Home Keys to Success in Medicine Cardiovascular System Respiratory System Locomotor System Endocrine and Metabolic System Kidneys and Urinary System Gastrointestinal Tract Central Nervous System Haematological System Integumental System Reproductive and Genital System Recommended Reading Forum Links
amazon astore
ydr
aces for paces
Hereditary Haemorrhagic Telangiectasia Title Ñ Hereditary Haemorrhagic Telangiectasia Definition Ñ Rare, autosomal dominant disorder Characterised by: Ñ Multiple small (< 5mm) telangiectasiae Of: Ñ Skin and mucous membranes Aetiology Ñ Autosomal dominant inheritance Pathogenesis Ñ Dilatation of capillaries and small arterioles Ñ Bleed easily Clinical Features History Age Ñ Tendency to bleed increases with age RS Ñ Epistaxis GIT Ñ Gastrointestinal bleeding from multiple sites Examination IS Ñ Telangiectasiae in the skin RS Ñ Telangiectasiae in the nose Ñ Arterio-venous malformations in the lungs: Associated clubbing and cyanosis GIT Ñ Telangiectasiae in the lips and mouth Ñ Cirrhosis of the liver: Due to telangiectasiae or multiple transfusions Ñ Massive intrahepatic shunting KUS Ñ Telangiectasiae in the urinary tract RAG Ñ Telangiectasiae in the vagina HS Ñ Anaemia Investigations Fluids Blood Haematology Ñ Anaemia Endoscopy GI endoscopy Ñ Visualise telangiectasiae Management Control Drugs Ñ Oral oestrogens: Some patients respond Surgery Ñ Cauterisation of lesions
Hereditary Haemorrhagic Telangiectasia
Title
Ñ Hereditary Haemorrhagic Telangiectasia
Definition
Ñ Rare, autosomal dominant disorder
Characterised by:
Ñ Multiple small (< 5mm) telangiectasiae
Of:
Ñ Skin and mucous membranes
Aetiology
Ñ Autosomal dominant inheritance
Pathogenesis
Ñ Dilatation of capillaries and small arterioles
Ñ Bleed easily
Clinical Features
History
Age
Ñ Tendency to bleed increases with age
RS
Ñ Epistaxis
GIT
Ñ Gastrointestinal bleeding from multiple sites
Examination
IS
Ñ Telangiectasiae in the skin
Ñ Telangiectasiae in the nose
Ñ Arterio-venous malformations in the lungs:
Associated clubbing and cyanosis
Ñ Telangiectasiae in the lips and mouth
Ñ Cirrhosis of the liver:
Due to telangiectasiae or multiple transfusions
Ñ Massive intrahepatic shunting
KUS
Ñ Telangiectasiae in the urinary tract
RAG
Ñ Telangiectasiae in the vagina
HS
Ñ Anaemia
Investigations
Fluids
Blood
Haematology
Endoscopy
GI endoscopy
Ñ Visualise telangiectasiae
Management
Drugs
Ñ Oral oestrogens:
Some patients respond
Surgery
Ñ Cauterisation of lesions
[ Back ] [ Up ] [ Next ]
[Up]
