bluuebunny/crossref_metadata_2025_split huggingface dataset:
Computed tomography lymphangiography findings in 27 cases of lymphangioleiomyomatosis
Authors: Zhang Chunyan , Chen Xiaobai , Wen Tingguo , Zhang Qijin , Huo Meng , Dong Jian , Shen Wen-bin , Wang Rengui 
Published: January 2017

Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema. The CT lymphangiography showed that 27/27 (100%) patients presented iliac or retroperitoneal lymphatic vessel dilation; 20/27 (74%) presented thoracic duct outlet obstruction; 9/27 (33%) presented pleural lymph leakage; and 13/27 (48%) presented an abnormal distribution of contrast agent in the contralateral lumbar trunk, 3/27 (11%) in the pulmonary field, 1/27 (4%) in the abdominal cavity, and 4/27 (15%) in the pelvic cavity. Five of the 27 patients (19%) had retroperitoneal lymphangioleiomyomas, with contrast agent accumulation in three cases during CT lymphangiography. Two of the 27 (7%) patients had enlarged lymph nodes in the retroperitoneum. All 27 patients (100%) diagnosed with LAM displayed various lung cysts on thoracic high-resolution CT scans. Conclusion CT lymphangiography findings can be used to diagnose most LAM cases accurately. Moreover, these findings also display the various lymphatic vessel abnormalities associated with LAM.
Other facial neuralgias
Authors: O’Neill Francis , Nurmikko Turo , Sommer Claudia 
Published: January 2017

Premise In this article we review some lesser known cranial neuralgias that are distinct from trigeminal neuralgia, trigeminal autonomic cephalalgias, or trigeminal neuropathies. Included are occipital neuralgia, superior laryngeal neuralgia, auriculotemporal neuralgia, glossopharyngeal and nervus intermedius neuralgia, and pain from acute herpes zoster and postherpetic neuralgia of the trigeminal and intermedius nerves. Problem Facial neuralgias are rare and many physicians do not see such cases in their lifetime, so patients with a suspected diagnosis within this group should be referred to a specialized center where multidisciplinary team diagnosis may be available. Potential solution Each facial neuralgia can be identified on the basis of clinical presentation, allowing for precision diagnosis and planning of treatment. Treatment remains conservative with oral or topical medication recommended for neuropathic pain to be tried before more invasive procedures are undertaken. However, evidence for efficacy of current treatments remains weak.
Dysregulations in circulating sphingolipids associate with disease activity indices in female patients with systemic lupus erythematosus: a cross-sectional study
Authors: Checa A , Idborg H , Zandian A , Sar D Garcia , Surowiec I , Trygg J , Svenungsson E , Jakobsson P-J , Nilsson P , Gunnarsson I , Wheelock C E 
Published: January 2017

Objective The objective of this study was to investigate the association of clinical and renal disease activity with circulating sphingolipids in patients with systemic lupus erythematosus. Methods We used liquid chromatography tandem mass spectrometry to measure the levels of 27 sphingolipids in plasma from 107 female systemic lupus erythematosus patients and 23 controls selected using a design of experiment approach. We investigated the associations between sphingolipids and two disease activity indices, the Systemic Lupus Activity Measurement and the Systemic Lupus Erythematosus Disease Activity Index. Damage was scored according to the Systemic Lupus International Collaborating Clinics damage index. Renal activity was evaluated with the British Island Lupus Activity Group index. The effects of immunosuppressive treatment on sphingolipid levels were evaluated before and after treatment in 22 female systemic lupus erythematosus patients with active disease. Results Circulating sphingolipids from the ceramide and hexosylceramide families were increased, and sphingoid bases were decreased, in systemic lupus erythematosus patients compared to controls. The ratio of C16:0-ceramide to sphingosine-1-phosphate was the best discriminator between patients and controls, with an area under the receiver-operating curve of 0.77. The C16:0-ceramide to sphingosine-1-phosphate ratio was associated with ongoing disease activity according to the Systemic Lupus Activity Measurement and the Systemic Lupus Erythematosus Disease Activity Index, but not with accumulated damage according to the Systemic Lupus International Collaborating Clinics Damage Index. Levels of C16:0- and C24:1-hexosylceramides were able to discriminate patients with current versus inactive/no renal involvement. All dysregulated sphingolipids were normalized after immunosuppressive treatment. Conclusion We provide evidence that sphingolipids are dysregulated in systemic lupus erythematosus and associated with disease activity. This study demonstrates the utility of simultaneously targeting multiple components of a pathway to establish disease associations.
Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups
Authors: Lopes S R M , Gormezano N W S , Gomes R C , Aikawa N E , Pereira R M R , Terreri M T , Magalhães C S , Ferreira J C , Okuda E M , Sakamoto A P , Sallum A M E , Appenzeller S , Ferriani V P L , Barbosa C M , Lotufo S , Jesus A A , Andrade L E C , Campos L M A , Bonfá E , Silva C A ,   Brazilian Childhood-onset Systemic Lupus Erythematosus Group
Published: January 2017

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1–23.4) vs 6.2 (0–17) vs 3.3 (0–14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0–9) vs 0 (0–6) vs 0 (0–7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.
A vision of ethics in efficient occupational healthcare
Authors: Hemberg Jessica , Nyman Helena , Hemberg Håkan 
Published: January 2017

Background: Occupational healthcare is nowadays more and more regulated by economic demands of gain and efficiency. Aim and research questions: The aim of this study is to reach a new understanding of ethics in occupational healthcare by uncovering this from occupational healthcare nurses’ own views. The research questions are as follows: (1) What is the core of an ethical value base in occupational healthcare? (2) What does it mean for occupational healthcare nurses to fulfill their ethical value base in occupational healthcare? Research design, participants, and research context: The study uses a hermeneutical approach. The context of the study is occupational healthcare. The material was collected through interviews with four occupational healthcare nurses in Finland. The texts were interpreted through hermeneutical reading. Ethical considerations: The data storage, study participation, and data handling for research purposes were approved by the participants when they provided their informed consent. Findings: The study revealed that the core of ethical values in occupational healthcare is something universal that concerns justice, honesty, and faithfulness. To fulfill the ethical value base means to continuously strive toward the inner core of love and bravely choose to listen to the this core in the name of the client and the universal good even if it involves a struggle or a sacrifice. Discussion: The ethical value base is not primarily tied to profession but is something that is general and universal. Nor do ethics and the implementation of ethical values stand in opposition to, for example, external values such as demands for efficiency and economic gain. However, ethics may promote efficiency. Conclusion: The fulfillment of nurses’ ethical value base in occupational healthcare requires that ethics first and foremost is allowed to enter the leadership and is prioritized on a leadership level. Creating caring cultures that encourage openness and support ethical discussions is fundamental.
Apoptosis and injuries of heavy ion beam and x-ray radiation on malignant melanoma cell
Authors: Qin Jin , Li Sha , Zhang Chao , Gao Dong-Wei , Li Qiang , Zhang Hong , Jin Xiao-Dong , Liu Yang 
Published: January 2017

This study aims to investigate the influence of high linear energy transfer (LET) heavy ion (12C6+) and low LET X-ray radiation on apoptosis and related proteins of malignant melanoma on tumor-bearing mice under the same physical dosage. C57BL/6 J mice were burdened by tumors and randomized into three groups. These mice received heavy ion (12C6+) and X-ray radiation under the same physical dosage, respectively; their weight and tumor volumes were measured every three days post-radiation. After 30 days, these mice were sacrificed. Then, median survival time was calculated and tumors on mice were proliferated. In addition, immunohistochemistry was carried out for apoptosis-related proteins to reflect the expression level. After tumor-bearing mice were radiated to heavy ion, median survival time improved and tumor volume significantly decreased in conjunction with the upregulated expression of pro-apoptosis factors, Bax and cytochrome C, and the downregulated expression of apoptosis-profilin (Bcl-2, Survivin) and proliferation-related proteins (proliferating cell nuclear antigen). The results indicated that radiation can promote the apoptosis of malignant melanoma cells and inhibit their proliferation. This case was more suitable for heavy ion (12C6+). High LET heavy ion (12C6+) radiation could significantly improve the killing ability for malignant melanoma cells by inducing apoptosis in tumor cells and inhibiting their proliferation. These results demonstrated that heavy ion (12C6+) presented special advantages in terms of treating malignant melanoma. Impact statement Malignant melanoma is a malignant skin tumor derived from melanin cells, which has a high malignant degree and high fatality rate. In this study, proliferating cell nuclear antigen (PCNA) can induce the apoptosis of malignant melanoma cells and inhibit its proliferation, and its induction effect on apoptosis is significantly higher than low LET X-ray; hence, it is expected to overcome its lower sensitivity to radiation. This study can provide theoretical basis for clinical trials, in which malignant melanoma is treated by heavy ion (12C6+), in order to accurately determine the clinical efficacy of heavy ion therapy. Clinical applications has revealed that local tumor control rate is high when heavy ion is used to treat malignant melanoma, indicating that heavy ion is an important direction in treating melanoma in the future.
Ruptured intracranial vertebral artery dissecting aneurysms: An evaluation of prognostic factors of treatment outcome
Authors: Urasyanandana K , Withayasuk P , Songsaeng D , Aurboonyawat T , Chankaew E , Churojana A 
Published: January 2017

Objective Intracranial spontaneous vertebral artery dissecting aneurysms commonly occur in the third to fifth decades of life, and are mostly associated with hypertension. Patients present with intracranial haemorrhage or thromboembolic events. Patients who present with intracranial haemorrhage carry about a 70% risk of recurrent bleeding. Patients with a posterior-inferior cerebellar artery (PICA) or ipsilateral dominant vertebral artery involve selecting which parent vessel could not be sacrificed. Recent reconstructive techniques such as stent-assisted coiling embolisation and flow-diverting stents are effective treatments of choice. Methods Seventeen patients presented subarachnoid haemorrhage and nine patients with other symptoms. Sacrificing the parent vertebral artery was the first choice for surgical or endovascular methods. Endovascular reconstructive treatment by stent-assisted coiling embolisation was indicated in dissecting vertebral artery aneurysms with ipsilateral dominant vertebral artery or PICA involvement. Clinical outcomes were determined using the modified Rankin Score (mRS) at 90 days, with favourable outcomes defined as 0 to 2. Results Of the patients presenting with ruptured aneurysms, 11 (61.1%) had a good clinical outcome, with a mRS of 0–2. Favourable Hunt and Hess grading (65%), mild to moderate GCS (65%) and total occlusion of aneurysms after treatment (65%) were significantly good prognostic factors in patients with ruptured vertebral artery dissecting aneurysms. Conclusion Endovascular parent vessel sacrifice could be the first choice to treat a ruptured vertebral artery dissecting aneurysm. Stent-assisted coiling to preserve the patency of the parent artery and its branches is a promising treatment for vertebral artery dissections.
Cerebral haemodynamics in early puerperium: A prospective study
Authors: Anzola GP , Brighenti R , Cobelli M , Giossi A , Mazzucco S , Olivato S , Pari E , Piras MP , Padovani A , Rinaldi F , Turri G 
Published: January 2017

Aim Prospective study on 900 consecutive puerperae to assess normal values and range of the blood flow velocity in the middle cerebral artery in both hemispheres. Material and method M1 and M2 segments of both middle cerebral arteries were assessed in all subjects within 96 hours of delivery. Mean flow velocity was recorded after adjusting for insonation angle. Lindegaard index (LI = middle cerebral artery–Internal Carotid Artery mean flow velocity ratio) was calculated whenever the mean flow velocity exceeded 100 cm/second. Asymmetry indexes were calculated inter hemispherically for M1 and M2 segments separately. Results Mean flow velocities were 74 ± 17 and 72 ± 17 in right and 73 ± 17 and 72 ± 17 cm/second in the left M1 and M2, respectively. A total of 136 subjects (12.1%) exceeded the threshold of 100 cm/second, but LI was consistently <3 in all of them. Mean flow velocity was inversely and independently correlated to haemoglobin levels and to parity. Mean asymmetry indexes were 0.25 ± 23 in M1 and 0.45 ± 25 in M2. Conclusion Mean flow velocity in the middle cerebral artery of healthy subjects in early puerperium is higher than in age-matched non-puerperal women and may exceed the threshold of 100 cm/second with no evidence of intracranial spasm, because of blood loss during delivery. Mean flow velocity is independently correlated with parity. Right-to-left mean flow velocity asymmetry may reach 50% as a consequence of a transient imbalance in vascular tone regulation.
Readiness to participate in advance care planning: A qualitative study of renal failure patients, families and healthcare providers
Authors: Hutchison Lauren A , Raffin-Bouchal Donna S , Syme Charlotte A , Biondo Patricia D , Simon Jessica E 
Published: January 2017

Objectives Advance care planning is the process by which people reflect upon their wishes and values for healthcare, discuss their choices with family and friends and document their wishes. Readiness represents a key predictor of advance care planning participation; however, the evidence for addressing readiness is scarce within the renal failure context. Our objectives were to assess readiness for advance care planning and barriers and facilitators to advance care planning uptake in a renal context. Methods Twenty-five participants (nine patients, nine clinicians and seven family members) were recruited from the Southern Alberta Renal Program. Semi-structured interviews were recorded, transcribed and then analyzed using interpretive description. Results Readiness for advance care planning was driven by individual values perceived by a collaborative encounter between clinicians and patients/families. If advance care planning is not valued, then patients/families and clinicians are not ready to initiate the process. Patients and clinicians are delaying conversations until “illness burden necessitates,” so there is little “advance” care planning, only care planning in-the-moment closer to the end of life. Discussion The value of advance care planning in collaboration with clinicians, patients and their surrogates needs reframing as an ongoing process early in the patient’s illness trajectory, distinguished from end-of-life decision making.
The family impact of caring for a child with juvenile dermatomyositis
Authors: Kountz-Edwards Samantha , Aoki Christa , Gannon Caitlin , Gomez Rowena , Cordova Matthew , Packman Wendy 
Published: January 2017

BackgroundJuvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin). Despite improvement in treatment regimens and the lowering of mortality rates, some children still lose their lives to JDM. This study assessed the effects of caring for a child diagnosed with JDM on the family system.MethodsParticipants included 36 mothers and 3 fathers of a child diagnosed with JDM. Parents were administered self-report measures, which assessed the overall family functioning (PedsQL-Family Impact Module), and the parents' mood and level of distress (profile of mood states). Additionally, parents were administered a semi-structured interview that included background information, psychosocial information, and sources of support.Results and conclusionFamilies of children with JDM reported difficulties in family functioning, communication problems, and an increased number of conflicts. Parents appeared to be experiencing higher than average levels of worry, worse physical functioning, and family relationships when compared to normative populations. Parents would benefit from psychosocial support due to the many challenges associated with caring for a child with JDM.
Factors that influence clinicians’ decisions to offer intravenous alteplase in acute ischemic stroke patients with uncertain treatment indication: Results of a discrete choice experiment
Authors: De Brún Aoife , Flynn Darren , Ternent Laura , Price Christopher I , Rodgers Helen , Ford Gary A , Rudd Matthew , Lancsar Emily , Simpson Stephen , Teah John , Thomson Richard G 
Published: January 2017

Background Treatment with intravenous alteplase for eligible patients with acute ischemic stroke is underused, with variation in treatment rates across the UK. This study sought to elucidate factors influencing variation in clinicians’ decision-making about this thrombolytic treatment. Methods A discrete choice experiment using hypothetical patient vignettes framed around areas of clinical uncertainty was conducted with UK-based clinicians. Mixed logit regression analyses were conducted on the data. Results A total of 138 clinicians completed the discrete choice experiment. Seven patient factors were individually predictive of increased likelihood of immediately offering IV alteplase (compared to reference levels in brackets): stroke onset time 2 h 30 min [50 min]; pre-stroke dependency mRS 3 [mRS 4]; systolic blood pressure 185 mm/Hg [140 mm/Hg]; stroke severity scores of NIHSS 5 without aphasia, NIHSS 14 and NIHSS 23 [NIHSS 2 without aphasia]; age 85 [68]; Afro-Caribbean [white]. Factors predictive of withholding treatment with IV alteplase were: age 95 [68]; stroke onset time of 4 h 15 min [50 min]; severe dementia [no memory problems]; SBP 200 mm/Hg [140 mm/Hg]. Three clinician-related factors were predictive of an increased likelihood of offering IV alteplase (perceived robustness of the evidence for IV alteplase; thrombolyzing more patients in the past 12 months; and high discomfort with uncertainty) and one with a decreased likelihood (high clinician comfort with treating patients outside the licensing criteria). Conclusions Both patient- and clinician-related factors have a major influence on the use of alteplase to treat patients with acute ischemic stroke. Clinicians’ views of the evidence, comfort with uncertainty and treating patients outside the license criteria are important factors to address in programs that seek to reduce variation in care quality regarding treatment with IV alteplase. Further research is needed to further understand the differences in clinical decision-making about treating patients with acute ischemic stroke with IV alteplase.
Classification of ulnar triangular fibrocartilage complex tears. A treatment algorithm for Palmer type IB tears
Authors: Atzei A. , Luchetti R. , Garagnani L. 
Published: January 2017

The classical definition of ‘Palmer Type IB’ triangular fibrocartilage complex tear, includes a spectrum of clinical conditions. This review highlights the clinical and arthroscopic criteria that enable us to categorize five classes on a treatment-oriented classification system of triangular fibrocartilage complex peripheral tears. Class 1 lesions represent isolated tears of the distal triangular fibrocartilage complex without distal radio-ulnar joint instability and are amenable to arthroscopic suture. Class 2 tears include rupture of both the distal triangular fibrocartilage complex and proximal attachments of the triangular fibrocartilage complex to the fovea. Class 3 tears constitute isolated ruptures of the proximal attachment of the triangular fibrocartilage complex to the fovea; they are not visible at radio-carpal arthroscopy. Both Class 2 and Class 3 tears are diagnosed with a positive hook test and are typically associated with distal radio-ulnar joint instability. If required, treatment is through reattachment of the distal radio-ulnar ligament insertions to the fovea. Class 4 lesions are irreparable tears due to the size of the defect or to poor tissue quality and, if required, treatment is through distal radio-ulnar ligament reconstruction with tendon graft. Class 5 tears are associated with distal radio-ulnar joint arthritis and can only be treated with salvage procedures. This subdivision of type IB triangular fibrocartilage complex tear provides more insights in the pathomechanics and treatment strategies. Level of evidence: II