Pierre Robin kuenzanisa (kana chirwere) chirwere chekuzvarwa chinokonzera kusanganiswa kwezvinhu zvinotevera zviripo panguva yekuzvarwa:
- imwe tsanga shoma shoma shoma (iyi inonzi micrognathia)
- cleft palate pamwe nekushayikwa kwakasiyana kwekunyemura, kazhinji kahovha kana kuti akafanana neU
- a high arched palate
- rurimi rwunoiswa kure kwazvo mumutsipa uye rinogona kudzivisa mhepo inokonzera kuoma kufema (glossoptosis)
- mune anenge 10 kusvika ku15 kubva pamatambudziko macroglossia (rurimi rukuru rusingawaniki), kana ankyloglossia (tongue tie) inogona kuvapo
- meno aripo panguva yekuberekwa uye mazino asina mazino
- kazhinji nzeve dzinotapukira
- auricular anomalies (75 muzana yematambudziko)
- kurasikirwa kwekunzwa (60 muzana yematambudziko - kazhinji conductive)
- kunze kwekuongorora chengetari kanonzi atresia (asingasviki)
- dzimwe nzeve dzisina kusimba dzinosanganisira kusagadzikana mukati memapfupa ekunze , mukati memvura miviri , uye mumvura
- manal deformities (rare)
Izvi zvisinganzwisisiki panguva yekuberekwa zvinotungamirira kumatambudziko ekutaura kuvana vane Pierre Robin. Muna 10-85 muzana yezviitiko zvimwe zviratidzo zvinoratidzika zvinogona kuitika kusanganisira:
- Matambudziko eziso (hypermetropia, myopia, astigmatism, corneal sclerosis, nasolacrimal duct stenosis)
- Zvinetso zvepfungwa zvakanyorwa mu5-58 muzana yematambudziko (benign mwoyo murmur, patent ductus arteriosis, patent foramen ovale, marital septal defect, uye pulmonary hypertension)
- Zvinetso zveMusculoskeletal zvinowanzoonekwa (70-80 muzana yematambudziko) uye zvinogona kusanganisira syndactyly, polydactyly, clinodactyly, uye oligodactyly, clubfeet, manyorerwo anonzi hyperextensible, hip anomalies, knee anomalies, scoliosis, kyphosis, lordosis, nezvimwe zvisingaiti zvemukati
- Kuipa kwehutano mukati mehutachiona hunoonekwa munenge makumi mashanu kubva mumakumi mashanu ematambudziko uye kunogona kusanganisira: kukurumidza kunonoka, kutaura kunonoka kwekutaura, hypotonia, uye hydrocephalus.
- Genitourinary defect is more rare but may include uncended testes, hydronephrosis or hydrocele.
Chiitiko chePierre Robin chikamu chinenge chikamu chevana muna 8500 kuberekwa, chinokanganisa varume nemadzimai zvakaenzana. Pierre Robin kuenzaniswa kunogona kuitika mukati mawo pachako asi kunobatanidza nemamwe mimwe mazita ekuberekwa anosanganisira: Stickler syndrome, CHARGE syndrome, Shprintzen syndrome, Mobius syndrome, trisomy 18 syndrome, trisomy 11q syndrome, kubvisa 4q syndrome, nezvimwe.
Pane zvidzidzo zvitatu pamusoro pezvinoita kuti Pierre Robin aenzane. Chokutanga ndechekuti mandibular hypoplasia inowanikwa mukati mevhiki ye7-11 yekutakura. Izvi zvinokonzera kuti rurimi rurambe ruchikwiridzirwa mumuromo wechipfu ichi kudzivirira kugadziriswa kwemararamiro ekurapa uye kukonzera U-shaped cleft palate. Izvo zvishoma zvishoma zve amniotic fluid inogona kuva chinhu.
Chidzidzo chechipiri ndechekuti kune kunonoka mukuvandudza kwehupenyu hwemurimi musculature, pharyngeal mbiru, uye marate pamwe chete nekuderera mu hypoglossal nerve conduction. Iyi nyanzvi inotsanangura kuti nei zviratidzo zvizhinji zvinogadzirisa neve makore anenge matanhatu.
Nharo yechitatu ndeyekuti dambudziko guru rinowanikwa panguva yekuvandudza rinoita kuti dysneurulation ye rhombencephalus (iyo hindbrain - chikamu cheuropi chine brainstere uye cerebellum).
Hakuna mushonga wePeter Robin. Kutungamirirwa kwechiitiko kunosanganisira kurapa zviratidzo zvevanhu. Mune dzimwe nguva mhashu yakaderera inowedzera nokukurumidza mukati megore rokutanga rehupenyu uye kazhinji inowanzoonekwa yakajeka nechekuchikoro chechikoro. Kukura kwepanyama kunowanzoporesa chero mapfupa (airway) matambudziko angave aripo. Dzimwe nguva gwara rekufamba-famba (rakadai seanophargyngeal kana muromo wemhepo) rinofanira kushandiswa kwenguva yakati. Cleft palate inofanira kugadziriswa chirwere sezvo inogona kukonzera matambudziko nekudya kana kufema. Vana vazhinji vane mararamiro aPaul Robin vachatoda chirwere chekutaura.
Kuoma kwehutano hwaPaul Robin kunosiyana zvikuru pakati pevanhu sevamwe vanhu vanogona kungova nehuviri hwezviratidzo hunoenderana nechiitiko ichi apo vamwe vanogona kuva nezvimwe zviratidzo zvinoshamwaridzana nazvo.
Utano hwepfungwa kana hutano hwepfungwa hunogonawo kuva hwakaoma kudarika pane humwe hutsika hwepaniofacial hunobatanidza naPilbert Robin. Zvidzidzo zvakaratidza kuti mararamiro aPierre Robin ari oga, (apo chirwere ichi chinoitika pasina imwe yakabatana syndrome), haigamuchiri kuwedzera kufa kwevanhu, kunyanya apo zvinetso zvepfungwa kana hutano hwematambudziko hazvisipo.
> Sources:
> Cleft Palate Foundation. Pierre Robin Sequence. http://www.cleftline.org/parents-individuals/publications/pierre-robin-sequence/
> Medline Plus. Pierre Robin zvakaenzanirana. https://www.nlm.nih.gov/medlineplus/ency/article/001607.htm
> Medscape. Pierre Robin Syndrome. http://emedicine.medscape.com/article/844143-overview#a4