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Biomedical subjects

B Gibson

Publications and source records attributed to B Gibson.

At least 55 records · Page 3Linked to original sources
PubMed1995

The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy.

Mitochondrial long chain fatty acid beta-oxidation provides the major source of energy in the heart. Deficiencies of human beta-oxidation enzymes produce sudden, unexplained death in childhood, acute hepatic encephalopathy, skeletal myopathy, or cardiomyopathy. Long chain 3-hydroxyacyl-CoA dehydrogenase [LCHAD; long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase, EC 1.1.1.211] catalyzes the third step in beta-oxidation, and this activity is present on the C-terminal portion of the alpha subunit of mitochondrial trifunctional protein. We used single-stranded conformation variance analysis of the exons of the human LCHAD (alpha subunit) gene to determine the molecular basis of LCHAD deficiency in three families with children presenting with sudden unexplained death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome). In all families, the mothers had acute fatty liver and associated sever complications during pregnancies with the affected infants. The analysis in two affected children revealed a G to C mutation at position 1528 (G1528C) of the alpha subunit of the trifunctional protein on both alleles. This is in the LCHAD domain and substitutes glutamine for glutamic acid at position 474 of mature alpha subunit. The third child had this G1528C mutation on one allele and a different mutation (C1132T) creating a premature termination codon (residue 342) on the second allele. Our results demonstrate that mutations in the LCHAD domain of the trifunctional protein alpha subunit in affected offspring are associated with maternal acute fatty liver of pregnancy. This is the initial delineation of the molecular basis of isolated LCHAD deficiency.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1995

Rapid and sensitive non-radioactive assay for the detection of clonal gene rearrangements in B-lineage acute leukaemia.

We have applied a simple and non-radioactive assay system used in conjunction with polymerase chain reaction amplification and high-resolution polyacrylamide gel electrophoresis to detect clonal immunoglobulin gene rearrangements in leukaemia patient DNA. This technique is as sensitive as isotopic methods, allowing the detection of clonal DNA diluted 1 in 10,000 in non-clonal DNA.

Base Sequence↗
PubMed1995

The management of leg ulcers in a patient with ulcerative colitis. This case study details the treatment of a patient with pyoderma gangrenosum on the shins of both legs.

Pyoderma gangrenosum is a rare condition which is sometimes associated with ulcerative colitis and Crohn's disease. The pathological basis is not completely understood but it may be caused by vasculitis. The lesions are typically said to have purple overhanging edges and a necrotic base. The condition may also occur with rheumatoid arthritis, multiple myeloma and leukaemia. The treatment of choice is systemic steroids but it may also respond to azathioprine, minocycline or clofazimine.

Adult↗
PubMed1994

Utilization of a questionnaire to provide follow-up services in an infant hearing screening program.

An important goal of any neonatal hearing screening program is to provide adequate monitoring and follow-up services for all newborns tested. This is often the most difficult component of the program to administer. An attempt was made to obtain follow-up information through the use of a questionnaire modeled after Jacobson and Jacobson (1990). This questionnaire was administered by mail or telephone to 119 parents whose infants had passed a hearing screening as neonates in four hospitals. Forty-four percent of all parents contacted by either means responded to the questionnaire; however, response rates varied by as much as 36 percent, depending on the hospital in which a child was born. Our results suggest that the successful use of follow-up questionnaires depends greatly upon hospital demographics. When limitations are considered, however, they may provide an alternative to on-site follow-up services for infants who pass initial screening.

Hearing Disorders↗
PubMed1994

Black-white disparities in health status in Missouri.

Historically, the overall health status as measured by life expectancy and infant mortality of Black and White Missourians has been significantly different. We analyzed birth, death, child death and communicable disease data to determine if Blacks have narrowed the gap in health status. The overall health status of Black and White Missourians improved over the last two decades. However, the gap in health status still remains, and in some cases, has gotten worse. This occurred despite the increase in health care financing through Medicare and Medicaid. To narrow the gap in health status between Blacks and Whites requires addressing prevention and the social problems along with the medical problems.

Adolescent↗
PubMed1993

The loss of a baby at birth: the role of the bereavement officer.

OBJECTIVE: To illustrate and emphasise the role of the Bereavement Officer in the management of perinatal death, as recommended and envisaged by a report of the Royal College of Obstetricians and Gynaecologists in 1985. DESIGN: A description of the training and responsibilities of the officer and an account of her involvement with all perinatal deaths within a given period. SETTING: Perinatal deaths in the 12 months, 1 October 1989 to 30 September 1990 from a regional neonatal intensive care unit within a maternity hospital. SUBJECTS: A total of 59 registerable deaths, three late terminations (severe fetal abnormality) and 12 late mid-trimester miscarriages were dealt with by the Bereavement Officer in this 12 month period. RESULTS: The introduction of this service to our unit has led to an improvement in our management of both perinatal and fetal deaths. CONCLUSION: The service has justified fully the recommendations made by the Royal College of Obstetricians and Gynaecologists. Reducing parents' distress will facilitate their grieving and any subsequent bereavement counselling.

Bereavement↗
PubMed1993

The environmental connection.

Both the World Health Organization and the Canadian Health for All concept recognize that healthy environments are fundamental to human health. Usually, the responsibility for environmental protection is assigned to public health inspectors. But there is more to a healthy environment than protection. Creating a healthy environment means "altering or adapting our social, economic and physical surroundings in ways that not only preserve but also enhance our health." Clearly, health promotion is an integral part of the concept. As experts in community health promotion, public health nurses (PHNs) could--and should--complement the work of health inspectors.

Canada↗
PubMed1992

Lipooligosaccharides (LOS) of some Haemophilus species mimic human glycosphingolipids, and some LOS are sialylated.

The lipooligosaccharides (LOS) of strains of Haemophilus ducreyi, Neisseria gonorrhoeae, Neisseria meningitidis, and Neisseria lactamica contain epitopes that are antigenically and structurally similar to carbohydrates present in human glycosphingolipids. LOS from strains of Haemophilus influenzae and H. influenzae biogroup aegyptius were tested for the binding of monoclonal antibodies (MAbs) that bind to human glycosphingolipids possessing Gal beta 1-4GlcNAc (MAb 3F11) and Gal alpha 1-4Gal beta 1-4Glc (MAb anti-Pk). In solid-phase radioimmunoassays, the LOS of 18 of 19 H. influenzae type b (Hib), 8 of 19 nontypeable H. influenzae, and 10 of 20 H. influenzae biogroup aegyptius strains bound MAb anti-Pk. The LOS of 13 of 19 Hib, 10 of 16 nontypeable H. influenzae, and 2 of 18 H. influenzae biogroup aegyptius strains bound MAb 3F11. Neuraminidase treatment of the strains increased the binding of MAb 3F11 by more than twofold in 47% of the H. influenzae strains, suggesting that sialic acid occluded the LOS structure recognized by MAb 3F11. The material released from neuraminidase-treated Hib LOS was confirmed to be sialic acid by high-performance anion-exchange chromatography. A recombinant plasmid containing genes involved in Hib LOS biosynthesis directed the expression (assembly) of the 3F11 epitope in Escherichia coli. These studies demonstrate that H. influenzae and H. influenzae biogroup aegyptius express at least two LOS epitopes that are similar to those present in human glycosphingolipids. Sialic acid was present on the LOS of some H. influenzae strains and prevented the binding of MAb 3F11 to its epitope. The oligosaccharide portion of sialylated LOS may also resemble sialylated oligosaccharides present in human glycosphingolipids (gangliosides).

Antibodies, Monoclonal↗
PubMed1992

Age-related changes in oral sensation.

Oral sensation (including two-point discrimination, oral stereognosis, vibrotactile detection, somesthetic sensitivity, proprioception, and thermal sensitivity) was studied in 60 healthy adults in five age categories: 20 to 34, 35 to 49, 50 to 64, 65 to 79, and 80 years and above. Thermal and somesthetic sensitivity as well as proprioception did not change with age. Ability to differentiate tactile and vibratory sensation on the lip decreased after age 80 (P less than .01), but vibration detection on the soft palate did not change. Stereognostic ability remained good up to age 80, and then declined for four of the nine shapes tested (P less than .01). Two-point discrimination deteriorated on the upper lip (P less than .01), on the cheeks (P less than .02), and on the lower lip (P less than .06). Two-point discrimination on the tongue and palate did not change. It was noted that oral sensation remained good with aging, showing only a slight decline in function after age 80.

Adult↗
PubMed1991

Enhanced thrombin generation in patients receiving intensive care.

Thrombin-antithrombin III complex (TAT) concentration was measured in 27 control and 155 intensive care patients to (a) establish normal reference ranges, (b) measure thrombin generation in critically ill patients, and (c) determine the characteristics of the TAT assay for the diagnosis of disseminated intravascular coagulation (DIC) in children. The normal reference range was 1-4.3 micrograms/l (median 2.3 micrograms/l), and 89.7% of patients had raised TAT concentrations. Median TAT concentrations in the presence of DIC (27 micrograms/l) were significantly higher than in its absence (8 micrograms/l). Sensitivity, specificity, and positive and negative predictive values of the assay were 97.3%, 28.3%, 76.3%, and 81.3%, respectively, at a cut off of 4 micrograms/l. Excess thrombin production occurs in the majority of critically ill children. The TAT assay is potentially useful in the diagnosis of DIC in children.

Adolescent↗
PubMed1991

Breslow thickness of cutaneous malignant melanoma in paraffin wax and frozen sections.

Breslow tumour thickness was measured in frozen and paraffin wax sections from 21 excision biopsies of cutaneous malignant melanomas by two observers. There was no consistent variation between frozen and paraffin wax sections, with recorded differences ranging from +0.3 mm to -0.2 mm. Interobserver differences ranged from +0.4 mm to -0.2 mm. The interobserver variations exceeded the intraobserver variations, but neither were significant. These findings show conclusively that, when using high quality frozen sections, there is no significant difference between Breslow thickness measured in frozen or paraffin wax sections and therefore that frozen sections can be used to microstage melanoma. Interobserver variations seem to be a more likely source of erroneous measurements of tumour thickness.

Biopsy↗
PubMed1990

Psychological disturbance in children with haemophilia.

OBJECTIVE: To assess the need for formal psychotherapeutic intervention in children attending a children's haemophilia clinic after some of them had been diagnosed as positive for HIV. DESIGN: Comparison of haemophiliac children with matched control groups of diabetic and healthy children. SETTING: The West of Scotland Children's Haemophilia Centre, Glasgow. PATIENTS: 43 Children aged 3 to 16 years with mild, moderate, and severe clotting disorders were matched with control groups of 46 diabetic children and 42 physically healthy children. INTERVENTIONS: Parents of children aged 3-5 years were interviewed with the behaviour screening questionnaire. Children aged 6 to 16 were assessed by parental and teacher report using standardised questionnaires and self report using a computerised depression inventory. All were scored numerically according to the presence of symptoms of emotional and behavioural problems. MAIN OUTCOME MEASURES: The groups were compared for mean scores on each rating device and for number of children achieving scores within the pathological range. RESULTS: In the 6-16 age group five haemophiliac children, five diabetic children, and three healthy children scored in the pathological range on the parent questionnaire, as did two, three, and five respectively on the teacher questionnaire and four, four, and eight on the depression inventory. There was no significant difference across the three groups. Analysis of mean scores similarly showed no significant difference across groups. In contrast, the single measure used for younger children showed an increase in behavioural difficulties among the diabetic children. CONCLUSION: Haemophiliac children attending the West of Scotland Centre were no more disturbed than their diabetic or healthy peers despite the identification of HIV infection within the clinic and the widespread adverse publicity associated with AIDS and HIV infection.

Adolescent↗
PubMed1990

Back-up for the venous pump: compression hosiery.

Graduated compression hosiery is used with a range of conditions caused by insufficient venous return. It is vital for patients to have the correct hosiery for their condition, and to understand its importance.

Bandages↗