title: The Stomatological Complications of Diamond-Blackfan Anemia: A Case Report.
abstract: Diamond-Blackfan Anemia (DBA) is a rare heterogeneous genetic disease characterized by severe anemia, reduction or absence of erythroid progenitors, and pro-apoptoptic hematopoiesis, which culminates in bone marrow failure. The disease generally manifests in infancy, as craniofacial, cardiac, genitourinary, and upper limb congenital anomalies. Therapy with corticoids is the treatment of choice, while blood transfusion is adopted during diagnosis and as a chronic approach if the patient does not respond to corticoids. This case report describes DBA in a patient that presented with lesions on the oral mucosa caused by secondary neutropenia. The stomatologist plays an important role in a transdisciplinary team and must remain attentive to the general health conditions of patients, since some oral lesions may be associated with systemic events.
authors: Dentistry School, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil.
authors: Rita Fabiane Teixeira
authors: RF
authors: Gomes
authors: Dentistry School, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil Dentistry School, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil mcmunerato@gmail.com.
authors: Maria Cristina
authors: MC
authors: Munerato
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001741:Black or African American; D029503:Anemia, Diamond-Blackfan / Q000097:blood* / Q000150:complications* / Q000175:diagnosis / Q000208:ethnology; D017209:Apoptosis; D001803:Blood Transfusion; D001938:Brazil; D015897:Comorbidity; D005260:Female; D006410:Hematopoiesis / Q000502:physiology; D006439:Hemodynamics; D006760:Hospitalization; D006801:Humans; D007249:Inflammation; D009061:Mouth Mucosa / Q000473:pathology; D009503:Neutropenia / Q000150:complications / Q000000981:diagnostic imaging*; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Blood transfusion; Delayed diagnosis; Diamond-Blackfan Anemia; Neutropenia; Oral manifestations; Steroids