Kidney abnormalities in sickle cell disease.

Patients with sickle cell disease exhibits numerous kidney structural and functional abnormalities, changes that are seen along the entire length of the nephron. Changes are most marked in patients with homozygous sickle cell anemia, but are also seen in those with compound heterozygous states and the sickle cell trait. The renal features of sickle cell disease include some of the most common reasons for referral to nephrologists, such as hematuria, proteinuria, tubular disturbances and chronic kidney disease. Therapy of these conditions requires specialized knowledge of their distinct pathogenic mechanisms. Spanish Haemathology and Hemotherapy Association has recently publicated their Clinical Practice Guidelines of SCD management. Renal chapter is reproduced in this article for Nefrología difussion.

[Quality control systems implementation in the Spanish Dialysis Units]. / Implantación de sistemas de gestión de calidad en las unidades de nefrología españolas.

OBJECTIVE: The aim of this study was to evaluate and analyze the implementation of a Quality Management Systems (QMS) and the use of Clinical Performance measures by the Nephrology Services and Hemodialysis Units in Spain. METHOD: The Quality Management Work Group of the Spanish Society of Nephrology (SEN) realized a survey that was directed to all the Spanish Nephrology Services and Hemodialysis Units. No exclusion criteria were defined for the study. The survey was based on a multichotomous self completing "Ad Hoc" questionnaire. RESULTS: The survey was answered by 46.7% of the polled centers (44.5% were public hospitals and 55.5% private centers). Of those replying the survey 70 % had a QMS, with a higher implementation in the area of Hemodialysis (HD). The ISO 9001-2000 was the prefer QMS model chosen by 76.4% of the centers. 68.6% of the centers with a QMS were certified by an external Auditing Group. 91.7% of the Nephrology Services and Hemodialysis units were using some clinical practice guideline. A high percentage of the centers had medical protocols and nursing plans (> 90%). A significantly higher implementation of QMS was observed in Private Hospitals and Hemodialysis Units (88.8 %) when compared to public Hospitals (46.1%) (X2: 31.5; p < 0.001). The ISO 9000 Standard certification was selected by 78,3% of the private centers and by 21,7% of the public centers (X2: 37.3; p < 0.001). The certification or accreditation were done by an external auditing group in 68. 1% of the private centers compared to 31.9% for the public Hospitals (X2: 24.8; p < 0.001). Although the rate of answers prevents from extracting definitive conclusions, the result seems to indicate that in the Spanish Nephrology Community a clear trend exists towards the use QMS. This tendency suggests, that in the near future, there will be a progressive implementation and routine use of QMS in the Nephrology Community in Spain.

Thrombocytopenia and anaphylaxis secondary to heparin in a hemodialysis patient.

AIMS: We describe a rare case of anaphylaxis and thrombocytopenia whose cause was heparin used during hemodialysis sessions. CASE REPORT: A 77-year-old woman suffered five consecutive episodes of vomiting, tachypnea, wheezing or rales, immediately after initiating hemodialysis. In the first of these episodes, arterial pressure was undetectable. In all of the episodes there was evidence of the presence of hypoxia (always below 60 mmHg) and thrombocytopenia (always below l00,000/microl,, with partial platelets recovery among episodes. The episodes started immediately after hemodialysis sessions and heparin infusion; either sodium heparin or enoxaparin was used. Utilization of different filters was not able to stop the episodes. These were stopped when a switch from heparin to hirudin was tested. Tryptase levels, as a marker of mast cells activation and anaphylaxis, were not increased in two of the episodes which were assessed. IgG antibodies against heparin-PF4 complex was detected at high levels. DISCUSSION: A diagnosis of concomitant anaphylaxis and thrombocytopenia caused by sodium heparin and a low-molecular weight heparin (enoxaparin) were assumed.

[Application of process management in nephrology. Hemodialysis process management]. / Aplicación de la gestión por procesos en nefrología. Gestión del proceso hemodiálisis.

Total Quality Management techniques have recently been introduced into clinical practice. We describe the application of process management to hemodialysis therapy in a Spanish public hospital. The "ownership" of the hemodialysis process and its limits have been defined. We present a flowchart with all the activities involved in the process and the task description. Monitoring indicators have been selected according to the recommendations of the US Committee on the National Report on Health Care Delivery. Data sources for indicators have also been described.

The pharmacokinetics of a single dose of calcitriol administered subcutaneously in continuous ambulatory peritoneal dialysis patients.

OBJECTIVES: To evaluate the kinetics of calcitriol (1,25(OH)2D3) administered subcutaneously. STUDY DESIGN: Calcitriol kinetics and efficacy after subcutaneous administration were studied in 13 CAPD patients with varying degrees of increased plasma levels of parathyroid hormone (i-PTH). A single dose of 2 micrograms of calcitriol was administered subcutaneously, and its serum levels at baseline and after 1, 2, 6, 12, and 24 hours were determined. Plasma ionized calcium and i-PTH were also determined at these periods. RESULTS: Serum calcitriol levels reached peak levels of 60 and 70 pg/mL at 1 and 2 hours after administration, respectively. These levels decreased thereafter, but remained above baseline values during 24 hours. The mean value of the area under the curve (AUC) was 809 +/- 226 pg/mL/hour. Plasma i-PTH levels showed a slight decrease after 1 and 2 hours, returning to baseline levels after this period. Plasma ionized calcium did not show significant changes during the study. A slight pain at the site of injection was mentioned by some patients. CONCLUSIONS: The subcutaneous route for calcitriol administration achieves theoretically adequate plasma levels in continuous ambulatory peritoneal dialysis (CAPD) patients. This is important when parenteral administration of calcitriol is considered in the treatment of secondary hyperparathyroidism.

An analysis of adequacy of dialysis in a selected population on CAPD for over 3 years: the influence of urea and creatinine kinetics.

Adequacy of dialysis is addressed to minimize the negative consequences of underdialysis. The results of the NCSD demonstrated that urea kinetic modelling (UKM) is a useful tool in the measurement of adequacy of dialysis: Kt/V values of 1 are considered adequate to prevent complications when daily protein intake is appropriate. For CAPD no similar prospective studies exist, and consequently there is no information comparable to that for haemodialysis. Preliminary data have communicated controversial results about the usefulness of UKM for CAPD prescription. On the other hand creatinine kinetics has been proposed as an alternative to UKM to define adequacy of dialysis on CAPD. However, these results should be considered preliminary because of the small size of the series and the short observation period. The objective of this paper is to study the role of these two kinetic models in defining adequacy of dialysis in a long-term, selected CAPD population. We have studied 56 patients treated for at least 3 years on CAPD (3-11 years; mean observation period 5 years; 3996 patient-months). Urea kinetic model (UKM) (urea weekly (W) Kt/V and NPCR) and creatinine kinetics (Efficacy number (EN) and K) were determined annually. According to the present data we can conclude that Kt/V is an appropriate tool for defining adequacy in patients on CAPD. Values of weekly Kt/V greater than 1.7-1.8, with NPCR more than 1 g/kg/day, assure an adequate clinical outcome. Conversely, values on the Kt/V scale less than 1.7 entail a greater rate of complications. Our data also confirm that maintaining a serum albumin > 536-551 mumol/l (3.7-3.8 g/dl) is related to lower morbidity and mortality, and should be a major objective for CAPD patients. Consequently a relationship between dose of dialysis in terms of urea mass balance and protein nutrition can be established. After 5 years on CAPD, when all residual renal function is generally lost, Kt/V values in excess of 1.9 are difficult to achieve. Based on currently available information, we do agree with the interest of performing a prospective study on adequacy of dialysis based on the urea kinetic model in CAPD, which definitely defines its role. On the other hand, creatinine kinetics did not show sufficient discriminative capacity in terms of adequacy of dialysis. EN and Kt of creatinine values in the lower range (4.5-6 and less than 45-50 1/week respectively) were not necessarily associated with a poorer clinical outcome.(ABSTRACT TRUNCATED AT 400 WORDS)

Staphylococcus aureus nasal carrier status (SANCS) in CAPD patients; is it induced or favored by subcutaneous rHu-erythropoietin?

Staphylococcus aureus nasal carriage status (SANCS) has been recognized as a risk factor for patients on CAPD, due to a higher probability of suffering peritoneal catheter infections. The use of subcutaneous drugs (insulin dependent diabetics, drug addicts, HD patients and antiallergic vaccines), has been associated with increased risk of SANCS. On CAPD, erythropoietin (EPO) is almost universally used by the subcutaneous route. The objective of this paper was to evaluate the incidence and prevalence of SANCS in 85 CAPD patients by means of nasal smear and the influence of SANCS on peritoneal and catheter infection rate. Patients were divided in four groups according to diabetic status and EPO treatment (mean dose 2000 u. twice a week). The prevalence of SANCS in control groups was 30% in non-diabetics and 23% in diabetics. EPO treated patients showed a prevalence of SANCS of 39% in non-diabetics and 45% in diabetics due to the presence of 7 and 5 carrier patients respectively. SANCS patients (29% of the population), suffered 45% of peritonitis and 42% of exit-site infections caused by S. aureus. In a prospective part of the study, there was no difference in the frequency of developing positive cultures among EPO and control (30% of patients). No male EPO treated patients developed SANCS. We conclude that it is necessary to monitor S. aureus nasal carrier status periodically in CAPD patients especially in women. Whether or not subcutaneous erythropoietin treatment is implicated pathogenetically with SANCS, is not clarified by our data because of the frequent spontaneous appearance of SANCS among CAPD patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[The treatment of anemia in patients on hemodialysis with recombinant human erythropoietin]. / Tratamiento de la anemia de los pacientes en hemodiálisis con eritropoyetina humana recombinante.

BACKGROUND: Anaemia of chronic renal failure is primarily due to relative erythropoietin deficiency. This hormone has been recently cloned and it is now available for clinical use. METHODS: Sixteen patients maintained on haemodialysis with non-complicated anaemia and on stable clinical condition were selected for 12 months' treatment with r-HuEPO. Our aim was to analyse the factors influencing r-HuEPO response and the modifications on main haematological and biochemical parameters and adverse reactions occurrence. RESULTS: All patients responded with an increase of haemoglobin (from 78 +/- 9 to 103 +/- 18 g/dl at second month of therapy, p less than 0.001) and blood transfusions were eliminated. Time of response and doses were very different to one another. R-HuEPO requirements decreased slowly with time. Neither transfusion number, nor hyperparathyroidism, nor ferritin levels, nor diabetic condition influenced r-HuEPO response. Serum ferritin decreased significantly from 1,772 +/- 1,791 to 1,116 +/- 1,240 ng/ml (p less than 0.05), especially in patients without iron overload. Serum vitamin B12 levels did not decrease significantly. Both uric acid and phosporus increased significantly after the treatment period (5.25 +/- 1.18 to 6.29 +/- 0.99 mg/dl and 5.78 +/- 1.29 to 6.69 +/- 1.55 mg/dl respectively, p less than 0.01). Platelet counts did not modify. It was necessary to adjust antihypertensive therapy in a few patients because of a mild rise in blood pressure, although important adverse reactions did not occur. CONCLUSIONS: Anaemia of haemodialysis patients improves with r-HuEPO treatment and reduces blood transfusion requirement. Adverse effects are not very remarkable.

Treatment of the anemia with human recombinant erythropoietin in CAPD patients.

Three homogenous groups of CAPD patients, all of them with plasma hemoglobin levels lower than 8 g/dl were studied. Group 1 included 8 patients who received EPO by the subcutaneous route (s.c), at doses of 20 u./kg daily; this dose was reduced when a hemoglobin level higher than 10.5 g/dl was reached. Group 2 included 7 patients treated with EPO by subcutaneous route but at doses of 2000 units twice a week. Group 3 was constituted by 4 patients receiving EPO by intraperitoneal route (i.p.), at doses of 4000 u/day, three days a week. All patients showed an increase in hematocrit and Hb levels after three months of treatment, but the mean EPO dose was quite different comparing the groups, maintaining the reached levels at the 9th month; reticulocyte count increased only during the first month. The rest of clinical and biochemical parameters did not suffer any significant modifications. Our features showed a higher profit, that is, higher increase in Hb level with lower dose of EPO in the s.c. group in respect to i.p. group. Furthermore, we have registered a marked increase in the frequency of exogenous peritonitis in these particular patients while using i.p. EPO. In conclusion, we feel that subcutaneous route for H-R-Erythropoietin is an ideal way for this treatment, resulting in a more adequate profit ratio than that described in hemodialysis patients. The intraperitoneal route is more expensive and risky for the peritoneum, probably as a consequence of the increase of manipulations.