Developing a digitally innovative ethics and professionalism curriculum for neonatal-perinatal medicine fellows: a 3-year multicenter pilot study.

OBJECTIVES: The purpose of this study was to develop and regionally pilot a digitally innovative curriculum in ethics and professionalism in neonatology and study the effects on trainee knowledge and confidence. STUDY DESIGN: We developed 13 modules in ethics for neonatology fellows and piloted them at three academic institutions utilizing a flipped-classroom approach. Baseline surveys in ethics knowledge and confidence in approaching ethical dilemmas were compared with repeat surveys after curriculum completion. Pre- and post-tests were also administered for all 13 modules. RESULTS: Forty-four of 49 eligible fellows participated (90% response rate). Pre/post comparisons demonstrated significant improvements in overall knowledge and in 8/13 modules, as well as improvement in overall confidence and individually when navigating 16/22 ethical dilemmas. CONCLUSIONS: After completing this curriculum, participants' knowledge scores and reported confidence in approaching ethical challenges significantly improved. Future steps include assessing the effects of this innovative curriculum via an ongoing international pilot.

A low cost, re-usable electricity-free infant warmer: evaluation of safety, effectiveness and feasibiliy.

Setting: Rural Rwandan hospitals, where thermoregulation is critical yet a challenge for pre-term, low-birth-weight (LBW) or sick newborns. Objective: To assess the safety, effectiveness, and feasibility of an inexpensive, reusable, non-electric warmer to complement kangaroo mother care (KMC). Methods: Prospective single-arm, non-randomized intervention study. Enrolled infants were hypothermic or at risk of hypothermia due to prematurity/LBW. Infants used the warmer in conjunction with KMC or as the sole source of external heat. Temperatures of the infant, warmer and air were measured for up to 6 h. Results: Overall, 33 patients used the warmer for 102 encounters: 43 hypothermic and 59 at risk of hypothermia. In 7/102 encounters (7%), the infant developed a temperature of >37.5°C (37.6°-38.2°C). For 43 hypothermic encounters and 59 at-risk encounters, hypothermia was corrected/prevented in respectively 41 (95%) and 59 (100%) instances. The warmer maintained goal temperature for the study duration in ⩾85% of uses. Two/12 warmers broke down after <10 uses. In no instances was the warmer used incorrectly. Conclusion: Our results are promising for this prototype design, and warrant testing on a wider scale.

Contexte : Des hôpitaux ruraux du Rwanda où la thermorégulation est cruciale mais complexe pour les nouveaux-nés prématurés, de faible poids de naissance (LBW) ou malades.Objectif : Evaluer la sécurité, l'efficacité et la faisabilité d'un réchauffeur peu coûteux, réutilisable et non électrique pour compléter la méthode kangourou (KMC).Méthode : Etude rétrospective d'intervention à un seul bras, non randomisée. Les nouveaux-nés enrôlés étaient en hypothermie ou à risque d'hypothermie liée à la prématurité ou au LBW. Les nouveaux-nés ont bénéficié du réchauffeur en conjonction avec la méthode KMC ou comme source unique de chaleur externe. Les températures des bébés, du réchauffeur et de l'air ont été mesurées pendant 6 h.Résultats : Ont bénéficié du réchauffeur 33 patients pour un total de 102 utilisations ; 43 étaient en hypothermie et 59 à risque d'hypothermie. Dans 7/102 utilisations (7%), le bébé a atteint une température de >37,5°C (37,6°­38,2°C). Dans 43 cas d'hypothermie et 59 cas à risque, l'hypothermie a été corrigée/prévenue dans 41 (95%) et 59 (100%) instances, respectivement. Le réchauffeur a maintenu la température souhaitée pendant la durée de l'étude dans ≥85% des utilisations. Deux réchauffeurs sur 12 ont été hors d'usage après moins de 10 utilisations. Il n'y a jamais eu d'utilisation incorrecte.Conclusion : Nos résultats sont prometteurs en ce qui concerne la conception de ce prototype et ils justifient une évaluation à plus grande échelle.

Marco de Referencia: En varios hospitales rurales de Rwanda, la termorregulación que es fundamental para los recién nacidos con bajo peso al nacer o enfermos, plantea dificultades.Objetivo: Evaluar la seguridad, la eficacia y la factibilidad de un dispositivo no eléctrico, de bajo costo y reutilizable que genera calor como complemento al método de la madre canguro (KMC).Métodos: Fue este un estudio prospectivo de intervención con un solo grupo, no aleatorizado. Se incluyeron lactantes que ya sea, estaban hipotérmicos o expuestos a la hipotermia debido a su prematuridad o el bajo peso al nacer. Con estos lactantes, se utilizó el calentador como fuente externa exclusiva de calor o en asociación con el KMC. Se midieron las temperaturas del lactante, el calentador y la temperatura ambiente durante un máximo de 6 h.Resultados: Se utilizó el dispositivo en 102 encuentros con 33 pacientes, de los cuales 43 estaban hipotérmicos y 59 estaban en riesgo de entrar en hipotermia. En siete de los 102 encuentros (7%), el lactante alcanzó una temperatura superior a 37,5°C (37,6°­38,2°C). La hipotermia se corrigió en 41 de los 43 encuentros con lactantes hipotérmicos (95%) y se evitó en 59 de las 59 ocasiones con bebés expuestos (100%). El calentador mantuvo la temperatura buscada durante todo el estudio en ≥85% de los encuentros en los cuales se utilizó. Dos de los 12 dispositivos exhibieron degradación después de menos de 10 utilizaciones. En ningún caso se utilizó el calentador de manera incorrecta.Conclusión: Los resultados obtenidos con este método prototipo son promisorios y se justifica realizar un ensayo clínico de mayor escala.

Calorically restricted diets decrease PCSK9 in overweight adolescents.

BACKGROUND AND AIMS: Nutritional therapy is the first line approach to treatment of hyperlipidemia in childhood. Proprotein convertase subtilisin kexin type 9 (PCSK9) is a key regulator of plasma cholesterol levels and a target of novel lipid-lowering pharmacotherapies. We examined the effects of an intensive nutritional intervention on PCSK9 levels in overweight adolescents with cardiovascular disease (CVD) risk factors. METHODS AND RESULTS: Twenty seven obese and overweight adolescents with CVD risk factors were assigned to either a low fat or low glycemic load diet. During an 8-week "Intensive Phase," assigned meals were delivered to the home, and all participants received weekly in-person home nutrition counseling and phone calls. The subjects then underwent a 4-month "Maintenance Phase" without food provision and with no in-person contact. Anthropometric measurements, laboratory data, and serum PCSK9 protein levels were measured at baseline, 8 weeks, and 6 months. PCSK9 decreased by 16.5% at 8 weeks (201.2 ± 56.3 vs 165.6 ± 58.4 ng/mL; p < 0.001); PCSK9 levels returned to baseline levels at 6 months, after the Maintenance Phase. Change in PCSK9 was associated with change in fasting insulin, HOMA-IR, and AUC insulin, independent of weight loss. CONCLUSIONS: PCSK9 decreased in youth participating in an intensive dietary intervention. Change in HOMA-IR was associated with change in PCSK9, independent of weight loss, suggesting an important relationship with insulin sensitivity. ClinicalTrials.gov Identifier: NCT01080339.

The ability of low-magnitude mechanical signals to normalize bone turnover in adolescents hospitalized for anorexia nervosa.

We sought to determine whether low-magnitude mechanical stimulation (LMMS) normalizes bone turnover among adolescents hospitalized for anorexia nervosa (AN). Brief, daily LMMS prevents the decline in bone turnover typically seen during bed rest in AN. LMMS may have application for patients with AN in the inpatient setting to protect bone health. INTRODUCTION: Malnourished adolescents with AN requiring medical hospitalization are at high risk for rapid reduction in skeletal quality. Even short-term bed rest can suppress normal patterns of bone turnover. We sought to determine whether LMMS normalizes bone turnover among adolescents hospitalized for complications of AN. METHODS: In this randomized, double-blind trial, we prospectively enrolled adolescent females (n = 41) with AN, age 16.3 ± 1.9 years (mean ± SD) and BMI 15.6 ± 1.7 kg/m2. Participants were randomized to stand on a platform delivering LMMS (0.3 g at 32-37 Hz) or placebo platform for 10 min/day for 5 days. Serum markers of bone formation [bone-specific alkaline phosphatase (BSAP)], turnover [osteocalcin (OC)], and bone resorption [serum C-telopeptides (CTx)] were measured. From a random coefficients model, we constructed estimates and confidence intervals for all outcomes. RESULTS: BSAP decreased by 2.8% per day in the placebo arm (p = 0.03) but remained stable in the LMMS group (p = 0.51, pdiff = 0.04). CTx did not change with placebo (p = 0.56) but increased in the LMMS arm (+6.2% per day, p = 0.04; pdiff = 0.01). Serum OC did not change in either group (p > 0.70). CONCLUSIONS: Bed rest during hospitalization for patients with AN is associated with a suppression of bone turnover, which may contribute to diminished bone quality. Brief, daily LMMS prevents a decline in bone turnover during bed rest in AN. Protocols prescribing strict bed rest may not be appropriate for protecting bone health for these patients. LMMS may have application for these patients in the inpatient setting.

Creating an integrated care model for childhood obesity: a randomized pilot study utilizing telehealth in a community primary care setting.

In an integrated care model, involving primary care providers (PCPs) and obesity specialists, telehealth may be useful for overcoming barriers to treating childhood obesity. We conducted a pilot study comparing body mass index (BMI) changes between two arms (i) PCP in-person clinic visits plus obesity specialist tele-visits ( PCP visits + specialist tele-visits) and (ii) PCP in-person clinic visits only ( PCP visits only), with ongoing tele-consultation between PCPs and obesity specialists for both arms. Patients (N = 40, 10-17 years, BMI ≥ 95th percentile) were randomized to Group 1 or 2. Both groups had PCP visits every 3 months for 12 months. Using a cross-over protocol, Group 1 had PCP visits + specialist tele-visits during the first 6 months and PCP visits only during the second 6 months, and Group 2 followed the opposite sequence. Each of 12 tele-visits was conducted by a dietitian or psychologist with a patient and parent. Retention rates were 90% at 6 months and 80% at 12 months. BMI (z-score) decreased more for Group 1 (started with PCP visits + specialist tele-visits) vs. Group 2 (started with PCP visits only) at 3 months (-0.11 vs. -0.05, P = 0.049) following frequent tele-visits. At 6 months (primary outcome), BMI was lower than baseline within Group 1 (-0.11, P = 0.0006) but not Group 2 (-0.06, P = 0.08); however, decrease in BMI at 6 months did not differ between groups. After crossover, BMI remained lower than baseline for Group 1 and dropped below baseline for Group 2. An integrated care model utilizing telehealth holds promise for treating children with obesity.

Skeletal outcomes by peripheral quantitative computed tomography and dual-energy X-ray absorptiometry in adolescent girls with anorexia nervosa.

We conducted the first comparison of dual-energy X-ray absorptiometry (DXA) and peripheral quantitative computed tomography (pQCT) outcomes in adolescent girls with anorexia nervosa. We observed deficits in bone density by both tools. pQCT assessments were associated with many of the same clinical parameters as have been previously established for DXA. INTRODUCTION: Adolescents with anorexia nervosa (AN) commonly exhibit bone loss, but effects on bone geometry are less clear. We compared measures obtained by DXA and pQCT in girls with AN. METHODS: Seventy females (age 15.5 ± 1.9 years ) with AN and 132 normal-weighted controls underwent tibial measures by pQCT including trabecular volumetric bone mineral density (vBMD) at the 3 % site, cortical vBMD and dimensions at the 38 % site, and muscle cross-sectional area (CSA) at the 66 % site. Participants with AN also underwent standard DXA measures. Independent t tests compared the pQCT results, while Pearson coefficient assessed correlations among DXA and pQCT measures. RESULTS: Trabecular vBMD Z-scores were lower in AN compared to controls (AN -0.31 ± 1.42 vs +0.11 ± 1.01, p = 0.01) and cortical vBMD Z-scores were higher (AN +0.18 ± 0.92 vs -0.50 ± 0.88, p < 0.001). Trabecular vBMD and cortical CSA Z-scores positively correlated with DXA BMD Z-scores (r range 0.57-0.82, p < 0.001). Markers of nutritional status positively correlated with Z-scores for trabecular vBMD, cortical CSA, section modulus, and muscle CSA (p < 0.04 for all). CONCLUSIONS: This study is the first to compare DXA and pQCT measurements in adolescent girls with AN. We observed deficits in BMD by both DXA and pQCT. pQCT assessments correlated well with DXA bone and body composition measures and were associated with many of the same clinical parameters and disease severity markers as have been previously established for DXA. The differences in cortical vBMD merit further study.

A randomized pilot study of dietary treatments for polycystic ovary syndrome in adolescents.

BACKGROUND: Evidence is lacking to recommend one diet over another when treating polycystic ovary syndrome (PCOS). OBJECTIVES: To obtain preliminary data, comparing the impact of a low-glycaemic load (LGL) vs. low-fat (LF) diet on biochemical hyperandrogenism in overweight and obese adolescents with PCOS. To ascertain feasibility of recruiting study participants, in partnership with an adolescent clinic, and implementing dietary interventions. METHODS: Randomized controlled trial of 19 overweight and obese adolescents with PCOS and not using hormonal contraceptives (HCs). Interventions comprised nutrition education, dietary counselling and cooking workshops to foster adherence to a LGL (45% carbohydrate, 35% fat, 20% protein) or LF (55% carbohydrate, 25% fat, 20% protein) diet over 6 months. Serum bioavailable testosterone was the primary outcome. RESULTS: Sixteen (LGL, n = 7; LF, n = 9) participants completed the study. Body fat percentage decreased (P < 0.05) in response to the interventions, with no difference between the LGL and LF groups (-1.2% vs. -2.2%; P = 0.16). Bioavailable testosterone did not change for either group (-0.4 vs. -1.8 ng dL(-1) ; P = 0.35). Regarding feasibility, recruiting adolescents posed a challenge, and use of HCs was a main reason for ineligibility. Participants attended 5.9 of 6 in-person visits and 2.6 of 3 cooking workshops, completed 4.9 of 6 telephone counselling calls, and reported high satisfaction with the diets and cooking workshops (≥8 on a 10-cm scale). CONCLUSIONS: Dietary interventions were beneficial for weight control but did not attenuate biochemical hyperandrogenism. Innovative strategies are needed to recruit adolescents for studies aimed at assessing independent effects of diet on features of PCOS.

Skeletal health in adult patients with classic galactosemia.

SUMMARY: This study evaluated bone health in adults with galactosemia. Associations between bone mineral density (BMD) and nutritional and biochemical variables were explored. Calcium level predicted hip and spine BMD, and gonadotropin levels were inversely associated with spinal BMD in women. These results afford insights into management strategies for these patients. INTRODUCTION: Bone loss is a complication of galactosemia. Dietary restriction, primary ovarian insufficiency in women, and disease-related alterations of bone metabolism may contribute. This study examined relationships between clinical factors and BMD in patients with galactosemia. METHODS: This cross-sectional sample included 33 adults (16 women) with classic galactosemia, mean age 32.0 ± 11.8 years. BMD was measured by dual-energy X-ray absorptiometry, and was correlated with age, height, weight, fractures, nutritional factors, hormonal status, and bone biomarkers. RESULTS: There was a significant difference in hip BMD between women and men (0.799 vs. 0.896 g/cm(2), p = 0.014). The percentage of subjects with BMD-Z <-2.0 was also greater for women than men [33 vs. 18 % (spine), 27 vs. 6 % (hip)], and more women reported sustaining fractures. Bivariate analyses yielded correlations between BMI and BMD-Z [at the hip in women (r = 0.58, p < 0.05) and spine in men (r = 0.53, p < 0.05)]. In women, weight was also correlated with BMD-Z (r = 0.57, p < 0.05 at hip), and C-telopeptides (r = -0.59 at spine and -0.63 hip, p < 0.05) and osteocalcin (r = -0.71 at spine and -0.72 hip, p < 0.05) were inversely correlated with BMD-Z. In final regression models, higher gonadotropin levels were associated with lower spinal BMD in women (p = 0.017); serum calcium was a significant predictor of hip (p = 0.014) and spine (p = 0.013) BMD in both sexes. CONCLUSIONS: Bone density in adults with galactosemia is low, indicating the potential for increased fracture risk, the etiology of which appears to be multifactorial.

A recess intervention to promote moderate-to-vigorous physical activity.

BACKGROUND: Schools provide a prime environment for interventions that attempt to increase physical activity and prevent obesity. OBJECTIVE: This study examined the effect of a 30-min, structured recess using 22 games of known energy expenditure on moderate-to-vigorous physical activity when compared to free play implemented with third graders from two elementary schools over 9 weeks. METHODS: Moderate-to-vigorous physical activity and other cardiovascular risk factors were assessed pre- and post-intervention in 27 children. RESULTS: Moderate-to-vigorous physical activity during recess increased significantly in intervention school children from 6.9 ± 0.8 to 14.9 ± 0.9 min pre- and post-intervention, respectively (adjusted mean change 8.0 ± 1.1; P < 0.0001), with no differences by gender or body mass index (BMI). In-school, moderate-to-vigorous physical activity also increased significantly more for intervention compared to control children (adjusted mean change 14 ± 4 min vs. 3 ± 3 min; P = 0.014, respectively). CONCLUSION: There was no significant difference in BMI and cardiovascular risk factors. A structured recess is feasible to implement and can significantly increase moderate-to-vigorous physical activity.

Attenuation of resting energy expenditure following hematopoietic SCT in children.

Children undergoing hematopoietic SCT (HSCT) typically receive parenteral nutrition (PN) due to gastrointestinal toxicities. Accurate determination of resting energy expenditure (REE) may facilitate optimal energy provision and help avoid unintended overfeeding or underfeeding. A multicenter, prospective cohort study of children undergoing allogeneic HSCT was performed, in which REE was measured by indirect calorimetry at baseline and twice weekly until 30 days after transplantation. Change in percent predicted REE over time from admission was analyzed using repeated measures regression analysis. In all, 26 children (14 females) with a mean (s.d.) age of 14.9 (4.2) years who underwent an HLA-matched sibling or unrelated donor transplantation were enrolled. Mean (s.d.) percent predicted REE at baseline was 92.4 (15.2). Baseline REE was highly correlated with lean body mass measured by dual energy X-ray absorptiometry (r=0.78, P<0.0001). REE decreased significantly over time, following a quadratic curve to a nadir of 79% predicted at 14 days post transplantation (P<0.001) and returned to near baseline by day 30. Children undergoing HSCT exhibit a significant reduction in REE in the early weeks after transplantation, a phenomenon that places them at risk for overfeeding. Serial measurements of REE or reductions in energy intake should be considered when PN is the primary mode of nutrition.

Callosal dysgenesis in fetuses with ventriculomegaly: levels of agreement between imaging modalities and postnatal outcome.

OBJECTIVE: To assess neurodevelopmental outcome of fetuses diagnosed with callosal abnormalities after referral for ventriculomegaly. METHODS: This sub-analysis of a prospective study of 430 fetuses, which were referred for ventriculomegaly and underwent sonography and magnetic resonance imaging (MRI), included those fetuses with a diagnosis of corpus callosal abnormalities after recruitment into the main study. Between three and six radiologists independently reviewed ultrasound and MR images and recorded central nervous system (CNS) abnormalities, with final diagnoses being decided by consensus. Postnatal outcomes of fetuses with callosal abnormalities were compared between those with and those without other abnormalities. RESULTS: Callosal abnormalities were detected in 13% (58/430) of the fetuses referred with ventriculomegaly. Callosal dysgenesis was isolated in 24% (14/58) of these cases, with the remainder complicated by CNS, karyotypic or other major abnormalities. Five fetuses diagnosed prenatally as having isolated callosal abnormalities had additional CNS findings on postnatal assessment. Preconference kappa for callosal abnormalities was 0.76 for ultrasound and 0.78 for MRI, indicating that these investigations had a similar level of operator dependence. Neurodevelopmental outcome was normal or showed only mild delay that resolved in 67% (8/12) children with isolated callosal abnormalities compared to 7% (2/27) in those with non-isolated callosal abnormalities (P = 0.003). CONCLUSION: Callosal abnormalities are present in a significant proportion of fetuses with a diagnosis of ventriculomegaly. Isolated callosal abnormalities are associated with normal neurodevelopmental outcome in approximately two-thirds of fetuses.

A role for collagen XXIII in cancer cell adhesion, anchorage-independence and metastasis.

Collagen XXIII is a transmembrane collagen previously shown to be upregulated in metastatic prostate cancer that has been used as a tissue and fluid biomarker for non-small cell lung cancer and prostate cancer. To determine whether collagen XXIII facilitates cancer cell metastasis in vivo and to establish a function for collagen XXIII in cancer progression, collagen XXIII knockdown cells were examined for alterations in in vivo metastasis as well as in vitro cell adhesion. In experimental and spontaneous xenograft models of metastasis, H460 cells expressing collagen XXIII shRNA formed fewer lung metastases than control cells. Loss of collagen XXIII in H460 cells also impaired cell adhesion, anchorage-independent growth and cell seeding to the lung, but did not affect cell proliferation. Corroborating a role for collagen XXIII in cell adhesion, overexpression of collagen XXIII in H1299 cells, which do not express endogenous collagen XXIII, enhanced cell adhesion. Consequent reduction in OB-cadherin, alpha-catenin, gamma-catenin, beta-catenin, vimentin and galectin-3 protein expression was also observed in response to loss of collagen XXIII. This study suggests a potential role for collagen XXIII in mediating metastasis by facilitating cell-cell and cell-matrix adhesion as well as anchorage-independent cell growth.

Frequency and cause of disagreements in imaging diagnosis in children with ventriculomegaly diagnosed prenatally.

OBJECTIVES: To assess the frequency and cause of variability in diagnosis on cranial sonography and magnetic resonance imaging (MRI) in children referred following prenatal diagnosis of ventriculomegaly. METHODS: Between 19 September 2003 and 16 March 2007, 119 infants with ultrasound and/or MRI studies performed within 13 months (median, 6 days) after birth, following prenatal referral for ventriculomegaly, were studied prospectively. There were 97 infants with ultrasound results and 53 with MRI, including 31 with both. Three sonologists and three pediatric neuroradiologists interpreted the postnatal ultrasound and MRI findings, blinded to prenatal diagnosis, and a final consensus diagnosis or group of diagnoses was obtained. Ventricular sizes as well as types of and reasons for any disagreement in diagnosis were recorded. Disagreements on a per patient basis were categorized as being major when they crossed diagnostic categories and had the potential to change patient counseling. Postnatal and prenatal diagnoses were compared. RESULTS: There was prospective agreement on 42/97 (43%) ultrasound and on 9/53 (17%) MRI readings. Prospective consensus was more likely when the number of central nervous system (CNS) anomalies was lower (P < 0.001 and P = 0.002 for ultrasound and MRI, respectively). In 24/55 (44%) ultrasound and 11/44 (25%) MRI examinations with disagreement in diagnosis, there was disagreement concerning the presence of ventriculomegaly. In 22/97 (23%) ultrasound studies and 22/53 (42%) MRI studies the disagreements were potentially important. Reasons for discrepancies in the reporting of major findings included errors of observation as well as modality differences in depiction of abnormalities. In comparing prenatal with postnatal diagnoses, there were 11/97 (11%) ultrasound and 27/53 (51%) MRI examinations with newly detected major findings, the most common being migrational abnormalities, callosal dysgenesis/destruction and interval development of hemorrhage. CONCLUSION: Variability in postnatal CNS diagnosis is common after a prenatal diagnosis of ventriculomegaly. This is due in part to a lack of standardization in the definition of postnatal ventriculomegaly.

Neurodevelopmental outcome of fetuses referred for ventriculomegaly.

OBJECTIVE: To characterize the delivery and postnatal neurodevelopmental outcomes of fetuses referred for ventriculomegaly (VM). METHODS: Under an internal review board-approved protocol, pregnant women were referred for magnetic resonance imaging (MRI) after sonographic diagnosis of VM and classified into one of four diagnostic groups: Group 1, normal central nervous system (CNS); Group 2, isolated mild VM (10-12 mm); Group 3, isolated VM > 12 mm; and Group 4, other CNS findings. Pregnancy outcome was obtained. Follow-up visits were offered with assessment of neurodevelopmental, adaptive and neurological functioning at 6 months and 1 year and/or 2 years of age. Atrial diameter and VM group differences in developmental outcomes were evaluated using repeated measures logistic regression and Fishers exact test, respectively. RESULTS: Of 314 fetuses, 253 (81%) were liveborn and survived the neonatal period. Fetuses in Groups 4 and 3 were less likely to progress to live delivery and to survive the neonatal period (60% and 84%, respectively) than were those in Groups 2 or 1 (93% and 100%, respectively, P < 0.001). Of the 143 fetuses followed postnatally, between 41% and 61% had a Bayley Scales of Infant Development (BSID-II) psychomotor developmental index score in the delayed range (< 85) at the follow-up visits, whereas the BSID-II mental developmental index and Vineland Adaptive Behavior composite scores were generally in line with normative expectations. Among those that were liveborn, neither VM group nor prenatal atrial diameter was related to postnatal developmental outcome. CONCLUSIONS: Diagnostic category and degree of fetal VM based on ultrasound and MRI measurements are associated with the incidence of live births and thus abnormal outcome. Among those undergoing formal postnatal testing, VM grade is not associated with postnatal developmental outcome, but motor functioning is more delayed than is cognitive or adaptive functioning.

Low prevalence of vitamin D deficiency among adolescents with anorexia nervosa.

UNLABELLED: Fifty adolescents with AN and 200 healthy girls underwent vitamin D screening. Girls with AN reported exceptional compliance with vitamin D supplementation and PTH concentrations were lower. Vitamin D deficiency was less common in the group with AN, but when race was considered, the trend was no longer significant. INTRODUCTION: The objective of this study was to determine whether patients with anorexia nervosa (AN) are more compliant with supplementation and have a lower prevalence of vitamin D deficiency than healthy controls. METHODS: Fifty adolescents with AN and 200 controls were compared using anthropometric and lifestyle data, serum 25-hydroxyvitamin D (25OHD) and parathyroid hormone (PTH) concentrations, and prevalence of vitamin D deficiency. RESULTS: The prevalence of deficiency (<20 ng/mL) was 2% in the AN group vs. 24% among controls (p = 0.003). 25OHD was similar among white participants with AN and white controls (39.5 vs. 36.0 ng/mL, p = 0.20), but higher than in non-white controls (20.6 ng/mL). Significantly more girls with AN reported vitamin D supplementation (86%) than the full control (14%) or white subgroup (27%) (p < 0.001). Participants with AN had lower PTH concentrations than controls, (27.8 vs. 47.4 pg/mL, p = 0.009), a trend that lost significance after age and race adjustment (41.7 pg/mL, p = 0.12). CONCLUSIONS: Compared to healthy controls, adolescents with AN had a lower prevalence of vitamin D deficiency and PTH concentration. However, 25OHD and PTH concentrations were similar after adjustment for race and age. The trend of lower PTH levels in adolescents with AN, accompanied by exceptional compliance with supplementation, may have bone health implications for these patients.

Bone cross-sectional geometry in adolescents and young women with anorexia nervosa: a hip structural analysis study.

INTRODUCTION: Better characterization of bone geometry in adolescents with anorexia nervosa (AN) may improve understanding of skeletal deficits in this population. Our objective was to determine whether hip cross-sectional geometry and bone strength were altered in adolescents with AN. METHODS: Measurements of the left total proximal femur and body composition were obtained in 85 adolescents with AN and 61 healthy controls by dual X-ray absorptiometry. The Hip Structural Analysis (HSA) program was used to determine aBMD, cross-sectional area (CSA), and section modulus (Z) at the femoral neck and shaft. Strength indices were calculated and corrected for lean mass. RESULTS: Femoral neck and shaft aBMD were lower in AN patients than healthy controls (-36% and -29%, p < 0.001). In both regions, bone CSA and Z were lower in AN sufferers (-11 to -35%, p < 0.001). While lean body mass correlated with HSA variables (r = 0.48 to 0.58, p < 0.001), body fat did not. AN sufferers had lower indices of both whole bone strength (-40%, p < 0.001) and relative bone strength (-36%, p < 0.001) than controls. CONCLUSIONS: Anorexia nervosa sufferers had decreased resistance to axial (CSA) and bending loads (Z) compared with healthy controls. Differences in strength properties were significant even when adjusted for lean mass, suggesting that not only decreased mechanical loading, but also known metabolic differences are likely responsible for deficits in bone strength in these patients.

Clinical evaluation of a portable lactate meter in type I glycogen storage disease.

High lactate concentrations occur in type I glycogen storage disease (GSD) whenever glycogenolysis occurs. Not only does hyperlactataemia cause acute clinical deterioration, but chronic lactate elevations have also been associated with many of the long-term complications in GSD. A portable finger-stick blood lactate meter has recently been marketed as a training tool for high-performance athletes, but it has not been tested as a clinical diagnostic tool. This study was performed to assess the accuracy of the portable lactate meter in subjects with GSD I who are predisposed to high lactate concentrations. A total of 166 intravenous and 39 capillary samples from 13 subjects were tested concomitantly on three different lactate meters. The meter readings were compared with the lactate concentration determined by the laboratory gold-standard enzymatic colorimetric assay. Almost no inter-meter variability was found. The lactate meter values had outstanding correlation with the laboratory lactate determination, although the meters were found to run 0.5 mmol/L higher than the laboratory assay. The meter deviation was independent of lactate concentration. More variability was noted with finger-stick capillary lactate determinations, but monitoring of trends with capillary samples should prove valuable as a method for determining long-term control or acute deterioration. The portable lactate meter is a highly accurate tool for monitoring lactate concentrations, and should prove valuable for monitoring metabolic control in patients with GSD type I and other disorders associated with hyperlactataemia.

Possible effect of DRGs on the classification of stroke: implications for epidemiological surveillance.

BACKGROUND AND PURPOSE: Accurate data on the distribution of stroke subtypes are essential for understanding the forces driving recent morbidity and mortality trends. The introduction of diagnosis-related groups (DRGs) in the 1980s may have affected the distribution of stroke subtypes as defined by International Classification of Diseases, Ninth Revision (ICD-9), discharge diagnosis codes. METHODS: The Pawtucket Heart Health Program cardiovascular surveillance data were used to examine trends in stroke classification for 1980 to 1991 in relation to the introduction of DRGs in 2 communities in Massachusetts and Rhode Island, where DRGs were implemented 2 years apart. Included were all hospital discharges for residents aged 35 to 74 with a primary ICD-9 diagnosis of 431 to 432, 434, or 436 to 437 (N=1386 in Rhode Island, N=1839 in Massachusetts). RESULTS: In each state, concurrently with the introduction of DRGs, the proportion of strokes classified as cerebral occlusion (ICD-9 434.0 to 434.9) increased, and the proportion classified as acute but ill-defined (ICD-9 436.0 to 436.9) decreased. Before DRGs, 30.0% of strokes in Rhode Island and 26.6% in Massachusetts were classified as cerebral occlusion, whereas 51.8% in Rhode Island and 51.7% in Massachusetts were classified as acute ill defined. After DRGs were instituted, the proportions of cerebral occlusion and acute, ill-defined stroke, respectively, were 70.9% and 8.5% in Rhode Island and 74.1% and 7.7% in Massachusetts (chi(2), all P<0.001). The proportions of strokes classified as intracerebral hemorrhage or transient cerebral ischemia remained constant. CONCLUSIONS: The implementation of DRGs may have influenced coding of strokes to the ICD-9. Findings highlight the limitations of hospital discharge data for evaluating stroke subtypes and demonstrate the need for community-based surveillance for monitoring specific trends in stroke.