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.

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.

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.

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

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.

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.

Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report.

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.

Malnutrition due to bisphosphonate-related osteonecrosis of the jaw in a chronic dialysis patient: case report.

Adequate nutrition is imperative for a successful outcome in dialysis patients. Excellent oral hygiene and an efficient mastication can help to correct several metabolic and endocrine disturbances as well as delay initiation of dialysis in patients with chronic renal failure. However, concerns exist about the risk of malnutrition and protein depletion. On the other hand, intravenous bisphosphonates are the current standard of care for the treatment of hypercalcemia of malignancy and for the prevention of skeletal complications associated with bone metastases. Recently, retrospective case studies have reported an association between long-term bisphosphonate therapy and osteonecrosis of the jaws. This complication occurs either spontaneously or after minor dento-alveolar surgery including extraction of teeth. A malnourished dialysis patient who showed the typical clinical features of bisphosphonate-related osteonecrosis of the jaw (BRONJ) without any obvious radiological changes in his panoramic radiograph is reported. To minimize the risk of BRONJ, patients initiated on bisphosphonates should optimize routine dental care and have their baseline oral health evaluated by both clinical and radiographic examinations before initiation of bisphosphonate therapy.

Renal Behçet's disease: an update.

OBJECTIVE: The aims of this study are (1) to report 33 patients with Behçet's disease (BD) having various renal manifestations, and (2) to update current data using our patients and published papers about BD and renal manifestations. METHODS: The PubMed database was searched using the terms BD or Behçet's syndrome. We found reports of 94 patients (including ours) with BD and specific renal diseases (amyloidosis, 39; glomerulonephritis [GN], 37; renal vascular disease, 19; interstitial nephritis, 1). RESULTS: The presentation of renal disease was edema/nephrotic syndrome in 12 patients (36%). Renal disease was incidentally diagnosed by routine urine analysis and measurement of serum creatinine level in 20 patients (61%). Renal failure was present in 23 patients (70%) and 5 of them have had cyclosporine treatment. The frequency of renal disease among BD patients has been reported to vary from less than 1 to 29%. CONCLUSIONS: The clinical spectrum of renal BD shows a wide variation. Amyloidosis (AA type), GN, and macroscopic/microscopic vascular disease are the main causes of renal BD. Patients with vascular involvement have a high risk of amyloidosis and amyloidosis is the most common cause of renal failure in BD. Several types of glomerular lesions are seen in BD. Current treatment options for renal BD are not evidence based. Radiological vascular intervention combined with immunosuppressive drugs can be useful in selected cases. Routine urine analysis and measurement of serum creatinine level are needed for early diagnosis of renal BD.

Trimetazidine in the prevention of contrast-induced nephropathy after coronary procedures.

OBJECTIVE: To evaluate the efficacy of trimetazidine (TMZ) in the prevention of contrast-induced nephropathy (CIN) in patients with high serum creatinine levels undergoing coronary angiography/angioplasty. METHODS: TMZ (20 mg thrice daily) was administered orally for 72 h starting 48 h before the procedure. All patients were given intravenous saline (0.9%) at a rate of 1 ml/kg of body weight per hour for 24 h starting 12 h beforehand. Serum creatinine levels were measured before the procedure, 48 h and 7 days after the procedure. Increase in serum creatinine level exceeding 0.5 mg/day or one quarter of the basal value is considered as CIN. Venous blood samples for serum total antioxidant capacity (TAC) measurement were drawn before and after coronary angiography. RESULTS: Basal serum creatinine levels and TAC were similar in TMZ and control groups. Serum creatinine levels in the control group increased significantly 2 days after the procedure, and returned to the baseline values on the seventh day. However, it did not change significantly on the second day, and even significantly decreased on the seventh day in the TMZ group. CIN developed in 2.5% (1/40) of patients in the TMZ group and in 16.6% (7/42) of patients in the control group (p<0.05). TAC values were not different between treatment groups. CONCLUSION: TMZ along with isotonic saline infusion is more effective than isotonic saline alone in reducing the risk of CIN in patients with pre-existing renal dysfunction.

Quantitative serum free light chain assay in the diagnostic evaluation of AL amyloidosis.

We compared a new serum immunoassay for quantitation of serum free light chains (FLC) with the conventional tests for clonal immunoglobulin production: bone marrow immunohistochemistry, serum immunofixation electrophoresis, and urine immunofixation electrophoresis. Serum samples from 169 patients with AL amyloidosis and 20 controls were examined. Elevated levels of kappa-FLC and lambda-FLC were found in 94% and 93% of patients with the respective clonal disease. However, false positive elevations of kappa-FLC and lambda-FLC were found in 30% and 44% of patients with clonal disease of the other light chain subtype. We found that the FLC level was a reliable test for the diagnosis of clonal disease when the FLC kappa:lambda ratio was abnormal and was comparable to the conventional tests in patients with AL amyloidosis. After a histologic tissue diagnosis of amyloidosis, determining the type as AL amyloidosis relies on a panel of hematologic tests to determine light chain clonality and the exclusion other forms of amyloidosis.

Efficacy and tolerability of amifostine in elderly cancer patients.

BACKGROUND: Amifostine is a cytoprotective agent used to prevent cisplatin nephrotoxicity. It is associated with dose-limiting acute toxicities of emetic symptoms (nausea and vomiting) and transient hypotension. OBJECTIVE: The aim of this study was to analyze the efficacy and tolerability of amifostine in elderly cancer patients. METHODS: This 18-month, prospective, comparative study was conducted at the Department of Internal Medicine, Adnan Menderes University Hospital (Aydin, Turkey). Adult (aged 40-<85 years) hospitalized patients with advanced-stage cancer without comorbid diseases were enrolled. Patients were divided into 2 groups: age <70 years (group 1) and ≥70 years (group 2). All patients were treated with amifostine + cisplatin-based chemotherapy (CT). Amifostine 910 mg/m(2) (maximum, 1500 mg) was administered as a 15-minute IV infusion. Clinical systolic and diastolic blood pressures (SBP and DBP, respectively) were measured at 0 minute (baseline), at 8 and 15 minutes of amifostine infusion, and at 30 minutes after the start of amifostine infusion. In addition to physical examination, chest radiography, electrocardiography, blood chemistry (including serum electrolytes and renal function tests), complete blood count, and complete urinalyses were performed before each CT administration and at the post-CT day of toxicity assessment. RESULTS: Thirty-five consecutive patients were enrolled (22 men, 13 women; mean [SD] age, 61 [12] years; group 1, n = 22; group 2, n = 13). Patients received a total of 153 CT cycles (median, 4 cycles/patient; group 1, 96 cycles; group 2, 57 cycles). Amifostine caused significant SBP and DBP reductions at 8 minutes of infusion compared with baseline in groups 1 (both P < 0.001) and 2 (P = 0.002 and P = 0.006, respectively). Overall, 20 patients (57.1%) experienced ≥ 1 symptomatic hypotensive episode; these rates were not significantly different between groups 1 (11 cases, 50.0%) and 2 (9 cases, 69.2%). Amifostine infusion was interrupted a similar number of times (6 times in group 1 and 4 times in group 2 [6.3% and 7.0% of administrations, respectively]) due to hypotension, but could be restarted in all. At 15 minutes, mean SBP and DBP values were not significantly different from baseline in either group. The mean baseline SBP values were similar between groups at baseline, and, overall, the differences in mean SBP and DBP values were not significant between groups at any time point. All other toxicities were comparable, and serum creatinine concentrations did not change significantly from baseline with CT in either group. CONCLUSIONS: In this study of the efficacy and tolerability of amifostine in elderly patients with advanced-stage cancer without comorbid diseases, amifostine was effective in reducing cisplatin-induced nephrotoxicity, with transient systolic and diastolic hypotension being the most prominent adverse effect. All other toxicities were either low grade or preventable. No significant differences in amifostine tolerability or toxicities were observed between the study groups.

MPO-ANCA-associated small vessel vasculitis presenting as fever of unknown origin. Report of one case.

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA) associated small vessel vasculitis which can present with various clinical manifestations, for which the mainstay of treatment is systemic corticosteroids and immunosuppressants. We report a case of a 54-year-old female admitted to the hospital because of fever during the last month, leukocytosis and elevated erythrocyte sedimentation rate. Persistence of elevated serum creatinine levels and accompanying hematuria led us to perform a renal biopsy, and MPA was diagnosed on the basis of light and immunofluorescence microscopy. Remission was induced with oral corticosteroids and cyclophosphamide therapy in conjunction with plasmapheresis (PF). The objective of this report was to assess the role of PF in the treatment of MPA and report on its utility in patients with MPA who are not responding to standard therapy or who require unacceptably high doses of steroids or immunosuppressants. In a patient presenting with fever of unknown origin, microscopic polyangiitis should also be considered in the differential diagnosis.

Nephrotic syndrome after allogeneic peripheral blood stem cell transplantation.

Nephrotic syndrome has been rarely reported after hematopoietic stem cell transplantation. We report a patient who developed nephrotic syndrome after allogeneic peripheral blood stem cell transplantation for acute myelogenous leukemia. Renal biopsy was performed and immunofluorescence and light microscopy were compatible with minimal change disease. The patient was treated with cyclophosphamide and prednisolone. Complete remission was achieved after three months. Previous reported cases are discussed.