Saccular aneurysm of the aortic cusp associated with discrete subaortic stenosis

A 54-year-old male who experienced a syncopal episode underwent aortic valve replacement for aortic stenosis and regurgitation. The aortic valve was incompetent as a result of thickening of the left coronary cusp and noncoronary cusp. In addition a saccular aneurysm was indicated on the left coronary cusp. A shelf of tissue protruding at right angles from the ventricular septum was particularly prominent below the right coronary cusp, resulting in subvalvular stenosis. The cause of the saccular aneurysm was most likely caused by the long-term effects of the jet stream instigated by discrete subaortic stenosis.     

Maximum permissive dosage of lactose and lactitol for transitory diarrhea, and utilizable capacity for lactose in Japanese female adults

This study aims to estimate the tolerable lactose intake which can be utilized in the digestion by lactase and in the fermentation by intestinal microbes in Japanese female adults. The first, the maximum permissive dosage of lactose not to induce transitory diarrhea was estimated based on the oral ingestion of lactose at several dose levels in all the subjects, and compared with that of lactitol which is not hydrolyzed by digestive enzymes. A second lactose tolerance test involving 10 g and 30 g of lactose was carried out in 10 subjects showing resistance to diarrhea, and serum glucose and insulin levels and the amount of hydrogen excreted in the breath were measured for comparison with those of glucose and lactitol. Subjects were 43 Japanese female adults (average: age 20.5+/-2.1 y, weight 51.3+/-5.1 kg) who had not been diagnosed as having either hypolactasia or being lactose intolerant. Serum glucose and insulin levels were scarcely elevated following the ingestion of both 10 g and 30 g of lactose, while the amount of hydrogen excreted in the breath was greatly increased following the ingestion of 30 g of lactose, but these levels were less following the ingestion of 10 g of lactose. In contrast, the ingestion of 15 g of glucose significantly increased blood glucose and insulin levels, while no hydrogen was detected in the breath. The maximum permissive dosage of lactose not to induce transitory diarrhea was 0.72 g/kg of body weight and that of lactitol was 0.36 g/kg of body weight in Japanese adults. The digestive capacity of lactase is less than 10 g of lactose by single ingestion, while intestinal microflora are able to ferment approximately 20-30 g of lactose. In addition, the ingestion of more than 10 g of lactose might be contributed as prebiotics.     

An intestinal volvulus caused by multiple magnet ingestion: an unexpected risk in children

It has been reported that ingested magnets can cause intestinal fistula formation or perforation, leading to intestinal obstruction. However, there are no previous case reports that magnet ingestion additionally caused an intestinal volvulus. We report herein the case of a 1-year-old boy in whom the ingested magnets caused a volvulus of part of the small intestine leading to the resection of the necrotic portion. We think that if more than one magnet is found as a foreign body in the intestine, they should be removed immediately by laparotomy. Clinicians who care for children should be aware of this unexpected risk.     

Remission induced by interferon alfa in a patient with massive osteolysis and extension of lymph-hemangiomatosis: a severe case of Gorham-Stout syndrome

The treatment of massive osteolysis with lymphangioma and/or hemangioma (Gorham-Stout syndrome) has been controversial. The authors report on a patient with multiple massive osteolyses and extensive lymph-hemangiomatosis whose lesions were reduced by interferon alfa therapy.A 2-year-old girl had complained of left chylothorax. Thoracoscopy showed an increase in small lymphatic vessels in the chest wall. The chylothorax was improved by coagulation of the lymphatic vessels. Later, multiple massive osteolyses appeared in the left 11th and 12th ribs, the TH10-L3 vertebrae, and the right femur. There were also hemangiomas in the liver and spleen, a tumor lesion in the left lower chest wall, and hemangiomatous change on the skin surface of the left back. The left lung had only a minimal air content. After OK-432 was injected into the femur and chest wall lesions, the femur lesion disappeared. Then, as right chylothorax appeared, OK-432 was injected into the right pulmonary cavity. The chylothorax disappeared, but pericardial effusion appeared. After steroid pulse therapy, pericardial effusion disappeared. During these treatments, the 7th to 10th ribs disappeared from the x-ray and scoliosis developed. One month later, a cloudy fluid collection in the right lung was found on computed tomography. Interferon alfa and steroid pulse therapy were started. Interferon alfa (1,500,000 units) was subcutaneously administered daily for 2 months and was gradually reduced and maintained at 1,500,000 unit/wk. Steroids were also reduced and maintained at 5 mg/d of predonine. Later, the progress of osteolysis and the extension of lymph-hemangiomatosis stopped. Ten months later, hemangioma in the back disappeared, and the 7th to 10th ribs, which had disappeared, reappeared. The interferon alfa therapy was stopped 14 months after it was administered. The patient's condition has been stable for 10 months since then. At this time, computed tomography shows regression of the hemangiomatous lesion in the back.The authors clinically diagnosed the patient as having Gorham-Stout syndrome with extension of lymph-hemangiomatosis. Interferon alfa with or without steroid therapy should be a choice for patients with extension lesions.     

Treatment of congenital pseudarthrosis of the tibia: a multicenter study in Japan

Treatment of congenital pseudarthrosis of the tibia (CPT) remains a challenge. To clarify the current situation in treatment, a multicenter study was carried out to obtain information on the results of CPT treatment. The objective of this study was to propose appropriate treatment guidelines for CPT. Records of 73 patients with CPT who underwent surgical treatment were collected from 32 hospitals. The modality of the treatment was 26 with Ilizarov technique, 25 with free vascularized fibular graft, 7 with a combination of the two techniques, 6 with intramedullary nailing with free bone grafting, 5 plating with free bone grafting, and 4 with other treatments. Fifty-four procedures resulted in union, 7 resulted in delayed union, 7 were left un-united, 1 underwent amputation, and the results were unknown in 4. According to the results of this study, the most acceptable methods of treatment of CPT are the Ilizarov method and the vascularized fibular graft.