bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of precocious puberty in a McCune–Albright syndrome patient using Chinese medicinal herbs combined with megestrol acetate: A case report
Authors: Sun Wen , Wang Yonghong , Yu Jian , Ji Min 
Published: February 2018

Introduction: McCune–Albright Syndrome (MAS) is a rare congenital sporadic disorder characterized by fibrous bone dysplasia, café-au-lait pigmented spots on the skin, and non-gonadotropin dependent precocious puberty (PP), which is caused by a postzygotic somatic activating mutation in the GNAS gene encoding the alpha subunit of Gs protein. In our case study, we recorded a girl with the onset of MAS and treated her with Chinese medicinal herbs combined with megestrol acetate. We aim to provide a method for the treatment of children with this rare form of precocious puberty. Case Presention: A 4-year-old girl presented with vaginal bleeding and enlarged breasts. The activating mutation of GNAS was not detected in the patient’s peripheral blood samples, as some had reported. Because of peripheral PP and fibrous dysplasia of the diagnosed bone, the patient was considered as MAS. We chose the Chinese medicinal herbs combined with megestrol acetate for treatment, and the patient was effectively treated. Conclusion: The combination therapy of Chinese medicinal herbs plus megestrol acetate in managing PP in an MAS is one of the useful treatments.
Implications of preliminary subsidence analyses for the Parnaíba cratonic basin
Authors: Rodríguez Tribaldos Verónica , White Nicky 
Published: February 2018

Abstract

Cratonic sedimentary basins are underlain by thick lithosphere and subside over hundreds of millions of years. The driving mechanism for these basins remains enigmatic due to the paucity of crustal-scale constraints. To address this shortcoming, an integrative study of the Parnaíba cratonic basin of NE Brazil is underway. We summarize field observations, well-log information, seismic reflection profiles and subsidence analyses with the objective of shedding light on possible basin-forming mechanisms. Lithological descriptions and borehole logs reveal a stratigraphic succession that consists of interbedded shallow marine and continental deposits. On seismic reflection profiles, this succession is divided into packages of relatively undisturbed reflections bounded by basin-wide disconformities. These disconformities are manifest as bright, rugose reflections that probably represent buried palaeo-landscapes. Backstripped and water-loaded subsidence curves calculated from boreholes distributed throughout the basin show that tectonic subsidence decreases exponentially over
c.
350 Ma. A simple model suggests that this subsidence pattern agrees with a thermal time constant of 70–100 Ma, placing a significant constraint on the likely basin-forming mechanism. This background trend is punctuated by minor increases in subsidence that follow basin-wide erosional disconformities. These departures could be indicative of transient epeirogenic uplift events caused by changing patterns of dynamic topography.
Spatial persistence of reduced specific ventilation following methacholine challenge in the healthy human lung
Authors: Geier E. T. , Neuhart I. , Theilmann R. J. , Prisk G. K. , Sá R. C. 
Published: February 2018

Specific ventilation imaging was used to identify regions of the healthy lung (6 supine subjects, ages 21–41 yr, 3 men) that experienced a fall in specific ventilation following inhalation of methacholine. This test was repeated 1 wk later and 3 mo later to test for spatial recurrence. Our data showed that 53% confidence interval (CI; 46%, 59%) of volume elements that constricted during one methacholine challenge did so again in another and that this quantity did not vary with time; 46% CI (28%, 64%) recurred 1 wk later, and 56% CI (51%, 61%) recurred 3 mo later. Previous constriction was a strong predictor for future constriction. Volume elements that constricted during one challenge were 7.7 CI (5.2, 10.2) times more likely than nonconstricted elements to constrict in a second challenge, regardless of whether the second episode was 1 wk [7.7 CI (2.9, 12.4)] or 3 mo [7.7 CI (4.6, 10.8)] later. Furthermore, posterior lung elements were more likely to constrict following methacholine than anterior lung elements (volume fraction 0.43 ± 0.22 posterior vs. 0.10 ± 0.03 anterior; P = 0.005), and basal elements that constricted were more likely than their apical counterparts to do so persistently through all three trials (volume fraction 0.14 ± 0.04 basal vs. 0.04 ± 0.04 apical; P = 0.003). Taken together, this evidence suggests a physiological predisposition toward constriction in some lung elements, especially those located in the posterior and basal lung when the subject is supine.NEW & NOTEWORTHY The spatial pattern of bronchoconstriction following methacholine is persistent over time in healthy individuals, in whom chronic inflammation and airway remodeling are assumed to be absent. This suggests that regional lung inflation and airway structure may play dominant roles in determining the spatial pattern of methacholine bronchoconstriction.
Ischemic conditioning increases strength and volitional activation of paretic muscle in chronic stroke: a pilot study
Authors: Hyngstrom Allison S. , Murphy Spencer A. , Nguyen Jennifer , Schmit Brian D. , Negro Francesco , Gutterman David D. , Durand Matthew J. 
Published: February 2018

Ischemic conditioning (IC) on the arm or leg has emerged as an intervention to improve strength and performance in healthy populations, but the effects on neurological populations are unknown. The purpose of this study was to quantify the effects of a single session of IC on knee extensor strength and muscle activation in chronic stroke survivors. Maximal knee extensor torque measurements and surface EMG were quantified in 10 chronic stroke survivors (>1 yr poststroke) with hemiparesis before and after a single session of IC or sham on the paretic leg. IC consisted of 5 min of compression with a proximal thigh cuff (inflation pressure = 225 mmHg for IC or 25 mmHg for sham) followed by 5 min of rest. This was repeated five times. Maximal knee extensor strength, EMG magnitude, and motor unit firing behavior were measured before and immediately after IC or sham. IC increased paretic leg strength by 10.6 ± 8.5 Nm, whereas no difference was observed in the sham group (change in sham = 1.3 ± 2.9 Nm, P = 0.001 IC vs. sham). IC-induced increases in strength were accompanied by a 31 ± 15% increase in the magnitude of muscle EMG during maximal contractions and a 5% decrease in motor unit recruitment thresholds during submaximal contractions. Individuals who had the most asymmetry in strength between their paretic and nonparetic legs had the largest increases in strength ( r2 = 0.54). This study provides evidence that a single session of IC can increase strength through improved muscle activation in chronic stroke survivors. NEW & NOTEWORTHY Present rehabilitation strategies for chronic stroke survivors do not optimally activate paretic muscle, and this limits potential strength gains. Ischemic conditioning of a limb has emerged as an effective strategy to improve muscle performance in healthy individuals but has never been tested in neurological populations. In this study, we show that ischemic conditioning on the paretic leg of chronic stroke survivors can increase leg strength and muscle activation while reducing motor unit recruitment thresholds.
Prognostic Significance of Pre- and Postoperative Lymphocyte Counts in Patients with Gastric Cancer
Authors: Saito Hiroaki , Kono Yusuke , Murakami Yuki , Shishido Yuji , Kuroda Hirohiko , Yamamoto Manabu , Fukumoto Yoji , Osaki Tomohiro , Ashida Keigo , Fujiwara Yoshiyuki 
Published: February 2018

<b><i>Background:</i></b> Although preoperative lymphopenia is reportedly a prognostic factor in cancer patients, the association between postoperative lymphopenia and patient prognosis has not been widely studied. <b><i>Methods:</i></b> We enrolled 352 patients who underwent surgery for gastric cancer (GC) between January 2005 and April 2013 to analyze correlations among pre- and postoperative lymphocyte counts (LCs) and prognosis in GC patients. <b><i>Results:</i></b> Pre- and postoperative (obtained 1 day after surgery) LCs were significantly correlated (<i>r</i> = 0.496, <i>p</i> &#x3c; 0.0001). Pre- and postoperative LCs of elderly patients were significantly lower than those of non-elderly patients. Postoperative lymphocyte count was significantly lower in patients with a differentiated tumor than in those with an undifferentiated tumor. Based on the results of receiver operating characteristic analysis, patients were classified into subgroups as: preoperative LC ≥1,676 (pre-LC<sup>High</sup>), preoperative LC &#x3c;1,676 (pre-LC<sup>Low</sup>); and as postoperative LC ≥855 (post-LC<sup>High</sup>), and postoperative LC &#x3c;855 (post-LC<sup>Low</sup>). Five-year overall survival rates significantly differed between pre-LC<sup>High</sup> (82.5%) and pre-LC<sup>Low</sup> (71.6%) groups (<i>p =</i> 0.023); and also between the post-LC<sup>High</sup> (81.5%) and post-LC<sup>Low</sup> (69.5%) groups (<i>p</i> = 0.0072). The 5-year disease specific survival rates were 91.3 and 82.4% in patients with post-LC<sup>High</sup> and those with post-LC<sup>Low</sup>, respectively, and differences were statistically significant (<i>p</i> = 0.015). Multivariate analysis indicated that postoperative lymphocyte count was an independent prognostic indicator, along with age, gender, tumor size, lymph node metastasis, and venous invasion. <b><i>Conclusions:</i></b> Postoperative lymphocyte count is a useful predictive factor for prognosis in GC patients.
Pediatric Dilated Cardiomyopathy‐Associated
LRRC10
(Leucine‐Rich Repeat–Containing 10) Variant Reveals LRRC10 as an Auxiliary Subunit of Cardiac L‐Type Ca
2+
Channels
Authors: Woon Marites T. , Long Pamela A. , Reilly Louise , Evans Jared M. , Keefe Alexis M. , Lea Martin R. , Beglinger Carl J. , Balijepalli Ravi C. , Lee Youngsook , Olson Timothy M. , Kamp Timothy J. 
Published: February 2018

Background

Genetic causes of dilated cardiomyopathy (DCM) are incompletely understood. LRRC10 (leucine‐rich repeat–containing 10) is a cardiac‐specific protein of unknown function. Heterozygous mutations in
LRRC10
have been suggested to cause DCM, and deletion of
Lrrc10
in mice results in DCM.

Methods and Results

Whole‐exome sequencing was carried out on a patient who presented at 6 weeks of age with DCM and her unaffected parents, filtering for rare, deleterious, recessive, and de novo variants. Whole‐exome sequencing followed by trio‐based filtering identified a homozygous recessive variant in
LRRC10
, I195T. Coexpression of I195T
LRRC10
with the L‐type Ca
2+
channel (Ca
v
1.2, β
2CN2
, and α
2
δ subunits) in HEK293 cells resulted in a significant ≈0.5‐fold decrease in I
Ca,L
at 0 mV, in contrast to the ≈1.4‐fold increase in I
Ca,L
by coexpression of
LRRC10
(n=9–12,
P
<0.05). Coexpression of
LRRC10
or I195T
LRRC10
did not alter the surface membrane expression of Ca
v
1.2.
LRRC10
coexpression with Ca
v
1.2 in the absence of auxiliary β
2CN2
and α
2
δ subunits revealed coassociation of Ca
v
1.2 and LRRC10 and a hyperpolarizing shift in the voltage dependence of activation (n=6–9,
P
<0.05). Ventricular myocytes from
Lrrc10

−/−

mice had significantly smaller I
Ca,L
, and coimmunoprecipitation experiments confirmed association between LRRC10 and the Ca
v
1.2 subunit in mouse hearts.

Conclusions

Examination of a patient with DCM revealed homozygosity for a previously unreported
LRRC10
variant: I195T. Wild‐type and I195T LRRC10 function as cardiac‐specific subunits of L‐type Ca
2+
channels and exert dramatically different effects on channel gating, providing a potential link to DCM.
Tri-phasic fever in dengue fever
Authors: D Pradeepa H , Rao Sathish B , B Ganaraj , Bhat Gopalakrishna , M Chakrapani 
Published: February 2018

Dengue fever is an acute febrile illness with a duration of 2–12 days. Our observational study observed the 24-h continuous tympanic temperature pattern of 15 patients with dengue fever and compared this with 26 others with fever due to a non-dengue aetiology. A tri-phasic fever pattern was seen among two-thirds of dengue fever patients, but in only one with an inflammatory disease. One-third of dengue fever patients exhibited a single peak temperature. Continuous temperature monitoring and temperature pattern analysis in clinical settings can aid in the early differentiation of dengue fever from non-dengue aetiology.
Effectiveness of aquatic exercises compared to patient-education on health status in individuals with knee osteoarthritis: a randomized controlled trial
Authors: Taglietti Marcelo , Facci Ligia Maria , Trelha Celita Salmaso , de Melo Fernanda Cristiane , da Silva Daniela Wosiack , Sawczuk Geovane , Ruivo Thamires Marques , de Souza Thaisley Barbosa , Sforza Chiarella , Cardoso Jefferson Rosa 
Published: February 2018

Objective: To compare the effectiveness of aquatic exercises with patient-education in individuals with knee osteoarthritis. Design: Randomized controlled trial with blinded assessor and intention-to-treat analysis. Setting: Aquatic Physiotherapy Centre and Primary Health Care Unit. Subjects: A total of 60 patients, aged 68.3 (SD = 4.8) with clinical symptoms and radiographic grading (Kellgren–Lawrence 1–4) of knee osteoarthritis were included. Interventions: An eight-week treatment protocol of aquatic exercise ( n = 31) (16 individual sessions, twice a week) and an educational program (group sessions, once a week) ( n = 29). Main measures: Before, after eight-week intervention, and a three-month follow-up with results for the following outcome measures: pain, function, quality of life, functional mobility, and depression. Results: At the end of treatment, the WOMAC (Western Ontario and McMaster Universities Osteoarthritis Index) functional capacity values reduced in favour of the aquatic exercise group for both the total score MD (mean difference) = –14.2; 95% CI (confidence interval) (−18; −10.5), P = 0.04 and the pain domain MD = −3.8 points; 95% CI (−8.71; −1), P = 0.021. The total score also reduced in the follow-up: MD = −12.3 points; 95% CI (−24.7; −6.1), P = 0.017. No differences were found for the outcomes functional mobility or depression. Conclusion: Aquatic exercise improved pain and function after eight weeks, and function at the three-month follow-up compared to the patient-education program.
A child with acute liver failure associated with influenza A and resolved with plasma exchange treatment
Authors: Yükselmiş Ufuk , Girit Saniye , Çağ Yakup , Özçetin Mustafa 
Published: February 2018

Introduction: Although liver support systems play a major role as a bridge to transplantation, they may sometimes serve as a lifesaving treatment eliminating this need. Case presentation: We report on a 4-year-old boy who developed acute liver failure due to an influenza A (H3N2) infection as confirmed by clinical and laboratory data (molecular typing, stage 3 encephalopathy, brain edema, increased levels of ammonia, bilirubin, and international normalized ratio 5.2). Testing for any possible underlying liver disease showed no congenital or acquired liver pathology. Oral oseltamivir treatment was initiated and liver support therapy with plasma exchange was performed as a bridge to transplantation. A total of three plasma exchange sessions every other day, with fresh frozen plasma 1.5 times the total blood volume for the first course and 1 times for the subsequent courses, were performed. After the first plasma exchange, encephalopathy improved to stage 2, accompanied by substantial decreases in the elevated liver function tests. At the end of three plasma exchange sessions, the patient’s clinical condition improved significantly. At 14 days after admission, deep tendon reflexes of the patient were normal and levels of alanine transaminase, aspartate transaminase, bilirubin, ammonia, and international normalized ratio returned to normal. Liver support treatment with plasma exchange resulted in complete recovery and the patient was discharged on the 17th day of admission. Conclusion: Acute supportive treatment with plasma exchange proved to be life-saving in our case.
Theory, social work methods and participation
Authors: Lub Vasco 
Published: February 2018

Summary This paper examines the use of theory in social work methods geared towards promoting participation of vulnerable groups, a subject that has been barely – if at all – researched empirically. The study comprised an analysis of 46 methods documented in the database of the Netherlands Institute for Social Development and interviews with social workers and educators. Findings Social work methods for participation are interspersed with theoretical notions. While the study shows that there is no shortage of theories in this field, what is more problematic are their use and integration. Incorporating theories in such a way that they provide an underpinning of the work method poses a challenge. Many theories appear to be presented out of window dressing, deviate from their original source in the literature, or are narrow in their paradigmatic focus, overlooking alternative theories that could promote or offer better nuanced participation perspectives. Applications The paper proposes criteria for a ‘theoretical underpinning’ and provides suggestions for a proper use of theory in social work methods.
Analysis of Iliac Artery Geometric Properties in Fenestrated Aortic Stent Graft Rotation
Authors: Doyle Matthew G. , Crawford Sean A. , Osman Elrasheed , Eisenberg Naomi , Tse Leonard W. , Amon Cristina H. , Forbes Thomas L. 
Published: February 2018

Introduction: A complication of fenestrated endovascular aneurysm repair is the potential for stent graft rotation during deployment causing fenestration misalignment and branch artery occlusion. The objective of this study is to demonstrate that this rotation is caused by a buildup of rotational energy as the device is delivered through the iliac arteries and to quantify iliac artery geometric properties associated with device rotation. Methods: A retrospective clinical study was undertaken in which iliac artery geometric properties were assessed from preoperative imaging for 42 cases divided into 2 groups: 27 in the nonrotation group and 15 in the rotation group. Preoperative computed tomography scans were segmented, and the iliac artery centerlines were determined. Iliac artery tortuosity, curvature, torsion, and diameter were calculated from the centerline and the segmented vessel geometry. Results: The total iliac artery net torsion was found to be higher in the rotation group compared to the nonrotation group (23.5 ± 14.7 vs 14.6 ± 12.8 mm−1; P = .05). No statistically significant differences were found for the mean values of tortuosity, curvature, torsion, or diameter between the 2 groups. Conclusion: Stent graft rotation occurred in 36% of the cases considered in this study. Cases with high iliac artery total net torsion were found to be more likely to have stent graft rotation upon deployment. This retrospective study provides a framework for prospectively studying the influence of iliac artery geometric properties on fenestrated stent graft rotation.
How I treat myelodysplastic syndromes of childhood
Authors: Locatelli Franco , Strahm Brigitte 
Published: February 2018

Abstract
Pediatric myelodysplastic syndromes (MDSs) are a heterogeneous group of clonal disorders with an annual incidence of 1 to 4 cases per million, accounting for less than 5% of childhood hematologic malignancies. MDSs in children often occur in the context of inherited bone marrow failure syndromes, which represent a peculiarity of myelodysplasia diagnosed in pediatric patients. Moreover, germ line syndromes predisposing individuals to develop MDS or acute myeloid leukemia have recently been identified, such as those caused by mutations in GATA2, ETV6, SRP72, and SAMD9/SAMD9-L. Refractory cytopenia of childhood (RCC) is the most frequent pediatric MDS variant, and it has specific histopathologic features. Allogeneic hematopoietic stem cell transplantation (HSCT) is the treatment of choice for many children with MDSs and is routinely offered to all patients with MDS with excess of blasts, to those with MDS secondary to previously administered chemoradiotherapy, and to those with RCC associated with monosomy 7, complex karyotype, severe neutropenia, or transfusion dependence. Immune-suppressive therapy may be a treatment option for RCC patients with hypocellular bone marrow and the absence of monosomy 7 or a complex karyotype, although the response rate is lower than that observed in severe aplastic anemia, and a relevant proportion of these patients will subsequently need HSCT for either nonresponse or relapse.
The ratio of ADSCs to HSC‐progenitors in adipose tissue derived SVF may provide the key to predict the outcome of stem‐cell therapy
Authors: Kilinc Mehmet Okyay , Santidrian Antonio , Minev Ivelina , Toth Robert , Draganov Dobrin , Nguyen Duong , Lander Elliot , Berman Mark , Minev Boris , Szalay Aladar A. 
Published: February 2018

AbstractBackgroundStromal vascular fraction (SVF) represents an attractive source of adult stem cells and progenitors, holding great promise for numerous cell therapy approaches. In 2017, it was reported that 1524 patients received autologous SVF following the enzymatic digestion of liposuction fat. The treatment was safe and effective and patients showed significant clinical improvement. In a collaborative study, we analyzed SVF obtained from 58 patients having degenerative, inflammatory, autoimmune diseases, and advanced stage cancer.ResultsFlow analysis showed that freshly isolated SVF was very heterogeneous and harbored four major subsets specific to adipose tissue; CD34high CD45− CD31− CD146− adipose‐derived stromal/stem cells (ADSCs), CD34low CD45+ CD206+CD31− CD146− hematopoietic stem cell‐progenitors (HSC‐progenitors), CD34high CD45− CD31+CD146+ adipose tissue‐endothelial cells and CD45−CD34−CD31−CD146+ pericytes. Culturing and expanding of SVF revealed a homogenous population lacking hematopoietic lineage markers CD45 and CD34, but were positive for CD90, CD73, CD105, and CD44. Flow cytometry sorting of viable individual subpopulations revealed that ADSCs had the capacity to grow in adherent culture. The identity of the expanded cells as mesenchymal stem cells (MSCs) was further confirmed based on their differentiation into adipogenic and osteogenic lineages. To identify the potential factors, which may determine the beneficial outcome of treatment, we followed 44 patients post‐SVF treatment. The gender, age, clinical condition, certain SVF‐dose and route of injection, did not play a role on the clinical outcome. Interestingly, SVF yield seemed to be affected by patient's characteristic to various extents. Furthermore, the therapy with adipose‐derived and expanded‐mesenchymal stem cells (ADE‐MSCs) on a limited number of patients, did not suggest increased efficacies compared to SVF treatment. Therefore, we tested the hypothesis that a certain combination, rather than individual subset of cells may play a role in determining the treatment efficacy and found that the combination of ADSCs to HSC‐progenitor cells can be correlated with overall treatment efficacy.ConclusionsWe found that a 2:1 ratio of ADSCs to HSC‐progenitors seems to be the key for a successful cell therapy. These findings open the way to future rational design of new treatment regimens for individuals by adjusting the cell ratio before the treatment.