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1. Alejandro Zambrano R1Audiología y OtoneurologíaCMN LA RAZAdomingo, 16 de junio de2013SÍNDROME DE CHARGE 2.…
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  • 1. Alejandro Zambrano R1Audiología y OtoneurologíaCMN LA RAZAdomingo, 16 de junio de2013SÍNDROME DE CHARGE
  • 2. OBJETIVOSActualizaciónCaracterísticasclínicasTratamiento
  • 3. INTRODUCCIÓNHall/Hittner• SíndromeAsociaciónCHARGER. PagonCHARGEA. Verloes1 Lubinsky MS: Properties of associations: identity, nature, and clinical criteria, with a commentary on why CHARGE and Goldenhar are not associations. Am J Med Genet 1994; 49: 21– 25.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 4. 1 Lubinsky MS: Properties of associations: identity, nature, and clinical criteria, with a commentary on why CHARGE and Goldenhar are not associations. Am J Med Genet 1994; 49: 21– 25.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 5. CHARGEColobomaMalformacionesCardiacasAtresia CoanasMalformacionesde OídoTrastornosUrogenitalesCrecimiento yDesarrolloRetardado1 Lubinsky MS: Properties of associations: identity, nature, and clinical criteria, with a commentary on why CHARGE and Goldenhar are not associations. Am J Med Genet 1994; 49: 21– 25.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 6. • CrestaneuralTrastornoblastogenéticoy procesoneurológico• 8q12• 2/3 ClínicaGen CHD71 Lubinsky MS: Properties of associations: identity, nature, and clinical criteria, with a commentary on why CHARGE and Goldenhar are not associations. Am J Med Genet 1994; 49: 21– 25.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 7. ETIOLOGÍATeratogénicos58% serieamericana64%DutchGEN CHD7MultifactorialCaracterísticasclínicasBlake’s yVerloesValorPredictivo31 Lalani SR, Safiullah AM, Fernbach SD et al: Spectrum of CHD7 Mutations in 110 Individuals with CHARGE Syndrome and Genotype-Phenotype Correlation. Am J Hum Genet 2006; 78: 303– 314.32 Aramaki M, Udaka T, Kosaki R et al: Phenotypic spectrum of CHARGE syndrome with CHD7 mutations. J Pediatr 2006; 148: 410– 414.
  • 8. CHD7CHD7MutaciónCromatinhelicasaDNAProteína 78q12.1DoscromatinasUnSNF2/SWIUn helicasaDosdominiosBRKPresente en10/17pacientes31 Lalani SR, Safiullah AM, Fernbach SD et al: Spectrum of CHD7 Mutations in 110 Individuals with CHARGE Syndrome and Genotype-Phenotype Correlation. Am J Hum Genet 2006; 78: 303– 314.32 Aramaki M, Udaka T, Kosaki R et al: Phenotypic spectrum of CHARGE syndrome with CHD7 mutations. J Pediatr 2006; 148: 410– 414.
  • 9. 1 Lubinsky MS: Properties of associations: identity, nature, and clinical criteria, with a commentary on why CHARGE and Goldenhar are not associations. Am J Med Genet 1994; 49: 21– 25.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 10. Los dominios de cromo• Reorganizan lisina-metilada dehistona del DNADominios SNF2/SWIProteínas que forman DNA• Dependientes ATP asa36 Jongmans M, Sistermans EA, Rikken A et al: Genotypic and phenotypic characterization of Noonan syndrome: new data and review of the literature. Am J Med Genet A 2005; 134:165– 170.37 Arrington CB, Cowley BC, Nightingale DR, Zhou H, Brothman AR, Viskochil DH: Interstitial deletion 8q11.2-q13 with congenital anomalies of CHARGE association. Am J Med Genet A 2005; 133: 326
  • 11. Dominio helicasa• Separación fragmentos DNA enreplicación, reparto, transcripcióny recombinaciónDominio BRK• Desconocida36 Jongmans M, Sistermans EA, Rikken A et al: Genotypic and phenotypic characterization of Noonan syndrome: new data and review of the literature. Am J Med Genet A 2005; 134:165– 170.37 Arrington CB, Cowley BC, Nightingale DR, Zhou H, Brothman AR, Viskochil DH: Interstitial deletion 8q11.2-q13 with congenital anomalies of CHARGE association. Am J Med Genet A 2005; 133: 326
  • 12. CHD7• Se expresa desde el día 10 deconcepción en tejidos que formaran• Vesícula óptica• Epitelio olfatorio• Oído interno• Sistema vascular• Corazón• Complejo preganglionar acústico-facial• Procencéfalo• Romboencéfalo• Primer arco braquial36 Jongmans M, Sistermans EA, Rikken A et al: Genotypic and phenotypic characterization of Noonan syndrome: new data and review of the literature. Am J Med Genet A 2005; 134:165– 170.37 Arrington CB, Cowley BC, Nightingale DR, Zhou H, Brothman AR, Viskochil DH: Interstitial deletion 8q11.2-q13 with congenital anomalies of CHARGE association. Am J Med Genet A 2005; 133: 326
  • 13. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 14. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 15. SEMA3ESEMA3E7q21S703LMutación de novoLalaniColobomaAtresia coanasMalformacionesde oídoHipoacusiaprofundaTetralogía deFallotCrecimiento yDesarrolloretardadoRelación CHD7 ySEMA3Edesconocida1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 16. ANOMALIAS CROMOSOMICASTrisomía 18Trisomía 13CHARGE1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 17. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 18. ANTECEDENTE PRENATALESDiabetes GestacionalAlcoholismoÁcidoretinoicoTalidomina1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 19. PATOGENIADía 36CócleaDía 37 cierrecolobomaDía 38DehiscenciamembranabucofaríngeaDía 40colonizacióntubocardiaco porcélulas tuboneuralAlteración endiferenciación• Mesodermo y ectodermo1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 20. Manif.ClínicasdismorfismoAlteracionesoftalmológicasAlteracionesauditivasAtresia coanasAlteracionesSNCAlteraciónRomboencéfaloAlteraciónHipotálamoCrecimientoretardadoAlteracionesgenitourinariasInmadurezinmunológicaTrastornosortopédicosRetraso mental20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.21 Khadilkar VV, Cameron FJ, Stanhope R: Growth failure and pituitary function in CHARGE and VATER associations. Arch Dis Childhood 1999; 80: 167– 170.
  • 21. MANIFESTACIONES CLINICASMás de 400 casosreportados en la literaturaRevisión y estudiosgrandes20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.21 Khadilkar VV, Cameron FJ, Stanhope R: Growth failure and pituitary function in CHARGE and VATER associations. Arch Dis Childhood 1999; 80: 167– 170.
  • 22. 20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.21 Khadilkar VV, Cameron FJ, Stanhope R: Growth failure and pituitary function in CHARGE and VATER associations. Arch Dis Childhood 1999; 80: 167– 170.
  • 23. DISMORFISMO Cara cuadrada Aumento diámetrobifrontal Puente nasal ancho Boca pequeña Parálisis facial Labio y paladarhendido presente en15-20% de los casos. Alteraciones dentales1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 24. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 25. ALTERACIONES OCULARES Colobomacorioretineal (40%) Microoftalmiapresente en 75-90% Coloboma C. uveal C. palpebral Hipoplasia del nervioóptico Enoftalmia Nistagmus Anquiloblefaro Atresia del conductolacrimal.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 26. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 27. ALTERACIONES AUDITIVAS YVESTIBULARES Pabellón auricular en95-100% Implantación baja Antero-versión Forma de asa Aplanada Apéndicespreauriculares Hipoplasia deconducto auditivo Microtía.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 28. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 29. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 30. OÍDO MEDIO: Ausencia musculo del estribo ventana oval hipoplasia yunque yestribo Otosclerosis Otitis crónica serosa.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 31. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 32. OÍDO INTERNO: Hipoplasia uncus temporal Displasia de Mondini no es especifica. Hipoplasia Utrícular y canales semicirculares Hipoplasia sáculo y cóclea Agenesia canales semicirculares Skull-rayos-X TAC y IRM1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 33. Las malformaciones de oído interno: Ausencia de nistagmos a estimulación calórica. Repuesta vestíbulo-ocular rotatoria ausente. Hipoacusia en 60-90% hipoacusia conductiva o mixta, frecuencias elevadas.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.2 Issekutz KA, Graham Jr JM, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005; 133: 309– 317.
  • 34. 16 Verloes A: Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133: 306– 308.17 Satar B, Mukherji SK, Telian SA: Congenital aplasia of semicircular canals. Otol Neurotol 2003; 24: 437– 446.
  • 35. 16 Verloes A: Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133: 306– 308.17 Satar B, Mukherji SK, Telian SA: Congenital aplasia of semicircular canals. Otol Neurotol 2003; 24: 437– 446.
  • 36. 16 Verloes A: Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133: 306– 308.17 Satar B, Mukherji SK, Telian SA: Congenital aplasia of semicircular canals. Otol Neurotol 2003; 24: 437– 446.
  • 37. ATRESIA DE COANAS Presente en 35-65% delos casos. Atresia coanas óseas ybilateral Presente en 38/130casos Hendidura laríngea Estenosis subglótica Traqueomalacia Parálisis de cuerdasvocales Reflujogastroesofágico18 Leclerc JE, Fearon B: Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 1987; 13: 265– 27216 Verloes A: Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133: 306– 308.
  • 38. 18 Leclerc JE, Fearon B: Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 1987; 13: 265– 27216 Verloes A: Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133: 306– 308.
  • 39. MALFORMACIONES DE SISTEMA NERVIOSOCENTRAL Presentes en 55-85%de los pacientes Anosmia o hipoplasiadel bulbo olfatorio esla alteración máscomún 18/18 pacientesdemostrado por IRM . Holoprosencefalia Hipoplasia de lóbulofrontal Hipoplasia de vermis Estenosis delacueducto de Sylvius20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 40.  Afección del VIII par Parálisis facial congénita asimétrica 50-90% Revisión Byerly’s 150 casos: VII (43%), IX y X (31%) II (2% ) VI (2%)20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 41. 20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 42. ALTERACION HIPOTALOMO-HIPOFISIS YSINDROME DE KALLMANN Causa de crecimiento retardado Hipoplasia genital Pubertad retardada. La sustitución hormonal es recomendada.20 Pinto G, Abadie V, Mesnage R et al: CHARGE syndrome includes hypogonadotropic hypogonadism and abnormal olfactory bulb development. J Clin Endocrinol Metab 2005; 90: 5621– 5626.1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 43. CRECIMIENTO RETARDADO Es poco frecuente La mayoría de los pacientes tiene peso y tallaacorde para la edad Se relaciona a alteraciones alimenticias yproblemas quirúrgicos La deficiencia de GH es rara1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 44. ALTERACIONES UROGENITALES 50-70% presentan esta alteración 80-90% hombres 15-25% mujeres Hipoplasia de los genitales externos es máscomún 80% de los niños presentan micropene, y/ocriptorquidia 1/3 parte de las niñas presentan hipoplasia delabios mayores1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 45.  Alteraciones del tracto urinario presente en 10-40% Principalmente riñón ectópico Riñón en herradura Alteraciones uretrales1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 46. OTRAS MALFORMACIONESVISCERALES Defectos congénitoscardiacos observadosen 50-85% pacientes Tetralogía de Fallot(1/3 de los casos) CIV CIA Coartación de aorta PDA presente 75% Arteria subclaviaaberrante Fistulatraqueoesofágica1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 47. INMUNODEFICIENCIA Poco común y muy variable Linfopenia células tipo T Deficiencia de subtipos de inmunoglobulinas tipoG21.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399
  • 48. TRASTORNOS ORTOPÉDICOS 37% presenta alteraciones de extremidadesinferiores. (54). Malformaciones vertebrales: Vertebras fusionadas Hemivertebras Unión cervico-craneal Numero de costillas Escoliosis en la pubertad 60%1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 49. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-39922 Jongmans M, Admiraal R, van der DK: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet 2005, eprint.
  • 50. RETRASO MENTAL Y ALTERACIONES DELCOMPORTAMIENTO Sedestación después de 1 año de edad Primeros pasos 24 o 36 meses IQ < 701.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.29 Raqbi F, Le BC, Morisseau-Durand MP, Dureau P, Lyonnet S, Abadie V: Early prognostic factors for intellectual outcome in CHARGE syndrome. Dev Med Child Neurol 2003; 45: 483– 488.
  • 51. CARACTERÍSTICAS CLÍNICAS EN SÍNDROME DE CHARGEQUE PRESENTA MUTACIÓN DEL GEN CHD7 La presencia de mutación CDH7 se reporta en: En 69/107 pacientes En 59/110 pacientes En 17/24 pacientes1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.29 Raqbi F, Le BC, Morisseau-Durand MP, Dureau P, Lyonnet S, Abadie V: Early prognostic factors for intellectual outcome in CHARGE syndrome. Dev Med Child Neurol 2003; 45: 483– 488.
  • 52. CRITERIOS DIAGNÓSTICOS Originalmente se necesitaba cumplir con 4 de losseis criterios Blake’s propuso nuevos criterios diagnósticos1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 53. 1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.
  • 54. DIAGNÓSTICO PRENATAL Polihidramnios 57% atresia de coanas fistula traqueo- esofágica Ausencia de canales semicirculares yalteraciones cardiacas detectada por ultrasonido1.1 D. Sanlaville. A. Verloes: CHARGE syndrome: an update.Eur J Hum Genet 2007:15:389-399.29 Raqbi F, Le BC, Morissea
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