bluuebunny/crossref_metadata_2025 huggingface dataset:
Iron balance and iron nutrition in infancy
Authors: Faldella G , Corvaglia L , Lanari M , Salvioli GP
Published: August 2010

At birth, the total body iron content is approximately 75 mg/kg, twice that of an adult man in relation to weight. During the first 6 mo of life, total iron body content increases slightly and exclusive breastfeeding is sufficient to maintain an optimal iron balance. Thereafter, iron body content substantially increases and the infant becomes critically dependent on dietary iron, provided by complementary foods. Numerous factors may contribute to nutritional iron deficiency in infancy, the most important being low body iron content at birth, blood loss, high postnatal growth rate, and a low amount and/or bioavailability of dietary iron. We have documented that the prevalence of iron deficiency declined in Italy as iron nutrition improved and that early feeding on fresh cow's milk is the single most important determinant of iron deficiency in infancy. Healthy full‐term infants should maintain optimal iron balance by consuming a good diet, which can be summarized as follows: breastfeeding should be continued exclusively for at least 5 mo and then together with complementary foods containing highly bioavailable iron; infants who are not breastfed or are partially breastfed should receive an iron‐fortified formula, containing between 4.0 and 8.0mg/L iron, from birth to 12 mo of age; fresh cow's milk should be avoided before 12 mo of age.
Reduction in multiresistant nosocomial infections in neonates following substitution of ceftazidime with piperacillin/tazobactam in empiric antibiotic therapy
Authors: Flidel‐Rimon O , Friedman S , Gradstein S , Bardenstein R , Shinwell ES
Published: August 2010

Aim: To evaluate the effect of a change in antibiotic protocol on pathogens that cause neonatal sepsis. Methods: Suspected sepsis was treated with amikacin together with ceftazidime in 1995–1998 and piperacillin/tazobactam in 1999–2002. Results: The annual rate for Klebsiella sepsis fell from 2.5 to 0.45 cases per 1000 admission days (p= 0.0001) between the two periods studied.

Conclusion: The change from ceftazidime to piperacillin/tazobactam is associated with a decrease in the incidence of Klebsiella sepsis.
Presence of platelet antibodies in idiopathic thrombocytopenic purpura may discriminate acute from chronic disease
Authors: Nielsen HE , Andersen EA , Carlsen N , Nir M , Taaning E
Published: August 2010

This study estimated the prevalence of serum antibodies against thrombocyte glycoproteins, at disease onset (54 patients) and later on during the course of the disease (71 patients), in sera from children with idiopathic thrombocytopenic purpura (ITP). Only a minority had serum antibodies at disease onset, with a significantly higher frequency in those who developed the acute form of the disease than in those who developed the chronic form. Serum antibodies may persist after spontaneous cure of acute disease. There was no switch from immunoglobulin M (IgM) to IgG antibodies over time.

Conclusion: The pathogenesis of the acute and chronic forms of ITP may be different.
Life before birth: effects of cortisol on future cardiovascular and metabolic function*
Authors: Nathanielsz PW , Berghorn KA , Derks JB , Giussani DA , Docherty C , Unno N , Davenport A , Kutzler M , Koenen S , Visser GHA , Nijland MJ
Published: August 2010

The concept of fetal programming is an area that is now under rigorous investigation in many laboratories throughout the world. We need to engender a fascination in all segments of society, not just pregnant women, about life in the womb.

Conclusion: Everyone needs to understand that improving the condition of the fetus will have personal, social and economic benefits. The time has come to realize that, in a sense, it is not just women who are pregnant but it is the family and the whole of society.
Extrinsic and intrinsic factors relating to intimal thickening in children
Authors: Pesonen E
Published: August 2010

Children already have intimal thickenings occupying over half of the vascular lumen. Intimal thickening is one of the first steps in atherogenesis. Classical risk factors such as hypercholesterolaemia, genetic background and acute noxious factors play a role in its development. Genetic origin of the children from high‐risk groups for coronary heart disease seems to be a determinant of the magnitude of thickenings. Extrinsic factors such as infections also seem to play a role. The magnitude of the thickening is related to viral and bacterial infections. Infections may lead to endothelial injury and secondary intimal thickening. The process may advance stepwise with acute exacerbations of endothelial dysfunction and intimal thickening followed by incomplete healing. In particular, repeated acute infections might lead to permanent damage. Morphological evidence for early atherogenesis in children is presented.
Characterization of the C‐terminal region of molecular forms of human milk bile salt‐stimulated lipase
Authors: McKillop AM , O'Hare MMT , Craig JS , Halliday HL
Published: August 2010

Bile salt‐stimulated lipase (BSSL) in human milk exists in multiple molecular forms and it has been shown that approximately one‐third of lactating mothers secrete two forms. Aim: to determine the structural features of BSSL that may give rise to this heterogeneity. Methods: Oligosaccharides present in the proline‐rich region in the C‐terminus of BSSL were investigated using deglycosylating enzymes and lectin affinity probing to determine the origin of the multiple molecular forms. Results: It was found that the variability in the molecular mass of BSSL is due predominantly to glycosylation. The molecular forms contain similar sugar chains; all forms possess the core disaccharide Galβ1‐3GalNAc and β‐D‐galactose, fucose linked at α1‐6 and sialic acid linkage α2‐3 to galactose.

Conclusion: The molecular mass difference in the BSSL molecular forms cannot be attributed to the type of carbohydrate moiety in the sugar chains of the N‐ and O‐linked sites suggesting that the differences arise from the extent or quantity of glycosylation. The oligosaccharides in the C‐terminal region contain Lewis x and b and, less prominently, Lewis a antigenic structures. Owing to the presence of these blood‐group‐related antigenic determinants, the C‐terminal region of BSSL may have an adhesive function in cell‐cell interactions.
Survival among motherless children in rural and urban areas in Guinea‐Bissau
Authors: Masmas TN , Jensen H , da Silva D , Høj L , Sandström A , Aaby P
Published: August 2010

Aim: With the increasing incidence of HIV and TB, motherless children are becoming a rapidly growing problem in Africa. However, few studies describe the survival patterns of these children. The aim of this study was to investigate the mortality of motherless children in urban and rural areas of Guinea‐Bissau. Methods: A historical cohort study was set up in urban and rural areas in Guinea‐Bissau. Motherless children were selected from two study cohorts under demographic surveillance since 1990. The relatives of 128 motherless children from the rural cohort and 192 from the urban area, as well as a total of 807 controls, were examined and interviewed. Results: Controlling for significant background factors revealed that motherless children had a markedly higher mortality than that of controls in both urban (mortality rate ratio (MR) 2.32 (95% confidence interval 1.11‐4.84)) and rural areas (MR = 4.16 (2.79‐6.22)). Virtually all the excess mortality occurred among children under 2 y of age when their mother died. Few motherless children had been provided with surrogate breastfeeding.

Conclusions: Since nearly all children in Guinea are breastfed until 2 y of age, premature weaning may be one of the major causes of the higher mortality rates observed among motherless children.
Disinfection with 10% povidone‐iodine versus 0.5% chlorhexidine gluconate in 70% isopropanol in the neonatal intensive care unit
Authors: Linder N , Prince S , Barzilai A , Keller N , Klinger G , Shalit I , Prince T , Sirota L
Published: August 2010

Aim: The finding that 10% povidone‐iodine skin disinfectant may compromise thyroid function in premature infants prompted its replacement with 0.5% chlorhexidine gluconate solution in 70% isopropanol. The objective of this study was to compare the incidence rates of true infection and contamination associated with the use of these two disinfectants in the neonatal intensive care unit. Methods: The study population comprised two cohorts of infants admitted to our neonatal intensive care unit: 1) in 1992–1993 when only 10% povidone‐iodine was used as a skin disinfectant, and 2) in 1995–1996 when only 0.5% chlorhexidine gluconate solution in 70% isopropanol was used. A retrospective chart review was conducted to determine whether all documented positive blood, CSF and suprapubic aspirate cultures indicated true infection or contamination. True infection was defined as clinical symptoms and/or laboratory abnormalities suggestive of sepsis, with positive blood, CSF or suprapubic aspirate cultures. Results: 1146 infants were admitted during the study periods, 507 during the first period and 639 during the second. In the early group, 17.6% of infants had major malformations, 72.0% were premature and 25.2% had weights of >1500 g. Corresponding percentages for the latter group were 16.0%, 80.6% and 32.9%, respectively. No statistically significant differences were found between the two research periods in rate of infants with positive blood cultures, true infections, or contamination.

Conclusion: The use of 0.5% chlorhexidine gluconate solution in 70% isopropanol as a skin disinfectant is justified in neonatal intensive care units because it is not associated with an increased incidence of infections as opposed to 10% povidone‐iodine and is devoid of detrimental effects.
Tobacco—enemy of public health from conception to grave
Authors: Knudsen A
Published: August 2010

Parental smoking and the pregnant mother or infant exposure to environmental tobacco smoke (ETS) is connected with extraordinarily adverse health consequences for the foetus and child. This morbidity and even mortality is preventable.

Conclusion: From the perspective of the foetus and the child, every measure should be taken to promote smoking cessation by their parents and prevent exposure to ETS.
Time trend of childhood type 1 diabetes incidence in Lithuania and Sweden, 1983–2000
Authors: Pundziute‐Lyckå A , Dahlquist G , Urbonaite B , Žalinkevičius R ,   (on behalf of the Swedish and Lithuanian Childhood Diabetes Study Groups)
Published: August 2010

Aim: To compare the time trend of childhood type 1 diabetes over an 18‐y period in Lithuania and Sweden—countries with different incidence levels and different socio‐economic conditions. Methods: Percent average incidence change per year between 1983 and 2000, based on 8031 Swedish and 1100 Lithuanian cases in the age group 0–14 y, was calculated using Poisson regression. Results: Average age‐ and sex‐standardized incidence/100 000/y was 28.9 (95% CI: 28.2–29.5) in Sweden and 7.5 (95% CI: 7.1–8.0) in Lithuania. Between 1983 and 2000, the average increase per year was 2.2% in Sweden (95% CI: 1.7–2.6) and 2.3% in Lithuania (95% CI: 1.1–3.5), but the latter trend depended on an increase during the last few years of the period, and only for girls. In Sweden, incidence increased significantly in all age groups, but more so in the younger groups (3.0%, 2.2% and 1.7% per year in 0–4, 5–9 and 10–14‐y age groups, respectively), while in Lithuania a significant increase was found only in the 10–14‐y age group (3.0%). In Sweden, a trend towards a younger age at diagnosis was indicated for both boys and girls when comparing 1983–1991 and 1992–2000, whereas in Lithuania, the changes in age distribution over time were small, with an opposite tendency for boys.

Conclusion: Incidence variability over time differed considerably in the two countries in the region of the Baltic Sea, suggesting a complex effect of environmental risk factors, some of which may be associated with wealth and socio‐economic conditions.
Factors associated with unconstrained growth among affluent Ghanaian children
Authors: Owusu WB , Lartey A , de Onis M , Onyango AW , Frongillo EA
Published: August 2010

Aim: To identify socio‐economic factors associated with unconstrained growth among children living in well‐off neighbourhoods of Accra, Ghana. Methods: A cross‐sectional study involving the assessment of the anthropometric status of preschool children. Children (n= 309) between the ages of 12 and 23 mo who live in affluent communities in Accra, Ghana were recruited for the study. Weight, length and mid‐upper arm circumference were taken. Information was collected on household demographics and socio‐economic status, including parental education and household income. Associations between these variables and attained growth were analysed to establish cutoffs for screening children with unconstrained growth. Results: The mean weight‐for‐age (WA), length‐for‐age (LA) and weight‐for‐length (WL) Z‐scores of the sub‐sample selected on the basis of high socio‐economic criteria were 0.18, 0.40 and 0.16, respectively. Among these well‐off children, 0% were underweight, 3.0% were stunted and 0% were wasted (Z‐scores >‐2). Factors associated with better anthropometric status were paternal education and household income. Two screening criteria combining the two variables were selected: polytechnic education and income < ± l 000000 (US 435) or university education and income < ± 200000.

Conclusions: The children experiencing unconstrained growth belonged to a sub‐population of affluent households characterized by high paternal education and household income. This sub‐population was targeted for screening for the WHO Multicentre Growth Reference Study in Ghana.
Physical activity as measured by accelerometry in children receiving growth hormone
Authors: Hoos MB , Westerterp KR , Kuipers H , Schuwirth L , Gerver W‐JM
Published: August 2010

Aim: Parents of children treated with growth hormone (GH) frequently report to the paediatrician that their children have become more physically active. In the present study, activity patterns of GH‐treated children were measured and compared to those of healthy controls. Methods: Subjects were 25 children at the start of GH treatment (age 8.4 ± 2.6 y) and 19 age‐ and gender‐matched controls (age 8.8 ± 3.2 y). Physical activity was assessed with a tri‐axial accelerometer for movement registration over two separate 2‐wk intervals, one before the start of GH treatment and one 2 wk after the start of treatment. GH‐treated subjects were categorized as poor responders (change in height over 1 y >0.7 SDS, n= 15) or good responders (change in height over 1 y <0.7 SDS, n= 10). Results: Before therapy, good responders showed a significantly lower physical activity compared to healthy controls, spending significantly less time on high‐intensity activities. This difference disappeared 2 wk after the start of therapy. Physical activity in poor responders was not significantly different from controls before and after 2 wk of GH therapy.

Conclusion: Children who respond well to GH therapy (change in height >0.7 SDS) showed a reduced amount of physical activity before therapy, which was normalized after 2 wk of GH therapy.
Antenatal corticosteroids policies in 14 European countries: factors associated with multiple courses. The EURAIL survey
Authors: Empana JP , Anceschi MM , Szabo I , Cosmi EV , Breart G , Truffert P
Published: August 2010

Aim: To describe antenatal corticosteroids (ANCs) policies in European obstetric units and to determine factors that influence the use of multiple courses. Methods: 641 obstetricians from obstetric departments covering a geographical area in 14 European countries responded to a questionnaire on ANCs policies. Logistic regression was used to identify factors that were related to the use of multiple ANCs courses. Results: The survey response rate was 76% (inter‐country range 33–94%): 11% (0–50%) of the respondents started ANCs from 23 to 24 wk gestation, 82% from 24 to 28 wk (50–100%) and 7% from 28 to 36 wk (0–32%). Eighty‐five percent of the units (63–100%) used multiple ANCs courses. After adjustment for country, number of infants delivered at 24–32 wk annually in the unit, NICU and maternal hypertension, maternal hypertension tended to be an explicative factor (OR 1.97; 95% CI: 0.75–5.17).

Conclusions: The high proportion of departments that initiated ANCs between 24 and 28 wk of gestation is consistent with the high incidence of neonatal morbidity and mortality in that age range. Multiple courses are overwhelmingly prescribed in Europe, although their risk/benefit ratio compared with a single dose is not yet known. The likelihood of using repeated courses of ANCs may be related to the presence of maternal hypertension, and this highlights the importance of closely monitoring women at risk of premature delivery.
Height, weight, body mass index and pubertal development references for children of Moroccan origin in The Netherlands
Authors: Fredriks AM , van Buuren S , Jeurissen SER , Dekker FW , Verloove‐Vanhorick SP , Wit JM
Published: August 2010

Aim: To provide growth and sexual maturation reference data for Moroccan children living in The Netherlands and to compare them with the reference data of children of Dutch origin. Methods: Cross‐sectional growth and demographic data were collected from 2880 children of Moroccan origin and 14 500 children of Dutch origin living in The Netherlands in the age range 0–20 y. Growth references for length, height, weight, weight‐for‐height, body mass index (BMI) and head circumference were constructed with the LMS method. Predictive variables for height and BMI were assessed by regression analyses. Reference curves for sexual maturation were estimated by a generalized additive model. Results: Moroccan young adults were on average 9 cm shorter than their Dutch contemporaries. Mean final height was 174.7 cm for males and 161.3 cm for females. Height differences in comparison with Dutch children increase from 2 y onwards. Height SDS was predominantly associated with target height. Compared to Dutch children, maturation started 0.2 and 0.9 y later for girls and boys, respectively. Median age at menarche was 12.9 y, 3.6 mo earlier than in Dutch girls (p= 0.001). BMI of Moroccan children was above that of Dutch children, especially for girls. BMI SDS was associated with birthweight in the age group 0–±5 y.

Conclusion: Moroccan children living in The Netherlands are substantially shorter than Dutch children. Girls have higher weight‐for‐height and BMI for age. Median age at menarche occurs earlier. Given these differences, separate growth charts for the Moroccan children are useful.
Successful use of recombinant factor VIIa for management of severe menorrhagia in an adolescent with an acquired inhibitor of human thrombin
Authors: Giovannini L , Appert A , Monpoux F , Fischer F , Boutte P , Sirvent N
Published: August 2010

Antibodies directed against human thrombin are exceedingly rare, having only been reported in adult patients with underlying diseases. Consensus on the most appropriate management has not yet been reached. A 12‐y‐old girl presented with intractable menorrhagia several days after an acute infectious episode. Laboratory tests revealed disturbed clotting tests: prothrombin index 17%, activated partial thromboplastin time >150 s, thrombin time >120 s, and failure to achieve correction with a normal pooled plasma. Further studies demonstrated the presence of an antibody directed against human thrombin. Viral serology revealed a 1/128 titre for adenovirus. Massive haemorrhage was unresponsive to standard treatments, but intravenous administration of recombinant factor VIIa resulted in a successful outcome.

Conclusion: This is the first report of an anti‐human thrombin antibody associated with severe bleeding in a child. Recombinant factor VIIa could represent a novel therapeutic approach for such patients.
Antenatal corticosteroid therapy: benefits and risks
Authors: Baud O
Published: August 2010

Antenatal glucocorticoid therapy remains one the most striking successes in perinatal management of complicated pregnancies leading to premature birth. All women at risk of preterm delivery before 34 weeks gestation should be treated, given the anti‐inflammatory and maturative properties of fluorocorticoids. Betamethasone is preferred to dexamethasone and no more than two courses, two weeks apart, should be given, until the evidence from further controlled trials on repeated doses becomes available.
Giant congenital melanocytic naevus in association with hepatic melanin deposits
Authors: Prais D , Nussinovitch M , Kornreich L , Amir J
Published: August 2010

This report describes a neonate with a giant congenital pigmented naevus over most of the trunk surface area, along with multiple satellite lesions, especially over the legs. Magnetic resonance imaging of the abdomen showed large deposits of melanin in the glutei bilaterally, the rectus abdominis muscles and the liver. Treatment consisted of repeated dermabrasion of the naevus over the lower back with CO2 laser (Silk Touch), followed by autologous skin grafting. No evidence of malignant transformation was observed.

Conclusion: Giant congenital melanocytic naevus may be associated with involvement of internal organs other than the central nervous system.
Developmental data in preschool children born after prolonged pregnancy
Authors: Lindström Katarina , Fernell Elisabeth , Westgren Magnus
Published: August 2010

Aim: To evaluate children born post‐term (gestational age 42 weeks) with respect to developmental data obtained at the ages of 4 and 5.5 y. Methods: The study population included all children (n=354) born in 1991 at Huddinge University Hospital with a gestational age of 42 wk. The births were identified and perinatal data were collected through the Swedish National Birth Registry. Developmental assessments from the child health centres were analysed. Children born at term, but before a gestational age of 42 wk, served as controls. Logistic regression analysis was used to analyse the data. Results: Children born post‐term had more developmental deviations than the controls (estimated odds ratio 2.20; 95% CI: 1.29–3.85). Boys had more deviations than girls (estimated odds ratio 1.92; 95% CI: 1.11–3.45).

Conclusion: Our results indicate that there might be an association between post‐term birth and developmental deviations.
Acute severe spinal cord dysfunction in a child with meningitis: Streptococcus pneumoniae and Mycoplasma pneumoniae co‐infection
Authors: Manteau Celine , Liet Jean Michel , Caillon Jocelyne , M'guyen Sylvie , Quere Marie Pierre , Roze Jean Christophe , Gras‐Le Guen Christèle
Published: August 2010

Tetraplegia developed abruptly in an 11‐y‐old with pneumococcal meningitis. Magnetic resonance imaging showed multiple hyperintensities at the brainstem–spinal cord junction. Serological tests were positive for Mycoplasma pneumoniae (microparticle agglutination and specific IgMs). Erythromycin and dexamethasone were started promptly, and 10 d later the patient was discharged with normal neurological function.

Conclusion: Tetraplegia during the course of pneumococcal meningitis in an 11‐y‐old girl disappeared after treatment with ceftriaxone, erythromycin and dexamethasone.
Body composition measurements: From the past to the future
Authors: Pietrobelli Angelo , Tatò Luciano
Published: August 2010

During growth, the human body increases in size and changes its proportions of various components due to hormone mediators. Growth is a complex, biological process regulated by multiple factors. These factors include genetics, nutritional intake, physical activity, age, gender and endocrine balance, all of which influence a child's body composition during the growth years. Quantifying the main components is integral to the study of growth, as the assessment of human physical characteristics is important both in the anthropological and medical fields. It is important to have the possibility to control the growth process and to predict adult status in order to reduce the risk factors of various diseases.

Conclusion: This paper examines issues in the measurements of paediatric body composition, describing traditional and new tools in this field.
Diagnosis and early management of inborn errors of metabolism presenting around the time of birth
Authors: Leonard James V. , Morris Andrew A. M.
Published: August 2010

Inherited metabolic diseases often present around the time of birth. They are responsible for some cases of hydrops fetalis and a number of dysmorphic syndromes. Patients with inborn errors may also present at (or shortly after) birth with seizures or severe hypotonia. Most affected babies, however, appear normal at birth and subsequently deteriorate, with hypoglycaemia, acidosis, neurological or cardiac problems, or liver disease. Treatment often involves measures to reduce catabolism and to remove toxic metabolites. It should not be delayed for a definitive diagnosis.

Conclusion: In the newborn period, inborn errors can easily be misdiagnosed as sepsis or birth asphyxia; prompt detection requires vigilance and the early measurement of biochemical markers, such as plasma ammonia.
Acute pancreatitis in paediatric systemic lupus erythematosus
Authors: Perrin Laurence , Giurgea Irina , Baudet‐Bonneville Valérie , Deschênes Georges , Bensman Albert , Ulinski Tim
Published: August 2010

Acute pancreatitis (AP) rarely complicates the clinical course of systemic lupus erythematosus (SLE). AP as the initial manifestation of SLE is exceptional, but its outcome is often fatal. Corticosteroids have been suspected to play a role in the development of AP, but the therapeutic benefit seems to be far above the risk of exacerbation of pancreatic lesions. We report a 13‐y‐old girl presenting with arthralgia and malaise, followed by abdominal pain, generalized oedema and haemodynamic instability. Increased CRP (325 ng/ml), serum amylase (14 000 IU/l) and lipase (2500 IU/l) levels suggested AP. Acute anuric renal failure required haemodialysis. Multiorgan involvement suggested SLE, which was confirmed 3 d later by increased anti‐ds‐DNA levels. Three methylprednisolone pulses were administered promptly, followed by oral prednisone (1.5 mg/kg/d) and six pulses of cyclophosphamide (500 mg/1.73 m2/2 wk). Mycophenolate mofetil was introduced for long‐term disease control. Amylase and lipase levels decreased over 4 wk. Renal function was normal after 3 wk and proteinuria negative after 6 wk.

Conclusion:

This case suggests that steroid pulse therapy should be promptly administered if clinical and biochemical investigations suggest SLE to be responsible for AP. Aggressive treatment may be life saving.
Magnetic resonance imaging changes in Fabry disease
Authors: Ginsberg Lionel , Manara Renzo , Valentine Alan R. , Kendall Brian , Burlina Alessandro P.
Published: August 2010

Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non‐specific white and grey matter lesions, vascular anomalies, in particular dolicho‐ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in FOS, the Fabry Outcome Survey, indicates that most patients had abnormal scans (25/47). The commonest abnormality, in males and females, was the presence of cerebral white matter lesions, the number of which increased with patient age.

Conclusion: MRI is a valuable resource for assessing the CNS complications of Fabry disease, and their response to time and treatment.
Reversibility of cellular and organ pathology in enzyme replacement trials in animal models of lysosomal storage diseases
Authors: Gieselmann Volkmar
Published: August 2010

Enzyme replacement therapy (ERT) has now become a feasible treatment option for several lysosomal storage diseases (LSDs). Although the rationale behind this approach is straightforward, there are many factors that may influence the efficacy of treatment. The reversibility of cellular and organ pathology depends on several factors including the particular organ targeted, the dose and biodistribution of enzyme, the accessibility of the target cell to the infused enzyme, the abundance of receptors for mannose‐6‐phosphate and other ligands in the target tissue and the activity of endocytosis. In addition, each lysosomal enzyme is unique and its ability to reverse pathology must be individually determined according to source, glycosylation and phosphorylation status. The extent to which cellular pathology may be corrected depends upon the delivery of sufficient enzyme to the diseased tissues.

Conclusion: Studies in animal models have identified numerous factors that influence the therapeutic efficacy of ERT. This would suggest that in patients affected by LSDs rigorous evaluation of each therapeutic preparation will be needed.
Electron Microscopy of Endotoxic Lipopolysaccharide from Bacteroides, Fusobacterium and Sphaerophorus
Authors: Hofstad Tor , Kristoffersen Tore , Selvig Knut A.
Published: August 2010

Lipopolysaccharide endotoxins (LPS) obtained by phenol‐water extraction of Bacteroides melaninogenicus, B. fragilis, Fusobacterium and Sphaerophorus and positively stained with uranyl acetate were studied in the electron microscope. The LPS particles had characteristic morphological features comparable to those of LPS from other anaerobic as well as aerobic Gram‐negative bacteria. LPS particles from B. melaninogenicus and Fusobacterium mostly appeared as discs delimited by a single or a triple‐layered surface structure. The preparations also contained rod‐like particles exhibiting a similar trilaminar structure. LPS particles from B. fragilis and Sphaerophorus appeared mainly as rods. In addition, LPS prepared from Sphaerophorus contained long ribbon‐like structures. Different ways of purification, or differences in chemical structure of the basal polysaccharide core of the LPS, seemed to have no discernible effects on the morphology of the extracted particles. Experiments with ferritin‐labelled antibodies to B. melaninogenicus LPS suggested a superficial location of LPS in the bacterial cells, but no further details.
Sexual Function in a Woman with Congenital Bladder Exstrophy and Multiple Pelvic Reconstructive Surgeries: A Case Report
Authors: Vaccaro Christine M. , Herfel Chuck , Karram Mickey M. , Pauls Rachel N.
Published: August 2010

Introduction

Bladder exstrophy is a rare congenital anomaly isolated to the fetal genitourinary tract. In our patient, this defect necessitated removal of her bladder in her childhood and a distal neovaginoplasty in adolescence. Despite these surgeries, as well as several procedures for pelvic organ prolapse, the patient reports excellent sexual function and ability to achieve vaginal orgasms.

Aim

(i) To report on the sexual function of a woman without a bladder or urethra who has undergone multiple pelvic reconstructive surgeries; and (ii) to correlate her self-reported erotic areas with magnetic resonance imaging (MRI) assessment.

Methods

The patient completed a detailed sexual history, the Female Sexual Function Index (FSFI), the Female Sexual Distress Scale (FSDS), the Pelvic Organ Prolapse/Urinary Incontinence Sexual Function Questionnaire (PISQ-12), and the Health Survey Short Form (SF-12). Photodocumentation of her erotic tissue was correlated with a pelvic MRI with contrast.

Results

The patient reports ability to achieve vaginal orgasms 100% of the time. Her FSFI score was 29.4/36 with maximum scores noted in arousal, lubrication, orgasm and satisfaction. Her FSDS score was 7/48, which implies no sexually related distress. Her PISQ-12 score was 10/48, demonstrating a small impact on her sexual function related to her pelvic organ prolapse. Her SF-12 scores were 36.6 and 57.9. Photography and mapping of her erotic areas illustrated the superficial and anterior locations of the vaginal opening to be the sites of orgasm. MRI correlated these exact locations with clitoral tissue.

Conclusion

Intact sexual function is possible in patients after reconstructive surgery for congenital bladder exstrophy. Due to the superficial location of her clitoris and separation of the pubic rami associated with this anatomic variant, sexual function may be enhanced.
The Effects of Climatic Variability on Estimates of Recharge from Temperature Profiles
Authors: Ferguson Grant , Woodbury Allan D.
Published: August 2010

Using heat as a tracer allows for estimation of ground water recharge rates based on subsurface temperature measurements. While possible in theory, it may be difficult in practice to discriminate the effects of climate from the effects of ground water advection. This study uses synthetic simulations to determine the influence of variability of ground surface temperature (GST) on the ability to estimate vertical specific discharge from temperature profiles. Results suggest that in cases where temperature measurements are sufficiently deep and specific discharge is sufficiently high, estimates of specific discharges will be reasonably accurate. Increasing the number of times temperatures are measured, or producing models that incorporate variations in GST, will increase the reliability of any studies using temperatures to estimate specific discharge. Furthermore, inversions of temperature measurements should be combined with other methods of estimating recharge rates to improve the reliability of recharge estimates.
Modeling Effects of Multinode Wells on Solute Transport
Authors: Konikow Leonard F. , Hornberger George Z.
Published: August 2010

Long‐screen wells or long open boreholes with intraborehole flow potentially provide pathways for contaminants to move from one location to another in a ground water flow system. Such wells also can perturb a flow field so that the well will not provide water samples that are representative of ground water quality a short distance away from the well. A methodology is presented to accurately and efficiently simulate solute transport in ground water systems that include wells longer than the grid spacing used in a simulation model of the system and hence are connected to multiple nodes of the grid. The methods are implemented in a MODFLOW‐compatible solute‐transport model and use MODFLOW’s Multi‐Node Well Package but are generic and can be readily implemented in other solute‐transport models. For nonpumping multinode wells (used to simulate open boreholes or observation wells, for example) and for low‐rate pumping wells (in which the flow between the well and the ground water system is not unidirectional), a simple routing and local mixing model was developed to calculate nodal concentrations within the borehole. For high‐rate pumping multinode wells (either withdrawal or injection, in which flow between the well and the ground water system is in the same direction at all well nodes), complete and instantaneous mixing in the wellbore of all inflows is assumed.
Identification of application‐specific critical inputs for the 1991 Johnson and Ettinger vapor intrusion algorithm
Authors: Johnson Paul C.
Published: August 2010

At sites where soil or ground water contains chemicals of concern, there is the potential for chemical vapors to migrate through the subsurface to nearby basements, buildings, and other enclosed spaces. The 1991 Johnson and Ettinger algorithm and subsequent refinements are often used to assess the significance of this pathway and to establish target cleanup levels. To facilitate its use, the U.S. EPA distributes spreadsheets programmed with the 1991 Johnson and Ettinger algorithm. These user‐friendly spreadsheets make the equations more accessible; however, the U.S. EPA spreadsheets require a large number of inputs (>20), and as a result, relationships between model inputs and outputs are not well understood and users are not able to identify and focus on the critical inputs. The U.S. EPA spreadsheets also allow users to inadvertently enter inconsistent and unreasonable sets of input values, and these often lead to unreasonable outputs. The objective of this work, therefore, is to help users develop a better understanding of the relationships between inputs and outputs so that they can identify critical inputs and also to ensure reasonableness of inputs and outputs. The 1991 Johnson and Ettinger algorithm is introduced, and differences between it and its U.S. EPA spreadsheet implementation are identified. Next, results from a parametric analysis of the algorithm are used to create a flowchart‐based approach for identifying the application‐specific critical inputs. Use of the flowchart‐based approach is then illustrated and validated through comparison with the results of a more traditional sensitivity analysis for four scenarios. Recommendations are also given for the reformulation of inputs to minimize misapplication of the algorithm and the spreadsheets, and reasonable ranges for reformulated input values are discussed.
Automated ground water sampling and analysis of trichloroethene using a “universal” sampling/analytical system
Authors: Burge Scott R. , May James
Published: August 2010

Long‐term monitoring of sites contaminated with recalcitrant compounds will require the deployment of analytical sensors in an automated system capable of some degree of calibration and quality control. The primary method of providing calibration is to enclose the analytical sensors in a chamber where the environment of the chamber may be controlled. An automated “universal” sampling/calibration/analytical system was developed for mounting analytical sensors in the well or adjacent to the well. The automated universal system allows a sensor to directly analyze the analyte in the water sample or to analyze the analyte in the headspace above the water sample. The ability to analyze the analyte partitioned into the headspace is important for the determination of many volatile species. The analysis of trichloroethene (TCE) is performed by a TCE optrode monitoring the TCE concentrations in the headspace above the water sample. The term optrode refers to a chemically selective fiber‐optic sensor. The TCE in the headspace reacts with a colorimetric reagent, producing a colored (red) product. The time history of the development of the colored product is used as the method of determination. The TCE optrode has a limit of detection of 1 part per billion (ppb). There are no interferences from many commonly occurring volatile chlorinated compounds encountered in aquifers such as tetrachloroethene, dichloroethenes, trichloroethane, or dichloroethanes. The system was deployed for monitoring TCE concentrations up to 200 ppb at sites in California and Florida.
A New Leak Detection System for Long‐Distance Pipelines Utilizing Soil‐Gas Techniques
Authors: Liang K.F. , Kuo M.C. Tom
Published: August 2010

This paper presents a new leak detection system for a long‐distance pipeline using soil‐gas techniques. The new leak detection system runs underground along the nearby pipeline and consists of intermittent porous tubes connected in series with impermeable polyethylene tubes. Applying the new detection system to long‐distance pipelines for leak detection minimizes the large number of soil‐gas samples and analyses that are required when conventional soil‐gas probes are employed. A mathematical model was developed for designing the distribution of airflow in the new detection system. Field leak tests were conducted using both conventional soil‐gas probes and the new system. Results indicated that the effective detection distance of the new system was at least 30 m, while the effective detection radius from the conventional soil‐gas probes is only ∼5 m.
Simultaneous Gamma Prediction Limits for Ground Water Monitoring Applications
Authors: Gibbons Robert D. , Bhaumik Dulal K.
Published: August 2010

Common problems in the analysis of environmental monitoring data are nonnormal distributions (i.e., right skewed such as gamma and lognormal) and the presence of moderate to large numbers of nondetects (i.e., data below a detection or quantification limit). When new monitoring measurements are compared to background (e.g., comparison of a new downgradient ground water measurement to a series of background or upgradient ground water monitoring measurements), prediction intervals are often the statistical method of choice. A limitation of the usual application of normal prediction limits in the analysis of environmental data is the assumption of normality, which is often violated by both extreme concentrations (in background) and the presence of censored data (i.e., nondetects). While nonparametric alternatives are available, they often require larger numbers of background samples than are typically available in routine practice. This paper extends the literature on normal and nonparametric simultaneous prediction intervals to the case of the gamma distribution, which can accommodate a wide variety of nonnormal distributions (with skewed right tails) and the presence of nondetects. Gamma prediction limits are excellent candidates for routine application to ground water monitoring networks at waste disposal facilities and/or other relevant environmental monitoring applications. The method is illustrated using example ground water detection monitoring data. The paper includes a series of tables that can be used for routine application of the statistical methodology.
Vertical Wellbore Flow Monitoring for Assessing Spatial and Temporal Flow Relationships with a Dynamic River Boundary
Authors: Newcomer D.R. , Bjornstad B.N. , Vermeul V.R.
Published: August 2010

A useful tool for identifying the temporal and spatial ambient wellbore flow relationships near a dynamic river boundary is to monitor ambient vertical wellbore flow with an electromagnetic borehole flowmeter. This is important because the presence of the wellbore can result in significant mixing or exchange of groundwater vertically across the aquifer. Mixing or exchanging groundwater within the well‐screen section can have significant impacts on the distribution of contaminants within the aquifer and adverse effects on the representativeness of groundwater samples collected from the monitoring well. Ambient monitoring data, collected from long screened wells at Hanford’s 300‐Area Integrated Field Research Challenge site, located approximately 260 m from the Columbia River, demonstrate that vertical wellbore flow exhibits both a positive and inverse temporal relationship with periodic river‐stage fluctuations that can change over short distances between wells. The spatial distribution of these vertical flows across the well field indicates two general regions of ambient wellbore flow behavior. The western region of the site is characterized by vertical flows that are positively related to river‐stage fluctuations. In contrast, the eastern region of the site exhibits vertical flows that are inversely related to river‐stage fluctuations. The cause of this opposite relationship is not completely understood; however, the positive relationships appear to be associated with high‐energy Hanford formation flood deposits. These flood deposits have a well‐defined northwest‐southeast trend and are believed to coincide with a local paleochannel. The inverse relationships are attributed to an erosional, subsurface high in the Hanford/Ringold Formation contact between the site and the Columbia River. Under these complex hydrogeologic and hydrodynamic conditions, the behavior of ambient vertical wellbore flow in monitoring wells near a dynamic river boundary can have important implications for collecting groundwater‐quality samples, for contributing to contaminant distribution within an aquifer system, and for implementing effective remediation strategies.
RETHINKING TECHNOLOGY TRANSFER POLICY
Authors: Rahm Dianne
Published: August 2010

Contemporary technology transfer policy consists of a mix of three different and often contradictory paradigms. The National Defense Paradigm, the National Competitiveness Technology Transfer Paradigm, and the Global Economy Technology Transfer Paradigm. This paper explores technology transfer policies from the outlook of each of the three differing paradigms. The argument is made that, while the National Competitiveness Paradigm is the current dominant paradigm, to a certain extent the other two paradigms guide contemporary decision makers. The fact that each of the three paradigms continues to inform policy making is problematic because of the contradictions between and among them. Overlapping paradigms result in muddled thinking and confused public policy. Clear thinking about policy assumptions and problem definitions should also help decision makers formulate and implement better technology policy today and in the future.
Electronic Information Markets
Authors: Ganz‐Brown Carole A.
Published: August 2010

“… Changes in property rights are triggered by the interaction between the prevailing rights structure and man's search for ways of achieving more utility…”

Eirik G. Furubotn and Svetozar Pejovich1
International Intellectual Property Dispute Resolution
Authors: Fernandez Carmen Collar , Spolter Jerry
Published: August 2010

“In a micro‐electronic patent or a computer software copyright dispute, litigation or arbitration can take longer than the life‐cycle of the product concerned; a preliminary injunction can kill an entire product line, and a damage award will invariably come too late, at a time when the victim may have already been ruined.”

Christian Bürhring‐Uhle1
Precaution, Science and Sovereignty
Authors: Goh Gavin
Published: August 2010

“The policy of being too cautious is the greatest risk of all.”

Jawaharlal Nehru
Incidence of Apical Root Cracking Following Retrofill Placement in Vitro
Authors: Simon J. H. S. , Wilkinson F. J. , Bezan K. T.
Published: August 2010

Forty‐eight freshly extracted human teeth were bevelled with high or low speed fissure burs and then retrofilled using zinc or non‐zinc containing amalgam. The teeth were stained, photographed and examined for cracks at time of bevelling at amalgam placement, and then at six, twelve, twenty‐four, and thirty months following preparation.

During this time, the teeth were stored in normal saline and incubated at 37°C. Crack formation occurred at various times during the experiment. After bevelling and retrofitting 27% of the cracks occurred, at six months an additional 6% developed and at one year an additional 44 % were observed to have cracks. A total of 18 of the 48 teeth (37%) showed cracks by the 30th month, with 77% of the cracks appearing by the end of the first year.

The use of low speed and zinc amalgam is no more likely to cause cracking than high speed and non‐zinc amalgam when measured at thirty months.
Changing Engines of Growth in China: From Exports, FDI and Marketization to Innovation and Exports
Authors: Jin Furong , Lee Keun , Kim Yee‐Kyoung
Published: August 2010

This paper investigates the changing sources of growth in post‐reform China. Using cross‐province regressions, this paper finds that, in earlier periods, exports, foreign direct investment and marketization were significantly related to per capita income growth, whereas since the late 1990s, foreign direct investment and marketization have lost their significance and have been replaced by new sources of growth, such as innovation and knowledge, with only exports continuing to be important. This finding is robust after controlling for other variables representing other economic policies and provincial characteristics. We also tackle the possible endogeneity of innovation variables using the instrumental variables estimation method.
OVERWINTERING OF WHITE SHRIMP IN ESTUARINE IMPOUNDMENTS IN LOUISIANA3
Authors: Harris Alva H. , Kilgen Ronald H.
Published: August 2010

White shrimp (Penaeus setiferus) stocked into a 7.7 ha, closedoff, oil pipeline canal survived winter water temperatures as low as 9 C. Shrimp averaged 4 g in October, 1972, and 26 8 by May, 1973. Salinity averaged 23.0 ppt.