bluuebunny/crossref_metadata_2025_split huggingface dataset:
Calorie Intake During Status Epilepticus and Outcome: A 5-Year Cohort Study
Authors: Rybitschka Anja , Semmlack Saskia , Kaplan Peter W. , De Marchis Gian Marco , Rüegg Stephan , Marsch Stephan , Sutter Raoul 
Published: May 2019

Objectives:Recommendations regarding nutrition during status epilepticus are lacking, and it is unclear whether restriction of calorie intake would result in beneficial effects or potential harm. We thus aimed to investigate associations between daily calorie intake and outcome in adult status epilepticus patients deriving from a 5-year cohort with a systematic and prospective collection of nutritional data.Design:Retrospective observational study.Setting:Medical ICUs at a tertiary academic medical care center.Patients:Consecutive patients with status epilepticus treated at the ICUs from 2012 to 2016 were included.Interventions:None.Measurements and Main Results:All patients with status epilepticus were monitored regarding nutrition support provided according to the guidelines. Relative risks of no return to baseline were estimated by Poisson regression with robust error variance and adjusted for potential confounders. Of 203 patients, 86 (42%) had return to baseline. Metabolic characteristics of patients with and without return to baseline did not differ. Patients without return to baseline received more calories and proteins per status epilepticus day, and increasing nutritional support was associated with ventilator-associated pneumonia (relative risk, 1.19; 95% CI, 1.09–1.28). Multivariable regression analysis revealed significant increases in relative risks for no return to baseline with every percent of days with nutrition (relative risk, 1.35; 95% CI, 1.05–1.74), with every 100 kcal (relative risk, 1.01; 95% CI, 1.002–1.01), and gram of protein intake (relative risk, 1.01; 95% CI, 1.001–1.01) per status epilepticus day, independent of potential confounders (including fatal etiology, duration and severity of status epilepticus, Charlson comorbidity index, and treatment with anesthetics).Conclusions:Our results indicate that increased calorie intake during status epilepticus is independently associated with unfavorable outcome. These findings require further validation and investigations into potential mediators, such as induction of ketogenesis, immunomodulating effects, and/or reduction of ICU-associated complications, such as infections.
Non-GNE Quadriceps Sparing Distal Myopathy in an Iranian Jewish Patient
Authors: Mishra Shri K. , Valles-Ayoub Yadira , Darvish Daniel , Khosa Shaweta , Moheb Negar , Trikamji Bhavesh , Rafiei Nastaran , Freundlich Robert 
Published: May 2019

Abstract
GNE myopathy is an autosomal-recessive distal myopathy. It is caused by a hypomorphic GNE gene, encoding the rate-limiting enzyme in sialic acid synthesis. This myopathy is prevalent in the Iranian Jewish (IJ) descendants because of a founder mutation GNE: p. M712T. We report a 52-year-old IJ woman who presented with a 20-year history of progressive distal muscle weakness. Physical examination and magnetic resonance imaging revealed lower-extremity weakness and atrophy. Electromyography confirmed myopathy. Genetic testing showed no mutations on the GNE gene. Muscle histochemistry demonstrated no rimmed vacuoles. The analysis of polysialylated neural cell adhesion molecule Western blot pattern was negative. Non-GNE myopathy with quadriceps sparing presentation has been previously described in a few cases of non-IJ descents. To the best of our knowledge, this is the first case of an IJ patient, presenting with quadriceps sparing myopathy, without associated GNE mutations and/or tubule-filamentous inclusions.
Is There an Association Between Prophylactic Femur Stabilization and Survival in Patients with Metastatic Bone Disease?
Authors: Philipp Travis C. , Mikula Jacob D. , Doung Yee-Cheen , Gundle Kenneth R. 
Published: May 2019

Abstract

Background
The femur is the most common site of metastasis in the appendicular skeleton, and metastatic bone disease negatively influences quality of life. Orthopaedic surgeons are often faced with deciding whether to prophylactically stabilize an impending fracture, and it is unclear if prophylactic fixation increases the likelihood of survival.

Questions/purposes
Is prophylactic femur stabilization in patients with metastatic disease associated with different overall survival than fixation of a complete pathologic fracture?

Methods
We performed a retrospective, comparative study using the national Veterans Administration database. All patient records from September 30, 2010 to October 1, 2015 were queried. Only nonarthroplasty procedures were included. The final study sample included 950 patients (94% males); 362 (38%) received prophylactic stabilization of a femoral lesion, and 588 patients (62%) underwent fixation of a pathologic femur fracture. Mean followup duration was 2 years (range, 0-7 years). We created prophylactic stabilization and pathologic fracture fixation groups for comparison using Common Procedural Terminology and ICD-9 codes. The primary endpoint of the analysis was overall survival. Univariate survival was estimated using the Kaplan-Meier method; between-group differences were compared using the log-rank test. Covariate data were used to create a multivariate Cox proportional hazards model for survival to adjust for confounders in the two groups, including Gagne comorbidity score and cancer type.

Results
After adjusting for comorbidities and cancer type, we found that patients treated with prophylactic stabilization had a lower risk of death than did patients treated for pathologic femur fracture (hazard ratio = 0.75, 95% CI, 0.62–0.89; p = 0.002).

Conclusions
In the national Veterans Administration database, we found greater overall survival between patients undergoing prophylactic stabilization of metastatic femoral lesions and those with fixation of complete pathologic fractures. We could not determine the cause of this association, and it is possible, if not likely, that patients treated for fracture had more aggressive disease causing the fracture than did those undergoing prophylactic stabilization. Currently, most orthopaedic surgeons who treat pathological fractures stabilize the fracture prophylactically when reasonable to do so. We may be improving survival in addition to preventing a pathological fracture; further study is needed to determine whether the association is cause-and-effect and whether additional efforts to identify and treat at-risk lesions improves patient outcomes.

Level of Evidence
Level III, therapeutic study.
Executive Function Profiles at Home and at School in 11-Year-Old Very Low Birth Weight or Very Low Gestational Age Children
Authors: Nyman Anna , Munck Petriina , Koivisto Mari , Hagelstam Camilla , Korhonen Tapio , Lehtonen Liisa , Haataja Leena ,   on behalf of the PIPARI Study Group
Published: May 2019

ABSTRACT:

Objective:
Executive function (EF) problems of children born at very low birth weight (VLBW; ≤1500 g) or very low gestational age (VLGA; <32 gestational weeks) may present differently at school compared to the home environment. Ecological assessment of EF including parent- and teacher-rated profiles and associated risk factors of 11-year-old children born at VLBW or VLGA was evaluated.

Methods:
A total of 125 VLBW or VLGA children and 132 controls were assessed using the Behavior Rating Inventory of EF, which includes 8 subscales that form the Behavioral Regulation and Metacognition Indexes. For VLBW or VLGA children, full-scale intelligence quotient (IQ) was assessed using the Wechsler Intelligence scale for Children, Fourth Edition. Neonatal data were collected systematically.

Results:
VLBW or VLGA children with full-scale IQ ≥ 70 had clinically significant problems in the Working Memory subscale at school. Although they had clinically significant problems at home in the Behavioral Regulation Index, the difference disappeared when adjusted for paternal education. Lower gestational age, lower birth weight z-score, surgical necrotizing enterocolitis, low paternal and maternal education, and lower full-scale IQ were identified to be risk factors for higher scores in ecological assessment of EF.

Conclusion:
VLBW or VLGA children in this cohort exhibit fewer EF problems in ecological assessment of EF compared to previous literature. EF problems of this study population vary by home and school setting and are emphasized in working memory at school. Screening for EF problems in school environment is recommended to target the support.
Optimizing surgical management of patients who decline blood transfusion
Authors: Han Esther S. , Arora Chetna , Kim Jin Hee , Hur Hye-Chun , Advincula Arnold P. 
Published: May 2019

Purpose of review
This review highlights the complexity of caring for gynecologic patients who refuse blood transfusion and discusses the importance of early, targeted perioperative and intraoperative medical optimization. We review alternative interventions and the importance of medical management to minimize blood loss and maximize hematopoiesis, particularly in gynecologic patients who may have significant uterine bleeding. The review also focuses on intraoperative interventions and surgical techniques to prevent and control surgical blood loss.

Recent findings
With improvements in surgical technique, greater availability of minimally invasive surgery, and increased use of preop UAE and cell salvage, definitive surgical management can be safely performed. New technologies have been developed that allow for safer surgeries or alternatives to traditional surgical procedures. Many medical therapies have been shown to decrease blood loss and improve surgical outcomes. Nonsurgical interventions have also been developed for use as adjuncts or alternatives to surgery.

Summary
The care of a patient who declines blood transfusion may be complex, but gynecologic surgeons can safely and successfully offer a wide variety of therapies depending on the patient's goals and needs. Medical management should be implemented early. A multidisciplinary team should be mobilized to provide comprehensive and patient-centered care.
What makes health services usable?: Insights from a qualitative study of caregivers of children with disabilities
Authors: Hilligoss Brian , Tanenbaum Sandra J. , Paul Marika H. , Ferrari Renée M. , Song Paula H. 
Published: May 2019

Background
The concept of usability from the field of user-centered design addresses the extent to which a system is easy to use, including under extreme conditions. Apart from applications to technologies, however, little attention has been given to understanding what shapes usability of health services more generally. Health service usability may impact the extent to which patients avail themselves of and benefit from those services.

Purpose
The aim of the study was to develop the concept of usability as it applies to health services, particularly for a high-need, complex patient population.

Approach
We conducted interviews and focus groups with 66 caregivers of children with disabilities and analyzed data through inductive coding and constant comparison.

Results
We find that before health services can be rendered usable for patients with complex health conditions, work is often required to develop trusting relationships with individual providers and to manage time demands and attendant challenges of physical access. In addition, our findings show that actions crucial to receiving benefits from one service often entail difficult tradeoffs either with other services or with other important features in the patient’s life-world. Finally, we propose the concept of configuration to capture the complex interdependent arrangement of connections to multiple health services, often for multiple household members, and other life-world factors (e.g., employment, transportation, living conditions). These configurations are dynamic, fragile, and vulnerable to shocks—events that destabilize them, often negatively impacting the relative usability of services and of the entire configuration. Collectively, these findings illustrate health service usability as a relational, situated, emergent property rather than an inherent feature of the service itself.

Practice Implications
System-centered design perspectives produce services that are usable for the mythical “ideal” user. To be truly “patient centered,” designs must “decenter” the health service and recognize it as one component of the patient’s life-world configuration.
Mini-Descemet Membrane Endothelial Keratoplasty for the Early Treatment of Acute Corneal Hydrops in Keratoconus
Authors: Bachmann Björn , Händel Alexander , Siebelmann Sebastian , Matthaei Mario , Cursiefen Claus 
Published: May 2019

Purpose:
To describe a new surgical option for the treatment of acute corneal hydrops in keratoconus and to present the first results.

Methods:
This is a retrospective analysis of 3 patients who presented to our clinic with a massive corneal hydrops in acute keratoconus and were treated by mini-Descemet membrane endothelial keratoplasty (DMEK). According to the size and the shape of the gap in the patient's Descemet membrane (DM), 1 DMEK graft was trephined with a round 5-mm punch. The other grafts were trimmed with a razor blade to a width of about 3 mm and a length adjusted to the length of the defect of the recipients' DM. The graft was inserted with a regular intraocular lens shooter. Correct unfolding of the graft was controlled by using intraoperative optical coherence tomography. At the end of the surgery, the graft was attached to the posterior corneal surface by a small air bubble. Thereafter, the complete anterior chamber was filled with 20% SF6 gas.

Results:
All 3 patients (age 32 ± 3 years on average) showed a rapid increase in uncorrected visual acuity from the logarithm of the minimum angle of resolution (LogMAR) 1.66 (±0.46) before mini-DMEK to the LogMAR 1.2 (±0.3) within 6 to 8 weeks after mini-DMEK. The thickest corneal point within the edematous cornea decreased in all 3 patients (1088 ± 280 μm before surgery vs. 630 ± 38 μm 1 week after surgery). One mini-DMEK failed in a first attempt. In this patient, the recipient DM was under strong tension and showed a pronounced dehiscence. Therefore, a small part of the recipient's DM around the preexisting gap in DM was removed before a second mini-DMEK graft was placed successfully. The other 2 patients developed partial graft detachment within 1 to 2 weeks after surgery. However, the corneas of these patients were dehydrated to physiological levels after mini-DMEK, and despite partial detachment, there was no relapse of the hydrops.

Conclusions:
Mini-DMEK could be helpful in patients with larger defects and detachments of DM in very ectatic corneas in the acute phase of corneal hydrops in acute keratoconus. These patients may not be successfully treated by intracameral gas application alone or in combination with pre-Descemetal sutures. Further investigations are needed to identify factors helping to decide on the best surgical approach in hydrops in acute keratoconus.
Effect of Scleral Lenses on Corneal Topography in Keratoconus: A Case Series of Cross-Linked Versus Non–Cross-Linked Eyes
Authors: Severinsky Boris , Fadel Daddi , Davelman Jenya , Moulton Eric 
Published: May 2019

Purpose:
To evaluate the changes in anterior corneal topography induced by short-time wear of scleral contact lenses (SLs) in keratoconic subjects with and without a history of corneal cross-linking (CXL).

Methods:
Nine keratoconic patients (14 eyes) were fitted with 18.5 mm SLs for optical rehabilitation. Subjects were divided into 2 groups: 7 eyes without a history of CXL (Non-CXL group) and 7 with a history of CXL (CXL group). Corneal topography was performed at baseline and after 2 and 5 hours of lens wear. The differences for simulated flat (Kflat), steep (Ksteep) and maximal (Kmax) corneal curvatures, central corneal astigmatism (CCA), and central cornea thickness were evaluated.

Results:
No statistically significant difference was detected between Non-CXL and CXL groups in any of these measures. Statistically significant flattening was detected in Ksteep Repeated measures analysis of variance ([RM-ANOVA), F (2,24) = 11.32, P < 0.0001], CCA [RM-ANOVA, F (2,24) = 15.34, P < 0.0001], and Kmax [RM-ANOVA, F (2,24) = 19.10, P < 0.0001). From baseline to 5 hours of SL wear, Ksteep decreased on average from 53.1 to 52.4 D, Kmax decreased from 56.7 to 55.8 D, and CCA decreased from 7.2 to 6.3 D. Kmax showed a trend toward more flattening in the Non-CXL group. Central cornea thickness showed significant thickening over time from baseline (451 μm) to 5 hours (458 μm) of SL wear [RM-ANOVA, F (1,12) = 319.3, P < 0.0001].

Conclusions:
Short-term scleral lens wear in keratoconic patients may cause flattening of the anterior cornea. A history of CXL treatment does not guarantee corneal shape stability after scleral lens wear. Practitioners should be aware of these changes because scleral lens wear may mask the signs of keratoconus progression.
Progressive High Hypermetropic Shift as a Refractive Surprise Following Glaucoma Filtration Surgery in a Phakic Child With Early-Onset Childhood Glaucoma Associated With Axenfeld-Rieger Anomaly
Authors: Kumar Praveen , Senthil Sirisha 
Published: May 2019

Purpose:
We report a case of progressive high hypermetropia following glaucoma filtration surgery in a child with Axenfeld-Rieger syndrome and congenital glaucoma.

Methods/Results:
We report a case of a 4-day-old female child presented as a case of Axenfeld-Rieger anomaly with secondary glaucoma in both eyes (OU), who underwent combined trabeculotomy with trabeculectomy in OU at the age of 3 weeks. On postoperative third month, cornea cleared and posterior embryotoxon was noted in OU with Habb’s striae in OS. Intraocular pressure (IOP) was controlled and fundus was normal in OU. Refraction was +2.00 D sph. in OD and 3.00D sph. in OS and was observed. On postoperative eighth month, IOP was controlled in OU, whereas retinoscopy showed refraction of +4.00D sph./−2.00D cyl at 30 degrees in OD and +10.00 D sph/−3.00 cyl at 120 degrees in OS, glasses were prescribed and was asked to review. After 4 years, the patient presented with blurring of vision, the best-corrected visual acuity noted was 20/100 in OD and 20/320 in OS. IOP was 28 and 14 mm Hg in OD and OS, respectively, but with healthy optic disc in OU. Refractive error had increased and was +5.50D sph in OD and +14.00D sph/−5.00 D cyl at 90 degrees in OS. Corneal topography showed cornea plana in OU, which was more in OS (K1: 32.6D and K2: 38.9D) compared with OD (K1: 38.7D and K2: 40.1D). The patient was started on glaucoma medications in OD. Four months later, IOP was controlled and refraction was stable in OU.

Conclusions:
Present case is the first to describe the unusual presentation of progressive high hypermetropia in a child with Axenfeld- Rieger anomaly with congenital glaucoma after surgical intervention for glaucoma. Childhood glaucoma is classically associated with myopic shift in refraction and refraction is one of the most important clinical parameter measured at every follow-up visit. Although progressive hypermetropic shift is a rare occurrence, clinicians should be aware of this possibility. Keratomerty should be performed when such refractive surprises arise, which may help detect the clinical condition and the etiology for such presentation.
Neglected Congenital Glaucoma With Lens Coloboma
Authors: Dhingra Deepika , Tekchandani Uday , Pandav Surinder S. , Kaushik Sushmita 
Published: May 2019

Purpose:
To report a case of lens coloboma in a case of neglected primary congenital glaucoma.

Materials and Methods:
A 5-year-old boy was brought by the parents with complaints of diminution of vision in both eyes noticed for 8 months. There was a history of enlargement of eyes since 1 year of age.

Results:
Clinical examination revealed bilateral large eyes with limbal stretching and Haab striae and lens coloboma in the right eye. Dilated examination revealed scalloped border of the crystalline lens superotemporally with broken zonules and lens coloboma in inferotemporal quadrant with absent zonules. There was advanced optic nerve head cupping in both eyes. This lens coloboma is likely an acquired condition due to extensive stretching of the lens and zonules secondary to globe enlargement in neglected buphthalmos.

Conclusion:
A neglected case of congenital glaucoma can lead to lens subluxation along with lens coloboma.
Regional Adoption of Primary Care—Mental Health Integration in Veterans Health Administration Patient-Centered Medical Homes
Authors: Leung Lucinda B. , Rose Danielle , Stockdale Susan , McGowan Michael , Yano Elizabeth M. , Graaff A. Laurie , Dresselhaus Timothy R. , Rubenstein Lisa V. 
Published: May 2019

ABSTRACT

Introduction:
Behavioral health integration is important, yet difficult to implement, in patient-centered medical homes. The Veterans Health Administration (VA) mandated evidence-based collaborative care models through Primary Care–Mental Health Integration (PC-MHI) in large PC clinics. This study characterized PC-MHI programs among all PC clinics, including small sites exempt from program implementation, in one VA region.

Methods:
Researchers administered a cross-sectional key informant organizational survey on PC-MHI among VA PC clinics in Southern California, Arizona, and New Mexico (n = 69 distinct sites) from February to May 2018. Researchers analyzed PC clinic leaders' responses to five items about organizational structure and practice management.

Results:
Researchers received surveys from 65 clinics (94% response rate). Although only 38% were required to implement on-site PC-MHI programs, 95% of participating clinics reported providing access to such services. The majority reported having integrated, colocated, or tele-MH providers (94%) and care management (77%). Most stated same-day services (59%) and “warm” handoffs (56%) were always available, the former varying significantly based on clinic size and distance from affiliated VA hospitals.

Conclusions:
Regional adoption of PC-MHI was high, including telemedicine, among VA patient-centered medical homes, regardless of whether implementation was required. Small, remote PC clinics that voluntarily provide PC-MHI services may need more support.