ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00671.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractEighty‐four patients with chronic granulomatous disease, who were registered in the All Japan Immunodeficiency Registry, were analysed for their clinical features.Family history, presumed mode of inheritance, age of onset, prognosis, major symptoms (particularly infections), complications and some laboratory data were summarized and discussed in comparison with some previous reports. This is the first epidemiological report on chronic granulomatous disease from the Orien

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00672.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractCytochrome b558is probably a component of an electron transport chain which is called NADPH oxidase. Cytochrome b558from the neutrophils of two male patients with chronic granulomatous disease (CGD) and their parents was studied. One of the CGD patients had decreased amounts of cytochrome b558, The α‐band of light absorption of cytochrome b558at 558 nm was found as a trace, and the peak of the γ‐band was shifted to 420 nm, whereas the normal γ‐band is at 426 nm. Intermediate amounts of cytochrome b558were present in his mother, whereas his father had a normal amount. Another patient had normal peaks of α‐ and γ‐bands of cytochrome b558. Thus, abnormalities in NADPH oxidase of the two patients

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00673.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractPolymorphonuclear leukocytes (PMNs) obtained from a chronic granulomatous disease (CGD) patient, pooled serum and bacteria were preincubated. CGD PMNs containing viable bacteria were then incubated for 60 minutes with either DL‐8280, SMX, TMP, ABPC, or GM.Only DL‐8280 killed almost all intracellular and extracellularE. coliON2, whereas other antimicrobial agents eradicated only extracellularE. coli. In contrast,Staph. aureuswas not killed by direct exposure to DL‐8280 for 60 minutes. This method using CGD PMNs preincubated with bacteria is useful for studies of the bactericidal agents penetrating into PMNs and inhibiting intracellular bacteria. DL‐8280 did not affect the control PMNs function when preincubated with PMNs. According to these data, DL‐8280 is able to penetrate CGD PMNs and kill the intracellul

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00674.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractThis paper describes a male infant, the younger brother of a girl previously reported to suffer from recurrent skin infections, with an abnormality of neutrophil adhesion to a surface and a lack of two neutrophil membrane proteins, gp 110 and gp 115. The male infant, now seven months old, suffers from a disease identical with that of his sister. Their parents, both healthy, have reduced levels of the neutrophil glycoproteins.Scanning electron microscopy of the infant's neutrophils attached to a glass surface revealed a considerable reduction of pseudopods compared to a normal control. His neutrophils suspended in PBS, however, were morphologically normal. When stimulated in suspension by N‐formyl‐methionyl‐leucyl‐phenylalanine, the neutrophils also showed normal morphological changes. These findings indicate that the function of the cytoskeleton of the neutrophils is not impaired, but the function of the cell surface membrane is defective.Neutrophil adhesion assay using adhesion rate to nylon fibers as an indicator revealed both siblings and their parents as having a reduced adhesion rate. This finding, combined with the fact that the disease occurred in a sister and brother, lends further support to an autosomal recessive mode of inheritance of the

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00675.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractWe studied monocyte chemotaxis in children with various types of primary immunodeficiency syndrome. The clinical and immunologic features of the patients with defective monocyte chemotaxis were then analyzed. Monocyte chemotaxis was defective in two patients with a monocyte disorder characterized by chronic candidiasis, absence of delayed‐type hypersensitivity, and normal T cell functions. Monocyte chemotaxis was also impaired in a patient with Chédiak‐Higashi syndrome, and in two of six patients with hyper‐IgE syndrome. Major infections in the patients with defective neutrophil chemotaxis but normal monocyte chemotaxis included recurrent pyogenic infections such as otitis media, superficial abscess, lymphadenitis and pneumonia. On the other hand, the clinical features of the patients with defective monocyte chemotaxis were characterized by chronic candidiasis. Delayed‐type hypersensitivity was negative in all of the patients in whom monocyte chemotaxis was defective, despite the absence of apparent T cell dysfunctions. These results demonstrated that there is defective monocyte chemotaxis in some types of primary immunodeficiency syndrome. The monocyte chemotaxis defect is probably responsible for candidiasis and related to the absence of delayed‐type hypers

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00676.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractComplement‐mediated opsonin activity was measured by the whole blood chemiluminescence method in 17 children with leukemia and lymphoma (6 with ALL, 7 with ANLL and 4 with non‐Hodgkin's lymphoma) during remission induction therapy. The opsonin activity, which was at the normal level before chemotherapy, was decreased in all of the children during the therapy. This decrease was especially remarkable in the children treated with L‐asparaginase. When the opsonin activity was decreased, the activities of other complement systems (C3, CH50) were also damaged. Among these activities, the opsonin activity was best maint

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00677.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00678.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00679.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

AbstractThe authors analyze and discuss the clinical and immunological features of hyper IgE syndrome in 40 patients suffering from the disease in Japan. There was no geographical difference observed and the sexual difference was in a ratio of 2 men to 1 woman. The syndrome appears early in infancy and begins mostly with skin lesions. The age at diagnosis is given in Fig. 1.Staphylococcus aureusis detected in every case as the pathogenic microorganism. Although the majority of the cases are of superficial infections, some cases are severe or of deep infections which become critical.The serum IgE levels are above 1,000 IU/ml in all cases and often allergic diseases are a complication. Neutrophil chemotactic defect is considered a secondary defect because a decreased in vitro chemotaxis may turn to normal skin window tests in vivo.These results indicate that lesions in lymphocytes and polymorphonuclear cells (PMNs) are not a complete defect but an incomplete defect or quantitative decline.While lymphocyte and PMN functions are damaged to various extents, the decrease or defect in the specific protective function against staphylococcus aureus is discussed.With the abnormal IgE production, specific IgE antibodies to staphylococcus aureus are produced as well as IgE antibodies to other allergens with frequent chances of sensitization.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1985.tb00680.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY