congenital erythropoietic porphyria;
oral health promotion;
porphyria;
rehabilitation;
D O I:
10.14693/jdi.v27i2.1124
中图分类号:
R78 [口腔科学];
学科分类号:
1003 ;
摘要:
Congenital erythropoietic porphyria (CEP) is an extremely rare disorder involved in chronic porphyrias. One of the recommended evaluations to determine disease severity and needs of a person with CEP is dental assessment. Objective: To present a case of CEP and its oral rehabilitation. Case Report: A 32-year-old female patient was admitted to the clinic with hyperkeratinization, skin fragility, fissures, and wounds in her perioral region and hands. Intraoral gingival bleeding, mucogingival stress, and missing teeth were recorded. After medical consultation, phase I and II periodontal therapies were performed. The patient expressed satisfaction of the results of the procedures in terms of the parameters evaluated. The rare occurrence of the disease may delay the diagnosis. An unmet need still exists for multidisciplinary orientation of patients with CEP. Conclusion: Dentists should have and follow guidelines for treating patients with CEP.
机构:
Lab Excellence Gr Ex, F-75015 Paris, France
Univ Paris, Ctr Rech Inflammat, INSERM U1149, F-75018 Paris, France
Hop Louis Mourier, AP HP, Ctr Francais Porphyries, 178 Rue Renouillers, F-92701 Colombes, FranceUniv Bordeaux, INSERM, CHU Bordeaux, BMGIC,U1035, F-33076 Bordeaux, France
Poli, Antoine
Puy, Herve
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机构:
Lab Excellence Gr Ex, F-75015 Paris, France
Univ Paris, Ctr Rech Inflammat, INSERM U1149, F-75018 Paris, France
Hop Louis Mourier, AP HP, Ctr Francais Porphyries, 178 Rue Renouillers, F-92701 Colombes, FranceUniv Bordeaux, INSERM, CHU Bordeaux, BMGIC,U1035, F-33076 Bordeaux, France
机构:
G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Faraci, Maura
Morreale, Giuseppe
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Morreale, Giuseppe
Boeri, Elio
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Boeri, Elio
Lanino, Edoardo
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Lanino, Edoardo
Dallorso, Sandro
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Dallorso, Sandro
Dini, Giorgio
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Dini, Giorgio
Scuderi, Francesca
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G Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Scuderi, Francesca
Cohen, Amnon
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San Paolo Hosp, Dept Pediat, Savona, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy
Cohen, Amnon
Cappelli, Barbara
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San Raphael Hosp, Bone Marrow Transplant Unit, Milan, ItalyG Gaslini Inst Children, Dept Hematol & Oncol, Bone Marrow Transplant Unit, Genoa, Italy