Thrombophilic dysfibrinogen Tokyo V with the amino acid substitution of gammaAla327Thr: formation of fragile but fibrinolysis-resistant fibrin clots and its relevance to arterial thromboembolism

Thrombophilic dysfibrinogen Tokyo V was identified in a 43-year-old man with recurrent thromboembolism. Based on analyses of the patient fibrinogen genes, the amino acid sequence of the aberrant fibrinogen peptide, and deglycosylation experiments, fibrinogen Tokyo V was shown to have an amino acid substitution of gamma Ala327Thr and possibly extra glycosylation at gamma Asn325 because the mutation confers the N-linked glycosylation consensus sequence Asn-X-Thr. The mutation resulted in impaired function and hypofibrinogenemia (hypodysfibrinogen). Polymerization of fibrin monomers derived from patient fibrinogen was severely impaired with a partial correction in the presence of calcium, resulting in very low clottability. Additionally, a large amount of soluble cross-linked fibrin was formed upon thrombin treatment in the presence of factor XIII and calcium. However, Tokyo V-derived fibrin was resistant to degradation by tissue plasminogen activator (tPA)-catalyzed plasmin digestion. The structure of Tokyo V fibrin appeared severely perturbed, since there are large pores inside the tangled fibrin networks and fiber ends at the boundaries. Taken together, these data suggest that Tokyo V fibrin clots are fragile, so that fibrinolysis-resistant insoluble fibrin and soluble fibrin polymers may be released to the circulation, partly accounting for the recurrent embolic episodes in the patient.     

A case of liver abscess co-infected with Desulfovibrio desulfuricans and Escherichia coli and review of the literature

A 73-year-old woman was admitted with consciousness disturbance following a fever. Abdominal computed tomography revealed a large liver abscess with which the presence of Desulfovibrio desulfuricans and Escherichia coli was confirmed by thorough blood and abscess content culture. Empiric meropenem treatment was switched to cefoperazone/sulbactam, followed by ampicillin/sulbactam based on susceptibility testing. Desulfovibrio desulfuricans is a common bacterium that rarely causes liver abscess and may be overlooked during co-infection due to overgrowth of the accompanying bacteria. Clinicians should bear Desulfovibrio desulfuricans in mind and select the appropriate antibiotics according to susceptibility testing when anaerobic bacteria are detected in a liver abscess.     

Type 1 Autoimmune Pancreatitis Extending along the Main Pancreatic Duct: IgG4-related Pancreatic Periductitis

We herein report a unique form of autoimmune pancreatitis (AIP) spreading along the main pancreatic duct (MPD). A 70-year-old man was referred for a small lesion at the pancreatic neck, accompanying an adjacent cyst and dilated upstream MPD. Four years earlier, health checkup images had shown a pancreatic cyst but no mass lesion. Endoscopic ultrasonography showed a contrast-enhanced, tumorous lesion, mainly occupying the MPD. With a preoperative diagnosis of ductal neoplasms mainly spreading in the MPD, Whipple''s resection was performed. The resected specimens showed MPD periductitis with IgG4-related pathology, indicating type 1 AIP. Clinicians should practice caution concerning the various AIP forms.     

Early Detection of High-grade Biliary Intraepithelial Neoplasia (BilIN-3) in the Cystic Duct Visualized by SpyGlass DS Cholangioscopy

An 84-year-old man was admitted with epigastralgia. Computed tomography showed contrast-enhanced wall thickness in the cystic duct. An endoscopic examination revealed short irregular stricture in the cystic duct, and per-oral cholangioscopy revealed a reddish papillary tumor at the stricture site. Surgical resection revealed high-grade biliary intraepithelial neoplasia (BilIN) at the stricture site of the cystic duct. To our knowledge, this is the first case of a solitary high-grade BilIN epithelium in the cystic duct detected by per-oral cholangioscopy.     

Time in range correlates glycated albumin measured immediately after 2 weeks of continuous glucose monitoring

AimsTime in range (TIR), an index of glycemic control and also blood glucose fluctuation, obtained from continuous glucose monitoring (CGM), has been increasing its importance along with the spread of CGM in recent years. For a while, glycated albumin (GA) has been also used as a glycemic control index during about 2-weeks in routine clinical practice. It has not yet been confirmed under optimal condition whether TIR and GA correlates. Clarification of the correlation between TIR and GA, which was measured immediately after 2-weeks of CGM, might be a finding that further supports the utility of TIR.MethodsGA was measured at the conclusion of 2-week CGM in 71 diabetes outpatients at our hospital, and the correlation between GA and indices such as TIR obtained from CGM was statistically analyzed.ResultsIt was found that TIR and time above range (TAR) were significantly correlated with GA. Upon performing multiple regression analysis, TIR, TAR and BMI. indicated a significant regression coefficient with respect to GA.ConclusionsThese findings further support the utility of TIR as a marker of glycemic control that it might also be correlated with GA, and also suggest a relation between GA and blood glucose fluctuation.     

Comparison of ticlopidine and cilostazol for the prevention of restenosis after percutaneous transluminal coronary angioplasty

Prevention of restenosis after percutaneous transluminal coronary angioplasty (PTCA) continues to be a significant problem. Recent controlled studies have demonstrated that cilostazol suppresses restenosis after PTCA. The effects of ticlopidine, another antiplatelet agent, were compared in terms of outcomes of patients randomized for treatment with the two drugs after PTCA. A total of 35 patients (47 lesions) were assigned prospectively and randomly to ticlopidine (17 patients, 24 lesions) and cilostazol (18 patients, 23 lesions) groups. Minimal luminal diameter (MLD) and percentage of stenosis to reference diameter were estimated before PTCA, just after the procedure and after 4 months follow-up. All patients underwent 4 months angiographic follow-up, at the end of which MLD was 2.03+/-0.71 mm in the ticlopidine group and 2.05+/-0.68 mm in the cilostazol group (p = 0.95), and the percentage of stenosis to reference diameter was 31.4+/-16.7% and 30.0+/-17.0%, respectively (p = 0.78). The restenosis rate was 12.5% in the ticlopidine group and 17.4% in the cilostazol group (p = 0.69), relatively low as compared to the 20% to 30% reported in previous studies. Adverse drug reactions during the follow-up period were observed in two of the ticlopidine group and none of the cilostazol group. We conclude that both ticlopidine and cilostazol are effective for the prevention of restenosis after PTCA, however the former may be associated with slight side effects.     

Stress response to laparoscopic liver resection

BACKGROUND: The magnitude of the systemic response is proportional to the degree of surgical trauma. Much has been reported in the literature comparing metabolic and immune responses, analgesia use, or length of hospital stay between laparoscopic and open procedures. In particular, metabolic and immune responses are represented by measuring various chemical mediators as stress responses. Laparoscopic procedures are associated with reduced operative trauma compared with open procedures, resulting in lower systemic response. As a result, laparoscopic procedures are now well accepted for both benign and malignant processes. Laparoscopic liver resection, specifically, is employed for symptomatic and some malignant tumors, following improvements in diagnostic accuracy, laparoscopic devices, and techniques. However, laparoscopic liver resection is still controversial in malignant disease because of complex anatomy, the technical difficulty of the procedure, and questionable indications. There are few reports describing the stress responses associated with laparoscopic liver resection, even though many studies reviewing stress responses have been performed recently in both humans and animal models comparing laparoscopic to conventional open surgery. Although this review examines stress response after laparoscopic liver resection in both an animal and human clinical model, further controlled randomized studies with additional investigations of immunologic parameters are needed to demonstrate the consequences of either minimally invasive surgery or open procedures on perioperative or postoperative stress responses for laparoscopic liver resection.     

Validity of catheter-tip Doppler technique in assessment of coronary flow velocity and application of spectrum analysis method

Measurement of coronary flow velocity in clinical cases contributes to understanding the pathophysiology of coronary circulation. To determine absolute coronary flow velocity, coronary blood flow was assessed with an end-mounted Doppler catheter (3Fr, 20 MHz), which was combined with a custom-designed fast-Fourier transformation analysis system. In vitro study using model circuit, actual flow velocity (8 to 96 cm/s) was well correlated with that determined by this catheter system (y = 1.01 X +1.5, r = 0.988). In a clinical study of 12 patients with normal coronary arteriograms, the Doppler catheter was positioned at the proximal left anterior descending artery. Clear flow velocity patterns, which consisted of predominant diastolic components and preceding small systolic components, were obtained in all cases. The peak flow velocity was 17 +/- 8 cm/s (mean +/- standard deviation) during systole and 44 +/- 12 cm/s during diastole in this portion. In 5 patients, the great cardiac vein flow, which reflects the left anterior descending artery flow, was simultaneously measured during rapid atrial pacing. During pacing, percent increases in flow velocity were well correlated with those in great cardiac vein flow (y = 0.90 x +6.4, r = 0.935). These results indicate that catheter-tip Doppler technique with fast-Fourier transformation analysis may be useful in quantitatively determining coronary flow velocity in clinical cases.     

Reversible respiratory failure due to rhabdomyolysis associated with cytomegalovirus infection

A 58-year-old woman presented with muscle weakness, whole body myalgia, and dyspnea. On admission, neurological examination showed proximal muscle weakness in the extremities. The weakness gradually extended to the bulbar and respiratory muscles, necessitating an artificial ventilator. Serum CK level was markedly increased (33,774 IU/L; normal <150 IU/L) and myoglobinuria was noted in urinalysis. There was no sign of renal failure. Nerve conduction study was normal, but needle EMG showed myopathic changes in the weak muscles. Serological studies for virus titers showed more than a four-fold increase of cytomegalovirus (CMV) antibody titer during the disease course. The IgM anti-GM2 antibody was also elevated in the acute phase and decreased in the recovery phase. The muscle weakness and respiratory failure gradually improved after intravenous methylprednisolone administration, and the serum CK level was normalized in several days. CMV infection was thought to have played a central role in the rhabdomyolysis, leading to critical but reversible respiratory muscle paralysis.