Glanzmann-Riniker syndrome - vertaling naar russisch
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Glanzmann-Riniker syndrome - vertaling naar russisch

COMBINED T CELL AND B CELL IMMUNODEFICIENCY THAT IS CAUSED BY A DEFECT IN SEVERAL GENES ENCODING FOR B AND T LYMPHOCYTES RESULTING IN INDIVIDUALS WITH NON-FUNCTIONAL IMMUNE SYSTEMS
Severe Combined Immunodeficiency; Severe Combined Immune Deficiency Syndrome; Severe combined immune deficiency; Severe Combined Immunodeficiency Disease; Severe combined immunodeficiency disorder; Severe combined immuno deficiency; Severe combined immunodeficiency disease; Alymphocytosis; Glanzmann–Riniker syndrome; Severe mixed immunodeficiency syndrome; Thymic alymphoplasia; Bubble baby syndrome; Glanzmann-Riniker syndrome; Bubble boy disease; Severe combined immunodeficiency syndrome; Bubble baby disease; Ashanthi De Silva
  • David Vetter inside his protective "bubble."

Glanzmann-Riniker syndrome         

медицина

лимфопеническая агаммаглобулинемия

атимолимфоплазия

alymphocytosis         

медицина

алимфоцитоз

лимфоцитарная аплазия

иммунопарез французского типа

severe combined immunodeficiency         

медицина

агаммаглобулинемия швейцарского типа

Definitie

Reye's syndrome
['re?z, 'r??z]
¦ noun a life-threatening metabolic disorder in young children, of uncertain cause.
Origin
1960s: named after the Australian paediatrician Ralph D. K. Reye.

Wikipedia

Severe combined immunodeficiency

Severe combined immunodeficiency (SCID), also known as Swiss-type agammaglobulinemia, is a rare genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations that result in differing clinical presentations. SCID involves defective antibody response due to either direct involvement with B lymphocytes or through improper B lymphocyte activation due to non-functional T-helper cells. Consequently, both "arms" (B cells and T cells) of the adaptive immune system are impaired due to a defect in one of several possible genes. SCID is the most severe form of primary immunodeficiencies, and there are now at least nine different known genes in which mutations lead to a form of SCID. It is also known as the bubble boy disease and bubble baby disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, have become famous for living in a sterile environment. SCID is the result of an immune system so highly compromised that it is considered almost absent.

SCID patients are usually affected by severe bacterial, viral, or fungal infections early in life and often present with interstitial lung disease, chronic diarrhea, and failure to thrive. Ear infections, recurrent Pneumocystis jirovecii (previously carinii) pneumonia, and profuse oral candidiasis commonly occur. These babies, if untreated, usually die within one year due to severe, recurrent infections unless they have undergone successful hematopoietic stem cell transplantation or gene therapy in clinical trials.

Vertaling van &#39Glanzmann-Riniker syndrome&#39 naar Russisch