Biomedical subjects
Publications and source records attributed to R L Bejar.
A child with neurofibromatosis exhibited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical homeostasis but had no effect on tumor growth. Radiation therapy arrested tumor enlargement and stabilized deteriorating visual function.
A 7-year-old girl presented with the physical and endocrinological stigmata of Cushing's disease. An adrenocorticotropic hormone (ACTH)-producing pituitary microadenoma was excised. Three weeks after trans-sphenoidal adenomectomy, the patient developed benign intracranial hypertension. Although ACTH levels had decreased to normal, the serum cortisol had fallen to subnormal levels. The child responded to exogenous steroid therapy, which was gradually tapered and discontinued after 5 months. Normal pituitary and adrenal functions persist 2 years later.
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A neonate without external malformation had undergone removal of a nasopharyngeal mass containing anterior and posterior pituitary tissue. Numerous endocrine evaluations since that time have confirmed polyhormonal hypopituitarism. Endocrine replacement has resulted in a healthy, normal child except for short stature. The literature dealing with pharyngeal pituitary, congenital absence of the pituitary, cyclopia, and other malformations of the pituitary suggests only vague theories to explain the infrequent anomalies found in this area. We propose that laminagrams of the sphenoid of newborns with sighus may aid in early diagnosis and appropriate replacement therapy.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Encephalocraniocutaneous lipomatosis is a rare hamartomatosis involving the craniofacial region and the central nervous system. The most prominent clinical features are large areas of scalp alopecia, soft subcutaneous craniofacial masses, lipomas, connective tissue nevi of the eyelids and surrounding areas, pterygium-like choriostoma of the ocular conjunctiva, mental retardation, motor deficit, and seizures. Of the eight patients reported previously, three had spinal cord evaluations and two had evidence of lipomatosis. We report the third patient with this association, review the literature of encephalocraniocutaneous lipomatosis, and stress the importance of spinal cord evaluation during the newborn period.