摘要:

ABSTRACT.Psychological studies in children with stanted growth have partly shown inconsistent results due to heterogeneity of samples and the use of non‐standardized test procedures. We have examined patients with proven hypopituitarism using a selection of standardized and age‐related psychological tests. Forty‐eight patients, 32 male, 16 female, age 6 to 26 years with isolated growth hormone deficiency (n = 22) and multiple pituitary deficiencies (n = 26) were investigated. The patients had had hormonal substitution therapy for 0.1‐16.8 years. Test procedures included evaluation of intelligence and various personality parameters. Full score IQ was in the average range. Despite these findings a high percentage of patients had delayed schooling or had to repeat a class. They were normal regarding emotional balance and frustration tolerance and did not show any tendency towards depression or psychosomatic disorders. Social behavior was characterized by an infantile attitude toward their personal environment. The patients showed a tendency to avoid aggressiveness in frustrating situations. In contrast to the results of these tests, a non‐standarized interview demonstrated that the majority of patients had serious problems because of their short stature, including difficulties in finding social contacts. Continuous medical and psychological guidance is therefore re

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11529.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.The renal acid excretion of eight children with salt‐losing congenital adrenal hyperplasia, was studied in three different situations: before treatment (period I), under glucocorticoid therapy (period II) and when both glucocorticoid and mineralocorticoid were given as replacement treatment (period III). Although administration of glucocorticoid therapy alone allowed the correction of acidemia, normalization of urinary net acid excretion was achieved only after mineralocorticoid was added to the treatmen

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11530.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Methionyl growth hormone (somatrem) in a daily dosage of 4 IU/m2body surface area was administered to 16 girls with Turner syndrome. Low dose ethinyl estradiol (0.1 μg/kg body weight) was added in girls aged 13 years or more. Mean (SD) height velocity increased from 3.4 (0.9) to 7.2 (1.7) and 5.3 (1.3) cm/year in the first and second year, respectively. Bone age advanced 1.8 years over 2 years and predicted adult height was increased. Apart from the occurrence of anti‐GH antibodies there were no side effects. In conclusion, somatrem is an efficacious and safe therapy for short stature in Turner syndrome over a period of 2 years. Longer follow‐up is needed before conclusions about its effect on final height can be d

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11531.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.The dependence of bronchial reactivity to exercise on baseline lung function was studied in 84 asthmatic children aged 7–16 years. The percentage fall in PEF values after a standard exercise running test was 8.4± 8.6 % in the 39 children with an attack rate of less than 10 per year and 29.6±23.3% in the 45 children with an attack rate of 10 or more per year (p<0.001). A significant negative correlation (r=‐0.46, p<0.001) was found for pre‐exercise MMEF and other sensitive tests of airway calibre with the response to exercise, but no such correlation was found between baseline PEF or SGawvalues and the exercise response. These results show that bronchial hyperreactivity to exercise is dependent on residual airway obstruction, but a wide variety of reactivity can occur. If the baseline flow‐values are less than three standard deviations below the mean reference, however, a clinically significant response to exercise can be

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11532.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Several reports have suggested a relationship between atopy and coeliac disease and atopy has also been linked to the pathogenesis of the mucosal damage. Conclusive epidemiological evidence of the relationship has not been satisfactorily established. The case‐control study reported here was undertaken to test the hypothesis that coeliac disease is linked to atopy. Eighty‐two coeliac disease cases and a group of 180 age matched controls and all their first degree relatives were investigated for atopy. Siblings of cases reported an increased prevalence of food intolerance, compared to siblings of controls. No increase in asthma, eczema, rhinitis, conjunctivitis, cow's milk protein allergy (CMPA) were detected in relatives of cases, compared to those of controls. When each index case and each control were investigated no increased prevalence of atopic conditions was found. Skin prick testing to major allergens was positive in a similar proportion of cases and controls. Serum total IgE of a random sample of cases and controls showed no difference in mean values. This study supports the null hypothesis: there is no difference in the prevalence of atopy in cases affected by coeliac disease and their relatives, compared to controls and their relatives. The sources of possible bias in previous reports are discus

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11533.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.We describe the characteristics of a group of 140 children with intussusception. They differed strikingly from the classical picture of intussusception given in textbooks and in publications concerning large series. We found a low incidence of intussusception especially in infants and young children. There were far more children of older ages, the delay in diagnosis was significantly longer and there was a very high percentage of leading points. These factors explained our low hydrostatic reduction rate. The weight of most children was under the fiftieth percentile. The percentage of small bowel intussusceptions was somewhat higher. These differences are important in the light of early diagnosis and evaluating the results of treatment.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11534.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Three children, two girls and one boy, with Gaucher disease of the Norrbottnian type were treated by allogeneic bone marrow transplantation (BMT). Two of the donors were heterozygotes for Gaucher disease, and the third did not carry the gene. Engraftment was achieved in all three children but the boy became a chimera. The children have been followed from 1.5 to 6 years after transplantation. In each case the outcome was favourable, and in two children (patients 1 and 3) the BMT has been life saving. In all three cases a positive biochemical effect was apparent. Almost all biochemical parameters normalized within one year. The authors conclude that BMT is the treatment of choice in severe cases of Gaucher disease. BMT should be considered early in the course of disease and vigorous efforts made to find a suitable donor.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11535.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Two age‐matched groups of patients with sickle cell anaemia (SS), one with persistent gross splenomegaly (PGS) and the other without palpable spleens, were studied to determine the clinical and laboratory features associated with PGS. The group with PGS had significantly larger mean liver size, higher serum indirect bilirubin levels, but lower mean haematocrit, blood cell counts and serum albumin : globulin ratio when compared to the control group without splenomegaly. Large palpable livers (>6 cm below the coastal margin) among the PGS group tended to be associated with digital clubbing. A retrospective analysis of their case files showed that patients with PGS had had predominantly anaemic crises while those without splenomegaly had had predominantly vaso‐occlusive crises over a 2‐year period. No difference was found between the 2 groups, in respect of anthropometric data, prevalence of hepatomegaly or frequency of hospital admissions and blood transfu

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11536.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Children aged 0 to 6 years old admitted to hospital due to an accident were registered. Registration was carried out for two yearly periods; September 1982 to September 1983 and June 1985 to June 1986. The incidence of accidents dropped from 125 per 1000 a year to 116 accidents per 1000 children a year. The youngest children showed the most significant decrease in incidence. Home accidents were the most common type of accidents in both registration periods. Accidents due to falling from heights and poisoning accidents increased in the second period. Children injured during the second registration period required 3.4 times fewer days of hospitalization compared with the first period. Fractures and thermal injuries demanded most hospital care in both registration periods.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11537.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11538.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY