scholarly journals Membranous nephropathy associated with thrombospondin type-1 domain-containing 7A (THSD7A) in an adult woman with eosinophilia

2019 ◽  
Vol 9 (1) ◽  
pp. 65-73 ◽  
Author(s):  
Sayuri Shirai ◽  
Shin’ichi Akiyama ◽  
Atsuko Kamijo-Ikemori ◽  
Tomo Suzuki ◽  
Daisuke Ichikawa ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Basement Membrane ◽  
Blood Cell ◽  
Membranous Nephropathy ◽  
Immunofluorescence Test ◽  
Indirect Immunofluorescence Test ◽  
Urinary Protein Level ◽  
Steroid Dosage ◽  
The Relationship

Abstract A 30-year-old woman on steroid therapy for eosinophilia presented with nephrotic syndrome during steroid tapering. She was diagnosed with membranous nephropathy (MN) stage II–III (positive for IgG1 and IgG4) by renal biopsy. There was no evidence of secondary MN. Her urinary protein level was controlled to 0.5 g/day or less, and her eosinophil count in white blood cell differential was stabilized at less than 10% without increasing the steroid dosage. The renal specimen did not show any enhanced granular expression of PLA2R along the glomerular basement membrane, and PLA2R was not detected in the patient’s serum. On retrospective analysis, enhanced granular staining for thrombospondin type-1 domain-containing 7A (THSD7A) in the glomeruli was detected in the biopsy, and anti-THSD7A IgG was detected in the serum using a commercial indirect immunofluorescence test (IFT). Based on these, the case was considered as THSD7A-associated MN with comorbid eosinophilia. The causal relationship between THSD7A-related MN and eosinophilia was unclear. However, a few cases of THSD7A-associated MN with eosinophilia have been reported, and further clarification on the relationship between THSD7A-related MN and eosinophilia is warranted.

Membranous nephropathy

2010 ◽  
pp. 3985-3988
Author(s):  
Dwomoa Adu
Keyword(s):  
Prostate Cancer ◽  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Basement Membrane ◽  
Autoimmune Diseases ◽  
Outer Surface ◽  
Glomerular Basement Membrane ◽  
Lupus Erythematosus ◽  
Membranous Nephropathy ◽  
Systemic Lupus

Membranous nephropathy, which accounts for 20 to 30% of cases of the nephrotic syndrome in adults, is defined histologically by the presence of subepithelial immune deposits on the outer surface of the glomerular basement membrane. The immune mechanisms that lead to this are uncertain, and most cases are of unknown cause (idiopathic), but the condition can be associated with autoimmune diseases (systemic lupus erythematosus), malignancy (in 10% of cases, most commonly lung and prostate cancer), drugs, and infections....

scholarly journals Nephrotic syndrome co-existing with type 1 diabetes in a 12-year-old boy: Case report and literature review

2019 ◽  
Vol 7 ◽  
pp. 2050313X1982773 ◽  
Author(s):  
Noor S Bawahab ◽  
Osama Y Safdar ◽  
Sarah A Nagadi ◽  
Asalh T Saeedi ◽  
Raghad W Mohammed Hussain
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Nephrotic Syndrome ◽  
Type 1 Diabetes Mellitus ◽  
Pediatric Nephrology ◽  
Regular Insulin ◽  
Response To Treatment ◽  
Low Protein ◽  
The Relationship

Occurrence of early nephrotic syndrome in type 1 diabetes mellitus patients is extremely rare. Herein, we report the case of a 12-year-old boy who presented to our pediatric nephrology clinic with generalized edema. He had been diagnosed with type 1 diabetes mellitus at age 9 and had been treated with regular insulin. Examinations revealed normal kidney function, hypoalbuminemia, proteinuria (4+), hyperlipidemia, and low protein-to-creatinine ratio. The patient was diagnosed with idiopathic nephrotic syndrome and was empirically administered prednisolone for 12 weeks. Subsequently, prednisolone was tapered over 10–12 weeks. The patient showed good response to treatment. In conclusion, co-existence of nephrotic syndrome and type 1 diabetes mellitus may suggest an immunological basis; therefore, further studies are needed to investigate the relationship between these two conditions.

Membranous nephropathy

2009 ◽  
pp. 261-312 ◽  
Author(s):  
Patrizia Passerini ◽  
Claudio Ponticelli
Keyword(s):  
Nephrotic Syndrome ◽  
Basement Membrane ◽  
End Stage Renal Disease ◽  
Membranous Nephropathy ◽  
Spontaneous Remission ◽  
Thromboembolic Events ◽  
Slow Progression ◽  
Life Threatening ◽  
Stage Renal Disease ◽  
End Stage

Membranous nephropathy (MN) is a glomerular disease which is characterized histologically by uniform thickening of the glomerular capillary due to the presence of immunoglobulin-containing deposits on the outer or subepithelial aspect of the glomerular basement membrane (GBM). It exists in idiopathic (etiology unknown) or secondary (causally associated with another disorder) forms. Differentiation of the idiopathic membranous nephropathy (IMN) from secondary forms of MN may be difficult. It is a frequent cause of the nephrotic syndrome in adults. It is also characterized by a tendency for spontaneous remission in some patients and by persistence of proteinuria and slow progression to end-stage renal disease (ESRD) in others. Those patients with severe and un-remitting nephrotic syndrome may also suffer from disabling and even life-threatening extra-renal complications, such as thromboembolic events.

Primary membranous nephropathy: comprehensive review and historical perspective

2019 ◽  
Vol 95 (1119) ◽  
pp. 23-31 ◽  
Author(s):  
Krishna C Keri ◽  
Samuel Blumenthal ◽  
Varsha Kulkarni ◽  
Laurence Beck ◽  
Tepsiri Chongkrairatanakul
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Adrenocorticotropic Hormone ◽  
Disease Process ◽  
Serologic Marker ◽  
Phospholipase A2 Receptor ◽  
Stage Renal Disease ◽  
End Stage ◽  
Over 40 Years

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in non-diabetic Caucasian adults over 40 years of age. It has an estimated incidence of 8–10 cases per 1 million. Fifty per cent of patients diagnosed with primary MN continue to have nephrotic syndrome and 30% of patients may progress to end-stage renal disease over 10 years. Although it was recognised as a distinct clinic-pathological entity in 1940s by immunofluorescence and electron microscopy, the pathogenesis and treatment have become more apparent only in the last decade. Discovery of M-type phospholipase A2 receptor (PLA2R) antibodies and thrombospondin type 1 domain-containing 7A antibodies has given new perspectives in understanding the pathogenesis of the disease process. Anti-PLA2R antibody is the first serologic marker that has promising evidence to be used as a tool to prognosticate the course of the disease. More importantly, therapeutic agents such as rituximab and adrenocorticotropic hormone analogues are the newer therapeutic options that should be considered in the therapy of primary MN.

A Decade of Disparities in Diabetes Technology Use and HbA1c in Pediatric Type 1 Diabetes: A Trans-Atlantic Comparison

2020 ◽  
Author(s):  
Ananta Addala ◽  
Marie Auzanneau ◽  
Kellee Miller ◽  
Werner Maier ◽  
Nicole Foster ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Technology Use ◽  
Hemoglobin A1c ◽  
Diabetes Technology ◽  
Design And Methods ◽  
Relationship Of ◽  
The Relationship ◽  
Pediatric Type 1 Diabetes

<b>Objective:</b> As diabetes technology use in youth increases worldwide, inequalities in access may exacerbate disparities in hemoglobin A1c (HbA1c). We hypothesized an increasing gap in diabetes technology use by socioeconomic status (SES) would be associated with increased HbA1c disparities. <p> </p> <p><b>Research Design and Methods: </b>Participants aged <18 years with diabetes duration ≥1 year in the Type 1 Diabetes Exchange (T1DX, US, n=16,457) and Diabetes Prospective Follow-up (DPV, Germany, n=39,836) registries were categorized into lowest (Q1) to highest (Q5) SES quintiles. Multiple regression analyses compared the relationship of SES quintiles with diabetes technology use and HbA1c from 2010-2012 and 2016-2018. </p> <p> </p> <p><b>Results: </b>HbA1c was higher in participants with lower SES (in 2010-2012 & 2016-2018, respectively: 8.0% & 7.8% in Q1 and 7.6% & 7.5% in Q5 for DPV; and 9.0% & 9.3% in Q1 and 7.8% & 8.0% in Q5 for T1DX). For DPV, the association between SES and HbA1c did not change between the two time periods, whereas for T1DX, disparities in HbA1c by SES increased significantly (p<0.001). After adjusting for technology use, results for DPV did not change whereas the increase in T1DX was no longer significant.</p> <p> </p> <p><b>Conclusions: </b>Although causal conclusions cannot be drawn, diabetes technology use is lowest and HbA1c is highest in those of the lowest SES quintile in the T1DX and this difference for HbA1c broadened in the last decade. Associations of SES with technology use and HbA1c were weaker in the DPV registry. </p>

scholarly journals Longitudinal Changes of Tongue Thickness and Tongue Pressure in Neuromuscular Disorders

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
George Umemoto ◽  
Shinsuke Fujioka ◽  
Hajime Arahata ◽  
Nobutaka Sakae ◽  
Naokazu Sasagasako ◽  
...  
Keyword(s):  
Neuromuscular Disorders ◽  
The Other ◽  
Tongue Pressure ◽  
Swallowing Function ◽  
Longitudinal Changes ◽  
Swallowing Dysfunction ◽  
The Relationship ◽  
Lateral Sclerosis

Abstract Background Swallowing dysfunction is related to major cause of adverse events and an indicator of shorter survival among patients with neuromuscular disorders (NMD). It is critical to assess the swallowing function during disease progression, however, there are limited tools that can easily evaluate swallowing function without using videofluoroscopic or videoendoscopic examination. Here, we evaluated the longitudinal changes in tongue thickness (TT) and maximum tongue pressure (MTP) among patients with amyotrophic lateral sclerosis (ALS), myotonic dystrophy type 1 (DM1), and Duchenne muscular dystrophy (DMD). Methods Between 2010 and 2020, TT and MTP were measured from 21 ALS, 30 DM1, and 14 DMD patients (mean ages of 66.9, 44.5, and 21.4 years, respectively) at intervals of more than half a year. TT was measured, by ultrasonography, as the distance from the mylohyoid muscle raphe to the tongue dorsum, and MTP was determined by measuring the maximum compression on a small balloon when pressing the tongue against the palate. Then we examined the relationship between these evaluations and patient background and swallowing function. Results Mean follow-up periods were 24.0 months in the ALS group, 47.2 months in the DM1group, and 61.1 months in the DMD group. The DMD group demonstrated larger first TT than the other groups, while the DM1 group had lower first MTP than the ALS group. The ALS group showed a greater average monthly reduction in mean TT than the DM1 group and greater monthly reductions in mean body weight (BW) and MTP than the other groups. Significant differences between the first and last BW, TT, and MTP measures were found only in the ALS group. Conclusions This study suggests that ALS is associated with more rapid degeneration of tongue function over several years compared to DMD and DM1.

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