Eradication of a cause of secondary membranous glomerulopathy might suppress nephrotic syndrome. Here we report a patient with membranous nephropathy (MN), whose proteinuria entered complete remission after treating actinomycosis. A 45-year-old woman presented with nephrotic syndrome and was diagnosed as having MN. Her urine protein excretion was 5.3 g/day and ...

Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF). Galactose has a high affinity for FSPF, and experimental data suggest that it could reduce its activity. We describe the case of a 48-year-old male with a nephrotic ...

Idiopathic nephrotic syndrome is the most frequent glomerular disease that presents during childhood and is mainly due to minimal change nephropathy (MCNS) and focal-segmental glomerulosclerosis (FSGS). Its treatment is still challenging, with up to 50% of the patients who are initially steroid sensitive (usually MCNS) being frequent relapsers and requiring ...

Congenital disorders of glycosylation (CDG) are inborn errors of metabolism presenting with multi-system organ involvement due to defective glycosylation of glycoproteins. We report here a case of microcephaly, hypotonia, seizure disorder and severe developmental delay since infancy in whom screening for CDG with transferring isoelectric focussing (TIEF) revealed a type ...

Primary peritonitis is a well-described infectious complication of nephrotic syndrome. Current data on the true incidence of peritonitis and efficacy of preventive pneumococcal vaccination are not clear in this group of children. In this nationwide study, among a total of 268 patients with an initial diagnosis of steroid sensitive nephrotic ...

Inhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive ...

Idiopathic nephrotic syndrome is the most frequent glomerular disease during childhood. Although immunosuppressive agents are usually effective, some severe cases remain difficult to treat. We describe a female patient with secondary steroid-resistant nephrotic syndrome who no longer responded to conventional treatment. Owing to cyclosporine toxicity, rituximab was administered. Three days ...

Nephrotic syndrome, a primarily paediatric disease, is associated with a high relapse rate. Studies have reported behavioral and psychological difficulties in children with nephrotic syndrome, their caregivers and siblings, a factor that is likely to influence the overall outcome of the disease in an adverse manner. In clinical practice, however, ...

Atypical protein kinase C (aPKC) is a central component of the evolutionarily conserved Par3-Par6-aPKC complex, one of the fundamental regulators of cell polarity. We recently demonstrated that these proteins interact with Neph-nephrin molecules at the slit diaphragm of the glomerular filtration barrier. Here, we report that podocyte-specific deletion of aPKClambda/iota ...

Several recent studies have demonstrated that the slit diaphragm of the glomerular epithelial cell (podocyte) is the structure likely to be the principal barrier in the glomerular capillary wall. Nephrin identified as a gene product mutated in congenital nephrotic syndrome located at the outer leaflet of plasma membranes of the ...

Minimal change nephrotic syndrome has been proposed to be a disorder of T cell dysfunction. It is hypothesized that a circulating factor(s) from activated T cells might alter glomerular permeability to protein. Some studies have provided evidence that up-regulation of interleukin-2 may be involved, not only in the pathophysiology of ...

Congenital nephrotic syndrome of the Finnish type (CNF) is due to NPHS1 mutation and is responsible for a variety of urinary protein losses. We report the case of a 4-month-old girl with a particularly severe form (proteinuria approximately 150 g/l) of CNF. She developed severe non-regenerative anaemia requiring bi-monthly blood ...

<p class="IJHDRabstract" style="margin: 0cm 1cm 6pt;"><span lang="EN-US"><span style="font-size: x-small; font-family: Century Schoolbook;">Nephrotic syndrome is a chronic clinical condition and drugs used in its treatment may result in severe side-effects. Renal transplantation or renal ablation and subsequent chronic dialysis treatment may be the only feasible way to patients. The present article ...

We present the case of a 23-year-old man with steroid-resistant nephrotic syndrome due to minimal change disease who was treated with rituximab. The patient was resistant to conventional therapy. We therefore treated him with a single dose of rituximab (375 mg/m(2)). One month after the administration of rituximab, complete remission ...

BACKGROUND: Nephrotic syndrome is a common childhood renal disorder; the prevalence of Urinary tract infection (UTI) in these patients is high. The increased prevalence of UTI are due to immunoglobulin loss, defective T cell function, presence of ascites and relative malnutrition. OBJECTIVE: The study is to evaluate the prevalence of ...

INTRODUCTION: Risk factors of renal involvement in Henoch-Schonlein nephritis (HSN) have been extensively studied, but their relations with the severity of glomerular lesions at the disease onset are much less known. MATERIALS AND METHODS: Data were collected retrospectively on 45 patients (age range, 2 to 15 years) with HSN to ...

INTRODUCTION: Slit diaphragm and/or podocyte's cytoskeleton alterations are related to proteinuria and nephrotic syndrome. In our population, focal and segmental glomerulosclerosis causing nephrotic syndrome is the more frequent biopsy demonstrated glomerulopathy. Our aim was search for alterations in some slit diaphragm-associated proteins in patients with nephrotic range proteinuria. METHODS: Renal ...

We report a patient with AH amyloidosis associated with lymphoplasmacytic leukemia that has remained in a stable state with a nephrotic syndrome for 17 months since the commencement of cyclic rituximab therapy aimed at suppression of pathogenetic gamma heavy chains. Free light chains in serum and CD20-positive cells in peripheral ...

Focal segmental glomerulosclerosis (FSGS) often leads to refractory nephrotic syndrome (NS). A high level of low-density lipoprotein (LDL) is a risk factor for the progression of NS. An 8-year-old girl presented with severe proteinuria refractory to steroid therapy. She was diagnosed with non-IgA diffuse mesangial proliferative glomerulonephritis. Oral prednisolone, methylprednisolone ...

Paraneoplastic glomerulopathies are rare manifestations of neoplastic disease to be distinguished from iatrogenic renal damage. Solid tumors are preferentially associated with membranous nephropathy, whereas Hodgkin's lymphomas are associated with minimal change disease. We report a 63-year-old Caucasian male diagnosed with a mesothelioma of the tunica vaginalis testis who, secondary to ...

The recent discovery of genes involved in familial forms of nephrotic syndrome represents a break-through in nephrology. To date, 15 genes have been characterized and several new loci have been identified, with a potential for discovery of new genes. Overall, these genes account for a large fraction of familial forms ...

BACKGROUND/AIMS: The present study evaluated the clinical efficacy and pharmacokinetics of microemulsion cyclosporine A (ME-CyA) with modification from postprandial to preprandial administration in adult patients with refractory nephrotic syndrome. METHODS: We investigated 19 patients with refractory nephrotic syndrome who had been switched from the postprandial administration of ME-CyA to preprandial ...

BACKGROUND: Kimura disease, often accompanied by nephrotic syndrome, is a rare, chronic inflammatory disorder of unknown cause. In this report, the clinical and histopathological characteristics of 20 Chinese patients with Kimura disease-associated nephrotic syndrome were retrospectively evaluated. METHODS: We report a case of Kimura disease that was diagnosed recently in ...

Nephrotic syndrome presenting in the first year of life is often challenging, with substantial risk of progression to end-stage renal disease (ESRD). Focal segmental glomerulosclerosis (FSGS) comprises up to 20% of biopsy-proven glomerular disease in children and adults. We report on a 9-month-old infant who presented with nephrotic syndrome, hypertension ...

The nephrotic syndrome is an unusual cause of the hypercoaguable state and thromboembolic complications. Here we report the case of a 42-year-old woman with nephrotic syndrome who presented with a pulseless lower extremity and a midpole renal infarct requiring urgent embolectomy of the leg. During her embolic evaluation, she was ...

Congenital nephrotic syndrome is rare and is often found incidentally while investigating other disorders. The diagnosis is made by typical clinical features and laboratory tests in infants < 3 months of age. It may be inherited, sporadic, acquired, or associated with a syndrome. We present the case of a former ...

Overgrowth syndromes such as Beckwith-Wiedemann syndrome, Sotos syndrome, and Weaver syndrome have an increased risk of neoplasia. Two previous cases of neuroblastoma have been reported in children with Weaver syndrome. We present a third description of a patient with Weaver syndrome and neuroblastoma. In a child with phenotypic characteristics consistent ...

Minimal change nephrotic syndrome (MCNS) has been associated with primary immunological disorders, such as lymphoma and thymoma. While several different explanations have been proposed, much of the literature has implicated activated T-lymphocytes in the pathogenesis. We report a patient with minimal change nephrotic syndrome presenting concurrently with thrombocytopenia and anemia, ...

The aim of the study was to present our experience in treating children with genetic forms of nephrotic syndrome and diagnosing these diseases. We retrospectively reviewed the clinical data, mutational analyses, histopathological features, treatment modalities, and outcome of 26 consecutive children (20 families) suffering from congenital and/or steroid-resistant nephrotic syndrome ...

An association between assisted reproduction technologies (ART) and abnormal genomic imprinting in humans has been recognized for several years; however, the magnitude of this risk and the spectrum of imprinting syndromes to which the risk applies remains unknown. Nine human imprinting syndromes have been identified but current evidence links ART ...

Children with nephrotic syndrome (NS) are at risk for sinovenous and arterial thrombosis, uncommon but serious complications of the nephrotic syndrome. Multiple factors are involved in the hypercoagulable state of patients with NS, for instance, enhanced platelet reactivity and deficiency of antithrombin III due to urinary loss of this protein. ...

A 78-year old woman with complicating solitary kidney had nephrotic syndrome. Renal biopsy specimens showed focal segmental glomerulosclerosis (FSGS). First, the patient was treated with angiotensin receptor blocker (ARB) and angiotensin converting enzyme inhibitor (ACEI). Proteinuria decreased from 10 to 6 g/day, but overall the nephrotic syndrome did not improve. ...

BACKGROUND: There is currently little information in literature about the pattern of glomerulonephritides (GN) in adults with nephrotic syndrome in this part of the world, particularly that involving the use of immunofluorescence (IMF) and electron microscopy (EM). A few studies reported are based on light microscopic study alone and hence ...

The aim of this retrospective study was to evaluate the results of the immunosuppressive regiment in managing of IgA nephropathy associated with primary nephrotic syndrome at the Nephrology Clinic, University of Sarajevo Clinics Centre in period of 1997-2007. We studied 19 patients (4 women and 15 men) with idiopathic nephrotic ...

The role of severe a1-antitrypsin (A1AT) deficiency in the predisposition of early-onset pulmonary emphysema and juvenile hepatic cirrhosis is well-established. Associated glomerulonephritis is unusual although it is well-recognized in children and young adults with the severe phenotype. We report the first adult case of A1AT deficiency presenting with nephrotic syndrome ...

Galloway-Mowat syndrome is a rare multisystem genetic disorder with constellation of neurological, skeletal, growth, facial, gastrointestinal and renal abnormalities. This case report describes Galloway-Mowat syndrome in a young boy suffering from congenital microcephaly, developmental delay, seizures and various dysmorphic features in whom nephrotic syndrome became apparent at 5 years of ...

Castleman's disease is a rare atypical lymphoproliferative disorder. Renal manifestations, such as proteinuria, hematuria, and renal dysfunction, are common in Castleman's disease; however, a nephrotic syndrome rarely occurs. We have encountered an unusual case of Castleman's disease of the plasma cell type characterized by nephrotic syndrome because of glomerulopathy mimicking ...

Hereditary forms of childhood nephrotic syndrome (H-CHNS) have long been counted as rare variants of steroid-resistant nephrotic syndrome (SRNS). This concept must be specified by two new findings: First, a study on nephrotic syndrome manifesting in the first year of life documents that H-CHNS are actually the predominant cause of ...

Congenital nephrotic syndrome is commonly associated with hypothyroidism. Thyroid hormone supplementation is recommended as standard of care. The hypothyroidism is postulated to occur secondary to chronic massive proteinuria with loss of thyroid binding globulin, thyroid hormone and iodine. Previous reports have indicated that thyroxin may be discontinued following bilateral nephrectomy. ...

We report the case of a 55-year-old Japanese woman with reactive AA amyloidosis associated with rheumatoid arthritis, in which inflammatory disease was completely suppressed with infliximab. Nephrotic syndrome was observed and renal biopsy specimens revealed amyloidosis deposits. Treatment with infliximab normalized the serum amyloid A (SAA) protein level, and subsequently ...

Autoimmune thyroiditis is rarely described in association with nephrotic syndrome. Herein we report a girl who developed autoimmune thyroiditis insidiously during the course of minimal change nephrotic syndrome. She was steroid-sensitive, but developed severe steroid dependency and did not respond to cyclophosphamide therapy. She went into stable remission with levamisole. ...

This report describes an uncommon case of nonamyloidotic fibrillary glomerulonephritis. A 5-year-old female European cat was presented with nephrotic syndrome. Serum biochemistry and urinalysis revealed a mild increase in cholesterol, low total protein, severe hypoalbuminemia, and high proteinuria with a high protein-to-creatinine ratio. An histologic examination revealed an interstitial nephritis ...

PURPOSE OF REVIEW: This review examines new literature published in 2006 and 2007 on steroid-sensitive nephrotic syndrome. RECENT FINDINGS: Steroid-sensitive nephrotic syndrome has long been thought to be due to lymphocyte-derived circulating factors leading to podocyte injury with subsequent proteinuria. New studies support this mechanism and implicate the T helper ...

PURPOSE OF REVIEW: Idiopathic nephrotic syndrome in children is commonly associated with minimal change disease and response to steroid therapy. Steroid-unresponsive nephrotic syndrome is often characterized by persistent proteinuria and progression to chronic kidney disease. Focal segmental glomerulosclerosis is the leading cause of steroid-unresponsive nephrotic syndrome in childhood. There is ...

A patient presenting with a swollen left leg and pleuritic chest pain was shown to have deep vein thrombosis (DVT) by Doppler studies. He was anticoagulated but required two further admissions with swelling of both legs before a diagnosis of nephrotic syndrome was considered and confirmed. Renal biopsy showed that ...

SummaryCongenital Nephrotic Syndrome of the Finish type (CNF) is a rare and severe disease. A neonate with CNF is described. The diagnosis carries a dramatically poorer prognosis than nephrotic syndrome diagnosed after one year. The clinical course is one of persistent oedema and recurrent infections leading to death. The gene ...

Situs inversus totalis is a rare congenital anomaly that often occurs concomitantly with other disorders. A spectrum of renal abnormalities of patients with situs inversus has been reported. Developmental anomalies, including agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney, have been reported. The association of situs inversus with nephrotic ...

Glomerular deposition of fibrillar collagen is a characteristic finding of genetically distinct conditions, including nail-patella syndrome and collagen type III glomerulopathy. A case of familial nephropathy in which steroid-sensitive nephrotic syndrome and glomerular deposits of fibrillar collagen are associated with multiple exostoses due to mutation of the EXT1 gene is ...

In order to examine whether any observed relationship between phonological awareness and reading ability in Down syndrome reflects the typical use of a phonologically based approach to reading, 12 children and young adults with Down syndrome were assessed for reading and phonological awareness skills. They were compared to a control ...

PURPOSE: Hodgkin's disease (HD) is a potentially curable malignant lymphoma with distinct histology, biologic behavior and clinical characteristics. Renal involvement in HD is rare, but it may be underestimated because renal lesions are not the major manifestation of the disease. The pathogenesis of proteinuria in HD remains unknown. CASE REPORT: ...