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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 199 records · Page 11Linked to original sources

Delayed renal failure with extensive mesangiolysis following bone marrow transplantation.

Within two years we have had the opportunity of observing seven leukemic children who were referred to our Pediatric Nephrology Unit for delayed renal failure following bone marrow transplantation (BMT). These children (3 to 12 years old), six with acute lymphoblastic leukemia (ALL) and one with acute non-lymphoblastic leukemia (ANLL), underwent BMT (4 autologous BMT, 3 allogeneic BMT) after the first remission in two, and after the second remission in five. Preparative regimen for BMT included cyclosphosphamide in three, cyclosphosphamide, vepeside and cytosine A in four, and a total body irradiation in a single dose of 10 grays (1000 R) in all of them. Three children were treated immediately after grafting with low dose cyclosporine for four to six months. Five to 10 months after BMT, four patients developed a hemolytic uremic syndrome with severe hypertension. The remaining three were found to have isolated renal insufficiency several months post-BMT. In the seven patients, renal biopsy showed a uniform pattern of severe glomerular involvement characterized by extensive lesions of mesangiolysis associated with severe arteriolonecrosis. A repeat biopsy performed one year later, in two patients showed severe scarring of the renal parenchyma with minor lesions of mesangiolysis. The similarity of the pathologic features observed suggests that the same mechanism might have been operative in the seven patients. It is very likely that the nephropathy is related to total body irradiation enhanced by chemotherapy. We conclude that current treatments of high risk leukemia might become a new cause of chronic renal failure. Further investigations are needed to know the exact incidence of this complication.

Bone Marrow Transplantation↗

EDTA Registry centre survey, 1986. Report from the European Dialysis and Transplant Association Registry.

This paper summarises the information given on the 1986 EDTA Registry centre questionnaire which was returned by 82% of the 2,065 known dialysis and transplant centres in 33 European countries. Information is given on the number of patients alive on haemodialysis according to the type of dialysis facilities available where the patient was receiving dialysis and the number of patients receiving special types of dialysis. The centre questionnaire also included questions on testing for HIV infection, serological evidence or symptoms of AIDS and the diagnosis of hepatitis B in patients and staff. The data given in response to these questions are presented together with data on the involvement of dietitians and social workers in the treatment of patients with end stage renal failure. Finally, information on transplant activity in Europe and the treatment policies of transplanting centres is provided.

AIDS Serodiagnosis↗

Cystic renal diseases in children.

Renal cysts are present in a wide variety of renal diseases, including those having a dysplastic nature and those of genetic origin. Genetic counseling requires clear differentiation between these types of cysts. This concept has made the classification of Potter useless because of the confusion it introduces between inherited diseases and developmental abnormalities. According to this classification, type I corresponds to autosomal recessive polycystic kidney disease (RPKD), type II to multicystic dysplasia, type III to autosomal dominant polycystic kidney disease (DPKD), and type IV to cystic dysplasia associated with urethral obstruction. The term "polycystic kidney disease" should be reserved for two hereditary cystic diseases, RPKD and DPKD, which are clearly distinct both by their modes of inheritance and by their pathologic characteristics. Both forms of polycystic disease may occur in children, but because the recessive form is much more prevalent, it is commonly called the "infantile form," whereas dominant polycystic disease is commonly known as the "adult form." The pediatric expression of these two forms and the problems of differential diagnosis will be examined.

Child↗

Renal allografts in cystinosis and mesangial demography.

Twenty-four biopsies of generally cadaveric renal allografts from 20 patients with cystinosis were examined by light, polarization, phase contrast and electron microscopy. Cystine crystals, or cytoplasmic crystalline spaces compatible with cystine, were observed in interstitial cells in 23 of the 24 biopsies and in glomeruli in six. Among the six, crystalline spaces were identified by electron microscopy in cells compatible with macrophages in the mesangium in two, and, in one of the latter, dark, presumably cystine-containing cells were also present in the mesangium. On the premise that cystine-containing cells derive from the host, these findings support the thesis that in man cells of the mononuclear phagocyte system of extrarenal origin may exist in the mesangium. However, in comparison with infiltration of the interstitium, infiltration of the glomerulus by macrophages from extrarenal sources is scant, as studied under conditions of renal transplantation.

Adolescent↗

[Study of blood cyclosporine after kidney or bone marrow transplantation. Comparison of immunofluorescence and radioimmunoassay].

The apparition of cyclosporine, immunodepressive drug, has largely improved the organ transplantations. However, the range of blood concentrations must be defined to allow the efficacity of cyclosporine therapy and to avoid toxic reactions, because there are very important variations for a same dosage according to the individuals and the diseases. Relative to the low concentrations to be determined (about one hundred ng/ml), the most useful methods for cyclosporine measurement are based on immunochemical assays. This work compare the two methods: radioimmunoassay (RIA) and fluorescence polarization immunoassay (FPIA) simultaneously performed on several hundred samples. A very significant correlation exists between the two techniques (r = 0,80). The advantages of immunofluorescent assay consists in rapidity, sensibility and facility to realize emergency analysis.

Bone Marrow Transplantation↗

[A case of diffuse inflammatory colonic pseudopolyposis caused by Crohn's disease].

Inflammatory pseudopolyposis of the colon is an uncommon anatomoclinical entity. It is generally associated with ulcerative colitis, Crohn's disease or schistosomiasis. We report a case of a pseudopolyposis in Crohn's disease involving the entire colon. This case was characterized by three particular facts: a) the pseudopolyps were composed of granulation tissues only, b) severe exudative enteropathy was the most important symptom, c) the endoscopic ablation of the polyps, performed for the first time in Crohn's disease pseudopolyposis, resulted in clinical and biological improvement.

Adult↗

[Computer-assisted diet therapy in pediatric kidney diseases].

The authors present a computer program written in UCSD Pascal) which monitors the dietary management of children with kidney diseases. Diet is established according to height, weight, chronological and statural age and recommended dietary allowances (USRDA). The composition of the prescribed diet and of food intake is given as amounts of animal and vegetable protein, fat, carbohydrates, energy, water, Na, K, Ca, P and renal solute load, per day and per kilo BW as compared to RDA. The amount of food is presented in tabular form, per day, per meal, per feed, or per tube-feeding with the schedule. It is possible to calculate the nutrients of food recipes. The food table includes 500 items, that can be modified as required. The drug table contains 100 items. The program calculates also average food consumption for dietary surveys. Diets, recipes and food tables may be viewed and modified before print out at each step of the calculation. The diet data bank stores 100 diets per floppy disk.

Child↗

Immunopathological findings in idiopathic nephrosis: clinical significance of glomerular "immune deposits".

Idiopathic nephrosis (IN), which includes minimal change (MCD), diffuse mesangial proliferation (DMP) and focal segmental glomerular sclerosis (FSGS), is classically characterized by the absence of significant deposits by immunofluorescence microscopy (IF), except for the focal lesions of segmental sclerosis and/or hyalinosis of FSGS, which fix IgM and C3 antiserums. Since IF is available in most centres, an increasing number of unexpected findings has been reported. In order to evaluate the clinical significance of the glomerular deposits revealed by IF in some instances, we reviewed the renal biopsy findings of 222 consecutive children presenting with IN and in whom IF microscopy was available. By light microscopy, 122 patients showed MCD, 10 DMP, and 90 FSGS with DMP (11 cases) or without (79 cases). By IF, 125 specimens were negative and served as controls; 54 showed mesangial IgM deposits, 24 mesangial IgG deposits (associated with Clq deposits in 16), 15 scattered granules of C3 and 4 predominant deposits of mesangial IgA. We correlated these findings with initial response to steroid therapy and outcome and could find no significant difference between the various categories defined by IF and the control group. Repeat biopsies, performed in 21 cases, showed the persistence of deposits in 11 and their transformation in 10. The particular problem raised by the patients who present with IN and mesangial IgA deposits is discussed. Our results demonstrate that patients presenting with IN and "positive IF", whether showing IgM, IgG and Clq, C3 or IgA, do not represent distinct clinicopathological entities.

Child↗

Acute pancreatitis in six non-transplanted uraemic children. A co-operative study from the French Society of Paediatric Nephrology.

Ten clinical episodes of acute pancreatitis (AP) occurred in six patients (mean age 10 years, range 3-15 years) with chronic renal failure (CRF) during a 9-year period (1977-1986). The underlying cause of CRF was vesicoureteral reflux (2); urethral valves (1); ureterohydronephrosis (1); nephronopthisis (1) and a haemolytic uraemic syndrome which occurred 12 years before (1). In all patients a diagnosis of AP was established both on clinical grounds and with a serum amylase level of greater than 600 IU/l. In 3 patients laparotomy was performed because of suspected appendicitis. All patients required exclusive parenteral feeding (mean duration 25 days) and 2 patients had a partial pancreatectomy. No patient developed pancreatic pseudocysts, 2 patients experienced one relapse (3 and 21 months later) and 1 patient had two relapses and died. Mean duration of follow up was 3 years (range 1-10 years). Possible aetiological factors were: choledochal cyst (1); parotitis without a rise in mumps antibodies (1); familial dyslipidaemia but without AP in other family members (1), and aluminium intoxication with hypercalcaemia and convulsive encephalopathy treated with valproic acid in 1 patient. Severe hyperparathyroidism with radiological signs was absent in all patients. Transplantation had been performed either before AP in 2 patients (1 and 3 years before AP) or had followed AP in 1 patient (7 years after) without occurrence or relapse of AP.

Acute Disease↗