Parameningeal alveolar rhabdomyosarcoma in a child

2021 ◽  
Vol 14 (7) ◽  
pp. e243267
Author(s):  
Abhinav Kumar ◽  
Vijay Pothula
Keyword(s):  
Poor Prognosis ◽  
The Body ◽  
Alveolar Rhabdomyosarcoma ◽  
Leptomeningeal Disease ◽  
Molecular Characterisation ◽  
Loss Of Vision ◽  
Presenting Symptoms ◽  
Prompt Treatment ◽  
Atypical Presentations ◽  
The Right

This is the case of a parameningeal alveolar rhabdomyosarcoma (ARMS) in a 13-year-old boy who presented with painless loss of vision in the right eye, but very few other physical signs. The ARMS diagnosis was confirmed with imaging and molecular characterisation of the tumour. Despite tolerating the initial chemotherapy and radiotherapy regimens, there was leptomeningeal recurrence and the patient unfortunately passed away. Parameningeal ARMS occurs in an area of the body, which leads to a wide variety of possible presenting symptoms, creating a long list of differentials that can delay treatment. This tumour subtype has a poor prognosis, and due to the location of the tumour around vital structures in the head, treatment toxicities must be taken into account. This highlights the necessity for having a strong index of suspicion for this tumour in atypical presentations in children, and the necessity for prompt treatment to prevent leptomeningeal disease from occurring.

scholarly journals A Multifocal Paraganglioma in an African Male Due to an Underlying SDHB Mutation

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A104-A105
Author(s):  
Nida Mishraz Siddiqui ◽  
Faheem Seedat ◽  
Saajidah Bulbulia ◽  
Amanda Krause ◽  
Reyna Daya ◽  
...  
Keyword(s):  
Genetic Counselling ◽  
Histopathological Examination ◽  
Resource Limitation ◽  
The Body ◽  
Screening Tools ◽  
Sdhb Mutation ◽  
Abdominal Tumor ◽  
Tissue Mass ◽  
Presenting Symptoms ◽  
The Right

Abstract Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from adrenal and extra-adrenal paraganglia. Up to forty percent are due to an underlying germline mutation. Mutations in the subunit B gene of the SDH complex (SDHB) are associated with PGL syndrome four. A 34-yr-old African man from Southern Africa presented with a two-year history of sustained hypertension associated with the classic triad of sweating, headaches and palpitations. Family history was contributory towards early unexpected deaths of his father (age 42) and two younger brothers (ages 13 and 14 respectively). On examination his blood pressure was persistently elevated measuring 146/87mmHg. In view of the classic presenting symptoms and hypertension onset at a young age, a PPGL was suspected. Biochemical investigations were positive with an elevated 24-hour urine normetanephrine level of 35807 (480-2424nmol/24hours), normal metanephrine level of 689 (264-1729nmol/24hours), an elevated normetanephrine:creatinine ratio of 3270 (28-158nmol/mmol creatinine) and an elevated methoxytyramine level 4941.69nmol/24 hours (<800nmol/24 hours). Computed tomography of the abdomen and neck revealed a homogenous soft tissue mass measuring 5.9cm x 3.6cm x 6.6cm anterior to the right kidney and separate from the right adrenal gland and a carotid body tumor measuring 3.6cm x 2.9cm x 4.1cm. Both were radio-avid on a [68Gallium]-DOTATATE-Positron Emission Tomography (PET)-CT. There were no features to suggest metastatic disease. Genetic testing is not available in South Africa; therefore, testing was done at an international laboratory. This revealed a pathological SDHB mutation variant, c.724C>A p.(Arg242Ser) and hence PGL4 syndrome. The patient underwent staged surgery with successful removal of the intra-abdominal tumor. Unfortunately, due to peri-operative complications associated with the second surgery, the patient demised. Histopathological examination of both tumors was consistent with a paraganglioma. Genetic counselling and testing were offered to all living first-degree relatives. His sister tested positive for the same pathological variant. His 6-week-old son will be offered counselling and testing at a later stage. To the best of our knowledge, we are the first to describe the missense SDHB mutation (pathogenic variant c.724C>A p.[Arg242Ser]) and the occurrence of an SDHB associated PGL in a family of African ethnicity. This case highlights the importance of genetic counselling and testing in patients with a confirmed PPGL. Due to resource limitation the African population remains under represented in genetic studies which limits the utility of precision medicine in this group. As such, our case is an important addition to the body of knowledge in this growing field and highlights the need for cost effective genetic screening tools in low resourced settings.

Our Bodies, Whose Property?

2013 ◽  
Author(s):  
Anne Phillips
Keyword(s):  
Labor Markets ◽  
Personal Autonomy ◽  
The Body ◽  
Body Parts ◽  
Complex Issue ◽  
Human Organs ◽  
Property Owners ◽  
The Common ◽  
The Difference ◽  
The Right

No one wants to be treated like an object, regarded as an item of property, or put up for sale. Yet many people frame personal autonomy in terms of self-ownership, representing themselves as property owners with the right to do as they wish with their bodies. Others do not use the language of property, but are similarly insistent on the rights of free individuals to decide for themselves whether to engage in commercial transactions for sex, reproduction, or organ sales. Drawing on analyses of rape, surrogacy, and markets in human organs, this book challenges notions of freedom based on ownership of our bodies and argues against the normalization of markets in bodily services and parts. The book explores the risks associated with metaphors of property and the reasons why the commodification of the body remains problematic. The book asks what is wrong with thinking of oneself as the owner of one's body? What is wrong with making our bodies available for rent or sale? What, if anything, is the difference between markets in sex, reproduction, or human body parts, and the other markets we commonly applaud? The book contends that body markets occupy the outer edges of a continuum that is, in some way, a feature of all labor markets. But it also emphasizes that we all have bodies, and considers the implications of this otherwise banal fact for equality. Bodies remind us of shared vulnerability, alerting us to the common experience of living as embodied beings in the same world. Examining the complex issue of body exceptionalism, the book demonstrates that treating the body as property makes human equality harder to comprehend.

ANALISIS YURIDIS NORMATIF PUTUSAN PERKARA MAHKAMAH AGUNG KONSTITUSI RI No. 004/PUU-II/2004

2013 ◽  
Vol 7 (1) ◽  
pp. 7
Author(s):  
Doni Budiono
Keyword(s):  
The Body ◽  
Supreme Courts ◽  
Military Justice ◽  
State Administration ◽  
The Supreme Court ◽  
Directorate General ◽  
The Government ◽  
Government Rules ◽  
The Right ◽  
Tax Court

The  authority  of justice in Indonesia  is executed by  the Supreme Courts and  the  justice  boards/body under the Supreme Courts, including  the general  justice, religious affairs justice, military justice,  state administration  justice,  and  the Constitution Court. According to  certainty in  the Act of  Tax Court, Article1, clause  (5),  tax  dispute   refers to the legal dispute arising in the  taxation  affairs between the  tax payer or the  body  responsible for the  tax with   the government   executives  ( Directorate General of Tax) as the consequence of   the issue of  the decree for the  appeal  to the Tax  Court in accordance with the  tax Act, including the  charge  against the  execution of collection   in accordance with the  Act of Tax Collection by force. The  formation of Tax Court is  designed by  the Executives, in this case, the  Department of Finance, specifically  the Directorate   General  of Tax  which has the right to issue  law  more technical about  tax accord to Article 14,  letter A,  President Decree  no. 44  year 1974,  concerning the  basic  organization of the Department.  Based on  it,  it  is clear that  in addition to execute the government  rules and policy,  this body  has to execute judicial   rules and policy. This is against the  principles of  Judicative  Power/Authority in Indonesia,  which   clearly states that this body  should be under the Supreme Court.   Therefore. It is suggested that   the Act  No UU no.14 Year 2012 concerning  Tax Court   be revised  in accordance with the system of  Power Division  of Justice  as  stated in 45 Constitutions.

Ayurveda dietary guidelines during Covid-19 Pandemic

2020 ◽  
Vol 11 (SPL1) ◽  
pp. 1477-1481
Author(s):  
Ishwari Gaikwad ◽  
Priyanka Shelotkar
Keyword(s):  
Dietary Habits ◽  
Relevant Literature ◽  
Dietary Guidelines ◽  
The Body ◽  
Daily Regimen ◽  
Web Page ◽  
Healthy State ◽  
Health Disorders ◽  
The Individual ◽  
The Right

The current world situation is both frightening and alarming due to the massive disruption caused by the Covid-19 pandemic. The next few days are censorious as we need to be very precautious in our daily regimen as well as dietary habits. Ayurveda offers knowledge about food based on certain reasoning. Indecent food custom is the chief cause for the rising development of health disorders in the current era. In classical texts of Ayurveda, the concept of diet explained well, ranging from their natural sources, properties and specific utility in pathological as well as physiological manner. In this work, the review of the relevant literature of Ahara (Diet) was carried out from Charak Samhita and other texts, newspapers, articles, web page related to the same.  Every human being is unique with respect to his Prakriti (Physical and mental temperament), Agni (Digestive capacity), Koshtha  (Nature of bowel) etc. For that reason, the specificity of the individual should be kept in mind. Ahara, when consumed in the appropriate amount at the right moment following all Niyamas (Guidelines) given in Ayurveda texts, gives immunity and keeps the body in a healthy state during pandemics such as Covid-19. Ultimately, this will help the human body to maintain its strength for life. This article reviews the concept of diet viz. combination of foods, their quantity and quality, methods of preparation and processing, which are to be followed during pandemics and are essential in maintenance and endorsement of health and preclusion of diseases.

Immunity boosting diet during Covid 19

2020 ◽  
Vol 11 (SPL1) ◽  
pp. 832-838
Author(s):  
Roshna Sukheoji Bhutada ◽  
Renu Rathi ◽  
Devyani Dasar
Keyword(s):  
Immune System ◽  
Food Studies ◽  
Egg Yolk ◽  
The Body ◽  
Leafy Vegetables ◽  
Green Leafy Vegetables ◽  
Custard Apple ◽  
The Right ◽  
Daily Physical Activities ◽  
Physiological Reactions

WHO declared Covid 19 /SARS -COV-2 as a global pandemic.Till date, there is no medicine for COVID-19. If the Infection arises in the body then the defence mechanism activated against infection. A recent study suggests that temporarily augmenting the body's immune system in the early stages of COVID-19 can help patient to avoid severe symptoms as it is rightly said prevention is better than cure. Ayurveda approaches to develop physiological reactions to facilitate immunity. Planning of diet is most important to boost immunity.As per many researches to provide supplementary food which contains Zinc, Vitamin C,Vitamin D and immunity boosting foodsuch as citrus natural products, custard apple, apple, papaya is among the Fruits. Vegetables include broccoli, onion, garlic and green leafy vegetables. Nuts, ginger, turmeric, pepper, egg yolk, shellfish, mushroom. The need of the hour is a quick boost to immune system to keep it fit, fighting. One should get the right amount of nutrients from the diet, supplementation regimen to boost immune system.In this review, there are few common supplements and super food studies have been included. It might be a torch bearer as sample menu and their alternatives are given for a normal adult. Needy may change contemplated according to age, sex, body mass index and daily physical activities.

A Case Report on Glanzmann’s Thrombasthenia: A Rare Platelet Function Disorder

2018 ◽  
Vol 2 (02) ◽  
pp. 59-60
Author(s):  
Farida Yasmin ◽  
Md. Anwarul Karim ◽  
Chowdhury Yakub Jamal ◽  
Mamtaz Begum ◽  
Ferdousi Begum
Keyword(s):  
Case Report ◽  
Platelet Function ◽  
The Body ◽  
Presenting Symptoms ◽  
Glanzmann’S Thrombasthenia ◽  
Glanzmann's Thrombasthenia ◽  
Platelet Function Disorders ◽  
Recurrent Epistaxis ◽  
Severe Epistaxis ◽  
History Of

Epistaxis in children is one of the important presenting symptoms for attending emergency department in paediatric patients. Recurrent epistaxis is common in children. Although epistaxis in children usually occurred due to different benign conditions, it may be one of the important presenting symptoms of some inherited bleeding disorder. Whereas most bleeding disorders can be diagnosed through different standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. In this article we describe one case report of platelet function disorders on Glanzmann’s thrombasthenia (GT). Our patient was a 10-year old girl who presented to us with history of recurrent severe epistaxis. She had a bruise on her abdomen and many scattered petechiae in different parts of the body. Her previous investigations revealed no demonstrable haemostatic anomalies. After performing platelet aggregation test, she was diagnosed as GT.

Harnessing clay nano-particles for stem-cell driven tissue regeneration, EPSRC

Impact ◽  
2018 ◽  
Vol 2018 (3) ◽  
pp. 26-28
Author(s):  
Jonathan Dawson ◽  
Richard Oreffo
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Blood Vessels ◽  
The Body ◽  
Biological Molecules ◽  
Nano Particles ◽  
Clay Particles ◽  
Clay Nanoparticles ◽  
Signalling Molecules ◽  
The Right

Gels made from clay could provide an environment able to stimulate stem-cells due to their ability to bind biological molecules. That molecules stick to clay has been known by scientists since the 1960s. Doctors observed that absorption into the blood stream of certain drugs was severely reduced when patients were also receiving clay-based antacid or anti-diarrhoeal treatments. This curious phenomenon was realized to be due to binding of the drugs by clay particles. This interaction is now routinely harnessed in the design of tablets to carefully control the release and action of a drug. Dr Dawson now proposes to use this property of clay to create micro-environments that could stimulate stem cells to regenerate damaged tissues such as bone, cartilage or skin. The rich electrostatic properties of nano (1 millionth of a millimetre) -scale clay particles which mediate these interactions could allow two hurdles facing the development of stem-cell based regenerative therapies to be overcome simultaneously. The first challenge - to deliver and hold stem cells at the right location in the body - is met by the ability of clays to self-organise into gels via the electrostatic interactions of the particles with each other. Cells mixed with a low concentration (less than 4%) of clay particles can be injected into the body and held in the right place by the gel, eliminating, in many situations, the need for surgery. Clay particles can also interact with large structural molecules (polymers) which are frequently used in the development of materials (or 'scaffolds'), designed to host stem cells. These interactions can greatly improve the strength of such structures and could be applied to preserve their stability at the site of injury until regeneration is complete. While several gels and scaffold materials have been designed to deliver and hold stem cells at the site of regeneration, the ability of clay nanoparticles to overcome a second critical hurdle facing stem-cell therapy is what makes them especially exciting. Essential to directing the activity of stem-cells is the carefully controlled provision of key biological signalling molecules. However, the open structures of conventional scaffolds or gels, while essential for the diffusion of nutrients to the cells, means their ability to hold the signalling molecules in the same location as the cells is limited. The ability of clay nano-particles to bind biological molecules presents a unique opportunity to create local environments at a site of injury or disease that can stimulate and control stem-cell driven repair. Dr Dawson's early studies investigated the ability of clay gels to stimulate the growth of new blood vessels by incorporating a key molecular signal that stimulates this process, vascular endothelial growth factor (VEGF). In a manner reminiscent of the observations made in the 60s, Dr Dawson and colleagues observed that adding a drop of clay gel to a solution containing VEGF caused, after a few hours, the disappearance of VEGF from the solution as it became bound to the gel. When placed in an experimental injury model, the gel-bound VEGF stimulated a cluster of new blood vessels to form. These exciting results indicate the potential of clay nanoparticles to create tailor-made micro-environments to foster stem cell regeneration. Dr Dawson is developing this approach as a means of first exploring the biological signals necessary to successfully control stem cell behaviour for regeneration and then, using the same approach, to provide stem cells with these signals to stimulate regeneration in the body. The project will seek to test this approach to regenerate bone lost to cancer or hip replacement failure. If successful the same technology may be applied to harness stem cells for the treatment of a whole host of different scenarios, from burn victims to those suffering with diabetes or Parkinson's.

scholarly journals Governing Nonconformity: Gender Presentation, Public Space, and the City in New Order Indonesia

2021 ◽  
pp. 1-20
Author(s):  
Benjamin Hegarty
Keyword(s):  
Public Space ◽  
Gender Nonconformity ◽  
The Body ◽  
New Order ◽  
Sexual Morality ◽  
National Belonging ◽  
Femininity And Masculinity ◽  
Gender Presentation ◽  
The Right ◽  
The City

The regulation of public space is generative of new approaches to gender nonconformity. In 1968 in Jakarta, the capital of Indonesia, a group of people who identified as wadam—a new term made by combining parts of Indonesian words denoting “femininity” and “masculinity”—made a claim to the city's governor that they had the right to appear in public space. This article illustrates the paradoxical achievement of obtaining recognition on terms constituted through public nuisance regulations governing access to and movement through space. The origins and diffuse effects of recognition achieved by those who identified as wadam and, a decade later, waria facilitated the partial recognition of a status that was legal but nonconforming. This possibility emerged out of city-level innovations and historical conceptualizations of the body in Indonesia. Attending to the way that gender nonconformity was folded into existing methods of codifying space at the scale of the city reflects a broader anxiety over who can enter public space and on what basis. Considering a concern for struggles to contend with nonconformity on spatial grounds at the level of the city encourages an alternative perspective on the emergence of gender and sexual morality as a definitive feature of national belonging in Indonesia and elsewhere.

scholarly journals Changes in Children’s Body Composition and Posture during Puberty Growth

Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 288
Author(s):  
Wojciech Rusek ◽  
Joanna Baran ◽  
Justyna Leszczak ◽  
Marzena Adamczyk ◽  
Rafał Baran ◽  
...  
Keyword(s):  
Body Composition ◽  
Adipose Tissue ◽  
Regression Analysis ◽  
Body Mass ◽  
Pelvic Obliquity ◽  
The Body ◽  
Body Posture ◽  
Older Children ◽  
The Difference ◽  
The Right

The main goal of our study was to determine how the age of children, puberty and anthropometric parameters affect the formation of body composition and faulty body posture development in children. The secondary goal was to determine in which body segments abnormalities most often occur and how gender differentiates the occurrence of adverse changes in children’s body posture and body composition during puberty. The study group consisted of 464 schoolchildren aged from 6–16. Body posture was assessed with the Zebris system. The composition of the body mass was tested with Tanita MC 780 MA body mass analyzer and the body height was measured using a portable stadiometer PORTSTAND 210. The participants were further divided due to the age of puberty. Tanner division was adopted. The cut-off age for girls is ≥10 years and for boys it is ≥12 years. The analyses applied descriptive statistics, the Pearson correlation, stepwise regression analysis and the t-test. The accepted level of significance was p < 0.05. The pelvic obliquity was lower in older children (beta = −0.15). We also see that age played a significant role in the difference in the height of the right pelvis (beta = −0.28), and the difference in the height of the right shoulder (beta = 0.23). Regression analysis showed that the content of adipose tissue (FAT%) increased with body mass index (BMI) and decreased with increasing weight, age, and height. Moreover, the FAT% was lower in boys than in girls (beta negative equal to −0.39). It turned out that older children (puberty), had greater asymmetry in the right shoulder blade (p < 0.001) and right shoulder (p = 0.003). On the other hand, younger children (who were still before puberty) had greater anomalies in the left trunk inclination (p = 0.048) as well as in the pelvic obliquity (p = 0.008). Girls in puberty were characterized by greater asymmetry on the right side, including the shoulders (p = 0.001), the scapula (p = 0.001) and the pelvis (p < 0.001). In boys, the problem related only to the asymmetry of the shoulder blades (p < 0.001). Girls were characterized by a greater increase in adipose tissue and boys by muscle tissue. Significant differences also appeared in the body posture of the examined children. Greater asymmetry within scapulas and shoulders were seen in children during puberty. Therefore, a growing child should be closely monitored to protect them from the adverse consequences of poor posture or excessive accumulation of adipose tissue in the body.

Voice In Ear

2021 ◽  
Vol 5 (1) ◽  
pp. 1-25
Author(s):  
Yang Gao ◽  
Yincheng Jin ◽  
Jagmohan Chauhan ◽  
Seokmin Choi ◽  
Jiyang Li ◽  
...  
Keyword(s):  
User Authentication ◽  
Low Cost ◽  
The Body ◽  
Ear Canal ◽  
Replay Attacks ◽  
Increasing Demand ◽  
The Right ◽  
Sound Conduction ◽  
Enrollment Data ◽  
Secure Authentication

With the rapid growth of wearable computing and increasing demand for mobile authentication scenarios, voiceprint-based authentication has become one of the prevalent technologies and has already presented tremendous potentials to the public. However, it is vulnerable to voice spoofing attacks (e.g., replay attacks and synthetic voice attacks). To address this threat, we propose a new biometric authentication approach, named EarPrint, which aims to extend voiceprint and build a hidden and secure user authentication scheme on earphones. EarPrint builds on the speaking-induced body sound transmission from the throat to the ear canal, i.e., different users will have different body sound conduction patterns on both sides of ears. As the first exploratory study, extensive experiments on 23 subjects show the EarPrint is robust against ambient noises and body motions. EarPrint achieves an Equal Error Rate (EER) of 3.64% with 75 seconds enrollment data. We also evaluate the resilience of EarPrint against replay attacks. A major contribution of EarPrint is that it leverages two-level uniqueness, including the body sound conduction from the throat to the ear canal and the body asymmetry between the left and the right ears, taking advantage of earphones' paring form-factor. Compared with other mobile and wearable biometric modalities, EarPrint is a low-cost, accurate, and secure authentication solution for earphone users.

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