Sait Sen

Diagnosis of renal amyloidosis using Congo red fluorescence

Early diagnosis and classification of amyloid deposition and differentiation from other glomerular fibrillar deposits relies on routine Congo red (CR) histochemistry. Congo red fluorescence (CRF) is an alternative method based on examination of the CR‐stained section by ultraviolet (UV) light. The aim of this study is to investigate the usefulness of CRF, especially when applied to frozen kidney sections. Congo red fluorescence was applied to sections of frozen kid‐ney biopsies prospectively and to paraffin sections retrospectively. The findings of CRF were compared to CR staining in bright light. Prospectively, 15 cases of amyloidosis were diagnosed on frozen sections and identical CR staining was found in all of the paraffin‐stained sections. There were no false positives or negatives. Retrospectively, 146 renal biopsies previously stained with CR were re‐evaluated with CRF. Eighty‐seven CR positive cases were confirmed by CRF, and one new case was identified. Congo red fluorescence is simple to perform and more pronounced, therefore easier to evaluate than CR in bright light. Congo red, when combined with immunohistochemistry, is still visible under UV whereas CR is masked in bright light. Although not widely used, the CRF method for detecting amyloid is simple to use with a high specificity and sensitivity, and may be applied successfully to frozen sections.

Plasmapheresis Therapy in Renal Transplant Patients: Five-Year Experience

INTRODUCTION Plasma exchange (PE) and double-filtration plasmapheresis (DFPP) have been used successfully in renal transplant patients as well as those with various other diseases over the last decade. In this retrospective study, we sought to explore the outcomes of plasmapheresis in renal transplant patients. PATIENTS AND METHODS We investigated 58 patients who received PE or DFPP therapy between 2005 and 2010. PE was performed using a Fresenius AS.TEC 204 device and DFPP, by an INFOMED HF 440 device. Indications for therapy, biopsy findings, number of PE/DFPP sessions, laboratory data, medications, complications as well as graft and patient survivals were recorded. RESULTS Overall mean age of subjects was 34.1 ± 8.8 years and 55% were female. Sixteen patients underwent 95 DFPP sessions and 42 underwent 215 PE sessions. Indications for therapy were acute humoral rejection (n = 39), recurrent focal segmental glomerulosclerosis (FSGS; n = 8), thrombotic microangiopathy (n = 6), and chronic humoral rejection (n = 5). Responses to therapy were 24/39 for acute humoral rejection, 1/5 for chronic rejection, 4/8 for FSGS, and 3/6 for thrombotic microangiography. No complication was observed in any patient. CONCLUSION PE/DFPP is a safe and successful method for treatment of acute humaral rejection as less so for recurrent FSGS and thrombotic microangiopathy. The outcomes among subjects with chronic humoral rejection were not satisfactory.

Pauci-immune necrotizing crescentic glomerulonephritis with crescentic and full moon extracapillary proliferation: clinico-pathologic correlation and follow-up study.

The prognostic value of the type and extent of extracapillary proliferation (ECP) in pauci-immune necrotizing crescentic glomerulonephitis (PIGN) was evaluated in this study. In 141 PIGN cases, all glomeruli with ECP were grouped according to type (cellular, fibrocellular and fibrous) and extent of the lesions in Bowmans space; (segmental, semicircumferential and circumferential, which might be termed full moon-FM). Cases with cellular and fibrous lesions involving ≥ 50% of glomeruli with ECP were classified as cellular and fibrous groups, respectively, while the remaining cases were classified as fibrocellular. Cases with segmental and circumferential (FM glomerulus) lesions involving ≥ 50% of glomeruli with ECP were classified as ECPI and ECPIII (FM) groups, respectively, while the rest were classified as ECPII. All the cases were classified according to Berden et al. Significant results were only nearly obtained for the FM group, including the need for dialysis. The Cox regression model revealed a 2.6-fold risk for FM cases regarding dialysis requirement. We propose that the percentage of FM glomeruli should be noted in the pathology report, and cases with more than 50% of FM glomeruli (FM group) should be identified in the group with increased risk of dialysis requirement. Our series also suggests that classification according to Berden et al. is of clinical relevance.

Hepatic amyloidosis: Morphologic spectrum of histopathological changes in AA and nonAA amyloidosis

In hepatic amyloidosis (HA), the relationships between the pattern and extent of amyloid deposition, morphologic changes, associated diseases and clinical data have not yet been demonstrated. In this study, we sought the correlation between the above mentioned parameters in HA. Liver biopsies of 34 HA were retrospectively analyzed for the type, distribution, and intensity of amyloid deposition and associated morphologic changes. AA and nonAA types were classified on the basis of immunohistochemistry. Follow-up clinical and laboratory findings were reviewed. Twenty-three out of 34 patients (67.6%) had AA, and 11 out of 34 patients (32.4%) had nonAA amyloidosis. The predominant localization pattern in AA amyloidosis was vascular (91.3%), and in nonAA amyloidosis it was mixed with other patterns (72.7%). We confirmed that nonAA amyloid involves the hepatic artery, as well as the portal and central vein, but deposition occurred more frequently in the sinusoidal areas. We detected a portal stromal pattern only in cases of nonAA amyloidosis with a mixed pattern of amyloid deposition. The pattern of amyloid deposition in liver differs between the AA and nonAA type amyloidosis. The distribution of amyloid within the liver is not a reliable method for distinguishing AA from nonAA amyloidosis. However, the histological pattern provides strong clues as to the etiology of the amyloid deposits, and could provide information on the clinical status and prognosis of these patients.

Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever

Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy.

Autopsy Findings of a Case with Oxalosis

Oxalosis, deposition of calcium oxalate in tissues, is the final stage of hyperoxaluric syndromes. Being a rare entity, it is often missed, or the diagnosis is delayed, since the definitive diagnosis requires special laboratory tests. Kidneys, the walls of blood vessels, and bones are the major sites for crystal deposition. We report the autopsy findings of a 4-year-old girl who presented with end-stage renal disease in which the clinical presentation was consistent with primary hyperoxaluria Type I. The case is unusual, as there was extensive crystal deposition throughout the body, including in tissues that are rarely involved, such as ovaries, fallopian tubes, uterus, thymus, salivary glands, pancreas, and bladder.

Amyloidosis of seminal vesicles; incidence and pathologic characteristics.

OBJECTIVE Amyloidosis is a rare disease with various etiologies with extracellular amyloid protein depositions. At present, at least 26 distinctive amyloid forms have been detected with different clinical importance and treatment. They have characteristic staning fetaures with Congo red. Amyloid may be detected in 2-10% of prostates that have been removed because of hyperplasia or carcinoma. Amyloidosis of seminal vesicles is accepted as senil amyloidosis and it is not accompanied by systemic amyloidosis or clinical symptoms. This condition is the most common form of localized amyloidosis. In this study we aimed to investigate incidence and histologic characteristics of amyloidosis of seminal vesicles in radical prostatectomy materials of the patients whose prostate carcinomas were treated surgically. MATERIAL AND METHOD Amyloid depositions in seminal vesicles of 207 radical prostatectomy materials that prostates had been removed due to localized prostate carcinoma. Amyloid depositions were confirmed with Congo red staining and polarization microscope. RESULTS Amyloidosis of seminal vesicles was detected in 10 (4.8%) of cases. Mean age of the patients is 66.2 years. Amyloid depositions tend to be nodular and bilateral in subepithelial region of affected seminal vesicles. Amyloid depositions were not detected in blood vessels in seminal vesicles or prostate parenchyma. CONCLUSION Localized amyloidosis of seminal vesicles is not an unusual finding. amyloidosis of seminal vesicles incidence in Turkish patients included in this study and histopathologic characteristics of these patients are not different from the other studies. Systemic AA amyloidosis is the most common form of amyloidosis in our country. To be aware of amyloidosis of seminal vesicles is of importance in discrimination from the other forms of amyloidosis.

Nephrosclerosis and carotid atherosclerosis: Lessons from kidney donor histology

Aim:  Carotid artery intima media thickness (CA‐IMT) measurement has been shown to be a safe and reproducible method to assess severity of atherosclerosis. The association between nephrosclerosis and systemic atherosclerosis is not clear. In this study, we investigated the association between CA‐IMT and nephrosclerosis in a group of kidney transplant donors.

The relationship between renal volume and histology in obese and nonobese kidney donors

Obesity and related kidney diseases have become a global epidemic problem. However, the underlying pathogenesis of obesity‐related renal diseases has not been clearly understood. In this study, we explored the link between renal volume (RV) determined by computed tomography (CT) and renal histology together with functional parameters in an obese population.

Progression of graft fibrosis under mammalian target of rapamycin inhibitor-based regimen

Aim:  Nephrotoxic potential of mammalian target of rapamycin inhibitors (mTORi) is different from calcineurin inhibitors (CNI). The aim of this study is to investigate the interstitial fibrosis (ci) and tubular atrophy (ct) progression from the baseline to first year under a mTORi‐based, CNI‐free regimen.