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Biomedical subjects

A M Wingen

Publications and source records attributed to A M Wingen.

53 records · Page 3Linked to original sources
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↗
PubMed1987

Complement membrane attack (MAC) in idiopathic IgA-glomerulonephritis.

Antigens of the membrane attack complex of complement (MAC), such as C5, C6, C9 and MAC-related neoantigen(s), were demonstrated in the mesangium of 23 cases with IgA-glomerulonephritis (IgA-GN) and two cases with Henoch-Schönlein purpura nephritis (HSP). High specificity of the polyclonal antibodies was verified by dot-blot analysis. Control specimens lacking immunoglobulin deposits were negative for MAC-related antigens. Markers of classical pathway activation (Clq and C4) were observed only in two of 24 and one of 23 cases of IgA-GN and HSP, respectively. Glomerular distribution patterns (mesangial vs. mesangio-peripheral) of immunoglobulin or complement deposits were correlated for IgA and C3b/iC3b (P less than 0.002), for IgA and properdin (P less than 0.002) and for IgA and MAC neoantigens (P less than 0.01). Double immunostaining experiments revealed co-localization of IgA and MAC neoantigens at identical mesangial and capillary sites. Glomerular distribution of the less pronounced IgG or IgM deposits did not correlate with that of any complement-derived antigen. The pattern of MAC-related antigens was found to be uniformly either mesangial or mesangio-peripheral. Staining for MAC-related antigens was less intense in IgA-GN cases with minimal glomerular lesions than in cases with more advanced non-sclerosing lesions. IgA, C3d, and MAC localized in corresponding glomerular sites. This is consistent with complete local activation of complement by glomerular IgA deposits via the alternative pathway. The possibility exists that MAC plays a pathogenetic role, such as by irritation of bystander cells, in IGA-GN and HSP.

Adolescent↗
PubMed1987

Evidence for developmental changes of type IV collagen in glomerular basement membrane.

Digestion of adult glomerular basement membrane (GBM) with collagenase releases a number of peptides of which the noncollagenous region of type IV collagen is detected by antibodies from patients with anti-GBM nephritis. In our study, 17 of 19 sera reacting with GBM, in indirect immunofluorescence, with cryostat sections of adult kidneys were negative with cryostat sections of fetal kidneys. However, after digestion with collagenase, a similar pattern of peptides was released from both fetal and adult GBM. SDS-PAGE immunoblotting revealed comparable antibody binding of all 19 sera with the peptides from either fetal or adult GBM. These findings suggest conformational differences between type IV collagen in fetal and adult GBM or masking of the antigen by other GBM constituents which may shield the antigen.

Adult↗
PubMed1986

Characterisation and specificity of glomerular basement membrane antigens identified by sera of patients with anti-GBM nephritis.

The sera of 21 patients positive for antibodies against GBM in indirect immunofluorescence tests were examined by immunoblotting. We demonstrated antibodies against 50, 48, 43 and 29 kD molecular weight peptides in 20 of 21 sera using collagenase-digested GBM, in 19 of 21 using trypsin-digested GBM, and in 10 of 21 using elastase-digested GBM. Although the spectrum of molecular weights of the antigenic proteins was similar in all three digests, they differed with respect to preservation of antigenicity upon reduction with mercaptoethanol. Many of the sera of patients and controls reacted with proteins unrelated to GBM, e.g. albumin and prealbumin. Furthermore, some control sera reacted with one single peptide of the above-mentioned specific GBM peptides. Our results suggest that the highly purified 29 kD peptide of the collagenase digest or the 50 kD peptide of the trypsin digest provide the best antigens to develop a screening test for antibodies against GBM. However, serum antibodies against these antigens will not be absolutely specific for anti-GBM antibody-mediated nephritis, as shown by the immunoblot experiments.

Adolescent↗
PubMed1985

Spontaneous remissions in frequently relapsing and steroid dependent idiopathic nephrotic syndrome.

We studied retrospectively the clinical course of 32 steroid sensitive nephrotic children with frequent relapses with or without steroid dependency associated with minimal glomerular lesions. Six hundred twenty-seven relapses were traced during a mean observation period of 7 years per patient. The median period between the beginning of significant proteinuria and the start of prednisone therapy was 5 days (range 0-194 days). At least one spontaneous remission occurred in 10 of the 15 frequent relapsers and in 11 of the 17 steroid dependent patients. Twenty-three percent of the relapses in frequent relapsers and 10% of the relapses in steroid dependent patients remitted spontaneously. The disappearance of proteinuria was documented in 79% of these relapses between day 4 and day 14 after onset. A delay of up to 10 days in starting prednisone therapy did not influence the further course of this or the subsequent relapse. Our results suggest that this delay is justified in the absence of progressive clinical signs and may, by the occurrence of spontaneous remissions, contribute to reduce the total amount of steroids needed.

Adolescent↗
PubMed1985

Acute renal failure in an infant with partial deficiency of hypoxanthine-guanine phosphoribosyltransferase.

A three week old boy presented with pneumonia, weight loss, metabolic acidosis and renal failure (serum creatinine 3.1 mg/100 ml, uric acid 11.5 mg/100 ml). Renal biopsy revealed severe crystal nephropathy. Low activity of hypoxanthine-guanine phosphoribosyltransferase (HPRT) in erythrocytes and fibroblasts suggested a partial deficiency of the enzyme. A family study proved the mother to be heterozygous and the maternal grandfather to be hemizygous for HPRT deficiency. The grandfather developed gouty nephropathy and uraemia. The propositus was treated with allopurinol and kept on low purine diet and high fluid intake with sodium bicarbonate. Thereafter GFR gradually improved. At the age of two and a half years, growth and psychomotor development were normal, but ultrasound examination still revealed a dense renal parenchyma. Partial HPRT deficiency is a newly recognised treatable form of renal failure in the newborn.

Acute Kidney Injury↗
PubMed1983

HLA phenotypes and idiopathic nephrotic syndrome in children.

Ninety-four children with idiopathic nephrotic syndrome (17 steroid-resistant with a histological diagnosis of a focal segmental glomerulosclerosis) were typed for HLA-A, B and DR antigens. The patients showed a significant increase of DR7 (58% vs 18%, p less than 0.0001) and of B8-DR3 (27% vs 5%, p less than 0.05). Combination of both markers (DR7 and B8-DR3) was observed in 14 per cent of patients but in none of the controls (relative risk 15.2). Patients with B8-DR3 and DR7 had a more severe course of INS.

Child↗
PubMed1980

[Kawasaki syndrome--a new disease?].

Kawasaki recognized in 1967 the acute febrile mucocutaneous lymph node syndrome (MCLS) as a well defined entity among a variety of hitherto unidentified atypical exanthems. The etiology is uncertain. There are close relations to infantile polyarteritis nodosa (IPN) which is probably the severe variant of Kawasaki's disease. Histologically it is a generalized necrotizing vasculitis, most probably caused by circulating immune complexes. The disease is supposed to be initiated by various infections in patients with certain predispositions. Considerations about etiology and pathogenesis as well as relations to IPN are mainly discussed theoretically. Therefore it is recommended to investigate the Kawasaki syndrome following a devised protocol.

Bacterial Infections↗
PubMed1979

[Morbus Hodgkin as a second malignancy in acute lymphoblastic leukaemia (author's transl)].

19 months after the clinical manifestation of acute lymphoblastic leukaemia, Hodgkin's disease, stage Ia, of the cervical lymphnodes developed in a 10 year old girl during continuous complete remission of leukaemia under chemotherapy. After a regional irradiation and after completing the antileukamic therapy the patient is at present off therapy, healthy and without signs of relapse of both malignant systemic diseases. The coincidence of acute lymphoblastic leukaemia in children with other malignant neoplasias is rare. The expected frequency of second malignancies and the theories concerning oncogenesis are shortly reviewed.

Child↗