We describe a patient with microcephaly, developmental delay, and nephrotic syndrome who had normal renal function and normal brain imaging studies. She does not have the Galloway-Mowat syndrome. The concurrence of nephrotic syndrome with microcephaly and developmental delay may be coincidental, or may reflect one of at least three syndromes: ...

Paraneoplastic nephrotic syndrome is rare, bur occurs mostly in lung cancer. The glomerular lesion usually exhibits membranous nephropathy. Although surgical therapy has been shown to be effective, the treatment of a paraneoplastic nephrotic syndrome has always been a challenge. Currently no standard therapy has been established, if the paraneoplastic nephrotic ...

We report the case of a young male who developed severe nephrotic syndrome within 2-3 weeks after being stung by 3 wasps. A percutaneous kidney biopsy specimen revealed mesangioproliferative glomerulonephritis with occasional subepithelial deposits suggestive of early membranous nephropathy. The patient was treated with oral prednisone 60 mg/day with no ...

A case of nephrotic syndrome due to AA amyloidosis in a young woman suffering from erythrodermic psoriasis and psoriatic arthropathy is reported. The nephrotic syndrome regressed completely during long-term (57 months) colchicine treatment. There are 39 case reports in the literature of psoriasis associated with amyloidosis. More than 85% of ...

A 56-year-old man presented with transient anemia in minimal-change nephrotic syndrome. Following nephrotic syndrome, anemia suddenly appeared without renal dysfunction. The anemia might be attributable to hemodilution because of significant correlations between the values of hemoglobin concentration and serum total protein or blood urea nitrogen during the clinical course. A ...

Wilson's disease responds to a variety of treatments including D-penicillamine and trientene. Nephrotic syndrome is a late complication of D-penicillamine treatment. We report a pediatric patient with Wilson's disease who developed nephrotic syndrome 2 wk after beginning D-penicillamine. His nephrosis resolved and his disease is quiescent with trientene treatment.

A 46-year-old woman developed nephrotic syndrome secondary to rheumatoid arthritis (RA). A renal biopsy showed deposition of amyloid fibrils in the subendothelial space of the glomerular capillary walls. After treatment with prednisolone (PSL, 40 mg/day), the levels of C-reactive protein (CRP) and serum amyloid A decreased to within normal limits ...

Children with hemophilia B who receive exogenous factor IX infusions may rarely develop inhibitors to the exogenous factor IX and require desensitization. Nephrotic syndrome has recently been described in some of these children. We report the renal clinicopathological findings in a child with severe factor IX deficiency, requiring induction of ...

It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism. However, the Budd-Chiari syndrome has never been reported in nephrotic patients. This is the first report of such an association that, most likely, depended ...

Modern medical technology has created new syndromes of severe and permanent brain damage. In the first 25 years of the right-to-die debate, the permanent vegetative state has been the paradigmatic neurologic syndrome for decisions to discontinue treatment. In the near future, however, a far more problematic syndrome may be even ...

BACKGROUND/PURPOSE: Right postpneumonectomy syndrome is a rare complication of pneumonectomy characterized by exertional dyspnea resulting from mediastinal shift into the empty hemithorax. Historically, this problem has been treated with thoracoplasty, muscle flap transposition, pericardial fixation, and plombage using a variety of materials. Significant postoperative complications have been reported with each ...

We report a nephrotic syndrome patient with eosinophilia who developed ileus, epigastralgia and malabsorption due to strongyloidiasis which became symptomatic by steroid therapy. The patient was then treated with thiabendazole and recovered. A percutaneous renal biopsy revealed minimal change nephrotic syndrome. This renal injury may be brought on by severe ...

Familial Mediterranean fever (FMF), a paroxysmal, self-limited, inflammatory disease of unknown etiology, may result in thrombotic complications after the development of nephrotic syndrome due to amyloidosis. It has been suggested that there is increased thrombogenic activity in the blood of patients with FMF who did not develop nephrotic syndrome. We ...

PURPOSE: The case of a patient with Cronkhite-Canada syndrome, who developed a protein-losing enteropathy, is reported. METHODS: After localization of the protein-losing region, a right colectomy was performed. RESULTS: Hypoproteinemia and ectodermal changes improved postoperatively. CONCLUSIONS: Surgery is an effective treatment for protein-losing enteropathy in Cronkhite-Canada syndrome. Ectodermal changes improve ...

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded ...

Familial idiopathic nephrotic syndrome is rare. Only about 3% of patients have affected siblings. The association of familial nephrotic syndrome with congenital abnormalities is even more uncommon. To our knowledge this is the first report of the association of steroid-sensitive nephrotic syndrome and postaxial hexadactyly in two brothers born to ...

The nephrotic syndrome represents a series of physiological results ensuing from the occurrence of significant urinary loss of protein. Although the common initiating event of proteinuria has long been established, the list of problems that result has continued to grow as our understanding of the pathophysiology of nephrosis has increased. ...

Glomerular epithelial protein 1 (GLEPP1) is a podocyte receptor membrane protein tyrosine phosphatase located on the apical cell membrane of visceral glomerular epithelial cell (VGEC) foot processes. Double label immunofluorescence, immunoelectron microscopy, and peroxidase immunohistochemistry were used to map the GLEPP1 distribution in the developing glomerulus and in minimal-change nephropathy ...

A 57-year-old man with monoclonal gamma-globulinemia was admitted because of edema and proteinuria. A renal biopsy specimen showed lobular glomerulonephritis associated with deposition of material that was positive for IgG, C3, C1q, fibrin, kappa light chain, and lambda light chain but was not stained by Congo red. Glomeruli showed massive ...

Recent reviews have claimed that the majority of patients with the nephrotic syndrome have plasma volume expansion (that is, they are overfilled). Here we attempt to re-establish balance to the debate on body fluid volume status in nephrotic patients by: (a) discussing the conflicting literature on plasma volume measurements in ...

We report a case of deep vein thrombosis in a young man with idiopathic nephrotic syndrome and resistance to activated protein C. We postulate that patients with concurrent nephrotic syndrome and factor V may have an increased risk of thrombosis. Screening for factor V Leiden may be indicated in patients ...

Nephrotic syndrome is a common kidney disease seen in both children and adults. The clinical syndrome includes massive proteinuria, hypoalbuminemia, edema, and usually hypercholesterolemia. Development of these clinical changes is closely correlated with profound structural changes in glomerular epithelial cells, or podocytes, which together with the glomerular basement membrane and ...

A 7-month-old girl with congenital nephrotic syndrome (CNS) of the Finnish type was referred to our group for surgical evaluation and recommendation of a treatment plan. A Medline literature search was conducted using data bases from 1980 to 1996, on the topics of "congenital nephrotic syndrome" and "surgery." Although there ...

Cutaneous T-cell lymphoma includes mycosis fungoides and the leukemic variant, Sézary syndrome. We report the first two cases of focal segmental glomerulosclerosis in patients with cutaneous T-cell lymphoma, with nephrotic range proteinuria and review the literature on renal disease coexisting with cutaneous T-cell lymphoma. We hypothesize that glomerular injury in ...

Data are sparse regarding the histological lesions associated with the primary nephrotic syndrome in adolescents. To our knowledge there are only two published articles that have specifically addressed the histopathological lesions that typify idiopathic nephrotic syndrome in the adolescent population. We reviewed our experience from the last 14 years of ...

The combination of captopril and indomethacin has been shown to control nephrotic proteinuria in an infant with congenital nephrotic syndrome of the Finnish type. We report the satisfactory control of congenital nephrotic syndrome by enalapril, maintaining normal serum albumin levels without albumin infusions. The haplotype data of our patient were ...

A one-year-and-ten-months-old male infant with Arima syndrome, a very rare genetic disorder, underwent urgent insertion of a catheter for continuous ambulatory peritoneal dialysis (CAPD) under general anaesthesia. During the procedure he showed QRS-interval changes caused by hyperkalaemia which was successfully treated with calcium gluconate. The management and intraoperative complications of ...

An association between nephropathy and malignant solid tumours or with lymphoproliferative disorders was repeatedly reported. This association is mainly manifested by a nephrotic syndrome. In Lee's study [14], 11% of the adult nephrotics whom they had seen over a ten-year period developed a carcinoma. Membranous glomerulonephritis (MGN) is the most ...

We report a patient who presented with severe nephrotic syndrome complicated with infrarenal aortic and right renal arterial thrombosis. The nephrotic syndrome frequently causes thromboembolic complications in veins, but arterial thrombosis is relatively rare, especially in the aorta. Various predisposing factors leading to thromboembolic complications are discussed. In this case, ...

The authors describe a 46-year-old Japanese woman who had Takayasu's arteritis associated with nephrotic syndrome due to mesangial proliferative glomerulonephritis with crescent. Although a few cases of focal and segmental mesangial proliferative glomerulonephritis associated with Takayasu's arteritis have been reported, nephrotic syndrome has not been reported previously in this situation.

A case of immunotactoid glomerulopathy in an 18-year-old man with an idiopathic hypereosinophilic syndrome is presented. The patient showed cervical lymphadenopathy, asymptomatic proteinuria of nephrotic range, and hematuria without any defined immunologic disease. Marked and prolonged hypereosinophilia was found in peripheral blood (eosinophil count; 6,248/mm3) and bone marrow (eosinophil series; ...

A 77-year-old woman complaining of anorexia and nausea was referred to the hospital with a diagnosis of advanced gastric cancer. The patient also had congestive heart failure with atrial fibrillation and severe hypoproteinemia. Proteinuria, hypoproteinemia and other laboratory data suggested that she had nephrotic syndrome. Total protein level was 4.6 ...

A case who developed Guillain-Barré syndrome (GBS) and nephrotic syndrome (NS) simultaneously is reported. In this case, the onset of nephrotic change of proteinuria coincided with the development of the neurological disturbance. Analysis of renal biopsy by light, immunofluorescent and electron microscopy showed minor glomerular abnormalities. The occurrence of GBS ...

The presentation of Hodgkin's disease is in the vast majority of cases rather classical. However, there are some rare syndromes which might be very puzzling. In this contribution, we will discuss some of these rare syndromes. We will concentrate on following topics: (1) pruritus (itching), (2) cutaneous manifestations of HD, ...

Two hundred and seventy two children with the nephrotic syndrome were seen and followed up at the Paediatric Renal Unit of the University of Nigeria Teaching Hospital, Enugu over 12 1/2 year period, between June 1983 and December 1995. Of these, there were nine (3.3%) children with homozygous sickle cell ...

Myasthenia gravis is caused by antibodies against acetylcholine receptors and is treated with inhibition or elimination of antibody production. We report a 43-year-old myasthenic female who was symptomatic until she developed proteinuria from nephrotic syndrome, which caused a marked drop in acetylcholine receptor antibody titer with remission of myasthenia. Treatment ...

This report describes cerebral venous sinus thrombosis, a rare and perhaps under-diagnosed complication of nephrotic syndrome. We review the pathophysiology of the coagulopathy associated with nephrotic syndrome including abrupt renal loss of antithrombin III. We propose a rationale approach to treating this condition with low-molecular-weight heparin and antithrombin III replacement.

Congenital nephrotic syndrome, a rare form of nephrosis, presents during the first year of life. We describe the case of a patient seen on the fifth day of life with seizures, hypocalcemia, hypothyroidism, and cerebral deep venous thromboses. Whereas these are generally long-term complications of this disorder, they may also ...

Minimal change nephrotic syndrome (MCNS) developed in a 17-year-old female and spontaneously remitted. One month later the nephrotic syndrome relapsed. Prednisolone therapy, 60 mg/day, was started and resulted in a full remission within a week and the prednisolone dose was subsequently tapered. Seven months later, when 10 mg/day of prednisolone ...

Congenital nephrotic syndrome of Finnish type is a rare disease in Taiwan characterized by intrauterine onset of massive urinary loss of protein. We describe a typical baby of congenital nephrotic syndrome with generalized edema occurring at 3 months of age. Renal biopsy at 4 months of age showed a tubular ...

Data compiled during the 1970s and early 1980s indicated that during these periods, membranous nephropathy was the most common cause of unexplained nephrotic syndrome in adults, followed in order of frequency by minimal-change nephropathy and focal segmental glomerulosclerosis (FSGS). However, we and others recently reported an increase in the incidence ...

We describe a bullous-hemorrhagic reaction of the ear lobes in a child with nephrosis after levamisole intake. This drug is used in children as an alternative treatment of corticosteroid-responsive nephrotic syndrome. Histologic evaluation of a lesion revealed necrotizing vasculitis involving mainly the large vessels of the deep plexus. The lesions ...

A syndrome of mutism and subsequent dysarthria occurs frequently in children after resection of a cerebellar tumour. The role of orofacial and speech motor control in this syndrome has not been studied systematically. We examined simple and complex orofacial movements during the mute phase and shortly after the resumption of ...

We report a case of Crohn's disease associated with nephrotic syndrome due to renal amyloidosis in a 21-year-old man in whom remission of both Crohn's disease and the nephrotic syndrome has been maintained with an elemental diet. The patient developed toxic megacolon and nephrotic syndrome due to renal amyloidosis. Intensive ...

Thromboembolic disease and progression to disseminated intravascular coagulation (DIC) are potential life-threatening complications for dogs with nephrotic syndrome. Platelet count, prothrombin time (PT), activated partial thromboplastin time (APTT), plasma concentration of fibrinogen degradation products (FDPs), antithrombin III (ATIII), protein C, and plasminogen were used to identify hemostatic abnormalities in a ...

In July 1994, a 70-year-old woman was diagnosed as having nephrotic syndrome with proteinuria of 8 to 10 g/day and a serum albumin level of 1.8 g/dl. She was hospitalized in August 1994 for investigation. The urinary findings then normalized, with urinary protein and occult blood both negative and total ...

Attachment of podocytes to the glomerular basement membrane is thought to be mediated primarily by alpha 3/beta 1-integrins and by cytoskeletal proteins including actin, talin, vinculin, and alpha-actinin. We analyzed the expression of those molecules in rat glomeruli at several time points during induction of podocyte foot process effacement and ...

The case of a 4-year-old boy with hemophilia B with inhibitor who developed nephrotic syndrome is described. The possible association between factor IX therapy and nephrotic syndrome in patients with hemophilia B is discussed. A chart review of a 4-year-old boy with hemophilia B and an inhibitor who developed nephrotic ...

The number and distribution of glomerular anionic sites using polyethyleneimine (PEI) and the ultrastructural changes in the adjacent glomerular basement membrane (GBM) of 33 children with nephrotic syndrome were studied. Compared to the number of PEI-labelled anionic sites in the lamina rara externa per 1000 nm length of the GBM ...

A 50-year-old male presented with nephrotic syndrome associated with transitional cell carcinoma of the bladder. Renal biopsy was suggestive of membranoproliferative glomerulonephritis. The proteinuria disappeared 4 weeks after the removal of tumour. This is the second reported case of association between nephrotic syndrome and transitional cell carcinoma of the bladder.