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Objective-To investigate cardiac function in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and clarify the clinical features of cardiomyopathy in MELAS. Patients-11 consecutive patients with MELAS (mean age at initial examination 11.3 years, range 4 to 16 years) were enrolled in the study. Six were followed for more than five years. Results-On echocardiographic examination, three patients showed increased left ventricular end diastolic posterior wall thickness (LVPWTd), exceeding 140% of the normal value. Four patients, including these three, had an ejection fraction of less than 50%, and two also had increased left ventricular end diastolic volume (LVEDV) exceeding 140% of the normal value (%N). The LVPWTd%N was correlated positively with the LVEDV%N (R = 0.669, p < 0.05) and negatively with the ejection fraction (R = −0.6701, p < 0.05). One patient died of heart failure aged 22 years. Conclusions-The cardiomyopathy in MELAS is characterised by an abnormally thick left ventricular wall with progressive dilatation and poor left ventricular contraction developing over several years, indicating hypertrophic cardiomyopathy advancing to dilated cardiomyopathy. (Heart 1998;80:292-295)

To detect a causative superantigen and to clarify a possible role for staphylococci in Kawasaki disease (KD), culture supernatants of individual bacterial isolates from 11 acute-stage patients were studied. Toxic shock syndrome toxin-1 (TSST-1) and antibody to TSST-1 and enterotoxins A (SEA), B (SEB), and C (SEC) in acute (mean, day 7) and late convalescent (mean, month 15) sera from 26 patients (12 with coronary artery aneurysms) and 22 age-matched controls were measured. Only 1 of 60 supernatants was mitogenic for human lymphocytes; it was 1 of the 4 Staphylococcus aureus isolates. Mitogenicity was neutralized by sera obtained after administration of intravenous gamma globulin (mean, week 4) but not by late convalescent sera. TSST-1 was detectable in 2 of 26 acute sera and 1 of 22 control sera. No KD but 1 control serum had IgM to TSST-1. IgG seroconversion rates to TSST-1, SEA, SEB, and SEC were 10%, 15%, 21% and 16%, respectively. These data do not support the involvement of toxin-producing staphylococci in KD.

A flow-injection analytical system for the determination of citrate in food has been developed. Citrate lyase, oxalacetate decarboxylase, and pyruvate oxidase were used, and the latter two enzymes were co-immobilized. Hydrogen peroxide, which was produced by three enzyme reactions, was monitored amperometrically with a platinum electrode. The carrier solution used was 0.1 M phosphate buffer (pH 7.0) containing lOmM MgC12, 80pM thiamine pyrophosphate (TPP), and 10pM flavine adenine dinucleotide (FAD). The detection limit was 0.02mM, and the range of determination was 0.lLl.OmM. The relative standard deviation was 1.23% for 10 successive determinations at the 0.5 mM level. The determination frequency was about 15 tests per hour. The present system was applied to the determination of citrate in several fruits. The results showed good agreement with those obtained using a conventional method (F-kit method).

We measured serum cytokeratin fragment 21–1 (CYFRA 21–1) levels by a solid-phase immunoradiometric assay in 102 healthy Japanese women, and set the reference value at 1.9 ng/ ml (mean +2 SD of the serum levels based on a linear distribution). Pretreatment serum CYFRA 21–1 levels were also analyzed in 235 women with benign (n = 94) or malignant (n = 141) gynecologic disease, and were compared with the serum levels of CA 125 and SCC. The respective positivity rates for CYFRA 21–1 and CA 125 were 64.0 and 77.2% in ovarian malignancy, while they were 4.2 and 30.8% in benign ovarian masses. CYFRA 21–1 had an accuracy of 61.3% in diagnosing ovarian malignancy, which was higher than that of CA 125 (53.4%). The positive predictive value of CYFRA 21–1 for ovarian malignancy reached 94.1%, which was significantly (p < 0.005) higher than that of CA 125 (68.8%). These findings indicate the potential usefulness of CYFRA 21–1 as a tumor marker for ovarian malignancy. In addition, the positivity rates fo CYFRA 21–1 in cervical cancer (51.2%) and endome-trial cancer (52.2%) were also similar to the respective rates for SCC and CA 125, which suggests that CYFRA 21–1 seems to be a general tumor marker for gynecologic malignancy.

The frequency of HCV vertical transmission was 6.2%. Half of the carrier infants became seronegative for HCV-RNA. HCV vertical transmission was significantly affected by the maternal serum ALT level prior to delivery.

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Vasospastic angina was confirmed in a 13-year-old female patient at autopsy. The patient's only symptom was recurrent toothache, which began when she was 10 years old. In November 2000, she was evaluated at our medical center; however, all examinations were judged normal. Six months later, she suffered a severe toothache. She went to school the next morning after the symptoms improved. She lost consciousness at school and was given cardiopulmonary resuscitation but could not be revived. At autopsy, her three coronary vessels showed marked intimal hyperplasia, and multiple focal myocardial infarctions were observed in the lateral wall of the left ventricle. The patient's only clinical symptom was toothache and none of the physicians realized that this was caused by angina. Vasospastic angina begins at a young age and is one of the causes of sudden death in adolescents.