Clinical characteristics of thrombotic microangiopathy following ABO incompatible living donor liver transplantation.

Thrombotic microangiopathy (TMA) may develop after living donor liver transplantation (LDLT), but the mechanism is not fully understood. We retrospectively analyzed all patients undergoing LDLT at our center, including TMA patients, to elucidate the clinical characteristics and presentation and to determine which patients have a higher risk of occurrence of TMA. In all, 57 adult patients were reviewed after LDLT at our institution. TMA was diagnosed by sudden and severe thrombocytopenia, followed by hemolytic anemia with fractionated erythrocytes in the blood smear. Clinical features were compared between the TMA group and the non-TMA group. Of the 57 patients, 4 were diagnosed with posttransplantation TMA. ABO blood group (ABO)-incompatibility, cyclophosphamide (CPA), and recipient blood group (type O) were closely correlated with the occurrence of TMA. Thrombocytopenia appeared 1 to 5 days before hemolytic anemia. Coagulative function markers stayed at the same level after TMA, while marked elevation was shown in fibrinolytic function markers such as plasminogen activator inhibitor type 1 (PAI-1). TMA occurred at a higher prevalence in ABO-incompatible graft recipients. Additional factors associated with ABO-incompatible transplantation, such as an overdose of immunosuppressants, may affect the likelihood of TMA. Sudden and severe thrombocytopenia presented before hemolytic anemia and the serum levels of PAI-1 correlated well with the clinical course of TMA. In conclusion, early recognition of thrombocytopenia and elevation of PAI-1 is crucial to diagnose TMA especially in ABO-incompatible LDLT.     

The Japanese experience with metabolic imaging in the clinical setting

Conclusions  Reduced utilization of fatty acids at rest often is observed in severely ischemic myocardium and possibly postischemic myocardium despite normal perfusion at rest. The role of metabolic imaging in identifying postischemic insult as ischemic memory imaging has been the focus of recent investigations. A number of reports from Japan show quite acceptable diagnostic accuracy of BMIPP imaging for detecting coronary patients without prior MI. In addition, recent data indicate that BMIPP imaging has prognostic value when applied in patients with documented or suspected coronary disease. The major advantage of BMIPP imaging is to demonstrate ischemic myocardium as an area of altered metabolism at rest. Thus this study is of clinical importance for elderly patients or for patients not suitable for a stress study. Furthermore, this radiopharmaceutical tracer may hold promise in demonstrating early alteration of energy metabolism in a variety of myocardial disorders.     

The importance of doxorubicin and methotrexate dose intensity in the chemotherapy of osteosarcoma

The relationship between dose intensity of cytotoxic agents and therapeutic results was examined in a retrospective analysis of 32 patients with non-metastatic high-grade osteosarcoma of the extremities. The average dose intensities of individual agents were 9.8 mg/m²/week for doxorubicin, 1.2 g/m²/week for methotrexate, and 10.5 mg/m²/week for cisplatinum. The dose intensities of doxorubicin and methotrexate were significantly correlated with the clinical results, while that of cisplatinum was not. These results indicate that maximal dose intensification of doxorubicin and methotrexate is an important determinant of treatment outcome for patients with osteosarcoma.     

Immunohistochemical and electromicroscopic study of a case of primary nodular pulmonary amyloidosis]

A 41-year-old man was hospitalized for further evaluation of an abnormal chest films which revealed a nodular shadow in the right middle lung field, which remarkably enlarged during one year. As no diagnostic procedures ruled out lung carcinoma of right S6, a right lower lobectomy was performed. Pathologically the nodules were composed of amorphous and eosionphilic materials which were diagnosed as amyloid by Congo-red stain and electron microscopic examination. Since there were no deposits in other organs and there was no abnormality of serologic and urinary protein analysis, a diagnosis of primary nodular pulmonary amyloidosis was established.     

Prognostication of sequelae in Bell's palsy

Four symptoms of sequelae (abnormal synkinesis, facial spasm, contracture, and crocodile's tear) which persisted or appeared after 6 months from onset were examined with 351 patients with Bell's palsy. The incidence of sequelae were 2% in the group having score (May-Hosomi's modified method, 90 points method) over 40 points within 1 months after onset. Other hand, the incidence of sequelae were 15% in the group having score under 40 points at 1 weeks from onset, 30% in the group having score under 40 points at 2 weeks, 57% in the group having score under 40 points at 3 weeks, and 76% in the group having score under 40 points at 1 month. From these findings, it was seen that the prognostication with a high accuracy was possible by the score.     

Occipital horn syndrome: report of a patient and review of the literature

We report an 18-year-old boy with occipital horn syndrome and we review the 20 cases previously published with this syndrome. The distinctive features common to all patients were unusual facial appearance, skeletal abnormalities, chronic diarrhea and genitourinary abnormalities. The skeletal abnormalities included occipital horns, short, broad clavicles, deformed radii, ulnae, and humeri, narrowing of the rib cage, undercalci-fied long bones with thin cortical walls and coxa valga. Occipital horn syndrome is inherited in an X-linked recessive fashion. Our analysis indicates that occipital horn syndrome is associated with a recognizable characteristic phenotype.     

Measurement of quality of life in rheumatoid arthritis]

This study was designed to explore the health status or quality of life (QOL) in 366 patients with rheumatoid arthritis in Japan. Physical, social, and emotional functions of the patients, namely the QOL, were measured by the modified health assessment questionnaire, the quality of well-being score, and the face scale, respectively. These functions were also evaluated by the new methods using visual analogue scales. The longer the duration of rheumatoid arthritis, the worse the QOL measures in these patients. A similar result was observed in the relationship between the stage classification of progression of rheumatoid arthritis and the QOL measures. In contrast, the traditional medical process measures, such as Lansbury activity index, sedimentation rate, and serum CRP concentration did not correlate with the duration of the disease. We conclude that the QOL measures in this study are useful for evaluation of the functional status and well-being of patients with rheumatoid arthritis. However, the clinical usefulness of these measures for evaluation of effectiveness and/or side effects of anti-rheumatic drugs still remains unknown.     

Effect of Ebrotidine on Gastric Mucosal Calcium Channel Activity

Ebrotidine is a new H2-receptor antagonist also known for its gastroprotective effect against ethanol-induced mucosal injury. In this study, we investigated the effect of ebrotidine on the activity of the gastric mucosal calcium channels. The channel complex was isolated from the solubilized gastric epithelial cell membranes by affinity chromatography on wheat germ agglutinin. After being labeled with [³H]PN200–110, the complex was reconstituted into phosphatidylcholine vesicles which exhibited active ⁴⁵Ca²⁺ uptake into intravesicular space and responded in a concentration-dependent manner to calcium channel activator, BAY K8644, as well as to calcium channel antagonist, PN200–110. The ⁴⁵Ca²⁺ uptake was inhibited by ebrotidine. Maximum inhibitory effect was attained at 50 μg/ml ebrotidine, at which point a 54.9% decrease in uptake occurred. The gastric mucosal calcium channels, on epidermal growth factor binding (EGF) in the presence of ATP, responded by an increase in tyrosine phosphorylation of 55 and 170 kDa proteins, and the vesicles containing the phosphorylated channels displayed a 48% greater ⁴⁵Ca²⁺ uptake. This phosphorylation process was inhibited by ebrotidine. Furthermore, ebrotidine also interfered with the binding of EGF to calcium channel protein. The results point toward the importance of KGF in the maintenance of gastric mucosal calcium homeostasis, and suggest that ebrotidine has the ability to protect the cellular integrity from calcium imbalance by modulating the ECF-stimulated gastric mucosal calcium channel phosphorylation.     

An autopsy case of deep cerebral venous thrombosis: serial CT, MRI and pathological findings]

A 17-year-old girl developed vomiting of sudden onset, followed by a state of confusion that progressed rapidly to coma within one day. Laboratory tests indicated iron deficiency anemia and reactive thrombocytosis, but there was no evidence of coagulopathy. There was no history of medication including the contraceptive pill, either. Emergency CT scan without contrast enhancement showed increased density along the course of the vein of Galen and internal cerebral veins. A repeated CT scan without contrast enhancement carried out 24 hours after the onset of the illness confirmed extensive bilateral hypodensity of the thalami, basal ganglia and adjacent white matter. There was also a prominent spontaneous increase in the density of the deep cerebral venous system. MRI was performed 3 days after the onset of the illness, which showed absence of a flow void in the region of the internal cerebral veins and septal veins on T1-weighed images. T2-weighted images showed low intensity in these veins. At autopsy, the bilateral internal cerebral veins were occluded by fresh thrombosis and hemorrhagic infarction was seen in the bilateral thalami.     

MR imaging of dural arteriovenous malformations with ocular signs

Summary Four patients with dural arteriovenous malformation (AVMs) draining into the cavernous sinus, who presented ophthalmic manifestations, were studied by magnetic resonance (MR) imaging. In all patients signal decrease in the involved cavernous sinus was demonstrated in coronal spinecho (SE) imaging. It is attributable to rapid venous flow in the sinus, and this high velocity signal loss is a fairly pathognomonic finding in this condition. We stress the validity of MR imaging in the primary diagnosis of dural AVMs with ophthalmic symptoms.