respiratory acidosis

出典: meddic

呼吸性アシドーシス

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2014/05/30 21:09:59」(JST)

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英文文献

  • Safety of carbonic anhydrase inhibitors.
  • Swenson ER.Author information University of Washington - Medical Service, VA Puget Sound Health Care System , 1660 S Columbian Way, S-111-PLUM, Seattle, WA 98108 , USA Erik.Swenson@va.gov.AbstractIntroduction: Carbonic anhydrase (CA) inhibitors have an impressive safety record despite the multiple functions that CA isozymes serve because they are not fully inhibited with most dosing. While reducing the targeted CA-dependent process sufficiently for disease control, residual activity and uncatalyzed rates in combination with compensations are adequate to avoid lethal consequences. Some drugs have in vitro selectivity differences against the 13 active isozymes, but none are convincingly selective in vivo or clinically. Efforts to synthesize selective inhibitors should result in safer drugs with fewer side effects. Areas covered: This review will focus on approved drugs with CA-inhibiting activity, whether used directly for this purpose or others. Side effects are discussed in relation to various organ systems and the disease being treated. Causes of side effects are considered, and strategies for symptom reduction are given. Expert opinion: Common side effects of paresthesias, dyspepsia, lassitude and fatigue in 30 - 40% of patients are generally tolerable or abate, but if not can be partially relieved by bicarbonate supplementation. The most important safety concerns are severe acidosis, respiratory failure and encephalopathy in patients with renal, pulmonary and hepatic disease where caution is critical, as is also the case in persons with sulfa drug allergies.
  • Expert opinion on drug safety.Expert Opin Drug Saf.2014 Apr;13(4):459-72. doi: 10.1517/14740338.2014.897328. Epub 2014 Mar 10.
  • Introduction: Carbonic anhydrase (CA) inhibitors have an impressive safety record despite the multiple functions that CA isozymes serve because they are not fully inhibited with most dosing. While reducing the targeted CA-dependent process sufficiently for disease control, residual activity and unca
  • PMID 24611470
  • Mutations in the lipoyltransferase LIPT1 gene cause a fatal disease associated with a specific lipoylation defect of the 2-ketoacid dehydrogenase complexes.
  • Tort F1, Ferrer-Cortès X, Thió M, Navarro-Sastre A, Matalonga L, Quintana E, Bujan N, Arias A, García-Villoria J, Acquaviva C, Vianey-Saban C, Artuch R, García-Cazorla A, Briones P, Ribes A.Author information 1Secció d'Errors Congènits del Metabolisme, Servei de Bioquímica i Genètica Molecular, Hospital Clinic, IDIBAPS, C/Mejía Lequerica s/n, Barcelona 08028, Spain.AbstractCofactor disorders of mitochondrial energy metabolism are a heterogeneous group of diseases with a wide variety of clinical symptoms, particular metabolic profiles and variable enzymatic defects. Mutations in NFU1, BOLA3, LIAS and IBA57 have been identified in patients with deficient lipoic acid-dependent enzymatic activities and defects in the assembly and activity of the mitochondrial respiratory chain complexes. Here, we report a patient with an early onset fatal lactic acidosis presenting a biochemical phenotype compatible with a combined defect of pyruvate dehydrogenase (PDHC) and 2-ketoglutarate dehydrogenase (2-KGDH) activities, which suggested a deficiency in lipoic acid metabolism. Immunostaining analysis showed that lipoylated E2-PDH and E2-KGDH were extremely reduced in this patient. However, the absence of glycine elevation, the normal activity of the glycine cleavage system and the normal lipoylation of the H protein suggested a defect of lipoic acid transfer to particular proteins rather than a general impairment of lipoic acid biosynthesis as the potential cause of the disease. By analogy with yeast metabolism, we postulated LIPT1 as the altered candidate gene causing the disease. Sequence analysis of the human LIPT1 identified two heterozygous missense mutations (c.212C>T and c.292C>G), segregating in different alleles. Functional complementation experiments in patient's fibroblasts demonstrated that these mutations are disease-causing and that LIPT1 protein is required for lipoylation and activation of 2-ketoacid dehydrogenases in humans. These findings expand the spectrum of genetic defects associated with lipoic acid metabolism and provide the first evidence of a lipoic acid transfer defect in humans.
  • Human molecular genetics.Hum Mol Genet.2014 Apr 1;23(7):1907-15. doi: 10.1093/hmg/ddt585. Epub 2013 Nov 20.
  • Cofactor disorders of mitochondrial energy metabolism are a heterogeneous group of diseases with a wide variety of clinical symptoms, particular metabolic profiles and variable enzymatic defects. Mutations in NFU1, BOLA3, LIAS and IBA57 have been identified in patients with deficient lipoic acid-dep
  • PMID 24256811

和文文献

  • Mitochonic Acid 5 (MA-5), a Derivative of the Plant Hormone Indole-3-Acetic Acid, Improves Survival of Fibroblasts from Patients with Mitochondrial Diseases
  • Suzuki Takehiro,Yamaguchi Hiroaki,Kikusato Motoi,Matsuhashi Tetsuro,Matsuo Akihiro,Sato Takeya,Oba Yuki,Watanabe Shun,Minaki Daichi,Saigusa Daisuke,Shimbo Hiroko,Mori Nobuyoshi,Mishima Eikan,Shima Hisato,Akiyama Yasutoshi,Takeuchi Yoichi,Yuri Akinori,Kikuchi Koichi,Toyohara Takafumi,Suzuki Chitose,Kohzuki Masahiro,Anzai Jun-ichi,Mano Nariyasu,Kure Shigeo,Yanagisawa Teruyuki,Tomioka Yoshihisa,Toyomizu Masaaki,Ito Sadayoshi,Osaka Hitoshi,Hayashi Ken-ichiro,Abe Takaaki
  • The Tohoku Journal of Experimental Medicine 236(3), 225-232, 2015
  • … Mitochondrial diseases are characterized by the dysfunction of the mitochondrial respiratory chain, caused by mutations in the genes encoded by either nuclear DNA or mitochondrial DNA. … Importantly, MA-5 improved the survival of fibroblasts established from patients with mitochondrial diseases under the stress-induced condition, including Leigh syndrome, MELAS (myopathy encephalopathy lactic acidosis and stroke-like episodes), Leber's hereditary optic neuropathy, and Kearns-Sayre syndrome. …
  • NAID 130005083526
  • 小児科におけるミトコンドリア病
  • 古賀 靖敏
  • 神経眼科 31(4), 457-463, 2014
  • ミトコンドリア病は,ミトコンドリアのエネルギー産生系酵素の遺伝的異常により引き起こされる難治性進行性疾患である.エネルギー産生障害を伴うことから,エネルギー依存度の高い中枢神経系,骨格筋,心筋などの臓器障害を来す事が多い.意識障害,けいれんを主訴に来院する場合も多く,それらの原因となる他の疾患を除外する必要がある.小児期におけるミトコンドリア病は,成人発症と比較し,発症様式,臨床症状,経過,予後な …
  • NAID 130005057117
  • 筋疾患とミトコンドリア病
  • 大越 教夫,石井 亜紀子
  • 神経眼科 31(4), 446-456, 2014
  • ミトコンドリア病は,ミトコンドリアDNAあるいは核DNA異常によって生じるミトコンドリアの呼吸鎖電子伝達系障害により多彩な臨床症状を来す疾患の総称である.障害されやすい臓器は,骨格筋,中枢神経系,心臓であり,特にミオパチーでは,外眼筋と四肢・体幹の骨格筋が障害されやすい.外眼筋症状はミトコンドリア病診断の重要症状の一つで,極めてゆっくり進行する眼瞼下垂と外眼筋麻痺を特徴とする.四肢の筋力低下は通常 …
  • NAID 130005057116

関連リンク

Respiratory Acidosis Definition Respiratory acidosis is a condition in which a build-up of carbon dioxide in the blood produces a shift in the body's pH balance and causes the body's system to become more acidic. This condition is ...
Respiratory Acidosis. Respiratory acidosis is an acid-base balance disturbance due to alveolar hypoventilation. ... Metabolism Metabolism rapidly generates a large quantity of volatile acid (carbon dioxide) and nonvolatile ...

関連画像

ShareRESPIRATORY ACIDOSISRespiratory AcidosisChronic Respiratory AcidosisRespiratory Acidosis Nursing ManagementNursing


★リンクテーブル★
リンク元呼吸性アシドーシス
関連記事respiratory」「acidosis」「respirator

呼吸性アシドーシス」

  [★]

respiratory acidosis
アシドーシス酸塩基平衡異常


酸塩基平衡異常とその代償 SP.660

  HCO3- pCO2
呼吸性アシドーシス
呼吸性アルカローシス
代謝性アシドーシス
代謝性アルカローシス

原因

pocket medicine
  • CNS抑制:鎮静薬、CNS外傷、慢性高CO2血症での酸素使用(低酸素による換気ドライブ低下)
  • 神経筋疾患:重症筋無力症、GBS、ポリオ関連脊髄炎、ALS、筋ジストロフィ、重症点リン酸血症
  • 上気道異常:急性気道閉塞、喉頭痙攣、閉塞性睡眠時無呼吸、食道挿管
  • 下気道異常:喘息、COPD
  • 肺実質障害(頻繁に低酸素血症を起こす→RR↑→呼吸性アルカローシス。最終的には呼吸筋疲労により呼吸性アシドーシス):肺炎、肺水腫、拘束性肺障害
  • 胸郭構造異常→気胸、動揺胸郭(フレイルチェスト)、後側彎症
  • 分時換気量改善能に限界のあるアシドーシス患者へのHCO3 IV後


代償変化

  • 腎臓でのHCO3-の再吸収亢進


respiratory」

  [★]

  • adj.
  • 呼吸の、呼吸器の、呼吸性の
breathbreathebreathingrespirationrespiratory mechanicsrespiratory organrespiratory tract

WordNet   license wordnet

「pertaining to respiration; "respiratory assistance"」

PrepTutorEJDIC   license prepejdic

「呼吸の,呼吸作用の,呼吸のための」


acidosis」

  [★] アシドーシス

WordNet   license wordnet

「abnormally high acidity (excess hydrogen-ion concentration) of the blood and other body tissues」

PrepTutorEJDIC   license prepejdic

「酸性症,酸中毒(血液中のアルカリ濃度が異常に低い状態)」


respirator」

  [★] 人工呼吸器

WordNet   license wordnet

「a breathing device for administering long-term artificial respiration」
inhalator

PrepTutorEJDIC   license prepejdic

「《米》人工呼吸装置 / (一般に)マスク;防毒マスク」




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