洞不全症候群の経過中に腎梗塞と脳梗塞を発症した先天性プロテインS欠損症の1例

Abstract

We report a case of hereditary protein S deficiency with both renal and cerebral infarction during follow-up of a patient with sick sinus syndrome. A 50-year-old man required permanent pacemaker implantation at our department in May 1991 for sick sinus syndrome. Based on his abdominal pain, nausea, vomiting, increased LDH and enhanced renal CT findings, we diagnosed right renal infarction. On December 16, 1991, he was admitted to our department for left hemiparesis and speech disturbance. Because of recurrent thrombotic episodes, we measured protein C and protein S in this patient and his family members. The index patient, his elder brother, and his niece were found to have protein S deficiency. The most common manifestations of protein S deficiency are thrombo- phlebitis of the lower limbs and pulmonary embolism. This is a very rare case of hereditary protein S deficiency with recurrent arterial thrombosis, especially renal infarction.