Ossifying fibroma of the sella turcica.

Ossifying fibroma of the sellar turcica is extremely rare. There are only sporadic case reports in the literature. One such case simulating pituitary adenoma is presented in an 18-year-old girl.     

Severe Malnutrition Associated with α-Heavy Chain Disease: Response to Tetracycline and Intensive Nutritional Support

A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. Appetite and dietary intake had been remarkably well preserved up until the week before admission. The severity of his depletion was evidenced by a body weight of only 38% of standard, multiple electrolyte deficiencies, and reduced metabolic expenditure, protein turnover, protein synthesis, and pancreatic function. Immunological defects included diminished lymphocyte numbers, lymphocyte transformation, gamma-globulin concentration, and cell-mediated immunity. A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. Treatment consisted initially of intravenous nutrition (because of the extreme malnutrition, severe diarrhea, and malabsorption of fluid, electrolytes, carbohydrates, and fat) and oral tetracycline. Response was dramatic, with a doubling of body weight within 6 wk, and resolution of malabsorption. He was discharged on a normal diet and long-term oral tetracycline (250 mg/day), and at 1-yr follow-up, nutritional status and gut function were normal despite persistence of duodenal mucosal abnormalities and markers of alpha-HCD and bacterial overgrowth. These results suggest that the malabsorption initially identified in this patient was not due simply to the mucosal abnormalities that characterize alpha-HCD, but was more a consequence of the superimposition of nutrient maldigestion and absorption resulting from the extreme state of protein deficiency and its effects on gut and pancreatic function.     

Can we understand partnerships in general practice? A pilot study

BACKGROUND: Partnerships have been investigated in different professions,but other than identifying problems, little work has been carriedout on general practice. OBJECTIVE: The aim of this present study was to develop methods for studyingpartnerships in general practice. METHOD: A tripartite methodological approach was used, with questionnairesadapted from other instruments in use in other professions,followed by an individual interview with each partner, and non-participantobservation at a partnership meeting. Results for one case-studypartnership are given. RESULTS: There were no major differences between the partners on alldimensions measured; the minor differences indicated by theresults of the questionnaires were corroborated by the partnerinterviews and observations. CONCLUSIONS: We conclude that the use of such techniques could provide supportto partnerships going through significant periods of change. Keywords. General practice, interview, observation, partnerships, questionnaire.     

The thyroid

The integument contains many components whose function is influenced by thyroid hormone. Thus, an increase or decrease in hormone levels results in a variety of cutaneous, hair, and nail changes. These changes are important to recognize, as they may aid in the early diagnosis of thyroid disorders. Other cutaneous disorders are more prevalent in patients with thyroid disease, and their recognition may likewise be helpful. Finally, several syndrome complexes occur with which thyroid disorders are regularly associated.     

Sensorineural hearing loss and the Marinesco-Sj?gren syndrome

Sensorineural hearing loss has not been previously reported in patients with the Marinesco-Sj?gren Syndrome. Two siblings are described where hearing was initially normal but subsequently deteriorated.     

The biomechanical and morphological changes in the medial collateral ligament of the rabbit after immobilization and remobilization

The effects of immobilization and remobilization on the biomechanical and morphological properties of the femur-medial collateral ligament-tibia complex and each of its components were investigated in the rabbit. Specimens that had been obtained after periods of unilateral immobilization of the knee and remobilization were evaluated for structural properties. In addition, the mechanical properties of the substance of the medial collateral ligament and the histological characteristics of both the substance of the ligament and its sites of insertion were evaluated. After immobilization, there were significant reductions in the ultimate load and energy-absorbing capabilities of the bone-ligament complex, and an increased number of failures occurred by tibial avulsion. The tissue of the medial collateral ligament also became less stiff as a result of immobilization. Histologically, the femoral and tibial insertion sites showed increased osteoclastic activity, resorption of bone, and disruption of the normal attachment of the bone to the ligament. With remobilization, the ultimate load and energy-absorbing capabilities of the bone-ligament complex improved but did not return to normal. Failure by tibial avulsion became less frequent, and the stress-strain characteristics of the medial collateral ligament returned to normal. Histologically, the sites of insertion of the ligament also showed evidence of recovery.     

MR imaging of pineal cysts

Identification of pineal cysts with imaging modalities other than magnetic resonance (MR) has been infrequent, despite a reported autopsy incidence of as high a 40%. We recently identified 15 patients in whom pineal cysts were seen on MR. These cysts ranged from 5 to 15 mm in maximum diameter. The cyst walls were thin, usually measuring less than 2 mm. The signal characteristics of the fluid within the cysts paralleled that of CSF, although it differed somewhat in signal intensity from CSF collections in which there was pulsatile motion. All lesions were midline in location. There was no significant mass effect, although mild flattening of the superior colliculi could be seen. Two cases were operated on; at operation, typical pineal cysts were found, with one containing hemosiderin-laden proteinaceous material, which accounted for its markedly higher signal intensity. It is important that this entity be recognized so that these lesions are not confused with pineal tumors.     

Development of OASYS-2: a system for the analysis of serial measurement of peak expiratory flow in workers with suspected occupational asthma.

BACKGROUND: Serial peak expiratory flow (PEF) measurement is usually the most appropriate first step in the confirmation of occupational asthma. Visual assessment of the plotted record is more sensitive and specific than statistical methods so far reported. The use of visual analysis is limited by lack of widespread expertise in the methods. A computer assisted diagnostic aid (OASYS-2) has been developed which is based on a scoring system developed from visual analysis. This removes the requirement for an experienced interpreter and should lead to the more widespread use of the technique. METHODS: PEF records were collected from workers attending an occupational lung disease clinic for investigation of suspected occupational asthma and from workers participating in a study of respiratory symptoms in a postal sorting office. PEF records were divided into two development sets and two gold standard sets. The latter consisted of records from workers in which a final diagnosis had been reached by a method other than PEF recording. An experienced observer scored individual work and rest periods for the two development set PEF records; linear discriminant analysis was used to compare measurements taken from development set 1 records with visual scores. Two equations were produced which allowed prediction of scores for individual work or rest periods. The development set 2 was used to determine how these scores should be used to produce a whole record score. The first gold standard set was used to determine the whole record score which best separated those with and without occupational asthma. The second set determined the sensitivity and specificity of the chosen score. RESULTS: Two hundred and sixty eight PEF records were collected from 169 workers and divided into two development sets (81 and 60 records) and two gold standard sets (60 and 67 records). Linear discriminant analysis produced equations predicting the score for work periods incorporating five indices of PEF change and one for rest periods using seven indices. These equations correctly predicted the score for development set 1 work and rest periods on 61% of occasions (kappa = 0.47). The whole record score for development set 2 records, after weighting for definite or definitely no occupational effect, correlated with the visual score (correlation coefficient 0.86). Comparison with gold standard set 1 identified a cut off which proved to have a sensitivity of 75% and a specificity of 94% for an independent diagnosis of occupational asthma when applied to gold standard set 2. CONCLUSIONS: These results suggest that the sensitivity and specificity of analysing PEF records for occupational asthma using OASYS-2 approaches that of visual analysis, but it should be absolutely reproducible. The performance of OASYS-2 is more specific and approaches the sensitivity of other statistical methods of analysis. The evaluation of a large number of PEF records from workers exposed to different sensitising agents suggests that these results should be robust and should be repeatable in clinical practice.     

Real-time imaging of Rab3a and Rab5a reveals differential roles in presynaptic function

We investigated the roles of two Rab-family proteins, Rab3a and Rab5a, in hippocampal synaptic transmission using real-time fluorescence imaging. During synaptic activity, Rab3a dissociated from synaptic vesicles and dispersed into neighbouring axonal regions. Dispersion required calcium-dependent exocytosis and was complete before the entire vesicle pool turned over. In contrast, even prolonged synaptic activity produced limited dispersion of Rab5a. A GTPase-deficient mutant, Rab3a (Q81L), dispersed more slowly than wild-type Rab3a, and decreased the rate of exocytosis and the size of the recycling pool of vesicles. While overexpression of Rab3a did not affect vesicle recycling, overexpression of Rab5a reduced the recycling pool size by 50%. We propose that while Rab3a preferentially associates with recycling synaptic vesicles and modulates their trafficking, Rab5a is largely excluded from recycling vesicles.