Autopsy and genetic diagnosis of 21-hydroxylase deficiency with bilateral testicular tumors in a case under no medication for over one year

The autopsy findings of an adult patient with 21-hydroxylase deficiency are presented. Genetic analysis of the 21-hydroxylase gene (CYP21A2) was performed for accurate diagnosis of congenital adrenal hyperplasia (CAH), and bilateral testicular tumors were characterized. We report a 29-year-old Japanese man who was diagnosed with CAH (21-hydroxylase deficiency) in infancy and had continued steroid therapy until the age of 28. However, for more than one year, he had not been treated for CAH and was found dead. In the medico-legal autopsy findings, both adrenal glands were enlarged, and hypertrophy of adrenal cortices and bilateral testicular tumors positive for melan-A were observed. Genomic DNA was prepared from cervical lymph nodes collected during autopsy, and CYP21A2 was PCR amplified and sequenced directly using newly designed primers. From the morphological findings, the bilateral testicular tumors were considered to be adrenogenital syndrome (TTAGS). Through the whole sequence of CYP21A2, the intron 2 splice mutation (656)A to (656)G was found. TTAGS were thought to be adrenal rests enlarged by ACTH stimulus. From the autopsy findings and the result of genetic analysis, he was diagnosed with the salt-wasting form of 21-hydroxylase deficiency and his cause of death was presumed to be heart failure based on abnormal electrolytes.     

Healing through Action? The Political Mobilization of Victims of Al Qaeda-Inspired Violence in Spain and the United Kingdom

Spain and the United Kingdom have experienced similar types of political violence. Since the 1960s, both countries have suffered casualties as a result of long-standing ethno-nationalist conflicts as well as terrorist attacks by Al Qaeda–inspired groups. In spite of these similarities, the two countries display a striking variation in the attitudes to victims. In Spain, Associations of Victims of Terrorism have been highly visible and influential, whereas United Kingdom-based organizations have not captured the public's imagination and the attention of policymakers in the same way. Spanish associations of victims have been present in the public sphere and have routinely provided their opinions on counterterrorist policy and appropriate government legislation whereas this sort of political activity is difficult to trace in the United Kingdom. As a result of this puzzle, the article tries to answer the following question: Why are associations of victims of terrorism by Al Qaeda–inspired attacks more influential in Spain than in the United Kingdom? The article will argue that political and sociocultural variables account for the difference. More specifically, the article demonstrates that the experience of both ethno-nationalist and jihadist political violence has affected party systems and cultural frames differently, hence providing distinct sets of political opportunities for victims’ associations to carry out their lobbying strategies.     

Allele frequencies for nine STR loci in Ovambo population using AmpFlSTR Profiler Kit

Allele frequencies for the nine short tandem repeat (STR) loci D3S1358, vWA, FGA, TH01, TPOX, CSF1PO, D5S818, D13S317, and D7S820 were investigated in 195 unrelated Ovambo (Bantus) population from Namibia. AmpFlSTR Profiler Kit was employed for amplification. For each locus, 6-19 alleles were observed. Comparison between Ovambo population data and that of other African populations was performed. AmpFlSTR Profiler detection system is a useful tool for individual identification in Ovambo population.     

When Do Countries Recentralize? Ideology and Party Politics in the Age of Austerity

The Spanish executive centralized political power to manage the politics of austerity better in the aftermath of the Great Recession. This article analyzes the reinforcement of the power of the central government and argues that three explanatory variables—economic crisis, ideology, and party politics—account for recentralization, which is defined as fiscal consolidation, concentration of competences, bureaucratic rationalization, and ideological convergence. The debate about the motives and nature of recentralization (de jure vs. de facto) further polarized the center-periphery cleavage. Regional prosovereignty parties interpreted the reversal of decentralization as another sign that accommodation within Spain was not possible and that contestation was the way forward.     

Longitudinal evaluation of immunohistochemical findings of milk aspiration: an experimental study using a murine model

To examine the longitudinal change of pathological findings of the lung and other organs in milk aspiration, an experimental study using a murine model was carried out. Either 0.5 or 1.0 ml cow's milk was instilled into the trachea of rats. From immediately after to 14 days after instillation, the animals were sacrificed, and the lungs, liver, kidneys, and spleen were removed. The results of immunostaining with anti-human α lactalbumin antibody indicated that not only the lung but also the kidney and spleen showed a positive reaction against the antibody over time. Experimentally aspirated milk was detectable in alveoli until 2 days after instillation. It was also detectable in renal tubules from 1 to 6h after instillation. Macrophages containing granules of aspirated milk were observed in splenic red pulp from 3h to 14 days after instillation. Detection of aspirated milk in other organs except the lung would be clear evidence of intravital milk aspiration and would suggest previous or recurrent milk aspiration.