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JOURNAL ARTICLE
Monica S Thakar, Brent R Logan, Jennifer M Puck, Elizabeth A Dunn, Rebecca H Buckley, Morton J Cowan, Richard J O'Reilly, Neena Kapoor, Lisa Forbes Satter, Sung-Yun Pai, Jennifer Heimall, Sharat Chandra, Christen L Ebens, Deepak Chellapandian, Olatundun Williams, Lauri M Burroughs, Blachy Davila Saldana, Ahmad Rayes, Lisa M Madden, Shanmuganathan Chandrakasan, Jeffrey J Bednarski, Kenneth B DeSantes, Geoffrey D E Cuvelier, Pierre Teira, Alfred P Gillio, Hesham Eissa, Alan P Knutsen, Frederick D Goldman, Victor M Aquino, Evan B Shereck, Theodore B Moore, Emi H Caywood, Mark T Vander Lugt, Jacob Rozmus, Larisa Broglie, Lolie C Yu, Ami J Shah, Jeffrey R Andolina, Xuerong Liu, Roberta E Parrott, Jasmeen Dara, Susan Prockop, Caridad A Martinez, Malika Kapadia, Soma C Jyonouchi, Kathleen E Sullivan, Jack J Bleesing, Sonali Chaudhury, Aleksandra Petrovic, Michael D Keller, Troy C Quigg, Suhag Parikh, Shalini Shenoy, Christine Seroogy, Tamar Rubin, Hélène Decaluwe, John M Routes, Troy R Torgerson, Jennifer W Leiding, Michael A Pulsipher, Donald B Kohn, Linda M Griffith, Elie Haddad, Christopher C Dvorak, Luigi D Notarangelo
BACKGROUND: Severe combined immunodeficiency (SCID) is fatal unless durable adaptive immunity is established, most commonly through allogeneic haematopoietic cell transplantation (HCT). The Primary Immune Deficiency Treatment Consortium (PIDTC) explored factors affecting the survival of individuals with SCID over almost four decades, focusing on the effects of population-based newborn screening for SCID that was initiated in 2008 and expanded during 2010-18. METHODS: We analysed transplantation-related data from children with SCID treated at 34 PIDTC sites in the USA and Canada, using the calendar time intervals 1982-89, 1990-99, 2000-09, and 2010-18...
June 20, 2023: Lancet
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JOURNAL ARTICLE
Lea Grümme, Hendrik Schulze-Koops
BACKGROUND: Common variable Immunodeficiency (CVID) is a primary immunodeficiency disorder and the most common form of severe antibody deficiency. Both children and adults are affected and clinical manifestations vary widely. Often, CVID manifests with infections, autoimmune phenomena or chronic lung disease, but it also frequently affects the liver. The differential diagnoses of hepatopathies in CVID patients are diverse and the characteristics of CVID patients often make it difficult to determine the correct diagnosis...
June 5, 2023: Allergy, Asthma, and Clinical Immunology
#23
JOURNAL ARTICLE
Naila Bashir, Nighat Haider, Ana Farooq, Mulazim Hussain
OBJECTIVE: To view the different patterns of presentation of HIV in pediatric population along with mode of transmission and associated co infections and co morbidities. METHODS: It was a retrospective study conducted at Pakistan Institute of Medical Sciences, Islamabad, in which we evaluated the records of pediatric patients diagnosed with HIV from 2005 to 2020. All the data like age, gender, area, presenting complaints, examination findings at the time of diagnosis, mode of transmission, co infection and co morbidities were recorded...
2023: Pakistan Journal of Medical Sciences Quarterly
#24
REVIEW
Paolo Maria Matricardi
Opposite to other immunoglobulin (Ig) classes and subclasses, there is no consensus on the definition of normal levels of serum total IgE. However, longitudinal studies on birth cohorts produced growth charts of total IgE levels in helminth-free and never atopic children and defining the normal ranges of total serum IgE concentration at the individual, rather than population, level. Accordingly, very 'low IgE producers' (i.e., children whose tIgE level belong to the lowest percentiles) became atopic while keeping their total IgE levels in a range considered 'normal' if compared to the general age-matched population but 'abnormally high' if projected on the tIgE growth chart against the trajectory of that child's own percentile levels...
May 6, 2023: Biomedicines
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REVIEW
Cristina Ardura-Garcia, Katharina Kainz, Maria Christina Mallet, Laura Petrarca, Jasna Rodman Berlot, Monique Slaats, Carmen Streibel, Susanne Vijverberg, Emma E Williams, Myrofora Goutaki, Diane M Gray, Anna Lavizzari, Rory E Morty, Marijke Proesmans, Dirk Schramm, Mirjam Stahl, Angela Zacharasiewicz, Alexander Moeller, Mariëlle W Pijnenburg
This review has been prepared by the Early Career Members and Chairs of the European Respiratory Society (ERS) Assembly 7: Paediatrics. We here summarise the highlights of the advances in paediatric respiratory research presented at the ERS International Congress 2022. The eight scientific groups of this Assembly cover a wide range of research areas, including respiratory physiology and sleep, asthma and allergy, cystic fibrosis (CF), respiratory infection and immunology, neonatology and intensive care, respiratory epidemiology, bronchology, and lung and airway developmental biology...
May 2023: ERJ Open Research
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JOURNAL ARTICLE
Paul Edgar Gray, Clementine David
Autoimmunity may be a manifestation of inborn errors of immunity (IEI) specifically as part of the subgroup of primary immunodeficiency (PID) known as primary immune regulatory disorders (PIRD). However, while making a single gene diagnosis can have important implications for prognosis and management, picking patients to screen can be difficult, against a background of a high prevalence of autoimmune disease in the population. This review compares the genetics of common polygenic and rare monogenic autoimmunity, and explores the molecular mechanisms, phenotypes and inheritance of autoimmunity associated with PIRD, highlighting the emerging importance of gain-of-function and non-germline somatic mutations...
April 27, 2023: Journal of Allergy and Clinical Immunology in Practice
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JOURNAL ARTICLE
Maemo Lesiapeto, Justine Shults, Mogakolodi Mmunyane, Mogomotsi Matshaba, Elizabeth D Lowenthal
BACKGROUND: HIV increases risk of atherosclerosis and cardiovascular diseases (CVD). This risk maybe even higher in adult survivors of perinatal HIV infection due to prolonged exposure to HIV and its treatments. Nutritional deprivation in early life may further increase CVD risk. SETTING: Botswana-Baylor Children's Clinical Centre of Excellence, Gaborone. METHODS: This study examined dyslipidemia in 18-24 year-olds with perinatally-acquired HIV with and without linear growth retardation ("stunting")...
April 17, 2023: Journal of Acquired Immune Deficiency Syndromes: JAIDS
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JOURNAL ARTICLE
Ditte Scofield, Nina Weis, Mikael Andersson, Merete Storgaard, Gitte Pedersen, Isik S Johansen, Terese L Katzenstein, Christian Graugaard, Morten Frisch, Ellen Moseholm
OBJECTIVES: To investigate psychosocial, sexual, reproductive and menopausal health in women with HIV (WWH) compared to women without HIV (WWOH) in Denmark. DESIGN: A nationwide cross-sectional study. METHODS: Data was retrieved from the SHARE study, a Danish nationwide cross-sectional survey examining psychosocial, sexual and reproductive health in people with HIV. Data from WWH, collected in 2021-2022, was matched 1:10 on age to a comparison group of WWOH from the nationally representative cohort study Project SEXUS...
March 29, 2023: AIDS
#29
JOURNAL ARTICLE
Ayelet Ollech, Amos J Simon, Atar Lev, Tali Stauber, Gilad Sherman, Michal Solomon, Aviv Barzilai, Raz Somech, Shoshana Greenberger
BACKGROUND: Patients with primary immunodeficiency disorders (PIDs) often suffer from recurrent infections because of their inappropriate immune response to both common and less common pathogens. These patients may present with unique and severe cutaneous infectious manifestations that are not common in healthy individuals and may be more challenging to diagnose and treat. OBJECTIVE: To describe a cohort of patients with PIDs with atypical presentations of skin infections, who posed a diagnostic and/or therapeutic challenge...
2023: Frontiers in Pediatrics
#30
REVIEW
Andrew Long, Anatole Kleiner, R John Looney
The understanding of immune dysregulation in many different diseases continues to grow. There is increasing evidence that altered microbiome and gut barrier dysfunction contribute to systemic inflammation in patients with primary immunodeficiency and in patients with rheumatic disease. Recent research provides insight into the process of induction and maturation of pathogenic age-associated B cells and highlights the role of age-associated B cells in creating tissue inflammation. T follicular regulatory cells are shown to help maintain B-cell tolerance, and therapeutic approaches to increase or promote T follicular regulatory cells may help prevent or decrease immune dysregulation...
January 2023: Journal of Allergy and Clinical Immunology
#31
Joana Lopes, Maurício Peixoto, Eulália Antunes, Isabel Silva, Sofia Caridade
Common variable immunodeficiency (CVID) is a disease characterized by severe antibody deficiency due to impaired B cell differentiation. It represents the most common form of primary immunodeficiency in children and adults, and its clinical manifestations include recurrent infections and chronic lung disease, gastrointestinal infections, and autoimmunity. Here, we present the case of a 47-year-old female patient with a history of CVID and recurrent Campylobacter jejuni bacteremia. She was undergoing biweekly administration of intravenous immunoglobulin for over 15 years...
November 2022: Curēus
#32
JOURNAL ARTICLE
Endalk Birrie Wondifraw, Ermias Sisay Chanie, FishaAlebel Gebreeyesus, Gebeyaw Biset, Birhanu Desu Tefera, Mulusew Zeleke
INTRODUCTION: Around the world, tuberculosis (TB) is the most common cause of mortality and morbidity in both adults and children. The incidence of tuberculosis (TB) is increased worldwide by co-infection with the human immunodeficiency virus (HIV), particularly in Sub-Saharan Africa. As a result, the study aimed to determine the incidence and predictors of tuberculosis among children on antiretroviral therapy at northeast Ethiopia Comprehensive Specialized Hospitals. METHODS: An institution-based retrospective follow-up study was carried out in northeast Ethiopia's Comprehensive Specialized Hospitals, among 362 children on antiretroviral therapy from January 1, 2007, to September 30, 2021...
December 2022: Heliyon
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JOURNAL ARTICLE
Marcus Matheus Quadros Santos, Bianca Alessandra Gomes do Carmo, Taymara Barbosa Rodrigues, Bruna Rafaela Leite Dias, Cleyton Abreu Martins, Glenda Roberta Oliveira Naiff Ferreira, Andressa Tavares Parente, Cíntia Yollete Urbano Pauxis Aben-Atha, Sandra Helena Isse Polaro, Eliã Pinheiro Botelho
The mother-to-child transmission (MTCT) of the human immunodeficiency virus (HIV) remains a serious public health problem in the Brazilian Rainforest. This study aimed to spatially analyze this type of infection between 2007 and 2018 in Pará, which is the second-largest Brazilian state in the Brazilian Rainforest and also has the highest MTCT of HIV in Brazil. We analyzed the incidence rates of HIV (including the acquired immunodeficiency syndrome (AIDS) by MTCT as the main route of infection in children younger than 13 years old and whose mothers live in Pará...
November 29, 2022: Geospatial Health
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Krista Crider, Jennifer Williams, Yan Ping Qi, Julie Gutman, Lorraine Yeung, Cara Mai, Julia Finkelstain, Saurabh Mehta, Clara Pons-Duran, Clara Menéndez, Cinta Moraleda, Lisa Rogers, Kelicia Daniels, Patricia Green
BACKGROUND: Description of the condition Malaria, an infectious disease transmitted by the bite of female mosquitoes from several Anopheles species, occurs in 87 countries with ongoing transmission (WHO 2020). The World Health Organization (WHO) estimated that, in 2019, approximately 229 million cases of malaria occurred worldwide, with 94% occurring in the WHO's African region (WHO 2020). Of these malaria cases, an estimated 409,000 deaths occurred globally, with 67% occurring in children under five years of age (WHO 2020)...
February 1, 2022: Cochrane Database of Systematic Reviews
#35
JOURNAL ARTICLE
Sylwia Kołtan, Marcin Ziętkiewicz, Elżbieta Grześk, Rafał Becht, Elżbieta Berdej-Szczot, Magdalena Cienkusz, Marlena Ewertowska, Edyta Heropolitańska-Pliszka, Natalia Krysiak, Aleksandra Lewandowicz-Uszyńska, Monika Mach-Tomalska, Aleksandra Matyja-Bednarczyk, Marcin Milchert, Katarzyna Napiórkowska-Baran, Karolina Pieniawska-Śmiech, Anna Pituch-Noworolska, Joanna Renke, Jacek Roliński, Iwona Rywczak, Agnieszka Stelmach-Gołdyś, Magdalena Strach, Hanna Suchanek, Joanna Sulicka-Grodzicka, Aleksandra Szczawińska-Popłonyk, Sławomir Tokarski, Ewa Więsik-Szewczyk, Beata Wolska-Kuśnierz, Krzysztof Zeman, Małgorzata Pac
At the beginning of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic, patients with inborn errors of immunity (IEI) appeared to be particularly vulnerable to a severe course of the disease. It quickly turned out that only some IEI groups are associated with a high risk of severe infection. However, data on the course of Coronavirus Disease 2019 (COVID-19) in patients with IEI are still insufficient, especially in children; hence, further analyses are required. The retrospective study included 155 unvaccinated people with IEI: 105 children and 50 adults (67...
2022: Frontiers in Immunology
#36
JOURNAL ARTICLE
Devesh Malik, Dennis W Simon, Kavita Thakkar, Deepa S Rajan, Kate F Kernan
Pediatric encephalitis has significant morbidity and mortality, yet 50% of cases are unexplained. Host genetics plays a role in encephalitis' development; however, the contributing variants are poorly understood. One child with anti-NMDA receptor encephalitis and ten with unexplained encephalitis underwent whole genome sequencing to identify rare candidate variants in genes known to cause monogenic immunologic and neurologic disorders, and polymorphisms associated with increased disease risk. Using the professional Human Genetic Mutation Database (Qiagen), we divided the candidate variants into three categories: monogenic deleterious or potentially deleterious variants (1) in a disease-consistent inheritance pattern; (2) in carrier states; and (3) disease-related polymorphisms...
November 2022: Genes and Immunity
#37
JOURNAL ARTICLE
Cristina Tomacinschii, Rodica Selvestru, Lilia Sinitina, Virgil Petrovici, Rodica Golban, Valentin Turea, Svetlana Sciuca
CONTEXT: Inherited errors of immunity (IEI) or primary immunodeficiencies are a group of genetic rare diseases predisposing to severe infections, autoimmunity, and malignancies. The overall risk for malignancies in pediatric IEI-patients is estimated to range from 4-25%, of which 60% of cases are of hematological origin. OBJECTIVE: Evaluation of hematological malignancies in pediatrics IEI. METHODS: Were included 13 patients 3 of which developed hematological malignancies and were evaluated for demographics, clinical features, and prognosis...
October 2022: Clinical Lymphoma, Myeloma & Leukemia
#38
JOURNAL ARTICLE
Thales Silva Antunes, Karina Mescouto Melo, Cláudia França Cavalcante Valente, Fabíola Scancetti Tavares
INTRODUCTION: Immunoglobulin represents the main therapy for patients with inborn errors of immunity (IEI) and it is a safe procedure, but adverse events (AEs) can occur with variable frequencies. OBJECTIVE: To evaluate the frequency of immediate AEs to intravenous immunoglobulin (IVIG) regular therapy in a pediatric cohort with IEI after a pre-IVIG infusion protocol. METHODS: This was a longitudinal study from 2011 to 2019 at a tertiary pediatric hospital in Brazil...
August 8, 2022: Hematology, Transfusion and Cell Therapy
#39
JOURNAL ARTICLE
Mariana Barros Marcondes, Cíntia Mitsue Pereira Susuki, Newton Key Hokama, Paula de Oliveira Montandon Hokama, Felipe Aguera Oliver, Paulo Sergio Chaib, Xingshun Qi, Fernando Gomes Romeiro
BACKGROUND: Common variable immunodeficiency (CVID) is a rare disease that affects children and adults and is often difficult to diagnose. Despite being one of the most frequent causes of immunodeficiency, involving gastrointestinal (GI), respiratory, and hematological systems, the disease onset can have heterogeneous and intermittent symptoms, frequently leading to diagnostic delay. GI symptoms are common and can include diarrhea, but the asymptomatic periods lead to overlooking the recurrent pattern...
September 4, 2022: BMC Gastroenterology
#40
JOURNAL ARTICLE
Ankur Kumar Jindal, Himanshi Chaudhary, Rahul Tyagi, Amit Rawat, Deepti Suri, Pratap Kumar Patra, Kanika Arora, Sanchi Chawla, Sameer Vyas, Munish Arora, Ridhima Aggarwal, Suprit Basu, Reema Bansal, Man Updesh Singh Sachdeva, Anju Gupta, Vignesh Pandiarajan, Naveen Sankhyan, Renu Suthar, Jitendra Kumar Sahu, Mini Singh, Reeta Mani, Rajni Sharma, Ruchi Saka, Kohsuke Imai, Osamu Ohara, Shigeaki Nonoyama, Lennart Hammarström, Koon Wing Chan, Yu Lung Lau, Surjit Singh
BACKGROUND/OBJECTIVES: Patients with primary antibody deficiency (PAD) are predisposed to develop meningoencephalitis, often considered to be enteroviral. However, there is a paucity of literature on this subject, and there are no studies from developing countries. METHODS: We analyzed our cohort of children with PAD who developed meningoencephalitis. RESULTS: This complication was observed in 13/135 (10.4%) patients with PAD - 5 patients had X-linked agammaglobulinemia (XLA), 7 had common variable immunodeficiency (CVID) and 1 had suspected nuclear factor kappa B essential modulator (NEMO) defect...
October 15, 2022: Journal of Neuroimmunology
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