Patients' representations of their end-stage renal disease: relation with mortality.

BACKGROUND: Self-regulation theory explains how patients' illness perceptions influence self-management behaviour (e.g. via adherence to treatment). Following these assumptions, we explored whether illness perceptions of ESRD-patients are related to mortality rates. METHODS: Illness perceptions of 182 patients participating in the NECOSAD-2 study in the period between December 2004 and June 2005 were assessed. Cox proportional hazard models were used to estimate whether subsequent all-cause mortality could be attributed to illness perception dimensions. RESULTS: One-third of the participants had died at the end of the follow-up. Mortality rates were higher among patients who believed that their treatment was less effective in controlling their disease (perceived treatment control; RR = 0.71, P = 0.028). This effect remained stable after adjusting for sociodemographic and clinical variables (RR = 0.65, P = 0.015). CONCLUSIONS: If we consider risk factors for mortality, we tend to rely on clinical parameters rather than on patients' representations of their illness. Nevertheless, results from the current exploration may suggest that addressing patients' personal beliefs regarding the effectiveness of treatment can provide a powerful tool for predicting and perhaps even enhancing survival.

Th1/Th2 cytokine imbalance in a family with hyper-IgE syndrome.

BACKGROUND: Hyperimmunoglobulin E (hyper-IgE) syndrome is a rare immunodeficiency characterised by recurrent skin and respiratory tract infections, skeletal and dental abnormalities, chronic eczema, and elevated serum IgE. We describe a family with four hyper-IgE syndrome patients (38, 37, 30 and 7 years old), in which we investigated the cytokine response to both specific and non-specific stimulation. METHODS: Whole blood from patients and volunteers was stimulated for either 24 or 48h at 37 degrees C with heat-killed Staphylococcus, C. albicans or a combination of IL-12 and IL-18. Cytokine concentrations in the plasma were measured by specific radioimmuno-assays or ELISA. RESULTS: Serum IgE ranged from 5,000 to 16,670 IU/ml, and neutrophil chemotaxis was normal in all four patients. Tumour necrosis factor, interleukin (IL)-1beta, IL-6 and IL-8 production after stimulation of whole-blood cultures with lipopolysaccharide or heat-killed S. aureus did not differ between the adult patients and four healthy controls. In contrast, when blood from patients and controls was stimulated with heat-killed S. aureus or C. albicans, a severe imbalance towards a Th2 phenotype was found, with 10- to 30-fold reduction in the IFNgamma/IL-10 ratios in the hyper-IgE syndrome patients. The IFNgamma production in the patients was less severely impaired when blood was non-specifically stimulated with a combination of IL-18 and IL-12. CONCLUSION: In this family with hyper-IgE syndrome, the imbalance in the Th1/Th2 cytokine production may have been involved in the pathogenesis of the recurrent infections and/or chronic eczema characteristic of this disease.

Fibrillary-immunotactoid glomerulopathy with renal deposits of IgAlambda: a rare cause of glomerulonephritis.

We describe a 24-year-old patient who presented with a nephrotic syndrome. His renal biopsy revealed a diffuse mesangioproliferative glomerulonephritis with eosinophilic deposits. Electron microscopy showed organized, Congo-red negative deposits, forming microtubules of about 20 nm width in the capillary walls and in the mesangium, establishing a diagnosis of fibrillary-immunotactoid glomerulopathy. Fibrillary-immunotactoid glomerulopathy is a rare cause of glomerulonephritis, characterized by Congo-red-negative glomerular deposits of fibrils, sometimes organized in microtubules, predominantly containing IgG and C3. Patients clinically present with the nephrotic syndrome, hematuria and hypertension. The pathogenesis of this glomerulopathy has not been elucidated yet. In our patient, the renal deposits contained IgAlambda. This peculiar feature is suggestive of an underlying paraproteinemia. However, in the serum no paraproteins or cryoglobulins were found, and also microscopical examination and immunophenotyping of the bone marrow did not point to the presence of a monoclonal plasma cell dyscrasia. Our patient was not treated with immunosuppressive drugs and he is currently progressing to end-stage renal disease.

[Ocular infection by Pseudomonas aeruginosa in a mechanically ventilated patient]. / Ooginfectie door Pseudomonas aeruginosa bij een mechanisch beademde patiënt.

A 59-year-old man developed bilateral keratitis several weeks after the initiation of mechanical ventilation because of respiratory failure and sepsis following abdominal surgery. Colonisation of the upper airways by P. aeruginosa had been established before. Invasion through corneal epithelial defects based on dehydration keratitis was the presumed route of infection. Despite aggressive treatment, including antibiotics, the infection was rapidly progressive in both eyes. The patient died of deterioration of his general condition. In order to prevent such eye infections in a patient on mechanical ventilation, there is a need of good eye care, prevention of corneal lesions and alertness, especially when the patient is colonised by virulent micro-organisms like P. aeruginosa.

Optimizing screening procedures for Staphylococcus aureus nasal carriage in patients on haemodialysis.

BACKGROUND: So far it remains unclear what the optimal screening method for detection of S. aureus nasal carriage in patients on haemodialysis is with regard to number of cultures performed, culture interval, and necessity of a broth-enrichment procedure. METHODS: A prospective, uncontrolled study was performed at the renal unit of a tertiary care centre, including all haemodialysis patients (n=91) attending the unit during the study period. The purpose was to determine the optimal screening method for S. aureus nasal carriage in patients on haemodialysis. RESULTS: When compared to the conventional culture method, inclusion of a broth-enrichment procedure increased the number of cultures positive for S. aureus significantly (31 vs 24%, P<0.0001). Of 91 patients 37% were S. aureus carriers (defined as at least 1 of 5 cultures positive), 33% were stable carriers (defined as at least 2 of 5 cultures positive). Fourth and 5th cultures, taken at subsequent dialysis sessions, captured only two additional carriers (6% of all carriers). With respect to culture results and identification of carrier status a short (1-h) and a long (>24-h) sampling procedure showed no significantly different results. CONCLUSIONS: S. aureus nasal carriage in haemodialysis patients can be conveniently established with three nasal cultures taken with 1-h intervals, and the inclusion of a broth-enrichment procedure.

An alternative route for multistep tumorigenesis in a novel case of hereditary renal cell cancer and a t(2;3)(q35;q21) chromosome translocation.

Through allele-segregation and loss-of-heterozygosity analyses, we demonstrated loss of the translocation-derivative chromosome 3 in five independent renal cell tumors of the clear-cell type, obtained from three members of a family in which a constitutional t(2;3)(q35;q21) was encountered. In addition, analysis of the von Hippel-Lindau gene, VHL, revealed distinct insertion, deletion, and substitution mutations in four of the five tumors tested. On the basis of these results, we conclude that, in this familial case, an alternative route for renal cell carcinoma development is implied. In contrast to the first hit in the generally accepted two-hit tumor-suppressor model proposed by Knudson, the familial translocation in this case may act as a primary oncogenic event leading to (nondisjunctional) loss of the der(3) chromosome harboring the VHL tumor-suppressor gene. The risk of developing renal cell cancer may be correlated directly with the extent of somatic (kidney) mosaicism resulting from this loss.

A familial case of renal cell carcinoma and a t(2;3) chromosome translocation.

Cytogenetic analysis was performed on peripheral blood lymphocytes of members of a family with inherited renal cell cancer. Four family members in three generations developed multiple/bilateral renal cell carcinomas of the clear cell type. In one additional case a bladder carcinoma was diagnosed. In two of the renal cell carcinoma patients a constitutional t(2;3)(q35;q21) was encountered, whereas in the two other (deceased) patients the presence of this translocation could be deduced. Also, the bladder cancer patient was found to be positive for t(2;3)(q35;q21). This is the third familial renal cell carcinoma-associated chromosomal translocation ever described. The previously reported cases also involved chromosome 3, thereby supporting the notion that this chromosome may play a crucial role in the development of renal cell carcinomas. Interestingly, the translocation breakpoints in these three families map at different locations, suggesting that multiple genes on chromosome 3 may be involved.

Nasal carriage and peritonitis by Staphylococcus aureus in patients on continuous ambulatory peritoneal dialysis: a prospective study.

OBJECTIVE: To establish whether or not patients on continuous ambulatory peritoneal dialysis (CAPD) using current infection control measures who are nasal carriers of Staphylococcus aureus are at risk for the development of S. aureus peritonitis. DESIGN: A prospective 22-month study analyzing nasal and skin/nasal (i.e., nasal and/or exit-site) carrier status for S. aureus and peritonitis episodes. Nasal swab cultures for S. aureus were taken with 1- to 3-month intervals; swab cultures from the catheter exit site were taken only when infection was suspected. SETTING: Renal unit, tertiary-care center. PATIENTS: All patients on CAPD at our center that could be observed during at least 2 months. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Nasal and skin/nasal carrier status, occurrence of peritonitis. RESULTS: Of 54 enrolled patients, 31 (57%) were nasal carriers for S. aureus: 6 of these 31 developed S. aureus peritonitis as opposed to none of 23 non-carriers (p = 0.03). The S. aureus peritonitis rate in 28 skin/nasal carriers was increased when compared to non-carriers (p = 0.02), but there was no difference between chronic and intermittent skin/nasal carriers (p = 0.63). CONCLUSIONS: In our population, nasal carriers are at increased risk for the development of S. aureusperitonitis. Further studies should evaluate the effect of eradication of nasal carriage of S. aureus and the effect of additional preventive hygienic measures on the occurrence of peritonitis by S. aureus.

Outcome and complications in patients treated with continuous ambulatory peritoneal dialysis (CAPD) at a single centre during 11 years.

OBJECTIVE: To evaluate outcome and complications in patients treated with CAPD at our centre a retrospective study was performed. METHODS: Relevant data from all 123 consecutive patients on the CAPD program from 1982 to 1994 were reviewed. RESULTS: Patient survival after 1, 2, 3 years was 89, 78, 69 and 50% respectively. The probability of having a functioning catheter after 1, 2, 3 and 5 years was 90, 77 and 68%, respectively. We observed 179 technical complications (42 intra-abdominal pressure, 137 catheter-related). Peritonitis (220 episodes, 62% gram-positive) occurred with a mean incidence of 1 episode in 13 treatment months and was the main reason (26 cases, 68% gram-positive) for catheter removal. (Mixed) Gram-negative peritonitis was associated with a higher mortality, and relatively more often resulted in termination of CAPD treatment when compared to gram-positive peritonitis. Introduction in 1988 of a new fluid exchange system (Twin Bag) and alcohol disinfection of hands was accompanied by a decreased incidence of peritonitis and exit-site infections, but to date patient, technique and catheter survival have not improved. CONCLUSION: Although therapeutic measures have resulted in a reduced incidence of peritonitis and exit-site infections, infectious and technical complications remain a serious threat to patient and technique survival in CAPD treatment.

Antineutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in a patient with anti-glomerular basement membrane disease.

An unusual case is described of a patient with both anti-glomerular basement membrane (GBM) disease and anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity (MPO-ANCA) presenting with acute renal failure. Four years before a seronegative arthritis of the left wrist was diagnosed. Tests for ANCA by indirect immunofluorescence were repeatedly negative. The diagnosis was made by renal biopsy and by testing the serum with specific enzyme-linked immunosorbent assays (ELISA) for MPO-ANCA and anti-GBM antibodies. To our knowledge, this is the first patient presenting with such findings in the Dutch literature.

[Tubulointerstitial nephritis caused by mesalazine (5-ASA) agents]. / Tubulo-interstitiële nefritis door mesalazine (5-ASA)-preparaten.

In five patients (4 men aged 34, 20, 22 and 29 years, and a woman of 34 years) tubulo-interstitial nephritis developed during treatment with 5-aminosalicylic acid (5-ASA) containing drugs for colitis ulcerosa, proctocolitis or Crohn's disease. Two patients had an impaired renal function before treatment with 5-ASA medication. In all patients there was only an incomplete recovery of renal function after cessation of the therapy. It is therefore necessary to monitor renal function regularly in patients receiving 5-ASA containing preparations.

Familial hypokalemia/hypomagnesemia and chondrocalcinosis.

OBJECTIVE: Familial occurrence of Bartter's syndrome is well known, but the simultaneous occurrence of hypokalemia/hypomagnesemia and chondrocalcinosis in one family has not been described. We present the clinical, laboratory and radiological findings of a family, in which 7 members were affected by disease. METHODS: A total of 43 members of the family could be interviewed concerning their general health, past diseases and joint complaints. Serum potassium and magnesium were determined in all and radiographic studies were performed in those who had hypokalemia and hypomagnesemia or those with merely articular complaints. Urinary excretion of potassium, magnesium and calcium were determined in the affected persons. RESULTS: Seven patients were found with hypokalemia and hypomagnesemia. Urinary potassium and magnesium excretion was inappropriately high when compared to the serum levels of these electrolytes. All patients had hypocalciuria and extensive chondrocalcinosis, mainly in the knees, elbows and shoulders. In one patient, most probably as a result of magnesium supplementation, a striking reduction of chondrocalcinosis was observed during a followup of 10 years. CONCLUSION: A family with familial hypokalemia/hypomagnesemia and chondrocalcinosis is described. The reduction of chondrocalcinosis, after years of magnesium supplementation in one patient, suggests that hypomagnesemia is an important factor in the pathogenesis of chondrocalcinosis in these patients.

A patient with postpartum primary hypothyroidism and acquired von Willebrand's disease.

Patients with hypothyroidism often complain about easy bruising. We describe a patient with a postpartum acquired bleeding tendency. Hypothyroidism was suspected and confirmed biochemically. After substitution therapy with levothyroxine the bleeding tendency recovered completely. The coagulation disorder appeared to be based on an acquired von Willebrand's disease, secondary to the hypothyroidism. In patients with von Willebrand's disease underlying diseases have to be excluded.

Association of autoantibodies to myeloperoxidase with different forms of vasculitis.

Antineutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase (MPO) were found in 53 patient sera that were routinely submitted for antineutrophil cytoplasmic antibody determination. Based on clinical and histologic criteria, 15 of these 53 patients were classified as having systemic necrotizing vasculitis of the polyarteritis group, 11 patients were classified as having Wegener's granulomatosis (WG), and 14 were classified as having idiopathic crescentic glomerulonephritis. The remaining 13 patients did not fulfill the diagnostic criteria for these disorders, although most of these patients had clinical symptoms compatible with these disorders. While all patients with WG had renal involvement, only 4 of the 15 patients with systemic necrotizing vasculitis of the polyarteritis group had glomerulonephritis. The sensitivity of autoantibodies to MPO for either systemic necrotizing vasculitis of the polyarteritis group, WG, or idiopathic crescentic glomerulonephritis was further tested in all our patients with these disorders (n = 104). Twenty-seven of 104 patients had autoantibodies to MPO. Furthermore, 69 of the remaining 77 patients had autoantibodies specific for the 29-kd serine protease, which has been reported to be specifically associated with WG. Sera from 8 patients were negative for either of these antibodies (92% sensitivity of autoantibodies to MPO and/or the 29-kd serine protease). The specificity of autoantibodies to MPO for either systemic necrotizing vasculitis of the polyarteritis group, WG, or idiopathic crescentic glomerulonephritis was also tested in selected groups of patients who had closely related diseases. Two of 144 patients had autoantibodies to MPO (specificity 99%).(ABSTRACT TRUNCATED AT 250 WORDS)

A sporadic case of acute Hantavirus nephropathy in The Netherlands.

A 32-yr-old man presented with acute renal failure preceded by a viral-like disease with high fever, bilateral loin pain, nausea, headache and slight thrombocytopenia. Renal biopsy revealed only minor tubulointerstitial abnormalities. Renal function completely normalised within 10 days after hospitalisation. The diagnosis of acute Hantavirus nephropathy was proved by serological examinations.

Non-Hodgkin lymphoma with unique localization in the kidneys presenting with acute renal failure.

An unusual case of acute renal failure due to infiltration of the kidneys by non-Hodgkin lymphoma is described. The diagnosis was made by renal biopsy. Extensive clinical, laboratory, and radiological examination revealed that the kidneys were the only localization of the lymphoma.

Difference in sodium sensitivity in human hypertensives.

The concept of sodium sensitivity of the blood pressure is discussed in relation to the "early" studies on rigid sodium restriction and the more recent studies on moderate sodium restriction in hypertensive patients. Data on the antihypertensive efficacy of moderate sodium restriction are conflicting, probably as a result of differences in sodium sensitivity of the study populations. Evidence is presented that the renin-angiotensin-aldosterone system and the adrenergic nervous system are important determinants of the blood pressure response to sodium depletion and sodium loading, respectively. The fact that sodium sensitivity is related to age and to the height of the blood pressure limits the therapeutic application of moderate sodium restriction in clinical practice.