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Biomedical subjects

A M Wingen

Publications and source records attributed to A M Wingen.

At least 19 recordsLinked to original sources
PubMed1992

Energy and nutrient intake of patients with mild-to-moderate chronic renal failure compared with healthy children: an Italian multicentre study.

Nutritional counselling is important in the management of children with chronic renal failure (CRF). In 1988, a controlled European multicentre study was started to evaluate the effects of a low-protein diet on the progression of CRF in children. To assess the energy, macro- and micronutrient intake, 4-day weighed dietary records were obtained from 50 children with low to moderate CRF (creatinine clearance 65 to 15 ml/min per 1.73 m2) and from 93 healthy children. The mean energy intake was 90%-93% of the recommended dietary allowance for Italian children in controls and 76%-88% in CRF patients. The mean protein intake was 2.1-3.1 g/kg per day in controls and 1.6-2.7 g/kg per day in CRF patients. Overall, the energy intake was 10% and the protein intake 33% lower in CRF patients than in healthy children. Children with CRF consumed less cholesterol, calcium and phosphorus than healthy children. The lower spontaneous intake of energy, protein and other nutrients should be taken into account when planning the nutrition of children with CRF.

Child↗
PubMed1992

Increased aggregation with normal surface charge and deformability of red blood cells in children with nephrotic syndrome.

Hemorheological risk factors for thromboembolic disease were evaluated in 25 pediatric patients with idiopathic nephrotic syndrome (NS). In patients with increased proteinuria (greater than 100 mg/m2/24 h) red blood cell (RBC) aggregation and plasma viscosity were significantly increased when compared with patients in remission (less than 100 mg/m2/24 h) and with healthy controls. RBC surface charge was normal during increased proteinuria and remission. RBC aggregation correlated positively with plasma viscosity, fibrinogen, alpha 2-macroglobulin, immunoglobulin M, and the degree of proteinuria, and negatively with plasma albumin levels. RBC aggregation showed no correlation to RBC surface charge. Hematocrit and RBC deformability (rheoscope) were similar in both patient groups and in controls. Increased RBC aggregation and plasma viscosity may contribute to the increased risk of venous thromboembolism in NS.

Blood Viscosity↗
PubMed1992

Multicentre randomized study on the effect of a low-protein diet on the progression of renal failure in childhood: one-year results. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

After an adaptation period of 6 months, 200 children (mean age 10.3 years; 138 boys and 62 girls) were stratified according to their renal diseases and the progression of renal failure during the adaptation period and randomized for low protein intake amounting to the WHO safe levels for age (0.8-1.1 g/kg/day) or free protein intake. Energy intake of both groups should be near the WHO recommendations. Compliance was controlled by written dietary diaries and urea-N excretion. 101 children had been randomized for the diet group and 99 for the control group. 165 of these patients finished their first year after randomization. Mean protein intake was 126% of the recommendations in the diet group vs. 187% in the control group while energy intake was similar in both groups (85 vs. 90%). SDS for height at start and 1 year later did not change (diet: -0.9 vs. -1.1; control: -0.9 vs. -0.9). Weight gain was not different. Only the stratification for two groups according to the progression of renal failure ('nonprogressive' and 'progressive' diseases) was used in the analyses. Progression of renal disease during the adaptation period proved to be a significant prognostic factor. Mean loss of GFR/year was similar in patients with 'nonprogressive' diseases in the control group (-1.1 ml) and the diet group (-1.3 ml). In patients with 'progressive' diseases mean loss of GFR was significantly higher in the diet group (-6.5 ml) than in the control group (-4.0 ml).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Low-protein diet in children with chronic renal failure--1-year results. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

In 1988 the European Study for Nutritional Treatment of Children with Chronic Renal Failure started its multicentre randomized trial to investigate the influence of protein intake on the progression of renal failure. A total of 284 children had been registered. Of these 221 were accepted for the study. The data from 105 patients after 1 year of study are available for preliminary analysis. Fifty children were randomized for the diet group and 55 for the control group. Both groups were comparable concerning age, glomuerlar filtration rate (GFR) and height standard deviation score for chronological age at the start of the study period and the distribution of primary renal diseases and sex. Limits for protein and energy intake were set according to the safe levels and recommendations given by the World Health Organization. The compliance with dietary prescriptions as calculated from dietary diaries was good. A low-protein diet did not do any harm to the children with respect to length gain and weight gain. The progression of renal failure was minimal in the diet group (mean loss of GFR 3.6 ml/min per 1.73 m2 per year) as well as in the control group (2.3 ml/min per 1.73 m2 per year). The differences between the diet group and the control group were statistically not significant when either all patients or only subgroups of various primary renal diseases were analysed. When only patients with a good compliance were considered (documented by dietary diaries or by urea nitrogen excretion) the same results were obtained. In summary, reduction of protein intake was accepted by the majority of patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

[Acute tubulointerstitial nephritis and uveitis (TINU syndrome) in childhood].

During the last two years we have observed three children, aged 12-15 years, who developed acute non-oliguric renal failure with concomitant uveitis. Acute interstitial nephritis with lympho-monocytic infiltrates was diagnosed in all cases by renal biopsy. While two patients went into spontaneous remission, renal function in the remaining child improved only after treatment with oral prednisone. Withdrawal of steroid medication was promptly followed by a relapse, necessitating steroid therapy for a total of 4 months. The clinical and histological findings were consonant with the so-called TINU syndrome. While the pathogenesis of this syndrome is unclear, the prognosis seems to be favorable and most cases resolve spontaneously. However, in some cases, prolonged therapy with corticosteroids may be required.

Acute Disease↗
PubMed1990

Human leucocyte antigens in idiopathic nephrotic syndrome in children.

An association of the idiopathic nephrotic syndrome (NS) with certain human leucocyte antigens (HLA) has been reported repeatedly. The aim of this study is to characterize further the clinical and histological features of patients with NS in relation to their HLA phenotypes. HLA antigens were determined in 132 paediatric patients with NS. In 91 steroid-sensitive patients (usually associated with minimal glomerular changes), the antigen frequencies of HLA-DR3, HLA-DR7, and HLA-B8, -DR3 combined were significantly increased compared with controls. The strongest association was observed with the combined occurrence of HLA-B8, -DR3, -DR7 (relative risk 21.5). This association and that with HLA-DR3 alone were strongest in the presence of frequent relapses and steroid dependence compared with children without or with infrequent relapses. The pattern of HLA antigens was similar in the 57 steroid-sensitive patients with biopsy-proven minimal glomerular changes. In 41 children with steroid-resistant NS (usually associated with focal segmental glomerulosclerosis) a similar trend for increased antigen frequencies was found but the data were significant only for the combined occurrence of HLA-B8, -DR3 and -DR7. In all patients combined the frequency of the HLA associations was significantly lower when the age of onset was greater than 8 years compared with that of younger patients. It is concluded that the immunogenetic background of the steroid-sensitive and steroid-resistant NS is different and age-dependent.

Adolescent↗
PubMed1990

Type III hyperlipoproteinemia in a child with hemolytic uremic syndrome.

The case of a 6-year-old girl with severe hyperlipoproteinemia and chronic renal failure that developed after hemolytic uremic syndrome (HUS) is reported. The patient was homozygous for apolipoprotein (apo) E2, and her very-low-density lipoprotein (VLDL)-cholesterol/serum-triglyceride (TG) ratio of 0.63 was unusually high. She was consistently diagnosed to have type III hyperlipoproteinemia (HLP). This is the first report of type III HLP in a child with chronic renal disease.

Apolipoprotein E2↗
PubMed1990

C5b-8 and C5b-9 modulate the collagen release of human glomerular epithelial cells.

Aside from their lytic function the late complement components C5b-9 stimulate release of prostanoids, interleukin 1 and oxygen radicals from a number of cells. Since C5b-9 has also been connected to the development of sclerosis in animal models of glomerulonephritis, we addressed the question whether C5b-9 would affect the collagen synthesis. We used human glomerular epithelial cells (GEC) obtained as primary outgrowth cultures. The cells were cultivated in the presence of 14C-proline. Collagen synthesis was quantitated by counting the radioactivity associated with collagenase digestible material. Furthermore, collagen was analyzed by SDS-PAGE. GEC in culture produce spontaneously some collagen type IV. Addition of sublytic doses of highly purified C5b-9 increased the collagen synthesis considerably within 12 to 24 hours. In the absence of C9, C5b-8 stimulated collagen synthesis to a similar extent, whereas in the absence of C7 or C8, the collagen synthesis was not enhanced. Furthermore, fluid-phase-formed C5b-9 complexes did not stimulate the collagen synthesis, indicating that assembly of the complex on the target membrane was required. Since C5b-9 deposits are found in sclerotic areas, our data support the hypothesis that C5b-9, by stimulating collagen synthesis as well as release, might contribute to the development of chronic nephritis.

Animals↗
PubMed1990

Early liver transplantation for primary hyperoxaluria type 1 in an infant with chronic renal failure.

Infantile oxalosis is the most severe form of primary hyperoxaluria type 1, an inborn metabolic disorder caused by a deficiency of the hepatic enzyme alanine: glyoxylate aminotransferase (AGT). Renal insufficiency occurs due to excessive production and renal deposits of oxalate. This report concerns a 22-month-old girl with severe type 1 primary hyperoxaluria and chronic renal failure. Liver transplantation was performed successfully as treatment of AGT deficiency. Endogenous creatinine clearance remained stable at about 10 ml/min per 1.73 m2 at 23 months after transplantation. It is suggested that liver transplantation offers potential cure of an otherwise fatal disease. However, it remains questionable if the procedure influences kidney function in the presence of advanced renal disease.

Acute Kidney Injury↗
PubMed1990

Comparison of different regimens of prednisone therapy in frequently relapsing nephrotic syndrome.

The long-term results of four different regimens of prednisone therapy were compared in 32 children with steroid sensitive, frequently relapsing idiopathic nephrotic syndrome with minimal glomerular lesions on renal biopsy. Prednisone was administered according to the following dosage schedules: 1) long-term daily, 2) standard intermittent, 3) standard alternate-day, and 4) short-term daily. Over a mean observation period of 7 years patients without steroid dependency received 19 mg/m2/day. Relapse free intervals were the longest with long-term daily prednisone therapy compared to the other three regimens. In frequently relapsing patients without steroid dependency the relapse free intervals were similar with either intermittent or alternate-day prednisone therapy (median 75d); however, they were significantly shorter with short-term prednisone therapy (median 33d). In frequently relapsing patients with steroid dependency the time of remission was generally shorter than in patients without steroid dependency (median 25d vs. 69d) with no benefit of any of the different forms of short-term treatment.

Adolescent↗
PubMed1989

Protein restriction in children with chronic renal failure?

Normal growth can be obtained in infants with congenital renal disease and advanced chronic renal failure by feeding age-corrected amounts of protein and calories. Malnutrition during the first 2 years of life leads to stunting which cannot be corrected later in life. Chronic administration of extra calories has not resulted in catch-up growth. The potential benefit of protein restriction for prevention of renal function has to be balanced against the risk of growth failure in children with chronic renal failure. The problem can only be solved by prospective controlled studies in different age groups.

Age Factors↗
PubMed1989

[Kidney involvement in autoimmune diseases].

New aspects of pathogenesis, diagnosis and treatment of renal involvement in classic generalized immune diseases are reported. In Good-pasture's syndrome survival is now possible with high-dose corticosteroids, cyclophosphamide pulse therapy and plasmapheresis. Renal transplantation can be performed with no recurrence of disease once the production of autoantibodies against basal membranes has stopped. Patients with Wegener's disease die if adequate treatment is not provided. Long-term cyclophosphamide therapy can induce complete remission. A solid-phase radioimmunoassay for detection of circulating autoantibodies to neutrophilic cytoplasmic antigens is helpful in the diagnosis and monitoring of therapy. In systemic lupus erythematosus, renal biopsy is only mandatory to solve the question as to whether high-dose steroid treatment should be prescribed: only in the case of diffuse proliferative glomerulonephritis will corticosteroids be beneficial. Intermittent pulse therapy with cyclophosphamide seems to slow down the progression of renal failure, whereas a positive effect of plasmapheresis has not been proven. Cyclosporin A reduces the dosage of corticosteroids needed.

Autoantibodies↗
PubMed1987

Anti-glomerular basement membrane antibody-mediated glomerulonephritis due to glue sniffing.

A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and deliberate sniffing of Pattex glue, a mixture of hydrocarbons which possibly may affect the structure of glomerular basement membrane. After treatment by repeated plasmapheresis and drug immunosuppression autoantibodies disappeared from serum but renal function was not influenced. Renal damage is a potential hazard for glue sniffing adolescents.

Adhesives↗
PubMed1987

Urinary excretion of glomerular basement membrane-related peptides in children with renal disorders.

Urinary excretion of glomerular basement membrane (GBM)-related peptides was analysed in 72 patients with a variety of renal diseases by immunoblotting using polyclonal antibodies against either collagenase or pepsin digests of human GBM. The specificity of the antibodies was verified by elution of antibodies bound to urinary GBM-related peptides on nitrocellulose blots and demonstration of reactivity of the eluted antibodies with the respective GBM digests. Furthermore, six mice immunized with urinary GBM-related peptides all developed focal linear deposits of mouse IgG along their GBM, linear and mesangial deposits of C3 in the glomeruli and serum antibodies reactive with human GBM. Monoclonal antibodies against urinary GBM-related peptides of one of the mice reacted with different peptides of the non-collagenous and collagenous domains of type IV collagen, the major structural protein of GBM. In the majority of the 75 patients' urines tested, excretion of GBM-related peptides with molecular weights of 33, 50, 80 and 150 kilodaltons (kD) was detectable. Patients with a diminished glomerular filtration rate (GFR) demonstrated excretion of the 33 kD peptide more frequently (91%) and never of the 80 kD peptide as compared with patients with normal GFR (33 kD [42%] 80 kD [87%]). The pattern of urinary GBM-related peptides was not specific for the underlying renal disease as in Alport's syndrome.

Adolescent↗