Adult Hirschsprung's disease diagnosed during forensic autopsy

We report a case of fatal Hirschsprung's disease (HD) discovered at autopsy. A 20-year-old man collapsed at home. Emergency medical personnel found him in cardiac arrest and all resuscitative efforts failed. He had a past history of chronic constipation since infancy. Forensic autopsy revealed a megacolon full of gas and stools. Microscopic examination showed absence of ganglion cells in a short segment of the rectum and enterocolitis in the left and transverse colon. HD is rarely described in adults. In many cases, patients complained of constipation since infancy but the affection remained misdiagnosed. The relative good tolerance of the disease is usually due to a short aganglionic bowel segment. Enterocolitis is a frequent and severe complication of HD in children but is rarely described in adults. This case suggests the importance of HD diagnosis in childhood in order to avoid fatal complications with forensic consequences.     

Whose deaths matter? Mortality, advocacy, and attention to disease in the mass media

Diseases capture public attention in varied ways and to varying degrees. In this essay, we use a unique data set that we have collected about print and broadcast media attention to seven diseases across nineteen years in order to address two questions. First, how (if at all) is mortality related to attention? Second, how (if at all) is advocacy, in the form of organized interest group activity, related to media attention? Our analysis of the cross-disease and cross-temporal variation in media attention suggests that who suffers from a disease as well as how many suffer are critical factors in explaining why some diseases get more attention than others. In particular, our data reveal that both the print and the broadcast media tend to be much less attentive to diseases that disproportionately burden blacks relative to whites. We also find a positive link between the size of organizational communities that take an interest in disease and media attention, though this finding depends on the characteristics of those communities. Finally, this study also reveals the limitations of relying on single-disease case studies-and particularly HIV/AIDS-to understand how and why disease captures public attention. Many previous inferences about media attention that have been drawn from the case of AIDS are not reflective of the attention allocated to other diseases.     

Maternal cigarette smoking during pregnancy and criminal/deviant behavior: a meta-analysis

A growing body of empirical literature has emerged examining the somewhat inconsistent relationship between maternal cigarette smoking (MCS) during pregnancy and children's subsequent antisocial behavior. To systematically assess what existing studies reveal regarding MCS as a criminogenic risk factor for offspring, the authors subjected this body of literature to a meta-analysis. The analysis reveals a statistically significant--yet rather small--overall mean "effect size" of the relationship between MCS and the likelihood children will engage in deviant/criminal behavior. In addition to being rather moderate in size, the MCS-crime/deviance relationship is sensitive to a number of methodological specifications across empirical studies--particularly those associated with sample characteristics. The implications of this modest, and somewhat unstable, relationship are discussed in terms of guidelines for future research on this subject and how existing theoretical perspectives may be integrated to explain the MCS-crime/deviance link.     

Gamma-hydroxybutyric acid stability and formation in blood and urine

Gamma-hydroxybutyric acid (GHB) can cause problems in interpretation of toxicological findings due to its endogenous nature, significant production in tissues after death and potential formation in stored samples. Our study was designed to determine the influence of storage conditions on GHB levels and its possible in vitro formation in blood and urine in cases where no exogenous use of GHB or its precursors was suspected. The samples were prepared by validated method based on liquid-liquid reextraction with adipic acid internal standard and MSTFA derivatization and assayed on a GC-MS operating in EI SIM mode. The first part of the study was performed with pooled blood and urine samples obtained from living and deceased subjects stored with and without NaF (1% w/v) at 4 and -20 degrees C over 8 months. In ante-mortem samples (both blood and urine) no significant GHB production was found. After 4 months of storage, the substantial GHB rise up to 100 mg/Lwas observed in post-mortem blood stored at 4 degrees C without NaF with subsequent gradual decrease in following months. The inhibition of GHB production was apparent during storage in NaF treated frozen blood samples. In post-mortem urine only slight temporary GHB levels were ascertained (up to 8 mg/L). The second part of our study was aimed to analyse 20 individual post-mortem blood samples stored at 4 degrees C for 16-27 days between autopsy and analysis without preservation followed by storage at 4 degrees C with NaF for 4 months. The temporary GHB production with maximum of 28 mg/Lwas detected in some samples.     

Death of an infant involving benzocaine

This report describes the death of a four-month-old Hispanic male which may be related to benzocaine toxicity. A toxicological evaluation revealed benzocaine at a concentration of 3.48 mg/L, and postmortem methemoglobin of 36% (normal 0.4-1.5). Methemoglobinemia is a complication of benzocaine toxicity. In light of the toxicology findings, the coroner investigated the source of the benzocaine and discovered that the child was treated with Zenith Goldline Allergen Ear Drops containing 0.25% w/v benzocaine and 5.4% w/v antipyrine. There was an admission by a caregiver that on the day prior to the child's death, he had been treated with three times the prescribed dose. Blood benzocaine concentrations in nine other unrelated cases were determined and concentrations ranged from <0.05-5.3 mg/L (mean 1.48 mg/L). Seven of the nine cases were positive for drugs of abuse, and one additional case was described as a known drug user. Methemoglobin in these benzocaine positive cases ranged from 6-69%; however, methemoglobin concentrations in postmortem cases are frequently elevated and should be interpreted with caution. The unknown significance of the benzocaine, and the circumstances of the case raise questions about the ultimate attribution of this death to SIDS.     

Forensic casework analysis using the HVI/HVII mtDNA linear array assay

The mitochondrial hypervariable regions I and II have proven to be a useful target for analysis of forensic materials, in which the amount of DNA is limited or highly degraded. Conventional mitochondrial DNA (mtDNA) sequencing can be time-consuming and expensive, limitations that can be minimized using a faster and less expensive typing assay. We have evaluated the exclusion capacity of the linear array mtDNA HVI/HVII region-sequence typing assay (Roche Applied Science) in 16 forensic cases comprising 90 samples. Using the HVI/HVII mtDNA linear array, 56% of the samples were excluded and thus less than half of the samples require further sequencing due to a match or inconclusive results. Of all the samples that were excluded by sequence analysis, 79% could be excluded using the HVI/HVII linear array alone. Using the HVI/HVII mtDNA linear array assay, we demonstrate the potential to decrease sequencing efforts substantially and thereby reduce the cost and the turn-around time in casework analysis.     

Reconfiguring the Animal/Human Boundary: The Impact of Xeno Technologies

The focus of this paper is on the symbolic and cultural as well as practical implications of what I term xeno technologies. I argue that these biomedical technologies, which aim to prolong individual human lives through the sacrifice of animal bodies, generate considerable anxiety and pose many intriguing issues for health care lawyers. In part, the concerns engendered by xeno technologies are attributable to the incalculable risks they may pose. This, coupled with public distrust of scientific evaluations of risk, undermines scientific attempts to present them as benign technologies. In this paper, however, I suggest that xeno technologies provoke a deeper cultural unease by raising, in acute new forms, historical and religious concerns about bodily mixing and rejection which challenge traditional notions of (human) self identity. The various ways in which xeno technologies render human and non-human bodies vulnerable and penetrable, pose multiple challenges to the animal/human boundary. In my view, they should force a radical re-thinking of notions of kinship, which should extend beyond the ȁ8easy caseȁ9 of human kinship with other great apes. Rather than addressing this issue, however, healthcare law makes valiant attempts to shore up the animal/human boundary. Such efforts at boundary maintenance may be traced at various sites, including the regulatory regime under the Human Fertilisation and Embryology Act 1990. I argue that lawȁ9s efforts to grapple with the ethical challenges posed by biotechnologies are doomed to incoherence unless it confronts the unreflective speciesism underpinning law, which designates animals as property and serves to obscure our kinship with them. My suggestion is that health care ethicists and lawyers should instead seek to expose the myriad ways in which biotechnologies may prove oppressive rather than liberatory for those who are made their human and animal subjects.     

Twelve short tandem repeat loci Y chromosome haplotypes: genetic analysis on populations residing in North America

A total of 2443 male individuals, previously typed for the 13 CODIS STR loci, distributed across the five North American population groups African American, Asian, Caucasian, Hispanic, and Native American were typed for the Y-STR loci DYS19, DYS385a/b, DYS389I/II, DYS390, DYS391, DYS392, DYS393, DYS437, DYS438 and DYS439 using the PowerPlex Y System. All population samples were highly polymorphic for the 12 Y-STR loci with the marker DYS385a/b being the most polymorphic across all sample populations. The Native American population groups demonstrated the lowest genetic diversity, most notably at the DYS393 and DYS437 loci. Almost all of the 12-locus haplotypes observed in the sample populations were represented only once in the database. Haplotype diversities were greater than 99.6% for the African Americans, Caucasians, Hispanics, and Asians. The Native Americans had the lowest haplotype diversities (Apaches, 97.0%; Navajo, 98.1%). Population substructure effects were greater for Y-haplotypes, compared with that for the autosomal loci. For the apportionment of variance for the 12 Y-STRs, the within sample population variation was the largest component (>98% for each major population group and approximately 97% in Native Americans), and the variance component contributed by the major population groups was less than the individual component, but much greater than among sample populations within a major group (11.79% versus 1.02% for African Americans/Caucasians/Hispanics and 15.35% versus 1.25% for all five major populations). When each major population is analyzed individually, the R(ST) values were low but showed significant among group heterogeneity. In 692 confirmed father-son pairs, 14 mutation events were observed with the average rate of 1.57x10(-3)/locus/generation (a 95% confidence bound of 0.83x10(-3) to 2.69x10(-3)). Since the Y-STR loci reside on the non-recombining region of the Y chromosome, the counting method is one approach suggested for conveying an estimate of the rarity of the Y-haplotype. Because the Y-STR loci are not all in disequilibrium to the same extent, the counting method is a very conservative approach. The data also support that autosomal STR frequencies can be multiplied by the upper bound frequency estimate of a Y-haplotype in the individual population group or those pooled into major population groups (i.e., Caucasian, African American, Hispanic, and Asian). These analyses support use of the haplotype population data for estimating Y-STR profile frequencies for populations residing in North America.     

Bigger is not Necessarily Better: An Analysis of Violence Against Women Estimates from the National Crime Victimization Survey and the National Violence Against Women Survey

Apparent differences between violence against women estimates from the National Crime Victimization Survey (NCVS) and the National Violence Against Women Survey (NVAWS) continue to generate confusion. How is it that two surveys purporting to measure the nature and extent of violence against women present such seemingly dissimilar estimates? The answer is found in the important, yet often over-looked details of each survey. Our objective is to clarify some of the reasons for apparent disparities between NCVS and NVAWS estimates by first identifying why published estimates are not comparable. Next, we adjust NCVS estimates to make them comparable to NVAWS estimates by restricting NCVS estimates to 1995 and including only persons age 18 or older, and by applying the NVAWS series victimization counting protocol to NCVS estimates. Contrary to findings in the literature, the NVAWS did not produce statistically greater estimates of violence against women compared to the NCVS. Further, incident counting protocols used in the NVAWS and the recalibrated NCVS increased the error, and decreased the reliability of the estimates.Michael R. Rand - The views expressed in this article do not necessarily represent those of the Bureau of Justice Statistics or the U.S. Department of Justice.