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Biomedical subjects

B Hunter

Publications and source records attributed to B Hunter.

At least 19 recordsLinked to original sources

Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy.

Hurler's syndrome, also known as mucopolysaccharidosis I (MPS I-H), is a rare condition inherited as an autosomal recessive trait. It is caused by a deficiency in alpha-L-iduronidase, an enzyme that participates in the degradation of the glycosaminoglycans (GAGs) heparin sulphate and dermatan sulphate. Children with Hurler's syndrome appear nearly normal at birth but, left untreated, show a progressive mental and physical deterioration caused by a build-up of GAGs in all organs of the body. Death is often caused by cardiac or respiratory failure and usually occurs before the second decade of life. In recent years, bone marrow transplantation (BMT) has been employed in the management of patients with Hurler's syndrome. However, the dental findings observed in these cases have not previously been reported in the dental literature. Here we report a patient aged 11 years and 6 months, presented to a Specialist Paediatric Dentistry Unit, who was successfully treated by BMT at 18 months of age.

Anodontia↗

ABO-incompatible liver transplantation: a new therapeutic option for patients with acute liver failure in Chile.

Different ways have been suggested to expand donor numbers for liver transplantation. Transplantation using ABO-incompatible hepatic grafts has recently been a controversial issue due to the high risk of hyperacute rejection mediated by preformed anti-ABO antibodies. We report three patients with acute liver failure who were transplanted with ABO-incompatible livers: A to O in two patients and A to B in one case. We used pre- and posttransplant total plasma exchange, splenectomy, and triple immunosuppression. All three patients are alive; one graft was lost, probably secondary to thrombotic microangiopathy with low isohemagglutinin titers of 1:8. One patient developed acute cellular rejection that was reversed with a bolus of methylprednisolone. No antibody-mediated rejection occurred. Financial and infectious considerations have to be considered. In our series, the final liver transplantation cost was higher than average for acute liver failure. Plasmapheresis has the highest cost of all the additional procedures. ABO-incompatible liver transplantation, because of the splenectomy it requires, has been associated with more infections due to encapsulated organisms. However, with splenectomy in our three patients, none had infections due to these bacteria. In our country, we do not consider ABO-incompatible liver transplantation as a first-line option, except for highly selected patients.

ABO Blood-Group System↗

Pediatric liver transplantation: ten years of experience in a multicentric program in Chile.

Liver transplantation is the only treatment for patients with terminal acute and chronic diseases. Liver transplantation was started in Chile in 1985; our pediatric program began in 1993. The aim of this paper work was to present our experience from 1993 through 2004. One hundred and thirty two orthotopic liver transplants (OLT) were performed in children of mean age 5 years and median age 4 years (8 months to 15 years). The most frequent indications were biliary atresia, (43.1%) and acute liver failure (ALF; 20.4%), whose frequent cause was unknown but viral hepatitis A was the second one. A complete liver was transplanted in 59 patients, reduced in 39, split in one, and as an auxiliary liver in another one. Living related liver transplantation was performed in 32 cases (24.2%), of which thirty included segments II and III, and two, a right liver. A terminal arterial anastomosis was performed in 102 (77.2%) recipients and a graft interposition in 32 patients (24.2%). In 16 cases, biliary reconstruction was performed through an enterobiliary anastomosis. Immunosuppression included cyclosporine (Neoral), steroids, and azathioprine with conversion to tacrolimus (Prograf) as indicated. Rejection episodes, which were always biopsy-proven, were treated either with methylprednisolone or with antibodies. Biliary complications were the most frequent (21.4%) and the second cause was vascular complications (13%). Sixty-six patients suffered an acute rejection episode. Actuarial graft survival was 81.3% at 1 year and 72% at 5 years, while actuarial graft survival for ALF was 75.9% at 1 year and 67.8% at 5 years. Our results are comparable to those reported by most international groups.

Adolescent↗

Living related liver transplantation. Why this option has been discarded in a pediatric liver transplant program in Chile.

Living related living transplantation (LRLT) has opened new possibilities for planning transplantation in better conditions for children with emergency situations and chronic liver diseases. Since we began the LRLT program in 1999, we have performed 57 pediatric liver transplants, 17 (29.8%) using living related donors (LRD). The aim of this study was to analyze the reasons why LRD were discarded as a therapeutic option. All pediatric patients were prospectively included in our Microsoft Excel database that was reviewed for obtaining information about causes why the LRLT could not be done. LRLT was proposed in 28 cases and performed in 17 (60.7%). The reasons for LRD rejection were: parent's fear of surgical complications in four cases; drug abuse in two; a mother without family support; medical reasons in two; and only one, due to anatomical reasons and in one case, cadaveric graft transplantation was performed while completing the father's evaluation. From these eleven cases, the indications for liver transplant were acute liver failure (ALF) in seven, biliary atresia in three, and Alagille syndrome in one. Nine were transplanted with cadaveric organs, but two patients with ALF died awaiting a liver. Efforts should be made to clarify the advantages and the disadvantages of LRD in each case, allowing parents to make a free, well-informed decision.

Attitude to Health↗

The dental management of a patient with hyperimmunoglobulinemia E syndrome: a case report.

Hyperimmunoglobulinemia E recurrent infection syndrome (also known as Job's syndrome) is a rare multi-system primary immunological disorder in which non-immunological abnormalities of the dentition, bones and connective tissue are also seen. A previous study has reported the occurrence of dental abnormalities in three-quarters of individuals diagnosed as suffering from this condition. The present authors report the case of a boy whose prolonged retention of the primary dentition was associated with delayed eruption of permanent teeth. They emphasize the need for early intervention in order to help minimize later orthodontic problems.

Child↗

Staple diet: a foreign body in a tooth.

The chance discovery of a foreign object embedded in a tooth is uncommon, and requires radiographic examination to determine the composition and location of the object. The authors describe the case of an 11-year-old boy who presented with a staple lodged in the root canal of the maxillary left permanent central incisor. This staple was localized using parallax techniques and successfully removed from the canal. The radiographic techniques of localization and possible methods of removal of foreign bodies are discussed.

Child↗

Special Care Dentistry: attitudes of Specialists in Paediatric Dentistry practising in the UK to the creation of a new specialty.

OBJECTIVES: This study was designed to examine the attitudes of Specialists in Paediatric Dentistry (SPDs) practising in the UK towards the creation of a specialty of Special Care Dentistry (SCD). DESIGN: Data were collected by postal questionnaire. Sample and methods. Two hundred and eleven dentists whose names were entered on the General Dental Council's Specialist List in Paediatric Dentistry, and who were resident and practising in the UK, were asked to complete a questionnaire consisting of both open and closed questions. RESULTS: Questionnaires were returned by 167 (79.2%) of the eligible SPDs. One hundred and sixty respondents (95.8%) expressed an opinion in relation to the creation of a specialty of SCD. One hundred and forty-three respondents (85.6%) stated that they supported such a proposal. However, slightly fewer (n = 149) respondents were prepared to indicate what they considered to be the remit of the proposed specialty. Ninety-two respondents (55.1%) considered that it should be restricted to the provision of specialist care for adults with 'special needs'; the remaining 57 respondents felt that it should provide specialist care across the entire age spectrum. CONCLUSIONS: Among SPDs, there is overwhelming support for the creation of a specialty of SCD, the majority view being that this should be dedicated to the provision of specialist care for adults with 'special needs'.

Attitude of Health Personnel↗

Outcomes of orthotopic liver transplantation in Chile.

Our liver transplant program was started in 1993 in a private clinic and a public hospital. Thereafter, a rapid increase in adults and pediatric candidates for this therapeutic option lead to this analysis of results in 165 orthotopic liver transplants (OLT) in 143 patients between November 1993 and December 2002. Seventy-four OLT were performed in 66 adult patients and 91 in the pediatric group. Liver grafts came from cadaveric donors in 145 cases (74 adults and 71 children). The technique of living-related donor was utilized in 20 pediatric cases. Main indications for OLT in the adult group were HCV cirrhosis, primary biliary cirrhosis; biliary atresia and acute liver failure were the indications in pediatric patients. Retransplantation was needed for 23 patients, including 9 adults and 14 children. The most frequent causes of death were sepsis, graft primary nonfunction, and vascular complications. Actuarial survivals at 1 and 5 years were 80.7% and 72.6% for the adult group and 82% and 74.8% for the pediatric group, respectively. Our results are comparable to those published by large, experienced, international centers, with much better financial support.

Adolescent↗

Epidemiology and results of liver transplantation for acute liver failure in Chile.

Acute liver failure (ALF) is a severe, life-threatening condition associated with a high mortality rate. The objective of this study is to present the experience of a Chilean liver transplant program with orthotopic liver transplantation (OLT) for ALF. All patients with the diagnosis of ALF evaluated in our program between January 1995 and May 2003 were included in the analyses of etiology and outcomes. Candidates for OLT activated on a national waiting list were transplanted with cadaveric or living-related donor (LRD) organs. Twenty-seven patients age 1 to 19 years (median, 7.4 years) were transplanted at a median weight of 30.7 kg including 17 cadaveric and 10 with LRD livers. Most frequent etiologies were hepatitis A in 10 cases (37%) and unknown in 12 (48.1%). One donor experienced superficial phlebitis. Four patients were retransplanted (14.8%). Twenty patients are alive with 1- and 5-year survival rates of 74.1% At a median follow up of 34 months (range = 2 to 120). Seven patients died due to sepsis, multiorganic failure, graft primary nonfunction, intracranial hemorrhage, and intraoperative cardiac arrest. This experience revealed results comparable to international reports, allowing survival of patients destined to die.

Adolescent↗

Vital pulpotomy in the primary dentition: attitudes and practices of Specialists in Paediatric Dentistry practising in the United Kingdom.

OBJECTIVES: This study was designed to examine the attitudes and practices of Specialists in Paediatric Dentistry practising in the United Kingdom in relation to vital pulpotomy in the primary dentition. DESIGN: Data were collected by postal questionnaire. SAMPLE AND METHODS: All dentists whose names were entered on the General Dental Council's Specialist List in Paediatric Dentistry, and who were resident and practising in the United Kingdom, were asked to complete a questionnaire consisting of nine questions. RESULTS: Questionnaires were returned by 184 (86.8%) of the eligible Specialists in Paediatric Dentistry. These yielded a sample group of 179 Specialists practising the primary vital pulpotomy technique. The most commonly used medicament was formocresol, with a 1 : 5 dilution being used by 66.5% of the sample. More than half (54.2%) of the Specialists practising the technique expressed concern regarding their preferred medicament. Without exception, their concerns related to the potential adverse effects of formocresol and formaldehyde. Seventy-five respondents (41.9% of the sample) were considering changing their technique. One hundred and thirty-seven Specialists stated that they routinely took preoperative radiographs, while postoperative radiographs were routinely taken by 107 Specialists (76.5% and 59.8% of the sample, respectively). Rubber dam isolation was used by 62.6% of the sample. CONCLUSIONS: The majority of Specialists are adhering to the UK national clinical guideline for pulp treatment of the primary dentition. However, significant numbers are considering changing their chosen technique. There is a lack of consensus on the use of pre- and postoperative radiographs; specific guidance with respect to their use is required.

Attitude of Health Personnel↗

Increased primary tooth size in a 47,XXY male: a first case report.

Increased tooth size has previously been reported in association with Klinefelter syndrome. However, until now, this observation has been restricted to the permanent dentition. In this paper, we report increased mesio-distal width in the primary incisor and molar teeth of a 47,XXY male.

Child↗

Effect of hens fed dietary flaxseed with and without a fatty liver supplement on hepatic, plasma and production characteristics relevant to fatty liver haemorrhagic syndrome in laying hens.

1. Two long-term experiments were conducted with Single Comb White Leghorn (SCWL) hens (line UCD-003) predisposed to fatty liver haemorrhagic syndrome (FLHS). The first investigated the effect of adding a fatty liver supplement to the diet of laying hens prior to the onset of lay, and continuing either until peak production or throughout 39 weeks into lay. The second experiment, lasting 9 months into lay, investigated the effect of adding a fatty liver supplement, with or without 100 g/kg dietary ground flaxseed, to the diet. Body weight, feed intake, plasma triglycerides (in experiment 2) and egg production were measured throughout the experiment. Liver weight, liver fat content, liver malondialdehyde (MDA) content and liver haemorrhage score and fatty acid content of liver fat (in experiment 2) were measured at the end of each experiment. 2. In experiment 1, hens given diets containing the fatty liver supplement had higher egg production and eggshell strength, but there was no difference in liver parameters including MDA content or haemorrhage score compared with controls. 3. At the end of experiment 2, hens on 100 g/kg flaxseed diets had lower body weight, liver weight, liver dry matter and fat content, and plasma triglyceride concentrations than hens given the control diets. 4. Liver haemorrhage score was positively correlated with liver weight, but not with liver fat content, plasma triglyceride concentration or liver MDA content. This suggests that reducing the liver lipid content or feeding fatty liver supplements may not be as effective in controlling FLHS as controlling the size of the liver.

Animal Feed↗

General anaesthesia for exodontia in children: experience of a dental teaching hospital in relation to changes in national guidance.

OBJECTIVES: To examine, in relation to changes in national guidance, trends in the provision of general anaesthesia for exodontia in children. DESIGN: Retrospective analysis of hospital records. SETTING: University Dental Hospital, Cardiff, UK. METHODS: Data were drawn from records of services provided in each of four index months during 1989, 1991, 1997 and 1999 (the years immediately preceding/following the Poswillo Report of 1990 and the General Dental Council's revised guidance of 1998). RESULTS: In the first year after publication of the Poswillo report, there was a 19.6% increase in use of 'chair case' general anaesthesia over the four index months. Subsequently, upon the emergence of additional providers, patient flow in 1997 had fallen to 65.8% of that seen at baseline. Following the General Dental Council's revised guidance of 1998, there was a further reduction in the provision of 'chair case' general anaesthesia (to 40.8% of that seen at baseline). CONCLUSIONS: In this unit, throughput of children receiving general anaesthesia for exodontia has fluctuated in periods marked by changing national guidance. However, it is not possible to attribute such fluctuations in patient flow solely to this factor.

Adolescent↗

The selective serotonin reuptake inhibitor paroxetine is effective in more generalized and in less generalized social anxiety disorder.

RATIONALE: The DSM-IV includes the specifier "generalized" to refer to social anxiety disorder (social phobia) patients if the fears include "most social situations". The focus on interventions such as the selective serotonin reuptake inhibitors (SSRIs) for generalized social anxiety disorder arguably runs the risk that inadequate treatment will be provided to patients with the non-generalized or discrete subtypes. There are, however, few data to address whether more generalized and less generalized subgroups of social anxiety disorder differ in response to medication. OBJECTIVE: To compare response of more generalized and less generalized social anxiety disorder to pharmacotherapy. METHODS: Data from three randomized placebo-controlled double-blind multicenter trials of the SSRI paroxetine in social anxiety disorder were pooled. Response on the Clinical Global Impression Global Improvement item was analyzed using logistic regression, and change in total Liebowitz Social Anxiety Score was analyzed using analysis of variance, with both models incorporating treatment (paroxetine vs placebo), subgroup (more generalized vs less generalized), demographic, and clinical variables. RESULTS: The prevalence of more generalized social anxiety disorder was higher in females than in males. However, there was no significant difference in terms of age or clinical characteristics (duration of condition, baseline pulse, mean arterial blood pressure). At treatment endpoint there were significant treatment effects (for paroxetine vs placebo), but no significant subgroup effects (for more generalized vs less generalized). CONCLUSIONS: Although the current database is limited insofar as few patients with discrete social anxiety disorder would have been included, it is helpful in addressing the value of medication for patients lying on the spectrum from generalized to non-generalized and discrete social anxiety disorder. Paroxetine was effective in both more generalized and in less generalized social anxiety disorder.

Adult↗

Emotion work in midwifery: a review of current knowledge.

AIM OF THE PAPER: To review the literature relating to emotional labour in the workplace and identify potential sources of emotion within midwifery work. RATIONALE: There is substantial evidence to indicate that the quality of the relationship between midwife and woman is significant in determining the quality of the childbirth experience for women. Despite this, there is a notable lack of research regarding midwives' experiences of participating in this relationship, and even less regarding the emotional issues involved. METHOD: Literature review of relevant midwifery, nursing and sociological literature. Discussion of the theoretical perspectives provided by sociological and nursing research relating to the management of emotion at work and critical consideration of their application to an analysis of midwifery work. FINDINGS: Although these theoretical perspectives may offer significant insights of relevance to midwifery, there is much more that needs to be uncovered. Midwifery work has the potential for creating high levels of emotion work and current changes in the organization of United Kingdom (UK) maternity care may further increase this. CONCLUSION: It is essential that midwives develop their understanding of emotion at work in order to improve their own working lives, and to meet the needs of childbearing women and their families. More research is needed in this field to develop a body of knowledge to inform midwifery education and practice.

Adaptation, Psychological↗

Comparison of male and female junior athletes' self-perceptions and body image.

Few studies have examined sex differences in body perceptions in sports where the pressure to display a certain physique is reduced. The aim of the present study was to investigate sex differences in physical self-perceptions and body image in junior athletes who are involved in sprint kayaking, a sport where a low body weight is relatively unimportant. 12 male and 13 female members (aged 13-17 yr.) of the British Canoe Union Junior Development Squad for sprint-kayak racing completed the Physical Self-perception Profile for Children and the Multidimensional Body-Self Relations Questionnaire. Multivariate analysis of variance indicated that girls reported significandy higher Sports Competence and lower Appearance Orientation scores than boys. This study has raised a number of issues surrounding the physical self-perceptions and body image of junior athletes involved in a power- and strength-based sport. It seems that male athletes may also experience pressures to conform to a male version of a 'beautiful body'.

Body Constitution↗

Repair of incisional hernias.

Incisional hernias develop in up to 11% of surgical abdominal wounds with a possible recurrence following repair of 44%. We describe our experience with a combined fascial and prosthetic mesh repair. Thirty-five patients (16M:19F) have been treated. The original operation was bowel related in 19 cases, gynaecological in 8, hepatopancreaticobiliary in 3 patients, aortic aneurysm repair in 2 and involved a thoraco-laparotomy in 3. The incisions were midline in 26 cases, transverse in 6, paramedian in 2 and rooftop in one patient. The hernias were considered subjectively to be large in 15, medium in 14 and small in 6 of the patients. A proforma was completed for each patient noting intra-operative and post-operative complications, post-operative hospital stay and analgesic requirements. Post-operative complications included seroma formation in 6 patients, deep vein thrombosis in one and a non-fatal pulmonary embolism in another. One patient developed a wound haematoma and one had a superficial wound infection. Post-operative in-hospital stay ranged from 1 to 27 days with a mean of 6.2 days. Of the 35 patients 33 were available for follow-up. Follow-up was for a median of 20.3 months (range 6.0 to 54.1 months). Two of these (6%) patients reported a persistent lump and one (3%) reported persistent pain but none of the remaining 33 was found to have a recurrence. We advocate this technique because it is applicable to all hernias, most of the mesh is behind the rectus sheath and has 2 points of fixation, it is relatively pain-free allowing early mobilisation, has a modest complication rate and a low recurrence rate.

Aged↗