Acetoacetate (AcAc) and 3-hydroxybutyrate (3HB), the two main ketone bodies of humans, are important vectors of energy transport from the liver to extrahepatic tissues, especially during fasting, when glucose supply is low. Blood total ketone body (TKB) levels should be evaluated in the context of clinical history, such as fasting time and ketogenic stresses. Blood TKB should also be evaluated in parallel with blood glucose and free fatty acids (FFA). The FFA/TKB ratio is especially useful for evaluation of ketone body metabolism. Defects in ketogenesis include mitochondrial HMG-CoA synthase (mHS) deficiency and HMG-CoA lyase (HL) deficiency. mHS deficiency should be considered in non-ketotic hypoglycemia if a fatty acid beta-oxidation defect is suspected, but cannot be confirmed. Patients with HL deficiency can develop hypoglycemic crises and neurological symptoms even in adolescents and adults. Succinyl-CoA-3-oxoacid CoA transferase (SCOT) deficiency and beta-ketothiolase (T2) deficiency are two defects in ketolysis. Permanent ketosis is pathognomonic for SCOT deficiency. However, patients with "mild" SCOT mutations may have nonketotic periods. T2-deficient patients with "mild" mutations may have normal blood acylcarnitine profiles even in ketoacidotic crises. T2 deficient patients cannot be detected in a reliable manner by newborn screening using acylcarnitines. We review recent data on clinical presentation, metabolite profiles and the course of these diseases in adults, including in pregnancy.

5. Saulyte J, Regueira C, Montes-Martinez A, Khudyakov P, Takkouche B. Active or passive exposure to tobacco smoking and allergic rhinitis, allergic dermatitis, and food allergy in adults and children: a systematic review and meta-analysis. PLoS Med 2014;11:e1001611. 6. Ohmen JD, Hanifin JM, Nickoloff BJ, Rea TH, Wyzykowski R, Kim J, et al. Overexpression of IL-10 in atopic dermatitis: contrasting cytokine patterns with delayed-type hypersensitivity reactions. J Immunol 1995;154: 1956-63. 7. Ricci G, Patrizi A, Federica B, Calamelli E, Dell'Omo V, Bendani B, et al. Cytokines levels in children affected by atopic and nonatopic eczema. Open Dermatol J 2008;2:18-21. 8. Howell MD, Novak N, Bieber T, Pastore S, Girolomoni G, Boguniewicz M, et al. Interleukin-10 downregulates anti-microbial peptide expression in atopic dermatitis. J Invest Dermatol 2005;125:738-45. 9. Ji S, Shin JE, Kim YS, Oh JE, Min BM, Choi Y. Toll-like receptor 2 and NALP2 mediate induction of human beta-defensins by fusobacterium nucleatum in gingival epithelial cells.

Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency and mitochondrial acetoacetyl-CoA thiolase (betaketothiolase or

A novel amidoxime-group-containing adsorbent of hollow-fiber form (AO-H fiber) was prepared by radiation-induced graft polymerization of acrylonitrile onto a polyethylene hollow fiber, followed by chemical conversion of the produced cyano group to an amidoxime group. Distribution of the amidoxime group was uniform throughout the hollow-fiber membrane. The fixed-bed adsorption column, 30 cm in length and charged with the bundle of AO-H fibers, was found to adsorb uranium from natural seawater at a sufficiently high rate: 0.66 mg uranium per g of adsorbent in 25 days.

1977Colella, M. B.; Siggia, S.; Barnes, R. M. Anal. Chem. 1980,52, 967. Graziani, R.; Bombieri, G.; Forsellini, E. J. Chem. SOC., Dalton Trans. 1972, 2059. Hirotsu, T.; Katoh, S.; Sugasaka, K.; Takai, N.; Sen6, M.; Itagaki, T.; Ouchi, H. Preprints of International Meeting on Recovery of Uranium from Seawater, Tokyo, 1983, p 80. Hirotsu, T.; Katoh, S.; Sugasaka, K.; Sen& M.; Itagaki, T. J . Chem. SOC., Dalton Trans. 1986a, 1609. Hirotau, T.; Katoh, S.; Sugasaka, K.; Sen6, M.; Itagaki, T. J. Chem. SOC., Dalton Trans. 1986b, 1983. Hirotsu, T.; Katoh, S.; Sugasaka, K.; SenG, M.; Itagaki, T. Sep. Sci. Technol. 1986c, 21, 1101. Hirotsu, T.; Katoh, S.; Sugasaka, K.; Sakuragi, M.; Ichimura, K.; Suda, Y.; Fujishima, M.; Abe, Y.; Misonoo, T.

AD-CMC is known to be caused by a gain-of-function mutation of the STAT1 gene. Chronic active hepatitis is a rare complication of AD-CMC, with currently unknown pathogenesis. It seems that the clinical phenotype in this patient is modified by autoimmune mechanisms and cytokine dysregulation. AD-CMC can be complicated by various immune disorders including autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.

Measurement of VEGF may be a useful tool for predicting the course of IH and monitoring the effectiveness of treatment.

Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of the distal extremities of young adults. Metastatic PMH can be fatal and there are no effective medications. We describe a case of a 15-year-old boy with metastatic PMH, who responded to treatment with everolimus, a mammalian target of rapamycin inhibitor. Immunohistochemistry showed that mammalian target of rapamycin was expressed in PMH biopsy specimens, which may explain the reduction in PMH tumor size following treatment.