M. Boney Dr., Rhode Island Hasbro Children's Hospital of CST2 Endocrinology and Metabolism, director of Charlotte in the American Academy of Pediatrics (AAP) Pediatric Conference organized by the report, boys hypergonadotropic hypogonadism, the most common cause - Klinefelter levy, affecting 1/660 of the men, the diagnosis rate is very low."75% of children with Klinefelter syndrome until the adult, none of access to diagnosis, and thus missed the opportunity to CST3take interventions to promote normal adolescent development." Newborn with Klinefelter syndrome appear completely normal, but afterchildhood, there are many diagnostic opportunities. The most common danger signs include: cryptorchidism, involving 25% to 40% of children with Klinefelter syndrome men; language development delay, more than 40% of children with symptoms; learning disabilities, involving more than 75% of children ; emotional and behavioral problems such as attention deficit hyperactivity disorder, involving more than 25 percent of the children. Klinefelter syndrome usually presented in CST4 kindergarten and preschool children with social adjustment problems between peers.The Boney Dr. said: "If a boy is 1 year old testicular yet scrotum, after which there appeared to speak later, is a" destructive elements "in kindergarten, learning disabilities in three age or grade performance, you should take into account the g the possibility of Chiari syndrome. "she said, in view of a boy at the age the testicular yet scrotum far less than 10 percent, when she could not touch the testicles will be referred to the urology before its nuclear type check to rule out CST5 Klinefelter syndrome.
Klinefelter syndrome is characterized by delayed puberty, and endocrinology have demonstrated increased serum follicle-stimulating hormone and luteinizing hormone levels, testosterone levels below their respective Tanner staging. The majority of the boys involved will continue to start puberty, but it will gradually slows until stagnation. The diagnosis of Klinefelter syndrome need to be corroborated by karyotype analysis.Approximately 90% of patients with CST6Klinefelter syndrome 47xxy 10% 47xxy/46xy less than 1% 48xxy.Klinefelter syndrome is very clear: testosterone replacement therapy to achieve normal pubertal development. These objectives include the normal trajectory of rapid growth and puberty; normal male muscle mass, body fat distribution and bone mineralization; induce secondary sexual characteristics; social and psychological well-being. Boys in puberty normal masculine intramuscular injection of small doses of long-acting testosterone ester therapy should begin at an initial dose of 50 mg every 3 to 4 weeks and gradually increased every 2 to 3 weeks from 200 to 300 in the period of 1 year mg.Once children enter the 4 Tanner, range and serum testosterone levels rose to more than 300 mg / dl, the transition to the maintenance treatment of percutaneous testosterone patch. Children will be very happy into maintenance therapy, because the patch can use, and brought about by fluctuations in serum testosterone levels is smaller than the intramuscular injection. Most children are more willing to paste the patch in the arm, thigh or back, rather than attached to the scrotum. Smear testosterone gel on the skin every day, is also a choice of alternative therapies. Using the gel during the serum hormone levels are very stable, but these products are cleaning up too much trouble.
Although the correct diagnosis and treatment of Klinefelter syndrome will be completely normal sexual function, but there is no way to prevent children with germ cell death. In order to preserve the possibility of offspring, many large centers has begun for patients with Klinefelter syndrome provide the service of the testicular biopsy to extract healthy sperm, the sperm will be stored for future assisted reproductive. For children with Klinefelter syndrome, looking for healthy sperm time window is very narrow, about 16 to 20 years of age must be biopsied, they are often not yet mature in children with emotional, would have to discuss with the extraction of sperm problem.
Condividi e segnala 
