Osteogenesis imperfecta (OI) is a rare disorder of type 1 collagen with 13 currently identified types attributable to inherited abnormalities in type 1 collagen amount, structure, or processing. The disease is characterized by an increased susceptibility to bony fracture. In addition to the skeletal phenotype, common additional extraskeletal manifestations include blue sclerae, dentinogenesis imperfecta, vascular fragility, and hearing loss. Medical management is focused on minimizing the morbidity of fractures, pain, and bone deformities by maximizing bone health. Along with optimizing Vitamin D status and calcium intake and physical/occupational therapy, individualized surgical treatment may be indicated. Pharmacological therapy with bisphosphonate medications is now routinely utilized for moderate to severe forms and appears to have a good safety profile and bone health benefits. New therapies with other anti-resorptives as well as anabolic agents and transforming growth factor (TGF)β antibodies are in development. Other potential treatment modalities could include gene therapy or mesenchymal cell transplant. In the future, treatment choices will be further individualized in order to reduce disease morbidity and mortality.

Metformin is a widely prescribed medication that has been used to treat children with type 2 diabetes in the United States for the past 15 years. Metformin now has a variety of clinical applications in pediatrics, and its potential clinical uses continue to expand. In addition to reviewing the current understanding of its mechanisms of action including the newly discovered effects on the gastrointestinal tract, we will also discuss current clinical uses in pediatrics, including in type 1 diabetes. Finally, we examine the existing state of monitoring for metformin efficacy and side effects and discuss prospective future clinical uses.

Parents of young children with type 1 diabetes (T1DM) maintain full responsibility for their child’s daily diabetes self-care and thus may be vulnerable to experiencing parenting stress. This study examined several psychological correlates of pediatric parenting stress in parents of young children with T1DM. Parents of 39 young children with T1DM (ages 2–7 years) completed measures of pediatric parenting stress, mealtime behavior problems, depressive symptoms, and fear of hypoglycemia. For parents of young children, higher stress frequency and difficulty were associated with higher parental depressive symptoms and fear. Regression analyses identified that 58% of the variance in stress frequency was associated with parental depressive symptoms. For stress difficulty, 68% of the variance was associated with parental depressive symptoms and fear. Pediatric parenting stress is common in parents of young children with T1DM. Stress and the psychological correlates measured in this study are amenable to intervention and should be regularly assessed in parents of young children with T1DM.

BACKGROUND.The purpose of the current study was to determine the prevalence of low bone mineral density (BMD) (ie, osteopenia) and identify factors associated with low BMD in young adult survivors of childhood acute lymphoblastic leukemia (ALL).METHODS.Dual energy x‐ray absorptiometry was used to evaluate BMD in 74 randomly selected, long‐term childhood ALL survivors initially treated in Minneapolis/St. Paul, Minnesota. Growth hormone (GH)‐releasing hormone–arginine stimulation testing was conducted to evaluate peak GH level, and insulin‐like growth factor I (IGF‐I) and other markers of endocrine functioning were also evaluated in relation to BMD.RESULTS.The mean age at the time of interview was 30 years, and the mean time since diagnosis was 24 years. Low BMD (Z‐score, ≤−1) was present in 24% of subjects, including 1 with osteoporosis. Low BMD was substantially more prevalent in men than in women and was strongly associated with short height. The mean height Z‐score for those with low BMD was −1.44, compared with a height Z‐score of −0.39 (P < .01) for those with normal BMD. GH insufficiency, low IGF‐I Z‐score, and current smoking were also suggestive risk factors for low BMD.CONCLUSIONS.In this long‐term follow‐up study of childhood ALL survivors, low BMD was found to be more prevalent than expected based on population normative data, specifically in men. The health consequences of early‐onset BMD problems in childhood ALL survivors need to be carefully monitored. Cancer 2008. © 2008 American Cancer Society.