scholarly journals The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy—A National Study in Children

2021 ◽  
Vol 10 (19) ◽  
pp. 4405
Author(s):  
Małgorzata Mizerska-Wasiak ◽  
Agnieszka Such-Gruchot ◽  
Karolina Cichoń-Kawa ◽  
Agnieszka Turczyn ◽  
Jadwiga Małdyk ◽  
...  
Keyword(s):  
Kidney Biopsy ◽  
Clinical Presentation ◽  
Immunofluorescence Microscopy ◽  
Clinical Course ◽  
National Study ◽  
Oxford Classification ◽  
Complement Component C3 ◽  
The Oxford Classification

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children.

scholarly journals Evaluation of Clinical and Pathological Characteristics of Patients with IgA Nephropathy Based on Oxford Classification System: Should Crescents be Included?

2017 ◽  
Vol 15 (1) ◽  
pp. 10-15
Author(s):  
Sibel Ersan ◽  
Omur Gokmen Sevindik ◽  
Caner Cavdar ◽  
Sibel Ada ◽  
Aykut Sifil ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Classification System ◽  
Clinical Course ◽  
Immunoglobulin A ◽  
Classification Systems ◽  
Oxford Classification ◽  
Crescent Formation ◽  
Pathological Characteristics ◽  
The Oxford Classification

Abstract Introduction. None of the classification systems in immunoglobulin A (IgA) nephropathy has been widely agreed or implemented by clinicians or pathologists. In order to meet this need, "Oxford Classification System", which is highly reproducible and predictive for clinical course, was developed in 2009. In the present study, we investigated clinical and pathological characteristics of patients with IgA nephropathy based on current classification and the predictivity of crescent presence on prognosis. Methods. The study comprised 40 patients with diagnosis of primary IgA nephropathy on renal biopsy. The biopsy findings and follow-up parameters of patients were retrospectively re-evaluated. Pathological findings were examined based on the Oxford classification system. The presence of crescent formation in the specimens was noted. Results. The presence of crescent formation was predictive of poor prognosis regarding the glomerular filtration rate (eGFR), the level of proteinuria, and mean arterial pressure (MAP). Conclusion: Considering the importance of crescent formation in prediction of the clinical course and need for immunosuppressive therapy, it is suggested that crescent presence can be included in this classification system.

scholarly journals FRI0212 THE ROLE OF AGE ON THE CLINICAL PRESENTATION AND RELAPSE RATES IN A LARGE COHORT OF 720 PATIENTS WITH GIANT CELL ARTERITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 689.1-690
Author(s):  
S. Monti ◽  
L. Dagna ◽  
C. Campochiaro ◽  
A. Tomelleri ◽  
G. Zanframundo ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Relapse Rate ◽  
Clinical Presentation ◽  
Age Groups ◽  
Age At Diagnosis ◽  
First Relapse ◽  
Time To Relapse

Background:Giant cell arteritis (GCA) is the most frequent systemic vasculitis after the age of 50 years old. Recent interest in the processes of immune and vascular aging have been proposed as a disease risk factor. Data on the impact of age at diagnosis of GCA on the clinical course of the disease are scarceObjectives:To assess the role of age at diagnosis of GCA on the risk and time to relapseMethods:Centres participating in the Italian Society of Rheumatology Vasculitis Study Group retrospectively enrolled patients with a diagnosis of GCA until December 2019. The cohort was divided in tertiles according to age at diagnosis (≤ 72; 73-79; > 79 years old). Negative binomial regression was used to assess the relapse rate according to age groups, and Cox regression for time to first relapse.Results:Of 720 patients enrolled in 14 Italian reference centres, 711 had complete follow-up data (female 50%; mean age 75±7). Median follow-up duration was 34 months (IQR 16;70). Patients in the older group at diagnosis (> 79 years) had more frequent visual loss compared to the 73-79 and ≤ 72 age groups (31% vs 20% vs 7%; p<0.001), but lower rates of general symptoms (56% vs 70% vs 77%; p<0.001). Large-vessel (LV)-GCA was less frequent in the older group (18% vs 22% vs 43%; p<0.001). At least one relapse occurred in 47% of patients. Median time to relapse was 12 months (IQR 6;23). Age did not influence the rate of relapses [18 per 100 persons/years (95%CI 15;21) vs 19 (95% CI 17;22) vs 19 (95%CI 17;22)], nor the time to first relapse (Figure 1). LV-GCA, presentation with significantly elevated c-reactive protein (> 50 mg/L) and general symptoms were independent predictors of relapse.Conclusion:Age at diagnosis of GCA influenced the clinical presentation and risk of ischaemic complications, but did not affect the relapse rate during follow-up. LV-GCA occurred more frequently in younger patients and was an independent predictor of relapse risk, highlighting the need for a correct characterization of the clinical subtype at the early stages of disease.Disclosure of Interests:Sara Monti: None declared, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Alessandro Tomelleri: None declared, Giovanni Zanframundo: None declared, Catherine Klersy: None declared, Francesco Muratore: None declared, Luigi Boiardi: None declared, Roberto Padoan: None declared, Mara Felicetti: None declared, Franco Schiavon: None declared, Milena Bond: None declared, Alvise Berti: None declared, Roberto Bortolotti: None declared, Carlotta Nannini: None declared, Fabrizio Cantini: None declared, Alessandro Giollo: None declared, Edoardo Conticini: None declared, angelica gattamelata: None declared, Roberta Priori: None declared, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Elena Treppo: None declared, Giacomo Emmi: None declared, Martina Finocchi: None declared, Giulia Cassone: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen, Rosario Foti Consultant of: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Speakers bureau: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Michele Colaci: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Carlo Salvarani: None declared, Carlomaurizio Montecucco: None declared

Is it possible to predict parameters of the Oxford classification of primary IgA Nephropathy from clinical laboratory data? Focus on the role of segmental glomerulosclerosis subtypes

2019 ◽  
Vol 215 (10) ◽  
pp. 152533
Author(s):  
Maria Luiza Gonçalves dos Reis Monteiro ◽  
Matheus Rodrigues Vieira ◽  
Lívia Helena Morais Pereira ◽  
Liliane Silvano Araújo ◽  
Crislaine Aparecida Silva ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Clinical Laboratory ◽  
Laboratory Data ◽  
Oxford Classification ◽  
Segmental Glomerulosclerosis ◽  
The Oxford Classification

scholarly journals Extended Abstract: Deficiency of Sodium Taurocholate Cotransporting Polypeptide (SLC10A1): A New Inborn Error of Metabolism with an Attenuated Phenotype

2017 ◽  
Vol 35 (3) ◽  
pp. 259-260 ◽  
Author(s):  
Frédéric M. Vaz ◽  
Hidde H. Huidekoper ◽  
Coen C. Paulusma
Keyword(s):  
Bile Salts ◽  
Clinical Presentation ◽  
Enterohepatic Circulation ◽  
Sodium Taurocholate ◽  
Elevated Plasma ◽  
Mild Phenotype ◽  
Functional Studies ◽  
Conjugated Bile Salts

We present the first patient with a defect in the Na+-taurocholate cotransporting polypeptide SLC10A1 (NTCP), which plays a key role in the enterohepatic circulation of bile salts. The clinical presentation of the child was mild and the child showed no signs of liver dysfunction or pruritus despite extremely elevated plasma bile salt levels (>100-fold upper-limit of normal). A homozygous point mutation was found in the SLC10A1 gene (resulting in amino acid change R252H) and functional studies confirmed the pathogenicity of the mutation. This confirms the role of NTCP as the major transporter of conjugated bile salts into the liver as part of the enterohepatic circulation and shows that other transporters partly can take over its function, resulting in a relatively mild phenotype. This work was published previously in [Vaz et al.: Hepatology 2015;61:260-267] and supplemented with some follow-up information of the patient.

Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations

2019 ◽  
Vol 90 (6) ◽  
pp. 695-703 ◽  
Author(s):  
Anshit Goyal ◽  
Lorenzo Rinaldo ◽  
Redab Alkhataybeh ◽  
Panagiotis Kerezoudis ◽  
Mohammed Ali Alvi ◽  
...  
Keyword(s):  
Natural History ◽  
Surgical Resection ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Univariate Analysis ◽  
Case Series ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Increased Risk

ObjectiveThere is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management.MethodsWe retrospectively reviewed the clinical course of patients diagnosed with ISCMs at our institution between 1995 and 2016. Haemorrhage was defined as clinical worsening in tandem with imaging changes visualised on follow-up MRI. Outcomes assessed included neurological status and annual haemorrhage rates.ResultsA total of 107 patients met inclusion criteria. Follow-up data were available for 85 patients. While 21 (24.7%) patients underwent immediate surgical resection, 64 (75.3%) were initially managed conservatively. Among this latter group, 16 (25.0%) suffered a haemorrhage during follow-up and 11 (17.2%) required surgical resection due to interval bleeding or neurological worsening. The overall annual risk of haemorrhage was 5.5% per person year. The rate among patients who were symptomatic and asymptomatic on presentation was 9.5% and 0.8%, respectively. Median time to haemorrhage was 2.3 years (0.1–12.3). Univariate analysis identified higher ISCM size (p=0.024), history of prior haemorrhage (p=0.013) and presence of symptoms (p=0.003) as risk factors for subsequent haemorrhage. Multivariable proportional hazards analysis revealed presence of symptoms to be independently associated with haemorrhage during follow-up (HR 9.39, CI 1.86 to 170.8, p=0.013).ConclusionLarge, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.

MO308COULD THE PRESENCE OF ANCAS IN IGA NEPHROPATHY WITH CRESCENTS HAVE A CLINICAL IMPLICATION?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Zaira Castañeda Amado ◽  
Alejandra Gabaldon ◽  
María Teresa Sanz ◽  
Roxana Bury ◽  
Cinthia Baldallo ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Kidney Biopsy ◽  
Clinical Presentation ◽  
Fibrinoid Necrosis ◽  
Oral Steroids ◽  
Common Clinical Presentation ◽  
The Mean ◽  
End Stage

Abstract Background and Aims IgA nephropathy (IgAN) is the most common glomerulonephritis. The presence of ANCAs in this pathology represents a rare coincidence. However, it is not clear if the presence of IgA or IgG ANCAs in these patients could have clinical significance. We aim to describe the presence of IgA and IgG ANCAs in patients diagnosed with IgAN with crescents, and its possible clinical implications. Method Retrospective study from 2013 to 2020, it included all patients diagnosed by kidney biopsy of IgAN with extracapillary proliferation. Outpatient follow-up time was up to 24 months. Demographics and clinicopathologic data, ANCAs subtype, characteristics of the biopsy and treatment at the time of diagnosis/follow up was recollected. Results From 2013 to 2020, 17 adults were diagnosed with IgAN and extracapillary proliferation. 5 patients presented ANCAs, 3 (17%) were IgA ANCAs and 2 (11%) were IgG ANCAs. At diagnosis, the median age was 48 years old (27-75 years, sd. 15), with 9 women (52%). At the time of diagnosis, the most common clinical presentation was hypertension (71%). The laboratory analysis showed that median hemoglobin was 11.7 mg/dl (8.4-14.9 mg/dL, sd. 1.5), median creatinine was 2.2 mg/dL (0.55-5.7 mg/dL, sd. 1.4) and median proteinuria was 3.5 g/mgCr (0.1-12 g/mgCr, sd. 3.5). 7 patients (41%) presented extracapillary proliferation less than 25%, 7 patients presented it between 25% and 50%, and 3 patients (17%) had it in more than 50%. 5 (30%) patients presented fibrinoid necrosis. 1 (6%) patient needed renal replacement therapy upon admission. In terms of treatment, all patients with ANCAs IgAN received endovenous steroids and cyclophosphamide. The mean follow-up time was 6 months. Oral steroids (59%) and mycophenolate (41%) were the most frequent treatments. At six months, the median creatinine was 1.9 mg/dL (0.4-7, sd. 1.78) and the median proteinuria was 1.45 g/gCr (0.12-5.9, sd. 1.84 g/gCr). 3 patients developed end-stage chronic kidney disease and requiring substitute renal therapy; 4 patients died. Statistical analysis did not show differences in clinical characteristics, demographics, kidney function, proteinuria, need for renal therapy replacement or mortality according to the presence or subtype of ANCA. ANCA negative patients presented less than 25% of extracapillary proliferation in renal biopsy (p = 0.04). ANCA positive patients presented more fibrinoid necrosis than ANCA negative patients (p=0.01). Conclusion Given the limited size of our sample, our results do not allow us to be conclusive, showing no significant differences between the ANCA subtypes. However, from the point of renal biopsy, it is observed that patients with negative ANCAs present less extracapillary proliferation; and that patients ANCA positive presented more fibrinoid necrosis.

Extracapillary Proliferative Glomerulonephritis in Children

PEDIATRICS ◽  
1975 ◽  
Vol 56 (3) ◽  
pp. 434-442
Author(s):  
Sudhir K. Anand ◽  
Carl W. Trygstad ◽  
Hari M. Sharma ◽  
James D. Northway
Keyword(s):  
Streptococcal Infection ◽  
Clinical Course ◽  
Renal Involvement ◽  
Therapeutic Agents ◽  
Proliferative Glomerulonephritis ◽  
The Mean ◽  
Histopathologic Changes ◽  
Better Than

The clinical course and renal pathology of 17 children with acute extracapillary proliferative glomerulonephritis is reported. Patients with systemic ses associated with renal involvement were excluded. The onset followed streptococcal infection in ten; of these, four have died, one has been transplanted, and the remaining five have completely healed. The mean follow-up of the latter five patients was 32 months (range, 18 to 57 months). Of the other seven patients without evidence of preceding streptococcal infection, two have died, two have been transplanted, and the remaining three all have hypertension, proteinuria, and reduced creatinine clearance. The mean follow-up of the latter three patients was 29 months (range, 14 to 38 months). The initial renal histopathologic changes and their progress in later renal biopsies is described. The role of various therapeutic agents is discussed. The prognosis in acute extracapillary proliferative glomerulonephritis following streptococcal infection appears to be better than in ones without preceding streptococcal infection.

Mastitis and More: A Pictorial Review of the Red, Swollen, and Painful Breast

2020 ◽  
Author(s):  
Genevieve A Woodard ◽  
Asha A Bhatt ◽  
Erica M Knavel ◽  
Katie N Hunt
Keyword(s):  
Clinical Presentation ◽  
Clinical Course ◽  
Fluid Collection ◽  
Percutaneous Biopsy ◽  
Imaging Features ◽  
Idiopathic Granulomatous Mastitis ◽  
Targeted Ultrasound ◽  
Pictorial Review ◽  
Acute Mastitis

Abstract Clinically, acute mastitis presents as a red, swollen, and painful breast. Targeted ultrasound can be performed to evaluate the extent of infection and for an underlying abscess. Noncomplicated mastitis or a small fluid collection may respond to oral antibiotics without further intervention, but a larger or more complex abscess may require single or serial percutaneous aspiration. Breast infections, particularly those complicated by an abscess, can have a prolonged clinical course, and close follow-up is required. Since the clinical presentation and imaging features of acute infectious mastitis can overlap with other etiologies, such as inflammatory breast cancer and idiopathic granulomatous mastitis, a percutaneous biopsy may be indicated to accurately diagnose patients.

Sign in / Sign up

Export Citation Format

Share Document