Pub Date : 2026-02-07DOI: 10.1186/s12890-026-04163-9
Liming He, Yibing Cheng, Li Huang, Zhenyu Zhang, Qunqun Zhang, Ling Gong, Tian Li, Xiulan Lu, Xiaodi Cai, Gangfeng Yan
Background: Metagenomic next-generation sequencing (mNGS) plays a critical role in the rapid detection of infectious pathogens. We aimed to analyze the clinical characteristics of Pneumocystis jirovecii infection in children without HIV infection and to evaluate the performance of mNGS in distinguishing P. jirovecii colonization from true infection.
Methods: A multicenter, retrospective analysis was conducted on critically ill, non-HIV-infected pediatric patients who tested positive for P. jirovecii via mNGS analysis of bronchoalveolar lavage fluid (BALF). Group differences were assessed using Mann-Whitney U-tests (for continuous data) and chi-square tests (for categorical data). Discriminatory performance was evaluated by calculating the area under the receiver operating characteristic curve.
Results: A total of 59 HIV-negative children (age range: 2 months to 14 years) from four children's hospitals were included and classified into two groups based on P. jirovecii status: P. jirovecii pneumonia (PCP; n = 51) and P. jirovecii colonization (PCC; n = 8). Compared with the PCC group, the PCP group had significantly higher serum C-reactive protein levels and median P. jirovecii read counts in mNGS (both P < 0.05). The optimal threshold value for discriminating P. jirovecii infection from colonization appeared to be 556 reads (sensitivity, 77.6%; specificity, 100.0%). Eighteen patients (35.3%) in the PCP group died. Compared with survivors, these patients were significantly younger, had lower T-cell subset counts (CD3+, CD4+, and CD8+), and a higher prevalence of primary immunodeficiency (all P < 0.05).
Conclusions: BALF mNGS analysis may have utility for differentiating between colonization and infection by P. jirovecii, warranting further investigation.
{"title":"Metagenomic next-generation sequencing to detect Pneumocystis jirovecii pneumonia in critically ill, HIV-negative children: a retrospective multicenter study.","authors":"Liming He, Yibing Cheng, Li Huang, Zhenyu Zhang, Qunqun Zhang, Ling Gong, Tian Li, Xiulan Lu, Xiaodi Cai, Gangfeng Yan","doi":"10.1186/s12890-026-04163-9","DOIUrl":"https://doi.org/10.1186/s12890-026-04163-9","url":null,"abstract":"<p><strong>Background: </strong>Metagenomic next-generation sequencing (mNGS) plays a critical role in the rapid detection of infectious pathogens. We aimed to analyze the clinical characteristics of Pneumocystis jirovecii infection in children without HIV infection and to evaluate the performance of mNGS in distinguishing P. jirovecii colonization from true infection.</p><p><strong>Methods: </strong>A multicenter, retrospective analysis was conducted on critically ill, non-HIV-infected pediatric patients who tested positive for P. jirovecii via mNGS analysis of bronchoalveolar lavage fluid (BALF). Group differences were assessed using Mann-Whitney U-tests (for continuous data) and chi-square tests (for categorical data). Discriminatory performance was evaluated by calculating the area under the receiver operating characteristic curve.</p><p><strong>Results: </strong>A total of 59 HIV-negative children (age range: 2 months to 14 years) from four children's hospitals were included and classified into two groups based on P. jirovecii status: P. jirovecii pneumonia (PCP; n = 51) and P. jirovecii colonization (PCC; n = 8). Compared with the PCC group, the PCP group had significantly higher serum C-reactive protein levels and median P. jirovecii read counts in mNGS (both P < 0.05). The optimal threshold value for discriminating P. jirovecii infection from colonization appeared to be 556 reads (sensitivity, 77.6%; specificity, 100.0%). Eighteen patients (35.3%) in the PCP group died. Compared with survivors, these patients were significantly younger, had lower T-cell subset counts (CD3<sup>+</sup>, CD4<sup>+</sup>, and CD8<sup>+</sup>), and a higher prevalence of primary immunodeficiency (all P < 0.05).</p><p><strong>Conclusions: </strong>BALF mNGS analysis may have utility for differentiating between colonization and infection by P. jirovecii, warranting further investigation.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146131282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-06DOI: 10.1186/s12890-026-04165-7
Ning Pang, Rongrong Fan, Mengqi Cao, Chaoqun Ma, Yingkun Liu, Lin Huang, Xiaohong Zhang, Yi Liu, Yanguo Liu
Introduction: Postoperative pulmonary air leaks is one of the most significant complications, which prolongs hospitalization and increases mortality. The sclerosant interleukin-2 (IL-2) has been widely used in China to reduce postoperative air leaks. However, the optimal suction pressure on digital drainage systems when combined with IL-2 treatment remains elusive among thoracic surgeons. This study aimed to determine the optimal suction pressure provided by digital drainage system, together with IL-2 therapy, for effectively sealing air leaks, and to identify the key factors influencing the healing of air leakage.
Methods: A retrospective analysis involving 94 patients was conducted. The collected information included patient demographics, IL-2 treatment regimens, the average suction pressure applied during the IL-2 therapy period, and air leak duration. The suction pressures were categorized into low (1 ≤ pressure < 5 cmH2O), medium (5 ≤ pressure < 9 cmH2O) and high (9 ≤ pressure ≤ 12 cmH2O) pressure groups. Univariate and multivariate analyses were employed to evaluate the impact of various variables on the time required for air leak sealing. The variables assessed included age, gender, diagnosis, pulmonary complications, resection site, surgery type, operative time, intraoperative blood loss, and suction pressure during IL-2 treatments.
Results: The analysis revealed that suction pressure was a statistically significant determinant of air leak duration (P = 0.036). In multivariate analysis, high suction pressure was independently associated with a longer air leak time by approximately 2.84 days (95% CI: -5.16, -0.52, P = 0.017) and 2.21 days (95% CI: -4.08, -0.35, P = 0.021) compared to low and medium pressure, respectively. Subgroup analysis showed no significant interaction between suction pressure and patient/surgical characteristics, indicating the benefit of lower suction was consistent across the cohort. The number of IL-2 treatments was also a strong predictor of air leak duration (P < 0.001), reflecting the need for repeated therapy in persistent leaks.
Conclusions: Patients receiving IL-2 therapy benefited more from mild suction pressures (low or medium) compared to high suction pressure, resulting in shorter air leak durations across a range of patient and surgical characteristics. These findings support the use of low-to-medium suction (1-9 cmH₂O) as part of the standard management protocol when IL-2 pleurodesis is employed.
简介:术后肺部漏气是最重要的并发症之一,它延长了住院时间,增加了死亡率。在中国,硬化性白细胞介素-2 (IL-2)已被广泛应用于减少术后空气泄漏。然而,在胸外科医生中,数字引流系统联合IL-2治疗时的最佳吸引压力仍然难以捉摸。本研究旨在确定数字引流系统提供的最佳吸入压力,结合IL-2治疗,有效密封漏气,并确定影响漏气愈合的关键因素。方法:对94例患者进行回顾性分析。收集的信息包括患者人口统计、IL-2治疗方案、IL-2治疗期间的平均吸入压力和漏气持续时间。吸入压力分为低压力组(1≤压力2O)、中压力组(5≤压力2O)和高压力组(9≤压力≤12 cmH2O)。采用单变量和多变量分析来评估各种变量对空气泄漏密封所需时间的影响。评估的变量包括年龄、性别、诊断、肺部并发症、切除部位、手术类型、手术时间、术中出血量和IL-2治疗期间的吸入压力。结果:分析显示,吸气压力是空气泄漏持续时间的决定因素(P = 0.036)。在多变量分析中,与低压和中压相比,高吸入压力与较长的漏气时间分别独立相关,分别为2.84天(95% CI: -5.16, -0.52, P = 0.017)和2.21天(95% CI: -4.08, -0.35, P = 0.021)。亚组分析显示,抽吸压力与患者/手术特征之间没有显著的相互作用,表明低抽吸的益处在整个队列中是一致的。IL-2治疗的次数也是空气泄漏持续时间的一个强有力的预测指标(P结论:与高吸入压力相比,接受IL-2治疗的患者从轻度吸入压力(低或中等)中获益更多,导致在一系列患者和手术特征中更短的空气泄漏持续时间。这些发现支持在采用IL-2胸膜融合术时使用低至中吸(1-9 cmH₂O)作为标准管理方案的一部分。
{"title":"Optimal suction pressure on digital drainage systems for the management of postoperative air leaks together with IL-2 therapy: a retrospective cohort study.","authors":"Ning Pang, Rongrong Fan, Mengqi Cao, Chaoqun Ma, Yingkun Liu, Lin Huang, Xiaohong Zhang, Yi Liu, Yanguo Liu","doi":"10.1186/s12890-026-04165-7","DOIUrl":"https://doi.org/10.1186/s12890-026-04165-7","url":null,"abstract":"<p><strong>Introduction: </strong>Postoperative pulmonary air leaks is one of the most significant complications, which prolongs hospitalization and increases mortality. The sclerosant interleukin-2 (IL-2) has been widely used in China to reduce postoperative air leaks. However, the optimal suction pressure on digital drainage systems when combined with IL-2 treatment remains elusive among thoracic surgeons. This study aimed to determine the optimal suction pressure provided by digital drainage system, together with IL-2 therapy, for effectively sealing air leaks, and to identify the key factors influencing the healing of air leakage.</p><p><strong>Methods: </strong>A retrospective analysis involving 94 patients was conducted. The collected information included patient demographics, IL-2 treatment regimens, the average suction pressure applied during the IL-2 therapy period, and air leak duration. The suction pressures were categorized into low (1 ≤ pressure < 5 cmH<sub>2</sub>O), medium (5 ≤ pressure < 9 cmH<sub>2</sub>O) and high (9 ≤ pressure ≤ 12 cmH<sub>2</sub>O) pressure groups. Univariate and multivariate analyses were employed to evaluate the impact of various variables on the time required for air leak sealing. The variables assessed included age, gender, diagnosis, pulmonary complications, resection site, surgery type, operative time, intraoperative blood loss, and suction pressure during IL-2 treatments.</p><p><strong>Results: </strong>The analysis revealed that suction pressure was a statistically significant determinant of air leak duration (P = 0.036). In multivariate analysis, high suction pressure was independently associated with a longer air leak time by approximately 2.84 days (95% CI: -5.16, -0.52, P = 0.017) and 2.21 days (95% CI: -4.08, -0.35, P = 0.021) compared to low and medium pressure, respectively. Subgroup analysis showed no significant interaction between suction pressure and patient/surgical characteristics, indicating the benefit of lower suction was consistent across the cohort. The number of IL-2 treatments was also a strong predictor of air leak duration (P < 0.001), reflecting the need for repeated therapy in persistent leaks.</p><p><strong>Conclusions: </strong>Patients receiving IL-2 therapy benefited more from mild suction pressures (low or medium) compared to high suction pressure, resulting in shorter air leak durations across a range of patient and surgical characteristics. These findings support the use of low-to-medium suction (1-9 cmH₂O) as part of the standard management protocol when IL-2 pleurodesis is employed.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146131213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-06DOI: 10.1186/s12890-026-04152-y
Sumin Jo, Hyunji Choi, Jaejun Lee, Taeyun Kim
{"title":"Association between steatotic liver disease (MASLD, met-ALD, and ALD) with preserved ratio impaired spirometry: a population-based study.","authors":"Sumin Jo, Hyunji Choi, Jaejun Lee, Taeyun Kim","doi":"10.1186/s12890-026-04152-y","DOIUrl":"https://doi.org/10.1186/s12890-026-04152-y","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146131226","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1186/s12890-026-04155-9
Qingcai Lin
Background: Optimal management for first-episode primary spontaneous pneumothorax (PSP) with pulmonary blebs remains uncertain, balancing recurrence prevention against procedural costs. This study compared video-assisted thoracoscopic surgery (VATS) and chest tube drainage in terms of recurrence prevention and cost-effectiveness, incorporating sensitivity analyses to evaluate robustness across variable assumptions.
Methods: In a retrospective cohort (2010-2020), 245 first-episode PSP patients with computed tomography (CT)-confirmed blebs were included. Propensity score matching (1:1, caliper = 0.02) balanced baseline characteristics (age, bleb size, etc.), generating 33 matched pairs. Primary outcomes were recurrence rate and incremental cost-effectiveness ratio (ICER).
Results: VATS reduced 5-year recurrence rates from 48.5% to 12.1% (P = 0.004; absolute risk reduction [ARR] = 36.4%, number needed to treat [NNT] = 2.75) and improved recurrence-free interval (hazard ratio [HR] = 0.166, P < 0.001). The base-case ICER was ¥160,300 per quality-adjusted life-year (QALY) gained (¥48,937 per recurrence avoided), with 99.14% probability of cost-effectiveness at China's World Health Organization (WHO)-recommended willingness-to-pay (WTP) threshold (¥287,247 / QALY). Sensitivity analyses confirmed robustness: ICER remained favorable at ¥156,338 / QALY when pneumothorax utility dropped to 0.5; a 20% cost increase yielded ¥192,200 / QALY. Discount rate variations (3%: ¥159,800 / QALY; 6%: ¥138,029 / QALY) maintained > 98.4% cost-effectiveness probability.
Conclusions: VATS reduces recurrence by 83.4% in first-episode PSP with blebs and demonstrates robust cost-effectiveness. Early surgical intervention should be considered for patients with blebs across diverse resource settings.
{"title":"Efficacy and cost-effectiveness of VATS versus chest tube drainage in first-episode primary spontaneous pneumothorax with blebs: a propensity score-matched retrospective study.","authors":"Qingcai Lin","doi":"10.1186/s12890-026-04155-9","DOIUrl":"https://doi.org/10.1186/s12890-026-04155-9","url":null,"abstract":"<p><strong>Background: </strong>Optimal management for first-episode primary spontaneous pneumothorax (PSP) with pulmonary blebs remains uncertain, balancing recurrence prevention against procedural costs. This study compared video-assisted thoracoscopic surgery (VATS) and chest tube drainage in terms of recurrence prevention and cost-effectiveness, incorporating sensitivity analyses to evaluate robustness across variable assumptions.</p><p><strong>Methods: </strong>In a retrospective cohort (2010-2020), 245 first-episode PSP patients with computed tomography (CT)-confirmed blebs were included. Propensity score matching (1:1, caliper = 0.02) balanced baseline characteristics (age, bleb size, etc.), generating 33 matched pairs. Primary outcomes were recurrence rate and incremental cost-effectiveness ratio (ICER).</p><p><strong>Results: </strong>VATS reduced 5-year recurrence rates from 48.5% to 12.1% (P = 0.004; absolute risk reduction [ARR] = 36.4%, number needed to treat [NNT] = 2.75) and improved recurrence-free interval (hazard ratio [HR] = 0.166, P < 0.001). The base-case ICER was ¥160,300 per quality-adjusted life-year (QALY) gained (¥48,937 per recurrence avoided), with 99.14% probability of cost-effectiveness at China's World Health Organization (WHO)-recommended willingness-to-pay (WTP) threshold (¥287,247 / QALY). Sensitivity analyses confirmed robustness: ICER remained favorable at ¥156,338 / QALY when pneumothorax utility dropped to 0.5; a 20% cost increase yielded ¥192,200 / QALY. Discount rate variations (3%: ¥159,800 / QALY; 6%: ¥138,029 / QALY) maintained > 98.4% cost-effectiveness probability.</p><p><strong>Conclusions: </strong>VATS reduces recurrence by 83.4% in first-episode PSP with blebs and demonstrates robust cost-effectiveness. Early surgical intervention should be considered for patients with blebs across diverse resource settings.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146117841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-02-05DOI: 10.1186/s12890-026-04151-z
Kadir Baturhan Ciflik, Busra Ozdemir Ciflik
{"title":"Evaluation of multimodal large language models for pneumothorax assessment in real-world clinical scenarios.","authors":"Kadir Baturhan Ciflik, Busra Ozdemir Ciflik","doi":"10.1186/s12890-026-04151-z","DOIUrl":"https://doi.org/10.1186/s12890-026-04151-z","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146123832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.
Methods: This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (P < 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.
Results: Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; P = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; P = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; P = 0.009), IgG (380 vs. 720 mg/dL; P = 0.007), and IgE (0.8 vs. 12 IU/mL; P = 0.008).
Conclusion: Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (P = 0.009, P = 0.007, P = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.
背景:联合免疫缺陷(CID)涉及B和T淋巴细胞发育和功能的严重缺陷。本研究检查了有和没有肺部表现的CID患者的临床和免疫表型。方法:这项回顾性多中心研究纳入了2009年至2022年间诊断为CID的53例患者,并进行了可用的胸部计算机断层扫描。根据肺部表现对患者进行分类。采用保守统计学阈值比较人口学、临床和实验室特征(P)。结果:53例患者(56.6%为男性)中,43例HRCT表现为肺部异常。常见的临床特征包括皮肤病变(43.4%)、生长失败(34%)和自身免疫(32.1%)。HRCT显示肺炎(28.3%)、支气管扩张(18.9%)、boop样肺间质性疾病(3.8%)及其他表现。使用儿童年龄校正参考范围,证实了严重的免疫缺陷:92%(49/53)的绝对淋巴细胞计数低于第5百分位数,94%(47/50)的CD3 + T细胞低于第5百分位数,96%(51/53)的CD4 + T细胞低于第5百分位数,94%(50/53)的CD19 + B细胞低于第5百分位数,98%(52/53)的低γ -球蛋白血症(IgG低于第5百分位数)。HRCT异常患者CD4 + t细胞计数(178比498细胞/µL, P = 0.008)和CD19 + b细胞计数(42比189细胞/µL, P = 0.009)显著降低。支气管镜检查发现了烟曲霉、肺炎链球菌和耐多药鲍曼不动杆菌。死亡患者的基线血小板(183,000 vs. 266,000细胞/ μ L, P = 0.009)、IgG (380 vs. 720 mg/dL, P = 0.007)和IgE (0.8 vs. 12 IU/mL, P = 0.008)均显著降低。结论:81.1%的伊朗CID患者有肺部表现。低基线血小板、IgG和IgE构成了死亡率的可靠预后三因素(P = 0.009, P = 0.007, P = 0.008)。年龄调整参考值范围的应用揭示了深刻的免疫缺陷。在资源有限的情况下,采用低剂量方案进行系统的HRCT监测,并将传染性后遗症与免疫介导的肺部疾病区分开来,可指导有针对性的管理。
{"title":"Demographic, clinical, and immunological features in combined immunodeficiency patients: a comparative analysis of those with and without pulmonary manifestations - a multicenter study from Iran.","authors":"Ghamartaj Khanbabaee, Matin Pourghasem, Mahnaz Jamee, Seyed Ahmad Tabatabaii, Mitra Khalili, Samin Sharafian, Mehrnaz Mesdaghi, Mahnaz Sadeghi-Shabestari, Armin Shirvani, Saeid Sadr, Arefeh Zahmatkesh, Samaneh Delavari, Narges Eslami, Nazanin Farahbakhsh, Mahboubeh Mansouri, Ebrahim Tabiei, Seyedeh Zalfa Modarresi, Abdolhamid Taghizadeh Behbahani, Golnaz Eslamian, Mazdak Fallahi, Javad Enayat, Shahrzad Fallah, Mahsa Pourghasem, Asghar Aghamohammadi, Zahra Chavoshzadeh","doi":"10.1186/s12890-026-04115-3","DOIUrl":"https://doi.org/10.1186/s12890-026-04115-3","url":null,"abstract":"<p><strong>Background: </strong>Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.</p><p><strong>Methods: </strong>This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (P < 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.</p><p><strong>Results: </strong>Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; P = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; P = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; P = 0.009), IgG (380 vs. 720 mg/dL; P = 0.007), and IgE (0.8 vs. 12 IU/mL; P = 0.008).</p><p><strong>Conclusion: </strong>Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (P = 0.009, P = 0.007, P = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146097049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}