释义 |
DictionarySeecystic fibrosisClarke-Hadfield syndrome
Clarke-Hadfield syndrome [klark had´fēld] congenital pancreatic infantilism, with hepatomegaly, bulky stools, and extensive atrophy of the pancreas in an undersized and underweight child.cys·tic fi·bro·sis , cystic fibrosis of the pancreas [MIM*219700] a congenital metabolic disorder in which secretions of exocrine glands are abnormal; excessively viscid mucus causes obstruction of passageways (including pancreatic and bile ducts, intestines, and bronchi), and the sodium and chloride content of sweat is increased throughout the patient's life; symptoms usually appear in childhood and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather. Detailed genetic mapping and molecular biology have been accomplished by the methods of reverse genetics; autosomal recessive inheritance, caused by mutation in the cystic fibrosis conductance regulator gene (CFTR) on chromosome 7q. Synonym(s): Clarke-Hadfield syndrome, fibrocystic disease of the pancreas, mucoviscidosis, viscidosisClarke-Hadfield syndrome (klahrk'had'feld) [Cecil Clarke, 20th-cent. Brit. physician; Geoffrey John Hadfield, Brit. pathologist, 1899–1968] An infrequently used eponym for cystic fibrosis. See: cystic fibrosisClarke, Cecil, 20th century English physician. Clarke-Hadfield syndrome - a congenital metabolic disorder in which secretions of exocrine glands are abnormal. Synonym(s): cystic fibrosis
Hadfield, Geoffrey, English physician, 1889-1968. Clarke-Hadfield syndrome - see under Clarke, Cecil |