Asunto(s)
Técnicas de Laboratorio Clínico/normas , Fibrosis Quística/complicaciones , Diabetes Mellitus , Hemoglobina Glucada/análisis , Adulto , Niño , Técnicas de Laboratorio Clínico/métodos , Diabetes Mellitus/sangre , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/etiología , Precisión de la Medición Dimensional , Humanos , Utilización de Procedimientos y Técnicas/estadística & datos numéricos , Mejoramiento de la Calidad , Reproducibilidad de los ResultadosRESUMEN
OBJECTIVE: To determine whether serum fructosamine correlates with glycemic control and clinical outcomes in patients being screened for cystic fibrosis-related diabetes (CFRD). METHODS: Fructosamine and percent predicted forced expiratory volume in 1s (FEV1) were measured in patients undergoing a 2h oral glucose tolerance test (OGTT) for CFRD screening. Fractional serum fructosamine (FSF) was calculated as fructosamine/total protein. RESULTS: FSF exhibited a positive correlation with 2h OGTT results (r2=0.3201, p=0.009), and ROC curve analysis suggested that FSF can identify patients with an abnormal OGTT (AUC=0.840, p=0.0002). FSF also exhibited a negative correlation with FEV1 (r2=0.3732, p=0.035). Patients with FSF≥3.70µmol/g had significantly lower FEV1 (median 47%) compared to those with FSF<3.70µmol/g (median 90%; p=0.015). CONCLUSIONS: FSF correlated with both OGTT results and FEV1, and reliably identified patients with abnormal OGTT results. This simple blood test shows potential as an effective tool in CFRD screening.
Asunto(s)
Fibrosis Quística , Diabetes Mellitus , Volumen Espiratorio Forzado , Fructosamina/sangre , Tamizaje Masivo/métodos , Adulto , Canadá , Correlación de Datos , Fibrosis Quística/sangre , Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico , Diabetes Mellitus/sangre , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/etiología , Femenino , Prueba de Tolerancia a la Glucosa/métodos , Hemoglobina Glucada/análisis , Humanos , Masculino , Reproducibilidad de los Resultados , Pruebas de Función Respiratoria/métodosRESUMEN
UNLABELLED: The objective of this study was to investigate the effect of docosahexaenoic acid (DHA) supplementation on blood and intestinal DHA levels and lung function in mild/moderately affected adult CF patients with the DeltaF508 genotype. BACKGROUND: Cystic Fibrosis (CF) patients often present with plasma fatty acid levels indicating low levels of linoleic (18:2n-6) and docosahexaenoic (22:6n-3) acids and an increased level of arachidonic acid (20:4n-6). Improved dietary fat intake or reducing fat malabsorption with pancreatic enzymes has failed to normalize this biochemical deficiency of DHA. METHODS: Five CF patients, aged 18-43, received 70 mg of DHA/kg body weight/d for six weeks. At baseline and at six weeks a physical exam, lung function, 3-day dietary intake, duodenal mucosal biopsy and blood sample were assessed. The blood was analyzed for plasma vitamin A, D and E levels, liver function tests, clinical chemistry (CBC, differential and electrolytes). Plasma and red blood cell fatty acid levels were also analyzed. At three weeks, assessment included a physical exam, lung function test and fasting blood sample (vitamin levels, liver function and clinical chemistry only). RESULTS: Pre- and post-measurements were compared for the four subjects who completed the study. An increase in DHA content (% w/w) was observed in all phospholipid fractions of plasma, red blood cell and mucosal samples. No significant differences in vitamin levels, liver function or lung function were observed. CONCLUSIONS: The study proves the concept that an increase in tissue DHA levels in CF patients can be achieved by supplementing for six weeks with 70 mg/kg/d DHA.
Asunto(s)
Fibrosis Quística/metabolismo , Suplementos Dietéticos , Ácidos Docosahexaenoicos/administración & dosificación , Duodeno/metabolismo , Ácidos Grasos/metabolismo , Administración Oral , Adolescente , Adulto , Cápsulas , Fibrosis Quística/sangre , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Eritrocitos/metabolismo , Ácidos Grasos/sangre , Femenino , Genotipo , Humanos , Mucosa Intestinal/metabolismo , Masculino , Mutación , Fosfatidilcolinas/sangre , Fosfatidilcolinas/metabolismo , Fosfatidiletanolaminas/sangre , Fosfatidiletanolaminas/metabolismoAsunto(s)
Carcinoma Hepatocelular/secundario , Neoplasias Hepáticas/patología , Trasplante de Hígado , Complicaciones Posoperatorias , Neoplasias Craneales/secundario , Hueso Temporal , Resultado Fatal , Humanos , Hepatopatías/cirugía , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos XRESUMEN
The development of pulmonary aspergilloma and invasive aspergillosis is a rare complication of cystic fibrosis. We describe a 29-year-old patient with cystic fibrosis who had invasive pulmonary aspergillosis that was not cured by amphotericin B, liposomal amphotericin B, or itraconazole. This patient was subsequently successfully treated and cured with the novel antifungal agent voriconazole.
Asunto(s)
Antifúngicos/uso terapéutico , Aspergilosis/tratamiento farmacológico , Fibrosis Quística/complicaciones , Enfermedades Pulmonares Fúngicas/tratamiento farmacológico , Pirimidinas/uso terapéutico , Triazoles/uso terapéutico , Adulto , Aspergilosis/etiología , Femenino , Humanos , Enfermedades Pulmonares Fúngicas/etiología , Resultado del Tratamiento , VoriconazolRESUMEN
The goals of this practice-based, observational study were to describe the prevalence of low bone mineral density in patients at the Edmonton Cystic Fibrosis Centre, and to determine if body mass index and previous systemic corticosteroid use of over one month's duration were predictors of low bone mineral density. One hundred and thirteen pediatric and adult patients were studied. Bone mineral density of the lumbar spine region was measured using dual-energy X-ray absorptiometry. A total of 42.5% of patients had a bone mineral density Z-score of less than -1 standard deviation. Low bone mineral density was apparent at nine to 12 years of age, and was most evident in the 20- to 34-year-old group. All but one patient under age 20 with a Z-score of less than -2.5 also had a body mass index below the fifth percentile. A low Z-score was also associated with previous systemic corticosteroid use of over one month's duration (relative risk 1.81, p=0.003). We conclude that low bone mineral density is common in cystic fibrosis patients. Low body mass index percentiles may be used to identify children and adolescents at risk of low bone mineral density. These patients may benefit from aggressive nutrition therapy. Systemic corticosteroid use should be assessed carefully, as it is a risk factor for low bone mineral density.