GH improves growth and clinical status in children with cystic fibrosis -- a review of published studies

Abstract

Children with cystic fibrosis (CF) have problems with poor linear growth and inadequate weight gain. Nutritional augmentation has been the mainstay of therapy for improving both weight and height in CF; however, inadequate growth continues to be a problem. Furthermore, protein catabolism has been documented even in non-acutely ill adults and children with CF, and could adversely affect longitudinal growth. Human recombinant GH has positive effects on nitrogen balance, and multiple studies have demonstrated improved height and weight in children treated with GH. The purpose of this article is to summarize studies evaluating GH use in children with CF. All published studies of GH use in children with CF have demonstrated significant improvement in height velocity and height Z score. All studies but one, in which subjects were treated only three times per week with GH, have demonstrated improvement in weight as reported by weight velocity and/or weight Z score, and one trial has demonstrated a substantial improvement when GH was used to augment nutritional therapy. Several reports suggest that GH treatment results in improved forced vital capacity, and multiple studies have found improved clinical status as measured by decreased hospitalizations and courses of intravenous antibiotics. Furthermore studies to date also suggest that GH results in improvement in exercise tolerance and bone accumulation. To date significant side effects, including glucose intolerance, have not been reported. Thus mounting evidence suggests that human recombinant GH provides safe and effective therapy in children with CF.

Mouse as a Model of Growth Retardation in Cystic Fibrosis

“…(2003) showed that growth parameters at age 3 positively predict lung function at age 6 in CF patients (3). Further evidence that growth stimulation is beneficial to CF patients is that treatment with GH not only stimulates growth and elevates serum Igf-1 levels, but may also improve clinical status (5). For example, a randomized, controlled trial found that administration of GH to CF patients improves clinical status as measured by lung capacity and reduced hospital visits (4).…”

“…For example, a randomized, controlled trial found that administration of GH to CF patients improves clinical status as measured by lung capacity and reduced hospital visits (4). It is suggested that the beneficial effects of growth hormone may be attributed to increased respiratory muscle strength and larger absolute lung capacity (5).…”

“…CF patients are also growth retarded (1)(2)(3), which is clinically relevant not only because it affects the quality of life for patients, but also because the severity of growth retardation correlates with the severity of lung disease (2,3). Furthermore, studies in which CF patients have benefited from chronically administered growth hormone suggest that generation of a growthpromoting physiologic state may improve lung function and clinical status (4,5).…”

Recombinant Human Growth Hormone in the Treatment of Patients With Cystic Fibrosis

“…The dominant clinical features are chronic lung disease and pancreatic insufficiency with poor nutrition and growth. 3,4 Treatment advances in CF over the past 25 years have improved measures of nutrition, pulmonary function, and mortality. The median age of survival has improved consistently from 1955 to the most recent data in 2008.…”

Hormônio de crescimento em crianças e adolescentes com fibrose cística

“…O processo inflamatório crônico tanto afeta a produção e a secreção do hormônio de crescimento (GH) como inibe o crescimento em nível tecidual (7,8). Dessa maneira, na FC, a suplementação nutricional isolada pode ser insuficiente para ocasionar a recuperação adequada do peso e da estatura.…”