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Saudi Journal of Anaesthesia 2024Malignant hyperthermia (MH), a rare inherited condition seen almost exclusively in the perioperative setting, is triggered by volatile anesthetics or an intravenous...
BACKGROUND
Malignant hyperthermia (MH), a rare inherited condition seen almost exclusively in the perioperative setting, is triggered by volatile anesthetics or an intravenous paralytic drug, succinylcholine. It can, however, occur without any exposure to anesthetic drugs, being associated with heat illness and rhabdomyolysis, thus presenting a little-known risk to young athletes exercising in hot environments.
OBJECTIVE
This study aimed to determine the first responder awareness of MH and its association with heat illness in young athletes within athletic and clinical environments.
METHODS
Awareness within the clinical milieu was assessed by an institutional chart review of 3296 charts. The identified heat illness cases were examined for treatment consistent with the management of a suspected episode of MH. Awareness among first responders in an athletic setting was examined by a survey administered to a total of 1,500 coaches and athletic trainers at the high school level along with emergency medical services providers across the United States.
RESULTS
No treatment consistent with the suspicion of MH was noted among clinical first responders, suggesting a lack of awareness. Survey administration also revealed a limited amount of knowledge of MH and its potential role in heat illness.
CONCLUSION
The results point to lack of awareness among pre-hospital and hospital-based first responders of the relationship between MH and heat illness in young athletes. An effort to educate these members of the healthcare community can contribute to an expeditious and life-saving intervention.
CLINICAL RELEVANCE
First responders who may interact with a young athlete have low knowledge of MH and its relationship to heat illness. Similar lack of awareness exists among hospital personnel who care for young individuals with heat illness. Educating the first responders about this condition can speed up the time to intervene and save lives.
PubMed: 38654868
DOI: 10.4103/sja.sja_858_23 -
The Journal of International Medical... Jun 2024Malignant hyperthermia (MH), characterized by severe myoclonus, pyrexia, tachycardia, hypertension, elevated muscle enzymes, and hypercapnia, often occurs in patients...
Malignant hyperthermia (MH), characterized by severe myoclonus, pyrexia, tachycardia, hypertension, elevated muscle enzymes, and hypercapnia, often occurs in patients with congenital deformities or genetic disorders. Although the reported incidence rate is as low as 1:5000 to 1:100,000, patients with MH exhibit rapid aggravation and an elevated mortality rate. Thus, MH is associated with substantial perioperative risk. Successful treatment of patients with MH largely depends on early diagnosis and timely effective treatment. This clinical report provides a detailed description of a patient with newly diagnosed MH who developed a rapid rise in body temperature, end-tidal carbon dioxide, and heart rate during maxillary osteotomy. After successful rescue, the patient recovered smoothly during the postoperative period, indicating the importance of intraoperative monitoring, early diagnosis, effective treatment, and postoperative monitoring. This case is expected to serve as a reference for future interventions and healthcare practices in managing other patients with MH.
Topics: Humans; Malignant Hyperthermia; Anesthesia, General; Male; Female; Adult; Osteotomy
PubMed: 38907364
DOI: 10.1177/03000605241261962 -
European Radiology Experimental Nov 2023Malignant tumors routinely present with irregular shapes and complex configurations. The lack of customization to individual tumor shapes and standardization of...
BACKGROUND
Malignant tumors routinely present with irregular shapes and complex configurations. The lack of customization to individual tumor shapes and standardization of procedures limits the success and application of thermal ablation.
METHODS
We introduced an automated treatment model consisting of (i) trajectory and ablation profile planning, (ii) ablation probe insertion, (iii) dynamic energy delivery (including robotically driven control of the energy source power and location over time, according to a treatment plan bespoke to the tumor shape), and (iv) quantitative ablation margin verification. We used a microwave ablation system and a liver phantom (acrylamide polymer with a thermochromic ink) to mimic coagulation and measure the ablation volume. We estimated the ablation width as a function of power and velocity following a probabilistic model. Four representative shapes of liver tumors < 5 cm were selected from two publicly available databases. The ablated specimens were cut along the ablation probe axis and photographed. The shape of the ablated volume was extracted using a color-based segmentation method.
RESULTS
The uncertainty (standard deviation) of the ablation width increased with increasing power by ± 0.03 mm (95% credible interval [0.02, 0.043]) per watt increase in power and by ± 0.85 mm (95% credible interval [0, 2.5]) per mm/s increase in velocity. Continuous ablation along a straight-line trajectory resulted in elongated rotationally symmetric ablation shapes. Simultaneous regulation of the power and/or translation velocity allowed to modulate the ablation width at specific locations.
CONCLUSIONS
This study offers the proof-of-principle of the dynamic energy delivery system using ablation shapes from clinical cases of malignant liver tumors.
RELEVANCE STATEMENT
The proposed automated treatment model could favor the customization and standardization of thermal ablation for complex tumor shapes.
KEY POINTS
• Current thermal ablation systems are limited to ellipsoidal or spherical shapes. • Dynamic energy delivery produces elongated rotationally symmetric ablation shapes with varying widths. • For complex tumor shapes, multiple customized ablation shapes could be combined.
Topics: Humans; Microwaves; Liver Neoplasms; Ablation Techniques; Models, Theoretical
PubMed: 37932631
DOI: 10.1186/s41747-023-00381-6 -
Brazilian Journal of Anesthesiology... 2023
Topics: Humans; Malignant Hyperthermia; Depression; Fatigue; Muscle Contraction
PubMed: 37532110
DOI: 10.1016/j.bjane.2023.07.009 -
Scientific Reports Jul 2023Initially diagnosed malignant pleural effusion (MPE) has different systematic treatments, and defining the best drainage regimen according to the responsiveness of MPE...
Initially diagnosed malignant pleural effusion (MPE) has different systematic treatments, and defining the best drainage regimen according to the responsiveness of MPE to different systematic treatments is important. This study compared the efficacy of hyperthermic intrathoracic chemotherapy (HITHOC) and pleural catheter drainage (IPCD) for initially diagnosed lung cancer with symptomatic MPE. We retrospectively reviewed the medical records of initially diagnosed lung cancer patients with symptomatic MPE between January 2018 and May 2022. The patients were treated with IPCD or HITHOC for local control of MPE after diagnosis. Systematic regimens were conducted during 1 month according to guidelines after local treatment. Intrathoracic MPE progression-free survival (iPFS) and overall survival (OS) were calculated, Univariate and multivariable Cox-regression were used to identify factors associated with iPFS and OS. A total of 33 patients were evaluated; 10 (30.3%) patients received IPCD, and 23 (69.7%) patients received HITHOC. No difference in the MPE control rate at 1 month was found between the IPCD group (90%) and HITHOC group (95.7%). However, this control rate was significantly higher in the HITHOC group (69.6%) than in the IPCD group (30%) at 3 months (P = 0.035). Multivariate analysis showed that receiving tyrosine kinase inhibitors (TKIs) or chemotherapy was a significant protective factor for iPFS (HR = 0.376, 95% CI 0.214-0.659, P = 0.007) and OS (HR = 0.321, 95% CI 0.174-0.594, P < 0.001). According to subgroup analysis, among patients treated with TKIs, those who received HITHOC had longer iPFS and OS than those who received IPCD (P = 0.011 and P = 0.002, respectively), but this difference was not found in the palliative care subgroup. Moreover, no patients treated with chemotherapy showed reaccumulation of MPE. Systematic TKIs or chemotherapy prolonged iPFS and OS for those initially diagnosed with lung cancer with symptomatic MPE. HITHOC prolonged iPFS and OS for those treated with systematic TKIs.
Topics: Humans; Pleural Effusion, Malignant; Retrospective Studies; Lung Neoplasms; Hyperthermia, Induced
PubMed: 37495659
DOI: 10.1038/s41598-023-39211-5 -
BMC Psychiatry Jun 2024Malignant hyperthermia is a potentially lethal condition triggered by specific anesthetic drugs, especially a depolarizing muscle relaxant of succinylcholine...
BACKGROUND
Malignant hyperthermia is a potentially lethal condition triggered by specific anesthetic drugs, especially a depolarizing muscle relaxant of succinylcholine (Suxamethonium). Despite the frequent use of succinylcholine with electroconvulsive therapy (ECT), there has been no reported case of potentially lethal malignant hyperthermia following ECT. In addition, the time interval between the administration of succinylcholine and the onset of malignant hyperthermia has not been outlined in the context of ECT.
CASE PRESENTATION
We present the case of a 79-year-old woman suffering from severe depression, who experienced severe malignant hyperthermia due to succinylcholine administration during an ECT session. She presented with a high fever of 40.2 °C, tachycardia of 140/min, hypertension with a blood pressure exceeding 200 mmHg, significant muscle rigidity, and impaired consciousness. These symptoms emerged two hours after ECT, which occurred in a psychiatric ward rather than an operating room, and reached their peak in less than 24 h. She was given 60 mg of dantrolene, which quickly reduced the muscular rigidity. Subsequently, she received two additional doses of 20 mg and 60 mg of dantrolene, which brought her fever down to 36.2 °C and completely eased her muscle rigidity within two days after ECT.
CONCLUSIONS
This is the first reported case of potentially lethal malignant hyperthermia after ECT. In addition, it highlights the delayed onset of malignant hyperthermia following an ECT procedure, emphasizing the necessity for psychiatrists to recognize its onset even after the treatment. In the light of potentially lethal consequences of malignant hyperthermia, it is critically important for psychiatrists to closely monitor both intraoperative and postoperative patient's vital signs and characteristic physical presentations, promptly identify any symptomatic emergence, and treat it immediately with dantrolene.
Topics: Humans; Succinylcholine; Electroconvulsive Therapy; Aged; Malignant Hyperthermia; Female; Neuromuscular Depolarizing Agents; Dantrolene; Psychiatrists
PubMed: 38834964
DOI: 10.1186/s12888-024-05846-5 -
Frontiers in Neurology 2023Andersen-Tawil syndrome (ATS) is a rare periodic paralysis caused by the KCNJ2 gene mutation. Here, we report on an ATS patient misdiagnosed with myodystrophy. A...
Andersen-Tawil syndrome (ATS) is a rare periodic paralysis caused by the KCNJ2 gene mutation. Here, we report on an ATS patient misdiagnosed with myodystrophy. A 66-year-old man presented with a 60-year history of episodic weakness in the proximal muscles of the upper and lower limbs. The man has been diagnosed with muscle pathology and has undergone genetic examinations in many hospitals since childhood. We conducted a correct diagnosis in combination with the patient's history, electrical physiology, and genetic analysis and identified a heterozygous KCNJ2 gene variant (c.220A > G; p.T74A). Patients with ATS can develop permanent myasthenia characterized by chronic progressive myopathy. ATS patients should also pay special attention to the risks of anesthesia in surgery, including malignant hyperthermia (MH), muscle spasms affecting tracheal intubation or ventilation, and ventilator weakness. Early diagnosis and therapy could help delay the onset of myasthenia and prevent risks associated with anesthesia accidents.
PubMed: 37456645
DOI: 10.3389/fneur.2023.1170693 -
BMC Pediatrics Oct 2023The CACNA1S gene encodes the alpha 1 S-subunit of the voltage-gated calcium channel, which is primarily expressed in the skeletal muscle cells. Pathogenic variants of... (Review)
Review
BACKGROUND
The CACNA1S gene encodes the alpha 1 S-subunit of the voltage-gated calcium channel, which is primarily expressed in the skeletal muscle cells. Pathogenic variants of CACNA1S can cause hypokalemic periodic paralysis (HypoPP), malignant hyperthermia susceptibility, and congenital myopathy. We aimed to study the clinical and molecular features of a male child with a CACNA1S variant and depict the molecular sub-regional characteristics of different phenotypes associated with CACNA1S variants.
CASE PRESENTATION
We presented a case of HypoPP with recurrent muscle weakness and hypokalemia. Genetic analyses of the family members revealed that the proband had a novel c.497 C > A (p.Ala166Asp) variant of CACNA1S, which was inherited from his father. The diagnosis of HypoPP was established in the proband as he met the consensus diagnostic criteria. The patient and his parents were informed to avoid the classical triggers of HypoPP. The attacks of the patient are prevented by lifestyle changes and nutritional counseling. We also showed the molecular sub-regional location of the variants of CACNA1S which was associated with different phenotypes.
CONCLUSIONS
Our results identified a new variant of CACNA1S and expanded the spectrum of variants associated with HypoPP. Early genetic diagnosis can help avoid diagnostic delays, perform genetic counseling, provide proper treatment, and reduce morbidity and mortality.
Topics: Humans; Male; Child; Hypokalemic Periodic Paralysis; Mutation; Phenotype; Muscle Weakness; Family; Calcium Channels, L-Type
PubMed: 37784084
DOI: 10.1186/s12887-023-04326-1 -
BMC Cancer Aug 2023Glioblastoma (GBM) is the most common primary, malignant brain tumour with a 5-year survival of 5%. If possible, a glioblastoma is resected and further treated with...
Study protocol for a multicenter randomised controlled trial on the (cost)effectiveness of biopsy combined with same-session MR-guided LITT versus biopsy alone in patients with primary irresectable glioblastoma (EMITT trial).
BACKGROUND
Glioblastoma (GBM) is the most common primary, malignant brain tumour with a 5-year survival of 5%. If possible, a glioblastoma is resected and further treated with chemoradiation therapy (CRT), but resection is not feasible in about 30% of cases. Current standard of care in these cases is a biopsy followed by CRT. Magnetic resonance (MR) imaging-guided laser interstitial thermal therapy (LITT) has been suggested as a minimally invasive alternative when surgery is not feasible. However, high-quality evidence directly comparing LITT with standard of care is lacking, precluding any conclusions on (cost-)effectiveness. We therefore propose a multicenter randomized controlled study to assess the (cost-)effectiveness of MR-guided LITT as compared to current standard of care (EMITT trial).
METHODS AND ANALYSIS
The EMITT trial will be a multicenter pragmatic randomized controlled trial in the Netherlands. Seven Dutch hospitals will participate in this study. In total 238 patients will be randomized with 1:1 allocation to receive either biopsy combined with same-session MR-guided LITT therapy followed by CRT or the current standard of care being biopsy followed by CRT. The primary outcomes will be health-related quality of life (HR-QoL) (non-inferiority) using EORTC QLQ-C30 + BN20 scores at 5 months after randomization and overall survival (superiority). Secondary outcomes comprise cost-effectiveness (healthcare and societal perspective) and HR-QoL of life over an 18-month time horizon, progression free survival, tumour response, disease specific survival, longitudinal effects, effects on adjuvant treatment, ablation percentage and complication rates.
DISCUSSION
The EMITT trial will be the first RCT on the effectiveness of LITT in patients with glioblastoma as compared with current standard of care. Together with the Dutch Brain Tumour Patient association, we hypothesize that LITT may improve overall survival without substantially affecting patients' quality of life.
TRIAL REGISTRATION
This trial is registered at ClinicalTrials.gov (NCT05318612).
Topics: Humans; Quality of Life; Glioblastoma; Biopsy; Hyperthermia, Induced; Adjuvants, Immunologic; Randomized Controlled Trials as Topic; Multicenter Studies as Topic
PubMed: 37612610
DOI: 10.1186/s12885-023-11282-7