Of 231 children with nephrotic syndrome, 87% were steroid sensitive and 13% steroid resistant. Of patients with steroid sensitive nephrotic syndrome, 38.8% were non-relapsers, 34.8% frequent relapsers and 26.4% infrequent relapsers. Among those with steroid resistant nephrotic syndrome, 37.5% had focal segmental glomerulosclerosis with a high mortality rate of 44%

Focal segmental glomerulosclerosis (FSGS) is one the most important causes of the nephrotic syndrome in adult patients. FSGS is not a disease entity. The identification of underlying causes of FSGS (secondary FSGS) has increased our insight into the pathogenesis of FSGS. Moreover, differentiating between primary (idiopathic) and secondary forms of ...

BACKGROUND: An intracardiac thrombus is extremely rare in children with the nephrotic syndrome (NS). OBJECTIVES: To present a case report of a child with steroid resistant NS and intracardiac thrombus. METHODS AND RESULTS: A 3.5-year-old boy with the first attack of steroid resistant NS was admitted to the hospital. A ...

Two patients with lymphoplasmacytic lymphoma, and monoclonal proteins of IgM in one, and IgG and lambda light chains in the second patient, nephrotic syndrome and acute renal failure are reported. A 58-year-old man previously treated for pre-B acute lymphoblastic leukemia, developed 3 years later nephrotic syndrome as a complication of ...

We report 3 patients with nephrotic syndrome ascribed to primary systemic AL amyloidosis that were successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone. M-protein in serum disappeared soon after VAD, and nephrotic syndrome gradually improved in parallel with a decrease in daily protein excretion in urine. Long-term follow-up of ...

In 2001, the Indian Pediatric Nephrology Group formulated guidelines for management of patients with steroid-sensitive nephrotic syndrome. In view of emerging scientific evidence, it was felt necessary to review the existing recommendations. Following a preliminary meeting in March 2007, a draft statement was prepared and circulated among pediatric nephrologists in ...

A 77-year-old woman with nephrotic syndrome secondary to idiopathic membranous nephropathy was treated with angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, cyclosporine A, and mycophenolate mofetil, without response. After more than 2 years of unremitting nephrosis, she began therapy with the herb Astragalus membranaceus, used by traditional Chinese physicians to treat ...

Nephrotic syndrome is associated with avid sodium retention, leading to the development of oedema and ascites. Studies in experimental animals suggest that sodium retention in nephotic syndrome is due to increased sodium re-absorption in the collecting duct, which is also the action site of vasoregulatory hormones. However, the mechanisms underlying ...

OBJECTIVE: We present a child with Denys-Drash syndrome recognized after surgery for mixed gonadal dysgenesis, and discuss screening procedures the urologist should consider in similar circumstances. CASE REPORT: A 1-year-old child with XY gonadal dysgenesis underwent genital reconstruction. The postoperative period was complicated by incisional drainage, which led to the ...

A 16-year-old man presented with severe nephrotic syndrome complicated by massive perirenal fluid. Percutaneous drainage of fluid was performed 3 times, followed by improvement in renal function and hypertension, but perirenal fluid recurred within days. Nephrotic syndrome was unresponsive to steroid therapy. A laparoscopic bilateral fenestration of Gerota's fascia and ...

We report the case of a 16-year-old male patient with steroid-dependent nephrotic syndrome who developed ischemic colitis. He was diagnosed as having nephrotic syndrome at 10 years of age and had been administered steroid, cyclosporine A, and mizoribine for 7 years. He presented with severe abdominal pain 5 days after ...

Renal disease is rare in children with juvenile idiopathic arthritis, although a number of associated nephropathies have been described, including mesangial glomerulonephritis. We report the presence of mesangial glomerulonephritis, revealed by a nephrotic syndrome, in a paediatric patient with juvenile idiopathic arthritis. Short-term steroid treatment induced a rapid remission of ...

We report on a 10-year-old child with nephrotic syndrome who developed recurrent lymphomatoid papulosis (LYP) 60 months after the first episode of proteinuria. LYP appeared when the child was taking low-dose cyclosporin and disappeared after the drug was replaced by prednisone at doses utilized for nephrotic syndrome (2 mg/kg). During ...

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 ...

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 ...

C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two ...

In this paper, a boy is reported with the association of congenital analbuminemia (CAA) and steroid-sensitive idiopathic nephrotic syndrome (INS), two conditions resulting independently in reduced colloid oncotic pressure. The unique occurrence helps confirm earlier reports that albumin is not the exclusive factor responsible for maintaining colloid oncotic pressure.

BACKGROUND: Anisakis simplex is a helmintic parasite of fish and shellfish that can induce in humans, after consumption, an immunoallergic response with multiorganic affectation, especially cutaneous (urticaria and angioedema), gastrointestinal and anaphylactic symptoms. Nephrotic syndrome can be produced by helmintic infection, especially schistosomiasis. OBJECTIVE: To report the unusual case of ...

Although venous thrombosis is a major complication in nephrotic syndrome, cerebral venous thrombosis (CVT) is rarely reported. We describe a 29-year-old male with nephrotic syndrome who suddenly developed headache and nausea. Although computed tomography scan and magnetic resonance imaging detected no abnormal lesions, phase-contrast magnetic resonance venography (PC MRV) demonstrated ...

We present the case of a 3-year-old boy with nephrotic syndrome with mild respiratory symptoms who was shown by ventilation-perfusion scan to have pulmonary embolism. He responded well to anticoagulant therapy.

We describe a child presenting with oligoclonal plasma IgM (1.2 g%) and nephrotic syndrome with focal segmental glomerulosclerosis. Oligoclonality was demonstrated by the analysis of the complementary determining region 3 (CDR 3) on immunoglobulin heavy chains and by two dimensional electrophoresis and Western blot analysis that showed the bulk of ...

The immunosuppressant tacrolimus (FK-506) is a calcineurin inhibitor with a widespread use for the prevention of graft rejection in transplantation medicine. Tacrolimus inhibits the activation of an essential transcription factor for the transcription of cytokine genes in T cells leading to a decreased production of cytokines such as IL-2 and ...

Sodium retention and edema are common features of nephrotic syndrome that are classically attributed to hypovolemia and activation of the renin-angiotensin-aldosterone system. However, numbers of clinical and experimental findings argue against this underfill theory. In this review we analyze data from the literature in both nephrotic patients and experimental models ...

This study was conducted to establish the functions and oxidative stress status in leukocytes of adult patients with nephrotic syndrome. Thirty adult patients with nephrotic syndrome and 32 controls were included. Phagocytosis ability, the killing ability of the micro-organism phagosited of polymorphonuclear leukocytes (PMNL) and monocytes, along with oxidative stress ...

A 77-year-old woman developed nephrotic syndrome associated with type III hyperlipoproteinemia (III HLP) and increased apolipoprotein E (apo E). Apo E analysis disclosed E2/E3 heterozygosity in phenotypic and genotypic expressions, without any other mutations. A renal biopsy showed intraluminal and subendothelial thrombus-like deposits in the dilated capillary loops of the ...

Although nephrotic syndrome is common among African children, the congenital forms of it are rare. This report describes the clinical presentation of a 6-week-old Nigerian infant who presented with marked pedal oedema, heavy proteinuria and serum hypoproteinaemia leading to the diagnosis of congenital nephrotic syndrome. This case is being reported ...

Pseudohyponatremia in idiopathic nephrotic syndrome with severe edema is attributed to hyperlipidemia that results in displacement of a portion of water phase of plasma. Current methods of measurement of serum electrolytes are unaffected by hyperlipidemia. In this report we demonstrate that patients with idiopathic nephrotic syndrome with severe edema and ...

Nephrotic syndrome is considered to be a late complication of psoriasis, reported usually in adults and characterized by IgA nephropathy or focal segmental glomerulosclerosis. We report on four children in whom steroid-resistant nephrotic syndrome either preceded (n = 3), by 41-120 months, or occurred simultaneously (n = 1) with psoriasis; ...

Glycation and lipid peroxidation are spontaneous reactions believed to contribute to the pathogenesis of nephrotic syndrome. Possible interrelations of glycated hemoglobin with reduced glutathione and malondialdehyde were evaluated in nephrotic syndrome patients. Eighteen nephrotic syndrome patients and 15 healthy controls were enrolled for this study. Glycated hemoglobin, reduced glutathione, malondialdehyde ...

We describe a 10-year-old boy with X-linked ichthyosis, Kallmann Syndrome and unilateral renal agenesis who presented with nephrotic syndrome. DNA analysis revealed deletion of the Steroid Sulfatase (STS) gene. STS deficiency in X-linked ichthyosis leads to cholesterol sulfate accumulation, which induces transglutaminase-1 dysfunction. Since the slit diaphragm of the glomerular ...

We report on a patient with nephrotic syndrome who developed superior sagittal sinus thrombosis. He presented with double vision due to bilateral sixth-nerve palsy and papilledema. The thrombosis resolved with intravenous heparin and oral corticosteroids. A month later there were no signs of ocular movement paresis, and both optic nerves ...

An 18-year-old girl with nephrotic syndrome presented with a life-threatening respiratory failure and a severe encephalopathy, shortly after being started on furosemide. The evaluation confirmed furosemide as the sole culprit for the clinical manifestations. This case highlights an unpredictable dose-response relationship of furosemide. The pathophysiological basis, differential diagnosis and clinical ...

A 27-day-old infant from a tribal area presented with congenital nephrotic syndrome (CNS). The presence of clinical features of syphilis, proven syphilis in the mother and complete recovery following penicillin therapy confirmed a diagnosis of congenital syphilis. In developing countries, treatable causes of CNS such as syphilis need to be ...

Schimke immuno-osseous dysplasia SIOD is a rare autosomal recessive disorder characterized by steroid resistant nephrotic syndrome, immune deficiency, and osseous dysplasia. SW/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 SMARCAL 1 is the gene responsible for SIOD but the underlying pathophysiologic mechanism is unclear, therefore, there ...

A 3-year old female goat with nephrotic syndrome was presented because of severe bilateral chemosis. The diagnosis was based on clinical findings, decreased packed cell volume, increased serum urea and creatinine, decreased serum protein and protein loss via the urinary tract. At post mortem examination, a chronic glomerulonephritis and interstitial ...

Spontaneous bacterial peritonitis is the most common infectious complication of childhood nephrotic syndrome, and Streptococcus pneumoniae is the preponderant bacterial pathogen. Spontaneous bacterial cellulitis, especially of the lower extremities, is another common infection encountered in the same patient group given that chronic edema acts as a potential culture medium. Gram-positive ...

To better understand the role of nitric oxide (NO) in pediatric nephrotic syndrome, we measured nitrite/nitrate (NOx) [NO2- and NO3-] in serum obtained from patients with several pediatric kidney diseases and investigated the locations of endothelial nitric oxide synthase (eNOS) and inducible nitric oxide synthase (iNOS). NOx in serum showed ...

Focal segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage kidney disease (ESKD). In recent years, animal models and studies of familial forms of nephrotic syndrome helped elucidate some mechanisms of podocyte injury and disease progression in FSGS. This article reviews some of the ...

Venous thrombosis is a known complication in the nephrotic syndrome, with the renal vein being the most frequent site. It can also occur in other parts of the venous system. Here, we describe a case of isolated portal vein thrombosis in a patient with nephrotic syndrome, which resolved with treatment.

Nephrotic syndrome can be associated with various neoplasms, especially solid tumors and lymphomas. This patient presented with painless hematuria of transitional cell carcinoma of urinary bladder, underwent transurethral resection, but developed recurrence 16 months later. Repeat resection was done and intravesical Bacillus Calmette-Guerin (BCG) injections were started. After six months, ...

OBJECTIVE: To report a case of Mauriac syndrome in a young child with poorly controlled type 1 diabetes mellitus. METHODS: We describe the typical features of Mauriac syndrome and review the clinical, laboratory, and ultrasound findings and follow-up results in our current patient. RESULTS: Hepatomegaly, growth impairment, and cushingoid features ...

We report the case of a young male patient with nephrotic syndrome and multiple venous thromboses. The patient presented various aggregated thrombophilic risk factors. He was found to be homozygous for factor V Leiden mutation and his anticardiolipin antibody and homocysteine levels were high. The association between nephrotic syndrome and ...

The nephrotic syndrome is associated with an increased risk ofatherosclerosis and endothelial dysfunction. Endothelial dysfunction was assessed and correlated with dyslipidemia and markers of inflammation in patients with nephrotic syndrome. 20 patients were divided into two groups: group A (10 patients with primary nephrotic syndrome) and group B, 10 controls. ...

OBJECTIVE: This study describes a patient with nephrotic syndrome who experienced recurrent pulmonary emboli as renal disease relapsed frequently. CLINICAL PRESENTATION: A 30-year-old male, who was diagnosed as having nephrotic syndrome at 12, presented with general edema, ascites and hypoalbuminemia. Although the patient responded well to steroid therapy, a common ...

INTRODUCTION: The nephrotic syndrome is associated with heightened risk for arterial and venous thrombosis. Multiple derangements of hemostasis and acquired risk factors such as hyperlipidemia and hypertension contribute to this risk. The prevalence in the nephrotic syndrome of high circulating levels of homocysteine and of low levels of the B ...

An elderly gentleman, on urinary drainage catheter for 3 months developed a purple discoloration of the urinary bag with the urine inside remaining clear. He was found to have a urinary tract infection with a strain of E. coli manifesting as a rare clinical entity reported in literature as the ...

We report a case of pneumatosis intestinalis in a diabetic patient with minimal change nephrotic syndrome, receiving immunosuppressive drugs and an alpha-glucosidase inhibitor. The patient was a 72-year-old female, who suffered from right lower abdominal pain. Abdominal CT showed massive air in the intestinal wall, compatible with pneumatosis intestinalis. Her ...

Although parasitic infections have been known to be associated with immune complex-mediated glomerular lesions, strongyloidiasis-related glomerulopathy has not been well documented. We report a patient with delayed-recognized disseminated strongyloidiasis who developed nephrotic syndrome 3 months after the beginning of the manifestations related to strongyloidiasis. A kidney biopsy showed minimal change ...

A case of a young adult with refractory nephrotic syndrome due to focal segmental glomerulosclerosis is reported. Several treatments had been used without success including steroids, cyclophosphamide, cyclosporine A, tacrolimus, and mycophenolate mofetil. Immunoadsorption was performed as a last resort to manage the nephrotic syndrome, which led to a drastic ...

Acute renal failure (ARF) is an uncommon complication in children with nephrotic syndrome. We report here the case of a 10-year-old male child with primary steroid-resistant nephrotic syndrome who was non-responsive to steroids and cyclophosphamide. A kidney biopsy revealed that he had focal segmental glomerulosclerosis. His treatment was initiated with ...