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Weekly Chest CasesArchive of Old Cases

Case No : 1065 Date 2018-03-26

  • Courtesy of Hee Kang / Kosin University Gospel Hospital, Busan
  • Age/Sex 53 / F
  • Chief ComplaintDyspnea
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Osler-Weber-Rendu disease (Hereditary hemorrhagic telangiectasia)
Radiologic Findings
Figure 1. Chest radiography shows two lobulated nodule and mass continuing to tortous vessels in left middle and lower lung zone
Figure 2-3. Axial CT images show the nodule and mass which is enhanced comparable to vessels and connected with the pulmonary artery and vein. They can be confirmed with pulmonary arteriovenous malformation.
Figure 4-5 Multifocal mucosal telangiectasias are seen in gastric endoscopy and colonoscopy.
Brief Review
She had history of recurrent epistaxis, getting bruises and chronic anemia. She was diagnosed with Osler-Weber-Rendu disease (Hereditary hemorrhagic telangiectasia) by meeting three of the criteria.
Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a rare autosomal dominant multiorgan pathology characterized by the presence of multiple small telangiectases of the skin, mucous membranes, gastrointestinal tract, and other organs, with associated recurrent episodes of bleeding from affected sites. Although everyday symptoms in these patients are dominated by epistaxis that may markedly alter their quality of life, more severe manifestations of the disease are due to the consequences of pulmonary arteriovenous malformations (PAVMs) and, less often, to liver, brain, or gastrointestinal tract involvement. The diagnosis of HHT requires the presence of at least three of the following Curacao criteria: recurrent spontaneous epistaxis, mucocutaneous telangiectasis, an autosomal dominant familial distribution of the disease, and visceral AVMs. PAVM is the most frequent lesion of the lung in patients with HHT. It is usually silent at the time of PAVM induced. Strokes and brain abscess can be diagnosed at low risk.
References
1. Govani F Shovlin C. Hereditary haemorrhagic telangiectasia: a clinical and scientific review European Journal of Human Genetics 2009;17:860–871.
2. Carette M Nedelcu C Tassart M Grange J Wislez M et. al. Imaging of Hereditary Hemorrhagic Telangiectasia CardioVascular and Interventional Radiology 2009;32:745-757.
Keywords
Lung, Hereditary,

No. of Applicants : 138

▶ Correct Answer : 124/138,  89.9%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Hyogo Prefectural Amagasaki General Medical Center , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Chonbuk National University Medical School , Korea (South) HYEJIN YANG
  • - NIMS, Hyderabad , India AMIT DADHANIA
  • - Ajou University School of Medicine , Korea (South) JUNG YONG JUN
  • - Gifu University Hospital , Japan TOMOHIRO ANDO
  • - Affilitated Hospital of Jining Medical University , China HUA SHENG JIANG
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - CH de la Cote Basque , France PAUL ARDILOUZE
  • - , Korea (South) HANKYUL KIM
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Ajou university hospital , Korea (South) Pae Sun Suh
  • - Chonbuk National University Hospital , Korea (South) SANGHEON KIM
  • - Ondokuz Mayis University , Turkey CETIN CELENK
  • - Chonbuk National University Hospital , Korea (South) EUN HA JUNG
  • - Soonchunhyang University College of Medicine , Korea (South) GOUN CHOI
  • - Japanese Red Cross Medical Center , Japan DAISUKE YAMADA
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) HEE SEOK CHOI
  • - Other , Korea (South) SEONGSU KANG
  • - , India SANTOSH KUMAR PADHY
  • - , Korea (South) DA HYUN LEE
  • - Asan Medical Center , Korea (South) HYUN JUNG KOO
  • - Freelance resident in Reunion Island , Reunion Fabien HO
  • - Chungbuk National University Hospital , Korea (South) CHANGHOON OH
  • - 罹…stanbul , Turkey AYHAN YILMAZ
  • - privato , Italy VINCENZO ENZO GENCHI
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Azienda Ospedaliera Universitaria Integrata di Verona , Italy GIAN ALBERTO SOARDI
  • - The Jikei university , Japan TAKU GOMI
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Kyungpook national university hospital , Korea (South) Nari Jung
  • - , India THE GREAT WARRIOR
  • - , France GUILLAUME VESSELLE
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Ajou University Hospital , Korea (South) Taeyang Ha
  • - Radiology resident , Cambodia HUY SOK
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - McGill University / University College London , Canada Ilan Azuelos
  • - Yonsei University, Wonju Severance Christian Hospital , Korea (South) JUYEON LEE
  • - HHS , Canada S LEE
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - , France JULIEN BALIQUE
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Asan Medical Center , Korea (South) JOOAE CHOE
  • - Chungbuk National University Hospital , Korea (South) YOUNGHUN JEON
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Seoul National University Hospital , Korea (South) JI HEE KANG
  • - VDC, HYDERABAD , India KARTHIK RAYASAM
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
  • - , United Kingdom KRISHNA PRASAD PREMNATH BELLAM
  • - NTUH , Taiwan KUEI-PIN CHUNG
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - Seoul National University Bundang Hospital , Korea (South) YOUNGJUNE KIM
  • - Samsung Medical Center , Korea (South) KYOWON GU
  • - Ewha Womans University Medical Center , Korea (South) YOUNGSUN KO
  • - Medicheck health care , Korea (South) Chae hun Lim
  • - , Korea (South) DAYOUNG KIM
  • - National Taiwan University Hospital Hsin-Chu Branch , Taiwan LI-TA KENG
  • - Other , Korea (South) MARI A
  • - Other , Korea (South) JIN YOUNG KIM
  • - Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
  • - CLINIQUE STE CLOTILDE , Reunion PATRICK MASCAREL
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Kyeongpook National University Hospital , Korea (South) MIN A LEE
  • - Other , Korea (South) HAYEON LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Centre Hospitalier Regional de Lanaudiere , Canada Matthieu Storme
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Soonchunhyang University Hospital Seoul , Korea (South) BODA NAM
  • - Yonsei University,Severance Hospital , Korea (South) KYUNGSUN NAM
  • - , Japan NAOMI YUASA
  • - Kyemyung University Dongsan Hospital , Korea (South) NAM YEOKYOUNG
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Chungbuk national university hospital , Korea (South) Miran Yeon Yeon
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Other , Korea (South) SANGHYUN PARK
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - , Korea (South) JANG SEONG WON
  • - Chonbuk National University Hospital , Korea (South) WEON JANG
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - Myongji Hospital , Korea (South) DAE WOONG PARK
  • - Louisiana State University - Shreveport , United States CARLOS H PREVIGLIANO
  • - Konkuk University Medical Center , Korea (South) YONG WONN KWON
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
  • - Yujin Yamazaki Hospital , Japan MASASHI TAKAHASHI
  • - Escola Paulista de Medicina , Brazil RENATO MASSON DE ALMEIDA PRADO
  • - TB centre kasaragod. , India rikhy krishnan
  • - MBAL BURGAS , Bulgaria VLADISLAV BOYANOV RUSINOV
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Gachon University Gil Hospital , Korea (South) KIHYUN LEE
  • - Chonbuk National University Hospital , Korea (South) SOL KI KIM
  • - , Australia SANKAR MONDAL
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Other , Korea (South) TAE SUN KANG
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - The University of Tokyo Hospital , Japan TOMOKI WADA
  • - , Japan YUMI MAEHARA
  • - , Korea (South) SEOYOUNG PARK
  • - Hamamatsu university school of medicine , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Chonnam National University Hwasun Hospital , Korea (South) JONG EUN LEE
  • - University of Utah , United States AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Kyemyung University Dongsan Hospital , Korea (South) DONG HYEON KIM
  • - Ajou University Hospital , Korea (South) YONG HO JANG
  • - Chungbuk National University Hospital , Korea (South) HYEONMI RYU
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - Samsung Medical Center , Korea (South) HYUN JUNG YOON
  • - Ajou University Hospital , Korea (South) YOO YOUNGJIN
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 7/138,  5.1%
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - Hospital Espa , Argentina JORGE PICOREL
  • - , United States MAANSI P
  • - Lilavati Hospital , India DHRUV UMESH SHARMA
  • - Grant medical college Mumbai , India VISHWANATH VASANT PUJARI
  • - Kaohsiung Veterans General Hospital , Taiwan MING-TING WU
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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