Preoperative platelet-to-lymphocyte ratio is a predictor of prognosis in patients with ampullary carcinoma.

AIM: To emphasize the significance of the platelet-to-lymphocyte ratio (PLR) in estimating the postoperative prognosis or survival measures in patients with carcinoma of the ampulla of Vater. METHODS: We retrospectively reviewed 82 patients, who underwent pancreaticoduodenectomy for ampullary carcinoma between July 2001 and April 2014. We investigated the predictive significance of the preoperative PLR for disease-free survival (DFS) or overall survival (OS). The possible correlations between the PLR and clinical or pathological features were also evaluated. RESULTS: The 5-year DFS and OS rates of the patients with carcinoma of the ampulla of Vater after pancreaticoduodenectomy were 51 % and 64 %, respectively. Multivariate analysis revealed a significantly worse OS in patients with a PLR ≥ 212 [hazard ratio (HR): 3.446; 95% confidence interval (CI): 1.4-8.43; p = 0.007], lymphovascular invasion (HR: 2.973; 95% CI: 1.25-7.03; p = 0.013), or pathological stage pT3/4 (HR: 2.761; 95% CI: 1-7.1; p = 0.035). Similarly, DFS was significantly worse in patients with lymphovascular invasion (HR: 2.24; 95% CI: 1.1-4.56; p = 0.025) or stage pT3/4 (HR: 2.243; 95% CI, 1.03-4.84; p = 0.04). CONCLUSION: The preoperative PLR shows a predictive significance for the prognosis of postoperative patients with carcinoma of the ampulla of Vater. We suggest that because of its predictive value, the PLR can be used in the development of further approaches to monitor and manage patients with poor prognosis Tab. 4, Fig. 1, Ref. 45).

A Very Rare Case -  Hairy Cell Leukemia in Patient with Sarcoidosis.

Although the coexistence of hairy cell leukemia with sarcoidosis has been reported in a few cases in the literature, in our case the patient had been diagnosed and followed about 10 years with sarcoidosis and massive splenomegaly. It has been demonstrated that T helper 1 cells exist in organs influenced by sarcoidosis. These cells produce IL-2 and IFN-γ and induce a nonspecific inflammatory response and granuloma formation. Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly.

Brentuximab vedotin for relapsed or refractory Hodgkin lymphoma: experience in Turkey.

Current treatment modalities can cure up to 70-80 % of patients with classical Hodgkin lymphoma. Approximately, 20-30 % of patients require further treatment options. Brentuximab vedotin has been approved for the treatment of relapsed and refractory Hodgkin lymphoma. In the present study, we report the experience with brentuximab vedotin as single agent in 58 patients with relapsed or refractory Hodgkin lymphoma. The objective response rate was 63.5 % with 13 complete responders (26.5 %) among 49 patients evaluated at the early phase of treatment (2-5 cycles). Upon treatment prolongation (≥6 cycles), 37 patients achieved a final objective response rate of 32.4 % with 21.6 % of complete and 10.8 % of partial response. Overall survival at 12 months was 70.6 %, and progression-free survival at 12 months was 32.8 %. Median overall survival could not be reached and median progression-free survival was 7 months. While the median duration of response was 9 months in the whole cohort, it was 11.5 months in the complete responders. Complete response rates in patients treated with >3 chemotherapy regimens before brentuximab vedotin were significantly lower (p = 0.016). Fourteen patients were subsequently transplanted. In conclusion, brentuximab vedotin provided a bridge to transplantation in approximately one quarter of the patients. The declining response rates during the course of treatment suggest that transplantation should be implemented early during brentuximab vedotin treatment.

Plasminogen activator inhibitor-1 levels in patients with primary varicose vein.

AIM: Plasmin is involved in extracellular matrix remodeling by activating some matrix metallo-proteinases and degrading extracellular matrix; therefore component of fibrinolytic system such as tissue plasminogen activator and plasminogen activators inhibitors (PAI-1) might have a role in the pathogenesis of vascular remodeling. In our study we aimed to investigate the levels of PAI-1 levels in patients with primary varicose veins (VV) and in their age and gender matched control group. METHODS: Forty-one consecutive patients with peripheral varicose veins and 37 healthy age and gender-matched control subjects were included in the study from the outpatient cardiology and cardiovascular surgery clinic. Study population consisted of 41 consecutive patients who met the inclusion criteria and diagnosed as having class II primary VV according to CEAP classification. Routine biochemical and hematological analysis were performed in all patients and control subjects. RESULTS: Plasma levels of PAI-1 were found to be lower in patients than those in control subjects (5.19±2.2 ng/mL vs. 6.47±2.6 ng/mL, P=0.025). Logistic regression analysis revealed that only the plasma levels of PAI-1 were found to be independently but inversely associated with the presence of primary VVs (Odds ratio: 0.80 CI: 0.64-0.99, P=0.04). CONCLUSION: We have shown that PAI-1 levels are significantly decreased in patients with pVVs and it has an independent association with the presence of pVVs. However, its exact relation and role via matrix metlalloproteinases on the pathogenesis of the disease remains to be elucidated in further studies.

Increased expression of exon 2 deleted MEFV transcript in familial Mediterranean fever patients.

A defect in MEFV gene expression regulation has been implicated in familial Mediterranean fever (FMF) etiopathophysiology. Here we show significantly higher expression level in second exon lacking MEFV transcript in FMF patients compared with healthy controls (P=0.026). Our results also point out a possible role of exon 2 deleted MEFV transcript in FMF pathogenesis.

Higher rates of streptococcal colonization among children in the Pacific Rim Region correlates with higher rates of group A streptococcal disease and sequelae.

Group A streptococcal (GAS) pharyngeal colonization rates were determined among 1061 asymptomatic students in Hawaii and American Samoa where acute rheumatic fever rates are high. All GAS isolates were emm sequence typed. Although pharyngeal colonization rates were low in Hawaii (3.4%), Pacific Islander children had significantly higher colonization rates (5.7% vs. 1.2% in other ethnic groups, p <0.05). The colonization rate was higher in American Samoa (13%). Few emm types that were infrequently observed in symptomatic infections in Hawaii were repeatedly identified in both sites. These emm types were previously described among asymptomatic children suggesting a type-specific association with pharyngeal colonization.

Ewing's sarcoma of the fibula presenting with venous thrombosis

Malignancies account for about 20% of incidentally diagnosed venous thromboembolism. Surgery- or chemotherapy-induced risk of thrombosis is also high in patients with cancer. We report on a young male with skeletal Ewing's sarcoma who presented with deep vein thrombosis in the affected limb, which is quite a rare clinical condition. Venous thrombosis of the lower extremities in young patients should prompt the clinician to search for underlying local malignancies (AU)

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Low plasma apelin levels in newly diagnosed type 2 diabetes mellitus.

OBJECTIVE: To investigate blood apelin concentrations in patients with newly diagnosed and untreated type 2 diabetes mellitus (T2DM) who had no additional disorder and to investigate the association of apelin with adiponectin, body mass indexes (BMI) and insulin sensitivity. METHODS: Forty patients with T2DM and 40 healthy controls were enrolled. Apelin levels were measured along with BMI, lipids, glucose, insulin and adiponectin levels, and HOMA-IR indexes. Age, sex and BMI were similar in the two groups. RESULTS: Plasma apelin and adiponectin levels were significantly lower in the diabetic group compared to controls (p<0.001, for both). Insulin levels and HOMA indexes were significantly higher in patients with T2DM (p<0.001 and p=0.001, respectively). Apelin levels were negatively correlated with age (r=-0.315, p=0.006), fasting blood glucose (r=-0.556, p<0.001) and HOMA indexes (r=-0.411, p=0.001), and positively correlated with plasma adiponectin levels (r=0.593, p<0.001). Plasma adiponectin was negatively correlated to plasma insulin (r=-0.379, p=0.001), fasting glucose (r=-0.604, p<0.001), HOMA-IR (r=-0.559, p<0.001) and BMI (=-0.229, p=0.04). CONCLUSIONS: Plasma apelin is reduced in newly diagnosed and untreated patients with T2DM having no confounders. Regulation of circulating apelin and adiponectin seems to be in the same manner in patients with T2DM. Dysregulation of apelin might be involved in the mechanism of establishment of overt diabetes mellitus as well as associated atherosclerotic complications.

Plasma apelin is lower in patients with elevated LDL-cholesterol.

Hypercholesterolemia is a major risk factor for atherosclerosis. Dysregulation of adipokines contribute to atherosclerotic diseases. Apelin has recently been shown to be secreted by the adipose tissue in association with hyperinsulinemia and inflammation. We searched plasma apelin levels in patients with elevated low density lipoprotein (LDL)-cholesterol having no additional disorder. Thirty-three patients with hypercholesterolemia and 50 age-, sex-, and body mass index-matched healthy controls were evaluated for their apelin, adiponectin and high sensitivity C-reactive protein (hsCRP) levels, and homeostasis model assessment (HOMA) indexes. Plasma apelin-12 and adiponectin were determined by ELISA and RIA, respectively. Plasma apelin levels were lower in patients with elevated LDL-cholesterol compared to healthy controls (p<0.001). Plasma adiponectin concentration was also lower in the dyslipidemic patients (p<0.001). hsCRP levels were similar in the two groups. Fasting plasma glucose was normal in both groups. HOMA indexes in the dyslipidemic group were higher than the controls (p=0.005). A mild to moderate negative correlation with HOMA and positive correlation with high density lipoprotein cholesterol of apelin was found in the dyslipidemic group. Plasma apelin is decreased in non-obese, non-diabetic and normotensive patients with elevated LDL-cholesterol. Low apelin levels in hypercholesterolemia seem associated with insulin resistance, which needs to be investigated in larger populations as well as in other atherosclerotic conditions.

The plasma levels of soluble P-selectin in subjects with prediabetes.

Prediabetes has been associated with an increased risk of cardiovascular disease and mortality. Soluble P-selectin (sP-selectin) is an index of platelet activation and also a risk factor for future vascular events. sP-selectin levels were investigated in prediabetic subjects who had no confounding factors such as hypertension, obesity or dyslipidaemia. sP-selectin, hsCRP levels and HOMA-IR indexes were measured in 40 prediabetic subjects (n = 24 for IFG and n = 16 for IGT) and age-, sex- and BMI-matched 40 healthy controls. sP-selectin levels in prediabetic subjects were not significantly different compared with those in controls (p = 0.12). Prediabetic group had similar hsCRP (p = 0.29), higher HOMA-IR indexes (p < 0.001) and lower HDL cholesterol levels (p = 0.001) when compared with healthy controls. The power of the study was 0.93 for sP-selectin, 0.7 for hsCRP and 1.0 for HOMA. Our data suggest that sP-selectin may not contribute to the prothrombotic state as well as the accelerated atherogenesis associated with prediabetes.

Proton magnetic resonance spectroscopy findings of a sacrococcygeal schwannoma.

Schwannoma is tumor of peripheral nerves, originating from Schwann cells. It is a rare nerve sheath tumor, which frequently occurs in the intracranial acoustic nerve and spinal nerves. We report on a 28-year-old woman who suffered from a large pelvic mass that was diagnosed to be sacrococcygeal schwannoma. The features of proton magnetic resonance spectroscopy (MRS) study are discussed. The magnetic resonance imaging instrument was a 1.5 T, Gyroscan Intera with a body coil as a radiofrequency transmitter and a signal receiver. T2-weighted images were obtained under the following conditions-turbo spin echo (TSE) T2: turbo spin echo repeat time (TR) = 4500 msec, echo time (TE) = 96 msec. Single voxel MRS was performed by the point-resolved spectroscopy technique with a long TE (136 msec). MRS measurement was performed on two different parts of the tumor. As well as strongly elevated choline and lipid signals, the tumor spectrum showed increased N-acetylaspartate resonances. MRS can be used effectively in the preoperative diagnosis of retroperitoneal and pelvic masses, which demonstrate unusual clinical features.

Brucellar breast abscess.

Brucellosis is an endemic disease seen in many countries. It may affect different organ systems. Brucellar breast abscess is a rare entity. We report the radiological findings of breast abscess due to brucella. A 63-year-old female was investigated with mammography, ultrasonography, magnetic resonance imaging (MRI), and magnetic resonance spectroscopy (MRS). A mass measuring 25 x 20 x 15 mm was detected in the left breast on mammography and ultrasonography. The mass was homogenously hyperintense on T1- and T2-weighted MRI images. On contrast-enhanced T1-weighted images, peripheral capsular enhancement was found. MR spectroscopic analysis of the mass revealed elevated lipid and acetate peaks. The diagnosis was provided by fine needle aspiration biopsy and specimen culture. The lesion had diminished in size after 12 months' treatment with combined tetracycline and rifampicine.

Whole blood versus red cells and plasma for exchange transfusion in ABO haemolytic disease.

Records of 381 neonates who underwent exchange transfusion (ET) due to ABO haemolytic disease at the Division of Neonatology of Hacettepe University, Ankara, Turkey, between January 1977 and December 2003 were reviewed. Records were kept for the type of blood used in ET, the number of ETs for each infant, adverse event attributable to ET and bilirubin levels before, and 4 and 8 h after each ET. Of 381 infants, 300 were transfused with whole blood, whereas 81 infants were transfused with O red cells suspended in A or B plasma. The re-exchange rate was higher in the whole blood group, compared with the erythrocyte and plasma group. Use of erythrocyte and plasma provided 30% reduction in the number of ETs per patient. Eight hours after the first ET, mean bilirubin levels were 84% of the pre-exchange values in the whole blood group and 73% of the pre-exchange values in the erythrocyte and plasma group (P = 0.001). As the use of O group red cells re-suspended in AB plasma decreased the re-exchange risk compared with O group whole blood, we suggest the use of O red cells re-suspended in AB plasma for the ET in cases of ABO haemolytic disease.

van der Knaap's leukoencephalopathy: report of five new cases with emphasis on diffusion-weighted MRI findings.

We present five new cases of van der Knaap's leukoencephalopathy, which is a rare abnormality characterized by infantile-onset white matter disease with swelling and mild clinical course. Based on the very rare histopathological data, this disease is considered a vacuolating myelinopathy with peripherally located intralamellar vacuoli. Although approximately 70 cases have so far been published, there are very limited data in the literature regarding diffusion-weighted imaging (DWI) findings of this recently discovered entity. In this paper, in addition to MRI, MRS and DWI findings in three patients are shown, apparent diffusion coefficient (ADC) maps are calculated, and ADC measurements made from various regions of interest are documented. MRI showed diffuse swelling and areas of T2 high signal in supratentorial white matter, sparing the cortex and central gray matter structures. MRS findings indicated some degree of neuronal loss. DWI and ADC maps showed increased diffusion rates, suggesting a different type of tissue damage than that would be expected in vacuolating myelinopathies. We believe that DWI and ADC mapping would be of help in providing a baseline for monitoring the progression of the disease.

Immunogenicity and efficacy of one dose measles-mumps-rubella (MMR) vaccine at twelve months of age as compared to monovalent measles vaccination at nine months followed by MMR revaccination at fifteen months of age.

BACKGROUND AND METHODS: measles is a common cause of morbidity and mortality in developing countries. Although the measles-mumps-rubella vaccine (MMR) is currently in use in developed countries, monovalent measles vaccine (MV) is routinely recommended by World Health Organization (WHO) at 9 months of age in Turkey, as in many other developing countries. In this study, 442 Turkish children received MV at 9 months of age and were revaccinated with MMR vaccine at 15 months of age. In the second group 495 children received MMR at 12 months of age with no earlier measles vaccination. Antibodies were measured before the first vaccination and 6 weeks after the MMR. All children had been followed for occurrence of measles infection for 60 months. Two vaccination schedules were compared for immunogenicity and protection rates. CONCLUSIONS: seroconversion and clinical protection rates were significantly higher in children who received only MMR at 12 months of age than in children revaccinated at 15 months of age. Seroconversion rate for measles was 69.9% in children who received MMR at 12 months of age and 90.3% in children revaccinated at 15 months of age (P=0.0003). While there was no measles case in children who were revaccinated, 12 (2.7%) children in the first group acquired measles during the follow-up period. Vaccination at 12 months of age appeared to be better than the current national standard. The late elimination of maternal antibodies and the inhibitory effect of a weak antibody response after the first dose of vaccine at 9 months may explain the better immunogenicity and efficacy of the MMR vaccine given at 12 months of age.

Breast milk beta-glucuronidase levels in hyperbilirubinemia.

Breast milk beta-glucuronidase was thought to be one of the etiological factors in the pathogenesis of late-onset breast-milk jaundice, but results of these studies are conflicting. In this study breast milk beta-glucuronidase levels were determined in groups with physiologic jaundice, early breast-feeding jaundice and late breast-milk jaundice. No difference in beta-glucuronidase levels of these three groups was found in samples taken on the 4th and 15th days of life. beta-glucuronidase activity in breast milk declined from the 4th to 15th day in all groups. These results imply that factors other than breast milk beta-glucuronidase activity should be investigated to reveal the pathogenesis of late-onset breast-milk jaundice.

Primary incisor triplication defect.

Conjoining abnormalities occur in less than 1 percent of the population. Triplication of primary teeth is even a rarer phenomenon with a prevalence of 0.02 percent. This study describes the clinical features and the light microscopic appearance of a case of triplicated primary teeth. The teeth with the conjoined defect were examined by light microscopy to evaluate the exact level and appearance of the fusion area. The light microscopic examination revealed grooves and cracks consisting of enamel and dentine in the fusion area, fusion was irregular and both dentin and cementum were confluent. It is concluded that the clinician should identify children were conjoined anomalies and should be aware of the oncoming problems.