An interprofessional clinic for adults with Turner syndrome: the patient perspective.

OBJECTIVES: This qualitative study assessed the value of a primary care-based interprofessional clinical team for adults with Turner syndrome (TS) utilizing patient perspectives. METHODS: Ten patients within one institution's interprofessional adult TS clinic participated in one of two semi-structured focus groups. Content analysis was used to classify content provided by participants into themes and sub-themes using Dedoose software. RESULTS: Participants found that their quality of care and life were both improved due to the presence of the interprofessional Adults with TS Clinic. Specifically, participants reported that the clinic helped address problems with finding knowledgeable providers and care gaps, made appointments more convenient and improved interprofessional communication. Participants also reported that the clinic helped them find a sense of community and increased personal confidence. Study participants suggested improvements to the clinic by expanding the scope of practice further, simplifying processes to schedule appointments, and potentially creating interprofessional clinics for other rare diseases as well. CONCLUSION: Pursuing avenues to create interprofessional clinics for adults with rare diseases has value from the patient perspective. This value can translate to improved patient outcomes through improvements in patient knowledge of their diagnosis, adherence to evidence-based care and quality of life.

The positive role of breastfeeding on infant health during the first 6 weeks: findings from a prospective observational study based on maternal reports.

This study aimed to report on adverse infant and maternal clinical outcomes, and investigate the relationship between infant feeding practice and such adverse clinical outcomes in infants during the first 6 weeks postpartum. From an eligible sample of 450 mother-term infant pairs recruited from the Coombe Women and Infants University Hospital in Dublin, 27.1% of infants (n=122) were maternally reported to have had an illness during the first 6 weeks that necessitated the provision of prescribed medication +/- general practitioner/paediatrician attendance +/- hospitalisation. Of these, 90 infants had > or =1 episode of infection +/- viral +/- gastro-intestinal-related condition. After adjustment, 'any' breastfeeding to 6 weeks was protective against such adverse infant outcomes (adjusted odds ratio [aOR] 0.44, P = 0.022). Attendance to the GP/paediatrician for > 1 visit (aOR 3.44, P = 0.000) and multiparity (aOR 1.76, P = 0.041) were also positively associated with such adverse infant outcomes. To decrease infant morbidity rates in Ireland, government investment in breastfeeding promotion, support and research should be a continued public health priority.

Weaning onto solid foods: some of the challenges.

Weaning--the transition from milk to solid food--influences life-long health. Dietary challenges during weaning include providing sufficient critical nutrients such as iron with minimal added sugar and fat and no added salt. This study assessed the inclusion of iron-containing red meat in infant diets before age one year, and the Irish commercial baby food environment. Of mothers with an infant under 30 months of age who were surveyed in shopping centres in Ireland (n195), 82% (n159) reported wanting more weaning information. A quarter (n24) of infants over age 12 months (n97) received no iron-containing red meat before age one year. A scan of commercial baby foods in Ireland identified 448 products. While all complied with baby food legislation, 15% (n69) were intrinsically high in sugar and fat, or contained added salt. This study indicates the need for specific guidance on best infant feeding practice in Ireland.

Maternal and infant nutritional supplementation practices in Ireland: implications for clinicians and policymakers.

This prospective Irish observational study examined maternal and infant nutritional supplement use. From an initial sample of 539 mothers recruited from the Coombe Women and Infants University Hospital in Dublin (during 2004-2006), 450 eligible mothers were followed up at 6 weeks and 6 months postpartum. Only 200 women (44.4%) complied with peri-conceptional folic acid at the recommended time with strong social patterning associated with its uptake. Almost 10% of the sample (n = 44) consumed a combined multivitamin and mineral supplement during pregnancy. A vitamin D-containing supplement was provided to only 5 (1.1%) and 15 (3.3%) infants at 6 weeks and 6 months, respectively. A national guideline that advises on adequate and safe use of both vitamin and multivitamin supplements during pregnancy with particular reference to vitamin A and D is warranted. Given the re-emergence of rickets in Ireland, and the reported morbidities associated with vitamin D insufficiency, promoting and monitoring compliance with 200 IU [5 microg] daily vitamin D supplements to all infants particularly those from higher risk groups from birth to 1 year, should be a public health priority.

Maternal health behaviours during pregnancy in an Irish obstetric population and their associations with socio-demographic and infant characteristics.

BACKGROUND/OBJECTIVES: To examine the prevalence and combined occurrence of peri-conceptional folic acid (FA) supplement use, smoking and alcohol consumption during pregnancy in a sample of women in Dublin, and determine the factors associated with these health behaviours. SUBJECTS/METHODS: A prospective observational study (2004-2006) involving the recruitment of 491 pregnant women from antenatal clinics in a Dublin maternity hospital, with postpartum follow-up of 450 eligible mothers. Data on FA use, maternal smoking and alcohol consumption patterns during pregnancy were collected from the antenatal patient-administered questionnaire, which was completed by participants, and returned to the investigator on the day of recruitment. RESULTS: The median gestational age of women at recruitment was 36 weeks. A combined 24.2% of mothers commenced FA at the recommended time, avoided alcohol consumption and smoking during pregnancy. In all, 35.3% of mothers reported to consuming alcohol, 20.9% smoked during pregnancy and 44.4% commenced FA at the recommended time. Mothers <25 years were more likely to have not taken FA at the recommended time (adjusted odds ratio (aOR): 4.0, 95% confidence interval (CI): 1.64-9.77) and were more likely to have smoked during pregnancy (aOR: 3.56, 95% CI: 1.32-9.57). Irish nationality positively predicted both alcohol consumption (aOR: 4.37, 95% CI: 1.88-10.15) and smoking (aOR: 10.92, 95% CI: 1.35-87.98) during pregnancy. CONCLUSIONS: Educational efforts are still necessary to convince women of Irish nationality, in particular, of the adverse effects of smoking and alcohol consumption on fetal outcome. Women <25 years should be specifically targeted in smoking cessation and FA promotional campaigns.

The effect of dietary supplements of omega 3 polyunsaturated fatty acids on the fatty acid composition of platelets and plasma choline phosphoglycerides.

1. The effects of dietary supplements of omega 3 polyunsaturated fatty acids on the fatty acid composition of platelets and plasma choline phosphoglycerides were studied in vegans and in omnivores. 2. A supplement of 18:3 omega 3 led to an increase in 20:5 omega 3 but was less effective than one of 20:5 omega 3 + 22:6 omega 3.

Effect of zinc supplementation on oxidative drug metabolism in patients with hepatic cirrhosis.

The pharmacokinetics of antipyrine were studied in seven zinc deficient patients with hepatic cirrhosis, before and after zinc supplementation. Each patient received zinc sulphate 660 mg daily for 30 days, restoring zinc status to normal as assessed by leucocyte zinc concentration. Antipyrine clearance was significantly reduced (P less than 0.05) and antipyrine elimination half-life increased (P less than 0.05) following administration of zinc sulphate without significant alteration in the apparent volume of distribution. It is concluded that supplementation of the zinc deficiency associated with hepatic cirrhosis impaired the hepatic oxidative metabolism of antipyrine.

Is the metacarpal index useful in the diagnosis of Marfan syndrome?

AIM: This study was undertaken to analyse the importance of the Metacarpal Index (MCI) in making the diagnosis of Marfan syndrome. PATIENTS AND METHODS: The characteristics of 42 patients with Marfan syndrome as defined by strict criteria were analysed. Fifty-one consecutive accident and emergency patients comprised the control group for the MCI measurements. RESULTS: Using MCI alone four (7.8%) of controls had an abnormal MCI, and seven (16.6%) of Marfan patients were normal. Thirty-two (76%) of Marfan patients had an abnormal MCI and three (13.4%) were equivocal. Using other skeletal parameters (upper segment to lower segment ratio, arm span 3 cm or more greater than height, or palate, pectus or scoliosis deformity) 40 (95%) of the Marfan group had an abnormality. Other parameters: 18 (43%) in the Marfan group gave a history of retinal detachment or ectopia lentis. Echocardiographic measurements showed aortic root dilatation in 29 (69%) and in 21 (50%) mitral valve prolapse was found; 37 (88%) had one or other cardiac abnormality. Using all parameters excluding MCI: All the patients would still have fulfilled the diagnostic criteria for the diagnosis of Marfan syndrome. CONCLUSION: The role of the MCI, a radiation dependent and time consuming measurement, is probably insignificant in diagnosis of the majority of Marfan patients. Combined with clinical measurements, an echocardiogram is probably the single most useful investigation, aiding both diagnosis and management.

Subcapsular fluid entrapment in extracapsular cataract surgery.

We report five cases of sudden shallowing of the anterior chamber associated with irrigation under the iris. We suspect that irrigation in this context forces fluids through the zonules into the posterior chamber, where it becomes trapped, causing anterior chamber shallowing. In our cases, this phenomenon was most commonly associated with irrigation during cortex removal in cases in which the cornea was open (associated with corneal transplantation) and with zonular dehiscence.

Sustained mental stress alters human jejunal motor activity.

The effect of prolonged mental stress on upper small bowel motility was studied in 11 healthy, medical students using a pressure-sensitive radio-pill. During eight hours of continuous observation, subjects were stressed for four hours with a modified dichotomous listening test. During the first two hours of stress, inhibition of fasting motor complexes occurred and this effect was marked in the seven subjects who showed an appreciable cardiovascular response to stress.

Lipids and fatty acids and their relationship to restenosis.

One hundred consecutive patients had fasting lipids and percutaneous fat biopsy performed at the time of percutaneous transluminal coronary angioplasty to determine if there was an association between restenosis and lipids or fatty acids. Angiographic follow-up and complete lipid and fatty acid results were available in 82 patients. Restenosis occurred in 37/82 (45%). Total cholesterol, triglyceride, high density lipoprotein, low density lipoprotein, and apolipoproteins A1 and B were not associated with restenosis. There was a significantly lower level of the monounsaturated fat palmitoleic acid (p less than 0.02), a trend towards a lower level of the monounsaturated fat oleic acid (p less than 0.09), and a trend towards a higher level of the saturated fat palmitic acid (p less than 0.08) in the restenosis group. The polyunsaturated fatty acids were not associated with restenosis. We conclude that lipids are not significantly associated with restenosis, and that lower levels of monounsaturated fatty acids may increase the risk of restenosis.