Effects of cardiopulmonary bypass on the development of lymphopenia and sepsis after cardiac surgery in children with congenital cardiopathy.

The objective of the present study was to investigate whether lymphopenia occurring after heart surgery with cardiopulmonary bypass (CPB) is related to apoptosis and or sepsis in children. The design was a prospective cohort study in a third level care hospital in Mexico City. In total, 68 children (31 girls and 37 boys) with congenital cardiopathy who needed corrective cardiac surgery with or without CPB were included. The samples were obtained from central blood before, immediately after and 24 h after surgery. Complete blood counts and lymphocyte apoptosis were analyzed. Systemic inflammatory response syndrome (SIRS), sepsis and the type of microorganism were recorded. A total of 53 patients received CPB and 15 did not. Lymphocyte count decreased after surgery in both groups (P<0.001). However, neutrophil count increased markedly in both groups. Apoptosis of B (CD19+) lymphocytes was higher in the non-CPB group (14, 2 and 21% before, immediately after and 24 h after surgery, respectively) than the CPB group (0, 2 and 3%, respectively), but apoptosis of cytotoxic T lymphocytes (CD8+) was higher in the CPB group (5, 4 and 3% before, immediately after and 24 h after surgery, respectively) than in the non-CPB group (2, 3 and 2%, respectively). However, the extent of apoptosis of T and B lymphocytes after surgery did not differ between groups. The CPB group had more complications than the non-CPB group [38 (71.7%) vs. 9 (60.0%)]. In conclusion, the decrease in lymphocyte count may be related to apoptosis of cytotoxic T lymphocytes in children receiving cardiac surgery with CPB and to apoptosis of B lymphocytes in those not receiving CPB. The decreased lymphocyte counts in both groups suggested that CPB is not the main cause of this decrease. Children who received CPB during surgery had more complications, such as sepsis and cardiogenic shock than did those who did not receive CPB.

[Epidemiology of Imperfect Osteogenesis: a Rare Disease in the Valencia Region.] / Epidemiología de la Osteogénesis Imperfecta: una enfermedad rara en la Comnitat Valenciana.

OBJECTIVE: Osteogenesis imperfecta (OI) is a rare connective tissue and bone disease that results in a bone fragility of varying severity. The objective was to determine and describe the OI in the Valencia Region (VR) during the period 2004 to 2014. METHODS: From the Rare Diseases Information System of the VR (SIER-CV) patients from 2004 to 2014 with the codes of the International Classification of Diseases for the OI were identified: 756.51 from the 9th Revision-Clinical Modification and Q78.0 from the 10th Revision. The information was validated by reviewing clinical documentation (mainly electronic health records) and a descriptive analysis of the confirmed cases (diagnosis of OI in the clinical documentation) was performed. RESULTS: 162 patients were identified with a code for OI. 145 of the 161 patients with available clinical documentation were confirmed as cases. The prevalence was 0.29 per 10.000 inhabitants. 93.1% were Spanish, 54.5% were women and they were treated in 25 different hospitals in the VR. The type of OI was known in the 26.4% of the cases and type I was the most common (9.7%). 6.2% of the patients died with an average death age of 60.8 years. 44.8% of patients received treatment with bisphosphonates and 10.4% had affected relatives. CONCLUSIONS: The real situation of the OI in the VR has been established, which will allow a better planning in the health actions to improve the quality of life of the affected ones and their families.

OBJETIVO: La Osteogénesis Imperfecta (OI) es una enfermedad rara del tejido conectivo y óseo que resulta en una fragilidad ósea de diversa severidad. El objetivo fue determinar y describir la situación de la OI en la Comunitat Valenciana (CV) durante el periodo 2004-2014. METODOS: A partir del Sistema de Información de Enfermedades Raras de la CV (SIER-CV) se identificaron los pacientes con los códigos de la Clasificación Internacional de Enfermedades pertenecientes a la OI: el 756.51 de la 9ª y/o el Q78.0 de la 10ª revisión, durante el período 2004-2014. Se validaron los datos mediante la revisión de documentación clínica (historia clínica electrónica principalmente) y de los casos confirmados (diagnóstico de OI presente en la documentación clínica) se elaboró un análisis descriptivo. RESULTADOS: Se identificaron 162 pacientes con código de OI. Se confirmaron 145 casos de los 161 con la documentación clínica accesible. La prevalencia fue de 0,29 por 10.000 habitantes. El 93,1% eran españoles, el 54,5% mujeres y fueron atendidos en 25 hospitales diferentes de la CV. Se identificó el tipo de OI en el 24,8% de los casos, siendo el tipo I el más habitual (9,7%). Fallecieron el 6,2% con una edad media al fallecimiento de 60,8 años. El 44,8% de pacientes recibió tratamiento con bifosfonatos y el 10,4% tenían familiares afectados. CONCLUSIONES: Se ha establecido la situación real de la OI en la CV, lo que permitirá una mejor planificación en las acciones sanitarias para mejorar la calidad de vida de los afectados y sus familiares.

Epidemiología de la Osteogénesis Imperfecta: una enfermedad rara en la Comunitat Valenciana / Epidemiology of Imperfect Osteogenesis: a rare disease in the Valencia Region

Fundamentos: La Osteogénesis Imperfecta (OI) es una enfermedad rara del tejido conectivo y óseo que resulta en una fragilidad ósea de diversa severidad. El objetivo fue determinar y describir la situación de la OI en la Comunitat Valenciana (CV) durante el periodo 2004-2014. Métodos: A partir del Sistema de Información de Enfermedades Raras de la CV (SIER-CV) se identificaron los pacientes con los códigos de la Clasificación Internacional de Enfermedades pertenecientes a la OI: el 756.51 de la 9ª y/o el Q78.0 de la 10ª revisión, durante el período 2004-2014. Se validaron los datos mediante la revisión de documentación clínica (historia clínica electrónica principalmente) y de los casos confirmados (diagnóstico de OI presente en la documentación clínica) se elaboró un análisis descriptivo. Resultados: Se identificaron 162 pacientes con código de OI. Se confirmaron 145 casos de los 161 con la documentación clínica accesible. La prevalencia fue de 0,29 por 10.000 habitantes. El 93,1% eran españoles, el 54,5% mujeres y fueron atendidos en 25 hospitales diferentes de la CV. Se identificó el tipo de OI en el 24,8% de los casos, siendo el tipo I el más habitual (9,7%). Fallecieron el 6,2% con una edad media al fallecimiento de 60,8 años. El 44,8% de pacientes recibió tratamiento con bifosfonatos y el 10,4% tenían familiares afectados. Conclusión: Se ha establecido la situación real de la OI en la CV, lo que permitirá una mejor planificación en las acciones sanitarias para mejorar la calidad de vida de los afectados y sus familiares (AU)

Background: Osteogenesis imperfecta (OI) is a rare connective tissue and bone disease that results in a bone fragility of varying severity. The objective was to determine and describe the OI in the Valencia Region (VR) during the period 2004 to 2014. Methods: From the Rare Diseases Information System of the VR (SIER-CV) patients from 2004 to 2014 with the codes of the International Classification of Diseases for the OI were identified: 756.51 from the 9th Revision-Clinical Modification and Q78.0 from the 10th Revision. The information was validated by reviewing clinical documentation (mainly electronic health records) and a descriptive analysis of the confirmed cases (diagnosis of OI in the clinical documentation) was performed. Results: 162 patients were identified with a code for OI. 145 of the 161 patients with available clinical documentation were confirmed as cases. The prevalence was 0.29 per 10.000 inhabitants. 93.1% were Spanish, 54.5% were women and they were treated in 25 different hospitals in the VR. The type of OI was known in the 26.4% of the cases and type I was the most common (9.7%). 6.2% of the patients died with an average death age of 60.8 years. 44.8% of patients received treatment with bisphosphonates and 10.4% had affected relatives. Conclusion: The real situation of the OI in the VR has been established, which will allow a better planning in the health actions to improve the quality of life of the affected ones and their families (AU)

[Congenital coronary fistula. Clinical presentation in 7 cases and literature review]. / Fistula coronaria congénita. Presentación de siete casos y revisión de la literatura.

UNLABELLED: Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.

Fistula coronaria congénita: presentación de siete casos y revisión de la literatura / Congenital coronary fistula: clinical presentation in 7 cases and literature review

Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.

Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular: Primer caso reportado / Scimitar syndrome associated to pulmonary atresia with interventricular communication: First reported case

Se presenta el caso de un masculino de 15 meses de edad con síndrome de cimitarra y atresia pulmonar con comunicación interventricular. El diagnóstico se hizo mediante cateterismo cardíaco y angiocardiografía y confirmado por el estudio de autopsia. Se hacen las consideraciones clínicas y quirúrgicas de esta excepcional asociación que a la fecha es, hasta donde sabemos la primera reportada en la literatura.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

[Scimitar syndrome associated to pulmonary atresia with interventricular communication. First reported case]. / Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular. Primer caso reportado.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

Tratamiento con estreptocinasa intrapleural del hemotórax coagulado posterior a cirugía cardíaca con derivación cardiopulmonar / Treatment with Intrapleural Streptokinase for Coagulated Hemothorax after Cardiac Surgery with Cardiopulmonary Bypass

Introducción y objetivos. El hemotórax coagulado es una complicación postoperatoria en la cirugía cardíaca con derivación cardiopulmonar. El objetivo del trabajo es presentar la experiencia en el tratamiento de esta complicación con la administración intrapleural de estreptocinasa. Métodos. De enero de 1996 a junio de 1999, 9 pacientes (6 varones y 3 mujeres), con rango de edad de uno a 75 años, presentaron hemotórax coagulado posterior a cirugía cardíaca con derivación cardiopulmonar, diagnosticado por clínica y radiografías del tórax. Todos fueron tratados mediante la administración intrapleural de estreptocinasa con una dosis fija de 250.000 U en los casos adultos y 12.000 U/kg en pacientes pediátricos, movilización con drenaje torácico pinzado y posterior apertura del mismo. Resultados. Se obtuvo drenaje que varió entre 100 y 1.200 ml de acuerdo con la edad del paciente y se observó mejoría clínica y radiológica en todos los casos. No hubo modificaciones en las pruebas de coagulación. Un paciente falleció por fallo multiorgánico a causa de la enfermedad de fondo, no relacionada con el procedimiento. El resto de los pacientes evolucionaron de manera satisfactoria y en el momento actual se encuentran clínicamente sin restricción ventilatoria. Conclusión. El tratamiento del hemotórax coagulado con estreptocinasa es una alternativa segura y evita el drenaje quirúrgico convencional (AU)