Kidney transplantation for end-stage renal disease secondary to familial Mediterranean fever.

Although kidney transplantation (KT) is widely used for treating renal amyloidosis secondary to familial Mediterranean fever (FMF), data concerning transplant outcome are limited and inconsistent. The aim of this study was to determine the long-term outcome of KT in patients with amyloidosis secondary to FMF. Kidney transplantation outcome in 24 patients with FMF was compared to that in 72 controls matched for age, gender of recipient, and type of the donor that underwent KT due to end-stage renal disease (ESRD) not caused by FMF. Mean follow-up time was 80.3 ± 55.1 months in the FMF group, vs. 86.5 ± 47.6 months in the control group. Death-censored graft survival at five and 10 yr in the FMF group was 95.8% and 78.4%, respectively, and was comparable to that in the control group. In the FMF group, five- and 10-yr patient survival (87.5 and 65.6%) was shorter than in the control group, but the difference was not statistically significant. The findings show that long-term outcome of KT in the patients with amyloidosis secondary to FMF was comparable to that in patients with ESRD not caused by FMF. Recurrence of amyloidosis in the allograft, gastrointestinal intolerance, and fatal infections remain as major complications during the post-transplant period.

Congenital megacalycosis with IgA nephropathy: a case report and review of the literature.

Congenital megacalycosis is a rare renal disease characterized by calyceal dilatation without pelvic or ureteral obstruction. If not accompanied by nephrolithiasis and urinary tract infection, this disease is completely benign and does not cause renal dysfunction. We present a case of congenital megacalycosis that was diagnosed at the age of 41 (oldest case in the literature) after admitting with hematuria and acute renal dysfunction. IgA nephropathy was also diagnosed in this patient. Since renal dysfunction is not likely in these patients, if encountered; renal biopsy should be performed although technically difficult to diagnose the cause of this dysfunction.

Elevated urinary angiotensinogen a marker of intrarenal renin angiotensin system in hypertensive renal transplant recipients: does it play a role in development of proteinuria in hypertensive renal transplant patients?

The aim of this study was to evaluate the relationship of local intrarenal renin angiotensin system (RAS) with hypertension and proteinuria in renal transplant recipients. Sixty-nine nondiabetic renal transplant recipients (39 male, mean age: 36.3 ± 11.5 years) were included in this study. All patients were in stable condition with GFR greater than 30 ml/min/1.73 m(2); (MDRD). Hypertension was defined to be present if there was a recorded diagnosis of hypertension, systolic blood pressure >130 mmHg and/or diastolic blood pressure >80 mmHg according to ambulatory blood pressure monitoring. None of the hypertensive patients were receiving RAS blockers. Spot urine samples were obtained to measure urinary angiotensinogen (AGT) using human AGT-ELISA, urinary creatinine and protein levels. The demographic properties and laboratory findings were similar between hypertensive and normotensive transplant recipients. Urinary AGT-creatinine ratio (UAGT/UCre) was significantly higher in hypertensive patients compared with the normotensives (8.98 ± 6.89 µg/g vs. 5.48 ± 3.33 µg/g; P = 0.037). Importantly, a significantly positive correlation was found between UAGT/Ucre levels and proteinuria in hypertensive patients (P = 0.01, r = 0.405). Local intrarenal RAS probably plays an important role in the development of hypertension and proteinuria in renal transplant recipients.

Factors affecting prognosis of the patients with severe hyponatremia.

INTRODUCTION: Hyponatremia is one of the most common electrolyte abnormalities in clinical practice. Data regarding factors that have impact on mortality of severe hyponatremia and outcomes of its therapeutic management is insufficient. The present study aimed to examine the factors associated with mortality and the outcomes of treatment in patients with severe hyponatremia. MATERIALS AND METHODS: Patients with serum Na≤115mequiv./L who were admitted to Ordu State Hospital and Ordu University Training and Research Hospital between 2014 and 2018 were included in the study. Demographic and laboratory features, severity of the symptoms, comorbid diseases, medications, and clinical outcome measures of the patients were obtained retrospectively from their medical records. Factors associated with in-hospital mortality, overcorrection and undercorrection were assessed. RESULTS: A total of 145 patients (median age 69 years and 58.6% female) met inclusion criteria. Diuretic use was the most common etiologic factor for severe hyponatremia that present in 50 (34.5%) patients. Sixty-seven (46.2%) patients had moderately severe while 8 patients (5.5%) had severe symptoms. The median increase in serum Na 24h after admission in the study population was 8.9mequiv./L (-6 to 19). Nonoptimal correction was seen in 92 (63.4%) patients. Hypertonic saline use was associated with overcorrection (OR, 3.07; 95% CI: 1.47-6.39; p=0.002). Avoidance of hypertonic saline (aOR, 2.52; 95% CI: 1.12-5.66; p=0.029) and having neuropsychiatric disorder (aOR, 2.60; 95% CI: 1.10-6.11; p=0.025) were associated with undercorrection. In-hospital mortality rate was 12.4% and having CKD and cancer, undercorrection of sodium and presence of severe symptoms were significantly associated with in-hospital mortality. CONCLUSION: Severe hyponatremia in hospitalized patients is associated with substantial mortality. The incidence of non-optimal correction of serum Na is high; under-correction, presence of severe symptoms, chronic kidney disease and cancer were the factors that increase mortality rate.

Daily sodium intake in chronic kidney disease patients during nephrology clinic follow-up: an observational study with 24-hour urine sodium measurement.

OBJECTIVE: To determine daily sodium intake in 'real practice' in a large group of chronic kidney disease (CKD) patients who were under regular follow-up in a nephrology clinic. METHODS: A total of 373 consecutive outpatients with CKD stages 1-5 (not on dialysis; men: 52.3%, mean age: 51.6 ± 15.4 years) were included in the study. All patients had at least 3 or more nephrology visits and received information on reducing their sodium intake. Data for systolic and diastolic blood pressure, number of antihypertensive medications and 2 consecutive 24-hour urinary sodium levels were obtained from the patients' medical records. RESULTS: The mean 24-hour urinary sodium levels of 2 consecutive urine samples were 168.8 ± 70.3 and 169.3 ± 67.4 mEq/day (p > 0.05). Only 14.7% of the patients had a sodium excretion <100 mmol/day. There was no difference in daily sodium intake from stages 1 to 4, but it was significantly lower in stage 5 (126.6 ± 60.5 mEq/day, p < 0.05). No relation was found between 24-hour urinary sodium output, number of antihypertensives or thiazide use. CONCLUSIONS: This study showed that almost 85% of CKD patients under regular nephrologic care were consuming more sodium than the recommended level. More robust measures should be devised to increase patient and physician compliance with reducing sodium intake in CKD.

Factors affecting prognosis of the patients with severe hyponatremia.

INTRODUCTION: Hyponatremia is one of the most common electrolyte abnormalities in clinical practice. Data regarding factors that have impact on mortality of severe hyponatremia and outcomes of its therapeutic management is insufficient. The present study aimed to examine the factors associated with mortality and the outcomes of treatment in patients with severe hyponatremia. MATERIALS AND METHODS: Patients with serum Na≤115mequiv./L who were admitted to Ordu State Hospital and Ordu University Training and Research Hospital between 2014 and 2018 were included in the study. Demographic and laboratory features, severity of the symptoms, comorbid diseases, medications, and clinical outcome measures of the patients were obtained retrospectively from their medical records. Factors associated with in-hospital mortality, overcorrection and undercorrection were assessed. RESULTS: A total of 145 patients (median age 69 years and 58.6% female) met inclusion criteria. Diuretic use was the most common etiologic factor for severe hyponatremia that present in 50 (34.5%) patients. Sixty-seven (46.2%) patients had moderately severe while 8 patients (5.5%) had severe symptoms. The median increase in serum Na 24h after admission in the study population was 8.9mequiv./L (-6 to 19). Nonoptimal correction was seen in 92 (63.4%) patients. Hypertonic saline use was associated with overcorrection (OR, 3.07; 95% CI: 1.47-6.39; p=0.002). Avoidance of hypertonic saline (aOR, 2.52; 95% CI: 1.12-5.66; p=0.029) and having neuropsychiatric disorder (aOR, 2.60; 95% CI: 1.10-6.11; p=0.025) were associated with undercorrection. In-hospital mortality rate was 12.4% and having CKD and cancer, undercorrection of sodium and presence of severe symptoms were significantly associated with in-hospital mortality. CONCLUSION: Severe hyponatremia in hospitalized patients is associated with substantial mortality. The incidence of non-optimal correction of serum Na is high; under-correction, presence of severe symptoms, chronic kidney disease and cancer were the factors that increase mortality rate.

AA type renal amyloidosis secondary to FMF: a long-term follow-up in two patients.

Renal amyloidosis, which leads to renal failure, is the most important long-term complication of familial Mediterranean fever (FMF). Resolution of nephrotic syndrome secondary to amyloidosis in FMF following colchicine treatment has rarely been reported. We describe two patients with FMF and nephrotic syndrome. These patients were treated with colchicine 1.5 mg/day and had a complete remission of nephrotic syndrome with a stable clinical course over 30 years. To our knowledge, our patients have the longest follow-up time without proteinuria.

Factors affecting prognosis of the patients with severe hyponatremia / Factores que afectan el pronóstico de los pacientes con graves hyponatremia

Introduction: Hyponatremia is one of the most common electrolyte abnormalities in clinical practice. Data regarding factors that have impact on mortality of severe hyponatremia and outcomes of its therapeutic management is insufficient. The present study aimed to examine the factors associated with mortality and the outcomes of treatment in patients with severe hyponatremia.Materials and methods: Patients with serum Na≤115mequiv./L who were admitted to Ordu State Hospital and Ordu University Training and Research Hospital between 2014 and 2018 were included in the study. Demographic and laboratory features, severity of the symptoms, comorbid diseases, medications, and clinical outcome measures of the patients were obtained retrospectively from their medical records. Factors associated with in-hospital mortality, overcorrection and undercorrection were assessed.Results: A total of 145 patients (median age 69 years and 58.6% female) met inclusion criteria. Diuretic use was the most common etiologic factor for severe hyponatremia that present in 50 (34.5%) patients. Sixty-seven (46.2%) patients had moderately severe while 8 patients (5.5%) had severe symptoms. The median increase in serum Na 24h after admission in the study population was 8.9mequiv./L (−6 to 19). Nonoptimal correction was seen in 92 (63.4%) patients. Hypertonic saline use was associated with overcorrection (OR, 3.07; 95% CI: 1.47–6.39; p=0.002). Avoidance of hypertonic saline (aOR, 2.52; 95% CI: 1.12–5.66; p=0.029) and having neuropsychiatric disorder (aOR, 2.60; 95% CI: 1.10–6.11; p=0.025) were associated with undercorrection. In-hospital mortality rate was 12.4% and having CKD and cancer, undercorrection of sodium and presence of severe symptoms were significantly associated with in-hospital mortality.Conclusion: Severe hyponatremia in hospitalized patients is associated with substantial mortality. ... (AU)

Introducción: La hiponatremia es una de las alteraciones electrolíticas más frecuentes en la práctica clínica. Los datos sobre los factores que tienen impacto en la mortalidad de la hiponatremia grave y los resultados de su manejo terapéutico son insuficientes. El presente estudio tuvo como objetivo examinar los factores asociados con la mortalidad y los resultados del tratamiento en pacientes con hiponatremia grave.Materiales y métodos: Se incluyeron en el estudio pacientes con Na sérico ≤ 115 mequiv./L que ingresaron en el Hospital Estatal de Ordu y en el Hospital de Investigación y Capacitación de la Universidad de Ordu entre 2014 y 2018. Las características demográficas y de laboratorio, la gravedad de los síntomas, las enfermedades comórbidas, los medicamentos y las medidas de resultado clínico de los pacientes se obtuvieron retrospectivamente de sus registros médicos. Se evaluaron los factores asociados con la mortalidad hospitalaria, la hipercorrección y la hipocorrección.Resultados: Un total de 145 pacientes (mediana de edad de 69 años y 58,6% mujeres) cumplieron los criterios de inclusión. El uso de diuréticos fue el factor etiológico más común para la hiponatremia grave que se presenta en 50 (34,5%) pacientes. Sesenta y siete (46,2%) pacientes tenían síntomas moderadamente graves, mientras que 8 pacientes (5,5%) tenían síntomas graves. El aumento medio del Na sérico 24 h después de la admisión en la población de estudio fue de 8,9 mequiv./L (-6 a 19). Se observó una corrección no óptima en 92 (63,4%) pacientes. El uso de solución salina hipertónica se asoció con sobrecorrección (OR, 3.07; 95% CI:1.47–6.39; p = 0.002). Evitar la solución salina hipertónica (aOR, 2.52; 95% CI: 1.12–5.66; p = 0.029) y tener un trastorno neuropsiquiátrico (aOR, 2.60; 95% CI: 1.10–6.11; p = 0.025) se asociaron con hipocorrección. ... (AU)

HFE gene mutation is a risk factor for tissue iron accumulation in hemodialysis patients.

INTRODUCTION: HFE gene mutations are responsible from iron overload in general population. Studies in hemodialysis patients investigated the effect of presence of HFE gene mutations on serum ferritin and transferrin saturation (TSAT) with conflicting results. However effect of HFE mutations on iron overload in hemodialysis patients was not previously extensively studied. METHODS: 36 hemodialysis patients (age 51.3 ± 15.6, (18/18) male/female) and 44 healthy control subjects included in this cross sectional study. Hemoglobin, ferritin, TSAT in the preceding 2 years were recorded. Iron and erythropoietin (EPO) administered during this period were calculated. Iron accumulation in heart and liver was detected by MRI. Relationship between HFE gene mutation, hemoglobin, iron parameters and EPO doses, and tissue iron accumulation were determined. FINDINGS: Iron overload was detected in nine (25%) patients. Hemoglobin, iron parameters, weekly EPO doses, and monthly iron doses of patients with and without iron overload were similar. There was no difference between control group and hemodialysis patients with respect to the prevalence of HFE gene mutations. Iron overload was detected in five of eight patients who had HFE gene mutations, but iron overload was present in 4 of 28 patients who had no mutations (P = 0.01). Hemoglobin, iron parameters, erythropoietin, and iron doses were similar in patients with and without gene mutations. HFE gene mutations remained the main determinant of iron overload after multivariate logistic regression analysis (P = 0.02; OR, 11.6). DISCUSSION: Serum iron parameters were not adequate to detect iron overload and HFE gene mutation was found to be an important risk factor for iron accumulation.

Safety of Percutaneous Ultrasound-Guided Kidney Biopsy in Patients with AA Amyloidosis.

BACKGROUND: Bleeding is the most frequent complication of kidney biopsy. Although bleeding risk in patients with AA amyloidosis after kidney biopsy has not been studied in a large population, AA amyloidosis has long been perceived as a risk factor for bleeding. The aim of the present study was to evaluate post-biopsy bleeding risk in patients with AA amyloidosis. METHODS: We retrospectively analyzed bleeding complications in 88 patients with AA amyloidosis and 202 controls after percutaneous kidney biopsy. All the kidney biopsies were performed under the guidance of real-time ultrasound with the use of an automated core biopsy system after a standard pre-biopsy screening protocol. Bleeding events were classified as major when transfusion of blood products or surgical or radiological intervention was required, or if the bleeding caused hypovolemic shock or death. Bleeding events that did not meet these criteria were accepted as minor. RESULTS: The incidence of post-biopsy bleeding was comparable between AA amyloidosis and control groups (5.7 vs. 5.0%, p = 0.796). Major bleeding events were observed in 3 patients from each group (p = 0.372). Selective renal angiography and embolization were applied to 2 patients from the AA amyloidosis group. One of these patients underwent colectomy and died because of infectious complications. Bleeding events were minor in 2.3% of the patients with AA amyloidosis and 3.5% of the controls (p = 0.728). CONCLUSIONS: AA amyloidosis was not associated with increased post-biopsy bleeding risk. Kidney biopsy is safe in AA amyloidosis when standard pre-biopsy screening is applied. Further data are needed to confirm these findings.

Relationship between vitamin D levels and depressive symptoms in renal transplant recipients.

OBJECTIVE: Vitamin D deficiency might influence the development of depression; however, the association between vitamin D and depression in renal transplant recipients has not been evaluated. We aimed to test if there is a relation between 25-hydroxy (OH) vitamin D levels and depressive symptoms in patients with kidney transplantation. METHODS: This was a cross-sectional and descriptive study. A total of 117 renal transplant recipients (44 female, 73 male; mean age, 39.0 ± 11.7 years) were included in the study. Patients were stratified to two groups according to the cut-off point (7) of depression subscale (D) of Hospital Anxiety Depression Scale (HADS), with or without depression risk. Blood biochemistry, glomerular filtration rate (GFR), and 25-OH vitamin D levels were determined. RESULTS: Depression scores were higher than cut-off point in 33.3% (n = 39) of patients. The mean 25-OH vitamin D level was 19.6 ± 12.0 µg/L. In the group with depression risk, 25-OH vitamin D levels were significantly lower than the other group (15.2 ± 9.2 µg/L and 21.9 ± 12.7 µg/L, respectively; p = 0.004). No significant difference was observed between the two groups in terms of demographic parameters, blood biochemistry, and GFR. A negative correlation was detected between HADS-D score and vitamin D levels (r = -0.365, p < 0.0001). CONCLUSION: Lower serum 25-OH vitamin D levels are associated with higher depressive symptom levels among renal transplantation recipients. This finding should be the basis for further clinical studies and for future prospects on vitamin D supplementation for prevention and treatment of depression in these patients.

Severe Demodex infestation of a coal miner.

We report a case of Demodex infestation in a 35 year old coal miner presenting with a 5 year history of scally papulopustular eruption on his face. He had been working inunderground coal tunnels in a humid- hot- dusty environment and he had been used to bath twice a day with hot water and multiple cleaners. The patient was treated successfully with oral metronidazol, topical permethrin, topical steroids and avoidance of undergraund mining . We believe his occupational environment made him prone to infestation by changes in sebum composition and/or viscosity, his bath habituation facilitated infestation, damaging the epidermal barrier function and his previous treatments exaggerated his infestation. During evaluation of the patient, specific occupational factors and habituations will be related with higher succession rates of treatment. We need to conduct further studies in order to draw a definite conclusion about the effect of the occupational environment on Demodex infestation.

The relation between urinary angiotensinogen and proteinuria in renal AA amyloidosis patients.

OBJECTIVE: The aim of this study was to evaluate the relationship of local intrarenal renin angiotensin system (RAS) with proteinuria in patients with renal AA amyloidosis. METHODS: Thirty-two patients with renal AA amyloidosis (19 male, mean age: 45 ± 13 years) and sixteen healthy controls (5 male, mean age: 32 ± 5 years) were included in this study. Spot urine samples were obtained to measure urinary angiotensinogen (AGT) using human AGT-ELISA, urinary creatinine and protein levels. Logarithmic transformations of urinary AGT-creatinine ratio log(UAGT/Ucre) and urinary protein-to-creatinine ratio (UPCR) were done to obtain the normal distributions of these parameters. RESULTS: Log(UAGT/UCre) was significantly higher in patients compared with the controls (1.88 ± 0.92 µg/g vs. 1.25 ± 0.70 µg/g; p = 0.023). Importantly a significantly positive correlation was found between log(UAGT/Ucre) and logUPCR in patients (r = 0.595, p = 0.006). CONCLUSIONS: Urinary AGT levels are higher in renal AA amyloidosis patients than in controls. Also, there is a significant positive correlation between urinary AGT and proteinuria in renal AA amyloidosis.