A case of supravalvular aortic stenosis with spontaneous remission of peripheral pulmonary stenosis

A 14-year-old junior high school boy was admitted to our institute. Previously he had been diagnosed as having peripheral pulmonary stenosis (Gay's classification, type IV) at the age of 2 years and 10 months. On this occasion, however, a diagnosis of supravalvular aortic stenosis was made, with a pressure gradient of about 120 mmHg, and all examinations showed spontaneous remission of peripheral pulmonary stenosis. He underwent a successful standard aortoplasty. This is the first reported case of spontaneous remission of peripheral pulmonary stenosis.     

Pulmonary sequestration associated with asymptomatic aspergillosis.

Intralobar pulmonary sequestration associated with asymptomatic aspergillosis is a rare case. We describe the case of a 65-year-old woman with intrapulmonary sequestration, anomalous systemic arterial supply to the left lower lobe and aspergillosis who underwent left lower lobectomy and ligation of an anomalous artery by Video-Assisted Thoracoscopic surgery (VATS). Pathological examination showed the parenchymal distortion and chronic inflammation. Aspergillus were found in the cyst. VATS lobectomy for intralobar pulmonary sequestration is a safe and valid procedure.     

Left ventricular asynergy in patients with impending myocardial infarction: two-dimensional echocardiographic assessment

To determine the clinical significance of regional left ventricular asynergy in patients with impending myocardial infarction, we recorded two-dimensional echocardiograms (2DE) serially and performed coronary angiography immediately after the hospital admission in nine patients with initial impending infarction and their last anginal attacks were within 48 hours. Left ventricular asynergy on the first 2DE was observed in six of nine patients during symptom-free periods (Group A: LV asynergy group). Five of the six patients had significant coronary artery lesions (greater than or equal to 75% stenosis) in at least one major coronary artery. Intracoronary filling defects were detected in four of the five patients. Another three patients without asynergy (Group B) had significant fixed stenosis. Coronary artery spasm was observed in two patients during coronary angiography, but no patient had intracoronary filling defects. Intracoronary nitroglycerin (0.1-0.3 mg) reduced the severity of coronary artery narrowing in two patients. In addition, urokinase (240,000-480,000 IU) via the corresponding vessel (PTCR) in the remaining seven patients resulted in reduction in the severity of coronary artery stenosis in four patients, but not in the remaining three patients. Left ventricular wall movement in the asynergy group improved rapidly and no asynergy was observed by the seventh hospital day in five of the six patients. Successful PTCR treatment resulted in improvement of left ventricular wall movement. No asynergy was found in the non-asynergy group throughout their hospitalizations. These findings indicated that abnormal left ventricular wall movement is found in patients with impending myocardial infarction, even during symptom-free periods, but the wall movement gradually improves. The 2DE observations are useful for estimating the clinical status and for planning precise therapy for impending myocardial infarction.     

Angiomyolipoma of the kidney; report of two cases with a low fat component

We report two cases of unilateral renal angiomyolipoma. In both cases, our preoperative diagnosis was renal cell carcinoma because no low density area compatible with a fatty tissue was noted in the tumors. Histological examination revealed both tumors to be angiomyolipoma mainly composed of myomatous cells and immature fat cells.     

PEUTZ‐JEGHERS SYNDROME ASSOCIATED WITH RENAL AND GASTRIC CANCER THAT DEMONSTRATED AN STK11 MISSENSE MUTATION

A 75‐year‐old male was admitted to the gastroenterology unit of Nagoya City University Hospital due to epigastralgia after surgical treatment for right renal cancer. Endoscopy revealed advanced type 1 gastric cancer in the corpus of the stomach and multiple polypoid lesions in the stomach and duodenum. X‐ray examination of the small intestine using barium showed multiple polyps in the upper jejunum. Faint pigmentation on the palm was also detected. Peutz‐Jeghers syndrome (PJS) was diagnosed, despite a lack of family history. Total gastrectomy, resection of part of the upper jejunum and intraoperative endoscopic polypectomy of duodenal polyps was performed. This is the second reported case of PJS associated with renal cancer. We also detected a missense mutation in the tumor suppressor gene STK11 that, when mutated, is causative for PJS.     

Discrete phase of calcium excess in the process of deterioration in an individual rat myocyte

While there have been many reports of the significant role of cytoplasmic free calcium ion in myocardial injury, these have been carried out in multicellular preparations. Since cell injury may occur inhomogeneously, it is necessary to observe the 'history' of an individual myocyte in order to investigate the detailed role of the calcium ion in the process of myocardial injury. We have observed the natural history of individual myocytes isolated from the left ventricle of rats with respect to changes in shape and cytoplasmic free calcium concentration ([Ca2+]i) measured with fura-2. We can discriminate four phases in the time course of cell deterioration. In the first phase (phase O), the myocyte is rod shaped, quiescent and responsive to electrical stimulation. The [Ca2+]i is stable. In the next phase (Phase 1), once initiated, the myocyte exhibits an asynchronous wavy contraction and gradually decreases in length. The [Ca2+]i gradually increases with some fluctuation. Phase 2 is characterized by rapid development of contracture with a marked increase in [Ca2+]i. In the period following establishment of contracture (Phase 3), changes in [Ca2+]i vary from cell to cell, possibly because of leakage of the dye caused by loss of cell membrane integrity. Our results indicate that, during naturally occurring cell deterioration, loss of [Ca2+]i control at the membrane of the sarcoplasmic reticulum precedes contracture and catastrophic increase in [Ca2+]i.