Natural history of retinal degeneration in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses.

Neuronal ceroid lipofuscinoses (NCL; Batten disease) are a group of inherited neurodegenerative diseases with a common set of symptoms including cognitive and motor decline and vision loss. Naturally occurring sheep models of CLN5 and CLN6 disease display the key clinical features of NCL, including a progressive loss of vision. We assessed retinal histology, astrogliosis, and lysosomal storage accumulation in CLN5 affected (CLN5-/-) and CLN6 affected (CLN6-/-) sheep eyes and age-matched controls at 3, 6, 12, and 18 months of age to determine the onset and progression of retinal pathology in NCL sheep. The retina of CLN5-/- sheep shows progressive atrophy of the outer retinal layers, widespread gliosis, and accumulation of lysosomal storage in retinal ganglion cells late in disease. In contrast, CLN6-/- retina shows significant atrophy of all retinal layers, progressive gliosis, and earlier accumulation of lysosomal storage. This study has highlighted the differential vulnerability of retinal layers and the time course of retinal atrophy in two distinct models of NCL disease. This data will be valuable in determining potential targets for ocular therapies and the optimal timing of these therapies for protection from retinal dysfunction and degeneration in NCL.

Substrate-Independent Epitaxial Growth of the Metal-Organic Framework MOF-508a.

Plasmachemical deposition is a substrate-independent method for the conformal surface functionalization of solid substrates. Structurally well-defined pulsed plasma deposited poly(1-allylimidazole) layers provide surface imidazole linker groups for the directed liquid-phase epitaxial (layer-by-layer) growth of metal-organic frameworks (MOFs) at room temperature. For the case of microporous [Zn (benzene-1,4-dicarboxylate)-(4,4'-bipyridine)0.5] (MOF-508), the MOF-508a polymorph containing two interpenetrating crystal lattice frameworks undergoes orientated Volmer-Weber growth and displays CO2 gas capture behavior at atmospheric concentrations in proportion to the number of epitaxially grown MOF-508 layers.

Rapid detection and characterisation of infectious bronchitis virus (IBV) from New Zealand using RT-PCR and sequence analysis.

AIMS: To develop a reverse transcriptase-polymerase chain reaction (RT-PCR) assay to detect infectious bronchitis virus (IBV) from commercially-raised poultry in New Zealand and compare results with those from virus isolation. To characterise the IBV isolates using sequence analysis. METHODS: Pooled tissue samples (trachea, kidney, caecal tonsils and cloacal swabs) from 164 broiler and 53 layer flocks located throughout New Zealand were collected in transport medium containing antibiotics. Tissues were homogenised and the resultant supernatant used directly in a RT-PCR assay, and also inoculated into the allantoic cavity of 10-day-old embryonated eggs for virus isolation. Primers for the RT-PCR were selected from an area close to the N-terminus of the S1 (spike) gene and bracketed the hypervariable region 1 (HVR 1). The RT-PCR amplimers were sequenced from both termini, and alignment was constructed and analysed. RESULTS: From the 217 field samples that were subjected to RT-PCR, 42 (19%) were positive. Twenty-nine (69%) of these RT-PCR-positive, and none of the RT-PCR-negative, samples yielded virus by isolation in chicken embryos. A phylogenetic tree constructed from these amplimers, that spanned the HVR of the S1 gene, revealed the IBV isolates clustered into two demarcated groups which had <60% homology. It is likely that the isolates of one group were derived from the live attenuated vaccine commonly used in New Zealand. CONCLUSIONS: The RT-PCR assay exhibited higher sensitivity than virus isolation and could be used for rapid diagnosis of IBV in the field. The prevalence of IBV appears to be surprisingly high in New Zealand although the use of pooled samples in the study did not allow accurate calculation of the prevalence in birds. Sequence analysis of a hypervariable region from the S1 gene was informative for the differentiation of closely-related strains.

The child and the fear of death.

The central hypothesis of this paper is that the innate fear of death in the human being is universal and that the child, least of all, is immune to death fear and its symbolic representation. This cuts across all ages and developmental levels. This paper is not concerned with the empirical knowledge of death, an area that has been extensively explored by others such as Nagy (1948), Piaget (1929), and Anthony (1940).Examination of the child and his relationship to death is important in order to reach the truth and understand the human meaning of the fear of death.The child's conception of himself and his relationship to the world is an ironic paradox. On one hand, he feels endowed with magical feelings of omnipotence. This feeling is the main defense against the fear of death. On the other hand, his wishes, both benevolent and malevolent, have power independent of him to influence events. The concept of chance is alien, and the differentiation between objective and wishful causation is obscured. Thus, the way in which the child perceives his world makes the terror of death more formidable.SEVERAL CONCLUSIONS ARE REACHED IN THIS PAPER: (1) that even in childhood, loss, endings, separations, and death are core concerns of the individual; (2) that fear of death in children is intensified by the absence of the intellectual equipment and the absence of the necessary defense mechanisms essential for comprehending the experience of loss; and (3) that repression of the fear of death is an evolutionary process which has its origin in childhood.

A suggested schema for utilization review for a Community Mental Health Center.

Three record-keeping models were examined by an interdisciplinary committee at the Rochester Mental Health Center to see which would best meet the needs for a standardized record format that could be used by an outpatient comprehensive community mental health center. The systems examined were: (1) Model Criteria Set, (2) Problem Oriented Record, and (3) a Combination Diagnosis and Standardized Problem List. A modification of the Combination System used at the Cleveland Metropolitan General Hospital was developed. Using this tool, an audit committee began the process of Utilization Review. It is a suggested schema to other outpatient mental health centers who are in the process of developing a utilization review process.