A case report: Severe bone marrow suppression caused by 6-mercaptopurin in Crohn's disease patient]

A 23-year-old man was admitted for treatment of acute exacerbation of ileitis and perianal abscess caused by Crohn's disease. After incision and drainage of the abscess, coupled with antibiotic therapy, 6-mercaptopurine (6-MP) was commenced. His white blood cell (WBC) count on day 12 after initiation of 6-MP was not decreased. However, on day 24 he was re-admitted because of severe myelosuppression (WBC: 300/microl), which was complicated by the recurrence of the perianal abscess. Myelosuppression was prolonged and required the administration of granulocyte colony stimulating factor (G-CSF). G-CSF was continued for 17 days to achieve recovery of his WBC count to a normal level.     

Case of severe ulcerative colitis successfully treated with intravenous cyclosporine without high dose corticosteroid]

A 44-year-old women developed marked myopathy one year earlier, when she was treated with intravenous prednisolone for acute severe exacerbation of ulcerative colitis. When she was admitted to our hospital for another severe exacerbation, intravenous cyclosporine A was administered as monotherapy because she could not tolerate corticosteroid. The treatment was successful and she obtained complete remission. Cyclosporine A monotherapy is considered to be a valuable alternative to proctocolectomy for severe ulcerative colitis patients who cannot tolerate corticosteroid.     

Aortic valve replacement with fresh or cryopreserved aortic allograft--initial experience in Japan]

Aortic allograft valves were harvested from non-infected (bacterial or viral) cadavers within 24 hours of death with a family consent, and were sterilized by 4 degrees C antibiotic solution for 48 hours. Then, the allograft was preserved in the 4 degrees C nutrient medium (fresh; TC-199, calf serum and HEPES buffer) or in liquid nitrogen (-196 degrees C) after freezing to -80 degrees C by a programmed freezer. 10% dimethylsulfoxide (DMSO) was used for cryopreservation. Following germ-free confirmation, aortic allograft valves were implanted in 5 patients having aortic regurgitation with good results. Three fresh and two cryopreserved allograft valves were used. Although the follow-up term is very short (maximum 1 year) at the present time, the valve function is quite satisfactory, confirmed by cardiac catheterization and echocardiography. This is the first report in Japan with regard to cryopreservation of allograft valves and clinical use of fresh or cryopreserved valves. We believe that realization and progress of allograft preservation by cryo-technique and establishment of the tissue bank are important for the development of cardiovascular surgery in Japan.     

Bilateral internal mammary artery grafts for coronary artery bypass operations in children

We performed myocardial revascularization with bilateral internal mammary arteries in eight children for coronary artery complications consequent to Kawasaki disease. Subjects included seven boys and one girl, ranging in age from 3 to 13 years (mean age, 8.3 +/- 3.4 years). The body surface area ranged from 0.65 to 1.65 m2 (average, 1.08 +/- 0.35 m2). Three patients had a previous myocardial infarction. The right internal mammary artery was anastomosed to the right coronary artery and the left internal mammary artery was sutured to the left anterior descending artery in all patients. The patients received an average of 2.4 grafts. Magnifying loupes of 3.5 X were used for anastomosis with 8-0 monofilament polypropylene sutures. Subjects were followed up from 12 to 38 months (23 +/- 10.8 months) after operation. All were doing well with no recurrence of angina, and body development was normal, including the sternum and thorax according to chest x-ray films and computed tomography of the chest. Patency of the bilateral internal mammary arteries was 100% in the early (within 1 month) postoperative period and remained so in the late (over 1 year) postoperative period. Anastomotic junctions between the internal mammary artery and the coronary artery developed well angiographically in the late postoperative period. The internal mammary artery is the graft of choice for pediatric myocardial revascularization because of its excellent long-term patency and growth potential. Bilateral internal mammary arteries should be used whenever indicated, and the use of bilateral internal mammary arteries did not adversely influence chest wall development in the children.     

Clinical experiences with a new membrane oxygenator with low priming volume (D702 MASTER FLO 51), studies during pulsatile and constant flow perfusion

The newly developed oxygenator "D702" is a compact hollow fiber membrane oxygenator with a priming volume of 170 ml. The maximum flow allowance is 4 liters per/minute. We used this oxygenator in 16 patients (11 infants and children, and 5 adults) undergoing various open heart surgery, and function of this oxygenator was studied. Pulsatile cardiopulmonary bypass was performed in 8 patients and nonpulsatile constant flow perfusion was employed in the remaining 8 patients. Our clinical experience showed excellent maintenance of PaO2 and PaCO2 during both pulsatile and constant flow bypasses. A low pressure drop was encountered across the membrane oxygenator, and therefore, this oxygenator is applicable for pulsatile cardiopulmonary bypass. The D702 is a very useful and applicable for a wide range of patients from infants to adults with a small body structure.     

Polyglutamine aggregates stimulate ER stress signals and caspase-12 activation

Accumulation of unfolded and malfolded proteins causes endoplasmic reticulum (ER) stress, stimulating unfolded protein response (UPR) and c-Jun N-terminal kinase (JNK) activation and activating caspase-12 located on the ER. Little is known about the relationship between the ER stress and polyglutamine [poly(Q)] aggregates. Poly(Q)72 repeats [poly(Q)(72)] induced the stimulation of ER stress signals such as JNK activation, upregulation of Grp78/Bip and caspase-12 activation in C2C5 cells. We prepared antiserum against the cleavage site of mouse caspase-12 at D(318) (anti-m12D318), and showed that poly(Q)(72) with perinuclear aggregates, cytoplasmic inclusions and nuclear inclusions stimulated JNK activation and anti-m12D318 immunoreactivity, but poly(Q)(72) with dispersed aggregates and small nuclear aggregates showed a significantly less effect. Poly(Q)(72) and poly(Q)(11) dispersed in cytoplasm did not. Anti-m12D318-positive cells showed apoptotic features. Unlike anti-m8D387 immunoreactivity, the anti-m12D318 immunoreactivity was not coaggregated with poly(Q). Ac-IETD-fmk (caspase-8 inhibitor) and Ac-DEVD-CHO (caspase-3 inhibitor) did not prevent the anti-m12D318 immunoreactivity induced by poly(Q)(72) aggregates. Anti-m12D318 immunoreactivity was detected in caspase-8(-/-) and caspase-3(-/-) mouse embryonic fibroblasts expressing poly(Q)(72) aggregates. Thus, caspase-12 was activated by poly(Q)(72) aggregates via a pathway independent of caspase-8 and caspase-3 activation, and caspase-12 activation was closely associated with poly(Q) aggregate-mediated cell death. Stimulation of ER stress signals may be involved in the pathogenesis of neurodegenerative disorders with poly(Q) expansion.     

Clinical features of oral allergy syndrome to plant foods allergens in Kanto regions]

BACKGROUND: Recently, it is recognized that the patients of oral allergy syndrome (OAS) to fruits are increasing. However, there are little knowledges of the background, character, and seriousness about these patients in Kanto regions. OBJECTIVE: We aimed to investigate the clinical features of OAS patients to plant origin foods in Kanto regions. METHODS: The patient, who visited Sagamihara National Hospital from 2000 to 2005 and developed some allergic symptoms to plant origin foods, were studied by a questionary survey. RESULTS: As for the 42 subjects, average age are 36 years old, male:female=8:34, merger of other allergic disease is 35 allergic rhinitis of 42 subjects (83%), 34 of asthma (81%), 14 of atopic dermatitis (33%). The causes of OAS symptoms are 32 rose-family fruits, 34 non-rose family fruits, 14 vegetables, 11 nuts, 2 grains subjects. As for the symptom, only in the oral and pharynx symptoms are found in 12, the systemic symptoms is 29, anaphylaxis is 11 subjects. Allergic rhinitis preceded on the 90% subjects with pollinosis, very high rate. On the other hand, the 20% of all subjects have no symptoms of allergic rhinitis. CONCLUSION: A nasal catarrh symptoms went ahead in most of the OAS subjects in Kanto regions. In addition, considering from some patients have no black alder pollinosis and/or are allergic to many non-rose-family fruits at high frequency, there might be a broad cross-reactivity between many pollens other than alder and plant origin foods.     

Extrarenal Wilms' tumor in the adult patient. A case report and review of the world literature

A case of an extrarenal Wilms' tumor arising in the retroperitoneal region of a 49-year-old male is reported. A review of the world literature indicates that the incidence of the tumor arising in the extrarenal region is extremely rare. A total of 14 cases have previously been reported, but the number of cases that occurred in adult patients is only 2. The clinical and pathologic features are briefly discussed.     

Detection of numerical alterations of chromosome 1 in cytopathological specimens of breast tumors by chromogen in situ hybridization

To investigate the effectiveness of chromogen in situ hybridization (CISH) in the diagnosis of breast tumors, numerical alterations of chromosome 1 were examined by CISH and fluorescence in situ hybridization (FISH) methods, and the presence of der(16)t(1;16) was also examined by FISH in imprinted cytology specimens from resected tissues of 14 carcinomas and five non-malignant lesions. The modal signal counts of chromosome 1 were compared between the specimens processed by CISH and FISH for each case. Aneusomies of the long arm of chromosome 1 were detected in 10 (71%) carcinomas as the major clones by both methods. In addition, one atypical papilloma demonstrated tetrasomy of 1q12 as a major clone by CISH, but such a clone was at first overlooked by FISH. Four other benign lesions showed disomic 1q12 signals as a major clone by both CISH and FISH. As additional information from FISH, eight cancers showed structural or numerical alterations of chromosome 16, and four showed der(16)t(1;16). In total, 10 carcinomas showed chromosome 16 alterations, and all of these overlapped with the carcinomas with 1q12 aneusomies. The CISH method provided almost the same results as the FISH method, and both methods were considered applicable in supportive diagnosis of cytological specimens of breast tumors. In addition, the CISH method was superior in the detection of numerical alterations in carcinoma cells by referring to the morphology of cells and in the detection of significant clones which might be missed under dark-field microscopy.