Article ; Online: Inherited causes of exocrine pancreatic insufficiency in pediatric patients: clinical presentation and laboratory testing.
Critical reviews in clinical laboratory sciences
2023 Volume 60, Issue 5, Page(s) 366–381
Abstract: Pediatric patients with exocrine pancreatic insufficiency (EPI) have symptoms that include abdominal pain, weight loss or poor weight gain, malnutrition, and steatorrhea. This condition can be present at birth or develop during childhood for certain ... ...
Abstract | Pediatric patients with exocrine pancreatic insufficiency (EPI) have symptoms that include abdominal pain, weight loss or poor weight gain, malnutrition, and steatorrhea. This condition can be present at birth or develop during childhood for certain genetic disorders. Cystic fibrosis (CF) is the most prevalent disorder in which patients are screened for EPI; other disorders also are associated with pancreatic dysfunction, such as hereditary pancreatitis, Pearson syndrome, and Shwachman-Diamond syndrome. Understanding the clinical presentation and proposed pathophysiology of the pancreatic dysfunction of these disorders aids in diagnosis and treatment. Testing pancreatic function is challenging. Directly testing aspirates produced from the pancreas after stimulation is considered the gold standard, but the procedures are not standardized or widely available. Instead, indirect tests are often used in diagnosis and monitoring. Although indirect tests are more widely available and easier to perform, they have inherent limitations due to a lack of sensitivity and/or specificity for EPI. |
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MeSH term(s) | Infant, Newborn ; Humans ; Child ; Feces ; Pancreatic Elastase ; Exocrine Pancreatic Insufficiency/diagnosis ; Exocrine Pancreatic Insufficiency/genetics ; Pancreas/physiology ; Cystic Fibrosis/diagnosis ; Cystic Fibrosis/genetics ; Cystic Fibrosis/complications |
Chemical Substances | Pancreatic Elastase (EC 3.4.21.36) |
Language | English |
Publishing date | 2023-03-06 |
Publishing country | England |
Document type | Journal Article |
ZDB-ID | 280641-1 |
ISSN | 1549-781X ; 1040-8363 ; 0590-8191 |
ISSN (online) | 1549-781X |
ISSN | 1040-8363 ; 0590-8191 |
DOI | 10.1080/10408363.2023.2179968 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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