Neuromuscular conditions associated with malignant hyperthermia in paediatric patients: A 25-year retrospective study

Bamaga, Ahmed K.; Riazi, Sheila; Amburgey, Kimberly; Ong, Shaun; Halliday, William; Diamandis, Phedias; Guerguerian, Anne‐Marie; Dowling, James J.; Yoon, Grace

doi:10.1016/j.nmd.2016.02.007

Cited by 27 publications

(13 citation statements)

References 24 publications

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“…Malignant hyperthermia is a rare genetic disorder (1 in 14,000 pediatric general anesthesia) associated with several forms of congenital myopathy and triggered by succinylcholine or inhalational anesthetics agents; clinical features include rapid onset of extremely high temperature (38.5–46 °C), usually heralded by masseters spasm, muscle rigidity, metabolic acidosis, and hemodynamic collapse. Specific treatment, with discontinuation of involved anesthetics, muscular relaxation with sodium dantrolene, and correction of metabolic acidosis, has dramatically reduced the mortality, once as high as 70%, to less than 5% [ 36 , 37 ].…”

Section: Increased Body Temperature As a Diagnostic Signmentioning

confidence: 99%

Fever in Children: Pearls and Pitfalls

et al. 2017

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Fever in children is a common concern for parents and one of the most frequent presenting complaints in emergency department visits, often involving non-pediatric emergency physicians. Although the incidence of serious infections has decreased after the introduction of conjugate vaccines, fever remains a major cause of laboratory investigation and hospital admissions. Furthermore, antipyretics are the most common medications administered to children. We review the epidemiology and measurement of fever, the meaning of fever and associated clinical signs in children of different ages and under special conditions, including fever in children with cognitive impairment, recurrent fevers, and fever of unknown origin. While the majority of febrile children have mild, self-resolving viral illness, a minority may be at risk of life-threatening infections. Clinical assessment differs markedly from adult patients. Hands-off evaluation is paramount for a correct evaluation of breathing, circulation and level of interaction. Laboratory markers and clinical prediction rules provide limited help in identifying children at risk for serious infections; however, clinical examination, prudent utilization of laboratory tests, and post-discharge guidance (“safety netting”) remain the cornerstone of safe management of febrile children.

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Section: Increased Body Temperature As a Diagnostic Signmentioning

confidence: 99%

Fever in Children: Pearls and Pitfalls

et al. 2017

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“…All clinical and laboratory findings found in the patient are part of the diagnostic criteria approved by Projeto Diatrizes, a joint initiative of the Brazilian Medical Association (Associação Médica Brasileira -AMB) and the Federal Council of Medicine (Conselho Federal de Medicina -CFM), which aims to reconcile information from the medical field, in order to standardize conduct that help doctors reason and make decisions. 9,11 Most of the time, the manifestations of malignant hyperthermia occur suddenly and early on. The syndrome can be fatal when not treated properly, leading the patient to have intense muscle catabolism and its serious consequences, especially at the respiratory and cardiovascular level.…”

Section: Discussionmentioning

confidence: 99%

“…1,4,9 There are also reports that susceptibility to MH increases in patients with certain congenital myopathies due to their association with mutation in the RYR1 gene. Medical professionals' prior knowledge of this information is essential in order to optimize the clinical care of these patients when they need surgery, as proposed by Bamaga et al 1,3,11 In order to assist health professionals and victims' families, a service for MH was created in Brazil in 1991 called Hotline (+ 55-11-55759873). It is located in the city of São Paulo, specifically at the Hospital São Paulo, which is part of Unifesp's Paulista School of Medicine (Escola Paulista de Medicina -EPM).…”

Section: Discussionmentioning

confidence: 99%

Malignant Hyperthermia in a Child After Magnetic Resonance Imaging: A Case Report

Almeida¹,

Colleti

2020

Rev. paul. pediatr.

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Objective: To report on a case of malignant hyperthermia in a child after a magnetic resonance imaging of the skull was performed using sevoflurane anesthesia. Case description: A 3-year-old boy admitted to the pediatric intensive care unit after presenting clinical and laboratory findings consistent with unspecified viral meningoencephalitis. While the patient was sedated, a magnetic resonance imaging of the skul was performed using propofol followed by the administration of sevoflurane through a laryngeal mask in order to continue anesthesia. Approximately three hours after the start of the procedure, the patient presented persistent tachycardia, tachypnea, generalized muscular stiffness and hyperthermia. With a diagnostic hypothesis of malignant hyperthermia, dantrolene was then administered, which immediately induced muscle stiffness, tachycardia, tachypnea and reduced body temperature. Comments: Malignant hyperthermia is a rare pharmacogenetic syndrome characterized by a severe hypermetabolic reaction after the administration of halogenated inhalational anesthetics or depolarizing muscle relaxants such as succinylcholine, or both. Although it is a potentially fatal disease, the rapid administration of continued doses dantrolene has drastically reduced the morbidity and mortality of the disease.

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“…MH is a rare and potentially lethal pharmacogenic disorder of skeletal muscle regulation [4], [5]. Most cases are triggered by administration of volatile anesthetics or depolarizing muscle relaxants such as succinylcholine 6 .…”

Section: Discussionmentioning

confidence: 99%

“…The majority of reported cases are caused by mutations in two genes: RYR1 gene (ryanodine receptor type 1) and CACNA1S gene (voltage-dependent L type calcium channel alpha 1S subunit) which are estimated to account for approximately 70% and 1% of MH susceptibility, respectively. These two genes are related to skeletal muscle calcium regulation 5 . Recently, mutations in STAC3 have been identified in five families with Native American Myopathy, associated with increased MH susceptibility [5], [18]…”

Section: Discussionmentioning

confidence: 99%

Malignant hyperthermia in a 4-year-old girl during anesthesia induction with sevoflurane and succinylcholine for congenital ptosis surgery

Ferhi

Dardour

Tej

et al. 2019

Saudi Journal of Ophthalmology

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Malignant hyperthermia (MH) is a rare pharmacogenic disorder of skeletal muscle calcium regulation, resulting from general anesthesia that can be fatal. Most cases are caused by administration of volatile anesthetics or depolarizing muscle relaxants. It has been generally reported that both of sevoflurane and succinylcholine can induce the delayed onset of MH. Here, we report a case of malignant hyperthermia in a four-year-old girl during anesthesia induction for unilateral congenital ptosis surgery, two minutes after sevoflurane and succinylcholine administration. The crisis was atypical but early recognized and managed by administration of dantrolene with symptomatic treatment.

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Neuromuscular conditions associated with malignant hyperthermia in paediatric patients: A 25-year retrospective study

Cited by 27 publications

References 24 publications

Fever in Children: Pearls and Pitfalls

Fever in Children: Pearls and Pitfalls

Malignant Hyperthermia in a Child After Magnetic Resonance Imaging: A Case Report

Malignant hyperthermia in a 4-year-old girl during anesthesia induction with sevoflurane and succinylcholine for congenital ptosis surgery

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