Hemoglobinopathies in the District of Antalya, Turkey

A screening program was conducted to ascertain the incidence of hemoglobinopathies in the district of Antalya, Turkey. The survey sample was selected from the household registration forms of health centers by systematic random sampling. Heparinized blood samples were collected from 1,616 subjects from 884 families.

The prevalence of p thalassemia traits with increased Hb A2 was 10.2%. This is higher than that found in previous studies performed in Antalya. The prevalence of abnormal hemoglobins (Hbs) was found to be 0.8%. Four subjects had Hb AS; five had Hb D-Los Angeles (B 121 [GH4] Glu-Gln); one had Hb Ube-2 (68 [E1] Asn-Asp), one had Hb P-Nilotic (fusion between B 22 and) and two had Hb D-like variants.     

Myxoid adrenal cortical carcinoma: a light and electron microscopic study

An adrenal cortical carcinoma demonstrating a peculiar myxoid background similar to that of a myxoma was found in a 41-year-old woman who also had parathyroid hyperplasia and clinical evidence of a pituitary tumor. The electron microscopic study demonstrated its value in establishing the diagnosis that was not possible to make with absolute certainty at the light microscopic level alone. The combination of endocrine disorders strongly suggests the possibility of an unusual multiple endocrine syndrome.     

Multifocal malignant pheochromocytoma presenting as a lung tumor

An unusual case of pheochromocytoma, which presented as a primary lung tumors, is described. The tumor was multifocal, arising in both the adrenal glands and in the chromaffin tissue around the thoracic aorta; it had invaded the lung and metastasized to the brain. Histologically the tumor had an unusual sarcomatous appearance, and the final diagnosis depended on the ultrastructual finding of scanty amounts of neurosecretory granules, typical of epinephrine and norepinephrine. This is the first reported case of pheochromocytoma presenting clinically as a lung tumor.     

Conjunctival impression cytology in pseudoexfoliative glaucoma and pseudoexfoliation syndrome

BACKGROUND: In this study, ocular surface changes and tear film functions in patients with pseudoexfoliative glaucoma (PEXG) and pseudoexfoliation (PEX) syndrome were evaluated. METHODS: This study is a randomized, double-blind and prospective study. Conjunctival impression cytology, tear film break-up time (TFBUT) test and basal Schirmer test (BST) were performed on 45 eyes of 45 subjects with PEXG (group I), 48 eyes of 48 subjects with PEX syndrome (group 2) and 50 eyes of 50 normal subjects (group 3). Impression cytology was graded 0-III based on the shape, eosinophilic-staining cytoplasm and nucleocytoplasmic ratio of the epithelial cells, and the shape, quantity and Periodic Acid Schiff-positive cytoplasm of the goblet cells. RESULTS: Impression cytology grading scores were 6.7% grade 0, 15.6% grade I, 37.7% grade II and 40% grade III in group 1, 6.3%, 18.7%, 43.8% and 31.2% in group 2 and 38%, 36.0%, 20% and 6% in group 3, respectively. The scores in groups 1 and 2 were significantly higher than in group 3 (P = 0.001, chi(2) = 39.84). The mean values of TFBUT of groups 1, 2 and 3 were 5.91 +/- 3.27, 7.39 +/- 4.82, 10.96 +/- 3.81 s and the mean values of BST were 7.82 +/- 3.56, 9.04 +/- 4.60 and 12.50 +/- 5.25 mm/5 min, respectively. The values of TFBUT and BST were lower in groups 1 and 2 than in group 3 (P = 0.001). CONCLUSION: The conjunctival changes in PEX and PEXG patients recorded in this study may explain the existence of tear film abnormalities in these patients. It has been shown that PEXG and PEX syndrome cause conjunctival surface changes and reduction of tear film functions.     

Does Aortic Root Enlargement Impair the Outcome of Patients With Small Aortic Root?

BACKGROUND: Posterior root enlargement procedures provide the implantation of suitable-sized prosthetic valves in patients with a small aortic root to prevent a high postoperative transvalvular gradient. The aim of this study was to evaluate long-term results of the posterior root enlargement. METHODS: Between 1985 and 2002, 124 patients underwent aortic valve replacement with a posterior root enlargement. The main indication was a small aortic valve orifice area to patient body surface area (indexed valve area < 0.85 cm2/m2). Fifty-four (44%) patients were male, and 70 (56%) were female with a mean age 39.1 +/- 14.3 years. Indications for operation were severe calcified aortic valve stenosis (37.1%), severe aortic insufficiency (25.8%), or combination (37.1%). Seventy-five (60%) patients received double-valve replacement. A pericardial patch was used in 100 patients (80.6%) and a Dacron patch was used in 24 patients. RESULTS: Operative mortality was 6.4% (8 patients). The causes of hospital mortality were low cardiac output syndrome (LCOS) (in 6 patients), cerebrovascular events (in 1 patient) and multiple organ failure (in 1 patient). Multivariate analysis demonstrated concomitant coronary revascularization to be a significant (p = 0.03) predictor for early mortality. There were six (5.4%) late deaths. Cox proportional hazards regression analysis demonstrated LCOS (p = 0.013) and infective endocarditis (p = 0.003) to be significant predictors for late mortality. Atrioventricular block required a permanent pacemaker was observed in 4 patients (3.2%). CONCLUSIONS: Posterior aortic root enlargement techniques can be easily applied without additional risks. Long-term survival and freedoms from valve-related complications are satisfactory.     

The effect of octreotide on pancreatic damage in TNBS-induced colitis

Inflammatory bowel disease, a chronic condition of the intestine, is associated with numerous extraintestinal manifestations, including pancreatitis. This study investigated the effect of octreotide administration on oxidative damage in a rat model of colitis induced by 2,4,6-trini-trobenzene sulfonic (TNBS) acid. Colonic and pancreatic malondialdehyde and glutathione levels are indicators of oxidative damage, and TNBS-induced colitis significantly increased the colonic and pancreatic malondialdehyde levels and decreased glutathione levels. Octreotide treatment was associated with decreased malondialdehyde levels and increased glutathione levels in the colonic and pancreatic tissue. The colonic mucosal structure was preserved and pancreatic inflammation decreased in rats treated with octreotide. Octreotide also significantly decreased nuclear factor-kB expression by immunohisto-chemistry in the colonic and pancreatic tissue compared with TNBS-induced colitis group. Octreotide appears to have protective effects against TNBS-induced colonic and pancreatic damage. These results imply the reduction in mucosal damage owing to the anti-inflammatory and antioxidant effects of octreotide.     

Effectiveness of a novel and scalable clinical decision support intervention to improve venous thromboembolism prophylaxis: a quasi-experimental study

ABSTRACT: BACKGROUND: Venous thromboembolism (VTE) causes morbidity and mortality in hospitalized patients, and regulators and payors are encouraging the use of systems to prevent them. Here, we examine the effect of a computerized clinical decision support (CDS) intervention implemented across a multi-hospital academic health system on VTE prophylaxis and events. METHODS: The study included 223,062 inpatients admitted between April 2007 and May 2010, and used administrative and clinical data. The intervention was integrated into a commercial electronic health record (EHR) in an admission orderset used for all admissions. Three time periods were examined: baseline (period 1), and the time after implementation of the first CDS intervention (period 2) and a second iteration (period 3). Providers were prompted to accept or decline prophylaxis based on patient risk. Time series analyses examined the impact of the intervention on VTE prophylaxis during time periods two and three compared to baseline, and a simple pre-post design examined impact on VTE events and bleeds secondary to anticoagulation. VTE prophylaxis and events were also examined in a prespecified surgical subset of our population meeting the public reporting criteria defined by the Agency for Healthcare Research and Quality (AHRQ) Patient Safety Indicator (PSI). RESULTS: Unadjusted analyses suggested that "recommended", "any", and "pharmacologic" prophylaxis increased from baseline to the last study period (27.1% to 51.9%, 56.7% to 78.1%, and 42.0% to 54.4% respectively; p < 0.01 for all comparisons). Results were significant across all hospitals and the health system overall. Interrupted time series analyses suggested that our intervention increased the use of "recommended" and "any" prophylaxis by 7.9% and 9.6% respectively from baseline to time period 2 (p < 0.01 for both comparisons); and 6.6% and 9.6% respectively from baseline to the combined time periods 2 and 3 (p < 0.01 for both comparisons). There were no significant changes in "pharmacologic" prophylaxis in the adjusted model. The overall percent of patients with VTE increased from baseline to the last study period (2.0% to 2.2%; p = 0.03), but an analysis excluding patients with VTE "present on admission" (POA) demonstrated no difference in events (1.3% to 1.3%; p = 0.80). Overall bleeds did not significantly change. An analysis examining VTE prophylaxis and events in a surgical subset of patients defined by the AHRQ PSI demonstrated increased "recommended", "any", and "pharmacologic" prophylaxis from baseline to the last study period (32.3% to 60.0%, 62.8% to 85.7%, and 47.9% to 63.3% respectively; p < 0.01 for all comparisons) as well as reduced VTE events (2.2% to 1.7%; p < 0.01). CONCLUSIONS: The CDS intervention was associated with an increase in "recommended" and "any" VTE prophylaxis across the multi-hospital academic health system. The intervention was also associated with increased VTE rates in the overall study population, but a subanalysis using only admissions with appropriate POA documentation suggested no change in VTE rates, and a prespecified analysis of a surgical subset of our sample as defined by the AHRQ PSI for public reporting purposes suggested reduced VTE. This intervention was created in a commonly used commercial EHR and is scalable across institutions with similar systems.     

Cytokine-like factor 1 gene expression is enriched in idiopathic pulmonary fibrosis and drives the accumulation of CD4+ T cells in murine lungs: evidence for an antifibrotic role in bleomycin injury

Idiopathic pulmonary fibrosis (IPF) is a progressive and typically fatal lung disease. To gain insight into the pathogenesis of IPF, we reanalyzed our previously published gene expression data profiling IPF lungs. Cytokine receptor-like factor 1 (CRLF1) was among the most highly up-regulated genes in IPF lungs, compared with normal controls. The protein product (CLF-1) and its partner, cardiotrophin-like cytokine (CLC), function as members of the interleukin 6 (IL-6) family of cytokines. Because of earlier work implicating IL-6 family members in IPF pathogenesis, we tested whether CLF-1 expression contributes to inflammation in experimental pulmonary fibrosis. In IPF, we detected CLF-1 expression in both type II alveolar epithelial cells and macrophages. We found that the receptor for CLF-1/CLC signaling, ciliary neurotrophic factor receptor (CNTFR), was expressed only in type II alveolar epithelial cells. Administration of CLF-1/CLC to both uninjured and bleomycin-injured mice led to the pulmonary accumulation of CD4(+) T cells. We also found that CLF-1/CLC administration increased inflammation but decreased pulmonary fibrosis. CLF-1/CLC leads to significantly enriched expression of T-cell-derived chemokines and cytokines, including the antifibrotic cytokine interferon-γ. We propose that, in IPF, CLF-1 is a selective stimulus of type II alveolar epithelial cells and may potentially drive an antifibrotic response by augmenting both T-helper-1-driven and T-regulatory-cell-driven inflammatory responses in the lung.