We describe a patient with nephrotic syndrome due to focal-segmental glomerulosclerosis, occurring 3 years after thymectomy and myasthenia gravis. Nine other cases of nephrotic syndrome associated with thymoma and myasthenia gravis reported in the literature are reviewed. The nephrotic syndrome may be due to T cell dysfunction associated with thymoma; ...

Nephrotic syndrome is characterized by water and sodium retention, which leads to edema formation. The nonosmotic stimulation of arginine vasopressin (AVP) release from the pituitary gland has been implicated to be one of the important factors of abnormal water retention in patients with nephrotic syndrome. It is not known, however, ...

A 52-year-old woman was diagnosed as having cerebellar ataxia, hypogonadotropic hypogonadism and retinochoroidal degeneration, the so-called, "Boucher-Neuhauser" syndrome proposed by Limber et al (Am J Med Genet 33:409, 1989). In addition, laboratory findings showed the elevation of serum calcium (Ca) levels, low urinary Ca excretion, and exaggerated reabsorption of filtrated ...

A case of recurrent Cushing's disease with nephrotic syndrome due to membranoproliferative glomerulonephritis (MPGN) is presented. Functional pituitary adenoma recurred 6 years after transsphenoidal pituitary adenomectomy. Due to infiltration into the surrounding tissues, transcranial surgery was performed. However, this failed to induce a remission and thus gamma knife therapy was ...

We report the case of a 33-year-old Japanese male who presented with thrombocytosis, lower limb edema, severe polyneuropathy with elevated cerebrospinal fluid (CSF) protein level and serum IgA lambda monoclonal component, fulfilling the manifestations of Crow-Fukase syndrome. A high level of soluble interleukin-6 receptor in the CSF was also found, ...

A 14-year-old girl with sickle cell disease and nephrotic syndrome developed bone pain, followed by pulmonary edema, seizures, coma, and bilateral flaccid paralysis. Fat embolism syndrome was diagnosed by cranial magnetic resonance imaging and an exchange transfusion was performed. Within 3 months, all symptoms had resolved. It is concluded that ...

Due to the concern that adolescents presenting with nephrotic syndrome are less likely to have minimal change disease than younger children, pediatric nephrologists have tended toward renal biopsy-tailored treatment rather than corticosteroid use in this population. The need for biopsy prior to treatment of nephrotic syndrome in adults has been ...

A 19-year-old male developed nephrotic syndrome during the course of chronic osteomyelitis complicating a traumatic suppurative arthritis of the knee. Renal biopsy revealed severe mesangial proliferative glomerulonephritis, and immunofluorescent microscopy demonstrated the presence of IgA. Nephrotic syndrome remitted during the treatment for chronic osteomyelitis, suggesting a close association of the ...

A 67-year-old woman, who had been diagnosed with classical rheumatoid arthritis (RA), was admitted to our hospital because of massive proteinuria. Biopsy of the kidney revealed deposition of amyloid fibrils in the subepithelial and subendothelial spaces of the glomerular capillary walls. Though the treatment with prednisolone and dipyridamole against nephrotic ...

We present a case of nephrotic syndrome, associated with small cell lung carcinoma. Renal biopsy revealed membranous glomerulonephritis, probably due to immune complex deposition with tumour antigen. Complete remission of the small cell lung carcinoma after chemotherapy was followed by regression of the nephrotic syndrome. This regression persisted even when ...

Congenital nephrotic syndrome is an uncommon disease with variable etiology, course and prognosis; its association with microcephaly is even more unusual. A case is reported here of congenital nephrotic syndrome because of focal glomerulosclerosis in a three-month-old female infant with microcephaly since birth. There were no known renal diseases nor ...

A 7-year-old boy with a 5-year history of steroid-unresponsive nephrotic syndrome due to minimal change disease presented with acute myocardial infarction. Angiography was suggestive of a dissected atherosclerotic plaque at the initial and mid portions of the right coronary artery, as well as a lesion in the mid portion of ...

The Galloway-Mowat syndrome, a rare inherited disorder, is characterized by congenital microcephaly with hypotonia and developmental delay, often hiatus hernia, and nephrotic syndrome manifested in infancy or in early childhood. The glomerular lesion has been poorly characterized in the few previous reports of this syndrome. We studied three siblings with ...

The association of Hodgkin's disease and minimal-change nephrotic syndrome is well established. We describe a gentleman who developed Hodgkin's disease that was treated successfully. Two years later, he developed minimal-change nephrotic syndrome that responded to steroids. Over the next 9 years, the patient experienced two episodes of nephrotic syndrome due ...

In order to clarify the T-cell abnormalities present in minimal-change nephrotic syndrome (MCNS), lymphocyte subsets were studied using two-color flow cytometry in children with MCNS and nephrotic syndrome caused by other types of glomerular disease, and normal healthy children. In MCNS, CD4+Leu8+ (suppressor-inducer) and CD3+CD25+ (IL-2 receptor positive T) cells ...

Nephrotic syndrome, although rare, is recognized as one of the paraneoplastic syndromes. A patient with documented Hodgkin's disease showed increased uptake of Ga-67 in the cortex of both kidneys in addition to multiple sites of lymphomatous involvement. The patient was not receiving chemotherapy or any nephrotoxic drugs, and no other ...

Three sibs born to consanguineous parents had congenital nephrotic syndrome, microcephaly, and psychomotor retardation. Pathology of the kidneys showed diffuse mesangial sclerosis with deposits of IgG and C3 in the mesangium and glomerular basement membranes. All three children died before the age of 3 years. Of 19 published cases of ...

A 22-month-old girl with nephrotic syndrome and microcephaly is described. She had dysmorphic facies and psychomotor retardation. Her parents were first-degree relatives and one of her siblings had died with nephrotic syndrome and renal failure in infancy. An autosomal recessive inheritance is suggested. The diagnosis of this rare combination is ...

Congenital nephrotic syndrome of the Finnish type (CNF) is an autosomal recessive disease with an incidence of 1 in 8000 in Finland. CNF is characterized by massive proteinuria and nephrotic syndrome at birth. In a recent report, deregulation of expression of the gene coding for the Pax-2 DNA-binding protein was ...

The nephrotic syndrome has long been recognized as a hypercoagulable state. Arterial thrombosis is a rare complication of the syndrome. Diuretics and steroids, standard treatment for exacerbations, have been implicated as contributing to the development of arterial thrombosis. The authors present the pathologic, clinical, and radiologic findings of a patient ...

We report on a 16-year-old girl with spondyloepiphyseal dysplasia, nephrotic syndrome, lymphopenia, and signs of defective cellular immunity. The manifestations are very similar to those reported by Spranger et al. [1991: J. Pediatr 119: 64-72] as Schimke immunoosseous dysplasia, except for age of onset. In Schimke immunoosseous dysplasia, growth retardations ...

Agnogenic myeloid metaplasia (AMM), a myeloproliferative disorder of clonal origin, can affect the kidneys via several mechanisms. Associated disorders include asymptomatic interstitial extramedullary hematopoiesis, obstructive uropathy and nephrolithiasis. Currently, there are no descriptions of the nephrotic syndrome associated with AMM. We present a case of AMM associated with the nephrotic ...

We report a 49-year-old Japanese male with nephrotic syndrome associated with Kimura's disease. Renal biopsy revealed diffuse podocytic detachment from the glomerular basement membrane (GBM). He had an episode of nephrotic syndrome when complete remission was induced with steroid therapy six years prior to the present admission. However, complete remission ...

Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Renal disease is rarely associated with it. We describe a young non-Oriental male with KD and relapsing steroid-responsive minimal-change nephrotic syndrome. This case of KD ...

Nephrotic syndrome is associated with thromboembolic disease due to a variety of factors. In addition, therapy with anticoagulants is often met with poor response, to warfarin for example, which occurs due to factors such as reduced serum albumin and binding sites, increased free warfarin with rapid metabolism and clearance, and ...

Two white female infants were seen with congenital nephrotic syndrome at age 6 weeks and 3 months, respectively. Both had hypocomplementemia, elevated antinuclear antibody and anti-double-stranded DNA titers, and diffuse proliferative glomerulonephritis with positive immunofluorescence in their initial renal biopsy samples. Although uncommon, infantile systemic lupus erythematosus should be considered ...

We describe our experience with spontaneous arterial thrombosis (AT) in two men with nephrotic syndrome secondary to minimal change glomerulonephritis. Arterial thrombosis developed shortly after a nephrotic crisis in each case. Serum antithrombin III, protein C and protein S levels were normal in both patients. The two cases emphasize the ...

The association of a spondyloepiphyseal dysplasia and disproportionate short stature with focal glomerular sclerosis is reported in two girls. Renal disease manifested by proteinuria at the age of 2.5 and 11 years, leading to treatment-resistant nephrotic syndrome over 15 and 45 months, respectively. One patient went into end-stage renal failure ...

We present here the first described case of Nail-patella syndrome (NPS) and pregnancy. Complications occurred during the pregnancy with the onset of preeclampsia at 22 weeks, leading to intrauterine fetal death at 24 weeks. The nephropathy of the NPS began clinically during the course of gestation. Postpartum, it persisted as ...

An unusual case of reversible reactive amyloidosis (AA) is described. A patient in the course of lobar pneumonia developed acute transient nephrotic syndrome and renal failure. Renal and liver biopsy showed amyloidosis and positive immunohistochemistry stains for amyloid A protein. The nephrotic syndrome resolved completely following 6 months of colchicine ...

We report a 14 year old Indian-Muslim girl who developed a fulminant, disseminated and fatal varicella infection while receiving steroids for nephrotic syndrome. The terminal phase of her illness was complicated by a bleeding dyscrasia and circulatory collapse. Varicella infection in healthy children is a benign disease. However in neonates ...

In the dog, massive proteinuria and/or the nephrotic syndrome have been commonly associated with renal amyloidosis and membranous glomerulonephritis. Primary glomerulopathies associated with the nephrotic syndrome in man also include minimal change nephrotic syndrome and focal glomerular sclerosis. A 4-year-old Collie dog is described with clinical, histological, immunohistological, and ultrastructural ...

Urinary oligosaccharides from nephrotic syndrome patients were investigated by Bio-Gel P-4 column chromatography and methylation analysis to clarify the electrostatic defects and size barrier defects in the glomerular basement membrane of nephrotic syndrome patients. The levels of urinary oligosaccharides (nmol/mg creatinine) in nephrotic syndrome patients were about 25 times the ...

In order to examine the changes in charge of the glomerular basement membrane (GBM) in nephrotic syndrome, anionic sites in the GBM were studied quantitatively. Renal biopsy specimens were obtained from 5 children with minimal change nephrotic syndrome (MCNS) and 5 with nephrotic syndrome and focal segmental glomerulosclerosis (FSGS). Biopsy ...

Most cases of spontaneous bacterial peritonitis (SBP) in association with nephrotic syndrome are children. The complication of SBP in adults with nephrotic syndrome is extremely rare. Herein, we report a 25-year-old man with nephrotic syndrome and chronic renal failure who suffered from SBP. Citrobacter freundii was isolated from ascites. Irreversible ...

Two Indian male children with infantile-onset heavy proteinuria (with nephrotic syndrome in 1) had thickening of the glomerular basement membrane with splitting and basket-weave appearance of lamina densa on electron microscopic evaluation of kidney tissue (like Alport's syndrome), with normal light microscopic findings and negative immunofluorescence. The proteinuria was non-familial ...

A 27-year-old man, who had been on steroid therapy for 2 months for his nephrotic syndrome, suddenly developed intra-abdominal bile collection (biloma). He had no previous history of abdominal surgery, trauma, or any disease of the hepatobiliary system. The cause of the biloma formation was due, probably, to cholecystitis in ...

The cardinal features of the nephrotic syndrome are albuminuria, hypoalbuminemia, and edema. Traditionally, albuminuria was thought to be responsible primarily for the development of hypoalbuminemia. A decreased plasma-albumin concentration accompanied by a decreased plasma-oncotic pressure was thought responsible for the development of edema and secondary salt retention by the kidney. ...

The pathophysiology, clinical features, complications, and pharmacologic management of adult idiopathic nephrotic syndrome are reviewed. Loss of plasma proteins in the urine is the primary process leading to the nephrotic syndrome, which is characterized by hypoalbuminemia, hyperlipidemia, and edema. The four principal causes, or subclasses, of adult idiopathic nephrotic syndrome ...

Pulmonary embolism is a rare complication of nephrotic syndrome. We report the case history of a 30 year old man who presented with nephrotic syndrome and became acutely dyspnoeic with cyanosis and peripheral circulatory failure while on treatment. The clinical and ECG findings were strongly suggestive of acute pulmonary embolism.

Abdominal complications were detected and investigated in 19 (10%) of 191 children with nephrotic syndrome who experienced 35 episodes of these complications. Fourteen children were Indian with steroid-responsive nephrotic syndrome, and 5 were black, of whom 4 had membranous nephropathy and 1 focal proliferative nephritis. All had clinical features of ...

Proteinuria in children with idiopathic nephrotic syndrome is secondary to a loss of charge selectivity of the glomerular basement membrane. Loss of anionic charges may be secondary to a defect of heparan sulfate proteoglycans, which is also found in the congenital nephrotic syndrome, or to cationic proteins, which neutralize the ...

Varying components of the syndrome of human immunodeficiency virus nephropathy (HIVN) have been described, the most pertinent including proteinuria/nephrotic syndrome, progressive azotemia, normal blood pressure, enlarged and hyperechoic kidneys, rapid progression to end-stage renal disease (ESRD), and no response to treatment regimens. The diagnosis of HIVN requires identification of excessive ...

The large protein excretion of nephrotic syndrome leads to severe hypoalbuminemia and massive edema. The ensuing hyperlipidemia may not respond to any treatment unless protein losses are corrected. Urinary excretion of clotting factors due to nonselective proteinuria explains, at least in part, the development of a procoagulable state. We report ...

A male patient with hyperimmunoglobulin E syndrome is described. Recurrent lymphadenitis and cutaneous staphylococcal abscesses were resistant to various antibiotics, and chemotaxis and hydrogen peroxide production of polymorphonuclear leukocytes were impaired. Following trimethoprim-sulfamethoxazole therapy, he was free from the above infections, and impaired polymorphonuclear leukocyte functions recovered and serum IgE ...

Recent reports suggest that cyclosporin A is beneficial in inducing remission of idiopathic nephrotic syndrome. Nephrotic syndrome is seen in 10-30% of patients with rapidly progressive glomerulonephritis. We report a case of a 69-year-old man with nephrotic syndrome, associated with idiopathic rapidly progressive glomerulonephritis, who was treated initially with corticosteroid ...

A 54-year-old woman with nephrotic syndrome underwent renal biopsy. By light microscopy, the glomerular capillary lumen was remarkably narrowed because of diffuse accumulation of Periodic acid Shiff (PAS) positive material along the glomerular capillary wall. By electron microscopy, collagenous fibers were observed in the mesangium and subendothelial area. The fibrous ...

A patient developed relapsing inflammatory demyelinating polyradiculoneuropathy associated with nephrotic syndrome. Renal biopsy showed focal-segmental glomerulosclerosis. The review of the literature disclosed that glomerulonephritis with and without nephrotic syndrome seems to be not uncommon in inflammatory demyelinating polyradiculoneuropathy, such as Guillain-Barré syndrome. membranous glomerulonephritis is the most frequent histologic diagnosis ...

A 26-year-old male presented with oedema, massive albuminuria and microscopic haematuria. Kidney biopsy revealed enlarged cellular glomeruli infiltrated by polymorphs and eosinophils with focal fibrin deposits along the basement membrane. Microfilariae were seen in the lumen of few glomerular capillaries. Antistreptolysin titre was negative. The absence of other aetiological factors ...

Simultaneous occurrence of IgA nephropathy and sarcoidosis is described in a 42-year-old Asian female, who earlier had a steroid-resistant, no light microscopy change nephrotic syndrome that resolved spontaneously. Occurrence of three, apparently unrelated conditions in this case may be fortuitous, or causally related. We discuss the possible causal relationship amongst ...