[Congenital chloride diarrhea]. / Kongenitale Chloriddiarrhöe.

Two siblings aged 18 months and 3 1/2 years with congenital chloride diarrhea are presented. This disease was first described by Gamble et al. and Darrow in 1945 and has not been observed to date in Switzerland. So far, 43 cases have been reported, half of them from Finland. The disease appears to be caused by an inborn defect of chloride transport across the mucous membranes of the distal ileum and colon. This defect leads to chronic liquid diarrhea. The clinical symptoms, diagnostic approach, and therapeutic problems and possibilities are discussed.

Adaptation of a single injection clearance method to physiological and pathophysiological facts. A review of data obtained in infancy and childhood.

A retrospective analysis of 395 51Chromiumethylenediaminotetraacetate single injection clearances performed in infants and children is presented. In 61% of infants and 30% of the children the clearance values were calculated on the basis of a plasma disappearance half time of the reference substance, which was longer than the standard study, i.e. on the basis of extrapolated data. Plasma creatinine and urea levels were found to be appropriate indicators for predicting the plasma disappearance half time of the marker substance. 14 additional patients were studied prospectively with a duration of the study predicted by means of the plasma creatinine and urea levels. In these patients, separate determinations of the clearances using either the data obtained during the standard time of procedure only, or the data of the entire study, clearly demonstrated that the clearances obtained by means of the standard procedure overestimated glomerular filtration rate. The analysis of the data in infants show that the plasma urea level is a reasonably good indicator for predicting the time schedule of the study whereas plasma creatinine should not be used. Additionally the retrospective data indicate that a prolongation of the study should be recommended in all infants. This study demonstrates the necessity and offers means of adapting the time schedule of isotope single injection clearances to physiological and pathophysiological facts.

Hemodialysis and renal transplantation in childhood: description of a program.

Over a 2-yr period eight kidneys have been transplanted in seven patients at the University Children's Hospital of Berne. Six of these patients are alive 4-24 mo after transplantation. The results seem encouraging and the procedure justified, especially in view of the chance of full rehabilitation, which is obviously less complete with chronic hemodialysis.