Reproductive hormones, bone mineral content, body composition, and testosterone therapy in boys and adolescents with Klinefelter syndrome

Adult patients with Klinefelter syndrome (KS) are characterized by a highly variable phenotype, including tall stature, obesity, and hypergonadotropic hypogonadism, as well as an increased risk of developing insulin resistance, metabolic syndrome, and osteoporosis. Most adults need testosterone replacement therapy (TRT), whereas the use of TRT during puberty has been debated. In this retrospective, observational study, reproductive hormones and whole-body dual-energy x-ray absorptiometry-derived body composition and bone mineral content were standardized to age-related standard deviation scores in 62 patients with KS aged 5.9–20.6 years. Serum concentrations of total testosterone and inhibin B were low, whereas luteinizing hormone and follicle-stimulating hormone were high in patients before TRT. Despite normal body mass index, body fat percentage and the ratio between android fat percentage and gynoid fat percentage were significantly higher in the entire group irrespective of tr eatment status. In patients evaluated before and during TRT, a tendency toward a more benefi cial body composition with a significant reduction in the ratio between android fat pe rcentage and gynoid fat percentage during TRT was found. Bone mineral content (BMC) did not differ from the reference, but BMC corrected for bone area was significantly low er when compared to the reference. This study confirms that patients with KS have an unf avorable body composition and an impaired bone mineral status already during childhood and adolescence. Systematic studies are needed to evaluate whether TRT during puberty will improve these parameters

Anesthetic Considerations in the Evaluation of Children with Glaucoma and Associated Conditions

Glaucoma in the pediatric population can be difficult to diagnose and treat without a child’s cooperation. Examination and intervention under anesthesia play a critical role in the management of childhood glaucoma, as detailed elsewhere. This requires a change in setting from the office to the operating room with the availability and assistance of a pediatric anesthesiologist, who may have to sedate or anesthetize the child repeatedly, sometimes even just to confirm the glaucoma diagnosis. An anesthesiologist with specialty training in pediatrics is pertinent, as childhood glaucoma can often be associated with syndromes involving other organ systems, hence making their anesthetic complicated and at times potentially dangerous. Once the glaucoma diagnosis is established, these children may have to return to the operating room multiple times for examinations and/or treatments; thus, having prior anesthetic records with any previous complications can offer vital information to both the pediatric anesthesiologist and pediatric glaucoma specialist. This chapter focuses on the preoperative workup of pediatric patients to ensure that they are optimized medically to undergo sedation and general anesthesia with the lowest possible risk. The intraoperative course is also described, including why sedation may be chosen over general anesthesia and how the various anesthetic drugs such as volatile anesthetics or intravenous induction agents can affect intraocular pressure measurements. The chapter finishes by describing potential postoperative complications related to anesthesia: nausea, vomiting, postoperative delirium, and agitation, along with a brief discussion on various syndromes that could potentially affect the delivery and choice of anesthetic