literature review of facebook

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A Literature Review of Research on Facebook Use

This paper represents a review of more than one hundred studies on the social networking website Facebook. An initial selection has been made and has led to the identification of eight main research themes: effects on the users, friendship, construction of impressions, privacy, use, Facebook and politics, self-expression and construal, social capital, and the merging of social spheres. This paper focuses on Facebook use, which is currently the most researched theme. How do individuals use Facebook and why? Six different categories are identified: initiating and maintaining relationships, learning about others, recognition, personality and willingness to communicate, social influence, and experience. The major studies for each category are presented, with an emphasis on the most influential ones in the field. The focus of this literary review is on the commonalities and differences that arise from the results. Hypotheses are presented when possible, and a few theoretical explanations are provided. As a result, one could notice that Facebook is mainly used to keep in touch with other people, but not in a conventional way as users tend to ‘spy’ on other users’ profiles. This phenomenon leads to a growing exhibitionism, which is in turn related to individuals’ personality traits. Use of Facebook is also influenced by peers and experience with the website. A few limitations are discussed, and gender is found to be a possible factor influencing Facebook use. Suggestions for further study are provided.

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Social media in higher education: A literature review of Facebook

• Published: 22 June 2017
• Volume 23 , pages 605–616, ( 2018 )

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• Ritesh Chugh 1 &
• Umar Ruhi 2  

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The rapid adoption of social media technologies has resulted in a fundamental shift in the way communication and collaboration take place. As staff and students use social media technologies in their personal lives, it is important to explore how social media technologies are being used as an educational tool. The aim of this paper is to analyse the role of social media, in particular, Facebook, as an educational tool in higher education. Through a review of the literature, this paper explores the myriad ways in which Facebook is being used as an educational instrument for learning and teaching. Multiple benefits of Facebook usage for learning and teaching have been identified such as increased teacher-student and student-student interaction, improved performance, the convenience of learning and higher engagement. The paper also highlights the potential problems and limitations of Facebook usage ranging from educators’ dominance to privacy concerns. Finally, Facebook usage guidelines that can be adopted by educators to encourage social media adoption are proposed. As social media usage continues to grow in higher education, future empirical research is warranted.

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Chugh, R., Ruhi, U. Social media in higher education: A literature review of Facebook. Educ Inf Technol 23 , 605–616 (2018). https://doi.org/10.1007/s10639-017-9621-2

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Received : 22 January 2017

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DOI : https://doi.org/10.1007/s10639-017-9621-2

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• DOI: 10.1007/s10639-017-9621-2
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Social media in higher education: A literature review of Facebook

• Ritesh Chugh , U. Ruhi
• Published in Education and Information… 22 June 2017
• Education, Computer Science

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A Systematic Review on the Impact of Facebook Usage on Academic Performance

2020, Reviews of Management Sciences

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Mast cell activation syndrome: An up-to-date review of literature

Öner Özdemir.

Department of Pediatric Allergy and Immunology, Sakarya University, Sakarya, Adapazarı 54100, Türkiye. moc.liamtoh@reno_rimedzo

Gökçe Kasımoğlu

Department of Pediatrics, Sakarya University, Sakarya, Adapazarı 54100, Türkiye

Ayşegül Bak

Hüseyin sütlüoğlu.

Department of Pediatrics, Kocaeli City Hospital, Kocaeli 50123, Türkiye

Süreyya Savaşan

Department of Pediatrics, Children’s Hospital of Michigan, Hematology/Oncology, Central Michigan University College of Medicine, Mount Pleasant, MI 48859, United States

Barbara Ann Karmanos Cancer Institute, Wayne State University, Detroit, MI 12345, United States

Corresponding author: Öner Özdemir, MD, Professor, Department of Pediatric Allergy and Immunology, Sakarya University, Adnan Menderes cad, Sakarya, Adapazarı 54100, Türkiye. moc.liamtoh@reno_rimedzo

Mast cells are a subtype of white blood cells and are involved in the immune system. These cells contain many chemical substances called mediators, which are involved in the allergic response. The fact that mast cells play a role in many events that require urgent intervention, especially anaphylaxis, has led to a more detailed study of these cells. The diseases also caused by dysfunctions of mast cells have been examined in many circumstances. For instance, mast cell activation syndrome is known as an augmented number of cells due to decreased cell death, resulting in clinical symptoms affecting many systems. The main common symptoms include flushing, hypotension, urticaria, angioedema, headache, vomiting and diarrhea. Although the underlying mechanism is not yet clearly known, we aim to review the literature in a broad perspective and bring together the existing knowledge in the light of the literature due to the diversity of its involvement in the body and the fact that it is a little known syndrome.

Core Tip: Mast cell activation syndrome is a rare disease that is mostly diagnosed based on clinical symptoms. It is a disorder that should be considered with specific signs and symptoms of mast cell activation in individuals with skin, gastrointestinal, cardiovascular, respiratory, and neurological system involvement.

INTRODUCTION

Mast cells have normal physiologic functions such as homeostasis, tissue repair, angiogenesis, and their role in the innate/acquired immune system[ 1 ]. Mast cell activation and liberation of mediators are needed for the maintenance of normal physiological processes[ 2 ]. However, there are also abnormal conditions, in which this process is not regulated and, like mast cell activation syndrome (MCAS), causes manifestations in different organ systems in the body. Therefore, MCAS is a disorder that should be considered and diagnosed clinically with specific signs and symptoms of activation in individuals with skin, gastrointestinal, cardiovascular, respiratory, and neurological system involvement[ 1 , 3 , 4 ].

MCAS is a rare disease that is mostly diagnosed based on clinical symptoms. Here, we summarize recent developments about this disease, which is rapidly increasing in the frequency of diagnosis today, in the light of current literature data. We also review the epidemiology, pathogenesis, and diagnosis of MCAS in a broader context and to examine the signs and symptoms that occur due to its clinical relationship with other systems.

HISTORICAL BACKGROUND

Mast cells were first described in 1863 as granular cells observed in a study using frog mesenteries and in 1877, following this examination, named the as yet unidentified cells ‘mastzellen’. A few years later, the relationship of mast cells with a pathological condition was first understood when they were found scattered in this area while examining the lesions of a case with urticaria pigmentosa (UP). The certainty that mast cells were associated with any disease only emerged in 1949 discovered the existence of mast cells in many organs in the autopsy of a one-year-old child who passed away from cachexia[ 5 ]. This was the first report of systemic mastocytosis (SM). In 1988, Travis et al [ 6 ] designed a classification scheme for mastocytosis and this was accepted by the scientific world as the first scheme by the National Institutes of Health of United States in 1991.

In 2010, a conference was organized to elaborate on this topic to modernize the classification and diagnostic criteria for mast cell disorders, especially MCAS. Mastocytosis was already well known, but MCAS also required to be described and some criteria established. The classification into primary, secondary, and idiopathic MCAS was first proposed at this conference for diagnostic purposes[ 1 ]. Two years later, Valent et al [ 7 ] published an update of the criteria based on learning and observation of the disease. In 2016, the World Health Organization published a second updated classification of mastocytosis[ 7 ].

EPIDEMIOLOGY

Epidemiologic studies are not yet sufficient to verify the incidence and prevalence of both mastocytosis and MCAS. The incidence of mastocytosis is currently thought to be 1/10000[ 8 , 9 ]. It is equally distributed by sex and can occur at any age. Epidemiologic studies on MCAS have not yet been conducted sufficiently and the frequency of these syndromes is much more hard to guess[ 10 ]. The incidence and prevalence of cases with monoclonal MCAS (MMCAS) and idiopathic MCAS are not well known and there is insufficient data in the literature[ 11 , 12 ].

PATHOGENESIS AND MECHANISMS INVOLVED

An increase in cell number due to decreased apoptosis, an abnormal activation in response to microenvironmental triggers, rather than neoplasia of mast cells have a role in the development and classification of the syndrome. The disease can be categorized into three main groups: Primary, secondary, and idiopathic[ 13 ].

In MCAS, the pathological behavior of mast cells is not due to abnormal mast cell proliferation but to chronic abnormal constitutive and reactive activation[ 5 ]. This abnormal activation may occur as a result of a change in the activation threshold, abnormal expression of receptors and mediators inducing an allergic immune response, changes in the tissue environment affecting the expression and function of mediators, or mutations in the regulatory genes of the cells[ 14 , 15 ]. Although the genetic basis is not fully understood, mutations and alternative variants have been detected in the c-KIT receptor (CD117+), which is responsible for the proliferation of mast cells in tissue via stem cell factor[ 12 ].

The best-defined and most focused physiopathologic role of mast cells is the allergic reaction caused by an abnormal response of mast cells to harmless antigens. Mastocytosis and MMCAS are both described as having clonal mutations in mast cells[ 16 , 17 ]. Although the pathogenic mechanisms of mastocytosis are comparatively well characterized, the mechanism of MMCAS has not been explained. Moreover, no mutations have been found in idiopathic MCAS and not enough is known about its pathogenesis[ 18 , 19 ].

PARAMETERS USED AS MCAS DIAGNOSTIC CRITERIA

The MCAS criteria were first recognized in 2012 and remain to be developed by an international consensus group. Three criteria are considered, all of which must be met for a diagnosis of MCAS to be made[ 20 ].

In the case of clinically episodic, recurrent, severe (mostly like anaphylaxis), and systemic (including at least two organ systems) typical MCAS symptoms; the criteria used in the diagnosis are following: (1) Serum tryptase level, one of the markers of MCAS in the laboratory, exceeding 120% + 2 ng/mL above the serum baseline value of the individual; and (2) clinical reaction to anti-mediator drugs that inactivate mast cell mediators or preclude their release, and finally iii) primary (clonal) and/or secondary diseases of mast cell activation ruled out[ 4 ].

The standard laboratory diagnostic marker for MCAS is serum tryptase, with a normal serum level defined to be between 0 and 11.4 ng/mL in adults. Studies have shown that blood samples should be taken within 1 to 4 h of the beginning of symptoms and that basal levels should be evaluated in advance during a symptom-free period of at least 24-48 h after complete recovery. However, some studies have suggested that normal tryptase levels do not diagnostically rule out MCAS[ 1 , 6 , 21 ].

In addition, other mediators such as histamine, prostaglandin D2, chromogranin-A, leukotriene E4, and urinary metabolites of histamine and 11-beta-prostaglandin are not well known about the increased levels required for the diagnosis of MCAS. These mediators are also thought to be less specific for the diagnosis of MCAS[ 6 ].

Biologic agents used therapeutically, such as antihistamines, leukotriene modifiers, mast cell stabilizers, cyclooxygenase inhibitors, or omalizumab, have been identified as supporting the diagnosis as a recognized reaction to drugs that act specifically on MCAS[ 19 , 22 ].

MCAS CLASSIFICATION

Clonal mast cell disorders are considered primary MCAS if they are due to the existence of c-KIT mutations (generally involving the D816V mutation or expression of CD25, CD2, or CD30 on mast cells). If mast cell activation is owing to an allergic or other hypersensitivity disorder, then it is considered secondary MCAS (non-clonal)[ 12 , 23 ]. In addition, if there is no clonality and no other specific reason can be recognized, then MCAS is accepted as idiopathic (Figure ​ (Figure1). 1 ). Combined types of MCAS have also been defined in which cases have both primary and secondary MCAS features and are categorized as mixed MCAS[ 24 ].

Classification of mast cell activation syndrome. MCAS: Mast cell activation syndrome; SM: Systemic mastocytosis; CM: Cutaneous mastocytosis.

CLINICAL SYMPTOMS AND SIGNS IN DIFFERENT ORGANS AND SYSTEMS IN MCAS

This syndrome involves different systems and organs in the body, resulting in different clinical symptoms and signs (Figure ​ (Figure2 2 )[ 3 ]. Common symptoms typically include symptoms suggestive of allergies such as flushing that may increase, decrease, and travel throughout the body, fatigue, cognitive dysfunction, irritation of the eyes, nose, mouth, and throat, lymph node inflammation, nausea, reflux, headache, dyspnea, palpitations, abdominal pain, diarrhea/constipation, anxiety, and mood disorders. In addition, dermatographism, fibromyalgia-type pain, joint hypermobility, benign growth anomalies, interstitial cystitis, menorrhagia, dysmenorrhea, vulvovaginitis, sensory neuropathy, dysautonomia, and various metabolic endocrinologic abnormalities can be seen affecting many organs and systems. Here, different system involvement will be discussed below one by one.

Clinical manifestations in different systems in patients with mast cell activation syndrome.

Cardiovascular system involvement

Common symptoms of MCAS include hypotension, tachycardia, syncope or near syncope, blood pressure changes, shock, and chest pain. In addition, postural orthostatic tachycardia syndrome (POTS) has some similar clinical symptoms as in MCAS, although the etiology is not fully known, suggesting that there may be a relationship between them and they may have a common pathogenesis[ 25 ]. Although the pathogenesis is not yet fully known, several pathophysiologic processes have been suggested to be involved in this syndrome. The first likely pathophysiologic mechanism is incomplete sympathetic neuropathy[ 26 ]. Some cases have been observed to have incomplete sympathetic denervation and irregular epinephrine reactions in the lower extremities[ 27 ]. As a result, inadequate vasoconstriction is thought to increase heart rate with sympathetic activation in response to congestion in the legs. Clinically, orthostatic intolerance symptoms should be present in patients with POTS, which mostly affects women of reproductive age[ 25 ].

POTS is characterized by symptoms of palpitations, chest pain, heart discomfort, headache, blurred vision, and dizziness. POTS, which has been described by many researchers with different names until today, is now characterized as 'orthostatic intolerance syndrome'. The pathognomonic feature of orthostatic intolerance is the manifestations of symptoms while upright and relief when in supine positions[ 25 ].

The first study targeting to investigate a relationship between MCAS and POTS was conducted in by Shibao et al [ 26 ]. Since flushing is a symptom in both POTS and MCAS, it is thought that MCAS may contribute to the pathogenesis of POTS. Since studies on the relationship between these two are very limited, more data are needed to better characterize the mechanisms. As a result, it is believed that more research should be done on patients who meet the criteria for both diseases.

Dermatologic involvement

Cases with MCAS often manifest flushing caused by the vasodilating effects of histamine and other mediators[ 28 ]. Flushing may be caused by exercise, alcohol, temperature, and emotional changes. Angioedema may occur especially on the lips and tongue. The flushing seen in mast cell disorders is episodic, lasts longer, and it is usually not accompanied by sweating[ 29 ]. Among other clinical findings, nonspecific urticaria, pruritus, etc. may occur[ 30 ].

Respiratory system involvement

Upper and lower respiratory tract symptoms are common in patients with MCAS. These symptoms include nasal congestion, nasal itching, shortness of breath, wheezing, bronchoconstriction, bronchospastic cough, throat swelling, and rhinorrhea[ 1 , 23 ]. Angioedema of the upper respiratory tract may also be seen, but it is rare[ 4 ].

Gastrointestinal tract involvement

General effects of MCAS on the gastrointestinal tract include nausea, vomiting, wandering abdominal pain, abdominal tenderness, gastroesophageal reflux, dysphagia, atypical chest pain, diarrhea, constipation, esophagitis, intestinal cramps, bloating, malabsorption, mouth sores, gastroparesis, and angioedema[ 31 ].

Irritable bowel syndrome (IBS) may be observed in some patients in association with MCAS. IBS is a common gastrointestinal disorder affecting the quality of life in a large quantity of the population. It is characterized by abdominal pain, alternating constipation, and diarrhea. It has been noted that impaired intestinal barrier function in IBS is caused by mast cell activation due to stress response. One of these stress responses is certain components in the food ingested, such as plant-derived substances. These components regulate mast cell activation[ 32 ]. Mast cell numbers have been shown to increase in the terminal ileum, jejunum, and colon of patients with IBS. At the same time, biopsies performed on atopic IBS patients showed a higher proportion of mast cells compared to non-atopic patients[ 33 ]. Mast cell numbers were found to be directly proportional to the severity of symptoms in IBS and it is thought that mast cell mediators may cause symptoms. The rise in the count of mast cells in the colon is one of the most consistent changes observed in IBS[ 34 ]. In addition, the severity of pain observed in patients with IBS is associated with the number of mast cells located close to enteric nerves. The released mediators activate enteric nerves[ 32 , 35 ].

Small intestinal bacterial overgrowth (SIBO) is common in MCAS. SIBO is a condition in which colon bacteria overgrow in the small intestine. It occurs as a result of anatomical abnormalities as well as motility, and metabolic, systemic, and immune system disorders. In this condition, intestinal symptoms include nausea, anorexia, and bloating due to malabsorption and impaired small intestinal motility. These clinical symptoms are probably caused by invasive strains. Studies have shown the presence of 3 main organisms, E. coli , Klebsiella , and Aeromonas species . The diagnosis of SIBO is made when the bacterial count exceeds 10³ organisms /mL in a patient with clinical symptoms[ 36 ]. The relationship between SIBO and MCAS is as follows; SIBO causes activation of mast cells and increase in T lymphocytes. T lymphocytes in turn secrete microparticles that again activate mast cells. Activated mast cells and T lymphocytes release cytokines that increase intestinal permeability[ 37 ]. This leads to a vicious cycle in which intestinal permeability is constantly impaired and inflammation is constantly increased[ 36 , 38 ].

In mice, abdominal surgeries such as colorectal surgery as well as laparoscopic procedures are known to increase the release of mast cell mediators in peritoneal fluid, extracellular matrix thickness and the risk of intra-abdominal adhesions. This suggests that it may increase the likelihood of intra-abdominal adhesions such as postoperative ileus in humans[ 38 ].

Bone-joint system involvement

Ehler-Danlos syndrome (EDS) is frequently associated with MCAS. Patients are increasingly being admitted to hospitals with EDS and MCAS. EDS is primarily presented by skin hyperextensibility, joint hypermobility, and tissue fragility. It is caused by a group of genetic disorders. Extensive gastrointestinal tract involvement also occurs in this syndrome. Esophagitis, gastroesophageal reflux, abdominal pain, and IBS are common gastrointestinal symptoms[ 35 ]. Patients with EDS have been reported to have increased gastrointestinal symptoms seen in MCAS. It is also thought that mast cell activation may be the cause of other symptoms seen in patients with EDS[ 18 ]. It is recommended that patients with EDS should also be screened for MCAS during evaluation. In addition, it is thought that IL-17A, which is highly produced as a result of mast cell activation disorder in MCAS, causes focal bone loss[ 27 ]. Therefore, it has been reported that osteoporosis may occur at an early age in patients with MCAS and EDS[ 26 ].

Allergic symptoms and disorders

Anaphylaxis is a severe systemic hypersensitivity reaction and increased levels of mast cell mediators such as tryptase and histamine have been detected during attacks[ 39 ]. Mast cells seem to be the primary cells that trigger anaphylaxis in humans[ 40 ]. In addition, activation of basophils results in histamine and mediators such as LTC-4 may be secreted and indirectly contribute to the development of symptoms. To date, no specific biomarker has been identified to follow suspected patients, classify the severity of reactions, or manage the disease. The underlying mechanisms leading to idiopathic anaphylaxis are not fully understood, but several theories have been proposed to explain the pathogenesis[ 41 ]. Researchers have observed that peripheral blood from patients with idiopathic anaphylaxis has higher mast cell counts in culture compared to healthy controls[ 42 ].

In another study, the relationship between MCAS and anaphylaxis was examined through some molecules via the ERK 1/2 pathway. Extracellular signal-regulating kinases (ERK 1/2) contribute to allergic responses by regulating degranulation, eicosanoid creation, and cytokine expression by mast cells, but the mechanisms emphasizing their positive effects on FcεRI-dependent signaling have not been fully elucidated. It has recently been demonstrated that mast cell activation and anaphylaxis are negatively regulated by AMP activated protein kinase (AMPK a type of serine-threonine kinase that is a central regulator of energetic metabolism). However, little was known about the association between ERK1/2-mediated positive and AMPK-mediated negative regulation of FcεRI signaling in mast cells. In one study, ERK1/2 activated FcεRI signaling in mast cells by abolishing the AMPK-dependent negative regulatory axis[ 43 ].

In conclusion, the possible relationship between anaphylaxis and MCAS has been examined through many theories and studies, but more clinical trials are needed to say anything definitive[ 29 ].

Neuropsychiatric symptoms and disorders

Headache, fatigue, weakness, lethargy, lack of attention, feelings of exhaustion, lethargy, and mild impairment of cognitive activity are common symptoms in cases with primary and secondary mast cell activation ( i.e. with mastocytosis and also with allergic disorders). These complaints might be owing to the psychosomatic effects of mast cell-mediated mediators, drugs, or (in adults) having a chronic disease[ 44 ]. Subjective neuropsychiatric symptoms alone should not be taken into consideration for mast cell disorders. Fairly, individual complaints must be along with signs and complaints affecting other organ systems before mast cell disorders can be thought of.

According to various studies and investigations, mast cells are considered to make a significant contribution to the pathophysiology of migraine[ 45 ]. Mast cells are found in the meninges and are thought to be included in the pathophysiology of migraine through events such as sequential neuropeptide release and vasodilation leading to mast cell degranulation[ 46 , 47 ]. Mast cells discharge hundreds of various mediators such as histamine, tryptase, and leukotrienes, and degranulation of meningeal mast cells contributes to the activation of the trigeminal vascular afferent pathway[ 48 ]. This is assumed to be one of the underlying mechanisms of migraine and pain. A recent study has examined the link between the parasympathetic nervous system, mast cells, and migraine, with research suggesting that endogenous acetylcholine activates meningeal mast cells and thus contributes to migraine pathophysiology[ 49 ]. However, although the data obtained are enlightening, further studies are required to explain the complex interaction between the autonomic nervous system, mast cells, and connective tissues of the meninges, cerebral vasculature, and other structures significant in the pathophysiology of migraine headaches[ 50 ].

MASTOCYTOSIS-MCAS RELATIONSHIP

Mastocytosis is divided into two categories: Systemic (SM) and cutaneous mastocytosis (CM). In SM, abnormally proliferating mast cells affect the skin, bone marrow, and other organs, causing various symptoms. Common symptoms include itching, abdominal cramps, and tachycardia[ 16 ]. CM is more common in young children and usually resolves spontaneously in puberty. In CM, mast cells gather in the skin but not in other organs. However, systemic symptoms may still be observed. This is because mediators secreted from activated mast cells accumulated in the skin are released into the circulation[ 29 ].

The symptoms of SM and MCAS are similar. Both conditions have symptoms of mast cell activation, such as facial flushing, abdominal cramps, and hypotension due to degranulation. Mastocytosis is mast cell proliferation with infiltration of dermis (CM) or other tissues and organs (SM). MCAS is increased and inappropriate activation of mast cells without clonal proliferation. The differences between these two conditions are as follows: (1) Patients with SM have an elevated baseline serum tryptase level, typically >20 ng/mL. In cases with MCAS, baseline serum tryptase levels are normal or slightly elevated; (2) multifocal mast cell aggregates are observed. It is characteristic to observe these aggregates in the bone marrow of cases with SM, but not in MCAS; and (3) it is characteristic to usually observe UP called maculopapular CM (MPCM) in patients with CM. However, UP/MPCM-like lesions are not observed in MCAS[ 17 ].

THE RELATIONSHIP BETWEEN PEOPLE WITH LONG-TERM CORONAVIRUS DISEASE 2019 AND MCAS

Long-term coronavirus disease 2019 (COVID-19) is an outcome of immune dysregulation. T and B cell deficiency, hyperactivity of innate immune cells, and an increase in proinflammatory cytokines are observed[ 7 ]. This dysfunction leads to a constant inflammatory reaction, pathogen reactivation, endothelial damage, host-microbiome dysfunction, and autoimmunity. Risk factors for the development of long-term COVID-19 include female gender, type 2 diabetes, autoimmune diseases, connective tissue, and allergic disorders[ 51 , 52 ].

People with long-term COVID-19 disease have major cardiac, neuropsychiatric, and pulmonary complaints. Multi-system disorders such as myocardial inflammation, POTS, dystonia, and myalgic encephalomyelitis/chronic fatigue syndrome can develop. Immune system effects include recurrent infection, autoimmunity, urticaria, allergic rhinitis, and asthma. MCAS is thought to be the possible mechanism underlying these effects[ 26 , 53 ].

Mast cells are the key producers of the inflammatory cytokines of COVID-19. A persistent inflammatory state with prolonged COVID-19 causes abnormal mast cell activation. One of the reasons for this is the maturation of mast cells in the pulmonary perivascular space[ 54 ]. Another cause is the discharge of substance P from immune cells due to severe acute respiratory syndrome coronavirus 2 infection. The secretion of this substance increases the stimulation of the G-protein X2 receptor and predisposes to MCAS formation[ 55 , 56 ].

It is thought that symptomatic improvement will be achieved with the treatment of MCAS in long-term COVID-19 cases. It is thought that stabilization of mast cells and reduction in related symptoms will be achieved by histamine blockade[ 57 , 58 ].

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of MCAS includes numerous diseases and nonspecific signs and symptoms: Infectious diseases, gastrointestinal (IBS, gastrinoma, VIPoma, eosinophilic gastroenteritis or esophagitis, inflammatory bowel disease, food intoxication, etc. ), cardiovascular (endocarditis or endomyocarditis, pulmonary embolism, aortic stenosis with syncope, myocardial infarction), endocrinological (medullary thyroid carcinoma, pheochromocytoma, carcinoid), neuropsychiatric disorders (anxiety/panic attacks, vasovagal syncope), urticaria and angioedema types, drug-induced itching/rash, vasculitis, and atopic dermatitis[ 59 ]. In addition, the differential diagnosis should include two disorders in which there is chronic systemic elevation of mast cell pre-performed mediators in granules and depends on the function of the granules without over activation of mast cells, namely histamine intolerance and hereditary alpha tryptasemia.

A thorough physical examination, together with a thorough history and laboratory evaluation of specific biomarkers, can help differentiate these disorders from MCAS[ 60 ].

TREATMENT PLANS OF MCAS

First, it is of paramount importance to explain to patients and their parents to elude any agents or interactions that could trigger anaphylactic or allergic reactions (Figure ​ (Figure3 3 )[ 18 ]. MCAS cases should also be counseled to take prophylactic anti-mediator therapy ( e.g. histamine receptor blockers) throughout their lives and to carry at least two self-administered epinephrine autoinjectors after being instructed on how to utilize these injectors in case of illness[ 41 ].

The mechanism of mast cell activation and release of mast cell mediators.

In cases with reaction-inducing (IgE-dependent) anaphylaxis and thus secondary MCAS, immunotherapy is generally suggested because its potential side effects are better known. The frequency of life-threatening MCAS events can be significantly reduced after inactivation of neoplastic mast cells. Aspirin has formerly been suggested as a potential therapeutic tool for the therapy of anaphylaxis in patients with SM[ 61 ], but it has been observed that the doses of aspirin required to prevent MCAS must be high and are not tolerated in many cases. Other medications contain mast cell regulatory agents and corticosteroids[ 7 , 62 ].

In addition, there is also an emerging class of drugs targeting mast cells, namely broad-acting tyrosine kinase inhibitors e.g. midostaurin or avapritinib. Some of these medications, such as midostaurin, not only inhibit mast cell proliferation but also block IgE-dependent allergic stimulation of mast cells. Hence, midostaurin might be an encouraging treatment to subdue the effects of MCAS, especially in cases with primary MCAS[ 19 , 63 ].

Another proposed interpretation of MCAS treatment is the use of specific IgE ( e.g. , omalizumab) in secondary MCAS cases with underlying IgE-dependent allergy. Furthermore, in cases with mixed MCAS (primary + secondary MCAS), multiple specific treatment modalities may be necessary after an individualized medicine approach[ 18 ]. For instance, cases with high levels of neoplastic mast cells, advanced SM, and severe IgE-dependent allergy may require a drug directed against the c-KIT mutation or combined treatment with cladribine and omalizumab to control MCAS events[ 18 , 62 ].

Although the mast cell is an obligatory cell for life, issues related to its activation may also be associated with disorders such as MCAS that may present with a wide variety of clinical findings and complaints. Awareness of this disorder should be increased so that MCAS can be easily differentiated from other diseases and treatment plans should be well known.

Conflict-of-interest statement: The authors whose names are listed above, certify that they have no affiliations with or involvement in any organization or entity with any financial interest, or non-financial interest in the subject matter or materials discussed in this article.

Provenance and peer review: Invited article; Externally peer reviewed.

Peer-review model: Single blind

Specialty type: Pediatrics

Country of origin: Türkiye

Peer-review report’s classification

Scientific Quality: Grade A

Novelty: Grade A

Creativity or Innovation: Grade A

Scientific Significance: Grade B

P-Reviewer: Poerwosusanta H, Indonesia S-Editor: Qu XL L-Editor: A P-Editor: Cai YX

Contributor Information

Öner Özdemir, Department of Pediatric Allergy and Immunology, Sakarya University, Sakarya, Adapazarı 54100, Türkiye. moc.liamtoh@reno_rimedzo .

Gökçe Kasımoğlu, Department of Pediatrics, Sakarya University, Sakarya, Adapazarı 54100, Türkiye.

Ayşegül Bak, Department of Pediatrics, Sakarya University, Sakarya, Adapazarı 54100, Türkiye.

Hüseyin Sütlüoğlu, Department of Pediatrics, Kocaeli City Hospital, Kocaeli 50123, Türkiye.

Süreyya Savaşan, Department of Pediatrics, Children’s Hospital of Michigan, Hematology/Oncology, Central Michigan University College of Medicine, Mount Pleasant, MI 48859, United States. Barbara Ann Karmanos Cancer Institute, Wayne State University, Detroit, MI 12345, United States.

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Histiocytosis and adult-onset orbital xanthogranuloma in 2023: a review of the literature and mini case series

Affiliations.

• 1 School of Medicine, University of Zagreb, Šalata 3B, Zagreb, Croatia. [email protected].
• 2 Department of Ophthalmology, University Hospital Center Zagreb, Kišpatićeva 12, Zagreb, Croatia. [email protected].
• 3 School of Medicine, University of Zagreb, Šalata 3B, Zagreb, Croatia.
• 4 Department of Ophthalmology, University Hospital Center Zagreb, Kišpatićeva 12, Zagreb, Croatia.
• 5 Department of Pathology, University Hospital Center Zagreb, Kišpatićeva 12, Zagreb, Croatia.
• 6 Division of Clinical Immunology and Rheumatology, Referral Centre for Systemic Lupus Erythematosus and Related Disorders, Department of Internal Medicine, University Hospital Centre Zagreb, Kišpatićeva 12, Zagreb, Croatia.
• PMID: 38951425
• DOI: 10.1007/s10792-024-03181-y

Purpose: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities.

Methods: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital. Clinical files of 4 patients with adult-onset xanthogranulomatous disease of the orbit and ocular adnexa (AOXGD) were reviewed retrospectively. Clinical, laboratory, radiological, histopathological, and immunohistochemical findings were reexamined.

Results: Reviewing medical records of our patients with AOXGD, we found significant overlap between histiocytosis and different immune disorders. A broad workup should be considered in these patients as they can harbour severe immune disfunctions and hematologic disorders. Preferred treatment modality depends on a histopathologic type of AOXGD, clinical presentation and systemic involvement and should be conducted multidisciplinary.

Conclusion: The diagnosis is often delayed because of its rarity and diverse clinical findings. Development of molecular genetic tests, detection of BRAF V600E mutation and different types of kinase mutations, mutations in transcriptional regulatory genes as well as tyrosine kinase receptors have shed a new light on the etiopathogenesis and potential targeted treatment of histiocytosis.

Keywords: Adult-onset asthma with periocular xanthogranuloma; Adult-onset orbital xanthogranuloma; Cutaneous and mucocutaneous histiocytosis; Histiocytosis; IgG4-related disease; Xanthogranuloma.

© 2024. The Author(s), under exclusive licence to Springer Nature B.V.

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• Asselin BL (2007) Histiocytosis syndromes. In: Kaczorowski JM, Christy C (eds) Pediatric clinical advisor, 2 edn. Garfunkel LC, Mosby: Philadelphia, pp 274–276. ISBN 978-0-323-03506–4
• Emile J-F, Cohen-Aubart F, Collin M, Fraitag S, Idbaih A, Abdel-Wahab O, Rollins BJ, Donadieu J, Haroche J (2021) Histiocytosis. Lancet 398:157–170. https://doi.org/10.1016/S0140-6736(21)00311-1 - DOI - PubMed - PMC
• Histiocytosis Syndromes in Children (1987) Writing group of the histiocyte society. Lancet Lond Engl 1:208–209
• Emile J-F, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel-Wahab O, Allen CE et al (2016) Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 127:2672–2681. https://doi.org/10.1182/blood-2016-01-690636 - DOI - PubMed - PMC
• Sivak-Callcott JA, Rootman J, Rasmussen SL, Nugent RA, White VA, Paridaens D, Currie Z, Rose G, Clark B, McNab AA et al (2006) Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review. Br J Ophthalmol 90:602–608. https://doi.org/10.1136/bjo.2005.085894 - DOI - PubMed - PMC

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Since 1980, the creative writing program at Linfield University and the Associated Students of Linfield University (ASLU) have supported the publication of a student-run literary magazine. First known as Test-Marketed Downpour, the name was changed to Camas: A Journal of Art & Literature in 2001. Open only to current Linfield students for many years, Camas now accepts submissions from the wider campus community – including alumni, faculty, staff and administration – residents of Yamhill County and members of the Confederated Tribes of the Grand Ronde.

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This project focuses on developing a measurement instrument to assess the perceived value of the tourism experience at destinations where there is wind power infrastructure visible in nature. The research involved developing a survey based on literature review, a qualitative study, and insights from sustainable consumer behaviour research. Notably, earlier research established that wind power infrastructure does not deter tourists to visit a destination. In the literature, four factors that influence tourist reactions to wind turbines are identified: visual impact, destination characteristics, tourist’s origin and familiarity, and value judgments. The findings of this project reveal that tourists value judgments on sustainability transitions and sustainable consumption influence their perceptions of wind power infrastructure in nature areas. This is shown in both qualitative and quantitative data.

The qualitative research included interviews at four Swedish destinations (i.e., the High Coast destination, Ånge municipality, Dalarna County and Kalmar County), and the findings suggest that tourists generally do not view wind turbines negatively, appreciating them for their contribution to sustainable development and energy independence. Preferences include having few turbines spaced apart and people seem to rely on information gained through social interaction to make judgments on their impact. Furthermore, our study of relevant hashtags on Instagram shows a positive depiction of wind turbines. The qualitative results were used to develop the conceptual framework behind the survey.

The survey was developed to measure pro-environmental and responsible tourism behavior. A short film was included in the survey to enhance familiarity with a destination with wind turbines in its nature. Opinions on wind power are analysed to understand respondents’ preferences. The survey also gauges the perceived value of tourism experience in nature areas, incorporating emotional, epistemic, eudemonic, and sustainable destination dimensions. The survey was tested with a panel of Swedish participants with 300 final responses. Results reveal significant relationships between various factors, affirming the survey’s validity. Major themes from qualitative analysis persist in quantitative data, emphasizing habituation, sustainability associations, concerns about negative impacts, and the influence of social context on opinions on wind power and its infrastructure in nature areas. Positive opinions on wind power shape attitudes, anticipated emotions, and intentions to visit nature areas at destinations with wind power infrastructure. The survey results show that participants who engage more in outdoor activities tend to have positive opinions on wind power. Those with favourable views of wind power attribute higher value to emotional, epistemic, spiritual and sustainability factors. Results align with the emerging concept of energy tourism, indicating interest in visiting wind power facilities, particularly among those with positive opinions on wind power. The conclusion chapter offers a discussion of the results and of the implications of adapting the survey tool in the real-life context of destinations where tourists might encounter wind power infrastructure in nature areas.

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Bundle Adjustment of Maxar WorldView and GeoEye Imagery...

Since the 1950s, bundle adjustment has been used to correct the geopositioning of imagery. With the increased availability of very high resolution satellite imagery, bundle adjustment has become an increasingly critical topic for both researchers and practitioners. Many studies have explored the bundle adjustment of satellite imagery from Maxar sensors, including the WorldView and GeoEye sensors. In this work, we review some of the recent and relevant literature pertaining to this topic.

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Book review: Pulling traditional legends into the modern world, ‘Chickaloonies 2′ is a giant leap forward

A page from from "Chickaloonies 2: Watering Ways" from Dimi Macheras and Casey Silver

“Chickaloonies 2: Watering Ways”

Dimi Macheras and Casey Silver; 80% Studios, 2024; 268 pages; $39.99

“You locked away your stories,” Drasco, a bear warrior who is a central character in “Chickaloonies 2: Watering Ways,” tells the mayor and citizens of a post-apocalyptic Palmer, Alaska. “You locked away your language, you locked away your culture. You’ve severed the source of your strength.”

Pointing at the exhibits in a shuttered Ahtna cultural center, Drasco continues, “These items are not meant to be locked away. They are alive!”

It’s the pivotal scene in a story that hinges on the recovery of culture as a means of overcoming challenges. In this action-packed fantasy story set in a Palmer that has been laid to waste by giant flying creatures called “moose-quitoes,” the challenge is getting to the source where the plague of monstrous insects is hatching from, and finding a way of stopping them. This will require looking finding guidance in the accumulated lore of the Ahtna.

“Chickaloonies,” an Alaska Native-rooted graphic novel series, is the brainchild of Dimi Macheras and Casey Silver. Macheras grew up in Chickaloon, enthralled by the traditional stories his late grandmother, revered elder Katherine Wade, and his late mother Patricia Wade — both Ahtna culture bearers — told him in his childhood. Growing up, he aided his mother in her work by illustrating the legends she preserved. Silver, originally from Rhode Island, is a talented artist and storyteller himself. Macheras and Silver share a comics partnership called 80% Studios, and “Chickaloonies” is their flagship work.

A page from from "Chickaloonies 2: Watering Ways" from Dimi Macheras and Casey Silver.

The first volume in the series, “First Frost,” was published in 2021 and introduced the primary characters. Sasquatch E. Moji is a 13-year-old behemoth of a child who speaks solely in emojis. Mister Yelly, a year younger and considerably smaller, is a bit of a scamp who depends on his older pal in a pinch but isn’t lacking in bravery himself.

The opening tale, which was fairly brief, took place in Chickaloon Village at a time when light had gone from the sky. It ended with Moji and Yelly, counseled by their grandmother, embarking on a quest atop a berry-powered snowmachine named Boba to find their missing cousin Vally. It was a brief and fun little fantasy, well-conceived with expressive and colorful manga-inspired art, but it merely set the stage.

“Watering Ways” is a giant leap forward for Macheras and Silver. It’s significantly longer and far more ambitious than its predecessor. The story opens with Moji and Yelly crossing a snow-covered landscape under a darkened sky as they come across ice creatures standing rigidly in a valley with human forms contained within them. Frightened by the sight, they hurriedly make for Palmer, where they find the city all but shut down. Quickly they learn of the moose-quitoes — giant, flying moose-mosquito hybrids spreading an unknown sickness among the residents — and are drawn into the mystery of where the creatures come from and how to combat them.

After encountering a storyteller named Baca, they visit Drasco for help finding their cousin. Initially reluctant, though not for long, Drasco instead sends them on their way and they return to the besieged Palmer. There these four and other key players assemble, and the story takes form as Moji and Yelly join in the quest to defeat the moose-quitoes.

“Watering Ways” is a standalone episode within the broader story arc of the series. It’s a fast-paced adventure that weaves Ahtna legends into an action-packed quest to save Palmer from the insect infestation that has overrun the town. It’s a tale filled with excitement, humor, entertaining characters, conflicts, camaraderie and a welcoming spirit that will appeal not just to the young readers who will make up its primary audience, but also to adults looking for a good yarn to escape into.

Macheras and Silver share both writing and artistic duties, which leads to a strong fusion of imagery and words. Often, and especially toward the end, the artwork alone tells the story. As artists, the duo use manga as their launching point, but their style reaches well beyond the form. Their use of deep colors creates a mood perfectly suited for the events occurring on the page. Echoes of the seminal 1960s and 1970s work of famed Marvel and DC artist Jack Kirby also surface subtly in a few panels, tastefully so and not as mimicry, connecting the duo’s work to the long, storied history of American comics.

“Chickaloonies” is a lot of fun, and it’s instructive along the way. Macheras and Silver draw from Ahtna lore to create an Alaska Native epic of their own. Stories passed through generations surface throughout the two books, offering key insight at critical points as the plot line unfolds. This knowledge is central to the narrative, and by including it, Macheras carries forward the work of his mother and grandmother, updating and adding to a tradition of oral literature and making it vibrant for modern readers.

The duo are working as part of a collective of Alaska comics producers operating under the name Luk’ae Tse’ Taas Comics (Fish Head Soup Comics) that is focused on bringing both Alaska themes and Native cultures to the world of graphic fiction. As a work geared toward younger readers, the “Chickaloonies” series is the most accessible of those being produced and provides an easy entry point for readers just getting acquainted with the shared universe that the creators are contributing to. It’s a new direction for Alaska arts and literature, and as this book makes clear, its potential is only beginning to be explored.

[ With the Luk’ae Tse’ Taas Comics collective, Alaska visual artists help build a broader universe ]

“When you embark on the journey of rediscovering the knowledge of your culture, listen for the voices of the ancestors,” Macheras and Silver write in a brief afterword to the book. “And remember the watering ways, as a beacon to help you find your way back home.” It’s the perfect coda for “Chickaloonies 2,” which pulls traditional legends into the modern world and crafts something at once both deeply rooted and intriguingly new.

David James

David A. James is a Fairbanks-based freelance writer, and editor of the Alaska literary collection “Writing on the Edge.” He can be reached at [email protected].