bluuebunny/crossref_metadata_2025 huggingface dataset:
The Effects of Leukocyte Filtration on Cell Salvaged Autologous Blood Transfusion on Lung Function and Lung Inflammatory and Oxidative Stress Reactions in Elderly Patients Undergoing Lumbar Spinal Surgery
Authors: Xu Lili , Shen Jianjun , Sun Jianliang , McQuillan Patrick M. , Hu Zhiyong
Published: February 2018

Background:

This study was designed to investigate the effects of leukocyte filtration of autologous salvaged blood on lung function, lung inflammatory reaction, and oxidative stress reaction in elderly patients undergoing lumbar spinal surgery.

Materials and Methods:

Sixty elderly patients undergoing lumbar spinal surgery were randomly divided into 2 groups: Leukocyte Filter group and Control group. Serum levels of inflammatory markers including white blood cell and polymorphonuclear count, neutrophil elastase, serum surfactant protein A, methane dicarboxylic aldehyde, superoxide dismutase, interleukin (IL)-6, IL-8, tumor necrosis factor-α, and respiratory function markers including dynamic respiratory system compliance, oxygenation index, and respiratory index were measured immediately before induction of anesthesia (T0), immediately before blood transfusion (T1), and 1 (T2), 6 (T3), and 12 hours (T4) after end of blood transfusion.

Results:

The Leukocyte Filter group had higher dynamic respiratory system compliance at T2, oxygenation index at T2 and T3, respiratory index and superoxide dismutase at T2, T3, and T4 than those in the Control group (P<0.05). The Leukocyte Filter group had lower white blood cell, polymorphonuclear count, neutrophil elastase, serum surfactant protein A, methane dicarboxylic aldehyde, IL-6, IL-8, and tumor necrosis factor-α at T2, T3, and T4 than those in the Control group (P<0.05). There were no significant differences in adverse reactions related specifically to blood transfusion or postoperative respiratory complications within 72 hours.

Conclusions:

Salvaged autologous blood leukocyte filtration can improve ventilation, promote gas exchange and oxygenation, and inhibit lung inflammatory and oxidative stress reactions in elderly patients undergoing lumbar spinal surgery.
The Five-Year Clinical and Angiographic Follow-Up Outcomes of Intracoronary Transfusion of Circulation-Derived CD34+ Cells for Patients With End-Stage Diffuse Coronary Artery Disease Unsuitable for Coronary Intervention—Phase I Clinical Trial
Authors: Sung Pei-Hsun , Lee Fan-Yen , Tong Meng-Shen , Chiang John Y. , Pei Sung-Nan , Ma Ming-Chun , Li Yi-Chen , Chen Yung-Lung , Wu Chiung-Jen , Sheu Jiunn-Jye , Lee Mel S. , Yip Hon-Kan
Published: February 2018

Objectives:

This study investigated the clinical and angiographic long-term outcomes of intracoronary transfusion of circulation-derived CD34+ cells for patients with end-stage diffuse coronary artery disease unsuitable for coronary intervention.

Design and Setting:

A single-center prospective randomized double-blinded phase I clinical trial. Thirty-eight patients undergoing CD34+ cell therapy were allocated into groups 1 (1.0 × 107 cells/each vessel; n = 18) and 2 (3.0 × 107 cells/each vessel; n = 20).

Patients:

Those with end-stage diffuse coronary artery disease were unsuitable for percutaneous and surgical coronary revascularization.

Interventions:

Intracoronary delivery of circulation-derived CD34+ cells.

Measurements and Main Results:

We prospectively evaluated long-term clinical and echocardiographic/angiographic outcomes between survivors and nonsurvivors. By the end of 5-year follow-up, the survival rate and major adverse cardio/cerebrovascular event were 78.9% (30/38) and 36.8% (14/38), respectively. During follow-up period, 31.6% patients (12/38) received coronary stenting for reason of sufficient target vessel size grown-up after the treatment. Endothelial function was significantly reduced in the nonsurvivors than the survivors (p = 0.039). Wimasis image analysis of angiographic findings showed that the angiogenesis was significantly and progressively increased from baseline to 1 and 5 years (all p < 0.001). The 3D echocardiography showed left ventricular ejection fraction increased from baseline to 1 year and then remained stable up to 5 years, whereas left ventricular chamber diameter exhibited an opposite pattern to left ventricular ejection fraction among the survivors. The clinical scores for angina and heart failure were significantly progressively reduced from baseline to 1 and 5 years (all p < 0.001).

Conclusions:

CD34+ cell therapy for end-stage diffuse coronary artery disease patients might contribute to persistently long-term effects on improvement of left ventricular function, angina/heart failure, and amelioration of left ventricular remodeling.
Novel Myelin Protein Zero Mutation in 3 Generations of Vermonters With Demyelinating Charcot–Marie–Tooth Disease
Authors: Lorance David K. , Mandigo Kelly A. , Hehir Michael K.
Published: February 2018

Objectives:

We report the clinical phenotype in 3 consecutive generations with demyelinating Charcot–Marie–Tooth disease that possess a novel sequence variant of myelin protein zero (MPZ).

Methods:

Family members from 3 consecutive generations were interviewed, examined, and studied with electrodiagnostic testing. Commercially available next-generation sequencing was performed for the proband. Single-gene analysis was performed for the remaining family members.

Results:

All patients demonstrated symmetric distal weakness; symmetric distal sensory loss; and diminished deep tendon reflexes. Electrodiagnostic testing was consistent with primary distal demyelination with secondary axon loss. Genetic testing identified a novel base-pair substitution of MPZ (c.314C>T), resulting in a missense variant (p.Pro105Leu).

Conclusions:

The novel MPZ base-pair substitution in this family is associated with inherited distal demyelinating neuropathy and should be reclassified as pathogenic for Charcot–Marie–Tooth.
What is in the Myopathy Literature?
Authors: Lacomis David
Published: February 2018

This review is focused on recent reports of sporadic inclusion body myositis (sIBM), myopathy in patients with human immunodeficiency virus type 1 (HIV) infection, and necrotizing autoimmune myopathy with antibodies to signal recognition particle. The sIBM articles cover associations with certain genetic polymorphisms, the possible pathogenic role of anti-cytosolic 5′-nucleotidase 1A antibody, and disease-related burden and health care costs. Another article addressed the possible pathogenic role of signal recognition particle antibody in necrotizing myopathy. A series of HIV patients with overlapping features of sIBM and polymyositis are highlighted as is a report of a patient with HIV and late-onset rod-body myopathy that may have improved with immunotherapy.
Molecular Profiling Defines Distinct Prognostic Subgroups in Childhood AML: A Report From the French ELAM02 Study Group
Authors: Marceau‐Renaut Alice , Duployez Nicolas , Ducourneau Benoît , Labopin Myriam , Petit Arnaud , Rousseau Alexandra , Geffroy Sandrine , Bucci Maxime , Cuccuini Wendy , Fenneteau Odile , Ruminy Philippe , Nelken Brigitte , Ducassou Stéphane , Gandemer Virginie , Leblanc Thierry , Michel Gérard , Bertrand Yves , Baruchel André , Leverger Guy , Preudhomme Claude , Lapillonne Hélène
Published: February 2018

Supplemental Digital Content is available in the text
TIME COURSE OF CHANGES IN OPTIC DISK NEOVASCULARIZATION AFTER A SINGLE INTRAVITREAL BEVACIZUMAB INJECTION
Authors: Falavarjani Khalil Ghasemi , Habibi Abbas , Khorasani Maryam Ashraf , Anvari Pasha , Sadda Srinivas R.
Published: February 2018

Purpose:

To evaluate the longitudinal changes in optic disk neovascularization (NVD) after intravitreal bevacizumab injection using optical coherence tomography angiography.

Methods:

In this prospective, interventional, case series, eyes with NVD secondary to diabetic retinopathy were enrolled. En face optical coherence tomography angiographic images were obtained from the optic disks before and 3 hours, 6 hours, 24 hours, 7 days, and 30 days after intravitreal bevacizumab injection. The size and flow area of the neovascularization were measured by two graders.

Results:

Eleven eyes of 9 patients with a mean age of 52.11 ± 9.48 years were included. The reduction in the NVD size and flow area was statistically significant at 24 hours, 7 days, and 30 days after injections compared with the baseline measurements (all P < 0.05). The decremental regression in the NVD size and flow area continued during the study course. The changes were not statistically significant in 3-hour and 6-hour measurements (all P > 0.05).

Conclusion:

In this study, statistically significant regression in the NVD size and flow area was observed as early as 24 hours after a single intravitreal bevacizumab injection, with a continued decreasing trend for at least a 1-month period.
CHOROIDAL NEOVASCULARIZATION ASSOCIATED WITH RETINAL PIGMENT EPITHELIAL TEAR IMAGED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY
Authors: Roizenblatt Marina , Zett Claudio , Saraiva Vinicius S. , Maia Andre , Magalhães Octaviano , Moraes Nilva S. B.
Published: February 2018

Purpose:

To report a patient presenting a retinal pigment epithelial tear in which optical coherence tomography angiography enabled the visualization of subfoveal choroidal neovascularization (CNV) not evidenced by the fluorescein angiography. She was treated with 3 monthly intravitreous anti-VEGF injections and intraretinal fluid resolution occurred.

Methods:

Observational case report.

Results:

A 62-year-old Caucasian woman presented with decreased visual acuity in the right eye for 3 months. Fundus biomicroscopy revealed a yellowish macular lesion associated with intraretinal hemorrhage. Fluorescein angiography showed a large hyperfluorescent area consistent with window defect. Optical coherence tomography showed a retinal pigment epithelial tear with subretinal fluid. However, there was no clear evidence of CNV on fluorescein angiography or OCT. Optical coherence tomography angiography confirmed the presence of an active CNV by the visualization of the neovascular network in the region corresponding to the scrolled up retinal pigment epithelium.

Conclusion:

This case report demonstrates that optical coherence tomography angiography can be useful to confirm the presence of CNV in cases where fluorescein angiography and OCT cannot establish the diagnosis. The reported case suggests the applicability of optical coherence tomography angiography in patients in whom retinal pigment epithelial tear is detected and associated CNV is suspected.
CHOROIDAL THICKNESS CHANGES IN A PATIENT DIAGNOSED WITH CENTRAL SEROUS CHORIORETINOPATHY DURING FOLLOW-UP FOR PACHYCHOROID PIGMENT EPITHELIOPATHY
Authors: Saito Wataru , Hashimoto Yuki , Hirooka Kiriko , Ishida Susumu
Published: February 2018

Purpose:

To evaluate chronological changes in choroidal thickness in a patient with pachychoroid pigment epitheliopathy (PPE) who was later diagnosed with central serous chorioretinopathy (CSC) during follow-up.

Methods:

Case report.

Results:

A 43-year-old man complained of metamorphopsia in the left eye. Funduscopy showed several punctate white subretinal lesions at the macula, but no macular serous retinal detachment in the left eye. Retinal pigment epithelium abnormality without serous retinal detachment at the macula on optical coherence tomography and choroidal vascular hyperpermeability on indocyanine green angiography suggested PPE in the left eye. Macular lesions disappeared with no treatment. Twenty-five months after the initial visit, PPE recurred in the left eye. Thirty-five months after the initial visit, the patient was diagnosed with CSC in the left eye. Macular serous retinal detachment spontaneously resolved. In the eye with PPE and CSC, the choroid was consistently thicker than that in the fellow eye during follow-up. The choroid became thicker during the alternate onsets of PPE and CSC and thinner after regression of these diseases.

Conclusion:

These results suggest that PPE and CSC overlap clinically and choroidal thickening is involved in the pathogenesis of both diseases.
RETINOCYTOMA WITH VITREOUS SEEDING: NEW INSIGHTS FROM ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY AND HIGH-RESOLUTION POSTERIOR SEGMENT ULTRASONOGRAPHY
Authors: Garoon Robert B. , Medina Carlos A. , Scelfo Christina , Harbour J. William
Published: February 2018

Purpose:

To report an atypical case of a patient with symptomatic retinocytoma associated with diffuse calcified vitreous seeds.

Methods:

Retrospective chart review.

Results:

A 46-year-old healthy woman presented with a history of floaters in the right eye for several months. She had been referred for abnormal findings in the retina and vitreous on routine examination. Visual acuity was 20/20. An incidental retinocytoma associated with extensive calcified vitreous seeding was observed. Enhanced depth optical coherence tomography showed an absence of normal retinal layers with numerous cystoid cavities throughout the lesion. High-resolution 20-MHz posterior B-scan ultrasonography demonstrated that the calcified vitreous seeds emanated from the peaked portion of the retinal tumor.

Conclusion:

Calcified vitreous seeding is a rare finding associated with retinocytomas. Enhanced depth imaging optical coherence tomography and high-resolution B-scan ultrasonography may be useful tools in the diagnosis of this uncommon retinal tumor.
Murine Typhus in South Texas Children
Authors: Howard Ashley , Fergie Jaime
Published: February 2018

Background:

Murine typhus is a zoonotic infection caused by Rickettsia typhi that remains endemic in South Texas. In 2003, only 9 Texas counties reported murine typhus compared with 41 counties in 2013.

Methods:

A retrospective study of children discharged with a confirmed diagnosis of murine typhus from Driscoll Children’s Hospital between January 1998 and September 2016.

Results:

Two hundred thirteen children (113 female) 3 months through 19 years of age (mean, 11.2 ± 4.5 years) were identified. Cases occurred throughout the year. Children were admitted after a mean of 7.7 ± 5.3 days of fever. The most common symptoms were fever (100%), poor appetite (71.9%), malaise/fatigue (69.0%) and headache (67.6%). The most common laboratory abnormalities were elevated C-reactive protein, hypoalbuminemia, elevated erythrocyte sedimentation rate, elevated transaminases and elevated band count with normal total white blood cell count. Children defervesced in a mean of 31.87 ± 21.36 hours after initiation of doxycycline. Hospitalization lasted for a mean of 2.7 ± 1.8 days when children were administered doxycycline within 24 hours of admission compared with, 4.1 ± 1.8 days, P ≤ 0.0001 when started later. Eleven patients (5.1%) were admitted to the pediatric intensive care unit and were older, P = 0.0009. No children died.

Conclusions:

Murine typhus is endemic in South Texas. Children who were treated earlier with doxycycline had a shorter hospitalization than were those who began therapy later. Recognition of murine typhus is important to prevent delay in treatment and development of complications.
Genetic variation in P2RX7 and pain tolerance
Authors: Kambur Oleg , Kaunisto Mari A. , Winsvold Bendik S. , Wilsgaard Tom , Stubhaug Audun , Zwart John A. , Kalso Eija , Nielsen Christopher S.
Published: February 2018

P2X7 is a nonselective cation channel activated by extracellular ATP. P2X7 activation contributes to the proinflammatory response to injury or bacterial invasion and mediates apoptosis. Recently, P2X7 function has been linked to chronic inflammatory and neuropathic pain. P2X7 may contribute to pain modulation both by effects on peripheral tissue injury underlying clinical pain states, and through alterations in central nervous system processing, as suggested by animal models. To further test its role in pain sensitivity, we examined whether variation within the P2RX7 gene, which encodes the P2X7 receptor, was associated with experimentally induced pain in human patients. Experimental pain was assessed in Tromsø 6, a longitudinal and cross-sectional population-based study (N = 3016), and the BrePainGen cohort, consisting of patients who underwent breast cancer surgery (N = 831). For both cohorts, experimental pain intensity and tolerance were assessed with the cold-pressor test. In addition, multisite chronic pain was assessed in Tromsø 6 and pain intensity 1 week after surgery was assessed in BrePainGen. We tested whether the single-nucleotide polymorphism rs7958311, previously implicated in clinical pain, was associated with experimental and clinical pain phenotypes. In addition, we examined effects of single-nucleotide polymorphisms rs208294 and rs208296, for which previous results have been equivocal. Rs7958311 was associated with experimental pain intensity in the meta-analysis of both cohorts. Significant associations were also found for multisite pain and postoperative pain. Our results strengthen the existing evidence and suggest that P2X7 and genetic variation in the P2RX7-gene may be involved in the modulation of human pain sensitivity.
Characteristics of patients with hepatitis C virus-related chronic liver diseases just before the era of oral direct-acting antiviral therapy in Italy
Authors: Sagnelli Evangelista , Stroffolini Tommaso , Sagnelli Caterina , Cacopardo Bruno , Andriulli Angelo , Babudieri Sergio , Coppola Nicola , Gaeta Giovanni B. , Almasio Piero L.
Published: February 2018

Background

In 2017, oral direct-acting antiviral (DAA) therapy for chronic hepatitis C virus (HCV) infection became available free of charge for all HCV-RNA-positive patients, irrespective of their fibrosis stage.

Aim

The aim of this study was to evaluate the characteristics of HCV-related chronic liver disease (CLD) in Italy just before the introduction of DAA therapy.

Patients and methods

Patients with CLD were enrolled in two national surveys conducted in 2001 and in 2014. The two surveys prospectively enrolled patients aged older than 18 years referring to Italian liver units throughout the country using a similar clinical approach and analytical methods.

Results

Out of the 12 564 patients enrolled, 8447 (67.3%) were anti-HCV-positive, with a decreasing trend from 69.0% in 2001 to 60.4% in 2014. During this period, an increasing trend over time was observed in the mean age of patients (55.6 vs. 59.1 years; P<0.01), in the proportion of patients with liver cirrhosis (19.4 vs. 28.2%; P<0.01), and in the circulation of genotype 4 (0 vs. 6.1%). The multiple logistic analysis showed that age older than 60 years, birth in southern Italy, and multiple etiology (HCV+hepatitis B virus or HCV+alcohol) are independent predictors of a likelihood of liver cirrhosis, whereas a higher level of education plays a protective role (odds ratio: 0.65; 95% confidence interval=0.57–0.76).

Conclusion

Currently, in Italy, chronic HCV infection plays a decreasing role in CLD, showing a shift toward older age groups and a more severe disease stage. These data, relating to just before the era of DAA therapy for this infection, represent up-to-date reference data for evaluating the effectiveness of DAAs in the future.
Clinical manifestations of sickle cell disease in India: misconceptions and reality
Authors: Jain Dipty , Mohanty Dipika
Published: February 2018

Purpose of review

In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India.

Recent findings

The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India. Strategies for improving quality of life and controlling of SCD have been suggested. Certain factors other than genetics also play an important role in clinical manifestation of the disorder.

The clinical diversity of SCD is described. The natural history of SCD in India is unfolding from newborn screening programme. The use of low-dose hydroxy urea therapy both in adults and children has brought down the incidences of crisis and provides great relief to the patients. The tailor-made programme for India as regards the control and management has been discussed.
Genetic Variants Associated With Obesity and Insulin Resistance in Hispanic Boys With Nonalcoholic Fatty Liver Disease
Authors: Rausch John C. , Lavine Joel E. , Chalasani Naga , Guo Xiuqing , Kwon Soonil , Schwimmer Jeffrey B. , Molleston Jean P. , Loomba Rohit , Brunt Elizabeth M. , Chen Yii‐Der Ida , Goodarzi Mark O. , Taylor Kent D. , Yates Katherine P. , Rotter Jerome I. ,   NASH Clinical Research Network
Published: February 2018

Background and Objectives:

Nonalcoholic fatty liver disease (NAFLD) disproportionately affects Hispanic boys. Further, obesity and insulin resistance are major risk factors for NAFLD. No gene localization studies had been performed on children with biopsy‐proven NAFLD. This study aims to identify genomic variants associated with increased adiposity and insulin resistance in a population of children with varying histologic severity of NAFLD.

Methods:

We conducted a genome‐wide association scan (GWAS) including 624,297 single‐nucleotide polymorphisms (SNPs) distributed among all 22 autosomal chromosomes in 234 Hispanic boys (up to 18 years of age) who were consecutively recruited in a prospective cohort study in the Nonalcoholic Steatohepatitis Clinical Research Network Studies. Traits were examined quantitatively using linear regression. SNPs with P value <10−5 and a minor allele frequency >5% were considered potentially significant.

Results:

Evaluated subjects had a median age of 12.0 years, body mass index (BMI) of 31.4, and hemoglobin A1C (Hgb A1C) of 5.3. The prevalence of NAFL, borderline NASH, and definite NASH were 23%, 53%, and 22%, respectively. The GWAS identified 10 SNPs that were associated with BMI z score, 6 within chromosome 2, and 1 within CAMK1D, which has a potential role in liver gluconeogenesis. In addition, the GWAS identified 9 novel variants associated with insulin resistance: HOMA‐IR (6) and HbA1c (3).

Conclusions:

This study of Hispanic boys with biopsy‐proven NAFLD with increased risk for the metabolic syndrome revealed novel genetic variants that are associated with obesity and insulin resistance.
Autoimmune Hepatitis in Children and Adolescents
Authors: Trevizoli Isadora de Carvalho , Pinedo Carolina Silva , Teles Vanessa Oliveira , Seixas Renata Belém Pessoa de Melo , de Carvalho Elisa
Published: February 2018

Objectives:

The aim of this study was to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health‐related quality of life (HRQoL).

Methods:

The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In addition, a questionnaire was applied which included signs, symptoms, and use of medications.

Results:

A total of 43 patients (mean age, 15.0 ± 3.9 years; 65.1% girls; 90.7% with type 1 AIH) was evaluated. Advanced liver disease was present in 30.2%, and 18.6% had sclerosing cholangitis. Treatment was effective in 93.1% of patients. The lowest HRQoL scores were associated with the school (67.7), emotional (68.2), and psychosocial (75.5) domains. Compared with healthy children, patients presented lower scores on the total, psychosocial, emotional, and school domains (P < 0.05). The presence of symptoms (β = 0.39, P < 0.01), extrahepatic autoimmune diseases (β = 0.27, P < 0.05), and a dislike of taking medication (β = 0.40, P < 0.01) negatively affected the psychosocial PedsQL 4.0 score, and the presence of symptoms (β = 0.40, P < 0.01) negatively affected the total PedsQL 4.0 score.

Conclusions:

AIH has a significant and negative effect on HRQoL among children and adolescents. The presence of symptoms, extrahepatic autoimmune diseases, and a dislike of taking medication were associated with the worsening of HRQoL. Providers should work with professionals trained to improve QoL to help improve treatment adherence and disease outcomes.
Clinical and Imaging Predictors of Surgical Splenorenal Shunt Dysfunction in Pediatric Patients
Authors: Woerner Andrew , Shivaram Giri , Koo Kevin S.H. , Hsu Evelyn K. , Dick Andre A.S. , Monroe Eric J.
Published: February 2018

Purpose:

Few established criteria exist to prompt angiographic evaluation and intervention for surgically created splenorenal shunts (SRS). Clinical and Doppler ultrasound (DUS) imaging predictors of shunt dysfunction were evaluated in this retrospective study.

Materials and Methods:

Consecutive patients undergoing SRS angiography over a 10‐year period were retrospectively identified. Preangiography platelet count and DUS measurements of spleen diameter, maximum splenic vein velocity, and maximum shunt velocity were assessed and compared to findings at subsequent catheter angiography.

Results:

Twenty‐six SRS angiograms were performed in 16 patients. Two of the 26 procedures were excluded from analysis due to insufficient baseline preangiography clinical and DUS data. In the remaining 24 cases, significant stenosis/occlusion was confirmed at angiography in 20, whereas wide patency was seen in 4. For the 20 cases of angiographically confirmed significant stenosis/occlusion, when compared to baseline post‐SRS creation to immediate preangiography evaluation there was a greater decrease in platelet count (−51.8% vs −19.4%), a greater increase in spleen diameter (+13.4% vs +3.7%), a greater increase in maximum shunt velocity (+74.7% vs +59.7%), and a greater decrease in splenic vein velocity (−25.0% vs −18.5%).

Conclusion:

Clinical evidence of splenic sequestration and DUS finding of increased maximum shunt velocity correlate with angiographic findings of SRS dysfunction and could be used to help predict the need for shunt intervention.
Rectal Picking Masquerading as Inflammatory Bowel Disease in Prader‐Willi Syndrome
Authors: Salehi Parisa , Lee Dale , Ambartsumyan Lusine , Sikka Natalie , Scheimann Ann O.
Published: February 2018

Prader‐Willi syndrome (PWS) is a genetic syndrome in which individuals have multisystem medical challenges. Gastroenterological difficulties in the syndrome include decreased vomiting, constipation, delayed gastric emptying, delayed colonic transit, dysphagia, increased choking, and increased risk of gastric dilation and rupture. In addition, self‐injurious behavior such as rectal picking may be present and severe enough to lead to rectal ulceration and bleeding. Many patients have extensive gastroenterological workup and treatment before their ultimate diagnosis of severe rectal picking. We describe 4 new cases of rectal picking in individuals with PWS leading to rectal bleeding and ulceration as well as a review of the literature of prior cases of severe rectal picking in PWS and potential treatment options. It is important to recognize these cases early in order to prevent unnecessary treatments and implement appropriate behavioral interventions.
Participating in a Multisite Study Exploring Operational Failures Encountered by Frontline Nurses
Authors: Melnyk Halia , Rosenfeld Peri , Glassman Kimberly S.
Published: February 2018

This article describes our experience participating in a multisite collaborative study involving frontline nurses and operational failures (OFs). We encountered a range of challenges conducting the study as proposed by the study's coordinating center (CC), which hindered our ability to meet our goals and objectives. We identified 3 general areas in which our expectations and actual experience diverged: 1) research resources, design, and methods; 2) CC communications and deliverables; and 3) influencing organizational change. Nurse executives considering collaborative research or assessing methods to engage clinical nurses in organizational improvements will benefit from our experience.
Developing a Topology of Syphilis in the United States
Authors: Bernstein Kyle T. , Grey Jeremy , Bolan Gail , Aral Sevgi O.
Published: February 2018

Background

In the United States, reported rates of syphilis continue to increase. Co-occurring epidemics of syphilis among men who have sex with men (MSM) and heterosexual populations create challenges for the prioritization of resources and the implementation of context-specific interventions.

Methods

State was the unit of analysis and was restricted to the 44 states with the most complete data of sex or sex partners for their reported adult syphilis cases. States were classified as high, medium, or low for reported congenital syphilis (CS) and MSM primary and secondary (P&S) syphilis rates. Average values of a range of ecologic state level variables were examined among the 9 categories created through the cross-tabulation of CS and MSM P&S syphilis rates. Patterns among ecologic factors were assessed across the 9 categories of states' syphilis rates.

Results

Among the 44 states categorized, 4 states had high rates of both CS and MSM P&S syphilis in 2015, whereas 12 states fell into the medium/medium category and 7 into the low category. Six states had high CS and medium MSM syphilis and 4 states had medium CS but high MSM syphilis. Several area-level factors, including violent crime, poverty, insurance status, household structure and income, showed qualitative patterns with higher rates of CS and MSM P&S syphilis. Higher proportions of urban population were found among states with higher CS rates; no trend was seen with respect to urbanity and MSM P&S syphilis.

Conclusions

Several area-level factors were associated with CS and MSM P&S syphilis in similar ways, whereas other ecologic factors functioned differently with respect to the 2 epidemics. Explorations of community and area-level factors may shed light on novel opportunities for population specific prevention of syphilis.
Fatal Diesel Poisoning
Authors: Srinivasa Murthy Abilash , Das Siddhartha , Bheemanathi Hanuman Srinivas
Published: February 2018

Hydrocarbons are volatile substances that are used in routine life activities for cooking or as automobile fuel. Diesel is one of the commonly used automobile fuels obtained from crude oil. Death due to poisoning by diesel is rarely reported. Most commonly affected is the respiratory system either after aspiration or ingestion. The most common presentation is chemical pneumonitis or aspiration pneumonitis from which patient usually recovers. Gas chromatography techniques help in the detection of volatile substances like diesel. Here, we report a rare case of fatal pediatric accidental diesel poisoning.
Enhanced Recovery Pathway in Microvascular Autologous Tissue-Based Breast Reconstruction: Should It Become the Standard of Care?
Authors: Kaoutzanis Christodoulos , Ganesh Kumar Nishant , O’Neill Dillon , Wormer Blair , Winocour Julian , Layliev John , McEvoy Matthew , King Adam , Braun Stephane A. , Higdon K. Kye
Published: February 2018

Background:

Enhanced recovery pathway programs have demonstrated improved perioperative care and shorter length of hospital stay in several surgical disciplines. The purpose of this study was to compare outcomes of patients undergoing autologous tissue–based breast reconstruction before and after the implementation of an enhanced recovery pathway program.

Methods:

The authors retrospectively reviewed consecutive patients who underwent autologous tissue–based breast reconstruction performed by two surgeons before and after the implementation of the enhanced recovery pathway at a university center over a 3-year period. Patient demographics, perioperative data, and 45-day postoperative outcomes were compared between the traditional standard of care (pre–enhanced recovery pathway) and enhanced recovery pathway patients. Multivariate logistic regression was performed to identify risk factors for length of hospital stay. Cost analysis was performed.

Results:

Between April of 2014 and January of 2017, 100 consecutive women were identified, with 50 women in each group. Both groups had similar demographics, comorbidities, and reconstruction types. Postoperatively, the enhanced recovery pathway cohort used significantly less opiate and more acetaminophen compared with the traditional standard of care cohort. Median length of stay was shorter in the enhanced recovery pathway cohort, which resulted in an extrapolated $279,258 savings from freeing up inpatient beds and increase in overall contribution margins of $189,342. Participation in an enhanced recovery pathway program and lower total morphine-equivalent use were independent predictors for decreased length of hospital stay. Overall 45-day major complication rates, partial flap loss rates, emergency room visits, hospital readmissions, and unplanned reoperations were similar between the two groups.

Conclusion:

Enhanced recovery pathway program implementation should be considered as the standard approach for perioperative care in autologous tissue–based breast reconstruction because it does not affect morbidity and is associated with accelerated recovery with reduced postoperative opiate use and decreased length of hospital stay, leading to downstream health care cost savings.

CLINICAL QUESTION/LEVEL OF EVIDENCE:

Therapeutic, III.
Unusual Case of Diffuse Penile Metastasis of Prostate Cancer on 68Ga PSMA PET/CT Imaging and 177Lu PSMA Posttherapy Scintigraphy
Authors: Vadi Shelvin Kumar , Kumar Rajender , Mittal Bhagwant Rai , Parihar Ashwin Singh , Singh Shrawan Kumar
Published: February 2018

An 85-year-old man with prostate cancer for metastatic workup underwent 68Gallium Prostate-Specific Membrane Antigen (68Ga-PSMA) PET/CT (68Ga-PSMA PET/CT), which revealed unusual tracer uptake in the shaft and glans of penis as well as multiple systemic metastases in liver, skeletal, and lymph nodes. The penile lesion was proved to be metastatic adenocarcinoma from prostate on fine needle aspiration cytology. The patient underwent 177Lutetium (177Lu)-labeled PSMA radioligand therapy, which also revealed diffuse tracer uptake in the penile shaft as well as other metastatic sites.
Is There Any Potential of FDG PET/CT in Monitoring Disease Activity in Familial Hemophagocytic Lymphohistiocytosis?
Authors: Tang YongXiang , Tan HongPei , Hu Shuo
Published: February 2018

FDG PET/CT was performed in a 30-year-old woman to detect the source of fever of unknown origin. The images showed widespread abnormal activity, consistent with lymphoma. However, lymph node biopsy revealed only inflammation, and a diagnosis of familial hemophagocytic lymphohistiocytosis was eventually made after familial hemophagocytic lymphohistiocytosis–specific gene was detected. After proper therapy, a follow-up FDG PET/CT examination 2 months later showed resolution of most of the previously seen lesions.
Active Bone Marrow With Focal FDG Accumulation Mimicking Bone Metastasis With a Case of Early Esophageal Cancer
Authors: Okuyama Chio , Sasaki Naomi , Nishimura Motoki , Matsushima Shigenori , Yoshimatsu Rika
Published: February 2018

A 68-year-old man underwent 18F-FDG PET/CT for the staging of esophageal carcinoma discovered by a medical checkup. Increased focal accumulation in some vertebrae, right humerus, and right femoral bone was noted on FDG-PET, whereas CT showed relatively high attenuation, and MRI showed hypointense lesions on T1- and T2-weighted imaging. A bone biopsy revealed mildly hypercellular bone marrow in the thoracic spine with FDG accumulation and markedly hypocellular bone marrow in the pelvic bone without an increased uptake. 111InCl scintigraphy showed a similar distribution and confirmed the diagnosis of bone marrow reconversion.
Amyloid PET Positivity in Different Primary Progressive Aphasia Phenotypes
Authors: Beaufils Emilie , Vercouillie Johnny , Vierron Emilie , Cottier Jean-Philippe , Camus Vincent , Mondon Karl , Guilloteau Denis , Hommet Caroline , Ribeiro Maria Joao
Published: February 2018

Purpose

Primary progressive aphasia (PPA) is a neurological syndrome in which language functions become progressively impaired with relative sparing of memory and other instrumental functions. The pathologic causes of PPA are heterogeneous, but studies suggest that logopenic PPA (LPA) is underpinned by Alzheimer disease (AD) pathology in a high proportion of cases. The purposes of this descriptive and retrospective study were to characterize 18F-florbetapir PET imaging in a group of patients with a clinical syndrome of PPA, to determine the value of clinical characterization based on language phenotype in predicting the underlying pathology of PPA with 18F-florbetapir, and to quantify amyloid load in PPA subjects classified as “positive” 18F-florbetapir scans. Then, we compare the quantification and distribution of 18F-florbetapir uptake with those of typical, predominantly amnestic AD patients.

Methods

We conducted a PET study with 18F-florbetapir in a cohort of 12 right-handed patients diagnosed with PPA: 3 patients with semantic-variant PPA, 5 with nonfluent PPA, 1 with LPA, and 3 unclassifiable patients. We evaluated amyloid deposition between APP groups and 11 patients with typical amnestic AD.

Results

Among the 12 patients with PPA syndrome, 8 (66.7%) were considered as amyloid positive. One of the 3 patients with semantic-variant PPA was 18F-florbetapir positive. In contrast, 4 of the 5 nonfluent-variant PPA, 2 of the 3 unclassifiable cases and the single patient with LPA were 18F-florbetapir positive. A significantly higher 18F-florbetapir uptake was observed in PPA 18F-florbetapir–positive patients compared with typical AD patients. This difference was observed in all regions of interest, except in posterior cingulate and temporal cortex.

Conclusions

These results suggest that 18F-florbetapir PET may be useful in a routine clinical procedure to improve the reliability of identifying AD pathology in patients with PPA syndrome, with different clinical subtypes of the PPA syndrome.
Key Components of the Safe Surgical Ward
Authors: Hassen Yasmin A. M. , Johnston Maximilian J. , Singh Pritam , Pucher Philip H. , Darzi Ara
Published: February 2018

Objective:

The aim of this study was to prioritize key factors contributing to safety on the surgical ward

Background:

There is a variation in the quality and safety of postoperative care between institutions. These variations may be attributed to a combination of process-related issues and structural factors. The aim of this study is to reach a consensus, by means of Delphi methodology, on the most influential of these components that may determine safety in this environment.

Methods:

The Delphi questionnaire was delivered via an online questionnaire platform. The panel were blinded. An international panel of safety experts, both clinical and nonclinical, and safety advocates participated. Individuals were selected according to their expertise and extent of involvement in patient safety research, regulation, or patient advocacy.

Results:

Experts in patient safety from the UK, Europe, North America, and Australia participated. The panel identified the response to a deteriorating patient and the care of outlier patients as error-prone processes. Prioritized structural factors included organizational and environmental considerations such as use of temporary staff, out-of-hours reduction in services, ward cleanliness, and features of layout. The latter includes dedicated areas for medication preparation and adequate space around the patient for care delivery. Potential quality markers for safe care that achieved the highest consensus include leadership, visibility between patients and nurses, and nursing team skill mix and staffing levels.

Conclusion:

International consensus was achieved for a number of factors across process-related and structural themes that may influence safety in the postoperative environment. These should be championed and prioritized for future improvements in patient safety at the ward-level.
Improved Glucose Tolerance in a Kidney Transplant Recipient With Type 2 Diabetes Mellitus After Switching From Tacrolimus To Belatacept: A Case Report and Review of Potential Mechanisms
Authors: de Graav Gretchen N. , van der Zwan Marieke , Baan Carla C. , Janssen Joop A.M.J.L. , Hesselink Dennis A.
Published: February 2018

The introduction of immunosuppressant belatacept, an inhibitor of the CD28-80/86 pathway, has improved 1-year outcomes in kidney transplant recipients with preexistent diabetes mellitus and has also reduced the risk of posttransplant diabetes mellitus. So far, no studies have compared a tacrolimus-based with a belatacept-based immunosuppressive regimen with regard to improving glucose tolerance after kidney transplantation. Here, we present the case of a 54-year-old man with type 2 diabetes mellitus who was converted from belatacept to tacrolimus 1 year after a successful kidney transplantation. Thereafter, he quickly developed severe hyperglycemia, and administration of insulin was needed to improve metabolic control. Six months after this episode, he was converted back to belatacept because of nausea, diarrhea, and hyperglycemia. After switching back to belatacept and within 4 days after stopping tacrolimus glucose tolerance improved and insulin therapy could be discontinued. Although belatacept is considered less diabetogenic than tacrolimus, the rapid improvement of glucose tolerance after switching to belatacept is remarkable. In this article, the potential mechanisms of this observation are discussed.
Emerging targets for reprograming the immune response to promote repair and recovery of function after spinal cord injury
Authors: Brennan Faith H. , Popovich Phillip G.
Published: February 2018

Purpose of review

In adult mammals, a traumatic spinal cord injury (SCI) elicits a chronic unregulated neuroinflammatory response accompanied by seemingly paradoxical suppression of systemic immunity. These SCI-induced changes in immune function contribute to poor neurological outcomes and enhanced morbidity or mortality. Nonspecific anti-inflammatory or proinflammatory therapies are ineffective and can even worsen outcomes. Therefore, recent experimental SCI research has advanced the understanding of how neuroimmune cross-talk contributes to spinal cord and systemic pathology.

Recent findings

It is now appreciated that the immune response caused by injury to the brain or spinal cord encompasses heterogeneous elements that can drive events on the spectrum between exacerbating pathology and promoting tissue repair, within the spinal cord and throughout the body. Recent novel discoveries regarding the role and regulation of soluble factors, monocytes/macrophages, microRNAs, lymphocytes and systemic immune function are highlighted in this review.

A more nuanced understanding of how the immune system responds and reacts to nervous system injury will present an array of novel therapeutic opportunities for clinical SCI and other forms of neurotrauma.
Mechanisms for lesion localization in neuromyelitis optica spectrum disorders
Authors: Bradl Monika , Reindl Markus , Lassmann Hans
Published: February 2018

Purpose of review

Neuromyelitis optica spectrum disorders (NMOSD) are severe inflammatory diseases of the central nervous system (CNS), with the presence of aquaporin 4 (AQP4)-specific serum antibodies in the vast majority of patients, and with the presence of myelin oligodendrocyte glycoprotein (MOG)-specific antibodies in approximately 40% of all AQP4-antibody negative NMOSD patients. Despite differences in antigen recognition, the preferred sites of lesions are similar in both groups of patients: They localize to the spinal cord and to the anterior visual pathway including retina, optic nerves, chiasm, and optic tracts, and – to lesser extent – also to certain predilection sites in the brain.

Recent findings

The involvement of T cells in the formation of NMOSD lesions has been challenged for quite some time. However, several recent findings demonstrate the key role of T cells for lesion formation and localization. Studies on the evolution of lesions in the spinal cord of NMOSD patients revealed a striking similarity of early NMOSD lesions with those observed in corresponding T-cell-induced animal models, both in lesion formation and in lesion localization. Studies on retinal abnormalities in NMOSD patients and corresponding animals revealed the importance of T cells for the very early stages of retinal lesions which eventually culminate in damage to Müller cells and to the retinal nerve fiber layer. Finally, a study on cerebrospinal fluid (CSF) barrier pathology demonstrated that NMOSD immunopathology extends beyond perivascular astrocytic foot processes to include the pia, the ependyma, and the choroid plexus, and that diffusion of antibodies from the CSF could further influence lesion formation in NMOSD patients.

The pathological changes observed in AQP4-antibody positive and MOG-antibody positive NMOSD patients are strikingly similar to those found in corresponding animal models, and many mechanisms which determine lesion localization in experimental animals seem to closely reflect the human situation.
Tendon healing affects the multiscale mechanical, structural and compositional response of tendon to quasi-static tensile loading
Authors: Freedman Benjamin R. , Rodriguez Ashley B. , Hillin Cody D. , Weiss Stephanie N. , Han Biao , Han Lin , Soslowsky Louis J.
Published: February 2018

Tendon experiences a variety of multiscale changes to its extracellular matrix during mechanical loading at the fascicle, fibre and fibril levels. For example, tensile loading of tendon increases its stiffness, with organization of collagen fibres, and increases cell strain in the direction of loading. Although applied macroscale strains correlate to cell and nuclear strains in uninjured tendon, the multiscale response during tendon healing remains unknown and may affect cell mechanosensing and response. Therefore, this study evaluated multiscale structure–function mechanisms in response to quasi-static tensile loading in uninjured and healing tendons. We found that tendon healing affected the macroscale mechanical and structural response to mechanical loading, evidenced by decreases in strain stiffening and collagen fibre realignment. At the micro- and nanoscales, healing resulted in increased collagen fibre disorganization, nuclear disorganization, decreased change in nuclear aspect ratio with loading, and decreased indentation modulus compared to uninjured tendons. Taken together, this work supports a new concept of nuclear strain transfer attenuation during tendon healing and identifies several multiscale properties that may contribute. Our work also provides benchmarks for the biomechanical microenvironments that tendon cells may experience following cell delivery therapies.