PEDIATRIC PULMONOLOGY, cilt.43, ss.634-637, 2008 (SCI İndekslerine Giren Dergi)
Cystic fibrosis (CF) is the most prevalent inheritable chronic disease in Caucasian children. The clinical syndrome of kwashiorkor is a well-recognized complication of CIF The edema of the face can be seen in kwashiorkor. As doll-like face is very rare and underestimated clinical presentation of CF patients complicated with hypoproteinemia we evaluated demographic features and laboratory characteristics of 5 patients diagnosed as CF with doll-like face. Methods: Between June 2005 and January 2008, 115 children were diagnosed as having CIF enrolled in our center Five infants that were diagnosed as CF with doll-like face before the age of 6 months participitated in study. Results: The incidence of doll-like face younger than 6 months of age were 9.4% in our center Forty-eight infants diagnosed as CIF without doll-like face before the age of 6 months participitated in the study as controls (group 2). Physical examination revealed doll-like face and pitting edema of lower extremities in group 1. Their weight and length were under the third centile. Laboratory findings of group 1 include: mean hemoglobin 7.6g/dl; mean total protein 4.4 g/dl; albumin 2.3 g/dl. When compared control group in order to; 11.4 g/dl (range 7.6-17.9); 6.2 g/dl (range 4.0-8.8); 4.7 g/dl (range 2.1-5.8), mean hemoglobin, total protein and albumin values were lower in group 1. Conclusion: In a subgroup of patients, doll-like face may be the presenting manifestation of CIF Especially in developing countries clinicians should be aware of in patients with malnutrition and doll-like face and CF should be considered in differential diagnosis.