平阳霉素及糖皮质激素与激光联合治疗儿童颌面部血管瘤

目的探讨和评价平阳霉素及糖皮质激素与激光联合应用治疗儿童颌面部血管瘤的疗效。方法自2002年3月至2005年2月,采用平阳霉素及糖皮质激素与激光联合治疗儿童颌面部血管瘤78例,瘤体面积0.4cm×0.8cm~5.0cm×8.0cm。7~10d注射一次平阳霉素配制液,剂量为0.5mg/kg,3~5次为一个疗程,一个疗程的总量不超过40mg;小于6岁的患儿配合口服强地松;最后联合可调脉宽倍频ND:YAG激光治疗。结果治疗后经6个月至3年的随访,治愈和基本治愈率88.46%,有效率100%。结论平阳霉素及糖皮质激素与激光联合应用治疗儿童颌面部血管瘤是一种安全、有效、易行的治疗方法。     

Subclinical course of adult visceral Niemann–Pick type C1 disease. A rare or underdiagnosed disorder?

Summary  We present the third case of Niemann–Pick disease type C without neurological symptoms. The patient was a 53-year-old woman without significant prior health problems who died of acute pulmonary embolism. Autopsy findings of hepatosplenomegaly, lymphadenopathy and ceroid-rich foam cells raised the suspicion of the visceral form of acid sphingomyelinase deficiency (Niemann–Pick disease type B; NPB) or a much rarer disorder, variant adult visceral form of Niemann–Pick disease type C (NPC). To verify the histopathological findings, SMPD1, NPC1 and NPC2 genes were analysed. Two novel sequence variants, c.1997G>A (S666N) and c.2882A>G (N961S) were detected in the NPC1 gene. No pathogenic sequence variants were found either in the SMPD1 gene mutated in NPB or in NPC2 gene. The pathogenicity of both NPC1 variants was supported by their location in regions important for the protein function. Both variations were not found in more than 300 control alleles. Identified sequence variations confirm the diagnosis of the extremely rare adult visceral form of Niemann–Pick disease type C, which is otherwise dominated by neurovisceral symptoms. Although only three patients have been reported, this (most probably underdiagnosed) form of NPC should be considered in differential diagnosis of isolated hepatosplenomegaly with foam cells in adulthood. Electronic supplementary material  Supplementary material is available for this article at     

Peripheral Vascular Complication from Coronary Angiography: a Case Report of Cholesterol Embolization

A 65-year-old man developed acute limb ischemia, severe abdominal wall and lower limb livedo reticularis following a coronary angiogram. The differential diagnoses of acute limb ischemia and multiple cholesterol emboli syndrome (MCES) are discussed. This work was performed at Long Island Jewish Medical Center, 270-05, 76^th Avenue, New Hyde Park, NY 11040.     

移植肝缺血-再灌注损伤程度的评估及其与肝移植患者预后的关系

目的探讨移植肝缺血-再灌注损伤程度的评估方法及其与肝移植患者预后的关系。方法218例良性终末期肝病患者，在移植肝恢复血液灌注后1h采取外周静脉血，测定丙氨酸转氨酶浓度（定义为基础肝功能），同时采用组织气体分析仪测定肝组织的氧分压，并取肝组织活检，计算水变性及坏死细胞百分比，分别对上述3项指标进行评分，再根据各指标得分之和将缺血-再灌注损伤程度划分为5级（0～Ⅳ级），统计围手术期（术后2周内）、术后近期（术后2周至1个月）、术后中远期（1个月以上）的患者死亡率。结果移植肝缺血再灌注损伤程度评为0级者157例（A组），死亡7例（4．5％），71．4％（5／7）死于术后3-6个月；缺血-再灌注损伤程度评为Ⅰ级者25例（B组），死亡5例（20．0％），80．0％（4／5）死于术后2周至3个月；缺血-再灌注损伤程度评为Ⅱ级者23例（C组），死亡5例（21．7％），80．0％（4／5）死于术后2周至3个月；缺血-再灌注损伤程度评为Ⅲ级者8例（D组），死亡7例（87．5％），85．7％（6／7）死于术后1个月内；缺血-再灌注损伤程度评为Ⅳ级者5例（E组），全部死亡，80．0％（4／5）死于术后1个月内。A组各期死亡率明显低于B组、C组（P〈0．05）和D组、E组（P〈0．01）；B组、C组间各期死亡率的差异无统计学意义（P〉0．05）；B组、C组各期死亡率均低于D组、E组（P〈0．05）。结论基础肝功能、组织氧分压以及水变性和坏死细胞百分比三项指标可基本反映移植肝缺血-再灌注损伤程度；缺血-再灌注损伤评级达Ⅲ～Ⅳ级者术后死亡率较高。     

Light emitting diodes for auramine O fluorescence microscopic screening of Mycobacterium tuberculosis.

We describe the simple adaptation of a standard fluorescent microscope for illumination using a 'Royal Blue' Luxeon light emitting diode (LED) and demonstrate that this form of illumination is suitable for the detection of auramine O stained Mycobacterium spp. The low cost, low power consumption, safety and reliability of LEDs makes them attractive alternatives to mercury vapour lamps.     

精神分裂症首次发病患者的磁共振弥散张量显像研究

目的探讨从未用过药的精神分裂症首次发病(以下简称首发)患者的重要白质及部分灰质区的磁共振弥散张量显像(DTI)的特点。方法选取9例首发精神分裂症患者(患者组)及9名年龄、性别、受教育程度与患者组相配对的健康者,应用DTI成像技术检测脑额颞交界处、内囊等白质区和颞中回灰质、中央前回、中央后回等灰质区的各向异性(FA)、表观扩散系数(ADC)及双侧海马体积。结果患者组及对照组组内左右两侧兴趣区FA值及ADC值的差异无统计学意义(P>0.05);患者组与对照组各感兴趣区FA值差异无统计学意义(P>0.05),但患者组颞中回灰质(8.655×10-9)、中央前回(7.816×10-9)、中央后回(7.855×10-9)ADC值高于对照组(分别为7.428×10-9,6.921×10-9,7.013×10-9;P=0.049,0.009,0.005);两组内及组间双侧海马体积比较,差异均无统计学意义(P>0.05)。结论首发精神分裂症患者与健康者DTI参数之间没有明显区别。     

阿立哌唑治疗难治性精神分裂症的疗效观察

目的观察阿立哌唑治疗难治性精神分裂症的疗效及不良反应。方法48例难治性精神分裂症患者,随机分为两组,分别给予阿立哌唑与氯氮平治疗,疗程8周。采用阳性与阴性症状量表(PANSS)、副反应量表(TESS)评定疗效及不良反应。结果阿立哌唑组显效率为65．4%,氯氮平组显效率为68．2%。氯氮平药物副反应明显高于阿立哌唑组。结论对难治性精神分裂症阿立哌唑疗效与氯氮平相似,但副反应少。