Biomaterial Database

Cytomegalovirus encephalitis in a child with adenosine deaminase-deficient severe combined immune deficiency: a neuropathologic study. 1998

T Takano, and M Zielenska, and L E Becker

Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.

Cytomegalovirus (CMV) encephalitis is exceptional in patients with genetically determined immune deficiency syndromes. Neuropathologic findings of CMV encephalitis were present at postmortem examination in a child we treated for severe combined immune deficiency. Cultured skin fibroblasts of this male infant revealed a deficiency of adenosine deaminase (ADA). Lacking a suitable bone marrow donor, we used transfusions of red blood cells as a source of ADA. However, the child developed encephalopathy and died at 16 months. The main neuropathologic findings were numerous, widely distributed inclusion-bearing and CMV antigen-positive cells; a complete lack of inflammatory reaction; and cell-to-cell fusion of infected cells reflecting the child's severely impaired immune response.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D003586	Cytomegalovirus Infections	Infection with CYTOMEGALOVIRUS, characterized by enlarged cells bearing intranuclear inclusions. Infection may be in almost any organ, but the salivary glands are the most common site in children, as are the lungs in adults.	CMV Inclusion,CMV Inclusions,Congenital CMV Infection,Congenital Cytomegalovirus Infection,Cytomegalic Inclusion Disease,Cytomegalovirus Colitis,Cytomegalovirus Inclusion,Cytomegalovirus Inclusion Disease,Cytomegalovirus Inclusions,Inclusion Disease,Perinatal CMV Infection,Perinatal Cytomegalovirus Infection,Renal Tubular Cytomegalovirus Inclusion,Renal Tubular Cytomegalovirus Inclusions,Salivary Gland Virus Disease,Severe Cytomegalovirus Infection,Severe Cytomegalovirus Infections,Infections, Cytomegalovirus,CMV Infection, Congenital,CMV Infection, Perinatal,Colitis, Cytomegalovirus,Congenital CMV Infections,Congenital Cytomegalovirus Infections,Cytomegalic Inclusion Diseases,Cytomegalovirus Colitides,Cytomegalovirus Inclusion Diseases,Cytomegalovirus Infection,Cytomegalovirus Infection, Congenital,Cytomegalovirus Infection, Perinatal,Cytomegalovirus Infection, Severe,Cytomegalovirus Infections, Severe,Disease, Cytomegalic Inclusion,Disease, Cytomegalovirus Inclusion,Diseases, Cytomegalovirus Inclusion,Inclusion Disease, Cytomegalic,Inclusion Disease, Cytomegalovirus,Inclusion Diseases,Inclusion Diseases, Cytomegalovirus,Inclusion, CMV,Inclusion, Cytomegalovirus,Infection, Congenital CMV,Infection, Congenital Cytomegalovirus,Infection, Cytomegalovirus,Infection, Perinatal CMV,Infection, Perinatal Cytomegalovirus,Infection, Severe Cytomegalovirus,Perinatal CMV Infections,Perinatal Cytomegalovirus Infections
D003587	Cytomegalovirus	A genus of the family HERPESVIRIDAE, subfamily BETAHERPESVIRINAE, infecting the salivary glands, liver, spleen, lungs, eyes, and other organs, in which they produce characteristically enlarged cells with intranuclear inclusions. Infection with Cytomegalovirus is also seen as an opportunistic infection in AIDS.	Herpesvirus 5, Human,Human Herpesvirus 5,Salivary Gland Viruses,HHV 5,Herpesvirus 5 (beta), Human,Cytomegaloviruses,Salivary Gland Virus,Virus, Salivary Gland,Viruses, Salivary Gland
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000243	Adenosine Deaminase	An enzyme that catalyzes the hydrolysis of ADENOSINE to INOSINE with the elimination of AMMONIA.	Adenosine Aminohydrolase,Aminohydrolase, Adenosine,Deaminase, Adenosine
D016511	Severe Combined Immunodeficiency	Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. It is inherited as an X-linked or autosomal recessive defect. Mutations occurring in many different genes cause human Severe Combined Immunodeficiency (SCID).	Bare Lymphocyte Syndrome,Immunodeficiency, Severe Combined,Omenn Syndrome,Immunodeficiency Syndrome, Severe Combined,Immunologic Deficiency, Severe Combined,Omenn's Syndrome,Reticuloendotheliosis, Familial,Severe Combined Immune Deficiency,Severe Combined Immunodeficiency Syndrome,Severe Combined Immunologic Deficiency,Bare Lymphocyte Syndromes,Combined Immunodeficiencies, Severe,Combined Immunodeficiency, Severe,Familial Reticuloendothelioses,Familial Reticuloendotheliosis,Immunodeficiencies, Severe Combined,Lymphocyte Syndrome, Bare,Lymphocyte Syndromes, Bare,Omenns Syndrome,Reticuloendothelioses, Familial,Severe Combined Immunodeficiencies,Syndrome, Bare Lymphocyte,Syndrome, Omenn,Syndrome, Omenn's,Syndromes, Bare Lymphocyte
D017809	Fatal Outcome	Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.	Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D018792	Encephalitis, Viral	Inflammation of brain parenchymal tissue as a result of viral infection. Encephalitis may occur as primary or secondary manifestation of TOGAVIRIDAE INFECTIONS; HERPESVIRIDAE INFECTIONS; ADENOVIRIDAE INFECTIONS; FLAVIVIRIDAE INFECTIONS; BUNYAVIRIDAE INFECTIONS; PICORNAVIRIDAE INFECTIONS; PARAMYXOVIRIDAE INFECTIONS; ORTHOMYXOVIRIDAE INFECTIONS; RETROVIRIDAE INFECTIONS; and ARENAVIRIDAE INFECTIONS.	Encephalomyelitis, Infectious, Viral,Infectious Encephalomyelitis, Viral,Viral Encephalitis,Encephalomyelitis, Viral Infectious,Viral Infectious Encephalomyelitis