Stone Man Research Articles

Stone Man Syndrome: A Rare and Debilitating Genetic Disorder with a Misguided Immune Response

Stone Man Syndrome, also known as Fibrodysplasia Ossificans Progressiva(FOP), is a rare genetic disorder characterized by progressive bone formation and connective tissue ossification. The condition affects approximately 1 in 2 million people worldwide and typically becomes apparent during early childhood. Individuals with Stone Man Syndrome experience the formation of extra bone in their muscles, tendons, and ligaments, which eventually leads to joint immobility and skeletal abnormalities. The condition progresses gradually and often leads to the fusion of the spinal column and rib cage, resulting in respiratory problems and limited mobility. FOP is caused by a mutation in the ACVR1 gene, which encodes a protein involved in bone formation and repair. The mutation leads to the activation of a specific signalling pathway, causing the body to produce excess bone tissue in response to injury or trauma. Currently, there is no cure for Stone Man Syndrome, and treatment options are limited. Physicians can only manage the symptoms of the condition and help patients maintain mobility through physical therapy, pain management, and surgery. However, surgery can be risky due to the risk of triggering new bone formation. The diagnosis of Stone Man Syndrome is typically based on clinical evaluation and genetic testing. Early diagnosis is critical to developing effective treatment plans and providing support for patients and their families. In conclusion, Stone Man Syndrome is a rare genetic disorder characterized by the progressive formation of extra bone tissue, leading to joint immobility and skeletal abnormalities. Although there is currently no cure for the condition, early diagnosis and management of symptoms can improve patients’ quality of life.

Knowledge and awareness about fibrodysplasia ossificans progressiva among dental students.

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder which is autosomal dominant distinguished by congenital malformations of large toes and flare ups, etc. It is a disorder of connective tissue, with heterotopic ossifications seen with skeletal muscles, tendons, and cartilages and also called as Stone man disease, myositis ossificans, and Munchmeyer disease. The main objective of the study is to assess and create the awareness about FOP among dental students. An online-based survey was conducted among 103 dental students, undergraduates and postgraduates. About 20 questionnaires were prepared and circulated among the students through the "Google forms" across Chennai. A survey questionnaire asked about the knowledge of fibrodysplasia, its causes, treatment, and diagnosis was enquired. Thus, the data obtained were analyzed statistically using the SPSS software. In our study, about 64% were undergraduates and 35% of them were postgraduates. About 66% of the participants were aware of fibrodysplasia and 32% of them were not aware. Most of the undergraduate students were more aware of fibrodysplasia (40%) when compared to postgraduates (29%). However, this is statistically not significant. Fibrodysplasia ossificans reported to have a high incidence in 1 in 2 million people worldwide. Hence, an early diagnosis of this disorder can prevent further complications. Although the survey has provided significant knowledge about fibrodysplasia, awareness still has to be created among dental students.

FENOMENA PENGEMIS SILVER MAN PADA USIA PRODUKTIF (Studi Kasus Di Perempatan Demangan, kab. Purworejo)

This study aims to find out the phenomenon of the shift in the art of stone man to Silver Man beggar due to termination of employment due to the Covid 19 Pandemic at one of the Traffic Lights in Purworejo Regency. This study was designed to use a qualitative descriptive approach with the type of phenomenology. The sampling technique used purposive and snowball sampling. While the data collection techniques were carried out by in-depth interviews, passive observation and documentation studies. The selected sample was determined by 3 respondents and met directly at the traffic lights on the Cangkrep - Bagelen and Demangan Crossroads, Banyuurip with the criteria of productive age youth who became Silver Man beggars. Based on the results of the study, 70% of the productive age youth of the respondents chose to become silver man due to the difficulty of finding a job, being a silver man beggar allows them to share with other jobs and 30% for the reason of helping the family economy. Keywords: Silver Man Beggar, Beggar in Productive Age

Stone, Labor, and the Building of Israel

Andrew Ross's Stone Men: The Palestinians Who Built Israel is a significant contribution to an emerging literature on Palestinian labor. Through an examination of various facets of the stone industry in both Israel and the West Bank, Ross develops a series of insights into the nature of settler colonialism, patterns of urban development, the political economy of Palestinian class formation, borders and migration, and the ecological impacts of occupation. By highlighting the ways in which Palestinians actually built Israel, Ross's book carries important implications for how we think about Palestinian political strategy and the debates around one- or two-state solutions.

Review: Justice for Some: law and the question of Palestine by Noura Erakat, Stone Men: the Palestinians who built Israel by Andrew Ross

Review: <i>Justice for Some: law and the question of Palestine</i> by Noura Erakat, <i>Stone Men: the Palestinians who built Israel</i> by Andrew Ross

Fibrodysplasie ossifiante progressive et hétéroplasie ossifiante progressive

Fibrodysplasia ossificans progressiva (FOP), or Münchmeyer's disease, is a rare, autosomal dominant genetic disorder, characterized by episodic progressive and cumulative heterotopic ossification, leading to a disabling metamorphosis into a “stone man”, with irremediable joint immobility, leaving families and health professionals often distraught, faced to the immeasurable progression of the disease. The involvement of a recurrent mutation of the activin-like kinase gene, ACVR1/ALK2 gene, an activating mutation of the (TGF)-β/BMP pathway, was demonstrated in 2006 by F. Kaplan, E. Shore and an international consortium. This major discovery has launched many research projects for understanding the pathophysiological basis and targeting specific therapies approaches. The present management of this pathology includes mainly preventive measures, and symptomatic treatment of disease flare-ups preceding the phenomenon of ossification, by various anti-inflammatory agents. Several innovative therapies are currently undergoing preclinical and clinical development, aimed at blocking the upstream and downstream signaling pathways of ACVR1/ALK2, marking a new era for the community of affected individuals and people engaged with FOP patients. Progressive osseous heteroplasia (POH) also belongs to the group of genetic disorders with extensive heterotopic ossification, characterized by the abnormal formation of bone within the connective tissues. It is an ultra-rare pathology (less than 100 cases described to date), severely progressive. This great rarity seems to be related to incomplete penetrance with wide variability of manifestations. The onset of ossification is episodic, as in FOP. On the other hand, these two conditions may be distinguished by the facts that POH is due to heterotopic ossification mainly of intramembranous origin, that there is no triggering factor leading to bone formation, there is no progression according to a characteristic pattern and by the absence of concomitant inflammatory factor. There is a direct differentiation of mesenchymal progenitors into osteoblasts. POH is associated with inactivating mutations of the GNAS gene. Treatments are currently limited to symptomatic measures, including ensuring cutaneous asepsis, adequate analgesia and non-steroidal anti-inflammatory drugs.

A BRIEF OVERVIEW OF THE VERY RARE FIBRODYSPLASIA OSSIFICANS PROGRESSIVA (STONE MAN SYNDROME)

Fibrodysplasia ossificans progressiva (FOP) is an extremely rare disorder with only about 800 million people affected worldwide. It occurs as a result of the mutation in Activin A receptor type 1 (ACVR1) gene leading to extraskeletal structure formation which further gradually progresses to skeletal deformities and premature death. This disorder is also known as stone man syndrome as it causes joints to become frozen resulting in mobility impairment. It is generally identified in early childhood and affects almost every organ. Though there is no approved treatment for this rare disorder, researchers are in progress. Supportive therapy and care can help the patients to some extent by providing temporary relief. In this review, we will briefly outline the information regarding FOP which is difficult to diagnose and whose misdiagnosis is dangerous.

Kashkul: Contemporary Art, Cultural Preservation, Research, and Translation in Kurdistan

I n the Kurdish language the word kashkul describes a Sufi’s traditional begging bowl. Over time, the term evolved to also describe a commonplace book, a receptacle for intellectual and spiritual wealth. In choosing Kashkul as its name, the research and arts collaborative at the American University of Iraq Sulaimani has taken a further metaphorical leap, pioneering over ten researcher- or artist-initiated projects that fulfill its mission to “study, create, and preserve culture in Iraq.” This spring, our collaborative opened the first sculpture garden in the region, as part of The Stone Man, a retrospective featuring over forty years of work by the Kurdish painter and Kashkul artistin -residence Ismail Khayat, who became well known for painting boulders within the no-man’s-land that stretched between the warring factions of the Kurdish civil war. As part of his Kashkul residency, he sought to transform another landscape, to explore the potentiality of public space, and to demonstrate how disparate elements can be brought together. Khayat is the first of many who will mentor emerging student-artists in Sulaimani through Kashkul’s artist-in-residence program. The student-artists who worked as Ismail’s apprentices in the sculpture garden on the campus of Kashkul’s home institution, the American University of Iraq Sulaimani, came from all over the country, as did the stones they painted. The garden is an outdoor art gallery, open to students at all times, a space that suggests the many possibilities students have to transform spaces with culture. Other Kashkul projects include Crux, which uses oral history and literary translation to study how devotion becomes violent and how violent devotion can become peaceful, and Mosul Lives, which collects extensive interviews that, in aggregate, give a picture of daily life in Mosul, before the Islamic State, before the American presence of 2003. The collaborative’s translation work is exhaustive, and its project Arrival selects, translates, analyzes, and compares literature from every century of Kurdish poetry. Kashkul’s projects do not just include our own investigations; we also collect and archive materials that will be available for others to study. Through a partnership with the UCLA Library’s International Digital Ephemera Project, much of our collection is available online. Kashkul runs on collaboration. Like the kashkuls that Kurdish scholars have made across the centuries, Kashkul believes in the individual, giving each of us the freedom and support to pursue projects of our own design. As the primary investigators of our own research projects at Kashkul, Pshtewan Kamal Babakir and I share a sense of urgency about reading, preserving, and translating Kurdish literature. We also strive to work in disciplines that are not well established in the Kurdish regions of Iraq. Through our work at Kashkul, we create a different future for ourselves and for our country. Our parents faced Saddam Hussein and the genocide he brought down on Kurds. Our parents took part in the revolution that resulted in the semiautonomous Kurdish region. The Kurdish intellectuals we study fought their own revolutions, investing in Kurdish linguistics , shaping a Kurdish alphabet. We have our own revolution before us now: to understand our culture for ourselves and then share it with the world. Editorial note: Turn to page 45 to read Pshtewan Kamal and Shene Mohammed’s bios. The portfolio of Kurdish poetry translations begins on page 42. PHOTO: ALANA MARIE LEVINSON LABROSSE Outpost Kashkul Contemporary Art, Cultural Preservation, Research, and Translation in Kurdistan by Pshtewan Kamal & Shene Mohammed 96 WLT JULY–AUGUST 2018 ...

Kashkul: Contemporary Art, Cultural Preservation, Research, and Translation in Kurdistan

I n the Kurdish language the word kashkul describes a Sufi’s traditional begging bowl. Over time, the term evolved to also describe a commonplace book, a receptacle for intellectual and spiritual wealth. In choosing Kashkul as its name, the research and arts collaborative at the American University of Iraq Sulaimani has taken a further metaphorical leap, pioneering over ten researcher- or artist-initiated projects that fulfill its mission to “study, create, and preserve culture in Iraq.” This spring, our collaborative opened the first sculpture garden in the region, as part of The Stone Man, a retrospective featuring over forty years of work by the Kurdish painter and Kashkul artistin -residence Ismail Khayat, who became well known for painting boulders within the no-man’s-land that stretched between the warring factions of the Kurdish civil war. As part of his Kashkul residency, he sought to transform another landscape, to explore the potentiality of public space, and to demonstrate how disparate elements can be brought together. Khayat is the first of many who will mentor emerging student-artists in Sulaimani through Kashkul’s artist-in-residence program. The student-artists who worked as Ismail’s apprentices in the sculpture garden on the campus of Kashkul’s home institution, the American University of Iraq Sulaimani, came from all over the country, as did the stones they painted. The garden is an outdoor art gallery, open to students at all times, a space that suggests the many possibilities students have to transform spaces with culture. Other Kashkul projects include Crux, which uses oral history and literary translation to study how devotion becomes violent and how violent devotion can become peaceful, and Mosul Lives, which collects extensive interviews that, in aggregate, give a picture of daily life in Mosul, before the Islamic State, before the American presence of 2003. The collaborative’s translation work is exhaustive, and its project Arrival selects, translates, analyzes, and compares literature from every century of Kurdish poetry. Kashkul’s projects do not just include our own investigations; we also collect and archive materials that will be available for others to study. Through a partnership with the UCLA Library’s International Digital Ephemera Project, much of our collection is available online. Kashkul runs on collaboration. Like the kashkuls that Kurdish scholars have made across the centuries, Kashkul believes in the individual, giving each of us the freedom and support to pursue projects of our own design. As the primary investigators of our own research projects at Kashkul, Pshtewan Kamal Babakir and I share a sense of urgency about reading, preserving, and translating Kurdish literature. We also strive to work in disciplines that are not well established in the Kurdish regions of Iraq. Through our work at Kashkul, we create a different future for ourselves and for our country. Our parents faced Saddam Hussein and the genocide he brought down on Kurds. Our parents took part in the revolution that resulted in the semiautonomous Kurdish region. The Kurdish intellectuals we study fought their own revolutions, investing in Kurdish linguistics , shaping a Kurdish alphabet. We have our own revolution before us now: to understand our culture for ourselves and then share it with the world. Editorial note: Turn to page 45 to read Pshtewan Kamal and Shene Mohammed’s bios. The portfolio of Kurdish poetry translations begins on page 42. PHOTO: ALANA MARIE LEVINSON LABROSSE Outpost Kashkul Contemporary Art, Cultural Preservation, Research, and Translation in Kurdistan by Pshtewan Kamal & Shene Mohammed 96 WLT JULY–AUGUST 2018 ...

Anaesthetic management of a child with stone man syndrome: Look before you leap!

Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway.

Stone Man Preacher: J. Walter Malone: The Autobiography of an Evangelical Quaker with Essays on Emma Brown Malone by John W. Oliver, Jr., and Emma Lapsansky

Stone Man Preacher: J. Walter Malone: The Autobiography of an Evangelical Quaker with Essays on Emma Brown Malone by John W. Oliver, Jr., and Emma Lapsansky

Munchmeyer's Disease-A case report

Fibrodysplasia ossificans progressive (FOP) is a rare autosomal dominant disease. It is also known as Myositis ossificans progressiva, Stone man disease or Munchmeyer's disease. Herein a rare case of Fibrodysplasia ossificans progressive is presented.

Poster 206 The Impact of Functional Rehabilitation on Quality of Life in a Patient with Fibrodysplasia Ossificans Progressiva (”Stone Man Syndrome“): A Case Report

PM&RVolume 6, Issue 9S p. S257-S258 Friday Poster Presentations - Poster Hall Poster 206 The Impact of Functional Rehabilitation on Quality of Life in a Patient with Fibrodysplasia Ossificans Progressiva (”Stone Man Syndrome“): A Case Report Deep Garg MD, Deep Garg MD University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this authorUsman F. Ahmad DO, Usman F. Ahmad DO University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this authorSeema R. Khurana DO, Seema R. Khurana DO University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this author Deep Garg MD, Deep Garg MD University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this authorUsman F. Ahmad DO, Usman F. Ahmad DO University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this authorSeema R. Khurana DO, Seema R. Khurana DO University of Miami Miller School of Medicine, Miami, FL, United StatesSearch for more papers by this author First published: 29 September 2014 https://doi.org/10.1016/j.pmrj.2014.08.598Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume6, Issue9SSeptember 2014Pages S257-S258 RelatedInformation

E31 Outcomes of ureteroscopy for stone disease in pregnancy: Results from a systematic review of the literature

Introduction: Our aim was to evaluate the clinical efficacy and safety of ureteroscopy as a primary treatment for preg- nant women with symptomatic ureteric stones who have failed conservative management. Materials and Methods: A systematic review of the literature from January 1990 to June 2011 was performed, including all English language ar- ticles. Outcome measures were clinical efficacy, in terms of stone clearance and need for additional procedures, and safety in terms of complications. Results: A total of 239 ab- stracts were screened and 15 studies were identified report- ing on 116 procedures. The surgical methods of stone man- agement employed were stone extraction with basket only (n = 55, 47%), laser fragmentation (n = 27, 23%; holmium, n = 20, pulse dye, n = 7), impact lithotripsy (n = 21, 18%), uretero- scopic lithotripsy (n = 6, 5%) and a combination of methods (n = 6, 5%). A post-operative stent was inserted in 64 of 116 procedures (55%). Complete stone clearance was seen in 100 of the 116 procedures (86%). There were 2 major complica- tions (1 ureteral perforation and 1 case of premature uterine contraction) and 7 minor complications (5 urinary tract infec- tions and 2 cases of post-operative pain). Conclusion: This review suggests that stone clearance using ureteroscopy is a relatively safe option in pregnancy with a high success rate.

Ultrasound-Guided Ankle Block in Stone Man Disease, Fibrodysplasia Ossificans Progressiva

In this case report, we describe the successful use of ultrasound-guided regional anesthesia in progressive fibrodysplasia ossificans (stone man disease), a condition commonly regarded as a contraindication for regional anesthesia. A patient with advanced fibrodysplasia ossificans progressiva presented with osteomyelitis of a foot and was scheduled for resection of the infected bones and soft tissue. Ultrasound imaging allowed us to identify the obscured anatomic landmarks for ankle block anesthesia and to restrict the injection of local anesthetics to the epifascial tissue and subcutaneous compartment. With this ankle block, the patient uneventfully underwent surgery without need for additional sedative or analgesic drugs.

Stone Men of Malekula on Malakula: An Ethnography of an Ethnography

This article examines the resonances of the voluminous ethnography, ‘Stone Men of Malekula’ (SMM) in contemporary Vanuatu. Anthropological research is politically charged in Vanuatu, in part because of how the weighty materiality of archival forms exercise significant local authority. However, alongside respect for this ‘evidential’ material is a healthy scepticism of anthropological authority. SMM, written by the maverick anthropologist John Layard in 1942 (based on fieldwork in 1914–15), has returned to Vanuatu in many guises over the years. It is used as formal evidence in land disputes and as a bone of contention within competing claims. Tracing the ways in which culture is written, read and materialized, exposes the paradoxes of knowledge and politics not only within anthropological critique but in Vanuatu villages.

Түрэг угсаатны хүн чулуу хөшөөний урлаг (Монгол Улсын нутаг дэвсгэрт байгаа хөшөөний жишээн дээр)

English title: Culture of Turkish stone man (In case of the Mongolia)

Characteristic Appearance on Bone Scintigraphy of a ???Stone Man???

Fibrodysplasia ossificans progressiva (FOP), also known as stone man is an extremely rare autosomal dominant disorder. Its incidence is reported to be approximately 1 in 2 million people. The disease is characterized by progressive replacement of fascia, tendons, muscles, and ligaments by bone, leading to encasement of the skeleton within a new extra skeleton. It usually appears in the first decade of life, more commonly in males. In the early stages, it may be associated with tender swellings in the muscles. There is no definitive therapy or effective prevention for this condition. We present a case of FOP with its characteristic bone scan appearances. Bone scan helps in the diagnosis of this rare disease, thus protecting the patient from unnecessary biopsies and confusion regarding its diagnosis.

Anesthesia in ‘stone man’: Myositis ossif icans progressiva

Myositis ossificans progressive is a rare disease leading to complete, ossification of the muscular .system. Very little information about this rare disease and its anesthetic implications has appeared in anesthetic literature. This disease is felt to have an autosomal dominant pattern of inheritance and is usually associated with anomalies o f the hands and feet. A f f licted patients are frequently misdiagnosed in childhood as having a rheumatologic disorder. Later in life (as true bone is formed in striated muscle, ligaments, and fascia), the correct diagnosis becomes obvious. Although muscles of the heart, diaphragm, larynx, and sphincters are spared, those of the chest wall are not, and pulmonary function progressively deteriorates. Death frequently occurs as a result of a pulmonary infection. Specific anesthetic considerations include positioning to avoid injury, potential need for fiber-optic intubation or tracheostomy, decreased thoracic compliance with the need for increased ventilating pressures, and the ultimate in disuse atrophy contraindicating the use of succinylcholine. Myositis ossificans progressive can present the anesthesiologist with interesting challenges. Anesthetic management will need to be individualized according to the severity of the disease.

Prehistoric Europe: From Stone Man to the Early Greeks.

Prehistoric Europe: From Stone Man to the Early Greeks.