Nephrotic syndrome complicating treatment with interferon alpha

We report two cases of nephrotic syndrome with minimal glomerular change complicating alpha-interferon therapy. CASE REPORTS: The first patient was a 60-year-old man with Waldenstr?m's disease who was given 1 million units of alpha-interferon three times a week for 22 months. Acute renal failure developed when a second protocol was started. Renal biopsy revealed intraglomerular deposits and no cellular proliferation. Total remission could not be achieved with corticosteroids. The second case was a 46-year-old man given high dose alpha-interferon (15 million units 3 times a week) for lymph node metastasis of a malignant melanoma. A nephrotic syndrome without renal failure developed during the third month of treatment. Minimal glomerular involvement was seen. Symptomatic treatment led to resolution of the nephrotic syndrome. DISCUSSION: Nine other cases of nephrotic syndrome complicating alpha-interferon therapy have been reported in the literature.     

Pseudomonas aeruginosa folliculitis after depilation]

Membranous glomerulonephritis is the most common glomerular disease associated with malignancy, the association of minimal change glomerulopathy with solid tumor is still uncommon. We report a 72-year-old man with nephrotic syndrome due to minimal change glomerular disease; an accurate seek of underlying malignancy revealed a cecum adenocarcinoma. We had a complete remission of nephrotic syndrome after surgery of carcinoma.     

Hodgkin's disease complicated by the nephrotic syndrome

A 12-year-old boy with nodular sclerosis Hodgkin's disease demonstrated nephrotic syndrome. A renal biopsy studied with light microscopy, electron microscopy, and immunofluorescence showed minimal change glomerulonephritis and some foci of fluorescence with anti-IgM. A literature review revealed that 35 cases of Hodgkin's disease with nephrotic syndrome have been reported. Possible etiologic mechanisms are discussed.     

Fatal renal vasculitis and minimal change glomerulonephritis complicating treatment with penicillamine. Report on two cases

Two cases with different and not previously described fatal renal complications during treatment with penicillamine are reported. A man with seronegative rheumatoid arthritis with features of systemic lupus erythematosus was treated with penicillamine for six months and developed a mild membranous glomerulonephritis and a severe renal vasculitis leading to uremia and death. A woman with primary biliary cirrhosis was treated with penicillamine for nine months and developed a nephrotic syndrome, the renal biopsy showing minimal change glomerulonephritis. The nephrotic syndrome responded to prednisone but the patient died, probably from septicemia. Penicillamine may thus cause glomerular damage without deposition of immune complexes. A restricted use of the drug is recommended.     

Strongyloidiasis associated with nephrotic syndrome

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 infection of Strongyloides stercoralis. It is important to rule out strongyloidiasis prior to corticosteroid therapy to patients from eosinophilia endemic areas.     

New taxane diterpenoids from the roots of Taxus mairei

OBJECTIVE: We tested the hypothesis that nitric oxide synthesis by the kidney is increased in children with primary nephrotic syndrome. METHODS: We examined the urinary excretion of nitrite, a stable metabolite of nitric oxide, using the Griess reaction, in children with nephrotic syndrome. RESULTS: In comparison with healthy children, patients with minimal change nephrotic syndrome had increased urinary nitrite excretion regardless of whether the disease was in relapse or remission (p < 0.025). In contrast, urinary nitrite excretion was similar in control subjects and patients with focal segmental glomerulosclerosis or IgA nephropathy. CONCLUSION: These findings indicate that measurement of urinary nitrite excretion may be a useful test to help discriminate between minimal change nephrotic syndrome and focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome.     

Cyclosporine in glomerular disease

Cyclosporine was introduced in 1981 as an immunosuppressive agent in renal transplantation. Its use was soon extended to the treatment of various glomerular disorders. In light of its known immunomodulating effects, the use of cyclosporine has been most prominent in those glomerular diseases thought to have an immune basis. The most careful studies of cyclosporine in glomerular diseases have been performed in the pediatric population with idiopathic nephrotic syndrome (i.e., minimal change disease and focal segmental glomerulosclerosis), although data are accumulating regarding efficacy and safety in adults with idiopathic nephrotic syndrome. In patients who are steroid-dependent, cyclosporine therapy can induce complete or partial remission in a significant proportion of cases; success rates in patients with steroid-resistant nephrotic syndrome are less encouraging. Treatment with cyclosporine allows for dose reduction or elimination of corticosteroids, and the consequent salutary effect on growth in the child and glucose and bone metabolism in all patients. Studies that suggest a potential benefit of cyclosporine in recurrent nephrotic syndrome in renal allografts and in other glomerular diseases are also discussed.     

Diffuse mesangial sclerosis: association with unreported congenital anomalies and placental enlargement

A case of diffuse mesangial sclerosis (DMS) associated with a number of undescribed congenital anomalies is reported. The occurrence of additional anomalies, especially ocular anomalies, is a common finding in DMS. However, neither megalocornea, Dandy-Walker malformation, postaxial hexadactyly, rocker-bottom feet, nor atrial septal defect, as observed in our patient, has been reported previously in association with DMS. This case might be considered an atypical manifestation of the Galloway-Mowat syndrome. In contrast to most cases of DMS, the patient revealed intrauterine proteinuria as the placenta was enlarged to 31% of birth weight. This case demonstrates that the large placenta, >25% of birth weight, is not only pathognomonic of the congenital nephrotic syndrome of the Finnish type but can also occur in DMS.     

Typhoid fever and nephrotic syndrome--an unusual association

We report a case of nephrotic syndrome seen in a 9-year-old boy having a significant temporal relationship with culture proven multidrug resistant Salmonella typhi infection. Such an association is quite unusual and probably has not been reported before.     

Changes of physico-chemical characteristics of red blood cells in children with nephrotic syndrome

Changes of milieu interieur in nephrotic syndrome (NS) have many consequences in various organs. We have measured the electrical charge of erythrocytes (Ery) with binding of alcian blue (AB) in 18 children with relapse of NS (12 minimal changes, 3 membranous and 3 mesangioproliferative glomerulonephritis) and 15 healthy children. The most important finding was that the binding of AB to Ery in patients with minimal change nephrotic syndrome (MCNS) and other aetiologies of NS was significantly less than that in the control group (p < 0.05). In addition, we have studied the thermal denaturation of the Ery membranes by differential scanning microcalorimetry. In some children with NS we have seen the splitting of B transition. We suppose that these phenomena occur as the result of structural change, which may involve lipoprotein components of the cytoskeletal network.     

Glomerular morphology in nephrotic heroin addicts

Renal biopsies of 23 heroin addicts who presented with the nephrotic syndrome were examined by light and electron microscopy. The majority of patients (14) showed focal segmental glomerular sclerosis on light microscopy, four patients showed "minimal change", and two were classified as "focal global sclerosis." In one case focal mesangial proliferation was the outstanding feature; one patient had diabetic glomerulosclerosis; and one had mesangiocapillary glomerulonephritis and dysproteinemia. Visceral epithelial swelling and proliferation were present in 14 patients on light on light microscopy. Electron microscopy showed distinct podocyte changes consisting of loss of foot processes, vacuolization, and cytoplasmic degeneration; focal separation of podocytes from basement membranes was found in 11 of 18 cases. In some instances a few electron-dense deposits were present in the mesangium. Membranous nephropathy was not encountered, although it occurs in 30 to 40% of unselected adult nephrotic individuals. Of 15 patients followed for 2 months to 5 years, one died of heroin overdose, eight went into renal failure, two improved, and four continued to have proteinuria. It is concluded that nephrotic syndrome of heroin addicts is most often associated with focal segmental glomerular sclerosis and occasionally with minimal change disease or focal global sclerosis. Conceivably these three conditions represent different phases of one disease process, although different reactions to heroin or its various vehicles and contaminants cannot be excluded. The morphologic resemblance to experimental aminonucleoside and N,N'-diacetylbenzidine-induced nephrosis suggests a possible toxic origin.     

Nephrotic syndrome, renal failure, and renal malignancy: an unusual tumor-associated glomerulonephritis

The association between malignancy and glomerular disease has been appreciated for over three decades. Although the relationship between membranous glomerulonephritis or minimal-change nephrotic syndrome and carcinoma or lymphoma, respectively, are the most widely known, several other glomerular lesions have been described in patients with malignancy. In this article, a patient who presented with nephrotic syndrome, volume overload, and renal failure, who was subsequently found to have a renal mass, is described. Resection of the mass, which proved to be a renal cell carcinoma, led to resolution of proteinuria and improvement of renal function. Pathology on the noninvolved portion of the kidney revealed a membranoproliferative glomerular lesion, a lesion usually associated with lymphomas and not previously described with renal carcinoma. Although a role of tumor antigens and anti-tumor antibodies in producing glomerular immune deposits has been speculated upon, the evidence for this assertion was spotty. However, reports of remission of proteinuria after tumor treatment or removal support a role of tumor products in pathogenesis. Although the association between proteinuria and malignancy is rare, it should be kept in mind, particularly in older patients with membranous glomerulonephritis where the possibility of malignancy needs to be further evaluated.     

Fatal hyperthrombotic condition complicating nephrotic syndrome

A twenty-year old woman was admitted to hospital with impaired consciousness and a left hemiparesis. A cerebral CAT-scanning was reported normal. Convulsions and respiratory insufficiency made intubation and mechanical ventilation necessary. Laboratory examination showed an extremely low concentration of albumin in plasma (109 microM) and gross albuminuria was present. No other signs of renal failure occurred. The patient deteriorated during three days and died with signs of cerebral incarceration, despite efforts to reduce intracranial pressure. Autopsy showed a large thrombus of the sagittal sinus as the presumed cause of death. No thrombus was found in the renal vein. The association between nephrotic syndrome and thrombosis is discussed.     

Primary Sj?gren's syndrome and glomerulonephritis

HISTORY AND CLINICAL FINDINGS: An 82-year-old woman with hypertension for 20 years developed a nephrotic syndrome with severe oedema followed by acute oliguric renal failure after a bout of bronchitis and a gastrointestinal infection. She also complained of xerostomia and dry eyes of recent onset. INVESTIGATIONS: Biochemical tests showed a serum creatinine level of 6.1 mg/dl, a 1:5120 antinuclear antibody (ANA) titre, and positive values for Ro(SS-A) and La(SS-B) antibodies. HLA-DR typing demonstrated HLA-DR3 (HLA-DRB1*0301) and DR13 (HLA-DRB1*13) antigens. Renal biopsy revealed minimal glomerular lesions with focal and segmental glomerulo-sclerosis as well as (hypertension-induced) benign nephrosclerosis and focal tubular atrophy with interstitial fibrosis. TREATMENT AND COURSE: After two hemofiltrations and concomitant administration of 100 mg prednisone renal function quickly improved and the proteinuria fell to 1 g/dl. At the same time the xerostomia improved. The nephrotic syndrome recurred 7 months later after the prednisone dose had been reduced to 10 mg/d, but after the dose had been raised to 50 mg/d and cyclosporin A (150 mg/d) had been added a lasting remission occurred and renal function became stable though impaired. CONCLUSION: The relatively rare association of glomerular disease (here focal segmental glomerulosclerosis) with Sj?gren's syndrome can, as in this case, be triggered by a viral infection. A genetic predisposition for Sj?gren's syndrome is suggested by the demonstration of HLA-DR3 alleles. Administration of steroids is indicated for the treatment of the nephrotic syndrome and, in case of recurrence, can be combined with cyclosporin A. Both drugs also influence the symptoms of Sj?gren's syndrome.     

Chlorambucil treatment of frequently relapsing nephrotic syndrome

Chlorambucil, in combination with prednisone, was compared with prednisone alone in a randomized controlled trial in 21 children with either steroid-dependent or frequently relapsing nephrotic syndrome to assess its effect on the duration of remission and the rate of relapse. All control patients treated with prednisone alone continued to relapse at the same rate, with all patients experiencing a return of proteinuria by seven months. Conversely, those who received the same prednisone therapy along with chlorambucil for six to 12 weeks remained in complete remission, without further medication, during 12 to 34 months of follow-up observation. Complications were minimal. Immediate side effects commonly reported with cyclophosphamide were not seen with chlorambucil. Comparison with published reports also suggests that remission induced by chlorambucil is more stable than that after cyclophosphamide. Chlorambucil appears to be of value in the frequently relapsing nephrotic patient, adding an effect that is unattainable with prednisone alone.     

Quantitative studies of the kallikrein-prokallikrein system in mixed saliva in oral squamous epithelial carcinoma

We report a 3-year-old girl with the association of spondyloepiphyseal dysplasia, nephrotic syndrome, and signs of defective cellular immunity. The findings are similar to those reported by Spranger et al., which have become known as Schimke immunoosseous dysplasia.     

Complete clinical remission and subsequent relapse of bronchiectasis-related (AA) amyloid induced nephrotic syndrome

Systemic amyloidosis normally has a dismal prognosis. However, there are several case reports of protracted survival, usually as a response to measures designed to retard the further deposition of amyloid fibrils. In AA amyloid, most commonly associated with inflammatory rheumatological, bowel, and chest diseases, such interventions have had some success, but the dramatic response of complete resolution of nephrotic syndrome as a result of the regular institution of postural chest drainage and antibiotic therapy, in the clinical context of bronchiectasis, has been previously reported only once. In both of our cases, after protracted remission, such therapy was abandoned by the patients, leading both to recurrence of nephrotic syndrome and also eventually to end-stage renal failure requiring dialysis.     

Nephrotic syndrome in autosomal dominant polycystic kidney disease

Urinary protein excretion is generally less than 1 g/24 h in autosomal dominant polycystic kidney disease (ADPKD), and the association of the nephrotic syndrome with this condition is considered rare. A patient with ADPKD associated with nephrotic-range proteinuria is described. She exhibited a relatively rapid impairment of her renal function. An open renal biopsy revealed focal segmental glomerulosclerosis (FGS) with features consistent with secondary FGS. Twenty-one patients with ADPKD and nephrotic syndrome were retrieved from the literature. Fourteen of them (including this case) had a histopathologic evaluation, and FGS was the dominant diagnoses (five patients). Next in frequency were minimal-change disease and membranous nephropathy, with two patients each. Five other patients had a variety of diagnoses. Thus, it is difficult to ascertain if these associations are coincidental or represent a specific pathogenetic relationship. The evaluation of the data also suggests that the presence of proteinuria and nephrotic syndrome accelerates the course of ADPKD toward ESRD.     

Variables discriminating between cryptogenic glomerular lesions in adults with the nephrotic syndrome

A univariate analysis of individual clinical and biochemical values of adult patients with cryptogenic nephrotic syndrome has shown that significant differences exist between patients with proliferative glomerulonephritis, 'minimal change' nephritis and membranous nephropathy. For any given adult patient with the condition, the most likely clinical diagnosis is proliferative disease and the next, minimal change. These two diagnoses together account for most cases. The best clinical discriminants between them are the systolic blood pressure and plasma cholesterol concentration. If the systolic pressure is greater than 145 mm Hg proliferative disease is more likely, but if the cholesterol is greater than 530 mgm/100ml, a minimal change lesion is more likely. A scattergram for combining these variables in clinical practice is given, showing a zone of uncertainty where renal biopsy would be indicated. Although single variables do not permit discrimination between membranous nephropathy and the other two groups, it is suggested that analytical techniques where combinations of variables are used may be helpful, and should be developed.     

Type AA renal amyloidosis in sarcoidosis

Sarcoidosis is an uncommon cause of secondary amyloidosis. We describe in this paper the case of a 39 years old patient, with a pulmonary and hepatosplenic sarcoidosis. A nephrotic syndrome led to a renal biopsy which showed AA type amyloidosis. As no other cause of amyloidosis has been found we admitted that it was a result of sarcoidosis which was associated with the unusual inflammatory syndrome.