Ectromelia (Phocomelia)

03/01/2009 - "The increased efficacy of CMX001 in C57BL/6 over A/Ncr following an intranasal infection with ectromelia appears to be mediated by a stronger Th1 cell mediated response. "
11/01/2014 - "We have used the intranasal mousepox (ectromelia) model to evaluate the efficacy of vaccination with Dryvax or ACAM2000 in conjunction with treatment using the broad spectrum antiviral, brincidofovir (BCV, CMX001). "
10/01/2010 - "HDP-CDV is a highly effective agent against a variety of orthopoxvirus infections in animal models of disease including vaccinia, cowpox, rabbitpox and ectromelia. "

06/11/1996 - "Using mice deficient for granzyme A, we show here that granzyme A plays a crucial role in recovery from the natural mouse pathogen, ectromelia, by mechanisms other than cytolytic activity."
01/01/2001 - "Recent studies have demonstrated that granzymes A and B make an important contribution to the clearance of the orthopoxvirus ectromelia, and in graft versus host disease. "
01/01/2009 - "Here we analysed the physiological relevance of those pro-apoptotic pathways in ectromelia infection, by incubating ectromelia-immune ex vivo Tc cells from granzyme A deficient (GzmB(+) Tc cells) or granzyme A and granzyme B deficient (GzmAxB(-/-) Tc cell) mice with ectromelia-infected target cells. "
06/11/1996 - "Granzyme A is critical for recovery of mice from infection with the natural cytopathic viral pathogen, ectromelia."
11/23/1999 - "In this paper, we show that mice lacking both granzyme A (gzmA) and granzyme B (gzmB), which are, beside perforin, key constituents of cytolytic vesicles, are as incapable as are perforin-deficient mice of controlling primary infections by the natural mouse pathogen ectromelia, a poxvirus. "

10/01/2006 - "Hexadecyloxypropyl-cidofovir is orally bioavailable and active in lethal animal models of vaccinia, cowpox, ectromelia and cytomegalovirus. "
12/01/2010 - "Among poxviruses, cidofovir has shown in vitro activity against orthopox [vaccinia, variola (smallpox), cowpox, monkeypox, camelpox, ectromelia], molluscipox [molluscum contagiosum] and parapox [orf] viruses. "
04/01/2005 - "The efficacy of cidofovir treatment of mice infected with ectromelia (mousepox) virus encoding interleukin-4."
10/01/2006 - "Alkoxyalkyl esters of cidofovir, an acyclic nucleoside phosphonate, have been shown to have antiviral activities several orders of magnitude greater than unmodified cidofovir against cytomegalovirus, herpes simplex virus, vaccinia, cowpox, ectromelia and adenoviruses in vitro. "
05/01/2011 - "Alkoxyalkyl esters of cidofovir (CDV) are orally active agents which inhibit the replication of a variety of double stranded DNA (dsDNA) viruses including variola, vaccinia, ectromelia, herpes simplex virus, cytomegalovirus, adenovirus and others. "

04/01/2005 - "The efficacy of cidofovir treatment of mice infected with ectromelia (mousepox) virus encoding interleukin-4."

01/01/2009 - "Interest in studies of the teratogenic effects of drugs increased in response to reports of the high incidence of phocomelia in patients treated with thalidomide. "
11/01/1981 - "In the case of thalidomide, the drug's ability to cause phocomelia tended to be species specific, and thus animal studies were unreliable indicators of teratogenicity in humans. "
06/02/2012 - "The results showed that the groups with the most classical limb malformations attributed to thalidomide (phocomelia, thumb absence/hypoplasia) had a significantly very higher frequency in exposed cases than in the ECEMC's cases. "
03/01/2012 - "Maternal thalidomide intake during limb development is known to cause limb reduction and subsequent amelia or phocomelia. "
10/01/2011 - "These developmental toxicology protocols were a direct result of the thalidomide tragedy from earlier that decade, when large numbers of women were exposed to the drug and over 10,000 cases of phocomelia resulted. "

04/01/2006 - "The purpose of the ultrasonographic study is to identify the sirenomelia as early as possible during pregnancy and to differentiate it from caudal regression syndrome. "
03/01/2016 - "Even though the etiology of caudal regression syndrome (CRS) and sirenomelia remains unknown we tend to believe that sirenomelia and CRS might be different entities."
03/01/2016 - "Sirenomelia: a review of embryogenic theories and discussion of the differences from caudal regression syndrome."
12/01/2014 - "To describe cases of sirenomelia and severe caudal regression syndrome (CRS), to report the prevalence of sirenomelia, and compare our findings with the literature. "
12/01/2014 - "Sirenomelia and severe caudal regression syndrome."

04/01/2013 - "Studies consider sirenomelia as the most severe form of caudal dysgenesis, VACTERL association, or axial mesodermal dysplasia, although others still support the idea of a different pathologic entity. "
06/01/2008 - "[Sirenomelia as a part of VACTERL association: a study of three cases]."
07/01/2012 - "The clinical phenotypic overlap between caudal dysgenesis, VACTERL association and sirenomelia in our patients is highlighted, lending support to the theory that these entities may be different manifestations of a single pathogenic process."
07/01/2010 - "Sirenomelia and VACTERL association are defects of blastogenesis of unknown cause. "
07/01/2010 - "Sirenomelia and VACTERL association in the offspring of a woman with diabetes."

01/01/2010 - "The importance of chromosome studies in Roberts syndrome/SC phocomelia and other cohesinopathies."
06/01/2012 - "Roberts syndrome and SC phocomelia (RBS/SC) are genetic autosomal recessive syndromes caused by establishment of cohesion 1 homolog 2 ( ESCO 2) mutation. "
06/01/2012 - "Establishment and characterization of Roberts syndrome and SC phocomelia model medaka (Oryzias latipes)."
04/15/2010 - "In humans, mutations in cohesion factors result in developmental abnormalities such as Cornelia de Lange, Roberts Syndrome/SC-Phocomelia, Rothman-Thompson Syndrome and others. "
02/01/2010 - "The Roberts syndrome/SC phocomelia spectrum--a case report of an adult with review of the literature."

03/01/1997 - "DNA available for 6 of 8 Old World (cowpox, variola, monkeypox, camelpox, ectromelia and vaccinia viruses) and 3 New World (skunkpox, volepox, and raccoonpox) resulted in amplicons that ranged in size from 510 to 1673 base pairs depending on the species, except for raccoonpox virus DNA which did not amplify. "
08/01/2003 - "The assay was evaluated in a blinded study with 322 coded samples that included genomic DNA from 48 different isolates of variola virus; 25 different strains and isolates of camelpox, cowpox, ectromelia, gerbilpox, herpes, monkeypox, myxoma, rabbitpox, raccoonpox, skunkpox, vaccinia, and varicella-zoster viruses; and two rickettsial species at concentrations mostly ranging from 100 fg/ microl to 1 ng/ microl. "
08/01/1995 - "The initial identification step used a primer pair of consensus sequences for amplifying an HA DNA fragment from the three known North American orthopoxviruses (raccoonpox, skunkpox, and volepox viruses), and a second pair for amplifying virtually the entire HA open reading frame of the Eurasian-African orthopoxviruses (variola, vaccinia, cowpox, monkeypox, camelpox, ectromelia, and gerbilpox viruses). "
09/01/1993 - "Genes encoding virus-specific proteins with molecular masses of 36 kDa and 12 kDa were mapped in HindIII-P and HindIII-U DNA fragments of vaccinia strain LIVP and ectromelia strain K-1 viruses, respectively, by hybrid selection of RNA to cloned DNA fragments followed by in vitro translation. "
02/01/1985 - "Elevated rate of DNA synthesis and its correlation to cAMP-phosphodiesterase activity during induction of polydactyly in mouse embryos heterozygous for Hemimelia-extra toe (Hmx)."

03/01/1998 - "This report supports the hypothesis that VATER association, VACTERL-H association, and sirenomelia may represent pathophysiologically related entities."
03/01/1998 - "It has been suggested that the VATER association may represent a less severe form of sirenomelia. "
11/01/1991 - "In addition, sirenomelia patients were found in 12%, the VATER association in 27%, and 27% could not be classified. "
01/01/1986 - "Review of the literature indicates that the basic defect in sirenomelia and the VATER association lies in the formation and differentiation of mesodermal tissue and that sirenomelia, the VATER association, and monozygotic twinning show a complex etiological interrelationship."
01/01/2003 - "Syndromes with associated preaxial reduction defects mainly Fanconi pancytopenia, VATER association, Rothmund-Thompson and Roberts phocomelia syndrome were excluded by proper clinical and cytogenetic studies. "

09/01/2014 - "Nine patients with fibular hemimelia who underwent tibial lengthening using the Ilizarov method were reviewed in the present study. "
01/01/2010 - "The aim of this study is to evaluate the long-term results of management of fibular hemimelia (Achterman-Kalamchi type-II) using the Ilizarov method. "
09/01/2014 - "Management of fibular hemimelia using the Ilizarov method at Siriraj Hospital in Thailand."
09/01/2001 - "Fourteen cases in thirteen patients, mean age 14.8 years, with radial hemimelia (one bilateral surgery) were treated with the Ilizarov method between June 1981 and December 1998 at the Lecco Hospital, Italy. "
09/01/1998 - "We report three patients (four limbs) with type-II fibular hemimelia who were treated by the Ilizarov technique and followed up for two to six years. "

11/01/2009 - "For several years, a genetic disease called transversal hemimelia (TH), also known as congenital amputation, has been spreading in Mediterranean Italian buffalo. "
03/01/2006 - "Although lengthening in fibular hemimelia is difficult, elongation with axis and foot correction may offer an alternative to amputation."
03/01/2006 - "Limb lengthening in fibular hemimelia type II: can it be an alternative to amputation?"
06/01/2002 - "The results of our cases and those reported in the literature suggest early amputation as the treatment of choice of Type II fibular hemimelia."
02/01/2002 - "Limb-lengthening versus amputation for fibular hemimelia."

12/01/2014 - "Simultaneous femoral and tibial lengthening in combined congenital complete fibular hemimelia and congenital short femur using Ilizarov ring external fixator."
01/01/2005 - "[Management of fibular hemimelia with the Ilizarov circular external fixator]."
01/01/2005 - "A 6-month-old female crossbred dog with unilateral radial hemimelia was treated with a circular external fixator. "
10/01/1999 - "The authors report their experience with 20 arthroscopies diagnosed in 20 children affected with congenital hypoplasia of the lower limb (shoft congenital femur, longitudinal peroneal hemimelia) performed prior to assembling the Ilizarov external fixator for femoral and/or tibial lengthening. "
01/01/2005 - "We evaluated the results of treatment with the Ilizarov circular external fixator for limb length inequality and deformities in patients with type IA, IB, and type II fibular hemimelia. "

09/01/2007 - "To assess the prosthesis adaptability at use of pedestal and transtibial prosthesis, recognize the average average age of amputation surgery, and the complications of the amputation in patients with fibular hemimelia. "
09/01/2003 - "We report a 5-year-old boy with a transverse partial hemimelia who developed allergic contact dermatitis to an adhesive used in his myoelectric prosthesis. "
11/01/2012 - "We present herein two cases: a 38 year-old female patient who underwent Chopart amputation after a gunshot wound and developed a plantar flexion deformity that prevented proper use of the prosthesis; and a 39 year-old male with sequela of fibular hemimelia, with severe foot deformity preventing proper use of the extremity and footwear. "

01/01/2010 - "The patient with ulnar hemimelia had a history of shish-kabab osteotomy at another center for ulnar bowing. "
01/01/2003 - "Bending osteotomy through the distal tibial physis in fibular hemimelia for stable reduction of the hindfoot."