Spontaneous perirenal hemorrhage in hemodialysis patient treated with selective embolization: A case series and review of the literature

Introduction: Spontaneous perirenal hemorrhage (SPH) or Wunderlich syndrome, is a rare but potentially life‐threatening condition. It is characterized by an unexpected bleeding in the kidneys and usually presents as an abdominal pain. Angiography and more recently selective renal arterial embolization are emerging as effective modalities for the diagnosis and treatment of SPH. In this article, we report a total of three cases of SPH in hemodialysis (HD) patients. Methods: This is the experience of diagnosis and treatment of SPH in HD patients. Findings: All three were female, between 37 and 54 years of age and were undergoing HD for end stage renal disease (ESRD). Two of patients presented with left flank or abdominal pain after termination of HD therapy, while the third patient presented with left abdominal pain during the dialysis session. All patients received anti‐coagulation therapy for HD, but no abnormal levels of coagulation index were found. These patients were diagnosed using CT and two of them were diagnosed with acquired cystic kidney disease (ACKD). Selective renal arterial embolization was performed in the case of active bleeding. Discussion: We are aware that HD patients have elevated risk of bleeding related complications, additionally the presence of an acute abdominal pain increases the suspicion of SPH as a possible cause. ACKD can be considered one of the possible risk factors for SPH in long‐term HD patients. Interventional treatment for kidney injury is useful and safe for active bleeding in most cases.     

Case Report: Atheroembolic renal disease in a 72‐year‐old patient through coronary intervention after myocardial infarction

Cholesterol embolization or atheroembolic renal disease (AERD) is an often underdiagnosed issue in patients featuring a prevalent risk profile. It is a multisystemic disease with progressive renal insufficiency due to foreign body reaction of cholesterol crystals flushed into a small vessel system of the kidneys from the arteriosclerotic plaques. The most common setting in which it occurs is iatrogenic after vascular catheterization and less frequent spontaneously. Typical clinical symptoms are delayed impairment of renal function, cutaneous manifestations such as livedo reticularis or purple toes with persistingly palpable arterial pulse, myalgia, systemic symptoms such as weight loss and fever, and abdominal and neurological symptoms. Diagnosis is generally made by clinical appearance, risk profile, and interval of time from intervention; a definitive diagnosis can only be made by renal biopsy. Even though the exact incidence is not known because most patients do not undergo biopsy due to older age, comorbidity, and other explanations for loss of renal function, it is estimated to be 4% after vascular intervention. Patient and renal outcome is dependent on comorbidity, risk profile, and preexisting chronic kidney disease (CKD). About 30% of patients are estimated to require maintenance dialysis and these patients have a high risk of death within 24 months after the first renal replacement therapy. Prognosis is also influenced by severity. The case reported is a 72‐year‐old male patient with preexisting CKD stage 3 undergoing percutaneous coronary intervention after myocardial infarction and consecutive AERD with typical clinical appearance 6 weeks after the event.     

Successful medical treatment of emphysematous pyelonephritis in chronic hemodialysis

Emphysematous pyelonephritis (EPN) is a life‐threatening renal infection caused by gas‐producing bacteria and fungi. It usually occurs in patients with diabetes and patients with urinary tract obstruction. A combination of systemic antibiotics, percutaneous catheter drainage, or open nephrectomy is typically required to achieve cure. Because of grim prognosis, resorting to interventional methods is frequently inevitable. We report the case of a 77‐year‐old woman with diabetes and end‐stage renal disease on chronic hemodialysis that presented with fever and left flank pain. A bubbly gas pattern inside the left kidney was demonstrated on abdominal computed tomography scan and blood cultures grew Escherichia coli. She was successfully treated solely with systemic antibiotics. This highlights the fact that prompt recognition of imaging findings associated with benign prognosis is essential for a favorable outcome. It allows for an effective management avoiding high‐risk interventions, especially in frail patients with multiple comorbidities. Finally, we review all published cases of EPN in chronic dialysis patients.     

Hemodialysis in a patient with severe hemophilia A and factor VIII inhibitor

Hemophilia A is a hereditary X‐linked recessive disease caused by mutations in the gene encoding factor VIII (FVIII), occurring in 1 out of 10,000 persons. Life expectancy and quality of life have dramatically improved recently in patients with hemophilia. Chronic kidney disease and need for renal replacement therapy in these patients are rare. The development of inhibitors to FVIII is the most serious complication of hemophilia and makes treatment of bleeds very challenging. We describe here a 28‐year‐old male patient with severe hemophilia A with presence of factor VIII inhibitor, who had end stage renal disease. Central venous access device was inserted along with infusion of factor eight inhibitor bypass activity before and after the procedure. He is currently on thrice weekly hemodialysis and doing well for 6 months without bleeding episodes. To our knowledge, hemophilia A with factor VIII inhibitor managed with hemodialysis has not been reported so far.     

Renal dysplasia masquerading as a renal mass in a child on hemodialysis

Pathological renal mass is uncommon in children. It is important to differentiate this from a benign mass or mass‐like lesion (pseudomass) for proper management. Renal dysplasia is a common finding in patients with end stage renal disease and can mimic a renal mass. Here, we report a 16‐year‐old girl on hemodialysis who was found to have a nodular right renal mass in the sonogram. Magnetic resonance imaging confirmed the nodular mass. She underwent right nephrectomy and the histopathology revealed features of renal dysplasia and end stage kidney disease without any evidence of malignancy. No further treatments were necessary. This case demonstrates that a nodular renal mass in dialysis patients does not always mean malignancy and could be a pseudomass from severe renal dysplasia. Since a sonogram may not be able to clearly define the etiology of solid mass in these patients, further evaluations including a renal histology and/or other imaging modalities are often necessary.     

A case of end-stage renal disease initially manifested with visual loss caused by uremic optic neuropathy

Uremic optic neuropathy (UON) is extremely rare, although sporadic cases have been reported. UON is sometimes regarded as a manifestation of uremic neuropathy. Here, we report a case of end-stage renal disease presenting as UON. A 22-year-old male was transferred to our nephrology department due to azotemia. Sudden deterioration of his vision occurred 3 days before his transfer. The patient's blood pressure was 150/90 mmHg, and he had no symptoms or signs of uremia, except for the visual disturbance. Blood tests showed an elevated serum creatinine of 6.0 mg/dL and serum BUN of 53.6 mg/dL. Both kidneys were decreased in size on ultrasound. His best-corrected vision was 20/62.5 in both the eyes. Ophthalmoscopy revealed hyperemia, swelling of both optic nerve heads, and blurred margins of both optic disks. These findings are compatible with UON. The patient's visual acuity and visual field improved following hemodialysis and corticosteroid treatment. The swelling of the patient's optic disks was also resolved. The patient is currently undergoing hemodialysis with the goal of vision restoration. Uremic optic neuropathy should be considered when patients with advanced chronic kidney disease complain of deterioration of their vision.     

Dialysis disequilibrium syndrome presenting as a focal neurological deficit

We report a patient suffering from chronic kidney disease who presented to us with severe pulmonary edema. His clinical, laboratory, and sonological parameters were suggestive of end-stage renal disease. Hemodialysis was initiated, and after 48 hours (3 sessions of hemodialysis) he became drowsy and a neurological examination revealed left upper limb monoplegia with left facial palsy. Urgent computerized tomography scan of the brain revealed diffuse hypodensity in the cerebral white matter bilaterally, and brain magnetic resonance imaging showed diffuse hyperintensity in the cerebral white matter bilaterally, right internal capsule and external capsule on fluid attenuated inversion recovery and T2 sequences (hypointense on T1 sequence). He made a gradual but complete neurological recovery and was discharged 2 weeks later with normal neurological status. A repeat brain magnetic resonance imaging on follow-up 6 weeks later revealed complete resolution of the white matter abnormalities.     

Nephrogenic systemic fibrosis: a nephrologist's perspective

Nephrogenic systemic fibrosis (NSF) is a debilitating disorder that affects patients with renal insufficiency. Recent evidence suggests that the development of NSF may be related to the administration of gadolinium-based contrast media (GBCM) in the setting of magnetic resonance imaging. As no treatment has consistently been effective in the management of NSF, strategies to prevent the development of this condition appear to be the best therapy. Identification of patients at greatest risk for developing NSF after exposure to GBCM is crucial. Risk factors include advanced chronic kidney disease (stages 4 and 5) and acute or chronic inflammatory events. The United States Food and Drug Administration has updated its public health advisory to include patients with moderate renal insufficiency (chronic kidney disease stage 3) as being at risk for developing NSF. However, these data require further verification and the vast majority of affected patients are already on renal replacement therapy. Another strategy in prevention may include consultation with a radiologist for imaging alternatives. If GBCM must be administered, immediate hemodialysis may be protective in patients already on hemodialysis; however, given the lack of data to support this, we do not recommend routine dialysis for patients not yet on dialysis or who are currently being treated with peritoneal dialysis. Decisions such as this should be made on a case by case basis after evaluating additional risk factors.     

Intensive dialysis and pregnancy

Pregnancy in women with end stage renal disease on renal replacement therapy is uncommon due to the physiologic changes associated with renal failure as well as the complexities and risk involved in managing these patients. As most of these women had long periods of illness with chronic kidney disease, the effects of their chronic illness together with the current societal trends to delay child bearing to a more advanced maternal age can hamper fertility. For those able to conceive, intensified hemodialysis (HD), through longer and/or more frequent dialysis sessions, offers improved maternal and neonatal outcomes. Intensified HD is most conveniently offered in the patient's home, where possible. This review expands the scope of the Implementing Hemodialysis in the Home website and associated supplement published previously in Hemodialysis International and includes information tailored to women of reproductive age. We describe the necessary counseling that women should receive before conception and before intensification of HD, and provide a detailed management strategy that includes nephrologic and obstetric care, should pregnancy occur.     

Poor correlation between coronary artery calcification and obstructive coronary artery disease in an end-stage renal disease patient

Vascular calcification is highly prevalent and often severe in patients with chronic kidney disease. Arterial calcification in patients with chronic kidney disease can result from the deposition of mineral along the intimal layer of arteries in conjunction with atheromatous plaques or from calcium deposition in the medial wall of arteries, also known as Monckeberg's sclerosis. Whether coronary artery calcium scores as measured by electron beam computed tomography correlate with occlusive atherosclerotic disease in the dialysis population is uncertain. Here we report a case of an asymptomatic patient with diabetes mellitus and end-stage renal disease undergoing maintenance hemodialysis, who was found to have extremely elevated coronary artery calcium scores on electron beam computed tomography, but varied degrees of atherosclerotic plaque in her coronary arteries on coronary angiography. This suggests that in addition to the calcification anticipated in a remodeled intima, a proportion of the calcification is also likely to be in the arterial media. Thus, this case demonstrates that even an extremely high coronary calcium score may not be a satisfactory surrogate marker for obstructive atherosclerosis in elderly diabetic dialysis patients.     

SPECT MIBI imaging for cardiac output and index in end stage renal disease

To compare cardiac output (CO) and cardiac index (CI) and left ventricular ejection fraction (LVEF) in end-stage renal disease (ESRD) with a control group using gated single photon emission computed tomography (SPECT)/computed tomography (CT) imaging. Altered cardiovascular function with increased CO secondary to arterio-venous fistulas (AVF) for dialysis has been reported in patients with ESRD. Thirty-two patients (18 with AVF or graft) referred for pre-renal transplant cardiac assessment using SPECT/CT were studied with 2 comparison groups, 42 normal weight (body mass index<30) and 46 obese (body mass index>30) patients. End-stage renal disease patients had overall reduced mean hemoglobin 11.6 mg/dL and elevated mean parathyroid hormone of 396 pg/mL. Gated SPECT using MIBI was performed after Bruce protocol apart from 4 renal patients who underwent cardiac stressing with adenosine. Cardiac output was calculated by product of stroke volume and resting heart rate and CI determined. Mean CI was 2.6 L/min/m(2) for renal disease group compared with 2.2 and 2.3 L/min/m(2) for the normal weight and obese groups, P=0.005 and 0.005 respectively (Wilcoxon's rank test). Cardiac output was increased for the renal group; 4.9 L/min, equal to the obese group but greater than normal weight group at 4.3 L/min. No significant difference in LVEF was seen between the 3 patient groups. No significant difference in CI or output was seen between the renal disease patients with AVF and those without fistulas. Cardiac ouput and CI, assessed using SPECT/CT, are increased in patients with ESRD. This may be independent of the presence of AVF or grafts and other factors such as anemia and hyperparathyroidism may contribute to this high output cardiac function. As LVEF is not increased for these patients, increased heart rate, may also contribute to elevated CO.     

Hepatitis C virus infection in patients with end‐stage renal disease

Chronic hepatitis C virus (HCV) infection is a major global health problem affecting 3–5 million people in the United States and over 100 million worldwide. Chronic HCV infection, which can lead to cirrhosis and hepatocellular carcinoma, also results in numerous other complications, including impairment of renal function. Because HCV is most often transmitted via parenteral exposure to blood or blood products, patients with end‐stage renal disease (ESRD) treated with hemodialysis are at particular risk for infection. Historically, the medications available to treat HCV infection in these patients had significant side effects and were not particularly effective in generating a sustained virologic response. Since 2011, a number of direct‐acting antiviral therapies have emerged that can lead to virological cure in the vast majority of patients, with low pill burden and few side effects. Here, we describe the biology and pathophysiology of HCV infection, and summarize current information on new therapies, with a particular focus on their application in patients with chronic kidney disease including ESRD.     

Status epilepticus induced by putrid salted gray mullet fish intoxication in patients with chronic kidney disease

Neurological and psychiatric symptoms are common presentations, but are often ignored in fresh salted gray Mullet fish intoxication. We report 2 patients with chronic renal failure at a predialyzed stage who developed refractory status epilepticus after ingestion of putrid salted gray Mullet. We warn consultant neurologists that fresh salted gray Mullet fish intoxication must be considered when patients with chronic kidney disease present with seizures or other unexplained neurological or psychiatric symptoms.     

Rationale and study design of a three‐period, 58‐week trial of ferric citrate as a phosphate binder in patients with ESRD on dialysis

Chronic kidney disease associated mineral and bone disorders arise as a result of aberrant bone mineral metabolism in patients with advancing levels of renal dysfunction and end‐stage renal disease. One of the cornerstones of treatment is the use of phosphate‐binding agents. We describe the rationale and study design for a clinical trial to assess the safety and efficacy of ferric citrate as a phosphate binder. This trial is a three‐period, international, multicenter, randomized, controlled clinical trial to assess the safety and efficacy of ferric citrate as a phosphate binder, consisting of a 2‐week washout period, a 52‐week safety assessment period in which subjects are randomized to ferric citrate or active control, and a 4‐week efficacy assessment period in which subjects randomized to ferric citrate in the safety assessment period are randomized to ferric citrate or placebo. Eligible subjects include end‐stage renal disease patients who have been treated with thrice‐weekly hemodialysis or peritoneal dialysis for at least 3 months in dialysis clinics in the United States and Israel. Primary outcome measure will be the effect of ferric citrate vs. placebo on the change in serum phosphorus. Safety assessments will be performed by monitoring adverse events, concomitant medication use, and sequential blood chemistries (including iron parameters, phosphorus, and calcium). This three‐period trial will assess the efficacy of ferric citrate as a phosphate binder. If proven safe and efficacious, ferric citrate will likely provide an additional phosphate binder to treat chronic kidney disease associated mineral and bone disorders.     

Recent advances in CKD and ESRD: A literature update

The past year has seen interesting publications in the fields of chronic kidney disease and end stage renal disease. This review highlights some of these important papers and places their findings in the context of clinical care.     

Hemodialysis‐associated soft tissue amyloidomas of the chest and abdominal wall

Amyloidoma is a highly unusual presentation of amyloidosis in tumoral or nodular form. Isolated soft tissue amyloidomas in individuals with end‐stage renal disease on chronic hemodialysis is exceedingly rare, particularly in the era of advanced dialysis technologies. We report the case of a 55‐year‐old male with end‐stage renal disease due to autosomal‐dominant polycystic kidney disease, on HD for over 30 years, who was found to have soft‐tissue, dialysis‐related (β₂‐microglobulin) amyloidomas (DRA). He presented with painful, palpable masses within the thoracic and abdominal walls. Serum β₂‐microglobulin level was only mildly elevated at 24.9 mg/L. Biopsy confirmed amyloidosis with positivity for Congo Red staining and apple‐green birefringence under polarized light. Amyloid subtyping with immunohistochemistry showed positive β₂‐microglobulin staining within the deposits. Conservative therapy involving pain management and close monitoring resulted in eventual improvement in symptoms and thus proved to be a viable option for treatment.     

Risk of major depression in patients with chronic renal failure on different treatment modalities: A matched‐cohort and population‐based study in Taiwan

The influence of different treatment modalities on the risk of developing major depression in patients with chronic renal failure (CRF) is not well understood. We aimed to explore the incidence of major depression among patients with CRF who were on different dialysis modalities, who had received renal transplantation (RT), and those who had not yet received any of the aforementioned renal replacement therapies. We conducted a population‐based retrospective cohort study using a national health insurance research database. This study investigated 89,336 study controls, 17,889 patients with chronic kidney disease on conservative treatment, 3823 patients on hemodialysis (HD), 351 patients on peritoneal dialysis (PD), and 322 patients who had RT. We followed all individuals until the occurrence of major depression or the date of loss to follow‐up. The PD group had the highest risk (hazard ratio [HR] 2.43; 95% confidence interval [CI] 1.26–4.69), whereas the RT group had the lowest risk (HR 0.18; 95% CI 0.03–1.29) of developing major depression compared with the control group. Patients initiated on PD had a higher risk of developing major depression than patients initiated on HD (pairwise comparison: HR 2.20; 95% CI 1.09–4.46). Different treatment modalities are associated with different risks of developing major depression in patients with CRF. Among renal replacement therapies, patients who have had RT have the lowest risk of developing major depression. Patients who initiate renal therapy on PD may have a higher risk of major depression compared with patients who initiate renal therapy on HD.     

Acute renal failure and chronic kidney disease following liver transplantation

Acute renal failure (ARF) and chronic kidney disease (CKD) are common complications after liver transplantation (LTx). The incidence of ARF post-LTx varies between 48% and 94%; 8% to 17% of patients require renal replacement therapy (RRT). The most common cause of ARF early after LTx is ischemic acute tubular necrosis, followed later by cyclosporine toxicity and sepsis. Preoperative serum creatinine >1.5 mg/dL and early hepatic allograft dysfunction are risk factors for the occurrence of postoperative ARF. Of patients with ARF due to the hepatorenal syndrome, approximately two-thirds will recover, although recovery may be delayed 3 months or longer after LTx. Mortality after LTx is affected modestly by the presence of ARF pretransplant (<2-fold increase), but increases markedly (up to 8-fold) in the face of ARF posttransplant. Mortality does not appear to be influenced by the mode of RRT used. The risk of CKD after LTx is approximately 18% at 5 years and increases to approximately 25% by 10 years after transplantation. Calcineurin inhibitor toxicity is the most common cause. Specific prognosticators for predicting CKD after LTx are presently lacking. The occurrence of CKD after LTx markedly impairs long-term survival.     

Hyponatremia in patients with chronic kidney disease

Hyponatremia is a common condition encountered in clinical practice. A number of studies have associated low serum sodium levels with increased mortality in various patient populations, such as hospitalized patients and patients with various comorbid conditions; recent studies have shown that individuals with chronic kidney disease also are afflicted by hyponatremia. However, few studies have focused on patients with hemodialysis. Evidence supporting the incidence and prevalence of hyponatremia, clinical characteristics and the association with patient outcomes with hemodialysis is limited. In the present review, we examined the physiology and pathophysiology of water and sodium balance with a special emphasis on changes occurring during end‐stage renal disease. The outcomes in patients undergoing hemodialysis were associated with low serum sodium. We evaluated the associations between hyponatremia and mineral bone abnormalities and discussed the elevated incidence and prevalence of difficult clinical outcomes associated with hyponatremia. We also provided specific recommendations for hemodialysis treatment in hyponatremic patients.