BACKGROUND AND OBJECTIVE: Our objective was to describe the clinical and genetics features of patients with cystic fibrosis (CF) attended in Madrid. PATIENTS AND METHOD: This was a descriptive, cross-sectional study of CF patients attended during 2001. Demographic, genetic, anthropometric, pancreatic insufficiency, diabetes mellitus, Pseudomonas aeruginosa colonization, lung function and body mass index (BMI) (z score) data were recorded and compared with the American CF Registry. RESULTS: 387 patients were included, most of them living in Madrid (n = 247 [63%]), 209 mal es (54%), with a mean age (SD) of 15.15 (10.42) years (younger than 18 years 248, 64.24%). F508del was the most common mutation (52.8% of chromosomes) with 104 homozygous patients. Pancreatic insufficiency was present in 310 subjects (80.1%), diabetes mellitus in 30 (7.8%) and P. aeruginosa colonization in 126 (33.1%). Lung function was measured in 309 patients: mean of FEV1 and FVC predicted values (SD) was 82.5 (27.11) and 89.32 (21.89), respectively. The mean BMI z score was -0.0796 (1.18). CONCLUSIONS: CF patients from Madrid have a good nutritional status, less P. aeruginosa colonization, less pancreatic insufficiency and better lung function than those of the American CF Registry. The lower prevalence of homozygous F508del in our population may explain, at least partly, our findings.

Estudio descriptivo en el que se valoran los ciclos de tratamiento antibióticointravenoso domiciliario (TAIVD) que se originaron en las unidades de fibrosis quística(FQ) de la Comunidad de Madrid en un período de 18 meses. Se analizan las características delos pacientes, el tipo de antibioterapia y los accesos venosos utilizados, así como los dispositivosde administración, las complicaciones surgidas y su resolución. Se valoró si existió mejoríaen los parámetros de función pulmonar al finalizar el tratamiento y se relacionó esta mejoríacon determinadas variables clínicas. Se incluyó en el estudio a los pacientes con FQ que recibieron TAIVD a lolargo de 18 meses (enero de 2002-junio de 2003). Se recogieron las siguientes variables delos enfermos: edad, sexo, colonización bacteriana de la vía aérea y función pulmonar en faseestable, previa y posterior al TAIVD. Se incluyó a 56 enfermos (31 varones y 25 mujeres) que recibieron un total de 90 ciclosde antibioterapia intravenosa. La edad media (DE) fue de 20,06 (8,07) años. El 57,1% estabacolonizado por Pseudomonas aeruginosa. Los antibióticos más utilizados fueron ceftazidima ytobramicina. Los pacientes permanecieron una media de 4,08 (5,09) días ingresados en el hospitaly 11,89 (4,96) en domicilio. En el 87,8% de los casos se utilizó la vía periférica, en el 6,7%Port-a-cath® y en el 7,8% Venocath® para la administración de los antibióticos. En el 26,7% delos casos se perdió el acceso venoso. La recanalización de la nueva vía periférica se realizó en el64,2% por la enfermera de la unidad de FQ correspondiente. Los pacientes mejoraron los parámetrosde función pulmonar de forma significativa tras finalizar el tratamiento. La TAIVD es una modalidad terapéutica que, ajustándose a unos criterios de inclusióny exclusión predeterminados, presenta escasas complicaciones y mejora la función pulmonar. A descriptive study of the home intravenous antibiotic treatment (HIVAT)course in cystic fibrosis (CF) units of Madrid in an 18 month period. Different patient featureswere recorded, antibiotherapy, intravenous access, complications and their resolutions.We accessed the improvement of the pulmonary function, forced vital capacity (FVC) and forcedexpiratory volume in one second (FEV1) at the end of the treatment. For an 18 months period (January 2002-June 2003) the patients with CFwho received HIVAT and fulfilled the previously fixed criteria were included. The next clinic variableswere collected: age, sex, bacterial colonization of respiratory tree, pancreatic functionand pulmonary function in steady phase, before and after HIAT. 56 patients, 31 male and 25 female, were given 90 courses of HIAT (34 patients receivedonly one course). Mean age was 20.06 (8.07) years. 57.1% of the patients were colonizedby Pseudomonas aeruginosa. The most frequently used antibiotics were ceftazidime and tobramycin.Courses of treatment lasted a mean of 4.08 (5.09) days inpatient and 11.89 (4.96)at home. In 87.7% of the course were used the intravenous cannulae, Port-a-cath® in 6.7%and Venocath® in 7.8%. The intravenous access was replaced, in the 64.4% of the cases, bythe nurse of the CF Unit, in the 21.4% in the emergency room of the nearest hospital and in11.9% in primary care center. Three occasions skin reactions were reported. The parameters ofpulmonary function improved significatively after HIVAT. The HIVAT is a therapeutic option that, following predetermined inclusion criteria,has a low complication rate and improves pulmonary function.

BACKGROUND AND OBJECTIVE:A descriptive study of the home intravenous antibiotic treatment (HIVAT) course in cystic fibrosis (CF) units of Madrid in an 18 month period. Different patient features were recorded, antibiotherapy, intravenous access, complications and their resolutions. We accessed the improvement of the pulmonary function, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) at the end of the treatment.PATIENTS AND METHOD:For an 18 months period (January 2002-June 2003) the patients with CF who received HIVAT and fulfilled the previously fixed criteria were included. The next clinic variables were collected: age, sex, bacterial colonization of respiratory tree, pancreatic function and pulmonary function in steady phase, before and after HIAT.RESULTS:56 patients, 31 male and 25 female, were given 90 courses of HIAT (34 patients received only one course). Mean age was 20.06 (8.07) years. 57.1% of the patients were colonized by Pseudomonas aeruginosa. The most frequently used antibiotics were ceftazidime and tobramycin. Courses of treatment lasted a mean of 4.08 (5.09) days inpatient and 11.89 (4.96) at home. In 87.7% of the course were used the intravenous cannulae, Port-a-cath in 6.7% and Venocath in 7.8%. The intravenous access was replaced, in the 64.4% of the cases, by the nurse of the CF Unit, in the 21.4% in the emergency room of the nearest hospital and in 11.9% in primary care center. Three occasions skin reactions were reported. The parameters of pulmonary function improved significatively after HIVAT.CONCLUSIONS:The HIVAT is a therapeutic option that, following predetermined inclusion criteria, has a low complication rate and improves pulmonary function.

Establecer las características clínicas y genéticas de los pacientes confibrosis quística (FQ) de la Comunidad de Madrid. Estudio descriptivo y transversal de los pacientes con FQ atendidos regularmentedurante el año 2001. Se recogieron datos demográficos, genéticos, antropométricos,presencia de insuficiencia pancreática, diabetes mellitus y colonización por Pseudomonas aeruginosa,valores espirométricos y de índice de masa corporal (puntuación Z), y se compararoncon los del registro americano del mismo año. Se incluyó a 387 pacientes –209 varones (54%)–, la mayoría residentes en Madrid(n = 247 [63%]), con una edad media (DE) de 15,15 (10,42) años (menores de 18 años, 248[64,24%]). La mutación más frecuente fue la F508del (52,8% de los cromosomas), con 104homocigotos (28,5%). Presentaron insuficiencia pancreática 310 (80,1%); diabetes mellitus,30 (7,8%), y colonización por P. aeruginosa, 126 (33,1%). La función pulmonar se midió en309 pacientes. La media del porcentaje predicho del volumen espiratorio forzado en el primersegundo fue de 82,5 (27,11) y la de la capacidad vital forzada de 89,32 (21,89). La media dela puntuación Z del índice de masa corporal fue de –0,0796 (1,18). La peor función pulmonarcorrespondió a los pacientes colonizados por P. aeruginosa, con peor estado de nutrición y demayor edad. La insuficiencia pancreática, la edad y la peor función respiratoria (volumen espiratorioforzado en el primer segundo) determinaron el peor estado nutricional. Los pacientes con FQ de nuestra comunidad tienen buen estado nutricional, menoscolonización por P. aeruginosa e insuficiencia pancreática y mejor función pulmonar quelos pacientes del registro americano. La menor presencia de la mutación F508del en homocigosispodría ser la causa, al menos en parte, de estos resultados. Our objective was to describe the clinical and genetics features ofpatients with cystic fibrosis (CF) attended in Madrid. This was a descriptive, cross-sectional study of CF patients attended during2001. Demographic, genetic, anthropometric, pancreatic insufficiency, diabetes mellitus,Pseudomonas aeruginosa colonization, lung function and body mass index (BMI) (z score) datawere recorded and compared with the American CF Registry. 387 patients were included, most of them living in Madrid (n = 247 [63%]), 209 males(54%), with a mean age (SD) of 15.15 (10.42) years (younger than 18 years, 248,64.24%). F508del was the most common mutation (52.8% of chromosomes) with 104 homozygouspatients. Pancreatic insufficiency was present in 310 subjects (80.1%), diabetesmellitus in 30 (7.8%) and P. aeruginosa colonization in 126 (33.1%). Lung function was measuredin 309 patients: mean of FEV1 and FVC predicted values (SD) was 82.5 (27.11) and89.32 (21.89), respectively. The mean BMI z score was –0.0796 (1.18). CF patients from Madrid have a good nutritional status, less P. aeruginosa colonization,less pancreatic insufficiency and better lung function than those of the American CFRegistry. The lower prevalence of homozygous F508del in our population may explain, at leastpartly, our findings.