Disturbances of prostacyclin metabolism in children with hemolytic-uremic syndrome and in first degree relatives

S. Túri, T. J. Beattie, J. J. Belch, A. V. Murphy

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    Abstract

    Plasma from 24 children with hemolytic uremic syndrome (HUS) (10 in acute, 14 in remission phase), 42 first degree relatives and 24 controls were studied for PGI2 supporting activity (PSA) from human umbilical arterial rings and the concentration of PGI2 metabolite (PGI2m). HUS patients in acute phase showed very low or absent level of plasma PSA, which remained depressed 3 months following presentation. Plasma from 2 out of 5 acute HUS patients showed inhibition against PGI2-like activity, and depressed preservation of PGI2 effect. The mean value of PGI2m in acute phase of HUS patients was elevated initially, but fell below control range by the day 14 and remained decreased at the end of 3rd month. Patients on long term remission showed a significantly lower concentration of plasma PGI2m. Eight of 14 HUS patients in remission and 18 of 42 family members had lower PSA levels than the controls. These studies confirmed a decreased PSA in HUS and suggest that persistently low PSA levels may reflect an inherited predisposition.
    Original languageEnglish
    Pages (from-to)193-198
    Number of pages6
    JournalClinical Nephrology
    Volume25
    Issue number4
    Publication statusPublished - 1986

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    Hemolytic-Uremic Syndrome
    Epoprostenol
    Umbilicus
    Human Activities

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    title = "Disturbances of prostacyclin metabolism in children with hemolytic-uremic syndrome and in first degree relatives",
    abstract = "Plasma from 24 children with hemolytic uremic syndrome (HUS) (10 in acute, 14 in remission phase), 42 first degree relatives and 24 controls were studied for PGI2 supporting activity (PSA) from human umbilical arterial rings and the concentration of PGI2 metabolite (PGI2m). HUS patients in acute phase showed very low or absent level of plasma PSA, which remained depressed 3 months following presentation. Plasma from 2 out of 5 acute HUS patients showed inhibition against PGI2-like activity, and depressed preservation of PGI2 effect. The mean value of PGI2m in acute phase of HUS patients was elevated initially, but fell below control range by the day 14 and remained decreased at the end of 3rd month. Patients on long term remission showed a significantly lower concentration of plasma PGI2m. Eight of 14 HUS patients in remission and 18 of 42 family members had lower PSA levels than the controls. These studies confirmed a decreased PSA in HUS and suggest that persistently low PSA levels may reflect an inherited predisposition.",
    author = "S. T{\'u}ri and Beattie, {T. J.} and Belch, {J. J.} and Murphy, {A. V.}",
    year = "1986",
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    Disturbances of prostacyclin metabolism in children with hemolytic-uremic syndrome and in first degree relatives. / Túri, S.; Beattie, T. J.; Belch, J. J.; Murphy, A. V.

    In: Clinical Nephrology, Vol. 25, No. 4, 1986, p. 193-198.

    Research output: Contribution to journalArticle

    TY - JOUR

    T1 - Disturbances of prostacyclin metabolism in children with hemolytic-uremic syndrome and in first degree relatives

    AU - Túri, S.

    AU - Beattie, T. J.

    AU - Belch, J. J.

    AU - Murphy, A. V.

    PY - 1986

    Y1 - 1986

    N2 - Plasma from 24 children with hemolytic uremic syndrome (HUS) (10 in acute, 14 in remission phase), 42 first degree relatives and 24 controls were studied for PGI2 supporting activity (PSA) from human umbilical arterial rings and the concentration of PGI2 metabolite (PGI2m). HUS patients in acute phase showed very low or absent level of plasma PSA, which remained depressed 3 months following presentation. Plasma from 2 out of 5 acute HUS patients showed inhibition against PGI2-like activity, and depressed preservation of PGI2 effect. The mean value of PGI2m in acute phase of HUS patients was elevated initially, but fell below control range by the day 14 and remained decreased at the end of 3rd month. Patients on long term remission showed a significantly lower concentration of plasma PGI2m. Eight of 14 HUS patients in remission and 18 of 42 family members had lower PSA levels than the controls. These studies confirmed a decreased PSA in HUS and suggest that persistently low PSA levels may reflect an inherited predisposition.

    AB - Plasma from 24 children with hemolytic uremic syndrome (HUS) (10 in acute, 14 in remission phase), 42 first degree relatives and 24 controls were studied for PGI2 supporting activity (PSA) from human umbilical arterial rings and the concentration of PGI2 metabolite (PGI2m). HUS patients in acute phase showed very low or absent level of plasma PSA, which remained depressed 3 months following presentation. Plasma from 2 out of 5 acute HUS patients showed inhibition against PGI2-like activity, and depressed preservation of PGI2 effect. The mean value of PGI2m in acute phase of HUS patients was elevated initially, but fell below control range by the day 14 and remained decreased at the end of 3rd month. Patients on long term remission showed a significantly lower concentration of plasma PGI2m. Eight of 14 HUS patients in remission and 18 of 42 family members had lower PSA levels than the controls. These studies confirmed a decreased PSA in HUS and suggest that persistently low PSA levels may reflect an inherited predisposition.

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