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1.
Korean Journal of Anesthesiology ; : 112-115, 2009.
Article in Korean | WPRIM | ID: wpr-22026

ABSTRACT

Lowe syndrome (the oculo-cerebro-renal syndrome of Lowe, OCRL) is a multi-system disorder that affects the eyes, nervous system, and kidney. OCRL is a rare X-linked recessive disease with a prevalence of approximately 1 : 500,000. The clinical features of OCRL include congenital cataracts, growth and mental retardation, areflexia, hypotonia, and renal tubular dysfunction (Fanconi-type). Chronic metabolic acidosis and hypotonia may be the most important component affecting management of the peri-anesthetic period during general anesthesia. However, problems such as electrolyte imbalance, seizure, fragility of the bone structures, and increased intraocular pressure should also be considered during the perioperative period. We report here the perioperative management of a patient with Lowe syndrome during the removal of multiple scalp cysts under general anesthesia.


Subject(s)
Humans , Acidosis , Anesthesia, General , Cataract , Eye , Intellectual Disability , Intraocular Pressure , Kidney , Muscle Hypotonia , Nervous System , Oculocerebrorenal Syndrome , Perioperative Period , Prevalence , Scalp , Seizures
2.
Korean Journal of Anesthesiology ; : 511-515, 2008.
Article in Korean | WPRIM | ID: wpr-99663

ABSTRACT

Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. The hemostatic aspects of antiphospholipid syndrome (APS) in patients requiring thrombectomy along with cardiopulmonary bypass present unique challenges to anesthesiologists.We present the case of a 36-year-old woman with thrombosis of the inferior vena cava as a result of APS with particular reference to the precautions that are necessary during the perioperative care.The particularly important things are the prevention of thrombotic or hemorrhagic complications, management of the associated thrombocytopenia and the laboratory methods of monitoring the perioperative anticoagulation in the setting of a prolonged clotting time.


Subject(s)
Adult , Female , Humans , Antiphospholipid Syndrome , Cardiopulmonary Bypass , Thrombectomy , Thrombocytopenia , Thrombosis , Vena Cava, Inferior
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