A neuropathological study of cerebrovascular abnormalities in a signal transducer and activator of transcription 3–deficient patient Marie-Olivia Chandesris,

Slides:

Advertisements

Similar presentations

Grover disease with a contagious twist

Advertisements

Bow Hunter's Syndrome after Contralateral Vertebral Artery Dissection

Nondissecting aneurysm of the thoracic aorta with arteritis in systemic lupus erythematosus Hisato Takagi, MD, PhDa, Yoshio Mori, MD, PhDa, Hisashi Iwata,

Calcific elastosis in the setting of weight gain

Epilepsy, hippocampal sclerosis and amygdalar atrophy following H1N1-influenza- associated encephalopathy in an adult Shirin Karimi, Taufik A. Valiante,

A case of intravascular epithelioid hemangioendothelioma occurring 14 years after coil embolization for an extracranial internal carotid artery aneurysm

Successful allogeneic hematopoietic stem cell transplantation for DOCK8 deficiency Vincent Barlogis, MD, Claire Galambrun, MD, Hervé Chambost, MD, Sylvie.

Morphologic and immunohistochemical characterization of granulomas in the nucleotide oligomerization domain 2–related disorders Blau syndrome and Crohn.

Unsuspected mild emphysema in nonsmoking patients with chronic asthma with persistent airway obstruction Arthur F. Gelb, MD, Alfred Yamamoto, MD, Thais.

Reconstruction of pulmonary artery with porcine small intestinal submucosa in a lamb surgical model: Viability and growth potential Lorenzo Boni, MD,

Interferon response factor 3 is essential for house dust mite–induced airway allergy Thomas Marichal, DVM, Denis Bedoret, DVM, PhD, Claire Mesnil, DVM,

Histopathologic features (all images at ×10 magnification).

Selective dysfunction of p53 for mitochondrial biogenesis induces cellular proliferation in bronchial smooth muscle from asthmatic patients Thomas Trian,

True extracranial carotid artery aneurysm in a child

Is 9 more than 2 also in allergic airway inflammation?

A clinicopathologic study of immunoglobulin G4-related disease of the femoral and popliteal arteries in the spectrum of immunoglobulin G4-related periarteritis

Pathergy response to skin prick testing

Asthma therapy and airway remodeling

Breast arterial calcification in chronic kidney disease: absence of smooth muscle apoptosis and osteogenic transdifferentiation W. Charles O'Neill, Amy.

Renal artery stenosis and aneurysms associated with neurofibromatosis

Menno H. Raber, MD, Robbert Meerwaldt, MD, PhD, Rob J. van Det, MD

Ζ Chain–associated protein of 70 kDa (ZAP70) deficiency in human subjects is associated with abnormalities of thymic stromal cells: Implications for T-cell.

Abdominal Aorta Aneurysms in Children: Single-Center Experience of Six Patients Caisheng Ye, MD, PhD, Henghui Yin, MD, PhD, Ying Lin, MD, PhD, Li Zhou,

Polyethylene glycol–modified adenosine deaminase improved lung disease but not liver disease in partial adenosine deaminase deficiency Raz Somech, MD,

Recurrent Tamponade and Aortic Dissection in Syphilis

Antineutrophil Cytoplasmic Antibody—Associated Multiple Giant Saccular Aortic Aneurysms Ryoji Kinoshita, MD, Tomohiro Mizuno, MD, PhD, Tsuyoshi Hachimaru,

Gregory D. Jay, BE, BS, Frank L. Ross, MD, Robert A

Linear IgA disease limited to the oral mucosa

Common VW-MR imaging pitfalls.

Role of hemodynamic forces in the ex vivo arterialization of human saphenous veins Xavier Berard, MD, PhD, Sébastien Déglise, MD, Florian Alonso, PhD,

William W. Lin, BA, Gregory S. McGee, MD, Bruce K

Figure MRI and neuropathologic characteristics of the tumefactive demyelinating lesion in our patient MRI and neuropathologic characteristics of the tumefactive.

Gain-of-function signal transducer and activator of transcription 1 (STAT1) mutation– related primary immunodeficiency is associated with disseminated.

An unusual case of the right subclavian artery aneurysm resulting from long-term repetitive blunt chest trauma Yukihiro Matsuno, MD, PhD, Narihiro Ishida,

The temporal relationship between the development of vein graft intimal hyperplasia and growth factor gene expression John R. Hoch, MD, Vida K. Stark,

Mast cell–derived plasminogen activator inhibitor type 1 promotes airway inflammation and remodeling in a murine model of asthma Ara Jo, PhD, Sun H.

De novo renal artery aneurysm presenting 6 years after transplantation: A complication of recurrent arterial stenosis? Shelby H. Burkey, MD, Miguel A.

Pulmonary alveolar proteinosis in adenosine deaminase–deficient mice

Clifford M. Sales, MD, Michael L. Marin, MD, Frank J

Giant cell arteritis manifesting as mesenteric ischemia

Src homology 2 domain–containing inositol 5-phosphatase 1 deficiency leads to a spontaneous allergic inflammation in the murine lung Sun-Young Oh, PhD,

A 33-Year-Old Man With Multiple Bilateral Pulmonary Pseudoaneurysms

The role of the mast cell in the pathophysiology of asthma

Photodynamic therapy of vein grafts: Suppression of intimal hyperplasia of the vein graft but not the anastomosis Glenn M. LaMuraglia, MD, Michael L.

IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis

A case of intravascular epithelioid hemangioendothelioma occurring 14 years after coil embolization for an extracranial internal carotid artery aneurysm

Dissecting aneurysm of common iliac artery in a long-distance runner

James M. Wong, MDa, Michelle A

Intraabdominal hemorrhage as a result of segmental mediolytic arteritis of an omental artery: Case report Dianne M. Heritz, MD *, Jagdish Butany, MD,

What is an “eosinophilic phenotype” of asthma?

Purine metabolism, immune reconstitution, and abdominal adipose tumor after gene therapy for adenosine deaminase deficiency Eyal Grunebaum, MD, Catherine.

Weiguo Chen, PhD, Gurjit K. Khurana Hershey, MD, PhD

Autophagy: Nobel Prize 2016 and allergy and asthma research

Vascularity of human atrioventricular valves: A myth or fact?

Lymphoid neogenesis in the giant papillae of patients with chronic allergic conjunctivitis Akira Matsuda, MD, PhD, Nobuyuki Ebihara, MD, PhD, Norihiko.

Primary immunodeficiency disorder caused by phosphoinositide 3–kinase δ deficiency Georgios Sogkas, MD, PhD, Mykola Fedchenko, MD, Akshay Dhingra, MSc,

Renal artery aneurysm formation secondary to pseudoxanthoma elasticum

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked: Forkhead box protein 3 mutations and lack of regulatory T cells Troy R. Torgerson, MD,

Biology of nasal polyposis

Long-term pathologic consequences of acute irritant-induced asthma

Esophageal eosinophilia caused by milk proteins: From suspicion to evidence based on 2 case reports Soledad Terrados Cepeda, MD, Dario Antolin-Amerigo,

Eyal Grunebaum, MD, Ernest Cutz, MD, Chaim M. Roifman, MD

Methicillin-susceptible Staphylococcus aureus brain abscess in common variable immunodeficiency after an 8-month gap in return to the immunologist Niraj.

Superficial temporal artery aneurysm associated with immunoglobulin G4-related disease Sosei Kuma, MD, PhD, Tsubasa Takeshima, MD, Takefumi Ohga, MD,

Immunopathology of atopic dermatitis

Natural history of cow’s milk allergy

Progression of asthma in childhood

IgG4-related small-sized occlusive vasculitis in Mikulicz's disease

Brachial artery aneurysm and thrombosis secondary to fibromuscular dysplasia Julia Louise Jones, MBBS, Upeksha de Silva, MBBS, FRACS, Hwei Choo Soh, MBBS,

IgG4-related small-sized occlusive vasculitis in Mikulicz's disease

Presentation transcript:

A neuropathological study of cerebrovascular abnormalities in a signal transducer and activator of transcription 3–deficient patient Marie-Olivia Chandesris, MD, Françoise Gray, MD, PhD, Patrick Bruneval, MD, Arshid Azarine, MD, Nathalie Boddaert, MD, PhD, Catherine Oppenheim, MD, PhD, François Paraire, MD, Emmanuel Touzé, MD, PhD, Damien Bonnet, MD, PhD, Olivier Hermine, MD, PhD, Olivier Lortholary, MD, PhD, Valérie Cormier-Daire, MD, PhD, Pierre Boutouyrie, MD, PhD, Ziad Mallat, PhD, Alain Fischer, MD, PhD, Capucine Picard, MD, PhD Journal of Allergy and Clinical Immunology Volume 136, Issue 5, Pages 1418-1421.e5 (November 2015) DOI: 10.1016/j.jaci.2015.05.021 Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Microscopic analysis of the basilar trunk. A, Preserved media (arrowhead), irregular thickening of the internal elastic lamina (arrow), and focal intimal hyperplasia (asterisk) in the nonaneurysmal part of the artery (hematoxylin and eosin). B, Fibrosis and complete loss of VSMCs in the media (arrowhead) in the aneurysmal part of the artery (hematoxylin and eosin). C, Thickening of the internal elastic lamina highlighted by elastic stain. D, Massive calcification (arrow) of media (von Kossa stain). Journal of Allergy and Clinical Immunology 2015 136, 1418-1421.e5DOI: (10.1016/j.jaci.2015.05.021) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 White matter and intracerebral small-vessel disease. A, Coronal section of the right cerebral hemisphere (Klüver and Barrera stain) revealing mild and ill-delimited myelin pallor in the deep white matter. B, Spongiosis and oligodendroglial tumefaction (Klüver and Barrera stain). C, Microglial activation (anti-CD68 staining). D, Astrocytic gliosis (glial fibrillary acidic protein). E, Dilation of the perivascular spaces with plasma exudate and accumulation of macrophages containing brownish pigment (hematoxylin and eosin). F and G, Convoluted and frizzy-like aspect of the small-vessel wall (Fig 2, F, hematoxylin and eosin; Fig 2, G, periodic acid–Schiff). Journal of Allergy and Clinical Immunology 2015 136, 1418-1421.e5DOI: (10.1016/j.jaci.2015.05.021) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Brain magnetic resonance imaging (3-Tesla magnetic resonance imaging) findings. A and B, Three-dimensional reconstruction of contrast-enhanced magnetic resonance angiographic data, revealing a 19.5 × 17–mm saccular aneurysm of the basilar artery. C and D, Axial fluid–attenuated inversion recovery images demonstrating the presence of white matter hyperintensities (arrow) and linear periventricular hyperintensities (arrowhead). Journal of Allergy and Clinical Immunology 2015 136, 1418-1421.e5DOI: (10.1016/j.jaci.2015.05.021) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E2 Microscopic appearance of the arteries in the circle of Willis. A, Full section of the basilar artery (hematoxylin and eosin stain). The vessel appears somewhat flattened, with a thin wall. B, Full section of the right vertebral artery (hematoxylin and eosin stain). Again, the vessel appears somewhat flattened, with a thin wall. Note the basophilic calcium deposits in the media and (focally) intima. C, Left middle cerebral artery (hematoxylin and eosin stain) showing focal endothelial hyperplasia. Note the eosinophilic deposits under the intima (arrow). D, Full section of the left vertebral artery section (von Kossa stain). The vessel appears somewhat flattened, with a thin wall. Note the calcium deposits (stained black with the von Kossa technique) in the adventitia (arrows). E-H, Right vertebral artery (Fig E2, E, orcein stain for elastin; Fig E2, F, hematoxylin and eosin stain; Fig E2, G, alcian blue stain; and Fig E2, H, von Kossa stain). The internal elastic membrane shows abnormal thickening, irregularities and disruptions (Fig E2, E) associated with alcianophilic (mucopolysaccharide) deposits (Fig E2, G) and basophilic (Fig E2, F) von Kossa–positive (Fig E2, H) calcium deposits. Journal of Allergy and Clinical Immunology 2015 136, 1418-1421.e5DOI: (10.1016/j.jaci.2015.05.021) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E3 Expression levels of TGF-β, phospho-SMAD2, STAT3, and phospho-STAT3 in VSMCs from a normal (control) artery and from the basilar artery aneurysm. TGF-β expression was higher in the aneurysm than in the normal artery. No difference was observed for phospho-SMAD2. Although both the control artery and basilar aneurysm artery expressed similar levels of STAT3, phospho-STAT3 was differentially expressed. The VSMCs in the basilar artery aneurysm did not express phospho-STAT3, whereas the protein was strongly expressed in the normal (control) artery. Immunohistochemistry was performed with specific primary antibodies (original magnification ×10). Journal of Allergy and Clinical Immunology 2015 136, 1418-1421.e5DOI: (10.1016/j.jaci.2015.05.021) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions