Higher Pentraxin-3 Levels are Associated With Inflammation in Familial Mediterranean Fever.

BACKGROUND: Circulating levels of Pentraxin-3 (PTX3) have been shown to increase in several inflammatory conditions. However, there is no information about the levels of PTX3 in patients with familial Mediterranean fever (FMF). This study was designed to evaluate the serum PTX3 levels in patients with FMF during attack and free-attack periods. METHODS: Twenty FMF patients in attack and free-attack period, and 20 age-, sex-, and body mass index-matched healthy controls were included in the study. Blood samples were obtained within the first 24 h of the attack period and between attacks, and levels of white blood cell, erythrocyte sedimentation rate, Fibrinogen, high sensitive CRP, and PTX3 were determined. RESULTS: PTX3 levels during the attack period were not significantly different from those in free-attack patients (4.9 ± 4.6 ng/ml vs. 2.8 ± 1.4 ng/ml, P > 0.05). However, both attack and free-attack patients had significantly higher PTX3 levels than healthy controls (4.9 ± 4.6 ng/ml vs. 1.8 ± 0.8 ng/ml, P < 0.001; 2.8 ± 1.4 ng/ml vs. 1.8 ± 0.8 ng/ml, P < 0.025, respectively). CONCLUSIONS: PTX3 levels were not markedly affected from FMF attacks, but high level of PTX3 in free-attack period of FMF patients shows ongoing subclinical inflammation. However, further studies are needed to determine its usefulness as a marker in clinical practice.

The effectiveness of oral essential aminoacids and aminoacids containing dialysate in peritoneal dialysis.

BACKGROUND/AIM: Oral essential amino acids (AAs) containing supplements (EAS) and AA containing dialysate (ACD) are frequently used in peritoneal dialysis (PD) patients with malnutrition. The present study was conducted to investigate two strategies and compare their effects on the malnutrition status of PD patients. MATERIALS AND METHODS: A total of 31 EAS, 14 ACD patients were enrolled in this study. Serum albumin levels were lower than 3.5 g/dL in all subjects. EAS group patients took five pills containing AAs three times a day with meals. In the other, 2.000 cc of 1.1% ACD was given to patients daily during the study. Demographic and laboratory parameters were analyzed and compared at baseline and 6th month. RESULTS: Significant increases in BMI, albumin, and protein in both groups. Mean albumin levels increased significantly by 0.54 g/dL in ACD group (p < 0.005) and 0.49 g/dL in EAS group (p < 0.001) following 6 months. Mean albumin and delta albumin levels did not differ between two groups. CONCLUSION: These strategies may play an important role in increasing albumin levels and improving the nutritional status of PD patients.

The effect of biocompatible peritoneal dialysis solutions on neutrophil to lymphocyte ratio.

INTRODUCTION: Long-term exposure to dialysis solutions is an important contributor to the ongoing inflammatory process in peritoneal dialysis (PD) patients. Some studies have shown amelioration of this adverse effect with biocompatible solutions. We aimed to compare the neutrophil-to-lymphocyte (N/L) ratio in PD patients using biocompatible and standard solutions and to find out the association between N/L ratio and peritonitis indices. MATERIALS AND METHODS: This was a cross-sectional, multicenter study involving 120 prevalent PD patients. Seventy-one patients (59%) were using biocompatible solutions and 49 patients (41%) were using standard solutions. From blood samples, N/L ratio and platelet-to-lymphocyte ratio were calculated and mean platelet volume, erythrocyte sedimentation rate and hs-CRP values were detected. Data regarding the peritonitis rate and time to first peritonitis episode were also recorded. RESULTS: Biocompatible and standard groups were similar regarding age and gender. N/L ratio and hs-CRP levels have been found significantly higher in patients using biocompatible solutions (3.75 ± 1.50 vs. 3.27 ± 1.3, p = 0.04 and 3.2 ± 2.5 vs. 1.8 ± 2.0, p < 0.01, respectively). Peritonitis rates and time to the first peritonitis episode were found similar in patients using both types of solutions (0.23 ± 0.35 vs. 0.27 ± 0.32, p = 0.36 and 32.8 ± 35.8 vs. 21.5 ± 26.9 months, p = 0.16, respectively). DISCUSSION: N/L ratio was significantly higher in biocompatible solution users in parallel to hs-CRP levels, so biocompatible solutions seem to be related with increased inflammation in PD patients. Although we cannot make a certain explanation, we assume that there may be an association between acidity of the peritoneal content and virulence of microorganisms.

Serum soluble fas ligand levels in familial Mediterranean fever.

INTRODUCTION: Fas/FasL system plays an important role in the regulation of cell life and death, and circulating levels of sFasL have been shown to increase in some inflammatory conditions. However, there is no sufficient information about the levels of sFasL in patients with FMF. This study was designed to evaluate the serum sFasL levels in patients with FMF during attack and attack-free periods. METHODS: Twenty-five FMF patients in attack and forty-four in free-attack period, and 20 age-, sex-, and BMI-matched healthy controls were included in this study. Participants with any chronic diseases were excluded. Blood samples were obtained within the first 24 h of the attack period and between febrile attacks, and levels of WBC, ESR, Fibrinogen, hsCRP and sFasL were determined. RESULTS: The levels of traditional acute phase reactants during the attack were significantly higher than the attack-free and controls (p < 0.05). The serum sFasL levels in the FMF study groups did not differ from the control group (0.70 ± 0.08 vs. 0.73 ± 0.12; 0.70 ± 0.08 vs. 0.83 ± 0.14; 0.73 ± 0.12 vs. 0.83 ± 0.14, respectively, p > 0.05). Moreover, the sFasL levels during the attack were not significantly different from those in attack-free patients (0.70 ± 0.08 vs. 0.83 ± 0.14, p > 0.05). CONCLUSION: In this study, we demonstrated that serum sFasL levels were not markedly affected in FMF and cannot be used as a supportive marker to differentiate attacks from attack-free periods. However, further studies are needed to determine its usefulness as a marker in clinical practice.

Is colchicine therapy effective in all patients with secondary amyloidosis?

OBJECTIVE: Although colchicine is effective on prevention and regression of amyloidosis in many cases, rate of unresponsiveness to colchicine therapy is not too low. However, there is no sufficient data about which factors effect to response of colchicine therapy on regression of amyloidosis. MATERIALS AND METHODS: 24 patients with renal amyloidosis were enrolled into the study. The patients were divided in two groups according to urinary protein excretions: non-nephrotic stage (14/24) and nephrotic stage (10/24). The patients were also categorized according to the etiology of amyloidosis; familial Mediterranean fever (FMF)-associated amyloidosis (15/24) versus rheumatoid disorders (RD)-associated amyloidosis (9/24). The changes of amount of proteinuria and estimated glomerular filtration rates were investigated after colchicine treatment started in these groups. RESULTS: The mean follow-up period was 27.7 ± 19.2 months. After initiating colchicine therapy, the degree of proteinuria was decreased higher than 50% in 11/14 (78%) of non-nephrotic patients and elevated only in three (22%) patients. In nephrotic group, proteinuria was increased in 5/10 (50%) of patients. Glomerular filtration rates were stable in nephrotic and non-nephrotic groups. Presenting with nephrotic syndrome was higher in RD-associated amyloidosis (RD_A) group (5/9) than FMF-associated amyloidosis (FMF_A) group (5/15) without statistical significance (p > 0.05). After colchicine treatment, proteinuria was decreased in 12/15 patients in FMF_A group, however, the significant decreasing of proteinuria was not observed in RD_A group (p = 0.05 vs. p > 0.05). CONCLUSION: Colchicine therapy was found more effective in low proteinuric stage of amyloidosis. The beneficial effect of colchicine therapy was not observed in patients with RD- associated amyloidosis.

Massive intracerebral hemorrhage associated with Wegener granulomatosis.

Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7-11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

Behcet's disease and IgA nephropathy.

Although Behçet's disease (BD) is a kind of systemic disease, renal involvement is rare, especially IgA nephropathy (IgAN). Renal manifestations in BD range from mild urinary abnormalities to glomerulonephritis with persistent renal failure, which includes minimal change disease, proliferative glomerulonephritis, rapidly crescentic glomerulonephritis, renal amyloidosis and IgA nephropathy. Amyloidosis seems to be the most common type of renal lesion in BD, and several cases of nephrotic syndrome secondary to amyloidosis have been documented. Co-occurrence of BD and IgA nephropathy has only been reported in only few cases. We describe two patients with the rare association of BD and IgAN. We suggested that it is important to periodically perform renal function assessment in patients with BD, through urinalysis and measurement of serum creatinine for detecting any abnormality and providing an early adequate treatment.

Acute kidney injury due to rhabdomyolysis in H1N1 influenza infection.

Acute kidney injury (AKI) is rarely reported in the clinical course of H1N1 infection and this condition is strongly related with increasing of mortality risk. However, there are no sufficient data about the development of AKI due to H1N1 infections. The recent reports were documented for elevation of creatinine phosphokinase levels in the course of influenza infection, but rhabdomyolysis was rarely reported. Herein, we present a 28-year-old female patient and a 19-year-old male patient with AKI in the course of H1N1 influenza infection due to rhabdomyolysis.

A patient with Crohn's disease who presented with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

Thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS) is characterized with fever, purpura, anemia due to microangiopathic hemolysis, thrombocytopenia, kidney damage, and neurologic symptoms. The development of TTP/HUS during the course of inflammatory bowel diseases was rarely reported. However, coexistence of TTP/HUS and Crohn's disease in the same patient has not been reported previously. We herein present a case of TTP/HUS who presented with Crohn's disease. He responded to cyclosporine treatment.

Renal injury due to hepatic hydatid disease.

BACKGROUND: Many studies on renal hydatid disease have been reported in the literature, and the disease process appears to be well defined. However, renal injury without direct renal invasion remains poorly understood. The present study aims to define the frequency and the property of the renal involvement in hydatid disease. METHODS: Eighty patients older than 18 years and diagnosed with liver echinococcosis were included in the study. The echinococcosis was diagnosed by the haemagglutination test and abdominal ultrasonography. Twenty-four-hour protein excretion was measured for patients who had elevated serum creatinine levels or whose urinalyses were positive for haematuria or proteinuria. Subsequently, renal biopsy was performed, and the specimens were examined by light microscopy and immunofluorescence staining. RESULTS: Haematuria was detected in 11 patients (13.75%), and proteinuria was detected in nine patients (11.25%). Percutaneous renal biopsy was applied to nine patients who gave signed consents to undergo the test. We detected four immunoglobulin A nephritis (together with tubulointerstitial nephritis in one patient), one membranoproliferative glomerulonephritis, one immunoglobulin M nephritis together with mesangiocapillary glomerulonephritis, one membranous glomerulonephritis, one amyloidosis and one tubulointerstitial nephritis. Renal hydatid cyst was detected only in four patients (5%). CONCLUSIONS: Hydatid disease, which affects the kidney, is not rare, and we suggest that urinalysis and, if indicated, renal biopsy should be performed for hepatic hydatid disease diagnosis.

Anti-cyclic citrullinated peptides positivity rate in patients with familial Mediterranean fever.

OBJECTIVES: To investigate the prevalence and levels of anti-cyclic citrullinated peptide antibodies (anti-CCP) in patients with familial Mediterranean fever (FMF) with and without arthritis. METHODS: Eighty-three patients with FMF and 43 healthy controls were included in the study. Thirty seven FMF patients had a history of arthritis, and 46 patients did not. Serum antibodies directed to the anti-CCP were assessed with a commercial enzyme-linked immunosorbent assay (ELISA) kit. Values <20U were considered negative, between 20 and 39U low, 40-99U moderate, and >100U high positive. RESULTS: Positivity rate of anti-CCP in the whole FMF group (14.5%) was three-fold higher than the control group (4.7%). However, the difference failed to achieve a statistically significant level (p=0.09). Anti-CCP levels were 21±30.1 in patients with arthritis and 13.1±10.3 in the non arthritic group (p<0.05). Anti-CCP positivity rates were 10/37 (27%) in patients with arthritis and 2/46 (4.3%) in patients without arthritis (p<0.005). Five FMF patients with arthritis (13.5%) had moderate-high anti-CCP levels (>40U/ml). Anti-CCP levels were between 20-39U/ ml in 2FMF patients without arthritis and in 2 healthy controls. Anti-CCP positivity rate is higher in FMF patients with arthritis (27%) than healthy controls (4.7%) (p<0.005). CONCLUSIONS: Anti-CCP prevalence is higher in FMF patients with arthritis than without arthritis, and that a significant proportion of FMF patients with arthritis (13.5%) had moderate-high titers of anti-CCP. Therefore, anti-CCP antibodies may not be a reliable indicator to differentiate between FMF arthritis and rheumatoid arthritis.

Nebivolol improves renal function in patients who underwent angioplasty due to renal artery stenosis: a pilot study.

Renal artery stenosis (RAS) is a progressive disease and may lead to chronic kidney disease by deterioration of renal functions. Endothelial dysfunction is an important causative factor for kidney damage after RAS revascularization. Nebivolol, a new generation beta blocker induces endothelium-related arterial relaxation by nitric oxide (NO) and may improve endothelial dysfunction. This pilot study tested the effect of nebivolol on the glomerular filtration rate (GFR) in a series of 33 patients with severe RAS (>70%) who underwent revascularization. After revascularization, nebivolol was added to antihypertensive treatment in 17 randomly selected patients while 16 patients (control group) continued their standard treatment. Estimated glomerular filtration rate (eGFR), proteinuria as well as nitrite and nitrate levels were measured at baseline and 6 months after the revascularization procedure. Six months after revascularization, eGFR increased from 44.8 to 50.6 ml/min in the nebivolol group. In contrast, eGFR did not change in the control group. Nitrite/nitrate levels decreased to a significant extent both in the nebivolol and in the control group. Proteinuria decreased more in the nebivolol group compared to the control group. These pilot data support a full-fledged clinical trial, testing whether nebivolol may be beneficial in the post-revascularization phase in patients with RAS.

Ileocolonic involvement in Behçet's disease: endoscopic and histological evaluation.

BACKGROUND: Our aim was to document the frequency of gastrointestinal (GI) involvement of Behçet's disease (BD) at endoscopic examination. Also, we aimed to analyze whether GI lesions were correlated with other systemic components of BD. METHODS: Fifty patients with BD were enrolled in the study. Patients in whom optimal colonoscopic examination of both the colon and terminal ileum could be performed were included. An ileum biopsy was taken from all cases. Upper GI endoscopy findings, presence of genital ulcers, uveitis, dermatological lesions, pathergy test, neurological involvement and peripheral vasculitis, and laboratory findings were recorded. Thirty age- and sex-matched persons who had perfect bowel cleansing and had a macroscopically normal colon and terminal ileum at colonoscopy without any systemic diseases were enrolled as the control group. RESULTS: Nine patients and 1 case had macroscopic ileum and colon lesions, respectively. Microscopic examination of BD patients with normal colon and terminal ileum at colonoscopy revealed 61% abnormal ileum histologies. That ratio was 30% for the control group (p < 0.001). None of the patients had any lesions suggesting BD at upper GI endoscopy. There was no correlation between macro- or microscopic GI involvement and other components of BD. CONCLUSIONS: Lower GI tract, especially ileum involvement, is frequent in BD. Most of the patients have inflammation in the ileum even in the absence of macroscopic lesions.

Gastroduodenal plasmacytoma presenting with jaundice.

The duodenum and gastrointestinal tract are rarely involved by multiple myeloma, either diffusely or as a focal mass. A 59-year-old man was diagnosed with multiple myeloma and given chemotherapy. Four months later, he developed jaundice. Gastroduodenal plasmacytoma causing extrahepatic biliary obstruction was diagnosed. To the best of our knowledge, this case is the first report of gastroduodenal plasmacytoma presenting with biliary obstruction.

An unusual cause of focal segmental glomerulosclerosis: psoriasis vulgaris.

Psoriasis, being limited to the skin, is generally a chronic inflammatory disorder. Several glomerular diseases have been distinguished due to renal histological findings of psoriatic patients to date. The underlying pathogenetic mechanisms of these associations remain unclear because of the limited number of cases. We report a second case of focal segmental glomerulosclerosis in a psoriatic patient.

Familial Mediterranean fever and membranous glomerulonephritis: report of a case.

Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease characterized by recurrent attacks of fever and painful episodes of sterile polyserositis. Kidney involvement may occur as a result of secondary amyloidosis during the course of FMF. Previously, different types of glomerulopathies such as IgM and IgA nephropathy, crescentic glomerulonephritis, diffuse proliferative glomerulonephritis, minimal change disease, and membranoproliferative glomerulonephritis were rarely reported. We herein represent a first case of membranous glomerulonephritis who had complete remission with colchicine treatment in the course of familial Mediterranean fever.

Effect of Local Polyhexanide Application in Preventing Exit-Site Infection and Peritonitis: A Randomized Controlled Trial.

Topical antibiotic and antiseptic agents have been documented to reduce exit-site infection (ESI) and peritonitis in PD. The aim of this randomized controlled study was to evaluate the efficacy of polyhexanide in the prevention of ESI and peritonitis. Patients were excluded if they had active infection, > 18 years of age, ESI and peritonitis within the previous 4 weeks, received PD for less than 3-months and history of allergy to either drug. All patients were followed up until catheter removal, death, switch to dialysis, transplantation or the end of the study. ESI, tunnel infection, peritonitis, catheter removal and microorganism cause of catheter-related infection were recorded prospectively during clinic follow-up. A total of 88 patients (41 povidone-iodine group; 47 polyhexanide group) were enrolled with a total follow-up duration of 480 and 555 patient-months for povidone-iodine and alternating group, respectively. There were no significant differences in the age, sex, BMI, time of PD, rate of DM, and S. aureus carriage state. A total of 8 ESI and 25 peritonitis episodes were detected during the study. ESI and peritonitis rates tended to be lower in polyhexanide group compared with the povidone-iodine group (0.06 episodes/patient-year vs. 0.12 episodes/patient-year; 0.26 episodes/patient-year vs. 0.32 episodes/patient-year, respectively), but were not significant statistically. Moreover, catheter removal was similar in both groups (0.04 / patient-year vs. 0.05 / patient-year). Polyhexanide is efficient and safe for the prevention of ESI and peritonitis and it may be used as an alternative procedure for the care of healthy exit sites.

Polyarteritis nodosa presenting with splenic infarction, bilateral renal infarction, and hematoma.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis and may affect multiple organ systems. Due to the rupture of microaneurysms, perirenal hematomas may occur in both kidneys. Spontaneous perirenal hematoma is an infrequent complication of isolated PAN. Also, involvement of the splenic arteries is a rare occurrence in polyarteritis nodosa. Perirenal hematoma rupture is a serious clinical condition. Patients with life-threatening visceral bleeding must be treated with a selective embolization procedure or radical surgery because nonoperative management has been associated with a significant mortality rate.

Idiopathic hypoparathyroidism mimicking diffuse idiopathic skeletal hyperostosis.

Idiopathic hypoparathyroidism is an uncommon disease caused by insufficient secretion of parathyroid hormone. Idiopathic hypoparathyroidism may cause various musculoskeletal findings, including diffuse ligamentous and entheseal ossifications. Diffuse idiopathic skeletal hyperostosis is a disorder of the elderly characterized by ossification of the anterior longitudinal ligament of the spine and various extraspinal ligaments. We present a 50-year-old man with idiopathic hypoparathyroidism who had been diagnosed as having DISH at 40 years of age. Resistant neck, left shoulder, and left hip pain did not improve with calcium and calcitriol treatment after diagnosis of idiopathic hypoparathyroidism.

Vascular endothelial growth factor and carotid intima-media thickness in patients with Behçet's disease.

There is morphologic evidence of subclinical atherosclerosis in Behçet's disease (BD) patients obtained by high-resolution B-mode ultrasonography (US). Vascular endothelial growth factor (VEGF) is a potent angiogenetic factor and a marker for endothelial dysfunction. VEGF could contribute to the pathological events in BD. VEGF could also be an important factor in the progression of atherosclerosis. In this study, we investigated whether there is correlation between intima-media thickness (IMT) of the carotid arteries and serum VEGF levels in BD patients and healthy controls. Twenty-one patients with BD (male/female: 15/6, mean age: 35.8 +/- 8.6 years) were individually matched to control subjects on the basis of age (within 3 years) and sex. Carotid IMT of the subjects was measured by high-resolution B-mode US. Mean IMT values of common carotid arteries were 0.86 +/- 0.18 mm for patients with BD, and 0.57 +/- 0.14 mm for healthy controls (p < 0.001). Mean VEGF levels were 130.41 +/- 58.28 pg/ml for patients with BD and 82.69 +/- 25.03 pg/ml for healthy controls (p < 0.001). There was no correlation between VEGF levels in the control group and in the BD group, but there was a significant correlation between VEGF levels and mean carotid IMT in the whole group (r = 0.317, p < 0.05). In conclusion, elevation of VEGF appears as a feature of the inflammatory reaction during the course of BD, not a direct determinant of subclinical atherosclerosis. On the other hand, the significant correlation between carotid IMT and serum VEGF levels in the whole group suggests that association between VEGF levels and carotid IMT warrants further investigation with larger sample sizes.