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.     

Coronavirus disease 2019 (COVID-19) in two pediatric patients with kidney disease on chronic immunosuppression: A case series

Coronavirus disease 2019 (COVID-19) is a highly infectious disease caused by the severe acute respiratory syndrome coronavirus 2 virus (SARS-CoV-2). While children appear to experience less severe disease than adults, those with underlying conditions such as kidney disease may be more susceptible to infection. Limited data are present for children with kidney disease, and there are limited prior reports of pediatric hemodialysis patients with COVID-19. This report describes the mild clinical disease course of COVID-19 in two pediatric patients with chronic kidney disease, one on hemodialysis and both on chronic immunosuppression. We review treatment in these patients, as well as our measures to reduce transmission among our hemodialysis patients and staff.     

What Went Wrong with Home Hemodialysis in the United States and What Can Be Done Now?

In 1973, almost 40% of the more than 10 000 dialysis patients were treated by home hemodialysis. Today, with more than a quarter of a million dialysis patients in the United States, fewer than 2000 are on home hemodialysis. A number of factors have contributed to this change. First, many nephrologists and administrators who were developing new dialysis units had little or no practical experience with dialysis for chronic renal failure. Second, more elderly and diabetic patients were admitted to treatment. Home hemodialysis was more difficult for such patients, and often their helpers were themselves were elderly. Third, hemodialysis machines were difficult to learn and operate. Fourth, following publication of the results of the National Cooperative Dialysis Study, there developed the erroneous concept that a Kt/V equal to 1.0 was “adequate dialysis.” As bigger dialyzers became available, there was a widespread shortening of dialysis time. This decrease in time was embraced by for‐profit dialysis facilities and inadequately educated patients, and assembly‐line dialysis became generally accepted. Finally, continuous ambulatory peritoneal dialysis, with its simplicity and short training time, began to fill the need of many patients for home dialysis and independence, at least temporarily. Fortunately, the trend is now reversing. Two developments clearly have benefits for home hemodialysis. The first is an increasing interest in the use of more frequent dialysis. The second is the development of new equipment designed specifically for use by the patient, and requiring a minimum of effort on the patient's part.     

Gustatory sweating recurring on peritoneal dialysis but resolving during periods of hemodialysis

Gustatory sweating is a rare disorder characterized by profuse sweating on the forehead, face, scalp, and neck occurring soon after ingesting food, which has been reported in diabetic patients. The mechanism is thought to be triggered by taste buds and not gastric stimulation. We report a case where gustatory sweating repeatedly developed on peritoneal dialysis that resolved on periods of hemodialysis. A 32-year-old woman with diabetic end-stage renal disease developed gustatory sweating shortly after beginning continuous ambulatory peritoneal dialysis despite excellent clearances. After 5 months, she changed to hemodialysis for 2 months and noticed complete resolution of her gustatory sweating; however, after her return to peritoneal dialysis 2 months later, her gustatory sweating recurred. While on peritoneal dialysis, she was treated with clonidine, which resulted in improvement but not resolution of her symptoms as had occurred on hemodialysis. Another period on hemodialysis resulted in the resolution of her symptoms that returned again after restarting peritoneal dialysis. Clonidine provided incomplete relief while topical glycopyrrolate was effective and without complications. We report recurrent gustatory sweating on peritoneal dialysis that resolved with hemodialysis. We have no data to suggest that intra-abdominal stimulation played a role, but rather that despite excellent clearances neuropathy may have played a role. Treatment with topical glycopyrrolate may be safe and effective given every third day if clonidine is ineffective.     

Short and long nightly hemodialysis in the United States

When hemodialysis first started in the United States in the 1960s, a large percentage of patients performed their treatments at home. However, because of reimbursement issues, home hemodialysis (HHD) gradually succumbed to an in-center approach and eventually a mindset. Since the introduction of nightly HHD by Uldall and Pierratos in 1993, there has been a resurgence of interest in HHD. This paper describes the different types of home hemodialysis being performed as of December 31, 2007 in this country. Because neither the United States Renal Data System (USRDS) nor the End Stage Renal Disease (ESRD) Networks break down home dialysis into the different modalities, a provider questionnaire was sent out to 2 major providers, a number of mid-level providers and other providers known to do HHD. In addition, a questionnaire was sent out to 3 machine providers to obtain the number of patients using their machine for HHD as of December 31, 2007. The results showed that 91.7% of patients are dialyzing in-center, 7.3% are doing peritoneal dialysis, and 0.7% are doing HHD. Currently about 1% of ESRD patients in the United States are doing home hemodialysis. NxStage, however, has started 1000 patients in the past year on short-daily home hemodialysis. Patients are beginning to understand that there are better options than 3 times a week in-center dialysis. And as a result of the "HEMO Study," nephrologists now believe that longer and more frequent dialysis is a better therapy for ESRD patients. Therefore, promotion of HHD should become a priority for the renal community in the future.     

New-onset psoriasis in a maintenance hemodialysis patient

New-onset psoriasis is extremely rare in hemodialysis (HD) patients, and several trials of dialysis therapies (HD and peritoneal dialysis) in psoriasis have indicated remarkable improvement in skin lesions and well-being even in patients without renal impairment. We describe a patient who developed severe psoriasis despite undergoing chronic maintenance hemodialysis for 5 years and was treated successfully with oral cyclosporin A.     

Mycobacterium fortuitum infection of a hemodialysis catheter in a pediatric patient

This case report discusses a pediatric patient who developed a hemodialysis catheter line infection from an uncommon etiology, Mycobacterium fortuitum. The initial presentation revealed a well appearing patient with a slow growing skin lesion near the site of the hemodialysis catheter. The treatment course was complicated by resistance to initial antibiotics leading to continued spread of the lesion. The diagnosis was confirmed via skin biopsy of the lesion that required 2 weeks to grow the atypical Mycobacterium. Treatment was successful after hemodialysis catheter removal, transition to peritoneal dialysis, and a prolonged antibiotic course. Mycobacterium fortuitum is a rare cause of dialysis catheter infections that is resistant to standard antibiotic treatment. Treatment success is improved after removal of the dialysis catheter and prolonged antibiotics based on susceptibilities. This case highlights the importance of keeping atypical Mycobacterium in the differential for patients with slow growing skin lesions near dialysis catheter sites with resistance to initial treatment.     

Recurrent Ochrobactrum anthropi and Shewanella putrefaciens bloodstream infection complicating hemodialysis

Bloodstream infections (BSIs) are common in hemodialysis, especially when the access is a catheter. These infections are more commonly gram-positive bacteria or gram-negative bacilli and on some occasions, fungi. Ochrobactrum anthropi and Shewanella putrefaciens are ubiquitous hydrophilic gram-negative bacilli. There have been three cases of O. anthropi BSI reported in hemodialysis patients (one from the United States and two from Vienna) and two cases of S. putrefaciens BSI in hemodialysis patients (one from the United States and the other from Japan). There have been few more cases reported of infections with these bacteria in peritoneal dialysis, especially outside the United States. We present a novel case of a patient with both recurrent O. anthropi and S. putrefaciens BSI complicating hemodialysis. There have been no reports in the literature of such a case. We also discuss the microbiology, clinical features, and the challenging aspects of treatment of such infections.     

Peritoneal Dialysis and Hemodialysis in Pregnancy

Management of women who require dialysis during pregnancy is an unusual and challenging clinical problem for both the nephrologist and the obstetrician. More than 200 pregnancies have been reported in women requiring hemodialysis, and more than 50 pregnancies in women on peritoneal dialysis. Surveys from several countries of pregnancy in dialysis patients provide data regarding management and outcomes. Conception rates are significantly lower in women on dialysis, and when these women do become pregnant, the spontaneous abortion rate usually exceeds 40%. However, in the pregnancies that continue, as many as 71% result in surviving infants. Pregnant women require longer, more intense hemodialysis, and enough experience has been reported to support the use of peritoneal dialysis as well as hemodialysis during pregnancy. The patients typically experience anemia, hypertension, and premature delivery. Most infants are small for gestational age, and the stillbirth rate is as high as 12%. While the potential problems are many, sufficient data has been accumulated to provide clinicians with guidelines to help them manage pregnant women on dialysis and to achieve successful outcomes.     

Sexual function in women receiving maintenance dialysis

While substantial attention has been paid to the issue of sexual dysfunction in men on chronic dialysis, less is known about this problem in women with end-stage renal disease. We sought to assess sexual dysfunction in women on chronic dialysis and determine whether patients discuss this problem with their providers and receive treatment. We prospectively enrolled women receiving chronic hemodialysis or peritoneal dialysis in Pittsburgh, PA. We asked patients to complete the 19-item Female Sexual Function Index (FSFI) to assess sexual function and a 5-item survey that assessed whether patients had discussed sexual dysfunction with their providers and/or received treatment for this problem in the past. We enrolled 66 patients; 59 (89%) on hemodialysis and 7 (11%) on peritoneal dialysis. All patients completed the FSFI, of whom 53 (80%) had FSFI scores <26.55, consistent with the presence of sexual dysfunction. Of 37 patients who were married or residing with a significant other, 27 (73%) had sexual dysfunction. Among 24 participants who reported having been sexually active over the previous 4 weeks, 11 (46%) had sexual dysfunction. Only 21% of patients with sexual dysfunction had discussed this problem with their gynecologist, renal or primary provider, and 3 (6%) reported having received treatment. Sexual dysfunction is common in women on dialysis, even among patients who are married or residing with a significant other and those who are sexually active. However, few women discuss this issue with their providers or receive treatment.     

Portable and wearable dialysis: where are we now?

Although dialysis is a life-saving treatment for patients with acute and chronic kidney disease, mortality remains high, with the survival of patients treated by regular hemodialysis similar to that of some solid organ tumors. Recent reports have suggested that a major increase in the dose of dialysis, delivered by frequent nocturnal dialysis, may improve survival. Unfortunately, only a minority of centers can offer this type of therapy, and only to a minority of their patients. Thus, to improve access to dialysis as well as increase the delivered dose of dialysis, a major change in the current paradigm of dialysis delivery is required. For many years, the "holy grail" of dialysis has been to develop a wearable or portable system, allowing patients to be treated while performing their normal activities of daily living. It is only recently with the advances in technology that such dialysis devices have been possible. Prototype devices for both hemodialysis and peritoneal dialysis have been studied with favorable results. Typically, these have been short-term studies, and longer term trials are eagerly awaited, to determine whether the current generation of wearable continuous dialysis devices cannot only remove waste products of metabolism and control volume but also maintain acid-base and electrolyte homeostasis and actually improve outcomes. In addition, a novel generation of dialysis devices based on nanotechnologies are being developed. Hopefully, these wearable continuous devices will be available as an option for routine clinical practice in the not-too-distant future.     

Role of peritoneal dialysis in the era of the resurgence of home hemodialysis

In recent years, there has been a resurgence of home hemodialysis (HHD) therapies. Given the small percentage of prevalent patients in the United States currently on home dialysis, an appropriate question is: What is the role of peritoneal dialysis (PD) in this era? Data suggest that in centers that are promoting the growth of HHD, their PD programs also tend to be very active. Furthermore, our experience and other data suggest that one should not worry about cannibalizing PD in order to grow HHD. Most HHD patients come from in‐center hemodialysis or those patients transitioning from PD to another therapy. In fact, data suggest that in order to promote the growth of HHD, a certain minimal infrastructure is needed in terms of staff. An active PD program not only supports a robust infrastructure that allows for HHD growth but also fosters profitability of a home program.     

Is there any interaction of resistin and adiponectin levels with protein‐energy wasting among patients with chronic kidney disease

The aim of this study was to evaluate the effects of adipocytokines including adiponectin, leptin, resistin, neuropeptide Y and ghrelin in chronic kidney disease (CKD) patients on appearance of protein‐energy wasting (PEW). One hundred fifty patients with mean age of 45.4 ± 15.9 years, without active infections or chronic inflammatory conditions were recruited into the study. Study groups were control group (consisting of 30 healthy volunteers with normal kidney functions), hemodialysis group, predialysis group, peritoneal dialysis group and kidney transplant group. Fasting morning serum leptin, ghrelin, acylated ghrelin, neuropeptide Y, adiponectin, resistin levels of all of the groups were measured. Anthropometric and nutritional assessments of all patients were obtained. Diagnosis of PEW was made according to definition recommended by the International Society of Renal Nutrition and Metabolism. Presence of PEW in hemodialysis (23.3%) and peritoneal dialysis (26.7%) groups were significantly higher than those of predialysis (3.3%), and transplantation (0%) groups. Adiponectin and resistin levels in predialysis, peritoneal dialysis and hemodialysis patients were significantly higher than control group (p: 0.0001). This study had given significant positive correlations between presence of PEW and serum resistin (r: 0.267, p: 0.001), and serum adiponectin levels (r: 0.349, p: 0.0001). There were no relationship between presence of PEW and ghrelin, acylated‐ghrelin, neuropeptide Y, and leptin levels of the groups. CKD patients except transplant patients had higher adiponectin and resistin levels than control group. PEW was found to be linearly correlated with resistin and adiponectin. High serum resistin and adiponectin levels might have a role in development of PEW among dialysis patients.     

Central Venous Catheter Access in Children on HD

Central venous catheters (CVC) remain a frequently used form of vascular access in children receiving chronic hemodialysis (HD). Whereas standard dual-lumen catheters (DL) are used in many centers, the Tesio catheter has proven to be superior to the DL catheter in terms of catheter survival and infection rates in at least one pediatric study (Sheth RD et al . Am J Kidney Dis. 2001). Recently, the Ash Split catheter became available which, while similar to the Tesio internally, being comprised of 2 separate catheters, may be preferable because of the requirement for only one skin puncture for placement vs. two for the Tesio. However, no pediatric data pertaining to the Ash Split catheter have been published to date. Therefore, we reviewed and compared our experience with a small number of Ash Split (# 16) and Tesio (# 8) catheters used for semipermanent vascular access in our chronic HD population with reference to survival, infectious complications, patency, and dialysis adequacy. The results of this experience are as follows:  

     

Early,Frequent, and Efficient Hemodialysis: A New Trend or Déjà Vu From the 1970s?

After more than a quarter century of dialysis, two factors are still present in dialysis treatment of chronic renal failure patients: inadequacy of technology (the artificial kidney acts as an artificial glomerulus) and inadequate use of technology in terms of dialysis initiation and frequency. This paper presents the results of two less unphysiological dialysis programs, introduced in Bologna at the beginning of the 1960s, which proved their clinical value and are now becoming trendy, at the end of this century. Features of these programs are twofold: (1) daily dialysis, which aims at making treatment more biologically suited to the patient; its validity relies on lower intra- and interdialytic osmotic fluctuations; (2) early dialysis, which aims at making the patient more biologically suited to the treatment. After more than 25 years it is evident that this treatment has fulfilled its original expectations versus late dialysis. There is a 40% improvement in survival, a 35% decrease in morbidity, and a 24% improvement in the cost/benefit ratio. This report is based on a retrospective analysis of our overall experience and clinical results of chronic hemodialysis carried out in 224 patients on early dialysis and 1210 patients on late dialysis in Bologna from 1967 to 1997. Based on this experience, the following should be regarded as particularly important indications for early dialysis: adequate dialysis facilities; symptomatic patients despite renal creatinine clearances between 15 and 20 mL/min; patients unable to comply with dietary measures; children, to allow for adequate development; patients with diabetes mellitus; candidates for renal transplantation.     

Severe hypertriglyceridemia in an infant on chronic hemodialysis

Severe hyperlipidemia is a risk factor for cardiovascular disease. Children with chronic kidney disease and end stage renal disease are at risk for development of hyperlipidemia. In this report, we describe a 7-month-old male infant with Denys–Drash syndrome who was found to have a “milky-layer” floating on the deaerator of the hemodialysis machine. Investigations showed severe hypertriglyceridemia of >1000 mg/dl. The patient had been on chronic continuous manual peritoneal dialysis until 6 months of age and recently had been switched to hemodialysis. Management included lowering of caloric intake and addition of medium chain triglyceride with reduction of the serum triglyceride levels to 300–400 mg/dl. Close monitoring of serum lipids and timely intervention is important to prevent serious complications associated with dyslipidemia. Observation of the “milky layer” in the deaerator of the hemodialysis machine may be an interesting visual clue of underlying severe hypertriglyceridemia.     

Impact of glucose levels on advanced glycation end products in hemodialysis

The current obesity epidemic throughout the western world has resulted in a considerable increase in the condition Type II diabetes mellitus. Recently, the World Health Organization has predicted that the global prevalence of Type II will increase from 175 million patients in 2003 to over 350 million by 2030. One of the major consequences of this disorder is renal failure, which presents itself as chronic kidney disease, and can progress to end-stage renal disease. Once diagnosed, patients are generally treated using dialysis due to a shortage of kidney donors. The fundamental process of dialysis still requires improvement because the survival rate of these patients is relatively poor. This has resulted in considerable research into improvements in hemodialysis membranes, and the challenge to find more suitable marker(s) in assessing the efficacy of the dialysis process. A class of compounds highlighted as a possible accumulative toxin is advanced glycation end products or AGEs. This is an article regarding the impact of hemodialysis and hemodiafiltration on glucose and AGE levels within the body and the consequences of a chronic hyperglycemic condition. It also highlights the negative aspects of using dextrose in conventional dialysis solutions (an area that has already been identified by peritoneal dialysis clinicians as problematic). The review concludes by suggesting several possible topics of future research.     

Insights into nephrologist training, clinical practice, and dialysis choice

There is variable emphasis on dialysis-specific training among US nephrology fellowship programs. Our study objective was to determine the association between nephrology training experience and subsequent clinical practice. We conducted a national survey of clinical nephrologists using a fax-back survey distributed between March 8, 2010 and April 30, 2010 (N = 629). The survey assessed the time distribution of clinical practice, self-assessment of preparedness to provide care for dialysis patients at the time of certification examination, distribution of dialysis modality among patients, and nephrologists' choice of dialysis modality for themselves if their kidneys failed. While respondents spent 28% of their time caring for dialysis patients, 38% recalled not feeling very well prepared to care for dialysis patients when taking the nephrology certification examination. Sixteen percent obtained additional dialysis training after fellowship completion. Only 8% of US dialysis patients use home dialysis; physicians very well prepared to care for dialysis patients at the time of certification or who obtained additional dialysis training were significantly more likely to provide care to home peritoneal dialysis patients. Even though 92% of US dialysis patients receive thrice weekly in-center hemodialysis, only 6% of nephrologists selected this for themselves; selection of therapy for self was associated with dialysis modalities used by their patients. Nephrology training programs need to ensure that all trainees are very well prepared to care for dialysis patients, as this is central to nephrology practice. Utilization of dialysis therapies other than standard hemodialysis is dependent, in part, on training experience.