cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.5241238
title: Erratum: Postoperative Neurological Deficit after Carotid Endarterectomy: A Mid-Term Outcome Analysis

description: <i>Background:</i> To evaluate the neurological outcome of postoperative neurological deficit (PND) in patients undergoing carotid endarterectomy (CEA). <i>Methods:</i> A total of 3.7% (n = 48) out of 1,290 consecutive patients developed PND and were assessed neurologically after a mid-term follow-up. <i>Results:</i> After a 4-year follow-up, these patients were neurologically reevaluated. Clinical assessment revealed that 48% (n = 13) of the patients had a Rankin scale score of 0 or 1, 56% (n = 14) had a National Institutes of Health Stroke Scale score of 0 or 1, and 68.5% (n = 17) reached the maximum score on the Barthel index. <i>Conclusions:</i> The neurofunctional prognosis of PND is good. Four years after CEA, almost half of the patients had normal or near-normal neuroclinical findings.
DOI: 10.6084/m9.figshare.5241247.v1
title: Erratum: β-Globin Gene Cluster Haplotypes in a Cohort of 221 Children with Sickle Cell Anemia or Sβ⁰-Thalassemia and Their Association with Clinical and Hematological Features

description: <i>Background/Aims:</i> β<sup>S</sup>-Haplotype prevalence and its associations with clinical and hematological characteristics were assessed in Brazilian children with sickle cell anemia or Sβ⁰-thalassemia. <i>Methods:</i> A retrospective randomized cohort study was undertaken with 208 SS and 13 Sβ⁰-thalassemia children derived from the Newborn Screening Program of the state of Minas Gerais. β<sup>S</sup>-Haplotypes were determined by PCR-RFLP. <i>Results:</i> Thirty-nine percent of the SS subjects had the CAR/CAR genotype, 33% had CAR/Ben, 24% had Ben/Ben, 1% had CAR/Atp, 1% had Ben/Atp, and 1% had Arab-Indian/Ben; 1% could not be characterized. Of the Sβ⁰-thalassemia children, 5 were CAR/undefined, 2 were Ben/undefined, and 1 was CAM/undefined. There was no significant association between β<sup>S</sup>-haplotypes and the total Hb, Hb F, MCV, MCH, WBC, and reticulocyte count among the SS children. Likewise, no significant association was detected between β<sup>S</sup>-haplotypes and the frequency of acute chest syndrome episodes, blood transfusions, splenic sequestration, or cerebrovascular disease (high-risk/conditional transcranial Doppler ultrasonography or clinical stroke). A limited number of Sβ⁰-thalassemia children precluded valid analyses. <i>Conclusions:</i> The prevalence of β<sup>S</sup>-haplotypes in this study is in agreement with the historical records of African slaves brought to the state of Minas Gerais. Furthermore, β<sup>S</sup>-haplotypes CAR and Ben were not associated with any analyzed feature of children with sickle cell anemia.
DOI: 10.6084/m9.figshare.5241235
title: Erratum: Currarino Triad: Surgical Management and Follow-Up Results of Three Cases

description: The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic constipation and pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their anorectal malformations were characterized as anal stenoses (4 patients), associated with Hirschsprung’s disease in 2 cases. In 3 patients, MRI showed tethered cord syndrome in addition to the presacral mass. There was hydrocephalus in 1 patient. Anal stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary colostomy (2 patients) had been performed previously due to Hirschsprung’s disease. We performed a posterior procedure via lumbar and sacral partial laminectomy-laminoplasty and transdural ligation of the neck of the meningocele for anterior sacral meningoceles, or alternatively, tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral meningoceles and 1 was a teratoma. One of them also had a spinal abscess. He required reoperation (twice) and appeared at the time to have improved with medical therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral meningocele or a solid tumor without severe anorectal malformation, it can be managed with posterior lumbar and sacral procedures. Such approaches are performed easily by transdural ligation of the neck of the anterior sacral meningocele or through tumor excision.
DOI: 10.6084/m9.figshare.5241241
title: Erratum: A Phase III Randomized Equivalence Study of Biosimilar Filgrastim versus Amgen Filgrastim in Patients Receiving Myelosuppressive Chemotherapy for Breast Cancer

description: <i>Background:</i> Filgrastim was developed to treat chemotherapy-induced neutropenia. This phase III study was designed to demonstrate bioequivalence of Amgen filgrastim and a biosimilar filgrastim developed by Hospira (Study GCF071; sponsored by Hospira). <i>Patients and Methods:</i> Breast cancer patients suitable for treatment with doxorubicin and docetaxel in the neoadjuvant/adjuvant or first-line metastatic setting were enrolled at 37 European centers. Patients were randomized (2:1) to receive Hospira filgrastim or Amgen filgrastim, after the end of chemotherapy. Filgrastim (5 µg/kg/day) was administered under double-blind conditions. Primary endpoint to demonstrate bioequivalence was duration of severe neutropenia (DSN) in cycle 1. <i>Results:</i> 184 patients were randomized to Hospira filgrastim and 95 to Amgen filgrastim. Mean DSN in cycle 1 was similar with Hospira filgrastim (1.6 days; n = 165) and Amgen filgrastim (1.3 days; n = 85), meeting predefined criteria for bioequivalence. Secondary endpoints supporting bioequivalence included mean time to absolute neutrophil count recovery and incidence of febrile neutropenia. The most common treatment-related adverse event with Hospira filgrastim was grade 1–2 bone pain. <i>Conclusions:</i> Hospira filgrastim and Amgen filgrastim are bioequivalent in efficacy with similar safety profiles. Hospira filgrastim may be useful for the prophylaxis of complications related to neutropenia caused by chemotherapy.
DOI: 10.6084/m9.figshare.5241229
title: Erratum: Comparison of IgE-Binding Capacity, Cross-Reactivity and Biological Potency of Allergenic Non-Specific Lipid Transfer Proteins from Peach, Cherry and Hazelnut

description: <i>Background:</i> Whether the observed clinical pattern of non-specific lipid transfer protein (nsLTP)-mediated food allergies is attributable to a primary sensitization by Pru p 3 from peach and subsequent cross-reactivity with Rosaceae- and non-Rosaceae-derived foods expressing homologous allergens is still unclear. <i>Objective:</i> To investigate the allergenic properties of nsLTPs from Rosaceae and non-Rosaceae foods. <i>Methods:</i> In peach-, cherry- or hazelnut-allergic patients, prevalence of sensitization, IgE-binding capacity, cross-reactivity and allergenic potency of Pru p 3 was compared with Pru av 3 (cherry) and Cor a 8 (hazelnut). <i>Results:</i> Frequency of sensitization to corresponding nsLTPs was 88, 85, and 77% in peach-, hazelnut- and cherry-allergic patients, respectively. Concomitant allergic reactions to cherry and hazelnut were reported in 51 and 44% of peach-allergic patients, respectively. In contrast to cherry allergy, hazelnut allergy was not strictly associated to peach allergy. Sensitization to Cor a 8 or Pru av 3 was strongly correlated with IgE reactivity to Pru p 3, even when subjects tolerated peach. Specific IgE was highest for Rosaceae LTPs, and cross-inhibition experiments confirmed a stronger IgE-binding capacity of Pru p 3 than Cor a 8. The biological potency of Pru p 3 and Pru av 3 was similar but stronger for both nsLTPs than that of Cor a 8. <i>Conclusion:</i> Clinical cross-reactivity of food-allergic patients in the Mediterranean area is likely attributed to a primary sensitization to Pru p 3 and serological cross-reactivity with homologous food nsLTPs. In comparison to Cor a 8, Rosaceae nsLTPs showed a stronger IgE-binding capacity and allergenic potency indicating a different epitope pattern.
DOI: 10.6084/m9.figshare.5241232
title: Erratum: Skin and Nail Responses after 1 Year of Infliximab Therapy in Patients with Moderate-to-Severe Psoriasis: A Retrospective Analysis of the EXPRESS Trial

description: <i>Background:</i> The EXPRESS study demonstrated the initial efficacy of infliximab in psoriasis affecting the skin and nails. <i>Objective:</i> To further assess how patients with a positive initial response to infliximab respond to 1 year of continuous infliximab treatment. <i>Methods:</i> A retrospective analysis of patients with moderate-to-severe plaque and nail psoriasis who initiated and continued infliximab treatment up to week 46 was conducted. <i>Results:</i> Among all nail psoriasis patients receiving 1 year of infliximab (n = 186), 74.6 and 54.1% achieved at least 75 or 90% improvement in the Psoriasis Area and Severity Index (PASI) at week 50. These PASI-75 (n = 138) and PASI-90 (n = 100) responders had respective mean improvements from baseline to week 50 of 93.6 and 97.3% for the PASI, 90.6 and 94.1% for the Dermatology Life Quality Index (DLQI) and 78.2 and 80.3% for the Nail Psoriasis Severity Index (NAPSI). <i>Conclusions:</i> Infliximab treatment for 1 year produced sustained improvement in PASI, DLQI and NAPSI scores.
DOI: 10.6084/m9.figshare.5241241.v1
title: Erratum: A Phase III Randomized Equivalence Study of Biosimilar Filgrastim versus Amgen Filgrastim in Patients Receiving Myelosuppressive Chemotherapy for Breast Cancer

description: <i>Background:</i> Filgrastim was developed to treat chemotherapy-induced neutropenia. This phase III study was designed to demonstrate bioequivalence of Amgen filgrastim and a biosimilar filgrastim developed by Hospira (Study GCF071; sponsored by Hospira). <i>Patients and Methods:</i> Breast cancer patients suitable for treatment with doxorubicin and docetaxel in the neoadjuvant/adjuvant or first-line metastatic setting were enrolled at 37 European centers. Patients were randomized (2:1) to receive Hospira filgrastim or Amgen filgrastim, after the end of chemotherapy. Filgrastim (5 µg/kg/day) was administered under double-blind conditions. Primary endpoint to demonstrate bioequivalence was duration of severe neutropenia (DSN) in cycle 1. <i>Results:</i> 184 patients were randomized to Hospira filgrastim and 95 to Amgen filgrastim. Mean DSN in cycle 1 was similar with Hospira filgrastim (1.6 days; n = 165) and Amgen filgrastim (1.3 days; n = 85), meeting predefined criteria for bioequivalence. Secondary endpoints supporting bioequivalence included mean time to absolute neutrophil count recovery and incidence of febrile neutropenia. The most common treatment-related adverse event with Hospira filgrastim was grade 1–2 bone pain. <i>Conclusions:</i> Hospira filgrastim and Amgen filgrastim are bioequivalent in efficacy with similar safety profiles. Hospira filgrastim may be useful for the prophylaxis of complications related to neutropenia caused by chemotherapy.
DOI: 10.6084/m9.figshare.5241235.v1
title: Erratum: Currarino Triad: Surgical Management and Follow-Up Results of Three Cases

description: The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic constipation and pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their anorectal malformations were characterized as anal stenoses (4 patients), associated with Hirschsprung’s disease in 2 cases. In 3 patients, MRI showed tethered cord syndrome in addition to the presacral mass. There was hydrocephalus in 1 patient. Anal stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary colostomy (2 patients) had been performed previously due to Hirschsprung’s disease. We performed a posterior procedure via lumbar and sacral partial laminectomy-laminoplasty and transdural ligation of the neck of the meningocele for anterior sacral meningoceles, or alternatively, tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral meningoceles and 1 was a teratoma. One of them also had a spinal abscess. He required reoperation (twice) and appeared at the time to have improved with medical therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral meningocele or a solid tumor without severe anorectal malformation, it can be managed with posterior lumbar and sacral procedures. Such approaches are performed easily by transdural ligation of the neck of the anterior sacral meningocele or through tumor excision.
DOI: 10.6084/m9.figshare.5241238.v1
title: Erratum: Postoperative Neurological Deficit after Carotid Endarterectomy: A Mid-Term Outcome Analysis

description: <i>Background:</i> To evaluate the neurological outcome of postoperative neurological deficit (PND) in patients undergoing carotid endarterectomy (CEA). <i>Methods:</i> A total of 3.7% (n = 48) out of 1,290 consecutive patients developed PND and were assessed neurologically after a mid-term follow-up. <i>Results:</i> After a 4-year follow-up, these patients were neurologically reevaluated. Clinical assessment revealed that 48% (n = 13) of the patients had a Rankin scale score of 0 or 1, 56% (n = 14) had a National Institutes of Health Stroke Scale score of 0 or 1, and 68.5% (n = 17) reached the maximum score on the Barthel index. <i>Conclusions:</i> The neurofunctional prognosis of PND is good. Four years after CEA, almost half of the patients had normal or near-normal neuroclinical findings.
DOI: 10.6084/m9.figshare.5241229.v1
title: Erratum: Comparison of IgE-Binding Capacity, Cross-Reactivity and Biological Potency of Allergenic Non-Specific Lipid Transfer Proteins from Peach, Cherry and Hazelnut

description: <i>Background:</i> Whether the observed clinical pattern of non-specific lipid transfer protein (nsLTP)-mediated food allergies is attributable to a primary sensitization by Pru p 3 from peach and subsequent cross-reactivity with Rosaceae- and non-Rosaceae-derived foods expressing homologous allergens is still unclear. <i>Objective:</i> To investigate the allergenic properties of nsLTPs from Rosaceae and non-Rosaceae foods. <i>Methods:</i> In peach-, cherry- or hazelnut-allergic patients, prevalence of sensitization, IgE-binding capacity, cross-reactivity and allergenic potency of Pru p 3 was compared with Pru av 3 (cherry) and Cor a 8 (hazelnut). <i>Results:</i> Frequency of sensitization to corresponding nsLTPs was 88, 85, and 77% in peach-, hazelnut- and cherry-allergic patients, respectively. Concomitant allergic reactions to cherry and hazelnut were reported in 51 and 44% of peach-allergic patients, respectively. In contrast to cherry allergy, hazelnut allergy was not strictly associated to peach allergy. Sensitization to Cor a 8 or Pru av 3 was strongly correlated with IgE reactivity to Pru p 3, even when subjects tolerated peach. Specific IgE was highest for Rosaceae LTPs, and cross-inhibition experiments confirmed a stronger IgE-binding capacity of Pru p 3 than Cor a 8. The biological potency of Pru p 3 and Pru av 3 was similar but stronger for both nsLTPs than that of Cor a 8. <i>Conclusion:</i> Clinical cross-reactivity of food-allergic patients in the Mediterranean area is likely attributed to a primary sensitization to Pru p 3 and serological cross-reactivity with homologous food nsLTPs. In comparison to Cor a 8, Rosaceae nsLTPs showed a stronger IgE-binding capacity and allergenic potency indicating a different epitope pattern.
DOI: 10.6084/m9.figshare.5241232.v1
title: Erratum: Skin and Nail Responses after 1 Year of Infliximab Therapy in Patients with Moderate-to-Severe Psoriasis: A Retrospective Analysis of the EXPRESS Trial

description: <i>Background:</i> The EXPRESS study demonstrated the initial efficacy of infliximab in psoriasis affecting the skin and nails. <i>Objective:</i> To further assess how patients with a positive initial response to infliximab respond to 1 year of continuous infliximab treatment. <i>Methods:</i> A retrospective analysis of patients with moderate-to-severe plaque and nail psoriasis who initiated and continued infliximab treatment up to week 46 was conducted. <i>Results:</i> Among all nail psoriasis patients receiving 1 year of infliximab (n = 186), 74.6 and 54.1% achieved at least 75 or 90% improvement in the Psoriasis Area and Severity Index (PASI) at week 50. These PASI-75 (n = 138) and PASI-90 (n = 100) responders had respective mean improvements from baseline to week 50 of 93.6 and 97.3% for the PASI, 90.6 and 94.1% for the Dermatology Life Quality Index (DLQI) and 78.2 and 80.3% for the Nail Psoriasis Severity Index (NAPSI). <i>Conclusions:</i> Infliximab treatment for 1 year produced sustained improvement in PASI, DLQI and NAPSI scores.
DOI: 10.6084/m9.figshare.5241211
title: Erratum: Delta-Centralization Fails to Control for Population Stratification in Genetic Association Studies

description: <i>Objective:</i> To investigate the validity of simulations and assumptions used to underpin the delta-centralization (DC) method for correcting for population stratification in genetic association studies; to assess the effectiveness of DC compared to genomic control (GC) under valid simulation conditions; and to highlight other studies employing similarly flawed simulations. <i>Methods:</i> DC and GC use data from unlinked null loci to correct the test statistic at the target locus, but differ in the way the correction is performed. We compare DC and GC under two simulation approaches: an invalid approach adopted by the originators of DC, which permits subpopulation allele frequency matching of null markers to the target locus; and a valid approach, based on the Balding-Nichols model, which does not allow subpopulation matching. <i>Results:</i> DC works under invalid simulation conditions (subpopulation allele frequency matching), but not under our valid ones. We use theoretical arguments to assert that there are no valid conditions under which DC might work. We identify several studies which have adopted similarly invalid simulation approaches in this field. <i>Conclusion:</i> DC fails to control for population stratification and should not be used. Other results from studies which have used the same invalid simulation approach should be treated cautiously.
DOI: 10.6084/m9.figshare.5241220
title: Erratum: Urinary Pigment Epithelium-Derived Factor as a Marker of Diabetic Nephropathy

description: <i>Background:</i> Pigment epithelium-derived factor (PEDF), a serine protease inhibitor, regulates extracellular matrix production in the kidney. We sought the association between urinary PEDF (uPEDF) and development of nephropathy among patients with type 2 diabetes (T2DM). <i>Methods:</i> Two human studies were performed in which uPEDF was determined by ELISA. These studies included (1) a cross-sectional study of T2DM (n = 228) and healthy controls (n = 49) and (2) a longitudinal study of hypertensive T2DM with microalbuminuria (MA; n = 42) treated with irbesartan for 6 months. An animal study was performed in which PEDF was measured in the kidney and urine samples of control rats, rats rendered diabetic with streptozotocin that were also fed a high-fat diet, and diabetic rats treated with irbesartan for 3 months. <i>Results:</i> Cross-sectional study: compared to controls, uPEDF was significantly higher in patients with diabetic nephropathy. uPEDF independently correlated with MA. In the MA group, uPEDF in patients with diabetic retinopathy was significantly higher than that in patients without diabetic retinopathy. Longitudinal study: irbesartan significantly decreased uPEDF in T2DM with MA. Animal study: in diabetic rats, increased PEDF was observed in both the urine and kidney samples. uPEDF showed a significant correlation with the expression of PEDF in the kidney. Irbesartan could significantly decrease the PEDF expression in the kidneys of diabetic rats as well as uPEDF. <i>Conclusion:</i> uPEDF may serve as a novel marker for screening for nephropathy among patients with T2DM and monitoring the response to therapy.
DOI: 10.6084/m9.figshare.5241223
title: Erratum: New Technologies for the Identification of Novel Genetic Markers of Disorders of Sex Development (DSD)

description: Although the genetic basis of human sexual determination and differentiation has advanced considerably in recent years, the fact remains that in most subjects with disorders of sex development (DSD) the underlying genetic cause is unknown. Where pathogenic mutations have been identified, the phenotype can be highly variable, even within families, suggesting that other genetic variants are influencing the expression of the phenotype. This situation is likely to change, as more powerful and affordable tools become widely available for detailed genetic analyses. Here, we describe recent advances in comparative genomic hybridisation, sequencing by hybridisation and next generation sequencing, and we describe how these technologies will have an impact on our understanding of the genetic causes of DSD.
DOI: 10.6084/m9.figshare.5241226
title: Erratum: Impact of Molecular Genetics on Congenital Adrenal Hyperplasia Management

description: Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders caused by mutations in genes encoding the enzymes involved in one of the 5 steps of adrenal steroid synthesis or the electron donor P450 oxidoreductase (POR) enzyme. Steroid 21-hydroxylase deficiency (21-OHD), the principal focus of this review, accounts for about 90–95% of all CAH cases, and its biochemical and clinical severity depends on the underlying <i>CYP21A2</i> gene disruption. Molecular genetic advancements have been achieved in recent years, and the aim of this review is to attempt to highlight its contribution to the comprehension and management of the disease. When possible, we will try to achieve this goal also by providing some results from our personal experience regarding: some aspects of <i>CYP21A2</i> gene analysis, with basic genotype/phenotype relationships; its crucial role in both genetic counselling and in prenatal diagnosis and treatment in families at risk for 21-OHD; its help in the comprehension of the severity of the disease in patients diagnosed by neonatal screening and possibly treated before an evident salt-loss crisis or before performing adequate blood sampling; its usefulness in the definition of post ACTH 17-hydroxyprogesterone values, discriminating between non-classic, heterozygote and normal subjects; and finally the contribution of genes other than <i>CYP21A2</i> whose function or dysfunction could influence 21-hydroxylase activity and modify the presentation or management of the disease.