New Spontaneous Model of Fibrodysplasia Ossificans Progressiva

We report the first known example of spontaneous, naturally occurring fibrodysplasia ossificans progressiva (FOP) in a mammal. The Southeast Asian mouse deer of the genus _Tragulus_ (Artiodactyla: Tragulidae) have an osseous sheath covering the lower back and upper thigh region consistent with the clinical definition of FOP. This heterotophic bone deposition is sex related apparently with a genetic basis - it only occurs in males and is lacking in females; it is present in all adults males, including both wild obtained and zoo bred animals. _Tragulus_ may offer the opportunity to examine many of the disease's most significant attributes experimentally

Pachydermoperiostosis-Like Disease In Captive Red Ruffled Lemurs (Varecia Variegatus Rubra)

Pachydermatoperiostosis, a rare form of hypertrophic osteoarthropathy, is of unknown etiology and previously thought limited to humans. The only periosteal reaction previously reported in prosimians is related to renal disease. Notation of hypertrophic osteoarthritis in three prosimians led to recognition that this was the first non-human documentation of the disease. Three related red ruffed lemurs (Varecia variegatus rubra) had diaphyseal periosteal reaction classic for hypertrophic osteoarthropathy. Workup was negative for known underlying causes and for the secondary hyperparathyroidism which produces bone alterations in black Eulemur macao, black and white Varecia variegatatus varigatus and ringtail Lemur catta lemurs. Recognition of facial coarsening allows identification of the primary form of hypertrophic osteoarthropathy, categorized in humans as primary hypertrophic osteoarthropathy. This is the first recognition of the phenomenon in the order primates, exclusive of humans, and represents a new model for this rare disease

A selection of open problems

AbstractThis is a collection of open problems which touch on Neil Hindman's mathematics and were collected in conjunction with the Conference on Ramsey Theory and Topological Algebra in his honor

Apparent sixth sense in theropod evolution: The making of a Cretaceous weathervane

Objective Two separate and distinctive skills are necessary to find prey: Detection of its presence and determination of its location. Surface microscopy of the dentary of albertosaurines revealed a previously undescribed sensory modification, as will be described here. While dentary “foramina” were previously thought to contain tactile sensory organs, the potential function of this theropod modification as a unique localizing system is explored in this study. Method Dentary surface perforations were examined by surface epi-illumination microscopy in tyrannosaurine and albertosaurine dinosaurs to characterize their anatomy. Fish lateral lines were examined as potentially comparable structures. Result In contrast to the subsurface vascular bifurcation noted in tyrannosaurines (which lack a lateral dentary surface groove), the area subjacent to the apertures in albertosaurine grooves has the appearance of an expanded chamber. That appearance seemed to be indistinguishable from the lateral line of fish. Conclusion Dentary groove apertures in certain tyrannosaurid lines (specifically albertosaurines) not only have a unique appearance, but one with significant functional and behavior implications. The appearance of the perforations in the dentary groove of albertosaurines mirrors that previously noted only with specialized neurologic structures accommodating derived sensory functions, as seen in the lateral line of fish. The possibility that this specialized morphology could also represent a unique function in albertosaurine theropods for interacting with the environment or facilitating prey acquisition cannot be ignored. It is suggested that these expanded chambers function in perceiving and aligning the body relative to the direction of wind, perhaps a Cretaceous analogue of the contemporary midwestern weathervane

Criminal Consumer Fraud: A Victim-Oriented Analysis

The poor and the elderly are the principal victims of the high pressure and often sophisticated sale techniques of criminal consumer fraud in this country. The problem is compounded when the poor and the elderly live in inner cities where legal assistance is costly and inadequate, and the courts, prosecutors, and investigators are overburdened. Yet the executive and legislative agencies responsible for law enforcement continue to assign a low priority to criminal consumer fraud. President Ford has reflected this orientation: For effective management [of law enforcement], we first have to have some hard decisions on priorities. As a starter, I would suggest a high priority on violent crime and street crime in the inner-city. There is where crime does the most damage to our whole urban structure. There is where crime hurts the poor who already suffer enough. The cruel irony of this statement is that one of the most significant causes of this suffering is criminal consumer fraud. The statement implicitly presumes that the economic impact of criminal fraud on low-income consumers is negligible because they are not consumers of expensive durable goods. But this presumption overlooks that rapidly expanding American institution, the installment plan and the special forms it takes in low-income areas. The harm resulting from the low-income family\u27s lack of shopping sophistication and its vulnerability to easy credit\u27\u27 does not end merely with higher prices and heavy indebtedness. Repossession, creditor harassment, and garnishment of wages take their greatest tolls on the poor. Herbert Edelhertz notes: A surprisingly large number of people living in ghettos do have something to lose, but unlike the established middle classes the asset in jeopardy is very often the only asset which stands between its owner and utter destitution

Odyssey in the evolution of a paleopathologist

A recent report suggesting perceived limitations of and opportunities in the study of paleopathology suggested the importance of incorporation of scientific methodologies. It seems reasonable to also explore how those methodologies are developed and, indeed, how one approaches paleopathology as a science. The development of one such paleopathologist is delineated from his serendipitous observations to application of hypothesis generation and subsequent testing approach developed during basic medical science education. This approach resulted in recognition of how much he thought he knew was actually contrary to the facts. A critical factor was the collaborative approach with specialists in other fields, wherein linguistic confusion was overcome and perspectives refined by point–counterpoint analysis of hypotheses. The limited reliability of tertiary information was clearly exposed through examination of primary sources – original articles rather than what might be referred to as "meta-analyses". It became clear that linguistics was not the only challenge; application of techniques had to be observed and validated. Without validation one might obtain precision (method repeatedly reveals same results) but at the expense of accuracy (assurance that the method actually assesses the question). Paleontological studies are generally limited to examination of organisms and their traces. Archeologically based studies incorporate additional sources of information (e.g., historic), but are no less subject to such semantic and methodological issues. Proof of concept studies provided new windows to recognition not only of disease but to previous anatomical challenges (e.g., localization of direct muscle attachment sites and distribution). Trans-phylogenetic representation of disease falsified speculation that "evolution" would preclude analysis through time. Pathology is an intrinsic component of life and transcends both species and time. Knowledge gained in a given species and time can be applied to similar disease manifestations in other species in modern time. Once speculations were tested and either verified or falsified, paleo-epidemiologic approach allowed identification of patterns of spread and even application of that knowledge to recognition of human migration patterns. Proof of concept studies provided new windows to recognition not only of disease but to previous anatomical challenges (e.g., localization of direct muscle attachment sites and distribution)

A new spontaneous model of fibrodysplasia ossificans progressiva.

Fibrodisplasia ossificante progressiva (FOP) é uma doença genética caracterizada por uma acentuada, progressiva e aparentemente incontrolável ossificação dos tendões, ligamentos, faciais e músculos estriados da formação de osso heterotópico resultando na imobilização em cadeiraa de rodas por 30 anos. Estudos mais avaçados relacionados a sua gestão foi sempre comprometida pela falta de um modelo animal natural. Esqueletos de mamíferos “Defleshed” foram examinados para demonstrar a evidência de formação de osso heterotópico. O cervo-rato do sudeste asiático do gênero Tragulus foi diagnosticado por possuir uma bainha ósseas cobrindo a parte inferior das costas e na região da coxa consistente com a definição clínica da FOP. Esta deposição óssea heterotophica está presente em todos os machos adultos, incluindo os obtidos selvagens e zoológico de animais desta linhagem. Nós relatamos o primeiro exemplo conhecido de natural de fibrodisplasia ossificante progressiva (FOP) em um mamífero não-humano. Tragulus pode oferecer a oportunidade de examinar muitos dos atributos mais importantes da doença experimentalmenteFibrodysplasia ossificans progressiva (FOP) is a genetic disorder characterized by relentlessly progressive and seemingly uncontrollable progressive ossification of tendons, ligaments, fascia, and striated muscle with heterotopic bone formation resulting in immobilization and wheel chair confinement by age 30. Progress in its management has been compromised by lack of a natural animal model. Defleshed mammal skeletons were examined for evidence of heterotopic bone formation. The Southeast Asian mouse deer of the genus Tragulus was found to have an osseous sheath covering the lower back and upper thigh region consistent with the clinical definition of FOP. This heterotophic bone deposition is present in all adults males, including both wild obtained and zoo bred animals. We report the first known example of spontaneous, naturally occurring fibrodysplasia ossificans progressiva (FOP) in a non-human mammal. Tragulus may offer the opportunity to examine many of the disease’s most significant attributes experimentally

The oldest record of aquatic amniote congenital scoliosis

This research was supported by the National Science Center (Narodowe Centrum Nauki, www.ncn.gov.pl), Poland, project no. 2011/ 01/N/ST10/06989 granted to D. Surmik. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. There was no additional external funding received for this study.We report the first occurrence of congenital scoliosis in an early Permian aquatic parareptile, Stereosternum tumidum from Paraná state, Brazil. The spine malformation is caused by a congenital hemivertebra. These observations give insight into the biomechanical aspects of underwater locomotion in an axial skeleton-compromised aquatic amniote. This is the oldest record of a hemivertebra in an aquatic animal.National Science Center (Narodowe Centrum Nauki, www.ncn.gov.pl