Anatomical reconstruction of the lateral ligaments using Gracillis tendon in chronic ankle instability; a new technique

Background

Many surgical technique have been described to assess the outcome of anatomical reconstruction of the lateral ligaments using Gracillis tendon. This technique aims to restore the stability of the ankle by reconstruction of the talofibular ligament (ATFL) and the calcaneofibular ligament (CFL) using the Gracillis tendon.

Methods

From January 2004 to February 2008; inclusive, 16 patients; 11 male and 5 female, underwent an anatomic reconstruction of the lateral ankle ligament for chronic ankle instability. Their ages ranged from 18 to 29 giving a mean age of 25 years. Patients were then subjected to radiologic and clinical assessments for a period of at least 33.5 months. For pain scoring the Americans Orthopaedic Foot and Ankle Society (AOFAS) scores were used; whilst subjective symptom was evaluated using the Olerud and Molander ankle scoring system.

Results

All patients returned for the final evaluation and subjective excellent or good results were recorded on self-assessment, pain scores, AOFAS and Karissons scores. Additionally Olerud and Molander ankle scoring was also done. During the final follow-up, the mean post-operative AOFAS score was 96 (range 80–100), the Visual analog score was 6 (range 0–4), Karissons score was 94.7 (range 80–100) and last but not least Olerud and Molander score was 87.5 (range 70–100). It was noted that the ankle range of motion was not affected by lateral ankle reconstruction. The talar tilt was reduced from a mean of 12–4° (p < 0.0001) and the anterior drawer was reduced from a mean of 11–4 mm (p < 0.001) by the ankle ligament reconstruction.

Conclusion

Anatomical reconstruction of the lateral ligaments of chronic ankle instability using Gracillis tendon graft resulted in successful results, excellent ankle stability, significant reduction in pain and negligible loss of ankle and hind foot motion.     

High-dose intravenous gammaglobulin in alloimmunized platelet transfusion recipients

High-dose intravenous gammaglobulin (polyvalent immunoglobulin G) has been shown to be of benefit in some patients with immune thrombocytopenic purpura (ITP), possibly by producing reticuloendothelial system blockade. We studied this approach in patients refractory to random donor platelet transfusion using an IV IgG preparation manufactured by the Swiss Red Cross. Eleven adult patients with acute leukemia received either 0.4 g IgG/kg/d intravenously X five days (four patients) or 0.6 g/kg/d X five days (seven patients). All patients had high levels of lymphocytotoxic antibody and poor responses to random donor platelets. Except for mild headaches in two patients, there were no side effects related to the IgG infusions. All patients had significant elevations of serum IgG on the day after completion of treatment. Either random donor or partially HLA-matched platelet transfusions were administered the day after and, in some cases, during the IgG therapy. No patient had an improvement in one hour posttransfusion platelet count increments. Two additional patients received pooled platelet concentrates incubated for 30 minutes at 37 degrees C with IgG at a final concentration of 3 g% prior to transfusions. These results indicate that high-dose IgG, an extremely expensive treatment, cannot be recommended for alloimmunized adults with leukemia.     

腹腔镜巨大前列腺囊腺瘤切除一例报告并文献复习

目的提高对巨大前列腺囊腺瘤的认识。方法回顾性分析收治的1例巨大前列腺囊腺瘤并成功进行腹腔镜切除的临床资料,结合文献复习进行讨论。结果本例患者成功行腹腔镜完整切除,手术时间125min,出血量60ml,术中双侧精囊输精管完整保留,术后会阴部坠胀不适消失,复查精液常规精子数目及活动度正常。已随诊12个月,肿瘤无复发。结论巨大前列腺囊腺瘤罕见,最终确诊要依靠病理诊断,其最有效的治疗是手术完整切除,而腹腔镜途径可作为完整切除的微创手段。     

Does preoperative needle localization lead to an increase in local breast cancer recurrence?

Between 1978 and 1981, 74 women with nonpalpable breast cancer underwent surgery after localization guides were placed. In 72 patients, guides were introduced parallel to the chest wall; in two the needle was positioned anteroposteriorly under computed tomographic guidance. Fifty-six cases (76%) were infiltrating cancer; 13 (17%), intraductal cancers; two (3%), inflammatory; and three (4%), lobular carcinoma in situ. Surgery was not used to treat the latter five patients. In the remaining 69 women, 42 (61%) were treated by means of modified radical mastectomy; six (9%), total mastectomy; 12 (17%), local excision and radiation therapy; and seven (10%), local excision alone; exact therapy for two women (3%) was unknown. At a minimum follow-up of 5 years, none of the 67 women in whom the parallel approach was used had a local recurrence. The authors conclude that preoperative placement of guides parallel to the chest wall does not appear to increase the risk of local breast cancer recurrence.     

Sclerosing cholangitis: CT findings

The value of computed tomography (CT) in the detection of primary sclerosing cholangitis (PSC) in the intrahepatic and extrahepatic biliary systems was assessed by comparing CT scans of 20 cases of PSC with cholangiographic findings. In 16 of 19 cases of extrahepatic duct disease demonstrated with cholangiography, CT demonstrated abnormalities of the common hepatic duct, or bile duct, including duct stenosis, mural nodularity, duct dilatation, wall thickening, and mural enhancement. CT demonstrated intrahepatic disease in all 20 cases, including duct dilatation, duct stenosis, pruning, and beading. CT was superior to cholangiography in characterization of the status of the intrahepatic duct system in 11 of 20 cases. In addition, CT demonstrated extrabiliary complications of PSC in 12 cases and superimposed cholangiocarcinoma in three cases. While cholangiography remains the standard for diagnosis and follow-up of PSC, CT can provide valuable information about the extent and complications of the disease.     

经关节入路微创钢板固定技术治疗股骨远端C型骨折

目的探讨经关节入路微创钢板固定(MIPPO)技术治疗股骨远端C型骨折的临床疗效。方法2002年4月～2005年2月,应用MIPPO技术治疗股骨远端C型骨折14例,按AO/ASIF分类：C1型3例,C2型6例,C3型5例。先行关节内骨折切开复位、松质骨螺钉固定,再行髁上部分骨折间接复位、经关节内切口插入髁支撑钢板或LISS钢板桥接固定骨折。结果12例患者获得10～32个月(平均18．4个月)随访,骨折均获愈合,愈合时间10周～12个月,平均4．6个月。按Kolmert和Wulff的评价标准：优4例,良5例,可2例,差1例,优良率为75%。结论应用MIPPO技术治疗股骨远端C型骨折实现了微创操作,具有创伤小、软组织干扰少、骨折愈合快等优点,疗效满意。     

Fukutin gene mutations in steroid-responsive limb girdle muscular dystrophy

OBJECTIVE: Defects in glycosylation of alpha-dystroglycan are associated with several forms of muscular dystrophy, often characterized by congenital onset and severe structural brain involvement, collectively known as dystroglycanopathies. Six causative genes have been identified in these disorders including fukutin. Mutations in fukutin cause Fukuyama congenital muscular dystrophy. This is the second most common form of muscular dystrophy in Japan and is invariably associated with mental retardation and structural brain defects. The aim of this study was to determine the genetic defect in two white families with a dystroglycanopathy. METHODS: The six genes responsible for dystroglycanopathies were studied in three children with a severe reduction of alpha-dystroglycan in skeletal muscle. RESULTS: We identified pathogenic fukutin mutations in these two families. Affected children had normal intelligence and brain structure and shared a limb girdle muscular dystrophy (LGMD) phenotype, had marked elevation of serum creatine kinase, and were all ambulant with remarkable steroid responsiveness. INTERPRETATION: Our data suggest that fukutin mutations occur outside Japan and can be associated with much milder phenotypes than Fukuyama congenital muscular dystrophy. These findings significantly expand the spectrum of phenotypes associated with fukutin mutations to include this novel form of limb girdle muscular dystrophy that we propose to name LGMD2L.     

GGA1 acts as a spatial switch altering amyloid precursor protein trafficking and processing

The beta-amyloid (Abeta) precursor protein (APP) is cleaved sequentially by beta-site of APP-cleaving enzyme (BACE) and gamma-secretase to release the Abeta peptides that accumulate in plaques in Alzheimer's disease (AD). GGA1, a member of the Golgi-localized gamma-ear-containing ARF-binding (GGA) protein family, interacts with BACE and influences its subcellular distribution. We now report that overexpression of GGA1 in cells increased the APP C-terminal fragment resulting from beta-cleavage but surprisingly reduced Abeta. GGA1 confined APP to the Golgi, in which fluorescence resonance energy transfer analyses suggest that the proteins come into close proximity. GGA1 blunted only APP but not notch intracellular domain release. These results suggest that GGA1 prevented APP beta-cleavage products from becoming substrates for gamma-secretase. Direct binding of GGA1 to BACE was not required for these effects, but the integrity of the GAT (GGA1 and TOM) domain of GGA1 was. GGA1 may act as a specific spatial switch influencing APP trafficking and processing, so that APP-GGA1 interactions may have pathophysiological relevance in AD.     

Late-onset epilepsy and occult cerebrovascular disease

The interface between cerebrovascular disease (CVD) and epilepsy is complex and multifaceted. Late-onset epilepsy (LOE) is increasingly common and is often attributed to CVD, and is indeed associated with an increased risk of stroke. This relationship is easily recognizable where there is a history of stroke, particularly involving the cerebral cortex. However, the relationship with otherwise occult, subcortical CVD is currently less well established yet causality is often invoked. In this review, we consider the diagnosis of LOE in clinical practice—including its behaviour as a potential mimic of acute ischemic stroke and transient ischemic attack; evidence for an association between occult CVD and LOE; and potential mechanisms of epileptogenesis in occult CVD, including potential interrelationships between disordered cerebral metabolism and perfusion, disrupted neurovascular unit integrity, blood–brain barrier dysfunction, and inflammation. We also discuss recently recognized issues concerning antiepileptic drug treatment and vascular risk and consider a variety of less common CVD entities associated with seizures.     

Identification and characterization of novel uroporphyrinogen decarboxylase gene mutations in a large series of porphyria cutanea tarda patients and relatives

Porphyria cutanea tarda (PCT) arises from decreased hepatic activity of uroporphyrinogen decarboxylase (UROD). Both genetic and environmental factors interplay in the precipitation of clinically overt PCT, but these factors may vary between different geographic areas. Decreased activity of UROD in erythrocytes was used to identify patients with UROD mutations among a group of 130 Spanish PCT patients. Nineteen patients (14.6%) were found to harbor a mutation in the UROD gene. Eight mutations were novel: M1I, 5del10, A22V, D79N, F84I, Q116X, T141I and Y182C. Five others were previously described: F46L, V134Q, R142Q, P150L and E218G. The new missense mutations and P150L were expressed in Escherichia coli. D79N and P150L resulted in proteins that were localized to inclusion bodies. The other mutations produced recombinant proteins that were purified and showed reduced activity (range: 2.3–73.2% of wild type). These single amino acid changes were predicted to produce complex structural alterations and/or reduced stability of the enzyme. Screening of relatives of the probands showed that 37.5% of mutation carriers demonstrated increased urinary porphyrins. This study emphasizes the role of UROD mutations as a strong risk factor for PCT even in areas where environmental factors (hepatitis C virus) have been shown to be highly associated with the disease.