Progressive outer retinal necrosis syndrome (PORN) is a variant of necrotizing herpetic retinopathy and the majority of the described cases were related to acquired immunodeficiency syndrome. We present a patient who is HIV negative with nephrotic syndrome and prednisolone use for 4 months who showed clinical features of PORN. Low ...

The association of malignancy and various glomerulopathies is a well recognized phenomenon. We report a case of nephrotic syndrome secondary to minimal-change disease in a patient with chronic lymphocytic leukemia (CLL) and review the literature on nephrotic syndrome in this disorder. Since the relative distribution of etiologies differ from what ...

The occurrence of nephrotic syndrome after an insect sting is rarely reported in the literature. A 2-year-old boy developed generalized edema 5 days after a bee sting. The laboratory investigations revealed nephrotic range proteinuria, hypoproteinemia, hypoalbuminemia and hyperlipidemia. The corticosteroid treatment induced prompt remission with resolution of edema and normalization ...

In neoplastic disorder-related nephrotic syndrome, focal glomerulosclerosis (FGS) has been reported mainly in hematological disorders like minimal change nephrotic syndrome (MCNS) in association with presumed T lymphocyte dysfunction. The association of FGS with cancer or solid tumor is rare. We report a case of nephrotic syndrome due to FGS in ...

A 73-year-old man was admitted to our hospital because of pleural effusion and nephrotic syndrome. Sjogren's syndrome (Sjs) was diagnosed based on a positive test for antibodies to Ro and La, and the result of labial salivary gland biopsy. The pleural effusion showed a high number of lymphocytes and high ...

We report a 17-year-old Saudi girl who presented with nephrotic syndrome at the age of 7 years. A renal biopsy revealed a mildly proliferative immune complex-mediated glomerulonephritis, which on ultrastructural examination revealed prominent thickening of the capillary basement membranes, along with marked splitting and lamellation of lamina densa resembling those ...

Waldenström's macroglobulinaemia (WM) is described as a disorder of plasmacytoid lymphocytes. The renal complications of WM are less common and severe than those of multiple myeloma. We present a case of WM complicated by nephrotic syndrome. A biopsy specimen of the kidney revealed the intraglomerular thrombi of immunoglobulin M paraprotein. ...

Hyperimmunoglobulinaemia E (hyper-IgE) syndrome (Job's syndrome) is a rare disease that presents with recurrent cutaneous and sinopulmonary infections, and which begins in infancy and is associated with extreme hyper-IgE. The pulmonary imaging features typically consist of recurrent alveolar lung infections, pneumatocoeles and, occasionally, pneumothorax. Various other causes of pneumatocoeles in ...

The molecular mechanisms maintaining the kidney glomerular filtration barrier remain poorly understood. Recent evidence suggests that mitochondrial dysfunction is a characteristic feature of kidney glomeruli in congenital nephrotic syndrome of the Finnish type (CNF). Here we searched for detailed functional evidence of mitochondrial lesion in CNF kidneys. We used histochemical ...

A 58-year-old male patient had secondary polycythemia associated with idiopathic nephrotic syndrome. The renal biopsy revealed membranous nephropathy, and the bone marrow biopsy disclosed hypercellular marrow with mild panhyperplasia. The concentration of serum erythropoietin was 8.5 mU/ml. The erythrocytosis was characterized by an increased red cell volume (40.2 ml/kg) and ...

We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of ...

We report an 11-year-old Japanese boy with Kimura disease and associated nephrotic syndrome. Before the diagnosis of Kimura disease was established, the patient had three episodes of swelling on the left cheek with subsequent nephrotic syndrome. Steroids were effective for both conditions. However, both conditions recurred within months of discontinuation ...

Nephrotic syndrome represents a constellation of symptoms including hyperalbuminuria, hypoalbuminemia, edema formation, hypercholesterolemia, hypertension, hypercoagulopathy, and increased infection risk. The hallmark of this syndrome is proteinuria greater than 3.5 grams per 24 hours, and the clinical features are secondary manifestations of an underlying primary glomerular or systemic disease. The objectives ...

Patients with primary thyroid failure on levothyroxine (LT4) replacement who develop nephrotic syndrome (NS) may rarely present with an increase in LT4 requirements. In this report, we describe a patient with thyroid failure following radioactive iodine ablation for Graves' disease who required an escalation of LT4 doses following the onset ...

Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic "cold" abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with ...

Visceral leishmaniasis is a parasitic disease endemic in the Mediterranean Basin, including Malta. Finnish-type congenital nephrotic syndrome is an autosomal recessive condition that presents in the first 3 months of life with heavy proteinuria, hypoalbuminemia with secondary edema, and hyperlipidemia. We present an infant with congenital nephrotic syndrome who had ...

There are a large number of glomerular diseases that may be responsible for a nephrotic syndrome, the most frequent in childhood being minimal change disease. In the past few years, the molecular genetic basis of several conditions that may cause a nephrotic syndrome have been identified. Denys-Drash syndrome and Frasier ...

We report a 21-year-old male with childhood-onset familial nephrotic syndrome and frequent relapses who manifested toxicity or treatment resistance to corticosteroids, cyclophosphamide, cyclosporin-A, and tacrolimus. Monotherapy with mycophenolate mofetil (MMF) resulted in maintenance of clinical remission for 14 months without noticeable toxicity, while allowing resolution of steroid-induced side effects. Our ...

We report the case of a 62-year-old man with nephrotic syndrome associated with stage B chronic lymphocytic leukemia (CLL). Kappa Bence Jones proteinuria and the glomerular deposition of kappa-light chain were observed. Although treatment with cyclophosphamide and prednisolone tended to reduce the level of proteinuria, the administration of angiotensin-converting enzyme ...

A 63-year-old man presented to our department with dyspnea and peripheral edema. A cystic mass in the right upper abdomen, consistent with echinococcal disease was discovered. Proteinuria was also present, and a nephrotic syndrome was diagnosed. The kidney biopsy revealed minimal change glomerulonephritis. Treatment with the antiechinococcal drug albendazole induced ...

Sandifer's syndrome is a rare manifestation of gastroesophageal reflux (GER) in children, occurring in association with abnormal movements of the head, neck, and upper part of the trunk. Out of 65 children with Sandifer's syndrome described in literature, only 2 were breast-fed. We report on a 15-day-old breast-fed girl affected ...

A 4-year-old boy with idiopathic steroid responsive nephritic syndrome developed bilateral sixth-nerve palsy and lethargy secondary to cerebral sinus thrombosis. Treatment with heparin, fresh frozen plasma as source of antithrombin III and vitamin K inhibitors may have prevented further sequels. However, anti-coagulation, as assessed by partial thromboplastin and prothrombin time, ...

Hyperhomocyst(e)inemia is an independent risk factor for atherothrombosis in several clinical settings in which renal function is impaired, but its prevalence in the nephrotic syndrome has not been investigated in detail, even though this syndrome provides an excellent model in which to study a possible link between albuminuria, proteinuria, and ...

A 10-year-old boy presenting with steroid resistant nephrotic syndrome developed Fournier gangrene of the scrotum. Antimicrobial drug therapy, intravenous albumin, excision of necrotic scrotum and left orchidectomy followed by skin grafting 3 weeks later led to an excellent cosmetic and medical result. Six months later he remains nephrotic on diuretic ...

The occurrence of nephrosis in the first 3 months of life is rare and is termed 'congenital nephrotic syndrome.' The congenital nephrotic syndrome is a group of heterogeneous diseases with a clinical course that differs markedly from the childhood nephrotic syndrome. The coexistence of a congenital nephrotic syndrome and gonadal ...

This is a randomized, double-blind, placebo-controlled, cross-over study to determine whether tuna fish oil decreased hyperlipidemia and proteinuria in children with steroid-resistant nephrotic syndrome. Five boys were supplemented with both 4 grams of tuna fish oil and placebo in a randomized order for 8 weeks of each treatment separated by ...

Nail-patella syndrome (NPS), also known as hereditary onycho-osteodysplasia, is an autosomal dominant pleiotropic disorder characterized by nailbed dysplasia or hypoplasia, absent or hypoplastic patellae, iliac horns and deformation or luxation of the radial head. Nephropathy is a known serious complication associated with NPS. In this report, we describe an adult ...

Patients with cirrhosis and with nephrotic syndrome have subnormal responses to diuretics. The mechanism of this effect in cirrhosis is decreased pharmacodynamics of response. Large doses of diuretic are not useful in this setting. Instead, more frequent administration of modest doses is required. In nephrotic syndrome, substantial amounts of diuretic ...

A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an ...

Ehrlichiae are rickettsial organisms recently shown to be human pathogens. Infections often cause fever, myalgia, and hematological abnormalities, and sometimes mild elevation in transaminases, creatinine, and urinary protein. We report a teenager with nephrotic syndrome from minimal change glomerulonephritis and serological evidence of ehrlichiosis. In the appropriate clinical setting, Ehrlichiae ...

We describe a patient who developed nephrotic syndrome at 5 months, with extensive glomerular and tubular damage on biopsy. The patient was treated with diuretics and was asymptomatic for a decade despite unremitting proteinuria. A repeat biopsy at 13 years of age showed remarkable healing with histopathological features consistent with ...

Side effects such as cutaneous vasculitis, which occur during prolonged levamisole treatment, may discourage the utilization of the drug in relapsing nephrotic syndrome. We describe a child who developed disseminated vasculitis during prolonged treatment with levamisole. The acute phase was characterized by hepatosplenomegaly, hemolytic anemia, IgM anticardiolipin and p-antineutrophil cytoplasmic ...

The mechanisms of sodium retention in edema-forming minimal change nephrotic syndrome (MCNS) have not been completely evaluated. The aim of this study was to characterize the transmembrane sodium transport in nephrotic syndrome by measuring the erythrocyte sodium-lithium countertransport (SLC) in vitro. Eighteen children with MCNS were studied in the edema-forming ...

Two children with congenital nephrotic syndrome are described (one with Finnish-type nephrosis, the other with diffuse mesangial sclerosis). Both children have had a prolonged and sustained clinical response with good physical health and normal growth patterns using captopril and indometacin as their sole treatment. No adverse effects have been noted. ...

Commonly reported renal complications of non-steroidal anti-inflammatory drugs (NSAID) include acute renal failure and/or acute interstitial nephritis; in rare cases a nephrotic syndrome was also observed. In most cases this was due to the development of secondary membranous nephropathy. Following withdrawal of the drug the nephrotic syndrome usually resolved rapidly. ...

We report the case of a young woman with primary antiphospholipid syndrome (APS), which presented with acute renal failure, hypoproteinemia, hypoalbuminemia and nephrotic proteinuria. Investigations showed total infarction of right kidney by extensive arterial and vein thrombosis and presence of anticardiolipin antibodies IgG isotype (anti-beta2-glycoprotein I-positive). She was submitted to ...

A 63-year-old man presented with acute symmetrical polysynovitis associated with pitting edema of both the hands and feet. He was seronegative for rheumatoid factor and no radiologically evident erosion was noted in the joints of his hands and feet. Evaluation excluded congestive heart failure, nephrotic syndrome, and hypothyroidism as the ...

Bisalbuminemia (or alloalbuminemia) is a relatively rare hereditary or acquired condition characterized by the presence of two distinct albumin bands, or, less commonly, a single widened albumin band, after agarose gel electrophoresis of serum. Bisalbumins are caused by point- or chain-mutations that occur with a population frequency of 1:10,000 to ...

We report a case of a 38-year-old man showing the yellow nail syndrome in association with minimal change nephrotic syndrome. Treatment with prednisone and vitamin E resulted in complete resolution of the nephrotic syndrome and slow improvement of the yellow nails, respectively. Although the rare yellow nail syndrome has been ...

A 21-year-old man was admitted to Kure National Hospital with nephrotic syndrome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months. Renal biopsy gave a diagnosis of membranoproliferative glomerulonephritis. Steroid therapy ...

A 35-year-old man with immunoglobulin A (IgA) nephropathy who developed hemolytic uremic syndrome (HUS) presented with transient elevation of serum creatinine, thrombocytopenia, and hemolytic anemia with fragmented red cells with nephrotic syndrome. Hemolytic anemia and the temporarily deteriorated renal function were improved after hemodialysis and plasma exchange. Histological findings were ...

Chronic strongyloidiasis is a mild disease and has never been reported to be associated with nephrotic syndrome. Disseminated strongyloidiasis is known to have high mortality, but it frequently is not diagnosed until autopsy. We report a patient with nephrotic syndrome developing disseminated strongyloidiasis after steroid therapy. The findings in renal ...

The authors describe two cases of cerebral venous thrombosis (CVT) in patients with nephrotic syndrome. The main clinical features of CVT were persistent headache, hemiparesis, and seizure, and the diagnosis was based on magnetic resonance imaging and magnetic resonance angiography. Both showed acquired deficiency of free protein S. The neurologic ...

BACKGROUND: Nephrin recently has been identified as a putative adhesion molecule, expressed in the glomerulus, in which mutations cause congenital nephrotic syndrome of Finnish type. We sought to determine whether expression of nephrin is altered in human glomeruli in patients with acquired nephrotic syndrome. METHODS: We performed PCR amplification of ...

BACKGROUND: It is widely accepted that minimal change nephrotic syndrome (MCNS) is the most common cause of nephrosis in children. Recent studies have demonstrated an increasing incidence of focal segmental glomerulosclerosis (FSGS) in adults. METHODS: To determine possible changes in the etiology of childhood nephrosis, the clinical charts of 152 ...

The nephrotic syndrome is defined by edema, hypoalbuminemia, proteinuria, and hyperlipemia with elevated cholesterol and triglyceride concentrations in serum or plasma. It may be primary or secondary to systemic disorders. Because of its incidence--between 2.0 and 7.0 cases per 100,000 children per year--this disorder is not uncommon in a busy ...

Galloway-Mowat Syndrome (GMS) has a wide variety of clinical manifestations and histologic findings. All reported cases had developed nephrotic syndrome in the first two years of life. We report a case of 12 years old boy with microcephaly, mental retardation, and typical dysmorphic features of GMS with a late onset ...

It is sometimes reported that the immunological abnormalities in myelodysplastic syndromes (MDS) induce autoimmune disease (i.e., acute systemic vasculitic syndrome, chronic cutaneous vasculitis, polyneuropathy, relapsing polychondritis, and steroid-responsive pulmonary disorders). We investigated the clinical features of patients with MDS accompanied by nephrotic syndrome. We enrolled 125 patients with MDS who ...

An adult golden-mantled flying fox (Pteropus pumilus) was diagnosed with nephrotic syndrome on the basis of the findings of proteinuria, hypoalbuminemia, hypercholesterolemia, and cranial edema. Membranoproliferative glomerulitis and interstitial nephritis were confirmed antemortem by renal biopsy. The bat had received seven injections of oxytocin in the period immediately prior to ...

In some individuals, chronic lymphocytic leukemia (CLL) may be associated with glomerular disease from membranous nephropathy with resultant nephrotic syndrome. CLL is characterized by abnormal immunoregulation with a malignant clonal proliferation of lymphocytes. The association between the abnormal clone and nephrotic syndrome is suggested in some cases by the remission ...