Glomerular tip changes in childhood minimal change nephropathy
Department of Pathology, University College London, London, UK. Pediatric Nephrology
(Impact Factor: 2.86).
09/2008; 23(8):1281-6. DOI: 10.1007/s00467-008-0823-0
Segmental glomerular lesions at the tubular opening, or tip changes, are found in the renal biopsies of adults in many disorders, including some initially considered to show minimal change nephropathy. The hypothesis was that similar tip changes occurred in children. We reviewed a consecutive series of 50 biopsies, diagnosed as minimal change nephropathy, from 49 children. Segmental lesions were found in five biopsies. One biopsy showed lesions at the glomerular hilum. The patient was in remission at follow-up. Four biopsies showed only tip changes. Three patients were in remission, two on no treatment at follow-up, and one on ciclosporin. The other had chronic hepatitis B infection, with persistent proteinuria and segmental lesions at different sites in glomeruli. The other 44 children were nearly all in remission, 18 without treatment at follow-up, and the rest on various immunosuppressants, but one had persistent proteinuria and multiple segmental lesions. Series of childhood minimal change nephropathy, similar to this one, are likely to include cases of the glomerular tip lesion, under the original definition of minimal change nephropathy plus tip changes. This should make little difference in clinical practice, because the clinical course should resemble that of minimal change nephropathy.
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ABSTRACT: The diagnosis of focal segmental glomerulosclerosis (FSGS) is a descriptive pathologic diagnosis that in certain clinical situations (ie, primary or idiopathic) becomes its own disease. The clinical diversity, varied histology, and nonspecific morphologic features of FSGS all contribute to the complexity and problematic nature in making a pathologic diagnosis of FSGS. The definitions of the disease and of the morphologic features have evolved during the last century.
To review historic and morphologic features of FSGS in order to demonstrate a practical approach in achieving a pathologic diagnosis of FSGS on kidney tissue.
In 2004 a working proposal on the pathologic (morphologic) classification of FSGS was published in an attempt to unify the complexity of diagnosing FSGS, and it has shown to be both reproducible and with unique clinical implications for each defined FSGS variant.
An accurate diagnosis of FSGS can be challenging. During the last few decades, numerous new scientific discoveries have enriched our knowledge of pathogenetic mechanisms of nephrotic syndrome. Thus, it is expected there will be a need for a further modification to a morphologic classification and that the pathologist's role in diagnosing FSGS will remain in evolution. This review recapitulates the history of the pathologic diagnosis of FSGS and a current morphologic classification, hopefully opening up a discussion for further modifications that reflect the status of knowledge evolving in the 21st century.
Archives of pathology & laboratory medicine 03/2009; 133(2):217-23. DOI:10.1043/1543-2165-133.2.217 · 2.84 Impact Factor
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ABSTRACT: The literature data point to the possibility of misinterpretation of the term focal segmental glomerulosclerosis, which is used by nephropathologists in histopathologic examination of renal biopsies. This term may reflect the description of nonspecific cicatrisation of glomeruli, or the disease entity characterized by nephrotic syndrome, or gross proteinuria, progression to renal insufficiency, resistance to sterydotherapy and recurrence in transplant kidney The aim of the study to present the difficulties in the histological assessment of glomerular sclerotic lesions, and to emphasize the importance of close cooperation between clinicians and pathologists in the interpretation of morphological diagnosis. The histopatologic diagnosis of focal segmental glomerulosclerosis must contain comment if this term reflects description of the type of glomerular injury or the specific disease entity. Definite diagnosis of focal segmental glomerulosclerosis requires a representative renal biopsy specimen, possibility to evaluate renal tissue using light microscopy, immunofluorescence, electron microscopy and the integration of morphological changes with clinical data. Histological picture of the renal tissue do not allow differentiating idiopathic and secondary focal segmental glomerulosclerosis. The proper interpretation of focal and segmental glomerular sclerotic lesions needs collaboration between nephrologists and pathologists.
Polski merkuriusz lekarski: organ Polskiego Towarzystwa Lekarskiego 06/2010; 28(168):482-5.
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ABSTRACT: The clinical significance of focal segmental glomerulosclerosis (FSGS) tip variant remains unclear. With the aim to determine its clinical and histological features, and natural history, we studied our cases of patients with this glomerular lesion.
This is a retrospective analysis. All native renal biopsies from patients diagnosed as FSGS, between 1998 and 2006, were revised for cases with tip variant. Glomerulosclerosis (GS), segmental lesions and interstitial fibrosis (IF) were quantified. We analysed clinical and follow-up data and compared with cases of FSGS not otherwise specified (NOS).
In 248 primary FSGS cases, 37 corresponded to tip variant (14.9%). Median age was 17 years (range 1-65); 13 (35.1%) patients were <15 years old, and 56.8% were males. All patients had nephrotic proteinuria. At diagnosis, there were no significant differences for age, renal function and proteinuria between cases with NOS and tip variant. IF, GS and percentage of glomeruli with segmental lesions were higher in NOS than GTL (P < 0.01). At follow-up (n = 25), 15 patients received steroids alone, and 10 steroids and a cytotoxic agent. At a median follow-up of 48.7 months (24.3-86.7), 7 patients (28.0%) progressed to chronic kidney disease (CKD), 4 (16.0%) developed end-stage renal disease (ESRD) and 9 (36.0%) had complete remission. In NOS patients (n = 93), 48 (51.6%) developed CKD (P = 0.04), 20 (21.5%) developed ESRD (P = 0.54%) and 13 (14.0%) had complete remission (P = 0.02).
Our work does not demonstrate a clearly favourable prognosis in a group of patients with FSGS tip variant. Although in the tip variant there are less chronic renal tissue damage and CKD, and more frequent complete remission of the nephrotic syndrome, there is an important percentage of patients who develop CKD and ESRD.
Nephrology Dialysis Transplantation 11/2010; 26(7):2215-21. DOI:10.1093/ndt/gfq668 · 3.58 Impact Factor
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