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Doll‐like face: Is it an underestimated clinical presentation of cystic fibrosis?
Author(s) -
Kose Mehmet,
Pekcan Sevgi,
Kiper Nural,
Aslan Ayse Tana,
Cobanoglu Nazan,
Yalcin Ebru,
Dogru Deniz,
Ozcelik Ugur
Publication year - 2008
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20819
Subject(s) - medicine , cystic fibrosis , kwashiorkor , hypoproteinemia , gastroenterology , pediatrics , complication , incidence (geometry) , edema , surgery , malnutrition , physics , optics
Cystic fibrosis (CF) is the most prevalant inheritable chronic disease in caucasian children. The clinical syndrome of kwashiorkor is well‐recognized complication of CF. 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 CF enrolled in our center. Five infants 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 the 6 months of age were 9.4% in our center. 48 infants diagnosed as CF without doll‐like face before the age of 6 months participitated in the study as controls (group2). 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 CF. 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. Pediatr Pulmonol. 2008; 43:634–637. © 2008 Wiley‐Liss, Inc.