bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Features and Outcome of Children With Hereditary Spherocytosis
Authors: Kiliç Mehmet A. , Özdemir Gul N. , Tahtakesen Tuba N. , Gökçe Müge , Uysalol Ezgi P. , Bayram Cengiz , Ayçiçek Ali , Aydoğan Gönül 
Published: May 2021

Objective:
The aim of this study was to evaluate the demographics, clinical, and laboratory findings and treatment responses of patients with hereditary spherocytosis (HS).

Materials and Methods:
Data of children with HS were examined. Diagnosis was based on clinical history, physical examination, family history, presence of spherocytes on peripheral blood smear, and osmotic fragility test.

Results:
A total of 101 patients were included. The median (range) age at diagnosis was 38.0 (1 to 188) months. Mild, moderate, and severe forms of HS were present in 29 (28.7%), 15 (14.9%), and 57 (56.4%) patients, respectively. Family history was available in 73 patients and 56 of these (76.7%) had a positive family history for HS. Forty-five (44.5%) patients needed regular transfusions and all of these had severe disease. Although most patients did not require transfusion postsplenectomy, 2 of 45 (4.4%) patients continued to require transfusion. Transfusion dependence was significantly (P<0.001) higher in patients with severe spherocytosis.

Conclusions:
In HS, splenomegaly, pallor, and jaundice are the most common clinical features. Splenectomy dramatically reduces hemolysis in most cases and virtually abolishes further requirement for transfusion.
Clinical Utilities of Electrocardiography in the Diagnosis of Myocardial Ischemia in Children With Sickle Cell Anemia: Correlation With Serum Cardiac Troponin I
Authors: Adeniyi Adewuyi T. , Okeniyi John A.O. , Adegoke Samuel A. , Oseni Saheed B.A. , Smith Olufemi S. , Abe-Dada Adedolapo A. 
Published: May 2021

Background:
Sickle cell anemia (SCA) is associated with recurrent vaso-occlusive crisis (VOC) and the risk of myocardial ischemia (MI). This study investigated the utility of electrocardiography (ECG) and cardiac troponin I (cTnI) in diagnosing MI during VOC.

Materials and Methods:
Children with SCA 5 to 15 years of age in VOC (patients) and age-matched and sex-matched steady-state controls were studied. Their ECG and cTnI levels were measured at contact and after 4 to 6 weeks.

Results:
One hundred eighty-six children (93 patients and 93 controls) were studied. The mean (SD) ages of the patients and controls were 8.8 (3.2) and 9.0 (3.1) years, respectively. The mean MI score was significantly higher for the patients, 1.7 (1.2), than the controls, 1.3 (1.0), P=0.002. A significantly higher proportion of the patients, 18 (19.4%), also had significant ischemia compared with the controls, 8 (8.6%), P=0.016. The median (interquartile range) serum cTnI level was significantly higher in the patients than the controls, P=0.006. All 7 of the patients with elevated cTnI had VOC. No significant correlation was found between MI score and cTnI in both groups.

Conclusions:
cTnI is elevated and ECG features of MI worsen during VOC. Longitudinal studies to investigate their evolvement over time are advocated.
A 5-Year Retrospective Study on Infant Homicide Presenting as Cot Death
Authors: Tse Rexson , Ioelu Leilani , Chen Matthew , McCarthy Sinead , Glenn Charley , Kesha Kilak , Morrow Paul , Stables Simon 
Published: May 2021

Abstract
Being found dead in cot or bed is the most common presentation encountered with infant deaths. These deaths are often associated with unsafe sleep environment. The postmortem examination in such cases is often negative, and along with family objections, cultural, and other factors, may lead to a coroner considering not authorizing a postmortem examination. However, not identifying a homicide is a potential risk if a postmortem examination is not performed. This 5-year retrospective study investigated the proportion of infant homicide death initially presenting as cot death. Of a total of 103 cases of infants initially presenting as being found dead in cot or bed, 3 (approximately 3%) were classified as homicides after postmortem examination. During the study period, a total of 9 infant homicides were reported, translating to 33% (3 of 9) of infant homicides presented as cot deaths. Postmortem radiology was a valuable adjunct, but was unable to recognize 2 of the homicide cases with traumatic head injuries (with subdural hemorrhage and brain injury only). We strongly advocate that all infant deaths presenting as cot death require a full postmortem examination.
Left Heart Decompression on Veno-Arterial Extracorporeal Membrane Oxygenation in Children With Dilated Cardiomyopathy and Myocarditis: An Extracorporeal Life Support Organization Registry Review
Authors: Choudhury Tarif A. , Ofori-Amanfo George , Choi Jaeun , Eisenberg Ruth E. , Rycus Peter , Medar Shivanand S. , Aydin Scott I. 
Published: May 2021

OBJECTIVES:
To describe the association between left heart decompression on veno-arterial extracorporeal membrane oxygenation and survival in patients with myocarditis and dilated cardiomyopathy. The secondary outcome is to study association of left heart decompression with survival in children with myocarditis compared with those with dilated cardiomyopathy.

DESIGN:
Retrospective study of a multicenter registry database.

SETTING:
Data reported to Extracorporeal Life Support Organization from international extracorporeal membrane oxygenation centers.

PATIENTS:
Patients less than or equal to 18 years old with a diagnosis of myocarditis or dilated cardiomyopathy receiving extracorporeal membrane oxygenation.

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
A total of 1,438 pediatric extracorporeal membrane oxygenation runs were identified. Thirty-seven percent of the patients had myocarditis (n = 532), whereas the rest had dilated cardiomyopathy. Survival to hospital discharge was 63%. Median extracorporeal membrane oxygenation duration was 148 hours with interquartile range (84–248 hr). Nineteen percent of patients (n = 274) had left heart decompression. Multivariable analysis revealed using left heart decompression (adjusted odds ratio, 1.42; 95% CI, 1.06–1.89; p = 0.02), e-cardiopulmonary resuscitation (adjusted odds ratio, 0.63; 95% CI, 0.51–0.79; p < 0.001), higher pH (adjusted odds ratio, 3.69; 95% CI, 1.80–7.53; p < 0.001), and diagnosis of myocarditis (adjusted odds ratio, 1.69; 95% CI, 1.35–2.08; p < 0.001) were associated with greater odds of survival. In the multivariable analysis for patients with dilated cardiomyopathy, left heart decompression failed to reveal a significant association with survival (20% among survivors vs 17% among nonsurvivors, 95% CI, –2.2% to 8.0%). Meanwhile in patients with myocarditis, the multivariable analysis failed to exclude the possibility that left heart decompression was associated with up to a three-fold greater odds of survival (adjusted odds ratio, 1.77; 95% CI, 0.99–.15).

CONCLUSIONS:
Retrospective review of the Extracorporeal Life Support Organization registry revealed an association between left heart decompression and greater odds of survival in children with myocarditis and dilated cardiomyopathy on extracorporeal membrane oxygenation. When comparing patients with dilated cardiomyopathy against those with myocarditis, we could not exclude a three-fold greater odds of survival associated with the use of left heart decompression. This finding warrants further prospective evaluation.
Neurological Complications Acquired During Pediatric Critical Illness: Exploratory “Mixed Graphical Modeling” Analysis Using Serum Biomarker Levels
Authors: Raghu Vineet K. , Horvat Christopher M. , Kochanek Patrick M. , Fink Ericka L. , Clark Robert S. B. , Benos Panayiotis V. , Au Alicia K. 
Published: May 2021

OBJECTIVES:
Neurologic complications, consisting of the acute development of a neurologic disorder, that is, not present at admission but develops during the course of illness, can be difficult to detect in the PICU due to sedation, neuromuscular blockade, and young age. We evaluated the direct relationships of serum biomarkers and clinical variables to the development of neurologic complications. Analysis was performed using mixed graphical models, a machine learning approach that allows inference of cause-effect associations from continuous and discrete data.

DESIGN:
Secondary analysis of a previous prospective observational study.

SETTING:
PICU, single quaternary-care center.

PATIENTS:
Individuals admitted to the PICU, younger than18 years old, with intravascular access via an indwelling catheter.

INTERVENTIONS:
None.

MEASUREMENTS:
About 101 patients were included in this analysis. Serum (days 1–7) was analyzed for glial fibrillary acidic protein, ubiquitin C-terminal hydrolase-L1, and alpha-II spectrin breakdown product 150 utilizing enzyme-linked immunosorbent assays. Serum levels of neuron-specific enolase, myelin basic protein, and S100 calcium binding protein B used in these models were reported previously. Demographic data, use of selected clinical therapies, lengths of stay, and ancillary neurologic testing (head CT, brain MRI, and electroencephalogram) results were recorded. The Mixed Graphical Model-Fast-Causal Inference-Maximum algorithm was applied to the dataset.

MAIN RESULTS:
About 13 of 101 patients developed a neurologic complication during their critical illness. The mixed graphical model identified peak levels of the neuronal biomarker neuron-specific enolase and ubiquitin C-terminal hydrolase-L1, and the astrocyte biomarker glial fibrillary acidic protein to be the direct causal determinants for the development of a neurologic complication; in contrast, clinical variables including age, sex, length of stay, and primary neurologic diagnosis were not direct causal determinants.

CONCLUSIONS:
Graphical models that include biomarkers in addition to clinical data are promising methods to evaluate direct relationships in the development of neurologic complications in critically ill children. Future work is required to validate and refine these models further, to determine if they can be used to predict which patients are at risk for/or with early neurologic complications.
Cardiovascular Risk Assessment in Women With Rheumatoid Arthritis Through Carotid and Femoral Artery Doppler Ultrasound
Authors: Sacilotto Nathália de Carvalho , Augusto André Ozela , Lucena Dayrana Alves , Godoy Marcos Roberto , Almeida Rogério Duque de , Pernambuco Roberta de Almeida , Lima Sônia Maria Alvarenga Anti Loduca , Sacilotto Roberto , Giorgi Rina Dalva Neubarth 
Published: May 2021

Objective
The aims of this study were to assess cardiovascular risk in patients with rheumatoid arthritis (RA) classified as low-risk by the Framingham score, before and after the multiplication by 1.5 recommended by the European League Against Rheumatism (EULAR), and to stratify them using carotid and femoral Doppler ultrasound (DUS) in order to optimize the assessment of the asymptomatic cardiovascular disease incidence.

Methods
Thirty-five female patients with RA and 35 healthy women (control group), both with low cardiovascular risk by Framingham score, were enrolled in the study (randomized). All of them underwent carotid and femoral DUS studies.

Results
The mean age at diagnosis was 44.57 years, the mean disease duration was 12.11 years and the mean disease activity score according to the Disease Activity Score 28 was 1.91, whereas according to the Clinical Disease Activity Index, it was 6.176. The sample was homogeneous. Within the RA group, 46% showed changes in the carotid and/or femoral DUS, compared with 14% in the control group (p = 0.004). Of the DUS with abnormalities, in the RA group, 31% of the carotid DUS and 81% of the femoral DUS (p = 0.005) showed intima-media thickness and/or atherosclerotic plaques. After EULAR 1.5 multiplication factor, 66% remained low cardiovascular risk. Of these, 35% of the patients showed changes in the carotid and/or femoral DUS, compared with 14% of the control group (p = 0.07).

Conclusions
The EULAR criteria are effective at identifying patients with high cardiovascular risk. Carotid DUS and, especially, femoral DUS are tools that can be used in clinical practice as ways to detect CVD even in its asymptomatic form.
Comparison of Sound Touch Elastography, Sound Touch Quantify, and 4 Serum Fibrosis Indexes for the Diagnosis of Liver Fibrosis in Patients With Chronic Hepatitis B
Authors: Dong Bingtian , Huang Shu , Chang Jiandong , Chen Xiaoyan , Yan Jianping 
Published: May 2021

Abstract
The aim of this research was to compare the use of shear wave elastography (sound touch elastography [STE] and sound touch quantify [STQ]) and serum liver fibrosis indexes in the evaluation and staging of chronic hepatitis B (CHB) liver fibrosis. Sound touch elastography is a form of 2-dimensional shear wave elastography, and STQ is a form of point shear wave elastography. Between June 2018 and March 2019, 122 patients with CHB were assessed using STE and STQ. Serum liver biomarkers tests were undertaken, and liver biopsy was performed, and these were used to assign a pathological stage based on the Scheuer scoring system. A receiver operating characteristic curve was used to analyze the diagnostic value of noninvasive methods for evaluating and staging liver fibrosis. The cutoff values of STE for liver fibrosis stages S2 to S4 were 8.85, 9.97, and 10.29 kPa, respectively, and the areas under the receiver operating characteristic (AUCs) curve were 0.703, 0.821, and 0.900, respectively. The cutoff values of STQ for liver fibrosis stages S2 to S4 were 11.31, 13.81, and 20.60 kPa, respectively, and the AUCs were 0.674, 0.807, and 0.893, respectively. The AUCs of STE and STQ in diagnosing fibrosis stage were significantly higher than those of liver serum biomarkers (P < 0.05). The AUCs for the ability of the aspartate transaminase-to-platelet ratio index, the fibrosis index based on the 4 factors, the King score, and the Forns index to diagnose S2 fibrosis were 0.502, 0.624, 0.542, and 0.616, respectively, and the AUCs for their ability to diagnose S4 fibrosis were 0.856, 0.861, 0.883, and 0.823, respectively. Both STE and STQ are noninvasive methods for the assessment of liver fibrosis in CHB patients, with better diagnostic performances than those of 4 serum fibrosis indexes.
Real-Time Tissue Elastography to Evaluate Hepatic Hypoxic-Ischemic Injury Caused by Brain Death
Authors: Zhang Guoying , Tang Ying , Yu Huimin , Kong Weina , Chen Yun , Liu Yang , Zhao Jingwen 
Published: May 2021

Abstract
This study aimed to explore the potential of real-time tissue elastography (RTE) in evaluating hepatic hypoxic-ischemic injury caused by brain death. We performed RTE and biopsy for 50 donated liver. Hematoxylin-eosin staining was used to observe hepatocyte acidophilic change. Liver grafts were divided into 2 groups, one nonacidophilic change (n = 7) and the other with acidophilic change (n = 43). Correlation and difference analysis were performed for hematoxylin-eosin staining results and RTE parameters. The result indicated that 4 of the 11 RTE parameters, namely, the area of low strain within the region of interest (%AREA), contrast (CONT), inverse difference moment (IDM), and correlation (CORR) were related to hepatocytes acidophilic change (r = 0.284, P = 0.046; r = 0.349, P = 0.013; r = −0.444, P = 0.001; r = −0.381, P = 0.00). Whereas %AREA and CONT of the nonacidophilic change group were lower than that of the acidophilic change group (P < 0.05), IDM and CORR in nonacidophilic change group were higher than that of the acidophilic change group (P < 0.05); the remaining parameters were not statistically different between 2 groups (P > 0.05). Analysis of receiver operating characteristic curve indicated that the area under the curve of %AREA, CONT, IDM, and CORR were 0.75, 0.79, 0.81, and 0.77, respectively. Based on this, we concluded that the quantitative analysis parameters of RTE could preliminary assess hepatic hypoxic-ischemic injury caused by brain death.
Ultrasound Elastography in the Differentiation of Simple Cyst and Type I Hydatid Cyst of the Liver
Authors: Durmaz Fatma , Ozgokce Mesut , Turkoglu Saim , Dundar İlyas , Goya Cemil 
Published: May 2021

Abstract
Hydatid cyst (HC) is a parasitic disease that is endemic particularly to the sheep-breeding regions of the world, our country included. Given their propensity to materialize most commonly in the liver, our study evaluates the value of ultrasound elastography in the differential diagnosis of simple cyst and type I HCs of the liver.
The study involved a total of 73 cysts (22 simple cysts, 51 type I HCs) that were removed using the puncture aspiration injection reaspiration technique between 2016 and 2018. All measurements were made using a Siemens ACUSON S2000 (Siemens Healthcare, Erlangen, Germany) device with a 4-MHZ 4C1 convex probe, an acoustic radiation force impulse elastography procedure, and a Virtual Touch IQ option. The difference between the mean shearwave elastography (SWE) values of both groups was analyzed with an independent t test.
Of the 70 patients involved in the study, 29 were female and 41 were male, with a mean ± SD age of 38.85 ± 17.62 years (range, 5–82 years). Upon the examination of the 22 simple cysts and the 51 HCs, the mean ± SD SWE values were found to be 2.6 ± 0.96 (0.96–4.25) and 2.8 ± 1.69 (0.66–4.84), respectively. No statistically significant difference was identified between the SWE values of type I HCs and simple cysts (P > 0.005).
Although HCs and simple cysts had similar radiological appearances, and a relative difference was noted in the differentiation of the elastographic measurements, this difference was not statistically significant. Accordingly, more comprehensive and various studies are needed.
Minimal Dissection Direct Frontalis Muscle Advancement Flap for Congenital Ptosis Repair
Authors: Dallalzadeh Liane O. , Park Kathryn S. , Korn Bobby S. , Kikkawa Don O. , Liu Catherine Y. 
Published: May 2021

Background:
Frontalis flap advancement is an alternative means of congenital ptosis repair from frontalis suspension utilizing autologous fascia or allogenic implants. Variations in technique, including flap division, location and number of skin incisions, and dissection planes, are described in the literature.

Materials and Methods:
A retrospective case series of patients with congenital myogenic ptosis treated with simplified, minimal dissection frontalis flap advancement involving a single upper eyelid crease incision with dissection in the preseptal and subcutaneous planes without division to mobilize the frontalis flap. Inclusion criteria: pediatric patients age < 18 years with either primary or recurrent congenital ptosis following previous surgical repair. Exclusion criteria: ptosis of neurogenic etiology or postoperative follow-up < 3 months. Primary outcome measures were postoperative margin-reflex distance 1 (MRD1), the difference in MRD1 between surgical and nonsurgical eyelids in unilateral ptosis, and lagophthalmos. Secondary outcome measures included recurrence and complications.

Results:
Twenty-six patients met inclusion criteria, 24 males and 2 females. Mean postoperative MRD1 at last follow-up was 2.9 ± 1.2 mm and the mean difference in MRD1 between surgical and nonsurgical eyelids at last follow-up was 1.1 ± 1.0 mm. Average lagophthalmos at postoperative month 2 to 5 was 0.6 ± 0.7 mm. One patient demonstrated postoperative lid retraction that required re-operation. Four of 31 eyelids (12.9%) demonstrated ptosis recurrence at 5, 12, or 24 months.

Conclusions:
In this retrospective series, the authors report clinically and statistically significant surgical eyelid height improvement and symmetry between nonsurgical and surgical eyelids in congenital ptosis patients treated with minimal dissection direct frontalis flap advancement.
Impact of the Craniofacial Surgery Simulation in Anterior Plagiocephaly on Orbits and Oculomotor Muscles: Biomechanical Analysis With a Finite Element Model
Authors: Dalmas Florian , Thollon Lionel , Beylerian Marie , Godio Raboutet Yves , David Thierry , Scavarda Didier , Denis Danièle , Pech-Gourg Grégoire 
Published: May 2021

Purpose:
The aim of this study was to show the displacements and strain induced by the supraorbital band advancement during a craniofacial surgery for an anterior plagiocephaly on the orbital bones and the orbital content thanks to a numerical surgical simulation using the finite element method.

Methods:
A three-dimensional (3D) finite element model of a child with an anterior plagiocephaly was entirely created from a tomodensitometry of a patient followed by our Craniofacial Pediatric team. Data of the tomodensitometry were computed with Slicer 3D to re-create the orbit geometry. Mesh production, properties of the model, and simulations of the fronto-orbital advancement were conducted on Hyperworks software (Altair Engineering, Inc., Detroit, MI, USA).

Results:
The resulting 3D Finite Element Model was used to perform the supraorbital advancement simulation. Displacement and strain patterns were studied for orbital bones, oculomotor muscles, and eyeballs. Relative high strain in the both trochlear area and excycloration of the right orbit are among the most interesting results as torsional strabismus as V-pattern strabismus are often described in children with an anterior plagiocephaly.

Conclusions:
This pediatric Finite-Element Model of both orbits of a child with an anterior plagiocephaly showed the impact of the fronto-orbital advancement on the oculomotor system. This model described the relationship between the craniofacial surgery and the strabismus in the unilateral coronal synostosis. The advantages of this model are the many opportunities for improvement, including postoperative period and additional surgical procedures.
Establishment of a C57BL/6 Mandibular Critical-Size Bone Defect Model
Authors: Yu Fei , Liu Lu , Xia Lunguo , Fang Bing 
Published: May 2021

Purpose:The goal of this study was to identify bone defects of critical size in C57BL/6 mouse mandibles.Materials and Methods:Twenty-four male mice were included in this study. All mice underwent surgeries on their left mandibles. Mandibular defects of 1.0 mm (n = 8), 1.6 mm (n = 8), and 2.3 mm (n = 8) were created. For the investigation of bone healing after an 8-week period, micro-computed tomography scans and histomorphology were performed.Results:Mandibular bone nonunions were seen 0/8 in the 1.0-mm group, 6/8 in the 1.6-mm group, and 8/8 in the 2.3-mm group. The outcome of micro-computed tomography showed that, after 8 weeks, the bone mineral density and the bone volume to total volume ratio were significantly different among the 3 groups. The defect gaps in the nonunion 1.6- and 2.3-mm groups were filled with connective tissue, and no obvious bone formation was found. Additionally, in quantitative analysis, according to the new bone fill calculations, the percentages were 91.85% ± 8.03% in the 1.0-mm group, 59.84% ± 20.60% in the 1.6-mm group, and 15.36% ± 8.28% in the 2.3-mm group, which indicated statistically significantly lower defect healing in the 2.3-mm group.Conclusions:The creation of 2.3-mm mandibular defects produces osseous nonunion in C57BL/6 mice.