Experience with cascade screening: A comprehensive family pedigree analysis of two index patients with Fabry disease.

The wide range of clinical symptoms observed in patients with Fabry disease (FD) often leads to delays in diagnosis and initiation of treatment. Delayed initiation of therapy may result in end-organ damage, such as chronic renal failure, hypertrophic cardiomyopathy, and stroke. Although some tools are available to identify undiagnosed patients, new comprehensive screening methods are needed. In this study, the outcomes of the cascade screening applied to three index cases with FD from 2 familes were investigated. In the pedigree analysis, 280 individuals were included; out of them, 131 individuals underwent genetic testing and cascade screening for FD. During the screening program, a total of 45 individuals were diagnosed, with a diagnostic ratio of 1:15. The average age at diagnosis for all individuals was 30.9 ± 17.7 years, and %25 were pediatric cases (mean age 9.5 ± 5.9 years). Thirty affected relatives were diagnosed from the two index cases in Family 1 and 15 individuals were diagnosed from one index case in Family 2. There were 13 consanguineous marriages observed among 2 pedigres, in two both spouses  were affected, leading to two homozygous affected daughters in one couple. In regions where there is a high prevalence of consanguineous marriages, implementing the cascade screening approach to identify all individuals at risk can be beneficial for patients with FD, specifically women and children.

Evaluation of the ischemia modified albumin levels in familial Mediterranean fever patients.

BACKGROUND: Familial Mediterranean fever (FMF) is an autoimmune disease with periodic fever attacks recurring with mutations in the MEFV gene and chronic inflammation. The new molecule which is formed as a result of the chemical changes made by oxidative free radicals in the albumin molecule during ischemic events is called ischemia modified albumin (IMA). AIM: The aim of this study is to evaluate the IMA levels as a predictor of the cardiovascular risk factor in FMF patients in the attack-free period and to evaluate the relationship between IMA and inflammation markers. METHODS: Forty FMF patients without any additional disease, non-smokers, and in their attacks-free period, 40 ankylosing spondylitis patients whose disease activity criteria is less than 4 from Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and 39 healthy adults were included in the cross-sectional analytical research. RESULTS: The value of IMA was statistically significantly higher in the AS group compared to the control group (p = 0.01). The positive correlations between IMA and ESR, LDL, total cholesterol, triglyceride, CRP, and fibrinogen were statistically significantly determined in FMF patients (respectively; r = 0.594; p < 0.001, r = 0.382; p = 0.015, r = 0.335; p = 0.034, r = 0.363; p = 0.021, r = 0.597; p < 0.001, r = 0.656; p < 0.001). The positive correlations between IMA and ESR, CRP were found in AS patients (respectively; r = 0.383; p = 0.015, r = 0.382; p = 0.015). CONCLUSION: IMA can be used as a predictor similar to cardiovascular risk factors and it is a precious marker for inflammation. The use of IMA in these fields and the multi-centred and comparative studies about predictability of it may contribute to science.

Which is the best choice for Helicobacter pylori eradication? Dual therapy or quadruple therapy?

Introduction: Standard triple therapy used to be the first-line treatment for Helicobacter pylori (Hp) infection, but today it is a treatment regimen with a low eradication rate due to increasing resistance to the antibiotics included in the triple therapy.Aim: To compare the eradication rates of dual treatment regimens and quadruple treatment regimens. Material and methods: Patients over 18 years of age, who were indicated to undergo upper gastrointestinal system (GIS) endoscopy for any reason, had their upper gastrointestinal endoscopy performed, were detected to have Hp as a result of the histopathological evaluation of the biopsy material, and in whom eradication control was also performed by histopathological evaluation, were included in the study. These patients were divided into 4 groups, each containing 50 people. Results: Considering the eradication rates of Hp-positive patients according to different treatment options, 78% (n = 39) of the patients in Group 1 were eradicated after the treatment while 66% (n = 33) of the patients in Group 3, 58% of the patients in Group 2 (n = 29), and 58% (n = 29) of the patients in Group 4 had Hp negative results after treatment. When high-dose dual treatments were compared, the highest eradication rate was obtained with rabeprazole, but no statistically significant difference was detected (p = 0.11). Conclusions: This is the first study to include dual therapies consisting of 3 different PPIs at the same time. The low eradication rates obtained in our study suggest that the determination of individualized treatment strategies in which CYP2C19 polymorphism is detected may result in higher eradication rates.

Can Ischemia Modified Albumin (IMA) Levels Be a Predictor of Acute Pancreatitis?

BACKGROUND: We aimed to evaluate the value of ischemia modified albumin (IMA) as a prognostic marker in acute pancreatitis (AP) patients, determine whether it is efficient in assessing the disease severity or not, and to estimate the correlation between IMA and the inflammatory markers, prognostic markers and scoring systems routinely used in clinical practice. METHODS: 100 adult patients (18 years and older) who have been hospitalized and evaluated with AP diagnosis in Tepecik Training and Research Hospital,, Department of Gastroenterology, between April 1, 2017 and April 1, 2018 have been enrolled in the study. Patients have been stratified disease etiology (biliary or non-biliary). The non-biliary group has been divided into subgroups as alcoholic, lipemic, or idiopathic. Disease severity has been categorized as mild, moderate, or severe pancreatitis according to the Atlanta classification. Ranson, Harmless Acute Pancreatitis Score (HAPS), Bedside Severity Index for Acute Pancreatitis (BISAP) scores have been determined for each patient. Patients have been grouped as necrotizing or edematous according to the Atlanta classification. RESULTS: According to our findings, IMA has been found to be correlated with disease severity, Ranson and BISAP scores, and procalcitonin levels. We have observed that some laboratory parameters including blood urea nitrogen and hematocrit levels and HAPS scoring system are not correlated to IMA. CONCLUSION: Our study is the first study to compare multiple prognostic factors with IMA in AP patients. In our study, the association between IMA and AP has been evaluated in the context of prognostic scoring and disease severity.

Serum iron and vitamin B 12 deficiency could indicate celiac disease by flexible spectral imaging color enhancement.

INTRODUCTION Celiac disease (CeD) is an autoimmune disease that can be delayed in diagnosis due to the presence of atypical and asymptomatic cases in adulthood. Herein we aimed to study the frequency of CeD and evaluate whether magnified endoscopy and magnified/FICE (flexible spectral imaging color enhancement) techniques contribute to the diagnosis in patients with serum iron and vitamin B12 deficiency. METHODS We evaluated 50 adult patients (10 males and 40 females) who had serum iron and vitamin B12 deficiency, prospectively. All the patients had undergone upper gastrointestinal system endoscopy by the same endoscopist. The second part of the duodenum was evaluated with white light, magnified, and magnified/FICE endoscopy. Biopsy specimens were evaluated by the same pathologist. The specimens diagnosed as CeD were classified according to the Modified Marsh-Oberhuber criteria. RESULTS 10 of 50 patients (20%) were diagnosed as CeD. The average age was 41±11 years (20-67 years). Thirty percent of CeD diagnosed patients had typical CeD symptoms. Six of 10 patients (60%) who were diagnosed as CeD had typical endoscopic images under white lighted endoscopy. All of these 10 patients (100%) showed villous irregularity, partial villous atrophy, or total villous atrophy consistent with CeD with magnified and magnified/FICE endoscopy. CONCLUSION The practical use of magnified/FICE endoscopy allows us to differentiate mucosal abnormalities of the duodenum and minimize false-negative results that indicate normal mucosal findings with conventional endoscopy.

Serum iron and vitamin B 12 deficiency could indicate celiac disease by flexible spectral imaging color enhancement

SUMMARY INTRODUCTION Celiac disease (CeD) is an autoimmune disease that can be delayed in diagnosis due to the presence of atypical and asymptomatic cases in adulthood. Herein we aimed to study the frequency of CeD and evaluate whether magnified endoscopy and magnified/FICE (flexible spectral imaging color enhancement) techniques contribute to the diagnosis in patients with serum iron and vitamin B12 deficiency. METHODS We evaluated 50 adult patients (10 males and 40 females) who had serum iron and vitamin B12 deficiency, prospectively. All the patients had undergone upper gastrointestinal system endoscopy by the same endoscopist. The second part of the duodenum was evaluated with white light, magnified, and magnified/FICE endoscopy. Biopsy specimens were evaluated by the same pathologist. The specimens diagnosed as CeD were classified according to the Modified Marsh-Oberhuber criteria. RESULTS 10 of 50 patients (20%) were diagnosed as CeD. The average age was 41±11 years (20-67 years). Thirty percent of CeD diagnosed patients had typical CeD symptoms. Six of 10 patients (60%) who were diagnosed as CeD had typical endoscopic images under white lighted endoscopy. All of these 10 patients (100%) showed villous irregularity, partial villous atrophy, or total villous atrophy consistent with CeD with magnified and magnified/FICE endoscopy. CONCLUSION The practical use of magnified/FICE endoscopy allows us to differentiate mucosal abnormalities of the duodenum and minimize false-negative results that indicate normal mucosal findings with conventional endoscopy.

RESUMO INTRODUÇÃO A doença celíaca (DC) é uma doença autoimune que pode ter seu diagnóstico atrasado devido à presença de casos atípicos e assintomáticos na idade adulta. Neste trabalho, objetivamos estudar a frequência de DC e avaliar se as técnicas de endoscopia magnificada e magnificada/Fice (flexible spectral imaging color enhancement) contribuem para o diagnóstico em pacientes com deficiência sérica de ferro e vitamina B12. MÉTODO Foram avaliados prospectivamente 50 pacientes adultos (10 homens e 40 mulheres) com deficiência sérica de ferro e vitamina B12. Todos os pacientes foram submetidos a endoscopia digestiva alta pelo mesmo endoscopista. A segunda parte do duodeno foi avaliada com endoscopia com luz branca, magnificada e magnificada/Fice. As amostras de biópsia foram avaliadas pelo mesmo patologista. Os espécimes diagnosticados como DC foram classificados de acordo com os critérios de Marsh-Oberhuber modificado. RESULTADOS Dez dos 50 pacientes (% 20) foram diagnosticados como DC. A idade média foi de 41±11 anos (20-67 anos). Trinta por cento dos pacientes diagnosticados com DC apresentaram sintomas típicos de DC. Seis dos dez pacientes (60%) diagnosticados com DC tinham imagens endoscópicas típicas sob endoscopia de luz branca. Todos esses dez pacientes (% 100) apresentaram irregularidade das vilosidades, atrofia das vilosidades parciais ou atrofia das vilosidades totais consistentes com a DC com endoscopia magnificada e magnificada/Fice. CONCLUSÃO O uso prático da endoscopia magnificada/Fice permite diferenciar anormalidades mucosas do duodeno e minimizar os resultados falso-negativos que apresentam achados mucosais normais com a endoscopia convencional.

Evaluation of fibrosis in chronic hepatitis C patients treated with direct-acting antivirals.

Background and Aim: This study is an evaluation of liver fibrosis measurements determined using transient elastography and aspartate aminotransferase-to-platelet ratio index (APRI) scores of patients diagnosed with chronic hepatitis C (CHC) who were treated with direct-acting antivirals (DAAs). Materials and Methods: The liver fibrosis measurements recorded using transient elastography, APRI scores, and the biochemical data from before and after treatment of 40 patients with CHC who were treated with DAA were reviewed. Patients who received paritaprevir+ritonavir/ombitasvir+dasabuvir were included in Group 1 (n=20), and patients who received sofosbuvir+ledipasvir±ribavirin in Group 2 (n=20). Results: The mean liver fibrosis measurement of the patients was 15.73±10.63 kPa (min-max: 5.20-45.00 kPa) before treatment and 2.56±8.84 kPa (min-max: 4.30-42.00 kPa) after treatment. There was a significant improvement in liver fibrosis with a regression of 20.16% at the end of treatment compared with the start (p=0.001) with no significant difference between treatment groups (p=0.542). The highest regression rate of 75% was seen in patients with F2 fibrosis at the end of treatment. Significant regression was also found in patients with F3 fibrosis, with a rate of 57.2%, and in those with F4 fibrosis, with a rate of 17.6% (p=0.035). Significant reduction was also observed in the APRI scores of patients at the end of treatment compared with the start of treatment (p<0.001), with no significant difference between treatment groups (p=0.328). Conclusion: Noninvasive assessments of CHC patients treated with DAA revealed regression in liver fibrosis measurements and APRI scores and significant improvements were seen in the stage of fibrosis in the early phases following treatment.

The relationship between malnutrition and quality of life in haemodialysis and peritoneal dialysis patients

SUMMARY BACKGROUND One of the most important factors affecting the quality of life of chronic kidney disease (CKD) patients is nutrition. Prevention of malnutrition increases patients' quality and length of life. In this study, we aimed to determine the frequency of malnutrition, quality of life, and the relationship between them in patients with end-stage renal disease (ESRD). METHOD The study was conducted with a total of 60 CKD patients including 50 haemodialysis patients and 10 peritoneal dialysis patients. Patients' data associated with socio-demographics, body mass index (BMI), waist circumference, triceps skin-fold thickness (TSFT), pre-dialysis systolic and diastolic blood pressure, Kt/V and urea reduction ratio (URR) values, laboratory parameters, Mini-Nutritional Assessment-Short Form (MNA-SF) and European Quality of Life 5-Dimensions (EQ5D) scale were recorded. FINDINGS Of the total 60 patients; 27 were male (45%), 33 were female (55%), 83.3% were receiving haemodialysis treatment (HD), and 16.7% were receiving peritoneal dialysis treatment (PD). The mean MNA-SF score was 10.4 ± 2.8 in the HD group and 10.5 ± 2.9 in the PD group; there was no difference between the scores of the HD and PD groups. The mean EQ5D score was 0.60 ± 0.29 in the HD group and 0.68 ± 0.33 in the PD group, no significant difference was found between the HD group and the PD group. The quality of life was found lower in malnourished group (p=0.001). CONCLUSION The quality of life needs to be increased by early diagnosis and treatment of malnutrition in patients at risk.

RESUMO INTRODUÇÃO O estado nutricional é um dos principais determinantes da qualidade de vida de pacientes com doença renal crônica (DRC) e a prevenção da desnutrição aumenta o tempo e a qualidade de vida nessa população. O objetivo do presente estudo foi determinar a prevalência de desnutrição, a qualidade de vida e a inter-relação entre esses fatores em pacientes com DRC em terapia dialítica. MÉTODOS Incluímos 60 pacientes com DRC estágio 5 sob terapia dialítica (50 pacientes em hemodiálise [HD] e 10 em diálise peritoneal [DP]). Os pacientes foram analisados com relação aos seus dados sociodemográficos, índice de massa corporal (IMC), circunferência abdominal, dobra cutânea triciptal, pressão arterial sistólica e diastólica pré-diálise, Kt/V e índice de remoção de ureia, parâmetros laboratoriais, miniavaliação nutricional (MNA) e questionário EuroQol-5 Dimensions (EQ-5D). RESULTADOS Do total de pacientes, havia 27 homens (45%) e 33 mulheres (55%), 83,3% em HD e 16,7% em DP. O MNA médio foi 10,4 ± 2,8 nos pacientes em HD e 10,5 ± 2,9 naqueles em DP, não havendo diferença significativa entre os grupos. O EQ-5D médio foi 0,60 ± 0,29 nos pacientes em HD e 0,68 ± 0,33 naqueles em DP, não havendo diferença estatisticamente significativa entre os grupos. A qualidade de vida foi pior nos pacientes desnutridos (p=0,001). CONCLUSÃO O diagnóstico e o tratamento precoce da desnutrição são necessários para melhorar a qualidade de vida dessa população.

The effect of hemodialysis on the body composition and cardiovascular disease markers in recently diagnosed end stage renal disease patients.

AIM: Uremic toxins and excess fluid contributes to increased cardiovascular (CV) risk. We aimed to determine the body fluid status in patients who are just starting hemodialysis (HD) and to determine the effects of excess fluid removed by HD on the CV system. METHODS: A total of 52 patients with chronic kidney disease (CKD) who had just started HD were included. Before the HD, the left atrial diameter was measured, the volumes were calculated, the pulse wave velocity (PWV) and the augmentation index (AIx) were measured, the bioimpedance analysis (BIA) was performed, the blood was taken for brain natriuretic peptide (BNP). When patients reached their dry weight with HD, the same measurements were repeated. RESULTS: Measurements were made to determine the volume status, and all parameters except the fat tissue index decreased significantly after HD. With the removal of fluid by HD, there was an average weight reduction of 4.38 kilograms. Positive correlations between PWV and age and cardiothoracic ratio (CTR) before HD were determined. Negative correlations were found between PWV and lean tissue mass (LTM) and intracellular water (ICW) before HD. At the end of the last HD, PWV was positively correlated with age, CTR, central pulse pressure Correlation between pulse wave velocity and LTI was negative. CONCLUSIONS: HD significantly improves PWV in patients reaching dry weight. Reduction of fluid excess by ultrafiltration in HD patients may reduce CV mortality by reducing arterial stiffness.

The effect of hemodialysis on the body composition and cardiovascular disease markers in recently diagnosed end stage renal disease patients

SUMMARY AIM: Uremic toxins and excess fluid contributes to increased cardiovascular (CV) risk. We aimed to determine the body fluid status in patients who are just starting hemodialysis (HD) and to determine the effects of excess fluid removed by HD on the CV system. METHODS: A total of 52 patients with chronic kidney disease (CKD) who had just started HD were included. Before the HD, the left atrial diameter was measured, the volumes were calculated, the pulse wave velocity (PWV) and the augmentation index (AIx) were measured, the bioimpedance analysis (BIA) was performed, the blood was taken for brain natriuretic peptide (BNP). When patients reached their dry weight with HD, the same measurements were repeated. RESULTS: Measurements were made to determine the volume status, and all parameters except the fat tissue index decreased significantly after HD. With the removal of fluid by HD, there was an average weight reduction of 4.38 kilograms. Positive correlations between PWV and age and cardiothoracic ratio (CTR) before HD were determined. Negative correlations were found between PWV and lean tissue mass (LTM) and intracellular water (ICW) before HD. At the end of the last HD, PWV was positively correlated with age, CTR, central pulse pressure Correlation between pulse wave velocity and LTI was negative CONCLUSIONS: HD significantly improves PWV in patients reaching dry weight. Reduction of fluid excess by ultrafiltration in HD patients may reduce CV mortality by reducing arterial stiffness.

RESUMO INTRODUÇÃO: Em pacientes com doença renal crônica (DRC), toxinas urêmicas e hipervolemia contribuem para aumentar o risco cardiovascular. Nosso objetivo foi determinar o estado de hidratação em pacientes com DRC iniciando hemodiálise (HD) e avaliar os efeitos da correção da hipervolemia sobre o sistema cardiovascular. MÉTODOS: Foram incluídos 52 pacientes que haviam acabado de iniciar HD. Antes do início da sessão, foram determinados o diâmetro e o volume atrial esquerdo, a velocidade de onda de pulso (VOP) e o índice de amplificação sistólica ("augmentation index", AI). Além disso, realizamos análise da composição corporal por bioimpedância elétrica (BIA) e mensuramos os níveis plasmáticos de peptídeo natriurético tipo B. Os mesmos procedimentos foram repetidos após os pacientes alcançarem o "peso seco". RESULTADOS: O peso corporal dos pacientes foi reduzido, em média, em 4,38 kg. Na BIA, todos os parâmetros, exceto o índice de gordura corporal, foram significativamente reduzidos após a hemodiálise. Antes da HD, a VOP se correlacionou positivamente com idade e razão cardiotorácica (RCT), e negativamente com a massa magra e a água intracelular. Ao final da hemodiálise, a VOP se correlacionou positivamente com idade, RCTe pressão de pulso central, correlacionando-se negativamente com a Lean Tissue Index (LTI). CONCLUSÃO: A hemodiálise melhora a VOP por meio da redução da volemia. O controle da hipervolemia via ultrafiltração pode reduzir a mortalidade cardiovascular por meio da redução da rigidez arterial.

The relationship between malnutrition and quality of life in haemodialysis and peritoneal dialysis patients.

BACKGROUND: One of the most important factors affecting the quality of life of chronic kidney disease (CKD) patients is nutrition. Prevention of malnutrition increases patients' quality and length of life. In this study, we aimed to determine the frequency of malnutrition, quality of life, and the relationship between them in patients with end-stage renal disease (ESRD). METHOD: The study was conducted with a total of 60 CKD patients including 50 haemodialysis patients and 10 peritoneal dialysis patients. Patients' data associated with socio-demographics, body mass index (BMI), waist circumference, triceps skin-fold thickness (TSFT), pre-dialysis systolic and diastolic blood pressure, Kt/V and urea reduction ratio (URR) values, laboratory parameters, Mini-Nutritional Assessment-Short Form (MNA-SF) and European Quality of Life 5-Dimensions (EQ5D) scale were recorded. FINDINGS: Of the total 60 patients; 27 were male (45%), 33 were female (55%), 83.3% were receiving haemodialysis treatment (HD), and 16.7% were receiving peritoneal dialysis treatment (PD). The mean MNA-SF score was 10.4 ± 2.8 in the HD group and 10.5 ± 2.9 in the PD group; there was no difference between the scores of the HD and PD groups. The mean EQ5D score was 0.60 ± 0.29 in the HD group and 0.68 ± 0.33 in the PD group, no significant difference was found between the HD group and the PD group. The quality of life was found lower in malnourished group (p=0.001). CONCLUSION: The quality of life needs to be increased by early diagnosis and treatment of malnutrition in patients at risk.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

Fludarabine Treatment of Patient with Chronic Lymphocytic Leukemia Induces a Digital Ischemia.

We report a 63-year-old man with a history of chronic lymphocytic leukemia (CLL) who presented with asymmetrical Raynaud's phenomenon of sudden onset which progressed to acral gangrene rapidly in a week. These symptoms began approximately one week after the fourth cycle of fludarabine and cyclophosphamide chemotherapy and were accompanied by pain, numbness, and cyanosis in the fingers of his right hand except the first finger. Fludarabine may play a role in acral vascular syndrome. The treatment with fludarabine in patients with evolving digital ischemia should be carried out with caution.

Exogenously administered adenosine attenuates renal damage in streptozotocin-induced diabetic rats.

BACKGROUND: Diabetic nephropathy (DNP) is one of the most serious complications of diabetes mellitus (DM). In the present study, we investigated the potential of adenosine as a therapeutic candidate for preventing DNP. METHODS: Twenty-one adult male rats were included in the study. Fourteen rats were administered a single dose of 60 mg/kg streptozotocin (STZ) to induce diabetes. Seven rats served as normal control group. Diabetic rats were randomly divided into two groups: one group was treated with 1 mL/kg saline/day (DM + saline) and the other group was treated with 5 mg/kg/day adenosine (DM + adenosine) for 6 weeks. After 6 weeks, biochemical parameters including urea, creatinine, blood urea nitrogen (BUN), kidney injury molecule-1 (KIM-1) and tumor necrosis factor-α (TNF-α) were measured in plasma samples. Also, kidneys were removed for histopathological assessment. RESULTS: Both of plasma KIM-1 and TNF-α levels were significantly higher in DM + saline group compared to controls. However, treatment of diabetic rats with adenosine significantly decreased the plasma KIM-1 and TNF-α levels compared to DM + saline group. Significant histopathological changes were observed in diabetic rats whereas adenosine treatment effectively prevented these changes. CONCLUSIONS: The findings of the present study suggest that adenosine may be a useful therapeutic agent for preventing DNP.

Acute kidney injury after near drowning: The way from the beach to hemodialysis.

Acute kidney injury (AKI) occurs in many different situations and may have a variable prognosis influenced by clinical setting, underlying cause, and comorbidity. This is important because of the high mortality and morbidity risk affecting many people around the world. Near-drowning related AKI requiring hemodialysis is very seldom reported in literature. Although cardiovascular and respiratory disorders are more frequently seen after this entity, we aimed to emphasize this rare but dangerous complication in near-drowning patients.

The effects of single hemodialysis session on arterial stiffness in hemodialysis patients.

Increased arterial stiffness in hemodialysis patients is a strong predictor of cardiovascular morbidity and mortality. Pulse wave velocity (PWV) and augmentation index (AIx), which are markers of arterial stiffness, were used to determine the severity of vascular damage noninvasively. This study aimed to investigate the effects of solute volume removal and hemodynamic changes on PWV and AIx of a single hemodialysis session. Thirty hemodialysis patients were enrolled in the study. Before initiation of hemodialysis, every 15 minutes during hemodialysis, and 30 minutes after the completion of the session, measurements of PWV and AIx@75 (normalized with heart rate 75 bpm) were obtained from each patient. Body composition was analyzed by bioimpedance spectroscopy device before and 30 minutes after completion of the hemodialysis session. During the hemodialysis, no significant change was observed in AIx@75. However, PWV decreased steadily during the session reaching statistically significant level at 135th minute (P = 0.026), with a maximal drop at 210th minute (P < 0.001). At 210th minute, decrease in PWV correlated positively with the decrease in central systolic blood pressure, central diastolic blood pressure, central pulse pressure, augmentation pressure, and AIx@75. Multiple regression analysis showed that decrease in PWV at 210th minute was associated with decrease in central systolic blood pressure and central pulse pressure. Ultrafiltration during hemodialysis had no significant effect on PWV and AIx@75. Delta urea correlated positively with delta PWV at 240th minute. A significant decrease in PWV was observed during hemodialysis and correlated with urea reduction; however, we were unable to document any effect of volume removal on arterial stiffness.

Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report / Complicación de la poliarteritis nodosa por síndrome de encefalopatía posterior reversible. Caso clínico

La hipertensión es uno de los principales problemas de salud pública, que afecta a muchas personas en todo el mundo. Se sabe que es un importante factor de riesgo para el desarrollo de enfermedades cerebrovasculares y cardiovasculares. Su clasificación como «primaria» o «secundaria» depende del proceso subyacente. En el 5-10% de los pacientes hipertensos, se trata de un problema «secundario» a otro proceso de creciente frecuencia en los centros de atención terciaria. Las causas más frecuentes de la hipertensión secundaria son: enfermedades del parénquima renal, estenosis de la arteria renal, hiperaldosteronismo primario, feocromocitoma y el síndrome de Cushing. La poliarteritis nodosa puede afectar a cualquier órgano y en diferentes grados. A continuación presentamos a un paciente joven hipertenso al que se le ha diagnosticado poliarteritis nodosa, cuya angiografía muestra múltiples microaneurismas que afectan al tronco celíaco, a la arteria renal y a la arteria mesentérica superior, asociada a un síndrome de encefalopatía posterior reversible de entidad neurológica poco visto (AU)

Hypertension (HT) represents a major public health problem affecting many individuals worldwide. It is well known to be an important risk factor for the development of cerebrovascular and cardiovascular diseases. Classifying hypertension as ‘primary’ or ‘secondary’ depends on the underlying mechanism. In 5 to 10% of hypertensive patients, HT develops ‘secondary’ to a separate mechanism that has been encountered with increasing frequency in the tertiary refferral centers. The frequent causes of secondary hypertension include renal parenchymal disease, renal artery stenosis, primary hyperaldosteronism, phaeochromocytoma and Cushing's syndrome. Polyarteritis nodosa (PAN) can involve any organ and in varying degrees. Here we present a young hypertensive patient diagnosed as PAN with the angiographic findings of multiple microaneurysms involving celiac, renal and superior mesenteric arteries and associated with a rarely seen neurological entity-PRES syndrome (AU)