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M Jadoul

Publications and source records attributed to M Jadoul.

89 records · Page 5Linked to original sources

Beta 2-microglobulin amyloidosis in a patient treated exclusively by continuous ambulatory peritoneal dialysis.

The histological demonstration of beta 2-microglobulin (B2M) amyloidosis in a 75-year-old man treated exclusively by continuous ambulatory peritoneal dialysis (CAPD) for 87 months is reported. Amyloid deposits were detected in all joints from which synovial specimens were obtained at autopsy. It is of note that the patient had never undergone hemodialysis and had suffered only one episode of peritonitis. Both features are of interest, as the few reports of dialysis-related amyloidosis (DRA) in CAPD include patients who have been hemodialyzed and have suffered repeated episodes of peritonitis, both conditions being incriminated in the genesis of B2M amyloidosis. We conclude that DRA may develop in the absence of artificial membranes or frequent peritonitis episodes.

Aged↗

Insulinomas in MEN-I patients: early detection and treatment of insulinomas in patients with the multiple endocrine neoplasia syndrome type-I.

In the multiple endocrine neoplasia syndrome type I (MEN-I syndrome), periodic screening of patients and their close relatives may improve prognosis and life expectancy. Although there is diffuse involvement of the pancreas with microadenomatosis, insulinomas in the MEN-I syndrome usually occur as single tumours. This is illustrated here by two patients with insulinomas and the MEN-I syndrome. Preoperative localization of the tumours was achieved accurately by digital subtraction angiography combined with dynamic computerized tomography after a bolus injection of contrast medium. At present, two and three years after elective surgery both patients are asymptomatic. The early detection and treatment of insulinomas is extremely important because of the high risk of cerebral damage associated with late diagnosis. Periodic investigation of MEN-I family members can promote the early diagnosis and treatment of insulinomas, especially in young patients, whose life expectancy and quality of life may be improved.

Adolescent↗

Pheochromocytoma-induced hypertensive encephalopathy revealing MEN-IIa syndrome in a 13-year old boy. Implications for screening procedures and surgery.

In the multiple endocrine neoplasia (MEN) type II syndrome, pheochromocytomas become manifest at a later age than medullary thyroid carcinomas (MTC) do. The present report concerns a 13-year-old boy, belonging to a MEN-IIA kindred, who was admitted because of convulsive seizures related to hypertensive encephalopathy. A pheochromocytoma was suspected immediately and appropriate medical therapy was initiated. A right adrenal pheochromocytoma was removed, as well as a pheochromocytoma of an accessory right adrenal gland. Today, three years later our patient is still asymptomatic and the results of the thyroid C-cell provocative tests remain normal. This case clearly justifies the conclusion that periodic investigation of MEN-II family members to detect both medullary thyroid carcinoma and pheochromocytoma should begin early in life, as the latter may be the initial life-threatening expression of the disease. Long-term follow-up of patients treated with unilateral adrenalectomy will permit better definition of the risk of contralateral recurrence in such cases.

Adolescent↗

Effect of renal transplantation on the radiological signs of dialysis amyloid osteoarthropathy.

We have assessed the fate of amyloid bone cysts after a successful renal transplantation in 5 patients who had been on dialysis for 7 to 15 years. The size of 34 cysts, typical of amyloid osteoarthropathy, was monitored on annual X-rays for 29 to 75 (mean 47) months after TP. In sharp contrast to their progression during dialysis, cysts did not increase in size and no new cysts appeared during follow-up. However, despite a successful graft, none of the 34 cysts regressed significantly. The stability of the bone cysts may reflect either the poor solubility of the beta 2-m amyloid deposits or the limited accessibility of bone deposits to lytic factors. A longer follow-up will perhaps be necessary to document a significant regression of the cysts. Strikingly, transplantation resulted within a few days in an almost complete relief of articular complaints; in the absence of evidence of amyloid regression, this probably reflects the anti-inflammatory effect of steroids.

Adult↗

Pathological aspects of beta(2)-microglobulin amyloidosis.

Histology remains the gold standard to diagnose beta(2)-microglobulin amyloidosis (A beta(2)M). Two diagnostic criteria are required: positive Congo red staining with typical birefringence under polarized light and immunostaining of amyloid deposits with a labeled anti-beta(2)M antibody. A beta(2)M is preferentially located in the joints. Small deposits are also found in various organs, mainly the heart and gastrointestinal tract. Pathologic studies have demonstrated a high prevalence of articular A beta(2)M early in the course of hemodialysis and peritoneal dialysis, antedating clinical manifestations by several years. The stages of beta(2)M amyloid formation have been delineated: beta(2)M amyloid deposits first on the surface of the cartilage, in the absence of macrophages (stage 1), and subsequently involves capsules and synovia (stage 2), with eventual recruitment of macrophages around large beta(2)M amyloid deposits (stage 3). Clinical manifestations are likely associated with the inflammation observed in stage 3. The factors triggering the fibrillar precipitation of beta(2)M remain unknown. Macrophages do not play a role: their presence is the consequence rather than the cause of beta(2)M amyloid deposits. Several substances coprecipitated with beta(2)M amyloid have been incriminated: highly sulfated glycosaminoglycans such as chondroitin or keratan sulfate, antiproteases such as alpha(2)-macroglobulin, and apolipoprotein E. As yet, no definitive conclusion has been reached.

Amyloid↗

Should dialysis machines be disinfected between patients' shifts?

The disinfection of dialysis machines is performed after each session in some units but only at the end of the day in other ones. In a multicentre study, in 15 units (five with and ten without disinfection after each session), the incidence of sero-conversion for HCV was reduced to zero, after reinforcement of general hygiene precautions only, without changing the above-mentioned machine disinfection policies. Recently reported episodes of blood contamination of dialysis machines beyond pressure transducers demonstrate that pressure isolator inspection and care should be added to the general hygiene precautions. In a second investigation, no difference was found in dialysate bacterial and endotoxin characteristics between dialysis machines, disinfected after each dialysis (n = 2) and those disinfected at the end of the day only (n = 2). In conclusion, the systematic disinfection of all dialysis machines after each session does not appear to help prevent HCV infection nor to have a detectable impact on optimal dialysate bacterial and endotoxin characteristics.

Bicarbonates↗

Serum leptin concentration in peritoneal dialysis patients: determinants, longitudinal evolution and circadian rhythm.

The serum concentration of leptin, an hormone secreted by adipocytes, is increased in obese and chronic renal failure patients. To determine the influence of peritoneal dialysis (PD) therapy on serum leptin levels, we analyzed its concentration in 23 patients on PD for an average of 26.8 +/- 7.1 months and compared it to that of 18 patients with chronic renal failure (creatinine clearance: 49.1 +/- 8.8 ml/min) and of 35 healthy control subjects. Leptin level was also reevaluated in 11 PD patients 9.3 +/- 1 months after the initial analysis. Finally, circadian leptin production was determined in 4 patients on automated PD (APD). Serum leptin was significantly higher in PD (31.9 +/- 7.8 ng/ml) than in chronic renal failure patients (15.2 +/- 5.9 ng/ml) and in healthy control subjects (9.6 +/- 1.1 ng/ml). Serum leptin level was significantly correlated with BMI in all three groups (except in PD males) and with the percentage of fat mass in both male and female PD patients. It did not correlate in PD patients with serum albumin concentration, free fat mass, residual diuresis, time on PD and characteristics of peritoneal permeability. At the second determination, serum leptin level had significantly increased in the PD patients although their respective BMI and serum creatinine concentration had remained virtually unchanged. Finally, in APD patients, the highest leptin level was observed at 08.00 a.m. These results demonstrate that serum leptin level is increased in PD patients and that it progressively rises under PD therapy. The circadian leptin production is delayed in APD patients probably suggesting a negative effect of the nocturnal glucose load on the regulation of its secretion.

Adult↗

Blood flow displayed by dialysis machines: is it accurate?

Blood flow is a major determinant of dialysis efficiency. We reported in 1980, at the EDTNA conference in Prague (1), that the actual blood flow may be much lower than displayed by the dialysis machines. We demonstrated that the Negative Inflow Pressure (NIP), induced by the arterial needle and therefore generally referred to as arterial pressure, partly collapses the proximal blood pump segment whose inner volume is decreased. Thus the actually pumped volume per revolution is lower than the displayed blood flow.

Bias↗

Scintigraphy with Indium-labelled leukocytes in acute pancreatitis.

The authors assessed the clinical and prognostic value of abdominal (ASCI) and pulmonary (PSCI) scintiscans with 111Indium-labelled leukocytes in the early phase of acute pancreatitis. A grading scale was constructed for both ASCI (scored 0 to 3) and PSCI (scored 0 to 2) according to the intensity of isotope fixation versus adjacent structures. Results were compared in accordance to the presence or absence of a Ranson's score greater than or equal to 3, the presence or absence of respiratory failure and of late pancreatic complications. ASCI showed an important lack of specificity making its use uninteresting. PSCI revealed a very significant correlation with evaluation by Ranson's score and appears to have a high sensitivity and specificity to evaluate the patients who will develop ARDS or risk of ARDS. This demonstrates the pathophysiological role of leukocytes in the early phase of severe acute pancreatitis. The test might also be a reliable test for the assessment of therapeutic efficiency in acute pancreatitis.

Abdomen↗