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https://www.readbyqxmd.com/read/29296930/gene-transfer-into-hematopoietic-stem-cells-reduces-hlh-manifestations-in-a-murine-model-of-munc13-4-deficiency
#1
Tayebeh Soheili, Amandine Durand, Fernando E Sepulveda, Julie Rivière, Chantal Lagresle-Peyrou, Hanem Sadek, Geneviève de Saint Basile, Samia Martin, Fulvio Mavilio, Marina Cavazzana, Isabelle André-Schmutz
Patients with mutations in the UNC13D gene (coding for Munc13-4 protein) suffer from familial hemophagocytic lymphohistiocytosis type 3 (FHL3), a life-threatening immune and hyperinflammatory disorder. The only curative treatment is allogeneic hematopoietic stem cell (HSC) transplantation, although the posttreatment survival rate is not satisfactory. Here, we demonstrate the curative potential of UNC13D gene correction of HSCs in a murine model of FHL3. We generated a self-inactivating lentiviral vector, used it to complement HSCs from Unc13d-deficient (Jinx) mice, and transplanted the cells back into the irradiated Jinx recipients...
December 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29290558/efficient-hydrogen-dependent-carbon-dioxide-reduction-by-escherichia-coli
#2
Magali Roger, Fraser Brown, William Gabrielli, Frank Sargent
Hydrogen-dependent reduction of carbon dioxide to formic acid offers a promising route to greenhouse gas sequestration, carbon abatement technologies, hydrogen transport and storage, and the sustainable generation of renewable chemical feedstocks [1]. The most common approach to performing direct hydrogenation of CO2 to formate is to use chemical catalysts in homogeneous or heterogeneous reactions [2]. An alternative approach is to use the ability of living organisms to perform this reaction biologically. However, although CO2 fixation pathways are widely distributed in nature, only a few enzymes have been described that have the ability to perform the direct hydrogenation of CO2 [3-5]...
December 20, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/29285554/hallux-saltans-due-to-stenosing-tenosynovitis-of-flexor-hallucis-longus-6-dynamic-sonography-and-arthroscopic-findings
#3
Edgar Leonardo Martinez-Salazar, Joao R T Vicentini, Anne H Johnson, Martin Torriani
Triggering of the toes is rare, with isolated cases reported in the literature involving predominantly the flexor hallucis longus (FHL) tendon, a condition known as "hallux saltans" (HS). We report the dynamic sonographic findings of a 42-year-old female with a 2-month history of ankle pain and triggering of right hallux, consistent with HS. Sonography demonstrated tenosynovitis and focal thickening of FHL at the level of hindfoot, with a fibrous band adjacent to the tendon. Dynamic ultrasound showed triggering and snapping of FHL tendon during hallux flexion and extension, respectively...
December 28, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/29260197/the-intraocular-lens-as-a-drug-delivery-device-in-vitro-screening-of-pharmacologic-substances-for-the-prophylaxis-of-posterior-capsule-opacification
#4
Christian Wertheimer, Stefan Kassumeh, Nick P Piravej, Olga Nilmayer, Christian Braun, Claudia Priglinger, Nikolaus Luft, Armin Wolf, Wolfgang J Mayer, Siegfried G Priglinger, Kirsten H Eibl-Lindner
Purpose: Numerous pharmacologic substances have been proposed for preventing posterior capsule opacification (PCO). The following trial was to compare those drugs to find more suitable options. IOL should then be modified by the pharmaceuticals as a drug-delivery device. Methods: A systematic literature search was performed to identify published substances. FHL-124 was used to determine cell proliferation and toxicity using a dye reduction test (XTT). Prescreened substances showing a reduction on cell growth without being toxic were soaked into an IOL...
December 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29235714/formate-hydrogenlyase-and-formate-secretion-ameliorate-h2-inhibition-in-the-hyperthermophilic-archaeon-thermococcus-paralvinellae
#5
Begüm D Topçuoğlu, Cem Meydan, Roberto Orellana, James F Holden
Some hyperthermophilic heterotrophs in the genus Thermococcus produce H2 in the absence of S° and have up to seven hydrogenases, but their combined physiological roles are unclear. Here, we show which hydrogenases in Thermococcus paralvinellae are affected by added H2 during growth without S°. Growth rates and steady-state cell concentrations decreased while formate production rates increased when T. paralvinallae was grown in a chemostat with 65 µM of added H2(aq) . Differential gene expression analysis using RNA-Seq showed consistent expression of six hydrogenase operons with and without added H2 ...
December 13, 2017: Environmental Microbiology
https://www.readbyqxmd.com/read/29216683/human-perforin-gene-variation-is-geographically-distributed
#6
Robin C Willenbring, Yasuhiro Ikeda, Larry R Pease, Aaron J Johnson
BACKGROUND: Deleterious mutations in PRF1 result in lethal, childhood disease, familial hemophagocytic lymphohistiocytosis type 2 (FHL 2). However, not all mutations in PRF1 are deleterious and result in FHL 2. Currently, these nondeleterious mutations are being investigated in the onset of numerous disorders, such as lymphomas and diabetes. Yet, there is still an overwhelmingly large amount of PRF1 mutations that are not associated with disease. METHODS: We conducted a post hoc analysis of the PRF1 mutations in the coding region using the recently published Exome Aggregation Consortium genomes, Leiden Open Variation Database, NCBI SNP database, and primary literature to better understand PRF1 variation in the human population...
December 7, 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29142095/patterns-of-intermuscular-inhibitory-force-feedback-across-cat-hindlimbs-suggest-a-flexible-system-for-regulating-whole-limb-mechanics
#7
Mark A Lyle, T Richard Nichols
Prior work has suggested that Golgi tendon organ feedback, via its distributed network linking muscles spanning all joints, could be used by the nervous system to help regulate whole limb mechanics if appropriately organized. We tested this hypothesis by characterizing the patterns of intermuscular force dependent feedback between the primary extensor muscles spanning the knee, ankle and toes in decerebrate cat hindlimbs. Intermuscular force feedback was evaluated by stretching tendons of selected muscles in isolation and in pairwise combinations and then measuring the resulting force dependent intermuscular interactions...
November 15, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/29110725/tendon-split-lengthening-technique-for-flexor-hallucis-longus-tendon-rupture
#8
Jae Yong Park, Chenyu Wang, Hee Dong Kim, Hyong Nyun Kim
BACKGROUND: Flexor hallucis longus (FHL) tendon rupture is a challenging injury to lead with clawing of the great toe when the FHL tendon is repaired too tight. When the diagnosis is delayed, the tendon ends may not be opposable because of contracture or poor tendon tissue. METHODS: A technique to reconstruct FHL tendon rupture without a free tendon graft is described. A split tendon lengthening is performed at the midfoot around the knot of Henry. Ankle block anesthesia is used to allow the patient's active movement of the interphalangeal (IP) joint to determine the appropriate length of the reconstructed tendon for maintaining balance and preventing the tendon from being too tight or too loose...
November 6, 2017: Journal of Orthopaedic Surgery and Research
https://www.readbyqxmd.com/read/29079740/anatomical-study-of-the-neurovascular-in-flexor-hallucis-longus-tendon-transfers
#9
Haijiao Mao, Wenwei Dong, Zengyuan Shi, Weigang Yin, Dachuan Xu, Keith L Wapner
The transfer of the flexor hallucis longus tendon or flexor digitorum longus tendon is frequently used for the treatment of posterior tibial tendon insufficiency or chronic Achilles tendinopathy. According to several anatomical studies, harvesting the flexor hallucis longus (FHL) tendon may cause nerve injury. Sixty-eight embalmed feet were dissected and anatomically classified to define the relationship between Henry's knot and the plantar nerves. Two different configurations were identified. In Pattern 1, which was observed in 64 specimens (94...
October 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29078829/what-do-you-do-with-the-achilles-if-you-have-no-fancy-toys
#10
REVIEW
Rajiv Shah, Sampat Dumbre Patil
Surgical management of Achilles disorders warrants excision of the degenerated tendon and removal of impinging bone. Resulting defects can be bridged by various methods. Although FHL is the most commonly used tendon for transfer, large defects in cases of chronic Achilles ruptures may be bridged by use of a distant donor tendon. Bony anchorage of a lengthened or transferred tendon into the calcaneus can be done with suture anchors or with interference screws. In developing countries, such implants may not be available or affordable, necessitating the adoption of innovative ways to anchor tendons into the calcaneus...
December 2017: Foot and Ankle Clinics
https://www.readbyqxmd.com/read/29022534/therapeutic-drug-monitoring-of-amlodipine-and-the-z-fhl-hhl-ratio-adherence-tools-in-patients-referred-for-apparent-treatment-resistant-hypertension
#11
E S W Jones, M Lesosky, M Blockman, S Castel, E H Decloedt, S L U Schwager, E D Sturrock, L Wiesner, B L Rayner
BACKGROUND: Non-adherence to antihypertensives is a cause of 'pseudo-treatment-resistant' hypertension. OBJECTIVE: To determine whether monitoring plasma amlodipine concentrations and inhibition of angiotensin-converting enzyme (ACE) can be adjunct adherence tools. METHODS: Patients with hypertension who were prescribed enalapril and amlodipine were enrolled. Blood pressures (BPs) were monitored and an adherence questionnaire was completed...
September 22, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28983225/revision-achilles-reconstruction-with-hamstring-autograft-and-fhl-tendon-transfer-in-an-athlete
#12
Sydney C Karnovsky, Mark C Drakos
No abstract text is available yet for this article.
October 2017: HSS Journal: the Musculoskeletal Journal of Hospital for Special Surgery
https://www.readbyqxmd.com/read/28980027/-insertional-achilles-tendinopathy-differentiated-diagnostics-and-therapy
#13
REVIEW
S F Baumbach, M Braunstein, M G Mack, F Maßen, W Böcker, S Polzer, H Polzer
Achilles tendinopathy at the calcaneal insertion is classified into insertional tendinopathy, retrocalcaneal and superficial bursitis. The aim of this study was to present the current evidence on conservative and surgical treatment of insertional tendinopathy of the Achilles tendon. Conservative first-line therapy includes reduction of activity levels, administration of non-steroidal anti-inflammatory drugs (NSAID), adaptation of footwear, heel wedges and orthoses or immobilization. In addition, further conservative therapy options are also available...
October 4, 2017: Der Unfallchirurg
https://www.readbyqxmd.com/read/28936583/effective-immunological-guidance-of-genetic-analyses-including-exome-sequencing-in-patients-evaluated-for-hemophagocytic-lymphohistiocytosis
#14
Sandra Ammann, Kai Lehmberg, Udo Zur Stadt, Christian Klemann, Sebastian F N Bode, Carsten Speckmann, Gritta Janka, Katharina Wustrau, Mirzokhid Rakhmanov, Ilka Fuchs, Hans C Hennies, Stephan Ehl
We report our experience in using flow cytometry-based immunological screening prospectively as a decision tool for the use of genetic studies in the diagnostic approach to patients with hemophagocytic lymphohistiocytosis (HLH). We restricted genetic analysis largely to patients with abnormal immunological screening, but included whole exome sequencing (WES) for those with normal findings upon Sanger sequencing. Among 290 children with suspected HLH analyzed between 2010 and 2014 (including 17 affected, but asymptomatic siblings), 87/162 patients with "full" HLH and 79/111 patients with "incomplete/atypical" HLH had normal immunological screening results...
November 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28935695/confirmed-efficacy-of-etoposide-and-dexamethasone-in-hlh-treatment-long-term-results-of-the-cooperative-hlh-2004-study
#15
Elisabet Bergsten, AnnaCarin Horne, Maurizio Aricó, Itziar Astigarraga, R Maarten Egeler, Alexandra H Filipovich, Eiichi Ishii, Gritta Janka, Stephan Ladisch, Kai Lehmberg, Kenneth L McClain, Milen Minkov, Scott Montgomery, Vasanta Nanduri, Diego Rosso, Jan-Inge Henter
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the HLH-94 study, with an estimated 5-year probability of survival (pSu) of 54% (95% confidence interval, 48%-60%), systemic therapy included etoposide, dexamethasone, and, from week 9, cyclosporine A (CSA). Hematopoietic stem cell transplantation (HSCT) was indicated in patients with familial/genetic, relapsing, or severe/persistent disease. In HLH-2004, CSA was instead administered upfront, aiming to reduce pre-HSCT mortality and morbidity...
December 21, 2017: Blood
https://www.readbyqxmd.com/read/28926379/tendon-length-calf-muscle-atrophy-and-strength-deficit-after-acute-achilles-tendon-rupture-long-term-follow-up-of-patients-in-a-previous-study
#16
Juuso Heikkinen, Iikka Lantto, Juuso Piilonen, Tapio Flinkkilä, Pasi Ohtonen, Pertti Siira, Vesa Laine, Jaakko Niinimäki, Ari Pajala, Juhana Leppilahti
BACKGROUND: In this prospective study, we used magnetic resonance imaging (MRI) to assess long-term Achilles tendon length, calf muscle volume, and muscle fatty degeneration after surgery for acute Achilles tendon rupture. METHODS: From 1998 to 2001, 60 patients at our center underwent surgery for acute Achilles tendon rupture followed by early functional postoperative rehabilitation. Fifty-five patients were reexamined after a minimum duration of follow-up of 13 years (mean, 14 years), and 52 of them were included in the present study...
September 20, 2017: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/28913923/an-induced-pluripotent-stem-cell-patient-specific-model-of-complement-factor-h-y402h-polymorphism-displays-characteristic-features-of-age-related-macular-degeneration-and-indicates-a-beneficial-role-for-uv-light-exposure
#17
Dean Hallam, Joseph Collin, Sanja Bojic, Valeria Chichagova, Adriana Buskin, Yaobo Xu, Lucia Lafage, Elsje G Otten, George Anyfantis, Carla Mellough, Stefan Przyborski, Sameer Alharthi, Viktor Korolchuk, Andrew Lotery, Gabriele Saretzki, Martin McKibbin, Lyle Armstrong, David Steel, David Kavanagh, Majlinda Lako
Age-related macular degeneration (AMD) is the most common cause of blindness, accounting for 8.7% of all blindness globally. Vision loss is caused ultimately by apoptosis of the retinal pigment epithelium (RPE) and overlying photoreceptors. Treatments are evolving for the wet form of the disease; however, these do not exist for the dry form. Complement factor H polymorphism in exon 9 (Y402H) has shown a strong association with susceptibility to AMD resulting in complement activation, recruitment of phagocytes, RPE damage, and visual decline...
November 2017: Stem Cells
https://www.readbyqxmd.com/read/28863723/flexor-digitorum-longus-fdl-or-flexor-hallucis-longus-fhl-harvesting-technical-tip-and-case-studies
#18
Bruce Lehnert, Meir Nyska, Wing Ip, Em Huynh, Ezequiel Palmanovich
BACKGROUND: Harvesting of the flexor digitorum longus (FDL) or the flexor hallucis longus (FHL) is a procedure used when tendon transfer is needed. It is commonly used in tibialis posterior reconstruction and Achilles tendon reconstruction. Harvesting of these tendons is sometimes difficult and time-consuming. It is important to obtain sufficient length to make a loop around the navicular bone or anchor it in the calcaneus. We describe a technique in which a loop is passed from proximal identification of the FDL or FHL through the tendon sheath, harvesting it from a minimal plantar approach...
November 2017: Foot & Ankle International
https://www.readbyqxmd.com/read/28800559/assessment-of-health-literacy-among-outpatient-clinics-attendees-at-ain-shams-university-hospitals-egypt-a-cross-sectional-study
#19
R Almaleh, Y Helmy, E Farhat, H Hasan, A Abdelhafez
OBJECTIVES: The aim of conducting this study is to measure health literacy (HL) and to investigate the factors associated with inadequate HL in a sample of outpatient clinics attendees (i.e. patients and companions) at Ain Shams University (ASU) Hospitals. STUDY DESIGN: It is a cross-sectional study. METHODS: The study was conducted on 805 attendees of the outpatient clinics at El-Demerdash University Hospital of ASU Hospitals. The Arabic versions of the Swedish Functional Health Literacy Scale and the European Health Literacy Survey Questionnaire-short version-were used to assess HL...
October 2017: Public Health
https://www.readbyqxmd.com/read/28757574/finding-a-balance-between-protection-and-pathology-the-dual-role-of-perforin-in-human-disease
#20
REVIEW
Robin C Willenbring, Aaron J Johnson
Perforin is critical for controlling viral infection and tumor surveillance. Clinically, mutations in perforin are viewed as unfavorable, as lack of this pore-forming protein results in lethal, childhood disease, familial hemophagocytic lymphohistiocytosis type 2 (FHL 2). However, many mutations in the coding region of PRF1 are not yet associated with disease. Animal models of viral-associated blood-brain barrier (BBB) disruption and experimental cerebral malaria (ECM) have identified perforin as critical for inducing pathologic central nervous system CNS vascular permeability...
July 25, 2017: International Journal of Molecular Sciences
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