ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05042.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.In Gaucher disease there is a deficiency of the lysosomal enzyme, cerebroside‐β‐glucosidase, as a result of which cerebroside (glucosylceramide) accumulates in various organs. In northern Sweden 22 patients with a juvenile form of this disease have been identified. In one such patient, a girl of 10 years, we have attempted enzyme replacement by renal transplantation. After this operation the hepatic glucocerebroside content fell significantly. In another child afflicted with Gaucher disease in whom splenectomy was performed for severe splenomegaly and hypersplenism there was a progressive increase in the level of this lipid. These findings suggest that enzyme replacement was achieved by transplantation of a normal o

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05043.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.Phase reactants were evaluated prospectively in babies suspected of having sepsis. Among 318 babies, there were 22 proven and 10 “very probable” cases of neonatal sepsis. Of the proven cases 14 survived and 8 died. The survivors had a positive latex C‐reactive protein (CRP) in 11 cases and an alpha1‐acid glycoprotein (AGP) level greater than 0.5 g/l in 12 cases. Among those who died, one had a positive latex CRP and none had AGP>0.5 g/l. These findings were supported by positive CRP and elevated AGP in almost all “very probable” cases, all of whom survived. These data in newborn infants support the hypothesis that acute phase reactants have a functional role in combatin

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05044.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.In 1958–1974 altogether 46 cases of bacteriologically verified infection of Listeria monocytogenes were diagnosed during pregnancy and the neonatal period. Eight pregnancies resulted in abortion and three in stillbirth. Thirty‐seven children were born alive, 17 of whom died, all but one within a few days. These children were divided into three groups according to their age at the onset of illness: 22 cases with “Early disease” (≤2 days), four cases with “Intermediate disease” (3–5 days) and eight cases with “Late disease” (≤6 days). Three children were apparently healthy. Septicemia or “Granulomatosis infantiseptica” dominated in “Early disease” and claimed as many as 13 deaths. In “Late disease” all the children had meningo‐encephalitis, and only one of them died. The symptoms were typical of purulent meningitis. The group of “Intermediate disease” consisted of overlapping cases of the other two groups. Ampicillin alone or combined with gentamicin seemed to be the drug of choice in the therapy of neonatal listeriosis. Of the surviving children, two were seriously damaged and two had moderate injuries. Fifteen children are apparently healthy. In cases where pregnancy terminated in abortions, stillborns or children with “Early disease”, the mothers often showed signs of infection. The mothers of the children with “Late disease” were apparently healthy. These children were infected from other sources, some of them nosocomially. If listeriosis is diagnosed during pregnan

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05045.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.Serum from normal persons and from 133 cystic fibrosis patients was examined for precipitins against faecal bacteria (E. coliandB. fragilis) and againstP. aeruginosa, by means of crossed immunoelectrophoresis. 27% of the normal sera contained 1–2 precipitins againstE. coli, 16% contained one precipitin againstB. fragilisand 6% contained one precipitin againstP. aeruginosa.In sera from cystic fibrosis patients, there was a significantly increased prevalence of precipitins againstE. coli(76%),B. fragilis(38%) andP. aeruginosa(63%), and the mean number of precipitins against each of these bacteria was 3 (range: 1–12), 1.4 (range: 1–4), and 16 (range: 1–60), respectively. Increased numbers of precipitins againstP. aeruginosawere correlated with chronic lung infection caused by this species and with poor prognosis, whereas such associations were not revealed as regards precipitins against the two other bacterial species. Increased numbers of precipitins againstE. coliandB. fragiliswere significantly associated with increased numbers of precipitins againstP. aeruginosa.It is suggested that this association is due to immune reactions in the intestinal mucosa, resulting in increased absorption of antigens from

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05046.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.Cell‐mediated immunity (CMI) was studied in a group of 48 children with urinary tract infections (UTI) using a whole blood micromethod for lymphocyte stimulation in vitro. The patients were subdivided into pyelonephritis group (27 cases) and lower urinary tract infection (LUTI) group (21 cases) on the basis of fever, erythrocyte sedimentation rate, C‐reactive protein and renal concentration capacity. At the acute stage of infection the lymphocyte responsiveness to leucoagglutinin (LA) and concanavalin A (Con A) was suppressed in both groups, but the suppression was much greater in those with pyelonephritis. By 6 weeks after infection the lymphocyte responses were normal in most but not all cases. We conclude that an acute pyelonephritis is associated with marked suppression of CMI and that the latter can be used as an additional criterion for establishing the level of infection. Patients with UTI did not generally appear to have any primary defect of CMI but when suppression of CMI was present, it seemed secondary to an ongoing infect

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05047.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.A serological investigation on infants with incidentally detected cytomegalovirus (CMV) excretion was made in an attempt to differentiate between congenital and acquired infections. Generally, each of the patients, 0–12 months old at the detection of CMV‐excretion, was studied by indirect immunofluorescence (IIF) test for IgM‐antibodies in cord serum and by complement fixation (CF) test performed on cord serum and a number of sera drawn after the detection of CMV. Two out of 4 patients with virologically confirmed congenital CMV‐infection, as shown by positive virus isolation within 1 week of age, had a positive CMV‐IgM‐test in cord serum. One of these two children also had a persistently high CMV‐CF‐titer from birth until 2 months of age, indicating congenital infection. In the remaining 46 cases, all with the CMV‐excretion detected after 3 weeks of age, in half of the cases after 5 months, no positive IgM‐reaction was recorded in cord serum. No persistently high CF‐titer could be demonstrated among 13 out of the 46 patients from whom sera were drawn at birth and at 1–4 months. Six out of these 13 patients had a CF‐titer rise after the period of 1–4 months, indicating acquired infection. However, also one of the congenitally infected children had a similar titer increase. Many patients lacked characteristic serological patterns, some of them in spite of access to sera drawn at birth, as well as at 1–4 months of age and later on. It could be concluded that the possibility of making a serological distinction of congenital and acquired infant CMV infection found, by chan

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05048.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.We studied the intestinal morphology, and the jejunal and rectal immunoglobulins of 16 children with partial IgA deficiency, defined as serum IgA concentration more than two standard deviations below the mean for age, but higher than the lower limit of sensitivity of single radial immunodiffusion (0.02 g/l). Five of the patients had been treated with phenytoin, 2 had juvenile rheumatoid arthritis, 2 had ulcerative colitis and 5 had recurrent upper respiratory tract infections. The jejunal morphology was normal in every case. In 6 cases normalization of serum IgA occurred during the follow‐up, while in one patient with ulcerative colitis the concentration fell below 0.02 g/l. In patients with recurrent infections, there was a decreased frequency of infections when the level of serum igA increased. In 4 patients, IgM‐containing cells predominated in both the jejunal and rectal mucosa, and IgM was increased in the intestinal juice. In 6 patients a significant increase in IgM‐containing cells or a decrease in IgA‐containing cells or both were seen in either the rectal or jejunal mucosa. There was no correlation between the number of IgA‐containing cells in the intestinal mucosa and the serum lev

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05049.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.A prospective study of the course of breast feeding was carried out in 75 randomly selected women. Weekly interviews were performed from the day after delivery until the termination of breast feeding, but in no case for longer than 6 months. In each case a detailed analysis was made of the factors leading to transient lactation crises or to complete cessation of breast feeding. A second group of 71 mothers, also randomly selected, was interviewed in retrospect only, 6 months after delivery, and served as controls. Twenty‐four weeks after delivery 47% of the mothers in the weekly interview group were still breast feeding. The corresponding figure in the control group was 38%. In both groups only few mothers terminated lactation for medical reasons, while about one fourth stopped for some other reason and about one half because of a combination of factors. Brief case reports are presented to illustrate how varying the factors were that threatened breast feedin

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05050.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY

摘要:

Abstract.During a period of one year a questionnaire was filled in by parents of children who were more than one year old and admitted to the paediatric department in Randers, Denmark. The questionnaire was especially concerned with factors describing the social background of the family and with selected life events. Family background variables such as low level of parents' education and low income were found to be associated with psychosomatic and psychic diseases of the children. No associations between these negative family background variables and respiratory tract infections were found. The data show a strikingly high frequency of selected life events. Noticeable intercorrelations were found between social factors, life events, and diagnostic groups. In the light of this, it was not considered meaningful to analyze life events independently of the family background variables. A combined measurement for family background variables and selected life events was strongly associated with psychosomatic and psychic diseases. The additional information due to life events over the information due to family background variables was pronounced. It was remarkable that stressful life events also were frequent among children with some somatic diseases. The inference of these results is a plea for application of analysis of individual social history to more hospitalized children. The results might further have implications for preventive work with respect to the changes which seem to have taken place in important social conditions.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1979.tb05051.x

出版商:Blackwell Publishing Ltd    

年代:1979

数据来源: WILEY