نتایج جستجو برای: pachydermoperiostosis

تعداد نتایج: 143  

Journal: :The Journal of rheumatology 2015
Gabriella Giancane Christine P Diggle Elizabeth G Legger Janneke Tekstra Berent Prakken Arjan B Brenkman Ian M Carr Alexander F Markham David T Bonthron Nico Wulffraat

Hypertrophic osteoarthropathy (HO) is a disorder characterized by changes to the skin and bones, and occurs either in a rare familial primary form [primary hypertrophic osteoarthropathy, (PHO)], also called pachydermoperiostosis (PDP), with a 9:1 male:female prevalence ratio, or more commonly secondary to an underlying pathology1. Key features include digital clubbing, periostosis with bone and...

2017
Hengyan Zhang Bo Yang

RATIONALE Pachydermoperiostosis (PDP) is a rare hereditary disorder that affects the skin and bones. PDP is characterized by periostosis, digital clubbing, and pachydermia. Previous studies demonstrated that increased prostaglandin E2 (PGE2) levels resulting from defective protein degradation pathways play a crucial role in PDP pathogenesis, and males were more commonly and severely affected th...

Journal: :Journal of Korean medical science 2016
Sihoon Lee So Young Park Hyun Jin Kwon Chul-Ho Lee Ok-Hwa Kim Yumie Rhee

Pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy, is a rare genetic disease affecting both skin and bones. Both autosomal dominant with incomplete penetrance and recessive inheritance of PDP have been previously confirmed. Recently, hydroxyprostaglandin dehydrogenase (HPGD) and solute carrier organic anion transporter family member 2A1 (SLCO2A1) were reported as pathogenic ...

مقدمه br> بیماری سلیاک یک علت شایع سندروم سوء جذب در نژاد قفقازی می باشد که با اسهال مزمن و استئاتوره و گاها آنمی فقر آهن و استئوپرومالاسمی و آرتریت تظاهر پیدا می کند. بیماری سلیاک می تواند منجر به استئوآرتروپاتی هیپرتروفیک ثانویه شود که در این صورت با کلابینگ و راکسیون پری استئال استخوانهای بلند مشخص می شود.معرفی مورد: بیمار یک آقای 22 ساله با سابقه بیماری سلیاک از 10 سال قبل می باشد که به عل...

Journal: :The Permanente journal 2013
Vishal Sharma Alka Sharma Vivek Kumar Sourabh Aggarwal

Vishal Sharma, MD, is a Senior Resident in the Department of Medicine at the University College of Medical Sciences, Delhi, India. E-mail: [email protected]. Alka Sharma, MD, is a Postgraduate Resident in the Department of Medicine at the University College of Medical Sciences, Delhi, India. E-mail: [email protected]. Vivek Kumar, MD, DM, is a Senior Resident in the Department of Medici...

Journal: :Blood 1995
M Fontenay-Roupie E Dupuy E Berrou G Tobelem M Bryckaert

Pachydermoperiostosis or primary hypertrophic osteoarthropathy (HOA) is a rare congenital growth disorder of connective tissue. We report a case of severe myelofibrosis in a patient with HOA. When cultured in vitro, patient bone marrow-derived fibroblasts displayed a high proliferative potential with a shortened doubling time (24 hours v 36 to 48 hours for normal fibroblasts). The role of plate...

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