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305) Thus, we consider that T-cell dysfunction and/or impairments in T-B cell interactions due to X-SCID may have played important roles in the onset of WS.
* Successful treatment for West syndrome with severe combined immunodeficiency.
- Several immune mechanisms are suspected in the unknown etiology of West syndrome (WS). We report a male infant who suffered from WS and X-linked T-B+NK- severe combined immunodeficiency (X-SCID) with a missense mutation of the IL2RG gene (c.202G>A, p.Glu68Lys). He promptly began vitamin B6 and valproic acid treatment, but infantile spasms (IS) and hypsarrhythmia persisted. Administration of intravenous immunoglobulin and the change to topiramate (TPM) at 7 months of age resulted in the rapid resolution of IS. The CD4/8 ratio in his peripheral blood increased from 0.04-0.09 to 0.20-1.95 following unrelated cord blood transplantation (UCBT). In vitro lymphocyte proliferation in response to phytohemagglutinin or concanavalin A and the ability of B lymphocytes to produce antibodies improved as well. Electroencephalogram findings became normal 1 month after UCBT. Thus, we consider that T-cell dysfunction and/or impairments in T-B cell interactions due to X-SCID may have played important roles in the onset of WS. Immune-modulating therapies along with the administration of TPM effectively treated this severe epileptic syndrome in our patient.
=>1.予定の, 支払期日がきて, 当然支払われるべき, 2.支払[提出]期日, 満期日, 会費
Overview of noun due
The noun due has 2 senses (first 1 from tagged texts)
1. (4) due -- (that which is deserved or owed; "give the devil his due")
2. due -- (a payment that is due (e.g., as the price of membership); "the society dropped him for
non-payment of dues")
Overview of adj due
The adj due has 4 senses (first 2 from tagged texts)
1. (6) due -- (owed and payable immediately or on demand; "payment is due")
2. (1) due -- (scheduled to arrive; "the train is due in 15 minutes")
3. due -- (suitable to or expected in the circumstances; "all due respect"; "due cause to honor
them"; "a long due promotion"; "in due course"; "due esteem"; "exercising due care")
4. ascribable, due, imputable, referable -- (capable of being assigned or credited to; "punctuation
errors ascribable to careless proofreading"; "the cancellation of the concert was due to the rain";
"the oversight was not imputable to him")
Overview of adv due
The adv due has 1 sense (no senses from tagged texts)
1. due -- (directly or exactly; straight; "went due North")
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