title: Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes.
abstract: OBJECTIVE
  Human Severe Combined Immunodeficiency (SCID) is a prenatal disorder of T lymphocyte development that depends on the expression of numerous genes. Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic/myeloproliferative disease typically presenting in early childhood.
  
  
  METHODS
  Two cases are described of immunodeficiency disorders, both treated with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone marrow transplantation. After treatment, these two different cases showed several similar oral lesions: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both subjects underwent dental and orthodontic treatment. The first phase comprised orthopaedic treatment using a removable appliance (Interim-G®) followed by rapid palatal expansion; in the second phase patients underwent tooth extraction and were treated using fixed appliances for 19 and 26 months, respectively (mean 2 years) to obtain final alignment and maximum intercuspation. In the third and final phase, reconstruction of malformed teeth was completed, and implant-supported protheses were applied.
  
  
  CONCLUSIONS
  The difficulties of managing and treating these diseases are discussed, with particular focus on tooth anomalies and malocclusion disorders. Collaboration between dentist and paediatrician in dealing with patients with a variety of oral lesions and tooth anomalies is important in order to prevent any other possible tooth lesions and ensure correct jaw development.
authors: Department of Orthodontics, University of Milan, Orthodontic Department, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Milan, Italy.
authors: G
authors: G
authors: Cossellu
authors: R
authors: R
authors: Seramondi
authors: S
authors: S
authors: Benedicenti
authors: G
authors: G
authors: Farronato
authors: G
authors: G
authors: Olivi
authors: F
authors: F
authors: Angiero
mesh_terms: D000293:Adolescent; D018906:Antineoplastic Agents, Alkylating / Q000009:adverse effects*; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002066:Busulfan / Q000009:adverse effects; D002648:Child; D003520:Cyclophosphamide / Q000009:adverse effects; D006801:Humans; D054429:Leukemia, Myelomonocytic, Juvenile / Q000150:complications* / Q000628:therapy; D008297:Male; D008310:Malocclusion / Q000209:etiology; D008558:Melphalan / Q000009:adverse effects; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000628:therapy; D014071:Tooth Abnormalities / Q000209:etiology*
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003520:Cyclophosphamide; D002066:Busulfan; D008558:Melphalan