XXXX in the first year of life.

@entity1 @entity8 @entity66 @entity600 @entity177 @entity2916 @entity74
To determine the frequency of @entity74 ( @entity8 ) in @entity1 and to compare the rate of progression of these conditions, we conducted a retrospective survey of 48 @entity1 who were seen with @entity8 before 1 year of age and who survived the first month of age. Seventeen @entity1 had recessive @entity8 ; six had dominant @entity8 . Eighteen @entity1 had insufficient data to categorize the type of @entity8 with certainty. Seven @entity1 were classified as "other"; three had @entity66 and the remainder had multiple @entity600 or @entity2916 . Renal ultrasonography and excretory urography accurately detected 15 of 17 @entity1 with recessive @entity8 , but only one @entity1 with dominant @entity8 was correctly diagnosed by excretory urography. The majority of @entity1 in all groups required antihypertensive therapy. The 17 @entity1 with recessive @entity8 have been followed up for 6.1 +/- 4.3 (SD) years. Eight @entity1 are doing well. Two @entity1 have died; five others have required treatment for @entity177 . Only one @entity1 has an estimated glomerular filtration rate within the normal range after 6 years of age. Long-term evaluation of most of the @entity1 with dominant @entity8 is not yet available; however, by age 42 months one @entity1 has required dialysis. To provide optimum genetic counseling and accurate diagnosis for @entity1 with @entity8 , a combination of careful family evaluation, radiography, and liver or kidney biopsy is required. The outcome of @entity1 who survive the neonatal period appears not to be so grim as previously feared, underscoring the importance of aggressive supportive care and the need for physician and family education.
