Osteogenesis imperfecta, Ehlers-Danlos syndrome, and Marfan syndrome form a group of genetic disorders of connective tissue. These disorders exhibit remarkable clinical heterogeneity which reflects their underlying biochemical and molecular differences. Defects in collagen types I and III have been found in all three syndromes.

Wilhelmina Strasheim, Marleen M. Kock, Andries W. Dreyer and Marthie M. Ehlers Department of Medical Microbiology, Faculty of Health Science, University of Pretoria, Private Bag x323, Arcardia Pretoria, 0007, Gauteng, South Africa Department of Medical Microbiology, Faculty of Health Science, University of Pretoria, National Health Laboratory Services, Tshwane Academic division (NHLS/TAD), Gaut...

Isolated visceral arteriopathies of the celiac and hepatic artery are rare. We present a case of a Caucasian man who presented with abdominal pain and was found to have a spontaneous celiac artery dissection. Genetic analysis revealed a mutation consistent with Ehlers-Danlos syndrome type IV. The patient died 2 months later from a spontaneous rupture of his hepatic artery.

Clairin’s method of obtaining Bäcklund transformations is applied to Einstein’s field equations for the interior of a uniformly rotating stationary axisymmetric perfect fluid. It is shown that for arbitrary pressure p and mass density μ the method does not give non-trivial Bäcklund transformations, while if μ+3p = 0 it gives the transformation of Ehlers. PACS number(s): 04.20.Jb, 04.20.Ex

We describe two patients with associations of hereditary neuropathies and heritable skeletal disorders not previously reported. The first patient had Marfan's syndrome and hereditary motor and sensory neuropathy Type 1. The second patient had Ehlers-Danlos syndrome, Klippel-Feil syndrome and tomaculous neuropathy.

How are the responses to distinct chemical features integrated to form an olfactory perceptual object? In this issue of Neuron, Davison and Ehlers show that individual piriform cortex neurons receive convergent input from up to 10% of main olfactory bulb glomeruli and are activated by specific spatial patterns of coactive glomeruli.

Kristen (Coen) Cruz Phil Baranski Anna Lard Dean Curosmith Michael Vitale Brandy Lee Hatcher Shannon G. Bicknell Will Pruett Julianna Brei Crawley Cambria Larson Matthew Jackson Jenny Jacobs Ashley Boehne Ehlers Michelle McDougall Jackson Jeff Whetstone Sarah Guadalupe Hosner Ryan Neely Matt McNally Anne Hitt Penn Ross Jackson Amanda Novoa Ian Daelucian Amber Julian Gabrielle Koizumi Sammy Brow...

Amniotic bands are described in two children with Ehlers-Danlos syndrome type IV and in one with severe osteogenesis imperfecta. Since the basic defect in both of these disorders rests in collagen, which constitutes the main component of the load bearing amnion, it is proposed that these cases provide further insight into the pathogenesis of amniotic bands.

BACKGROUND Skin malformations that resembled manifestations of Ehlers-Danlos-Syndrome were described in a variety of domestic animals during the last century as cutis hyperelastica, hyperelastosis cutis, dermatosparaxis, dermal/collagen dysplasia, dermal/cutaneous asthenia or Ehlers-Danlos-like syndrome/s. In 2007, the mutation responsible for Hereditary Equine Regional Dermal Asthenia (HERDA) ...

BACKGROUND Spinal deformities in Ehlers-Danlos syndrome are usually progressive and may require operative treatment. There is limited number of studies describing late results of surgery in this disease. METHODS This is a retrospective study of the records of 11 patients with Ehlers-Danlos syndrome type IV, treated surgically between 1990 and 2007. All patients underwent surgical treatment fo...