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Two cases of red cell aldolase deficiency associated with congenital nonspherocytic hemolytic anemia are reported. The proband is a fourteen-month-old Japanese boy. Consanguineous marriage was not proven but probable in this family, as the parents were born in the same small island. The proband had moderate to mild anemia aggravated by upper respiratory infections, 1 cm hepatomegaly and 2.5 cm splenomegaly, but was unremarkable in other respects and has thus far not shown mental or growth retardation. He did not have dysmorphic features. The red cell aldolase activity was 6% of the normal mean. The enzyme was unstable with respect to heat, and Km for fructose 1,6-diphosphate (F-1,6-DP) was high. The parents and other heterozygotes showed intermediate activity between that of the proband and that of normal subjects. Red cell F-1,6-DP concentration in this case was remarkably increased. Red cell glucose consumption, and lactate formation, as well as hexose monophosphate shunt activity, were decreased as compared with a comparable reticulocyte-rich hereditary spherocytosis patient. Hexose monophosphate dehydrogenase by a high concentration of F-1,6-DP in his red cells. As a result of family study, another homozygous aldolase deficiency case associated with hemolytic anemia was found. He is 13 years old and a nephew of the proband's paternal grandmother. His hemolytic anemia also is moderate to mild and aggravated by upper respiratory infections. He does not seem to have mental or growth retardation, nor does he possess dysmorphic features.

Alcoholic patients with intensive suicidal ideation accompanied with alexithymia are characterized by the inability to communicate feelings. Therefore, the possibility of a suicidal attempt in those patients should always be kept in mind even though no suicide message is expressed.

Background/Aims: Previous studies using near-infrared spectroscopy (NIRS) have reported increases in both oxygenated hemoglobin (oxyHb) and deoxygenated hemoglobin (deoxyHb) during the paper version Trail Making Test (TMT), a neuropsychological test for evaluating cognitive and executive functions. We measured oxyHb and deoxyHb in the prefrontal cortex during the computer version TMT. Methods: Fifteen healthy students first performed TMT-A and then TMT-B; another 7 students first performed TMT-B and then TMT-A. The mean concentrations of Hb (10 s before TMT, during TMT and 30–40 s after TMT) were determined and analyzed by ANOVA. Results: oxyHb increased while deoxyHb decreased during the TMT. There was a significant order effect on the change in deoxyHb, but not in oxyHb. oxyHb significantly increased in the bilateral prefrontal cortices both in TMT-A and TMT-B. The increase tended to be more prominent in TMT-B than in TMT-A. deoxyHb significantly decreased in the bilateral prefrontal cortices, both in TMT-A and TMT-B. Conclusion: The results suggest that blood flow increases in the prefrontal cortex during the performance of TMT, and that the bilateral prefrontal cortices are involved in the performance of the computer version TMT.

BackgroundExcess visceral adipose tissue (VAT) is closely associated with the presence of coronary artery plaques that are vulnerable to rupture. Patients with diabetes mellitus (DM) have more VAT than patients without DM, but the extent to which VAT contributes to the characteristics of coronary plaques before and after the development of DM is not fully understood.MethodsWe retrospectively evaluated 456 patients (60% male, age 64 ± 16 years) who were suspected to have cardiovascular disease and underwent 64-slice computed tomography angiography (CTA). Seventy-one (16%) patients had vulnerable plaques (CT density < 50 Hounsfield Units, positive remodeling index > 1.05, and adjacent spotty areas of calcification).ResultsPatients were divided into tertiles according to the VAT area. There were stepwise increases in noncalcified and vulnerable plaques with increasing tertiles of VAT area in patients without DM, but not in patients with DM. Multivariate analysis showed that a larger VAT area was significantly associated with a higher risk of vulnerable plaque in patients without DM (odds ratio 3.17, 95% confidence interval 1.08–9.31, p = 0.04), but not in patients with DM.ConclusionsThe VAT area is associated with the characteristics of coronary plaques on CTA in patients without DM, but not in patients with DM. VAT may be a significant cardiometabolic risk factor that is associated with plaque vulnerability before the development of DM. CTA findings may help to improve risk stratification in such patients.

We conducted a double-blind, placebo-controlled, parallel group study to assess the anti-glycation effect of mixed herbal extract (MHE) in individuals with pre-diabetes mellitus. MHE was produced using hot water extraction from Anthemis nobilis (Roman chamomile), Crataegus oxyacantha (hawthorn berry), Houttuynia cordata (dokudami), and Vitis vinifera (grape leaf). We also assessed whether MHE showed favorable effects on one's quality of life (QOL). Design: The subjects consisted of 26 volunteers (male: 21; female: 5; age: 50.5 ± 8.5 years) with pre-diabetes mellitus (HbA1c: 5.5-6.7%). They were divided into two groups, the Test Group (13 subjects, age: 52.8 ± 8.2 years) and the Control Group (12 subjects, age: 49.3 ± 7.8 years). The Test Group was administered 1,200 mg of MHE (solid substance) per day for 8 weeks. The Control Group was administered a placebo. Results: The inter-group analysis using the Anti-Aging QOL Common Questionnaire (AAQol) showed that the score for the parameters, "muscular pain/stiffness", "headache", "easily angered", "reluctance to talk with others", "memory lapse", and "inability to readily make judgments" was significantly improved in the Test Group (p < 0.05). In terms of sugar metabolism, no significant variation was observed in fasting blood glucose, HbA1c, glycoalbumin, and insulin. A significant variation was not observed in the Test Group with regard to 3-deoxyglucosone (3DG), an intermediate of glycation, and N ε-(carboxymethyl)lysine (CML) and pentosidine, advanced glycation endproducts (AGEs), in blood after 8 weeks. However, in the subjects with HbA1c of equal to or higher than 5.9%, the subclass inter-group analysis showed that the supplementation of MHE significantly inhibited (p < 0.05) an increase of CML in the Test Group, while CML increased in the Control Group. There was no significant variation in the Test Group regarding the oxidative stress markers, 8-hydroxy-2'-deoxyguanosine (8-OHdG) and isoprostane in urine. The skin elasticity index (R2) obtained by using the cutometer started to decrease significantly in the Control Group after 4 weeks (p < 0.05), while the index showed a tendency that the skin elasticity was successfully maintained in the Test Group. There was no adverse event which was associated with the test product. Conclusion: These results suggest that MHE may improve the symptoms related to QOL as well as inhibit the generation of CML, one of AGEs, in individuals with abnormal sugar metabolism. Furthermore, the 8 weeks supplementation of MHE was considered to be safe.

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