Renal transplantation in Caroli's disease.
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Biomedical subjects
Publications and source records attributed to R Rault.
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Although chronic dialysis patients support the use of advance directives, they rarely complete them. We asked 80 chronic dialysis patients (60 receiving in-center hemodialysis and 20 receiving peritoneal dialysis) why they had not completed an advance directive, and gave them the opportunity to complete a dialysis-specific living will and to designate a health care proxy. Questionnaires containing the dialysis-specific living will, patient demographic information, and questions about advance directives were distributed during a routine hemodialysis session or peritoneal dialysis clinic visit by a nurse working in the unit. Forty-one hemodialysis patients and 14 peritoneal dialysis patients completed the questionnaires (69% response rate). The mean age was 53 +/- 15 years and the mean time on dialysis was 5 +/- 5 years. Fifty-eight percent of the patients were women, 57% were white, 67% were hospitalized in the past year, 23% were employed, 70% had children, and 21% lived alone (43% lived with a partner and 11% lived with parents). All patients thought advance directives were a good idea, but only 35% had completed one and only seven (14%) had discussed wishes for life-sustaining therapy with their nephrologist; 34 patients (67%) had discussed their wishes with their family. Most said they had not completed an advance directive because their family knew what they would want (12 of 32 patients [38%]). Thirty-nine patients who completed the questionnaire also completed the dialysis-specific living will (71%). Those who did not complete the dialysis-specific living will chose not to because they were not sure what they would want done (12 of 16 patients [75%]). The only demographic factor that influenced completion of the dialysis-specific living will was having children: more patients with children did not complete the will (12 of 37 patients [32%] with children v two of 16 patients [13%] without children; P = 0.02). The dialysis-specific living will asks about choices for life-sustaining treatment (cardiopulmonary resuscitation and dialysis) based on one's health state (current health; permanent coma; terminal illness; mild, moderate, or severe stroke; dementia). Using patient-specific advance directives that focus on health states rather than life-sustaining interventions (eg, the dialysis-specific living will) and repeated discussion of advance directives and advance care planning initiated by dialysis unit staff may improve the completion of advance directives by chronic dialysis patients. The appropriate focus of such issues should include family involvement and health states rather than treatment interventions.
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Because little is known about the stability of knowledge, attitudes, and behavior toward advance directives in chronic hemodialysis patients, we chose to determine whether providing written information on advance directives affects chronic hemodialysis patients' knowledge, attitudes, and behavior toward advance directives over time. Various patient demographic factors were also assessed for association with the above parameters. Thirty-one chronic in-center hemodialysis patients (55% women, 48% African-Americans, 81% on dialysis for more than 3 years) completed a questionnaire consisting of patient demographic features and agreement or disagreement with statements concerning knowledge, attitudes, and behavior toward advance directives. The responses were scored from 1 (strongly agree) to 5 (strongly disagree). Patients completed the questionnaire before, shortly after (1 to 3 months), and distant to (6 to 7 months) receiving written information on advance directives. Receiving written information on advance directives did not improve patients' understanding of living wills (58% understood before, 77% shortly after, and 58% distant to receiving the information) and only transiently improved understanding of a health care proxy (32% before, 67% shortly after [P < 0.006], 55% distant [P = not significant]) and the hospital policy on advance directives (35% before, 61% shortly after [P < 0.02], 48% distant [P = not significant]). Patients' attitudes about advance directives and perceived barriers to their use were not different before, shortly after, or distant to receiving information. After receiving information on advance directives, more patients (13% before, 48% shortly after, 37% distant; P < 0.002) and their family members (10% before, 30% shortly after, 20% distant; P < 0.02) completed advance directives.(ABSTRACT TRUNCATED AT 250 WORDS)
Forty-three chronic in-center hemodialysis patients were asked about their knowledge, attitudes, and behavior towards advance directives. The survey collected patient demographic data and assessed attitudes, knowledge, and behavior towards advance directives by agreement or disagreement with statements. Sixty-six percent of patients did not know what a health care proxy was, and 42% did not know the legal status of a living will. However, 90% of older patients (> or = 60 yr of age) understood living wills. Although 77% of the patients had discussed their wishes about life-sustaining care with someone close to them, only 17% had had such discussions with their doctor. Twenty-one percent had completed an advance directive. Fifty percent of patients agreed with the statement that a potential problem with advance directives was that patients could change their minds about heroic treatments; 51% agreed that advance directives might interfere with a doctor's judgment. Only 26% agreed that a dialysis unit's promotion of the use of advance directives might lead to undertreatment. Patient age, race, marital status, and time on dialysis did not affect responses. A prior failed transplant and the educational level completed by the patient influenced some specific responses. Despite some concern, 79% of patients agreed that a dialysis unit policy asking patients whether they had completed an advance directive was a good idea.
BACKGROUND: Toxicity to interleukin-2 (IL-2) tumor immunotherapy is manifested principally by the vascular leak syndrome, hypotension, and a hyperdynamic response with low systemic vascular resistance. Nitric oxide (.N = O), a recently discovered biological mediator of vascular smooth muscle relaxation, is produced in increased amounts by numerous cell types exposed to a number of inflammatory cytokines. PURPOSE: Our purpose was to determine if there is an increased production of .N = O in patients receiving IL-2 tumor immunotherapy, and, if so, whether increases in .N = O production correlate with hemodynamic instability. METHODS: Twelve patients undergoing immunotherapy trials with IL-2 and anti-CD3 monoclonal antibody-activated lymphocytes (T-AK cells) were studied. Plasma levels of nitrate (NO3-), the stable end metabolic product of .N = O synthesis, were measured before and at the end of IL-2 treatment cycles. RESULTS: We observed a ninefold increase in plasma levels of NO3- in patients after 7 days of treatment (P less than .0001). A significant decrease in both systolic and diastolic blood pressures was observed in all patients (P less than .001). CONCLUSIONS: We propose that mediated induction of .N = O synthase enzyme leads to progressive increases in .N = O production which, in turn, produces clinically significant hypotension. IMPLICATIONS: Since .N = O synthesis can be competitively inhibited by L-arginine analogues, a possible pharmacologic modulation of .N = O production could potentially contribute to better management of toxic side effects seen in IL-2 cancer therapies.
There are few data about the prevalence and characteristics of reported sleep disorders in chronic dialysis patients and, although insomnia is often used as a marker of uremia, there are few data relating complaints of sleep to adequacy of dialysis. We therefore surveyed 48 hemodialysis (HD) patients, 22 continuous peritoneal dialysis (PD) patients, and 41 healthy control subjects about disordered sleep. The questionnaire included demographic data, questions characterizing the reported sleep problems, and linear analogue scales quantitating the severity of the sleep disturbance and feelings of anxiety, worry, and sadness. Kt/V determinations were also made for each dialysis patient. Fifty-two percent of the HD, 50% of the PD, and 12% of the control subjects reported problems sleeping (P less than 0.001, all dialysis patients v controls). No differences between HD and PD in characteristics of sleep problems were seen. Sleep severity scale results confirmed sleep disorders (7.2 in those with v 0.95 in those without sleep disorders, where 0 = sleep a little problem and 10 = a big problem, P less than 0.001). Caffeine intake (P less than 0.05) and worry (P less than 0.004) were the only factors associated with reported sleep disturbances. Kt/V values (1.4 +/- 0.3) did not predict reported sleep problems. Mean reported hours of sleep per night (5.5 +/- 2 v 5.8 +/- 1.4) and desired hours of sleep per night (8.3 +/- 2 v 7.6 +/- 1.3) were similar among dialysis patients and controls reporting sleep problems. Dialysis patients and controls without self-reported sleep disorders slept a mean of 7.1 +/- 2.4 and 7 +/- 1.1 h/night, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
Two cases of glomerulonephritis associated with non-Hodgkin's lymphoma (NHL) are described. The first patient presented with the nephrotic syndrome and normal renal function, whereas the second suffered from recurrent acute renal failure together with a unique pattern of IgM deposition within glomerular capillaries. Our review of the literature suggests that this association, although rare, has been documented in a sufficient number of cases to show that it is more than coincidental. Whereas the most common renal lesion associated with Hodgkin's disease is minimal change disease, more advanced glomerular changes are found in the patients with NHL. This is reflected in the higher incidence of renal failure in the latter patients. Treatment of the lymphomas has been shown to result in improvement or even cure of the renal disease, although long-term follow-up is rarely available in the reported cases.
British successors of Richard Bright came to regard the disease which bore his name as consisting of several distinct clinical and pathological types, one of which was referred to as contracted granular kidney. The insidious nature of this form of the disease, the lack of clear-cut precipitating factors and, above all, the associated cardio-circulatory disorders gave rise to much speculation and debate. Whether the renal disease or the vascular disease was the primary and essential change was a question which sharply divided eminent Victorian physicians and gave rise to a bitter quarrel between Sir George Johnson and Sir William Gull. The anser to this conundrum, that the kidney was sometimes the cause and sometimes the consequence of circulatory disease was suggested by Mahomed's discovery of essential hypertension but confirmation had to await the invention of a clinically useful sphygmomanometer.
Calcium carbonate is frequently used in large doses as a phosphorus binder in hemodialysis patients, which often results in hypercalcemia. In most studies in which calcium carbonate is prescribed to control serum phosphorus levels the patients are not given calcitriol. However, calcitriol may be necessary for suppression of parathyroid hormone. The risk of hypercalcemia when calcium supplements are used in conjunction with calcitriol has not previously been examined in detail. We reviewed the charts of 74 hemodialysis patients (119 patient dialysis years) to determine the relationship of serum calcium to calcitriol, calcium therapy, and PTH levels. Twenty-eight patients (38%) were hypercalcemic at some point. Calcitriol therapy significantly increased the risk of hypercalcemia, independently of calcium therapy (p = 0.032). However, patients on a low dose of calcitriol were more than twice as likely to be hypercalcemic than patients on higher doses. Mean PTH levels were lower in the patients on the lower doses of calcitriol, indicating less severe hyperparathyroid disease. We conclude that hypercalcemia is a common complication in hemodialysis patients on calcitriol and calcium carbonate. Whether lowering the dialysate calcium, as suggested by other investigators, will successfully decrease the risk of hypercalcemia without worsening hyperparathyroidism remains to be determined.
Three patients treated with intravenous immunoglobulin developed respiratory difficulty and decreased renal function. Previously reported adverse reactions to this class of drugs have included pulmonary toxicity but not, to the best of our knowledge, renal toxicity. Renal dysfunction was mild in one patient but severe in two patients, one of whom required temporary hemodialysis. In all three patients, renal function returned to the pretreatment level after stopping the drug. Urinalysis and urine sodium concentration at the onset of renal failure were consistent with pre renal azotemia although renal biopsy performed later in one patient showed acute tubular necrosis. The exact mechanism of toxicity is unknown, but the fact that all three cases occurred with a particular immunoglobulin preparation would suggest that a contaminant, possibly aggregated IgG might be responsible.
Seven patients with chronic renal failure developed a peculiar abnormality of the fingers referred to as pseudoclubbing. All had radiological evidence of severe secondary hyperparathyroidism and elevated parathormone levels measured by C-terminal assay. Treatment with vitamin D and calcium, parathyroidectomy or renal transplantation resulted in radiological healing but the deformity of the finger tips did not improve. Although these patients represent a group with severe secondary hyperparathyroidism, autonomous secretion of parathormone is not a necessary accompaniment of this disorder.
Forty patients undergoing hemodialysis were examined for bruits transmitted from the vascular access arm. In patients with forearm access, the bruits were localized to the clavicular and cervical regions on the same side. However, transmission to the sternal edge was noted in 8 of 10 patients with upper arm access. Compression of the fistula is a useful maneuver that allows the clinician to distinguish transmitted murmurs from those arising in cardiac valves.
Ten dialysis-treated patients with hypercalcemia (11.5 +/- 0.3 mg/dl, mean +/- SE) due to renal osteodystrophy were compared with 30 control dialysis-treated patients who were not hypercalcemic (9.5 +/- 0.1 mg/dl). The hypercalcemic patients were more disabled than the control patients. Fifty percent of the hypercalcemic patients and 37 percent of the control patients had a mineralization defect (p greater than 0.6). In the control group, intact parathyroid hormone level was significantly higher in patients with osteitis fibrosa than in those with osteomalacia (247 +/- 39 pg/ml versus 60 +/- 20 pg/ml, respectively, p less than 0.005) whereas in the hypercalcemic patients, parathyroid hormone measurements did not discriminate between these two types of bone disease. Osteomalacia was more severe and bone aluminum staining was stronger in the hypercalcemic patients than in the control patients (2.02 +/- 0.47 versus 0.35 +/- 0.11 mm/mm2 tissue area, p less than 0.001). The mean serum calcium level fell from 11.2 +/- 0.2 mg/dl to 10.5 +/- 0.3 mg/dl (p less than 0.01) in eight hypercalcemic patients treated with 24,25-dihydroxyvitamin D. It is concluded that hypercalcemia in patients undergoing dialysis is associated with an increase in bone aluminum level, and with more severe osteomalacia. Intact parathyroid hormone levels are useful for predicting bone histomorphometric parameters but only when hypercalcemia is not present. The drug, 24,25-dihydroxyvitamin D, was effective in lowering the serum calcium level.
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