Ten years follow up study of steroid therapy for congenital encephalomyopathy.

Two 10-year-old boys with mental retardation and myopathy which were present since birth are described. Both had elevated serum creatine phosphokinase (CK) and one of them had a positive family history. The clinical features were consistent with Fukuyama type congenital muscular dystrophy, but muscle biopsies suggested an inflammatory process. Adrenal cortical steroids were given and they were followed up until 10 years of age. Serum CK showed a significant response to the treatment, and mental retardation in case 1 and motor dysfunction in case 2 improved. It is postulated that an inflammatory process might be a causative factor in some patients with congenital muscular dystrophy.

[A case of degenerative type of progressive myoclonus epilepsy].

This report describes a patient with degenerative type of progressive myoclonus epilepsy (PME), who showed slowly progressive deterioration of the central nervous system; intellectual impairment, dysarthria, and involuntary movements, particularly action myoclonus and dystonia. The patient was a 19-year-old woman who had no hereditary factors. At the age of 4, she developed action myoclonus in the upper limbs bilaterally. Her condition became gradually worse, and at the age of 15, she was admitted to our hospital because of involuntary movement in the upper limbs. First physical examination revealed mild mental retardation, action myoclonus, dystonia, and delayed adolescence. As giant SEP characteristic of PME and Ramsay Hunt syndrome was found, she was tentatively diagnosed as having Ramsay Hunt syndrome without epilepsy, and delayed adolescence. Now, she is 19 years old, and unable to walk alone because of involuntary movements and paralysis. But she has not developed epilepsy. As she has not been compatible with progressive myoclonus epilepsy (PME) and progressive myoclonic ataxia (PMA) classified by Marseille Consensus Group, she has been diagnosed as having an atypical PME syndrome.

[The study of allergic children under one-year old. The degree of influence on allergic factors to atopic dermatitis by multiple factor analysis].

We investigated the allergic factors of 94 allergic children; 69 atopic dermatitis, 21 wheegy children, 3 urticaria and 1 milk allergy child under one year by multiple factor analysis. The results were as follows; 1) Investigating by relative range ratio the 3 factors, RAST score to Dermatophagoides farinae and egg white, and family history of allergic disease, had strong influence to the existence of atopic dermatitis. 2) By multiple factor analysis type III, the 6 groups had tight relationship to atopic dermatitis group. (1) IgE RIST more than 61 IU/ml group. (2) Eosinophil counts more than 401/mm3 group. (3) RAST score to Dermatophagoides farinae more than 0.70 PRU/ml group. (4) RAST score to egg white more than 0.70 PRU/ml group.

[The study of allergic children under one-year old. The measurement of IgE and IgG4 antibody titers to egg white, milk and soybean].

We measured the IgE and IgG4 antibody titers to egg white, and soybean of 94 allergic children under one year. (21 wheezy children, 35 atopic dermatitis, 34 wheezy children with atopic dermatitis, and 4 other allergic children). The results were as follows; 1) The positive ratio of IgE antibody titers to egg white was higher than the other IgE antibody titers, and of IgG4 antibody titers to milk was higher than the other IgG4 antibody titers. 2) Of each allergic symptoms, the positive ratio of IgG4 antibody titers to milk was higher than the others in wheezy children group. And the positive ratio of IgE antibody titers to egg white and milk were higher than the others in atopic children and wheezy children with atopic dermatitis. 3) The positive ratio of IgE antibody titers to egg white and milk were higher than the others in the group showed IgE RIST more than 21 IU/ml, and then the positive ratio of IgE antibody titers to egg white, milk and soybean and IgG4 antibody titers to egg white were higher than the others in the group showed eosinophil counts more than 301/mm3.

[Investigation of the genetics in allergic children: the first report--Twin study on HLA in allergic disease].

Twenty-two twins' families were investigated to determine the influence of a genetic factor in the etiology of allergic diseases in children. Zygosity was confirmed by eight red cell blood types and HLA type. Eight pairs of monozygotic twins were divided by a probability of monozygosity as calculated from Essen-Möller's method. Concordance rate of allergic disease and asthma were 88% in MZ, 86% in DZ, 75%, 73% and that of positive rate of DF-specific IgE antibody were 60% in MZ, 58% in DZ. In DZ, not sharing the same HLA haplotypes, concordance of the positive rate of DF-specific IgE antibody was 0%. Concomitant coefficiency of total serum IgE levels were 0.593 in MZ, 0.063 in DZ. These data suggested that IgE production may be influenced by genetic factors.

[Investigation of the genetics of allergic children, the second report--family study to determine the major gene in allergic disease using affected sib pair method].

Twenty-six families with allergic siblings were investigated to define the mode of inheritance of asthma and DF specific IgE antibody production in children. The affected sib pair method was used to establish the linkage between disease susceptibility genes and HLA antigens. The affected sib pair method revealed that in asthmatic families chi 2 = 4.9 (no significance) and in DF-specific IgE positive families chi 2 = 6.2 (p less than 0.05). This study suggests that the gene of DF-specific IgE antibody production is linked to HLA haplotypes, but the mode of inheritance of DF-specific IgE antibody production couldn't be defined.

[The investigation of asthmatic children by multiple factor analysis. I. The relation between severity of asthma and allergic factors].

In a previous study, we analysed the severity of bronchial asthma in childhood by the class II multiple factor analysis method. At that time, the titers of IgE and IgG4 antibodies to the three major food (cow's milk, egg white, and soybeans) and inhalation antigens such as house dust and mites were, however, left unevaluated because of a lack of independence. The quantification of their relative ratios was introduced into the present study, which made it possible to compare their individual potential as risk factors. Exercise induced asthma had the most outstanding effects on the severity of bronchial asthma, followed by counts of peripheral eosinophils and house dust and mites, in this order. Regarding food antigens, the presence of IgG4 and IgE antibody to egg white was the most critical factor, following by titers of IgG4 to cow's milk and soybeans. Complications with atopic dermatitis and allergic rhinitis little affected the severity. Bronchial asthma becomes manifest through a complicated mechanism involving a variety of causative factors. The results given above suggest that the contribution of individual risk factors can be quantified.

[Clinical research of specific IgE and IgG4 antibody in allergic children. Correlation specific IgE antibody to specific IgG4 antibody to food allergen].

We analysed the correlation specific IgE antibodies to specific IgG4 antibodies to food allergens in 150 asthmatic children by Multiple Factor Analysis-Type II and examined the role of both antibodies. The following results were obtained. 1) Of soybean, there was the stronger influence power between specific IgE and IgG4 antibody than the other 2 food allergens. 2) IgE antibody to soybean had the strong influence power to IgG4 antibodies to egg white and cow's milk and IgG4 antibody to soybean had same influence power to IgE antibodies to egg white and cow's milk. So, the specific IgE and IgG4 antibodies to soybean may be play the role of "the bride" between specific IgE antibody group and specific IgG4 antibody group of food allergens.

[The investigation of asthmatic children by multiple factor analysis. III. The changes in allergic factors selected by multiple factor analysis in 3 anti-allergic during treated groups over one year].

We investigated the difference in the influence on 10 allergic factors of 3 anti-allergic drugs: azelastine, tranilast, ketotifen. We determined the ranking of 10 allergic factors using the relative range ratio calculated by Multiple factor analysis. The ranking as follows: (1) Eosinophil count (2) IgE RAST score to H.D. (3) IgG4 antibody titers to egg white (4) IgE RAST score to egg white (5) IgE RAST score to D.f. (6) IgG4 antibody titers to milk (7) IgE RIST (8) IgG4 antibody titers to soybean (9) IgE RAST score to soybean (10) IgE RAST score to milk. Then we split 122 asthmatic children (mean age: 9.3) into 3 groups and gave each group a different one of the 3 anti-allergic drugs for one year. From daily records of asthma attacks and clinical findings, we classified the children into improved and not-improved groups within their respective anti-allergic drug group, and we investigated the changes in and the change ratios of the 10 allergic factors by the Student t method over this one year. The results were as follows. 1) There was no difference in the chang of 3 allergic factors (Eosinophil count, IgG4 antibody titers to egg white and IgE RIST) among the 3 anti-allergic drug treated groups. 2) The specific IgE antibodies to H.D. and D.f. had a remarkable decline tendency in treated groups by azelastine and tranilast. 3) The specific IgE antibody to egg white had a remarkable decline tendency in treated group by ketotifen.

[The investigation of asthmatic children by multiple factor analysis. II. The influence of 17 allergic factors on atopic dermatitis and allergic rhinitis in asthmatic children].

We investigated possible influence of 17 allergy-associated factors on atopic dermatitis and allergic rhinitis using Multiple factor analysis in 150 asthmatic children. Atopic dermatitis was complicated in ninety-seven cases and allergic rhinitis in ninety-seven cases. 17 allergy-associated factors were as follows: 1) sex, 2) age, 3) onset age of asthma, 4) family history of allergy, 5) peripheral eosinophil counts, 6) IgE RIST, 7) IgE RAST score to egg white, 8) IgE RAST score to milk, 9) IgE RAST score to soybean, 10) IgG4 antibody titers to egg white, 11) IgG4 antibody titers to milk, 12) IgG4 antibody titers to soybean, 13) IgE RAST score to house dust, 14) IgE RAST score to Dermatophagoides farinae, 15) severity of asthma, 16) exercise-induced asthma, 17) atopic dermatitis or allergic rhinitis. We concluded as follows: 1) Factors which more strongly influenced both atopic dermatitis and allergic rhinitis were IgE RAST score to D.f., positive family history of allergy, IgE RIST and eosinophil counts. 2) Combination with high levels of IgG4 antibody to 3 food allergens such as egg-white, milk and soybean and IgE RAST to egg-white has a strong influence on atopic dermatitis, but high levels of IgG4 antibody to 3 food allergens except high level of IgG4 antibody to soybean have a weak influence on allergic rhinitis.

[Clinical research on specific IgE and IgG4 antibodies in allergic children. Correlation specific IgE and IgG4 antibodies to food allergens to other allergic factors].

We examined the correlation specific IgE and IgG4 antibodies to food allergens to other allergic factors in 150 asthmatic children by Multiple Factor Analysis-Type II. The following results were obtained. 1) 3 explanation variables (IgE RIST, Eosinophil count, Family history of allergy) had the strong influence power to 8 objective variables of food allergens. 2) By calculating the category score of explanation variables, the high IgE RAST score to Dermatophagoides farinae group had the strong influence power to the high score groups of IgE RAST and IgG4 antibody titers to food allergens. The fact indicated the linkage between specific IgE and IgG4 antibodies to food allergens to specific IgE antibody to Dermatophagoides farinae. 3) In the same way, the severe group of asthma had the strong influence power to the high score groups of IgE RAST and IgG4 antibody titers to food allergens, and specific IgG4 antibody group had a same directivity of specific IgE antibody group to the other allergic factors. So specific IgG4 antibody may play a "role of reaginic antibody".

[Study of IgE.IgG4 antibodies on eczema in infants. II. Clinical characteristics of infants with egg white.RAST 4+].

The author made an assessment in his first report, regarding relationship to the degree of skin involvement. As a result, it was suggested that the extent of eczema is a good index for determining the severity of the allergy and also that the severity of the allergy early in the infancy has a good correlation with egg white.specific IgE antibody. Therefore, an assessment had been made this time regarding the clinical characteristics of 32 infants who have strong reactions (egg white.RAST 4+) to egg white. The results were as follows; 1. All the infants with egg white.RAST 4+ had a systemic eczema, and their IgE antibodies showed high values of 445.6 +/- 626.9 IU/ml. 2. There were many cases with positive multiple allergens. Particularly, all the infants with egg white.RAST 1+ - 2+ were negative to rice and wheat, while 7 cases positive RAST score to rice and 9 cases to wheat were seen out of the 32 who showed the RAST 4+ to egg.white early in the infancy (rice; p less than 0.02 and wheat; p less than 0.01). 3. If the growth curves of the group from which egg and milk were completely eliminated are compared with the curves of the healthy infants, the height and head circumference were entirely in the normal range while the weight and Kaup index had a trend of being a little low, though they were within the normal range. 4. Regarding family history of allergies, past histories of atopic dermatitis on the mother's side were observed at a high rate.

[IgE.IgG4 antibody in eczema in infants between 5-7 months of age. I. Relationship to the degree of eczema involvement].

Assessments have been made of serum IgE and IgA value, specific IgE and IgG4 antibody titers to foods, house dust, mite and percent peripheral++ eosinophil count in 191 infants with and without eczema between 5-7 months of age. Eczema patients were divided into 4 groups according to the degree of eczema involvement and laboratory data were compared between these and not eczematous group. The results were as follows; 1) IgE antibody titers were higher as the degree of eczema involvement increased. 2) RAST positive rates to foods increased with the degree of eczema involvement. Positive rate was highest to egg white followed by milk, soybean, wheat and rice. 3) Only 9 cases were RAST-positive to rice or wheat and all of them had IgE antibodies to other allergens tested. 4) A value of 10 IU/ml, which is the lowest measurable value of IgE in infants was considered to be a little too high to speculate allergy to some foods. 5) Specific IgG4 antibodies were positive only to milk. 6) All the 18 cases with positive milk-specific IgG4 antibody were all negative in milk-specific IgE antibody, and conversely all the 14 cases with positive milk-specific IgE antibody were negative in milk-specific IgG4 antibody. From these results, it was concluded that food allergy is related to the degree of eczema involvement in infants between 5-7 months of age.

[A double blind study of the effectiveness of ketotifen in preventing the development of asthma in atopic dermatitis patients].

Many asthmatic children have experienced atopic dermatitis in their younger days. As it is very difficult to cure childhood asthma we attempted to determine the anti-allergic drug effects in preventing the development of asthma by using ketotifen on atopic dermatitis patients. The study was designed as a placebo controlled double blind trial of 128 atopic dermatitis patients aged from 2-34 months. 91 patients were given complete analysis in the study, 33 patients were given only a safety rate and 4 patients were dropped. The 91 patients were followed for 52 weeks. Our primary finding was that the development of bronchial asthma was inhibited in the ketotifen group compared to the placebo controlled group with a statistically significant degree (p less than 0.001). We also found that clinical symptoms of atopic dermatitis were significantly improved in the ketotifen group (p less than 0.001). Only 5 patients complained of mild side effects.

[Clinical epidemiology of bronchial asthma in children report. No. III. Comparison of prevalence of asthma with 1982 and 1990 and environmental factors].

UNLABELLED: In 1982, our group carried out a survey by the questionnaires and on the site medical check-ups to observe and occurrence of asthma and other allergic symptoms in elementary and junior high school children in Izu Ohshima island. Same procedure was repeated in 1990 for 1145 children of the same age group to see any data fluctuation. The occurrence of allergic symptoms with relation to living conditions was also researched. RESULT: 114 students (11.0%) had either suffered from asthma in the past, or were still currently exhibiting symptoms, this marked a 7.5% rise (108 students) over the 1982 research data. However, patients in need of current treatment numbered 48 (4.5%), which was similar to the 1982 data of 82 (4.3%). For other allergic symptoms, rhinitis showed a marked increase over the 1982 data. The relation between living conditions and asthma was analyzed by Multiple Factor Analysis Quantification Theory. RESULT: 1) Family history of allergy, 2) Selection of food products upon starting solids during infancy, 3) Infection of lower bronchus during infancy all seemed to have had much influence on the occurrence of asthma.

[Clinical significance of specific IgG4 antibody in serum].

Specific IgG4 antibodies in sera were measured by ELISA in allergic patients who were diagnosed as susceptible to one or more allergens among mite, milk, soybean or egg white, and also in a non-allergic control group, and their diagnostic significance was investigated. The results obtained were as follows. 1. The level of specific IgG4 antibody was significantly higher in each allergic group than in the non-allergic group. 2. The positive rates in the specific IgG4 antibody determination were higher than those in RAST in the milk-, soybean- and egg white-allergic groups. 3. In each allergic group, the causative allergens were detected more accurately by measuring both specific IgG4 antibody and IgE antibody (RAST) than IgE alone. 4. The positive rates in the specific IgG4 antibody determination were higher than those in the skin test in each allergic group. 5. It was demonstrated that the combination of the skin test with specific IgG4 antibody measurement ensured a more accurate detection of causative allergens than the skin test alone. These results indicated that the measurement of the specific IgG4 antibody is a helpful method to detect the causative allergens in allergic patients.

[Does clinical stage have any influence on the results of provocation tests in allergic children? I. The best clinical stage for food provocation tests].

To investigate the best clinical stage for food provocation tests, twenty allergic patients who had early allergic reactions induced by any kind of food were tested. The patients were classified into two groups. Group I; open-food challenge tests were performed on eleven patients within 48 hours of admission. Group II; the tests were performed on nine patients seven or more days after admission. In these food provocation tests, there were nine positive results in Group II (p < 0.05). The percentage of eosinophils in the blood was higher in cases of positive provocation than in cases of negative provocation (p < 0.05). Moreover the percentage of eosinophils decreased after admission day by day in the provocation-negative patients of Group II, but there was no change in the provocation-positive patients. These results suggest that individual sensitivity to food decreased after admission. Therefore, in order to assess the actual sensitivity of allergic children it is necessary that food provocation tests be performed within two days of admission or before admission.