scholarly journals Henoch-Schönlein Nephritis Manifesting with Purpura 15 years after Diagnosis of IgA Nephropathy

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Hideaki Yamabe ◽  
Mitsuaki Kaizuka ◽  
Satoru Tsunoda ◽  
Tasuku Nagasawa ◽  
Kazuo Nomura ◽  
...  

Henoch-Schönlein nephritis or immunoglobulin A (IgA) vasculitis is characterized by purpura, arthralgia, abdominal pain, and glomerulonephritis with glomerular IgA deposition. Notably, the presence of purpura is essential to diagnose this disease. We report the case of a patient in whom proteinuria and haematuria were detected during screening tests and he was diagnosed with IgA nephropathy at 20 years of age. Corticosteroids were administered for 7 years and were subsequently tapered. At 35 years of age, he noticed purpura on his lower extremities and was diagnosed with anaphylactoid purpura. Following the appearance of purpura, urinalysis revealed an increase in urinary protein levels from 0.7 g/g creatinine (Cr) to 1.4 g/gCr, and his serum Cr levels increased from 1.1 mg/dL to 1.35 mg/dL. Two months later purpura subsided, and his urinary protein level and serum Cr level were restored to the former levels. Although the cause remains unknown, an interval may occasionally be observed between the appearance of purpura and urinary abnormalities. However, to our knowledge to date, a 15-year interval is the longest interval, in such cases, reported in the literature.

2019 ◽  
Vol 10 (6) ◽  
pp. 33-38
Author(s):  
Sayani Chaudhuri ◽  
Utpal Kumar Biswas ◽  
Arun Kumar

Background: Preeclampsia is a hypertensive disorder of pregnancy affecting multiple systems and characterized chiefly by hypertension and proteinuria in a previously normotensive and non proteinuric women. The main underlying cause for its pathophysiology is an imbalance between the physiological vasoconstrictor and vasodilator molecules in circulation leading to maternal endothelial dysfunction. Hydrogen sulphide (H2S) is a physiological vasodilatory gasotransmitter which plays an important role in the development of hypertension and proteinuria in preeclampsia. Aims and Objectives: The aim of this study was to determine the serum level of hydrogen sulphide and spot urinary protein levels in preeclampsia cases and compare it with age matched controls which were normal pregnant women and to find any correlation, if exists, between these two parameters. Materials and Methods: Serum level of H2S and spot urinary protein level were measured in one hundred pregnant women with preeclampsia and the values were compared with age matched controls. Results: The mean serum H2S level was 32.31 ± 12.62μmol/L in patients which was significantly lower (p<0.001) when compared to controls where mean was 114.50 ±20.35μmol/L. The mean spot urinary protein level was found to be 11.83 ± 5.06 mg/dl in preeclampsia cases which was significantly higher (p<0.001) than in controls where it was 7.18 ± 2.38 mg/dl. A negative correlation was found between the serum level of H2S and both the systolic BP (r=-0.725, p<0.001) and diastolic BP (r= - 0.639, p<0.001) in preeclampsia patients.A negative correlation was also observed between the serum levels of H2S and spot urinary protein in preeclampsia (r=-0.541, p<0.001). Conclusion: The present study has elucidated that the serum levels of hydrogen sulphide decreases and the spot urinary protein levels increases in preeclampsia when compared to normal pregnant women and hydrogen sulphide shows a negative correlation with both systolic and diastolic BP in preeclampsia. This study also demonstrates that,there exists a negative correlation between the serum H2S level and spot urinary protein level in preeclampsia patients.


2019 ◽  
Vol 2019 ◽  
pp. 1-11 ◽  
Author(s):  
Satoshi Ida ◽  
Ryutaro Kaneko ◽  
Kanako Imataka ◽  
Kazuya Murata

Previous studies involving patients with diabetes have indicated that sarcopenia is related to renal function. The objective of the present study was to investigate the association between sarcopenia and urinary albumin level, urinary protein level, and estimated glomerular filtration rate (eGFR) in patients with diabetes. A meta-analysis of observational studies was conducted. A literature search was performed using MEDLINE, Cochrane Controlled Trials Registry, and ClinicalTrials.gov. Data were extracted from studies investigating the association between sarcopenia and urinary albumin level, urinary protein level, and eGFR and by calculating odds ratio (OR) and 95% confidence intervals (CIs). Statistical analysis was performed using a random-effects model to calculate pooled OR and 95% CI. Six studies (2662 patients) that met the criteria were included in the meta-analysis. Sarcopenia was significantly associated with urinary albumin level with a pooled OR of 2.11 (95% CI, 1.55–2.88; P<0.001). The pooled ORs of the associations between sarcopenia and urinary protein level and decreased eGFR were 1.82 (95% CI, 1.13–2.92; P=0.01) and 3.75 (95% CI, 1.24–11.41), respectively. Sarcopenia was significantly associated with urinary albumin level, urinary protein level, and decreased eGFR. However, further investigations are needed, including meta-analyses with a larger number of studies.


Author(s):  
Lu Yang ◽  
Yan Wang ◽  
Aobulikasimu Nuerbiye ◽  
Ping Cheng ◽  
Jin-Hui Wang ◽  
...  

Periostracum cicadae, the cast-off shell of the cicada Cryptotympana pustulata Fabricius, is used in traditional Chinese medicine for its diaphoretic, anticonvulsive, sedative, antipyretic, and antiallergic effects. However, the exact pathogenesis of immunoglobulin A nephropathy (IgAN) remains unclear, thereby hindering investigations to identify novel therapeutic agents. A rat IgAN model was established by administration of bovine serum albumin, lipopolysaccharide, and carbon tetrachloride, which simultaneously established blood stasis and a heat syndrome model. The animals were sacrificed to detect changes in protein levels in urine and blood. Immunofluorescence was performed to assess IgA deposition in the glomeruli. Tumor necrosis factor-&alpha; (TNF-&alpha;), interleukin-1&beta; (IL-1&beta;), and interleukin 6 (IL-6) levels were measured in bronchoalveolar lavage fluid (BALF) by enzyme-linked immunosorbent assay. Hematology and eosin, periodic acid-Schiff, TUNEL, and immunohistochemical staining were performed to evaluate histopathological changes in kidney tissues. Additionally, target-related proteins were measured by western blotting. Periostracum cicadae resulted in a reduction in blood and urine protein levels. Serum TNF-&alpha;, IL-1&beta;, and IL-6 levels significantly decreased in the periostracum cicadae-treated groups compared to the IgAN group. Furthermore, a reduction in MCP-1, TLR4, and IgA expression levels and a dose- dependent increase in caspace-3 expression were observed in response to periostracum cicadae treatment. TGF-&beta;1 levels decreased, whereas that of Fas increased in the kidney tissues of the periostracum cicadae-treated groups. The findings of the present study indicate that periostracum cicadae induces apoptosis and improves kidney inflammation and fibrosis in IgA nephropathy rat models.


Author(s):  
Raghvendra Narayan ◽  
Shivani Singh

Nephrotic syndrome is a common renal problem in childhood and is characterised by generalised oedema, massive proteinuria, hypoalbuminemia and hyperlipidemia. There are various laboratory methods to quantify proteinuria. Among them 24-hour urinary protein estimation is considered a gold standard for diagnosis of nephrotic syndrome. Nephrotic range proteinuria is considered when 24-hour urinary protein is more than 40 mg/m2/hr. There is scarce literature available regarding the changes in quantitative proteinuria when there is marked hypoalbuminemia (serum albumin less than 2.5 gm/dL). This series is about three patients of nephrotic syndrome (6 yers old male, 4 years old male and 5 years old male), having marked hypoalbuminemia and their 24-hour urinary protein level resulted into non-nephrotic range. All the patients underwent relevant physical, clinical examinations and laboratory blood and urine investigations(Haemoglobin, Mantoux test, chest x-ray, urine routine, urine culture and sensitivity, lipid profile, serum albumin and 24 hour urinary protein). All the cases were managed with Prednisolone and diuretics like Furosemide and were followed up till the subside of proteinuria and oedema conditions.


1997 ◽  
Vol 8 (5) ◽  
pp. 817-819
Author(s):  
Y Terada ◽  
T Yamada ◽  
O Nakashima ◽  
S Sasaki ◽  
H Nonoguchi ◽  
...  

This study investigated the mRNA expression of the platelet-derived growth factor (PDGF) A-chain and B-chain and PDGF-beta receptor in glomeruli of 15 immunoglobulin A (IgA) nephropathy kidneys and those with minimal-change lesion (N = 7), membranous nephropathy (N = 3), and focal segmental glomerulonephritis (N = 5), by using competitive RT-PCR methods. The level of PDGF B-chain and beta receptor mRNA expression in IgA nephropathy was significantly higher than in the other forms of glomerulonephritis, but mRNA expressions of PDGF A-chain were not significantly different. Significant correlations were observed between the urinary protein level and the mRNA level of PDGF-beta receptor expression and PDGF B-chain expression, and between the serum creatinine level and the mRNA level of PDGF-beta receptor expression. The PDGF B-chain and beta-receptor may be upregulated and accelerate cell proliferation in a paracrine or autocrine manner and may play a role in the pathogenesis of IgA nephropathy.


2019 ◽  
Vol 9 (1) ◽  
pp. 08-08 ◽  
Author(s):  
Mohsen Akhavan Sepahi ◽  
Muhammed Mubarak

Immunoglobulin A vasculitis nephritis (IgAVN) or Henoch-Schönlein purpura nephritis (HSPN) is the most common type of secondary IgA nephropathy (IgAN), particularly in children. Overall, its prognosis is good, but in a significant number of cases, it does lead to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Various classifications exist to predict the long-term outcome of kidney disease in these diseases, but none is universally accepted. We searched PubMed, Web of Science Embase, EBSCO, Scopus and directory of open access journals (DOAJ) with keywords of Henoch-Schönlein purpura nephritis, immunoglobulin A vasculitis nephritis, immunoglobulin A nephropathy, IgA vasculitis, extracapillary proliferation, IgA nephropathy, leukocytoclastic vasculitis, endstage renal disease, Oxford classification, crescent and childhood IgA vasculitis. IgAVN in children presents most often with crescents and endocapillary proliferation, with relapsing and remitting course clinically. Due to morphological resemblance of IgAVN and IgAN, the Oxford MEST-C scores could be applied for determining the long-term outcomes in the former disease. A critical concern in applying Oxford classification for IgAVN is that limited number of children with IgAVN exists in any one center and also a relatively short period of follow-up. Hence, further work in this regard is necessary. Preliminary evidence suggests that Oxford MEST-C classification is valid in predicting long-term kidney outcomes in children with IgAVN and the classification can also be used in adults. However, further, large scale, multicenter, international collaborative studies are needed to address the unmet issues.


2016 ◽  
Vol 25 (2) ◽  
pp. 235-238 ◽  
Author(s):  
Camelia Cojocariu ◽  
Carol Stanciu ◽  
Codrina Ancuta ◽  
Mihai Danciu ◽  
Stefan Chiriac ◽  
...  

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary.We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature.Abbreviations: CDI: Clostridium difficile infection; CRP: C-reactive protein; EGD: esophagogastroduodenoscopy; ESR: erythrocyte sedimentation rate; IgA: immunoglobulin A; WBC: white blood cell.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
HIROAKI TANAKA ◽  
Takayuki Fujii ◽  
JUNYA KOSHIZAKA ◽  
NOBUAKI YAMAUCHI ◽  
MAYU MORIMOTO ◽  
...  

Abstract Background and Aims In the treatment of idiopathic focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome, the remission of proteinuria is considered to be an important goal. The partial remission of proteinuria improves renal survival, whereas it may progressively reduce the renal function. A study searched for a novel partial remission more accurately reflecting the long-term renal outcome. The goal of proteinuria reduction for improving the renal prognosis remains to be clarified. We examined factors associated with the long-term renal outcome of idiopathic FSGS. Method Of 148 patients with FSGS diagnosed based on kidney biopsy between 1981 and 2018, a retrospective cohort study was conducted involving 33 who had undergone immunosuppressive therapy for nephrotic syndrome, and had been followed-up for ≥1 year, excluding those with secondary FSGS. We examined the renal prognosis, regarding a 50% decrease in the estimated glomerular filtration rate (eGFR) as an outcome. We calculated the rate of decrease in the urinary protein level 4 and 8 months after the start of treatment, and estimated the rate of decrease associated with renal hypofunction using ROC analysis. Based on the results of ROC analysis, Cox’s proportional hazard analysis was performed using factors contributing to renal hypofunction as covariates. Results Concerning the background of the 33 patients, the mean follow-up period was 11.4 years, and there were 24 males. The mean age was 49.8 years, and the mean blood pressure was 100.5 mmHg. The mean urinary protein level, albumin (Alb) level, eGFR, and total cholesterol (TCho) level were 7.4 g/day, 2.1 g/dL, 44.3 mL/min/1.73 m2, and 369 mg/dL, respectively. Corticosteroid therapy was selected in 21 patients, whereas it was combined with steroid pulse therapy in 12. The daily dose of prednisolone was 37.3 mg. On ROC analysis, the rate of decrease in the urinary protein level after 4 months was 83.1% (AUC: 0.74, sensitivity: 0.80, specificity: 0.74), and that after 8 months was 85.7% (AUC: 0.78, sensitivity: 0.90, specificity: 0.65). Cox’s proportional hazard analysis, in which the data were adjusted with the sex, blood pressure, urinary protein level at the start of treatment, Alb level, eGFR, and treatment methods, showed that the rate of decrease in the urinary protein level after 4 months was significantly correlated with renal hypofunction: after 4 months: hazard ratio, 0.19 (95%CI: 0.04-0.77); p=0.0202; after 8 months: hazard ratio, 0.34 (95%CI: 0.05-1.37); p=0.1359. Conclusion In the treatment of idiopathic FSGS with nephrotic syndrome, the rate of decrease in the urinary protein level 4 months after the start of treatment was correlated with the long-term renal outcome.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Songtao Feng ◽  
Yueming Gao ◽  
Jingyuan Cao ◽  
Di Yin ◽  
Linli Lv ◽  
...  

Abstract Background and Aims In recent years, it has been found that targeted mRNA detection of sediment cells in urine can be used as novel biomarkers for the diagnosis of diabetic nephropathy (DN). However, its value in predicting the progression of DN is not clear. The purpose of this study is to seek for urinary mRNA markers that evaluate the prognosis of DN through systems biological screening, clinical verification and prospective studies. Method GEO database and “Nephroseq” platform were searched, and the transcriptome data of DN glomeruli and tubules and their clinical information were obtained. Weighted gene co-expression network analysis (WGCNA) combined with gene ontology (GO) annotation and KEGG pathway enrichment analysis were used to screen the hub genes negatively related to eGFR, and the hub genes were used as candidate markers for mRNA detection in urine sediment in DN patients. A total of 91 patients with DN diagnosed by renal biopsy were included, and 60 patients with type 2 diabetes and 61 healthy people were selected as the control groups. The mRNA expression of candidate molecules was detected by Taqman probe quantitative PCR, and the correlation between mRNA expression and eGFR and urinary protein levels were analyzed. Patients with DN were followed up for a median time of 21 months, and the primary end point was defied as end stage renal disease or eGFR decreasing by more than 50%. Multivariate Cox regression was used to evaluate the value of mRNA in predicting DN progression. Results GSE30528 and GSE30529 datasets were selected for analysis, including mRNA expression data of 9 cases of DN and 13 cases of normal glomeruli; and 10 cases of DN and 12 cases of normal tubules respectively. The clinical data of the patients in this study, including gender, race, age and eGFR, were searched on the Nephroseq platform. The gene modules negatively related to eGFR were screened by WGCNA. GO and KEGG analysis showed that the main function of the gene modules in both datasets were related to the activation of inflammatory cells and chemokines pathway. Through the screening of hub genes and the comparison of expression levels, CCL5, CXCL1, CXCL6 and CXCL12 were finally obtained as candidate genes. Quantitative PCR showed that the levels of CCL5 and CXCL1 was significantly increased in DN group, CCL5 was negatively correlated with eGFR and positively correlated with urinary protein level, while CXCL1 was negatively correlated with eGFR, but had no significant correlation with urinary protein level. Multivariate Cox regression showed that eGFR, urinary protein level, degree of renal fibrosis and urinary CCL5 were independent risk factors of primary end point. Conclusion The activation of chemokine signal pathway in renal tissue is involved in the progression of DN. Urinary CCL5 mRNA can independently predict the prognosis of DN and may be served as a novel biomarker for the progression of DN.


2013 ◽  
Vol 84 (2) ◽  
pp. 410-411 ◽  
Author(s):  
Luca Rampoldi ◽  
Anna Köttgen ◽  
Olivier Devuyst

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