Congenital nephrotic syndrome with ventriculomegaly and a normal karyotype is a rare association. We report four cases, three of which were conceived consecutively by one couple. All the cases were associated with elevated maternal serum alpha-fetoprotein. Renal histology in one fetus demonstrated colloid filled cysts distributed in the corticomedullary area. ...

BACKGROUND: Nephrotic syndrome is characterized by severe proteinuria and sodium and water retention. Although endothelin (ET) 1 can cause natriuresis or antinatriuresis, the role played by ET-1 in proteinuria and in sodium retention due to nephrotic syndrome remains unclear. METHODS: We investigated the role played by the ET-1 system in ...

BACKGROUND: Nephrin is a cell-adhesion protein that is defective in congenital nephrotic syndrome of the Finnish type (CNF). Nephrin is synthesized by the podocytes and is localized to the slit membrane between individual foot processes of the podocytes. Although nephrin is apparently pivotal in the development of CNF, the role ...

OBJECTIVE: To investigate whether the outpatient, syndrome-based approach of the Integrated Management of Childhood Illness (IMCI) protocol could be extended to the inpatient arena to give clear and simple minimum standards of care for poorly resourced facilities. METHODS: A prospective, one-year admission cohort retrospectively compared hypothetical performance of syndrome-based management ...

We present a 28-year-old patient with chronic myeloid leukemia (CML) in chronic phase complicated with nephrotic syndrome. The bone marrow cells revealed the presence of Philadelphia chromosome, the cytogenetic hallmark of CML, that results from a balanced, reciprocal translocation between the long arms of chromosomes 9 and 22, t(9;22)(q34;q11). This ...

The association of malignancy with nephrotic syndrome and renal histopathologic abnormalities is well documented. We report a case of intravascular lymphomatosis (IVL) presenting as nephrotic syndrome which was diagnosed by renal biopsy. Histological examination of the renal biopsy specimens showed dissemination of neoplastic lymphoid cells throughout the glomerular capillary bed. ...

Congenital nephrosis is a rare disease with universally accepted poor prognosis; despite developments in the pathogenesis and management, these children are always a challenge to the caregivers and families. Reported here, is a case of a 6-month-old female infant with infantile nephrotic syndrome, who went into spontaneous resolution within a ...

PURPOSE: To report a case of minimal change nephrotic syndrome (MCNS) after photodynamic therapy using verteporfin. DESIGN: Interventional case report. METHODS: After four cycles of photodynamic therapy, general weakness with generalized edema developed in an otherwise healthy 66-year-old woman, resulting in dyspnea and ascites. Urinalysis showed heavy proteinuria (4+) with ...

Our report discusses a 29 year old female patient with nephrotic syndrome due to lupus nephritis, biopsy-proven World Health Organization classification Types IVb and V that was controlled with low-density lipoprotein (LDL) apheresis. She was initially treated with steroid therapy, including methylprednisolone pulse therapy, and the serological activity of her ...

Pediatricians are often faced with both bad temper and decreased daily activity in children with persistent nephrotic syndrome. These problems, which might affect both mental and physical development, have been explained as a consequence of general fatigue due to systemic edema and/or long-term hospitalization. However, other factors, such as cerebral ...

A 49-year-old Japanese female was initially diagnosed as having monoclonal gammopathy of undetermined significance in June 1993 (IgG lambda: 3,120 mg/dl). Four years later, she developed AL amyloidosis complicated by nephrotic syndrome and renal failure. Before receiving 5 courses of MP therapy (melphalan plus prednisolone), her serum IgG level had ...

Nonsteroidal anti-inflammatory drugs (NSAIDs) are well known to cause fluid and electrolyte abnormalities and renal failure. NSAIDs also may cause an acute allergic interstitial nephritis (AIN) and the nephrotic syndrome, characterized by histologic pathology consistent with minimal change disease in patients with previously normal renal function. The nephrotoxic potential of ...

A 72-year-old man was diagnosed as having nephritic syndrome complicated by Waldenström's macroglobulinemia (WM). A monoclonal IgM lambda protein and decreased serum complements were observed. The renal biopsy disclosed the capillary occluded by thrombi which was stained with IgG, IgA, IgM, C4, lambda light chain and slight kappa light chain ...

A 55-year-old woman presented with Guillain-Barré syndrome, nephrotic syndrome and multiple tubular dysfunction under occupational exposure to an organic solvent, which contained acetone as the principal solvent. In this case, the onset of the nephrotic syndrome and tubular dysfunction coincided with the development of the neurological manifestation. Renal biopsy demonstrated ...

We present a 30-year-old man with nephrotic syndrome presenting with bilateral perirenal massive collection compatible with transudation. After drainage of collections kidney biopsy was performed and the histologic diagnosis was focal and segmental glomerulosclerosis. The patient was treated with cyclophosphamide, prednisone, furosemide and enalapril. After remission of nephrotic syndrome renal ...

Kimura's disease is a chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes of head and neck area or parotid gland. It has a high frequency of an association with nephrotic syndrome. Reported cases of nephrotic syndrome and Kimura's disease were mostly from adult patients ...

A nephrotic patient with membranoproliferative glomerulonephritis type II (MPGN II) was treated with cyclosporin A (CSA) and alternate-day low-dose prednisolone. This patient developed the nephrotic syndrome twice. The second episode of the nephrotic syndrome was steroid resistant, and therefore this patient was treated with a CSA regimen. During treatments with ...

We describe one patient with localized Castleman's disease (CD) of the mixed hyaline vascular and plasma cell type located at the mesentery of the small bowel, associated with systemic amyloidosis and nephrotic syndrome. A true nephrotic syndrome has rarely been reported in patients with CD. In the literature, it has ...

A patient presented with symptoms of cerebellar degeneration and nephrotic syndrome. A work-up at that time failed to reveal an underlying disease; however, 20 months later Hodgkin's disease was diagnosed. Hodgkin's lymphadenopathy developed 2 wk after prednisone therapy for the nephrotic syndrome had been discontinued. Systemic polychemotherapy resulted in complete ...

We present a 13-year-old boy who developed hyperthyroidism during the clinical course of idiopathic nephrotic syndrome treated with glucocorticoid. He had a second relapse of minimal change nephrotic syndrome, and complete remission of nephrotic syndrome was achieved immediately with oral glucocorticoid. However, when the steroid dosage was reduced, signs of ...

The hematological malignancies associated with nephrotic syndrome are mainly Hodgkin's and non-Hodgkin's lymphomas and chronic lymphocytic leukemia. Acute myelogenous leukemia (AML) has rarely been described in association with the nephrotic syndrome. The clinical course of a 44-year-old patient with AML who presented with nephrotic syndrome is described and the clinicopathologic ...

Recent clinical reports have demonstrated that the progression and prognosis of renal diseases are possibly influenced by apolipoprotein E (apoE) genotypes and alleles. In this study we investigated whether apoE genotypes and alleles can be a prognostic criterion for the steroid responsiveness in childhood nephrotic syndrome. One hundred and seven ...

Strongyloides stercoralis is endemic in the southwestern islands Amami and Ryukyu in Japan. Systemic strongyloidiasis occurs in immunocompromised hosts. We report here on a 60-year-old patient with minimal-change nephrotic syndrome (MCNS) without eosinophilia or HTLV-I infection. She was treated with corticosteroid for MCNS and died of disseminated strongyloidiasis. The patient ...

Arterial and venous thrombotic events can lead to severe complications in the nephrotic syndrome, but may remain clinically silent in a substantial proportion of patients. Intracardiac thrombi associated with multiorgan thrombosis have been described in autopsy cases of the earlier literature, but have never been documented in vivo. We here ...

Meniere's syndrome is defined as the clinical disorder associated with the histopathological finding of endolymphatic hydrops (ELH). Clinically, Meniere's syndrome includes the following features: recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Recognized causes of Meniere's syndrome include: 1) idiopathic, also known as Meniere's disease; 2) posttraumatic, following ...

Lithium-induced nephrotic syndrome is a rare complication of lithium therapy and is even rarer in children. Most reported cases have been secondary to minimal change disease, which reverses within 1-4 weeks on discontinuation of lithium therapy. However, focal glomerulosclerosis (FSGS) has occasionally been reported and is not always reversible with ...

OBJECTIVE: It is widely accepted that minimal change nephrotic syndrome is the most common cause of nephrosis in children. Recent studies have demonstrated an increasing incidence of focal segmental glomerulosclerosis in adults and children. This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic syndrome in ...

The pharmacokinetics (PK) and safety of fluvastatin, a hydroxymethylglutaryl-coenzyme A reductase inhibitor, were assessed in subjects with renal impairment and nephrotic syndrome. In a single-center, open-label, parallel-group study, a single dose of fluvastatin 40 mg was administered to subjects (8 per group, n = 48) with nephrotic syndrome (group II), ...

Oedema is the commonest presenting symptom and sign in nephrotic syndrome. Hypercholesterolaemia, thromboembolic events, and infectious complications may also be features. Three patients are described, each of whose nephrotic syndrome presented with a less common symptom or sign--recurrent pleural effusion, hypercholesterolaemia and oedema, pulmonary embolism--and, as a result, experienced some ...

Extramedullary hematopoiesis being an important feature of agnogenic myeloid metaplasia (AMM), a chronic myeloproliferative disease of clonal origin, may affect the kidneys, but this condition is usually asymptomatic. Until now, there is only one reported case of nephrotic syndrome associated with AMM. We present a patient with AMM who had ...

We describe here the clinicopathological findings in a child with congenital nephrotic syndrome (CNS) non-responsive to medical therapy who developed acquired cystic kidney disease (ACKD) in both native kidneys after long-term peritoneal dialysis. This case indicates that CNS is a further pathologic condition related to the development of ACKD.

BACKGROUND: Thromboembolism is known as a major complication of nephrotic syndrome, but only 4 cases of portal vein thrombosis have been reported as a complication of nephrotic syndrome. All of these 4 cases had acute symptoms, and 3 of 4 were in relapsing phase of nephrotic syndrome when thrombi were ...

Galloway-Mowat syndrome is an autosomal recessive disorder characterized by early onset nephrotic syndrome and central nervous system anomalies. Mutations in podocyte proteins, such as nephrin, alpha-actinin 4, and podocin, are associated with proteinuria and nephrotic syndrome. The genetic defect in Galloway-Mowat syndrome is as yet unknown. We postulated that in ...

We report a 12-year-old child with multiple autoimmune components consisting of minimal change nephrotic syndrome (MCNS), Hashimoto's thyroiditis and vitiligo. This boy presented with MCNS, complicated by acute renal failure, and subsequently developed Hashimoto's thyroiditis and vitiligo. Conventional prednisone treatment did not induce remission, but multiple high dose infusions of ...

On a database of the Japanese medical literature between 1970 and 2000, there were 29 patients with West syndrome showing spontaneous remission. In 25/29 (86%) of the patients, spontaneous remission was preceded by viral infections, among which exanthema subitum predominated. In 16/29 (55%), the spasms recurred a median of 2 ...

Although infectious complications of nephrotic syndrome are common, group B Streptococcus is a rare pathogen in these patients. We present a 4-year-old child with nephrotic syndrome who developed group B streptococcal cellulitis and bacteremia, an association not previously discussed in the literature, and review the factors that predispose patients with ...

A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is ...

The nephrotic syndrome is an extremely rare occurrence in systemic sclerosis (SSc). Here we describe a 39-year-old woman with SSc who developed the nephrotic syndrome along with the expression of antiribosomal P antibody but not of anti-double-stranded DNA antibody in her serum. Although a renal biopsy specimen showed minimal changes ...

We report on a patient with nephrotic syndrome with myasthenia gravis and malignant thymoma status post thymectomy and radiotherapy. This 44-year-old woman underwent a thymectomy and localized radiotherapy for invasive thymoma in 1991. She also took azathioprine and pyridostigmine regularly for the control of her symptoms of myasthenia gravis following ...

It is widely accepted that amyloidosis in Waldenström's macroglobulinemia (WM) is exclusively due to amyloid light-chain deposition. However, only a small number of previous reports have actually characterized the type of amyloid in WM. We now report the third patient with WM and amyloid A protein (AA) amyloidosis. This patient ...

Nephrotic syndrome is characterized by marked urinary excretion of albumin and other intermediate-size plasma proteins. This results in a profound alteration of the metabolism of many plasma proteins and protein-bound substances, as well as certain cellular and tissue proteins. This review summarizes available data on the effect of nephrotic syndrome ...

We report the finding of an absent cerebellar hemisphere and partial absence of the cerebellar vermis in a child with dysmorphic features, spondyloepiphyseal dysplasia, steroid resistant nephrotic syndrome secondary to focal segmental glomerulosclerosis and T-cell lymphopenia (Schimke immuno-osseous dysplasia). These findings have not, to our knowledge, been described before and ...

Many acquired and familial renal diseases in man lead to kidney dysfunction and nephrotic syndrome. These diseases share a common pathological fate in the form of glomerular dysfunction and proteinuria. Classification of the disease is difficult because the onset of pathological appearance in congenital nephrotic syndrome (CNS) varies considerably. Recently, ...

Malignant mesothelioma can present insidiously with progressive breathlessness and chest pain. Paraneoplastic, or non-chest related, presentations are very rare. The case of an elderly man with occupational exposure to asbestos who presented with nephrotic syndrome due to minimal change nephropathy in the context of advanced pleural mesothelial malignancy is reported.

We present four cases with nephrotic syndrome, microcephaly and severe developmental delay. In the differential diagnosis the Galloway-Mowat syndrome, PEHO syndrome, ARC syndrome and the carbohydrate-deficient glycoprotein (CDG) syndrome are considered and discussed. One case may fall into the Galloway-Mowat spectrum and another case was diagnosed with the CDG syndrome. ...

A 7-year-old boy with moyamoya disease developed sustained hypertension, nephrotic syndrome, hyperreninemia, and occlusion of the right renal artery. After right nephrectomy, hyperreninemia and hypertension improved. Proteinuria was resolved after nephrectomy, in parallel with the decrease in plasma renin activity. Moyamoya disease can cause nephrotic-range proteinuria, which is caused hemodynamically ...

The peristaltic movement of the gut is a function of the alternating contraction and relaxation of circular and longitudinal muscles. This movement is induced by a tendon-like connective-tissue net (TCTN) in the circular and longitudinal muscles, which are both rooted in a connective-tissue plexus layer (CTPL). In children with a ...

A case of human ehrlichiosis (caused by infection with Ehrlichia chaffeensis) is presented. The patient was a female Naval Academy midshipman with a 26-day history of daily field training with the U.S. Marines near Quantico, Virginia. She presented with a several-day history of myalgias, fever, and frontal headache. During her ...

A common form of methicillin-resistant Staphylococcus aureus (MRSA) associated glomerulonephritis is either an endocapillary proliferative glomerulonephritis or a crescentic glomerulonephritis. This report describes the development of reversible nephrotic syndrome following MRSA infection in a patient with amyloid A amyloidosis. The patient had been diagnosed as having rheumatoid arthritis for 50 ...

The aim of this study was to investigate the human leukocyte antigen (HLA) profile of children with nephrotic syndrome in the southern part of Turkey. Seventy-eight children with nephrotic syndrome were studied for the frequency of class I and class II human leukocyte antigens. Forty-seven of them were steroid sensitive ...