Quality of life, self-reported outcomes and impact of education among people with moderate and severe hemophilia A: An integrated perspective from a Latin American country.

Collecting and interpreting self-reported outcomes among people with hemophilia A supports the understanding of the burden of the disease and its treatment to improve holistic care. However, in Colombia, this information is limited. Therefore, this study aimed to describe the knowledge, perception and burden of hemophilia A from the patients' perspective. A cross-sectional study was conducted in the context of a hemophilia educational bootcamp held from November 29th to December 1st, 2019, in Medellin, Colombia. The bootcamp was organized by a hemophilia patient association responsible for contacting and inviting patients with hemophilia A (PwHA). Information on patients' health beliefs, treatment experiences, and health-related quality of life (HRQoL) was obtained through focus groups, individual interviews and the Patient Reported Outcomes, Burdens and Experiences (PROBE) questionnaire. A total of 25 moderate or severe PwHA were enrolled in this study and completed the PROBE questionnaire. Acute pain was the most frequently reported symptom, with 88% of the patients reporting the use of pain medication. Difficulty with activities of daily living was reported by 48%. Furthermore, 52% reported having more than 2 spontaneous bleeding events in the last year. Treatment was administered at home for 72% of patients, with regular prophylaxis as the most common treatment regimen. In terms of overall HRQoL, the median EQ-5D VAS score was 80 (IQR: 50-100). PwHA in Colombia still suffer from disease complications related to bleeding events, pain and disability that affect their HRQoL, which highlights the need to develop patient-centered initiatives to improve the wellness of this population.

Trypanosoma cruzi infection in transfusion medicine

ABSTRACT Introduction: Infection by Trypanosoma cruzi is challenging to blood bank supplies in terms of accurate diagnosis, mostly due to its clinical complexity. Infected individuals may remain asymptomatic for years, albeit they may have circulating parasites potentially transferable to eventual receptors of a transfusion. Objective: Although risk donors are systematically excluded through a survey, an important residual risk for transmission remains, evidencing the need to implement additional actions for the detection of T. cruzi in blood banks. Method: A review of the scientific literature is presented with the objective of identifying relevant publications on this subject. Results: We discuss the diagnostic considerations of this chronic infection on transfusion medicine and some recent advances in the processing of blood and derivatives units. Conclusion: Finally, recommendations are made on how the transmission of T. cruzi can be avoided through the implementation of better diagnostic and pathogen control measures at blood banks.

Trypanosoma cruzi infection in transfusion medicine.

INTRODUCTION: Infection by Trypanosoma cruzi is challenging to blood bank supplies in terms of accurate diagnosis, mostly due to its clinical complexity. Infected individuals may remain asymptomatic for years, albeit they may have circulating parasites potentially transferable to eventual receptors of a transfusion. OBJECTIVE: Although risk donors are systematically excluded through a survey, an important residual risk for transmission remains, evidencing the need to implement additional actions for the detection of T. cruzi in blood banks. METHOD: A review of the scientific literature is presented with the objective of identifying relevant publications on this subject. RESULTS: We discuss the diagnostic considerations of this chronic infection on transfusion medicine and some recent advances in the processing of blood and derivatives units. CONCLUSION: Finally, recommendations are made on how the transmission of T. cruzi can be avoided through the implementation of better diagnostic and pathogen control measures at blood banks.

Diurnal changes of arterial oxygen saturation and erythropoietin concentration in male and female highlanders.

In Caucasians and Native Americans living at altitude, hemoglobin mass is increased in spite of erythropoietin concentrations ([Epo]) not markedly differing from sea level values. We hypothesized that a nocturnal decrease of arterial oxygen saturation (SaO2) causes a temporary rise of [Epo] not detected by morning measurements. SaO2 (continuous, finger oximeter) and [Epo] (ELISA, every 4 h) were determined in young highlanders (altitude 2600 m) during 24 h of usual daily activity. In Series I (six male, nine female students), SaO2 fell during the night with the nadir occurring between 01:00 and 03:00; daily means (range 92.4-95.2%) were higher in females (+1.7%, P < 0.01). [Epo] showed opposite changes with zenith occurring at 04:00 without a sex difference. Mean daily values (22.9 ± 10.7SD U/L) were higher than values obtained at 08:00 (17.2 ± 9.5 U/L, P < 0.05). In Series II (seven females), only SaO2 was measured. During follicular and luteal phases, SaO2 variation was similar to Series I, but the rhythm was disturbed during menstruation. While daily [Epo] variations at sea level are not homogeneous, there is a diurnal variation at altitude following changes in SaO2 Larger hypoventilation-dependent decreases of alveolar PO2 decreases during the night probably cause a stronger reduction of SaO2 in highlanders compared to lowlanders. This variation might be enlarged by a diurnal fluctuation of Hb concentration. In spite of a lower [Hb], the higher SaO2 in women compared to men led to a similar arterial oxygen content, likely explaining the absence of differences in [Epo] between sexes.

Erythropoietin reduces storage lesions and decreases apoptosis indices in blood bank red blood cells.

BACKGROUND: Recent evidence shows a selective destruction of the youngest circulating red blood cells (neocytolysis) trigged by a drop in erythropoietin levels. OBJECTIVE: The aim of this study was to evaluate the effect of recombinant human erythropoietin beta on the red blood cell storage lesion and apoptosis indices under blood bank conditions. METHODS: Each one of ten red blood cell units preserved in additive solution 5 was divided in two volumes of 100mL and assigned to one of two groups: erythropoietin (addition of 665IU of recombinant human erythropoietin) and control (isotonic buffer solution was added). The pharmacokinetic parameters of erythropoietin were estimated and the following parameters were measured weekly, for six weeks: Immunoreactive erythropoietin, hemolysis, percentage of non-discocytes, adenosine triphosphate, glucose, lactate, lactate dehydrogenase, and annexin-V/esterase activity. The t-test or Wilcoxon's test was used for statistical analysis with significance being set for a p-value <0.05. RESULTS: Erythropoietin, when added to red blood cell units, has a half-life >6 weeks under blood bank conditions, with persistent supernatant concentrations of erythropoietin during the entire storage period. Adenosine triphosphate was higher in the Erythropoietin Group in Week 6 (4.19±0.05µmol/L vs. 3.53±0.02µmol/L; p-value=0.009). The number of viable cells in the Erythropoietin Group was higher than in the Control Group (77%±3.8% vs. 71%±2.3%; p-value <0.05), while the number of apoptotic cells was lower (9.4%±0.3% vs. 22%±0.8%; p-value <0.05). CONCLUSIONS: Under standard blood bank conditions, an important proportion of red blood cells satisfy the criteria of apoptosis. Recombinant human erythropoietin beta seems to improve storage lesion parameters and mitigate apoptosis.

Erythropoietin reduces storage lesions and decreases apoptosis indices in blood bank red blood cells

ABSTRACT Background: Recent evidence shows a selective destruction of the youngest circulating red blood cells (neocytolysis) trigged by a drop in erythropoietin levels. Objective: The aim of this study was to evaluate the effect of recombinant human erythropoietin beta on the red blood cell storage lesion and apoptosis indices under blood bank conditions. Methods: Each one of ten red blood cell units preserved in additive solution 5 was divided in two volumes of 100 mL and assigned to one of two groups: erythropoietin (addition of 665 IU of recombinant human erythropoietin) and control (isotonic buffer solution was added). The pharmacokinetic parameters of erythropoietin were estimated and the following parameters were measured weekly, for six weeks: Immunoreactive erythropoietin, hemolysis, percentage of non-discocytes, adenosine triphosphate, glucose, lactate, lactate dehydrogenase, and annexin-V/esterase activity. The t-test or Wilcoxon's test was used for statistical analysis with significance being set for a p-value <0.05. Results: Erythropoietin, when added to red blood cell units, has a half-life >6 weeks under blood bank conditions, with persistent supernatant concentrations of erythropoietin during the entire storage period. Adenosine triphosphate was higher in the Erythropoietin Group in Week 6 (4.19 ± 0.05 µmol/L vs. 3.53 ± 0.02 µmol/L; p-value = 0.009). The number of viable cells in the Erythropoietin Group was higher than in the Control Group (77% ± 3.8% vs. 71% ± 2.3%; p-value <0.05), while the number of apoptotic cells was lower (9.4% ± 0.3% vs. 22% ± 0.8%; p-value <0.05). Conclusions: Under standard blood bank conditions, an important proportion of red blood cells satisfy the criteria of apoptosis. Recombinant human erythropoietin beta seems to improve storage lesion parameters and mitigate apoptosis.

Standardization of a Protocol for Obtaining Platelet Rich Plasma from blood Donors; a Tool for Tissue Regeneration Procedures.

BACKGROUND: To develop a protocol for obtaining autologous platelet rich plasma in healthy individuals and to determine the concentration of five major growth factors before platelet activation. This protocol could be integrated into the guidelines of good clinical practice and research in regenerative medicine. METHODS: Platelet rich plasma was isolated by centrifugation from 38 healthy men and 42 women ranging from 18 to 59 years old. The platelet count and quantification of growth factors were analyzed in eighty samples, stratified for age and gender of the donor. Analyses were performed using parametric the t-test or Pearson's analysis for non-parametric distribution. P < 0.05 was considered statistically significant. RESULTS: Our centrifugation protocol allowed us to concentrate basal platelet counts from 1.6 to 4.9 times (mean = 2.8). There was no correlation between platelet concentration and the level of the following growth factors: VEGF-D (r = 0.009, p = 0.4105), VEGF-A (r = 0.0068, p = 0.953), PDGF subunit AA (p = 0.3618; r = 0.1047), PDGF-BB (p = 0.5936; r = 0.6095). In the same way, there was no correlation between donor gender and growth factor concentrations. Only TGF-ß concentration was correlated to platelet concentration (r = 0.3163, p = 0.0175). CONCLUSIONS: The procedure used allowed us to make preparations rich in platelets, low in leukocytes and red blood cells, and sterile. Our results showed biological variations in content of growth factors in PRP. The factors influencing these results should be further studied.

Sobrevida de los recién nacidos en la unidad de cuidados Intensivos neonatales: Hospital Central de San Cristóbal. 2012-2013 / Survival and associated factors in infants admitted to the neonatal intensive care unit: Hospital Central de San Cristóbal, Táchira State. 2012-2013

Introducción: La mortalidad neonatal es el indicador básico para valorar la calidad de la atención en salud del recién nacido. Constituye el 60% o más del total de la mortalidad infantil y 23% de la mortalidad perinatal. La creación de unidades de cuidados intensivos neonatales (UCIN) han mejorado la sobrevida de los recién nacidos sobre todo pre término. El objetivo fue evaluar el tiempo de sobrevida de los recién nacidos en UCIN y analizar los principales factores que la afectan. Métodos: Se realizó un estudio observacional, retrospectivo, predictivo en la Unidad de Cuidados Neonatales (UCIN) del Hospital Central de San Cristóbal. La función de supervivencia se evaluó mediante el estimador no paramétrico de Kaplan Meier siendo el evento a analizar la ocurrencia de la muerte durante la estadía. Resultados: La media estimada de tiempo en UCIN fue de 16,53 (IC95%: 13,93 - 19,13) días, la cual se prolonga si se omiten los ingresos que duran menos de 3 días. La edad gestacional menor de 37 semanas, y el peso al nacimiento menor de 1500 gramos se asocian a tiempos estimados de sobrevida significativamente más cortos (p<0,01). Conclusiones: La función de supervivencia puede detallar los principales problemas de funcionamiento de la UCIN. Es necesario establecer protocolos más eficaces con los equipos diagnósticos y asistenciales, como la disposición de los medicamentos e insumos de manera efectiva y oportuna para mejorar la sobrevida de los recién nacidos de bajo peso y menores de 37 semanas.

Introduction: Neonatal mortality is the main indicator to assess the quality of health care of newborns. It represents 60% or more of total infant mortality and 23 % of perinatal mortality. The creation of neonatal intensive care units (NICU) has improved infant survival especially of preterm newborns. The objective of this study was to assess the survival time of newborns in the NICU and to analyze the main factors that may be involved. Methods: A predictive, retrospective, observational study was conducted in the NICU of the Hospital Central of San Cristobal, Táchira state in Venezuela. The survival function was assessed by means of the non-parametric estimator of Kaplan Meier. The event to be analyzed was the occurrence of death during the stay care. Results: The estimated average time in NICU was 16.53 days (IC95: 13.93-19.13), which is becomes longer if the hospitalization periods under 3 days are omitted. Less than 37 weeks gestational age and birth weight less than 1500 grams are associated with estimated survival times significantly shorter (p<0,01). Conclusions: The survival function can detail the major operating problems of the NICU. It is necessary to establish more effective protocols with appropriate diagnostic and health care equipments, such as effective and timely availability of medications and medical supplies in order to improve the survival of the newborn babies with birth weight under 1500 grams and less than 37 weeks gestation.

Prevalence of antibodies against transfusion-transmissible infections (TTI) in blood donors from the Colombian eastern region.

BACKGROUND: The main purpose of this study was to establish the prevalence of antibodies against five transfusion-transmissible infections (TTIs) in blood donors from one of the most important blood banks in Colombia. METHODS: A cross-sectional, descriptive and case control study was performed from a database of Higuera-Escalante blood bank, for a period of a year. Serum was used for donor screening. Surface antigens for hepatitis B (HbsAg), anti-hepatitis C antibodies, Chagas disease, syphilis, and HIV were identified. Chemiluminescent Microparticle Immunoassay (CMIA, Abbott Diagnostics) was performed. RESULTS: From 41,575 total donors analyzed, 1,226 were reactive for any of the infectious markers (total prevalence of 2.95%). The prevalence of specific infections was: Chagas disease 0.49%, HbsAg 0.21%, HCV 0.45%, HIV 0.12%, and syphilis 1.68%. Reactivity was more frequent in men (n = 785, 64%) with a mean age of 36.35 years. HIV was present in the youngest donors with a mean age of 26.5 years (IC 95%: 23.6 - 27.6); on the other hand, Chagas disease was found in the oldest donor population, with a mean age of 40 years (IC 95%: 39.1 - 41.3). CONCLUSIONS: Identifying the prevalence of circulating antibodies against transfusion transmissible infections allows us to establish an epidemiological profile of donors inhabiting the geographic catchment area of our blood bank. Total prevalence in this study was 2.95% for any of the five markers. Syphilis prevalence demonstrates its high distribution within the blood donor population of our country, although this result could be influenced by the high rate of false-reactive test. Chagas disease is endemic in Santander, Colombia, which correlates with the results obtained in this study.

Hemoglobina: una molécula modelo para el investigador / Hemoglobine: a model molecule to researcher

Se presenta la revisión general sobre la hemoglobina, una de las proteínas más estudiadas y mejor caracterizadas. La gran variedad de aspectos científicos que incluye y la importancia que juega en la biología hace que, aunque los primeros estudios científicos se hayan realizado desde el siglo XIX, aún hoy aparezcan sorprendentes descubrimientos acerca de esta molécula, tales como las nuevas globinas, neuroglobina y citoglobina y las llamativas interacciones con el óxido nítrico. Asimismo, el estudio de las hemoglobinopatías constituye un gran reto para la medicina moderna en la medida en que ponga al servicio de sus pacientes los resultados de la investigación científica básica

Alteraciones bioquímicas de los eritrocitos almacenados en condiciones estándar de banco de sangre / Erythrocytes blood bank storage

La forma más habitual de almacenamiento de eritrocitos es en fase líquida. Durante el almacenamiento se producen cambios deletéreos que disminuyen la viabilidad y funcionalidad en el receptor y conllevan a efectos colaterales. Se revisaron los mecanismos que generan los cambios bioquímicos de los eritrocitos