Zosteriform pemphigoid after zoster: Wolf's isotopic response

The Wolf isotopic response describes the occurrence of a new, unrelated disease that appears at the same location as a previously healed disease. The most common primary skin disorder of this phenomenon is herpes zoster and less frequently, herpes simplex. We report a case of 79‐year‐old woman who have bullous pemphigoid (BP) with dermatomal distribution that developed at the site of previously healed herpes zoster. Based on clinical, histological and immunofluorescence findings, the patient was diagnosed with localized BP in a site of prior herpes zoster. BP developing at the site of healed herpes zoster is the first reported case. Recognition of this phenomenon is important for correct clinicopathologic diagnosis and may improve our understanding of the underlying pathophysiologic processes.     

Rhabdomyolysis,Acute Renal Failure,and Multiple Focal Neuropathies After Drinking Alcohol Soaked with Centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49‐year‐old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

Une présentation rare du syndrome d’intolérance au greffon renal : la pemphigoïde bulleuse

Bullous pemphigoid is an autoimmune bullous cutaneous disease. We report the case of a 60 year-old male patient whose kidney allograft failed and was on hemodialysis for the previous 16 months. After tapering immunosuppressive medication, he presented simultaneous bullous eruption and kidney allograft intolerance syndrome. Investigation showed a positive BP180 anti-basement membrane zone antibody and skin biopsy was consistent with bullous pemphigoid. The patient was treated with corticotherapy and bullous pemphigoid resolved. The development of new onset diabetes and concerns over long term immunosuppression, halted the decision to continue corticotherapy and the patient underwent graft nephrectomy, with resolution of the kidney allograft intolerance syndrome without recurrence of the bullous disease. The occurrence of bullous pemphigoid in patients with failed renal allograft is rare, with only eleven cases reported in literature. This case illustrates how graft nephrectomy can provide a definitive cure to bullous pemphigoid in this setting.     

Calciphylaxis in end‐stage liver and renal disease patients before and after transplant

Calciphylaxis is a rare vascular disorder characterized by calcification of arterioles which causes tissue inflammation and necrosis. It is associated with the metabolic disturbances seen in end‐stage renal disease (ESRD) and has also been described in patients with cirrhosis with preserved kidney function. Characteristic calciphylaxis lesions are black eschars surrounded by retiform purpura, and the gold standard for diagnosis is skin biopsy. Reported 1‐year mortality rates range between 45% and 80%. No treatment modality has been evaluated in a prospective randomized trial, and reports of treatment efficacy vary. Kidney transplant has been reported as a successful therapy for calciphylaxis; however, cases exist of the initial onset of calciphylaxis following kidney transplant as well as simultaneous liver‐kidney (SLK) transplant. The decision to maintain a patient with end‐stage renal and liver disease on the waiting list for SLK transplant following the onset of calciphylaxis must consider the high 1‐year mortality associated with this condition. More research is necessary to understand how to allocate donor allografts to manage patients with calciphylaxis and ESRD and/or cirrhosis effectively.     

Utility of perioperative skin perfusion pressure measurement for treatment of ulcers caused by arteriovenous access ischaemic steal

Arteriovenous access ischaemic steal is a serious complication following arteriovenous fistula (AVF) construction. The aim of treatment is to improve distal circulation without impairing the function of the fistula. Therefore, any repair should be performed with intraoperative monitoring. We report 2 cases of this disorder treated using perioperative measurement of skin perfusion pressure (SPP) for preoperative surgical planning and intraoperative guidance. A 73‐year‐old woman with a left cubital AVF developed gangrene of the tip of the left little finger. Arteriovenous access ischaemic steal was suspected. The SPP of the little finger was 18 mm Hg, which increased to 65 mm Hg after manual occlusion of the fistula. A 58‐year‐old woman with a left antebrachial AVF had gangrene of the tip of the left middle finger. The SPP was 19 mm Hg, and steal syndrome was suspected based on angiography and the SPP with manual occlusion of the fistula. In both cases, serial plication of the fistula was performed based on intraoperative perfusion pressure monitoring, leading to the successful resolution of the ischaemic steal syndrome. In both cases, haemodialysis could be continued through the repaired fistula.     

The epidemiology of end-stage renal disease in Iran in an international perspective.

INTRODUCTION: The epidemiology of end-stage renal disease (ESRD) and renal replacement therapy (RRT) is under continuous evolution all over the world. We report here the epidemiological analysis of ESRD and RRT in Iran and discuss it against the background of the international situation. METHODS: This epidemiological report is based on data from centre questionnaires which were collected in Iran from 1997 onwards, with a response rate of 100%. RESULTS: The prevalence/incidence of RRT patients were 238/49.9 p.m.p. in the year 2000. Haemodialysis and kidney transplantation were the most common RRT modalities, accounting for 53.7% and 45.5% of prevalent RRT patients, respectively. The proportion treated by peritoneal dialysis was very low (<1%). Home haemodialysis was not performed. The majority of haemodialysis centres used synthetic membranes (70%) and 100% of the sessions were performed using acetate as a buffer; 42.5% of haemodialysis patients were treated with a twice-weekly regimen, whilst 49.6% were on the standard thrice-weekly regimen. The majority of RRT patients in Iran were young to middle aged. The great majority of renal allografts came from living donors (mainly unrelated to recipients). The main renal diseases leading to ESRD were diabetes and hypertension. The third most common category was "cause unknown". CONCLUSION: The epidemiology of RRT in Iran is characterized by: (i) young patient age (younger than the international average); (ii) high proportion of patients receiving renal allograft; (iii) use of living-unrelated donors as the major source of renal allografts.     

Diabetic end-stage renal disease in the indigenous population of the Commonwealth of the Northern Mariana Islands

The number of cases of treated end-stage renal disease (ESRD) attributable to type 2 diabetes and survival after the onset of renal replacement therapy was examined in the Commonwealth of the Northern Mariana Islands (CNMI). All Chamorros and Carolinians to receive renal replacement therapy for ESRD between January 1982 and December 2002 were identified. Changes in survival over time were examined by dividing the study into three equal periods. Of 180 new cases of ESRD, 137 (76%; 101 Chamorros, 36 Carolinians) were attributed to diabetes. Ninety-nine subjects, 80% of whom had diabetic ESRD, began renal replacement therapy in the last 7 years of the study compared with 81 (72% with diabetic ESRD) in the previous 14 years. All 137 of the diabetic subjects received haemodialysis. During the 21-year study period, 79 of the diabetic subjects receiving dialysis died. The median survival after the onset of haemodialysis was 37 months in the first time period (1982-1988), 47 months in the second period (1989-1995) and 67 months in the third period (1996-2002). The death rate in the first period was 4.3 times (95% CI, 2.1-8.9) as high and the second period was 2.9 times (95% CI, 1.5-5.8) as high as the most recent period, after adjustment for age, sex and ethnicity in a proportional-hazards analysis. The number of diabetic patients in CNMI who are receiving renal replacement therapy is rising rapidly. Considerable improvement in survival after the onset of haemodialysis has occurred over the past 21 years.     

Acute pancreatitis during haemodialysis.

Sir, Acute pancreatitis tends to develop more frequently in patientswith end-stage renal disease (ESRD) than in the general population,even in those managed by haemodialysis [1]. A case of acutepancreatitis in a patient on haemodialysis is presented hereinto remind the nephrology     

Immunofluorescence of Autoimmune Bullous Diseases

Autoimmmune bullous diseases of skin and mucosa are uncommon, disabling, and potentially lethal diseases. For a quick and reliable diagnosis immunofluorescence is essential. This article describes two variants of immunofluorescence. The direct method uses a skin or mucosal biopsy of the patient to detect in vivo bound antibodies. Indirect immunofluorescence uses patient’s serum and a substrate to visualize circulating autoantibodies. These two methods supplemented with advanced techniques allow reliable classification of autoimmune bullous diseases; not only the main entities pemphigus and pemphigoid, but also subclasses within these groups. This is important because prognosis and therapy vary among different variants of autoimmune bullous diseases.     

Left ventricular geometry and cardiovascular mortality based on haemodialysis patient autopsy analyses

Aim: In end‐stage renal disease (ESRD) patients, left ventricular hypertrophy (LVH) is common and a risk for cardiovascular events. LVH is geometrically classified into two major groups, concentric and eccentric, and accumulating evidence suggests eccentric LVH has a more negative effect than concentric LVH on ESRD outcome. However, there have been very few studies on the cardiac findings from ESRD patient autopsy in which the relationship between LVH geometry and mortality was analyzed. Methods: An observational study was performed with the autopsy findings in 30 haemodialysis patient cases between 2001 and 2006 at Mitsui Memorial Hospital, Tokyo. Between those who died of a cardiovascular cause and those who died of non‐cardiovascular causes, we compared the heart/bodyweight ratio, left ventricular dilatation, and the extent of fibrosis of the left ventricle. Results: Heart/bodyweight ratio was significantly higher (P < 0.0001) in the cardiovascular mortality group (n = 11, 11.7 ± 2.5 g/kg) compared to the non‐cardiac cause of death group (n = 19, 8.05 ± 0.7 g/kg). The dilatation of the left ventricle was significantly more frequent in the cardiovascular than the non‐cardiac cause of death group (P = 0.016). Additionally, the fibrotic area of left ventricular cross‐section was larger in the cardiovascular (1.63 ± 1.6%) than the non‐cardiac group (0.83 ± 1.7%, P = 0.04). Conclusion: This autopsy study indicates that eccentric LVH in haemodialysis patients is closely associated with cardiovascular mortality. LVH geometry, as well as LVH severity, is worthy of consideration as a clinical predictor for cardiovascular mortality.     

The spectrum of renal involvement in epidermolysis bullosa dystrophica hereditaria: report of two cases

Epidermolysis bullosa dystrophica Hallopeau-Siemens (EBDH) is one of the most severe inherited epidermolyses, a group of mechanobullous dermatological disorders. We observed two patients presenting with a severely multilating type of EBDH who developed biopsy-proven renal disease, which substantially altered the evolution and pathogenesis of their disease. In a boy, chronic postinfectious glomerulonephritis developed, most probably due to recurring superinfections of bullous skin lesions. He also experienced acute oliguric renal failure due to severe diarrhea during exacerbation of EBDH. A female patient developed a nephrotic syndrome due to secondary amyloidosis. Hypoalbuminemia caused further fluid losses through bullous skin lesions, aggravating intravascular hypovolemia and leading to rapid renal failure secondary to bilateral renal vein thrombosis. The study shows that, although rare, renal complications may alter the natural course of EBDH.     

Rhabdomyolysis, acute renal failure, and multiple focal neuropathies after drinking alcohol soaked with centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49-year-old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

Kidney diseases in dystrophic epidermolysis bullosa: case report

Dystrophic epidermolysis bullosa is a genetic skin disease of which the recessive subtype also named Hallopeau-Siemens is the most severe. It is due to lack of expression of type VII collagen which is essential for dermal anchoring. Severe obstructive uropathies of the urethral and bladder area may occur during the first years of life, in relation to local bullous activity. As in acquired bullous diseases, glomerular complications may occur in the dystrophic form during the second and third decade. They consist mostly in AA amylosis or mesangial immunoglobuline A glomerulonephritis, in relation to chronic inflammation and repeated cutaneous infections. End stage renal failure occurs in most cases. Only haemodialysis was used as suppletive treatment in four patients for a short period.     

A population-based, prospective study of blood pressure and risk for end-stage renal disease in China

The association between BP and risk for ESRD has not been well characterized in Asian populations. This study examined the relationship between level of BP and incidence of ESRD in a prospective cohort study of 158,365 Chinese men and women who were 40 yr and older. Measurement of BP and covariables were made in 1991 following a standard protocol. Follow-up evaluations were conducted in 1999 to 2000 and included interviewing participants or proxies and obtaining medical records and death certificates for ESRD cases. During 1,236,422 person-years of follow-up, 380 participants initiated renal replacement therapy or died from renal failure (30.7 cases per 100,000 person-years). Compared with those with normal BP, the multivariate adjusted hazard ratios (95% confidence interval) of all-cause ESRD for prehypertension and stage 1 and stage 2 hypertension were 1.30 (0.98 to 1.74), 1.47 (1.06 to 2.06), and 2.60 (1.89 to 3.57), respectively (P < 0.001 for trend). The corresponding hazard ratios (95% confidence interval) of glomerulonephritis-related ESRD were 1.32 (0.82 to 2.11), 1.48 (0.83 to 2.61), and 3.40 (2.02 to 5.74), respectively (P <0.001 for trend). Systolic BP was a stronger predictor of ESRD than diastolic BP or pulse pressure. This study provides novel data on the incidence of ESRD and on the association between BP and glomerulonephritis-related ESRD from a nationally representative sample of adults in China. These results document the importance of high BP as a modifiable risk factor for ESRD in China. Strategies to prevent ESRD should incorporate the prevention, treatment, and control of BP.     

Two Patients With History of STEC‐HUS,Posttransplant Recurrence and Complement Gene Mutations

Hemolytic uremic syndrome (HUS) is a disease of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. About 90% of cases are secondary to infections by Escherichia coli strains producing Shiga‐like toxins (STEC‐HUS), while 10% are associated with mutations in genes encoding proteins of complement system (aHUS). We describe two patients with a clinical history of STEC‐HUS, who developed end‐stage renal disease (ESRD) soon after disease onset. They received a kidney transplant but lost the graft for HUS recurrence, a complication more commonly observed in aHUS. Before planning a second renal transplantation, the two patients underwent genetic screening for aHUS‐associated mutations that revealed the presence of a heterozygous CFI mutation in patient #1 and a heterozygous MCP mutation in patient #2, and also in her mother who donated the kidney. This finding argues that the two cases originally diagnosed as STEC‐HUS had indeed aHUS triggered by STEC infection on a genetic background of impaired complement regulation. Complement gene sequencing should be performed before kidney transplantation in patients who developed ESRD following STEC‐HUS since they may be undiagnosed cases of aHUS, at risk of posttransplant recurrence. Furthermore, genetic analysis of donors is mandatory before living‐related transplantation to exclude carriers of HUS‐predisposing mutations.     

Increasing AV fistulas: revisiting a time-tested solution

Despite having the lowest complication rate of all hemodialysis accesses, the prevalence of autologous arteriovenous (AV) fistulas has declined to 28% in the United States. The reasons for this decline include high early AV fistula failure rates, long maturation times, the frequent need for emergent dialysis, unavailable or poor pre-ESRD programs and planning, patient resistance to the realities of impending ESRD, and financial disincentives to AV fistula placement. Despite these barriers, programs throughout the country have demonstrated the ability to increase AV fistula prevalence to more than 50%. The strategies employed have included increased reliance on upper arm brachiocephalic and transposed basilic vein fistulas, the use of preoperative imaging to identify the best sites for fistula creation, and aggressive attempts at salvage of nonmaturing fistulas. Other groups have systematically and successfully replaced failed grafts with upper arm brachiocephalic or bracheobasilic fistulas. These experiences clearly show that exceeding the National Kidney Foundation Dialysis Outcomes Quality Initiative (NKF-DOQI) goal of more than 50% fistula placement is achievable in the United States. Declining numbers of AV fistulas are the result of a combination of factors, including changes in our patient population and learned practice patterns coupled with a failure of our delivery system to provide education, timely referral, and incentives for fistula placement. Increasing AV fistula prevalence in the United States is achievable and will improve patient outcomes and decrease the costs of ESRD.     

Anaesthesia for renal transplantation in sickle cell disease

Sickle cell disease (SCD) is associated with many pathological and functional abnormalities affecting all organ systems. Renal manifestations of SCD may result in end-stage renal disease (ESRD), which can be treated by chronic haemodialysis or renal transplantation. Renal transplantation was successfully performed in a 25-yr-old male with sickle cell beta-thalassaemia and nephrotic syndrome. We present a case report of this patient, a discussion of the renal complications associated with SCD and the perioperative management of a patient with SCD undergoing renal transplantation.     

Antihypertensive Medications in End‐Stage Renal Disease

Hypertension is almost universal in end‐stage renal disease (ESRD) and contributes to the substantial cardiovascular (CV) morbidity and mortality observed in these patients. The management of blood pressure (BP) in ESRD is complicated by a number of factors, including missed dialysis treatments, intradialytic changes in BP, medication removal with dialysis, and poor correlation of BPs obtained in the dialysis unit with those at home and with CV outcomes. Control of extracellular volume with ultrafiltration and dietary sodium restriction represents the principal strategy to manage hypertension in ESRD, and antihypertensive medications are subsequently added if this strategy is inadequate. While reduction in BP with medication improves CV outcomes, few head‐to‐head clinical trials have been performed to firmly establish the superiority of one antihypertensive medication class over another. Therefore, individualization of therapy is necessary, and patient comorbidities must be considered. Angiotensin‐converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), and beta‐blockers are reasonable first‐line agents for most patients. ACE inhibitors and ARBs exert cardioprotective effects that are independent of BP reduction. Medications that are removed with dialysis may be preferred in patients who are prone to develop intradialytic hypotension. Intradialytic hypertension can be managed with challenging the patient's dry weight and using nondialyzable medications. Within a class of antihypertensive medications, there may be large variability in drug removal with dialysis, which must be considered upon medication selection. Studies demonstrate that even thrice‐weekly dosing of medication after dialysis has robust BP‐lowering effects, which may be a useful regimen in nonadherent patients.     

Bullous pemphigoid-associated nephropathy: report of two cases and review of the literature

Bullous pemphigoid has previously been reported in association with a variety of renal lesions. Two additional cases are presented in this report in which the nephropathy preceded the onset of the skin disease: one case with membranous glomerulopathy and one case of renal allograft rejection with concurrent membranous pathology. Both patients had positive immunofluorescence of the skin, typical of bullous pemphigoid. Institution of systemic corticosteroid therapy resulted in a satisfactory clinical response and cessation of the blistering process. These cases and a review of the literature suggest that the occurrence of an immune process involving these two different basement membranes is not merely coincidental. Many cases have been described in which the severity of the skin lesions paralleled that of the renal disease. Although the possibility of multiple distinct autoimmune processes cannot be excluded, anti-basement-zone antibody interactions or allograft rejection-induced immune stimulation are possible unifying mechanisms for the simultaneous skin and renal involvement observed in these two cases.     

Preserving function and long-term patency of dialysis access

Of the 283, 932 patients with end stage renal disease (ESRD) receiving replacement therapy in the US in 1996, 62% were being treated with haemodialysis. Improved survival of haemodialysis patients coupled with the inability to provide enough renal transplants for the growing ESRD population has resulted in an increase in the average length of time patients spend on dialysis. Vascular accesses are, therefore, required to function for longer periods of time. Maintenance of a reliable access to the circulation has been described as the Achilles' heel of modern haemodialysis. Preserving access function and long-term patency are essential for efficient dialysis delivery.