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Background Sjogren-Larsson syndrome is a rare inherited neurocutaneous disorder characterized by congenital ichthyosis, spasticity, intellectual disability, seizures, and ophthalmologic changes. Most individuals with Sjogren-Larsson syndrome live well into adulthood and often require surgical intervention to manage their symptomatology. Aims The aim of this work was to review the clinical aspects of Sjogren-Larsson syndrome, highlight the unique anesthetic considerations associated with this disease, and provide practical recommendations about anesthetic management. Methods A retrospective case review from February 2013 to October 2019 was performed based on subject participation in a Sjogren-Larsson syndrome longitudinal study at the University of Nebraska Medical Center. Anesthetic and surgical records were reviewed for the following data: age, sex, relevant comorbid conditions, anesthetic induction and maintenance agents, intravenous and oral analgesics, muscle relaxants, and anesthetic-related complications. Results Fourteen patients with Sjogren-Larsson syndrome undergoing 48 anesthetic events were identified. A variety of anesthetic techniques was utilized. No serious adverse events were encountered. The most common clinical observations were related to the ichthyosis seen in Sjogren-Larsson syndrome, which led to difficulty in adherence of electrocardiogram leads and intravenous catheter dressings. Conclusions We found that anesthesia can be safely administered in patients with Sjogren-Larsson syndrome. Providers should be aware of anesthetic management issues in Sjogren-Larsson syndrome including challenges placing and securing lines and monitors secondary to the ichthyosis.

Introduction Pediatric craniofacial reconstruction has historically resulted in extensive blood loss necessitating transfusion. This single-center quality improvement initiative evaluates the impact of perioperative practice changes on the allogeneic transfusion rate for children 24 months and younger of age undergoing craniofacial reconstruction. Methods At project initiation, an appointed core group of anesthesiologists provided all intraoperative anesthetic care for patients undergoing craniofacial reconstruction. Standardized anesthetic guidelines established consistency between providers. Using the Plan-do-check-act methodology, practice changes had been implemented and studied over a 5-year period. Improvement initiatives included developing a temperature-management protocol, using a postoperative transfusion protocol, administering intraoperative tranexamic acid, and a preincisional injection of 0.25% lidocaine with epinephrine. For each year of the project, we acquired data for intraoperative and postoperative allogeneic transfusion rates. Results A cohort of 119 pediatric patients, ages 4-24 months, underwent anterior or posterior vault reconstruction for craniosynostosis at a tertiary children's hospital between March 2013 and November 2018. Intraoperative and postoperative transfusion of allogeneic blood products in this cohort decreased from 100% preintervention to 22.7% postintervention. Conclusions Interdepartmental collaboration and practice modifications using sequential Plan-do-check-act cycles resulted in a bundle of care that leads to a sustainable decrease in the rate of intraoperative and postoperative allogeneic blood transfusions in patients less than 24 months of age undergoing craniosynostosis repair. This bundle decreases the risk of transfusion-related morbidity for these patients. Other institutions looking to achieve similar outcomes can implement this project.

Neonates with laryngeal webs pose unique challenges to the anesthesiologist. We present a 2-day-old neonate with aphonia and stridor who underwent microdirect laryngoscopy. Intraoperatively, a Cohen type 4 laryngeal web was diagnosed not immediately amenable to resection. Therefore, the decision was made for endotracheal intubation and subsequent tracheostomy. After endotracheal intubation, there was acute respiratory compromise and oxygen desaturation that improved moderately after urgent tracheostomy. A chest radiograph revealed a large pneumothorax. Our experience suggests that in the presence of high-grade laryngeal webs, the possibility of intraoperative development of pneumothorax should be considered if respiratory difficulties are encountered.

The mechanization of field operations like seeding, spraying and harvesting in continuous zero-tillage may lead to a severe compaction of the surface layer of coarse textured tropical soils, especially when mulch is sparse or missing. Therefore, a 2 year (1982–1984) field experiment was initiated on an Alfisol in Nigeria to study the effect of tillage, mechanization and mulch on soil structure and physical properties. Three zero-tillage treatments and a plough treatment were compared. The disk-plough and one of the no-till treatments were highly mechanized: all the field work was performed with tractors and machines, and consisted of secondary bush clearing, crop cultivation and harvest. On the other two no-till treatments, the impact of machine load was reduced, wither by hand harvesting or by performing all field operations manually. These four tillage-traffic systems were either treated with mulch or left unmulched. There were four growing seasons, with maize ( Zea mays L.) as a test crop. After 2 years of zero-tillage the bulk density (BD) and penetration resistance (PR) were significantly greater on plots with high mechanization compared with hand treated plots. Plots with hand harvest but otherwise mechanized were in between. Because of the hard-setting nature of the soil, the plougheed plots with and without mulch exhibited a dramatic change in PR and BD during the season. On no-till the infiltration transmissivity ( A ) was greater and BD and PR were less in the mulched compared with the unmulched treatments. The gravel content of the topsoil was negatively correlated with BD and positively correlated with A . Geostatistical analysis revealed that within the experimental area there was a similar spatial distribution of gravel content and A after the first season. Because of the superimposing effect of gravel on BD, which cannot be accounted for by considering the gravel content per se, BD was adjusted by means of covariance analysis for evaluation of the treatment effects already mentioned. It was concluded that mechanization of a no-till system on sandy Alfisols may only be successful in the long run if appropriate measures like mulching, crop rotation and fallow systems are applied to regenerate soil structure and to enhance macroporosity.

We have used perturbed-angular-correlation (PAC) spectroscopy of dilute /sup 111/In dopants to characterize the local structure of YBa/sub 2/Cu/sub 3/O/sub 7-//sub x/ vs T from room temperature to 950 /sup 0/C. We obtain highly reproducible results with fine-grain powder samples that are prepared by nitrate evaporation and cooled very slowly after firing. These samples display orthorhombic YBa/sub 2/Cu/sub 3/O/sub 7/ x-ray diffraction patterns at room temperature and have a very sharp Meissner flux exclusion transition at T/sub c/ = 92.7 K. PAC spectra, which clearly indicate nontetragonal local symmetry, do not change at temperatures where diffraction measurements indicate an orthorhombic-to-tetragonal phase transition. We interpret this observation as evidence that the ''tetragonal'' phase is in these materials retains local orthorhombic order.

Eine chemische Analyse beinhaltet drei Elemente: Probenvorbereitung, analytische Messung und Datenverarbeitung. Hochentwickelt sind inzwischen Mestechnik und Datenverarbeitung; dagegen hat die Probenvorbereitung mit dieser Entwicklung nicht Schritt gehalten und ist das schwachste Glied im Verlauf einer chemischen Analyse.

(1945). Mail Questionnaire as a Research Problem. The Journal of Psychology: Vol. 20, No. 2, pp. 293-320.

The development of volatile compounds arising from radicals during intermediate reaction steps of the lipoxygenase-linoleic-acid-reaction (from soy) was investigated in model experiments with defined conditions. The results indicate as follows: Among the volatile compounds formed, hexanal has a special position. Its development is closely connected to the enzymatic formation of the 13-linoleic-acid-hydroperoxide because it is formed from the 13-linoleic-acid-peroxy-radical, which is a direct precursor of the 13-linoleic-acid-hydroperoxide. The other volatile products are apparently formed in the same way as by autoxidation. Their development is favoured by lack of oxygen, when the primarily formed linoleic-acid-radicals cannot react rapidly with the oxygen-radicals to form hydroperoxides. A small part of the volatile compounds is formed by autoxidation which always accompanies the enzymatic reaction.

The feasibility of designing and constructing a high explosive source which faithfully reproduces the direct- and airblast-induced ground motions resulting from a 1-kt nuclear surface burst has been investigated. A preliminary charge design incorporating subsurface and surface high explosive charges was developed, and shown to theoretically reproduce the desired simulation conditions. The subsurface charge employs techniques first used in the MINE THROW I simulation of the JOHNIE BOY nuclear event. The surface charge consists of an elevated disc of explosive (ANFO) with a thickness varying from 1 m to 0.1 m with a total diameter of about 40 m. Detonation spacings and an initiation system for this novel charge design were investigated. A complete fieldable charge was not constructed because of difficulties in experimentally determining the detonation properties of thin sheets of ANFO. Steps leading to a future fieldable charge which simulate airblast, ground motion, and cratering effects of a 1-kt nuclear surface burst are made, based on results of this initial effort. (Author)

During incubation of soja-lipoxygenase with linolic acid, volatile compounds are formed the development of which can be seen in two possible ways:from preformed linolic-acid-hydroperoxides splitproducts arise or volatile substances of different chemical nature are built depending on the reaction conditions like temperature, O2-pressue, partner-concentration etc. By trials with hydroperoxyde-decomposing enzymes (peroxidase) and by means of radio-active labelled linolic-acid-hydroperoxides the pathways mentioned above were investigated. The results indicate that the volatile compounds are built from by-products; n-hexanal was formed from these by-products as well as from decomposed hydroperoxide. The previously proposed reaction-scheme has this been ascertained by experimental means.

Three cases of listeriosis are presented: The first patient was a 72-year-old man, who had an operative removal of a thymom one and a half year ago. In 1983 he developed polyradiculitis and was treated with cortison. Three months later he developed meningeal symptoms. In the cerebrospinal fluid 10(5) per ml Listeria monocytogenes could be isolated. Under treatment with chloramphenicol and gentamycin he was cured. The second case was a female 84-year-old patient, who suddenly developed drowsiness and meningism. In the cerebrospinal fluid Listeria in low concentration was isolated. In spite of therapy with penicillin G and gentamycin in high dosage the patient got centralnervous spasms and died on the second day. The third patient was a premature born infant (31. week of pregnancy) with shortness of breath and hydrocephalus, that was treated with ventriculo-peritoneal shunts on both sides. In her fourth month she got a shunt-defect; Listeria in low concentration was isolated out of the cerebrospinal fluid. Treated with gentamycin intrathecal and amoxycillin she was successfully cured. Epidemiology, pathogenesis and new knowledge about diagnosis and therapy of Listeria are presented.

Soya- and oats-lipoxygenase (E.C. 1.13.1.13.) are incubated by 14C-marked linoleic acid. The volatile aldehydes arising thereby are isolated. The activity of the components separated by gaschromatography is written down by a printing indicator and the impulses/min are registered and printed out by a ratemeter. Thus the aldehydes which are produced by the enzymatic oxydation with lipoxygenase from the molecule of the linoleic acid can be determined. The composition of the mixture of aldehyde is calculated in mol-% from the measured impulses per peak. A possible origin of pathway is indicated for the main reaction products hexanal (soya-lipoxygenase) and non-trans-2-enal (oats-lipoxygenase).

view study has implications for the growing field of child socialization. Admitting the need for better methods, we believe none the less that our data support the hypothesis of class level stratification among 'teen age children. So far this idea, when advanced at all, has rested on fragmental case studies which have been made, in turn, the basis for rather sweeping generalizations. Quantification, along with clearer conceptual definition, are on the way and greatly needed. In comparison with what is known about individual personality variants, we know little about the simplest subgroupal structures, for example pairs, chains, cliques and factions. This is, we believe, a fertile field for the educational sociologist.