Results 181 to 190 of about 161,849 (206)

Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia

Pediatric Nephrology, 2005
Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment.
Kazuo Obata, Atsunori Yoshino, Masataka Honda, Toshiro Nagai, Hiroyoshi Matsukura, Yoshihiko Ueda, Hirokazu Kanegane, Satoru Sakazume, Yasuki Katada, Toshio Miyawaki   +9 more
openaire   +3 more sources

Lung transplantation in patients with x-linked agammaglobulinemia

Transplantation Proceedings, 2003
Lung transplantation is an established procedure to treat patients with end-stage lung disease. The criteria for recipient selection are broadening to include patients with congenital defects of the immune system, such as X-linked hypogammaglobulinemia (XLA). We report 2 cases of successful double lung transplantation in patients with XLA.
A. Solé, I. Moreno, Rosario Vicente, P. Morales, J.J Torres, A Lanuza, D Hernández   +6 more
openaire   +3 more sources

A Case of X-Linked Agammaglobulinemia Diagnosed in Adulthood

Clinical Immunology, 2001
X-linked agammaglobulinemia (XLA), caused by mutations in Bruton's tyrosine kinase (BTK), typically presents in early childhood. We report here the case of a male diagnosed at age 23 years with hypogammaglobulinemia, originally classified as common variable immunodeficiency (CVID).
Lan Tian, Donn M. Stewart, David L. Nelson   +2 more
openaire   +3 more sources

A case of X-linked agammaglobulinemia with progressive encephalitis

Pediatric Neurology, 2004
This report describes a case of agammaglobulinemia with progressive encephalitis. The patient was a 6-year-old male who was diagnosed as having Bruton-type agammaglobulinemia at age 6 months. After the diagnosis was made, he received monthly intravenous immunoglobulin replacement with a residual immunoglobulin G level of more than 400 mg/dL. At 5 years
Kazuo Nagashima, Naohide Shiroma, Takao Ohta, Hideki Hasegawa, Tsuyoshi Omi   +4 more
openaire   +3 more sources

Expression of the Gene Defect in X-Linked Agammaglobulinemia

New England Journal of Medicine, 1986
Although X-linked agammaglobulinemia was one of the first immunodeficiencies described,1 the genetic defect responsible for this disorder has not yet been identified.
Allan R. Pickard, Rebecca H. Buckley, Jack W. Singer, Debra S. Miller, Mary Ellen Conley, Philip J. Fialkow, Wendy H. Raskind, Persymphonie Brown   +7 more
openaire   +2 more sources

X‐linked agammaglobulinemia complicated with endobronchial tuberculosis

Acta Paediatrica, 2010
AbstractAim:  We report a case of X‐linked agammaglobulinemia complicated with endobronchial tuberculosis.Methods:  We observed the patient’s clinical course and analysed his data retrospectively.Results: Interestingly, the T‐cell proliferation activity in this patient was intact, and the CD4‐positive T cells produced interferon gamma.
Chihiro Kawakami, Toshio Miyawaki, Kimitaka Takitani, Hirokazu Kanegane, Hiroshi Tamai, Akiko Inoue   +5 more
openaire   +2 more sources

An International Survey of Allogeneic Hematopoietic Cell Transplantation for X-Linked Agammaglobulinemia

Journal of Clinical Immunology, 2023
Akira Nishimura, R. Uppuluri, Revathi Raj, V. Swaminathan, Yi-fei Cheng, R. Abu-Arja, Bin Fu, A. Laberko, M. Albert, F. Hauck, G. Bucciol, V. Bigley, S. Elcombe, G. Kharya, C. Pronk, Claudia Wehr, B. Neven, K. Warnatz, I. Meyts, T. Morio, A. Gennery, H. Kanegane   +21 more
semanticscholar   +1 more source

Maternal germinal mosaicism of X‐linked agammaglobulinemia

American Journal of Medical Genetics, 2001
X-linked agammaglobulinemia (XLA) is an immunodeficiency caused by abnormalities in tyrosine kinase (BTK), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of BTK protein and analyzed the BTK gene (BTK) in peripheral blood mononuclear cells from two siblings with XLA and additional family members ...
Hirokazu Kanegane, Noriaki Shinomiya, Mariko Sakamoto, Toshio Miyawaki, Satoshi Tsukada, Hideki Fujii   +5 more
openaire   +2 more sources

X-Linked Agammaglobulinemia and Bruton’s Tyrosine Kinase

1994
The genetic defect associated with human X-linked agammaglobulinemia (XLA) and murine X-linked immunodeficiency (XID) was recently identified as the deficiency of function of a new cytoplasmic tyrosine kinase called Bruton’s tyrosine kinase (Btk)1,2,3,4. The phenotypes associated with these immunodeficiencies indicate that Btk plays a crucial role in B
Satoshi Tsukada, Owen N. Witte
openaire   +3 more sources

Molecular and Cellular Aspects of X-Linked Agammaglobulinemia

1995
Publisher Summary The chapter discusses the current knowledge in the field of X-Linked Agammaglobulinemia (XLA) in the context of the recent developments. XLA, by being the first human disease caused by a defect in the function of a cytoplasmic tyrosine kinase (PTK)– the XLA product designated Btk (Bruton's agammaglobulinemia tyrosine kinase)– has ...
C. I. E. Smith, Paschalis Sideras
openaire   +3 more sources