PubMed Health⌕ Search

Biomedical subjects

R H Kauffmann

Publications and source records attributed to R H Kauffmann.

At least 37 records · Page 2Linked to original sources
PubMed1992

Dialysis associated mucormycosis and desferrioxamine treatment: a case report with review of the role of oxygen radicals.

A hemodialysis patient with pulmonary and cerebral mucormycosis is reported. Recently the occurrence of this infection in dialysis patients has been associated with desferrioxamine (DFO) treatment. A causal relation seems likely, but has not been proved. Several pathogenetic mechanisms are discussed, in particular the influence of DFO on host neutrophil defenses through a decreased oxygen radical production.

Adult↗
PubMed1989

Tubulointerstitial nephritis associated with pyrazinamide.

Modern antituberculous therapy consists of a combination of several drugs, some of which (e.g. rifampicin and streptomycin) may cause impairment of renal function. Pyrazinamide therapy has been associated with dose-dependent hepatotoxicity, hyperuricaemia, arthralgia and arthritis. The patient described in this report developed renal failure, fever, arthritis and arthralgia during administration of isoniazid, rifampicin, streptomycin and pyrazinamide. The renal biopsy showed tubulo-interstitial nephritis. After withdrawal of pyrazinamide, while continuing all other drugs, both renal function and histological findings improved which points to an association of renal failure with pyrazinamide.

Acute Kidney Injury↗
PubMed1984

Circulating and mesangial secretory component-binding IgA-1 in primary IgA nephropathy.

In a prospective study of 38 patients who presented with hematuria of renal origin, 15 patients were found to have primary IgA nephropathy and 23 had other renal disorders. Sera and renal biopsy specimens of these patients were studied for the presence of macromolecular IgA1 and IgA2 using monoclonal antibodies, and the presence of J-chain as demonstrated either by immunofluorescence or its capacity to bind free secretory component. Circulating macromolecular IgA was found exclusively in the sera of patients (80%) with primary IgA nephropathy. In these sera the polymer/monomer ratio for IgA1 (0.64 +/- 0.13) was significantly higher than for normal human serum (0.39 +/- 0.01) (P less than 0.001), while no differences were found for IgA2. The polymeric IgA1 was isolated from serum by gel chromatography and was shown to have the capacity to bind free secretory component. Direct two-color immunofluorescence studies revealed the presence of only IgA1 in the mesangial deposits and also its capacity to bind free secretory component. We conclude (1) that demonstration of circulating macromolecular IgA in patients with renal hematuria is of diagnostic value and (2) that antigenetic similarities between the circulating and the mesangial macromolecular IgA suggest that dimeric IgA1 is deposited in the mesangium of patients with primary IgA nephropathy.

Antibodies, Monoclonal↗
PubMed1983

Presence of circulating macromolecular IgA in patients with hematuria due to primary IgA nephropathy.

The relation between renal histologic features and the presence of circulating immune complexes was studied in 50 patients with hematuria. Primary IgA nephropathy was found in 25 patients, and various other forms of glomerulopathy were seen in the remaining 25 patients. Circulating immune complexes were detected with the 125I-C1q-binding assay, the conglutinin-binding assay, and the anti-IgA inhibition binding assay, the latter detecting specifically IgA-containing immune complex-like material. The 125I-C1q-binding assay gave negative findings for all patients except one. With the conglutinin-binding assay, immune complexes were found in a similar frequency for patients with and without IgA nephropathy. However, the anti-IgA inhibition binding assay gave positive results only in patients with primary IgA nephropathy (68 percent) and in none of the other patients. Sucrose density ultracentrifugation, as well as experiments in which the anti-IgA inhibition binding assay was performed with and without pretreatment of serum with polyethylene glycol, showed the presumed IgA immune complexes to have intermediate sedimentation coefficients (11 to 21S). The presence and level of this macromolecular IgA in the circulation correlated significantly (p less than 0.001) with the presence of hematuria in patients who had this clinical manifestation intermittently. Furthermore, a significant correlation (r = 0.69, p less than 0.0001) was found between the degree of hematuria and the degree of positive findings of the anti-IgA inhibition binding assay. This study shows that in patients presenting with hematuria, a positive finding on the anti-IgA inhibition binding assay is restricted to patients with primary IgA nephropathy and therefore could be of diagnostic value.

Binding Sites, Antibody↗
PubMed1983

The significance of immunofluorescent immunoglobulin inclusions in polymorphonuclear leucocytes for the detection of circulating immune complexes.

Cytoplasmic inclusions of immunoglobulins and complement, detected by fluorescent antibodies in polymorphonuclear leucocytes (PMNs) that have been incubated with sera of certain patients, are considered to represent immune complexes (IC). The usefulness of this test--the indirect PMN phagocytosis test (IPPT)--for the detection of circulating IC was investigated using preparations of free and heat-aggregated immunoglobulins. Free IgG and IgM were phagocytozed by PMNs at high concentrations only, while free IgA was not phagocytozed at all. Normal human serum slightly enhanced the uptake of free IgG and IgM, but not of free IgA. Aggregates of IgG and IgA underwent phagocytosis at low concentrations, but IgM aggregates were not taken up more readily than free IgM. The uptake of IgG aggregates decreased in the presence of serum, while there was no influence upon the phagocytosis of IgA aggregates. Phagocytosis of C3 occurred only with IgG aggregates. In the presence of aggregates of IgG or IgA the phagocytosis of free immunoglobulins of other classes, in particular IgM, increased. The results of the IPPT for patients' sera showed that inclusions of C3 were found more frequently in combination with IgA or IgM than with IgG. Comparison with the 125I-Clq binding assay and the anti-IgA inhibition binding assay disclosed significant correlation between the phagocytosis of IgG and the precipitation of 125I-Clq and between the phagocytosis of IgA and the results of the anti-IgA inhibition binding assay. The PMN phagocytosis test may be useful for the detection of IgG and IgA containing IC but inclusion of IgM and C3 should be interpreted with some reserve.

Antigen-Antibody Complex↗
PubMed1982

Combined hereditary deficiency of the sixth component of complement and factor VIII coagulant activity in a Dutch family.

Prompted by previous observations of defective blood clotting in rabbits deficient in the sixth component of complement (C6), and the discovery of a patient with both C6 and factor VIII deficiency, an evaluation was made of the haemostatic functions in this individual and his family members. The family contained three members homozygous for C6 deficiency (C6D); two of them were deficient also in factor VIII. In addition, one other member of the family was only deficient in factor VIII. The only C6D member without haemophilia A had a normal recalcification time without clinical symptoms of a bleeding disorder. Reconstitution of factor VIII and C6 deficient plasma from the various members of the family in this study with purified human C6 did not result in a change in the recalcification time. The results obtained from this study also indicate that there is no linkage between the inheritance of C6 and factor VIII.

Adult↗
PubMed1981

The clinical implications and the pathogenetic significance of circulating immune complexes in infective endocarditis.

Circulating immune complexes were determined with the 125I-Clq binding assay and the conglutinin binding assay in a prospective, longitudinal study of 40 patients with infective endocarditis, 34 patients with endocardial defects and nonseptic fever and 25 patients with septicemia without endocarditis. Fourteen patients with uncomplicated valvular lesions constituted a control group. Upon admission, 63 percent of the patients with infective endocarditis had a positive 125I-Clq binding assay versus 9, 12 and 7 percent, respectively, of the other three groups (p less than 0.001). The incidence of positive conglutinin binding assays became significantly higher during the course of infective endocarditis (53 percent) than during the course of nonseptic fever (21 percent), but, upon admission, this difference was not significant. The high incidence of Clq-binding immune complexes among patients with infective endocarditis could be attributed mainly to those patients with the characteristic features of subacute endocarditis. The incidence of circulating immune complexes in acute endocarditis was low and did not contribute to making the clinically important distinction from septicemia without endocarditis. A rise in the 125I-Clq binding assay levels during the course of infective endocarditis correlated significantly (p less than 0.01) with failure of antibiotic treatment. With the 125I-Clq binding assay, significantly higher levels were found in patients with signs of renal involvement of cutaneous vasculitis than in patients without these extracardiac manifestations of endocarditis. These results show that the determination of circulating immune complexes has clinical implications for both the diagnosis and the management of infective endocarditis and that circulating immune complexes are probably involved in the development of glomerulonephritis and vasculitis.

Adolescent↗
PubMed1981

The specific detection of IgA in immune complexes.

An assay to detect IgA in circulating immune complexes (IC) using low avidity goat IgM antibody against human polyclonal IgA is described. The binding of this antibody to IgA coupled to Sepharose 6B is inhibited by IgA-containing IC. The specificity and sensitivity of this anti-IgA inhibition assay (a-IgA-InhA), was evaluated with aggregated purified immunoglobulins, sera of patients with Henoch-Schönlein purpura and normal human sera. Aggregated immunoglobulins of the IgA class, but not monomeric IgA, were reactive. Sucrose density ultracentrifugation showed that large IgA constituents (greater than 19S) were found only in the sera of patients with Henoch-Schönlein purpura. Both these sera and normal human serum contained smaller IgA components (between 7S and 19S), probably small polymers of IgA, which were reactive in this assay and interfered with detection of IgA-containing IC. Redissolved precipitates obtained from normal serum with polyethylene glycol showed reduced reactivity in the test, whereas the inhibitory activity of IgA-containing IC in sera of patients with Henoch-Schönlein purpura was retained in the precipitates. Precipitation of sera with polyethylene glycol allowed detection of smaller quantities of IgA-containing IC in patients with Henoch-Schönlein purpura.

Antigen-Antibody Complex↗
PubMed1981

Presence of immune complex-like material in sera of patients with paraproteinaemia.

Immunoglobulins of all classes as well as C3 are phagocytosed by normal human granulocytes from sera containing paraproteins. The material that was phagocytosed had the sedimentation properties of immune complexes. Cytostatic treatment did not seem to have a clearcut influence on the presence or absence of these complexes. There was little correlation with two other immune complex detecting tests. The Clq binding test was frequently found positive in paraproteinaemic sera but without apparent correlation to IgG phagocytosis. On the other hand the conglutinin binding test was rarely positive, although C3 was frequently phagocytosed.

Antigen-Antibody Complex↗
PubMed1981

Plasmapheresis in rapidly progressive Henoch-Schoenlein glomerulonephritis and the effect on circulating IgA immune complexes.

Two adult patients with Henoch-Schoenlein purpura and rapidly progressive glomerulonephritis were treated with plasmapheresis. One patient also received cyclophosphamide. Both patients recovered their renal function. Before plasmapheresis circulating IgA immune complexes were demonstrated in both patients by two assays with specificity for IgA. The level of IgA immune complexes decreased after each plasma exchange. IgA immune complexes disappeared in the patient who was treated with cyclophosphamide but remained present in the other patient. Plasma exchange may be a useful form of therapy for patients with Henoch-Schoenlein purpura and progressive renal failure. Measurement of circulating IgA immune complexes may provide insight into the in vivo effect of plasmapheresis.

Antigen-Antibody Complex↗
PubMed1980

Allergic vasculitis. A histological and immunofluorescent study of lesional and non-lesional skin in relation to circulating immune complexes.

This study concerns 57 patients who fulfilled histological criteria for the diagnosis allergic vasculitis. For 37 of these patients, biopsy specimen were available from lesional and adjacent non-lesional skin. Histological signs of vasculitis were found at both sites, but in clinically normal skin the perivascular infiltrate was less dense and neutrophils and eosinophils were sparse or absent. Fibrin was found in only ten patients and occurred less frequently in non-lesional skin. Deposits of immune complexes and/or complement were detected by immunofluorescence in 49 of the 57 patients. Hardly any differences between lesional and non-lesional skin were found with immunofluorescent microscopy. Circulating immune complexes were detected in 45 of the 56 available sera. A relationship was found between the class of immunoglobulin in immune complexes in the vessel wall and in the circulation. Moreover, the class of immunoglobulin seemed to be related to the course, the extracutaneous involvement, and the presence of associated diseases. No explanation was found for the histological differences observed between lesional and non-lesional skin.

Antigen-Antibody Complex↗
PubMed1980

Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA.

In Henoch-Schönlein purpura immune complexes in inflamed vessel walls characteristically contain immunoglobulin A(IgA). To determine whether IgA is also the predominant immunoglobulin in circulating immune complexes, we compared the results of three immune complex assays with specificities for different classes of immunoglobulins in a longitudinal study of 37 patients (30 children and seven adults) with Henoch-Schönlein purpura. Circulating IgA-containing immune complexes were detected by their reactivity with a low avidity anti-IgA antibody in 27 of the 37 patients. IgA was simultaneously present in cutaneous vessel walls in 95 percent of the patients with circulating IgA-containing immune complexes. High levels of IgA-containing immune complexes were found only during the initial phase of the disease. Immune complexes containing bound complement breakdown products were demonstrated by binding to conglutinin. IgA was found in these immune complexes in 17 patients, IgG in 17 and IgM in nine patients. There was no apparent relation with the class of immunoglobulin in the deposits. Conglutinin-binding immune complexes were present later in the course of the disease and after remission. C1q-binding immune complexes were only found in two patients. These findings suggest that immune complexes-containing IgA may initiate the vasculitis of Henoch-Schönlein purpura, whereas complement-reacted immune complexes containing immunoglobulins of the other classes appear in the circulation in a later phase.

Adolescent↗
PubMed1980

Extreme potassium loss in a patient with severe graft versus host disease.

A 19-year-old male, suffering from post-hepatitis aplastic anaemia, was transplanted with bone marrow cells from his HLA-identical, MLC non-reactive brother. Haematological recovery ensued, but the patient also developed grade IV graft-versus-host disease (GVHD). In addition to involvement of skin, liver and gut, the kidney seemed affected by GVHD since the patient has hypokalaemia and severe hyperkaluria. Other causes of urinary potassium loss were excluded. The amount of potassium loss correlated well with the severity of the GVH-reaction. Although coagulation disorders prohibited a kidney biopsy, the clinical course suggested GVHD to be the cause of the urinary potassium loss.

Adult↗