Kirkon vai valtion kirjat? : Uskontokuntasidonnaisuuden ongelma Suomen väestökirjanpidossa 1839-1904

The Population Register – run by the Church or the state? The problem posed by the obligation to belong to a religious community in the registration of births and deaths in Finland between 1839 and 1904 The Lutheran Church of Finland is the nation’s largest church; approximately 82 per cent of Finns were members in 2007. The Church ran an official register of its members until 1999, when the state then undertook this task. The registration of births and deaths by the Church has a long history dating back to the 17th century, when Bishop Johannes Gezelius Sr. decreed that all parish members would have to be recorded in parish registers. These registers were used to control how well parish members knew the Christian doctrine and, gradually, also if they were literate. Additionally, the Church attempted to ensure by means of the parish registers that parish members went to Holy Communion annually. Since everyone was a member of the Lutheran Church, the state also took advantage of the parish registers and used them for the purposes of tax collection and conscription. The main research theme of “The Population Register – run by the Church or the state?” goes back to these times. The actual research period covers the years of 1839–1904. At that time Finland was under Russian rule, although autonomous. In the late 19th century the press and different associations in Finland began to engage in public debate, and the country started moving from a submissive society to a civic one. The identity of the Lutheran Church also became more prominent when the Church Act and the General Synod were realised in 1869. A few years earlier, municipal and parish administrations had been separated, but the general registration of births and deaths was left to the Church to see to. In compliance with the constitution of the country, all the inhabitants in principle still had to be Lutheran. In practice, the situation was different. The religious and ideological realms diversified, and the Lutheran concept of religion was no longer acceptable to everyone. The conflict was reflected in the registration of births and deaths, which was linked to the Lutheran Church and its parish registers. Nobody was allowed to leave the Church, there was no civil register, and the Lutheran Church did not consent to record unbaptized children in the parish registers. Therefore such children were left without civil rights. Thus the obligation to belong to a religious community had become a problem in the registration of births and deaths. The Lutheran clergy also appealed to the 1723 privileges, according to which they had been exempted from the drawing up of additional population registers. In 1889 Finland passed the Dissenters Act. By virtue of this act the Baptists and the Methodists left the state Church, but this was not the case with the members of the free churches. The freethinkers had to retain their church membership, as the law did not apply to them. This meant that the unbaptized children of the members of the free churches or those of freethinkers were still not entered in any registers. The children were not able to go to school, work for the state or legally marry. Neither were they able to inherit property, as they did not legally exist. The system of parish registers was created when everyone was required to be a member of the Lutheran Church, but it did not work when liberal attitudes eventually penetrated the sphere of religion, too. The government´s measures to solve the problem were slow and cautious, partly because Finland was part of Russia, partly because there were only about 100 unbaptized children. As the problem group was small and the state´s resources were limited, no general civil register was established. The state accepted the fact that in spite of the problems, the Evangelical Lutheran Church and the congregations of dissenters were the only official establishments to run populations registers in the country, and for social purposes, too. In 1900 the Diet of Finland finally approved a limited civil register, which unbaptized children and unregistered foreigners would be recorded in. Due to political reasons the civil register did not come into existence until 1917, after the actual research period.

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.

New Governance and the EU Courts : The Experimentalist Architecture of Judicial Decision-Making

The dissertation examines the role of the EU courts in new governance. New governance has raised unprecedented interest in the EU in recent years. This is manifested in a plethora of instruments and actors at various levels that challenge more traditional forms of command-and-control regulation. New governance and political experimentation more generally is thought to sap the ability of the EU judiciary to monitor and review these experiments. The exclusion of the courts is then seen to add to the legitimacy problem of new governance. The starting point of this dissertation is the observation that the marginalised role of the courts is based on theoretical and empirical assumptions which invite scrutiny. The theoretical framework of the dissertation is deliberative democracy and democratic experimentalism. The analysis of deliberative democracy is sustained by an attempt to apply theoretical concepts to three distinctive examples of governance in the EU. These are the EU Sustainable Development Strategy, the European Chemicals Agency, and the Common Implementation Strategy for the Water Framework Directive. The case studies show numerous disincentives and barriers to judicial review. Among these are questions of the role of courts in shaping governance frameworks, the reviewability of science-based measures, the standing of individuals before the courts, and the justiciability of soft law. The dissertation analyses the conditions of judicial review in each governance environment and proposes improvements. From a more theoretical standpoint it could be said that each case study presents a governance regime which builds on legislation that lays out major (guide)lines but leaves details to be filled out at a later stage. Specification of detailed standards takes place through collaborative networks comprising members from national administrations, NGOs, and the Commission. Viewed this way, deliberative problem-solving is needed to bring people together to clarify, elaborate, and revise largely abstract and general norms in order to resolve concrete and specific problems and to make law applicable and enforceable. The dissertation draws attention to the potential of peer review included there and its profound consequences for judicial accountability structures. It is argued that without this kind of ongoing and dynamic peer review of accountability in governance frameworks, judicial review of new governance is difficult and in some cases impossible. This claim has implications for how we understand the concept of soft law, the role of the courts, participation rights, and the legitimacy of governance measures more generally. The experimentalist architecture of judicial decision-making relies upon a wide variety of actors to provide conditions for legitimate and efficient review.