Von Hippel-Lindau Syndrome (VHL) hi eng nge ni? A awlsam zawngin sawi ila.

Von Hippel-Lindau Syndrome hi i hre tawh em? I la nei lo mai thei. A buaithlak deuhin a lang thei a, mahse genetic condition hmuh tur awm lo tak a ni. A awlsam zawngin, VHL an tih gene-a mutation a awm chuan taksa hmun hrang hrangah tumor leh fluid-filled cyst a siam a. He thu i hriat hian hlauthawng suh. Fiah tak leh awlsam takin sawi ila.

VHL syndrome hi eng nge ni tak tak?

VHL hi kan genes inthlak danglamna avanga lo awm a ni. Hei hian i taksa peng hrang hrang a thlen thei a, entirnan, .

• Mit
• Thluak
• Spinal cord a ni
• Beng chhung lam
• Adrenal glands a awm a
• Pancreas a ni
• Kidney a ni
• Nau neihna taksa peng hrang hrang

Tumor hi hmun hrang hrangah a lo awm thei a, chu chu...

A tha ber chu heng growth tam zawk hi benign an ni . Chu chu cancer vei an ni lo tihna a ni. Mahse, hetiang natna vei mi chu midang aiin kal leh pancreatic cancer vei theihna a sang deuh hlek a ni. Chuvang chuan hei hi hriat chian a pawimawh hle.

Engvangin nge he VHL syndrome hi a lo awm?

A tlangpuiin kan taksaa VHL gene hian cell te chu thunun theih loha inthen darh turin a veng a, tumor thanna a control thin. Kan taksaah vengtu ang mai a ni. Mahse VHL syndrome nei mi pakhatah chuan he gene hi a inthlak emaw, a danglam emaw a ni. Tichuan chu vengtu hna chu a thawk tha lo. Chuvang chuan cell pangngai lote chu thunun theih lohvin an thang lian a, tumor an siam tan thin.

Hetiang dinhmun hi a thlen theih dan tlangpui pahnih a awm a:

1. Nu leh pa hnen atanga rochun: VHL vei mi 10 zinga 8 velin an nu emaw, an pa emaw hnen atangin an rochun thin. Autosomal dominant pattern-a rochun a ni a, chu chu nu leh pa pakhatin he natna hi an neih chuan an fate pawhin an rochun theihna chance 50% an nei tihna a ni.

2. Random occurrence: Mi 10 zinga 2 velah chuan hei hi nu leh pa atanga rochun thil a ni lo. Hetiang dinhmun hi embryo anga a lo pian tirh lamah genes random change ( spontaneous mutation) vang a ni thei.

VHL natna lanchhuahna chu engte nge ni?

VHL natna lan chhuah dan hi mi hrang hrangah a inang lo thei hle. Hei hi a chhan chu i taksaa tumor awmna hmunah a symptoms a innghat a ni. Mi thenkhat chu kum tam tak chhung chu symptom nei lovin an kal thei.

Kum 26 vel atanga natna lan chhuah tan fo mahse, mi thenkhat chuan kum 65 an tlin thlengin natna lanchhuahna lian tham an nei lo thei a, natna lan chhuah tanna kum pawh hi tumor chi hrang hrang a zirin a inang lo thei bawk. Entir nan:

• Mit natna (retinal hemangioblastomas): Kum 12-25 inkar
• Thluak emaw, ruhro emaw a thisen kalna kawng tumor (hemangioblastomas): Kum 18-35 inkar
• Kidney tumor emaw cancer (renal cell): kum 25-50 inkar
• Inner ear tumors (endolymphatic sac tumors): kum 24-35 inkar a ni

Engtin nge VHL hi hmuhchhuah a nih?

I chhungkuaa mi tu emaw chuan he natna hi a neih chuan, emaw, i natna lan chhuah danin i doctor-in VHL i vei nia a rinhlelh chuan, genetic test turin a refer ang che . Hei hi thisen sample hmanga tih a ni. Hei hi VHL gene-a inthlak danglamna i neih leh neih loh hriat chian theihna awmchhun a ni.

Doctor-in VHL a rinhlelh theihna hmun tlangpui chu:

• Kum naupan lai atanga mit natna (eye hemangioblastoma) hmuhchhuah.
• Kum 40 a tlin hmain kidney cancer a ni.
• Thluak emaw, ruhro emaw-ah tumor (hemangioblastomas) tam tak neih.
• Kidney emaw pancreas emaw-ah tumor emaw cyst tam tak neih.

VHL i vei tih hmuhchhuah a nih chuan a dawt leh tur pawimawh ber chu active surveillance a ni.

Active Surveillance tih hi eng nge ni?

Hei hi thil lian tak angin a lang thei a, mahse a awlsam zawngin sawi ila, symptom i nei lo a nih pawhin i medical team chuan an lo enkawl reng ang che tihna a ni. Chumi awmzia chu tumor thar a lo awm emaw, a hlui a lian emaw chuan rang takin hriat theih a ni a, a tul anga enkawl tan theih a ni. Hei hi VHL enkawlna thinlung a ni.

Heng test te hi i kum a zirin a danglam thin.

• Naupan lai (kum 1-4) atang): Thla 6 atanga thla 12 danah mit lam doctor hnenah i mit enfiah tir thin ang che. Kum 2 hnuah kum tin i thisen sang enfiah tir thin ang che.
• Naupan lai (kum 5-10): Mit enfiah chhunzawm zel a ni. Tin, zun emaw thisen test (metanephrines) hmangin adrenal gland tumor (pheochromocytomas) a awm leh awm loh enfiah thin a ni bawk.
• Tleirawl (kum 11 atanga): Kum engemaw zat danah MRI scan hmangin thluak leh ruhro (spinal cord) en thin a ni. Hearing pawh an check thin.
• Puitling (kum 15 atanga): Kum hnih danah pumpui MRI scan hmangin kal, pancreas leh adrenal glands te enfiah thin a ni.

I doctor chuan he schedule hi i duh angin a siam ang.

VHL enkawl dan tur chu engte nge ni?

VHL enkawl dan hi tumor chi, a lian leh a awmna hmunah a innghat a ni. Tumor chu cancer ni lo, te, leh benign a nih chuan enkawlna engmah awm lovin enfiahna a tawk thei.

Enkawl dan tlangpui engemaw zat a awm a:

1. Damdawi: Tun hnaia damdawi an rawn siam belzutifan (Welireg) hian VHL kaihhnawih natna hrik (kidney cancer, hemangioblastomas) engemaw zat tihtlem leh darh zel tur tihtawp kawngah a hlawhtling hle.

2. Surgery: VHL enkawl dan tlangpui chu surgery a ni. Cyst natna lanchhuahna thlentu, a lo thang lian emaw, cancer chhinchhiahna lantir mek emaw chu operation hmangin an paih chhuak thin.

3. Radiation therapy: Tumor tihchhiat nan high-energy rays hman a ni. Hei hi a bik takin thluak leh beng chhunga tumor thenkhat lo awm tan chuan hman a ni.

4. Enkawlna dang: Tunlai hian kidney cancer enkawlna a awm a, chungte chu Ablation (khaw lum lutuk emaw, khaw lum lutuk emaw hmanga tumor tihchhiat) leh Immunotherapy te hi a ni.

I medical team chuan eng enkawlna nge i tan a tha ber tih an rel ang.

VHL leh rilru hahdamna nena khawsa

VHL ang chi natna hlauhawm tak i nei tih i hriat hian hlauhna, lungkhamna leh buaina i neih hi thil pangngai a ni. Scan neih tura inbuatsaih leh result nghah lai hian stress ("scanxiety") hi a sang hle.

Hetiang natna nei hian nun hrisel leh hlim tak nei turin thil engemaw zat i tih theih a awm a:

• Ei leh in hrisel: Thlai, thei, sa thau lo, leh sangha ang chi protein tam tak awmna ei leh in inthlau tak ei rawh. Meizial zuk loh vek tur a ni.
• Exercise: Nitin kea kal ang chi exercise awlsam tak hian stress a tiziaawm thei a, i taksa a ti hrisel thei bawk.
• Rilru hahdamna: Yoga, meditation, etc. tih ang chi thil ti la, rilru thlamuanna thlen thei thil (lehkhabu tha chhiar, hla ngaihthlak ang chi) atan hun siam rawh.
• Ṭanpuina dîl rawh: Hemi chungchâng hi i chhûngte leh i ṭhian hnai tak takte nên sawipui rawh. I rilru put hmang sawi rawh. Ṭanpuina i mamawh a nih chuan (Doctor hmuh, homework-a ṭanpui) chu sawi hlau suh.
• I medical team ring rawh: I doctor hnenah zawhna emaw, ngaihtuahna emaw i neih chuan zawt rawh. I symptoms leh test result te journal-a ziah hi a \angkai hle bawk.