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A metal-lustrous self-standing film, named “porphyrin foil”, was formed from a glass-forming polymeric porphyrin. The amorphous glass nature of the porphyrin foil played a key role in spontaneously producing a smooth surface. Its sharp contrast in intense absorption and specular reflection of light at each wavelength provided a brilliant metallic lustre. [ABSTRACT FROM AUTHOR]

In this study, heat transfer in a square cavity filled with a Phase Change Material (PCM) under a sinusoidal wall temperature during solidification and melting is analyzed. All surfaces of the cavity are insulated except one surface, which is under the sinusoidal temperature change. The governing equations and boundary conditions are made dimensionless to reduce the number of governing parameters into two as dimensionless frequency and Stefan number. The governing equations were solved numerically by using Finite Volume Method for a wide range of Stefan number (0.1 < Ste < 1.0) and dimensionless frequency (0.23 < ω * < 2.04). Based on the obtained results, a chart in terms of Stefan number and dimensionless frequency is obtained to divide the heat transfer process in the cavity into three regions as uncompleted, completed, and overheated phase-change processes. For the uncompleted process, some parts of the cavity are inactive, and no phase change occurs in those parts of the cavity during the melting and freezing process. For the overheated phase change, the temperature of the cavity highly increases (or decreases), causing the sensible heat storage to compete with latent thermal storage. In the completed process, almost all thermal storage is done by the utilization of latent heat. The suggested graph helps thermal designers to avoid wrong designs and predict the type of thermal storage (sensible or latent) in the cavity without doing any computations. [ABSTRACT FROM AUTHOR]

Phenylketonuria (PKU) and hyperphenylalaninemia (HPA), both identified in newborn screening, are attributable to variants in PAH . Reportedly, the p.R53H(c.158G>A) variant is common in patients with HPA in East Asia. Here, we aimed to define the association between p.R53H and HPA phenotype, and study the long-term outcome of patients with HPA carrying p.R53H. We retrospectively reviewed the genotype in 370 patients detected by newborn screening, and identified the phenotype in 280 (117, HPA; 163, PKU). p.R413P(c.1238G>C) was the most frequently found ( n = 117, 31.6%) variant, followed by p.R53H ( n = 89, 24.1%). The odds ratio for heterozygous p.R53H to cause HPA was 48.3 (95% CI 19.410-120.004). Furthermore, we assessed the non-linear association between the phenylalanine (Phe) value and elapsed time using the follow-up data of the blood Phe levels of 73 patients with HPA carrying p.R53H. The predicted levels peaked at 161.9 μmol (95% CI 152.088-172.343) at 50-60 months of age and did not exceed 360 μmol/L during the 210-month long observation period. The findings suggest that patients with HPA, carrying p.R53H, do not need frequent Phe monitoring as against those with PKU. Our study provides convincing evidence to determine clinical management of patients detected through newborn screening in Japan.

A 74-year-old Japanese man with myelodysplastic syndrome (MDS) received chemotherapy with azacitidine. From the second day after starting the administration, he complained of fever, cough and shortness of breath. Chest roentgenography and computed tomography showed consolidations and ground-glass opacities. His symptoms grew from worse to life-threatening. We diagnosed him with azacitidine-induced pneumonitis and began administering corticosteroids. Thereafter, his symptoms and radiographic abnormalities improved. Azacitidine is a hypomethylating agent that improves the survival of MDS patients. Although this drug is commonly well tolerated and rarely causes severe lung injury, it is important to consider the potentially serious adverse effects of azacitidine-induced pneumonitis.

We report two cases of bilateral racemose hemangioma in patients with hemoptysis. Case 1 was a 62-year-old woman who visited a local clinic complaining of hemoptysis. Bronchoscopy revealed multiple pulsating tumorous lesions and she was referred to our department. Chest Multidetector CT (MDCT) disclosed bilateral enlarged and convoluted, abnormal bronchial arteries and primary racemose hemangioma was diagnosed. Bronchial artery embolization was selected, but not conducted because of concern regarding the arteriovenous shunt and escape into the greater circulation. In combination with thoracoscopic mini-thoracotomy, ligation and separation of the bronchial artery were performed. Case 2 was a 68-year-old man who was transferred to our department with a chief complaint of hemoptysis. MDCT revealed bilateral bronchiectasis and a convoluted and enlarged, abnormal bronchial artery along the mediastinum. He was diagnosed as having secondary racemose hemangioma. First, bronchial artery embolization was conducted, but hemostasis was difficult, thus surgical ligation was conducted. In both cases, MDCT was effective for diagnosis and surgical ligation is very important as a therapeutic option for racemose hemangioma.