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Biomedical subjects

Ristan M Greer

Publications and source records attributed to Ristan M Greer.

6 recordsLinked to original sources

Pubertal development and its influences on bone mineral density in Australian children and adolescents with cystic fibrosis.

BACKGROUND: Pubertal delay is thought to contribute to suboptimal peak bone mass acquisition in young people with cystic fibrosis (CF), leading to an increased fracture incidence. This study aims to compare pubertal development in young people with CF with that of a local healthy population and assess the influence it has on areal bone mineral density (aBMD). METHODS: Tanner stage, age of menarche, bone age (BA), sex hormone levels and aBMD were examined in 85 individuals with CF (aged 5.3-18.1 years, 39 females) and 100 local controls (5.6-17.9 years, 54 females). RESULTS: Tanner stage and age of menarche were not significantly different between controls and CF. Tanner stage-adjusted mean values for follicle stimulating hormone (FSH), luteinizing hormone (LH) and testosterone (T) were lower in males with CF (FSH: P = 0.004, LH: P = 0.01 and T: P = 0.002). Bone age was delayed in adolescents with CF compared to controls (chronological age-BA: controls = 0.13 years (SE = 0.16), CF = 0.95 years (SE = 0.22), P = 0.003). Areal bone mineral density (adjusted for age, sex, height and lean tissue mass) was not significantly different between CF and controls. Moderate negative correlations were found between delayed BA and weight (r = -0.41, P < 0.001) and height (r = -0.41, P < 0.001). CONCLUSIONS: There was no evidence of clinical pubertal delay or low aBMD (adjusted for short stature and lean tissue mass) in young people with CF when compared with a local population, despite lower nutritional markers, height and weight and delayed skeletal maturation.

Adolescent↗

Vitamin A levels in patients with CF are influenced by the inflammatory response.

BACKGROUND: Serum vitamin A, normally depressed in inflammatory conditions, is frequently low in people with CF. Vitamin A is important in respiratory epithelial regeneration and repair. We hypothesised that serum vitamin A would be associated with inflammation and disease severity. METHODS: Serum vitamin A (as retinol), C-reactive protein (CRP), vitamin E, 25-hydroxy vitamin D (25OHD), 1,25-dihydroxy vitamin D (1,25(OH)(2)D), weight, and lumbar spine bone mineral density (LSBMD) were measured in 138 subjects with CF (5-56 years) and 138 control subjects (5-48 years). FEV(1), presence of CF liver disease (CFLD) and hospital admissions were recorded in those with CF. RESULTS: Serum vitamin A level was lower in CF subjects than in controls (mean, 95% CI: 1.29, 1.0-1.37 vs. 1.80, 1.7-1.87 micromol/l, p < 0.0001), and inversely correlated with CRP (r(s) = -0.37, p < 0.0001). CF subjects with low vitamin A (45%) level had poorer FEV(1), weight z-score, LSBMD z-score, and higher CRP compared with those with normal levels. In the CF group CRP, vitamin E, 1,25(OH)(2)D, presence of CFLD, admissions, and age were associated with vitamin A level. CONCLUSIONS: Serum vitamin A is negatively associated with CRP in subjects with CF, consistent with normal population studies. It is important to distinguish between low serum vitamin A associated with the inflammatory response and that due to poor nutritional stores. The role of vitamin A in CF warrants further study, in the contexts both of chronic recurrent inflammatory disease and acute pulmonary exacerbation.

Adolescent↗

Adjusting body cell mass for size in women of differing nutritional status.

BACKGROUND: Body cell mass (BCM) may be estimated in clinical practice to assess functional nutritional status, eg, in patients with anorexia nervosa. Interpretation of the data, especially in younger patients who are still growing, requires appropriate adjustment for size. Previous investigations of this general issue have addressed chemical rather than functional components of body composition and have not considered patients at the extremes of nutritional status, in whom the ability to make longitudinal comparisons is of particular importance. OBJECTIVE: Our objective was to determine the power by which height should be raised to adjust BCM for height in women of differing nutritional status. DESIGN: BCM was estimated by (40)K counting in 58 healthy women, 33 healthy female adolescents, and 75 female adolescents with anorexia nervosa. The relation between BCM and height was explored in each group by using log-log regression analysis. RESULTS: The powers by which height should be raised to adjust BCM were 1.73, 1.73, and 2.07 in the women, healthy female adolescents, and anorexic female adolescents, respectively. A simplified version of the index, BCM/height(2), was appropriate for all 3 categories and was negligibly correlated with height. CONCLUSIONS: In normal-weight women, the relation between height and BCM is consistent with that reported previously between height and fat-free mass. Although the consistency of the relation between BCM and fat-free mass decreases with increasing weight loss, the relation between height and BCM is not significantly different between normal-weight and underweight women. The index BCM/height(2) is easy to calculate and applicable to both healthy and underweight women. This information may be helpful in interpreting body-composition data in clinical practice.

Adolescent↗

Panoptic versus conventional ophthalmoscope.

BACKGROUND: The 'ease of use' and accuracy in measurement of the vertical optic cup/disc ratio (VCDR) was compared between the conventional direct ophthalmoscope (CO) and Panoptic direct ophthalmoscope (PO) in a group of 'naïve' first year medical students to determine which would be more suitable for non-ophthalmologists. METHODS: In this quasi-randomized method comparison study, eight students received an introductory session on ophthalmoscopy then examined 18 eyes (9 left, 9 right) with each ophthalmoscope in a private practice. The subjects were the eight students themselves plus two other subjects. Each subject (n = 10) had one eye dilated. Students determined a VCDR and a subjective score of 'ease of use' on a scale of 1 (difficult) to 10 (easy). A consultant ophthalmologist (GAG) determined the benchmark VCDR for each eye with each ophthalmoscope. RESULTS: Of 288 eye examinations, there were 111 measurements of VCDR using the CO (47 undilated, 64 dilated), and 140 measurements using the PO (75 undilated, 65 dilated). Differences in the students' estimated VCDR and the benchmark were similar for the CO and PO (P = 0.67). 'Ease of use' was scored in 288 eyes and the median score was higher in the PO overall (CO: median 8, IQR 6-9; PO median 9, IQR 8-10; P < 0.0001), and within each session (P < 0.0001 for each session). CONCLUSIONS: Medical students found the PO much easier to use, with accuracy of rating the VCDR similar to the CO. This comparison would support the wider use of the PO amongst medical students, general practitioners and other primary care providers.

Adolescent↗

Abnormalities of the PTH-vitamin D axis and bone turnover markers in children, adolescents and adults with cystic fibrosis: comparison with healthy controls.

Abnormalities of calcium and vitamin D metabolism in cystic fibrosis (CF) are well documented. We tested the hypothesis that alterations in calcium metabolism are related to vitamin D deficiency, and that bone resorption is increased relative to accretion in patients with CF. Calcitropic hormones, electrolytes, osteocalcin (OC) and bone alkaline phosphatase (BAP), (markers of bone mineralisation), urinary deoxypyridinoline [total (t) Dpd, a marker of bone resorption] and lumbar spine bone mineral density (LS BMD), expressed as a z-score, were measured in 149 (81 M) CF and 141 (61 M) control children aged 5.3-10.99 years, adolescents aged 11-17.99 years and adults aged 18-55.9 years. Data were analysed by multiple regression to adjust for age. In patients, FEV(1)% predicted and CRP (as disease severity markers), genotype and pancreatic status (PS) were recorded. The distribution of PTH differed between groups ( P<0.0001), with CF levels both below and above the control range. 25OH vitamin D (25OHD) was not different in control and CF subjects ( P=0.06). Active hormonal vitamin D (1,25(OH)(2)D) was lower in the CF group ( P<0.0001), not explained by 25OHD or disease severity, as was serum magnesium ( P<0.0001). OC was decreased in CF adults ( P=0.004), and tDpd increased in CF adolescents ( P=0.003) and adults ( P=0.03). The ratio of OC to tDpd (a measure of bone coupling) was similar in CF and control children, but decreased in CF adolescents ( P=0.04) and adults ( P=0.02), suggesting decreased overall bone accrual in CF adolescents and uncoupling of bone balance in adults. 1,25(OH)2D was weakly correlated with OC in CF children ( r=0.43, P=0.01), and with tDpd in CF and control adolescents ( r=0.33, P=0.05 and r=0.36, P=0.02, respectively); thus there was limited evidence of association of calcitropic hormones, which had an abnormal pattern in all age groups, with bone turnover. There was no association between calcitropic hormones or bone turnover markers and LS BMD z-score. Despite vitamin D sufficiency, abnormalities of calcium metabolism and bone turnover markers were still apparent and bone accretion was decreased relative to resorption in the CF adolescent and adult groups. These changes were not fully explained by disease severity or genotype, but are consistent with reports of decreased BMD and unique bone histomorphometry in older subjects with CF.

Adolescent↗

The outcome in Australian children with hyperinsulinism of infancy: early extensive surgery in severe cases lowers risk of diabetes.

AIMS: Hyperinsulinism of infancy (HI) is characterized by unregulated insulin secretion in the presence of hypoglycaemia, often resulting in brain damage. Pancreatic resection for control of hypoglycaemia is frequently resisted because of the risk of diabetes mellitus (DM). We investigated retrospectively 62 children with HI from nine Australian treatment centres born between 1972 and 1998, comparing endocrine and neurological outcome in 28 patients receiving medical therapy alone with 34 who required pancreatic resection to control their hypoglycaemia. METHODS: History, treatment and clinical course were ascertained from file audit and interview. Risk of DM (hazard ratio) attributable to age at surgery (< vs. > or = 100 days at last pancreatectomy) and extent of resection (< vs. > or = 95%) were calculated using Cox proportional hazards regression and categorical variables compared by the chi2-test. Neurological outcome (normal, mild deficit or severe deficit) was derived from the most authoritative source. RESULTS: Surgically treated patients had a greater birthweight, earlier presentation and higher plasma insulin levels. Of 18 infants < 100 days and 16 > or = 100 days of age at surgery, four (all > or = 100 days) became diabetic as an immediate consequence of surgery and five (two < 100 days and three > or = 100 days) became diabetic 7-18 years later. Surgery > or = 100 days and pancreatectomy > or = 95% were associated with development of diabetes (HR = 12.61, CI 1.53-104.07 and HR = 7.03, CI 1.43-34.58, respectively). Neurodevelopmental outcome was no different between the surgical and medical groups with 44% overall with neurological deficits. Patients euglycaemic within 35 days of the first symptom of hypoglycaemia (Group A) had a better neurodevelopmental outcome than those still hypoglycaemic > 35 days from first presentation (Group B) (P = 0.007). Prolonged hypoglycaemia in Group B was due either to delayed diagnosis or to need for repeat surgery because of continued hypoglycaemia. Within Group A, medically treated patients (who presented later with apparently milder disease) had a higher incidence of neurodevelopmental deficit (n = 15, four mild, three severe deficit) compared with surgically treated patients (n = 18, two mild, none severe deficit) (P < 0.025). CONCLUSIONS: Poor neurodevelopmental outcome remains a major problem in hyperinsulinism of infancy. Risk of diabetes mellitus with pancreatectomy varies according to age at surgery and extent of resection. Patients presenting early with severe disease have a better neurodevelopmental outcome and lower risk of diabetes if they are treated with early extensive surgery.

Age of Onset↗