RESUMEN
BACKGROUND: The single-breath diffusing capacity of the lungs (DLCOSB) test measures the extent to which carbon monoxide (CO) passes from the lung air sacs into the blood. The accessible alveolar volume (VASB) is measured by inert gas during a 10-second period. The single-breath transfer coefficient of the lung for carbon monoxide (KCOSB) is the DLCOSB divided by VASB. Cystic fibrosis (CF) disease comprises progressive airway obstruction with bronchiectasis and parenchyma fibrosis. Yet, the KCOSB appears insignificant in the assessment of pulmonary function in CF. OBJECTIVES: To challenge the precision of normal KCOSB in CF. METHODS: The authors collected pulmonary function tests (PFT) data from 74 confirmed CF patients (mean age 26 ± 10 years) with various levels of pulmonary disease severity. Tests included spirometry, DLCOBP, and body plethysmography (BP). Anatomical dead space was calculated by deducting anatomical dead space from total lung capacity TLC(BP) to establish alveolar volume (VABP) and to determine KCOBP. We also included individual data of arterial pCO2 blood-gas level. RESULTS: KCOSB values were normal or higher in most patients, regardless of patient FEV1 value (R2 = 0.2204; P < 0.02). In contrast, the measurements of KCOBP were low corresponding with low FEV1 values, and negatively correlated with the elevation of trapped air and pCO2 levels (R2 = 0.1383; P = 0.0133, P > 0.05, respectively). CONCLUSIONS: The 10- second perfusion time of the inert gas during DLCOSB represent the communicative alveolar volume in CF patients with advanced pulmonary disease. The findings justify the use of DLCOSB with the deterioration of FEV1 and elevation of pCO2 levels.
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Fibrosis Quística/complicaciones , Enfermedades Pulmonares/etiología , Capacidad de Difusión Pulmonar , Adolescente , Adulto , Niño , Fibrosis Quística/fisiopatología , Femenino , Humanos , Enfermedades Pulmonares/fisiopatología , Mediciones del Volumen Pulmonar , Masculino , Persona de Mediana Edad , Pletismografía Total , Pruebas de Función Respiratoria , Espirometría , Adulto JovenRESUMEN
OBJECTIVES: Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy characterized by chronic lung disease and cyclic vomiting due to hyper-adrenergic crises. Most FD patients are in a depleted nutritional state; however, the phenotype of the disease is quite different between patients, as for the severity of lung disease and the intensity and frequency of these pathognomonic crises. In this study we wanted to investigate whether resting energy expenditure (REE) levels are increased in this population, and if correlations exist between REE levels and phenotype severity. METHODS: Data was collected from 12 FD patients (6/6 m/f). REE measurements were conducted by indirect calorimeter. Measured REE % predicted were correlated with pulmonary function, severity of the scoliosis, serum C-reactive protein, yearly frequency of hyperadrenergic crisis, hospital admissions and the use of nocturnal noninvasive positive pressure ventilation. RESULTS: Mean REE was 112 ±13% predicted with 50% being in a hypermetabolic state (REE/HBâ>â110%). Body mass index (BMI) was below normal range in 75% of patients, and reduced energy intake was also decreased in 75%. No significant correlations to disease severity factors were found. When dividing the subjects to REE levels above or below 125% predicted, Patients with REE above 125% predicted presented with significantly lower inspiratory capacity (42.7% predicted vs 62.8% predicted; P = 0.04). CONCLUSIONS: Hypermetabolic state was described in 50% of FD patients. The Low BMI is explained by combination of relative anorexia and increased REE. The REE levels are related to the underling respiratory disease.
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Disautonomía Familiar/metabolismo , Metabolismo Energético , Adulto , Índice de Masa Corporal , Calorimetría Indirecta , Disautonomía Familiar/fisiopatología , Femenino , Humanos , Pulmón/fisiopatología , Masculino , Estado Nutricional , Estudios Retrospectivos , Grosor de los Pliegues Cutáneos , Delgadez/metabolismoRESUMEN
OBJECTIVES: Ataxia-Telangiectasia (A-T) individuals often present with respiratory muscle weakness, causing recurrent respiratory system infections, asthma-like symptoms, and chronic cough life-threatening events. The cough flow volume maneuver may reveal powerless airflow needed for efficient cough. The study aims to explore cough ability in relation to the flow/volume maneuver. METHODS: Data collected retrospectively from clinical charts of 35 A-T patients (age 12.7 ± 4.9 years) included forced expiratory and cough flow/volume maneuvers performed on the same day. Analysis compared among the maneuvers matching indices, numbers of cough-spikes, flow rate decay, and the reference data of similar ages. Adjusted to age, BMI, and number of hospitalizations prior to the tests, values were correlated with the cough indices. RESULTS: Cough peak-flow (C-PF) was propagated within 90 ± 20 ms compared with peak expiratory flow (PEF > 200 ms). C-PF measured values were higher than expiratory peak-flow measured values (3.27 ± 1.53 L/s versus 3.02 ± 1.52 L/s, respectively, but C-PF (%predicted) values were significantly lower than expiratory peak-flow (%predicted) (46 ± 15 versus 68 ± 20 %predicted, respectively, p < 0.002). The number of spikes/maneuver was low when compared with reference (2.0 ± 0.8 versus 6-12 spikes) and cough vital-capacity was lower than expiratory vital capacity (0.95 ± 0.43 versus 1.03 ± 0.47; p < 0.01). Inefficient C-PF was more prevalent in patients suffering from recurrent respiratory illness. The length of wheelchair confinement duration mostly influenced the C-VC level. CONCLUSIONS: The cough flow-volume curve can be applied as a method to follow cough ability in patients with A-T who showed a significantly reduced cough capacity. Further studies are needed to establish if the findings may aid decisions regarding cough assistance.
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Ataxia Telangiectasia/fisiopatología , Tos/fisiopatología , Adolescente , Niño , Femenino , Volumen Espiratorio Forzado , Humanos , Masculino , Ápice del Flujo Espiratorio , Capacidad VitalRESUMEN
BACKGROUND: Many preschool children will perform correct peak-flow but will not exhale to residual volume, thus limiting the determination of airways obstruction. The maximal flow measured at function residual capacity (V'maxFRC) is independent of lung empting and could potentially serve as a parameter for describing flow at low lung volumes. The study determines the detection of airway obstruction/dilation in asthmatic preschool children by V'maxFRC, compared to FEV1 and FEF25-75. METHODS: Children performed bronchial provocation test (BPT; n = 26) or received bronchodilators (Post-BD; n = 31). V'maxFRC was extracted at inspiratory capacity point of flow/volume maneuvers. The %change of V'maxFRC from baseline was compared with changes in various spirometry indices and to values obtained from our previously studied healthy control children. RESULTS: FEV1, FEF25-75, and V'maxFRC decreased by 30.9 ± 12.2%, 46.2 ± 10.9%, and 36.6 ± 8.0%, respectively, while FRC increased by 37.0 ± 24.9% at end of the BPT. Post-BD spirometry values increased by 17.1 ± 16.1%, 47.0 ± 42.2, and 45 ± 24%, respectively (p < 0.0001). A positive response to bronchodilators was observed in 15/31 (48%) children by FEV1, in 22/31 (71%) children by V'maxFRC, and in 21/31 children by FEF25-75. CONCLUSION: V'maxFRC detects airway obstruction/dilation in young asthmatic children similar to FEF25-75 and FEV1. V'maxFRC may be a valuable index in preschool children who cease exhalation prematurely. Digitally measured V'maxFRC should confirm the actual values in a wider age range in healthy and disease states.
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Obstrucción de las Vías Aéreas/diagnóstico , Asma/diagnóstico , Asma/tratamiento farmacológico , Pruebas de Provocación Bronquial , Broncodilatadores/farmacología , Niño , Preescolar , Femenino , Capacidad Residual Funcional , Humanos , Masculino , Pruebas de Función RespiratoriaRESUMEN
BACKGROUND: Cystic fibrosis (CF) patients are predisposed to infection and colonization with different microbes. Some cause deterioration of lung functions, while others are colonizers without clear pathogenic effects. Our aim was to understand the effects of Nocardia species in sputum cultures on the course of lung disease in CF patients. MATERIAL AND METHODS: A retrospective study analyzing the impact of positive Nocardia spp. in sputum of 19 CF patients over a period of 10 years, comparing them with similar status patients without Nocardia growth. Pulmonary function tests (PFTs) are used as indicators of lung disease severity and decline rate in functions per year is calculated. RESULTS: No significant difference in PFTs of CF patients with positive Nocardia in sputum was found in different sub-groups according to number of episodes of growth, background variables, or treatment plans. The yearly decline in PFTs was similar to that recognized in CF patients. The control group patients showed similar background data. However, a small difference was found in the rate of decline of their PFTs, which implies a possibly slower rate of progression of lung disease. CONCLUSIONS: The prognosis of lung disease in CF patients colonized with Nocardia does not seem to differ based on the persistence of growth on cultures, different treatment plans or risk factors. Apparently, Nocardia does not cause a deterioration of lung functions with time. However, it may show a trend to faster decline in PFTs compared to similar status CF patients without isolation of this microorganism in their sputum.
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Fibrosis Quística/fisiopatología , Nocardia/aislamiento & purificación , Pruebas de Función Respiratoria , Adolescente , Adulto , Estudios de Casos y Controles , Niño , Fibrosis Quística/microbiología , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Nocardia/crecimiento & desarrollo , Factores de Riesgo , Esputo/microbiología , Adulto JovenRESUMEN
RATIONALE: Airways obstruction and lung volume restriction, major features of lung disease in cystic fibrosis (CF), may regress independently, causing dysanapsis between these parameters. OBJECTIVES: To explore the significance of dysanapsis (FEF(25-75)/FVC) ratio in CF. METHODS: Yearly best spirometry data, collected during 8.6 ± 1 year per patient, was determined from 93 patients with CF. Three groups were formed according to initial FEV(1). Group-N (n = 35; control, FEV(1) above 80%predicted); Group-B (n = 38; FEV(1) below 80% predicted); and Group-LT (n = 20; data collected before lung transplantation). The yearly decline in spirometry indices was defined in relation to the preceding year. Decline exceeding -2 z scores from Group-N in each index was considered "rapid decline." MEASUREMENTS AND MAIN RESULTS: Group-N's yearly decline of FEV(1), FEF(25-75), and FEF(25-75)/FVC were similar and reached -1.88 ± 2.93%, -1.41 ± 3.37%, and -1.81 ± 4.48%, respectively. Rapid decline was equal to -6.5%, -10.8%, and -8.1%, respectively. Group-B's indices declined faster than that of Group-N, but did not exceed 1 z score. Group-LT showed a rapid decline solely in FEF(25-75)/FVC (mean z score = -6.4 ± 2.5; P < 0.0001), which sprouted abruptly from the regular course of regression 4 ± 1.3 years before transplantation. The rapid decline in FEF(25-75)/FVC was found in 19 of 20 patients from Group-LT and five patients from other groups (now waiting for transplantation). The phenomenon did not correlate with initial FEV(1) (%predicted) or age. Having airway hyperreactivity increased the risk of rapid decline in FEF(25-75)/FVC. CONCLUSIONS: Roughly 4 years before lung transplantation and independent of FEV(1) (%predicted), FEV(1) decline rate, or age, an abrupt rapid dysanapsis occurs. Findings imply insufficient ventilation/lung volume unit and insinuate a powerful marker for estimating lung translation time in CF.
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Obstrucción de las Vías Aéreas/etiología , Fibrosis Quística/complicaciones , Fibrosis Quística/cirugía , Adolescente , Obstrucción de las Vías Aéreas/fisiopatología , Niño , Comorbilidad , Fibrosis Quística/epidemiología , Fibrosis Quística/fisiopatología , Femenino , Volumen Espiratorio Forzado , Humanos , Trasplante de Pulmón , Masculino , Pruebas de Función Respiratoria , Estudios Retrospectivos , Espirometría , Adulto JovenRESUMEN
AIM: Bronchial hyper-reactivity (BHR) provides a tool for asthma diagnosis, assessment of severity and response to treatment. The effect of beclomethasone dipropionate in ultrafine particles (BDP-HFA) on BHR as measured by the adenosine challenge test in young children has not yet been determined. Our aim was to determine the effect of BDP-HFA (100 µg twice daily) on BHR as evaluated by a reduction of 20% from baseline FEV1 (PC20-FEV1) values in young asthmatic children. METHODS: Twenty-one young children (13 males), mean age 4.95 ± 1.05 years, with partially controlled or controlled asthma completed a double-blind randomized, placebo-controlled, cross-over study. Each child received 4 weeks of treatment with either 100 µg BDP-HFA twice daily or placebo, and after a 2-week washout period the other way around. Primary outcomes were PC20-FEV1 concentration, and the stage number at which FEV1 values dropped by 20%. RESULTS: Following 4 weeks of treatment, median PC20-FEV1 was 81.28 mg/mL while on BDP-HFA, compared with 9.64 mg/mL on placebo (p < 0.001). The median increase in stages required to achieve PC20 on BDP-HFA compared with placebo was three (95% CI 2.28-4.86). CONCLUSION: Four weeks of treatment with BDP-HFA resulted in significantly decreased BHR in young children.
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Antiasmáticos/uso terapéutico , Beclometasona/uso terapéutico , Hiperreactividad Bronquial/tratamiento farmacológico , Adenosina Monofosfato , Antiasmáticos/administración & dosificación , Beclometasona/administración & dosificación , Hiperreactividad Bronquial/diagnóstico , Preescolar , Estudios Cruzados , Método Doble Ciego , Femenino , Humanos , Masculino , Tamaño de la PartículaRESUMEN
BACKGROUND: Exercise ventilatory limitation conventionally defined by reduced breathing reserve (BR) may underestimate the effect of lung disease on exercise capacity in patients with mild to moderate obstructive lung diseases. OBJECTIVE: To investigate whether ventilatory limitation may be present despite a normal BR in Cystic Fibrosis (CF). METHODS: Twenty adult CF patients (age 16-58y) with a wide range of pulmonary obstruction severity completed a symptom-limited incremental exercise test on a cycle ergometer. Operating lung volumes were derived from inspiratory capacity (IC) measurement during exercise and exercise tidal flow volume loop analysis. RESULTS: six patients had a severe airway obstruction (FEV1<45% predicted) and conventional evidence of ventilatory limitation (low BR). Fourteen patients had mild to moderate-severe airway obstructive (FEV1 46-103% predicted), and a normal BR [12-62 L/min, BR% (17-40)]. However, dynamic respiratory mechanics demonstrated that even CF patients with mild to moderate-severe lung disease had clear evidence of ventilatory limitation during exercise. IC was decreased by (median) 580 ml (range 90-1180 ml) during exercise, indicating dynamic hyperinflation. Inspiratory reserve volume at peak exercise was 445 ml (241-1350 ml) indicating mechanical constraint on the respiratory system. The exercise tidal flow met or exceeded the expiratory boundary of the maximal flow volume loop over 72% of the expiratory volume (range 40-90%), indicating expiratory flow limitation. CONCLUSION: Reduced BR as a sole criterion underestimates ventilatory limitation during exercise in mild to moderate-severe CF patients. Assessment of dynamic respiratory mechanics during exercise revealed ventilatory limitation, present even in patients with mild obstruction.
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Fibrosis Quística , Adolescente , Adulto , Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico , Ejercicio Físico , Prueba de Esfuerzo , Humanos , Capacidad Inspiratoria , Mediciones del Volumen Pulmonar , Persona de Mediana Edad , Adulto JovenRESUMEN
OBJECTIVE: To define the neurologic characteristics and course of ataxia-telangiectasia (A-T). STUDY DESIGN: Retrospective cross-sectional chart study of 57 children (ages 2 to 19 years) followed at an A-T clinic. Cerebellar and extracerebellar symptoms were graded according to degree of functional impairment. Head circumferences were plotted from the charts and z-scores were calculated and compared with that of family members. RESULTS: Ataxia was present in 87.7%, followed by dysarthria (82.1%), dysmetria (75.4%), bradykinesia (69.2%), hyperkinetic movements (58.9%), and dystonia (15.8%). All features aggravated with age. The most striking clinical observation in our patients was low head circumference (z-score below 1), which was present in 60.9%; 17% had true microcephaly (z-score below 2). Microcephaly appeared postnatally, was proportionate to height and weight, and did not correlate with severity of ataxia or genotype. CONCLUSIONS: In addition to cerebellar ataxia, extrapyramidal symptoms, especially bradykinesia, were frequent and disabling. Microcephaly is an integral part of A-T; understanding its pathogenesis may shed light on the mechanism by which ATM mutation causes dysfunction in the nervous system.
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Ataxia Telangiectasia/epidemiología , Cefalometría , Microcefalia/epidemiología , Adolescente , Envejecimiento , Ataxia Telangiectasia/genética , Niño , Preescolar , Estudios Transversales , Disartria/epidemiología , Disartria/etiología , Discinesias/epidemiología , Discinesias/etiología , Femenino , Humanos , Masculino , Mutación , Trastornos de la Motilidad Ocular/epidemiología , Trastornos de la Motilidad Ocular/etiología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
BACKGROUND: Treatment of respiratory exacerbations in Cystic Fibrosis (CF) is important in slowing disease progression. The treatment may be given either at home or at the hospital. The aim of our study was to compare both treatment settings. MATERIAL/METHODS: We retrospectively analyzed data of 139 treatments in 54 CF patients (age range 12-47 y) treated for respiratory exacerbations at the hospital (n = 84) and/or at home (n = 55). Primary outcomes were improvement in pulmonary function tests (PFTs), weight gain and duration of treatment in relation to treatment setting. Secondary outcomes were these same parameters, but in relation to different clinical preconditions and CF-related complications. RESULTS: Mean improvement in FEV1 (% predicted) was similar between the hospital and home treatments (14.3 ± 34.4% vs. 14.3 ± 20.2%, respectively; NS), yet treatment duration was significantly shorter at the hospital (9.7 ± 6.7 vs. 16.3 ± 9.1 days, respectively; P < 0.02), especially for patients colonized with Pseudomonas aeruginosa (11.1 ± 5.5 vs. 18.0 ± 11.0 days, respectively; p<0.01). At the hospital, a subgroup of patients with CF-related complications improved their FEV1 significantly more than those at home (13.1 ± 19.4% vs. 1.9 ± 14.9%, respectively; P < 0.02), particularly patients with CF-related diabetes mellitus (CFRDM) (11.4 ± 18.7% vs. 1.7 ± 14.6%, respectively; P < 0.05). Patients tended to gain more weight at the hospital compared to home treatment (1.36 ± 4.6 kg and 0.49 ± 3.6 kg respectively; P = 0.06). CONCLUSIONS: Hospital treatment for acute respiratory exacerbations in CF may be superior to home treatment, as indicated by a shorter duration of hospitalization, better improvement in FEV1 in patients with CF-related complications, CFRDM in particular and a trend toward better weight gain.
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Fibrosis Quística/patología , Fibrosis Quística/terapia , Progresión de la Enfermedad , Hospitalización , Adolescente , Adulto , Antropometría , Niño , Recuento de Colonia Microbiana , Fibrosis Quística/microbiología , Fibrosis Quística/fisiopatología , Femenino , Volumen Espiratorio Forzado , Humanos , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Pseudomonas aeruginosa/aislamiento & purificación , Espirometría , Adulto JovenRESUMEN
BACKGROUND: Pneumothorax can be classified as traumatic, iatrogenic or spontaneous (SP), which can be subdivided into primary spontaneous pneumothorax (PSP), a condition without preexisting lung disease, or secondary spontaneous pneumothorax (SSP) a complication of a preexisting lung disease. Recurrence rate of PSP is 30% whereas for SSP rate is unknown. This article explores the experience of a tertiary center over 20 years. METHODS: A retrospective case review of patients hospitalized with pneumothorax to investigate the natural history and treatment of SP in a young population in a single tertiary center was conducted. A search of the digital archive (going back to 01/01/1995) of Sheba Medical Center identified hospitalized patients below the age of 40. RESULTS: The database was composed of the records of 750 patients (612 males, 138 females) who were hospitalized. The recurrence risk for SP after nonoperative treatment was significantly higher. Women were found to have an increased risk of SSP when having SP (OR 2.78). Asthma was the most prevalent disease causing SSP in young people. CONCLUSIONS: In this large cohort, we found that operative procedure has clear protective effect from recurrence in SP, so surgery should be positively considered when treating SP in hospitalized patients. Among young people and particularly in pediatric patients, when females have a SP, we strongly recommend looking for primary lung disease. More studies are needed to determine the risk factors and produce clear guidelines regarding surgery as first treatment.
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Enfermedades Pulmonares , Neumotórax , Adolescente , Niño , Femenino , Humanos , Masculino , Neumotórax/epidemiología , Neumotórax/etiología , Neumotórax/terapia , Recurrencia , Estudios Retrospectivos , Factores de RiesgoRESUMEN
OBJECTIVES: Patients with cystic fibrosis (CF) presenting with meconium ileus (MI) tend to have worse outcomes than those without MI. We evaluated the clinical characteristics and survival rates among Israeli patients with CF with and without MI after a prolonged follow-up (15-30 years). PATIENTS AND METHODS: A multicenter retrospective study. Forty-nine patients with CF, representing 13.8% of all patients with CF in Israel, presented with MI (current age 17.4 +/- 7.9 years) between 1975 and 2006. They were compared with 38 patients with CF (current age 19.3 +/- 6.5 years) without MI matched by sex and CF transmembrane conductance regulator mutation. RESULTS: A total of 66.2% of patients with MI and 73.6% without MI were followed for a prolonged period (24.9 +/- 2.7 years). Of the patients with MI, 31 were managed operatively, whereas 18 were treated successfully with gastrograffin enema, with similar clinical outcomes. Five patients in the MI group and 3 in the control group died during the study period. Bacterial colonization, z score of body mass index, and pulmonary function tests were similar in patients with and without MI in the long term. In younger patients, many clinical parameters were more prevalent in patients with MI (P = 0.004). However, these differences disappeared after the long-term follow-up (up to 31-years). CONCLUSIONS: Patients with CF presenting with MI had similar pulmonary function and nutritional status, as well as survival rates as did the control patients without MI. The distinct genetic mutation found in our population may explain in part the favorable results compared with other studies. In addition, it seems that early diagnosis and treatment of MI in patients with CF may be beneficial, subsequently lowering morbidity, and increasing survival.
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Fibrosis Quística/complicaciones , Ileus/complicaciones , Meconio , Adolescente , Adulto , Factores de Edad , Índice de Masa Corporal , Niño , Fibrosis Quística/mortalidad , Fibrosis Quística/terapia , Diatrizoato de Meglumina/uso terapéutico , Progresión de la Enfermedad , Enema , Femenino , Humanos , Ileus/terapia , Lactante , Israel , Pulmón , Masculino , Pruebas de Función Respiratoria , Estudios Retrospectivos , Factores de Riesgo , Análisis de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Treatment of hemato-oncologic patients is often associated with severe pulmonary complications. Pulmonary function is routinely evaluated in older children, whereas in young patients, forced spirometry measurements are infrequently performed. AIM: To assess severity of airway disease using forced spirometry measured prior to and after treatment (over 3 years) in hemato-oncologic children aged 3-7 years in comparison to a healthy population. MATERIAL/METHODS: 42-children (18-males; age 3-7 years old) with hemato-oncologic illnesses participated in the study. Spirometry was performed before the definitive treatment and up-to 3-years thereafter. Values were compared to those of healthy children of corresponding age. RESULTS: Most children (n=38) showed only minor long term airflow impairment (z-scores of FEV1 at last measurement was -0.00 to -0.45 SD). Prior to definitive treatment eight children presented severe airflow limitation (z-score =-1.35 + or - 0.72; -1.61 + or - 0.66 and -2.49 + or - 0.34 for FEV1, FEV0.5; and FEF25-75 respectively). Four of eight children resumed normal pulmonary function; the spirometry values of the other four children further deteriorated, in association with GVHD and development of bronchiolitis obliterans. CONCLUSIONS: Our study suggests that it is important to follow spirometry in young children with hemato-oncologic diseases in order to detect these patients, whose condition may have prognostic implications for their treatment.
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Neoplasias Hematológicas/fisiopatología , Espirometría/métodos , Obstrucción de las Vías Aéreas/complicaciones , Obstrucción de las Vías Aéreas/epidemiología , Obstrucción de las Vías Aéreas/fisiopatología , Estudios de Casos y Controles , Niño , Femenino , Estudios de Seguimiento , Neoplasias Hematológicas/complicaciones , Humanos , Israel/epidemiología , Masculino , Prevalencia , Capacidad Vital/fisiologíaRESUMEN
RATIONALE: Advances in spirometry measurement techniques have made it possible to obtain measurements in children as young as 3 years of age; however, in practice, application remains limited by the lack of appropriate reference data for young children, which are often based on limited population-specific samples. OBJECTIVES: We aimed to build on previous models by collating existing reference data in young children (aged 3-7 yr), to produce updated prediction equations that span the preschool years and that are also linked to established reference equations for older children and adults. METHODS: The Asthma UK Collaborative Initiative was established to collate lung function data from healthy young children aged 3 to 7 years. Collaborators included researchers with access to pulmonary function test data in healthy preschool children. Spirometry centiles were created using the LMS (lambda, micro, sigma) method and extend previously published equations down to 3 years of age. MEASUREMENTS AND MAIN RESULTS: The Asthma UK centile charts for spirometry are based on the largest sample of healthy young Caucasian children aged 3-7 years (n = 3,777) from 15 centers across 11 countries and provide a continuous reference with a smooth transition into adolescence and adulthood. These equations improve existing pediatric equations by considering the between-subject variability to define a more appropriate age-dependent lower limit of normal. The collated data set reflects a variety of equipment, measurement protocols, and population characteristics and may be generalizable across different populations. CONCLUSIONS: We present prediction equations for spirometry for preschool children and provide a foundation that will facilitate continued updating.
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Asma/diagnóstico , Asma/fisiopatología , Espirometría/normas , Niño , Preescolar , Volumen Espiratorio Forzado , Humanos , Valores de Referencia , Pruebas de Función Respiratoria , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Reino Unido , Población BlancaRESUMEN
BACKGROUND: Autogenic drainage (AD) is used for airway clearance in individuals with cystic fibrosis (CF). The mechanical insufflator-exsufflator (MI-E) device is commonly used in patients with neuromuscular diseases for airway clearance by increasing inspiratory volume and expiratory flow. MI-E use has not been studied extensively in CF patients. OBJECTIVES: To examine whether there are advantages to using the MI-E device in patients with CF. SUBJECTS: Fourteen males and eight females from the national center for CF, with an average FEV1 of 54% were recruited DESIGN: Prospective cross-over trial (IRB 3009-16-SMC). INTERVENTION: Subjects received either AD or MI-E in a random order. Sputum was collected and weighed immediately after treatment. Subjects performed lung function tests at baseline, 20 minutes after and 1 hour after treatment; additionally, a 2-minute walk test was performed at the end of all lung function tests. Saturation, dyspnea scores while resting and after a 2-minute walk and subjective fatigue were recorded. RESULTS: Thirty-six percent more sputum was collected following MI-E than AD treatment (P < .0001). A significant difference in saturation in response to the 2MWT was noted in both treatments significantly less desaturation was recorded after the 2MWT in the MI-E treatment (P < .01). CONCLUSIONS: Treatment with the MI-E was more effective for clearing sputum in CF subjects, initial evidence suggests that the MI-E may be successfully incorporated into treatment protocols. Further studies are needed to assess the long-term benefits of MI-E in patients with CF.
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Fibrosis Quística/terapia , Drenaje/métodos , Respiración Artificial/métodos , Adulto , Estudios Cruzados , Fibrosis Quística/fisiopatología , Espiración , Femenino , Humanos , Masculino , Estudios Prospectivos , Pruebas de Función Respiratoria , Esputo , Adulto JovenRESUMEN
OBJECTIVE: The use of flexible bronchoscopy (FOB) and bronchoalveolar lavage (BAL) in investigating pediatric patient with airway abnormalities and pulmonary infiltrates are indispensable and are now a routine procedure in many centers. Immunocompromised and cancer patients, especially after bone marrow transplantation, and children who have undergone surgery for congenital heart disease (CHD) are at high risk for pulmonary disease. Our aim was to study the diagnostic rate, safety, and clinical yield of FOB in critically ill pediatric patients. DESIGN: : Retrospective chart review. SETTING: Pediatric intensive care unit in a tertiary university hospital. PATIENTS: Three hundred nineteen children who underwent 335 FOB procedures. The indications for bronchoscopy included infectious agent identification in immune-competent patients with new pulmonary infiltrates seen on chest radiograph (46%) and in patients with fever and neutropenia with respiratory symptoms (18%), airway anatomy evaluation in patients with upper airway obstruction (16%), CHD (15%), and airway trauma (5%). Data were obtained by reviewing the patients' charts, bronchoscopy reports, and laboratory results. MEASUREMENTS AND MAIN RESULTS: The diagnostic rate of FOB procedures was 79%. FOB and BAL resulted in alteration of management (positive clinical yield) in 70 patients (23.9%). A definite infectious organism was identified in 56 patients (17.6%). The clinical yield in patients with cancer or primary immune deficiency (38.7%) was significantly higher compared with patients with CHD (20.4%, p < 0.01) and pneumonia (17%, p < 0.01). Major complications were observed in two procedures (prolonged apnea), and minor complications (transient desaturation, stridor, and minor bleeding) were observed in 45 patients (14%). CONCLUSIONS: FOB and BAL have an important role in the evaluation of airway abnormality and pulmonary infiltrate in pediatric patients, in whom rapid and accurate diagnosis is crucial for survival. We suggest that FOB should be considered as an initial diagnostic tool in those critically ill patients.
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Lavado Broncoalveolar/métodos , Broncoscopios , Huésped Inmunocomprometido , Enfermedades Pulmonares/diagnóstico , Distribución de Chi-Cuadrado , Niño , Preescolar , Estudios de Cohortes , Cuidados Críticos/métodos , Enfermedad Crítica , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Humanos , Unidades de Cuidado Intensivo Pediátrico , Enfermedades Pulmonares/inmunología , Enfermedades Pulmonares/terapia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Masculino , Docilidad , Probabilidad , Respiración Artificial/métodos , Estudios Retrospectivos , Medición de Riesgo , Sensibilidad y Especificidad , Traumatismos Torácicos/diagnóstico , Traumatismos Torácicos/terapiaRESUMEN
OBJECTIVE: To identify the characteristics of CF patients with hemoptysis in Israel and to compare clinical features and risk factors to a control group of CF patients without hemoptysis. DESIGN: Retrospective chart review. PATIENTS: All CF patients in Israel who experienced hemoptysis between 2001 and 2005 and a control group of sex- and age-matched patients with no history of hemoptysis. RESULTS: 40/440 CF patients (9.1%) experienced hemoptysis during the study period, 50% were male. Ten patients (25%) were under 13 years old at the first hemoptysis episode. Pulmonary exacerbation was the precipitating factor in 90%. Twenty three patients showed moderate or major hemoptysis. 35/40 patients responded well to conservative therapy. Bronchial artery embolization (BAE) was performed in 5 patients with no recurrence of bleed within 24 h. However all of these patients experienced recurrent hemoptysis. One patient died during the follow-up period because of end stage lung disease. Pulmonary function tests, body-mass index, coagulation tests, pancreatic status, presence of bronchiectasis, sputum cultures and genetic mutations were similar in the two groups. A high incidence (57.5%) of associated diseases including cystic fibrosis related diabetes, cirrhosis and portal hypertension, and distal intestinal obstruction syndrome was found among hemoptysis patients, compared to only 5.2% in the control group (p<0.001). CONCLUSIONS: Hemoptysis, even major, did not seem to be a risk factor for mortality in our patients. A higher incidence of hemoptysis was found in our pediatric patients compared to other series. BAE shows a high immediate rate of success in controlling hemoptysis, but does not prevent future disease.
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Fibrosis Quística/epidemiología , Hemoptisis/epidemiología , Sistema de Registros , Adolescente , Adulto , Niño , Fibrosis Quística/complicaciones , Femenino , Hemoptisis/complicaciones , Hemoptisis/etiología , Humanos , Hipertensión Portal/complicaciones , Israel/epidemiología , Masculino , Prevalencia , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Management of pediatric patients with malignant and hematological diseases is frequently associated with pulmonary complications. We assessed pulmonary function at diagnosis and during a 5-year follow-up to identify risk factors associated with pulmonary deterioration. PROCEDURE: Ninety patients (age range 3-20) who were treated at the Pediatric Hematology-Oncology Department, Sheba Medical Center, Israel, were entered into the study. Pulmonary function testing was performed at diagnosis and at least twice during the study period. RESULTS: At diagnosis and thereafter values of spirometry, total lung capacity, functional residual capacity and diffusion capacity were significantly lower than predicted (P < 0.002 for all indices). The ratio between residual volume and total lung capacity (RV/TLC) was significantly higher than normal at diagnosis and throughout the study (P < 0.001). Age and treatment modalities did not show any effect on lung-function during the study. A subgroup of seven patients (8%) developed Bronchiolitis obliterans (BO) after stem cell transplantation and development of graft versus host disease (GVHD). These patients' baseline FEF25-75 values (small airway disease) were significantly lower than FEF25-75 values of controls and other patients while all other parameters were similar. The RV/TLC in the BO patients gradually increased relative to other patients during the 5-year follow-up. CONCLUSION: Lung-function in pediatric hemato-oncological patients at diagnosis is lower than predicted. Abnormal baseline FEF25-75 may be a risk factor for the development of BO in the setting of GVHD after treatment. Careful monitoring, especially of FEF25-75 and RV/TLC at baseline and in the first period after diagnosis.
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Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Pulmón/efectos de los fármacos , Pulmón/efectos de la radiación , Neoplasias/terapia , Radioterapia/efectos adversos , Trasplante de Células Madre/efectos adversos , Adolescente , Adulto , Bronquiolitis Obliterante/etiología , Niño , Preescolar , Terapia Combinada , Femenino , Enfermedad Injerto contra Huésped/etiología , Humanos , Masculino , Pruebas de Función RespiratoriaRESUMEN
BACKGROUND: Inspiratory capacity (IC) is often overlooked as an important measured index of spirometry in patients with cystic fibrosis (CF). Abnormally low IC may indicate the onset of static/dynamic hyperinflation, which may be accompanied by dyspnea and an increase in the work of breathing. This cross-sectional study sought to determine whether measuring IC during spirometry, may add clinical value to FEV1 measurements in CF subjects. METHODS: Anthropometric, clinical, spirometry, and static lung volume data were gathered retrospectively from 98 of 165 subjects with CF (mean ± SD age 26.8 ± 11.0 y) registered in The Edmond and Lily Safra Children's Hospital, Sheba Medical Centre, Israel. We compared the IC (% predicted) to FEV1, static lung volumes, and hospitalization days/year. RESULTS: IC decreased alongside FEV1 decline but at a slower pace (r2 = 0.32). Incremental trapped air, as measured by residual volume (RV), and a rapid elevation in the ratio of RV to total lung capacity occurred when IC deteriorated below 60% predicted values. The unique combination of IC < 50% predicted and FEV1 > 40% predicted induced an increase of up to 125 hospitalization days/year compared to subjects having IC > 50% predicted (up to 73 d/y, P < .001). CONCLUSIONS: Measuring IC in CF subjects may reveal silent worsening of lung function as indicated by a decline in IC < 50% predicted while FEV1 is still > 40% predicted. This condition may lead to inefficient breathing at high lung volumes, which may explain a subjective sensation of breathlessness and lead to an increase in hospitalization days/year.