Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pituitary dwarfism is a genetically heterogeneous group of disorders associated with a variety of pathogenetic mechanisms which may involve any level of the hypothalamic-pituitary-somatomedin-peripheral tissue axis. Developmental defects of the pituitary, such as congenital absence of the pituitary, and developmental defects of the hypothalamus, such as anencephaly and holoprosencephaly, have been described. Degeneration of the hypothalamus and pituitary, as in histiocytosis X, may also result in pituitary dwarfism. In "idiopathic" forms of pituitary dwarfism, defects limited to either the hypothalamus or pituitary have been suggested by releasing hormone stimulation studies and it is quite likely that specific defects of the hypothalamus, and pituitary, as well as defects in releasing hormonal synthesis and secretion and growth hormone synthesis and secretion all exist. In Laron dwarfism the basic defect appears to lie in the ability to generate somatomedin, in the Pygmy the defect appears to involve peripheral unresponsiveness to somatomedin. Further pathologic studies of the hypothalamus and pituitary in pituitary dwarfism; analysis of growth hormone and somatomedin cellular receptors; and elucidation of the structure of growth hormone releasing factor and techniques for its synthesis and assay should allow for the delineation of the specific pathogenetic mechanism in each of these syndromes.
 ...
PMID:Hereditary forms of growth hormone deficiency and resistance. 82 85

We used constructive interference in steady state (CISS) 3D Fourier transform (3DFT) MRI data sets to obtain three-dimensional (3D) virtual MRI endoscopic views of the intracranial cerebrospinal fluid (CSF) spaces, processing them with a commercially available perspective endoscopic algorithm. We investigated the potential of the intracranial virtual MRI endoscopy applied to visualisation of the pathology in 13 patients with surgically confirmed trigeminal neuralgia (3), hemifacial spasm (3), acoustic neuroma (3), suprasellar germinoma (1), Langerhans cell histiocytosis (1), lateral ventricle nodules (1) and pituitary dwarfism (1). All images were acquired using a 1.5-T imager employing a circular polarised head coil. The CISS-3DFT data sets were transferred to a workstation for processing with the perspective endoscopic algorithm. Postprocessing for virtual MRI endoscopy was possible for all data sets. The lesions in 12 patients, and their complex anatomical relationships with the surrounding structures, were well seen on the 3D images. A small acoustic neuroma in the internal auditory meatus was not seen using virtual endoscopy. Although virtual MRI endoscopy has limitations, it provides 3D images which cannot be acquired using any other procedure.
 ...
PMID:Virtual MRI endoscopy of the intracranial cerebrospinal fluid spaces. 983 93