The Matter of Kidney Biopsy in Monoclonal Gammopathy of Renal Significance: A Case Report of a New Pattern of Immunoglobulin-Storing Histiocytosis.

Monoclonal gammopathy of renal significance (MGRS) represents a group of disorders, characterized by paraproteinemia which causes renal damage. These disorders never meet the diagnostic criteria for multiple myeloma (MM) or lymphoproliferative disease. Crystal-storing histiocytosis is one of the rarest patterns of MGRS, characterized by an accumulation of light chains of crystals within histiocyte's cytoplasm, located in bone marrow or other extramedullary sites such as the kidney, cornea, or thyme. A very few cases have been described as immunoglobulin-storing histiocytosis (IgSH) without evidence of crystals. In the recent literature, only 3 cases of IgSH have been described so far, none renal. In all cases, these very peculiar histopathological patterns are associated with lymphoproliferative or plasma cellular disorders. Here, we report a very unusual IgSH pattern in a kidney biopsy, which led to prompt detection and early therapeutic intervention, in a patient with otherwise misdiagnosed MGRS.

Kidney rupture: an unusual and oligosymptomatic complication in a dialysis patient with acquired cystic disease.

Acquired cystic kidney disease (ACKD) is a complication of end-stage renal disease, the prevalence of which is related to dialysis duration; incidence of ACKD and associated conditions (neoplasia, hemorrhage) have decreased with improvements in renal transplantation and with the ageing of the dialysis population. This report regards spontaneous kidney rupture in a 57-year old patient, on home hemodialysis for 11 years, with ACKD for 5 years. At the end of a dialysis session, the patient reported sudden onset of colicky flank pain, followed by macrohematuria. Pain remitted with low doses of pain relievers, leaving dull flank discomfort. The patient self diagnosed a renal colic, and called the hospital two days later. At referral, two large hemorrhagic renal masses (7 and 2.8 cm) were found at ultrasound and CT scan. At surgery, kidney rupture was diagnosed. This case highlights the life threatening complications associated with ACKD, and underlines that massive renal hemorrhage may occur with relatively minor symptoms.

Ankylosing spondylitis: a difficult diagnosis in patients on long-term renal replacement therapy.

We report the case of a 48-year-old male, whose musculoskeletal manifestations, previously related to long-term renal replacement therapy (RRT), were diagnosed as ankylosing spondylitis when symptoms changed their pattern on daily hemodialysis (DHD). The patient started RRT in 1981; in 1985 he received a cadaver graft, which failed in 1987. Secondary hyperparathyroidism, amyloid geoids, bilateral carpal tunnel syndrome and high aluminium levels were present. Musculoskeletal pain, reported since 1986, involved feet, heels, hips, shoulders, hands, spine. Symptoms impairing daily life did not improve after parathyroidectomy. He developed chronic hypotension and recurrent atrial fibrillation. In 1994 and 1998, because of thoracic pain, coronarography was performed (normal on both occasions). In June 2000, DHD was started. Equivalent renal clearance increased from 9-12 to 15-17 mL/min. Well-being remarkably improved. In September 2000, musculoskeletal pain worsened and bilateral Achilles tendinitis occurred. The worsening of musculoskeletal symptoms despite the improvements in well-being and other dialysis related symptoms prompted a re-evaluation of the case. The diagnosis of ankylosing spondylitis was based on: history of plantar fasciitis, bilateral Achilles tendinitis, inflammatory spinal pain with limitation of lumbar spine mobility (positive Schober test), radiological evidence of grade 2 bilateral sacroiliitis, presence of HLA-B27. This diagnosis cast light on the episodes of chest pain, explained by enthesopathy at the costosternal and manubriosternal joints and atrial fibrillation, due to HLA-B27 associated impairment in heart conduction. This case exemplifies the difficulty of differential diagnosis of multisystem illness in patients with long RRT follow-up.

Why Our Patients Like Daily Hemodialysis.

The option of daily hemodialysis (HD) was discussed in November 1998 with a group of 35 HD patients on home or self-care/limited-care HD in a single, freestanding unit. After the meeting, 3 patients on home HD chose to switch to daily HD. The clinical success of the first patient and the immediate followers was one of the main reasons for further extension of this experience. At the time of this writing (February 2000), 10 patients were on a daily HD program (8 at home and 2 in a self-care/limited-care center) and one was in training for home daily HD. One further patient who tried 1 month of daily HD dropped out for logistic reasons. On daily HD, patients are dialyzed 2 - 3 hours/day, 6 days/ week, with blood flow of 270 - 300 mL/min, on bicarbonate dialysate with individually determined levels of Na and K. The schedule is flexible and a switch to 3 - 4 dialyses/week is occasionally allowed for working needs or for vacation. In addition to the well-known clinical advantages (better well-being, blood pressure control, nutrition, etc.), some patients preferred daily HD because of easier organization of daily activities, including work schedule. Patients initially feared frequent needle punctures and excessive burden on partners, but those concerns proved to be less a problem than anticipated. All current patients are willing to continue daily HD; only a nursing shortage limits further extension of the program in the self-care/limited-care center.

Lost to follow-up: the bottleneck of early referral to renal units?

BACKGROUND: Early referral is a major goal in chronic kidney diseases; however, loss to follow-up, potentially limiting its advantages, has never been studied. MATERIAL/METHODS: In order to assess the prevalence and causes of loss to follow-up, a telephone inquiry was performed in a renal outpatient unit, mainly dedicated to early referral of diabetic patients. Patients were considered to be in follow-up if there was at least one check-up in the period February 2001-February 2002, and lost to follow-up if the last check-up had occurred in the previous year. The reasons for loss to follow-up were related to typical clinical-biochemical parameters to define a "drop-out profile". RESULTS: 195 patients were on follow-up: median creatinine 1.4 mg/dL, age 64, 76.9% diabetics. 81 patients were lost to follow-up: creatinine 1.4 mg/dL, age 70, 73.8% diabetics. A telephone number was available in 87.6% of the cases; 25 were not found, 7 had died, 24 were non-compliant, 1 was bed-ridden, 12 had changed care unit, 2 had started dialysis. Renal care was shorter in those lost to follow-up; among the latter, serum creatinine and age were significantly lower in non-compliant patients. A logistic regression model confirmed the significance of lower serum creatinine at last check-up in non-compliant patients (p=0.018). CONCLUSIONS: Loss to follow-up is a problem in nephrology; lack of awareness probably causes the higher drop-out rate at lower creatinine levels. The initial period of care may be crucial for long-term compliance. Further studies are needed to tailor organizational and educational interventions.

Low-protein vegetarian diet with alpha-chetoanalogues prior to pre-emptive pancreas-kidney transplantation.

BACKGROUND: Pre-emptive pancreas-kidney transplantation is increasingly considered the best therapy for irreversible chronic kidney disease (CKD) in type 1 diabetics. However, the best approach in the wait for transplantation has not yet been defined. AIM: To evaluate our experience with a low-protein (0.6 g/kg/day) vegetarian diet supplemented with alpha-chetoanalogues in type 1 diabetic patients in the wait for pancreas-kidney transplantation. METHODS: Prospective study. Information on the progression of renal disease, compliance, metabolic control, reasons for choice and for drop-out were recorded prospectively; the data for the subset of patients who underwent the diet while awaiting a pancreas-kidney graft are analysed in this report. RESULTS: From November 1998 to April 2004, 9 type 1 diabetic patients, wait-listed or performing tests for wait-listing for pancreas-kidney transplantation, started the diet. All of them were followed by nephrologists and diabetologists, in the context of integrated care. There were 4 males and 5 females; median age 38 years (range 27.9-45.5); median diabetes duration 23.8 years (range 16.6-33.1), 8/9 with widespread organ damage; median creatinine at the start of the diet: 3.2 mg/dl (1.2-7.2); 4 patients followed the diet to transplantation, 2 are presently on the diet, 2 dropped out and started dialysis after a few months, 1 started dialysis (rescue treatment). The nutritional status remained stable, glycemia control improved in 4 patients in the short term and in 2 in the long term, no hyperkalemia, acidosis or other relevant side effect was recorded. Proteinuria decreased in 5 cases, in 3 from the nephrotic range. Albumin levels remained stable; the progression rate was a loss of 0.47 ml/min of creatinine clearance per month (ranging from an increase of 0.06 to a decrease of 2.4 ml/min) during the diet period (estimated by the Cockroft-Gault formula). CONCLUSIONS: Low-protein supplemented vegetarian diets may be a useful tool to slow CKD progression whilst awaiting pancreas-kidney transplantation.

Patients on renal replacement therapy for 20 or more years: a clinical profile.

BACKGROUND: Long-term survivors are living evidence of the goals and limits of renal replacement therapy (RRT). METHODS: A cross-sectional study was performed on all cases (188 patients) with RRT follow-up >/=20 years in Piemonte, northern Italy (4 350 000 inhabitants, 22 dialysis centres). Study included revision of clinical charts and assessment of functional (Karnofsky scale, Ks) and nutritional status (subjective global assessment, SGA). According to treatment history, patients were sorted into three groups: group 1, 56 patients always on dialysis; group 2, 40 patients on dialysis with previous graft; group 3, 92 grafted patients. RESULTS: Age differed between group 1 and groups 2 and 3 (59.5+/-11.5 vs 51.5+/-7.9 and 51.0+/-9.0 years; P=0.001). Prevalence of comorbidity was higher in groups 1 and 2 (94.6% and 95%) compared with group 3 (81.5%), reflecting selection during follow-up. Twenty-two cases (11.7%) had no comorbidity; these patients were younger (44.3+/-8.5 years) and 17 out of 22 had a functioning graft. The most common comorbidities were vasculopathy (73.4%), bone disease (72.9%) and cardiopathy (33.5%). Severe visual impairment was a common problem (18%), with a higher prevalence in patients with cardiovascular comorbidity (32%). Severe depression was found in 13.3% of cases. Despite comorbidity, functional scores (Ks) were good (higher in group 3 (88.1+/-15) than in groups 1 and 2 (67.9+/-21.9 and 75.5+/-18, respectively); P=0.000) and 64% of patients were well nourished. The combination of cardiovascular comorbidity, bone disease and visual impairment may reflect the premature ageing of RRT patients. CONCLUSION: Despite the high prevalence of comorbidity, long-term follow-up may promote good clinical conditions at least in some patients, highlighting the therapeutic potentials of dialysis in an era of reconsideration of open acceptance of RRT.

Daily Dialysis and Flexible Schedules: How to Assess Kt/V and EKRc ?

Despite the growing interest in daily hemodialysis (DHD), logistic and economic factors limit its dissemination. Not the least of these factors is the lack of uniform criteria for measuring efficiency. From November 1998 to November 2000, 19 patients were on DHD in our unit. The dialysis prescription was bicarbonate buffer; 6 sessions per week; 2 - 3 hours; blood flow 250 - 350 mL/min; individual K, HCO3 , and Na levels; membrane 1.6 - 2 m2 (polysulfone, polycarbonate). The prescription represented the minimum dialysis requirement; patients were free to add up to 30 minutes per session, further increase or any decreases needed confirmation by the caregivers. The aim of the study was to assess Kt/Vurea variability in this clinical setting, and to identify the minimum number of dialysis sessions required to obtain a reliable estimate of weekly Kt/Vurea [relative error (RE) < 10%]. We studied 169 dialysis sessions in 13 clinically stable patients on DHD for ≥ 3 months, with ≥ 3 Kt/Vurea measurements within 2 weeks (median: 10; range: 3 - 32 sessions), tested in the same laboratory. To assess variability, we employed the simplest formula (the Lowrie Kt/Vurea ), the widely used Daugirdas II formula, and the derived single-pool equivalent renal clearance (EKRc ), according to Casino. The variability of Kt/Vurea per session was high (Lowrie: RE = 2.5% - 22.1%; Daugirdas II and EKRc : RE = 3.6% - 24%). Averaging several dialysis sessions leads to a more reliable estimate of weekly efficiency (6 sessions: RE = 0; 3 sessions, Lowrie formula: Kt/Vurea RE = 1.1% - 9.7%; Daugirdas II and EKRc : RE = 1.6% - 10.6%). In patients with wide time variations, variability may be lower if weekly efficiency is determined on the basis of "average hourly Kt/Vurea ," which is calculated by dividing Kt/Vurea by the number of hours in the studied sessions, and then multiplying by the hours of dialysis performed in the whole week (Lowrie formula, Kt/Vurea : RE = 4.8% - 16.6% for 1 session, 2.1% - 7.3% for 3 sessions). Once again, the RE decreases sharply when data from 3 sessions are considered. Therefore, for flexible DHD, we suggest averaging the data from ≥ 3 sessions for weekly Kt/Vurea assessment.

Daily Dialysis: Toward a New Standard in Well-Being.

Daily hemodialysis (DHD) is a promising option; however, logistic obstacles and clinical perplexities limit its dissemination. Understanding the mechanisms of, and the time until, the onset of improved well-being may help to quantify clinical advantages and to define the minimum length of a "trial" of daily dialysis. By following 30 patients treated in 4 centers, this study aimed to determine how long a period of time is needed until a patient experiences subjective improvement. From November 1998 to November 2000, 30 patients tried at least 2 weeks of short daily dialysis in four Northern Italian centers of Piemonte and Valle d'Aosta. The DHD (2 - 3 hours; blood flow 270 - 350 mL/min; individual HCO3 , Na, K) was performed at home or in a center. Motivations to try DHD, fears and concerns regarding DHD, and changes in perceived well-being were assessed by semi-structured interview. The main clinical indications for a trial of DHD were poor tolerance of conventional treatment, cardiovascular disease, and hypertension or hypotension; only 6 patients had no comorbidity at start. The patients' main reasons for choosing DHD were related to job problems and the search for a better treatment. Most of the patients continued DHD because of improved well-being; logistic reasons accounted for the drop-outs (5 patients). The main fears were related to logistic aspects, vascular access problems, and excessive involvement of the partner on home dialysis. Improved well-being was reported by 28 of 30 patients; 2 patients reported no difference. Subjective improvement was perceived within 2 weeks in 22 of 30 patients, and within 1 month in 28 of 30 patients. An offer of a 2 - 4 week trial of DHD may help patients and caregivers to determine whether subjective and objective benefits outweigh logistic problems and whether a permanent transfer to DHD is worthwhile.