scholarly journals Hemophagocytic syndrome triggered by intense physical activity and viral infection in a young adult female with three heterozygous mutations in Munc-18-2

2017 ◽  
Vol 74 (5) ◽  
pp. 494-497
Author(s):  
Olivera Markovic ◽  
Dragana Janic ◽  
Milorad Pavlovic ◽  
Ljiljana Tukic ◽  
Srdja Jankovic ◽  
...  
Keyword(s):  
Physical Activity ◽  
Viral Infection ◽  
Protein Function ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Diagnostic Procedures ◽  
Acceptor Side ◽  
Complete Clinical Remission ◽  
Life Threatening ◽  
Strenuous Physical Activity ◽  
Intense Physical Activity

Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially life-threatening, hyperinflammatory syndrome caused by severe hypercytokinemia due to a highly stimulated, but ineffective immune response. Case report. We reported a 19-year-old woman presenting with fever, muscle and joint pain and sore throat. After diagnostic procedures we made the diagnosis of hemophagocytic lymphohistiocytosis (7 of 8 HLH-2004 diagnostic criteria) caused by Ebstein-Barr viral infection and trigerred by the intense physical activity. Genetic analysis showed three different sequence changes in Munc-18-2, two splice acceptor side mutations/changes affecting exon 10 (c.795?4 C > T) and exon 15 (c.1247?10 C > T) and a missense mutation c.1375 C > T; p.Arg 459 Trp. All mutations were in heterozygous state and their significance in pathogensis of HLH is not clear. After treatment with corticosteroids and cyclosporin A complete clinical remission was achieved. Conclusion. The presented case history suggests the possibility that mutations of undetermined clinical significance in a gene associated with primary HLH may underlie some cases of secondary HLH, probably by causing a partial, rather than total or subtotal, impairment of encoded protein function. Our case also suggests that strenuous physical activity (in apparent synergy with viral infection) can trigger HLH.

scholarly journals Spontaneous Pneumomediastinum from Running Sprints

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Joseph W. Turban
Keyword(s):  
Physical Activity ◽  
Lung Cancer ◽  
Asthma Exacerbation ◽  
Rare Condition ◽  
Intrathoracic Pressure ◽  
Benign Condition ◽  
Spontaneous Pneumomediastinum ◽  
Free Air ◽  
Life Threatening ◽  
Intense Physical Activity

Spontaneous pneumomediastinum (SPM) is a fairly rare condition, caused by increased intrathoracic pressure, leading to free air in the mediastinal structures. Underlying lung conditions are associated with increased incidence of SPM, including asthma, interstitial lung disease, pneumonia, bullous lung, and radiation therapy for lung cancer. It is often preceded by Valsalva maneuvers, vomiting, coughing, asthma exacerbation, sneezing, childbirth, or intense physical activity. A case of SPM is presented in a 15-year-old male, who complained of throat pain and dyspnea while running sprints at football practice. Workup revealed SPM, and he was subsequently admitted and treated conservatively. His symptoms resolved in 2 days and he was discharged and suffered no further recurrences. In contrast to secondary pneumomediastinum, SPM is usually a benign condition although life-threatening conditions can rarely arise. Differentiating between these two conditions has important prognostic indications. There is a paucity of prospectively collected data regarding SPM, and considerable variation in recommendations concerning the extent of workup.

scholarly journals Pseudomonas-Contaminated Pool Triggering an Episode of Idiopathic Palmoplantar Hidradenitis

2021 ◽  
pp. 411-416
Author(s):  
Rasheed K. Barradah
Keyword(s):  
Physical Activity ◽  
Pseudomonas Infection ◽  
Intense Physical Activity

Idiopathic palmoplantar hidradenitis (IPPH) is a disorder that mainly affects the palms and soles of children. Although many cases have been reported to occur after recreational swimming activities, whether IPPH is caused by intense physical activity or by Pseudomonas infection has yet to be proven. We report a case of a 3-year-old girl who presented with IPPH after swimming in a pool with evidence of <i>P. aeruginosa</i> contamination, further solidifying the association between Pseudomonas and IPPH.

scholarly journals Pacemaker-Generated Stress Fracture of The Second Rib: A Case Report

2020 ◽  
Author(s):  
Jus Ksela ◽  
Mark Racman ◽  
Rok Zbacnik ◽  
Anze Djordjevic ◽  
Matevz Jan
Keyword(s):  
Physical Activity ◽  
Computed Tomography ◽  
Female Patient ◽  
Stress Fracture ◽  
Pulse Generator ◽  
Pacemaker Implantation ◽  
Permanent Pacemaker ◽  
Direct Trauma ◽  
The Right ◽  
Intense Physical Activity

Abstract Background: Pocket-related complications following the implantation of cardiovascular implantable electronic devices primarily include pocket hematoma, infection, skin erosion or decubitus, device migration, and Twiddler's syndrome, with other pathologies such as nerve impairment or bone lesions being extremely rarely encountered. We report a case of a 20-year old asthenic, non-athlete female patient presenting with a device-generated fracture of the second rib several months after sub-muscular permanent pacemaker implantation due to repeated bilateral pre-pectoral pocket infections.Case presentation: A 20-year old female patient was readmitted to our institution 9 months following sub-pectoral implantation of a permanent pacemaker, complaining of severe pocket-related pain, which arose spontaneously in the absence of direct trauma, intense physical activity or vigorous coughing, and was associated with normal day-to-day activity. To rule out a pacemaker re-infection, a native computed tomography and a positron emission tomography - computed tomography of the thorax were performed. Both modalities excluded an infection but showed a healing fracture and a focus of enhanced metabolic activity in the anterolateral part of the right second rib, indicating a non-traumatic or stress fracture of the bone. Consequently, a complete extraction of the pulse generator and both leads was performed and the smallest available single-chamber pulse generator with a single atrial electrode was implanted in the sub-fascial, pre-muscular pocket in the now recovered and uninfected left subclavicular region, alleviating patient’s severe pain symptoms and significantly enhancing her quality of life.Conclusions: In the absence of direct trauma, intense physical activity or vigorous coughing, we assume that in this asthenic girl a normal day-to-day motion of the right shoulder has persistently forced the sub-muscularly placed pulse generator toward thoracic wall, putting increased repetitive pressure force on the underlying bones, finally causing a fatigue stress fracture of the second rib. In asthenic phenotype patients with small thorax and short subclavicular distance, a sub-muscular pacemaker implantation can potentially cause unique and unexpected pocket-related adverse events necessitating advanced diagnostics and timely treatment.

scholarly journals Children and nutrition

2017 ◽  
Vol 14 (3) ◽  
pp. 2345
Author(s):  
Evren Ebru Altıncı
Keyword(s):  
Physical Activity ◽  
Role Models ◽  
Growth And Development ◽  
Eating Habits ◽  
Development Stage ◽  
Optimal Performance ◽  
External Factors ◽  
Energy Depletion ◽  
Balanced Diet ◽  
Intense Physical Activity

It is possible to raise healthy generations by improving the awareness levels of the role models (parents, teachers etc.) who will shape the perspectives of future parents towards nutrition and physical activity. That children receive adequate levels of nutrition from proper sources, which is vital for children’s growth and development, is considerably under the control of external factors (e.g. family and friends’ eating habits, third person’s care/knowledge and experiences such as teachers/trainers etc.). Considering nutrition and the intense physical activity of preschoolers, which is an inherent characteristic of that development stage, and especially the children who are guided to do more intense physical activity, the case becomes more complex. Thus, with the aim to address children’s energy depletion from intense physical activity, it is suggested to provide children with attractive alternatives. Including some of these alternatives, this study aims to reveal the significance of an adequate and balanced diet as well as proper planning of physical activity and periodic assessments at the appropriate time and conditions so that the children who start regular physical activity display optimal performance.

Strenuous physical activity in young adulthood and risk of breast cancer (United States)

1995 ◽  
Vol 6 (4) ◽  
pp. 347-353 ◽  
Author(s):  
Robert Mittendorf ◽  
Matthew P. Longnecker ◽  
Polly A. Newcomb ◽  
Amy T. Dietz ◽  
E. Robert Greenberg ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Physical Activity ◽  
United States ◽  
Young Adulthood ◽  
Strenuous Physical Activity

scholarly journals ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Disseminated Intravascular Coagulation and Hemophagocytic Lymphohistiocytosis: A Potentially Fatal Presentation

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Uroosa Ibrahim ◽  
Amina Saqib ◽  
Maryam Rehan ◽  
Jean Paul Atallah
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Anaplastic Large Cell Lymphoma ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Lymph Node Biopsy ◽  
Intravascular Coagulation ◽  
Primary Disorder ◽  
Life Threatening ◽  
Large Cell Lymphoma

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.

Living on the Border

2019 ◽  
pp. 135-159
Author(s):  
Katie Marie Whitmore ◽  
Michele R. Buzon ◽  
Stuart Tyson Smith
Keyword(s):  
Physical Activity ◽  
Traumatic Injury ◽  
New Kingdom ◽  
Peaceful Coexistence ◽  
Burial Practices ◽  
Public Displays ◽  
The Third ◽  
Egyptian Culture ◽  
Strenuous Physical Activity ◽  
The Relationship

Tombos is located at the Third Cataract of the Nile River in modern-day Sudan and marks an important literal and figurative boundary between Egyptian and Nubian interaction. During the New Kingdom Period (1400–1050 BCE), the cemetery at Tombos in Upper Nubia exhibits the use of Egyptian mortuary practices, including monumental pyramid complexes, likely used by both immigrant Egyptians and local Nubians. Despite the influence of Egyptian culture during this colonial period, there are several public displays of Nubian identity in burial practices found at Tombos. This mixture of Egyptian and Nubian burial practices extends into the postcolonial period at Tombos. Paleopathological analyses indicate that the Nubian and Egyptian individuals living at colonial Tombos enjoyed access to nutritional food resources and displayed low levels of skeletal markers of infection, traumatic injury, and strenuous physical activity. While the Tombos sample is likely not representative of all Egyptian-Nubian interaction during the New Kingdom, the individuals examined appear to have benefited from the relationship. In contrast with many situations of frontier interaction, the bioarchaeological evidence indicates a relatively peaceful coexistence between Egyptians and Nubians at Tombos, and the construction of a new biologically and culturally entangled community.

The Contribution of mHealth in the Care of Obese Pediatric Patients

2016 ◽  
pp. 126-146 ◽  
Author(s):  
Elpis Vlachopapadopoulou ◽  
Dimitrios I. Fotiadis
Keyword(s):  
Physical Activity ◽  
Diabetes Mellitus ◽  
Remote Monitoring ◽  
Chronic Health Condition ◽  
Health Condition ◽  
Self Management ◽  
Closed Loops ◽  
Global Health Issue ◽  
Life Threatening ◽  
Motivational Approaches

In this chapter the evolution of mHealth solutions for monitoring and treatment of children suffering from obesity is discussed. Nowadays, obesity emerges as a major chronic health condition that affects the general population, both children and adults. mhealth solutions are already used for self-management, remote monitoring and counseling of several chronic conditions, including diabetes mellitus, heart failure, Parkinson's disease, etc. Today, those solutions can result to closed loops, which support health self-management for chronic diseases, in a personalized manner. Concerning childhood obesity, those solutions can combine targeted games and motivational approaches towards both physical activity and diet, which could help in addressing this serious and global health issue, in the direction of minimizing co-morbidities and eventually preventing serious, life threatening events.

scholarly journals Nivolumab treatment of relapsed/refractory Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis in adults

Blood ◽  
2020 ◽  
Vol 135 (11) ◽  
pp. 826-833 ◽  
Author(s):  
Pengpeng Liu ◽  
Xiangyu Pan ◽  
Chong Chen ◽  
Ting Niu ◽  
Xiao Shuai ◽  
...  
Keyword(s):  
T Cells ◽  
Cd8 T Cells ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Sequencing Analysis ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Life Threatening ◽  
Programmed Death Protein 1 ◽  
Epstein Barr

Abstract Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome triggered by EBV infection. It often becomes relapsed or refractory (r/r), given that etoposide-based regimens cannot effectively clear the virus. r/r EBV-HLH is invariably lethal in adults without allogeneic hematopoietic stem cell transplantation. Here, we performed a retrospective analysis of 7 r/r EBV-HLH patients who were treated with nivolumab on a compassionate-use basis at West China Hospital. All 7 patients tolerated the treatment and 6 responded to it. Five of them achieved and remained in clinical complete remission with a median follow-up of 16 months (range, 11.4-18.9 months). Importantly, both plasma and cellular EBV-DNAs were completely eradicated in 4 patients. Single-cell RNA-sequencing analysis showed that HLH syndrome was associated with hyperactive monocytes/macrophages and ineffective CD8 T cells with a defective activation program. Nivolumab treatment expanded programmed death protein-1–positive T cells and restored the expression of HLH-associated degranulation and costimulatory genes in CD8 T cells. Our data suggest that nivolumab, as a monotherapy, provides a potential cure for r/r EBV-HLH, most likely by restoring a defective anti-EBV response.

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