Use of Doppler ultrasound and 3-dimensional contrast-enhanced MR angiography in the diagnosis and follow-up of post-traumatic high-flow priapism in a child.

We report a 7-year-old boy with post-traumatic arterial priapism. Doppler US could not reliably identify or exclude a fistula. MR angiography did not demonstrate an arteriovenous fistula and the child was treated conservatively. The ideal imaging modality should demonstrate the presence or absence of a clinically significant causative lesion which, in high-flow arterial priapism, may need intervention. Three-dimensional, contrast-enhanced MR angiography appears to fulfil these requirements. On the basis of the non-invasive imaging findings, invasive intervention was avoided in this case with a successful outcome.

An aberrant left subclavian artery aneurysm with right aortic arch: report of a case.

The case of a 41-year-old man who developed an aneurysm in his aberrant left subclavian artery is described. The patient had a right aortic arch. After a successful aortosubclavian artery bypass, symptoms due to brain ischemia disappeared. This is a very rare disease that is sometimes associated with an aortic anomaly, therefore the optimal therapeutic procedure need to be carefully selected, including the operative indications and approach.

Possible prediction of adverse reactions to fluorouracil by the measurement of urinary dihydrothymine and thymine.

Dihydropyrimidine dehydrogenase (DPD) deficiency with a defect of the pyrimidine catabolic pathway has recently become the focus of considerable attention, due to the severe 5-fluorouracil (5-FU) toxicities occurring in DPD deficiency patients. Studies also suggest that 5-FU toxicities could occur in another pyrimidine metabolic disorder, dihydropyrimidinuria (DHPuria). This study shows that urinary dihydrothymine (DHT) and thymine (THY) are useful indexes for detection of DPD deficiency and DHPuria. We measured urinary DHT and THY in 276 Japanese adults to establish reference ranges. When males and females were compared, both DHT and THY levels were found to be significantly higher in females. The reference ranges (mean +/- SD with logarithmic values) for males were found to be 1.56-5.70 micromol/g of creatinine for DHT and 0.40-1.47 micromol/g of creatinine for THY. The reference ranges for females were found to be 1.89-8.33 micromol/g of creatinine for DHT and 0.58-2.30 micromol/g of creatinine for THY. In addition to this study we analyzed a DPD deficiency case and a DHPuria case. In the DPD deficiency case, the THY concentrations of all urine samples were out of the reference range. However, uracil levels in most of the samples were within the normal range. The DHPuria case excreted large amounts of DHT and dihydrouracil, both out of the normal range.

[A case of locally advanced breast cancer successfully treated with intra-arterial infusion chemotherapy of high-dose epirubicin].

A case of locally advanced breast cancer was treated with intra-arterial infusion chemotherapy using high-dose epirubicin. The 1st and 2nd cycle consisted of 210 mg (day 1, 4, 7) and 150 mg (day 1, 4, 7), respectively. After completion of 2 cycles of the regimen, remarkable loco-regional control and improved QOL were obtained. Leucopenia (nadir; 1,000/mm3) was the dose limiting factor, but well-tolerated with supportive therapy. The patient underwent salvage surgery and maintenance intra-venous chemotherapy (epirubicin; 30 mg/3 weeks). At this writing, the patient is enjoying favorable QOL. These findings suggested that this modality was effective for cases with exctensive loco-regional involvement.

[Long-term survival in a case of multiple liver metastasis from postoperative gastric cancer effectively treated by hepatic intraarterial infusion chemotherapy using MMC and pirarubicin].

We experienced a case of multiple liver metastasis from postoperative gastric cancer who showed long-term survival with hepatic arterial infusion chemotherapy (HAI) of MMC and pirarubicin. A catheter was inserted into the hepatic artery, and 4 mg of MMC and 20 mg of pirarubicin were administered through an implantable port catheter every two to four weeks. The total dose of MMC and pirarubicin by the time of this report was 164 mg and 820 mg, respectively. The follow-up CT scan 2 months after the beginning of HAI showed a decrement of the liver tumors. The decrease rate at 12 and 17 months was 50% and 70%, respectively, which was diagnosed as partial response (PR). The therapeutic effect at 49 months is still PR without any sign of tumor enlargement of extra hepatic lesion.

[Epstein-Barr virus-infected T-cell malignancy in an adult patient with Behçet's disease-like symptoms].

A 20-year-old woman was hospitalized on November 11, 1994 with Behçet's disease-like symptoms (fever, genital ulcer and aphtha in the oral cavity). Bilateral cervical lymph node swelling was also noted and diagnosed as lymphadenitis on biopsy. Chronic active Epstein-Barr virus infection (CAEBV) was diagnosed based on the high titer of antibodies to the EBV capsid antigen, early antigen, and nuclear antigen. She was treated with prednisolone and acyclovir and all symptoms improved. However, ten months after onset of symptoms, T-cell malignancy was diagnosed on bone marrow aspiration, which revealed 34.9% blast cells that had rearrangement of TCR-beta. She died on May 8, 1995, despite anticancer therapy. In analyzing the blast cells, the monoclonal junctional DNA structure of the EBV terminal repeat was analyzed by Southern blotting and provided definitive evidence for the monoclonality of EBV-infected T cells. These findings strongly suggest that EBV plays a pathogenic role in T-cell malignancy. EBV-infected T-cell malignancy, such as this case, is very rare in Japan, especially in adult.

[The clinical efficacy of imipenem/cilastatin sodium in orthopedic infections and drug levels in the bone tissue].

We evaluated the clinical efficacy of imipenem/cilastatin sodium (IPM/CS--a carbapenem antibiotic) against orthopedic infections, and the drug levels of the bone tissues were determined. The clinical efficacies for 6 patients in the infection group were good in 3 cases, and fair in the other 3; giving an efficacy rate of 50%. Bacteriologically, 8 strains were isolated from patients with the infection and an eradication rate of 87.5% was obtained upon the treatment. In 39 patients that were given the drug prophylactically, no postoperative infections occurred. Mean IPM levels in the bone and the bone marrow at 1 hour after administration in 5 patients of the prophylactic group were 17.3 micrograms/ml and 5.9 micrograms/g, respectively. The ratio of concentrations the bone to those in the bone marrow was 34.6%. The results of this study suggest that IMP/CS reaches to the bone tissue providing sufficient concentrations and that the drug is efficacious for the prophylaxis and the treatment of orthopedic infections.

[Aging and the focal macular electroretinogram].

Focal macular electroretinograms (MERGs) were recorded with a fundus monitor through an infrared television fundus camera in 112 eyes of 112 normal subjects (68 males, 44 females), using three stimulus spots 5 degrees, 10 degrees, and 15 degrees in diameter. The amplitude and the implicit time of the a-wave, h-wave, and oscillatory potentials (OPs), and the ratio of amplitude of the b-wave to the a-wave (b/a ratio) at each stimulus spot were compared in terms of aging and sex. The amplitude of all components decreased significantly (p < 0.01-0.05) after the fifth decade, and there was a tendency for OPs to decrease more than the a-wave and b-wave. There was no significant effect of age on the h/a ratio and the implicit time of each component. The ratio of increase of amplitude to the enlargement of stimulus spot showed no significant changes with age. There was no significant difference in each component in terms of amplitude and implicit time with sex. The decrease of amplitude of all components after the fifth decade suggests aging of the macular cones. The tendency toward decreasing OPs also suggests aging of the inner retina in the macular region. The proportional changes of amplitude recorded with three different sizes of stimulus spots indicate that there is no significant effect of aging on the fovea, parafovea, or perifovea.

[Relationship between left atrial spontaneous contrast echo and coagulofibrinolytic findings].

Transesophageal echocardiography (TEE) has been used to identify patients with left atrial thrombi (LAT) and spontaneous contrast echo (SCE). Patients with LAT or SCE are at high risk for cerebral embolism. The aim of this study was to examine the relationship between SCE detected with TEE [5MHz] and coagulation and fibrinolysis factors. We took blood samples from 83 patients to measure plasma levels of TAT, PIC, D-dimer, fibrinogen and thrombomodulin (TM) just before TEE examination. The patients were classified into four groups according to SCE grade based on echo density and area of the left atrium occupied by SCE. Plasma TAT and D-dimer were higher in the high grade SCE group than in the lower SCE group. Plasma TM was lower in the high grade SCE group. From these results, high grade SCE detected by TEE correlates with activation of coagulation and fibrinolysis.

[Reoperation for lung cancer: indications and operation methods in cardiopulmonary function].

A second pulmonary resection after initial operation for lung cancer was performed 20 patients (10 with a second primary lung cancer, 8 with a metastatic lung cancer, 2 with lung abscess in pulmonary aspergillosis). All patients had radical lobectomy in first operation. Eight patients underwent completion pneumonectomy, one patient had another lobectomy, two patients underwent wedge resection after initial ipsilateral lobectomy. One patient underwent contralateral lobectomy seven patients had contralateral wedge resection after initial lobectomy. Any patient did not dead within 30 days after the reoperation, not hospital death and major complications. When a second pulmonary resection (especially, completion pneumonectomy) is required, its radicality and the need to preserve residual respiratory function and cardiac function (FEV1.0 more than 800 ml/body and %FVC more than 35%, total pulmonary vascular resistance at the unilateral pulmonary artery artery occlusion test less than 700 dyne.sec.cm-5/m2) must be considered in making the decision to operation. The five-year survival rate after reoperation for patients with lung cancer was 41.0% according to the Kaplan-Meier methods. Patients with a second primary lung cancer (the three-year survival rate, 83.5%) have appeared to do better than those with pulmonary metastasis from lung cancer (the five-year survival rate, 25.6%) These results suggest that reoperation for lung cancer can be done safety, patients undergoing reoperation have a reasonable prospect for long-term survival.

[The relation between the anaerobic threshold and exercise-induced myocardial ischemia in patients with ischemic heart disease].

The aim of this study was to clarify whether the anaerobic threshold (AT) in patients with ischemic heart disease is determined by exercise-induced myocardial ischemia. A) The reproducibility of the VO2 at the AT (AT VO2) were studied. In 13 patients with exercise-induced myocardial ischemia, submaximal Treadmill exercise tests were performed twice using a cardiopulmonary monitoring system. The reproducibility of the AT VO2 was good (r = 0.92), and the mean +/- SD was -1.3 +/- 8.2%. B) The change of the AT VO2 after percutaneous transluminal coronary angioplasty (PTCA) was studied. In 30 patients who underwent successful PTCA, submaximal Treadmill exercise tests were performed before and after PTCA using a cardiopulmonary monitoring system. After PTCA both the exercise duration and the peak VO2 increased significantly (569.0 +/- 200.8 sec vs 681.9 +/- 206.9 sec, p < 0.001: 19.5 +/- 3. 4ml/min/kg vs 21.3 +/- 3.7ml/min/kg, p < 0.001). On the other hand, the AT VO2 did not increase (13.7 +/- 3.0 ml/min/kg vs 13.9 +/- 3.2ml/min/kg, NS). The significant increase of the AT VO2, more than 15.1%, was recognized only in 5 patients. Neither did the AT VO2 increase even in patients without hibernating myocardium. In conclusion, there are many cases in which AT is not determined by exercise-induced myocardial ischemia.

[A case of secondary myelodysplastic syndrome following chemotherapy for lung cancer].

A case of secondary myelodysplastic syndrome (MDS) following chemotherapy for lung cancer is reported. A 78-year-old man, with a smoking history of 20 cigarettes/day for 55 years, was incidentally, diagnosed as having stage IV squamous cell carcinoma of the lung in 1987 during admission for transurethral resection of bladder cancer. He received combination chemotherapy of mitomycin C, vincristin, and cisplatin for his lung cancer between July and September 1988. His clinical course remained almost stable until October 1989, when his blood count showed severe anemia and thrombocytopenia. He was diagnosed as having secondary MDS induced by cytotoxic agents used for the treatment of lung cancer, based on the dysplastic findings of precursor cells in the bone marrow and the chromosome abnormality of 51XY, +8, +9, +21, 3p-, 5q-, +2mar. He died of infection with the progression of MDS in March 1990.

Sideroblastic anemia showing unique response to pyridoxine.

We treated and followed up for 6 years a patient with pyridoxine-responsive sideroblastic anemia. The patient was a boy age 1 year and 9 months, who was diagnosed on the basis of peripheral red cell morphology and an increased number of sideroblasts in the bone marrow. Bone marrow erythroblasts showed a marked reduction of delta-aminolevulinic acid synthase (ALA-S) activity. The response of the patient to pyridoxine and its active form, pyridoxal phosphate, was unique. After the first course of pyridoxal phosphate therapy (300 to 500 mg/day i.v. for 4 days), all hematological data were restored to normal and remained normal for 29 months without the further administration of pyridoxal phosphate. The second course of pyridoxal phosphate therapy (500 mg/day i.v. for 2 days) was effective for 6 months. The third, fourth, and fifth courses of the therapy consisted of daily oral pyridoxine hydrochloride at a dose of 180 mg/day for 4 to 6 weeks, and the respective periods of hematological remission were 7, 12, and greater than 18 months. These observations suggest the presence of a complicated ALA-S activating or inactivating system, or both, in our patient.