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Biomedical subjects

A M Wingen

Publications and source records attributed to A M Wingen.

At least 37 records · Page 2Linked to original sources
PubMed1996

24-hour blood pressure monitoring in children and adolescents after recovery from hemolytic uremic syndrome.

24-hour blood pressure monitoring is a valuable method for the diagnosis of arterial hypertension as well as for assessment of the diurnal rhythm of the arterial blood pressure (BP). The nocturnal decrease of blood pressure ("dipping") may be attenuated or abolished in children with advanced renal failure and glomerular diseases. Arterial hypertension is a longlasting problem in children who had recovered from hemolytic uremic syndrome (HUS). We therefore performed BP monitoring in 11 children and adolescents (age 1.3 to 18.8 years, 6 males, 5 females) after HUS using a portable oscillometric device (SpaceLabs 90207). Six of the subjects had a normal renal function (group A). The other 5 patients had impaired renal function with a glomerular filtration rate <60 ml/min/1.73 m2 (group B). Nocturnal dipping was calculated as nocturnal mean blood pressure minus diurnal mean blood pressure given in per cent of diurnal mean blood pressure. Two of the patients in group A had diurnal mean BP above the 95th percentile of the German collaboration study, but none of the group was hypertensive during the night, and nocturnal dipping was 13.6% (9.7-15.5%, median and range) for systolic BP and 23.7% (15.5-29.9%) for diastolic BP which is very similar to healthy children. All of the patients had a normal diurnal BP rhythm. From patients of group B, 4 had elevated diurnal mean BP and also 4 were hypertensive during the night. Nocturnal dipping was 1.4% (0.7-4.1%) for systolic and 6.8% (0-10.7%) for diastolic BP which is clearly attenuated compared to group A. We therefore conclude that arterial hypertension is more common in patients after HUS if they have impaired renal function, and diurnal rhythm of arterial blood pressure is attenuated in these patients. However, nocturnal dipping of blood pressure is not disturbed in children after HUS without renal insufficiency, even if they were hypertensive.

Adolescent↗
PubMed1995

Serum insulin-like growth factors (IGFs) and IGF binding proteins 1, 2, and 3 in children with chronic renal failure: relationship to height and glomerular filtration rate. The European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

Serum levels of insulin-like growth factor I (IGF-I), IGF-II, and IGF binding protein 1 (IGFBP-1), IGFBP-2, and IGFBP-3 were measured in 94 children with chronic renal failure (CRF). The results were compared with their respective age-dependent normal ranges, and the relationship with height and residual glomerular filtration rate (GFR) was examined. Each IGF and IGFBP was quantified by specific RIA. Serum IGF-I and IGF-II levels were in the normal range throughout their entire childhood in the vast majority of cases. The mean age-related IGF-I (0.07 +/- 0.14 SD score) and IGF-II levels (0.06 +/- 0.11 SD) were similar. Age-related IGF-II but not IGF-I levels showed a weak inverse linear correlation with residual GFRs (r = -0.24, P < 0.02). Mean age-related IGFBP-1 serum levels (1.04 +/- 0.09 SD) were slightly elevated, whereas mean age-related serum IGFBP-2 levels (3.25 +/- 0.20 SD) and serum IGFBP-3 levels (2.61 +/- 0.12 SD) were markedly elevated. Significant inverse correlations were found between GFRs and age-related IGFBP-1 (r = -0.42, P < 0.001), IG-FBP-2 (r = -0.56, P < 0.001), and IGFBP-3 (r = -0.28, P < 0.005), but the increase in IGFBP-2 with declining GFR was relatively more pronounced than the respective increase in IGFBP-1 and IGFBP-3. The correlation between age-related IGF-I and relative height in prepubertal children with CRF (n = 54, r = 0.43, P < 0.001) was lower than in prepubertal controls (n = 68, r = 0.67, P < 0.001), and the slope of the regression line was significantly less steep, indicating that the normal relationship between IGF-I and height is disturbed in CRF. The normal relationship between IGFBP-3 and height was disrupted in CRF. Forward stepwise regression analysis revealed that height in CRF is correlated with IGF-I and inversely correlated with IGFBP-2. We conclude that the imbalance between normal IGFs and excessive IGFBP serum levels in CRF plays a pathogenic role in the growth failure of these children.

Body Height↗
PubMed1993

Evaluation of protein intake by dietary diaries and urea-N excretion in children with chronic renal failure. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

In 1988 a European multicentre, randomized trial was started in order to analyse the influence of protein intake on the progression of chronic renal failure in children. Compliance to the dietary prescriptions, i.e. protein intake, was checked by written dietary diaries and in addition by urinary urea-N excretion. This provided a unique chance to compare both methods in non-hospitalized children. Of a total of 200 patients 123 were selected, in whom at least 4 consecutive dietary diaries plus 4 completely collected 24-hour urine samples were available. Whereas urea-N excretion and simultaneously recorded protein intake did not correlate well, mean urinary urea-N excretion and mean protein intake of at least 4 observations in each patient correlated highly (r = 0.803, p = 0.0001). The difference between protein-N intake and urea-N excretion was not a constant amount of 0.031 g/kg/day as proposed by Maroni et al. [1985] but figured at 0.085 +/- 0.061 g/kg/day and was highly correlated to protein intake (r = 0.839, p = 0.0001). The correlation of protein intake and urea-N excretion was best described by the formula: protein-intake (g/kg/day) = (urea-N excretion [g/kg/day]x 15.39) -0.8 or protein intake (g/kg/day) = urea-N excretion (g/kg/day) x 9.5. Maroni's formula underestimated the high protein intake of young children. In only a few patients dietary diaries severely underestimated protein intake as compared to calculation by urea-N excretion.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
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↗