scholarly journals Juvenile fibromyalgia: A call for diagnostic refinement, a case report

2020 ◽  
Author(s):  
Elisha Peterson ◽  
Caylynn Yao ◽  
Sangeeta D. Sule ◽  
Julia C. Finkel

Abstract BackgroundFibromyalgia is a clinical syndrome consisting of widespread musculoskeletal tenderness and various somatic complaints including nonrestorative sleep, mood disorders such as anxiety or depression, abdominal pain, and/or headaches. There is a great deal of heterogeneity in its expression which leads to difficulty in identifying predisposing factors. A singular review of patients in an academic pediatric pain clinic reveal immune system dysfunction, mood disorders, infection, postural orthostatic tachycardia syndrome, complex regional pain syndrome, and hypermobility are premorbid conditions. It is unclear if these premorbid conditions confer a distinct fibromyalgia clinical phenotype that can provide insight into targeted therapies. Current diagnostic measures for fibromyalgia do not allow for this level of discrimination and are not validated in children.Case Presentation20 children who demonstrated widespread musculoskeletal pain, tenderness to pressure on exam and multiple somatic complaints were diagnosed with fibromyalgia. Average time from start of pain to diagnosis is 2 years. Over half the patients have psychopathology, a third have an immune system dysfunction related to autoimmunity or an infectious exposure, a third with orthostatic intolerance or postural orthostatic tachycardia syndrome, a quarter relating to hypermobility, and a quarter of the cohort with dysmenorrhea were pre or comorbid conditions. Effective therapeutic regimens among patients varied widely from responding to medical monotherapy to multimodal treatment. Trigger point injections worsened pain in one fibromyalgia patient but decreased pain in another. Patients with comorbid autoimmunity report appreciating a difference between a flare in their arthritis as opposed to a flare in their fibromyalgia. Such varying responses within the same clinical syndrome suggest distinct phenotypes within fibromyalgia which is difficult to distinguish using our current diagnostic tools.ConclusionThere is a need for clear diagnostic criteria for both the recognition of juvenile fibromyalgia and tools to distinguish phenotypes within fibromyalgia. Currently, the recognition of clinical symptoms renders it an often-overlooked neuropathic pain condition. This case series suggest there are different phenotypes within fibromyalgia. Some patients respond remarkably to serotonin norepinephrine reuptake inhibitors alone whereas others require multidisciplinary therapy. A diagnostic tool refined to capture these nuances can facilitate targeted treatment recommendations.

PEDIATRICS ◽  
2022 ◽  
Vol 149 (Supplement_1) ◽  
pp. S91-S98
Author(s):  
Mark W. Hall ◽  
Joseph A. Carcillo ◽  
Timothy Cornell

CONTEXT Immune system dysfunction is poorly represented in pediatric organ dysfunction definitions. OBJECTIVE To evaluate evidence for criteria that define immune system dysfunction in critically ill children and associations with adverse outcomes and develop consensus criteria for the diagnosis of immune system dysfunction in critically ill children. DATA SOURCES We conducted electronic searches of PubMed and Embase from January 1992 to January 2020, using medical subject heading terms and text words to define immune system dysfunction and outcomes of interest. STUDY SELECTION Studies of critically ill children with an abnormality in leukocyte numbers or function that is currently measurable in the clinical laboratory in which researchers assessed patient-centered outcomes were included. Studies of adults or premature infants, animal studies, reviews and commentaries, case series (≤10 subjects), and studies not published in English with inability to determine eligibility criteria were excluded. DATA EXTRACTION Data were abstracted from eligible studies into a standard data extraction form along with risk of bias assessment by a task force member. RESULTS We identified the following criteria for immune system dysfunction: (1) peripheral absolute neutrophil count <500 cells/μL, (2) peripheral absolute lymphocyte count <1000 cells/μL, (3) reduction in CD4+ lymphocyte count or percentage of total lymphocytes below age-specific thresholds, (4) monocyte HLA-DR expression <30%, or (5) reduction in ex vivo whole blood lipopolysaccharide-induced TNFα production capacity below manufacturer-provided thresholds. LIMITATIONS Many measures of immune system function are currently limited to the research environment. CONCLUSIONS We present consensus criteria for the diagnosis of immune system dysfunction in critically ill children.


Viruses ◽  
2021 ◽  
Vol 13 (7) ◽  
pp. 1287
Author(s):  
T. Walter ◽  
Jennifer Iudicello ◽  
Debra Cookson ◽  
Donald Franklin ◽  
Bin Tang ◽  
...  

Methamphetamine (METH) use disorder is highly prevalent among people with HIV (PWH) and is a significant public health problem. HIV and METH use are each associated with immune system dysfunction; however, the combined effects on the immune system are poorly understood. This cross-sectional project measured soluble immune biomarkers in plasma and cerebrospinal fluid (CSF) collected from a control group, people with a history of a METH use disorder (METH+), PWH with no history of METH use disorder (HIV+), and PWH with a history of METH use disorder (HIV+/METH+). HIV, METH, and immune dysfunction can also be associated with affective and cognitive deficits, so we characterized mood and cognition in our participants. Two factor analyses were performed for the plasma and CSF biomarkers. Plasma IL-8, Ccl2, VEGF, and 8-isoprostane loaded onto one factor that was highest in the HIV+/METH+ group (p < 0.047) reflecting worse inflammation, vascular injury, and oxidative stress. This plasma factor was also negatively correlated with delayed recall (R = −0.49, p = 0.010), which was worst in the HIV+/METH+ group (p = 0.030 compared to the control group). Overall, these data implicate that combined HIV-1 infection and METH use may exacerbate inflammation, leading to worse cognition.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Madeleine Johansson ◽  
Fabrizio Ricci ◽  
Janin Schulte ◽  
Margaretha Persson ◽  
Olle Melander ◽  
...  

AbstractPostural orthostatic tachycardia syndrome (POTS) is a cardiovascular autonomic disorder with poorly understood etiology and underlying pathophysiology. Since cardiovascular morbidity has been linked to growth hormone (GH), we studied GH levels in patients with POTS. We conducted an age-sex-matched case–control study in patients with POTS (age 31 ± 9 years; n = 42) and healthy controls (32 ± 9 years; n = 46). Plasma GH levels were measured using high-sensitivity chemiluminescence sandwich immunoassay. The burden of orthostatic intolerance symptoms was assessed by the Orthostatic Hypotension Questionnaire (OHQ), consisting of a symptom assessment scale (OHSA) and a daily activity scale (OHDAS). POTS patients had significantly higher composite OHQ score than controls, more symptoms and less activity. Supine heart rate and diastolic blood pressure (BP), but not systolic BP, were significantly higher in POTS. Median plasma GH levels were significantly lower in POTS (0.53 ng/mL) than controls (2.33 ng/mL, p = 0.04). GH levels were inversely related to OHDAS in POTS and supine systolic BP in POTS and controls, but not heart rate neither group. POTS is associated with lower GH levels. Impairment of daily life activities is inversely related with GH in POTS. A higher supine diastolic BP is inversely associated with GH levels in POTS and healthy individuals.


2021 ◽  
Vol 22 (5) ◽  
pp. 2333
Author(s):  
Yulong Sun ◽  
Yuanyuan Kuang ◽  
Zhuo Zuo

In the process of exploring space, the astronaut’s body undergoes a series of physiological changes. At the level of cellular behavior, microgravity causes significant alterations, including bone loss, muscle atrophy, and cardiovascular deconditioning. At the level of gene expression, microgravity changes the expression of cytokines in many physiological processes, such as cell immunity, proliferation, and differentiation. At the level of signaling pathways, the mitogen-activated protein kinase (MAPK) signaling pathway participates in microgravity-induced immune malfunction. However, the mechanisms of these changes have not been fully elucidated. Recent studies suggest that the malfunction of macrophages is an important breakthrough for immune disorders in microgravity. As the first line of immune defense, macrophages play an essential role in maintaining homeostasis. They activate specific immune responses and participate in large numbers of physiological activities by presenting antigen and secreting cytokines. The purpose of this review is to summarize recent advances on the dysfunction of macrophages arisen from microgravity and to discuss the mechanisms of these abnormal responses. Hopefully, our work will contribute not only to the future exploration on the immune system in space, but also to the development of preventive and therapeutic drugs against the physiological consequences of spaceflight.


Author(s):  
Lian-Mei Zhao ◽  
Yun-Long Jia ◽  
Ming Ma ◽  
Yu-Qing Duan ◽  
Li-Hua Liu

2011 ◽  
Vol 77 (4) ◽  
pp. 573-579 ◽  
Author(s):  
Maryam Khojasteh-Fard ◽  
Mina Tabrizi ◽  
Mahsa M. Amoli

Author(s):  
Chandralekha Ashangari ◽  
Samreen F Asghar ◽  
Sadaf Syed ◽  
Amna A Butt ◽  
Amer Suleman

Background: Postural orthostatic tachycardia syndrome (POTS) is an autonomic disturbance characterized by the clinical symptoms of orthostatic intolerance, mainly light headedness, fatigue, sweating, tremor, anxiety, palpitation, exercise intolerance and near syncope on upright posture. These are relieved on lying down. Patients also have a heart rate >120 beats/min (bpm) on standing or increase their heart rate by 30 bpm from a resting heart rate after standing for 10 min. A nerve conduction study (NCS) is a medical diagnostic test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body. The aim of this study is to demonstrate median, ulnar, peroneal, tibial nerve conduction results POTS patients. Methods: 177 patients were selected randomly from our clinic with POTS. Nerve conduction results of median, ulnar, peroneal, tibial nerves were reviewed from electronic medical records. Results: Out of 177 patients, 151 patients are females (85%, n=151, age 32.07±11.10), 26 patients are males (15%, n=26, age 29.08±17.40).Median nerve conduction results are 57.83 m/sec ±7.58 m/sec, Ulnar nerve conduction results are 56.62 m/sec ±6.85 m/sec, Peroneal nerve conduction results are 49.96 m/sec ±6.85 m/sec, Tibial nerve conduction results are 50.70 m/sec ±6.86 m/sec. Conclusion: The nerve conduction velocities tend to be within normal range in Postural Orthostatic Tachycardia Syndrome (POTS) patients.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Antonio Lacquaniti ◽  
Susanna Campo ◽  
Teresa Casuscelli Di Tocco ◽  
Paolo Monardo

Abstract Background and Aims Uremic toxins, poor removed by conventional hemodialysis (HD), represent independent risk factors for mortality in end-stage renal disease (ESRD). Middle uremic toxin molecules were associated to pathological features of uremia, such as immune dysfunction and inflammation. These two entities are not mutually exclusive, but they could represent two sides of the same coin. ESRD-associated inflammation is closely related to the activation of innate immune system. Free light chain (FLC) may be a specific assessment of inflammation, representing a direct function of adaptive immunity through B-cell lineage production rather than a general marker of inflammation. While several studies have assessed the relation between FLCs and mortality risk in chronic kidney disease (CKD), FLCs, as uremic toxins in non-multiple myeloma dialyzed patients, were marginally analyzed. The aim of this prospective study was to evaluate the clinical impact of FLCs levels in HD patients, during a 2-years follow-up analysing the relations with biomarkers of inflammation, such as C-reactive protein (CRP) and procalcitonin (PCT), main lymphocytes subsets, such as CD4+ and CD8+ T cell count and high mobility group box (HMGB) -1 levels, as expression of the innate immune system. The potential link between FLCs levels and mortality risk was assessed. Method 190 patients on chronic hemodialysis at the Nephrology and Dialysis Unit of Papardo Hospital in Messina, Italy, were enrolled and followed for 2 years. Inclusion criteria were: age &gt;18 years, absence or &lt;200 ml/die residual diuresis, κ/λ ratio within the renal reference range (0.37–3.1). Receiver operating characteristics (ROC) analysis was performed to estimate the cut-off points of HMGB-1 and cFLC. Kaplan-Meier survival analysis and Cox proportional multivariate hazards model were used for clinical outcome. Results HD patients were characterized by high FLC levels. κFLC values were 182.3 (IQR: 140.2 – 216.1) mg/L, whereas λFLC levels were 108.2 (IQR: 72.7 – 143.2) mg/L. The median combined (c) FLC concentration was 182.9 mg/L (IQR = 207.8 – 330.2), which was extremely greater than the median reported in the general population (normal range = 9.3 – 43.3 mg/L) and in CKD patients [68.9 mg/L (IQR = 49.4 – 100.9)]. No differences in cFLC levels were revealed according to dialysis techniques. HD patients showed significant reduction of CD4+ and CD4+/CD8+ ratio. High HMGB1 levels were detected in HD patients (161.3 ± 39.7 ng/ml) and positively related to PCT and cFLC (r = - 0.38; p &lt; 0.001), with an inverse relation to CD4+/CD8+ ratio. cFLC positively correlated with β2 microglobulin, hemoglobin, and HMGB1. Conversely, an inverse correlation was revealed with surrogate markers of inflammation, such as CRP, procalcitonin, neutrophil counts. There were 49 deaths during the follow-up. The majority (23/49) of deaths were attributed to cardiovascular disease, the remainder to infection and malignancy. cFLCs and sHMGB-1 levels in this group were significantly elevated. By ROC analysis, HMGB-1 levels &gt; 100.9 ng/mL and cFLC &gt; 223.4 mg/l were associated with a significantly lower survival rate (p &lt; 0.02 by log-rank test) than for patients with lower levels when using Kaplan-Meier analysis. After adjusting for confounding factors, by Cox proportional hazards method, the difference remained statistically significant (p = 0.02) Conclusion Our study demonstrated an independent relation between high cFLC levels and mortality in HD patients. cFLCs represent a potential biomarker of “inflammunity”, a physiopathological process playing a pivotal role in ESRD, based on a vicious circle between inflammation and immune dysfunction. Further in-depth examinations should be verify our findings, determining whether therapeutic measures targeting cFLC balance, such as hemodiafiltration and expanded dialysis, would be helpful to reduce the “inflammunity” process, characterizing dialyzed patients.


2016 ◽  
Vol 56 (13) ◽  
pp. 1185-1192 ◽  
Author(s):  
Elizabeth M. Keating ◽  
Ryan M. Antiel ◽  
Karen E. Weiss ◽  
Dustin Wallace ◽  
Seth J. Antiel ◽  
...  

Adolescents with postural orthostatic tachycardia syndrome (POTS) often have pain and functional impairment. This study evaluated how parental attributions of children’s symptoms relate to child functional impairment. Adolescents with chronic pain and clinical symptoms suggestive of autonomic dysfunction (fatigue, dizziness, nausea) that attended a multidisciplinary chronic pain clinic completed measures of depression, anxiety, and functioning (n = 141). Parents of 114 of these patients completed the Parent Pain Attribution Questionnaire (PPAQ), a measure indicating the extent they believe physical and psychosocial factors account for their child’s health condition. Patients were retrospectively grouped as to whether or not they had significant POTS on tilt table testing (n = 37). Greater parental attribution to physical causes was associated with increased levels of functional disability whether patients had POTS ( r = 0.45, P = .006) or not ( r = 0.25, P = .03). These results suggest that providers should advocate a more comprehensive family-oriented rehabilitative approach to treatment.


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