Nephrotic syndrome and fibrillary glomerulonephritis

Laufer, J; Augarten, A.; Szeinberg, Amir; Rapoport, Jayson; Katzenelson, D; Yahav, Yaacov

doi:10.1046/j.1365-2796.1997.00164.x

Cited by 6 publications

(3 citation statements)

References 12 publications

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“…Due to the increase in patient lifespans, previously unobserved disease associations have been detected. Renal involvement in CF patients is rare, but recently, renal pathologies such as glomerulosclerosis, mesangial proliferation, nephrocalcinosis and microscopic hematuria, tubular damage, fibrillary glomerulonephritis 13) , and amyloidosis 9) have been reported.…”

Section: Discussionmentioning

confidence: 99%

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

et al. 2013

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Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

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Section: Discussionmentioning

confidence: 99%

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

et al. 2013

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“…Several anatomical and pathological reports describe renal abnormalities in association with CF although there is still a gap in clinical reporting. Glomerular alterations including glomerulosclerosis [5], deposits of immune complexes [6], IgA nephropathy [7] and mesangial proliferations, nephrocalcinosis and microscopic hematuria [8], tubular injury [5,9], diabetic nephropathy [10], fibrillary glomerulonephritis [11] and amyloidosis [12,13] have all been described in patients with CF. There are to our knowledge, only two cases that of nephrotic syndrome related to minimal change disease [14,15] which have been reported in the medical literature.…”

Section: Discussionmentioning

confidence: 99%

Cystic fibrosis and renal disease: a case report

Al-Shawwa

Rao

2007

J Med Case Reports

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BackgroundCystic fibrosis (CF) is an autosomal recessive disease that is predominantly seen in the Caucasian population and involves multiple organs. Traditionally it has been thought that the kidney is the only organ which does not seem to be generally affected by the disease although the cystic fibrosis transmembrane conductance regulator (CFTR) gene is expressed in the kidney.Case presentationWe report the case of an 11 year old boy with cystic fibrosis and nephrotic syndrome and review the literature that describes nephrotic syndrome and renal involvement in cystic fibrosis.ConclusionWith continued advances in the management of cystic fibrosis and improvement in life expectancy, several unrecognized co-morbidities are expected to emerge. It is important to screen patients for possible co-morbidities. Urine analysis may be helpful in this group of patients and any proteinuria should raise the suspicion of cystic fibrosis-related renal disease.

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“…IT combined with lymphoproliferative disease and other diseases in adult patients has been reported. Diseases combined with IT have included malignant lymphoma, 36–38 cystic fibrosis, 39 HCV hepatitis, 15,20,40,41 HIV infections, 42,43 cytomegalovirus infections, 44 amyloidosis (combination of lambda amyloidosis and monoclonal lambda FGN), 45 gastric cancer, 46 diabetes mellitus, 47,48 chronic myelocytic leukemia, 49 idiopathic hypereosinophilic syndrome, 50 and familial Mediterranean fever 51 . In children, besides reports of primary IT/FGN, 52,53 there have been other reports of Down syndrome, 54 sickle‐cell anemia, 55 Charcot–Marie–Tooth disease, 56 and factor H deficiency 57 .…”

Section: Classification Of Gddmentioning

confidence: 99%

Pathology of glomerular deposition diseases

Joh

2007

Pathology International

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In routine diagnosis on renal biopsy, one of the confusing fields for pathological diagnoses is the glomerulopathies with fibrillary structure. The primary glomerulopathies with a deposit of ultrastructural fibrillary structure, which are negative for Congo-red stain but positive for immunoglobulins, include fibrillary glomerulonephritis and immunotactoid glomerulopathy. Several paraproteinemias including cryoglobulinemia, monoclonal gammopathy, and light chain deposition disease as well as hematopoietic disorders including plasmacytoma, plasma cell dyscrasia, and B cell lymphoproliferative disorders involve glomerulopathy with an ultrastructural fibrillary structure. A rare glomerulopathy with fibrillary structure that stains negative for Congo-red as well as for immunoglobulins has been also reported. The pathological diagnoses of these glomerulopathies can include either glomerular diseases, or paraproteinemic diseases, or hematopoietic diseases. The terminology is still confusing when glomerular diseases can be combined with paraproteinemic diseases and/or hematopoietic diseases. Therefore, the generic term, 'glomerular deposition disease' (GDD), has been proposed by pathologists with a requirement for clinicians to detect autoantibodies, paraproteins as well as to carry out a bone marrow check. An attempt has been made to rearrange the diseases with related disorders of fibrillary deposits, based on detailed clinical and pathological finding and to elucidate the correlation between GDD, paraproteinemia, and hematopoietic disorder.

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Nephrotic syndrome and fibrillary glomerulonephritis

Cited by 6 publications

References 12 publications

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

Cystic fibrosis and renal disease: a case report

Pathology of glomerular deposition diseases

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