Biomedical subjects
Publications and source records attributed to G Morrow.
Menkes steely hair disease (MSHD) is a rare disorder which typically results in severe mental retardation and death in early childhood. A 21-month-old boy with an atypical milder form was presented by Procopis et al. [1981]. A second child with the atypical form is presented here who has survived to age 9 years and is doing well clinically.
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We describe the clinical application of DNA restriction fragment analysis to the genetic evaluation of a family with a child deficient in ornithine transcarbamylase (OTC). The results of protein loading studies and the interpretation of the DNA haplotype profiles for the human OTC gene are reported. DNA restriction fragment analysis may be a reliable technique for the prenatal diagnosis of OTC deficiency and identification of obligate carriers of this gene.
Female CD-1 mice immunized against the bacterium Streptococcus pneumoniae type III were exposed to 9-GHz pulsed microwaves (pulse repetition rate 970-1,000, pulse width 1.0 microseconds, peak power 1 W/cm2) at an average incident power density of 1 mW/cm2 (calculated SAR congruent to 0.47 W/kg) for 2 h per day for 5 days. Circulating antibody titers for the microwave-exposed animals were not significantly different from those of the sham-irradiated animals, and there were no differences in any of the hematological parameters analyzed, indicating that 9-GHz pulsed microwaves at 1 mW/cm2 do not alter the immune response of mice immunized against S pneumoniae.
Selective inhibition of peripheral esterases by tri-ortho-tolyl phosphate in the mouse resulted in an increase in the analgetic activity of heroin, without affecting the activity of morphine. In vitro inhibition of esterases by paraoxon reduced the affinity of heroin for the opiate receptor, while that of morphine was unaffected. These results suggest that both central and peripheral esterases are involved in the metabolism of heroin and that interference with critical esterases can alter its pharmacologic and toxicologic effects.
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One hundred forty-four patients and 68 physicians at three cancer centers were studied for their perceptions of the consent procedure, in which they participated one to three weeks earlier, for chemotherapy by one of 65 investigational protocols. Patients recalled the procedure positively and relied heavily on physician's advice. They felt most physicians wanted them to accept; 29% felt their participation in the decision was not encouraged. Primary reasons for accepting were trust in the physician, belief the treatment would help, and fear the disease (viewed as highly serious) would get worse without it. Nearly a fourth did not recall the information given that treatment was investigational. The consent form played no role in decision-making for 69%. Physicians believed therapeutic benefits would exceed potential problems for most patients; they viewed 41% of the patients as less than eager for details of treatment, a third as avoiding the seriousness of the discussion, and a third as passive in decision-making. The perceptual set of both parties places inadvertent constraint on patients' autonomy in decision making.
"What Happened to Pity?," a BBC documentary on the grief responses to the Aberfan disaster in 1966, powerfully affects viewers and is an effective tool for the study of sadness. Observations in the classroom and in the laboratory demonstrate that the great majority of viewers experienced some degree of sadness along with anger and sympathy. Subjects classified as high loss/grief experienced more intense and extended sadness than did those who had not had significant losses. They also displayed more apprehension, anger, shame-guilt, giving-up, anxiety, and irritation. Subjects experiencing the most sadness, compared with those less affected, were more initiating and talkative and their relationships in the laboratory were closer, more gratifying, task-oriented, therapeutic, and trusting. With the film they displayed more sympathy, role-identification, personalization, and like, but felt more drained. With respect to stable personality features they were warm, sharing, introspective, dependent, and more personally (than monetarily) motivated to volunteer for the study.
Moped injuries are an important cause of traffic-related injuries in children. An attempt was made to define the epidemiology as well as the nature and severity of injuries sustained in 88 moped-related accidents. Bicycle injuries among children (579) were used as a control. The patients with moped injuries were younger than expected (mean 12.8 years with a minimum legal driving age of 14 years in the study area). Among 26 hospital admissions due to moped accidents, there were 11 admissions to the intensive care unit, demonstrating the severe nature of the injuries. Fifty percent of the patients had orthopaedic injuries and 46% had neurologic injuries. These injuries resulted in an average length of hospitalization of 8.5 days (intensive care unit admissions lasted an average of 14.6 days). Recommendations are made to aid pediatricians in the counseling of patients and parents.
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Nomifensine and a proposed dihydroxy metabolite produced stimulation of motor behavior in mice with nomifensine being more potent. Weak cage-climbing behavior (stereotypy) was also produced. The stimulatory effects were greater in mice in which dopamine receptor sensitivity was increased by long-term haloperidol. Both of the analogs were potent inhibitors of dopamine and norepinephrine uptake in vitro with nomifensine approximately 3 times more potent than the metabolite. In contrast, the two analogs had weak affinity for the post-synaptic dopamine receptor (as measured by displacement of ligand binding in vitro) with dihydroxynomifensine approximately 6 times more potent than nomifensine. These results suggest that the behavioral effects of nomifensine are largely dependent on presynaptic catecholamine mechanisms but that weak direct dopamine agonist properties do exist, particularly in vivo where the drug may be metabolized to a more active form.
Continuous insulin infusion (CII) was used to increase intravenous glucose tolerance in 10 extremely premature (26.2 +/- 0.04 weeks, means +/- SEM) very low birth weight (819 +/- 53 g) hyperglycemic infants. CII was continued for 3-36 days. Over the first 72 h of insulin administration the mean amount of glucose tolerated rose from 0.35 +/- 0.06 to 0.67 +/- 0.06 g/kg/h and caloric intake derived from intravenous glucose increased from 29 to 56 kcal/kg/day. Insulin doses required to maintain normoglycemia ranged from 0.005-0.052 U/kg/h initially to 0.002-0.086 U/kg/h after 72 h of CII. Plasma insulin levels were significantly higher during insulin infusion. The low insulin doses required to maintain normoglycemia were consistent with a state of relative insulin deficiency, rather than insulin resistance. Mean plasma insulin/glucose ratios were significantly higher in normoglycemic versus hyperglycemic infants (0.40 +/- 0.08 vs. 0.14 +/- 0.05). Less than 1% of all blood glucose estimations were less than 25 mg/dl. Seventy-eight percent were within the normal range (greater than 45, less than 130 mg/dl). The rate of weight gain increased during CII in 8 of the 10 infants. CII may be useful in extremely premature, very-low-birth-weight infants in whom glucose intolerance persists despite conservative treatment, and either severely limits caloric intake, or results in life-threatening hyperglycemia.
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1. Isolated perfused rat hearts, acetoacetate oxidation was significantly enhanced by propionate. 2. No enhancement of acetoacetate oxidation could be observed in vitamin B12-deficient rat hearts when propionate was present. 3. Although the mechanisms of enhancement are not clear, ketone clearance is enhanced by propionate and may provide an insight into the therapy of certain diseases characterized by ketosis.