Hliam a awm chuan hliam a lo awm ang bawkin, he collagen siam tam lutuk hian fibrosis emaw, vun hliam ang maia khauhna emaw a thlen thin.

Systemic Scleroderma chi hrang hrang lian ber ber a awm em?

Ni e, chi thum a awm. Eng nge an nih i en ang u.

1. Diffuse cutaneous systemic sclerosis: Hetiang chiah hian naupang vun thim zia chu taksa pum puiah hmuh theih a ni. Tin, taksa chhungril peng hrang hrangte hnathawh danah harsatna a awm thei bawk. Ngaihtuah teh, kut, ke leh hmai chauh ni lovin, rilru leh pum ang chi hmunte pawh a thim tan ta a ni.

2. Limited cutaneous systemic sclerosis (CREST syndrome): Hemi hi a bik deuh a ni. CREST syndrome an ti bawk . CREST hi acronym a ni a, he dinhmun langsar ber panga zinga letter hmasa berte inzawmkhawma siam a ni.

• C - Calcinosis: Hei hian vun hnuaiah calcium a awm a, hnah te te a siam tihna a ni.
• R - Raynaud’s phenomenon: Hei hi kut zungtang (kut leh ke) te chu khaw lum emaw, stress hnuaia awm emaw a nih chuan a var, a sen, a rawn sen leh thin hi a ni. Taksa lum leh vawt tihreh a harsa ta hle.
• E - Esophageal motility dysfunction: Chumi awmzia chu kawr atanga chaw kawng (esophagus) thlenga chaw phurtu tube hian kan ei hian hna a thawk tha lo tihna a ni. Hei hian thinlung a ti na thei a ni.Chutiang thil chu a thleng thei a ni.
• S - Sclerodactyly: Kut zungtang vun thim leh khauh. Kut zungtang chu a hring thei a, a nghet thei bawk a, sausage ang maiin.
• T - Telangiectasia: Vun chhunga thisen kalna kawng te te chu a lian a, spider web emaw red dots angin a lang.

3. Systemic sclerosis sine scleroderma: Hei hi taksa chhungril peng hrang hrangte a nghawng a, vun a thim emaw, a khauh emaw awm lovin a nghawng a ni. ‘Sine scleroderma’ tih awmzia chu ‘scleroderma tel lo (vun khauh)’ tihna a ni. Chumi awmzia chu vunah hian danglamna lian tham awm lo mah se, chhungril lam taksa peng hrang hrangte chu a nghawng thei tihna a ni.

Tute nge Juvenile Systemic Scleroderma vei tam ber? Engtiang chiahin nge a hluar?

He Juvenile Systemic Scleroderma tia koh hian kum eng pawh ni se, mipa leh hmeichhia pawh nise a nghawng thei tak zet a ni. Mahse, naupang tê tê, chu chu naupang kum 10 hnuai lam tan chuan a tlem hle. ‘Juvenile’ tih thumal ngei pawh hian hei hi naupangte nghawng thei dinhmun a ni tihna a ni. United States ang ram statistics i en chuan Scleroderma vei naupang 5,000 atanga 7,000 inkar an awm an ti. Scleroderma vei zawng zawng zinga 2% vel chu kum 10 an tlin hmain he natna hian an vei a, 7% dang chuan kum 10 leh 19 inkarah he natna hi an tawk a, chuvangin, hei hi natna tlemte anga ngaih a ni

Juvenile Systemic Scleroderma natna lan chhuah dan chu engte nge ni?

Hetiang dinhmunah hian symptoms hian naupang vun leh chhungril lam a nghawng thei a ni. Chu bakah, Systemic Scleroderma vei naupang tam tak pawhin Raynaud’s phenomenon an tih natna an nei bawk.

Vun leh tissue te nghawng thei symptoms

A hmasain, hei hian vun leh a kaihhnawih tissue te a nghawng dan hi han en ila:

• Vun hian a elasticity a hloh thin. A rough, tight, leh hmuhnawm lo tak a lo ni ta a ni.
• Kut hnathawh a tlahniam. Vun chu a tight deuh a, a bik takin kut zungtang leh kutphah ah te, kut ben leh thil man harsa tak a ni.
• Vunah hian thisen kalna sen a lang a, a bik takin kut, hmai leh nail vel (telangiectasias) ah te a lang thin. Chungte chu rulhut te tak te ang maiin an lang thei.
• Calcinosis chu vun hnuaiah emaw hmun dangah emaw calcium deposit te te te siam a ni. Chûngte chu a châng chuan a harsa thei hle.

A tirah chuan kut leh ke vun chu a hring leh a lianin a lang thei. Hun a kal zel a, naupang vun chu a tight thei a, a thim thei a, a hrual, a pilna hmun, a nih loh leh khur te te a nei thei bawk. Entirnan, naupang thenkhat kut zungtang chu sausage ang maiin a hring thei a, chutah chuan a khauh a, a eng thei bawk.

Internal organs tichhe thei symptoms te

Systemic Scleroderma hian naupang chhungril lam a nghawng thei bawk. A lan chhuah dan chu:

• Joint swelling, stiffness, leh natna te a awm bawk. Arthritis ang maiin.
• Kut zungtang ah ulcer a awm. Heng ulcer te hi a dam theih nan hun rei tak a ngai a ni.
• Digestive system lama harsatna awm thei. Entirnan, thinlung natna, ei harsa, bawlhhlawh chhuak (diarrhea), leh pum na.
• Thluak lama harsatna a awm. I khuh reng emaw, thawk harsa emaw i nei thei.
• Kidney lama harsatna nei. Hei hian thisen sang (hypertension) a thlen thei bawk .
• Chau tih hriat reng reng. Thil tenawm tak tih zawh hnu pawha chau vat.
• Muscle chak lohna.

A tam zawkah chuan he dinhmun hi a na lo. Chuvangin naupang thenkhat chuan Scleroderma an vei tih pawh an hre lo mai thei. Chuvangin naupang Systemic Sclerosis vei nia hriat chuan doctor pan fo tur a ni a, thisen sang, lung, kal, chaw kawng, leh thinlung lama harsatna a neih leh neih loh enfiah a ngai a ni.

Raynaud-a phenomenon chu a ni

Hei hi symptom danglam deuh a ni. Raynaud-a phenomenon chu naupang kut zungtang leh ke ruhte chu khawsik a tawrh emaw, a stress lai emaw a rawng a inthlak hian a ni. A hmasa berin an rawn var a , chutah chuan an rawn sen a , a tawpah chuan an rawn sen leh ta a ni . Hei hi natna lan chhuah tirh lamah hmuh theih a ni. A châng chuan hei hi Systemic Sclerosis a awm tih hriat theihna hmasa ber a ni thei bawk.

Raynaud-a phenomenon nena lo awm thei symptom dangte chu:

• Chau tih hriat reng reng
• Joint natna
• Chaw ei harsa
• Pum a na
• Chest inflammation a awm thin
• Kawthalo
• Thâwk harsa

Engvangin nge Juvenile Systemic Scleroderma hi a lo awm?

Dik tak chuan Juvenile Systemic Scleroderma awm chhan dik tak hi hriat a la ni lo . Mahse, zirchianna atanga a lan dan chuan kan thisen kalna kawng chhunga cell awmte a chhiat avangin vun chhunga connective tissue cell, fibroblast an tih te chu a thawk nasa lutuk a ni. Heng fibroblast te hi collagen ang chi protein siamtu cell te an ni. Chuvangin, Systemic Scleroderma symptoms hi he collagen overproduction vang a ni.

Engvangin nge naute vun hi a tight em em?

Systemic Scleroderma-ah chuan naupang tissue-a protein collagen an tih tam lutuk avangin vun a tight thin. Kan sawi tawh ang khan immune system hna chu taksa hi natna hrik leh natna laka humhim hi a ni. Mahse, Scleroderma vei naupangah chuan immune system hian a overreact a, taksa chu a cell hrisel tak tak hmangin a humhim a tum a – chu chu a cells a bei tihna a ni . Chuvang chuan naupang cell-te chuan collagen tam lutuk an siam tan ta a ni. He collagen tam lutuk hi naupang vun leh chhungril taksa peng hrang hrangah a awm a, a hmel khauh tak, thim tak a siam a, chu chu hliam tuar ang mai a ni.

Engtin nge Systemic Scleroderma hi hmuhchhuah a nih?

I fa doctor chuan thil engemaw zat ngaihtuah chungin Systemic Scleroderma chu a hmuchhuak ang.

• Symptoms leh taksa chhinchhiahna, vun thim leh kut zungtang inthlak danglam te.
• Vun inthlak danglamna bik, a hmun thim, an lian leh te, an pianzia leh an rawng te.
• Medical history kimchang leh taksa enfiahna chuan naupang natna hmasa te leh chhungkaw zingah hetiang dinhmun hi an nei tawh em tih hriat theih a ni ang .

Systemic Scleroderma hi eng test nge hman thin?

Scleroderma ang chi natna dangte tihbo nan te, scleroderma hi engtiang chiahin nge a thawh tih hriat nan te, vun tih loh taksa peng dangte pawhin a nghawng em tih hriat nan test hrang hrang an nei thin. Autoimmune proteins test hian natna kal zel dan tur ngaihtuahna engemaw zat a pe thei a ni. A tlem hle a, doctor-te chuan vun biopsy an ti thin .

Systemic Scleroderma test dang thenkhat chu:

• Thisen test: Autoimmune protein level leh kal hnathawh dan tehna.
• X-ray test: Naupang vun, ruh leh chhungril lam (lung leh intestines ang chi) inthlak danglamna zawng rawh.
• Esophagus-a ei leh in dan enfiahna atana test: Hei hian ka atanga chaw ei tur phurtu tube hnathawh dan en tihna a ni.
• Pulmonary function test: Nausen lungin hna a thawk tha em tih hriatna atana thaw test a ni.
• Heart ultrasound (Echocardiography): I naute thinlung hnathawh dan en rawh.

Juvenile Systemic Scleroderma hi tute nge ni?

Systemic Scleroderma hi i fate general practitioner, heng natna hrang hranga specialist rheumatologist , dermatologist , emaw taksa peng hrang hrang (digestive system, heart, lungs, kidneys)-a specialist doctor team-in an enfiah a, an enkawl a, an enkawl bawk.

Juvenile Systemic Scleroderma hi engtin nge enkawl a nih?

Systemic scleroderma enkawlna tum ber chu inflammation tihtawp te, natna zual zel tur venna te, leh chhungril taksa peng hrang hrang tihchhiat loh te hi a ni.

Vun venhimna

I vun venhim hian i vun leh i taksa peng hrang hrangah thisen a kal tam thei ang berin a pui a ni. Hei hi Raynaud-a phenomenon nei naupangte tan a pawimawh hle . I fa vun venhimna atana i tih theih thenkhat chu hetiang hi a ni:

• A natna hmun, a bik takin kut zungtang leh ke ruhte hliam hi pumpelh tur a ni.Mittui te te pawh hi harsatna lian tak a lo ni thei.
• I fa kut leh ke chu khawsik lakah venghim rawh. Room lum takah dah la. Thlasik lai chuan (kan ram tlangram ang hmun lum zawkah) i fa chu thawmhnaw layer dang, beng khuhna, mittens, leh socks lum tak tak nei kawrfual hak ang chi te nen inthuam rawh. Wool hi cotton emaw synthetic fabric emaw aiin a lum zawk. Tin, thawmhnaw thick pakhat ha ai chuan layer te tak te engemaw zat hak a tha zawk.
• Meizial zu emaw, i naute chu meizuk emaw tih loh tur.
• I fate chu khawsik damdawi, pseudoephedrine tih telna pek loh tur.
• Ni êng lutuk lakah i fa chu humhim rawh. Doctor-in a tih angin sunscreen hmang rawh.
• I fa vunah hian astringents, taksa emaw hmai emaw scrub, leh detergent na tak tak, vun ti vawt thei hmang suh.
• Doctor-in a sawi angin i fa vun a tifai reng theih nan lotion hmang thin ang che.

Taksa lam enkawlna

Basic stretching leh guided exercise program, physical therapist leh occupational therapist-te tih hian naupang chu a insiamrem theihna tur, a ruhkawr a che theihna tur range, a taksa ruh chakna leh a natna hmuna thisen kal theihna tur a vawng reng thei a ni. Heng enkawlna te hian ruh inzawmna (joint contracture) a veng thei bawk a, chu chu ruh (joint) a tikhauh (stiffening) a ni. A tul chuan doctor chuan splints emaw braces emaw hman a rawt thei bawk.

Inzai

A tlem hle a, ruh emaw, vun natna emaw, hliam na tak emaw siam\hat nan kut ruh (orthopaedic hand surgery) emaw, cosmetic surgery emaw neih a ngai mai thei. Mahse, operation hma chuan condition chu remission -ah a awm tur a ni a , chu chu kum engemaw zat chhung chu inactive a ni tihna a ni.

I fa doctor chuan autologous bone marrow transplantation chu enkawlna atan a ngai mai thei. Tunah hian enkawl dan dang pawh zirchian mek a ni.

Systemic Scleroderma tan hian eng damdawi nge pek thin?

I doctor chuan i fa dinhmun enkawl nan damdawi chi hrang hrang a pe thei ang. Entir nan:

• Corticosteroids: Taksa, ruh leh vun a inflammation a tihziaawm. Steroid hian taksa peng hrang hranga inflammation hmasa ber enkawlnaah pawh a pui thei bawk. Mahse, systemic sclerosis fibrotic stage hnuhnung zawkah chuan steroid hi a hlawk vak lo tlangpui.
• Non-steroidal anti-inflammatory drugs (NSAIDs): Entirnan , ibuprofen leh naproxen teNSAID ang damdawi hi a chang chuan arthritis vei naupangte ruh natna tihziaawmna atan an hmang thin. Mahse, kal hnathawh a chhiat hunah NSAID hman tam lutuk lo turin fimkhur a ngai.
• Immune modulator damdawi dang: Hengte hi autoimmune response tihtlem nan leh inflammation leh a hnu lama scarring awm lo turin pek thin a ni.
• Thisen kalna kawng tizau thei leh thisen kal dan tichaktu damdawi: Hengte hi Raynaud-a phenomenon enkawlna atan hman a ni .

Ka fa hian Systemic Scleroderma a vei a nih chuan eng nge ka beisei ang?

Scleroderma hi damdawi chiang tak a awm lo. Mahse, i fa chu a dinhmun enkawl leh nunpui turin i pui thei a ni.

Systemic Scleroderma hi natna rei tak , rei tak, leh zawi zawiin a lo zual zel a ni. Thla tam tak emaw, kum tam tak emaw a daih thei. I fa thlir dan chu a natna lan chhuah dan a zau zia leh taksa (a bik takin vun leh chhungril lam) a nghawng nasat danah a innghat a ni.

Naupang lung, thinlung emaw, kal lama harsatna nei te chu harsatna, ruh inzawmna leh cosmetic inthlak danglamna te an nei tam zawk. Hetiang dinhmun hian naupang ruh thanna a nghawng thei bawk.

Systemic Scleroderma hi a bo vek lo fo thin a, mahse kum engemaw zat chu a level ang chiah khan a na zual lo thei a ni.

Juvenile Systemic Scleroderma hi eng chen nge i dam theih ang?

Systemic Scleroderma vei hmuhchhuah naupang dam rei zawng hi a natna nasat dan azirin a inang lo a, a bik takin an chhungril taksa peng hrang hrang a nghawng em tih a zirin a inang lo. Hemi chungchang hi i doctor nena inbiakna neih a pawimawh a, hriatthiamna chiang tak i neih theih nan.

Juvenile Systemic Scleroderma hi ven theih a ni em?

A chhan dik tak hriat a nih loh avangin Systemic Scleroderma ven dan tur chiang tak a awm lo.

Ka fa Systemic Scleroderma vei nia hriat chu engtin nge ka enkawl ang?

Systemic Scleroderma vei naupangte chu a pangngai thei ang bera nun an hmang tur a ni. Sikul kal te, inkhelh te, thiltih hrang hranga tel te an ni tur a ni. A tlangpuiin naupangte taksa tihchak theih zat (a him phawt chuan) tihkhawtlai a awm lo. Exercise hian chak lohna a veng a, taksa chakna, inthlak danglam theihna leh tuar theihna a tipung bawk.

Engtikah nge ka fa chu doctor hnenah ka hruai ang?

I fa chu regular checkup turin hruai tur a ni. A bik takin internal organs tichhe thei symptoms te chu fimkhur rawh. I fa chu natna nasa tak a tuar a nih chuan , a taksa peng hrang hrang a tihchet harsat chuan, a nih loh leh a nitin nun tichhe thei symptoms a neih chuan, .Doctor pan nghal rawh.

Doctor hnenah eng zawhna nge ka zawh ang?

Hetiang zawhna zawh hian a pui ang che:

• Ka fa dinhmun hi engtiang chiahin nge a pawi?
• Engtin nge in lama ka fa vun venhimna ka puih theih ang?
• Ka fa hi eng chen nge physical therapy a kal tur?
• Natna a zual zel chuan eng symptom nge ka fimkhur ang?
• Ka fa hian operation a mamawh em?

Scleroderma chi dang a awm em?

Systemic Scleroderma chi hrang hrang bakah hian Scleroderma chi hnih a awm a. Anni chu:

• Localized scleroderma: Hetiang chi hian taksa hmun thenkhatah vun chauh a nghawng a. A hnuaia taksa ruh emaw ruh emaw-ah pawh a darh thei a, a awm lo thei bawk. Internal organs a nghawng tlem hle.
• Systemic scleroderma (systemic sclerosis tia hriat bawk): Hei hi he thuziak chhung zawnga kan sawi tawh chu a ni. Hetiang dinhmunah hian vun hi taksa khawi hmunah pawh a thim thei a ni. Vun inthlak danglamna bakah hian taksa peng hrang hrang, kal, thinlung, lung leh chaw kawng (gastrointestinal system)-ah te scar tissue a lo awm thei bawk. Systemic sclerosis vei naupangte hian vun inthlak danglamna nasa tak avangin an ruh (joint movement) a tlem thei hle.

He thuziaka i hriat reng tur pawimawh ber berte

Juvenile Systemic Scleroderma hi naupangte zingah a awm lo hle a, vun a ti thim a, a tikhauh a, a chang chuan chhungril taksa peng hrang hrang a nghawng bawk. Autoimmune natna a ni.

• A lan chhuah dan: Vun a tikhauh, kut zungtang a hring, Raynaud-a phenomenon (khaw lum laiin kut zungtang rawng a inthlak), ruh na, chaw ei lama harsatna, leh thawk harsatna te a awm thei.
• A chhan: Hriat chian chi ni lo mah se, taksa hriselna (immune system) tha lo leh collagen siam tam lutuk vang nia ngaih a ni.
• Enkawl dan: Natna thunun, vun venhim, physical therapy leh a tul chuan damdawi hmang. Damdawi famkim awm lo mah se, a natna lan chhuah dan chu enkawl theih a ni.
• A pawimawh ber: I fain natna lan chhuah dan a neih chuan damdawi lam thurawn zawng nghal rawh. I fate chu medical checkup neih fo turin refer a pawimawh hle a, nun pangngaia an awm theih nan tanpui a pawimawh hle. Lungngai suh, he dinhmun hi Doctor te puihnain i tuar thei a ni.

` Scleroderma, naupang vun natna, vun tikhauh, collagen, autoimmune natna, Raynaud-a phenomenon, CREST syndrome