3D spiral CT imaging of bone anomalies in a case of diastematomyelia.

The case of a 48-year-old woman, suffering from a diastematomyelia, is presented. This case and the diagnostic findings are used to demonstrate the demands on imaging methods with respect to a new classification of split cord malformations (SCMs) recently published. Although MRI is the method of choice for imaging of the spinal cord generally, only X-ray methods and especially conventional computer tomography provide the information necessary for correct classification of SCMs. Additional 3D-reconstructions from suitable CT-data are helpful in visualizing complex anomalies of bony structures found in most cases of SCM.     

Leukocyte integrin expression in patients undergoing cardiopulmonary bypass

BACKGROUND: The recruitment of leukocytes to vascular endothelium is controlled by adhesion events mediated through the beta2 integrins, whereas the response of extravasated leukocytes within the tissues is controlled through the beta1 integrins. Although cardiopulmonary bypass (CPB) has been shown to be associated with a systemic inflammatory response and elevated levels of beta2 integrins on leukocytes, its effect on the beta1 integrins is not known. This study investigated the effect of the protease inhibitor aprotinin on the expression of the beta1 and beta2 integrins on circulating leukocytes in patients undergoing CPB. METHODS: Patients undergoing primary elective coronary artery bypass grafting were randomized into full-dose aprotinin or placebo groups. Blood samples were obtained at nine time points preoperatively, intraoperatively, and up to 6 days postoperatively. The surface expression of the beta1 integrins VLA-1, -3, -4, -5, and -6 and of the beta2 integrins CD11a/CD18, CD11b/CD18, and CD11c/CD18 was measured by flow cytometry on gated neutrophil and monocyte subpopulations in whole blood. RESULTS: Expression of the beta1 integrins was not significantly altered during the study period and, therefore, aprotinin had no effect on the expression of these molecules. Of the beta2 integrins, CD11b/CD18 expression was significantly increased on neutrophils at 15 minutes after onset of CPB in the placebo group (p < 0.01) but not in the aprotinin group. CONCLUSIONS: This study showed that expression of the beta1 integrins on neutrophils and monocytes did not alter during the first 6 days after CPB. Expression of the beta2 integrin CD11b/CD18 increased significantly on neutrophils during CPB in control patients but not in patients treated with full-dose aprotinin.     

Leptomeningeal Carcinomatosis after Major Remission to Taxane-based Front-line Therapy in Patients with Advanced Breast Cancer

Aim: To determine the incidence of leptomeningeal carcinomatosis (LMC), as the first manifestation of systemic progression, in breast cancer patients after obtaining a major response (complete response, CR or >80% partial response, PR) to first-line taxane-based chemotherapy treated between 1996 and 2000 in our Medical Oncology Unit. Patients and methods: Patients with histologically proven breast cancer having either metastatic disease, or high-risk locoregional disease that were entered into treatment protocols with first-line taxane (paclitaxel or docetaxel) plus anthracyclines or mitoxantrone combinations and developed LMC as the first evidence of progression after major response (CR or >80% PR) were analyzed in the present study (n=155). Results: Seven patients with a median age of 54 (range: 40–70) years developed LMC as their first evidence of progression after taxane-based regimens with a median interval of 6 months (range: 2–18) from start of treatment to diagnosis of LMC. Five patients received intrathecal (i.t.) methotrexate treatment and whole brain radiotherapy (RT), while one patient received i.t. methotrexate and RT to lumbar spine. Two patients responded to treatment for LMC, while two achieved stable disease and three progressed. Two patients had elevated cerebrospinal fluid tumor markers (more than serum marker levels) that proved useful in monitoring response to treatment. Median survival after LMC was 3.6 months (range: 1–31+) and correlated positively to the interval from the initiation of taxane-based therapy to LMC (r=0.84, P=0.019). Seven out of 86 responders (8.1%; 95% confidence interval, 2.4–13.9) developed LMC as the first sign of progression after a major response to first-line chemotherapy. Conclusions: LMC after a major response to front-line taxane-based regimens represents a grave disease manifestation and its incidence appears increased when compared in retrospect to non-taxane-treated patients. Prospective evaluation of the incidence of LMC after taxane versus non-taxane-based treatment from large randomized multi-institutional trials is warranted and identification of potential prognostic factors might help identify patients requiring appropriate prophylactic therapy.     

Medico-legal risks associated to hand and wrist trauma

BACKGROUNDAcute hand and wrist injuries are common and may lead to long-term disability if not managed adequately. Claims for negligence have been increasing in medical practice over the past few decades, with hand and wrist injuries and their treatment representing a significant percentage of orthopedic surgery lawsuits. There is no available literature regarding medical malpractice claims in hand and wrist injuries and surgery in Greece.AIMTo identify claims related to hand and wrist trauma and surgery and to define the reasons of successful litigations.METHODSWe performed a retrospective study of all legal claims of negligence for hand and upper extremity surgery that went to a trial, attributed to all surgical specialties, in Greece for a 20-year period. Data was further analyzed to identify claims related to hand and wrist trauma and surgery.RESULTSThere were six malpractice claims related to hand and wrist trauma that ended in a trial. A missed diagnosis, which resulted in failure of initial management of the injury, was the main reason for a claim. Three of the six cases resulted in complete or partial loss of a finger. Two cases are still open, requiring an expert witness’s report, two cases were closed in favor of the defendant, and two cases were closed in favor of the plaintiff with a mean compensation of €2000 (€1000-€3000).CONCLUSIONMissed diagnosis was the main reason for a malpractice claim. Better understanding of factors leading to successful claims will help surgeons improve their practice to minimize legal implications and litigation.     

Association of liver cirrhosis related IgA nephropathy with portal hypertension

A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.     

Primary Hepatic Lymphoma: Dilemmas in Diagnostic Approach and Therapeutic Management

Primary hepatic lymphoma (PHL) is a very rare malignancy and is characterized by liver involvement at presentation with no affectation of the spleen, lymph nodes, peripheral blood, bone marrow, or other tissues until at least 6 months after diagnosis. PHL should be considered in the differential diagnosis in a patient with space-occupying liver lesions and normal levels of alpha-fetoprotein and CEA. A computed tomography (CT) scan is the commonly used modality for staging lymphomas. The widespread use of positron emission tomography/CT results in the improvement in the accuracy of detecting the extent of disease, response evaluation, and prognostication. The liver biopsy, due to its pleomorphic appearances in the needle biopsy specimen, can be very challenging. Current literature favors the combination of chemotherapy as the frontline treatment for its least invasiveness and improved survival. Favorable prognosis of PHL can be obtained by early surgery combined with chemotherapy in strictly selected patients. However, the optimal therapy is still unclear and the outcomes are uncertain.     

Immunohistochemical expression of cell-cycle proteins in gastric precancerous lesions

Background: The early indicator for the subject predisposed to gastric cancer is abnormal proliferation of gastric epithelial cells, such as atrophic gastritis (AG), intestinal metaplasia (IM), and dysplasia, which have been considered as precancerous lesions of gastric cancer. To determine whether p53 protein, cyclins D1, and D3, and p27^kip1 play a role in the carcinogenesis pathway of gastric cancer, we performed an immunohistochemical study of their expression in gastric precancerous lesions. Methods: A total of 1 45 endoscopic gastric biopsy specimens of AG, IM, and gastric dysplasia were studied. These molecular markers were localized by immunohistochemistry. Results: P53 was expressed in 15% of cases with gastric dysplasia and not in the pre‐dysplastic stages of the gastric mucosa. All cases were concerning high‐grade dysplasia. Cyclin D1 protein was almost undetectable in the precancerous lesions of gastric cancer. Cyclin D3 protein overexpression was seen in 10% of biopsies with IM, and 50% of biopsies with gastric dysplasia. High expression of p27^kip1 protein was demonstrated in all cases of chronic gastritis. As atrophy, IM, and dysplasia develop, expression of p27^kip1 protein is suppressed. In total, 15% of dysplastic cases showed no expression of p27^kip1 protein. Conclusions: (i) P53 mutation must be a late event during the development of gastric cancer. (ii) Cyclin D1 protein overexpression may not play a role in the progression from normal to neoplastic gastric mucosa, while overexpression of cyclin D3 is an earlier event during gastric carcinogenesis, and its role must be further evaluated. (iii) Reduced expression of p27^kip1 is a rather early event in gastric tumorigenesis, before dysplastic changes occur.     

Neurological manifestations of connective tissue diseases mimicking multiple sclerosis

The objective of the study was to analyze retrospectively the clinical, laboratory and imaging findings of multiple sclerosis (MS), such as the manifestations in a cohort of 132 patients referred to the neurology in and outpatient clinic. The proposed clinical and laboratory diagnostic criteria for MS and connective tissue disorders were systematically assessed in 132 consecutive patients. Cerebrospinal fluid serology and brain or spinal cord MRI were studied in all cases. In patients suspected for connective tissue disorder, schirmer test, rose bengal staining and biopsy of minor salivary glands were performed. A total of 115 (87%) patients were diagnosed to have definite MS, while 17 (13%) were diagnosed to have connective tissue disorder. Positive neurological and MRI findings were observed in both groups. The majority of patients with connective tissue disorder demonstrated extra-neurological manifestations like Raynaud’s phenomenon, arthritis, livedo reticularis, purpura and presence of multiple autoantibodies in their sera. All patients with MS should be screened systematically for connective tissue disorder. In the absence of pathognomonic clinical and laboratory findings, the diagnosis of MS is a diagnosis of exclusion.