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Neutrophil actin dysfunction(NAD)

MedGen UID:
338036
Concept ID:
C1850380
Disease or Syndrome
Synonym: NAD
 
Monarch Initiative: MONDO:0009755
OMIM®: 257150
Orphanet: ORPHA625

Definition

Neutrophil actin dysfunction (NAD) is an immunologic disorder characterized by early onset of recurrent infections, including oral, skin, and respiratory. Organisms are mainly bacterial and fungal. Patients tend to develop fever and hepatosplenomegaly with continued infection; bone marrow transplant is an effective treatment. The disorder results from impaired neutrophil mobility and chemotaxis associated with abnormal actin dynamics. Although a causative mutation has not been identified, studies have shown an association between the disorder and increased levels of a 47-kD F-actin-binding protein known as LSP1 (153432) and decreased levels of an unknown 89-kD protein (summary by Coates et al., 1991 and Howard et al., 1998). [from OMIM]

Clinical features

From HPO
Recurrent bacterial infections
MedGen UID:
334943
Concept ID:
C1844383
Finding
Increased susceptibility to bacterial infections, as manifested by recurrent episodes of bacterial infection.
Abnormality of metabolism/homeostasis
MedGen UID:
867398
Concept ID:
C4021768
Finding

Professional guidelines

PubMed

Halis H, Gunes T, Korkut S, Saraymen B, Şen A, Bastug O, Öztürk A, Kurtoğlu S
Med Hypotheses 2016 Sep;94:77-80. Epub 2016 Jun 14 doi: 10.1016/j.mehy.2016.06.017. PMID: 27515207

Recent clinical studies

Clinical prediction guides

Jung LK
Clin Immunol Immunopathol 1991 Oct;61(1):41-54. doi: 10.1016/s0090-1229(06)80006-1. PMID: 1959239

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