Rubutun Pierre Robin (ko ciwo) wani yanayi ne wanda zai haifar da haɗuwa da siffofin da suke ciki a lokacin haihuwar su:
- wani ƙananan ƙananan ƙaƙƙarfan ƙananan ƙaƙƙarfan ƙaƙa (wannan ake kira micrognathia)
- gwangwadon ƙuƙwalwa tare da bambancin da ba shi da launi, yawanci dawaki ko U-dimbin yawa
- wani babban arched palate
- wani harshe da aka mayar da shi a cikin kuturu kuma zai iya hana jirgin sama da ke haifar da numfashi na numfashi (glossoptosis)
- a cikin kimanin kashi 10 cikin dari na macroglossia (babban harshe mai mahimmanci), ko ankyloglossia (harshe na harshe) na iya kasancewa
- hakora a lokacin haihuwar juna da kuma malformationn hakori
- m kunne sau da yawa
- magungunan kwayoyin halitta (kashi 75 cikin dari)
- Rushewar sauraro (kashi 60 cikin dari - yawancin hali)
- waje canjin atresia (rare)
- wasu kunnuwan kunnuwa da suka hada da abubuwan da ke kunshe a cikin ƙasushin kunnuwan ciki, magungunan semicircular , da kuma suturar kayan aiki
- ƙananan nakasar (rare)
Wadannan abubuwan haɗari a lokacin haihuwar suna haifar da matsalolin maganganu a yara tare da jerin Pierre Robin. A kashi 10-85 bisa dari na lokuta wasu bayyanarwar tsarin na iya faruwa ciki har da:
- Matsalar ido (hypermetropia, myopia, astigmatism, corneal sclerosis, nasolacrimal duct stenosis)
- An ladafta matsalolin kwakwalwa a cikin kashi 5-58 na lokuta (jinin zuciya na zuciya, lactus arteriosis, patent foramen ovale, gurguntacciyar ƙetare, da hawan jini)
- Ana iya lura da matsalolin muskotkeletal sau da yawa (kashi 70 zuwa 80 na lokuta) kuma zasu iya haɗawa da sutura, polydactyly, clinodactyly, da oligodactyly, kulob din, abubuwan da suka dace, wadanda suka hada da hanzarin hanzari, gajiyar gwiwoyi, scoliosis, kyphosis, lordosis, da sauran mawuyacin spine
- Abubuwa masu ban sha'awa a cikin tsarin kulawa na tsakiya suna lura da kimanin kashi 50% na lokuta kuma zasu iya haɗawa da: jinkirin raya kasa, jinkirta magana, hypotonia, da hydrocephalus.
- Kuskuren kwayar cutar ya fi sauki amma zai iya haɗawa da gwaji marasa lafiya, hydronephrosis ko hydrocele.
Yanayin da Pierre Robin ya faru shine kusan 1 a cikin haihuwar 8500, yana daidai da maza da mata daidai. Rubutun Pierre Robin zai iya faruwa a ciki kuma na kansa amma yana hade da wasu wasu kwayoyin halitta ciki har da: Ciwon ƙwayar cuta, Ciwo na CHARGE, Ciwo na Shprintzen, Ciwo na Mobius, ciwo na 18, ciwo na 11q, maye gurbin ciwo 4q, da sauransu.
Akwai ka'idodi guda uku game da abin da ke haifar da rubutun Pierre Robin. Na farko shi ne cewa hypoplasia mandibular ya faru a lokacin makon bakwai na 7 na ciki. Wannan yana haifar da haɓakaccen harshe wanda ya rage a cikin ɓangaren murya wanda ya hana rufe ƙullon ɗalibai da kuma haifar da fadin U-shaped. Rabin rage yawan ruwa mai ruwa zai iya zama factor.
Magana ta biyu shine cewa akwai jinkiri a cikin cigaban yanayin da ke cikin harshe na harshe, ginshiƙan pharyngeal, da kuma fadin tare da jinkirta a hawan ƙwayar magunguna. Wannan ka'idar ta bayyana dalilin da yasa yawancin cututtuka suka yanke game da kimanin shekaru 6.
Na uku ka'idar ita ce babbar matsala ta faru a lokacin ci gaban da ke haifar da hawan rhombencephalus (kakanan baya - ɓangaren kwakwalwa da ke dauke da kwakwalwa da kuma cerebellum).
Babu magani don rubutun Pierre Robin. Gudanar da yanayin ya shafi kasancewa da alamun bayyanar mutum. A mafi yawancin lokuta ƙananan jaw na tsiro a hanzari a farkon shekara ta rayuwa kuma yawancin yakan nuna al'ada game da nau'o'in digiri. Hanyoyin halitta sukan shawo kan matsalolin na numfashi (iska) wanda zai iya kasancewa. Wani lokaci wani jirgin ruwa na wucin gadi (kamar na hawan nasophargyngeal ko na baki) ya kamata a yi amfani dashi tsawon lokaci. Dole ne a gyara gyaran gyare-gyaren gyare-gyare a hankali kamar yadda zai iya haifar da matsaloli tare da ciyarwa ko numfashi. Yawancin yara da Pierre Robin jerin zasu buƙaci maganin maganganu.
Girman rubutun Pierre Robin ya bambanta sosai a tsakanin mutane kamar yadda wasu mutane kawai suna da alamun bayyanar da ke cikin wannan yanayin yayin da wasu zasu iya samun alamun alaƙa da yawa.
Tsarin zuciya ko jijiyoyin zuciya na tsakiya na iya zama da wuya a gudanar da su fiye da wasu abubuwanda ke haɗaka da craniofacial da aka haɗa tare da Pierre Robin. Nazarin ya nuna cewa zancen Pierre Robin ya ɓace, (lokacin da yanayin ya faru ba tare da wani ciwo mai haɗuwa) ba, yakan karu yawan haɗari ga mace, musamman ma inda cutar zuciya ko na tsakiya ba ta samuwa.
> Sources:
> Shafin Fassarar Fassara. Pierre Robin Tsarin. http://www.cleftline.org/parents-individuals/publications/pierre-robin-sequence/
> Ƙarin Madauki. Pierre Robin jerin. https://www.nlm.nih.gov/medlineplus/ency/article/001607.htm
> Medscape. Pierre Robin Syndrome. http://emedicine.medscape.com/article/844143-overview#a4