儿童难治性肾病综合征的探讨95例临床分析

本文对95例肾病综合征进行分析,探讨了性别、年龄、发病迁延时间、浮肿、蛋白尿、血尿、血压、尿素氮、血浆蛋白、血浆胆固醇、免疫球蛋白、补体C_3与激素反应及分型的关系。认为下列综合分析可做为判定难治性肾病综合征的参考。难治性肾病多分布在7岁以上,激素治疗4～8周血浆蛋白尚未恢复,尿镜检反复出现红细胞及颗粒管型,血清r—球蛋白不低,而补体C_3降低,提示难治性肾病。各种感染常常是造成肾病综合心难以控制,甚至死亡的重要因素。     

继发性肝癌的外科治疗

目的探讨继发性肝癌的手术适应证和方法。方法对52例继发性肝癌的临床病例资料进行回顾性分析。结果分别采用了左半肝切除、右半肝切除、左外叶切除、右前叶切除、右后叶切除和肝楔形切除术。术后1、3、5年的生存率分别为28.3%、19.4%和11.9%。结论对适合手术的继发性肝癌,行肝切除术可以获得满意的效果。     

Long-term results of surgical treatment of dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis

Background contextLarge, prominent osteophytes along the anterior aspect of the cervical spine have been reported as a cause of dysphagia. Improvement of swallowing after surgical resection has been reported in a few case reports with short-term follow-up. The current report describes outcomes of a series of five patients with surgical treatment for this rare disorder, with a long-term follow-up.PurposeTo study the clinical and radiographic outcomes of a case series of patients surgically treated for dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis (DISH).Study designRetrospective review of a case series.Patient sampleFive cases from a University Hospital.Outcome measuresClinical and imagenological follow-up.MethodsThe records of five patients with dysphagia who had undergone anterior surgical resection of prominent osteophytes secondary to DISH were reviewed. Extrinsic esophageal compression secondary to anterior cervical osteophytes was radiographically confirmed via preoperative barium esophagogram swallowing study. All patients underwent anterior cervical osteophytes resection without fusion. Postoperatively, patients were followed-up clinically and radiographically with routine lateral cervical radiographs.ResultsPreoperative esophagogram showed that the esophageal obstruction was present at one level in three cases and two levels in two cases. The C3–C4 level was involved in three cases, C4–C5 in three cases, and C5–C6 in one case. There were no postoperative complications, including recurrent laryngeal nerve palsy, wound infection, or hematomas. All patients had resolution of dyphagia soon after surgery (within 2 weeks). Postoperative radiographs demonstrated complete removal of osteophytes. At final follow-up, ranging from 1 to 9 years (average 59.8 months, median 53 months), no patients reported recurrence of dysphagia. Final radiographic examination demonstrated minimal regrowth of the osteophytes.ConclusionsAlthough rarely indicated, surgical resection of anterior cervical osteophytes from DISH causing dyphagia produces good clinical and radiographical outcomes. After thorough evaluation to rule out other intrinsic or extrinsic causes of swallowing difficulty, surgical treatment of this uncommon condition might be considered.     

旋转铰链型与单纯铰链型膝关节肿瘤假体置换的临床比较研究

目的 研究膝关节肿瘤保肢手术两种假体置换的术后效果及其比较.方法 随访我院1999年6月2006年3月治疗的72例膝关节肿瘤患者，失访9例，余63例中40例采用单纯铰链型假体置换(A组)，23例采用旋转铰链型假体置换（B组）63例随访时间9月~8年平均3年9个月，按MSTS膝关节评分标准、ambulation scores、关节屈伸度及感染、复发、手术时间进行评估。结果：A组：手术时间2.6625±0.3362h，复发5例，转移死亡3例，感染2例； B组：手术时间2.5722±0.2878h，复发3例，转移死亡2例，感染2例 。B组1例术后发生旋转轴聚乙烯轴套断裂关节脱位，再次手术更换轴套后未再出现并发症，两组无假体松动病例。两组手术时间无统计学意义；去除复发及转移死亡病例后,剩50例, A组32例， MSTS评分平均23.0/30分。 B组18例，MSTS评分平均23.7/30分。两组MSTS评分p>0.05，无统计学意义；ambulation scores各分项总分均p<0.05，有统计学意义．两组关节伸直时为0°或接近0°,屈曲度A组119.3750±17.49°，B组125.0000±15.34°，无统计学意义（p>0.05）。结论： 1. 按ambulation scores标准，在步行舒适度和快速步行方面，旋转型铰链假体置换组与单纯铰链型假体置换比较有统计学意义；2.而MSTS标准则未能体现出两组术后功能方面的统计学意义，因此ambulation scores较MSTS评分更灵敏，更适合对膝关节保肢术后功能评价；3.两组在手术时间、术后临床屈伸功能及并发症方面，如复发、感染，未见显著差别，单纯铰链型不会出现脱位，而可旋转铰链型假体有脱位的可能。     

Detergent fractionation with subsequent subtractive suppression hybridization as a tool for identifying genes coding for plasma membrane proteins

Abstract:  The identification of tumor-specific proteins located at the plasma membrane is hampered by numerous methodological pitfalls many of which are associated with the post-translational modification of such proteins. Here, we present a new combination of detergent fractionation of cells and of subtractive suppression hybridization (SSH) to gain overexpressed genes coding for membrane-associated or secreted proteins. Fractionation of subcellular components by digitonin allowed sequestering mRNA of the rough Endoplasmatic reticulum and thereby increasing the percentage of sequences coding for membrane-bound proteins. Fractionated mRNAs from the cutaneous T-cell lymphoma (CTCL) cell line HuT78 and from normal peripheral blood monocytes were used for SSH leading to the enrichment of sequences overexpressed in the tumor cells. We identified some 21 overexpressed genes, among them are GPR137B, FAM62A, NOMO1, HSP90, SLIT1, IBP2, CLIF, IRAK and ARC. mRNA expression was tested for selected genes in CTCL cell lines, skin specimens and peripheral blood samples from CTCL patients and healthy donors. Several of the detected sequences are clearly related to cancer, but have not yet been associated with CTCL. qPCR confirmed an enrichment of these mRNAs in the rough endoplasmic reticulum fraction. RT-PCR confirmed the expression of these genes in skin specimens and peripheral blood of CTCL patients. Western blotting verified protein expression of HSP90 and IBP2 in HuT78. GPR137B could be detected by immunohistology in HuT78 and in keratinocytes of dysplastic epidermis, but also in sweat glands of healthy skin. In summary, we developed a new technique, which allows identifying overexpressed genes coding preferentially for membrane-associated proteins.     

Interaction of estrogen receptor‐α ligand binding domain with nuclear proteins of aging mouse brain

After the interaction of estrogen with the ligand binding domain (LBD) of mouse estrogen receptor‐α (mERα) and hormone‐responsive elements of target genes, many nuclear proteins are recruited to regulate the expression of specific genes. Because it is not known which brain proteins interact with LBD or whether these proteins vary with age and sex, we used pull‐down assay and far Western blotting to detect five nuclear proteins of 160, 140, 87, 60, and 46 kD in the mouse brain. These interacting proteins were identified as PELP1, RIP140, PGC1α, BAF60, and ADA3, respectively. The level of PELP1, RIP140, PGC1α, and BAF60 decreased drastically in old compared with adult male mice, whereas the ADA3 level showed no significant change. PELP1, PGC1α, and BAF60 levels were lower in old male compared with female mice. Thus we report the identification and interaction of five nuclear proteins with mERα‐LBD, indicating their role in estrogen signaling and brain functions during aging. © 2009 Wiley‐Liss, Inc.     

A randomized,double‐blind,placebo controlled,multi‐center study of intravenous iron sucrose and placebo in the treatment of restless legs syndrome

Iron deficiency may exacerbate symptoms in the Restless Legs Syndrome (RLS). We investigated the effect of intravenous iron sucrose or placebo on symptoms in patients with RLS and mild to moderate iron deficit. Sixty patients with primary RLS (seven males, age 46 (9) years, S‐ferritin ≤45 μg/L) recruited from a cohort of 231 patients were randomly assigned in a 12‐months double‐blind, multi‐centre study of iron sucrose 1000 mg (n = 29) or saline (n = 31). The primary efficacy variable was the RLS severity scale (IRLS) score at week 11. Median IRLS score decreased from 24 to 7 (week 11) after iron sucrose and from 26 to 17 after placebo (P = 0.123, N.S. for between treatment comparison). The corresponding scores at week 7 were 12 and 20 in the two groups (P = 0.017). Drop out rate because of lack of efficacy at 12 months was 19/31 after placebo and 5/29 patients after iron sucrose (Kaplan–Meier estimate, log rank test P = 0.0006) suggesting an iron induced superior long term RLS symptom control. Iron sucrose was well tolerated. This study showed a lack of superiority of iron sucrose at 11 weeks but found evidence that iron sucrose reduced RLS symptoms both in the acute phase (7 weeks) and during long‐term follow up in patients with variable degree of iron deficiency. Further studies on target patient groups, dosing and dosing intervals are warranted before iron sucrose could be considered for treatment of iron deficient patients with RLS. © 2009 Movement Disorder Society     

B cells as a target of immune modulation

B cells have recently been identified as an integral component of the immune system; they play a part in autoimmunity through antigen presentation, antibody secretion, and complement activation. Animal models of multiple sclerosis (MS) suggest that myelin destruction is partly mediated through B cell activation (and plasmablasts). MS patients with evidence of B cell involvement, as compared to those without, tend to have a worse prognosis. Finally, the significant decrease in new gadolinium-enhancing lesions, new T2 lesions, and relapses in MS patients treated with rituximab (a monoclonal antibody against CD20 on B cells) leads us to the conclusion that B cells play an important role in MS and that immune modulation of these cells may ameliorate the disease. This article will explore the role of B cells in MS and the rationale for the development of B cell–targeted therapeutics. MS is an immune-mediated disease that affects over 2 million people worldwide and is the number one cause of disability in young patients. Most therapeutic targets have focused on T cells; however, recently, the focus has shifted to the role of B cells in the pathogenesis of MS and the potential of B cells as a therapeutic target.