bluuebunny/crossref_metadata_2025_split huggingface dataset:
Occurrence of giant focal forms of congenital hyperinsulinism with incorrect visualization by 18F DOPA‐PET/CT scanning
Authors: Kühnen Peter , Matthae Ritana , Arya Ved , Hauptmann Kathrin , Rothe Karin , Wächter Simon , Singer Michael , Mohnike Wolfgang , Eberhard Traugott , Raile Klemens , Lauffer Lina M. , Iakoubov Roman , Hussain Khalid , Blankenstein Oliver 
Published: April 2014

SummaryContextCongenital hyperinsulinism (CHI) is a rare disease characterized by severe hypoglycaemic episodes due to pathologically increased insulin secretion from the pancreatic beta cells. When untreated, CHI might result in irreversible brain damage and death. Currently, two major subtypes of CHI are known: a focal form, associated with local distribution of affected beta cells and a nonfocal form, affecting every single beta cell. The identification of focal forms is important, as the patients can be cured by limited surgery. 18F DOPA‐PET/CT is an established non‐invasive approach to differentiate focal from nonfocal CHI.ObjectiveThe purpose of this study was to identify possible limitations of 18F DOPA‐PET/CT scan in patients with focal forms nonfocal CHI.DesignA retrospective chart review of 32 patients (from 2008 through 2013) who underwent 18F DOPA‐PET/CT and partial pancreatectomy for focal CHI at the reference centres in Berlin, Germany and London, UK.ResultsIn most cases (n = 29, 90·7%), 18F DOPA‐PET/CT was sufficient to localize the complete focal lesion. However, in some patients (n = 3, 9·3%), 18F DOPA‐PET/CT wrongly visualized only a small portion of the focal lesion. In this group of patients, a so‐called ‘giant focus’ was detected in histopathological analysis during the surgery.ConclusionsOur data show that in most patients with focal CHI 18F DOPA‐PET/CT correctly predicts the size and anatomical localisation of the lesion. However, in those patients with a ‘giant focal’ lesion 18F DOPA‐PET/CT is unreliable for correct identification of ‘giant focus’ cases.
Current concepts in the diagnosis and management of type 1 gastric neuroendocrine neoplasms
Authors: Kaltsas Gregory , Grozinsky‐Glasberg Simona , Alexandraki Krystallenia I. , Thomas Dimitrios , Tsolakis Apostolos V. , Gross David , Grossman Ashley B. 
Published: April 2014

SummaryThe vast majority of gastrin‐related gastrointestinal neuroendocrine neoplasms (GI‐NENs) develop in the context of chronic atrophic gastritis (type 1), a condition closely related to autoimmune thyroid diseases. These neoplasms are defined as gastricNENs type 1 (GNEN1) and have recently been shown to constitute the commonestGI‐NENs in a prospective study. GNEN1s are usually multiple and follow a relative indolent course, raising questions regarding the extent that such patients should be investigated and the appropriate therapeutic interventions needed. Recently, a number of consensus statements and guidelines have been published from various societies dealing with the diagnosis and management ofGI‐NENs. Endocrinologists are among the many different medical specialties involved in GNEN1s diagnosis and management. However, despite recent advances, few randomized trials are available, and thus existing evidence remains relatively weak compared to other malignancies. The purpose of this review is to provide recent evidence along with currently employed modalities addressing the diagnosis, management, long‐term follow‐up and potential comorbidities ofGNEN1s.
Efficacy and safety of growth hormone treatment in adults with growth hormone deficiency: a systematic review of studies on morbidity
Authors: van Bunderen Christa C. , van Varsseveld Nadège C. , Erfurth Eva Marie , Ket Johannes C.F. , Drent Madeleine L. 
Published: April 2014

SummaryDue to the positive effects demonstrated in randomized clinical trials on cardiovascular surrogate markers and bone metabolism, a positive effect of growth hormone (GH) treatment on clinically relevant end‐points seems feasible. In this review, we discuss the long‐term efficacy and safety of GH treatment in adult patients with growth hormone deficiency (GHD) with emphasis on morbidity: fatal and nonfatal cardiovascular disease (CVD) and stroke, fractures, fatal and nonfatal malignancies and recurrences, and diabetes mellitus. A positive effect of GH treatment on CVD and fracture risk could be concluded, but study design limitations have to be considered. Stroke and secondary brain tumours remained more prevalent. However, other contributing factors have to be taken into account. Regrowth and recurrences of (peri)pituitary tumours were not increased in patients with GH treatment compared to similar patients without GH treatment. All fatal and nonfatal malignancies were not more prevalent in GH‐treated adults compared to the general population. However, follow‐up time is still relatively short. The studies on diabetes are difficult to interpret, and more evidence is awaited. In clinical practice, a more individualized assessment seems appropriate, taking into consideration the underlying diagnosis of GHD, other treatment regimens, metabolic profile and the additional beneficial effects of GH set against the possible risks. Large and thoroughly conducted observational studies are needed and seem the only feasible way to inform the ongoing debate on health care costs, drug safety and clinical outcomes.
Quality of total mesorectal excision and depth of circumferential resection margin in rectal cancer: a matched comparison of the first 20 robotic cases
Authors: Barnajian M. , Pettet D. , Kazi E. , Foppa C. , Bergamaschi R. 
Published: April 2014

AbstractAimThere are concerns about the impact of robotic proctectomy on the quality of total mesorectal excision (TME) and the impact of laparoscopic proctectomy on the depth of the circumferential resection margin (CRM). The aim of this study was to compare the first 20 consecutive robotic proctectomies performed in our unit with matched series of open and laparoscopic proctocolectomy performed by the same surgeon.MethodData on the first 20 consecutive patients treated with robotic proctectomy for rectal cancer, <12 cm from the anal verge, by the senior author (RB) were extracted from a prospectively maintained database. Groups of patients treated with open and laparoscopic proctectomy, matched for age, gender and body mass index (BMI) with those undergoing robotic proctectomy, were selected. The quality of the TME was judged as complete, nearly complete or incomplete. CRM clearance was reported in millimetres. Physiological parameters and operative severity were assessed.ResultsAge (P = 0.619), Physiological and Operative Severity Score for the Enumeration of Morbidity and Mortality (POSSUM) score (P = 0.657), operative severity score (P = 0.977), predicted mortality (P = 0.758), comorbidities (P = 0.427), previous abdominal surgery (P = 0.941), tumour height (P = 0.912), location (P = 0.876), stage (P = 0.984), neoadjuvant chemoradiation (P = 0.625), operating time (P = 0.066), blood loss (P = 0.356), ileostomy (P = 0.934), conversion (P = 0.362), resection type (P = 1.000), flatus (P = 0.437), diet (P = 0.439), length of hospital stay (P = 0.978), complications (P = 0.671), reoperations (P = 0.804), reinterventions (P = 0.612), readmissions (P = 0.349), tumour size (P = 0.542; P = 0.532; P = 0.238), distal margin (P = 0.790), nodes harvested (P = 0.338) and pathology stage (P = 0.623) did not differ among the three groups. The quality of TME showed a trend to be lower following robotic surgery, although this was not statistically significant [open 95/5/15 (complete/nearly complete/incompete) vs laparoscopic 95/5/15 vs robotic 80/5/15; P = 0.235], but the degree of clearance at the CRM was significantly greater in robotic patients [open 8 (0–30) mm vs laparoscopic 4 (0–30) mm vs robotic 10.5 (1–30) mm; P = 0.02].ConclusionThe study reports no statistically significant difference between open and laparoscopic techniques in the quality of TME during the learning curve of robotic proctectomy for rectal cancer and demonstrates an improved CRM.
Treatment of textile dyeing wastewater by electrocoagulation using Fe and Al electrodes: optimisation of operating parameters using central composite design
Authors: Kobya Mehmet , Gengec Erhan , Sensoy Mehmet Tonay , Demirbas Erhan 
Published: April 2014

Textile dyeing wastewater was treated by electrocoagulation using aluminium and iron plate electrodes. Response surface methodology and central composite design were applied in the experiments and in statistical data analysis. A current density of 30–100 A m−2, an initial pH of 4–8, and an operating time of 10–40 min were chosen as independent variables, and the chemical oxygen demand, total organic carbon, and turbidity removal efficiencies and the operating cost were selected as responses in the electrocoagulation process. The developed quadratic models for the responses and the experimental data were in good agreement with model predictions statistically (R2 ≥ 0.92, Adj R2 ≥ 0.82, and Prob > F < 0.004). The optimised operating variables (initial pH, current density, and operating time) and the maximum total organic carbon, chemical oxygen demand, and turbidity removal efficiencies for textile dyeing wastewater were 5.5, 63.2 A m−2, 30.4 min, 77%, 82%, and 94% for the iron electrode and 5.6, 52.5 A m−2, 33.9 min, 68%, 69% and 99% for the aluminium electrode respectively. Minimum operating costs for the iron and aluminium electrodes under optimum conditions were €2.1 m−3 (€1.0 kg−1 COD) and €2.4 m−3 (€1.6 kg−1 COD). The iron electrode was found to be superior to the aluminium electrode in terms of removal efficiencies and operating cost for the treatment of textile dyeing wastewater.
The preparation and dyeing properties of pineapple leaf fibres modified with a cationic modifier
Authors: Dong Chaohong , Lu Zhou , Zhang Xiao , Zhu Ping , Li Nana 
Published: April 2014

Pineapple leaf fibres were modified with cationic modifier GX‐H23 and dodecyl dimethyl benzyl ammonium chloride (surfactant 1227) to change their surface electric properties. After modification, the pineapple leaf fibres were dyed separately with CI Reactive Red 15 under traditional and salt‐free dyeing conditions. Based on dye uptake analysis, the influence of different modification and dyeing conditions on the dyeing properties of pineapple leaf fibres were evaluated. The results showed that the modified pineapple leaf fibres under salt‐free dyeing conditions exhibited superior dye uptake, especially when the cationic modifier GX‐H23 was used. The optimum parameters for modification with surfactant 1227 were: 15 g/l of surfactant 1227, 10 g/l of sodium hydroxide, and 90 °C for 30 min; the optimum parameters for modification with cationic modifier GX‐H23 were: 15 g/l of cationic modifier GX‐H23, 20 g/l of sodium hydroxide, and 60 °C for 40 min.
Are different soil metals near the homes of pregnant women associated with mild and severe intellectual disability in children?
Authors: McDermott Suzanne , Bao Weichao , Tong Xin , Cai Bo , Lawson Andrew , Aelion C Marjorie 
Published: April 2014

AimWe explored the association of relatively low concentrations of metals in the soil proximal to maternal residence during pregnancy, with intellectual disability. We hypothesized different metals would be associated with mild versus severe intellectual disability.MethodWe used a mixed methods design, starting with a retrospective cohort from 1996 to 2002, of 10 051 pregnant mothers, soil sampling in the areas where these mothers resided during pregnancy, and follow‐up of their children to determine if there was an intellectual disability outcome. We tested the soil and then predicted the soil concentration at the maternal homes, and modeled the association with the severity of the child's intellectual disability.ResultsWe found a significant positive association between mild intellectual disability and soil mercury (p=0.007). For severe intellectual disability, there was a significant positive association with the soil arsenic and lead (p=0.025).InterpretationThis is the first report of the differential impact of metals in soil and severity of intellectual disability in children. Soil mercury concentration in the area the mother lived during pregnancy is associated with significantly increased odds of mild intellectual disability; a combination of arsenic and lead is associated with significantly increased odds of severe intellectual disability. These associations are present when controlling for maternal, child, and neighborhood characteristics.
Contribution of socio‐economic status on the prevalence of cerebral palsy: a systematic search and review
Authors: Solaski Myrill , Majnemer Annette , Oskoui Maryam 
Published: April 2014

AimThe association between socio‐economic status (SES) and cerebral palsy (CP) remains controversial. Preterm birth, low birthweight, and postnatal injuries are accepted mediating risk factors for CP, but the question remains whether SES confers additional risk. The aim of this study was to analyse existing knowledge on the relationship between SES and the risk of CP.MethodWe conducted a systematic search and review of potentially relevant research relating to SES and CP published from 1980 to 2012. Heterogeneity between studies did not allow for data aggregation or meta‐analysis; therefore, a narrative review was used to summarize the findings.ResultsTwelve studies were included in the systematic review. Of these, eight found low SES to be a risk factor for increased CP prevalence. Three studies detected statistically significant associations even after controlling for birthweight and gestational age as variables. Two of these studies also accounted for additional confounding variables (multiple births and timing of CP acquisition) and continued to detect contributory effects of SES. Linear negative correlations between CP prevalence and SES were shown by three studies.InterpretationEvidence suggests that the effect of SES on CP prevalence goes beyond that of the mediating factors preterm birth, low birthweight, and postnatal trauma. These associations were seen in area‐based and, to a lesser extent, individual measures of SES. A better understanding of mediating factors is imperative in developing targeted public health intervention programmes to reduce the prevalence of CP.
Reliability of a novel, semi‐quantitative scale for classification of structural brain magnetic resonance imaging in children with cerebral palsy
Authors: Fiori Simona , Cioni Giovanni , Klingels Katrjin , Ortibus Els , Van Gestel Leen , Rose Stephen , Boyd Roslyn N , Feys Hilde , Guzzetta Andrea 
Published: April 2014

AimTo describe the development of a novel rating scale for classification of brain structural magnetic resonance imaging (MRI) in children with cerebral palsy (CP) and to assess its interrater and intrarater reliability.MethodThe scale consists of three sections. Section 1 contains descriptive information about the patient and MRI. Section 2 contains the graphical template of brain hemispheres onto which the lesion is transposed. Section 3 contains the scoring system for the quantitative analysis of the lesion characteristics, grouped into different global scores and subscores that assess separately side, regions, and depth. A larger interrater and intrarater reliability study was performed in 34 children with CP (22 males, 12 females; mean age at scan of 9y 5mo [SD 3y 3mo], range 4y–16y 11mo; Gross Motor Function Classification System level I, [n=22], II [n=10], and level III [n=2]).ResultsVery high interrater and intrarater reliability of the total score was found with indices above 0.87. Reliability coefficients of the lobar and hemispheric subscores ranged between 0.53 and 0.95. Global scores for hemispheres, basal ganglia, brain stem, and corpus callosum showed reliability coefficients above 0.65.InterpretationThis study presents the first visual, semi‐quantitative scale for classification of brain structural MRI in children with CP. The high degree of reliability of the scale supports its potential application for investigating the relationship between brain structure and function and examining treatment response according to brain lesion severity in children with CP.
Quantitative comparison of cortical and deep grey matter in pathological subtypes of unilateral cerebral palsy
Authors: Scheck Simon M , Pannek Kerstin , Fiori Simona , Boyd Roslyn N , Rose Stephen E 
Published: April 2014

AimThe aim of this study was to quantify grey matter changes in children with unilateral cerebral palsy (UCP), differentiating between cortical or deep grey matter (CDGM) lesions, periventricular white matter (PWM) lesions, and unilateral and bilateral lesions.MethodIn a cross‐sectional study we obtained high resolution structural magnetic resonance images from 72 children (41 males, 31 females, mean age 10y 9mo [SD 3y 1mo], range 5y 1mo–17y 1mo) with UCP (33 left, 39 right hemiplegia; Manual Ability Classification System level I n=29, II n=43; Gross Motor Function Classification System level I n=46, II n=26), and 19 children with typical development (CTD; eight males, 11 females, mean age 11y 2mo [SD 2y 7mo], range 7y 8mo–16y 4mo). Images were classified by lesion type and analyzed using voxel‐based morphometry (VBM) and subcortical volumetric analysis.ResultsDeep grey matter volumes were not significantly different between children with CDGM and PWM lesions, with the thalamus, putamen, and globus pallidus being reduced unilaterally in both groups compared with CTD (p≤0.001). Children with CDGM lesions additionally showed widespread cortical changes involving all lobes using VBM (p<0.01). Children with bilateral lesions had reduced thalamus and putamen volumes bilaterally (p<0.001). The thalamic volume was reduced bilaterally in children with unilateral lesions (p=0.004).InterpretationLesions to the PWM cause secondary changes to the deep grey matter structures similar to primary changes seen in CDGM lesions. Despite having a unilateral phenotype, grey matter changes are observed bilaterally, even in children with unilateral lesions.
Congenital cytomegalovirus is associated with severe forms of cerebral palsy and female sex in a retrospective population‐based study
Authors: Smithers‐Sheedy Hayley , Raynes‐Greenow Camille , Badawi Nadia , McIntyre Sarah , Jones Cheryl A ,   the Australian Cerebral Palsy Register Group
Published: April 2014

AimCongenital cytomegalovirus (cCMV) infection can result in poor outcomes including cerebral palsy (CP). The aim of this study was to describe the incidence and comorbidities of CP reported to the Australian Cerebral Palsy Register (ACPR) as attributed to cCMV infection.MethodThis was a retrospective population‐based study. Cases were drawn from Australian state CP registers with population level ascertainment, 1993 to 2003 (n=2265; 56.4% males, Gross Motor Function Classification System [GMFCS] ratings available for Victorian cases only: 70% GMFCS levels I to III and 30% GMFCS levels IV to V). Clinical data were extracted and cases with cCMV reported as a known cause were compared with cases where cCMV was not reported.ResultsChildren with cCMV (n=34; 12 males, 22 females; mean [SD] gestational age, 36.4 wk [4.4], range 24–41 wk) accounted for 1.5% of CP cases; 2.9 per 100 000 live births, (95% confidence intervals 1.9–3.9). When compared with CP cases where cCMV was not reported, proportionally, more CP cases with cCMV were born to younger mothers (p<0.001), were female (64% vs 43%, p=0.014), had spastic quadriplegia (73% vs 21%, p<0.001), required wheeled mobility i.e. GMFCS IV or V (78% vs 28%, p<0.001), had epilepsy (70% vs 30%, p<0.001), deafness (40% vs 2%, p<0.001), functional blindness (20% vs 5%, p<0.001), and severe communication impairment (71% vs 25%, p<0.001).InterpretationcCMV is an important potentially preventable cause of CP and is associated with severe disability and female sex in cases reported to the ACPR. Future studies utilising prospective sample collection for cCMV testing are needed to confirm these findings.
Standardizing corneal nerve fibre length for nerve tortuosity increases its association with measures of diabetic neuropathy
Authors: Edwards K. , Pritchard N. , Vagenas D. , Russell A. , Malik R. A. , Efron N. 
Published: April 2014

AbstractAimsRecent studies on corneal markers have advocated corneal nerve fibre length as the most important measure of diabetic peripheral neuropathy. The aim of this study was to determine if standardizing corneal nerve fibre length for tortuosity increases its association with other measures of diabetic peripheral neuropathy.MethodsTwo hundred and thirty‐one individuals with diabetes with either predominantly mild or absent neuropathic changes and 61 control subjects underwent evaluation of diabetic neuropathy symptom score, neuropathy disability score, testing with 10‐g monofilament, quantitative sensory testing (warm, cold, vibration detection) and nerve conduction studies. Corneal nerve fibre length and corneal nerve fibre tortuosity were measured using corneal confocal microscopy. A tortuosity‐standardised corneal nerve fibre length variable was generated by dividing corneal nerve fibre length by corneal nerve fibre tortuosity. Differences in corneal nerve morphology between individuals with and without diabetic peripheral neuropathy and control subjects were determined and associations were estimated between corneal morphology and established tests of, and risk factors for, diabetic peripheral neuropathy.ResultsThe tortuosity‐standardised corneal nerve fibre length variable was better than corneal nerve fibre length in demonstrating differences between individuals with diabetes, with and without neuropathy (tortuosity‐standardised corneal nerve fibre length variable: 70.5 ± 27.3 vs. 84.9 ± 28.7, P < 0.001, receiver operating characteristic area under the curve = 0.67; corneal nerve fibre length: 15.9 ± 6.9 vs. 18.4 ± 6.2 mm/mm2, P = 0.004, receiver operating characteristic area under the curve = 0.64). Furthermore, the tortuosity‐standardised corneal nerve fibre length variable demonstrated a significant difference between the control subjects and individuals with diabetes, without neuropathy, while corneal nerve fibre length did not (tortuosity‐standardised corneal nerve fibre length variable: 94.3 ± 27.1 vs. 84.9 ± 28.7, P = 0.028; corneal nerve fibre length: 20.1 ± 6.3 vs. 18.4 ± 6.2 mm/mm2, P = 0.084). Correlations between corneal nerve fibre length and established measures of neuropathy and risk factors for neuropathy were higher when a correction was made for the nerve tortuosity.ConclusionsStandardizing corneal nerve fibre length for tortuosity enhances the ability to differentiate individuals with diabetes, with and without neuropathy.