Abstract
21 children with CCD have been treated by us since the disease was first diagnosed in Finland in 1961. All were born from a pregnancy complicated by hydramnios /2–6 l/, and 19/21 2 or more weeks before term. Watery diarrhea /about 1000 ml/m2/day/ was present from the first day of life, with Cl concentration of the fecal fluid exceeding 100 meq/1. Absence of meconiurn was recorded in 14/21. Hyponatremia and hypochloridemia tended to develop, and later hypopotassemia and alkalosis with extreme dehydration. The diarrhea only decreased with dehydration. Normal fluid composition and hydration could be maintained through adequate water and electrolyte replacement. For this a solution of NaCl and KCl was used. The dose and Na/K-ratio were adjusted to maintain normal pH and electrolyte concentration, chloriduria, normal renin and aldosterone activity and normal body content of potassium. Normal development was thus achieved and renal changes could be avoided.
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