Thoracic and abdominal pain in a 28-year-old woman with a failing kidney transplant.

This case report describes a young, immunocompromised patient who presented with thoracic pain. After an extensive workup, she was diagnosed with a varicella zoster virus infection with involvement of the gastric mucosa, pancreas and lungs for which she was treated with acyclovir. Although the viral load decreased significantly, the patient had persistent postherpetic neuralgia and nausea.

Bile Cast Nephropathy Caused by Obstructive Pancreatic Carcinoma and Failed ERCP.

Bile cast nephropathy is an often overlooked condition of acute renal injury in the setting of high serum bilirubin. While the exact pathophysiology remains unknown, possible mechanisms of renal injury are tubular obstruction from bile casts, direct toxicity from bile acids, and decreased renal perfusion due to hemodynamic changes. We present a patient with hyperbilirubinemia as a result of common bile duct obstruction due to pancreatic adenocarcinoma who developed anuric acute renal injury. Urine analysis showed bile casts that were highly suggestive for bile cast nephropathy. The patient underwent hemodialysis and bile drainage with full restoration of renal function.

Listeria Endocarditis: A Diagnostic Challenge.

A 74-year-old hemodialysis patient with a history of an atrial septum defect closure, coronary bypass surgery, and a St. Jude aortic prosthetic valve was diagnosed with pneumonia and volume overload. Blood cultures were positive for Listeria monocytogenes, and amoxicillin was given for 2 weeks. Immediately after discontinuation of amoxicillin, fever relapsed. Transthoracic and transesophageal echocardiography showed no sign of endocarditis. Given the fever relapse and 3 positive minor Duke criteria, an 18F-FDG PET-CT scan (18F-fluorodeoxyglucose-positron emission tomography-computed tomography) scan was performed. This scan showed activity at the aortic root, proximal ascending aorta, and inferior wall of the heart, making Listeria monocytogenes endocarditis a likely explanation. Amoxicillin was given for 6 weeks with good clinical result. Diagnosing a life-threatening Listeria monocytogenes endocarditis can be challenging and an 18F-FDG PET-CT scan can be helpful.

The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.

BACKGROUND: Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers. OUTCOMES: Partial and complete remission, treatment resistance, relapse, complications, renal survival. RESULTS: Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy. LIMITATIONS: Retrospective design, variable treatment protocols. CONCLUSIONS: The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.

Plasma concentration of von Willebrand factor predicts mortality in patients on chronic renal replacement therapy.

BACKGROUND: Traditional cardiovascular risk factors do not explain the high incidence of cardiovascular mortality and morbidity in patients with end-stage renal disease. A prothrombotic state could accelerate the process of vascular disease in these patients. METHODS: In this study, four platelet activation markers (NAP-2, P-selectin, GP1b and RANTES) and two endothelial cell activation markers (von Willebrand factor and its propeptide) were measured in 671 haemodialysis patients and 275 patients on continuous ambulatory peritoneal dialysis (PD). All were long-term dialysis patients. The risk of all-cause and cardiovascular mortality was assessed in relation to these markers after a mean follow-up time of 2.5 years. RESULTS: The von Willebrand factor showed a positive correlation with total mortality in the haemodialysis patients. In an unadjusted model, the hazard rate (HR) of total mortality was 2.4 [95% confidence interval (95% CI) 1.7-3.4] in the upper quartile of von Willebrand factor compared with the lowest quartile. It remained statistically significant (HR 1.8; 95% CI 1.2-2.6) after adjustment for traditional risk factors. In contrast, no significant correlation was found between von Willebrand factor levels and total mortality in PD patients. Finally, no relationship between platelet activation markers and total mortality was found in either the haemodialysis or the PD patients. CONCLUSION: It can be concluded that chronic endothelial cell activation, but not platelet activation, is related to all-cause mortality in end-stage renal disease patients on long-term dialysis.

[Renal manifestation of Henoch-Schönlein purpura in adults]. / Renale manifestatie van henoch-schönleinpurpura bij volwassenen.

For a few months a 19-year-old man had red maculae on both feet which spread once a month. He also had macroscopic haematuria. A 45-year-old man was admitted with a deep venous thrombosis of his left leg and acute renal insufficiency. He had a history of purpura on his legs, a skin biopsy of which had shown leukoclastic vasculitis. A 37-year-old woman had persistent hypertension and nephrotic syndrome following childbirth. She had suffered from skin problems and oedema since the age of 6. All 3 patients proved to have Henoch-Schönlein purpura with renal symptoms. In principle Henoch-Schönlein purpura is treated with supportive measures, but if there is renal involvement it is advised to start symptomatic treatment of proteinuria and blood pressure. Depending on the degree of renal involvement, immunosuppresives may also be prescribed.

A haemodialysis patient with back pain: brown tumour as a cause of spinal cord compression under cinacalcet therapy.

A 43-year-old haemodialysis patient was admitted to hospital because of paroxysmal pain in the upper abdominal region radiating to the back. Laboratory tests showed severe hyperparathyroidism [intact parathyroid hormone (iPTH) 69 pmol/L; reference range: 1.3-6.8 pmol/L], hypercalcaemia (2.79 mmol/L), hyperphosphataemia (1.6 mmol/L) and elevated serum total alkaline phosphatase (200 U/L). After developing a disturbed sensation and paraesthesia in both feet, epidural compression of the spinal cord was suspected. Magnetic resonance imaging showed a tumour that severely compressed the myelum of the thoracic spine. Histological investigation revealed a brown tumour or osteoclastoma, an erosive bony lesion caused by increased osteoclastic activity and peritrabecular fibrosis. A brown tumour is a benign tumour that is a rare complication of severe renal hyperparathyroidism. The brown tumour developed despite a 1-year treatment of the patient with cinacalcet, which, however, did not result in a major decrease in serum iPTH concentration (from 110 to 69 pmol/L: 37% reduction). Urgent decompressive neurosurgery and subtotal parathyroidectomy resulted in a complete recovery.

[Hyperchloraemic metabolic acidosis in a patient with an ureteroileostomy according to Bricker]. / Hyperchloremische metabole acidose bij een patiënt met een brickerlis.

A 79-year-old male with a Bricker loop and chronic renal failure was admitted to hospital because progressive dyspnoea. This was due to severe hyperchloraemic metabolic acidosis. Hyperchloraemic acidosis can occur if urinary diversions are constructed from the colon or ileum. Contact between intestinal mucosa and urine may cause reabsorption of ammonium and chloride, and secretion of bicarbonate. Hyperchloraemic acidosis is rarely seen with an incontinent ileal loop due to its small absorbing surface area and the rapid drainage of urine from the loop. Hyperchloraemic acidosis in a patient with a Bricker loop may point to prolonged contact between the ileum and urine. A loopogram is necessary to investigate the cause. In our patient the loopogram showed that the incorporated bowel segment was too long. After shortening of the Bricker loop, the patient recovered from the hyperchloraemic metabolic acidosis.

Stent placement in patients with atherosclerotic renal artery stenosis and impaired renal function: a randomized trial.

BACKGROUND: Little is known about the efficacy and safety of renal artery stenting in patients with atherosclerotic renal artery stenosis (ARAS) and impaired renal function. OBJECTIVE: To determine the efficacy and safety of stent placement in patients with ARAS and impaired renal function. DESIGN: Randomized clinical trial. Randomization was centralized and computer generated, and allocation was assigned by e-mail. Patients, providers, and persons who assessed outcomes were not blinded to treatment assignment. SETTING: 10 European medical centers. PARTICIPANTS: 140 patients with creatinine clearance less than 80 mL/min per 1.73 m(2) and ARAS of 50% or greater. INTERVENTION: Stent placement and medical treatment (64 patients) or medical treatment only (76 patients). Medical treatment consisted of antihypertensive treatment, a statin, and aspirin. MEASUREMENTS: The primary end point was a 20% or greater decrease in creatinine clearance. Secondary end points included safety and cardiovascular morbidity and mortality. RESULTS: Forty-six of 64 patients assigned to stent placement had the procedure. Ten of the 64 patients (16%) in the stent placement group and 16 patients (22%) in the medication group reached the primary end point (hazard ratio, 0.73 [95% CI, 0.33 to 1.61]). Serious complications occurred in the stent group, including 2 procedure-related deaths (3%), 1 late death secondary to an infected hematoma, and 1 patient who required dialysis secondary to cholesterol embolism. The groups did not differ for other secondary end points. LIMITATION: Many patients were falsely identified as having renal artery stenosis greater than 50% by noninvasive imaging and did not ultimately require stenting. CONCLUSION: Stent placement with medical treatment had no clear effect on progression of impaired renal function but led to a small number of significant procedure-related complications. The study findings favor a conservative approach to patients with ARAS, focused on cardiovascular risk factor management and avoiding stenting.

The prevalence of atherosclerotic renal artery stenosis in risk groups: a systematic literature review.

OBJECTIVE: We performed a literature review and analysis to improve the insight in the prevalence of renal artery stenosis (RAS) in risk groups. METHODS: Relevant studies were identified by a MEDLINE and EMBASE database search (1966 to December 2007), complemented by hand searching of reference lists. Review was restricted to English language studies, using any form of angiography as diagnostic method. Studies were grouped in risk group categories sharing similar clinical characteristics, and pooled prevalence rates were calculated for each category. RESULTS: Forty studies, involving a total number of 15 879 patients, were identified. The following pooled prevalence rates (95% confidence interval; sample size risk group) of RAS were found: suspected renovascular hypertension, 14.1% (12.7-15.8%; n = 1931); hypertension and diabetes mellitus, 20% (14.9-25.1%; n = 240); coronary angiography (CAG) in consecutive patients, 10.5% (9.8-11.2%; n = 8011); CAG in hypertensive patients, 17.8% (15.4-20.6%; n = 836); CAG and suspected renovascular disease, 16.6% (14.8-18.5%; n = 1576); congestive heart failure, 54.1% (45.7-62.3%; n = 135); peripheral vascular disease, 25.3% (23.6-27.0%; n = 2632); abdominal aortic aneurysm, 33.1% (27.4-39.2%; n = 239) and end-stage renal failure, 40.8% (27-55.8%; n = 49.) In patients with an incidentally discovered RAS, hypertension and renal failure were present in 65.5 and 27.5%, respectively. CONCLUSION: RAS has a high prevalence in risk groups, especially in those with extrarenal atherosclerosis, end-stage renal failure and heart failure. These findings are important when screening for RAS or prescription of an angiotensin converting enzyme inhibitor or angiotensin-II receptor blocker is considered.

Renal function as a risk indicator for cardiovascular events in 3216 patients with manifest arterial disease.

AIM: To establish whether impaired renal function is an independent predictor of cardiovascular disease (CVD) and death in an unselected high-risk population with CVD. METHODS AND RESULTS: In 3216 patients with CVD, the estimated glomerular filtration rate (GFR) was assessed with the Modification of Diet in Renal Disease (MDRD)-equation. Primary outcomes were all vascular events (including stroke, myocardial infarction, end-stage renal disease and vascular death) and all cause death. During a median follow-up of 39 months, 378 patients had a vascular event (11.7%) and 337 patients died (10.5%). The adjusted hazard ratio (HR) of an estimated GFR90 ml/min per 1.73 m(2) was 1.8 (95% CI, 1.2-2.6) for vascular events and 1.4 (95% CI 0.9-2.0) for all cause death. For stroke and cardiac events as separate outcomes, similar HR's were found. Subgroup analysis according to localization of vascular disease at presentation or presence of the risk factors hypertension, diabetes and albuminuria had no influence on the hazard ratios. CONCLUSIONS: The presence of moderate to severe renal insufficiency is an independent risk factor for adverse CVD events in high-risk patients with a history of vascular disease. Localization of vascular disease or presence of other risk factors had no influence on the impact of renal function alone.

Renal blood flow measurements with use of phase-contrast magnetic resonance imaging: normal values and reproducibility.

PURPOSE: To assess the validity and the direct, short-term, and long-term reproducibility of renal blood flow (RBF) measurements with phase-contrast (PC) magnetic resonance (MR) imaging. MATERIALS AND METHODS: In 20 healthy volunteers, RBF measurements were repeated with and without repositioning. Internal validity was assessed by comparing the total RBF with the difference in aortic flow above and below the renal arteries. In 19 healthy volunteers, RBF measurements were performed at two different occasions. In 40 healthy volunteers, RBF measurements were performed to assess normal values as a function of age. Analyses were performed according to Bland and Altman. RESULTS: The technical success rate ranged from 78% to 85%. Total RBF and the difference in aortic flow rates showed good agreement (Pearson correlation coefficient, 0.72; P = .002). Directly repeated measurements had a mean difference of 54 mL/min in total RBF with a coefficient of variation (CV) of 17%. For repeated measurements with repositioning, the mean difference in total RBF was 74 mL/min (CV, 23%). Repeated measurements on different occasions showed a CV of 20%. The mean total RBF of the 40 healthy volunteers was 838 mL/min +/- 244 (SD). CONCLUSIONS: RBF measurement with PC MR has a success rate greater than 75%. The demonstrated internal reliability of this method and fair reproducibility of the flow parameters is crucial for further studies of the renal artery with MR imaging.

Increased albumin and fibrinogen synthesis rate in patients with chronic renal failure.

BACKGROUND: Hypoalbuminemia and hyperfibrinogenemia are frequently observed in patients with chronic renal failure (CRF) and are both associated with cardiovascular diseases. The mechanisms responsible for hypoalbuminemia and hyperfibrinogenemia in CRF are unknown. METHODS: In the present study, both albumin and fibrinogen kinetics were measured in vivo in predialysis patients (N = 6), patients on peritoneal dialysis (N = 7) and control subjects (N = 8) using l-[1-13C]-valine. RESULTS: Plasma albumin concentration was significantly lower in patients on peritoneal dialysis compared to control subjects (P < 0.05). Plasma fibrinogen was significantly increased in both predialysis patients (P < 0.01) as well as patients on peritoneal dialysis (P < 0.001) in comparison to control subjects. In contrast to albumin, fibrinogen is only lost in peritoneal dialysate and not in urine. The absolute synthesis rates (ASR) of albumin and fibrinogen were increased in patients on peritoneal dialysis (ASR albumin, 125 +/- 9 mg/kg/day versus 93 +/- 9 mg/kg/day, P < 0.05; ASR fibrinogen, 45 +/- 4 mg/kg/day versus 29 +/- 3 mg/kg/day, P < 0.01) compared to control subjects. Albumin synthesis is strongly correlated with fibrinogen synthesis (r2 = 0.665, P < 0.0001, N = 21). In this study, the observed hypoalbuminemia in patients on peritoneal dialysis is likely not explained by malnutrition, inadequate dialysis, inflammation, metabolic acidosis, or insulin resistance. We speculate that peritoneal albumin loss is of relevance. CONCLUSION: Synthesis rate of albumin and fibrinogen are coordinately up-regulated. Both albumin and fibrinogen are lost in peritoneal dialysis fluid. To compensate protein loss, albumin synthesis is up-regulated, but the response, in contrast to predialysis patients, does not fully correct plasma albumin concentrations in peritoneal dialysis patients. The increase in fibrinogen synthesis introduces an independent risk factor for atherosclerosis, since plasma fibrinogen pool is enlarged.

Prevalence of atherosclerotic renal artery stenosis in patients starting dialysis.

BACKGROUND: Atherosclerotic renal artery stenosis (ARAS) can lead to end-stage renal failure (ESRF). We determined the prevalence of ARAS in patients 45 years of age or older starting renal replacement therapy. METHODS: Forty-nine of 80 consecutive patients (37 males, 12 females) starting renal replacement therapy in our centre gave informed consent and underwent spiral computed tomographic angiography of their renal arteries. A renal artery diameter reduction of 50% or more assessed by two radiologists was considered as a significant stenosis. RESULTS: Twenty of 49 patients (41%) had an ARAS, and in eight cases (16%) this was bilateral or unilateral with a single kidney. Women were more likely to have an ARAS than men; 75 (9/12) vs 30% (11/37, P<0.01). However, relatively more women declined participation. Non-participants and participants did not differ in respect to other relevant clinical data. Nonetheless, findings in these patients would be negative, the prevalence of ARAS would still be 31% in women and 22% in men (NS). In 13 patients with ARAS the registered diagnosis of ESRF either was hypertension, renovascular disease or unknown. Assuming that in these patients atherosclerotic renovascular disease was the cause of renal failure, a total of 13 patients (13/49, 27%) entered the dialysis programme because of this problem. CONCLUSIONS: These results suggest that ARAS is an important cause of ESRF.

Repeated intervention for in-stent restenosis of the renal arteries.

PURPOSE: To assess the long-term technical success of repeated endovascular intervention in stenosed renal artery stents. MATERIALS AND METHODS: Fifteen patients with stenoses >or=50% in a renal stent placed because of an ostial atherosclerotic renal artery stenosis were included in this study. In the presence of increased blood pressure or decreased renal function, the in-stent restenosis was treated with percutaneous transluminal angioplasty (PTA) in the stent or placement of a second stent if the stenosis was located too distally in the stent. The results of these repeat interventions were evaluated by angiography. RESULTS: The 15 patients had a total of 20 stenosed stents. Eighteen of these in-stent stenoses were treated with PTA and two were treated with placement of a second stent. Angiographic follow-up was available in 16 arteries, showing in-stent restenosis in four (25%; mean follow-up, 11 mo). The cumulative patency rates after repeat endoluminal intervention were 93% (95% CI: 80%-106%) and 76% (95% CI: 52%-101%) after 6 and 12 months, respectively. Renal function remained stable or improved in most patients (80%) after repeated intervention in the stent, and hypertension was classified as improved or cured in 47% of patients after 1 year. CONCLUSION: Patients with stenosed renal artery stents can be treated successfully with PTA in a majority of cases, with a long-term success rate of 75% and stable renal function 1 year after repeated intervention.

Long-term effects of arterial stenting on kidney function for patients with ostial atherosclerotic renal artery stenosis and renal insufficiency.

It is uncertain whether renal artery stent placement in patients with atherosclerotic renovascular renal failure can prevent further deterioration of renal function. Therefore, the effects of renal artery stent placement, followed by patency surveillance, were prospectively studied in 63 patients with ostial atherosclerotic renal artery stenosis and renal dysfunction (i.e., serum creatinine concentrations of >120 micromol/L (median serum creatinine concentration, 171 micromol/L; serum creatinine concentration range, 121 to 650 micromol/L). Pre-stent renal (dys) function was stable for 28 patients and declining for 35 patients (defined as a serum creatinine concentration increase of > or =20% in 12 mo). The median follow-up period was 23 mo (interquartile range, 13 to 29 mo). Angioplasty to treat restenosis was performed in 12 cases. Five patients reached end-stage renal failure within 6 mo, and this was related to stent placement in two cases. Two other patients died or were lost to follow-up monitoring within 6 mo, with stable renal function. For the remaining 56 patients, the treatment had no effect on serum creatinine levels if function had previously been stable; if function had been declining, median serum creatinine concentrations improved in the first 1 yr [from 182 micromol/L (135 to 270 micromol/L ) to 154 micromol/L (127 to 225 micromol/L ); P < 0.05] and remained stable during further follow-up monitoring. In conclusion, stent placement, followed by patency surveillance, to treat ostial atherosclerotic renal artery stenosis can stabilize declining renal function. For patients with stable renal dysfunction, the usefulness is less clear. The possible advantages must be weighed against the risk of renal failure advancement with stent placement.

Vascular Effects of ACE Inhibition Independent of the Renin-Angiotensin System in Hypertensive Renovascular Disease : A Randomized, Double-Blind, Crossover Trial.

-To evaluate whether ACE inhibition and angiotensin II type 1 blockade exert beneficial effects on NO availability independent of their blood pressure-lowering effect, we used a double-blind crossover design to study vascular function in 18 patients with hypertensive renovascular disease during 6 weeks of therapy with enalapril (Ena) and valsartan (Val) compared with non-renin-angiotensin system-mediated treatment with the alpha(1)-blocker doxazosin (Dox). Control measurements were performed in 13 age-matched volunteers. Forearm blood flow was assessed with venous occlusion plethysmography, and serotonin and nitroprusside were used as endothelium-dependent and -independent vasodilators, respectively. Blood pressure was similar during all treatment periods. Serotonin-induced vasodilation was decreased in patients during Dox treatment (n=12) compared with control subjects (n=13) (increase 42+/-20% versus 107+/-65%, P:<0.05). Crossover from Dox to Val (n=6) had no effect on serotonin response (increase 50+/-14%), but crossover to Ena (n=6) caused a significant improvement (increase 79+/-39%, P:<0.05 versus Dox). In an assessment of all patients, serotonin-induced vasodilation during Ena (n=12, increase 75+/-31%) was increased compared with both Val and Dox (43+/-14% and 42+/-20%, respectively; both P:<0.05 versus Ena). The nitroprusside response remained unaltered during all treatment periods. In conclusion, ACE inhibition improves the impaired endothelium-dependent vascular function in patients with hypertensive renovascular disease. This effect is unrelated to blood pressure-lowering or angiotensin II-mediated effects.