Natural cytotoxicity in immunodeficiency diseases: Preservation of natural killer activity and the in vivo appearance of radioresistant killing

Pierce, Glenn F.; Polmar, Stephen H.; Schacter, Bernice; Brovall, Charlotte; Hornick, David L.; Sorensen, Ricardo U.

doi:10.1016/0198-8859(86)90319-8

Cited by 10 publications

(3 citation statements)

References 36 publications

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“…Of the patients in the present study, 40% showed increased absolute numbers of NK cells. The NK activity has been suggested to have an important role in maintaining host resistance to viral infections in CHH patients with Band T-lymphopenia [17,18]. Our ®ndings partly support this idea, however, we did not ®nd any correlation between the numbers of NK cells and infectious history.…”

Section: Discussionsupporting

confidence: 71%

Susceptibility to infections and in vitro immune functions in cartilage-hair hypoplasia

Mäkitie

Kaitila

Savilahti

1998

European Journal of Pediatrics

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Section: Discussionsupporting

confidence: 71%

Susceptibility to infections and in vitro immune functions in cartilage-hair hypoplasia

Mäkitie

Kaitila

Savilahti

1998

European Journal of Pediatrics

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“…The degree of in vitro immunodeficiency, determined as in vitro responsiveness of lymphocytes to PHA, did not correlate with the severity of infectious problems; even marked T-cell deficiency was not always associated with infections. Pierce et al reported normal or supranormal levels of spontaneous natural killer cell activity in CHH patients [25,26] and suggested spontaneous natural cytotoxicity to be of major importance in these patients [24]. The incidence of malignancies is increased as observed in our series and others [7,8,31].…”

Section: Discussionsupporting

confidence: 63%

Cartilage-hair hypoplasia — clinical manifestations in 108 Finnish patients

1993

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Cartilage-hair hypoplasia is an autosomal recessive metaphyseal chondrodysplasia with short-limbed short stature, hypoplastic hair, and defective immunity and erythrogenesis. We have analysed the clinical outcome of 108 Finnish patients. Birth length was below -2.0 SD in 70% of the patients; the adult heights ranged from -11.4 SD to -5.2 SD. The sitting height percentage was increased in all but 4 patients. Six patients had normal hair. Increased ligamentous laxity was present in 95%, limited extension of the elbows in 92%, increased lumbar lordosis in 85%, thoracal deformity in 68%, genu varum in 63% and scoliosis in 21% of the patients. Defective cellular immunity had been observed in 88% and increased susceptibility to infections in 56% of the patients. Six patients had died of primary infections. The incidence of malignancies was 6%. Childhood anaemia had occurred in 79% of the patients. It was usually mild, but severe in 14 patients. Hirschsprung disease had been observed in 8, anal stenosis in 1 and oesophageal atresia in 1 patient. The intrafamilial variation of the syndrome was considerable as studied in 16 sibships.

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“…About half of the patients with Wiskott-Aldrich syndrome have normal NK cell activity against K562 target cells, but all of these patients have severely depressed NK cell activity against virus-infected target cells, despite normal IFN-a titers (405). Some patients with SCID show a depressed NK cell activity and some display normal or augmented activity (95, 399,404,405,[408][409][410][411]. In SCID patients with elevated NK cell activity, most circulating lymphocytes have the characteristics of Cllb+ LGLs (409-411).…”

Section: Congenital Defects Of Nk Cellsmentioning

confidence: 99%