scholarly journals Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review

Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 818
Author(s):  
Leah Shin ◽  
Jeffrey T. Gardner ◽  
Harry Dao

Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the setting of inconclusive preliminary results. Dapsone is a commonly used treatment, but many therapeutic agents have emerged in recent years. The objective of this study is to provide a comprehensive overview of updates on the diagnosis and management of LAD. Materials and Methods: A literature search was conducted from May to June of 2021 for articles published in the last 5 years that were related to the diagnosis and management of LAD. Results: False-negative results in cases of drug-induced LAD and the presence of IgG and IgM antibodies on immunofluorescence studies were reported. Serration pattern analysis has been reported to be useful in distinguishing LAD from sublamina densa-type LAD. Rituximab, omalizumab, etanercept, IVIg, sulfonamides, topical corticosteroids, and others have been used successfully in adult and pediatric patients with varying disease severity. Topical corticosteroids were preferred for pediatric patients while rituximab and IVIg were used in adults with recalcitrant LAD. Sulfonamides were utilized in places without access to dapsone. Conclusion: In cases where preliminary biopsy results are negative and clinical suspicion is high, repeat biopsy and additional diagnostic studies should be used. Patient factors such as age, medical comorbidities, and disease severity play a role in therapeutic selection.

Author(s):  
Mohammad Nabavi ◽  
Afshin Rezaeifar ◽  
Ali Sadeghinia ◽  
Saba Arshi ◽  
Sima Bahrami ◽  
...  

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may be triggered by some diseases and medications. For the latter one, non-steroidal anti-inflammatory drugs (NSAIDs) have been identified as one of the potential causative agents to develop LABD. Here, a rare case of drug-induced LABD is introduced. A 13-month-old Iranian boy presented with a history of generalized blisters, displaying the classic “string of pearls” sign who was eventually diagnosed as a case of LABD. In his admission, he was diagnosed whit Mucocutaneous lymph node syndrome and treated with aspirin.  Some features like appearing the characteristic lesions one week following the administration of aspirin, rapid clearance of lesions after the withdrawal of the drug, and reappearance of new lesions after readministration of aspirin were highly suggestive of aspirin-induced LABD. To establish the diagnosis, we used the “Naranjo probability score” which determined the probable causative role of aspirin. The diagnosis was confirmed by showing the positive IgA deposition in the basement membrane zone in a direct immunofluorescence study of the skin biopsy. The child was treated with dapsone with dramatical response to the drug.


Diagnostics ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1091
Author(s):  
Ali A. Rabaan ◽  
Raghavendra Tirupathi ◽  
Anupam A Sule ◽  
Jehad Aldali ◽  
Abbas Al Mutair ◽  
...  

Real-time RT-PCR is considered the gold standard confirmatory test for coronavirus disease 2019 (COVID-19). However, many scientists disagree, and it is essential to understand that several factors and variables can cause a false-negative test. In this context, cycle threshold (Ct) values are being utilized to diagnose or predict SARS-CoV-2 infection. This practice has a significant clinical utility as Ct values can be correlated with the viral load. In addition, Ct values have a strong correlation with multiple haematological and biochemical markers. However, it is essential to consider that Ct values might be affected by pre-analytic, analytic, and post-analytical variables such as collection technique, specimen type, sampling time, viral kinetics, transport and storage conditions, nucleic acid extraction, viral RNA load, primer designing, real-time PCR efficiency, and Ct value determination method. Therefore, understanding the interpretation of Ct values and other influential factors could play a crucial role in interpreting viral load and disease severity. In several clinical studies consisting of small or large sample sizes, several discrepancies exist regarding a significant positive correlation between the Ct value and disease severity in COVID-19. In this context, a revised review of the literature has been conducted to fill the knowledge gaps regarding the correlations between Ct values and severity/fatality rates of patients with COVID-19. Various databases such as PubMed, Science Direct, Medline, Scopus, and Google Scholar were searched up to April 2021 by using keywords including “RT-PCR or viral load”, “SARS-CoV-2 and RT-PCR”, “Ct value and viral load”, “Ct value or COVID-19”. Research articles were extracted and selected independently by the authors and included in the present review based on their relevance to the study. The current narrative review explores the correlation of Ct values with mortality, disease progression, severity, and infectivity. We also discuss the factors that can affect these values, such as collection technique, type of swab, sampling method, etc.


2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Davide Geat ◽  
Mattia Giovannini ◽  
Ezio Gabriele Barlocco ◽  
Riccardo Pertile ◽  
Stefania Farina ◽  
...  

Abstract Background Several studies have investigated the efficacy of balneotherapy in atopic dermatitis (AD), including a pediatric open randomized clinical trial conducted at the Comano thermal spring water center, which showed a significant reduction in AD severity and an improvement of the quality of life. However, so far many studies on balneotherapy in pediatric AD have included relatively small populations without identifying patients’ characteristics associated with their response. The aim of the present study was to identify any features associated with the clinical response to the Comano thermal spring water balneotherapy in a large cohort of pediatric AD patients. Methods An observational study was conducted on 867 children aged ≤16 years (females 50.5%, mean patient’s age 5.9 years, standard deviation ±3.6 years) with mild to severe AD who underwent balneotherapy at the Comano thermal spring water center (Comano, Trentino, Italy) from April to October 2014. Patients were stratified according to their disease severity, which was evaluated using five SCORing Atopic Dermatitis (SCORAD) categories before and immediately after a thermal spring water balneotherapy course. Potential characteristics associated with the patients’ clinical response to Comano thermal spring water balneotherapy were investigated. Results A statistically significant improvement in AD severity was observed after Comano thermal spring water balneotherapy (p < 0.0001). A significantly higher percentage of patients achieving improvement in AD severity was reported among children ≤4 years old (p < 0.0001) with early-onset AD (p < 0.0001), severe AD (p < 0.0001) or coexistent reported food allergies (p < 0.01). The therapy was well tolerated, and no relevant adverse effects were reported during the treatment course. Conclusions Comano thermal spring water balneotherapy is a safe complementary treatment for pediatric patients with AD, as it was able to reduce the disease severity, especially in children ≤4 years old, with early onset AD, severe AD or concomitant food allergies.


CNS Drugs ◽  
2021 ◽  
Vol 35 (3) ◽  
pp. 283-289
Author(s):  
Neeranjali S. Jain ◽  
Claire W. Ruan ◽  
Shanil R. Dhanji ◽  
Richard J. Symes

2013 ◽  
Vol 27 (3) ◽  
pp. 137-145 ◽  
Author(s):  
Donald M. Arnold ◽  
Ishac Nazi ◽  
Theodore E. Warkentin ◽  
James W. Smith ◽  
Lisa J. Toltl ◽  
...  

2021 ◽  
pp. 088307382110646
Author(s):  
Melissa A. Wright ◽  
Cristina C. Trandafir ◽  
Gary R. Nelson ◽  
Aimee O. Hersh ◽  
C. J. Inman ◽  
...  

Autoimmune encephalitis is an increasingly recognized entity in children. When treated promptly, favorable outcomes are seen in a majority of pediatric patients. However, recognition of autoimmune encephalitis in young patients is challenging. Once autoimmune encephalitis is suspected, additional difficulties exist regarding timing of treatment initiation and duration of treatment, as evidence to guide management of these patients is emerging. Here, we review available literature regarding pediatric autoimmune encephalitis and present our institution's comprehensive approach to the evaluation and management of the disease. These guidelines were developed through an iterative process involving both pediatric neurologists and rheumatologists.


2020 ◽  
pp. 089719002097775 ◽  
Author(s):  
Divita Singh ◽  
Mikayla Johnson ◽  
Caleah S. Kitchens ◽  
Andrew Boone

Prosthetic joint infections (PJIs) remain a major complication of arthroplasty, most of which are caused by Staphylococcus aureus and gram-negative bacteria. Unfortunately, cultures are false negative in upward of 7 percent of patients with suspected PJIs, and commonly in infections caused by rare rapidly growing mycobacterium (RGM) species. Guidelines recommend 6 months of antimycobacterial therapy for bone diseases caused by RGM, with empiric therapy consists of an oral macrolide (clarithromycin or azithromycin) plus tobramycin and imipenem-cilastatin. Definitive treatment of PJI due to RGM should be guided by antimicrobial susceptibility, however, most microbiology laboratories are unable to differentiate between M. chelonae and M. abscessus. Furthermore, treatment of M. chelonae PJI is challenging due to multidrug resistance and the dearth of oral antibiotics for therapy. This case report investigates a patient with PJI caused by M. chelonae and M. abscessus. The initial treatment with imipenem-cilastatin was complicated by drug induced seizures, further limiting therapy options.


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