ALL WGs (743)
BREAST (47)CARD (27)
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DIGE (45)EM (19)
GYNE (49)H&N (75)
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Criteria: EM Found: 19 cases
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Case 1 - ecp25, EM & Nephropathology
Presented by: Mihatsch Michael J., Basel, Switzerland

Introduction to session (not a Case presentation)
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Case 2 - ecp25, EM & Nephropathology
Presented by: Carmen Valbuena, Porto, Portugal

 32 years old female with nephrotic proteinuria (4.5 g/day) and sCr of 1.1 mg/dl.
 She has normal blood pressure and the urine sediments were normal during the last two years.
 She was asymptomatic, and reported no relevant past medical history.
 Two of her brothers, followed elsewhere, had received kidney transplants as young adults, for end stage renal failure attributed to “focal and segmental glomerulosclerosis”.
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Case 3 - ecp25, EM & Nephropathology
Presented by: Rui Miguel Costa, Vila Real, Portugal

Inherited or acquired metabolic disorders are responsible for renal intracellular accumulation of phospholipidis. Ultrastrucutural analysis reveals typical myeloid or zebra bodies. Previously thought to be exclusive in Fabry disease, chloroquine/hydroxychloroquine toxicity can cause the same structures. Recent studies revealed curvilinear bodies in renal cells in those cases, never described in Fabry`s nephropathy. We report a 31-year-old patient with Systemic Erithematous Lupus under long term therapy with hydroxychloroquine. The presence of zebra bodies in electron microscopy lead to initial interpretation of Fabry disease but genetic analysis showed no mutation. Further reevaluation revealed curvilinear bodies in renal cells, supporting hydroxycholoroquine induced renal phospholipidosis.
Diapositivo1Diapositivo2PAS (400x)EM1 (5200x)EM2 (5200x)Diapositivo6EM3 (7500x)
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Case 4 - ecp25, EM & Nephropathology
Presented by: Fernanda Carvalho, Lisbon, Portugal

A 22 year old Caucasian female patient was referred to nephrology consultation for azotemia (serum creatinine: 1.9mg/dl). Urinary sediment was irrelevant and she had no proteinuria, hematuria or hearing loss. She had several close relatives with a diagnosis of chronic kidney disease from unknown causes; some of them were CKD stage 5, either on hemodialysis or transplanted. The patient had a diagnosis of hypertension since she was 16 and hyperuricemia. The renal ultrasound showed kidney of normal dimensions and cortico-medullar differentiation, with bilateral cortical cysts.

In short: Non proteinuric inherited renal phenotype, transmitted in an autosomal dominant pattern.

Affected individuals displayed hyperuricemia/gout and/or renal cysts.
PedigreeSlide 1PASTrichEMUromodControl
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Case 5 - ecp25, EM & Nephropathology
Presented by: Javier Gimeno-Beltran, Spain

This lecture is not a Case presentation.
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Case 6 - ecp25, EM & Nephropathology
Presented by: Helmut Hopfer, Basel, Switzerland

A 39 year old male patient received a deceased donor kidney in 2005 because of renal failure due to hypertensive nephrosclerosis. There were 5 HLA-mismatches and no donor-specific antibodies. Due to malcompliance with his immunosuppressive drugs, he experienced two biopsy proven episodes of interstitial cellular rejections 6 and 35 months after transplantation.

40 months after transplantation a diagnostic biopsy was taken because of a rise in serum creatinine and a newly diagnosed proteinuria (140 mg protein/mmol creatinine; baseline 13).
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Case 7 - ecp25, EM & Nephropathology
Presented by: Eva Honsova , Prague, Czech Republic

A 55 y/o woman was admitted to our hospital to be considered for the second renal transplant. She had agenesis of the left kidney and several stenotic segments in the right ureter which were associated with repeated pyenonephritides during her childhood. Ureteroplastic operation and partial resection of the right kidney were performed when she was 15 year old. Her renal function slowly deteriorated and at the age of 35 she started haemodialysis. A year later she received a cadaveric kidney and that graft functioned for 18 years. She underwent graftectomia and after one year on dialysis she received the second cadaveric kidney. The surgery was followed by delayed graft function, and a renal biopsy was performed on the day 6. C4d was negative and acute T-cell mediated rejection (ATCMR) grade IIA was diagnosed. She was treated with solumedrol. Her renal function slowly improved and the serum creatinine level (S-Cr) stabilized on 170μmol/l (1.9mg/dl). She felt well and came to the hospital 3 months later for a protocol graft biopsy. The morphological features were again consistent with ATCMR grade II (C4d was negative). She was treated with thymoglobulin and S-Cr improved and descended on the level of 110μmol/l. She remained well but her renal function slowly progressed and a new graft biopsy for graft dysfunction (S-Cr 160-200μmol/l) was performed 2 years later. It showed morphology of transplant glomerulopathy in a marginal sample (C4d was negative). Repeated cross match and FACS were negative. She developed progressive anemia and infection by parvovirus B19 was diagnosed (with consistent bone marrow smear and IgM antibodies in serum). Now, 5 years after second transplantation, her S-Cr is around 300μmol/l (3.39mg/dl) and proteinuria has reached 4g/day. In the last graft biopsy sample C4d was negative, transplant glomerulopathy and also interstitial fibrosis with tubular atrophy have progressed.
Fig.1a HE with elastineFig.1b PASFig.2b PAS protocol biopsyFig.3a PASMFig.3b EMFig.2a antiSV40 protocol biopsyFig.4b EM cg
Fig.4a PAS
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Case 8 - ecp25, EM & Nephropathology
Presented by: Jolanta Kowalewska, Bialystok, Poland

The patient was a 49-year-old white male who underwent kidney transplantation 18 moths ago. The cause of his native kidney failure was reported to be hereditary nephritis.

After the transplantation his serum creatinine level was stable at 1.5 mg/dl. Approximately 14 months post transplantation the serum creatinine level increased to 3.3 mg/dl. No proteinuria was noted. The patient underwent a kidney transplant biopsy that showed interstitial lymphoplasmacytic inflammation and tubulitis, C4d(-), and no evidence of vascular rejection. These changes were diagnosed as acute cellular rejection (Banff IA). Treatment with steroids was initiated and resulted in decrease in serum creatinine level to 2.8 mg/dL.

Four months later (18 month post transplantation the serum creatinie again was found to be elevated, this time to 3.8 mg/dL. At this point the main clinical differential diagnosis included refractory rejection vs. cyclosporine toxicity.

The second biopsy was performed.
Image8.EM.native kidney
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Case 9 - ecp25, EM & Nephropathology
Presented by: Johan Mölne, 413 45 Göteborg, Sweden

Male, born 1968. Nephrotic syndrome 1999 and renal biopsy showed a slight membranous pattern by light microscopy. Immunohistochemistry displayed IgG dominant deposits in a membranous pattern and subepithelial deposits were seen on electron microscopy. Diagnosis membranous glomerulonephritis.

Despite immunosuppressive treatment (ACTH, cyclophosphamide, ciclosporin) progression to end stage renal disease and put on dialysis 2007. Living donor transplantation 2008 with AB0 incompatible donor (B to A). Early humoral rejection and never obtained good function, GFR at 1 year 25 ml/min. Creeping creatinine and 2012 asharp increase to 354mmol/L and quickly developed nephrosis.

A renal transplant biopsy was performed.
Slide 1 (PAS)Slide 2 (PAS)Slide 3 (Ag-Jones)Slide 4 (C5b-9)EM 1EM 2EM 3
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Case 1 - ecp24, Electron Microscopy
Presented by: Alessandro Franchi, Firenze, Italy

A 15-year old girl presented with pain of the right knee of 4 month duration, which increased with sport activities. Radiographic examination showed a small exophytic lesion of the posterior aspect of femoral metaphysis, with mixed radiolucent and dense appearance. After surgical biopsy, for which a histological diagnosis of juxtacortical chondromyxoid fibroma was rendered, the lesion was removed with marginal margins. Eighteen months after surgery, the patient noted a swelling at the site of previous surgery. Radiographic examination showed a surface lesion originating from the cortical bone at the site of previous surgery, with irregular, ill-defined calcifications/ossifications. After incisional biopsy confirming recurrent disease, the patient underwent surgical removal of the lesion with wide margins. The patient was free of disease at 10 month follow-up.
Presentation XRay LateralRecurrence - XRay LateralHematoxylin & EosinHematoxylin & Eosin 2cam5.2s100 proteinp63
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