12例开放性腋动脉损伤的诊治

目的探讨开放性腋动脉损伤的诊治体会。方法1996年-2002年，共诊治12例开放性腋动脉损伤的患者，从受伤到入院时间为3～14h，入院时均处于不同程度的休克状态，桡动脉搏动减弱或消失。在积极抗休克治疗的同时，急诊进行清创、血管神经探查和修复术。其中行腋动脉修补5例，腋动脉直接吻合6例，自体静脉移植修复1例。结果伤后8h内腋动脉再通8例，8～16h内腋动脉再通4例，无病例发生截肢或死亡。术后伤口均Ⅰ期愈合，桡动脉搏动良好。结论对开放性腋动脉损伤早期诊断和及时手术探查是控制休克、获取良好疗效的关键。     

The floating hip injury: which should we fix first?

In floating hip injuries, the priority of the steps in fixation has not been well described. We have treated these patients in accordance with the following protocols. In patients with pelvic ring fractures, the external fixation should be performed first, and in patients with acetabular fractures, the femoral fractures should be internally fixated first. The purpose of the present study was to review our experience with these fracture treatments to evaluate our strategy. For 8 years, 31 patients with femoral fractures associated with ipsilateral fractures: 26 unstable pelvic ring fractures and 7 unstable acetabular fractures were surgically treated at our level 1 trauma center. We reviewed the admission, operation, and follow-up records retrospectively and evaluated the order of the fixation and the complications. Two patients with acetabular fractures failed to follow our protocol outlined at the preoperative planning stage. There were no surgical difficulties other than two patients with the hemipelvis dislocated proximally who required traction of the fixated femur. The complications that were thought to be related to our protocol were one fat embolism syndrome and one pubic fracture displacement. One heterotopic ossification seemed to occur due to using a single approach for both fractures. We believe our protocol to be generally effective, however, in patients with acetabular and vertical shear type pelvic ring fractures, the internal or external fixation of the femur should be performed before the internal fixation of the pelvis.     

Electrophysiological studies of brain plasticity of the motor system

Neurological Sciences - Brain plasticity, intended as the pattern of functional and structural changes in responses to environmental physiological or pathological events, is underlined by several...     

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。     

Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

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.