Severe combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are the only curative treatments available, and the best-known prognostic factors for success are age at diagnosis, age at hematopoietic stem cell transplantation, and the comorbidities that develop in between. There are no evidence-based guidelines for standardized clinical care for patients with SCID during the time between diagnosis and definitive treatment, and we aim to generate a consensus management strategy on the supportive care of patients with SCID. First, we gathered available information about SCID diagnostic and therapeutic guidelines, then we developed a document including diagnostic and therapeutic interventions, and finally we submitted the interventions for expert consensus through a modified Delphi technique. Interventions are grouped in 10 topic domains, including 123 ''agreed'' and 38 ''nonagreed'' statements. This document intends to standardize supportive clinical care of patients with SCID from diagnosis to definitive treatment, reduce disease burden, and ultimately improve prognosis, particularly in countries where newborn screening for SCID is not universally available and delayed diagnosis is the rule. Our work intends to provide a tool not only for immunologists but also for primary care physicians and other specialists involved in the care of patients with SCID. (J Allergy Clin Immunol 2019;144:897-905.)
Cardona JA et al. Ocurrencia de miasis cavitaria equina (Gasterophilus Spp) y su relación con las úlceras gástricas ¤ Para citar este artículo: Cardona JÁ, Álvarez A, Paredes E. Ocurrencia de miasis cavitaria equina (Gasterophilus Spp) y su relación con las úlceras gástricas secundarias en la mucosa escamosa en AbstractThe aim of this study was to determine the ocurrence of equine cavitary myiasis (Gasterophilus spp) in the CholChol slaughtering house in Temuco, Chile and its relation with the presentation of secondary gastric ulcers in the non-glandular squamous mucosa of the horses' (Equus caballus) stomachs. Of 240 stomachs evaluated post mortem, 145 were selected and characterized as positive to gasterophilosis (60.4%), of which 75 were males (20 stallions and 55 geldings) and 70 were mares, with ages ranging from 1 to 16 years. Every stomach was evaluated, characterized and photographed post mortem. Information about sex, age, location of the Gasterophilus larvae, presence and degree of ulceration of the squamous mucosa, were recorded in the respective forms and posteriorly digitalized. Global analysis of the location of larvae in the stomach of horses positive to gasterophilosis, established that 91.7% were located in the glandular mucosa, while 8.3% were located in the squamous mucosa. In the stomachs with presence of larvae in the antral/piloric regions of the glandular mucosa, 87.6% had secondary ulcers in the nonglandular squamous mucosa, due to the increase in the gastric fill line following alterations in gastric emptying, of which 69% were classified in categories 3 to 4 with a number of larvae higher tan 30, while 18.6% were found in categories 1 or 2 with less than 30 larvae per site. Regarding the location of larvae in the squamous mucosa, 8.3% of the stomachs showed primary mechanic ulcers, all classified between grades 1 and 2. Of all the animals evaluated, 62.1% had more tan 60 larvae, of which 60.1% had grade 3 or 4 secondary squamous ulcers, 15.9% had between 30 and 60 larvae per site, and 14.5% had less than 30 larvae per site. The study showed significant statistical dependence (p<0.05) between the number of larvae located in the glandular mucosa and the degree of severity of the secondary gastric ulcers in the squamous mucosa. Concluding, a high ocurrence of cavitary gastric myiasis was observed in slaughtering horses in the south of Chile, with squamous ulcers secondary to abnormal gastric emptying being the
BACKGROUND: Chronic granulomatous disease (CGD) is a primary immunode ciency with increased susceptibility to several bacteria, fungi, and mycobacteria, caused by defective or null superoxide production by the NADPH oxidase enzymatic complex. Accepted treatment consists mainly of antimicrobial prophylaxis. The role of human recombinant subcutaneous interferon gamma (IFNγ) is less clear, as available clinical evidence on its safety and e cacy is scarce and con icting. OBJECTIVE: We aimed to assess the e cacy and safety of IFNγ as an added treatment for CGD when compared to antimicrobial prophylaxis alone. METHODS: A literature search was conducted using MeSH terms "Chronic granulomatous disease" AND ("interferon gamma" OR "interferon-gamma"), as well as antibiotics, placebo, no therapy, clinical trial, trial; on MEDLINE, EMBASE, LILACS, WHOs, CENTRAL, KOREAMED, The Cochrane Library, clinicaltrials.gov, and abstracts from meetings, from 1976 to July 2022. We included clinical trials (CT) and prospective follow-up studies and registered the number of serious infections (requiring hospitalization and IV antibiotics) and deaths; adverse events, and autoimmune complications, in patients treated for CGD with antimicrobial prophylaxis plus IFN-γ, versus antimicrobial prophylaxis alone. We assessed the quality of the studies using Risk of Bias and STROBE. We performed a meta-analysis by calculating both Peto odds ratio (OR), and Risk Reduction (RR) through the Mantel-Haenzsel method with a xed effect model, using Review Manager 5.4, and we reported the number needed to treat (NNT). RESULTS: We identi ed 54 matches from databases, and 4 from other sources. We excluded 12 duplicates, 7 titles, and 9 abstracts for relevance, after which we had 30 eligible studies. Twenty-four were then excluded after reading the full text. Six papers were included: one randomized CT, and 5 follow-up studies. In total, 324 patients with Chronic granulomatous disease were followed for 319 months under treatment with antibiotic prophylaxis plus interferon-gamma or placebo (or antibiotic prophylaxis alone), reported between the years 1991 and 2016. Three of the studies included a control group, allowing for the aggregate analysis of e cacy (prevention of serious infections). The aggregate OR was 0.49, with a 95% con dence interval of 0.19 to 1.23. The Risk Ratio for serious infection was 0.56 (95%CI 0.35-0.90) under IFNG. The meta-analysis thus favors interferon-gamma for a risk reduction of serious infection. DISCUSSION: The results from this meta-analysis support the use of IFNg in the treatment of patients with CGD. However, we found insu cient clinical evidence and believe more clinical trials are needed to better assess the e cacy and long-term safety of IFNγ.
BACKGROUND: Chronic granulomatous disease (CGD) is a primary immunodeficiency with increased susceptibility to several bacteria, fungi, and mycobacteria, caused by defective or null superoxide production by the NADPH oxidase enzymatic complex. Accepted treatment consists mainly of antimicrobial prophylaxis. The role of human recombinant subcutaneous interferon gamma (IFNγ) is less clear, as available clinical evidence on its safety and efficacy is scarce and conflicting. OBJECTIVE: We aimed to assess the efficacy and safety of IFNγ as an added treatment for CGD when compared to antimicrobial prophylaxis alone. METHODS: A literature search was conducted using MeSH terms “Chronic granulomatous disease” AND (“interferon gamma” OR “interferon-gamma”), as well as antibiotics, placebo, no therapy, clinical trial, trial; on MEDLINE, EMBASE, LILACS, WHOs, CENTRAL, KOREAMED, The Cochrane Library, clinicaltrials.gov, and abstracts from meetings, from 1976 to July 2022. We included clinical trials (CT) and prospective follow-up studies and registered the number of serious infections (requiring hospitalization and IV antibiotics) and deaths; adverse events, and autoimmune complications, in patients treated for CGD with antimicrobial prophylaxis plus IFN-γ, versus antimicrobial prophylaxis alone. We assessed the quality of the studies using Risk of Bias and STROBE. We performed a meta-analysis by calculating both Peto odds ratio (OR), and Risk Reduction (RR) through the Mantel-Haenzsel method with a fixed effect model, using Review Manager 5.4, and we reported the number needed to treat (NNT). RESULTS: We identified 54 matches from databases, and 4 from other sources. We excluded 12 duplicates, 7 titles, and 9 abstracts for relevance, after which we had 30 eligible studies. Twenty-four were then excluded after reading the full text. Six papers were included: one randomized CT, and 5 follow-up studies. In total, 324 patients with Chronic granulomatous disease were followed for 319 months under treatment with antibiotic prophylaxis plus interferon-gamma or placebo (or antibiotic prophylaxis alone), reported between the years 1991 and 2016. Three of the studies included a control group, allowing for the aggregate analysis of efficacy (prevention of serious infections). The aggregate OR was 0.49, with a 95% confidence interval of 0.19 to 1.23. The Risk Ratio for serious infection was 0.56 (95%CI 0.35-0.90) under IFNG. The meta-analysis thus favors interferon-gamma for a risk reduction of serious infection. DISCUSSION: The results from this meta-analysis support the use of IFNg in the treatment of patients with CGD. However, we found insufficient clinical evidence and believe more clinical trials are needed to better assess the efficacy and long-term safety of IFNγ.
BackgroundBee venom immunotherapy is a safe and effective treatment, indicated in patients with previous history of severe systemic reactions to bee venom, demonstrating succesful desensitization in more than 90% of cases with standardized extract. Currently in Mexico there is no standardized extract commercially available for treatment, despite of having high activity of beekeeping and occupational exposure with at least 17,478 registered stings per year and an annually honey production of nearly 70 tons.MethodsWe present the clinical progress of 2 patients with history of severe systemic reactions to bee venom and occupational exposure, both with demonstrated sensitization by specific IgE and who underwent specific immunotherapy with standardized extract (Alk-US) reaching a maintenance weekly dose of 100 mcg (PLA2) for the last 4 years.ResultsBoth patients sufered of accidental stings after reached the maintenance dose presenting mild local reactions to stings. Both patients had very different clinical course presenting a wide variety of adverse reactions during desensitization protocol; from mild local to generalized reactions all generally well tolerated allowed to reach the maintenance dose with succesful desensitization proved by accidental exposure without severe systemic reactions.ConclusionsBee venom specific immunotherapy with standardized extract is a well tolerated and efective treatment preventing the development of life threathening reactions in sensitized patients. It is important to promote the use and availability of standardized extract in developing countries with poor safety measures and high occupational exposure.
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