-
Andes Pediatrica : Revista Chilena de... Apr 2021Juvenile myasthenia gravis (JMG) is an autoimmune disease affecting the neuromuscular junction that appears before 19 years of age with varying degrees of weakness of...
INTRODUCTION
Juvenile myasthenia gravis (JMG) is an autoimmune disease affecting the neuromuscular junction that appears before 19 years of age with varying degrees of weakness of different muscle groups. The main treatment is pharmacological, but thymectomy has also demonstrated to improve remission rates.
OBJECTIVE
To describe the clinical characteristics and postoperative course of pediatric patients with JMG who underwent video-assisted thoracoscopic (VATS) thymectomy. Clinical Serie: Six pa tients who underwent VATS thymectomy between March 2011 and June 2019. The age range at diag nosis was between 2 and 14 years and the average age at surgery was 7 years. All patients were under treatment with pyridostigmine bromide associated with immunosuppression with corticosteroids before surgery. The interval between diagnosis and thymectomy was 21.5 months on average. VATS was performed by left approach, and there was no perioperative morbidity or mortality. The average hospital stay was 2 days. Three patients remain with no symptoms and without corticotherapy. Two patients were on corticosteroids, but in smaller doses than previous to surgery. One patient presented a crisis requiring hospitalization and ventilatory support during follow-up.
CONCLUSION
VATS thy mectomy is part of the treatment for JMG. In this series, it appears as a safe approach and its results were favorable.
Topics: Adolescent; Adrenal Cortex Hormones; Child; Child, Preschool; Cholinesterase Inhibitors; Female; Humans; Length of Stay; Male; Myasthenia Gravis; Postoperative Period; Pyridostigmine Bromide; Thoracic Surgery, Video-Assisted; Thymectomy; Treatment Outcome
PubMed: 34106164
DOI: 10.32641/andespediatr.v92i2.2955 -
BMJ Case Reports Dec 2019A 24-year-old otherwise healthy male presented to us with unilateral ptosis and contralateral lid retraction with limitation of extraocular movements; the disease had a...
A 24-year-old otherwise healthy male presented to us with unilateral ptosis and contralateral lid retraction with limitation of extraocular movements; the disease had a gradual chronic course, which raised a suspicion of ocular myasthenia. Ice pack test was performed, which improved the ptosis; further investigations confirmed the diagnosis of ocular myasthenia. Patient was started on pyridostigmine and oral prednisolone which improved the extraocular movements and ptosis.
Topics: Blepharoptosis; Cholinesterase Inhibitors; Cold Temperature; Diagnosis, Differential; Diagnostic Techniques, Ophthalmological; Eye Diseases; Eye Movements; Follow-Up Studies; Glucocorticoids; Humans; Male; Myasthenia Gravis; Prednisolone; Pyridostigmine Bromide; Treatment Outcome; Young Adult
PubMed: 31818887
DOI: 10.1136/bcr-2019-231296 -
Turkish Journal of Medical Sciences Aug 2020Sugammadex, which offsets the effects of neuromuscular blocking agents (NMBs), has advantages over traditional reversal agents like pyridostigmine, as it enables fast...
BACKGROUND/AIM
Sugammadex, which offsets the effects of neuromuscular blocking agents (NMBs), has advantages over traditional reversal agents like pyridostigmine, as it enables fast and reliable recovery from neuromuscular blockade. This study compared the incidence of early postoperative chest radiographic abnormalities (CRA) between sugammadex (group S) and pyridostigmine (group P) following video-assisted thoracoscopic (VAT) lobectomy for lung cancer.
MATERIALS AND METHODS
We performed a retrospective cohort analysis by reviewing the medical records of patients who underwent VAT lobectomy at a single university medical center. We defined the early postoperative CRA as a characteristic appearance on chest radiograph up to 2 days after surgery. Arterial blood gas analysis (ABGA), surgical time, anaesthesia time, extubation time, and the total dose of rocuronium were analysed. Postoperative nausea and vomiting (PONV) and pain scores were observed until 2 days after surgery.
RESULTS
A total of 257 patients underwent VAT lobectomy during the study period; 159 were included in the final analysis. Ninety patients received sugammadex while 69 received pyridostigmine. The incidence of early postoperative atelectasis was significantly lower in group S than in group P (26.7%, 95% CI: 17.5%‒35.8% and 43.5%, 95% CI: 31.8%‒55.2%, respectively, P = 0.013). The median dose of rocuronium was higher in group S than in group P (120 mg vs. 90 mg, P < 0.001). ABGA, extubation time, and PONV were similar in both groups.
CONCLUSION
Sugammadex decreased the incidence of CRA in the early postoperative period despite higher NMB consumption.
Topics: Aged; Cholinesterase Inhibitors; Female; Humans; Lung; Lung Neoplasms; Male; Middle Aged; Neuromuscular Nondepolarizing Agents; Pneumonectomy; Postoperative Complications; Pulmonary Atelectasis; Pyridostigmine Bromide; Radiography, Thoracic; Retrospective Studies; Sugammadex; Thoracic Surgery, Video-Assisted
PubMed: 32366060
DOI: 10.3906/sag-2001-26 -
Exposure to Gulf war illness-related chemicals exacerbates alcohol- induced liver damage in rodents.Research Square Jan 2024Gulf War Illness (GWI) describes a series of symptoms suffered by veterans of the Gulf war consisting of cognitive, neurological and gastrointestinal dysfunctions. Two...
Gulf War Illness (GWI) describes a series of symptoms suffered by veterans of the Gulf war consisting of cognitive, neurological and gastrointestinal dysfunctions. Two chemicals associated with GWI are the insecticide permethrin (PER) and the nerve gas prophylactic pyridostigmine-bromide (PB). In this study we assessed the effects of PER and PB exposure on pathology and subsequent alcohol (EtOH)-induced liver injury, and the influence of a macrophage depletor, PLX3397, on EtOH-induced liver damage in PER/PB- treated mice. Male C57BL/6 mice were injected daily with vehicle or PER/PB for 10 days, followed by 4 months recovery, then treatment with PLX3397 and a chronic-plus-single-binge EtOH challenge for 10 days. PER/PB exposure resulted in the protracted increase in liver transaminases in the serum and induced chronic low-level microvesicular steatosis and inflammation in GWI vs Naïve mice up to 4 months after cessation of exposure. Furthermore, prior exposure to PER/PB also resulted in exacerbated response to EtOH-induced liver injury, with enhanced steatosis, ductular reaction and fibrosis. The enhanced EtOH-induced liver damage in GWI-mice was attenuated by strategies designed to deplete macrophages in the liver. Taken together, these data suggest that exposure to GWI-related chemicals may alter the liver's response to subsequent ethanol exposure.
PubMed: 38313276
DOI: 10.21203/rs.3.rs-3838282/v1 -
International Journal of Environmental... Sep 2020The microbiota's influence on host (patho) physiology has gained interest in the context of Gulf War Illness (GWI), a chronic disorder featuring dysregulation of the...
The microbiota's influence on host (patho) physiology has gained interest in the context of Gulf War Illness (GWI), a chronic disorder featuring dysregulation of the gut-brain-immune axis. This study examined short- and long-term effects of GWI-related chemicals on gut health and fecal microbiota and the potential benefits of Lacto-N-fucopentaose-III (LNFPIII) treatment in a GWI model. Male C57BL/6J mice were administered pyridostigmine bromide (PB; 0.7 mg/kg) and permethrin (PM; 200 mg/kg) for 10 days with concurrent LNFPIII treatment (35 μg/mouse) in a short-term study (12 days total) and delayed LNFPIII treatment (2×/week) beginning 4 months after 10 days of PB/PM exposure in a long-term study (9 months total). Fecal 16S rRNA sequencing was performed on all samples post-LNFPIII treatment to assess microbiota effects of GWI chemicals and acute/delayed LNFPIII administration. Although PB/PM did not affect species composition on a global scale, it affected specific taxa in both short- and long-term settings. PB/PM elicited more prominent long-term effects, notably, on the abundances of bacteria belonging to and families and the genus . LNFPIII improved a marker of gut health (i.e., decreased lipocalin-2) independent of GWI and, importantly, increased butyrate producers (e.g., , ) in PB/PM-treated mice, indicating a positive selection pressure for these bacteria. Multiple operational taxonomic units correlated with aberrant behavior and lipocalin-2 in PB/PM samples; LNFPIII was modulatory. Overall, significant and lasting GWI effects occurred on specific microbiota and LNFPIII treatment was beneficial.
Topics: Amino Sugars; Animals; Gastrointestinal Microbiome; Gulf War; Male; Mice; Mice, Inbred C57BL; Persian Gulf Syndrome; Polysaccharides; RNA, Ribosomal, 16S
PubMed: 32992640
DOI: 10.3390/ijerph17197081 -
International Journal of Environmental... Jan 2022mutations lead to complex neurodevelopmental syndromes, including infantile hypotonia with psychomotor retardation and characteristic facies (IHPRF) and congenital...
mutations lead to complex neurodevelopmental syndromes, including infantile hypotonia with psychomotor retardation and characteristic facies (IHPRF) and congenital contractures of limbs and face, hypotonia, and developmental delay (CLIFAHDD), which are recessively and dominantly inherited, respectively. We present a patient in whom congenital myasthenic syndrome (CMS) was suspected due to the occurrence of hypotonia and apnea episodes requiring resuscitation. For this reason, treatment with pyridostigmine was introduced. After starting the treatment, a significant improvement was observed in reducing the apnea episodes and slight psychomotor progress. In the course of further diagnostics, CMS was excluded, and CLIFAHDD syndrome was confirmed. Thus, we try to explain a possible mechanism of clinical improvement after the introduction of treatment with pyridostigmine in a patient with a mutation in the gene.
Topics: Contracture; Humans; Membrane Proteins; Muscle Hypotonia; Mutation; Pyridostigmine Bromide; Sleep Apnea, Central; Syndrome
PubMed: 35055596
DOI: 10.3390/ijerph19020775 -
BMC Neurology Nov 2022Spinal and bulbar muscular atrophy (SBMA) is an X-linked recessive hereditary neuromuscular disorder caused by the expanded trinucleotide repeat in the androgen...
BACKGROUND
Spinal and bulbar muscular atrophy (SBMA) is an X-linked recessive hereditary neuromuscular disorder caused by the expanded trinucleotide repeat in the androgen receptors gene. The major clinical manifestations of SBMA consist of weakness in the bulbar and limb muscles, fasciculations, tremors, cramps, sensory impairment, and gynecomastia. However, atypical SBMA cases may lead to misdiagnosis. Muscular fatigue and decremental responses to repetitive nerve stimulation (RNS), despite being observed in some SBMA patients, are usually occurred in MG patients, and patient with the symptom of mastication fatigue was rarely reported. In addition, cardiological investigations have been performed in SBMA patients and several ECG alterations were identified. Here we report an SBMA patient presenting with a rare onset symptom of mastication fatigue, who has been detected with a positive titin antibody in the serum and showed a WPW pattern electrocardiogram.
CASE PRESENTATION
The patient showed mildly progressive fatigue in the muscles of mastication over 3 years. Neurological examination showed facial muscle weakness and a wasting tongue with fasciculations, but the weakness, wasting, or fasciculations were not obvious in the limbs. 3-Hz RNS showed a decremental response in bilateral orbicularis oculi. The test of titin antibody was positive in the serum, and the electrocardiogram showed a WPW pattern ECG. Genetic analysis revealed an increased number (39 repeats) of tandem CAG repeats in the AR gene, which confirmed the diagnosis of SBMA. The fatigue symptom was significantly improved after oral pyridostigmine bromide treatment.
CONCLUSION
This case calls for more attention to muscular fatigue as the onset symptoms of Kennedy's disease. ECG screening is of importance in SBMA patients and further studies are needed to investigate the titin antibody in SBMA patients as well as other neurogenic disorders.
Topics: Humans; Male; Bulbo-Spinal Atrophy, X-Linked; Connectin; Mastication; Fasciculation; Muscular Atrophy, Spinal; Fatigue
PubMed: 36376797
DOI: 10.1186/s12883-022-02971-0 -
Boletin Medico Del Hospital Infantil de... 2024This work aimed to show which treatments showed efficacy against coronavirus disease 2019 (COVID-19); therefore, the results of 37 clinical trials started in 2020 and...
This work aimed to show which treatments showed efficacy against coronavirus disease 2019 (COVID-19); therefore, the results of 37 clinical trials started in 2020 and completed in 2021 are reviewed and discussed here. These were selected from databases, excluding vaccines, computational studies, in silico, in vitro, and those with hyperimmune sera from recovered patients. We found 34 drugs, one vitamin, and one herbal remedy with pharmacological activity against symptomatic COVID-19. They reduced mortality, disease progression, or recovery time. For each treatment, the identifier and type of trial, the severity of the disease, the sponsor, the country where the trial was conducted, and the trial results are presented. The drugs were classified according to their mechanism of action. Several drugs that reduced mortality also reduced inflammation in the most severe cases. These include some that are not considered anti-inflammatory, such as Aviptadil, pyridostigmine bromide, anakinra, imatinib, baricitinib, and bevacizumab, as well as the combination of ivermectin, aspirin, dexamethasone, and enoxaparin. Nigella sativa seeds with honey have also been reported to have therapeutic activity. On the other hand, tofacitinib, novaferon with ritonavir, and lopinavir were also effective, as well as in combination with antiviral therapies such as danoprevir with ritonavir. The natural products colchicine and Vitamin D3 were only effective in patients with mild-to-moderate COVID-19, as was hydroxychloroquine. Drug repositioning has been the main tool in the search for effective therapies by expanding the pharmacological options available to patients.
Topics: Humans; COVID-19; Ritonavir; Antiviral Agents; SARS-CoV-2; Biological Products; Pandemics
PubMed: 38503318
DOI: 10.24875/BMHIM.23000016 -
Brain, Behavior, & Immunity - Health Dec 2022Chemical overexposures and war-related stress during the 1990-1991 Gulf War (GW) are implicated in the persisting pathological symptoms that many GW veterans continue to...
Chemical overexposures and war-related stress during the 1990-1991 Gulf War (GW) are implicated in the persisting pathological symptoms that many GW veterans continue to endure. These symptoms culminate into a disease known as Gulf War Illness (GWI) and affect about a third of the GW veteran population. Currently, comprehensive effective GWI treatment options are unavailable. Here, an established GWI mouse model was utilized to explore the (1) long-term behavioral and neuroinflammatory effects of deployment-related GWI chemicals exposure and (2) ability of the immunotherapeutic lacto-N-fucopentaose III (LNFPIII) to improve deficits when given months after the end of exposure. Male C57BL6/J mice (8-9 weeks old) were administered pyridostigmine bromide (PB) and DEET for 14 days along with corticosterone (CORT; latter 7 days) to emulate wartime stress. On day 15, a single injection of the nerve agent surrogate diisopropylfluorophosphate (DFP) was given. LNFPIII treatment began 7 months post GWI chemicals exposure and continued until study completion. A battery of behavioral tests for assessment of cognition/memory, mood, and motor function in rodents was performed beginning 8 months after exposure termination and was then followed by immunohistochemcal evaluation of neuroinflammation and neurogenesis. Within tests of motor function, prior GWI chemical exposure led to hyperactivity, impaired sensorimotor function, and altered gait. LNFPIII attenuated these motor-related deficits and improved overall grip strength. GWI mice also exhibited more anxiety-like behavior that was reduced by LNFPIII; this was test-specific. Short-term, but not long-term memory, was impaired by prior GWI exposure; LNFPIII improved this measure. In the brains of GWI mice, but not in mice treated with LNFPIII, glial activation was increased. Overall, it appears that months after exposure to GWI chemicals, behavioral deficits and neuroinflammation are present. Many of these deficits were attenuated by LNFPIII when treatment began long after GWI chemical exposure termination, highlighting its therapeutic potential for veterans with GWI.
PubMed: 36405424
DOI: 10.1016/j.bbih.2022.100553 -
Computational and Structural... 2022Gulf War Illness (GWI) is a chronic illness that affects upward of 32% of deployed Veterans to the 1991 Gulf War (GW). The symptoms are medically unexplained, ranging...
Gulf War Illness (GWI) is a chronic illness that affects upward of 32% of deployed Veterans to the 1991 Gulf War (GW). The symptoms are medically unexplained, ranging across cognitive deficits, fatigue, gastrointestinal problems, and musculoskeletal pain. Research indicates that chemical warfare agents play a key role in the onset and progression of GWI. The Khamisiyah ammunition storage that housed chemical warfare agents such as sarin, an acetylcholinesterase (AChE) inhibitor, was demolished during the GW, releasing toxicants into the atmosphere affecting deployed troops. Exposure to other chemical agents such as pyridostigmine bromide, N,N-diethyl-m-toluamide, permethrin and chlorpyrifos, were also prevalent during the war. These additional chemical agents have also been shown to inhibit AChE. AChE inhibition induces an acetylcholine build-up, disrupting signals between nerves and muscles, which in high doses leads to asphyxiation. Little is known about low dose exposure. As bioactive compounds tend to interact with multiple proteins with various physiological effect, we aimed to identify other potential shared targets to understand the extent in which these chemicals could lead to GWI. We followed a reverse screening approach where each chemical is computationally docked to a library of protein targets. The programs PharmMapper and TargetNet were used for this purpose, and further analyses were conducted to mark significant changes in participants with GWI. Previously published work on DNA methylation status in GWI was reanalyzed focusing specifically on the predicted shared targets indicating significant changes in DNA methylation of the associated genes. Our findings thus suggest that exposure to GWI-related agents may converge on similar targets with roles in inflammation, neurotransmitter and lipid metabolism, and detoxification which may have impacts on neurodegenerative-like disease and oxidative stress in Veterans with GWI.
PubMed: 36420170
DOI: 10.1016/j.csbj.2022.11.006