Long-Term Outcome of Infants with Severe Chronic Kidney Disease

Background and objectives: In 2000, we reported the outcome of 101 children with a GFR <20 ml/min per 1.73 m(2) at 0.3 yr of age (range 0.0 to 1.5 yr). Long-term data on such young children are scarce. Design, setting, participants, & measurements: Mortality, treatment modalities, and growth were reanalyzed 9.9 yr later. Results: Of the 101 patients, 28 died and three were lost to follow-up during 13.90 yr (range 0.03 to 22.90 yr). One-, 2-, 5-, 10-, 15-, 20-, and 22-yr survivals were 87, 81, 77, 75, 73, 72, and 64%, respectively. Fifty-one children had comorbidities. Sixty-six percent were tube fed for 1.7 yr (range 0.1 to 6.9 yr), 37% had a gastrostomy, and 13% had a Nissen funcloplication. Mean height SD score (SD) was -0.42 (2.33) at birth (n = 40), -2.07 (1.34) at 0.5 (n = 62), -1.93 (1.38) at 1 (n = 72), -1.14 (1.14) at 5 (n = 67), -1.04 (1.15) at 10 (n = 62), -1.84 (1.32) at 15 (n = 40), and -1.68 (1.52) at age >= 18 yr (n = 32). Comorbidities adversely influenced growth (P < 0.01) and final height (P = 0.02): Mean height SD score (SD) was -1.16 (1.38) in otherwise normal adults. Conclusions: Growth and final height in infants with severe chronic kidney disease are influenced by comorbidity. Intensive feeding and early transplantation resulted in a mean adult height within the normal range in patients without comorbidities. Overall mortality is comparable to that of older children. Clin J Am Soc Nephrol 5: 10-17, 2010. doi: 10.2215/CJN.05600809