Lethal neonatal Menkes' disease with severe vasculopathy and fractures

A male neonate presented with an acute onset of severe intra-abdominal bleeding, haemorrhagic shock and multiple fractures leading to death on d 27. Menkes' disease was diagnosed at autopsy and confirmed by copper accumulation studies on cultured fibroblasts. Such an early onset of fatal complications in this condition has not been previously reported. New insights into the pathogenesis of Menkes' disease provided by DNA mutation analysis and difficulties in neonatal diagnosis are discussed. Menkes' disease should be considered in male infants with pathological fractures and other signs of connective tissue disease, even in the neonatal period.     

Hypoxaemia in infants with respiratory tract infections

Nineteen infants who were graduates from special care baby units underwent two overnight tape recordings of oxygen saturation (SaO2) and breathing movements; one during an upper (n = 12) or lower (n = 7) respiratory tract infection and the other when free of infection. Baseline SaO2 was lower during infection (median 99.6 vs 100%, p less than 0.01), with four patients having values (84.3-95.5%) below the normal lower limit for full-term infants (97%). The median number of apnoeic pauses was also lower during respiratory tract infection (4.7 vs 15.7/h, p less than 0.02). The median number of episodic desaturations (SaO2 less than or equal to 80%) did not change significantly (1.3 vs 1.9/h, p greater than 0.05), with the exception of one patient who had extremely increased values during infection for both apnoeic pauses (63/h) and desaturations (112/h). No infant, however, was considered clinically hypoxaemic. Clinically unsuspected hypoxaemia may thus occur during respiratory tract infection in a proportion of infants graduating from special care baby units. Such hypoxaemia may have potentially deleterious effects.     

Risk factors for early termination of breast feeding in Brazil

A prospective study was undertaken to identify possible factors related to the duration of breast feeding. Two hundred and thirty-eight mothers who had delivered normal single babies with birth weights greater than 2.5 kg and had initiated breast feeding were randomly selected at the maternity hospital, Hospital de Clinicas de Porto Alegre, Brazil, and followed by mail questionnaires until termination of breast feeding, or until the end of the first year. If no reply was received, telephone contact or home visits were made. The group of mothers who stopped breast feeding prior to the end of the third month was compared with those who extended breast feeding beyond three months with respect to socioeconomic, biological, environmental, medical and psychological factors. The variables with a significant coefficient of association with early termination of breast feeding were maternal education, past experience with breast feeding, help of a maid, help with housework provided by a relative, breast feeding orientation during prenatal care and encouragement from the husband. These factors act simultaneously, with interactions among them.     

The potential of phage therapy in cystic fibrosis: Essential human-bacterial-phage interactions and delivery considerations for use in Pseudomonas aeruginosa-infected airways

As antimicrobial-resistant microbes become increasingly common and a significant global issue, novel approaches to treating these infections particularly in those at high risk are required. This is evident in people with cystic fibrosis (CF), who suffer from chronic airway infection caused by antibiotic resistant bacteria, typically Pseudomonas aeruginosa. One option is bacteriophage (phage) therapy, which utilises the natural predation of phage viruses upon their host bacteria. This review summarises the essential and unique aspects of the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways. The current evidence regarding phage biology, phage-bacterial interactions, potential airway immune responses to phages, previous use of phages in humans and method of phage delivery to the lung are also summarised.     

Increased Müller cell density during diabetes is ameliorated by aminoguanidine and ramipril

Background: The Müller cell, the major glial cell in the retina, may be important in diabetes. The purpose of this project was to examine the localisation of glutamine synthetase in control and diabetic Müller cells and to determine whether the number of Müller cells is altered during diabetes. We also examined whether two experimental treatments of diabetes, aminoguanidine and ramipril, ameliorated these changes. Methods: Normal Sprague‐Dawley rats rendered diabetic by a single injection of streptozotocin (50 mg/kg) were treated with either aminoguanidine, ramipril or standard water. Following 12 weeks, animals were sacrificed, their eyes removed and the retinae processed for glutamine synthetase immunocytochemistry. The level of glutamine synthetase was quantified in control and diabetic animals and the number of Müller cells counted for each of the treatment groups. Results: In all retinae examined, glutamine synthetase labelled Müller cells along their entire cellular extent and endfeet were more intensely labelled. Following 12 weeks of diabetes, there was a small increase in the level of glutamine synthetase labelling in somata and endfeet compared with controls (ANOVA, P < 0.05). The number of Müller cells was increased following 12 weeks of diabetes (ANOVA, P < 0.0001). This effect was ameliorated by treatment with ramipril and aminoguanidine. Conclusions: These data suggest that Müller cells are altered in number following 12 weeks of diabetes. Moreover, the two experimental treatments were beneficial in preventing this change in Müller cells. Further work is required to establish the mechanisms underlying the change to Müller cells during diabetes.     

A Bar Code and Radio-Frequency Identification System for Transfusion Safety

This presentation will describe a pilot study of radio-frequency (RF) identification tags ("chips") that was conducted in parallel with standard procedures for the collection and testing of Red Blood Cells (Greater Chesapeake and Potomac Region, American Red Cross Biomedical Services, Baltimore, MD) and transfusion (Georgetown University Hospital, Washington, DC). The purpose of the study was to evaluate whether multi-write RF chips could be attached to blood bags, programmed, and used to facilitate the collection of information from (1) a blood bag manufacturer to (2) a blood collection center and, subsequently, to (3) a hospital transfusion service.     

脊髓损伤后神经细胞黏附分子的表达

目的:观察大鼠脊髓损伤后神经细胞黏附分子的表达,分析其与神经修复的关系。方法:实验于2006-02/05在兰州大学第二附属医院骨科研究所完成。成年雄性Wistar大鼠36只,分为空白对照组(n=3)、假手术组(n=3)和损伤组(n=30)。损伤组采用Allen法制成急性外伤模型。分别于术后1,3,5,7,10d麻醉下处死。假手术组仅实施手术不行打击,空白对照组不做任何处理,均在实验初处死。对各组大鼠在处死前以改良的Tarlov评分检测行为及肢体运动功能,观察伤后各时相点大鼠后肢的活动、肌力等,以此评定损伤后大鼠运动功能的恢复情况。测量各组各时间点脊髓灰质中阳性反应细胞并测其灰度值,用免疫组织化学的方法测定脊髓片神经细胞黏附分子的表达。结果:纳入动物数量为36只,损伤组死亡4只,最终进入结果分析32只。①损伤组脊髓损伤后1d改良的Tarlov评分低于空白对照组和假手术组(P<0.01),损伤后10d高于损伤后1d(P<0.01),但评分仍低于假手术组(P<0.01)。②损伤后1d肉眼可见脊髓组织肿胀明显,软脊膜紧绷。光镜下可见中心灰质血管破裂。白质神经纤维水肿,轴突与髓鞘之间间隙增大,1d后肿胀逐渐加重。损伤5d后脊髓肿胀逐渐消退,10d后轴突及髓鞘已退变成空泡。③空白对照组、假手术组脊髓灰质神经细胞黏附分子的表达甚少,损伤组1,3,5,7,10d表达增加,与空白对照组、假手术组比较,差异有显著性意义[(175.38±4.51),(163.15±5.98),(196.28±6.57),(217.42±2.36),(228.36±2.62),(248.31±3.47)(245.28±3.87),P<0.01]。结论:脊髓损伤后神经细胞黏附分子表达增加,提示神经细胞黏附分子可能参与损伤后神经的修复。