Articular Syndrome Characteristics in Children with Mucopolysaccharidosis Type I

Background. Mucopolysaccharidosis type I is disease from the group of lysosomal storage disease developing due to mutations in the IDUA gene. It leads to the accumulation of glycosaminoglycans (GAGs) in organs and tissues. Joints damage in this disease is systemic and progressive.Objective. The aim of the study. Nowadays, relevant issue is to investigate the effects of various types of pathogenetic therapy on the state of the osteoarticular system in patients with severe and mild phenotypes of MPS I to prevent further progression of joint pathology.Methods. The study included 46 patients diagnosed with “mucopolysaccharidosis type I”. 35 children had severe phenotype (Hurler syndrome) and 11 — with mild phenotypes (Hurler-Scheie and Scheie syndromes). The onset age of clinical manifestations in osteoarticular system, the state of large and small joints, and the presence of cervical stenosis according to the therapy were evaluated in these patients.Results. The osteoarticular system pathology can be usually revealed in all patients with MPS I, in both mild and severe phenotypes. The contractures of shoulder, ulnar, wrist, and small hand joints have been revealed in most patients with Hurler syndrome, regardless of the administered therapy. Hip joints pathology was observed in children who was administered with: enzyme replacement therapy (ERT) — in 46.7% of cases, hematopoietic stem cell transplantation (HSCT) in combination with ERT — in 34.4% of cases. Patients with Hurler syndrome administered with HSCT in combination with ERT had cervical stenosis statistically significantly more rarely (p = 0.018) compared to patients treated with ERT only. Patients with Hurler syndrome who were on ERT had statistically significantly lower growth rates than patients after HSCT in combination with ERT. Lesions in ulnar, wrist, knee and small hand joints were the most common in children with mild phenotypes (in 90% of cases).Conclusion. Combined therapy (HSCT and ERT) in patients with Hurler syndrome reduces severe manifestations in osteoarticular system.

Rheumatoid arthritis onset after COVID-19 infection: a case report

At the end of 2019, coronavirus disease (COVID-19) outbreak is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV2). Worldwide researchers and physician try to explore the mechanisms of damage induced by virus, they focus on the short-term and long-term immune-mediated consequences induced by the virus infection. Every day discover a new pathological condition induced by virus and new symptoms and disease may occur after recovery from disease. Our case report is 41 years old, Indian lady who presented to our primary health care centre complaining of multiple small hand joints pain, both elbows and knees pain with swelling of them and prolonged morning stiffness, diagnosed seropositive rheumatoid arthritis (RA) (arthritis, positive rheumatoid factor (RF), and X-ray changes) after 1 month recovery from COVID-19 infection. She did not have any joint pain and she had negative RF before COVID-19 infection with no family history of RA.

‘The End Lies in the Beginning’: Embracing Childhood and Old Age in Susan Hill's Ghost Novels The Small Hand and Dolly

Susan Hill's novels The Small Hand (2010) and Dolly (2012) evoke Victorian tropes of age inversion drawn from children's literature and ghost narratives that undermine the boundaries established between childhood and old age. Given their neo-Victorian features, Hill's two novels engage in dialogue with these Victorian tropes, but, in comparison, Hill's spectral entities literally denote that these life stages are interrelated and should be embraced.

AB0183 T-CELL LARGE GRANULAR LYMPHOCYTE LEUKEMIA IN PATIENTS WITH FELTY’S SYNDROME

Background:Felty’s syndrome (FS) is a rare subtype of seropositive rheumatoid arthritis (RA) and is characterized by neutropenia and splenomegaly. Some researchers suggest that FS and T-cell large granular leukemia (T-LGLL) may have common pathogenetic relationships [1].Objectives:to characterize the clinical and laboratory manifestations of FS, to evaluate the frequency of T-LGLL and Sjogren’s syndrome (SS) in this group of patients with RA, neutropenia and splenomegaly (pts).Methods:We observed 18 pts with ACCP-positive (100%) and RF-positive (94.5%) RA diagnosed according to ACR 2010 criteria, who also had neutropenia and hepatosplenomegaly. All 18 pts underwent T-LGLL diagnostics using blood smears and phenotyping of peripheral lymphocytes for the presence of granular lymphocytes, determination of T-cell clonality using the rearrangement of the γ-chain of the T-cell receptor of lymphocytes by PCR, histological/immunohistochemical study of bone marrow biopsy specimens for the presence of LGL invasion studies, as well as the study of 4 spleens after splenectomy. SS was diagnosed in 8 out of 18 pts (44.5%) according ACR 2016 criteria.Results:Twelve (66.6%) of 18 pts with RA, neutropenia and splenomegaly were diagnosed with T-LGLL, the patients were divided in 2 groups: FS (6 pts) and RA+T-LGL (12 pts). Pts with FS debuted with arthritis of small hand joints, extremely rarely with extra-articular manifestations, mainly at a young age (36.5±3.9 years), and developed neutropenia after 10 years of RA. Pts with T-LGLL debuted at a younger age (39,5±4,5 and 51,5 + 7,8 years, respectively), had a longer course of RA before the development of neutropenia (14.3±3.3 and 5±1.5 years, respectively, p=0.03), and more often had extra-articular manifestations at the onset of the disease. RA activity did not differ between groups and in most cases was characterized by a mild course of articular syndrome. Though the course of RA+T-LGLL group was characterized by low (50%) and moderate (33%) DAS28-CRP activity and active synovitis in only 41.5% of pts, severe joint deformities (stage III and IV) developed in 58.5% of pts. Pts with T-LGLL showed a higher incidence of hepatomegaly (75% and 16.5%, respectively, p=0.02) and more severe neutropenia (p=0.02). The development of severe leukopenia (<1x109) and massive hepatosplenomegaly was observed only in pts with T-LGLL, which required splenectomy in 4 cases. SS was more often detected in the FS group than in the RA+T-LGLL group (83.5% and 25%, respectively, p = 0.02).Conclusion:Clinical and laboratory manifestations of FS and T-LGLL are extremely close, therefore, pts who are diagnosed with FS should be examined to exclude T-LGLL.References:[1]Liu, Xin, and Thomas P Loughran Jr. “The spectrum of large granular lymphocyte leukemia and Felty’s syndrome.” Current opinion in hematology vol. 18,4 (2011): 254-9. doi:10.1097/MOH.0b013e32834760fb.Disclosure of Interests:None declared

Production Activities and Value Chain Analysis of Sericulture in Western Inner Terai Region of Nepal

The study was conducted in Nov 2019 – Feb 2020 with the objective of assessing the whole value chain of sericulture products in the Western inner terai region of Nepal. The beginning of sericulture in the region was due to some personal interest but gained popularity and huge return and turned out to achieve the objectives of food security and source of employment. As per the finding and research, Bi-voltine Silkworm (Bombyx mori) was mainly reared which feeds upon the leaves of the mulberry plant. The interview result indicates that producers produce cocoon at an average of 30.05 kg per ropani in one production round and production was made twice a year. In this region, three marketing channels were identified (producer to the primary collector/small hand processor) and (producer to primary processor or reelers) and the producer himself as the processor and distributor. Producers sold the cocoon to primary collectors at NRs 500per kg cocoon which was 15% more than percent sales while selling to the government, fetching an average BC ratio of 1.3. Problems recorded as per farmers were lack of appropriate technology to widen the scope of sericulture and modern silkworm rearing techniques, lack of proper irrigation facilities, and governmental aids and support. This research will address various problems and make an emphasis to make regional sericulture production mature and profitable.

A QR Code Based Group Pairing Approach for Mobile Ad Hoc Networks

Due to the rapid growth of small and smart hand-held devices, mobile ad hoc networks (MANets) are becoming very common nowadays. MANets may consist of a number of small hand-held devices having limited resources in terms of memory, battery and processing power. In order to provide services to the users, these devices are capable of communicating with each other through some radio technology, such as WiFi, Bluetooth or Infrared. Since radio channels are inherently vulnerable to various security threats, it requires that devices in MANets must establish a secure association amongst themselves before exchanging any sensitive information or data. The process of establishing a secure channel between two devices is referred to as device pairing or device association. Device pairing do not rely on traditional mechanisms for security due to the impulsive and ad hoc interactions among the devices. Due to this, researchers have proposed many schemes/protocols to deal with this issue; however, the issue of group pairing (i.e. secure association of more than two devices) is less addressed issue in the literature yet. There could be many scenarios (such as confidential office meetings, paring of group of home appliances in smart-homes, etc) of MANets, where secure group communications is desired. Consequently, this research focuses on this issue and proposes a QR (quick response) code based scheme to establish a secure channel between a numbers of devices. The proposed system is implemented and tested on modern hand-held devices and a usability study of the implemented system is also carried out.

Sonographic Diagnosis of COVID-19: A Review of Image Processing for Lung Ultrasound

The sustained increase in new cases of COVID-19 across the world and potential for subsequent outbreaks call for new tools to assist health professionals with early diagnosis and patient monitoring. Growing evidence around the world is showing that lung ultrasound examination can detect manifestations of COVID-19 infection. Ultrasound imaging has several characteristics that make it ideally suited for routine use: small hand-held systems can be contained inside a protective sheath, making it easier to disinfect than X-ray or computed tomography equipment; lung ultrasound allows triage of patients in long term care homes, tents or other areas outside of the hospital where other imaging modalities are not available; and it can determine lung involvement during the early phases of the disease and monitor affected patients at bedside on a daily basis. However, some challenges still remain with routine use of lung ultrasound. Namely, current examination practices and image interpretation are quite challenging, especially for unspecialized personnel. This paper reviews how lung ultrasound (LUS) imaging can be used for COVID-19 diagnosis and explores different image processing methods that have the potential to detect manifestations of COVID-19 in LUS images. Then, the paper reviews how general lung ultrasound examinations are performed before addressing how COVID-19 manifests itself in the images. This will provide the basis to study contemporary methods for both segmentation and classification of lung ultrasound images. The paper concludes with a discussion regarding practical considerations of lung ultrasound image processing use and draws parallels between different methods to allow researchers to decide which particular method may be best considering their needs. With the deficit of trained sonographers who are working to diagnose the thousands of people afflicted by COVID-19, a partially or totally automated lung ultrasound detection and diagnosis tool would be a major asset to fight the pandemic at the front lines.

GPU-accelerated periodic source identification in large-scale surveys: measuring P and P

ABSTRACT Many inspiraling and merging stellar remnants emit both gravitational and electromagnetic radiation as they orbit or collide. These gravitational wave events together with their associated electromagnetic counterparts provide insight about the nature of the merger, allowing us to further constrain properties of the binary. With the future launch of the Laser Interferometer Space Antenna (LISA), follow-up observations and models are needed of ultracompact binary (UCB) systems. Current and upcoming long baseline time domain surveys will observe many of these UCBs. We present a new fast periodic object search tool capable of searching for generic periodic signals based on the conditional entropy algorithm. This new implementation allows for a grid search over both the period (P) and the time derivative of the period ($\dot{P}$). To demonstrate the usage of this tool, we use a small, hand-picked subset of a UCB population generated from the population synthesis code cosmic , as well as a custom catalogue for varying periods at fixed intrinsic parameters. We simulate light curves as likely to be observed by future time domain surveys by using an existing eclipsing binary light-curve model accounting for the change in orbital period due to gravitational radiation. We find that a search with $\dot{P}$ values is necessary for detecting binaries at orbital periods less than ∼10 min. We also show it is useful in finding and characterizing binaries with longer periods, but at a higher computational cost. Our code is called gce (GPU-accelerated Conditional Entropy). It is available on Github (https://github.com/mikekatz04/gce).

300,000-year-old wooden tools from Gantangqing, southwest China

Early Palaeolithic wooden implements are exceptionally rare. The best known are the spears and throwing sticks from Schöningen, Germany dated to ca. 300–330 thousand years (kyr) ago 1,2 and the 171-kyr old digging sticks from Poggetti Vecchi, Italy 3. Here, we report a unique assemblage of 35 wooden implements from the site of Gantangqing, southwestern China, which was found associated with stone tools, antler soft hammers and cut-marked bones, and is dated to ca. 361–250 kyr at 95% probability. The wooden implements include digging sticks and complete, small, hand-held pointed tools. The tips of several had starch grains of plants with starch-rich rhizomes. We suggest that most of these tools were used for digging the rhizomes of sub-aquatic plants on the edges of an ancient lake. This discovery provides the earliest tangible evidence for the exploitation of sub-surface plant foods in the Middle Pleistocene. It also highlights the probable role of plant foods (especially underground storage organs [USOs]) in sub-tropical and tropical environments in which a wide variety of edible plants were available, and implies that hominins had a detailed knowledge of which plants, and which parts of plants, were edible. These tools also show that organic materials played an important role in Middle Pleistocene technology, and the sophistication of many of these tools off-sets the seemingly “primitive” design of stone tools in the East Asian Early Palaeolithic.

Certification of a Pilot with Charcot-Marie-Tooth Disease

BACKGROUND: Charcot-Marie-Tooth disease (CMT) is a rare hereditary motor and sensory neuropathy. This is a report of a pilot with this condition with a discussion of the challenges for the regulator in the assessment for medical certification of pilots with a neurological disability.CASE REPORT: A pilot with CMTX1 declared his condition to the United Kingdom Civil Aviation Authority when his brother was diagnosed with the same condition. Apart from high arched feet and some difficulty playing sports, he had no problems until his mid-forties, when he very slowly developed increasing weakness with foot dorsiflexion and later wasting and weakness of the small hand muscles. He reported no problems with any flying activity. On clinical examination, it seemed likely that the disability would have an impact on his ability to undertake all the flying tasks of a commercial pilot, including those required in emergencies.DISCUSSION: A modified Medical Flight Test (MFT) specifically tailored by the regulator to test areas of functional impairment allowed the successful certificatory assessment of a pilot with this condition; an approach which could apply to any pilot with a rare neurological disability.Jagathesan T, OBrien M, Rattray A. Certification of a pilot with Charcot-Marie-Tooth disease. Aerosp Med Hum Perform. 2021; 92(2):124126.