Hyponatremic Dehydration as a Presentation of Cystic Fibrosis

• Published in: Pediatric emergency care Vol. 22; no. 11; pp. 725 - 727
• Main Authors: Ballestero, Yolanda, Hernandez, María Isabel, Rojo, Pablo, Manzanares, Javier, Nebreda, Verónica, Carbajosa, Helena, Infante, Elena, Baro, María
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins, Inc 01-11-2006; Lippincott Williams & Wilkins
• Subjects: Alkalosis - etiology; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Chlorides - blood; Cystic Fibrosis - complications; Cystic Fibrosis - diagnosis; Cystic Fibrosis - epidemiology; Dehydration - etiology; Diagnostic Tests, Routine - statistics & numerical data; Female; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Hyponatremia - etiology; Infant; Infant, Newborn; Intensive care medicine; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Medical sciences; Neonatal Screening; Other diseases. Semiology; Pneumology; Prevalence; Respiratory system : syndromes and miscellaneous diseases; Retrospective Studies; Seasons; Spain - epidemiology; Sweat - chemistry; Sweating; Vomiting - etiology; Weight Loss; Spain; Human; Hydroelectrolytic balance disorder; Intensive care; Hyponatremia; Respiratory disease; Chlorine; Metabolic alkalosis; Metabolic diseases; Cystic fibrosis; Acid base balance disorder; Dehydration; Inorganic element; Genetic disease; Metabolic disorder; Hypochloremia; Sodium; Digestive diseases; Child; Resuscitation; Pancreatic disease
• ISSN: 0749-5161; 1535-1815
• DOI: 10.1097/01.pec.0000245170.31343.bb

BACKGROUND:The purpose of this study is to present a case report of a child with hyponatremic dehydration diagnosed after CF and to review the cases of 13 patients with CF who had the same initial presentation in our hospital.
METHODS:This report reviewed the clinical records of children diagnosed with CF to ascertain the prevalence of metabolic alkalosis with electrolyte depletion as the presentation of CF. It also used sweat tests to diagnose a child with CF.
RESULTS:The laboratory tests of a 12-month-old girl presented 3 times to the `pediatric emergency department with vomiting and weight loss showed hyponatremia, hypochloremia, and metabolic alkalosis. The patient was subsequently diagnosed with CF by means of 2 positive sweat tests. Meanwhile, the review of the clinical records of all children diagnosed with CF from 1985 to 2004 (N = 77) showed that the prevalence of metabolic alkalosis with electrolyte depletion as the presentation of CF was 16.8%. The age of the infants ranged from 3 to 14 months. All episodes took place during summer.
CONCLUSIONS:There are not many causes of metabolic alkalosis with hyponatremic dehydration, and one of them is CF. This report emphasizes sodium depletion as a common sign of CF presentation. This is most important in countries where the neonatal screening test for CF is not available because the disease may be asymptomatic or oligosymptomatic for several months or even years. Cystic fibrosis should be considered in differential diagnosis of any child presenting with unexplained hyponatremic dehydration.