Propionic acidemia: report of a case that is successfully managed by peritoneal dialysis and sodium benzoate therapy

Propionic acidemia is a rare hereditary disease which is an autosomal recessive disorder. Defect of propionyl CoA carboxylase results in abnormal accumulation of propionate and its metabolites which interfere the pathway of glycine cleavage and the urea cycle. This organic acidemia is characterized by a wide spectrum of clinical and biochemical findings, including recurrent vomiting, difficult feeding, lethargy, hypotonia, metabolic ketoacidosis, hyperglycinemia and hyperammonemia during the acute episodes. We present a male newborn infant who sustained this disorder and was managed successfully with blood exchange transfusion, peritoneal dialysis, supplemented with sodium benzoate and sodium bicarbonate therapy. Urine gas chromatography disclosed significant elevation of propionate and its metabolites which subsided 2 days after peritoneal dialysis. Special designed formula was then given with restriction of protein intake and supplement with sodium benzoate and sodium carbonate. Prenatal genetic counseling is necessary in further pregnancy. Diagnosis can be obtained when propionyl CoA carboxylase activity is low in cultured amniotic fluid cells or chorion villi sample or when there is abnormally high methylcitrate level in amniotic fluid.     

Old pulmonary embolism causing intrabronchial bleeding

A 51-year-old man was admitted because of hemoptysis. Physical examinations and chest XP revealed no abnormal findings. Fiberoptic bronchoscopy showed pulsatile bleeding at the orifice of right B6. Right bronchial arteriography showed a markedly dilated and tortous bronchial artery and shunting to the pulmonary arterial system in the middle and lower lobes. Pulmonary arteriography showed complete obstruction of the right middle lobar and lower lobe segmental arteries (A6, 9, 10). The hemoptysis was thought to be due to increased blood flow of the right bronchial artery, which compensated for reduced right pulmonary arterial flow. Right middle and lower lobe resection was done to prevent further hemoptysis. The resected specimen revealed old thromboemboli in the right middle and lower lobe pulmonary arteries. In this case old pulmonary embolism should be considered as a cause of intrabronchial bleeding.     

A Bronchial Response Comparison of Exercise and Methacholine in Asthmatic Subjects

The authors compared the inhaled methacholine and exercise responses in 22 stable unmedicated asthmatic patients. The exercise and methacholine challenges were performed at one to three week intervals. Bronchial responsiveness to methacholine was measured in relation to the concentration of methacholine (PC₂₀M). The response to exercise was expressed as the percentage of fall in FEV₁, from the pre-exercise FEV₁ The findings showed that 21 of 22 subjects demonstrated a fall in FEV₁, of more than 20% after methacholine challenge, while only 9/22 subjects experienced a similar decrease in FEV₁. All 9 of these positive response exercise cases completed three consecutive exercise challenges prior to the methacholine challenge. Of these cases, five were refractory to the repeated exercise challenge, and the PD₂₀M at the nonexercise stage was significantly lower than the postexercise state. In fact, the methacholine challenge sensitivity actually decreased (PD₂₀ increased) after repeated exercise. The authors concluded that methacholine seems to be a more sensitive bronchial provocation test than exercise. Second, only 55.6% of the exercise test-positive subjects were refractory to the second exercise challenge. Therefore, other factors besides the release of mediators should be considered in exercise induced asthma. Third, methacholine sensitivity actually decreased (PD₂₀ increases) after repeat exercise challenge.     

Effect of temperature in focal ischemia of rat brain studied by 31P and 1H spectroscopic imaging

³¹P, ¹H and lactate spectroscopic imaging was used to evaluate the effects of hypothermia on focal cerebral ischemia produced by middle cerebral artery occlusion. The effects on high energy phosphate metabolism, pH, lactate and NAA were investigated in 24 spontaneously hypertensive rats subjected to either permanent or transient ischemia. Under either normothermic (37.5°C) or hypothermic (32°C) conditions, with permanent 6-h occlusion, there was little difference between groups in either the NMR measurements or the volume of infarction. In animals that underwent 3 h of ischemia followed by 12 h of reperfusion, the ischemic changes in lactate, pH, NAA, and high-energy phosphate returned toward control values, and there was a protective effect of hypothermia (infarct volume of 211 ± 26 and 40 ± 14 mm³ in normothermic and hypothermic groups, respectively). Thus, hypothermia did not ameliorate the changes in lactate, pH, NAA, or high energy phosphate levels occurring during ischemia, however, during reperfusion there was an improvement in both the recovery of these metabolites and pathological outcome in hypothermic compared with normothermic animals.     

口服Sumatriptan治疗偏头痛的临床评价

本文报告口服Sumatriptan 100mg对偏头痛急性发作119例次的治疗结果。治疗后4h内显效91例次(76.5％),好转16例次(13.4％),无效12例次(10.1％),总有效率为89.9％。对偏头痛伴随症状恶心、呕吐和畏光、畏声的缓解率分别为94.2％、96％和94.3％。     

16例胸膜间皮瘤临床分析

１６例胸膜间皮瘤，其中１例有石棉接触史，占６．１７％；＞４０岁者占８１．５％。主要临床表现为胸痛、气短、咳嗽、低热和胸腔积液，临床上易误诊为结核性胸膜炎、肺癌胸膜转移等。胸部ｘ线检查及胸部ＣＴ对该病的诊断有帮助，大都有特征性表现。确诊靠针刺胸膜活检及开胸活检。胸膜间皮瘤的治疗主要采用手术、放疗和化疗，对于局限型治疗首选手术切除。肿瘤的良、恶性、疾病的分期及治疗与预后有一定关系。     

Risk factors of acute pancreatitis]

A total of 689 operative cases of acute necrotic pancreatitis (ANP) were collected from 42 hospitals all over the country by the ANP Cooperative Study Group in the years of 1987 to 1989. General data, laboratory findings, pathology of the pancreas and the type of surgical treatment were analysed according to the outcome of the patient. The results showed that 37% of the cases were related to cholelithiasis, the highest incidence was in age above 60, and female patients were predominant and with higher mortality. Laboratory findings at admission, WBC greater than 20,000/mm3, Hb greater than 15 g%, blood sugar greater than 200 mg%, serum bilirubin greater than 5 mg%, BUN greater than 20 mg%, and serum calcium less than 7 mg% were related to the markedly elevated mortality rate. Operative findings noted in patients with bloody or cloudy peritoneal fluid, necrotic changes up to 75% of the pancreas, more than 2 areas of extrapancreatic involvements and resection of a large portion of the pancreas was found to be necessary were all related to a higher mortality. And emergency operations performed within 24 hours after the onset of the disease was also related with a highest mortality rate.     

Laparoscopic nephrectomy in a porcine model.

Laparoscopic nephrectomy was performed in 15 male pigs, the procedure was successful in 14. Extraction of the intact kidney through a 5-cm lower abdomen incision was done in 7 animals; complete destruction and evacuation of the kidney was accomplished by a round-knife suction device through a 1-cm port in another 7 pigs. Grossly, the specimen consisted of sausage-like tubular renal tissue and a small amount of tissue debris. Pathology revealed that the glomerular and tubular structures were well preserved, no interstitial hematoma could be found. Four ports were usually used, one 1-cm umbilical camera port, one 0.5-cm port for ureter traction, and two 1-cm working ports along the midclavicular line. All the pigs recovered uneventfully. The average operation time was 3 h 20 min. The application of endo-GIA (United States Surgical Corporation) for renal hilum reduced the operative time to 2 h 20 min. Complications included renal vein tear during endoclip application and cutting in the first case, mild subcutaneous emphysema in 2 cases. This first pig received exploratory laparotomy for the repair and ligation of the renal vein. No more major complications occurred with increasing experience. From this porcine experiment, we conclude that the combination of laparoscopy, a tissue destroyer and an endobag for the entrapment of kidney seem to be a promising technique for clinical nephrectomy.     

Neuromuscular block with vecuronium reduces the rapidly extracted auditory evoked potentials index during steady state anesthesia

PURPOSE: During clinical monitoring, vecuronium appeared to reduce the rapidly extracted auditory evoked potentials index (A-line ARX index or AAI) to some extent. A prospective and randomized study was designed to analyze this phenomenon. METHODS: Forty adult patients undergoing elective surgery were studied. After tracheal intubation, anesthesia was maintained with an end-tidal isoflurane concentration (F(ET)ISO) of 1.0% for 20 min, then a 10-mL dose of either vecuronium 0.05 mg*kg(-1), 0.1 mg*kg(-1), 0.2 mg*kg(-1) or saline was administered in a randomized, double-blind design. The AAI and bispectral index (BI(hx)) were monitored throughout the study and analyzed off-line. RESULTS: BI(hx) was unaltered after the administration of saline or vecuronium. The mean of the averaged (per patient) AAI values recorded from two minutes to ten minutes after the administration of saline or vecuronium 0.05 mg*kg(-1) did not differ significantly from the corresponding mean recorded from 15 min to 20 min after F(ET)ISO maintained 1.0% (P = 0.678, 0.169), however after the administration of vecuronium 0.1 mg*kg(-1) or 0.2 mg*kg(-1), AAI was reduced from 18.3, 18.0 to 14.8, 13.4 (P = 0.016, 0.017). CONCLUSIONS: Neuromuscular block with vecuronium reduces AAI in patients during steady state anesthesia without surgical stimuli, while BI(hx) is unaltered. The cut-off values of AAI for events should be determined according to the level of neuromuscular blockade when monitoring the depth of anesthesia/sedation.