ABSTRACT
Bridging the survival gap for children with cancer, between those (the great majority) in low and middle income countries (LMIC) and their economically advantaged counterparts, is a challenge that has been addressed by twinning institutions in high income countries with centers in LMIC. The long-established partnership between a Central American consortium--Asociación de Hemato-Oncología Pediátrica de Centro América (AHOPCA)--and institutions in Europe and North America provides a striking example of such a twinning program. The demonstrable success of this endeavor offers a model for improving the health outcomes of children with cancer worldwide. As this remarkable enterprise celebrates its 15th anniversary, it is appropriate to reflect on its origin, subsequent growth and development, and the lessons it provides for others embarking on or already engaged in similar journeys. Many challenges have been encountered and not all yet overcome. Commitment to the endeavor, collaboration in its achievements and determination to overcome obstacles collectively are the hallmarks that stamp AHOPCA as a particularly successful partnership in advancing pediatric oncology in the developing world.
Subject(s)
Cancer Care Facilities/organization & administration , Child Health Services/organization & administration , Disease Management , International Cooperation , Neoplasms/prevention & control , Pediatrics/organization & administration , Central America , Child , Conservation of Natural Resources , Europe , HumansABSTRACT
OBJECTIVE: To analyze the frequency of the haplotypes of ß-globin gene cluster in randomly selected patients with sickle cell disease (SCD), attended in the Children's Hospital of Panama. METHODS: Five polymorphic sites in the ß-globin gene cluster were analyzed by polymerase chain reaction (PCR) followed by restriction digestion and agarose gel electrophoresis in a total of 100 patients, including 95 homozygous for HbS (sickle cell anemia) and 5 compound heterozygotes for HbS and HbC genes (HbSC disease). RESULTS: The Bantu haplotype was predominant with a frequency of 51%, followed by the Benin (30%), Senegal (8.5%), and Cameroon (4%); other haplotypes were also identified. Genotype was CAR/CAR in 39 patients, BEN/BEN in 22, SEN/SEN in 6, CAM/CAM in 4, ARB/ARB in 1, CAR/BEN in 15, CAR/SEN in 5, CAR/Hp5 in 3, CAR/Hp1 in 1, BEN/Hp11 in 1, Atp Hp1/Hp1 in 2, and Atp Hp5/Hp5 in 1 individual. Hemoglobin concentrations, hematocrit, and mean corpuscular hemoglobin concentration values did not differ among homozygous forms of haplotypes. The mean HbF in all patients was 15.39 ± 1.21, whereas SEN/SEN patients had higher HbF than BEN/BEN patients (24.26 ± 4.18 vs. 13.17 ± 2.39, respectively, P < 0.05). The percentage of reticulocytes was highest in BEN/BEN and CAR/CAR, and it was associated with worst prognosis. CONCLUSION: The results show the presence of common ß(S) haplotypes in Panama; the prevalence of African origin, and the similarity in the Panamanian and Colombian distribution of haplotypes.
Subject(s)
Anemia, Sickle Cell/genetics , Fetal Hemoglobin/genetics , Haplotypes , Hemoglobin, Sickle/genetics , Polymorphism, Genetic , beta-Globins/genetics , Adolescent , Child , Child, Preschool , Erythrocytes, Abnormal/pathology , Female , Genotype , Hospitalization , Humans , Infant , Male , Multigene Family , Panama , Polymerase Chain ReactionABSTRACT
OBJECTIVE: To correlate the clinical and hematological features of ß-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD). METHODS: A total of 95 patients with SCD and 40 healthy children were studied. The ß-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured. RESULTS: Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group. CONCLUSION: Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.
Subject(s)
Anemia, Sickle Cell/blood , Anemia, Sickle Cell/genetics , Haplotypes , Lipid Peroxidation , Multigene Family , Oxidative Stress , beta-Globins/genetics , Adolescent , Anemia, Sickle Cell/pathology , Child , Child, Preschool , Female , Fetal Hemoglobin/genetics , Fetal Hemoglobin/metabolism , Glutathione Disulfide/blood , Humans , Infant , Male , Nitrates/blood , Nitrites/blood , Oxidoreductases/blood , Severity of Illness Index , beta-Globins/metabolismABSTRACT
Cancer is the commonest cause of disease-related death in children over 5 years of age in various parts of Latin America, but the survival rates are improving. This study assessed the health status and health-related quality of life (HRQL) of more than 200 survivors of cancer in childhood in the countries of a Central American consortium devoted to pediatric hematology-oncology. Patients' self-reports and parental proxy assessments were collected using interviewer-administered Spanish-language questionnaires, and physicians provided assessments using self-complete questionnaires, based on the complementary Health Utilities Index (HUI) Mark 2 (HUI2) and Mark 3 (HUI3) health status classification systems. Inter-rater agreement, measured by intra-class correlation (ICC), was fair to moderate (0.34
Subject(s)
Health Status Indicators , Interviews as Topic , Neoplasms/psychology , Quality of Life , Surveys and Questionnaires , Adolescent , Central America , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Male , Neoplasms/mortality , Neoplasms/physiopathology , Survival RateABSTRACT
The dramatic reduction of pediatric cancer mortality rates has been one of the greatest accomplishments of contemporary medicine. About 80% of children with cancer are now expected to be cured by current therapies. However, most of the world's children have no access to cancer treatment. The translation of effective pediatric cancer therapies to impoverished regions of the world presents an enormous challenge to the health care profession. Over the past 20 years, efforts have been under way to extend adequate cancer treatment to an increasing number of children in developing countries. These initiatives, collectively designated "twinning programs," consist essentially of a partnership between a pediatric cancer unit in a developing country and a group of health care providers in the developed world. Here we review the twinning programs that have been implemented in Central America, discuss their impact on the development of local resources and the outcome of childhood cancer, and propose a collaborative research initiative aimed at improving the international dissemination of progress in pediatric hematology-oncology.
Subject(s)
Child Health Services/organization & administration , International Cooperation , Medical Oncology/organization & administration , Neoplasms/therapy , Pediatrics/organization & administration , Central America , Child , Humans , Italy , North AmericaABSTRACT
Se realizó una revisión retrospectiva de todos los pacientes pediátricos hospitalizados durante los últimos 6 años (1985-1990) con diagnóstico de leucemia aguda y que habían sido admitidos a este hospital durante un episodio de neutropenia y fiebre. Fueron evaluados un total de 68 pacientes y 94 episodios granulocitopénicos. La neutropenia ocurrió con un promedio de 9 días después de la administración de la quimioterapia inductiva o reinductiva y se resolvió en los sobrevivientes después de un promedio de 16 días de iniciada. La etiología bacteriana del proceso febril fue documentada en el 63 (por ciento) de los episodios de neutropenia y predominaron los gérmenes gram-negativos, especialmente la Pseudomonas aeroginosa. Encontramos una tasa de resistencia antimicrobiana elevada de estos patógenos a los antibióticos utilizados tradicionalmente en el hospital y no así a los agentes antimicrobianos de uso restrigido tales como: netilmicina, amikacina y las cefalosporinas de tercera generación. Hubo evidencia clínica y/o microbiológica de infección por Candida albicans en un número apreciable de casos. La mortalidad global fue del 50 (por ciento), fue menor en los pacientes con (p= 0.008), en los que no tenían evidencia de infección micótica (p= 0.12) y significativamente menor en aquellos pacientes que recuperaban un nivel adecuado de neutrófilos circulantes (p 0.0001) se proponen varios esquemas terapéuticos para tratar de disminuir la alta tasa de mortalidad y la prolongada hospitalización de los pacientes
Subject(s)
Child , Adolescent , Humans , Leukemia , Neutropenia , PediatricsABSTRACT
En las últimas décadas muchos investigadores han estudiado la asociación de la Leucemia Aguda y el Síndrome de Down, llegando a conclusiones muy importantes al igual que a disyuntivas sobre las cuales aún se discute hoy en día
