OBJECTIVE: To study the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) levels in differential diagnosis of growth hormone deficiency (GHD). METHODS: To measure serum IGFBP-3 levels by RIA in nor...OBJECTIVE: To study the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) levels in differential diagnosis of growth hormone deficiency (GHD). METHODS: To measure serum IGFBP-3 levels by RIA in normal children and adolescents, GHD children and short-stature children without GHD. RESULTS: Serum level of IGFBP-3 in 129 children with untreated GHD and with no pubertal development was 1.6 +/- 0.9 mg/L, which was less than that in normal group of the same age, but overlapped with the normal children in Tanner stage I. After six-month treatment with recombinant human growth hormone (rhGH), serum level of IGFBP-3 in 59 GHD significantly increased from 1.3 +/- 0.7 mg/L to 2.7 +/- 0.9 mg/L, accompanied by an increase of body heights, growth velocities and serum level of IGF-1. Serum level of IGFBP-3 in 55 short-stature children without GHD was 3.3 +/- 2.2 mg/L, which was not significantly different from that in normal group. CONCLUSION: Serum IGFBP-3 level can reflect the status of GH secretion in children with GHD and is a useful marker for differential diagnosis of GHD.展开更多
Background Cystic fibrosis(CF)is an autosomal recessive genetic disorder caused by loss-of-function mutations in the CF transmembrane conductance regulator gene.CF-related pancreatic lesions are known to cause exocrin...Background Cystic fibrosis(CF)is an autosomal recessive genetic disorder caused by loss-of-function mutations in the CF transmembrane conductance regulator gene.CF-related pancreatic lesions are known to cause exocrine dysfunctions such as pancreatic insufficiency,and endocrine dysfunctions,including CF-related diabetes.In a previous study,we generated rabbits with CF using CRISPR/Cas9(Clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9)-mediated gene editing.Methods Rabbits with CF were subjected to histological analysis with a focus on CF-associated pancreatic lesions.Endocrine function-related assays were conducted to evaluate CF-related pancreatic endocrine disorders in these animals.Results We report that rabbits with CF develop spontaneous pancreatic lesions at a young age,characterised by pancreatic inflammation and fibrosis,vacuolar degeneration,epithelium mucus-secretory cell metaplasia and pancreatic duct dilation.The size of the pancreatic islets in the rabbits with CF is significantly smaller than that of the wild-type animals.Consistent with these pathological findings,young rabbits with CF exhibited signs of pancreatic endocrine-related disorders such as lower insulin levels and impaired glucose metabolism.Conclusions Our results suggest that the CF rabbit could serve as a valuable model for translational research on CF-related pancreatic endocrine dysfunction.展开更多
文摘OBJECTIVE: To study the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) levels in differential diagnosis of growth hormone deficiency (GHD). METHODS: To measure serum IGFBP-3 levels by RIA in normal children and adolescents, GHD children and short-stature children without GHD. RESULTS: Serum level of IGFBP-3 in 129 children with untreated GHD and with no pubertal development was 1.6 +/- 0.9 mg/L, which was less than that in normal group of the same age, but overlapped with the normal children in Tanner stage I. After six-month treatment with recombinant human growth hormone (rhGH), serum level of IGFBP-3 in 59 GHD significantly increased from 1.3 +/- 0.7 mg/L to 2.7 +/- 0.9 mg/L, accompanied by an increase of body heights, growth velocities and serum level of IGF-1. Serum level of IGFBP-3 in 55 short-stature children without GHD was 3.3 +/- 2.2 mg/L, which was not significantly different from that in normal group. CONCLUSION: Serum IGFBP-3 level can reflect the status of GH secretion in children with GHD and is a useful marker for differential diagnosis of GHD.
基金supported by National Institutes of Health grants DK134361(to KZ and JX).
文摘Background Cystic fibrosis(CF)is an autosomal recessive genetic disorder caused by loss-of-function mutations in the CF transmembrane conductance regulator gene.CF-related pancreatic lesions are known to cause exocrine dysfunctions such as pancreatic insufficiency,and endocrine dysfunctions,including CF-related diabetes.In a previous study,we generated rabbits with CF using CRISPR/Cas9(Clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9)-mediated gene editing.Methods Rabbits with CF were subjected to histological analysis with a focus on CF-associated pancreatic lesions.Endocrine function-related assays were conducted to evaluate CF-related pancreatic endocrine disorders in these animals.Results We report that rabbits with CF develop spontaneous pancreatic lesions at a young age,characterised by pancreatic inflammation and fibrosis,vacuolar degeneration,epithelium mucus-secretory cell metaplasia and pancreatic duct dilation.The size of the pancreatic islets in the rabbits with CF is significantly smaller than that of the wild-type animals.Consistent with these pathological findings,young rabbits with CF exhibited signs of pancreatic endocrine-related disorders such as lower insulin levels and impaired glucose metabolism.Conclusions Our results suggest that the CF rabbit could serve as a valuable model for translational research on CF-related pancreatic endocrine dysfunction.
