Clinical Manifestations and Pathogenesis of Tonsillar Focal Diseases: IgA Nephropathy and Palmoplantar Pustulosis

2011 ◽  
pp. 1-5 ◽  
Author(s):  
Yasuaki Harabuchi
Keyword(s):  
Iga Nephropathy ◽  
Clinical Manifestations ◽  
Palmoplantar Pustulosis

scholarly journals Immunoglobulin A Nephropathy as the First Clinical Presentation of Wilson Disease: A Case Report and Literature Review

2021 ◽  
Author(s):  
Yong-Zhe Zhang ◽  
Geng Jian ◽  
Ping He ◽  
Rui Yu ◽  
Mi Tian ◽  
...  
Keyword(s):  
Renal Function ◽  
Iga Nephropathy ◽  
Wilson Disease ◽  
Genetic Disorder ◽  
Immunoglobulin A ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Kidney Injury ◽  
Copper Metabolism ◽  
Normal Liver

Abstract Background: Wilson disease (WD) is a rare genetic disorder of copper metabolism. The difference in copper tissue accumulation lead to various clinical manifestations, including some atypical presentations. The complex clinical picture makes it easy to miss and misdiagnose, even delay the best chance for treatment. Case presentation: A 26-year-old male patient who had nephritis-range proteinuria and elevated serum creatinine. The renal pathology indicated Immunoglobulin A (IgA) nephropathy and tubular injury which was inconsistent with glomerular lesions. Cirrhosis was also detected by imaging examination. Considering both kidney injury and liver damage, WD was suspected. According to further detected results of abnormal copper metabolism, corneal Kayser-Fleischer rings(K-F rings), and genetic disorder of ATP7B gene, he was finally diagnosed as a case of WD.The patient was given oral penicillamine and zinc sulfate daily and he was also prescribed losartan to control proteinuria on the premise of monitoring renal function and blood pressure. During the 2 years follow-up, the patient’s 24h uric cooper dropped to normal. The sign of tremor hands disappeared. The Urine protein and renal function keep stable. The patient had normal liver function and maintained good quality of daily life. Conclusions: In some cases, IgA nephropathy patients with suspicious and unexplained neurological and liver symptoms cannot be ignored. They may eventually be diagnosed with WD.

scholarly journals Immunoglobulin A (IgA) Nephropathy in Protocol Graft Kidney Biopsy done at six months Post Transplantation in a Tertiary Care Center Hospital of Nepal

2017 ◽  
Vol 6 (1) ◽  
pp. 6-11
Author(s):  
Midhan Shrestha ◽  
Dibya Singh Shah
Keyword(s):  
Iga Nephropathy ◽  
Care Center ◽  
Tertiary Care ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
University Teaching ◽  
Time Interval ◽  
Post Transplantation ◽  
Protocol Biopsy ◽  
Post Transplant

Background: Renal transplantation is the treatment of choice for end stage renal disease. The focus of interest has been to increase the life of the transplanted graft. Recurrence of native kidney disease or occurrence of denovo glomerulonephritis has adverse effects in graft survival. Protocol graft biopsy done at fixed time interval after transplantation aids in early identification of post-transplant glomerulonephritis before development of clinical signs and symptoms. This study describes the incidence of post-transplant IgA Nephropathy in protocol renal graft biopsies done at six months post- transplantation.Materials and Methods: This is a hospital based observational descriptive study, done in Tribhuvan University Teaching Hospital, Kathmandu, Nepal, a tertiary medical referral center in the capital. Protocol biopsy of the graft kidney was performed at six months post-transplantation in all recipients who underwent kidney transplantation in this hospital between 2071 Kartik and 2072 Ashwin.Results: Protocol biopsy was performed in total 47 recipients. Mean age of the recipients was 33.7 years ±10.83 years. The study population consisted of 33 (70.2%) male and 14 (29.8%) female recipients. IgA Nephropathy was present in 6 (12.8%) recipients.Conclusion: Our study demonstrates that IgA Nephropathy does occur in patients with stable GFR and without any clinical or laboratory abnormalities. Protocol biopsy is valuable in detection of early histologic abnormalities before onset of clinical manifestations, thus helping in prompt management with aim to prolong the graft survival.Journal of Nobel Medical CollegeVolume 6, Number 1, Issue 10 (January-June, 2017)

scholarly journals Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

2020 ◽  
Vol 12 ◽  
pp. 1759720X2091286
Author(s):  
Shuang Liu ◽  
Mingwei Tang ◽  
Yihan Cao ◽  
Chen Li
Keyword(s):  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Epidemiological Studies ◽  
Sapho Syndrome ◽  
Palmoplantar Pustulosis ◽  
Disease Modifying Antirheumatic Drugs ◽  
Severe Acne ◽  
Relapsing Remitting ◽  
Systemic Manifestations ◽  
Inflammatory Drugs

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a spectrum of heterogeneous diseases characterized by osteoarticular and dermatological manifestations. Osteitis and hyperostosis are core clinical manifestations in SAPHO syndrome, typically affecting multiple areas and possibly progressing to irreversible osteoarticular damage. Most patients with SAPHO have cutaneous involvement, mainly manifested as palmoplantar pustulosis and severe acne. Systemic manifestations are uncommon but occasionally reported. Epidemiological studies suggest the annual prevalence of SAPHO syndrome varies from 0.00144 in 100,000 in Japanese individuals to fewer than 1 in 10,000 in White individuals. The precise etiopathogenesis of SAPHO remains unclear, but it is generally considered an autoinflammatory syndrome that may be related to various etiologies, such as immune dysfunction, infection and genetic predisposition. Owing to the relapsing–remitting disease course, the goal of management is to improve clinical symptoms and prevent disease progression. Various treatments, including nonsteroidal anti-inflammatory drugs, conventional disease-modifying antirheumatic drugs, bisphosphonates, biologics, and antibiotics, are promising options for alleviating the disease.

scholarly journals Clinical Significance of Galactose-Deficient IgA1 by KM55 in Patients with IgA Nephropathy

2019 ◽  
Vol 44 (5) ◽  
pp. 1196-1206 ◽  
Author(s):  
Kai Zhang ◽  
Qiongqiong Li ◽  
Yaru Zhang ◽  
Wenya Shang ◽  
Li Wei ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Clinical Significance ◽  
Clinical Manifestations ◽  
Renal Tissue ◽  
Healthy Controls ◽  
Activity Assessment ◽  
Activation Products ◽  
The Difference ◽  
Positive Correlations ◽  
High Level

Background: Aberrant galactose-deficient IgA1 molecules (Gd-IgA1) are important causal factors in IgA nephropathy (IgAN); however, the detection of Gd-IgA1 in IgAN is complicated and instable. A monoclonal antibody, KM55, which specifically recognizes Gd-IgA1 has been developed. In the present study, we further explored the clinical significance of Gd-IgA1 using KM55. Methods: In this study, we enrolled 75 patients with IgAN and 80 healthy controls and detected the plasma Gd-IgA1 levels using the KM55 ELISA method. We also stained ­mesangial Gd-IgA1 deposition using KM55. Results: We observed that the levels of plasma Gd-IgA1 in IgAN patients were elevated compared to the corresponding levels of healthy controls. Patients were divided into 2 groups based on the median of Gd-IgA1. Patients with high Gd-IgA1 levels had significantly higher levels of uric acid (UA) and IgA. The other clinical manifestations demonstrated that there were no differences in age, sex, blood pressure, initial proteinuria, hematuria, estimated glomerular filtration rate and Oxford pathological classification between the 2 groups of patients. In addition, positive correlations were observed between Gd-IgA1 and Bb, C3a, C4d and MAC. Mesangial Gd-IgA1 was positive in IgAN but negative in the normal renal tissue adjacent to neoplasm. We next analyzed the correlation between plasma Gd-IgA1 and mesangial Gd-IgA1 deposition. The results showed that a high level of plasma Gd-IgA1 was related to the deposition of mesangial Gd-IgA1, although the difference was not significant. Conclusion: We verified the elevated level of plasma and ­mesangial Gd-IgA1 in patients with IgAN by KM55, which provided an alternative, easy, and reliable tool for diagnosis and activity assessment of IgAN. The level of plasma Gd-IgA1 positively correlated with levels of UA, total IgA levels, and complement activation products.

scholarly journals Serum Soluble CD89-IgA Complexes Are Elevated in IgA Nephropathy without Immunosuppressant History

2020 ◽  
Vol 2020 ◽  
pp. 1-6 ◽  
Author(s):  
Haiting Wu ◽  
Xiaoyan Wang ◽  
Zhe Yang ◽  
Qing Zhao ◽  
Yubing Wen ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Healthy Subjects ◽  
Sandwich Elisa ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Healthy Individuals ◽  
Oxford Classification ◽  
Age And Gender ◽  
Capture Antibody ◽  
And Gender

Purpose. CD89 (FcαRI), the receptor of IgA, can shed from cells to form complexes with IgA in serum and is supposed to participate in the pathogenesis of IgA nephropathy (IgAN). There are contradictory results on their utility in clinical practice. This study is aimed at investigating whether sCD89-IgA complexes can help in the diagnosis or evaluation of the disease. Methods. A sandwich ELISA was established using anti-CD89 as a capture antibody and HRP-conjugated anti-IgA as a detection antibody. This method was used to measure serum levels of sCD89-IgA complexes in IgAN patients without immunosuppressant history and healthy subjects. Correlations between serum levels of sCD89-IgA complexes and disease severity were analyzed. Results. Serum sCD89-IgA complexes increased with age (P<0.001). IgAN patients had higher sCD89-IgA complex levels compared with age- and gender-matched normal healthy individuals (P<0.001). Serum sCD89-IgAN significantly predicted IgAN diagnosis (AUC = 0.762 (0.640-0.883), P<0.001). But sCD89-IgA complexes did not correlate with baseline clinical manifestations, oxford classification, or renal function deteriorate speed. Conclusions. Serum sCD89-IgA complexes can guide diagnosis of IgAN in patients without immunosuppressant history, but provide limited help in clinicopathologic prediction.

scholarly journals Immunoglobulin a nephropathy as the first clinical presentation of Wilson disease: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yong-Zhe Zhang ◽  
Geng Jian ◽  
Ping He ◽  
Rui Yu ◽  
Mi Tian ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Wilson Disease ◽  
Genetic Disorders ◽  
Genetic Disorder ◽  
Immunoglobulin A ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Kidney Injury ◽  
Copper Metabolism ◽  
Atypical Presentations

Abstract Background Wilson disease (WD) is a rare genetic disorder of copper metabolism. Differences in copper tissue accumulation lead to various clinical manifestations, including some atypical presentations. The complex clinical features of WD make diagnosis challenging, delaying the best chance for treatment. Case presentation We report a case of a 26-year-old man with nephritis-range proteinuria and elevated serum creatinine. The renal pathology indicated immunoglobulin A (IgA) nephropathy and tubular injury, which was inconsistent with glomerular lesions. Cirrhosis was also detected by imaging examination. Considering both kidney injury and liver damage, WD was suspected. Based on results showing abnormal copper metabolism, corneal Kayser–Fleischer rings, and genetic disorders in the ATP7B gene, the patient was finally diagnosed with WD. After treatment with oral penicillamine, zinc sulfate and losartan, the patient showed alleviation of both WD and nephropathy after 3 years of follow-up. He maintained a good quality of daily life. Conclusion This case highlights that unexplained neurological and liver symptoms in patients with IgA nephropathy can be clues for WD.

scholarly journals IgA nephropathy suspected to be combined with Fabry disease or Alport syndrome: a case report

2019 ◽  
Vol 48 (3) ◽  
pp. 030006051989129
Author(s):  
Wen Hao ◽  
Lina Ao ◽  
Chenli Zhang ◽  
Lei Zhu ◽  
Deqiong Xie
Keyword(s):  
Fabry Disease ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Alport Syndrome ◽  
Immunoglobulin A ◽  
Clinical Manifestations ◽  
Hereditary Diseases ◽  
Zebra Bodies ◽  
Gene Test ◽  
Laboratory Examinations

Immunoglobulin A (IgA) nephropathy is the most common glomerular disease, and it often manifests as persistent microscopic hematuria or gross hematuria. Fabry disease and Alport syndrome are hereditary diseases caused by mutation of genes, and these diseases are rare in China. At present, patients can be diagnosed with IgA nephropathy by clinical manifestations and laboratory examinations, but there is still controversy about the simultaneous diagnosis of Alport syndrome and Fabry disease in patients with IgA nephropathy. The present case was a 17-year-old girl with hematuria and proteinuria who underwent a renal biopsy. Light microscopy and immunofluorescence showed that IgA was deposited in the mesangium. Under electron microscopy, zebra bodies with a lamellated structure were detected. A gene test showed a COL4A3 gene mutation. The patient was administered prednisone 40 mg once a day and dispersible tablets of mycophenolate mofetil 0.75 g two times a day. The patient’s condition showed a trend of remission. The findings in our case emphasize the importance of renal biopsy and gene detection in hereditary kidney disease, especially for Fabry disease and its rare coexistence with Alport syndrome.

scholarly journals IgA Vasculitis and IgA Nephropathy: Same Disease?

2021 ◽  
Vol 10 (11) ◽  
pp. 2310
Author(s):  
Evangeline Pillebout
Keyword(s):  
Iga Nephropathy ◽  
Genetic Background ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Therapeutic Strategies ◽  
Iga Vasculitis ◽  
Therapeutic Trials ◽  
Renal Prognosis ◽  
Systemic Form

Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies.

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