Juvenile nephronophthisis associated with new skeletal abnormalities, tapetoretinal degeneration and liver fibrosis.

This report concerns a boy presenting renal disease with tubulointerstitial nephropathy which suggests familial juvenile nephronophthisis, hepatosplenomegaly due to congenital hepatic fibrosis, tapetoretinal degeneration, probable endocrine involvement and congenital skeletal abnormalities. The associations presented in this paper have not previously been reported.

Prognostic indicators in idiopathic IgA mesangial nephropathy.

Univariate survivorship analysis of a cohort of 365 patients with idiopathic IgA mesangial nephropathy and at least one year of further observation since the apparent onset (mean = 7.79 +/- 6.19 years; median = 6.16 years) has been performed. Observations for at least one year (mean = 5.05 +/- 3.66; median = 4.08 years) after biopsy was available for 292 of these. One immunohistological, four clinical, and six histological features were associated with increased risk of developing renal failure: (i) older at onset; (ii) no history of recurrent macroscopic haematuria; (iii) proteinuria of more than 1 g/day; (iv) arterial hypertension at the time of biopsy; (v) extent of glomerular obsolescence; (vi) extent of segmental glomerulosclerosis; (vii) presence of interstitial fibrosis; (vii) presence of diffuse intracapillary proliferation; (ix) presence of extracapillary proliferation; (x) presence of segmental thickening of glomerular basement membrane; (xi) extension of IgA deposits to the peripheral capillary loops shown by immunofluorescence. Only features (iii), (v), (vii) and (xi) proved to be independent prognostic indicators in the multivariate survivorship analysis (Cox regression model).

The detection of monocytes in human glomerulonephritis.

Renal biopsy specimens from 343 patients with primary or secondary glomerulonephritis (GN) were examined for monocytes by the non-specific esterase reaction. Large numbers of monocytes per glomerulus (M/G) were found in essential cryoglobulinemia GN (29 pts, M/G 30.6 +/- 22.4), in acute post-infectious GN (27 pts, M/G 9.1 +/- 8.3), in rapidly progressive crescentic GN (20 pts, M/G 5.6 +/- 2.7), in systemic lupus GN (61 pts, M/G 5.0 +/- 5.6), and in IgA-GN associated with chronic liver disease (5 pts, M/G 6.4 +/- 5.9) or Schönlein-Henoch purpura (15 pts, M/G 3.3 +/- 6.4). Clinico-histological correlation showed that monocyte infiltration was correlated with the extent of proteinuria (all groups), with the presence of endoluminal "thrombi" (cryoglobulinemia GN), of polymorphonuclear leukocyte infiltration (post-infectious GN), of cellular crescents (crescentic GN), of "active" lesions (lupus GN), and with the extension of lesions to the peripheral capillary walls (IgA-associated GN). The M/G index was negligible in renal amyloidosis (21 pts), in idiopathic membranoproliferative GN (10 pts), in idiopathic IgA mesangial GN (63 pts), in membranous GN (40 pts), in focal glomerulosclerosis (29 pts), in minimal change nephropathy (18 pts), and in diabetic glomerulosclerosis (5 pts). The results confirm the participation of cells of the monocyte-macrophage series in the genesis of proliferative lesions, both intracapillary and extracapillary, in immune-mediated human GN and suggest their direct involvement in glomerular injury.

Idiopathic IgA mesangial nephropathy. Clinical and histological study of 374 patients.

Histological features and data on the natural history after 1 to 45 years (mean 6.56 +/- 8.55) of total apparent duration and 1 to 13 years (mean 3.48 +/- 5.04) of post-biopsy follow-up, are reported in 374 patients (mean age, 33.9 +/- 11.9 yrs) with idiopathic mesangial IgA nephropathy, who presented with a history of macroscopic hematuria (56%), recurrent in two-thirds of the patients, or with persistent microscopic hematuria and no previous episodes of gross hematuria (44%). Mesangial cell proliferation ranged from minimal to diffuse. Associated varying degrees of extracapillary proliferation, segmental and global glomerular sclerosis, tubulo-interstitial damage and arteriolar hyalinosis usually correlated with each other and with the extent of mesangial proliferation (P less than 0.05). The actuarial curve of progression to renal death showed a 75% survival after 20 years from apparent onset. Progression to renal failure was more rapid in patients with: an older age at onset (P = 0.0582); male sex (P = 0.0730); no history of recurrent gross hematuria (P = 0.0406); high blood pressure (P = 0.0011); more marked global (P = 0.0007) and segmental (P = 0.0026) glomerular sclerosis; more severe interstitial sclerosis (P = 0.0147); more diffuse and global mesangial proliferation (P = 0.0820); mesangio-parietal pattern at immunofluorescence (P = 0.0778). However, all these parameters showed a poor predictive value if applied to any single patient.

Plasma exchange in acute renal failure by cortinarius speciosissimus.

Mushroom poisoning by Cortinarius speciosissimus (C. spec.) leading to irreversible renal failure has been described; in a recent report, an expected bad prognosis has probably been overcome as a result of the very early use of hemoperfusion. In Sep. '81, a healthy couple of wife and husband, both aged 38, had gastrointestinal symptoms 2 days after ingestion of C. spec. On day 8 an acute renal failure developed and the same day, before dialysis, plasma exchange (PE) was undertaken. Renal biopsies disclosed a common pattern of tubular necrosis, scattered infiltrates and interstitial edema. Diuresis and partial recovery started in the man on day 10, while failure persisted over six months in the woman. Further to individual sensitivity to C. spec. toxins, renal damage, once established, may be irreversible, irrespectively of early treatment. Limited usefulness of PE in C. spec. poisoning is probably related to the long latency between ingestion and occurrence of the first renal symptoms.

Immunohistological diagnosis of drug-induced hypersensitivity nephritis.

The immunohistological findings in 10 cases of DIHN and in 6 cases of R-ARF were compared with the patterns of experimental models and human examples of immunological nephritis. In most cases the simultaneous involvement of glomerular and extraglomerular structures was observed. A linear pattern on tubules together with a granular pattern on glomeruli and other structures was more frequently seen in Rifampicin adverse reactions. Direct and indirect immunofluorescence techniques performed in these last cases gave no evidence of the presence of the antigen and specific antibodies in the kidneys.

[Immunohistological aspects of nephropathy with minimal lesions]. / Aspetti immunoistologici della nefropatia a lesioni minime.

Renal biopsies from 32 patients, whose light microscopy showed a pattern of minimal changes nephropathy, have been submitted to immunohistological examination. On the basis of immunofluorescence pattern we separated the cases into three groups: the first group (12 cases) was characterized by absence of any glomerular deposit. The second group (9 cases) showed focal and segmental deposits of IgM and sometimes C3 on capillary walls. In the third group (11 cases) we observed slight deposits of IgM and sometimes C3 "comma like" over glomerular structures in all glomeruli. The site of these last deposits was sometimes mesangium and sometimes the capillary walls. The results of immunohistological examinations gave no information about the pathogenetic mechanisms of minimal changes nephropathy. We observed that the patients enclosed in the second group had a worse clinical prognosis, compared with the other two groups.

[Glomerulonephritis in the Schoenlein-Henoch syndrome in childhood]. / Die Glomerulonephritis beim Schoenlein-Henoch-Syndrome

Ten children suffering from anaphylactoid purpura Schoenlein-Henoch and presenting urinary abnormalities of various degrees and reported. The histological and immunohistochemical tests allowed to distinguish two types of glomerulonephritis in these patients: focal glomerulonephritis and extracapillary glomerulonephritis. The prognosis was always favorable: no relationship was found between the clinical signs and symptoms and the findings in the kidney. The authors discuss, on a practical and theoretical level, the indications for kidney biopsies in children with the Schoenlein-Henoch syndrome.