[Clinical and nutritional aspects in patients with cystic fibrosis: 20 years of follow-up in the Clinical Hospital-Federal University of Minas Gerais (HC-UFMG)].
BACKGROUND
Cystic fibrosis (CF) is the most common severe autosomal recessive disease in caucasian population. The life expectancy of patients with CF has improved dramatically during the past three decades. A better approach to these patients depends on a better knowledge of clinical course and prognosis of CF. The purpose of the present study was to identify aspects clinical and nutritional on admission at Center of CF (HC-UFMG).


METHODS
Over a period of 20 years 127 patients with CF were admitted, submitted to a systematic protocol and prospectively followed. Data associated with demographic conditions, clinical presentation, nutritional and laboratory findings on admission were studied. The median follow-up was 44 months. The genotype was performed by PCR method. The survival analysis was carried out by Kaplan-Meier method.


RESULTS
The median age at diagnosis was 33 months. Sixty-one per cent of patients at diagnosis had chronic pulmonary and gastrointestinal symptoms. Seventeen patients (16%) were homozygous for DF508 mutation and 30 (28%) were heterozygous. There was a standard prevalence of malnutrition of 63% at diagnosis and of 45% at the end of follow up. Twenty patients (15,7%) died during follow up. The estimated probability of survival after one year from diagnosis was 96% and after five years was 80%.


CONCLUSIONS
CF diagnosis has been later in our country and the survival is shorter than in developed countries. Management of cystic fibrosis in pediatric specializing centers results in a better nutritional state in spite of clinical progression of the disease.
Reis
F J
FJ
Departamento de Pediatria, Unidade de Pneumologia Pediátrica do Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG.
Oliveira
M C
MC
Penna
F J
FJ
das GR Oliveira
M
M
Oliveira
E A
EA
Monteiro
A P
AP
D000293:Adolescent; D017668:Age of Onset; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D002713:Chlorine / Q000032:analysis; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000235:genetics / Q000401:mortality*; D005260:Female; D005500:Follow-Up Studies; D005838:Genotype; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D009748:Nutrition Disorders / Q000453:epidemiology; D009752:Nutritional Status; D016019:Survival Analysis; D013542:Sweat / Q000737:chemistry
D002713:Chlorine