Anaesthetic implications of Costello syndrome

Costello syndrome is a disorder that primarily involves ectodermal tissues and is characterized by mental and growth retardation, distinctive coarse facies, redundant skin (neck, palms and soles), and papillomata (perioral, nasal and anal). Of primary concern to anaesthesiologists are potential airway difficulties related a short neck, macroglossia, hypertrophied tonsillar and supraglottic tissues, laryngeal papillomata and choanal atresia. A significant percentage of patients also have cardiac involvement which may manifest as congenital heart defects, arrhythmias, valvular dysfunction, or hypertrophic cardiomyopathy. Central nervous system involvement includes developmental delay and seizure disorders while endocrine abnormalities have been reported including hypothalamic-pituitary dysfunction resulting in hypopituitarism, hypothyroidism, and hypoadrenalism. The authors present a 2-year old child with Costello syndrome who required anaesthesia for direct laryngoscopy, rigid bronchoscopy, bilateral pressure equalization tubes and tonsillectomy. The perioperative implications of the syndrome are discussed.     

Outcome of reflux therapy on pediatric chronic sinusitis.

OBJECTIVE: The cause of pediatric chronic sinusitis is multifactorial, but nasal edema appears to be the initial pathologic step. The objective of this study is to evaluate gastronasal reflux as a possible cause of pediatric sinusitis. METHODS: Thirty children with chronic sinusitis were believed to be appropriate candidates for functional endoscopic sinus surgery. Children were evaluated retrospectively for their response to reflux therapy with regard to their sinus symptoms and avoidance of sinus surgery. RESULTS: Two of the 30 children were eventually excluded because they were taken to surgery for the specific purpose of contact point release. Chart review at 24-month follow-up indicated that 25 of the 28 children (89%) avoided sinus surgery. CONCLUSION: After reflux treatment, the number of children requiring sinus surgery was dramatically reduced. The results of this preliminary pediatric study indicate that gastronasal reflux should be evaluated and treated before sinus surgical intervention.     

Enteroatmospheric fistula management by endoscopic gastrostomy PEG tube

Management of small‐bowel fistulas which are in an open abdomen and have no soft tissue overlay or a fistula tract involves many complications and challenges. Controlling the local leakage of enteric contents has a central role in the success of medical treatment. There are several methods to deal with fistula discharge but unfortunately, the technical solutions only partially address such problems and a definitive management of fistula discharge still remains an insoluble challenge. We describe a simple and cheap method to control fistula leakage by using a percutaneous endoscopic gastrostomy tube.     

Effect of Aerobic Exercise Training on Maternal Weight Gain in Pregnancy: A Meta-Analysis of Randomized Controlled Trials

Background

Weight gains in pregnancy within the recommended guidelines are associated with healthy fetal and maternal outcomes; higher weight gains are associated with fetal macrosomia. This study was a systemic review of randomized controlled trials on the effect of aerobic training on maternal weight in pregnancy.

Methods

The study data source was publications through May 2012 in the MEDLINE (PubMed) database. The citation lists of randomized controlled trials on the effect of aerobic training and maternal weight were extracted. Data on participants'' characteristics, study quality, population, intervention, treatment outcome (maternal weight gain) were collected and analyzed.

Results

There were 11 randomized controlled studies using body weight (kg) as measure of treatment outcome. A total of 1177 subjects were recruited in the 11 studies. The mean± SD weight gain (kg) for the exercise (11.31± 7.44kg) and control (14.42± 6.60kg) groups; Meta-analysis result indicated significant effect of aerobic training on maternal weight (t= -7.580, p= .000) at p< 0.05.

Conclusion

It was concluded that aerobic training is an effective tool in maternal weight gain control in pregnancy. More randomized controlled trials are warranted.     

Abnormalities in brain biochemistry associated with lack of dystrophin: studies of the mdx mouse.

Biochemical abnormalities have been reported in dystrophin-deficient muscle of boys with Duchenne (severe Xp21) muscular dystrophy or in the murine (mdx) model of the disease. These abnormalities include altered energy metabolism and responses to osmotic shock. In contrast, the situation in brain is less well understood and it is probable that dystrophin is playing a different role (or roles) in this organ. In this study we conclude that the elevation in choline-containing compounds reported in mdx brain is confined to cerebellum and hippocampus in older (> 6 months) mice. We report alterations in glucose metabolism in mdx brain under normal, awake conditions, and a reduced response of brain metabolism to the gamma-aminobutyric acid(A) receptor agonist muscimol. Using brain cortical slices we found no difference in the response of dystrophic tissue to hypoosmotic shock, but increased, substrate-dependent oxygen consumption rates at low oxygen partial pressures.     

The use of 7-amino actinomycin D in identifying apoptosis: simplicity of use and broad spectrum of application compared with other techniques

The detection and quantitation of apoptotic cells is becoming increasingly important in the investigation of the role of apoptosis in cellular proliferation and differentiation. The pathogenesis of hematologic disorders such as aplastic anemia and the development of neoplasia are believed to involve dysregulation of apoptosis. To quantitate accurately the proportion of apoptosis cells within different cell types of a heterogeneous cell population such as blood or bone marrow, a method is required that combines the analysis of large numbers of cells with concurrent immunophenotyping of cell surface antigens. In this study, we have evaluated such a method using the fluorescent DNA binding agent, 7-amino actinomycin D (7AAD), to stain three diverse human cell lines, induced to undergo apoptosis by three different stimuli. Flow cytometric analysis defines three populations on the basis of 7AAD fluorescence and forward light scatter. We have shown by cell sorting and subsequent morphological assessment and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling that the populations defined by 7AAD represent live, apoptotic, and late-apoptotic/dead cells. This method is quick, simple, reproducible, and cheap and will be a valuable tool in the investigation of the role of apoptosis in normal physiology and in disease states.     

Nanotherapy silencing the interleukin‐8 gene produces regression of prostate cancer by inhibition of angiogenesis

Interleukin‐8 (IL‐8) is a pro‐angiogenic cytokine associated with aggressive prostate cancer (CaP). We detected high levels of IL‐8 in sera from patients with CaP compared with healthy controls and patients with benign prostatic hypertrophy. This study examines the role of IL‐8 in the pathogenesis of metastatic prostate cancer. We developed a biocompatible, cationic polylactide (CPLA) nanocarrier to complex with and efficiently deliver IL‐8 small interfering RNA (siRNA) to CaP cells in vitro and in vivo. CPLA IL‐8 siRNA nanocomplexes (nanoplexes) protect siRNA from rapid degradation, are non‐toxic, have a prolonged lifetime in circulation, and their net positive charge facilitates penetration of cell membranes and subsequent intracellular trafficking. Administration of CPLA IL‐8 siRNA nanoplexes to immunodeficient mice bearing human CaP tumours produced significant antitumour activities with no adverse effects. Systemic (intravenous) or local intra‐tumour administration of IL‐8 siRNA nanoplexes resulted in significant inhibition of CaP growth. Magnetic resonance imaging and ultrasonography of experimental animals demonstrated reduction of tumour perfusion in vivo following nanoplex treatment. Staining of tumour sections for CD31 confirmed significant damage to tumour neovasculature after nanoplex therapy. These studies demonstrate the efficacy of IL‐8 siRNA nanotherapy for advanced, treatment‐resistant human CaP.     

A longitudinal study of otitis media with effusion among 2- to 5-year-old African-American children in child care

OBJECTIVE: To prospectively document the prevalence of otitis media with effusion (OME) in 86 African-American children between ages 2 and 5 years. STUDY DESIGN: Eighty-six children in center-based child care whose ear status had been followed from infancy continued to be observed. Middle ear status was assessed by pneumatic otoscopy and tympanometry biweekly. RESULTS: The prevalence of OME decreased as children became older. The mean proportion of examinations demonstrating bilateral OME (BOME) ranged from 12% between 24 to 30 months to 4% between 54 to 60 months of age. The mean proportion of exams revealing bilateral normal ears increased from 77% at 24 to 30 months to 88% at 54 to 60 months of age. Although 60 children had experienced BOME that lasted 4 months or longer in the 6- to 24-month age period, only 8 of these children experienced at least 4 months of continuous BOME between 24 to 60 months. CONCLUSIONS: The proportion of time with BOME decreased progressively with increasing age in this population. Only 8 of 60 children who had experienced more than 4 consecutive months of BOME before 2 years of age continued to manifest persistent effusion or experience recurrences of prolonged BOME after 2 years of age.