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      A Review of the Management of Two Common Clinical Problems Found in Patients with Cystic Fibrosis: Cystic Fibrosis-Related Diabetes and Poor Growth

      Hormone Research in Paediatrics

      S. Karger AG

      Cystic fibrosis, Diabetes, Growth

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          Background: Cystic fibrosis-related diabetes (CFRD) and glucose intolerance often occur in teens and adults with CF. The initial deficiency is an impaired first-phase insulin response; as patients age, peak insulin response is delayed and less robust than normal. Decreased insulin sensitivity (insulin resistance) is also present in patients with CF. Insulin is the only currently recommended therapy for all types of CFRD, and many clinicians find that basal/bolus regimens are optimal. Another common finding in children with CF is poor linear growth and inadequate weight gain. Recombinant human growth hormone (rhGH) is being explored in this patient population; studies overall show improvements in height and weight, without development of glucose intolerance. Conclusions: rhGH may be a useful growth-promoting therapy in children with CF.

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          Most cited references 22

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          The HER-2-targeting antibodies trastuzumab and pertuzumab synergistically inhibit the survival of breast cancer cells.

          Trastuzumab (herceptin) and pertuzumab (Omnitarg, 2C4) are recombinant humanized monoclonal antibodies that target different extracellular regions of the HER-2 tyrosine kinase receptor. We explored combination effects of these agents in the HER-2-overexpressing BT474 breast cancer cell line. Trastuzumab and 2C4 synergistically inhibited the survival of BT474 cells, in part, because of increased apoptosis. Trastuzumab increased 2C4-mediated disruption of HER-2 dimerization with the epidermal growth factor receptor and HER-3. Combination drug treatment reduced levels of total and phosphorylated HER-2 protein and blocked receptor signaling through Akt but did not affect mitogen-activated protein kinase. These results suggest that combining HER-2-targeting agents may be a more effective therapeutic strategy in breast cancer rather than treating with a single HER-2 monoclonal antibody.
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            A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto

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              Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis.

              To determine the relation of growth and nutritional status to pulmonary function in young children with cystic fibrosis (CF). The relation of weight-for-age (WFA), height-for-age (HFA), percent ideal body weight (%IBW), and signs of lung disease at age 3 years with pulmonary function at age 6 years was assessed in 931 patients with CF. Associations of changes in WFA from age 3 to 6 on pulmonary function were also assessed. WFA, HFA, and %IBW were poorly associated with lung disease at age 3 years, but all were strongly associated with pulmonary function at age 6 years. Those with WFA below the 5th percentile at age 3 had lower pulmonary function at age 6 compared with those above the 75th percentile (FEV(1): 86 +/- 20 [SD] versus 102 +/- 18 % predicted, respectively). Pulmonary function was highest in those whose WFA remained >10th percentile from age 3 to 6 (FEV(1): 100 +/- 19 % predicted) and lowest in those who remained <10th percentile (84 +/- 21 % predicted). Patients with signs and symptoms of lung disease at age 3 years had lower pulmonary function at age 6 years. Aggressive intervention early in life aimed at growth and nutrition and/or lung disease may affect pulmonary function.

                Author and article information

                Horm Res Paediatr
                Hormone Research in Paediatrics
                S. Karger AG
                December 2007
                10 December 2007
                : 68
                : Suppl 5
                : 113-116
                Division of Pediatric Endocrinology, Columbus Children’s Hospital, Ohio State University, Columbus, Ohio, USA
                110603 Horm Res 2007;68:113–116
                © 2007 S. Karger AG, Basel

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                Page count
                References: 40, Pages: 4
                Pediatric Clinical Case Sessions


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