Background. Human SCID (Severe Combined Immunodeficiency) 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. Various oral lesions may appear in both of these immune deficiency disorders. Case Reports. We describe two cases that had been treated for systemic diseases (SCID and JMML) with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone-marrow transplantation. Both developed closely similar clinical findings in the oral cavity: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both were subjected to dental and orthodontic treatment to correct malocclusion and oral disease. Results The treatment was performed in 2 phases. The first phase comprised orthopedic treatment using a removable appliance (Interim-G®) followed by rapid palatal expansion; in the second phase both patients underwent tooth-extraction and were treated using fixed appliances for a mean period of 2 years (19 and 26 months) to obtain final alignment and maximal interdigitation. The third and final phase will comprise reconstruction of malformed teeth and implant-supported rehabilitation.
Tooth Developmental Anomalies in One Patient with Severe Combined Immunodeficiency Disease and One with Juvenile Myelomonocytic Leukemia: Clinical feature and treatment outcome
BENEDICENTI, STEFANO;ANGIERO, FRANCESCA
In corso di stampa
Abstract
Background. Human SCID (Severe Combined Immunodeficiency) 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. Various oral lesions may appear in both of these immune deficiency disorders. Case Reports. We describe two cases that had been treated for systemic diseases (SCID and JMML) with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone-marrow transplantation. Both developed closely similar clinical findings in the oral cavity: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both were subjected to dental and orthodontic treatment to correct malocclusion and oral disease. Results The treatment was performed in 2 phases. The first phase comprised orthopedic treatment using a removable appliance (Interim-G®) followed by rapid palatal expansion; in the second phase both patients underwent tooth-extraction and were treated using fixed appliances for a mean period of 2 years (19 and 26 months) to obtain final alignment and maximal interdigitation. The third and final phase will comprise reconstruction of malformed teeth and implant-supported rehabilitation.
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