scholarly journals Case Report: Reactive Lymphohistiocytic Proliferation in Infant With a Novel Nonsense Variant of IL2RG Who Received BCG Vaccine

2021 ◽  
Vol 9 ◽  
Author(s):  
Amal M. Yahya ◽  
Suleiman Al-Hammadi ◽  
Nidal O. AlHashaykeh ◽  
Salwa S. Alkaabi ◽  
Abdulghani S. Elomami ◽  
...  

We present here a male young infant with X-linked severe combined immunodeficiency (MIM#300400) due to the novel nonsense variant of IL2RG (interleukin 2 receptor, gamma; MIM#308380), NM_000206.2(IL2RG):c.820_823dup p.Ser275Asnfs*29. He developed aggressive reactive lymphohistiocytic proliferation after receiving the live-attenuated Bacillus Calmette-Guérin (BCG) vaccine at birth. This report advocates for modifying the current practice of early use of BCG. The natural history of his disease also suggests considering IL2RG variants as a potential cause of “X-linked recessive Mendelian susceptibility to mycobacterial disease” (MSMD). His reactive lymphohistiocytic proliferation and massive hepatosplenomegaly simulated hemophagocytic lymphohistiocytosis (HLH, likely triggered by the BCG disease). This entity was masked by the absence of fever and markedly elevated inflammatory biomarkers. Thus, his findings stimulate discussion on the need to modify the diagnostic criteria of HLH, in order to accommodate conditions, such IL2RG variants that block systemic inflammation.

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Sílvia Bacalhau ◽  
Cristina Freitas ◽  
Rosalina Valente ◽  
Deolinda Barata ◽  
Conceição Neves ◽  
...  

In high-burden countries,Mycobacterium bovisBacillus Calmette-Guérin (BCG) vaccine is administered in newborn to prevent severeMycobacterium tuberculosisinfection. Because life-threatening disseminated BCG disease may occur in children with primary immunodeficiency, vaccination strategy against tuberculosis should be redefined in non-high-burden countries. We report the case of a patient with X-linked severe combined immunodeficiency (SCID) who developed disseminated BCG disease, highlighting the specific strategies adopted.


F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1399
Author(s):  
Domingos Sousa ◽  
Sérgio Antunes Silva ◽  
Catarina Jorge ◽  
Ana Isabel Rodrigues ◽  
Rita Martins Fernandes ◽  
...  

The Bacillus Calmette Guérin (BCG) vaccine was initially produced as a vaccine against tuberculosis. BCG is an attenuated live strain of Mycobacterium bovis and has been widely used as an immunotherapy over the last years in bladder cancer. We describe a case of a 61-year-old Caucasian male with previous bladder cancer, who had been treated for the last 15 months with instillation of BCG, admitted with 3-week evolution history of fever (38ºC), asthenia, anorexia and a weight loss of 6kg. The patient’s condition deteriorated leading to sepsis. A liver biopsy was performed showing granulomatous dispersed through all the parenchyma. Empirical therapy for M. bovis was started with good response. Even though it is rare, BCGitis must be ruled out in any patient submitted to immunotherapy with BCG and prompt therapy started if disseminated disease is present, which improves the outcome.


2021 ◽  
Vol 41 (1) ◽  
pp. 24-30
Author(s):  
Badria Al Waili ◽  
Nasra Al Mufarajii ◽  
Samiyah Al Hashmi ◽  
Abdullah Al Ajmi ◽  
Nashat Al Sukaiti

BACKGROUND: Bacillus Calmette-Guérin (BCG) vaccine-related complications are frequently observed in children in Oman. There are a few regional studies on BCG complications, but none from Oman. OBJECTIVE: Evaluate the spectrum of BCG-vaccine related complications and immune status in Omani children. DESIGN: Retrospective cross-sectional study. SETTING: Referral tertiary hospital. METHODS: Children aged younger than 13 years old and with complications of BCG vaccination recorded from 2006-2018 were included in this study. Clinical characteristics, treatment, immune workup and outcome were reviewed from hospital records. MAIN OUTCOME MEASURES: Different BCG vaccine-related complications categorized by the site of involvement. SAMPLE SIZE: 226. RESULTS: Of the 226 children had BCG-vaccine related complications, 99% received BCG vaccine immediately after birth. The median age of presentation was 4 months. The most common complication was isolated BCG lymphadenitis (85%, n=192), followed by BCG-related osteomyelitis (10.2%, n=23) and disseminated BCG infection (4.9%, n=11). The median age of presentation of disseminated BCG was 5 months, with different organs involved. Out of 11 children with disseminated BCG infection, 72.7% (n=8) had primary immune deficiency (PID), including chronic granulomatous disease (CGD, n=5), severe combined immunodeficiency (SCID) (n=2); 1 patient had Mendelian susceptibility to mycobacterial disease (IFNGR2 deficiency); 2 patients with PID not yet identified and the 1 with a non-specific PID had blood or saliva samples sent for whole-exome sequencing. CONCLUSION: Because of the spectrum of BCG vaccine-related complications, including the most severe in children with PID, we suggest that delaying the BCG vaccine from birth to 6 months may prevent disseminated BCG diseases and their complications in children with PID because any PID will have been identified before 6 months. Further studies are needed to guide this recommendation. LIMITATIONS: Single center-based study that may not provide a full overview of all BCG vaccine-related complications in Oman. Unavailability of details of some microbiological results and an inability to determine the detailed management for all patients. CONFLICT OF INTEREST: None.


Author(s):  
Janine Hensel ◽  
Kathleen M. McAndrews ◽  
Daniel J. McGrail ◽  
Dara P. Dowlatshahi ◽  
Valerie S. LeBleu ◽  
...  

ABSTRACTThe Bacillus Calmette-Guerin (BCG) vaccine provides protection against tuberculosis (TB), and is proposed to provide protection to non-TB infectious diseases. The COVID-19 outbreak results from infection with the novel coronavirus SARS-CoV-2 (CoV-2) and was declared a pandemic on March 11th, 2020. We queried whether the BCG vaccine offers protection against CoV-2 infection. We observed that countries with a current universal BCG vaccination policy have a significantly lower COVID-19 incidence than countries which never had a universal BCG policy or had one in the past. However, population density, median age, TB incidence, urban population, and, most significantly, CoV-2 testing rate, were also connected with BCG policy and could potentially confound the analysis. By limiting the analysis to countries with high CoV-2 testing rates, defined as greater than 2,500 tests per million inhabitants, these parameters were no longer statistically associated with BCG policy. When analyzing only countries with high testing rates, there was no longer a significant association between the number of COVID-19 cases per million inhabitants and the BCG vaccination policy. Although preliminary, our analyses indicate that the BCG vaccination may not offer protection against CoV-2 infection. While reporting biases may confound our observations, our findings support exercising caution in determining potential correlation between BCG vaccination and COVID-19 incidence, in part due significantly lower rates of CoV-2 testing per million inhabitants in countries with current universal BCG vaccination policy.


2019 ◽  
Vol 74 (3) ◽  
pp. 305-331
Author(s):  
John Owen Havard

John Owen Havard, “‘What Freedom?’: Frankenstein, Anti-Occidentalism, and English Liberty” (pp. 305–331) “If he were vanquished,” Victor Frankenstein states of his monstrous creation in Mary Shelley’s Frankenstein (1818), “I should be a free man.” But he goes on: “Alas! what freedom? such as the peasant enjoys when his family have been massacred before his eyes, his cottage burnt, his lands laid waste, and he is turned adrift, homeless, pennyless, and alone, but free.” Victor’s circumstances approximate the deracinated subject of an emergent economic liberalism, while looking to other destitute and shipwrecked heroes. Yet the ironic “freedom” described here carries an added charge, which Victor underscores when he concludes this account of his ravaged condition: “Such would be my liberty.” This essay revisits the geographic plotting of Frankenstein: the digression to the East in the nested “harem” episode, the voyage to England, the neglected episode of Victor’s imprisonment in Ireland, and the creature’s desire to live in South America. Locating Victor’s concluding appeal to his “free” condition within the novel’s expansive geography amplifies the political stakes of his downfall, calling attention to not only his own suffering but the wider trail of destruction left in his wake. Where existing critical accounts have emphasized the French Revolution and its violent aftermath, this obscures the novel’s pointed critique of a deep and tangled history of English liberty and its destructive legacies. Reexamining the novel’s geography in tandem with its use of form similarly allows us to rethink the overarching narrative design of Frankenstein, in ways that disrupt, if not more radically dislocate, existing rigid ways of thinking about the novel.


2020 ◽  
Author(s):  
Rawand Essa

BACKGROUND In the span of COVID-19, the mortality rate has been different from one country to another. As a country in the Middle East Iraq has a critical position, lies between Iran and Turkey while both countries coronavirus cases increase on daily basis, while Iranian mortality rate record is high similar to Turkey. After Wuhan city of China, Lombard of Italy, Qum city in Iran has the highest number of COVID-19 as a first country in the Middle East. OBJECTIVE aim of this study is to show the effect of BCG vaccine during pandemic diseases, especially nowadays at the time of COVID-19. One of the crucial observations is the government preparedness and strategic planning prior pandemics, in which the BCG vaccine is an attenuated live vaccine for control of tuberculosis (TB). BCG vaccine has a non-specific immune effect that is used against pathogens like bacteria and viruses, through the promotion of pro-inflammatory cytokines' secretion. METHODS An epidemiological study has been performed, and it shows that some countries are more prone to contagious diseases like COVID-19, regardless of the main cultural, religious, societal similarities among the three mentioned countries. The information data has been collected from WHO reports and worldometer in 18 February 2020 to 10 May 2020. Regarding the efficacy of the BCG vaccine, relevant data has been retrieved from Google scholar, Pub-med and BCG world-atlas. RESULTS COVID-19 mortality rates are at peak in Iran and Turkey while the mortality rate is very low in Iraq, while the patients that died in Iraq all had history of other long-term diseases as heart disease, blood pressure, cancer etc. CONCLUSIONS From the experiences of the three countries in the life span of COVID-19, the historical plan of BCG vaccine in Iraq in cooperation with WHO since the last decades it shows that COVID-19 mortality rate is lower than other countries due to the early vaccination of the Iraqis, otherwise Iraq is more fragile than Iran and Turkey due to the poor conditions of Iraq in terms of economics, politics, war and other aspects.


2020 ◽  
Author(s):  
Camila Massae Sato ◽  
Omar Ariel Espinosa Domínguez ◽  
Gutembergue Santos de Sousa ◽  
Fabiane Verônica da Silva ◽  
Roberta Olmo Pinheiro ◽  
...  

BACKGROUND Leprosy contacts are sensitive indicator of the magnitude of severity, and a high risk for infection and disease, especially if the individuals are exposed to the bacillus with multibacillary cases, which reinforces the need for chemoprophylactic measures, such as the use single-dose rifampicin (SDR) combined with BCG vaccine. OBJECTIVE Our study will analyze the serological response of anti-NDO-LID, HO-1/HMOX and serum IFN-γ concentration as a prophylactic measure after the use of single-dose rifampicin (SDR) combined with the Bacillus Calmette-Guérin vaccine (BCG) among multibacillary leprosy household contacts. METHODS Intervention study based on a prospective cohort of household contacts of new cases of multibacillary leprosy diagnosed and registered in the Information System on Diseases of Compulsory Declaration (SINAN – Sistema de Informação de Agravos de Notificação) and residents in the municipalities of Cuiabá and Cáceres, Mato Grosso, in the years 2019 and 2020. Contacts with no signs and symptoms compatible with the disease of the intervention group will receive single-dose rifampicin (SDR) combined with BCG vaccine and will be compared to the control group. The follow-up of the cohort will last 12 months. The analyses of the anti-NDO-LID, HO-1/HMOX and serum concentration of IFN-γ tests as immunological markers before, 6 and 12 months after the intervention will be conducted in a reference laboratory (FIOCRUZ). RESULTS The study is still recruiting participants and is expected to be completed in September 2020. CONCLUSIONS Our study seeks to analyze the response of anti-NDO-LID, HO-1/HMOX tests and serum IFN-γ concentration as a prophylactic measure after the use of single-dose rifampicin (SDR) combined with the Bacillus Calmette-Guérin vaccine (BCG) in home contacts of multibacillary leprosy cases. CLINICALTRIAL Brazilian Registry of Clinical Trials (ReBEC), RBR-6yg2z9. Retrospectively registered on 3 de Out. de 2019.


2002 ◽  
Author(s):  
R Phillips ◽  
H Ejere ◽  
C Preston ◽  
Asiamah ◽  
E

Author(s):  
Lyndsey Stonebridge

Hannah Arendt’s analysis of the failure of human rights to address statelessness is well known. Less commented upon is how important literature was to her thought. This chapter shows how Arendt’s 1940s essays on Kafka connect the history of the novel to shifting definitions of legal and political sovereignty. Arendt reads The Castle as a blueprint for a political theory that is also a theory of fiction: in the novel K, the unwanted stranger, demolishes the fiction of the rights of man, and with it, the fantasy of assimilation. In a parallel move, Kafka also refuses to assimilate his character into the conventions of fiction. Arendt’s reading changes the terms for how we might approach the literature of exile and of human rights.


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