Cholic Acid Biosynthesis: THE ENZYMATIC DEFECT IN CEREBROTENDINOUS XANTHOMATOSIS

Cholic acid biosynthesis is defective in individuals with cerebrotendinous xanthomatosis (CTX) and is associated with the excretion of 5β-cholestane-3α,7α, 12α,25-tetrol, an intermediate in the 25-hydroxylation pathway of cholic acid in CTX. To define the enzymatic defect in CTX, two suspected precursors of cholic acid, namely 5β-[7β-³H]cholestane-3α,7α, 12α-triol and 5β-[24-¹⁴C]cholestane-3α,7α, 12α,24S,25-pentol were examined by both in vivo and in vitro experiments. A third precursor, 5β-[7β-³H]-cholestane-3α,7α, 12α,25-tetrol, was compared with them in vitro.     

Mucinous tumors of the ovary with argyrophil cells. An immunohistochemical analysis

Forty-five cases of mucinous tumors of the ovary were studied for argyrophilia. Argyrophil cells were identified in seven of the 22 cystadenomas (32%), five of the 11 borderline tumors (45%), and two of the 12 carcinomas (17%). These 14 tumors and two additional mucinous tumors known to contain argyrophil cells were studied further by immunohistochemical methods for the localization of calcitonin, gastrin, somatostatin, adrenocorticotropin (ACTH), serotonin, neurotensin, and lysozyme. Serotonin immuno-reactivity was identified in 15 of the 16 cases. Among the peptide hormones, there was a high frequency of positivity for ACTH, gastrin, and somatostatin. Despite the demonstration of reactivity for these hormones, there was no clinical evidence of syndromes of hormone excess in the patients. Lysozyme was present in all but one of the benign and borderline tumors, but was not identified in the carcinomas. Lysozyme was also found in normal and neoplastic gastric and endocervical epithelium, indicating that its presence is not useful in differentiating gastrointestinal and müllerian-type epithelium. The results of this study confirm the previously recognized intestinal characteristics of the epithelium of many mucinous tumors, but also raise the question whether the simple, uniformly mucinous epithelium that is most common within these tumors and is generally regarded as endocervical in type may occasionally be gastric in nature.     

Radioimmunoassay to determine postprandial changes in plasma neuropeptide Y levels in awake dogs

A specific, precise and sensitive double-antibody radioimmunoassay for Neuropeptide Y (NPY) has been developed. There was no appreciable cross-reactivity with the structurally related peptides, peptide YY (PYY) and pancreatic polypeptide (PP). The minimum detectable plasma NPY level was 3 nM. Application of radioimmunoassay to canine models revealed that portal and systemic NPY levels increased significantly following a standard meal.     

Phaeomycotic cyst (chromoblastomycosis) of the neck

An unusual fungal infection of the neck caused by Phialophora verrucosa (chromoblastomycosis) is presented. The patient acquired this fungus by cutting his chin on a wooden floor. Surgical and medical therapy of this unusual disease is discussed.     

Aural Myiasis

Indian Journal of Otolaryngology and Head & Neck Surgery -