Exhaled nitric oxide (NO) may aid in monitoring pulmonary disease. The single-breath NO profile (subjects with nose clip) was described as a NO peak followed by a plateau (NO(PLAT)). Published exhaled NO values vary greatly, possibly due to contamination with nasal NO and differing respiratory maneuvers. We developed a technique to measure pulmonary NO, without nasal NO, by having the subject maintain a positive expiratory pressure (ensuring vellum closure), and we examined the variation in NO(PLAT) over a range of expiratory flows (4.2 to 1,550 ml/s). NO(PLAT) values rose almost 35-fold (3.2 +/- 1.4 ppb to 110.5 +/- 54.8 ppb) with decreasing flow, described by NO(PLAT) = 208.6795 x (flow rate)(-0.5995). However, NO excretion showed an almost 11-fold rise as flow increased. In summary, we present a simple technique for measuring exhaled NO without contamination by nasal NO. There is a marked flow dependence of exhaled NO concentration and excretion. Exhaled pulmonary NO is best measured at very low flow rates to amplify the signal and must be related to the expiratory flow employed.

Exhaled nitric oxide (ENO) is used increasingly as a surrogate marker of airway inflammation in research protocols that may incorporate standard efficacy measures, such as spirometry before and after bronchodilator, which could affect ENO measurements. In seven healthy volunteers and 11 mild asthmatic subjects, we measured ENO before and serially for 1 h after spirometry. On two additional days in the subjects with asthma, we reexamined the effect of spirometry as before, followed by the serial measurement of ENO for 1 h after two puffs of salbutamol (100 microgram/puff) by metered-dose inhaler or matching placebo. As early as 1 min after spirometry, ENO fell by 13% and 10% in the normal and asthmatic subjects, respectively. In both groups, ENO returned to baseline over 1 h. In the asthmatic subjects, salbutamol caused a significant mean increase of the order of 10 parts per billion in ENO (p < 0.001) for 1 h as compared with placebo inhaler. We conclude that spirometry and beta2-agonist may perturb ENO values and recommend that studies control for these factors.

No abstract

Because the occurrence of localized hypoperfusion is possibly not restricted to only the acute phase in KD, brain SPECT and MRI should also be performed in KD patients with neurologic symptoms.

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 ...

Recent advances in brain decoding have made it possible to classify image categories based on neural activity. Increasing numbers of studies have further attempted to reconstruct the image itself. However, because images of objects and scenes inherently involve spatial layout information, the reconstruction usually requires retinotopically organized neural data with high spatial resolution, such as fMRI signals. In contrast, spatial layout does not matter in the perception of “texture,” which is known to be represented as spatially global image statistics in the visual cortex. This property of “texture” enables us to reconstruct the perceived image from EEG signals, which have a low spatial resolution. Here, we propose an MVAE-based approach for reconstructing texture images from visual evoked potentials measured from observers viewing natural textures such as the textures of various surfaces and object ensembles. This approach allowed us to reconstruct images that perceptually resemble the original textures with a photographic appearance. The present approach can be used as a method for decoding the highly detailed “impression” of sensory stimuli from brain activity.

Controversy exists as to whether high frequency oscillation (HFO) increases the risk of intraventricular hemorrhage (IVH) compared with conventional mechanical ventilation (CMV). We compared the risk of intracranial hemorrhage after phenylephrine-induced hypertension, combined with a hypovolemic, hypotensive insult followed by rapid volume replacement in two groups of newborn beagle puppies (one group on a piston pump HFO and the other on CMV).A total of 12 beagle puppies (6 on HFO and 6 on CMV) survived the protocol. Arterial blood gas analysis and arterial blood pressures through the study were of the same magnitude in both groups. The length of time for which the puppies remained hypertensive and hypotensive also did not vary significantly between the groups.Intraventricular hemorrhages were observed in two of six CMV puppies and two of six HFO puppies. One animal in each group had a white matter hemorrhage. Subarachnoid hemorrhages were seen in 4 animals on CMV and 3 on HFO.This study indicated that HFO does not increase the risk of any kind of intracranial hemorrhage, including IVH, in beagle puppies.

In 1966 Spiro et al. described the fi rst case of myopathy in which structures resembling fetal myotubes persisted into adult life. 1 In 1969 the fi rst severe infantile form was described. 2 X-linked severe infantile myotubular myopathy is a severe muscular disease characterized by neonatal hypotonia, severe global muscular weakness and respiratory distress in affected male subjects. 3 The MTM1 gene, which is mutated in X-linked severe infantile myotubular myopathy, is located on Xq28 and was identifi ed in 1996 using positional cloning. 4 The majority of patients die during the fi rst year of life. The cause of death is usually respiratory insufficiency caused by severe muscle hypotonia and weakness. Prolonged survival is observed in the milder form, or as a result of prolongation of ventilation support. 3 None of the studies in the literature discuss the complete atrioventricular block (CAVB) in this disorder. Here, we describe an autopsy case of X-linked severe infantile myotubular myopathy with CAVB. Case reportAn 11-year-old boy with X-linked severe infantile myotubular myopathy was admitted to Teikyo University Hospital because of decreased motor activity. His elder brother and two maternal cousins died in early infancy due to congenital myopathy. He had a history of severe neonatal asphyxia and sequential hypotonia with dyspnea, and had been placed on a respirator since infancy. Findings diagnostic of congenital myotubular myopathy, such as central nuclei and peripheral halo of muscle fi bers, were demonstrated in his biopsied muscle. He had deletions of nucleotides 1484 -1487 in the myotubularin gene. He was able to speak Patient Report Fig. 1 Electrocardiogram at the (a) 35th and (b) 38th hospital day. Fig. 2As compared with the (a) normal atrioventricular (AV) node, the (b) AV node in the present patient was densely fi brosed with reduced numbers of capillaries (HE). Arrows, border of AV nodes.