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The literature review presents data from a limited number of available studies conducted over the last two decades on immunological deficiency in congenital heart defects (CHDs), which is the cause of frequent infectious complications before and after cardiac surgery. Several studies based on screenings at various levels indicate the presence of primary and secondary immunodeficiency in CHDs, in particular about 13 genetic syndromes in which CHD is combined with immunodeficiency. The available data suggests a greater severity of immunological disorders in patients with critical CHDs, cyanotic CHDs, and conotruncal defects with T-cell dysfunction and deficiency of immunoglobulins (especially the IgG class, mainly IgG4) than in patients with shunts and obstructive defects. To identify defects in the T- and B-cell components of the immune system, quantification of the DNA of T-cell receptor excision circles (TRECs) and K-deleting recombination excision circles (KRECs)-by-products of the maturation of T- and B-cell receptors- has proven helpful in the world practice of neonatal screening. It allows the evaluation of a number of functionally mature T- and B-cells. In Russia, however, its widespread use started only in 2023. Data on the use of this assay in infants with CHDs are represented by isolated case reports. In Russia, a combination of CHD and primary immunodeficiency was found in 37% of cases in the Sverdlovsk Region. We conducted our own study of 200 children with CHD; 5% of cases were syndromic forms of CHD. 48.5% of children were admitted to the cardiac surgery clinic in critical condition. A decrease in the TREC level was detected in 23.5% of cases, including all children with syndromic CHD. In the group of patients with immunological disorders, there were significantly more children with cyanotic CHD, children admitted in critical condition, and children with conotruncal defects. Infectious complications in the postoperative period (sepsis, pneumonia, tracheobronchitis, postoperative wound infection) were observed significantly more often in 47 children with reduced TREC levels compared to children with normal TREC levels (P = .00000, in 36% and 3.6%, respectively). The analysis of publications confirms the prognostic value of TREC and KREC screening for targeted preoperative preparation to reduce postoperative complications and decrease the risk of mortality in CHDs.
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http://dx.doi.org/10.2174/0115733963325523250320065040 | DOI Listing |
Eur J Case Rep Intern Med
August 2025
Medical Subspecialities Department, Rheumatology Section, King Fahad Medical City, Riyadh, Saudi Arabia.
Unlabelled: Concurrent presentation of pulmonary nocardiosis and granulomatosis with polyangiitis (GPA) is exceptionally rare and diagnostically challenging, given the overlapping clinical and radiological features. We report a 54-year-old female with fever, cough, weight loss, and arthralgia. Chest imaging showed multiple pulmonary nodules; serology revealed positive anti-neutrophil cytoplasmic antibodies -proteinase 3, and lung biopsy demonstrated necrotizing granulomatous inflammation with Nocardia species.
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September 2025
Department of Internal Medicine, Hospital Universitario San Agustín, Asturias, Spain.
Background: Although splenomegaly is a common finding in Epstein-Barr virus (EBV) infection, splenic infarction is rarely reported and may be under-recognised, especially in adults. Neurological complications such as aseptic meningitis are also uncommon but documented. The simultaneous occurrence of both complications in the context of primary EBV infection is exceptional.
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August 2025
Department of Internal Medicine, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, USA.
Unlabelled: Autoimmune haemolytic anaemia (AIHA) is caused by antibody-mediated destruction of red blood cells. There are two broad categories of AIHA: warm and cold, both categorized by the thermal reactivity of the autoantibodies. Cold agglutinin disease (CAD) occurs at temperatures below normal body temperature and primarily involves IgM antibodies.
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July 2025
Servicio de Alergia e Inmunología, Hospital Británico de Buenos Aires, Argentina.
Introduction: Interstitial lung disease is a major complication in patients with common variable immunodeficiency. There are some publications that try to shed light on the pathophysiology of this non-infectious complication, most of them highlight the role of follicular T cells and CD21 B cells. Moreover, there are no guidelines based on randomized controlled studies on the treatment of patients with interstitial lung disease and the published case series or small uncontrolled studies describe a wide range of response rates to treatment.
View Article and Find Full Text PDFMed Trop Sante Int
July 2025
Unité des maladies infectieuses et tropicales et CIC Inserm 1424, Centre hospitalier de Cayenne, Cayenne, Guyane.
Tahiti or the "myth of Paradise", Bora Bora, "the Pearl of the Pacific". Who has never wanted to take a plane and come and land on the heavenly beaches of Polynesia, a French territory at the antipodes of mainland France lost in the middle of the Pacific? However, we do not imagine that 60% of Polynesians live below the metropolitan low-income threshold or that life expectancy is lower than that of the mainland due to the high prevalence of cardiovascular diseases with three quarters overweight population.In addition to non-transmissible metabolic diseases, various pathologies common to temperate countries present specificities in Polynesia, leading to sometimes different management and medical reasoning.
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