Gigantism Research Papers - Academia.edu (original) (raw)

In 1886 Pierre Marie used the term "acromegaly" for the first time and gave a full description of the characteristic clinical picture. However several others had already given clear clinical descriptions before him and sometimes had given the disease other names. After 1886, it gradually became clear that pituitary enlargement (caused by a pituitary adenoma) was the cause and not the consequence of acromegaly, as initially thought. Pituitary adenomas could be found in the great majority of cases. It also became clear that acromegaly and gigantism were the same disease but occurring at different stages of life and not different diseases as initially thought. At the end of the 19th and beginning of the 20th century most information was derived from case descriptions and post-mortem examinations of patients with acromegaly or (famous) patients with gigantism. The stage was set for further research into the pathogenesis, diagnosis and therapy of acromegaly and gigantism.

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- Gigantism, Acromegaly, Pituitary, Pituitary Neoplasms

Espaces de Virchow-Robin ; Dilatation des espaces périvasculaires géants ; Liquide céphalospinal (LCS)

- by Michel Lefranc
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- Magnetic Resonance Imaging, Gigantism, Hydrocephalus, Subarachnoid space

Exceptionally preserved fossils from the Palaeozoic era provide crucial insights into arthropod evolution, with recent discoveries bringing phylogeny and character homology into sharp focus. Integral to such studies are anomalocaridids, a clade of stem arthropods whose remarkable morphology illuminates early arthropod relationships and Cambrian ecology. Although recent work has focused on the anomalocaridid head, the nature of their trunk has been debated widely.Here we describe new anomalocaridid specimens from the Early Ordovician Fezouata Biota of Morocco, which not only show well-preserved head appendages providing key ecological data, but also elucidate the nature of anomalocaridid trunk flaps, resolving their homology with arthropod trunk limbs. The new material shows that each trunk segment bears a separate dorsal and ventral pair of flaps, with a series of setal blades attached at the base of the dorsal flaps. Comparisons with other stem lineage arthropods indicate that anomalocaridid ventral flaps are homologous with lobopodous walking limbs and the endopod of the euarthropod biramous limb, whereas the dorsal flaps and associated setal blades are homologous with the flaps of gilled lobopodians (for example, Kerygmachela kierkegaardi, Pambdelurion whittingtoni) and exites of the ‘Cambrian biramous limb’. This evidence shows that anomalocaridids represent a stage before the fusion of exite and endopod into the ‘Cambrian biramous limb’, confirming their basal placement in the euarthropod stem, rather than in the arthropod crown or with cycloneuralian worms. Unlike other anomalocaridids, the Fezouata taxon combines head appendages convergently adapted for filter-feeding with an unprecedented body length exceeding 2m, indicating a new direction in the feeding ecology of the clade. The evolution of giant filter-feeding anomalocaridids may reflect the establishment of highly developed planktic ecosystems during the Great Ordovician Biodiversification Event.

- by Peter Van Roy
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- Paleobiology, Paleontology, Functional Morphology, Invertebrate Biology

The Lancet Diabetes & Endocrinology. June 2017
Summary
Gigantism and acromegaly are endocrinological conditions of the greatest antiquity.1 While mythology and literature abounds with descriptions of giants, the oldest reported palaeopathological cases are remains of a person with acromegaly from 9500 to 11 500 years ago found in New Mexico, USA, and remains of a giant with signs of acromegaly from ancient Egypt (Giza; c 2425 BCE [5th Dynasty]). In 1901, a skeleton was found in the Mastaba K2 tomb near Beit Khallaf, Egypt, which is estimated to date from the 3rd Dynasty (c 2700 BCE).

- by Francesco Maria Galassi and +1
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- Ancient History, Archaeology, Egyptology, Ancient myth and religion

Sauropod (or, more correctly, eusauropod) dinosaurs are highly distinctive, not only in their overall body form, but also in respect to many details of their anatomy. In comparison with basal dinosaurs, typical sauropods are characterized by small skulls, elongate necks, massive bodies, and an obligatory quadrupedal stance with elongate forelimbs and straight limbs in general. Tracing the anatomical changes that led to this distinctive body plan through sauropodomorph evolution is problematic as a result of the incompleteness of many basal taxa and phylogenetic uncertainty at the base of the clade. The decrease in skull size in sauropodomorphs seems to be abrupt at the base of the clade, but it is even more pronounced toward sauropods. Major changes in the sauropod skull are a relative shortening and broadening of the snout and an enlargement and retraction of the nares. Although the ultimate causes for these evolutionary changes are certainly manifold, most if not all of them seem to be related to the ecological and biomechanical requirements of the transition from a carnivorous to an herbivorous lifestyle, in which the skull is
mainly used as a cropping device. A relatively elongate neck seems to be ancestral for sauropodomorphs, but the neck is further elongated on the lineage toward sauropods, especially by incorporation of two additional vertebrae at the base of Sauropoda. The relatively simple structure of the cervical vertebrae in basal sauropodomorphs might be a secondary reduction relative to basal saurischians as a result of changes in neck biomechanics in connection with the reduction of the size of the skull. Thus, the more complicated structure of sauropod cervicals probably reflects changing biomechanical requirements in connection with an elongation of the neck and
an increase in body size, as does the opisthocoelous structure of the cervical vertebral centra. Limb evolution in sauropodomorphs is dominated by adaptations toward increasing body size and thus graviportality, with the limbs getting straighter and the distal limb segments relatively shorter. Body size increase in sauropodomorphs seems to have been rapid but even-paced, with the ancestral body size of the clade being in the 0–10 kg category, and the ancestral body size for sauropods probably being in the 1,000–10,000 kg category.

Recent evidence for feathers in theropods has led to speculations that the largest tyrannosaurids, including Tyrannosaurus rex, were extensively feathered. We describe fossil integument from Tyrannosaurus and other tyrannosaurids (Albertosaurus, Daspletosaurus, Gorgosaurus and Tarbosaurus), confirming that these large-bodied forms possessed scaly, reptilian-like skin. Body size evolution in tyrannosauroids reveals two independent occurrences of gigantism; specifically, the large sizes in Yutyrannus and tyrannosaurids were independently derived. These new findings demonstrate that extensive feather coverings observed in some early tyrannosauroids were lost by the Albian, basal to Tyrannosauridae. This loss is unrelated to palaeoclimate but possibly tied to the evolution of gigantism, although other mechanisms exist.

- by Darren H Tanke
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- Biological Sciences, Gigantism, Fossils, Biological evolution

Ein Flügelfragment aus Seeablagerungen der Meisen heim-Formation des Unteren Rotliegend (Autunium) bei Obermoschel ist der erste Nachweis von Riesen-Urlibellen für das Permokarbon des Saar-Nahe-Beckens. Mit einer Flügelspannweite von mindestens 60 Zentimetern war das Tier in etwa so groß wie Meganeura monyi aus dem Stefanium von Commentry in Frankreich und ist das derzeit größte in Deutschland nachgewiesene Insekt. Als einen wahrscheinlichen Faktor, der den Riesenwuchs dieser Stammgruppenvertreter der heutigen Libellen erst möglich machte, diskutieren wir den hohen Sauerstoffgehalt der Atmosphäre im Oberkarbon bis Mittelperm. Die Paläoumwelt des Obermoschel-Sees war durch größere Seen und Flussläufe geprägt und bot den großen Meganeuriden die offenen Flächen, die sie als agile Flugjäger bevorzugten.
Abstract First record of giant griffenflies (Odonatoptera: Meganisoptera: Meganeuridae) in the Permocarboniferous of the Saar-Nahe Basin (Rhineland-Palatinate, SW-Germany)
A wing fragment from lake deposits of the Meisenheim Formation (Lower Rotliegend, Autunian) near Obermoschel is the first record of giant griffenflies for the Permo-Carboniferous of the Saar-Nahe Basin. With a reconstructed wing span of at least 60 centimetres the animal was comparable in size to the iconic Meganeura monyi from the Stephanian of Commentry/France and is currently the largest known insect from Germany. The increased partial pressure of oxygen in the Upper Carboniferous to mid-Permian is discussed as a possible factor controlling the maximum size of griffenflies. The palaeoenvironment of Lake Obermoschel was characterized by large lakes and fluvial systems that provided the open spaces preferred by meganeurids, which were probably agile hawker predators.

- by Markus Poschmann
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- Palaeoecology, Palaeontology, Odonatology, Gigantism

The herbivorous sauropod dinosaurs of the Jurassic and Cretaceous periods were the largest terrestrial animals ever, surpassing the largest herbivorous mammals by an order of magnitude in body mass. Several evolutionary lineages among Sauropoda produced giants with body masses in excess of 50 metric tonnes by conservative estimates. With body mass increase driven by the selective advantages of large body size, animal lineages will increase in body size until they reach the limit determined by the interplay of bauplan, biology, and resource availability. There is no evidence, however, that resource availability and global physicochemical parameters were different enough in the Mesozoic to have led to sauropod gigantism.We review the biology of sauropod dinosaurs in detail and posit that sauropod gigantism was made possible by a specific combination of plesiomorphic characters (phylogenetic heritage) and evolutionary innovations at different levels which triggered a remarkable evolutionary cascade. Of these key innovations, the most important probably was the very long neck, the most conspicuous feature of the sauropod bauplan. Compared to other herbivores, the long neck allowed more efficient food uptake than in other large herbivores by covering a much larger feeding envelope and making food accessible that was out of the reach of other herbivores. Sauropods thus must have been able to take up more energy from their environment than other herbivores.The long neck, in turn, could only evolve because of the small head and the extensive pneumatization of the sauropod axial skeleton, lightening the neck. The small head was possible because food was ingested without mastication. Both mastication and a gastric mill would have limited food uptake rate. Scaling relationships between gastrointestinal tract size and basal metabolic rate (BMR) suggest that sauropods compensated for the lack of particle reduction with long retention times, even at high uptake rates.The extensive pneumatization of the axial skeleton resulted from the evolution of an avian-style respiratory system, presumably at the base of Saurischia. An avian-style respiratory system would also have lowered the cost of breathing, reduced specific gravity, and may have been important in removing excess body heat. Another crucial innovation inherited from basal dinosaurs was a high BMR. This is required for fueling the high growth rate necessary for a multi-tonne animal to survive to reproductive maturity.The retention of the plesiomorphic oviparous mode of reproduction appears to have been critical as well, allowing much faster population recovery than in megaherbivore mammals. Sauropods produced numerous but small offspring each season while land mammals show a negative correlation of reproductive output to body size. This permitted lower population densities in sauropods than in megaherbivore mammals but larger individuals.Our work on sauropod dinosaurs thus informs us about evolutionary limits to body size in other groups of herbivorous terrestrial tetrapods. Ectothermic reptiles are strongly limited by their low BMR, remaining small. Mammals are limited by their extensive mastication and their vivipary, while ornithsichian dinosaurs were only limited by their extensive mastication, having greater average body sizes than mammals.

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monoostotic fi brous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies. Association of MAS with GH excess is rare, and in most of the instances somatotropinoma has not been documented. Treatment of patients of MAS with acromegaly is diffi cult because of thickened calvarium and dysplastic skull bone. We report a 17-year-old girl, who presented with cranio-facial fi brous dysplasia, caféau-lait macules and also had acromegaly due to pituitary macroadenoma, and treated with gamma knife radiosurgery.

- by Sridhar Subbiah
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- Magnetic Resonance Imaging, Radiosurgery, Adolescent, Gigantism
- by Karen Black
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- Vertebrate Palaeontology, Marsupials, Biological Sciences, Quaternary palaeontology
- by Mike Archer
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- Vertebrate Palaeontology, Marsupials, Biological Sciences, Quaternary palaeontology

A jaw fragment of a giant temnospondyl from the Upper Triassic or Lower Jurassic of Lesotho (southern Africa), initially regarded as a Triassic mastodonsaurid because of its size, is redescribed in detail and considered to be a member of the Brachyopoidea (Brachyopidae + Chigutisauridae sensu Warren and Marsicano [2000]) based on its dental morphology, presence of a well-developed ectopterygoid tusk, and the concavity of the ventral margin of the skull in lateral view. Recognition of the specimen as a brachyopid, rather than as a chigutisaurid, is of palaeobiogeographical significance in representing one of the youngest known brachyopids from Gondwana. The Lesotho specimen is also of palaeobiological interest in that an estimate of its overall size indicates that it represents one of the largest amphibians sensu lato ever known.

- by J.Sébastien Steyer
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- Southern Africa, Lesotho, Jurassic, Gigantism

I wrote about my big madular and it is very biutiful to watch because i love it very much from the bottom of mi hart.i strongly advise you to read this bullshit.

- by Tudor Savin
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- Gigantism

En el mundo ficcional rabelaisiano, universo dominado por la abundancia, las dimensiones y cantidades colosales, el cuerpo humano no puede menos que alcanzar su afirmación hiperbólica más completa: el gigantismo. El objetivo de este trabajo es estudiar las indicaciones que, sin concernir directamente la descripción del cuerpo de los gigantes, llevan al lector a inferir el aspecto colosal de tales personajes.

- by Susana G Artal Maillie
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- Renaissance drama, French Renaissance Literature, Rabelais, Gigantism

Aim: Macrodystrophia lipomatosa is a rare cause of gigantism of limb which can be confused with other common causes like congenital lymphedema. It presents usually with loss of function and cosmetic problems. Four cases are described with... more

- by Rizwan Khan
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- Gigantism, Case Report, Child, Foot
- by Karen Black
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- Vertebrate Palaeontology, Marsupials, Quaternary palaeontology, Gigantism

Endocrine disorders during growth and development that occur due to the secretion of excessive growth hormones are very rare in archaeological and modern populations. The human skeletal remains analyzed in this paper belong to an unusually tall woman, approximately 35-40 years of age, buried at the Đurine ćelije site (15th-17th century) in Serbia, who may have suffered from overgrowth syndrome. Individuals with this condition have an unusually tall stature and accompanying pathological changes related to organ systems and joint diseases. The aim of this paper is to show how the aforementioned disorder affected the health status of an individual (grave No. 7a) and the performance of daily activities, but also the attitude of the community towards deformities in the life of that same person and after death. Differential diagnosis was performed since other diseases tend to lead to similar health problems. During the analysis, changes in growth were noticed, and her stature was determined to be 186.42 cm. Compared to the documented stature of the medieval population from this period, the skeletal remains show a significant deviation from the average, especially when it comes to female individuals. Other pathological changes observed in this woman are osteoarthritis, spondyloarthropathy, and periosteal new bone formation.

- by Maja Miljević-Đajić
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- Paleopathology, Bioanthropology, Gigantism, Endocrine disorders
- by Catherine Gaither
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- Bioarchaeology, Bioarchaeology, Mimesis, Peru

Macrodystrophia lipomatosa is a rare form of non-hereditary congenital localized gigantism. Radiographs and magnetic resonance imaging of one such case in a 3-year-old girl is presented in which the entire right-lower limb was affected. Radiographs revealed excessive soft tissue hypertrophy and exostoses-like bony overgrowth from the metatarsals. Magnetic resonance images showed excess unencapsulated fatty proliferation and fatty infiltration of the involved muscles.

This report suggests the association of congenital diaphragmatic hernia in Simpson-Golabi-Behmel syndrome by describing two unrelated males with this malformation. One male was the maternal half-nephew of our previously reported 8-year-old boy with this syndrome.Review of the skeletal roentgenograms of these 2 affected males, and those of the previously reported 8-year-old, documents flare of the iliac wings, narrow sacroiliac notches, and the presence of two carpal ossification centers as a newborn (“advanced bone age”).We also report the follow-up of the 8-year-old boy, now 16 years old, who continues to have significant overgrowth and speech, dental, developmental, and adjustment problems. © 1993 Wiley-Liss, Inc.

- by Emily Chen
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- Genetics, Gigantism, Follow-up studies, American

A very tall skeleton was found during archaeological excavations in the territory of Fidenae, an administrative centre of the Roman territorial organization, situated along the Via Salaria about 7 km north of Rome (Italy). The individual was a young male, dated back to the Imperial Age (3 rd century AD), presenting a very tall but normally proportioned stature, estimated around 202 cm. The long bones showed incomplete epiphyseal union; therefore, the stature would probably have been taller, if he had lived longer. In this work, the metric data are compared with those from the Roman Imperial Age population, and differential diagnosis is discussed.

- by Simona Minozzi and +1
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- Endocrinology, Anthropology, Paleopathology, Gigantism

We reported on thirteen children affected with malformative syndromes associated with mental retardation. MRI examination showed in all of them different types of anomalies; agenesis of corpus callosum, ventricular dilatation and cerebral matter alterations were the most frequent findings. The authors give a brief description of the clinical reports and of the anomalies observed with MRI; they conclude that MRI seems to be a valuable tool especially for the study of the alterations of the cerebral matter and their correlation with mental retardation.

- by Roberto Rossi
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- Magnetic Resonance Imaging, Adolescent, Intellectual Disability, Mental Retardation

Growth hormone (GH) modulates the hypothalamic release of somatostatin and GH-releasing hormone; however, there has been no evidence of GH autoregulation on the pituitary somatotroph. To determine the effects of GH on its own regulation, we examined the pituitaries of giant transgenic mice expressing a GH agonist (E117L), dwarf transgenic mice expressing a GH antagonist (G119K), and dwarf mice devoid of the GH receptor/binding protein (GHR/BP). In the E117L transgenic mice, the number and distribution of pituitary GH-immunoreactive cells were unchanged from nontransgenic littermate controls; an ultrastructural examination revealed typical, densely granulated somatotrophs. In contrast, the pituitaries of the G119K mice contained both moderately granulated somatotrophs and a sparsely granulated (SG) population with well-developed synthetic organelles and a distinct juxtanuclear globular GH-staining pattern. GHR/BP-deficient mice exhibited a marked reduction in the intensity of cytoplasmic GH immunoreactivity; however, prominent GH staining in the juxtanuclear Golgi was seen. GH-immunoreactive cells were increased in number, and the reticulin network pattern was distorted; stains for proliferating cell nuclear antigen confirmed mild hyperplasia. Electron microscopy showed that the somatotrophs were hyperactive SG cells with prominent endoplasmic reticulum membranes, large Golgi complexes, and numerous mitochondria. These findings are consistent with synthetic and secretory hyperactivity in pituitary somatotrophs due to the reduced GH feedback regulation. The changes are most striking in animals that are devoid of GHR/BP and less marked in animals expressing a GH antagonist; both models had reduced insulin-like growth factor-I levels, but the more dramatic change in the GHR/BP animals can be explained by abrogated GH signaling. This represents

- by Sylvia L Asa
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- Electron Microscopy, Transgenic Mice, Growth Hormone, Gigantism

- by Heinrich Mallison
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- Theoretical Physics, Biology, Vertebrate Palaeontology, Vertebrate Paleontology

The skeletons of 2 famous acromegalic giants: Charles Byrne (1761-1783) and Henri Cot = Joseph Dusorc (1883-1912) and the embalmed body of the famous acromegalic giant Édouard Beaupré (1881-1904) all ended up in the medical collections of museums despite the fact that these patients had never donated or even refused to donate their corpses, nor had their relatives given permission. The corpse of the acromegalic giant John Aasen (1890-1938) was voluntarily donated to a physician annex collector of trivia from acromegalic giants. The autopsy on the acromegalic giant John Turner (1874-1911) was performed during his funeral ceremony without the relatives being informed. Only recently, the acromegalic giant Alexander Sizonenko (1959-2012) was made a financial offer during his life in exchange for his body after his death. The case-histories of these 6 patients and also the circumstances that led to the (in-) voluntary donation of their bodies are reviewed.

- by Wouter Herder
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- Medical Ethics, Gigantism, Physicians, Acromegaly

Familial acromegaly/gigantism occurring in the absence of multi- ple endocrine neoplasia type I (MEN-1) or the Carney complex has been reported in 18 families since the biochemical diagnosis of GH excess became available, and the genetic defect is unknown. In the present study we examined 2 unrelated families with isolated acro- megaly/gigantism. In family A, 3 of 4 siblings were

- by Mario Vaisman
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- Genetics, Polymorphism, Adolescent, Gigantism

X-linked acro-gigantism (X-LAG) is a new syndrome of pituitary gigantism, caused by microduplications on chromosome Xq26.3, encompassing the gene GPR101, which is highly upregulated in pituitary tumors. We conducted this study to explore the clinical, radiological and hormonal phenotype and responses to therapy in patients with X-LAG syndrome. The study included 18 patients (13 sporadic) with X-LAG and a microduplication in chromosome Xq26.3. All sporadic cases had unique duplications and the inheritance pattern in 2 families was dominant with all Xq26.3 duplication carriers being affected. Patients began to grow rapidly as early as 2-3 months of age (median 12 months). At diagnosis (median delay 27 months), patients had a median height and weight SDS score of >+3.9 SDS. Apart from the increased overall body size, the children had acromegalic symptoms including acral enlargement and facial coarsening. More than a third of cases had increased appetite. Patients had marked hypersec...

- by Elisa Verrua
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- Adolescent, Biological Sciences, Gigantism, Child

The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304* (or p.R304*; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304* carrier frequency in 936 Mid Ulster, 1000 Greater Belfast (both in NI) and 2094 Republic of Ireland (ROI) volunteers and in 116 acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027-0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011-0.0047) and zero in ROI (0-0.0014). R304* prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P < 0.05), but not in ROI (2/29, 6.8%) vs. non-Irish patients (0-2.41%). Haploblock conservation supported a common ancestor for all the 18 identified Irish pedigrees (81 carriers, 30 affected). Time to most recent common ancestor (tMRCA) was 2550 (1275-5000) years. tMRCA-based simulations predicted 432 (90-5175) curren...

- by Mark Thomas
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- Genetics, Risk, Adolescent, Ireland
- by Özlem Sert
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- Ottomanism, Turkish politics, Gigantism, City and Regional Planning
- by Paola Catalano
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- Ancient History, Archaeology, Funerary Archaeology, Indian ancient history

Entre les années 1970 et 1980, les écologistes ont identifié les principes théoriques qui auraient pu guider la gestion des villes et des territoires, et être mis en pratique dans des expériences micro-territoriales, germes d'autres mondes possibles. Mais une idéologie dominante couronne Megalopolis comme la seule forme possible de vie ensemble.

- by Ilaria Agostini
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- Ivan Illich, Regionalism, Lewis Mumford, Gigantism

The relaxation of predation and interspecific competition are hypothesized to allow evolution toward "optimal" body size in island environments, resulting in the gigantism of small organisms. We tested this hypothesis by studying a small teleost (ninespined stickleback, Pungitius pungitius) from four marine and five lake (diverse fish community) and nine pond (impoverished fish community) populations. In line with theory, pond fish tended to be larger than their marine or lake conspecifics, sometimes reaching giant sizes. In two geographically independent cases when predatory fish had been introduced into ponds, fish were smaller than those in nearby ponds lacking predators. Pond fish were also smaller when found in sympatry with three-spined stickleback (Gasterosteus aculeatus) than those in ponds lacking competitors. Size-at-age analyses demonstrated that larger size in ponds was achieved by both increased growth rates and extended longevity of pond fish. Results from a common garden experiment indicate that the growth differences had a genetic basis: pond fish developed two to three times higher body mass than marine fish during 36 weeks of growth under similar conditions. Hence, reduced risk of predation and interspecific competition appear to be chief forces driving insular body size evolution toward gigantism.

- by Gábor Herczeg
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- Evolutionary Biology, Evolution, Gigantism, Seawater

Based on the fossil record, we explore the macroevolutionary relationship between species richness and gigantism in cowries (Cypraeidae), the best-studied family of gastropods, with a global diversity distribution that parallels that of tropical corals, mangroves and seagrasses. We introduce Vicetia bizzottoi sp. nov. based on a Priabonian fossil found in northeastern Italy, the largest documented cowrie found so far and the youngest of a lineage of Eocene Gisortiinae species. The Gisortiinae stratigraphic record in western Europe indicates that species selection favoured large size and armouring of the shell. Palaeoecology and per-stage species richness suggest that gigantism occurred in peripheral habitats with respect to diversity hotspots, where smaller species were favoured. The Eocene-Oligocene boundary was marked by a turnover and the Chattian global warming favoured small-sized species of derived clades. Species selection leading to gigantism is further documented in Miocene lineages of Zoila and Umbilia, in the southern hemisphere, two extant genera distributed at the periphery of modern diversity hotspots, suggesting that the negative relationship between size and diversity is a recurring pattern in the evolutionary history of cowries. This palaeontological evidence is projected onto the existing hypotheses that explain analogous biogeographic patterns in various other taxa. Likewise, body size-species richness negative relationship was possibly driven in cowries by physiological, ecological and life history constraints.

- by Stefano Dominici
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- Evolutionary Biology, Adaptation, Paleoecology, Marine biodiversity

Giant gastropods are among the largest mollusks in the fossil record, but their potential as paleoseasonality archives has received little attention. Here, we combine stable isotope and trace element analyses with microscopic observations and growth modeling on shells of two species of the gastropod genus Campanile: the extinct Campanile giganteum from Lutetian (~45 Ma) deposits in the Paris Basin (France), the longest gastropod known from the fossil record, and its modern relative Campanile symbolicum from southwestern Australia. The C. giganteum shells contain original aragonite and have pristine nacre in their apertures. We show that these gastropods attained growth rates exceeding 600 mm/year along their helix, depositing over 300 cm 3 aragonite per year. High growth rates and excellent preservation make C. giganteum excellent archives for reconstructing environmental change at high (potentially daily) temporal resolution, while providing enough material for methods such as clumped isotope analysis. Growth models show that Campanile gastropods grew nearly year-round, albeit slower in winter. Stable oxygen isotope ratios in modern C. symbolicum faithfully record a seasonal variability of 18-25°C in sea surface temperature, only failing to record the coolest winter temperatures (down to~16°C). Similarly, C. giganteum specimens likely record a nearly complete seasonal temperature range. Assuming constant sea water isotope composition, their oxygen isotope seasonality of up to 2.5‰ would translate to a Lutetian temperature range of 21-32°C in the Paris Basin. We hypothesize that these high and seasonally variable temperatures formed the breeding ground for the Lutetian shallow marine biodiversity hotspot in the Paris Basin.

- by Niels de Winter
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- Paleoclimatology, Stable Isotopes, Mollusks, Gigantism

[first published in "Open monument : research into ephemeral, commemorative architecture and modernist patrimony ; [on the occasion of the Exhibition Open Monument at Kunstraum Kreuzberg, Bethanien Berlin, 4th May to 16th June 2013] / ed. by Bauer Stephane and Marta Jecu."]
The article deals with an unlikely unlikely alliance of time forging enthusiasts that group together as fossil manufacturers of all sorts: artists interested in future paleontology, poetic technology fans, animatronics workers and designers in dinosaur parks, inventors of lost civilizations, and archaeologists of theme parks and lost movie sets.

- by Tiron Stefan
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- Paleontology, Evolution, Philosophy of Time, Forgery, Fakery, Fraud

Although there are several reports on infant and childhood growth in patients with Sotos syndrome, there is little information on the final height achieved and puberty. Growth data on 40 patients (20 female and 20 male) aged 2-31 years were collected. These showed that patients with Sotos syndrome are excessively tall at birth, during infancy, and during childhood. Disproportionately long limbs constitute much of the increase in stature. However, the combination of advanced bone age and early onset of menarche led to a mean (SD) final height of 172.9 (5.7) cm in women. This is within the normal range for the population. Most of the men also attained a final height (mean, 184.3 cm; SD, 6.0) within the normal range, although exceptions were more likely in men than in women. Therefore, these results show that most patients with Sotos syndrome do not require intervention to limit their adult height. (Arch Dis Child 1999;80:339-342)

- by Jeremy Kirk and +1
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- Anthropometry, Adolescent, Growth, Gigantism

Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked multiple congenital abnormality/intellectual disability syndrome characterized by pre-and post-natal overgrowth, distinctive craniofacial features, macrocephaly, variable congenital malformations, organomegaly, increased risk of tumor and mild/moderate intellectual deficiency. In 1996, Glypican 3 (GPC3) was identified as the major gene causing SGBS but the mutation detection rate was only 28-70%, suggesting either genetic heterogeneity or that some patients could have alternative diagnoses. This was particularly suggested by some reports of atypical cases with more severe prognoses. In the family reported by Golabi and Rosen, a duplication of GPC4 was recently identified, suggesting that GPC4 could be the second gene for SGBS but no point mutations within GPC4 have yet been reported. In the genetics laboratory in Tours Hospital, GPC3 molecular testing over more than a decade has detected pathogenic mutations in only 8.7% of individuals with SGBS. In addition, GPC4 mutations have not been identified thus raising the question of frequent misdiagnosis. In order to better delineate the phenotypic spectrum of SGBS caused by GPC3 mutations, and to try to define specific clinical criteria for GPC3 molecular testing, we reviewed the clinical features of all male cases with a GPC3 mutation identified in the two molecular laboratories providing this test in France (Tours and Paris). We present here the results of the analysis of 42 patients belonging to 31 families and including five fetuses and three deceased neonates.

- by Jenneke Van Den Ende and +1
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- Genetics, Intellectual Disability, Gigantism, Congenital Heart Defects

We describe a pediatric disorder (which we have termed X-linked acrogigantism [X-LAG]) that is caused by an Xq26.3 genomic duplication and is characterized by early-onset gigantism resulting from an excess of growth hormone. Duplication of GPR101 probably causes X-LAG. We also found a recurrent mutation in GPR101 in some adults with acromegaly. Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and others.) The New England Journal of Medicine Downloaded from nejm.org on December 7, 2014. For personal use only. No other uses without permission.

- by Fabio Faucz
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- Adolescent, Gigantism, Child, Human Growth Hormone

Obesity is characterized by dysfunctional white adipose tissue (WAT) that ultimately may lead to metabolic diseases. Calorie restriction (CR) reduces the risk for age and obesity-associated complications. The impact of CR on obesity has been examined with human intervention studies, which showed alterations in circulating adipokines. However, a direct effect of CR on the human adipocyte secretome remains elusive. Therefore, the effect of a 96 h low glucose CR on the secretion profile of in vitro cultured mature human SGBS adipocytes was investigated by using proteomics technology. Low-glucose CR decreased the adipocyte triglyceride contents and resulted in an altered secretion profile. Changes in the secretome indicated an improved inflammatory phenotype. In addition, several adipocyte-secreted proteins related to insulin resistance showed a reversed expression after low-glucose CR. Furthermore, 6 novel CR-regulated adipocyte-secreted proteins were identified. Since resveratrol (RSV) mimics CR we compared results from this study with data from our previous RSV study on the SGBS adipocyte secretome. The CR and RSV adipocyte secretomes partly differed from each other, although both treatment strategies lead to secretome changes indicating a less inflammatory phenotype. Furthermore, both treatments induced SIRT1 expression and resulted in a reversed expression of detrimental adipokines associated with metabolic complications.

- by Edwin Mariman
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- Obesity, Proteomics, Intellectual Disability, Biological Sciences

X-linked acrogigantism (X-LAG) syndrome is a newly-described form of inheritable pituitary gigantism that begins in early childhood and is usually associated with markedly elevated growth hormone (GH) and prolactin secretion by mixed pituitary adenomas/hyperplasia. Microduplications on chromosome Xq26.3 including the GPR101 gene cause X-LAG syndrome. In individual cases random GH-releasing hormone (GHRH) levels have been elevated. We performed a series of hormonal profiles in a young female sporadic X-LAG syndrome patient and subsequently undertook in vitro studies of primary pituitary tumor culture following neurosurgical resection. The patient demonstrated consistently elevated circulating GHRH levels throughout preoperative testing, which was accompanied by marked GH and prolactin hypersecretion; GH demonstrated a paradoxical increase following TRH administration. In vitro, the pituitary cells showed baseline GH and prolactin release that was further stimulated by GHRH administra...

- by amira mohamed
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- Biological Sciences, Growth Hormone, Somatostatin, Gigantism

Mulinia lateralis, the dwarf surfclam, is a suitable model for bivalve genetics because it is hardy and has a short generation time. In this study, gynogenetic and triploid M . lateralis were successfully induced. For gynogenesis, eggs were fertilized with sperm irradiated with ultraviolet light and subsequently treated with cytochalasin B to block the release of the second polar body (PB2). Triploidy was induced by blocking PB2 in normally fertilized eggs. The survival of gynogenetic diploids was very low, only 0.7% to 8 days post-fertilization (PF), compared with 15.2% in the triploid groups and 27.5% in the normal diploid control. Larvae in all groups metamorphosed at 8-10 days PF, and there was no significant post-larval mortality. At sexual maturation (2-3 months PF), all gynogenetic diploids were female, and there was no significant difference ( P > 0.05) in sex ratio between diploids and triploids. These results suggested that the dwarf surfclam may have an XX-female, XY-male sex determination with Ydomination. Compared with diploids, triploids had a relative fecundity of 59% for females and 80% for males. Eggs produced by triploid females were 53% larger ( P C 0.001) in volume than those from diploid females. In both length and weight measurements at three months PF, the gynogenetic diploids were not significantly ( P > 0.33) different from normal diploid females, suggesting that inbreeding depression was minimal in meiosis I1 gynogens.

- by Ximing Guo
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- Genetics, Fertility, Polyploidy, Gigantism

This study examines the skeletons of giant non-metamorphosing (GNM) Xenopus laevis tadpoles, which arrest their development indefinitely before metamorphosis, and grow to excessively large sizes in the absence of detectable thyroid glands. Cartilage growth is isometric; however, chondrocyte size is smaller in GNM tadpoles than in controls. Most cartilages stain weakly with alcian blue, and several cartilages are calcified (unlike controls). However, cartilages subjacent to periosteum-derived bone retain strong affinities for alcian blue, indicating a role for periosteum-derived bone in the retention of glycosaminoglycans during protracted larval growth. Bone formation in the head, limb, and axial skeletons is advanced in comparison with stage-matched controls, but arrests at various mid-metamorphic states. Both dermal and periosteum-derived bones grow to disproportionately large sizes in comparison to controls. Additionally, mature monocuspid teeth form in several GNM tadpoles. Advances in skeletal development are attributable to the old ages and large sizes of these tadpoles, and reveal unexpected developmental potentials of the pre-metamorphic skeleton.

- by Ryan Kerney and +1
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- Biomedical Engineering, Anatomy, Gigantism, Cartilage