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Biomedical subjects

M J Bennett

Publications and source records attributed to M J Bennett.

At least 235 records · Page 13Linked to original sources
PubMed1985

Riboflavin-responsive ethylmalonic-adipic aciduria.

A patient presenting with a condition resembling Reye's syndrome was found to have a urinary organic acid excretion pattern similar to those previously described in a single patient with ethylmalonic-adipic aciduria. The present patient responded clinically to riboflavin supplementation and his fibroblasts, when cultured in riboflavin-depleted medium, showed an abnormal reduction in the rate of butyrate oxidation.

Adipates↗
PubMed1985

Potentiation of the combination of pancuronium and metocurine by halothane and isoflurane in humans with and without renal failure.

Dose-response relationships for a 1:4 weight ratio-mixture of pancuronium and metocurine were studied during inhalational anesthesia with halothane and isoflurane in patients with and without renal failure. The time for recovery from 10 to 20% of control thumb twitch tension also was determined. In subjects with normal renal function, relaxant doses required for 95% twitch height suppression (ED95) were 50% of those predicted by simple addition of effects when used with a balanced anesthetic technique, 37% of predicted when used with 1.3 MAC halothane, and 25% of predicted when used with 1.3 MAC isoflurane (P less than 0.05). In subjects with renal failure, ED95 values for the combination were 40% of predicted when used with 1.2 MAC halothane and 45% of predicted when used with 1.2 MAC isoflurane (NS). For relaxants used singly in renal failure, pancuronium alone was slightly enhanced by 1.2 MAC halothane (85% of predicted), while 1.1 MAC isoflurane reduced the ED95 to 57% of predicted (P less than 0.05). Similar results were obtained for metocurine alone when used in renal failure (77 and 58% of predicted when used with halothane and isoflurane, respectively) (NS). Predicted values are published results for balanced anesthesia in normals. Recovery times were prolonged twofold in renal failure (P less than 0.05). Thus, the combination of pancuronium and metocurine is synergistic to the same degree in normals and in renal failure patients, but the total blockade produced by the combination is enhanced by halothane and isoflurane only in normals.

Adult↗
PubMed1985

Focal behavioral psychotherapy for acute narcissistic injury: "de mopes"--report of a case.

In order to be equal to the task of serving the mental health needs of the community, mental health professionals must develop methodology which is both effective and affordable, avoiding both undertreatment and overtreatment of those who need and seek their services. This fact is underscored by epidemiological data which suggest that up to 25 percent of the U.S. population suffers some form of demoralization, either alone or in combination with a diagnosable psychiatric disorder. Using a case example drawn from the classical folk literature, the author presents a model for understanding the demoralized state and treating it through a simple focal model of psychotherapy based on behavioral prescription. This technique is held to be humane and cost-effective. The author considers the possible reasons for the traditional psychiatric community's neglect of the ubiquitous problem of demoralization and its behavioral concomitant, moping.

Adaptation, Psychological↗
PubMed1985

Urinary sediment dolichol excretion in patients with Batten disease and other neurodegenerative and storage disorders.

Nonesterified dolichols have been measured in the urinary sediment of 20 patients with the late infantile and juvenile forms of neuronal ceroid lipofuscinosis (Batten disease), in 15 patients with other storage and neurodegenerative disorders and in 10 control subjects. Dolichols were measured by a high performance liquid chromatographic method and were related to urinary creatinine concentration. The levels of dolichols in Batten disease were not significantly elevated when compared to the normal subjects or to patients with other neurodegenerative disorders. The highest levels seen were in two patients with mucopolysaccharidosis types II and IV, respectively. Measurement of dolichols in urinary sediment is of little value in the diagnosis of Batten disease or in furthering our understanding of the underlying primary defect.

Adolescent↗
PubMed1984

Biochemical investigations on a patient with a defect in cytosolic acetoacetyl-CoA thiolase, associated with mental retardation.

A severely mentally retarded boy with two normal siblings was persistently found to excrete elevated amounts of 3-hydroxybutyrate and acetoacetate. Enzyme analysis in cultured fibroblasts revealed a probable deficiency in cytosolic acetoacetyl-CoA thiolase which was about half the control activity with normal mitochondrial thiolase activities. Treatment with reduced dietary fat was initiated and a rapid reduction of the ketosis to biochemical normality was demonstrated. Shortly after initiating dietary treatment he presented with severe gastrointestinal problems and the histological features of colitis cystica superficialis. This appeared to respond to intravenous hydrocortisone therapy with an apparent complete recovery.

Acetyl-CoA C-Acetyltransferase↗
PubMed1984

Glutaric aciduria type II: biochemical investigation and treatment of a child diagnosed prenatally.

Two sibs with the acute neonatal form of glutaric aciduria type II (deficient in vivo activity of multiple acyl-CoA dehydrogenases) are described. In the second case diagnosis was made prenatally on the basis of reduced oxidation of palmitate by cultured amniotic fluid cells. With prompt intervention in the neonatal period and a carefully controlled diet later, this second progressed well up to 4 months of age but died suddenly of cardiac failure, probably attributable to accumulation of fat. Neither patient showed any congenital morphological abnormality. Cultured fibroblasts from the second case showed a marked defect in the oxidation of a range of substrates requiring acyl-CoA dehydrogenases for their catabolism, but residual activity for some substrates was quite high. Large quantities of sarcosine were excreted in urine, again suggesting that the mutation leaves some residual dehydrogenation activity. Butyryl-, octanoyl- and palmitoyl-CoA dehydrogenases were present in essentially normal quantities in postmortem liver.

Adipates↗
PubMed1984

Long term follow up of newborns tested with the auditory response cradle.

The auditory response cradle is being used in a mass hearing screening project. Babies are assessed in the first week after birth by the fully automatic, microprocessor controlled cradle. The test, lasting from two to 10 minutes, compares physiological auditory responses to natural behaviour measured in control trials. More than 5000 babies have been tested and full follow up information at the age of 7 to 9 months is available from over two thirds of these. Less detailed information is available for 71% and 64% of those babies who have been followed up at 18 months and three years of age respectively. A total of 439 of 5553 neonates tested failed the first screening test. Eighty eight (1 X 6%) failed a second screening test while still in the maternity unit but 61 of these were subsequently shown to be normal, giving a false positive rate of 1 X 1%. The babies who failed the screening tests included 9 with sensorineural hearing loss, three with secretory otitis media, and three with abnormal auditory brain stem response tests. One child who passed the initial screening tests was found to have a moderately severe hearing loss at the age of 18 months.

Age Factors↗
PubMed1984

The routine investigation of urinary organic acids in selected paediatric patients: results over a 2 1/2-year period.

Over a 2 1/2-year period 13 patients with inborn errors of organic acid metabolism, excluding undifferentiated lactic acidosis, have been diagnosed in our laboratories. The diagnostic yield in patients who had not previously been investigated by organic acid chromatography was 1 in 25, the majority of cases having presented with metabolic acidosis. A larger number of non-specific abnormalities were also detected. This type of investigation is beset with pitfalls and is extremely labour intensive.

Acidosis↗
PubMed1984

Experience with a simple high-performance liquid chromatography method for the analysis of purine and pyrimidine nucleosides and bases in biological fluids.

Purine and pyrimidine nucleosides and bases were analysed using an isocratic reverse-phase HPLC technique and utilising simple sample preparation. The method has been successfully used to separate a large number of biologically occurring nucleic acid products within a 30-minute period and to identify them by their chromatographic and optical properties. A number of metabolic disorders have been identified, including hypoxanthine-guanine phosphoribosyl transferase and ornithine carbamyl transferase deficiencies. The method has been in routine use for 2 years performing both quantitative and qualitative analysis and has proved to be robust and reliable.

Asphyxia Neonatorum↗