BACKGROUND/AIMS: Percutaneous procedures for treating patients with hepatolithiasis associated with intrahepatic biliary stricture (IHBS) have been shown to have a relatively lower rate of successful stone removal than without IHBS. The reason is tight stenosis or acute angulation of intrahepatic bile ducts (IHDs). We suggest that a bilateral approach to IHDs would improve the success rate of stone removal in patients with complicated IHBS. METHODS: Conventional cholangioscopic electrohydrolithotripsy (EHL) was performed in 82 patients without IHBS. Percutaneous transhepatic stricture dilation and cholangioscopic EHL through unilateral access was performed to treat 41 patients with hepatolithiasis with IHBS. In 21 patients with complicated stricture and multiple stones, removal of hepatolithiasis was achieved by a bilateral approach. The rate of complete stone clearance and complication was reviewed retrospectively. RESULTS: Complete stone clearance was achieved in 92.7% (76/82) of patients using a unilateral approach without IHBS, in 61.0% (25/41) of cases using a unilateral approach with IHBS, and in 85.7% (18/21) of cases using a bilateral approach with IHBS (p<0.05). The number of sessions of PTCS was 2.7+/-0.3, 3.9+/-0.3, and 2.7+/-0.5 respectively. The overall complication rate was 14.6% (21/144), and didn't differ between groups. CONCLUSIONS: A bilateral approach to IHDs may be a useful alternative treatment in unresectable patients with multiple hepatolithiasis associated with complicated IHBS.
Bile Ducts, Intrahepatic ; Constriction, Pathologic ; Factor IX ; Humans ; Retrospective Studies

Percutaneous transhepatic cholangioscopy (PTCS) is the primary treatment option for general cases of intrahepatic duct stones. However, there are no reports on the use of PTCS for intrahepatic duct stones in patients who had undergone living donor liver transplantation (LDLT). We experienced two cases of successful intrahepatic stone removal by the use of PTCS in LDLT patients. With these cases, we have confirmed that PTCS management can be safely performed not only for a general bile duct stone, but also for a bile duct stone that develops in a patient that had previously undergone liver transplantation.
Bile Ducts ; Factor IX ; Humans ; Liver ; Liver Transplantation ; Living Donors

BACKGROUND: To prevent sudden unexpected movement of patients during surgery, muscle relaxants are used to maintain intense neuromuscular blockade.They are administered by intermittent bolus or continuous infusion.Rocuronium is often used for continuous infusion because it is known to lack cumulative effects. The purpose of this study was to compare recovery times from intense neuromuscular block to reappearance of muscle twitches after 0.1 Hz single twitch stimulation. MATERIALS AND METHODS: Seventy five patients were randomized to one of 3 groups.Patients in group 1 were administered a single bolus of rocuronium; groups 2 and 3 were given continuous infusion of rocuronium for 1 and 2 h, respectively.During anesthesia, neuromuscular blockade was monitored by TOF-watch(R) and regulated so as not to exceed more than 5 (PTC) during the infusion. After infusion, PTC was counted every 5 min until single twitch heights had reappeared and the time till the twitch reappeared was calculated. RESULTS: There was a good correlation between the time it took to observe a PTC and the first response of TOF time in each group.In a parallelism test, there were no significant differences. There were also no significant differences in recovery times from PTC to the reappearance of a single twitch between groups. CONCLUSIONS: There are no significant differences in recovery times-from deep neuromuscular blockade to reappearance of single twitch-regardless of the infusion time.When a PTC occurs during deep neuromuscular blockade, it may predict the remaining time of reappearance of a single twitch response.
Androstanols ; Anesthesia ; Factor IX ; Humans ; Muscles ; Neuromuscular Blockade

Factor IX ; genetics ; Hemophilia B ; genetics ; Humans ; Male ; Mutation

Cerebral venous thrombosis (CVT) rarely recurs, and the factors associated with a recurrence remain unclear. Recently, however, elevated plasma coagulation factor VIII has been considered a factor related to recurrent venous thromboembolism. Here we report a patient who had recurrent CVT associated with significantly elevated levels of factor VIII despite the chronic use of an antiplatelet agent. Factor IX was also elevated in this patient. These findings suggest that elevated factor VIII is a factor underlying the recurrence of CVT, and that prolonged anticoagulation therapy may have to be considered in patients with elevated coagulation factor levels.
Blood Coagulation Factors ; Factor IX ; Factor VIII* ; Humans ; Plasma ; Recurrence ; Venous Thromboembolism ; Venous Thrombosis*

The study was to explore the change of coagulation factor VIII and IX activities in the platelet suspension collected by platelet apheresis during storage at 22 degrees C. 18 samples of platelet concentrates were collected by the cs-3000 plus and stored at 22 degrees C and then FVIII: C, FIX: C activities were detected at 0, 12, 24, 48, 72, 96, 120 hours respectively by SYSMEX CA-1500. The results showed that FVIII: C activity was (100.51 + 44.02)% at 0 hour, and then decreased dramatically to 10% - 40% of primary level from 12 to 120 hours, while FIX: C activity was (120.93 +/- 20.50)% at 0 hour and decreased to 10% - 35% of primary level from 24 to 120 hours. In conclusion, FVIII and FIX in the platelet concentrates stored at 22 degrees C could keep their biological activities at physiologically high levels.
Blood Platelets ; Blood Preservation ; methods ; Factor IX ; metabolism ; Factor VIII ; metabolism ; Humans ; Platelet Transfusion ; Plateletpheresis ; methods

Among the patients with hemophilia, 10~15% have hemophilia B, and 1~3% of the hemophilia B patients develop inhibitor to factor IX clotting activity. Allergic reactions to concentrates containing factor IX (FIX) are serious complications during the treatment of hemophilia B patients with inhibitor. Although treatment with recombinant activated factor VII (FVIIa) is generally recommended in these patients, it is limited by the agent's short half-life, a lack of experience with its use in this manner and the prohibitive cost. We report here on a case of a 9-year-old boy with severe hemophilia B with inhibitor and he had a history of anaphylaxis to FIX. The patient was successfully treated with a desensitization protocol with escalating doses of FIX in addition to administering premedications.
Anaphylaxis ; Child ; Factor IX ; Factor VIIa ; Half-Life ; Hemophilia A ; Hemophilia B ; Humans ; Hypersensitivity

BACKGROUND: Korean National Health Insurance reimburses factor VIII (FVIII) and factor IX (FIX) clotting factor concentrate (CFC) infusions to discrepant activity levels, allowing elevation of FVIII activity to 60 IU/dL and FIX to 40 IU/dL. We aimed to assess hemostatic response to these target levels using global hemostatic assays. METHODS: We enrolled 34 normal healthy men, 34 patients with hemophilia A, and 36 with hemophilia B, with residual factor activity of 3 IU/dL or less and without inhibitors. Patients with hemophilia A and B received injected CFCs according to reimbursement guidelines. Fifteen minutes after injection, we assessed hemostatic response with global hemostatic assays: thrombin generation assay (TGA), thromboelastography (TEG), and clot waveform analysis (CWA). RESULTS: Normal healthy men and patients with hemophilia A and B were 36.7, 37.2, and 35.1 years old, respectively. FVIII and recombinant FIX concentrate doses were 28.8 IU/kg and 43.6 IU/kg. Post-infusion FVIII activity rose from 0.5 IU/dL to 69.4 IU/dL, while FIX activity rose from 1.4 IU/dL to 46.8 IU/dL. Post-infusion peak thrombin concentrations in hemophilia A and B were 116.6 nM/L and 76.4 nM/L (P < 0.001). Post-infusion endogenous thrombin potential (ETP) in hemophilia A and B was 1349.8 nM/min and 915.6 nM (P < 0.001). TEG index of hemophilia A and B was 0.11 and −0.51 (P=0.006). CONCLUSION: Current reimbursed doses for FIX concentrates are insufficient to achieve hemostatic responses comparable to those after reimbursed doses for FVIII concentrates in terms of peak thrombin concentration, ETP, and TEG index.
Factor IX* ; Factor VIII* ; Hemophilia A ; Hemophilia B ; Humans ; Male ; National Health Programs ; Thrombelastography ; Thrombin

BACKGROUND: Risk factors for the development of inhibitors in previously untreated patients (PUPs) have been reported; this is not the case in previously treated patients (PTPs) owing to fewer studies. Risk factors may differ for the development of PTP versus PUP inhibitors. We aimed to identify risk factors for PTP inhibitor development. METHODS: Participants were patients at a hemophilia treatment center in Korea with current or past history of factor VIII or factor IX alloantibodies. Observed inhibitors were classified as PUP or PTP inhibitors based on the cumulative number of exposure days. We compared the type and severity of hemophilia, mutation type, and family history of inhibitor between PUPs and PTPs. Events within 3 months before the first inhibitor detection, such as change of the factor concentrate used, short-term high exposure or continuous infusion of factor concentrate, history of surgery, infection, diagnosis of cancer, use of immunosuppressive or immunomodulator agents, and vaccination were compared between PUPs and PTPs. RESULTS: We observed 5 PUP inhibitors and 5 PTP inhibitors in 115 patients with hemophilia A. Events that might be related to the development of inhibitors within 3 months prior to the first inhibitor detection were observed in all 5 PTPs. On the contrary, no such events were observed in any PUPs. The observed events included a change in the factor concentrate used, subsequent chemotherapy, and short-term high exposure to factor concentrates for controlling hemorrhage and surgeries. CONCLUSION: Our results suggest a greater role of nongenetic factors in PTP inhibitor development.
Diagnosis ; Drug Therapy ; Factor IX ; Factor VIII ; Hemophilia A ; Hemorrhage ; Humans ; Isoantibodies ; Korea ; Prevalence ; Risk Factors ; Vaccination

Hemophilia, a genetically determined disorder, can be divided into hemophilia A and hemophilia B. Hemophilia A, defined as a sex-linked recessive trait hemorrhagic disease of males characterized by a deficiency of factor VIII occurs about seven times more frequently than hemophilia B which is characterized by a deficiency of factor IX. The complications resulting from hemophilia occurs in virtually every system. This paper is concerned with a rare complication of hemophilia, iliacus hematoma with femoral neuropathy. On case of spontaneous hemorrhage of iliacus muscle with femoral neuropathy in hemophilia was decompressed through a small incision with sump drainage under cover of AHF(Anti-hemophilic factor) concentrated plasma cryoperecipitate followed AHF replacement therapy.
Drainage ; Factor IX ; Factor VIII ; Femoral Neuropathy ; Hematoma ; Hemophilia A ; Hemophilia B ; Hemorrhage ; Humans ; Male ; Plasma