SUMMARY One hundred and fourteen children with primary nephrotic syndrome were followed up prospectively for periods of between 5 and 14 years. Urine samples from 94 of them became proteinfree during the initial 8-week course of prednisone, and the outcome for these children was good: 74 of them have been free of symptoms for at least 3 years, 18 have had relapses during the last 3 years, and only one child still has proteinuria. All these children have normal renal function and blood pressure. One child died accidentally. Twenty children did not respond to the initial prednisone treatment. Thirteen of them had remissions later, of whom 2 have had relapses during the last 3 years. Seven were totally resistant to prednisone 4 of whom died in renal failure, the remaining 3 have persistent proteinuria with normal levels of creatinine; one has high blood pressure too. Remission during the initial treatment indicated a good prognosis, but two-thirds of the initial non-responders also fared well.

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SUMMARY Clinical findings in 21 Finnish children with congenital chloride diarrhoea are reported. Inheritance of this disease by the autosomal recessive mode is established. All children were born 1-8 weeks prematurely. Hydramnios was present in every case and no meconium was observed; intrauterine onset of diarrhoea is thus apparent. In most cases the diarrhoea or passing of large volumes of 'urine' was noted on the first day of life and the abdomen was usually large and distended. The neonatal weight loss was abnormally large, and was associated with hypochloraemia and hyponatraemia. Some infants survived the neonatal period without adequate therapy. They presented later with failure to thrive and usually had hypochloraemia, hypokalaemia, and metabolic alkalosis associated with hyperaldosteronism. However, these features may be absent and the diagnosis is based on a history of hydramnios and diarrhoea, and a faecal Cl-concentration which always exceeds 90 mmol/l when fluid and electrolyte deficits have been corrected. Lower faecal C1-concentrations were seen only in chronic hypochloraemia, which is also associated with achloriduria.Adequate treatment consists of full continuous replacement of the faecal losses of water, NaCl, and KCI. This should be given intravenously in the early neonatal period; later a solution can be taken orally with meals. The dose has to be adjusted to maintain normal serum electrolyte concentrations, normal blood pH, and some chloriduria. This therapy prevents the renal lesions and the retarded growth and psychomotor development which were seen in the children who were diagnosed late and in those who received inadequate replacement therapy. The watery diarrhoea persists and increases slightly with age, though patients learn to live with their disease and to make an adequate social adjustment.In 1945 Gamble et al. and Darrow both described a child who had persistent watery diarrhoea, a high faecal concentration of C1-, hypochloraemia. hypokalaemia, and metabolic alkalosis, and they diagnosed the illness as 'congenital alkalosis with diarrhoea'. Since then 19 more cases have been reported from outside Finland: from the United

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