Distribution and characterization of Poleroviruses affecting food legumes in Central/West Asia and North Africa (CWANA) region and Australia

During the past 15 years, surveys to identify virus diseases affecting cool-season food legume crops in Australia and 11 CWANA countries (Algeria, China, Egypt, Ethiopia, Lebanon, Morocco, Sudan, Syria, Tunisia, Uzbekistan and Yemen) were conducted. More than 20,000 samples were collected and tested for the presence of 14 legume viruses by the tissue-blot immunoassay (TBIA) using a battery of antibodies, including the following Luteovirus monoclonal antibodies (McAbs): a broad-spectrum legume Luteovirus (5G4), BLRV, BWYV, SbDV and CpCSV. A total of 195 Luteovirus samples were selected for further testing by RT-PCR using 7 primers (one is degenerate, and can detect a wide range of Luteoviridae virus species and the other six are species-specific primers) at the Virology Laboratory, QDAF, Australia, during 2014. A total of 145 DNA fragments (represented 105 isolates) were sequenced. The following viruses were characterized based on molecular analysis: BLRV from Lebanon, Morocco, Tunisia and Uzbekistan; SbDV from Australia, Syria and Uzbekistan; BWYV from Algeria, China, Ethiopia, Lebanon, Morocco, Sudan, Tunisia and Uzbekistan; CABYV from Algeria, Lebanon, Syria, Sudan and Uzbekistan; CpCSV from Algeria, Ethiopia, Lebanon, Morocco, Syria and Tunisia, and unknown Luteoviridae species from Algeria, Ethiopia, Morocco, Sudan, Uzbekistan and Yemen. This study has clearly shown that there are a number of Polerovirus species, in addition to BWYV, all can produce yellowing/stunting symptoms in pulses (e.g. CABYV, CpCSV, and other unknown Polerovirus species). Based on our knowledge this is the first report of CABYV affecting food legumes. Moreover, there was about 95% agreement between results obtained from serological analysis (TBIA) and molecular analysis for the detection of BLRV and SbDV. Whereas, TBIA results were not accurate when using CpCSV and BWYV McAbs . It seems that the McAbs for CpCSV and BWYV used in this study and those available worldwide, are not virus species specific. Both antibodies, reacted with other Polerovirus species (e.g. CABYV, and unknown Polerovirus). This highlights the need for more accurate characterization of existing antibodies and where necessary the development of better, virus-specific antibodies to enable their use for accurate diagnosis of Poleroviruses.

Responses of fruit flies (Tephritidae: Dacinae) to novel male attractants in north Queensland, Australia, and improved lures for some pest species

Male fruit fly attractants, cue-lure and methyl eugenol (ME), have been successfully used for the last 50 years in the monitoring and control of Dacini fruit flies (Bactrocera and Dacus species). However, over 50% of Dacini are non-responsive to either lure, including some pest species. A new lure, zingerone, has been found to weakly attract cue- and ME-responsive species in Malaysia. In Australia it attracted a weakly cue-responsive minor pest Bactrocera jarvisi (Tryon) and three non-responsive' species. Similar compounds were tested in Queensland and attracted cue- and ME-responsive species and two non-responsive' species. In this study, 14 novel compounds, including raspberry ketone formate (RKF) (Melolure) and zingerone, were field tested in comparison with cue-lure and ME at 17 sites in north Queensland. The most attractive novel lures were isoeugenol, methyl-isoeugenol, dihydroeugenol and zingerone. Several non-responsive' species responded to the new lures: Bactrocera halfordiae (Tryon), a species of some market access concern, was most attracted to isoeugenol; B.barringtoniae (Tryon), B.bidentata (May) and B.murrayi (Perkins) responded to isoeugenol, methyl-isoeugenol and dihydroeugenol; two new species of Dacus responded to zingerone. Bactrocera kraussi (Hardy), a cue-responsive minor pest in north Queensland, was significantly more attracted to isoeugenol than cue-lure. The cue-responsive D.absonifacies (May) and D.secamoneaeDrew were significantly more attracted to zingerone than cue-lure. Bactrocera yorkensisDrew & Hancock, a ME-responsive species was significantly more attracted to isoeugenol, methyl-isoeugenol and dihydroeugenol than ME. The preferential response to RKF or cue-lure was species specific. Six species were significantly more attracted to RKF, including the pests B.tryoni (Froggatt), B.frauenfeldi (Schiner) and minor pest B.bryoniae (Tryon); eight species were significantly more attracted to cue-lure including the pest B.neohumeralis (Hardy). These findings have significance in the search for optimal male lures for pest species elsewhere in the world.

Vedestä vai Hengestä? : Uuras Saarnivaaran kasteteologia

Of water or the Spirit? Uuras Saarnivaara s theology of baptism The aim of the study was to investigate PhD and ThD Uuras Saarnivaara s views on baptism as well as their possible changes and the reasons for them. Dr Saarnivaara said himself that he searched for the truth about the relationship between baptism and faith for decades, and had faltered in his views. The method of this research is systematic analysis. A close study of the source material shows that Dr Saarnivaara s views on baptism have most likely changed several times. Therefore, special attention was paid to the time periods defined by when his literary works were published. This resulted in revealing the different perspectives he had on baptism. The fact that Dr Saarnivaara worked on two continents Europe and North America added a challenge to the research process. At the beginning of the research, I described Dr Saarnivaara s phases of life and mapped out his vast literary production as well as presented his theological basis. Saarnivaara s theological view on the means of grace and their interrelation in the church was influenced by the Laestadian movement, which caused him to adopt the view that the Holy Spirit does not dwell in the means of grace, but in the believers. Thus the real presence of Christ in the means of grace is denied. God s word is divided into Biblical revelation and proclamation by believers through the means of grace. Also, the sacraments are overshadowed by the preached word. Because grace is received through the word of the gospel preached publicly or privately by a believer, the preacher s status gains importance at the expense of the actual means of grace. Saarnivaara was intrigued by the content of baptism from the time he was a student until the end of his life. As a young theologian, he would adopt the opinions of his teachers as well as the view of the Evangelical Lutheran Church of Finland, which at the time was dominated by the pietistic movement and the teachings of J. T. Beck. After Saarnivaara had converted to the Laestadian movement, moved to the United States and started his Luther research, he adopted a view on baptism which was to a great extent in accordance with Luther and the Lutheran Symbolical Books. Saarnivaara considered his former views on baptism unbiblical and publicly apologised for them. In the 1950s, after starting his ministry within the Finnish neopietistic movements, Saarnivaara adopted a Laestadian-neopietistic doctrine of baptism. During his Beckian-pietistic era, Saarnivaara based his baptism theology on the event of the disciples of Jesus being baptised by John the Baptist, the revival of Samaria in the Book of Acts and the conversion of Cornelius and his family, all cases where the receiving of the Holy Spirit and the baptism were two separate events in time. In order to defend the theological unity of the Bible, Saarnivaara had to interpret Jesus teachings on baptism in the Gospels and the teachings of the Apostles in the New Testament letters from a viewpoint based on the three events mentioned above. During his Beckian-pietistic era, the abovementioned basic hermeneutic choice caused Saarnivaara to separate baptism by water and baptism by the Holy Spirit in his salvation theology. Simultaneously, the faith of a small child is denied, and rebirth is divided into two parts, the objective and the subjective, the latter being moved from the moment of baptism to a possible spiritual break-through at an age when the person possesses a more mature understanding. During his Laestadian-Lutheran era, Saarnivaara s theology of baptism was biblically consistent and the same for all people regardless of the person s age. Small children receive faith in baptism through the presence of Christ. The task of other people s faith is limited to the act of bringing the child to the baptism so that the child may receive his/her own faith from Christ and be born again as a child of God. The doctrine of baptism during Saarnivaara s Laestadian-neopietistic era represents in many aspects the emphases he presented during his first era, although they were now partly more radical. Baptism offers grace; it is not a means of grace. Justification, rebirth and salvation would take place later on when a person had reached an age with a more mature understanding through the word of God. A small child cannot be born again in baptism because being born again requires personal faith, which is received through hearing and understanding the law and the gospel. Saarnivaara s views on baptism during his first and third era are, unlike during his second era, quite controversial. The question of the salvation of a small child goes unanswered, or it is even denied. The central question during both eras is the demand of conversion and personal faith at a mature age. The background for this demand is in Saarnivaara s anthropology, which accentuates man s relationship to God as an intellectual and mental matter requiring understanding, and which needs no material instruments. The two first theological eras regarding Saarnivaara s doctrine of baptism lasted around ten years. The third era lasted over 40 years until his death.

Viimeinen matka : Toimintatutkimus kirkollisen hautauskentän professiostruktuurista

This study is a qualitative examination of the professional structure of the ecclesias-tical funeral field. The research material is based on 13 funeral cases in the archdio-cese. The researcher participated in all the funerals and memorial events, interviewed the closest survivors, the officials of the funeral agency and the ecclesiastical actors. The material was collected by means of observation and recording of the interviews, and was later transcribed and analyzed. The actors in this study are the survivors, the funeral agencies and the church. The survivors act as the buyers and users of the products (funeral services) who require both the funeral agencies and the church to assist them with the problems that the death has caused. The numbers of actions related to the death and to the funerals - the rituals of death - are placed on the action field, which in this study is called the funeral field. In this field, the researchquestion focused space and power, and the actions on the funeral field are highly ritualized. The theoretical model comes from Pierre Bourdieu. The study showed an action structure on the funeral field in which the survivors first contacted a funeral agency, which then contacted the other actors of the field, re-served the date and place for the funeral, and organised the funeral arrangements. The funeral agencies arranged an opportunity for the survivors to have a last look at the deceased when he or she was placed in the coffin, and they held a moment of the prayer (if desired) before removing the deceased from the hospital's chapel. The sur-vivors contacted the pastor of the funeral much later. The pastor also participated in the memorial event. The survivors contacted the church musician through via pastor. In some cases, the survivors had neither met nor even seen the musician prior to the actual funeral service. Still, the music was of great importance to the survivors. In the research interviews, tensions emerged to some extent between the funeral agencies and the ecclesiastical actors; these actors attempted to resolve these tensions through organising negotiations. In the beginning of the 20th century, the family took an active part in the preparations of the deceased and in the arrangements of the funerals, whereas this study showed that these days, survivors often transfer the preparations to the funeral agencies. The professional side of the funeral field has grown. The funeral agencies can be seen as providers of full services that act on the survivors' behalf, aspiring to high individu-ality and aiming to fulfil the survivors' wishes. In practise, the role of the church in carrying out the last journey was reduced in the research cases to the actual funeral. In several cases, the pastor or the cantor of the funeral had never before seen the per-son in the coffin at any stage of life or death. The proportion of cremations in funeral cases has increased rapidly, however, special issues related to these cremations (such as the possibility of holding a funeral service for the already cremated deceased) have seen little consideration in the church. In the church's liturgies on funeral rites, cremation is frequently overlooked. The pastors or the cantors did not participate in either the burial of the funeral urn or in the scattering of the deceased's ashes. The verger took care of it. The parishes had no adopted standard practices for cremations, yet in each case for the survivors that moment was crucial.

"Towards 'a real reunion'?" : Archbishop Aleksi Lehtonen's efforts for closer relations with the Church of England 1945–1951

Mika KT Pajusen väitös "Towards 'a real reunion'?" – Archbishop Aleksi Lehtonen's efforts for closer relations with the Church of England 1945–1951 on yleiseen kirkkohistoriaan lukeutuva tutkimus Englannin kirkon ja Suomen evankelis-luterilaisen kirkon välisistä suhteista Aleksi Lehtosen arkkipiispakaudella 1945–1951. Suhteita on tutkittu kolmesta näkökulmasta: ekumeenisesta, poliittisesta ja kirkkopoliittisesta. Tutkimuskausi alkaa pastori H.M. Waddamsin joulukuussa 1944 Suomeen tekemän vierailun jälkimainingeista ja päättyy arkkipiispa Lehtosen kuolemaan pääsiäisenä 1951. Kirkollisten suhteiden kehitystä rytmittivät lukuisat vierailut, jotka osoittivat Englannin kirkon asenteen muuttumisen sodan aikaisesta neuvostomyönteisyydestä kylmän sodan aikaiseen täysin vastakkaiseen kantaan. Englantilaiset vieraat kohtasivat Suomessa sekä kirkon että yhteiskunnan ylimmän johdon. Molemmat maat olivat valmiita tukemaan hyviä kirkollisia suhteita tilanteen niin salliessa, joskaan eivät kovin suunnitelmallisesti. Suomen evankelis-luterilainen kirkko käytti hyviä suhteita Englannin kirkkoon saadakseen tukea ja ymmärrystä omalle kirkolleen ja yhteiskunnalleen kokemaansa Neuvostoliiton uhkaa vastaan erityisesti vaaran vuosina 1944–1948. Englannin kirkko halusi tukea suomalaista sisarkirkkoaan, mutta varoi, ettei tuottaisi tuellaan enemmän haittaa kuin hyötyä suhteessa Neuvostoliittoon. Sodan jälkeinen ekumeeninen jälleenrakentaminen lähensi kirkkoja toisiinsa. Lehtonen pyrki jatkamaan 1930-luvun kirkkojen välisiä, ehtoollisvieraanvaraisuuden saavuttaneita neuvotteluita kohti täyttä kirkollista yhteyttä. Häntä motivoi sekä evankelis-katolinen teologia että pyrkimys tukea oman maan ja kirkon läntisiä yhteyksiä. Tämä haastoi Englannin kirkon ekumeenisen linjan, joka Suomen kirkon sijasta pyrki jatkamaan neuvotteluja Tanskan, Norjan ja Islannin luterilaisten kirkkojen kanssa, joilla ei vielä ollut virallista ekumeenista sopimusta Englannin kirkon kanssa. Lehtosen pyrkimyksistä huolimatta Englannin kirkko päätyi jättämään Suomen tilanteen hautumaan. Sillä se tarkoitti suhteiden koetinkivenä olleen historiallisen piispuuden leviämistä läpi Suomen kirkon ennen kuin katsoi olevansa valmis jatkamaan kohti täyttä kirkollista yhteyttä. Molemmissa kirkoissa vaikutti pieni, innokkaiden, lähempiä suhteita toivoneiden kirkollisten vaikuttajien ydinjoukko. Englantilaisia Suomen-ystäviä motivoi tarve auttaa Suomea hankalassa poliittisessa tilanteessa. Suomessa arkkipiispa Lehtonen tuki korkeakirkollista liturgista liikettä, jolla oli läheinen yhteys anglikaanisuuteen, mutta joka sai vastaansa vanhoilliset pietistit. Suomen kirkon yleinen mielipide asettui etupäässä pietistiselle kannalle, jolle anglikaanisuus näyttäytyi teologisesti sekä liian katolisena että liian reformoituna. Kirkolliset suhteet tasaantuivat vuoden 1948 Lambeth-konferenssin jälkeen, joka rohkaisi anglikaanisia kirkkoja hyväksymään 1930-luvun neuvottelujen lähempiin kirkollisiin suhteisiin tähtäävät suositukset. Lehtonen näytti tyytyvän tähän. Samaan aikaan lähempää kirkollista kanssakäymistä tukenut ekumeeninen jälleenrakennus tuli tiensä päähän. Lehtonen jatkoi läheisempien suhteiden edistämistä, mutta hänen intonsa hiipui yhdessä heikkenevän terveydentilan kanssa. Osoituksena Lehtosen linjan kapeudesta Suomen evankelis-luterilaisen kirkon piispoista ei löytynyt hänen kuoltuaan ketään, joka olisi jatkanut hänen aktiivista anglikaanimyönteistä linjaansa.

Der Weg in den Holocaust : Mit Bildern aus dem Leben der juedischen Gemeinde in Schmallenberg.

Photocopies of a catalogue for an exhibition in Schmallenberg on occasion of the town���s 750th anniversary in November 1994.

The church must become the people of Soweto : Manas Buthelezi’s existential-Christocentric understanding of the church

The aim of this study has been to discern what Manas Buthelezi (1935-), a black South African Lutheran theologian and later also a bishop, regards as the requirements a church has to fulfill in order to be credible in the apartheid society. Buthelezi’s dissertation and several articles written between the years 1968 and 1993 are the sources of this study. Also the lectures held in Heidelberg in 1972 are referred to. Systematic analysis is the method used. The question of the credibility of the church is studied through three concepts that play an important role in Buthelezi’s ecclesiological thought, namely the wholeness of life, incarnation and liberation. The notion of the wholeness of life stems from the African tradition. Buthelezi takes the concept into the Christian church: the church should realize that God is the Creator of all life and Christ the lord of every aspect of human existence. Life is one entity coram Deo. However, the church is not to become the world; solidarity between the two must remain critical as the church is also called to play a prophetic role in the society. The church is in an open relationship with the world. It has a unique message of forgiveness and reconciliation. Nevertheless, the message is not a possession of the church but it is addressed to the whole world. The meaning of incarnation comes close to that of the wholeness of life. Following the example of Christ’s incarnation, the church must become human in the reality of the people. The church in Soweto is to become the people of Soweto, that is, the church must become as vulnerable as the people are. An incarnate church cannot be immune to the oppression that people experience, because the people are the church. The church is therefore bound to suffer. Buthelezi’s theology of the cross is pragmatic: the suffering of the church aims at the liberation of the oppressed. At times the physical presence of the church by the side of the suffering people is the only way to preach the incarnate gospel. In the South Africa of the late 1960s onwards the liberation of the oppressed black people was high on the agenda of Black Theology. As a leader of the early South African Black Theology, Buthelezi is concerned about the racial injustice in his country. He urges the churches to join the struggle against it as one people of God. The notions of liberation and the wholeness of life emerge in Buthelezi’s holistic understanding of liberation that involves the inner liberation of the black spirit and the liberation of the economic, social and political aspects of life. Interpreting Tillich’s correlation method in the South African situation, and also paralleling other liberation theologians, Buthelezi takes the existential situation of the people as the starting point for liberation. The gospel has to respond to the existential questions of people. The church is called to work for the liberation of society but it must also be liberated itself. Buthelezi initiated the LWF statement on the status confessionis in South Africa (1977). In line with the statement, he calls for church unity on the human level. For the unity to be true, it has to be experienced on the grassroots’ level. All the three concepts covered urge the church to come down from any ivory tower and out of any spiritual haven it might hide in. A lot of the credibility of the church derives from the behavior of the people. Buthelezi’s concentration on how the people who constitute the church should live their faith leaves less attention to how God constitutes the church. I have labeled Buthelezi’s understanding of the church existential-Christocentric due to the emphasis he lays on the need of the church to take the existential situation of the people seriously and on the other hand, on Christ as the exemplar for the church.

Arts, gentrification, and planning for creativity

Recent decades have seen an almost obsessive focus on creativity in an urban development context. Yet, creativity has come to be prized not so much for the intrinsic values of imagination, innovation and experimentation as for the possibility to exploit these qualities as a means of urban revitalization and wealth generation. This policy emphasis has both contributed to the misplaced assumption that artistic activity causes gentrification and displacement while, at the same time, often setting in motion programs that are detrimental to the creative environments such policies claim to support. It is time to end the current approach to creative city planning, which treats the arts as amenities to catalyze land development and lure upscale consumption.

Cortinarius subgenus Telamonia p.p. in North Europe

Cortinarius is the largest genus of Agaricales with a worldwide distribution. So far, over 4000 Cortinarius names and combinations have been published. Cortinarius spp. form ectomycorrhizae with different trees and shrubs. A majority of the Cortinarius species have narrow ecological preferences and many form ectomycorrhiza with only one or few host species. The subgenus Telamonia sensu lato (s. lat.), comprising the greatest number of species, is the most poorly known of the subgenera of Cortinarius. The centre of diversity is in the northern hemisphere, although some species of the group are also recognized in the southern hemisphere. The aim of this thesis was to study the taxonomy of Cortinarius subgenus Telamonia p.p. species based on morphological and molecular data, as well as to study the ecology and distribution of the species in North Europe. The taxonomical problems encountered and the difficulty in finding and studying all the relevant names and types slowed down the study. The diversity of the subgenus Telamonia s. lat. in North Europe (excluding sect. Hydrocybe, Icrustati and Anomali) was found to be far greater than previously thought. Even many of the common species have not yet been described. So far, ca. 200 species have been recognised from the Nordic countries, but the sampling in most groups does not cover the whole diversity and especially the southern deciduous forest species are underrepresented in our study. In most cases phylogenetic (only based on ITS data) and morphological species recognition were in concordance, but in a few cases morphologically delimited species had almost identical ITS sequences, raising the question as to whether ITS is always variable enough for species recognition. The opposite situation, in which a morphologically uniform species included two phylogenetically distinct lineages, however, was also encountered, suggesting the possibility of cryptic species in Cortinarius. In our studies no taxa below species level were recognised and the aforementioned results indicate that presumably they can only be recognised genetically. Based on our preliminary results a revision of the infrageneric classification in Cortinarius subgenus Telamonia s. lat. is needed, and more sections should be established for a meaningful and functional classification. Many groups have turned out to be artificial, and it seems evident that many characteristics have been over- or underemphasised. Many morphological characteristics, however, are useful in the identification of telamonioid species and e.g. some spore characteristics have often been overlooked. Our studies have concentrated on North Europe, but we have found some similarities with North European and North American taxa.

Phylogeography and hybrid swarms: history of brackish water bivalve diversity in North European marginal seas

This study addressed the large-scale molecular zoogeography in two brackish water bivalve molluscs, Macoma balthica and Cerastoderma glaucum, and genetic signatures of the postglacial colonization of Northern Europe by them. The traditional view poses that M. balthica in the Baltic, White and Barents seas (i.e. marginal seas) represent direct postglacial descendants of the adjacent Northeast Atlantic populations, but this has recently been challenged by observations of close genetic affinities between these marginal populations and those of the Northeast Pacific. The primary aim of the thesis was to verify, quantify and characterize the Pacific genetic contribution across North European populations of M. balthica and to resolve the phylogeographic histories of the two bivalve taxa in range-wide studies using information from mitochondrial DNA (mtDNA) and nuclear allozyme polymorphisms. The presence of recent Pacific genetic influence in M. balthica of the Baltic, White and Barents seas, along with an Atlantic element, was confirmed by mtDNA sequence data. On a broader temporal and geographical scale, altogether four independent trans-Arctic invasions of Macoma from the Pacific since the Miocene seem to have been involved in generating the current North Atlantic lineage diversity. The latest trans-Arctic invasion that affected the current Baltic, White and Barents Sea populations probably took place in the early post-glacial. The nuclear genetic compositions of these marginal sea populations are intermediate between those of pure Pacific and Atlantic subspecies. In the marginal sea populations of mixed ancestry (Barents, White and Northern Baltic seas), the Pacific and Atlantic components are now randomly associated in the genomes of individual clams, which indicates both pervasive historical interbreeding between the previously long-isolated lineages (subspecies), and current isolation of these populations from the adjacent pure Atlantic populations. These mixed populations can be characterized as self-supporting hybrid swarms, and they arguably represent the most extensive marine animal hybrid swarms so far documented. Each of the three swarms still has a distinct genetic composition, and the relative Pacific contributions vary from 30 to 90 % in local populations. This diversity highlights the potential of introgressive hybridization to rapidly give rise to new evolutionarily and ecologically significant units in the marine realm. In the south of the Danish straits and in the Southern Baltic Sea, a broad genetic transition zone links the pure North Sea subspecies M. balthica rubra to the inner Baltic hybrid swarm, which has about 60 % of Pacific contribution in its genome. This transition zone has no regular smooth clinal structure, but its populations show strong genotypic disequilibria typical of a hybrid zone maintained by the interplay of selection and gene flow by dispersing pelagic larvae. The structure of the genetic transition is partly in line with features of Baltic water circulation and salinity stratification, with greater penetration of Atlantic genes on the Baltic south coast and in deeper water populations. In all, the scenarios of historical isolation and secondary contact that arise from the phylogeographic studies of both Macoma and Cerastoderma shed light to the more general but enigmatic patterns seen in marine phylogeography, where deep genetic breaks are often seen in species with high dispersal potential.

Structural-stratigraphic reconstruction of the lower Whakataki formation, north island, New Zealand

This thesis details a Miocene aged sedimentary rock formation located in north island New Zealand. Mapping, stratigraphic logging and petrographic analysis of the rock formation ascertained that it was deposited in a deep-marine, tectonically active region. The work details the make-up of the sedimentary rocks using geochemistry and microscopy to define their origin. This definition was used to interpret the depositional model of the sediments detailing how they were transported and how they were emplaced.

Neurological manifestations and molecular genetics of acute intermittent porphyria in North Western Russia

Acute intermittent porphyria (AIP, MIM #176000) is an inherited metabolic disease due to a partial deficiency of the third enzyme, hydroxymethylbilane synthase (HMBS, EC: 4.3.1.8), in the haem biosynthesis. Neurological symptoms during an acute attack, which is the major manifestation of AIP, are variable and relatively rare, but may endanger a patient's life. In the present study, 12 Russian and two Finnish AIP patients with severe neurological manifestations during an acute attack were studied prospectively from 1995 to 2006. Autonomic neuropathy manifested as abdominal pain (88%), tachycardia (94%), hypertension (75%) and constipation (88%). The most common neurological sign was acute motor peripheral neuropathy (PNP, 81%) often associated with neuropathic sensory loss (54%) and CNS involvement (85%). Despite heterogeneity of the neurological manifestations in our patients with acute porphyria, the major pattern of PNP associated with abdominal pain, dysautonomia, CNS involvement and mild hepatopathy could be demonstrated. If more strict inclusion criteria for biochemical abnormalities (>10-fold increase in excretion of urinary PBG) are applied, neurological manifestations in an acute attack are probably more homogeneous than described previously, which suggests that some of the neurological patients described previously may not have acute porphyria but rather secondary porphyrinuria. Screening for acute porphyria using urinary PBG is useful in a selected group of neurological patients with acute PNP or encephalopathy and seizures associated with pain and dysautonomia. Clinical manifestations and the outcome of acute attacks were used as a basis for developing a 30-score scale of the severity of an acute attack. This scale can easily be used in clinical practice and to standardise the outcome of an attack. Degree of muscle weakness scored by MRC, prolonged mechanical ventilation, bulbar paralysis, impairment of consciousness and hyponatraemia were important signs of a poor prognosis. Arrhythmia was less important and autonomic dysfunction, severity of pain and mental symptoms did not affect the outcome. The delay in the diagnosis and repeated administrations of precipitating factors were the main cause of proceeding of an acute attack into pareses and severe CNS involvement and a fatal outcome in two patients. Nerve conduction studies and needle EMG were performed in eleven AIP patients during an acute attack and/or in remission. Nine patients had severe PNP and two patients had an acute encephalopathy but no clinically evident PNP. In addition to axonopathy, features suggestive of demyelination could be demonstrated in patients with severe PNP during an acute attack. PNP with a moderate muscle weakness was mainly pure axonal. Sensory involvement was common in acute PNP and could be subclinical. Decreased conduction velocities with normal amplitudes of evoked potentials during acute attacks with no clinically evident PNP indicated subclinical polyneuropathy. Reversible symmetrical lesions comparable with posterior reversible encephalopathy syndrome (PRES) were revealed in two patients' brain CT or MRI during an acute attack. In other five patients brain MRI during or soon after the symptoms was normal. The frequency of reversible brain oedema in AIP is probably under-estimated since it may be short-lasting and often indistinguishable on CT or MRI. In the present study, nine different mutations were identified in the HMBS gene in 11 unrelated Russian AIP patients from North Western Russia and their 32 relatives. AIP was diagnosed in nine symptom-free relatives. The majority of the mutations were family-specific and confirmed allelic heterogeneity also among Russian AIP patients. Three mutations, c.825+5G>C, c.825+3_825+6del and c.770T>C, were novel. Six mutations, c.77G>A (p.R26H), c.517C>T (p.R173W), c.583C>T (p.R195C), c.673C>T (p.R225X), c.739T>C (p.C247R) and c.748G>C (p.E250A), have previously been identified in AIP patients from Western and other Eastern European populations. The effects of novel mutations were studied by amplification and sequencing of the reverse-transcribed total RNA obtained from the patients' lymphoblastoid or fibroblast cell lines. The mutations c.825+5G>C and c.770T>C resulted in varyable amounts of abnormal transcripts, r.822_825del (p.C275fsX2) and [r.770u>c, r.652_771del, r.613_771del (p.L257P, p.G218_L257del, p.I205_L257del)]. All mutations demonstrated low residual activities (0.1-1.3 %) when expressed in COS-1 cells confirming the causality of the mutations and the enzymatic defect of the disease. The clinical outcome, prognosis and correlation between the HMBS genotype and phenotype were studied in 143 Finnish and Russian AIP patients with ten mutations (c.33G>T, c.97delA, InsAlu333, p.R149X, p.R167W, p.R173W, p.R173Q, p.R225G, p.R225X, c.1073delA) and more than six patients in each group. The patients were selected from the pool of 287 Finnish AIP patients presented in a Finnish Porphyria Register (1966-2003) and 23 Russian AIP patients (diagnosed 1995-2003). Patients with the p.R167W and p.R225G mutations showed lower penetrance (19% and 11%) and the recurrence rate (33% and 0%) in comparison to the patients with other mutations (range 36 to 67% and 0 to 66%, respectively), as well as milder biochemical abnormalities [urinary porphobilinogen 47±10 vs. 163±21 mol/L, p<0.001; uroporphyrin 130±40 vs. 942±183 nmol/L, p<0.001] suggesting a milder form of AIP in these patients. Erythrocyte HMBS activity did not correlate with the porphobilinogen excretion in remission or the clinical of the disease. In all AIP severity patients, normal PBG excretion predicted freedom from acute attacks. Urinary PBG excretion together with gender, age at the time of diagnosis and mutation type could predict the likelihood of acute attacks in AIP patients.

'Hero to healing' drink driving program for Indigenous communities in Far North Queensland

Issue addressed: Alcohol-related road crashes are a leading cause of the injury burden experienced by Indigenous Australians. Existing drink driving programs are primarily designed for the mainstream population. The ‘Hero to Healing’ program was specifically developed with Indigenous communities and is underpinned by the Community Reinforcement Approach (CRA). This paper reports on the formative evaluation of the program from delivery in two Far North Queensland communities. Methods: Focus groups and semistructured interviews were conducted with drink driver participants (n = 17) and other Elders and community members (n = 8) after each program. Qualitative content analysis was used to categorise the transcripts. Results: The CRA appealed to participants because of its flexible nature and encouragement of rearranging lifestyle factors, without specific focus on alcohol use. Participants readily identified with the social and peer-related risk and protective factors discussed. Cofacilitation of the program with Elders was identified as a key aspect of the program. More in-depth discussion about cannabis and driving, anger management skills and relationship issues are recommended. Conclusions: Participants’ recognition of content reinforced earlier project results, particularly the use of kinship pressure to motivate younger family members to drink drive. Study findings suggest that the principles of the CRA are useful; however, some amendments to the CRA components and program content were necessary. So what?: Treating drink driving in regional and remote Indigenous Australian communities as a community and social issue, rather than an individual phenomenon, is likely to lead to a reduction in the number of road-related injuries Indigenous people experience.