Transvenous Embolization for Carotid-Cavernous Fistula in a Patient with Vascular Type of Ehlers-Danlos Syndrome—Direct Superior Ophthalmic Vein Approach: Case Report

The vascular type of Ehlers-Danlos syndrome (vEDS) is an autosomal dominant hereditary disease characterized by connective tissue fragility throughout the body, including the arteries, viscera, and gastrointestinal tract. We report a case in which we performed transvenous embolization (TVE) via direct superior ophthalmic vein (SOV) approach to treat a direct carotid-cavernous fistula (CCF) in a patient with Ehlers-Danlos syndrome (EDS). The patient was a 37-year-old woman who developed tinnitus in her left ear and a headache during examination in the outpatient clinic of another hospital in order to make a definitive diagnosis of vEDS, and she was referred to our hospital and examined. Based on the results of all of the studies she was diagnosed with a CCF. Conservative treatment was attempted, but was not very effective. Because of progressing aphasia, TVE was performed via the SOV direct cut. There were no intraoperative or postoperative complications. It has been reported that cerebral angiography is generally contraindicated in vEDS and that the morbimortality associated with endovascular treatment is very high. When performing treatment it is necessary to be sufficiently aware of the risks it entails.     

Ferritin Localization on the Erythroblast Cell Membrane and Ropheocytosis in Hypersiderotic Human Bone Marrows

1. Preferential ferritin localization on specialized areas of the plasma membrane of erythroblasts with subsequent invagination into ropheocytotic vesicles, and the occurrence of ropheocytosis without ferritin being present in thevesicles, has been previously described.

2. In a study of bone marrows from patients with hypersiderosis we haveconfirmed the previous observations, but we frequently found nonferritinropheocytosis in erythroblasts adjacent to ferritin laden reticulum cells. Inaddition, ferritin localization along the outer surface of erythroblasts wasnoted, particularly between continuous surfaces of two adjacent erythroblasts.Such intercellular accumulations of ferritin were not necessarily associatedwith a high ferritin content of adjacent cells.

3. These observations did not favor the hypothesis that ferritin iron wasbeing transferred directly from reticulum cells to adjacent erythroblasts. It issuggested that the accumulation of ferritin along extended stretches of theerythroblast surface may signify that iron which is detached from siderophilinbut not immediately transported across the cell membrane is incorporated intoapoferritin produced on the cell membrane.

Submitted on January 12, 1966 Accepted on April 4, 1966     

Catecholaminergic Polymorphic Ventricular Tachycardia with QT Prolongation

The QT interval in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) is typically normal. However, CPVT patients are sometimes misdiagnosed as concealed long QT syndrome (LQTS), because patients with LQTS also manifest with syncope or sudden death following periods of exertion or extreme emotion. We report a CPVT patient with a pathogenic RyR2 mutation associated with a marked QT prolongation, which normalized after flecainide therapy.     

Regulatory mechanisms of C4b‐binding protein (C4BP)α and β expression in rat hepatocytes by lipopolysaccharide and interleukin‐6

Summary. Background: C4b‐binding protein (C4BP), a multimeric protein structurally composed of α chains (C4BPα) and a β chain (C4BPβ), regulates the anticoagulant activity of protein S (PS). Patients with sepsis have increased levels of plasma C4BP, which appears to be induced by interleukin (IL)‐6. However, it is not fully understood how lipopolysaccharide (LPS) and IL‐6 affect the plasma C4BP antigen level and C4BPα and C4BPβ expression in hepatocytes. Objectives: To assess the effect of LPS and IL‐6 on plasma C4BP, PS–C4BP complex levels, PS activity, and C4BP expression by rat liver in vivo and on C4BP expression by isolated rat hepatocytes in vitro. Results: Plasma C4BP antigen level transiently decreased from 2 to 12 h after LPS (2 mg kg^?1) injection, and then it abruptly increased up to 24 h after LPS injection. Plasma C4BP antigen level increased until 8 h after IL‐6 (10 μg kg^?1) injection, and then gradually decreased up to 24 h after IL‐6 injection. LPS significantly decreased the protein and mRNA expression of both C4BPα and C4BPβ in rat hepatocytes, and this effect was inhibited by NFκB and MEK/ERK inhibitors. IL‐6 mediated increase in C4BPβ expression in rat hepatocytes, which leads to increased plasma PS–C4BP complex level and to decreased plasma PS activity, was inhibited by inhibition of STAT‐3. Conclusion: LPS decreases both C4BPα and C4BPβ expression via the NFκB and MEK/ERK pathways, whereas IL‐6 specifically increases C4BPβ expression via the STAT‐3 pathway, causing an increase in plasma PS–C4BP complex, and thus decreasing the anticoagulant activity of PS.     

Treatment of massive bleeding with prothrombin complex concentrate: argument for

See also Godier A, Susen S, Samama C‐M. Treatment of massive bleeding with prothrombin complex concentrate: argument against. This issue, pp 2592–5.