bluuebunny/crossref_metadata_2025 huggingface dataset:
Autism Spectrum Disorder in Males with Sex Chromosome Aneuploidy: XXY/Klinefelter Syndrome, XYY, and XXYY
Authors: Tartaglia Nicole R. , Wilson Rebecca , Miller Judith S. , Rafalko Jessica , Cordeiro Lisa , Davis Shanlee , Hessl David , Ross Judith
Published: March 2017

Objective:

Neurodevelopmental concerns in males with sex chromosome aneuploidy (SCA) (XXY/Klinefelter syndrome, XYY, XXYY) include symptoms seen in autism spectrum disorder (ASD), such as language impairments and social difficulties. We aimed to: (1) evaluate ASD characteristics in research cohorts of SCA males under DSM-IV compared to DSM-5 criteria, and (2) analyze factors associated with ASD diagnoses in SCA.

Methods:

Evaluation of participants with XXY/KS (n=20), XYY (n=57) and XXYY (n=21) included medical history, cognitive/adaptive testing, Social Communication Questionnaire, Social Responsiveness Scale, Autism Diagnostic Observation Schedule, Autism Diagnostic Interview-Revised, and DSM ASD criteria. Clinical impressions of ASD diagnostic category using the ADOS and DSM-IV criteria were compared to ADOS-2 and DSM-5 criteria. T-tests compared cognitive, adaptive, SES and prenatal vs. postnatal diagnoses between ASD and no ASD groups.

Results:

ASD rates in these research cohorts were 10% in XXY/KS, 38% in XYY, and 52% in XXYY using ADOS-2/DSM-5, and were not statistically different compared to DSM-IV criteria. In XYY and XXYY, the ASD group had lower verbal IQ and adaptive functioning compared to those without ASD. Many children without ASD still showed some social difficulties.

Conclusion:

ASD rates in males with SCA are higher than reported for the general population. Males with Y chromosome aneuploidy (XYY and XXYY) were 4.8 times more likely to have a diagnosis of ASD than the XXY/KS group, and 20 times more likely than males in the general population (1 in 42 males, CDC 2010). ASD should be considered when evaluating social difficulties in SCA. Studies of SCA and Y-chromosome genes may provide insight into male predominance in idiopathic ASD.
Associations Among Behavioral Sleep Disturbance, Family Functioning, and Controller Medication Adherence in Children with Asthma
Authors: Sonney Jennifer T. , Segrin Chris , Ward Teresa M.
Published: March 2017

Objective:

This study examined the associations among child sleep disturbances, family functioning, and asthma controller medication adherence in school-age children with persistent asthma.

Methods:

Thirty-four children aged 6 to 11 years and a parent independently reported on asthma control and controller medication adherence. Parents also reported on family functioning (behavior control) and child sleep disturbances (bedtime resistance, daytime sleepiness). Hierarchical linear regression models tested sleep disturbance variables as predictors of family functioning and medication adherence. A mediation model tested behavior control as a mediator between sleep disturbance and medication adherence.

Results:

Seventy-nine percent of the children had well-controlled asthma. Despite a mean of 9.48 hours of child sleep per night, 73% of the children had clinically significant disturbed sleep. Controlling for child age, bedtime resistance accounted for 32% of the variance in family behavior control (F
(1,31) = 14.75, p < .01). Behavior control also significantly mediated the relationship between total sleep disturbance and medication adherence, with a standardized indirect effect of β = −.17 (95% confidence interval [CI], −.47 to −.03) for parent-reported adherence and β = −.12 (95% CI, −.36 to −.01) for child-reported adherence.

Conclusion:

Child behavioral sleep disturbances significantly predicted family behavior control. Although child sleep disturbances did not significantly predict asthma controller medication adherence, there was a significant indirect effect of sleep disturbance on medication adherence through compromised family behavior control. Developmentally appropriate behavioral sleep interventions may improve family functioning and child asthma controller medication adherence. Family functioning may also be an entry point for intervention to improve medication adherence.
RETINAL DETACHMENT AND RETROBULBAR CYSTS IN A LARGE COHORT OF OPTIC NERVE COLOBOMA
Authors: Chen Yufeng N. , Patel C.K. , Kertes Peter J. , Devenyi Robert G. , Blaser Susan , Lam Wai-Ching
Published: March 2017

Purpose:

To examine the relationship between retinal detachment and retrobulbar cysts in patients with optic nerve coloboma (ONC) and Morning Glory syndrome (MGS).

Methods:

Patients diagnosed with either ONC or MGS were identified through a search of the Sick Kids database. Seventy-one patients either agreed to come in for a B-scan or had an incidental orbital B-scan or magnetic resonance imaging or both. Eyes with orbital B-scan ultrasound and/or magnetic resonance imaging images were assessed independently by two ophthalmologists and a radiologist for the presence of retrobulbar cysts. Retinal detachment was identified clinically with either indirect ophthalmoscopy or from fundus photographs.

Results:

Forty-five of 71 (63%) and 26/71 (37%) patients had ONC and MGS, respectively. Retinal detachment occurred significantly more often in eyes with MGS than with ONC (9/17 [53%] vs. 5/45 [11%], P = 0.03, respectively). Retrobulbar cysts were not detected more often in MGS than in ONC (11/45 [24%] vs. 7/26 [27%]; P = 1.0). Eyes with retrobulbar cysts were significantly more likely to be associated with retinal detachment than those without (7/18 [39%] vs. 7/53 [13%]; P = 0.04).

Conclusion:

Retinal detachment occurs more frequently in MGS than in ONC in a cohort of patients referred to a specialist children's retinal service. Eyes with retrobulbar cysts are more likely to be associated with retinal detachment.
RUTHENIUM-106 BRACHYTHERAPY IN THE TREATMENT OF CIRCUMSCRIBED CHOROIDAL HEMANGIOMA
Authors: Naseripour Masood , Maleki Arash , Astaraki Arezoo , Sedaghat Ahad , Jaberi Ramin , Lee Stacey , Azma Zohreh , Silpa-Archa Sukhum
Published: March 2017

Purpose:

To examine the efficacy and safety of Ruthenium-106 plaque radiotherapy in the treatment of circumscribed choroidal hemangioma.

Methods:

Twenty-one eyes of 21 patients diagnosed with symptomatic circumscribed choroidal hemangioma who underwent Ruthenium-106 plaque radiotherapy were included in the study. Clinical response, ancillary tests finding improvement, and major side effects were evaluated.

Results:

From the initial to the 1-year follow-up visits, vision improved in 12 eyes (57%), was stable in 7 eyes (33%), and became worse in 2 eyes (10%). Based on fluorescein angiography and optical coherence tomography, subretinal fluid and cystoid macular edema resolved in all patients. Changes in logarithm of minimum angle of resolution visual acuity (P = 0.038); tumor thickness (P = 0.0001) and largest diameter (P = 0.007) on ultrasonography; and subfoveal thickness on optical coherence tomography (P < 0.0001), were statistically significant between the initial and the 1-year follow-up visits. Side effects as observed during the follow-up period included: radiation-related retinopathy in 5 (24%) eyes, radiation-related papillopathy in 1 eye (5%), and subretinal fibrosis in 2 eyes (10%). Subretinal fibrosis was the only permanent radiation-related side effect.

Conclusion:

Ruthenium-106 plaque radiotherapy is an effective and safe method of treatment for symptomatic circumscribed choroidal hemangiomas. The incidence of permanent visual loss is low with prompt treatment of complications.
CORRELATION BETWEEN CENTRAL FOVEAL THICKNESS AND VISUAL ACUITY IN PATIENTS WITH IDIOPATHIC VITREOMACULAR TRACTION
Authors: Uzel Mehmet M. , Citirik Mehmet , Ilhan Cagri , Tekin Kemal
Published: March 2017

Purpose:

To evaluate the impact of preoperative central foveal thickness (CFT) on visual acuity after surgery in patients with idiopathic vitreomacular traction and to calculate a cut-off value in preoperative CFT.

Methods:

Thirty-five patients with idiopathic vitreomacular traction were evaluated retrospectively. A complete ophthalmological examination including spectral domain optical coherence tomography was performed preoperatively and at 12 months after the surgery. Receiver operating characteristic analysis was used to determine the critical point for the CFT associated with improvement of 10 or more letters in visual acuity on the Early Treatment Diabetic Retinopathy Study.

Results:

Among 35 patients, the mean CFT at postoperative 12 months was significantly decreased from baseline (P = 0.001). Preoperative CFT and visual improvement were not significantly correlated (r = −0.090, P = 0.605), whereas preoperative CFT and preoperative visual acuity were significantly correlated (r = 0.757, P < 0.001). Improvement in CFT and visual improvement were also correlated significantly (r = 0.449, P = 0.007). According to the receiver operating characteristic analysis, the threshold CFT was 471 μm. When 15 patients with CFT thickness under 471 μm were evaluated, preoperative CFT was significantly correlated with improvement in visual acuity after the surgery (r = 0.561, P = 0.030).

Conclusion:

Preoperative CFT is important for the visual prognosis of patients with vitreomacular traction. Preoperative CFT of these patients should be considered in surgical decisions.
MULTIMODALITY OCULAR IMAGING IN A CASE REPORT OF HYPERVISCOSITY SYNDROME ASSOCIATED WITH LYMPHOPLASMACYTIC LEUKEMIA: THE IMAGES TELL THE STORY
Authors: Choi Rene Y. , Jacoby Rachel , Shakoor Akbar
Published: March 2017

Purpose:

To report the clinical course of a patient with ocular manifestations of hyperviscosity syndrome associated with Waldenström macroglobulinemia, and for the first time, video imaging of mobile emboli in the conjunctival and retinal vasculature.

Methods and Patient:

A 60-year-old woman with newly diagnosed Waldenström macroglobulinemia, with no visual complaints was evaluated by the Ophthalmology service for a baseline ocular examination.

Results:

At presentation, ocular examination revealed a visual acuity of 20/25 in each eye. Slit lamp examination showed mobile white emboli throughout the conjunctival vasculature of both eyes, which was captured on video. Dilated fundus examination revealed peripheral vascular occlusion along with extensive collateral formation in both eyes and as dilation of vessels at the posterior pole of the left eye. Mobile arterial and venous emboli were also observed in the retinal vasculature, which were captured with slit-lamp color and infrared reflectance video imaging.

Conclusion:

Hyperviscosity syndrome is a rare circulation sequelae that occurs when blood is thickened secondary to an increase in immunoglobulins or a hyperproliferation of blood components as in bone marrow dyscrasias. The increase in viscosity is plainly observable in the retinal circulation, and this syndrome is often diagnosed based on visual symptoms and fundus examination. We report a patient with Waldenström macroglobulinemia who presented with multiple ocular manifestations of hyperviscosity syndrome despite the absence of visual complaints. Ophthalmologists should be aware that regardless of the lack of any subjective visual changes, ocular findings of hyperviscosity syndrome should prompt quick referral to a Hematology Oncologist for evaluation of other end-organ damage. We also for the first time, provide video documentation of mobile emboli in the conjunctival and retinal vasculature.
IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSING PROGRESSIVE OPTIC NERVE EDEMA IN CRYPTOCOCCAL MENINGITIS
Authors: Werner Astrid C. , Vuong Laurel N. , Hedges Thomas R. , Baumal Caroline R.
Published: March 2017

Purpose:

To report an human immunodeficiency virus–positive patient undergoing therapy for cryptococcal meningitis who developed progressive optic disk edema that was steroid responsive.

Methods:

Observational case report.

Results:

One month after commencing antifungal treatment for cryptococcal meningitis, the patient developed bilateral, progressive, recurrent optic disk edema with subretinal fluid that coincided with initiation of highly active antiretroviral therapy and recovery of CD4+ cell counts. Lumbar puncture revealed normal opening pressure, and cerebrospinal fluid showed no recurrence of cryptococcal infection. There was no evidence of uveitis. The patient rapidly improved with a 5-day course of high-dose intravenous methylprednisolone.

Conclusion:

Recurrent optic disk edema with loss of vision after treatment of cryptococcal meningitis in the setting of normal intracranial pressure may represent a unique manifestation of immune reconstitution inflammatory syndrome localized to the optic nerve without uveitis. This is consistent with the temporal relationship between starting highly active antiretroviral therapy, CD4+ count recovery, and the development of progressive disk edema in the study patient. Isolated optic nerve inflammation as a manifestation of immune reconstitution inflammatory syndrome has not been widely reported.
The Association Between Clinical Features Seen on Fundus Photographs and Glaucomatous Damage Detected on Visual Fields and Optical Coherence Tomography Scans
Authors: Alhadeff Paula A. , De Moraes Carlos G. , Chen Monica , Raza Ali S. , Ritch Robert , Hood Donald C.
Published: March 2017

Purpose:

To classify the appearance of the optic disc seen on fundus photographs of healthy subjects and patients with or suspected glaucoma whose diagnosis was based upon visual fields (VFs) and spectral-domain optical coherence tomography (sdOCT) results.

Patients and Methods:

One eye of 100 patients with or suspected glaucoma and 62 healthy subjects were prospectively tested with 24-2 and 10-2 VF and macular and disc sdOCT cube scans. All eyes with or suspected glaucoma had a 24-2 mean deviation better than −6.0 dB and an abnormal appearing disc on stereophotographs. The retinal ganglion cell plus inner plexiform layer (RGC+) from the macular scans and the retinal nerve fiber layer (RNFL) from the macular and disc scans were segmented and converted to probabilities plots. An eye was considered “glaucoma” if the sdOCT probability plots showed an abnormality in a region that corresponded to a defect seen on the 24-2 and/or 10-2 VF total deviation plot. Similarly, an eye was considered “suspect” only if both the sdOCT and VF plots were normal. Healthy subjects (normal VFs and sdOCT) were classified as “controls” and used as reference for comparisons. Glaucoma specialists reviewed the stereophotographs and classified eyes based on the presence of signs suggestive of glaucomatous optic neuropathy.

Results:

The pattern of clinical signs of glaucomatous optic neuropathy seen on stereophotographs was statistically different between glaucoma (P<0.001) and suspects (P<0.001) vs. controls and explained up to 68% of the total variance of the diagnosis based upon sdOCT and VFs. Vertical cup-to-disc>0.6, focal neuroretinal rim thinning, focal RNFL loss, and violation of the ISNT rule had the best performance to differentiate glaucoma and suspects from controls. Compared with the suspect group, glaucoma eyes (abnormal sdOCT and VF tests) were more likely to have vertical cup-to-disc>0.6 (92% vs. 69%, P=0.003), diffuse rim (53% vs. 9%, P<0.001) and RNFL (61% vs. 26%, P<0.001) thinning, and β-zone parapapillary atrophy (68% vs. 17%, P<0.001).

Conclusions:

Focal and diffuse signs of glaucoma damage seen on stereophotographs often match damage shown on VFs and sdOCT. In addition, damage shown on VFs and sdOCT is often missed during clinical evaluation. Longitudinal studies ought to differentiate focal signs of glaucoma damage seen on stereophotography from false-positives or very early loss.
M&S Smart System Contrast Sensitivity Measurements Compared With Standard Visual Function Measurements in Primary Open-Angle Glaucoma Patients
Authors: Liu Jessica L. , McAnany J. Jason , Wilensky Jacob T. , Aref Ahmad A. , Vajaranant Thasarat S.
Published: March 2017

Purpose:

To evaluate the nature and extent of letter contrast sensitivity (CS) deficits in glaucoma patients using a commercially available computer-based system (M&S Smart System II) and to compare the letter CS measurements to standard clinical measures of visual function.

Methods:

Ninety-four subjects with primary open-angle glaucoma participated. Each subject underwent visual acuity, letter CS, and standard automated perimetry testing (Humphrey SITA 24-2). All subjects had a best-corrected visual acuity (BCVA) of 0.3 log MAR (20/40 Snellen equivalent) or better and reliable standard automated perimetry (fixation losses, false positives, and false negatives <33%). CS functions were estimated from the letter CS and BCVA measurements. The area under the CS function (AUCSF), which is a combined index of CS and BCVA, was derived and analyzed.

Results:

The mean (± SD) BCVA was 0.08±0.10 log MAR (∼20/25 Snellen equivalent), the mean CS was 1.38±0.17, and the mean Humphrey Visual Field mean deviation (HVF MD) was −7.22±8.10 dB. Letter CS and HVF MD correlated significantly (r=0.51, P<0.001). BCVA correlated significantly with letter CS (r=−0.22, P=0.03), but not with HVF MD (r=−0.12, P=0.26). A subset of the subject sample (∼20%) had moderate to no field loss (≤−6 dB MD) and minimal to no BCVA loss (≤0.3 log MAR), but had poor letter CS. AUCSF was correlated significantly with HVF MD (r=0.46, P<0.001).

Conclusions:

The present study is the first to evaluate letter CS in glaucoma using the digital M&S Smart System II display. Letter CS correlated significantly with standard HVF MD measurements, suggesting that letter CS may provide a useful adjunct test of visual function for glaucoma patients. In addition, the significant correlation between HVF MD and the combined index of CS and BCVA (AUCSF) suggests that this measure may also be useful for quantifying visual dysfunction in glaucoma patients.
Visual Field Defects in Young Patients With Open-angle Glaucoma: Comparison Between High-tension and Normal-tension Glaucoma
Authors: Park Ji-Hye , Yoo Chungkwon , Park Jinhwan , Kim Yong Yeon
Published: March 2017

Purpose:

To investigate the clinical features of young Korean patients with open-angle glaucoma (OAG), and to compare visual field (VF) defects between patients with normal-tension glaucoma (NTG) and those with high-tension glaucoma (HTG).

Materials and Methods:

Patients with OAG younger than 40 years of age were divided into NTG and HTG groups based on untreated intraocular pressure (IOP) level. The patients were matched according to the mean deviation of standard automated perimetry. VFs were divided into 2 pairs of corresponding areas across the horizontal meridian, and the depths of VF defects were compared. Tilt ratio and torsion degree of their optic discs were also compared between the 2 groups.

Results:

Thirty-four pairs of NTG and HTG eyes were matched. Although untreated IOP showed intergroup differences, there was no difference in age, refractive error, tilt ratio, torsion degree, and depth of VF defects between the 2 groups. However, NTG eyes showed deeper VF defects in the superior paracentral area than in the inferior paracentral area (−8.29±9.33 vs. −4.45±6.03 dB, P=0.038), whereas the HTG eyes showed symmetric VF defects. Moreover, the superior paracentral area showed deeper VF defects compared with the superior arcuate area in the NTG group (P=0.002).

Conclusions:

Young patients with NTG showed asymmetric VF defects in the paracentral area, whereas those with HTG did not show such asymmetric defects. This suggests that the level of IOP may affect the pattern of VF defects in young patients with OAG. However, these findings may not be generalizable to other populations.
Relationship of Optic Nerve Structure and Function to Peripapillary Vessel Density Measurements of Optical Coherence Tomography Angiography in Glaucoma
Authors: Rao Harsha L. , Pradhan Zia S. , Weinreb Robert N. , Dasari Srilakshmi , Riyazuddin Mohammed , Raveendran Sharanya , Puttaiah Narendra K. , Venugopal Jayasree P. , Rao Dhanaraj A.S. , Devi Sathi , Mansouri Kaweh , Webers Carroll A.B.
Published: March 2017

Purpose:

To evaluate the sectoral and global structure-structure (vessel density-retinal nerve fiber layer thickness) and structure-function (vessel density-visual sensitivity loss) relationships of peripapillary vessel density measurements on optical coherence tomography angiography in primary open-angle glaucoma and to determine if fractional polynomial (FP) models characterize the relationships better than linear models.

Materials and Methods:

In a cross-sectional study, structure-structure and structure-function relationships of peripapillary vessel densities were determined in 227 eyes of 143 subjects (63 control and 164 primary open-angle glaucoma eyes) who had undergone standard automated perimetry and optical coherence tomography testing within 6 months of each other, using linear and FP models. FP model evaluates the relationship between the dependent and the best-fitting fractional powers of the independent variable. Strength of relationship was reported as coefficient of determination (R
2).

Results:

R
2 values for structure-structure associations using linear models (0.53 for superotemporal sector, 0.61 for inferotemporal, and 0.53 for average measurements) were significantly less (P<0.05) than that determined using FP models (0.57, 0.65, and 0.55, respectively). R
2 values for structure-function associations using linear models (0.35 for superotemporal vessel density-inferotemporal visual sensitivity loss, 0.49 for inferotemporal vessel density-superotemporal visual sensitivity loss, and 0.39 for average vessel density-average visual sensitivity loss) were significantly less than that determined using FP models (0.43, 0.58, and 0.47, respectively).

Conclusions:

The inferotemporal peripapillary vessel density showed the strongest association with the corresponding retinal nerve fiber layer thickness and visual sensitivity loss in the global and sectoral regions studied. The FP models were significantly better than linear models in describing these relationships.
A Double-Blind, Placebo-Controlled, Pilot Study of Riluzole Monotherapy for Acute Bipolar Depression
Authors: Park Lawrence T. , Lener Marc S. , Hopkins Matthew , Iadorola Nicolas , Machado-Vieira Rodrigo , Ballard Elizabeth , Nugent Allison , Zarate Carlos A.
Published: March 2017

Background

Glutamatergic system abnormalities are implicated in the pathophysiology and treatment of both major depressive disorder and bipolar depression (BDep). Subsequent to studies demonstrating the rapid and robust antidepressant effects of ketamine, an N-methyl-d-aspartate receptor antagonist, other glutamatergic modulators are now being studied in clinical trials of mood disorders. A previous open-label study found that riluzole, administered in combination with the mood stabilizer lithium, had antidepressant effects.

Methods

We conducted a randomized, double-blind, placebo-controlled trial of riluzole monotherapy for the treatment of BDep. Nineteen subjects aged 18 to 70 years with bipolar disorder currently experiencing a depressive episode were tapered off of excluded medications and randomized to receive riluzole (50–200 mg/d) or placebo for 8 weeks. Rating scale scores (Montgomery-Åsberg Depression Rating Scale, Hamilton Rating Scale for Depression, Hamilton Rating Scale for Anxiety, and Young Mania Rating Scale) were obtained weekly.

Results

No significant differences in depressive symptoms were observed between subjects treated with riluzole and those receiving placebo (P = 0.12). Anxiety scores were significantly lower in the placebo group (P = 0.046). An interim analysis was conducted that resulted in stopping the study because of futility; no subjects had achieved treatment response.

Conclusions

Although we found no change in severity of depressive symptoms in BDep patients receiving riluzole compared with placebo, this trial was limited by the relatively high number of subject withdrawals and the small sample size. Thus, while riluzole monotherapy did not demonstrate efficacy for BDep, further studies examining riluzole as adjunctive therapy for this disorder may be warranted.

Clinical Trials Identifier NCT00054704.
The Challenges in Predicting ECMO Survival, and a Path Forward
Authors: Huesch Marco , Brehm Christopher
Published: March 2017

Extracorporeal membrane oxygenation (ECMO) support is a life-saving but complex technique for patients suffering from severe cardiac or pulmonary dysfunction. Increasingly greater utilization in the last 15 years means that a suite of mortality risk analytics is both feasible for researchers and required by clinicians, patients, administrators, and insurers. We argue that to date, research into such risk analytics has been insufficient and does not adequately reflect the various indications and configurations of extracorporeal life support (ECLS). We propose a path to address these challenges and ensure that clinicians and researchers obtain robust, specific, risk analytics.
The Origins of Continuous Renal Replacement Therapy
Authors: Bartlett Robert H.
Published: March 2017

Peter Kramer’s presentation at the 1982 ASAIO meeting was the inspiration that led to continuous renal replacement therapy in intensive care.
Is severe hypercalcemia immediately life-threatening?
Authors: Guimard Camille , Batard Eric , Lavainne Frédéric , Trewick David
Published: March 2017

Objective

Severe hypercalcemia is often considered an emergency because of a potential risk of cardiac arrest or coma. However, there is little evidence to support this. The aim of our study was to assess whether severe hypercalcemia (Ca>4 mmol/l or 16 mg/dl) was associated with immediately life-threatening cardiac arrhythmias or neurological complications in patients admitted to the Emergency Department (ED).

Methods

A retrospective observational study was carried out over a 5-year period (2008–2012). Eligible patients were admitted to the Adult Emergency Department of Nantes University Hospital and had a calcium concentration in excess of 4 mmol/l. There were no exclusion criteria. The primary outcome was the number of life-threatening cardiac arrhythmias and/or neurological complications during the stay in the ED. The secondary outcomes were correlation between calcium concentrations/ECG QTc intervals and mortality.

Results

A total of 126 204 adult patients had calcium concentrations measured. Thirty one (0.025%) patients had severe hypercalcemia as defined in our study. The median calcium concentration was 4.3 mmol/l (Q1, 4.2; Q3, 4.7) and the median albumin-adjusted calcium concentration was 4.3 mmol/l (Q1, 4.1; Q3, 4.7). No patient presented with a life-threatening cardiac event during stay in the ED. The median ED stay was 7 h 32 min. One patient presented with a coma of multifactorial origin. There was no correlation between calcemia and QTc intervals (P=0.60). Mortality at 1 year was 55% (17 patients).

Conclusion

We found no cases of immediately life-threatening cardiac arrhythmias or neurological complications associated with hypercalcemia above 4 mmol/l over a 5-year period in a large tertiary ED.
Cost-effectiveness of Antihypertensive Medication
Authors: Tajeu Gabriel S. , Mennemeyer Stephen , Menachemi Nir , Weech-Maldonado Robert , Kilgore Meredith
Published: March 2017

Background:

Antihypertensive medication decreases risk of cardiovascular disease (CVD) events in adults with hypertension. Although black adults have higher prevalence of hypertension and worse CVD outcomes compared with whites, limited attention has been given to the cost-effectiveness of antihypertensive medication for blacks.

Objective:

To compare the cost-effectiveness of antihypertensive medication treatment versus no-treatment in white and black adults.

Research Design:

We constructed a State Transition Model to assess the costs and quality-adjusted life-years (QALYs) associated with either antihypertensive medication treatment or no-treatment using data from the REasons for Geographic and Racial Differences in Stroke (REGARDS) study and published literature. CVD events and health states considered in the model included stroke, coronary heart disease, heart failure, chronic kidney disease, and end-stage renal disease.

Subjects:

White and black adults with hypertension in the United States, 45 years of age and above.

Measures:

Yearly risk of CVD was determined using REGARDS data and published literature. Antihypertensive medication costs were determined using Medicare claims. Event and health state costs were estimated from published literature. All costs were adjusted to 2012 US dollars. Effectiveness was assessed using QALYs.

Results:

Antihypertensive medication treatment was cost-saving and increased QALYs compared with no-treatment for white men ($7387; 1.14 QALYs), white women ($7796; 0.89 QALYs), black men ($8400; 1.66 QALYs), and black women ($10,249; 1.79 QALYs).

Conclusions:

Antihypertensive medication treatment is cost-saving and increases QALYs for all groups considered in the model, particularly among black adults.
Influenza in children
Authors: Kondrich Janienne , Rosenthal Michele
Published: March 2017

Purpose of review

We review the current information and evidence available on the global burden of disease in the pediatric population, clinical presentation and complications, testing, treatment, and immunization.

Recent findings

In addition to multiple other risk factors for influenza complications, children with neurologic and neuromuscular disorders are significantly higher risk for serious complications. In practice, there is no lower age limit for children with influenza who can be treated with oseltamivir. The quadrivalent live attenuated influenza vaccine was not recommended for use during the 2016–2017 season due to poor effectiveness.

Influenza infection causes a significant burden of disease each year in the pediatric population worldwide. Both healthy and chronically ill children can fall prey to complications either due to the virus itself or secondary bacterial infection. Children within high-risk groups should be tested and treated with neuraminidase inhibitors. Immunization against influenza is well tolerated and effective.
Six-Month Follow-up of a Regional Evidence-based Practice Fellowship Program
Authors: Kim Son Chae , Stichler Jaynelle F. , Ecoff Laurie , Gallo Ana-Maria , Davidson Judy E.
Published: March 2017

OBJECTIVE

This study examined a 6-month follow-up of a regional evidence-based practice (EBP) fellowship program and the predictors of EBP adoption at hospital units.

BACKGROUND

The immediate beneficial effects of a regional EBP program are known, but the medium-term effects are not certain.

METHODS

A matched pretest/posttest study was conducted using a mailed questionnaire 6 months after the completion of a 9-month regional EBP fellowship program among 3 annual cohorts of participants.

RESULTS

Statistically significant improvements in the mean scores of EBP beliefs, EBP implementation, and group cohesion were found (P < .05). Of the 47 participants who completed their EBP projects, more than three-quarters reported EBP adoption at their own hospital units, and EBP beliefs were a positive predictor of EBP adoption (odds ratio, 1.12; 95% confidence interval, 1.02-1.22; P = .017).

CONCLUSIONS

The outcome improvements continued 6 months after the fellowship program, and strong EBP beliefs predicted EBP adoption in the participants’ units.
Mobile Phone and Internet Use Mostly for Sex-Seeking and Associations With Sexually Transmitted Infections and Sample Characteristics Among Black/African American and Hispanic/Latino Men Who Have Sex With Men in 3 US Cities
Authors: Allen Jacob E. , Mansergh Gordon , Mimiaga Matthew J. , Holman Jeremy , Herbst Jeffrey H.
Published: March 2017

Background

Men who have sex with men (MSM) have a relatively high prevalence of sexually transmitted infections (STIs). This study examines the association of self-reported STIs and use of mobile phones and/or computer-based Internet to meet sexual partners among black and Hispanic/Latino MSM in the United States.

Methods

Black and Hispanic/Latino MSM (N = 853) were recruited from 3 US cities (Chicago, IL; Kansas City, MO; and Fort Lauderdale, FL) via online and community outreach. Men completed a computer-assisted, self-interview assessment on demographics, use of mobile phones and computer-based Internet for sex-seeking, sexual risk behavior, and self-reported bacterial STIs in the past year. Multivariable logistic regression was used to model independent associations of STIs and use of these technologies to meet sexual partners.

Results

Twenty-three percent of the sample reported having an STI in the past year; 29% reported using a mobile phone and 28% a computer-based Internet mostly for sex-seeking; and 22% reported using both. Number of male sexual partners (past year) was associated with any STI (adjusted odds ratio, 1.03; 95% confidence interval, 1.01–1.06). Adjusting for human immunodeficiency virus status, number of male sexual partners (past year), and demographic variables, men who reported use of both mobile phones and computer-based Internet for sex-seeking had increased odds of reporting an STI (adjusted odds ratio, 2.59; 95% confidence interval, 1.75–3.83), as well as with separate reports of chlamydia, gonorrhea, and syphilis (P’s < 0.05).

Conclusions

Enhanced community education regarding STI prevention, testing, and treatment options are necessary among this subpopulation of MSM who may benefit from messaging via Internet and mobile phone application sites.
Studying the Retinal Source of Photophobia by Facial Electroretinography
Authors: Tyler Christopher W. , Likova Lora T.
Published: March 2017

Purpose

Photophobia is a debilitating clinical condition that disrupts the ability to use vision for everyday tasks in bright lighting conditions. The goal of the study is to develop a methodology to study the neural basis of photophobia and the contribution of the melanopic pathway to its etiology with differential chromatic responses by means of standard electroencephalographic recording equipment.

Methods

We introduce and validate the approach of recording wavelength-specific electroretinographic (ERG) responses from the face electrodes of the high-density whole-head electroencephalography recording system under light-adapted conditions.

Results

ERGs recorded in this way to whole-field chromatic stimuli exhibit striking differences between the photophobic and non-photophobic groups. The control responses were consistent with photopic intensity in peak time, and in the ordering of peak times as a function of wavelength condition, indicating a predominantly cone source of the signals. The photophobic responses, on the other hand, were substantially slowed relative to controls, with the peak times conforming to a different order as a function of wavelength condition than controls, implying that the cone response has been suppressed and that the responses derived from a different photoreceptor system consistent with mediation by melanopic retinal ganglion cells.

Conclusions

The results will be important for determining the neural pathways involved in photophobia and potential approaches to its treatment on the basis of this etiology.
Sellar Atypical Teratoid/Rhabdoid Tumor (AT/RT)
Authors: Nakata Satoshi , Nobusawa Sumihito , Hirose Takanori , Ito Shinji , Inoshita Naoko , Ichi Shunsuke , Amatya Vishwa J. , Takeshima Yukio , Sugiyama Kazuhiko , Sonoda Yukihiko , Haga Hironori , Hirato Junko , Nakazato Yoichi , Yokoo Hideaki
Published: March 2017

Atypical teratoid/rhabdoid tumors (AT/RTs) are rare aggressive tumors of the central nervous system that predominantly affect infants. Although adult AT/RT are rare, accumulated cases have revealed adult-specific AT/RT in the sellar region. Twelve previously reported cases of sellar AT/RT exclusively occurred in adult females, suggesting biological differences from conventional infant AT/RT. We herein investigated a series of 6 sellar AT/RT for histopathologic features, the molecular status of the INI1/SMARCB1 gene, and clinical courses. All 6 cases were adult females, ranging in age from 21 to 69 years old. Tumors were histologically characterized by a hemangiopericytoma-like stag-horn vasculature within a dense, diffuse proliferation of jumbled cells and a small number of scattered rhabdoid cells. This vascular pattern is not a common finding in AT/RT and appears to be a characteristic histology of sellar AT/RT. Biallelic alterations in the INI1 gene were identified by fluorescence in situ hybridization, direct sequencing, and multiple ligation-dependent probe amplification analyses in 4 of the 5 cases analyzed. Three of the 4 cases harbored 2 different mutations, presumably on different alleles (compound heterozygous mutations), and 1 case of which had a splice-site mutation. Combined with previous findings, the prevalence of compound heterozygous mutations and splice-site mutations was significantly higher in sellar AT/RT than in pediatric AT/RT. Sellar AT/RT represent a clinicopathologically and possibly genetically distinct variant of AT/RT showing a characteristic demography, different patterns of INI1 alterations, and a histology featured by a unique vasculature.
ALK Expression Is a Novel Marker for the WNT-activated Type of Pediatric Medulloblastoma and an Indicator of Good Prognosis for Patients
Authors: Łastowska Maria , Trubicka Joanna , Niemira Magdalena , Paczkowska-Abdulsalam Magdalena , Karkucińska-Więckowska Agnieszka , Kaleta Magdalena , Drogosiewicz Monika , Tarasińska Magdalena , Perek-Polnik Marta , Krętowski Adam , Dembowska-Bagińska Bożenna , Grajkowska Wiesława , Pronicki Maciej , Matyja Ewa
Published: March 2017

ALK gene rearrangements were identified in a variety of cancers, including neuroblastoma, where the presence of ALK expression is associated with adverse prognosis. ALK mutations have recently been found in the pediatric brain tumor medulloblastoma, and microarray data indicate that ALK is highly expressed in a subset of these tumors. Therefore, we investigated whether ALK expression correlates with transcriptional profiles and clinical features of medulloblastoma. Tumors from 116 medulloblastoma patients were studied at diagnosis for the detection of ALK expression at the RNA level by an application of NanoString technology and at the protein level by immunohistochemistry using antibody ALK clone D5F3. The results indicate that ALK expression, at both the RNA and the protein levels, is strongly associated with the WNT-activated type of tumors and therefore may serve as a useful marker for the detection of this type of medulloblastoma. Importantly, ALK protein expression alone is also an indicator of good prognosis for medulloblastoma patients.
Medial Row Perforators Are Associated with Higher Rates of Fat Necrosis in Bilateral DIEP Flap Breast Reconstruction
Authors: Kamali Parisa , Lee Michelle , Becherer Babette E. , Wu Winona , Curiel Daniel , Tran Bao Ngoc N. , Tobias Adam M. , Lin Samuel J. , Lee Bernard T.
Published: March 2017

Background:

The purpose of this study was to evaluate perfusion-related complications in bilateral deep inferior epigastric perforator (DIEP) flap breast reconstruction based on perforator selection.

Methods:

A retrospective review of a prospectively maintained database was performed on all patients undergoing bilateral DIEP flap reconstruction at a single institution between 2004 and 2014. The hemiflaps were separated into three cohorts based on perforator location: lateral row only, medial row only, and medial plus lateral rows. Postoperative flap-related complications were compared and analyzed.

Results:

There were 728 total hemiflaps: 263 (36.1 percent) based on the lateral row, 225 (30.9 percent) based on the medial row, and 240 (33.0 percent) based on both the medial and lateral rows. The groups were well matched by perforator number and flap weight. Fat necrosis occurrence was significantly higher in flaps based solely on the medial row versus lateral row perforators (24.5 percent versus 8.2 percent; p < 0.001). There was no statistically significant difference in fat necrosis between flaps based only on the lateral row versus flaps based on both the medial and lateral rows (8.2 percent versus 11.6 percent; p = 0.203). Generally, within the same row, increasing the number of perforators decreased the incidence of fat necrosis.

Conclusions:

Perforator selection is critical for minimizing perfusion-related flap complications. In bilateral DIEP flaps, lateral row–based perforators result in significantly less fat necrosis than medial row–based perforators. The authors’ data suggest that the addition of a lateral row perforator to a dominant medial row perforator may decrease the risk of fat necrosis.

CLINICAL QUESTION/LEVEL OF EVIDENCE:

Therapeutic, III.
Validation of the Clinician-Graded Electronic Facial Paralysis Assessment
Authors: Chong Lauren S. H. , Eviston Timothy J. , Low Tsu-Hui (Hubert) , Hasmat Shaheen , Coulson Susan E. , Clark Jonathan R.
Published: March 2017

Background:

Facial paralysis remains a debilitating condition despite advances in medical, surgical, and adjunctive interventions. Established grading systems used to assess facial paralysis and interventional outcomes have well-described limitations. The Electronic Facial Paralysis Assessment, a clinician-graded zone-based facial function scale, has recently emerged as a grading tool that may provide greater sensitivity when assessing incomplete paralysis and postsurgical improvement. The authors perform the first comprehensive validation of this tool.

Methods:

Video recordings of 83 facial paralysis patients were assessed. Grading was performed in two sittings by three individuals with varying degrees of experience in assessing facial paralysis. Interobserver reliability; intraobserver reliability; administration time; and agreement with the Facial Disability Index, House-Brackmann, Sunnybrook, and Sydney facial grading systems were assessed.

Results:

The Electronic Facial Paralysis Assessment demonstrated high intra observer and interobserver reliability (intraclass correlation coefficient, 0.84 to 0.91 and 0.81 to 0.83, respectively). It correlated well with the House-Brackmann, Sunnybrook, and Sydney facial grading systems (Spearman rho, 0.73, 0.77 and 0.77, respectively). In subdomain analysis, it correlated well with the Sunnybrook and Sydney systems in dynamic movement (Spearman rho, 0.90 and 0.89, respectively) and synkinesis (Spearman rho, range 0.74 and 0.72, respectively). It had poor agreement with the Facial Disability Index (Spearman rho, 0.25). The mean time to complete the tool was 116 ± 61 seconds.

Conclusions:

The Electronic Facial Paralysis Assessment is a valid facial assessment tool with high reliability and correlation with the established facial paralysis grading systems. It also provides an efficient and detailed analysis of paralysis according to each facial zone.

CLINICAL QUESTION/LEVEL OF EVIDENCE:

Diagnostic, II.
Lung Cancers Associated With Cystic Airspaces: Natural History, Pathologic Correlation, and Mutational Analysis
Authors: Fintelmann Florian J. , Brinkmann Jesaja K. , Jeck William R. , Troschel Fabian M. , Digumarthy Subba R. , Mino-Kenudson Mari , Shepard Jo-Anne O.
Published: March 2017

Purpose:

The aim of the study was to investigate the natural history of non–small cell lung cancers (NSCLCs) associated with cystic airspaces, including histopathology and molecular analysis.

Materials and Methods:

A total of 34,801 computed tomographic (CT) scans of 2954 patients diagnosed with NSCLC between 2010 and 2015 were evaluated for association with a cystic airspace. Characteristics on serial CT, 18F-fludeoxyglucose positron emission tomography, and pathologic analysis were recorded.

Results:

Cystic airspaces were associated with 1% of NSCLC cases (12 men and 18 women; median age, 66 y [range, 44 to 87 y]). Of the total number of patients, 97% had a smoking history. Twenty-four adenocarcinomas, 4 squamous cell carcinomas, and 2 poorly differentiated carcinomas were distributed throughout all lobes and were predominantly peripheral. Some cystic airspaces appeared in previously normal lungs, whereas others were preceded by subcentimeter nodules. Twenty of 30 cases demonstrated increased soft tissue due to wall thickening, increased loculations, enlargement and/or increased attenuation of a mural nodule, or replacement by a mass. 18F-fludeoxyglucose uptake was present if solid components measured >8 mm. Twenty of 30 patients demonstrated >1 cystic lesion or ground-glass nodule, lymphadenopathy, or evidence of prior lung resection. Pathologic analysis revealed that cystic airspaces correspond to a check-valve mechanism, adenocarcinoma superimposed on emphysema, cystification, and adenocarcinoma parasitizing a preexisting bulla. Fourteen of 26 tumors and 64% of adenocarcinomas tested positive for an alteration of KRAS with or without other alterations.

Conclusions:

Cystic airspaces preceded by nodules can evolve into NSCLCs. Wall thickening and/or mural nodularity may develop. Location in the periphery of the upper lobes, emphysema, additional cystic lesions or ground-glass nodules, lymphadenopathy, and prior lung cancer should further increase suspicion. Cystic airspaces on CT can be due to a check-valve mechanism obstructing the small airways, lepidic growth of adenocarcinoma in an area of emphysema, cystification of tumor due to degeneration, or adenocarcinoma growing along the wall of a preexisting bulla. KRAS mutations are the predominant genetic alterations.
Restrictive Transfusion Practices After Esophagectomy Are Associated With Improved Outcome
Authors: Towe Christopher W. , Gulack Brian C. , Kim Sunghee , Ho Vanessa P. , Perry Yaron , Donahue James M. , Linden Philip A.
Published: March 2017

Objective:

Blood transfusion has been associated with poor outcomes in many disciplines, yet transfusion practices and related outcomes in esophagectomy are unknown. We analyzed the Society of Thoracic Surgeons General Thoracic Database to determine patient factors associated with transfusion after esophagectomy, risk-adjusted variation in transfusion practice among institutions, and the association of transfusion practice with mortality.

Methods:

We performed a retrospective review of patients undergoing esophagectomy for cancer from October 2008 to December 31, 2014. Patient comorbidities and procedure variables were used to construct a risk model for transfusion. Using this model, each institution was assigned an observed to expected (O:E) transfusion rate. We examined institutional factors associated with variation in O:E transfusion rate. Finally, O:E transfusion rate was compared to risk-adjusted mortality to determine if there was an association of transfusion practice and survival.

Results:

Seven thousand one hundred thirty-seven patients underwent esophagectomy at 182 institutions during the study period. The median unadjusted transfusion rate was 23.1%. The risk model for transfusion demonstrated patients who received transfusions were more likely to be older, female, and have low preoperative hemoglobin and other comorbidities, such as CAD, COPD, and low creatinine clearance. Patients who received a minimally invasive procedure were less likely to have received a transfusion.

After adjusting for the characteristics above, 13 centers (7.1%) were classified as having lower than average O:E transfusion rate and 16 centers (8.7%) were classified as higher than average O:E transfusion rate.

Institutions with lower than expected transfusion rates also had lower risk-adjusted perioperative mortality than institutions with higher than expected transfusion rates (median [IQR] = 0.90 [0.77–0.94] vs. 0.99 [0.94–1.06], P = 0.028).

Conclusion:

Age, female sex, CAD, COPD, renal insufficiency, and open technique are associated with transfusion after esophagectomy, while tumor stage and preoperative chemoradiation are not. There is wide variation in transfusion practice. Centers with lower than expected transfusion rate also had lower than expected perioperative mortality. At an institutional level, lower transfusion rates are associated with improved outcomes.