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Sample records for neurocutaneous vascular syndromes

  1. Neurocutaneous syndromes; Neurokutane Erkrankungen

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    Niederstadt, T. [Universitaetsklinikum Muenster (Germany). Inst. fuer Klinische Radiologie; Kurlemann, G. [Muenster Univ. (Germany). Medizinische Fakultaet

    2007-09-15

    Neurocutaneous Syndromes or phakomatoses are a heterogenous group of congenital diseases. They are characterized by dysplasias of tissues derived from the neuroektoderm. Skin alterations may be helpful in the interpretation of cerebral lesions. Recently, the genetic and pathophysiologic alterations of many phakomatoses have been elucidated. In this paper the radiologic findings and clinical signs of the most common neurocutaneous diseases (Neurofibromatosis 1 and 2, Tuberous Sclerosis Complex and Sturge Weber Syndrome) will be discussed. (orig.)

  2. Neurocutaneous syndrome: A prospective study

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    Radheshyam Purkait

    2011-01-01

    Full Text Available Background: Neurocutaneous syndromes (NCS are a group of genetic disorders that produce a variety of developmental abnormalities of the skin along with an increased risk of neurological complications. Cutaneous manifestations usually appear early in life and progress with time, but neurological features generally present at a later age. There is a paucity of data regarding the evolution of skin lesions and their correlation with the central nervous system involvement in children. Aim: The primary objective was to track the course of skin lesions in various forms of NCS in the pediatric age group. Our secondary aim was to assess whether there was any predictive value of the lesions in relation to the neurological manifestations. Materials and Methods: This prospective longitudinal study was conducted at a tertiary care pediatric dermatology referral clinic of the Institute of Child Health, Kolkata, West Bengal. Children between the age group 0 and 12 years were included in the study on the basis of standard diagnostic criteria for different NCS, during the period from March, 2000 to February, 2004, and each of the enrolled cases were followed up for a duration of six years. Results: The study population comprised of 67 children (35 boys, 32 girls.The mean age of presentation was 33.8±27.8 months (range 10 days to 111 months. The various forms of NCS observed was neurofibromatosis 1(NF1 (n=33, tuberous sclerosis complex (TSC (n=23, Sturge Weber syndrome (n=6, ataxia telangiectasia (n=2, PHACE syndrome (n=1, incontinentia pigmenti (n=1, and hypomelanosis of Ito (n=1. The presentations were varied, ranging from predominantly cutaneous to primarily neurological, depending on the disease entity and age group concerned. There was a significant increase in the number of café au lait macules (CALMs with time (P=0.0002 in NF1, unlike that of hypopigmented macules of TSC (P=0.15. Statistically, no relation was documented between the evolution of skin

  3. Genetics of neurocutaneous disorders: basic principles of inheritance as they apply to neurocutaneous syndromes.

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    Dies, Kira A; Sahin, Mustafa

    2015-01-01

    Neurocutaneous disorders vary widely in clinical presentation as well as genetic cause and inheritance pattern. Recent advancements in genetic research have identified many of the causal genes for neurocutaneous disorders, allowing families to receive genetic testing and genetic counseling to better understand carrier risks, recurrence risks for future generations, and reproductive options such as prenatal testing and preimplantation diagnosis. Examples of specific neurocutaneous disorders are utilized to illustrate the various inheritance patterns seen in this heterogeneous group of disorders, including autosomal dominant, autosomal recessive, X-linked dominant, X-linked recessive, de novo, and somatic and germline mosaicism. © 2015 Elsevier B.V. All rights reserved.

  4. Epileptogenesis in neurocutaneous disorders with focus in Sturge Weber syndrome [version 1; referees: 2 approved

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    Anna Pinto

    2016-03-01

    Full Text Available Epilepsy is a major morbidity in Sturge Weber syndrome, a segmental vascular neurocutaneous disorder classically associated with facial angiomas, glaucoma, and leptomeningeal capillary-venous type vascular malformations. The extent of the latter correlates with neurological outcome. Post-zygotic mosaicism for the activating mutation p.R183Q of the GNAQ gene has been identified as the major cause. GNAQ encodes for an alpha subunit of a heterotrimeric G protein critical to blood vessel development. The earlier the timing of the mutation in development, the more severe the involvement, e.g. from isolated port-wine stains to the full syndrome. The strongest predictors of adverse outcomes are MRI and the presence of angiomas involving any part of the forehead, delineated inferiorly from the outer canthus of the eye to the top of the ear, and including the upper eyelid.  The neurological course may be progressive and the typical constellation of symptoms is focal onset seizures, hemiparesis, headache, stroke-like episodes, behavior problems, intellectual disability, and visual field deficits. Antiseizure medications are effective in about half of patients. The presence of localized seizures, focal neurological deficits, and drug resistant epilepsy indicate epilepsy surgical evaluation. Earlier seizure onset, i.e. before six months of age, is associated with a more severe course with significant residual deficits. Factors contributing to epileptogenesis include decreased brain tissue perfusion due to abnormal venous drainage, anoxic injury contributing to cerebral calcification, breakdown of the blood-brain barrier, and the presence of developmental cortical malformations. Pre-symptomatic prophylactic treatment may be a future option to modify the course of the disease including the associated epileptogenesis.

  5. Neurocutaneous Syndromes

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    ... of seizures , learning disabilities, attention deficit disorder (ADHD) , autism , and speech problems . Therapy and specialists can help manage those symptoms. Neurofibromatosis Type 2 Neurofibromatosis type 2 is less common, ...

  6. Neurocutaneous syndrome with mental delay, autism, blockage in intracellular vescicular trafficking and melanosome defects.

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    Buoni, S; Zannolli, R; de Santi, M; Macucci, F; Hayek, J; Orsi, A; Scarinci, R; Buscalferri, A; Cuccia, A; Zappella, M; Miracco, C

    2006-08-01

    We evaluated a 11-year-old male patient with mental delay, autism and brownish and whitish skin spots. The former resembled those of neurofibromatosis, the latter those of tuberous sclerosis. The patient received a complete clinical work-up to exclude neurofibromatosis, tuberous sclerosis, or any other known neurocutaneous disease, with biochemistry, chromosome analysis and analysis of skin specimens. Being all the other tests not significant, two main ultrastructural defects were observed. The first was a blockage in intracellular vescicular trafficking with sparing of the mitochondria; the second an aberrant presence of melanosomes in vacuoles of several cell lines and abnormal transfer of these organelles to keratinocytes. This patient presented with a unique clinical picture distinct from neurofibromatosis or tuberous sclerosis or any other known neurocutaneous disease. The ultrastructural abnormalities suggested a defect in cell trafficking involving several cell lines and compartments.

  7. Multidisciplinary approach for evaluation of neurocutaneous ...

    African Journals Online (AJOL)

    Abdelrahim A. Sadek

    2015-03-05

    Mar 5, 2015 ... Abstract Background: Neurocutaneous syndromes (NCS) are a broad term for a group of neuro- logic disorders that involve the nervous system and the skin. The most common examples are neu- rofibromatosis type 1 (NF-1) and type 2 (NF-2), tuberous sclerosis (TS), Sturge–Weber syndrome. (SWS) ...

  8. Neurocutaneous Melanosis: A Case Report

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    Seo, Yoon Nae; Jeong, Hae Woong; In, Hyun Sin [Dept. of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2011-12-15

    Neurocutaneous melanosis is a rare disorder characterized by the presence of a large or multiple congenital melanocytic nevus with proliferation of melanocytes in the central nervous system. The prognosis of neurocutaneous melanosis is extremely poor and its diagnostic approach requires understanding its brain magnetic resonance imaging findings. We report a patient with asymptomatic neurocutaneous melanosis and its radiologic findings.

  9. INCIDENCE AND CLINICOPATHOLOGICAL FEATURES OF NEUROCUTANEOUS DISORDERS

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    S. Kayalvizhi Money

    2017-08-01

    Full Text Available BACKGROUND Neurocutaneous disorders are genetically determined disorders showing both cutaneous and neurologic involvement. The definition includes both hereditary and non-hereditary phenotypes, but excludes acquired disorders. Either they follow the established Mendelian modes of inheritance or they represent lethal mutations surviving by mosaicism or they belong to the group of chromosomal disorders. MATERIALS AND METHODS This study was conducted at the Department of Dermatology, Government KAPV Medical College, Trichy, for a period of 12 months from January 2016 to December 2016. Patients were selected among those attending the outpatient department with signs and symptoms pertaining to neurocutaneous syndromes. Preliminary information like age, sex, educational qualification, present and past illness, family history elicited. Dermatological examination consisted of thorough screening of patients to detect the cutaneous markers for neurocutaneous disorders. A detailed systemic examination was done, particularly central nervous system. RESULTS In this study, neurofibromatosis (68.8% topped the list followed by tuberous sclerosis complex (18.3% and other rarer disorders like xeroderma pigmentosum (2.7%, giant congenital melanocytic naevus (1.8%, Sturge-Weber syndrome (0.9%, Waardenburg syndrome (1.8%, epidermal naevus syndrome (1.8%, naevus comedonicus (0.9%, Elejalde syndrome (0.9%, oculocutaneous albinism (0.9% and Adams-Oliver syndrome (0.9%. CONCLUSION In this study of 109 cases of neurocutaneous syndromes, neurofibromatosis topped the list followed by tuberous sclerosis complex. Classical features of xeroderma pigmentosum was observed in 1 patient. Sturge-Weber syndrome with unilateral port wine stain with seizures was reported in our study. Two cases of Waardenburg syndrome, epidermal nevus syndrome and giant congenital melanocytic nevus were reported in my study. One case of unilateral nevus comedonicus, Elejalde syndrome, oculocutaneous

  10. Multidisciplinary approach for evaluation of neurocutaneous ...

    African Journals Online (AJOL)

    Multidisciplinary approach for evaluation of neurocutaneous disorders in children in Sohag University Hospital, Upper Egypt. ... rhabdomyoma. Conclusion: Neurocutaneous disorders had multiple clinical presentations and required a team work approach including various specialties in their evaluation and management.

  11. Abdominal vascular syndromes: characteristic imaging findings

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    Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D' Ippolito, Giuseppe, E-mail: leandrocleite@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Mediciana. Departmento de Diagnostico por Imagem

    2016-07-15

    Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

  12. MR imaging of symptomatic neurocutaneous melanosis in children

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    Byrd, S.E. [Div. of Neuroimaging, Dept. of Radiology, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States); Darling, C.F. [Div. of Neuroimaging, Dept. of Radiology, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States); Tomita, T. [Div. of Pediatric Neurosurgery, Dept. of Surgery, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States); Chou, P. [Dept. of Pathology, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States); De Leon, G.A. [Dept. of Pathology, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States); Radkowski, M.A. [Div. of Neuroimaging, Dept. of Radiology, Children`s Memorial Hospital and Northwestern Medical School, Chicago, IL (United States)

    1997-01-01

    Neurocutaneous melanosis is a syndrome consisting of cutaneous nevi and melanocytosis of the leptomeninges. Over a 5-year period (1989-1994) we evaluated with MR imaging the central nervous system of five children with a confirmed histologic diagnosis of neurocutaneous melanosis. The children ranged in age from 7 to 10 years and consisted of two girls and three boys. They all had multiple pigmented skin lesions (cutaneous nevi) and presented with seizures, signs of raised intracranial pressure, cranial nerve palsies and/or myelopathy. The MR studies were performed with T1-weighted, T2-weighted and T1-weighted post-gadolinium images of the brain in addition to T1-weighted post-gadolinium images of the entire spine. The MR findings in all the children consisted of marked, diffuse enhancement of thickened leptomeninges surrounding the brain and spinal cord which was only demonstrated on the post-gadolinium T1-weighted images and mild to moderate hydrocephalus. We present our MR findings and compare these findings with other imaging findings in the literature. Our findings represent part of a spectrum of imaging abnormalities seen in patients with neurocutaneous melanosis. (orig.). With 6 figs., 1 tab.

  13. Maternal homocystinuria and Moebius syndrome? Vascular aetiology

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    Gupta, N.; Anthony, M Y

    2011-01-01

    A case of Moebius syndrome is reported in an infant of a mother known to have pyridoxine-unresponsive homocystinuria. The authors suggest that Moebius syndrome could result from early vascular insufficiency or disruption occurring early in development related to maternal homocystinuria.

  14. Maternal homocystinuria and Moebius syndrome? Vascular aetiology.

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    Gupta, N; Anthony, M Y

    2011-02-14

    A case of Moebius syndrome is reported in an infant of a mother known to have pyridoxine-unresponsive homocystinuria. The authors suggest that Moebius syndrome could result from early vascular insufficiency or disruption occurring early in development related to maternal homocystinuria. Moebius syndrome consists of congenital complete or partial facial nerve palsy with or without paralysis of other cranial nerves and often in association with other malformations of the limbs and orofacial structures, but usually without gross structural brain abnormalities.

  15. Arteriovenous Malformations and Other Vascular Malformation Syndromes

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    Whitehead, Kevin J.; Smith, Matthew C. P.; Li, Dean Y.

    2013-01-01

    Vascular malformations are a disruption of the normal vascular pattern in which it is expected that a capillary network of microscopic vessels lies interposed between high-pressure arteries that deliver blood and thin-walled veins that collect low-pressure blood for return to the heart. In the case of arteriovenous malformations, arteries or arterioles connect directly to the venous collection system, bypassing any capillary bed. Clinical consequences result from rupture and hemorrhage, from dramatically increased blood flow, or from the loss of capillary functions such as nutrient exchange and filtering function. These malformations can occur sporadically or as a component of inherited vascular malformation syndromes. In these and other hereditary vascular malformation syndromes, genetic studies have identified proteins and pathways involved in vascular morphogenesis and development. A common theme observed is that vascular malformations result from disruption in pathways involved in vascular stability. Here we review the vascular malformations and pathways involved in hereditary hemorrhagic telangiectasia, capillary malformation–arteriovenous malformation, cerebral cavernous malformations, and mucocutaneous venous malformations. PMID:23125071

  16. Hypertension in Metabolic Syndrome: Vascular Pathophysiology

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    Yolanda Mendizábal

    2013-01-01

    Full Text Available Metabolic syndrome is a cluster of metabolic and cardiovascular symptoms: insulin resistance (IR, obesity, dyslipemia. Hypertension and vascular disorders are central to this syndrome. After a brief historical review, we discuss the role of sympathetic tone. Subsequently, we examine the link between endothelial dysfunction and IR. NO is involved in the insulin-elicited capillary vasodilatation. The insulin-signaling pathways causing NO release are different to the classical. There is a vasodilatory pathway with activation of NO synthase through Akt, and a vasoconstrictor pathway that involves the release of endothelin-1 via MAPK. IR is associated with an imbalance between both pathways in favour of the vasoconstrictor one. We also consider the link between hypertension and IR: the insulin hypothesis of hypertension. Next we discuss the importance of perivascular adipose tissue and the role of adipokines that possess vasoactive properties. Finally, animal models used in the study of vascular function of metabolic syndrome are reviewed. In particular, the Zucker fatty rat and the spontaneously hypertensive obese rat (SHROB. This one suffers macro- and microvascular malfunction due to a failure in the NO system and an abnormally high release of vasoconstrictor prostaglandins, all this alleviated with glitazones used for metabolic syndrome therapy.

  17. COURSE PECULIARITIES OF NEUROCUTANEOUS MELANOSIS IN CHILDREN

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    Tatyana M. Prygunova

    2016-01-01

    Full Text Available Neurocutaneous melanosis is part of a group of hereditary diseases characterized by large and/or multiple pigmented nevi, melanosis or melanoma of the pia mater, with no evidence of malignant skin lesions and involvement of other organs. The disease was described over 150 years ago, but its pathogenesis has not been studied yet, and treatment methods have not been developed yet. Different disease courses due to the pronounced polymorphism of clinical symptoms complicate the diagnosis, and the low efficacy of the symptomatic treatment worsens the disease prognosis. The article describes the experience of managing children with phakomatoses not similar to each other neither in debut and course nor in response to the therapy and prognosis. Early diagnosis of neurocutaneous melanosis in children allows to carry out timely symptomatic treatment and dynamical monitoring, and to improve the survival of patients.

  18. Neurocutaneous melanosis: radiological-pathological correlation

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    Peretti-Viton, P.; Gorincour, G.; Lambot, K.; Brunel, H.; Raybaud, C. [Department of Neuroradiology, La Timone Hospital, Marseille (France); Feuillet, L.; Cherif, Ali A. [Department of Neurology, La Timone Hospital, Marseille (France); Pellissier, J.F. [Department of Neuropathology, La Timone Hospital, Marseille (France)

    2002-06-01

    We report the case of a young patient with neurocutaneous melanosis (NCM) who presented with temporary aphasia and right hemiparesis followed by progressive coma and death. To our knowledge, this is the first case of this disease examined by CT, MRI, angiography and in which an autopsy was performed to assert the diagnosis with histology. Besides, we discuss differential diagnoses and interest of MRI for early diagnosis. (orig.)

  19. Syndromes associated with vascular tumors and malformations: a pictorial review.

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    Nozaki, Taiki; Nosaka, Shunsuke; Miyazaki, Osamu; Makidono, Akari; Yamamoto, Asako; Niwa, Tetsu; Tsutsumi, Yoshiyuki; Aida, Noriko; Masaki, Hidekazu; Saida, Yukihisa

    2013-01-01

    Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system has been strongly recommended in recent years because of the need for separate therapeutic measures for patients with vascular tumors and malformations. In the ISSVA classification system, vascular tumors, which are neoplastic, are distinguished from vascular malformations, which are caused by vascular structural anomalies and are not neoplastic, on the basis of the presence or absence of neoplastic proliferation of vascular endothelial cells. It is important that radiologists be familiar with the development, diagnosis, and treatment of vascular tumors and malformations, especially the imaging features of low- and high-flow vascular malformations. Some vascular tumors and malformations develop in isolation, whereas others develop within the phenotype of a syndrome. Syndromes that are associated with vascular tumors include PHACE syndrome. Syndromes that are associated with vascular malformations include Sturge-Weber, Klippel-Trénaunay, Proteus, blue rubber bleb nevus, Maffucci, and Gorham-Stout syndromes, all of which demonstrate low flow, and Rendu-Osler-Weber, Cobb, Wyburn-Mason, and Parkes Weber syndromes, all of which demonstrate high flow. Because imaging findings may help identify such syndromes as systemic, it is important that radiologists familiarize themselves with these conditions.

  20. Encephalocraniocutaneous lipomatosis: A rare neurocutaneous syndrome

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    Gokhale N

    2007-01-01

    Full Text Available Encephalocraniocutaneous lipomatosis is a congenital hamartomatous disorder with unique ocular, cutaneous and neurological features. A 13-year-old boy presented with history of mental retardation and delayed developmental milestones. Bulbar conjunctiva of left eye showed hypertrophy with a soft reddish limbal nodule encroaching on the cornea. Dermatological examination showed multiple patches of alopecia, soft papules in the left perioral and periorbital areas, soft masses over the right axilla, trunk and in the lumbosacral region suggestive of lipomas. The CT scan of the brain revealed well-defined, hypodense lesions in both the cerebellar hemispheres suggestive of lipomas. The constellation of these findings led us to a diagnosis of encephalocraniocutaneous lipomatosis.

  1. Dandy-Walker malformation and neurocutaneous melanosis in a three-month-old infant

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    Mahgoub, Shaza Mohamed; Yassin, Rehab Omer; Osman, Atika Mohamed

    2013-01-01

    Dandy-Walker Malformation (DWM) is a rare congenital malformation of the brain. It is characterized by cystic enlargement of the fourth ventricle which is communicating with an enlarged posterior fossa, cerebellar dysgenesis, high tentorial insertion and hydrocephalus. Neurocutaneous Melanosis (NCM) is a congenital neurocutaneous syndrome characterized by large or multiple melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. We report three months old boy who presented with projectile vomiting associated with a noticeable increase in head size. Several congenital nevi were seen all over his body with evident signs of hydrocephalus. The association of DWM and NCM is a rare complex, and to our knowledge, this is the eleventh case to be reported in the literature. In this article, we discuss the proposed pathogenesis, classification and management of the condition. PMID:27493376

  2. Hemothorax in vascular Ehlers-Danlos syndrome.

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    Álvarez, Kevin; Jordi, López; Jose Angel, Hernández

    2017-10-16

    Vascular Ehlers-Danlos syndrome (EDS IV) is a rare genetic disorder characterized by an alteration in the COL3A1 gene which encodes type III collagen. It is the most common type of collagen in vessels of medium size and certain organs such as the intestines and the uterus. The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs. A high level of clinical suspicion is required for diagnosis. It is a complex disease whose management requires a multidisciplinary team to treat the different complications that may occur. We report the case of a 50-year-old man diagnosed with EDS IV detected incidentally after hemothorax secondary to a coughing spell. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  3. Brain vascular changes in Cockayne syndrome.

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    Hayashi, Masaharu; Miwa-Saito, Naho; Tanuma, Naoyuki; Kubota, Masaya

    2012-04-01

    Cockayne syndrome (CS) and xeroderma pigmentosum (XP) are caused by deficient nucleotide excision repair. CS is characterized by cachectic dwarfism, mental disability, microcephaly and progeria features. Neuropathological examination of CS patients reveals dysmyelination and basal ganglia calcification. In addition, arteriosclerosis in the brain and subdural hemorrhage have been reported in a few CS cases. Herein, we performed elastica van Gieson (EVG) staining and immunohistochemistry for collagen type IV, CD34 and aquaporin 4 to evaluate the brain vessels in autopsy cases of CS, XP group A (XP-A) and controls. Small arteries without arteriosclerosis in the subarachnoid space had increased in CS cases but not in either XP-A cases or controls. In addition, string vessels (twisted capillaries) in the cerebral white matter and increased density of CD34-immunoreactive vessels were observed in CS cases. Immunohistochemistry findings for aquaporin 4 indicated no pathological changes in either CS or XP-A cases. Hence, the increased subarachnoid artery space may have caused subdural hemorrhage. Since such vascular changes were not observed in XP-A cases, the increased density of vessels in CS cases was not caused by brain atrophy. Hence, brain vascular changes may be involved in neurological disturbances in CS. © 2011 Japanese Society of Neuropathology.

  4. Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome.

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    Batagini, Nayara Cioffi; Gornik, Heather; Kirksey, Lee

    2015-01-01

    Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant collagen vascular disorder. Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications. Spontaneous splenic rupture is a rare but hazardous complication related to this syndrome. To date, only 2 cases have been reported in the literature. Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension. © The Author(s) 2015.

  5. A newborn with neurocutaneous melanocytosis and Dandy-Walker malformation.

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    De Cock, Jens; Snauwaert, Julie; Van Rompaey, Walter; Morren, Marie-Anne; Demaerel, Philippe

    2014-03-01

    Neurocutaneous melanocytosis is a rare congenital dysplasia of the neuroectodermal melanocyte precursor cells that leads to proliferation of melanin-producing cells in the skin and leptomeninges. We describe a newborn with a giant congenital melanocytic nevus on his back, buttocks, and thighs. His brain magnetic resonance imaging study revealed bilateral T1 hyperintense lesions in the cerebellum and in the amygdala, hydrocephalus, and a Blake's pouch cyst, consistent with neurocutaneous melanocytosis and Dandy-Walker malformation. Neurocutaneous melanocytosis has a wide clinical spectrum that includes hydrocephalus, epilepsy, cranial nerve palsy, increased intracranial pressure, and sensorimotor deficits. Copyright © 2014. Published by Elsevier Inc.

  6. Is Pseudoexfoliation Syndrome a Risk Factor for Cerebro Vascular Disease?

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    Kan, Emrah; Yılmaz, Ahmet; Demirağ, Mehmet Derya; Çalık, Murat

    2017-01-01

    To determine the relationship between cerebro vascular disease and pseudoexfoliation syndrome. This cross-sectional case control study consisted of 50 patients with ischemic-type cerebro vascular disease and 50 control subjects. All subjects were investigated for diabetes mellitus and hypertension status and underwent a detailed ophthalmic examination. A diagnosis of pseudoexfoliation syndrome was made if characteristic greyish particulate matter was found on the anterior lens capsule after pupillary dilatation by slit-lamp examination. All subjects were compared in terms of pseudoexfoliation syndrome, diabetes mellitus, and hypertension. Pearson Chi Square and Student's t test were used for statistical analysis. Logistic regression analyses of the risk factors between groups were also made. The presence of pseudoexfoliation syndrome was significantly higher in patients with cerebro vascular disease when compared to the control subjects (p = 0.02). The frequency of diabetes mellitus was similar between the two groups. Arterial hypertension was significantly more frequent in the patient group when compared to the control subjects (p cerebro vascular disease. In the present study, we found that pseudoexfoliation syndrome frequency was found to be higher in patients with cerebro vascular disease than in control subjects. A slit-lamp examination of the eye could be an important marker that indicates the risk of cerebro vascular disease. We recommend an evaluation of all subjects with pseudoexfoliation syndrome for the presence of cerebro vascular disease. Longitudinal studies with larger populations are needed to confirm this relationship.

  7. Vascular risk factors and adipocyte dysfunction in metabolic syndrome

    NARCIS (Netherlands)

    Hajer, G.R.

    2008-01-01

    The cluster of vascular risk factors closely associated with obesity, consists of fasting and postprandial dyslipidemia, hypertension, and insulin resistance, also known as metabolic syndrome, is associated with an increased cardiovascular morbidity and mortality. In addition, adipose tissue in

  8. Neurocutaneous melanocytosis, hemimegalencephaly and large ovarian cyst in a newborn

    Energy Technology Data Exchange (ETDEWEB)

    Derinkuyu, Betul E.; Boyunaga, Oznur [Gazi University School of Medicine, Department of Radiology, Division of Pediatric Radiology, Besevler, Ankara (Turkey); Unal, Sezin; Ergenekon, Ebru [Gazi University School of Medicine, Department of Pediatrics, Division of Neonatology, Besevler, Ankara (Turkey); Ucar, Murat [Gazi University School of Medicine, Department of Radiology, Besevler, Ankara (Turkey)

    2015-08-15

    We report a case of a newborn girl with neurocutaneous melanocytosis, hemimegalencephaly and a large ovarian cyst. She also had melanocyte deposition in the filum terminale. The ultrasound and the magnetic resonance imaging findings are discussed. (orig.)

  9. Dandy-Walker Malformation Associated with Neurocutaneous Melanosis

    Science.gov (United States)

    Cho, In Yong; Kim, Sung-Hak

    2011-01-01

    Neurocutaneous melanosis associated with Dandy-Walker malformation is a rare dysmorphogenesis that is associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the central nervous system. In this article, we present a 2-month-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. In addition, we reviewed the literature and discussed the pathogenesis based on the preferred hypotheses. PMID:22259699

  10. Systemic Multiple Aneurysms Caused by Vascular Ehlers-Danlos Syndrome.

    Science.gov (United States)

    Gui, Xinyu; Li, Fangda; Wu, Lingeer; Zheng, Yuehong

    2016-07-01

    Systemic multiple aneurysms are rare and usually associated with collagen tissue disease, such as Ehlers-Danlos syndrome (EDS) or Marfan syndrome. In the present case, we describe a 39-year-old male patient with systemic multiple aneurysms and acute intraperitoneal hemorrhage who was clinically diagnosed with vascular EDS. Coil embolization of the distal segment of the common hepatic artery was performed, which resolved the patient's symptoms. With this case presentation, we aim to increase the awareness of vascular EDS among clinicians and emphasize the extreme fragility of the arteries in patients with vascular EDS. © The Author(s) 2016.

  11. [Treatment of the thoracic outlet vascular syndrome].

    Science.gov (United States)

    Davidović, L B; Lotina, S I; Vojnović, B R; Kostić, D M; Colić, M M; Stanić, M I; Djorić, P D

    1998-01-01

    The title "Thoracic Outlet Syndrome" (TOS) was introduced by Peet in 1956 [1]. In 1958 Charles Rob defined TOS as a "set of symptoms that may exist due to compression on the brachial plexus and on subclavian vessels in the region of the thoracic outlet" [2]. Compression due to cervical rib was first described by Galenus and Veaslius in the 2nd century A.D. The first unsuccessful resection of the cervical rib in patients with TOS was performed by Coote in 1861 [4]. In 1905 Murphy first made a successful resection of the cervical rib in patients with TOS and subclavian artery aneurysm [5]. He also removed the normal first rib in patients with TOS using the supraclavicular approach for the first time [6]. In 1920 Law described ligaments and other structures originating in soft tissue associated with TOS [8], while Adson and Coffey in 1927 emphasized the role of the scalene anticus muscle in TOS [3]. Ochsner, Gage and DeBakey in 1935 named it the "scalenus anticus syndrome", and made the first successful resection of the anterior scalene muscle [9]. In 1966 David Ross introduced the transaxillary resection of the first rib to relieve TOS [11]. The aim of the paper is to describe the treatment of patients with vascular TOS. Over a six-year-period (1990-1997) 12 patients with vascular TOS were evaluated at our Centre. Seven (58%) were female and 5 (42%) male patients, average age 33.1 years. Eleven of them had congenital TOS, and one acquired TOS after trauma at neck-shoulder region. Seven patients had arterial and 5 venous TOS. Two patients with arterial TOS had ischaemia of the upper extremity due to embolism of the brachial artery. In one of them axillary artery was completely thrombosed, and in the other postenotic dilatation of the subclavian artery was present. The other 5 patients with arterial TOS demonstrated only hand pain and radial puls during hyperabduction of the arm. One of our patients with venous TOS had also symptoms and signs of hand oedema during

  12. Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Shantala Arunkumar

    2014-07-01

    Full Text Available Sturge-Weber syndrome (SWS also known as encephalotrigeminal angiomatosis. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. An ipsilateral or bilateral facial cutaneous vascular malformation Port Wine Stain (PWS usually affects the upper face. Other clinical manifestations are seizures, glaucoma, hemiparesis, mental retardation and delayed developmental milestones. The main objective of this case report is to unravel such a rarest syndrome with bilateral port-wine stain, which has intraoral manifestation of pyogenic granuloma involving gingiva in an 11 year old boy.

  13. Compartment syndrome and popliteal vascular injury complicating unicompartmental knee arthroplasty

    NARCIS (Netherlands)

    Kort, Nanne Pieter; Van Raay, Jos J. J. A. M.; van Horn, Jim R.

    Popliteal vascular injury and the compartment syndrome of the leg are rare but important complications of knee arthroplasties. Early diagnosis and treatment are of paramount importance in preventing the devastating complications of these conditions. To our knowledge, these complications have not

  14. Arterial complications of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Eagleton, Matthew J

    2016-12-01

    Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the medium-sized arteries. Complications involving the hollow organs, such as spontaneous colonic perforation, are observed as well. Given the fragility of the soft tissue, open and endovascular intervention on patients with vascular EDS is fraught with high complication rates. A PubMed search was performed to identify manuscripts published related to vascular EDS. This search included more than 747 articles. These findings were cross-referenced using key terms, including endovascular, embolization, surgery, genetics, pathophysiology, connective tissue disorders, vascular complications, systematic review, type III collagen, and COL3A1. The references in key articles and review articles were evaluated for additional resources not identified in the PubMed search. Care must be taken to balance the risk of intervention vs the risk of continued observation. Life-threatening hemorrhage, however, mandates intervention. With careful, altered approaches to tissue handling, endovascular approaches may provide a safer option for managing the arterial complications observed in patients with vascular EDS. Additional hope may also be found in the use of pharmacologic agents that reduce the incidence and severity of the arterial complications. Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  15. The metabolic vascular syndrome - guide to an individualized treatment.

    Science.gov (United States)

    Hanefeld, Markolf; Pistrosch, Frank; Bornstein, Stefan R; Birkenfeld, Andreas L

    2016-03-01

    In ancient Greek medicine the concept of a distinct syndrome (going together) was used to label 'a group of signs and symptoms' that occur together and 'characterize a particular abnormality and condition'. The (dys)metabolic syndrome is a common cluster of five pre-morbid metabolic-vascular risk factors or diseases associated with increased cardiovascular morbidity, fatty liver disease and risk of cancer. The risk for major complications such as cardiovascular diseases, NASH and some cancers develops along a continuum of risk factors into clinical diseases. Therefore we still include hyperglycemia, visceral obesity, dyslipidemia and hypertension as diagnostic traits in the definition according to the term 'deadly quartet'. From the beginning elevated blood pressure and hyperglycemia were core traits of the metabolic syndrome associated with endothelial dysfunction and increased risk of cardiovascular disease. Thus metabolic and vascular abnormalities are in extricable linked. Therefore it seems reasonable to extend the term to metabolic-vascular syndrome (MVS) to signal the clinical relevance and related risk of multimorbidity. This has important implications for integrated diagnostics and therapeutic approach. According to the definition of a syndrome the rapid global rise in the prevalence of all traits and comorbidities of the MVS is mainly caused by rapid changes in life-style and sociocultural transition resp. with over- and malnutrition, low physical activity and social stress as a common soil.

  16. Hypomelanosis of Ito: Case report of a rare neurocutaneous ...

    African Journals Online (AJOL)

    2015-03-26

    Mar 26, 2015 ... hamartoma in the frontal lobe. Discussion. Hypomelanosis of Ito (HI) is a sporadic neurocutaneous disorder characterized cutaneously by hypopigmented skin lesions arranged in whorls or streaks along the lines of Blaschko1. The lines of Blaschko are relatively con- sistent and distinct from dermatomal ...

  17. Vascular hand-arm vibration syndrome--magnetic resonance angiography.

    Science.gov (United States)

    Poole, C J M; Cleveland, T J

    2016-01-01

    The diagnosis of vascular hand-arm vibration syndrome (HAVS) requires consistent symptoms, photographic evidence of digital blanching and sufficient exposure to hand-transmitted vibration (HTV; A(8) > 2.5 m/s2). There is no reliable quantitative investigation for distinguishing HAVS from other causes of Raynaud's phenomenon and from normal individuals. Hypothenar and thenar hammer syndromes produce similar symptoms to HAVS but are difficult to diagnose clinically and may be confused with HAVS. Magnetic resonance angiography (MRA) is a safe and minimally invasive method of visualizing blood vessels. Three cases of vascular HAVS are described in which MRA revealed occlusions of the ulnar, radial and superficial palmar arteries. It is proposed that HTV was the cause of these occlusions, rather than blows to the hand unrelated to vibration, the assumed mechanism for the hammer syndromes. All three cases were advised not to expose their hands to HTV despite one of them being at Stockholm vascular stage 2 (early). MRA should be the investigation of choice for stage 2 vascular HAVS or vascular HAVS with unusual features or for a suspected hammer syndrome. The technique is however technically challenging and best done in specialist centres in collaboration with an occupational physician familiar with the examination of HAVS cases. Staging for HAVS should be developed to include anatomical arterial abnormalities as well as symptoms and signs of blanching. Workers with only one artery supplying a hand, or with only one palmar arch, may be at increased risk of progression and therefore should not be exposed to HTV irrespective of their Stockholm stage. © The Author 2015. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  18. Imaging features of vascular compression in abdomen: Fantasy, phenomenon, or true syndrome

    Science.gov (United States)

    Srisajjakul, Sitthipong; Prapaisilp, Patcharin; Bangchokdee, Sirikan

    2017-01-01

    Vascular structures in the abdomen can compress or be compressed by adjacent structures. Classic imaging findings of vascular compressions, including median arcuate ligament syndrome, superior mesenteric artery syndrome, nutcracker syndrome, portal biliopathy, May-Thurner syndrome, and ureteropelvic junction obstruction will be discussed here. It is important to correlate imaging findings and clinical data to identify asymptomatic vascular compression which requires no treatment, intermittent vascular compression with nonspecific or vague clinical manifestation, and the subset of patients with true syndromes who will benefit from treatment. PMID:28744083

  19. Imaging features of vascular compression in abdomen: Fantasy, phenomenon, or true syndrome

    Directory of Open Access Journals (Sweden)

    Sitthipong Srisajjakul

    2017-01-01

    Full Text Available Vascular structures in the abdomen can compress or be compressed by adjacent structures. Classic imaging findings of vascular compressions, including median arcuate ligament syndrome, superior mesenteric artery syndrome, nutcracker syndrome, portal biliopathy, May-Thurner syndrome, and ureteropelvic junction obstruction will be discussed here. It is important to correlate imaging findings and clinical data to identify asymptomatic vascular compression which requires no treatment, intermittent vascular compression with nonspecific or vague clinical manifestation, and the subset of patients with true syndromes who will benefit from treatment.

  20. Neurocutaneous melanosis in association with dandy-walker complex with extensive intracerebral and spinal cord involvement.

    Science.gov (United States)

    Sung, Kyoung-Su; Song, Young-Jin

    2014-07-01

    Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

  1. [Pulmonary reperfusion syndrome after pulmonary stent implants in a patient with vascular tortuosity syndrome].

    Science.gov (United States)

    Berenguer Potenciano, M; Piris Borregas, S; Mendoza Soto, A; Velasco Bayon, J M; Caro Barri, A

    2015-01-01

    Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  2. The versatile reverse flow sural artery neurocutaneous flap: A case series and review of literature

    Directory of Open Access Journals (Sweden)

    Fok Margaret

    2008-04-01

    Full Text Available Abstract Background Reverse flow sural neurocutaneous flap has been utilized more frequently during the past decade to cover vital structures around the foot and ankle area. The potential advantages are the relatively constant blood supply, ease of elevation and preservation of major vascular trunks in the leg. The potential disadvantages remain venous congestion, donor site morbidity and lack of sensation. Methods This descriptive case series was conducted at Queen Mary Hospital, Hong Kong, from 1997 to 2003. Ten patients having undergone reverse flow sural neurocutaneous flap were identified through medical records. There were six females (60% and four males (40%, with an average age of 59.8 years. The defects occurred as a result of trauma in five patients (50%, diabetic ulcers in four (40% and decubitus ulcer in one (10% paraplegic patient. The defect site included non weight bearing heel in four (40%, tendo Achilles in two (20%, distal tibia in two (20%, lateral malleolus in one (10% and medial aspect of the midfoot in one patient (10%. The maximum flap size harvested was 14 × 6 cm. Preoperative doppler evaluation was performed in all patients to identify perforators and modified plaster of paris boot was used in the post operative period. A detailed questionnaire was developed addressing variables of interest. Results There was no flap failure. Venous congestion was encountered in one case. The donor site was relatively unsightly but acceptable to all patients. The loss of sensation in the sural nerve distribution was transient in all patients. Conclusion Reverse sural artery flap remains to be the workhorse flap to resurface the soft tissue defects of the foot and ankle. Anastomosis of the sural nerve to the digital plantar nerve can potentially solve the issue of lack of sensation in the flap especially when used for weight bearing heel.

  3. Vascular Dysfunction: A Key Player in Chronic Cardio-renal Syndrome.

    Science.gov (United States)

    Tomiyama, Hirofumi; Yamashina, Akira

    2015-01-01

    This review summarizes the current methods for the functional assessment of vascular damage (e.g., assessment of endothelial function, measurement of pulse wave velocity, and pressure wave analysis) and describes the association between vascular dysfunction and chronic cardio-renal syndrome. Vascular dysfunction may contribute to the development and progression of heart failure. Additionally, vascular dysfunction, especially increased arterial stiffness and abnormal pressure wave reflection and central hemodynamics, has been reported to accelerate renal function decline. Furthermore, renal dysfunction worsens vascular pathophysiological abnormalities. Therefore, the functional assessment of vascular damage may be useful in the management of cardio-renal syndrome.

  4. Hypothenar Hammer Syndrome: Long-Term Results After Vascular Reconstruction.

    Science.gov (United States)

    Kitzinger, Hugo B; van Schoonhoven, Joerg; Schmitt, Rainer; Hacker, Stefan; Karle, Birgit

    2016-01-01

    Hypothenar hammer syndrome is a rare vascular lesion of the distal ulnar artery in Guyon tunnel caused by acute or repetitive blunt trauma to the hypothenar eminence. Described treatment options vary greatly, from nonoperative management treatments to surgical interventions. The aim of this study was to evaluate the long-term outcomes of patients after surgical reconstruction of the ulnar artery. In this retrospective study, the results of 12 patients treated for hypothenar hammer syndrome were evaluated. Preoperative and postoperative examinations of the hand were recorded. Function impairment was assessed with the "Disabilites of the Arm, Shoulder and Hand" questionnaire. Comparisons were also made based on ulnar artery patency versus occlusion. All patients were evaluated for ulnar artery patency as determined by Allen's test and magnetic resonance angiography. All patients were men with an average age of 42.8 years. In 3 patients, a direct end-to-end anastomosis of the ulnar artery was performed, and 9 patients received a reconstruction with a reverse interpositional vein graft. Nine vascular reconstructions remained patent after a mean follow-up period of 56.9 months. These patients had a complete or at least partial relief of their pain, dysesthesia, and cold intolerance compared with preoperatively. Patients with reoccluded ulnar arteries were statistically significant younger (P = 0.036) than patients with patent ulnar artery. They also had a higher pain level (P = 0.009) and a longer follow-up period (P = 0.036) than those with patent reconstruction. There was a trend for higher functional impairment in patients with reoccluded ulnar artery (P = 0.100). Smoking habits showed no influence on ulnar artery patency. For patients with symptomatic hypothenar hammer syndrome and failed nonoperative treatment, surgical intervention is a good option. After more than 4.5 years after surgery 9 of 12 vascular reconstructions remained patent (75% patency rate

  5. Mitoprotection attenuates myocardial vascular impairment in porcine metabolic syndrome.

    Science.gov (United States)

    Yuan, Fang; Hedayat, Ahmad F; Ferguson, Christopher M; Lerman, Amir; Lerman, Lilach O; Eirin, Alfonso

    2017-12-01

    The metabolic syndrome (MetS) leads to cardiac vascular injury, which may reflect in increased retention of endothelial progenitor cells (EPC). Coronary endothelial cell (EC) mitochondria partly regulate vascular function and structure. We hypothesized that chronic mitoprotection would preserve EC mitochondria and attenuate coronary vascular injury and dysfunction in swine MetS. Pigs were studied after 16 weeks of diet-induced MetS, MetS treated for the last 4 weeks with the mitochondria-targeted peptide elamipretide (ELAM, 0.1mg/kg SC q.d), and lean controls (n=6 each). Cardiac remodeling and function were assessed in vivo by multi-detector-CT, and coronary artery and sinus blood samples collected. EC mitochondrial density, apoptosis, oxidative stress, endothelial nitric oxide (eNOS) immunoreactivity, myocardial microvascular density (3D micro-CT), and coronary endothelial function (organ bath) were assessed ex-vivo. The number and arteriovenous gradient of CD34+/KDR+ EPC was calculated by FACS (a negative net gradient indicating EPC retention). MetS and MetS+ELAM pigs developed similar MetS (obesity, hyperlipidemia, insulin resistance, and hypertension). EC mitochondrial density decreased in MetS compared to lean, but normalized in MetS+ELAM. ELAM also attenuated EC oxidative stress and apoptosis, and improved subendocardial microvascular density. ELAM-induced vasculoprotection was reflected in decreased coronary retention of EPC. ELAM also partly improved eNOS immunoreactivity, coronary endothelial function, and vessel maturity, whereas myocardial perfusion was unaffected. Chronic mitoprotection improved coronary EC mitochondrial density and decreased vascular remodeling and dysfunction. Yet, additional mitochondria-independent mechanisms likely contribute to MetS-induced cardiac vascular injury.

  6. Frailty and Geriatric Syndromes in Vascular Surgical Ward Patients.

    Science.gov (United States)

    McRae, Prudence J; Walker, Philip J; Peel, Nancye M; Hobson, Denise; Parsonson, Fiona; Donovan, Peter; Reade, Michael C; Marquart, Louise; Mudge, Alison M

    2016-08-01

    Preoperative frailty is an important predictor of poor outcomes but the relationship between frailty and geriatric syndromes is less clear. The aims of this study were to describe the prevalence of frailty and incidence of geriatric syndromes in a cohort of older vascular surgical ward patients, and investigate the association of frailty and other key risk factors with the occurrence of one or more geriatric syndromes (delirium, functional decline, falls, and/or pressure ulcers) and two hospital outcomes (acute length of stay and discharge destination). This prospective cohort study was conducted in a vascular surgical ward in a tertiary teaching hospital in Brisbane, Australia. Consecutive patients aged ≥65 years, admitted for ≥72 hr, were eligible for inclusion. Frailty was defined as one or more of functional dependency, cognitive impairment, or nutritional impairment at admission. Delirium was identified using the Confusion Assessment Method and a validated chart extraction tool. Functional decline from admission to discharge was identified from daily nursing documentation of activities of daily living. Falls were identified according to documentation in the medical record cross-checked with the incident reporting system. Pressure ulcers, acute length of stay, and discharge destination were identified by documentation in the medical record. Risk factors associated with geriatric syndromes, acute length of stay, and discharge destination were assessed using multivariable logistic regression models. Of 110 participants, 43 (39%) patients were frail and geriatric syndromes occurred in 40 (36%). Functional decline occurred in 25% of participants, followed by delirium (20%), pressure ulcers (12%), and falls (4%). In multivariable logistic analysis, frailty [odds ratio (OR) 6.7, 95% confidence interval (CI) 2.0-22.1, P = 0.002], nonelective admission (OR 7.2, 95% CI 2.2-25.3, P = 0.002), higher physiological severity (OR 5.5, 95% CI 1.1-26.8, P = 0

  7. PHACE syndrome in antenatally diagnosed posterior fossa anomaly

    Directory of Open Access Journals (Sweden)

    Seema Pavaman Sindgikar

    2014-01-01

    Full Text Available PHACE is a neurocutaneous syndrome, an acronym to describe patients with facial segmental hemangiomas and other malformations. We describe a newborn antenatally diagnosed to have posterior fossa anomaly and subsequently as PHACE syndrome.

  8. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Murray, Mitzi L; Pepin, Melanie; Peterson, Suzanne; Byers, Peter H

    2014-12-01

    The purpose of this study was to characterize the nature and magnitude of pregnancy risks in women with vascular Ehlers-Danlos syndrome. Pregnancy-related death rate was determined by a review of pedigrees of families with vascular Ehlers-Danlos syndrome. Maternal morbidity was characterized through semistructured interviews with women with vascular Ehlers-Danlos syndrome or their next of kin. Pregnancy-related deaths occurred in 30 of 565 deliveries (5.3%). There was no difference in Kaplan-Meier survival curves between parous versus nulliparous women with vascular Ehlers-Danlos syndrome. Interviews with 39 women indicated that 46% of deliveries were uncomplicated. The most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%). Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). There were 5 maternal deaths in 76 deliveries (6.5%). The risk of pregnancy-related complications is increased in women with vascular Ehlers-Danlos syndrome compared with the general population; however, survival data indicate that pregnancy does not appear to affect overall mortality compared with nulliparous women with vascular Ehlers-Danlos syndrome. The data were insufficient to determine whether mode or timing of delivery influenced risk of complications. Women with vascular Ehlers-Danlos syndrome should be engaged in a shared decision-making process when contemplating pregnancy and pregnancy management.

  9. A rare neurocutaneous syndrome and a rare association | Ali ...

    African Journals Online (AJOL)

    Among the many causes of ataxia, Ataxia -telangictasia is an autosomal recessive1, multi system disease affecting the skin, nervous system & immune system. It's prevalence has been estimated at 1 to 2 per 100,000 It is a neurodegenerative disorder2 in which there is progressive cerebellar ataxia, occulocutaneous ...

  10. Vascular Ehlers-Danlos Syndrome Without the Characteristic Facial Features

    Science.gov (United States)

    Inokuchi, Ryota; Kurata, Hideaki; Endo, Kiyoshi; Kitsuta, Yoichi; Nakajima, Susumu; Hatamochi, Atsushi; Yahagi, Naoki

    2014-01-01

    Abstract As a type of Ehlers-Danlos syndrome (EDS), vascular EDs (vEDS) is typified by a number of characteristic facial features (eg, large eyes, small chin, sunken cheeks, thin nose and lips, lobeless ears). However, vEDs does not typically display hypermobility of the large joints and skin hyperextensibility, which are features typical of the more common forms of EDS. Thus, colonic perforation or aneurysm rupture may be the first presentation of the disease. Because both complications are associated with a reduced life expectancy for individuals with this condition, an awareness of the clinical features of vEDS is important. Here, we describe the treatment of vEDS lacking the characteristic facial attributes in a 24-year-old healthy man who presented to the emergency room with abdominal pain. Enhanced computed tomography revealed diverticula and perforation in the sigmoid colon. The lesion of the sigmoid colon perforation was removed, and Hartmann procedure was performed. During the surgery, the control of bleeding was required because of vascular fragility. Subsequent molecular and genetic analysis was performed based on the suspected diagnosis of vEDS. These analyses revealed reduced type III collagen synthesis in cultured skin fibroblasts and identified a previously undocumented mutation in the gene for a1 type III collagen, confirming the diagnosis of vEDS. After eliciting a detailed medical profile, we learned his mother had a history of extensive bruising since childhood and idiopathic hematothorax. Both were prescribed oral celiprolol. One year after admission, the patient was free of recurrent perforation. This case illustrates an awareness of the clinical characteristics of vEDS and the family history is important because of the high mortality from this condition even in young people. Importantly, genetic assays could help in determining the surgical procedure and offer benefits to relatives since this condition is inherited in an autosomal dominant

  11. Segmental neurofibromatosis type 1 (NF1) associated with Cobb syndrome: case report.

    Science.gov (United States)

    Pascual-Castroviejo, I; Pascual-Pascual, S-I; Viaño, J

    2008-12-01

    We present a 3-month-old girl who showed segmental NF1 and Cobb syndrome. She has a cutaneous vascular malformation located on the middle T (4)-T (6) region superimposed on a giant cutaneous café-au-lait spot. Magnetic resonance arteriography (MRA) revealed bilateral renal artery stenosis, extensive hypertrophy of the spinal epidural venous plexus, coarctation and tubular hypoplasia of the aortic arch and proximal portion of descending aorta. To the best of our knowledge the association of both neurocutaneous disorders has not being previously described.

  12. Periodontal Management of Sturge-Weber Syndrome

    Directory of Open Access Journals (Sweden)

    Butchibabu Kalakonda

    2013-01-01

    Full Text Available Sturge-Weber syndrome (SWS is a sporadic disorder and is frequent among the neurocutaneous syndromes specifically with vascular predominance. This syndrome consists of constellation of clinical features like facial nevus, seizures, hemiparesis, intracranial calcifications, and mental retardation. It is characterized by focal port-wine stain, ocular abnormalities (glaucoma, and choroidal hemangioma and leptomeningeal angioma most often involving occipital and parietal lobes. The present paper reports three cases of SWS with oral manifestations and periodontal management, which included thorough scaling and root planing followed by gingivectomy with scalpel and laser in cases 1 and 3 consecutively to treat the gingival enlargement. However, the treatment in case 2 was deferred as the patient was not a candidate for periodontal surgery.

  13. An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex: A case report.

    Science.gov (United States)

    Rai, Santosh; Kalakoti, Piyush; Syed, Mm Aarif; Thacker, Purujit J; Jain, Rishi; Kalra, Gaurav

    2011-07-01

    Congenital melanocytic nevi are among the several known risk factors for the development of melanoma. Neurocutaneous melanosis is a rare, congenital, non-hereditary disorder characterized by the presence of multiple and/or giant congenital melanocytic nevi. It is a rare condition, with fewer than 200 cases reported in the literature. Its association with tuberous sclerosis complex, a form of the neurocutaneous syndrome, is an unusual finding which, to the best of our knowledge, has not been documented in the English literature so far. Herein we present the first case documenting such an association in a 16-year-old post-pubertal Indian girl. In this report, we describe the case of a 16-year-old Indian girl who presented to our hospital with swelling on the scalp which had progressed from the hairline to just above the left brow, causing mechanical ptosis. She was born with a black-pigmented triangular patch covered with hair over the scalp which had increased in size over a period of eight years after birth. An X-ray of her skull and ultrasonography revealed soft tissue swelling in the left temporofrontoparietal region. Magnetic resonance imaging of her brain showed the presence of 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp, a few small tubers with cortical dysplasia in the left frontoparietal region with asymmetric dilatation, and the presence of calcified subependymal nodules within the left lateral ventricle. These findings were suggestive of tuberous sclerosis. A histopathological examination of the swelling was suggestive of congenital melanocytic nevi. The patient underwent surgery. Excision of the tumor with primary skin grafting was done, with the graft being taken from the medial aspect of the right thigh. This case warrants further research to provide concrete evidence of an association of neurocutaneous melanoma with tuberous sclerosis complex. Research should be conducted to prove whether this is an unusual association or a new

  14. An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex: A case report

    Directory of Open Access Journals (Sweden)

    Thacker Purujit J

    2011-07-01

    Full Text Available Abstract Introduction Congenital melanocytic nevi are among the several known risk factors for the development of melanoma. Neurocutaneous melanosis is a rare, congenital, non-hereditary disorder characterized by the presence of multiple and/or giant congenital melanocytic nevi. It is a rare condition, with fewer than 200 cases reported in the literature. Its association with tuberous sclerosis complex, a form of the neurocutaneous syndrome, is an unusual finding which, to the best of our knowledge, has not been documented in the English literature so far. Herein we present the first case documenting such an association in a 16-year-old post-pubertal Indian girl. Case presentation In this report, we describe the case of a 16-year-old Indian girl who presented to our hospital with swelling on the scalp which had progressed from the hairline to just above the left brow, causing mechanical ptosis. She was born with a black-pigmented triangular patch covered with hair over the scalp which had increased in size over a period of eight years after birth. An X-ray of her skull and ultrasonography revealed soft tissue swelling in the left temporofrontoparietal region. Magnetic resonance imaging of her brain showed the presence of 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp, a few small tubers with cortical dysplasia in the left frontoparietal region with asymmetric dilatation, and the presence of calcified subependymal nodules within the left lateral ventricle. These findings were suggestive of tuberous sclerosis. A histopathological examination of the swelling was suggestive of congenital melanocytic nevi. The patient underwent surgery. Excision of the tumor with primary skin grafting was done, with the graft being taken from the medial aspect of the right thigh. Conclusion This case warrants further research to provide concrete evidence of an association of neurocutaneous melanoma with tuberous sclerosis complex. Research should be

  15. Between a rock and a hard place: clinical and imaging features of vascular compression syndromes.

    Science.gov (United States)

    Eliahou, Ruth; Sosna, Jacob; Bloom, Allan I

    2012-01-01

    Vascular compression syndromes are caused by the entrapment of vessels between rigid or semirigid surfaces in a confined anatomic space. Chronic entrapment may lead to arterial ischemia and embolism, venous stasis and thrombosis, and hematuria. These syndromes are usually seen in otherwise healthy young patients, among whom underdiagnosis is common. Most occurrences of vascular compression are associated with an underlying anatomic abnormality. In a small percentage of cases, other contributing factors, including repetitive microtrauma, may cause pathologic changes leading to the onset of pain and other symptoms of vascular and neural compression. Hence, the diagnosis must be based on both clinical and radiologic findings. Because some cases of vascular entrapment become symptomatic only when specific physical maneuvers are performed, dynamic diagnostic imaging methods are especially useful. Digital subtraction angiography has been the mainstay of imaging-based diagnosis for most vascular compression syndromes, but other methods (eg, color Doppler ultrasonography, computed tomographic angiography, and magnetic resonance angiography) are used with increasing frequency for initial diagnostic evaluation. Because vascular compression syndromes are caused by the external compression of vessels, endoluminal treatment alone is rarely adequate and surgical decompression is likely to be required for optimal and durable clinical benefit. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.321115011/-/DC1.

  16. Sturge -Weber Syndrome - Three Classic variants

    Directory of Open Access Journals (Sweden)

    R S Sathawane

    2006-01-01

    Full Text Available Sturge-Weber syndrome (SWS, also known as encephalotrigeminal angiomatosis, a sporadic, non-familial, congenital disorder consists of congenital hamartomatous malformations that may affect the eye, skin and central nervous system at different times. Sturge-Weber syndrome is classified as 1 Complete trisymptomatic: - when all three organ systems i.e. eye, skin and CNS are involved 2 Incomplete bisymptomatic:- when the involvement is either oculocutaneous or neurocutaneous, and 3 Incomplete monosymptomatic: when there is only neural or cutaneous involvement. Failure of proper vascular development is believed to be the most likely cause of this condition. The malformed blood vessels or hemangiomas may lead to port-wine stain, epilepsy and glaucoma depending on its location. Three classic variants with typical findings are discussed.

  17. Metabolic Vascular Syndrome: New Insights into a Multidimensional Network of Risk Factors and Diseases.

    Science.gov (United States)

    Scholz, Gerhard H; Hanefeld, Markolf

    2016-10-01

    Since 1981, we have used the term metabolic syndrome to describe an association of a dysregulation in lipid metabolism (high triglycerides, low high-density lipoprotein cholesterol, disturbed glucose homeostasis (enhanced fasting and/or prandial glucose), gout, and hypertension), with android obesity being based on a common soil (overnutrition, reduced physical activity, sociocultural factors, and genetic predisposition). We hypothesized that main traits of the syndrome occur early and are tightly connected with hyperinsulinemia/insulin resistance, procoagulation, and cardiovascular diseases. To establish a close link between the traits of the metabolic vascular syndrome, we focused our literature search on recent original work and comprehensive reviews dealing with the topics metabolic syndrome, visceral obesity, fatty liver, fat tissue inflammation, insulin resistance, atherogenic dyslipidemia, arterial hypertension, and type 2 diabetes mellitus. Recent research supports the concept that the metabolic vascular syndrome is a multidimensional and interactive network of risk factors and diseases based on individual genetic susceptibility and epigenetic changes where metabolic dysregulation/metabolic inflexibility in different organs and vascular dysfunction are early interconnected. The metabolic vascular syndrome is not only a risk factor constellation but rather a life-long abnormality of a closely connected interactive cluster of developing diseases which escalate each other and should continuously attract the attention of every clinician.

  18. Metabolic Vascular Syndrome: New Insights into a Multidimensional Network of Risk Factors and Diseases

    Science.gov (United States)

    Scholz, Gerhard H.; Hanefeld, Markolf

    2016-01-01

    Background Since 1981, we have used the term metabolic syndrome to describe an association of a dysregulation in lipid metabolism (high triglycerides, low high-density lipoprotein cholesterol, disturbed glucose homeostasis (enhanced fasting and/or prandial glucose), gout, and hypertension), with android obesity being based on a common soil (overnutrition, reduced physical activity, sociocultural factors, and genetic predisposition). We hypothesized that main traits of the syndrome occur early and are tightly connected with hyperinsulinemia/insulin resistance, procoagulation, and cardiovascular diseases. Methods To establish a close link between the traits of the metabolic vascular syndrome, we focused our literature search on recent original work and comprehensive reviews dealing with the topics metabolic syndrome, visceral obesity, fatty liver, fat tissue inflammation, insulin resistance, atherogenic dyslipidemia, arterial hypertension, and type 2 diabetes mellitus. Results Recent research supports the concept that the metabolic vascular syndrome is a multidimensional and interactive network of risk factors and diseases based on individual genetic susceptibility and epigenetic changes where metabolic dysregulation/metabolic inflexibility in different organs and vascular dysfunction are early interconnected. Conclusion The metabolic vascular syndrome is not only a risk factor constellation but rather a life-long abnormality of a closely connected interactive cluster of developing diseases which escalate each other and should continuously attract the attention of every clinician. PMID:27921043

  19. Retinal vascular tortuosity in DiGeorge syndrome complicated by solar retinopathy.

    Science.gov (United States)

    De Niro, Jennifer E; Randhawa, Sandeep; McDonald, H Richard

    2013-01-01

    To report a case of vascular tortuosity associated with DiGeorge syndrome that was complicated by solar retinopathy. Case report and literature review. A 56-year-old woman with DiGeorge syndrome with secondary schizophrenia and developmental delay presented with decreased vision that was worse in her left eye. Ocular examination revealed bilateral retinal vascular tortuosity involving both the arteries and veins. Both eyes had an abnormal foveal light reflex with a central yellowish hue, which was more pronounced in the left eye. Optical coherence tomography showed disruption of the photoreceptor inner segment-outer segment junction and retinal pigment epithelium centrally, which was also more prominent in the left eye. DiGeorge syndrome is associated with retinal vascular tortuosity in a large percentage of patients. The patient's decreased vision is likely caused by solar retinopathy (prolonged sun gazing as a result of the secondary schizophrenia and developmental delay).

  20. [Application of the tension skin flap with different shapes in the pedicle of the reverse neurocutaneous island flap].

    Science.gov (United States)

    Li, Meng; Lan, Xu; Zheng, Ping; Liu, Xing-Yan; Gao, Qiu-Ming; Song, Ming-Jia

    2013-08-01

    To investigate the effects of the tension skin flap with different shapes on the transplantation of the reverse neurocutaneous island flap. From January 2006 to January 2012,there were 21 patients in the study (including 15 males and 6 females), and aged from 14 to 58 years old (35 years old on average). Tension skin flaps with different shapes (triangle ,round and ellipse) were used to improve the blood supply of the reverse neurocutaneous island flap. The tension skin flaps in the pedicle were designed triangularly (10 patients), spherically (8 patients) or elliptically (3 patients). There were 5 patients with defects in the hand (the size from 5.0 cm x 2.0 cm to 8.0 cm x 5.0 cm), and 16 patients with defects in the foot and inferior segment of leg, or around the ankle (the size from 6.0 cm x 4.0 cm to 13.0 cm x 7.0 cm). And all the patients were with the tendon and bone exposed. All the flaps were reversal transplanted, including 5 dorsal neurocutaneous flaps of foot, 4 superficial peroneal neurocutaneous flaps, 4 saphenous neurocutaneous flaps, 3 sural neurocutaneous flaps, 2 superficial radial neurocutaneous flaps, 3 lateral neurocutaneous flaps of forearm. And the survival rate, appearance and sensory recovery of the flaps were analyzed. The distant part of the reversed sural neurocutaneous island flap in 1 case necrosized and healed after dressing change. The other flaps survived entirely, and the donor site all healed primarily. The follow-up time was from 3 months to 2 years (averaged 7 months), and all the flaps had recovered pain and warm sensation with perfect appearance. The tension skin flap in the pedicle can enhance the blood supply and promote survival rate of the reverse neurocutaneous island flap, and can also improve its appearance.

  1. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy

    Directory of Open Access Journals (Sweden)

    Elaheh Malakan Rad

    2014-01-01

    Full Text Available Goldenhar syndrome (GS or oculo-auriculo-vertebral dysplasia (OAVD, involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA, severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.

  2. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy.

    Science.gov (United States)

    Rad, Elaheh Malakan

    2014-09-01

    Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.

  3. Diffusion tensor imaging differentiates vascular parkinsonism from parkinsonian syndromes of degenerative origin in elderly subjects

    Energy Technology Data Exchange (ETDEWEB)

    Deverdun, Jérémy [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Laboratoire Charles Coulomb, CNRS UMR 5221 - Université Montpellier II, Montpellier (France); I2FH, Institut d’Imagerie Fonctionnelle Humaine, Hôpital Gui de Chauliac, CHRU de, Montpellier (France); Menjot de Champfleur, Sophie [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Clinique du Parc, Castelnau-le-Lez (France); Cabello-Aguilar, Simon [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); I2FH, Institut d’Imagerie Fonctionnelle Humaine, Hôpital Gui de Chauliac, CHRU de, Montpellier (France); Maury, Florence [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Molino, François [Laboratoire Charles Coulomb, CNRS UMR 5221 - Université Montpellier II, Montpellier (France); Institut de Génomique Fonctionnelle, UMR 5203 - INSERM U661 - Université Montpellier II - Université, Montpellier I (France); Charif, Mahmoud [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Leboucq, Nicolas [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Ayrignac, Xavier; Labauge, Pierre [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); and others

    2014-11-15

    Background and Purpose: The etiologic diagnosis of parkinsonian syndromes is of particular importance when considering syndromes of vascular or degenerative origin. The purpose of this study is to find differences in the white-matter architecture between those two groups in elderly patients. Materials and Methods: Thirty-five patients were prospectively included (multiple-system atrophy, n = 5; Parkinson's disease, n = 15; progressive supranuclear palsy, n = 9; vascular parkinsonism, n = 6), with a mean age of 76 years. Patients with multiple-system atrophy, progressive supranuclear palsy and Parkinson's disease were grouped as having parkinsonian syndromes of degenerative origin. Brain MRIs included diffusion tensor imaging. Fractional anisotropy and mean-diffusivity maps were spatially normalized, and group analyses between parkinsonian syndromes of degenerative origin and vascular parkinsonism were performed using a voxel-based approach. Results: Statistical parametric-mapping analysis of diffusion tensor imaging data showed decreased fractional anisotropy value in internal capsules bilaterally in patients with vascular parkinsonism compared to parkinsonian syndromes of degenerative origin (p = 0.001) and showed a lower mean diffusivity in the white matter of the left superior parietal lobule (p = 0.01). Fractional anisotropy values were found decreased in the middle cerebellar peduncles in multiple-system atrophy compared to Parkinson's disease and progressive supranuclear palsy. The mean diffusivity was increased in those regions for these subgroups. Conclusion: Clinically defined vascular parkinsonism was associated with decreased fractional anisotropy in the deep white matter (internal capsules) compared to parkinsonian syndromes of degenerative origin. These findings are consistent with previously published neuropathological data.

  4. Metabolic syndrome, dysglycaemia and vascular disease: making sense of the evidence

    OpenAIRE

    Preiss, David; Sattar, Naveed

    2007-01-01

    “ ‘Diagnosis' of metabolic syndrome by any current criteria is not needed and adds little to clinical practice. Attention and resources should be focused on the major modifiable risk factors and more should be done to engender sustainable lifestyle changes in all with vascular disease”

  5. Cardio-cephalic neural crest syndrome: A novel hypothesis of vascular neurocristopathy.

    Science.gov (United States)

    Komiyama, M

    2017-12-01

    A novel hypothesis proposes that "cardio-cephalic neural crest (NC) syndrome," i.e. cephalic NC including cardiac NC, contributes to the concurrent occurrence of vascular diseases in the cardio- and cerebrovascular regions. NC is a transient structure present in early embryogenesis. Cephalic NC provides mesenchymal cells to the vascular media in these regions. Concurrent cardio- and cerebrovascular lesions have been reported in PHACE syndrome, ACTA2 mutation syndrome, and less frequently in the spontaneous occlusion of the circle of Willis (so-called moyamoya disease). Cardiovascular lesions in these syndromes include coarctation of the aorta, persistent truncus arteriosus, patent ductus arteriosus, and coronary artery disease, and cerebrovascular lesions include agenesis and stenosis/occlusion of the internal carotid arteries, and moyamoya phenomenon. These concurrent vascular lesions both in the cardio- and cerebrovascular regions might be related to cephalic NC. This hypothesis, although not proven, may facilitate a better understanding of the above-mentioned NC-related vascular pathologies and lead to appropriate diagnostic and therapeutic approaches for clinicians and chart future direction for researchers.

  6. Popliteal vascular entrapment syndrome caused by a rare anomalous slip of the lateral head of the gastrocnemius muscle

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Patrick T.; Moyer, Adrian C.; Huettl, Eric A. [Mayo Clinic Scottsdale, Department of Radiology, Scottsdale (United States); Fowl, Richard J.; Stone, William M. [Mayo Clinic Scottsdale, Department of Vascular Surgery, Scottsdale (United States)

    2005-06-01

    Popliteal vascular entrapment syndrome can result in calf claudication, aneurysm formation, distal arterial emboli, or popliteal vessel thrombosis. The most commonly reported causes of this syndrome have been anomalies of the medial head of the gastrocnemius muscle as it relates to the course of the popliteal artery. We report two cases of rare anomalous slips of the lateral head of the gastrocnemius muscle causing popliteal vascular entrapment syndrome. (orig.)

  7. Parenchymal Neurocutaneous Melanosis in Association with Intraventricular Dermoid and Dandy-Walker Variant: A Case Report

    Science.gov (United States)

    Won, Yoo Dong; Kim, Ki Tae; Chang, Eun Deok; Huh, Pil Woo

    2006-01-01

    Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation. PMID:16799276

  8. Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy-walker variant: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Joo; Won, Yoo Dong; Kim, Ki Tae; Chang, Eun Deok; Huh, Pil Woo [The Catholic University of Korea, College of Medicine, Uijongbu (Korea, Republic of)

    2006-06-15

    Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

  9. Retinal Vascular Tortuosity in a Patient with Weill-Marchesani Syndrome

    Directory of Open Access Journals (Sweden)

    Kevin Gallagher

    2011-01-01

    Full Text Available Weill-Marchesani syndrome (WMS is a rare connective tissue disorder with characteristic phenotypic skeletal and ocular manifestations. A 28-year-old myopic female presented with an 8-month history of bilateral blurred vision. On examination, she was noted to be of short stature with brachydactyly. On ocular examination, she was found to be spherophakic with bilateral inferiorly subluxated lenses. Serum and urine homocysteine were normal and a syphilis screen was negative. A diagnosis of Weill-Marchesani syndrome was made. Fundoscopy revealed bilateral tortuous retinal vessels. We report the first illustrated case of retinal vascular tortuosity as an ocular manifestation of Weill-Marchesani syndrome.

  10. Sturge Weber Syndrome: review of literature with case illustration

    Directory of Open Access Journals (Sweden)

    Satyarthee Guru Dutta

    2017-03-01

    Full Text Available Sturge-Weber syndrome (SWS also called as encephalotrigeminal angiomatosis, is a sporadically occurring rare neuro-cutaneous syndrome, characterized by vascular malformation with capillary venous angiomas involving face, choroidal layer of eye globe and leptomeninges responsible for ophthamological as well as neurological signs and symptoms. Authors report an interesting case, a six year old girl, who presented with seizures, facial port wine stain and normal psychomotor development. CT scan showed left cerebral hemiatrophy, left frontal and parieto occipital calcification with cortical calcification in left high frontal convexity. Cranial MRI scan also confirmed finding of left cerebral hemiatrophy and also revealed presence of gyriform cortical calcification, prominent flow voids seen in left basal ganglia. Her seizure is well controlled with antiepileptic medication. The pertinent literature is reviewed and management of such cases is discussed briefly.

  11. Melanose neurocutânea. Relato de caso com melanoma maligno do sistema nervoso central Neurocutaneous melanosis. Case report of a malignant melanoma of the central nervous system

    Directory of Open Access Journals (Sweden)

    J.M. Juang

    1998-03-01

    Full Text Available A melanose neurocutânea é uma síndrome rara caracterizada por nevos melanocíticos congênitos gigantes e excessiva melanose da leptomeninge. A síndrome parece representar um erro na morfogênese do neuroectoderma embrionário. Os autores apresentam o caso e necropsia de um paciente masculino de 19 anos que desenvolveu melanoma maligno do sistema nervoso central.The neurocutaneous melanosis is a rare syndrome in which the congenital melanocytic nevi and excessive melanosis are the main features. The syndrome seems to be a morphogenesis error of the embryonic neuroectoderm. A clinical case with necroscopy in a 19 year-old man who had developed malign melanoma in his central nervous system is reported.

  12. The dysexecutive syndrome associated with ischaemic vascular disease and related subcortical neuropathology: a Boston process approach.

    Science.gov (United States)

    Lamar, Melissa; Price, Cate C; Giovannetti, Tania; Swenson, Rod; Libon, David J

    2010-01-01

    The introduction of diagnostic criteria for vascular dementia has helped to re-define the impact of various subcortical neuropathologies on aging; however, state-of-the-art neuroimaging techniques and autopsy studies suggest that not all structural brain alterations associated with vascular dementia are exclusive to this neurodegenerative process alone. Thus, a detailed analysis of the cognitive phenotype associated with ischaemic vascular disease is key to our understanding of subcortical neuropathology and its associated behaviors. Over the past twenty years, we have operationally defined this cognitive phenotype using the Boston Process Approach to neuropsychological assessment. This has led to both an empirical, as well as a theoretical understanding of three core constructs related to the dysexecutive syndrome associated with ischaemic vascular disease affecting periventricular and deep white matter as well as subcortical structures connecting these regions with the prefrontal cortex. Thus, difficulties with mental set, cognitive control and mental manipulation negatively impact executive functioning. This review will outline the subtle markers underlying this prefrontal dysfunction, i.e., the dysexecutive phenotype, associated with ischaemic vascular disease and relate it to fundamental impairments of gating subserved by basal ganglia-thalamic pathways within and across various dementia syndromes.

  13. The Dysexecutive Syndrome Associated with Ischaemic Vascular Disease and Related Subcortical Neuropathology: A Boston Process Approach

    Directory of Open Access Journals (Sweden)

    Melissa Lamar

    2010-01-01

    Full Text Available The introduction of diagnostic criteria for vascular dementia has helped to re-define the impact of various subcortical neuropathologies on aging; however, state-of-the-art neuroimaging techniques and autopsy studies suggest that not all structural brain alterations associated with vascular dementia are exclusive to this neurodegenerative process alone. Thus, a detailed analysis of the cognitive phenotype associated with ischaemic vascular disease is key to our understanding of subcortical neuropathology and its associated behaviors. Over the past twenty years, we have operationally defined this cognitive phenotype using the Boston Process Approach to neuropsychological assessment. This has led to both an empirical, as well as a theoretical understanding of three core constructs related to the dysexecutive syndrome associated with ischaemic vascular disease affecting periventricular and deep white matter as well as subcortical structures connecting these regions with the prefrontal cortex. Thus, difficulties with mental set, cognitive control and mental manipulation negatively impact executive functioning. This review will outline the subtle markers underlying this prefrontal dysfunction, i.e., the dysexecutive phenotype, associated with ischaemic vascular disease and relate it to fundamental impairments of gating subserved by basal ganglia-thalamic pathways within and across various dementia syndromes.

  14. Subclavian artery aneurysm in a patient with vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Yasuda, Shota; Imoto, Kiyotaka; Uchida, Keiji; Uranaka, Yasuko; Kurosawa, Kenji; Masuda, Munetaka

    2016-02-01

    We describe our experience of surgical treatment in a 28-year-old woman with vascular Ehlers-Danlos syndrome. A right subclavian artery aneurysm was detected. The right vertebral artery arose from the aneurysm. Digital subtraction angiography showed interruption of the left vertebral artery. The aneurysm was excised and the right vertebral artery was anastomosed end-to-side to the right common carotid artery under deep hypothermia and circulatory arrest. The patient remained very well 4 years after surgery, with no late vascular complication. © The Author(s) 2014.

  15. Spinal Anaesthesia for Cesarean Section in a Patient with Vascular Type Ehlers-Danlos Syndrome

    Directory of Open Access Journals (Sweden)

    Jeffrey M. Carness

    2018-01-01

    Full Text Available We report the administration of spinal anaesthesia for cesarean delivery in a parturient with vascular Ehlers-Danlos syndrome. Parturients who genetically inherit this disorder are at risk for significant morbidity and mortality. Risks during pregnancy include premature labor, uterine prolapse, and uterine rupture. Additionally, such laboring parturients are at increased risk of hemodynamic volatility, vascular stress, and severe postpartum hemorrhage. Instrumented delivery and cesarean delivery bring additional risks. Nonpregnancy-related complications include excessive bleeding, intestinal rupture, cardiac valvular dysfunction, and arterial dissection. Despite the complexity of this condition, literature focusing on specific intraoperative anaesthetic management is sparse.

  16. Association of Metabolic Syndrome with the Cardioankle Vascular Index in Asymptomatic Korean Population

    Directory of Open Access Journals (Sweden)

    Su-Hyun Nam

    2015-01-01

    Full Text Available Aim. Metabolic syndrome is characterized by a cluster of atherosclerotic cardiovascular risk factors. The cardioankle vascular index (CAVI reflects arterial stiffness and may be used as an indicator of atherosclerotic cardiovascular disease. In this study, we investigated the association of CAVI with metabolic syndrome. Methods. A total of 1,144 adults were included in this study. We measured CAVIs and examined blood samples to identify metabolic syndrome according to WHO Asia Pacific criteria and NCEP-ATPIII criteria. AST, ALT, r-GTP, BUN, creatinine, high sensitivity C-reactive protein, and uric acid were also measured. Results. CAVI values were significantly higher in subjects with metabolic syndrome than those without metabolic syndrome and increased according to the number of metabolic syndrome components present. Subjects with high fasting blood sugar levels or high blood pressure showed high CAVI values. Multiple regression analysis showed that age, sex, diastolic blood pressure, and uric acid were independent predictors of CAVI. Conclusion. Subjects with metabolic syndrome had high CAVIs, which indicated arterial stiffness, and were closely associated with an increase in the number of metabolic risk factors. The individual risk factors for metabolic syndrome have the synergistic effect of elevating arterial stiffness in asymptomatic Korean population.

  17. Presymptomatic genetic analysis during pregnancy for vascular type Ehlers–Danlos syndrome

    Science.gov (United States)

    Naing, Banyar Than; Watanabe, Atsushi; Tanigaki, Shinji; Ono, Masae; Iwashita, Mitsutoshi; Shimada, Takashi

    2014-01-01

    The vascular type of Ehlers–Danlos syndrome (EDS), EDS type IV (Online Mendelian Inheritance in Man [MIM] #130050) is characterized by thin, translucent skin, easy bruising, and arterial, intestinal, and/or uterine fragility during pregnancy, which may lead to sudden death. It is an autosomal dominant inherited disorder caused by type III procollagen gene (COL3A1: MIM #120180) mutations. Approximately 50% of the COL3A1 mutations are inherited from an affected parent, and 50% are de novo mutations. Each child of an affected individual has a 50% chance of inheriting the mutation and developing the disorder. Pregnant women with vascular EDS are at an increased risk of uterine and arterial rupture during the peripartum period, with high maternal morbidity and mortality rates. We report the first case of an asymptomatic 35-year-old woman at a risk of complications of vascular EDS who underwent presymptomatic evaluation during pregnancy. The sequencing results of both her brother and mother had a one-base-pair deletion, resulting in Glutamate at position 730 changing to Lysine and causing a frame shift and premature termination codon at 61 amino acids from the mutation position (p. Glu730Lysfs*61) on exon 32 of COL3A1. This deletion caused frameshift, leading to a premature termination codon (TAG) at 181 nucleotides downstream in exon 35, which could not be detected by previous total RNA (ribonucleic acid) method. Thus, she was at risk of complications of vascular EDS, and diagnostic testing was employed at 8 weeks of pregnancy to minimize the risk of developing vascular EDS-related complications. The negative presymptomatic diagnostic result allowed the patient to choose normal delivery at term. Vascular EDS is a serious disorder, with high mortality, especially in high-risk women with vascular EDS during pregnancy. The presymptomatic genetic testing of vascular EDS during pregnancy for a high-risk family can help with the early establishment of preventive measures

  18. Vascular thoracic outlet syndrome developed after minimally invasive repair of pectus excavatum.

    Science.gov (United States)

    Kılıç, Burcu; Demirkaya, Ahmet; Turna, Akif; Kaynak, Kamil

    2013-09-01

    The Nuss procedure is a minimally invasive surgical repair technique for pectus excavatum with fewer delayed complications compared to open procedures. We report the case of a 22-year-old man with deep pectus excavatum who developed vascular thoracic outlet syndrome after the Nuss procedure. Further evaluation demonstrated that the first rib was causing severe obstruction of the right subclavian artery. The patient showed clinical features of subclavian artery compression. A first rib resection, division of the anterior scalene muscle and fibrous bands provided complete relief of the complaints. The forced structural and spatial changes produced by the elevation of the depressed upper chest might have caused this complication. Vascular thoracic outlet syndrome should be kept in mind as a possible complication in patients who have undergone minimally invasive repair of pectus excavatum, and this complication can be treated by first rib resection.

  19. Role of Cox-2 in Vascular Inflammation: An Experimental Model of Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Nicolás F. Renna

    2013-01-01

    Full Text Available The objective of this work was to demonstrate the role of COX-2 enzyme at the vascular in experimental model of metabolic syndrome. SHR male WKY rats were employed; they were distributed in 8 groups (n=8 each: control (W; W + L: WKY rats receiving 20 mg/kg of lumiracoxib by intraesophageal administration; SHR; SHR + L: SHR + 20 mg/kg of lumiracoxib by intraesophageal administration; Fructose-Fed Rats (FFR: WKY rats receiving 10% (w/v fructose solution in drinking water during all 12 weeks; FFR + L: FFR + 20 mg/kg of lumiracoxib by intraesophageal administration; Fructose-Fed Hypertensive Rats (FFHR: SHR receiving 10% (w/v fructose solution in drinking water during all 12 weeks; and FFHR + L: FFHR + 20 mg/kg of lumiracoxib by intraesophageal administration. Metabolic variables, blood pressure, morphometric variables, and oxidative stress variables were evaluated; also MMP-2 and MMP-9 (collagenases, VCAM-1, and NF-κB by Westernblot or IFI were evaluated. FFHR presented all variables of metabolic syndrome; there was also an increase in oxidative stress variables; vascular remodeling and left ventricular hypertrophy were evidenced along with a significant increase in the expression of the mentioned proinflammatory molecules and increased activity and expression of collagenase. Lumiracoxib was able to reverse vascular remodeling changes and inflammation, demonstrating the involvement of COX-2 in the pathophysiology of vascular remodeling in this experimental model.

  20. RETINAL AND CHOROIDAL VASCULAR OCCLUSION FOLLOWING AQUEOUS MISDIRECTION SYNDROME IN A PATIENT WITH SICKLE CELL TRAIT.

    Science.gov (United States)

    Dewundara, Samantha; Nassiri, Nariman; Kim, Johnstone M; Kadikoy, Huseyin; Amde, Wendewessen; Tannir, Justin; Hughes, Bret A; Abrams, Gary W

    2017-05-09

    To report a patient with retinal and choroidal vascular occlusion as a presenting sign of sickle cell trait following the development of aqueous misdirection syndrome. Retrospective chart review. A patient treated for bilateral chronic angle-closure glaucoma with sequential EX-PRESS glaucoma filtration device surgery developed sequential bilateral aqueous misdirection syndrome. The left eye developed retinal arterial and localized choroidal vascular occlusions subsequent to an acute elevation in intraocular pressure and possibly the use of oral acetazolamide. The patient was subsequently found to have sickle cell trait. The right eye developed aqueous misdirection with acute elevation of intraocular pressure as well, but the patient was not treated with oral acetazolamide and did not develop vascular occlusion. Retinal and choroidal vascular occlusions can be the presenting sign of a patient with sickle cell trait. Sickle cell screening may be beneficial in African American or Middle Eastern patients after an acute rise in intraocular pressure, particularly before initiation of treatment with oral carbonic anhydrase inhibitors.

  1. Sustained release nitrite therapy results in myocardial protection in a porcine model of metabolic syndrome with peripheral vascular disease

    OpenAIRE

    Bradley, Jessica M.; Islam, Kazi N.; Polhemus, David J.; Donnarumma, Erminia; Brewster, Luke P.; Tao, Ya-Xiong; Goodchild, Traci T.; Lefer, David J.

    2015-01-01

    In a clinically relevant porcine model of metabolic syndrome and peripheral vascular disease, treatment with a novel sustained-release nitrite formulation restored cardiac endothelial nitric oxide synthase, enhancing myocardial nitrite levels, reduced oxidative stress, and improved ex vivo coronary vascular function via endothelium-independent vasodilation mechanism in obese Ossabaw swine.

  2. Berberine Attenuates Vascular Remodeling and Inflammation in a Rat Model of Metabolic Syndrome.

    Science.gov (United States)

    Li, Xiao-Xing; Li, Chuan-Bao; Xiao, Jie; Gao, Hai-Qing; Wang, He-Wen; Zhang, Xin-Yu; Zhang, Cheng; Ji, Xiao-Ping

    2015-01-01

    Berberine is a natural product that shows benefits for metabolic syndrome (MS). However, the effects of berberine on the improvement of vascular inflammation and remodeling in MS remain unclear. This study aimed to investigate whether berberine could prevent vascular remodeling and inflammation in the MS condition. A rat model of MS was established, and MS rats were divided into two groups: MS group without berberine treatment, and MSB group with berberine treatment (each group n-10). Ten normal Wistar rats were used as controls (NC group). Vascular damage was examined by transmission electron microscopy and pathological staining. Compared to the NC group, the secretion of inflammatory factors was increased and the aortic wall thicker in the MS group. The MSB group exhibited decreased secretion of inflammatory factors and improved vascular remodeling, compared to the MS group. In addition, the levels of p38 mitogen-activated protein kinase (p38 MAPK), activating transcription factor 2 (ATF-2) and matrix metalloproteinase 2 (MMP-2) were significantly decreased in the MSB group compared to the MS group. In conclusion, our data show that berberine improves vascular inflammation and remodeling in the MS condition, and this is correlated with the ability of berberine to inhibit p38 MAPK activation, ATF-2 phosphorylation, and MMP-2 expression.

  3. CUBITAL TUNNEL SYNDROME: REVIEW OF 14 ANTERIOR SUBCUTANEOUS TRANSPOSITIONS OF THE VASCULARIZED ULNAR NERVE

    Directory of Open Access Journals (Sweden)

    M. Farzan

    2005-06-01

    Full Text Available Anterior transposition of the ulnar nerve is widely implemented for treatment of cubital tunnel ‎syndrome. However, preservation of the extrinsic blood supply of the ‎ulnar nerve may result in better clinical outcomes. Fourteen patients with cubital tunnel ‎syndrome, 11 ‎men and 3 women, were treated by anterior subcutaneous transposition of the ulnar nerve. The extrinsic blood supply of the ulnar nerve was ‎preserved. The average age at the time of operation was 33 years. The average follow-up period was 44 months. Post-operative outcome assessment by an independent examiner was based on the modified Bishop rating system. Nine patients had excellent or good outcomes. Five patients had a fair outcome. There ‎were no complications or recurrence of symptoms. Anterior subcutaneous ‎transposition of the vascularized ulnar nerve is an effective method of surgical ‎treatment for patients with cubital tunnel syndrome.

  4. Metabolic syndrome and the development of vascular disease and type 2 diabetes in high-risk patients

    NARCIS (Netherlands)

    Wassink, A.M.J.

    2009-01-01

    Abdominal obesity and its associated insulin resistance play a key role in the clustering of vascular risk factors, known as Metabolic Syndrome. Subjects with Metabolic Syndrome are at increased risk for the development of both type 2 diabetes and cardiovascular disease. Type 2 diabetes and

  5. [Vascular and/or cardiac manifestations of type IV Ehlers-Danlos syndrome. 9 cases].

    Science.gov (United States)

    de Wazières, B; Coppere, B; Durieu, I; Fest, T; Ninet, J; Levrat, R; Vuitton, D A; Dupond, J L

    1995-10-14

    Type IV Ehlers-Danlos syndrome, a rare disease caused by abnormal synthesis of type III collagen, often leads to vascular fragility. We report 9 cases (6 men and 3 women, mean age 35 years). For 7 of the patients, the inaugural signs were arterial complications including haemoperitoneum in 2 patients with multiple aneurysmal dystrophy of the abdominal arteries, one case of ruptured subclavian artery, two dissections of the renal artery, one case with rupture of a cerebral aneurysm, one rupture of the mesenteric artery and a haematoma after arterial puncture. Other vascular manifestations were acrosyndrome (n = 4), varicose veins (n = 3), and prolapsus of the mitral valve (n = 2). In addition, 8 of the 9 patients presented extravascular signs. There was a history of familial disease in 5 cases. Pregnancy was completed to term in three patients: a cesarean section was required in one case and intra-uterine growth retardation was seen in 2. Morbidity was important with hemiparesia, blindness and paraparesis sequellae. One patient died from haemorrhage. This series of patients with type IV Ehlers-Danlos syndrome illustrates the severity of this disease whose prevalence is often underestimated. The disease is transmitted by autosomal dominant inheritance, underscoring the importance of familial testing for early diagnosis. Clinicians should be aware of the vascular manifestations and avoid invasive punctures or operations except in exceptional indications.

  6. Presymptomatic genetic analysis during pregnancy for vascular type Ehlers–Danlos syndrome

    Directory of Open Access Journals (Sweden)

    Naing BT

    2014-06-01

    Full Text Available Banyar Than Naing,1 Atsushi Watanabe,1,2 Shinji Tanigaki,3 Masae Ono,4 Mitsutoshi Iwashita,3 Takashi Shimada1,21Department of Biochemistry and Molecular Biology, Nippon Medical School, Tokyo, Japan; 2Division of Clinical Genetics, Nippon Medical School Hospital, Tokyo, Japan; 3Department of Obstetrics and Gynecology, Kyorin University School of Medicine, Tokyo, Japan; 4Department of Pediatrics, Kyorin University School of Medicine, Tokyo, JapanAbstract: The vascular type of Ehlers–Danlos syndrome (EDS, EDS type IV (Online Mendelian Inheritance in Man [MIM] #130050 is characterized by thin, translucent skin, easy bruising, and arterial, intestinal, and/or uterine fragility during pregnancy, which may lead to sudden death. It is an autosomal dominant inherited disorder caused by type III procollagen gene (COL3A1: MIM #120180 mutations. Approximately 50% of the COL3A1 mutations are inherited from an affected parent, and 50% are de novo mutations. Each child of an affected individual has a 50% chance of inheriting the mutation and developing the disorder. Pregnant women with vascular EDS are at an increased risk of uterine and arterial rupture during the peripartum period, with high maternal morbidity and mortality rates. We report the first case of an asymptomatic 35-year-old woman at a risk of complications of vascular EDS who underwent presymptomatic evaluation during pregnancy. The sequencing results of both her brother and mother had a one-base-pair deletion, resulting in Glutamate at position 730 changing to Lysine and causing a frame shift and premature termination codon at 61 amino acids from the mutation position (p. Glu730Lysfs*61 on exon 32 of COL3A1. This deletion caused frameshift, leading to a premature termination codon (TAG at 181 nucleotides downstream in exon 35, which could not be detected by previous total RNA (ribonucleic acid method. Thus, she was at risk of complications of vascular EDS, and diagnostic testing was employed

  7. Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Byers, Peter H; Belmont, John; Black, James; De Backer, Julie; Frank, Michael; Jeunemaitre, Xavier; Johnson, Diana; Pepin, Melanie; Robert, Leema; Sanders, Lynn; Wheeldon, Nigel

    2017-03-01

    Vascular Ehlers Danlos syndrome (vEDS) is an uncommon genetic disorders characterized by arterial aneurysm, dissection and rupture, bowel rupture, and rupture of the gravid uterus. The frequency is estimated as 1/50,000-1/200,000 and results from pathogenic variants in COL3A1, which encodes the chains of type III procollagen, a major protein in vessel walls and hollow organs. Initial diagnosis depends on the recognitions of clinical features, including family history. Management is complex and requires multiple specialists who can respond to and manage the major complications. A summary of recommendations for management include: Identify causative variants in COL3A1 prior to application of diagnosis, modulate life style to minimize injury, risk of vessel/organ rupture, identify and create care team, provide individual plans for emergency care ("vascular EDS passport") with diagnosis and management plan for use when traveling, centralize management at centers of excellence (experience) when feasible, maintain blood pressure in the normal range and treat hypertension aggressively, surveillance of vascular tree by doppler ultrasound, CTA (low radiation alternatives) or MRA if feasible on an annual basis. These recommendations represent a consensus of an international group of specialists with a broad aggregate experience in the care of individuals with vascular EDS that will need to be assessed on a regular basis as new information develops. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  8. Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

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    Filipa Pereira

    2015-01-01

    Full Text Available Ehlers-Danlos syndrome (EDS is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.

  9. Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

    Science.gov (United States)

    Pereira, Filipa; Cardoso, Teresa; Sá, Paula

    2015-01-01

    Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome. PMID:26175915

  10. Vascularized Lymph Node Flap Transfer and Lymphovenous Anastomosis for Klippel-Trenaunay Syndrome with Congenital Lymphedema

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    Shan Shan Qiu, MD

    2014-06-01

    Full Text Available Summary: A female patient with Klippel-Trenaunay syndrome, including hypertrophic bone and soft tissue in the forelimbs, bilateral lower limbs lymphedema, port-wine stains, and superficial veins of Servelle, was presented. The diagnosis of lymphedema was confirmed by lymphoscintigraphy and indocyanine green lymphography. The novel treatments consisted of vascularized lymph node transplantation to the left lymphedematous extremity and lymphovenous anastomosis to the right lymphedematous extremity. Significant improvements in subjective and objective clinical outcome were observed early in the postoperative period with continued improvements during the follow-up period.

  11. Tendon vascularity in overhead athletes with subacromial pain syndrome and its correlation with the resting subacromial space.

    Science.gov (United States)

    Tsui, Sammi Sin Mei; Leong, Hio Teng; Leung, Vivian Yee Fong; Ying, Michael; Fu, Siu Ngor

    2017-05-01

    Supraspinatus tendinopathy is one of the common causes of subacromial pain syndrome (SAPS) in overhead athletes. Changes in tendon vascularity have been reported in painful tendons; however, the prevalence and distribution have not been investigated in young overhead athletes. We conducted a cross-sectional study of 47 overhead athletes (male, 31; female, 16) aged 18 to 36 years with SAPS for >3 months. A sonographer graded the severity of the tendinopathy and area of vascularization. Ultrasound imaging was used to measure supraspinatus tendon thickness, vascularity, and resting subacromial space. A self-written program was used to semiquantify the intensity of vascularity, expressed as the vascular index. The majority (87.2%) of the participants had signs of tendinopathy in the supraspinatus tendon, and 40 (85.1%) of the tendinopathic tendons had vascularity. The majority (66.0%) of the vascularized subjects presented with minimal increase in vascularity, and 19.1% had moderate to severe vascularization. Most (79.2%) of the vascularization was observed in the pericortical region. The vascular index was negatively correlated with the resting subacromial space in male athletes with a reduced subacromial space (ρ = -0.63; P = .038). Of overhead athletes with SAPS, 87.2% had supraspinatus tendinopathy with minimal to moderate vascularization, with the majority of vascularization occurring in the pericortical region. In male athletes with a reduced subacromial space, greater vascularity in the supraspinatus tendon was associated with a smaller resting subacromial space. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  12. Ehlers-Danlos syndrome associated with fatal spontaneous vascular rupture in a dog.

    Science.gov (United States)

    Uri, M; Verin, R; Ressel, L; Buckley, L; McEwan, N

    2015-01-01

    A 7-month-old male cross breed dog was presented with hyperextensible skin and atrophic scarring. A diagnosis of Ehlers-Danlos syndrome was made based on clinical signs, histopathology and electron microscopy. Two weeks after presentation, the dog died suddenly. Post-mortem examination revealed haemothorax and rupture of the left subclavian artery. Histological findings, including Goldner's modified Masson's trichrome staining and transmission electron microscopy of the subclavian artery, revealed abnormalities in the structure and arrangement of collagen fibrils, suggesting that the defective collagen formation extended to the vasculature. To the authors' knowledge, this is the first report of Ehlers-Danlos syndrome with vascular involvement in animals. Copyright © 2014 Elsevier Ltd. All rights reserved.

  13. Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall.

    Science.gov (United States)

    Perrucci, Gianluca L; Rurali, Erica; Gowran, Aoife; Pini, Alessandro; Antona, Carlo; Chiesa, Roberto; Pompilio, Giulio; Nigro, Patrizia

    2017-01-01

    Marfan syndrome (MFS) is a connective tissue disorder with multiple organ manifestations. The genetic cause of this syndrome is the mutation of the FBN1 gene, encoding the extracellular matrix (ECM) protein fibrillin-1. This genetic alteration leads to the degeneration of microfibril structures and ECM integrity in the tunica media of the aorta. Indeed, thoracic aortic aneurysm and dissection represent the leading cause of death in MFS patients. To date, the most effective treatment option for this pathology is the surgical substitution of the damaged aorta. To highlight novel therapeutic targets, we review the molecular mechanisms related to MFS etiology in vascular smooth muscle cells, the foremost cellular type involved in MFS pathogenesis.

  14. Occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of the lung: report of a case.

    Science.gov (United States)

    Sa, Young Jo; Kim, Young Du; Moon, Seok-Whan; Kim, Chi-Kyung; Ki, Chang Seok

    2013-12-01

    An 8-year-old male presented with a cystic lung lesion in the left lower lobe, which was initially detected during surgery for a spontaneous rupture of the sigmoid colon at the age of 6 years. Tissue fragility and a tendency to bleed easily were noted during the surgery, which strongly suggested vascular Ehlers-Danlos syndrome. Although there was no abnormality in the hemostasis screening test, or any suspicious hereditary problem in his pedigree, genetic gene testing for vascular Ehlers-Danlos syndrome was recommended, and showed a de novo mutation in the COL3A1 gene. This report presents the case of patient with occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of lung, in addition to a duplicated infrarenal vena cava.

  15. Endothelial Progenitor Cell Dysfunction in Myelodysplastic Syndromes: Possible Contribution of a Defective Vascular Niche to Myelodysplasia

    Directory of Open Access Journals (Sweden)

    Luciana Teofili

    2015-05-01

    Full Text Available We set a model to replicate the vascular bone marrow niche by using endothelial colony forming cells (ECFCs, and we used it to explore the vascular niche function in patients with low-risk myelodysplastic syndromes (MDS. Overall, we investigated 56 patients and we observed higher levels of ECFCs in MDS than in healthy controls; moreover, MDS ECFCs were found variably hypermethylated for p15INK4b DAPK1, CDH1, or SOCS1. MDS ECFCs exhibited a marked adhesive capacity to normal mononuclear cells. When normal CD34+ cells were co-cultured with MDS ECFCs, they generated significant lower amounts of CD11b+ and CD41+ cells than in co-culture with normal ECFCs. At gene expression profile, several genes involved in cell adhesion were upregulated in MDS ECFCs, while several members of the Wingless and int (Wnt pathways were underexpressed. Furthermore, at miRNA expression profile, MDS ECFCs hypo-expressed various miRNAs involved in Wnt pathway regulation. The addition of Wnt3A reduced the expression of intercellular cell adhesion molecule-1 on MDS ECFCs and restored the defective expression of markers of differentiation. Overall, our data demonstrate that in low-risk MDS, ECFCs exhibit various primary abnormalities, including putative MDS signatures, and suggest the possible contribution of the vascular niche dysfunction to myelodysplasia.

  16. Hypoplastic left heart syndrome is associated with structural and vascular placental abnormalities and leptin dysregulation.

    Science.gov (United States)

    Jones, Helen N; Olbrych, Stephanie K; Smith, Kathleen L; Cnota, James F; Habli, Mounira; Ramos-Gonzales, Osniel; Owens, Kathryn J; Hinton, Andrea C; Polzin, William J; Muglia, Louis J; Hinton, Robert B

    2015-10-01

    Hypoplastic left heart syndrome (HLHS) is a severe cardiovascular malformation (CVM) associated with fetal growth abnormalities. Genetic and environmental factors have been identified that contribute to pathogenesis, but the role of the placenta is unknown. The purpose of this study was to systematically examine the placenta in HLHS with and without growth abnormalities. HLHS term singleton births were identified from a larger cohort when placenta tissue was available. Clinical data were collected from maternal and neonatal medical records, including anthropometrics and placental pathology reports. Placental tissues from cases and controls were analyzed to assess parenchymal morphology, vascular architecture and leptin signaling. HLHS cases (n = 16) and gestational age-matched controls (n = 18) were analyzed. Among cases, the average birth weight was 2993 g, including 31% that were small for gestational age. When compared with controls, gross pathology of HLHS cases demonstrated significantly reduced placental weight and increased fibrin deposition, while micropathology showed increased syncytial nuclear aggregates, decreased terminal villi, reduced vasculature and increased leptin expression in syncytiotrophoblast and endothelial cells. Placentas from pregnancies complicated by fetal HLHS are characterized by abnormal parenchymal morphology, suggesting immature structure may be due to vascular abnormalities. Increased leptin expression may indicate an attempt to compensate for these vascular abnormalities. Further investigation into the regulation of angiogenesis in the fetus and placenta may elucidate the causes of HLHS and associated growth abnormalities in some cases. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Delayed presentation of compartment syndrome of the thigh secondary to quadriceps trauma and vascular injury in a soccer athlete.

    Science.gov (United States)

    How, Moo Ing; Lee, Puah Ken; Wei, Tan See; Chong, Chua Tai

    2015-01-01

    Compartment syndrome isolated to the anterior thigh is a rare complication of soccer injury. Previous reports in the English literature on sports trauma-related compartment syndrome of the thigh are vague in their description of the response of thigh musculature to blunt trauma, magnetic resonance imaging (MRI) findings of high-risk features of compartment syndrome, vascular injury in quadriceps trauma, and the role of vascular study in blunt thigh injury. We present herein the rare case of a 30-year-old man who developed thigh compartment syndrome 8 days after soccer injury due to severe edema of vastus intermedius and large thigh hematoma secondary to rupture of the profunda femoris vein. MRI revealed "blow-out" rupture of the vastus lateralis. Decompressive fasciotomy and vein repair performed with subsequent split-skin grafting of the wound defect resulted in a good functional outcome at 2-years follow-up. A high index of suspicion for compartment syndrome is needed in all severe quadriceps contusion. Vascular injury can cause thigh compartment syndrome in sports trauma. MRI findings of deep thigh muscle swelling and "blow-out" tear of the vastus lateralis are strongly suggestive of severe quadriceps injury, and may be a harbinger of delayed thigh compartment syndrome. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  18. [Endothelial dysfunction as a marker of vascular aging syndrome on the background of hypertension, coronary heart disease, gout and obesity].

    Science.gov (United States)

    Vatseba, M O

    2013-09-01

    Under observation were 40 hypertensive patients with coronary heart disease, gout and obesity I and II degree. Patients with hypertension in combination with coronary heart disease, gout and obesity, syndrome of early vascular aging is shown by increased stiffness of arteries, increased peak systolic flow velocity, pulse blood presure, the thickness of the intima-media complex, higher level endotelinemia and reduced endothelial vasodilation. Obtained evidence that losartan in complex combination with basic therapy and metamaks in complex combination with basic therapy positively affect the elastic properties of blood vessels and slow the progression of early vascular aging syndrome.

  19. Cancer risk in patients with manifest vascular disease: effects of smoking, obesity, and metabolic syndrome.

    Science.gov (United States)

    van Kruijsdijk, Rob C M; van der Graaf, Yolanda; Peeters, Petra H M; Visseren, Frank L J

    2013-07-01

    Patients with vascular disease may be at increased risk of cancer because of shared risk factors and common pathogenesis. Patients with vascular disease (n = 6,172) were prospectively followed for cancer incidence. Standardized incidence ratios (SIRs) were calculated to compare the cancer incidence of the study population with that of the general population. Multivariable-adjusted hazard ratio's (HRs) of cancer were estimated for smoking status, pack-years, body mass index, waist circumference and visceral adipose tissue (VAT), and metabolic syndrome (MetS). During a median follow-up of 5.5 years, 563 patients were diagnosed with cancer. Patients with vascular disease were at increased risk of cancer [SIR = 1.19; 95% confidence interval (CI), 1.10-1.29]. Specifically, risk of lung cancer (SIR = 1.56; 95% CI, 1.31-1.83), as well as bladder cancer (SIR = 1.60; 95% CI, 1.11-2.24) and cancer of the lip, oral cavity, or pharynx in men (SIR = 1.51; 95% CI, 0.89-2.39), and colorectal (SIR = 1.71; 95% CI, 1.11-2.53) and kidney cancer (SIR = 2.92; 95% CI, 1.05-6.38) in women was increased. A relation between smoking and cancer risk was observed (HR for current smokers = 1.37; 95% CI, 1.05-1.73), whereas an increase in VAT was associated with higher breast cancer risk in women (HR = 1.42; 95% CI, 1.03-1.96). No relation between MetS and cancer risk was found. Patients with vascular disease have a 19% higher cancer risk compared to the general population. Smoking increased cancer risk and abdominal obesity is a risk factor for breast cancer in female patients with vascular disease. These results call for awareness of the increased cancer risk in patients with vascular disease among physicians and underline the necessity of lifestyle improvement not only for reducing cardiovascular risk.

  20. Effects of black raspberry on lipid profiles and vascular endothelial function in patients with metabolic syndrome.

    Science.gov (United States)

    Jeong, Han Saem; Hong, Soon Jun; Lee, Tae-Bum; Kwon, Ji-Wung; Jeong, Jong Tae; Joo, Hyung Joon; Park, Jae Hyoung; Ahn, Chul-Min; Yu, Cheol Woong; Lim, Do-Sun

    2014-10-01

    Black raspberry (Rubus occidentalis) has been known for its anti-inflammatory and anti-oxidant effects. However, short-term effects of black raspberry on lipid profiles and vascular endothelial function have not been investigated in patients with metabolic syndrome. Patients with metabolic syndrome (n = 77) were prospectively randomized into a group with black raspberry (n = 39, 750 mg/day) and a placebo group (n = 38) during a 12-week follow-up. Lipid profiles, brachial artery flow-mediated dilatation (baFMD), and inflammatory cytokines such as IL-6, TNF-α, C-reactive protein, adiponectin, sICAM-1, and sVCAM-1 were measured at the baseline and at the 12-week follow-up. Decreases from the baseline in the total cholesterol level (-22.8 ± 30.4 mg/dL vs. -1.9 ± 31.8 mg/dL, p raspberry than in the placebo group. Increases in baFMD at the 12-week follow-up were significantly greater in the group with black raspberry than in the placebo group (0.33 ± 0.44 mm vs. 0.10 ± 0.35 mm, p raspberry. The use of black raspberry significantly decreased serum total cholesterol level and inflammatory cytokines, thereby improving vascular endothelial function in patients with metabolic syndrome during the 12-week follow-up. Copyright © 2014 John Wiley & Sons, Ltd.

  1. Frailty syndrome and the risk of vascular dementia: the Italian Longitudinal Study on Aging.

    Science.gov (United States)

    Solfrizzi, Vincenzo; Scafato, Emanuele; Frisardi, Vincenza; Seripa, Davide; Logroscino, Giancarlo; Maggi, Stefania; Imbimbo, Bruno P; Galluzzo, Lucia; Baldereschi, Marzia; Gandin, Claudia; Di Carlo, Antonio; Inzitari, Domenico; Crepaldi, Gaetano; Pilotto, Alberto; Panza, Francesco

    2013-03-01

    Frailty is a clinical syndrome generally associated with a greater risk for adverse outcomes such as falls, disability, institutionalization, and death. Cognition and dementia have already been considered as components of frailty, but the role of frailty as a possible determinant of dementia, Alzheimer's disease (AD), and vascular dementia (VaD) has been poorly investigated. We estimated the predictive role of frailty syndrome on incident dementia and its subtypes in a nondemented, Italian, older population. We evaluated 2581 individuals recruited from the Italian Longitudinal Study on Aging sample population consisting of 5632 subjects aged 65 to 84 years and with a 3.9-year median follow-up. A phenotype of frailty according to a modified measurement of Cardiovascular Health Study criteria was operationalized. Dementia, AD, and VaD were classified using current published criteria. Over a 3.5-year follow-up, 65 of 2581 (2.5%) older subjects, 16 among 252 frail individuals (6.3%), of which 9 were affected by VaD (3.6%), developed overall dementia. In a proportional hazards model, frailty syndrome was associated with a significantly increased risk of overall dementia (adjusted hazard ratio: 1.85; 95% confidence interval: 1.01-3.40) and, in particular, VaD (adjusted hazard ratio: 2.68; 95% confidence interval: 1.16-7.17). The risk of AD or other types of dementia did not significantly change in frail individuals in comparison with subjects without frailty syndrome. In our large population-based sample, frailty syndrome was a short-term predictor of overall dementia and VaD. Copyright © 2013 The Alzheimer's Association. Published by Elsevier Inc. All rights reserved.

  2. Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association.

    Science.gov (United States)

    Marnet, Dominique; Vinchon, Matthieu; Mostofi, Keyvan; Catteau, Benoit; Kerdraon, Olivier; Dhellemmes, Patrick

    2009-12-01

    Neurocutaneous melanosis represents a rare congenital but nonheritable phakomatosis defined as the association of giant or multiple congenital nonmalignant melanocytic nevi with leptomeningeal melanosis or melanoma of the central nervous system. We describe the case of an adolescent with a giant congenital bathing trunk melanocytic nevus who developed progressive intracranial hypertension due to leptomeningeal melanosis confirmed by surgical biopsy. Brain and spine magnetic resonance images showed posterior fossa malformation compatible with the Dandy-Walker complex, hydrocephalus, and extensive enhancement of posterior fossa then spine. Shunt placement, corticotherapy, and chemotherapy were attempted leading to transient relief but the boy died 12 months after the onset of primary neurological symptoms. We discuss diagnosis, pathogenesis, management, and prognosis in the light of data from the recent literature. Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy-Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.

  3. Salter-Harris II injury of the proximal tibial epiphysis with both vascular compromise and compartment syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Clement Nicholas D

    2009-06-01

    Full Text Available Abstract We present a case of a Salter-Harris II injury to the proximal tibia associated with both vascular compromise and compartment syndrome. The potential complications of this injury are limb threatening and the neurovasular status of the limb should be continually monitored. Maintaining anatomic reduction is difficult and fixation may be needed to achieve optimal results.

  4. Ultrastructural scoring of skin biopsies for diagnosis of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Ong, Kim-Thanh; Plauchu, Henri; Peyrol, Simone; Roux, Elisabeth; Errazuriz, Elisabeth; Khau Van Kien, Philippe; Arbeille, Brigitte; Gaulier, Alain; Georgescou, Gabriela; Collignon, Patrick; Germain, Dominique P; Gaveau, Marie-Noëlle; Perdu, Jérôme; Laurent, Stéphane; Bruneval, Patrick; Boutouyrie, Pierre

    2012-06-01

    Vascular Ehlers-Danlos syndrome (vEDS) results from a mutation in the gene encoding alpha-1, type III pro-collagen (COL3A1) and confers fragility to skin, ligament and vascular tissue. We tested the value of skin biopsy for diagnosis of vEDS through an ultrastructure scoring procedure. Study design was a multicentric, case-control, blinded trial consisting of two phases: phase 1 was to identify an ultra-structure score providing the best discriminative value for vEDS and phase 2 was to replicate this result in a different population. We enrolled 103 patients, 66 cases defined through the revised nosology for Ehlers-Danlos syndromes and 37 control subjects selected from patients referred for other pathologies. Ultrastructure of extracellular matrix was read by three to five experienced pathologists blinded for diagnosis. We used the receiver operating curves and logistic regression analysis for ranking ultrastructure scores. We created a detailed description of lesions observed in vEDS patients with 27 items (coded 0 or 1). In the phase 1 (17 cases and 20 controls), abnormal fibroblast shape, presence of lysosomes in the fibroblast and abnormal basal lamina were found to be independent discriminative items. Addition of these three items (defining an ultrastructure score) had the best diagnosis value (area under the curve (AUC) = 0.96). In the phase 2 (49 cases, 17 controls), ultrastructure score provided odds ratio of 9.76 (95 % CI 2.91-32.78), and AUC of 0.90. The ultrastructure score of skin biopsy has predictive value for the diagnosis of vEDS. Presence of two or more signs (either abnormal fibroblast, presence of lysosomes in the fibroblast or abnormal basal lamina) is very evocative of vEDS.

  5. Carotid ultrasonographic and brain computerized tomographic findings in patients with vascular ocular syndromes

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    Iwamoto, Toshihiko; Matsushima, Chikage; Shimizu, Souichirou; Takasaki, Masaru; Iwasaki, Takuya; Usui, Masahiko [Tokyo Medical Coll. (Japan)

    2002-02-01

    To clarify the characteristics of cerebrovascular lesions in subtypes of vascular ocular syndrome, including amaurosis fugax (AF), retinal artery occlusion (RAO), and retinal vein occlusion (RVO), 93 patients with vascular ocular syndrome were studied by means of carotid ultrasonography (US) and brain computerized tomography (CT). The subjects comprised 21 patients with AF, 37 with RAO, and 35 with RVO who were sequentially given these diagnoses by the department of ophthalmology. On the basis of US findings, carotid lesions were defined as the presence of plaque or stenotic changes. CT findings were assessed for the presence and distribution of low-density areas (LDAs). Mean age was similar in each group, ranging from 64.5 to 67.4 years. The RAO group had high rates of men, hypertension, and smokers. US showed that the prevalence of carotid lesions ipsilateral to the affected eye was high in the RAO group and that severe stenosis and ulcerated plaque were present in 28.6% of the AF group and 45.9% of the RAO group. On CT examination, cerebral infarctions appeared as LDAs in about 10% of the patients in each group, and the incidence and distribution of LDAs were similar. Of 13 patients with cerebral infarction, only 2 were presumably due to carotid lesions; the others had a variety of causes. The discrepancy between US and CT findings was attributed to the small number of patients with cerebral infarction, since most patients had visual defects as an initial symptom. Our results suggest that extracranial carotid lesions, considered to be a major risk factor for stroke, should be carefully assessed in patients with AF or RAO to prevent further stroke. (author)

  6. Amlodipine enhances amelioration of vascular insulin resistance, oxidative stress, and metabolic disorders by candesartan in metabolic syndrome rats.

    Science.gov (United States)

    Sueta, Daisuke; Nakamura, Taishi; Dong, Yi-Fei; Kataoka, Keiichiro; Koibuchi, Nobutaka; Yamamoto, Eiichiro; Toyama, Kensuke; Yasuda, Osamu; Ogawa, Hisao; Kim-Mitsuyama, Shokei

    2012-06-01

    The pharmacological advantage of combination of an angiotensin receptor blocker (ARB) and a calcium-channel blocker (CCB) is not fully defined. This study was undertaken to elucidate the potential benefit of their combination in metabolic syndrome. SHR/NDmcr-cp (SHRcp), a rat model of human metabolic syndrome, were divided into four groups, and were administered (i) vehicle, (ii) candesartan (an ARB) 0.3 mg/kg/day, (iii) amlodipine (a CCB) 3 mg/kg/day, and (iv) candesartan 0.3 mg/kg/day plus amlodipine 3 mg/kg/day, for 4 weeks. Candesartan, amlodipine, or their combination significantly ameliorated the impairment of vascular endothelium-dependent relaxation with acetylcholine in SHRcp. However, the impairment of insulin-induced vasodilation in SHRcp was partially improved by candesartan alone, but not by amlodipine alone. Interestingly, amlodipine added to candesartan synergistically enhanced the improvement of impaired insulin-induced vasodilation by candesartan, indicating the synergistic improvement of vascular insulin resistance by the combination of these drugs. Candesartan alone, but not amlodipine alone, significantly attenuated vascular superoxide and NADPH oxidase subunit p22phox in SHRcp. Amlodipine added to candesartan synergistically enhanced the reduction of vascular p22phox levels and superoxide by candesartan in SHRcp, suggesting the association of vascular insulin resistance with oxidative stress. Furthermore, the combination of candesartan with amlodipine synergistically decreased the increase in visceral adipocyte size, serum free-fatty acid, and tumor necrosis factor-α in SHRcp. ARB and CCB combination synergistically ameliorated vascular insulin resistance in metabolic syndrome, being associated with the synergistic attenuation of vascular oxidative stress and metabolic disorders.

  7. Sjogren-Larsson syndrome: 2 case reports

    NARCIS (Netherlands)

    Galoin-Bertail, C.; Ogier de Baulny, H.; Wanders, R.; Schiff, M.; Bellavoine, V.; Mlika, A.; Benoist, G.; Baruteau, J.

    2012-01-01

    Sjogren-Larsson syndrome (SLS) is a neurocutaneous autosomal recessive disease caused by fatty aldehyde dehydrogenase (FADH) deficiency. This enzyme is involved in the biosynthesis pathways of some fatty acids, phytanic acid, and leukotrienes. The main features of the disease are its association

  8. Distinct effects of losartan and atenolol on vascular stiffness in Marfan syndrome.

    Science.gov (United States)

    Bhatt, Ami B; Buck, J Stewart; Zuflacht, Jonah P; Milian, Jessica; Kadivar, Samoneh; Gauvreau, Kimberlee; Singh, Michael N; Creager, Mark A

    2015-08-01

    We conducted a randomized, double-blind trial of losartan (100 mg QD) versus atenolol (50 mg QD) for 6 months in adults with Marfan syndrome. Carotid-femoral pulse wave velocity (PWV), central augmentation index (AIx), aortic diameter and left ventricular (LV) function were assessed with arterial tonometry and echocardiography. Thirty-four subjects (18 female; median age 35 years, IQR 27, 45) were randomized. Central systolic and diastolic blood pressure decreased comparably with atenolol and losartan (p = 0.64 and 0.31, respectively); heart rate decreased with atenolol (p = 0.02), but not with losartan. PWV decreased in patients treated with atenolol (-1.15 ± 1.68 m/s; p = 0.01), but not in those treated with losartan (-0.22 ± 0.59 m/s; p = 0.15; between-group difference p = 0.04). In contrast, AIx decreased in the losartan group (-9.6 ± 8.6%; p losartan reduces the AIx. By improving vascular stiffness via distinct mechanisms of action, there is physiologic value to considering the use of both medications in individuals with Marfan syndrome. © The Author(s) 2015.

  9. Vascular Quadrilateral Space Syndrome in 3 Overhead Throwing Athletes: An Underdiagnosed Cause of Digital Ischemia.

    Science.gov (United States)

    Rollo, Johnathon; Rigberg, David; Gelabert, Hugh

    2017-07-01

    Vascular quadrilateral space syndrome (vQSS) is an underdiagnosed cause of extremity ischemia, pain, and paresthesia in overhand throwing athletes. The mechanism of vQSS is thought to result from repeated abduction and external rotation of the arm leading to a distraction injury of the posterior circumflex humoral artery (PCHA) as this courses through the quadrilateral space. This trauma may cause dissection and dissecting aneurysm formation. Thrombus from this arterial injury then embolizes down the arm resulting in the symptomatic presentation. Patients were often presented after multiple embolic events, which have resulted in obliteration of digital arteries. Later stages of presentation may include ischemic ulceration and gangrene. We report 3 cases of vQSS in overhand throwing athletes. All 3 underwent surgical correction and have returned to competition. We discuss presentation, diagnosis, imaging findings, management, outcomes, and review of the literature. Prompt recognition of this syndrome is essential to optimal treatment, which includes PCHA ligation and division with or without thrombolytic therapy. Increased awareness of vQSS is needed among coaches and athletic trainers who often identify the symptoms and initiate the treatment. When treated promptly, these athletes return to baseline functional status. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Vascular smooth muscle cell phenotypic changes in patients with Marfan syndrome.

    Science.gov (United States)

    Crosas-Molist, Eva; Meirelles, Thayna; López-Luque, Judit; Serra-Peinado, Carla; Selva, Javier; Caja, Laia; Gorbenko Del Blanco, Darya; Uriarte, Juan José; Bertran, Esther; Mendizábal, Yolanda; Hernández, Vanessa; García-Calero, Carolina; Busnadiego, Oscar; Condom, Enric; Toral, David; Castellà, Manel; Forteza, Alberto; Navajas, Daniel; Sarri, Elisabet; Rodríguez-Pascual, Fernando; Dietz, Harry C; Fabregat, Isabel; Egea, Gustavo

    2015-04-01

    Marfan's syndrome is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix microfibrils and chronic tissue growth factor (TGF)-β signaling. TGF-β is a potent regulator of the vascular smooth muscle cell (VSMC) phenotype. We hypothesized that as a result of the chronic TGF-β signaling, VSMC would alter their basal differentiation phenotype, which could facilitate the formation of aneurysms. This study explores whether Marfan's syndrome entails phenotypic alterations of VSMC and possible mechanisms at the subcellular level. Immunohistochemical and Western blotting analyses of dilated aortas from Marfan patients showed overexpression of contractile protein markers (α-smooth muscle actin, smoothelin, smooth muscle protein 22 alpha, and calponin-1) and collagen I in comparison with healthy aortas. VSMC explanted from Marfan aortic aneurysms showed increased in vitro expression of these phenotypic markers and also of myocardin, a transcription factor essential for VSMC-specific differentiation. These alterations were generally reduced after pharmacological inhibition of the TGF-β pathway. Marfan VSMC in culture showed more robust actin stress fibers and enhanced RhoA-GTP levels, which was accompanied by increased focal adhesion components and higher nuclear localization of myosin-related transcription factor A. Marfan VSMC and extracellular matrix measured by atomic force microscopy were both stiffer than their respective controls. In Marfan VSMC, both in tissue and in culture, there are variable TGF-β-dependent phenotypic changes affecting contractile proteins and collagen I, leading to greater cellular and extracellular matrix stiffness. Altogether, these alterations may contribute to the known aortic rigidity that precedes or accompanies Marfan's syndrome aneurysm formation. © 2015 American Heart Association, Inc.

  11. Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease

    Directory of Open Access Journals (Sweden)

    Scott Paul J

    2005-01-01

    Full Text Available Abstract Background Asthma and sickle cell disease are common conditions that both may result in pulmonary complications. We hypothesized that children with sickle cell disease with concomitant asthma have an increased incidence of vaso-occlusive crises that are complicated by episodes of acute chest syndrome. Methods A 5-year retrospective chart analysis was performed investigating 48 children ages 3–18 years with asthma and sickle cell disease and 48 children with sickle cell disease alone. Children were matched for age, gender, and type of sickle cell defect. Hospital admissions were recorded for acute chest syndrome, cerebral vascular accident, vaso-occlusive pain crises, and blood transfusions (total, exchange and chronic. Mann-Whitney test and Chi square analysis were used to assess differences between the groups. Results Children with sickle cell disease and asthma had significantly more episodes of acute chest syndrome (p = 0.03 and cerebral vascular accidents (p = 0.05 compared to children with sickle cell disease without asthma. As expected, these children received more total blood transfusions (p = 0.01 and chronic transfusions (p = 0.04. Admissions for vasoocclusive pain crises and exchange transfusions were not statistically different between cases and controls. SS disease is more severe than SC disease. Conclusions Children with concomitant asthma and sickle cell disease have increased episodes of acute chest syndrome, cerebral vascular accidents and the need for blood transfusions. Whether aggressive asthma therapy can reduce these complications in this subset of children is unknown and requires further studies.

  12. [Platelet-vascular and red cell components of hemostasis in hemodialyzed patients suffering from sever hemorrhagic fever with renal syndrome].

    Science.gov (United States)

    Davidovich, I M; Parshina, T A

    1999-01-01

    To evaluate effect of hemodialysis on functional state of platelet-vascular and red cell components of hemostasis in patients suffering from severe hemorrhagic fever with renal syndrome (SHFRS). Parameters of platelet-vascular and red cell hemostasis were studied in 51 patients with SHFRS who were divided into two groups. 27 patients of group 1 received conservative treatment, 24 patients of group 2 were put on dialysis. In oliguria, red cell deformity, spontaneous and induced aggregation was similar in both groups. There was a statistically significant anemia, platelet hyperreaction. The detected disorders in hemostasis are attributed to severity of the disease but not effects of hemodialysis.

  13. Vascular-type Ehlers-Danlos syndrome caused by a hitherto unknown genetic mutation: a case report

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    Kashizaki Fumihiro

    2013-02-01

    Full Text Available Abstract Introduction Vascular-type Ehlers-Danlos syndrome is an autosomal dominant disease that causes arterial spurting, intestinal perforation, uterine rupture and hemopneumothorax due to decreased production of type III collagen. The average age at death is 48 years old, and it is considered to be the most severe form of Ehlers-Danlos syndrome. We report the case of a 64-year-old Japanese woman and her 38-year-old daughter who were diagnosed with this disease. Case presentation A 64-year-old Japanese woman was referred to our hospital because of right anterior chest pain following cough and pharyngeal discomfort. Pleurisy was suspected due to the presence of right pleural effusion, so the next day she was referred to our department, where a detailed examination led to the diagnosis of hemothorax. The bleeding that caused the right hemothorax was difficult to control, so our patient was transferred to the Department of Thoracic Surgery for hemostasis control. Our patient’s personal history of uterine hemorrhage and skin ulcers, as well as the finding of skin fragility during surgery, were indicative of a weak connective tissue disease; therefore, after improvement of the hemothorax, a genetic analysis was performed. This revealed a heterozygous missense mutation in COL3A1, c.2411 G>T p.Gly804Val (exon 36. A detailed investigation conducted at a later date revealed that her daughter also had the same genetic mutation. This led to the diagnosis of vascular-type Ehlers-Danlos syndrome characterized by a new gene mutation. Conclusion We report a new genetic mutation associated with vascular-type Ehlers-Danlos syndrome. We present the clinical and imaging findings, and the disease and treatment course in this patient. We believe this information will be important in treating future cases of vascular-type Ehlers-Danlos syndrome in patients with this mutation.

  14. Vascular endothelial growth factor (VEGFA gene variation in polycystic ovary syndrome in a Tunisian women population

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    Assila Ben Salem

    2016-10-01

    Full Text Available Abstract Background Polycystic ovary syndrome (PCOS is characterized by the growth of a number of small cysts on the ovaries which leads to sex hormonal imbalance. Women who are affected by this syndrome suffer from irregular menstrual cycles, decline in their fertility, excessive hair growth, obesity, acne and most importantly cardiac function problems. The vascular endothelial growth factor (VEGF plays a pivotal role in tissue vascularization in general and in the pathogenesis of many diseases. The PCOS was found to be associated with high expression levels of VEGF. In women who undergo assisted reproductive procedures (ART, VEGF was found to be a key mediator of other factors to control ovary angiogenesis. Here, we set out to examine the association of VEGFA gene polymorphism with PCOS and its components in a population of Tunisia women to enhance our understanding of the genetic background leading angiogenesis and vascularization abnormalities in PCOS. Methods The association of VEGFA gene with PCOS and its components was examined in a cohort of 268 women from Tunisia involving 118 PCOS patients and 150 controls. VEGFA gene variations were assessed through the analysis of the following SNPs rs699947 (A/C, rs833061 (C/T, rs1570360 (G/A, rs833068 (G/A, rs3025020 (C/T, and rs3025039 (C/T. The linkage disequilibrium between SNPs was assessed using HAPLOVIEW software while combination of SNPs into haplotypes in the population and the reconstruction of the cladogram were carried-out by PHASE and ARLEQUIN programs, respectively. Genetic association and genotype-phenotype correlations were calculated by logistic regression and non-parametric tests (Kruskall-Wallis and Mann–Whitney tests, respectively, using StatView program. Results We observed 10 haplotypes in our studied cohort whereH1 (ACGG, H2 (ACAG, H7 (CTGG and H8 (CTGA were the most frequent. We observed the association of the genotype CT of the SNP rs30225039 with PCOS phenotype (P = 0

  15. Oral phenotype and scoring of vascular Ehlers–Danlos syndrome: a case–control study

    Science.gov (United States)

    Frank, Michael; Gogly, Bruno; Golmard, Lisa; Naveau, Adrien; Chérifi, Hafida; Emmerich, Joseph; Gaultier, Frédérick; Berdal, Ariane; Jeunemaitre, Xavier; Fournier, Benjamin P J

    2012-01-01

    Objective Vascular Ehlers–Danlos syndrome (vEDS) is a rare genetic condition related to mutations in the COL3A1 gene, responsible of vascular, digestive and uterine accidents. Difficulty of clinical diagnosis has led to the design of diagnostic criteria, summarised in the Villefranche classification. The goal was to assess oral features of vEDS. Gingival recession is the only oral sign recognised as a minor diagnostic criterion. The authors aimed to check this assumption since bibliographical search related to gingival recession in vEDS proved scarce. Design Prospective case–control study. Setting Dental surgery department in a French tertiary hospital. Participants 17 consecutive patients with genetically proven vEDS, aged 19–55 years, were compared with 46 age- and sex-matched controls. Observations Complete oral examination (clinical and radiological) with standardised assessment of periodontal structure, temporomandibular joint function and dental characteristics were performed. COL3A1 mutations were identified by direct sequencing of genomic or complementary DNA. Results Prevalence of gingival recession was low among patients with vEDS, as for periodontitis. Conversely, patients showed marked gingival fragility, temporomandibular disorders, dentin formation defects, molar root fusion and increased root length. After logistic regression, three variables remained significantly associated to vEDS. These variables were integrated in a diagnostic oral score with 87.5% and 97% sensitivity and specificity, respectively. Conclusions Gingival recession is an inappropriate diagnostic criterion for vEDS. Several new specific oral signs of the disease were identified, whose combination may be of greater value in diagnosing vEDS. PMID:22492385

  16. [Vascular dementia

    NARCIS (Netherlands)

    Leeuw, H.F. de; Gijn, J. van

    2004-01-01

    Vascular dementia is one of the most frequently occurring dementia syndromes. Its prevalence is about 5% among subjects above 85 years of age. Elevated blood pressure and atherosclerosis are the most important risk factors. According to international criteria, vascular dementia usually occurs within

  17. Aerobic interval training reduces vascular resistances during submaximal exercise in obese metabolic syndrome individuals.

    Science.gov (United States)

    Mora-Rodriguez, Ricardo; Fernandez-Elias, V E; Morales-Palomo, F; Pallares, J G; Ramirez-Jimenez, M; Ortega, J F

    2017-08-12

    The aim of this study was to determine the effects of high-intensity aerobic interval training (AIT) on exercise hemodynamics in metabolic syndrome (MetS) volunteers. Thirty-eight, MetS participants were randomly assigned to a training (TRAIN) or to a non-training control (CONT) group. TRAIN consisted of stationary interval cycling alternating bouts at 70-90% of maximal heart rate during 45 min day(-1) for 6 months. CONT maintained baseline physical activity and no changes in cardiovascular function or MetS factors were detected. In contrast, TRAIN increased cardiorespiratory fitness (14% in VO2PEAK; 95% CI 9-18%) and improved metabolic syndrome (-42% in Z score; 95% CI 83-1%). After TRAIN, the workload that elicited a VO2 of 1500 ml min(-1) increased 15% (95% CI 5-25%; P < 0.001). After TRAIN when subjects pedaled at an identical submaximal rate of oxygen consumption, cardiac output increased by 8% (95% CI 4-11%; P < 0.01) and stroke volume by 10% (95% CI, 6-14%; P < 0.005) being above the CONT group values at that time point. TRAIN reduced submaximal exercise heart rate (109 ± 15-106 ± 13 beats min(-1); P < 0.05), diastolic blood pressure (83 ± 8-75 ± 8 mmHg; P < 0.001) and systemic vascular resistances (P < 0.01) below CONT values. Double product was reduced only after TRAIN (18.2 ± 3.2-17.4 ± 2.4 bt min(-1) mmHg 10(-3); P < 0.05). The data suggest that intense aerobic interval training improves hemodynamics during submaximal exercise in MetS patients. Specifically, it reduces diastolic blood pressure, systemic vascular resistances, and the double product. The reduction in double product, suggests decreased myocardial oxygen demands which could prevent the occurrence of adverse cardiovascular events during exercise in this population. CLINICALTRIALS. NCT03019796.

  18. Vascular Ehlers–Danlos syndrome with cryptorchidism, recurrent pneumothorax, and pulmonary capillary hemangiomatosis-like foci

    Science.gov (United States)

    Park, Min A.; Shin, So Youn; Kim, Young Jin; Park, Myung Jae; Lee, Seung Hyeun

    2017-01-01

    Abstract Rationale: Vascular Ehlers–Danlos syndrome (vEDS) is a rare autosomal dominant inherited collagen disorder caused by defects or deficiency of pro-alpha 1 chain of type III procollagen encoded by COL3A1. vEDS is characterized not only by soft tissue manifestations including hyperextensibility of skin and joint hypermobility but also by early mortality due to rupture of arteries or vital organs. Although pulmonary complications are not common, vEDS cases complicated by pneumothorax, hemothorax, or intrapulmonary hematoma have been reported. When a patient initially presents only with pulmonary complications, it is not easy for clinicians to suspect vEDS. Patient concerns: We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. Diagnoses: Routine laboratory findings were unremarkable. Chest high resolution computed tomographic scan showed age-unmatched hyperinflation of both lungs, atypical cystic changes and multifocal ground glass opacities scattered in both lower lobes. His slender body shape, hyperflexible joints, and hyperextensible skin provided clue to suspicion of a possible connective tissue disorder. Interventions: The histological examination of the lung lesions showed excessive capillary proliferation in the pulmonary interstitium and pleura allowing the diagnosis of pulmonary capillary hemangiomatosis (PCH)-like foci. Genetic study revealed COL3A1 gene splicing site mutation confirming his diagnosis as vEDS. Outcomes: Although his diagnosis vEDS is notorious for fatal vascular complication, there was no evidence of such complication at presentation. Fortunately, he has been followed up for 10 months without pulmonary or vascular complications. Lessons: To the best of our knowledge, both cryptorchidism and PCH-like foci have never been reported yet as complications of vEDS, suggesting our case might be a new variant of this condition. This case emphasizes the

  19. Maternal endothelial function and vascular stiffness after HELLP syndrome: a case-control study.

    Science.gov (United States)

    Orabona, R; Sciatti, E; Vizzardi, E; Bonadei, I; Prefumo, F; Valcamonico, A; Metra, M; Frusca, T

    2017-11-01

    independently predicted endothelial dysfunction at 6 months to 4 years postpartum, after correcting for uterine artery pulsatility index, birth-weight percentile, and maternal blood pressure, age and body mass index. Women with both previous HELLP and early-onset IUGR had a significantly higher prevalence of endothelial dysfunction (P = 0.001). Similar vascular abnormalities were found in women previously affected by HELLP syndrome and those with previous PE without HELLP. However, a history of HELLP syndrome, IUGR and early-onset PE seems to identify a subgroup of women with a higher risk for future development of endothelial dysfunction. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

  20. Effect of intermittent vitamin D3 on vascular function and symptoms in chronic fatigue syndrome--a randomised controlled trial.

    Science.gov (United States)

    Witham, M D; Adams, F; McSwiggan, S; Kennedy, G; Kabir, G; Belch, J J F; Khan, F

    2015-03-01

    Low 25-hydroxyvitamin D levels are common in patients with chronic fatigue syndrome; such patients also manifest impaired vascular health. We tested whether high-dose intermittent oral vitamin D therapy improved markers of vascular health and fatigue in patients with chronic fatigue syndrome. Parallel-group, double-blind, randomised placebo-controlled trial. Patients with chronic fatigue syndrome according to the Fukuda (1994) and Canadian (2003) criteria were randomised to receive 100,000 units oral vitamin D3 or matching placebo every 2 months for 6 months. The primary outcome was arterial stiffness measured using carotid-femoral pulse wave velocity at 6 months. Secondary outcomes included flow-mediated dilatation of the brachial artery, blood pressure, cholesterol, insulin resistance, markers of inflammation and oxidative stress, and the Piper Fatigue scale. As many as 50 participants were randomised; mean age 49 (SD 13) years, mean baseline pulse wave velocity 7.8 m/s (SD 2.3), mean baseline office blood pressure 128/78 (18/12) mmHg and mean baseline 25-hydroxyvitamin D level 46 (18) nmol/L. 25-hydroxyvitamin D levels increased by 22 nmol/L at 6 months in the treatment group relative to placebo. There was no effect of treatment on pulse wave velocity at 6 months (adjusted treatment effect 0.0 m/s; 95% CI -0.6 to 0.6; p = 0.93). No improvement was seen in other vascular and metabolic outcomes, or in the Piper Fatigue scale at 6 months (adjusted treatment effect 0.2 points; 95% CI -0.8 to 1.2; p = 0.73). High-dose oral vitamin D3 did not improve markers of vascular health or fatigue in patients with chronic fatigue syndrome. www.controlled-trials.com, ISRCTN59927814. Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Spontaneous Colon Perforations Associated with a Vascular Type of Ehlers-Danlos Syndrome

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    Akira Yoneda

    2014-05-01

    Full Text Available Ehlers-Danlos syndrome, vascular type (vEDS (MIM #130050 is an autosomal dominant disorder caused by mutation in the type III collagen gene, COL3A1, leading to fragility of blood vessels, bowel and uterus that leads to spontaneous rupture. We report a previously undiagnosed vEDS patient with bowel complications. A 20-year-old female patient was referred to our hospital with abdominal pain. Computed tomography showed notable dilatation of the sigmoid colon with intraperitoneal fluid. Laparotomy revealed dilatation of the sigmoid colon, breakdown of serosa and muscularis propria of the sigmoid colon with impending perforation, and intra-abdominal hemorrhage caused by breakdown of the mesenterium. Resection of the sigmoid colon with Hartmann's pouch and an end colostomy were performed. Physical examination showed joint hypermobility, translucent skin with venous prominence and facial structure abnormalities. Genetic analysis using cDNA extracted from the patient's fibroblasts by reverse transcriptase polymerase chain reaction direct sequencing showed a missense mutation within the triple helix region of COL3A1 (c.2150 G>A; Gly717Asp.

  2. Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

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    Okada, Takuya, E-mail: okabone@gmail.com [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Interventional Radiology Department (France); Frank, Michael, E-mail: michael.frank@egp.aphp.fr [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Rare Vascular Diseases Reference Center (France); Pellerin, Olivier, E-mail: olivier@pellerin.as; Primio, Massimiliano Di, E-mail: massimiliano.di.primio@gmail.com; Angelopoulos, Georgios, E-mail: giorginos78@msn.com [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Interventional Radiology Department (France); Boughenou, Marie-Fazia, E-mail: marie-fazia.boughenou@egp.aphp.fr [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Anesthesia and Surgical Intensive Care Unit (France); Pagny, Jean-Yves, E-mail: jean-yves.pagny@egp.aphp.fr [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Interventional Radiology Department (France); Messas, Emmanuel, E-mail: emmanuel.messas@egp.aphp.fr [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Rare Vascular Diseases Reference Center (France); Sapoval, Marc, E-mail: marc.sapoval2@egp.aphp.fr [Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, Interventional Radiology Department (France)

    2013-05-09

    PurposeTo evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.MethodsWe retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.ResultsFive consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).ConclusionEmbolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.

  3. Blue Rubber Bleb Nevus Syndrome Showing Vascular Skin Lesions Predominantly on the Face

    Directory of Open Access Journals (Sweden)

    Ayumi Korekawa

    2015-07-01

    Full Text Available An 81-year-old Japanese man presented with dark blue papules and nodules on his face. There were multiple soft papules and nodules, dark blue in color, compressive, and ranging in size from 2 to 10 mm. A few similar lesions were seen on the patient's right dorsal second toe and right buccal mucosa. There were no skin lesions on his trunk and upper limbs. The patient's past history did not include gastrointestinal bleeding or anemia. Histopathological examination showed dilated vascular spaces lined by the normal epithelium extending beneath the dermis and into the subcutaneous fat. Endoscopy of the gastrointestinal tract to check for colon involvement was not performed. X-ray images of the limbs revealed no abnormalities in the bones or joints. Laboratory investigations did not show anemia. Although we failed to confirm a diagnosis by endoscopy, the skin lesions, histopathological findings, lack of abnormal X-ray findings, and the presence of oral lesions as a part of gastrointestinal tract guided the diagnosis of blue rubber bleb nevus syndrome (BRBNS. Skin lesions of BRBNS occur predominantly on the trunk and upper limbs. However, the present case showed multiple skin lesions predominantly on the face. Therefore, it is important for clinicians to know about a possible atypical distribution of skin lesions in BRBNS.

  4. Relationship between Blood Stasis Syndrome Score and Cardioankle Vascular Index in Stroke Patients

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    Ki-Ho Cho

    2012-01-01

    Full Text Available Blood stasis syndrome (BSS in traditional Asian medicine has been considered to correlate with the extent of atherosclerosis, which can be estimated using the cardioankle vascular index (CAVI. Here, the diagnostic utility of CAVI in predicting BSS was examined. The BSS scores and CAVI were measured in 140 stroke patients and evaluated with respect to stroke risk factors. Receiver operating characteristic (ROC curve analysis was used to determine the diagnostic accuracy of CAVI for the diagnosis of BSS. The BSS scores correlated significantly with CAVI, age, and systolic blood pressure (SBP. Multiple logistic regression analysis showed that CAVI was a significant associate factor for BSS (OR 1.55, P=0.032 after adjusting for the age and SBP. The ROC curve showed that CAVI and age provided moderate diagnostic accuracy for BSS (area under the ROC curve (AUC for CAVI, 0.703, P<0.001; AUC for age, 0.692, P=0.001. The AUC of the “CAVI+Age,” which was calculated by combining CAVI with age, showed better accuracy (0.759, P<0.0001 than those of CAVI or age. The present study suggests that the CAVI combined with age can clinically serve as an objective tool to diagnose BSS in stroke patients.

  5. Applied anatomical study of the vascularized ulnar nerve and its blood supply for cubital tunnel syndrome at the elbow region

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    Mei-xiu-li Li

    2015-01-01

    Full Text Available Cubital tunnel syndrome is often accompanied by paresthesia in ulnar nerve sites and hand muscle atrophy. When muscle weakness occurs, or after failure of more conservative treatments, anterior transposition is used. In the present study, the ulnar nerve and its blood vessels were examined in the elbows of 18 adult cadavers, and the external diameter of the nutrient vessels of the ulnar nerve at the point of origin, the distances between the origin of the vessels and the medial epicondyle of the humerus, and the length of the vessels accompanying the ulnar nerve in the superior ulnar collateral artery, the inferior ulnar collateral artery, and the posterior ulnar recurrent artery were measured. Anterior transposition of the vascularized ulnar nerve was performed to treat cubital tunnel syndrome. The most appropriate distance that the vascularized ulnar nerve can be moved to the subcutaneous tissue under tension-free conditions was 1.8 ± 0.6 cm (1.1-2.5 cm, which can be used as a reference value during the treatment of cubital tunnel syndrome with anterior transposition of the vascularized ulnar nerve.

  6. Applied anatomical study of the vascularized ulnar nerve and its blood supply for cubital tunnel syndrome at the elbow region.

    Science.gov (United States)

    Li, Mei-Xiu-Li; He, Qiong; Hu, Zhong-Lin; Chen, Sheng-Hua; Lv, Yun-Cheng; Liu, Zheng-Hai; Wen, Yong; Peng, Tian-Hong

    2015-01-01

    Cubital tunnel syndrome is often accompanied by paresthesia in ulnar nerve sites and hand muscle atrophy. When muscle weakness occurs, or after failure of more conservative treatments, anterior transposition is used. In the present study, the ulnar nerve and its blood vessels were examined in the elbows of 18 adult cadavers, and the external diameter of the nutrient vessels of the ulnar nerve at the point of origin, the distances between the origin of the vessels and the medial epicondyle of the humerus, and the length of the vessels accompanying the ulnar nerve in the superior ulnar collateral artery, the inferior ulnar collateral artery, and the posterior ulnar recurrent artery were measured. Anterior transposition of the vascularized ulnar nerve was performed to treat cubital tunnel syndrome. The most appropriate distance that the vascularized ulnar nerve can be moved to the subcutaneous tissue under tension-free conditions was 1.8 ± 0.6 cm (1.1-2.5 cm), which can be used as a reference value during the treatment of cubital tunnel syndrome with anterior transposition of the vascularized ulnar nerve.

  7. Liver fat percent is associated with metabolic risk factors and the metabolic syndrome in a high-risk vascular cohort

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    McHenery Christine

    2010-06-01

    Full Text Available Abstract Objective To determine whether liver fat percent (LFP is associated with the metabolic syndrome independently of visceral fat area (VFA. Methods 43 High-risk vascular patients not on lipid-lowering therapy were evaluated for the Adult Treatment Panel III (ATPIII metabolic syndrome criteria and underwent magnetic resonance imaging (MRI to quantify VFA and subcutaneous fat area (SFA at the L4-L5 disc and liver magnetic resonance spectroscopy (MRS to quantify LFP. Comparisons: 1. Baseline differences in patients with and without the metabolic syndrome 2. Forward binary logistic regression analysis of predictors of the metabolic syndrome with VFA, SFA and LFP as independents 3. Correlates of LFP. Results 43 patients were included in analysis. Patients with metabolic syndrome had greater VFA, SFA and LFP than patients without the metabolic syndrome (all p Conclusions LFP is associated with the metabolic syndrome and renders the current gold standard of VFA redundant in this analysis. This measure of obesity-related cardiovascular risk requires further validation and evaluation in a prospective cohort.

  8. Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration.

    Science.gov (United States)

    Busch, Albert; Hoffjan, Sabine; Bergmann, Frauke; Hartung, Birgit; Jung, Helena; Hanel, Daniela; Tzschach, Andeas; Kadar, Janos; von Kodolitsch, Yskert; Germer, Christoph-Thomas; Trobisch, Heiner; Strasser, Erwin; Wildenauer, René

    2016-08-03

    The vascular type represents a very rare, yet the clinically most fatal entity of Ehlers-Danlos syndrome (EDS). Patients are often admitted due to arterial bleedings and the friable tissue and the altered coagulation contribute to the challenge in treatment strategies. Until now there is little information about clotting characteristics that might influence hemostasis decisively and eventually worsen emergency situations. 22 vascular type EDS patients were studied for hemoglobin, platelet volume and count, Quick and activated partial thromboplastin time, fibrinogen, factor XIII, von Willebrand disease, vitamin D and platelet aggregation by modern standard laboratory methods. Results show a high prevalence of over 50 % for platelet aggregation disorders in vascular type EDS patients, especially for collagen and epinephrine induced tests, whereas the plasmatic cascade did not show any alterations. Additionally, more than half of the tested subjects showed low vitamin D serum levels, which might additionally affect vascular wall integrity. The presented data underline the importance of detailed laboratory screening methods in vascular type EDS patients in order to allow for targeted application of platelet-interacting substances that might be of decisive benefit in the emergency setting.

  9. Successful segmental thermal ablation of varicose saphenous veins in a patient with confirmed vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Frank, Michael; Says, Jerome; Denarié, Nicolas; Sapoval, Marc; Messas, Emmanuel

    2016-04-01

    We describe here the successful scheduled treatment of varicose veins by radiofrequency segmental thermal ablation in a 43-year-old patient with vascular Ehlers-Danlos syndrome. Her venous disease started at the age of 16 years, 1 year prior to her first major Ehlers-Danlos syndrome-related event which led to the diagnosis of her genetic condition. Surgical stripping was contra-indicated because of Ehlers-Danlos syndrome at the age of 18 years. More than 20 years later, her venous disease had become highly symptomatic despite daily compression and pain medication. Venous reassessment evidenced incompetent right and left great saphenous and left small saphenous veins, with increased diameters of both sapheno-femoral and sapheno-popliteal junctions. Radiofrequency endovenous ablation rather than surgery was considered because of its minimally invasive nature and because of standardized energy delivery.All intended-to-be-treated incompetent saphenous vein segments were occluded successfully, followed by an important improvement of clinical disease severity at day 30, persistent at 1 year post-treatment. Duplex ultrasound confirmed closure and fibrotic retraction of all treated venous segments at 1 year. This report shows that radiofrequency endovenous ablation may be a safe and effective therapy of varicose veins in patients with diagnosed vascular Ehlers-Danlos syndrome. © The Author(s) 2015.

  10. DASH-like diets high in protein or monounsaturated fats improve metabolic syndrome and calculated vascular risk.

    Science.gov (United States)

    Root, Martin M; Dawson, Hannah R

    2013-01-01

    Weight-loss diets with varying proportions of macronutrients have had varying effects on weight loss, and components of metabolic syndrome and risk factors for vascular diseases. However, little work has examined the effect of weight-neutral dietary changes in macronutrients on these factors. This is an investigation using the OMNI Heart datasets available from the NHLBI BioLINCC program. This study compared a DASH-like diet high in carbohydrates with similar diets high in protein and high in unsaturated fats. Measures of metabolic syndrome, except waist, and measures of risk factors for vascular diseases were taken at the end of each dietary period. All 3 diets significantly lowered the number of metabolic syndrome components (p ≤ 0.002) with a standardized measure of changes in metabolic syndrome components, suggesting that the high-protein, high-fat diet was most efficacious overall (p = 0.035). All 3 diets lowered a calculated 10-year risk of cardiovascular disease, with the high-protein and unsaturated fat diet being the most efficacious (p fat diet showed a slightly decreased calculated 9-year risk of diabetes (p = 0.11). Of the 3 weight-neutral diets, those high in protein and unsaturated fats appeared partially or wholly most beneficial.

  11. Circulating vascular progenitor cells and central arterial stiffness in polycystic ovary syndrome.

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    Cecile Dessapt-Baradez

    Full Text Available Subjects with Polycystic ovarian syndrome (PCOS are at increased risk of Type 2 diabetes mellitus (T2DM. The mechanism of this enhanced risk is unclear. Circulating vascular progenitor cells (VPC are immature bone marrow derived cells capable of differentiating into mature endothelial cells. VPC number/function and central arterial stiffness predict cardio-metabolic disease in at-risk populations.We studied VPC and arterial stiffness measures in non-obese PCOS subjects as compared to age and body mass index (BMI matched healthy controls in a cross-sectional study.Fourteen subjects with PCOS and 12 controls of similar age, BMI (all <30 kg/m(2 and metabolic profile were studied. VPC number and in vitro function were studied by flow cytometry and tube formation assays respectively. Augmentation index (AIx, a measure of central arterial stiffness, and central (aortic blood pressures (BP were measured by applanation tonometry.Subjects with PCOS had a reduced number, mean±SEM, of circulating CD34(+133(+ VPCs (317.5±51.0 vs. 558.3±101.2, p = 0.03 and impaired in vitro tube formation (completed tube area 1.0±0.06 vs. 1.2±0.05×10(6 µm(2 p = 0.02. PCOS subjects had significantly higher AIx (18.4±1.9% vs. 4.9±2.0% and this difference remained significant even after adjustments for age, BMI and smoking (p = 0.003 in multivariate analyses. Central systolic and pulse pressure were higher in PCOS subjects but these differences were not statistically significant after adjustment for age. Brachial systolic and pulse pressures were similar. VPC number/function and arterial stiffness or BP measures were not correlated.Non-obese PCOS is characterized by a reduced VPC number, impaired VPC function and increased central arterial stiffness. These changes in novel vascular risk markers may explain the enhanced risk of T2DM and CVD in PCOS.

  12. Open repair and venous inflow plication of the arteriovenous fistula is effective in treating vascular steal syndrome.

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    Patel, Mitul S; Davies, Mark G; Nassar, George M; Naoum, Joseph J

    2015-07-01

    Vascular steal syndrome related to a dialysis arteriovenous fistula (AVF) can lead to symptoms of distal ischemia, limb loss, digit ulceration, and gangrene. Several complex procedures have been used to augment and restore distal limb perfusion while maintaining a functional AVF. We reviewed our experience in treating AVF-related vascular steal syndrome by simple plication of the initial AVF inflow segment. Clinical data of 26 patients (15 men; mean age, 58 years; range, 26-80) with vascular steal syndrome related to their AVF underwent plication during a 36-month period. There were 18 brachial-cephalic AVFs and 8 brachial-basilic AVFs with vein transposition. Relevant clinical variables, imaging studies, and treatment variables were analyzed. Eighty-four percent of patients had hypertension, 62% were diabetics, and 15% had a previous limb or digit amputated. Hand pain, skin ulceration, or gangrene was present in 96%, 15%, and 12% of patients, respectively; 19% of patients had more than one symptom. Twelve (46%) patients had an aortic arch and upper extremity arteriogram, of which 67% showed evidence of arterial disease. One patient required percutaneous balloon-expandable stent treatment of a proximal left subclavian artery stenosis to improve flow. Duplex-derived volume flow measurements of the AVF were obtained with an average flow of 1.95 ± 0.83 L/min. Open repair and venous inflow plication was performed in all 26 patients. Average flow reduction in patients with preoperative and postoperative flow measurements was 0.6 ± 0.5 L/min (P functioning access out to 1 year. Two remaining patients who did not improve and proceeded to ligation of the AVF. Surgical plication of the initial AVF inflow segment offers a simple solution to preserve the dialysis access and resolve symptoms related to vascular steal associated with high volume flow through the AVF. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Utilization of Cupping Therapy in the Treatment of Vascular Thoracic Outlet Syndrome in a Collegiate Pitcher: A Case Study

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    Stephen A. Cage

    2017-12-01

    Full Text Available Objective: Present a clinical case detailing the effectiveness of dry cupping therapy in treating thoracic outlet syndrome. The utilization of dry cupping therapy on a 20-year-old collegiate baseball pitcher with diagnosed thoracic outlet syndrome is presented. Background: Thoracic outlet syndrome is a relatively rare musculoskeletal condition affecting 1/100,000 patients annually. Dry cupping therapy is an ancient therapeutic modality that utilizes various means of suction with the goal of decompressing myofascial layers. Treatment: Following diagnosis, patient was successfully treated in two weeks using dry cupping therapy. The patient experienced no further incidence of thoracic outlet syndrome symptoms and was able to complete the remainder of his competitive season. Uniqueness: The patient’s thoracic outlet syndrome was diagnosed at an early stage, leading to the need of clearance from a vascular specialist before returning to competition. To the author’s knowledge, there are currently no published case reports detailing the use of cupping therapy to treat thoracic outlet syndrome. Conclusion: Cupping therapy may be a viable treatment option when seeking to address tight musculature. Further research needs to be conducted to determine optimal parameters for cupping therapy as a therapeutic modality.

  14. Six uneventful pregnancy outcomes in an extended vascular Ehlers-Danlos syndrome family.

    Science.gov (United States)

    Baas, Annette F; Spiering, Wilko; Moll, Frans L; Page-Christiaens, Lieve; Beenakkers, Ingrid C M; Dooijes, Dennis; Vonken, Evert-Jan P A; van der Smagt, Jasper J; Knoers, Nine V; Koenen, Steven V; van Herwaarden, Joost A; Sieswerda, Gertjan Tj

    2017-02-01

    Vascular Ehlers-Danlos Syndrome (vEDS) is caused by heterozygous mutations in COL3A1 and is characterized by fragile vasculature and hollow organs, with a high risk of catastrophic events at a young age. During pregnancy and delivery, maternal mortality rates up until 25% have been reported. However, recent pedigree analysis reported a substantial lower pregnancy-related mortality rate of 4.9%. Here, we describe an extended vEDS family with multiple uneventful pregnancy outcomes. In the proband, a 37-year-old woman, DNA-analysis because of an asymptomatic iliac artery dissection revealed a pathogenic mutation in COL3A1 (c.980G>A; p. Gly327Asp). She had had three uneventful vaginal deliveries. At the time of diagnosis, her 33-year-old niece was 25 weeks pregnant. She had had one uneventful vaginal delivery. Targeted DNA-analysis revealed that she was carrier of the COL3A1 mutation. Ultrasound detected an aneurysm in the abdominal aorta with likely a dissection. An uneventful elective cesarean section was performed at a gestational age of 37 weeks. The 40-year-old sister of our proband had had one uneventful vaginal delivery and an active pregnancy wish. Cascade DNA-screening showed her to carry the COL3A1 mutation. Computed Tomography Angiography (CTA) of her aorta revealed a type B dissection with the most proximal entry tear just below the superior mesenteric artery. Pregnancy was therefore discouraged. This familial case illustrates the complexity and challenges of reproductive decision-making in a potentially lethal condition as vEDS, and highlights the importance of a multidisciplinary approach. Moreover, it suggests that previous pregnancy-related risks of vEDS may be overestimated. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Impact of a lifestyle program on vascular insulin resistance in metabolic syndrome subjects: the RESOLVE study.

    Science.gov (United States)

    Vinet, Agnes; Obert, Philippe; Dutheil, Frederic; Diagne, Lamine; Chapier, Robert; Lesourd, Bruno; Courteix, Daniel; Walther, Guillaume

    2015-02-01

    Impaired insulin-dependent vasodilation might contribute to microvascular dysfunction of metabolic syndrome (MetS). The aims of this study were to assess the insulin vasoreactivity in MetS, and to evaluate the effects of a lifestyle program. DESIGN, SETTING, PARTICIPANTS, AND OUTCOME MEASURES: Laser Doppler measurements were used to assess cutaneous blood flux (CBF) and flowmotion in response to iontophoresis of insulin and acetylcholine (ACh) in 38 MetS and 18 controls. Anthropometric, plasma insulin, glycemia, and inflammatory markers were measured. MetS subjects (n = 24) underwent a 6-month lifestyle intervention (M6) with a 3-week residential program (D21). The absolute and relative peak insulin and ACh CBF were significantly higher in controls than in MetS subjects. Significant inverse correlations were found between peak insulin CBF and glycemia, insulin and glycated hemoglobin, active plasminogen activator inhibitor-1 (PAI-1), C-reactive protein (CRP), and IL-6. With respect to flowmotion, MetS subjects showed lower values in total spectrum CBF and in all its components (except respiratory one). At D21 and M6, peak insulin CBF increased and was no longer different from control values whereas peak ACh CBF did not change. From D21, all the different components and the total CBF spectrum became similar to the control values. The changes in peak insulin CBF and in endothelial component between M6 and baseline were inversely correlated with the change in CRP and PAI-1. The local vasodilatory effects to insulin and its overall flowmotion are impaired in MetS subjects in relation to inflammation. The lifestyle intervention reversed this insulin-induced vascular dysfunction in parallel to decreased inflammation level.

  16. Vascular Vertigo

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    Mazyar Hashemilar

    2017-02-01

    Full Text Available Vertigo is a common complaint in neurology and medicine. The most common causes of vertigo are benign paroxysmal positional vertigo, vestibular neuritis, Meniere’s disease, and vascular disorders. Vertigo of vascular origin is usually limited to migraine, transient ischemic attacks, and ischemic or hemorrhagic stroke. Vascular causes lead to various central or peripheral vestibular syndromes with vertigo. This review provides an overview of epidemiology and clinical syndromes of vascular vertigo. Vertigo is an illusion of movement caused by asymmetrical involvement of the vestibular system by various causes. Migraine is the most frequent vascular disorder that causes vertigo in all age groups. Vertigo may occur in up to 25% of patients with migraine. The lifetime prevalence of migrainous vertigo is almost 1%. Cerebrovascular disorders are estimated to account for 3% to 7% of patients with vertigo. Vestibular paroxysmia has been diagnosed in 1.8% to 4% of cases in various dizziness units. Vasculitic disorders are rare in the general population, but vertigo may be seen in almost up to 50% of patients with different vasculitic syndromes. Conclusions: Migraine, cerebrovascular disorders especially involving the vertebrobasilar territory, cardiocirculatory diseases, neurovascular compression of the eighth nerve, and vasculitis are vascular causes of vertigo syndromes.

  17. Methanol extract of Sorbus commixta cortex prevents vascular inflammation in rats with a high fructose-induced metabolic syndrome.

    Science.gov (United States)

    Kang, Dae Gill; Sohn, Eun Jin; Lee, An Sook; Kim, Jin Sook; Lee, Dae Ho; Lee, Ho Sub

    2007-01-01

    Feeding high fructose (Frc) to rats induces a moderate increase in blood pressure, which is associated with insulin resistance. The present study was designed to evaluate the effect of the methanol extract of Sorbus commixta cortex (MSC) on vascular inflammation in a rat model of the metabolic syndrome induced by a high Frc-diet. Male Sprague-Dawley rats were divided into 4 groups and treated for 7 weeks as follows: 1) control, 2) high Frc-diet group, 3) Frc/MSC1 group; high Frc-diet group treated with MSC (100 mg/kg/day), and 4) Frc/MSC2 group; high Frc-diet group treated with MSC (200 mg/kg/day). High Frc-induced decreases of the expression level of aortic endothelial nitric oxide synthase (ecNOS) while the production of cyclic GMP (cGMP) was restored by treatment with MSC. On the contrary, increases of the expression level of endothelin-1 (ET-1) in the aorta, the transcription factor, the cytokine related with vascular inflammation, and the adhesion molecules were suppressed by MSC treatment. Moreover, MSC treatment was shown to lessen the thickening noted in the aortic intima and media of the high Frc-diet group. Our findings suggest that MSC may have an anti-vascular inflammatory effect on rats with a high Frc-induced metabolic syndrome.

  18. Epidermal nevus syndrome and dysplatic kidney disease.

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    Azar Nickavar

    2014-08-01

    Full Text Available Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this association, it is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders.

  19. Metabolic syndrome and incidence of type 2 diabetes in patients with manifest vascular disease

    NARCIS (Netherlands)

    Wassink, A.M.J.; Graaf, van der Y.; Soedamah-Muthu, S.S.; Spiering, W.; Visseren, F.L.J.

    2008-01-01

    Risk reduction in patients with clinically manifest vascular disease focuses on preventing new vascular events and not on prevention of type 2 diabetes. However, given the common pathophysiological pathways involved in the development of atherosclerosis and type 2 diabetes, it is probable that

  20. Next-generation sequencing and a novel COL3A1 mutation associated with vascular Ehlers-Danlos syndrome with severe intestinal involvement: a case report.

    Science.gov (United States)

    Cortini, Francesca; Marinelli, Barbara; Seia, Manuela; De Giorgio, Barbara; Pesatori, Angela Cecilia; Montano, Nicola; Bassotti, Alessandra

    2016-10-31

    The vascular type of Ehlers-Danlos syndrome is an autosomal dominant connective tissue disorder caused by a mutation in the COL3A1 gene encoding pro-alpha1 chain of type III collagen. The vascular type of Ehlers-Danlos syndrome causes severe fragility of connective tissues with arterial and intestinal ruptures and complications in surgical and radiological treatments. We present a case of a 38-year-old Italian woman who was diagnosed as having the vascular type of Ehlers-Danlos syndrome. Genetic testing, conducted by Target Enrichment approach (Agilent Technologies), identified a new mutation c.1493G>A, p.G498D in exon 21 of COL3A1 gene (heterozygous state). This mutation disrupts the normal glycine-X-Y repetitions of type III procollagen by converting glycine to aspartic acid. We report a new genetic mutation associated with the vascular type of Ehlers-Danlos syndrome. We also describe clinical and genetic findings that are important to understand the genotype/phenotype correlation in patients with the vascular type of Ehlers-Danlos syndrome.

  1. An Isolated Pulmonary Hematoma Mimicking a Lung Tumor as the Initial Finding of Vascular Ehlers-Danlos Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Ju; Lee, Ki Nam; Choi, Pil Jo [Dept. of Radiology, Dong-A University Medicine Center, Dong-A University College of Medicine, Busan (Korea, Republic of); Ki, Chang Seok [Dept. of Radiology, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2012-09-15

    The vascular type of Ehlers-Danlos syndrome (vEDS) is an uncommon inherited disorder characterized by abnormalities in type III collagen, presenting itself as arterial dissection or rupture. We report a case of an isolated pulmonary hematoma mimicking a lung tumor in an 18-year-old man which turned out to be the initial finding of vEDS. Pneumothorax and hemothorax occurred repeatedly for 15 months following the surgical removal of the mass, and were treated by repeated left upper and lower lobectomy and thoracotomy. The diagnosis of vEDS was confirmed by pathologic and genetic studies.

  2. Sustained release nitrite therapy results in myocardial protection in a porcine model of metabolic syndrome with peripheral vascular disease.

    Science.gov (United States)

    Bradley, Jessica M; Islam, Kazi N; Polhemus, David J; Donnarumma, Erminia; Brewster, Luke P; Tao, Ya-Xiong; Goodchild, Traci T; Lefer, David J

    2015-07-15

    Metabolic syndrome (MetS) reduces endothelial nitric oxide (NO) bioavailability and exacerbates vascular dysfunction in patients with preexisting vascular diseases. Nitrite, a storage form of NO, can mediate vascular function during pathological conditions when endogenous NO is reduced. The aims of the present study were to characterize the effects of severe MetS and obesity on dyslipidemia, myocardial oxidative stress, and endothelial NO synthase (eNOS) regulation in the obese Ossabaw swine (OS) model and to examine the effects of a novel, sustained-release formulation of sodium nitrite (SR-nitrite) on coronary vascular reactivity and myocardial redox status in obese OS subjected to critical limb ischemia (CLI). After 6 mo of an atherogenic diet, obese OS displayed a MetS phenotype. Obese OS had decreased eNOS functionality and NO bioavailability. In addition, obese OS exhibited increased oxidative stress and a significant reduction in antioxidant enzymes. The efficacy of SR-nitrite therapy was examined in obese OS subjected to CLI. After 3 wk of treatment, SR-nitrite (80 mg · kg(-1) · day(-1) bid po) increased myocardial nitrite levels and eNOS function. Treatment with SR-nitrite reduced myocardial oxidative stress while increasing myocardial antioxidant capacity. Ex vivo assessment of vascular reactivity of left anterior descending coronary artery segments demonstrated marked improvement in vasoreactivity to sodium nitroprusside but not to substance P and bradykinin in SR-nitrite-treated animals compared with placebo-treated animals. In conclusion, in a clinically relevant, large-animal model of MetS and CLI, treatment with SR-nitrite enhanced myocardial NO bioavailability, attenuated oxidative stress, and improved ex vivo coronary artery vasorelaxation.

  3. [Correlation of severity classification of acute respiratory distress syndrome by the Berlin definition with extra vascular lung water index and pulmonary vascular permeability index].

    Science.gov (United States)

    Zhu, Jinyuan; Wang, Xiaohong; Yang, Xiaojun; Wang, Xiaoqi; Ma, Xigang

    2015-05-19

    To explore the correlation of severity classification of acute respiratory distress syndrome (ARDS) by the Berlin definition with extra vascular lung water index (EVLWI) and pulmonary vascular permeability index (PVPI). A total of 70 cases with ARDS at intensive care unit of our hospital from July 2012 to July 2014 were divided into three groups of mild (n = 20), moderate (n = 30) and severe (n = 20) according to the Berlin definition. The scores of acute physiology and chronic health evaluation (APACHE) II and sequential organ failure assessment (SOFA) within 24 h of admission were recorded. And the values of EVLWI and PVPI of three groups from Day 1-4 were monitored by pulse indicator continuous cardiac output (PiCCO). Receiver operating characteristic (ROC) curve was drawn for these parameters and the area under curve was compared. Meanwhile blood gas was analyzed and oxygenation index (OI) calculated. And the correlations of EVLWI and PVPI with OI were analyzed. Comparisons of EVLWI, PVPI and OI were made for three groups at different timepoints: As the severity of ARDS aggravated, EVLWI and PVPI of three groups increased significantly at any timepoint while OI decreased sharply (P 0.05). There was no sharp decline of EVLWI or PVPI in severe ARDS group (P > 0.05). And OI increased significantly from Day 1-4 in three groups (P 2.95 at Day 4 of admission was used as the best threshold value for judging prognosis. And the sensitivity was 70% and specificity 92%. OI had negative correlation with EVLWI and PVPI in three groups from Day 1-4 [(r = -0.685, P = 0.000) and (r = -0.631, P = 0.000)]. Both EVLWI and PVPI reflect adequately the severity of ARDS by the Berlin definition. And the dynamic trend of PVPI is superior to that of EVLWI.

  4. Twenty-seven years follow-up of a patient with congenital retinocephalofacial vascular malformation syndrome and additional congenital malformations (Bonnet-dechaume-blanc syndrome or wyburn-mason syndrome

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    Schmidt D

    2010-02-01

    Full Text Available Abstract Purpose Follow-up of vascular changes in a patient with congenital retinocephalofacial vascular malformation syndrome. Methods MRI and cerebral angiography. Results In a 36-year-old man, magnetic resonance im aging of the skull and cerebral angiography revealed left intracranial arteriovenous malformations. Follow-up observation of 27 years revealed no essential change of retinal and cerebral arteriovenous malformations. Additional congenital deficits in this patient were described. Conclusion Patients with retinal arteriovenous malformations should be early examined with neuroradiological methods.

  5. A síndrome ombro-mão nas hemiplegias vasculares Shoulder-hand syndrome in cerebrovascular hemiplegia

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    Sebastião E. Melo Souza

    1968-09-01

    Full Text Available São expostos os dados obtidos em 18 casos de síndrome ombro-mão, encontrados mediante revisão de 175 pacientes acometidos de afecção cerebro-vascular aguda. Em todos os casos a síndrome se instalou no lado paralisado, evidenciando a importância da imobilização. O início se deu, na maioria das vezes, por edema de mão, aparecendo dentro de duas a três semanas após o icto. Os resultados eletromiográficos obtidos em 7 casos, são comentados. O tratamento de maior êxito se deve à cinesiterapia, podendo-se associar a griseofulvina, útil no alívio da dor.From 175 cases of cerebrovascular disease, 18 patients with shoulder-hand syndrome are studied. In all patients the syndrome involved the paralysed limb, a fact that points to immobility as a enhancing factor. Hand edema occurring two to three weeks after the stroke was the first sign of the syndrome. Eletromyographic data were evaluated in 7 patients. Griseofulvine (for pain and kinesiotherapy are the best available tratment.

  6. The Open Lung Approach Improves Pulmonary Vascular Mechanics in an Experimental Model of Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Santos, Arnoldo; Lucchetta, Luca; Monge-Garcia, M Ignacio; Borges, Joao Batista; Tusman, Gerardo; Hedenstierna, Goran; Larsson, Anders; Suarez-Sipmann, Fernando

    2017-03-01

    To test whether positive end-expiratory pressure consistent with an open lung approach improves pulmonary vascular mechanics compared with higher or lower positive end-expiratory pressures in experimental acute respiratory distress syndrome. Experimental study. Animal research laboratory. Ten pigs, 35 ± 5.2 kg. Acute respiratory distress syndrome was induced combining saline lung lavages with injurious mechanical ventilation. The positive end-expiratory pressure level resulting in highest compliance during a decremental positive end-expiratory pressure trial after lung recruitment was determined. Thereafter, three positive end-expiratory pressure levels were applied in a random order: hyperinflation, 6 cm H2O above; open lung approach, 2 cm H2O above; and collapse, 6 cm H2O below the highest compliance level. High fidelity pressure and flow sensors were placed at the main pulmonary artery for measuring pulmonary artery resistance (Z0), effective arterial elastance, compliance, and reflected pressure waves. After inducing acute respiratory distress syndrome, Z0 and effective arterial elastance increased (from 218 ± 94 to 444 ± 115 dyn.s.cm and from 0.27 ± 0.14 to 0.62 ± 0.22 mm Hg/mL, respectively; p mechanics compared with higher or lower positive end-expiratory pressure settings.

  7. Cervical vascular and upper airway asymmetry in Velo-cardio-facial syndrome: correlation of nasopharyngoscopy with MRA.

    Science.gov (United States)

    Oppenheimer, Avi G; Fulmer, Susan; Shifteh, Keivan; Chang, Ja-Kwei; Brook, Allan; Shanske, Alan L; Shprintzen, Robert J

    2010-06-01

    Velo-cardio-facial syndrome (VCFS), the most common genetic syndrome causing cleft palate, is associated with internal carotid and vertebral artery anomalies, as well as upper airway asymmetry. Medially displaced internal carotid arteries, often immediately submucosal, present a risk of vascular injury during pharyngeal flap surgery for velopharyngeal insufficiency (VPI). We evaluate the frequency and spectrum of cervical vascular anomalies in a large cohort of VCFS patients correlating MRA with nasopharyngolaryngoscopy in detecting at risk carotid arteries. Furthermore, we assess the relationship with respect to laterality between cervical vascular patterns and the asymmetric abnormalities of these subjects' upper airways. Cervical MRAs of 86 subjects with VCFS and 50 control subjects were independently reviewed by three neuroradiologists. The course of the internal carotid and vertebral arteries was identified within the pharyngeal soft tissues. Medial deviation, level of bifurcation, dominance, anomalous origin, and vessel tortuosity were recorded. Nasopharyngoscopy examinations were available for retrospective review in 43 patients and were assessed for palatal and posterior pharyngeal wall symmetry, true vocal cord motion and size, and for the presence or absence of carotid pulsations. The endoscopic findings were compared with MRA results. Of the 86 subjects, 80 (93%) had one or more vascular anomalies. 42 subjects (49%) were found to have medial deviation of at least one internal carotid artery. In 24 subjects (28%) the anomalous internal carotid artery was directly submucosal; four of these were bilateral (5% of the total sample, 17% of those with a submucosal internal carotid). Other carotid anomalies included low carotid bifurcation (44 subjects or 51%), anomalous origin of the right common carotid (32 cases, or 37%), and two cases of internal carotid agenesis/hypoplasia. Vertebral artery anomalies included vessel tortuosity (34 cases, or 40%), hypoplasia

  8. Vascular affection in relation to oxidative DNA damage in metabolic syndrome.

    Science.gov (United States)

    Abd El Aziz, Rokayaa; Fawzy, Mary Wadie; Khalil, Noha; Abdel Atty, Sahar; Sabra, Zainab

    2018-02-01

    Obesity has become an important issue affecting both males and females. Obesity is now regarded as an independent risk factor for atherosclerosis-related diseases. Metabolic syndrome is associated with increased risk for development of cardiovascular disease. Urinary 8-oxo-7,8-dihydro-2'-deoxyguanosine concentration has been used to express oxidation status. Twenty-seven obese patients with metabolic syndrome, 25 obese patients without metabolic syndrome and 31 healthy subjects were included in our study. They were subjected to full history and clinical examination; fasting blood sugar (FBS), 2 hour post prandial blood sugar (2HPP), lipid profile, urinary 8-oxo-7,8-dihydro-2'-deoxyguanosine and carotid duplex, A/B index and tibial diameters were all assessed. There was a statistically significant difference ( p = 0.027) in diameter of the right anterior tibial artery among the studied groups, with decreased diameter of the right anterior tibial artery in obese patients with metabolic syndrome compared to those without metabolic syndrome; the ankle brachial index revealed a lower index in obese patients with metabolic syndrome compared to those without metabolic syndrome. There was a statistically insignificant difference ( p = 0.668) in the 8-oxodG in the studied groups. In obese patients with metabolic syndrome there was a positive correlation between 8-oxodG and total cholesterol and LDL. Urinary 8-oxodG is correlated to total cholesterol and LDL in obese patients with metabolic syndrome; signifying its role in the mechanism of dyslipidemia in those patients. Our study highlights the importance of anterior tibial artery diameter measurement and ankle brachial index as an early marker of atherosclerosis, and how it may be an earlier marker than carotid intima-media thickness.

  9. Vascular type Ehlers-Danlos Syndrome with fatal spontaneous rupture of a right common iliac artery dissection: case report and review of literature.

    Science.gov (United States)

    Abayazeed, Aly; Hayman, Emily; Moghadamfalahi, Mana; Cain, Darren

    2014-02-01

    Vascular Ehlers-Danlos Syndrome (previously Ehlers-Danlos IV) is a rare autosomal dominant collagen vascular disorder caused by a 2q31 COL3A1 gene mutation encoding pro-alpha1 chain of type III collagen (in contrast to classic Ehlers-Danlos, caused by a COL5A1 mutation). The vascular type accounts for less than 4% of all Ehlers-Danlos cases and usually has a poor prognosis due to life threatening vascular ruptures and difficult, frequently unsuccessful surgical and vascular interventions. In 70% of cases, vascular rupture or dissection, gastrointestinal perforation, or organ rupture is a presenting sign. We present a case of genetically proven vascular Ehlers-Danlos with fatal recurrent retroperitoneal hemorrhages secondary to a ruptured right common iliac artery dissection in a 30-year-old male. This case highlights the need to suspect collagen vascular disorders when a young adult presents with unexplained retroperitoneal hemorrhage, even without family history of such diseases.

  10. Atypical Presentation of Sjögren-Larsson Syndrome

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    D. Papathemeli

    2017-01-01

    Full Text Available Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95. This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if intelligence is normal.

  11. Angiogenesis Research to Improve Therapies for Vascular Leak Syndromes, Intra-abdominal Adhesions, and Arterial Injuries

    Science.gov (United States)

    2008-04-01

    myelofibrosis, myelodysplastic syndrome, glioblastoma, ocular melanoma, AML, mantle- cell lymphoma, Waldenstrom’s macroglobinaemia, ovarian...glioma, skin cancer prevention, basal cell nevus syndrome, Barrett’s oesophagus, hepatocellular, oesophogael, prostate, cervical, colorectal, head...VEGFR, RTK, FLT3, TIE2 • Phase I: Solid tumours XL880 (Exelixis) C-met, RTK • Phase I: Solid tumours • Phase II: Papillary renal cell carcinoma XL999

  12. Coronary vascular disease event risk and metabolic syndrome prevalence in patients enrolled in an assertive treatment community program.

    Science.gov (United States)

    Ramudo Cela, Luis; Ávila González, María José; Yáñez Rubal, Juan Carlos; Díaz Platas, Lucía María; Martín Herranz, María Isabel; Díaz Del Valle, Juan Carlos

    2017-11-02

    To examine the risk of coronary vascular disease event (CVDE) and the prevalence metabolic syndrome (MS) and its cardiovascular risk factors (CVRF) in patients with severe mental illnesses enrolled in an assertive treatment community program (ATC) in Spain. We carried out a cross-sectional descriptive study with all of the patients included in an ATC program in 2016 in a health area with 547,328 inhabitants in Galicia, Spain. We identified the CVRF in all the individuals, and calculated MS and 10-year CVDE. We also compared the prevalence of all traits in our cohort and the general population. The 10-year median of coronary vascular disease event (CVDE10) was 8.4%. The percentage of individuals with high CVDE10 (>5%) was 41.2% The CVDE10 median was higher in men than women (10.5% vs 5.1%, penrolled in ATC programs had a 1.5-times higher prevalence of MS and 8 times higher CVDE10 than those reported in the general population. Individual CVRF were also higher in the SMD patients. Prevention, early detection, and comprehensive treatment are important issues for patients with severe mental illnesses. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. A Novel Approach for Reconstruction of Finger Neurocutaneous Defect: A Sensory Reverse Dorsal Digital Artery Flap from the Neighboring Digit.

    Science.gov (United States)

    Feng, Shi-Ming; Sun, Qing-Qing; Cheng, Jian; Wang, Ai-Guo

    2017-11-01

    Providing soft tissue coverage for finger neurocutaneous defects presents aesthetic and sensory challenges. A common source for reconstruction of soft tissue defects of the fingers is the same finger. However, when the donor areas are damaged by concomitant injuries, this option is not available. The present study aims to reconstruct finger neurocutaneous defects using a sensory reverse dorsal digital artery flap from the neighboring digit and to evaluate the efficacy of this technique. The study included 16 patients, with an average age of 34.9 years (range, 20-53 years) at the time of surgery, from May 2010 to June 2013. The sensory reverse dorsal digital artery flap was used in all 16 patients, who had a combination of soft tissue and digital nerve defects. The mean size of the soft tissue defects was 3.1 cm × 2.0 cm, and the mean flap size was 3.3 cm × 2.2 cm. The length of the nerve defects ranged from 1.3 to 2.5 cm (mean, 2.0 cm), which were reconstructed with dorsal branches of the proper digital nerve transfer. The active motion of the fingers (injured and donor) and the flap sensibility (static two-point discrimination) were measured. The appearance and functional recovery of the injured finger and the donor site were assessed using the Michigan Hand Outcomes Questionnaire. All flaps survived completely. No complications were reported, and no further flap debulking procedure was required. At the mean follow-up period of 24 months (range, 18-30 months), the mean static two-point discrimination was 6.5 mm (range, 5-10 mm) of the reconstructed area; the mean ranges of motions of the injured finger and the opposite finger at the proximal interphalangeal and distal interphalangeal joints were 102.2° and 103.5°, and 70.3° and 76.5°, respectively. The average ranges of motions of the metacarpophalangeal and proximal interphalangeal joints of the donor fingers were 90° and 103.4°, respectively. Based on the Michigan Hand Outcomes Questionnaire, 10 patients

  14. PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients

    Energy Technology Data Exchange (ETDEWEB)

    Bracken, Jennifer; Robinson, Ian; Snow, Aisling; Rea, David; Phelan, Ethna [Our Lady' s Children' s Hospital, Department of Radiology, Dublin (Ireland); Watson, Rosemarie; Irvine, Alan D. [Our Lady' s Children' s Hospital, Department of Dermatology, Dublin (Ireland)

    2011-09-15

    PHACE (posterior fossa defects, haemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities) syndrome describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma. To report the spectrum of clinical and imaging abnormalities seen in a cohort of children. A retrospective review of the clinical and imaging records of all patients diagnosed with PHACE syndrome between 1998 and 2009 was performed. Information sought included patient demographics, craniofacial segments involved, imaging findings and other extracutaneous abnormalities. Twelve patients were diagnosed with PHACE syndrome over 11 years. All patients had a segmental craniofacial haemangioma. Involved facial segments, in order of frequency, were frontotemporal (12), maxillary (8), mandibular (5) and frontonasal (1). The most common extracutaneous abnormalities were neurovascular anomalies (10), with many patients having multiple anomalies. The spectrum of arterial anomalies ranged from hypoplasia (9) to ectasia (3), anomalous origin/course (2) and persistent fetal anastomosis (2). Other anomalies found included cardiac anomalies (3), coarctation of the aorta (2), posterior fossa malformations (1) and sternal region anomalies (1). Intracranial anomalies are the most common extracutaneous feature of PHACE syndrome. The contribution of the radiologist in the recognition of such anomalies is important for the diagnosis of PHACE syndrome. (orig.)

  15. PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients.

    LENUS (Irish Health Repository)

    Bracken, Jennifer

    2012-02-01

    BACKGROUND: PHACE (posterior fossa defects, haemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities) syndrome describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma. OBJECTIVE: To report the spectrum of clinical and imaging abnormalities seen in a cohort of children. MATERIALS AND METHODS: A retrospective review of the clinical and imaging records of all patients diagnosed with PHACE syndrome between 1998 and 2009 was performed. Information sought included patient demographics, craniofacial segments involved, imaging findings and other extracutaneous abnormalities. RESULTS: Twelve patients were diagnosed with PHACE syndrome over 11 years. All patients had a segmental craniofacial haemangioma. Involved facial segments, in order of frequency, were frontotemporal (12), maxillary (8), mandibular (5) and frontonasal (1). The most common extracutaneous abnormalities were neurovascular anomalies (10), with many patients having multiple anomalies. The spectrum of arterial anomalies ranged from hypoplasia (9) to ectasia (3), anomalous origin\\/course (2) and persistent fetal anastomosis (2). Other anomalies found included cardiac anomalies (3), coarctation of the aorta (2), posterior fossa malformations (1) and sternal region anomalies (1). CONCLUSION: Intracranial anomalies are the most common extracutaneous feature of PHACE syndrome. The contribution of the radiologist in the recognition of such anomalies is important for the diagnosis of PHACE syndrome.

  16. Vascular thoracic outlet syndrome. Longer posterior rib stump causes poor outcome

    NARCIS (Netherlands)

    Geven, Leontien I.; Smit, Andries J.; Ebels, Tjark

    Objective: To assess the role of the relative length of the posterior rib stump in outcome after transaxittary first rib resection for thoracic outlet syndrome. Methods: All patients with a transaxittary first rib resection between January 1990 and February 2004 were selected. Relative rib stump

  17. [Antiphospholipid syndrome in valvular heart diseases, ischemic heart disease and vascular thrombosis].

    Science.gov (United States)

    Grabowski, M; Brzezińska, A

    2000-01-01

    The antiphospholipid syndrome (APS) leads to venous and arterial thrombosis, cardiac diseases, neurological, gastroenterological and dermatological complications. The role of antiphospholipid antibodies in genesis of thrombi by interaction with plasma clotting factors is well known. There is no evidence of their influence on valvular heart diseases or atherogenesis. This paper presents views and opinions about APS and related cardiovascular complications.

  18. [Behcet syndrome: thirty comments with lung and vascular injury of peripheral vessels].

    Science.gov (United States)

    Sekkach, Youssef; Elomri, Naoual; Jira, Mohamed; Elqatni, Mohamed; Fatihi, Jamal; Mekouar, Fadwa; Smaali, Jihane; Badaoui, Mohamed; Hammi, Salaheddine; Amezyane, Taoufik; Abouzahir, Ali; Khattabi, Abdessadek El; Ghafir, Driss

    2012-02-01

    Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations. We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required). Data were retrieved and analysed with two softwares (Access(®) and Epi Info(®)). We observed 30 cases with vascular lesions on a series of 92 patients with Behcet's disease. Most patients were male, with an average age around 40. The venous manifestations, concerning essentially the lower limbs (deep and superficial thrombosis) were found at 27 patients (90 %), and the average of age during the appearance of the venous lesions was 40 years. Arterial lesions appear more late in 13 patients (43 %) (average of age 43 years). We noted, on the other hand, 11 cases of aneurysms and five cases of arterial thrombosis. The use of corticosteroids was necessary in all cases in association with the others drugs (anticoagulants, colchicine, immunosuppressors). Among the patients having had aneurysms, six were treated surgically. The outcome was favorable for most patients. Two patients had pulmonary embolism and two post-surgery complications. One patient died in the consequences of an intragastric break of an aneurysm of the abdominal aorta. The vascular involvement in Behcet's disease is manifested primarily by thrombophlebitis. Achieving blood pressure, less common, is problematic therapeutic because of the recurrent and life threatening. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  19. Systemic vascular phenotypes of Loeys-Dietz syndrome in a child carrying a de novo R381P mutation in TGFBR2: a case report.

    Science.gov (United States)

    Uike, Kiyoshi; Matsushita, Yuki; Sakai, Yasunari; Togao, Osamu; Nagao, Michinobu; Ishizaki, Yoshito; Nagata, Hazumu; Yamamura, Kenichiro; Torisu, Hiroyuki; Hara, Toshiro

    2013-11-12

    Loeys-Dietz syndrome, also known as Marfan syndrome type II, is a rare connective tissue disorder caused by dominant mutations in transforming growth factor-beta receptors (TGFBR1 and 2). We report a 7-year-old Japanese boy with Loeys-Dietz syndrome who carried a novel, de novo missense mutation in TGFBR2 (c.1142g > c, R381P). He showed dysmorphic faces and skeletal malformations that were typical in previous cases with Loeys-Dietz syndrome. The cardiac studies disclosed the presence of markedly dilated aortic root and patent ductus aorteriosus. The cranial magnetic resonance imaging (MRI) and angiography (MRA) detected the tortuous appearances of the bilateral middle cerebral and carotid arteries. This study depicts the systemic vascular phenotypes of a child with Loeys-Dietz syndrome that were caused by a novel heterozygous mutation of TGFR2. A large cohort with serial imaging studies for vascular phenotypes will be useful for delineating the genotype-phenotype correlations of Loeys-Dietz syndrome.

  20. Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    B. Routy

    2013-01-01

    Full Text Available Catastrophic antiphospholipid syndrome (CAPS is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.

  1. Inhibition of Secretory Phospholipase A(2) in Patients with Acute Coronary Syndromes: Rationale and Design of the Vascular Inflammation Suppression to Treat Acute Coronary Syndrome for 16 Weeks (VISTA-16) Trial

    NARCIS (Netherlands)

    Nicholls, Stephen J.; Cavender, Matthew A.; Kastelein, John J. P.; Schwartz, Gregory; Waters, David D.; Rosenson, Robert S.; Bash, Dianna; Hislop, Colin

    2012-01-01

    Background The action of secretory phospholipase A(2) (sPLA(2)) on lipoproteins may render them more susceptible to oxidation, thereby promoting vascular inflammation and increasing cardiovascular risk. Patients with acute coronary syndrome face a high risk of early, recurrent cardiovascular events

  2. Angiogenesis Research to Improve Therapies for Vascular Leak Syndromes, Intra-Abdominal Adhesions, and Arterial Injuries

    Science.gov (United States)

    2009-04-01

    syndrome in a murine model. Fertil Steril . 2008 Oct 18. 9 3. Fainaru O, Adini I, Benny O, Bazinet L, Pravda E, D’Amato R, Folkman J. Doxycycline...cause of morbidity and mortality in surgical patients. They are the number one cause of bowel obstruction and infertility , and a major source of...adhesion formation in a murine model. Fertil Steril 61: 1136-1140. 2. Rodgers KE, Johns DB, Girgis W, Campeau J, diZerega GS (1997) Reduction of

  3. Neuropsychological syndromes associated with Alzheimer's/vascular dementia: a latent class analysis.

    Science.gov (United States)

    Libon, David J; Drabick, Deborah A G; Giovannetti, Tania; Price, Catherine C; Bondi, Mark W; Eppig, Joel; Devlin, Kathryn; Nieves, Christine; Lamar, Melissa; Delano-Wood, Lisa; Nation, Daniel A; Brennan, Laura; Au, Rhoda; Swenson, Rod

    2014-01-01

    Epidemiologic autopsy studies show mixed Alzheimer's disease (AD)/vascular pathology in many patients. Moreover, clinical research shows that it is not uncommon for AD and vascular dementia (VaD) patients to be equally impaired on memory, executive, or other neurocognitive tests. However, this clinical heterogeneity has not been incorporated into the new diagnostic criteria for AD (Dubois et al., 2010; McKhann et al., 2011). The current research applied Latent Class Analysis (LCA) to a protocol of six neuropsychological parameters to identify phenotypic subtypes from a large group of AD/VaD participants. Follow-up analyses examined difference between groups on neuroradiological parameters and neuropsychological measures of process and errors. 223 AD/VaD patients were administered a comprehensive neuropsychological protocol. Measures of whole brain and hippocampal volume were available for a portion of the sample (n = 76). LCA identified four distinct groups: moderate/mixed dementia (n = 54; 24.21%), mild/mixed dementia (n = 91; 40.80%); dysexecutive (n = 49, 21.97%), and amnestic (n = 29, 13.00%). Follow-up analyses comparing the groups on neuropsychological process and error scores showed that the dysexecutive group exhibited difficulty sustaining mental set. The moderate/mixed group evidenced pronounced impairment on tests of lexical retrieval/naming along with significant amnesia. Amnestic patients also presented with gross amnesia, but showed relative sparing on other neuropsychological measures. Mild/mixed patients exhibited milder memory deficits that were intermediary between the amnestic and moderate/mixed groups. There are distinct neuropsychological profiles in patients independent of clinical diagnosis, suggesting that the two are not wholly separate and that this information should be integrated into new AD diagnostic paradigms.

  4. A Novel Vascular Endothelial Growth Factor Receptor Participates in White Spot Syndrome Virus Infection in Litopenaeus vannamei

    Directory of Open Access Journals (Sweden)

    Shihao Li

    2017-11-01

    Full Text Available Vascular endothelial growth factor (VEGF signaling pathway is known to play key roles in endothelial cell proliferation, migration, angiogenesis, vascular permeability, inhibition of apoptosis, and virus infection. In the present study, a novel VEGFR gene (LvVEGFR2 was identified and characterized from Litopenaeus vannamei. The deduced amino acid sequence of LvVEGFR2 possessed typical features of VEGFRs reported in other species, including six IG-like domains, a transmembrane motif, a protein kinase (PK domain, and one tyrosine-PK active site. The transcripts of LvVEGFR2 were mainly detected in hemocytes and lymphoid organ (Oka. Subcellular localization analysis showed that LvVEGFR2 was a membrane protein. Its expression level was obviously upregulated in hemocytes and Oka of the shrimp after white spot syndrome virus (WSSV infection. Knockdown of LvVEGFR2 gene expression by double-strand RNA mediated interference could lead to a decrease of virus copy number in WSSV-infected shrimp. The interaction between LvVEGFR2 and different LvVEGFs (LvVEGF1, LvVEGF2, and LvVEGF3 in shrimp was analyzed at the transcription level and protein level, respectively. Knockdown of LvVEGF2 or LvVEGF3 could downregulate the expression level of LvVEGFR2, and injection of the recombinant LvVEGF2 or LvVEGF3 could upregulate the expression level of LvVEGFR2. Yeast two-hybrid analysis showed that LvVEGFR2 could interact with LvVEGF2 and LvVEGF3 directly. The study improved our understanding on the VEGF signaling pathway of shrimp and its role during WSSV infection.

  5. Effect of metformin treatment on endometrial vascular indices in anovulatory obese/overweight women with polycystic ovarian syndrome using three-dimensional power doppler ultrasonography.

    Science.gov (United States)

    Mohsen, Iman Abdel; Elkattan, Eman; Nabil, Hala; Khattab, Sherif

    2013-06-01

    Metformin has been shown to be an effective treatment for anovulatory polycystic ovary syndrome (PCOS) patients in terms of menstrual cyclicity, ovulation, and pregnancy, as well as reduction of early miscarriage rate. The aim of the study is to assess the effect of metformin on the endometrial vascular indices in anovulatory obese PCOS women using three-dimensional power Doppler sonography (3DPDUS). A prospective study was set to determine the beneficial effects of metformin on PCOS patients. Fifty anovulatory obese PCOS patients were compared with another 50 healthy volunteers who were age- and body mass index-matched (control group). PCOS patients were treated with metformin (Glucophage; MerckSerono) 850 mg 3 times a day for 6 months. Assessment of the endometrial thickness and volume, uterine Doppler indices, and Doppler vascular indices of the endometrium and subendometrium in the periovulatory and midluteal phases were performed with 3DPDUS. There was a significant increase in the endometrial thickness, endometrial volume, and endometrial and subendometrial vascularity indices (vascularization index, flow index, vascularization flow index) after 6 months of metformin treatment in PCOS women, whereas there was no change in the resistance index and the pulsatility index of the uterine artery in both periovulatory and midluteal phases. Metformin, owing to its metabolic, endocrine, vascular, and anti-inflammatory effects, improves markers of endometrial receptivity. Copyright © 2012 Wiley Periodicals, Inc.

  6. Vascular Ehlers-Danlos syndrome with cryptorchidism, recurrent pneumothorax, and pulmonary capillary hemangiomatosis-like foci: A case report.

    Science.gov (United States)

    Park, Min A; Shin, So Youn; Kim, Young Jin; Park, Myung Jae; Lee, Seung Hyeun

    2017-11-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a rare autosomal dominant inherited collagen disorder caused by defects or deficiency of pro-alpha 1 chain of type III procollagen encoded by COL3A1. vEDS is characterized not only by soft tissue manifestations including hyperextensibility of skin and joint hypermobility but also by early mortality due to rupture of arteries or vital organs. Although pulmonary complications are not common, vEDS cases complicated by pneumothorax, hemothorax, or intrapulmonary hematoma have been reported. When a patient initially presents only with pulmonary complications, it is not easy for clinicians to suspect vEDS. We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. Routine laboratory findings were unremarkable. Chest high resolution computed tomographic scan showed age-unmatched hyperinflation of both lungs, atypical cystic changes and multifocal ground glass opacities scattered in both lower lobes. His slender body shape, hyperflexible joints, and hyperextensible skin provided clue to suspicion of a possible connective tissue disorder. The histological examination of the lung lesions showed excessive capillary proliferation in the pulmonary interstitium and pleura allowing the diagnosis of pulmonary capillary hemangiomatosis (PCH)-like foci. Genetic study revealed COL3A1 gene splicing site mutation confirming his diagnosis as vEDS. Although his diagnosis vEDS is notorious for fatal vascular complication, there was no evidence of such complication at presentation. Fortunately, he has been followed up for 10 months without pulmonary or vascular complications. To the best of our knowledge, both cryptorchidism and PCH-like foci have never been reported yet as complications of vEDS, suggesting our case might be a new variant of this condition. This case emphasizes the importance of comprehensive physical examination and history-taking, and the clinical

  7. Distally based saphenous neurocutaneous perforator flap combined with vac therapy for soft tissue reconstruction and hardware salvage in the lower extremities.

    Science.gov (United States)

    Wen, Gen; Wang, Chun-Yang; Chai, Yi-Min; Cheng, Liang; Chen, Ming; Yi-Min, L V

    2013-11-01

    The complex wound with the exposed hardware and infection is one of the common complications after the internal fixation of the tibia fracture. The salvage of hardware and reconstruction of soft tissue defect remain challenging. In this report, we presented our experience on the use of the distally based saphenous neurocutaneous perforator flap combined with vacuum-assisted closure (VAC) therapy for the coverage of the soft tissue defect and the exposed hardware in the lower extremity with fracture. Between January 2008 and July 2010, seven patients underwent the VAC therapy followed by transferring a reversed saphenous neurocutaneous perforator flap for reconstruction of the wound with exposed hardware around the distal tibia. The sizes of the flaps ranged from 6 × 3 cm to 15 × 6 cm. Six flaps survived completely. Partial necrosis occurred in one patient. There were no other complications of repair and donor sites. Bone healing was achieved in all patients. In conclusion, the reversed saphenous neurocutaneous perfortor flaps combined with the VAC therapy might be one of the options to cover the complex wound with exposed hardware in the lower extremities. © 2013 Wiley Periodicals, Inc.

  8. Postcardiac injury syndrome following vascular interventional radiofrequency ablation for paroxysmal atrial fibrillation

    Directory of Open Access Journals (Sweden)

    Shungo Yukumi

    2015-01-01

    Full Text Available Postcardiac injury syndrome (PCIS occurs following a pericardial or myocardial injury. On the other hand, PCIS following cardiac catheter intervention is rare and can be difficult to diagnose because of its delayed onset. A 24-year-old man underwent radiofrequency ablation (RFA for paroxysmal atrial fibrillation and suffered from general fatigue and left-sided pleural effusion three months after the procedure. His symptoms and effusion were effectively treated within a month by administrating nonsteroidal anti-inflammatory drugs. However, seven months later, he developed left-sided chest pain and low-grade fever. Computed tomography showed a thickening of the parietal pleura and reccurence of the pleural effusion. Pleural biopsy by video-assisted thoracoscopy demonstrated chronic pleuritis with a non-necrotizing granulomatous reaction. Given the previous RFA, and in the absence of infection or malignant disease, he was diagnosed with PCIS and treated with colchicine.

  9. Associations of cardiovascular risk factors, carotid intima-media thickness and manifest atherosclerotic vascular disease with carpal tunnel syndrome

    Directory of Open Access Journals (Sweden)

    Liira Helena

    2011-04-01

    Full Text Available Abstract Background The role of atherosclerosis in carpal tunnel syndrome (CTS has not previously been addressed in population studies. The aim of this study was to investigate the associations of cardiovascular risk factors, carotid artery intima-media thickness (IMT, and clinical atherosclerotic diseases with CTS. Methods In this cross sectional study, the target population consisted of subjects aged 30 or over who had participated in the national Finnish Health Survey in 2000-2001. Of the 7977 eligible subjects, 6254 (78.4% were included in our study. Carotid IMT was measured in a sub-sample of subjects aged 45 to 74 (N = 1353. Results Obesity (adjusted odds ratio (OR 2.4, 95% confidence interval (CI 1.1-5.4, high LDL cholesterol (OR 3.8, 95% CI 1.6-9.1 for >190 vs. 200 vs. Conclusions Our findings suggest an association between CTS and cardiovascular risk factors in young people, and carotid IMT and clinical atherosclerotic vascular disease in older people. CTS may either be a manifestation of atherosclerosis, or both conditions may share similar risk factors.

  10. Gómez-López-Hernández Syndrome: A Rare Cause of Bilateral Nonscarring Alopecia.

    Science.gov (United States)

    Saricam, Merve Hatun; Tekin, Burak; Unver, Olcay; Ekinci, Gazanfer; Ergun, Tulin

    2015-01-01

    Gómez-López-Hernández syndrome is a rare neurocutaneous disorder characterized by the triad of rhombencephalosynapsis, parietal alopecia, and trigeminal anesthesia. We report a 16-year-old girl with bilateral parietotemporal alopecia in whom cranial magnetic resonance imaging revealed rhombencephalosynapsis, suggesting a diagnosis of Gómez-López-Hernández syndrome. Neurologic examination and neuroimaging may be warranted in select patients with parietal alopecia to exclude this uncommon entity. © 2015 Wiley Periodicals, Inc.

  11. ''Iatrogenic Gilbert syndrome''--a strategy for reducing vascular and cancer risk by increasing plasma unconjugated bilirubin.

    Science.gov (United States)

    McCarty, Mark F

    2007-01-01

    diagnosis of hyperbilirubinemia, and presumably could be used to induce an ''iatrogenic Gilbert syndrome''. Other drugs, such as rifampin, can raise serum bilirubin through competitive inhibition of hepatocyte bilirubin uptake--although unfortunately rifampin is not as safe as probenecid. Measures which can safely achieve moderate serum elevations of bilirubin may prove to have value in the prevention and/or treatment of a wide range of disorders in which oxidants play a prominent pathogenic role, including many vascular diseases, cancer, and inflammatory syndromes. Phycobilins, algal biliverdin metabolites that are good substrates for biliverdin reductase, may prove to have clinical antioxidant potential comparable to that of bilirubin.

  12. Metabolic syndrome predicts vascular changes in whole body magnetic resonance imaging in patients with long standing diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Findeisen Hannes M

    2010-08-01

    Full Text Available Abstract Background Although diabetic patients have an increased rate of cardio-vascular events, there is considerable heterogeneity with respect to cardiovascular risk, requiring new approaches to individual cardiovascular risk factor assessment. In this study we used whole body-MR-angiography (WB-MRA to assess the degree of atherosclerosis in patients with long-standing diabetes and to determine the association between metabolic syndrome (MetS and atherosclerotic burden. Methods Long standing (≥10 years type 1 and type 2 diabetic patients (n = 59; 31 males; 63.3 ± 1.7 years were examined by WB-MRA. Based on the findings in each vessel, we developed an overall score representing the patient's vascular atherosclerotic burden (MRI-score. The score's association with components of the MetS was assessed. Results The median MRI-score was 1.18 [range: 1.00-2.41] and MetS was present in 58% of the cohort (type 2 diabetics: 73%; type 1 diabetics: 26%. Age (p = 0.0002, HDL-cholesterol (p = 0.016, hypertension (p = 0.0008, nephropathy (p = 0.0093, CHD (p = 0.001 and MetS (p = 0.0011 were significantly associated with the score. Adjusted for age and sex, the score was significantly (p = 0.02 higher in diabetics with MetS (1.450 [1.328-1.572] compared to those without MetS (1.108 [0.966-1.50]. The number of MetS components was associated with a linear increase in the MRI-score (increase in score: 0.09/MetS component; r2 = 0.24, p = 0.038. Finally, using an established risk algorithm, we found a significant association between MRI-score and 10-year risk for CHD, fatal CHD and stroke. Conclusion In this high-risk diabetic population, WB-MRA revealed large heterogeneity in the degree of systemic atherosclerosis. Presence and number of traits of the MetS are associated with the extent of atherosclerotic burden. These results support the perspective that diabetic patients are a heterogeneous population with increased but varying prevalence of atherosclerosis

  13. Vascular complications (splenic and hepatic artery aneurysms) in the occipital horn syndrome: report of a patient and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Mentzel, H.-J. [Institute of Diagnostic and Interventional Radiology, University of Jena (Germany)]|[Institute of Diagnostic and Interventional Radiology, Bachstrasse 18, D-07 740 Jena (Germany); Seidel, J.; Vogt, L. [Department of Paediatrics, University of Jena, Friedrich-Schiller-Universitaet Jena, Jena/Thueringen (Germany); Vogt, S.; Kaiser, W.A. [Institute of Diagnostic and Interventional Radiology, University of Jena (Germany)

    1999-01-01

    We report an 18-year-old boy with occipital horn syndrome who developed aneurysms of the splenic and hepatic arteries. Occipital horn syndrome, also called X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is characterised by a skeletal dysplasia which includes occipital horns, broad clavicles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified long bones and coxa valga. Distinctive features common to all patients are unusual facial appearance, hypermobility of finger joints, limitation of extension of elbows, chronic diarrhoea and genitourinary abnormalities. In this case report we describe the difficulties encountered in the diagnostic management of patients with EDS-related vascular lesions. (orig.) With 5 figs., 2 tabs., 12 refs.

  14. Effects on Pulmonary Vascular Mechanics of Two Different Lung-Protective Ventilation Strategies in an Experimental Model of Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Santos, Arnoldo; Gomez-Peñalver, Eva; Monge-Garcia, M Ignacio; Retamal, Jaime; Borges, João Batista; Tusman, Gerardo; Hedenstierna, Goran; Larsson, Anders; Suarez-Sipmann, Fernando

    2017-11-01

    To compare the effects of two lung-protective ventilation strategies on pulmonary vascular mechanics in early acute respiratory distress syndrome. Experimental study. University animal research laboratory. Twelve pigs (30.8 ± 2.5 kg). Acute respiratory distress syndrome was induced by repeated lung lavages and injurious mechanical ventilation. Thereafter, animals were randomized to 4 hours ventilation according to the Acute Respiratory Distress Syndrome Network protocol or to an open lung approach strategy. Pressure and flow sensors placed at the pulmonary artery trunk allowed continuous assessment of pulmonary artery resistance, effective elastance, compliance, and reflected pressure waves. Respiratory mechanics and gas exchange data were collected. Acute respiratory distress syndrome led to pulmonary vascular mechanics deterioration. Four hours after randomization, pulmonary vascular mechanics was similar in Acute Respiratory Distress Syndrome Network and open lung approach: resistance (578 ± 252 vs 626 ± 153 dyn.s/cm; p = 0.714), effective elastance, (0.63 ± 0.22 vs 0.58 ± 0.17 mm Hg/mL; p = 0.710), compliance (1.19 ± 0.8 vs 1.50 ± 0.27 mL/mm Hg; p = 0.437), and reflection index (0.36 ± 0.04 vs 0.34 ± 0.09; p = 0.680). Open lung approach as compared to Acute Respiratory Distress Syndrome Network was associated with improved dynamic respiratory compliance (17.3 ± 2.6 vs 10.5 ± 1.3 mL/cm H2O; p mechanics similarly. The use of higher positive end-expiratory pressures in the open lung approach strategy did not worsen pulmonary vascular mechanics, improved lung mechanics, and gas exchange but at the expense of a lower cardiac index.

  15. Identification and classification of traditional Chinese medicine syndrome types among senior patients with vascular mild cognitive impairment using latent tree analysis.

    Science.gov (United States)

    Fu, Chen; Zhang, Nevin Lianwen; Chen, Bao-Xin; Chen, Zhou Rong; Jin, Xiang Lan; Guo, Rong-Juan; Chen, Zhi-Gang; Zhang, Yun-Ling

    2017-05-01

    To treat patients with vascular mild cognitive impairment (VMCI) using traditional Chinese medicine (TCM), it is necessary to classify the patients into TCM syndrome types and to apply different treatments to different types. In this paper, we investigate how to properly carry out the classification for patients with VMCI aged 50 or above using a novel data-driven method known as latent tree analysis (LTA). A cross-sectional survey on VMCI was carried out in several regions in Northern China between February 2008 and February 2012 which resulted in a data set that involves 803 patients and 93 symptoms. LTA was performed on the data to reveal symptom co-occurrence patterns, and the patients were partitioned into clusters in multiple ways based on the patterns. The patient clusters were matched up with syndrome types, and population statistics of the clusters are used to quantify the syndrome types and to establish classification rules. Eight syndrome types are identified: Qi deficiency, Qi stagnation, Blood deficiency, Blood stasis, Phlegm-dampness, Fire-heat, Yang deficiency, and Yin deficiency. The prevalence and symptom occurrence characteristics of each syndrome type are determined. Quantitative classification rules are established for determining whether a patient belongs to each of the syndrome types. A solution for the TCM syndrome classification problem for patients with VMCI and aged 50 or above is established based on the LTA of unlabeled symptom survey data. The results can be used as a reference in clinic practice to improve the quality of syndrome differentiation and to reduce diagnosis variances across physicians. They can also be used for patient selection in research projects aimed at finding biomarkers for the syndrome types and in randomized control trials aimed at determining the efficacy of TCM treatments of VMCI.

  16. MRI sagittal abdominal diameter is a stronger predictor of metabolic syndrome than visceral fat area or waist circumference in a high-risk vascular cohort

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    Michel R Hoenig

    2010-07-01

    Full Text Available Michel R HoenigUniversity of Queensland, Brisbane, Queensland, AustraliaObjective: To determine whether sagittal abdominal diameter (SAD is associated with the metabolic syndrome independently of visceral fat area (VFA and waist circumference (WC.Methods: Forty-three high-risk vascular patients were evaluated for metabolic syndrome criteria and underwent magnetic resonance imaging (MRI to quantify SAD and VFA at the L4–L5 disc.Comparisons: 1. Baseline differences in patients with and without the metabolic syndrome 2. Forward binary logistic regression analysis of predictors of the metabolic syndrome with SAD, VFA and WC as independents 3. Correlates of SAD.Results: Patients with metabolic syndrome had greater SAD, VFA and WC than patients without the metabolic syndrome (P < 0.01. Of SAD, VFA and WC, only SAD was associated with metabolic syndrome on forward binary logistic regression; beta 0.68, Wald’s statistic 10.8 (P = 0.001 and c-statistic 0.89 (P < 0.001. A > 22.7 cm SAD threshold identified metabolic syndrome with a 91% sensitivity and 80% specificity. SAD correlated with waist circumference (r = 0.918, high-density lipoprotein-cholesterol (r = –0.363, triglyceride (r = 0.401, fasting glucose (r = 0.428 and the QUICK index of insulin sensitivity (r = –0.667 (all P < 0.05.Conclusions: MRI-measured SAD is associated with the metabolic syndrome and renders the current gold standard of VFA redundant. This measure of obesity-related cardiovascular risk requires validation and evaluation in a prospective cohort.Keywords: obesity, insulin resistance

  17. Neuropeptide deficient mice have attenuated nociceptive, vascular, and inflammatory changes in a tibia fracture model of complex regional pain syndrome

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    Guo Tian-Zhi

    2012-11-01

    Full Text Available Abstract Background Distal limb fracture in man can induce a complex regional pain syndrome (CRPS with pain, warmth, edema, and cutaneous inflammation. In the present study substance P (SP, Tac1−/− and CGRP receptor (RAMP1−/− deficient mice were used to investigate the contribution of neuropeptide signaling to CRPS-like changes in a tibia fracture mouse model. Wildtype, Tac1−/−, and RAMP1−/− mice underwent tibia fracture and casting for 3 weeks, then the cast was removed and hindpaw mechanical allodynia, unweighting, warmth, and edema were tested over time. Hindpaw skin was collected at 3 weeks post-fracture for immunoassay and femurs were collected for micro-CT analysis. Results Wildtype mice developed hindpaw allodynia, unweighting, warmth, and edema at 3 weeks post-fracture, but in the Tac1−/− fracture mice allodynia and unweighting were attenuated and there was no warmth and edema. RAMP1−/− fracture mice had a similar presentation, except there was no reduction in hindpaw edema. Hindpaw skin TNFα, IL-1β, IL-6 and NGF levels were up-regulated in wildtype fracture mice at 3 weeks post-fracture, but in the Tac1−/− and RAMP1−/− fracture mice only IL-6 was increased. The epidermal keratinocytes were the cellular source for these inflammatory mediators. An IL-6 receptor antagonist partially reversed post-fracture pain behaviors in wildtype mice. Conclusions In conclusion, both SP and CGRP are critical neuropeptide mediators for the pain behaviors, vascular abnormalities, and up-regulated innate immune responses observed in the fracture hindlimb. We postulate that the residual pain behaviors observed in the Tac1−/− and RAMP1−/− fracture mice are attributable to the increased IL-6 levels observed in the hindpaw skin after fracture.

  18. Blood pressure-independent effect of candesartan on cardio-ankle vascular index in hypertensive patients with metabolic syndrome

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    Kanako Bokuda

    2010-07-01

    Full Text Available Kanako Bokuda1, Atsuhiro Ichihara1,2, Mariyo Sakoda1, Asako Mito1, Kenichiro Kinouchi1, Hiroshi Itoh11Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan; 2Department of Endocrinology and Anti-Aging Medicine, Keio University School of Medicine, Tokyo, JapanAbstract: Angiotensin receptor blockers (ARBs are known to reduce the cardiovascular risk in hypertensive patients. This study was designed to examine the effect of an ARB candesartan on subclinical atherosclerosis assessed by cardio-ankle vascular index (CAVI in comparison with calcium channel blockers (CCBs alone in hypertensive patients with metabolic syndrome (MetS. A total of 53 consecutive hypertensive patients with MetS were randomly assigned to the candesartan group, in which candesartan was added on, or the CCBs group, in which CCBs were added on. Clinical and biological parameters were obtained before and after the 12-month treatment period. The primary measure of efficacy was the %change in CAVI. When treated with candesartan, but not CCBs, CAVI significantly decreased from 8.7 to 7.7 by 11%. Blood pressure (BP significantly decreased with both treatments, but the differences between groups were not significant. The changes in other parameters remained unchanged in both the groups. Analysis of covariance found that both the BP reduction and the therapy difference contributed to the decrease in CAVI, but the BP reduction was not involved in the decrease in CAVI caused by the difference in the therapy. Candesartan may be a better antihypertensive drug than CCBs to that subclinical atherosclerosis of patients with MetS.Keywords: albuminuria, ambulatory blood pressure, calcium channel blockers, carotid ­intima-media thickness

  19. Modification of the liver fatty acids by Hibiscus sabdariffa Linnaeus (Malvaceae) infusion, its possible effect on vascular reactivity in a metabolic syndrome model.

    Science.gov (United States)

    Pérez-Torres, Israel; Zúñiga Muñoz, Alejandra; Beltrán-Rodríguez, Ulises; Díaz-Díaz, Eulises; Martínez-Memije, Raúl; Guarner Lans, Verónica

    2014-01-01

    We investigated the effects of Hibiscus sabdariffa Linnaeus (HSL)-fed infusion on the fatty acid (FA) profile in liver of metabolic syndrome (MS) rats and its possible effect on vascular reactivity. Body mass, intra-abdominal fat, triglycerides, insulin, blood pressure, saturated, monounsaturated FA, NEFAs, Δ(9)-, Δ(6)-desaturases and vasoconstriction were increased, while vasorelaxation, polyunsaturated FA, endothelial nitric oxide and [Formula: see text]/[Formula: see text] ratio decreased in MS versus Control, but HSL infusion modified it and increased Δ(5)-desaturase. The results suggest that the alteration in FA liver metabolism in the MS contributes to impaired vascular reactivity, but treatment with of HSL infusion can improve this condition.

  20. Vascular ossification – calcification in metabolic syndrome, type 2 diabetes mellitus, chronic kidney disease, and calciphylaxis – calcific uremic arteriolopathy: the emerging role of sodium thiosulfate

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    Sowers James R

    2005-03-01

    Full Text Available Abstract Background Vascular calcification is associated with metabolic syndrome, diabetes, hypertension, atherosclerosis, chronic kidney disease, and end stage renal disease. Each of the above contributes to an accelerated and premature demise primarily due to cardiovascular disease. The above conditions are associated with multiple metabolic toxicities resulting in an increase in reactive oxygen species to the arterial vessel wall, which results in a response to injury wound healing (remodeling. The endothelium seems to be at the very center of these disease processes, acting as the first line of defense against these multiple metabolic toxicities and the first to encounter their damaging effects to the arterial vessel wall. Results The pathobiomolecular mechanisms of vascular calcification are presented in order to provide the clinician – researcher a database of knowledge to assist in the clinical management of these high-risk patients and examine newer therapies. Calciphylaxis is associated with medial arteriolar vascular calcification and results in ischemic subcutaneous necrosis with vulnerable skin ulcerations and high mortality. Recently, this clinical syndrome (once thought to be rare is presenting with increasing frequency. Consequently, newer therapeutic modalities need to be explored. Intravenous sodium thiosulfate is currently used as an antidote for the treatment of cyanide poisioning and prevention of toxicities of cisplatin cancer therapies. It is used as a food and medicinal preservative and topically used as an antifungal medication. Conclusion A discussion of sodium thiosulfate's dual role as a potent antioxidant and chelator of calcium is presented in order to better understand its role as an emerging novel therapy for the clinical syndrome of calciphylaxis and its complications.

  1. A New COL3A1 Mutation in Ehlers-Danlos Syndrome Vascular Type With Different Phenotypes in the Same Family.

    Science.gov (United States)

    Cortini, Francesca; Marinelli, Barbara; Romi, Silvia; Seresini, Agostino; Pesatori, Angela Cecilia; Seia, Manuela; Montano, Nicola; Bassotti, Alessandra

    2017-04-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a rare and severe connective tissue disorder caused by mutations in the collagen type III alpha I chain ( COL3A1) gene. We describe a pathogenetic heterozygous COL3A1 mutation c.3140 G>A, p. Gly1047Asp, identified using next-generation sequencing, in a 40-year-old Italian female. The genetic test performed on her relatives, which present different clinical phenotypes, confirmed that they carry the same mutation in heterozygous state. This finding confirms that mutations causing vEDS have an incomplete penetrance.

  2. VASCULAR DEMENTIA

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    Maria Alekseyevna Cherdak

    2010-01-01

    vascular cognitive disorders and vascular dementia (VD. The heterogeneity of vascular cognitive disorders, concurrence of vascular and neurodegenerative diseases are discussed. Data from studies of specific therapy for VD are given.

  3. Vascular manifestations of Behcet's disease

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    Regina Georgiyeva Goloeva

    2010-01-01

    Conclusion. Vascular disorders in BD were diagnosed in one fourth of the patients, mainly in young male patients. Severe thromboses with the development of chronic venous insignificance, Budd-Chiari syndrome, pulmonary and iliac artery aneurysms, and arterial thromboses were observed in male patients only. Vascular events were associated with erythema nodosum and epididymitis; in these concomitances, the vascular risk was substantially increased. Vascular death rates were 2,2%.

  4. Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

    Science.gov (United States)

    Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

    2018-02-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

  5. Gastric antral vascular ectasia and solitary rectal ulcer syndrome - two rare diagnoses as the cause of anemia in a single patient: case report.

    Science.gov (United States)

    Kunovský, Lumír; Dastych, Milan; Kroupa, Radek; Hemmelova, Beata; Muckova, Katarina; Chovancova, Miroslava; Kucerova, Lenka; Dolina, Jiri

    Gastric antral vascular ectasia (GAVE) and solitary rectal ulcer syndrome (SRUS) are both mentioned in the literature as rare causes of iron deficiency anemia and gastrointestinal (GI) bleeding. GAVE accounts for up to 4 % of upper non-variceal GI bleeding; SRUS is a rare benign disorder that presents with rectal bleeding. We present the case of a 75-year-old patient who was admitted to our facility with anemia. In the same patient, we encountered chronic bleeding from GAVE and SRUS. Both diagnoses were treated endoscopically: GAVE by argon plasma coagulation and a subsequent treatment with proton pump inhibitors and SRUS by adrenaline injection and clipping, consecutively treated with mesalazine enemas. The patient was successfully cured, resulting in a stable level of hemoglobin and no recurrent GI bleeding. We report a unique case of chronic GI bleeding caused by two uncommon diagnoses. The co-occurrence of GAVE and SRUS has not been previously described or published.Key words: anemia - endoscopy - gastric antral vascular ectasia (GAVE) - gastrointestinal bleeding - solitary rectal ulcer syndrome (SRUS).

  6. The pathogenesis of ovarian hyperstimulation syndrome: in vivo studies investigating the role of interleukin-1beta, interleukin-6, and vascular endothelial growth factor.

    Science.gov (United States)

    Pellicer, A; Albert, C; Mercader, A; Bonilla-Musoles, F; Remohí, J; Simón, C

    1999-03-01

    To evaluate systemic and ovarian changes in levels of interleukin (IL)-1beta, IL-6, and vascular endothelial growth factor (VEGF) in response to hCG administration to determine which may be the potential initiator of vascular effects and to identify the main source of the substance; to evaluate serum and follicular fluid levels of these cytokines as markers of ovarian hyperstimulation syndrome (OHSS), and to compare levels of these cytokines under basal conditions in women with normal ovulation and those with polycystic ovary syndrome (PCOS). Prospective controlled study. In vitro fertilization program at the Instituto Valenciano de Infertilidad, Valencia, Spain. Women undergoing IVF, in whom the first two study objectives were analyzed, and women with normal ovulation and patients with PCOS undergoing retrieval of immature oocytes in natural cycles or cycles stimulated for IUI but cancelled during induction of ovulation, in whom the third study objective was analyzed. Serum was collected before and after hCG administration, and follicular fluid was collected at ovum pick-up. Serum and follicular fluid levels of IL-1beta, IL-6, and VEGF. There was a significant increase in serum VEGF levels after hCG administration in patients who were at risk for OHSS compared with those who were not at risk for OHSS. Significantly lower VEGF levels were found in the follicular fluid of patients who were at risk; this decrease was the only useful marker to discriminate between the two groups. Moreover, both groups had similar cytokine production under basal conditions. An increase in serum E2 occurred coincident with a decrease in IL-1beta, IL-6, and VEGF in patients with PCOS. Vascular endothelial growth factor seems to be the mediator of hCG on the vascular tree. There was an early systemic increase in VEGF that may have significance in the development of OHSS. A decrease in the follicular fluid VEGF concentration is a valid marker to identify women in whom OHSS will develop

  7. Vascular biology of preeclampsia.

    Science.gov (United States)

    Myatt, L; Webster, R P

    2009-03-01

    Preeclampsia, a pregnancy-specific syndrome characterized by hypertension, proteinuria and edema, resolves on delivery of the placenta. Normal pregnancy is itself characterized by systemic inflammation, oxidative stress and alterations in levels of angiogenic factors and vascular reactivity. This is exacerbated in preeclampsia with an associated breakdown of compensatory mechanisms, eventually leading to placental and vascular dysfunction. The underlying pathology of preeclampsia is thought to be a relatively hypoxic or ischemic placenta. Both the placenta and maternal vasculatures are major sources of reactive oxygen and nitrogen species which can interact to produce peroxynitrite a powerful prooxidant that covalently modifies proteins by nitration of tyrosine residues, to possibly alter vascular function in preeclampsia. The linkage between placental hypoxia and maternal vascular dysfunction has been proposed to be via placental syncytiotrophoblast basement membranes shed by the placenta or via angiogenic factors which include soluble flt1 and endoglin secreted by the placenta that bind vascular endothelial growth factor (VEGF) and placental growth factor (PIGF) in the maternal circulation. There is also abundant evidence of altered reactivity of the maternal and placental vasculature and of the altered production of autocoids in preeclampsia. The occurrence of preeclampsia is increased in women with preexisting vascular disease and confers a long-term risk for development of cardiovascular disease. The vascular stress test of pregnancy thus identifies those women with a previously unrecognized at risk vascular system and promotes the development of preeclampsia. Preexisting maternal vascular dysfunction intensified by placental factors is possibly responsible for the individual pathologies of preeclampsia.

  8. Parry-Romberg Syndrome Associated with Localized Scleroderma

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    Jelena Maletic

    2010-06-01

    Full Text Available Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea.

  9. Cardio-ankle vascular index is associated with cardiovascular target organ damage and vascular structure and function in patients with diabetes or metabolic syndrome, LOD-DIABETES study: a case series report.

    Science.gov (United States)

    Gómez-Marcos, Manuel Ángel; Recio-Rodríguez, José Ignacio; Patino-Alonso, María Carmen; Agudo-Conde, Cristina; Gómez-Sánchez, Leticia; Gomez-Sanchez, Marta; Rodríguez-Sanchez, Emiliano; Maderuelo-Fernandez, Jose Angel; García-Ortiz, Luís

    2015-01-16

    The cardio ankle vascular index (CAVI) is a new index of the overall stiffness of the artery from the origin of the aorta to the ankle. This index can estimate the risk of atherosclerosis. We aimed to find the relationship between CAVI and target organ damage (TOD), vascular structure and function, and cardiovascular risk factors in Caucasian patients with type 2 diabetes mellitus or metabolic syndrome. We included 110 subjects from the LOD-Diabetes study, whose mean age was 61 ± 11 years, and 37.3% were women. Measurements of CAVI, brachial ankle pulse wave velocity (ba-PWV), and ankle brachial index (ABI) were taken using the VaSera device. Cardiovascular risk factors, renal function by creatinine, glomerular filtration rate, and albumin creatinine index were also obtained, as well as cardiac TOD with ECG and vascular TOD and carotid intima media thickness (IMT), carotid femoral PWV (cf-PWV), and the central and peripheral augmentation index (CAIx and PAIx). The Framingham-D'Agostino scale was used to measure cardiovascular risk. Mean CAVI was 8.7 ± 1.3. More than half (54%) of the participants showed one or more TOD (10% cardiac, 13% renal; 48% vascular), and 13% had ba-PWV ≥ 17.5 m/s. Patients with any TOD had the highest CAVI values: 1.15 (CI 95% 0.70 to 1.61, p < 0.001) and 1.14 (CI 95% 0.68 to 1.60, p < 0.001) when vascular TOD was presented, and 1.30 (CI 95% 0.51 to 2.10, p = 0.002) for the cardiac TOD. The CAVI values had a positive correlation with HbA1c and systolic and diastolic blood pressure, and a negative correlation with waist circumference and body mass index. The positive correlations of CAVI with IMT (β = 0.29; p < 0.01), cf-PWV (β = 0.83; p < 0.01), ba-PWV (β = 2.12; p < 0.01), CAIx (β = 3.42; p < 0.01), and PAIx (β = 5.05; p = 0.04) remained after adjustment for cardiovascular risk, body mass index, and antihypertensive, lipid-lowering, and antidiabetic drugs. The

  10. The Higher Response of Vascular Endothelial Growth Factor and Angiotensin-II to Human Chorionic Gonadotropin in Women with Polycystic Ovary Syndrome

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    Junwei Qu

    2015-02-01

    Full Text Available Background: This research investigated the response of vascular active factors, vascular endothelial growth factor (VEGF and angiotensin-II (AT-II to ovarian stimulation during 24 hours in patients with polycystic ovary syndrome (PCOS. Materials and Methods: In this clinical trial study, 52 patients with PCOS and 8 control cases were stimulated with human chorionic gonadotropin (HCG on the 4th to 7th day of the patients’ natural or induced menstrual cycles. We measured VEGF and AT-II by radioimmunoassay before the injection (0 hour and 3, 8, 12, 18 and 24 hours after the stimulation. Results: After ovarian stimulation, there was substantially higher level of VEGF in typical PCOS patients than the other three groups at the 3 hour time point (p<0.05, while there were no significant differences in VEGF at all the other time points among the four groups. As for AT-II, before and at all time points after the ovarian stimulation, it seemed that the AT-II levels in patients’ sera with different phenotypes of PCOS by the Rotterdam criteria were all higher than in the control group although the differences were not statistically significant. The level of AT-II in typical PCOS patients was also significantly higher than the other three groups at the 3 hour time point (p<0.05, while no significant differences at all the other time points among the four groups were observed. Conclusion: The response to the stimulation varied among patients with different phenotypes of PCOS according to the Rotterdam criteria. Serum VEGF and AT-II were possible contributors to an increased risk of developing ovarian hyperstimulation syndrome (OHSS in patients with typical PCOS during the early follicular phase (3 hours after ovarian stimulation (Registration Number: NCT02265861.

  11. Transcriptome analysis of skin fibroblasts with dominant negative COL3A1 mutations provides molecular insights into the etiopathology of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Chiarelli, Nicola; Carini, Giulia; Zoppi, Nicoletta; Ritelli, Marco; Colombi, Marina

    2018-01-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII), which is the major expressed collagen in blood vessels and hollow organs. The majority of disease-causing variants in COL3A1 are glycine substitutions and in-frame splice mutations in the triple helix domain that through a dominant negative effect are associated with the severe clinical spectrum potentially lethal of vEDS, characterized by fragility of soft connective tissues with arterial and organ ruptures. To shed lights into molecular mechanisms underlying vEDS, we performed gene expression profiling in cultured skin fibroblasts from three patients with different structural COL3A1 mutations. Transcriptome analysis revealed significant changes in the expression levels of several genes involved in maintenance of cell redox and endoplasmic reticulum (ER) homeostasis, COLLs folding and extracellular matrix (ECM) organization, formation of the proteasome complex, and cell cycle regulation. Protein analyses showed that aberrant COLLIII expression is associated with the disassembly of many structural ECM constituents, such as fibrillins, EMILINs, and elastin, as well as with the reduction of the proteoglycans perlecan, decorin, and versican, all playing an important role in the vascular system. Furthermore, the altered distribution of the ER marker protein disulfide isomerase PDI and the strong reduction of the COLLs-modifying enzyme FKBP22 are consistent with the disturbance of ER-related homeostasis and COLLs biosynthesis and post-translational modifications, indicated by microarray analysis. Our findings add new insights into the pathophysiology of this severe vascular disorder, since they provide a picture of the gene expression changes in vEDS skin fibroblasts and highlight that dominant negative mutations in COL3A1 also affect post-translational modifications and deposition into the ECM of

  12. Transcriptome analysis of skin fibroblasts with dominant negative COL3A1 mutations provides molecular insights into the etiopathology of vascular Ehlers-Danlos syndrome.

    Directory of Open Access Journals (Sweden)

    Nicola Chiarelli

    Full Text Available Vascular Ehlers-Danlos syndrome (vEDS is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII, which is the major expressed collagen in blood vessels and hollow organs. The majority of disease-causing variants in COL3A1 are glycine substitutions and in-frame splice mutations in the triple helix domain that through a dominant negative effect are associated with the severe clinical spectrum potentially lethal of vEDS, characterized by fragility of soft connective tissues with arterial and organ ruptures. To shed lights into molecular mechanisms underlying vEDS, we performed gene expression profiling in cultured skin fibroblasts from three patients with different structural COL3A1 mutations. Transcriptome analysis revealed significant changes in the expression levels of several genes involved in maintenance of cell redox and endoplasmic reticulum (ER homeostasis, COLLs folding and extracellular matrix (ECM organization, formation of the proteasome complex, and cell cycle regulation. Protein analyses showed that aberrant COLLIII expression is associated with the disassembly of many structural ECM constituents, such as fibrillins, EMILINs, and elastin, as well as with the reduction of the proteoglycans perlecan, decorin, and versican, all playing an important role in the vascular system. Furthermore, the altered distribution of the ER marker protein disulfide isomerase PDI and the strong reduction of the COLLs-modifying enzyme FKBP22 are consistent with the disturbance of ER-related homeostasis and COLLs biosynthesis and post-translational modifications, indicated by microarray analysis. Our findings add new insights into the pathophysiology of this severe vascular disorder, since they provide a picture of the gene expression changes in vEDS skin fibroblasts and highlight that dominant negative mutations in COL3A1 also affect post-translational modifications and deposition

  13. Vascular Ehlers–Danlos Syndrome in siblings with biallelic COL3A1 sequence variants and marked clinical variability in the extended family

    Science.gov (United States)

    Jørgensen, Agnete; Fagerheim, Toril; Rand-Hendriksen, Svend; Lunde, Per I; Vorren, Torgrim O; Pepin, Melanie G; Leistritz, Dru F; Byers, Peter H

    2015-01-01

    Vascular Ehlers–Danlos Syndrome (vEDS), also known as EDS type IV, is considered to be an autosomal dominant disorder caused by sequence variants in COL3A1, which encodes the chains of type III procollagen. We identified a family in which there was marked clinical variation with the earliest death due to extensive aortic dissection at age 15 years and other family members in their eighties with no complications. The proband was born with right-sided clubfoot but was otherwise healthy until he died unexpectedly at 15 years. His sister, in addition to signs consistent with vascular EDS, had bilateral frontal and parietal polymicrogyria. The proband and his sister each had two COL3A1 sequence variants, c.1786C>T, p.(Arg596*) in exon 26 and c.3851G>A, p.(Gly1284Glu) in exon 50 on different alleles. Cells from the compound heterozygote produced a reduced amount of type III procollagen, all the chains of which had abnormal electrophoretic mobility. Biallelic sequence variants have a significantly worse outcome than heterozygous variants for either null mutations or missense mutations, and frontoparietal polymicrogyria may be an added phenotype feature. This genetic constellation provides a very rare explanation for marked intrafamilial clinical variation due to sequence variants in COL3A1. PMID:25205403

  14. Comparison of blood pool and extracellular gadolinium chelate for functional MR evaluation of vascular thoracic outlet syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Ruth P., E-mail: ruthplim74@gmail.com [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Austin Health, Department of Radiology, Heidelberg, Victoria 3084 (Australia); The University of Melbourne, School of Medicine, Parkville, Victoria 3010 (Australia); Bruno, Mary, E-mail: mary.bruno@nyumc.org [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Rosenkrantz, Andrew B., E-mail: Andrew.rosenkrantz@nyumc.org [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Kim, Danny C., E-mail: danny.kim@nyumc.org [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Mulholland, Thomas, E-mail: Thomas.mulholland@nyumc.org [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Kwon, Jane, E-mail: jane.kwon@nyumc.org [New York University School of Medicine, Bernard and Irene Schwartz Center for Biomedical Imaging, Department of Radiology, 660 1st Avenue, New York, NY 10016 (United States); Palfrey, Amy P., E-mail: amy.pastva10@stjohns.edu [St John' s University, Department of Psychology, 8000 Utopia Parkway, Jamaica-Queens, NY 11439 (United States); Ogedegbe, Olugbenga, E-mail: Olugbenga.Ogedegbe@nyumc.org [New York University School of Medicine, Clinical and Translational Science Institute, 227 E30th St, 8th Floor, New York, NY 10016 (United States)

    2014-07-15

    Objective: To compare performance of single-injection blood pool agent (gadofosveset trisodium, BPA) against dual-injection extracellular contrast (gadopentetate dimeglumine, ECA) for MRA/MRV in assessment of suspected vascular TOS. Materials and methods: Thirty-one patients referred for vascular TOS evaluation were assessed with BPA (n = 18) or ECA (n = 13) MRA/MRV in arm abduction and adduction. Images were retrospectively assessed for: image quality (1 = non-diagnostic, 5 = excellent), vessel contrast (1 = same signal as muscle, 4 = much brighter than muscle) and vascular pathology by two independent readers, with a separate experienced reader providing reference assessment of vascular pathology. Results: Median image quality was diagnostic or better (score ≥3) for ECA and BPA at all time points, with BPA image quality superior at abduction late (BPA 4.5, ECA 4, p = 0.042) and ECA image quality superior at adduction-early (BPA 4.5; ECA 4.0, p = 0.018). High qualitative vessel contrast (mean score ≥3) was observed at all time points with both BPA and ECA, with superior BPA vessel contrast at abduction-late (BPA 3.97 ± 0.12; ECA 3.73 ± 0.26, p = 0.007) and ECA at adduction-early (BPA 3.42 ± 0.52; ECA 3.96 ± 0.14, p < 0.001). Readers readily identified arterial and venous pathology with BPA, similar to ECA examinations. Conclusion: Single-injection BPA MRA/MRV for TOS evaluation demonstrated diagnostic image quality and high vessel contrast, similar to dual-injection ECA imaging, enabling identification of fixed and functional arterial and venous pathology.

  15. Diagnostic criteria for vascular dementia

    NARCIS (Netherlands)

    Scheltens, P.; Hijdra, A. H.

    1998-01-01

    The term vascular dementia implies the presence of a clinical syndrome (dementia) caused by, or at least assumed to be caused by, a specific disorder (cerebrovascular disease). In this review, the various sets of criteria used to define vascular dementia are outlined. The various sets of criteria

  16. Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2.

    Science.gov (United States)

    Tasaki, Yuko; Shimizu, Masaki; Inoue, Natsumi; Mizuta, Mao; Nakagishi, Yasuo; Wada, Taizo; Yachie, Akihiro

    2016-04-01

    To assess the role of angiopoietin (Ang)-1 and Ang-2 and to investigate the clinical significance of serum levels of them in systemic juvenile idiopathic arthritis (s-JIA)-associated macrophage activation syndrome (MAS), we determined these levels in 51 patients with s-JIA, 11 patients with polyarticular JIA (poly-JIA), 12 patients with virus associated hemophagocytic syndrome (VAHS), 12 patients with Kawasaki disease (KD), and 15 age-matched healthy controls (HC). The results were compared with clinical features of MAS. During the MAS phase, serum Ang-1 levels were significantly decreased compared with those during the active and inactive phases. Serum Ang-2/1 ratio were significantly elevated during the MAS phase, compared with those during the active and inactive phases. There was a rapid increase in the Ang-2/1 ratio at the onset of MAS. Serum Ang-1 and the Ang-2/1 ratio significantly correlated with measures of disease activity, including AST and LDH. Ang-2/1 dysregulation was also observed in patients with VAHS, whereas not observed in most cases of KD. The homeostasis of vascular endothelial function by Ang-1 and Ang-2 is disrupted in MAS. Serum Ang-1 levels and the Ang-2/1 ratio might represent promising indicators of disease activity for MAS. Copyright © 2016 Elsevier Ltd. All rights reserved.

  17. "Choke" vessels between vascular territories of the abdominal wall: literature review and rare case of Leriche's syndrome.

    Science.gov (United States)

    Ye, Xuan; Rozen, Warren M; Alonso-Burgos, Alberto; Ashton, Mark W

    2012-11-01

    We undertook a review of the anatomical changes of "choke" vessels between the internal thoracic artery (ITA) and deep inferior epigastric artery (DIEA), as highlighted by a case of aortoiliac occlusive disease (Leriche's syndrome), and discuss the physiological concepts observed with regard to surgical delay procedures within the abdominal wall performed prior to abdominal cutaneous free flaps and coronary artery bypass grafting. Computed tomographic angiography (CTA) was undertaken on a patient with a rare case of Leriche's syndrome and a literature review of over 200 references on the anatomy, physiology and clinical uses of choke vessels in the abdominal wall was undertaken. The CTA demonstrated that in patients with Leriche's syndrome, there is a marked dilatation of all ITA-DIEA pathways and increased flow through choke vessels. If these changes can be surgically replicated in the form of a delay procedure for patients seeking to undergo autologous breast construction, this could improve the outcomes of abdominal cutaneous free flaps and coronary artery bypass grafting. We accordingly propose three surgical methods for augmenting blood flow to the abdominal wall: a) ligation of the DIEA; b) ligation of the distal ITA; and c) creation of an arterio-venous fistulae in the DIEA. Our review of the literature confirmed the viability of these propositions. The dilatation of choke vessels in response to increased haemodynamic stress may thus be utilised to enhance blood supply to tissues prior to transfer and can be achieved through simple and minimally invasive methods. Copyright © 2012 Wiley Periodicals, Inc.

  18. Sturge-Weber syndrome.

    Science.gov (United States)

    Comi, Anne M

    2015-01-01

    Sturge-Weber syndrome is the third most common neurocutaneous disorder, after neurofibromatosis and tuberous sclerosis, and impacts approximately 1 in 20000 live births. Sturge-Weber syndrome is not inherited, but rather occurs exclusively sporadically, in both males and females and in all races and ethnic backgrounds. Sturge-Weber syndrome presents at birth with a capillary malformation on the face (port-wine birthmark) with later diagnosis of abnormal vasculature in the eye and the brain which result in a range of complications. The underlying somatic mosaic mutation causing both Sturge-Weber syndrome and isolated port-wine birthmarks was recently discovered and is an activating mutation in GNAQ. When a newborn presents with a facial port-wine birthmark on the upper face, that child has a 15-50% risk of developing Sturge-Weber syndrome brain and/or eye involvement, depending on the extent of the birthmark, and close monitoring and appropriate screening is essential for early diagnosis and optimal treatment. Treatment options include laser therapy for lightening of the birthmark, eye drops and surgery for glaucoma management, and aggressive anticonvulsant treatment, low dose aspirin, and neurosurgery where necessary. Future possible treatments based upon new knowledge of the somatic mutation and downstream pathways are currently being considered and studied. © 2015 Elsevier B.V. All rights reserved.

  19. Vascular Cures

    Science.gov (United States)

    ... is the first national program to bring the power of the patient to vascular research and care. ... Our recent national Vascular Research Summit brought together leaders from 31 institutions to generate collaborative projects for ...

  20. The Vascular Depression Hypothesis: Mechanisms Linking Vascular Disease with Depression

    Science.gov (United States)

    Taylor, Warren D.; Aizenstein, Howard J.; Alexopoulos, George S.

    2013-01-01

    The ‘Vascular Depression’ hypothesis posits that cerebrovascular disease may predispose, precipitate, or perpetuate some geriatric depressive syndromes. This hypothesis stimulated much research that has improved our understanding of the complex relationships between late-life depression (LLD), vascular risk factors, and cognition. Succinctly, there are well-established relationships between late-life depression, vascular risk factors, and cerebral hyperintensities, the radiological hallmark of vascular depression. Cognitive dysfunction is common in late-life depression, particularly executive dysfunction, a finding predictive of poor antidepressant response. Over time, progression of hyperintensities and cognitive deficits predicts a poor course of depression and may reflect underlying worsening of vascular disease. This work laid the foundation for examining the mechanisms by which vascular disease influences brain circuits and influences the development and course of depression. We review data testing the vascular depression hypothesis with a focus on identifying potential underlying vascular mechanisms. We propose a disconnection hypothesis, wherein focal vascular damage and white matter lesion location is a crucial factor influencing neural connectivity that contributes to clinical symptomatology. We also propose inflammatory and hypoperfusion hypotheses, concepts that link underlying vascular processes with adverse effects on brain function that influence the development of depression. Testing such hypotheses will not only inform the relationship between vascular disease and depression but also provide guidance on the potential repurposing of pharmacological agents that may improve late-life depression outcomes. PMID:23439482

  1. Supravalvular aortic stenosis associated to infectious endocarditis and cerebral vascular disease in a patient with Williams-Beuren Syndrome.

    Science.gov (United States)

    De Rubens Figueroa, Jesús; Marhx, Alfonso; López Terrazas, Javier; Palacios Macedo, Alexis

    2015-01-01

    The Williams-Beuren syndrome is a rare genetic disease characterized by: (a) typical facial features; (b) psychomotor retardation with a specific neurocognitive profile; (c) cardiovascular condition and (d) likely transient hypocalcemia in infancy. The objective of this study was to describe the clinic evolution and diagnosis of patient with this syndrome that was associated with endocarditis caused by Streptococcus parasanguis in the ascending aorta and an aneurism located in the fronto-temporal area, which produced a parenchymal hematoma in the left lobe, and subarachnoid hemorrhage. He was treated with ceftriaxone and dicloxacillin. Then we proceeded to correct the aneurysm and perform vegetation resection in aortic arteries with supravalvular aortic stenosis correction. The evolution after one year has been favorable and is currently without neurologic sequelae. A 5-year-old male patient presented a diagnosis of supravalvular aortic stenosis. After cardiac catheterization was performed, he presented a fever and right side paresis. The echocardiogram showed multiple vegetations in the ascendant aortic arch and the supraortic arteries. The blood cultures reported S. parasanguis. The magnetic resonance showed a subarachnoid hemorrhage with an aneurysm and a hematoma. Copyright © 2014 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  2. Garcia-Hafner-Happle syndrome: A case report and review of a rare sub-type of epidermal nevus syndrome.

    Science.gov (United States)

    Desai, Soaham Dilip; Vora, Rita; Bharani, Sheela

    2014-01-01

    Garcia-Hafner-Happle syndrome, also known as Fibroblast growth factor receptor 3 epidermal nevus syndrome, is a new neurocutaneous phenotype, which has been identified in 2008 by Garcı'a-Vargas et al. The disorder is caused by a mosaic R248C mutation of the FGFR3 gene, which is characterized by a keratinocytic epidermal nevus, acanthosis nigricans, and neurological abnormalities like seizures, intellectual impairment, cortical atrophy, and underdevelopment of corpus callosum. The epidermal nevus syndromes represent a group of distinct disorders in which an epidermal nevus is associated with abnormalities in other organ systems like central nervous system, cardiovascular system, genitourinary system, eyes, and bone. Recently, nine well-defined different epidermal nevus syndromes (ENSs) have been identified on clinical, histopathologic, and molecular basis. We present here the details of a patient with the clinical features and skin biopsy findings suggestive of Garcia-Hafner-Happle syndrome.

  3. Dissociating Statistically-Determined Alzheimer's Disease/Vascular Dementia Neuropsychological Syndromes Using White and Gray Neuroradiological Parameters.

    Science.gov (United States)

    Price, Catherine C; Tanner, Jared J; Schmalfuss, Ilona M; Brumback, Babette; Heilman, Kenneth M; Libon, David J

    2015-01-01

    There is remarkable heterogeneity in clinical Alzheimer's disease (AD) or vascular dementia (VaD). 1) To statistically examine neuropsychological data to determine dementia subgroups for individuals clinically diagnosed with AD or VaD and then 2) examine group differences in specific gray/white matter regions of interest. A k-means cluster analysis requested a 3-group solution from neuropsychological data acquired from individuals diagnosed clinically with AD/VaD. MRI measures of hippocampal, caudate, ventricular, subcortical lacunar infarction, whole brain volume, and leukoaraiosis (LA) were analyzed. Three regions of LA volumes were quantified and these included the periventricular (5 mm around the ventricles), infracortical (5 mm beneath the gray matter), and deep (between periventricular and infracortical) regions. Cluster analysis sorted AD/VaD patients into single domain amnestic (n = 41), single-domain dysexecutive (n = 26), and multi-domain (n = 26) phenotypes. Multi-domain patients exhibited worst performance on language tests; however, multi-domain patients were equally impaired on memory tests when compared to amnestic patients. Statistically-determined groups dissociated using neuroradiological parameters: amnestic and multi-domain groups presented with smaller hippocampal volume while the dysexecutive group presented with greater deep, periventricular, and whole brain LA. Neither caudate nor lacunae volume differed by group. Caudate nucleus volume negatively correlated with total LA in the dysexecutive and multi-domain groups. There are at least three distinct subtypes embedded within patients diagnosed clinically with AD/VaD spectrum dementia. We encourage future research to assess a) the neuroradiological substrates underlying statistically-determined AD/VaD spectrum dementia and b) how statistical modeling can be integrated into existing diagnostic criteria.

  4. Somatic GNAQ Mutation is Enriched in Brain Endothelial Cells in Sturge-Weber Syndrome.

    Science.gov (United States)

    Huang, Lan; Couto, Javier A; Pinto, Anna; Alexandrescu, Sanda; Madsen, Joseph R; Greene, Arin K; Sahin, Mustafa; Bischoff, Joyce

    2017-02-01

    Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder characterized by facial and extracraniofacial capillary malformations and capillary-venule malformations in the leptomeninges. A somatic mosaic mutation in GNAQ (c.548G>A; p.R183Q) was found in SWS brain and skin capillary malformations. Our laboratory showed endothelial cells in skin capillary malformations are enriched for the GNAQ mutation. The purpose of this study is to determine whether the GNAQ mutation is also enriched in endothelial cells in affected SWS brain. Two human SWS brain specimens were fractionated by fluorescence-activated cell sorting into hematopoietic (CD45), endothelial (CD31, VE-Cadherin, and vascular endothelial growth factor receptor 2), and perivascular (platelet-derived growth factor receptor beta) cells and cells negative for all markers. The sorted cell populations were analyzed for GNAQ p.R183Q mutation by droplet digital polymerase chain reaction. SWS patient-derived brain endothelial cells were selected by anti-CD31-coated magnetic beads and cultured in endothelial growth medium in vitro. The GNAQ p.R183Q mutation was present in brain endothelial cells in two SWS specimens, with mutant allelic frequencies of 34.7% and 24.0%. Cells negative for all markers also harbored the GNAQ mutation. The mutant allelic frequencies in these unidentified cells were 9.2% and 8.4%. SWS patient-derived brain endothelial cells with mutant allelic frequencies of 14.7% and 21% survived and proliferated in vitro. Our study provides evidence that GNAQ p.R183Q mutation is enriched in endothelial cells in SWS brain lesions and thereby reveals endothelial cells as a source of aberrant Gαq signaling. This will help to understand the pathophysiology of SWS, to discover biomarkers for predicting cerebral involvement, and to develop therapeutic targets to prevent neurological impairments in SWS. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. A Rare Cause of Pheochromocytoma; Neurofibromatosis Type 1-Noonan Syndrome

    Directory of Open Access Journals (Sweden)

    Mazhar Müslüm Tuna

    2014-09-01

    Full Text Available Neurofibromatosis (NF Type 1 (NF-1 is an autosomal dominant disease with a prevalence of about 1/3000. NF-1 is a neurocutaneous syndrome characterized by cafe au lait macules, neurofibroma, optic glioma, lisch nodules, and symptoms involving other systems. Noonan syndrome (NS is a clinically heterogeneous disorder predominantly characterized by dysmorphic facial features, congenital heart disease, proportionate post-natal short stature, neck abnormalities, and chest deformities. NF-NS is a very rare overlapping syndrome sharing many features of both syndromes. Coexistence of pheochromocytoma, which can be life-threatening if not treated properly, is also a very rare complication of this disorder. Here, we report a patient who was admitted with a mass in the right upper quadrant and was diagnosed with pheochromocytoma and NFNS. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 227-31

  6. Relationship between adipokines, inflammation, and vascular reactivity in lean controls and obese subjects with metabolic syndrome Relação entre as adipocinas, inflamação e reatividade vascular em controles magros e pacientes obesos com síndrome metabólica

    Directory of Open Access Journals (Sweden)

    Luciana Bahia

    2006-10-01

    Full Text Available PURPOSE: Metabolic syndrome is an important risk factor for cardiovascular disease. Adipokines interfere with insulin action and endothelial cell function. We investigated the relationship among adipokines, metabolic factors, inflammatory markers, and vascular reactivity in obese subjects with metabolic syndrome and lean controls. METHODS: Cross-sectional study of 19 obese subjects with metabolic syndrome and 8 lean volunteers evaluated as controls. Vascular reactivity was assessed by venous occlusion pletysmography measuring braquial forearm blood flow (FBF and vascular resistance (VR responses to intra-arterial infusions of endothelium-dependent (acetylcholine-Ach and independent (sodium nitroprusside-SNP vasodilators. Blood samples were obtained to evaluate C reactive protein (CRP, plasminogen activator inhibitor 1 (PAI-1, fibrinogen, adiponectin, resistin, and lipid profile. Patients were classified with regard to insulin resistance through the HOMA-IR index. RESULTS: PAI-1, CRP and fibrinogen were higher and adiponectin was lower in metabolic syndrome subjects compared to controls. Metabolic syndrome subjects had impaired vascular reactivity. Adiponectin and PAI-1 were associated with insulin, HOMA-IR, triglycerides, and HDLc; and resistin with CRP. Adiponectin was associated with VR after Ach in the pooled group and resistin with D FBF after Ach in the metabolic syndrome group. CONCLUSION: Metabolic syndrome subjects exhibited low levels of adiponectin and high levels of CRP, fibrinogen, and PAI-1. Adiponectin and PAI-1 correlated with insulin resistance markers. Adiponectin and resistin correlated with vascular reactivity parameters. An adipocyte-endothelium interaction might be an important mechanism of inflammation and vascular dysfunction.A Síndrome Metabólica é um importante fator de risco para doenças cardiovasculares. As adipocinas interferem com a ação da insulina e com a função endotelial. OBJETIVO: Investigar a rela

  7. Vascular pathology in the throwing athlete.

    Science.gov (United States)

    Dugas, J R; Weiland, A J

    2000-08-01

    Vascular pathology in the upper extremity of a throwing athlete comprises a spectrum of serious disorders apt to threaten the patient's career and the viability of the involved parts. Such pathology includes digital vessel thrombosis, proximal thrombosis with distal embolization, vessel aneurysm, and vessel compression, such as in thoracic outlet syndrome and quadrilateral space syndrome. This article provides a description of vascular disorders prone to result from sports activities and a review of published data relevant to throwing athletes. Recognition of vascular compromise as a cause for dead arm syndrome or painful digital dysfunction among athletes is essential to prevent the grave consequences of progressive ischemia.

  8. The type of variants at the COL3A1 gene associates with the phenotype and severity of vascular Ehlers–Danlos syndrome

    Science.gov (United States)

    Frank, Michael; Albuisson, Juliette; Ranque, Brigitte; Golmard, Lisa; Mazzella, Jean-Michael; Bal-Theoleyre, Laurence; Fauret, Anne-Laure; Mirault, Tristan; Denarié, Nicolas; Mousseaux, Elie; Boutouyrie, Pierre; Fiessinger, Jean-Noël; Emmerich, Joseph; Messas, Emmanuel; Jeunemaitre, Xavier

    2015-01-01

    Vascular Ehlers–Danlos syndrome (vEDS) is a rare and severe autosomal dominant disorder caused by variants at the COL3A1 gene. Clinical characteristics and course of disease of 215 molecularly proven patients (146 index cases and 69 relatives) were analysed. We found 126 distincts variants that were divided into five groups: (1) Glycine substitutions (n=71), (2) splice-site and in-frame insertions–deletions (n=36), (3) variants leading to haplo-insufficiency (n=7), (4) non-glycine missense variants within the triple helix (n=4 variants), and (5) non-glycine missense variants or in-frame insertions–deletions, in the N- or C-terminal part of the protein (n=8). Overall, our cohort confirmed the severity of the disease with a median age at first complication of 29 years (IQR 22–39), the most frequent being arterial (48%) and digestive (24%) ruptures. Groups 2 and 1 were significantly more severe than groups 3–5, with extreme median ages at first major complication of 23–47 years. Patients of groups 3–5 had a less typical phenotype and remarkably absence of digestive events. The distribution of glycine-replacing amino acids was strongly biased towards more destabilizing residues of the collagen assembly. Thus the natural course of vEDS and the clinical phenotype of patients are influenced by the type of COL3A1 variant. This study also confirms that patients with variants located in the C- and N-termini or leading to haplo-insufficiency have milder course of the disease and less prevalent diagnostic criteria. These findings may help refine diagnostic strategy, genetic counselling and clinical care. PMID:25758994

  9. Early vascular healing after titanium-nitride-oxide-coated stent versus platinum-chromium everolimus-eluting stent implantation in patients with acute coronary syndrome.

    Science.gov (United States)

    Varho, Ville; Kiviniemi, Tuomas O; Nammas, Wail; Sia, Jussi; Romppanen, Hannu; Pietilä, Mikko; Airaksinen, Juhani K; Mikkelsson, Jussi; Tuomainen, Petri; Perälä, Anssi; Karjalainen, Pasi P

    2016-07-01

    Data on early vascular healing response of novel stent designs are scarce. In this randomized prospective trial, we sought to compare early neointimal coverage of cobalt-chromium-based titanium-nitride-oxide-coated bioactive stents (CoCr-BAS) versus platinum-chromium everolimus-eluting stents (PtCr-EES) at 2-month follow-up in patients with acute coronary syndrome (ACS). Forty patients with ACS were randomized to receive either CoCr-BAS (n = 19) or PtCr-EES (n = 21). Neointimal strut coverage and strut apposition were examined by optical coherence tomography; and coronary flow reserve (CFR), fractional flow reserve (FFR) and index of microcirculatory resistance (IMR) were assessed using a coronary pressure wire at 2 months. Two patients in the PtCr-EES underwent OCT out of the time frame of the study, and were excluded from analysis. At 63 ± 8 days, 302 cross-sections (3412 struts) were analysed in the CoCr-BAS group, and 324 cross-sections (3460 struts) in the PtCr-EES group. Median [IQR] neointimal thickness was 203 [108] µm and 42.2 [41] µm for CoCr-BAS and PtCr-EES, respectively (p  0.05 for all). CoCr-BAS showed earlier and more adequate neointimal coverage of struts at 2 months, compared with PtCr-EES, but with more neointimal hyperplasia. Functional healing as assessed by CFR, FFR, and IMR was similar between the two stent arms.

  10. After acute coronary syndrome, diabetic patients with peripheral vascular disease remain at high risk of cardiovascular events despite secondary prevention measures.

    Science.gov (United States)

    Lafitte, Marianne; Barandon, Laurent; Pucheu, Yann; Pillois, Xavier; Gin, Henri; Bonnet, Jacques; Couffinhal, Thierry

    2010-02-01

    Peripheral vascular disease (PVD) is associated with a high risk of cardiovascular events after an acute coronary syndrome (ACS). The impact of suboptimal risk-factor control and drug prescription on morbidity and mortality rates in patients with PVD following an ACS remains to be established. To assess whether a global atherosclerosis management programme and optimal secondary prevention could benefit high-risk PVD patients after an ACS. A total of 851 ACS patients underwent an intensified intervention focusing on evaluating risk factors and atherosclerosis lesions, and on optimizing treatment and education. We compared its impact on long-term risk factors, medication observance and cardiovascular outcomes in patients with coronary artery disease (CAD) alone (n=715, 84.0%) and with both CAD and PVD (n=136). At a median follow-up of 18.6months, both groups reached recommended secondary prevention goals and showed no significant differences in rates of drug prescription. PVD was not associated with minor cardiovascular events (hazard ratio [HR] 1.32, 95% confidence interval [CI] 0.57-3.02) but remained independently associated with major (HR 2.15, 95% CI 1.12-4.13) and total (HR 1.76, 95% CI 1.05-2.93) cardiovascular events. Compared to patients with CAD alone, this risk was significantly higher in CAD patients with both PVD and diabetes (HR 2.87, 95% CI 1.52-5.43), but not in PVD patients without diabetes (HR 1.35, 95% CI 0.71-2.56) or diabetic patients without PVD (HR 1.11, 95% CI 0.68-1.81). Despite optimization of risk-factor control and drug prescription after ACS, patients with both PVD and diabetes carry a 2.9-fold higher risk of cardiovascular events at 18-month follow-up versus patients with CAD alone. This excess risk was not significant in PVD patients without diabetes or in diabetic patients without PVD.

  11. [Relationship between plasma vascular endothelial growth factor and tumor necrosis factor-alpha and obstructive sleep apnea hypopnea syndrome in children].

    Science.gov (United States)

    Li, Jian; Li, Chuang; Chai, Liping; Gong, Weichi

    2014-01-01

    To investigate the relation of plasma vascular endothelial growth factor(VEGF) and tumor necrosis factor-α (TNF-α) with obstructive sleep apnea-hypopnea syndrome(OSAHS) in children. Eighty children were recruited from October 2008 to March 2009, including 60 children with snoring and 20 healthy children without snoring as control. Plasma VEGF or TNF-α concentration was measured by enzyme-linked immunosorbent assay (ELISA) respectively. Sixty children with snoring underwent an overnight polysomnography test their PSG data, including whole night mean saturation (MSaO2), lowest oxygen saturation (LSaO2), desaturation cumulate time/total sleep time (DCT/TST), oxygen desaturation index 3 (ODI3), apnea-hypopnea index (AHI), obstructive apnea index (OAI), were collected and analysed. SPSS 13.0 software was used to analyze the data. The levels of plasma VEGF and TNF-α in children with OSAHS(540.45 pg/ml and 311.94 pg/ml) were higher than those in children with snoring alone (234.45 pg/ml and 97.55 pg/ml) or those in healthy children (259.80 pg/ml and 120.70 pg/ml), with statistically significant differences(HC value:14.176 and 15.571, P 0.05). The differences in plasma VEGF or TNF-α levels between children with moderate and severe hypoxemia and children with mild hypoxemia were not statistically significant (P > 0.05). Spearman rank correlation analysis showed no significant correlation between plasma level of VEGF or TNF-α and LSaO2, MSaO2, ODI3, DCT/TST, OAI, AHI or BMI (r values were 0.05). Plasma levels of VEGF and TNF-α increase in children with OSAHS.

  12. Sturge-Weber Syndrome: A Review.

    Science.gov (United States)

    Higueros, E; Roe, E; Granell, E; Baselga, E

    2017-06-01

    Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder caused by a somatic activating mutation in GNAQ; it affects 1 in every 20,000 to 50,000 newborns. It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. Seizures are the most common neurological manifestation and typically present in the first months of life. Glaucoma may be present at birth or develop later. Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Standard treatment for Sturge-Weber syndrome includes laser treatment for the Port-wine stain, anticonvulsants, and medical or surgical treatment for the glaucoma. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma. Copyright © 2016 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. A Turkish family with Sjögren-Larsson syndrome caused by a novel ALDH3A2 mutation

    Directory of Open Access Journals (Sweden)

    Faruk Incecik

    2013-01-01

    Full Text Available Sjögren-Larsson syndrome (SLS is an inherited neurocutaneous disorder caused by mutations in the aldehyde dehydrogenase family 3 member A2 (ALDH3A2 gene that encodes fatty aldehyde dehydrogenase. Affected patients display ichthyosis, mental retardation, and spastic diplegia. More than 70 mutations in ALDH3A2 have been discovered in SLS patients. We diagnosed two brothers age of 12 and 20 years with characteristic features of this rare syndrome. Magnetic resonance imaging showed demyelinating disease in both of them. We described a novel homozygous, c. 835 T > A (p.Y279N mutation in exon 6 in two patients.

  14. Vascular Anomalies Encountered During Pancreatoduodenectomy: Do They Influence Outcomes?

    National Research Council Canada - National Science Library

    Shukla, Parul J; Barreto, Savio G; Kulkarni, Aniruddha; Nagarajan, Ganesh; Fingerhut, Abe

    2010-01-01

    .... Other vascular abnormalities such as replaced common hepatic artery with a hepatomesenteric trunk and celiomesenteric trunk and arcuate ligament syndrome leading to celiac artery stenosis are also...

  15. Ehlers-Danlos Syndrome

    Science.gov (United States)

    ... skinned people, the underlying blood vessels are very visible through the skin. Ehlers-Danlos syndrome, vascular type, ... history of Ehlers-Danlos syndrome and you're thinking about starting a family, you may benefit from ...

  16. A content analysis of cognitive and affective uses of patient support groups for rare and uncommon vascular diseases: comparisons of may thurner, thoracic outlet, and superior mesenteric artery syndrome.

    Science.gov (United States)

    Walker, Kimberly K

    2015-01-01

    Rare disease patients are the predominant group of patients who are now connecting online to patient support groups, yet research on their uses of support groups has received little attention. This is a content analysis of three vascular diseases of differing degrees of rarity. Wall posts from Facebook patient support groups for May Thurner syndrome, thoracic outlet syndrome, and superior mesenteric artery syndrome were analyzed over a period of two years. Using Uses and Gratifications as the theoretical framework, the study purpose was to assess how variations in health condition and rarity of condition affect online support group user needs. Results indicated common main cognitive and affective uses across conditions, indicating a consistent pattern of needs communicated by all patients. However, there were nuanced differences in subcategories of cognitive and affective uses between the most and least rare disorders, which inform areas for tailored support mechanisms. Additionally, these vascular patients used their respective support groups primarily for cognitive reasons, especially for the rarest conditions, which informs of basic medical informational needs these patients face related to tests, treatment, surgery, and diagnoses.

  17. Pediatric central nervous system vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

    2015-09-15

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  18. Vascular ring

    DEFF Research Database (Denmark)

    Schmidt, Anne Mette S; Larsen, Signe H; Hjortdal, Vibeke E

    2018-01-01

    BACKGROUND: Vascular ring is a rare cause of recurrent respiratory infections, dysphagia and stridor. Surgical repair is considered safe but the long-term outcomes are unclear. The purpose of this study was to investigate the mortality and morbidity following vascular ring surgery in a single...... age of 1.4 years (range 0.008-64 years) were operated for vascular ring. Median follow-up was 6.8 years (range 2.4-34 years). Presenting symptoms were stridor (52%), dysphagia or vomiting (52%) and recurrent respiratory infections (48%). There were no early or late deaths. Three months postoperatively...

  19. Vascular anomalies

    Directory of Open Access Journals (Sweden)

    Murthy Jyotsna

    2005-01-01

    Full Text Available Management of vascular anomalies is an emerging multidisciplinary, super-specialisation field involving several surgical, medical and radiological specialties. Over the years, development in this field has been limited because of complex nomenclature and lack of consensus on the best practice for treatment of some of the more complex vascular anomalies. It was only in 1996 that the International Society of the Study of Vascular Anomalies defined nomenclature for the anomalies and gave clear guidelines on management, allowing for improved clinical practices. As in all fields of clinical medicine, the correct diagnosis of the vascular anomalies is essential to choose the appropriate treatment. This paper gives clear guidelines for diagnosis, understanding of the anomalies and discusses their management.

  20. Vascular Dementia

    OpenAIRE

    Maria Alekseyevna Cherdak; O V Uspenskaya

    2015-01-01

    This is the author accepted manuscript. The final version is available from Elsevier via http://dx.doi.org/10.1016/S0140-6736(15)00463-8 Vascular dementia is one of the most common causes of dementia after Alzheimer's disease, causing around 15% of cases. However, unlike Alzheimer's disease, there are no licensed treatments for vascular dementia. Progress in the specialty has been difficult because of uncertainties over disease classification and diagnostic criteria, controversy over the e...

  1. The microcephaly-capillary malformation syndrome in two brothers with novel clinical features.

    Science.gov (United States)

    Pavlović, Milen; Neubauer, David; Al Tawari, Asma; Heberle, Lada Cindro

    2014-10-01

    Microcephaly-capillary malformation syndrome is a newly described neurocutaneous entity that is characterized by congenital and progressive microcephaly, intractable epilepsy, profound developmental delay, multiple small capillary malformations on the skin, and poor somatic growth. Recently, mutations in the STAMBP gene have been identified as causative in the pathogenesis of this syndrome. We describe two brothers (ages 7 and 12 years) from consanguineous parents of Saudi ancestry. Along with the established main clinical features of this syndrome, these boys exhibited certain novel and distinctive phenotypic features (congenital hypothyroidism and autistic-like behavior with intermittent repetitive hand-flapping movements). Genetic studies revealed the presence of homozygous pathogenic STAMPB mutation. This report presents the longest follow-up of patients with microcephaly-capillary syndrome so far reported and emphasize the syndrome's phenotype variability. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Vascular Access Procedures

    Science.gov (United States)

    ... News Physician Resources Professions Site Index A-Z Vascular Access Procedures A vascular access procedure inserts a flexible, ... the limitations of Vascular Access Procedures? What are Vascular Access Procedures? A vascular access procedure involves the insertion ...

  3. Vascular Access for Hemodialysis

    Science.gov (United States)

    ... Adequacy Eating & Nutrition for Hemodialysis Vascular Access for Hemodialysis What is a vascular access? A vascular access ... Set Up the Vascular Access Well before Starting Hemodialysis Patients should set up a vascular access well ...

  4. What Is Vascular Disease?

    Science.gov (United States)

    ... Policy What Is Vascular Disease? What Is Vascular Disease? Vascular disease is any abnormal condition of the blood ... Privacy Policy × Your ticket for the: What Is Vascular Disease? Title What Is Vascular Disease? USD Close Print

  5. Proteus Syndrome with Arteriovenous Malformation

    Directory of Open Access Journals (Sweden)

    Ali Asilian

    2017-01-01

    Full Text Available Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. We report a case of a 10-year-old boy with confirmed Proteus syndrome characterized by high flow vascular malformation (arteriovenous [AV] malformation unlike the usual vascular malformations seen in this syndrome. This case adds a new perspective to the established clinical findings of the Proteus syndrome.

  6. Cranial nerve vascular compression syndromes of the trigeminal, facial and vago-glossopharyngeal nerves: comparative anatomical study of the central myelin portion and transitional zone; correlations with incidences of corresponding hyperactive dysfunctional syndromes.

    Science.gov (United States)

    Guclu, Bulent; Sindou, Marc; Meyronet, David; Streichenberger, Nathalie; Simon, Emile; Mertens, Patrick

    2011-12-01

    The aim of this study was to evaluate the anatomy of the central myelin portion and the central myelin-peripheral myelin transitional zone of the trigeminal, facial, glossopharyngeal and vagus nerves from fresh cadavers. The aim was also to investigate the relationship between the length and volume of the central myelin portion of these nerves with the incidences of the corresponding cranial dysfunctional syndromes caused by their compression to provide some more insights for a better understanding of mechanisms. The trigeminal, facial, glossopharyngeal and vagus nerves from six fresh cadavers were examined. The length of these nerves from the brainstem to the foramen that they exit were measured. Longitudinal sections were stained and photographed to make measurements. The diameters of the nerves where they exit/enter from/to brainstem, the diameters where the transitional zone begins, the distances to the most distal part of transitional zone from brainstem and depths of the transitional zones were measured. Most importantly, the volume of the central myelin portion of the nerves was calculated. Correlation between length and volume of the central myelin portion of these nerves and the incidences of the corresponding hyperactive dysfunctional syndromes as reported in the literature were studied. The distance of the most distal part of the transitional zone from the brainstem was 4.19  ±  0.81 mm for the trigeminal nerve, 2.86  ±  1.19 mm for the facial nerve, 1.51  ±  0.39 mm for the glossopharyngeal nerve, and 1.63  ±  1.15 mm for the vagus nerve. The volume of central myelin portion was 24.54  ±  9.82 mm(3) in trigeminal nerve; 4.43  ±  2.55 mm(3) in facial nerve; 1.55  ±  1.08 mm(3) in glossopharyngeal nerve; 2.56  ±  1.32 mm(3) in vagus nerve. Correlations (p  nerves and incidences of the corresponding diseases. At present it is rather well-established that primary trigeminal neuralgia, hemifacial spasm and vago

  7. Vascular dementia.

    Science.gov (United States)

    O'Brien, John T; Thomas, Alan

    2015-10-24

    Vascular dementia is one of the most common causes of dementia after Alzheimer's disease, causing around 15% of cases. However, unlike Alzheimer's disease, there are no licensed treatments for vascular dementia. Progress in the specialty has been difficult because of uncertainties over disease classification and diagnostic criteria, controversy over the exact nature of the relation between cerebrovascular pathology and cognitive impairment, and the paucity of identifiable tractable treatment targets. Although there is an established relation between vascular and degenerative Alzheimer's pathology, the mechanistic link between the two has not yet been identified. This Series paper critiques some of the key areas and controversies, summarises treatment trials so far, and makes suggestions for what progress is needed to advance our understanding of pathogenesis and thus maximise opportunities for the search for new and effective management approaches. Copyright © 2015 Elsevier Ltd. All rights reserved.

  8. [Vascular trauma].

    Science.gov (United States)

    Furuya, T; Nobori, M; Tanaka, N

    1999-07-01

    Vascular trauma is essentially acute arterial obstruction, often combined with hemorrhage, fracture, and infection. It can be both life-threatening and limb-threatening and needs an emergency operation. In vascular trauma patient, multiple fracture and organ injury, such as brain, lung, liver, spleen, kidney, or gastrointestinal tract should be evaluated to decide treatment priority. When the pulse distal from the injured site is absent or diminished, vascular trauma is most likely and reconstruction should be accomplished within "the golden time (6-8 hours)". Intimal damage followed by platelet aggregation and thrombus formation will necessitate resection and repair of the site instead of simple thrombectomy. Although autogenous vein is the first choice, artificial graft can be implanted for short segment in non-infected field.

  9. Imaging evaluation of fetal vascular anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Koch, Bernadette L.; Laor, Tal [MLC 5031 Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Adams, Denise M. [Cincinnati Children' s Hospital Medical Center, Department of Pediatrics and Hemangioma and Vascular Malformation Center, Cincinnati, OH (United States); Gupta, Anita [Cincinnati Children' s Hospital Medical Center, Department of Pathology, Cincinnati, OH (United States); Lim, Foong-Yen [Cincinnati Children' s Hospital Medical Center, Pediatric Surgery and Fetal Center of Cincinnati, Cincinnati, OH (United States)

    2015-08-15

    Vascular anomalies can be detected in utero and should be considered in the setting of solid, mixed or cystic lesions in the fetus. Evaluation of the gray-scale and color Doppler US and MRI characteristics can guide diagnosis. We present a case-based pictorial essay to illustrate the prenatal imaging characteristics in 11 pregnancies with vascular malformations (5 lymphatic malformations, 2 Klippel-Trenaunay syndrome, 1 venous-lymphatic malformation, 1 Parkes-Weber syndrome) and vascular tumors (1 congenital hemangioma, 1 kaposiform hemangioendothelioma). Concordance between prenatal and postnatal diagnoses is analyzed, with further discussion regarding potential pitfalls in identification. (orig.)

  10. Monozygotic twins discordant for vascular malformations and dysregulated growth

    NARCIS (Netherlands)

    Oduber, Charlène E. U.; Bliek, Jet; van der Horst, Chantal M. A. M.; van Steensel, Maurice A. M.; Hennekam, Raoul C. M.

    2010-01-01

    There is a large group of disorders characterized by non-cranial vascular malformations and a dysregulated growth, of which the prototype may be Klippel-Trenaunay syndrome (KTS). The aetiology of KTS and vascular malformations-dysregulated growth (VM-DG) syndromes resembling KTS is obscure, but

  11. Systemic vascular phenotypes of Loeys-Dietz syndrome in a child carrying a de novo R381P mutation in TGFBR2: a case report

    OpenAIRE

    Uike, Kiyoshi; Matsushita, Yuki; Sakai, Yasunari; Togao, Osamu; Nagao, Michinobu; Ishizaki, Yoshito; Nagata, Hazumu; Yamamura, Kenichiro; Torisu, Hiroyuki; Hara, Toshiro

    2013-01-01

    Background Loeys?Dietz syndrome, also known as Marfan syndrome type II, is a rare connective tissue disorder caused by dominant mutations in transforming growth factor-beta receptors (TGFBR1 and 2). Case presentation We report a 7-year-old Japanese boy with Loeys?Dietz syndrome who carried a novel, de novo missense mutation in TGFBR2 (c.1142g?>?c, R381P). He showed dysmorphic faces and skeletal malformations that were typical in previous cases with Loeys-Dietz syndrome. The cardiac studies di...

  12. Contemporary vascular smartphone medical applications.

    Science.gov (United States)

    Carter, Thomas; O'Neill, Stephen; Johns, Neil; Brady, Richard R W

    2013-08-01

    Use of smartphones and medical mHealth applications (apps) within the clinical environment provides a potential means for delivering elements of vascular care. This article reviews the contemporary availability of apps specifically themed to major vascular diseases and the opportunities and concerns regarding their integration into practice. Smartphone apps relating to major vascular diseases were identified from the app stores for the 6 most popular smartphone platforms, including iPhone, Android, Blackberry, Nokia, Windows, and Samsung. Search terms included peripheral artery (arterial) disease, varicose veins, aortic aneurysm, carotid artery disease, amputation, ulcers, hyperhydrosis, thoracic outlet syndrome, vascular malformation, and lymphatic disorders. Forty-nine vascular-themed apps were identified. Sixteen (33%) were free of charge. Fifteen apps (31%) had customer satisfaction ratings, but only 3 (6%) had greater than 100. Only 13 apps (27%) had documented medical professional involvement in their design or content. The integration of apps into the delivery of care has the potential to benefit vascular health care workers and patients. However, high-quality apps designed by clinicians with vascular expertise are currently lacking and represent an area of concern in the mHealth market. Improvement in the quality and reliability of these apps will require the development of robust regulation. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. VASCULAR SURGERY

    African Journals Online (AJOL)

    Venous thromboembolism (VTE) is a complex and serious condition encompassing deep vein thrombosis (DVT) and pulmonary embolism (PE), usually in the lower extremities.1,2. Thromboses can result from venous stasis, vascular injury or hypercoagulability, and those involving the deep veins proximal to the knee are ...

  14. Parry-Romberg syndrome in an elderly male: A rare case report

    Directory of Open Access Journals (Sweden)

    Daljit Singh

    2017-07-01

    Full Text Available Progressive hemifacial atrophy (PHA of the face was first described by Caleb Hillier Parry in 1825 and Moritz Heinrich Romberg in 1846, hence the name Parry Romberg Syndrome was coined. It is a neurocutaneous syndrome characterized by progressive atrophy of the skin and tissues lying underneath like subcutaneous fat, muscle and bone. It is more common in females. Various etiologies have been proposed like autoimmune, hyperactivity of nervous system, infections etc., but none can fully explain its pathogenesis. Here we report a case of Parry-Romberg syndrome in a 70-year-old male which is the oldest case to present with this syndrome to the best of our knowledge.

  15. Síndrome metabólica na doença arterial coronariana e vascular oclusiva: uma revisão sistemática Metabolic syndrome in coronary artery and occlusive vascular diseases: a systematic review

    Directory of Open Access Journals (Sweden)

    Daniela Reis Elbert Farias

    2010-06-01

    Full Text Available Atualmente, a síndrome metabólica (SM se mostra altamente prevalente, sendo associada a fatores de risco para doenças crônicas não transmissíveis, tais como diabetes mellitus tipo 2, doenças ateroscleróticas e coronarianas. O objetivo desta revisão sistemática foi descrever os resultados de estudos que investigaram a associação da SM com a doença arterial coronariana e doenças vasculares oclusivas. Foi realizada a revisão sistemática com dados de estudos originais publicados entre 1999 e 2008, escritos em inglês ou português, utilizando-se as bases de dados Medline, Pubmed, Highwire Press e Science Direct. Foram incluídos artigos que fizeram o diagnóstico da SM através do critério do National Cholesterol Education Program - Adult Treatment Panel III (NCEP ATP III, 2001. Foram excluídos estudos realizados com animais, de suplementação e que realizaram administração oral ou endovenosa de qualquer substância, assim como aqueles de baixa qualidade metodológica e com amostra inicialmente heterogênea. Apesar da heterogeneidade entre os estudos, observou-se que indivíduos com SM apresentam maior probabilidade (risco = 2,13 de desenvolverem as doenças vasculares oclusivas, doença coronariana, diabetes mellitus e acidente vascular encefálico. Mudanças no estilo de vida, como práticas alimentares saudáveis, atividade física regular e a cessação do tabagismo devem ser incentivadas pelos profissionais da saúde a fim de minimizar as complicações e a morbimortalidade associada à SM.

  16. Is cutis verticis Gyrata-Intellectual Disability syndrome an underdiagnosed condition? A case report and review of 62 cases.

    Science.gov (United States)

    Tucci, Arianna; Pezzani, Lidia; Scuvera, Giulietta; Ronzoni, Luisa; Scola, Elisa; Esposito, Susanna; Milani, Donatella

    2017-03-01

    Cutis Verticis Gyrata-Intellectual Disability (CVG-ID) syndrome is a rare neurocutaneous syndrome characterized by intellectual disability and scalp folds and furrows that are typically absent at birth and are first noticed after puberty. First reported in 1893, the syndrome was mainly identified in subjects living in psychiatric institutions, where it was found to have a prevalence of up to 11.4%. Most patients were reported in the literature during the first half of the 20th century. CVG-ID is now a less reported and possibly under-recognized syndrome. Here, we report a patient with CVG-ID that was diagnosed using the novel approach of magnetic resonance imaging and we conduct a systematic review of all patients reported in the last 60 years, discussing the core clinical features of this syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  17. Update on Vascular Dementia.

    Science.gov (United States)

    Khan, Ayesha; Kalaria, Raj N; Corbett, Anne; Ballard, Clive

    2016-09-01

    Vascular dementia (VaD) is a major contributor to the dementia syndrome and is described as having problems with reasoning, planning, judgment, and memory caused by impaired blood flow to the brain and damage to the blood vessels resulting from events such as stroke. There are a variety of etiologies that contribute to the development of vascular cognitive impairment and VaD, and these are often associated with other dementia-related pathologies such as Alzheimer disease. The diagnosis of VaD is difficult due to the number and types of lesions and their locations in the brain. Factors that increase the risk of vascular diseases such as stroke, high blood pressure, high cholesterol, and smoking also raise the risk of VaD. Therefore, controlling these risk factors can help lower the chances of developing VaD. This update describes the subtypes of VaD, with details of their complex presentation, associated pathological lesions, and issues with diagnosis, prevention, and treatment. © The Author(s) 2016.

  18. [Heptopulmonary syndrome].

    Science.gov (United States)

    Cuadrado, Antonio; Díaz, Ainhoa; Iruzubieta, Paula; Salcines, José Ramón; Crespo, Javier

    2015-01-01

    Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  19. In vivo intrabursal administration of bioactive lipid sphingosine-1-phosphate enhances vascular integrity in a rat model of ovarian hyperstimulation syndrome.

    Science.gov (United States)

    Di Pietro, Mariana; Pascuali, Natalia; Scotti, Leopoldina; Irusta, Griselda; Bas, Diana; May, María; Tesone, Marta; Abramovich, Dalhia; Parborell, Fernanda

    2017-06-01

    Can the bioactive lipid sphingosine-1 phosphate (S1P) act as an endothelial barrier-enhancing molecule and, in turn, restore the vascular integrity and homoeostasis in a rat model of ovarian hyperstimulation syndrome (OHSS). In vivo administration of S1P may prevent the early onset of OHSS and decrease its severity. Although advances in the prediction and treatment of OHSS have been made, complete prevention has not been possible yet. S1P in follicular fluid from women at risk of developing OHSS are lower in comparison from women who are not at such risk and administration of S1P in an OHSS rat model decreases ovarian capillary permeability. We used an animal model that develops OHSS in immature Sprague-Dawley rats. The rats were randomly divided into three groups: the control group, which was injected with 10 IU of pregnant mare's serum gonadotropin (PMSG), and 10 IU of hCG 48 h later; the OHSS group, which was injected with excessive doses of PMSG (50 IU/day) for four consecutive days, followed by hCG; and the OHSS + S1P group, which was injected with the same doses of PMSG and hCG as the OHSS group and then treated with 5 μl S1P (1 mM) under the bursa of both ovaries, whereas the other groups of animals received the S1P vehicle. Rats were killed by decapitation 48 h after the hCG injection for ovary, endometrium and blood collection. The ovaries were weighed and then used for subsequent assays, while the serum was used for hormone assays. One of the ovaries from each rat (n = 6) was used for Western immunoblot and the other for immunohistochemical analysis. Statistical comparisons between groups were carried out. S1P administration reduced the ovarian weight (P < 0.05), and decreased the concentration of serum progesterone in the OHSS group compared to the OHSS group without treatment (P < 0.001). The percentage of antral follicles in the OHSS group was lower than that in the control group. S1P increased the percentage of antral follicles (P < 0.05) and

  20. The clinical usefulness of extravascular lung water and pulmonary vascular permeability index to diagnose and characterize pulmonary edema: a prospective multicenter study on the quantitative differential diagnostic definition for acute lung injury/acute respiratory distress syndrome.

    Science.gov (United States)

    Kushimoto, Shigeki; Taira, Yasuhiko; Kitazawa, Yasuhide; Okuchi, Kazuo; Sakamoto, Teruo; Ishikura, Hiroyasu; Endo, Tomoyuki; Yamanouchi, Satoshi; Tagami, Takashi; Yamaguchi, Junko; Yoshikawa, Kazuhide; Sugita, Manabu; Kase, Yoichi; Kanemura, Takashi; Takahashi, Hiroyuki; Kuroki, Yuichi; Izumino, Hiroo; Rinka, Hiroshi; Seo, Ryutarou; Takatori, Makoto; Kaneko, Tadashi; Nakamura, Toshiaki; Irahara, Takayuki; Saito, Nobuyuki; Watanabe, Akihiro

    2012-12-11

    Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is characterized by features other than increased pulmonary vascular permeability. Pulmonary vascular permeability combined with increased extravascular lung water content has been considered a quantitative diagnostic criterion of ALI/ARDS. This prospective, multi-institutional, observational study aimed to clarify the clinical pathophysiological features of ALI/ARDS and establish its quantitative diagnostic criteria. The extravascular lung water index (EVLWI) and the pulmonary vascular permeability index (PVPI) were measured using the transpulmonary thermodilution method in 266 patients with PaO2/FiO2 ratio ≤ 300 mmHg and bilateral infiltration on chest radiography, in 23 ICUs of academic tertiary referral hospitals. Pulmonary edema was defined as EVLWI ≥ 10 ml/kg. Three experts retrospectively determined the pathophysiological features of respiratory insufficiency by considering the patients' history, clinical presentation, chest computed tomography and radiography, echocardiography, EVLWI and brain natriuretic peptide level, and the time course of all preceding findings under systemic and respiratory therapy. Patients were divided into the following three categories on the basis of the pathophysiological diagnostic differentiation of respiratory insufficiency: ALI/ARDS, cardiogenic edema, and pleural effusion with atelectasis, which were noted in 207 patients, 26 patients, and 33 patients, respectively. EVLWI was greater in ALI/ARDS and cardiogenic edema patients than in patients with pleural effusion with atelectasis (18.5 ± 6.8, 14.4 ± 4.0, and 8.3 ± 2.1, respectively; P definitive diagnosis of ALI/ARDS (specificity, 0.90 to 0.95), and a value < 1.7 ruled out an ALI/ARDS diagnosis (specificity, 0.95). PVPI may be a useful quantitative diagnostic tool for ARDS in patients with hypoxemic respiratory failure and radiographic infiltrates. UMIN-CTR ID UMIN000003627.

  1. Fetal origin of vascular aging

    Directory of Open Access Journals (Sweden)

    Shailesh Pitale

    2011-01-01

    Full Text Available Aging is increasingly regarded as an independent risk factor for development of cardiovascular diseases such as atherosclerosis and hypertension and their complications (e.g. MI and Stroke. It is well known that vascular disease evolve over decades with progressive accumulation of cellular and extracellular materials and many inflammatory processes. Metabolic syndrome, obesity and diabetes are conventionally recognized as risk factors for development of coronary vascular disease (CVD. These conditions are known to accelerate ageing process in general and vascular ageing in particular. Adverse events during intrauterine life may programme organ growth and favour disease later in life, popularly known as, ′Barker′s Hypothesis′. The notion of fetal programming implies that during critical periods of prenatal growth, changes in the hormonal and nutritional milieu of the conceptus may alter the full expression of the fetal genome, leading to permanent effects on a range of physiological.

  2. The effect of prepregnancy obesity and sFlt-1-induced preeclampsia-like syndrome on fetal programming of adult vascular function in a mouse model.

    Science.gov (United States)

    Byers, Benjamin D; Betancourt, Ancizar; Lu, Fangxian; Hankins, Gary D V; Longo, Monica; Saade, George R; Bytautiene, Egle

    2009-04-01

    The purpose of this study was to test the hypothesis that prepregnancy obesity and soluble fms-like tyrosine kinase-1 (sFlt-1)-induced preeclampsia lead to altered vascular function in the offspring later in life. CD-1 female mice were placed on a low-fat (LF) or high-fat (HF) diet before mating. On day 8 of pregnancy, the HF mice were injected with adenovirus that carried either sFlt-1 (HF sFlt-1) or murine immunoglobulin G2alpha Fc fragment (HF mFc). LF dams received saline solution. After being weaned, all offspring were placed on a standard diet. At 3 months of age, the carotid artery was isolated for in vitro vascular reactivity studies. Among male offspring, the response to phenylephrine was significantly lower in the HF sFlt-1 group. The response to serotonin in males and to thromboxane in females was lower in the HF sFlt-1 and HF mFc groups. In females, the HF sFlt-1 and LF groups displayed less relaxation to acetylcholine. The response to phenylephrine was significantly lower in females than males in the HF mFc and LF groups. The response to thromboxane was significantly lower in the HF sFlt-1 females, compared with males. Prepregnancy obesity and preeclampsia alter fetal programming of adult vascular function. The mechanism is complex and gender specific.

  3. Epidermal nevus syndrome associated with anterior scleral staphyloma and ectopic bone and cartilaginous intraocular tissue.

    Science.gov (United States)

    Miyagawa, Yasuhiro; Nakazawa, Mitsuru; Kudoh, Takashi

    2010-01-01

    Epidermal nevus syndrome encompasses a group of congenital neurocutaneous anomalies characterized by epidermal nevi in association with cerebral, ocular, and skeletal abnormalities. We report herein the case of a Japanese girl with epidermal nevus syndrome associated with complex ocular choristoma and discuss the histopathological findings. A mass lesion was noted on the left eyeball of a newborn Japanese girl. The lesion appeared to be a scleral staphyloma. Linear and diffuse acanthoses were also apparent on the face. Skin biopsy revealed an epidermal nevus. Histopathological examination of the enucleated left eyeball demonstrated that extremely thin sclera adjacent to the corneal limbus resulted in anterior staphyloma, and ectopic osteocartilaginous tissues were present in the posterior sclera. Epidermal nevus syndrome associated with complex ocular choristoma was diagnosed. The anterior staphylomatous lesion observed in this case has not been reported previously.

  4. The LIPT-Study: On Risk Markers of Vascular Thrombosis in Polycystic Ovary Syndrome. A Randomized, Double-Blind, Placebo-Controlled Study of the Effect of Liraglutide

    DEFF Research Database (Denmark)

    Frøssing, Signe; Nylander, Malin; Kistorp, Caroline Michaela

    2015-01-01

    Overweight and insulin resistance (IR) are central pathogenic features of the Polycystic Ovary Syndrome (PCOS), and weight loss is the main treatment option. PCOS is also associated with signs of a chronic inflammation, activation of the coagulation system, defect endothelial function and increased...... arterial stiffness, all regarded as risk factors or markers for the development of cardiovascular disease. These factors are not taken into account in the definition of the syndrome, which is based on the 3 Rotterdam criteria. An uncertainty of the clinical risk of cardiovascular disease (CVD...

  5. Spinal Cord Vascular Disease

    Directory of Open Access Journals (Sweden)

    Abdoreza Ghoreishi

    2017-02-01

    Full Text Available The spinal cord is subject to many of the same vascular diseases that involve the brain, but its anatomy and embryology render it susceptible to some syndromes that do not have intracranial counterparts.The embryonic arterial supply to the spinal cord derives from intradural vessels that enter at each spinal level and divide to follow the dorsal and ventral roots. SPINAL CORD ISCHEMIA: The midthoracic levels of the spinal cord are traditionally considered to be the most vulnerable to compromise from hypoperfusion, but more recent evidence suggests that the lower thoracic cord is at greater risk . The actual prevalence of spinal cord infarction is unknown, but is generally cited as representing 1% to 2% of all central neurovascular events and 5% to 8% of all acute myelopathies. Weakness (100%, sensory loss (89%, back pain at onset (82%, and urinary complaints requiring catheterization (75% were the most common symptoms of cord ischemia at the time of presentation . Weakness most commonly affects both legs. Examination typically reveals flaccid paresis accompanied by diminished superficial and tendon reflexes below the level of the lesion. Preservation of strength and reflexes suggests the rare syndrome of posterior spinal artery territory infarction. Weakness most commonly affects both legs. Examination typically reveals flaccid paresis accompanied by diminished superficial and tendon reflexes below the level of the lesion. Preservation of strength and reflexes suggests the rare syndrome of posterior spinal artery territory infarction.   Aortic pathologies with regional hemodynamic compromise are the most common cause of spinal cord infarction, accounting for 30% to 40% of cases.                                                                                 The medical management of spinal cord ischemia is generally supportive and focused on reducing risk for

  6. Anaesthesia for vascular emergencies.

    Science.gov (United States)

    Ellard, L; Djaiani, G

    2013-01-01

    Patients presenting with vascular emergencies including acute aortic syndrome, ruptured thoracic or abdominal aortic aneurysms, thoracic aortic trauma and acute lower limb ischaemia have a high risk of peri-operative morbidity and mortality. Although anatomical suitability is not universal, endovascular surgery may improve mortality and the results of ongoing randomised controlled trials are awaited. Permissive hypotension pre-operatively should be the standard of care with the systolic blood pressure kept to 50-100 mmHg as long as consciousness is maintained. The benefit of local anaesthesia over general anaesthesia is not definitive and this decision should be tailored for a given patient and circumstance. Cerebrospinal fluid drainage for prevention of paraplegia is often impractical in the emergency setting and is not backed by strong evidence; however, it should be considered postoperatively if symptoms develop. We discuss the pertinent anaesthetic issues when a patient presents with a vascular emergency and the impact that endovascular repair has on anaesthetic management. Anaesthesia © 2012 The Association of Anaesthetists of Great Britain and Ireland.

  7. CLOVES syndrome.

    Science.gov (United States)

    Bloom, Jacob; Upton, Joseph

    2013-12-01

    A cohort of patients with overgrowth syndromes has been identified with congenital lipomatous overgrowth, dysregulated fat deposits, and mixed vascular malformations. The acronym CLOVES was given on a heuristic basis to stand for congenital lipomatous overgrowth (CLO), vascular malformation (V), epidermal nevi (E), and scoliosis and spinal deformities (S). These patients have upper limb anomalies with variable phenotypes. Although hand anomalies alone cannot make the diagnosis, the foot, truncal, cutaneous and spinal anomalies are particularly diagnostic. CLOVES syndrome has emerged as a distinct clinical entity diagnosed by clinical and radiographic examinations. The overgrowth pattern is now easily distinguished from other overgrowth syndromes. Copyright © 2013 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

  8. Further delineation of FKBP14-related Ehlers-Danlos syndrome: A patient with early vascular complications and non-progressive kyphoscoliosis, and literature review.

    Science.gov (United States)

    Dordoni, Chiara; Ciaccio, Claudia; Venturini, Marina; Calzavara-Pinton, Piergiacomo; Ritelli, Marco; Colombi, Marina

    2016-08-01

    FKBP14-related Ehlers-Danlos syndrome (EDS) is an extremely rare recessive connective tissue disorder described for the first time in 2012 by Baumann and coworkers. The causal gene, FKBP14, encodes a member of the F506-binding family of peptidyl-prolyl cis-trans isomerases. The paucity of patients described so far makes this disorder poorly defined at clinical level. Here, we report an additional pediatric patient, who is compound heterozygous for a recurrent and a novel FKBP14 mutation, and compare his phenotype with those available in literature. This evaluation confirms that kyphoscoliosis (either progressive or non-progressive), myopathy, joint hypermobility, and congenital hearing loss (sensorineural, conductive, or mixed) are the typical features of the syndrome. Since the patient showed a severe cardiovascular event in childhood and atlantoaxial instability, this report expands the phenotype of the disorder and the allelic repertoire of FKBP14. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  9. Compartment syndrome of thigh and lower leg with disruption of the popliteal vascular bundle after being run over by a 25-ton truck

    Directory of Open Access Journals (Sweden)

    Burghardt Rolf D

    2013-10-01

    Full Text Available 【Abstract】Compartment syndrome of the thigh is a rare condition, potentially resulting in devastating functional outcome. Increasing intracompartmental pressure which suppresses microcirculation and capillary perfusion may lead to cellular anoxia and muscle ischemia. The muscle compartments in the thigh have a more compliant fascia and blend anatomically into the open compartments of the pelvis, thus compensating higher volumes than the compartments in the lower leg. We present a previously unreported case in which the limb of a 36-year-old man was run over by a 25-ton truck. He presented with a sensomotor deficit in his left lower leg with full paralysis of the shank muscles and absence of all foot pulses. CT scan showed a huge haematoma in the thigh with active bleeding out of the popliteal artery into the haematoma which has already expanded into the muscle compartments of the lower leg. The limb had a disastrous compartment syndrome of the thigh and lower leg with disruption of the popliteal neurovascular bundle; however, no bones in the limb were fractured. A complete fasciotomy of all the lower limb muscle compartments was immediately performed. The artery was reconstructed with interposition of the smaller saphenous vein, which was already interrupted through the initial trauma. Key words: Compartment syndromes; Thigh; Hemorrhage; Popliteal artery; Peroneal nerve

  10. TAFRO Syndrome.

    Science.gov (United States)

    Igawa, Takuro; Sato, Yasuharu

    2018-02-01

    TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Det hepatopulmonale syndrome

    DEFF Research Database (Denmark)

    Eibye, Simone Bendix; Christensen, Erik

    2016-01-01

    The hepatopulmonary syndrome (HPS) - a complication of liver disease - seems to be underdiagnosed, probably because of decreased awareness. HPS consists of the triade liver disease, intrapulmonary vascular dilatation and as a consequence arterial hypoxaemia. No medical therapy has proven effective...

  12. Pathophysiology of Headaches with a Prominent Vascular Component

    Directory of Open Access Journals (Sweden)

    Juan A Pareja

    1996-01-01

    Full Text Available Vascular changes, whether preliminary or secondary, seem to accompany most headaches. The literature concerning pathophysiological mechanisms in headaches where vascular phenomena are a major, integral part, ie, migraine and cluster headache syndrome, is reviewed and the most common forms of headache associated with cerebrovascular disease are discussed. Emphasis is placed on the vascular phenomena and on the abundant hypotheses and theories regarding headache mechanisms. This review also presents alternative explanatory models, and compares the available anatomical, physiological and biochemical results.

  13. Intestinal vascular anomalies in children.

    Science.gov (United States)

    Frémond, B; Yazbeck, S; Dubois, J; Brochu, P; Garel, L; Ouimet, A

    1997-06-01

    Vascular anomalies are an uncommon cause of gastrointestinal bleeding in childhood. Confusing nomenclature has made objective comparisons of published cases difficult and has interfered with an established consensus regarding diagnosis and therapeutic modalities. The purpose of this study was to clarify the situation by reviewing the records of all children who had intestinal vascular anomalies who were referred to our institution from 1975 to 1995. Thirteen lesions were identified in nine children (five boys and four girls). The median age at clinical onset was 8 years. Only two patients presented with a complex syndrome (Klippel-Trenaunay, 1; Osler-Rendu-Weber, 1). Diagnosis, location, and extension of these anomalies was only possible by angiography, which indicated that seven patients had isolated venous malformations and two had arteriovenous malformations. Because the lesions did not involve the serosa, intraoperative localization was a major problem. The main findings were a few slightly dilated mesenteric veins. Treatment was conservative in four children and surgical in five. Pathological findings on resected bowel demonstrated dilated and abnormal veins in the mucosa and submucosa. Selective angiography should not be delayed in patients with gastrointestinal bleeding if results of all other investigations are negative. Because these lesions are rarely recognizable on operative inspection, precise preoperative angiographic localization of intestinal vascular anomalies is essential to allow for a safe and limited resection of the involved bowel segment. Based on a better understanding of the natural history of these lesions, a classification of vascular anomalies of intestines in children is proposed.

  14. Polycystic ovary syndrome and obesity do not affect vascular parameters related to early atherosclerosis in young women without glucose metabolism disturbances, arterial hypertension and severe abnormalities of lipid profile.

    Science.gov (United States)

    Barcellos, Cristiano Roberto Grimaldi; Lage, Silvia Helena Gelás; Rocha, Michelle Patrocínio; Hayashida, Sylvia Asaka Yamashita; Baracat, Edmund Chade; Romano, Angela; Brito, Vinicius Nahime; Marcondes, José Antonio Miguel

    2013-04-01

    The aim of this study was to evaluate the influence of polycystic ovary syndrome (PCOS) and obesity on vascular parameters related to early atherosclerosis (VP-EA) [brachial flow-mediated dilation (FMD), carotid intima-media thickness (CIMT) and carotid arterial compliance (CAC)] in women with minor cardiovascular risk factors (CVRFs). Twenty-five young women with PCOS and 23 eumenorrheic women matched for body mass index (BMI) were studied. The women were subdivided according to BMI and PCOS status, and comparisons were done between PCOS and Control group, regardless of BMI, and between Obese and Lean group, regardless of the presence of PCOS. Insulin resistance was higher in PCOS-group than in control-group and in obese-group than in lean-group. The median of all VP-EA evaluated were similar between PCOS-group and Control-group [FMD: 6.6 versus 8.4% (p = NS); CIMT: 48.0 versus 47.0 mm.10-2 (p = NS); CAC: 6.2 versus 5.6N-1.m4.10-10 (p = NS)] and between obese-group and lean-group [FMD: 7.8 versus 6.6% (p = NS); CIMT: 48.0 versus 47.0 mm.10-2 (p = NS); CAC: 5.7 versus 6.3N-1.m4.10-10 (p = NS)]. These results suggest that PCOS and obesity do not affect VP-EA in women with minor CVRFs.

  15. Comparison of the effects of letrozole and cabergoline on vascular permeability, ovarian diameter, ovarian tissue VEGF levels, and blood PEDF levels, in a rat model of ovarian hyperstimulation syndrome.

    Science.gov (United States)

    Şahin, Nur; Apaydın, Nesin; Töz, Emrah; Sivrikoz, Oya Nermin; Genç, Mine; Turan, Gülüzar Arzu; Cengiz, Hakan; Eskicioğlu, Fatma

    2016-05-01

    To evaluate the effects of letrozole and cabergoline in a rat model of ovarian hyperstimulation syndrome (OHSS). In this prospective, controlled experimental study, the 28 female Wistar rats were divided into four subgroups (one non-stimulated control and three OHSS-positive groups: placebo, letrozole, and cabergoline). To induce OHSS, rats were injected with 10 IU of pregnant mare serum gonadotropin from day 29 to day 32 of life, followed by subcutaneous injection of 30 IU hCG on day 33. Letrozole rats received with a single dose of 0.1 mg/kg letrozole via oral gavage, on the hCG day. Cabergoline rats received with a single dose of 100 µg/kg cabergoline via oral gavage, on the hCG day. All animals were compared in terms of body weight, vascular permeability (VP), ovarian diameter, ovarian tissue VEGF expression (assessed via immunohistochemical staining), and blood pigment epithelium-derived growth factor (PEDF) levels. The OHSS-positive placebo group (group 2) exhibited the highest VP, ovarian diameter, extent of VEGF staining, and lowest PEDF level, as expected. No significant difference was evident between the letrozole and cabergoline groups in terms of any of body weight; VP; PEDF level; ovarian diameter; or the staining intensity of, or percentage staining for, VEGF in ovarian tissues. Letrozole and cabergoline were equally effective to prevent OHSS, reducing the ovarian diameter, VP, and PEDF and VEGF levels to similar extents.

  16. Plant Vascular Biology 2013: vascular trafficking.

    Science.gov (United States)

    Ursache, Robertas; Heo, Jung-Ok; Helariutta, Ykä

    2014-04-01

    About 200 researchers from around the world attended the Third International Conference on Plant Vascular Biology (PVB 2013) held in July 2013 at the Rantapuisto Conference Center, in Helsinki, Finland (http://www.pvb2013.org). The plant vascular system, which connects every organ in the mature plant, continues to attract the interest of researchers representing a wide range of disciplines, including development, physiology, systems biology, and computational biology. At the meeting, participants discussed the latest research advances in vascular development, long- and short-distance vascular transport and long-distance signalling in plant defence, in addition to providing a context for how these studies intersect with each other. The meeting provided an opportunity for researchers working across a broad range of fields to share ideas and to discuss future directions in the expanding field of vascular biology. In this report, the latest advances in understanding the mechanism of vascular trafficking presented at the meeting have been summarized.

  17. Comparison of Ticagrelor Versus Prasugrel for Inflammation, Vascular Function, and Circulating Endothelial Progenitor Cells in Diabetic Patients With Non-ST-Segment Elevation Acute Coronary Syndrome Requiring Coronary Stenting: A Prospective, Randomized, Crossover Trial.

    Science.gov (United States)

    Jeong, Han Saem; Hong, Soon Jun; Cho, Sang-A; Kim, Jong-Ho; Cho, Jae Young; Lee, Seung Hun; Joo, Hyung Joon; Park, Jae Hyoung; Yu, Cheol Woong; Lim, Do-Sun

    2017-08-28

    This study compared adenosine-associated pleiotropic effects of the 2 P2Y12 receptor antagonists on vascular function, systemic inflammation, and circulating endothelial progenitor cells (EPCs). Both ticagrelor and prasugrel have potent antiplatelet effects. However, only ticagrelor inhibits cellular uptake of adenosine. Using a randomized, crossover design with 10-week follow-up ticagrelor or prasugrel was administered to type 2 diabetic patients with non-ST-segment elevation acute coronary syndrome requiring stent implantation. A total of 62 patients underwent randomization in a 1:1 ratio to receive ticagrelor or prasugrel for 5 weeks followed by a direct cross over to the alternative treatment for 5 additional weeks. Brachial artery flow-mediated dilation, inflammatory markers, and number of circulating EPCs were compared. Improvement in brachial artery flow-mediated dilation was greater in the ticagrelor group (0.15 ± 0.19 mm vs. -0.03 ± 0.18 mm; p ticagrelor compared with prasugrel decreased interleukin 6 (-0.58 ± 0.43 pg/ml vs. -0.05 ± 0.24 pg/ml; p Ticagrelor compared with prasugrel significantly increased absolute numbers of circulating EPCs CD34+/KDR+ (42.5 ± 37.8 per μl vs. -28.2 ± 23.7 per μl; p ticagrelor significantly decreased inflammatory cytokines such as interleukin 6 and tumor necrosis factor alpha and increased circulating EPCs, contributing to improved arterial endothelial function in diabetic non-ST-segment elevation acute coronary syndrome patients. Thus, data support that pleiotropic effects of ticagrelor beyond its potent antiplatelet effects could contribute to additional clinical benefits. (Comparison of Ticagrelor vs. Prasugrel on Inflammation, Arterial Stiffness, Endothelial Function, and Circulating Endothelial Progenitor Cells in Diabetic Patients With Non-ST Elevation Acute Coronary Syndrome [NSTE-ACS] Requiring Coronary Stenting; NCT02487732). Copyright © 2017 American College of Cardiology Foundation. Published

  18. Bioprinting for vascular and vascularized tissue biofabrication.

    Science.gov (United States)

    Datta, Pallab; Ayan, Bugra; Ozbolat, Ibrahim T

    2017-03-15

    Bioprinting is a promising technology to fabricate design-specific tissue constructs due to its ability to create complex, heterocellular structures with anatomical precision. Bioprinting enables the deposition of various biologics including growth factors, cells, genes, neo-tissues and extra-cellular matrix-like hydrogels. Benefits of bioprinting have started to make a mark in the fields of tissue engineering, regenerative medicine and pharmaceutics. Specifically, in the field of tissue engineering, the creation of vascularized tissue constructs has remained a principal challenge till date. However, given the myriad advantages over other biofabrication methods, it becomes organic to expect that bioprinting can provide a viable solution for the vascularization problem, and facilitate the clinical translation of tissue engineered constructs. This article provides a comprehensive account of bioprinting of vascular and vascularized tissue constructs. The review is structured as introducing the scope of bioprinting in tissue engineering applications, key vascular anatomical features and then a thorough coverage of 3D bioprinting using extrusion-, droplet- and laser-based bioprinting for fabrication of vascular tissue constructs. The review then provides the reader with the use of bioprinting for obtaining thick vascularized tissues using sacrificial bioink materials. Current challenges are discussed, a comparative evaluation of different bioprinting modalities is presented and future prospects are provided to the reader. Biofabrication of living tissues and organs at the clinically-relevant volumes vitally depends on the integration of vascular network. Despite the great progress in traditional biofabrication approaches, building perfusable hierarchical vascular network is a major challenge. Bioprinting is an emerging technology to fabricate design-specific tissue constructs due to its ability to create complex, heterocellular structures with anatomical precision

  19. Down Syndrome: A Cardiovascular Perspective

    Science.gov (United States)

    Vis, J. C.; Duffels, M. G. J.; Winter, M. M.; Weijerman, M. E.; Cobben, J. M.; Huisman, S. A.; Mulder, B. J. M.

    2009-01-01

    This review focuses on the heart and vascular system in patients with Down syndrome. A clear knowledge on the wide spectrum of various abnormalities associated with this syndrome is essential for skillful management of cardiac problems in patients with Down syndrome. Epidemiology of congenital heart defects, cardiovascular aspects and…

  20. Down syndrome: a cardiovascular perspective

    NARCIS (Netherlands)

    Vis, J. C.; Duffels, M. G. J.; Winter, M. M.; Weijerman, M. E.; Cobben, J. M.; Huisman, S. A.; Mulder, B. J. M.

    2009-01-01

    This review focuses on the heart and vascular system in patients with Down syndrome. A clear knowledge on the wide spectrum of various abnormalities associated with this syndrome is essential for skilful management of cardiac problems in patients with Down syndrome. Epidemiology of congenital heart

  1. [Vascular tumours and malformations, classification, pathology and imaging].

    Science.gov (United States)

    Wassef, M; Vanwijck, R; Clapuyt, P; Boon, L; Magalon, G

    2006-01-01

    The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects in vascular morphogenesis. These two types of lesions have different clinical behaviour and require different diagnostic and therapeutic strategies. The most frequent vascular tumour is infantile hemangioma. Its clinical aspects and evolution are well-known. New data have been recently obtained concerning the phenotype of tumour cells and its histogenesis. Of the numerous new vascular tumours, which have been recently described, only the congenital hemangiomas, the vascular tumours associated with the Maffucci syndrome and the tumours that may be complicated by a profound thrombocytopenia (Kasabach and Merritt phenomenon) will be considered. Vascular malformations can be classified according to the vessel(s) types they are composed of. A classification table is presented, separating the malformations of vascular trunks from tissular malformations which are more intimately embedded in the surrounding tissues. The different syndromes associated with vascular anomalies take also place in this table. The clinical, imaging and histological aspects of the most frequent malformations (capillary, venous, lymphatic and arteriovenous) are presented. This classification intend to clarify the nosology and terminology of the complex field of vascular tumours and malformation and to offer a common language to the different physicians and specialists contributing, preferably with a interdisciplinary approach, to the diagnosis and treatment of these difficult lesions.

  2. Inter-dependent mechanisms behind cognitive dysfunction, vascular biology and Alzheimer’s dementia in Down syndrome: multi-faceted roles of APP

    Directory of Open Access Journals (Sweden)

    Dean eNizetic

    2015-12-01

    Full Text Available People with Down syndrome (DS virtually all develop intellectual disability (ID of varying degree of severity, and also have a high risk of early Alzheimer’s disease (AD. Intellectual disability prior to the onset of dementia, and its relationship to the onset of dementia in DS is a complex phenomenon influenced by many factors, and scarcely understood. Unravelling the causative factors and modulators of these processes remains a challenge, with potential to be informative for both ID and AD, for the development of early biomarkers and/or therapeutic approaches. We review the potential relative and inter-connected roles of the chromosome 21 gene for amyloid precursor protein (APP, in both pathological conditions. Rare non-DS people with duplication of APP (dupAPP get familial early onset AD (FEOAD with virtually 100% penetrance and prominent cerebrovascular pathology, but don’t suffer from ID before dementia onset. All of these features appear to be radically different in DS. On the other hand, rare individuals with partial trisomy 21 (T21 (with APP, but not DS-critical region in trisomy have been described having ID. Likewise, partial T21 DS (without APP trisomy show a range of ID, but no AD pathology. We review the multi-faceted roles of APP that might affect cognitive functioning. Given the fact that both Aβ secretion and synaptic maturation/plasticity are dependent on neuronal activity, we explore how this conflicting inter-dependency might affect cognitive pathogenesis in a dynamic way in DS, throughout the lifespan of an individual.

  3. Evaluation of Effectiveness of Embolization in Pelvic Congestion Syndrome with the New Vascular Occlusion Device (ArtVentive EOS™): Preliminary Results

    Energy Technology Data Exchange (ETDEWEB)

    Pyra, Krzysztof, E-mail: k.pyra@poczta.fm [Medical University of Lublin, Department of Interventional Radiology and Neuroradiology (Poland); Woźniak, Sławomir, E-mail: slavwo7572@gmail.com [Medical University of Lublin, III Gynecology Clinic (Poland); Drelich-Zbroja, Anna, E-mail: zbroanna@interia.pl; Wolski, Andrzej, E-mail: andrzej.s.wolski@gmail.com; Jargiełło, Tomasz, E-mail: tojarg@interia.pl [Medical University of Lublin, Department of Interventional Radiology and Neuroradiology (Poland)

    2016-08-15

    PurposeThis study aimed to collect confirmatory data in support of the safety and efficiency of the ArtVentive EOS™ for the treatment of the pelvic congestion syndrome (PCS). This study was based on the OCCLUDE 1 Study Protocol approved by the Local Ethics Committee.Materials and MethodsA prospective study carried out in June and July 2014 included 12 women aged 21–48 years (mean 31 years) scheduled for PCS embolization using the ArtVentive EOS™. The inclusion criteria were clinical symptoms of PCS documented by transvaginal Doppler ultrasound and pelvic MRI. The pelvic pain was assessed by VAS score from 0 to 10 (0 represents lack of pain and 10 unbearable pain). A decrease in pelvic pain intensity based on the VAS was considered a clinical success.ResultsSuccessful embolization procedures with ArtVentive EOS™ were performed in 11 out of 12 patients. Nine patients underwent unilateral embolization of the left ovarian vein, and two had bilateral embolization of the ovarian veins. Complete ovarian vein occlusion confirmed by post deployment venography was achieved in all 11 patients. Procedures lasted from 19 to 45 min (average 28 min). Pain intensity decrease was observed in all 11 patients—a decrease of 5.6 points—from 7.3 pre-procedure to 1.6 post-embolization (standard deviation: 0.67). In one case, the left ovarian vein was injured by guide wire manipulation with contrast extravasation—not clinically significant.ConclusionsThe use of ArtVentive EOS™ for occlusion of the ovarian veins in PCS patients is safe and effective.

  4. Inter-Dependent Mechanisms Behind Cognitive Dysfunction, Vascular Biology and Alzheimer's Dementia in Down Syndrome: Multi-Faceted Roles of APP.

    Science.gov (United States)

    Nizetic, Dean; Chen, Christopher L; Hong, Wanjin; Koo, Edward H

    2015-01-01

    People with Down syndrome (DS) virtually all develop intellectual disability (ID) of varying degree of severity, and also have a high risk of early Alzheimer's disease (AD). ID prior to the onset of dementia, and its relationship to the onset of dementia in DS is a complex phenomenon influenced by many factors, and scarcely understood. Unraveling the causative factors and modulators of these processes remains a challenge, with potential to be informative for both ID and AD, for the development of early biomarkers and/or therapeutic approaches. We review the potential relative and inter-connected roles of the chromosome 21 gene for amyloid precursor protein (APP), in both pathological conditions. Rare non-DS people with duplication of APP (dupAPP) get familial early onset AD (FEOAD) with virtually 100% penetrance and prominent cerebrovascular pathology, but don't suffer from ID before dementia onset. All of these features appear to be radically different in DS. On the other hand, rare individuals with partial trisomy 21 (T21) (with APP, but not DS-critical region in trisomy) have been described having ID. Likewise, partial T21 DS (without APP trisomy) show a range of ID, but no AD pathology. We review the multi-faceted roles of APP that might affect cognitive functioning. Given the fact that both Aβ secretion and synaptic maturation/plasticity are dependent on neuronal activity, we explore how this conflicting inter-dependency might affect cognitive pathogenesis in a dynamic way in DS, throughout the lifespan of an individual.

  5. Calcium gluconate infusion is as effective as the vascular endothelial growth factor antagonist cabergoline for the prevention of ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Nikita Naredi

    2013-01-01

    Full Text Available Background: Ovarian hyperstimulation syndrome (OHSS is an iatrogenic and potentially life-threatening disease process, which may occur in healthy young women undergoing controlled ovarian hyperstimulation for assisted reproduction. As the treatment is largely empirical, prevention forms the mainstay of management. Objective: The present study was aimed to evaluate the effectiveness of intravenous (IV calcium gluconate infusion in comparison to the dopamine agonist cabergoline (Cb2 in preventing OHSS in high risk patients undergoing assisted reproductive technique cycles. Materials and Methods: It was a comparative study wherein the 202 high risk patients undergoing in vitro-fertilization over a period of 18 months after meeting the strict inclusion and the exclusion criteria, were randomly divided into two groups (98 subjects in Group I and 104 in Group II. Women in Group I were administered IV calcium gluconate while the remaining 104 received the dopamine agonist Cb2. The 104 patients belonging to Group II were started Cb2 0.5 mg/day from the day of ovulation trigger and continued until the next 8 days while the 98 high risk patients from Group I were infused with 10 ml of 10% calcium gluconate solution in 200 ml physiologic saline within 30 min of ovum pick up and continued thereafter on day 1, day 2 and day 3. Results: The occurrence of OHSS was seen in only nine patients (in the calcium infusion group, when compared with 16 patients (9.2% vs. 15.4% who were administered Cb2, but it was not statistically significant. However, only one had severe OHSS in Group I, whereas two women were diagnosed as severe OHSS belonging to the Cb2 arm. Conclusion: Our results document that calcium infusion can effectively prevent severe OHSS and decreases OHSS occurrence rates when used for high-risk patients, but does not suggest its superiority over Cb2. With comparable success rates, either of them can be employed as a preventive strategy for OHSS.

  6. Depression in vascular dementia.

    Science.gov (United States)

    Naarding, Paul; de Koning, Inge; dan Kooten, Fop; Dippel, Diederik W J; Janzing, Joost G E; van der Mast, Rose C; Koudstaal, Peter J

    2003-04-01

    To study the presence of different dimensions of depression in subjects with vascular dementia. After a stroke, cognitive, affective and behavioural disturbances are common. It has been suggested that the nature of affective symptomatology can help to differentiate organic from psychological depression. Cognitive and affective symptoms were assessed in 78 stroke patients and a principal component analysis was performed on these symptoms. Also, a discriminant analysis was carried out to establish the contribution of different symptoms on the diagnosis 'depressive disorder' and 'dementia'. (1) Principal component analysis revealed three distinct sub-syndromes: one with predominantly mood symptoms, one with essentially psychomotor symptoms, and one with vegetative symptoms; (2) mood, psychomotor and vegetative symptoms were all independently and strongly related to a diagnosis of major depressive disorder according to DSM-III-R criteria; (3) the psychomotor factor was also firmly associated with dementia; and (4) discriminant analysis gave further support for our conclusion that some of the depressive features, in particular the psychomotor factor, are at least partly related to the organic brain damage from stroke. The results indicate that different dimensions of depression could be discerned in a group of stroke patients and that the symptom profile of depression in these patients can be affected by the presence of dementia. Copyright 2003 John Wiley & Sons, Ltd.

  7. Collagen vascular disease

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001223.htm Collagen vascular disease To use the sharing features on this page, ... previously said to have "connective tissue" or "collagen vascular" disease. We now have names for many specific conditions ...

  8. Impact of the individual components of the metabolic syndrome and their different combinations on the prevalence of atherosclerotic vascular disease in type 2 diabetes: the Diabetes in Germany (DIG study

    Directory of Open Access Journals (Sweden)

    Benke Inge

    2007-04-01

    Full Text Available Abstract Background One of the major controversies surrounding the metabolic syndrome (MetS in type 2 diabetes is whether its single components act synergistically as risk factors for atherosclerotic vascular disease (AVD. We aimed to answer this by evaluating the relationship, and its various combinations to AVD in comparison to single traits in a population-based study with type 2 diabetes in Germany. Methods and results 4020 unselected patients with type 2 diabetes aged 35 – 80 years. MetS was: diabetes plus ≥ 2 traits of the MetS by AHA/NHBLI definition. AVD was: history of myocardial infarction and/or coronary revascularization and/or stroke. The occurrence of AVD in relation to overall MetS/single traits/combinations was presented as OR (95% CI. Multiple logistic regression, including established cardiovascular risk factors, modeled their associations. The prevalence of overall MetS was 74.4% and the OR for AVD was 1.41 (1.12–1.78, which however was higher for hypertension as single trait (OR 4.76. Different combinations of MetS presented a wide range of ORs (0.47 to 10.90 and strong sex differences. Some clusters of MetS including hypertension and low HDL-cholesterol presented a higher risk factor than single traits or their sum, whereas the others out of 11 possible carried no increased AVD risk. Multiple logistic regression showed independent association between AVD and overall MetS. Conclusion The overall MetS in type 2 diabetes comprises 11 heterogenous clusters of traits. Overall MetS increases the risk of AVD in type 2 diabetes and individual traits in some clusters with hypertension and low HDL-cholesterol may act synergistically as risk factors particularly in women.

  9. The Vascular Factor Plays the Main Role in the Cause of Pain in Men with Chronic Prostatitis and Chronic Pelvic Pain Syndrome: The Results of Clinical Trial on Thermobalancing Therapy

    Directory of Open Access Journals (Sweden)

    Simon Allen

    2017-11-01

    Full Text Available Chronic pain in patients with chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS, NIH category III is difficult to treat without understanding its cause. The main symptom of chronic prostatitis is pain. In this study, we would like to explain the origin of pain in men with CP/CPPS and its therapy. Forty-five patients with CP/CPPS have received thermobalancing therapy (TT enabled by Dr Allen’s therapeutic device (DATD for six months as mono-therapy. The control group comprised 45 men with CP/CPPS did not receive TT. Before and after six months the National Institute of Health Chronic Prostatitis Symptom Index (NIH-CPSI scores, prostatic volume (PV by ultrasound measurement and uroflowmetry (Qmax were compared between the groups. Baseline characteristics have shown no difference. After TT, significant improvements in pain score (p < 0.001, quality of life index (QoL (p < 0.001, decrease of PV (p < 0.001, and increase Qmax (p < 0.001 were determined. There were not noteworthy changes in the control group. Chronic pain due to CP/CPPS happens as a consequence and challenges at the capillary level, namely pathological capillary activity. In response to initial triggers—such as inflammation, cold, psychological and other factors—constriction and spontaneous expansion of capillaries follows, creating a continuous secondary trigger—i.e., the micro-focus of hypothermia—which in turn provokes expansion of capillaries. The additional tissue due to vascular changes into the prostate increases pressure on nociceptors causing pain. TT relieves chronic pelvic pain by eliminating the lasting focus of hypothermia in the affected prostate tissue.

  10. Moyamoya Vasculopathy in PHACE Syndrome: Six New Cases and Review of the Literature.

    Science.gov (United States)

    Tortora, Domenico; Severino, Mariasavina; Accogli, Andrea; Martinetti, Carola; Vercellino, Nadia; Capra, Valeria; Rossi, Andrea; Pavanello, Marco

    2017-12-01

    PHACE syndrome (Posterior fossa malformations, large cervicofacial infantile Hemangiomas, Arterial anomalies, aortic coarctation and Cardiac abnormalities, and Eye abnormalities) is a neurocutaneous disorder including posterior fossa malformations, hemangiomas, arterial lesions, cardiac defects, and eye abnormalities. PHACE arteriopathies may be progressive and recently have been categorized based on the risk of acute ischemic stroke, increasing attention to the potentially devastating consequences of cerebrovascular complications in this syndrome. In contrast, the natural history of arteriopathy in PHACE syndrome remains poorly understood. At the moment, there are no established surgical guidelines for high-risk vasculopathies, including quasi-moyamoya, in this syndrome. We described the clinicoradiologic features of a small series of 6 patients with PHACE syndrome and quasi-moyamoya (5 female, age range 4 months to 12 years), focusing on the clinical course and surgical outcome of 3 children who were treated with encephaloduroarteriosynangiosis and encephalomyosynangiosis. In addition, we reviewed the radiologic, clinical, and surgical aspects of moyamoya vasculopathy in PHACE syndrome, providing information on 15 additional published cases. Although the natural history of arteriopathy in PHACE syndrome is poorly understood, patients with high-risk vasculopathies, such as quasi-moyamoya disease, may benefit of revascularization by using encephaloduroarteriosynangiosis and encephalomyosynangiosis. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. [Vascular malformations (I). Concept, classification, pathogenesis and clinical features].

    Science.gov (United States)

    Redondo, P

    2007-04-01

    Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress and may grow during lifetime. Clinical presentation of vascular malformations is extremely variable and ranges from asymptomatic spots of mere aesthetic concern to lesions with high blood flow or located in critical sites that may be life-threatening. Given the low incidence of these disorders it is difficult to establish therapeutic guidelines. In addition to a correct classification of vascular anomalies, it is necessary a multidisciplinary approach for the follow-up and management of these patients. The first part of this review focuses on the different classifications of vascular anomalies, maintaining as reference the one proposed by the International Society for the Study of Vascular Anomalies (ISSVA). Additionally, clinical features of the different subtypes of vascular anomalies as well as their association in certain syndromes are reviewed.

  12. Cardiopulmonary Manifestations of Collagen Vascular Diseases.

    Science.gov (United States)

    Jawad, Hamza; McWilliams, Sebastian R; Bhalla, Sanjeev

    2017-10-09

    The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population. Interstitial pneumonia with autoimmune features (IPAF) is a newly described entity that encompasses interstitial lung disease in patients with clinical, serologic, or morphologic features suggestive of but not diagnostic of collagen vascular disease; these patients are thought to have better outcomes than idiopathic interstitial pneumonias. Interstitial lung disease and pulmonary hypertension determine the prognosis in collagen vascular disease patients. IPAF is a new term to label patients with possible collagen vascular disease-related interstitial lung disease. Collagen vascular disease patients are at increased risk for various malignancies.

  13. Encephalocraniocutaneous lipomatosis (Haberland syndrome: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Kalyan Koti

    2013-01-01

    Full Text Available Encephalocraniocutaneous lipomatosis (ECCL is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.

  14. Vascular grading of angiogenesis

    DEFF Research Database (Denmark)

    Hansen, S; Grabau, D A; Sørensen, Flemming Brandt

    2000-01-01

    The study aimed to evaluate the prognostic value of angiogenesis by vascular grading of primary breast tumours, and to evaluate the prognostic impact of adding the vascular grade to the Nottingham Prognostic Index (NPI). The investigation included 836 patients. The median follow-up time was 11...... years and 4 months. The microvessels were immunohistochemically stained by antibodies against CD34. Angiogenesis was graded semiquantitatively by subjective scoring into three groups according to the expected number of microvessels in the most vascular tumour area. The vascular grading between observers...... was moderately reproduced (kappa = 0.59). Vascular grade was significantly associated with axillary node involvement, tumour size, malignancy grade, oestrogen receptor status and histological type. In univariate analyses vascular grade significantly predicted recurrence free survival and overall survival for all...

  15. Vascular risk factors, cognitve decline, and dementia

    Directory of Open Access Journals (Sweden)

    E Duron

    2008-04-01

    Full Text Available E Duron, Olivier HanonBroca Hospital, Paris, FranceAbstract: Dementia is one of the most important neurological disorders in the elderly. Aging is associated with a large increase in the prevalence and incidence of degenerative (Alzheimer’s disease and vascular dementia, leading to a devastating loss of autonomy. In view of the increasing longevity of populations worldwide, prevention of dementia has turned into a major public health challenge. In the past decade, several vascular risk factors have been found to be associated with vascular dementia but also Alzheimer’s disease. Some longitudinal studies, have found significant associations between hypertension, diabetus mellitus, and metabolic syndrome, assessed at middle age, and dementia. Studies assessing the link between hypercholesterolemia, atrial fibrillation, smoking, and dementia have given more conflicting results. Furthermore, some studies have highlighted the possible protective effect of antihypertensive therapy on cognition and some trials are evaluating the effects of statins and treatments for insulin resistance. Vascular risk factors and their treatments are a promising avenue of research for prevention of dementia, and further long-term, placebo-controlled, randomized studies, need to be performed.Keywords: dementia, hypertension, diabetus mellitus, hypercholesterolemia, metabolic syndrome

  16. Clinical presentations and epidemiology of vascular dementia.

    Science.gov (United States)

    Smith, Eric E

    2017-06-01

    Cerebrovascular and cardiovascular diseases cause vascular brain injury that can lead to vascular cognitive impairment (VCI). VCI is the second most common neuropathology of dementia and mild cognitive impairment (MCI), accounting for up to one-third of the population risk. It is frequently present along with other age-related pathologies such as Alzheimer's disease (AD). Multiple etiology dementia with both VCI and AD is the single most common cause of later life dementia. There are two main clinical syndromes of VCI: post-stroke VCI in which cognitive impairment is the immediate consequence of a recent stroke and VCI without recent stroke in which cognitive impairment is the result of covert vascular brain injury detected only on neuroimaging or neuropathology. VCI is a syndrome that can result from any cause of infarction, hemorrhage, large artery disease, cardioembolism, small vessel disease, or other cerebrovascular or cardiovascular diseases. Secondary prevention of further vascular brain injury may improve outcomes in VCI. © 2017 The Author(s); published by Portland Press Limited on behalf of the Biochemical Society.

  17. Venous Thromboembolism in Pediatric Vascular Anomalies

    Directory of Open Access Journals (Sweden)

    Taizo A. Nakano

    2017-07-01

    Full Text Available The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy. Vascular malformations, tumors, and complex combined syndromes demonstrate varying degrees of prothrombotic activity and consumptive coagulopathy depending on the vessels involved and the pattern and extent of abnormal growth. The clinical impact of venous thromboembolism in pediatric vascular anomalies varies from painful syndromes that disrupt quality of life to life-threatening embolic disease. There remains little literature on the study, evaluation, and treatment of thrombosis in pediatric vascular anomalies. However, there have been great advances in our ability to image complex lesions, to surgically and interventionally augment disease, and to provide enhanced supportive care including patient education, compression therapy, and strategic use of anticoagulation.

  18. Uterine vascular lesions.

    Science.gov (United States)

    Vijayakumar, Abhishek; Srinivas, Amruthashree; Chandrashekar, Babitha Moogali; Vijayakumar, Avinash

    2013-01-01

    Vascular lesions of the uterus are rare; most reported in the literature are arteriovenous malformations (AVMs). Uterine AVMs can be congenital or acquired. In recent years, there has been an increasing number of reports of acquired vascular lesions of the uterus following pregnancy, abortion, cesarean delivery, and curettage. It can be seen from these reports that there is confusion concerning the terminology of uterine vascular lesions. There is also a lack of diagnostic criteria and management guidelines, which has led to an increased number of unnecessary invasive procedures (eg, angiography, uterine artery embolization, hysterectomy for abnormal vaginal bleeding). This article familiarizes readers with various vascular lesions of the uterus and their management.

  19. The metabolic syndrome and vascular disease

    NARCIS (Netherlands)

    Olijhoek, Jobien Karen

    2006-01-01

    In the Western population cardiovascular diseases are the most common cause of mortality and morbidity. There are several important risk factors for cardiovascular diseases, among them hypertension, hypercholesterolemia, diabetes and obesity. The clustering of cardiovascular risk factors associated

  20. Vascular grading of angiogenesis

    DEFF Research Database (Denmark)

    Hansen, S; Grabau, D A; Sørensen, Flemming Brandt

    2000-01-01

    years and 4 months. The microvessels were immunohistochemically stained by antibodies against CD34. Angiogenesis was graded semiquantitatively by subjective scoring into three groups according to the expected number of microvessels in the most vascular tumour area. The vascular grading between observers...

  1. Compartmental syndromes in children.

    Science.gov (United States)

    Matsen, F A; Veith, R G

    1981-01-01

    Compartmental syndromes are reported in 24 children after injuries and surgery. In these cases, increased tissue pressure compromised local perfusion and neuromuscular function. Compartmental syndromes occurred in the interosseous compartments of the hand, the volar and dorsal compartments of the forearm, and the four compartments of the leg. The most common etiologies were fracture, vascular injury, and tibial osteotomy. In many instances, clinical data were sufficient to establish the diagnosis. However, in young patients or in patients with neurologic or vascular injuries, tissue pressure measurement helped to resolve otherwise ambiguous findings. The most significant determinant of the quality of the end result was the duration of the compartmental syndrome prior to surgical decompression. We conclude that prompt diagnosis and decompression of compartmental syndromes can minimize the sequela from these conditions.

  2. Vascular cognitive impairment and vascular dementia

    Directory of Open Access Journals (Sweden)

    Klavdija Ovčar

    2017-09-01

    Full Text Available In the developed world, five to ten percent of people older than 65 years have dementia. One fifth of dementia etiologies are due to vascular brain lesions (VaD – vascular dementia. A milder form is called vascular cognitive impairment (VCI. The main clinical criteria for VaD are: 1. cognitive decline verified with standardized cognitive test/scale, 2. evidence of the associated vascular brain lesion, 3. excluded reversible causes of cognitive decline. The main risk factors for VaD are age, atherosclerosis, diabetes and hypertension. They play a key role in pathogenesis of the cognitive impairment. Depending on the damaged brain region, different cognitive domains may be affected with or without other neurological signs. These diversities in the clinical picture challenge the correct diagnosis. Unique feature of VaD is its progression, which can be stopped, if patients receive an appropriate treatment.The treatment of VCI and VaD symptoms is similar to that in Alzheimer’s disease. More importantly, VCI may be slowed down or even stopped with proper secondary stroke prevention and good rehabilitation. The most efficient is primary stroke prevention with healthy lifestyle and treatment of acquired risk factors.

  3. Vascular disease burden in Indian subjects with vascular dementia.

    Science.gov (United States)

    Chandra, Mina; Anand, Kuljeet Singh

    2015-01-01

    Vascular disease factors like hypertension, diabetes mellitus, dyslipidaemia, and ischaemic heart disease contribute to the development of vascular dementia. As comorbidity of vascular disease factors in vascular dementia is common, we investigated the vascular disease burden in subjects with vascular dementia. To investigate the vascular disease burden due to four vascular disease factors: hypertension, diabetes mellitus, dyslipidaemia, and ischaemic heart disease in Indian subjects with vascular dementia. In this study, 159 subjects with probable vascular dementia (as per NINDS-AIREN criteria) attending the memory clinic at a tertiary care hospital were assessed for the presence of hypertension, diabetes mellitus, dyslipidaemia, and ischaemic heart disease using standardised operational definitions and for severity of dementia on the Clinical Dementia Rating (CDR) scale. The data obtained was subjected to appropriate statistical analysis. Dyslipidaemia (79.25 per cent) was the most common vascular disease factor followed by hypertension (73.58 per cent), ischaemic heart disease (58.49 per cent), and diabetes mellitus (40.80 per cent). Most subjects (81.1 per cent) had two or more vascular disease factors. Subjects with more severe dementia had more vascular disease factors (sig 0.001). People with moderate to severe dementia have a significantly higher vascular disease burden; therefore, higher vascular disease burden may be considered as a poor prognostic marker in vascular dementia. Subjects with vascular dementia and their caregivers must manage cognitive impairment and ADL alongside managing serious comorbid vascular diseases that may worsen the dementia.

  4. Vascular Dysfunction in Horses with Endocrinopathic Laminitis.

    Directory of Open Access Journals (Sweden)

    Ruth A Morgan

    Full Text Available Endocrinopathic laminitis (EL is a vascular condition of the equine hoof resulting in severe lameness with both welfare and economic implications. EL occurs in association with equine metabolic syndrome and equine Cushing's disease. Vascular dysfunction, most commonly due to endothelial dysfunction, is associated with cardiovascular risk in people with metabolic syndrome and Cushing's syndrome. We tested the hypothesis that horses with EL have vascular, specifically endothelial, dysfunction. Healthy horses (n = 6 and horses with EL (n = 6 destined for euthanasia were recruited. We studied vessels from the hooves (laminar artery, laminar vein and the facial skin (facial skin arteries by small vessel wire myography. The response to vasoconstrictors phenylephrine (10-9-10-5M and 5-hydroxytryptamine (5HT; 10-9-10-5M and the vasodilator acetylcholine (10-9-10-5M was determined. In comparison with healthy controls, acetylcholine-induced relaxation was dramatically reduced in all intact vessels from horses with EL (% relaxation of healthy laminar arteries 323.5 ± 94.1% v EL 90.8 ± 4.4%, P = 0.01, laminar veins 129.4 ± 14.8% v EL 71.2 ± 4.1%, P = 0.005 and facial skin arteries 182.0 ± 40.7% v EL 91.4 ± 4.5%, P = 0.01. In addition, contractile responses to phenylephrine and 5HT were increased in intact laminar veins from horses with EL compared with healthy horses; these differences were endothelium-independent. Sensitivity to phenylephrine was reduced in intact laminar arteries (P = 0.006 and veins (P = 0.009 from horses with EL. Horses with EL exhibit significant vascular dysfunction in laminar vessels and in facial skin arteries. The systemic nature of the abnormalities suggest this dysfunction is associated with the underlying endocrinopathy and not local changes to the hoof.

  5. Vascular remodelling in asthma.

    Science.gov (United States)

    Walters, Eugene Haydn; Soltani, Amir; Reid, David William; Ward, Chris

    2008-02-01

    We review the recent literature, focusing on 2006 and 2007, to produce an update on the patho-biology of angiogenesis and vascular endothelial growth factor in the asthmatic airway. In terms of conceptual development in asthma research, airway inflammation and remodelling have been regarded as separate processes or perhaps as sequential, with early inflammation leading later to remodelling. Recent insights identify a central role for vascular endothelial growth factor in stimulating both inflammation and vascular remodelling coincidentally, with the full panoply of vascular endothelial growth factor mediated events being complex and wide. Both nitric oxide and matrix metalloproteinase-9 induction may be important downstream pathogenic mechanisms. Virus-mediated exacerbations are a prime manifestation of the oscillating trajectory of clinical asthma. The early stimulation of vascular endothelial growth factor production is probably a central aetiological mechanism, with secondary inflammation and angiogenesis. The time scale of the latter, especially, fits with the time scale of clinico-physiological changes after exacerbation. These vascular endothelial growth factor induced changes are potentially modifiable with therapy. Insights into the importance of vascular endothelial growth factor and angiogenesis in asthma pathogenesis now lead to potential new therapeutic possibilities and elucidate why recent advances in asthma therapeutics have been so successful.

  6. Migraine in metabolic syndrome.

    Science.gov (United States)

    Guldiken, Baburhan; Guldiken, Sibel; Taskiran, Bengur; Koc, Gonul; Turgut, Nilda; Kabayel, Levent; Tugrul, Armagan

    2009-03-01

    Recent studies suggest that insulin resistance is more common in patients with migraine. Insulin resistance underlies the pathogenesis of obesity, diabetes, and hypertension that are components of metabolic syndrome. As migraine is associated with an increased risk of vascular disorders, such as stroke, and migraine patients have higher diastolic blood pressure than healthy individuals, we aimed to investigate the 1-year prevalence of migraine in metabolic syndrome. Two hundred ten patients with metabolic syndrome were enrolled in the study. Migraine was diagnosed according to International Classification of Headache Disorders-II criteria. Migraine prevalence was estimated as 11.9% in men and 22.5% in women with metabolic syndrome. Of the metabolic syndrome components, diabetes, increased waist circumference, and body mass index were significantly more frequent in patients with migraine in contrast to those without migraine (Pmigraine prevalence in metabolic syndrome was higher than in the general population.

  7. Understanding Vascular Endothelium

    OpenAIRE

    Gimbrone, Michael A.

    2014-01-01

    Understanding Vascular Endothelium : Nature’s Container for Blood The entire cardiovascular system, from the chambers of the heart to the smallest capillaries of peripheral tissues, is lined by a single-cell-thick continuous layer—the vascular endothelium. For many years, this gossamer membrane was thought to function largely as an inert barrier, passively separating the reactive components of the circulating blood from the cells and connective tissue matrix of the various organs of the body....

  8. Hypercholesterolaemia and vascular dementia

    OpenAIRE

    Appleton, Jason P.; Scutt, Polly; Sprigg, Nikola; Bath, Philip M.

    2017-01-01

    Vascular dementia (VaD) is the second commonest cause of dementia. Stroke is the leading cause of disability in adults in developed countries, the second major cause of dementia and the third commonest cause of death. Traditional vascular risk factors–diabetes, hypercholesterolaemia, hypertension and smoking–are implicated as risk factors for VaD. The associations between cholesterol and small vessel disease (SVD), stroke, cognitive impairment and subsequent dementia are complex and as yet no...

  9. Spontaneous hemothorax caused by rupture of an intercostal artery aneurysm in neurofibromatosis Type I: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Shim, Chang Min; Na, Jae Beom; You, Jin Jong; Chung, Sung Hoon [Gyeongsang National Univ. College of Medicine, Pusan (Korea, Republic of)

    2001-01-01

    Neurofibromatosis type I (NF-1) is the most common neurocutaneous syndrome. Associated vascular abnormalities are arterial occlusion, aneurysm, ectasia and arteriovenous malformation. Spontaneous massive hemothorax due to rupture of an arterial aneurysm is rare but fatal. It is, therefore, essential to determine the location of an aneurysm and provide immediate surgical or the interventional treatment. We report a case of spotaneous hemothorax caused by rupture of an intercostal arterial aneurysm diagnosed by CT and angiography.

  10. SMART risk factor screening in patients at high vascular risk

    NARCIS (Netherlands)

    Brouwer, B.G.

    2008-01-01

    Studies presented in this thesis focused on the relationship between the presence of coronary heart disease and intra-abdominal fat, and the relationship between leisure-time physical activity and the presence of metabolic syndrome, incidence of type 2 diabetes and recurrence of vascular events in

  11. Language Impairment in Alzheimer's Disease and Vascular Dementia.

    Science.gov (United States)

    Lempinen, Maire; And Others

    A study of 21 patients with Alzheimer's Disease and 25 with vascular dementia, the two most common forms of dementia, investigated language impairments in the dementia syndrome to see if analysis of language disturbances is helpful in differential diagnosis. Diagnostic assessment included a neurological examination, detailed medical history,…

  12. Vascular related pregnancy complications: genetics and remote cardiovascular risk

    NARCIS (Netherlands)

    A.L. Berends (Anne)

    2008-01-01

    textabstractPreeclampsia and intrauterine growth restriction (IUGR) are common vascular related pregnancy syndromes of unknown cause. Both preeclampsia and IUGR are responsible for a significant maternal and perinatal morbidity and mortality worldwide. Preeclampsia affects approximately 2.5-3.0% of

  13. Recent Advances In The Management Of Cerebro-Vascular

    African Journals Online (AJOL)

    drclement

    Benin City, Nigeria. E-mail: bfunmi@uniben.edu. INTRODUCTION. According to the World Health. Organization, stroke or cerebro-vascular accident (CVA) has been defined as a syndrome of rapidly .... factors include cigarette smoking, heavy alcohol consumption .... agents are aimed at intracellular calcium, free oxygen.

  14. Antioxidants and vascular health.

    Science.gov (United States)

    Bielli, Alessandra; Scioli, Maria Giovanna; Mazzaglia, Donatella; Doldo, Elena; Orlandi, Augusto

    2015-12-15

    Oxygen free radicals and other reactive oxygen species (ROS) are common products of normal aerobic cellular metabolism, but high levels of ROS lead to oxidative stress and cellular damage. Increased production of ROS favors vascular dysfunction, inducing altered vascular permeability and inflammation, accompanied by the loss of vascular modulatory function, the imbalance between vasorelaxation and vasoconstriction, and the aberrant expression of inflammatory adhesion molecules. Inflammatory stimuli promote oxidative stress generated from the increased activity of mitochondrial nicotinamide adenine dinucleotide phosphate oxidase, particularly of the Nox4 isoform, with the consequent impairment of mitochondrial β-oxidation. Vascular dysfunction due to the increase in Nox4 activity and ROS overproduction leads to the progression of cardiovascular diseases, diabetes, inflammatory bowel disease, and neurological disorders. Considerable research into the development of effective antioxidant therapies using natural derivatives or new synthetic molecules has been conducted. Antioxidants may prevent cellular damage by reducing ROS overproduction or interfering in reactions that involve ROS. Vitamin E and ascorbic acid are well known as natural antioxidants that counteract lipid peroxidative damage by scavenging oxygen-derived free radicals, thus restoring vascular function. Recently, preliminary studies on natural antioxidants such as goji berries, thymus, rosemary, green tea ginseng, and garlic have been conducted for their efficacy in preventing vascular damage. N-acetyl-cysteine and propionyl-L-carnitine are synthetic compounds that regulate ROS production by replacing endogenous antioxidants in both endothelial and smooth muscle cells. In this review, we consider the molecular mechanisms underlying the generation of oxidative stress-induced vascular dysfunction as well as the beneficial effects of antioxidant therapies.

  15. Eisenmenger Syndrome in Pregnancy

    Directory of Open Access Journals (Sweden)

    Shi-Min Yuan

    Full Text Available Abstract Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.

  16. Atrial fibrillation and vascular disease-a bad combination

    DEFF Research Database (Denmark)

    Bjerring Olesen, Jonas; Gislason, Gunnar Hilmar; Torp-Pedersen, Christian

    2012-01-01

    This article provides an overview of (i) the risk of stroke associated with vascular disease (acute coronary syndromes and peripheral artery disease) in patients with atrial fibrillation, (ii) the frequent coexistence of vascular disease in patients with atrial fibrillation and, (iii......) the cardiovascular risk associated with the coexisting of the two diseases. The literature on this topic is relatively sparse, and we discuss results from both clinical trials and observational studies. There is a clear indication of an increased stroke risk associated with vascular disease in patients with atrial...... fibrillation. Indeed, patients with atrial fibrillation often had coexisting vascular disease (around 18%), and the combination of the two diseases substantially increases the risk of future cardiovascular events. The increased risk associated with peripheral artery disease in atrial fibrillation is even more...

  17. Vascularized epiphyseal transplant.

    Science.gov (United States)

    Innocenti, Marco; Delcroix, Luca; Romano, G Federico; Capanna, Rodolfo

    2007-01-01

    In skeletally immature patients, the transfer of vascularized epiphysis along with a variable amount of adjoining diaphysis may provide the potential for growth of such a graft, preventing future limb length discrepancy. This article describes the authors' experience with the vascularized transfer of the proximal fibular epiphysis in the reconstruction of large bone defects including the epiphysis in a series of 27 patients ranging in age from 2 to 11 years. The follow-up, ranging from 2 to 14 years, has been long enough to allow some evaluation of the validity, indications, and limits of this reconstructive option.

  18. Serotonin syndrome

    Science.gov (United States)

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

  19. Rheumatic diseases and autoimmune vascular dementia.

    Science.gov (United States)

    Atzeni, Fabiola; Pipitone, Nicolò; Iaccarino, Luca; Masala, Ignazio Francesco; Weiss, Ronen; Alciati, Alessandra; Doria, Andrea; Chapmanand, Joab; Sarzi-Puttini, Piercarlo

    2017-10-13

    Vascular dementia (VD) comes second after Alzheimer's disease (AD) as a cause of impaired cognition. VD is not a specific nosological entity, but rather a syndrome encompassing a number of diseases caused by impaired supply of blood to the brain. Systemic autoimmune disorders such as systemic lupus erythematosus, rheumatoid arthritis, vasculitis and antiphospholipid syndrome (APS) can be associated with dementia. VD is often related to the presence of traditional cardiovascular risk factors, but it may also be associated with a host of disorders affecting the brain blood vessels, neuronal cells, or both. It is important to entertain in the differential diagnosis of VD, to recognize and to cure them accurately in order to preserve life's quality of our patients. Copyright © 2017. Published by Elsevier B.V.

  20. Critical Endothelial Regulation by LRP5 during Retinal Vascular Development.

    Directory of Open Access Journals (Sweden)

    Wei Huang

    Full Text Available Vascular abnormalities in the eye are the leading cause of many forms of inherited and acquired human blindness. Loss-of-function mutations in the Wnt-binding co-receptor LRP5 leads to aberrant ocular vascularization and loss of vision in genetic disorders such as osteoporosis-pseudoglioma syndrome. The canonical Wnt-β-catenin pathway is known to regulate retinal vascular development. However, it is unclear what precise role LPR5 plays in this process. Here, we show that loss of LRP5 function in mice causes retinal hypovascularization during development as well as retinal neovascularization in adulthood with disorganized and leaky vessels. Using a highly specific Flk1-CreBreier line for vascular endothelial cells, together with several genetic models, we demonstrate that loss of endothelium-derived LRP5 recapitulates the retinal vascular defects in Lrp5-/- mice. In addition, restoring LRP5 function only in endothelial cells in Lrp5-/- mice rescues their retinal vascular abnormalities. Furthermore, we show that retinal vascularization is regulated by LRP5 in a dosage dependent manner and does not depend on LRP6. Our study provides the first direct evidence that endothelium-derived LRP5 is both necessary and sufficient to mediate its critical role in the development and maintenance of retinal vasculature.

  1. Hypercholesterolaemia and vascular dementia.

    Science.gov (United States)

    Appleton, Jason P; Scutt, Polly; Sprigg, Nikola; Bath, Philip M

    2017-07-15

    Vascular dementia (VaD) is the second commonest cause of dementia. Stroke is the leading cause of disability in adults in developed countries, the second major cause of dementia and the third commonest cause of death. Traditional vascular risk factors-diabetes, hypercholesterolaemia, hypertension and smoking-are implicated as risk factors for VaD. The associations between cholesterol and small vessel disease (SVD), stroke, cognitive impairment and subsequent dementia are complex and as yet not fully understood. Similarly, the effects of lipids and lipid-lowering therapy on preventing or treating dementia remain unclear; the few trials that have assessed lipid-lowering therapy for preventing (two trials) or treating (four trials) dementia found no evidence to support the use of lipid-lowering therapy for these indications. It is appropriate to treat those patients with vascular risk factors that meet criteria for lipid-lowering therapy for the primary and secondary prevention of cardiovascular and cerebrovascular events, and in line with current guidelines. Managing the individual patient in a holistic manner according to his or her own vascular risk profile is recommended. Although the paucity of randomized controlled evidence makes for challenging clinical decision making, it provides multiple opportunities for on-going and future research, as discussed here. © 2017 The Author(s).

  2. Depression in vascular dementia.

    NARCIS (Netherlands)

    Naarding, P.; Koning, I. de; Kooten, F. van; Dippel, D.W.; Janzing, J.G.E.; Mast, R.C. van der; Koudstaal, P.J.

    2003-01-01

    OBJECTIVE: To study the presence of different dimensions of depression in subjects with vascular dementia. BACKGROUND: After a stroke, cognitive, affective and behavioural disturbances are common. It has been suggested that the nature of affective symptomatology can help to differentiate organic

  3. Vascular management in rotationplasty.

    Science.gov (United States)

    Mahoney, Craig R; Hartman, Curtis W; Simon, Pamela J; Baxter, B Timothy; Neff, James R

    2008-05-01

    The Van Nes rotationplasty is a useful limb-preserving procedure for skeletally immature patients with distal femoral or proximal tibial malignancy. The vascular supply to the lower limb either must be maintained and rotated or transected and reanastomosed. We asked whether there would be any difference in the ankle brachial index or complication rate for the two methods of vascular management. Vessels were resected with the tumor in seven patients and preserved and rotated in nine patients. One amputation occurred in the group in which the vessels were preserved. Four patients died secondary to metastatic disease diagnosed preoperatively. The most recent ankle brachial indices were 0.96 and 0.82 for the posterior tibial and dorsalis pedis arteries, respectively, in the reconstructed group. The ankle brachial indices were 0.98 and 0.96 for the posterior tibial and dorsalis pedis arteries, respectively, in the rotated group. Outcomes appear similar using both methods of vascular management and one should not hesitate to perform an en bloc resection when there is a question of vascular involvement.

  4. Pulmonary vascular complications in portal hypertension and liver disease: A concise review

    Directory of Open Access Journals (Sweden)

    M. Porres-Aguilar

    2013-01-01

    Full Text Available Chronic liver disease and/or portal hypertension may be associated with one of the two pulmonary vascular complications: portopulmonary hypertension and hepatopulmonary syndrome. These pulmonary vascular disorders are notoriously underdiagnosed; however, they have a substantial negative impact on survival and require special attention in order to understand their diagnostic approach and to select the best therapeutic options. Portopulmonary hypertension results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. On the other hand, abnormal intrapulmonary vascular dilations, profound hypoxemia, and a wide alveolar-arterial gradient are the hallmarks of the hepatopulmonary syndrome, resulting in difficult-to-treat hypoxemia. The aim of this review is to summarize the latest pathophysiologic concepts, diagnostic approach, therapy, and prognosis of portopulmonary hypertension and hepatopulmonary syndrome, as well as to discuss the role of liver transplantation as a definitive therapy in selected patients with these conditions.

  5. Renal posttransplant's vascular complications

    Directory of Open Access Journals (Sweden)

    Bašić Dragoslav

    2003-01-01

    Full Text Available INTRODUCTION Despite high graft and recipient survival figures worldwide today, a variety of technical complications can threaten the transplant in the postoperative period. Vascular complications are commonly related to technical problems in establishing vascular continuity or to damage that occurs during donor nephrectomy or preservation [13]. AIM The aim of the presenting study is to evaluate counts and rates of vascular complications after renal transplantation and to compare the outcome by donor type. MATERIAL AND METHODS A total of 463 kidneys (319 from living related donor LD and 144 from cadaveric donor - CD were transplanted during the period between June 1975 and December 1998 at the Urology & Nephrology Institute of Clinical Centre of Serbia in Belgrade. Average recipients' age was 33.7 years (15-54 in LD group and 39.8 (19-62 in CD group. Retrospectively, we analyzed medical records of all recipients. Statistical analysis is estimated using Hi-squared test and Fischer's test of exact probability. RESULTS Major vascular complications including vascular anastomosis thrombosis, internal iliac artery stenosis, internal iliac artery rupture obliterant vasculitis and external iliac vein rupture were analyzed. In 25 recipients (5.4% some of major vascular complications were detected. Among these cases, 22 of them were from CD group vs. three from LD group. Relative rate of these complications was higher in CD group vs. LD group (p<0.0001. Among these complications dominant one was vascular anastomosis thrombosis which occurred in 18 recipients (17 from CD vs. one from LD. Of these recipients 16 from CD lost the graft, while the rest of two (one from each group had lethal outcome. DISCUSSION Thrombosis of renal allograft vascular anastomosis site is the most severe complication following renal transplantation. In the literature, renal allograft thrombosis is reported with different incidence rates, from 0.5-4% [14, 15, 16]. Data from the

  6. Octopus automutilation syndrome.

    Science.gov (United States)

    Reimschuessel, R; Stoskopf, M K

    1990-05-01

    This paper describes an automutilation syndrome (OAS) in three species of captive octopuses, Octopus dolfleini, O. bimaculoides, and O. maya, characterized by external arm and mantle lesions. Three clinical patterns in nine animals had similar and characteristic gross and histopathologic features. Axial nerve or brachial artery lesions were observed in six of the nine cases and vascular lesions were seen in two of eight cases with mantle ulcerations. A relationship between automutilation in the octopus and dysesthesias due to neural or vascular pathology is proposed.

  7. Engineering vascularized skeletal muscle tissue

    NARCIS (Netherlands)

    Levenberg, Shulamit; Rouwkema, Jeroen; Macdonald, Mara; Garfein, Evan S.; Kohane, Daniel S.; Darland, Diane C.; Marini, Robert; van Blitterswijk, Clemens; Mulligan, Richard C.; D'Amore, Patricia A.; Langer, Robert

    2005-01-01

    One of the major obstacles in engineering thick, complex tissues such as muscle is the need to vascularize the tissue in vitro. Vascularization in vitro could maintain cell viability during tissue growth, induce structural organization and promote vascularization upon implantation. Here we describe

  8. Congenital vascular anomalies: current perspectives on diagnosis, classification, and management

    Directory of Open Access Journals (Sweden)

    Blei F

    2016-07-01

    Full Text Available Francine Blei,1 Mark E Bittman2 1Vascular Anomalies Program, Lenox Hill Hospital, Northwell Health, 2Department of Radiology, New York University Langone Medical Center, New York, NY, USA Abstract: The term "congenital vascular anomalies" encompasses those vascular lesions present at birth. Many of these lesions may be detected in utero. This review serves to apprise the readership of newly identified diagnoses and updated classification schemes. Attention is focused on clinical features, patterns of presentation, clinical manifestations and behavior, diagnostic tools, and treatment modalities. It is an invigorating period for this field, with a surge in vascular anomalies-related basic and clinical research, genetics, pharmacology, clinical trials, and patient advocacy. A large number of professional conferences now include vascular anomalies in the agenda, and trainees in multiple specialties are gaining expertise in this discipline. We begin with a summary of classification schemes and introduce the updated classification adopted by the International Society for the Study of Vascular Anomalies. Disease entities are described, with liberal use of photographs, as many diagnoses can be established based on a thorough history and visual appearance and it is thus essential to develop a familiarity with diagnosis-specific physical features. Peripheral (non-central nervous system vascular anomalies are the focus of this review. We focus on those entities in which diagnostic radiology is routinely used and accentuate when histologic confirmation is essential. We also underscore some differences in approach to the pediatric vs adolescent or adult patient. A list of Internet-based resources is included, with hyperlinks to informative sites. References are limited to seminal discoveries and review articles. We hope that our enthusiasm in writing this review will be shared by those who read this review. Keywords: vascular anomalies, hemangiomas, vascular

  9. Renal vascular and thrombotic effects of cyclosporine.

    Science.gov (United States)

    Remuzzi, G; Bertani, T

    1989-04-01

    Cyclosporine A (CyA) given to prevent xenograft rejection induces renal function impairment. In the last few years many studies have been devoted to understanding the mechanism(s) of CyA-induced renal insufficiency. In humans, several specific findings--interstitial fibrosis, toxic tubulopathy, peritubular capillary congestion, arteriolopathy--have been associated with CyA administration. It is now recognized that CyA renal toxicity mainly manifests under three different syndromes: (1) acute reversible decrease in glomerular filtration rate (GFR), (2) acute microvascular disease with the pattern of thrombotic microangiopathy, and (3) chronic irreversible renal damage. This review analyzes the available evidence that the clinical syndromes of CyA nephrotoxicity are related to changes induced by CyA on renal vessels. Experimental studies have failed to document that the activation of renin-angiotensin axis or sympathetic nervous system plays a relevant role in the development of CyA-associated renal vasoconstriction, which is the main causal factor of acute reversible decrease in GFR, whereas it is possible that changes in arachidonic acid metabolites with vasoactive properties contribute to this CyA-induced phenomenon. In this context, findings of increased urinary TxB2 and protective effect of TxA2 receptor blocking are of particular interest. Since the introduction of CyA in clinical practice, a syndrome of thrombotic microangiopathy resembling hemolytic uremic syndrome/thrombotic thrombocytopenic purpura has been recognized in humans and reproduced in experimental animals. This is a rare form of vascular toxicity attributed to CyA which may have a poor prognosis and possibly results from a direct toxic effect of CyA on vascular endothelium. The syndrome of chronic progressive deterioration of renal function associated with CyA was first recognized in humans. Until recently the possibility of reproducing this syndrome in animals in order to better understand its

  10. Brain Vascular Imaging Techniques

    Directory of Open Access Journals (Sweden)

    Bàrbara Laviña

    2016-12-01

    Full Text Available Recent major improvements in a number of imaging techniques now allow for the study of the brain in ways that could not be considered previously. Researchers today have well-developed tools to specifically examine the dynamic nature of the blood vessels in the brain during development and adulthood; as well as to observe the vascular responses in disease situations in vivo. This review offers a concise summary and brief historical reference of different imaging techniques and how these tools can be applied to study the brain vasculature and the blood-brain barrier integrity in both healthy and disease states. Moreover, it offers an overview on available transgenic animal models to study vascular biology and a description of useful online brain atlases.

  11. Plant Vascular Biology 2010

    Energy Technology Data Exchange (ETDEWEB)

    Ding, Biao

    2014-11-17

    This grant supported the Second International Conference on Plant Vascular Biology (PVB 2010) held July 24-28, 2010 on the campus of Ohio State University, Columbus, Ohio. Biao Ding (Ohio State University; OSU) and David Hannapel (Iowa State University; ISU) served as co-chairs of this conference. Biao Ding served as the local organizer. PVB is defined broadly here to include studies on the biogenesis, structure and function of transport systems in plants, under conditions of normal plant growth and development as well as of plant interactions with pathogens. The transport systems cover broadly the xylem, phloem, plasmodesmata and vascular cell membranes. The PVB concept has emerged in recent years to emphasize the integrative nature of the transport systems and approaches to investigate them.

  12. Pathophysiology of vascular dementia

    Directory of Open Access Journals (Sweden)

    Rizzo Claudia

    2009-11-01

    Full Text Available Abstract The concept of Vascular Dementia (VaD has been recognized for over a century, but its definition and diagnostic criteria remain unclear. Conventional definitions identify the patients too late, miss subjects with cognitive impairment short of dementia, and emphasize consequences rather than causes, the true bases for treatment and prevention. We should throw out current diagnostic categories and describe cognitive impairment clinically and according to commonly agreed instruments that document the demographic data in a standardized manner and undertake a systematic effort to identify the underlying aetiology in each case. Increased effort should be targeted towards the concept of and criteria for Vascular Cognitive Impairment and Post-Stroke Dementia as well as for genetic factors involved, especially as these categories hold promise for early prevention and treatment.

  13. Vascular cognitive impairment

    Directory of Open Access Journals (Sweden)

    N.V. Vakhnina

    2014-01-01

    Full Text Available Vascular pathology of the brain is the second most common cause of cognitive impairment after Alzheimer's disease. The article describes the modern concepts of etiology, pathogenetic mechanisms, clinical features and approaches to diagnosis and therapy of vascular cognitive impairment (VCI. Cerebrovascular accident, chronic cerebral circulatory insufficiency and their combination, sometimes in combination with a concomitant neurodegenerative process, are shown to be the major types of brain lesions leading to VCI. The clinical presentation of VCI is characterized by the neuropsychological status dominated by impairment of the executive frontal functions (planning, control, attention in combination with focal neurological symptoms. The diagnosis is based on comparing of the revealed neuropsychological and neurological features with neuroimaging data. Neurometabolic, acetylcholinergic, glutamatergic, and other vasoactive drugs and non-pharmacological methods are widely used to treat VCI. 

  14. Pathophysiology of vascular dementia

    OpenAIRE

    Rizzo Claudia; Duro Giovanni; Iemolo Francesco; Castiglia Laura; Hachinski Vladimir; Caruso Calogero

    2009-01-01

    Abstract The concept of Vascular Dementia (VaD) has been recognized for over a century, but its definition and diagnostic criteria remain unclear. Conventional definitions identify the patients too late, miss subjects with cognitive impairment short of dementia, and emphasize consequences rather than causes, the true bases for treatment and prevention. We should throw out current diagnostic categories and describe cognitive impairment clinically and according to commonly agreed instruments th...

  15. Pulmonary vascular imaging

    Energy Technology Data Exchange (ETDEWEB)

    Fedullo, P.F.; Shure, D.

    1987-03-01

    A wide range of pulmonary vascular imaging techniques are available for the diagnostic evaluation of patients with suspected pulmonary vascular disease. The characteristics of any ideal technique would include high sensitivity and specificity, safety, simplicity, and sequential applicability. To date, no single technique meets these ideal characteristics. Conventional pulmonary angiography remains the gold standard for the diagnosis of acute thromboembolic disease despite the introduction of newer techniques such as digital subtraction angiography and magnetic resonance imaging. Improved noninvasive lower extremity venous testing methods, particularly impedance plethysmography, and ventilation-perfusion scanning can play significant roles in the noninvasive diagnosis of acute pulmonary emboli when properly applied. Ventilation-perfusion scanning may also be useful as a screening test to differentiate possible primary pulmonary hypertension from chronic thromboembolic pulmonary hypertension. And, finally, angioscopy may be a useful adjunctive technique to detect chronic thromboembolic disease and determine operability. Optimal clinical decision-making, however, will continue to require the proper interpretation of adjunctive information obtained from the less-invasive techniques, applied with an understanding of the natural history of the various forms of pulmonary vascular disease and with a knowledge of the capabilities and shortcomings of the individual techniques.

  16. Det hepatopulmonale syndrome

    DEFF Research Database (Denmark)

    Eibye, Simone; Christensen, Erik

    2016-01-01

    The hepatopulmonary syndrome (HPS) - a complication of liver disease - seems to be underdiagnosed, probably because of decreased awareness. HPS consists of the triade liver disease, intrapulmonary vascular dilatation and as a consequence arterial hypoxaemia. No medical therapy has proven effectiv...... perhaps with the exception of garlic, which has been effective in one controlled clinical trial. However, liver transplantation seems to improve HPS in most cases. Liver patients with unexplained hypoxaemia should be investigated for HPS....

  17. Peripheral Vascular Disease

    Science.gov (United States)

    ... bones and very flexible joints. People with this syndrome often have aneurysms. What are the symptoms? Aortic aneurysms may cause shortness of breath, a croaky or raspy voice, backache, or pain in your left shoulder or between your shoulder blades. An aortic aneurysm ...

  18. [How Treatable is Vascular Dementia?].

    Science.gov (United States)

    Mori, Etsuro

    2016-04-01

    Vascular dementia is an umbrella term, encompassing the pathological changes in the brain due to cerebrovascular disease that result in dementia. Vascular dementia is the second most common form of dementia, after Alzheimer's disease. In this paper, I outline the concept of vascular dementia, the key aspects of the disease that are yet to be clarified, and the current status of clinical trials. Assessing these factors, I discuss how treatable vascular dementia presently is. Use of the term'vascular dementia'is riddled with uncertainties regarding disease classification, and non-standardized diagnostic criteria. There are difficulties in determining the exact relationship between cerebrovascular pathology and cognitive impairment. The comorbid effects of Alzheimer's pathology in some individuals also present an obstacle to reliable clinical diagnosis, and hinder research into effective management approaches. Vascular dementia is preventable and treatable, as there are established primary and secondary prevention measures for the causative cerebrovascular diseases, such as vascular risk factor intervention, antiplatelet therapy, and anticoagulation, amongst others. However, unlike Alzheimer's disease, there are no established symptomatic treatments for vascular dementia. Clinical trials of cholinesterase inhibitors and memantine indicate that they produce small cognitive benefits in patients with vascular dementia, though the exact clinical significance of these is uncertain. Data are insufficient to support the widespread use of these drugs in vascular dementia. Rehabilitation and physical and cognitive exercise may be beneficial, but evidence of cognitive benefit and relief of neuropsychiatric symptoms due to exercise is lacking.

  19. [Estrogens and vascular thrombosis].

    Science.gov (United States)

    Colmou, A

    1982-09-01

    The incidence of thromboses among young women has increased with widespread use of oral contraceptives (OCs) due to the significant thromboembolic risk of estrogen. Estrogens intervene at the vascular, platelet, and plasma levels as a function of hormonal variations in the menstrual cycle, increasing the aggregability of the platelets and thrombocytes, accelerating the formation of clots, and decreasing the amount of antithrombin III. Estrogens are used in medicine to treat breast and prostate cancers and in gynecology to treat dysmenorrhea, during the menopause, and in contraception. Smoking, cardiovascular disease and hypertension, hypercholesterolemia, and diabetes are contraindicators to estrogen use. Thrombosis refers to blockage of a blood vessel by a clot or thrombus. Before estrogens are prescribed, a history of phlebitis, obesity, hyperlipidemia, or significant varicosities should be ruled out. A history of venous thrombosis, hyperlipoproteinemia, breast nodules, serious liver condition, allergies to progesterone, and some ocular diseases of vascular origin definitively rule out treatment with estrogens. A family history of infarct, embolism, diabetes, cancer, or vascular accidents at a young age signals a need for greater patient surveillance. All patients receiving estrogens should be carefully observed for signs of hypertension, hypercholesterolemia, hypercoagulability, or diabetes. Nurses have a role to play in carefully eliciting the patient's history of smoking, personal and family medical problems, and previous and current laboratory results, as well as in informing the patients of the risks and possible side effects of OCs, especially for those who smoke. Nurses should educate patients receiving estrogens, especially those with histories of circulatory problems, to avoid standing in 1 position for prolonged periods, avoid heat which is a vasodilator, avoid obesity, excercise regularly, wear appropriate footgear, and follow other good health

  20. The pathobiology of vascular dementia.

    Science.gov (United States)

    Iadecola, Costantino

    2013-11-20

    Vascular cognitive impairment defines alterations in cognition, ranging from subtle deficits to full-blown dementia, attributable to cerebrovascular causes. Often coexisting with Alzheimer's disease, mixed vascular and neurodegenerative dementia has emerged as the leading cause of age-related cognitive impairment. Central to the disease mechanism is the crucial role that cerebral blood vessels play in brain health, not only for the delivery of oxygen and nutrients, but also for the trophic signaling that inextricably links the well-being of neurons and glia to that of cerebrovascular cells. This review will examine how vascular damage disrupts these vital homeostatic interactions, focusing on the hemispheric white matter, a region at heightened risk for vascular damage, and on the interplay between vascular factors and Alzheimer's disease. Finally, preventative and therapeutic prospects will be examined, highlighting the importance of midlife vascular risk factor control in the prevention of late-life dementia. Copyright © 2013 Elsevier Inc. All rights reserved.

  1. Kounis syndrome: a narrative review

    African Journals Online (AJOL)

    symptoms in anaphylaxis are largely due to peripheral vasodilation and increased vascular permeability. However, there is ... the disproportionate mortality rate of those with cardiac disease that suffer a hypersensitivity reaction. Kounis ..... Apical ballooning syndrome (ABS), or Tako-Tsubo cardiomyopathy. (a reversible left ...

  2. Rare variant of misme syndrome – a case report with review of literature

    Directory of Open Access Journals (Sweden)

    Dwivedi Ashish Kumar

    2016-09-01

    Full Text Available MISME syndrome, also known as neurofibromatosis type-2 (NF2, stands for multiple inherited schwannomas, meningiomas, and ependymomas (MISME in the peripheral and central nervous system. It is a rare disorder of autosomal dominant inheritance due to mutations of a tumor-suppressor gene on the chromosome 22q12. Clinically, it is characterized by multiple benign tumors arising in both the central and the peripheral nervous system, particularly from the bilateral vestibular nerve in more than 90% of the patients and more than two thirds of them develop spinal tumors. Simultaneous occurrence of bilateral vestibular schwanoma with cervical and lumbar ependymoma without neuro cutaneous marker with weakness of limb as initial presentation is rare finding in single patient. Here, we are reporting a rare case of MISME syndrome harbouring bilateral vestibular schwanoma with cervical and lumbar ependymoma tumors in a 45 year old male patient having no other lesion and neurocutaneous marker with weakness of limb as initial presentation without posterior subcapsular cataract.

  3. Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report

    Directory of Open Access Journals (Sweden)

    Choi Sung-Kyu

    2005-07-01

    Full Text Available Abstract Background Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder with characteristic features of skin and central nervous system involvement. Gastrointestinal involvement is rare, but the risk of malignancy development is considerable. Zollinger-Ellison syndrome is caused by gastrin-secreting tumors called gastrinomas. Correct diagnosis is often difficult, and curative treatment can only be achieved surgically. Case presentation A 41-year-old female affected by neurofibromatosis type 1 presented with a history of recurrent epigastric soreness, diarrhea, and relapsing chronic duodenal ulcer. Her serum fasting gastrin level was over 1000 pg/mL. An abdominal CT scan revealed a 3 × 2-cm, well-enhanced mass adjacent to the duodenal loop. She was not associated with multiple endocrine neoplasia type 1. Operative resection was performed and gastrinoma was diagnosed by immunohistochemical staining. The serum gastrin level decreased to 99.1 pg/mL after surgery, and symptoms and endoscopic findings completely resolved without recurrences. Conclusion Gastrinoma is difficult to detect even in the general population, and hence symptoms such as recurrent idiopathic peptic ulcer and diarrhea in neurofibromatosis type 1 patients should be accounted for as possibly contributing to Zollinger-Ellison syndrome.

  4. Lymphatic Malformation, Retinoblastoma, or Facial Cleft: Atypical Presentations of PHACE Syndrome.

    Science.gov (United States)

    Fernández-Ibieta, María; López-Gutiérrez, Juan Carlos

    2015-01-01

    PHACE syndrome is a neurocutaneous disorder characterized by large cervicofacial infantile hemangiomas and associated anomalies: posterior fossa brain malformation, hemangioma, arterial cerebrovascular anomalies, coarctation of the aorta and cardiac defects, and eye/endocrine abnormalities of the brain. When ventral developmental defects (sternal clefting or supraumbilical raphe) are present the condition is termed PHACE. In this report, we describe three PHACE cases that presented unique features (affecting one of the organ systems described for this syndrome) that have not been described previously. In the first case, a definitive PHACE association, the patient presented with an ipsilateral mesenteric lymphatic malformation, at the age of 14 years. In the second case, an anomaly of the posterior segment of the eye, not mentioned before in PHACE literature, a retinoblastoma, has been described. Specific chemotherapy avoided enucleation. And, in the third case, the child presented with an unusual midline frontal bone cleft, corresponding to Tessier 14 cleft. Two patients' hemangiomas responded well to propranolol therapy. The first one was followed and treated in the pre-propranolol era and had a moderate response to corticoids and interferon.

  5. Lymphatic Malformation, Retinoblastoma, or Facial Cleft: Atypical Presentations of PHACE Syndrome

    Directory of Open Access Journals (Sweden)

    María Fernández-Ibieta

    2015-01-01

    Full Text Available PHACE syndrome is a neurocutaneous disorder characterized by large cervicofacial infantile hemangiomas and associated anomalies: posterior fossa brain malformation, hemangioma, arterial cerebrovascular anomalies, coarctation of the aorta and cardiac defects, and eye/endocrine abnormalities of the brain. When ventral developmental defects (sternal clefting or supraumbilical raphe are present the condition is termed PHACE. In this report, we describe three PHACE cases that presented unique features (affecting one of the organ systems described for this syndrome that have not been described previously. In the first case, a definitive PHACE association, the patient presented with an ipsilateral mesenteric lymphatic malformation, at the age of 14 years. In the second case, an anomaly of the posterior segment of the eye, not mentioned before in PHACE literature, a retinoblastoma, has been described. Specific chemotherapy avoided enucleation. And, in the third case, the child presented with an unusual midline frontal bone cleft, corresponding to Tessier 14 cleft. Two patients’ hemangiomas responded well to propranolol therapy. The first one was followed and treated in the pre-propranolol era and had a moderate response to corticoids and interferon.

  6. Cushing syndrome

    Science.gov (United States)

    Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... called exogenous Cushing syndrome . Prednisone, dexamethasone, ...

  7. Scleroderma overlap syndrome.

    Science.gov (United States)

    Balbir-Gurman, Alexandra; Braun-Moscovici, Yolanda

    2011-01-01

    Overlap syndrome is an entity that satisfies the criteria of at least two connective tissue diseases (CTD). These conditions include systemic sclerosis (SSc), dermatomyositis or polymyositis, Sjogren's syndrome, rheumatoid arthritis and systemic lupus erythematosus. A combined pathology has impact on the clinical features, diagnosis and treatment. To analyze the features of SSc patients with overlap syndrome registered in the European (EUSTAR) database at our center and to review the literature focusing on clinical and diagnostic issues and new treatments. We studied the medical records of 165 consecutive SSc patients and reviewed cases with scleroderma overlap syndrome. A PubMed search for the period 1977 to 2009 was conducted using the key words "overlap syndrome", "systemic sclerosis", "connective tissue disease" and "biological agents." Forty patients satisfied the criteria for scleroderma overlap syndrome. The incidence of additional connective tissue diseases in the whole group and in the overlap syndrome group respectively was: dermatomyositis or polymyositis 11.5% and 47.5%, Sjogren's syndrome 10.3% and 42.5%, rheumatoid arthritis 3.6% and 15.4%, and systemic lupus erythematosus 1.2% and 5.0%. Coexistence of SSc and another CTD aggravated the clinical course, especially lung, kidney, digestive, vascular and articular involvement. Coexisting non-rheumatic complications mimicked SSc complications. An additional rheumatic or non-rheumatic disease affected treatment choice. The definition of scleroderma overlap syndrome is important, especially in patients who need high-dose corticosteroids for complications of a CTD. The use of novel biological therapies may be advocated in these patients to avoid the hazardous influences of high-dose steroids, especially renal crisis. In some overlap syndrome cases, biological agents serve both conditions; in others one of the conditions may limit their use. In the absence of formal clinical trials in these patients a

  8. Pediatric vascularized composite allotransplantation.

    Science.gov (United States)

    Doumit, Gaby; Gharb, Bahar Bassiri; Rampazzo, Antonio; Papay, Francis; Siemionow, Maria Z; Zins, James E

    2014-10-01

    Vascularized composite allotransplantation (VCA) has experienced a growing acceptance, which has led to a debate centered on extending the indications of the procedure to include pediatric patients. The aim of this article was to discuss such indications based on the evidence in pediatric solid organ transplantation, reconstructive surgery in children, and VCA in adult patients. Papers published on the outcomes of pediatric solid organ transplantation, growth after replantation of extremities, vascularized autologous tissue transfer, craniofacial surgery, orthognathic procedures, facial fractures, and outcomes after repair of peripheral nerves in children were reviewed. Although the outcomes of solid organ transplantation in children have improved, the transplanted organs continue to have a limited lifespan. Long-term immunosuppressive therapy exposes the patients to an increased lifetime risk of infections, diabetes, hypertension, dyslipidemia, cardiovascular disease, and malignancy. Growth impairment and learning disabilities are other relevant drawbacks, which affect the pediatric recipients. Nonadherence to medication is a common cause of graft dysfunction and loss among the adolescent transplant recipients. Rejection episodes, hospitalizations, and medication adverse effects contribute negatively to the quality of life of the patients. Although normal growth after limb transplantation could be expected, pediatric facial transplant recipients may present with arrest of growth of transplanted midfacial skeleton. Considering the non-life-threatening nature of the conditions that lead to eligibility for VCA, it is suggested that it is premature to extend the indications of VCA to include pediatric patients under the currently available immunosuppressive protocols.

  9. Temporary vascular shunting in vascular trauma: A 10-year review ...

    African Journals Online (AJOL)

    Five patients with non-viable limbs had the vessel ligated. Conclusions. A TIVS in the damage control setting is both life- and limb-saving. These shunts can be inserted safely in a facility without access to a surgeon with vascular surgery experience if there is uncontrollable bleeding or the delay to definitive vascular surgery ...

  10. Temporary vascular shunting in vascular trauma: A 10-year review ...

    African Journals Online (AJOL)

    part of a damage control procedure, 7 patients were referred from a hospital without access to vascular surgical facilities with the TIVS in situ, and in the remaining 6 patients the TIVS was inserted during repair of a lower limb fracture with an associated vascular injury. Damage control procedure. Twenty-two patients had a ...

  11. Additive Manufacturing of Vascular Grafts and Vascularized Tissue Constructs.

    Science.gov (United States)

    Elomaa, Laura; Yang, Yunzhi Peter

    2017-10-01

    There is a great need for engineered vascular grafts among patients with cardiovascular diseases who are in need of bypass therapy and lack autologous healthy blood vessels. In addition, because of the severe worldwide shortage of organ donors, there is an increasing need for engineered vascularized tissue constructs as an alternative to organ transplants. Additive manufacturing (AM) offers great advantages and flexibility of fabrication of cell-laden, multimaterial, and anatomically shaped vascular grafts and vascularized tissue constructs. Various inkjet-, extrusion-, and photocrosslinking-based AM techniques have been applied to the fabrication of both self-standing vascular grafts and porous, vascularized tissue constructs. This review discusses the state-of-the-art research on the use of AM for vascular applications and the key criteria for biomaterials in the AM of both acellular and cellular constructs. We envision that new smart printing materials that can adapt to their environment and encourage rapid endothelialization and remodeling will be the key factor in the future for the successful AM of personalized and dynamic vascular tissue applications.

  12. Nutrition and vascular dementia.

    Science.gov (United States)

    Perez, L; Heim, L; Sherzai, A; Jaceldo-Siegl, K; Sherzai, A

    2012-04-01

    The objective of this review was to elucidate the relationship between VaD and various nutritional factors based on epidemiological studies. Vascular dementia (VaD) is the second most common type of dementia. The prevalence of VaD continues to increase as the US population continues to grow and age. Currently, control of potential risk factors is believed to be the most effective means of preventing VaD. Thus, identification of modifiable risk factors for VaD is crucial for development of effective treatment modalities. Nutrition is one of the main modifiable variables that may influence the development of VaD. A systematic review of literature was conducted using the PubMed, Web of Science, and CINAHL Plus databases with search parameters inclusive of vascular dementia, nutrition, and vascular cognitive impairment (VCI). Fourteen articles were found that proposed a potential role of specific nutritional components in VaD. These components included antioxidants, lipids, homocysteine, folate, vitamin B12, and fish consumption. Antioxidants, specifically Vitamin E and C, and fatty fish intake were found to be protective against VaD risk. Fried fish, elevated homocysteine, and lower levels of folate and vitamin B12 were associated with increased VaD. Evidence for dietary lipids was inconsistent, although elevated midlife serum cholesterol may increase risk, while late-life elevated serum cholesterol may be associated with decreased risk of VaD. Currently, the most convincing evidence as to the relationship between VaD and nutrition exists for micronutrients, particularly Vitamin E and C. Exploration of nutrition at the macronutrient level and additional long term prospective cohort studies are warranted to better understand the role of nutrition in VaD disease development and progression. At present, challenges in this research include limitations in sample size, which was commonly cited. Also, a variety of diagnostic criteria for VaD were employed in the studies

  13. The Danish Vascular Registry, Karbase

    Directory of Open Access Journals (Sweden)

    Eldrup N

    2016-10-01

    Full Text Available Nikolaj Eldrup,1,2 Charlotte Cerqueira,3 Louise de la Motte,2,4 Lisbet Knudsen Rathenborg,2,4 Allan K Hansen2,5 1Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, 2Karbase, The Danish Vascular Registry, Aarhus, 3Registry Support Centre (East – Epidemiology and Biostatistics, Research Centre for Prevention and Health, Capital Region of Denmark, 4Department of Vascular Surgery, Rigshospitalet, Copenhagen University Hospital, Copenhagen, 5Department of Vascular Surgery, Aalborg University Hospital, Aalborg, Denmark Aim: The Danish Vascular Registry (DVR, Karbase, is monitoring arterial and advanced vein interventions conducted at all vascular departments in Denmark. The main aim of the DVR is to improve the quality of treatment for patients undergoing vascular surgery in Denmark by using the registry for quality assessment and research. Study population: All patients undergoing vascular interventions (surgical and endovascular at any vascular department in Denmark are registered in the DVR. The DVR was initiated in 1989, and each year, ~9,000 procedures are added. By January 2016, .180,000 procedures have been recorded. Since 2001, data completeness has been .90% (compared to the Danish National Patient Register. Main variables: Variables include information on descriptive patient data (ie, age, sex, height, and weight and comorbidity (ie, previous cardiovascular disease and diabetes. Process variable includes waiting time (time from event to medical contact and treatment and the type of procedures conducted. Outcome variables for in-hospital complications (ie, wound complications, myocardial infarction, stroke, amputation, respiratory complications, and renal insufficiency and 30-day patency are submitted. Variables for medical treatment (antithrombotic and statin treatment, amputation, and survival are extracted from nationwide, administrative registers. Conclusion: The DVR reports outcome on key indicators for

  14. Duane Syndrome

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

  15. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  16. Aspects of Subcortical Ischaemic Vascular Disease : Early clinical manifestations and associations with Type 2 diabetes mellitus

    OpenAIRE

    Harten, van, B.

    2006-01-01

    Summary Subcortical ischaemic vascular disease (SIVD) is an important cause of cognitive impairment in elderly patients. Screening and diagnostic tests are needed to identify these patients. The HIV dementia scale (HDS) is a reliable and quantitative scale for identifying HIV dementia1. The cognitive profile of HIV dementia has subcortical features that resemble subcortical ischaemic vascular disease (SIVD). The clinical syndrome is characterized by early impairment of attention and executive...

  17. The role of radiotherapy in the management of POEMS syndrome

    OpenAIRE

    Suh, Yang-Gun; Kim, Young-Suk; Suh, Chang-Ok; Kim, Yu Ri; Cheong, June-Won; Kim, Jin Seok; Cho, Jaeho

    2014-01-01

    Background POEMS syndrome is a paraneoplastic syndrome caused by an underlying plasma cell proliferative disease. In this study, we examined the treatment outcomes and role of radiotherapy in the management of POEMS syndrome. Methods In total, 33 patients diagnosed with POEMS syndrome were analyzed. These patients presented with osteosclerotic myeloma (OSM, n = 13), Castleman’s disease (CD, n = 4), OSM with CD (n = 10), and vascular endothelial growth factor elevation without gross lesions (V...

  18. Dynamic adaption of vascular morphology

    DEFF Research Database (Denmark)

    Okkels, Fridolin; Jacobsen, Jens Christian Brings

    2012-01-01

    The structure of vascular networks adapts continuously to meet changes in demand of the surrounding tissue. Most of the known vascular adaptation mechanisms are based on local reactions to local stimuli such as pressure and flow, which in turn reflects influence from the surrounding tissue. Here ...

  19. Hamartomatous polyposis syndromes

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

    2014-01-01

    Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ...

  20. Nodular regenerative hyperplasia of the liver, CREST syndrome and primary biliary cirrhosis: an overlap syndrome?

    Science.gov (United States)

    McMahon, R F; Babbs, C; Warnes, T W

    1989-01-01

    Nodular regenerative hyperplasia of the liver (NRHL) has been found in association with collagen vascular diseases, after drug therapy, with autoimmune disease, and with a variety of haematological disorders. The association of NRHL with the syndrome of Calcinosis cutis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia (CREST syndrome) has only been reported on two previous occasions. The liver disease usually associated with CREST syndrome is primary biliary cirrhosis (PBC) and recently nodular hyperplasia of the liver has been reported in patients with early stage PBC. We present a case of NRHL with CREST syndrome and serological and biochemical features of PBC, a newly recognised overlap syndrome. Images Figure PMID:2583572

  1. [Intracranial hypertension in Proteus syndrome].

    Science.gov (United States)

    Dandine, J-B; James, S; Van Garsse, A; Born, J-D

    2007-11-01

    Proteus syndrome, described for the first time in 1979, is a sporadic congenital poly-malformation syndrome named for its highly variable manifestations. We report the case of a 36-year-old male patient with several malformations including skull hyperostosis and huge frontal sinus hypertrophy compressing the brain. He complained of increasing headache for 5 years. Cerebrospinal fluid pressure monitoring revealed severe hypertension. The patient underwent frontoparietal craniectomy, which allowed partial decompression. Postoperatively headaches decreased and the intracranial pressure normalized. Proteus syndrome is a genetic disease with a mosaic pattern. Only a hundred cases have been reported, mostly in childhood. Common manifestations include disproportionate overgrowth of the limbs and the skull, various subcutaneous tumors, vascular, renal and pulmonary malformations. Brain abnormalities are not common in this syndrome. When present, retardation or seizure disorders are typically seen. Intracranial hypertension is described for the first time in this syndrome.

  2. Nodular regenerative hyperplasia of the liver, CREST syndrome and primary biliary cirrhosis: an overlap syndrome?

    OpenAIRE

    McMahon, R F; Babbs, C; Warnes, T. W.

    1989-01-01

    Nodular regenerative hyperplasia of the liver (NRHL) has been found in association with collagen vascular diseases, after drug therapy, with autoimmune disease, and with a variety of haematological disorders. The association of NRHL with the syndrome of Calcinosis cutis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia (CREST syndrome) has only been reported on two previous occasions. The liver disease usually associated with CREST syndrome is primary biliary ci...

  3. Caffeine's Vascular Mechanisms of Action

    Directory of Open Access Journals (Sweden)

    Darío Echeverri

    2010-01-01

    Full Text Available Caffeine is the most widely consumed stimulating substance in the world. It is found in coffee, tea, soft drinks, chocolate, and many medications. Caffeine is a xanthine with various effects and mechanisms of action in vascular tissue. In endothelial cells, it increases intracellular calcium stimulating the production of nitric oxide through the expression of the endothelial nitric oxide synthase enzyme. Nitric oxide is diffused to the vascular smooth muscle cell to produce vasodilation. In vascular smooth muscle cells its effect is predominantly a competitive inhibition of phosphodiesterase, producing an accumulation of cAMP and vasodilation. In addition, it blocks the adenosine receptors present in the vascular tissue to produce vasoconstriction. In this paper the main mechanisms of action of caffeine on the vascular tissue are described, in which it is shown that caffeine has some cardiovascular properties and effects which could be considered beneficial.

  4. The Danish Vascular Registry, Karbase

    DEFF Research Database (Denmark)

    Eldrup, Nikolaj; Cerqueira, Charlotte; de la Motte, Louise

    2016-01-01

    AIM: The Danish Vascular Registry (DVR), Karbase, is monitoring arterial and advanced vein interventions conducted at all vascular departments in Denmark. The main aim of the DVR is to improve the quality of treatment for patients undergoing vascular surgery in Denmark by using the registry...... for quality assessment and research. STUDY POPULATION: All patients undergoing vascular interventions (surgical and endovascular) at any vascular department in Denmark are registered in the DVR. The DVR was initiated in 1989, and each year, ∼9,000 procedures are added. By January 2016, >180,000 procedures...... have been recorded. Since 2001, data completeness has been >90% (compared to the Danish National Patient Register). MAIN VARIABLES: Variables include information on descriptive patient data (ie, age, sex, height, and weight) and comorbidity (ie, previous cardiovascular disease and diabetes). Process...

  5. Social media in vascular surgery.

    Science.gov (United States)

    Indes, Jeffrey E; Gates, Lindsay; Mitchell, Erica L; Muhs, Bart E

    2013-04-01

    There has been a tremendous growth in the use of social media to expand the visibility of various specialties in medicine. The purpose of this paper is to describe the latest updates on some current applications of social media in the practice of vascular surgery as well as existing limitations of use. This investigation demonstrates that the use of social networking sites appears to have a positive impact on vascular practice, as is evident through the incorporation of this technology at the Cleveland Clinic and by the Society for Vascular Surgery into their approach to patient care and physician communication. Overall, integration of social networking technology has current and future potential to be used to promote goals, patient awareness, recruitment for clinical trials, and professionalism within the specialty of vascular surgery. Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

  6. Complex regional pain syndromes (CRPS) type 1 validating case histories

    National Research Council Canada - National Science Library

    P. Berger

    2003-01-01

    The treatment of patients with complex regional pain syndrome (CRPS) type 1 is challenging and unpredictable as the condition presents with vascular and neuropathic symptoms after nil or even minor injury to a peripheral nerve...

  7. [Central blood pressure and vascular damage].

    Science.gov (United States)

    Pérez-Lahiguera, Francisco; Rodilla, Enrique; Costa, José Antonio; Pascual, José María

    2015-07-20

    The aim of this study was to assess the relationship between central blood pressure and vascular damage. This cross-sectional study involved 393 never treated hypertensive patients (166 women). Clinical blood pressure (BP), 24h blood pressure (BP24h) and central blood pressure (CBP) were measured. Vascular organ damage (VOD) was assessed by calculating the albumin/creatinine ratio (ACR), wave pulse pressure velocity and echocardiographic left ventricular mass index (LVMI). Patients with VOD had higher values of BP, BP24h, and CBP than patients without ACR. When comparing several systolic BP, systolic BP24h had a higher linear correlation with CBP (Z Steiger test: 2.26; P=.02) and LVMI (Z Steiger test: 3.23; P=.01) than PAC. In a multiple regression analysis corrected by age, sex and metabolic syndrome, all pressures were related with VOD but systolic BP24h showed the highest correlation. In a logistic regression analysis, having the highest tercile of systolic BP24h was the stronger predictor of VOD (multivariate odds ratio: 3.4; CI 95%: 2.5-5.5, P=.001). CBP does not have more correlation with VOD than other measurements of peripheral BP. Systolic BP24h is the BP measurement that best predicts VOD. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  8. Noninfarct vascular dementia and Alzheimer dementia spectrum.

    Science.gov (United States)

    Emery, V Olga; Gillie, Edward X; Smith, Joseph A

    2005-03-15

    Vascular dementia is an overarching superordinate category of which multiinfarct vascular dementia is only one subtype. To contribute to the definition of vascular dementia, method involved investigation of mental status, oral language and comprehension in 81 consecutive vascular patients comprising two vascular samples: cerebral infarct sample (n=43) and cerebral noninfarct sample (n=38). To determine baseline, method also involved investigation of 36 demographically equivalent normal elderly. Results indicate both vascular samples performed significantly worse than normal elderly. Results further indicate there were no robust, reliable, significant differences between cerebral infarct and cerebral noninfarct patients. The lack of significant differences between cerebral infarct and cerebral noninfarct vascular samples brings into focus the ambiguous transition between diffuse, generalized disease and the multifocality underlying the vascular dementia-Alzheimer dementia spectrum. Cross-cutting infarct and noninfarct vascular populations were vascular factors of arteriosclerosis, abnormal blood pressure, diabetes mellitus, abnormal electrocardiogram, peripheral vascular disease, and other variables implicated in the distal causality of both infarct and noninfarct vascular dementias. Results indicate cerebral infarction is not the only path to the final common phenotype of vascular dementia. Vascular dementia is reconceptualized so as to include noninfarct vascular dementia: vascular dementia caused by underlying vascular factors other than cerebral infarction. It is suggested that one form of the subtype of noninfarct vascular dementia is Alzheimer-type vascular dementia.

  9. Update on management of ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Chin-Der Chen

    2011-03-01

    Full Text Available Ovarian hyperstimulation syndrome (OHSS is a relatively common complication of ovarian stimulation and can be life threatening. The pathophysiology of OHSS is characterized by increased capillary permeability, leading to leakage of fluid from the vascular compartment, with third-space fluid accumulation and intravascular dehydration. The increased intra-abdominal pressure indicated that OHSS may be considered a compartment syndrome. Vascular endothelial growth factor, also known as vascular permeability factor, has emerged as one of the mediators intrinsic to the development of OHSS. Conventional management is focused on supportive care until the spontaneous resolution of the condition. The standard of care for treatment—monitoring of appropriate clinical parameters, fluid balance management, thrombosis prophylaxis, and ascites treatment—should prevent severe morbidity in most cases. This review will cover inpatient and outpatient management. The potential therapeutic approach targeting the vascular endothelial growth factor system will be discussed.

  10. Ehlers-Danlos Syndrome in Orthopaedics

    Science.gov (United States)

    Shirley, Eric D.; DeMaio, Marlene; Bodurtha, Joanne

    2012-01-01

    Ehlers-Danlos syndrome is a heterogeneous connective tissue condition characterized by varying degrees of skin hyperextensibility, joint hypermobility, and vascular fragility. Joint dislocations, musculoskeletal pain, atrophic scars, easy bleeding, vessel/viscera rupture, severe scoliosis, and obstetric complications may occur. These manifestations are secondary to abnormal collagen, with specific molecular defects in types I, III, and V collagen; they may also be related to tenascin-X, which has been identified in some patients. Ehlers-Danlos syndrome has been classified into 6 types, with variable degrees of joint instability, skin hyperextensibility, wound healing difficulty, and vascular fragility. Diagnosis begins with recognition of the signs and symptoms of global hypermobility and referring appropriate patients for genetic consultation. It is important to accurately identify patients with Ehlers-Danlos syndrome to initiate appropriate musculoskeletal treatment, optimize anesthetic and postoperative management, perform appropriate vascular screening, and help families address their concerns with other families and advocacy groups. PMID:23016112

  11. Association of germline mutation in the PTEN tumour suppressor gene and Proteus and Proteus-like syndromes

    NARCIS (Netherlands)

    Zhou, X.; Hampel, H.; Thiele, H.; Gorlin, R. J.; Hennekam, R. C.; Parisi, M.; Winter, R. M.; Eng, C.

    2001-01-01

    The molecular aetiology of Proteus syndrome (PS) remains elusive. Germline mutations in PTEN cause Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome, which are hereditary hamartoma syndromes. Some features-eg, macrocephaly, lipomatosis, and vascular malformations-can be seen in all three

  12. Epigenetic Diabetic Vascular Complications

    Directory of Open Access Journals (Sweden)

    Ali Ahmadzadeh-Amiri

    2016-01-01

    Full Text Available Diabetic vascular complications (DVC influence several vital organ systems including cardiovascular, renal, ocular and nervous systems making it a major public health problem. Although extensive researches were performed in this field, the exact mechanisms responsible for these organ damages in diabetes remain obscure. Several metabolic disturbances have been involved in its complication and change in genes associated with these pathways occurred. Gene expression to produce a biologically active protein can be controlled by transcriptional and translational alteration on the head of genes without change in nucleotide composition. These epigenetic adjustments are steady, but possibly reversible and can be transmitted to future generation. Gene expression can be regulated by three epigenetic mechanisms including DNA methylation, histone modifications and noncoding microRNAs (miRNAs activity. Epigenetic studies must be directed to better realize the role of epigenetic changes to the etiology of DVC and knowledge of epigenetic would play a pivotal role in the application of individualized medicine. Application and development of high technology sequencing combined with more sensitive and advanced methodologies for epigenome studying help to determine specific epigenetic events that stimulate gene responses in patients with diabetes mellitus.

  13. [Extremity vascular traumas].

    Science.gov (United States)

    Angelini, Romeo; Rutolo, Ferdinando; Cozzolino, Giuseppe; D'Amario, Vanessa; Spigonardo, Francesca

    2005-01-01

    The Authors report on a series of 61 vascular traumas treated over a 7 years, separated in two groups. The first one includes 35 cases, that are street accidents, on the work and gunshot wounds. The second group includes 26 iatrogenic causes due to arterial catheterism. All patients underwent ecocolor Doppler directly in the operating theatre and, when this diagnostic procedure was not enough, pre-operating angiography was used (10 cases of complex traumas of the lower limb). One death was reported far each groups (3.27%). In 55 cases (90.1%), limb savage was achieved. In the others 4 (6.93%) of the first group, limb demolition was necessary for different causes. In the first group, severe neurological sequelaes were observed in 2 cases and motor deficits caused by tendon lesions in 1 case. The good results obtained are the result of the short ischemic interval between the acute event and treatment, thanks to a multidisciplinary approach of a specific equipe, that is rapid as possible.

  14. Vascular injuries during gynecological laparoscopy: the vascular surgeon's advice

    Directory of Open Access Journals (Sweden)

    Marcello Barbosa Barros

    Full Text Available CONTEXT: Iatrogenic vascular problems due to laparoscopy are a well recognized problem and lead to significant repercussions. In this context, a ten-year review of cases topic is presented, based on experience gained while heading two important vascular surgery services. CASES: Five patients with vascular injuries during elective laparoscopy are described. These patients presented with seven lesions of iliac vessels. All cases were evaluated immediately and required laparotomy, provisional hemostasis and urgent attendance by a vascular surgeon. Direct suturing was performed in three cases. One aortoiliac bypass and one ilioiliac reversed venous graft were made. Venous lesions were sutured. One case of a point-like perforation of the small bowel was found. There were no deaths and no complications during the postoperative period. DISCUSSION: Important points on this subject are made, and advice is given. There needs to be immediate recognition of the vascular injury, and expert repair by a vascular surgeon is recommended, in order to significantly reduce the degree of complications.

  15. Trauma vascular, visión del cirujano vascular

    Directory of Open Access Journals (Sweden)

    Dr. D. Cristián Salas

    2011-09-01

    Full Text Available El 3% de todas las lesiones en trauma tiene un componente vascular. Con los conflictos armados del siglo pasado se lograron grandes avances en este campo. A partir de la Guerra de Vietnam gracias a las mejoras en el manejo prehospitalario, traslado de pacientes, y avances en técnica quirúrgica se lograron tasas de sobrevida y de amputaciones que se han mantenido estables hasta la fecha. El diagnóstico de lesiones vasculares en extremidades se realiza con el examen físico, sin embargo las lesiones de vasos torácicos y abdominales requieren de imágenes de apoyo, siempre que el paciente se encuentre estabilizado, generalmente tomografía axial computada. La mayoría de las lesiones vasculares son por trauma penetrante, comprometiendo principalmente las extremidades. Con el desarrollo de los procedimientos invasivos vasculares en los últimos años se ha observado un aumento de lesiones vasculares iatrogénicas. Hoy en día muchos pacientes con trauma vascular son manejados por vía endovascular.

  16. Role of preoperative vascular ultrasonography in hemodialysis vascular access operation.

    Science.gov (United States)

    Siribumrungwong, Boonying; Tomtitchong, Prakitpunthu; Kanpirom, Kitti

    2010-12-01

    Preoperative vascular mapping increase rate of successful hemodialysis vascular access operation. Several studies recommend using this procedure routinely. But some studies recommend using this procedure in selected patients. So this study aims to determine the impacts of preoperative vascular mapping in unfavorable-examined patients. 55 patients were studied retrospectively from August 2006 to October 2009. Before April 2008, the operative plans were based on physical examination (group 1). After April 2008, the surgeon did preoperative vascular mapping prior to the operation in unfavorable-examined patients (group 2). The results were compared. There were high maturation rates in favorable-examined patients. In unfavorable-examined patients, preoperative vascular mapping can identified nonpalpable favorable vein which successful maturation of 18.75%. Complementary duplex scan decrease rate of unsuccessful operation significantly (p = 0.037) but does not increase maturation rate. Careful physical examination is important part before operation. Preoperative vascular mapping has benefit only in patients with unfavorable-examined patients. It finds some nonpalpable favorable vein and decrease unsuccessful exploration.

  17. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Pınar Zeyneloğlu

    2012-04-01

    Full Text Available Renal failure is a common major complication in patients with advanced cirrhosis and generally indicates a poor prognosis when combined with liver failure. Hepatorenal syndrome (HRS is characterised by a combination of disturbances in circulatory and kidney function. Arterial pressure is decreased in the systemic circulation due to reduced total systemic vascular resistance. Kidney dysfunction is caused by reduction in renal blood flow. The diagnosis of HRS is based on exclusion of other disorders that cause acute kidney injury in cirrhosis as there are no specific tests. There are two types of HRS with different characteristics and prognostics. Liver transplantation is the treatment of choice for all patients without contraindication. The best approach to the pharmacologic management is the administration vasoconstrictor drugs based on the pathogenesis. Many vasoconstrictors including vasopressin analogues (terlipressin, ornipressin and vasopressin, somatostatin analogues (octreotide and alpha-adrenergic analogues (midodrine and norepinephrine have been studied. In most of the studies intravenous albumin therapy was coadministered with vasoconstrictor drugs and suggested that albumin should be considered as the component of pharmacologic intervention in patients with HRS. Renal replacement therapy in the form of hemodialysis or continuous venovenous hemofiltration has been used in the management of HRS patients awaiting transplantation or in those with acute potentially reversible conditions. The artificial hepatic support systems require further investigation. (Journal of the Turkish Society Intensive Care 2012; 10: 37-44

  18. Diabetes and Retinal Vascular Dysfunction

    Directory of Open Access Journals (Sweden)

    Eui Seok Shin

    2014-01-01

    Full Text Available Diabetes predominantly affects the microvascular circulation of the retina resulting in a range of structural changes unique to this tissue. These changes ultimately lead to altered permeability, hyperproliferation of endothelial cells and edema, and abnormal vascularization of the retina with resulting loss of vision. Enhanced production of inflammatory mediators and oxidative stress are primary insults with significant contribution to the pathogenesis of diabetic retinopathy (DR. We have determined the identity of the retinal vascular cells affected by hyperglycemia, and have delineated the cell autonomous impact of high glucose on function of these cells. We discuss some of the high glucose specific changes in retinal vascular cells and their contribution to retinal vascular dysfunction. This knowledge provides novel insight into the molecular and cellular defects contributing to the development and progression of diabetic retinopathy, and will aid in the development of innovative, as well as target specific therapeutic approaches for prevention and treatment of DR.

  19. ( Elaeis guineensis Jacq ) vascular wilt

    African Journals Online (AJOL)

    Effet de la jachére sur l'expérimentation de la fusariose vasculaire du palmier à huile ( Elaeis guineensis Jacq ) : Effects of the fallow in the expression of oil-palm ( Elaeis guineensis Jacq ) vascular wilt.

  20. Astronaut Preflight Cardiovascular Variables Associated with Vascular Compliance are Highly Correlated with Post-Flight Eye Outcome Measures in the Visual Impairment Intracranial Pressure (VIIP) Syndrome Following Long Duration Spaceflight

    Science.gov (United States)

    Otto, Christian; Ploutz-Snyder, R.

    2015-01-01

    The detection of the first VIIP case occurred in 2005, and adequate eye outcome measures were available for 31 (67.4%) of the 46 long duration US crewmembers who had flown on the ISS since its first crewed mission in 2000. Therefore, this analysis is limited to a subgroup (22 males and 9 females). A "cardiovascular profile" for each astronaut was compiled by examining twelve individual parameters; eleven of these were preflight variables: systolic blood pressure, pulse pressure, body mass index, percentage body fat, LDL, HDL, triglycerides, use of anti-lipid medication, fasting serum glucose, and maximal oxygen uptake in ml/kg. Each of these variables was averaged across three preflight annual physical exams. Astronaut age prior to the long duration mission, and inflight salt intake was also included in the analysis. The group of cardiovascular variables for each crew member was compared with seven VIIP eye outcome variables collected during the immediate post-flight period: anterior-posterior axial length of the globe measured by ultrasound and optical biometry; optic nerve sheath diameter, optic nerve diameter, and optic nerve to sheath ratio- each measured by ultrasound and magnetic resonance imaging (MRI), intraocular pressure (IOP), change in manifest refraction, mean retinal nerve fiber layer (RNFL) on optical coherence tomography (OCT), and RNFL of the inferior and superior retinal quadrants. Since most of the VIIP eye outcome measures were added sequentially beginning in 2005, as knowledge of the syndrome improved, data were unavailable for 22.0% of the outcome measurements. To address the missing data, we employed multivariate multiple imputation techniques with predictive mean matching methods to accumulate 200 separate imputed datasets for analysis. We were able to impute data for the 22.0% of missing VIIP eye outcomes. We then applied Rubin's rules for collapsing the statistical results across our 200 multiply imputed data sets to assess the canonical

  1. Heritability of Retinal Vascular Fractals

    DEFF Research Database (Denmark)

    Vergmann, Anna Stage; Broe, Rebecca; Kessel, Line

    2017-01-01

    Purpose: To determine the genetic contribution to the pattern of retinal vascular branching expressed by its fractal dimension. Methods: This was a cross-sectional study of 50 monozygotic and 49 dizygotic, same-sex twin pairs aged 20 to 46 years. In 50°, disc-centered fundus photographs, the reti...... vasculature may affect the retinal response to potential vascular disease in later life....

  2. EXAMINATION RESULTS OF CHILDREN WITH CERVICAL SYNDROME

    Directory of Open Access Journals (Sweden)

    N. H. Bakhteeva

    2010-01-01

    Full Text Available By the example of examination of 80 children aged from 4 to 18 with cervical syndrome it is indicated, that the diagnosed abnormalities of hemodynamics in vertebrobasilar basin in patients of all age groups are connected both with bone and vascular pathology of the cervical part of the spine. The pathology has functional or congenital character. Early detection of discicirculatory vascular injuries in the cervical part of the spine in children with cervical syndrome will allow to define the therapeutic management of patients and to prolong juvenile osteochondrosis clinical behaviour.

  3. The Danish Vascular Registry, Karbase

    Science.gov (United States)

    Eldrup, Nikolaj; Cerqueira, Charlotte; de la Motte, Louise; Rathenborg, Lisbet Knudsen; Hansen, Allan K

    2016-01-01

    Aim The Danish Vascular Registry (DVR), Karbase, is monitoring arterial and advanced vein interventions conducted at all vascular departments in Denmark. The main aim of the DVR is to improve the quality of treatment for patients undergoing vascular surgery in Denmark by using the registry for quality assessment and research. Study population All patients undergoing vascular interventions (surgical and endovascular) at any vascular department in Denmark are registered in the DVR. The DVR was initiated in 1989, and each year, ∼9,000 procedures are added. By January 2016, >180,000 procedures have been recorded. Since 2001, data completeness has been >90% (compared to the Danish National Patient Register). Main variables Variables include information on descriptive patient data (ie, age, sex, height, and weight) and comorbidity (ie, previous cardiovascular disease and diabetes). Process variable includes waiting time (time from event to medical contact and treatment) and the type of procedures conducted. Outcome variables for in-hospital complications (ie, wound complications, myocardial infarction, stroke, amputation, respiratory complications, and renal insufficiency) and 30-day patency are submitted. Variables for medical treatment (antithrombotic and statin treatment), amputation, and survival are extracted from nationwide, administrative registers. Conclusion The DVR reports outcome on key indicators for monitoring the quality at all vascular departments in Denmark for the purpose of quality improvement. Furthermore, data are available for research and are being used in international collaborations on changes in clinical practices. PMID:27822118

  4. [Cutaneous vascular anomalies in children].

    Science.gov (United States)

    Yilmaz, L; Kacenelenbogen, N

    2015-09-01

    Vascular anomalies, which are erroneously categorized under the term angiomas, are a highly heterogeneous group of lesions that are poorly understood and affect a mean of 5 to 10 % of children. The fortuitous discovery of propranolol's efficacy in one of these entities has made them a topical issue. The paper's main objective is to inform family doctors of the various types of vascular anomalies, clarify their classification, and provide a common terminology. Its secondary objective is to provide a decision tree that enables primary care doctors to avoid diagnostic pitfalls, successfully detect cases, and optimize management. Systematic review. According to a recent study, 71,3 % of publications use the term hemangioma erroneously, regardless of the authors' field. The key for family doctors is to use one international classification only, that of the International Society for the Study of Vascular Anomalies (ISSVA), in order to facilitate management and comprehension between the different healthcare levels. The diagnosis of vascular anomalies is clinical in 90 % of cases, so all family doctors can, whilst using a decision tree, diagnose a vascular anomaly and refer only those that are complex for specialist care. The most common vascular anomaly is infantile hemangioma in infants, which spontaneously regresses around the age of 5-7 years in 90 % of cases. Watchful waiting and regular follow-up suffice, therefore, in such settings.

  5. Sturge-Weber syndrome

    Directory of Open Access Journals (Sweden)

    Natarajan Manivannan

    2012-01-01

    Full Text Available Encephalotrigeminal angiomatosis (Sturge-Weber syndrome is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

  6. Hemiplegia Following Mild Head Injury in a Child with Sturge-Weber Syndrome - A Diagnostic Dilemma.

    Science.gov (United States)

    Ahmed, Shameem; Paul, Siba Prosad

    2016-08-01

    Sturge-Weber syndrome (SWS) is a neurocutaneous disorder with skin, eye, and brain involvement. Hemiplegia in children with SWS after a mild head injury is known to occur in up to one-fifth of cases. A3-year male child presented with a sudden onset hemiplegia following a mild head injury. He was known to have seizure disorder and was being treated with sodium valproate. CTscan of the brain showed contusion. He was admitted for neurological observations and the patient made complete recovery with conservative treatment. MRI scan of the brain done 5 days later which showed venous malformation of choroid plexus on the left side. These changes were considered to be consistent with a preexisting cerebral lesion which coincidentally got detected at neuroimaging done after the mild head injury. There is need for good seizure control as it is likely to be associated with better neurological outcome. The case emphasizes the need for clinical correlation with findings at neuroimaging in children with SWS presenting with head injuries.

  7. Williams syndrome

    Science.gov (United States)

    A support group can be helpful for emotional support and for giving and receiving practical advice. The following organization provides additional information about Williams Syndrome: Williams Syndrome Association -- www.williams-syndrome.org

  8. WIEDEMANN SYNDROME

    African Journals Online (AJOL)

    hi-tech

    BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH - WIEDEMANN. SYNDROME: CASE REPORT. P. ANOOP and M. A. ANJAY. SUMMARY. Beckwith-Wiedemann syndrome is the most common overgrowth malformation syndrome. The classical features include macrosomia, macroglossia, ...

  9. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

  10. Brown Syndrome

    Science.gov (United States)

    ... extraction) have also been linked to acquired Brown syndrome. Inflammation of the tendon-trochlea complex (from adult and juvenile rheumatoid arthritis, systemic lupus erythematosus and sinusitis) can be ... syndrome hereditary? Hereditary cases of Brown syndrome are rare. ...

  11. Asperger Syndrome

    Science.gov (United States)

    ... Page You are here Home » Disorders » All Disorders Asperger Syndrome Information Page Asperger Syndrome Information Page What research is being done? ... Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Asperger syndrome (AS) is a developmental disorder. It is ...

  12. Vascular endothelial growth factor coordinates islet innervation via vascular scaffolding

    Science.gov (United States)

    Reinert, Rachel B.; Cai, Qing; Hong, Ji-Young; Plank, Jennifer L.; Aamodt, Kristie; Prasad, Nripesh; Aramandla, Radhika; Dai, Chunhua; Levy, Shawn E.; Pozzi, Ambra; Labosky, Patricia A.; Wright, Christopher V. E.; Brissova, Marcela; Powers, Alvin C.

    2014-01-01

    Neurovascular alignment is a common anatomical feature of organs, but the mechanisms leading to this arrangement are incompletely understood. Here, we show that vascular endothelial growth factor (VEGF) signaling profoundly affects both vascularization and innervation of the pancreatic islet. In mature islets, nerves are closely associated with capillaries, but the islet vascularization process during embryonic organogenesis significantly precedes islet innervation. Although a simple neuronal meshwork interconnects the developing islet clusters as they begin to form at E14.5, the substantial ingrowth of nerve fibers into islets occurs postnatally, when islet vascularization is already complete. Using genetic mouse models, we demonstrate that VEGF regulates islet innervation indirectly through its effects on intra-islet endothelial cells. Our data indicate that formation of a VEGF-directed, intra-islet vascular plexus is required for development of islet innervation, and that VEGF-induced islet hypervascularization leads to increased nerve fiber ingrowth. Transcriptome analysis of hypervascularized islets revealed an increased expression of extracellular matrix components and axon guidance molecules, with these transcripts being enriched in the islet-derived endothelial cell population. We propose a mechanism for coordinated neurovascular development within pancreatic islets, in which endocrine cell-derived VEGF directs the patterning of intra-islet capillaries during embryogenesis, forming a scaffold for the postnatal ingrowth of essential autonomic nerve fibers. PMID:24574008

  13. Paraneoplastic endocrine-metabolic syndromes

    Directory of Open Access Journals (Sweden)

    Marco Grandi

    2013-04-01

    Full Text Available BACKGROUND The paraneoplastic syndromes (PS are characterized by the presence of biochemical alterations, signs and symptoms expressive of cancer distance action into the patient’s organism. Sometimes these syndromes can precede the evidence of malignancy even of some years or can correspond to cancer relapse. PS, even being characterized by general symptoms (fever, anorexia, cachexia, may occur with neurological, rheumathological, osteoarticular, vascular, haematological, nephrological and endocrinological/metabolic symptoms; the latter ones are discussed in this article. AIM OF THE STUDY Here we will focus on the most common PS: paraneoplastic hypercalcemia, inappropriate secretion of antidiuretic hormone (SIADH and paraneoplastic Cushing syndrome. CONCLUSIONS Our work can be useful in the diagnosis and therapeutic management of paraneoplastic syndromes.

  14. Contribution of the cerebral SPECT in the field of evaluation of the hemodynamic cerebral vascular accident risk in the Limb shaking syndrome; Apport de la TEMP cerebrale dans le cadre de l'evaluation du risque d'AVC hemodynamique dans le Limb Shaking Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Lauer, V.; Wolff, V.; Marescaux, C. [CHU de Strasbourg, Unite neurovasculaire, service de neurologie, 67 (France); Namer, I.J. [CHU de Strasbourg, service de biophysique et medecine nucleaire, 67 -Strasbourg (France)

    2010-07-01

    The limb shaking syndrome (L.S.S.) is characterized by uncontrollable shaking of members that are caused by a passage in the upright or by an hyper extension of the neck and occur in a patient with internal carotid stenosis. To investigate the pathophysiology of L.S.S. we used brain SPECT (SPECT-E.C.D. or H.M.P.A.O.) to measure cerebral perfusion in the supine position and standing in three patients. (N.C.)

  15. Accurate and Simple Evaluation of Vascular Anastomoses in Monochorionic Placenta using Colored Dye

    NARCIS (Netherlands)

    Lopriore, Enrico; Slaghekke, Femke; Middeldorp, Johanna M.; Klumper, Frans J.; van Lith, Jan M.; Walther, Frans J.; Oepkes, Dick

    2011-01-01

    The presence of placental vascular anastomoses is a conditio sine qua non for the development of twin-to-twin transfusion syndrome (TTTS) and twin anemia polycythemia sequence (TAPS)(1,2). Injection studies of twin placentas have shown that such anastomoses are almost invariably present in

  16. Incidental radiological discovery of a hydroclayx by a reno-vascular ...

    African Journals Online (AJOL)

    A 47-year old patient without significant antecedents consultedfor a single episode of hematuria. The urine culture was negative. CT highlighted a hydrocalycosis related to extrinsic compression by a vascular pedicle. It corresponds to Fraley's syndrome. The patient was asymptomatic so the treatment consisted of a survey ...

  17. PTEN hamartoma tumor syndrome and Gorham-Stout phenomenon

    NARCIS (Netherlands)

    Hopman, Saskia M. J.; van Rijn, Rick R.; Eng, Charis; Bras, Johannes; Alders, Marielle; van der Horst, Chantal M.; Hennekam, Raoul C. M.; Merks, Johannes H. M.

    2012-01-01

    PTEN hamartoma tumor syndrome (PHTS) is a group of syndromes caused by mutations in PTEN. GorhamStout phenomenon (GSP) is a rare condition characterized by proliferation of vascular structures in bones, resulting in progressive osteolysis. Here we present a 1-year-old boy with PHTS and GSP. The

  18. Degenerative disk vascularization on MRI: correlation with clinical and histopathologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Staebler, A. [Dept. of Diagnostic Radiology, Klinikum Grosshadern, Muenchen (Germany); Scheidler, J. [Dept. of Diagnostic Radiology, Klinikum Grosshadern, Muenchen (Germany); Seiderer, M. [Dept. of Diagnostic Radiology, Klinikum Grosshadern, Muenchen (Germany); Reiser, M. [Dept. of Diagnostic Radiology, Klinikum Grosshadern, Muenchen (Germany); Weiss, M. [Dept. of Pathology, Klinikum Grosshadern, Muenchen (Germany); Kroedel, A. [Dept. of Orthopedic Surgery, Klinikum Grosshadern, Muenchen (Germany)

    1996-02-01

    Fifty-tree patients with localized painful spine syndrome were investigated prospectively by contrast-enhanced MRI. Pain was not predominantly radiating and there was no clinical evidence of spinal infection. In all patients, sagittal SE T1-weighted, fast-SE T2-weighted or turbo-STIR, and T1-weighted frequency-selective fat-suppressed images were obtained. We identified 37 vascularized disks in 26 patients. In 18 patients the changes had occurred spontaneously, in 6, the affected disk had been operated on previously, and 2 patients had spondylolisthesis. In 15 patients, vascularization was accompanied by medullary edema adjacent to the vertebral endplates. In one of the vascularized disks, herniation was also found. In seven patients, ventral diskectomy was performed. Histopathologic findings confirmed disk vascularization in six of seven cases. Degenerative, band-like disk vascularization is a feature which is associated with local pain. It is demonstrated by contrast-enhanced MRI. Degenerative disk vascularization is an important differential diagnosis to bacterial spondylodiskitis. It can be a cause of pain in patients with postdiskectomy syndrome. (orig./MG)

  19. Sleep and vascular disorders.

    Science.gov (United States)

    Plante, Gérard E

    2006-10-01

    It is not surprising that cardiovascular diseases such as congestive heart failure and coronary insufficiency can give rise to varying degrees of sleep impairment; it is less readily appreciated that certain physiologic events occurring during sleep-as well as long-term unsatisfactory sleep-may cause or increase the risk of cardiovascular conditions such as hypertension, atherosclerosis, stroke, and cardiac arrythmias. Heart rate abnormalities during sleep in normotensive subjects predict later cardiovascular disease, and their early identification alerts the physician to undertake preventive measures. Maneuvers, such as induction of hypoxia, can elicit abnormal blood pressure responses during sleep, and such responses have been used to identify impending cardiovascular problems that could become therapeutic targets. The spontaneously hypertensive rat has been used to examine the effect of sympathetic nervous system (SNS) activity on the heart under a variety of experimental conditions, including quiet and paradoxical sleep. The results have disclosed significant differences between the responses of spontaneously hypertensive rats and normal rats to SNS stimulation. Exploration of other pathophysiologic pathways affected by exposure to light and dark, including those responsive to the cyclic production of melatonin, will improve our understanding of the effect of disruptions of the circadian cycle on cardiovascular function. There is growing evidence that melatonin can influence important processes such as fluid, nitrogen, and acid-base balance. Human subjects whose nocturnal arterial blood pressure fails to show the "normal" decrement during sleep ("nondippers") are also prone to sleep poorly, exhibit increased SNS activity during sleep, and have an increased risk of total and cardiovascular disease mortality. Chronic sleep deficit is now known to be a risk factor for obesity and may contribute to the visceral form of obesity that underlies the metabolic syndrome

  20. From placenta to podocyte: vascular and podocyte pathophysiology in preeclampsia.

    Science.gov (United States)

    Wagner, Steven J; Craici, Iasmina M; Grande, Joseph P; Garovic, Vesna D

    2012-09-01

    Preeclampsia is a disorder of hypertension and proteinuria that affects 6 - 8% of normal pregnancies. Recent research has revealed many molecular mechanisms that may contribute to systemic endothelial dysfunction, glomerular capillary endotheliosis, dysregulation of the glomerular filtration apparatus, and podocyte loss. An ischemic placenta elaborates soluble FMS-like tyrosine kinase 1 (sFlt-1), a soluble receptor for vascular endothelial growth factor (VEGF). A variety of mediators, including nitric oxide, Angiotensin II receptor autoantibodies (AT1AA), and endothelin-1 may serve to maintain placental ischemia and systemic endothelial dysfunction. Endothelin-1 and decreased vascular endothelial growth factor may adversely affect overall expression and distribution of podocyte foot process proteins, leading to proteinuria. Podocyte derangements may lead to podocyte apoptosis and loss, as evidenced by the detection of live podocytes and podocyte products in the urine of preeclamptic women. In this review, we explore recent research elucidating the interactions of placenta, endothelium, and podocyte leading to the clinical syndrome of preeclampsia.

  1. [Cockett's syndrome, May-Thurner syndrome, or iliac vein compression syndrome].

    Science.gov (United States)

    Gil Martín, A R; Carreras Aja, M; Arrieta Ardieta, I; Labayen Azparren, I

    2014-01-01

    Iliac vein compression syndrome (also known as May-Thurner syndrome or Cockett's syndrome) is a rare clinical entity in which the left common iliac vein is compressed when it passes between the right common iliac artery and the spine. The sustained compression and trauma caused by the pulsatile force of the artery on the vein damage the intima and lead to the formation of membranes or bands in the vascular lumen that hinder or obstruct the flow of blood in the vein, favoring thrombus formation. The current treatment strategy of choice is endovascular vein patch angioplasty and stenting with the aim of improving the caliber of the lumen and enabling normal venous drainage. We present two cases of May-Thurner syndrome and review the clinical and CT findings. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  2. Imaging of peripheral vascular disease

    Directory of Open Access Journals (Sweden)

    Mo Al-Qaisi

    2009-03-01

    Full Text Available Mo Al-Qaisi1, David M Nott1, David H King1, Sam Kaddoura2, Mo Hamady31Charing Cross Hospital, London, UK; 2Royal Brompton Hospital, London, UK; 3St. Mary’s Hospital, London, UKAbstract: This illustrated review article gives an evidence-based update on the different modalities used for imaging peripheral vascular disease (duplex ultrasound, computed tomography angiography, magnetic resonance angiography, and digital subtraction angiography. After discussing the latest technological developments for each modality, their limitations are also highlighted. The evidence is presented for the various modalities’ roles in the imaging of peripheral vascular disease, including problem-solving applications. The strengths and weaknesses of each modality are therefore critically appraised, including the salient technological, clinical, and financial aspects. This review allows the general and specialist practitioner to make an informed decision on how best to deploy imaging tests in peripheral vascular disease as part of an evidence-based approach. The article concludes with a rational imaging algorithm for the investigation of peripheral vascular disease.Keywords: imaging, peripheral, vascular, duplex, angiography, arterial 

  3. Optimal treatment of vascular birthmarks.

    Science.gov (United States)

    Werner, Jochen A; Dünne, Anja A; Lippert, Burkard M; Folz, Benedikt J

    2003-01-01

    Optimal functional and cosmetic outcomes for vascular lesions require a thorough preoperative diagnosis of vascular anomalies, since correct classification of each vascular lesion has a direct influence on the treatment of choice. Many different classification systems have been discussed controversially. Based on clinical practicality and significant accuracy of forecast with regard to the clinical course of a lesion, Mulliken and Glowacki's biologic classification gained most clinical relevance and has become accepted as the official classification scheme by the International Society for the Study of Vascular Anomalies (ISSVA). Based on comprehensive description of relevant literature results, the current communication shall give an overview of differing, internationally accepted treatments. Although conservative management can be proposed for uncomplicated hemangiomas occurring in infancy, the proliferative progression of these lesions provides an adequate indication for treatment, while vascular malformations, which usually persist throughout life, always require therapeutic intervention if they start to cause clinical symptoms. Based on individual parameters (such as diameter, location and growth dynamics), different treatments, including cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgery, and/or embolization, can be performed successfully. Currently, however, none of these treatments represents the solitary treatment of choice.

  4. Vascular calcification: Inducers and inhibitors

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Donghyun, E-mail: dhlee@cau.ac.kr [Department of Biomedical Engineering, Division of Integrative Engineering, Chung-Ang University, 221 Heukseok-Dong, Dongjak-Gu, Seoul 156-756 (Korea, Republic of)

    2011-09-15

    Highlights: {center_dot} Types of vascular calcification processes. {center_dot} Inducers of vascular calcification. {center_dot} Inhibitors of vascular calcifications. {center_dot} Clinical utility for vascular calcification therapy. {center_dot} Implications for the development of new tissue engineering strategies. - Abstract: Unlike the traditional beliefs, there are mounting evidences suggesting that ectopic mineral depositions, including vascular calcification are mostly active processes, many times resembling that of the bone mineralization. Numbers of agents are involved in the differentiation of certain subpopulation of smooth muscle cells (SMCs) into the osteoblast-like entity, and the activation and initiation of extracellular matrix ossification process. On the other hand, there are factors as well, that prevent such differentiation and ectopic calcium phosphate formation. In normal physiological environments, activities of such procalcific and anticalcific regulatory factors are in harmony, prohibiting abnormal calcification from occurring. However, in certain pathophysiological conditions, such as atherosclerosis, chronic kidney disease (CKD), and diabetes, such balances are altered, resulting in abnormal ectopic mineral deposition. Understanding the factors that regulate the formation and inhibition of ectopic mineral formation would be beneficial in the development of tissue engineering strategies for prevention and/or treatment of such soft-tissue calcification. Current review focuses on the factors that seem to be clinically relevant and/or could be useful in developing future tissue regeneration strategies. Clinical utilities and implications of such factors are also discussed.

  5. Trauma vascular, visión del cirujano vascular

    OpenAIRE

    Dr. D. Cristián Salas

    2011-01-01

    El 3% de todas las lesiones en trauma tiene un componente vascular. Con los conflictos armados del siglo pasado se lograron grandes avances en este campo. A partir de la Guerra de Vietnam gracias a las mejoras en el manejo prehospitalario, traslado de pacientes, y avances en técnica quirúrgica se lograron tasas de sobrevida y de amputaciones que se han mantenido estables hasta la fecha. El diagnóstico de lesiones vasculares en extremidades se realiza con el examen físico, sin embargo las lesi...

  6. The role of nitric oxide in portal hypertensive systemic and portal vascular pathology.

    Science.gov (United States)

    Hartleb, M; Michielsen, P P; Dziurkowska-Marek, A

    1997-01-01

    Hypotension, low systemic vascular resistance and reduced sensitivity to vasoconstrictor are features of hyperdynamic syndrome in portal hypertension (PH) and are pathogenetic factors triggering most serious clinical complications of liver cirrhosis. Nitric oxide (NO) is a powerful vasodilating agent, released from vascular endothelium cell and effecting relaxation of vascular smooth muscle. An increased release of NO has been proposed to play a role in the pathogenesis of vasodilation and vascular hypocontractility associated with PH. In agreement with this hypothesis, the whole-body production of NO has been found to be increased in PH, and the measurement of NOS mRNA expression in different organs suggest that the splanchnic vascular system is a major source of NO release. Consequently, NO could play a role in the development of the splanchnic hyperaemia, collateral circulation and portal hypertensive gastropathy. Furthermore, increased generation of NO in central circulation likely accounts for pulmonary vasorelaxation and cardiac dysfunction found in cirrhosis. By contrast, PH-associated endothelial dysfunction seems to invalidate the capability of intrahepatic and intrarenal vasculature to produce NO. A deficient NO release in these vascular territories might contribute to enhancement of PH and development of the hepatorenal syndrome. Overall NO hyperproduction is either the cause (induction of iNOS) or the consequence (stimulation of ecNOS) of the hyperdynamic syndrome. This incertitude results from the yet undefined significance of mild and transitory activation of the endotoxin-cytokines axis for iNOS induction and contradictory data on specific iNOS and ecNOS activities. A contribution of each isoform of NOS to pathogenesis of the hyperdynamic syndrome probably depends on the model of PH in animal studies and the aetiology or severity of cirrhosis in human studies.

  7. MEDNIK syndrome: a novel defect of copper metabolism treatable by zinc acetate therapy.

    Science.gov (United States)

    Martinelli, Diego; Travaglini, Lorena; Drouin, Christian A; Ceballos-Picot, Irene; Rizza, Teresa; Bertini, Enrico; Carrozzo, Rosalba; Petrini, Stefania; de Lonlay, Pascale; El Hachem, Maya; Hubert, Laurence; Montpetit, Alexandre; Torre, Giuliano; Dionisi-Vici, Carlo

    2013-03-01

    MEDNIK syndrome-acronym for mental retardation, enteropathy, deafness, neuropathy, ichthyosis, keratodermia-is caused by AP1S1 gene mutations, encoding σ1A, the small subunit of the adaptor protein 1 complex, which plays a crucial role in clathrin coat assembly and mediates trafficking between trans-Golgi network, endosomes and the plasma membrane. MEDNIK syndrome was first reported in a few French-Canadian families sharing common ancestors, presenting a complex neurocutaneous phenotype, but its pathogenesis is not completely understood. A Sephardic-Jewish patient, carrying a new AP1S1 homozygous mutation, showed severe perturbations of copper metabolism with hypocupremia, hypoceruloplasminemia and liver copper accumulation, along with intrahepatic cholestasis. Zinc acetate treatment strikingly improved clinical conditions, as well as liver copper and bile-acid overload. We evaluated copper-related metabolites and liver function retrospectively in the original French-Canadian patient series. Intracellular copper metabolism and subcellular localization and function of copper pump ATP7A were investigated in patient fibroblasts. Copper metabolism perturbation and hepatopathy were confirmed in all patients. Studies in mutant fibroblasts showed abnormal copper incorporation and retention, reduced expression of copper-dependent enzymes cytochrome-c-oxidase and Cu/Zn superoxide dismutase, and aberrant intracellular trafficking of Menkes protein ATP7A, which normalized after rescue experiments expressing wild-type AP1S1 gene. We solved the pathogenetic mechanism of MEDNIK syndrome, demonstrating that AP1S1 regulates intracellular copper machinery mediated by copper-pump proteins. This multisystem disease is characterized by a unique picture, combining clinical and biochemical signs of both Menkes and Wilson's diseases, in which liver copper overload is treatable by zinc acetate therapy, and can now be listed as a copper metabolism defect in humans. Our results may also

  8. Vascular trauma in Penang and Kuala Lumpur Hospitals.

    Science.gov (United States)

    Lakhwani, M N; Gooi, B H; Barras, C D J

    2002-12-01

    either primary or autogenous reverse LSV repair complicated by sepsis or critical ischaemia. Vascular trauma, especially in conjunction with severe soft tissue, nerve or orthopaedic injury carries colossal physical, psychological, financial and social costs. Associated nerve and venous injury portended poor outcome in this study. Whilst orthopaedic trauma was a common association, the concurrence of occult vascular trauma and soft tissue injury without fracture emphasises the crucial importance of thorough and rapid clinical vascular assessment, investigation and surgical intervention. Fasciotomy, especially for the lower limb, is important for the prevention of compartment syndrome and its, limb-threatening sequelae. Primary preventive road safety promotion and interventions, with attention to high-risk groups (young males and motorcyclists), is urgently required.

  9. Isolated medial foot compartment syndrome after ankle sprain.

    Science.gov (United States)

    Cortina, Josep; Amat, Carles; Selga, Jordi; Corona, Pablo Salvador

    2014-03-01

    Foot compartment syndrome is a serious potential complication of foot crush injury, fractures, surgery, and vascular injury. An acute compartment syndrome isolated to the medial compartment of the foot after suffering an ankle sprain is a rare complication. We report the case of a 31-year-old man who developed a medial foot compartment syndrome after suffering a deltoid ligament rupture at ankle while playing football. The patient underwent a medial compartment fasciotomy with resolution of symptoms. Compartment syndromes of the foot are rare and have been reported to occur after severe trauma. But, there are some reports in the literature of acute exertional compartment syndrome. In our case, the compartment syndrome appeared after an ankle sprain without vascular injuries associated. Copyright © 2013 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.

  10. Metabolic Syndrome 2016: a forgotten or existing entity?

    Directory of Open Access Journals (Sweden)

    Andreas Melidonis

    2017-01-01

    Full Text Available Metabolic syndrome is a premorbid situation, a constellation of metabolic risk factors and defines the need for clinical treatment of obese who have multiple additional risk factors. Nowadays there is a tendency of evolution of metabolic syndrome in a syndrome of renal, cardiac and vascular indices which contribute to atherosclerosis and exacerbate vascular endothelial dysfunction. Metabolic syndrome cannot be considered as a predictor in cardiovascular risk assessment models with a short study period (e.g., 10-year risk. The presence of metabolic syndrome is closely linked with an increased risk of diabetes and cardiovascular disease development. It is also associated with the urgent need to assess the overall cardiovascular risk and the presence of subclinical atherosclerosis by noninvasive imaging. Metabolic syndrome can bring together the fields of cardiovascular disease and diabetes in an effort to limit the risks of both aforementioned situations.

  11. DOWN SYNDROME WITH MOYAMOYA SYNDROME

    National Research Council Canada - National Science Library

    Mohan Makwana; R. K. Vishnoi; Jai Prakash Soni; Kapil Jetha; Suresh Kumar Verma; Pradeep Singh Rathore; Monika Choudhary

    2017-01-01

    ...,” in which the arterial changes are seen among patients with various syndromes or other disease processes- Down syndrome, sickle cell anaemia, neurofibromatosis type-1, congenital heart disease...

  12. Vascular anomalies of the cerebellopontine angle; Vaskulaere Erkrankungen des Kleinhirnbrueckenwinkels

    Energy Technology Data Exchange (ETDEWEB)

    Papanagiotou, P.; Grunwald, I.Q.; Politi, M.; Struffert, T.; Ahlhelm, F.; Reith, W. [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

    2006-03-15

    Vascular anomalies of the cerebellopontine angle are rare compared to tumors in this area. Irritation of the trigeminal, facial, or vestibulocochlear nerve may cause trigeminal neuralgia, hemifacial spasm and vertigo, or tinnitus accordingly. Vessel loops in the cerebellopontine cisterns may cause compression at the root entry or exit zone of the cranial nerves V, VII, and VIII, a phenomenon which is called ''vascular loop syndrome.'' Megadolichobasilar artery and aneurysms of the vertebrobasilar system can also lead to dislocation and compression of the cranial nerves and brain stem. Three-dimensional CISS MR imaging and MR angiography are useful in the detection of neurovascular compression. Microvascular decompression is an effective surgical procedure in the management of compression syndromes of the cranial nerves V, VII, and VIII. (orig.) [German] Gegenueber den Raumforderungen stellen vaskulaere Veraenderungen des Kleinhirnbrueckenwinkels eher eine Ausnahme dar. Trigeminusneuralgie, hemifazialer Spasmus und Schwindel oder Tinnitus koennen bei Irritationen des Nervus trigeminus, facialis und vestibulocochlearis auftreten. Schlingenbildungen der Gefaesse in den Kleinhirnzisternen koennen eine Kompression der Eintritts- oder Austrittszonen der Hirnnerven V, VII und VIII am Hirnstamm hervorrufen, was als ''Vascular-loop-Syndrom'' bezeichnet wird. Zu Verlagerungen und Kompressionen von Hirnnerven und Hirnstamm koennen, wenn auch seltener, die Megadolichobasilaris und Aneurysmen des vertebrobasilaeren Systems fuehren. Bezueglich der Bildgebung ist die Magnetresonanztomographie (MRT) die Methode der Wahl. Die 3D-CISS-Sequenz und die MR-Angiographie sind hilfreich zur Darstellung der neurovaskulaeren Kompression. Die Methode der mikrovaskulaeren Dekompression ist eine wirkungsvolle Methode zur Beseitigung gefaessbedingter Kompressionen der Hirnnerven V, VII und VIII. (orig.)

  13. Heritability of Retinal Vascular Fractals

    DEFF Research Database (Denmark)

    Vergmann, Anna Stage; Broe, Rebecca; Kessel, Line

    2017-01-01

    , the branching pattern of the retinal vessels demonstrated a higher structural similarity in monozygotic than in dizygotic twin pairs. The retinal vascular fractal dimension was mainly determined by genetic factors, which accounted for 54% of the variation. The genetically predetermination of the retinal......Purpose: To determine the genetic contribution to the pattern of retinal vascular branching expressed by its fractal dimension. Methods: This was a cross-sectional study of 50 monozygotic and 49 dizygotic, same-sex twin pairs aged 20 to 46 years. In 50°, disc-centered fundus photographs......, the retinal vascular fractal dimension was measured using the box-counting method and compared within monozygotic and dizygotic twin pairs using Pearson correlation coefficients. Falconer's formula and quantitative genetic models were used to determine the genetic component of variation. Results: The mean...

  14. [Alzheimer's disease and vascular dementia].

    Science.gov (United States)

    Nagata, Ken

    2014-04-01

    Alzheimer's disease (AD) and vascular dementia (VaD) are the two major forms of dementia in the elderly, and they had been separated categorically on the basis of pathogenetic mechanisms and clinical operationalized criteria. However, it was claimed that this strict separation might steered toward the overdiagnosis of vascular dementia, this dichotomy has been reevaluated in the light of recent epidemiological and neuropathological knowledge. Cerebrovascular disease (CVD) is now considered as one of the vascular risk factors to the onset and evolution of Alzheimer's disease. Futhermore, the term "AD with CVD" has been used to classify patients fulfilling the clinical criteria for possible AD and who also present clinical or brain imaging evidence of relevant CVD.

  15. Chemerin reduces vascular nitric oxide/cGMP signalling in rat aorta: a link to vascular dysfunction in obesity?

    Science.gov (United States)

    Neves, Karla Bianca; Lobato, Núbia S; Lopes, Rhéure Alves Moreira; Filgueira, Fernando P; Zanotto, Camila Ziliotto; Oliveira, Ana Maria; Tostes, Rita C

    2014-07-01

    The adipokine chemerin has been implicated in cardiovascular complications associated with obesity and the metabolic syndrome. Chemerin has direct effects on the vasculature, augmenting vascular responses to contractile stimuli. As NO/cGMP signalling plays a role in vascular dysfunction associated with obesity and the metabolic syndrome, we hypothesized that chemerin induces vascular dysfunction by decreasing NO/cGMP signalling. Aortic rings from male Wistar rats (10-12 weeks of age) were incubated with chemerin (0.5 or 5 ng/ml for 1 h) or vehicle and isometric tension was recorded. Vasorelaxation in response to ACh (acetylcholine), SNP (sodium nitroprusside) and BAY 412272 [an sGC (soluble guanylate cyclase) stimulator] were decreased in chemerin-treated vessels. The NOS (NO synthase) cofactor BH4 (tetrahydrobiopterin), an O2- (superoxide anion) scavenger (tiron) and a SOD (superoxide dismutase) mimetic (tempol) abolished the effects of chemerin on ACh-induced vasodilation. eNOS (endothelial NOS) phosphorylation, determined by Western blotting, was increased in chemerin-treated vessels; however, the enzyme was mainly in the monomeric form, with decreased eNOS dimer/monomer ratio. Chemerin decreased the mRNA levels of the rate-limiting enzyme for BH4 biosynthesis GTP cyclohydrolase I. Chemerin-incubated vessels displayed decreased NO production, along with increased ROS (reactive oxygen species) generation. These effects were abrogated by BH4, tempol and L-NAME (NG-nitro-L-arginine methyl ester). sGC protein expression and cGMP levels were decreased in chemerin-incubated vessels. These results demonstrate that chemerin reduces NO production, enhances NO breakdown and also decreases NO-dependent cGMP signalling, thereby reducing vascular relaxation. Potential mechanisms mediating the effects of chemerin in the vasculature include eNOS uncoupling, increased O2- generation and reduced GC activity.

  16. Tissue-Engineered Vascular Rings from Human iPSC-Derived Smooth Muscle Cells

    Directory of Open Access Journals (Sweden)

    Biraja C. Dash

    2016-07-01

    Full Text Available There is an urgent need for an efficient approach to obtain a large-scale and renewable source of functional human vascular smooth muscle cells (VSMCs to establish robust, patient-specific tissue model systems for studying the pathogenesis of vascular disease, and for developing novel therapeutic interventions. Here, we have derived a large quantity of highly enriched functional VSMCs from human induced pluripotent stem cells (hiPSC-VSMCs. Furthermore, we have engineered 3D tissue rings from hiPSC-VSMCs using a facile one-step cellular self-assembly approach. The tissue rings are mechanically robust and can be used for vascular tissue engineering and disease modeling of supravalvular aortic stenosis syndrome. Our method may serve as a model system, extendable to study other vascular proliferative diseases for drug screening. Thus, this report describes an exciting platform technology with broad utility for manufacturing cell-based tissues and materials for various biomedical applications.

  17. Vascular education and training in Asia.

    Science.gov (United States)

    Jirasiritham, Sopon

    2008-12-01

    Cardiovascular diseases have become more prevalent and threatening to the health of the population of Asia due to the rapidly growing number of aging people. The Asian Society for Vascular Surgery unites 13 member organizations: Japan, Korea, China, India, Hong Kong, Singapore, Malaysia, Taiwan, Bangladesh, Philippines, Saudi Arabia, Indonesia, and Thailand. The essential mission of the Asian Society for Vascular Surgery is to improve training in vascular surgery to increase the number of competent vascular surgeons in Asia. Almost every member country has its own vascular training program. Most curricula for vascular surgery training are composed of basic vascular research, clinical vascular medicine, vascular investigation, and open and endovascular surgery, with the period of training ranging from 2 to 4 years.

  18. Vascular Gene Expression: A Hypothesis

    Directory of Open Access Journals (Sweden)

    Angélica Concepción eMartínez-Navarro

    2013-07-01

    Full Text Available The phloem is the conduit through which photoassimilates are distributed from autotrophic to heterotrophic tissues and is involved in the distribution of signaling molecules that coordinate plant growth and responses to the environment. Phloem function depends on the coordinate expression of a large array of genes. We have previously identified conserved motifs in upstream regions of the Arabidopsis genes, encoding the homologs of pumpkin phloem sap mRNAs, displaying expression in vascular tissues. This tissue-specific expression in Arabidopsis is predicted by the overrepresentation of GA/CT-rich motifs in gene promoters. In this work we have searched for common motifs in upstream regions of the homologous genes from plants considered to possess a primitive vascular tissue (a lycophyte, as well as from others that lack a true vascular tissue (a bryophyte, and finally from chlorophytes. Both lycophyte and bryophyte display motifs similar to those found in Arabidopsis with a significantly low E-value, while the chlorophytes showed either a different conserved motif or no conserved motif at all. These results suggest that these same genes are expressed coordinately in non- vascular plants; this coordinate expression may have been one of the prerequisites for the development of conducting tissues in plants. We have also analyzed the phylogeny of conserved proteins that may be involved in phloem function and development. The presence of CmPP16, APL, FT and YDA in chlorophytes suggests the recruitment of ancient regulatory networks for the development of the vascular tissue during evolution while OPS is a novel protein specific to vascular plants.

  19. Angioembolisation in vaginal vascular malformation.

    Directory of Open Access Journals (Sweden)

    Srivastava D

    2001-01-01

    Full Text Available Vaginal arteriovenous malformations are rare entities and their most common presentation is vaginal haemorrhage. This case report describes a 22-year-old woman who presented at 20 weeks of gestation with slow growing soft and tender swelling at anterior vaginal wall. Diagnosis was confirmed as vaginal vascular malformation on contrast enhanced magnetic resonance imaging. The mass did not subside after delivery and patient developed dyspareunia. It was successfully treated by angioembolisation using polyvinyl alcohol particles. Angioembolisation being safe and effective should be the treatment of first choice for symptomatic vaginal vascular malformation.

  20. Hypertonic saline reduces vascular leakage in a mouse model of severe dengue.

    Science.gov (United States)

    Tan, Grace Kai Xin; Ng, Jowin Kai Wei; Tan, Kar Wai; Angeli, Veronique; Moochhala, Shabbir; Ooi, Eng Eong; Alonso, Sylvie

    2013-01-01

    Dengue (DEN) is a mosquito-borne viral disease and represents a serious public health threat and an economical burden throughout the tropics. Dengue clinical manifestations range from mild acute febrile illness to severe DEN hemorrhagic fever/DEN shock syndrome (DHF/DSS). Currently, resuscitation with large volumes of isotonic fluid remains the gold standard of care for DEN patients who develop vascular leakage and shock. Here, we investigated the ability of small volume of hypertonic saline (HTS) suspensions to control vascular permeability in a mouse model of severe DEN associated with vascular leakage. Several HTS treatment regimens were considered and our results indicated that a single bolus of 7.5% NaCl at 4 mL per kg of body weight administered at the onset of detectable vascular leakage rapidly and significantly reduced vascular leak for several days after injection. This transient reduction of vascular leakage correlated with reduced intestine and liver damage with restoration of the hepatic functions, and resulted in delayed death of the infected animals. Mechanistically, we showed that HTS did not directly impact on the viral titers but resulted in lower immune cells counts and decreased systemic levels of soluble mediators involved in vascular permeability. In addition, we demonstrated that neutrophils do not play a critical role in DEN-associated vascular leakage and that the therapeutic effect of HTS is not mediated by its impact on the neutrophil counts. Together our data indicate that HTS treatment can transiently but rapidly reduce dengue-associated vascular leakage, and support the findings of a recent clinical trial which evaluated the efficacy of a hypertonic suspension to impact on vascular permeability in DSS children.

  1. Kindler syndrome

    Directory of Open Access Journals (Sweden)

    Kaviarasan P

    2005-01-01

    Full Text Available Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma. The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome, Cockayne syndrome, dyskeratosis congenita, epidermolysis bullosa, Rothmund-Thomson syndrome and xeroderma pigmentosum. Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication.

  2. Extracellular vesicles as mediators of vascular inflammation in kidney disease.

    Science.gov (United States)

    Helmke, Alexandra; von Vietinghoff, Sibylle

    2016-03-06

    Vascular inflammation is a common cause of renal impairment and a major cause of morbidity and mortality of patients with kidney disease. Current studies consistently show an increase of extracellular vesicles (EVs) in acute vasculitis and in patients with atherosclerosis. Recent research has elucidated mechanisms that mediate vascular wall leukocyte accumulation and differentiation. This review addresses the role of EVs in this process. Part one of this review addresses functional roles of EVs in renal vasculitis. Most published data address anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis and indicate that the number of EVs, mostly of platelet origin, is increased in active disease. EVs generated from neutrophils by activation by ANCA can contribute to vessel damage. While EVs are also elevated in other types of autoimmune vasculitis with renal involvement such as systemic lupus erythematodes, functional consequences beyond intravascular thrombosis remain to be established. In typical hemolytic uremic syndrome secondary to infection with shiga toxin producing Escherichia coli, EV numbers are elevated and contribute to toxin distribution into the vascular wall. Part two addresses mechanisms how EVs modulate vascular inflammation in atherosclerosis, a process that is aggravated in uremia. Elevated numbers of circulating endothelial EVs were associated with atherosclerotic complications in a number of studies in patients with and without kidney disease. Uremic endothelial EVs are defective in induction of vascular relaxation. Neutrophil adhesion and transmigration and intravascular thrombus formation are critically modulated by EVs, a process that is amenable to therapeutic interventions. EVs can enhance monocyte adhesion to the endothelium and modulate macrophage differentiation and cytokine production with major influence on the local inflammatory milieu in the plaque. They significantly influence lipid phagocytosis and antigen presentation by

  3. Carotid Stump Syndrome

    Directory of Open Access Journals (Sweden)

    Lara Toufic Dakhoul MD

    2014-08-01

    Full Text Available Objectives. To highlight the case of a patient with multiple transient ischemic attacks and visual disturbances diagnosed with carotid stump syndrome and managed with endovascular approach. Case Presentation. We present the case of a carotid stump syndrome in an elderly patient found to have moderate left internal carotid artery stenosis in response to an advertisement for carotid screening. After a medical therapeutic approach and a close follow-up, transient ischemic attacks recurred. Computed tomographic angiography showed an occlusion of the left internal carotid artery and the presence of moderate stenosis in the right internal carotid artery, which was treated by endovascular stenting and balloon insertion. One month later, the patient presented with visual disturbances due to the left carotid stump and severe stenosis of the left external carotid artery that was reapproached by endovascular stenting. Conclusion. Considerations should be given to the carotid stump syndrome as a source of emboli for ischemic strokes, and vascular assessment could be used to detect and treat this syndrome.

  4. The social media: its impact on a vascular surgery practice.

    Science.gov (United States)

    Turnipseed, William D

    2013-04-01

    Social media has revolutionized interpersonal communication and has become a commonly used public informational resource. This study evaluates the impact of intranet informatics on a specialty practice of vascular surgery. Referral patterns for patients with chronic compartment syndrome (CCS) and popliteal entrapment syndrome (PAES) between 2008 and 2011 were analyzed. Demographics included referral source (physicians, nonphysicians), media resource, and case volume change. Prior to 2008, referrals came from local or regional sports medicine practices (100%). Since 2008 this pattern has changed; local/regional (80%), national (15%), and international (5%). Physician referrals dropped from 97% to 70%, and nonphysician referrals increased from 3% to 30%. Both CCS procedures and PAES procedures increased as remote geographic and public referrals increased. Referral change was associated with social media searches using applications such as PubMed and Google. Social media is an evolving source of medical information and patient referrals which physicians should cautiously embrace.

  5. Moebius' syndrome with unilateral cerebellar hypoplasia.

    Science.gov (United States)

    Harbord, M G; Finn, J P; Hall-Craggs, M A; Brett, E M; Baraitser, M

    1989-01-01

    A case is reported of a child with Moebius' syndrome who also has unilateral cerebellar hypoplasia. We suggest that this combination of abnormalities could result from a vascular disruption occurring in the basilar artery early in its development. Images PMID:2810343

  6. POLAND SYNDROME WITH DEXTROCARDIA: CASE REPORT

    African Journals Online (AJOL)

    2010-11-11

    Nov 11, 2010 ... involvement of the internal mammary artery (which originates at the origin of the subclavian artery) in addition to the Moebius syndrome phenotype whereby ... dextroposition resulting from the thoracic pathology caused by the presumptive vascular event. This perspective is supported by the observation that.

  7. Vascular ultrasound for atherosclerosis imaging

    NARCIS (Netherlands)

    C.L. de Korte (Chris); H.H.G. Hansen (Hendrik); A.F.W. van der Steen (Ton)

    2011-01-01

    textabstractCardiovascular disease is a leading cause of death in the Western world. Therefore, detection and quantification of atherosclerotic disease is of paramount importance to monitor treatment and possible prevention of acute events. Vascular ultrasound is an excellent technique to assess the

  8. Signaling circuitry in vascular morphogenesis.

    Science.gov (United States)

    Warren, Carmen M; Iruela-Arispe, M Luisa

    2010-05-01

    In this mini-review, we have highlighted the recent breakthroughs in growth factor signaling that have made conceptual changes in our understanding of how blood vessels are formed. Studies conducted over the past few years have focused on understanding the cell biology of vascular morphogenesis. The major themes include characterization of the different cell types that comprise a vascular sprout, as well as the regulatory influence of cell-cell and cell-matrix interactions on signaling outcomes. In addition, novel trends have emerged, including nonconventional ways in which vascular endothelial growth factor contributes to cell survival and metabolic balance. The growth of new capillary sprouts from a preexisting vascular network requires a highly coordinated cellular response to both growth factors and morphogens. This response is sensed and triggered by cell surface receptors responsible for the activation of an intracellular cascade that efficiently initiates migration and proliferation programs. While the molecular players that coordinate these effects have been identified, recent findings have expanded our understanding of how context, in particular cell-cell and cell-matrix interactions, affects endothelial cell responses to growth factors.

  9. Vascular complications in orthopedic surgery.

    Science.gov (United States)

    Chervu, A; Quinones-Baldrich, W J

    1988-10-01

    Vascular complications may be seen secondary to trauma or in the perioperative period following elective surgery. Prompt recognition and correction of these problems are of utmost importance to assure functional viability of the affected extremity. Evaluation may be complicated by the presence of preexisting atherosclerotic occlusive disease in the elderly patient. Relevant points in the history and physical examination include mechanism of injury, preexisting disease, evaluation of motor and sensory function, and presence and character of pulses. Noninvasive vascular studies should be obtained in all patients. Absolute indications for angiography include absent pulses, signs and symptoms of ischemia, a bruit, and a posterior knee dislocation; decreased pulses, a significant hematoma, and proximity of the fracture fragment are relative indications. Controversial issues in the management of combined orthopedic and vascular injuries include the use of internal versus external fixation, the use of prosthetic versus autogenous material, and the need for venous reconstruction. Popliteal artery trauma is still associated with a high limb loss rate, and careful evaluation of knee injuries is necessary. Vascular compromise may also complicate joint replacement surgery. These complications are preventable, and management is greatly simplified by a detailed preoperative evaluation.

  10. Silk Biomaterials with Vascularization Capacity.

    Science.gov (United States)

    Han, Hongyan; Ning, Hongyan; Liu, Shanshan; Lu, Qiang; Fan, Zhihai; Lu, Haijun; Lu, Guozhong; Kaplan, David L

    2016-01-20

    Functional vascularization is critical for the clinical regeneration of complex tissues such as kidney, liver or bone. The immobilization or delivery of growth factors has been explored to improve vascularization capacity of tissue engineered constructs, however, the use of growth factors has inherent problems such as the loss of signaling capability and the risk of complications such as immunological responses and cancer. Here, a new method of preparing water-insoluble silk protein scaffolds with vascularization capacity using an all aqueous process is reported. Acid was added temporally to tune the self-assembly of silk in lyophilization process, resulting in water insoluble scaffold formation directly. These biomaterials are mainly noncrystalline, offering improved cell proliferation than previously reported silk materials. These systems also have appropriate softer mechanical property that could provide physical cues to promote cell differentiation into endothelial cells, and enhance neovascularization and tissue ingrowth in vivo without the addition of growth factors. Therefore, silk-based degradable scaffolds represent an exciting biomaterial option, with vascularization capacity for soft tissue engineering and regenerative medicine.

  11. Markers of Early Vascular Ageing.

    Science.gov (United States)

    Kotsis, Vasilios; Antza, Christina; Doundoulakis, Ioannis; Stabouli, Stella

    2017-01-01

    Cardiovascular damage is clinically manifested as coronary artery disease, heart failure, stroke and peripheral artery disease. The prevalence of these adverse conditions is higher with advancing age. Although many patients present cardiovascular damage late in their life, it is common to see patients with early atherosclerosis in cardiovascular intensive care units at ages lower than 50 years in men and 55 for women. In this review of the literature we identified risk factors of early vascular damage. The classic risk factors such as age, gender, diabetes mellitus, dyslipidemia, smoking, alcohol, hypertension, obesity, family history and newer biomarkers such as hs-CRP, folic acid, homocysteine, fibrinogen are neither strong nor predictive of the individual patient's risk to present early cardiovascular disease. All these risk factors have been used to propose risk scores for possible future events but we still lack a single strong marker indicating new onset of disease that will predict the future independently of the classical factors. The role of vascular imaging techniques to identify patients with subclinical atherosclerotic vascular damage before clinical disease, including the effect of known and unknown risk factors on the vascular tree, seems to be very important for intensifying preventive measures in high risk patients. Early arteriosclerosis measured from pulse wave velocity is associated with reduced arterial elasticity and is associated with future cardiovascular events. Vascular measurements may better represent the continuum of cardiovascular disease from a young healthy to an aged diseased vessel that is going to produce adverse clinical events. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  12. Subclinical hypothyroidism after vascular complicated pregnancy

    NARCIS (Netherlands)

    Zanden, M. van der; Hop-de Groot, R.J.; Sweep, F.C.; Ross, H.A.; Heijer, M. den; Spaanderman, M.E.A.

    2013-01-01

    OBJECTIVE: Women with a history of vascular complicated pregnancy are at risk for developing remote cardiovascular disease. It is associated with underlying cardiovascular risk factors both jeopardizing trophoblast and vascular function. Subclinical hypothyroidism may relate to both conditions.

  13. ESRD QIP - Vascular Access - Payment Year 2018

    Data.gov (United States)

    U.S. Department of Health & Human Services — This dataset includes facility details, performance rates, vascular access topic measure score, and the state and national average measure scores for the vascular...

  14. NEURO-VASCULAR INJURIES ASSOCIATED WITH LIMB ...

    African Journals Online (AJOL)

    hi-tech

    2000-12-01

    Dec 1, 2000 ... Results: Road traffic accidents were the main cause of fractures associated with neuro- vascular ... the patients with vascular or nerve injury associated with fractures .... of traumatic aorto-iliac dissection injury in a child with.

  15. Self-management of vascular risk factors

    OpenAIRE

    Sol-de Rijk, B.G.M.

    2009-01-01

    Summary The aim of this thesis was to provide insight into the potential of a self-management approach in treatment of vascular risk factors and to develop a self-management intervention. Furthermore to examine if this intervention, based on self-efficacy promoting theory, is effective in reducing vascular risk factors in patients with established vascular diseases. Patients with different manifestations of vascular diseases appeared to have high levels of self-efficacy concerning the self-ma...

  16. Vascular smooth muscle function: defining the diabetic vascular phenotype.

    Science.gov (United States)

    Bruno, Rosa Maria; Ghiadoni, Lorenzo

    2013-10-01

    In this issue of Diabetologia, a meta-analysis performed by Montero and co-authors (Diabetologia doi 10.1007/s00125-013-2974-1 ) demonstrates a significant impairment of vascular smooth muscle (VSM) function in type 2 diabetic patients. Endothelial function and VSM function between type 2 diabetic and healthy individuals were associated, especially in the microcirculation, confirming the hypothesis that unresponsiveness of VSM cells to NO may amplify the consequences of reduced NO availability. This study suggests a novel interpretation for endothelial dysfunction in diabetic patients, indicating VSM cells as key players. Causative mechanisms of VSM dysfunction, which seems to be a feature of the vascular phenotype of type 2 diabetes mellitus, are largely unexplored in humans. Future studies should also address the crucial issue of the prognostic significance of VSM dysfunction in diabetic patients, and possibly in other conditions characterised by high cardiovascular risk.

  17. Ocular findings in Jacobsen syndrome.

    Science.gov (United States)

    Lee, William B; O'Halloran, Henry S; Grossfeld, Paul D; Scher, Colin; Jockin, Yvette M; Jones, Christopher

    2004-04-01

    To discuss the ophthalmic findings and their clinical significance in 10 new cases of Jacobsen syndrome (mental retardation, craniofacial anomalies, congenital heart defects, and blood dyscrasias) and to review the ophthalmic findings in all previously reported cases in the literature. Ten new cases of Jacobsen syndrome were collected and studied prospectively for detection of abnormal ophthalmologic examination findings. A total of 63 previously reported cases were identified from Medline and analyzed for ophthalmologic abnormalities. The most common ophthalmologic findings in the new cases of Jacobsen syndrome included strabismus (90.0%), refractive error (90.0%), and ptosis (70.0%). Facial dysmorphism was also common and included hypertelorism, epicanthal folds, and down-slanting palpebral fissures. Uncommon ophthalmic findings included 5 patients with retinal vascular tortuosity, 1 with glaucoma, and 3 with amblyopia. In 63 cases reviewed, 36 reported ophthalmologic abnormalities. The most common findings included facial anomalies and ptosis. Only 5 of the 63 patients had evidence of strabismus, and none were reported to have retinal vascular tortuosity. To prevent unnecessary vision loss in children with Jacobsen syndrome, proper screening for amblyogenic factors is imperative. We recommend a baseline complete ophthalmologic examination with subsequent follow-up examinations depending on the particular findings noted during the initial screening visit.

  18. Inflammation-regulated mRNA stability and the progression of vascular inflammatory diseases.

    Science.gov (United States)

    Herman, Allison B; Autieri, Michael V

    2017-11-15

    Cardiovascular disease remains a major medical and socioeconomic burden in developed and developing societies, and will increase with an aging and increasingly sedentary society. Vascular disease and atherosclerotic vascular syndromes are essentially inflammatory disorders, and transcriptional and post-transcriptional processes play essential roles in the ability of resident vascular and inflammatory cells to adapt to environmental stimuli. The regulation of mRNA translocation, stability, and translation are key processes of post-transcriptional regulation that permit these cells to rapidly respond to inflammatory stimuli. For the most part, these processes are controlled by elements in the 3'-UTR of labile, proinflammatory transcripts. Since proinflammatory transcripts almost exclusively contain AU-rich elements (AREs), this represents a tightly regulated and specific mechanism for initiation and maintenance of the proinflammatory phenotype. RNA-binding proteins (RBPs) recognize cis elements in 3'-UTR, and regulate each of these processes, but there is little literature exploring the concept that RBPs themselves can be directly regulated by inflammatory stimuli. Conceptually, inflammation-responsive RBPs represent an attractive target of rational therapies to combat vascular inflammatory syndromes. Herein we briefly describe the cellular and molecular etiology of atherosclerosis, and summarize our current understanding of RBPs and their specific roles in regulation of inflammatory mRNA stability. We also detail RBPs as targets of current anti-inflammatory modalities and how this may translate into better treatment for vascular inflammatory diseases. © 2017 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.

  19. KUDESAN EFFICACY IN ADOLESCENTS WITH METABOLIC SYNDROME

    Directory of Open Access Journals (Sweden)

    M.B. Kolesnikova

    2011-01-01

    Full Text Available Metabolic abnormalities in metabolic syndrome affect the functioning of practically all organs and systems, and most seriously — cardio-vascular system. Cardio-vascular abnormalities in metabolic syndrome manifest as arterial hypertension, Riley-Day syndrome and endothelial dysfunction that can lead to decrease of adaptive and reserve capabilities. Co-enzyme Q10 possesses cardioprotective,  stress-protective and anti-ischaemic activity. Clinical study performed on 40 children aged 10 to 17 years with constitutive obesity, complicated metabolic syndrome, has proven validity of co-enzyme Q10 treatment in patients with metabolic syndrome. The use of co-enzyme Q10 15 mg/day during 30 days has lead to improvement of psycho-emotional condition, decrease in anxiety complaints, sleep improvement, decrease in asthenic syndrome symptoms, improvement in electrophysiological heart indices Key words: metabolic syndrome, co-enzyme Q10. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 102–106.

  20. Specificity of haemostasis abnormalities for vascular phenotypes.

    Science.gov (United States)

    Lowe, G D; Haverkate, F

    1998-01-01

    Atherothrombosis is a systemic disease, hence it is difficult to prove the specificity of haemostasis abnormality for any single vascular phenotype. Associations between haemostatic variables and any given phenotype, e.g. (vascular) dementia, should be interpreted with caution, given the overlaps of vascular disease phenotypes, risk factors, and haemostatic variables.

  1. 21 CFR 870.4450 - Vascular clamp.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Vascular clamp. 870.4450 Section 870.4450 Food and... CARDIOVASCULAR DEVICES Cardiovascular Surgical Devices § 870.4450 Vascular clamp. (a) Identification. A vascular clamp is a surgical instrument used to occlude a blood vessel temporarily. (b) Classification. Class II...

  2. Hippocampal atrophy in subcortical vascular dementia

    NARCIS (Netherlands)

    van de Pol, L.A.; Gertz, H.J.; Scheltens, P.; Wolf, H

    2011-01-01

    Background and Purpose: New research criteria for subcortical vascular dementia (SVaD) have been suggested to define a more homogeneous subgroup of vascular dementia. Hippocampal (Hc) atrophy is a hallmark of Alzheimer's disease (AD), but it also occurs in other dementia disorders including vascular

  3. Aberrant Radial Artery Causing Carpal Tunnel Syndrome

    Directory of Open Access Journals (Sweden)

    Zinon T. Kokkalis

    2016-07-01

    Full Text Available Anatomical vascular variations are rare causes of carpal tunnel syndrome. An aberrant medial artery is the most common vascular variation, while an aberrant radial artery causing carpal tunnel syndrome is even more rare, with an incidence ranging less than 3%. This article reports a patient with compression of the median nerve at the carpal tunnel by an aberrant superficial branch of the radial artery. An 80- year- old man presented with a 5-year history of right hand carpal tunnel syndrome; Tinel sign, Phalen test and neurophysiological studies were positive. Open carpal tunnel release showed an aberrant superficial branch of the radial artery with its accompanying veins running from radially to medially, almost parallel to the median nerve, ending at the superficial palmar arterial arch. The median nerve was decompressed without ligating the aberrant artery. At the last follow-up, 2 years after diagnosis and treatment the patient is asymptomatic.

  4. Aberrant Radial Artery Causing Carpal Tunnel Syndrome.

    Science.gov (United States)

    Kokkalis, Zinon T; Tolis, Konstantinos E; Megaloikonomos, Panayiotis D; Panagopoulos, Georgios N; Igoumenou, Vasilios G; Mavrogenis, Andreas F

    2016-06-01

    Anatomical vascular variations are rare causes of carpal tunnel syndrome. An aberrant medial artery is the most common vascular variation, while an aberrant radial artery causing carpal tunnel syndrome is even more rare, with an incidence ranging less than 3%. This article reports a patient with compression of the median nerve at the carpal tunnel by an aberrant superficial branch of the radial artery. An 80- year- old man presented with a 5-year history of right hand carpal tunnel syndrome; Tinel sign, Phalen test and neurophysiological studies were positive. Open carpal tunnel release showed an aberrant superficial branch of the radial artery with its accompanying veins running from radially to medially, almost parallel to the median nerve, ending at the superficial palmar arterial arch. The median nerve was decompressed without ligating the aberrant artery. At the last follow-up, 2 years after diagnosis and treatment the patient is asymptomatic.

  5. Near-infraread spectroscopy during peripheral vascular surgery

    DEFF Research Database (Denmark)

    Schroeder, Torben Veith; Eiberg, Jonas Peter; Vogt, Katja

    1997-01-01

    Original,Near-infraread spectroscopy,Vascular disease,Vascular by-pass surgery,Perioperative oxymetry......Original,Near-infraread spectroscopy,Vascular disease,Vascular by-pass surgery,Perioperative oxymetry...

  6. [Moebius syndrome: therapeutic proposals from 2 cases].

    Science.gov (United States)

    Braye, F; Souchere, B; Franc, C; Freidel, M

    1996-12-01

    Moebius syndrome is a congenital bilateral palsy of the sixth and seventh cranial nerves. It results a total absence of facial expression and a severe strabismus. Social life is greatly disturbed. Other anomalies may be associated, especially other cranial palsies and Poland syndrome. The etiology of this syndrome isn't clearly established. Stem necrosis secondary to a vascular deficiency is often admitted. We report two observations. We emphasize the importance of a complete maxillo-facial treatment including maxillo-mandibular anomaly. Both patient underwent orthognathic surgery. The first one for class II and the second for class III anomaly. One patient underwent a facial reanimation by temporal muscle transfer. Orthognathic surgery must be realized prior to facial reanimation. A correction of the strabismus is possible. Moebius syndrome is a rare (200 observations) but very severe malformation. Maxillofacial surgery is able to improve the morphological and relational aspect of Moebius syndrome.

  7. Acute compartment syndrome caused by uncontrolled hypothyroidism.

    Science.gov (United States)

    Modi, Anar; Amin, Hari; Salzman, Matthew; Morgan, Farah

    2017-06-01

    Acute compartment syndrome is increased tissue pressure exceeding perfusion pressure in a closed compartment resulting in nerve and muscle ischemia. Common precipitating causes are crush injuries, burns, substance abuse, osseous or vascular limb trauma. This is a case of 42year old female with history of hypothyroidism who presented to emergency room with acute onset of severe pain and swelling in right lower extremity. Physical examination was concerning for acute compartment syndrome of right leg which was confirmed by demonstration of elevated compartmental pressures. No precipitating causes were readily identified. Further laboratory testing revealed uncontrolled hypothyroidism. Management included emergent fasciotomy and initiating thyroid hormone replacement. This case represents a rare association between acute compartment syndrome and uncontrolled hypothyroidism. We also discuss the pathogenesis of compartment syndrome in hypothyroid patients and emphasize the importance of evaluating for less common causes, particularly in setting of non-traumatic compartment syndrome. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Twin-to-twin transfusion syndrome : from placental anastomoses to long-term outcome

    NARCIS (Netherlands)

    Lopriore, Enrico

    2006-01-01

    Twin-to-twin transfusion syndrome (TTTS) is a severe complication of monochorionic twin pregnancies associated with high perinatal mortality and morbidity rates. Placental vascular anastomoses, almost invariably present in monochorionic placentas, are the essential anatomical substrate for the

  9. Proteus syndrome: a rare cause of hemihypertrophy and macrodactyly on bone scanning

    NARCIS (Netherlands)

    Joshi, U.; van der Sluijs, J.A.; Teule, G.J.; Pijpers, R.

    2005-01-01

    Proteus syndrome is a rare, sporadic genetic disorder characterized by overgrowth of multiple different tissues in a mosaic pattern. It is associated with connective tissue nevi, epidermal nevi, disproportionate overgrowth of multiple tissues, vascular malformations, characteristic tumors, and

  10. Glomerular involution in children with frequently relapsing minimal change nephrotic syndrome: an unrecognized form of glomerulosclerosis?

    NARCIS (Netherlands)

    Dijkman, H.B.P.M.; Wetzels, J.F.M.; Gemmink, J.H.; Baede, J.; Levtchenko, E.N.; Steenbergen, E.

    2007-01-01

    Global glomerulosclerosis can be divided in the vascular (obsolescent) type and the glomerulopathic (solidified) type. In biopsies from children with recurrent nephrotic syndrome owing to minimal change nephropathy (MCN), we noticed small, globally sclerosed glomeruli that appeared to be distinct

  11. [Menopause: Hypertension and vascular disease].

    Science.gov (United States)

    Zilberman, J M

    2018-01-28

    Hypertension is the main cardiovascular risk factor affecting 25% of women. Hormone changes and hypertension after menopause may lead to higher target organ damage and cardiovascular disease such as increased arterial stiffness, coronary diseases, chronic heart failure and stroke. The physiopathological mechanisms involved in the development of hypertension and cardiovascular diseases in menopausal women are controversial. There are pharmacokinetic and pharmacodynamic differences in both sexes, the women have more coughing when using the converting-enzyme inhibitors, more cramps when using thiazide diuretics and more oedema in the inferior limbs when using calcium antagonists. The aim of this review is to analyse possible physiopathological mechanisms involved in hypertension after menopause and to gain a better understanding of the biological effects mediated by vascular ageing in women when the level of oestrogen protective effect decreases over the vascular system. Copyright © 2017 SEH-LELHA. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Diagnostic imaging of vascular leiomyosarcomas; Bildgebende Diagnostik von vaskulaeren Leiomyosarkomen

    Energy Technology Data Exchange (ETDEWEB)

    Kreft, B.; Flacke, S.; Textor, J.; Schild, H.H. [Bonn Univ., Radiologische Klinik (Germany); Zhou, H. [Bonn Univ., Pathologisches Inst. (Germany); Remig, J. [Chirurgische Klinik, Univ. Bonn (Germany)

    2004-02-01

    Primary vascular leiomyosarcomas are very rare tumors, with the venous variety most often arising from the inferior caval vein and the arterial variety from the pulmonal artery. The tumors show either an exclusive intra- or extravascular pattern or a mixed growth pattern. The clinical symptoms depend on tumor location, with intraluminal tumors of the inferior caval vein causing edema or a Budd-Chiari syndrome. Leiomyosarcomas of the pulmonal artery can mimic chronic central or recurrent peripheral pulmonary embolism. Contrast enhanced spiral CT with multiplanar reconstruction is the diagnostic method of choice when a vascular leiomyosarcoma is suspected. MRI with MR-angiography can be added. If a tumor of undetermined origin shows a broad contact with a vessel and/or an intraluminal component, possible primary vascular leiomyosarcoma should be included in the differential diagnosis. (orig.) [German] Primaere vaskulaere Leiomyosarkome sind sehr seltene Tumoren, die venoes meistens von der Vena cava inferior und arteriell von der Pulmonalarterie ausgehen. Die Tumoren zeigen entweder ein komplett extra- bzw. intravaskulaeres oder ein gemischtes Wachstum. Die klinischen Symptome der venoesen Tumoren richten sich nach der Lokalisation der Tumoren, wobei intraluminale Tumoren im Bereich der Vena cava inferior durch Oedeme oder ein Budd-Chiari-Syndrom auffallen koennen. Leiomyosarkome der Pulmonalarterien koennen chronische zentrale oder rezidivierende periphere Lungenembolie imitieren. Die kontrastverstaerkte Spiral-CT mit multiplanaren Rekonstruktionen ist die diagnostische Methode der Wahl bei V.a. ein vaskulaeres Leiomyosarkom, die durch die MRT in Kombination mit der MR-Angiographie ergaenzt werden kann. Differenzialdiagnostisch sollte bei unklarer Organzugehoerigkeit von Tumoren mit Kontakt zu Venen und/oder intraluminalem Anteil an die Moeglichkeit eines primaeren vaskulaeren Leiomyosarkoms gedacht werden. (orig.)

  13. Ehlers-Danlos syndrome type IV

    Directory of Open Access Journals (Sweden)

    Germain Dominique P

    2007-07-01

    Full Text Available Abstract Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS, is an inherited connective tissue disorder defined by characteristic facial features (acrogeria in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular

  14. Ehlers-Danlos syndrome type IV

    Science.gov (United States)

    Germain, Dominique P

    2007-01-01

    Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae) are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular complication in a patient suffering

  15. Angiogenesis, Cancer, and Vascular Aging

    OpenAIRE

    Junji Moriya; Tohru Minamino

    2017-01-01

    Several lines of evidence have revealed that the angiogenic response to ischemic injury declines with age, which might account for the increased morbidity and mortality of cardiovascular disease (CVD) among the elderly. While impairment of angiogenesis with aging leads to delayed wound healing or exacerbation of atherosclerotic ischemic diseases, it also inhibits the progression of cancer. Age-related changes of angiogenesis have been considered to at least partly result from vascular aging o...

  16. Dumping Syndrome

    Science.gov (United States)

    ... Intestinal Pseudo-obstruction Irritable Bowel Syndrome (IBS) Definition & Facts Symptoms & Causes Diagnosis Treatment Eating, Diet, & Nutrition Clinical Trials Irritable Bowel Syndrome (IBS) in Children Lactose Intolerance Ménétrier’s Disease Microscopic Colitis Ostomy Surgery of the ...

  17. Piriformis syndrome

    Science.gov (United States)

    Pseudosciatica; Wallet sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis ... Sciatica is the main symptom of piriformis syndrome. Other symptoms include: Tenderness or a dull ache in ...

  18. Alagille Syndrome

    Science.gov (United States)

    ... Liver Tumors Biliary Atresia Cirrhosis of the Liver Galactosemia Gilbert’s Syndrome Diseases of the Liver Glycogen Storage ... Liver Tumors Biliary Atresia Cirrhosis of the Liver Galactosemia Gilbert’s Syndrome Diseases of the Liver Glycogen Storage ...

  19. Reye Syndrome

    Science.gov (United States)

    ... Liver Tumors Biliary Atresia Cirrhosis of the Liver Galactosemia Gilbert’s Syndrome Diseases of the Liver Glycogen Storage ... Liver Tumors Biliary Atresia Cirrhosis of the Liver Galactosemia Gilbert’s Syndrome Diseases of the Liver Glycogen Storage ...

  20. Zellweger Syndrome

    Science.gov (United States)

    ... Zellweger syndrome (ZS, the most severe form), neonatal adrenoleukodystrophy (NALD), and Infantile Refsum disease (IRD, the least ... Zellweger syndrome (ZS, the most severe form), neonatal adrenoleukodystrophy (NALD), and Infantile Refsum disease (IRD, the least ...

  1. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    Criton S

    1995-01-01

    Full Text Available Proteus syndrome is a hamartomatous disorder characterised by focal overgrowths that can involve any structure of the body. An eleven-year-old girl with Proteus syndrome has been described with clitoromegaly.

  2. Overlap syndromes

    NARCIS (Netherlands)

    Beuers, Ulrich; Rust, Christian

    2005-01-01

    In hepatology, the term overlap syndrome describes variant forms of the major hepatobiliary autoimmune diseases, autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic

  3. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These ... doctors agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  4. Reye Syndrome

    Science.gov (United States)

    Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently ... a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, ...

  5. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

  6. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete X ... work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  7. Felty syndrome

    Science.gov (United States)

    Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

  8. Rett Syndrome

    Science.gov (United States)

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

  9. Alport Syndrome

    Science.gov (United States)

    ... body. Many people with Alport syndrome also have hearing problems and abnormalities with their eyes. Other signs and ... and inherited type of Alport syndrome. For example, hearing and vision problems tend to be more common in males than ...

  10. Moebius Syndrome

    Science.gov (United States)

    ... eye sensitivity; motor delays; high or cleft palate; hearing problems and speech difficulties. Children with Moebius syndrome are ... eye sensitivity; motor delays; high or cleft palate; hearing problems and speech difficulties. Children with Moebius syndrome are ...

  11. Heart and Down Syndrome

    Science.gov (United States)

    ... 4602 [email protected] Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ... Helpline » Follow us Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ...

  12. Down Syndrome: Education

    Science.gov (United States)

    ... 4602 [email protected] Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ... Helpline » Follow us Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ...

  13. Dental Issues & Down Syndrome

    Science.gov (United States)

    ... 4602 [email protected] Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ... Helpline » Follow us Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ...

  14. Down Syndrome: Education

    Science.gov (United States)

    ... Our Team Financial Information NDSS History About Down Syndrome Down Syndrome Preferred Language Guide Down Syndrome Facts Down ... Our Team Financial Information NDSS History About Down Syndrome Down Syndrome Down Syndrome Facts Preferred Language Guide Publications ...

  15. Facts About Usher Syndrome

    Science.gov (United States)

    ... Usher Syndrome > Facts About Usher Syndrome Facts About Usher Syndrome This information was developed by the National Eye ... is the best person to answer specific questions. Usher Syndrome Defined What is Usher syndrome? Usher syndrome is ...

  16. International Rett Syndrome Foundation

    Science.gov (United States)

    ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ...

  17. Vascular Aging and Arterial Stiffness

    Directory of Open Access Journals (Sweden)

    Luana de Rezende Mikael

    Full Text Available Abstract Cardiovascular diseases (CVD account annually for almost one third of all deaths worldwide. Among the CVD, systemic arterial hypertension (SAH is related to more than half of those outcomes. Type 2 diabetes mellitus is an independent risk factor for SAH because it causes functional and structural damage to the arterial wall, leading to stiffness. Several studies have related oxidative stress, production of free radicals, and neuroendocrine and genetic changes to the physiopathogenesis of vascular aging. Indirect ways to analyze that aging process have been widely studied, pulse wave velocity (PWV being considered gold standard to assess arterial stiffness, because there is large epidemiological evidence of its predictive value for cardiovascular events, and it requires little technical knowledge to be performed. A pulse wave is generated during each cardiac contraction and travels along the arterial bed until finding peripheral resistance or any bifurcation point, determining the appearance of a reflected wave. In young individuals, arteries tend to be more elastic, therefore, the reflected wave occurs later in the cardiac cycle, reaching the heart during diastole. In older individuals, however, the reflected wave occurs earlier, reaching the heart during systole. Because PWV is an important biomarker of vascular damage, highly valuable in determining the patient’s global cardiovascular risk, we chose to review the articles on vascular aging in the context of cardiovascular risk factors and the tools available to the early identification of that damage.

  18. [Capgras syndrome].

    Science.gov (United States)

    Alcoverro Fortuny, O; Sierra Acín, A C

    2001-01-01

    The authors report a case of Capgras' syndrome in a 16-years-old child, who had been hospitalized for psychotic disorder. A review of the literature is performed. Most authors state that Capgras' syndrome would represent a symptom of underlying medical o functional disorders, although the term syndrome is used. The main etiopathogenic hypothesis of this syndrome are put forward (psychodynamic, disconnection, neuropsychological and medical).

  19. Vascularization regenerative medicine and tissue engineering

    CERN Document Server

    Brey, Eric M

    2014-01-01

    A Complex and Growing Field The study of vascularization in tissue engineering and regenerative medicine (TERM) and its applications is an emerging field that could revolutionize medical approaches for organ and tissue replacement, reconstruction, and regeneration. Designed specifically for researchers in TERM fields, Vascularization: Regenerative Medicine and Tissue Engineering provides a broad overview of vascularization in TERM applications. This text summarizes research in several areas, and includes contributions from leading experts in the field. It defines the difficulties associated with multicellular processes in vascularization and cell-source issues. It presents advanced biomaterial design strategies for control of vascular network formation and in silico models designed to provide insight not possible in experimental systems. It also examines imaging methods that are critical to understanding vascularization in engineered tissues, and addresses vascularization issues within the context of specific...

  20. Metabolic Syndrome

    Science.gov (United States)

    ... much saturated fat, and does not get enough physical activity may develop metabolic syndrome. Other causes include insulin resistance and a family ... you’re overweight. It also includes getting more physical activity and eating a ... syndrome treatment If you already have metabolic syndrome, making ...

  1. Goodpasture Syndrome

    Science.gov (United States)

    ... necessary. Eating, Diet, and Nutrition Eating, diet, and nutrition have not been shown to play a role in causing or preventing Goodpasture syndrome. Points to Remember Goodpasture syndrome is a pulmonary-renal syndrome, which is a group of acute illnesses ...

  2. [Reye's syndrome].

    Science.gov (United States)

    Yoshida, I

    2000-11-01

    A nationwide survey on Reye's syndrome(RS) was described. And problems between RS and influenza virus such as etiology, pathophysiology, differential diagnosis and epidemiology were reviewed. So-called aspirin issue on RS was re-evaluated according to recent advance of RS research. Finally future aspect of Reye's syndrome was also discussed.

  3. Reye's Syndrome

    Science.gov (United States)

    ... Page You are here Home » Disorders » All Disorders Reye's Syndrome Information Page Reye's Syndrome Information Page What research is being done? Much ... Information from the National Library of Medicine’s MedlinePlus Reye's Syndrome × What research is being done? Much of the ...

  4. [Cardiorenal syndrome].

    Science.gov (United States)

    Salleck, D; John, S

    2017-09-13

    Patients in the intensive care unit often suffer from cardiorenal syndrome, which can have an important influence on the patient's outcome. The heart and kidney influence each other via organ crosstalk. We screened and evaluated current publications on cardiorenal syndromes and their therapy. A key role in the management of cardiorenal syndromes is renal decongestion via loop diuretics.

  5. Skin Findings in Williams Syndrome

    Science.gov (United States)

    Kozel, Beth A.; Bayliss, Susan J.; Berk, David R.; Waxler, Jessica L; Knutsen, Russell H.; Danback, Joshua R.; Pober, Barbara R.

    2014-01-01

    Previous examination in a small number of individuals with Williams syndrome (also referred to as Williams-Beuren syndrome) has shown subtly softer skin and reduced deposition of elastin, an elastic matrix protein important in tissue recoil. No quantitative information about skin elasticity in individuals with Williams syndrome is available; nor has there been a complete report of dermatologic findings in this population. To fill this knowledge gap, 94 patients with Williams syndrome aged 7-50 years were recruited as part of the Skin and Vascular Elasticity (WS-SAVE) study. They underwent either a clinical dermatologic assessment by trained dermatologists (2010 WSA family meeting) or measurement of biomechanical properties of the skin with the DermaLab™ suction cup (2012 WSA family meeting). Clinical assessment confirmed that soft skin is common in this population (83%), as is premature graying of the hair (80% of those 20 years or older), while wrinkles (92%) and abnormal scarring (33%) were detected in larger than expected proportions. Biomechanical studies detected statistically significant differences in dP (the pressure required to lift the skin), dT (the time required to raise the skin through a prescribed gradient), VE (viscoelasticity) and E (Young’s modulus) relative to matched controls. The RT (retraction time) also trended longer but was not significant. The biomechanical differences noted in these patients did not correlate with the presence of vascular defects also attributable to elastin insufficiency (vascular stiffness, hypertension, and arterial stenosis) suggesting the presence of tissue specific modifiers that modulate the impact of elastin insufficiency in each tissue. PMID:24920525

  6. Klippel-Trénaunay Syndrome with Intracranial Arteriovenous Malformation: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Mahniya F. Sadiq

    2014-01-01

    Full Text Available Klippel-Trénaunay syndrome (KTS is a rare vascular congenital anomaly affecting less than 200,000 people in the United States. Vascular malformations associated with KTS tend to affect slow flow systems: venous, capillary, and lymphatic systems. The nature of the syndrome leads to a higher risk for the development of arteriovenous malformations. Our case presentation describes a patient with KTS and an associated rare presentation of intraventricular arteriovenous malformation (AVM.

  7. The Role of Oxidative Stress in the Pathogenesis of Diabetic Vascular Complications

    Directory of Open Access Journals (Sweden)

    Shuji Sasaki

    2012-08-01

    Full Text Available Oxidative stress has been paid increasing attention to as an important causative factor for diabetic vascular complications. Among possible various sources, accumulating evidence has indicated that NAD(PH oxidase may be the most important source for reactive oxygen species production in diabetic vascular tissues. The mechanisms underlying activation and up-regulation of NAD(PH oxidase has been supposed to be mediated by high glucose-induced protein kinase C (PKC activation. In this review article, activation of local renin-angiotensin II system induced by chymase activation is also shown to amplify such a PKC-dependent activation of NAD(PH oxidase. Additionally, human evidence showing the beneficial effect of antioxidants on diabetic vascular complications. Bilirubin has been recognized as a strong endogenous antioxidant. Here markedly lower prevalence of vascular complications is shown in diabetic patients with Gilbert syndrome, a congenital hyperbilirubinemia, as well as reduced markers of oxidative stress and inflammation. Lastly, statin, angiotensin II receptor blocker, chymase inhibitor, bilirubin and biliverdin, PKC β isoform inhibitor, and glucagon-like peptide-1 analog, are shown to serve as antioxidants and have some beneficial effect on diabetic vascular complications, via inhibiting PKC-NAD(PH oxidase activation, supporting the notion that this mechanism may be an effective therapeutic target for preventing diabetic vascular complications.

  8. Drug hypersensitivity syndrome

    Directory of Open Access Journals (Sweden)

    Rashmi Kumari

    2011-01-01

    Full Text Available Drug hypersensitivity syndrome (DHS is an adverse drug reaction commonly associated with the aromatic antiepileptic drugs (AEDs, viz., phenytoin (PHT, carbamazepine (CBZ, phenobarbital (PB, lamotrigine, primidone, etc. It can also be caused by other drugs, such as sulfonamides, dapsone, minocycline, gold derivatives, cyclosporine, captopril, diltiazem, terbinafine, azathioprine and allopurinol. Diagnosis of DHS may be difficult because of the variety of clinical and laboratory abnormalities and manifestations and because the syndrome may mimic infectious, neoplastic or collagen vascular disorders. The risk for developing hypersensitivity within 60 days of the first or second prescription in new users of PHT or CBZ was estimated to be 2.3-4.5 per 10,000 and 1-4.1 per 10,000, respectively. The syndrome is defined by the fever, skin rash, lymphadenopathy and internal organ involvement within the first 2-8 weeks after initiation of therapy. Internal manifestations include, among others, agranulocytosis, hepatitis, nephritis and myositis. Insufficient detoxification may lead to cell death or contribute to the formation of antigen that triggers an immune reaction. Cross-reactivity among PHT, CBZ and PB is as high as 70%-80%. Management mainly includes immediate withdrawal of the culprit drug, symptomatic treatment and systemic steroids or immunoglobulins.

  9. Ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Pratap Kumar

    2011-01-01

    Full Text Available Ovarian hyperstimulation syndrome (OHSS is an iatrogenic complication of assisted reproduction technology. The syndrome is characterized by cystic enlargement of the ovaries and a fluid shift from the intravascular to the third space due to increased capillary permeability and ovarian neoangiogenesis. Its occurrence is dependent on the administration of human chorionic gonadotrophin (hCG. β-hCG and its analogs, estrogen, estradiol, prolactin, histamine and prostaglandins have all been implicated in OHSS but now it is increasingly better understood that the vasoactivesubstances such as interleukins, tumor necrosis factor-α, endothelin-1, and vascular endothelial growth factor (VEGF secreted by the ovaries have been implicated in increasing vascular permeability. Enlargement of the ovaries causes abdominal pain, nausea and vomiting. Leakage of fluid from follicles, increased capillary permeability leading to third spacing (due to the release of vasoactive substances, or frank rupture of follicles can all cause ascites. Due to leakage of fluid through the impaired blood vessels both within and outside the ovary there is massive fluid-shift from the intra-vescular bed to the third compartment results in intravascular hypovolemia with concomitant development of edema, ascites, hydrothorax and/or hydropericardium. Low-dose gonadotrophin protocols have been implemented to reduce the risks of fertility treatment in polycystic ovary syndrome patients. Prophylactic albumin administration may interrupt the development of OHSS by increasing the plasma oncotic pressure and binding mediators of ovarian origin. OHSS is significantly lower in an antagonist protocol than in an agonist protocol. Cabergoline inhibits partially the VEGF receptor 2 phosphorylation levels and associated vascular permeability without affecting luteal angiogenesis reduces the ′early′ (within the first 9 days after hCG onset of OHSS. To prevent thrombosis, subcutaneous heparin

  10. Vascular Thalamic Amnesia: A Reappraisal

    Science.gov (United States)

    Carlesimo, Giovanni Augusto; Lombardi, Maria Giovanna; Caltagirone, Carlo

    2011-01-01

    In humans lacunar infarcts in the mesial and anterior regions of the thalami are frequently associated with amnesic syndromes. In this review paper, we scrutinized 41 papers published between 1983 and 2009 that provided data on a total of 83 patients with the critical ischemic lesions (i.e. 17 patients with right-sided lesions, 25 with left-sided…

  11. HRCT of the lung in collagen vascular diseases; HRCT der Lunge bei Kollagenosen

    Energy Technology Data Exchange (ETDEWEB)

    Diederich, S. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany); Roos, N. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany); Schmitz-Linneweber, B. [Medizinische Klinik B, Westfaelische Wilhelms-Univ., Muenster (Germany); Gaubitz, M. [Medizinische Klinik B, Westfaelische Wilhelms-Univ., Muenster (Germany); Peters, P.E. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany)

    1996-07-01

    Collagen vascular diseases, representing systemic soft tissue disorders, may cause a broad spectrum of pathologic changes of the respiratory tract. The type and extent of manifestations can vary considerably among individuals and entities. This survey describes the chest radiographic and, in particular, high-resolution computed tomographic and, in particular, high-resolution computed tomographic (HRCT) findings of individual lesions of the respiratory tract. It includes fibrosing alveolitis (alveolitis, interstitial pneumonia, pulmonary fibrosis) and bronchial (bronchitis/bronchiolitis, bronchiectasis), pleural and vascular manifestations, as well as lymphadenopathy and abnormalities related to therapy. We present typical patterns of changes in progressive systemic sclerosis (PSS, scleroderma), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD, Sharp syndrome), Sjoegren syndrome, overlap syndrome and rheumatoid arthritis (RA). Furthermore, we describe findings which are specific for individual entities such as esophageal involvement in PSS, acute pneumonitis and pulmonary hemorrhage in SLE, lymphoproliferative disease in Sjoegren syndrome and necrobiotic nodules in RA. (orig.) [Deutsch] Die Kollagenosen koennen als systemische Bindegewebserkrankungen auch zu einem breiten Spektrum pathologischer Veraenderungen am Respirationstrakt fuehren, wobei sich Art und Ausmass der Manifestationen innerhalb einzelner Entitaeten und zwischen verschiedenen Krankheitsbildern erheblich unterscheiden koennen. In der vorliegenden Uebersicht werden die entsprechenden Befunde von Thoraxuebersichtsaufnahme und insbesondere hochaufloesender Computertomographie (HRCT) beschrieben. Beruecksichtigt werden dabei die fibrosierende Alveolitis (Alveolitis, interstitielle Pneumonie, Lungenfibrose), bronchiale (Bronchitis/Bronchiolitis, Bronchiektasen), pleurale und vaskulaere Manifestationen sowie Lymphadenopathie und therapie-induzierte Befunde. Typische Befundmuster

  12. Ghrelin improves vascular autophagy in rats with vascular calcification.

    Science.gov (United States)

    Xu, Mingming; Liu, Lin; Song, Chenfang; Chen, Wei; Gui, Shuyan

    2017-06-15

    This study aimed to investigate whether ghrelin ameliorated vascular calcification (VC) through improving autophagy. VC model was induced by nicotine plus vitamin D 3 in rats and β-glycerophosphate in vascular smooth muscle cell (VSMC). Calcium deposition was detected by von Kossa staining or alizarin red S staining. ALP activity was also detected. Western blot was used to assess the protein expression. Ghrelin treatment attenuated the elevation of calcium deposition and ALP activity in VC model both in vivo and in vitro. Interesting, the protein levels of autophagy markers, LC3 and beclin1 were significantly upregulated by ghrelin in VC model. An autophagy inhibitor, 3-methyladenine blocks the ameliorative effect of ghrelin on VC. Furthermore, protein expressions of phosphate-AMPK were increased by ghrelin treatment both in calcified aorta and VSMC. The effect of ghrelin on autophagy induction and VC attenuation was prevented by AMPK inhibitor, compound C. Our results suggested that ghrelin improved autophagy through AMPK activation, which was resulted in VC amelioration. These data maybe throw light on prevention and therapy of VC. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Effect of vascular burden as measured by vascular indexes upon vascular dementia: a matched case-control study

    OpenAIRE

    Takahashi, Paul Y; Caldwell, Casey R; Targonski, Paul V

    2012-01-01

    Paul Y Takahashi, Casey R Caldwell, Paul V TargonskiPrimary Care Internal Medicine, Mayo Clinic, Rochester MN, USABackground: Vascular dementia (VaD) is a challenging illness that affects the lives of older adults and caregivers. It is unclear how multiple vascular risk factor exposures (polyvascular disease) affect VaD.Purpose: To determine the relationship between multiple vascular risk exposures, as counted on an index in cases with VaD, compared with healthy age-/gender-matched controls.M...

  14. Vascular response to ischemia in the feet of falanga torture victims and normal controls--color and spectral Doppler findings

    DEFF Research Database (Denmark)

    Torp-Pedersen, Søren; Amris, Kirstine; Holm, Christian Cato

    2009-01-01

    OBJECTIVE: To investigate whether signs of chronic compartment syndrome could be found in plantar muscles of falanga torture victims with painful feet and impaired gait. The hypothesis was that the muscular vascular response to two minutes ischemia would be decreased in torture victims compared...

  15. Vascular dementia: Facts and controversies

    Directory of Open Access Journals (Sweden)

    Pavlović Aleksandra

    2013-01-01

    Full Text Available Vascular dementia (VaD is the second most frequent dementia after Alzheimer’s disease, and is diagnosed during lifetime in 20% of demented patients. Five­year survival rate in VaD is 39%, while it is estimated to be 75% in healthy persons of the same age. It is therefore important to make correct diagnosis of VaD early in the course of the disease. Risk factors for VaD are identical to stroke risk factors, and there are significant possibilities for the prevention of vascular cognitive decline. Cognitive decline develops acutely or step­by­step within three months after stroke, but more gradual progression of intellectual decline is also possible. Neurological examination can reveal pyramidal and extrapyramidal signs, pseudobulbar palsy, gait disturbance and urinary incontinence. Neuropsychological profile comprises the loss of cognitive set shifting, decline in word fluency, verbal learning difficulties, perseverations, difficulties in complex figure copying, and in patients with cortically located lesions also problems with speech and praxia. The basis of the diagnosis is, besides history, neurological examination and neuropsychological assessment, computed tomography and/ or magnetic resonance brain imaging. Vascular risk factors control is the most important measure in VaD prevention. Modern guidelines for the treatment of cognitive decline in VaD emphasize that donepezil can be useful in the improvement of cognitive status at the level of Class IIa recommendation at the level of evidence A, while memantine may be useful in patients with mixed VaD and Alzheimer’s disease dementia. [Projekat Ministarstva nauke Republike Srbije, br. 175022 i br. 175033

  16. MIGRAINE AND STROKE: VASCULAR COMORBIDITY

    Directory of Open Access Journals (Sweden)

    Donata eGuidetti

    2014-10-01

    Full Text Available Several comorbidities are associated to migraine.Recent meta-analyses have consistently demonstrated a relationship between migraine and stroke, which is well-defined for ischaemic stroke and migraine with aura, even stronger in females on oral contraceptives or smokers. However, there seems to be no clear-cut association between stroke in migraineurs and the common vascular risk factors, at least in the young adult population. Migraineurs also run an increased risk of hemorrhagic stroke, while the association between migraine and cardiovascular disease remains poorly defined.Another aspect is the relationship between migraine and the presence of silent brain lesions. It has been demonstrated that there is an increased frequency of ischaemic lesions in the white matter of migraineurs, especially silent infarcts in the posterior circulation territory in patients with at least 10 attacks per month. Although there is a higher prevalence of patent foramen ovale (PFO in migraineurs, the relationship between migraine and PFO remains controversial and PFO closure is not a recommended procedure to prevent migraine. As an increased frequency of cervical artery dissections has been observed in migrainous patients, it has been hypothesized that migraine may represent a predisposing factor for cervical artery dissection. There still remains the question as to whether migraine should be considered a true vascular disease or if the comorbidity between migraine and cerebrovascular disease may have underlying shared risk factors or pathophysiological mechanisms. Although further studies are required to clarify this issue, current evidence supports a clinical management where MA patients should be screened for other concomitant vascular risk factors and treated accordingly.

  17. Dengue Virus Nonstructural Protein 1 Induces Vascular Leakage through Macrophage Migration Inhibitory Factor and Autophagy.

    Directory of Open Access Journals (Sweden)

    Hong-Ru Chen

    2016-07-01

    Full Text Available Dengue virus (DENV is the most common mosquito-borne flavivirus; it can either cause mild dengue fever or the more severe dengue hemorrhagic fever (DHF and dengue shock syndrome (DSS. One of the characteristic features of DHF/DSS is vascular leakage; although DENV nonstructural protein 1 (NS1 has been proved to induce vascular leakage after binding to Toll-like receptor 4, the down-stream mechanism has not yet been fully understood. In the sera of DENV-infected patients, the concentrations of DENV NS1 and inflammatory cytokine macrophage migration inhibitory factor (MIF are positively correlated with disease severity, but whether DENV NS1 induces vascular leakage through MIF secretion remains unknown. We demonstrated that recombinant NS1 induced vascular leakage and MIF secretion both in human endothelial cell line HMEC-1 and in mice. Furthermore, these phenomena were inhibited in the presence of anti-NS1 antibodies both in vitro and in vivo. DENV NS1 also induced LC3-I to LC3-II conversion and p62 degradation in endothelial cell line, which indicated the formation of autophagy. To clarify whether MIF or autophagy mediated DENV NS1-induced vascular leakage, various inhibitors were applied. The results showed that DENV NS1-induced vascular leakage and VE-cadherin disarray were blocked in the presence of MIF inhibitors, anti-MIF-antibodies or autophagy inhibitors. An Atg5 knockdown clone further confirmed that autophagy formation of endothelial cells was required in NS1-induced vascular leakage. Furthermore, DENV NS1-induced LC3 puncta were also decreased in the presence of MIF inhibitors, indicating that MIF mediated DENV NS1-induced autophagy. Taken together, the results suggest a potential mechanism of DENV-induced vascular leakage and provide possible therapeutic targets against DHF/DSS.

  18. Spinal vascular malformations; Spinale Gefaessmalformationen

    Energy Technology Data Exchange (ETDEWEB)

    Yilmaz, U. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2012-05-15

    Spinal vascular malformations are a group of rare diseases with different clinical presentations ranging from incidental asymptomatic findings to progressive tetraplegia. This article provides an overview about imaging features as well as clinical and therapeutic aspects of spinal arteriovenous malformations, cavernomas and capillary telangiectasia. (orig.) [German] Spinale Gefaessmalformationen sind eine Gruppe seltener Erkrankungen mit unterschiedlichen klinischen Praesentationen, die vom asymptomatischen Zufallsbefund bis zur progredienten Tetraparese reichen. Dieser Artikel gibt einen Ueberblick ueber radiologische Befunde sowie klinische und therapeutische Aspekte von spinalen arteriovenoesen Malformationen, Kavernomen und kapillaeren Teleangiektasien. (orig.)

  19. Vascular comorbidities in multiple sclerosis

    DEFF Research Database (Denmark)

    Thormann, Anja; Magyari, Melinda; Koch-Henriksen, Nils

    2016-01-01

    To investigate the occurrence of vascular comorbidities before and after the clinical onset of multiple sclerosis. In this combined case–control and cohort study, all Danish born citizens with onset of multiple sclerosis 1980–2005 were identified from the Danish Multiple Sclerosis Registry...... and randomly matched with controls regarding year of birth, gender, and municipality on January 1st in the year of multiple sclerosis (MS) onset (index date). Individual-level information on comorbidities was obtained from several independent nationwide registries and linked to the study population by unique...

  20. Diagnosis and management of vascular injuries in the shoulder girdle of the overhead athlete.

    Science.gov (United States)

    Reeser, Jonathan C

    2007-10-01

    Vascular injuries represent a rare cause of shoulder pain and functional limitation among overhead athletes. Complaints of heaviness, fatigue, paresthesias, and effort-related pain should prompt the sports medicine clinician to consider vascular pathology as a possible cause of such symptoms. Position-dependent compression of the subclavian and axillary vessels within the thoracic outlet may result in functional limitation and a decline in overhead athletic performance, particularly when symptoms occur in the dominant upper limb. Treatment options include physical therapy and (in the case of thrombus) thrombolysis, but surgical decompression of the neurovascular bundle is generally advocated. This article reviews the diagnosis and management of effort thrombosis (also known as Paget-Schroetter syndrome), arterial thoracic outlet syndrome, and entrapment of the posterior circumflex humeral artery within the quadrilateral space. Familiarity with these conditions may help to minimize the risk of delayed diagnosis and associated morbidity.

  1. Validity of the clinical diagnostic criteria for vascular dementia: a critical review. Part II.

    Science.gov (United States)

    Wiederkehr, Sandra; Simard, Martine; Fortin, Claudette; van Reekum, Robert

    2008-01-01

    This review is the second of a two-part series focusing on the validity of eight clinical criteria for vascular dementia. Sixteen studies were selected according to their purposes and quality of experimental design. The analysis revealed that criteria for vascular dementia are not interchangeable; the eight criteria sets yielded different sensitivity and specificity results, as well as marked variability in incidence, prevalence, and frequency rates. Although the State of California Alzheimer's Disease Diagnostic and Treatment Centers (ADDTC) were the most sensitive and useful criteria in clinical settings and the National Institute of Neurological Disorders and Stroke-Association Internationale pour la Recherche et l'Enseignement en Neurosciences (NINDS-AIREN) were the most specific and useful criteria in research, all criteria shared similar flaws. A definition of the cognitive syndrome, associated vascular causes or lesions, and methods of assessment should be clearly specified in the future. Suggestions for improvement are made.

  2. Hepatic oxidative stress, genotoxicity and vascular dysfunction in lean or obese zucker rats

    DEFF Research Database (Denmark)

    Løhr, Mille; Folkmann, Janne Kjærsgaard; Sheykhzade, Majid

    2015-01-01

    Metabolic syndrome is associated with increased risk of cardiovascular disease, which could be related to oxidative stress. Here, we investigated the associations between hepatic oxidative stress and vascular function in pressurized mesenteric arteries from lean and obese Zucker rats at 14, 24...... of the vascular wall. There was increased maximal response to acetylcholine-mediated endothelium-dependent vasodilatation in both strains of rats. Collectively, the results indicate that obese Zucker rats only displayed a modest mesenteric vascular dysfunction, with no increase in hepatic oxidative stress...... and 37 weeks of age. Obese Zucker rats had more hepatic fat accumulation than their lean counterparts. Nevertheless, the obese rats had unaltered age-related level of hepatic oxidatively damaged DNA in terms of formamidopyrimidine DNA glycosylase (FPG) or human oxoguanine DNA glycosylase (hOGG1...

  3. DOWN SYNDROME WITH MOYAMOYA SYNDROME

    Directory of Open Access Journals (Sweden)

    Mohan Makwana

    2017-04-01

    Full Text Available BACKGROUND Moyamoya disease is a disorder of blood vessels in the brain, specifically the internal carotid arteries and the arteries that branch from them. The primary idiopathic form “moyamoya disease” has been distinguished from an associated form of “moyamoya syndrome,” in which the arterial changes are seen among patients with various syndromes or other disease processes- Down syndrome, sickle cell anaemia, neurofibromatosis type-1, congenital heart disease, fibromuscular dysplasia, activated protein C resistance, or head trauma. There have been only 47 previous cases of moyamoya syndrome in association with Down syndrome reported in the world literature. Recently, we have come across a Case of Downs’ Syndrome with Moyamoya Syndrome. Because of its rarity we want to report our case.

  4. [Vascular depression, limits of the concept].

    Science.gov (United States)

    Lebert, Florence

    2004-09-01

    The concept of vascular depression has recently been reassessed and more clearly delineated. The diagnostic criteria for vascular depression require a major depression associated with evidence of confluent or diffuse vascular lesions in the subcortical regions on MRI. The clinical symptoms are not specific, but they are often associated with mild cognitive decline. Ischemia is probably the main factor for vascular depression, but the relationship between ischemic lesions and clinical symptoms remains not well explained. The apolipoproteine E genotype is not a risk factor for vascular depression, but it is associated with more severe hyperintensities on MRI. A pharmacological resistance has been described in vascular depression, but, in recent studies, clinical improvement has been observed with antidepressants in more than 80% of cases. A neuropsychological follow-up is recommended, because dementia may appear with 25% of patients.

  5. Protecting against vascular disease in brain

    Science.gov (United States)

    2011-01-01

    Endothelial cells exert an enormous influence on blood vessels throughout the circulation, but their impact is particularly pronounced in the brain. New concepts have emerged recently regarding the role of this cell type and mechanisms that contribute to endothelial dysfunction and vascular disease. Activation of the renin-angiotensin system plays a prominent role in producing these abnormalities. Both oxidative stress and local inflammation are key mechanisms that underlie vascular disease of diverse etiology. Endogenous mechanisms of vascular protection are also present, including antioxidants, anti-inflammatory molecules, and peroxisome proliferator-activated receptor-γ. Despite their clear importance, studies of mechanisms that underlie cerebrovascular disease continue to lag behind studies of vascular biology in general. Identification of endogenous molecules and pathways that protect the vasculature may result in targeted approaches to prevent or slow the progression of vascular disease that causes stroke and contributes to the vascular component of dementia and Alzheimer's disease. PMID:21335467

  6. Pancreatic and Colonic Abscess Formation Secondary to HELLP Syndrome

    Directory of Open Access Journals (Sweden)

    James M. O’Brien

    2015-01-01

    Full Text Available Preeclampsia and the variant HELLP syndrome are systemic conditions associated with vascular changes resulting in vasoconstriction. Most commonly, patients present with elevated blood pressure and proteinuria, with a background of complaints such as headache, scotoma, and right upper quadrant pain. The systemic vascular changes experienced can target any organ system, oftentimes with more than one organ system being involved. We present the case of a patient admitted with HELLP syndrome who subsequently developed multisystem organ dysfunction, including placental abruption, disseminated intravascular coagulopathy, acute renal failure, colitis, abdominal ascites, pancreatitis, and the development of pancreatic and colonic abscesses.

  7. A combined vascular surgical and clinical genetics approach to diffuse aneurysmal disease

    Science.gov (United States)

    Jones, KA; Canham, N; Renton, S; Pollitt, R; Nesbitt, M; Kopcke, D; Islam, L; Buckley, J; Ghali, N; Vandersteen, A

    2015-01-01

    We report two patients who presented with extensive aneurysmal disease, in association with minimal external physical signs. Patient 1 remained genetically undiagnosed despite multiple structural, biochemical and genetic investigations. He made a good recovery following surgery for popliteal and left axillary artery aneurysms. Patient 2 was diagnosed with vascular type Ehlers–Danlos syndrome, associated with a high degree of tissue and blood vessel fragility, and is being managed conservatively. Early multidisciplinary assessment of such patients facilitates accurate diagnosis and management. PMID:26264107

  8. Novel Vascular Malformation in an Affected Newborn with Deletion Del(4)(q31.3)

    OpenAIRE

    de León Ojeda, Norma Elena; Soriano-Torres, Michel; Cabrera, Mercedes J.; Benítez Ramos, Dunia Bárbara

    2012-01-01

    We report on a newborn male patient with a terminal deletion in the long arm of the chromosome 4 with a congenital heart defect unreported before in association with this syndrome. The patient had multiple congenital anomalies including a pointed duplicated fingernail, low set posteriorly rotated ears, large anterior fontanel, micrognathia, glabellar capillary vascular malformation, and Interrupted Aortic Arch type C. The patient died due to multiple congenital malformations; a peripheral chr...

  9. Jellyfish Envenomation Resulting In Vascular Insufficiency And Neurogenic Injury of Upper Limb

    Directory of Open Access Journals (Sweden)

    Choong CYL

    2015-11-01

    Full Text Available Following a week after a jellyfish sting, a young man presented with regional cyanosis and threat of distal gangrene secondary to vascular spasm in the forearm. The patient also suffered from transient paresis and numbness of the affected upper limb. Contrasted imaging revealed unopacified vessels in the distal forearm and worsening swelling warranted emergency surgical fasciotomy for impending compartment syndrome. This case highlights the occurrence of jellyfish envenomation and the need for early treatment.

  10. Vascular adaption to physical inactivity in humans

    OpenAIRE

    Bleeker, M.W.P.

    2006-01-01

    This thesis presents studies on vascular adaptation to physical inactivity and deconditioning. Although it is clear that physical inactivity is an important risk factor for cardiovascular disease, the underlying physiological mechanisms have not yet been elucidated. In contrast to physical inactivity, exercise decreases the risk for cardiovascular disease. This beneficial effect of exercise is partly due to changes in vascular function and structure. However, far less is known about vascular ...

  11. Hand-arm vibration syndrome: A rarely seen diagnosis.

    Science.gov (United States)

    Campbell, Rebecca A; Janko, Matthew R; Hacker, Robert I

    2017-06-01

    Hand-arm vibration syndrome (HAVS) is a collection of sensory, vascular, and musculoskeletal symptoms caused by repetitive trauma from vibration. This case report demonstrates how to diagnose HAVS on the basis of history, physical examination, and vascular imaging and its treatment options. A 41-year-old man who regularly used vibrating tools presented with nonhealing wounds on his right thumb and third digit. Arteriography revealed occlusions of multiple arteries in his hand with formation of collaterals. We diagnosed HAVS, and his wounds healed after several weeks with appropriate treatment. HAVS is a debilitating condition with often irreversible vascular damage, requiring early diagnosis and treatment.

  12. Hand-arm vibration syndrome: A rarely seen diagnosis

    Directory of Open Access Journals (Sweden)

    Rebecca A. Campbell, BA

    2017-06-01

    Full Text Available Hand-arm vibration syndrome (HAVS is a collection of sensory, vascular, and musculoskeletal symptoms caused by repetitive trauma from vibration. This case report demonstrates how to diagnose HAVS on the basis of history, physical examination, and vascular imaging and its treatment options. A 41-year-old man who regularly used vibrating tools presented with nonhealing wounds on his right thumb and third digit. Arteriography revealed occlusions of multiple arteries in his hand with formation of collaterals. We diagnosed HAVS, and his wounds healed after several weeks with appropriate treatment. HAVS is a debilitating condition with often irreversible vascular damage, requiring early diagnosis and treatment.

  13. Enhanced Recovery after Vascular Surgery

    Directory of Open Access Journals (Sweden)

    Milena D. Stojanovic

    2018-01-01

    Full Text Available The beginnings of the enhanced recovery after surgery (ERAS program were first developed for patients in colorectal surgery, and after it was established as the standard of care in this surgical field, it began to be applied in many others surgical areas. This is multimodal, evidence-based approach program and includes simultaneous optimization of preoperative status of patients, adequate selection of surgical procedure and postoperative management. The aim of this program is to reduce complications, the length of hospital stay and to improve the patients outcome. Over the past decades, special attention was directed to the postoperative management in vascular surgery, especially after major vascular surgery because of the great risk of multiorgan failure, such as: respiratory failure, myocardial infarction, hemodynamic instability, coagulopathy, renal failure, neurological disorders, and intra-abdominal complications. Although a lot of effort was put into it, there is no unique acceptable program for ERAS in this surgical field, and there is still a need to point out the factors responsible for postoperative outcomes of these patients. So far, it is known that special attention should be paid to already existing diseases, type and the duration of the surgical intervention, hemodynamic and fluid management, nutrition, pain management, and early mobilization of patients.

  14. Neuroradiological findings in vascular dementia

    Energy Technology Data Exchange (ETDEWEB)

    Guermazi, Ali; Miaux, Yves; Suhy, Joyce; Pauls, Jon; Lopez, Ria [Synarc, Inc., Department of Radiology Services, San Francisco, CA (United States); Rovira-Canellas, Alex [Hospital General Universitari Vall d' Hebron, Unita de Resonancia Magnetica, Barcelona (Spain); Posner, Holly [Eisai, Inc., Teaneck, NJ (United States)

    2007-01-15

    There are multiple diagnostic criteria for vascular dementia (VaD) that may define different populations. Utilizing the criteria of the National Institute of Neurological Disorders and Stroke and Association Internationale pour la Recherche et l'Enseignement en Neurosciences (NINDS-AIREN) has provided improved consistency in the diagnosis of VaD. The criteria include a table listing brain imaging lesions associated with VaD. The different neuroradiological aspects of the criteria are reviewed based on the imaging data from an ongoing large-scale clinical trial testing a new treatment for VaD. The NINDS-AIREN criteria were applied by a centralized imaging rater to determine eligibility for enrollment in 1,202 patients using brain CT or MRI. Based on the above data set, the neuroradiological features that are associated with VaD and that can result from cerebral small-vessel disease with extensive leukoencephalopathy or lacunae (basal ganglia or frontal white matter), or may be the consequence of single strategically located infarcts or multiple infarcts in large-vessel territories, are illustrated. These features may also be the consequence of global cerebral hypoperfusion, intracerebral hemorrhage, or other mechanisms such as genetically determined arteriopathies. Neuroimaging confirmation of cerebrovascular disease in VaD provides information about the topography and severity of vascular lesions. Neuroimaging may also assist with the differential diagnosis of dementia associated with normal pressure hydrocephalus, chronic subdural hematoma, arteriovenous malformation or tumoral diseases. (orig.)

  15. [Classification of vascular anomalies (tumours and malformations). Clinical characteristics and natural history].

    Science.gov (United States)

    Redondo, P

    2004-01-01

    Vascular anomalies are divided into tumours and malformations. Haemangiomas are the most frequent amongst the former. Not normally present at birth, except in a premonitory form, they grow for 10-12 months due to hyperplasia, to subsequently undergo a progressive involution for a period that might last from ten to twelve years. They have an incidence of up to 12% in newborns; they are more common amongst girls; and are divided into superficial, deep and compound. Congenital haemangiomas and those that do not undergo involution are considered to be rare entities. Vascular malformations, with a lower incidence than haemangiomas, are always present at birth, they grow by hypertrophy and never undergo involution. According to the classification of the ISSVA, vascular malformations are divided - depending on the vessel affected - into capillary or venular (port-wine stain), venous, lymphatic, arteriovenous and combined or complex. Each of these has certain defining clinical and haemodynamic peculiarities. Within the final group are included some with a low flow, such as the Klippel-Trenaunay syndrome (venous and lymphatic venular vascular malformation associated with the muscular-skeletal hypertrophy of an extremity), and others with a high flow, such as the Parkes-Weber syndrome.

  16. Proatherogenic pathways leading to vascular calcification

    Energy Technology Data Exchange (ETDEWEB)

    Mazzini, Michael J. [Department of Cardiology, Boston University Medical Center, Boston, MA (United States); Schulze, P. Christian [Department of Medicine, Boston University Medical Center, Boston, MA (United States)]. E-mail: christian.schulze@bmc.org

    2006-03-15

    Cardiovascular disease is the leading cause of morbidity and mortality in the western world and atherosclerosis is the major common underlying disease. The pathogenesis of atherosclerosis involves local vascular injury, inflammation and oxidative stress as well as vascular calcification. Vascular calcification has long been regarded as a degenerative process leading to mineral deposition in the vascular wall characteristic for late stages of atherosclerosis. However, recent studies identified vascular calcification in early stages of atherosclerosis and its occurrence has been linked to clinical events in patients with cardiovascular disease. Its degree correlates with local vascular inflammation and with the overall impact and the progression of atherosclerosis. Over the last decade, diverse and highly regulated molecular signaling cascades controlling vascular calcification have been described. Local and circulating molecules such as osteopontin, osteoprogerin, leptin and matrix Gla protein were identified as critical regulators of vascular calcification. We here review the current knowledge on molecular pathways of vascular calcification and their relevance for the progression of cardiovascular disease.

  17. Vascular tumors and malformations in children, Introduction.

    Science.gov (United States)

    Maguiness, Sheilagh M

    2016-03-01

    Over the past decade, I have been amazed at the growth in the field of vascular anomalies. The recognition of vascular birthmarks as a defined area of medicine is a relatively recent event. The International Society for the Study of Vascular Anomalies (ISSVA) was founded by Drs John Mulliken and Anthony Young in the late 1970s. Mulliken and Glowacki's sentinel 1982 paper on the biologic classification of vascular anomalies further established the field, by providing clarity of nomenclature and unifying concepts that had previously been lacking. ©2016 Frontline Medical Communications.

  18. [Diagnosis and management of vascular anomalies].

    Science.gov (United States)

    Philandrianos, C; Degardin, N; Casanova, D; Petit, P; Bartoli, J-M; Bardot, J; Magalon, G

    2011-06-01

    Vascular anomalies are a complex pathological group. They are especially difficult to study because of confusion in the terminology used. The classification developed by the International Society for the Study of Vascular Anomalies (ISSVA) in 1996 allows using a common scientific language. There are two groups of lesions: vascular tumor and vascular malformation. The management of these anomalies is difficult and must involve an interdisciplinary approach including specialists in plastic surgery, radiology, pediatry and dermatology. We propose a simplified approach for the management of these pathologies. This approach is coming from the experience of Marseille (France) multidisciplinary team. Copyright © 2009 Elsevier Masson SAS. All rights reserved.

  19. Tumor vascular disruption using various radiation types

    Directory of Open Access Journals (Sweden)

    JJ Bevelacqua

    2014-04-01

    Full Text Available The feasibility of disrupting a tumor’s vascular structure with various radiation types and radionuclides is investigated. Calculated absorbed dose profiles for photons and 4He ions suggest that low-energy beta-gamma and alpha emitting radionuclides can deposit sufficient absorbed dose to disrupt a tumor’s vascular structure while minimizing the dose outside the blood vessel. Candidate radionuclides uniformly distributed in microspheres are theoretically investigated with respect to their vascular disruption potential and to offer an alternative to 90Y microsphere therapy. Requisite activities of candidate low-energy beta-gamma and alpha emitting radionuclides to facilitate vascular disruption are calculated.

  20. Vascular function in health, hypertension, and diabetes

    DEFF Research Database (Denmark)

    Nyberg, Michael Permin; Gliemann, Lasse; Hellsten, Ylva

    2015-01-01

    muscle, which can affect muscle function. Central aspects in the vascular impairments are alterations in the formation of prostacyclin, the bioavailability of NO and an increased formation of vasoconstrictors and reactive oxygen species (ROS). Regular physical activity effectively improves vascular...... to the formation of vasodilators such as nitric oxide (NO) and prostacyclin. In essential hypertension and type II diabetes, the endothelial function and regulation of vascular tone is impaired with consequent increases in peripheral vascular resistance and inadequate regulation of oxygen supply to the skeletal...