Clinical analysis of 11 children with pancreatic cystic fibrosis
10.3760/cma.j.issn.0578-1310.2017.05.013
- VernacularTitle: 儿童胰腺囊性纤维化11例临床分析
- Author:
Guoli WANG
1
,
2
,
3
;
Chunna ZHAO
;
Jin ZHOU
;
Feihong YU
;
Huiqing SHEN
;
Jing ZHANG
;
Shunying ZHAO
;
Xiwei XU
Author Information
1. Department of Gastroenterology, Beijing Children′s Hospital
2. Faculty of Digestive Diseases, Capital Medical University
3. National Clinical Research Center for Digestive Diseases, Beijing 100045, China
- Publication Type:Clinical Trail
- Keywords:
Cystic fibrosis;
Child;
Clinical features
- From:
Chinese Journal of Pediatrics
2017;55(5):373-376
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To increase the recognition of pancreatic cystic fibrosis (PCF) in children and facilitate diagnosing and treatment of this rare entity.
Method:This is a retrospective analysis of children who presented to Beijing Children′s Hospital affiliated to Capital Medical University from January 2010 to December 2015. We describe their clinical features, laboratory testing and management.
Result:Eleven children were diagnosed with PCF by genetic testing or sweat chloride test during these 5 years, including 4 boys and 7 girls. Their age ranged from 0.5-14.3 (mean 9.0±3.9) years. Family history was positive in 3 children. Significant clinical findings on presentation were: malnutrition 6, including 2 cases of mild, moderate and severe malnutrition each; diarrhea 4 (yellow mushy or watery stool with frequency ranging from 2-5 times a day), including 1 case of acute diarrhea and 3 of chronic diarrhea, 3 of them had steatorrhea; abdominal pain 3. All of them had pancreatic lesions shown by abdominal ultrasound. Blood tests showed 6 cases had elevated serum amylase and lipase. The main treatment was pancreatic replacement therapy and nutritional support.
Conclusion:PCF is rare in children. Malnutrition, diarrhea and abdominal pain are the main clinical manifestations. Treatment is mostly pancreatic enzymes replacement and supportive care.
