Patient characteristics in Behçet's Syndrome and their associations with major organ involvement: a single-centre experience of 2118 cases.

Objectives: To evaluate the demographic and clinical characteristics of patients with Behçet's syndrome (BS), and to define their associations with the presence of major organ involvement (MOI).Method: Medical records of 2118 patients (964 males, 1154 females) were analysed retrospectively. MOI was defined as the presence of at least one of vascular, eye, nervous, or gastrointestinal system involvement. Univariable and multivariable binary and ordinal logistic regression analyses were applied to assess the factors that were potentially associated with MOI.Results: The mean ± sd age at diagnosis was 30.5 ± 9.4 years. Genital ulcer and joint involvement were more common in females (both p < 0.001), while MOI was more frequent in males (p < 0.001). Genital ulcer (p < 0.001) and vascular involvement (p = 0.006) were more common in patients with a younger age at diagnosis, while joint involvement was more common in older patients. A total of 1097 patients (51.8%) had at least one MOI, 322 (15.2%) at least two MOIs, and 48 (2.3%) at least three MOIs. Male gender, smoking history, and absence of genital ulcer were significantly associated with MOI in multivariable binary logistic regression. Multivariable ordinal regression analyses confirmed the association between MOI and male gender and smoking, but not the protective effect of genital ulcers. In both regression analyses, we found no significant effects of age, human leucocyte antigen-B51, skin involvement, or joint involvement on MOI.Conclusion: Male gender and positive smoking history have a significant influence on the presence of MOI in patients with BS.

Photoreceptor outer segment layer thickness measured manually on images from spectral domain optical coherence tomography in healthy volunteers.

BACKGROUND AND OBJECTIVE: To investigate photoreceptor outer segment layer thickness measured with a manual technique on images from spectral domain optical coherence tomography (OCT) in healthy volunteers. MATERIALS AND METHODS: In 60 eyes of 30 healthy volunteers, a spectral domain OCT device (Spectralis, Heidelberg Engineering) was used to obtain cross-sectional images of the retina. For each volunteer, two images of each eye were obtained in one sitting. Images were digitally enlarged and the manual calipers feature of the device's software was used to measure, at the lowest point in the fovea, the thickness of the photoreceptor outer segment layer. All measurements were performed by the same investigator. Repeatability was evaluated with the Bland-Altman repeatability coefficient, and intersubject variability with Pearson's coefficient of variation. RESULTS: The mean values of measurements across all the volunteers were as follows: right eye first image 38.1 micrometers, right eye second image 37.9 micrometers, left eye first image 37.9 micrometers, left eye second image 37.9 micrometers. The repeatability coefficient, i.e. the difference between repeated measurements which would be exceeded in only 5% of cases, was 1.6 micrometers. Coefficients of variation for the right eye were 3.4% for the first images and 3.4% for the second images, and for the left eye they were 3.2 and 4.0% respectively. CONCLUSION: With a manual method based on spectral domain OCT, the thickness of the photoreceptor outer segment layer at the central fovea can be measured within a useful range of repeatability and appears to be relatively constant across healthy volunteers.

An evaluation of diaphragmatic movements in hemiplegic patients.

OBJECTIVE: The aim of this study was to evaluate the effect of hemiplegia on diaphragmatic movements using motion-mode ultrasonography. METHODS: 23 hemiplegic patients who were diagnosed with a single-hemisphere lesion (mean age 60.5 years; 13 males and 10 females) and a control group of 20 patients (13 males and 7 females) were all evaluated by ultrasonography. Ultrasonography recordings were made of the amplitude of diaphragmatic movement during spontaneous and deep breathing. The patients underwent lung function tests. RESULTS: When the hemiplegic and control groups were compared, the forced vital capacity, forced expired volume in 1 s, maximum inspiratory pressure and maximum expiratory pressure values were significantly lower in the groups with right and left hemiplegia (p<0.05). When a comparison was made between the right hemiplegic group and the control group and between the left hemiplegic group and the control group in terms of diaphragmatic excursions, for both groups, no significant difference was determined between the movements of the right hemidiaphragm during spontaneous and deep breathing and those of the left hemidiaphragm in spontaneous respiration. In contrast, for both hemiplegic groups, a significant decrease was noted in the movements of the left hemidiaphragm in deep respiration. CONCLUSION: The diaphragm is both contralaterally innervated and ipsilaterally innervated, and innervation exhibits marked variations from person to person. This provides an explanation for varying diaphragmatic movements in hemiplegic cases during deep respiration.

Simvastatin therapy in lymphocyte cross-match-positive kidney transplantation candidates.

OBJECTIVES: Recent identification of several mechanisms by which statins decrease recruitment of monocytes and T cells into the arterial wall and inhibit both T-cell and B-cell activation and proliferation in vitro prompted us to study the immunomodulatory effects of statins. In this study, we examined the effect of simvastatin therapy on lymphocyte cross-match positivity in kidney transplantation candidates. METHODS: Simvastatin therapy (20 mg/d) was administered to 25 patients (18 men, 7 women of mean age 34 +/- 11.7 years who displayed positive lymphocyte cross-matches between July 2002 and October 2004. The etiologies of end-stage renal disease were vesicoureteral reflux (n = 5), urinary stone disease (n = 4), glomerulonephritis (n = 6), amyloidosis secondary to familial Mediterranean fever (n = 1), and unknown (n = 9). RESULTS: The lymphocyte cross-match became negative in 10 patients 4-9 months, and successful kidney transplantation was performed in 6 of them. The serum creatinine levels of these patients ranged between 0.8 and 1.4 mg/dL. Two patients required higher doses, but none suffered from adverse effects. The remaining 4 patients are still undergoing pretransplantation evaluation. CONCLUSION: Simvastatin therapy seems to be a cost-effective and useful method for lymphocyte cross-match-positive kidney transplantation candidates compared with immunoadsorption or intravenous immunoglobulin use.

Sirolimus as primary immunosuppression agent in kidney transplant recipients: Akdeniz University experience.

OBJECTIVES: Recently usage of sirolimus as the primary immunosuppressant is widening among kidney transplant recipients. We reviewed the clinical follow-up of patients transplanted at our center using sirolimus protocols. METHODS: Sirolimus including primary immunosuppressive treatment protocols were begun in February 2002. Among the 21 patients (15 men, six women) who received sirolimus, six patients were prescribed sirolimus + prednisolone; seven, sirolimus + mycophenolate mofetil + prednisolone; and eight, sirolimus + cyclosporine + prednisolone. The mean age of the patients was 32.9 +/- 7.3 years and the mean posttransplantation follow-up, 13.2 +/- 4.5 months. RESULTS: Three patients experienced acute rejection episodes, which were treated successfully with steroids. None of the patients had either hematologic or wound healing problems. Lymphoceles developed in eight patients. Serum creatinine level was 1.4 +/- 0.5 mg/dL at 12 months. There was a serious increase in serum cholesterol and triglyceride levels starting from the first month posttransplant (total cholesterol levels pretransplant and at 1 month, respectively: 159.3 +/- 29.5 and 255.7 +/- 52.3 mg/dL, P = .0001; triglycerides pretransplant and at 1 month, respectively: 146.9 +/- 89.5 and 215.1 +/- 102.5 mg/dL, P = .001). Despite routine antihyperlipemic treatment those high levels were maintained for 12 months. CONCLUSIONS: We achieved 100% graft and patient survival rates for 1 year among patients who were using sirolimus. But the most important role in defining the morbidity and mortality in this group of patients is cardiovascular events; for this reason the abnormalities in the lipid profile must be taken seriously.

Tacrolimus plus low-dose mycophenolate mofetil in renal transplant recipients: better 2-year graft and patient survival than with a higher mycophenolate mofetil dose.

OBJECTIVES: Mycophenolate mofetil (MMF) has become more widely prescribed in recent years, but its adverse effects on the gastrointestinal system and bone marrow restrict its use in certain settings. The aim of this study was to compare the demographic features and clinical data for 173 renal transplant recipients who received tacrolimus (TAC) plus 1 g/d MMF (group I, n = 112) versus TAC plus 2 g/d MMF (group II, n = 61 patients) over a 2-year period. Each patient received similar TAC doses. METHODS: We compared demographic data and clinical data for each case: acute rejection (AR) episodes, chronic rejection (CR) episodes, death, graft loss, development of posttransplantation diabetes mellitus (PTDM), and posttransplantation hypertension rates. RESULTS: Demographic features were similar. There were also no significant differences between groups I and II with respect to number of AR episodes (17/112 vs 12/61, respectively), number of CR episodes (4/112 vs 1/61, respectively), PTDM, and hypertension rate (P > .05). Kaplan-Meier survival analysis revealed 2-year graft survival rates of 94% in group I versus 83% in group II. The corresponding 2-year patient survival rates were 100% in group I versus 91% in group II. The graft survival and patient survival rates in group I were significantly higher than those in group II (log-rank 0.005 and 0.001, respectively). CONCLUSIONS: The 2-year graft and patient survival rates for the renal transplant recipients in this study suggest that the combination of a full TAC dose with 1 g/d MMF is a better choice than 2 g/d MMF.

Mercury poisoning: an unusual cause of polyarthritis.

Mercury is a toxic metal that is widely used in everyday life. It has organic and inorganic forms that are both toxic. As acute mercury poisoning is uncommon, diagnosis is difficult if the exposure is not manifest. It has usually a slow onset and non-specific symptoms. In this paper we report a patient who developed polyarthritis after mercury exposure.

Hand orthosis as a writing aid in writer's cramp.

Writer's cramp is a focal, task-specific dystonia of the hand and wrist. It primarily affects people who do a significant amount of writing, and causes difficulties in writing. We present five cases with writer's cramp who showed improvement in their writing ability with an applied hand orthosis.