RESUMEN
BACKGROUND: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. METHODS: Transglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the study, all receiving a gluten-free diet. Patients with elevated TGA (>50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum. RESULTS: Four new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal biopsies. In total, 10 cases of CD (1.2%, 1:83) indicate a prevalence rate about three times higher than the general prevalence of CD in Norway and Sweden. No CD patients were detected in the Danish CF cohort. Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet. CONCLUSIONS: Systematic screening for CD in a Scandinavian cohort of CF patients revealed a higher prevalence of CD than in the general population. Clinical signs of CD are difficult to differentiate from CF with malabsorption, and patients may go undiagnosed for a long time. In a population where CD is common we recommend screening for CD in patients with CF.
Asunto(s)
Enfermedad Celíaca/epidemiología , Fibrosis Quística/epidemiología , Adolescente , Adulto , Enfermedad Celíaca/sangre , Niño , Preescolar , Comorbilidad , Estudios Transversales , Fibrosis Quística/sangre , Femenino , Humanos , Inmunoglobulina A/sangre , Lactante , Masculino , Persona de Mediana Edad , Prevalencia , Países Escandinavos y Nórdicos/epidemiología , Adulto JovenRESUMEN
OBJECTIVES: Typing of Pseudomonas aeruginosa isolates from Norwegian cystic fibrosis (CF) patients with chronic Pseudomonas lung infection in order to see whether cross-infection might have occurred. METHODS: Isolates from 60 patients were collected during the years 1994-98, and typed by pulsed field gel electrophoresis. RESULTS: Seventy-one strains were identified. One large cluster of identical strains included 27 patients, and 13 smaller clusters of 2-4 patients were found (26 patients). Seven patients had a strain not shared by other patients (private strains). Harboring the main cluster strain was significantly associated with participation in summer camps and training courses (P = 0.004, chi-squared test). There were no associations with regular admissions to hospital (intravenous antibiotic courses) or smaller social gatherings of short duration. Small clusters and private strains were not associated with any of the risk factors. All strains were sensitive to colistin. The minimal inhibitory concentrations were generally lower in Norwegian P. aeruginosa strains compared with isolates from Danish patients. CONCLUSIONS: Our results indicate that cross-infection with P. aeruginosa between cystic fibrosis patients has occurred.
Asunto(s)
Infección Hospitalaria/complicaciones , Fibrosis Quística/complicaciones , Infecciones por Pseudomonas/complicaciones , Pseudomonas aeruginosa/clasificación , Adolescente , Adulto , Técnicas de Tipificación Bacteriana , Niño , Preescolar , Infección Hospitalaria/microbiología , Fibrosis Quística/microbiología , Electroforesis en Gel de Campo Pulsado , Humanos , Lactante , Noruega , Infecciones por Pseudomonas/microbiología , Esputo/microbiologíaRESUMEN
PURPOSE: To assess whether MR imaging can improve characterization of ethmomaxillary opacification diagnosed at CT in patients with cystic fibrosis (CF) in order to select patients that may benefit from functional endoscopic sinus surgery (FESS). MATERIAL AND METHODS: Sixty-two CF patients (26 females and 36 males) aged 4-50 years (median 20 years) with ethmomaxillary sinus disease at CT underwent MR examination of the paranasal sinuses (coronal T1 and STIR sequences). FESS had been performed in 28 of the patients prior to this study. MR signal intensities were interpreted as mucosal thickening or infectious material, according to a previous study. RESULTS: Three major maxillary sinus MR patterns could be distinguished: Air-filled, oval-shaped pus-filled, and streaky-shaped pus-filled sinus lumen. For air-filled maxillary sinuses with mucosal thickening, CT and MR imaging were diagnostically equivalent. Where CT showed homogeneous opacification of the maxillary sinuses, MR imaging differentiated between thickened mucosa and pus-filled areas. Patients who had undergone FESS most commonly had air-filled or streaky-shaped pus-filled maxillary sinus lumen. In non-operated patients oval-shaped pus-filled sinus lumen was most common and could occur without ethmoid disease. CONCLUSION: MR imaging of the paranasal sinuses can differentiate between infectious material and thickened mucosa and should be used to select CF patients with pus-filled areas that can be eradicated with FESS.
Asunto(s)
Fibrosis Quística/complicaciones , Senos Etmoidales , Imagen por Resonancia Magnética , Seno Maxilar , Enfermedades de los Senos Paranasales/diagnóstico , Tomografía Computarizada por Rayos X , Adolescente , Adulto , Niño , Preescolar , Endoscopía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de los Senos Paranasales/cirugía , Estudios ProspectivosRESUMEN
Gastrointestinal complications of cystic fibrosis are becoming more common because patients with cystic fibrosis are living longer. There are 227 cystic fibrosis patients in Norway today, almost half of whom are more than 18 years old. Meconiumileus-equivalent is a complication which increases with age. It is a term used to describe partial or complete intestinal obstruction occurring in patients with cystic fibrosis. It results from abnormally viscid mucofaeculent material in the terminal ileum and right proximal colon. Some patients may experience acute complete obstruction, but most of them suffer from chronic partial obstruction, with recurring colicly abdominal pain and some distension. Of 70 adult cystic fibrosis patients at Aker hospital over a seven year period, 26% had symptoms and signs of this disorder. Conservative treatment is preferable, and surgery should be avoided.
Asunto(s)
Fibrosis Quística/complicaciones , Obstrucción Intestinal/etiología , Meconio , Abdomen Agudo/diagnóstico , Abdomen Agudo/diagnóstico por imagen , Adulto , Femenino , Humanos , Obstrucción Intestinal/diagnóstico por imagen , Obstrucción Intestinal/terapia , Masculino , RadiografíaRESUMEN
Long-term treatment with pivampicillin and pivmecillinam for 6-24 months in five adults and one child reduced the total serum carnitine concentrations to 3.7-14.0 mumol/l (reference value 25-66 mumol/l). The muscle carnitine was reduced to 0.3-0.7 mumol/g wet weight (reference value 3-5 mumol/g) in two cases. All patients had asthenia and muscle symptoms with weakness and pain. One showed signs of carnitine depletion in the liver, with increased secretion of dicarboxylic acids (C6, C8, C10) in urine and limited ketone body formation during prolonged fasting (32 hours). The serum carnitine increased slowly after cessation of therapy and reached normal concentrations after 6-12 months. All symptoms caused by carnitine depletion disappeared after the serum carnitine reached 20 mumol/l. This was achieved on a normal diet without carnitine supplement.
Asunto(s)
Amdinocilina Pivoxil/efectos adversos , Carnitina/sangre , Pivampicilina/efectos adversos , Adulto , Anciano , Amdinocilina Pivoxil/administración & dosificación , Carnitina/deficiencia , Niño , Femenino , Humanos , Masculino , Pivampicilina/administración & dosificación , Factores de TiempoRESUMEN
The pharmacokinetics of imipenem (MK-787) and cilastatin (MK-791) were studied in 30 patients with cystic fibrosis (CF) receiving the drug for therapeutic purposes at doses of 11 mg/kg given as a 30-min infusion. The serum concentrations and urine elimination were studied after the first dose and during steady state. The concentrations were assayed by high-pressure liquid chromatography. The total areas under the imipenem serum concentration curves (AUCs) to infinity were 30.4 +/- 6.8 mg.h/l after the first dose compared with 29.1 +/- 7.1 mg.h/l during steady state (NS). The cilastatin AUCs on the 2 days were 40.3 +/- 9.3 and 38.3 +/- 0.4 mg.h/l (NS), respectively. The urinary recovery of imipenem was 47.8 +/- 17.8% after the first dose and 57.8 +/- 24.2% during steady state (NS). The amounts of cilastatin eliminated in the urine during 6 h were 6/7.3 +/- 22.9% after the first dose and 60.5 +/- 17.0% of the dose during steady state (NS). The mean half-life of imipenem in these CF patients was 1.2 +/- 0.4 h on the first day of the examination and within the same range during steady state. The distribution volume (Vd beta) was in the range of 28 liters, the total body clearance was 16.3 liters/h (285 ml/min) on the first day. The t1/2 of cilastatin was 0.59 +/- 0.14 h after the first dose and 0.61 +/- 0.14 h during steady state. Thus patients with CF eliminated cilastatin more quickly than imipenem.
Asunto(s)
Antibacterianos/farmacocinética , Cilastatina/farmacocinética , Fibrosis Quística/metabolismo , Imipenem/farmacocinética , Adolescente , Adulto , Combinación Cilastatina e Imipenem , Combinación de Medicamentos , Estudios de Evaluación como Asunto , Femenino , Humanos , MasculinoRESUMEN
Long-term treatment with pivampicillin and pivmecillinam for 6-24 months in five adults and one child reduced the total serum carnitine concentration to 3.7-14 mumol/l (reference value: 25-66 mumol/l). Muscle carnitine was reduced to 0.3-0.7 mumol/g wet weight (reference value: 3-5 mumol/g) in two cases. All patients had muscle symptoms with weakness, asthenia and pains. One showed signs of carnitine depletion in the liver with increased secretion of dicarboxylic acids (C6, C8, C10) in urine and limited ketone body formation during prolonged fasting. Serum carnitine increased slowly after cessation of therapy and reached normal concentrations after 6-12 months. All symptoms caused by carnitine depletion disappeared. This was achieved on a normal diet without carnitine supplementation.
Asunto(s)
Amdinocilina Pivoxil/efectos adversos , Carnitina/sangre , Carnitina/deficiencia , Pivampicilina/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , Niño , Femenino , Humanos , Masculino , Factores de Tiempo , Deficiencia de Vitamina B/sangre , Deficiencia de Vitamina B/inducido químicamenteRESUMEN
Imipenem, a new N-formimidoyl thienamycin was given together with cilastatin to 20 patients with cystic fibrosis and pulmonary infection due to Pseudomonas aeruginosa. The antibiotic was given in short-term infusions for 9-14 days (mean 11.5) in a dose of 45-60 mg/kg body weight/day. Good clinical results were obtained in all patients with significant improvement of clinical score, pulse rate, vital capacity and FEV1.0 (P less than 0.001). Blood PO2 increased and WBC decreased significantly. A slight increase in the minimum inhibitory concentration was noted during treatment but all strains examined were fully susceptible at follow-up one month later. The peak serum concentration was significantly increased in patients receiving the high dose of imipenem, but the sputum concentration was low in all patients and there was no difference in clinical or bacteriological outcome. The plasma and urinary clearance increased with body weight and was inversely correlated to clinical score. Imipenem/cilastatin appears a good alternative for the treatment of pulmonary infections caused by P. aeruginosa in cystic fibrosis.
Asunto(s)
Fibrosis Quística/complicaciones , Infecciones por Pseudomonas/tratamiento farmacológico , Adolescente , Adulto , Niño , Cilastatina , Combinación Cilastatina e Imipenem , Ciclopropanos/farmacocinética , Ciclopropanos/uso terapéutico , Combinación de Medicamentos/farmacocinética , Combinación de Medicamentos/uso terapéutico , Farmacorresistencia Microbiana , Femenino , Humanos , Imipenem , Masculino , Infecciones por Pseudomonas/etiología , Pseudomonas aeruginosa/efectos de los fármacos , Esputo/análisis , Tienamicinas/farmacocinética , Tienamicinas/uso terapéuticoRESUMEN
A boy suffering from cystic fibrosis (CF) complicated by generalized amyloidosis is reported. His condition was fairly good during the first 10 years of life, but after this time he had repeated pulmonary infections caused by Staphylococcus aureus. From the age of 14, he developed increasing hepato- and splenomegaly and a liver biopsy revealed massive amyloidosis. At the age of 16 he died of respiratory and cardiac failure. Post-mortem examination revealed wide-spread amyloidosis in addition to pulmonary and pancreatic findings characteristic of CF.