alpha-chymotrypsin and Growth-Disorders

Exocrine pancreatic insufficiency in children can lead to lifelong complications related to malnutrition and poor growth. The clinical presentation can be subtle in the early stages of insufficiency as the large functional capacity of the pancreas is gradually lost. The pediatrician plays a crucial role in the early identification of these children to ensure a timely referral so that a diagnosis can be made and therapy initiated. Early nutritional therapy allows for prevention and correction of deficiencies, which leads to improved outcomes and survival. When insufficiency is suspected, the workup should start with an indirect test of exocrine pancreatic function, such as fecal elastase, to establish the diagnosis. Once a diagnosis is established, further testing to delineate the etiology should be pursued, with cystic fibrosis being high on the differential list and assessed for with a sweat test. Assessment of anthropometry at every visit is key, as is monitoring of laboratory parameters and physical examination findings that are suggestive of malabsorption and malnutrition. The mainstay of management is administration of exogenous pancreatic enzymes to facilitate digestion and absorption. [Pediatr Ann. 2019;48(11):e441-e447.].

Topics: Acyl-CoA Dehydrogenase, Long-Chain; Anus, Imperforate; Child; Child Nutrition Disorders; Chymotrypsin; Congenital Bone Marrow Failure Syndromes; Cystic Fibrosis; Dietary Fats; Ectodermal Dysplasia; Enzyme Replacement Therapy; Exocrine Pancreatic Insufficiency; Feces; Growth Disorders; Hearing Loss, Sensorineural; Humans; Hypothyroidism; Intellectual Disability; Lipid Metabolism, Inborn Errors; Mitochondrial Diseases; Muscular Diseases; Nose; Nutrition Assessment; Pancreas; Pancreatic Diseases; Pancreatic Elastase; Pancreatic Function Tests; Pancreatitis, Chronic; Shwachman-Diamond Syndrome; Steatorrhea; Trypsinogen

The effect of supplementing a cornsoybean diet (C) with glucose (G) or maltose (M) on young broilers (from hatch to 3 wk of age) affected by stunting syndrome (SS) was studied. Stunting syndrome was induced by orally administering an inoculum prepared from the intestines of SS broiler chicks. Relative to the M diet, the G diet improved growth and feed utilization and increased feed intake in naive (NA) control chickens. The C diet was intermediate in this respect. In contrast to the NA chickens, diet did not affect growth or feed utilization in SS chicks. Changes in the relative weights of the gastrointestinal tract segments were evident by 1 wk of age and hypertrophy of these segments persevered to 3 wk of age. Stunting syndrome infection was accompanied by a significant increase in pancreatic trypsin-specific activity during Weeks 1 and 2, and in chymotrypsin activity at 1 wk. During this time, amylase-specific activity was not affected. At 3 wk of age, the specific activities of amylase, trypsin, and chymotrypsin in the pancreas were lower in the inoculated vs control birds. Whereas no significant effect of SS was observed with activities of amylase in the intestinal contents, trypsin activity was higher in SS chicks at 1 wk, and that of chymotrypsin lower during Weeks 2 and 3. Relative to NA chicks, the maltase and saccharase activities of SS chicks were much lower during Week 1, but increased later on and were similar to NA chick values at 2 and 3 wk. Whereas the level of blood plasma proteins did not vary from 1 to 3 wk in the NA chicks, it increased gradually in SS chicks to a level that significantly exceeded that in their NA counterparts. Blood plasma glucose and triglyceride levels were slightly lower in the SS chicks (NS), and the blood plasma cholesterol level was significantly reduced during Week 2. Relative to NA chicks, SS infection caused a significant increase in plasma calcium during Weeks 2 and 3, accompanied by a significant reduction in blood plasma phosphorus at 2 wk only. No difference was observed in the blood plasma level of uric acid, which peaked in both treatments during Week 2, or in D-beta-hydroxybutyric acid level, which was quite stable during the experimental period. Stunting syndrome infection was accompanied by a dramatic increase in amylase and alkaline phosphatase activities in the blood plasma, and by a slight but significant decrease in activity of lactic dehydrogenase. Stunting syndrome was concluded to be an afflic

Topics: Aging; alpha-Glucosidases; Amylases; Animal Feed; Animals; Blood Proteins; Body Weight; Calcium; Chickens; Chymotrypsin; Digestive System; Eating; Food, Fortified; Glucose; Growth Disorders; Intestinal Mucosa; Intestines; Lipids; Male; Maltose; Organ Size; Phosphorus; Poultry Diseases; Sucrase; Syndrome; Tissue Extracts; Trypsin

Topics: Amylases; Carboxypeptidases; Chromatography; Chromatography, Gel; Chymotrypsin; Diarrhea, Infantile; Duodenum; Edema; Endopeptidases; Female; Growth Disorders; Humans; Hypoproteinemia; Infant, Newborn; Intestinal Secretions; Lipase; Metabolism, Inborn Errors; Trypsin

Topics: Amylases; Anemia; Body Weight; Carboxypeptidases; Child, Preschool; Chronic Disease; Chymotrypsin; Diet Therapy; Duodenum; Edema; Exocrine Glands; Feces; Female; Growth Disorders; Humans; Hypoproteinemia; Intestinal Secretions; Lipase; Lipid Metabolism, Inborn Errors; Lipids; Metabolism, Inborn Errors; Nitrogen; Pancreas; Pancreatin; Psychomotor Disorders; Trypsin