11 beta-Hydroxydehydroepiandrosterone in a case of virilizing adrenal adenoma: isolation from urine and mitochondrial conversion from dehydroepiandrosterone.

11 beta-Hydroxydehydroepiandrosterone has been isolated from the urine of a 14-yr-old girl with a virilizing adrenal adenoma. Its excretion was estimated to be 0.4 mg/24 h by gas chromatography and the compound was further identified by mass spectrometry. When [7-3H]dehydroepiandrosterone was incubated with mitochondria prepared from the adenoma, approximately 10% was converted to 11 beta-hydroxydehydroepiandrosterone. The identity of the radioactive 11 beta-hydroxydehydroepiandrosterone was verified by reversed isotopic dilution, its conversion to 11 beta-hydroxyandrostenedione, and its mobility in several chromatographic systems. This is the first demonstration of an 11 beta-hydroxylase from a human source having an affinity for dehydroepiandrosterone.

Pressure-pain thresholds and MRI effusions in TMJ arthralgia.

It has been suggested that MRI-depicted effusions identify patients with TMJ arthralgia. The Research Diagnostic Criteria (RDC) propose a pressure-pain threshold (PPT) of 1 pound for the identification of TMJ arthralgia. The hypotheses in this study were that: (1) there is no association between MRI-depicted effusions and TMJ arthralgia, and (2) a PPT of 1 pound does not discriminate between subjects with and those without arthralgia. Thirty females with TMJ disc displacement with reduction were divided into two groups based on the presence or absence of the self-report of TMJ pain. Bilateral TMJ PPTs and MRIs were obtained. Increasing palpation pressure from 1 to 3 pounds increased the sensitivity for identifying arthralgia from 22% to 100%, with a corresponding decrease in the specificity from 100% to 81%. The sensitivity and specificity of effusions for identifying arthralgia were 85% and 28%, respectively. These results suggest that the use of palpation pressures greater than 1 pound is a valid test for TMJ arthralgia. However, TMJ effusions lack adequate specificity for identifying TMJ arthralgia and were not associated with pain.

Interexaminer reliability and clinical validity of the temporomandibular index: a new outcome measure for temporomandibular disorders.

AIMS: The operational definitions for the Craniomandibular Index (CMI) were redesigned to conform precisely to those of the Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD), resulting in a single examination protocol, the Temporomandibular Index (TMI). The objectives were to evaluate interexaminer reliability of the TMI as well as its criteria and construct validity for measurement of TMD severity. METHODS: Interexaminer reliability of the TMI was assessed on 12 subjects. Criterion validity of the TMI was evaluated relative to the CMI, the latter having established validity. Construct validity of the TMI was evaluated for its capacity to differentiate TMD patients (n = 79) from normal subjects (n = 20) and to detect changes in severity over time. RESULTS: The examiner's average TMI scores were 0.27 +/- 0.19 (SD) and 0.26 +/- 0.20. Agreement was excellent, with an intraclass correlation coefficient (ICC) of 0.93. The scores for the TMI and the CMI correlated highly, with an ICC of 0.97. Statistical contrasts between the symptomatic groups and the normal subjects were highly significant (P < .001). In 20 TMD patients who underwent treatment for their disorder, their mean change of 0.12 from their pretreatment TMI scores was highly significant (P < .001). CONCLUSION: This study has provided statistical evidence for the clinical reliability and validity of the TMI, which indicates that the RDC examination protocol is appropriate for determining TMD severity by the TMI algorithm, and diagnosis of TMD subtypes by the RDC algorithm.

Osteochondroma of the mandibular condyle. A case report.

Osteochondroma of the mandibular condyle is extremely rare and may cause signs and symptoms like those seen in patients with temporomandibular joint dysfunction. Differentiation between osteochondroma and condylar hyperplasia is not possible on histologic grounds alone, but the radiographic and intraoperative findings together are usually sufficient to establish a definite diagnosis.

The effects of HCG, indomethacin, flufenamic acid and aspirin in the immature female rat.

Immature female rats (21 to 23 days old, 35 to 45 g) were injected subcutaneously with 2-5 i. u. HCG 18 hr before autopsy. Ovaries and uteri were removed; wet weight, dry weight and uterine protein content were determined. Ovarian and uterine weights, ovarian blood volume and uterine protein content were increased after HCG treatment. When immature female rats were pretreated with indomethacin, flufenamic acid or aspirin, the ovarian effects of HCG were inhibited: only slight increases in ovarian weight and blood volume were observed. Indomethacin attenuated the increases in uterine weight, and protein content, but neither flufenamic acid nor aspirin were effective in inhibiting these responses. The possible role of prostaglandins and of oestrogen as mediators of these responses is discussed.

A case of adrenogenital syndrome with aberrant 11beta-hydroxylation.

A 17 year old female patient with hypertension, amenorrhoea and hirsutism was found to have subnormal levels of plasma and urinary cortisol, significant plasma levels of Reichstein's compound S and 21-deoxycortisol, high urinary levels of THS and pregnanetriolone as well as elevated levels of plasma and urinary testosterone. Treatment with 0.5 mg/day of dexamethasone or 25 mg/day cortisone reduced her hypertension and restored her menstrual cycles, but also resulted in the development of moon face, body striae and a gain in weight. Lower doses of cortisone were without effect. The deficient cortisol production coupled with the presence of unusual intermediates such as Reichstein's compound S and 21-deoxycortisol can be explained by a shift in the substrate specificity of 11beta-hydroxylase from C-21-hydroxylated substrates (i.e. compound S) to C-21-deoxy substrates (i.e. 17-hydroxyprogesterone).

Familial HDL deficiency due to marked hypercatabolism of normal apoA-I.

In this article, we describe a 46-year-old man with severe high-density lipoprotein (HDL) deficiency and his kindred. In the proband, HDL cholesterol and apolipoprotein (apo) A-I levels were 5 and 4.5 mg/dL, respectively. Xanthomata, xanthelasma, arcus corneae, and hepatosplenomegaly were not present. The proband had coronary artery disease, but it was impossible to state whether the HDL deficiency cosegregated with premature coronary artery disease in this kindred. Pedigree analysis was suggestive of a codominant familial disease. Polymerase chain reaction amplification of the apoA-I gene of the proband, followed by subcloning and sequencing, did not reveal any mutation in either the coding regions or intron-exon junctions. A kinetic study using deuterated leucine to endogenously label apoA-I was performed to elucidate the metabolic basis of the apoA-I deficiency. We demonstrated marked hypercatabolism of apoA-I in the proband, with a fractional catabolic rate more than 10 times faster than normal; the plasma residence time of apoA-I in the proband was only 0.38 day compared with 4.10 days in a control subject. The apoA-I production rate was also substantially decreased in the proband. The association of a normal apoA-I gene sequence with marked hypercatabolism of apoA-I is similar to that described in Tangier disease. However, except for the presence of mild, diffuse, corneal deposits, this patient had no evidence of the reticuloendothelial cholesterol deposition characteristic of Tangier disease. This study establishes that a form of severe hypoalphalipoproteinemia distinct from Tangier disease can be caused by marked hypercatabolism of a normal A-I apolipoprotein.