Exocrine pancreas insufficiency

Figure 12 Degree of exocrine pancreas insufficiency in various clinical conditions evaluated by (he indirect ponciedauiyl test N is the number of patients studied. (Fiom Ref. 5Z)...

The intragastric predigestion by gastric lipase [66] is not impaired in patients with exocrine pancreas insufficiency. For the completion of digestion in the... 

Enzyme replacement for exocrine pancreas insufficiency must be adjusted on an individual basis considering age, body weight, weight for height, growth rate, fat intake, supplementary energy needs (particularly for pulmonary function), and clinical as well as laboratory evidence of malabsorption. Clinical symptoms include abdominal pain, distension, intolerance of fatty foods, and character of stools (consistency, number, odor). A more objective assessment involves microscopic examination of stool for neutral and split fats, measurement of fecal chymotrypsin or elastase [96]. 

On US, the pancreas with CF is characteristically of an echogenic texture secondary to fatty infiltration (Fig. 4.17). An enlarged pancreas may be seen initially with a subsequent atrophy later in life. Pancreatic duct dilatation and calcifications may be seen. Small cysts (anechoic areas) without vascular communication can be identified. Although a hyperechogenic pancreas is very typical of CF, some other diseases such as Schwachman-Diamond syndrome (exocrine pancreas insufficiency associated with bone marrow dysfunction, cyclic neutropenia, metaphyseal diastasis and growth retardation), hemosiderosis, chronic pancreatitis, and administration of steroids may also reveal this feature (Feigelson et al. 2000). 

Casellas F, Guamer L, Vaquero E, Antolin M, de Gracia X, Malagelada JR I Iydrogen breath test with glucose in exocrine pancreatic insufficiency. Pancreas 1998 16 481 186. 

Exocrine pancreatic insufficiency is most commonly caused by cystic fibrosis, chronic pancreatitis, or pancreatic resection. When secretion of pancreatic enzymes falls below 10% of normal, fat and protein digestion is impaired and can lead to steatorrhea, azotorrhea, vitamin malabsorption, and weight loss. Pancreatic enzyme supplements, which contain a mixture of amylase, lipase, and proteases, are the mainstay of treatment for pancreatic enzyme insufficiency. Two major types of preparations in use are pancreatin and pancrelipase. Pancreatin is an alcohol-derived extract of hog pancreas with relatively low concentrations of lipase and proteolytic enzymes, whereas pancrelipase is an enriched preparation. On a per-weight basis, pancrelipase has approximately 12 times the lipolytic activity and more than 4 times the proteolytic activity of pancreatin. Consequently, pancreatin is no longer in common clinical use. Only pancrelipase is discussed here. 

Use Anal aim of exocrine pancreas substitution therapy is to eliminate malabsorption and to maintain adequate nutrition. Treatment of exocrine insufficiency of the pancreas ought to be easily achieved by administering an extract of mammalian (e.g., porcine) pancreas. Unfortunately, successful treatment is sometimes not easy to achieve and elimination of malabsorption can be difficult to maintain, notwithstanding the availability of potent porcine pancreatic extracts. Problems inherent in these pancreatin preparations themselves, like particularities in the environment in which the enzymes are expected to work, must also be considered. 

Complete agenesis of the pancreas is usually incompatible with life. Therefore, pancreatic aplasia or hypoplasia is uncommon in humans. Patients with pancreatic hypoplasia will have a normal development of the pancreas in size and shape but later in life will have a replacement of the glandular elements with fatty tissue. Some of these patients will present with an abnormal exocrine pancreatic insufficiency and normal endocrine function. Children with syndromes such as Schwachman-Diamond, Beckwith-Wiedemann, polysplenia bilobed lungs, and congenital heart disease have a higher relative risk of hypoplasia of the pancreas (Gazelle et al. 1998). 

Pancreas A study of pancreatic insufficiency in 233 HIV positive patients foxmd no association between didanosine use and exocrine pancreatic insufficiency (p = 0.43) in contrast to previous reports [153 ]. 

CP is an inflammatory condition that usually results in functional and structural damage to the pancreas. In most patients CP is progressive and loss of pancreatic function is irreversible. Permanent destruction of pancreatic tissue usually leads to exocrine and endocrine insufficiency. Cystic fibrosis may be associated with pancreatic exocrine insufficiency in children and is discussed in Chap. 30. 

S. Scharp6 and L. Iliano. A composition for the treatment of exocrine insufficiency of the pancreas, and the use of said composition. European Patent No. 387945. 

The pancreas serves as a secretory- (exocrine) and hormone- (endocrine) producing organ. Exocrine functions are difficult to assess in clinical studies in healthy infants but, as noted above, can be assessed by directly quantifying lumenal concentrations of enzymes and bicarbonate before and after a stimulus. It is only when pancreatic exocrine secretion is dramatically decreased that a deceleration of growth velocity occurs (Huynh and Couper, 2000). Severe pancreatic insufficiency can be monitored by measnring fat or certain enzymes (e.g., trypsin) in stools. Pancreatic endocrine dysfunction is most often manifested as diabetes, which can be assessed by obtaining serum insulin concentrations, blood, and urine glucose (Huynh and Couper, 2000). 

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