Key wordsaortic aneurysm, 3D computed tomography, tuberous sclerosis.Tuberous sclerosis (TS) is an autosomal dominant disorder associated with the development of multiple systemic hamartomas. Its classic triad includes seizure, mental retardation, and facial angiofibroma, and it is also known to be accompanied by various other complications. However, there are few reports on patients with TS who have developed an aortic aneurysm. We report a 2-year-old boy with TS who developed asymptomatic descending aortic aneurysm. Case reportA 26-month-old boy was admitted to The University of Tokyo Hospital, Tokyo, Japan, for further examination and treatment of an abnormal mass. His family history was unremarkable. He was born at the gestational age of 37 weeks and 4 days, and on the third day of life he underwent surgical excision of sacrococcygeal chordoma. Before the surgery, systemic examination revealed a subcortical nodule, harmatoma of the ocular fundus, and the rabdomyoma of myocardium. The patient was diagnosed with TS. Since then, he had had regular check-ups at the nearby hospital. He visited the nearby hospital at the age of 1 year and 10 months, at which time his parents reported that he has had recurring upper respiratory tract infections over the previous 2 months. Because chest X-ray revealed an abnormal mass, he was referred to The University of Tokyo Hospital, Tokyo, Japan, for further examination and treatment. On admission, his height was 88 cm, body weight was 11.4 kg, heart rate was 130 bpm, body temperature was 37.0 ° C, and blood pressure was 80/56 mmHg. Oxygen saturation on room air was 97%. His general condition was good. On physical examination, he had vitiligo of various sizes on his back as well as on his abdomen. A Shagreen patch was also found on his back. Chest auscultation was normal. No significant murmurs were detected, and no mass was found in his abdomen by palpation. There were no neurological abnormalities.Blood examination did not reveal any abnormalities in inflammatory reactions, antinuclear antibody levels, nor the coagulation system. Chest X-ray revealed a mass shadow (Fig. 1). On echocardiography, aneurysm of the descending aorta and a high-intensity area in the ventricular septum were detected; the latter seemed to be a trace of cardiac rhabdomyoma. 3D computed tomography (CT) scans on day 3 of hospitalization revealed a thoracic aortic aneurysm, which extended to the abdominal aorta with a length of 6.5 cm and a diameter of 3.5 cm, as well as a daughter lesion with a diameter of 2.5 cm (Fig. 2). There was also atelectasis of the left lower lung lobe, which was assumed to have resulted from the mass of the aneurysm.During the admission, we performed a brain CT, which indicated that there were no aneurysms in the brain arteries. We diagnosed the patient as having aneurysms of the thoracic and abdominal aorta combinined with TS, since there were no findings that suggested the possibility of vasculitis. On the eighth day of admission, artificial vessel replacement of the aorta was ...
Electrical output efficiency from renewable energy sources tends to be lower because the converters, such as power conditioning subsystems (PCSs), are less efficient in partial load. Operation in partial load is common because of the fluctuations are typical in natural energy. We proposed and developed a high-efficiency converter system that uses a renewable energy source such as photovoltaics (PV), lithium-ion capacitors (LiCs) for temporary energy storage, a converter for external output, a pyranometer, and a controller. PV and LiCs are directly connected without a converter, and LiCs store electrical energy with ultra-high efficiency (approximately 99.4%) of PV output. The converter for external output can convert with high efficiency at the rating at one stroke from the stored energy in LiCs, while continuing to generate PV power. In general, a PCS for PV is operated at low efficiency at low solar radiation intensity of approximately 350 W/m 2 or below, and the operation stops when solar radiation intensity is less than approximately 200 W/m 2 . In the proposed system, LiCs temporarily store energy when solar radiation intensity is less than 350 W/m 2 or when the conversion efficiency of PCS drops below 80%. After solar radiation intensity increases or LiCs are recharged, PCS can output at over 92% efficiency by converting stored energy in LiCs at one stroke. As a result, the proposed system increased annual gross power generation in Japan by more than 12%.
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