Isolated pancreatic amylase deficiency: Probable error in maturation

Mehta, Devendra I.; Wang, Hugh H.; Akins, R.E.; Wang, Lisa; Proujansky, Roy

doi:10.1016/s0022-3476(00)96936-1

Cited by 8 publications

(3 citation statements)

References 10 publications

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“…By comparison, about 10% of a carbohydrate load is malabsorbed by patients with chronic pancreatitis [184]. Patients with isolated pancreatic amylase deficiency, a rarely reported condition, have carbohydrate maldigestion that may produce abdominal distension, flatulence, loose stools, and poor weight gain [185][186][187][188]. Symptoms resolve with pancreatic enzyme replacement therapy.…”

Section: Human Studiesmentioning

confidence: 99%

Human Pancreatic Digestive Enzymes

2007

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A primary function of the pancreas is to produce digestive enzymes that are delivered to the small intestine for the hydrolysis of complex nutrients. Much of our understanding of digestive enzymes comes from studies in animals. New technologies and the availability of the sequence of the human genome allow for a critical review of older reports and assumptions based on animal studies. This report updates our understanding of human pancreatic digestive enzymes with a focus on new insights into the biology of human proteases, lipases and amylases.

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Section: Human Studiesmentioning

confidence: 99%

Human Pancreatic Digestive Enzymes

2007

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“…Isolated amylase deficiency is usually due to a maturational defect in young children and resolves by 2–3 years of age and does not produce clinical symptoms as the functional capacity of salivary amylase is enough to compensate well to prevent symptoms. 142 …”

Section: Individual Syndromes Related To Epi In Childrenmentioning

confidence: 99%

Exocrine Pancreatic Insufficiency in Children – Challenges in Management

Sankararaman,

Schindler

2023

PHMT

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Cystic fibrosis (CF) is the leading etiology for exocrine pancreatic insufficiency (EPI) in children, followed by chronic pancreatitis, Shwachman-Diamond syndrome, and other genetic disorders. Management of EPI in children poses several unique challenges such as difficulties in early recognition, lack of widespread availability of diagnostic tests and limited number of pediatricspecific pancreatic centers. Pancreatic enzyme replacement therapy is the cornerstone of EPI management and in young children difficulties in administering pancreatic enzymes are frequently encountered. Patients with EPI also should be screened for fat-soluble vitamin deficiencies and receive appropriate supplementation. Among disorders with EPI in children, CF is the relatively well-studied condition, and most management recommendations for EPI in children come from expert consensus and conventional practice guidelines. The impact of EPI can be greater in children given their high metabolic demands and rapid growth. Early diagnosis and aggressive management of EPI prevent consequences of complications such as malnutrition, fat-soluble vitamin deficiencies, and poor bone health and improve outcomes. Management by multi-disciplinary team is the key to success.

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“…Additionally, isolated amylase deficiency was identified in a large retrospective pediatric database of endoscopic pancreatic function testing (ePFT) ( 63 ). An error in mRNA processing or protein secretion was suggested by Mehta et al in a reported pediatric case with isolated amylase deficiency diagnosed after repeated ePFTs (20 and 33 months of age), despite detecting normal pancreatic amylase messenger RNA by reverse-transcriptase polymerase chain reaction in the duodenal fluid ( 64 ). Understanding of isolated pancreatic enzyme deficiencies as pathologic or physiologic is overall limited and represents area for future research.…”

Section: Assessment Of Exocrine Functionmentioning

confidence: 99%

Assessment of exocrine pancreatic function in children and adolescents with direct and indirect testing

et al. 2022

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The exocrine pancreas plays an important role in digestion. Understanding of the physiology and regulation of exocrine function provides insight into disease processes and basis of functional testing. Specifically, exocrine pancreatic insufficiency (EPI) can cause maldigestion and thus a proper assessment of exocrine pancreatic function is important. There are indirect and direct methods for evaluating pancreatic function. Indirect methods are varied and include stool, serum, urine, and breath tests. Fecal elastase is a commonly used indirect test today. Direct methods involve stimulated release of pancreatic fluid that is collected from the duodenum and analyzed for enzyme activity. The most used direct test today is the endoscopic pancreatic function test. Indirect pancreatic function testing is limited in identifying cases of mild to moderate EPI, and as such in these cases, direct testing has higher sensitivity and specificity in diagnosing EPI. This review provides a comprehensive guide to indirect and direct pancreatic function tests as well as an in-depth look at exocrine pancreatic function including anatomy, physiology, and regulatory mechanisms.

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Isolated pancreatic amylase deficiency: Probable error in maturation

Cited by 8 publications

References 10 publications

Human Pancreatic Digestive Enzymes

Human Pancreatic Digestive Enzymes

Exocrine Pancreatic Insufficiency in Children – Challenges in Management

Assessment of exocrine pancreatic function in children and adolescents with direct and indirect testing

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