A novel mutation of the ACADM gene (c.145C>G) associated with the common c.985A>G mutation on the other ACADM allele causes mild MCAD deficiency: a case report.

A female patient, with normal familial history, developed at the age of 30 months an episode of diarrhoea, vomiting and lethargy which resolved spontaneously. At the age of 3 years, the patient re-iterated vomiting, was sub-febrile and hypoglycemic, fell into coma, developed seizures and sequels involving right hemi-body. Urinary excretion of hexanoylglycine and suberylglycine was low during this metabolic decompensation. A study of pre- and post-prandial blood glucose and ketones over a period of 24 hours showed a normal glycaemic cycle but a failure to form ketones after 12 hours fasting, suggesting a mitochondrial β-oxidation defect. Total blood carnitine was lowered with unesterified carnitine being half of the lowest control value. A diagnosis of mild MCAD deficiency (MCADD) was based on rates of 1-14C-octanoate and 9, 10-3H-myristate oxidation and of octanoyl-CoA dehydrogenase being reduced to 25% of control values. Other mitochondrial fatty acid oxidation proteins were functionally normal. De novo acylcarnitine synthesis in whole blood samples incubated with deuterated palmitate was also typical of MCADD. Genetic studies showed that the patient was compound heterozygous with a sequence variation in both of the two ACADM alleles; one had the common c.985A>G mutation and the other had a novel c.145C>G mutation. This is the first report for the ACADM gene c.145C>G mutation: it is located in exon 3 and causes a replacement of glutamine to glutamate at position 24 of the mature protein (Q24E). Associated with heterozygosity for c.985A>G mutation, this mutation is responsible for a mild MCADD phenotype along with a clinical story corroborating the emerging literature view that patients with genotypes representing mild MCADD (high residual enzyme activity and low urinary levels of glycine conjugates), similar to some of the mild MCADDs detected by MS/MS newborn screening, may be at risk for disease presentation.

Peer rejection and social information-processing factors in the development of aggressive behavior problems in children.

The relation between social rejection and growth in antisocial behavior was investigated. In Study 1,259 boys and girls (34% African American) were followed from Grades 1 to 3 (ages 6-8 years) to Grades 5 to 7 (ages 10-12 years). Early peer rejection predicted growth in aggression. In Study 2,585 boys and girls (16% African American) were followed from kindergarten to Grade 3 (ages 5-8 years), and findings were replicated. Furthermore, early aggression moderated the effect of rejection, such that rejection exacerbated antisocial development only among children initially disposed toward aggression. In Study 3, social information-processing patterns measured in Study 1 were found to mediate partially the effect of early rejection on later aggression. In Study 4, processing patterns measured in Study 2 replicated the mediation effect. Findings are integrated into a recursive model of antisocial development.

Response decision processes and externalizing behavior problems in adolescents.

Externalizing behavior problems of 124 adolescents were assessed across Grades 7-11. In Grade 9, participants were also assessed across social-cognitive domains after imagining themselves as the object of provocations portrayed in six videotaped vignettes. Participants responded to vignette-based questions representing multiple processes of the response decision step of social information processing. Phase 1 of our investigation supported a two-factor model of the response evaluation process of response decision (response valuation and outcome expectancy). Phase 2 showed significant relations between the set of these response decision processes, as well as response selection, measured in Grade 9 and (a) externalizing behavior in Grade 9 and (b) externalizing behavior in Grades 10-11, even after controlling externalizing behavior in Grades 7-8. These findings suggest that on-line behavioral judgments about aggression play a crucial role in the maintenance and growth of aggressive response tendencies in adolescence.

Real-Time Decision Making and Aggressive Behavior in Youth: A Heuristic Model of Response Evaluation and Decision (RED).

Considerable scientific and intervention attention has been paid to judgment and decision-making systems associated with aggressive behavior in youth. However, most empirical studies have investigated social-cognitive correlates of stable child and adolescent aggressiveness, and less is known about real-time decision making to engage in aggressive behavior. A model of real-time decision making must incorporate both impulsive actions and rational thought. The present paper advances a process model (response evaluation and decision; RED) of real-time behavioral judgments and decision making in aggressive youths with mathematic representations that may be used to quantify response strength. These components are a heuristic to describe decision making, though it is doubtful that individuals always mentally complete these steps. RED represents an organization of social-cognitive operations believed to be active during the response decision step of social information processing. The model posits that RED processes can be circumvented through impulsive responding. This article provides a description and integration of thoughtful, rational decision making and nonrational impulsivity in aggressive behavioral interactions.

Commandeering Aesop’s Bamboo Canon: A 19th Century Confederacy of Creole Fugitive Fables

In my thesis, “Commandeering Aesop’s Bamboo Canon: A 19th Century Confederacy of Creole Fugitive Fables,” I ask and answer the ‘Who? What? Where? When? Why?” of Creole Literature using the 19th century production of Aesopian fables as clues to resolve a set of linguistic, historical, literary, and geographical enigmas pertaining the ‘birth-place(s)’ of Creolophone Literatures in the Caribbean Sea, North and South America, as well as the Indian Ocean. Focusing on the fables in Martinique (1846), Reunion Island (1826), and Mauritius (1822), my thesis should read be as an attempt capture the links between these islands through the creation of a particular archive defined as a cartulary-chronicle, a diplomatic codex, or simply a map in which I chart and trace the flight of the founding documents relating to the lives of the individual authors, editors, and printers in order to illustrate the articulation of a formal and informal confederation that enabled the global and local institutional promotion of Creole Literature. While I integrate various genres and multi-polar networks between the authors of this 19th century canon comprised of sacred and secular texts such as proclamations, catechisms, and proverbs, the principle literary genre charted in my thesis are collections of fables inspired by French 17th century French Classical fabulist, Jean de la Fontaine. Often described as the ‘matrix’ of Creolophone Literature, these blues and fables constitute the base of the canon, and are usually described as either ‘translated,’ ‘adapted,’ and even ‘cross-dressed’ into Creole in all of the French Creolophone spaces. My documentation of their transnational sprouting offers proof of an opaque canonical formation of Creole popular literature. By constituting this archive, I emphasize the fact that despite 200 years of critical reception and major developments and discoveries on behalf of Creole language pedagogues, literary scholars, linguists, historians, librarians, archivist, and museum curators, up until now not only have none have curated this literature as a formal canon. I also offer new empirical evidence in order to try and solve the enigma of “How?” the fables materially circulated between the islands, and seek to come to terms with the anonymous nature of the texts, some of which were published under pseudonyms. I argue that part of the confusion on the part of scholars has been the result of being willfully taken by surprise or defrauded by the authors, or ‘bamboozled’ as I put it. The major paradigmatic shift in my thesis is that while I acknowledge La Fontaine as the base of this literary canon, I ultimately bypass him to trace the ancient literary genealogy of fables to the infamous Aesop the Phrygian, whose biography – the first of a slave in the history of the world – and subsequent use of fables reflects a ‘hidden transcript’ of ‘masked political critique’ between ‘master and slave classes’ in the 4th Century B.C.E. Greece.

This archive draws on, connects and critiques the methodologies of several disciplinary fields. I use post-colonial literary studies to map the literary genealogies Aesop; use a comparative historical approach to the abolitions of slavery in both the 19th century Caribbean and the Indian Ocean; and chart the early appearance of folk music in early colonial societies through Musicology and Performance Studies. Through the use of Sociolinguistics and theories of language revival, ecology, and change, I develop an approach of ‘reflexive Creolistics’ that I ultimately hope will offer new educational opportunities to Creole speakers. While it is my desire that this archive serves linguists, book collectors, and historians for further scientific inquiry into the innate international nature of Creole language, I also hope that this innovative material defense and illustration of Creole Literature will transform the consciousness of Creolophones (native and non-native) who too remain ‘bamboozled’ by the archive. My goal is to erase the ‘unthinkability’ of the existence of this ancient maritime creole literary canon from the collective cultural imaginary of readers around the globe.