P Esparza Paz, A Sarasua Miranda, N García de Andoin Barandiaran, A Nogués Pérez
{"title":"[Renal angiomyolipoma: a case with a difficult diagnosis].","authors":"P Esparza Paz, A Sarasua Miranda, N García de Andoin Barandiaran, A Nogués Pérez","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"495-7"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Exogenous surfactant. A rescue treatment in a neonate with varicella complicated with pneumonia].","authors":"A Clington, L Pereira-da-Silva, M Serelha","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"493-4"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J Villalba Nogales, I Herráiz Sarachaga, R Bermúdez-Cañete Fernández, M J Maitre Azcárate, J Mora de Oñate, A González Rocafort, M Quero Jiménez
Objectives: To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study.
Patients and methods: We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 39.7 months).
Results: The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p < 0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p < 0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p < 0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p < 0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p < 0.0001. In 23 % of the patients, aortic insufficiency significantly increased. Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7 % and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.
目的:评价新生儿球囊主动脉瓣成形术的即时效果和长期超声心动图结果,并确定预测结果的变量。确定血流动力学和超声心动图研究的一致程度。患者和方法:我们分析了26例手术的结果,18例男孩和8例女孩(225:1),年龄2至49天(16.1 - 12.9天)。所有手术均于1989年6月至2001年6月在马德里Ramón y Cajal医院儿科心脏病科进行。随访0 ~ 144个月(39.5 ~ 39.7个月)。结果:超声心动图观察到的直接效果是最大多普勒梯度显著降低(从77.8降至32.4 mm Hg;p < 0.0001)和中等多普勒梯度(从41.7到18.5 mm Hg;P < 0.05)。血流动力学研究显示左心室收缩压显著降低(从119.8 mm Hg降至82.8 mm Hg;p < 0.0001)和主动脉收缩压升高(从56.8到66.6 mm Hg;P < 0.007)。血流动力学峰梯度由63.1减小到17.7 mm Hg;P < 0.0001。23%的患者主动脉功能不全明显加重。超声心动图随访显示左心室舒张距离增大,最大和中多普勒梯度减小。该手术的初步成功率为68.7%,45个月时无瓣膜手术的存活率为65.8%。比例风险分析显示,瓣膜成形术后的梯度是未来瓣膜手术的预测因素。
{"title":"[Balloon valvoplasty for critical aortic valve stenosis in neonates].","authors":"J Villalba Nogales, I Herráiz Sarachaga, R Bermúdez-Cañete Fernández, M J Maitre Azcárate, J Mora de Oñate, A González Rocafort, M Quero Jiménez","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study.</p><p><strong>Patients and methods: </strong>We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 39.7 months).</p><p><strong>Results: </strong>The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p < 0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p < 0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p < 0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p < 0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p < 0.0001. In 23 % of the patients, aortic insufficiency significantly increased. Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7 % and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.</p>","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"444-51"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A Granados Molina, E García Menor, S Jaraba Caballero, I Ibarra de la Rosa, E Ulloa Santamaría, J L Pérez Navero, J M feminine Arizón de Prado, C Merino Cejas
Ventricular assist devices have demonstrated their utility in patients with intractable cardiac failure, both as support until complete myocardial recovery and as a bridge to transplantation. Specific pediatric pneumatic paracorporeal systems can be applied even in infants. Long-term survival has been reported although experience is limited. We report the case of an 8-year-old boy with dilated cardiomyopathy awaiting cardiac transplantation. The patient developed profound cardiogenic shock with multiorgan failure while being evaluated for heart transplantation. He was given biventricular assistance with the MEDOS-HIA system (MEDOS-Helmholtz Institute). Maximum stroke volume ventricles of 25 and 22 ml were used, achieving a cardiac output of 2.2 l/min in both ventricles. The patient was supported with ventricular assistance for 9 days, but multiple organ failed to improve and transplantation became impossible. Progressive loss of peripheral circulatory resistance unresponsive to treatment developed and ventricular assistance was discontinued. The previous severe shock and advanced and progressive multiorgan failure could be responsible for the poor outcome of our patient despite maintenance of adequate cardiac output. Nevertheless, the use of ventricular assist devices is a real therapeutic alternative in children with severe cardiogenic shock, allowing them to recover completely or undergo heart transplantation. Patient selection, the choice of a system of appropriate size, and early implantation seem to be the cornerstones for obtaining good results.
{"title":"[External ventricular assist devices as circulatory support in patients with dilated cardiomyopathy].","authors":"A Granados Molina, E García Menor, S Jaraba Caballero, I Ibarra de la Rosa, E Ulloa Santamaría, J L Pérez Navero, J M feminine Arizón de Prado, C Merino Cejas","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Ventricular assist devices have demonstrated their utility in patients with intractable cardiac failure, both as support until complete myocardial recovery and as a bridge to transplantation. Specific pediatric pneumatic paracorporeal systems can be applied even in infants. Long-term survival has been reported although experience is limited. We report the case of an 8-year-old boy with dilated cardiomyopathy awaiting cardiac transplantation. The patient developed profound cardiogenic shock with multiorgan failure while being evaluated for heart transplantation. He was given biventricular assistance with the MEDOS-HIA system (MEDOS-Helmholtz Institute). Maximum stroke volume ventricles of 25 and 22 ml were used, achieving a cardiac output of 2.2 l/min in both ventricles. The patient was supported with ventricular assistance for 9 days, but multiple organ failed to improve and transplantation became impossible. Progressive loss of peripheral circulatory resistance unresponsive to treatment developed and ventricular assistance was discontinued. The previous severe shock and advanced and progressive multiorgan failure could be responsible for the poor outcome of our patient despite maintenance of adequate cardiac output. Nevertheless, the use of ventricular assist devices is a real therapeutic alternative in children with severe cardiogenic shock, allowing them to recover completely or undergo heart transplantation. Patient selection, the choice of a system of appropriate size, and early implantation seem to be the cornerstones for obtaining good results.</p>","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"480-3"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C García Sanz, M Rupérez Lucas, J López-Herce Cid, D Vigil Escribano, G Garrido Cantarero
Objective: To analyze and compare the prognostic value of the pediatric index of mortality (PIM) score and lactate values on admission to a pediatric intensive care unit (PICU).
Patients and methods: We performed a prospective study of 500 consecutive children: 237 girls (47.4 %) and 263 boys (52.6 %) with a mean age of 51.5 59.7 (range: 3 days-18 years) admitted to our PICU. PIM scores and blood lactate concentrations were determined on admission. The predictive ability of PIM and lactate concentrations in relation to mortality and length of stay in the PICU were analyzed.
Results: Thirty-six patients (7.2 %) died. According to the PIM score, the mean probability of death in children who died was 23.6 % 28.9, which was significantly higher than that in surviving children (3.4 % 7.3; p < 0.001). The area under the ROC curve for PIM was 0.81 0.03 (95 % CI: 0.74-0.89). Lactate level in nonsurvivors was 4.9 % 3.5 mmol/L, which was significantly higher than that in survivors (1.9 % 1.5 mmol/L; p < 0.001). The area under the ROC curve for blood lactate was 0.76 0.04 (95 % CI: 0.67-0.85). No statistically significant differences were found between either ROC curves. In survivors, a significant relationship was found between PIM score and length of stay in the PICU while in nonsurvivors an inverse relationship was found between blood lactate concentrations and length of stay.
Conclusions: Both PIM score and blood lactate concentrations on admission to the PICU have a moderate prognostic value in critically-ill children. The prognostic value of the PIM score is greater than that of blood lactate concentration but is more difficult to obtain, whereas blood lactate determination is fast and easy.
{"title":"[Prognostic value of the pediatric index of mortality (PIM) score and lactate values in critically-ill children].","authors":"C García Sanz, M Rupérez Lucas, J López-Herce Cid, D Vigil Escribano, G Garrido Cantarero","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objective: </strong>To analyze and compare the prognostic value of the pediatric index of mortality (PIM) score and lactate values on admission to a pediatric intensive care unit (PICU).</p><p><strong>Patients and methods: </strong>We performed a prospective study of 500 consecutive children: 237 girls (47.4 %) and 263 boys (52.6 %) with a mean age of 51.5 59.7 (range: 3 days-18 years) admitted to our PICU. PIM scores and blood lactate concentrations were determined on admission. The predictive ability of PIM and lactate concentrations in relation to mortality and length of stay in the PICU were analyzed.</p><p><strong>Results: </strong>Thirty-six patients (7.2 %) died. According to the PIM score, the mean probability of death in children who died was 23.6 % 28.9, which was significantly higher than that in surviving children (3.4 % 7.3; p < 0.001). The area under the ROC curve for PIM was 0.81 0.03 (95 % CI: 0.74-0.89). Lactate level in nonsurvivors was 4.9 % 3.5 mmol/L, which was significantly higher than that in survivors (1.9 % 1.5 mmol/L; p < 0.001). The area under the ROC curve for blood lactate was 0.76 0.04 (95 % CI: 0.67-0.85). No statistically significant differences were found between either ROC curves. In survivors, a significant relationship was found between PIM score and length of stay in the PICU while in nonsurvivors an inverse relationship was found between blood lactate concentrations and length of stay.</p><p><strong>Conclusions: </strong>Both PIM score and blood lactate concentrations on admission to the PICU have a moderate prognostic value in critically-ill children. The prognostic value of the PIM score is greater than that of blood lactate concentration but is more difficult to obtain, whereas blood lactate determination is fast and easy.</p>","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"394-400"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22143629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Congenital hyperferritinemia and cataract syndrome].","authors":"J A García-Erce","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"497-8; author reply 498-500"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J Martínez Baylach, N Pardo García, M Torrent Español, E Moliner Calderón, I Anquela Sanz, J Cubells Rieró
Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowly-progressive malignant disease. We report five cases of LCH with the same histopathologic basis but different outcome.
{"title":"[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base].","authors":"J Martínez Baylach, N Pardo García, M Torrent Español, E Moliner Calderón, I Anquela Sanz, J Cubells Rieró","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowly-progressive malignant disease. We report five cases of LCH with the same histopathologic basis but different outcome.</p>","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"484-7"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M Domínguez Salgado, R Santiago Gómez, J Campos Castelló, M J Fernández de Péres Villalaín
Headaches are common during childhood and become more frequent in adolescence. The rational, cost-effective evaluation of children with headache begins with a careful history. The first step is to identify the temporal pattern of the headache -acute, acute-recurrent, chronic-progressive, chronic-nonprogressive, or mixed. The next step is a physical and neurologic examination. Neuroimaging is not routinely warranted in the evaluation of childhood headache and should be reserved for use in children with acute or chronic-progressive patters of abnormalities in neurologic examination. Pediatric migraine differs from adult migraine. Recent studies indicate the need to revise diagnostic criteria for pediatric migraine, which would allow its real prevalence in this age group to be determined. The sensitivity and specificity of the International Headache Society (IHS) criteria for childhood migraine would be increased if the minimum duration of migraine were reduced and if a diagnosis of migraine were allowed when severe headache is associated with nausea, even though the criteria of location, quality, and aggravation by physical activity are not fulfilled. There are no differences in the fulfillment of the IHS criteria for migraine and tension-type headache between children and adolescents. Independent of age, the intensity of headache and the presence or absence of nausea are the most important features for differentiating the two major types of idiopathic headache.Migraines, migraine variants, tension headache and other types of headache often present for the first time during childhood and require close follow-up by the pediatrician. Investigation into this disorder is still developing.
{"title":"[Childhood headache. A diagnostic approach].","authors":"M Domínguez Salgado, R Santiago Gómez, J Campos Castelló, M J Fernández de Péres Villalaín","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Headaches are common during childhood and become more frequent in adolescence. The rational, cost-effective evaluation of children with headache begins with a careful history. The first step is to identify the temporal pattern of the headache -acute, acute-recurrent, chronic-progressive, chronic-nonprogressive, or mixed. The next step is a physical and neurologic examination. Neuroimaging is not routinely warranted in the evaluation of childhood headache and should be reserved for use in children with acute or chronic-progressive patters of abnormalities in neurologic examination. Pediatric migraine differs from adult migraine. Recent studies indicate the need to revise diagnostic criteria for pediatric migraine, which would allow its real prevalence in this age group to be determined. The sensitivity and specificity of the International Headache Society (IHS) criteria for childhood migraine would be increased if the minimum duration of migraine were reduced and if a diagnosis of migraine were allowed when severe headache is associated with nausea, even though the criteria of location, quality, and aggravation by physical activity are not fulfilled. There are no differences in the fulfillment of the IHS criteria for migraine and tension-type headache between children and adolescents. Independent of age, the intensity of headache and the presence or absence of nausea are the most important features for differentiating the two major types of idiopathic headache.Migraines, migraine variants, tension headache and other types of headache often present for the first time during childhood and require close follow-up by the pediatrician. Investigation into this disorder is still developing.</p>","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"432-43"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J Mallo Castaño, L M Rodríguez Fernández, C de la Fuente Domínguez
{"title":"[Antenatal diagnosis and isotopic studies in urethral ectopia].","authors":"J Mallo Castaño, L M Rodríguez Fernández, C de la Fuente Domínguez","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":"57 5","pages":"490-1; author reply 491-2"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141793","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}