Renal failure in a patient with two renal diseases: renal amyloidosis and rapidly progressive glomerulonephritis.
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Publications and source records attributed to J Almirall.
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We studied 134 patients with Steinert's myotonic dystrophy (MD) in order to determine the prevalence of chronic hypercapnia, the level of muscle weakness and forced expiratory volume at which hypercapnic respiratory failure is likely to occur, and how clinical assessment might help predict hypercapnic respiratory failure. Subjects were divided into five classes with a muscular disability rating scale (MDRS): 0 = no clinical impairment (n = 9), I = minimal signs of impairment (n = 11), II = distal weakness (n = 41), III = moderate proximal weakness (n = 62), and IV = nonambulatory (n = 11). The prevalence of hypercapnia (PaCO2 > or = 43 mm Hg) was found to be 0%, 27%, 29%, 45% and 55% for MDRS 0 to 4, respectively (p = 0.03). A multiple regression analysis limited to clinical data showed that daytime hypersomnolence was a significant cofactor with the MDRS (p = 0.01) in predicting PaCO2 (r = 0.40). Among respiratory parameters, FVC, respiratory muscle strength (RMS), and maximal inspiratory pressure against occluded airways (PImax) were found to be predictors of nearly equal strength, explaining 16%, 15%, and 14% of the PaCO2 variance, respectively. In multiple regression analysis, sex, daytime sleepiness, and the expected/observed FVC ratio for a given RMS were found to be significant cofactors with PImax in predicting PaCO2 (r = 0.51). It is concluded that respiratory insufficiency should be suspected in MD patients with proximal weakness or daytime sleepiness. The likelihood of hypercapnia also increases with volume restriction and respiratory muscle weakness. Our study suggests that the combination of inspiratory muscle weakness and loading plays a predominant role in the pathogenesis of chronic alveolar hypoventilation in MD patients. The occurrence of daytime hypersomnolence suggests that other factors, such as low central ventilatory drive or sleep apnea, might play an additional role.
Despite the fact that the epidemiology of community-acquired pneumonia and nosocomial Legionella infection is well known, there are no specific reports dealing with severe cases of Legionella pneumophila pneumonia admitted to intensive care units. We undertook a prospective study upon 84 patients with a reliable diagnosis of L. pneumophila pneumonia that required ICU admission. The study assessed the prognostic factors, clinical, radiological and outcome variables of both nosocomial (n = 33) and community-acquired (n = 51) cases of L. pneumophila pneumonia. The following variables were more common in nosocomial acquired as compared to community-acquired Legionella pneumonia: Chronic obstructive pulmonary disease (COPD) (64 versus 41%), cardiac disease (39 versus 10%), chronic renal failure (21 versus 4%), alcoholism (54 versus 18%), septic shock (33 versus 16%), and unilateral chest X-ray involvement (61 versus 39%). The crude mortality rate in this study was 30% (25 of 84) with no differences when comparing mortality between nosocomial (9, 27%) to community-acquired (16, 31%) types. The univariate analysis showed that cardiac disease, diabetes mellitus, creatinine > or = 1.8 mg/dl, septic shock, chest X-ray extension, mechanical ventilation, hyponatremia < or = 136 mEq/L, PACO2/FIO2 < 130, and blood urea levels > or = 30 mg/dl were factors related to poor outcome. On the other hand, the following two variables were related to better outcome: adequate treatment for Legionella and pneumonia improvement. The logistic regression analysis demonstrated that APACHE II score > 15 at admission (RR: 11.5; 95% CI 1.75 to 76.1; p = 0.025), and serum Na levels < or = 136 (RR: 21.3; 95% CI 1.11 to 408; p = 0.023), were the only independent factors related to death. On the other hand, improving pneumonia is associated with better outcome in Legionnaires' disease than for patients not having improving pneumonia (RR: 0.019; 95% CI: 0.036 to 0.106; p < 0.0001). A better understanding of the prognostic factors in cases of severe Legionella pneumonia will optimize our therapeutic approach in this disease and help to decrease both its mortality and morbidity rates.
To study the influence of vitamin D receptor (VDR) gene polymorphism on parathyroid cell function in chronic renal failure, 85 patients who had serum PTH levels <12 pmol/L (the low intact PTH [iPTH] group) and 46 patients who had serum iPTH levels >60 pmol/L (the high iPTH group) were selected out of a total dialysis population of 170 individuals. As a result of subsequent exclusions based on several criteria in both groups (diabetic patients, serum aluminum levels, serum calcium levels, and time on dialysis), the final low iPTH group consisted of 34 patients and the final high iPTH included 32 patients. A healthy control population (n = 120) and 162 of the 170-patient dialysis population served as control groups. VDR gene polymorphism was determined by digestion with the BsmI enzyme and single-strand conformation polymorphism analysis of PCR amplified fragments. Serum iPTH levels were lower in patients with the BB genotype than in those with the Bb or bb genotype, both in the total dialysis population and when the various exclusion criteria were applied. No differences in genotypic and allelic frequencies were found between the healthy control population and the high iPTH group. However, the genotypic distribution was significantly different in the low iPTH group of patients before and after applying all exclusion criteria (P = 0.037 and P = 0.018, respectively). In the final selected population, the bb genotype was less frequent in the low iPTH group than in the total dialysis population (14.7% versus 36.4%; odds ratio, 0.3; confidence interval, 0.11 to 0.82; P = 0.01). Conversely, the BB genotype was over-represented in the low iPTH group (23.3% versus 19.7%; odds ratio, 1.9; confidence interval, 0.85 to 4.3; P = 0.1). In addition, the bb genotype and the b allele frequencies were lower in the low iPTH group than in the high iPTH group (14.7% versus 34.4%, P = 0.06, and 41.2% versus 60.9%, P = 0.02, respectively), and the BB genotype and the B allele were significantly more frequent in the low PTH group than in the high iPTH group (32.3% versus 12.5%, P = 0.05, and 58.8% versus 39.1%, P = 0.02, respectively). Thus, VDR gene polymorphism influences parathyroid function in chronic renal failure.
Renal involvement is unusual in giant cell (temporal) arteritis (GCA). Microscopic hematuria and minimal proteinuria responsive to steroid therapy have been described as the most frequent renal alterations. The nephrotic syndrome in GCA has been rarely reported. We describe 2 cases of GCA with nephrotic syndrome, one at the time of diagnosis, the other 2 years after treatment. Percutaneous renal biopsy in both cases disclosed secondary renal amyloidosis confirmed by permanganate sensitivity and immunohistochemical analysis. To our knowledge, cases of GCA and secondary amyloidosis have not been reported.
The effect of aerosol challenge with distilled water, and with isotonic and hypertonic saline on the respiratory system of the anaesthetized rabbit was investigated. Nebulisation of hypertonic (3.6%) saline caused an increase in the extravascular lung water without altering the total body weight. Morphometrical investigations revealed an increase of the subepithelial tissue compartment (connective tissue and smooth muscle) of the airway wall. X-ray microanalysis showed higher content of Na, K, and Cl in this compartment already 10 min after nebulisation of hypertonic saline. The formation of oedema was associated with a significant decrease in both compliance and gas exchange.
BACKGROUND: Fine needle aspiration is a useful technique as a monitorization method after kidney transplantation for diagnosis of rejection. In addition to the cells of the intrarenal inflammatory infiltrate, the material obtained contains an important quantity of parenchymatous renal cells. The expression of class II HLA antigens in renal tubular cells has been related with episodes of rejection. The aim of this study was to determine the clinical usefulness of fine needle aspiration in diagnosis and control of kidney transplant rejection. PATIENTS AND METHODS: HLA-DR expression of renal tubular cells obtained in 155 fine needle aspirations carried out in 36 consecutive kidney transplantations were prospectively studied. The technique used was that of alkaline phosphatase. The study period included the two months following the graft. RESULTS: Rejection was considered to occur on expression of HLA-DR in more than 20% of tubular cells and as thus this method showed a sensitivity and specificity of 78 and 79%, respectively. Likewise, a good correlation was observed between the persistence of tubular HLA-DR expression following rejection treatment and severity of the same. The group of patients with stable renal function presented a slight, transitory expression within the first 10-12 days after transplantation. CONCLUSIONS: HLA-DR expression of the tubular cells obtained by aspiration puncture is a useful method for the diagnosis and control of kidney transplant rejection.
The validation of an automatic urea analyser used in the monitoring of hemodialysis processes is reported. The analyser can indirectly determine dialysis parameters as dialysis delivery (KT/V) and protein catabolism (PCRn). These parameters are useful for the prescription and optimization of hemodialysis. The analyser, based on a previously-reported flow-injection analytical biosystem, was connected on-line to the effluent of a dialysis machine during several hemodialysis sessions. The urea concentration data were continuously processed and dialysis parameters were obtained in quasi real time by means of the integration of an adjusted time-dependent exponential function. These values were compared with those obtained by applying the methods traditionally employed in hospital laboratories. The evaluation comprised 24 data sets from several patients of different gender and age. No significant differences were found between the KT/V and PCRn results obtained with the usual method and those results produced by the analyser proposed here.
In order to better understand the potential malignancy of renal neoplasms arising in patients with acquired renal cystic disease and to try and establish differences from other renal tumours we analysed DNA ploidy as well as the level of S-phase fraction in 11 neoplasms associated with acquired cystic disease by means of flow cytometry. The results were correlated with known prognostic factors such as nuclear grade, size and stage, as well as the clinical behaviour of the tumours. We found a close relationship between DNA aneuploidy and high S-phase fraction and a poor clinical outcome. We also found some differences in the DNA ploidy profile of these tumours when compared with those reported in other renal neoplasms.
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All patients with severe pneumonias (community-acquired and nosocomial) who required treatment in the intensive care unit (ICU) were included in a 3-year prospective study. Predictive factors for a fatal outcome were analyzed in 127 patients. An etiologic diagnosis was made in 70 (55.1%) patients. Culture of sputum or tracheobronchial secretions were used only as criteria for microbiologic diagnosis of Legionella pneumophila. The pathogens most frequently identified were L pneumophila, Streptococcus pneumoniae, and Pseudomonas aeruginosa. Viruses were not detected as causative agents. A total of 54 patients died (mortality rate, 42.5%). The univariate analysis showed the following factors associated with mortality: advanced age (> or = 70 years); presence of septic shock, requirement of mechanical ventilation, and Simplified Acute Physiology Score [SAPS] index > 12 at the time of admission to the ICU or when symptoms appeared in patients already admitted to the ICU; development of any complication during ICU hospitalization; and P aeruginosa as the etiologic agent of the pneumonia. When all variables were introduced by a stepwise method, the final model included advanced age (> or = 70 years), SAPS index > 12, presence of septic shock, requirement of mechanical ventilation, bilateral pulmonary involvement, and P aeruginosa as the etiologic agent of pneumonia as prognostic factors associated with a fatal outcome.
Isolated mesangial C3 proliferative glomerulonephritis is a nephropathy poorly defined among glomerular diseases. Clinical picture is characterized by episodes of gross hematuria and/or persistent or recurrent microhematuria and/or proteinuria. Short-term prognosis (less than 3 years) is considered to be benign, although not much information is available in reference to long-term follow-up. We reviewed all renal biopsies performed in our center between 1978 and 1992 (n = 2,200) in order to study clinical course of these patients. Isolated mesangial C3 deposition was found in 11 cases (0.5%). Isolated proteinuria (> 0.5 g/d) was present in 3 patients and nephrotic syndrome in 2. Hematuria with proteinuria was detected in 5 patients. In one patient hematuria was the only clinical finding. Arterial hypertension was observed in 4 cases. At the time of renal biopsy, renal function was accurately normal in all but one patient. In our series, renal function showed no changes during the first 3 years, accordingly to data referred to in the literature. After 7 years of follow-up, however, renal function was declining in 4 of 5 patients who developed terminal renal failure up to requiring hemodialysis. Therefore, initial benign prognosis ascribed to this glomerulonephritis would not be confirmed in a long-term follow-up.
111In-labeled platelet scintigraphy was used to confirm immunological intolerance as the cause of prolonged febrile syndrome in hemodialyzed patients with a nonfunctioning renal allograft. Thirty-six patients with febrile syndrome (> 38 degrees C) and a nonfunctioning renal graft were studied. Thirteen patients were under minimal steroids (5-10 mg/day) and 23 were free of immunosuppressive therapy. A control group of 6 patients without fever and with a nonfunctioning renal graft was also included. The labeling of autologous platelets with 111In-mercaptopyridine was performed following a modified technique of Thakur. Scans were obtained at 24 and 48 hr after injection of 100-200 microCi of 111In-labeled platelets. A platelet uptake index (PUI) was calculated to evaluate the results of the scintigraphy. A PUI > 1.5 at 24 or 48 hr was considered positive and suggestive of immunological activity in the nonfunctioning renal allograft. In the study group the PUI was considered positive in 26 patients and negative in 10. In 3 patients with positive PUI, fever disappeared after steroid treatment, and transplantectomy was performed in the remaining 23. In 8 of the 10 patients with a negative PUI, fever disappeared with antimicrobial therapy. In the control group, a negative PUI was obtained in all cases. The sensitivity of PUI in demonstrating immunological intolerance of the nonfunctioning renal allograft was 93% with a specificity of 100%. Our results suggest that this new approach with 111In-labeled platelet scintigraphy may constitute a good marker for discriminating the origin of the febrile syndrome in patients with a nonfunctioning renal allograft. A positive PUI (> 1.5) strongly suggested immunological intolerance of the nonfunctioning allograft.
BACKGROUND: In advanced cases of secondary hyperparathyroidism (HPT) in terminal chronic renal failure, the oral treatment of calcitriol is not possible due to the development of hypercalcemia. It has been demonstrated that calcitriol directly inhibits the secretion of parathormone (PTH), independently of calcium. Therefore the intravenous administration of high and intermittent doses of calcitriol (ivVD) has been proposed as an alternative treatment for resistant HPT. METHODS: The response of PTHi to treatment over 6 months with ivVD was evaluated in 18 patients with moderate-severe HPT resistant to the classical schedules. RESULTS: An important decrease was observed in the PTHi values (basal: 698 +/- 277 pg/ml, end of treatment: 272 +/- 200; p < 0.05). Alkaline phosphatase values followed a parallel course (basal: 476 +/- 286 U/l, end of treatment: 301 +/- 276 U/l, p < 0.05). Fifty-five percent of the patients presented hypercalcemia at some time (Ca > or = 11.5 mg/dl) being controlled by a decrease in the doses of calcitriol or calcium in dialysis fluid. No response was observed in 3 patients (17%) with treatment being discontinued due to presentation of uncontrollable hypercalcemia. CONCLUSIONS: The administration of intravenous calcitriol at high and intermittent doses is effective in a considerable number of patients with hyperparathyroidism resistant to the classical oral schedules. Its use may avoid surgical parathyroidectomy in some cases.
Recent in vitro and in vivo studies have shown that calcium acetate (CaAC) is a more effective phosphorus binder than, among other calcium salts, calcium carbonate (CaCO3). More efficient binding allows serum phosphorus to be controlled with a lower dose; moreover, less calcium seems to be absorbed when CaAC is used. These properties could reduce the incidence of hypercalcemia; however, in clinical practice few reports have compared these two calcium salts, and results disagree. We evaluated in a 24-week prospective cross-over study the clinical efficiency of CaCO3 and CaAC in 10 selected chronic hemodialysis patients. Only 7 patients completed the study period. The patients were randomly assigned to start treatment with one of the two calcium salts; after 12 weeks they shifted to the other treatment. Serum analytical tests included weekly control of calcium, phosphorus, and alkaline phosphatase. PTH values (intact molecule) were obtained initially and at the end of every study period. The same good control of the phosphorus level (4.79 +/- 0.6 vs. 4.94 +/- 0.8 mg/dl) was obtained with CaAC (mean doses 4.1 +/- 0.3 g/day) as with CaCO3 (mean doses 4.01 +/- 0.8 g/day). The mean serum calcium levels were similar (10.36 +/- 0.5 vs. 10.20 +/- 0.5 mg/dl). The dose of elemental calcium administered was significantly less with CaAC (957 +/- 83 mg/day) than with CaCO3 (1,590 +/- 317 mg/day). However, the incidence of hypercalcemia (Ca > 11 mg/dl) was similar during the two treatment periods (13% with CaAC vs. 14% with CaCO3). Also the incidence of Ca x P products 765 was comparable (9.5 vs. 11.9%).(ABSTRACT TRUNCATED AT 250 WORDS)
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A 67-year-old woman with rheumatoid arthritis presented rapidly progressive glomerulonephritis (RPGN) after 5 months of D-penicillamine (250 mg/day) treatment. Light microscopy study showed severe glomerulonephritis with crescent formation in 60% of the glomeruli and infiltration of inflammatory cells in the wall of an arteriole. Immunofluorescence revealed scanty granular IgG, IgA and C3 deposits along the capillary walls and mesangium. The patient was treated with steroid pulse, plasmapheresis, cyclophosphamide and antiplatelet agents. A complete recovery of renal function was achieved in a few weeks. This new case of RPGN in the course of D-penicillamine treatment emphasizes the need for frequent monitoring of renal function and evaluation of urinary sediment and proteinuria in these patients. The prompt discontinuation of D-penicillamine and vigorous treatment measures could allow for a good prognosis as in this case.
The relevance of eosinophilia in the physiopathology of transplant rejection has yet to be established. The appearance of eosinophilia has been occasionally associated with an adverse prognosis on graft rejection episodes. The aim of the present study was to evaluate the role and prognostic implications of blood and graft eosinophilia in kidney transplant rejection. We have examined the intrarenal infiltrate in 173 fine-needle aspiration biopsies from 36 consecutively transplant patients, and blood samples obtained simultaneously with fine-needle aspirations. Two different immunosuppressive regimens were administered: triple therapy (azathioprine + prednisone + antilymphocytic globulin) in patients with posttransplant acute tubular necrosis and cyclosporine A monotherapy in the rest of the patients. Comparing the two immunosuppressive groups, more elevated eosinophil values were observed in the monotherapy group during stable graft and also at the rejection episode. In the monotherapy group, a significant increase in the eosinophil values, in peripheral blood samples and in the intragraft infiltrates were noted at the rejection episode with respect to the stable situation. Following pulsed-steroid treatment an immediate disappearance of the eosinophils was evident. In contrast, no differences could be demonstrated between these two clinical situations in the TT group. Higher rates of eosinophils in the intrarenal infiltrate with respect to peripheral blood samples were observed during rejection episodes, suggesting some role of the eosinophils in the physiopathology of graft rejection.(ABSTRACT TRUNCATED AT 250 WORDS)