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Stenotrophomonas maltophilia is a gram-negative bacterium that can be routinely identified in the aquatic environment, and has become an emergent, multi-drug-resistant life-threatening organism in some circumstances. This bacterium was identified as a presumptive causative agent in cases of polysystemic granulomatous disease in one diamondback terrapin (Malaclemys terrapin) and one mata mata (Chelus fimbriata). Both chelonians presented with lethargy, hyporexia, and multifocal subcutaneous masses diagnosed by physical examination and in one individual on computed tomography (CT). The primary hematologic finding was moderate to severe heterophilic leukocytosis, and both turtles were treated with a combination of supportive care, systemic antibiotics, and/or surgical resection of subcutaneous masses. Medical and surgical management were unsuccessful, and one patient was euthanized and the other found deceased. Necropsies revealed multisystemic granulomas, and cultures identified growth of Stenotrophomonas maltophilia. A postmortem investigation into possible risk factors identified comorbidities including shell disease, repeated administration of injectable antibiotics, low environmental temperature, and aquatic pH changes as possible contributing factors. This bacterium appears to be an emerging organism of concern and should be considered as a potential cause of granulomas in aquatic chelonians. [ABSTRACT FROM AUTHOR]

Shaan Lalvani, Rebecca M Brown Department of Neurology, The Mount Sinai Hospital, New York, NY, USACorrespondence: Rebecca M Brown, MD, PhD, The Mount Sinai Hospital, Department of Neurology, 1216 5th Avenue, New York, NY, 10029, USA, Tel +1 2128248579, Email Rebecca.Brown@mssm.eduAbstract: Neurofibromatosis Type I (NF1) is a complex genetic condition that affects multiple organ systems and presents a unique set of challenges for clinicians in its management. NF1 is a tumor predisposition syndrome that primarily affect the peripheral and central nervous systems via the impact of haploinsufficiency upon neural crest lineage cells including Schwann cells, melanocytes, fibroblasts, etc. NF1 can further lead to pathology of the skin, bones, visual system, and cardiovascular system, all of which can drastically reduce a patient’s quality of life (QOL). This review provides a comprehensive examination of the many specialties required for the care of patients with Neurofibromatosis Type 1 (NF1). We delve into the pathogenesis and clinical presentation of NF1, highlighting its diverse manifestations and the challenges they pose in management. The review underscores the importance of a multidisciplinary approach to NF1, emphasizing how such an approach can significantly improve patient outcomes and overall QOL. Central to this approach is the role of the NF expert, who guides a multidisciplinary team (MDT) comprising healthcare professionals from many areas of expertise. The MDT collaboratively addresses the multifaceted needs of NF1 patients, ensuring comprehensive and personalized care. This review highlights the need for further investigation to optimize the workflow for NF1 patients in an MDT setting, and to improve implementation and efficacy.Keywords: multisystemic, multidisciplinary team, quality of life, neurofibromatosis, NF expert, NF1, tumor predisposition syndrome

Introduction: Chest pain in post-COVID-19 patients can be due to serious alarming post-COVID-19 sequelae, such as Coronary Artery Disease (CAD), pulmonary embolism, myocarditis, etc. Approximately one-fifth of patients attending clinical Outpatient Department (OPD) following COVID-19 recovery present with persistent chest pain. There is limited knowledge about the clinical profile of patients experiencing persistent post-COVID-19 chest pain. Aim: To describe the clinical profile of such patients to fill the knowledge gap and acquire new insights into patients with post-COVID-19 persistent chest pain. Materials and Methods: A cross-sectional study was conducted, enrolling a total of 259 patients with persistent chest pain (i.e., chest pain lasting more than 24 weeks after COVID-19 diagnosis). After detailed history-taking and clinical and laboratory examinations, observed data were collected, compiled, evaluated, and analysed to achieve the study objectives. Results: Out of 259 patients, 133 (51.4%) had cardiac {85 (32.8%)} or pulmonary {48 (18.5%)} abnormalities. Among patients with cardiac abnormalities, CAD, arrhythmia, myocarditis/cardiomyopathy, pericarditis, and PAH were detected in 38 (14.6%), 18 (6.9%), 7 (2.7%), 11 (4.2%), and 11 (4.2%), respectively. Meanwhile, among patients with pulmonary abnormalities, 36 (13.9%) patients had organic residual lesions in the lung parenchyma, 7 (2.7%) had pleuritis, and 28 (10.8%) had pulmonary function abnormalities. The remaining 126 (48.6%) patients experienced chest pain due to non cardiopulmonary aetiologies like gastrointestinal {45 (17.3%)}, musculoskeletal {38 (14.6%)}, psychomotor {35 (13.5%)}, autonomic {8 (3.3%)}, etc. Conclusion: This study found that chest pain in post-COVID-19 patients arises due to multisystemic aetiologies such as cardiac, pulmonary, visceral, autonomic, psychomotor, musculoskeletal, etc. A wide spectrum of serious cardiac abnormalities (such as CAD, arrhythmia, myocarditis, pericarditis, PAH, etc.) contributes to about 1/3rd of cases of persistent chest pain in post-COVID-19 patients.

Introduction: Chest pain in post-COVID-19 patients can be due to serious alarming post-COVID-19 sequelae, such as Coronary Artery Disease (CAD), pulmonary embolism, myocarditis, etc. Approximately one-fifth of patients attending clinical Outpatient Department (OPD) following COVID-19 recovery present with persistent chest pain. There is limited knowledge about the clinical profile of patients experiencing persistent post-COVID-19 chest pain. Aim: To describe the clinical profile of such patients to fill the knowledge gap and acquire new insights into patients with post-COVID-19 persistent chest pain. Materials and Methods: A cross-sectional study was conducted, enrolling a total of 259 patients with persistent chest pain (i.e., chest pain lasting more than 24 weeks after COVID-19 diagnosis). After detailed history-taking and clinical and laboratory examinations, observed data were collected, compiled, evaluated, and analysed to achieve the study objectives. Results: Out of 259 patients, 133 (51.4%) had cardiac {85 (32.8%)} or pulmonary {48 (18.5%)} abnormalities. Among patients with cardiac abnormalities, CAD, arrhythmia, myocarditis/cardiomyopathy, pericarditis, and PAH were detected in 38 (14.6%), 18 (6.9%), 7 (2.7%), 11 (4.2%), and 11 (4.2%), respectively. Meanwhile, among patients with pulmonary abnormalities, 36 (13.9%) patients had organic residual lesions in the lung parenchyma, 7 (2.7%) had pleuritis, and 28 (10.8%) had pulmonary function abnormalities. The remaining 126 (48.6%) patients experienced chest pain due to non cardiopulmonary aetiologies like gastrointestinal {45 (17.3%)}, musculoskeletal {38 (14.6%)}, psychomotor {35 (13.5%)}, autonomic {8 (3.3%)}, etc. Conclusion: This study found that chest pain in post-COVID-19 patients arises due to multisystemic aetiologies such as cardiac, pulmonary, visceral, autonomic, psychomotor, musculoskeletal, etc. A wide spectrum of serious cardiac abnormalities (such as CAD, arrhythmia, myocarditis, pericarditis, PAH, etc.) contributes to about 1/3rd of cases of persistent chest pain in post-COVID-19 patients. [ABSTRACT FROM AUTHOR]

Attachment resilience is the ability of attachment relationships to survive adversity and maintain their functions in the face of stress and is a critical component of personal and family resilience. This property is the result of interactive influences across multiple systems of care. In this way, security is transferred across what we call chains of security. This paper will delve into how family keyworkers (i.e., professionals who are referential and responsible for family intervention plans) function as a critical link within these chains of security for hard-to-reach families: first, through attachment-like relationships that foster attachment resilience within families that are vulnerable or face adversity, and second, as receptors and transmitters of security by means of attachment-centered supervision. Sensitivity, repair, and mentalization are highlighted as key processes constant across these multisystemic levels. [ABSTRACT FROM AUTHOR]

As our understanding of the process of resilience has become more culturally and contextually grounded, researchers have had to seek innovative ways to account for the complex, reciprocal relationship between the many systems that influence young people's capacity to thrive. This paper briefly traces the history of a more contextualized understanding of resilience and then reviews a social–ecological model to explain multisystemic resilience. A case study is then used to show how a multisystemic understanding of resilience can influence the design and implementation of resilience research. The Resilient Youth in Stressed Environments study is a longitudinal mixed methods investigation of adolescents and emerging adults in communities that depend on oil and gas industries in Canada and South Africa. These communities routinely experience stress at individual, family, and institutional levels from macroeconomic factors related to boom-and-bust economic cycles. Building on the project's methods and findings, we discuss how to create better studies of resilience which are able to capture both emic and etic accounts of positive developmental processes in ways that avoid the tendency to homogenize children's experience. Limitations to doing multisystemic resilience research are also highlighted, with special attention to the need for further innovation. [ABSTRACT FROM AUTHOR]

Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.

Aims:Lipoid proteinosis (LP) is a rare genodermatosis with relatively increased prevalence in Turkey. Dermatologists commonly recognize the disease due to the prominent mucocutaneous findings. Neuropsychiatric involvement is common, and the findings are heterogeneous. This study aimed to examine the cranial magnetic resonance imaging (MRI) findings of LP patients along with their neuropsychiatric involvement.Methods:This single-center, retrospective study included patients diagnosed with LP from March 2017 through March 2020. Demographics, neuropsychiatric complaints, and physical examination findings were retrieved from the medical records. The same radiologist evaluated all cranial MRI images to search for the calcifications within the temporal lobe, the mesolimbic region, and particularly the amygdala. The secondary end-points were the presence of additional radiological findings localized to other areas of the brain.Results:Eight patients [age, mean±standard deviation: 30.2±9.0 years (range 23-51), female: 50%] were eligible. Two (25%) patients had clear neuropsychiatric involvement. One patient had mental retardation and history of intractable epilepsy during childhood. Another patient had complaints of severe amnesia and difficulties in concentrating. Five patients (62.5%) showed the typical symmetric bean-shaped or oval calcifications in the uncal part of the temporal lobes involving the amygdala nuclei of hippocampi. In addition to the calcifications, one patient had arachnoid cysts. Cranial MRI findings were normal in the remaining three patients. MRI findings were normal in the patient with mental retardation.Conclusions:In this study, complaints and neuropsychiatric evaluation were inconsistent with the findings on cranial MRI among patients with LP.

Background and objectives: Multisystem involvement in spinal muscular atrophy (SMA) is gaining prominence since different therapeutic options are emerging, making the way for new SMA phenotypes and consequent challenges in clinical care. Defective immune organs have been found in preclinical models of SMA, suggesting an involvement of the immune system in the disease. However, the immune state in SMA patients has not been investigated so far. Here, we aimed to evaluate the innate and adaptive immunity pattern in SMA type 1 to type 3 patients, before and after nusinersen treatment. Methods: Twenty one pediatric SMA type 1, 2, and 3 patients and 12 adult SMA type 2 and 3 patients were included in this single-center retrospective study. A Bio-Plex Pro-Human Cytokine 13-plex Immunoassay was used to measure cytokines in serum and cerebrospinal fluid (CSF) of the study cohort before and after 6 months of therapy with nusinersen. Results: We detected a significant increase in IL-1β, IL-4, IL-6, IL-10, IFN-γ, IL-17A, IL-22, IL-23, IL-31, and IL-33, in serum of pediatric and adult SMA patients at baseline, compared to pediatric reference ranges and to adulthealthy controls. Pediatric patients showed also a significant increase in TNFα and IL-17F levels at baseline. IL-4, IFN-γ, Il-22, IL-23, and IL-33 decreased in serum of pediatric SMA patients after 6 months of therapy when compared to baseline. A significant decrease in IL-4, IL-6, INF-γ, and IL-17A was detected in serum of adult SMA patients after treatment. CSF of both pediatric and adult SMA patients displayed detectable levels of all cytokines with no significant differences after 6 months of treatment with nusinersen. Notably, a higher baseline expression of IL-23 in serum correlated with a worse motor function outcome after treatment in pediatric patients. Moreover, after 6 months of treatment, patients presenting a higher IL-10 concentration in serum showed a better Hammersmith Functional Motor Scale Expanded (HFMSE) score. Discussion: Pediatric and adult SMA patients show an inflammatory signature in serum that is reduced upon SMN2 modulating treatment, and the presence of inflammatory mediators in CSF. Our findings enhance SMA knowledge with potential clinical and therapeutic implications. [ABSTRACT FROM AUTHOR]

Background and objectivesMultisystem involvement in spinal muscular atrophy (SMA) is gaining prominence since different therapeutic options are emerging, making the way for new SMA phenotypes and consequent challenges in clinical care. Defective immune organs have been found in preclinical models of SMA, suggesting an involvement of the immune system in the disease. However, the immune state in SMA patients has not been investigated so far. Here, we aimed to evaluate the innate and adaptive immunity pattern in SMA type 1 to type 3 patients, before and after nusinersen treatment.MethodsTwenty one pediatric SMA type 1, 2, and 3 patients and 12 adult SMA type 2 and 3 patients were included in this single-center retrospective study. A Bio-Plex Pro-Human Cytokine 13-plex Immunoassay was used to measure cytokines in serum and cerebrospinal fluid (CSF) of the study cohort before and after 6 months of therapy with nusinersen.ResultsWe detected a significant increase in IL-1β, IL-4, IL-6, IL-10, IFN-γ, IL-17A, IL-22, IL-23, IL-31, and IL-33, in serum of pediatric and adult SMA patients at baseline, compared to pediatric reference ranges and to adult healthy controls. Pediatric patients showed also a significant increase in TNF-α and IL-17F levels at baseline. IL-4, IFN-γ, Il-22, IL-23, and IL-33 decreased in serum of pediatric SMA patients after 6 months of therapy when compared to baseline. A significant decrease in IL-4, IL-6, INF-γ, and IL-17A was detected in serum of adult SMA patients after treatment. CSF of both pediatric and adult SMA patients displayed detectable levels of all cytokines with no significant differences after 6 months of treatment with nusinersen. Notably, a higher baseline expression of IL-23 in serum correlated with a worse motor function outcome after treatment in pediatric patients. Moreover, after 6 months of treatment, patients presenting a higher IL-10 concentration in serum showed a better Hammersmith Functional Motor Scale Expanded (HFMSE) score.DiscussionPediatric and adult SMA patients show an inflammatory signature in serum that is reduced upon SMN2 modulating treatment, and the presence of inflammatory mediators in CSF. Our findings enhance SMA knowledge with potential clinical and therapeutic implications.

Theoretical work in resilience has continuously evolved to inform and respond to advances in empirical work. In order to further scientific inquiry, it must continue to do so. This narrative overview of the field of resilience science focuses on contemporary challenges confronted by theoretical models of individual resilience and proposes a taxonomic structure for resilience—the multidimensional taxonomy of individual resilience (MTIR). The goal of the MTIR is to articulate a systematic framework within which extant theoretical and empirical work can be nested. Consistent with existing work, the MTIR organizes resilience into two primary branches—manifested resilience and generative resilience. These two components are then organized into subdomains that demonstrate evidence of conceptual distinctiveness. The specification of the subdomains in the MTIR draws support from a diverse body of work on resilience across disciplines and in multiple global contexts. The MTIR makes several critical advances, including expanding and refining the definitions and components of resilience in psychology, providing a clearer framework for conceptualizing mixed profiles of resilience, and tempering assumptions regarding the relational dependencies across domains of resilience. Finally, the utility of the MTIR in organizing research in resilience and advancing theory-testing and development is discussed. [ABSTRACT FROM AUTHOR]

Aims: Lipoid proteinosis (LP) is a rare genodermatosis with relatively increased prevalence in Turkey. Dermatologists commonly recognize the disease due to the prominent mucocutaneous findings. Neuropsychiatric involvement is common, and the findings are heterogeneous. This study aimed to examine the cranial magnetic resonance imaging (MRI) findings of LP patients along with their neuropsychiatric involvement. Methods: This single-center, retrospective study included patients diagnosed with LP from March 2017 through March 2020. Demographics, neuropsychiatric complaints, and physical examination findings were retrieved from the medical records. The same radiologist evaluated all cranial MRI images to search for the calcifications within the temporal lobe, the mesolimbic region, and particularly the amygdala. The secondary end-points were the presence of additional radiological findings localized to other areas of the brain. Results: Eight patients [age, mean±standard deviation: 30.2±9.0 years (range 23-51), female: 50%] were eligible. Two (25%) patients had clear neuropsychiatric involvement. One patient had mental retardation and history of intractable epilepsy during childhood. Another patient had complaints of severe amnesia and difficulties in concentrating. Five patients (62.5%) showed the typical symmetric bean-shaped or oval calcifications in the uncal part of the temporal lobes involving the amygdala nuclei of hippocampi. In addition to the calcifications, one patient had arachnoid cysts. Cranial MRI findings were normal in the remaining three patients. MRI findings were normal in the patient with mental retardation. Conclusions: In this study, complaints and neuropsychiatric evaluation were inconsistent with the findings on cranial MRI among patients with LP. [ABSTRACT FROM AUTHOR]

Alkaptonuria (AKU) is a multisystemic autosomal recessive disease due to deficiency of enzyme homogentisate dioxygenase leading to accumulation of homogentisic acid. Urine becoming dark on oxygenation or alkalinization is the first symptom but is often ignored. The patient usually presents with pigment deposition in connective tissue and cartilage after the third decade. This pigment deposition not only alters the aesthetics but also leads to alteration in the activity of different tissues due to inflammation and subsequent fibrosis or calcification. We are presenting an adult male who was asymptomatic till 5 years back when he started having backache for which he was taking analgesics off and was diagnosed to be having AKU at the age of 58 years.

Teaching point: Tuberculosis (TB) is a rare clinical disease in the musculoskeletal system. Multifocal bone involment and multisystemic spread of this disease is extremely uncommon and difficult to diagnose.

The past decade has seen growing interest in interventions that build resilience as a complementary practice to trauma-informed care. From school-based programs focused on self-regulation and academic success to programs that support the well-being of disadvantaged populations or healthcare workers at risk of burnout, the concept of resilience is being used most commonly for programming that builds the capacity of individuals to adapt under conditions of adversity. Critiques have raised concerns that resilience-promoting programs demonstrate bias toward changing individual-level factors such as cognitions (e.g., mindfulness and grit), behavior (e.g., expressing gratitude and changing personal routines), or attachments (e.g., feeling secure in relationships) which help people adapt to socially toxic situations without changing access to the resources they require to overcome exposure to adverse psychosocial factors. This trend belies advances to the theory of resilience which support a more contextualized, multisystemic understanding of how external protective factors (resources) enhance individual qualities (ruggedness) and vice versa. Building on a multisystemic description of resilience, the R2 Resilience Program© was developed and piloted with six different populations ranging from clients of urban social services to workers in a long-term care facility, managers in the health care sector, staff of a Fortune 500 corporation, students in a primary to grade 12 school, and adult volunteers affiliated with an international NGO. Focused on building both individual ruggedness and enhancing people's resources (the two Rs), the program provides contextualized content for each population by selecting from 52 resilience promoting factors with a strong evidence base to create training curricula that enhance the personal qualities and social, physical, and institutional resources most likely to support resilience. This paper reviews the justification for a multisystemic approach to designing resilience interventions and then explains the process of implementation of the R2 program. Preliminary findings are reported, which suggest the program is experienced as effective, with evaluations ongoing. [ABSTRACT FROM AUTHOR]

The past decade has seen growing interest in interventions that build resilience as a complementary practice to trauma-informed care. From school-based programs focused on self-regulation and academic success to programs that support the well-being of disadvantaged populations or healthcare workers at risk of burnout, the concept of resilience is being used most commonly for programming that builds the capacity of individuals to adapt under conditions of adversity. Critiques have raised concerns that resilience-promoting programs demonstrate bias toward changing individual-level factors such as cognitions (e.g., mindfulness and grit), behavior (e.g., expressing gratitude and changing personal routines), or attachments (e.g., feeling secure in relationships) which help people adapt to socially toxic situations without changing access to the resources they require to overcome exposure to adverse psychosocial factors. This trend belies advances to the theory of resilience which support a more contextualized, multisystemic understanding of how external protective factors (resources) enhance individual qualities (ruggedness) and vice versa. Building on a multisystemic description of resilience, the R2 Resilience Program© was developed and piloted with six different populations ranging from clients of urban social services to workers in a long-term care facility, managers in the health care sector, staff of a Fortune 500 corporation, students in a primary to grade 12 school, and adult volunteers affiliated with an international NGO. Focused on building both individual ruggedness and enhancing people’s resources (the two Rs), the program provides contextualized content for each population by selecting from 52 resilience promoting factors with a strong evidence base to create training curricula that enhance the personal qualities and social, physical, and institutional resources most likely to support resilience. This paper reviews the justification for a multisystemic approach to designing resilience interventions and then explains the process of implementation of the R2 program. Preliminary findings are reported, which suggest the program is experienced as effective, with evaluations ongoing.

Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.

Background: There have been reports of a new hyperinflammatory syndrome in children defined as the Paediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS). Our hospital has experienced a great proportion of children attending an Emergency Department (ED) with possible PIMS-TS so far reported in the UK. Objectives: We describe the clinical and biochemical findings in children with possible PIMS-TS in the context of a local ED. Settings: Queen Elizabeth Hospital (QEH), Woolwich, a District General Hospital (DGH) in South London. Participants: From 14th March to 18th May 2020, children presenting to QEH and transferred to tertiary care for possible PIMS-TS, with a history of fever and hyperinflammatory symptoms, raised inflammatory markers and without a clear clinical or microbial cause were identified. Demographic data, clinical and laboratory data were recorded as median [range]. Results: 17 children (12 male) were identified aged 11 [1-16] years. 17/17 had a fever; other common symptoms were conjunctival injection, rash and gastrointestinal symptoms. Lymphopenia and raised inflammatory markers were evident. 15/17 were tested with nasopharyngeal and oropharyngeal SARS-CoV-2 PCR swabs and 15/15 were negative. Before transfer, one child required intubation and four required inotropes. All children were transferred to a tertiary unit, 10 within the first 24 hours. After transfer, 2/17 had microbial causes evident on urine/stool culture. Conclusions: PIMS-TS is proving challenging to diagnose in a DGH ED because of heterogeneity of symptoms and laboratory markers, overlapping with other diseases, and cardiac complications despite deceptively benign presentations. There is an urgent need to review the approach to a febrile child in this setting, to optimise identification of PIMS-TS. Prognostic markers and risk stratification methods would help paediatricians working in the ED and general paediatric wards.

Cardiomyopathy (CMP) is a rare disease in the pediatric population, with a high risk of morbidity and mortality. The genetic etiology of CMPs in children is extremely heterogenous. These two factors play a major role in the difficulties of establishing standard diagnostic and therapeutic protocols. Isolated CMP in children is a frequent finding, mainly caused by sarcomeric gene variants with a detection rate that can reach up to 50% of analyzed cohorts. Complex multisystemic forms of pediatric CMP are even more heterogenous. Few studies in literature take into consideration this topic as the main core since it represents a rarity (systemic CMP) within a rarity (pediatric population CMP). Identifying etiology in this cohort is essential for understanding prognosis, risk stratification, eligibility to heart transplantation and/or mechanical-assisted procedures, preventing multiorgan complications, and relatives’ recurrence risk calculation. The previous points represent a cornerstone in patients’ empowerment and personalized medical care approach. The aim of this work is to propose a new approach for an algorithm in the setting of the diagnostic framework of systemic pediatric CMP. On the other hand, during the literature review, we noticed a relatively common etiologic pattern in some forms of complex/multisystem CMP. In other words, certain syndromes such as Danon, Vici, Alström, Barth, and Myhre syndrome share a common pathway of directly or indirectly defective “autophagy” process, which appears to be a possible initiating/triggering factor for CMPs. This conjoint aspect could be important for possible prognostic/therapeutic implications in this category of patients. However, multicentric studies detailed functional and experimental models are needed prior to deriving conclusions.

Teaching point: Tuberculosis (TB) is a rare clinical disease in the musculoskeletal system. Multifocal bone involment and multisystemic spread of this disease is extremely uncommon and difficult to diagnose. [ABSTRACT FROM AUTHOR]

A multisystemic model of resilience suggests that the capacity of one system to cope with atypical stress improves the capacity of co-occurring systems. In this paper, we review research demonstrating this relationship, where the more resilient caregivers are, the more likely children are to experience the promotive and protective factors they require for optimal growth and development in both home and school settings. We examine research from the last two decades on school- or family-based resilience promoting interventions, and advocate for a new perspective which adopts a multisystemic view of resilience in order to redirect the focus of the international research agenda, which places emphasis on children rather than systems. The implications of this multisystemic approach to resilience are discussed in relation to the design of programs that promote the well-being of parents and teachers in ways that contribute to more supportive and stable home and school environments for children. [ABSTRACT FROM AUTHOR]

• This is the largest cohort of PCR confirmed, natural Babesia rossi infections published. • Clinical, hematological, biochemical and pathology is described. • Odds ratios confirmed which clinical and biochemical measures associat.e with poor outcome. • ROC curves identified TT4, bilirubin, urea and cortisol are most predictive of death. • Cut-offs for these measures allow clinicians and researchers to objectively classify cases. Babesia rossi causes the most severe clinical disease in dogs of all the babesia parasites. We included 320 naturally-infected dogs that presented for care at the Onderstepoort Veterinary Academic Hospital between 2006 and 2016. All dogs had mono-infections confirmed by multiplex PCR. The data allowed more accurate clinical classification of the disease and identified parameters that were associated with disease severity and death. Odds ratios for dying were significant (P < 0.05) for increased band neutrophil count, collapse at presentation; presence of cerebral signs; hypoglycaemia; hyperlactatemia; high urea, high creatinine; hyperbilirubinaemia; hypercortisolaemia; and hypothyroxinaemia. Joint component analysis confirmed that the variables with significant odds ratios grouped together with death. Yet, multivariate logistic regression was unable to identify a group of significant independent predictors of death. Receiver Operator Characteristic curves indicated that low total thyroid hormone, high bilirubin, high serum urea and high cortisol concentrations were the variables with the highest sensitivity and specificity for death. These data provide both the clinician and researcher with a set of easily-measured laboratory and clinical assessments to classify cases into those that are uncomplicated and those that are complicated. The disease is complex and multisystemic and probably involves mechanisms more proximal in the pathogenesis than those that have been evaluated. [ABSTRACT FROM AUTHOR]

Caprine arthritis encephalitis virus (CAEV) is a lentivirus that infects both goats and sheep and is closely related to maedi-visna virus that infects sheep; collectively, these viruses are known as small ruminant lentiviruses (SRLV). Infection of goats and sheep with SRLV typically results in discrete inflammatory diseases which include arthritis, mastitis, pneumonia or encephalomyelitis. SRLV-infected animals concurrently demonstrating lentivirus-associated lesions in tissues of lung, mammary gland, joint synovium and the central nervous system are either very rare or have not been reported. Here we describe a novel CAEV promoter isolated from a sheep with multisystemic lentivirus-associated inflammatory disease including interstitial pneumonia, mastitis, polyarthritis and leukomyelitis. A single, novel SRLV promoter was cloned and sequenced from five different anatomical locations (brain stem, spinal cord, lung, mammary gland and carpal joint synovium), all of which demonstrated lesions characteristic of lentivirus associated inflammation. This SRLV promoter isolate was found to be closely related to CAEV promoters isolated from goats in northern California and other parts of the world. The promoter was denoted CAEV-ovine-MS (multisystemic disease); the stability of the transcription factor binding sites within the U3 promoter sequence are discussed.

Ehlers–Danlos syndrome (EDS)—hypermobility type (HT) is considered to be the most common subtype of EDS and the least severe one; EDS-HT is considered to be identical to the joint hypermobility syndrome and manifests with musculoskeletal complaints, joint instability, and soft tissue overuse injury. Musculoskeletal complaints manifest with joint pain of non-inflammatory origin and/or spinal pain. Joint instability leads to dislocation or subluxation and involves peripheral joints as well as central joints, including the temporomandibular joints, sacroiliac joints, and hip joints. Soft tissue overuse injury may lead to tendonitis and bursitis without joint inflammation in most cases. Ehlers–Danlos syndrome-HT carries a high potential for disability due to recurrent dislocations and subluxations and chronic pain. Throughout the years, extra-articular manifestations have been described, including cardiovascular, autonomic nervous system, gastrointestinal, hematologic, ocular, gynecologic, neurologic, and psychiatric manifestations, emphasizing the multisystemic nature of EDS-HT. Unfortunately, EDS-HT is under-recognized and inadequately managed, leading to neglect of these patients, which may lead to severe disability that almost certainly could have been avoided. In this review article we will describe the known manifestations of the extra-articular systems.

Multisystemic resilience has been conceptualised as involving a constellation of protective factors which operate at different levels to promote adaptation and thriving despite experiences of adversity. We used network modelling to discover how protective factors at two different systemic levels (intrapersonal strengths and social-ecological resources) interrelate, drawing on survey data from 5283 emerging adults ( M = 24.53 years; 52% female) in Brazil, China, Indonesia, Russia, Thailand, the US and Vietnam. Results indicated that the level of connectivity within and between protective factor levels was similar between the countries, but that there was substantial variation in the specific interrelations among protective factors (both within and between levels), including the presence of some country-specific negative interrelations between protective factors at different levels. The findings support the importance of cultural context in studies of resilience, with implications for the development of appropriate resilience-building interventions for this age group., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© 2022 Society for the Study of Emerging Adulthood and SAGE Publishing.)

Ehlers-Danlos syndrome (EDS)--hypermobility type (HT) is considered to be the most common subtype of EDS and the least severe one; EDS-HT is considered to be identical to the joint hypermobility syndrome and manifests with musculoskeletal complaints, joint instability, and soft tissue overuse injury. Musculoskeletal complaints manifest with joint pain of non-inflammatory origin and/or spinal pain. Joint instability leads to dislocation or subluxation and involves peripheral joints as well as central joints, including the temporomandibular joints, sacroiliac joints, and hip joints. Soft tissue overuse injury may lead to tendonitis and bursitis without joint inflammation in most cases. Ehlers-Danlos syndrome-HT carries a high potential for disability due to recurrent dislocations and subluxations and chronic pain. Throughout the years, extra-articular manifestations have been described, including cardiovascular, autonomic nervous system, gastrointestinal, hematologic, ocular, gynecologic, neurologic, and psychiatric manifestations, emphasizing the multisystemic nature of EDS-HT. Unfortunately, EDS-HT is underrecognized and inadequately managed, leading to neglect of these patients, which may lead to severe disability that almost certainly could have been avoided. In this review article we will describe the known manifestations of the extra-articular systems. [ABSTRACT FROM AUTHOR]

National and state surveys over the past several decades have concluded that school social workers, despite an awareness of and training in macro level practice strategies, are highly individualistic in their practice focus. Although clinical skills are necessary, they are insufficient for effective school social work practice in the 21st century. No studies have moved beyond asking what tasks school social workers perform to asking what predicts these tasks. The purpose of this study was to explore which factors appear to shape and influence school social work practice in Tennessee. The authors found no evidence that school social workers' characteristics (for example, gender, race, education, grade levels served) affect their choice of interventions, but they did find evidence that organizational and systemic sociocultural factors affect the types of interventions that school social workers perform. The authors discuss the implications for practice and organizational change. [ABSTRACT FROM AUTHOR]

Caprine arthritis encephalitis virus (CAEV) is a lentivirus that infects both goats and sheep and is closely related to maedi-visna virus that infects sheep; collectively, these viruses are known as small ruminant lentiviruses (SRLV). Infection of goats and sheep with SRLV typically results in discrete inflammatory diseases which include arthritis, mastitis, pneumonia or encephalomyelitis. SRLV-infected animals concurrently demonstrating lentivirus-associated lesions in tissues of lung, mammary gland, joint synovium and the central nervous system are either very rare or have not been reported. Here we describe a novel CAEV promoter isolated from a sheep with multisystemic lentivirus-associated inflammatory disease including interstitial pneumonia, mastitis, polyarthritis and leukomyelitis. A single, novel SRLV promoter was cloned and sequenced from five different anatomical locations (brain stem, spinal cord, lung, mammary gland and carpal joint synovium), all of which demonstrated lesions characteristic of lentivirus associated inflammation. This SRLV promoter isolate was found to be closely related to CAEV promoters isolated from goats in northern California and other parts of the world. The promoter was denoted CAEV-ovine-MS (multisystemic disease); the stability of the transcription factor binding sites within the U3 promoter sequence are discussed. [ABSTRACT FROM AUTHOR]

Multisystemic therapy (MST) is effective for decreasing or preventing delinquency and other externalizing behaviors and increasing prosocial or adaptive behaviors. The purpose of this project was to review the literature examining the efficacy of MST for other child psychological and health problems reflecting non-externalizing behaviors, specifically difficulties related to child maltreatment, serious psychiatric illness [Serious psychiatric illness was defined throughout the current review paper as the 'presence of symptoms of suicidal ideation, homicidal ideation, psychosis, or threat of harm to self or others due to mental illness severe enough to warrant psychiatric hospitalization based on the American Academy of Child and Adolescent Psychiatry (Level of care placement criteria for psychiatric illness. American Academy of Child and Adolescent Psychiatry, Washington, DC, ) level of care placement criteria for psychiatric illness' (Henggeler et al. in J Am Acad Child Psy 38:1331-1345, p. 1332, ). Additionally, youth with 'serious emotional disturbance (SED)' defined as internalizing and/or externalizing problems severe enough to qualify for mental health services in public school who were 'currently in or at imminent risk of a costly out-of-home placement' (Rowland et al. in J Emot Behav Disord 13:13-23, pp. 13-14, ) were also included in the serious psychiatric illness category.], and health problems (i.e., obesity and treatment adherence for diabetes). PubMed, Web of Science, MEDLINE, and PsycINFO databases; Clinicaltrials.gov; DARE; Web of Knowledge; and Cochrane Central Register of Controlled Trials were searched; and MST developers were queried to ensure identification of all relevant articles. Of 242 studies identified, 18 met inclusion criteria for review. These were combined in a narrative synthesis and critiqued in the context of review questions. Study quality ratings were all above mean scores reported in prior reviews. Mixed support was found for the efficacy of MST versus other treatments. In many cases, treatment effects for MST or comparison groups were not sustained over time. MST was efficacious for youth with diverse backgrounds. No studies discussed efficacy of MST provided in different treatment settings. Four studies found MST more cost-effective than a comparison treatment, leading to fewer out-of-home placements for youth with serious psychiatric illness or lower treatment costs for youth with poorly controlled diabetes. [ABSTRACT FROM AUTHOR]

Canine monocytic ehrlichiosis (CME) caused by Ehrlichia canis is a multisystemic tick-borne disease. The ubiquity of the rickettsia throughout the body organs leads to its involvement in the pathology of organs. The induced profound lymphoplasmacytosis of all parenchymal organs and the bleeding tendencies in all body systems, results in a great variety of clinical signs. This review sets out to present the pathological changes associated with CME, and in so doing attempts to correlate them with clinical manifestations and broaden their scope for the awareness and benefit of the practicing veterinarian. Understanding of this concept may result in an increased inclusion of CME in the differential diagnoses of many organ related problems. This approach may result in the improvement of the diagnosis of CME and consequently the initiation of a specific treatment at an early stage with an improved prospect of cure. [ABSTRACT FROM AUTHOR]

Although research has shown that multi-systemic interventions (MSIs) improve youth psychosocial functioning, MSI dissemination has been hampered by low levels of parent participation. The current pilot project was designed to examine the effectiveness of a brief motivational interviewing (MI) protocol to increase parental engagement in an MSI for youth suspended from middle and high school. Preliminary findings suggest that parents who received brief MI were more likely to attend a parent-training workshop and reported greater satisfaction with the parenting workshops than parents who did not. Parents in the brief MI group also reported a relatively high level of satisfaction with the MI procedures. Implications for future research and MSIs are discussed. [ABSTRACT FROM AUTHOR]

Developments in the theories of identity, culture, and traumatology enrich our cross-cultural understanding of mental health dynamics, case conceptualization, and developing effective intervention models to help victims of complex and cumulative traumas especially in different cultures and minority populations. Identity traumas, along with preidentity and nonidentity traumas, contribute to forming cultures. On the other hand, cultures may contribute to delivering some types of culture- and social-made serious traumatic conditions that can be transferred cross-generationally, such as poverty and caste systems. Most current interventions are designed to help with single trauma and ignore the cumulative trauma dynamics as well the collective identity and culture-specific traumas. This understanding entails revising our culturally limited and single-trauma-based interventions to help clients who belong to different cultures or to minority victims of culture- and social-made traumas as well as those who are victims of cumulative traumas. Multisystemic, multimodal, multicomponent flexible and fiddle therapy models emerged as potentially more effective in the treatment of disorders resulting from cumulative and identity traumas. They are more ecologically valid and culturally competent. Specific models of multisystemic, multimodal therapies— the wraparound psychosocial rehabilitation approach, for torture survivors, and the summer day and after-school treatment, for child victims of cumulative traumas—are discussed. [ABSTRACT FROM PUBLISHER]

Langerhans cell histiocytosis (LCH) is a rare group of idiopathic diseases characterized by abnormal proliferation of bone marrow-derived dendritic cells called histiocytes. It is more common in childhood, but in the literature, some adult cases have been reported. For several reasons, the disease is more familiar to pediatricians than it is to physicians handling adult cases; therefore, the diagnosis of adult cases is often delayed or missed. The clinical course of LCH is variable, ranging from a self-healing solitary bone lesion to a widely disseminated life-threatening disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of perilesional skin. In this report, we present a case of LCH in a 66-yearold woman with bone, skin, and pituitary gland involvement; this case emphasizes that a patient with LCH should undergo careful multidisciplinary evaluation. [ABSTRACT FROM AUTHOR]

African-American adolescents have the highest rates of asthma morbidity and mortality, yet there are few successful behavioral interventions to improve illness management for this group. Mental health providers have an opportunity to expand their services and impact by targeting adolescents with poor asthma management. We describe the adaptation of Multisystemic Therapy (MST), an intensive, home and community-based treatment originally used with antisocial youth, for improving asthma management. We present a case study to illustrate the treatment model. Feasibility was demonstrated with eight African American adolescents with chronic, persistent asthma, suggesting that further evaluation of MST for youth with asthma and poor illness management is warranted. [ABSTRACT FROM AUTHOR]

We examined client outcomes from the implementation of Multisystemic Therapy (MST) in a statewide child and adolescent mental health system. Specifically, we examined (1) the validity of therapist-rated MST outcome measures by comparing them to ratings of functional impairment and level of service needs by CAMHD care coordinators, who provide case management and care coordination services, (2) potential client and service predictors of therapist-rated outcomes, and (3) improvement in youth functioning around the time of entry to and exit from MST compared with rates of improvement reported in randomized controlled trials (RCTs) by the developers of MST. Results suggested that therapist-rated MST outcomes were valid indicators of treatment success. Similar to other findings in the MST literature, few client or service characteristics predicted these outcomes. Finally, although MST entry–exit effect sizes were lower than the mean derived from RCTs published by the developers, they were within the 95% confidence interval. Together, these findings support the implementation of MST in complex systems of care with continued attention to quality assurance and ongoing use of data for evaluation. [ABSTRACT FROM AUTHOR]

Multisystemic Therapy (MST) is a well-validated, evidenced-based treatment for serious clinical problems presented by adolescents and their families. Across randomized clinical trials, MST has been effective in reducing out-of-home placements, delinquent behavior, substance use, and psychiatric symptomatology compared to services currently available in the community. This article is an introduction to the MST approach and outlines key clinical features, describes the theoretical underpinnings, and discusses the empirical support for MST's effectiveness with a variety of serious clinical issues. [ABSTRACT FROM AUTHOR]

We describe a program for promoting resilience for adolescents, a multisystemic approach based on the interpersonal theory of Harry Stack Sullivan (1953), designed to empower adolescents, especially high risk minority youths and those who must overcome adversity. A system is developed so as to promote resilience in the adolescent and support by his/her family and school, as the host program, to develop empathic relatedness, use of tender feelings, self exploration, and the management of anxieties, in the context of asking for and accepting help from others. Staff interventions emphasize ways adults can become allies during these youths' quest for resilience, achieved in the context of the pursuit of a mutually identified and accepted goal. All participants report and manifest behavioral benefits from this program. [ABSTRACT FROM PUBLISHER]

Children and adolescents diagnosed with conduct disorder present a formidable challenge to school social workers. Five guidelines for planning interventions with this population are presented. These guidelines are based on a synthesis of child development research and suggest that attention to the biological makeup of students as well as the design of multimodal and multisystemic interventions are critical to improving outcomes for children and adolescents diagnosed with conduct disorder. [ABSTRACT FROM AUTHOR]

Cardiomyopathy (CMP) is a rare disease in the pediatric population, with a high risk of morbidity and mortality. The genetic etiology of CMPs in children is extremely heterogenous. These two factors play a major role in the difficulties of establishing standard diagnostic and therapeutic protocols. Isolated CMP in children is a frequent finding, mainly caused by sarcomeric gene variants with a detection rate that can reach up to 50% of analyzed cohorts. Complex multisystemic forms of pediatric CMP are even more heterogenous. Few studies in literature take into consideration this topic as the main core since it represents a rarity (systemic CMP) within a rarity (pediatric population CMP). Identifying etiology in this cohort is essential for understanding prognosis, risk stratification, eligibility to heart transplantation and/or mechanical-assisted procedures, preventing multiorgan complications, and relatives' recurrence risk calculation. The previous points represent a cornerstone in patients' empowerment and personalized medical care approach. The aim of this work is to propose a new approach for an algorithm in the setting of the diagnostic framework of systemic pediatric CMP. On the other hand, during the literature review, we noticed a relatively common etiologic pattern in some forms of complex/multisystem CMP. In other words, certain syndromes such as Danon, Vici, Alström, Barth, and Myhre syndrome share a common pathway of directly or indirectly defective "autophagy" process, which appears to be a possible initiating/triggering factor for CMPs. This conjoint aspect could be important for possible prognostic/therapeutic implications in this category of patients. However, multicentric studies detailed functional and experimental models are needed prior to deriving conclusions.

The article presents author's views regarding various facts related to blastomycosis in horses and mentions a case of cutaneous lesions caused by blastomyces dermatitidis. Topics discussed include need of understanding of the disease epidemiology, diagnosis and prognosis, disease spread through direct skin penetration via bites, puncture wounds or abrasions and role of neutrophils, monocytes and tissue macrophages.

Fabry disease is an X-linked inborn disease caused by deficit of alpha-galactosidaseA. This results in accumulation of glycosphingolipids in all cells and tissues. All males should receive enzyme replacement treatment in case of very low or undetectable levels of alpha-galactosidaseA. Female carriers and males with marginally levels of alpha-galactosidaseA should be treated in case of renal, neurologic o cardiac manifestations. There are two intravenous formulations of human recombinant enzyme, agalsidase alpha and agalsidase beta, showing similar efficacy and safety. Patients with amenable mutations of alpha-galactosidase can be treated with oral migalastat hydrochloride. Migalastat hydrochloride is a pharmacological chaperone that facilitates trafficking of alpha-galactosidaseA to lysosomes increasing enzyme activity. Patients treated with migalastat hydrochloride had significant improvements in left ventricular mass and gastrointestinal symptoms., (Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.)