bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age
Authors: Drissi Farouk , Meurette Guillaume , Baayen Corine , Wyart Vincent , Cretolle Célia , Guinot Audrey , Podevin Guillaume , Lehur Paul-Antoine 
Published: February 2019

BACKGROUND:
Hirschsprung disease is a rare congenital disease typically requiring surgical treatment during childhood. Quality of life and social condition at adult age can be impaired by disease-specific sequelae.

OBJECTIVE:
This study aimed to assess the quality of life and social outcome of adult patients operated on for Hirschsprung disease during childhood.

DESIGN:
Patients operated on for Hirschsprung disease during childhood were identified and specific questionnaires were sent to them.

SETTINGS:
Data from 2 referral centers were used.

PATIENTS:
Patients who completed the questionnaires regarding quality of life and social condition were included.

MAIN OUTCOME MEASURES:
The Hirschsprung’s Disease and Anorectal Malformations Quality of Life disease-specific questionnaire (8 dimensions explored; each scored from 0 to 100 maximum score) and a sociodemographic questionnaire were sent to identified patients. Sociodemographic data were compared with those of the French general population.

RESULTS:
Thirty-four patients had Hirschsprung disease (men, 76%; mean age, 32 years) were included in the study. Mean total Hirschsprung’s Disease and Anorectal Malformations Quality of Life score was 611 of 800 (maximum score 800). The 2 most impaired dimensions were “physical symptoms” and “diarrhea” (62.9/100 and 73.6/100). Fecal continence was only marginally affected (mean score, 89/100). Patients with Hirschsprung disease achieved better educational levels than the French general population. Parental and marital status did not differ between the 2 groups.

LIMITATIONS:
This study had the limitations inherent to a retrospective study.

CONCLUSION:
The quality of life of adult patients with Hirschsprung disease sequelae is marginally impaired in this study. Despite the consequences of this congenital abnormality, the condition eventually achieved can be considered as satisfactory. See Video Abstract at http://links.lww.com/DCR/A917.
Dried Blood Spot Self-Sampling by Guardians of Children With Epilepsy Is Feasible: Comparison With Plasma for Multiple Antiepileptic Drugs
Authors: Linder Camilla , Neideman Mirja , Wide Katarina , von Euler Mia , Gustafsson Lars L. , Pohanka Anton 
Published: February 2019

Background:
Dried blood spot (DBS) is an attractive matrix alternative to plasma for the measurement of antiepileptic drug concentrations with the possibility of self-sampling at home. The aim of this study was to evaluate whether DBS concentrations from a children population could be used as an alternative to plasma concentrations in a clinical routine laboratory.

Methods:
Children with epilepsy using carbamazepine (CBZ), lamotrigine (LTG), levetiracetam (LEV), or valproic acid (VPA) had capillary blood collected for routine plasma analysis. DBS samples were collected by guardians or nurses, and the quality of sampling was compared between the groups. DBS samples were analyzed with liquid chromatography–tandem mass spectrometry methods and plasma samples with immunochemical methods. In the comparison between DBS and plasma concentrations, previously analyzed sample data were pooled with data in this study and resulted in 190 comparison pairs. A bioanalytical cross-validation according to European Medicines Agency was performed. Clinicians evaluated the results to understand if a DBS concentration was linked to a different clinical dose recommendation for the patient in comparison with plasma concentrations.

Results:
Comparison of DBS sample quality showed that 2.3% of the capillary DBS collected by guardians were rejected and 8.0% of the capillary DBS collected by nurses. For DBS, a conversion factor of 0.85 for CBZ and 1.65 for VPA was applied for the comparison with plasma. LTG and LEV results were directly comparable. In the cross-validation, 88% of CBZ, 75% of LTG, 74% of LEV, and 94% of VPA comparisons were within 20% of the difference of the mean, although LEV had a few major differences (+31% to −40%). In 4 of the 190 comparisons, the clinical evaluation indicated a risk of conflicting decisions regarding the need for dose adjustment when using DBS concentrations. However, the risk of negative patient outcomes was considered negligible.

Conclusions:
Our study demonstrates that a combination of bioanalytical cross-validation and clinical evaluation is an effective way to describe the applicability of DBS as an alternative to plasma, taking into account how therapeutic drug monitoring is used in specific patient groups. For LTG, converted CBZ and VPA, DBS is a feasible alternative for self-sampling at home. DBS for LEV can only be recommended for nonadherence queries due to the high variability of the plasma/DBS concentration ratios.
Organizational factors affecting successful physician–system integration: A qualitative study of Washington State health executives
Authors: Nguyen Ann M. , Johnson Christopher E. , Wood Suzanne J. , Dowling William L. 
Published: February 2019

Background
Health care delivery is moving toward a value-based environment, which calls for increased integration between physician groups and health systems. Health executives sit at a key nexus point for determining how and when physician–system integration occurs.

Purpose
The objective of this study was to identify the organizational factors that health executives perceived to have made physician–system integration successful.

Methodology/Approach
We used a multiple-case study research design. We conducted semistructured, qualitative interviews with 25 health executives in the roles of CEO, chief medical officer, chief financial officer and physician group chief executives from eight of Washington State's largest integrated delivery systems. To guide our analysis, we employed open systems theory and Porter's Value Chain to identify physician group and hospital factors that were integral to successful integration.

Results
Using the executives' perspectives, the factors grouped into three themes: (1) organizational structure—a mix of integration contracts united by common structural characteristics between physician groups and hospitals); (2) organizational culture—alignment of leadership between physician groups and hospitals; and (3) strategic resources—designated resources to establish and support care coordination activities.

Conclusion
Our work indicates that health systems should focus on the pathway to integration success through the alignment of structure (not just the integration contract), culture, and resources and not on an end goal of the physician employment model.

Practice Implications
Health system executives are key drivers for when and how physician groups are integrated into health services organizations. This article provides executives with an evidence-based model to aid in formulating integration approaches that combine elements of organizational structure, organizational culture, and strategic resources.
Clozapine Metabolism in East Asians and Caucasians
Authors: Ruan Can-Jun , Zang Yan-Nan , Wang Chuan-Yue , Cheng Yu-Hang , Sun Chuan , Spina Edoardo , de Leon Jose 
Published: February 2019

AbstractPurpose/BackgroundIn clozapine therapeutic drug monitoring (TDM) studies, Chinese reached the same concentrations using half the dosage Caucasians use. Defining clozapine poor metabolizers (PMs) requires stratification by ethnicity, smoking, and sex.Methods/ProceduresAfter sex and smoking stratification in 129 Chinese inpatients (mean, 8.8 TDM samples per patient), we explored the association between the total concentration-dose (C/D) ratio and CYP1A2 (*1C, *1F, and *7) and CYP2C19 alleles (*2 and *3). A systematic literature review identified 22 clozapine TDM prior studies (13 in Caucasians and 7 in East Asians).Findings/ResultsIn our Chinese sample, the mean total clozapine C/D ratio (ng/mL per mg/d) was 1.96 for 22 male smokers, 2.07 for 5 female smokers, 2.47 for 36 male nonsmokers, and 2.95 for 66 female nonsmokers. CYP1A2 *1C had no significant effects, and CYP1A2 *1F had small effects. Five clozapine PMs (4%) needed low clozapine doses of 75 to 115 mg/d to get therapeutic concentrations. Using the same methodology in a published Italian sample, we found 5 PMs (3.3% of 152). In the systematic review, the clozapine C/D ratio (ng/mL per mg/d) was higher when comparing: (1) weighted mean values of 1.57 in 876 East Asians versus 1.07 in 1147 Caucasians and (2) ranks of 8 East Asians versus 13 Caucasian samples (P< 0.001).Implications/ConclusionsFuture TDM studies need to further explore the frequency of clozapine PMs after sex and smoking stratification in East Asian and Caucasian patients. Compared with Caucasians, East Asians appear to have a clinically relevant decrease in clozapine clearance.
The Relationship Between Work Environment Satisfaction and Retention Intention Among Nursing Administrators in Taiwan
Authors: LIN Chiou-Fen , HUANG Chung-I , YANG Che-Ming , LU Meei-Shiow 
Published: February 2019

ABSTRACTBackgroundNursing administrators are essential to ensuring the quality of healthcare provided in hospitals. The nursing manpower shortage that has affected hospitals in Taiwan over the past decade has weighed particularly heavily on nursing administrators, who are expected to maintain high levels of nursing care quality in frequently understaffed healthcare settings.PurposeThe objective of this study was to explore the relationship between work environment satisfaction and nursing administrator retention in Taiwan.MethodsThis study used a cross-sectional, questionnaire-based survey to collect data from a sample population of nursing administrators. A set of indicators of quality nursing work environments was developed and included in the questionnaire. A total of 1,829 questionnaires were distributed, and the effective response rate was 95.57%.ResultsThe average overall rate of satisfaction with the current work environment across all domains was 3.59 (SD= 0.61). The highest level of satisfaction was found in the domain of safe practice environment (M= 3.83,SD= 0.70), and the lowest was found in the domain of informatics (M= 3.38,SD= 0.91). Length of administrative position tenure was significantly correlated with retention. Each of the eight domains significantly influenced retention. The domain of support and caring was the most significant predictor of nursing administrator retention.Conclusions/Implications for PracticeLength of administrator position tenure was significantly correlated with nursing administrator retention. Moreover, intention to stay among junior administrators was particularly affected by the support and caring domain. Therefore, it is recommended that nursing departments develop effective strategies to assist and encourage junior administrators to strengthen their career prospects and satisfaction.
Iatrogenic Inner Ear Dehiscence After Lateral Skull Base Surgery: Therapeutic Dilemma and Treatment Options
Authors: Bartholomew Ryan A. , Poe Dennis , Dunn Ian F. , Smith Timothy R. , Corrales Carleton Eduardo 
Published: February 2019

Objective:
To describe a series of patients with iatrogenic inner ear dehiscence after lateral skull base surgery and discuss treatment options.

Patients:
Three patients with history of recent lateral skull base surgery, symptoms consistent with inner ear dehiscence, and radiographically proven bony dehiscence on postoperative imaging.

Interventions:
All patients were initially managed conservatively with serial outpatient visits. Two patients with large cochlear and vestibular dehiscences had round window reinforcement procedures. One patient had transmastoid resurfacing for repair of an iatrogenic posterior semicircular canal dehiscence.

Main Outcome Measures:
Anatomical location of dehiscences; treatment options; subjective auditory and vestibular symptoms pre-dehiscence, post-dehiscence and after dehiscence repair; pre- and post-audiogram when available.

Results:
Patient ages were 46, 52, and 60 with two of three being women. None of the patients had subjective auditory or vestibular symptoms of inner ear dehiscence before initial skull base surgery, but they all had development of these symptoms afterwards. All patients were initially managed conservatively, but all ultimately required a surgical procedure. The two patients who elected for round window reinforcements, and the one patient who required transmastoid resurfacing, had significant improvement of symptoms.

Conclusions:
Iatrogenic inner ear dehiscence after skull base surgery is best dealt with and repaired intraoperatively. Should intraoperative repair not be possible, transcanal round window reinforcement is a minimally invasive option for medial otic capsule dehiscence, although long-term outcomes are unclear. For lateral otic capsule dehiscence, a transmastoid approach is recommended.

Level of Evidence: IV
Cochlear Implantation in Patients With Neurofibromatosis Type 2
Authors: Tolisano Anthony M. , Baumgart Bethany , Whitson Johanna , Kutz Joe Walter 
Published: February 2019

Objective:
To describe cochlear implantation (CI) outcomes in patients with neurofibromatosis type 2 (NF2).

Study Design:
Retrospective case series.

Setting:
A multidisciplinary NF2 clinic at a university hospital.

Patients/Interventions:
Patients with NF2 who underwent CI.

Main Outcome Measures:
Pre- and postimplantation audiometric data, including pure-tone average (PTA) and AzBio Sentence scores.

Results:
Eight patients with NF2 underwent CI. The mean age at implantation was 28.6 years (range: 17–63 yr) and six were female. The average length of deafness before CI was 3.5 years (range: 0.3–10 yr). Two patients underwent previous microsurgical resection via middle fossa craniotomy and one patient was treated with stereotactic radiotherapy before CI. Two tumors were growing at the time of CI, five tumors were not growing for an average period of 3.8 years (range: 1–6 yr), and one tumor had undergone previous gross total resection. Mean preoperative unaided PTA was 103.1 dB (range: 81.3–115 dB) and all preoperative AzBio scores were 0% in the ear to be implanted. These improved to a mean postoperative PTA of 30.9 dB (range: 12.5–43.8 dB) and mean postoperative AzBio score of 20% (range: 0–82%) with an average follow-up length of 16.6 months (range: 1.4–27.6 mo). Data logging demonstrated that six patients were daily CI users and two were nonusers, one of whom had normal hearing in the contralateral ear.

Conclusions:
CI is an effective option for rehabilitating hearing loss in patients with NF2; however, patients with normal contralateral hearing or poor follow-up do not perform as well.
Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio as Prognostic Hematologic Markers of Bell's Palsy: A Meta-analysis
Authors: Oya Ryohei , Takenaka Yukinori , Imai Takao , Sato Takashi , Oshima Kazuo , Ohta Yumi , Inohara Hidenori 
Published: February 2019

Objective:
Bell's palsy (BP) is the most common cause of unilateral peripheral facial paralysis, and inflammation has been proposed as the main pathological cause. The study aim was to investigate the relationship between hematologic inflammatory markers, including the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR), and BP.

Data Sources:
The following key words were used to search PubMed and Scopus for English language articles: Bell's palsy, facial palsy, facial paresis or facial paralysis, neutrophil, lymphocyte, and platelet.

Study Selection:
Articles related to BP with NLR or PLR data.

Data Extraction:
The data included patient profiles, House–Brackmann score, treatment modality, NLR, and PLR.

Data Synthesis:
Seven articles were selected. A random effect model was used to analyze the aggregated data. Six of these articles that included the NLR and two that included the PLR of BP and control patients were analyzed for the difference between BP and control patients. Three articles that included the NLR of the recovery and nonrecovery groups were analyzed for the relationship between NLR and recovery.

Conclusions:
The NLR was significantly higher for the BP patients than for the controls. Furthermore, the NLR was significantly lower for the recovery group than for the nonrecovery group. A high NLR was associated with poor prognosis and related to the severity of facial nerve inflammation. There was no significant difference between the PLRs of the BP patients and controls. The NLR, but not the PLR, was found to be a useful prognostic indicator of BP.
Efficacy of Different Endoscopic Stents in the Management of Postoperative Biliary Strictures
Authors: Yang Qin , Liu Jiaye , Ma Wenjie , Wang Junke , Li Fuyu , Bramer Wichor M. , Peppelenbosch Maikel P. , Pan Qiuwei 
Published: February 2019

Background:
Endoscopic stents are the first-line treatment in the management of benign biliary stricture (BBS) which include multiple plastic stents (MPSs), fully covered self-expandable metal stents (FCSEMS) including the conventional and modified ones (FCSEMS-C and FCSEMS-M) and biodegradable stents. However, different stents have their distinct advantages and disadvantages. We aim to conduct this systematic review to compare the efficacy of different stents in the management of BBS.

Methods:
Several databases were searched from inception through March 2018. Studies including >10 patients with postoperative stricture treated with endoscopic stents were enrolled. Pooled odds ratio of outcomes were calculated to compare MPS with FCSEMS. Weighted pooled rates were calculated to show the efficacy of FCSEMS-M, FCSEMS-C, and MPS.

Results:
Twenty-two articles were reviewed including 4 randomized controlled studies, 4 cohort studies, and 14 case series studies. Comparing FCSEMS with MPS, the pooled odds ratio was 0.48 [95% confidence interval (CI): 0.22-1.05] (P=0.07) for stricture resolution, 0.3 (95% CI: 0.1-0.92) (P=0.03) for adverse event, 1.9 (95% CI: 0.3-12) (P=0.49) for stent migration, and 1.38 (95% CI: 0.36-5.3) (P=0.34) for stricture recurrence. The pooled rates for stricture recurrence of MPS, FCSEMS-C, and FCSEMS-M were 19%, 19%, and 7%, respectively. The pooled rates for stent migration were 4% of MPS, 25% of FCSEMS-C, and 3% of FCSEMS-M.

Conclusions:
FCSEMS-M is more favorable in the management of BBS comparing with MPS or FCSEMS-C. The biodegradable stent may be a promising option but currently available data is insufficient to draw a firm conclusion.
The clinical usefulness of elastography in the evaluation of nonalcoholic fatty liver disease patients: A biopsy-controlled study
Authors: Darweesh Samar K. , Omar Heba , Medhat Eman , Abd-Al Aziz Rasha A. , Ayman Hedy , Saad Yasmin , Yosry Ayman 
Published: February 2019

Background
We aimed at determination of the usefulness of elastography [acoustic radiation force impulse (ARFI) and FibroScan] for evaluation of nonalcoholic fatty liver disease (NAFLD) patients.

Patients and methods
A prospective cross-sectional study included 60 biopsy-proven NAFLD patients (mean age: 45 years) was carried out. All patients were subjected to lab works, liver biopsy, and measurement of liver stiffness by ARFI and FibroScan and steatosis by controlled attenuation parameter (CAP). CAP measurements were adjusted for the presence of NAFLD and presence or absence of diabetes and according to BMI.

Results
Linear regression analysis showed that CAP is an independent predictor for significant hepatic steatosis (P<0.001). No significant difference was found in diagnostic accuracy between adjusted and nonadjusted CAP values for diagnosis of mild (>S1) or significant (>S2) hepatic steatosis (P=0.17 and 0.29 respectively). The median ARFI velocities for F1, F2, F3, and F4 were 0.92, 1.08, 1.07, and 2.58 m/s, respectively. Although there was an overall significant increase in ARFI values across the fibrosis grades (P<0.04), the difference in ARFI values was only significant between fibrosis grades F1 and F4 (P=0.02).

Conclusion
Elastography is a promising noninvasive tool for diagnosis and grading of hepatic steatosis and fibrosis in patients with NAFLD/nonalcoholic steatohepatitis with good sensitivity and specificity, especially in moderate to marked grades.
Treatment strategies for patients with decompensated liver cirrhosis due to hepatitis C virus infection eligible for liver transplantation: real-life data from five German transplant centers
Authors: Sandmann Lisa , Dörge Petra , Wranke Anika , Vermehren Johannes , Welzel Tania M. , Berg Christoph P. , Grottenthaler Julia M. , Weiss Karl-Heinz , Langel Jessica , Sterneck Martina , von Wulffen Moritz , Manns Michael P. , Wedemeyer Heiner , Hardtke Svenja , von Hahn Thomas 
Published: February 2019

Background
Even with highly effective direct-acting antivirals (DAAs) treatment of patients with decompensated hepatitis C (HCV) cirrhosis remains challenging. Clinical deterioration and the need for liver transplantation (LT) may arise despite previous antiviral treatment. It is unclear whether in patients with high Model for End-Stage Liver Disease (MELD) antiviral treatment is too risky and should thus be deferred until after LT. Treatment choices that are currently made in the real-world setting are unclear.

Methods
We performed a retrospective multicenter data analysis of patients with decompensated HCV cirrhosis (MELD ≥15) that presented to liver transplant centers that are part of the German Center for Infection Research when highly active DAA therapy was available. Choice of treatment strategy (DAA first vs. transplantation first) was analyzed and correlated with baseline and outcome parameters.

Results
Thirty-five patients fulfilled the inclusion criteria and their mean MELD score was 18.5±3.78 (median: 17, interquartile range=16–19). In the majority of patients (85.7%) DAA therapy was initiated before LT; survival rates and change in MELD were numerically better in this group compared with those where DAA therapy was withheld (82.1 vs. 40%, P=0.078; ΔMELD: −2.68±6.2 vs. 5.8±14.4, P=0.157). However, DAA treatment was more often initiated in patients with better liver function (MELD: 18±3.54 vs. 21.8±3.9, P=0.008). Three patients discontinued DAA treatment because of clinical deterioration; these patients all had a MELD score above 20 at the start of therapy.

Conclusion
At liver transplant centers in Germany DAA before LT is attempted in the majority of cases. It appears to be associated with an improved outcome and seems safe at least in individuals with MELD below or equal to 20.