Napiórkowska-Baran Katarzyna, Ziętkiewicz Marcin, Więsik-Szewczyk Ewa, Matyja-Bednarczyk Aleksandra, Tykwińska Marta, Alska Ewa, Rosada Tomasz, Szynkiewicz Ewa, Lubański Jakub, Schmidt Oskar, Szymczak Bartłomiej, Koperska Kinga, Bartuzi Zbigniew
Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University Torun, 85-067 Bydgoszcz, Poland.
Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdansk, 80-210 Gdansk, Poland.
J Clin Med. 2023 Sep 17;12(18):6018. doi: 10.3390/jcm12186018.
An improved recognition of inborn errors of immunity (IEI) is associated with an increase in life expectancy and a higher incidence of complications and related conditions. The aim of the study was to analyze factors enabling the primary prevention: BMI, smoking and selected laboratory tests (morphology with smear, creatinine, eGFR, total protein, albumin, ferritin, folic acid, vitamin B12, vitamin D3) included in the protocols of standard of care for adult patients with primary antibody deficiencies (PADs). The study included 94 participants ≥ 18 years old, diagnosed with PADs. Overweight was found in 17%, obesity in 14% and underweight in 15% of patients; 11.5% of patients smoked. Leukopenia was diagnosed in 16%, neutropenia in 8.5%, lymphopenia in 22.5% and thrombocytopenia in 14% of patients. A decreased concentration of hemoglobin was found in 32%, total protein in 19%, albumin in 17%, vitamin D3 in 52%, vitamin B12 in 6.5%, folic acid in 34% and ferritin in 26% of patients. Creatinine concentrations were elevated in 16% of patients, while in 20%, eGFR was reduced. Only a holistic assessment of comorbidities and complications of deficiency, as well as regular follow-up and lifestyle changes, can yield the best results in the long-term care of patients.
对先天性免疫缺陷(IEI)认识的提高与预期寿命的增加以及并发症和相关病症的更高发病率相关。本研究的目的是分析促成一级预防的因素:纳入成年原发性抗体缺陷(PAD)患者护理标准方案中的体重指数(BMI)、吸烟情况以及选定的实验室检查(涂片形态学、肌酐、估算肾小球滤过率[eGFR]、总蛋白、白蛋白、铁蛋白、叶酸、维生素B12、维生素D3)。该研究纳入了94名年龄≥18岁、被诊断为PAD的参与者。发现17%的患者超重,14%的患者肥胖,15%的患者体重过轻;11.5%的患者吸烟。16%的患者被诊断为白细胞减少症,8.5%的患者为中性粒细胞减少症,22.5%的患者为淋巴细胞减少症,14%的患者为血小板减少症。32%的患者血红蛋白浓度降低,19%的患者总蛋白降低,17%的患者白蛋白降低,52%的患者维生素D3降低,6.5%的患者维生素B12降低,34%的患者叶酸降低,26%的患者铁蛋白降低。16%的患者肌酐浓度升高,20%的患者eGFR降低。只有对缺陷的合并症和并发症进行全面评估,以及定期随访和改变生活方式,才能在患者的长期护理中取得最佳效果。