Two cases of sudden malignant hyperthershoulde fever in general anaesthetic in the family

Malignant hyperthermia is a rare and life-threatening complication of general anaesthetic, with an incidence rate of about 1/10000 in patients receiving general anaesthetic, characterized by rapid progression of high fever, acidosis, hypercarbonemia,hypertension, transverse muscle dissolution and cardiac arrestMalignant hyperthermiadiagnosisbased onclinicalperformance or laboratory examination, the most common and earliest signs are unexplained tachycardia, accompanied by a sharp increase in arterial blood carbon dioxide pressure (PaCO2)The two cases of children we reported were siblings of the same parents, although there was no caffeine fluororanean isostosis contraction test, but according to Larach and other reported scoring criteria, 2 cases of children can be diagnosed with malignant hyperthermia, is reported belowcases 1 male, 6 years old, body mass 21.5 kg, ASA grade IWith "burst abdominal pain with vomiting 1d" as the main complaint admitted to the hospital,diagnosisfor acute appendicitis, intended in the general anaesthetic down
laparoscopicappendectomyIn the past, the child denied a history of drug allergies and denied a history of surgery and trauma Preoperative abdominal CT prompt appendicitis, appendicitis, chest X-rays show double lung texture enhancement, abdominal ultrasound promptright lower abdomen block major abnormal laboratory examination: white blood cell count 20.1 x 109/L, neutrophil 0.903, clotted enzyme intime activity 74%, C-reaction protein 41.8 mg/L, creatine 31.1 mmol/L, creatine kinase 317U/L, creatine kinase-and-MB32U/L The remaining laboratory tests were not unusual The child went into the operating room at 23:00, shizhiqing, blood pressure 110/62mmHg (1mmHg - 0.133kPa), heart rate 112 times / min, body temperature 36.8 degrees C 23:15 aaesthetic induction, induced by the use of sufffintani 3 sg, propofol 50 mg, amber choline (commodity name scoolin) 30mg simultaneous inhalation of oxygen and heptafluorone ethers After about 1min, the child suddenly appears to have a stiff body, closed teeth, body temperature began to rise, up to 37.8 degrees C, and the heart rate continued to rise, 150 to 160 times / min According to the child's performance, the initial suspicion of malignant high fever Immediately changed to pure oxygen inhalation, due to the closure of the teeth, can not carry out trachea intubation, line mask method ventilation At the same time, ethanol bath, ice pack placed in the aorta cooling, parallel head cooling 23:40 child chest pattern, change ventilator, leave stomach intestinal decompression to prevent misabsorption, arterial puncture tube and emergency examination arterial blood gas analysis, pH 6.94, PaCO286mm, K-3.8mol/L, remaining base (BE)-14 .2mmol/L, given sodium bicarbonate 200ml pickling acid, 0.9% sodium chloride solution 200ml expansion of the disease treatment; 23:53 children heart rate 156 times / min, arterial oxygen saturation 0.98, body temperature maintained at 38.5 to 39.0 degrees C reviewed blood gas pH 7.1, PaCO293mmHg, K-4.6mmol/L, BE-2.1mmol/L, indicating earlier remission of acidosis 23:55 The temperature of the child reached a maximum of 39.2 degrees C and continued to cool down physically 0:00 child heart rate 172 times/min, arterial blood oxygen saturation 1.00, review blood gas pH 7.1, PaCO293mmHg, K-4.6mmol/L, BE5.2mmol/L 0:02, the heart rate of the child 180 times/min, the chest pattern disappears 0:06 to stop dripping into sodium bicarbonate, 0:09 children began to recover consciousness, autobreath recovery, pupils and other large, still ventilator methodto assist ventilation Heart rate dropped to 158 times/min, arterial blood oxygen saturation 0.98 to 1.00, muscle tone decreased, blood gas pH 7.37, PaCO251mmHg, K-3.9mmol/l, BE3.6mmol/L, indicating that acidosis has been corrected 0:25 children breathe autonomously, at this time the child is clear, the answer is accurate, the heart rate is still about 158 times / min, arterial blood oxygen saturation is maintained at 0.98 to 1.00 0:28 children's consciousness clear, accurate response to answer, pupils and other large, muscle tone reduction, heart rate reduced to about 140 times / min, arterial oxygen saturation in 0.99 to 1.00, children's self-breathing stable, 0:30 children's independent breathing smooth, conscious, accurate response to the answer, pupil, Holes such as large, reduced muscle tone, review blood gas pH 7.43, PaCO240mmHg, arterial blood oxygen pressure 98mmHg, K.4.1mmol/L, BE2.0mmol/L, vital signs stable, mask oxygen absorption into the children rescue ward After entering the ward, the child's condition is stable, sage, breathing is stable, heart rate is about 130 times /min, body temperature is 37.0 degrees C, arterial oxygen saturation 0.98 test-related laboratory indicators, creatine kinase 11879U/L, creatine kinase isoenzyme-MB252U/L, creatinine 3814 ?g/L, serum CK-MB isoenzyme mass of 308 sg/L, myocardin I055?g/L After active treatment, fever, abdominal pain, tachycardia, etc have been controlled, vital signs are stable, review the above indicators for sexual decline, 5d after transfer back to the ward, 13d after discharge 2 female, 13 years old, height 164 cm, body mass 85kg, ASA grade I To "head swelling pain for half a year, aggravated with vomiting 9h" as the main complaint to be admitted to hospital, diagnosed as "intracranial occupation." It is proposed to have a pre-positional lesions excision in the under-cranial underthesia of general anaesthetic In the past, he denied a history of medication and food allergies, and described his brother's history of amber choline allergy Preoperative chest X-rays: right lung lower field (lower leaf?) Lung texture is blurred, inflammatory reaction; head CT: right frontal lobe occupies position, glioma; laboratory examination has no obvious abnormalities 13:35 children into the operating room, generally in good condition, clear mind Blood pressure 110/65mmHg, heart rate 89 times/min, arterial blood oxygen pressure 0.99 Anesthetic induction using suffintenney 20 mg, relying on mieest 18mg, shun aquku ammonium 13mg, muscle pine satisfaction after insertion under the visual laryngoscope No 7.0 reinforced trachea catheter, intubation process is smooth Anaesthetic maintenance using 2% heptafluoroetheration, laughing gas: oxygen: 1:1 inhalation, riffentanus pumped in After mechanical ventilation exhalation of the end of carbon dioxide pressure (PETCO2) gradually increased, 14:58 line of blood gas analysis, PaCO2 46.6mmHg, touch body surface temperature is high, body temperature probe show body temperature 38.3 degrees C, suspected of malignant hyperthermia, replace the anaesthetic machine and respiratory lines, stop inhaling heptatric ether, change to pure oxygen inhalation Using propofol 22 ml/h, riffini 6 ml/h to maintain anesthesia while physically cooling (ethanol wipe, ice salt water in the groin) 15:35 Review Blood Gas Analysis, pH 7.207, PaCO2 72.5mmHg, K-4.79mmol/L, 15:50PaCO2 reached the highest, at this time the blood gas analysis results are as follows: pH 7.190, PaCO2 80.6mm, K-4.92mmol/L A total of 620mg of injectable sodium acetate was given to the drug to cool down at 15:23, 16:07 and 17:08 respectively, and a total of 30mg was given to the furcem at 15:45 and 16:64, and 16:41 was given to glycol 250ml intravenous driptose to maintain urine volume and protect kidney function Throughout the process, intravenous deoxyrepinephrine is injected, continuously pumping epinephrine, norepinephrine to maintain hemodynamic stability Blood pressure remained at around 80/50mmHg and heart rate fluctuated between 120 and 140 times/min 17:14 Surgery ended, with tracheotomy into the children's rescue ward, out of the operating room blood pressure 100/60mmHg, heart rate 138 times / min, PETCO2 54mmHg, body temperature 41.2 degrees C, blood gas analysis pH 7.279, PaCO2 46.2mmHg, K -4.98mmol/L transferred to the child rescue ward after perfecting the relevant examination, creatine kinase 896U/L (normal range 29 to 200U/L), myoglobin 1257 sg/L (normal value 0 to 105.7?g/L), creatine 82.2m/L (normal range 45 to 84m/Lmol) On the day of surgery, creatine kinase and creatine increased significantly, and creatine kinase isoenzyme-MB showed a gradual upward trend in the days after surgery On the 5th day after surgery, the level of myoglobin, creatine kinase and creatine kinase isoenzyme-MB was significantly higher than before, creatine kinase 4418U/L, creatine 1072.8 sg/L, again with high fever, considering malignant high fever recurrence, timely treatment removed the trachea catheter on the 7th day after surgery and then transferred back to the general ward, where the patient's vital signs were stable and recovered well After surgery, patients unconscious disorder and personality change symptoms occurred, regularly review the head MR did not see the signs of tumor recurrence, 1 year later the patient for the craniofacial repair came back to our hospital, to "skull defect, glioma surgery" for the diagnosis of income to our hospital It is proposed to underage the undergoing cranial repair technique, preoperative patient surate 618 smol/L (normal range 142 to 420 smol/L), creatine kinase 237U/L (normal range of 145U/L), the remaining laboratory test did not show significant abnormalities patients enter the operating room at 9:01, blood pressure 110/63mmHg, heart rate 75 times/min, arterial blood oxygen pressure 0.98 Anesthetic induced use of propofol 150mg, Rocobromine 50mg, Seffentani 20 sg, myosone satisfied with the insertion of 7.0 trachea duct scatheterunder under the visual laryngos Anesthesia maintains the continuous pumping of propofol and rifenite, the patient's vital signs are stable during surgery, no PETCO2 and abnormal lysage of body temperature, after surgery waiting for the patient's self-respiratory recovery, removal of trachea catheters, and return to the ward discussion malignant high fever is induced by inhalation of anesthetics and depolarized myocarditis amber choline, and a few can also be induced by intense activity and fever In recent years, there have been cases of ketamine, lidocaine and neurostabilizer (chlor-nitrogen, fluoroquinol) induced by malignant high fever Malignant hyperthermal susceptible people without induced factors can not clinical performance, because muscle biopsy is a invasive test, the current determination of malignant hyperthermal susceptible people is more inclined to carry out genetic testing In the absence of a genetic diagnosis, the following patients may be considered as malignant hyperthermal susceptibility: (1) i.e or immediate family members have confirmed that there is a muscle disease associated with RYR1-, CACNA1S, or STAC3-gene mutations (2) I or my immediate family members have a history of malignant hypertherctary fever triggered by general anaesthetic drugs (3) I or my immediate family members have elevated creatine kinase associated with high temperature, vigorous activity or statins, muscle stiffness or horizontal muscle dissolution therefore, patients with the above conditions should be given special attention Malignant hypertheruccian people receive general anesthesia should pay attention to the following points: (1) avoid the use of inhaled anaesthetic and chlorinated amber choline (2) Placing activated carbon in the respiratory circuit has been shown to reduce the incidence of malignant high fever (3) Monitor the center temperature when the operating time is greater than 30min The probability of malignant high fever is very low, but once it occurs, the fatality rate is very high in the absence of sodium dancurin In China, Danqulin sodium is a typical orphan medicine, at present, there is no formal channels of import, nor domestic production of Danqulin sodium rescue treatment measures to use cooling, organ protection and other treatment measures, after 2015, a very few hospitals in China in the medical service for the record or emergency to ask the hospital for approval, and in the case of informed consent of the patient's family, the use of foreign donations or emergency purchase of sodium dancuril in abroad successfully rescue malignant high fever patients, but its mortality rate is still very high These 2 cases are sudden, our hospital did not have a reserve of danqulin sodium, so the corresponding treatment measures, due to the timely discovery, active treatment, are successful rescue The second patient in this paper describes his brother's history of amber choline allergy, but because the family's lack of medical knowledge does not mention the history of malignant hypertherance, the history of the disease was not further followed before surgery This case suggests that we must pay attention to the family history and the inquiry of the history of anesthesia in the past, and the lack of temperature monitoring in malignant hyperthermia events increases the relative risk of death by 13.8 times, so the monitoring of body temperature should be strengthened during surgery