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ABSTRACT Objectives: This study primarily aimed to investigate the clinical determinants of the Modified Incremental Step Test (MIST) in adults with non-cystic fibrosis bronchiectasis (NCFB). A secondary objective was to compare the cardiopulmonary responses after the MIST and Incremental Shuttle Walk Test (ISWT), two commonly adopted symptom-limited maximum field tests in chronic respiratory diseases. Methods: Forty-six patients with clinically stable bronchiectasis participated in this cross-sectional study. MIST and ISWT were performed to determine exercise capacity, while disease severity, fatigue, and quality of life were assessed using the Bronchiectasis Severity Index (BSI), the Fatigue Severity Scale (FSS), and St. George's Respiratory Questionnaire (SGRQ), respectively. Quadriceps muscle strength was evaluated using a hand-held dynamometer, walking speed with a wireless inertial sensing device, and the level of physical activity (steps/day) with a pedometer. Results: The BSI score, quadriceps muscle strength, daily step count, and the SGRQ total score explained 61.9% of the variance in the MIST (p < 0.001, R2 = 0.67, AR2 = 0.619). The BSI score (r = -0.412, p = 0.004), quadriceps muscle strength (r = 0.574, p = 0.001), daily step count (r = 0.523, p < 0.001), walking speed (r = 0.402, p = 0.006), FSS score (r = -0.551, p < 0.001), and SGRQ total score (r = -0.570, p < 0.001) correlated with the MIST. The patients achieved higher heart rates (HR), HR%, desaturation, dyspnea, and leg fatigue in the MIST compared to the ISWT (p < 0.05). Conclusions: Disease severity, quadriceps muscle strength, physical activity level, and quality of life were determinants of MIST. The advantages of the MIST, including higher cardiopulmonary response than ISWT and greater portability, which facilitates its use in various settings, make MIST the preferred choice for investigating symptom-limited exercise capacity in patients with NCFB.
RESUMO Objetivos: Este estudo teve como objetivo principal investigar os determinantes clínicos do Teste do Degrau Incremental Modificado (TDIM) em adultos com bronquiectasia não fibrocística (BNFC). Um objetivo secundário foi comparar as respostas cardiopulmonares após o TDIM e o Teste Graduado de Caminhada (TGC), dois testes de campo máximos amplamente adotados e limitados por sintomas em doenças respiratórias crônicas. Métodos: Quarenta e seis pacientes com bronquiectasia clinicamente estável participaram deste estudo transversal. O TDIM e TGC foram realizados para determinar a capacidade de exercício, enquanto a gravidade da doença, fadiga e qualidade de vida foram avaliadas usando o Índice de Gravidade da Bronquiectasia (BSI), a Escala de Gravidade da Fadiga (FSS) e o Questionário Respiratório de Saint George (SGRQ), respectivamente. A força muscular do quadríceps foi avaliada usando um dinamômetro manual, a velocidade de caminhada com um dispositivo de sensor inercial sem fio e o nível de atividade física (passos/dia) com um pedômetro. Resultados: O escore BSI, a força muscular do quadríceps, a contagem diária de passos e o escore total do SGRQ explicaram 61,9% da variação no TDIM (p < 0,001, R2 = 0,67, AR2 = 0,619). O escore BSI (r = -0,412, p = 0,004), a força muscular do quadríceps (r = 0,574, p = 0,001), a contagem diária de passos (r = 0,523, p < 0,001), a velocidade de caminhada (r = 0,402, p = 0,006), o escore FSS (r = -0,551, p < 0,001) e o escore total do SGRQ (r = -0,570, p < 0,001) correlacionaram-se com o TDIM. Os pacientes atingiram maiores frequências cardíacas (FC), FC%, dessaturação, dispneia e fadiga nas pernas no TDIM em comparação com o TGC (p < 0,05). Conclusões: A gravidade da doença, a força muscular do quadríceps, o nível de atividade física e a qualidade de vida foram determinantes do TDIM. As vantagens do TDIM, incluindo uma resposta cardiopulmonar mais elevada que no TGC e maior portabilidade, que facilita sua utilização em diversos ambientes, fazem do TDIM a escolha preferencial para investigar a capacidade de exercício limitada por sintomas em pacientes com BNFC.
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Resumen Introducción: La bronquiolitis obliterante postinfecciosa es una enfermedad pulmonar poco frecuente; existen limitados reportes en Sudamérica. Caso clínico: En esta serie se reportan 10 pacientes con esta enfermedad diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú). La mediana de edad al diagnóstico fue de 19 meses. Todos los pacientes presentaron el antecedente de infección respiratoria aguda grave. Los síntomas más frecuentes fueron tos, dificultad respiratoria, sibilancias e hipoxemia; el patrón de atenuación en mosaico fue la característica más frecuente en la tomografía. Todos tenían serología positiva para adenovirus. Se administró tratamiento con pulsos de metilprednisolona, azitromicina, hidroxicloroquina y corticoides inhalados. Ningún paciente falleció durante el seguimiento. Conclusiones: En los niños previamente sanos con antecedente de infección respiratoria aguda grave y sintomatología obstructivo bronquial persistente se debe considerar el diagnóstico de bronquiolitis obliterante postinfecciosa. Este es el primer reporte en Perú con un régimen terapéutico adaptado a nuestra institución.
Abstract Background: Postinfectious bronchiolitis obliterans is a rare lung disease; there are limited reports in South America. Case report: We report 10 patients with this disease diagnosed at the Instituto Nacional de Salud del Niño-Breña (Lima-Peru). The median age at diagnosis was 19 months and all patients had a history of severe acute respiratory infection. The most frequent symptoms were cough, respiratory distress, wheezing, and hypoxemia. The mosaic attenuation pattern was the most frequent on the tomography. All the patients had positive serology for adenovirus. The treatment received was methylprednisolone pulses, azithromycin, hydroxychloroquine, and inhaled corticosteroids. No patient died during the follow-up. Conclusions: In previously healthy children with a history of severe acute respiratory infection and persistent bronchial obstructive symptoms, the diagnosis of postinfectious bronchiolitis obliterans should be considered. This is the first report in Peru with a therapeutic regimen adapted to our institution.
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Background- Bronchiectasis is a common chronic respiratory disease. A noticeable drop in lung function during exacerbations and recovery during convalescence has been revealed in patients with asthma or chronic obstructive pulmonary disease (COPD). Hence this study was planned with the objectives to compare lung functions during acute exacerbation and convalescence in patients with bronchiectasis. This was a prospective cohort study Methods- conducted in 50 patients over a duration of 18 months. Measurement of exacerbations and convalescence visits comprised of spirometry, sputum bacteriology, serum and sputum biomarker. Student's paired t test, Fischer's exact test or Chi square test was used to analyze the signicance of difference. P value <0.05 was considered as statistically signicant. In this study, Results- there was a signicant association of mMRC dyspnoea score in exacerbation and in convalescence. (p <0.0001). The analysis showed that mMRC dyspnoea score was shifted to lower scores in convalescence compared to that in exacerbation. FEV1, FVC and FEV1/FVC) and 6 MWT distance were signicantly lower in patients in exacerbation as compared in convalescence. Conclusion- There was marked improvement in lung functions in convalescence as compared to patients in acute exacerbation
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Primary ciliary dyskinesia (PCD) is an autosomal recessive hereditary disease that includes various forms of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. Kartagener?s syndrome is a rare disorder and the prevalence is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. The defective movement of cilia leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. The diagnosis is made clinically and confirmed through electron microscopy, which reveals abnormalities of structural organization of the axoneme in cilia from respiratory epithelia and in spermatozoa. Underlying structural defects include 1) absent inner and/or outer dynein arms, 2) tubular defects, and 3) radial spoke defects. We hereby report a rare case of Kartagener?s syndrome, in an infertile male with immotile sperms. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in making the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.
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BACKGROUND Bronchiectasis and cavitation are key features of acute and chronic pulmonary infections. Cavitary lesions may carry unfavourable prognosis with regard to complete restoration of pulmonary function in few patients. OBJECTIVES To evaluate type and site of bronchiectasis, its relation with cavity formation on computed tomography and providing an insight to sorting out subjects suited for physiotherapy. A radiological sign-“Feeding bronchus sign” has been discussed with reference to bronchiectasis, its origin, natural course and a suggestion of guarded future management and rehabilitation. METHODS Total 150 chest CT scans with presence of cavity and bronchiectasis were retrospectively reviewed and followed up for type, site of bronchiectasis, signs of active infection, site of cavity and presence of “feeding bronchus sign”. Final diagnosis was confirmed by sputum sample, acid-fast bacillus test or culture or polymerase chain reaction. RESULTS Out of 150 cases, 70 (46%) had chronic and 80 (53%) had active infection. 33 (22%) had solitary and 117 (78%) had multiple cavities. 37 (34.6 %) patients had cylindrical, 11 (7.3 %) had varicose, 27 (18%) had cystic bronchiectasis, 23 (15.3%) had cylindrical and varicose, 19 (12.6%) had cylindrical and cystic and 33 (22%) had all three types. “Feeding bronchus sign” was observed in 102 (68%) patients. Radiological evidence of disease progression was seen in 21 patients, improvement in 19 and no change in 17 on follow-up CT. CONCLUSION Patients with positive “Feeding bronchus sign” are at risk for increased disease transmission and secondary opportunistic infections. Improvement and maintenance of quality of life is ultimate goal of management. Apart from antibiotics, pulmonary rehabilitation also plays an important role in cavitary lung disease
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This paper summarized the current status of the commonly used quality of life assessment scales of bronchiectasis （BE） at home and abroad， and compared the characteristics and differences of each scale from the basic framework， domain， item pool， application status and quality assessment， so as to provide a basis for the development and application of scales based on the mode of combination of disease and syndrome. There are currently 12 assessment tools for evaluating the quality of life in BE with good reliability， validity， and responsiveness， but only quality of life - bronchiectasis （QOL-B） and bronchiectasis health questionnaire （BHQ） are specifically developed for BE patients. The development process of these scales is mostly rooted in western culture and modern medicine， making it difficult to highlight the characteristics and advantages of traditional Chinese medicine （TCM）. The content of the scales lacks the outcome indicators that BE patients are most concerned about， and cannot fully reflect the subjective feelings of patients. The development and assessment of scales lacks clinical research data and methodological support. Under the guidance of TCM syndrome differentiation and treatment， the development of scales based on the mode of combination of disease and syndrome can emerge the characteristics of TCM， reflect the true feelings of patients， and make up for the limitations of the existing scales of BE. Therefore， based on domestic and international scales， factors of China's national conditions and cultural characteristics of TCM should be considered to develop the scale suitable for Chinese people， which requires the construction of a theoretical model for the combination of disease and syndrome， by taking patients as the center and evaluating the performance and quality of the scale.
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ABSTRACT BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare and heterogeneous disease that is difficult to diagnose and requires complex and expensive diagnostic tools. The saccharin transit time test is a simple and inexpensive tool that may assist in screening patients with PCD. OBJECTIVES: This study aimed to compare changes in the electron microscopy findings with clinical variables and saccharin tests in individuals diagnosed with clinical PCD (cPCD) and a control group. DESIGN AND SETTING: An observational cross-sectional study was conducted in an otorhinolaryngology outpatient clinic from August 2012 to April 2021. METHOD: Patients with cPCD underwent clinical screening questionnaires, nasal endoscopy, the saccharin transit time test, and nasal biopsy for transmission electron microscopy. RESULTS: Thirty-four patients with cPCD were evaluated. The most prevalent clinical comorbidities in the cPCD group were recurrent pneumonia, bronchiectasis, and chronic rhinosinusitis. Electron microscopy confirmed the clinical diagnosis of PCD in 16 of the 34 (47.1%) patients. CONCLUSION: The saccharin test could assist in screening patients with PCD due to its association with clinical alterations related to PCD.
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@#Objective To study the correlation of preoperative hemoglobin amount with venous thromboembolism (VTE) after surgical treatment of bronchiectasis and the clinical significance. Methods A retrospective study was performed on patients with bronchiectasis who underwent surgical treatment in our center from June 2017 to November 2021. The differences in blood parameters between the VTE patients and non-VTE patients were compared. The relationship between preoperative hemoglobin and VTE was confirmed by quartile grouping and receiver operating characteristic (ROC) curve. Results A total of 122 patients were enrolled, including 50 males and 72 females, with a mean age of 52.52±12.29 years. The overall incidence of VTE after bronchiectasis was 9.02% (11/122). Preoperative hemoglobin amount (OR=0.923, 95%CI 0.870-0.980, P=0.008) and D-dimer amount (OR=1.734, 95%CI 1.087-2.766, P=0.021) were independent influencing factors for VTE after bronchiectasis. The incidence of VTE after bronchiectasis decreased gradually with the increase of preoperative hemoglobin amount. The area under the ROC curve (AUC) of postoperative D-dimer alone was 0.757, whereas the AUC of postoperative D-dimer combined with preoperative hemoglobin amount was 0.878. Conclusion Low preoperative hemoglobin is an independent risk factor for postoperative VTE. Postoperative D-dimer combined with preoperative hemoglobin amount has a better predictive performance compared with postoperative D-dimer alone for postoperative VTE.
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Objective The epidemiological characteristics and risk factors of infection of non-tuberculous mycobacterial (NTM) by elderly bronchiectasis patients in Huai 'an area were analyzed, and the theoretical basis for prevention of NTM infection by elderly bronchiectasis patients in Huai 'an area was provided. Methods Among the 371 elderly patients with bronchiectasis admitted to our hospital from January 2020 to June 2022 were selected and divided into control group and NTM group according to whether they had NTM or not. The NTM strains were isolated and identified. Clinical data of patients were collected from the medical record system. Independent risk factors of NTM infection in elderly patients with ramus were analyzed by univariate analysis and logistic regression, including gender, age, previous smoking status, number of ramus, pulmonary cavities, hypoproteinemia, and CD4+T cell level. Results A total of 108 cases NTM infection (29.11%) among the 371 patients with branch enlargement. There was no statistical significance in cough, phlegm, hemoptysis and fever between the two groups (P>0.05). The proportion of chest tightness and shortness in NTM group was significantly higher than that in control group (P20 years (OR=1.692), number of branchial dilated lobe ≥5 (OR=2.671) and thin-walled cavity (OR=2.458) were independent risk factors for NTM infection in elderly patients with branchial dilated lobe (P20 years, and thin-walled cavity. Patients should actively quit smoking, improve the body immunity, and prevent NTM infection in patients with bronchiectasis.
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Bronchiectasis is characterized by a “vicious cycle” involving compromised host defense， mpaired clearance of airway mucus， bacterial colonization， infections， and inflammation， leading to frequent acute exacerbations and diminished quality of life. Based on the hypothesis of “vicious circle”， this paper explores the treatment of bronchiectasis by traditional Chinese medicine （TCM） from three aspects. Firstly， dissipating excessive “phlegm” secretion and enhancing airway clearance form the foundation of the treatment， aiming to improve the condition of mucus hypersecretion. Secondly， invigorating spleen for strengthening vital energy can improve the function of immune system and reduce recurrent infections and acute attacks. Lastly， clearing heat and purging the lung can alleviate infection and inflammatory damage. By employing these methods， TCM can disrupt the progression of the “vicious circle”， delaying disease advancement， minimizing acute exacerbations， and improving the quality of life for patients.
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This paper summarized the experience in treating bronchiectasis with Mahuang Shengma Decoction （麻黄升麻汤）. The pathogenesis of bronchiectasis is “lung-spleen qi deficiency” as the root， and “phlegm-heat obstructing the lung” as the branch. The key point of treatment is to improve the internal environment of phlegm， heat， and deficiency. According to clinical experience， Mahuang Shengma Decoction is good at raising the yang qi to dissipate fire， clearing the upper and warming the lower， which is in accord with the pathogenesis of bronchiectasis. In clinical practice， Mahuang Shengma Decoction is usually used as the basic formula， and the heat-clearing medicinals and center-warming medicinals of the formula will be adjusted according to the abnormal exuberance of heat or cold of the pathogenesis； and the formula can also be modified in accordance with the symptoms. At the same time， importance should be attached to the application of Mahuang （Herba Ephedrae）， and its dosage should be flexibly adjusted according to the constraint degree of the pathogenic qi， so as to expel the constraint fire， bank up earth to generate metal， regulate heat and cold simultaneously， and treat both the root and the branch.
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Objective To analyze the epidemiological characteristics and risk factors of bronchiectasis in patients with chronic obstructive pulmonary disease (COPD) in Suining area, and to provide theoretical basis for COPD prevention and treatment of bronchiectasis. Methods A total of 582 patients with ACUTE exacerbation of COPD (AECOPD) admitted to grade A Hospitals in Suining area from 2019 to 2020 were selected as the investigation objects. According to whether the patients had bronchiectasis, they were divided into control group (without bronchiectasis, n=485) and observation group (with bronchiectasis, n=97). The risk factors of bronchiectasis in COPD patients were analyzed by univariate analysis and logistic regression. The clinical data of the two groups were compared, including age, sex, whether there were other diseases, respiratory tract infection, proportion of purpuric sputum, colonization rate of Pseudomonas aeruginosa. Results Of 16.84% (49/291) AECOPD patients occurred Bronchiectasis. The colonization rate of Pseudomonas aeruginosa in observation group was significantly higher than that in control group (P<0.05). The values of FVC and FEV1/FVC in observation group were significantly lower than those in control group (P<0.05). The levels of serum CRP and PCT in the observation group were significantly higher than those in the control group (P<0.05). Male (OR=2.515), high proportion of GOLD grade III/IV (OR=3.654), smoking (OR=3.472), diabetes (OR=3.829) and bacterial infection (OR=4.159) were independent risk factors for bronchiectasis in COPD patients (P<0.05). Conclusion COPD patients in Suining area have a high risk of bronchiectasis.The lung function was declined significantly.It has a high colonization rate of Pseudomonas aeruginosa. Patients who are male, have a high proportion of GOLD class III/IV, smoke, have diabetes, and have bacterial infections should be given interventions that can reduce the risk of bronchiectasis in COPD patients.
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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of systemic small vasculitis characterized by the detection of ANCA in serum. Bactericidal permeability enhancing protein (BPI) is one of the target antigens of ANCA. BPI-ANCA-associated vasculitis is not common clinically, and the combination of bronchiectasis is not accidental. The paper reported a case of BPI-ANCA-associated vasculitis with renal damage combined with bronchiectasis. We reviewed relevant literature to explore the characteristics of BPI-ANCA-associated vasculitis and the correlation between bronchiectasis and ANCA-associated vasculitis, so as to improve the clinician's understanding on this disease.
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Objective To analyze the characteristics and influencing factors of bronchiectasis in patients with chronic obstructive pulmonary disease (COPD) in Qiaokou District of Wuhan from 2016 to 2020. Methods The clinical data of 412 COPD patients admitted to Wuhan First Hospital from January 2016 to December 2020 were retrospectively analyzed. Patients were divided into combined group (162 cases) and non-combined group (250 cases) according to whether they were complicated with bronchiectasis by the high-resolution chest CT examination. The differences in basic data, laboratory indexes, and lung function indexes between the two groups of patients were compared. Multivariate logistic regression analysis was used to explore the influencing factors for COPD patients complicated with bronchiectasis from 2016 to 2020 in Qiaokou District of Wuhan. Results The proportion of COPD patients with smoking history, the proportion of pulmonary tuberculosis history, the proportion of producing yellow-white sputum and yellow sputum, and the duration of symptoms in the combined group were significantly higher than those in the non-combined group (P<0.05). The PO2 and albumin levels of patients in the combined group were lower than those in the non-combined group (P<0.05), but the proportion of PCO2, hemoglobin, sputum culture positive, and the proportion of Pseudomonas aeruginosa infection were significantly higher than those in the non-combined group (P<0.05). The values of the FEV1, FVC, FEV1/FVC, and FEV1% pred of patients in the combined group were significantly lower than those in the non-combined group (P<0.05). The results of multivariate logistic regression analysis showed that smoking history (OR=3.39, 95% CI: 2.02-5.56), pulmonary tuberculosis history (OR=3.09 , 95%CI: 1.85-5.16), duration of symptoms (OR=3.48, 95% CI: 1.93-6.29) and Pseudomonas aeruginosa infection (OR=3.76, 95% CI: 1.98-7.15) were the risk factors affecting COPD with bronchiectasis (P<0.05). Conclusion The pulmonary function of COPD patients with bronchiectasis decreased significantly in Qiaokou District of Wuhan from 2016 to 2020. The smoking history, tuberculosis history, duration of symptoms, and Pseudomonas aeruginosa infection are risk factors affecting COPD patients complicated with bronchiectasis.
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Introduction: Two different validated scores are currently used to assess the severity of bronchiectasis: the FACED score and the Bronchiectasis Severity Index (BSI). The study was conducted to evaluate clini-cal etiology in bronchiectasis patients. And to compare the results of the assessment of bronchiectasis severity obtained via FACED and BSI scores. Methods: The study was conducted at a tertiary care hospital in the outpatients of the department of respiratory medicine. Detailed clinical history and necessary investigations were done. BSI and the FACED score were calculated. Statistical analysis was performed using the SPSS package. Results: According to the FACED score, we found 28 patients with mild bronchiectasis, 17 with moder-ate, and 5 with severe bronchiectasis. The frequency of patients with low, intermediate, and high BSI was 24, 21, and 5, respectively. Moreover, we observed a weak but statistically significant association of 43% agreement between FACED and BSI scores: Fisher’s exact test(p=0.399), tau-b de Kendall (-0.123; p = 0.337) and kappa test (0.032; p = 0.878). Conclusions: There is a small but significant correlation between the two scales (BSI and FACED). a ten-dency is observed for patients to be classified with a higher BSI compared to the FACED score.
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SUMMARY OBJECTIVE: Bronchiectasis is a chronic respiratory disease characterized by inflammation, irreversible dilation of the bronchi, and recurrent pulmonary infections, with a high morbidity and mortality rate, but is less studied from the point of view of its prevalence and associated factors not directly related to respiratory prognosis. As it is a disease related to the exacerbation of the inflammatory process and oxidative stress, this study searched to investigate the micronucleus frequency in patients with and without bronchiectasis treated at a specialized pulmonology service in a hospital in the extreme south of Brazil. METHODS: Patients with a confirmed tomographic diagnosis of bronchiectasis were defined as cases. Mutagenicity was evaluated by the micronucleus test in patients' oral mucosa cells. Data collection was performed through a questionnaire containing socioeconomic, demographic, lifestyle, and health condition information. RESULTS: Of the 95 patients involved in this study, 21 (22.1%) were diagnosed with bronchiectasis aged between 12 and 89 years. There was no significant difference in the frequency of micronucleus between patients with and without bronchiectasis. There was a significant positive association between age and frequency of micronucleus among patients with bronchiectasis, but this association does not occur among patients without the disease. CONCLUSION: This is the first study to investigate data on the prevalence and clinical and epidemiological aspects of this chronic disease in Brazil, especially those related to the genotoxicity outcome.
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Kartagener?s syndrome is a rare disorder. The estimated prevalence of Kartagener?s syndrome is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. There is a defective movement of cilia, which leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. We hereby report a rare case of Kartagener?s syndrome, an infertile male with azoospermia. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in such patients so that the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.
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Abstract Background: Inborn errors of immunity, mainly Predominantly Antibody deficiencies with normal IgG levels are unrecognized in adults with lung diseases such as bronchiectasis or recurrent pneumonia. Objective: To determine IgM, IgA, IgG2 subclass deficiencies, and Specific antibody deficiency (anti-pneumococcal polysaccharide antibodies) in adults with non-cystic fibrosis bronchiectasis or recurrent pneumonia. Methods: Cross-sectional study. Consecutive patients with non-cystic fibrosis bronchiectasis or recurrent pneumonia were recruited in Cali, Colombia. IgG, IgA, IgM, and IgE, IgG2subclass and IgG anti-pneumococcal serum levels were measured. Results: Among the 110 participants enrolled, Antibody deficiencies with normal serum IgG levels were found in 11(10%) cases. IgA deficiency (3 cases), IgM deficiency (2 cases) and IgG2 deficiency (2 cases) were the most frequent primary immunodeficiencies. In addition, IgG2+IgA deficiency, Ataxia-telangiectasia, Hyper-IgE syndrome and Specific Antibody Deficiency(anti-polysaccharides) were found in one case each. Conclusions: Predominantly antibody deficiencies with normal IgG levels are an important etiology of non-cystic fibrosis bronchiectasis and recurrent pneumonia in adults.
Resumen Antecedentes: Los Errores Innatos de la Inmunidad principalmente las Deficiencias Predominantemente de anticuerpos con niveles normales de IgG no se conocen en adultos con enfermedades pulmonares como las bronquiectasias o la neumonía recurrente. Objetivo: Determinar las deficiencias de IgM, IgA y de subclase de IgG2 y la Deficiencia Específica de Anticuerpos (anticuerpos antineumocócicos de polisacáridos) en adultos con Bronquiectasias no Fibrosis Quística (BQnoFQ) o neumonía recurrente. Métodos: Estudio observacional prospectivo. Se reclutaron 110 pacientes consecutivos con BQnoFQ o neumonía recurrente en Cali, Colombia. Se midieron los niveles séricos de IgG, IgA, IgM e IgE, subclase IgG2 y anticuerpos anti-neumococo. Resultados: Se encontraron deficiencias de anticuerpos con niveles normales de IgG en el 10% de los sujetos; Cuatro casos con IgG2 baja, incluido 1 caso de deficiencia de IgG2 + IgA, 1 caso de ataxia-telangiectasia, 3 deficiencias de IgA (IgAD), 2 deficiencias selectiva de IgM (IgMD), 1 síndrome de Hiper-IgE (HIES-AR) y 1 deficiencia específica de anticuerpos. Ocho pacientes fueron diagnosticados con enfermedades relacionadas con la hipogammaglobulinemia IgG. Conclusiones: Las deficiencias predominantemente de anticuerpos con niveles normales de IgG son una etiología importante de BQnoFQ y neumonía recurrente en adultos. Los sujetos con bronquiectasias o neumonía recurrente requieren una evaluación exhaustiva de la respuesta inmune humoral y clínica.
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Background & objectives: Haemoptysis in children is potentially life-threatening. In most cases, the bleeding arises from the systemic circulation, and in 5-10 per cent of cases, it arises from the pulmonary circulation. The role of computed tomography angiography (CTA) in this setting is important. This study was undertaken (i) to study the role of single-phase split-bolus dual energy contrast-enhanced multidetector row CTA (DECTA) in the evaluation of haemoptysis in children; (ii) to analyze the patterns of abnormal vascular supply in the various aetiologies encountered. Methods: A retrospective study of 86 patients who underwent split bolus DECTA for the evaluation of haemoptysis was performed. Final diagnoses were categorized as normal computed tomography, active tuberculosis (TB), post-infectious sequelae, non-TB active infection, cystic fibrosis (CF), non-CF bronchiectasis, congenital heart disease (CHD), interstitial lung disease, vasculitis, pulmonary thromboembolism and idiopathic pulmonary haemosiderosis. Abnormal bronchial arteries (BAs) and non-bronchial systemic collateral arteries (NBSCs) were assessed for number and site and their correlation with underlying aetiologies. Results: A total of 86 patients (45 males, age from 0.3 to 18 yr, mean 13.88 yr) were included in the study; among these only two patients were less than five years of age. The most common cause of haemoptysis was active infection (n=30), followed by bronchiectasis (n=18), post-infectious sequelae (n=17) and CHD (n=7). One hundred and sixty five abnormal arteries were identified (108 BA and 57 NBSC), and were more marked in bronchiectasis group. Interpretation & conclusions: Active infections and bronchiectasis are the most common causes of haemoptysis in children. While post-infectious sequelae are less common, in patients with haemoptysis, the presence of any abnormal arteries correlates with a more frequent diagnosis of bronchiectasis. NBSCs are more common in post-infectious sequelae and CHD
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SUMMARY OBJECTIVE: The objective of this study was to evaluate the quality of life of patients with noncystic fibrosis bronchiectasis during a 1-year follow-up by using the EuroQol - 5 Dimensions - 3 Levels (EQ-5D-3L) questionnaire. METHODS: A cohort study was conducted with 100 patients with noncystic fibrosis bronchiectasis and followed up with face-to-face visits or by telephone contact every 3 months for 1 year. All patients were recruited from a single referral center for bronchiectasis. At the time of recruiting and at the end of 1 year, the EQ-5D-3L questionnaire was applied to evaluate the patients' quality of life. Variables, such as exacerbation, emergency care, comorbidities, hemoptysis, colonization, and hospitalization, were assessed. RESULTS: Of the 100 patients, 99 completed the study and 72% were women. There were no marked limitations in the mobility and self-care domains during the follow-up. At the end of the follow-up, 32 patients were extremely anxious or depressed. The quality of life assessed by using EQ-5D-3L had an initial mean score of 0.545 and of 0.589 after 1 year, which was statistically significant (p=0.011). CONCLUSION: Patients with noncystic fibrosis bronchiectasis have a poor quality of life, and the EQ-5D-3L questionnaire may be a tool for monitoring patients with bronchiectasis.