Reviewing Omenn syndrome

European Journal of Pediatrics

Volumn 160, Issue 12, 2001, Pages 718-725

  Aleman, Kees ^a   Noordzij, Jeroen G ^b   De Groot, Ronald ^a   Van Dongen, Jacques J ^b   Hartwig, Nico G ^a  

^a SOPHIA CHILDREN S HOSPITAL   (Netherlands)

^b ERASMUS MEDICAL CENTER   (Netherlands)

Author keywords

Eosinophilia; Erythrodermia; Recombination activating genes; Severe combined immunodeficiency

Indexed keywords

IMMUNOGLOBULIN E;

ALOPECIA; ARTICLE; BONE MARROW TRANSPLANTATION; CLINICAL FEATURE; CONTROLLED STUDY; DISEASE COURSE; EOSINOPHILIA; ERYTHEMA; FEMALE; HEPATOSPLENOMEGALY; HUMAN; IMMUNE DEFICIENCY; IMMUNOGLOBULIN BLOOD LEVEL; INFECTION; LABORATORY TEST; LEUKOCYTE; LYMPHADENOPATHY; LYMPHOCYTE COUNT; LYMPHOCYTOSIS; MAJOR CLINICAL STUDY; MALE; MORTALITY; OMENN SYNDROME; PATHOPHYSIOLOGY; PRIORITY JOURNAL; STEM CELL TRANSPLANTATION; SURVIVAL; TREATMENT OUTCOME;

EID: 0035213341     PISSN: 03406199     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004310100816     Document Type: Article

References (46)

1. Genetic immunodeficiencies: Cutaneous manifestations and recent progress
2. Rapidly fatal familial histiocytosis associated with eosinophilia and primary immunological deficiency
3. Successful unrelated umbilical cord blood transplantation in a child with Omenn's syndrome
4. T-cell receptor analysis in Omenn's syndrome: Evidence for defects in gene rearrangement and assembly
5. Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn syndrome
6. In vitro cell death of activated lymphocytes in Omenn's syndrome
7. Clinical and immunological findings in four infants with Omenn's syndrome: A form of severe combined immunodeficiency with phenotypically normal T cells, elevated IgE, and eosinophilia
8. Combined immunodeficiency presenting as the Letterer-Siwe syndrome
9. CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2-mediated condition
10. Association of a lymphocyte purine enzyme deficiency (5′-nucleotidase) with combined immunodeficiency
11. Immunophenotyping of blood lymphocytes in childhood. Reference values for lymphocyte subpopulations
12. Restricted heterogeneity of T lymphocytes in combined immunodeficiency with hypereosinophilia (Omenn's syndrome)
13. Deficit immunitaire mixte et grave aec hypereosinophilie
14. Omenn's disease
15. Absence of lymphocyte ecto-5′-nucleotidase in infants with reticuloend-otheliosis and eosinophilia (Omenn's syndrome)
16. Omenn syndrome
17. Syndrome of erythroderma, failure to thrive, and diarrhea in infancy: A manifestation of immunodeficiency
18. Treatment of Omenn syndrome by bone marrow transplantation
19. Oligoclonal expansion of CD45RO + T lymphocytes in Omenn syndrome
20. Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency
21. Omenn disease: Termination in lymphoma
22. Omenn's syndromepathologic arguments in favor of a graft versus host pathogenesis: A report of nine cases
23. Clinical and immune recovery from Omenn syndrome after bone marrow transplantation
24. Imbalances in subsets of T lymphocytes in an inbred pedigree with Omenn's syndrome
25. Mismatched bone marrow transplantation for Omenn syndrome: A variant of severe combined immunodeficiency
26. The lymph node pathology of Omenn's syndrome
27. Expansion of CD3 + CD4-CD8- T cell population expressing high levels of IL-5 in Omenn's syndrome
28. A case of Omenn-like immunodeficiency syndrome
29. Omenn's syndrome
30. N-terminal truncated human RAG 1 proteins can direct TCR but not Ig gene rearrangements
31. RAG and RAG defects
32. Combined immunodeficiency and reticuloendotheliosis with eosinophilia
33. Familial reticuloendotheliosis with eosinophilia
34. Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome
35. The Omenn's syndrome: Histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100 +/T-6 + Langerhans-like cells
36. Omenn's reticulosis associated with the nephrotic syndrome
37. T helper type 2-like cells and therapeutic effects of interferon-gamma in combined immunodeficiency with hypereosinophilia (Omenn's syndrome)
38. Helper T cell dysfunctions in a patient with Omenn's syndrome: Modulation by IFN-gamma therapy
39. The V(D)J recombination activating gene, RAG-1
40. Omenn phenotype with short-limbed dwarfism
41. RAG mutations in human B cell-negative SCID
42. Intrathymic restriction and peripheral expansion of the T-cell repertoire in Omenn syndrome
43. Omenn syndrome: Familial reticoloendotheliosis with eosinophilia and combined immunodeficiency
44. Partial V(D)J recombination activity leads to Omenn syndrome
45. Novel T-lymphocyte population in combined immunodeficiency with features of graft-versus-host disease
46. A lymphoproliferative syndrome, "cutaneous dystrophy" and combined immune deficiency with lack of helper T-cell factor