Article Text

  1. S. A. Ali,
  2. R. A. Vidal
  1. University of South Alabama, Mobile


Background Cystic fibrosis is an inherited disease that is a significant cause of severe respiratory disease and failure to thrive in the pediatric population. The key to diagnosis of CF is first to have a high index of suspicion.

Case Report We report a 7-month-old black female with a history significant for failure to thrive, who presented to our facility with acute respiratory failure requiring intubation and mechanical ventilation. Past medical history was significant for hospitalization at 4 months of age for respiratory syncytial virus (RSV) pneumonia and respiratory failure. Both of her parents were of African American descent and her two older siblings had no history of infections or failure to thrive. On admission to our facility the patient appeared to be undernourished with a weight less than 5% for age. Initial laboratory examination revealed total white blood count 13,900/mm3 with 65% granulocytes, 20% bands, 9% lymphocytes, PT 20.5 sec. Chest radiography revealed bilateral interstitial infiltrates. Respiratory cultures obtained via the endotracheal tube grew methicillin-sensitive Staphylococcus aureus and Enterobacter. A workup for immunodeficiency and failure to thrive was undertaken. A sweat chloride test was preformed and was reported to be 95 mmol/L (normal range, 0-40 mmol/L). The patient was started on total parenteral nutrition and pancreatic enzymes. A high calorie formula was subsequently introduced with good results. The baby was successfully extubated and was weaned to room air. At the time of discharge, the patient was tolerating her formula well and had gained 500 grams.

Discussion Cystic fibrosis is an autosomal recessive disorder in which carriers of the gene are asymptomatic. CF is most often thought of as a disease affecting those of Caucasian descent. Children who present with failure to thrive with or without respiratory symptoms should have a sweat chloride test performed as part of their workup, regardless of race or family history.

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