X-linked dilated cardiomyopathy with a large hot-spot deletion in the dystrophin gene.

A 36-year-old Japanese man was hospitalized with coughing and exertional dyspnea (NYHA class I). He was diagnosed as having congestive heart failure, and was treated with diuretics and a beta-adrenergic blocking agent. He responded well to the treatment and his symptoms completely disappeared within a few days. Based on his clinical, laboratory, and molecular genetic findings, he was diagnosed as having X-linked dilated cardiomyopathy (XLDCM). He was found to have a large deletion in the dystrophin gene, involving exons 45-55. This is the first report on a Japanese XLDCM patient with a mutation in the central hot-spot region of this gene.

[Infected emphysematous bullae in a patient with diabetes mellitus].

A 42-year-old woman with diabetes mellitus was admitted to our hospital because of fever, coughing, and dyspnea. Coarse crackles were audible and respiratory sounds were weak in the right lung field. Laboratory examination revealed a high erythrocyte sedimentation rate, a high level of serum C-reactive protein, a high blood sugar level, and hypoxemia. A chest roentgenogram revealed cystic lesions with fluid levels, and an infiltration shadow in the right lung field. A chest computed tomographic scan revealed many cystic lesions with fluid levels and an infiltration shadow. Our diagnosis was infected emphysematous bullae. A tube was inserted percutaneously for drainage and to allow injection of antibiotics into the cystic lesion. The cystic lesion then vanished. Percutaneous drainage and washing with antibiotics can be used to treat infected emphysematous bulla that have thick closed cystic walls.

[Incidence of hip fracture and prognosis in Ehime Prefecture].

Hip fracture among the elderly has increasingly attracted public health concern in Japan. For the purpose of revealing the epidemiological features of hip fracture, post-treatment prognosis, and related factors, we studied all cases of hip fracture which occurred in Ehime Prefecture during 1992 who were admitted to hospitals or clinics, and followed up the cases for a subsequent two years. The incidence rate of hip fracture was 29.2 per 100,000 for males, and 84.0 for females, which are much lower than in Europe and the Untied States. Compared with rates reported previously in Japan, the age--specific incidence rates for males in Ehime were almost identical to the respective rates estimated by a nationwide survey and the rates for Tottori. However, the rate for males 80 years old or more was found to be lower than the corresponding rate for Kagawa. In females, the age--specific incidence rates for Ehime were similar to the Japanese averages, and the rates for Tottori and Kagawa. Falling from a standing position was a leading cause of hip fracture among older patients. Osteosynthesis was the main treatment modality elected trochanteric fractures. On the other hand, prosthetic replacement was predominant in cervical fracture. Multivariate analysis using a multiple logistic model showed that medical facility category, age, type of treatment, and cause of fracture had statistically significant relations to mobility at the time of discharge. Of patients who could walk at the time of discharge, 83.7% (159/190) were alive two years after discharge. Multiple logistic model analysis identified gender as a significant contributing factor to this mortality. Among the survivors, 81.8% (130/159) retain the ability to walk. Logistic model analysis revealed that older age experience a significantly higher risk in losing mobility.

[A study of ceftriaxone concentration in serum and tissue of patients with chronic sinusitis].

After an intravenous drip infusion of 1 g or 2 g of ceftriaxone (CTRX), its concentrations in serum and tissue were determined using the bioassay for 18 surgical patients with chronic paranasal sinusitis. 1. The serum levels observed at 75-105, 135-165, 195-225 minutes after an intravenous drip infusion of 1 g, averaged 98 +/- 38, 92 +/- 13 and 73 +/- 13 micrograms/ml, respectively. 2. The tissue levels at 75-105, 135-165, 195-225 minutes after an intravenous drip infusion of 1 g, averaged 27 +/- 13, 27 +/- 7 and 25 +/- 9 micrograms/g. 3. CTRX appears to be a useful drug for the treatment of chronic paranasal sinusitis.

[Cardiovascular complications of interferon therapy in chronic hepatitis C].

Severe cardiovascular complications (myocardial infarction, ventricular fibrillation et al.) have been reported in patients who have been administrated extremely high dose interferon (IFN) or cardio-toxic drugs, or who have had cardiovascular diseases. But cardiomyopathy, myocarditis and atrioventricular block were reported in patients who administered low dose IFN and no cardio-toxic drugs or had no cardiovascular diseases. We report here cardiovascular complications during IFN therapy for chronic hepatitis C. Cardiovascular complications that necessitated stopping IFN administration in 643 treated chronic hepatitis C patients are observed in 4 patients (0.62%). Second-degree atrioventricular block occurred in two patients, myocarditis in one patient and severe sinus bradycardia in one patient. So we have to check risk factors of cardiovascular complications before starting IFN therapy and electrocardiogram during IFN therapy.

Revised technique of cardiopulmonary bypass in one-stage repair of interrupted aortic arch complex.

Twenty infants with interrupted aortic arch associated with various intracardiac anomalies underwent primary complete repair using pulsatile high-flow cardiopulmonary bypass with a short period of circulatory arrest. Age at repair ranged from 11 to 126 days (mean age, 43 days). Weight ranged from 2.2 to 5.5 kg (mean weight, 3.4 kg). Associated cardiac lesions included ventricular septal defect (14 patients), truncus arteriosus (3), transposition of the great arteries (2), and aortopulmonary window (1 patient). Left ventricular outflow tract obstruction was relieved in 2 patients. The aortic arch was reconstructed with a polytetrafluoroethylene graft in 7 patients and by direct anastomosis in the most recent 11 patients. An arterial cannulation method has been devised to facilitate direct anastomosis between the ascending aorta and the descending aorta, to lessen circulatory arrest time, and to prevent dangerous laceration and postoperative narrowing of the thin, small ascending aorta at the cannulation site. Two patients died, a surgical mortality rate of 10%. There has been one late death, which was due to severe truncal valve insufficiency. The other patients are doing well with a mean follow-up of 3 years 6 months. Restenosis of the direct anastomosis has not been noted in any patient. However, subaortic stenosis with pressure gradients of 30 to 40 mm Hg developed in 3 patients. In conclusion, one-stage repair including direct anastomosis for the aortic arch reconstruction and repair of all coexisting intracardiac defects is thought to be the treatment of choice.

[STA-ACA anastomosis with interposed vein graft; a case report].

We report our experience with the use of three saphenous vein grafts for treating advanced occlusive cerebrovascular disease in the anterior cerebral artery (ACA) in two patients. Superficial temporal artery (STA)-ACA anastomosis with interposed vein graft is thought to be indicated for patients who have STA of insufficient length to anastomose to the ACA. Vein graft has already been demonstrated to be applicable in extracranial-intracranial artery bypass, as well as in aorto-coronary bypass. Two factors considered to be essential for successful revascularization are technical proficiency, including protection from drying, smooth approximation of vessels, and proper orientation of the graft, and the relative size of donor and recipient vessels. Because the vein graft must be anastomosed with the proximal ACA to reduce the size discrepancy between vessels, prolonged interruption of circulation during suturing is inevitable. Our operative results suggest that anastomosis to a superficial branch of the ACA just distal to the interhemispheric fissure seems to be preferable to anastomosis using vein graft, and we recommend the option of vein graft only when the STA cannot be used.

[Experience of the malignant complete intrathoracic goiter: discussion on 13 cases of Japan].

A 56-year-old woman was found to have a mediastinal mass on routine examination. Chest X-ray, CT scan, MRI and superior vena cavography showed a right upper mediastinal tumor invading the SVC. And it was diagnosed malignant intrathoracic goiter by histological examination by percutaneous needle biopsy. On thoracotomy and mediastinotomy, the tumor located only in the right upper mediastinum and had no relation to the cervical thyroid gland. Histological examination revealed papillary adenocarcinoma. We reported a malignant complete intrathoracic goiter and added discussion on another 12 cases seen in Japanese literature.

Detection of Residual penicillin in milk by sensitive enzyme immunoassay.

A convenient and precise method by an application of a highly sensitive enzyme immunoassay of penicillin to detect the veterinary penicillin residue in milk was studied. The procedures of the assay to detect more than 0.01 microgram/ml of ampicillin in milk were developed. Five commercial milk samples were tested by the assay procedure, and no veterinary ampicillin residue was detected in any of them.