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1.
Magn Reson Med Sci ; 23(2): 153-160, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-36754388

ABSTRACT

PURPOSE: Despite the usefulness of blood oxygenation level-dependent (BOLD) MRI in assessing glomerulonephritis activity, its relationship with histological findings remains unclear. Because glomerulonephritis presents multiple complex injury patterns, analysis of each pattern is essential. We aimed to elucidate the relationship between the histological findings of the kidney and BOLD MRI findings in mesangial proliferative glomerulonephritis. METHODS: Children under 16 years of age diagnosed with mesangial proliferative glomerulonephritis by kidney biopsy at our university hospital between January 2013 and September 2022 were included in this study. Cortical and medullary spin relaxation rate (R2*) values were measured using BOLD MRI at 3T within two weeks before and after the kidney biopsy. The R2* values, including the fluctuations with low-dose oxygen administration, were retrospectively examined in relation to the cortical (mesangial proliferation, endothelial cell proliferation, crescent, sclerosis, and fibrosis) and medullary findings (fibrosis). RESULTS: Sixteen times kidney biopsies were performed for glomerulonephritis during the study period, and one patient was excluded because of comorbidities; the remaining 14 patients included six boys with a mean age of 11.9 ± 3.5 years at the BOLD examination. None of the patients had medullary fibrosis. Among the kidney tissue parameters, only sclerosis showed a significant correlation with R2* values: medulla with R2* values under atmospheric pressure (r = 0.53, P < 0.05) and cortex with the rate of change in R2* values with low-dose oxygen administration (r = -0.57, P < 0.03). In the multiple regression analysis, only sclerosis was an independent contributor to the change in R2* values with oxygen administration in the cortex (regression coefficient -0.109, P < 0.05). CONCLUSION: Since the R2* values reflect histological changes in the kidney, BOLD MRI may facilitate the evaluation of mesangial proliferative glomerulonephritis, potentially reducing the patient burden.


Subject(s)
Glomerulonephritis , Oxygen , Male , Child , Humans , Adolescent , Retrospective Studies , Sclerosis , Kidney/diagnostic imaging , Magnetic Resonance Imaging , Glomerulonephritis/diagnostic imaging , Fibrosis
2.
Int J Mol Sci ; 23(15)2022 Jul 24.
Article in English | MEDLINE | ID: mdl-35897725

ABSTRACT

A clear identification of the etiology of glomerular disease is essential in patients with diabetes. Renal biopsy is the gold standard for assessing the underlying nephrotic pathology; however, it has the risk for potential complications. Here, we aimed to investigate the feasibility of urinary fluorescence imaging using an enzyme-activatable probe for differentiating diabetic kidney disease and the other glomerular diseases. Hydroxymethyl rhodamine green (HMRG)-based fluorescent probes targeting gamma-glutamyl transpeptidase (GGT) and dipeptidyl-peptidase (DPP) were used. Urinary fluorescence was compared between groups which were classified by their histopathological diagnoses (diabetic kidney disease, glomerulonephritis, and nephrosclerosis) as obtained by ultrasound-guided renal biopsy. Urinary fluorescence was significantly stronger in patients with diabetic kidney disease compared to those with glomerulonephritis/nephrosclerosis after DPP-HMRG, whereas it was stronger in patients with nephrosclerosis than in patients with glomerulonephritis after GGT-HMRG. Subgroup analyses of the fluorescence performed for patients with diabetes showed consistent results. Urinary fluorescence imaging using enzyme-activatable fluorescence probes thus represents a potential noninvasive assessment technique for kidney diseases in patients with diabetes.


Subject(s)
Diabetes Mellitus , Diabetic Nephropathies , Glomerulonephritis , Nephrosclerosis , Diabetic Nephropathies/diagnostic imaging , Fluorescent Dyes , Glomerulonephritis/diagnostic imaging , Humans , Optical Imaging/methods , Rhodamines , gamma-Glutamyltransferase
3.
Abdom Radiol (NY) ; 47(1): 288-296, 2022 01.
Article in English | MEDLINE | ID: mdl-34633496

ABSTRACT

BACKGROUND: Renal parenchymal fibrosis is the most important determinant of kidney disease progression and it is determined via biopsy. The aim of this study is to evaluate the renal stiffness noninvasively by magnetic resonance elastography (MRE) and to compare it with clinicopathologic parameters in glomerulonephritis and AA amyloidosis patients. METHODS: Thirty-four patients with glomerular filtration rate (GFR) over 20 ml/min/1.73m2 had non-contrast MRE prospectively. Kidney stiffness values were obtained from whole kidney, cortex, and medulla. Values were correlated with GFR, albuminuria, proteinuria, and degree of fibrosis that are assessed via renal biopsy. Patients were grouped clinicopathologically to assess the relation between stiffness and chronicity. RESULTS: Mean whole kidney, cortex, and medulla stiffnesses were 3.78 (± 1.26), 3.63 (± 1.25), and 4.77 (± 2.03) kPa, respectively. Mean global glomerulosclerosis was 22% (± 18%) and median segmental glomerulosclerosis was 4% (min-max: 0%-100%). Extent of tubulointerstitial fibrosis was less than 25% in 26 of the patients (76.5%), 25%-50% in 6 of the patients (17.6%), and higher than 50% in 2 of the patients (5.9%). Fourteen patients were defined to have chronic renal parenchymal injury. MRE-derived stiffness values correlated negatively with parameters of fibrosis. Lower stiffness values were observed in patients with chronic renal injury compared to those without (P < 0.05 for whole kidney and medulla MRE-derived stiffness). CONCLUSION: MRE-derived stiffness values were lower in patients with chronic injury. Stiffness decreases as glomerulosclerosis and tubulointerstitial fibrosis progresses in patients with primary glomerulonephritis and AA amyloidosis. With future studies, there may be a role for MRE to assess renal function in concert with conventional markers.


Subject(s)
Elasticity Imaging Techniques , Glomerulonephritis , Amyloidosis , Fibrosis , Glomerulonephritis/complications , Glomerulonephritis/diagnostic imaging , Humans , Liver Cirrhosis/pathology , Magnetic Resonance Imaging , Serum Amyloid A Protein
4.
MAGMA ; 34(6): 823-831, 2021 Dec.
Article in English | MEDLINE | ID: mdl-34275036

ABSTRACT

OBJECTIVE: Children are often sedated for renal blood oxygenation level-dependent (BOLD) magnetic resonance imaging (MRI) and may require low-dose oxygen administration. It is unclear whether low-dose oxygen administration affects results of BOLD MRI. We investigated the effect of low-dose oxygen administration on renal BOLD MRI and its variation by the presence or absence of renal disease. MATERIALS AND METHODS: We retrospectively examined children undergoing MRI for renal disease between 2013 and 2020. Patients were divided into glomerulonephritis and non-glomerulonephritis groups; spin relaxation time (T2*) was determined using a 3.0 T MRI system. RESULTS: The study included 10 children (5 patients in each group); patient characteristics between the groups did not differ significantly. In the entire cohort, oxygen administration reduced mean spin relaxation rate (R2*) value in the medulla (p < 0.04). The mean R2* value decreased with oxygen administration in the non-glomerulonephritis group, whereas this was not observed in the glomerulonephritis group. The responses to oxygen administration of the two groups differed significantly in the cortex (p < 0.05) and medulla (p < 0.02). DISCUSSION: Low-dose oxygen administration affects the results of BOLD MRI. We suggest that understanding the fluctuations due to oxygen administration is useful in monitoring the disease activity of glomerulonephritis.


Subject(s)
Glomerulonephritis , Oxygen , Child , Glomerulonephritis/diagnostic imaging , Humans , Kidney/diagnostic imaging , Magnetic Resonance Imaging , Retrospective Studies
5.
BMC Neurol ; 21(1): 278, 2021 Jul 13.
Article in English | MEDLINE | ID: mdl-34256721

ABSTRACT

BACKGROUND: Idiopathic intracranial hypertension (IIH) is defined by an increased cerebrospinal fluid pressure in the absence of inflammation, structural obstructions, or mass lesions. Although the underlying pathogenesis of IIH is not fully understood, associations with specific risk factors as obesity, obstruction of cerebral venous sinuses, medications, endocrine or systemic conditions and chronic kidney disease have been described. Immune-complex glomerulonephritis as IgA-nephropathy is a frequent cause of chronic kidney failure, which was reported previously in one IIH patient. To date, there is no knowledge about the variable relation of immune-complex nephritis, kidney function and the course of IIH. CASE PRESENTATION: We report three cases (two females) of concurrent diagnosis of IIH and immune-complex glomerulonephritis. All patients presented with typical IIH symptoms of headache and visual disturbances. Two patients had been diagnosed with IgA-nephropathy only few weeks prior to IIH diagnosis. The third patient had been diagnosed earlier with terminal kidney failure due to a cryoglobulin glomerulonephritis. CONCLUSION: We propose a possible link between renal deposition of immune-complexes and increased cerebrospinal fluid pressure. Pathophysiological hypotheses and clinical implications are discussed. We recommend clinical awareness and further systematic research to obtain more information on the association of IIH and immune-complex glomerulonephritis.


Subject(s)
Glomerulonephritis/complications , Glomerulonephritis/diagnostic imaging , Immune Complex Diseases/complications , Immune Complex Diseases/diagnostic imaging , Pseudotumor Cerebri/complications , Pseudotumor Cerebri/diagnostic imaging , Adult , Cranial Sinuses/diagnostic imaging , Female , Headache/diagnostic imaging , Headache/etiology , Humans , Male , Obesity/complications , Obesity/diagnostic imaging , Risk Factors , Vision Disorders/diagnostic imaging , Vision Disorders/etiology , Young Adult
6.
CEN Case Rep ; 10(4): 527-536, 2021 11.
Article in English | MEDLINE | ID: mdl-33905105

ABSTRACT

We had a 72-year-old man with advanced gastric cancer, poorly differentiated adenocarcinoma, receiving chemotherapy with S-1 (tegafur, gimeracil, and oteracil potassium) plus oxaliplatin. Ascites developed despite remission of gastric cancer and metastasis. Given no malignant cells in ascites, leg edema, renal impairment, hypoalbuminemia, and massive proteinuria, we diagnosed as nephrotic syndrome with microscopic hematuria. Renal biopsy showed membranoproliferative glomerulonephritis with no deposition of immunoglobulins and complements. Of note, electronic microscopy found organized deposits with microtubular structures in the glomerular capillary lumens and subendothelial spaces. The liquid chromatography-tandem mass spectrometry method detected fibrinogen alpha chain, beta chain, gamma chain, and fibronectin, and we eventually diagnosed cryofibrinogen-associated glomerulonephritis. Cryofibrinogen was not detected in plasma. He was expired at 5 months following renal biopsy due to the progression of refractory nephrotic syndrome. In addition to the detailed assessment of specifically organized deposits, the analysis using liquid chromatography-tandem mass spectrometry method is useful to diagnose cryofibrinogen-associated glomerulonephritis. We should consider cryofibrinogen-associated glomerulonephritis as a differential diagnosis when the patients with malignancy showed abnormal urinalysis and renal impairment, though it is a rare disease.


Subject(s)
Adenocarcinoma/complications , Cryoglobulins/metabolism , Fibrinogens, Abnormal/metabolism , Glomerulonephritis/etiology , Kidney/metabolism , Stomach Neoplasms/complications , Aged , Glomerulonephritis/diagnostic imaging , Glomerulonephritis/pathology , Humans , Kidney/ultrastructure , Male , Tomography, X-Ray Computed
7.
Pediatr Nephrol ; 35(11): 2113-2120, 2020 11.
Article in English | MEDLINE | ID: mdl-32556960

ABSTRACT

BACKGROUND: To evaluate the efficacy of adjuvant systemic corticosteroids in reducing kidney scarring. A previous study suggested that use of adjuvant systemic corticosteroids reduces kidney scarring in children radiologically confirmed to have extensive pyelonephritis. Efficacy of corticosteroids for children with febrile urinary tract infection (UTI) has not been studied. METHODS: Children aged 2 months to 6 years with their first febrile UTI were randomized to corticosteroids or placebo for 3 days (both arms received antimicrobial therapy); kidney scarring was assessed using 99mTc-dimercaptosuccinic acid kidney scan 5-24 months after the initial UTI. RESULTS: We randomized 546 children of which 385 had a UTI and 254 had outcome kidney scans (instead of the 320 planned). Rates of kidney scarring were 9.8% (12/123) and 16.8% (22/131) in the corticosteroid and placebo groups, respectively (p = 0.16), corresponding to an absolute risk reduction of 5.9% (95% confidence interval: - 2.2, 14.1). CONCLUSION: While children randomized to adjuvant corticosteroids tended to develop fewer kidney scars than children who were randomized to receive placebo, a statistically significant difference was not achieved. However, the study was limited by not reaching its intended sample size. CLINICAL TRIAL REGISTRATION: Clinicaltrials.gov , NCT01391793, Registered 7/12/2011 Graphical abstract.


Subject(s)
Adrenal Cortex Hormones/administration & dosage , Glomerulonephritis/prevention & control , Urinary Tract Infections/drug therapy , Adjuvants, Pharmaceutic/administration & dosage , Adjuvants, Pharmaceutic/adverse effects , Adrenal Cortex Hormones/adverse effects , Age Factors , Anti-Bacterial Agents/therapeutic use , Child, Preschool , Double-Blind Method , Female , Fever , Glomerulonephritis/diagnostic imaging , Humans , Infant , Male
8.
Indian J Pathol Microbiol ; 63(Supplement): S47-S49, 2020 Feb.
Article in English | MEDLINE | ID: mdl-32108627

ABSTRACT

Collagenofibrotic glomerulopathy (CFG) is a rare idiopathic kidney disease characterized by abnormal deposition of atypical Type III collagen fibers in the glomerulus causing subendothelial and mesangial expansion, manifesting as progressive renal dysfunction accompanied by proteinuria. The majority of CFG cases reported in literature are from Japan where this disease entity was initially recognized. There is an increased awareness and diagnosis of this rare renal disease in India with the recent increase in utilization of electron microscopy (EM) in clinical diagnostic settings. We describe a 28-year-old Bangladeshi woman who presented with hypertension and nephrotic range proteinuria not amenable to treatment with steroids and cyclophosphamide, whose renal biopsy demonstrated diagnostic ultrastructural features of CFG. This illustrative case is presented to highlight the role of EM analysis for diagnostic accuracy in renal biopsy evaluation in addition to demonstrating the unusual renal biopsy findings of this rare entity.


Subject(s)
Collagen Type III/analysis , Glomerulonephritis/diagnostic imaging , Kidney Diseases/diagnostic imaging , Kidney Glomerulus/pathology , Rare Diseases/diagnostic imaging , Adult , Biopsy , Female , Fibrosis , Humans , India , Kidney/pathology , Microscopy, Electron, Transmission , Proteinuria/etiology , Rare Diseases/pathology
9.
Radiology ; 292(3): 676-684, 2019 09.
Article in English | MEDLINE | ID: mdl-31287390

ABSTRACT

Background Glomerulonephritis refers to renal diseases characterized by glomerular and tubulointerstitial fibrosis. Multifrequency US time-harmonic elastography enables the noninvasive quantification of tissue elasticity. Purpose To assess the diagnostic performance of US time-harmonic elastography for the early detection of glomerulonephritis. Materials and Methods From August 2016 through May 2017, study participants with biopsy-proven glomerulonephritis were prospectively examined with US time-harmonic elastography. Participants were subdivided according to chronic kidney disease (CKD) stage. All participants underwent elastography of both kidneys to generate full-field-of-view maps of renal shear wave speed (SWS). SWS was determined separately for the whole renal parenchyma, cortex, and medulla and was correlated with quantitative B-mode findings such as renal length and parenchymal thickness. Diagnostic performance of renal elastography was assessed with receiver operating characteristic curve analysis. Results Fifty-three participants with glomerulonephritis (mean age ± standard deviation, 49 years ± 14) and 30 healthy volunteers (mean age, 37 years ± 11) were evaluated. Age-adjusted renal SWS was lower in participants with glomerulonephritis than in healthy volunteers in the parenchyma, cortex, and medulla, with mean values of 1.55 m/sec (95% confidence interval [CI]: 1.51 m/sec, 1.59 m/sec) and 1.69 m/sec (95% CI: 1.64 m/sec, 1.74 m/sec; P < .001), respectively, in parenchyma, 1.80 m/sec (95% CI: 1.75 m/sec, 1.84 m/sec) and 2.08 m/sec (95% CI: 2.02 m/sec, 2.13 m/sec; P < .001) in cortex, and 1.25 m/sec (95% CI: 1.21 m/sec, 1.29 m/sec) and 1.33 (95% CI: 1.27 m/sec, 1.38 m/sec; P = .03) in medulla. Age-adjusted renal cortex SWS was lower in participants with glomerulonephritis and stage 1 CKD (preserved renal function) than in healthy volunteers (mean, 1.88 [95% CI: 1.81, 1.96] vs 2.08 [95% CI: 2.02, 2.13]; P < .001). In participants with CKD, renal cortex SWS values showed a positive association with estimated glomerular filtration rate (n = 39; r = 0.56; P < .001). Exploratory diagnostic performance of US time-harmonic elastography (area under the receiver operating characteristic curve [AUC], 0.89; 95% CI: 0.82, 0.97) outperformed that of B-mode parameters such as parenchymal thickness (AUC, 0.64; 95% CI: 0.51, 0.77; P < .001) and renal length (AUC, 0.55; 95% CI: 0.40, 0.68; P < .001) in identifying glomerulonephritis. Conclusion US time-harmonic elastography depicts abnormal renal stiffness in glomerulonephritis, particularly among patients with early disease and preserved renal function. Advanced chronic kidney disease is associated with further cortical softening. Time-harmonic elastography outperforms B-mode-based size quantification. © RSNA, 2019 Online supplemental material is available for this article.


Subject(s)
Elasticity Imaging Techniques/methods , Glomerulonephritis/diagnostic imaging , Adult , Aged , Early Diagnosis , Female , Humans , Kidney/diagnostic imaging , Male , Middle Aged , Prospective Studies , Reproducibility of Results , Young Adult
10.
CEN Case Rep ; 8(3): 221-225, 2019 08.
Article in English | MEDLINE | ID: mdl-30945174

ABSTRACT

A 69-year-old woman who presented with severe renal dysfunction and diffuse alveolar hemorrhage was diagnosed with pulmonary-renal syndrome (PRS) based on the coexistence of serum myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane (GBM) antibodies (Ab). Hemodialysis was started; plasma exchange and intravenous methylprednisolone pulse therapy were administered followed by oral prednisolone administration. Pulmonary hemorrhage decreased; however, renal dysfunction persisted. On maintenance hemodialysis and prednisolone therapy, MPO-ANCA and anti-GBM Ab became negative at 4 and 10 months, respectively; thereafter, they became positive again at 18 and 35 months, respectively. At 36 months, there was relapse of pulmonary hemorrhage. Plasma exchange and intravenous methylprednisolone pulse therapy were administered; pulmonary hemorrhage ceased, and both antibodies became negative. It is known that PRS cases that are double positive for ANCA and anti-GBM Ab occasionally relapse after remission, and, even though they are double positive at initial diagnosis, most relapses occur with reappearance or re-elevation of ANCA but with absence of anti-GBM-Ab. Therefore, this was a rare relapsing case that presented with double-positive serology. Further, our observation that the reappearance of ANCA preceded that of anti-GBM-Ab suggests that ANCA contribute to the reproduction of anti-GBM Ab.


Subject(s)
Antibodies, Antineutrophil Cytoplasmic/blood , Autoantibodies/blood , Glomerulonephritis/immunology , Hemorrhage/immunology , Lung Diseases/immunology , Aged , Female , Glomerulonephritis/diagnostic imaging , Hemorrhage/diagnostic imaging , Humans , Lung Diseases/diagnostic imaging , Recurrence
13.
Kidney Int ; 95(2): 363-374, 2019 02.
Article in English | MEDLINE | ID: mdl-30522769

ABSTRACT

Platelet-leukocyte interactions promote acute glomerulonephritis. However, neither the nature of the interactions between platelets and immune cells nor the capacity of platelets to promote leukocyte activation has been characterized in this condition. We used confocal intravital microscopy to define the interactions of platelets with neutrophils, monocytes, and endothelial cells in glomerular capillaries in mice. In the absence of inflammation, platelets underwent rapid on/off interactions with immune cells. During glomerulonephritis induced by in situ immune complex formation, platelets that interacted with neutrophils or monocytes, but not with other intraglomerular cells, were retained in the glomerulus for prolonged durations. Depletion of platelets inhibited both neutrophil recruitment and activation. Inhibition of platelet activating factor reduced neutrophil recruitment without impacting reactive oxygen species generation, while blocking CXC chemokine ligand 7 (CXCL7) reduced both responses. In contrast, inhibition of the adenosine diphosphate and thromboxane A2 pathways inhibited neutrophil reactive oxygen species generation without affecting neutrophil adhesion. Thus, platelet retention in glomerular capillaries following immune complex deposition stems from prolongation of platelet interactions with immune cells but not other substrates. Pro-inflammatory mediators play divergent roles in promoting neutrophil retention and activation in glomerular capillaries.


Subject(s)
Blood Platelets/immunology , Cell Communication/immunology , Glomerulonephritis/immunology , Kidney Glomerulus/cytology , Neutrophils/immunology , Animals , Blood Platelets/metabolism , Capillaries/cytology , Capillaries/diagnostic imaging , Capillaries/immunology , Disease Models, Animal , Glomerulonephritis/diagnostic imaging , Glomerulonephritis/pathology , Humans , Inflammation Mediators/immunology , Inflammation Mediators/metabolism , Intravital Microscopy , Kidney Glomerulus/blood supply , Kidney Glomerulus/immunology , Lymphocyte Activation/immunology , Male , Mice , Mice, Transgenic , Microscopy, Confocal , Monocytes/immunology , Neutrophil Infiltration/immunology , Neutrophils/metabolism
14.
IEEE Trans Med Imaging ; 37(12): 2718-2728, 2018 12.
Article in English | MEDLINE | ID: mdl-29994669

ABSTRACT

Transplantable kidneys are in very limited supply. Accurate viability assessment prior to transplantation could minimize organ discard. Rapid and accurate evaluation of intra-operative donor kidney biopsies is essential for determining which kidneys are eligible for transplantation. The criterion for accepting or rejecting donor kidneys relies heavily on pathologist determination of the percent of glomeruli (determined from a frozen section) that are normal and sclerotic. This percentage is a critical measurement that correlates with transplant outcome. Inter- and intra-observer variability in donor biopsy evaluation is, however, significant. An automated method for determination of percent global glomerulosclerosis could prove useful in decreasing evaluation variability, increasing throughput, and easing the burden on pathologists. Here, we describe the development of a deep learning model that identifies and classifies non-sclerosed and sclerosed glomeruli in whole-slide images of donor kidney frozen section biopsies. This model extends a convolutional neural network (CNN) pre-trained on a large database of digital images. The extended model, when trained on just 48 whole slide images, exhibits slide-level evaluation performance on par with expert renal pathologists. Encouragingly, the model's performance is robust to slide preparation artifacts associated with frozen section preparation. The model substantially outperforms a model trained on image patches of isolated glomeruli, in terms of both accuracy and speed. The methodology overcomes the technical challenge of applying a pretrained CNN bottleneck model to whole-slide image classification. The traditional patch-based approach, while exhibiting deceptively good performance classifying isolated patches, does not translate successfully to whole-slide image segmentation in this setting. As the first model reported that identifies and classifies normal and sclerotic glomeruli in frozen kidney sections, and thus the first model reported in the literature relevant to kidney transplantation, it may become an essential part of donor kidney biopsy evaluation in the clinical setting.


Subject(s)
Deep Learning , Glomerulonephritis/diagnostic imaging , Image Interpretation, Computer-Assisted/methods , Kidney/diagnostic imaging , Transplants/diagnostic imaging , Algorithms , Frozen Sections , Humans , Kidney Transplantation
15.
Reumatismo ; 70(2): 111-114, 2018 Jul 06.
Article in English | MEDLINE | ID: mdl-29976046

ABSTRACT

Immunoglobulin A (Ig A) nephropathy is the most frequent primary glomerulonephritis. Renal limited disease is the most widespread clinical form of the disease. Pulmonary involvement may also be seen concomitantly and capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement. In this paper, for the first time in literature, we describe an Ig A nephropathy patient with multiple pulmonary cavities as one of the presenting features of the disease. Also, no other etiology for the cavities was found other than Ig A nephropathy. Herein, possible pathogenesis might be capillaritis or deposition of immune complexes. As a result, it should be kept in mind that pulmonary cavity may be the presenting feature of Ig A nephropathy especially with other frequent signs of the disease.


Subject(s)
Glomerulonephritis, IGA/complications , Glomerulonephritis/etiology , Hemorrhage/etiology , Lung Diseases/diagnostic imaging , Biopsy , Glomerulonephritis/diagnostic imaging , Glomerulonephritis, IGA/diagnosis , Glomerulonephritis, IGA/pathology , Glomerulonephritis, IGA/therapy , Hemorrhage/diagnostic imaging , Humans , Kidney/pathology , Lung Diseases/etiology , Male , Middle Aged , Pleural Effusion/etiology , Renal Dialysis , Tomography, X-Ray Computed
16.
BMC Nephrol ; 19(1): 129, 2018 06 08.
Article in English | MEDLINE | ID: mdl-29884135

ABSTRACT

BACKGROUND: Morbidity associated with monoclonal gammopathy of renal significance is high due to the severe renal lesions and the associated systemic alterations. Accordingly, early diagnosis is fundamental, as is stopping the clonal production of immunoglobulins using specific chemotherapy. CASE PRESENTATION: A 75-year-old man with chronic renal disease of unknown origin since 2010 experienced rapid worsening of renal function over a period of 6 mos. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy showed the presence of C3 glomerulonephritis, with exclusive deposits of C3 visible on immunofluorescence and a membranoproliferative pattern on light microscopy. Skin biopsy showed endothelial deposition of complement. Given both the renal and cutaneous involvement the patient was considered to have monoclonal gammopathy of renal significance. We considered an underlying pathogenic mechanism for the renal alteration secondary to activation of the alternative complement pathway by the anomalous immunoglobulin. Despite treatment with plasmapheresis, bortezomib and steroids, advanced chronic kidney disease developed. CONCLUSIONS: The possible underlying cause of the monoclonal gammopathy of renal significance suggests that monoclonal gammopathy should be considered in adult patients with membranoproliferative glomerulonephritis.


Subject(s)
Complement C3/analysis , Glomerulonephritis/complications , Glomerulonephritis/diagnostic imaging , Paraproteinemias/complications , Paraproteinemias/diagnostic imaging , Aged , Glomerulonephritis/therapy , Humans , Male , Paraproteinemias/therapy
17.
J Ultrasound ; 21(2): 81-87, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29691759

ABSTRACT

PURPOSE: To compare contrast-enhanced ultrasonography (CEUS)-derived time-intensity (TI) curves with histological findings in kidneys of patients affected by chronic glomerulonephritides (GN) in the early stage of disease. METHODS: Research ethics committee approval and patient written informed consent were obtained. Thirty-one patients who showed clinical and laboratory signs of GN, with preserved renal function, were consecutively enrolled. They underwent kidney CEUS, from which TI curves were obtained, and kidney biopsy. TI curves were compared with clinical data, ultrasound (US) Doppler, and histological parameters. RESULTS: The persistence of contrast agent signal during the wash-out phase was found to be correlated with the degree of disease activity (p = 0.016) and in particular with the presence of mesangial hyperplasia (p = 0.008). No correlation was observed between TI curves and clinical or Doppler US-derived parameters. CONCLUSIONS: The persistence of contrast agent signal in the wash-out phase of CEUS appears to reflect a disturbance of perfusion in glomerular capillaries in the early stages of GN. We found that the histological element directly correlated with the prolonged wash-out was mesangial hyperplasia.


Subject(s)
Contrast Media , Glomerulonephritis/diagnostic imaging , Kidney/diagnostic imaging , Ultrasonography/methods , Chronic Disease , Female , Glomerulonephritis/pathology , Humans , Hyperplasia/diagnostic imaging , Hyperplasia/pathology , Kidney/pathology , Male , Middle Aged , Severity of Illness Index
18.
J Med Case Rep ; 12(1): 24, 2018 Feb 01.
Article in English | MEDLINE | ID: mdl-29386039

ABSTRACT

BACKGROUND: Posterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting feature of postinfectious glomerulonephritis, which has been reported in approximately 5% of hospitalized children, and it has been reported in very few cases of adult patients with sickle cell anemia. We report a very rare case of posterior reversible encephalopathy syndrome that occurred in a child with sickle cell anemia. This presentation should be differentiated from other neurologic manifestations that occur in patients with sickle cell anemia, because management is totally different. CASE PRESENTATION: We report what is to our knowledge the first reported case of a 9-year-old Saudi girl with sickle cell anemia who developed posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis. This occurred after full recovery from acute chest syndrome and severe vaso-occlusive crisis. CONCLUSIONS: The purpose of this report is to emphasize that all efforts should be made to explore the causes of different neurologic manifestations that occur in patients with sickle cell anemia, because this will require different pathways of management.


Subject(s)
Acute Chest Syndrome/etiology , Anemia, Sickle Cell/complications , Glomerulonephritis/complications , Vascular Diseases/etiology , Acute Chest Syndrome/diagnostic imaging , Acute Chest Syndrome/physiopathology , Acute Chest Syndrome/therapy , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/physiopathology , Anti-Bacterial Agents/therapeutic use , Anticonvulsants/therapeutic use , Antihypertensive Agents/therapeutic use , Blood-Brain Barrier , Cerebrovascular Circulation , Child , Female , Fluid Therapy , Glomerulonephritis/diagnostic imaging , Glomerulonephritis/physiopathology , Glomerulonephritis/therapy , Humans , Magnetic Resonance Imaging , Neuroimaging , Posterior Leukoencephalopathy Syndrome , Treatment Outcome , Vascular Diseases/diagnostic imaging , Vascular Diseases/physiopathology , Vascular Diseases/therapy
19.
Med Ultrason ; 1(1): 27-31, 2018 Feb 04.
Article in English | MEDLINE | ID: mdl-29400364

ABSTRACT

AIM: To analyze the relationship between shear wave velocity in the kidney measured by point shear wave elastography using Virtual Touch Quantification (VTQ) (Siemens Acuson S2000) and histological parameters obtained from renal biopsies, in patients with chronic glomerulonephritis (CGN). MATERIAL AND METHODS: The study group included 20 patients (mean age 47.95±13.59 years) with different types of CGN, that had underwent renal biopsy and 57 normal controls (mean age 38.07±17.32 years). In all patients, five valid stiffness measurements were obtained in each kidney, with the patient in lateral decubitus. Regarding the histological results, we assessed the presence or absence of glomerulosclerosis, interstitial fibrosis, and arteriolo-hyalinosis. RESULTS: In patients with CGN we obtained the following mean values of VTQ values: right kidney: 2.12±0.81 m/s, left kidney 1.65±0.54 m/s, while in the normal controls significantly higher VTQ values were obtained: right kidney 2.69±0.72 m/s (p=0.004), left kidney 2.48±0.73 m/s (p<0.0001). In patients with CGN no statistically significant correlations between VTQ values and eGFR (r=0.37, p=0.12) or proteinuria (r=0.2, p=0.37) were found. We found significantly lower VTQ values in patients with interstitial fibrosis (1.46 vs. 1.99 m/s, p<0.05) and also in patients with arteriolo-hyalinosis (1.55 vs. 2.47 m/s, p<0.05). CONCLUSION: Our pilot study shows that shear wave velocity values in patients with CGN are significantly lower compared to normal controls, and there is a tendency to decrease with the decrease of eGFR, with the presence of interstitial fibrosis and of arteriolo-hyalinosis.


Subject(s)
Elasticity Imaging Techniques/methods , Glomerulonephritis/diagnostic imaging , Glomerulonephritis/pathology , Image Processing, Computer-Assisted/methods , Adult , Biopsy , Chronic Disease , Female , Humans , Kidney/diagnostic imaging , Kidney/pathology , Male , Middle Aged , Pilot Projects , Prospective Studies , Reproducibility of Results
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