title: Early severe anemia as the first sign of cystic fibrosis.
abstract: Severe anemia is reported to occur rarely in patients with cystic fibrosis (CF). This study aimed to determine the factors associated with early severe anemia in infants with CF. This study included 231 infants with CF from 3 pediatric CF centers ten year period that were retrospectively reviewed in terms of severe anemia as the first sign of CF. Factors that could affect anemia, such as age, pancreatic insufficiency, mutations, vitamin A and E, and albumin level were evaluated. Clinical and laboratory findings in CF patients that presented with severe anemia and no respiratory symptoms were compared to those in CF patients that did not present with severe anemia. Severe anemia as the first sign of CF was noted in 17 of 231 patients. Patient age, prolonged PT/INR and the albumin level differed significantly between the 2 groups of patients (P < 0.001). Feeding pattern, pancreatic insufficiency, vitamin E and A levels, and the types of genetic mutations did not differ between the 2 groups. The mean hemoglobin level was 5.59 ± 0.21 g/dL and respiratory symptoms began a mean 6.3 months after diagnosis of CF in the anemia group.
  
  
  CONCLUSIONS
  In early infancy severe anemia in the absence of respiratory symptoms can be the first sign of CF. CF should be considered in the differential diagnosis of severe anemia in infants. Anemia can occur several months before respiratory symptoms in patients with CF and may be caused due to several reasons.
  
  
  BACKGROUND
  • Severe anemia as a first sign is reported to occur rarely in patients with cystic fibrosis. • Although anemia is well known in cystic fibrosis, factors that cause severe anemia are not known clearly. What is New: • This study shows the importance of severe anemia as the first sign of cystic fibrosis. • Anemia can occur several months before respiratory symptoms in patients with CF.
authors: Pediatric Pulmonology Department, Gazi University Faculty of Medicine, Besevler, 06500, Ankara, Turkey.
authors: Tugba
authors: T
authors: Sismanlar
authors: Pediatric Pulmonology Department, Gazi University Faculty of Medicine, Besevler, 06500, Ankara, Turkey. aysetugbapp@gmail.com.
authors: Ayşe Tana
authors: AT
authors: Aslan
authors: Pediatric Pulmonology Department, Erciyes University Faculty of Medicine, Kayseri, Turkey.
authors: Mehmet
authors: M
authors: Köse
authors: Pediatric Pulmonology Department, Necmettin Erbakan University, Meram Medicine Faculty, Konya, Turkey.
authors: Sevgi
authors: S
authors: Pekcan
authors: Pediatric Metabolism and Genetic Department, Gazi University Faculty of Medicine, Ankara, Turkey.
authors: Fatih Süheyl
authors: FS
authors: Ezgü
authors: Medical Education Department, Gazi University Faculty of Medicine, Ankara, Turkey.
authors: Işıl İrem
authors: Iİ
authors: Budakoğlu
authors: Pediatric Hematology Department, Gazi University Faculty of Medicine, Ankara, Turkey.
authors: İdil
authors: İ
authors: Yenicesu
mesh_terms: D017668:Age of Onset; D000740:Anemia / Q000209:etiology* / Q000628:therapy; D016022:Case-Control Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies
keywords: Anemia; Cystic fibrosis; Infant