Abstract
The functional defects in CMA appear to be in the mucosa itself. 10 fragments of small intestine from 3 patients with CMA were studied in vitro to determine electrical parameters (short-circuit current, Isc, and ionic conductance, G) and net ionic and glucose fluxes (Jnet). db cAMP (1mM) stimulated Isc and net Cl− secretion; somatostatin 14 (10−6 M) did not alter Isc and stimulated Na and Cl absorption; ouabain (0.1mM) inhibited Isc; glucose (10mM) stimulated Isc and Na absorption. The intracellular glucose accumulation was low (C/M = 2.6 ± 0.3 vs 15.8 ± 1.5 in control at 10mM glucose in the medium); it was further reduced by phloridzin and ouabain. Peroxydase permeability and G were within control range. The results indicate that the jejunal epithelium of CMA patients is not leaky; the basic transport processes are present (Na-K ATPase, glucose-Na cotransport, Cl− secretion). We speculate that the epithelial abnormality is an imbalance between decreased absorption and unaltered secretion.
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Desjeux, J., Grasset, E., Navarro, J. et al. TRANSPORT PROPERTIES OF ISOLATED JEJUNAL EPITHELIUM FORM CHILDREN WITH CONGENTIAL MICROVILLUS ATROPHY (CMA). Pediatr Res 20, 703 (1986). https://doi.org/10.1203/00006450-198607000-00108
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DOI: https://doi.org/10.1203/00006450-198607000-00108