Benefica chirurgia. A global surgery project focusing on hernia surgery.

BACKGROUND: The aim of international health cooperation projects is to alleviate the deficiencies in the area of health in low resource settings. Hernia surgery is a procedure that is well suited to these missions, due to its low morbidity, the fact that it can be performed on an outpatient basis, and the improvement in quality of life that it provides. OBJECTIVE: To describe the results of Benefica Chirurgia (BC), a Spanish non-profit humanitarian association in hernia pathology. METHODS: Five one-week surgical campaigns were carried out in Ecuador between 2015 and 2019, involving anesthetists, general and pediatric surgeons. Surgical and medical equipment was provided and transported by BC. ASA I/II patients underwent surgery. RESULTS: Surgery was performed on 240 patients with hernia pathology on 27 days. Sixty-three per cent of patients were male and the mean age was 48.2 years (range: 1-83). Hernia location was inguinal in 113 patients, umbilical in 101, and other in 26. The anesthetic technique used was spinal in 185 patients (77.1%), local plus intravenous sedation in 31 (12.9%), and general in 24 (10%). The surgical technique used was hernioplasty in 191 patients, herniorrhaphy in 31, incisional hernia repair in 15 and herniotomy in three. Surgery was performed on an outpatient basis in 98.4% of cases. Morbidity was 2%. Long-term postoperative evaluation is very complex. CONCLUSION: These campaigns make a significant contribution to health in low resource settings and provide great personal satisfaction for those involved. Standards achieved in the immediate postoperative period were similar to those obtained at the surgeons' centers in Europe. However, it is difficult to establish the rates of recurrence and chronic pain.

Hernia diafragmática congénita en la gestación gemelar / Congenital diaphragmatic hernia in twin gestation

La hernia diafragmática congénita (HDC) en un feto de una gestación gemelar es una situación extremadamente infrecuente y de difícil manejo. La cirugía fetal no parece una opción válida debido al alto riesgo de rotura prematura de membranas y/o parto prematuro. La interrupción fetal selectiva debe hacerse antes de la semana 22, y tampoco está exenta de riesgos. El manejo expectante se asocia a la prematuridad y a fetos de bajo peso característicos de una gestación gemelar. Por tanto, el asesoramiento a los padres por parte de un equipo multidisciplinar es crucial en la toma de decisiones. La cesárea programada a término parece ser la vía más razonable en estos casos, sobre todo si es el segundo gemelo el afecto por HDC. Se presentan 6 casos de gestaciones gemelares bicoriales con uno de los fetos afecto de HDC con diferentes manejos y evoluciones obstétricas.(AU)

Congenital diaphragmatic hernia (CDH) in a twin gestation foetus is an extremely infrequent and difficult situation to manage. Foetal surgery does not seem to be an option due to the high risk of premature rupture of membranes and/or preterm delivery. Selective foetal termination must be done before 22 weeks and is also not without risk. Expectant management is associated with prematurity and low weight foetuses characteristic of twin gestation. Therefore, parental counselling by a multidisciplinary team is crucial in decision making. Programmed caesarean section at term seems to be the most reasonable route in these cases, especially if the second twin is affected by CDH. Six cases of bicornuate twin gestations with one of the foetuses affected by CDH are presented, with different management and obstetric outcomes.(AU)

Cómo reestructurar una Sección de Medicina Fetal en tiempos de pandemia COVID-19. Medidas de seguridad y limpieza de los equipos ecográficos / How to organize a Fetal Medicine Unit in the context of COVID-19 pandemic. Safe measures for obstetric scans and equipment cleaning

En situación de pandemia, los tres principios básicos en la atención sanitaria son priorizar los recursos, mantener el confinamiento del paciente para evitar la transmisión comunitaria y el colapso sanitario, y reducir la asistencia no prioritaria con el fin de evitar la exposición del paciente y de salvaguardar la salud del profesional sanitario. El control antenatal debe mantenerse durante el periodo de crisis sanitaria, independientemente del estado de alerta COVID-19. La ecografía obstétrica es una prueba fundamental para la toma de decisiones clínicas durante el embarazo, con un impacto en el manejo del binomio madre-feto y en el resultado perinatal, por lo que se ha de garantizar su realización. Con el fin de reducir las visitas presenciales al mínimo número posible, estas se intentarán organizar teniendo en cuenta los controles ecográficos establecidos. Basados en la evidencia científica y en las principales guías nacionales e internacionales, hemos elaborado este documento que incluye las principales recomendaciones para el cuidado antenatal de la gestante en el contexto de la pandemia por SARS-CoV-2. En él se recoge cómo debe reestructurarse una Sección de Medicina Fetal ante esta nueva situación, qué medidas de seguridad deben seguirse para la realización de las exploraciones ecográficas y técnicas invasivas, y de qué modo debe procederse para la limpieza y desinfección de los equipos ecográficos. Estas recomendaciones deberán adaptarse a los diferentes medios teniendo en cuenta la infraestructura del centro y sus recursos

During a pandemic, the three basic principles are. to prioritize medical resources, ensure patients' lockdown in order to avoid community transmission and prevent healthcare collapse, and keep the number of visits to an absolute minimum to avoid patient exposure and safeguard healthcare workers. Antenatal care must be maintained during a health crisis, regardless of the COVID-19 state of alert. Routine and specialist obstetric ultrasound scans are essential for clinical decision-making during pregnancy, as it has a direct impact on the management of mothers and fetuses and on the perinatal outcome. In an attempt to minimize in-person visits, these will be organized according to the established ultrasound schedule. Based on scientific evidence, and on existing main national and international guidelines, this document has been prepared, in which proposals and options are provided for managing pregnant women in the context of the SARS-CoV-2 pandemic. It includes how a Fetal Medicine Unit facing this health crisis should be restructured, what safety measures should be followed in the performance of obstetric scans and invasive procedures, and how ultrasound rooms, equipment and transducers should be cleaned and disinfected. These recommendations should be adapted to different units based on their resources and infrastructure

[How to organize a Fetal Medicine Unit in the context of COVID-19 pandemic. Safe measures for obstetric scans and equipment cleaning]. / Cómo reestructurar una Sección de Medicina Fetal en tiempos de pandemia COVID-19. Medidas de seguridad y limpieza de los equipos ecográficos.

During a pandemic, the three basic principles are. to prioritize medical resources, ensure patients' lockdown in order to avoid community transmission and prevent healthcare collapse, and keep the number of visits to an absolute minimum to avoid patient exposure and safeguard healthcare workers. Antenatal care must be maintained during a health crisis, regardless of the COVID-19 state of alert. Routine and specialist obstetric ultrasound scans are essential for clinical decision-making during pregnancy, as it has a direct impact on the management of mothers and fetuses and on the perinatal outcome. In an attempt to minimize in-person visits, these will be organized according to the established ultrasound schedule. Based on scientific evidence, and on existing main national and international guidelines, this document has been prepared, in which proposals and options are provided for managing pregnant women in the context of the SARS-CoV-2 pandemic. It includes how a Fetal Medicine Unit facing this health crisis should be restructured, what safety measures should be followed in the performance of obstetric scans and invasive procedures, and how ultrasound rooms, equipment and transducers should be cleaned and disinfected. These recommendations should be adapted to different units based on their resources and infrastructure.

Cómo reestructurar una Sección de Medicina Fetal en tiempos de pandemia COVID-19. Medidas de seguridad y limpieza de los equipos ecográficos / How to organize a Fetal Medicine Unit in the context of COVID-19 pandemic. Safe measures for obstetric scans and equipment cleaning

En situación de pandemia, los tres principios básicos en la atención sanitaria son priorizar los recursos, mantener el confinamiento del paciente para evitar la transmisión comunitaria y el colapso sanitario, y reducir la asistencia no prioritaria con el fin de evitar la exposición del paciente y de salvaguardar la salud del profesional sanitario. El control antenatal debe mantenerse durante el periodo de crisis sanitaria, independientemente del estado de alerta COVID-19. La ecografía obstétrica es una prueba fundamental para la toma de decisiones clínicas durante el embarazo, con un impacto en el manejo del binomio madre-feto y en el resultado perinatal, por lo que se ha de garantizar su realización. Con el fin de reducir las visitas presenciales al mínimo número posible, estas se intentarán organizar teniendo en cuenta los controles ecográficos establecidos. Basados en la evidencia científica y en las principales guías nacionales e internacionales, hemos elaborado este documento que incluye las principales recomendaciones para el cuidado antenatal de la gestante en el contexto de la pandemia por SARS-CoV-2. En él se recoge cómo debe reestructurarse una Sección de Medicina Fetal ante esta nueva situación, qué medidas de seguridad deben seguirse para la realización de las exploraciones ecográficas y técnicas invasivas, y de qué modo debe procederse para la limpieza y desinfección de los equipos ecográficos. Estas recomendaciones deberán adaptarse a los diferentes medios teniendo en cuenta la infraestructura del centro y sus recursos

During a pandemic, the three basic principles are. to prioritize medical resources, ensure patients’ lockdown in order to avoid community transmission and prevent healthcare collapse, and keep the number of visits to an absolute minimum to avoid patient exposure and safeguard healthcare workers. Antenatal care must be maintained during a health crisis, regardless of the COVID-19 state of alert. Routine and specialist obstetric ultrasound scans are essential for clinical decision-making during pregnancy, as it has a direct impact on the management of mothers and fetuses and on the perinatal outcome. In an attempt to minimize in-person visits, these will be organized according to the established ultrasound schedule. Based on scientific evidence, and on existing main national and international guidelines, this document has been prepared, in which proposals and options are provided for managing pregnant women in the context of the SARS-CoV-2 pandemic. It includes how a Fetal Medicine Unit facing this health crisis should be restructured, what safety measures should be followed in the performance of obstetric scans and invasive procedures, and how ultrasound rooms, equipment and transducers should be cleaned and disinfected. These recommendations should be adapted to different units based on their resources and infrastructure

Effects of different cryopreservation methods on DNA integrity and sperm chromatin quality in men.

BACKGROUND: Cryopreserved human sperm are used in assisted reproductive technology. However, the effect of cryopreservation on sperm DNA integrity is unclear. OBJECTIVES: The objectives of this study were to: (i) determine the impact of semen cryopreservation on human sperm DNA integrity and chromatin structure; (ii) test if parameters obtained from TUNEL and SCSA® correlate; and (iii) verify correlation between sperm motility, morphology and viability with TUNEL and SCSA® parameters. MATERIALS AND METHODS: Men attending a fertility clinic were recruited and grouped according to their sperm parameters (n = 9/group): normozoospermia, oligoasthenoteratozoospermia and teratozoospermia. Each semen sample was processed as follow: (i) directly frozen at -80 °C; (ii) diluted in Sperm Maintenance Medium, cooled for 30 min at 4 °C and frozen at -80 °C; (iii) diluted in Sperm Maintenance Medium; or (iv) in SpermFreeze. Each mixture from method (iii) and (iv) was then suspended for 30 min in liquid nitrogen vapor and plunged into liquid nitrogen. After at least two months of storage, samples were thawed at room temperature and analyzed for motility and viability, TUNEL and SCSA® assays. RESULTS: Progressive motility and viability decreased after freeze-thawing. TUNEL scores increased significantly in all samples after freezing-thawing while no significant change in the DNA fragmentation index (DFI) from SCSA® was observed. No change in the percentage high DNA stainability (HDS) was observed in normozoospermic samples; however it was significantly increased in all the methods in oligoasthenoteratozoospermic and in the methods (ii)-(iv) in teratozoospermic samples. The DFI and TUNEL scores correlated significantly with each other and inversely with sperm motility, viability and morphology. DISCUSSION AND CONCLUSION: Cryopreservation seems to be deleterious for the integrity of human sperm DNA and compaction. However, the sperm DFI was not affected during cryopreservation under the various methods of storage tested. Clinicians and investigators should take this information into consideration when using cryopreserved sperm for assisted reproduction.

[Study of pulmonary hypertension and long-term respiratory clinic in children with congenital diaphragmatic hernia]. / Estudio de la hipertensión pulmonar y la clínica respiratoria a largo plazo en los niños con hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: The aim of this study is to identify respiratory clinic and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH) and whether these could be predicted by prenatal measures. MATERIAL AND METHODS: We studied fetal ultrasound: Observed/expected Lung to Head Ratio (O/E LHR) and classified patients according to their outcome (group 1: O/E LHR <25%, group 2: 26-35%, group 3: 36-45%, group 4: >55%) as well as the severity of PAH (group 0: non-PAH, group 1: mild, group 2: moderate, group 3: severe) in echocardiograms at birth, 1st, 6th, 12th and 24 months of life. We also evaluated gestational age, weight, bronchodilator treatment and number of hospital admissions. RESULTS: 58 patients with CDH, 13 without prenatal diagnosis. 36 patients out of 45 had O/E LHR calculated at 22.4 ± 5.8 weeks. O/E LHR had significant association with the severity of PAH at birth and in the 1st, 6th, 12th and 24th months (p <0.05). At 6 months, only 30.4% had PAH without any association with a higher risk of hospital admission [OR 1.07 (0.11-10.1)] and only three patients (5.1%) required bronchodilator treatment. CONCLUSION: In CDH, PAH and the respiratory clinic improve over time, being uncommon the need for treatment as of the 6th month. O/E LHR predicts the presence and severity of PAH in short and long term.

INTRODUCCION Y OBJETIVOS: Nuestro objetivo es estudiar la presencia en hernia diafragmática congénita (HDC) de clínica respiratoria e hipertensión pulmonar (HTP) a largo plazo y si estas pueden predecirse prenatalmente. MATERIAL Y METODOS: Estudiamos en ecografía fetal: Lung to Head Ratio observado/esperado (LHR O/E) y clasificamos a los pacientes según su resultado (grupo 1: LHR O/E <25%, grupo 2: 26-35%, grupo 3: 36-45%, grupo 4: >55%) así como la gravedad de HTP (grupo 0: no HTP, grupo 1: leve, grupo 2: moderada, grupo 3: grave) en los ecocardiogramas al nacimiento, 1º, 6º, 12º y 24º meses de vida. Estudiamos también edad gestacional, peso, tratamiento broncodilatador y número de ingresos hospitalarios. RESULTADOS: Se identificaron 58 pacientes con HDC, 13 de ellos sin diagnóstico prenatal. De los 45 restantes, 36 tenían calculado el LHR O/E registrado a las 22,4 ± 5,8 semanas. El LHR O/E se relacionó significativamente con la gravedad de la HTP al nacimiento y en los meses 1º, 6º, 12º y 24º (p <0,05). A los 6 meses únicamente el 30,4% presentaban HTP sin que ello asociara más riesgo de ingresos hospitalarios [OR 1,07 (0,11-10,1)] y siendo solo n = 3 (5,1%) los que precisaban algún tipo de tratamiento broncodilatador. CONCLUSION: En HDC, la HTP y la clínica respiratoria mejoran con el tiempo, siendo infrecuente la necesidad de tratamiento a partir del 6º mes. El LHR O/E predice la presencia y gravedad de HTP a corto y largo plazo.

Estudio de la hipertensión pulmonar y la clínica respiratoria a largo plazo en los niños con hernia diafragmática congénita / Study of pulmonary hypertension and long-term respiratory clinic in children with congenital diaphragmatic hernia

Introducción y objetivos. Nuestro objetivo es estudiar la presencia en hernia diafragmática congénita (HDC) de clínica respiratoria e hipertensión pulmonar (HTP) a largo plazo y si estas pueden predecirse prenatalmente. Material y métodos. Estudiamos en ecografía fetal: Lung to Head Ratio observado/esperado (LHR O/E) y clasificamos a los pacientes según su resultado (grupo 1: LHR O/E 55%) así como la gravedad de HTP (grupo 0: no HTP, grupo 1: leve, grupo 2: moderada, grupo 3: grave) en los ecocardiogramas al nacimiento, 1º, 6º, 12º y 24º meses de vida. Estudiamos también edad gestacional, peso, tratamiento broncodilatador y número de ingresos hospitalarios. Resultados. Se identificaron 58 pacientes con HDC, 13 de ellos sin diagnóstico prenatal. De los 45 restantes, 36 tenían calculado el LHR O/E registrado a las 22,4 ± 5,8 semanas. El LHR O/E se relacionó significativamente con la gravedad de la HTP al nacimiento y en los meses 1º, 6º, 12º y 24º (p < 0,05). A los 6 meses únicamente el 30,4% presentaban HTP sin que ello asociara más riesgo de ingresos hospitalarios [OR 1,07 (0,11-10,1)] y siendo solo n = 3 (5,1%) los que precisaban algún tipo de tratamiento broncodilatador. Conclusión. En HDC, la HTP y la clínica respiratoria mejoran con el tiempo, siendo infrecuente la necesidad de tratamiento a partir del 6º mes. El LHR O/E predice la presencia y gravedad de HTP a corto y largo plazo

Introduction and objectives. The aim of this study is to identify respiratory clinic and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH) and whether these could be predicted by prenatal measures. Material and methods. We studied fetal ultrasound: Observed/expected Lung to Head Ratio (O/E LHR) and classified patients according to their outcome (group 1: O/E LHR 55%) as well as the severity of PAH (group 0: non-PAH, group 1: mild, group 2: moderate, group 3: severe) in echocardiograms at birth, 1st, 6th, 12th and 24 months of life. We also evaluated gestational age, weight, bronchodilator treatment and number of hospital admissions. Results. 58 patients with CDH, 13 without prenatal diagnosis. 36 patients out of 45 had O/E LHR calculated at 22.4 ± 5.8 weeks. O/E LHR had significant association with the severity of PAH at birth and in the 1st, 6th, 12th and 24th months (p< 0.05). At 6 months, only 30.4% had PAH without any association with a higher risk of hospital admission [OR 1.07 (0.11-10.1)] and only three patients (5.1%) required bronchodilator treatment. Conclusion. In CDH, PAH and the respiratory clinic improve over time, being uncommon the need for treatment as of the 6th month. O/E LHR predicts the presence and severity of PAH in short and long term

[The role of fetal magnetic resonance imaging in the study of congenital diaphragmatic hernia]. / El papel de la resonancia magnética fetal en el estudio de la hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: Different echographic and fetal magnetic resonance (MRI) measurements have been described in the diagnosis of associated malformations and the prognosis of congenital diaphragmatic hernia (CDH). We have reviewed our experience searching for useful isolated or combined parameters and how MRI can complement ultrasound. MATERIAL AND METHODS: We evaluated 29 fetuses with CDH. We examined ultrasonography: Lung to Head (LHR o/e) and in MRI: ipsilateral lung volume (IPV) and total expressed as percentage of observed / expected lung volume (VPT o/e) and percentage of herniated liver (PHH). We studied: survival, ECMO and associated malformations. RESULTS: LHR o/e was the measure that best predicted survival (p< 0.05). VPT o/e did not predict survival or the need of ECMO (p> 0.05). PHH ≥19% was related to the need of ECMO. IPV < 2 cc required ECMO more frequently (p< 0.018) and when it was 0 cc in all cases. No combination of MR measurements was superior to LHR o/e in prediction of survival. MRI complemented the ultrasound in 4 cases: diaphragmatic eventration diagnosed with HDC, right HDC with fluid in the sac that suggested thoracic cyst, differentiation between spleen and lung that measured together overestimated the LHR and/or suspicion of Cornelia de Lange due to facial malformations. CONCLUSIONS: Not a single or combined MRI measurement exceeds LHR o/e in survival prediction. MRI is related to prognosis and can be used to support ultrasound in making decisions. MRI occasionally provides complementary morphological information.

INTRODUCCION Y OBJETIVOS: Distintas medidas ecográficas y de resonancia magnética fetal (RM) han sido descritas en el diagnóstico de malformaciones asociadas y el pronóstico de la hernia diafragmática congénita (HDC). Hemos revisado nuestra experiencia buscando parámetros aislados o combinados útiles y cómo la RM puede complementar a la ecografía. MATERIAL Y METODOS: Evaluamos 29 fetos con HDC. Revisamos en ecografía: Lung to Head Ratio observado/esperado (LHR o/e) y en RM: volumen pulmonar ipsilateral (VPI) y total expresado como porcentaje del volumen pulmonar observado/esperado (VPT o/e) y porcentaje de hígado herniado (PHH). Estudiamos: supervivencia, oxigenación con membrana extracorpórea (ECMO) y malformaciones asociadas. RESULTADOS: El LHR o/e fue la medida que mejor predijo supervivencia (p< 0,05). El VPT o/e no predijo supervivencia ni la necesidad de ECMO (p> 0,05). El PHH ≥19% se relacionó con necesidad de ECMO. El VPI menor de 2 cc requirió ECMO más frecuentemente (p< 0,018) y cuando fue de 0 cc en todos los casos. Ninguna combinación de medidas de RM fue superior al LHR o/e en predicción de supervivencia. La RM complementó a la ecografía en 4 casos: eventración diafragmática diagnosticada como HDC, HDC derecha con líquido en el saco que sugería quiste torácico, diferenciación entre bazo y pulmón que medidos juntos sobreestimaba el LHR o/e y sospecha de síndrome de Cornelia de Lange por malformaciones faciales. CONCLUSIONES: Ninguna medida aislada o combinada de RM supera al LHR o/e en la predicción de supervivencia. La RM se relaciona con el pronóstico y puede usarse como apoyo de la ecografía en la toma de decisiones. La RM aporta ocasionalmente información morfológica complementaria.

El papel de la resonancia magnética fetal en el estudio de la hernia diafragmática congénita / The role of fetal magnetic resonance imaging in the study of congenital diaphragmatic hernia

Introducción y objetivos. Distintas medidas ecográficas y de resonancia magnética fetal (RM) han sido descritas en el diagnóstico de malformaciones asociadas y el pronóstico de la hernia diafragmática congénita (HDC). Hemos revisado nuestra experiencia buscando parámetros aislados o combinados útiles y cómo la RM puede complementar a la ecografía. Material y métodos. Evaluamos 29 fetos con HDC. Revisamos en ecografía: Lung to Head Ratio observado/esperado (LHR o/e) y en RM: volumen pulmonar ipsilateral (VPI) y total expresado como porcentaje del volumen pulmonar observado/esperado (VPT o/e) y porcentaje de hígado herniado (PHH). Estudiamos: supervivencia, oxigenación con membrana extracorpórea (ECMO) y malformaciones asociadas. Resultados. El LHR o/e fue la medida que mejor predijo supervivencia (p 0,05). El PHH ≥19% se relacionó con necesidad de ECMO. El VPI menor de 2 cc requirió ECMO más frecuentemente (p <0,018) y cuando fue de 0 cc en todos los casos. Ninguna combinación de medidas de RM fue superior al LHR o/e en predicción de supervivencia. La RM complementó a la ecografía en 4 casos: eventración diafragmática diagnosticada como HDC, HDC derecha con líquido en el saco que sugería quiste torácico, diferenciación entre bazo y pulmón que medidos juntos sobreestimaba el LHR o/e y sospecha de síndrome de Cornelia de Lange por malformaciones faciales. Conclusiones. Ninguna medida aislada o combinada de RM supera al LHR o/e en la predicción de supervivencia. La RM se relaciona con el pronóstico y puede usarse como apoyo de la ecografía en la toma de decisiones. La RM aporta ocasionalmente información morfológica complementaria (AU)

Introduction and objectives. Different echographic and fetal magnetic resonance (MRI) measurements have been described in the diagnosis of associated malformations and the prognosis of congenital diaphragmatic hernia (CDH). We have reviewed our experience searching for useful isolated or combined parameters and how MRI can complement ultrasound. Material and methods. We evaluated 29 fetuses with CDH. We examined ultrasonography: Lung to Head (LHR o/e) and in MRI: ipsilateral lung volume (IPV) and total expressed as percentage of observed / expected lung volume (VPT o/e) and percentage of herniated liver (PHH). We studied: survival, ECMO and associated malformations. Results. LHR o/e was the measure that best predicted survival (p 0.05). PHH ≥19% was related to the need of ECMO. IPV < 2 cc required ECMO more frequently (p < 0.018) and when it was 0 cc in all cases. No combination of MR measurements was superior to LHR o/e in prediction of survival. MRI complemented the ultrasound in 4 cases: diaphragmatic eventration diagnosed with HDC, right HDC with fluid in the sac that suggested thoracic cyst, differentiation between spleen and lung that measured together overestimated the LHR and/or suspicion of Cornelia de Lange due to facial malformations. Conclusions. Not a single or combined MRI measurement exceeds LHR o/e in survival prediction. MRI is related to prognosis and can be used to support ultrasound in making decisions. MRI occasionally provides complementary morphological information (AU)

Prognostic factors and outcomes for pediatric patients receiving an haploidentical relative allogeneic transplant using CD3/CD19-depleted grafts.

Haploidentical hematopoietic stem cell transplantation using T-cell-depleted grafts is a valid option for pediatric patients with hematological malignancies in need of an allogeneic transplantation and lacking an HLA-identical donor. Seventy-five transplantations were performed in 70 patients. Thirty-eight patients had ALL, 32 had AML, 3 had advanced myelodysplastic syndromes and 2 juvenile myelomonocytic leukemia; 19 were in first CR, 30 in second CR, 12 in greater than second CR and 14 were considered to be in refractory disease at time of transplantation. Four patients developed graft failure. Among engrafted patients, the median time to neutrophil and platelet recovery was 13 (range 8-20) and 10 days (range 8-70), respectively. In 64 (85%) cases, ⩾1 infections were diagnosed after transplant. The probability of nonrelapse mortality by day +100 after transplantation was 10±4%. With a median follow-up of 22 months, the probability of relapse was 32±6% and disease-free survival was 52±6%. Haploidentical transplantation using CD3/CD19 depletion is associated with encouraging results especially in patients in early phase of disease. Killer-cell Ig-like receptor B haplotype donors confer a rapid natural killer cells expansion early after transplantation, resulting in lower probability of relapse and suggesting a GvL effect apart from graft-versus-host reactions. Donor infusion of high numbers of CD34+ cells is recommended in order to improve T-cell reconstitution.

Dichorionic twin pregnancy discordant for anencephaly: two cases with different management.

BACKGROUND: Prevalence ofanencephaly in dichorionic twins is higher than in singleton pregnancies. The authors report two cases with two different management strategies. CASE 1: Spontaneous dichorionic diamniotic twin pregnancy with the second twin diagnosed with anencephaly at 12 weeks gestation. Selective feticide was performed at the age of 13.2 weeks. Vaginal delivery occurred at 39 weeks, and birth weight was 2,850 g. CASE 2: Dichorionic diamniotic twin pregnancy discordant for anencephaly in the second twin was diagnosed at 13 weeks gestation. An expectant management was decided. Preterm delivery occurred at 35 weeks due to hydramnios of the affected fetus, delivering a healthy newborn weighing 2,300 g and an anencephalic neonate who died immediately after delivery. CONCLUSION: Anencephaly should be diagnosed as soon as possible, idealistically at 11-13+6 weeks ultrasound (US) scan, in order to offer the most appropriate counselling to the parents, ranging from selective feticide or expectant management. This short series suggests that selective early feticide may increase gestational age and birth weight.

Transformación de síndrome mielodisplásico a leucemia linfoblástica aguda: 2 casos nuevos / Transformation of myelodysplastic syndrome to acute lymphoblastic leukemia: 2 new cases

Los síndromes mielodisplásicos (SMD) son desórdenes clonales de las células hematopoyéticas, con riesgo variable de transformación a leucemia mieloblástica aguda. La evolución a leucemia linfoblástica aguda (LLA) es extremadamente rara, con muy pocos casos pediátricos. Este artículo describe 2 nuevos casos de SMD que progresaron a LLA, junto a una revisión de la literatura, observando un pronóstico similar a los adultos (AU)

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells, with a variable risk of transformation to acute myeloid leukemia. Progression into acute lymphoblastic leukemia (ALL) is an extremely rare event, with very few cases published in children. In this report, we describe two cases of myelodysplastic syndromes that progressed to ALL. Moreover, we review previously reported cases of MDS transformation to acute lymphoblastic leukemia in the pediatric population whose prognosis seems to be similar to that for adults (AU)

[Transformation of myelodysplastic syndrome to acute lymphoblastic leukemia: 2 new cases]. / Transformación de síndrome mielodisplásico a leucemia linfoblástica aguda: 2 casos nuevos.

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells, with a variable risk of transformation to acute myeloid leukemia. Progression into acute lymphoblastic leukemia (ALL) is an extremely rare event, with very few cases published in children. In this report, we describe two cases of myelodysplastic syndromes that progressed to ALL. Moreover, we review previously reported cases of MDS transformation to acute lymphoblastic leukemia in the pediatric population whose prognosis seems to be similar to that for adults.

Varicella zoster central nervous system vasculitis after allogeneic hematopoietic stem cell transplant successfully treated with cyclophosphamide.

We report the first case of varicella zoster central nervous system vasculitis, to our knowledge, which responded to intravenous pulses of cyclophosphamide in an immunocompromised child with severe and progressive disease, without sequelae.

[Ophthalmological manifestations in Fabry's disease. Four clinical cases showing deficient alpha-galactosidase-A activity]. / Manifestaciones oftalmológicas en la enfermedad de Fabry. A propósito de 4 casos con actividad deficiente de a-galactosidasa A.

CASE REPORT: Fabry's disease is an illness produced by an alteration in the catabolism of the glycosphingolipids. We report ophthalmologic findings in 4 people, detected after 113 patient evaluations from an analytical, cardiological and genetic point of view. DISCUSSION: Fabry's disease is uncommon and shows variable ophthalmologic affectation. Some patients with Fabry's disease do not present ocular affectation, while, on the other hand, healthy carriers with important ocular alterations have been described. The deposit of glycosphingolipids produces affectation at the corneal, crystalline, vascular and retinal levels. The vascular alterations affect not only the veins but also the arteries, as we report in our patients.

Manifestaciones oftalmológicas en la enfermedad de Fabry. A propósito de 4 casos con actividad deficiente de alfa-galactosidasa A / Ophthalmological manifestations in Fabry´s disease. Four clinical cases showing deficient alpha-galactosidase-A activity

Caso clínico: La enfermedad de Fabry es una enfermedad producida por una alteración en el catabolismo de los glucoesfingolípidos. Se muestran las alteraciones oftalmológicas de cuatro pacientes detectados tras evaluar desde el punto de vista analítico, cardiológico y genético a 113 enfermos. Discusión: La enfermedad de Fabry es una enfermedad infrecuente con afectación oftalmológica inconstante existiendo enfermos con Fabry sin afectación ocular y portadores sanos, con importantes alteraciones oculares. El depósito de glucoesfingolípidos produce afectación a nivel corneal, cristaliniano, vascular y retiniano. Las alteraciones vasculares afectan no sólo a las venas sino también a las arterias como mostramos en nuestros pacientes

Case report: Fabrys disease is an illness produced by an alteration in the catabolism of the glycosphingolipids. We report ophthalmologic findings in 4 people, detected after 113 patient evaluations from an analytical, cardiological and genetic point of view. Discussion: Fabry’s disease is uncommon and shows variable ophthalmologic affectation. Some patients with Fabry’s disease do not present ocular affectation, while, on the other hand, healthy carriers with important ocular alterations have been described. The deposit of glycosphingolipids produces affectation at the corneal, crystalline, vascular and retinal levels. The vascular alterations affect not only the veins but also the arteries, as we report in our patients (Arch Soc Esp Oftalmol 2008; 83: 713-718)

Buscar bibliografía. PUBMED (2) / Search bibliography. PUBMED (2)

En el número anterior aprendimos a manejar Pubmed. Ahora vamos a limitar mejor nuestra búsqueda, usando la base de datos de palabras clave y las preguntas clínicas; también aprenderemos cómo buscar artículos en unas determinadas revistas, así como los recursos de Pubmed para conseguir el artículo completo online (AU)

La salud de las mujeres en la prensa / Women’s health in press

La salud como tema noticiable en los medios de comunicación puede ser tratada de diferentes formas y perspectivas, lo que influye de manera notable en los conocimientos que la sociedad adquiere al respecto. En este trabajo se realizan algunas consideraciones sobre cómo se abordan las noticias de salud en la prensa, poniendo énfasis en los análisis realizados al respecto desde las publicaciones científicas (AU)