[Carcinoma of the appendix discovered accidentally during abdominal hysterectomy].
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Adenocarcinoma, Mucinous↗
Biomedical subjects
J Nagy
Publications and source records attributed to J Nagy.
Light chain deposition nephropathy in necropsy material.
The clinical and necropsy findings on 7 patients with monoclonal immunoglobulin light chain deposition nephropathy are described (4 cases with a myelomatous and 3 with a non-myelomatous background). All patients had moderate proteinuria and progressive azotemia. Three myelomatous and all three non-myelomatous patients died from uremia after a mean time of 12 and 23 months, respectively, from the first presentation. Immunohistochemically, 6 patients had kappa, and one had lambda light chain deposition. Light microscopically, interstitial fibrosis, tubular atrophy and arteriolar hyalinosis were present in all cases. The glomeruli showed no changes (1 case), or displayed patterns of mesangial widening: mild (1 case), nodular (mesangial nodules, 4 cases) or global lobular expansion (1 case). Mesangial nodules were observed either with or without lamellation. Around the nodules, microaneurysms were seen in 2 cases. Mesangial nodular expansion was accompanied by crescents in 56% of the glomeruli in a male patient suffering from kappa light chain deposition nephropathy without myeloma. The present findings and a review of the literature indicate the following mesangial changes in light chain deposition nephropathy: 1. no changes, 2. mild expansion, 3. nodular expansion with and without lamellation, and 4. lobular expansion. Subtypes 2, 3 and 4 may be present in parallel, may occur with or without cellular proliferation, and may be accompanied by crescents. The term nodular glomerulosclerosis to describe mesangial nodular expansion is not completely correct, and hence its use is not recommended.
Aged↗
Hyperperfusion injury in IgA nephropathy.
The authors have found morphological alterations identical with hyperfiltration glomerular injury in the renal biopsies of 23 out of 100 patients with IgA nephropathy. In 15 of them the lesions were similar to the different stages of segmental glomerular hyalinosis and sclerosis with deposition of IgM and C3. At the time of renal biopsy, the serum creatinine of all except 8 patients was normal (maximally 170 mumol/1), only 13 had hypertension, and all except 2 were proteinuric. During the follow-up period (mean +/- 2Sd = 138 +/- 76 months) all of them became hypertensive. In 10 patients end-stage renal failure developed, and in another 10, serum creatinine also became elevated (range 124 - 504 mumol/1). On the basis of these data the development of focal segmental hyalinosis and sclerosis should be interpreted as a bad prognostic sign in IgA nephropathy.
Adult↗
Hyperperfusion injury in IgA nephropathy.
The authors have found morphological alterations identical with hyperfiltration glomerular injury in the renal biopsies of 23 out of 100 patients with IgA nephropathy. In 15 of them the lesions were similar to the different stages of segmental glomerular hyalinosis and sclerosis with deposition of IgM and C3. At the time of renal biopsy, the serum creatinine of all except 8 patients was normal (maximally 170 mumol/1), only 13 had hypertension, and all except 2 were proteinuric. During the follow-up period (mean +/- 2Sd = 138 +/- 76 months) all of them became hypertensive. In 10 patients end-stage renal failure developed, and in another 10, serum creatinine also became elevated (range 124 - 504 mumol/1). On the basis of these data the development of focal segmental hyalinosis and sclerosis should be interpreted as a bad prognostic sign in IgA nephropathy.
Adult↗
[The effect of clinical and morphological parameters on the prognosis of IgA glomerulonephritis].
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Adolescent↗
[Light-chain nephropathy].
The authors deal with the clinicopathology of the renal, alterations in light-chain disease in connection with 6 cases. The disease was recognized by the monotype (in 5 cases kappa, in 1 case lambda) immunoreactivity of the light-chain paraprotein deposited in the basal membranes of the renal tissue. Electron microscopic examinations proved the fine-granulated, electrodense character of the paraprotein. Multiple myeloma was found in 3 cases and plasma cell dyscrasia of non-tumorous characteristic in 3 cases in the background of the deposition. The renal involvement appeared clinically in the picture of proteinuria without nephrosis syndrome and in progressing azotemia. Chronic renal insufficiency developed during some months in 5 patients. Morphologically renal impairment manifested in interstitial fibrosis, tubular atrophy and ateriolar hyalinosis was seen. These were associated with different glomerular alterations, for instance in 3 cases with nodular glomerulosclerosis. In 1 patient with plasma cell dyscrasia of non-tumorous characteristic nodular glomerulosclerosis and semilunar formation was observed in 56% of the glomeruli. In an other patient with myeloma the simultaneous existence of cylinder nephropathy and light-chain nephropathy was demonstrated. Both observations are unusual phenomena in plasma cell dyscrasia.
Basement Membrane↗
[Markers of peripheral B lymphocytes and their function in IgA nephropathy].
Peripheral blood B-lymphocyte markers and functions were observed in 21 patients with IgA nephropathy (IgA NP), 18 patients with systemic lupus erythematosus (SLE) and 16 controls. IgA NP B-lymphocytes similarly to that of SLE B-lymphocytes expressed significantly higher positivity with Leu 1 (CD 5) monoclonal antibody than controls. CD 5 positive B-lymphocytes are thought to be a distinct subset of the B-cells (autoregulatory B-lymphocytes) inducible in IgA NP by lipopolysaccharide (LPS) stimulation in parallel to their expression of surface IgM heavy chain positivity. The activated state of IgA NP B-lymphocytes have been proved by their higher OKIa (HLA-DR) positivities but lower IOB1a (CD 21, C3b-receptor) and decreased IgG-Fc-receptor (ox- rosette) expression. IgA NP B-lymphocytes showed a higher IgA but also IgG and IgM polyclonal immunoglobulin production than control B-lymphocytes in co-cultures with T-lymphocytes. Not only regulatory T-lymphocyte subsets but also serum derived from IgA NP patients stimulated the immunoglobulin production of IgA NP B-lymphocytes.
Antibodies, Monoclonal↗
The consequences of tubulo-interstitial changes for renal function in glomerulopathies. A morphometric and cytological analysis.
Morphometric investigation of the structures of the cortex in kidneys exhibiting various types of glomerulopathy revealed the following: 1. In various types of glomerulonephritis, diabetic glomerulosclerosis, and glomerular amyloidosis there are significant correlations between the severity of fibrosis of the renal cortical interstitium and tubular atrophy resulting from chronic interstitial inflammation, and the serum creatinine concentration, creatinine clearance, inulin clearance and PAH clearance. 2. As illustrated with the example of membranoproliferative glomerulonephritis type I, if glomerulopathy alone is present, there is no elevation of the serum creatinine concentration, even if the glomerular inflammatory changes are severe; neither are severe renal amyloidosis that is confined to the glomeruli and severe isolated diabetic glomerulosclerosis associated with elevation of the serum creatinine concentration. 3. There is a significant negative correlation between the severity of interstitial fibrosis resulting from chronic inflammation and the total number and cross-sectional area of the intertubular capillaries; i.e., the total cross-sectional area and number of capillaries per unit area decrease as the fibrosis of the cortical interstitium increases. 4. Cases of glomerulonephritis in which there is accompanying fibrosis of the renal cortical interstitium have a significantly worse long-term prognosis than those in which there is only severe glomerulitis. 5. Obliteration of the post-glomerular capillaries leads to an increase in the cross-sectional area of the glomerular capillary convolution, the morphological equivalent of an increase in intraglomerular pressure. 6. The cause of the disease of the renal cortical interstitium that may accompany the various types of glomerulonephritis is not known. It is considered possible, as a working hypothesis, that this inflammation represents a T-cell stimulated autoimmune process in which fibroblast proliferation occurs, leading to an increase in numbers of fibrocytes in the renal cortical interstitium and thus to increased production of collagen.
Creatinine↗
Antinuclear autoantibodies in sera of patients with IgA nephropathy.
IgA nephropathy (IgAN), assumed to be a chronic immune complex glomerulonephritis, has been sometimes associated with various autoimmune diseases and autoimmune phenomena including autoantibody production. The current study was aimed at thoroughly investigating the frequency of raised autoantibody titers against five common nuclear autoantigens in a large number of patients with IgAN as well as patients with other primary immune complex glomerulopathies and normal controls. The incidence of autoantibodies (greater than 2 SD less than 3 SD of controls) in the IgAN group ranged between 13% in the case of anticardiolipin antibodies, and 19% in the case of antiribonucleoprotein antibodies, yet was not found to be significantly different from the incidence observed in the other control groups. In none of the subjects a titer above 3 SD of the means of controls was found. Our study clearly points to the fact that IgAN, while being an immune-mediated disease, is not a clear-cut autoimmune condition. The finding of autoantibodies in these patients may be merely incidental.
Antibodies, Antinuclear↗
[Critical analysis of osteosynthesis of fractures of the femoral diaphysis].
Authors analyse the osteosyntheses of 98,417 segmental fractures of the femoral diaphysis. Beside the 72 per cent of excellent and good results in 19 per cent complications were found, especially after using plate osteosynthesis. In spite of the fact that Kuentscher nailing seems to be the method of choice in this paper the problem is not analysed from the viewpoint of indication but from that of the causes of prolonged healing. On the basis of their own experiences they call attention to the danger of complications. They think the choice of the most suitable method of treatment of very great importance and also the regular control, the aftertreatment and readiness to cooperation of the patients.
Adolescent↗
Peripheral B-lymphocyte markers and function in IgA nephropathy.
Peripheral blood B-lymphocyte markers and functions were observed in 21 patients with IgA nephropathy (IgA NP) and in 16 controls. IgA NP B lymphocytes expressed significantly higher positivity with Leu 1 (CD 5) monoclonal antibody than controls. CD 5 positive B lymphocytes are thought to be a distinct subset of the B cells (autoregulatory B lymphocytes) inducible in IgA NP by lipopolysaccharide (LPS) stimulation parallel to the higher expression of surface IgM heavy chain positivity. The activated state of IgA NP B lymphocytes has been proved by their higher OKIa (HLA-DR) positivities but lower IOB1a (CD 21, C3d-receptor) and decreased IgG-Fc-receptor (ox-rosette) expression. IgA NP B lymphocytes showed a higher IgA but also IgG and IgM polyclonal immunoglobulin production than control B lymphocytes in co-cultures with T lymphocytes. Not only regulatory T lymphocyte subsets but also serum derived from IgA NP patients stimulated the immunoglobulin production of IgA NP B cells.
Adult↗
[Incidence of food allergy and zinc deficiency in children treated for atopic dermatitis].
Among the 134 children who were admitted to hospital with atopic dermatitis, authors found 41, suffering from food allergy and 39, being hypozincaemic; the latter ones belonged to the group of patients with serious and extended ekzemic laesions. The lymphoblast transformation with aspecific mitogen in the blood of ten hypozincaemic patients with negative tuberculinplaster-reaction in 9 cases showed pathological decreased level, and after one month of zinc intake, 8 children's lymphoblast transformation became normal.
Adolescent↗
[IgA and IgG antibodies to Chlamydia in IgA nephropathy as well as in mesangiocapillary and membranous glomerulonephritis].
It might be supposed that, among the antigens causing chronic immune complex glomerulonephritis (IC GN), there are foreign materials, e.g. bacterial antigens penetrating the mucosal barrier. To put this hypothesis to the test, the presence and titres of IgA and IgG antibodies against Chlamydia (C., one of the most frequent bacteria causing mucosal inflammation) have been studied in the sera of 70 patients with IgA nephropathy (IgA NP), of 25 with mesangiocapillary GN (MCGN) and of 27 with membranous GN (MGN) using a single serovar (L2) inclusion immunoperoxidase assay. Significantly more IgA (titres greater than or equal to 8) and IgG (titres greater than or equal to 32) antibodies were found in the sera of IgA NP and MCGN patients than in healthy controls. These results are compatible with the hypothesis that there are some similarities between the clinical and morphological picture of IgA NP and MCGN. Furthermore, it may be assumed that in renal patients with an active C. infection (high IgG titres with IgA seropositivity) C. antigens may play a role in the production of nephropathogenic IC developing in antibody excess.
Adult↗
[Antibodies against nutritional antigens in IgA nephropathy].
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Adult↗
Plasma factors influencing PGI2-like activity in patients with IgA nephropathy and Henoch-Schönlein purpura.
Plasma factors influencing vascular PGI2-like activity (PSA) were studied in 45 patients with IgA nephropathy, 18 with Henoch-Schönlein purpura, including 8 children with nephrotic syndrome, and 41 controls. The results were compared with the levels of plasma high-density lipoprotein (HDL), low-density lipoprotein (LDL) and fatty acid components of plasma phospholipids. The plasma of 38 of 45 patients with IgA nephropathy and 14 with Henoch-Schönlein purpura showed a diminished ability or no ability to support PSA. Twenty-three patients with IgA nephropathy and 10 with Henoch-Schönlein purpura exhibited an inhibitory activity against PGI2 production. The plasma HDL level was lower, while the LDL level and the LDL/HDL ratio were significantly higher in IgA nephropathy and Henoch-Schönlein purpura cases than in the controls. A high LDL/HDL ratio was associated with a low plasma PSA. The levels of arachidonic acid and its precursor were not lower in the plasma of patients than in the controls. The decreased PGI2 synthesis may play an important role in the pathogenesis of IgA nephropathy and Henoch-Schönlein purpura, but it can not be explained by reduced PG precursors. LDL may have an inhibitory, and HDL a protective effect on PGI2 synthesis.
Adult↗
The effects of plasma from IgA nephropathy patients on vascular prostacyclin and platelet cyclic AMP production.
The effects of plasma from 10 IgA nephropathy patients and from ten controls were studied on vascular prostacyclin (PGI2) production, the cyclic AMP (cAMP) level and the aggregation of normal platelets. The ability of the plasma to support PGI2-like activity (PSA) was significantly lower in the group of patients (18.0 +/- 13.3%) than in the controls (52.6 +/- 12.9%). The concentration of 6-keto-PGF1 alpha in the supernatant of the vascular tissue was also lower following incubation with patient plasma than with control plasma (p less than 0.001). The reduced PGI2 released by the patient plasma led to a significantly lower platelet cAMP than that following the control plasma (p less than 0.01). There was a significantly positive correlation between the 6-keto-PGF1 alpha and the plasma PSA, and also between both the plasma PSA and 6-keto-PGF1 alpha concentrations and the platelet cAMP level. These findings suggest that a vascular PGI2 defect may cause a reduced cAMP production and an uninhibited aggregation of platelets, which might play a role in the pathogenesis of IgA nephropathy.
6-Ketoprostaglandin F1 alpha↗
Asperger's syndrome: a review of clinical features.
The term Asperger's Syndrome (AS) refers to a clinical picture characterized by social isolation in combination with odd and eccentric behaviour. While the syndrome has been recognized for some time, diagnostic criteria for the disorder have not yet been established or evaluated. The objective of this paper is to describe some of the clinical features of AS. Twenty-eight children and adolescents with AS were compared to a group of psychiatric outpatients (matched on age and sex) who were also socially impaired. The AS subjects showed a range of social impairments essentially different than those seen in the control group. The sex ratio, family history, and presence of neurologic disease in some AS cases, suggest that the etiology of the disorder is similar to that in autism. The methodological limitations of the study are discussed, and a revised set of diagnostic criteria are proposed for AS.
Adolescent↗
[Mesangiocapillary glomerulonephritis, type 3].
Authors describe in 2 cases of type 3 mesangiocapillary glomerulonephritis the typical histological, pathological and clinical features of the disease. It is considered to be an ultrastructural variant of mesangiocapillary glomerulonephritis accompanied by focal subepithelial deposits reminiscent of membraneous glomerulonephritis. As the background of immune-complex deposition the increase of capillary permeability observed often in diabetes mellitus is discussed. On the basis of its more benign course this pattern is thought to be a separate subtype.
Adult↗