Miracle-dz/UniFAR-FTUs huggingface dataset:
The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair

Abstract Objective: We sought to determine the prevalence of attention-deficit/hyperactivity disorder in a population of children who underwent neonatal heart surgery involving repair of the aortic arch for Norwood Stage I, interrupted aortic arch, and combined repair of aortic coarctation with ventricular septal defect. Methods: Children between the ages of 5 and 16 were surveyed using the ADHD-IV and the Child Heath Questionnaire-50. Classification as attention-deficit/hyperactivity disorder was defined for this study as either a parent-reported diagnosis of attention-deficit/hyperactivity disorder or ADHD-IV inattention score of ⩾93 percentile. Results: Of the 134 surveys, 57 (43%) were returned completed. A total of 25 (44%) children either had a diagnosis of attention-deficit/hyperactivity disorder and/or ADHD-IV inattention score ⩾93 percentile. Eleven of the 13 (85%) children with interrupted aortic arch, 3 of the 7 (42.9%) children with combined coarctation/ventricular septal defect repair, and 9 of the 33 (27.3%) children with hypoplastic left-heart syndrome were classified as having attention-deficit/hyperactivity disorder. Only 7 of the 25 (28%) children received medical treatment for this condition. Quality of life indicators in the Child Heath Questionnaire-50 Questionnaire were highly correlated with the ADHD-IV scores. Conclusion: The risks for the development of attention-deficit/hyperactivity disorder are multifactorial but are significantly increased in this post-surgical population. This study revealed a low treatment rate for attention-deficit/hyperactivity disorder, and a significant impact on the quality of life in these children.
Role of speckle tracking echocardiography in the assessment of post-repair left ventricular function in patients with late presentation of anomalous origin of the left coronary artery from the pulmonary artery

Abstract The aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p<0.001), but not the septal wall of the left ventricle (p=0.65). Moreover, the strain values by the speckle tracking method revealed significant differences between the patient and the control group regarding the global strain (p=0.016) and anterior, lateral, and posterior segments of the left ventricle. Although postoperative conventional echocardiography revealed normal global left ventricular function with acceptable ejection fraction, abnormal myocardial deformation of the variable segments of the left ventricle with regional and global myocardial dysfunction were well defined by speckle tracking echocardiography.
Cardiopulmonary Responses of Wistar Kyoto, Spontaneously Hypertensive, and Stroke-prone Spontaneously Hypertensive Rats to Particulate Matter (PM) Exposure

Humans with underlying cardiovascular disease, including stroke, are more susceptible to ambient particulate matter (PM)-induced morbidity and mortality. We hypothesized that stroke-prone spontaneously hypertensive rats (SHRSP) would be more susceptible than healthy Wistar Kyoto (WKY) rats to PM-induced cardiac oxidative stress and pulmonary injury. We further postulated that PM-induced injury would be greater in SHRSP than in spontaneously hypertensive rats (SHR) based on the greater disease severity in SHRSP than SHR. First, male WKY and SHRSP were intratracheally (IT) instilled with saline or 1.11, 3.33, or 8.33 mg/kg of oil combustion PM and responses were analyzed 4 or 24 h later. Second, SHR and SHRSP were IT instilled with saline or 3.33 or 8.33 mg/kg of the same PM and responses were analyzed 24 h later. Pulmonary injury and inflammation were assessed in bronchoalveolar lavage fluid (BALF) and cardiac markers in cytosolic and mitochondrial fractions. BALF neutrophilic inflammatory response was induced similarly in all strains following PM exposure. BALF protein leakage, γ-glutamyl transferase, and N-acetylglucosaminidase activities, but not lactate dehydrogenase activity, were exacerbated in SHRSP compared to WKY or SHR. Pulmonary cytosolic and cardiac mitochondrial ferritin levels decreased, and cardiac cytosolic superoxide dismutase (SOD) activity increased in SHRSP only. Pulmonary SOD activity decreased in WKY and SHRSP. Cardiac mitochondrial isocitrate dehydrogenase (ICDH) activity decreased in PM-exposed WKY and SHR; control levels were lower in SHRSP than SHR or WKY. In summary, strain-related differences exist in pulmonary protein leakage and oxidative stress markers. PM-induced changes in cardiac oxidative stress sensitive enzymes are small, and appear only slightly exacerbated in SHRSP compared to WKY or SHR. Multiple biological markers may be differentially affected by PM in genetic models of cardiovascular diseases. Preexisting cardiovascular disease may influence susceptibility to PM pulmonary and cardiac health effects in a disease-specific manner.
A Novel Method for Expediting the Development of Patient-Reported Outcome Measures and an Evaluation Across Several Populations

Item response theory (IRT) models provide an appropriate alternative to the classical ordinal confirmatory factor analysis (CFA) during the development of patient-reported outcome measures (PROMs). Current literature has identified the assessment of IRT model fit as both challenging and underdeveloped. This study evaluates the performance of Ordinal Bayesian Instrument Development (OBID), a Bayesian IRT model with a probit link function approach, through applications in two breast cancer-related instrument development studies. The primary focus is to investigate an appropriate method for comparing Bayesian IRT models in PROMs development. An exact Bayesian leave-one-out cross-validation (LOO-CV) approach is implemented to assess prior selection for the item discrimination parameter in the IRT model and subject content experts’ bias (in a statistical sense and not to be confused with psychometric bias as in differential item functioning) toward the estimation of item-to-domain correlations. Results support the utilization of content subject experts’ information in establishing evidence for construct validity when sample size is small. However, the incorporation of subject experts’ content information in the OBID approach can be sensitive to the level of expertise of the recruited experts. More stringent efforts need to be invested in the appropriate selection of subject experts to efficiently use the OBID approach and reduce potential bias during PROMs development.
Recurrence analysis in 66 cases with grade I and grade II chondrosarcomas in the extremities

Objective: We analyzed chondrosarcomas in long bones to assess whether Grade I and II chondrosarcomas should both be grouped as low grade malignant tumors (musculoskeletal Tumor Society Stage I or Stage II), and to explore rational surgical treatment for Grade I and II chondrosarcomas.
HNF1α Inactivation Promotes Lipogenesis in Human Hepatocellular Adenoma Independently of SREBP-1 and Carbohydrate-response Element-binding Protein (ChREBP) Activation*

Biallelic inactivating mutations of the transcription factor 1 gene (TCF1), encoding hepatocyte nuclear factor 1α (HNF1α) were identified in 50% of hepatocellular adenomas (HCA) phenotypically characterized by a striking steatosis. To understand the molecular basis of this aberrant lipid storage, we performed a microarray transcriptome analysis validated by quantitative reverse transcription-PCR, Western blotting, and lipid profiling. In mutated HCA, we showed a repression of gluconeogenesis coordinated with an activation of glycolysis, citrate shuttle, and fatty acid synthesis predicting elevated rates of lipogenesis. Moreover, the strong down-regulation of liver fatty acid-binding protein suggests that impaired fatty acid trafficking may also contribute to the fatty phenotype. In addition, transcriptional profile analysis of the observed deregulated genes in non-HNF1α-mutated HCA as well as in non-tumor livers allowed us to define a specific signature of the HNF1α-mutated HCA. In these tumors, lipid composition was dramatically modified according to the transcriptional deregulations identified in the fatty acid synthetic pathway. Surprisingly, lipogenesis activation did not operate through sterol regulatory element-binding protein-1 (SREBP-1) and carbohydrate-response element-binding protein (ChREBP) that were repressed. We conclude that steatosis in HNF1α-mutated HCA results mainly from an aberrant promotion of lipogenesis that is linked to HNF1α inactivation and that is independent of both SREBP-1 and ChREBP activation. Finally, our findings have potential clinical implications since lipogenesis can be efficiently inhibited by targeted therapies.
Parents and end-of-life decision-making for their child: roles and responsibilities

Background Whether parents want to be and should be the decision-maker for their child in end-of-life matters are contested clinical and ethical questions. Previous research outcomes are equivocal. Method A qualitative interview method was used to examine the views and experiences of 25 bereaved parents in end-of-life decision-making for their child. Data were analysed thematically. Results Three types of decision-making roles were identified: self-determined, guided (both involving active decision-making) and acquiescent (passive). The majority of parents had been active in the decision-making process for their child. They perceived themselves as the ultimate end-of-life decision-maker. This was perceived as part of their parental responsibility. A minority of parents did not consider that they had been an active, ultimate decision-maker. Generally, parents in the self-determined and guided groups reported no negative consequences from their decision-making involvement. Importantly, parents in the acquiescent group described their experience as difficult at the time and subsequently, although not all difficulties related directly to decision-making. Parents considered that in principle parents should be the end-of-life decision-maker for their child, but understood personal characteristics and preference could prevent some parents from taking this role. Conclusions This study unequivocally supports parents’ desire to fulfil the end-of-life decision-making role. It provides a nuanced understanding of parents’ roles and contributes evidence for the ethical position that parents should be the end-of-life decision-makers for their child, unless not in the child's best interests. On the whole, parents want this role and can manage its consequences. Indeed, not being the end-of-life decision-maker could be detrimental to parents’ well-being.
Fascia Augmentation of the Vocal Fold: Graft Yield in the Canine and Preliminary Clinical Experience

Introduction Glottal insufficiency resulting from vocal fold bowing, hypomobility, or scar is frequently treated by injection augmentation. Injection augmentation with fat, collagen, gel foam, polytef, and recently, fascia lata has been previously reported. Variable graft yield and poor host‐tissue tolerance have motivated the continued search for an ideal graft substance.
Individual voxel‐based subtype prediction can differentiate progressive supranuclear palsy from idiopathic parkinson syndrome and healthy controls

Voxel‐based morphometry (VBM) shows a differentiated pattern in patients with atypical Parkinson syndrome but so far has had little impact in individual cases. It is desirable to translate VBM findings into clinical practice and individual classification. To this end, we examined whether a support vector machine (SVM) can provide useful accuracies for the differential diagnosis. We acquired a volumetric 3D T1‐weighted MRI of 21 patients with idiopathic Parkinson syndrome (IPS), 11 multiple systems atrophy (MSA‐P) and 10 progressive supranuclear palsy (PSP), and 22 healthy controls. Images were segmented, normalized, and compared at group level with SPM8 in a classical VBM design. Next, a SVM analysis was performed on an individual basis with leave‐one‐out cross‐validation. VBM showed a strong white matter loss in the mesencephalon of patients with PSP, a putaminal grey matter loss in MSA, and a cerebellar grey matter loss in patients with PSP compared with IPS. The SVM allowed for an individual classification in PSP versus IPS with up to 96.8% accuracy with 90% sensitivity and 100% specificity. In MSA versus IPS, an accuracy of 71.9% was achieved; sensitivity, however, was low with 36.4%. Patients with IPS could not be differentiated from controls. In summary, a voxel‐based SVM analysis allows for a reliable classification of individual cases in PSP that can be directly clinically useful. For patients with MSA and IPS, further developments like quantitative MRI are needed. Hum Brain Mapp, 2011. © 2011 Wiley‐Liss, Inc.
The regularized SNN-TA model for recognition of noisy speech

The segmental neural network (SNN) architecture was introduced at BBN by Zavaliagkos et al. (1994) for rescoring the N-best hypothesis yielded by a standard continuous density hidden Markov model (CDHMM) applied to automatic speech recognition. An enhanced connectionist model, called SNN with trainable amplitude of activation functions (SNN-TA), presented for use instead of the CDHMM to perform the recognition of isolated words. Viterbi-based segmentation is then introduced, relying on the level building algorithm, that can be combined with the SNN-TA to obtain a hybrid framework for continuous speech recognition. The present paradigm is applied to the recognition of isolated digits, collected in a real car environment under several noisy conditions (traffic, speed, road conditions, etc.) using a microphone placed far from the talker. We stress the fact that robustness to noise can be increased by improving the generalization capabilities of the speech recognizer. In this perspective, while CDHMM completely lack of a proper regularization theory, a regularized SNN-TA model is discussed, which yields effective generalization and noise-tolerance, outperforming the CDHMM on the noisy task under consideration.
Value and cost of teaching hospitals: A prospective, multicenter, inception cohort study

ObjectiveTo examine variations in case-mix, structure, resource use, and outcome performance among teaching and nonteaching intensive care units (ICU). Design:Prospective inception cohort study. Patients: Aconsecutive sample of 15,297 patients at 35 hospitals, which compared 8,269 patients admitted to 20 teaching ICUs at 18 hospitals vs. 7,028 patients admitted to 17 non-teaching ICUs at 17 hospitals. Interventions:None. Measurements:We selected demographic, physiologic, and treatment information for an average of 415 patients at each ICU, and collected data on hospital and ICU structure. Outcomes were compared using ratios of observed to risk-adjusted predicted hospital death rates, ICU length of stay, and resource use. Main Results:When compared to nonteaching ICUs, teaching ICUs had twice the number of physicians who regulary provided services and cared for significantly younger and more severely ill (p < .001) patients. Risk-adjusted ICU length of stay was similar, but resource use was significantly (p < .001) greater in teaching ICUs, with $3,000 (10.5%) of estimated total costs for an average ICU admission related to increased use of diagnostic testing and invasive procedures in teaching ICUs. Risk-adjusted hospital death rates were not significantly different (p = .1) between all teaching and nonteaching ICUs, but were significantly (p < .05) better in four teaching ICUs, but in only one nonteaching ICU. The 14 hospitals that were members of the Council of Teaching Hospitals had significantly better risk-adjusted outcome in their 16 ICUs than all others (odds ratio = 1.21, confidence interval 1.06 to 1.38, p = .004). Conclusions:Teaching ICUs care for more complex patients in a substantially more complicated organizational setting. The best risk-adjusted survival rates occur at teaching ICUs, but production cost is higher in teaching units, secondary to increased testing and therapy. Teaching ICUs are also successfully transferring knowledge to trainees who, after their training, are achieving equivalent results at slightly lower cost in nonteaching ICUs. (Crit Care Med 1993; 21:1432–1442)
Objectively measured secondhand smoke exposure and mental health in children: evidence from the Scottish Health Survey.

OBJECTIVE To examine the association between objectively assessed secondhand smoke (SHS) exposure and mental health in a representative sample of British children. DESIGN Cross-sectional study. SETTING Community-based population sample from the 2003 Scottish Health Survey. PARTICIPANTS Nine hundred one nonsmoking children (mean [SD] age, 8.3 [2.5] years). MAIN EXPOSURE Exposure to SHS was determined from salivary cotinine level and self-report. MAIN OUTCOME MEASURE Psychological distress assessed using the Strengths and Difficulties Questionnaire (SDQ). RESULTS Forty percent of the sample demonstrated high SHS exposure (cotinine level >0.70 ng/mL). Children with higher cotinine levels were more likely to live in areas of greater socioeconomic deprivation. Participants in the highest cotinine quartile (>0.70 ng/mL) had significantly higher total SDQ scores compared with those in the lowest quartile (age- and sex-adjusted mean difference = 2.8; 95% confidence interval, 1.6 to 3.9). There was evidence of a dose-response effect across the cotinine group (P trend = .001). Of the SDQ subscales, the strongest associations with cotinine levels emerged for hyperactivity and conduct disorder. These associations remained statistically significant after adjustment for possible confounders including social deprivation, single-parent status, body mass index, chronic illness, and physical activity. CONCLUSION Objectively assessed SHS exposure was associated with poorer mental health among children.
Structure of aspartoacylase, the brain enzyme impaired in Canavan disease

Aspartoacylase catalyzes hydrolysis of N-acetyl-l-aspartate to aspartate and acetate in the vertebrate brain. Deficiency in this activity leads to spongiform degeneration of the white matter of the brain and is the established cause of Canavan disease, a fatal progressive leukodystrophy affecting young children. We present crystal structures of recombinant human and rat aspartoacylase refined to 2.8- and 1.8-Å resolution, respectively. The structures revealed that the N-terminal domain of aspartoacylase adopts a protein fold similar to that of zinc-dependent hydrolases related to carboxypeptidases A. The catalytic site of aspartoacylase shows close structural similarity to those of carboxypeptidases despite only 10–13% sequence identity between these proteins. About 100 C-terminal residues of aspartoacylase form a globular domain with a two-stranded β-sheet linker that wraps around the N-terminal domain. The long channel leading to the active site is formed by the interface of the N- and C-terminal domains. The C-terminal domain is positioned in a way that prevents productive binding of polypetides in the active site. The structures revealed that residues 158–164 may undergo a conformational change that results in opening and partial closing of the channel entrance. We hypothesize that the catalytic mechanism of aspartoacylase is closely analogous to that of carboxypeptidases. We identify residues involved in zinc coordination, and propose which residues may be involved in substrate binding and catalysis. The structures also provide a structural framework necessary for understanding the deleterious effects of many missense mutations of human aspartoacylase.
Selection of a nucleopolyhedrovirus isolate from Helicoverpa armigera as the basis for a biological insecticide.

BACKGROUND The cotton bollworm, Helicoverpa armigera, is an insect that causes damage in a wide range of crops in Spain. Seven isolates of H. armigera single nucleopolyhedrovirus (HearSNPV) from the Iberian Peninsula were subjected to molecular and biological characterization and compared with a Chinese genotype (HearSNPV-G4). RESULTS The estimated sizes of the Iberian genomes varied between 116.2 and 132.4 kb, compared to 131.4 kb of the HearSNPV-G4 reference genome. Phylogenetic analysis based on the lef-8, lef-9 and polh genes revealed that the Iberian strains were more closely related to one another than to other HearSNPV isolates. Occlusion body (OB) concentration-mortality responses (LC50 values) did not differ significantly among Iberian isolates when tested against a Helicoverpa armigera colony from Oxford (UK). Despite being the fastest killing isolate, HearSNPV-SP1 was as productive as isolates with lower virulence, with an average yield of 3.1 × 10(9) OBs larva(-1) . OBs of HearSNPV-SP1 and HearSNPV-G4 were similarly pathogenic against a recently established colony from southern Spain, although HearSNPV-SP1 was faster killing than HearSNPV-G4 against a range of instars. CONCLUSION The insecticidal properties of HearSNPV-SP1 mean that this strain is likely to prove useful as the basis for a biological insecticide for control of Helicoverpa armigera in Spain.
Noncoordinate regulation of epithelial Na channel and Na pump subunit mRNAs in kidney and colon by aldosterone.

Distal colon and renal cortical collecting ducts are major effectors of aldosterone-dependent Na homeostasis. Na is absorbed by entry through an apical amiloride-sensitive Na channel and extruded by Na-K-ATPase at the basolateral membrane. Using a ribonuclease protection assay, we studied, in vivo, aldosterone regulation of alpha-, beta-, gamma-subunits of the rat epithelial Na channel (rENaC) and alpha 1- and beta 1-subunits of Na-K-ATPase. In the kidney, Na-K-ATPase mRNAs were also assayed over discrete tubular segments by in situ hybridization. In rat colon, all three rENaC mRNAs were decreased by adrenalectomy, with a major effect on beta- and gamma-subunits, and were restored with 7 days, but not 2 days, of aldosterone treatment; in the kidney, however, only alpha-transcripts varied. Na-K-ATPase alpha 1- and beta 1-subunit mRNAs in both organs were not (in the case of the beta 1-subunit) or were mildly (in the case of the alpha 1-subunit) affected after adrenalectomy. Our conclusions are as follows: 1) Transcripts of rENaC and Na-K-ATPase subunits are not coordinately regulated by aldosterone in vivo; i.e., modulation involves mainly the Na channel, not Na-K-ATPase; the effect is not of comparable magnitude on each subunit mRNA and differs between tissues. 2) The delay of the aldosterone effect on transcripts is much longer than that required to restore normal Na transport in adrenalectomized rats, indicating that rENaC and Na-K-ATPase subunit transcript levels may depend on unidentified early aldosterone-induced proteins.