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1、肌肉溶解综合症（Lysis syndrome）The pathophysiology and diagnosis of rhabdomyolysis: Su Lei, Meng FansuChinese Journal of emergency medicine, 2007, eleventhRhabdomyolysis (rhabdomyolysis, RM) refers to a series of factors affecting striated muscle cell membrane, membrane channel and its energy supply of mult
2、iple genetic or acquired diseases resulting in striated muscle injury. The change of cell membrane integrity, the leakage of cellular contents, including myoglobin (myoglobin, Mb), creatinekinase (creatine pphos-phokinases, CPK) and other enzymes, ions and small molecules of toxic substances, often
3、accompanied by the threat of metabolic disorders and acute renal failure life (ARF). In 1881 Fleche first reported by muscle caused by compression of rhabdomyolysis after 1970s reported stroke, poisoning and infection causes of non traumatic rhabdomyolysis (NRM).1 etiologyRhabdomyolysis caused by tr
4、auma has been referred to as gap syndrome and crush syndrome. Compartment syndrome refers to a limb due to trauma or by local performance after extrusion osteofascial compartment pressure caused by increased muscle ischemia; and crush syndrome refers to systemic muscle injury caused by the direct fl
5、ow of trauma or ischemia - reperfusion. Broadly speaking, rhabdomyolysis is caused by destruction of skeletal muscles leading to release of cell contents into the blood and excretion of urine. In addition to trauma factors, non traumatic factors, including genetic etiology, excessive exercise, muscl
6、e extrusion, ischemic metabolic abnormalities, extreme body temperature, drugs, poisons, infections, and other factors can lead to rhabdomyolysis.1.1 traumaAny major cause of muscle damage or ischemia can lead to rhabdomyolysis, including direct and indirect injuries.1.2 non traumaticAlthough rhabdo
7、myolysis was first found in trauma patients, rhabdomyolysis, at least, is more than 5 times more traumatic than rhabdomyolysis.(1) excessive exertion due to impaired substrate utilization or lack of exertional rhabdomyolysis caused by energy metabolism occurs frequently in strenuous exercise, such a
8、s military training, weight lifting, and marathon running.(2) muscle ischemia due to shock, carboxyhemoglobinemia, asthma, drowning caused by generalized muscle ischemia; local dressing too tight, long time use of anti shock garment and air splint caused by local muscle ischemia; the braking time ca
9、used by long time surgery and spinal cord injury; in addition, due to the blocking of heparin induced gas the thrombus, diving causes embolism, vasculitis caused by arterial and venous.(3) excessive high temperature and low temperature, frostbite or overheating can cause rhabdomyolysis.(4) electroly
10、te and osmotic pressure changes and metabolic diseases and electrolyte disorders (low potassium, low phosphorus), severe edema, diabetic ketoacidosis, hyperosmolar diabetic coma, hypothyroidism and other metabolic disorders can lead to rhabdomyolysis, there are not many reports, but can be fatal.(5)
11、 genetic and autoimmune disease genetic disorder caused by glycogen and lipid metabolism disorders can cause rhabdomyolysis, mainly in the family history, high incidence of children, Yi Fufa, has nothing to do with the sports can not associated with muscle necrosis, myoglobinuria, including muscle a
12、trophy, polymyositis, dermatomyositis, McArdledisease, palm carnitine acyl transferase deficiency and respiratory chain enzyme deficiency caused by rhabdomyolysis.Drug and alcohol according to the literature, drug-induced rhabdomyolysis amounted to more than 150 kinds of statins (simvastatin, lovast
13、atin, pravastatin) caused by rhabdomyolysis is clear; if statins and other drugs (erythromycin, clarithromycin, azithromycin, itraconazole, warfarin, digoxin, dicoumarin, non Kyrgyzstan Betsey, cyclosporine, chlorzoxazone, etc.) rhabdomyolysis combined to increase the chance of occurrence of alcohol
14、ism, is also one of the reasons causing rhabdomyolysis.(7) infection is one of the causes of rhabdomyolysis, and influenza viruses are the most common viruses causing rhabdomyolysis,Herpes simplex virus, EB virus, Coxsackie virus and HIV infection caused by RM have been reported, but the exact mecha
15、nism is still not very clear; Legionella bacteria are the most common cause of rhabdomyolysis, also Streptococcus, Salmonella and other reports.2 diagnostic criteria2.1 clinical and laboratory features(1) muscle swelling and limb weakness in varying degrees;(2) black, brown, urine suggesting myoglob
16、in;(3) the creatine kinase and other muscle enzymes (AST, aldolase, lactate dehydrogenase) were increased;(4) creatinine, urea, uric acid; high, low or high share K + Ca +, Pa3 + and high metabolic acidosis (lactic acid);(5) thrombocytopenia or DIC;Some can be hot and the white blood cells rise;(7)
17、30% patients with acute renal failure;Trauma is often associated with low blood volume shock, metabolic disturbances, and heart damage.2.2 laboratory testing methods and evaluation(1) creatinephosphokinase (CK) and its isoenzymes as a major muscle enzyme, when muscle cell injury or death when CK rel
18、eased into blood plasma CK1000 U/L showed that the muscle damage, when the CK20 000 U/L appear myoglobinuria, caused by the exclusion of other causes of injury can be diagnosed with muscle injury. There are CK in myocardium, skeletal muscle and brain. In order to further identify the source of CK, i
19、sozyme analysis is often performed. Normal CK-MB/CK1% is used, and when its ratio is between 1% and 3%, it can be indicated that skeletal muscle is damaged.(2) blood and urine myoglobin detection was positive.(3) acyl carnitine was used as an indicator of the exact etiology of rhabdomyolysis associa
20、ted with fatty acid oxidation disease.(4) urinary excretion of two carboxylic acids was used as a criterion for determining the metabolic deficit or rhabdomyolysis of the corresponding enzyme defect.(5) molecular point mutation analysis was used to diagnose metabolic myopathy and rhabdomyolysis asso
21、ciated with the deficiency of medium chain coenzyme A dehydrogenase (MCAD).Long chain triglyceride three lipid load test or fasting test was used to understand ketone body failure to assist in diagnosis of fatty acid metabolism disorders, myopathy, and rhabdomyolysis.(7) 99Te-Pyrophoshate Scintigrap
22、hy was used to assess the extent of rhabdomyolysis.2.3 pathological changes(1) muscle biopsy showed no symptoms of muscle injury, rhabdomyolysis in 50% so pathological means necessary, muscle biopsy is not the diagnosis of non traumatic striated muscle tissue visible, part of the muscle fibers disap
23、peared and interstitial infiltration of inflammatory cells.(2) renal biopsy showed that when combined with acute renal failure, the distal nephron had myoglobin tubular formation; proximal tubular necrosis and epithelial cell shedding; monoclonal antibody En and Vision were positive.-3 complications
24、Rhabdomyolysis is frequently found in clinical complications, including electrolyte disturbances, metabolic acidosis, low blood volume, renal failure, disseminated intravascular coagulation, and acute muscular atrophy. Hyperkalemia can be life-threatening, and treatment is tricky because, despite hi
25、gh potassium levels, total potassium is lacking. Because lactic acid is produced in large quantities, along with uric acid, phosphate, sulfate, and blood potassium, the body is in a state of severe acidosis. Hyperkalemia, low blood volume and other factors lead to renal dysfunction of myoglobin. 33%
26、 patients with rhabdomyolysis eventually develop renal failure; on the contrary, 7% to 15% of the acute renal failure is due to rhabdomyolysis.3.1 acute renal failureMuscle necrosis so that a large number of liquid flow to third gap caused by effective blood volume reduction in hypovolemic shock; mu
27、scle injury cell necrosis, release of potassium, phosphoric acid, lactic acid, creatine kinase and myosin, the venous return absorbed into the blood circulation caused by hyperkalemia and acidosis;A large number of myoglobin direct damage to renal tubules, and cause small renal tubular obstruction,
28、causing acute renal failure; potential vasoactive substances cause renal ischemia, thereby reducing glomerular filtration rate.3.2 inflammation and MODSRhabdomyolysis, increased TNF- concentration in serum alpha, the IL-6 release of pro-inflammatory mediators, cytokines and inflammatory mediators re
29、lease promoting endothelial injury, cause inflammation reaction. The combined effect of endothelial damage and coagulation damage on rhabdomyolysis promotes the development of sepsis and further develops MODS.4 treatment4.1 General treatment principles(1) to prevent further muscle damage removal rev
30、ersible cause muscle damage, such as compartment syndrome, including measures taken to quickly remove the pressure body and transferred to the hospital, make the brake body relax, get rid of anti shock garment etc.(2) correct the low blood volume and renal ischemia, once the rhabdomyolysis occurs, a
31、s early as possible to enter a large number of liquid, to ensure the volume of blood and dilute the myoglobin already reached the kidneys.(3) promote the excretion of myoglobin from the kidneys, and use sodium bicarbonate to clean the urine. By means of promoting the release of Fe2 + myoglobin, redu
32、ce the vasodilator effect of myoglobin in direct and indirect toxic effects on renal tubular potential, can improve renal blood flow, reduce renal ischemia; at the same time as a free radical scavenger, reduce free radical damage to the kidney.(4) blood purification therapy rhabdomyolysis when conce
33、ntration of urea nitrogen and creatinine, blood K + increases rapidly, early continuous renal replacement therapy (CRRT) can effectively reduce serum creatinine, urea nitrogen and serum potassium concentration. Myoglobin has a molecular weight of 17000 and can not be eliminated by plasma replacement
34、 by dialysis membranes. The above treatment can shorten the recovery time of renal function and play an important role in the treatment of rhabdomyolysis. In addition, a large number of inflammatory mediators release during rhabdomyolysis, and CRRT plays an important role in eliminating inflammatory
35、 mediators and avoiding secondary damage to other organs.4.2 treatment of traumatic rhabdomyolysisThe first aid and early treatment of crush injury include relieving external force, fixing the injured limb, resisting shock, anti infection and alkalization urine. It should be pointed out that the occ
36、urrence of crush and crush syndrome is related to the increase of muscle ischemia and the increase of the pressure in the fascia cavity. If the limbs are swollen rapidly and the distal blood circulation is impaired, the fascia cavity should be cut as early as possible so as to improve the circulatio
37、n of the limbs and reduce the absorption of harmful substances. For patients with delayed diagnosis and treatment, a decompression should be performed in a timely manner, and good prognosis is expected. Amputation is considered if necessary.Treatment of 4.3 exertional rhabdomyolysisThe key to treatm
38、ent is early detection, early diagnosis, early treatment, timely treatment of complications, to prevent further deterioration of the disease. When muscle damage is 100 g and globulin levels exceed 515 mg/L, urine color turns brown or black urine, suggesting that exercise-induced rhabdomyolysis may c
39、ause kidney damage and should be highly valued.Rhabdomyolysis is a muscle by a large number of muscle cells released into the blood circulation in a syndrome after injury, multiple trauma and non trauma reasons can lead to its occurrence, can cause acidosis, electrolyte disorder, coagulation disorde
40、rs, low blood volume and acute renal failure, induced sepsis and MODS, which can be fatal. Because of its primary and secondary characteristics, it is suggested that the striated muscle should be classified as another important organ. Interventions included early monitoring, removal of inducements,
41、alkalization of urine, diuresis, and blood purification. Patients with rhabdomyolysis require intensive monitoring. Early diagnosis and timely intervention are of vital importance for the prognosis.-Malignant hyperthermiaNo.1 Hospital of Peking University Department of Anesthesiology Xu XuMalignant
42、hyperthermia (Malignant, hyperthermia, MH) is an inherited myopathy characterized by high metabolism and triggered by exposure to certain anaesthetic agents.The onset may be several hours after anesthesia. Non depolarizing muscle relaxants can delay seizures.The pathogenesis of sarcoplasmic reticulu
43、m calcium intake to reduce the weight (weight intake is essential for termination of muscle contraction), caused by sustained muscle contracture, high metabolism symptoms: metabolic acidosis, tachycardia, hypercapnia, glycogenolysis, hypoxemia, high fever.Some patients may contact the trigger drug r
44、epeatedly before the onset of the disease. A typical MH is triggered by succinylcholine (Sch) or a volatile anestheticincidence and epidemiology* China: no data* Western statistics: MH genetic quality in the crowd: 1/10000,The incidence of anesthesia was in children, 15000 in adults, 50000 in 1/, an
45、d more in men than in women.The mortality rate is about 10%.* Japan: the first case reported in 1960 was the morbidity of anesthesia: 1/70000, male / female, =3.6/1* is not a genetic quality of people, the use of drugs will trigger the inevitable onset.* when there was no specific treatment, the mor
46、tality rate was 80%, 10% at the time of effective treatment.etiologyAutosomal dominant inheritance is associated with a 50% chance of developing the disease in children.F, FMH+M, M=F, M+F, M+FMH, M+FMH, M=1/220% of the diseased families found mutations in nineteenth pairs of chromosomes that determi
47、ne the ryanodine receptor (a, calcium, release, channel). In addition, two mutant genes, fc20 and fc34, were found but not related to ryanodine receptor., demonstrating genetic polymorphisms in this syndrome.clinical characteristics and diagnosisThe initial symptoms of MH are increased PetCO2 and de
48、creased PaO2, tachycardia and arrhythmia, muscle weakness (muscle relaxant can not be relieved), shortness of breath (with spontaneous breathing), unexplained tachycardia, usually the first symptom.Other symptoms include: fever and cyanosis. When MH is suspected, the following laboratory examination is helpful to the diag