Absence Of End-stage Renal Disease Research Articles

Clinical Features of Cardiac Calcified Amorphous Tumor: A Narrative Review.

A cardiac calcified amorphous tumor (CAT) is a non-neoplastic cardiac mass composed of calcified nodules surrounded by amorphous fibrous tissue in a context of degeneration and chronic inflammation. Although CAT cases are increasingly reported, its clinical manifestation remains unclear. We aimed to conduct a narrative review of CAT and identify its clinical characteristics. We conducted a comprehensive literature search using PubMed, with the keyword "Cardiac Calcified Amorphous Tumor" to identify relevant articles. A total of 113 articles published between 1997 and 2022 were retrieved. The clinical features allowing for assess patient background, differences with and without end-stage renal disease (ESRD), symptom-related factors, risk factors for embolism caused by CAT, and features of CAT, such as shape, location, mobility, pathology, and treatment, were statistically analyzed in 106 criteria-matched cases. The mean patient age was 60.2 ± 18.6 years, with 45 men and 61 women. Of the data collected from 21 countries, 52 patients were from Japan. Mobile CAT was more common in the chronic renal failure group. Linear, club-shaped, and spindle-shaped CAT tended to be mobile and more common in the ESRD group as well as located in the mitral valve region. The shape, mobility, location, and underlying mechanism of CAT depended on the presence or absence of ESRD. The risk of embolism was considered high because mobile CAT was more common in the ESRD group, and CAT originated in the mitral valve region. Hence, early diagnosis based on periodic examination in patients with ESRD and aggressive surgical treatment are necessary.

A Comparative Analysis of Bleeding Peptic Ulcers in Hospitalizations With and Without End-Stage Renal Disease.

End-stage renal disease (ESRD) patients are highly susceptible to peptic ulcer bleeding (PUB). We aimed to assess the influence of ESRD status on PUB hospitalizations in the United States (USA). We analyzed the National Inpatient Sample to identify all adult PUB hospitalizations in the USA from 2007 to 2014, which were divided into two subgroups based on the presence or absence of ESRD. Hospitalization characteristics and clinical outcomes were compared. Furthermore, predictors of inpatient mortality for PUB hospitalizations with ESRD were identified. Between 2007 and 2014, there were 351,965 PUB hospitalizations with ESRD compared to 2,037,037 non-ESRD PUB hospitalizations. PUB ESRD hospitalizations had a higher mean age (71.6 vs. 63.6 years, P < 0.001), and proportion of ethnic minorities i.e., Blacks, Hispanics, and Asians compared to the non-ESRD cohort. We also noted higher all-cause inpatient mortality (5.4% vs. 2.6%, P < 0.001), rates of esophagogastroduodenoscopy (EGD) (20.7% vs. 19.1%, P < 0.001), and mean length of stay (LOS) (8.2 vs. 6 days, P < 0.001) for PUB ESRD hospitalizations compared to the non-ESRD cohort. After multivariate logistic regression analysis, Whites with ESRD had higher odds of mortality from PUB compared to Blacks. Furthermore, the odds of inpatient mortality from PUB decreased by 0.6% for every 1-year increase in age for hospitalizations with ESRD. Compared to the 2011 - 2014 study period, the 2007 - 2010 period had 43.7% higher odds (odds ratio (OR): 0.696, 95% confidence interval (CI): 0.645 - 0.751) of inpatient mortality for PUB hospitalizations with ESRD. PUB hospitalizations with ESRD had higher inpatient mortality, EGD utilization, and mean LOS compared to non-ESRD PUB hospitalizations.

Sepsis in end-stage renal disease patients: are they at an increased risk of mortality?

Objectives This study aims to examine the outcome of end-stage renal disease (ESRD) patients admitted with sepsis to the intensive care unit (ICU). Design Single centre, retrospective cohort study Setting The study was conducted in the Intensive Care Department of King Abdulaziz Medical City, Riyadh, Saudi Arabia. Participants Data were extracted from a prospectively collected ICU database from 2002 to 2017. Patients were considered to have sepsis based on the sepsis-3 definition and were stratified into 2 groups based on the presence or absence of ESRD. Primary and secondary outcomes The primary outcome of the study was in-hospital mortality. Secondary outcomes included ICU mortality, ICU and hospital lengths of stay, and mechanical ventilation duration. Results A total of 8803 patients were admitted to the ICU with sepsis during the study period. 730 (8.3%) patients had ESRD. 49.04% of ESRD patients with sepsis died within their hospital stay vs. 31.78% of non-ESRD patients. ESRD septic patients had 1.44 greater odds of dying within their hospital stay as compared to septic non-ESRD patients (OR 1.44, 95% CI 1.03–1.53). Finally, the predictors of hospital mortality in septic ESRD patients were found to be mechanical ventilation (OR 3.36; 95% CI 2.27–5.00), a history of chronic liver disease (OR 2.26; 95% CI 1.26–4.07), and use of vasopressors (OR 1.74; 95% CI 1.19–2.54). Among patients with ESRD, hospital mortality was higher in subgroups of patients with chronic cardiac (OR 1.86 (1.36–2.53) vs. 1.19 (0.96–1.47)) and chronic respiratory illnesses (OR 2.20 (1.52–3.20) vs. 1.21 (0.99–1.48)). Conclusion ESRD patients admitted to the intensive care unit with sepsis are at greater odds of mortality compared to patients with non-ESRD. This risk is particularly increased if these patients have a concomitant history of chronic cardiac and respiratory illnesses. Key Messages Sepsis and bacterial infections are very common in ESRD patients and following cardiovascular disease; sepsis is the second leading cause of death in patients with ESRD. This study aims to examine the outcome of patients with end-stage renal disease (ESRD) patients admitted with sepsis to the intensive care unit (ICU). The results of this study have shown that end-stage renal disease is associated with greater odds of ICU and hospital mortality among septic patients admitted to an intensive care unit. ESRD patients were also more likely to be started on vasopressors and mechanical ventilation.

Outcomes of isolated inframalleolar interventions for chronic limb-threatening ischemia in diabetic patients

BackgroundInframalleolar disease is present in many diabetic patients presenting with tissue loss. The aim of this study was to examine the patient-centered outcomes after isolated inframalleolar interventions. MethodsA database of patients undergoing lower extremity endovascular interventions for tissue loss (critical limb-threatening ischemia, Wound, Ischemia, and foot Infection [WIfI] stage 1-3) and a de novo intervention on the index limb between 2007 and 2017 was retrospectively queried. Those patients with isolated inframalleolar interventions on the dorsalis pedis and medial and lateral tarsal arteries were identified. Patients with concomitant superficial femoral artery and tibial interventions were excluded. Intention-to-treat analysis by patient was performed. Patient-oriented outcomes of clinical efficacy (absence of recurrent symptoms, maintenance of ambulation, and absence of major amputation), amputation-free survival (AFS; survival without major amputation), and freedom from major adverse limb events (above-ankle amputation of the index limb or major reintervention [new bypass graft, jump or interposition graft revision]) were evaluated. ResultsThere were 109 patients (48% male; average age, 65 years; 153 vessels) who underwent isolated inframalleolar interventions for tissue loss. All patients had diabetes, and 53% had chronic renal insufficiency (47% of these were on hemodialysis). The majority of the patients had WIfI stage 3 disease. Technical success was 81%, with a median of one vessel treated per patient. Thirty-four percent of interventions were a direct revascularization of the intended angiosome in the foot. The 30-day major adverse cardiovascular event rate was 0%. The majority of patients underwent some form of planned forefoot surgery (single digit, multiple digits, ray or transmetatarsal amputation). Wound healing at 3 months in those not requiring amputation was 76%. Predictors for wound healing were improved pedal runoff score (<7), absence of infection, direct angiosome revascularization, and absence of end-stage renal disease. Those in whom the primary wounds or the initial amputation site failed to heal ultimately underwent below-knee amputations. The clinical efficacy was 25% ± 7% (mean ± standard error of the mean) at 5 years. The 5-year AFS rate was 33% ± 8%, and the 5-year freedom from major adverse limb events was 27% ± 9%. On Cox proportional multivariate analysis, predictors for AFS were absence of significant coronary disease, postprocedure pedal runoff score <7 (good runoff), WIfI stage <3, and absence of end-stage renal disease. ConclusionsInframalleolar intervention can be successfully performed in high-risk limbs with acceptable short-term results. However, long-term AFS remains poor because of the underlying disease process.

VESS03. Outcomes of Isolated Inframalleolar (Pedal Artery) Interventions for Limb-Threatening Critical Ischemia

Inframalleolar (pedal) artery disease has been demonstrated to be a predictor of limb salvage and wound healing in this cohort of patients. The aim of this study was to examine the patient-centered outcomes after isolated inframalleolar (pedal artery) interventions. A database of patients undergoing lower extremity endovascular interventions for tissue loss between 2007 and 2017 was retrospectively queried. Patients with tissue loss (Rutherford 5 and 6) were selected, and those undergoing isolated inframalleolar intervention on the dorsalis pedis and medial and lateral tarsal arteries were identified. Patients with concomitant superficial femoral artery and tibial interventions were excluded. Intention-to-treat analysis by patient was performed. Patient-oriented outcomes of clinical efficacy (absence of recurrent symptoms, maintenance of ambulation, and absence of major amputation), amputation-free survival (survival without major amputation), and freedom from major adverse limb events (above-ankle amputation of the index limb or major reintervention [new bypass graft, jump/interposition graft revision]) were evaluated. There were 109 patients (48% male; average age, 65 years; 153 vessels) who underwent isolated inframalleolar intervention for tissue loss. All patients had diabetes, 94% had hypertension, 63% had hyperlipidemia, and 53% had chronic renal insufficiency (47% of these were on hemodialysis). The majority of the patients had Wound, Ischemia, and foot Infection (WIfI) stage 3 disease. Technical success was 81%, with a median of one vessel treated per patient and a mean preoperative pedal runoff score of 12 that improved to a mean of 6 after intervention (<7 is considered good pedal runoff); 34% of interventions were a direct revascularization of the intended angiosome. Overall major adverse cardiovascular event rate was <1% at 90 days after the procedure. The majority of patients underwent some form of planned forefoot amputations (single digit, multiple digits, ray amputation, or transmetatarsal amputation). Wound healing at 3 months was 76%. Those who failed to heal underwent below-knee amputations. The clinical efficacy was 25% ± 7% (mean ± standard error of the mean) at 5 years. The 5-year amputation-free survival rate was 33% ± 8%, and the 5-year major adverse limb event rate was 27% ± 9%. On Cox proportional multivariate analysis, predictors for amputation-free survival were absence of significant coronary disease, a pedal runoff <7, WIfI stage 3 disease, and absence of end-stage renal disease. Predictors for wound healing were improved pedal runoff score (<7), absence of infection, direct angiosome revascularization, and absence of end-stage renal disease. Inframalleolar intervention can be successfully performed in high-risk limbs with high expectation of good short-term results. Long-term patient-centered results remain poor because of the underlying disease process.

Outcomes of Isolated Tibial Endovascular Intervention for Rest Pain in Patients on Dialysis

Tibial interventions for critical limb ischemia are frequent in patients with end-stage renal disease (ESRD) presenting with critical ischemia. The aim of this study was to examine impact of ESRD on the patient-centered outcomes following tibial endovascular Intervention for rest pain. A database of patients undergoing lower extremity endovascular interventions between 2006 and 2016 was retrospectively queried. Patients with rest pain (Rutherford 4) were identified. Patients with claudication (Rutherford 1 to 3) and tissue loss (Rutherford 5 and 6) were excluded. Patients were categorized by the presence or absence of ESRD. Patient-orientated outcomes of clinical efficacy (CE; absence of recurrent symptoms, maintenance of ambulation, and absence of major amputation), amputation-free survival (AFS; survival without major amputation), and freedom from major adverse limb events (MALEs; above ankle amputation of the index limb or major reintervention new bypass graft, jump/interposition graft revision) were evaluated. A total of 829 patients (56% male, average age 59years; 658 nonhemodialysis [non-HD] and 171 HD) underwent isolated tibial intervention in one leg for rest pain. Technical success was 99% with a median of 2 vessels treated per patient. There was no difference in the distribution of Trans-Atlantic Inter-Society Consensus I lesions, but both the modified Society for Vascular Surgery (SVS) runoff score and the pedal runoff score were worse in the HD group. The 30-day major adverse cardiac events and 30-day MALEs were equivalent in both groups. CE was 38±9% and 19±8% at 5years for the non-HD and HD groups, respectively (P<0.01). Overall, AFS was 45±8% and 18±9% at 5years for the non-HD and HD groups, respectively (P<0.01). Freedom from MALE was 41±9% and 21±8% at 5years for the non-HD and HD groups, respectively (P<0.01). Patients with ESRD who present with rest pain have equivalent short-term outcomes to those not on dialysis but do not achieve long-term satisfactory CE and AFS after isolated tibial intervention for rest pain.

Calcific uraemic arteriolopathy (calciphylaxis) in patients on renal replacement therapy.

Calcific uraemic arteriolopathy (calciphylaxis) is an unusual and potentially fatal condition characterised by small-vessel calcification and ischaemic skin necrosis. It mainly affects patients with end-stage renal disease (ESRD) on haemodialysis, but may rarely occur in the absence of ESRD in conditions such as primary hyperparathyroidism, malignancy, alcoholic liver disease and connective tissue disease. We reviewed the records of all patients diagnosed with calciphylaxis while on renal replacement therapy at Tygerberg Hospital, Cape Town, South Africa, between 1990 and 2014, to describe its presentation, course and final outcome. Nineteen patients developed calciphylaxis over this period. Their median age was 34 years and 13 (68.4%) were female. Fifteen (78.9%) had received a kidney transplant. All patients had painful skin lesions that rapidly progressed to infarction. Small-vessel calcification was seen on skin biopsy in 13 patients. Twelve patients had hyperparathyroidism. Several of the transplanted patients had been treated for graft rejection in the year preceding the diagnosis. Treatment consisted of good wound care and efforts to normalise serum calcium and phosphate levels. Five patients received an urgent parathyroidectomy. The outcome was fatal in 17 patients, with sepsis being the main cause of death. In our patients, calciphylaxis carried a worse prognosis than previously reported internationally. It should always be considered in the differential diagnosis of painful skin lesions in the dialysis or transplant patient.

Hepatic and pancreatic resection in patients with end-stage renal disease: a propensity analysis

BackgroundHepatic and pancreatic surgery is rarely performed in patients with end-stage renal disease (ESRD). The present authors used a national clinical database to characterize outcomes and perioperative risk in ESRD patients who require hepatic or pancreatic resection. MethodsThe 2005–2011 National Surgical Quality Improvement Program database was queried for all patients undergoing hepatic or pancreatic resection. Patients were classified by the presence or absence of ESRD. The independent effects of ESRD on outcomes were assessed after propensity score adjustment and multivariable logistic regression. ResultsOf the 27 376 patients submitted to hepatic or pancreatic procedures identified in the database, 101 patients were found to have preoperative ESRD. Patients with ESRD experienced perioperative mortality at a rate similar to that in those without ESRD (5.0% versus 2.3%; P = 0.08). After risk adjustment, the presence of ESRD was associated with three-fold higher odds of postoperative sepsis (adjusted odds ratio: 2.98, P = 0.014), but no significant differences in mortality or major complication rates. ConclusionsHepatic and pancreatic resections can be performed safely in selected patients with ESRD. These patients may have an increased risk for the development of postoperative sepsis. Further study is needed to characterize modifiable risk factors that impact outcomes in patients with ESRD who require hepatic or pancreatic resection.

Coronary artery calcification is associated with insulin resistance index in patients with type 1 diabetes

The objective of the present study was to evaluate the risk factors associated with the presence of coronary artery calcification (CAC) in patients with type 1 diabetes (T1D). A cross-sectional study was conducted on 100 consecutive T1D patients without coronary artery disease, with at least 5 years of diabetes and absence of end-stage renal disease. Mean age was 38 ± 10 years and 57% were males. CAC score was measured by multidetector computed tomography (Siemens Sensation 64 Cardiac). The insulin resistance index was measured using the estimated glucose disposal rate (eGDR). The eGDR was lower among CAC-positive patients than among CAC-negative patients, suggesting an increased insulin resistance. In a logistic regression model adjusted for age (at 10-year intervals), eGDR, diabetic nephropathy and gender, CAC was associated with age [OR = 2.73 (95%CI = 1.53-4.86), P = 0.001] and with eGDR [OR = 0.08 (95%CI = 0.02-0.21), P = 0.004]. In T1D subjects, insulin resistance is one of the most important risk factors for subclinical atherosclerosis.

Atypical Calciphylaxis in a Patient Receiving Warfarin Then Resolving With Cessation of Warfarin and Application of Hyperbaric Oxygen Therapy

Calciphylaxis is a rare, usually fatal vasculopathic disorder characterized by cutaneous ischemia and necrosis due to calcification of arterioles. Although calciphylaxis is most frequently associated with end-stage renal disease (ESRD) and secondary hyperparathyroidism, it has been reported infrequently among patients on warfarin. No standard treatment has been established for atypical calciphylaxis; however, a potentially beneficial treatment is hyperbaric oxygen therapy (HBOT). A high degree of clinical suspicion, early diagnosis, and understanding the pathophysiology of this disease promotes the optimal management of this extremely morbid and often fatal condition. We present a 63-year-old Polynesian woman with biopsy-proven calciphylaxis in the absence of ESRD or elevated serum calcium levels while taking warfarin. Therapeutic dose enoxaparin was substituted for warfarin and she received 40 sessions of HBOT during which lower extremity ulcers resolved. Warfarin has been implicated when calciphylaxis presents in an atypical fashion. No guidelines exist for treatment of atypical calciphylaxis in the setting of concomitant warfarin therapy. Up to 80% of calciphylaxis patients die within 1 year of diagnosis. Our patient was changed to low-molecular-weight heparin and received HBOT. We present what we believe is the first case of atypical calciphylaxis thought to be attributable to warfarin treated with a therapeutic substitution of anticoagulant and HBOT leading to resolution of cutaneous lesions.

Intestinal Necrosis due to Sodium Polystyrene Sulfonate (Kayexalate) in Sorbitol

Sodium polystyrene sulfonate (SPS, Kayexalate) has been implicated in the development of intestinal necrosis. Sorbitol, added as a cathartic agent, may be primarily responsible. Previous studies have documented bowel necrosis primarily in postoperative, dialysis, and transplant patients. We sought to identify additional clinical characteristics among patients with probable SPS-induced intestinal necrosis. Rhode Island Hospital surgical pathology records were reviewed to identify all gastrointestinal specimens reported as containing SPS crystals from December 1998 to June 2007. Patient demographics, medical comorbidities, and hospital courses of histologically verified cases of intestinal necrosis were extracted from the medical records. Twenty-nine patients with reports of SPS crystals were identified. Nine cases were excluded as incidental findings with normal mucosa. Nine patients were excluded as their symptoms began before SPS administration or because an alternate etiology for bowel ischemia was identified. Eleven patients had confirmed intestinal necrosis and a temporal relationship with SPS administration suggestive of SPS-induced necrosis. Only 2 patients were postoperative, and only 4 had end-stage renal disease (ESRD). All patients had documented hyperkalemia, received oral SPS, and developed symptoms of intestinal injury between 3 hours and 11 days after SPS administration. Four patients died. Intestinal ischemia is a recognized risk of SPS in sorbitol. Our series highlights that patients may be susceptible even in the absence of ESRD, surgical intervention, or significant comorbidity.

Patients with end-stage renal disease and acute myocardial infarction have poor short-term outcomes despite modern cardiac intensive care

Cardiovascular disease has a tremendous impact on patients with end-stage renal disease (ESRD). We sought to describe the outcomes of patients with ESRD and acute myocardial infarction (AMI). We analyzed consecutive AMI patients admitted to a single coronary care unit, according to the presence or absence of ESRD. Multivariate modeling was used to determine the independent relationship between ESRD and in-hospital mortality. Data were available for 2025 AMI patients. ESRD was present in 82 patients (4%). Coronary angiography and revascularization were used aggressively in patients with ESRD, although less than among patients without ESRD (78 vs. 91%, P<0.001; and 60 vs. 79%, P<0.001, respectively). Length of stay was longer among patients with ESRD (9.5 vs. 5.0 days, P<0.001). In-hospital death occurred in 23.2% of the patients with ESRD compared with 9.0% of patients without ESRD (P<0.001). On multivariate analysis, the presence of ESRD was independently associated with the risk of in-hospital death (odds ratio 2.6, 95% confidence intervals, 1.3-4.9, P=0.005), even after controlling for the use of revascularization. Patients with ESRD and AMI are at tremendous risk for short-term mortality despite the aggressive use of coronary revascularization and modern coronary intensive care.

Obesity Is Associated With Family History of ESRD in Incident Dialysis Patients

Obesity is an established risk factor for chronic kidney disease and aggregates in families. The objective of this study is to examine the relationship between obesity and family history of end-stage renal disease (ESRD). Data were collected from 25,883 incident patients with ESRD in US ESRD Network 6 (Georgia, North Carolina, and South Carolina) dialysis clinics between 1995 and 2003. Family history is defined as a first- or second-degree relative with ESRD. Body mass index (BMI) at dialysis therapy initiation was classified as underweight (BMI < 18.5 kg/m2), normal (BMI, 18.5 to <25 kg/m2), overweight (BMI, 25 to < 30 kg/m2), obese (BMI, 30 to <35 kg/m2), or morbidly obese (BMI > or = 35 kg/m2). Twenty-three percent of patients reported a family history of ESRD. Of patients reporting a family history of ESRD, 5.5% were underweight, 32.5% had normal BMI, 28.0% were overweight, 17.3% were obese, and 16.7% were morbidly obese. After controlling for age, race, sex, primary cause of ESRD, history of diabetes, history of hypertension, and estimated glomerular filtration rate at dialysis therapy initiation, reported family history of ESRD was associated with being overweight (odds ratio [OR], 1.17; 95% confidence interval [CI], 1.08 to 1.26), obese (OR, 1.25; 95% CI, 1.14 to 1.37), and morbidly obese (OR, 1.40; 95% CI, 1.27 to 1.55). Obesity at dialysis therapy initiation was associated independently with reported family history of ESRD. This finding suggests that behavioral factors, adiposity-related genes, and gene-by-BMI interaction may contribute to familial risk for ESRD. This finding also suggests that management of obesity may be even more important for patients with a family history of ESRD than for the general population.

Cardiac Troponins and Renal Failure

In this issue of Circulation , Khan and colleagues present the results of an elegantly performed meta-analysis of the prognostic implications of elevated levels of troponin T and I among asymptomatic patients with end-stage renal disease.1 After systematically collating the results of 28 cohort studies involving 3931 patients, they noted that an elevated troponin T level identified a group of end-stage renal disease patients with high mortality risk. Although elevated troponin I was also associated with increased risk, the exact effect size was difficult to assess because of a lack of standardization of current assays. The authors noted that despite the consistent association between elevated troponin T and mortality, the effect might be overestimated because of publication bias and study heterogeneity. Nonetheless, this meta-analysis, along with the specific reports of the largest published cohort studies,2,3 provides robust evidence that troponin T elevation in the setting of end-stage renal disease is ominous. Article p 3088 The development of troponin measurement in the absence of end-stage renal disease is an excellent paradigm for the modern evolution of clinical tests. Troponin T and troponin I are both integral parts of the cardiac muscle infrastructure and play critical roles in excitation-contraction coupling.4 The diagnostic importance of troponin elevation stems from its release into the bloodstream when there is some type of damage to cardiac myocyte cell-wall integrity.5 The high sensitivity of troponin for detecting even small myocardial infarctions has led to widespread recommendations for its routine measurement for the diagnosis of myocardial infarction.6 It could be argued, though, that were elevated troponin to diagnose myocardial infarctions that were of little prognostic …

Management of methylmalonic acidaemia by combined liver–kidney transplantation

Methylmalonic acidaemia (MMA) is a rare autosomal recessive inborn error of metabolism that typically presents in infancy with recurrent episodes of metabolic acidosis, developmental delay and failure to thrive. The disease course is complicated by the development of chronic tubulointerstitial nephritis progressing to end-stage renal disease in adolescence. We describe two adolescents with cobalamin-nonresponsive MMA (mut0) who developed polyuria, chronic tubulointerstitial nephritis, dystonia but normal synthetic liver function. Both patients received combined liver-kidney transplantation (CLKT), preceded by a single pretransplant haemodialysis for clearance of methylmalonic acid. Post CLKT there was 95-97% reduction in serum and urine methylmalonic acid, leading to significant liberalization of dietary protein intake and a consequent increase in body mass index, muscle strength and energy. In addition, renal function normalized and clinical neurological status stabilized. We propose that CLKT be considered as a therapeutic option early in the course of cobalamin-nonresponsive MMA. Progressive tubulointerstitial nephritis with disabling polyuria is a confounder in patient management even in the absence of end-stage renal disease. Successful CLKT restores methylmalonyl-CoA mutase enzyme levels in the liver and kidney, improves clearance of methylmalonic acid with resultant dietary protein liberalization, and offers excellent graft and patient outcomes with improvement in quality of life.

Infrainguinal Bypass in Patients with End-stage Renal Disease: Survival and Ambulation

The purpose of this study was to evaluate the outcomes of infrainguinal bypass surgery and ambulatory status in patients with end-stage renal disease (ESRD). Vascular registry data and the medical records of patients requiring infrainguinal bypass surgery from 1985 through 1995 were reviewed retrospectively. Patients with chronic limb-threatening ischemia requiring maintenance hemodialysis or peritoneal dialysis for ≥ 6 months were compared to a randomly selected group of patients under going foot salvage infrainguinal bypass in the absence of ESRD. Primary outcomes were mortality, amputation, and ambulatory status at 30 days and 1 year. Data were analyzed using Pearson's chi-square methods, Fisher's Exact test, Mann-Whitney U, life table analyses, and Quality of Life-Class (QL-Class) ranking. There were 57 patients with ESRD who underwent 66 infrainguinal bypass proce dures. Mean age was 65.8 ±9.8 years (41-85 years). The 30-day operative mortality rate was 12.3% (7 patients). The cumulative survival at 1 year was 51.8% ±0.9%, and at 2 years it was 32.8% ± 1.3%. The cumulative limb loss was 29.7% ± 1.1% at 1 year and 36.7% ±2.6% at 2 years. In the comparison group, 46 patients without ESRD underwent 50 infrainguinal bypass procedures. The mean age of these patients was 72.3 ±9.1 years (36-90 years). The cumulative survival for the patients without ESRD was significantly higher (p &lt; 0.001) both at 1 year (91.1% ±0.6%) and 2 years (88.8% ±0.8%). The cumulative limb loss for the comparison group was significantly lower (p &lt; 0.001) at 1 year (4.1% ±0.4%) and at 2 years (6.3% ±0.5%). At both 30 days and 1 year, the QL- Class walking status rating was lower for the group with ESRD compared to patients without ESRD (p &lt; 0.001). Patients with ESRD have a high mortality rate in the first 24 months after infrain guinal bypass grafting for foot salvage surgery compared with a similar group of patients without ESRD. Although foot salvage can be achieved in some survivors with ESRD, the ambulatory rate is low. These results support a very conservative approach when recom mending infrainguinal bypass grafting for foot salvage surgery for patients with ESRD.

Calciphylaxis in a patient with Crohn's disease in the absence of end-stage renal disease

Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification previously described in patients with end-stage renal disease and hyperparathyroidism. Early diagnosis may be important in improving the poor outcome in these patients since early intervention may forestall the development of life-threatening complications. We describe a patient with Crohn's disease complicated by short-bowel syndrome and modest renal insufficiency (not requiring renal replacement therapy) who developed calciphylaxis. It appears that longstanding Crohn's disease and the short-bowel syndrome accelerated the development of calciphylaxis as the chronic renal disease was not end stage. Considering the possibility of calciphylaxis in this setting may avoid delaying the diagnosis and its consequences.

Pancreas-transplant outcome in relation to presence or absence of end-stage renal disease, timing of transplant, surgical technique, and donor source.

The differences in pancreas-transplant outcome according to recipient status, surgical approach, and donor source are illustrated by an analysis of results at one institution with experience in several categories. From July 1978 to January 1988, 210 pancreas transplants were performed, and 67 grafts are still functioning, the longest for 9.7 yr. Since October 1984, a uniform immunosuppressive protocol has been used, antilymphocyte globulin, cyclosporin, azathioprine, and prednisone for induction and the last three drugs for maintaining antirejection therapy. During this period, 110 pancreas transplants were performed, 62 in nonuremic non-kidney transplants, 28 in recipients of a previous kidney, and 20 simultaneous with a kidney; 64 with bladder and 43 with enteric drainage; and 25 from related and 85 from cadaver donors. The overall patient survival rate at 1 yr was 91%, and there were no significant differences between the various categories. Graft survival rates, however, differed between the various categories created by combinations of the above variables. With bladder drainage, 1-yr function rates were 58% (n = 30), 47% (n = 15), and 77% (n = 19) in recipients of a pancreas transplant alone, a pancreas after a kidney, or a simultaneous pancreas-kidney transplant; with enteric drainage, 1-yr function rates were 33% (n = 32) and 36% (n = 11) in the pancreas transplant alone and pancreas after kidney categories (enteric drainage was not done in double-transplant patients).(ABSTRACT TRUNCATED AT 250 WORDS)