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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 217 records · Page 12Linked to original sources
PubMed1988

De novo membranous glomerulonephritis in renal allografts in children.

The incidence of de novo membranous glomerulonephritis (MGN) in transplanted kidneys is around 1 to 2%. In our series, of the 310 grafts that were examined by immunofluorescence microscopy (IF), 29 (9.3%) showed subepithelial IgG deposits, a pattern consistent with the diagnosis of MGN. Transplant biopsy had been performed because of the occurrence of proteinuria in 8 patients (pts), for suspected rejection in 16 and systematically in the remaining 9 who had no proteinuria and a normal renal function. In all cases the lesions were identified by IF and were confirmed by electron microscopy (EM) in 14 pts. Granular deposits of IgG were diffuse in 22 cases and segmental in 7. Sequential specimens were available in 17 pts and showed the persistence or the increase of the IgG deposits in all patients but one in whom they had disappeared. Age at transplantation (Tx) ranged from 2 years 3 months to 16 years. Grafts were from cadaveric donors in 27 pts and from living related donors in 2 pts. In none of the recipients was MGN the nephropathy of the native kidneys. With a follow-up of 18 to 74 months, 6 pts never developed a proteinuria. In the remaining 23, proteinuria developed 1 to 70 months after Tx, associated with a nephrotic syndrome (NS) in 6 pts.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

[Renal transplantation in the child].

The informations available for Europe show that, on December 31, 1986, 50% of the 1,616 registered children under 15 years of age had a functional renal graft. Only 5% of these children had been transplanted without prior dialysis, which is usually possible only with a graft taken from a living donor. With brain-dead donors, generally adults, numerous patients may remain on the waiting list after several years of dialysis. The preparation of recipients, the juridical aspects of such transplantations in children, the principles of selection of recipients for corpse kidney grafts are reported. The technical aspects of graft surgery and the per- and postoperative supervision are then reported. The results of renal transplantation have improved since the advent of cyclosporine: patients' survival remains almost 100% on the whole of the renal transplantations performed in children since 1985. The results obtained with a second transplantation after failure of the first are comparable and sometimes better in some series. Finally, the quality of everyday life of the grafted child, its rehabilitation after transplantation, its social and professional insertion are emphasized. Furthermore, the possibilities of statural growth under cyclosporine therapy are better than those when one could use only traditional immunosuppressors.

Cadaver↗
PubMed1988

Survival on renal replacement therapy: data from the EDTA Registry.

Extensive survival data are presented from the EDTA Registry's files for patients who started renal replacement therapy in 1970-1974 compared to 1980-1984. The contribution of the different treatment modalities (haemodialysis, continuous peritoneal dialysis, and transplantation) to the survival of patients according to geographical region is also shown. Survival on renal replacement therapy, irrespective of treatment modality and of primary renal disease, was best in the 10-14-year-old patients, with 58% at 10 years and 52% at 15 years, and decreased with rising age to 28% at 10 years and 16% at 15 years in patients aged 45-54 when they commenced therapy in 1970-1974. When comparing the 0-4-year-old with the 10-14-year-old cohort of the paediatric patients, 5-year survival rates for patients starting renal replacement therapy in the early eighties declined from 85% to 70% with decreasing age. Treatment policy, as reflected by the proportion of patients on different modes of therapy, varied markedly between European regions but affected survival to a small extent only. The large population with diabetic nephropathy incurred annual mortality rates 2-3 times greater than those observed in patients with 'standard' primary renal diseases. Haemodialysis and continuous peritoneal dialysis, although not comparable because of important differences in selection policy, yielded similar survival rates. Patients and graft survival rates have improved markedly when comparing patients starting renal replacement therapy in the early seventies with the eighties; particularly for cadaveric transplantation. Patient survival after second grafting was similar to that after first grafting, with 83% at 5 years after second cadaveric grafting in the 15-44-year-old cohort, vs 85% after first cadaver transplantation in 1980-1984. Second cadaveric graft survival was superior to average first-graft survival for those recipients whose first graft had been functioning for more than 1 year. However, second-graft survival in rapid rejectors of a first graft as well as third cadaveric graft survival were curtailed by the large number of early losses, with only 52% of third grafts functioning at 1 year. For living related donor transplantation, parents were mostly used in children whilst identical siblings predominated in adults older than 45. In the early eighties, patient survival was 92% at 5 years for recipients younger than 15, 87% for the 15-45 year old cohort and 72% for those aged 45 or older.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1987

Alport's syndrome as a cause of renal failure in Europe.

We studied the geographical distribution, male to female ratio, and age at the start of renal replacement therapy (RRT) for end-stage renal failure (ESRF) in 600 patients with hereditary nephritis with nerve deafness (Alport's syndrome) reported to the European Dialysis and Transplant Association Registry since 1975. Annual age- and sex-specific acceptance rates for RRT showed a variable peak incidence according to country, ranging between, 0 and 2.4 patients per million population in males aged 15-24 years, but with only about half this incidence in females. In Scandinavian countries there were very few females who started RRT, and males were older than in the rest of Europe. The overall male to female ratio was 4:1. The median age at the start of RRT was: males (n = 479) 24.3 years (1st quartile 19.5 years; 3rd quartile 31.5 years); females (n = 121) 31.5 years (1st quartile 23.0 years; 3rd quartile 43.2 years). Our study provided confirmation that males reach ESRF earlier than females. In addition, we detected previously unrecognized geographical differences.

Adolescent↗
PubMed1987

Late symptoms in infantile cystinosis.

Late symptoms of infantile cystinosis were evaluated in 19 patients aged 15-26 years who had a high graft survival following kidney transplantation. The end-stage cystinotic kidney was responsible for renal hypertension in 5 patients following grafts. Photophobia did not increase in relation to age, but 3 patients became blind and 1 lost the sight in one eye at 25 years of age. Two patients developed clinical symptoms of hypothyroidism, and 15 other patients had a compensated hypothyroidism. Four patients developed permanent insulin-dependent diabetes and 2 developed transient insulin-dependent diabetes after transplantation. The oral glucose tolerance test was abnormal in 11 of 14 patients on low-dose prednisone. Liver enlargement was noted in 10 cases, but only 3 patients developed clinical symptoms of portal hypertension. Symptoms of hypersplenism were observed in 6 cases leading to splenectomy. Repeat gross epistaxis was observed in 7 of the patients when on dialysis and persisted after transplantation in 1 patient, who died from nasal bleeding. A particular encephalopathy developed in 2 patients at the ages of 17 and 24, characterized by speech difficulties, pyramidal symptoms and cranial nerve deficit; one died at the age of 21. The mean adult height of these patients was 136.5 cm in males and 124 cm in females, and their psychosocial adjustment was related to the extra-renal complications of cystinosis rather than to the renal status.

Adolescent↗
PubMed1987

Ocular changes in some progressive hereditary nephropathies.

Ocular involvement is a common feature of three hereditary nephropathies: cystinosis, nephronophthisis and Alport's syndrome. The follow-up of 25 cases of infantile cystinosis over a period of 26 years demonstrated that the corneal and retinal epithelium were affected in the same way as the kidney epithelium. Corneal involvement induced photophobia and discomfort, but actual blindness was mainly due to retinal involvement, and therefore no corneal graft was performed. The use of topical cysteamine appears to be promising, but its production raises many questions so no definitive conclusions may be made. Since 1965, 51 patients with nephronophthisis have had ocular examinations; all patients had characteristic clinical symptoms and histological findings. The first group, which consisted of 18 children, all under 10 years of age, was found to have obvious chorioretinal degeneration. The second group, which consisted of 11 children, had a normal ocular examination and normal electroretinogram (ERG). The third group, consisting of 22 children, had a normal clinical examination but ERG tracings with variable alterations. In 28 instances of Alport's syndrome, two types of ocular abnormalities have been observed. In six cases, an anterior lenticonus was noted, which caused a major decline in visual acuity. Retinopathy, which did not affect vision, was observed in 13 cases.

Adolescent↗
PubMed1987

Cyclosporin in the treatment of idiopathic nephrotic syndrome in children.

Thirty-five children (12 girls, 23 boys), aged from 1 year and 5 months to 14 years at the onset of idiopathic nephrotic syndrome, received cyclosporin A (CyA) because of steroid toxicity or failure to respond to steroids. The initial oral dose was 6 mg/kg per day and this was adjusted to obtain trough plasma levels of 50-150 ng/ml. The duration of treatment was between 2 and 8 months. In patients who responded to CyA treatment, the dosage was tapered off; treatment was stopped if found to be ineffective. Of the 35 children, 20 were frequent-relapsing steroid responders who suffered serious side-effects from steroid therapy. Seventeen of them either went into remission or did not relapse despite the withdrawal of prednisone. Prednisone doses could be lowered but not stopped in 1 patient and the remaining 2 patients relapsed when prednisone was tapered off. At the final examination, 10 of the 12 children in whom CyA was tapered off and who had initially responded to CyA had relapsed. A second course was given to these 10 patients and 3 failed to respond. Five children were partial steroid responders and CyA induced a remission in 1 and a partial remission in another. Among the 10 children who were steroid resistant, only 1 responded to CyA, 2 had a partial response and 7 failed to respond to CyA. A reduction of glomerular filtration rate occurred in 8 patients, 7 of whom had either persistent nephrotic syndrome or were in relapse, which suggests that factors other than CyA nephrotoxicity may have been operative. Complete reversal occurred in only 4 patients. Significant histological changes, likely to be related to CyA, were seen in 2 repeat renal biopsies out of the 11 performed.

Child↗
PubMed1987

Hypertension following renal transplantation in children.

The files of 334 consecutive cadaver kidney (CK) and of 27 living related (LR) transplantations (T) in children and adolescents performed from 1973 to 1984 have been reviewed. Following cadaver transplantation, 52 patients (15%) never had hypertension (HT), 41 patients (12%) had only initial HT up to 6 months after transplantation and 18 other patients (5%) exhibited transient HT episodes while on high-dose steroid therapy. Finally, 209 patients (62%) had HT for periods longer than 6 months and 16 patients (5%) until death or graft failure within the first 3 months. Chronic graft rejection was the major cause of HT, but other factors either isolated or in association were also present. Renal artery stenosis (RAS) was diagnosed in 43 cases (13%) 2-17 months post-transplantation; 10 of these were operated upon (5 successfully) and 9 underwent transluminal angioplasty with a single success. Nine cases of RAS resolved spontaneously. HT was attributed to the host kidney in 10 cases (3%) and to recurrence of primary renal disease in 9 (3%). HT observed after CKT was sometimes severe and difficult to control. Acute complications from HT were recorded in 35 cases, with 6 deaths and 2 severe neurological sequelae. Among 25 LRT, 11 cases (40%) had no HT 13 (48%) had HT for longer than 6 months. In this group, no case of RAS was observed and only one complication (without sequelae) was noted. In conclusion, HT is a frequent and sometimes severe complication post-transplantation in children and adolescents.

Child, Preschool↗
PubMed1987

Glomerular lesions in the transplanted kidney in children.

The glomerular pathology of 634 transplant specimens (526 biopsies and 108 transplantectomies) from 410 children was studied. Three types of glomerulopathies were observed: (1) recurrent glomerulonephritis (GN) (40 of 142 patients with glomerular nephropathy), (2) de novo GN (52 grafts), and (3) transplant glomerulopathy (29 grafts). The study of recurrent GN is considered of great interest because of the possible insight into the nature of the original disease and the opportunity to observe the evolution of the disease in sequential biopsies of the transplant. The two major forms of de novo GN were membranous GN and IgG linear deposits along glomerular and tubular basement membranes. Transplant glomerulopathy, although distinctive morphologically, may resemble membranoproliferative GN (MPGN) or thrombotic microangiopathy.

Biopsy↗