In recent decades, treatment for cystic fibrosis (CF) has greatly increased the life span of those suffering from the genetic disorder. When the Cystic Fibrosis Foundation (CFF) was established in 1955, individuals born with the disease were not expected to live to attend elementary school. Today, because of the efforts of the foundation, people with CF are living into their 30s, 40s, and beyond. CF victims have lung and digestive problems due to the thick secretion. Among other problems is lack of growth. As with any disorder, early diagnosis and treatment is of utmost importance. A new study compared pubertal and adult height among CF patients who were screened a s newborns and those whose CF was diagnosed after symptoms appeared.
The goal of the study was to assess the long-term growth benefit of newborn screening, adolescent peak height velocity, and adult height. Those parameters were compared between the screened (diagnosed early via newborn screening) and the control (identified generally by symptoms) in the Wisconsin Randomized Clinical Trial. The study comprised data from 107 children born from 1985 through 1994 and followed through 2012 were analyzed. Peak height velocity was estimated and compared between the two groups.
The investigators found that meconium ileus (nonfunctioning bowel due to thick meconium secretions; 25 subjects) was associated with the poorest pubertal growth and adult height, including one child who did not experience apparent peak height velocity; children with pancreatic sufficiency (18) achieved the best growth (normal peak height velocity and adult height). In children with pancreatic insufficiency without meconium ileus (64), the subgroup most likely to benefit from newborn screening, screened children had similar peak height velocity but better adult height compared to controls. Specifically, among boys, the screened group (22) achieved normal peak height velocity (9.5 cm at 13.5 years); the control group (19) had similar onset age (13.6 years) but 0.6-cm lower growth. Among girls, the screened group (10) had somewhat later (12.5 years vs. 11.7 years) and lower peak height velocity (7.3 cm vs. 7.9 cm) than the controls (13), coinciding with later menarche (onset of menses; 13.6 years vs. 12.2 years). Adult height was taller in the screened than the control. Differences in adult height were primarily due to newborn screening and better prepubertal growth.
