Tixraaca Pierre Robin

Tixraaca Pierre Robin (ama cilladda) waa xaalad uu ilmuhu yaryahay daanka hoose ee caadiga ah, carrabka oo hunguriga dib ugu dhaca, iyo neefsashada oo dhib ku ah. Waxay joogtaa dhalashada.

Sababaha saxda ah ee isku xigxiga Pierre Robin lama garanayo. Waxay ka mid noqon kartaa cilladaha hiddo-wadaha badan.

Daanka hoose ayaa si tartiib ah u soo baxa dhalashada ka hor, laakiin waxaa laga yaabaa inuu si dhakhso leh u koro sannadaha ugu horreeya nolosha.

Calaamadaha xaaladdan waxaa ka mid ah:

• Kala jeexan
• Dhadhan-sare
• Sanka oo aad u yar yar oo garka yar leh
• Sanka dhabarka cunaha ugu jira
• Infekshannada dhegaha ee soo noqnoqda
• Furitaanka yar ee saqafka sare ee afka, oo sababi kara ceejis ama cabitaanno dib uga soo baxa sanka
• Ilkaha soo baxa marka ilmuhu dhasho
• Carrabka weyn oo la barbar dhigo daanka

Bixiye daryeel caafimaad ayaa badanaa ogaan kara xaaladan inta lagu jiro baaritaanka jirka. La-tashiga khabiir ku takhasusay hidde-wadaha ayaa meesha ka saari kara dhibaatooyinka kale ee la xiriira cilladan.

Kala hadal dhakhtarka ilmahaaga jagooyinka hurdada ee aaminka ah. Dhallaanka qaarkood ee leh taxanaha Pierre-Robin waxay u baahan yihiin inay seexdaan calooshooda halkii ay dhabarka ka seexan lahaayeen si looga hortago in carrabkoodu dib ugu soo laabto hawo-mareenkooda.

Xaaladaha dhexdhexaadka ah, canugga wuxuu u baahan doonaa in tuubo la galiyo sanka iyo marinka hawada si looga fogaado xannibaadda marin-haweedka. Xaaladaha daran, qalliin ayaa loo baahan yahay si looga hortago xannibaadda hawo-mareenka sare. Carruurta qaarkood waxay u baahan yihiin qalliin si ay uga samaystaan ​​dalool hawo-mareenkooda ama ay u dhaqaajiyaan daamankooda hore.

Quudinta waa in si taxaddar leh loo sameeyaa si looga fogaado ku mergasho iyo cabitaanno dareeraha ah ee hawo-mareenka. Ilmuhu waxaa laga yaabaa inuu u baahdo in lagu quudiyo tuubo si looga hortago ceejinta.

Kheyraadka soo socda ayaa ku siin kara macluumaad dheeraad ah oo ku saabsan taxanaha Pierre Robin:

• Cilmi-baarista Cilladaha Dhalashada ee Carruurta - www.birthdefects.org/pierre-robin-syndrome
• Hay'adda Cleft Palate Foundation - www.cleftline.org
• Ururka Qaranka ee Dhibaatooyinka Nadir --rarediseases.org/rare-diseases/pierre-robin-sequence

Cuncunista iyo dhibaatooyinka quudintu waxay iskood iskaga bixi doonaan sannadaha ugu horreeya maadaama daanka hoose uu u weynaanayo cabir caadi ah. Khatar sare ayaa jirta oo dhibaatooyin ah haddii marin-haweedka ilmaha aan laga ilaalin inuu xannibo.

Dhibaatooyinkan ayaa dhici kara:

• Dhibaatooyinka neefsashada, gaar ahaan marka ilmuhu seexdo
• Qaybaha ceejinta
• Wadnaha oo hawl gab ah
• Dhimasho
• Dhibaatooyinka quudinta
• Ogsijiin dhiig oo hooseysa iyo dhaawaca maskaxda (neefsashada oo ku adkaata darteed)
• Nooca dhiig karka ee loo yaqaan sambabada dhiig karka

Ilmaha ku dhasha xaaladan waxaa badanaa laga helaa dhalashada.

Wac daryeel bixiyahaaga haddii cunugaaga uu qabo ceejis dhacdooyin ah ama dhibaatooyin neefsashada ah. Xirnaanshaha marin-haweedka ayaa sababi kara buuq sare marka ilmuhu neefsanayo. Waxay kaloo u horseedi kartaa maqaarka oo midab noqda (cyanosis).

Sidoo kale wac haddii ilmahaagu qabo dhibaatooyin kale oo xagga neefsashada ah.

Ma jiro ka hortag la yaqaan. Daaweyntu waxay yareyn kartaa dhibaatooyinka neefsashada iyo ceejinta.

Pierre Robin syndrome; Dhismaha Pierre Robin; Pierre Robin anomaly

• Dhallaanka adag ee jilicsan

Dhar V. Syndromes leh muujinta afka. Gudaha: Kliegman RM, Stanton BF, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Buugga Buugga Caafimaadka Caruurta. 21aad ed. Philadelphia, PA: Elsevier; 2020: cutubka 337.

Purnell CA, Gosain AK. Tixraaca Pierre Robin Gudaha: Rodriguez ED, Losee JE, Neligan PC, eds. Qalliinka caaga ah: Volume Three: Craniofacial, Madaxa iyo Qalliinka Neck iyo Qalliinka caaga carruurta. 4aad. Philadelphia, PA: Elsevier; 2018: cutubka 36.