Fronto-orbital leptomeningeal cyst manifested with exophthalmus

A 41-year-old female patient with fronto-orbital leptomeningeal cyst is presented. After decompression of the cyst, repair of bone and dura defects were achieved with autogenous bone grafts and pericranial flap. Our case was very interesting in several aspects: a head trauma at 3 years of age and manifestation of clinical symptoms (headaches and exophthalmia) after almost 4 decades that had been progressive for 6 months and a rare location (fronto-orbital region) that required a special management. A thorough history is crucial in such cases because clinical symptoms might appear after decades. An old fracture that is surrounded by thin calvarial tissue in conjunction with intracranial cystic formation should make one take a leptomeningeal cyst into consideration in differential diagnosis. Extensive dissection and adequate access osteotomies followed by repair with autologous and vascular tissues are the key factors for a successful outcome in the management of fronto-orbital pathologies.     

Renal hypoplasia and situs inversus totalis

A spectrum of renal abnormalities of patients with situs inversus has been reported. Renal dysplasia is the most common. Herein is described for the first time, an association of situs inversus totalis, unilateral congenital renal hypoplasia and external ear cartilage deformity.     

Phrenic nerve injury after blunt trauma

Phrenic nerve injury resulting from blunt trauma is unusual and may closely mimic diaphragmatic rupture. Diagnosis remains difficult and is often delayed. A prompt diagnosis requires a high index of suspicion. We describe one patient with phrenic nerve injury in whom the diagnosis was made late at the time of injury. Radiograph, ultrasonography, and computed tomography were helpful in the diagnosis. Video-assisted thoracic surgery was performed on our patient for diagnostic purposes. Left phrenic nerve injury and pericardial injury were found. Diaphragmatic plication was performed through a miniature left posterolateral thoracotomy. This case was presented to show the unusual nature of phrenic nerve injury.     

Allosteric modulation of native cochlear P2X receptors: insights from comparison with recombinant P2X2 receptors

Extracellular adenosine 5'-triphosphate (ATP)-gated ion channels assembled from P2X receptor subunits exhibit subunit-selective allosteric modulation by protons and divalent cations. In voltage-clamped guinea-pig cochlear outer hair cells (OHC) and Deiters' cells (DC), H(+) and Cu(2+), but not Zn(2+), enhanced the P2X receptor-mediated inward currents. Acid pH (6.5) potentiated OHC ATP-gated currents by 45%. Co-application of Cu(2+) (1-40 microM) with ATP increased the response by 20%. In DCs, ATP-gated currents were potentiated 85% by acid pH, and 70% by Cu(2+). Alkaline pH inhibited ATP-gated inward currents by 73% in OHCs and 85% in DCs. Zn(2+) was either ineffective (1-10 microM) or inhibitory (40-400 microM). Recombinant rat P2X(2) receptor-mediated inward currents in XENOPUS oocytes displayed allosteric modulation that was different from the native guinea-pig cochlear P2X receptors. The oocyte ATP-gated inward current was potentiated 450% by shifting from pH 7.5 to pH 6.5, and 130% with 40 microM Cu(2+). The enhanced response to ATP with acid pH and Cu(2+) is a signature of the P2X(2) subunit. In contrast to native guinea-pig cochlear cells, extracellular Zn(2+) (40 microM) increased the recombinant ATP-gated inward current by 200% in oocytes. These results suggest that the positive allosteric modulation of cochlear OHC and DC ATP-gated ion channels by protons and Cu(2+) arises in part from the P2X(2) receptor subunit, with additional regulatory elements.     

Comparative study of microfibrillar collagen hemostat (Colgel) and oxidized cellulose (Surgicel) in high transfusion-risk cardiac surgery

OBJECTIVE: The effects of microfibrillar collagen hemostat (Colgel) and oxidized cellulose (Surgicel) on bleeding and allogeneic transfusions were compared in cardiac operations with a predicted high risk of bleeding. METHODS: Between August 1999 and November 2001, 71 patients undergoing elective, high risk of bleeding operations were studied after giving informed consent. The procedures included repeat cardiac operations (aorta-coronary bypass operations or valvular operations), ascending aortic aneurysm repair necessitating deep hypothermic circulatory arrest, and ascending aortic grafting without deep hypothermic circulatory arrest. Subjects were excluded if they had recent (<5 days) acetylsalicylic acid ingestion, thrombolytic therapy, or anticoagulant therapy (heparin <4 hours preoperatively or warfarin <3 days preoperatively). Consenting subjects were randomized to receive either Colgel or Surgicel. RESULTS: Chest tube drainage in the first 24 hours was 373 +/- 143 mL in the Colgel group and 571 +/- 144 mL in the Surgicel group (P =.01). Total postoperative chest tube drainage was 423 +/- 154 mL (range, 280-1100 mL) in the Colgel group and 677 +/- 128 mL (range, 285-1350 mL) in the Surgicel group (P =.01). In addition, chest tube drainage was compared between the 2 groups every 3 hours after operation. Blood loss in the first 3 postoperative hours was significantly less in the Colgel group (132 +/- 41 vs 228 +/- 57 mL, P <.001). In the following 3-hour interval, this significant difference persisted (67 +/- 24 vs 121 +/- 49 mL, P <.001). CONCLUSIONS: In conclusion, the easy application, low cost, and significant blood-loss reduction effect of microfibrillar collagen powder renders this agent attractive for cardiac operations associated with high risk of bleeding.     

Cartilage graft or fascia in tympanoplasty in patients with low middle ear risk index (anatomical and audological results)

The aim of this study was to compare anatomic and audiological results of cartilage graft with temporal fascia graft in type 1 tympanoplasty patients with low middle ear risk index (MERI). In this retrospective study, 63 patients that underwent type 1 tympanoplasty with chondroperichondrial island graft between July 2009 and November 2010 were compared with 45 patients in whom temporal muscle fascia was used. Patients in both groups had low MERI values varying between 1 and 3. Five and nine patients underwent masteidectomy in cartilage and fascia group, respectively. Mean duration of follow-up was 11.9 ± 3.7 (5–17) months. Mean value was calculated at pre-operative and post-operative hearing threshold 0.5, 1, 2, 4 kHz, and air bone gap (ABG) gain was compared in both cartilage and fascia groups. when pre-operative and post-operative ABG gain were compared, significant decrease was seen in ABG levels (p < 0.001). However, no significant difference was seen in ABG gain values (p = 0.608), which was 10.1 ± 7.00 dB in cartilage group and 10.8 ± 5.38 dB in fascia group. In both groups, age, sex, and the addition of mastoidectomy procedure had no significant effect on ABG gain and success. Cartilage is a graft material that may be preferred without concern about the effects on hearing results, especially, in patients with low MERI values. The addition of mastoidectomy had no impact on the outcome of operation and audiological results. However, further studies with larger case series may be carried out to further clarify the issue.     

Urgent thoracotomy for penetrating chest trauma: analysis of 158 patients of a single center

Background

Penetrating injuries to the chest present a frequent and challenging problem, but the majority of these injuries can be managed non-\operatively. The aim of this study was to describe the incidence of penetrating chest trauma and the ultimate techniques used for operative management, as well as the diagnosis, complications, morbidity and mortality.

Methods

A retrospective 9-year review of patients who underwent an operative procedure following penetrating chest trauma was performed. The mechanism of injury, gender, age, physiological and outcome parameters, including injury severity score (ISS), chest abbreviated injury scale (AIS) score, lung injury scale score, concomitant injuries, time from admission to operating room, transfusion requirement, indications for thoracotomy, intra-operative findings, operative procedures, length of hospital stay (LOS) and rate of mortality were recorded.

Results

A total of 1123 patients who were admitted with penetrating thoracic trauma were investigated. Of these, 158 patients (93 stabbings, 65 gunshots) underwent a thoracotomy within 24 h after the penetrating trauma. There were 146 (92.4%) male and 12 (7.6%) female patients, and their mean age was 25.72 ± 9.33 (range, 15-54) years. The mean LOS was 10.65 ± 8.30 (range, 5-65) days. Patients admitted after a gunshot had a significantly longer LOS than those admitted with a stab wound (gunshot, 13.53 ± 9.92 days; stab wound, 8.76 ± 6.42 days, p < 0.001). Patients who died had a significantly lower systolic blood pressure (SBP) on presentation in the emergency room (42.94 ± 36.702 mm Hg) compared with those who survived (83.96 ± 27.842 mm Hg, p = 0.001). The overall mortality rate was 10.8% (n = 17). Mortality for patients with stab wounds was 8/93 (8.6%) compared with 9/65 (13.8%) for patients with gunshot wounds (p = 0.29). Concomitant abdominal injuries (p = 0.01), diaphragmatic injury (p = 0.01), ISS (p = 0.001), chest AIS score (p < 0.05), ongoing output (p = 0.001), blood transfusion volume (p < 0.01) and SBP (p = 0.001) were associated with mortality.

Conclusion

Penetrating injuries to the chest requiring a thoracotomy are uncommon, and lung-sparing techniques have become the most frequently used procedures for lung injuries. The presence of associated abdominal injuries increased the mortality five-fold. Factors that affected mortality were ISS, chest AIS score, SBP, ongoing chest output, blood transfusion volume, diaphragmatic injury and associated abdominal injury.     

Antibody-based therapy in Alzheimer's disease

INTRODUCTION: Alzheimer's disease (AD) is a progressive, age-dependent, neurodegenerative disorder being the most common cause of dementia. The pathological hallmarks are extracellular amyloid-beta (Aβ) deposition and intracellular neurofibrillary tangles containing hyperphosphorylated tau. Stunning results in vaccination trials in transgenic animals led to an antibody-based approach to develop immunotherapeutics for AD. Thus, several monoclonal antibodies raised against Aβ as well as polyclonal antibodies are currently in clinical testing and some of them already entered Phase III clinical trials. New upcoming experimental approaches like antibodies recognizing specific conformational epitopes, single-chain variable fragment antibodies, or intrabodies give hope of further drug development for this incurable disease. AREAS COVERED: The rationale and putative mode of action of antibody-based immunotherapy in AD and delineates correlations for other neurodegenerative diseases. Current clinical trials and recent results of therapeutic antibodies in AD are presented. EXPERT OPINION: Antibodies against Aβ will not only broaden the therapeutic repertoire but also our knowledge about the pathology in AD. Further results of the clinical trials and new antibody-based approaches will shed light on the importance of Aβ and its removal in AD.     

Read-through compound 13 restores dystrophin expression and improves muscle function in the mdx mouse model for Duchenne muscular dystrophy

Molecules that induce ribosomal read-through of nonsense mutations in mRNA and allow production of a full-length functional protein hold great therapeutic potential for the treatment of many genetic disorders. Two such read-through compounds, RTC13 and RTC14, were recently identified by a luciferase-independent high-throughput screening assay and were shown to have potential therapeutic functions in the treatment of nonsense mutations in the ATM and the dystrophin genes. We have now tested the ability of RTC13 and RTC14 to restore dystrophin expression into skeletal muscles of the mdx mouse model for Duchenne muscular dystrophy (DMD). Direct intramuscular injection of compound RTC14 did not result in significant read-through activity in vivo and demonstrated the levels of dystrophin protein similar to those detected using gentamicin. In contrast, significant higher amounts of dystrophin were detected after intramuscular injection of RTC13. When administered systemically, RTC13 was shown to partially restore dystrophin protein in different muscle groups, including diaphragm and heart, and improved muscle function. An increase in muscle strength was detected in all treated animals and was accompanied by a significant decrease in creatine kinase levels. These studies establish the therapeutic potential of RTC13 in vivo and advance this newly identified compound into preclinical application for DMD.     

Concentrations of connective tissue growth factor in patients with nonalcoholic fatty liver disease: Association with liver fibrosis

Aim: In this study, we aimed to investigate the relationship between the histological fibrosis stage of nonalcoholic fatty liver disease (NAFLD) and serum connective tissue growth factor (CTGF) to determine the usefulness of this relationship in clinical practice. Methods: Serum samples were collected from 51 patients with biopsy-proven NAFLD and 28 healthy controls, and serum levels of CTGF were assayed by ELISA. Results: Levels of CTGF were significantly higher in patients with NAFLD compared with controls (P=0.001). The serum CTGF levels were significantly increased, that correlated with histological fibrosis stage, in patients with NAFLD [in patients with no fibrosis (stage 0) 308.2 ± 142.9, with mild fibrosis (stage 1-2) 519.9 ± 375.2 and with advanced fibrosis (stage 3-4) 1353.2 ± 610 ng/l, P < 0.001]. Also serum level of CTGF was found as an independent predictor of histological fibrosis stage in patients with NAFLD (β = 0.662, t=5.6, P <0.001). The area under the ROC curve was estimated 0.931 to separate patients with severe fibrosis from patients with other fibrotic stages. Conclusion: Serum levels of CTGF may be a clinical utility for distinguishing NAFLD patients with and without advanced fibrosis.