1582 autonomic dysfunction associated with secretory diarhea: a new syndrome

ABSTRACT A 1 year old male had features of autonomic dysfunction including lack of axon flare in response to intradermal histamine, abnormal pupillary response to conjunctival instillation

of methacholine, absent fungiform papillae of the tongue, lack of overflow tears, decreased response to deep pain, and a history of recurrent aspiration pneumonias and failure to thrive. The

patient differed from individuals with familial dysautonomia (FD) in that 1) he was not of Jewish heritage, 2) sweating was absent, 3) corneal reflexes were present, 4) temperature and

vasomotor instability were absent. The patient differed from individuals with congenital sensory neuropathy with anhidrosis (CSN) in that 1) overflow tearing and fungiform papillae of the

tongue were absent, 2) deep tendon reflexes were present, and 3) methacholine caused prompt pupillary miosis. True secretory diarrhea is a feature of neither FD or CSN, but was present in

our patient. During a 9-day fast stool volume averaged 20 gm/kg/day. Stool electrolytes were Na 111 mEq/L, K 26 mEq/L, Cl 55 mEq/L. There was no evidence of malabsorption. Enteropathic

bacteria and parasites were absent from the stool. No histologic abnormalities were evident in biopsies of the small bowel or rectum. The diarrhea improved during treatment with tincture of

opium. Studies in animals and man have demonstrated that the autonomic nervous system contributes to the control of secretion from the small bowel and colon. This is the first report of a

syndrome of a congenital autonomic dysfunction associated with secretory diarrhea. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Pediatrics, UCLA Medical Center,

Los Angeles Paul E Hymen, Melvin B Heyman, Edward J Feldman, William E Berquist & Marvin E Ament Authors * Paul E Hymen View author publications You can also search for this author

inPubMed Google Scholar * Melvin B Heyman View author publications You can also search for this author inPubMed Google Scholar * Edward J Feldman View author publications You can also search

for this author inPubMed Google Scholar * William E Berquist View author publications You can also search for this author inPubMed Google Scholar * Marvin E Ament View author publications

You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Hymen, P., Heyman, M., Feldman, E. _et al._

1582 AUTONOMIC DYSFUNCTION ASSOCIATED WITH SECRETORY DIARHEA: A NEW SYNDROME. _Pediatr Res_ 15 (Suppl 4), 707 (1981). https://doi.org/10.1203/00006450-198104001-01599 Download citation *

Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-01599 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content: Get shareable

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