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Deletion of chr7p22 and chr15q11: Two Familial Cases of Immune Deficiency: Extending the Phenotype Toward Dysimmunity.
Sloboda, Natacha; Sorlin, Arthur; Valduga, Mylène; Beri-Dexheimer, Mylène; Bilbault, Claire; Fouyssac, Fanny; Becker, Aurélie; Lambert, Laëtitia; Bonnet, Céline; Leheup, Bruno.
Afiliación
  • Sloboda N; Clinic Genetics Department, Children Hospital, CHRU Nancy, Nancy, France.
  • Sorlin A; Clinic Genetics Department, Children Hospital, CHRU Nancy, Nancy, France.
  • Valduga M; Genetics Laboratory, CHRU Nancy, Nancy, France.
  • Beri-Dexheimer M; Genetics Laboratory, CHRU Nancy, Nancy, France.
  • Bilbault C; Infantile Medicine Department (Neuropediatrics), Children Hospital, CHRU Nancy, Nancy, France.
  • Fouyssac F; Infantile Medicine Department (Hematopediatrics), Children Hospital, CHRU Nancy, Nancy, France.
  • Becker A; Genetics Laboratory, CHRU Nancy, Nancy, France.
  • Lambert L; Clinic Genetics Department, Children Hospital, CHRU Nancy, Nancy, France.
  • Bonnet C; Genetics Laboratory, CHRU Nancy, Nancy, France.
  • Leheup B; Clinic Genetics Department, Children Hospital, CHRU Nancy, Nancy, France.
Front Immunol ; 10: 1871, 2019.
Article en En | MEDLINE | ID: mdl-31474980
Background: We report here two new familial cases of associated del15q11 and del7p22, with the latter underlining the clinical variability of this deletion. Two siblings patients presented a similar familial imbalanced translocation, originating from a balanced maternal translocation, with deletions of 7p22 and of 15q11 [arr[GRCh37] 7p22.3-p22.2(42976-3736851)x1, 15q11.1-q11.2(20172544-24979427)x1]. Methods: We used aCGH array, FISH, and karyotype for studying the phenotype of the two patients. Results: The 7p22 deletion (3.5 Mb) contained 58 genes, including several OMIM genes. Patients 1 and 2 exhibited acquisition delays, morphological particularities, and hypogammaglobulinemia, which was more severe in patient 1. Patient 1 presented also with cerebral vasculitis. Conclusion: We discuss here how the PDGFa, CARD11, LFNG, GPER1, and MAFK genes, included in the deletion 7p22, could be involved in the clinical and biological features of the two patients.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cromosomas Humanos Par 7 / Cromosomas Humanos Par 15 / Deleción Cromosómica / Síndromes de Inmunodeficiencia Límite: Child / Female / Humans / Male Idioma: En Revista: Front Immunol Año: 2019 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Suiza

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cromosomas Humanos Par 7 / Cromosomas Humanos Par 15 / Deleción Cromosómica / Síndromes de Inmunodeficiencia Límite: Child / Female / Humans / Male Idioma: En Revista: Front Immunol Año: 2019 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Suiza