We present a 30-year-old male patient who was initially diagnosed as minimal change nephrotic syndrome, 5 years later, the patient developed a localized form of idiopathic retroperitoneal fibrosis (IRF). An elevated ESR and concomitant nephrotic syndrome in the patient suggested the immunologic nature of IRF, IRF has been reported in ...

This case illustrates the utility of all modalities of the renal biopsy in arriving at a correct diagnosis in an adult patient with nephrotic syndrome. Unlike the clinical situation in children, where minimal change disease is presumed to underlie the nephrotic syndrome unless the patient shows steroid resistance, the list ...

A patient with massive edema caused by nephrotic syndrome showed a nine-point decline in hematocrit after an otherwise uneventful renal biopsy. Investigation showed no evidence of hemorrhage. The decrease in hematocrit was shown to be dilutional, occurring as a result of reabsorption of edema fluid into the vascular space that ...

Thrombotic phenomena are well-recognized complications of nephrotic syndrome attributable to loss of intermediate-sized antithrombotic proteins in the urine, resulting in a hypercoaguable state. As such, nephrotic syndrome may be associated with a reduction in circulating antithrombin III and free protein S levels. Associated spontaneous thrombotic complications are generally venous in ...

We describe a male infant with early myoclonic encephalopathy (EME) associated with the congenital nephrotic syndrome, microcephaly, multiple minor anomalies, and cerebellar hypoplasia. He had erratic and massive myoclonus, and partial seizures from the neonatal period. Electroencephalography showed the so-called suppression-burst pattern. He died of disseminated intravascular coagulation caused by ...

This report documents the occurrence of a nephrotic syndrome in five children with Hodgkin disease. In two cases the nephrotic syndrome predated the diagnosis of lymphoma by 6 months and 12 months respectively, while in the other three, the two disorders occurred simultaneously. The nephrotic syndrome resolved in four cases ...

We report a 40-month-old black male with nephrotic syndrome who developed chylothorax associated with superior vena cava (SVC) thrombosis. To our knowledge, this is the third reported case of spontaneous SVC thrombosis in a nephrotic patient and the first in which chylothorax was also present. Ultrasonography of the pleura and ...

Malignancy is a cause of membranous glomerulonephritis. We report a patient with an otherwise asymptomatic squamous cell carcinoma of the esophagus whose presenting manifestation was membranous glomerulonephritis and nephrotic syndrome. Perinuclear anti-neutrophil cytoplasmic antibody was positive. This is the first reported case of perinuclear anti-neutrophil cytoplasmic antibody associated with paraneoplastic ...

Acute renal failure (ARF) associated with idiopathic nephrotic syndrome has been reported in adults with advanced age but is a rare event in children. We have reviewed the literature on this subject and report an additional pediatric case. The pathogenetic mechanisms which may lead to ARF during the course of ...

Tertian malaria is endemic in south-eastern Anatolia. As in Europe and America, in south-eastern Anatolia, an etiological agent is seldom identified in nephrotic syndrome. Two patients with Plasmodium vivax and nephrotic syndrome are described here. The possible relationship between Plasmodium vivax and nephrotic syndrome should be explored in children in ...

Nephrotic syndrome may be associated not only with malignant neoplasia, but also rarely with benign solid tumours. Here we report a case of a young woman suffering from nephrotic syndrome, histologically classed as a minimal change glomerular lesion, associated with a posterior mediastinal neurilemmoma (ancient neurilemmoma). Surgical removal of the ...

We report the first case of the use of ibuprofen for the management of steroid-resistant nephrotic syndrome. A 41 year-old man with nephrotic syndrome, secondary to focal segmental glomerulosclerosis, had persistent nephrotic range proteinuria despite aggressive treatment with steroids and cyclophosphamide. His steroid-resistant nephrotic syndrome resolved rapidly when he was ...

The congenital nephrotic syndrome of the Finnish type (CNF) is a rare autosomal recessive disease with proteinuria starting already in utero, prematurity and nephrotic syndrome developing within the first weeks of life. The basic defect of this disease is unknown but has been suggested to be restricted to the kidney ...

Nephrotic syndrome has been recently shown to cause erythropoietin (EPO) deficiency in humans and experimental models. However, efficacy and safety of recombinant EPO (rEPO) in the treatment of the associated anemia has not been previously investigated. We report a patient with nephrotic syndrome and only moderately impaired renal function. This ...

Nephrotic syndrome presenting in the 1st year of life is often associated with a very poor prognosis for normal renal function. A small proportion of patients, particularly boys, presenting after the first 3 months of life with idiopathic-type, steroid-sensitive nephrosis, have a much better prognosis and may achieve sustained remission. ...

A review is presented of 164 nephrotic patients who were older than 60 years at the onset of their nephrotic syndrome, together with a review of more than 1,000 patients from the literature. The nephrotic syndrome in elderly patients is as common as in younger adults, but is often misdiagnosed ...

We describe a patient with acute myeloblastic leukemia (AML) who developed nephrotic syndrome after receiving several courses of chemotherapy, including macrophage-colony-stimulating factor (M-CSF). At the onset of nephrotic syndrome, the patient remained in a hematological remission. A renal biopsy showed diffuse mesangial proliferation with marked glomerular infiltration of macrophages and ...

We describe the first reported case of familial hemophagocytic syndrome (FHS) with concurrent minimal change nephrotic syndrome (MCNS). This is a case report of a 30-month-old girl who presented to Children's Memorial Hospital with pancytopenia and heavy proteinuria. This patient presented with anemia, neutropenia, thrombocytopenia, hypertriglyceridemia, and proteinuria. A brother ...

Membranous glomerulonephritis is the most common glomerular disease associated with malignancy, the association of minimal change glomerulopathy with solid tumor is still uncommon. We report a 72-year-old man with nephrotic syndrome due to minimal change glomerular disease; an accurate seek of underlying malignancy revealed a cecum adenocarcinoma. We had a ...

Chronic lymphocytic leukemia is a common disease in the elderly but is rarely associated with a nephrotic syndrome. The rarity of this association suggests that leukemic cells may have certain properties or features that may lead to the development of glomerulonephritis. Effective medical treatment of the leukemia may not necessarily ...

OBJECTIVE: We tested the hypothesis that nitric oxide synthesis by the kidney is increased in children with primary nephrotic syndrome. METHODS: We examined the urinary excretion of nitrite, a stable metabolite of nitric oxide, using the Griess reaction, in children with nephrotic syndrome. RESULTS: In comparison with healthy children, patients ...

A 55-year-old man with chronic inflammatory demyelinating polyradiculoneuropathy developed the nephrotic syndrome. Renal biopsy showed stage I membranous glomerulonephritis. Review of the literature revealed the association of these two rare syndromes, considered to be due to immunologic dysfunction, in two other cases, as well as several cases of the acute ...

Various malignancies are associated with the paraneoplastic evolution of the nephrotic syndrome. Renal biopsy in these instances frequently shows membranous glomerulonephritis. We describe a patient who had metastatic bronchial carcinoid tumor with development of microscopic hematuria and subsequent nephrotic syndrome in conjunction with another paraneoplastic process, a malignancy-related neuropathy. A ...

Lipoprotein(a) [Lp(a)] is a plasma lipoprotein whose structure and composition closely resemble that of low-density lipoproteins, but contains an additional protein called apolipoprotein(a) [apo(a)]. Factors which modulate plasma Lp(a) concentrations are poorly understood. The influence of nephrotic syndrome on Lp(a) levels was investigated in 103 patients with nephrotic syndrome: 72 ...

The Mpv17 mouse strain is a recessive transgenic mouse mutant that develops glomerulosclerosis and nephrotic syndrome at a young age. The phenotype results from a loss of function of a gene coding for a hydrophobic peroxisomal protein of 176 amino acids of 20 kDa following its destruction by retroviral integration. ...

A 62-year-old woman presented with nephrotic syndrome and severe anemia although the renal function was not impaired. Renal biopsy revealed the histology of membranoproliferative glomerulonephritis, and the proteinuria was resistant to steroid therapy. Iron deficiency, bleeding and other causes of anemia were ruled out, however, her serum erythropoietin level was ...

Between April 1982 and September 1994, 167 renal biopsies were performed in 167 children at King Khalid University Hospital, Riyadh, Saudi Arabia. The data were analysed to show a correlation between clinical presentation and histological findings. Nephrotic syndrome was the most common indication for renal biopsy, accounting for 77% of ...

Systemic amyloidosis normally has a dismal prognosis. However, there are several case reports of protracted survival, usually as a response to measures designed to retard the further deposition of amyloid fibrils. In AA amyloid, most commonly associated with inflammatory rheumatological, bowel, and chest diseases, such interventions have had some success, ...

Type I carbohydrate-deficient glycoprotein (CDG) syndrome is a genetic multisystem disorder generally without overt renal problems. We report a neonate with neurological abnormalities and congenital nephrotic syndrome of diffuse mesangial sclerosis type. Serum transferrin isoelectric focusing showed the typical abnormalities of type I CDG syndrome. Normal transferrin focusing findings in ...

At autopsy, massive nonamyloid fibrillar deposits, immunoreactive to IgG and kappa light chain, were found in glomeruli, liver, and bone marrow of a 72-year-old woman. The patient suffered from severe nephrotic syndrome, hepatomegaly and cholestasis, normochromic anemia, and IgG kappa monoclonal gammopathy. Fibrillary glomerulopathies, most often denoted as fibrillary glomerulonephritis ...

Membranous nephropathy has been described in association with many malignancies including various lymphomas. However, it has not been previously described as a complication of benign solid adenolymphoma of the parotid, also called Warthin's tumour. We describe a patient who presented with an adenolymphoma of the parotid, and developed a severe ...

Secondary or reactive (AA) amyloidosis is a well-known complication of certain rheumatic diseases, particularly rheumatoid arthritis (RA). This case report describes a patient with RA complicated by amyloidosis and the nephrotic syndrome, which regressed after treatment with azathioprine. The AA amyloidosis was documented by renal and lymph node biopsies and ...

We report a 2-year-old girl with nephrotic syndrome, microcephaly, seizures and psychomotor retardation. Histological studies of a renal biopsy revealed focal glomerular sclerosis with mesangiolysis and capillary microaneurysms. Dysmorphic features were remarkable: abnormal-shaped skull, coarse hair, narrow forehead, large low-set ears, almond-shaped eyes, low nasal bridge, pinched nose, thin lips ...

A 9-year-old boy with typical features of congenital erythropoietic porphyria who had received more than 50 blood transfusions developed the steroid-resistant nephrotic syndrome in the presence of normal glomerular function and glucosuria. Renal biopsy showed focal segmental glomerulosclerosis and widespread iron deposits. Magnetic resonance scanning revealed advanced siderosis of liver ...

A female, term neonate presented with generalized edema, heavy proteinuria, hypoalbuminemia and hyperlipidemia in the second week of life. The clinical and laboratory features were compatible with the diagnosis of congenital nephrotic syndrome. Treatment included albumin infusion, empirical penicillin, steroid and continuous arterio-venous hemofiltration. Intestinal perforation developed at the 19th ...

The efficacy of cyclosporin-A, a potent immunosuppressive agent, has not been clearly proven in the management of childhood nephrotic syndrome. The occurrence of early relapse as the dose of cyclosporin is tapered or as soon as the drug is stopped and the potential nephrotoxic effects of prolonged treatment have caused ...

A study was conducted in filarial endemic area for diagnosis of occult filariasis in various clinical conditions in children. Thirty-five age- and sex-matched controls (endemic-15, non-endemic-10, disease control-10), 16 classical lymphatic filariasis, and 92 occult filariasis (clinical conditions which fall in the spectrum of occult filariasis and suspected to be ...

From 1981 to 1987, renal specimens obtained from 91 children with idiopathic nephrotic syndrome were categorized into 5 histologic subgroups, according to the WHO classification. We analyzed differences in the ratios of the size of the mesangial stalks and attached capillary, including the Bowman's space between the capillary tufts (E), ...

A 16-year-old patient with relapsing nephrotic syndrome had three episodes of deep vein thrombosis after the age of 15 years. An autosomal inherited genetic abnormality of clotting factor V, resistance to activated protein C, was found. The clinical course showed that this resistance and the nephrotic syndrome potentiated each other ...

Decrease of the anionic charge of the glomerular basement membrane and especially the reduced amount of heparan sulphate proteoglycan in the lamina rara externa has been suggested to be the basic pathogenetic defect in congenital nephrotic syndrome. In the present study the anionic charge of glomeruli was examined in the ...

We present a case of diabetic nephrotic syndrome complicated with cerebral embolism in whom two intracardiac thrombi were found in both right and left ventricles without obvious abnormality echocardiographically. Both thrombi regressed after anticoagulant therapy. This represents an intracardiac thrombotic complication of nephrotic syndrome, presumably with hypercoagulable state as the ...

Although lipoprotein abnormalities of the nephrotic syndrome are assumed to be related to the presence of proteinuria, this topic has not been investigated extensively. We measured lipoproteins from 19 nonuremic patients during and after remission of the nephrotic syndrome in an effort to determine the extent of their putative atherogenicity. ...

While renal tissue from one fetus and a newborn with congenital nephrotic syndrome, Finnish type (FCNS), showed a normal basement membrane (BM) localization and composition, in another type of congenital nephrotic syndrome, diffuse mesangial sclerosis (DMS), most glomeruli demonstrated a completely disorganized matrix. In the latter, hyalinized glomerular segments were ...

A 15 month old boy with typical features of congenital nephrotic syndrome (CNS) is reported, who in addition to the renal pathology had an associated clinical hypothyroidism with low levels of total and free thyroxine and triiodothyronine and an elevated serum TSH. Improvement in the physical parameters and mental status ...

We present three children with congenital nephrotic syndrome and previously undescribed facial features. These include: (1) small mouth (intercommissural distance < 3rd percentile); (2) tented upper lip; (3) small nose (interalar distance -2 SD below mean); (4) fullness of cheeks giving a 'jowly' appearance; (5) overhanging outer thirds of upper ...

Renal disease is often accompanied by urinary loss of proteins. When this loss of proteins exceeds the arbitrary value of 3 to 5 grams per day, in the presence of decreased levels of serum albumin, increased serum cholesterol and edema, one defines this clinical entity as nephrotic syndrome. Causes of ...

A case of systemic lupus erythematosus (SLE) associated with minimal-change nephrotic syndrome (MCNS) is described. A 41-year-old woman with SLE presented with symptoms of nephrotic syndrome. Renal biopsy revealed minor glomerular abnormalities without the deposition of immune complexes. The initial heavy proteinuria promptly decreased after the prednisolone dosage was increased ...

The histological features of haemorrhoids are detailed in this study with special attention to the mucosal changes. These include significant crypt distortion, with stromal hyperplasia which bear a remarkable resemblance to the changes seen in mucosal prolapse syndrome. We propose that the mucosal prolapse syndrome should include haemorrhoids as part ...

Several types of autoimmune complications of chronic lymphocytic leukemia (CLL) have been previously reported. However, the tendency to develop autoantibodies is usually restricted to the hematopoietic system. We report a 68-year-old man who had developed dermatomyositis after ten years of chemotherapy for CLL. He also had secondary nephrotic syndrome at ...

Abdominal migraine and cyclic vomiting are both self-limiting episodic conditions of children, with periods of complete normality between episodes. The clinical features of both syndromes show considerable similarity, and resemble those found in association with migraine headaches. It is proposed that cyclic vomiting is a condition related to migraine.