Positive fertility outcomes in a female with classic congenital adrenal hyperplasia following bilateral adrenalectomy.

Background: Classic congenital adrenal hyperplasia (CAH) requires lifetime steroid replacement and supraphysiologic glucocorticoid dose is often required for adequate adrenal androgen suppression. Patients often suffer from long-term co-morbidities and female infertility is common.

Case Presentation: We report the use of laparoscopic bilateral adrenalectomy as a treatment for a 21 year old female with classic simple virilizing CAH and infertility.

Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type I.

Objective: To assess the relationship between pubertal progression and change in plexiform neurofibroma (PN) burden over time in pediatric and young adult patients with neurofibromatosis type 1 and PNs.

Study Design: Analyses accounted for sex, age, race, and chemotherapy. Forty-one patients with neurofibromatosis type 1 (15 female and 26 male patients) were studied at the National Institutes of Health.

Bone mineral density in children and young adults with neurofibromatosis type 1.

Concern for impaired bone health in children with neurofibromatosis type 1 (NF-1) has led to increased interest in bone densitometry in this population. Our study assessed bone mineral apparent density (BMAD) and whole-body bone mineral content (BMC)/height in pediatric patients with NF-1 with a high plexiform neurofibroma burden. Sixty-nine patients with NF-1 (age range 5.

(111)In-octreotide scintigraphy for identification of metastatic medullary thyroid carcinoma in children and adolescents.

Context: Most medullary thyroid cancers (MTC) express somatostatin receptors; therefore, (111)In-octreotide somatostatin receptor scintigraphy (SRS) may be useful in detecting sites of metastases in children with MTC.

Objective: The aim of the study was to evaluate tumor metastases in children and adolescents with MTC using SRS in comparison to conventional imaging.

Design And Setting: A case series was conducted as part of baseline evaluation for cancer treatment protocol at the National Institutes of Health Clinical Center.