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In a retrospective clinicopathological study, 48 kidney biopsy specimens from 16 children (mean age, 7 years) and 17 adults (mean age, 33 years) with histological evidence of focal glomerular sclerosis (FGS) were examined using light, immunofluorescence and electron microscopy. The histopathological findings were related to the clinical course of each patient. At the clinical onset of the disease, the nephrotic syndrome was seen more commonly in children (12/16) than adults (7/17), while the incidence of both hypertension (children 1/16 versus adults, 9/17) and renal insufficiency (children, 0/16 versus adults, 7/17) was greater in adults. Despite a shorter average follow-up, (adults 3 10/12 years versus children, 7 years), the incidence of hypertension (adults, 13/17 versus children, 7/16) and renal functional impairment (adults, 13/17 versus children, 3/16) remained greater in the adult patients. One child and three adults died in renal failure while two adults underwent transplantation and on requires regular dialysis therapy. Nine of 15 pediatric patients treated with corticosteroids experienced partial or complete remission in either their nephrotic syndrome or level of urine protein excretion, while just 3 of 6 adult patients treated with corticosteroids experienced a partial remission, but never became protein-free. There was an excellent correlation in all patients between the degree of functional renal impairment and the extent of glomerular and nonglomerular histopathological damage in the kidney. It is concluded that in the adults, FGS represents a more severe and progressive disease process and is less responsive to therapy.
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PMID:Focal glomerular sclerosis: contrasting clinical patterns in children and adults. 110 46

Three siblings (two brothers and a sister) of Polish origin, presented in late middle age with the features of the nephrotic syndrome and hypertension. Glomerular deposition of amyloid was found in all on renal biopsy. No amyloid was seen on rectal or gingival biopsy. Their mother and a maternal aunt died in middle age with a clincally similar disease. These cases are compared with other forms of hereditary amyloidosis which are briefly reviewed. The hereditary amyloidoses are classified with particular emphasis on neural and renal involvement.
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PMID:Familial renal amyloidosis. Case reports, literature review and classification. 113 43

A 28 year old woman, with diabetes since age 18, had the nephrotic syndrome, hypertension and renal insufficiency. The initial renal biopsy specimen revealed diffuse glomerulosclerosis with early nodular changes. After an initial decline in renal function, her creatinine clearance progressively improved and has remained normal. Within 2 years she had a spontaneous remission of the nephrotic syndrome despite the presence of more pronounced nodular glomerular lesions. Although the renal hemodynamic functions were normal, certain tubular functions were impaired. Since we found no etiology for the nephrotic syndrome other than diabetic glomerulopathy, the complete remission of the nephrotic syndrome and improvement in renal function were very unusual events.
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PMID:Spontaneous remission of the nephrotic syndrome in diabetic nephropathy. 116 52

Comparative morphological and clinical studies of 2,500 patients suffering from glomerulonephritis, enabled us to divide the different forms of diffuse glomerulonephritis into 3 distinct groups and to separate these groups from the focal glomerulonephritides. The different forms of diffuse glomerulonephritis in group I are: 1. endocapillary (acute) glomeruloenphritis (of the post-streptococcal type), 2. mesangioproliferative glomerulonephritis, 3. mesangioproliferative glomerulonephritis with focal crescents, 4. mesangioproliferative glomerulonephritis with focal scarring, 5. minimal proliferating intercapillary glomerulonephritis without nephrotic syndrome. It is emphasised that these forms can transform into one another, that they seldom occur with nephrotic syndrome, and with varying frequency with hypertension. Group II consists of: 1. minimal proliferating intercapillary glomerulonephritis with nephrotic syndrome, 2. focal sclerosing glomerulonephritis, 3. perimembranous glomerulonephritis, 4. membranoproliferative glomerulonephritis, 5. lobular glomerulonephritis. It is stressed that these glomerulonephritis forms usually do not develop out of group I type glomerulonephritis forms, and that in this group a nephrotic syndrome is the most prominent clinical syndrome. In the third group are 1. mesangioproliferative glomerulonephritis with diffuse crescents, 2. necrotising glomerulonephritis. It is shown that this form of glomerulonephritis does not usually develop from either group I of II forms. The fourth group of focal glomerulonephritis is uncommon. This disease is characterized by a necrotising and proliferative inflammatory lesion found segmentally and focally in the glomeruli. Most of the other glomeruli appearing normal. It is emphasised that in the literature the diagnosis focal glomerulonephritis is made far too often. This is because glomeruli in which the inflammatory process in a few lobules is of varying prominence, are included in the focal glomerulonephritis group. The classification of the different forms of glomerulonephritis into 3 groups here described, is thought of as a basic classification. It is compared with Ellis' classification (1942), with which it has much in common.
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PMID:The different forms of glomerulonephritis morphological and clinical aspects, analyzed in 2500 patients. 124 3

In a prospective study of renal involvement in 100 consecutively hospitalized patients with hepatosplenic schistosomiasis mansoni, 15 exhibited persistent proteinuria of varying degree, which in 6 instances was accompanied by hypertension. Nine patients had the nephrotic syndrome. The most common glomerular lesion in this group was membrano-proliferative glomerulonephritis. Surgical biospy obtained during splenectomy in 15 patients without clinical evidence of renal involvement showed glomerular lesions in 6 instances. Focal proliferative glomerulonephritis was the most common lesion in this group. The detection of silent glomerular lesions in patients with Schistosoma mansoni infection suggests that the glomerular alterations may precede clinical manifestations of renal disease.
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PMID:Renal involvement in patients with hepatosplenic Schistosomiasis mansoni. 125 74

Early renal biopsies performed in children with primary nephritic syndrome of acute onset have identified various glomerular pathological patterns with distinct evolution. Complete recovery has been observed in those cases with diffuse endocapillary proliferation. However, progressive disease has resulted from most of the other forms. A histological type characterized by diffuse glomerular endocapillary proliferation with a variable number of glomeruli affected by extracapillary proliferation with crescents, has been observed. A diffuse form of this entity, that is, crescents in 80 to 100 per cent of the glomeruli, has been described in the so-called "rapidly progressive glomerulonephritis". This lesion usually progresses to renal insufficiency in less than six months. Information concerning the focal form, that is, the presence of crescents in less than 80 per cent of the glomeruli, is limited and the long-term prognosis of this morphological finding has not been conclusively established. This communication deals with: 1) history and initial clinical features in 48 patients with focal endo and extracapillary glomerulonephritis, and 2) the correlations between streptococcal etiology, initial features, percentage of glomeruli with crescents and the final outcome, in 27 cases followed for more than two years. Age of patients at the onset of the disease was between 11 months and 14 years; 63 percent of the patients were male. Streptococcal infection was documented in 20 out of the 48 cases. Nephritic syndrome at onset was found in all the 48 cases. Twenty-nine patients had significant proteinuria and in 16 of these, the characteristics of the nephrotic syndrome were also present; 10 patients presented severe arterial hypertension and in other 6, acute renal failure was an initial feature.
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PMID:[Development and prognosis of diffuse endocapillary and focal extracapillary proliferative glomerulonephritis]. 127 65

Nine hundred and forty children with hematuria were admitted to the nephrology service from 1958 to 1973. Percutaneous renal biopsies were performed in all of them. Thirty cases out of this group had recurrent hematuria and constitute the clinical material of this study. The clinical picture was: acute nephrotic syndrome in 19; monosymptomatic hematuria in 8, anaphylactoid purpura nephritis in 2, and hematuria associated with nephrotic syndrome in one patient. All patients with nephritic syndrome showed in their biopsies various types of glomerular lesions; most of the patients with monosymptomatic hematuria had normal glomeruli; at the light microscopy, the immunofluorescence was positive in some of them. Both patients with anaphylactoid nephritis showed diffuse endocapillary and focal extracapillary proliferation and in the only one with nephrotic syndrome, the hematuria was familial and the biopsy showed features of Alport's syndrome. Twenty-four patients who were followed for over two years showed no relationship between the age of onset, sex, initial significant proteinuria, hypertension, frequency of bouts of hematuria and the clinical evolution. At the end of the study, 7 patients had prolonged remission: the light microscopy showed normal glomeruli, endocapillary proliferation and endo and extracapillary proliferation with less than 30% of the glomeruli affected by "crescents". The remaining cases were still active and one of them with endo and extracapillary glomerulonephritis with more than 30% of the glomeruli affected by "crescents", developed chronic renal insufficiency. In conclusion, the prognosis of recurrent hematuria of glomerular origen is related with the type of glomerular lesions and constitutes an indication for renal biopsy. Renal specimens must be studied under light microscopy and immunofluorescence techniques; electromicroscopy is required when hematuria is present in more than one member of the family.
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PMID:[Recurrent hematuria of glomerular origin]. 127 66

The clinical and morphological analysis is reported of the changes evolving in 12 cases of mesangial proliferative glomerulonephritis based on repeated biopsies carried out at intervals from 1 to 7 years. Clinical findings at the time of the first biopsy included in 2 cases nephrotic syndrome, and in the remaining cases proteinuria ranging from 0.3 to 2/1000. In all cases erythrocytes in urine were present ranging from 4-6 to 50-100 erythrocytes per field of vision. Hypertension was found in 4 cases, and increased serum creatinine level in 1 patient. At the time of repeated biopsy in 11 cases clinical evidence of improvement was noted with decreased proteinuria anderythrocytes in urine. In 1 case worsening was found and several months later signs of renal failure developed which led to death. The morphological examinations demonstrated in the first biopsies mesangial proliferative glomerulonephritis. In second biopsies these findings were confirmed. In 1 case with unfavourable outcome the changes became more pronounced, and in the remaining ones no significant differences were noted in relation to the first biopsy which evidenced lack of a close correlation between the clinical condition and the morphological state of the kidneys.
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PMID:[Evolution of morphological and clinical changes in mesangial proliferative glomerulonephritis]. 127

Loop diuretics (furosemide, bumetanide, muzolimine, piretamide, torasemide) are powerful drugs capable of increasing sodium excretion and urine output even when renal function is markedly impaired. In patients with chronic renal failure (CRF), loop diuretics may be given to control extracellular volume (ECV) expansion responsible for hypertension. But the use of loop diuretics in chronic uremia is mostly helpful when impaired renal function co-exists with nephrotic syndrome or chronic heart failure. Due to their powerful natriuretic activity, loop diuretics have been administered also to patients on maintenance dialysis to reduce the frequency of and/or to curtail dialysis time. In this condition, however, the increase of sodium and water excretion is very limited; whereas the use of diuretics in high dosage is not devoid of risky side effects such as neurologic lesions, cramps, deafness, weakness, muscle pain. In some patients with oliguric form of acute renal failure (ARF), loop diuretics increase sodium excretion and urine output. They do not affect the mortality rate for ARF but may facilitate the treatment of patients by reverting an oliguric form to a non-oliguric form of ARF.
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PMID:The place of loop diuretics in the treatment of acute and chronic renal failure. 129 11

A 3-year old child was admitted for a third relapse of nephrotic syndrome associated with intracranial hypertension related to dural sinus thrombosis (tomodensitometry). The treatment consisted in the association of low dose heparin and fresh frozen plasma. After a 3 year-follow-up, there was no neurologic sequelae, and the nephrotic syndrome was on complete remission. The radiologic features and the management of sinus thrombosis are discussed.
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PMID:[Intracranial venous sinus thrombosis in nephrotic syndrome]. 133 61


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