Клинический случай поздней диагностики агаммаглобулинемии у ребенка дошкольного возраста

Background. Agammaglobulinemia is a disease from the group of primary immune deficiencies with impaired antibodies production characterized by significant decrease or complete absence of B-cells. Due to the absence of pathogenetic treatment, severe bacterial lesions of various organs and tissues can develop, and they can lead to the patient death. Timely history taking and clinical and laboratory data analysis will allow to reveal this pathology early and start the therapy that can save patient's life. Clinical case description. This article demonstrates clinical case of agammaglobulinemia late diagnosis with development of severe complications and reversal of clinical manifestations in response to pathogenetic therapy. Conclusion. Despite the disease onset in the second half-year period of life, the presence of recurrent purulent processes resistant to standard treatment and the significant decrease of all immunoglobulin levels, agammaglobulinemia was suspected only at preschool age. The outcomes were disabled patient, and considerable aggravation of the quality of life.Обоснование. Агаммаглобулинемия — это заболевание, относящееся к группе первичных иммунодефицитов с нарушением продукции антител, характеризующееся существенным снижением или практически полным отсутствием В-клеток. При отсутствии патогенетического лечения формируются тяжелые бактериальные поражения различных органов и тканей, что может послужить причиной летального исхода у пациента. Своевременный учет анамнеза, клинико-лабораторных данных позволит вовремя заподозрить данную патологию и начать терапию, что спасет жизнь больному. Описание клинического случая. В статье приводится клинический случай поздней диагностики агаммаглобулинемии с формированием тяжелых осложнений и купированием клинических проявлений на фоне патогенетической терапии. Заключение. Несмотря на дебют заболевания во втором полугодии жизни, наличие повторяющихся гнойных процессов, не поддающихся стандартной терапии, в сочетании с резким снижением уровней иммуноглобулинов всех классов, агаммаглобулинемия была заподозрена только к дошкольному возрасту. Исходом стали инвалидизация пациента, значительное ухудшение качества жизни

Chronic coronary syndromes without standard modifiable cardiovascular risk factors and outcomes: the CLARIFY registry

Background and Aims: It has been reported that patients without standard modifiable cardiovascular (CV) risk factors (SMuRFs—diabetes, dyslipidaemia, hypertension, and smoking) presenting with first myocardial infarction (MI), especially women, have a higher in-hospital mortality than patients with risk factors, and possibly a lower long-term risk provided they survive the post-infarct period. This study aims to explore the long-term outcomes of SMuRF-less patients with stable coronary artery disease (CAD). Methods: CLARIFY is an observational cohort of 32 703 outpatients with stable CAD enrolled between 2009 and 2010 in 45 countries. The baseline characteristics and clinical outcomes of patients with and without SMuRFs were compared. The primary outcome was a composite of 5-year CV death or non-fatal MI. Secondary outcomes were 5-year all-cause mortality and major adverse cardiovascular events (MACE—CV death, non-fatal MI, or non-fatal stroke). Results: Among 22 132 patients with complete risk factor and outcome information, 977 (4.4%) were SMuRF-less. Age, sex, and time since CAD diagnosis were similar across groups. SMuRF-less patients had a lower 5-year rate of CV death or non-fatal MI (5.43% [95% CI 4.08–7.19] vs. 7.68% [95% CI 7.30–8.08], P = 0.012), all-cause mortality, and MACE. Similar results were found after adjustments. Clinical event rates increased steadily with the number of SMuRFs. The benefit of SMuRF-less status was particularly pronounced in women. Conclusions: SMuRF-less patients with stable CAD have a substantial but significantly lower 5-year rate of CV death or non-fatal MI than patients with risk factors. The risk of CV outcomes increases steadily with the number of risk factors