Myelodysplasia during the course of myeloma. Restriction of 17p deletion and p53 overexpression to myeloid cells.

We report a case of myelodysplastic syndrome (MDS) occurring during the course of multiple myeloma (MM) treated by alkylating agents. Karyotype showed unbalanced t(5;17), resulting in 17p deletion. Dysgranulopoïesis typical of the '17p-syndrome' and p53 mutation and overexpression were present. A combination of FISH and immunophenotype analysis (FICTION, analysis) showed that 17p deletion was restricted to myeloid cells, and that p53 overexpression was also restricted to myeloid cells. These findings strongly argue against a common clonal origin of MM and MDS, and support the hypothesis that MM and MDS were clonally unrelated, and that MDS was indeed secondary to treatment with alkylating agents.

[Results of infrainguinal revascularization in patients with end-stage renal disease]. / Résultats des revascularisations sous-inguinales chez les malades en insuffisance rénale terminale.

OBJECTIVES: The aim of this retrospective study was to evaluate the efficacy of bypass in patients with endstage renal disease (ESRD) and to determine predictive factors and precise bypass indications. METHOD: Forty one patients with ESRD underwent 50 bypass, 6 limbs were stage II and 44 stage III or IV according to Leriche and Fontaine classification. Revascularisations procedures were 47 infrainguinal bypass and 3 miscellaneous. Median follow up was 17,0+/-15,7 months. RESULTS: Perioperative mortality rate was 12% (n = 6). Morbidity was as follow : 1 perioperative major imputation and 8 secondary ones. There were 26 secondary death (12 from cardiac events), cumulative survival rate declined to 42,9+/-7,7% and limb salvage rate to 77,2+/-7,5% at 2 years. Primary and secondary potency rates were 53,5+/-10,4% and 70,6+/-10%. Quality of life was good in 28% of revascularised patients. Among risk factors, myocardial events showed a statistical significance in predicting survival, good runoff and bypass occlusion showed a statistical significance in predicting limb salvage. CONCLUSION: Revascularisation can be performed in ESRD patients. However to improve the results full evaluation of myocardial risks, skin lesions and infection of the feet, available autologous vein and nutritional status may be needed in those patients.

Glomerular injury in chronic myelomonocytic leukemia.

Among 825 cases of de novo myelodysplastic syndromes (MDS) diagnosed over a period of 13 years in our center, 4 had clinically significant glomerulopathy. All 4 fulfilled diagnostic criteria of chronic myelomonocytic leukemia (CMML), and could be classified in the low or intermediate risk groups according to two scoring systems. Presenting symptoms of renal involvement were edema in 3 cases and acute renal failure in the remaining patient. Three patients had the nephrotic syndrome. Renal biopsy (performed in 2 cases but considered as contraindicated in the other cases) showed AL amyloidosis on one case and extracapillary glomerulonephritis in the other case. The 4 patients were treated with V16 or hydroxyurea and two had renal improvement. Only one previous case of MDS associated with glomerulopathy has been reported before and also very probably had CMML. This, and the response of renal disease to chemotherapy in 2 of our patients suggests a possible relationship between the two disorders. More systematic investigation of glomerular function, in CMML, could possibly disclose a higher incidence of cases of glomerular injury in this type of MDS.

Exposure of hemodialysis patients to di-2-ethylhexyl phthalate.

The migration of di-2-ethylhexyl phthalate (DEHP) from dialyzers was studied in 21 patients with chronic renal failure undergoing maintenance hemodialysis. The circulating concentrations of DEHP were measured by high performance liquid chromatography in blood of patients obtained from the inlet and the outlet of the dialyzer during a 4-h dialysis session. During treatment of renal failure using plasticized tubing, the plasma level of DEHP increased. On average, an estimated 75.2 mg of DEHP was extracted from the dialyzer during a single dialysis session, with a range of 44.3-197. 1 mg. On the other hand, the total amount of DEHP retained by the patient during the dialysis session was evaluated by the difference between the AUCout and the AUCin and ranged from 3.6 to 59.6 mg. The rate of extraction of DEHP from the dialyzer was correlated (r=0.705, P<0.05) with serum lipid content (cholesterol and triglyceride).So, we confirmed that patients on hemodialysis are always regularly exposed to considerable amounts of DEHP. However, several metabolic effects have been reported in various animal species following treatment with DEHP, such as changes in lipid metabolism and in hepatic microsomal drug-metabolizing enzyme activities. DEHP is now a well-known hepatic peroxisomal proliferator in rodents and an inducer of many peroxisomal and non-peroxisomal enzymes. So, lipid metabolism modifications and hepatic changes observed in hemodialysis patients could be explained from chronic exposition to DEHP. In the coming years, it seems necessary to reconsider the use of DEHP as a plasticizer in medical devices. Highly unacceptable amounts of DEHP leached during the dialysis session could be easily avoided by careful selection of hemodialysis tubing.

[Muscle infarction. An unknown complication of diabetes mellitus]. / L'infarctus musculaire. Une complication méconnue du diabète.

INTRODUCTION: Diabetic muscle infarction (MI) is a rare and little-known complication of diabetes mellitus. CASE REPORT: We report a case of relapsing MI in which magnetic resonance imaging (MRI) suggested the diagnosis. A 53-year-old man with multi-complicated type II diabetes mellitus was admitted to our unit for illness and deep tumefaction of the right thigh. Because of unconclusive MRI, a muscular biopsy of the lesion was performed and MI confirmed. Three months after, a left relapse of MI occurred. Immediate treatment with immobilization and heparinotherapy permitted a rapid recovery. CONCLUSION: About 70 previously reported cases are reviewed. The mean age at presentation was about 40 years. MI was usually seen in patients with long-standing diabetes with multiple end organ microvascular complications. Homo- or heterolateral recurrences are reported in almost half of the patients. MRI is the best imaging technique for suggesting the diagnosis.

Molecular identification of Pasteurella dagmatis peritonitis in a patient undergoing peritoneal dialysis.

Pasteurella dagmatis was identified as the etiologic agent of peritonitis in a continuous ambulatory peritoneal dialysis patient by utilizing a molecular kit in our hospital's clinical laboratory. This method would appear a useful approach to identify a species of Pasteurella not included in the existing database of commercial identification kits when discrepancies exist between phenotypic tests.

[Pregnancy and hemodialysis]. / Grossesse en hémodialyse chronique.

Pregnancy is a rare occurrence in patients on chronic hemodialysis (CHD). The rate of successful pregnancies amounts to almost 60%, thanks to modifications of the dialysis schedule and a specifically adapted obstetrical and neonatal management. We report on seven pregnancies occurring between 1995 and 2001 in six women with a mean age of 32 years (22-39 years), on HD for a mean period of 36 months (12-96 months). Maternal and fetal complications, and the long-term outcome of mothers and children are reported, and the collaborative approaches adopted by obstetrician, pediatrician and nephrologist are discussed. The frequency and length of HD was systematically increased. One patient chose to terminate her pregnancy at 20 weeks of gestation. The mean gestational age for the six other pregnancies was 31 weeks (24-34 weeks) with an average weight at birth of 1495 g (660-1920 g). One neonate born at 24 weeks died 2 days following delivery. One patient was treated with uterine artery embolization for post-partum haemorrhage. Pediatric evaluation of the five children, who were followed up for a period ranging between 2.5 to 5.5 years, showed a good long-term outcome. In conclusion, pregnancy needs not be counterindicated or systematically terminated in patients on CHD, particularly if transplantation is not possible, if the patient refuses it, or if she is relatively old and there is a long waiting period before transplantation.

[Acute interstitial nephritis and streptococcal infection]. / Néphrite interstitielle aiguë et infection streptococcique.

Two patients with acute renal failure and streptococcal erysipela are reported. In both cases, renal biopsy showed acute interstitial nephritis with a predominantly mononuclear infiltrate. Beta lactam antibiotic without steroid therapy was followed by complete recovery of renal function. It seems likely that some cases of AIN related to infection are erroneously attributed to antibiotic hypersensitivity.

Quantification of beta 2-microglobulin and albumin in plasma and peritoneal dialysis fluid by a noncompetitive immunoenzymometric assay.

An enzyme-linked immunosorbent assay (ELISA) was developed for measuring beta 2-microglobulin (beta 2m) and albumin in continuous ambulatory peritoneal dialysis (CAPD) fluid. Plasma concentrations of beta 2m were twofold greater in hemodialysis patients (41.3 +/- 13.3 mg/L) than in CAPD patients (23.6 +/- 5.5 mg/L) matched for duration of treatment. Measurement of beta 2m in CAPD fluid showed a substantial loss of this protein, approximately 31% of total body beta 2m, compared with a 5% loss of a protein of middle molecular mass (albumin). Because of the molecular sieving effects of the peritoneal membrane, peritoneal clearance of beta 2m was sixfold greater than that of albumin. Whether beta 2m losses prevent or delay the incidence of dialysis-induced amyloidosis in these patients remains to be established.

Plasma level of lipoprotein Lp(a) is high in predialysis or hemodialysis, but not in CAPD.

Plasma Lp(a) lipoprotein level was determined in chronic renal failure (CRF) patients, 24 before initiation of dialysis, 18 undergoing hemodialysis, and 24 on continuous ambulatory peritoneal dialysis (CAPD). Eighteen healthy subjects were studied as controls. Median of Lp(a) level in both predialysis and dialysis patients was significantly increased: 23.5 mg/dl (range: 0 to 109) and 24.0 mg/dl (range: 1.4 to 90), respectively, as compared to healthy controls: 4.7 mg/dl (range: 1.8 to 27; P less than 0.001). By contrast, the median Lp(a) level in CAPD patients, 2.4 mg/dl (range: 0 to 39.5), was similar to the control group. Whether the CAPD procedure reduces the Lp(a) level in CRF patients has to be established in a prospective study.

Apo AIV in plasma and dialysate fluid of CAPD patients: comparison with other apolipoproteins.

Plasma lipids and apolipoproteins were determined in 18 patients with chronic renal failure on haemodialysis (HD), 18 patients on continuous ambulatory peritoneal dialysis (CAPD), and 18 healthy controls. Significant differences were observed between the two dialysis procedures, CAPD patients showed elevated plasma cholesterol, apo AI and apoB. No significant difference in plasma apo AIV concentration was observed between the two dialysis groups. Measurement of apolipoproteins in the dialysate fluid of five patients on CAPD showed losses of all apolipoproteins (AI, AII, AIV, B, CII, CIII and E). Although daily losses of apo AI and apo AIV were similar (255 and 221 mg/day respectively), the peritoneal clearance of apo AIV was threefold that of apo AI and 12-fold that of apo B. In view of the inverse correlation between peritoneal clearance and the molecular mass of proteins, about 13% of total plasma apo AIV was lost daily as lipoprotein-free form.