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Literature DB >> 10685928

Proteus syndrome and immunodeficiency.

D Hodge¹, S A Misbah, R F Mueller, E J Glass, P A Chetcuti.

Abstract

A 10 year old boy with Proteus syndrome presented with a pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the Proteus syndrome.

Entities: Chemical Disease Species

Mesh：

Substances：
Immunoglobulin G

Year: 2000 PMID： 10685928 PMCID： PMC1718237 DOI： 10.1136/adc.82.3.234

Source DB: PubMed Journal: Arch Dis Child ISSN： 0003-9888 Impact factor: 3.791

4 in total

1. Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin.

Authors: T A Waldmann; W D Terry
Journal: J Clin Invest Date: 1990-12 Impact factor: 14.808

2. Proteus syndrome.

Authors: K Sayama; N Hato; O Matsuda; S Shiraishi; Y Miki
Journal: Dermatology Date: 1994 Impact factor: 5.366

3. Immunological study in primary intestinal lymphangiectasia.

Authors: D Heresbach; J L Raoul; N Genetet; P Noret; L Siproudhis; M P Ramée; J F Bretagne; M Gosselin
Journal: Digestion Date: 1994 Impact factor: 3.216

4. The proteus syndrome. Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections.

Authors: H R Wiedemann; G R Burgio; P Aldenhoff; J Kunze; H J Kaufmann; E Schirg
Journal: Eur J Pediatr Date: 1983-03 Impact factor: 3.183

4 in total

5 in total

Review 1. When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach.

Authors: S Jolles; H Chapel; J Litzman
Journal: Clin Exp Immunol Date: 2017-01-30 Impact factor: 4.330

2. Proteus syndrome: evaluation of the immunological profile.

Authors: Vassilios Lougaris; Vincenzo Salpietro; Maricia Cutrupi; Manuela Baronio; Daniele Moratto; M R Pizzino; Kshitij Mankad; Silvana Briuglia; Carmelo Salpietro; Alessandro Plebani
Journal: Orphanet J Rare Dis Date: 2016-01-13 Impact factor: 4.123