Biomedical subjects
Publications and source records attributed to R Pinto.
We describe seven patients from five different families with GM2 gangliosidosis, variant B1. To our knowledge these are the first juvenile cases reported in the literature.
The present study investigated the attitudes and values of community and mental health center board and management staff of four Community Mental Health Centers and one mental health clinic toward the recent federal mandate calling for increased citizen involvement in center evaluation activities. Three related areas were address: (a) general attitudes toward citizen participation, (b) types of program-evaluation activities in which citizen input would be most useful; and (c) types of individuals who would best serve on citizen review groups. The results indicated that although board members are somewhat more optimistic about benefits received from citizen involvement, overall there was close agreement between the board and staff respondents in the three areas studied. These results were interpreted as substantiating the view that community mental health center boards typically reflect a provider orientation in their approach to mental health governance. A missing evaluation component in most centers is citizen participation as it reflects the values of its service consumers. It is advocated that only by developing consumer participation mechanisms will centers more readily achieve the goal of responsiveness to community needs.
Sixty-four chronic stabilised schizophrenics were studied for 18 months in order to assess the possible difference in therapeutic effects and side effects between flupenthixol decanoate and fluphenazine decanoate. Although certain differences in the BPRS sub-scores in favour of flupenthixol were present at various stages in the study, there was no significant difference between the two drugs in the overall antipsychotic scores at the end of the assessment period; however, more patients on fluphenating required additional therpay for depression or anxiety during the trial period.
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The effect of the verapamil upon the stability of the membranes of lysosomes isolated from rabbit hearts was studied by means of the acid phosphatase activity, the mapping enzyme of the cytoplasmic organelles. A dose of 2.5 mg of verapamil was injected endovenously in each animal. After homogenization of a sample taken from the lower end of the heart, the lysosomes were isolated by fractionated centrifugation and refrigerated at 2 degrees C. The lysosomal activity of non treated animals was 43.1 +/- 8.9x10(-1)mU/mg. The activity of lysosomes isolated from verapamil treated animals without previous treatment "in vitro" with digitonin was 8.7 +/- 1.6x10(-1)mU/mg and the activity of those with previous treatment of digitonin was 9.1 +/- 2.1x10(-1)mU/mg. The activity of lysosomes isolated from the control group and treated "in vitro" by digitonin was 193.9 +/- 40.5x10(-10)mU/mg. Consequently, the verapamil proved to have a strong capacity of protection of the lysosome membranes when "in vivo".
Roentgen videodensitometry (RVD) during myelography is described in 13 patients with a variety of intraspinal lesions. Abnormalities in the RVD tracing are discussed and correlated with the type of spinal canal obstruction. Intramedullary, intradural and extradural processes may be differentiated by RVD.
A case of metatropic dwarfism is presented. Particular reference is made to the differential diagnosis of this form.
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Four normal 18-20 yr-old men were studied on 3 occasions, from 0830 h to 1500 h. The baseline for each study consisted of 3 or 4 measurements of the respective hormone obtained between 0830 and 0900 h. In the control studies mean testosterone (T) fell by 43% (P less than 0.01) during the final 30 min. The fall was gradual throughout the day and was significant by 1100 h (P less than 0.05). Administration of LH and LH-releasing hormone (LHRH) at 0900 h resulted in 9-fold (5 min) and 3-fold (30 min) higher concentrations of LH respectively. LH declined more slowly after LHRH. Titers of T rose to the 0830-0900 h mean 130 min after LH but were never significantly elevated; the occurrence of a significant drop in mean T was delayed for 70 min. After LHRH there was a nonsignificant 24% increase of the mean T followed by a slow decline; however, T did not fall significantly below the mean baseline level. In contrast, in 2 of the 4 subjects LHRH resulted in rises in T levels (P less than 0.05) above the basal titers. Testosterone-binding globulin (TeBG) mean titers showed no diurnal rhythm in the control studies. There were statistically significant elevations of mean TeBG 150 min after LH and 340 to 370 min after LHRH, as well as sustained increases during the final 30 to 210 min of 1 or 2 individuals in each group. The reason for these increases in TeBG is not presently known. Estrogen analyses performed in all studies on 2 of the subjects revealed: 1) afternoon titers of estrone were lower than baseline in all 6 studies, 2) there was no diurnal rhythm for estradiol in control studies, and 3) estradiol increased during the final 2.5 to 3 h after LHRH (P less than 0.01), but after LH it was not altered in 1 man and was lower in the other.
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Central nervous system (CNS) involvement is rare in scleroderma unless there are concomitant abnormalities in renal or lung function or malignant hypertension. A 43-year-old woman with typical scleroderma developed subacute encephalopathy despite absence of the above abnormalities. Cerebral angiography demonstrated a focal arteritis. The patient improved while being given corticosteroids. We believe this case indicates that cerebral arteritis can occur in scleroderma.
A patient with diffuse and focal cerebral dysfunction was found to have absent peripheral pulses. Cerbral angiography revealed evidence of an arteritis with bilateral high grade carotid stenosis. When there was no laboratory confirmation of the arteritis, an iatrogenic etiology (ergotism) was suspected. This was later confirmed by the patient. The pertinent literature on ergotism is reviewed, and it is emphasized that ergotism may develop in patients on therapeutic doses of the drug.