Relapse of the nephrotic syndrome in a patient who was in remission for 20 years is discussed. The initial renal biopsy performed 3 years after the onset of the nephrotic syndrome showed focal and segmental glomerulosclerosis (FSGS). The second renal biopsy performed 20 years later was compatible with a diagnosis ...

A 6 year old girl is reported who suffered from dermatitis herpetiformis, nephrotic syndrome and celiac disease. HLA typing in our patient disclosed the HLA antigens B8 and DW3, which are known to be frequently associated with nephrotic syndrome, celiac disease and dermatitis herpetiformis. In the literature six cases have ...

We report a case of IgA nephropathy that presented with nephrotic syndrome without hematuria. Renal biopsy revealed typical features of IgA nephropathy on light microscopy, immunofluorescence microscopy, and electron microscopy. A dramatic response occurred on corticosteroid and immunosuppressive therapy, characterized by clearance of proteinuria and a fall of serum IgA ...

The clinical picture and histopathological findings in 11 infants with infantile nephrotic syndrome are presented. Three infants, who presented in the perinatal period, had congenital nephrotic syndrome (Finnish type); six of eight infants who presented in the 1st yr of life had mesangial sclerosis nephrotic syndrome, while the remaining two ...

We have evaluated lymphocyte subsets both quantitatively and functionally in children with nephrotic syndrome. Ten children had minimal change nephrotic syndrome (MCNS), seven had focal segmental glomerular sclerosis (FSGS) and four had recovered from MCNS. Two patients with FSGS had decreased OKT4+ cells and a decreased T4/T8 ratio. Patients with ...

A case of nephrotic syndrome due to minimal lesion glomerulonephritis associated with Hodgkins disease is described. The course of the nephrotic syndrome was relapsing, preceeding the development of lymphoma by eighteen months. Treatment of this nephrotic syndrome with repeated courses of Prednisone and Cyclophosphamide resulted only in partial improvement of ...

The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes and Fc-mu receptor-bearing cells. It is suggested that a ...

Electron microscopic examination was carried out on 10 glomeruli from 4 infants with congenital nephrotic syndrome of the Finnish type (CNF) and 5 glomeruli from 1 control. The thickness of the peripheral glomerular basement membrane (GBM) and lamina densa were measured. The width of the GBM was thinner in CNF ...

A case of necrotizing angiitis is reported. After acute inflammatory changes had subsided, the patient developed the nephrotic syndrome. Postmortem examination disclosed a unique sclerotic glomerular lesion with deposition of eosinophilic material which contained IgM, IgA, C1, and C3. The lesion was distributed in rather selective areas perfused by some ...

Plasma-mediated inhibition of normal lymphoproliferation is an unexplained immunologic abnormality frequently observed in nephrotic syndrome. Since hyperlipidemia, also common in nephrotic syndrome, has been linked with in vitro and in vivo immunodeficiency in other diseases, we have quantitated plasma-mediated inhibition of lymphoproliferation and related it to the degree of hyperlipidemia ...

Fractional chloride reabsorption in the thick ascending limb of the loop of Henle, measured by clearance techniques, is subnormal in patients with Bartter's syndrome. This defect is a marker for the syndrome and, presumably, is the cause of the supranormal tubular secretion of potassium that characterizes the disorder. The potassium ...

The mitogen response of peripheral blood lymphocytes to phytohaemagglutinin is depressed in rats with nephrotic syndrome induced by the administration of the aminonucleoside of puromycin. The mitogen response is normal after the administration of aminonucleoside of puromycin, but before the nephrotic syndrome develops. Nephrotic syndrome serum depresses the mitogen response ...

An administration of protamine sulfate combined with aminonucleoside of puromycin (AN) could produce in rats severe nephrotic syndrome and histological changes similar to focal glomerular sclerosis more distinctly than AN alone. This may be attributed to the action of protamine which seemed to enhance the effect of AN, causing polyanion ...

In recent years tremendous advances have been made in the clinicohistological correlates of glomerular diseases, as a result of light microscopy and electron microscopy of the kidney, and also immunological evaluation of these diseases. However, current knowledge concerning the immunopathology, pathophysiology, and treatment of most causes of the nephrotic syndrome ...

Giant lymph node hyperplasia (GLH) is an unusual form of benign lymphoid hyperplasia which, although it often occurs in the mediastinum, has not previously been reported to involve the thymus. In this report, a case of GLH with involvement of the thymus and with associated nephrotic syndrome and myelofibrosis is ...

A patient with markedly elevated diaphragm due to massive ascites secondary to nephrotic syndrome demonstrated an intense early diastolic sound with low- and medium-pitch. This abnormal sound coincided closely with the "D" point of the anterior mitral valve echogram. This sound remarkably diminished in intensity during inspiration with lowering of ...

The nephrotic syndrome complicating malignancy is an unusual occurrence. We report here four cases of nephrotic syndrome associated with malignant tumours. Three cases were associated with bronchogenic carcinoma and one with adenocarcinoma of colon. One of the four cases developed nephrotic syndrome before the discovery of bronchogenic carcinoma. All patients ...

A 32-year-old female with a relapsing minimal-change nephrotic syndrome developed a massive pulmonary embolism during the treatment of prednisolone and diuretics. The use of diuretics in addition to a hypercoagulable state associated with nephrotic syndrome per se and corticosteroid was considered to be a direct causative factor for the event. ...

A patient with lymphatic filariasis, developing the nephrotic syndrome is reported. Renal biopsy showed a diffuse mesangial proliferative glomerulonephritis with C3 deposition in the glomeruli. Glomerulonephritis associated with filariasis has been reported previously, however definite identification of filarial antigen in glomerular deposits or of specific antibodies in kidney eluates is ...

Previously we found that alpha 2-acid glycoprotein fraction from urine of patients with the nephrotic syndrome stimulated the lipoprotein lipase reaction in vivo and in vitro. The activator was separated from the alpha 1-acid glycoprotein and identified as a glycosaminoglycan. The studies reported here were undertaken to characterize and quantify ...

Six successive cases of congenital nephrotic syndrome are described. Each one showed flexion deformities of the knees and hips, widely open anterior and posterior fontanelles, and wide separation of the skull sutures. These abnormalities were present not only in cases in which the renal histology was of the microcystic Finnish ...

Kidney biopsies from fourteen patients with the nephrotic syndrome were studied by light, immunofluorescence and electron microscopy. Morphologically, all cases showed moderate diffuse mesangial cell proliferation. In all cases, immunofluorescent microscopy demonstrated diffuse and generalized deposits of IgM as the sole or predominant immunoglobulin. Electron microscopic examination showed electron dense ...

Nephrotic syndrome has been observed in association with different types of neoplasia. This appears to be the first report of the occurrence of the nephrotic syndrome due to membrano proliferative glomerulonephritis in association with carcinoma of the oesophagus. Although proteinuria was present before excision of the tumour, the nephrotic phase ...

In children in the acute phase of steady state edema in minimal change nephrotic syndrome renal prostaglandin synthesis as determined by urinary PGE2 and PGE2alpha excretion was evaluated. Age matched healthy children served as controls. In our patients highly significantly increased PGF2alpha excretion accompanied by high urinary aldosterone excretion with ...

In six patients with nephrotic syndrome and in two control subjects freeze-fracture studies of isolated glomeruli obtained by needle biopsies were performed. In the nephrotic patients replicas of freeze-fractured podocytes showed an increased number of cell junctions. These were represented by incomplete occluding junctions and communicating junctions (gap junctions). There ...

Glomerular morphometry was performed on needle biopsy specimens from 53 children with the nephrotic syndrome with minimal change (MC), focal global glomerulosclerosis (FGS) and segmental glomerulosclerosis (SGS). There were significantly fewer epithelial cells and more mesangial cells in SGS compared with MC and FGS. The number of epithelial cells decreased ...

Two patients on long-term lithium therapy developed the nephrotic syndrome. Ultrastructural study of a renal biopsy in each case showed extensive glomerular epithelial cell foot process fusion. Lithium was withdrawn in each case. The nephrotic syndrome cleared without steroid therapy and has not recurred 18 months later in one patient. ...

A young adult patient had an unusual acute idiopathic nephrotic syndrome. This nephrotic syndrome was remarkable for (1) association with acute renal failure and hypertension, (2) finding of minimal glomerular changes with a linear fixation of the anti-human IgG conjugate along the glomerular capillary wall without demonstrable antiglomerular basement membrane ...

Three of five siblings developed a steroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis within a four-month period. Two of the siblings with nephrotic syndrome (Patients 1 and 2) also have sickle cell anemia; the third (Patient 3) carries the thalassemia trait. The dizygotic twin brother of Patient 2 has sickle ...

Acquired deficiencies of functional Hageman factor (factor XII) and prekallikrein, proteins involved in the plasma kinin-generating system, have been previously reported in the nephrotic syndrome. The basis for these changes, however, is not fully understood. We have examined the levels of Hageman factor and prekallikrein by functional and radioimmunoassays in ...

In a sibship of four, Friedreich's ataxia and minimal lesion nephrotic syndrome occurred in two siblings, a third sibling had Friedreich's ataxia, but no evidence of nephrotic syndrome; the fourth sibling had neither condition. The chance of Freidreich's ataxia and minimal lesion nephrotic syndrome occurring in two siblings is small, ...

This case of congenital nephrotic syndrome is not easy to classify. Treatment with usually effective doses of cyclophosphamide and prednisolone was unsuccessful, whereas a treatment with gammaglobulin was concomitant with a decrease in the frequency of infections, improvement of the nephrotic syndrome, growth, psychomotor development and bone maturation. Electron microscopy ...

We have described a case, with renal biopsy findings and serologic studies, of a patient who had the nephrotic syndrome after sustaining multiple bee stings and who demonstrated a favorable response to steroid therapy. Bee stings have previously been implicated in the development of this syndrome, though the reported cases ...

A case report of a female patient with Graves' disease complicated by nephrotic syndrome with high LATS activity in urinary gamma-globulin is presented. When in the hyperthyroid state with high LATS activity in the serum, she was treated with antithyroid drugs, excess iodine, and finally radioisotopes. Mild hypothyroidism occurred transiently ...

A 27-year old man with a long history of ulcerative colitis developed a nephrotic syndrome. Renal biopsy revealed a minimal change lesion and further evaluation revealed that the patient had colonic carcinoma. Total colectomy and a course of prednisone were followed by a complete and lasting remission of the nephrotic ...

The excretion of protein and cholesterol in 24 h urine was measured in 42 patients with the nephrotic syndrome. The finding of a positive correlation (r = 0.76, p < 0.01) between urinary cholesterol and urinary protein would be compatible with an enhanced glomerular filtration of plasma lipoproteins as the ...

Plasma procoagulant activities of factors IX, VIII, VII, IX, and V were determined in 21 adult patients with nephrotic syndrome of diverse etiologies. The studies were performed during the initial presentation and prior to institution of any therapy. Procoagulant activities of all these coagulation factors were found to be markedly ...

Two brothers with nephrotic syndrome are reported. Their parents were first cousins. The nephrotic syndrome developed at the age of 14 and 15 years and was resistant to the treatment with corticosteroid. The elder patient fell into renal failure about 2-1/2 years after the development of nephrotic syndrome, and the ...

Seven patients with slight but definite proliferative changes in the glomeruli achieved complete remission of the nephrotic syndrome when treated with prednisone. The patterns of response and, in three cases, repeated relapse and response suggested that this was more than a coincidental spontaneous cure. A controlled trial of corticosteroids in ...

The clinical and bioptic aspects of 211 cases of primary mesangioproliferative glomerulonephritis with urinary abnormalities lasting mre than 1 year were reviewed. We observed the following clinical syndromes: 1) Persistent proteinuria, isolated or with microhematuria: - a) with latent onset: 39% with hypertension (H); 10% with renal failure (RF); - ...

A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid ...

Patients with the nephrotic syndrome have low blood levels of 25-hydroxyvitamin D3 (25OHD3) due to urinary losses of the sterol. It is not known whether supplementation of this metabolite could raise its blood levels in these patients. The changes in the plasma levels of 25OHD3 and its kinetic behavior were ...

Although the nephrotic syndrome has been reported in association with a wide variety of neoplastic diseases, it has only rarely been noted as a complication of breast carcinoma. We describe a patient who presented with the nephrotic syndrome and who was subsequently found to have carcinoma of the breast. The ...

A 48-year-old male presented with the nephrotic syndrome 3 years after resection and irradiation of a thymoma. Renal biopsy revealed membranous glomerulonephritis by electron microscopy. IgA, IgG, and C3 deposition were present in the glomerular basement membrane by immunofluorescence. There was no evidence of other autoimmune diseases or causes of ...

Gastrointestinal protein loss was studied by intravenous application of 51Cr-albumin and the measurement of fecal excretion of 51Cr. As compared to controls, fecal excretion of 51Cr was largely increased in 28 patients with nephrotic syndrome due to glomerular disease and in 11 patients with hypoproteinemia in the course of different ...

In 20 children with idiopathic nephrotic syndrome, the morphological changes in the glomeruli were compared with the index of selectivity of proteinuria. For the determination of the index of selectivity, the clearances of IgG and transferrin were used. A high correlation was found between the morphological changes and the index ...

A patient with nephrotic syndrome was found to have amyloidosis secondary to an otherwise asymptomatic Crohn's disease. Resection of a major portion of the affected bowel and long-term colchicine therapy were followed by a complete clinical remission of the nephrotic syndrome, most probably due to a significant resolution of amyloidosis. ...

Micropuncture studies in rats and dogs have provided evidence for a cause-and-effect relationship between peritubular protein concentration and proximal tubular reabsorption (PTR). If this effect is obtained in man, hypoalbuminemia in nephrotic syndrome should lead to a fall in PTR. Sodium excretion, however, is very low in nephrotic patients; but ...

Cardiovascular mortality and morbidity were assessed, after a mean follow-up period of 5 years, in an unselected series of 159 adults presenting with the nephrotic syndrome between 1972 and 1975. 60% of the deaths were attributed to terminal renal failure, and the incidence of deaths from ischaemic heart-disease (IHD) was ...

We report 2 cases of cystinuria in which a nephrotic syndrome developed during treatment with alpha-mercaptopropionylglycine. This syndrome resolved after withdrawal of the drug and it did not recur when the alpha-mercaptopropionylglycine was given again in lower doses. The hypothesis is made that the nephrotic syndrome was dose-related. The alpha-mercaptopropionylglycine ...