alpha-chymotrypsin and Hypothyroidism

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

Both synthesis and degradation of proteins are reduced in the hypothyroid state. The possible involvement of serum trypsin and chymotrypsin inhibitors has been studied in a family that, for four successive generations, had clinical or subclinical hypothyroidism. Increased trypsin- and chymotrypsin-inhibiting activity was demonstrated in the sera of the four clinically hypothyroid women. Cellulose acetate electrophoresis of the sera of these patients and of two other subclinical hypothyroid family members disclosed the distinct appearance of an additional fraction in the alpha 2-globulin zone. The serum protein electrophoretic pattern changes observed in familial hypothyroidism might be genetically determined. Such changes could precede the clinical onset of the disease, thus serving as a possible indicator of the hypothyroid state.

Topics: Adult; Aged; Child, Preschool; Chymotrypsin; Female; Humans; Hypothyroidism; Male; Middle Aged; Pedigree; Protease Inhibitors; Trypsin Inhibitors

Rat cardiac ventricular myosins were obtained from fetuses, from young normal animals, and from hypophysectomized adults. The purified proteins were compared by several techniques: (i) electrophoresis in non-denaturing conditions (pyrophosphate buffer), (ii) one- and two-dimensional analysis after proteolytic cleavage, (iii) immunological blotting after electrophoretic purification, and (iv) competitive enzyme-linked immunosorbent assay. Antibodies specific to each of the two major isoenzymes of adult rat heart (V1 and V3 according to the terminology of Hoh et al. (Hoh, J. F. Y., McGrath, P. A., and Hale, P. T. (1978) J. Mol. Cell. Cardiol. 10, 1053-1076) were used for the immunological studies. The heavy chains of the ventricular myosin isoenzymes of fetuses (V3F) were indistinguishable from those of the V3H isoenzyme present in hypophysectomized adults; both proteins differed from the V1 isoform of young animals. The light chains of V3F, V3H, and V1 were the same, except that V3F contained in addition a small amount of the embryonic light chain (Whalen, R. G., and Sell, S. M. (1980) Nature 286, 731-733). These results strongly suggest that adaptation of the adult rat heart to the hormonal deficiencies of hypophysectomy is mediated by the synthesis of the same myosin heavy chain form which is predominant in fetal hearts.

Topics: Aging; Animals; Chymotrypsin; Electrophoresis, Polyacrylamide Gel; Enzyme-Linked Immunosorbent Assay; Female; Fetus; Heart; Hypophysectomy; Hypothyroidism; Myocardium; Myosins; Peptide Fragments; Pregnancy; Rats; Rats, Inbred Strains