Czech Family Research Articles

SAYING KADDISH IN CZECHOSLOVAKIA

Shortly after the Second World War, Jewish communities in the Czech lands began to remember the Jewish victims of the conflict through a ceremony called the “tryzna”. This article investigates the structure and timing of tryzny to understand how Czech‐Jews memorialized the tragedy that had recently befallen their community. By 1952, it became standard practice for Jewish communities to host a tryzna in March to commemorate the Nazi liquidation of the so‐called “Czech family camp” at Auschwitz‐Birkenau in 1944. The proliferation of tryzny ensured that Czech‐Jews mourned and commemorated the dead of the Second World War in a religious and then increasingly public way. What began as small community events, coalesced and grew into national mourning ceremonies. Tryzny link a national story of loss and an awareness of the larger Jewish genocide with Jewish funerary practices. These tryzny evolved within a communist state, in a world where the concept of the “Holocaust” had not yet entered international consciousness.

Re-familisation of the Czech Family Policy and its Causes

In this paper we analyse the shift of family related policies (benefits, daycare services and labour market policies) from the state-socialist model toward re-familisation in the Czech Republic after 1989. We demonstrate that the policies negatively influence the possibilities for parents—and especially mothers—to balance work and family life. We concentrate on the question of why the government implemented such policies, despite their negative consequences. Our analysis indicates that the communist ideological legacy (disadvantageous conditions for ideology of feminism, poor experiences with the quality of daycare) and the communist economic legacy (pressures on the public budget) have played a major role in shaping post-communist family policies. Our analysis also indicates that this legacy may be beginning to break down as the policies of re-familisation are coming into conflict with the needs and aspirations of the population and policy makers become more and more forced to confront the fertility problem.

Association between Neuromedin U Gene Variants and Overweight and Obesity

Neuromedin U (NMU) is an anorexic neuropeptide expressed in the hypothalamus. Mice lacking the NmU gene are hyperphagic and obese, whereas mice overexpressing Nmu are hypophagic and lean. Our objective was to investigate whether variants in NMU are associated with human obesity. The coding region of NMU was analyzed for variants in obese Czech children and obese Danish adults. Identified missense variants were investigated for cosegregation with obesity in families or association with obesity in the general population. The study was performed at Steno Diabetes Center, Denmark, and Department of Pediatrics, Charles University, Czech Republic. A total of 289 Czech children and adolescents with early-onset obesity and 84 Danish obese adults were analyzed for variants in NMU. A NMU Ala19Glu polymorphism was genotyped in 5851 Danish subjects of the Inter99 cohort, and a rare NMU Arg165Trp mutation was sequenced in the proband family and in 53 lean and unrelated Czech subjects. The rare NMU Arg165Trp variant cosegregated with childhood obesity in a Czech family. Homozygous carriers of the Glu allele of the NMU Ala19Glu polymorphism were more common in the overweight and obese subjects; the Glu/Glu frequency was 0.4 (95% confidence interval, 0.2-0.6) among 2586 lean subjects (BMI < 25 kg/m2) and 0.9 (95% confidence interval, 0.7-1.1) among 3265 overweight and obese subjects (body mass index >or= 25 kg/m2) [odds ratio, 2.5 (1.2-5.3); P = 0.01]. Amino acid variants in NMU associate with overweight and obesity, suggesting that NMU is involved in energy regulation in humans.

Cardiac conduction alterations in a French family with amyloidosis of the finnish type with the p.Asp187Tyr mutation in the GSN gene

Familial amyloidosis of the Finnish type (FAF) is a rare autosomal-dominant disorder caused by the accumulation of a 71-amino acid amyloidogenic fragment of mutant gelsolin, an actin-modulating protein. The main symptoms include corneal lattice dystrophy, progressive cranial and peripheral neuropathy, and skin changes. To date, only two mutations in the GSN gene have been described: the p.Asp187Asn mutation in most patients and the p.Asp187Tyr mutation in a Danish and Czech family. We report on the third family with the p.Asp187Tyr mutation and the first French FAF family. Severe cardiac conduction alterations in three patients were mainly caused by cardiac sympathetic denervation. These findings demonstrate the cardiological involvement of the FAF phenotype and suggest that cardiological follow-up is required in FAF patients.

Carl Joseph Vyborny

Carl Joseph Vyborny

Exon 5 of the RET proto-oncogene: A newly detected risk exon for familial medullary thyroid carcinoma, a novel germ-line mutation Gly321Arg

Familial medullary thyroid carcinoma (FMTC) is an autosomal dominant inherited disease, characterized by germ-line mutations in the RET proto-oncogene, mainly in exons 10 and 11, but also in exons 13, 14 and 15. Recently, mutations in exons 8 and 16 associated with FMTC were also described. In the herein presented study, single strand conformation polymorphism (SSCP) method for rapid screening of mutations in the RET proto-oncogene and fluorescent sequencing method were used. In one Czech family with FMTC, we have identified a novel missense point mutation of the RET proto-oncogene in exon 5, that results in substitution of arginine by glycine at codon 321 in the cadherin-like domain of ret protein. It seems that this mutation causes FMTC as no other mutation was found in the classical risk exons (10, 11, 13, 14, 15 and 16) of the RET proto-oncogene. The mutation cosegregates with medullary thyroid carcinoma (MTC) or C cell hyperplasia (CCH) in two patients; two other family members are mutation carriers without clinical signs of MTC so far. To improve the diagnosis of FMTC, analysis of exon 5 of the RET proto-oncogene should be considered in families with no identified classical RET mutations.

Complete loss of the cytoplasmic carboxyl terminus of the KCNQ2 potassium channel: a novel mutation in a large Czech pedigree with benign neonatal convulsions or other epileptic phenotypes.

Benign neonatal familial convulsions (BNFCs) represent a rare epileptic disorder with autosomal dominant mode of inheritance. To date, two voltage-gated potassium (K+) channel genes, KCNQ2 and KCNQ3, have been identified in typical BNFC families. The study of new pedigrees may help detect new mutations and define genotype-phenotype correlations. A large Czech family was detected in which BNFC was inherited together with a broad range of various nonneonatal epileptic phenotypes. Genetic linkage study and direct mutation analysis were performed to find the disease-causing mutation. In seven patients with BNFCs and no recurrence of seizures, a novel two-base-pair deletion (1369del2) was identified within the coding sequence of the KCNQ2 gene. The mutation led to a putative protein that lacked nearly all its carboxyl terminus part, which plays a critical role for the accurate expression of the functional K+ channels. Three patients with generalized tonic-clonic seizures (GTCSs), all without any history of BNFCs, also displayed 1369del2. Three other patients with other idiopathic epileptic phenotypes did not have the mutation. A novel 2-bp deletion within the coding sequence of the potassium channel KCNQ2 gene was detected in patients from a large and heterogeneous family with BNFCs or non-BNFC seizures.

Constructing Policy Networks: Social Assistance Reform in the Czech Republic

Czech social insurance and family transfers experienced fundamental reform in 1995 but Czech social assistance benefits still lack comprehensive change. This article explains the delay in Czech social assistance reform by appling policy network studies in a postcommunist context. Although few organizations perticipate in the Czech social assistance policy network, they are divided: they lack the common interests and exchangable resources necessary to create dialogue and compromise. The organizations of disabled citizens are the only interest group active in the policy network, with the operators of state social care institutions their primary opponents. In the late 1990s, this already fragmented issue network suffered further division when the EU entered as a powerful network actor with another set of policy goals. Until the policy network structure facilitates more dialogue and consensus‐building, hopes for comprehensive social assistance reform will linger unfulfilled.

The Rites of Responsibility: The Cinematic Rhetoric of Claude Lanzmann's Shoah (1985)

Geller | The Rites of Responsibility: The Cinematic Rhetoric of Claude Lanzmann's Shoah (1985) The Rites of Responsibility: The Cinematic Rhetoric of Claude Lanzmann's Shoah (1985) Jay Geller Vanderbilt University Shoah, Claude Lanzmann's extraordinary "documentary" of the Holocaust, consists of interviews with surviving victims, bystanders, and perpetrators as well as visits to the killing fields as they exist today. The film is known, however, almost as much for its length of nine-and-a-half hours as it is for its content. In making Shoah, Lanzmann and his crew shot more than 350 hours offilm over aperiod ofsome fouryears. Even limiting the project to this vast sum of film required extensive planning and selection . After another five years of effort, the film was edited down to its final screening time. Lanzmann admits that some of that original footage consisted of setting up scenes and botched shots that were easily excised, but that much "magnificent work" also found its way to the cutting room floor.1 How did the director and the editors, Ziva Postee and Anna Ruiz, choose what and what not to show on the screen? Among the scenes removed were interviews with several members of the Polish resistance (as well as with additional Warsaw ghetto fighters); Lanzmann has justified their exclusion by arguing that they did not have sufficient filmic presence.2 Inclusion was guided, Lanzmann states in his Cahiers du Cinéma interview, by how well a scene comported with his idea of the film's "general architecture"—the exact nature of which he never explicates .3 He has also discussed how he structured the section devoted to the Czech family camp in Auschwitz about the themes ofknowledge/ignorance, deception, and resistance. Yet the problematic which Lanzmann repeatedly returns to in his own commentaries on his film—as well as echoed in much of the critical literature, especially the work of Shoshana Felman—is witness: the problematic ofwitnessing an event whose telos was to eliminate all witness.4 The film neither illustrates nor mimetically represents that which was to have been witnessed: there is no archival footage, no docudramatics. Nor does it function merely to transmit the witness of others, of those interviewed; rather it itself acts like a witness. The film bears witness; it testifies for the dead, for those whom Primo Levi called the true witnesses of the Holocaust.5 It attempts to incarnate them, to bring them back alive. The film bears witness, because the Shoah, like all traumatic experiences, was never actually witnessed as such—nor was it ever integrated into the memory, history, or life-experience ofmodem Euro-Americans.6 Further, the Shoah as an event could not be experienced by any one individual since by its very nature it was experienced from incommensurable perspectival positions: those who see but do not understand (victims), who see but overlook (bystanders), who see but try to hide/render invisible (perpetrators). The film acts out the trauma by bringing these witnesses together—and those oftwo others, the filmmaker/ narrator/interviewer/inquirer Lanzmann and his alter-ego, the historian Raul Hilberg—before the audience. The film would make each member of the audience bear witness. Yet the bystanders, too, bore witness. In the Cahiers du Cinema interview Lanzmann refers to the Poles as the "les témoins," the witnesses.7 How do the filmgoers then not become like these bystanders? There is no natural and necessary relationship between knowledge and action, between knowledge and responsibility for that knowledge. Moreover, since the audience experiences the montage of witness in time, its members do not have a synchronic tableau (neither vivant nor mort) before them; they can assume no godlike perches from which each can observe the clash of testimony, watch the false testimony discorroborated. Rather the film educates its audience about how to become a certain kind of witness. Since it is the individual who belies the attempt to erase, who becomes the exception that proves and subverts the rule of silence, so, like the individual survivors whose testimony is heard, the viewers are to become such individuals. The film's heuristic principle derives from an aspect of Lanzmann's background that seems to have...

Danish type gelsolin related amyloidosis: 654G-T mutation is associated with a disease pathogenetically and clinically similar to that caused by the 654G-A mutation (familial amyloidosis of the Finnish type)

Background—Familial amyloidosis of the Finnish type (FAF, Finnish hereditary amyloidosis) is caused by a 654G-A mutation in the gelsolin gene on chromosome 9 resulting in the expression of mutant Asn-187...

FAMILY LAW IN CZECHOSLOVAKIA AFTER THE REVOLUTION

This article describes the main legal changes brought about in Czech family law since the revolution of 1989. These are set in the context of the current social conditions and the theoretical discussions which are presently taking place.

Gelsolin-derived familial amyloidosis caused by asparagine or tyrosine substitution for aspartic acid at residue 187.

Dominantly inherited familial amyloidosis, Finnish type (FAF) is caused by the accumulation of a 71-amino acid amyloidogenic fragment of mutant gelsolin (GSN). FAF is common in Finland but is very rare elsewhere. In Finland and in two American families, the mutation is a G654A transition leading to an Asp to Asn substitution at residue 187. We found the same mutation in a Dutch family but a Danish FAF family had a G654T mutation, predicting Asp to Tyr at residue 187. We also found the G654T transversion in a Czech family. Using GSN polymorphisms, different haplotypes were found in the Danish and Czech families. We conclude that substitution of the uncharged Asn or Tyr for the acidic Asp at residue 187 creates a conformation that may be preferentially amyloidogenic for GSN.

INCIDENCE OF A WEAK HL‐A W27 ANTIGEN IN A CZECH FAMILY

In two members of a Czech family a weak W 27 antigen was found. The lymphocytes of the two individuals showed an identical positive cytotoxic reaction only with some sera having anti-W 27 specificity; anti-W 27 of both the said sera and those showing negative cytotoxic test was absorbed by the lymphocytes. The thrombocyte absorption capacity was substantially poorer.

Arctic Diary of Josef Pospíšil 1872-1874

The paper treats of the up-to-now unknown diary of Josef Pospíšil who was one of the five countrymen from Moravia and Bohemia participating in the arctic expedition of Weyprecht and Payer on board the "Tegetthoff" in 1872-1874. Arctic Austro-Hungarian expedition became known through its involuntary being drifted to the northern part of the Barents Sea. After 435 days, on August 30, 1873, its members discovered by chance the up-to-that-time unknown group of islands which they called the Land of Francis Joseph. The progress of the expedition was described by Payer in his book "Die österreichisch-ungarische Nordpol-Expedition in den Jahren 1872-1874, nebst einer Skizze der zweiten deutschen Nordpol-Expedition 1869-1870 und der Polar-Expedition von 1871" (Wien 1876). More details regarding the spirits, morals and mutual relations between individual members of the expedition may be lerned, however, from diaries by individual members of the crew. Meanwhile other participants have published at least fragments of their diaries, the diary of Josef Pospíšil has remained unknown till the author of this paper obtained it from the descendants of the Pospíšil family. It is a thin notebook, 98 x 162 mm in size, comprising 55 pages, and written in broken German. Josef Pospíšil descended from a Czech family, being the son of the tailor Antonín Pospíšil. He was born on June 29, 1850 in Přerov, Moravia in the house No 436 in the present Blahoslav Street. He became a machine locksmith and metal-turner. On board the ship he worked as a stoker. He died in Melk, Austria, on Dec. 19, 1906. The discovered notes of Josef Pospíšil represent not only a further proof of the participation of Czech explorers in important geographical expeditions in the 19th century, but also bring evidence on the desperate struggle for life of a group of polar explorers cut by polar ice for more than two years from the other world, the more interesting that it is described from the point of view of a subordinate member of the crew, and not of the captain.