摘要:

ABSTRACT.Clinical and laboratory evaluation of 60 boys with hemizygous, 12 girls with homozygous, and 11 girls with heterozygous erythrocyte glucose‐6‐phosphate dehydrogenase deficiency was made during haemolytic crisis. The main clinical symptoms were paleness, dark urine and oliguria. Only one patient needed peritoneal dialysis. Coexistence of glucose‐6‐phosphate dehyrogenase deficiency associated with haemoglobinopathy was found to be higher than expected (32 out of 83 cases). Also, the high prevalence of glucose‐6‐phosphate dehydrogenase deficiency among females with homozygous and heterozygous disease was surprising. The precipitating factors of haemolysis were variable. Rather than antimalarial and antipyretic‐analgesic drugs, infections seemed to be the main haem

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11386.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.The epidemiology of infantile hydrocephalus (IH) in the birth years 1983‐86 was investigated in the south‐western health care region of Sweden. The study was made as a continuation of a previous one which had shown a significant increase in the prevalence of IH in 1967‐82. That rise was entirely referable to the relatively larger number of preterm IH infants born in 1979‐82 and was considered to be due to the enhanced survival, especially of very preterm infants. The present series comprised 57 liveborn IH infants‐27 preterms and 30 born at term. The livebirth prevalence of IH was 0.64 per 1000, 0.30 for preterm and 0.34 for fullterm infants. The high prevalence of preterm IH infants in 1979‐82 had persisted, but had not increased further. This might indicate an improved outcome in preterm survivors in the period 1983‐86, as the survival rate had continued to increase. The striking predominance of a perinatal aetiology of IH in very preterm infants could be confirmed: 90 % had had intraventricular haemorrhages verified by ultrasound in the postpartum period. The outcome in very preterm surviving infants with IH was still alarmingly poor: 78% had cerebral palsy, 72 % mental deficiency, and

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11387.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Out of 109 children with infantile spasms (IS), prospectively tested during the years 1976 to 1979 in Denmark, 52 children were examined by cranial computed tomography (CT). The classification of IS into cryptogenic (CR), symptomatic (SY) and doubtful (DO) was done clinically without considering the CT‐findings. Sixty per cent of the scannings were abnormal. Only 6/30 (20%) of the children in ACTH treatment were found to develop cerebral atrophy which means that this finding is not an obligatory side‐effect of ACTH treatment of children with IS. Normal CT‐findings were found in 50% of the CR and 50% of the SY + DO‐groups, and could not be used as a prognostic tool for estimating the mental development. This was also the case for children with cerebral atrophy. Abnormal CT‐findings (minus atrophy) were highly correlated to the group with clinical symptoms and indicate an extremely unsatisfying long‐term mental prognosis. CT‐scanning is a valuable tool for the examination of clearing children with inf

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11388.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.The cerebrospinal fluid (CSF) concentration of the inhibitory neurotransmitter gamma‐aminobutyric acid (GABA) was analysed in 41 children with febrile convulsions (FC), 41 febrile controls of similar age (control group 1), and 59 controls, who had no fever and/or were outside the age range for FC (control group 2). A significant correlation between CSF‐GABA and age was demonstrated for controls (1 + 2) (r= 0.63, p<0.00001), as well as for patients with FC (r= 0.42, p= 0.003). Patients with FC did not differ significantly from control group 1 in respect to CSF‐GABA. Duration of FC was related to both CSF‐GABA and age (GABA: r=−0.29, p<0.05; age: r =−0.32, p1 year of age, a significant negative correlation between CFC‐GABA and body temperature was found (r=−0.34, p = 0.01). The low CSF‐GABA in the FC‐labile age group, the negative correlation of CSF‐GABA to body temperature, and the negative correlation of the duration of FC to both CSF‐GABA and age, all indicate that GABA could be of importance in th

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11389.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.The etiology of severe mental retardation has not previously been studied in Jordan. In a group of 203 mainly severely mentally retarded children born 1975–1985, we found the etiology to be related to two main factors: (1) sequele from high perinatal morbidity or meningitis in infancy leading to a combination of severe mental retardation and cerebral palsy; (2) a high degree of intermarriage and a high frequency of retarded siblings indicating that genetic causes of MR are common. The results underscore the importance of further development of antenatal and perinatal care, especially for poor people and the need of genetic councellin

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11390.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Of 1200 people randomly sampled from the total Finnish population, cerebral palsy was known to 95.4% and its meaning to 60.7%. Nine of ten (88.4%) would let their children play with a child suffering from cerebral palsy. A total of 48% stated that they would, other circumstances being equal, hire persons with cerebral palsy. Half (47.5%) of the subjects regarded it as possible and a further 28.0% as fully possible for individuals with cerebral palsy to make and maintain a family of their own. A good knowledge of cerebral palsy was independently related to a good basic education, age of more than 24 years, female sex and Finnish as native language. A positive attitude toward people with cerebral palsy was independently related to Finnish as native language and a good basic education. A good knowledge of cerebral palsy fostered a positive attitude toward people with this disease.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11391.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.Fifteen children with aortic stenosis were examined with Echo‐Doppler cardiography in order to evaluate the use of the continuity equation to calculate the aortic valve area. The results were compared to invasive estimates of valve area calculated with the Gorlin's equation. A close correlation between the two methods, r=0.94, was found and it is concluded that even in children an accurate estimate of valve area can be expected with the use of the continuity equatio

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11392.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.tb11393.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.tb11394.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

ABSTRACT.A 12‐year‐old girl, born full term after severe pre‐ and peripartal asphyxia, with transient abnormal newborn neurology and slight motor developmental deviations but no cerebral palsy syndrome, is described. At the age of 5, she developed a slowly progressive dyskinetic hemisyndrome, initially uncoordinated hyperkinetic movements in her left arm, dystonic posturing then supervened. Since the age of 11, her right side had also slowly become involved. As extensive examinations excluded other causes, it is concluded that this case represents a delayed onset dyskinetic ‘CP‐syndrome’ subsequent to a postanoxic non‐progressive insult to the basal ganglia, but with a wellcompensated functional integrity before

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1990.tb11395.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY