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ISSN 0185-3252 (Print)
Revista de la Asociación Médica del Centro Médico ABC

2016, Number 4

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An Med Asoc Med Hosp ABC 2016; 61 (4)

Meconium ileus as first manifestation of cystic fibrosis

Tinajero IMG, Lamshing SP, Casaubon GP, Hernández AEJ

Language: Spanish
References: 17
Page: 305-309
PDF size: 290.79 Kb.


ABSTRACT

Cystic fibrosis is the most common lethal inherited disease in white population, it is inherited as an autosomal recessive pattern. Most patients are diagnosed in children under 1 year. Meconium ileus is the earliest symptom of this disease and occurs in 6-20% of cases. We present the case of a preterm female newborn, with distended intestinal loops by prenatal ultrasound. At birth a severe meconium ileus was confirmed with preserved pancreatic and liver functions. She underwent surgical treatment at 18 hours of life and a diagnosis of cystic fibrosis was confirmed on sweat chloride test greater than 60 mEq/L. It has been reported that cases of cystic fibrosis presenting with meconium ileus during the neonatal period are associated with more complications and poor long-term prognosis. Treatment should be multidisciplinary with close monitoring in intensive care. It has been reported that early diagnosis and early management can offer a better prognosis for these patients.


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