Search

We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early-onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly resistant to treatment, lidocaine and CBZ may prove effective in treating this disorder. Future studies should be performed in order to determine whether there are common specific mechanisms of seizure generation related to the sodium channel in these patients.

Fibroblast growth factor 23 (FGF23) is a potent regulator of Pi and 1,25-(OH)(2)D homeostasis. Early postpartum infants show intriguing changes in serum levels of Ca, Pi, PTH and 1,25-(OH)(2)D. However, the role of FGF23 in the early neonatal mineral metabolism has not been clarified. In order to evaluate the significance of FGF23 during the early postpartum period, we examined the circulating FGF23 levels using an intact FGF23 ELISA and a C-terminal FGF23 ELISA either in 22 umbilical cord blood samples (the cord blood) or in 22 term infants at 5 days of life (the 5-day-old infant). We also compared these ranges with those of 11 healthy adults. Data were expressed as mean+/-SD, and analyzed by two-way ANOVA, followed by the Tukey's test. C-terminal FGF23 in the cord blood, the 5-day-old infants and the healthy adults were 73.3+/-22.4, 81.0+/-28.2 and 39.0+/-7.8 RU/ml, respectively. Intact FGF23 in the cord blood, the 5-day-old infants and the healthy adults were 3.9+/-1.6, 21.8+/-17.6, and 27.6+/-7.3 pg/ml, respectively. Immunoprecipitation assays using anti-FGF23 antibodies demonstrated that the intact 32 kDa FGF23 was low and the fragmented FGF23 of 18 kDa was abundant in the cord blood compared with those in the healthy adults. In conclusion, our observations indicated that the intact FGF23/C-terminal FGF23 ratio was very low due to the fragmentation of FGF23 during the early postpartum period and might have a considerable contribution to the Pi homeostasis in the healthy term infants.

116 immunizations were given to 61 children with febrile convulsion or epilepsy who had not had a seizure for 1 year since the last attack. In 92 of the 116 immunizations the electroencephalogram (EEG) was examined before and after immunization. No adverse effects on the EEG were observed in 19 immunizations with Japanese encephalitis, measles, mumps or rubella vaccines. Epileptic spikes reappeared after 10 immunizations and epileptic spikes increased after 10 immunizations among 73 given for diphtheria, acellular pertussis and tetanus (DPT), diphtheria and tetanus (DT), or Bacillus Calmette-Guerin (BCG). A convulsion was observed once in one child 7 days after immunization with BCG. A follow-up EEG examination is necessary after children with convulsive disorders are immunized.

Antibody-dependent cellular cytotoxicity (ADCC) and natural killer (NK) activity were examined using MT-2 cells persistently infected by HTLV-1 as target cells, and mononuclear cells as effector cells, from healthy one-week-old newborn babies, infants, children and adults. More than 10% of ADCC was observed in 17 newborn babies out of 22 (77.3%) and in all 67 healthy one-month-old babies to adults, by adding serum from anti-HTLV-1 positive carriers. When anti-HTLV-1 negative serum was added, less than 10% of ADCC was observed. If infants without anti-HTLV-1 antibodies were breast-fed they had the possibility of HTLV-1 vertical transmission. There was no significant decrease in NK activity between 90 healthy newborn babies, infants, children, or adults. These results suggest that ADCC and NK activity protect against the transmission of HTLV-1 from mother to child.

Theophylline serum concentrations (TSC) in ten asthmatic children during RTC therapy were closely examined in order to compare the values for periods with and without acute asthma attacks.1) Average TSC during the periods without attacks (fluctuation from day to night) remained above 10μg/ml. on the contrary, during acute attacks, TSC decreased significantly (P

(1) 油性食品のレトルト殺菌後の品質変化を検討するために,ハンバーグを酸素透過性,光透過性の異なる6種類の包装材料に包装し, 30℃の暗所,明所および40℃暗所の各条件下で貯蔵し,過酸化物価, TBA値,官能検査によって品質の評価を行った。(2) 貯蔵中,塩化ビニリデン積層フィルムの酸素透過度は減少傾向をたどった。(3) 包装材料の酸素透過性は明らかに油脂の酸化および食味の低下に大きな影響を与えた。(4) 光線のもとで貯蔵した場合,明らかに品質劣化が促進されたが,その程度には包装材料の酸素透過性が関係していた。(5) 貯蔵温度は明らかに食味の低下に大きな影響を与えたが,化学変化には貯蔵温度の影響が明確には現われなかった。(6) 以上の結果から,油性食品をレトルト食品とした場合,その品質保持のためには酸素透過性の低い包装材料を用い,光線のあたらない条件下で貯蔵すべきであることを確認した。

Intravenous acyclovir and vidarabine were compared in the treatment of varicella-zoster virus (VZV) infection in 25 immunocompromised children--13 with acute lymphocytic leukemia, three with other types of cancer, two with immunodeficiency and in seven undergoing prednisolone treatment. Thirteen had varicella and 12 had herpes zoster. Acyclovir was given intravenously to five patients with varicella and to four with herpes zoster at a dose of 5-10 mg/kg every eight hours. Vidarabine was given intravenously to eight patients with varicella and to eight with herpes zoster at a dose of 10 mg/kg/day. In varicella, vidarabine significantly shortened the time from the start of treatment to cessation of new lesion formation compared with acyclovir. However, there was no significant difference in time to complete crusting between the two treatments. In herpes zoster, acyclovir significantly shortened the time from the onset of the skin lesions to complete crusting. A slight raise of GOT in two cases was reported. While acyclovir and vidarabine were equally effective for VZV infection, in herpes zoster acyclovir was more effective.

Two patients suffering juvenile rheumatoid arthritis (JRA) with the symptom of pericarditis who were found unresponsive to high doses of conventional oral steroid therapy, were theated with pulse therapy.The first patient was admitted to the hospital again at the age of 6 11/12 due to the third recurrence. This patient, taking oral dose of prednisolone 2mg/kg daily, showed no improvement, but instead, he developed pericarditis on the 11th day after the treatment.The second patient is a 14 year 11 month old girl who was admitted to the hospital due to muscle pain and pericarditis along with fever and rheumatoid rash, which lasted for two weeks prior to this admission. For both patients a large amount of pericardial effusion was revealed by echocardiogram. Development of cardiac tamponade was being feared and there were discussion on the indication of pericardiocentesis. Inorder to improve pericarditis and acute systemic symtoms both patients received conventional pulse therapy of methylprednisolone 30mg/kg/day intravenously for three consecutive days a week for three weeks.From the first day of pulse therapy, the high fever declined. Pericardial effusion disappeared within the three weeks. The patients showed significantly better clinical responses. No side effects were observed during the pulse therapy except a slight moonface.Pulse therapy is indicated when the patients are unresponsive to high doses of conventional oral steroid therapy or have a risk of cardiac dysfunction to be developed.