Assessment of pulmonary hypertension in the pediatric catheterization laboratory: Current insights from the magic registry

Objectives: To assess protocols, demographics, and hemodynamics in pediatric patients undergoing catheterization for pulmonary hypertension (PH). Background: Pediatric specific data is limited on PH. Methods: Review of the Mid‐Atlantic Group of Interventional Cardiology (MAGIC) collaboration PH registry dataset. Results: Between November 2003 and October 2008, seven institutions submitted data from 177 initial catheterizations in pediatric patients with suspected PH. Pulmonary arterial hypertension associated with congenital heart disease (APAH‐CHD) (n = 61, 34%) was more common than idiopathic PAH (IPAH) (n = 36, 20%). IPAH patients were older with higher mean pulmonary arterial pressures (mPAP) (P < 0.01). Oxygen lowered mPAP in patients with IPAH (P < 0.01) and associated PAH not related to congenital heart disease (APAH‐non CHD) (P < 0.01). A synergistic effect was seen with inhaled Nitric Oxide (iNO) (P < 0.01). Overall 9/30 (29%) patients with IPAH and 8/48 (16%) patients with APAH‐non CHD were reactive to vasodilator testing. Oxygen lowered pulmonary vascular resistance index (PVRI) in patients with APAH‐CHD (P < 0.01). There was no additive effect with iNO but a subset of patients required iNO to lower PVRI below 5 WU·m². General anesthesia (GA) lowered systemic arterial pressure (P < 0.01) with no difference between GA and procedural sedation on mPAP or PVRI. Adverse events were rare (n = 7) with no procedural deaths. Conclusions: Pediatric patients with PH demonstrate a higher incidence of APAH‐CHD and neonatal specific disorders compared to adults. Pediatric PH patients may demonstrate baseline mPAP < 40 mm Hg but > 50% systemic illustrating the difficulty in applying adult criteria to children with PH. Catheterization in children with PH is relatively safe. © 2010 Wiley‐Liss, Inc.     

First experience With the biostar‐device for various applications in pediatric patients With congenital heart disease

Background: Interventional closure of atrial septal defects (ASD) and surgical tunnel fenestrations in Fontan patients has become the procedure of choice for many years. Recently, the BioSTAR Occluder, a modification of the Starflex device with a resorbable matrix has become available. Patients: Ten Biostar devices were implanted in nine children with interatrial septal defects, one within a fontan baffle, eight with secundum atrial septal defects. The age of the patients ranged from 11 months to 17 years, the body weight ranged from 12.9–78 kg. Results: 10 BioSTAR devices were implanted in nine patients. In one patient, two BioSTAR devices were used to occlude multiple defects within the oval fossa. All defects were successfully and uneventfully occluded. Mean procedure time was 56 (range 28–125). Mean fluoroscopy time was 4.8 (range 1.1 to 13.0) min. None of the nine patients showed residual shunts after device implantation. After 30 days no shunt was seen in the control transthoracic echocardiography. No adverse effects like allergic reactions, tachyarrhythmia or thrombembolic events occurred in any of the patients. Conclusion: The BioSTAR closure device is a safe and effective device for the closure of a variety of interatrial shunts in children including multifenestrated interatrial defects and fontan fenestrations, however, possible long term consequences (e.g., fractures, recurrent shunts after scaffold degradation) remain to be studied. © 2009 Wiley‐Liss, Inc.     

Radiofrequency recanalization of an occluded long segment left pulmonary artery in bidirectional cavopulmonary anastomosis

In patients with hypoplastic left heart syndrome who experience the Norwood surgical palliative program, development of the pulmonary artery branches is crucial. Balloon dilation and stenting may relieve potential stenosis but complete occlusion remains difficult to address with transcatheter therapy. We report the use of radiofrequency followed by balloon dilation and stenting to treat a long segment left pulmonary artery occlusion in a 15‐month‐old boy bidirectional Glenn. The left pulmonary artery was successfully recanalized with an excellent short‐term outcome. This further enhances the interest of radiofrquency for recanalization of occluded pulmonary artery branches, as an alternative to surgical reconstruction. © 2009 Wiley‐Liss, Inc.     

Prognosis of children with mixed phenotype acute leukemia treated on the basis of consistent immunophenotypic criteria

Background

Mixed phenotype acute leukemia (MPAL) represents a diagnostic and therapeutic dilemma. The European Group for the Immunological Classification of Leukemias (EGIL) scoring system unambiguously defines MPAL expressing aberrant lineage markers. Discussions surrounding it have focused on scoring details, and information is limited regarding its biological, clinical and prognostic significance. The recent World Health Organization classification is simpler and could replace the EGIL scoring system after transformation into unambiguous guidelines.

Design and Methods

Simple immunophenotypic criteria were used to classify all cases of childhood acute leukemia in order to provide therapy directed against acute lymphoblastic leukemia or acute myeloid leukemia. Prognosis, genotype and immunoglobulin/T-cell receptor gene rearrangement status were analyzed.

Results

The incidences of MPAL were 28/582 and 4/107 for children treated with acute lymphoblastic leukemia and acute myeloid leukemia regimens, respectively. In immunophenotypic principal component analysis, MPAL treated as T-cell acute lymphoblastic leukemia clustered between cases of non-mixed T-cell acute lymphoblastic leukemia and acute myeloid leukemia, while other MPAL cases were included in the respective non-mixed B-cell progenitor acute lymphoblastic leukemia or acute myeloid leukemia clusters. Analogously, immunoglobulin/T-cell receptor gene rearrangements followed the expected pattern in patients treated as having acute myeloid leukemia (non-rearranged, 4/4) or as having B-cell progenitor acute lymphoblastic leukemia (rearranged, 20/20), but were missing in 3/5 analyzed cases of MPAL treated as having T-cell acute lymphobastic leukemia. In patients who received acute lymphoblastic leukemia treatment, the 5-year event-free survival of the MPAL cases was worse than that of the non-mixed cases (53±10% and 76±2% at 5 years, respectively, P=0.0075), with a more pronounced difference among B lineage cases. The small numbers of MPAL cases treated as T-cell acute lymphoblastic leukemia or as acute myeloid leukemia hampered separate statistics. We compared prognosis of all subsets with the prognosis of previously published cohorts.

Conclusions

Simple immunophenotypic criteria are useful for therapy decisions in MPAL. In B lineage leukemia, MPAL confers poorer prognosis. However, our data do not justify a preferential use of current acute myeloid leukemia-based therapy in MPAL.     

妈咪爱联合蒙脱石散治疗小儿秋季腹泻86例疗效观察

目的观察妈咪爱与蒙脱石散剂联合治疗小儿秋季腹泻的疗效。方法将86例秋季腹泻患儿随机分为观察组和对照组各43例,均给予口服思密达及对症治疗,观察组加用口服妈咪爱,比较2组疗效。结果观察组显效率和总有效率均高于对照组,差异有统计学意义(P<0.05)。2组均无明显不良反应。结论妈咪爱联合蒙脱石散剂治疗小儿秋季腹泻疗效显著,值得临床推广应用。     

三聚氰胺污染奶粉致儿科泌尿系统结石44例分析

目的分析三聚氰胺污染奶粉喂养致儿科泌尿系统结石的临床诊疗特点。方法回顾性分析2008年9月至2008年12月所收治的44例泌尿系统结石患儿的临床资料,比较内科补液、碱化尿液治疗前后肾结石的变化。结果平均住院14d后复查肾脏B超未见结石者24例（54.6%）,结石较入院时缩小者18例（40.9%）,无明显变化者2例（4.5%）。结论4个月～2岁婴儿为三聚氰胺污染奶粉引起泌尿系统结石的主要发病人群。三聚氰胺致泌尿系统结石密度要低于其他性质的结石,结构松散,容易排出,内科排石治疗效果要远远好于其他性质的结石。     

布洛芬精氨酸盐注射液的制备及稳定性考察

目的:制备布洛芬精氨酸盐注射液并对其进行稳定性考察。方法:优化处方工艺后对布洛芬精氨酸盐注射液进行稳定性考察。采用反相高效液相色谱法测定布洛芬含量。结果:影响因素试验、加速试验及长期试验结果表明,布洛芬精氨酸盐注射液稳定。结论:布洛芬精氨酸盐注射液处方设计合理,工艺可行,质量稳定。     

液相色谱-质谱联用法检测非法制剂中的布洛芬

目的采用液相色谱-质谱联用法检测非法制剂中的布洛芬。方法选用Venusil MPC18色谱柱(150mm×4.6mm,5μm),以0.01mol/L醋酸铵溶液(含0.1%的三乙胺,用冰醋酸调pH至3.5)-乙腈(47:53)为流动相,对非法制剂的提取液进行液相色谱-质谱分析。结果通过比较对照品的色谱、紫外光谱及质谱行为,对非法生产的制剂进行定量测定和定性鉴别,在待检非法制剂中均检出了布洛芬。结论液相色谱-质谱联用法选择性强,灵敏度高,可用于分析检测非法制剂中的布洛芬。     

布洛芬-去甲丹皮酚偶合物的制备及其稳定性评价

目的制备布洛芬-去甲丹皮酚偶合物（IB-RP）,并考察其稳定性。方法碳酰亚胺法制备IB-RP,利用红外光谱、核磁共振和质谱对其进行结构表征,高效液相色谱法考察不同pH环境和大鼠血浆对其稳定性的影响。结果结构表征结果显示成功制备IB-RP,偶合物稳定性在高温条件下发生降解,随着pH值的上升其降解速度呈明显上升的趋势。另外,偶合物在血浆中迅速发生降解,其半衰期约为3 min。结论建立制备IB-RP的方法,其稳定性受pH值的影响,血浆中可迅速降解。