[Forced spirometry in healthy preschool children]. / Espirometría forzada en preescolares sanos bajo las recomendaciones de la ATS/ERS: estudio CANDELA.

INTRODUCTION: Reference values for spirometry in healthy preschool children have not yet been obtained in accordance with American Thoracic Society (ATS) and the European Respiratory Society (ERS) guidelines. The objective was to establish reference values for spirometry in healthy preschoolers under the ATS/ERS 2007 statement. MATERIAL AND METHOD: Children of at least 2 and under 7 years of age were tested in 9 pediatric pulmonary function laboratories. The technicians were trained to apply a standardized protocol to perform spirometry. RESULTS: Valid spirometry results were obtained in 455 (81.54%) out of 558 children: 242 boys (53.2%) and 213 girls (46.8%). Ages were as follows: 31 at least 2 and under 3 years old; 96, at least 3 and under 4; 108, at least 4 and under 5; 122, at least 5 and under 6 years, and 98, at least 6 and under 7 years. Formulas were used to calculate the reference values for all the spirometry variables in preschoolers. CONCLUSIONS: Spirometry is feasible in the majority of preschool children under the new guidelines. The availability of the reference values presented is an important step, both for the care of preschoolers and for further research on pulmonary function.

Development and validation of a questionnaire to assess asthma control in pediatrics.

OBJECTIVE: To develop and validate a questionnaire to assess asthma control in children (CAN). DESIGN: Two versions of the CAN (for carers and children) were developed. Both versions were validated in an observational, prospective, multicenter study performed in 38 hospital outpatient clinics throughout Spain. Four hundred fifteen patients and their carers agreed to participate. Of these, 414 patients under 14 years old with frequent episodic or persistent (moderate to severe) asthma completed the questionnaire on 3 occasions (baseline, week 2, and week 12). For patients aged 2-8 the questionnaire was only completed by the carers, but for patients aged 9-14 the questionnaire was completed by the carers and the children. Clinician ratings of asthma control were used as a gold standard to assess the sensitivity, specificity, PPV and NPV of the new measure. RESULTS: Evaluable responses were obtained from 215 carers for children aged 2-8 years and 199 children aged 9-14 years, and their parents. Using a questionnaire total score cut-off of 8 the patient version had a sensitivity of 76.3% and a specificity of 62.9%. For carer version these values were 73% and 69.7%, respectively. A cut point of 8 was selected to maximize the screening accuracy of the CAN questionnaire. Effect sizes in patients with clinician-rated improvements in asthma control were 0.33 and 0.57 for the carer and child versions, respectively. CONCLUSIONS: The screening accuracy and validity of the CAN questionnaire make it suitable for use in research and clinical practice. The sensitivity and specificity were close to 70%, which is acceptable for the study objective: obtain a tool to measure the level of asthma control.

Upregulation of COX-1 and COX-2 in nasal polyps in cystic fibrosis.

BACKGROUND: Since abnormalities in prostanoid metabolism occur in the lower airway of patients with cystic fibrosis (CF), it is likely that they could also be detected in the nose. METHODS: The degree of mRNA and protein expression of cyclo-oxygenase (COX) enzymes 1 (COX-1) and 2 (COX-2) was examined using quantitative reverse competitive polymerase chain reaction (RT-PCR) and Western blot analysis in the nasal polyps from 10 patients with CF, nasal polyps from 10 non-CF patients and 11 nasal mucosa specimens. The results are presented as 10(6) cDNA molecules/mug total RNA and the densitometric ratio between protein and beta-actin. RESULTS: COX-1 mRNA levels were significantly higher in CF nasal polyps (median 2.34, 25-75th percentiles 1.6-3.2) than in the nasal mucosa (0.78, 0.11-1.21), while there was no difference with non-CF nasal polyps (1.11, 0.80-3.15). COX-1 protein levels were significantly higher in CF nasal polyps (3.63, 2.71-4.27) than in nasal mucosa (1.55, 0.66-2.33) and non-CF nasal polyps (2.19, 1.72-3.68). COX-2 mRNA was significantly higher in CF nasal polyps (3.34, 2.42-7.05) than in nasal mucosa (1.69, 0.19-3.50). No differences were found in COX-2 mRNA expression between CF and non-CF polyps (1.38, 0.12-6.07). COX-2 protein levels were also significantly higher in CF nasal polyps (0.23, 0.04-0.34) than in non-CF nasal polyps (0.011, 0.009-0.016) or nasal mucosa (0.014, 0.014-0.016). CONCLUSIONS: Upregulation in the expression of COX-1 and COX-2 could explain the high production of prostanoids reported in CF. These findings raise questions regarding the potential use of selective or non-selective COX-2 non-steroidal anti-inflammatory treatment in CF.

Antimicrobial therapy for pulmonary pathogenic colonisation and infection by Pseudomonas aeruginosa in cystic fibrosis patients.

Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosis patients. The organism can only be eradicated in the early stage of colonisation, while reduction of bacterial density is desirable during chronic colonisation or exacerbations. Monthly, or at least 3-monthly, microbiological culture is advisable for patients without previous evidence of P. aeruginosa colonisation. Cultures should be performed at least every 2-3 months in patients with well-established colonisation, and always during exacerbations or hospitalisations. Treatment of patients following the first isolation of P. aeruginosa, but with no clinical signs of colonisation, should be with oral ciprofloxacin (15-20 mg/kg twice-daily for 3-4 weeks) plus inhaled tobramycin or colistin (intravenous treatment with or without inhaled treatment can be used as an alternative), while patients with acute infection should be treated for 14-21 days with high doses of two intravenous antimicrobial agents, with or without an inhaled treatment during or at the end of the intravenous treatment. Maintenance treatment after development of chronic P. aeruginosa infection/colonisation (pathogenic colonisation) in stable patients (aged>6 years) should be with inhaled tobramycin (300 mg twice-daily) in 28-day cycles (on-off) or, as an alternative, colistin (1-3 million units twice-daily). Colistin is also a possible choice for patients aged<6 years. Treatment can be completed with oral ciprofloxacin (3-4 weeks every 3-4 months) for patients with mild pulmonary symptoms, or intravenously (every 3-4 months) for those with severe symptoms or isolates with ciprofloxacin resistance. Moderate and serious exacerbations can be treated with intravenous ceftazidime (50-70 mg/kg three-times-daily) or cefepime (50 mg/kg three-times-daily) plus tobramycin (5-10 mg/kg every 24 h) or amikacin (20-30 mg/kg every 24 h) for 2-3 weeks. Oral ciprofloxacin is recommended for patients with mild pulmonary disease. If multiresistant P. aeruginosa is isolated, antimicrobial agents that retain activity are recommended and epidemiological control measures should be established.

Tratamiento antimicrobiano frente a la colonización pulmonar por Pseudomonas aeruginosa en el paciente con fibrosis quística / No disponible

No disponible

[Comparative efficacy of oral deflazacort versus oral prednisolone in children with moderate acute asthma]. / Estudio comparativo de la eficacia del deflazacort frente a prednisolona en el tratamiento de la crisis asmática moderada.

OBJECTIVES: To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute moderate asthma in children. PATIENTS AND METHODS: We performed a prospective, randomized, parallel group trial of children aged 6 to 14 years old with a diagnosis of asthma who presented to the pediatric emergency department for moderate asthma exacerbation. All patients were administered short-acting beta2-adrenergic agonists. The intervention groups received either oral deflazacort (1.5 mg/kg) or prednisolone (1 mg/kg) for 7 days. The primary outcome measure was forced expiratory volume in 1 second (FEV1) and secondary outcome measures were pulmonary symptom score index, peak expiratory flow rate (PEFR), hospitalization rate and the use of rescue beta2-agonists. Patients were evaluated at the start of treatment (visit 1), on day 2 (visit 2) and on day 7 (visit 3). RESULTS: Of the 54 children enrolled, two were hospitalized on visit 2 (one from each group). Baseline clinical data were similar in both groups: FEV1: 53 and 51 %; bronchodilator test: 119 and 121 %; PEFR: 169 and 165 L/min; symptom score: 6 and 6.5 for the deflazacort and prednisolone groups, respectively. On visit 2, all measures improved: FEV1: 122.2 and 126.5 % (p < 0.05); PEFR: 164 and 149 L/min (p < 0.05); symptom score: -4.4 and -3.8 (p < 0.05), without significant differences between groups. On visit 3 all variables continued to show improvement: FEV1: 133.2 and 132.5 % (p < 0.05); PEFR: 1115.7 and 187.6 L/min (p < 0.05); symptom score: -5.4 and -5.9 (p < 0.05), without significant differences between groups. No adverse effects were reported. CONCLUSIONS: Deflazacort and prednisolone show similar efficacy in improving pulmonary function and in producing clinical improvement in the management of acute moderate asthma in children.

Estudio comparativo de la eficacia del deflazacort frente a prednisolona en el tratamiento de la crisis asmática moderada / Comparative efficacy of oral deflazacort versus oral prednisolone in children with moderate acute asthma

Objetivos Evaluar la eficacia y tolerancia del deflazacort frente a la prednisolona en el tratamiento de la agudización moderada de asma en niños. Pacientes y métodos Estudio de intervención, multicéntrico, prospectivo, abierto, aleatorizado, grupos paralelos en niños de 6 a 14 años diagnosticados de asma, en situación de agudización moderada tratados todos con agonistas Beta2-adrenérgicos de corta acción. Los grupos de intervención recibieron deflazacort (1,5 mg/kg) o prednisolona (1 mg/kg) durante 7 días. La medida principal de eficacia fue el volumen espiratorio forzado en el primer segundo (FEV1) y como medidas secundarias se evaluaron la escala clínica de gravedad, el flujo espiratorio máximo (PEF), el índice de hospitalización y la utilización de medicación Beta2-agonista de rescate. Todos los sujetos fueron controlados al inicio del tratamiento (visita 1), al segundo día (visita 2) y al séptimo día (visita 3) del estudio. Resultados Se incluyeron en el estudio 54 pacientes, de los cuales dos requirieron hospitalización (uno de cada grupo). Los valores iniciales fueron similares para ambos grupos: FEV1, 53 y 51 por ciento; test de broncodilatación, 19 y + 21 por ciento;PEF, 169 y 165 l/min; escala de gravedad, 6,1 y 6,5 para los grupos deflazacort y prednisolona, respectivamente. En la visita 2, todos los parámetros mostraron mejoría: FEV1, +22,2 y 26,5 por ciento (p < 0,05); PEF, +64 y 49 l/min (p < 0,05); escala de gravedad -4,4 y -3,8 (p < 0,05), sin diferencias significativas entre ambos grupos. En la visita 3 todos los parámetros continuaron mejorando: FEV1, +33,2 y +32,5 por ciento (p < 0,05); PEF, +115,7 y 87,6 l/min (p < 0,05); escala de gravedad -5,4 y -5,9 (p < 0,05), también sin diferencias significativas entre los dos grupos. No se registraron efectos adversos en ningún paciente. Conclusiones En el tratamiento de la agudización moderada de asma en niños, deflazacort tiene una eficacia similar a prednisolona como se refleja tanto en la mejoría clínica de los pacientes como en la función pulmonar (AU)

Infecciones en la primera infancia: ¿beneficio o perjuicio en el asma? / No disponible

No disponible

Use of the Advia 120 hematology analyzer in the differential cytologic analysis of biological fluids (cerebrospinal, peritoneal, pleural, pericardial, synovial, and others).

The centralization of our laboratories and the demand for new parameters to measure have led to an increase in the number of biological fluid samples, which are generally sent for urgent analysis. Due to this they cannot be processed by manual methods. Meeting this increased demand for assistance is a challenge for the laboratory, and the challenge has been met by the automated hematology area. A study of the reliability of the Advia 120 hematology analyzer has been carried out through leukocyte and red blood cell counting of 179 biological fluids: cerebrospinal, peritoneal or ascitic, pleural, pericardial, synovial, and others. The automated leukocyte counts of cerebrospinal fluid samples containing up to 0.150 x 10(9) leukocytes/L are correlated with counts obtained with the manual reference method in a Neubauer counting chamber (r = 0.958; P = .0001). Applying Passing-Bablok regression analysis to these results indicates a slope p of 1.155 (95% confidence interval [CI], 0.915-1.347) and an ordinate intercept b of 0.0076 (95% CI, 0.012-0.034), showing the results to be perfectly interchangeable. In the comparison of the manual analysis of the leukocyte differential using the May-Grünwald-Giemsa staining method with the analysis using the automated method, the percentage of polymorphonuclear granulocytes of the Advia 120 basophil/lobularity method is significantly correlated (r = 0.844; P = .0001) with that obtained with the manual count. The results of Passing-Bablok regression analysis (p = 0.859 [95% CI, 0.58-1.190]; b = 8.8 [95% CI, -12.09-24.2]) indicate that these two counting methods are also perfectly interchangeable. Automated leukocyte and differential counts of peritoneal or ascitic fluids also show good correlations with the manual method, and the results are not statistically different. Pretreating synovial fluid samples with hyaluronidase enzyme allows their processing on the Advia 120; no significant differences were found between manual and automated methods with respect to leukocyte counts and differentials. Finally, results with pleural fluid samples indicated that leukocyte and differential counts obtained with the Advia 120 showed significant differences from results obtained with manual methods because of the high incidence of mesothelial, lymphoid, and other tumoral cells in this kind of fluid sample. This result shows that use of hematology analyzers is questionable for these kinds of samples, especially from oncology patients with tumors. A procedure is proposed for the processing of these pleural fluids.

Lung transplantation in young infants with interstitial pneumonia.

OBJECTIVES: We describe our experience with infants suffering from interstitial pneumonia referred for lung transplantation. METHODS: From April 1998 to December 2000, three infants were admitted to our lung transplantation program: a 9-month-old girl (patient 1) suffering from surfactant protein C deficiency who had high oxygen requirements (fraction of inspired oxygen: 70% to 90%), and two boys, ages 2 (patient 2) and 9 months (patient 3), who were ventilator-dependent due to chronic pneumonitis of infancy. RESULTS: Patients were transplanted at the age of 5 months (patient 2) and 13 months (patients 1 and 3) at 87 to 105 days after being accepted for lung transplantation. All cases underwent a sequential double lung transplant on cardiopulmonary bypass. The immunosuppressive regime included tacrolimus, prednisone, and azathioprine. Patients 2 and 3 also received basiliximab. Two cases suffered a mild rejection episode that responded to high-dose steroids. Patient 2 was ventilator-dependent for 8 months after transplant, owing to severe bronchomalacia and left main bronchus stenosis. Bronchial stenosis resolved after pneumatic dilatation and endobronchial stenting. This patient also presented with a pulmonary artery anastomosis stricture that required percutaneous balloon dilatation. All three patients are at home, carrying out normal activities for their age, with no respiratory symptoms after a period of 8 to 29 months of follow-up. CONCLUSIONS: Interstitial pneumonia of infancy is a rare disease with a bad prognosis and no specific treatment; therefore, lung transplantation represents a good therapeutic option for these infants.

Budesonide/formoterol in a single inhaler versus inhaled corticosteroids alone in the treatment of asthma.

The aim of this study was to evaluate the efficacy (expressed as effect on lung function) and tolerability of Symbicort (budesonide/formoterol in a single inhaler) in children with asthma. This was a double-blind, double-dummy, randomized, parallel-group, multicenter trial. After a 2-4-week run-in period, 286 asthmatic children (177 boys, 109 girls; mean age, 11 years; mean forced expiratory volume in 1 sec (FEV(1)), 75% predicted normal), previously treated with inhaled corticosteroids (average dose 548 microg/day), were randomized to 12 weeks' treatment with either budesonide/formoterol 80/4.5 microg, two inhalations twice daily (n = 148), or an equivalent dose of budesonide 100 microg, two inhalations twice daily (n = 138). Efficacy variables included morning and evening peak expiratory flow (PEF), spirometery, asthma symptoms, and use of rescue medication (beta(2)-agonists). Serial FEV(1) assessments were carried out on a subgroup of children (budesonide/formoterol, n = 41; budesonide, n = 40) at randomization and at week 12. Relative to baseline, morning PEF (primary variable) increased to a significantly greater extent with budesonide/formoterol than with budesonide alone (7.22% predicted normal vs 3.45% predicted normal; P < 0.001). Evening PEF also increased significantly with budesonide/formoterol (6.13% predicted normal vs. 2.73% predicted normal; P < 0.001), as did mean FEV(1) and serial FEV(1) measured over 12 hr (both P < 0.05). Similar improvements in asthma symptoms and rescue medication use were observed in both groups. The two treatment groups were similar in terms of their adverse-event profile and rates of discontinuation. Budesonide/formoterol in a single inhaler provided rapid improvements in PEF and FEV(1) compared to inhaled budesonide alone. These improvements were sustained throughout the study period. Budesonide/formoterol was well-tolerated in children with moderate persistent asthma.

[Pilot study on knowledge about asthma and its treatment among Spanish teachers]. / Estudio piloto de los conocimientos sobre el asma y su tratamiento entre los educadores españoles.

UNLABELLED: This was a study aimed to know the knowledge about asthma. SUBJECTS AND METHOD: 933 teachers of 27 schools from 7 cities (children aged between 4 and 15 years) using a self-administered questionnaire Half of them knew asthma prevalence and its hereditary nature. Nearly all related asthma with allergy, 38% with exercise, 25%had students who suffered from asthma attacks in the school, and 91% admitted to have limited information and wish to improve it. CONCLUSION: Our survey reveals that Spanish teachers have a limited information about asthma.

Estudio piloto de los conocimientos sobre el asma y su tratamiento entre los educadores españoles / Knowledge about asthma by primary school Spanish teacher's: a pilot study

FUNDAMENTO: Estudio para evaluar los conocimientos sobre el asma de los profesores mediante una encuesta autoadministrada. SUJETOS Y MÉTODO: Participaron 933 profesores de 27 colegios de 7 ciudades (niños de 4 a 15 años). RESULTADOS: La mitad conocían la prevalencia del asma y su carácter hereditario. Casi todos relacionaban asma con alergia, aunque sólo un 38 por ciento con el ejercicio. Un 25 por ciento había tenido niños con crisis en clase. El 91 por ciento admitía tener escasos conocimientos y deseaba mejorarlos. CONCLUSIÓN: La encuesta revela unos conocimientos limitados sobre el asma entre los profesores españoles (AU)

[Validity of the Spanish version of the Pediatric Quality of Life Questionnaire for evaluating quality of life in asthmatic children]. / Validación de la versión española del Pediatric Quality of Life Questionnaire en la valoración de la calidad de vida del niño asmático.

BACKGROUND: To analyze the clinical utility of the quality of life questionnaire for asthmatic children (PAQLQ) and to validate it for use in clinical practice. PATIENTS AND METHOD: 1,012 children between 6 and 14 years of age, with a diagnosis of mild to moderate asthma attending 48 Spanish hospitals were included in the study. The patients' socio-demographic and clinical characteristics were recorded and all patients were administered the PAQLQ and EQ-5D questionnaires on two occasions: at the baseline visit and at 2-3 months from baseline in patients with uncontrolled asthma(group A) and at baseline and 15 days from baseline in patients with controlled asthma (group B). The feasibility, validity, reliability and sensitivity to change of the PAQLQ were assessed. RESULTS: The PAQLQ proved to be feasible for use in children over 7 years of age with mild or moderate asthma. The PAQLQ did not show a statistically significant relationship with socio-demographic variables, nor with the majority of clinical variables, with the exception of asthma severity, number of exacerbations, symptoms and use ofon- demand short-term beta2 agonist drugs. Correlations between PAQLQ and EQ-5D dimensions were strongest between dimensions measuring similar attributes. Cronbach's *coefficients for the PAQLQ ranged from 0.88 for the limitations in activities dimension to 0.96 for the overall score. The intraclass correlation coefficient for PAQLQscores in group B ranged from 0.71 (limitation of activities)to 0.83 (overall score). The effect size between both visits ranged from 0.49 to 0.69. CONCLUSIONS: The Spanish version of the PAQLQ proved to be valid for use in children with mild to moderate asthma.

DNase use in the daily care of cystic fibrosis: who benefits from it and to what extent? Results of a cohort study of 199 patients in 13 centres. DNase National Study Group.

UNLABELLED: Short-term clinical trials with DNase have shown minor to moderate benefits in cystic fibrosis patients. This study was performed to analyse the effectiveness of DNase use in daily practice and to obtain information on its effects in the long term and at different disease stages. Patients being treated in 13 specialised units were included if they started DNase treatment before June 1996. Baseline data before DNase use and data during the DNase treatment period were recorded. Of the 199 patients included in the study 166 continued on DNase treatment while the data were being collected. The mean age (95% CI) was 14.5 (13.7; 15,2) years; 103 (51.8%) patients were female. The mean maximum change in forced expiratory volume in 1 s (FEV(1)) was observed during the first month of treatment [11.1% (6.1; 16.1)]. By the end of the first and the second year of treatment mean changes in FEV(1) were 3.3% (-1.1; 7. 6) and 5.1% (-0.7; 10.9) respectively; at the end of the same periods 34% of patients had improved their baseline FEV(1) by 10% or more but in around 50% of patients the level fell below the baseline. A large inter-individual variability in changes in pulmonary function after the start of DNase treatment was documented. In addition, the medium-term response to treatment was correlated with early response during the first 3 months. No consistent changes in exacerbation pattern were found during the first year of treatment. CONCLUSIONS: The benefits of DNase use in daily practice are limited but apparently can be maintained in the medium term in some patients. A large inter-individual variability in response to DNase treatment has been documented and the benefits are doubtful in around 50% of patients. This observation points to the need to set up a withdrawal trial in these patients, using as an eligibility criterion the early response observed during the first 3 months of treatment.

Tratamiento a largo plazo con dornasa alfa en la fibrosis quística / Long term treatment with rhDnase for cystic fibrosis

Objetivo. Evaluar la respuesta al tratamiento a largo plazo con rhDnasa en pacientes afectos de fibrosisquística. Diseño. Estudio retrospectivo, multicentro. Población. Doscientos seis pacientes. Metodología. En trece unidades de fibrosis quística de España sereunieron los pacientes que iniciaron tratamiento con 2.50 mg. de rhDnasa cada 24 horas, entre junio de 1994 y junio de 1996. Resultados. En la valoración inicial la media de peso fue de 40.06 kg y la talla 148 cms. La media de la Capacidad Vital Forzada (CVF) fue de 65.8 por ciento con respecto a su valor de predicción y la del Volúmen Espiratorio Forzado del primer minuto (VEF1) del 54.39 por ciento. A los tres años de tratamiento ha disminuído el número de pacientes con respuesta nula para ambos parámetros y ha aumentado significativamente el porcentaje de casos con respuesta buena o excelente para la CVF, mientras que para el VEF1 se mantuvo con valores similares. Conclusiones. El tratamiento con rhDnasa nebulizada es eficaz en el 60 por ciento de los enfermos afectos de fibrosis quística

[Intravascular hemolysis following percutaneous occlusion of the ductus arteriosus]. / Hemólisis intravascular tras el cierre percutáneo del conducto arterioso.

Transcatheter occlusion of patent ductus arteriosus has become a safe and successful technique, but it's not free of complications. We present the case of a two-year-old boy who underwent routine transcatheter closure of his patent ductus arteriosus, using a "coil" device. Twenty hours later he developed severe persistent hemolysis in association with residual ductal flow. Patient's clinical situation became stable when the device was removed. Pulmonary embolization of the device and hemolysis are the main complications of percutaneous closure of the patent ductus arteriosus. Hemolysis occurs rarely (0.5%) and is always associated with the presence of residual ductal flow. Several approaches to this problem have been described. Mild cases may require no intervention; however, when severe hemolysis is present, removal of the device may be needed, proceeding with surgical repair of the patent ductus arteriosus.

[Bone mass in patients with cystic fibrosis of the pancreas. Relationship with anthropometric parameters and genotype]. / Masa ósea en pacientes con fibrosis quística de páncreas. Relación con parámetros antropométricos y genotipo.

BACKGROUND: Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibrosis of the pancreas. The relationship of BMD values with nutritional status, respiratory function and the cystic transmembrane regulator genotype was also evaluated. PATIENTS AND METHODS: BMD expressed as grams of hydroxyapatite/cm2 was measured by dual-energy X-ray absorptiometry in the lumbar spine (L2-L4) in 41 patients (21 males and 20 women; age range: 4-21 years) with cystic fibrosis of the pancreas and compared with that of 471 normal controls (256 males and 215 women; age range: 1-20 years). Twenty patients were prepubertal, 9 pubertal and 12 young adults. RESULTS: Clinical repercussion of the disease evaluated by clinical and anthropometric data (weight, height and body mass index) and respiratory function was considered moderate. Height z score (mean [MSE]) was -0.53 (0.28), weight -0.81 (0.21) and body mass index -0.82 (0.12) BMD z score values (mean [MSE]) were -1.14 (0.17) and differed significantly (p < 0.001) from those of normal age- and sex-matched controls. No significant differences were observed between males and women or among prepubertal, pubertal and young adult patients. BMD z score values less than-1 z score were found in 53% and under -2 z score in 8%. Cystic transmembrane regulator genotype was studied in 36 patients (17 were F508/-, 10 F508/F508, 5 G542X/- and 4 diverse) and did not predict bone mineral status. A statistically significant correlation was found between BMD z score values and height z score, weight z score, body mass index z score and clinical assessment according to Shwachman criteria. A negative and statistically significant correlation was observed between BMD z score and functional score. CONCLUSIONS: The decrease in BMD values in CF patients begins early in life and appears to be related to the degree of clinical expression of the disease.