bluuebunny/crossref_metadata_2025_split huggingface dataset:
Functional Alignment Within the Fusion in Adult Spinal Deformity (ASD) Improves Outcomes and Minimizes Mechanical Failures
Authors: Ani Fares , Ayres Ethan W. , Soroceanu Alex , Mundis Gregory M. , Smith Justin S. , Gum Jeffrey L. , Daniels Alan H. , Klineberg Eric O. , Ames Christopher P. , Bess Shay , Shaffrey Christopher I. , Schwab Frank J. , Lafage Virginie , Protopsaltis Themistocles S. ,   International Spine Study Group
Published: September 2023

Study Design.
Retrospective review of an adult deformity database.

Objective.
To identify pelvic incidence (PI) and age-appropriate physical function alignment targets using a component angle of T1-pelvic angle within the fusion to define correction and their relationship to proximal junctional kyphosis (PJK) and clinical outcomes.

Summary of Background Data.
In preoperative planning, a patient’s PI is often utilized to determine the alignment target. In a trend toward more patient-specific planning, age-specific alignment has been shown to reduce the risk of mechanical failures. PI and age have not been analyzed with respect to defining a functional alignment.

Methods.
A database of patients with operative adult spinal deformity was analyzed. Patients fused to the pelvis and upper-instrumented vertebrae above T11 were included. Alignment within the fusion correlated with clinical outcomes and PI. Short form 36-Physical Component Score (SF36-PCS) normative data and PI were used to compute functional alignment for each patient. Overcorrected, under-corrected, and functionally corrected groups were determined using T10-pelvic angle (T10PA).

Results.
In all, 1052 patients met the inclusion criteria. T10PA correlated with SF36-PCS and PI (R=0.601). At six weeks, 40.7% were functionally corrected, 39.4% were overcorrected, and 20.9% were under-corrected. The PJK incidence rate was 13.6%. Overcorrected patients had the highest PJK rate (18.1%) compared with functionally (11.3%) and under-corrected (9.5%) patients (P<0.05). Overcorrected patients had a trend toward more PJK revisions. All groups improved in HRQL; however, under-corrected patients had the worst 1-year SF36-PCS offset relative to normative patients of equivalent age (−8.1) versus functional (−6.1) and overcorrected (−4.5), P<0.05.

Conclusions.
T10PA was used to determine functional alignment, an alignment based on PI and age-appropriate physical function. Correcting patients to functional alignment produced improvements in clinical outcomes, with the lowest rates of PJK. This patient-specific approach to spinal alignment provides adult spinal deformity correction targets that can be used intraoperatively.
Utilization of Synthetic Human Angiotensin II for Catecholamine-Resistant Vasodilatory Shock in Critically Ill Children: A Single-Center Retrospective Case Series
Authors: Tezel Oguzhan , Hutson Tamara K. , Gist Katja M. , Chima Ranjit S. , Goldstein Stuart L. , Stanski Natalja L. 
Published: September 2023

OBJECTIVES:
To describe our institutional experience utilizing adjunctive synthetic angiotensin II in critically ill children with catecholamine-resistant vasodilatory shock (CRVS).

DESIGN:
Single-center, retrospective case series.

SETTING:
PICU and cardiac ICU (CICU) at a large, quaternary children’s hospital in the United States.

PATIENTS:
Twenty-three pediatric patients with CRVS who were prescribed synthetic angiotensin II at the discretion of bedside clinicians from January 2018 to April 2023.

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
Twenty-three patients (20 in PICU, 3 in CICU) with a median age of 10.4 years (interquartile range [IQR] 1.5–18.5) received angiotensin II over the study period, 70% of whom died. At the time of angiotensin II initiation, 17 patients (74%) were receiving one or more forms of extracorporeal therapy, and median Pediatric Logistic Organ Dysfunction-2 Score-2 in the prior 24 hours was 9 (IQR 7–11). The median time between initiation of the first vasoactive agent and angiotensin II was 127 hours (IQR 13–289), and the median total norepinephrine equivalent (NED) at initiation was 0.65 μg/kg/min (IQR 0.36–0.78). The median duration of therapy was 27 hours (IQR 4–68), and at each timepoint assessed, patients had median improvement in NED and mean arterial pressure (MAP) with treatment. Survivors initiated angiotensin II nearly 3 days earlier in vasoactive course (91.5 hr vs 161 hr, p = 0.23), and had both greater reduction in NED (–75% [IQR –96 to –50] vs +2.1% [IQR –55 to 33], p = 0.008) and greater increase in MAP (+15 mm Hg [IQR 10–27] vs –1.5 mm Hg [IQR –27 to 18], p = 0.052) at angiotensin II discontinuation.

CONCLUSIONS:
We demonstrate reduction in NED and improved MAP following initiation of angiotensin II in critically ill children with CRVS. Further prospective work is needed to examine optimal timing of angiotensin II initiation, appropriate patient selection, and safety in this population.
Threshold effect of systemic immune inflammatory index on hypertension in American adults (NHANES 2017–2020)
Authors: Shi Yumeng , Zhou Wei 
Published: September 2023

Background and aims:
Research reports on the relationship between systemic immune-inflammation index (SII) and hypertension are limited. We aim to evaluate the possible relationship between SII and hypertension.

Methods and results:
We conducted a cross-sectional analysis of adults from the National Nutrition and Health Database from 2017 to 2020. In this study, 13 742 participants were included. The exposure variable in this study was the systemic inflammatory state as defined by SII and an outcome was hypertension. There is a nonlinear association between SII and hypertension. Two piecewise logistic regression models were used to analyze the relationship between them and calculated the cut-off point of SII to be 501.2. We found that SII on the left side of the cut-off point has nothing to do with the prevalence of hypertension. However, in patients with SII at least 501.2, the prevalence of hypertension increased with the increase of SII (odds ratio, 3.13; 95% confidence interval, 2.04–4.81). The nonlinear association between LgSII and hypertension still existed stably in the subgroups of sex, age, BMI, diabetes, and cardiovascular disease (P for interaction > 0.05).

Conclusion:
A cross-sectional analysis reported the threshold effect of SII on and hypertension and calculated the turning point of SII to be 501.2.
Recurrent Vascularizing Keratitis in Infants With Hereditary Mucoepithelial Dysplasia Related to SREBF1 Mutation
Authors: Kim Seonghwan , Jeong Hyunchul , Ko Jung Min , Kwon Ohsang , Oh Joo Youn 
Published: September 2023

Purpose:
This study aims to present ophthalmic manifestations of 2 infants with hereditary mucoepithelial dysplasia (HMD) related to SREBF1 mutation over a 5-year period.

Methods:
Two female infants with an unremarkable perinatal history were evaluated for photophobia that had been manifest since 3 months after birth and diffuse scalp alopecia. Complete ocular examinations under anesthesia were performed, as well as genetic and systemic workup.

Results:
Both patients had vascularizing keratitis in both eyes, characterized by the growth of corneal new vessels from the 360 degrees periphery to the center and the formation of stromal leucomatous opacity at the leading edge. The keratitis partially regressed in response to topical corticosteroids and waxed and waned during the 5 years of follow-up. In addition, the loss of scalp hair developed in a cyclical pattern, causing diffuse scalp alopecia in the patients. Rheumatologic, nutritional, and developmental evaluations were within normal ranges. Whole-exome sequencing identified a heterozygous c.1669C>T (p.Arg557Cys) pathogenic variant in the SREBF1 gene associated with HMD in both patients.

Conclusions:
In pediatric patients with recurrent vascularizing keratitis and diffuse scalp alopecia starting early in life, HMD should be considered, and genetic tests and collaboration with dermatologists and pediatricians on the diagnosis should be provided.
Successful Management of Fungal Keratitis Caused by Multidrug-Resistant Cladosporium Species Using Povidone-Iodine
Authors: Ramatchandirane Balamurugan , Chandrika Yarra Mano , Najeeb Shaista 
Published: September 2023

Abstract:
We report a unique case of fungal keratitis, caused by the Cladosporium species, which is found to be resistant to multiple drugs, including voriconazole. Cladosporium belongs to the dematiaceous group of fungus, which is present ubiquitous in air, water, food etc. There are only few case reports available about Cladosporium keratitis which shows the successful management with voriconaozle. But in our case, the Cladosporium species was found to be resistant to multiple drugs, including voriconazole, and was successfully managed by topical povidone iodine (PVI) 5%.
Donor lymphocyte infusion for prophylaxis and treatment of relapse in pediatric hematologic malignancies after allogeneic hematopoietic stem cell transplant
Authors: Hou Ming-Hsin , Lee Chih-Ying , Ho Cheng-Yin , Yu Ting-Yen , Hung Giun-Yi , Huang Fang-Liang , Chiou Tzeon-Jye , Liu Chun-Yu , Yen Hsiu-Ju 
Published: September 2023

Background:
Donor lymphocyte infusion (DLI) is effective for managing patients with hematologic malignancies after allogeneic hematopoietic stem cell transplant (HSCT). However, few studies have explored its optimal use in pediatric populations. Herein, we report our single-center experiences of DLI and factors for predicting its outcomes.

Methods:
This retrospective study included pediatric patients who had received DLI (between June 1998 and December 2022) after allogeneic HSCT. Data regarding patient characteristics, preemptive DLI disease status, and DLI characteristics were collected. The primary outcomes were overall survival (OS), event-free survival (EFS), and graft-vs-host-disease (GVHD) development.

Results:
The study cohort comprised 17 patients with acute leukemia, 3 with chronic leukemia, and 3 with lymphoma. Prophylactic, preemptive, and therapeutic DLI were used in seven, seven, and nine patients, respectively. Patients’ median age and DLI dose were 9 years and 4.6 × 107 CD3+ cells/kg, respectively. The 5-year OS, EFS, and nonrelapse mortality were 43.5%, 38.3%, and 13.3%, respectively. Approximately 39% of the patients developed grade III or IV acute GVHD, whereas moderate/severe chronic GVHD (cGVHD) occurred in 30% of the evaluable patients. Patients’ disease status before HSCT (p = 0.009) and DLI (p = 0.018) were the key factors influencing EFS. The implementation of a dose escalation schedule was associated with a marginal reduction in the risk of moderate/severe cGVHD (p = 0.051). A DLI dose of ≥5 × 107 CD3+ cells/kg was significantly associated with a high moderate to severe cGVHD risk (p = 0.002) and reduced OS (p = 0.089).

Conclusion:
Patients’ disease status before HSCT and DLI may help predict EFS. The use of DLI as a prophylactic and preemptive modality leads to a favorable 5-year EFS. To safely deliver DLI in children, clinicians must maintain vigilant monitoring and prepare patients in advance when escalating the dose to ≥5 × 107 CD3+ cells/kg.
Translabyrinthine Craniotomy Is Associated with Higher Risk of Asymptomatic Dural Venous Sinus Thrombosis in Vestibular Schwannoma Resection
Authors: Elms Hunter L. , Straka David M. , Abouelella Dina K. , Francis Howard W. , Kaylie David M. , Cunningham Calhoun D. 
Published: September 2023

Abstract

Objective
Characterize the incidence, risk factors, and patient outcomes of dural venous sinus thrombosis identified on postoperative imaging after retrosigmoid or translabyrinthine craniotomy for vestibular schwannoma resection.

Study Design
Retrospective cohort study.

Setting
Single tertiary academic referral center.

Patients
Eighty-one patients 19 to 82 years of age with vestibular schwannomas, 58% female.

Interventions
Retrosigmoid or translabyrinthine craniotomy with postoperative magnetic resonance imaging/magnetic resonance venography.

Main Outcome Measures
Association between operative approach, age, sex, body mass index, tumor size, dominant sinus, operative time, laterality, and perioperative cerebrospinal fluid (CSF) leaks with rate of thrombosis.

Results
Translabyrinthine craniotomy was associated with the highest relative risk of thrombosis (odds ratios [OR] = 19.82, 95% confidence interval [CI] = 1.75–224, p = 0.007), followed by male sex (OR = 5.53, 95% CI = 1.63–18.8, p = 0.035). Other patient and demographic risk factors were not associated with increased rates of dural venous thrombosis, nor was there an association with postoperative CSF leak. 81% (25/31) of thrombi had resolved within 3 years of surgery.

Conclusions
Translabyrinthine approach and male sex most strongly predicted postoperative dural venous thrombosis after postauricular craniotomy for vestibular schwannoma resection.

Professional Practice Gap and Educational Need
Better understanding of risk factors and management of dural venous thrombosis after vestibular schwannoma surgery.

Learning Objective
Characterize clinically significant risk factors for dural venous thrombosis in vestibular schwannoma surgery.

Desired Result
Identification of patient and operative risk factors for dural venous thrombosis.

Level of Evidence
III.

Indicate IRB or IACUC
Exempt.
Feeding Tube Placement, Complications, and Treatment Responses in a Large Eosinophilic Esophagitis Patient Population
Authors: Borinsky Stephanie A. , Cameron Brenderia A. , Xue Zeyun , LaFata Sean , Kiran Akshatha , Ocampo Adolfo A. , McCallen Justin , Lee Christopher J. , Redd Walker D. , Cotton Cary C. , Eluri Swathi , Reed Craig C. , Dellon Evan S. 
Published: September 2023

Objectives:
Feeding tubes can provide a temporary or long-term solution for nutritional therapy. Little is known regarding the use of feeding tubes in patients with eosinophilic esophagitis (EoE). We sought to describe the characteristics and outcomes in EoE patients requiring tube feeding.

Methods:
This was a retrospective cohort study of EoE patients at a large tertiary care health system. Demographics, clinical characteristics, and endoscopic findings were extracted from medical records, and patients who had a feeding tube were identified. Patients with and without a feeding tube were compared. Details about the tube, complications, and treatment were extracted. Growth, global symptomatic, endoscopic, and histopathologic (<15 eos/hpf) responses were compared before and after the initiation of feeding tube therapy.

Results:
We identified 39 of 1216 EoE patients who had a feeding tube (3%). Feeding tube patients were younger (mean age 6.3 years), reported more vomiting, and had a lower total endoscopic reference score than non-feeding tube patients (P < 0.01 for all). Tubes were used for therapy for an average of 6.8 years, with most patients (95%) receiving both pharmacologic and formula treatment for EoE. An emergency department visit for a tube complication was required in 26%. Tube feeding improved body mass index z score (P < 0.01), symptomatic response (42%), endoscopic response (53%), and histologic response (71%).

Conclusions:
Among EoE patients, only a small subset required a feeding tube and predominantly were young children with failure to thrive. Feeding tubes significantly improved growth and, when used in combination with other treatments, led to reduced esophageal eosinophilic inflammation.
Expedited Laparoscopic Cholangiogram and Liver Biopsy in the Workup of Biliary Atresia
Authors: Lai Krista , Eldredge R. , Notrica David M. , Wadera Sheetal , Jamshidi Ramin , Lee Justin , Padilla Benjamin , Garvey Erin M. 
Published: September 2023

Purpose:
Recent studies demonstrate the success of Kasai portoenterostomy for biliary atresia (BA) is linearly related to infant age at time of Kasai. We sought to review the feasibility and safety of laparoscopic needle micropuncture cholangiogram with concurrent core liver biopsy (if needed) for expedited exclusion of BA in patients with direct conjugated hyperbilirubinemia.

Methods:
Expedited laparoscopic cholangiogram and liver biopsy were instituted at our facility for infants with direct hyperbilirubinemia for whom clinical exam and laboratory workup failed to diagnose. A retrospective chart review was performed in infants <1 year with hyperbilirubinemia from 2016 to 2021. Demographics, preoperative evaluation, procedure details, and complications were reviewed.

Results:
Two hundred ninety-seven infants with unspecified jaundice were identified, of which, 86 (29%) required liver biopsy. Forty-seven percutaneous liver biopsies were obtained including 8 (17%) in whom BA could not be excluded. Laparoscopic cholangiogram was attempted in 47 infants following basic workup; BA was diagnosed in 22 infants (47%) of which 3 were <18 days old. Biliary patency was demonstrated laparoscopically in 22 of 25 (88%); 3 (12%) required conversion to open cholangiogram. Infants with percutaneous liver biopsy had an average delay of 3 days (range: 2–36) to cholangiogram. Preoperative studies and liver biopsy alone did not reliably exclude the diagnosis of BA.

Conclusion:
Laparoscopic cholangiogram with liver biopsy is a safe procedure resulting in the confirmation or exclusion of BA in infants. Forty-seven percent of infants who underwent laparoscopic cholangiogram were found to have BA; those who were surgical candidates underwent Kasai during the same operation.
Supratentorial extra-axial anaplastic ependymoma: a rare case report
Authors: Osman Abukar Mahamed , Çiftçi Ulvi , Sever Nurten 
Published: September 2023

Introduction and importance:
Ependymomas are central nervous system tumors arising from the ependymal lining of the ventricle and spinal cord. Supratentorial extra-axial ependymomas are very rare, most commonly affecting the pediatric population and rarely in adults.

Case presentation:
The authors report a case of a 71-year-old female with a headache and blurred vision. An MRI scan revealed a lesion at the parafalcine region of the occipital lobe. A parieto-occipital craniotomy was performed. When the dura was opened during the operation, the extra-axially located, well-circumscribed, dirty yellow-white tumor dissected from the surrounding tissue was excised entirely by microdissection. Histopathological examination revealed supratentorial extra-axial anaplastic ependymoma. The patient received postoperative radiation therapy (54 Gray over 30 fractions). No recurrence of the tumor was observed during the 4-year follow-up.

Discussion:
Supratentorial ependymomas at the extra-axial region are uncommon; extra-axial anaplastic ependymoma and meningiomas have similar radiological findings, such as a dural tail, subarachnoid plane, and diffuse enhancement after contrast injection. This close similarity might cause misdiagnoses. Total surgical resection was followed by adjuvant radiotherapy and close follow-up in the gold standard of the treatment.

Conclusion:
The authors report a rare case of anaplastic ependymomas located at the extra-axial region. Anaplastic tumors are prone to recurrence despite total resection and radiation therapy; hence, a close follow-up is warranted.
Melatonin versus chloral hydrate on sleep induction for recording electroencephalography in children: a randomized clinical trial
Authors: Fazli Bahareh , Hosseini Seyed-Ahmad , Behnampour Nasser , Langari Alale , Habibi-koolaee Mahdi 
Published: September 2023

Background:
Electroencephalography (EEG) plays an essential role in the diagnosis of seizures. EEG recording in children is done with partial sleep deprivation and sedative drugs. To compare the effectiveness of melatonin and chloral hydrate on sleep induction and EEG recording in children.

Materials and methods:
In a parallel blinded randomized clinical trial study, 78 patients (6 months–5 years) were included to record EEG. Patients were randomly divided into two groups to receive melatonin (0.4 mg/kg) or chloral hydrate (0.5 ml/kg). After receiving the sedative drug, the start and duration of sedation, recovery time, side effects, and epileptiform waves in the EEG were recorded. The data was analyzed using SPSS version 16, and the significance level was determined to be less than 0.05.

Results:
A total of 78 children, including 34 girls (43.6%) and 44 boys (56.4%) (average age of 27.15±17.15 months), were examined. Success in the induction of sedation was reported by melatonin in 36 patients (92%) and chloral hydrate in 37 patients (95%), which was similar between the two drugs (P=0.5). The start time (P=0.134) and the duration of sedation (P=0.408) were alike between the two drugs. However, compared to the chloral hydrate, the recovery time in the melatonin group was significantly shorter (P<0.001). Side effects were not seen in melatonin, while six children (15%) using chloral hydrate had mild side effects (P=0.013). Epileptiform waves in EEGs were reported to be similar and positive for melatonin in 18 children (50%) and chloral hydrate in 16 children (43%) (P=0.410).

Conclusion:
The findings show that using melatonin in the dose prescribed in this study had similar effects to success in inducing sedation with the minimum quantity of chloral hydrate. Regardless of the start time and duration of sedation, the shorter recovery time and the absence of side effects are the advantages of using melatonin.
Preoperative echocardiography in patients with hip fracture: a prospective cohort study
Authors: Rostagno Carlo , Tozzetti Camilla 
Published: September 2023

Introduction:
Although clinical guidelines suggest echocardiography before surgery in patients with hip fracture, no prospective studies exist in the literature. The aim of the present investigation was to assess whether preoperative echocardiography affects time to surgery, length of hospital stay (LOS), and in hospital mortality in high-risk patients who need surgery for a hip fracture.

Material and methods:
In the study entered 255 consecutive patients with hip fracture referred to a multidisciplinary hip fracture unit. Seven hundred seventeen patients referred before the implementation of routine echocardiographic examination were considered as control group. Echocardiography was performed in patients with systolic murmurs, unstable clinical conditions, recent decompensation of heart failure or hospital admission for coronary disease. Time to surgery, LOS, and in hospital mortality in patients who underwent preoperative echocardiography (high-risk group) were compared with patients who did not undergo echo (low-risk group) and with the control group.

Results:
122/255 patients underwent echocardiography. The two groups did not differ for age, sex, type or fracture, and notably time to surgery and LOS. Coronary heart disease, atrial fibrillation, and two or more comorbidities were more frequent in the echo group. Overall clinical characteristics were not different from group of patients referred before the implementation of the echocardiographic protocol but in these last patients time to surgery and LOS were significantly longer. As well in hospital mortality was not significantly different in the two groups (1.9 in echo vs. 1.7% in nonecho) but lower than in the control group (4.1% ).

Conclusions:
An echocardiographic guide may be useful in a high-risk group with a hip fracture to choose the proper anesthetic setting, referral for intensive care observation after surgery and, optimal medical treatment, including fluid management without delay in time to surgery.