Leiner's disease and deficiency of C5

Miller, Michael E.; Koblenzer, Peter J.

doi:10.1016/s0022-3476(72)80151-3

Cited by 30 publications

(33 citation statements)

References 3 publications

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“…Furthermore, since our case showed severe seborrhoeic dermatitis, but did not have persistent diarrhoea or failure to thrive, it must be possible to have a partial form of Miller's syndrome, and the statement of Miller and Koblenzer (1972) that 'a diagnosis of seborrhoeic dermatitis, no matter how severe, is not in itself enough to extablish a diagnosis of Leiner's disease', needs to be modified.…”

Section: Discussionmentioning

confidence: 95%

“…The brother of one case also had low immunoglobulins attributed to transient immunodeficiency, but patients with low immunoglobulins did not show the opsonization defect. Miller and Koblenzer (1972) stressed the importance of diarrhoea for diagnosis of Leiner's disease. It is possible that diarrhoea itself might be associated with a protein-losing enteropathy and consequent deficiency of IgG and other proteins.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).

Evans¹,

Holzel²,

MacFarlane³

1977

Archives of Disease in Childhood

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).

Evans¹,

Holzel²,

MacFarlane³

1977

Archives of Disease in Childhood

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“…Subsequently Miller and colleagues [59,60] proposed that the cause of this yeast opsonisation defect was a functional inadequacy of the C5 component of complement and that this was the basic defect underlying Leiner's disease [43]. The association of the opsonic defect with both C5 dysfunction and Leiner's disease proved to be incorrect [69,18], although the molecular mechanisms involved continued to remain obscure for several years.…”

Section: Common Opsonic Deficiency and Mbpmentioning

confidence: 99%

Mammalian lectins in activation and clearance mechanisms involving the complement system

Reid,

Turner

1994

Springer Semin Immunopathol

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CRP and the mammalian lectins containing collagen-like structure (collectins) can clearly participate in a variety of antibody-independent recognition and clearance mechanisms which result in the neutralisation and elimination of pathogenic organisms (Fig. 2). Only CRP and MBP appear to have the capacity to activate complement, and while all the collectins (MBP, SP-A, SP-D and conglutinin) can utilise the C1q receptor, only conglutinin shows specificity for iC3b. The C-type lectin domains in the globular heads of the collectins have the capacity to recognise a range of specific carbohydrate structures which are found on the surfaces of pathogens commonly associated with infections in blood, lung and amniotic fluids. The proposed presentation of the carbohydrate-collectin complex to C1q receptors, via the collagen-like regions in the collectins is an attractive hypothesis for the triggering of protective mechanisms and there are already a number of publications which support this view. However, the precise manner by which these collagen 'stalks' interact with the C1q receptor and the complete characterisation of the receptor on a variety of different cell types remains to be elucidated. Similarly, whether or not there is a role for any of these lectins in autoimmunity/immunopathology has not, as yet, been addressed.

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“…The clinical entity was first described by Leiner in 1908 and emphasized later by Miller et al [154][155][156] who demonstrated deficient opsonic activity for yeast particles in such a patient's serum. These investigations associated this disease with an abnormality of C5 function.…”

Section: Defects Of the Complement Systemmentioning

confidence: 99%

Treatment of the immunodeficiency diseases — progress toward replacement therapy emphasizing cellular and macromolecular engineering

Pahwa

O’Reilly

et al. 1978

Springer Semin Immunopathol

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An increasing challenge to physicians is represented by a sizable population of patients who suffer from immunodeficiency diseases. These diseases may involve deficiencies or imbalances of the specific immunologic defenses represented by the T-cell-mediated immunities, B-cell-mediated or humoral immunities, major biologic amplification systems of either cell-mediated or humoral immunities, and the fundamental effector mechanisms. The consequent extraordinary heterogeneity of these diseases implies that curative, corrective, or even replacement and supportive therapy, to be most effective, must be based on fundamental understanding of the nature of each disease, its specific diagnosis and analysis of its pathogenesis. Although much progress has been made toward understanding these diseases over the past 25 years, analyses are often incomplete, sometimes controversial, and occasionally entirely lacking, rendering therapeutic efforts less than ideal. Nonetheless, dramatic advances in therapy have been forthcoming. With rapidly improving understanding of the basic deficiencies underlying the diseases and with improvement in the therapeutic armamentarium, one of the most rewarding and indeed dramatic branches of medicine has been that derived from continuing efforts to treat patients with primary immunodeficiency diseases. It will be our purpose in this chapter to outline this rapidly developing field from a focus in our own experience with large numbers of patients having all currently recognized forms of primary immunodeficiency. It seems likely, from data and new therapeutic agents already at hand and under clinical and laboratory investigation, that the therapy of primary immunodeficiency will continue to develop rapidly in the years ahead and will surely be an important guide to improved treatment of secondary immunodeficiencies as well.

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Leiner's disease and deficiency of C5

Cited by 30 publications

References 3 publications

Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).

Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).

Mammalian lectins in activation and clearance mechanisms involving the complement system

Treatment of the immunodeficiency diseases — progress toward replacement therapy emphasizing cellular and macromolecular engineering

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