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Glucose-6-phosphate dehydrogenase is an enzyme that protects the erythrocytes against oxidative damage. G6PD deficiency is a common disorder in Gulf Countries that may present with hemolytic anemia and acute renal failure after exposure to oxidative triggers, such as drug, infection or some foodstuffs. In this study, a severe and complicated case of a G6PD deficient patient was presented. A 65-year old G6PD deficient patient presented with acute renal failure and intravascular hemolysis after eating fava beans. Therapeutic plasma exchange was used in the patient for two consecutive days. Clinical and laboratory findings were improved with plasma exchange. Although supportive therapy is enough for mild cases, therapeutic plasma exchange can be life-saving in complicated cases.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cause of chronic kidney disease. The intriguing role of innate immune system and inflammation become a target for potential therapeutic approach to slow progression. When toll-like receptors (TLRs) signaling and their receptors activate, they start a cascade of intracellular signaling that induces the production of the inflammatory cytokines and chemokines. Thus, we aim to investigate the association of TLRs between progression of ADPKD. Ninety ADPKD patients and ninety matched controls were enrolled this prospective study and were followed during 3 years. TLR-2 and TLR-4 gene polymorphisms and expressions were measured. Hypertension was diagnosed with ambulatory blood pressure monitoring. Rapid progression was defined as sustained decline in estimated glomerular filtration rate (eGFR) of more than 5 mL/min per 1.73 m2 per year. TLR-4Asp299Gly polymorphisms were significantly different between patient and control group (P

Background Overweight and obesity were recently associated with a poor prognosis in patients with autosomal dominant polycystic kidney disease (ADPKD). Whether the metabolic consequences of obesity as defined by the metabolic syndrome (MS) are also linked with disease progression remains untested. Methods Eligible ADPKD patients with different stages of CKD (n = 105) and 105 non-diabetic controls matched for CKD stage were enrolled in the study. Groups were evaluated at baseline for presence of MS, blood markers of metabolism, homeostasis model assessment of insulin resistance (HOMA-IR) score, and biochemical markers of inflammation (hs-CRP, IL-1 beta, IL-6, TNF-alpha and PON-1). MS was defined according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Patients were followed for 12 months and progression defined as a decrease in baseline eGFR > 10%. Results MS and hypertension were more prevalent amongst ADPKD patients than in the control group. Meanwhile, markers of inflammation such as hs-CRP (3.63 [3.45-5.17] vs. 4.2 [3.45-8.99] mg/dL; p = 0.014), IL-6 (21.65 [14.1-27.49] vs. 24.9 [16.23-39.4] pg/mL; p = 0.004) and IL-1 beta (21.33 [15.8-26.4] vs. 26.78 [18.22-35] pg/mL; p < 0.001) levels were all more elevated in ADPKD patients than in non-diabetic CKD subjects. In multivariate analysis having a truncating PKD1 mutation predicted (OR 1.25 [1.09-1.43]; p = 0.002) fulfilling the MS criteria. Finally, ADPKD patients fulfilling MS criteria had a significantly more rapid progression during 12 months of follow-up than did those that did not (OR 3.28 [1.09-9.87]; p = 0.035). Conclusions Our data supports the notion that dysmetabolisms part of the ADPKD phenotype and associated with a poor outcome, especially in patients with a truncating PKD1 mutation.

Renal interstitial fibrosis is an important pathological feature of autosomal dominant polycystic kidney disease (ADPKD), which progressively develops to end-stage renal disease (ESRD). It has been shown that apelin and transforming growth factor-β1 (TGF-β1) play important roles in the renal fibrosis process. The aim of the present study is to evaluate the relationship of these fibrosis markers and ADPKD. Forty-five patients with ADPKD and 28 healthy controls were studied cross-sectionally. Estimated glomerular filtration rate (eGFR), apelin, TGF-β1 were measured in all participants, using conventional methods. Apelin levels were lower (1.2 ± 0.9 ng/mL vs. 2.5 ± 1.3 ng/mL, P

Primary gastric mantle cell lymphoma is a rare form of gastointestinal tumour. Although peritoneal carcinomatosis accompanied by malignant ascites is relatively common, mantle cell lymphoma presenting with ascites is rare. Also, effusions involving pericardial and pleural cavities are uncommon during the course of lymphomas. We report the first case in which pericardial, pleural and peritoneal effusion of a primary gastric mantle cell lymphoma.

Background Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder with unclear disease mechanism. Currently, overt hypertension and increased renal volume are the best predictors of renal function. In this study, we assessed the usefulness of selected circulating microRNAs (miRs) to predict disease progress in a cohort with ADPKD. Methods Eighty ADPKD patients (44.6 ± 12.7 years, 40% female, 65% hypertensive) and 50 healthy subjects (HS; 45.4 ± 12.7, 44% female) were enrolled in the study. Serum levels of 384 miRs were determined by Biomark Real Time PCR. Groups were compared using the limma method with multiple-testing correction as proposed by Smyth (corrected p 10% loss of GFR over the following 12 months (cut-off >2.2 AU, sensitivity 83%, specificity 78%, area 0.872 [95% CI: 0.790–0.953, p

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cause of chronic kidney disease. The intriguing role of innate immune system and inflammation become a target for potential therapeutic approach to slow progression. When toll-like receptors (TLRs) signaling and their receptors activate, they start a cascade of intracellular signaling that induces the production of the inflammatory cytokines and chemokines. Thus, we aim to investigate the association of TLRs between progression of ADPKD. Ninety ADPKD patients and ninety matched controls were enrolled this prospective study and were followed during 3 years. TLR-2 and TLR-4 gene polymorphisms and expressions were measured. Hypertension was diagnosed with ambulatory blood pressure monitoring. Rapid progression was defined as sustained decline in estimated glomerular filtration rate (eGFR) of more than 5 mL/min per 1.73 m

Renal interstitial fibrosis is an important pathological feature of autosomal dominant polycystic kidney disease (ADPKD), which progressively develops to end-stage renal disease (ESRD). It has been shown that apelin and transforming growth factor-β1 (TGF-β1) play important roles in the renal fibrosis process. The aim of the present study is to evaluate the relationship of these fibrosis markers and ADPKD. Forty-five patients with ADPKD and 28 healthy controls were studied cross-sectionally. Estimated glomerular filtration rate (eGFR), apelin, TGF-β1 were measured in all participants, using conventional methods. Apelin levels were lower (1.2 ± 0.9 ng/mL vs. 2.5 ± 1.3 ng/mL, P 0.001), while TGF-β1 levels were higher in the patient group according to healthy controls (466.5 ± 200.5 ng/L vs. 367.1 ± 163.45 ng/L, P = 0.031), respectively. Apelin was negatively correlated with TGF-β1 and highly sensitive C-reactive protein (hs-CRP); and positively correlated with eGFR. In all subjects, eGFR was independently predicted by TGF-β1 and apelin. Apelin and TGF-β1 may be used as biomarkers of renal fibrosis that is an important pathological feature of ADPKD, which progressively develops to ESRD in ADPKD patients.

To evaluate whether the ratio between the number of metastatic lymph nodes and the number of total removed lymph nodes (MLR) is related survival in patients with locally advanced (stage 3) gastric cancer treated chemo-radiotherapy.We included 179 patients with locally advanced lymph node-positive gastric cancer treated chemo-radiotherapy within this study. The cut-off values, area under curve (AUC), sensitivity, and specificity were calculated using the receiver operating characteristic (ROC) curve technique for MLR.The sensitivity and specificity of MLR for mortality were 71.54% and 51.79%, respectively, when the cutoff level was accepted as the ratio ≥ 0.3. The AUC for the predictive value of MLR with regard to mortality was 0.609 (95% confidence interval [CI]: 0.533-0.681, p: 0.0135). Overall survival rates were significantly lower in patients with high MLR than in those with low MLR (15 months vs. 35 months, p: 0.002, respectively). In multivariate analysis, overall survival rates were significantly associated with MLR status (low or high).The simple and easily obtainable MLR is an independent predictor for mortality in patients with locally advanced gastric cancer even if chemo-radiotherapy, which is known to increase local control, was given.

Background/Aims: Early occurrence of hypertension is the prominent feature of autosomal dominant polycystic kidney disease (ADPKD). The role of angiotensin-converting enzyme (ACE) gene polymorphism and endothelial nitric oxide synthase (eNOS) gene polymorphism in the clinical course of ADPKD is not well understood. However, data about the expression of these genes are lacking. Thus, we aimed to investigate the polymorphisms and expressions of both the ACE and eNOS genes that affect hypertension in ADPKD. Methods: Whole blood samples were obtained from all participants. ACE and eNOS gene polymorphisms and their expressions were analyzed in 78 ADPKD patients and 30 controls. Gene expressions were assessed by quantitative real-time PCR. Twenty-four-hour blood pressure monitoring was performed for the diagnosis of hypertension in all study participants. Results:eNOS expression and the estimated glomerular filtration rate were found to be significantly higher in ADPKD patients without hypertension than in those with hypertension. Each unit of increase in eNOS expression led to a 0.88-fold decrease (95% CI: 0.80-0.96) in the risk of hypertension in multiple logistic regression analysis. Conclusions:eNOS gene expression is independently predictive of hypertension in the ADPKD population. This study showed, for the first time, a novel link between eNOS gene expression and hypertension in ADPKD.

A 63-year-old male presented with headache and weakness lasting for 2 months. Laboratory studies revealed the following: hemoglobin of 7.1 g/dL, white blood cells of 105x109/L, and platelets of 34x109/L. A diagnosis of acute promyelocytic leukemia was made by peripheral blood smear and bone marrow evaluations including morphological and genetic studies. The patient received one cycle of a chemotherapy regimen including cytarabine and idarubicin. During the aplastic phase, on day +6 from the end of chemotherapy, the patient developed fever, swelling on the left side of the face, infraorbital edema, and yellow-black discoloration of the upper palate (Figure 1). A paranasal sinus CT scan revealed a defect of the nasal septum and skin, and subcutaneous edema was seen at the maxillary and nasal level (Figure 2). The patient underwent surgical debridement. Histopathological assessment of the debridement specimen showed mucormycosis-associated hyphae. Culture of the nasal discharge was positive for Mucor spp. Liposomal amphotericin B was initiated at 5 mg/kg/day, but the patient died on the 30th hospital day. Informed consent was obtained. Figure 1 Yellow-black discoloration of upper palate. Figure 2 Paranasal sinus CT scan revealed a defect of the nasal septum and skin and subcutaneous edema. Mucormycosis is rapidly progressive and mortality for this infection is high [1,2,3,4]. Hematologic malignancies, long-term corticosteroid use, and immunosuppressive therapies are predisposing factors for mucormycosis. For management, mucormycosis should be considered early in high-risk patients, and surgical debridement together with effective antifungal therapy should be applied as soon as possible.

Cardiovascular disease (CVD) is the main cause of mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Prior to hypertension early vascular changes and inflammation have been reported. We aimed to investigate long pentraxin 3 (PTX-3), which has been recently described as a biomarker of inflammation, and its relation with endothelial dysfunction in early ADPKD patients. Twenty-five ADPKD patients without hypertension and 25 healthy controls were studied cross-sectionally. Hypertension was diagnosed with ambulatory blood pressure monitoring. Plasma concentrations of PTX-3 and proteinuria levels were obtained from each participant. Endothelial dysfunction was assessed using ischemia-induced forearm flow-mediated vasodilation (FMD). PTX-3 levels were higher in ADPKD patients compared to healthy controls (4.2 [1.2–10.1] vs. 1.4 [0.4–3.1] ng/ml, p

Background/Aims: Early onset of hypertension and its consequences account for the great majority of deaths in patients with autosomal dominant polycystic kidney disease (ADPKD). Renin-angiotensin system (RAS) components have been shown in ADPKD kidneys independent of systemic RAS. Thus, we examined the urinary angiotensinogen (UAGT) levels as a biomarker of intrarenal RAS status in ADPKD patients with/without hypertension and healthy subjects. Methods: Eighty-four ADPKD patients (43 with hypertension and 41 without hypertension) and 40 healthy controls were studied cross-sectionally. Patients with glomerular filtration rate Results: UAGT/UCre levels were higher in hypertensive ADPKD patients (23.7 ± 8.4) compared with normotensive ADPKD patients (16.6 ± 5.2) and healthy controls (6.9 ± 3.3; p < 0.001). In univariate analysis, UAGT correlated with systolic blood pressure, diastolic blood pressure (DBP) and proteinuria. The independence of these correlations was analyzed in a regression model, and UAGT was shown to be significantly predicted by proteinuria and DBP. Conclusion: Intrarenal RAS activation which is monitored by UAGT levels clinically may be a harbinger of hypertension and kidney disease in ADPKD patients.