IL‐1 receptor antagonist defect (DIRA) in a pediatric patient, receiving adalimumab with good clinical response

Author(s):  
Juan Carlos Bustamante‐Ogando ◽  
Selma Scheffler‐Mendoza ◽  
Marco Antonio Yamazaki‐Nakashimada ◽  
Marimar Saez‐de‐Ocariz
2021 ◽  
Author(s):  
Diego A Sandoval ◽  
Inés Rama ◽  
María Quero ◽  
Miguel Hueso ◽  
Francisco Gómez ◽  
...  

Abstract Hemodialysis patients present more morbidity and mortality risk in coronavirus disease 2019. In patients who may develop severe symptoms the process called “viral sepsis” seems to be a crucial mechanism. In those cases, the hemodialysis procedure provides an excellent tool to explore the benefit of some extracorporeal therapies. We reported the outcome of 4 hemodialysis patients with severe COVID-19 treated with Seraph®100 hemoperfusion device. Three of four cases presented a good clinical response after hemoperfusion. In conclusion, the treatment with Seraph®100 device may be a simultaneous treatment to improve the hemodialysis patients with severe SARS-CoV-2.


2013 ◽  
Vol 14 (S1) ◽  
Author(s):  
Zoe Ash ◽  
Richard Hodgson ◽  
Andrew Grainger ◽  
Sibel Z Aydin ◽  
Concepcion Castillo-Gallego ◽  
...  

2020 ◽  
Vol 8 ◽  
Author(s):  
Francesca I. Calò Carducci ◽  
Maria Antonietta De Ioris ◽  
Chiara Agrati ◽  
Rita Carsetti ◽  
Daniela Perrotta ◽  
...  

Background: In severe acute respiratory syndrome-related coronavirus (SARS-CoV-2) critically ill adults, hyperinflammation plays a key role in disease progression. The clinical manifestations of SARS-CoV-2 infection among children are much less severe compared with adult patients and usually associated with a good prognosis. However, hyperinflammation in SARS-CoV-2-infected pediatric patients has been described as pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 or as Kawasaki-like disease but is still little known, and optimal management has to be defined. The World Health Organization (WHO) on the 15th of May 2020 has developed a preliminary case definition for multisystem inflammatory disorder in children and adolescents with coronavirus disease 2019 (COVID-19) and stated for an urgent need to collect data on this condition. Here, we report two adolescent patients affected by COVID-19 presenting with multisystem inflammatory disorder, 3–4 weeks after the first symptoms of SARS-CoV-2 infection, treated with the interleukin-1 receptor antagonist anakinra and glucocorticoids with good clinical response.Cases: We report two patients chronically ill appearing, with high fever, severe gastrointestinal involvement, and increased biomarkers of inflammation onset 3–4 weeks after paucisymptomatic SARS-CoV-2 infection. They had no lung involvement, but abdominal ultrasound and CT scan showed thickening of the bowel wall. SARS-CoV-2 PCR was positive on ileum biopsy in both patients, whereas it was negative on other common sampled sites. They have been admitted to the pediatric intensive care unit and have been treated with a combination of anakinra 6–8 mg/kg/day i.v. and a standard dose of methylprednisolone 2 mg/kg/day in addition to lopinavir/ritonavir 400 mg q12h and low molecular weight heparin 100 UI/kg q12h with good clinical response.


1997 ◽  
Vol 1 (4) ◽  
pp. 193-195 ◽  
Author(s):  
Seung-Kyung Hann ◽  
Dunlu Chen ◽  
Jean-Claude Bystryn

Background: Vitiligo is associated with autoantibodies to melanocytes. The role of these antibodies in the pathogenesis of the disease is still unknown. Objective: The purpose of this study was to examine the role of vitiligo antibodies in the pathogenesis of the disease by studying whether or not there is a correlation between changes in their level and response to therapy with systemic steroid. Methods: Antibodies to the 40 to 45 kD, 75 kD, and 90 kD vitiligo antigens were measured prior to and following systemic steroid therapy in 10 patients with active vitiligo. Results: Four months following initiation of therapy, seven (78%) of nine patients with good clinical response to steroid treatment had a significant decrease in the level of vitiligo antibodies. By contrast, one patient who had no response to treatment had a slight increase in antibody levels. Conclusion: These findings suggest that one mechanism by which corticosteroids can cause repigmentation in vitiligo is by decreasing the level of vitiligo antibodies, and support the notion that vitiligo antibodies are involved in the pathogenesis of this disease.


2017 ◽  
Vol 8 (5) ◽  
pp. 755-761 ◽  
Author(s):  
J. Kim ◽  
B.S. Lee ◽  
B. Kim ◽  
I. Na ◽  
J. Lee ◽  
...  

The therapeutic effect of probiotics in atopic dermatitis (AD) remains controversial and varies according to the individual patient. We aimed to identify a population of AD patients with a good clinical response to probiotic treatment. We recruited 76 children with a median age of 7.1 years who suffered from moderate to severe AD. After a 2-week washout period, all patients were given Lactobacillus plantarum CJLP133 at a dosage of 1×1010 colony-forming units once a day for 12 weeks. We measured eosinophil counts in the peripheral blood, the proportion of CD4+CD25+Foxp3+ regulatory T (Treg) cells in CD4+ T cells, serum total immunoglobulin E (IgE) levels, and specific IgE against common allergens before the start of the treatment (T1) and at discontinuation (T2). Responders were defined as patients with at least a 30% reduction in the SCORing of AD (SCORAD) index after treatment. There were 36 responders and 40 non-responders after probiotic treatment. The median SCORAD was reduced from 29.5 (range 20.6-46.3) at T1 to 16.4 (range 6.3-30.8) at T2 in the responder group (P<0.001). In multivariable logistic regression analysis, a good clinical response was significantly associated with high total IgE levels (aOR 5.1, 95% CI 1.1-23.6), increased expression of transforming growth factor (TGF)-β (aOR 4.6, 95% CI 1.3-15.9), and a high proportion of Treg cells in CD4+ T cells (aOR 3.7, 95% CI 1.1-12.7) at T1. In the responder group, the proportion of Treg cells was significantly increased after 12 weeks of treatment (P=0.004), while TGF-β mRNA expression was decreased (P=0.017). Our results suggest that a subgroup of patients with a specific AD phenotype showing an immunologically active state (high total IgE, increased expression of TGF-β, high numbers of Treg cells) may benefit from probiotic treatment with L. plantarum CJLP133.


Author(s):  
A Jochems ◽  
A Martinez-Feito ◽  
C Plasencia ◽  
B Hernández-Breijo ◽  
A Mezcúa ◽  
...  

2009 ◽  
Vol 69 (2) ◽  
pp. 394-399 ◽  
Author(s):  
F Van den Bosch ◽  
B Manger ◽  
P Goupille ◽  
N McHugh ◽  
E Rødevand ◽  
...  

Objectives:To evaluate the effectiveness of adalimumab in patients with psoriatic arthritis (PsA) and identify predictors of good clinical response for joint and skin lesions.Methods:Patients received adalimumab 40 mg every other week in addition to standard therapy in this prospective, 12-week, open-label, uncontrolled study. Four definitions of good clinical response were used: ⩾50% improvement in American College of Rheumatology response criteria (ACR50), good response according to European League Against Rheumatism (EULAR) guidelines, a ⩾3-grade improvement in Physician Global Assessment of psoriasis (PGA) and a ⩾50% improvement in the Nail Psoriasis Severity Index (NAPSI). Response predictors were determined by logistic regression with backward elimination (selection level was 5%).Results:Of 442 patients, 94% completed 12 weeks of treatment. At week 12, 74%, 51% and 32% of the patients had achieved ACR20, 50 and 70, respectively; 87% and 61% experienced moderate and good responses according to EULAR criteria, respectively. The percentage of patients with PGA results of “clear/almost clear” increased from 34% (baseline) to 68%. The mean NAPSI score was reduced by 44%. No new safety signals were detected. A lower Health Assessment Questionnaire Disability Index (HAQ-DI) score, greater pain assessment, male sex and absence of systemic glucocorticoid therapy were strongly associated with achievement of ACR50 and good response according to EULAR criteria. In addition, greater C-reactive protein concentration and polyarthritis predicted ACR50, and non-involvement of large joints predicted a good response according to EULAR criteria.Conclusions:Adalimumab was effective in patients with PsA. Lower impairment of physical function, greater pain, male sex and no systemic treatment with glucocorticoids were factors that increased the chance of achieving a good clinical response.


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