viernes, 2 de abril de 2010

hipertermia maligna

----- Mensaje reenviado ----

De: Victor Whizar-Lugo <>
Enviado: vie,2 abril, 2010 11:53
Asunto: Hipertermia maligna


Anestesiología y Medicina del Dolor

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Anestesiología y Medicina del Dolor

No:09001 Abril 2010

Estimad@ Maximo Jesus Cuadros Chavez:


Hipertermia maligna
Malignant Hyperthermia
Denborough, Michael A. M.D., M.B., Ch.B., D.Phil., D.Sc., F.R.A.C.P., F.R.C.P., F.N.Z.C.A.
Anesthesiology 2008;108:156-157.  doi: 10.1097/01.anes.0000296107.23210.dd
Additional material related to this article can be found on the Anesthesiology Web site. Go to, click on Enhancements Index, and then scroll down to find the appropriate article and link. Supplementary material can also be accessed on the Web by clicking on the ArticlePlus link either in the Table of Contents or at the top of the Abstract or HTML version of the article.

A local family is described in which there have been ten deaths attributable to general anesthesia. The pattern of inheritance of the abnormality is compatible with that due to an incompletely penetrant dominant gene or genes. Spinal anesthesia produced no ill effects when used in the one member of the family who survived a reaction following a general anesthetic.

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Estado actual sobre hipertermia maligna y tratamiento con Dantrium i.v.
Current State of Malignant Hyperthermia And the Use of Dantrium IV as Treatment.
Rosenberg H.
Anesthesiology News 2010:36:3
Malignant hyperthermia (MH) is an autosomally inherited disorder characterized by an increase in heart rate, respiratory rate, body temperature, and muscle rigidity when the patient is exposed to potent volatile anesthetic gases and succinylcholine. The prevalence and incidence of MH are difficult to determine because patients display no characteristic signs until anesthetized with one of the triggering agents, and even then do not always develop the disorder. This article discusses the clinical presentation of MH, treatment using Dantrium® IV, and provides information on educational resources such as the Malignant Hyperthermia Association of the United States.
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Tendencias y evolución de hipertermia maligna en Estados Unidos de Norteamérica, 2000 a 2005.
Trends and outcomes of malignant hyperthermia in the United States, 2000 to 2005.
Rosero EB, Adesanya AO, Timaran CH, Joshi GP.
Department of Surgery, Division of Vascular Surgery, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas 75390-9157, USA.
Anesthesiology. 2009 Jan;110(1):89-94.

BACKGROUND: Malignant hyperthermia (MH) is a potentially fatal pharmacogenetic disorder with an estimated mortality of less than 5%. The purpose of this study was to evaluate the current incidence of MH and the predictors associated with in-hospital mortality in the United States. METHODS: The Nationwide Inpatient Sample, which is the largest all-payer inpatient database in the United States, was used to identify patients discharged with a diagnosis of MH during the years 2000-2005. The weighted exact Cochrane-Armitage test and multivariate logistic regression analyses were used to assess trends in the incidence and risk-adjusted mortality from MH, taking into account the complex survey design. RESULTS: From 2000 to 2005, the number of cases of MH increased from 372 to 521 per year. The occurrence of MH increased from 10.2 to 13.3 patients per million hospital discharges (P = 0.001). Mortality rates from MH ranged from 6.5% in 2005 to 16.9% in 2001 (P < 0.0001). The median age of patients with MH was 39 (interquartile range, 23-54 yr). Only 17.8% of the patients were children, who had lower mortality than adults (0.7% vs. 14.1%, P < 0.0001). Logistic regression analyses revealed that risk-adjusted in-hospital mortality was associated with increasing age, female sex, comorbidity burden, source of admission to hospital, and geographic region of the United States. CONCLUSIONS: The incidence of MH in the United States has increased in recent years. The in-hospital mortality from MH remains elevated and higher than previously reported. The results of this study should enable the identification of areas requiring increased focus in MH-related education.

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Papel del CACNA 1S en la predisposición para hipertermia maligna

The role of CACNA1S in predisposition to malignant hyperthermia.
Carpenter D, Ringrose C, Leo V, Morris A, Robinson RL, Halsall PJ, Hopkins PM, Shaw MA.
MH Investigation Unit, Academic Unit of Anaesthesia, St James's University Hospital, Leeds, LS9 7TF, UK.
BMC Med Genet. 2009 Oct 13;10:104.
BACKGROUND: Malignant hyperthermia (MH) is an inherited pharmacogenetic disorder of skeletal muscle, characterised by an elevated calcium release from the skeletal muscle sarcoplasmic reticulum. The dihydropyridine receptor (DHPR) plays an essential role in excitation-contraction coupling and calcium homeostasis in skeletal muscle. This study focuses on the gene CACNA1S which encodes the alpha1 subunit of the DHPR, in order to establish whether CACNA1S plays a major role in MH susceptibility in the UK. METHODS: We investigate the CACNA1S locus in detail in 50 independent MH patients, the largest study to date, to identify novel variants that may predispose to disease and also to characterise the haplotype structure across CACNA1S. RESULTS: We present CACNA1S cDNA sequencing data from 50 MH patients in whom RYR1 mutations have been excluded, and subsequent mutation screening analysis. Furthermore we present haplotype analysis of unphased CACNA1S SNPs to (1) assess CACNA1S haplotype frequency differences between susceptible MH cases and a European control group and (2) analyse population-based association via clustering of CACNA1S haplotypes based on disease risk. CONCLUSION: The study identified a single potentially pathogenic change in CACNA1S (p.Arg174Trp), and highlights that the haplotype structure across CACNA1S is diverse, with a high degree of variability

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Hipertermia maligna postoperatoria: un análisis de casos del Registro Norteamericano de hipertermia maligna.
Postoperative malignant hyperthermia: an analysis of cases from the North American Malignant Hyperthermia Registry.
Litman RS, Flood CD, Kaplan RF, Kim YL, Tobin JR.
Department of Anesthesiology and Critical Care, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Anesthesiology. 2008 Nov;109(5):825-9.
BACKGROUND: The initial presentation of malignant hyperthermia (MH) may begin in the postoperative period. However, the maximal latency period between the end of anesthesia care and the onset of postoperative MH is unknown. The authors hypothesized that this latency period is short and is not manifested by hyperthermia as the initial presenting sign. The authors sought to test this hypothesis and to describe the clinical characteristics of postoperative MH by analysis of suspected cases in the North American Malignant Hyperthermia Registry. METHODS: Of 528 possible or suspected cases of MH in the North American Malignant Hyperthermia Registry, the authors identified 64 possible reports of postoperative MH. The records were reviewed in detail by the authors, each of whom assigned a qualitative score of "likely," "not likely," "not enough information available," or "not applicable" (where MH was not the final definitive diagnosis). Postoperative MH was confirmed after a consensus meeting of the three senior authors who reviewed in detail all possible "likely" cases. RESULTS: The authors identified postoperative MH in 10 subjects. All received volatile agents and 5 also received succinylcholine. All demonstrated signs characteristic of acute MH, including generalized rigidity, hypercapnia and/or tachypnea, tachycardia, and hyperthermia. No subject demonstrated hyperthermia as the presenting sign. The latency period between the anesthesia finish time and the onset of a sign indicative of acute MH ranged from 0 to 40 min. CONCLUSIONS: Postoperative MH is uncommon, occurring in 10 of 528 suspected MH cases (1.9%) reported to the North American Malignant Hyperthermia Registry. Postoperative MH began shortly after completion of the anesthetic care. Hyperthermia was not a presenting sign of MH.

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Hipertermia maligna

Malignant hyperthermia.
Rosenberg H, Davis M, James D, Pollock N, Stowell K.
Department of Medical Education and Clinical Research, Saint Barnabas Medical Center, Livingston, NJ 07039, USA.
Orphanet J Rare Dis. 2007 Apr 24;2:21.
Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane and the depolarizing muscle relaxant succinylcholine, and rarely, in humans, to stresses such as vigorous exercise and heat. The incidence of MH reactions ranges from 1:5,000 to 1:50,000-100,000 anesthesias. However, the prevalence of the genetic abnormalities may be as great as one in 3,000 individuals. MH affects humans, certain pig breeds, dogs, horses, and probably other animals. The classic signs of MH include hyperthermia to marked degree, tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, muscle rigidity, and rhabdomyolysis, all related to a hypermetabolic response. The syndrome is likely to be fatal if untreated. Early recognition of the signs of MH, specifically elevation of end-expired carbon dioxide, provides the clinical diagnostic clues. In humans the syndrome is inherited in autosomal dominant pattern, while in pigs in autosomal recessive. The pathophysiologic changes of MH are due to uncontrolled rise of myoplasmic calcium, which activates biochemical processes related to muscle activation. Due to ATP depletion, the muscle membrane integrity is compromised leading to hyperkalemia and rhabdomyolysis. In most cases, the syndrome is caused by a defect in the ryanodine receptor. Over 90 mutations have been identified in the RYR-1 gene located on chromosome 19q13.1, and at least 25 are causal for MH. Diagnostic testing relies on assessing the in vitro contracture response of biopsied muscle to halothane, caffeine, and other drugs. Elucidation of the genetic changes has led to the introduction, on a limited basis so far, of genetic testing for susceptibility to MH. As the sensitivity of genetic testing increases, molecular genetics will be used for identifying those at risk with greater frequency. Dantrolene sodium is a specific antagonist of the pathophysiologic changes of MH and should be available wherever general anesthesia is administered. Thanks to the dramatic progress in understanding the clinical manifestation and pathophysiology of the syndrome, the mortality from MH has dropped from over 80% thirty years ago to less than 5%.

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Alteraciones mitocondriales y anestesia general: una serie de casos y revisión
Mitochondrial disorders and general anaesthesia: a case series and review.
Footitt EJ, Sinha MD, Raiman JA, Dhawan A, Moganasundram S, Champion MP.
Department of Paediatric Metabolic Medicine, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Lambeth Palace Road, London SE1 7EH, UK.
Br J Anaesth. 2008 Apr;100(4):436-41.
Patients with mitochondrial disease are at risk of metabolic decompensation and often require general anaesthesia (GA) as part of their diagnostic work up and subsequent management. However, the evidence base for the use of GA is limited and inconclusive. We have documented the practice and outcome in the use of GA in paediatric patients with mitochondrial disease using a retrospective case review study of 38 mitochondrial patients who had undergone 58 anaesthetics within the regional metabolic service for the period 1989-2005. A variety of anaesthetic agents were used and the pattern of use reflects that seen in standard paediatric practice. There were no episodes of malignant hyperthermia and no documented intraoperative events attributable to the GA. Three postoperative adverse events were noted; one episode of hypovolaemia, one episode of acute on chronic renal failure, and one episode of metabolic decompensation 12 h post-muscle biopsy. Despite theoretical concern about this group of patients, adverse events after GA are rare and in most cases unrelated to the anaesthesia. Further prospective studies of GA in mitochondrial disease are required to create evidence-based clinical guidelines for safe practice
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Anestesiología y Medicina del Dolor


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