OSLER – WEBER – RENDU SYNDROME: A RARE CASE OF UPPER GASTROINTESTINAL BLEEDING

نویسندگان

چکیده

BACKGROUND: Osler-Weber-Rendu syndrome (Hereditary hemorrhagic telengiectasia) cases present with recurrent epistaxis, gastrointestinal bleeding (hematemesis, melena), and arteriovenous malformations involving almost all organs of body. Internal can occur from (telengiectasia) stomach or small bowel. CASE PRESENTATION: We a case who presented melena gross anemia (hemoglobin 3.5 gm/dL). He was admitted transfused red cell concentrates along intravenous fluids. His investigations including oesophagogastroscopy, colonoscopy were normal. Computed tomography mesenteric angiography revealed 02 sites telangiectasias. The telangiectasias embolized coiled resulting in successful control symptomatic improvement. CONCLUSION: emergency. Their management be worrisome challenging for both clinicians syndrome.

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منابع مشابه

Osler-Weber-Rendu Syndrome.

Telangiectasia may be identified by visual inspection during physical examination of the skin and oral cavity or by endoscopy. Diagnosis is made after clinical examination and genetic testing based on the Curacao criteria: telangiectasia in the face, hands or oral cavity; recurrent epistaxis; arteriovenous malformations with visceral involvement; and a positive family history. Diagnosis is conf...

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Osler-Weber-Rendu syndrome.

Osler–Weber–Rendu syndrome, also known as Hereditary Hemorrhagic Telangiectasia (HHT), is an autosomal dominant disorder. Telangiectasias and Arterio-Venous Malformations (AVMs) are vascular lesions present in HHT, most commonly causing epistaxis and gastrointestinal bleeding. While epistaxis presents as early as childhood, the gastrointestinal manifestations of HHT develop with increasing age.

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Osler-Weber-Rendu Syndrome

Presentation Age-related penetrance is seen in HHT. [2] It does not present at birth but commonly presents with recurrent epistaxis, usually in the teenage years. People with the condition develop mucocutaneous lesions, usually involving the nasal mucosa, lips and tongue. These lesions are sharply demarcated red-purple macules, papules or spider-like lesions comprising a mat of tortuous vessels...

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ژورنال

عنوان ژورنال: Khyber Medical University Journal

سال: 2022

ISSN: ['2305-2643', '2305-2651']

DOI: https://doi.org/10.35845/kmuj.2022.21271