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Abstract Fanconi anemia is a rare autosomal recessive disease. In this disease, cytokine pathways can induce the bone marrow failure that is observed in individuals with Fanconi anemia. Interleukin IL-17 exhibits a protective effect in organisms because it induces neutrophil recruitment and shows a pathological role in several models of autoimmune diseases, periodontal disease, cancer, allograft rejection, and graft versus host disease. Polymorphisms in the IL17A and IL17RA genes were evaluated from DNA in saliva, comparing individuals with or without Fanconi anemia, using models of genotypic transmission (additive, dominant, and recessive). Polymorphisms in the IL17A and IL17RA genes (rs2241044 [C allele], rs879577 [C allele], rs9606615 [T allele], and rs2241043 [C allele]) were risk factors for developing Fanconi anemia. We also performed an analysis of gene markers with clinical variables in the Fanconi group. Polymorphisms in the IL17A gene (rs3819025 [A allele] and rs2275913 [G allele], respectively) were associated with an age of less than 20 years (p = 0.026; RP 0.65) and the female sex (p = 0.043; RP 0.88). The IL17RA gene was also associated with age and the presence of leukoplakia (a potentially malignant oral disorder). An age of less than 20 years was associated with rs917864 (T allele; p = 0.036; RP 0.67). The presence of leukoplakia was associated with rs17606615 (T allele; p = 0.042; RP 0.47). To our knowledge, this is the first study that associates IL17A and IL17RA gene polymorphisms with Fanconi anemia and examines rs2241044 polymorphisms in scientific literature thus far.

https://doi.org/10.1590/1807-3107bor-2023.vol37.0012

Immune reconstitution after allogenic stem cell transplantation: An observational study in pediatric patients
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Belinovski, Aline Risson

; Pelegrina, Polliany Dorini

; Lima, Alberto Cardoso Martins

; Dumke, Cilmara Cristina Kuwahara

; Rodrigues, Adriana Mello

; Loth, Gisele

; Benini, Fernanda Moreira de Lara

; Rodrigues, Ana Luiza Melo

; Motta, Fábio Araujo

; Prando, Carolina

; Bonfim, Carmem

Hematology, Transfusion and Cell Therapy

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2023, Volume 45 Nº 2 Páginas 235 - 244

Resumo: En | Texto: En | PDF: En

Abstract Introduction The immune reconstitution (IR) after the allogenic hematopoietic stem cell transplantation (allo-HSCT) is a progressive process intrinsically correlated to the therapeutic success. It is essential to understand the interfering factors in IR to prevent the HSCT-related mortality. Methods We retrospectively evaluated the clinical outcomes, absolute lymphocyte counts (ALCs) and lymphocyte subtypes at different time-points of 111 pediatric patients with allogeneic HSCT for malignant and non-malignant diseases from 2013 to 2018. Results The ALCs gradually increased on D+30, D+100, and D+180 (medians 634/μL, 1022/μL and 1541/μL, respectively). On D+100, the CD3+CD8+ achieved the highest recovery rate (68%), followed by the CD16+CD56+ (47%), CD3+CD4+ (39%) and CD19+ (8%). The adequate ALC recovery was associated with age < 8 years, bone marrow grafts, myeloablative conditioning, non-use of serotherapy and non-haploidentical donors. The ALC and CD3+CD8+ on D+100 counts were higher in patients with the cytomegalovirus infection. The CD3+CD4+ recovery was associated with an age < 8 years, a non-malignant disease and a lower incidence of acute graft-versus-host disease ≥ grade 2. Furthermore, the ALC recovery on D+100 resulted in a higher overall survival, regardless of the disease type (HR 3.65, 1.05 - 12.71, p= 0.04). Conclusion Several factors influenced the IR after the allo-HSCT. The ALC ≥ 500/μL on D+100 was a simple IR predictor of survival, easily available to resource-limited centers.

https://doi.org/10.1016/j.htct.2022.05.008

Self-perception of periodontal health status among individuals with Fanconi anemia
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Perdoncini, Nicole Nichele

; Furquim, Camila Pinheiro

; Bonfim, Carmem Maria Sales

; Soares, Geisla Mary Silva

; Torres-Pereira, Cassius Carvalho

Hematology, Transfusion and Cell Therapy

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Dez 2021, Volume 43 Nº 4 Páginas 453 - 458

Resumo: En | Texto: En | PDF: En

ABSTRACT Introduction: Fanconi anemia (FA) is a rare genetic disease characterized by congenital malformations and bone marrow failure. One of the most common oral diseases in individuals with FA is periodontitis and adequate self-perception of periodontal status could contribute to its prevention and early detection. Aim: To compare oral health self-perception, measured by a questionnaire, with the clinical oral condition of patients with FA. Methods and Results: Fifty-six patients with FA, over 11 years of age, answered a questionnaire about dental history and self-reported oral health. Decayed, missing, and filled teeth (DMFT), Visible Plaque Index (VPI) and Gingival Bleeding Index (GBI) were measured. The median age of participants was 21 years (min 11, max 44), 31 (55%) were females and 25 (45%) males. Thirty-five (62.5%) participants rated their oral condition as satisfactory and 7 (12.5%) participants reported tooth mobility, 10 (17.9%) exposed roots and 21 (37.5%) gingival bleeding. Clinical examination detected average DMFT = 5.23, VPI = 31.36% and GBI = 33.77%. The gingival bleeding report was more frequent among individuals with higher GBI (p = 0.014). The DMFT was higher in those who had already undergone dental treatments (p = 0.031). There was an association between participants who presented dental caries and who rated their oral health as poor (p = 0.03). The question “Do your gums bleed easily?” had good accuracy in the evaluation of periodontal disease (p = 0.68). Conclusion: Oral health self-perception of individuals with FA about gingival inflammation was associated with their gingival bleeding index.

https://doi.org/10.1016/j.htct.2020.07.009

Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Consensus on genetically modified cells. II: CAR-T cell therapy for patients with CD19+ acute lymphoblastic leukemia
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Seber, Adriana

; Castro Junior, Claudio Galvãode de

; Kerbauy, Lucila N

; Hirayama, Alexandre V

; Bonfim, Carmem

; Fernandes, Juliana Folloni

; Souza, Mair

; Schafell, Rony

; Nabhan, Samir

; Loggetto, Sandra Regina

; Simões, Belinda Pinto

; Rocha, Vanderson

; Lima, Marcos de

; Guerino-Cunha, Renato L

; Bittencourt, Henrique

Hematology, Transfusion and Cell Therapy

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2021, Volume 43 Supl. 2 Páginas 13 - 21

Resumo: En | Texto: En | PDF: En

ABSTRACT Chimeric antigen receptor T (CAR-T) cell therapy is a novel therapeutic modality for acute lymphoblastic leukemia (ALL) with robust outcomes in patients with refractory or relapsed disease. At the same time, CAR-T cell therapy is associated with unique and potentially fatal toxicities, such as cytokine release syndrome (CRS) and neurological toxicities (ICANS). This manuscript aims to provide a consensus of specialists in the fields of Hema- tology Oncology and Cellular Therapy to make recommendations on the current scenario of the use of CAR-T cells in patients with ALL.

https://doi.org/10.1016/j.htct.2021.09.002

Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: children and adolescents
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Nabarrete, Juliana Moura

; Pereira, Andrea Z

; Garófolo, Adriana

; Seber, Adriana

; Venancio, Angela Mandelli

; Grecco, Carlos Eduardo Setanni

; Bonfim, Carmem Maria Sales

; Nakamura, Claudia Harumi

; Fernandes, Daieni

; Campos, Denise Johnsson

; Oliveira, Fernanda Luisa Ceragioli

; Cousseiro, Flávia Krüger

; Rossi, Flávia Feijó Panico

; Gurmini, Jocemara

; Viani, Karina Helena Canton

; Guterres, Luciana Fernandes

; Mantovani, Luiz Fernando Alves Lima

; Darrigo Junior, Luiz Guilherme

; Albuquerque, Maria Isabel Brandão Pires e

; Brumatti, Melina

; Neves, Mirella Aparecida

; Duran, Natália

; Villela, Neysimelia Costa

; Zecchin, Victor Gottardello

; Fernandes, Juliana Folloni

einstein (São Paulo)

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2021, Volume 19 elocation eAE5254

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

RESUMO O Consenso Brasileiro de Nutrição em Transplante de Células-Tronco Hematopoiéticas: crianças e adolescentes foi elaborado com a participação de nutricionistas, médicos nutrólogos e médicos hematologistas pediátricos de 10 centros brasileiros que são referência em transplante de células-tronco hematopoiéticas. O objetivo foi salientar a importância do estado nutricional e da composição corporal durante o tratamento, bem como as principais características relacionadas à avaliação nutricional do paciente. As intenções, ao se estabelecer o consenso, foram aprimorar e padronizar a terapia nutricional durante o transplante de células-tronco hematopoiéticas. O consenso foi aprovado pela Sociedade Brasileira de Transplante de Médula Óssea.

ABSTRACT The Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: Children and Adolescents was developed by dietitians, physicians, and pediatric hematologists from 10 Brazilian reference centers in hematopoietic stem cell transplantation. The aim was to emphasize the importance of nutritional status and body composition during treatment, as well as the main characteristics related to patient´s nutritional assessment. This consensus is intended to improve and standardize nutrition therapy during hematopoietic stem cell transplantation. The consensus was approved by the Brazilian Society of Bone Marrow Transplantation.

https://doi.org/10.31744/einstein_journal/2021ae5254

Investigation of MHC gamma block C4A and C4B polymorphisms in unrelated hematopoietic stem cell transplantation
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Getz, Joselito

; Goldenstein, Monica

; Bonfim, Carmem

; Funke, Vaneuza Moreira

; Colturato, Vergílio

; Hamerschlak, Nelson

; Torres, Margareth

; Sayer, David

; Boldt, Angelica

; Pasquini, Ricardo

; Pereira, Noemi Farah

Hematology, Transfusion and Cell Therapy

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Set 2020, Volume 42 Nº 3 Páginas 221 - 229

Resumo: En | Texto: En | PDF: En

ABSTRACT Background: Immunological life-threatening complications frequently occur in post-hematopoietic stem cell transplantation (HSCT), despite matching recipient and donor (R/D) pairs for classical human leukocyte antigens (HLA). Studies have shown that R/D non-HLA disparities within the major histocompatibility complex (MHC) are associated with adverse effects post-HSCT. Methods: We investigated the impact of mismatches of single-nucleotide polymorphisms (SNPs) in C4A/C4B genes, for showing the highest diversity in the MHC gamma block, on 238 patients who underwent HLA 10/10 unrelated donor (URD) HSCT. The endpoints were acute graft-versus-host disease (aGVHD), chronic graft-versus-host disease (cGVHD) and mortality. One hundred and twenty-nine R/D pairs had 23 C4-SNPs typed by PCR-SSP (Gamma-Type™v.1.0), and 109 R/D pairs had these 23 SNPs identified by next-generation sequencing (NGS) using the Illumina platform. Results: The percentage of patients who received HSC from HLA 10/10 donors with 1-7 mismatches was 42.9%. The R/D pairs were considered C4 mismatched when bearing at least one disparity. These mismatches were not found to be risk factors for aGVHD, cGVHD or mortality after unrelated HSCT when SNPs were analyzed together (matched or mm ≥ 1), independently or according to the percentage of incompatibilities (full match for 23 SNPs; 1-3 mm and >3 mm). An exception was the association between 1-3 mismatches at the composite of SNPs C13193/T14952/T19588 with the development of aGVHD (P = 0.012) and with grades III-IV of this disease (P = 0.004). Conclusion: Our data are not consistent with the hypothesis that disparities in C4A/C4B SNPs increase the risks of post-HSCT adverse effects for the endpoints investigated in this study.

https://doi.org/10.1016/j.htct.2019.06.004 460 downloads

A consensus document for the clinical management of invasive fungal diseases in pediatric patients with hematologic cancer and/or undergoing hematopoietic stem cell transplantation in Brazilian medical centers
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Carlesse, Fabianne

; Daudt, Liane Esteves

; Seber, Adriana

; Dutra, Álvaro Pimenta

; Melo, Analy Salles de Azevedo

; Simões, Belinda

; Macedo, Carla Renata Donato

; Bonfim, Carmem

; Benites, Eliana

; Gregianin, Lauro

; Batista, Marjorie Vieira

; Abramczyk, Marcelo

; Tostes, Vivian

; Lederman, Henrique Manoel

; Lee, Maria Lúcia de Martino

; Loggetto, Sandra

; Castro Junior, Cláudio Galvão de

; Colombo, Arnaldo Lopes

Brazilian Journal of Infectious Diseases

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Dez 2019, Volume 23 Nº 6 Páginas 395 - 409

Resumo: En | Texto: En | PDF: En

ABSTRACT In the present paper we summarize the suggestions of a multidisciplinary group including experts in pediatric oncology and infectious diseases who reviewed the medical literature to elaborate a consensus document (CD) for the diagnosis and clinical management of invasive fungal diseases (IFDs) in children with hematologic cancer and those who underwent hematopoietic stem-cell transplantation. All major multicenter studies designed to characterize the epidemiology of IFDs in children with cancer, as well as all randomized clinical trials addressing empirical and targeted antifungal therapy were reviewed. In the absence of randomized clinical trials, the best evidence available to support the recommendations were selected. Algorithms for early diagnosis and best clinical management of IFDs are also presented. This document summarizes practical recommendations that will certainly help pediatricians to best treat their patients suffering of invasive fungal diseases.

https://doi.org/10.1016/j.bjid.2019.09.005 894 downloads

PERFIL CLÍNICO DE CRIANÇAS SUBMETIDAS A TRANSPLANTE DE CÉLULAS-TRONCO HEMATOPOIÉTICAS
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Rodrigues, Jéssica Alline Pereira

; Lacerda, Maria Ribeiro

; Gomes, Ingrid Meireles

; Paes, Márcio Roberto

; Ribeiro, Renata Perfeito

; Bonfim, Carmem Maria Sales

Cogitare Enfermagem

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2019, Volume 24 elocation e55967

Resumo: En Es Pt | Texto: En Es Pt | PDF: En | PDF: Es | PDF: Pt

ABSTRACT Objective to identify the clinical profile of children in the hematopoietic stem cell post-transplant period. Method quantitative, cross-sectional, retrospective study, performed in a transplantation service of the South of Brazil, with data from the medical records of children less than 12 years of age, who had undergone transplantation. Measures of central tendency, dispersion and frequency were used for the analysis and the chi-squared and Fisher’s tests to associate variables. Results the mean age was 6.2 years, males, with 92 (66.7%), the diagnosis of Fanconi anemia, with 42 (30.4%), and unrelated allogeneic transplantation, with 71 (51.4%), were predominant. Hospital discharge occurred within 30 days after transplantation for 85 (61.6%) patients and 48 (34.8%) were readmitted. Catheter failures occurred in 11 children (8.0%) and the main outpatient clinical intercurrences were pain, cough, runny nose and fever. Viral infection was associated with the unrelated transplant and graft-versus-host disease. Conclusion the profile identified corroborates the care planning for this population, contributing to the nursing practice.

RESUMEN Objetivo identificar el perfil clínico de niños tras realización de trasplante de células madre hematopoyéticas. Método investigación cuantitativa, trasversal, retrospectiva, en servicio trasplantador de Sur/Brasil, con datos de prontuarios de niños con 12 años sin cumplir, sometidos a trasplante. Para análisis, se utilizaron medidas de tendencia central, dispersión, frecuencias y prueba chi cuadrada y Fisher a fin de asociar variables. Resultados el promedio de edad fue de 6,2 años, predominio del sexo masculino, 92 (66,7%), diagnóstico Anemia de Fanconi 42 (30,4%) y trasplante alógeno sin parentesco 71 (51,4%). El alta hospitalario ocurrió en hasta 30 días tras el trasplante para 85 (61,6%) y 48 (34,8%) se reingresaron. Las pérdidas del catéter atingieron 11 niños (8%) y las principales complicaciones clínicas ambulatorias fueron dolor, tos, secreción nasal y fiebre. Infección viral fue asociada al trasplante sin parentesco y enfermedad del injerto contra huésped. Conclusión el perfil identificado corrobora el planeamiento de cuidados a esa población, contribuyendo con la práctica de enfermería.

RESUMO Objetivo identificar o perfil clínico de crianças em pós-transplante de células-tronco hematopoiéticas. Método pesquisa quantitativa, transversal, retrospectiva, em serviço transplantador do Sul/Brasil, com dados de prontuários de crianças com 12 anos incompletos, submetidas a transplante. Para análise utilizaram-se medidas de tendência central, dispersão, frequências e testes do qui-quadrado e Fisher para associar variáveis. Resultados a média de idade foi de 6,2 anos, predomínio do sexo masculino 92 (66,7%), diagnóstico Anemia de Fanconi 42 (30,4%) e transplante alogênico não aparentado 71 (51,4%). A alta hospitalar aconteceu em até 30 dias pós-transplante para 85 (61,6%) e 48 (34,8%) foram reinternadas. As perdas do cateter acometeram 11 crianças (8%) e as principais intercorrências clínicas ambulatoriais foram dor, tosse, coriza e febre. Infecção viral esteve relacionada ao transplante não aparentado e doença do enxerto contra hospedeiro. Conclusão o perfil identificado corrobora o planejamento de cuidados a esta população, contribuindo com a prática de enfermagem.

https://doi.org/10.5380/ce.v24i0.55967 87 downloads

IN TIME: IMPORTÂNCIA E IMPLICAÇÕES GLOBAIS DATRIAGEM NEONATAL PARA A IMUNODEFICIÊNCIA GRAVE COMBINADA
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Meehan, Cristina A.

; Bonfim, Carmem

; Dasso, Joseph F.

; Costa-Carvalho, Beatriz Tavares

; Condino-Neto, Antonio

; Walter, Jolan E.

Revista Paulista de Pediatria

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Dez 2018, Volume 36 Nº 4 Páginas 388 - 397

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

https://doi.org/10.1590/1984-0462/;2018;36;4;00020 3243 downloads

10.

Late chimerical status after bone marrow transplantation in severe aplastic anemia according to two different preparatory regimens
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Quiroga, Marcia

; Pereira, Noemi Farah

; Bitencourt, Marco Antonio

; Bonfim, Carmem

; Monteiro, Marianna Gendre de Mesquita

; Pasquini, Ricardo

Hematology, Transfusion and Cell Therapy

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Jun 2018, Volume 40 Nº 2 Páginas 112 - 119

Resumo: En | Texto: En | PDF: En

ABSTRACT Background: This study investigated the influence of two conditioning regimens on the chimerical status of 104 patients with acquired severe aplastic anemia. Methods: Patients were monitored for at least 18 months after related bone marrow transplantation and reaching partial or complete hematologic recovery. Group I patients (n = 55) received 200 mg/kg cyclophosphamide alone and Group II (n = 49) received 120 mg/kg cyclophosphamide associated with 12 mg/kg busulfan. Patients were classified in three chimerism levels according to the percentage of donor cells in the peripheral blood. Results: Chimerism ≤50% occurred in 36.4% of Group I and none of Group II; chimerism 51–90% was found in 20.0% of Group I and 10.2% of Group II; and chimerism >90% was found in 43.6% of Group I versus 89.8% of Group II. A significant association (p-value < 0.001) was found between conditioning type and chimerism levels. A higher number of infused cells was associated with higher levels of chimerism only in Group I (p-value = 0.013). Multivariate analysis showed that chimerism >90% is associated with the cyclophosphamide plus busulfan conditioning (p-value < 0.001) and higher number of infused cells (p-value = 0.009), suggesting that these factors are predictive of graft outcome. Regarding hematological recovery, higher chimerism levels were associated with higher neutrophil (p-value = 0.003) and platelet counts (p-value < 0.001) in Group I only. These results show that myeloablative conditioning favors full donor chimerism and non-myeloablative conditioning predisposes to mixed chimerism or autologous recovery of hematopoiesis. Conclusion: These data show that autologous recovery depends on the intensity of immunosuppression and that the immunosuppressive function of cyclophosphamide alone can induce this type of hematopoietic recovery.

https://doi.org/10.1016/j.htct.2017.11.011 514 downloads

11.

Body composition of Fanconi anemia patients after hematopoietic stem cell transplantation
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Silva, Priscilla Peixoto Policarpo da

; Schmit, Daniella

; Bonfim, Carmem

; Campos, Denise Johnsson

; Rabito, Estela Iraci

; Vilela, Regina Maria

Revista Brasileira de Hematologia e Hemoterapia

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Dez 2017, Volume 39 Nº 4 Páginas 318 - 324

Resumo: En | Texto: En | PDF: En

Abstract Introduction: Fanconi anemia is a rare genetic disease linked to bone marrow failure; a possible treatment is hematopoietic stem cell transplantation. Changes in the nutritional status of Fanconi anemia patients are not very well known. This study aimed to characterize body composition of adult, children and adolescent patients with Fanconi anemia who were submitted to hematopoietic stem cell transplantation or not. Methods: This cross-sectional study enrolled 63 patients (29 adults and 34 children and adolescents). Body composition was assessed based on diverse methods, including triceps skin fold, arm circumference, arm muscle area and bioelectrical impedance analysis, as there is no established consensus for this population. Body mass index was also considered as reference according to age. Results: Almost half (48.3%) of the transplanted adult patients were underweight considering body mass index whereas eutrophic status was observed in 66.7% of the children and adolescents submitted to hematopoietic stem cell transplantation and in 80% of those who were not. At least 50% of all groups displayed muscle mass depletion. Half of the transplanted children and adolescents presented short/very short stature for age. Conclusion: All patients presented low muscle stores, underweight was common in adults, and short stature was common in children and adolescents. More studies are needed to detect whether muscle mass loss measured at the early stages of treatment results in higher risk of mortality, considering the importance of muscle mass as an essential body component to prevent mortality related to infectious and non-infectious diseases and the malnutrition inherent to Fanconi anemia.

https://doi.org/10.1016/j.bjhh.2017.06.004 1061 downloads

12.

Unrelated hematopoietic stem cell transplantation in the pediatric population: single institution experience
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Marinho, Daniela Hespanha

; Zanis Neto, José

; Bonfim, Carmem Maria Sales

; Funke, Vaneuza Araujo Moreira

; Ribeiro, Lisandro Lima

Revista Brasileira de Hematologia e Hemoterapia

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Ago 2015, Volume 37 Nº 4 Páginas 236 - 241

Resumo: En | Texto: En | PDF: En

OBJECTIVE: Hematopoietic stem cell transplantation has been successfully used to treat the pediatric population with malignant and non-malignant hematological diseases. This paper reports the results up to 180 days after the procedure of all unrelated hematopoietic stem cell transplantations in pediatric patients that were performed in one institution.METHODS: A retrospective review was performed of all under 18-year-old patients who received unrelated transplantations between 1995 and 2009. Data were analyzed using the log-rank test, Cox stepwise model, Kaplan-Meier method, Fine and Gray model and Fisher's exact test.RESULTS: This study included 118 patients (46.8%) who received bone marrow and 134 (53.2%) who received umbilical cord blood transplants. Engraftment occurred in 89.47% of the patients that received bone marrow and 65.83% of those that received umbilical cord blood (p-value < 0.001). Both neutrophil and platelet engraftments were faster in the bone marrow group. Acute graft-versus-host disease occurred in 48.6% of the patients without statistically significant differences between the two groups (p-value = 0.653). Chronic graft-versus-host disease occurred in 9.2% of the patients with a higher incidence in the bone marrow group (p-value = 0.007). Relapse occurred in 24% of the 96 patients with malignant disease with 2-year cumulative incidences of 45% in the bone marrow group and 25% in the umbilical cord blood group (p-value = 0.117). Five-year overall survival was 47%, with an average survival time of 1207 days, and no significant differences between the groups (p-value = 0.4666).CONCLUSION: Despite delayed engraftment in the umbilical cord blood group, graft-versus-host disease, relapse and survival were similar in both groups.

https://doi.org/10.1016/j.bjhh.2015.05.004 1521 downloads

13.

Adrenoleucodistrofia ligada ao X: correlação entre o escore de Loes e parâmetros do tensor de difusão
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Ono, Sergio Eiji

; Carvalho Neto, Arnolfo de

; Gasparetto, Emerson Leandro

; Coelho, Luiz Otávio de Mattos

; Escuissato, Dante Luiz

; Bonfim, Carmem Maria Sales

; Ribeiro, Lisandro Lima

Radiologia Brasileira

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Dez 2014, Volume 47 Nº 6 Páginas 342 - 349

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Objetivo: Avaliar a correlação dos parâmetros do tensor de difusão com o escore de Loes e se os parâmetros do tensor de difusão poderiam indicar alterações estruturais precoces. Materiais e Métodos: As medidas do tensor de difusão foram obtidas em 30 exames de 14 pacientes com adrenoleucodistrofia ligada ao X e foram correlacionadas com o escore de Loes. Um grupo controle de 28 pacientes masculinos foi avaliado para estabelecer medidas do tensor de difusão pareadas por idade. Análises estatísticas intra e interobservador foram feitas. Resultados: As medidas do tensor de difusão apresentam forte correlação com o escore de Loes segundo o coeficiente de Pearson (r), com valores de –0,86, 0,89, 0,89 e 0,84 para a fração de anisotropia e difusibilidades média, radial e axial (p < 0,01). A análise da mudança nas medidas do tensor de difusão no estágio inicial da doença indica que os valores de difusibilidades média e radial podem ajudar a predizer a progressão da doença. Conclusão: As medidas de parâmetros do tensor de difusão poderiam ser usadas como um adjunto ao escore de Loes, auxiliando no monitoramento da doença e alertando uma possível progressão do escore de Loes na faixa de interesse para decisões terapêuticas.

Objective: The present study was aimed at evaluating the correlation between diffusion tensor imaging parameters and Loes score as well as whether those parameters could indicate early structural alterations. Materials and Methods: Diffusion tensor imaging measurements were obtained in 30 studies of 14 patients with X-linked adrenoleukodystrophy and were correlated with Loes scores. A control group including 28 male patients was created to establish agematched diffusion tensor imaging measurements. Inter- and intraobserver statistical analyses were undertaken. Results: Diffusion tensor imaging measurements presented strong Pearson correlation coefficients (r) of –0.86, 0.89, 0.89 and 0.84 for fractional anisotropy and mean, radial and axial diffusivities (p < 0.01). Analysis of changes in diffusion tensor measurements at early stage of the disease indicates that mean and radial diffusivities might be useful to predict the disease progression. Conclusion: Measurements of diffusion tensor parameters can be used as an adjunct to the Loes score, aiding in the monitoring of the disease and alerting for possible Loes score progression in the range of interest for therapeutic decisions.

https://doi.org/10.1590/0100-3984.2013.1886 7624 downloads

14.

Processo de brincar da criança hospitalizada guiado pelo modelo lúdico
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Santos, Dayane Regina dos

; Bonfim, Carmem Maria Sales

; Mazza, Verônica de Azevedo

; Wall, Marilene Loewen

; Mercês, Nen Nalú Alves das

Cogitare Enfermagem

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Set 2014, Volume 19 Nº 3 Páginas 617 - 620

Resumo: En Es Pt | Texto: En Es Pt | PDF: En | PDF: Es | PDF: Pt

Playing is fundamental for the healthy development of the child, and during hospitalization can be configured as a care strategy, as long as it is applied systematically and grounded in theory. This study's objective is to report the play process guided by the Ludic Model. The participants were three male children, receiving inpatient treatment in a bone marrow transplantation service, between September and October 2012. Data collection occurred through instruments adapted from the Ludic Model, and progressed in three stages: getting to know the child, caring and playing, and accompanying the evolution of the ludic behavior. The children were participative, demonstrating autonomy, pleasure, and maintenance of the occupational role. The Ludic Model was considered an important tool for guiding the comprehensive and humanized care in this context.

El jugar es fundamental para el desarrollo saludable del niño y durante la hospitalización puede configurarse como estrategia de cuidado, desde que su aplicación sea sistematizada y fundamentada. El objetivo del estudio fue relatar el proceso de jugar de acuerdo con el Modelo Lúdico. Los participantes fueron tres niños del sexo masculino, hospitalizados en un servicio de trasplante de médula, entre septiembre y octubre de 2012. Los datos fueron obtenidos por medio de instrumentos adaptados del Modelo Lúdico y eso se desarrolló en tres etapas: conociendo el niño, cuidando y jugando y acompañando la evolución del comportamiento lúdico. Los niños fueron participativos, demostrando autonomía, placer y manutención del papel ocupacional. El Modelo Lúdico fue considerado importante herramienta para nortear el cuidado integral y humanizado en ese contexto.

O brincar é fundamental para o desenvolvimento saudável da criança e durante a hospitalização pode configurar-se como estratégia de cuidado, desde que sua aplicação seja sistematizada e fundamentada. O objetivo do estudo é relatar o processo de brincar guiado pelo Modelo Lúdico. Os participantes foram três crianças do sexo masculino, hospitalizadas em um serviço de transplante de medula óssea, entre setembro e outubro de 2012. A coleta dos dados se deu por meio de instrumentos adaptados do Modelo Lúdico e se desenvolveu em três etapas: conhecendo a criança, cuidando e brincando e acompanhando a evolução do comportamento lúdico. As crianças foram participativas, demonstrando autonomia, prazer e manutenção do papel ocupacional. O Modelo Lúdico foi considerado importante ferramenta para nortear o cuidado integral e humanizado nesse contexto.

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15.

Vitamin D deficiency in children and adolescents submitted to hematopoietic stem cell transplantation
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Campos, Denise Johnsson

; Biagini, Gleyne Lopes Kujew

; Funke, Vaneuza Araujo Moreira

; Bonfim, Carmem Maria Sales

; Boguszewski, César Luiz

; Borba, Victória Zeghbi Cochenski

Revista Brasileira de Hematologia e Hemoterapia

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Abr 2014, Volume 36 Nº 2 Páginas 126 - 131

Resumo: En | Texto: En | PDF: En

Background: Sub-optimal levels of vitamin D have been found to be highly prevalent in all age groups, with epidemiologic studies demonstrating a link between vitamin D deficiency and disease susceptibility, such as infection and cancer, and mortality rates. In adult transplant patients, it has been suggested that the immunomodulatory properties of vitamin D may have an important role in the prevention and treatment of graft-versus-host disease. Objective: The objective of this study was to assess serum 25-hydroxyvitamin D levels of children and adolescents submitted to allogeneic hematopoietic stem cell transplantation. Methods: Serum 25-hydroxyvitamin D levels of 66 patients, aged 4-20 years, were assessed at three stages: before hospitalization for hematopoietic stem cell transplantation and at 30 and 180 days after hematopoietic stem cell transplantation. The control group consisted of 25 healthy children. Results: At the pre-hematopoietic stem cell transplantation stage, patients had lower levels of 25-hydroxyvitamin D compared to controls (25.7 ± 12.3 ng/mL vs. 31.9 ± 9.9 ng/mL; p-value = 0.01), and a higher prevalence of 25-hydroxyvitamin D deficiency (32% vs. 8%; p-value = 0.01). Prevalence increased significantly after hematopoietic stem cell transplantation (p-value = 0.01) with half of the patients having vitamin D deficiency at 180 days after transplantation. At this stage, mean serum 25-hydroxyvitamin D levels were 20.9 ± 10.9 ng/mL, a significant decline in relation to baseline (p-value = 0.01). No correlation was found between 25-hydroxyvitamin D levels and vitamin D intake, graft-versus-host disease, corticoid use or survival rates. Conclusion: Low levels of 25-hydroxyvitamin D were detected even before hematopoietic stem cell transplantation and were significantly lower at 180 days after hematopoietic stem cell transplantation, thus recommending vitamin D supplementation for children and adolescents submitted to hematopoietic stem cell transplantation.

https://doi.org/10.5581/1516-8484.20140029 2088 downloads Citado 2 vezes em SciELO

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