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1.
Arch Bronconeumol ; 2024 May 04.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-38782632

RESUMO

INTRODUCTION: The effectiveness of home high flow nasal cannula (HFNC) for the treatment of chronic respiratory failure in patients with chronic respiratory diseases (CRDs) has not been summarized. We aimed to conduct a systematic review of the effectiveness, adherence, and safety of HFNC in the long-term treatment of patients with chronic respiratory diseases and respiratory failure. METHODS: A systematic review was conducted. PubMed, Web of science, and SCOPUS were search up to August 2023. Long-term HFNC studies (≥4 weeks) reporting dyspnea; exacerbations, hospitalizations; peripheral oxygen saturation (SpO2), comfort; patient experience, health-related quality of life or partial pressure of carbon dioxide (paCO2) were included. RESULTS: Thirteen articles (701 patients) based on 10 studies were selected: randomized control trials (n=3), randomized crossover trials (n=2), crossover (n=3) and retrospective (n=2) studies. COPD (n=6), bronchiectasis (n=2), COPD/bronchiectasis (n=1) and ILD (n=1) were the underlined CRDs. HFNC reduced exacerbations when compared to usual care/home respiratory therapies (n=6). Quality of life outcomes were also in favor of HFNC in patients with COPD and bronchiectasis (n=6). HFNC had significant effects on hospitalizations, paCO2, and lung function. Adherence ranged from 5.2 to 8.6h/day (n=5). Three studies reported no events, 3 non-serious events and 2 no differences compared with other home respiratory therapies. CONCLUSIONS: HFNC seems more effective than usual care or other home respiratory therapies in reducing exacerbations and improving quality of life in patients with COPD and bronchiectasis, while presenting good adherence and being safe. Its apparently superior effectiveness needs to be better studied in future real-world pragmatic trials.

2.
Ann Hematol ; 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38763941

RESUMO

REHem-AR was created in 2013. The progressive implementation of neonatal screening for haemoglobinopathies in Spanish autonomous communities where the registry had not been implemented, as well as the addition of new centres during this period, has considerably increased the sample of patients covered. In this study, we update our previous publication in this area, after a follow-up of more than 5 years. An observational, descriptive, multicentre and ambispective study of adult and paediatric patients with haemoglobinopathies and rare anaemias registered in REHem was performed. The data are from a cross-sectional analysis performed on 1 June, 2023. The study population comprised 1,756 patients, of whom 1,317 had SCD, 214 had thalassaemia and 224 were diagnosed with another condition. Slightly more than one third of SCD patients (37%) were diagnosed based on neonatal bloodspot screening, and the mean age at diagnosis was 2.5 years; 71% of thalassaemia patients were diagnosed based on the presence of anaemia. Vaso-occlusive crisis and acute chest syndrome continue to be the most frequent complications in SCD. HSCT was performed in 83 patients with SCD and in 50 patients with thalassaemia. Since the previous publication, REHem-AR has grown in size by more than 500 cases. SCD and TM are less frequent in Spain than in other European countries, although the data show that rare anaemias are frequent within rare diseases. REHem-AR constitutes an important structure for following the natural history of rare anaemias and enables us to calculate investment needs for current and future treatments.

3.
Polymers (Basel) ; 16(3)2024 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-38337320

RESUMO

This study is focused on investigating the rheological and mechanical properties of highly oxidized graphite (GrO) incorporated into a poly (lactic acid) (PLA) matrix composite. Furthermore, the samples were annealed at 110 °C for 30 min to study whether GrO concentration has an effect on the elastic modulus (E') after treatment. The incorporation of GrO into PLA was carried out by employing an internal mixing chamber at 190 °C. Six formulations were prepared with GrO concentrations of 0, 0.1, 0.5, 1, 1.5, and 3 wt%. The thermal stability, thermomechanical behavior, and crystallinity of the composites were evaluated utilizing thermogravimetric analysis (TGA), dynamic mechanical analysis (DMA), and differential scanning calorimetry DSC, respectively. The thermal stability (according to Tmax) of the PLA/GrO composites did not change substantially compared with PLA. According to DSC, the crystallinity increased until the GrO concentration reached 1 wt% and afterward decreased. Regarding the heat treatment of the PLA/GrO composites, the E' increased (by two orders of magnitude) at 80 °C with the maximum value achieved at 1 wt% GrO compared with the non-heat-treated composites.

4.
J Cancer Educ ; 39(1): 50-57, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37875743

RESUMO

Multiple myeloma, the second most common hematologic malignancy worldwide, is an aggressive disease with high morbidity and mortality rates. Although myeloma remains incurable, new treatments have improved patients' life expectancy and quality of life. However, as these therapies are administered for prolonged and often indefinite periods, their success depends on high treatment adherence and significant patient engagement. This study aimed to evaluate the impact of a novel digital educational strategy on treatment adherence, quality of life, and the development of complications in patients with newly diagnosed myeloma. To this end, a two-arm, randomized, prospective, double-blind study was conducted to compare the conventional educational approach alone or combined with the novel digital strategy. This strategy was based on some principles of the Persuasive Systems Design model and incorporated the educational recommendations of patients and caregivers. Compared to the control group that only received information through the conventional educational approach, patients randomized to the digital strategy showed significantly higher treatment adherence and quality of life, associated with increased functionality and rapid reincorporation into daily routines. The digital strategy empowered patients and caregivers to understand the disease and therapeutic options and helped patients recall treatment information and implement healthy lifestyle habits. These results support that patient-targeted educational strategies can positively influence treatment adherence and thus improve their quality of life.


Assuntos
Mieloma Múltiplo , Humanos , Mieloma Múltiplo/tratamento farmacológico , Qualidade de Vida , Estudos Prospectivos , Cooperação e Adesão ao Tratamento , Estilo de Vida
5.
Ann Hematol ; 103(2): 583-591, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37923805

RESUMO

Although it is known that increasing age is associated with increased morbidity and mortality in allogeneic transplantation (allo-HSCT), individualization of the process may allow to perform it in progressively older patients.This study analyzed the outcome of 97 patients older than 60 years with a first allo-HSCT performed at our institution between 2011 and 2019.Median age was 66 years (range 60-79) and 15.4% were older than 70 years. The most frequent diagnosis was acute leukemia (50.5%), and 58.8% received a myeloablative conditioning. With a median follow-up of 33.9 months (range 7.9-111.5), at 3-years overall survival (OS) was 50%; progression-free survival (PFS), 46%; cumulative incidence of relapse, 22%; and non-relapse mortality (NRM), 32%. There were no significant differences in OS (p = 0.415), PFS (p = 0.691), cumulative incidence of relapse (p = 0.357) or NRM (p = 0.658) between patients of 60-64 years (n = 37), 65-69 (n = 45) and ≥ 70 years (n = 15). No differences were observed either depending on the intensity of the conditioning regimen in terms of OS (p = 0.858), PFS (p = 0.729), cumulative incidence of relapse (p = 0.416) or NRM (p = 0.270).In conclusion, older adults can safely and effectively undergo allo-HSCT with proper patient selection and individualized transplantation procedures.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Humanos , Idoso , Pessoa de Meia-Idade , Estudos de Viabilidade , Estudos Retrospectivos , Leucemia Mieloide Aguda/complicações , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Recidiva , Condicionamento Pré-Transplante/métodos , Doença Enxerto-Hospedeiro/etiologia
6.
Transplant Cell Ther ; 29(10): 621.e1-621.e6, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37454760

RESUMO

Children with acquired hypocellular bone marrow failure of unknown cause (AHBMF) are usually diagnosed either with severe aplastic anemia (SAA) or refractory cytopenia of childhood (RCC). Patients with AHBMF who lack a matched donor and who failed or relapsed after immunosuppressive therapy (IST) need alternative therapies. Haploidentical hematopoietic stem cell transplantation (haplo-HSCT) offers a curative treatment for these patients. We report a multicenter Spanish experience with haplo-HSCT in pediatric patients with AHBMF. Eleven pediatric patients (SAA, n = 9; RCC, n = 2) underwent haplo-HSCT with different lymphodepletion strategies. Most patients (10 of 11) had previously failed to respond or relapsed after IST. The conditioning regimen was reduced intensity in SAA and myeloablative in RCC. Patients with SAA received low-dose radiotherapy as part of their conditioning regimen. All patients engrafted. Viral reactivation was common (8 of 11). Acute GVHD grade ≥II was seen in 5 patients. Chronic GVHD was diagnosed in 4 of the long-term survivors. Transplantation-associated microangiopathy was a frequent complication in SAA patients and was related to worse outcome. Two patients died of transplantation-related complications. Overall survival was 81%, with a median follow-up of 36 months. Haplo-HSCT can be a successful salvage curative treatment for pediatric patients with AHBMF, but with significant toxicities that must be addressed. Transplantation-associated microangiopathy was the most critical complication.

7.
Front Pediatr ; 11: 1140637, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37020654

RESUMO

Introduction: Studies addressing the role of haploidentical as alternative to HLA-matched donors for stem cell transplantation (SCT) often include patients with diverse hematological malignancies in different remission statuses. Methods: We compared outcomes of children with acute lymphoblastic leukemia (ALL) undergoing SCT in second complete remission (CR2) from haploidentical (n = 25) versus HLA-matched donor (n = 51). Results: Patients were equally distributed across both groups according to age, immunophenotype, time to and site of relapse, relapse risk-group allocation, and minimal residual disease (MRD) before SCT. Incidence of graft failure, acute graft versus host disease (GVHD), and other early complications did not differ between both groups. We found no differences in overall survival (58.7% versus 59.5%; p = .8), leukemia free survival (LFS) (48% versus 36.4%; p = .5), event free survival (40% versus 34.4%; p = .69), cumulative incidence (CI) of subsequent relapse (28% versus 40.9%; p = .69), treatment related mortality (24% versus 23.6%; p = .83), CI of cGVHD (4.5% versus 18.7%; p = .2), and chronic GVHD-free and leukemia-free survival (44% versus 26.3%; p = .3) after haploidentical donor SCT. Chronic GVHD (HR = 0.09; p=.02) had protective impact, and MRD ≥ 0.01% before SCT (HR = 2.59; p=.01) had unfavorable impact on LFS. Discussion: These results support the role of haploidentical donor SCT in children with ALL in CR2.

8.
Acta Psychol (Amst) ; 235: 103900, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37004420

RESUMO

The Roberts Apperception Test for Children (RATC) is a projective method developed by McArthur and Roberts (1982) to assess children and adolescents' behavioural, social and emotional functioning, concerns, conflicts and emotional management strategies through their perceptions of common interpersonal situations of everyday life. The aim of the present study is to contribute to the validation of the RATC in a forensic sample (N = 75) [constituted by a group of juvenile delinquents detained in educational centres (n = 40, 12-17 years old, 1-10 years of schooling) and a group of maltreated adolescents integrated in residential care (n = 35, 11-16 years old, 5-10 years of schooling)], studying its psychometric properties, such as reliability and criterion validity (convergent and discriminant validity), considering the results obtained in other instruments as external validation criteria: Wechsler Intelligence Scale for Children - Third Edition (WISC-III) and Youth Self-Report (YSR). It also aims to search for some indicators based on means and standard deviations to interpret the scores obtained in RATC for these forensic contexts, through the comparisons within forensic groups and the comparisons of the forensic groups with a community sample. The RATC showed minimally acceptable reliability and adequate validity indices, considering that this instrument is a projective method. This limitation is compensated by the clinical value of the data obtained from the projection of individuals' thoughts, concerns, conflicts and problem-solving styles, which are useful to assess their emotional and behavioural characteristics and psychological functioning. The results also show statistically significant differences between the two forensic groups on RATC scales, as well as between them and the community sample, as expected, underlining their different characteristics.


Assuntos
Emoções , Resolução de Problemas , Humanos , Criança , Adolescente , Reprodutibilidade dos Testes , Inquéritos e Questionários , Autorrelato , Psicometria
9.
Polymers (Basel) ; 15(2)2023 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-36679186

RESUMO

In this study, poly(lactic acid) (PLA) blended with different natural waxes (beeswax, candelilla, carnauba, and cocoa) was investigated. Different wax amounts, 3, 5, 10, and 15 wt%, were incorporated into the PLA using a Brabender internal mixer. The blends were characterized by thermogravimetric analysis (TGA), differential scanning calorimetry (DSC), rotational rheometer (RR), dynamic mechanical analysis (DMA), and contact angle to observe the effect of the different waxes on the PLA physicochemical, rheological, mechanical behavior, and wetting properties. The complex viscosity of the blends was studied by employing a RR. The effect of the addition of the waxes on the mechanical properties of PLA was evaluated by DMA in the tension modality. A slight decrease in the thermal stability of PLA was observed with the addition of the waxes. However, in the case of the mechanical properties, the cocoa wax showed a considerable effect, especially in the elongation at break of PLA. Likewise, waxes had an essential impact on the water affinity of PLA. Specifically, with the addition of cocoa, the PLA became more hydrophilic, while the rest of the waxes increased the hydrophobic character.

10.
Leukemia ; 37(1): 53-60, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36310183

RESUMO

Chimeric antigen receptor T cells targeting CD19 (CART-19) have shown remarkable efficacy for relapsed/refractory (R/R) B-cell precursor acute lymphoblastic leukemia (BCP-ALL). We investigated whether prior use of inotuzumab ozogamicin (InO), an anti-CD22 antibody conjugated to calicheamicin, may impact CAR T-cell manufacturing or efficacy via pre-CART-19 depletion of the B-cell compartment. In this international, retrospective analysis, 39 children and young adults receiving InO before (n = 12) and/or after (n = 27) T-cell apheresis as bridging therapy to CART-19 treatment were analyzed. Median age at infusion was 13 years (range 1.4-23 years). Thirty-four out of 39 patients (87.2%) obtained complete remission. With a median follow-up of 18.2 months after CART-19 infusion, 12-month event-free survival (EFS) was 53.3% (95% confidence interval (CI): 38.7-73.4) and overall survival (OS) was 77.8% (95% CI: 64.5-93.9). Seventeen patients (44%) relapsed with a median of 159 days (range 28-655) after CART-19 infusion. No difference in day 28 minimal residual disease negative complete response rate, 12-month OS/EFS, or incidence of CD19-positive or -negative relapses was observed among patients receiving InO before or after apheresis. Compared to published data for patients treated with CART-19 therapy without prior InO exposure, response and OS/EFS for patients treated with InO prior to CART-19 are similar.


Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras , Receptores de Antígenos Quiméricos , Adulto Jovem , Humanos , Criança , Lactente , Pré-Escolar , Adolescente , Adulto , Inotuzumab Ozogamicina , Estudos Retrospectivos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Terapia Baseada em Transplante de Células e Tecidos
11.
An Pediatr (Engl Ed) ; 96(4): 309-318, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35523687

RESUMO

INTRODUCTION: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different centers that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH). RESULTS: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS). HSTC performance from year 2004, in first CR with ATG-based conditioning in addition to acute graft versus host disease (aGvHD) development, increased event free survival (EFS). Treatment with imatinib as well as undetectable minimal residual disease (MRD) prior to HSCT, combined with aGvHD, reduced risk of relapse (RR). Patient age less than 10 years when HSCT, first CR and ATG-based conditioning were associated to a lower transplant related mortality (TRM). CONCLUSIONS: Patients that could achieve first CR that also received ATG-based conditioning had a better OS and EFS, so HSCT should be considered for this group of patients.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Leucemia-Linfoma Linfoblástico de Células Precursoras , Doença Aguda , Criança , Humanos , Mesilato de Imatinib/uso terapêutico , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia
12.
EJIFCC ; 33(4): 325-333, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36605301

RESUMO

Herein we described a retrospective analysis of a 13-year-old female patient with facial dysmorphia and immune disorder caused by BCL11B gene mutation. The patient upon physical examination presented a particular face (thin eyebrows, small mandible, and widened eye distance), delayed language and motor development. Supplementary examination showed expansion of CD8+, absence of type 2 Innate Lymphoid Cells, increased IgG and altered distribution of T cells. Genetic testing revealed a heterozygous frameshift variation in exon 4 of the BCL11B gene; c.1887_c.1893delCGGCGGG (p.Gly630Glyfs*91). Finally, a BCL11B gene mutation could lead to abnormal development of the nervous and immune systems, therefore, it is necessary to consider this syndrome in patients with the clinical and immunological phenotype described below.

13.
Polymers (Basel) ; 13(24)2021 Dec 07.
Artigo em Inglês | MEDLINE | ID: mdl-34960830

RESUMO

In this study, polymer blends with a mechanical property balance based on poly(lactic acid) (PLA), stiff polymer, and elongated polymer were developed. First, the binary blends PLA-elongated polymer [ethyl vinyl acetate (EVA) or polyethylene], or PLA-stiff polymer [polystyrene or poly(styrene-co-methyl methacrylate) (SMMA)] blends were studied using dynamic mechanic analysis (DMA) and analyzed using Minitab statistical software to determine the factors influencing the elongation or stiffness of the blends. Then, ternary blends such as elongation-poly(lactic acid)-stiff, were made from the binary blends that presented optimal performance. In addition, three blends [EVA-PLA-SMMA (EPS)] were elaborated by studying the mixing time (5, 15, and 15 min) and the added time of the SMMA (0, 0, and 10 min). Specifically, the mixing time for EPS 1, EPS 2, and EPS 3 is 5 min, 15 min, and 15 min (first EVA + PLA for 10 min, plus 5 min PLA-EVA and SMMA), respectively. Mechanical, thermal, rheological, and morphological properties of the blends were studied. According to DMA, the results show an increase in elongation at break (εb) and do not decrease the elastic module of poly(lactic acid). Nevertheless, EPS 3 excels in all properties, with an εb of 67% and modulus of elasticity similar to PLA. SMMA has a significant role as a compatibilizing agent and improves PLA processability.

14.
Polymers (Basel) ; 13(20)2021 Oct 19.
Artigo em Inglês | MEDLINE | ID: mdl-34685356

RESUMO

Due to its relatively simple structure, low-density polyethylene (LDPE) can be considered as a model polymer for the study of its properties. Herein, the effect of processing variables on the microstructure and crystallinity of injection-molded LDPE specimens was quantitatively determined. The polymer was injected at different temperature conditions in the barrel and the mold. The specimens were characterized by scanning electron microscopy and X-ray diffraction. With the data obtained, an analysis of variance (ANOVA) was carried out, and response surface graphs (SRP) were constructed to quantify and to observe the behavior of the processing variables, respectively. Different models were obtained to predict the effect of the experimental factors on the response variables. The results showed that the interaction of the two temperatures has the greatest effect on the size of the spherulite, while the temperature of the mold affects the crystallinity. The SRP showed different behaviors: for the spherulite, the size increases with the mold temperature, while for the crystallinity, higher values were observed at an intermediate mold temperature and a low melt temperature. The results presented herein are valuable for setting empirical relations between the microstructure, crystallinity, and the molding conditions of LDPE.

15.
Polymers (Basel) ; 13(14)2021 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-34301099

RESUMO

The use of biomaterials as a replacement for thermoplastic polymers is an environmentally sound strategy. In this work, hydrogels of cellulose isolated from wheat husk were modified by UV irradiation (353 nm) to improve mechanical performance. The cellulose was dissolved with a solvent system N,N-dimethylacetamide/lithium chloride (DMAc/LiCl). Infrared spectroscopy showed that the peak height at 1016 cm-1, associated with the C-O bonds of the glycosidic ring, increases with irradiation time. It was determined that the increase in this signal is related to photodegradation, the product of a progressive increase in exposure to UV radiation. The viscoelastic behavior, determined by dynamic mechanical analysis and rotational rheometry, was taken as the most important parameter of this research, showing that the best results are recorded with 15 min of UV treatment. Therefore, at this time or less, the chemical crosslinking is predominant over the photodegradation, producing an increase in the modules, while with 20 min the photodegradation is such that the modules suffer a significant reduction.

16.
Eur J Haematol ; 106(6): 842-850, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33713387

RESUMO

BACKGROUND: Defibrotide is approved in European Union for the treatment of severe sinusoidal obstruction syndrome (SOS) after HSCT. However, it has also been used for SOS prophylaxis, moderate SOS and in other complications such as transplant-associated thrombotic microangiopathy (TAM). The objective of this study was to evaluate current uses, effectiveness and safety of defibrotide in patients with HSCT. METHODS: This multicenter, retrospective study included patients treated with defibrotide for any indication at 28 HSCT centers of the Grupo Español de Trasplante Hematopoyetico (GETH) including the pediatric subgroup Grupo Español de Trasplante de Medula en Niños (GETMON). RESULTS: Three hundred and eighty eight patients treated with defibrotide between January 2011 and December 2018 were included. 253 patients were children, and 135 patients were adults. In total, 332 transplants were allogeneic, and the remainder were autologous. Main indications for defibrotide use were severe/very severe SOS in 173 patients, SOS prophylaxis in 135 patients, moderate SOS in 41 patients, TAM in six patients and suspected SOS in 33 patients. Overall survival (OS) at day +100 in the SOS prophylaxis group was 89% (95% CI, 87%-91%). In the group of patients with moderate and severe/very severe SOS, the OS at day +100 was 80% (95% CI, 74%-86%) and 62% (95% CI, 59%-65%), respectively (P = .0015). With a longer follow-up, median of 2 years (4 months-7 years), OS was 63% (95% CI, 59%-67%) in the SOS prophylaxis patients. OS for patients with moderate and severe/very severe SOS groups was 53% (95% CI, 47%-61%) and 26% (95% CI, 22%-30%), respectively (P = .006). 191 patients died, and SOS was the main cause of death in 23 patients (12%). CONCLUSIONS: Defibrotide has an acceptable safety profile with an improved response in severe/very severe SOS compared with historical controls, mainly in pediatric patients. Use of defibrotide for prophylaxis may improve prognosis of patients at high risk of complications due to endothelial damage such as those who receive a second transplant. SOS has an important impact on the HSCT long-term survival, as can be concluded from our study.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Polidesoxirribonucleotídeos/administração & dosagem , Microangiopatias Trombóticas , Adolescente , Adulto , Idoso , Aloenxertos , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Espanha/epidemiologia , Taxa de Sobrevida , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/mortalidade , Microangiopatias Trombóticas/prevenção & controle
17.
An Pediatr (Engl Ed) ; 2021 Mar 26.
Artigo em Espanhol | MEDLINE | ID: mdl-33781716

RESUMO

INTRODUCTION: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different center that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH). RESULTS: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS). HSTC performance from year 2004, in first CR with ATG-based conditioning in addition to acute graft versus host disease (aGvHD) development, increased event free survival (EFS). Treatment with imatinib as well as undetectable minimal residual disease (MRD) prior to HSCT, combined with aGvHD, reduced risk of relapse (RR). Patient age less than 10 years when HSCT, first CR and ATG-based conditioning were associated to a lower transplant related mortality (TRM). CONCLUSIONS: Patients that could achieve first CR that also received ATG-based conditioning had a better OS and EFS, so HSCT should be considered for this group of patients.

18.
Molecules ; 26(1)2020 Dec 24.
Artigo em Inglês | MEDLINE | ID: mdl-33374385

RESUMO

This study aimed to obtain a third-generation snack from native rice starch (NS), rice starch modified by extrusion (MS), nopal flour (NF) and xanthan gum (XG). These raw materials were characterized by proximal analysis, pH, particle size distribution, water absorption index (WAI) and water solubility index (WSI), degree of substitution (DS), differential scanning calorimetry (DSC), rheology, Fourier transform infrared spectroscopy (FT-IR) and scanning electron microscopy (SEM). The analysis of the response variables in the nine formulations of the snack: expansion index (EI), apparent density (AD), hardness (H), luminosity (L*) and tendency to green-red (a*), was performed through a composite central design (CCD), the selected formulations were characterized by SEM. Results showed an increase in WAI, 4.69 ± 0.04, and WSI, 12.61 ± 0.10, for MS, higher than NS values due to chemical modification. According to the color analysis the NF obtained a value of 60.73 ± 0.008 in L* and -6.51 ± 0.004 in a* with green tendency. The DS value obtained was 0.09 ± 0.005, being within the FDA's permissible range for food use. By FTIR analysis, the acetyl group was corroborated. Finally, employing microwave cooking, snacks made from NS with concentrations of NF (5%) and XG (0%) obtained the highest EI value, 4.47, as well the low Dap and D value (0.37 g/cm3, 2.25 N, respectively), corroborated by SEM analysis.


Assuntos
Farinha/análise , Opuntia/química , Oryza/química , Lanches , Amido/química , Algoritmos , Fenômenos Químicos , Análise de Alimentos , Modelos Teóricos , Solubilidade , Análise Espectral , Amido/ultraestrutura
19.
Br J Haematol ; 190(5): 764-771, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32314348

RESUMO

Blinatumomab and inotuzumab ozogamycin represent promising alternatives to conventional chemotherapy in acute lymphoblastic leukaemia (ALL). We analysed data from 29 children with ALL treated under compassionate use with blinatumomab, inotuzumab or both. The complete remission (CR) rate in a heavily pretreated population with overt relapse was 47·6%. At earlier stages (first/second CR), both antibodies represented a useful tool to reduce minimal residual disease, and/or avoid further toxic chemotherapy until stem cell transplantation. Six patients developed grade 3 reversible non-haematological toxicity. The 12-month overall survival and event-free survival rates were 50·8 ± 26·4% and 38·9 ± 25·3% with blinatumomab, 45·8 ± 26% and 27·5 ± 25% with inotuzumab.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras B , Adolescente , Anticorpos Biespecíficos/administração & dosagem , Anticorpos Biespecíficos/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Hematologia , Humanos , Lactente , Inotuzumab Ozogamicina/administração & dosagem , Inotuzumab Ozogamicina/efeitos adversos , Masculino , Oncologia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/mortalidade , Estudos Retrospectivos , Sociedades Médicas , Espanha/epidemiologia , Taxa de Sobrevida
20.
Molecules ; 24(13)2019 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-31269663

RESUMO

This study aimed to obtain a second-generation snack by extrusion from the by-product of rice milling enriched with amaranth. The raw material used was amaranth flour (AF), rice starch (NS) and modified rice starch (MS), which were evaluated by the analysis of substitution degree (SD), differential scanning calorimetry (DSC), viscosity (RVA), Fourier transform infrared spectroscopy (FT-IR) and X-ray diffraction (XRD). The snacks were expanded by extrusion and microwave oven, as a reference method. The samples were evaluated in hardness (D), expansion index (EI), apparent density (DAP), and protein content (P). Afterward, the optimized samples were evaluated by scanning electron microscopy (SEM) and resistant starch (RS). During the thermal characterization, a clear trend in the decrement in gelatinization temperatures was observed (78.35 to 63.90 °C in NS and MS respectively). The curves obtained in RVA analyses showed typical behavior of native (6.35 Pa.s) and extruded starches (2.88 Pa.s), with a significant decrease in viscosity peak. Through the analysis of FT-IR, the introduction of the functional acetyl group (stretching at a wavelength of 1735 cm-1) was corroborated. Snack samples results showed a maximum hardness in MS, with a value of 121 N, and the NS (100%) presented the highest EI value (1.41). The lowest DAP values were obtained for the MS (0.48 g/cm3, 100%) and AF (0.49 g/cm3, 100%) samples. P increased to a higher concentration of AF. In the optimum formulation, the SEM image showed that the expanded microwave sample increased the porosity and obtained an RS value of 8.2%. The formulation obtained in the present study presents high characteristics to be used in the development of a healthy snack.


Assuntos
Amaranthus/química , Oryza/química , Lanches , Amido/química , Acetilação , Amilose/química , Cor , Cristalização , Dureza , Espectroscopia de Infravermelho com Transformada de Fourier , Propriedades de Superfície , Temperatura , Viscosidade , Difração de Raios X
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