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[Renal angiomyolipoma: a case with a difficult diagnosis]. 【肾血管平滑肌脂肪瘤1例难诊断】。
Pub Date : 2002-11-01
P Esparza Paz, A Sarasua Miranda, N García de Andoin Barandiaran, A Nogués Pérez
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引用次数: 0
[Exogenous surfactant. A rescue treatment in a neonate with varicella complicated with pneumonia]. (外源性表面活性剂。新生儿水痘合并肺炎抢救治疗[j]。
Pub Date : 2002-11-01
A Clington, L Pereira-da-Silva, M Serelha
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引用次数: 0
[Balloon valvoplasty for critical aortic valve stenosis in neonates]. [新生儿主动脉瓣狭窄的球囊成形术]。
Pub Date : 2002-11-01
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。
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引用次数: 0
[External ventricular assist devices as circulatory support in patients with dilated cardiomyopathy]. [扩张型心肌病患者的外心室辅助装置循环支持]。
Pub Date : 2002-11-01
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.

心室辅助装置在顽固性心力衰竭患者中已经证明了它们的实用性,既可以作为心肌完全恢复的支持,也可以作为移植的桥梁。特定的儿科气动副躯体系统甚至可以应用于婴儿。虽然经验有限,但有长期存活的报道。我们报告一例8岁男孩扩张型心肌病等待心脏移植。该患者在接受心脏移植评估时发生了严重的心源性休克并伴有多器官衰竭。采用MEDOS-HIA系统(MEDOS-Helmholtz研究所)给予双心室辅助。使用最大卒中容积为25和22 ml的心室,两个心室的心输出量为2.2 l/min。患者接受了9天的心室辅助治疗,但多器官未能改善,无法进行移植。进行性外周循环阻力丧失,对治疗无反应,停止心室辅助。先前的严重休克和晚期和进行性多器官功能衰竭可能是导致患者预后不良的原因,尽管维持了足够的心输出量。然而,对于患有严重心源性休克的儿童,使用心室辅助装置是一种真正的治疗选择,可以使他们完全恢复或接受心脏移植。患者选择,选择合适大小的系统,早期植入似乎是获得良好效果的基石。
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引用次数: 0
[Prognostic value of the pediatric index of mortality (PIM) score and lactate values in critically-ill children]. [小儿死亡指数(PIM)评分和乳酸值对危重儿童的预后价值]。
Pub Date : 2002-11-01
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.

目的:分析比较儿科重症监护病房(PICU)入院时儿童死亡指数(PIM)评分与乳酸值对预后的预测价值。患者和方法:我们对500名连续入住PICU的儿童进行了前瞻性研究:237名女孩(47.4%)和263名男孩(52.6%),平均年龄为51.5 - 59.7岁(范围:3天-18岁)。入院时测定PIM评分和血乳酸浓度。分析PIM和乳酸浓度与病死率和PICU住院时间的预测能力。结果:死亡36例(7.2%)。根据PIM评分,死亡儿童的平均死亡概率为23.6% 28.9,显著高于存活儿童的平均死亡概率(3.4% 7.3;P < 0.001)。PIM的ROC曲线下面积为0.81 0.03 (95% CI: 0.74-0.89)。非幸存者的乳酸水平为4.9% - 3.5 mmol/L,显著高于幸存者(1.9% - 1.5 mmol/L;P < 0.001)。血乳酸曲线下面积为0.76 0.04 (95% CI: 0.67 ~ 0.85)。两组ROC曲线间无统计学差异。在幸存者中,PIM评分与PICU住院时间之间存在显著关系,而在非幸存者中,血乳酸浓度与住院时间之间存在反比关系。结论:PIM评分和入PICU时血乳酸浓度对危重患儿预后均有中等预测价值。PIM评分的预后价值大于血乳酸浓度,但更难获得,而血乳酸浓度的测定快速简便。
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引用次数: 0
[Congenital hyperferritinemia and cataract syndrome]. [先天性高铁蛋白血症与白内障综合征]。
Pub Date : 2002-11-01
J A García-Erce
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引用次数: 0
[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base]. 朗格汉斯细胞组织细胞增多症:具有相同组织病理基础的多种表现。
Pub Date : 2002-11-01
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.

朗格汉斯细胞组织细胞增多症(LCH),以前称为组织细胞增多症X,是一种罕见的疾病。其特点是组织细胞、嗜酸性粒细胞和朗格汉斯细胞聚集和增殖,电镜下可见Birbeck颗粒。它涉及单个器官或系统,也可以表现为多系统疾病。临床表现可能有很大差异,从自发消退的良性自限性到缓慢进展的恶性疾病。我们报告了5例LCH病例,其组织病理基础相同,但结果不同。
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引用次数: 0
[beta-hCG-induced precocious puberty?]. [β - hcg引起的性早熟?]
Pub Date : 2002-11-01
P Betrián Blasco, M C Boldova Aguar, J I Labarta Aizpún, A Carboné Bañeres, C Calvo Escribano
{"title":"[beta-hCG-induced precocious puberty?].","authors":"P Betrián Blasco,&nbsp;M C Boldova Aguar,&nbsp;J I Labarta Aizpún,&nbsp;A Carboné Bañeres,&nbsp;C Calvo Escribano","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":7778,"journal":{"name":"Anales Espanoles De Pediatria","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22141792","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}
引用次数: 0
[Childhood headache. A diagnostic approach]. (儿童头痛。诊断方法]。
Pub Date : 2002-11-01
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.

头痛在儿童时期很常见,在青春期更为常见。合理的,具有成本效益的评估儿童头痛开始于仔细的历史。第一步是确定头痛的时间模式——急性、急性复发、慢性进行性、慢性非进行性或混合性。下一步是身体和神经检查。在儿童头痛的评估中,神经影像学不是常规的保证,应该保留用于在神经检查中出现急性或慢性进展型异常的儿童。儿童偏头痛不同于成人偏头痛。最近的研究表明,需要修订儿童偏头痛的诊断标准,这将使其在这个年龄组的真实患病率得以确定。如果减少偏头痛的最短持续时间,如果在严重头痛伴有恶心时允许诊断偏头痛,即使不符合位置、质量和身体活动加重的标准,国际头痛学会(IHS)儿童偏头痛标准的敏感性和特异性将会提高。儿童和青少年在偏头痛和紧张性头痛的IHS标准的实现上没有差异。与年龄无关,头痛的强度和有无恶心是区分两种主要类型特发性头痛的最重要特征。偏头痛、变型偏头痛、紧张性头痛和其他类型的头痛通常在儿童时期首次出现,需要儿科医生的密切随访。对这种疾病的调查仍在进行中。
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引用次数: 0
[Antenatal diagnosis and isotopic studies in urethral ectopia]. 尿道异位的产前诊断和同位素研究。
Pub Date : 2002-11-01
J Mallo Castaño, L M Rodríguez Fernández, C de la Fuente Domínguez
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引用次数: 0
期刊
Anales Espanoles De Pediatria
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