Dental Approach in the Pediatric Oncology Patient: Characteristics of the Population Treated at the Dentistry Unit in a Pediatric Oncology Brazilian Teaching Hospital

OBJECTIVES: The objective of this paper was to characterize the population seen at the dentistry unit of the hematology-oncology service of the Oncology-Hematology Service, Instituto da Criança at the Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo. Oral problems resulting from cancer therapy increase the risk of infection, length of hospital stay, treatment cost and negative impact on the course and prognosis of the disease. METHOD: Of the 367 medical records of cancer patients seen from November 2007 until December 2008: 186 with a cancer diagnosis and complete clinical data were selected, while 20 with a cancer diagnosis and incomplete records were excluded; 161 medical records with only hematological diagnosis were also excluded. The following characteristics were assessed: ethnicity, gender, age, diagnosis and characteristics of the neoplasm, cancer therapy status and performed dental procedures. RESULTS: Review of 1,236 visits indicated that 54% (n=100) of the patients had blood cancers, 46% (n=86) had solid tumors and 63% were undergoing anticancer therapy. The proportion of males (52.7%) in the study population was slightly greater. The most common cancer was acute lymphocytic leukemia (32.2%). Cancer occurred more often among those patients aged 5 to 9 years. The most common dental procedures were restorative treatment, preventive treatment and removal of infectious foci. CONCLUSION: The characteristics of the studied population were similar to those of the general Brazilian and global populations, especially regarding gender and diagnosis distributions. The aim of implementation of the dentistry unit was to maintain good oral health and patients' quality of life, which is critical to provide oral care and prevent future oral problems

A longitudinal study of the peripheral and central auditory pathways in individuals with acute lymphoid leukemia

Objective: To characterize the peripheral and central auditory pathways in individuals with Acute Lymphoid Leukemia (ALL) and compare assessment results before and during chemotherapy. Method: The study included 17 subjects with ALL, divided into two age groups: 3 to 6 (11 individuals) and 7 to 16 years old (6 individuals). Each subject was evaluated twice (before and 3 to 6 months after chemotherapy treatment) with the following procedures: medical history survey, otoscopy, Pure-Tone Threshold (PTA) and speech audiometry, acoustic immittance measures, Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP). Results: PTA was normal. Tympanometry was abnormal in the second assessment in 2 individuals aged 3 to 6 years. One subject in each age group had absent ipsilateral acoustic reflexes. In high-frequency audiometry, 1 individual had abnormal results. BAEP was abnormal in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years and 2 (first assessment) and 1 individual (second assessment) aged 7 to 16 years. As for LLAEP, P1 latency was increased in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years. Conclusion: No hearing loss was identified in the behavioral audiological assessment. BAEP was more affected in the 3-to-6-year-old group, with greater impairment in the lower brainstem in the first and second assessments. In LLAEP, P1 was the most impaired component in children aged 3 to 6 years, and P2 and N2 were so for those 7 to 16 years old, especially in the second assessment

Dental anomalies in children submitted to antineoplastic therapy

Cancer is the third most frequent cause of death in children in Brazil. Early diagnosis and medical advances have significantly improved treatment outcomes, which has resulted in higher survival rates and the management of late side effects has become increasingly important in caring for these patients. Dental abnormalities are commonly observed as late effects of antineoplastic therapy in the oral cavity. The incidence and severity of the dental abnormalities depend on the child's age at diagnosis and the type of chemotherapeutic agent used, as well as the irradiation dose and area. The treatment duration and aggressivity should also be considered. Disturbances in dental development are characterized by changes in shape, number and root development. Enamel anomalies, such as discoloration, opacities and hypoplasia are also observed in these patients. When severe, these abnormalities can cause functional and esthetic sequelae that have an impact on the children's and adolescents' quality of life. General dentists and pediatric dentists should understand these dental abnormalities and how to identify them aiming for early diagnosis and appropriate treatment.Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo Instituto da Criança ITACI Hemato-oncology Service, Dentistry DepartmentSão Leopoldo Mandic Campinas School of DentistryUniversidade Federal de São Paulo (UNIFESP) School of Medicine Pediatric Oncology Institute, GRAACC-IOP, Department of DentistryUniversidade Estadual Paulista Júlio de Mesquita Filho (UNESP) Instituto de Ciência e Tecnologia (http://www.fosjc.unesp.br/)Faculdade de Medicina da Universidade de São Paulo Department of PediatricUNIFESP, School of Medicine Pediatric Oncology Institute, GRAACC-IOP, Department of DentistrySciEL

Retinoblastoma: A Three-Year-Study at a Brazilian Medical School Hospital

OBJECTIVE: To present the characteristics and treatment outcomes of patients with retinoblastoma. METHODS: A retrospective case series was conducted to review the records of all new patients diagnosed with retinoblastoma between 2003 and 2005. Eyes with early disease, or advanced disease with potential vision were treated with chemotherapy (carboplatin and etoposide) in conjunction with early local therapy (laser or cryo). Radiotherapy was used in cases where the disease did not respond to the above protocols or in recurrent cases. Eyes in the late stage of disease with no potential vision in the initial examination or eyes and where conservative treatment had failed were enucleated. RESULTS: In total, we reviewed 28 new cases of retinoblastoma, 15 of which were unilateral and 13 of which were bilateral (46%). These data correspond to a mean of 9.3 new cases per year (0.77 case/ month). The mean age at diagnosis was 33.8 months for unilateral cases, and 19.15 months for bilateral cases (p=0.015). Leucocoria was the major presenting symptom (75%). All but one patient with unilateral disease had the affected eye enucleated due to advanced disease (mean follow-up: 39.91 months). Among the 13 bilateral cases, 13 eyes (50%) were enucleated, 11 eyes (42.4%) were saved with chemotherapy in conjunction with local therapy and 2 eyes (7.6%) were saved using external beam radiotherapy (mean follow-up: 41.91 months). In unilateral and bilateral disease, pathology data revealed choroid involvement in 50% and 30%, respectively, and optic nerve invasion in 92% and 50%, respectively. CONCLUSION: In this population, retinoblastoma was diagnosed too late and most eyes were consequently enucleated. In cases with bilateral disease, half of the eyes were preserved

New concepts and outcomes for children with hepatoblastoma based on the experience of a tertiary center over the last 21 years

OBJECTIVE: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years. PATIENTS AND METHODS: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system. The following data were analyzed: age at diagnosis, comorbidities, prematurity, treatment modalities, histopathological findings, surgical details and complications, treatment outcomes, chemotherapy schedules, side effects and complications. Treatment outcomes included the occurrence of local or distant recurrence, the duration of survival and the cause of death. The investigation methods were ultrasonography, CT scan, serum alpha-fetoprotein level measurement and needle biopsy. Chemotherapy was then planned, and the resectability of the tumor was reevaluated via another CT scan. RESULTS: The mean numbers of neoadjuvant cycles and postoperative cycles of chemotherapy were 6±2 and 1.5±1.7, respectively. All children except one were submitted for surgical resection, including 50 partial liver resections and 7 liver transplantations. Statistical comparisons demonstrated that long-term survival was associated with the absence of metastasis (p=0.04) and the type of surgery (resection resulted in a better outcome than transplantation) (p=0.009). No associations were found between vascular invasion, incomplete resection, histological subtype, multicentricity and survival. The overall 5-year survival rate of the operated cases was 87.7%. CONCLUSION: In conclusion, the experience of a Brazilian tertiary center in the management of hepatoblastoma in children demonstrates that long survival is associated with the absence of metastasis and the type of surgery. A multidisciplinary treatment involving chemotherapy, surgical resection and liver transplantation (including transplantations using tissue from living donors) led to good outcomes and survival indexes

Discrimination of acute lymphoblastic leukemia from systemic-onset juvenile idiopathic arthritis at disease onset

OBJECTIVE: To assess clinical and laboratory features that differentiate acute lymphoblastic leukemia from systemic juvenile idiopathic arthritis at disease onset. METHODS: Fifty-seven leukemia patients with musculoskeletal involvement, without blasts on peripheral blood and without glucocorticoid therapy at disease onset and 102 systemic juvenile idiopathic arthritis patients (International League of Associations for Rheumatology criteria) were retrospectively evaluated. The following features were examined: fever, rheumatoid rash, arthritis, limb pain, hepatomegaly, splenomegaly, pericarditis, myocarditis, pleuritis, weight loss, bleeding, anemia, leukopenia, neutropenia, thrombocytopenia, erythrocyte sedimentation rate, and lactic dehydrogenase levels. RESULTS: The median age at disease onset was significantly higher in leukemia patients than in those with systemic-onset juvenile idiopathic arthritis (5.8 vs. 3.8 years). In addition, the frequencies of limb pain, hepatomegaly, weight loss and hemorrhagic manifestations were significantly higher in leukemia patients than in systemic-onset juvenile idiopathic arthritis patients (70% vs. 1%, 54% vs. 32%, 30% vs. 8%, and 9% vs. 0%, respectively). Likewise, the frequencies of anemia, leukopenia, neutropenia, thrombocytopenia and high lactic dehydrogenase levels were statistically higher in leukemia patients than in patients with systemic-onset juvenile idiopathic arthritis (88% vs. 57%, 39% vs. 1%, 60% vs. 1%, 77% vs. 1%, and 56% vs. 14%, respectively). Remarkably, multivariate analysis revealed that limb pain (OR = 553; 95% CI =46.48-6580.42) and thrombocytopenia (OR = 754.13; 95% CI =64.57-8806.72) were significant independent variables that differentiated leukemia from systemic-onset juvenile idiopathic arthritis. The R2 of the Nagelkerke test was 0.91, and the Kaplan-Meier survival curves were similar for acute lymphoblastic leukemia patients with and without limb pain. CONCLUSION: Our study emphasizes the importance of investigating leukemia in patients presenting with musculoskeletal manifestations and, in particular, limb pain associated with thrombocytopenia

Evaluation of the main late endocrine effects in children and adolescents after treatment of malignancies

OBJETIVO: Relatar os efeitos endócrinos tardios em crianças e adolescentes após tratamento oncológico e associá-los à doença de base e ao tratamento. SUJEITOS E MÉTODOS: Foram realizadas avaliações clínicas e laboratoriais visando à detecção de distúrbios endócrinos em 320 pacientes após terapia oncológica, seguidos por seis anos. RESULTADOS: Em 94 pacientes, detectaram-se: 32 pacientes apresentaram baixa estatura (nove em tratamento com hormônio de crescimento), 14 tiveram puberdade precoce (10 em uso de análogo de GnRH) e 19 revelaram ser portadores de distúrbios de tireoide (12 com hipotireoidismo, seis com nódulos de tireoide e um com tireoidite linfocitária crônica). Obesidade foi encontrada em 18 deles. Seis com diabetes insípido e cinco com puberdade atrasada, três com pan-hipopituitarismo. Houve associação entre a radioterapia e a presença de endocrinopatias. CONCLUSÃO: Noventa e quatro de 320 (30%) dos pacientes fora de terapia apresentaram alteração endócrina, o que enfatiza a importância do seguimento precoce e regular, possibilitando-lhes, com tratamento, melhor qualidade de vida.OBJECTIVE: To report the main endocrine effects after cancer treatment in children and adolescents and associate them to the disease and its treatment. SUBJECTS AND METHODS: Clinical and lab evaluation for endocrinopathy was performed in 320 patients after cancer therapy have been followed for six years. RESULTS: The most prevalent endocrine late effects in patients were: 32 patients had short stature, nine of them were under growth hormone therapy. Precocious puberty was found in 14 patients, 10 of them received GnRH analog. Thyroid diseases were present in 19 patients (12 with hypothyroidism; six with thyroid nodules/cysts; one with chronic lymphocytic thyroidytis). Obesity was found in 18 patients. Six presented insipidus diabetes, five delayed puberty and three panhypopituitarism. Radiation was associated with the appearance of the aforementioned endocrinopathies. CONCLUSION: Ninety four of 320 (30%) patients presented endocrine late effects which emphasize the importance for these patients to be regularly followed-up in order to precociously diagnose endocrine late effects and provide them a better quality of life

Mandibular radiomorphometric assessment of bone mineral density in survivors of pediatric hematopoietic stem-cell transplantation

OBJECTIVE: Hematopoietic stem-cell transplantation (HSCT) childhood survivors of hematologic malignancies are prone to develop late osteopenia and osteoporosis. The purpose of this retrospective study was to quantitatively and qualitatively assess bone mineral density (BMD) in HSCT childhood survivors and to compare the effectiveness of both qualitative and quantitative assessment methods. METHODS: DESIGN BMD assessment using panoramic radiographs of childhood HSCT survivors aged 3.69-18.88 years using two radiomorphometric indexes. Case-control double-blinded comparison of panoramic radiographic images from childhood HSCT survivors and age- and sex-matched healthy controls. Quantitative assessment was performed by measuring the cortical bone width bilaterally at the mental foramen level. Qualitative assessment was performed using the mandibular cortical index bilaterally on all panoramic images. RESULTS: Radiographs were taken 6.59-83.95 months after bone marrow transplantation [median±SD=25.92 ±24.9 months]. Fifty-two panoramic radiographic images were analyzed: 21 from HSCT survivors and 31 from healthy controls aged 3.69-25.1 years [mean±SD=11.89±5.28 years]. The mandibular cortical bone width was 17% smaller in childhood HSCT survivors than in healthy controls (case group: 2.420, control group: 3.307; p=0.00617). Qualitative analysis revealed an increased frequency of severe mandibular cortical erosion in childhood HSCT survivors, although no significant difference was observed (case group: 1.540, control group: 1.490; p=0.32). The interobserver agreement was 85% (Kappa index). CONCLUSIONS: HSCT childhood survivors exhibit quantitative and qualitative mandibular bone impairments. Further studies are needed to establish an association between mandibular cortical bone impairment and osteoporosis

Crescimento e puberdade após tratamento da leucemia linfoblástica aguda

Over the last 20 years, after combining treatment of chemotherapy and radiotherapy, there has been an improvement in the survival rate of acute lymphoblastic leukemia patients, with a current cure rate of around 70%. Children with the disease have been enrolled into international treatment protocols designed to improve survival and minimize the serious irreversible late effects. Our oncology unit uses the international protocol: GBTLI LLA-85 and 90, with the drugs methotrexate, cytosine, arabinoside, dexamethasone, and radiotherapy. However, these treatments can cause gonadal damage and growth impairment. PATIENTS AND METHOD: The authors analyzed 20 children off therapy in order to determine the role of the various doses of radiotherapy regarding endocrinological alterations. They were divided into 3 groups according to central nervous system prophylaxis: Group A underwent chemotherapy, group B underwent chemotherapy plus radiotherapy (18 Gy), and group C underwent chemotherapy plus radiotherapy (24 Gy). Serum concentrations of LH, FSH, GH, and testosterone were determined. Imaging studies included bone age, pelvic ultrasound and scrotum, and skull magnetic resonance imaging. RESULTS: Nine of the patients who received radiotherapy had decreased pituitary volume. There was a significant difference in the response to GH and loss of predicted final stature (Bayley-Pinneau) between the 2 irradiated groups and the group that was not irradiated, but there was no difference regarding the radiation doses used (18 or 24 Gy). The final predicted height (Bayley-Pinneau) was significantly less (P = 0.0071) in both groups treated with radiotherapy. Two girls had precocious puberty, and 1 boy with delayed puberty presented calcification of the epididymis. CONCLUSION: Radiotherapy was been responsible for late side effects, especially related to growth and puberty.Nos últimos 20 anos, após o tratamento de pacientes portadores de leucemia linfoblástica aguda, com quimioterapia e radioterapia, houve melhora na taxa de sobrevivência e cura em torno de 70%. Crianças portadoras da doença foram envolvidas em protocolos de tratamento internacionais que visavam melhorar a sobrevida e minimizar os graves e irreversíveis efeitos tardios. A nossa unidade utiliza o protocolo internacional GBTLI LLA-85 e 90, com as drogas metrotexate, citosina, arabinoside, dexametasona e radioterapia .Entretanto, estes tratamentos podem causar insuficiências gonadais e prejuízo no crescimento. PACIENTES E MÉTODO: Os autores analisaram 20 crianças fora de terapia a fim de determinar o papel das várias doses de radioterapia sobre alterações endocrinológicas. Foram divididos em três grupos baseados na profilaxia do sistema nervoso central: o grupo A foi submetido à quimioterapia, o grupo B à quimioterapia mias radioterapia (18Gy) e o grupo C à quimioterapia mais radioterapia (24 Gy). Foram avaliadas as concentrações séricas de LH, FSH, GH e testosterona. Os estudos de imagem incluiram idade óssea, ultrassonografia pélvica, escrotal e ressonância nuclear magnética do crânio. RESULTADOS: Houve diferenças significativas nas respostas do hormônio de crescimento e prejuízo na estatura final (Bayley-Pinneau) entre os dois grupos irradiados e o grupo que não foi irradiado, mas não houve diferenças quando se compararam as doses de radiação utilizadas (18 ou 24 Gy). A previsão da altura final (Bayley-Pinneau) foi menor (p= 0,0071) nos dois grupos tratados com radioterapia. Duas meninas apresentaram puberdade precoce e um menino teve atraso puberal associado a calcificação do epidídimo. CONCLUSÃO: A radioterapia é responsável por efeitos colaterais especialmente quanto ao crescimento e puberdade