scholarly journals Epidemiological and clinical aspects of immunoglobulin A vasculitis in childhood: a retrospective cohort study

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Breda Luciana ◽  
Carbone Ilaria ◽  
Casciato Isabella ◽  
Cristina Gentile ◽  
Eleonora Agata Grasso ◽  
...  

Abstract Background A retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schӧnlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo. Methods This is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria. Results Two-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients. Conclusions Hereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura.

2020 ◽  
pp. archdischild-2019-318677
Author(s):  
Steven Hirschfeld ◽  
Florian B Lagler ◽  
Jenny M Kindblom

Children have the right to treatment based on the same quality of information that guides treatment in adults. Without the proper evaluation of medicinal products and devices in paediatric clinical trials that are designed to meet the rigorous standards of the competent authorities, children are discriminated from advances in medicine. There are regulatory, scientific and ethical incentives to address the knowledge gap regarding efficacy and safety of medicines in the paediatric population. High-quality clinical trials involving children of all ages can generate data that will ultimately close the knowledge gaps and support decision making.For clinical trials that enrol children, the needs are specialised and often resource intensive. Prerequisites for successful paediatric clinical trials are personnel with training in both paediatrics and neonatology and expertise in clinical trials in these populations. Moreover, national and international networks for efficient collaboration, dissemination of information, and sharing of resources and expertise are also needed, together with competent, efficient and high-quality local infrastructure with effective processes. Monitoring and oversight bodies with the relevant competence, including expertise in paediatrics, is also an important prerequisite for paediatric clinical trials. Compromise in any of these components will compromise the downstream results.This paper discusses the structures and competences needed in order to perform effective, high-quality paediatric clinical trials with the ultimate goal of better medicines and treatments for children. We propose a model of examining the process as a series of components that each has to be optimised, then all the components are actively optimised to function together as an ecosystem, and the resulting ecosystem functions well with the general research system and the healthcare delivery system.


2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Annisa Meivira Budiman ◽  
Sumartini Dewi ◽  
Marietta Shanti Prananta

Background Systemic sclerosis is a chronic progressive multisystem autoimmune disease in connective tissue, characterized by its heterogeneous clinical manifestation. The purpose of this study is to give information regarding clinical manifestations and laboratory findings of systemic sclerosis patients to establish diagnosis of disease. Methods This study was conducted using descriptive quantitative design in September−October 2016. Data was collected from medical records of patients visiting Rheumatology Clinic Dr. Hasan Sadikin General Hospital from 1 July 2015−30 June 2016 using total sampling method. The collected data were expected to comprise patient’s clinical manifestation and laboratory finding. Results Most of patients had cutaneous 57 (100.0%) and musculoskeletal 40 (70.2%) involvement. Some of the disease manifestations were Raynaud’s phenomenon 38 (66.7%), fingertip lesion 33 (57.9%), stiffness in skin 34 (59.6%), and arthalgia 29 (50.9%). Gastrointestinal involvements were present in 29 (50.9%) patients. Renal involvement were determined from urinalysis result showed proteinuria 10 (17.5%) and hematuria 8 (14.0%), found in 24 (42.1%) patients, while pulmonary and cardiac involvements were found in 30 (52.6%) patients, acknowledged from clinical symptoms such as dyspnea 12 (21.1%). Identification of autoantibodies was found in 12 (21.1%) patients, with 10 (17.5%) patients had reactive ANA and 3 (3.5%) had positive anti-Scl70. Conclusion Most of systemic sclerosis patients had cutaneous involvement. Renal, pulmonary, and cardiac involvement were concluded based on laboratory findings. Keywords: Systemic sclerosis, clinical manifestation, laboratory finding


2020 ◽  
Vol 8 ◽  
pp. 232470962092556 ◽  
Author(s):  
Amanda S. Weissman ◽  
Viral Sanjay Patel ◽  
Omar Mushfiq

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.


2021 ◽  
Author(s):  
Valbona Selimaj Kontoni ◽  
Philippe Lepage ◽  
Marc Hainaut ◽  
Véronique Yvette Miendje Deyi ◽  
Wesley Mattheus ◽  
...  

Abstract BackgroundEnteric fever (EF) is a major public health problem and a witness of the global health disparities. It is caused by Salmonella enterica serovar typhi (Salmonella ser. Typhi) and Salmonella enterica serovar Paratyphi A,B,C (Salmonella ser. Paratyphi) and is estimated to infect 12-26 million persons yearly.Paediatric data on enteric fever in Europe are scarce. A case series of EF was analysed to describe the clinical presentation, laboratory characteristics and diagnostic challenges identified in a paediatric population in Brussels.MethodsWe performed a retrospective study of all lab-confirmed cases of enteric fever in children aged 0-15 years at two Brussels teaching hospitals, between January 2005 and December 2020. We reviewed age, gender, travel history, consultations before diagnosis, hospitalisation duration, clinical symptoms and laboratory findings.ResultsThere were 34 positive isolates of S.typhi and S.paratyphi: 31 patients had positive blood culture, 1 patient had positive bone drainage, 2 patients had positive stool culture (one was excluded for missing data). There were 20 girls(60%). Median age was 3.5 years (range 5 months to 14 years). Travel to EF endemic areas was present in 55% of patients. Diagnosis was delayed in 80% of children. Eosinopenia was present in 93% of the cohort. The patients had not received any preventive travel education or vaccination.ConclusionsEnteric fever poses diagnostic challenges to clinicians. Eosinopenia in a febrile patient coming from the tropics should raise suspicion of EF. Travelers to endemic areas, should be better educated about EF risks and typhoid fever vaccination must be promoted.


2018 ◽  
Vol 3 (3) ◽  
pp. 130
Author(s):  
Aqsa Iqbal ◽  
Nicole Stahl ◽  
Erin M Davis

Introduction: Immunoglobulin A vasculitis (formerly known as Henoch Schonlein Purpura) is the most common pediatric vasculitis, which occurs typically at the age of 3-15 years. Mononucleosis, group A Streptococcus, Campylobacter and Mycoplasma are some of the common infectious causes of immunoglobulin A vasculitis. Immunoglobulin A vasculitis is a clinically diagnosed disease. Most common clinical features include nonthrombocytopenic nonpruritic palpable purpura, gastrointestinal involvement, arthritis or arthralgia and renal involvement. Biopsy of skin and/or gastrointestinal lesions con rm the diagnosis, although this is not necessary. Immunoglobulin A vasculitis is a self-limiting disease, which resolves spontaneously. Steroids can be used for the treatment of moderate to severe disease and for the prevention of renal complications. Prognosis relies upon various factors among which involvement of kidneys dictates poor prognosis and requires close follow up. Case Presentation: We are presenting a case of immunoglobulin A vasculitis in a 39-year-old German male following in uenza virus infection. The infectious agent that cause immunoglobulin A vasculitis in our patient was In uenza A virus. Biopsy of the skin lesion con rmed the diagnosis of immunoglobulin A vasculitis in our patient. The patient responded to steroids and his skin and GI ndings resolved. Conclusion: Our case report adds to the literature of medicine by describing in uenza virus as a cause of immunoglobulin A vasculitis in young adult patients. Considering immunoglobulin A vasculitis after in uenza and outside of the typical age of 3-15 years, can help to make earlier diagnosis and prevent complications.


Dermatology ◽  
2021 ◽  
pp. 1-8
Author(s):  
Zexing Song ◽  
Yingli Nie ◽  
Liu Yang ◽  
Juan Tao

<b><i>Background:</i></b> Immunoglobulin A vasculitis (IgAV) is the most common vasculitis in children. Although childhood IgAV is generally considered as a self-limited disease, progressive course and poor prognosis could occur in some cases which mostly result from severe renal involvement and gastrointestinal (GI) involvement. <b><i>Methods:</i></b> We performed a retrospective study of pediatric patients diagnosed as IgAV in our institution from 2016 to 2019. Patients were divided into groups based on the occurrence and severity of GI and renal involvement. Analysis of variance (ANOVA) and Kruskal-Wallis test were used to compare results of laboratory parameters among groups and prediction models were built by using logistic regression analysis. <b><i>Results:</i></b> A total of 286 patients were enrolled. GI involvement occurred in 148 (51.7%) patients, 30 (20.3%) of which were severe cases. Renal involvement developed in 120 (42.0%) patients, 22 (18.3%) of which were severe cases. Compared with patients with only cutaneous manifestations, white blood cell (WBC) count, neutrophil-to-lymphocyte ratio (NLR), and D-dimer levels were higher in those with GI involvement, and D-dimer level was found to be positively associated with severity. Increased NLR and lower complement 3 (C3) were found in patients with renal involvement, but only C3 was relevant in distinguishing moderate and severe cases. The prediction model for severe renal involvement was: Logit (P) = 6.820 + 0.270 (age) + 0.508 (NLR) − 16.130 (C3), with an AUC of 0.914. The prediction model for severe GI involvement was: Logit (P) = −5.459 + 0.005 (WBC) + 1.355 (D-dimer) – 0.020 (NLR), with an AUC of 0.849. <b><i>Conclusion:</i></b> Our data suggest C3 to be an exclusive predictor for severe renal involvement and D-dimer level to be positively associated with the severity of GI involvement. Prediction models consisting of the above parameters were built for obtaining prognostic information in the early phase of IgAV.


2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Annisa Meivira Budiman ◽  
Sumartini Dewi ◽  
Marietta Shanti Prananta

Background Systemic sclerosis is a chronic progressive multisystem autoimmune disease in connective tissue, characterized by its heterogeneous clinical manifestation. The purpose of this study is to give information regarding clinical manifestations and laboratory findings of systemic sclerosis patients to establish diagnosis of disease. Methods This study was conducted using descriptive quantitative design in September−October 2016. Data was collected from medical records of patients visiting Rheumatology Clinic Dr. Hasan Sadikin General Hospital from 1 July 2015−30 June 2016 using total sampling method. The collected data were expected to comprise patient’s clinical manifestation and laboratory finding. Results Most of patients had cutaneous 57 (100.0%) and musculoskeletal 40 (70.2%) involvement. Some of the disease manifestations were Raynaud’s phenomenon 38 (66.7%), fingertip lesion 33 (57.9%), stiffness in skin 34 (59.6%), and arthalgia 29 (50.9%). Gastrointestinal involvements were present in 29 (50.9%) patients. Renal involvement were determined from urinalysis result showed proteinuria 10 (17.5%) and hematuria 8 (14.0%), found in 24 (42.1%) patients, while pulmonary and cardiac involvements were found in 30 (52.6%) patients, acknowledged from clinical symptoms such as dyspnea 12 (21.1%). Identification of autoantibodies was found in 12 (21.1%) patients, with 10 (17.5%) patients had reactive ANA and 3 (3.5%) had positive anti-Scl70. Conclusion Most of systemic sclerosis patients had cutaneous involvement. Renal, pulmonary, and cardiac involvement were concluded based on laboratory findings. Keywords: Systemic sclerosis, clinical manifestation, laboratory finding


2020 ◽  
Vol 26 (41) ◽  
pp. 5261-5277
Author(s):  
Peter J. Wilkin ◽  
Minnatallah Al-Yozbaki ◽  
Alex George ◽  
Girish K. Gupta ◽  
Cornelia M. Wilson

On 11th March 2020, the World Health Organisation (WHO) announced a pandemic caused by a novel beta-coronavirus SARS-CoV-2, designated COVID-19. The virus emerged in December 2019 in Wuhan, China, has spread across the world as a global pandemic. The traditional use of medicines from plants can be traced back to 60,000 years. Global interest in the development of drugs from natural products has increased greatly during the last few decades. Essential oils (EOs) have been studied through the centuries and are known to possess various pharmaceutical properties. In the present review, we have highlighted the current biology, epidemiology, various clinical aspects, different diagnostic techniques, clinical symptoms, and management of COVID-19. An overview of the antiviral action of EOs, along with their proposed mechanism of action and in silico studies conducted, is described. The reported studies of EOs&#039; antiviral activity highlight the baseline data about the additive and/or synergistic effects among primary or secondary phytoconstituents found in individual oils, combinations or blends of oils and between EOs and antiviral drugs. It is hoped that further research will provide better insights into EOs&#039; potential to limit viral infection and aid in providing solutions through natural, therapeutically active agents.


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