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Sean Mckeag - One of the best experts on this subject based on the ideXlab platform.

  • Spontaneous and Drug-induced Arteritis/Polyarteritis in the Göttingen Minipig—Review:
    Toxicologic pathology, 2018
    Co-Authors: Zuhal Dincer, Virginie Piccicuto, Ursula Junker Walker, Andreas Mahl, Sean Mckeag
    Abstract:

    Arteritis/Polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Gottingen minipigs, arteritis/Polyarteritis is an occasionally observed background change. In the minipig, this finding differs in frequency and nature from age-related Polyarteritis nodosa in rats or monkeys, and Beagle pain syndrome in dogs. In minipigs, it can be present in a single small- or medium-sized artery of an organ or a few organs and is most commonly recorded in the cardiac and extracardiac blood vessels, vagina, oviduct, rectum, epididymis, spinal cord, pancreas, urinary bladder, kidneys, and stomach. The etiology is unknown although it has been considered in minipigs as well as in rats, dogs, and monkeys to be possibly immune mediated. This background change is important with respect to its nature and distribution in the minipig in order to distinguish it from drug-induced vascular changes, which might occur in similar locations and have similar morphologic features. This review summarizes the morphology, incidence, and predilection sites of arteritis as a spontaneously occurring background change and as a drug-induced vasculopathy in the minipig, and also describes the main aspects to consider when evaluating vascular changes in Gottingen minipig toxicity studies and their human relevance.

  • spontaneous and drug induced arteritis Polyarteritis in the gottingen minipig review
    Toxicologic Pathology, 2018
    Co-Authors: Zuhal Dincer, Virginie Piccicuto, Ursula Junker Walker, Andreas Mahl, Sean Mckeag
    Abstract:

    Arteritis/Polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Gottingen minipigs, arteritis/Polyarteritis is an occasionally observed background change. In the minipig, this finding differs in frequency and nature from age-related Polyarteritis nodosa in rats or monkeys, and Beagle pain syndrome in dogs. In minipigs, it can be present in a single small- or medium-sized artery of an organ or a few organs and is most commonly recorded in the cardiac and extracardiac blood vessels, vagina, oviduct, rectum, epididymis, spinal cord, pancreas, urinary bladder, kidneys, and stomach. The etiology is unknown although it has been considered in minipigs as well as in rats, dogs, and monkeys to be possibly immune mediated. This background change is important with respect to its nature and distribution in the minipig in order to distinguish it from drug-induced vascular changes, which might occur in similar locations and have similar morphologic features. This review summarizes the morphology, incidence, and predilection sites of arteritis as a spontaneously occurring background change and as a drug-induced vasculopathy in the minipig, and also describes the main aspects to consider when evaluating vascular changes in Gottingen minipig toxicity studies and their human relevance.

Isabella Ceccherini - One of the best experts on this subject based on the ideXlab platform.

  • prevalence of cecr1 mutations in pediatric patients with Polyarteritis nodosa livedo reticularis and or stroke
    Pediatric Rheumatology, 2015
    Co-Authors: Roberta Caorsi, Alice Grossi, Antonella Insalaco, M Alessio, Silvana Martino, Elisabetta Cortis, A Morreale, Francesco Caroli, A Martini, Isabella Ceccherini
    Abstract:

    Methods Pediatric patients of Caucasian Italian origin with the following diseases/manifestations were included in the study: i) histologically confirmed polyartiritis nodosa (PAN) or cutaneous polyartiritis nodosa (cPAN), ii) persistent livedo reticularis with elevation of acute phase reactants, iii) ischemic or hemorrhagic strokes with systemic inflammation. Direct sequencing of CECR1 gene (exons 1-9) was performed with Sanger analysis.

  • sat0484 prevalence of cecr1 mutations in pediatric patients with Polyarteritis nodosa livedo reticularis and or stroke
    Annals of the Rheumatic Diseases, 2015
    Co-Authors: Roberta Caorsi, Alice Grossi, Antonella Insalaco, M Alessio, Silvana Martino, Elisabetta Cortis, A Morreale, Francesco Caroli, A Martini, Isabella Ceccherini
    Abstract:

    Background Mutations of CECR1 have been recently reported as causative of an inflammatory condition characterized by Polyarteritis nodosa, cerebral stroke and immunodeficiency; the clinical manifestations of the disease are heterogeneous with a wide range of severity. Objectives To analyze the prevalence of CECR1 mutations in pediatric patients with Polyarteritis nodosa, livedo reticularis and/or stroke. Methods Pediatric patients of Caucasian Italian origin with the following diseases/manifestations were included in the study: i) histologically confirmed polyartiritis nodosa (PAN) or cutaneous polyartiritis nodosa (cPAN), ii) persistent livedo reticularis with elevation of acute phase reactants, iii) ischemic or hemorrhagic strokes with systemic inflammation. Direct sequencing of CECR1 gene (exons 1-9) was performed with Sanger analysis. Results Up to January 2015, 27 patients from 25 families were included in the study. Homozygous or compound heterozygous CECR1 mutations with deleterious effects (G47R, G47A, P251L, R312X, E328D, T360A) were detected in 6 patients. A heterozygous causative mutation (G47V) was observed in 2 affected brothers, their father and the unaffected brother. So far a second mutation has not been detected; loss of heterozygosity could not be demonstrated and the mother is being investigated for a possible interstitial deletion. In the remaining patients common polymorphisms (L46L, N53N, H335R, Y453Y) were detected. The mean age of onset of the disease in genetically confirmed patients was 24 months (range 6 months – 5 years); all of them presented fever, elevation of acute phase reactants, livedo reticularis and a skin biopsy suggestive for vasculitis; two of them presented subcutaneous nodules while one of them presented ulcerations at extremities. Hypertension was detected in four patients, while one presented miocarditis. 3 patients presented one or more cerebral stroke during their disease course, while in 3 patients peripheral neuropathy was detected. 4 patients presented intestinal involvement (ranging from recurrent abdominal pain to intestinal perforation) and 2 patients presented growth delay, independent from steroidal treatment. Low immunoglobulin levels were detected in two patients. The clinical characteristics of the two heterozygous patients were similar: fever, livedo reticularis, increased acute phase reactants and hypogammaglobulinemia were detected in both; cerebral stroke occurred in one of them. Conclusions CECR1mutations are present in the Italian population and associated with severe cases of ADA2 deficiency. A clinical heterogeneity has been detected in genetically confirmed patients. In a few patients a typical phenotype was associated to incomplete or negative genotype, thus supporting the hypothesis of a genetic heterogeneity of this condition. Disclosure of Interest None declared

Zuhal Dincer - One of the best experts on this subject based on the ideXlab platform.

  • Spontaneous and Drug-induced Arteritis/Polyarteritis in the Göttingen Minipig—Review:
    Toxicologic pathology, 2018
    Co-Authors: Zuhal Dincer, Virginie Piccicuto, Ursula Junker Walker, Andreas Mahl, Sean Mckeag
    Abstract:

    Arteritis/Polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Gottingen minipigs, arteritis/Polyarteritis is an occasionally observed background change. In the minipig, this finding differs in frequency and nature from age-related Polyarteritis nodosa in rats or monkeys, and Beagle pain syndrome in dogs. In minipigs, it can be present in a single small- or medium-sized artery of an organ or a few organs and is most commonly recorded in the cardiac and extracardiac blood vessels, vagina, oviduct, rectum, epididymis, spinal cord, pancreas, urinary bladder, kidneys, and stomach. The etiology is unknown although it has been considered in minipigs as well as in rats, dogs, and monkeys to be possibly immune mediated. This background change is important with respect to its nature and distribution in the minipig in order to distinguish it from drug-induced vascular changes, which might occur in similar locations and have similar morphologic features. This review summarizes the morphology, incidence, and predilection sites of arteritis as a spontaneously occurring background change and as a drug-induced vasculopathy in the minipig, and also describes the main aspects to consider when evaluating vascular changes in Gottingen minipig toxicity studies and their human relevance.

  • spontaneous and drug induced arteritis Polyarteritis in the gottingen minipig review
    Toxicologic Pathology, 2018
    Co-Authors: Zuhal Dincer, Virginie Piccicuto, Ursula Junker Walker, Andreas Mahl, Sean Mckeag
    Abstract:

    Arteritis/Polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Gottingen minipigs, arteritis/Polyarteritis is an occasionally observed background change. In the minipig, this finding differs in frequency and nature from age-related Polyarteritis nodosa in rats or monkeys, and Beagle pain syndrome in dogs. In minipigs, it can be present in a single small- or medium-sized artery of an organ or a few organs and is most commonly recorded in the cardiac and extracardiac blood vessels, vagina, oviduct, rectum, epididymis, spinal cord, pancreas, urinary bladder, kidneys, and stomach. The etiology is unknown although it has been considered in minipigs as well as in rats, dogs, and monkeys to be possibly immune mediated. This background change is important with respect to its nature and distribution in the minipig in order to distinguish it from drug-induced vascular changes, which might occur in similar locations and have similar morphologic features. This review summarizes the morphology, incidence, and predilection sites of arteritis as a spontaneously occurring background change and as a drug-induced vasculopathy in the minipig, and also describes the main aspects to consider when evaluating vascular changes in Gottingen minipig toxicity studies and their human relevance.

Ferry Breedveld - One of the best experts on this subject based on the ideXlab platform.

  • vasculitis due to cholesterol embolism
    The American Journal of Medicine, 1997
    Co-Authors: Yvo Sijpkens, Rudi G J Westendorp, Folkert J Van Kemenade, Sjoerd Van Duinen, Ferry Breedveld
    Abstract:

    N ecrotizing vasculitis is a term applied to disorders in which there is segmental inflammation with fibrinoid necrosis of the blood vessels. The vasculitis syndromes are often grouped according to the size of the affected blood vessels.’ The name “Polyarteritis nodosa” is restricted to those clinical entities in which there is vasculitis in medium-sized and small arteries .without involvement of smaller vessels.‘a2 A clinical picture resembling Polyarteritis nodosa can be caused by embolization of cholesterol crystals. Cholesterol embolism, however, is merely considered as a pseudovasculitis syndrome.3’4 We report a patient with manifestations of vasculitis, who died from a hemorrhagic stroke. Autopsy revealed multiple arteries with cholesterolclefts and necrotizing vasculitis.

Roberta Caorsi - One of the best experts on this subject based on the ideXlab platform.

  • prevalence of cecr1 mutations in pediatric patients with Polyarteritis nodosa livedo reticularis and or stroke
    Pediatric Rheumatology, 2015
    Co-Authors: Roberta Caorsi, Alice Grossi, Antonella Insalaco, M Alessio, Silvana Martino, Elisabetta Cortis, A Morreale, Francesco Caroli, A Martini, Isabella Ceccherini
    Abstract:

    Methods Pediatric patients of Caucasian Italian origin with the following diseases/manifestations were included in the study: i) histologically confirmed polyartiritis nodosa (PAN) or cutaneous polyartiritis nodosa (cPAN), ii) persistent livedo reticularis with elevation of acute phase reactants, iii) ischemic or hemorrhagic strokes with systemic inflammation. Direct sequencing of CECR1 gene (exons 1-9) was performed with Sanger analysis.

  • sat0484 prevalence of cecr1 mutations in pediatric patients with Polyarteritis nodosa livedo reticularis and or stroke
    Annals of the Rheumatic Diseases, 2015
    Co-Authors: Roberta Caorsi, Alice Grossi, Antonella Insalaco, M Alessio, Silvana Martino, Elisabetta Cortis, A Morreale, Francesco Caroli, A Martini, Isabella Ceccherini
    Abstract:

    Background Mutations of CECR1 have been recently reported as causative of an inflammatory condition characterized by Polyarteritis nodosa, cerebral stroke and immunodeficiency; the clinical manifestations of the disease are heterogeneous with a wide range of severity. Objectives To analyze the prevalence of CECR1 mutations in pediatric patients with Polyarteritis nodosa, livedo reticularis and/or stroke. Methods Pediatric patients of Caucasian Italian origin with the following diseases/manifestations were included in the study: i) histologically confirmed polyartiritis nodosa (PAN) or cutaneous polyartiritis nodosa (cPAN), ii) persistent livedo reticularis with elevation of acute phase reactants, iii) ischemic or hemorrhagic strokes with systemic inflammation. Direct sequencing of CECR1 gene (exons 1-9) was performed with Sanger analysis. Results Up to January 2015, 27 patients from 25 families were included in the study. Homozygous or compound heterozygous CECR1 mutations with deleterious effects (G47R, G47A, P251L, R312X, E328D, T360A) were detected in 6 patients. A heterozygous causative mutation (G47V) was observed in 2 affected brothers, their father and the unaffected brother. So far a second mutation has not been detected; loss of heterozygosity could not be demonstrated and the mother is being investigated for a possible interstitial deletion. In the remaining patients common polymorphisms (L46L, N53N, H335R, Y453Y) were detected. The mean age of onset of the disease in genetically confirmed patients was 24 months (range 6 months – 5 years); all of them presented fever, elevation of acute phase reactants, livedo reticularis and a skin biopsy suggestive for vasculitis; two of them presented subcutaneous nodules while one of them presented ulcerations at extremities. Hypertension was detected in four patients, while one presented miocarditis. 3 patients presented one or more cerebral stroke during their disease course, while in 3 patients peripheral neuropathy was detected. 4 patients presented intestinal involvement (ranging from recurrent abdominal pain to intestinal perforation) and 2 patients presented growth delay, independent from steroidal treatment. Low immunoglobulin levels were detected in two patients. The clinical characteristics of the two heterozygous patients were similar: fever, livedo reticularis, increased acute phase reactants and hypogammaglobulinemia were detected in both; cerebral stroke occurred in one of them. Conclusions CECR1mutations are present in the Italian population and associated with severe cases of ADA2 deficiency. A clinical heterogeneity has been detected in genetically confirmed patients. In a few patients a typical phenotype was associated to incomplete or negative genotype, thus supporting the hypothesis of a genetic heterogeneity of this condition. Disclosure of Interest None declared