Strongyloidiasis, a chronic infection caused by the intestinal parasite Strongyloides stercoralis, is prevalent in the Nansei Islands of Japan. Here, we report our findings on a case of strongyloidiasis complicated with steroid-resistant minimal change nephrotic syndrome in a 69-year-old male resident of Fukuoka Prefecture who had lived in Yakushima, one ...

We report a child with idiopathic nephrotic syndrome whose condition was complicated by extensive pneumatosis intestinalis during a nephrotic relapse. The concomitant use of steroid and immunosuppressive agents and a preceding norovirus gastroenteritis infection were identified as risk factors.

OBJECTIVE: To identify early prognostic factors for idiopathic nephrotic syndrome (INS) in childhood. STUDY DESIGN: A retrospective analysis of 103 patients with INS at onset, all treated in a single center with the same induction protocol, was conducted. Minimum length of follow-up was 2 years; median length of follow-up was ...

Fabry disease is an X-linked lysosomal storage disorder caused by mutations of the GLA gene and deficiency in alpha-galactosidase A activity. Glycosphingolipids accumulation causes renal injury that manifests early during childhood as tubular dysfunction and later in adulthood as proteinuria and renal insufficiency. Nephrotic syndrome as the first evidence of ...

A 21-year-old man with lymphadenopathy and Coombs-positive hemolytic anemia had been treated with steroid maintenance therapy. He developed nephrotic syndrome with size increase of lymphadenopathy. Lymph node examination disclosed angioimmunoblastic T-cell lymphoma (AITL). Light microscopy of a renal biopsy specimen showed typical features of membranous nephropathy (MN), such as bubbling ...

A 76-year-old man developed minimal change nephrotic syndrome. Treatment with prednisolone failed to induce sustained remission, and cyclosporine was added. Symptoms for the syndrome improved, and the doses of prednisolone and cyclosporine were gradually decreased. However, he had repeated relapses of the syndrome with gradually elevated CRP concentration despite no ...

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 ...

Spontaneous remission is a well known characteristic of idiopathic membranous nephropathy, but contemporary studies describing predictors of remission and long-term outcomes are lacking. We conducted a retrospective, multicenter cohort study of 328 patients with nephrotic syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy. Spontaneous remission occurred in ...

Nephrotic syndrome in children is a clinical manifestation of different histopathological subtypes of glomerulopthies. There is a paucity of studies about the histopathological spectrum of childhood nephrotic syndrome in our country. A cross sectional study was carried out from July 2005 to June 2007 with the aim to see the ...

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 ...

Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS) have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of ...

OBJECTIVE: To observe the influence of prednisolone treatment on bone mineral density (BMD) in children with idiopathic nephrotic syndrome. METHODS: Duel-energy X-ray absorptiometry of lumbar spine (L1-L4) was performed on 40 patients (18 first episode and 22 relapsers) of steroid sensitive idiopathic nephrotic syndrome. RESULTS: Patients of first episode and ...

Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. In adults, diabetes mellitus is the most common secondary cause, and focal segmental glomerulosclerosis and membranous nephropathy are the most common primary causes. ...

The approach to the pediatric patient with membranous nephropathy (MN) can be challenging to the practitioner. The clinical presentation of the child with this histologic entity usually involves some degree of proteinuria ranging from persistent, subnephrotic-ranged proteinuria to overt nephrotic syndrome. Patients often have accompanying microscopic hematuria and may have ...

Numerous reports during the last 60 years have reported a strong association between idiopathic nephrotic syndrome and atopic disorders. Idiopathic nephrotic syndrome can be precipitated by allergic reactions and has been associated with both aeroallergens (pollens, mold, and dust) and food allergies. Patients with idiopathic nephrotic syndrome also may show ...

Congenital nephrotic syndrome (CNS) is a rare kidney disorder characterized by heavy proteinuria, hypoproteinemia, and edema starting soon after birth. The majority of cases are caused by genetic defects in the components of the glomerular filtration barrier, especially nephrin and podocin. CNS may also be a part of a more ...

Congenital nephrotic syndrome is a rare clinical entity defined as massive proteinuria leading to symptoms within the infant's first 3 months of life. Although the association between congenital nephrotic syndrome and cytomegalovirus infection has been identified, association with haemophagocytosis has not been reported in the literature. In this case report ...

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 ...

Nephrotic syndrome, an entity described at the beginning of the XXth century, has still an unclear pathophysiology, genetic and immunologic factors being incriminated. Here we reported 3 cases of nephrotic syndrome with genetic involvement. The article presented tremendous progress that have been made over the past few years in enhancing ...

We present a case of Rhodococcus equi primary retroperitoneal abscesses without pulmonary involvement in an immunocompromised patient with severe nephrotic syndrome. No risk factors for exposure to R. equi were present. The infection was successfully treated with long-term combination antibiotic treatment including linezolid and tigecycline.

We present a case of nephrotic syndrome secondary to a membranous glomerulonephritis (MG), in a nonsmoking female with a solitary pulmonary nodule, which did not show growth during 2 years of followup. A biopsy by videothoracoscopy showed a granulomatous non-neoplastic process with giant multinucleated cells. The appearance of a nephrotic ...

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 ...

A 25-year-old man developed nephrotic syndrome and severe hypertension following occupational exposure to mercury vapor whilst working at a fluorescent light factory. A renal biopsy confirmed minimal-change disease on light microscopy, immunofluorescence and electron microscopy. He was also noted to be polycythemic which was initially treated with venesection. His blood ...

Nephrotic syndrome is caused by increased permeability of the glomerular filtration barrier for macromolecules. The identification of mutations of various podocyte-expressed proteins as causes of familial nephrotic syndrome has significantly contributed to shedding light into the molecular pathogenesis of nephrotic proteinuria and into the physiology of the glomerular sieve. More ...

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 ...

Renal involvement in Churg-Strauss syndrome (CSS) is not uncommon, but nephrotic syndrome is rarely reported in patients with CSS. A 25-year-old woman with a long history of bronchial asthma presented to our hospital with a chief complaint of generalized edema. Laboratory studies revealed normocytic normochromic anemia, marked eosinophilia, positive anti-neutrophil ...

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 ...

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 ...

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 ...

The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of ...

Nephrotic syndrome is characterised by proteinuria >3.5 g/24h, oedema, hypoalbuminaemia and hyperlipidaemia. Several glomerular diseases, either primary or secondary, may lead to nephrotic syndrome. Investigations for nephrotic syndrome include immunological and infectious evaluations. Renal biopsy is often mandatory, except in diabetes. Depending on aetiology specific treatment, often with immunosuppressive agents, ...

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 ...

Remarkable advances have been made in the past decade in understanding the pathophysiology of idiopathic nephrotic syndrome. Although the initiating events leading to the onset of proteinuria still are not well defined, it has become increasingly clear that many glomerular diseases can be classified as podocytopathies, with injury to the ...

INTRODUCTION: Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fluid in the interstitial space, most commonly in the extremities. Primary lymphoedema, a developmental abnormality of the lymphatic system, may become evident later in life when a triggering event exceeds the capacity of normal lymphatic ...

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 ...

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 ...

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 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 ...

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 ...

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: 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, ...

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 ...

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. ...

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 ...

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, ...

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. ...

<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 ...

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. ...

Thyroid hormones play an important role in the growth of the kidney and maintenance of its functions. Prolonged hypothyroidism is known to be accompanied by changes in renal morphology such as thickening of the glomerular and tubular basement membranes as well as increased mesangial matrix. Increased transcapillary leakage of plasma ...