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CLARKE-HADFIELD SYNDROME

A generalised mess of infants, children and young adults, in which there is widespread dysfunction of the exocrine glands, characterised by signs of chronic pulmonary malady (due to excess mucus manufacture in the respiratory tract), pancreatic deficiency, abnormally tall levels of electrolytes in the sweat and occasionally by biliary cirrhosis. There is an ineffective immunologic defense against bacteria in the lungs. Pathologically, the pancreas shows obstruction of the pancreatic ducts by amorphous eosinophilic concretions, with consequent deficiency of pancreatic enzymes, resulting in steatorrhoea and azotorrhoea and intestinal malabsorption. The degree of involvement of organs and glandular systems may vary greatly, with consequent variations in the clinical picture. Inheritance: autosomal recessive.
tunica mucosa bronchiorum   tunica mucosa cavitatis tympani   tunica mucosa coli   tunica mucosa ductus deferentis   tunica mucosa gastrica   tunica mucosa intestini tenuis   tunica mucosa laryngis   tunica mucosa linguae   (0)
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