Tugba Sismanlar1, Ayşe Tana Aslan2, Mehmet Köse3, Sevgi Pekcan4, Fatih Süheyl Ezgü5, Işıl İrem Budakoğlu6, İdil Yenicesu7. 1. Pediatric Pulmonology Department, Gazi University Faculty of Medicine, Besevler, 06500, Ankara, Turkey. 2. Pediatric Pulmonology Department, Gazi University Faculty of Medicine, Besevler, 06500, Ankara, Turkey. aysetugbapp@gmail.com. 3. Pediatric Pulmonology Department, Erciyes University Faculty of Medicine, Kayseri, Turkey. 4. Pediatric Pulmonology Department, Necmettin Erbakan University, Meram Medicine Faculty, Konya, Turkey. 5. Pediatric Metabolism and Genetic Department, Gazi University Faculty of Medicine, Ankara, Turkey. 6. Medical Education Department, Gazi University Faculty of Medicine, Ankara, Turkey. 7. Pediatric Hematology Department, Gazi University Faculty of Medicine, Ankara, Turkey.
Abstract
UNLABELLED: 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. CONCLUSION: 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. WHAT IS KNOWN: • 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.
UNLABELLED: 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. CONCLUSION: 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. WHAT IS KNOWN: • 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: P M Farrell; M R Kosorok; A Laxova; G Shen; R E Koscik; W T Bruns; M Splaingard; E H Mischler Journal: N Engl J Med Date: 1997-10-02 Impact factor: 91.245
Authors: M S Marcus; S A Sondel; P M Farrell; A Laxova; P M Carey; R Langhough; E H Mischler Journal: Am J Clin Nutr Date: 1991-09 Impact factor: 7.045
Authors: Jie Xu; Alessandra Livraghi-Butrico; Xia Hou; Carthic Rajagopalan; Jifeng Zhang; Jun Song; Hong Jiang; Hong-Guang Wei; Hui Wang; Mohamad Bouhamdan; Jinxue Ruan; Dongshan Yang; Yining Qiu; Youming Xie; Ronald Barrett; Sharon McClellan; Hongmei Mou; Qingtian Wu; Xuequn Chen; Troy D Rogers; Kristen J Wilkinson; Rodney C Gilmore; Charles R Esther; Khalequz Zaman; Xiubin Liang; Michael Sobolic; Linda Hazlett; Kezhong Zhang; Raymond A Frizzell; Martina Gentzsch; Wanda K O'Neal; Barbara R Grubb; Y Eugene Chen; Richard C Boucher; Fei Sun Journal: JCI Insight Date: 2021-01-11