Elmer S. Lightner

Growth hormone excess and sexual precocity in polyostotic fibrous dysplasia (McCune-Albright syndrome): Evidence for abnormal hypothalamic function

A 5-5/12-year-old boy with gigantism and precocious puberty associated with the McCune-Albright syndrome is presented. Serum concentrations of growth hormone were extremely elevated (128-163 ng/ml) and were not suppressed by hyperglycemia or chlorpromazine. Serum LH (7.4 +/- 1.0 SD mIU/ml) and FSH (5.3 +/- 0.3 SD mIU/ml) concentrations were in the range observed in midpuberty. The secretion pattern of LH was episodic. The administration of estradiol suppressed the secretion of FSH and had an apparent positive feedback effect on release of LH. These findings are compatible with abnormal hypothalamic function as the mechanism for the endocrinopathies associated with the McCune-Albright syndrome.

X‐short arm deletion gonadal dysgenesis in two siblings due to unique translocation (Xp‐;16p+)

A family demonstrating short arm deletion of the X chromosome as a consequence of X‐16 balanced translocation in the mother is reported. The two Xp‐ sisters exhibit clinical signs of gonadal dysgenesis, while the balanced carriers are phenotypically normal. To our knowledge this represents the only example of both the balanced carrier state for an X translocation and its genetic consequence occurring in the offspring, as well as the involvement of X‐16 interchange. Literature data of 37 additional cases of verified X translocations are discussed.

Phenotypic malformations in association with maternal trimethadione therapy.

demonst ra ted . Sulkin and associates ~2 repor ted two pat ients who developed orchitis and significant rises in ant ibody titer after accidental laboratory infection with bat salivary gland virus a group B arbovirus. Orchitis has also been no ted in association with influenza, dengue, ph lebo tomus fever, smal lpox and varicella, and possibly echovirus 11 and vaccinia infect ions? Since bo th aseptic meningi t is and orchitis are f requent manifesta t ions of m a n y enteroviral infections, it is suprising that the dual occurrence has no t been previously reported. More f requent uti l ization of viral diagnost ic services would p robab ly reveal o ther cases of orchitis associated with enterovira l infections. Echovirus 6 infect ion should be considered when orchitis, with or wi thout meningitis, occurs in a pa t ient with a history of previous , mumps vaccinat ion or na tu ra l infection; re infect ion with mumps virus must also be considered.

Bromocriptine-unresponsive prolactin macroadenoma in a prepubertal female

Macroprolactinomas occur rarely in prepubertal children. A prepubertal 11 10/12-year-old female with a macroprolactinoma presented with a diagnosis of anorexia nervosa. Findings included severe headaches, anorexia, weight loss, and growth failure. The initial serum prolactin level was 2,916 ng/ml. This value fell after beginning treatment with bromocriptine, but later rose despite a doubling of the dose. Transsphenoidal surgical resection was performed and postoperative radiation given. The use of bromocriptine, surgical resection, and radiation therapy in the treatment of macroprolactinomas are discussed.

Vincent A. Fulginiti, MD (1931-2013): A Visionary Leader in Pediatric Education, Research, and Bioethics.

Vincent A. Fulginiti, MD was internationally recognized as a Pediatric Infectious Disease expert. Vincent A. Fulginiti, MDwas a leader in medical education and a mentor to many. Vincent A. Fulginiti, MD published extensively in the fields of pediatric infectious disease, medical education and medical ethics. Vincent A. Fulginiti, MD maintained the highest level of personal and professional ethics and was beloved by his family, his friends and his faculty.

The Diabetic Child and Young Adult

The Diabetic Child and Young Adult , by Dr Mimi M. Belmonte, will be a welcome addition to the library of families who have children with type 1 diabetes mellitus. The table of contents is well organized and the index is well done. All sections of the book are well written; particularly helpful are the sections on control of diabetes and on tests to evaluate diabetic control. The glossary of terms is especially helpful to nonprofessionals. This book is a welcome addition to previous discussions of this chronic and complicated illness of infants, children, and young adults.