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22 children, with the onset of the nephrotic syndrome between 1 and 6 years of age, were studied by renal biopsy because of steroid resistance or after many years of steroid-sensitive disease. Six chi

SummaryThirty cases with proteinuria either asymptomatic or associated with hypertension were selected through rigid Criteria. 17 patients are normotensive and 13 have a blood Pressure at one or multiple control over 140-90 mmHg. Renal biopsies on such patients emphasize the frequency of vascular lesions. Arteriolar lesions were the only abnormality in twenty one cases. Glomerular lesions were observed in only 8 patients. In 14 patients, with a mean age of 21, vascular lesions are noticed in the absence of hypertension. Our own observation and data of the litterature are in favour of a primary arteriolar disease.

Renal failure, which developed in two patients with sickle-cell anemia, was treated by maintenance hemodialysis for six and seven months. Medullary cystic disease appeared to cause the uremia in a 32-year-old black man with sickle-cell anemia; in the second patient, a 24-year-old black woman, histologic changes in a renal biopsy were consistent with So-Called "sickle nephropathy." When uremia supervened each patient's transfusion requirement, which had been stable at 2 units per month, increased abruptly to 5 and 6 units per month, and then fell, upon initiation of maintenance hemodialysis, to 1 unit per month. Vascular access for dialysis was provided by an internal arteriovenous fistula in one patient and an arterioarterial bovine carotid heterograft in the other. There were neither hemorrhagic nor thrombotic complications of the dialysis regimen. Coincident congestive heart failure in both patients responded well to digitalis. (N Engl J Med 291: 431–435, 1974)

Diagnostic procedures and long-term prognosis in bilateral renal cortical necrosis. Thirty-eight patients with bilateral renal cortical necrosis (BRCN) were studied with special reference to etiology, diagnostic procedures and ultimate prognosis. BRCN was of obstetrical origin in 26 patients and more frequent during the third trimester of pregnancy (21%) than earlier (1.5%). Renal biopsy, renal arteriography and hemodynamic data were useful procedures in the early differentiation of total from patchy BRCN. In patients with patchy BRCN, the percentage of destroyed glomeruli on the kidney biopsy specimen was lower than in those with total BRCN, renal arteriography showed that the cortical nephrogram was present but non-homogeneous and mean renal blood flow (MRBF) (85Kr method) fell within the range observed in patients with acute tubular nephropathy undergoing full recovery. In patients with total BRCN, cortical tissue was uniformly necrotic, the cortical nephrogram was completely absent, MRBF was always below 50 ml/100 g·min and a first component was never recognizable. Biological evidence of intravascular coagulation was inconstant. Intrarenal vascular thrombi were only found in the renal biopsy specimens of those patients with short survival. Partial recovery occurred in 16 patients and GFR increased over a one year period. Subsequent deterioration of renal function occurred in nine patients requiring chronic hemodialysis and/or renal transplantation. Diagnostic et pronostic lointain des necroses corticales bilaterales des reins. Trente-huit malades atteints de necrose corticale bilaterale des reins (NCR) ont ete specialement etudies du point de vue de l'etiologie, des methodes de diagnostic et du pronostic lointain. La NCR a ete d'origine obstetricale chez 26 malades et a ete plus souvent observee pendant le troisieme trimestre de la grossesse (21%) que plus precocement (1, 5%). La biopsie renale, l'arteriographie renale et les donnees hemodynamiques ont ete utiles pour distinguer les NCR totales et partielles. Chez les malades atteints de NCR partielle, la biopsie renale a montre que le pourcentage de glomerules detruits etait plus bas que dans les NCR totales, l'arteriographie renale a montre que la nephrographie corticale etait presente mais non-homogene et le flux sanguin renal moyen (FSRM) (methode au 85Kr) restait compris dans les limites observees chez les malades atteints de nephropathie tubulaire aigue avec guerison complete. Chez les malades atteints de NCR totale, le tissu renal cortical etait uniformement necrotique, la nephrographie corticale totalement absente, le FSRM etait toujours inferieur a 50 ml/100 g·mn et aucun premier composant n'etait individualisable. Les preuves biologiques d'une coagulation intravasculaire ont ete inconstantes. Des thrombi vasculaires intrarenaux n'ont ete rencontres en biopsie que chez les malades ayant une courte survie. Une recuperation partielle a ete observee chez 16 malades et la FG a continue a s'elever au-dela de la premiere annee. Une aggravation secondaire de la fonction renale est survenue chez neuf malades, necessit ant des hemodialyses periodiques et/ou une transplantation renale.

Renal biopsies were performed on 18 patients afflicted with moderately severe or severe rheumatoid arthritis ranging from 20 to 58 years in age and with minimal to mild urine abnormalities. No abnormality was found in 11 cases. Seven biopsies revealed minimal increase in mesangial cells and matrix on light microscopy with focal tubular atrophy in some. Electron microscopy showed slight increase in mesangium, presence of ‘deposits’ in the latter and epithelial cells and fusion of podocytes in several places. Microtubular structures were found in one case. Immunofluorescent studies were negative in all cases.

Light and electron microscopic studies were performed on renal tissue from four involved members of a family with hereditary nephritis. The two females studied had no apparent deafness or renal insufficiency, but a nonspecific mild increase in mesangial matrix was noted on light microscopic examination of the renal biopsy specimen. The two males had neurosensory deafness. One had advanced renal insufficiency with glomerular sclerosis, whereas the other had no azotemia and less histopathologic change. However, all four had a characteristic ultrastructural lesion consisting of glomerular basement membrane splitting separated by clear spaces with the accumulation of small dense particles between the thin layers. This change may be specific for certain forms of hereditary nephritis.

Summary: Nephropathy associated with the administration of methicillin and other antibiotics, occurred in four patients. High-output renal failure, hyperchloraemic acidosis and mild proteinuria were observed typically. Eosinophilia was a characteristic associated feature, and a skin rash was present in two. Recovery occurred in each case, but impairment of renal function was pro-longed. Renal biopsy performed in two patients revealed marked interstitial infiltration with mononuclear cells and eosinophils, together with patchy tubular necrosis. Follow-up renal biopsy in one patient at five months revealed residual tubular damage, interstitial cellular infiltration, and areas of interstitial fibrosis. “Methicillin kidney” appears to be a definite clinicopathological entity with features which distinguish it from other causes of renal failure in patients with severe staphylococcal infections. Because prolonged impairment of renal function may occur despite cessation of the drug, steroid therapy may be indicated during the acute phase.

Review of 1 μ Epon embedded sections from 42 renal biopsy specimens revealed a striking and consistent increase in the number of interstitial mast cells in several types of kidney disease. Interstitial mast cells were increased more than 25-fold in case of chronic glomerulonephritis (6), chronic pyelonephritis (5), chronic renal allograft rejection (5), analgesic nephropathy (1), and malignant nephrosclerosis (1). In addition, accumulation of a heretofore unrecognized type of renal cell with a hypervacuolated cytoplasm and varying numbers of metachromatic cytoplasmic granules was observed within tubular epithelium, particularly in chronic glomerulonephritis and chronic pyelonephritis. Similar appearing cells have been observed in exceptional circumstances in other epithelia where they have been termed "schollenleukocyten" or "globule leukocytes." The frequency of occurrence of these cells in several forms of renal disease is documented, and light and electron microscopic evidence indicating that they are degranulated mast cells is presented. These tubular mast cells are poorly visualized in routine histologic sections, even with toluidine blue, staining. The mechanism by which they appear in renal tubular epithelium and their function in renal disease are not known.

Participation of immunoglobulin E in immune-mediated glomerulonephritis. Renal biopsy specimens from 146 consecutive patients were studied by direct immunofluorescent tests for immunoglobulins G, M, A, D, E, third component of complement and fibrinogen; 46 of the patients had nephrotic syndrome. Glomerular-bound Ig was detected in glomeruli of 85 patients, and significant immunoglobulin E (IgE) in 22 of them. The granular glomerular deposits of six of the 22 patients comprised IgE as the dominant or codominant Ig class; in one patient it was the only Ig seen. In all other instances IgE was a relatively less obvious Ig component of glomerular deposits. No correlation was detected between serum IgE concentrations and glomerular IgE deposits. The six patients with notable IgE deposits had persistent disease clinically and generalized glomerular disease immunohistochemically; two of the six patients had rapidly progressive glomerulonephritis. Clinical data implicated the β-hemolytic streptococcus as etiologic agent in three of the six patients, and suggested it in another.

23 of 42, or 55%, of patients with systemic lupus erythematous had immunoglobulin deposits along the epidermal basement membrane of uninvolved skin (positive lupus band test [LBT]). In patients with low serum complement levels, 91% had a positive LBT), as compared with 15% in those with normal complement levels. The LBT was positive in 70% of patients with clinical and laboratory evidence of renal disease, but in only 31% of patients without renal disease. 81% of patients with the more severe histologic forms of lupus nephritis, i.e., proliferative glomerulonephritis and membranous glomerulonephritis, and positive tests, whereas only 23% with mesangial glomerulitis or normal histologic findings were positive. Immunoglobulins of the same class found in the skin were detected in the glomeruli of patients examined by renal biopsy. These results suggest that there is a relationship between the occurrence of immunoglobulin in the epidermal basement membrane and the presence of the more severe forms of lupus nephritis.

A Case of nephrogenic diabetes insipidus due to extensive sarcoid infiltration of the kidney is reported. Serum and urinary calcium were normal. Significant renal functional improvement followed administration of steroids. Repeat renal biopsy after one year suggested significant histological improvement.

Acute renal failure, microangiopathic hemolytic anemia, and intravascular clotting occurred in a 27-year-old woman during the second week after elective cesarean section of a term pregnancy. Clinical and renal functional recovery were complete after treatment with heparin. Renal biopsy showed arteriolar thrombosis, ischemic changes, and deposition of granular material (fibrinlike) in glomeruli. Analysis of renal tissue 10 months later showed resolution of the thrombotic process. The majority of previously described cases of this postpartum syndrome have ended fatally or with chronic renal failure. Although the etiology and mechanism of vascular injury remain unknown, early recognition of the clinical syndrome and treatment with heparin provide a chance for recovery.

A method which utilizes a modified semipermanent mounting medium and cold storage to preserve sections of renal tissue stained for immunofluorescence microscopy is described. Results demonstrate that tissue prepared and stored as described undergoes no alteration in the pattern or intensity of immunofluorescent staining or in tissue morphology for periods exceeding 2 years. The use of this method and standardized immunofluorescent reagents facilitates centralized interpretation of renal immunopathology in collaborative studies of renal disease.

Glomerulonephritis associated with antibody to glomerular basement membrane, shown by linear staining of the glomerular basement membrane with fluoresceinated anti-IgG antisera, was found in only 10 out of 400 (2·5%) renal biopsy specimens studied by immunofluorescence. Seven of these cases had rapidly progressive glomerulonephritis, five with lung haemorrhage (Goodpasture's syndrome) and two without, and three had less severe nephritis without lung haemorrhage. Circulating antibody to glomerular basement membrane, measured by a passive haemagglutination technique and by indirect immunofluorescence, was detected in the serum of all patients with rapidly progressive glomerulonephritis by both techniques but only by the passive haemagglutination method in two of the other three patients. Two patients died of their lung haemorrhage, one despite bilateral nephrectomy, and lung haemorrhage and circulating antibody to glomerular basement membrane persisted after bilateral nephrectomy in another patient.

A serial renal biopsy was performed in a patient with acute renal failure caused by potassium bromate intoxication. A marked tubular necrosis along with near-normal glomeruli were observed on the fourth day of the onset. A montht here after, remarkable regeneration of the tubular epithelium was noted. However, a histologically demonstrable contracted kidney continued to patient to undergo continuous hemodialysis. In our case, it was felt to be difficult to estimate the prognosis of a renal failure by renal biopsies alone, particularly those at the early stages of the disease, indicating the prognostic importance of a serial renal biopsy in such circumstances.

A woman with oliguric post partum renal failure was studied by means of the xenon-133 washout technique, renal biopsy, and selective renal arteriogram. An arteriovenous fistula was found in the lower

Summary: A twenty-four year old man with infectious mononucleosis developed acute glomerulonephritis. Investigations including renal biopsy suggested that this was an immune complex-mediated glomerulonephritis, not associated with a streptococcal infection. Such a mechanism may be responsible for other reported cases of “nephritis” in infectious mononucleosis.

10 out of 44 renal biopsy specimens obtained from patients with focal and segmental sclerosing glomerulopathy (-nephritis) (focal sclerosing lesion, sclerose s. hyalinose segmentaire et focale) were examined with the electron microscope and 11 by immunofluorescence microscopy. Particularly ultrastructural alterations are described in detail. A nephrotic syndrome was observed in 33 (75%), proteinuria in 11 (25%), and erythrocyturia in 21 (48%) patients. As the characteristic glomerular lesion a progressive increase in mesangial matrix material (sclerosis) is found which in the beginning concerns only few glomeruli and within these only a portion of the capillary tuft. The remaining glomeruli histologically show minimal changes. Electron microscopy, however, reveals these glomeruli as pathologically altered, too, indicating that the underlying mechanism is a diffuse one. Progression of sclerosing processes finally results in complete sclerosis of more and more capillary loops and glomeruli, respectively. Lightmicroscopically identifiable PAS-positive hyaline deposits were present in about 80%, thus representing an important diagnostic tool. Electron dense deposits, identical with typical immune deposits, are a regular finding in sclerosing areas and very rarely occur in minimally changed glomeruli, too. Immunofluorescence microscopy reveals a corresponding segmental deposition pattern of mostly IgM and C3 globulins. These findings are discussed with regard to an immune pathogenesis of the focal and segmental lesion.

Persistent renal dysfunction followed methoxyflurane anesthesia in two patients. In one polyuria, hypernatremia and azotemia developed. The serum creatinine level rose from 0.9 mg/100 ml preoperatively to a peak value of 2.9 mg/100 ml. Twenty-two months postoperatively the 24 hour creatinine clearance averaged 40 ml/min, and the maximum urinary osmolality was 424 mOsm/kg after dehydration and vasopressin. In the second patient the serum creatinine level rose slowly to a peak value of 7.5 mg/100 ml on the 39th postoperative day; 14 months later it was 3.8 mg/100 ml. Renal biopsy specimens obtained on the 21st and 24th postoperative days showed tubular epithelial degeneration ranging from swelling to necrosis, deposition of birefringent crystals and increased interstitial connective tissue. Repeat renal biopsy specimens obtained at 812 and 15 months postoperatively revealed more severe interstitial fibrosis. Renal insufficiency following methoxyflurane anesthesia is reviewed. The need for careful selection of patients and antibiotics, and for limitation of the anesthetic dose is apparent. The renal insufficiency can vary greatly in severity and clinical presentation, and may persist to varying degrees.

In 1957 Rich [1] presented observations in 20 children with the nephrotic syndrome who died between several months and five years after the onset of edema. One-half of these children died from infection. He described a progressive sclerosis of glomeruli starting in the juxtamedullary region of the kidney and suggested that this was the usual way in which lipoid nephrosis progressed. A few years later, studies of renal biopsy specimens from patients with the idiopathic nephrotic syndrome revealed a particular group that was characterized by the presence of focal sclerosing glomerular lesions as distinct from others with minimal or diffuse glomerular lesions [2–4]. It is only in recent years that a clear correlation has been established between these focal lesions and a specific clinical course. A high incidence of microscopic hematuria, corticosteroid resistance and developing renal insufficiency in nephrotic patients with this finding has now been reported by most authors [5–17]. Since the lesions under discussion are characterized by focal involvement of glomeruli and by the sclerotic changes which take place in affected glomeruli, the terms focal glomerular sclerosis (FGS) or focal sclerosing glomerulonephropathy [17] seem appropriate. Other names such as focal sclerosing glomerulonephritis [4, 16] or focal glomerulosclerosis [11, 15, 20] have been proposed. In our opinion the word ‘glomerulonephritis' should be avoided because of the confusion it may introduce in reference to focal and segmental glomerulonephritis [9]. The term ‘focal gbmerulosclerosis' is also confusing since, for decades, the word ‘glomerulosclerosis' has been used for the small sclerotic glomeruli often found in infant kidneys [18, 19]. The frequent histologic finding of FGS in nephrotic patients whose clinical presentation at onset could not be distinguished from that of ‘nephrosis,' or who might have exhibited minimal glomerular lesions on a previous biopsy specimen [3, 5, 8, 13, 14, 16, 17], raises an important question as to the relationship between ‘minimal lesion nephrotic syndrome' (M LNS) and this ‘disease' (FGS). A recent report of the possible recurrence of the nephrotic syndrome and the focal lesion in transplanted kidneys suggests that a humoral factor may be operative which antedates the glomerular structural changes. Accurate diagnosis of FGS in nephrotic patients may therefore be of considerable practical importance.

Glomerular disease remains an important cause of renal disease in children. We retrospectively assessed the clinical features and histopathological diagnoses in kidney biopsy specimens in children (n=27). The medical records and histopathological diagnoses between 2003 and 2006 were reviewed. In each patient, clinical presentation, age at onset of disease, gender, presence of nephrotic range proteinuria, hematuria, hypertension and histopathological diagnoses were evaluated. SLE nephritis (n=8) and membranoproliferative glomerulonephritis (MPGN) (n=8) were the most frequent glomerular diseases and nephrotic syndrome (n=21) was the frequent clinical manifestation. Key words: Renal biopsy, Glomerular diseases doi:10.3126/jnps.v29i1.1597 J. Nepal Paediatr. Soc. Vol.29(1) p.26-29

In a group of 400 nephrotic patients, both adults and children, the histological picture seen on renal biopsy, the selectivity of differential protein clearances, and the response to corticosteroid therapy where applied were studied. The only discernible difference was that of the relative incidence of underlying renal disease; in particular, the much greater incidence of “minimal change” lesions and the near absence of glomerular disease secondary to systemic disorders in children. Highly selective differential protein clearances were strongly associated with response to steroids within eight weeks, and this depended on the relation between this type of protein clearance and the minimal change lesion, which was the only histological appearance associated with complete response to corticosteroid therapy within eight weeks. Neither renal biopsy nor studies of proteinuria allowed prediction of which responding patients would subsequently relapse. Studies of differential protein clearances allow the paediatrician to avoid renal biopsy with safety in nephrotic children aged 1 to 5 years, but cannot distinguish any given renal disease with certainty. Generalized diseases affecting the kidney are usually associated with poorly selective differential protein clearances. Within all groups the most severe changes were usually associated with the least selective differential protein clearances, and vice versa.

A case of acute renal failure during intermittent rifampicin therapy (600 mg once a week) is reported. The anuria persisted for 6 days and was followed by prompt recovery. A renal biopsy showed tubula