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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 181 records · Page 10Linked to original sources
PubMed1990

The use of continuous peritoneal dialysis in Europe for the treatment of children with end-stage renal failure: data from the EDTA Registry.

The demographic data on the use of continuous peritoneal dialysis in Europe for children starting renal replacement therapy under the age of 15 years was obtained from data collected by the Registry of the European Dialysis and Transplant Association--European Renal Association (EDTA Registry) on individual patient questionnaires 1980-1986. Continuous ambulatory peritoneal dialysis (CAPD) and its variants appeared to be increasingly utilised as treatment for children with end-stage renal failure (ESRF) and accounted for approximately 25% of all renal replacement therapy (RRT). Important differences in its use in various European countries are demonstrated. The proportional contribution of CAPD to treatment was higher during the first year of RRT and gradually decreased thereafter. No significant sex differences existed in the use of this treatment. Approximately 70% of all patients on CAPD were older than 6 years of age, but it is in those under 6 years that the highest proportion are put on CAPD as first method of treatment for end-stage renal failure. The most common cause of abandonment of this treatment was peritonitis, which contributed 50% of the drop-out rate.

Adolescent↗
PubMed1990

Kidney transplantation in primary oxalosis: data from the EDTA Registry.

This paper reports the results of 98 first kidney transplantations in patients with oxalosis as the primary renal disease as recorded by the EDTA Registry. There were 79 patients who received a cadaveric (CAD) graft and 15 patients with a living related donor (LRD) graft; the type of donor was not recorded for four patients. Initial graft survival appeared to be better after LRD as compared to CAD grafts but at 3 years the poor survival was similar with 23% for LRD and 17% for CAD grafts. CAD graft survival did not differ between children and adults and was not affected by the waiting time on dialysis. A slight improvement was observed in grafts performed in the years 1983-1986 as compared to grafts performed in earlier years. The causes of failure reported were mainly rejection (33%) and recurrence of primary renal disease (31%). In view of the poor results related to recurrence of oxalosis in the graft, the potential of combined kidney and liver transplantation is discussed.

Adolescent↗
PubMed1990

Recombinant human growth hormone treatment in short children with chronic renal disease, before transplantation or with functioning renal transplants: an interim report on five European studies.

Growth retardation is common in children with chronic renal disease. Final adult height is often reduced, even in children with a functioning renal transplant. The five European studies considered here aim to investigate the efficacy and safety of recombinant human growth hormone therapy (rhGH) in two groups of short children with chronic renal disease. The first group comprises 29 prepubertal children with preterminal chronic renal failure (i.e. before renal transplantation), and the second group comprises 39 prepubertal and pubertal children with functioning renal transplants. The median bone age retardation in the groups at the start of treatment was between 2.2 and 3.7 years; this did not change during the first year of treatment. This interim report concentrates on patients who have been treated for at least 1 year (i.e. 22 children from the first group, and 28 children from the transplant group (15 prepubertal and 13 pubertal children). The median height velocity increased from 4.8 cm/year to 10.0 cm/year in the first group (the chronic renal failure group), from 2.6 cm/year to 6.2 cm/year in prepubertal children with renal transplants and from 3.8 cm/year to 6.7 cm/year in pubertal children with renal transplants. The corresponding changes in height velocity SDS were from -1.3 to 5.1 for the chronic renal failure group and -2.8 to 2.3 for the prepubertal children with renal transplants. Renal function declined in the chronic renal failure group but this decline corresponded to expected progression of the disease. Some of the children with renal transplants showed a decreased renal function, which in most cases was explained by non-compliance or chronic rejection.

Adolescent↗
PubMed1990

[A comparative trial of OKT3 and antilymphocyte serum in the preventive treatment of rejection after kidney transplantation in children].

The monoclonal antibody OKT3, administered for 2 to 3 weeks in a dosage of 0.1 mg/kg, in combination with azathioprine and prednisone, proved more effective than conventional antilymphocyte serum for the prevention of renal transplant rejection. Side effects were less marked with OKT3 than with antilymphocyte serum. Infectious complications, especially viral, occurred with the same incidence in both groups. In the long term, following discontinuation of OKT3 or antilymphocyte serum, incidence of rejection episodes and graft survival rates were identical in both groups. OKT3 in a lower dosage of 0.05 mg/kg was less effective, with several patients developing rejection during treatment, and induced more common and earlier anti-OKT3 immunization.

Antibodies, Monoclonal↗
PubMed1990

[Nail-patella syndrome without extra-renal lesions. A new hereditary glomerular nephropathy].

We observed an unusual glomerular disease in eight pediatric patients. Clinical features of this early, progressive renal disease included increased blood pressure in many cases, extrarenal hematologic and pulmonary symptoms, and, in one of our patients and two genetically related children, hemolytic uremic syndrome with thrombotic microangiopathy resulting in permanent renal failure. Histologic studies showed major and complex modifications of glomerular capillary walls and electron microscopy disclosed numerous bundles of fibrillar collagen within the extracellular glomerular matrix. Clinical and genetic investigations out-ruled hereditary osteo-onychodysplasia, a condition in which fibrillar collagen is found within glomerular basement membranes. In addition results of family studies were consistent with autosomal recessive transmission of this new entity.

Adolescent↗
PubMed1990

[Early failures in kidney transplantation. A retrospective study of 53 cases].

Among the 652 consecutive renal transplants performed from January 1973 through December 1988, all graft failures within 60 days of transplantation were retrospectively analyzed. The 53 early failures were divided into four groups on the basis of pathologic findings in the removed transplant). 1) Irreversible vascular rejection (17 cases); in ten patients, this rejection occurred between the fourth and eight days and was manifested by uniform clinical features. 2) Thrombosis of the renal artery (18 cases), with several subgroups: a) early postoperative thrombosis (5 cases) with primary anuria; b) thrombosis following reoperation (6 cases) for urine leakage or occlusion; c) thrombosis with a clotting disorder,; d) and five cases of thrombosis with clinical manifestations of vascular rejection but with isolated thrombosis upon histologic examination. 3) Thrombosis of the renal vein (12 cases), with primary anuria in nine cases, and rupture of the kidney in three. 4) The last group includes six cases due to a variety of causes: one immediately non-viable -kidney, two severe, irreversible cases of tubulonephritis, one cortical necrosis secondary to circulatory collapse in the recipient, one complete necrosis of the ureter requiring removal of the transplant, and one immediate recurrence of hemolytic uremic syndrome. Among the risk factors for early failure, attention is drawn to the young age of the donor or recipient that increases the risk of thrombosis. Furthermore, the risk of vascular rejection was decreased in patients preventively treated by OKT3.

Anuria↗
PubMed1990

[Treatment of cystinosis using cysteamine].

Eighteen pediatric patients with cystinosis were treated with cysteamine. Treatment was started at a variable age, between 10 months and 7 years, and was continued for 6 months to eight years. Results were evaluated on renal function changes and growth. Whereas 65% of patients with cystinosis develop terminal renal failure before the age of ten years, three (16%) of our 18 patients given cysteamine started dialysis before the age of ten and all three had first received cysteamine only after 4 1/2 years of age. The plasma creatinine level was also lower in treated patients as compared with a control multicenter group. Growth was also significantly improved by treatment, especially in those children treated before the age of 26 months who were taller by 2 SD at age five and 2.5 SD at age eight than untreated controls. Some children who complied strictly with the treatment regimen had an normal stature (- 1 SD) at ten years of age. In conclusion, the effectiveness of cysteamine seems obvious in this group of patients if rigorous compliance with the drug dosing schedule is achieved.

Child↗
PubMed1990

Perceptions of and preferences for sweet taste in uremic children.

Uremic children have low energy intakes, with little appetite for sweet foods. Likewise, sucrose-rich diets are poorly accepted by uremic rats, which suggests that uremia causes a relative aversion for sucrose. Hemodialyzed children (no. = 39, mean age = 160 mo) and healthy controls (no. = 25, mean age = 122 mo) were compared for perception of sweet taste intensity in two familiar foods (soft white cheese and apple sauce) and for preference for sweetness. The food stimuli were prepared in five sucrose concentrations: 1%, 5%, 10%, 15%, and 20% for cheese; 10%, 20%, 30%, 40%, and 60% for apple sauce. Children were presented with pairs of stimuli of adjacent concentrations and asked, in a forced choice, to identify the sweeter stimulus and to express their preferences. The hemodialyzed children made more mistakes (19%) than the controls (5%) when asked to rank sweetness in the soft cheese (i.e., with low concentrations). Both groups made an equal number of mistakes when asked to rank sweetness in the apple sauce. Preferences for sweetness were markedly different. In cheese, the highest sucrose concentration was preferred by 21.9% of the hemodialyzed children vs 41% of the controls. The lowest sucrose concentration was selected by 15.6% of the hemodialyzed children vs 4.6% of the controls. Similar preference trends were observed for apple sauce. We conclude that abnormally low preferences for sweet foods can contribute to insufficient caloric intake in uremic children.

Adolescent↗
PubMed1989

Organ transplantation in children.

Organ transplantations have opened a new field in medicine and particularly in pediatrics. The kidney was the first organ to be regularly transplanted and there are now more than 2000 children who have received a kidney graft. Cadaver kidneys or living-related donor (LRD) kidneys can be used since an adult kidney may be grafted in a young child. Cadaver graft survival exceeds 85% at 1 year in recent single centre reports and patient survival is above 95%, the results being even better with LRD. Some complications may be observed in the long term, such as bone osteonecrosis, hypertension or infections. Rehabilitation is generally remarkable and growth which remained abnormal in 1/3 of cases under conventional treatment seems to improve markedly with cyclosporin. In the future, the development of kidney transplantation could lead hopefully to a drastic decrease in the number of children on dialysis. Liver transplantation is now performed in children with biliary atresia and metabolic diseases. There is no age limit for grafting a liver, the only problem being to find a pediatric cadaver donor of similar size. It is also possible to use an adult liver after hepatectomy. There were 170 liver grafts recorded in children in Europe in December 1986, and the long-term survival thanks to cyclosporin is exceeding 80% in some units. Thanks to cyclosporin, programs of cardiac transplantation for children are rapidly developing. The main indications are gross-congenital cardiac malformations or severe functional abnormality not compatible with life. Newborns have been grafted with success. The only problem is also to find a suitable pediatric donor.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1989

Infantile chronic tubulo-interstitial nephritis with cortical microcysts: variant of nephronophthisis or new disease entity?

Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11-22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts.

Child, Preschool↗
PubMed1989

Dark cells of cystinosis: occurrence in renal allografts.

Twenty-four biopsies of renal allografts, generally cadaveric, from 20 patients with cystinosis were examined by light, polarization, phase contrast, and electron microscopy. The unusual dark cells previously reported in the native kidneys and livers of patients with cystinosis were observed in 12 of the 24 biopsies. The cells were present in the interstitium in all of these 12 biopsies, in glomeruli in one biopsy, and in the tubular lumen in two biopsies. They were evident by light and electron microscopy in stained and unstained ultrathin sections, and could be discerned solely in Epon sections. The dark appearance resulted from the presence of dark, fine granular material in the cytoplasm and nucleus and in cytoplasmic inclusions. The cells were judged to be macrophages. They were present as early as 3 months following transplantation and bore no relationship to interstitial crystals or inflammation. The dark cells have two important implications: cystine storage may not be limited to lysosomes, and dark cells are a morphologic alternative to the traditional identifying configuration of cystine in tissues, namely crystals.

Adolescent↗
PubMed1989

Contribution of toxic nephropathies to end-stage renal failure in Europe: a report from the EDTA-ERA registry.

In countries which reported to the registry of the European Dialysis and Transplant Association (EDTA)-European Renal Association, 2.4% of 147 092 treated patients were recognized as having analgesic nephropathy (AN) as the cause of end-stage renal failure (ESRF) on 31 December 1986. A small number of patients had other specific drug nephropathies, but these do not yet make an important contribution to ESRF treatment programmes. It is possible that more patients have ESRF due to AN, and evidence from studies of age distribution, the demography of urothelial malignancies, a special study of diagnostic criteria, data on sales of analgesics, and a study of regional areas of high incidence lend some support to that view. Changes in prescription and self-medication practices over the last 20 years have almost eradicated analgesic nephropathy from the U.K. and Sweden, two countries in which there was previously a high frequency of ESRF due to AN. In other countries where action has been taken more recently, cases are still reported fairly frequently, and this seems likely to continue for several years to come.

Adolescent↗