Ulandelelwano lukaPeter Robin (okanye i-syndrome) yimeko engumntwana obangela ukudibanisa kwezi zinto ezilandelayo ngexesha lokuzalwa:
- umhlathi omncinane ongaphantsi ophuculweyo (oku kuthiwa yi-micrognathia)
- i-palate i-palate ngokungafani kakuhle komlomo we-cleft, ngokuqhelekileyo i-horsehoe okanye i-U
- i palate ephakamileyo
- ulwimi olubekwe kude kakhulu emqaleni kwaye lunokubangela ukuba umoya ophefumulayo uphefumle (i-glossoptosis)
- malunga neepesenti ezi-10-15 zamatyala ama-macroglossia (ulwimi oluqhelekanga), okanye i- ankyloglossia (ulwimi lwesikolo)
- amazinyo akhona ngexesha lokuzalwa kunye nokungasebenzi kwamazinyo
- izifo zentloko rhoqo
- izikhalazo ze-auricular (ama-75 ekhulwini amatyala)
- ukulahlekelwa kwindlebe (ama-60 ekhulwini amatyala - ngokuqhelekileyo aqhuba)
- ingqungquthela yangaphandle yokucocwa kweengqungquthela (atresia)
- ezinye iimpazamo zomlomo ezibandakanya ukungavumeleki kwamathambo endlebe, ngaphakathi kwimijelo yamachiza kunye ne-vestibular aqueduct
- ukuguqulwa kwamanzi (okungaqhelekanga)
Ezi ziqhelo ngexesha lokuzalwa zihlala zikhokelela kwiingxaki zokuthetha kubantwana abanokulandelelana kukaParob Robin. Kwimizuzu eyi-10-85 yamatyala eminye imbonakalo yenkqubo ingenzeka kuquka:
- Iingxaki zamehlo (i-hypermetropia, i-myopia, i-astigmatism, i-corneal sclerosis, i-dome stenosis)
- Iingxaki ze-cardiovascular ziye zabhalwa kwi-5-58 ekhulwini yamatyala (ukukhathazeka kwenhliziyo, i-patent ductus arteriosis, i-patent foramen ovale, i-pathology septal defect, kunye ne-pulmonary hypertension)
- Iingxaki zeMisculoskelet ziqaphelwa rhoqo (ama-70-80 ekhulwini amatyala) kwaye zibandakanya i-syndactyly, i-polydactyly, i-clinodactyly, ne-oligodacly, i-clubfeet, i-hypothextensible joints, i-anomalies ye-hip, i-knee anomalies, i-scoliosis, i-kyphosis, i-lordosis kunye nezinye izinto ezingaqhelekanga
- Ukungaqhelekanga kwinkqubo yesantya ephakathi kubonakala malunga nama-50% amacala kwaye ingaquka: ukulibaziseka kokuphuhliswa, ukulibaziseka kwenkulumo, i-hypotonia kunye ne-hydrocephalus.
- Iziphene ze-Genitourinary ziqabile kodwa zingabandakanya ii-testes ezingavumelekanga, i-hydronephrosis okanye i-hydrocele.
Iziganeko zePeter Robin ngokulandelelana zi malunga ne-1 ngo-8500 zokuzalwa, ezichaphazela amadoda nabesifazane ngokulinganayo. Ulandelelwano lukaPierre Robin lunokuthi luvele kwaye luqulethe kodwa luhambelana nenani leemeko zemizimba kubandakanya: I-Stickler syndrome, i-CHARGE syndrome, isifo se-Shprintzen, i-Mobius syndrome, i-trisomy 18 syndrome, i-trisomy 11q syndrome, isusa i-4q syndrome kunye nabanye.
Kukho iingcamango ezintathu malunga noko kubangelwa ulandelelwano lukaPeter Robin. Eyokuqala kukuba i-mandibular hypoplasia iqhubeka ngeveki yesi-7-11 yokukhulelwa. Oku kubangela ukuba ulwimi luhlale luphakamileyo emlonyeni womlomo luvimbela ukuvalwa kwamashalofu ase-palatal nokudala i-U-shaped cleft palate. Ukunciphisa inani le-amniotic fluid kungaba yinto.
Ingcamango yesibini kukuba kukho ukulibaziseka ekuphuhlisweni kwengqondo kweelwimi, iintsika ze-pharyangeal, kunye nenaliti ehamba kunye nokulibaziseka kwi-hypoglossal nerve conduction. Le ngcamango ichaza ukuba kutheni iimpawu ezininzi zisombulula malunga neminyaka engama-6 ubudala.
Ingongoma yesithathu kukuba ingxaki enkulu iyenzeka ngexesha lophuhliso oluphumela kwi-dysneurulation ye-rhombencephalus (i-hindbrain-isahlulo seengqondo eziqukethe ingqondo kunye ne-cerebellum).
Akukho pheko lokulandelelana kukaParob Robin. Ulawulo lwalo mqathango lubandakanya ukunyanga iimpawu zomntu ngamnye. Kwimeko ezininzi umhlathi ophantsi ukhula ngokukhawuleza kunyaka wokuqala wobomi kwaye ngokuqhelekileyo ubonakala uqhelekileyo malunga ne-nursergarten. Ukukhula ngokwemvelo kudla ukuphilisa naluphi na iingxaki zokuphefumula (ezindizayo) ezikhoyo. Ngamanye amaxesha inqwelo-moya yomhlaba (njenge-nasophargyngeal okanye yomlomo) kufuneka isetyenziswe ixesha elide. I-flaft palate kufuneka ilungiswe ngokugqithiseleyo njengoko ingabangela iingxaki ngokutya okanye ukuphefumla. Abantwana abaninzi abanokulandelelana kwePeter Robin baza kufuna unyango lwentetho.
Uxinzelelo lukaPeter Robin lulandelelana kakhulu phakathi kwabantu njengoko abanye abantu banokuba neempawu ezinxulumene nale meko ngelixa abanye banokuba nempawu ezininzi.
Iimpawu zesifo se-Cardiovascular okanye eziphakathi kwamanzi zinokuba nzima ngakumbi ukulawula ngaphezu kwezinye izinto eziqhelekileyo ezinxulumene noPet Rob Rob. Ucwaningo luye lwabonisa ukuba ulandelelaniso lukaParob Robin, (xa imeko ivela ngaphandle kwesinye i-syndrome ehambelana nayo), ayinakwandisa ukwanda komngcipheko wokufa, ngakumbi apho iingxaki zesifo senhliziyo okanye zesistim ezingekho.
> Imithombo:
> I-Cleft Palate Foundation. Ulungelelaniso lukaParob Robin. http://www.cleftline.org/parents-individuals/publications/pierre-robin-sequence/
> Medline Plus. Ulandelelwano lukaParob Robin. https://www.nlm.nih.gov/medlineplus/ency/article/001607.htm
> Medscape. Pierre Robin Syndrome. http://emedicine.medscape.com/article/844143-overview#a4