Congenital and infantile nephrotic syndrome reported from the Eastern world is rare and might be a different entity from that in the West. In a retrospective review of 10 nephrotic syndrome in Thai infants (5 girls and 5 boys), 7 were diagnosed with congenital nephrotic syndrome and 3 with infantile ...

Idiopathic nephrotic syndrome is the most common form of nephrotic syndrome in children, but little is known about the etiology of the disease. To obtain new insights into the etiology of the disease, we studied circannual patterns of initial episodes and relapses of steroid-sensitive nephrotic syndrome (SSNS). From 1986 to ...

We report the case of a 12- year-old Japanese boy who was receiving cyclosporine (Cs A) for steroid-dependent nephrotic syndrome despite a prior episode of Cs A-associated posterior encephalopathy. At the third relapsing of nephrotic syndrome, Cs A was initiated. Eight days after the Cs A therapy, the boy was ...

Although most childhood nephrotic syndromes respond to steroid treatment, steroid resistant nephrotic syndrome (SRNS) is also common and is particularly difficult to treat. This study investigated the role of glycosaminoglycans (GAG) in the pathogenesis and clinical course of nephrotic syndrome in children. Thirty-four children (21 males and 13 females, mean ...

Kimura disease is a rare inflammatory condition of unknown aetiology. It typically presents in young Asian males with the triad of non-tender subcutaneous swellings in the head and neck region, peripheral eosinophilia and raised serum IgE. About 16% of cases have associated renal disease. We present the case of a ...

We describe the beneficial effects of treatment with cyclosporin A in a 10-year-old boy with hyperimmunoglobulin E and refractory nephrotic syndrome. The patient was initially resistant to steroid therapy with prednisolone alone. Additional therapy with cyclosporin A was then prescribed, effectively reducing levels of serum immunoglobulin E and preventing flare ...

This study investigated the possible intrafamilial similarity of mutans streptococcal strains in some families with a child with Down syndrome using chromosomal DNA fingerprinting. The isolates were genotyped using arbitrarily primed polymerase chain reaction with the OPA 02 and OPA 03 primers. The results showed that five children with Down ...

The identification of the underlying gene defect in some cases of steroid resistant nephrotic syndrome (SRNS) has recently led to a critical breakthrough in the understanding of the pathogenesis of nephrotic syndromes. The more severe form of hereditary nephrotic syndromes is the congenital nephrotic syndrome of the Finnish type (CNF). ...

In nephrotic syndrome there is an increased tendency for bacterial infections due to immunological changes secondary to proteinuria, treatment (including steroids), and other as yet unknown causes. However, necrotizing fasciitis (NF) is an uncommon complication of the disease and has rarely been reported in nephrotic children. We report a 14-month-old ...

OBJECTIVE: To present a case with nephrotic syndrome due to minimal change disease and polycythemia. CLINICAL PRESENTATION and INTERVENTION: A 20-year-old female was admitted to our clinic for edema and severe proteinuria present with minimal change disease since the age of 7 years. Polycythemia was found during the last activation ...

OBJECTIVE: To evaluate the safety and efficacy of the hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (statins) as a potential treatment option for the dyslipidemia associated with childhood nephrotic syndrome. DATA SOURCES: Searches of MEDLINE (1966-April 2004), Cochrane Library, International Pharmaceutical Abstracts (1977-April 2004), and an extensive manual review of journals ...

We report a patient with Pompe disease who developed reversible nephrotic syndrome during prolonged, high-dose, experimental, enzyme replacement therapy with recombinant human acid alpha-glucosidase (rhGAA). Because of the development of antibodies to rhGAA and concomitant clinical decline, escalating doses of rhGAA were administered as part of an experimental immune tolerance ...

The simultaneous presentation of B-cell chronic lymphocytic leukaemia and nephrotic syndrome is described. The leukaemia responded to cytotoxic therapy but prednisolone was required to achieve remission of the nephrotic syndrome. The nephrotic syndrome relapsed when the leukaemia again became active and the white cell count rose above 20x10(9)/l, and it ...

We observed the occurrence of congenital nephrotic syndrome (CNS) and distinct ocular anomalies in two unrelated families. Eleven children from both families presented with a similar course of renal disease starting with nephrotic syndrome and renal failure prenatally or immediately after birth that resulted in death before the age of ...

We found spontaneous echoes in two teenagers with nephrotic syndrome and profound hypoalbuminemia, both having normal cardiac structure, function and output. The phenomenon disappeared after the level of albumin normalized. In one patient, all spontaneous echoes disappeared following convalescence, the level of albumin in the serum then being documented at ...

Idiopathic nephrotic syndrome is the most common glomerular disorder of childhood. Recurrence of nephrotic syndrome immediately following renal transplantation is rapid, results in a high rate of graft loss, and represents the most severe form of nephrotic syndrome. This review discusses the molecular heterogeneity of pediatric nephrotic syndrome across the ...

Intra-vascular lymphoma is usually reported as a rare and fatal disorder. We describe here the first case of an intra-vascular lymphoma revealed by a nephrotic syndrome for which a durable remission has been obtained by 8 cycles of bi-mensual CHOP and Rituximab therapy. In this report, 18 fluorodesoxyglucose tomoscintigraphy is ...

Unlike hepatitis B and C, renal involvement has been extremely uncommon in patients with hepatitis Avirus (HAV) infection. Nephrotic syndrome has been documented as a rare complication in association with HAV infection. In this report, we describe a patient with serologically documented HAV infection, who presented with nephrotic syndrome. The ...

Serum immunoglobulin E (IgE) and absolute eosinophil count (AEC) were assayed in 48 children with idiopathic nephrotic syndrome and 20 controls. The mean serum IgE (p < 0.001) and AEC (p < 0.05) were significantly raised in active nephrotic syndrome compared with controls. The mean values of IgE and AEC ...

The aim was to explore the comorbidity between Angelman syndrome and autism spectrum disorders (ASDs). Identification of autism in children with Angelman syndrome presents a diagnostic challenge. In the present study, 16 children with Angelman syndrome, all with a 15q11-13 deletion, were examined for ASDs. Thirteen children with Angelman syndrome ...

Kidney podocytes and their slit diaphragms form the final barrier to urinary protein loss. This explains why podocyte injury is typically associated with nephrotic syndrome. The present study uncovered an unanticipated novel role for costimulatory molecule B7-1 in podocytes as an inducible modifier of glomerular permselectivity. B7-1 in podocytes was ...

Castleman's is an uncommon lymphoproliferative disorder secondary to lymphoid follicle hyperplasia and marked capillary proliferation with endothelial hyperplasia. This illness can be associated with glomerulonephritis (GN). Here, we report a case with steroid-dependent nephrotic syndrome secondary to proliferative mesangial glomerulonephritis in a patient with Castleman's disease, that was diagnosed several ...

A 16-year-old patient with steroid-dependent nephrotic syndrome with more than 35 relapses developed severe relapsing idiopathic thrombocytopenic purpura (ITP). At the age of 2 years, nephrotic syndrome was diagnosed and successfully treated with a standard prednisone regimen. Frequent relapses occurred. Treatment with oral cyclophosphamide followed by cyclosporine was successful, but ...

PURPOSE OF REVIEW: This review provides a concise update of the most recent literature related to the diagnosis and care of patients with congenital nephrotic syndrome. This topic is of particular interest in light of the rapidly growing body of literature regarding mutations of proteins such as nephrin and podocin ...

PURPOSE OF REVIEW: Exciting new therapeutic approaches to the treatment or prevention of Alzheimer's disease involve preventing, slowing or reversing beta-amyloid accumulation. These interventions may also apply to the treatment of Alzheimer's disease in Down syndrome. The purpose of the current review is therefore to summarize developments and advances in ...

Arterial thrombosis is relatively rare compared with venous thrombosis in nephrotic syndrome. However, the assessment of its pathogenesis and risk factors in individual patient with nephrotic syndrome is necessary to allow appropriate prophylactic management because it is a potentially serious problem. Hereby, with review of the literature, we report a ...

A 68-year-old woman and a 72-year-old man presented with distal weakness of the limbs and numbness following an influenza vaccination within 2 weeks. Moreover, Guillain-Barré syndrome (GBS) was diagnosed in two patients. Pericarditis was diagnosed in the first patient who also had precordial chest pain with referral to trapezius ridge, ...

Posterior leukoencephalopathy syndrome (PLES) is an acute neurological disorder. The most plausible hypothesis for the pathophysiology of PLES is the loss of autoregulation and consequent vasogenic edema. PLES is mostly attributed to severe or sudden elevations of arterial blood pressure. A number of reports, however, describe patients with PLES without ...

We report a 71-year-old woman with antiepiligrin cicatricial pemphigoid associated with nephrotic syndrome. She presented with a six-month history of pruritic blisters over her trunk and legs. She also had episodes of recurrent painful oral erosions. A skin biopsy showed a subepidermal bulla, and a direct immunofluorescence (DIF) study revealed ...

BACKGROUND: The atherogenic serum lipoprotein(a) [Lp(a)] is significantly elevated in patients with nephrotic syndrome. The underlying mechanism for this elevation is poorly understood. METHODS: We investigated in 207 patients with nondiabetic nephrotic syndrome and 274 controls whether the apolipoprotein(a) [apo(a)] kringle-IV repeat polymorphism explains the elevated Lp(a) levels in these ...

To develop an experimental animal model for immune-mediated glomerulonephritis and nephrotic syndrome, nine healthy dogs were sensitized by intravenous injection with 1 microg of endotoxin and 5 mg of native bovine serum albumin. After 1 week, 120 mg of cationized bovine serum albumin was injected intravenously 5 times a week. ...

A 70-year-old woman was admitted to our hospital for the treatment of diffuse scleroderma and marked edema in the lower extremities. Renal biopsy revealed membranous change, interstitial nephritis, and intimal hyperplasia of the small arteries. The patient was diagnosed as having mixed connective tissue disease (MCTD) presenting with nephrotic syndrome ...

Nephrotic syndrome in infancy and childhood is known to be associated with a hypercoagulable state and thromboembolic complications, but cerebral sinovenous thrombosis (CST) is a very rare and serious one, with only a few isolated reports in the literature. A case is presented of a 9-year-old boy with nephrotic syndrome ...

A 17-year-old white male with a diagnosis of Guillain-Barré syndrome (GBS) developed severe proteinuria and edema 5 days later. Renal biopsy confirmed minimal change nephrotic syndrome (MCNS). To our knowledge this is the first report of such an association between ex novo MCNS and GBS in a pediatric patient. The ...

The authors report a case of adult-onset minimal change nephrotic syndrome (MCNS) that was resistant to steroid and cyclophosphamide therapy. Introduction of cyclosporin induced an usual cutaneous reaction of severe flushing attacks. Tacrolimus successfully alleviated both the nephrotic syndrome and the cutaneous side effect associated with cyclosporin use. The antiproteinuric ...

A young girl presented at 1 month of age with nephrotic syndrome. By incorporating clinical and pathologic data, the diagnosis of congenital nephrotic syndrome, Finnish type, was made. The differential diagnosis of early onset nephrotic syndrome, as it pertains to this patient is discussed. This article highlights recently discovered glomerular ...

Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). A third distinct type, membranous nephropathy, is rare in children. Other causes of isolated nephrotic syndrome can be subdivided into two major categories: rare genetic disorders, and secondary ...

Ornipressin is a vasopressin analogue that can cause potent splanchnic vasoconstriction. It has been shown that, in combination with albumin infusion, ornipressin is able to reverse hepatorenal syndrome. However, its clinical use is limited by possible severe ischaemic complications. In our case, a 47-year-old man received a right hemihepatectomy for ...

BACKGROUND: Gastrointestinal disturbances are encountered frequently in the course of the nephrotic syndrome but intussusception is a rare association. It may be the result of incoordinate motility and bowel wall edema. OBJECTIVE: To report a case of intussusception associated with relapsing nephrotic syndrome. CASE REPORT: The authors reviewed the case ...

The congenital nephrotic syndrome (CNS) is an uncommon disorder with onset of the nephrotic syndrome usually in the first three months of life. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The clinical course is marked by failure ...

Nephrotic syndrome is a rare manifestation of malignancy associated with paraneoplastic syndrome. Paraneoplastic nephrotic syndrome has been reported in various malignancies: malignant lymphoma, colon cancer, lung cancer and prostate cancer. However, an ovarian carcinoma associated with nephrotic syndrome has rarely been reported. Only six cases of ovarian carcinoma associated paraneoplastic ...

Stevens-Johnson syndrome (SJS) is a mucocutaneous disease that can be lethal. It can occur in association with altered immunological conditions and the administration of various drugs, including corticosteroids. We report a case of SJS in a 14-year-old male with nephrotic syndrome, who was treated with oral prednisolone for 6 weeks. ...

OBJECTIVE: To evaluate the clinical usefulness of combined furosemide and human albumin for the treatment of diuretic-resistant edema in patients with nephrotic syndrome and cirrhosis. DATA SOURCES: Clinical literature was accessed through MEDLINE (1966-May 2002). Key search terms included furosemide, albumin, human albumin solution, nephrotic syndrome, and cirrhosis. DATA SYNTHESIS: ...

Allgrove's syndrome, i.e., achalasia, addisonianism, alacrima (OMIM 231550) is an autosomal recessive disorder recently associated with the AAAS gene coding for the Aladin protein. However, the pathophysiology of achalasia in Allgrove's syndrome remains obscure. Here we investigated the histopathology of the cardia in Allgrove's syndrome. Myectomy specimens from 10 children ...

We present a case report of a 10-year-old male with nephrotic syndrome who presented with a complaint of shortness of breath. The patient had been recently hospitalized for an exacerbation of nephrotic syndrome and had received steroid and diuretic therapy. Initial vital signs showed tachycardia and mild tachypnea. After being ...

We describe a patient with rheumatoid arthritis (RA) who developed a nephrotic syndrome during treatment with a fully human recombinant monoclonal antibody against TNFalpha (adalimumab, Humira, Abbott). The proteinuria disappeared spontaneously after cessation of anti-TNFalpha treatment and relapsed after rechallenge, pointing to anti-TNFalpha as the culprit. Although renal biopsy disclosed ...

We report on a middle-aged Caucasian male who presented with nephrotic syndrome that on 2 consecutive recurrences was accompanied by a pulsating tumor suggesting temporal arteritis. Renal biopsies showed features of a low-grade mesangial-proliferative glomerulonephritis. The resected tumor in the temporal region revealed a lesion consistent with angiolymphoid hyperplasia with ...

The diagnosis of congenital nephrotic syndrome (NS) is a challenge both for clinicians and for pathologists. We observed three cases in a series of 50 children with NS nonresponsive to therapy, corresponding to one case each of minimal change disease, Finnish-type glomerulopathy, and diffuse mesangial sclerosis--two histopathologic studies were performed ...

An alert and oriented 27-year-old African American woman with AIDS presented with a 10-day history of fever, cough productive of yellow sputum, nausea, and vomiting and a 1-day history of excruciating headache and photophobia. Her condition rapidly deteriorated into a coma with decorticate and then decerebrate posture, and she died ...

We report a girl with congenital nephrotic syndrome (CNS) associated with cytomegalovirus (CMV) infection and histological findings on renal biopsy that suggested a causal relationship between the two. She was subsequently found to be homozygous for a nonsense mutation in the NPHS2 gene encoding podocin (R138X), which is the true ...