RESUMEN
Five patients with CF (cystic fibrosis) dead between 1974 to 1982 at ages ranging from one to six months are presented. All of them showed edema, hypoalbuminemia and anemia in a severely compromised clinical situation, and failure to gain weight in spite of being breast-fed in the first weeks of life, in four of them. All of them were second or third degree malnourished babies (Gomez classification) at admission. Five children presented edema, two severe, two moderate and one mild. Hematocrit values ranged from 19% to 39% (means 26.4%), and albuminemia from 1.60 to 3.00 g/% (means 2.14 g/%). Two patients presented antecedents of dead brothers. All of them received substitution therapy with pancreatic enzymes. The children dead within seven and seventeen days of admission (means ten days) of broncho-pulmonar disfunction. In this work, we wish to call the pediatrician's attention about the importance of making this diagnostic presumption in the first months of the life.
Asunto(s)
Fibrosis Quística/patología , Bronquios/patología , Duodeno/patología , Femenino , Vesícula Biliar/patología , Humanos , Lactante , Hígado/patología , Masculino , Páncreas/patologíaRESUMEN
Fecal alpha-1-antitrypsin clearance (A-1-At Cl) was performed on 47 pediatric age patients with various digestive diseases: 6 with ulcerative colitis, 5 with celiac disease, 6 with cow milk protein intolerance, 1 with intestinal lymphangiectasia, 1 with non specific diarrhea and the control group was composed of 10 children without digestive disease. The group of patients with digestive disease showed values of fecal A-1-At Cl significantly higher than the control and non specific diarrhea groups (p less than 0.05). Just 1 child with cow milk intolerance had A-1-At Cl within the range of values of the control group x = 2 S.D. All children with non specific diarrhea excepting one had values falling within the control range. The patient with thalassemia major had a very elevated value of A-1-At Cl. The cause of this finding remains unknown at present. The fecal A-1-At Cl. is a non invasive, cost saving, useful and simpler method than the traditional techniques for the diagnosis of protein losing enteropathy in childhood.
Asunto(s)
Heces/análisis , Enteropatías Perdedoras de Proteínas/metabolismo , alfa 1-Antitripsina/metabolismo , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , alfa 1-Antitripsina/análisisRESUMEN
UNLABELLED: 24 children whose ages ranged from 10 days to 14 years (x 5.5. ys.), 16 males and 8 females, hospitalized in our Service on account of extra-digestive were studied. Creatinine and amylase determination were performed on serum and 24 hs. urine specimens; lipase activity was measured only in serum. Amylase-creatinine clearance ratio varied between 1.3 and 5.8% (x 3.1 +/- 1.3). Serum amylase ranged from 36 to 460 U/l (x 123 U/l). Five patients had hyperamylasemia. 2 were urlian parotiditis, 2 were under clinical surveillance after surgery and the last one was a septic meningitis. All of them showed amylase-creatinine ratio values up to 2.5%. Serum lipase ranged from 6 to 197 U/l (x 79 U/l), falling into the normal interval. No abnormal clearance was found. CONCLUSIONS: hyperamylasemia is not specific for pancreatic disturbances in children; the serum lipase and the amylase-creatinine clearance ratio seem to be useful tools to rule out pancreatic involvement in hyperamylasemic patients.