scholarly journals Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: A retrospective analysis of data from the Japan Renal Biopsy Registry

PLoS ONE ◽  
2021 ◽  
Vol 16 (9) ◽  
pp. e0257397
Author(s):  
Naoki Nakagawa ◽  
Masashi Mizuno ◽  
Sawako Kato ◽  
Shoichi Maruyama ◽  
Hiroshi Sato ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Biopsy ◽  
Treatment Outcomes ◽  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Clinical Characteristics ◽  
Diastolic Pressure ◽  
Remission Rate ◽  
Type I ◽  
C3 Glomerulonephritis

The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies has provided insights into these two distinct diseases. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. Although these diseases have poor outcomes, limited Japanese literature confined to small, single-center cohorts exist on these diseases. We retrospectively analyzed 81 patients with MPGN type I and III from 15 hospitals in the Japan Renal Biopsy Registry to compare demographic, clinical characteristics and treatment outcomes of patients with IC-MPGN to those with C3GN. Of the 81 patients reviewed by immunofluorescence findings in kidney biopsies, 67 patients had IC-MPGN and 14 patients had C3GN. Age at diagnosis and systolic and diastolic pressure were higher and proteinuria and impaired renal function were significantly more prevalent in patients with IC-MPGN than those with C3GN. About 80% of the patients in both groups were treated with immunosuppressive therapy. At last follow-up (median 4.8 years), complete remission rate of proteinuria was significantly higher in patients with C3GN (64.3%) than in those with IC-MPGN (29.9%; P = 0.015). The renal survival rate was lower in patients with IC-MPGN when compared to C3GN (73.1% vs. 100%; log-rank, P = 0.031). Systolic blood pressure and renal function at baseline were independent predictors of progression to end-stage kidney disease. The overall prognosis of patients with C3GN is more favorable than for patients with IC-MPGN.

scholarly journals Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II

2021 ◽  
Vol 9 ◽  
Author(s):  
Xue He ◽  
Yueling Zhu ◽  
Haidong Fu ◽  
Chunyue Feng ◽  
Zhixia Liu ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Clinical Manifestations ◽  
Arachnoid Cysts ◽  
Type I ◽  
Type Ii ◽  
Abernethy Malformation ◽  
Congenital Vascular Malformation ◽  
Congenital Portosystemic Shunt

This report describes an 8-year-old male who presented with clinical manifestations including systemic edema, heavy proteinuria, hypoproteinemia, and persistent hypocomplementemia. Arachnoid cysts and focal nodular hyperplasia were also detected. Imaging examination and renal biopsy were performed, and Abernethy malformation type II with immune complex-mediated membranoproliferative glomerulonephritis was considered the diagnosis. Due to the persistence of embryonic vessels, Abernethy malformation is a rare congenital vascular malformation of the splanchnic venous system, which can be classified as type I (end-to-side shunt) and type II (side-to-side shunt). Abernethy malformation with glomerulonephritis remains extremely rare. In the patient described, glomerulonephritis mediated by immune complex with “full-house” positive immunohistochemistry was confirmed on renal biopsy. In addition, he was treated with glucocorticoids and tacrolimus. Whether surgical treatment is necessary should be determined according to the state of the disease in the later stages. The present case reflects the association between the congenital portosystemic shunt and the renal region and, to the authors' knowledge, may be the first report to describe arachnoid cysts as a symptom of Abernethy malformation.

scholarly journals Clinical Characteristics and Treatment Outcomes in Endogenous Cushing’s Syndrome: A 15-Year Experience from Thailand

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Wasita Warachit Parksook ◽  
Nitchakarn Laichuthai ◽  
Sarat Sunthornyothin
Keyword(s):  
Treatment Outcomes ◽  
Cushing’S Syndrome ◽  
Clinical Characteristics ◽  
Remission Rate ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
Normal Body Mass Index ◽  
Ectopic Acth ◽  
Free Cortisol

The most common subtype of endogenous Cushing’s syndrome (CS) is Cushing’s disease (CD), with higher proportions of adrenal CS reported from Asia, compared to other continents. However, little was known about CS in this territory. This study was to investigate the distribution, clinical characteristics, and treatment outcomes of CS in a single tertiary hospital in Thailand. We performed a retrospective evaluation of 82 patients with endogenous CS during 2001–2015. The most common subtype was CD, followed by adrenal CS and ectopic ACTH syndrome (EAS), respectively. Weight gain was the most common presentation. Normal body mass index (BMI), Asian cutoff, was observed in 33% of patients. Specific features of CS (plethora, muscle weakness, bruising, and/or wide purplish striae) were documented in less than half of patients. The median age, adrenocorticotropic hormone (ACTH), and urinary free cortisol (UFC) concentrations were significantly different among 3 subtypes of CS and were highest among patients with EAS. An initial remission rate after transsphenoidal surgeries in CD was 62%, with higher rates in pituitary microadenomas compared to macroadenomas. All patients with unilateral adrenal disease achieved CS remission after adrenal surgeries. Patients with EAS achieved CS remission mostly from bilateral adrenalectomy. The highest mortality rate was observed in the EAS group. These findings were consistent with previous studies in Asia, with more proportions ACTH-independent CS.

scholarly journals Predictors and moderators of outcomes in mindfulness-based cognitive therapy intervention for early breast cancer patients

2021 ◽  
pp. 1-8
Author(s):  
Noriko Tamura ◽  
Sunre Park ◽  
Yasunori Sato ◽  
Yasuko Sato ◽  
Yuka Takita ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Treatment Outcomes ◽  
Cognitive Therapy ◽  
Cancer Patients ◽  
Clinical Characteristics ◽  
Remission Rate ◽  
Controlled Trial ◽  
Depression Scale ◽  
Optimal Number ◽  
Breast Cancer Patients

Abstract Objectives To deliver mindfulness-based cognitive therapy (MBCT) efficiently, the present study aimed (1) to identify predictors and moderators of patients who benefit from MBCT for psychological distress and (2) to explore the initial treatment reaction to identify the optimal number of sessions that produce a significant clinical effect. Methods This is the secondary analysis of a randomized controlled trial of MBCT for breast cancer patients (N = 74). We classified the participants into remitters vs. non-remitters, and responder vs. non-responders, according to the total score of the Hospital Anxiety and Depression Scale at the end of the intervention. We conducted multivariate analyses to explore for predictors of response and remission. We adopted generalized estimating equations to explore the optimal number of sessions. Results Sociodemographic and clinical backgrounds did not have significant influence on the treatment outcomes of the MBCT. Better program adherence, which was represented as the participants’ better attendance to the MBCT program, was a significant predictor of both remission and response [odds ratio (OR) = 1.90, 95% confidence interval (CI) 1.25–2.89, p = 0.003, and OR = 1.72, 95% CI 1.12–2.65, p = 0.013, respectively]. It was not until seventh session that the remission rate exceeded 50% and the response rate showed significance. Significance of results Sociodemographic and clinical characteristics did not significantly influence the treatment outcomes, while homework minutes and class attendance had significant effects on treatment outcomes. This implies that MBCT is recommended to any cancer patient, if he/she is motivated to the program, regardless of their sociodemographic and clinical characteristics. Patients are encouraged to attend a standard MBCT program (eight sessions) and do the assigned homework as intensely as possible. Further studies with larger sample and objective measurements are desired.

Hydrocephalus in Spinal Muscular Atrophy: A Case Report and Review of the Literature

2020 ◽  
Author(s):  
Jeetendra P. Sah ◽  
Aaron W. Abrams ◽  
Geetha Chari ◽  
Craig Linden ◽  
Yaacov Anziska
Keyword(s):  
Spinal Muscular Atrophy ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Muscular Atrophy ◽  
Communicating Hydrocephalus ◽  
Type I ◽  
Review Of The Literature ◽  
Radiographic Findings ◽  
Comprehensive Review ◽  
The Relationship

AbstractIn this article, we reported a case of spinal muscular atrophy (SMA) type I noted to have tetraventricular hydrocephalus with Blake's pouch cyst at 8 months of age following intrathecal nusinersen therapy. The association of hydrocephalus with SMA is rarely reported in the literature. Development of hydrocephalus after intrathecal nusinersen therapy is also reported in some cases, but a cause–effect relationship is not yet established. The aim of this study was to describe the clinical characteristics of a patient with SMA type I and hydrocephalus, to review similar cases reported in the literature, and to explore the relationship between nusinersen therapy and development of hydrocephalus. The clinical presentation and radiographic findings of the patient are described and a comprehensive review of the literature was conducted. The adverse effect of communicating hydrocephalus related to nusinersen therapy is being reported and the authors suggest carefully monitoring for features of hydrocephalus developing during the course of nusinersen therapy.

Effect on renal function of change from high to moderate protein intake in type I diabetic patients

Diabetes ◽  
1987 ◽  
Vol 36 (1) ◽  
pp. 73-79 ◽  
Author(s):  
W. L. Kupin ◽  
P. Cortes ◽  
F. Dumler ◽  
C. S. Feldkamp ◽  
M. C. Kilates ◽  
...  
Keyword(s):  
Renal Function ◽  
Protein Intake ◽  
Diabetic Patients ◽  
Type I

Increase in remission rate in newly diagnosed type I diabetic subjects treated with azathioprine

Diabetes ◽  
1985 ◽  
Vol 34 (12) ◽  
pp. 1306-1308 ◽  
Author(s):  
L. C. Harrison ◽  
P. G. Colman ◽  
B. Dean ◽  
R. Baxter ◽  
F. I. Martin
Keyword(s):  
Remission Rate ◽  
Type I ◽  
Newly Diagnosed

scholarly journals POS1120 PRESENCE OF TOPHI IS ASSOCIATED WITH A RAPID DECLINE IN THE RENAL FUNCTION IN PATIENTS WITH GOUT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 839.1-839
Author(s):  
Y. J. Oh ◽  
K. W. Moon
Keyword(s):  
Renal Function ◽  
Renal Dysfunction ◽  
Clinical Characteristics ◽  
Multivariate Logistic Regression Analysis ◽  
Joint Involvement ◽  
Rapid Decline ◽  
Symptom Duration ◽  
Tophaceous Gout ◽  
Duration Of Symptoms ◽  
Increased Risk

Background:Gout is the most common inflammatory arthritis resulting from a chronic deposition of MSU crystals in the joints and other soft tissues. After the process of repeated tissue damage and repair due to gout, tophi could be formed around the affected joints. Intra-articular tophi may sometimes result in bone destruction, joint deformities, and dysfunction which can adversely affect the patient’s quality of life. Furthermore, early-onset tophaceous gout patients are more likely to develop renal dysfunction, however, few studies have investigated if the presence of tophi is related with the progression of renal dysfunction in gout patients.Objectives:We aimed to compare clinical characteristics of patients with and without tophi at the time of the diagnosis of gout and investigate the effect of tophi on the renal function in gout patients.Methods:Data of 257 patients who were first diagnosed with gout at the Kangwon National University Hospital from January 2012 to December 2018 were retrospectively studied. Patients were divided into 2 groups according to the presence of tophi at the diagnosis. We compared clinical characteristics and the progression of renal dysfunction between the two groups.Results:Of all patients, 66 (25.5%) initially presented with tophi. Patients with tophi were older, had a longer duration of symptoms, and had a higher prevalence of multiple joint involvement than those without tophi. The decline in the eGFR was more prominent in patients with tophi than in those without (-4.8±14.5 ml/min/1.73m2vs. -0.7±11.9 ml/min/1.73m2, respectively; P=0.039). In multivariate logistic regression analysis, a prolonged symptom duration (odds ratio [OR], 1.010; 95% confidence interval [CI], 1.004–1.017; P=0.001) and multiple joint involvement (OR, 3.027; 95% CI, 1.831–5.004; P<0.001) were significantly associated with increased risk of formation of tophi. The presence of tophi was significantly associated with a rapid decline in the eGFR (β=-0.141; P=0.035).Conclusion:A prolonged symptom duration and multiple joint involvement were independent risk factors for tophi as the presenting symptom in gout patients. The presence of tophi was associated with a declining renal function. Therefore, an early diagnosis and active treatment are important in tophaceous gout.References:[1]Bardin T, Richette P. Definition of hyperuricemia and gouty conditions. Curr Opin Rheumatol 2014;26:186-91.[2]Liu F, Du GL, Song N, Ma YT, Li XM, Gao XM, et al. Hyperuricemia and its association with adiposity and dyslipidemia in Northwest China: results from cardiovascular risk survey in Xinjiang (CRS 2008-2012). Lipids Health Dis 2020;19:58.[3]Dalbeth N, Merriman TR, Stamp LK. Gout. Lancet 2016;388:2039-52.[4]Ruoff G, Edwards NL. Overview of Serum Uric Acid Treatment Targets in Gout: Why Less Than 6 mg/dL? Postgrad Med 2016;128:706-15.[5]Bieber A, Schlesinger N, Fawaz A, Mader R. Chronic tophaceous gout as the first manifestation of gout in two cases and a review of the literature. Semin Arthritis Rheum 2018;47:843-8.Disclosure of Interests:None declared.

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