Text Box: PERINATAL FEATURES OF CONGENITAL CHLORIDE DIARRHEA IN PRETERM INFANTS Sayed MH1, Azab A2, Salah ME2 1 Pediatric Department, Cairo University, Egypt 2 National Research Center, Cairo, Egypt Objective: Congenital chloride diarrhea (CCD) is a rare autosomal recessive inherited disorder due to defective chloride-bicarbonate exchange in the distal ileum and colon. To increase awareness of this disorder, the perinatal features as well as the biochemical findings of 7 Kuwaiti infants with congenital chloride diarrhea are reported. Methods: Over the 4-year period from January 1995 to December 1998, 7 cases of CCD were diagnosed in one regional hospital in Kuwait, where about 6000 non-selected deliveries take place yearly. Medical records of all 7 cases diagnosed as CCD were retrospectively reviewed. Antenatal and early neonatal features as well as biochemical findings at the time of diagnosis were all recorded. Results: All infants were delivered prematurely, of low birth weight, and showing features of intrauterine growth retardation. Polyhydramnios and dilated fetal bowel loops on antenatal ultrasound have been found in all 7 cases reported. The serum abnormalities observed are those of moderate to severe hypochloremia, hyponatremia, and hypokalemic metabolic alkalosis. The stool chloride concentrations always exceeded that of the serum chloride and were greater than 100 mmol/L in all 7 patients. Hyperreninemia and hyperaldosteronism were found in all cases. Conclusion: This report has shown that CCD is not uncommon in Kuwaiti infants. With the help of antenatal ultrasound examination, more cases of congenital chloride diarrhea can be detected in the neonatal period. Early recognition of such cases allows for appropriate therapy and prevention of associated complications. 1156