Anaesthetic management of a child with Bartter’s syndrome

We report the anaesthetic management of an eight-year-old asthmatic boy with Bartter’s syndrome who had bilateral orchidopexy with caudal epidural analgesia. Bartter’s syndrome is a rare congenital disorder characterized by hypokalaemic hypochloraemic metabolic alkalosis, hyperaldosteronism, hyperreninaemia and hyperplasia of the juxtaglomerular apparatus of the kidneys. Characteristically, although these patients are normotensive they may be hypovolaemic. They may have unstable baroreceptor responses and show marked resistance to vasopressors. Hence, fluid, acid-base and electrolyte imbalances along with haemodynamic instability pose particular problems in their anaesthetic management. Previous case reports have described the management of these patients with general anaesthesia, our patient had his orchidopexy with caudal epidural analgesia using plain bupivacaine 0.5%. The patient was haemodynamically stable throughout surgery and was comfortable with caudal analgesia as the sole anaesthetic. Hypovalaemia, acid-base status and electrolyte imbalance were treated before instituting caudal epidural analgesia. We present this case report which describes the anaesthetic considerations in the light of the pathophysiology of Bartter’s syndrome.     

Evaluation of myocardial energy status in vivo by NMR spectroscopy

Summary NMR spectroscopy is a powerful and non-invasive technique with which to study cardiac energy metabolism in vivo. This mcthod makes use of the "spin" properties of certain atomic nuclei. The naturally occurring phosphorus nucleus (P-31) is visible by NMR and phosphorus-31 NMR spectra contain signals from the major components of energy metabolism. In vivo, the phosphocreatine to ATP ratio (PCr/ATP) is used as an index of the energy status and viability of the myocardium. However, it is the response of this metabolic index to differing physiological and pharmacological stresses that has helped to elucidate the mechanisms that regulate cellular respiration and to highlight abnormalities in heart failure. As there are many technical difficulties involved with cardiac NMR, 31-phosphorus studies of skeletal muscle have provided an indirect way of studying abnormalities in myocardial metabolism in vivo.One of the unique features of NMR is that it permits in vivo measurements of fluxes through key enzymes in energy metabolism using magnetization transfer. Determination of the rates of energy transfer through the creatine kinase reaction and energy turnover in vivo will provide new insights into the control of energy metabolism in health and disease. Alternatively, carbon-13 NMR can be used to measure fluxes through the different metabolic pathways of synthesis and catabolism following administration of selectively labelled carbon-13 substrates. In conclusion, the non-invasive and versatile nature of NMR spectroscopy makes it an ideal method to assess and evaluate energy metabolism in vivo.     

Laminar organization of the extraocular muscles of the rabbit.

Studies of bilateral hemispheric somatosensory evoked potentials (SEP) were carried out on 17 comatose patients following closed-head injury. Control evoked potentials were obtained from 74 normal volunteers. A prognosis of potential recovery or nonrecovery from coma was made in all 17 patients based on the SEP analyses. All subjects underwent bilateral median and peroneal nerve stimulation and responses were averaged from scalp electrodes placed over the somatotopic sensory cortex for the wrist and leg. The prognostic outcome of all 17 patients was accurately predicted following SEP analysis. Results show that 4 of 17 patients with prognoses of “positive recovery” had eight defined SEP peaks present within a time base analysis of 300 ms. Twelve patients with prognoses of “negative recovery” had five or less SEP peaks present within a time base of 500 ms. The majority of the “negative recovery” patients showed only SEP primary waveform components consisting of two peaks. The data suggest a potentially useful method using SEP analysis to prognosticate the possible recovery from clinical coma.     

Huntington's Disease — Imbalance of free amino acids in the cerebrospinal fluid of patients and offspring at-risk

Summary A total of 27 different amino acids were determined in the fasting, morning lumbar CSF of 12 patients with Huntington's Disease (HD), 8 at-risk offspring and 16 non-choreic control patients. A significant (P<0.001) decrease was observed for asparagine, isoleucine, leucine, phenylalanine, histidine, arginine, -aminoadipic acid and homocarnosine in patients with HD compared to the non-choreic controls. Only tyrosine was increased in HD. These alterations were to an extent more pronounced in 5 neurophysiologically conspicuous offspring. The alterations suggest that amino acid imbalance is an early metabolic disturbance in HD.

---

Zusammenfassung Bei 12 Patienten mit manifester Huntingtonscher Krankheit (HD), 8 nicht erkrankten Nachkommen und 16 nicht choreatischen Kontrollpatienten wurden 27 verschiedene Aminosäuren im Liquor cerebrospinalis (nüchterner Lumballiquor) untersucht. Asparagin, Isoleucin, Leucin, Phenylalanin, Histidin, Arginin, -Aminoadipinsäure und Homocarnosin waren signifikant (P<0.001) erniedrigt bei Patienten gegenüber den Kontrollen. Diese Veränderungen waren bei 5 neurophysiologisch auffälligen Nachkommen teilweise ausgeprägter, was für eine frühzeitige metabolische Störung spricht. Tyrosin war als einzige Aminosäure erhöht. Die Veränderungen sprechen für eine Aminosäurenimbalanz bei Huntingtonscher Krankheit, deren Bedeutung besprochen wird.

     

Plasma levels and half lives of thioridazine and some of its metabolites

Summary Plasma-levels of thioridazine, mesoridazine, sulphoridazine and two other metabolites were determined in ten older chronic psychotic patients on thioridazine therapy. The plasma-level before the morning dose of thioridazine was the most reliable parameter for clinical studies. An intra-individual relationship between lower doses of thioridazine and plasma-levels was found. The percentage contribution of psychoactive compounds to the total sum of thioridazine plus metabolites ranged from 43–74%. The mean early disappearance half-life of thioridazine was 5 hours, and its mean late disapperance half-life was 26 hours.     

癌性恶病质代谢的研究现状

恶病质是癌症病人死亡的常见原因之一。与单纯性饥饿引起的营养不良不同．它的主要代谢改变是葡萄糖合成率的变化，糖异生、糖酵解增加，脂肪动员和氧化加速．蛋白质合成减少。     

试论中西医理论结合研究

中西医结合是推进中医学发展的重要途径。中西医理论结合是中西医结合的重要组成部分。试以神经-内分泌-免疫系统为例，探索中西医理论结合研究的切入点。从生理、病理及致病因素等几方面，用中医学的太过、不及、气血等理论阐释现代医学的神经-内分泌-免疫调节网络。     

烧伤患者三重酸碱平衡紊乱124例回顾性分析

目的探讨烧伤患者三重酸碱平衡紊乱的临床特征与治疗方法。方法回顾性分析我科自1998-01～2004-04发生三重酸碱平衡紊乱的烧伤患者124例,其中呼碱+代酸+代碱85例,呼酸+代酸+代碱39例。所有患者均予祛除原发失衡病因、根据血pH情况补酸补碱、限碱、纠正电解质紊乱等治疗。结果复查血气分析纠正率89.5%,死亡率9.7%。结论休克、吸入性损伤、脓毒症是烧伤患者发生三重酸碱失衡的主要原因。临床以呼碱型三重酸碱失衡多见,呼酸型三重酸碱失衡则大多伴有吸入性损伤。烧伤合并三重酸碱失衡往往缺乏典型临床表现,死亡率较低。及时祛除引起原发失衡的病因是治疗的关键。     

药物代谢中的药酶特性与临床研究近况

目的:为了促进药物代谢酶在临床上的深入研究。方法:以近几年国内外大量有代表性的论文为依据进行分析、归纳、整理。结果:药物代谢酶大多具有遗传多态性,其活性受相关药物影响,遗传多态性和药物之间代谢性相互作用是临床药物反应千变万化的重要因素。结论:药物代谢酶的认识和研究有助于临床合理用药和减少药物不良反应。     

Metabolic risk factors in patients with ureteropelvic junction obstruction and renal calculi

PURPOSE: We performed a prospective study to determine the incidence and spectrum of metabolic abnormalities predisposing to stone formation in patients with ureteropelvic junction obstruction and renal calculi. MATERIALS AND METHODS: A total of 47 consecutive patients with congenital ureteropelvic junction obstruction underwent metabolic evaluation of stone risk factors. Of these patients 21 had associated stones (study group), while 26 did not (control group). Logistical regression, Wilcoxon rank sum and Fisher's exact tests were performed to determine whether there was a significant difference between these groups in regard to the presence of metabolic risk factors. RESULTS: Demographically and symptomatically the 2 groups were equivalent except that the study patients were older. The 24-hour urinary excretion of calcium was significantly higher in study than in the control patients (p = 0.007). While the incidence of hypercalciuria and hyperuricosuria was also higher in the study population, these differences were not significant (p = 0.08 and 0.07, respectively). CONCLUSIONS: Metabolic abnormalities predisposing to stone formation are present more frequently in patients with ureteropelvic junction obstruction who have associated stones compared to those who do not. As such, urinary stasis alone does not explain stone formation in these cases. Rather, the local physiological environment of urine likely has a predisposing role. In addition to restoring unobstructed urinary flow, consideration should be given to metabolic evaluation and prophylactic treatment for affected patients.