Dichloroacetate treatment for severe refractory metabolic acidosis during neonatal sepsis

We describe a preterm neonate with documented group B Streptococcus sepsis and associated metabolic acidosis whose lactic acidemia was refractory to conventional sodium bicarbonate therapy but responded well to dichloroacetate treatment.     

代谢性疾病相关脑病的诊治

脑病是脑功能障碍的总称,由各类代谢紊乱引起的脑功能障碍称之为代谢性脑病,如维生素代谢异常性疾病、叶酸代谢障碍、尿素循环障碍及线粒体疾病等均可引起脑功能障碍。一些先天代谢性疾病相关脑病,可以通过特殊饮食、补充相关辅酶等得到良好控制,因此,在不可逆的脑功能损害发生前,早期诊断和治疗非常重要。癫痫是代谢性脑病的最常见表现。代谢性脑病的病因多源,起病隐匿,诊断代谢性脑病对临床医生是个很大的挑战。对于临床表现为癫痫伴生长发育迟缓、智力障碍和多系统受累的患儿,均应及时进行代谢性疾病的相关检查,如尿有机酸分析、血氨基酸分析、血酰基肉碱分析、脑脊液的相关代谢产物检测以及基因诊断。     

Episodes of severe metabolic acidosis in a patient with 3-methylglutaconic aciduria

Persistent excretion of 3-methylglutaconic acid was found in a 6-month-old infant with multiple minor physical malformations and delayed development. During two episodes of intercurrent viral illness, the patient developed severe metabolic acidosis and excreted large amounts of lactate, 3-hydroxybutyrate and acetoacetate. The excretion of 3-methylglutaconic acid did not change during these episodes, nor did it increase following leucine loading. In vitro studies suggest that in this patient, as in the majority of other patients with 3-methylglutaconic aciduria, a primary defect in leucine metabolism is not responsible for the biochemical abnormality.     

Comparison of albumin versus bicarbonate treatment for neonatal metabolic acidosis

In this study the effectiveness of 4.5% human albumin was compared with 4.2% sodium bicarbonate for neonatal metabolic acidosis, using a randomised controlled trial. The change in median pH following bicarbonate was more than twice that in the albumin group. This was statistically significant. Conclusion In normotensive infants, use of bicarbonate to correct metabolic acidosis may be more effective than use of albumin. Received: 5 March 1998 / Accepted in revised form: 7 July 1998     

Complete recovery from severe metabolic acidosis associated with isoniazid poisoning in a young boy

Isoniazid (INH) poisoning is a known cause of metabolic acidosis and seizures. The following case report describes a 14-year-old boy with an INH overdose who suffered profound metabolic acidosis (pH of 6.69) and who completely recovered with no obvious sequelae.     

��ͯ�Ŵ���л���Բ�����ϼ�����

??Abstract??Due to the metabolic disturbance?? hyperammonemia?? hypoglycemia?? metabolic acidosis and energy deficiency usually presented in many kinds of Inborn Errors of Metabolism ??IEM???? which could cause metabolic encephalopathy with poor outcome. The blood and urine samples collected and tested during the acute stage were very important for the early diagnosis and proper treatment. Early initiation of management including supportive therapy?? removal of toxic metabolite?? provision of optimum vitamins and cofactors?? specific drugs and special dietary management?? was critical for increasing the survival rate and decreasing the morbidity.     

重症小儿胸部损伤诊治的临床观察

目的 探讨重症小儿胸部损伤的诊断治疗原则.方法 回顾分析1998年3月～2007年10月收治的117例重症胸部损伤患儿,其中男83例(71%),女34例(29%),车祸伤50例,锐器伤46例,高处坠落伤15例,挤压伤4例,枪弹伤2例.发生休克29例(32%).92例(79%)给予胸腔闭式引流等保守治疗,25例(21%)接受剖胸手术.结果 109例(93%)患儿治疗后恢复良好,8例(7%)死亡,均为多发伤,其中手术中死亡2例,分别为合并颅脑损伤和脾破裂.结论 小儿胸部损伤病情多样,正确及时的诊治措施是抢救患儿生命的关键.     

儿童重症急性胰腺炎的诊治进展

儿童重症急性胰腺炎的发病率较低,起病急骤凶险,且症状不典型,有些症状缺乏特异性,病死率相对较高.本文将对重症急性胰腺炎的诊断、治疗进展作一综述.     

遗传性代谢病诊治的过去和未来

遗传性代谢病或称先天性代谢缺陷 (InbornErrorMetabolism ,IEM) ,是参与体内代谢的某种酶、运载蛋白、膜或受体等的编码基因发生突变 ,使其编码的产物功能不良而引发代谢失常的一类疾病。这类疾病大多在新生儿、婴幼儿或儿童时期发病 ,有些虽在成年期发病 ,但在儿童时期已有潜在病变。故遗传性代谢病的诊断、防治属儿科范畴。遗传代谢病的种类很多 ,目前已报道的达 5 0 0余种 ,是人类疾病中病种最多的一类疾病。且随着检测诊断技术不断进步 ,还会发现更多的病种。由于每种病的发生率很低 ,又是临床工作者多感生疏的病 ,故诊断较困难。这…     

儿童严重急性呼吸综合征的诊治

20 0 2年 11月广东地区流行一种不明原因的肺炎 ,这种肺炎病情发展迅速 ,具有明显传染性 ,抗生素治疗无效 ,部分患者预后严重。经广东地区医学专家讨论 ,命名为非典型肺炎。此后非典型肺炎相继在香港地区和其他国家陆续报道 ,并受到医学界严密关注。临床及病原学科技工作者进行大量的研究 ,对此病的认识不断深入 ,致病原也逐步明朗。由于此病极易引起急性呼吸窘迫综合征 (ARDS) ,WHO将其命名为严重急性呼吸综合征 (SARS)。近期研究表明病原体为冠状病毒变异株[1] ,WHO也将其命名为SARS病毒。我院从 2 0 0 3年 2月 5日开始陆续收治一…     

Hyperchloraemic metabolic acidosis following open cardiac surgery

Aims: To describe acid–base derangements in children following open cardiac surgery on cardiopulmonary bypass (CPB), using the Fencl–Stewart strong ion approach. Methods: Prospective observational study set in the paediatric intensive care unit (PICU) of a university children''s hospital. Arterial blood gas parameters, serum electrolytes, strong ion difference, strong ion gap (SIG), and partitioned base excess (BE) were measured and calculated on admission to PICU. Results: A total of 97 children, median age 57 months (range 0.03–166), median weight 14 kg (range 2.1–50), were studied. Median CPB time was 80 minutes (range 17–232). Predicted mortality was 2% and there was a single non-survivor. These children showed mild metabolic acidosis (median standard bicarbonate 20.1 mmol/l, BE –5.1 mEq/l) characterised by hyperchloraemia (median corrected Cl 113 mmol/l), and hypoalbuminaemia (median albumin 30 g/l), but no significant excess unmeasured anions or cations (median SIG 0.7 mEq/l). The major determinants of the net BE were the chloride and albumin components (chloride effect –4.8 mEq/l, albumin effect +3.4 mEq/l). Metabolic acidosis occurred in 72 children (74%) but was not associated with increased morbidity. Hyperchloraemia was a causative factor in 53 children (74%) with metabolic acidosis. Three (4%) hyperchloraemic children required adrenaline for inotropic support, compared to eight children (28%) without hyperchloraemia. Hypoalbuminaemia was associated with longer duration of inotropic support and PICU stay. Conclusions: In these children with low mortality following open cardiac surgery, hypoalbuminaemia and hyperchloraemia were the predominant acid–base abnormalities. Hyperchloraemia was associated with reduced requirement for adrenaline therapy. It is suggested that hyperchloraemic metabolic acidosis is a benign phenomenon that should not prompt escalation of haemodynamic support. By contrast, hypoalbuminaemia, an alkalinising force, was associated with prolonged requirement for intensive care.     

新生儿高未结合胆红素血症与代谢性酸中毒的关系

目的　探讨代谢性酸中毒对新生儿高未结合胆红素血症的影响。方法　对 2 0 0例新生儿高未结合胆红素血症患儿进行血浆二氧化碳结合力 (CO2 CP)检测。结果　CO2 CP低于正常值 182例 (91% )。结论　及时发现并纠正代谢性酸中毒 ,有利于新生儿高未结合胆红素血症患儿黄疸的消退。