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.     

Remifentanilversus propofol for radio frequency treatment of atrial flutter

PURPOSE: Radio frequency treatment in cardiology generates short acute pain during the heating process. The present study evaluates two techniques used for sedation/analgesia for this procedure. METHODS: Two groups of 20 patients each were studied prospectively. Patients were randomized to receive sedation for the procedure using either a patient-controlled analgesia device with remifentanil (Group R), or a target controlled infusion of propofol (Group P). Patients in Group R had a basal infusion of remifentanil 0.02-0.04 microg x kg(-1) x min(-1) with self administered bolus doses of 0.3 microg x kg(-1) i.v. every minute as required, with a delivery time greater than 30 sec. Patients in Group P had an initial plasma target concentration set at 3-4 microg x mL(-1). RESULTS: Sedation scores were significantly higher in Group P, and two patients required supplementation with remifentanil and insertion of an laryngeal mask airway. Pain scores were higher in Group R, and two patients experienced muscular rigidity, one with transient apnea. Systolic blood pressure decreased significantly in Group P, and at the end of the procedure, PaCO(2) values were higher in that group (P < 0.01). Recovery time was significantly longer in Group P. Patient and physician satisfaction scores were similar in the two groups. CONCLUSIONS: A basal infusion of remifentanil plus remifentanil patient controlled analgesia and target controlled infusion of propofol were adequate but not optimal techniques for sedation/analgesia for radio frequency treatment of atrial flutter.