bluuebunny/crossref_metadata_2025_split huggingface dataset:
Complete remission of both immunoglobulin light chain amyloidosis and psoriasis after autologous hematopoietic stem cell transplantation
Authors: Chen Wencui , Ren Guisheng , Zuo Ke , Huang Xianghua 
Published: December 2018

Abstract

Rationale:
Immunoglobulin light chain amyloidosis (AL amyloidosis) is characterized by the deposition of abnormal amyloid protein produced by a pathological plasma cell clone in various organs and soft tissues. Hematopoietic stem cell transplantation (HSCT) is an effective way to treat AL amyloidosis. Psoriasis is a common autoimmune disease (AID) and HSCT is a potential treatment for severe AIDs. We report a rare case of AL amyloidosis coincidence with psoriasis obtained continuous complete remission of the 2 diseases by autologous hematopoietic stem cell transplantation (ASCT).

Patient concerns:
A 58-year-old man with a 30-year history of psoriasis complaining of edema and hypotension for 2 weeks was referred to our institution. His urine protein was quantified 2.83 g/day, without hematuria and decrease of glomerular filtration rate.

Diagnosis:
Renal biopsy confirmed AL amyloidosis and multiple myeloma was excluded by bone marrow cytomorphologic examination.

Interventions:
Chemotherapy regimen based on bortezomib and thalidomide had achieved hematologic partial remission, but the kidney had no response and psoriasis was still active. Furthermore, he received a standard myeloablative conditioning with high dose melphalan followed by ASCT.

Outcomes:
The erythema with slivery scales of psoriasis vulgaris gradually improved and almost disappeared after granulocyte implantation. He obtained persistent hematological complete remission, organ response and recovery of psoriasis.

Lessons:
We report a rare case of AL amyloidosis coincidence with psoriasis treated by ASCT. The outcome of this patient indicated that ASCT has therapeutic values both in AL amyloidosis and AIDs.
Comparison of efficacy and safety of combination therapy with statins and omega-3 fatty acids versus statin monotherapy in patients with dyslipidemia
Authors: Choi Hye Duck , Chae Seung Min 
Published: December 2018

Abstract

Objective:
Dyslipidemia is a major risk factor for the development of cardiovascular disease. Both statins and omega-3 fatty acids demonstrate beneficial effects on lipid concentrations. The goal was to evaluate the safety and efficacy of combination therapy with statins and omega-3 fatty acids.

Methods:
We performed a systematic review and meta-analysis of published data to compare the safety and efficacy of combination therapy with statins and omega-3 fatty acids versus statin monotherapy in patients with dyslipidemia. Six articles were assessed in the present meta-analysis (quantitative assessment) and qualitative assessment.

Results:
In terms of efficacy, the combination treatment afforded a significantly greater reduction in total cholesterol/high-density lipoprotein cholesterol than statin alone did [standard difference in means = −0.215; 95% confidence interval (CI) −0.359–−0.071]. However, there was no significant difference in low-density lipoprotein (LDL) cholesterol between the 2 groups. Qualitative assessment of other lipid parameters was performed. Combination therapy with statins and omega-3 fatty acids was generally more effective on lipid concentration than statin monotherapy. In terms of safety, there were no significant differences in total adverse events between the 2 groups. Gastrointestinal adverse events were found to be significantly increased in patients receiving combination therapy using the fixed-effects model (relative risk = 0.547; 95% CI 0.368–0.812).

Conclusions:
We suggest that combination therapy with statins and omega-3 fatty acids enhances lipid profile, except LDL cholesterol, compared with statin monotherapy. Nevertheless, statin and omega-3 fatty acid combination should be cautiously recommended, taking into account the clinical importance of LDL cholesterol and safety issues associated with their concomitant use.
Sudden unilateral vision loss in an adult with Behcet disease
Authors: Gao Yunxia , Lyu Shuyuan , Bao Li , Zhang Ming 
Published: December 2018

Abstract

Rationale:
Behcet disease is an autoimmune vasculitis, involving multiple systems and organs. It is often chronic and recurrent, and involves all levels of arteries and veins.

Patient concerns:
A 40-year-old Chinese male with sudden vision loss in his right eye for 3 days was reported.

Diagnoses:
The patient was diagnosed as bilateral uveitis (obstructive retinal vasculitis in the right eye) and Behcet disease.

Interventions:
The patient was given topical corticosteroids and compound tropicamide eye drops in our department. In addition, he was also hospitalized in the Rheumatology Department, and received systemic infusion of methylprednisolone and cyclophosphamide.

Outcomes:
Best corrected visual acuity vision acuity of the left eye was 20/20 and that of the right eye was no light perception after 2 weeks. During the telephonic follow-up after 1 year, the patient reported 2 recurrent episodes of uveitis in the left eye, which improved after treatment at the local hospital.

Lessons:
It cautions ophthalmologists of the high possibility of vasculitis in young and middle-aged patients with retinal vascular occlusion. It also reminds the ophthalmologists investigate systemic diseases, and obtain detailed medical history, not just depending on specific laboratory findings. This case further suggests that Behcet disease has diverse manifestations, complex diagnosis, and severe lesions, and the recurrence rate must be controlled to minimize irreversible consequences such as sudden vision loss during recurrence.
Racial/ethnic differences in reporting versus rating of healthcare experiences
Authors: Chung Sukyung , Mujal Gabriella , Liang Lily , Palaniappan Latha P. , Frosch Dominick L. 
Published: December 2018

Abstract
Asians are reported to have poorer healthcare experience than non-Hispanic Whites (NHWs), but the sources of the differences are not understood. One explanation is Asian's reluctance to choose extreme responses in survey. We thus sought to compare NHW-Asian differences in responses to healthcare experience surveys when asked to report versus rate their experiences. Patients of an outpatient care system in 2013 to 2014 in the United States were studied. Patient experience surveys were sent after randomly selected clinic visits. Responses from 6 major Asian subgroups and NHWs were included (N = 61,115). The surveys used a combined questionnaire of Clinician and Group Consumer Assessment of Healthcare Providers and Systems (CG-CAHPS) and Press Ganey surveys. CG-CAHPS questions are framed as “reporting” and Press Ganey questions as “rating” of experiences. We compared the proportion of favorable (or top box) responses to 2 related questions, one from CG-CAHPS and another from Press Ganey, and assessed racial/ethnic differences when using each of the 2 related questions, using a Pearson chi-squared test for independence. All Asian subgroups were less likely to select top box than NHWs for all questions. The Asian-NHW differences in "rating” questions were larger than the difference in related “reporting” questions. Of those who chose top box to CG-CAHPS questions (e.g., “Yes” on a question asking “Waited < 15 minutes”), their responses to related Press Ganey questions varied widely: 47% to 57% of Asian subgroups versus 67% of NHWs rated wait time as “Very good.” The extent of racial/ethnic differences in patient-reported experiences varies based on how questions are framed. The observed poorer experiences by Asians are in part explained by their worse rating of similar objectively measurable experiences.
The clinical impact of patients with bloodstream infection with different groups of Viridans group streptococci by using matrix-assisted laser desorption ionization–time of flight mass spectrometry (MALDI-TOF MS)
Authors: Su Ting-Yi , Lee Ming-Hsun , Huang Ching-Tai , Liu Tsui-Ping , Lu Jang-Jih 
Published: December 2018

Abstract
The accuracy of matrix-assisted laser desorption ionization–time of flight mass spectrometry (MALDI-TOF MS) for identifying viridans group streptococcus (VGS) was improving. However, the clinical impact of identifying VGS had not been well recognized. Our study had comprehensively studied the clinical manifestations and outcome of VGS blood stream infection by using MALDI-TOF MS for identification.
This retrospective study enrolled 312 adult patients with a monomicrobial blood culture positive for VGS. Blood culture was examined through MALDI-TOF MS.
The most common VGS species were the Streptococcus anginosus group (38.8%) and Streptococcus mitis group (22.8%). Most species showed resistance to erythromycin (35.6%), followed by clindamycin (25.3%) and penicillin (12.5%). Skin and soft tissue infection and biliary tract infection were significantly related to S. anginosus group bacteremia (P = .001 and P = .005, respectively). S. mitis group bacteremia was related to infective endocarditis and bacteremia with febrile neutropenia (P = .005 and P < .001, respectively). Infective endocarditis was also more likely associated with S. sanguinis group bacteremia (P = .009). S. anginosus group had less resistance rate to ampicillin, erythromycin, clindamycin, and ceftriaxone (P = .019, <.001, .001, and .046, respectively). A more staying in intensive care unit, underlying solid organ malignancy, and a shorter treatment duration were independent risk factors for 30-day mortality. This study comprehensively evaluated different VGS group and their clinical manifestations, infection sources, concomitant diseases, treatments, and outcomes. Categorizing VGS into different groups by MALDI-TOF MS could help clinical physicians well understand their clinical presentations.
Long-term follow-up of endovascular repair of iatrogenic superior vena cava injury
Authors: Altuwaijri Talal , Nouh Thamer , Alburakan Ahmed , Altoijry Abdulmajeed 
Published: December 2018

Abstract

Rationale:
This report aimed to present the long-term follow-up results of the management of superior vena cava (SVC) rupture during balloon angioplasty in an attempt to relieve SVC obstruction as a result of hemodialysis (HD) catheter-related central vein stenosis.

Patient concerns:
We present a case of a 42-year-old woman with end-stage renal disease on HD for 4 years, initially from an autogenous fistula for 2.5 years and then from a right internal jugular vein (IJV) catheter. She presented with clinical manifestations of SVC obstruction (dilated anterior chest wall and abdominal wall veins and facial swelling), which progressed over the last 1.5 years.

Diagnosis:
A venogram confirmed right and left brachiocephalic vein and SVC obstruction.

Interventions:
She underwent balloon angioplasty of the SVC through the right IJV, during which the dilated area ruptured, resulting in right hemothorax and hypovolemic shock. A covered stent was placed over the bleeding site, and the patient recovered.

Outcomes:
Rapid and skilled endovascular intervention through placement of a covered stent at the bleeding site can be lifesaving.

Lessons:
It is superior to open surgical management in terms of complexity and morbidity especially in patients who are poor surgical candidates, and its durability is proving to be comparable.
Successful pregnancy and delivery in uremic patients with maintenance hemodialysis
Authors: Cao Yun , Zhang Yide , Wang Xinmei , Zhang Yuquan , Fan Yaping , Shi Hui , Dai Houyong 
Published: December 2018

Abstract

Rationale:
It is reported that successful pregnancies in dialyzed uremic women are rare. Over the past years, despite advances in clinical management and technology in dialysis for pregnancy in patients receiving maintenance hemodialysis, uremia remains a high risk factor for adverse outcomes in mother and fetus.

Patient concerns:
In this article, we present a case of pregnancy in a 34-year-old uremic woman on dialysis. After the pregnancy was diagnosed and confirmed, intensive dialysis and multidisciplinary care according to the recommendations in the available literatures were provided.

Diagnoses:
Single pregnancy of 31+4 GWs (gestational weeks), fetal growth restriction, polyhydramnios, and uremia.

Outcomes:
At 31 weeks’ gestation and 4 days, she was admitted to our hospital due to premature rupture of membranes and abdominal pain. Then a female baby weighed 1700 g was delivered successfully. After one year of follow-up, the mother feels well and the baby is healthy.

Lessons:
Intensive dialysis, detailed management and multidisciplinary approaches are necessary for optimal outcomes in uremic pregnant mother and fetus.
Fetal congenital heart disease
Authors: Cai Meiying , Huang Hailong , Su Linjuan , Lin Na , Wu Xiaoqing , Xie Xiaorui , An Gang , Li Ying , Lin Yuan , Xu Liangpu 
Published: December 2018

Abstract

Background:
Congenital heart disease (CHD) is one of the most common birth defects; however, the mechanisms underlying its development are poorly understood. Recently, heritable genetic factors, including copy number variations (CNVs) and single nucleotide polymorphisms (SNPs), have been implicated in its etiology. The aim of this study was to investigate the utility of a SNP array for the prenatal diagnosis of CHD and the improvement of prenatal genetic counseling and to compare this approach to traditional chromosome analysis.

Methods:
One hundred and fortysix cases of CHD detected by prenatal echocardiography were analyzed. Of these, 110 were isolated CHD and 36 were of CHD with extracardiac defects. SNP analysis was performed using the Affymetrix CytoScan HD platform, which was followed by karyotype analysis. All annotated CNVs were validated by fluorescence in situ hybridization.

Results:
Karyotype analysis identified chromosomal abnormalities in 19 of 146 cases. In addition to the 15 chromosomal abnormalities that were consistent with the results of karyotype analysis, the SNP array identified abnormal CNVs in an additional 15.2% (22/145) cases; of these, 15 were pathogenic CNVs, three were variations of uncertain clinical significance, and four were benign CNVs. The rates at which the SNP array detected pathogenic CNVs differed significantly between cases of isolated CHD and CHD with extracardiac defects (13.6% vs. 72.2%, P = .001). The results of the SNP array also affected the rate of pregnancy termination.

Conclusion:
Combining SNP array with cytogenetic analyses is particularly effective for identifying chromosomal abnormalities in CNVs in fetuses with CHD, which also affects obstetrical outcomes.
ABO incompatible living donor liver transplantation using dual grafts and pure laparoscopic donor right hepatectomy
Authors: Han Young Seok , Ha Heontak , Han Ja Ryung , Lim Kyoung Hoon , Chun Jae Min 
Published: December 2018

Abstract

Rationale:
Because of the shortage of deceased donors, living donor liver transplantation (LDLT) has become the main procedure to treat patients with end-stage liver disease in Asian countries. However, many potential donors are excluded because of donor safety and graft volume issues. In addition, large abdominal wounds after open surgery for hepatectomy could be a reason for hesitating to agree to liver donation, particularly when attempting to recruit young female donors.

Patient concerns:
On volumetric computed tomography (CT) examination, remnant liver volume was too small to guarantee the safety of the male donor, and the right hemiliver volume of the female donor was not sufficient to meet the recipient‘s metabolic demand. The young female donor also worried about a large abdominal wound following open surgery.

Interventions:
We performed ABO-incompatible LDLT using dual grafts and right-sided graft was obtained by pure laparoscopic donor right hepatectomy in a young female donor.

Outcomes:
The postoperative course was uneventful in both donors and the recipient is presently doing well in satisfactory condition 7 months after liver transplantation.

Lessons:
We overcame these volumetric and cosmetic issues through dual living donor liver grafts using a combination of conventional surgery for 1 donor and laparoscopic right hepatectomy for a second ABO-incompatible donor. We think this procedure can be a good option for the expansion of donor pools.
Tumor control of third ventricular central neurocytoma after gamma knife radiosurgery in an elderly patient
Authors: Park Sue-Jee , Jung Tae-Young , Kim Seul-Kee , Lee Kyung-Hwa 
Published: December 2018

Abstract

Rationale:
Central neurocytoma is rare benign tumor that occurs in high probability in young adults in the lateral ventricle. Herein, we report an unusual case of an elderly woman who was diagnosed with central neurocytoma isolated to the third ventricle. This deeply located tumor was effectively treated using gamma knife radiosurgery (GKR).

Patient concerns:
A 79-year-old woman was admitted to hospital with gait disturbance and cognitive dysfunction.

Diagnosis:
Brain magnetic resonance imaging (MRI) revealed a homogenously enhancing multilobulated mass in the posterior third ventricle measuring 1.8 cm in size. The tumor was diagnosed as a central neurocytoma isolated to the third ventricle.

Interventions:
Neuronavigation-guided endoscopic third ventriculostomy and biopsy were performed. One week following surgery, GKR was performed using a prescribed dose of 14 Gy with 50% isodose lines, and a target volume of 1.62 cc.

Outcomes:
Three months after GKR, brain MRI revealed a decrease in the size (to 1.4 cm) of the multilobulated strong enhancing mass in the posterior third ventricle, and the patient's symptom of confusion was improved.

Lessons:
Previous studies have reported that tumors in unusual locations, such as those isolated to the third ventricle, are different according to age, either in young children or elderly individuals. Although complete surgical resection is an effective treatment for central neurocytoma, it is often difficult to approach these tumors through surgery. GKR could, therefore, be an alternative primary treatment option for deeply located central neurocytomas in elderly patients.
Sarcoidosis of the medulla oblongata causing intractable hiccoughs and numbness of extremities
Authors: Chen Xi-yuan , Ren Zhuo-chao , Huang Xiao-jun 
Published: December 2018

Abstract

Rationale:
Sarcoidosis is a multisystem disorder characterized by noncaseating granulomas. The nervous system is involved in 5 to 16% of the patients. However, neurosarcoidosis in the medulla oblongata presenting as hiccough is remarkably rare.

Patient concern:
A 55-year-old female was admitted to our hospital suffering from intractable hiccough and progressive numbness of extremities.

Diagnosis:
The MR imaging revealed a circumscribed mass lesion located on the medulla oblongata. The mass was hyperintense on T2-weighted images and enhanced homogeneously with gadolinium-diethylenetriamine penta-acetic acid. The cerebrospinal fluid analysis showed a moderately elevated protein content and a significant lymphocytosis 86.5%. Electrocardiogram (ECG) showed complete atrioventricular block. Bilateral supraclavicular, hilar, and mediastinal lymphadenopathy was diagnosed in a CT scan. Transbranchial needle aspiration biopsy revealed noncaseating granuloma consisting of epithelioid cells, lymphocytes, and rare multinucleated giant cells which was consistent with sarcoidosis. The diagnosis of multisystemic sarcoidosis was made.

Interventions and outcomes:
The patient underwent a permanent pacemaker insertion, and was successfully treated with corticosteroids.

Lessons:
It is important to consider neurosarcoidosis in the differential diagnosis of intramedullary lesion, since a right recognition may lead to appropriate treatment with steroids and avoid needlessly extensive surgery.
Characterisation of gut, lung, and upper airways microbiota in patients with non-small cell lung carcinoma
Authors: Bingula Rea , Filaire Marc , Radosevic-Robin Nina , Berthon Jean-Yves , Bernalier-Donadille Annick , Vasson Marie-Paule , Thivat Emilie , Kwiatkowski Fabrice , Filaire Edith 
Published: December 2018

Abstract

Background:
Several studies have confirmed the important role of the gut microbiota in the regulation of immune functions and its correlation with different diseases, including cancer. While brain-gut and liver-gut axes have already been demonstrated, the existence of a lung-gut axis has been suggested more recently, with the idea that changes in the gut microbiota could affect the lung microbiota, and vice versa. Likewise, the close connection between gut microbiota and cancer of proximal sites (intestines, kidneys, liver, etc.) is already well established. However, little is known whether there is a similar relation when looking at world's number one cause of death from cancer—lung cancer.

Objective:
Firstly, this study aims to characterise the gut, lung, and upper airways (UAs) microbiota in patients with non-small cell lung cancer (NSCLC) treated with surgery or neoadjuvant chemotherapy plus surgery. Secondly, it aims to evaluate a chemotherapy effect on site-specific microbiota and its influence on immune profile. To our knowledge, this is the 1st study that will analyse multi-site microbiota in NSCLC patients along with site-specific immune response.

Methods:
The study is a case-controlled observational trial. Forty NSCLC patients will be divided into 2 groups depending on their anamnesis: Pchir, patients eligible for surgery, or Pct-chir, patients eligible for neoadjuvant chemotherapy plus surgery. Composition of the UAs (saliva), gut (faeces), and lung microbiota (from broncho-alveolar lavage fluid (BALF) and 3 lung pieces: “healthy” tissue distal to tumour, peritumoural tissue and tumour itself) will be analysed in both groups. Immune properties will be evaluated on the local (evaluation of the tumour immune cell infiltrate, tumour classification and properties, immune cell phenotyping in BALF; human neutrophil protein (HNP) 1–3, β-defensin 2, and calprotectin in faeces) and systemic level (blood cytokine and immune cell profile). Short-chain fatty acids (SCFAs) (major products of bacterial fermentation with an effect on immune system) will be dosed in faecal samples. Other factors such as nutrition and smoking status will be recorded for each patient. We hypothesise that smoking status and tumour type/grade will be major factors influencing both microbiota and immune/inflammatory profile of all sampling sites. Furthermore, due to non-selectivity, the same effect is expected from chemotherapy.