Nutrition and Growth in Cystic Fibrosis

“…Study of mice with a neuronspecific deletion of Cftr would help elucidate the role of neuronal Cftr. Second, chronic inflammation may also contribute to growth retardation in CF (7). There is evidence that CF patients have elevated systemic cytokines (37,38) and CF mice have elevated expression of inflammatory mediators in the intestine (39).…”

Section: Growth In Cystic Fibrosis Micementioning

confidence: 99%

Mouse as a Model of Growth Retardation in Cystic Fibrosis

et al. 2006

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Cystic fibrosis (CF) is an autosomal recessive disease that results in lung failure and premature death. A long recognized symptom of CF is growth failure, which is clinically relevant because it correlates with the severity of lung disease. We describe growth retardation in a mouse model of CF and discuss its potential for modeling certain aspects of human growth retardation. Mice with a null mutation in Cftr (cystic fibrosis transmembrance conductance regulator) were compared with wild-type (WT) mice at 31, 45, and 84 d of age. CF mice were severely growth retarded in weight and length compared with wild-type controls. Serum insulin like growth factor I (Igf-1) was lower in CF mice by 31-55% (depending on age and sex) and it significantly correlated with the size of mice after controlling for gender, age, and Cftr genotype. There was a marginally significant deficiency of serum growth hormone (Gh) in CF females, but not males. Our findings were consistent with models of an energy deficit in rodents. We, therefore, assessed food intake and found no difference between CF and WT mice, suggesting that CF mice had a malabsorption-mediated energy deficit. We argue that CF mice are suited to study the effects of intestinal disease on growth as well as other proposed growth-modulating processes. (Pediatr Res 59: [191][192][193][194][195] 2006)

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“…In the pancreas, activation of Ca 2ϩ signaling by G protein-coupled receptors (GPCRs) 1 plays a central role in digestive enzyme secretion (3). Abnormal enzyme secretion can lead to numerous diseases, including malnutrition in cystic fibrosis (4) and, more commonly, acute pancreatitis (5). Several studies have shown a strong association between aberrant Ca 2ϩ signaling and pancreatitis (6 -8).…”

Section: Camentioning

confidence: 99%

Aberrant Localization of Intracellular Organelles, Ca2+ Signaling, and Exocytosis in Mist1 Null Mice

Luo

Shin

Wang

et al. 2005

Journal of Biological Chemistry

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Ca2؉ signaling and exocytosis are highly polarized functions of pancreatic acinar cells. ؊/؊ cells. Deletion of Mist1 also led to mislocalization of the Golgi apparatus and markedly reduced digestive enzyme content. The combination of aberrant Ca 2؉ signaling and reduced digestive enzyme content resulted in poor secretion of digestive enzymes. Yet, food consumption and growth of Mist1 ؊/؊ mice were normal for at least 32 weeks. These findings reveal that Mist1 is critical to normal organelle localization in exocrine cells and highlight the critical importance of maintaining cellular architecture and polarized localization of cellular organelles in generating a propagating apical-to-basal Ca 2؉ wave. The studies also reveal the spare capacity of the exocrine pancreas that allows normal growth and development in the face of compromised exocrine pancreatic function.

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Nutrition and Growth in Cystic Fibrosis

Cited by 26 publications

References 32 publications

Respiratory training with a specific device in cystic fibrosis: A prospective study

Respiratory training with a specific device in cystic fibrosis: A prospective study

Mouse as a Model of Growth Retardation in Cystic Fibrosis

Aberrant Localization of Intracellular Organelles, Ca2+ Signaling, and Exocytosis in Mist1 Null Mice

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