scholarly journals Congenital hyperextension deformity of the knees due to arthrogryposis multiplex congenita? Case report

Case reports ◽  
2020 ◽  
Vol 6 (2) ◽  
pp. 137-145
Author(s):  
Paola Andrea Romero-Campiño ◽  
Liliana Sandoval-Tristancho ◽  
María Camila Jaramillo ◽  
Anna Claici ◽  
Lina Paola Montaña-Jiménez
Keyword(s):  
Multidisciplinary Treatment ◽  
Diagnostic Errors ◽  
Lower Limbs ◽  
Congenital Deformity ◽  
Active Movement ◽  
Arthrogryposis Multiplex Congenita ◽  
Distal Perfusion ◽  
The Right ◽  
High Level ◽  
Adequate Prenatal Care

Introduction: Arthrogryposis multiplex congenita is a disorder characterized by non-progressive joint contractures. It has an estimated prevalence of 1 in every 3 000-5 000 live births, with the same male-to-female ratio.Case presentation: This is the case of a male newborn with adequate prenatal care checkup appointments, who presented with a congenital deformity of the lower limbs. On physical examination, he had hyperextension of the knees (passive flexion of 20° in the left leg and 30° in the right leg), and painful active movement. On admission, peripheral pulses had good intensity, and adequate distal perfusion was found. Barlow and Ortolani maneuvers were negative, and no midline lesions were observed in the spine. The patient was diagnosed with arthrogryposis multiplex congenita and received multidisciplinary treatment to avoid early morbidity and mortality.Conclusion: To attain satisfactory clinical development in patients with arthrogryposis, it is essential to have a high level of antenatal suspicion, as well as appropriate prenatal checkups. All this allows for proper management, minimizing diagnostic errors, avoiding unnecessary procedures, and performing effective and timely treatment with outstanding results.

IMMUNOTHERAPY FOR NSCLC: THE RIGHT TREATMENT FOR THE RIGHT PERSON

2019 ◽  
Vol 65 (1) ◽  
pp. 27-41
Author(s):  
Yelena Artamonova
Keyword(s):  
Life Expectancy ◽  
Tumor Cells ◽  
Advanced Nsclc ◽  
Objective Response ◽  
Combination Group ◽  
Line Treatment ◽  
Phase 3 ◽  
Median Response ◽  
The Right ◽  
High Level

Lung cancer is the leading cause of mortality from malignant tumors all over the world. Since most patients at the time of diagnosis already have stage III-IV of the disease, the search for new effective treatment strategies for advanced NSCLC is the most important problem of modern oncology. The results of the study of the anti-PD1 monoclonal antibody pembrolizumab were a real breakthrough in the treatment of NSCLC. In the KEYN0TE-001 study, the expression of PD-L1 on tumor cells was validated as a predictive biomarker of the drug's efficiency. Pembrolizumab demonstrated the possibility of achieving long-term objective responses, and a 4-year 0S with all histological types in the subgroup of pre-treated patients with PD-L1 expression> 50% was 24.8% and 15.6% in the PD-L1> 1% group. In a phase 2/3 randomized study KEYN0TE-10 in the 2nd line treatment of NSCLC with PD-L1 expression > 1% pembrolizumab significantly increased life expectancy compared to docetaxel and confirmed the possibility of longterm duration of objective responses, even after cessation of treatment. Then the focus of research shifted to the 1st line of treatment. About 30% of patients with NSCLC have a high level of PD-L1 expression on tumor cells and demonstrate the most impressive response to pembrolizumab therapy. A randomized phase 3 study KEYN0TE-024 compared the effectiveness of pembrolizumab monotherapy with a standard platinum combination in patients with advanced NSCLC with a high level of PD-L1 expression without EGFR mutations or ALK translocation. Compared with the platinum doublet the administration of pembrolizumab significantly increased all estimated parameters, including the median of progression-free survival (mPFS was 10.3 months versus 6 months; HR = 0.50; 95% CI 0.37-0.68, p < 0.001), the objective response rate (ORR 44.8% versus 27.8%), duration of response (in the pembrolizumab arm the median was not reached, in the chemotherapy (CT) group - 6.3 months). Despite the approved crossover, the use of pembrolizumab in the 1st line of treatment more than doubled the life expectancy of NSCLC patients with high PD-L1 expression as compared to CT: the median overall survival (OS) was 30.0 months versus 14.2 months (HR = 0.63, p = 0.002), 1-year OS 70.3% versus 54.8%; 2-year OS - 51.5% versus 34.5%. The remaining population to study were untreated patients with any level of PD-L1 expression. A randomized phase 3 study KEYNOTE-189 evaluated the effectiveness of adding pembrolizumab to the platinum combination in the 1st line treatment of non-squamous NSCLC without EGFR and ALK mutations with any PD-L1 expression. The addition of pembrolizumab to the standard 1st line CT significantly increased all estimated efficacy indicators including OS, PFS and ORR. After a median follow-up of 10.5 months the median OS in the pembrolizumab combination group was not reached and in CT group was 11.3 months. The estimated 12-months survival was 69.2% and 49.4% respectively (HR = 0.49; 95% CI 0.38-0,64; p <0.001). The median PFS was 8.8 months versus 4.9 months, alive 1 year without progression 34.1% and 17.3% of patients respectively (HR = 0.52; p <0.001). The ORR in the group with pembrolizumab reached 47.6% versus 18.9% in CT group, moreover the tumor regressions were much longer. Finally a randomized 3-phase study KEYN0TE-407 evaluated the effectiveness of adding pembrolizumab to 1st-line CT of NSCLC with squamous histology with any PD-L1 expression. As the first analysis showed, the addition of permboli-zumab significantly increased OS of patients with squamous NSCLC, median OS 15.9 months versus 11.3 months in the groups of pembrolizumab + CT and placebo + CT respectively (HR = 0.64; 95% CI 0,49-0.95; p = 0.0006), median PFS 6.4 months and 4.8 months respectively (HR = 0.56; 95% CI 0.450.70; p <0, 0001) and OrR 57.9% versus 38.4%, the median response duration 7.7 months versus 4.8 months. Thus, the convincing advantages of using pembrolizumab in 1st line therapy were demonstrated in 3 randomized phase 3 studies: in monotherapy of NSCLC of any histological subtype with high PD-L1 expression, and in combination with CT in squamous and non-squamous hystologies regardless of the level of PD-L1 expression.

scholarly journals Sirenomelia: two case reports

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Asiyeh Shojaee ◽  
Firooze Ronnasian ◽  
Mahdiyeh Behnam ◽  
Mansoor Salehi
Keyword(s):  
Case Reports ◽  
Three Dimensional ◽  
First Trimester ◽  
Mendelian Inheritance ◽  
Lower Limbs ◽  
Congenital Deformity ◽  
White Family ◽  
Her Family ◽  
Caudal Regression ◽  
History Of

AbstractBackgroundSirenomelia, also called mermaid syndrome, is a rare lethal multi-system congenital deformity with an incidence of one in 60,000–70,000 pregnancies. Sirenomelia is mainly characterized by the fusion of lower limbs and is widely associated with severe urogenital and gastrointestinal malformations. The presence of a single umbilical artery derived from the vitelline artery is the main anatomical feature distinguishing sirenomelia from caudal regression syndrome. First-trimester diagnosis of this disorder and induced abortion may be the safest medical option. In this report, two cases of sirenomelia that occurred in an white family will be discussed.Case presentationWe report two white cases of sirenomelia occurring in a 31-year-old multigravid pregnant woman. In the first pregnancy (18 weeks of gestation) abortion was performed, but in the third pregnancy (32 weeks) the stillborn baby was delivered by spontaneous vaginal birth. In the second and fourth pregnancies, however, she gave birth to normal babies. Three-dimensional ultrasound imaging showed fusion of the lower limbs. Neither she nor any member of her family had a history of diabetes. In terms of other risk factors, she had no history of exposure to teratogenic agents during her pregnancy. Also, her marriage was non-consanguineous.ConclusionThis report suggests the existence of a genetic background in this mother with a Mendelian inheritance pattern of 50% second-generation incidence in her offspring.

Jam processing: Effect of pectin replacement by locust bean gum on its characteristics

2020 ◽  
pp. 1-12
Author(s):  
Aida Mekhoukhe ◽  
Nacer Mohellebi ◽  
Tayeb Mohellebi ◽  
Leila Deflaoui-Abdelfettah ◽  
Sonia Medouni-Adrar ◽  
...  
Keyword(s):  
Cold Water ◽  
Physicochemical Characteristics ◽  
Water Soluble ◽  
Sensory Profile ◽  
Locust Bean Gum ◽  
Locust Bean ◽  
Preference Map ◽  
The Right ◽  
High Level ◽  
The Impact

OBJECTIVE: the present work proposed to extract Locust Bean Gum (LBG) from Algerian carob fruits, evaluate physicochemical and rheological properties (solubility). It aimed also to develop different formulations of strawberry jams with a mixture of LBG and pectin in order to obtain a product with a high sensory acceptance. METHODS: the physicochemical characteristics of LBG were assessed. The impact of temperature on solubility was also studied. The physical and the sensory profile and acceptance of five Jams were evaluated. RESULTS: composition results revealed that LBG presented a high level of carbohydrate but low concentrations of fat and ash. The LBG was partially cold-water-soluble (∼62% at 25°C) and needed heating to reach a higher solubility value (∼89% at 80 °C). Overall, the sensorial acceptances decreased in jams J3 which was formulated with 100% pectin and commercial one (J5). The external preference map explained that most consumers were located to the right side of the map providing evidence that most samples appreciated were J4 and J2 (rate of 80–100%). CONCLUSION: In this investigation, the LBG was used successfully in the strawberry jam’s formulation.

scholarly journals When brain damage “improves” perception: neglect patients can localize motion-shifted probes better than controls

2015 ◽  
Vol 114 (6) ◽  
pp. 3351-3358 ◽  
Author(s):  
Stefania de Vito ◽  
Marine Lunven ◽  
Clémence Bourlon ◽  
Christophe Duret ◽  
Patrick Cavanagh ◽  
...  
Keyword(s):  
Right Hemisphere ◽  
Visual Fields ◽  
Critical Factor ◽  
Significant Shift ◽  
Stimulus Motion ◽  
Attentional Processes ◽  
Position Shift ◽  
The Right ◽  
High Level ◽  
Better Than

When we look at bars flashed against a moving background, we see them displaced in the direction of the upcoming motion (flash-grab illusion). It is still debated whether these motion-induced position shifts are low-level, reflexive consequences of stimulus motion or high-level compensation engaged only when the stimulus is tracked with attention. To investigate whether attention is a causal factor for this striking illusory position shift, we evaluated the flash-grab illusion in six patients with damaged attentional networks in the right hemisphere and signs of left visual neglect and six age-matched controls. With stimuli in the top, right, and bottom visual fields, neglect patients experienced the same amount of illusion as controls. However, patients showed no significant shift when the test was presented in their left hemifield, despite having equally precise judgments. Thus, paradoxically, neglect patients perceived the position of the flash more veridically in their neglected hemifield. These results suggest that impaired attentional processes can reduce the interaction between a moving background and a superimposed stationary flash, and indicate that attention is a critical factor in generating the illusory motion-induced shifts of location.

scholarly journals The Rapid Extraction of Gist—Early Neural Correlates of High-level Visual Processing

2012 ◽  
Vol 24 (2) ◽  
pp. 521-529 ◽  
Author(s):  
Frank Oppermann ◽  
Uwe Hassler ◽  
Jörg D. Jescheniak ◽  
Thomas Gruber
Keyword(s):  
Visual Processing ◽  
Time Course ◽  
Occipital Cortex ◽  
Stimulus Onset ◽  
Gamma Band ◽  
Oscillatory Activity ◽  
Rapid Extraction ◽  
The Right ◽  
High Level ◽  
Individual Objects

The human cognitive system is highly efficient in extracting information from our visual environment. This efficiency is based on acquired knowledge that guides our attention toward relevant events and promotes the recognition of individual objects as they appear in visual scenes. The experience-based representation of such knowledge contains not only information about the individual objects but also about relations between them, such as the typical context in which individual objects co-occur. The present EEG study aimed at exploring the availability of such relational knowledge in the time course of visual scene processing, using oscillatory evoked gamma-band responses as a neural correlate for a currently activated cortical stimulus representation. Participants decided whether two simultaneously presented objects were conceptually coherent (e.g., mouse–cheese) or not (e.g., crown–mushroom). We obtained increased evoked gamma-band responses for coherent scenes compared with incoherent scenes beginning as early as 70 msec after stimulus onset within a distributed cortical network, including the right temporal, the right frontal, and the bilateral occipital cortex. This finding provides empirical evidence for the functional importance of evoked oscillatory activity in high-level vision beyond the visual cortex and, thus, gives new insights into the functional relevance of neuronal interactions. It also indicates the very early availability of experience-based knowledge that might be regarded as a fundamental mechanism for the rapid extraction of the gist of a scene.

scholarly journals Hierarchical decision processes that operate over distinct timescales underlie choice and changes in strategy

2016 ◽  
Vol 113 (31) ◽  
pp. E4531-E4540 ◽  
Author(s):  
Braden A. Purcell ◽  
Roozbeh Kiani
Keyword(s):  
Decision Making ◽  
Neural Mechanism ◽  
Decision Processes ◽  
Response Strategy ◽  
Limited Information ◽  
Decision Strategy ◽  
Stimulus Response ◽  
Hierarchical Decision ◽  
The Right ◽  
High Level

Decision-making in a natural environment depends on a hierarchy of interacting decision processes. A high-level strategy guides ongoing choices, and the outcomes of those choices determine whether or not the strategy should change. When the right decision strategy is uncertain, as in most natural settings, feedback becomes ambiguous because negative outcomes may be due to limited information or bad strategy. Disambiguating the cause of feedback requires active inference and is key to updating the strategy. We hypothesize that the expected accuracy of a choice plays a crucial rule in this inference, and setting the strategy depends on integration of outcome and expectations across choices. We test this hypothesis with a task in which subjects report the net direction of random dot kinematograms with varying difficulty while the correct stimulus−response association undergoes invisible and unpredictable switches every few trials. We show that subjects treat negative feedback as evidence for a switch but weigh it with their expected accuracy. Subjects accumulate switch evidence (in units of log-likelihood ratio) across trials and update their response strategy when accumulated evidence reaches a bound. A computational framework based on these principles quantitatively explains all aspects of the behavior, providing a plausible neural mechanism for the implementation of hierarchical multiscale decision processes. We suggest that a similar neural computation—bounded accumulation of evidence—underlies both the choice and switches in the strategy that govern the choice, and that expected accuracy of a choice represents a key link between the levels of the decision-making hierarchy.

Abstract TP156: Robot-Guided Ankle and Knee Therapeutic Training Improves Motor Functions in Stroke

Stroke ◽  
2016 ◽  
Vol 47 (suppl_1) ◽  
Author(s):  
Yunju Lee ◽  
Kai Chen ◽  
Richard L Harvey ◽  
Elliot J Roth ◽  
Li-Qun Zhang
Keyword(s):  
Range Of Motion ◽  
Knee Flexion ◽  
Haptic Feedback ◽  
Berg Balance Scale ◽  
Lower Limbs ◽  
Active Movement ◽  
Stroke Survivors ◽  
Movement Training ◽  
Knee Rehabilitation

Introduction: Stroke survivors develop substantial disability such as weakness, spasticity, increased stiffness, and reduced range of motion in lower limbs, contributing to reduced quality of life. It is important to stretch impaired ankle and knee to increase range of motion and reduce spasticity, and to conduct active movement training to improve balance and locomotion. Hypothesis: We addressed the hypotheses that robot-aided ankle and knee training will reduce motor impairments and improve balance and gait functions, and the improvements will maintain to the 6-weeks follow-up. Methods: Seven male stroke survivors participated in the robot-guided ankle and knee rehabilitation training using a pair of ankle and knee rehabilitation robots over 18 training sessions (3 sessions/week for 6 weeks). Three evaluations were done before and after training, and 6 weeks follow-up. Each session involved passive stretching under intelligent control and active movement training under real-time, audiovisual and haptic feedback. About equal time was spent on the ankle and knee training. Results: We found significant improvement in 6-Minute Walk Test (6MWT: 294.8 m pre-training to 386.4 m post training; p<0.01), Berg Balance Scale (BBS, 45 pre to 52 post; p<0.05), ankle active range of motion (AROM) (-11.7° pre to 1.7° post; p<0.05, a negative value means not being able to reach 0° dorsiflexion), passive ROM in dorsiflexion (12.7° pre to 23.3° post; p<0.01), and dorsiflexion muscle strength (-0.3 Nm pre to 5.7 Nm post; p<0.05, negative means lower than the passive torque at 0° ankle dorsiflexion). The knee had significant improvement in AROM in extension against the load of the robot (34.8° pre to 15.9° knee flexion post; p<0.05) and maximal flexion strength at 90° knee flexion (19.3 Nm pre to 31.7 Nm post; p<0.01). At the follow-up, the outcomes were found as similar results of post evaluation, e.g., 379m (p<0.05) in 6MWT, 51 (p<0.05) in BBS, and 5.2 Nm (p=0.05) in dorsiflexion strength. Conclusions: In conclusions, robot-guided stretching and active movement training reduced impairments at the knee and ankle of stroke survivors resulting in improved mobility. Furthermore, the effect of training was maintained at the 6-weeks follow-up after the treatment.

Foix-Chavany-Marie syndrome caused by a disconnection between the right pars opercularis of the inferior frontal gyrus and the supplementary motor area

2012 ◽  
Vol 117 (5) ◽  
pp. 844-850 ◽  
Author(s):  
Juan Martino ◽  
Enrique Marco de Lucas ◽  
Francisco Javier Ibáñez-Plágaro ◽  
José Manuel Valle-Folgueral ◽  
Alfonso Vázquez-Barquero
Keyword(s):  
Supplementary Motor Area ◽  
Diffusion Tensor ◽  
Anterior Part ◽  
Inferior Frontal Gyrus ◽  
Motor Area ◽  
Lower Limbs ◽  
Rare Type ◽  
Orofacial Movement ◽  
Pars Opercularis ◽  
The Right

Foix-Chavany-Marie syndrome (FCMS) is a rare type of suprabulbar palsy characterized by an automaticvoluntary dissociation of the orofacial musculature. Here, the authors report an original case of FCMS that occurred intraoperatively while resecting the pars opercularis of the inferior frontal gyrus. This 25-year-old right-handed man with an incidentally diagnosed right frontotemporoinsular tumor underwent surgery using an asleep-awake-asleep technique with direct cortical and subcortical electrical stimulation and a transopercular approach to the insula. While resecting the anterior part of the pars opercularis the patient suffered sudden anarthria and bilateral facial weakness. He was unable to speak or show his teeth on command, but he was able to voluntarily move his upper and lower limbs. This syndrome lasted for 8 days. Postoperative diffusion tensor imaging tractography revealed that connections of the pars opercularis of the right inferior frontal gyrus with the frontal aslant tract (FAT) and arcuate fasciculus (AF) were damaged. This case supplies evidence for localizing the structural substrate of FCMS. It was possible, for the first time in the literature, to accurately correlate the occurrence of FCMS to the resection of connections between the FAT and AF, and the right pars opercularis of the inferior frontal gyrus. The FAT has been recently described, but it may be an important connection to mediate supplementary motor area control of orofacial movement. The present case also contributes to our knowledge of complication avoidance in operculoinsular surgery. A transopercular approach to insuloopercular gliomas can generate FCMS, especially in cases of previous contralateral lesions. The prognosis is favorable, but the patient should be informed of this particular hazard, and the surgeon should anticipate the surgical strategy in case the syndrome occurs intraoperatively in an awake patient.

Simulation and Implementation of HLA-Based Branch Predictor of Multi-Pipeline Processor

2012 ◽  
Vol 204-208 ◽  
pp. 4952-4957
Author(s):  
Ji Hua Ye ◽  
Qi Xie ◽  
Yao Hong Xiahou
Keyword(s):  
Simulation System ◽  
New Method ◽  
High Level Architecture ◽  
Hit Rate ◽  
Branch Predictor ◽  
The Right ◽  
High Level ◽  
Two Sides

Researched how the multi-pipeline processor accelerates the running of thread ,found that when the branch predictor facing the random branch instruction, the hit rate will become very low, so bring out a new method that using the free pipeline to accelerate the running of branch instruction. If the right prediction from branch predictor is less than 70% and there is a free pipeline, then using two pipelines to run the two sides of a branch instruction at the same time. In order to test the new method, the HLA (High Level architecture) architecture-based simulation system is established, the results show that the new method can really reduce the time when processing the random branch instructions.

scholarly journals A Case of Multiple Posterior Intercostal Artery Common Trunks in Conjunction with Additional Arterial Variations

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Nicholas R. Fanselow ◽  
Nolan Wallace ◽  
Daniel Sehi ◽  
Lokesh Coomar ◽  
John Martin ◽  
...  
Keyword(s):  
Vertebral Artery ◽  
Intercostal Space ◽  
Thoracic Wall ◽  
Thoracic Cavity ◽  
Arterial Variation ◽  
Arterial Variations ◽  
First Case ◽  
Male Cadaver ◽  
The Right ◽  
High Level

Several thoracic vasculature variations were observed in an 81-year-old male cadaver during routine dissection. These included 5 common trunks of posterior intercostal arteries, a descending branch of the right vertebral artery, and atypical neurovascular relationships within intercostal spaces. On the right side, two common trunks of posterior intercostal arteries were observed supplying the 4th-7th intercostal spaces and 9th-11th intercostal spaces, respectively. There was also a small accessary branch supplying the 9th intercostal space. The first three posterior intercostal spaces on the right were supplied by a descending branch of the vertebral artery. On the left side, three common trunks of posterior intercostal arteries were encountered, supplying intercostal spaces 3-5, 6-7, and 11 plus the subcostal space. An atypical neurovascular relationship was observed in the right 6th intercostal space, as well as the left 2nd, 3rd, and 6th intercostal spaces. This is the first case report that presents 5 common trunks of posterior intercostal arteries, as well as common trunks in conjunction with other arterial variation in the posterior thoracic wall. These variations carry a high level of clinical significance and may be helpful in guiding decision-making related to surgical procedures related to the posterior thoracic cavity and spine.

Sign in / Sign up

Export Citation Format

Share Document