Abstract:
:Hyperprostaglandin E syndrome (HPS), the prenatal variant of Bartter's syndrome, is characterized by a marked and selective stimulation of prostaglandin E (PGE2) synthesis. In the study group HPS patients showed increased urinary levels of PGE2, an index of renal, and of 11 alpha-hydroxy-9,15-dioxo-2,3,4,5,20-pentanor-19-carboxyprostano ic acid (PGE-M), an index of systemic PGE2 synthesis of 470% and of 570%, respectively. In addition, plasma concentration of PGE-M was also elevated 6.3-fold when compared with a control group. The urinary levels of other prostanoids were unaltered. During indomethacin treatment in both groups prostanoid excretion rates were suppressed to similar levels. To investigate the origin of stimulated prostanoid biosynthesis in HPS patients CD14+ monocytes were isolated from plasma samples, and the prostanoid synthesis was analyzed. The pattern and amounts of metabolites synthesized from endogenous arachidonic acid pools did not vary significantly between monocytes of the HPS and the control group. Thromboxane A2 (TXA2) was formed as the major prostanoid product. Using PGH2 as an exogenous substrate, again no difference in PGE2 biosynthesis was observed, indicating no difference in PGE-synthetic activity between both groups. Additionally, mRNA expression analysis of CD14+ monocytes via RT-PCR delineated the constitutive expression of cyclooxygenase-1, cyclooxygenase-2, and thromboxane synthase mRNA in cells from HPS patients and controls without statistical differences between these two groups. In conclusion, our data show that monocytes are not the source for the increased PGE2 biosynthesis in children with HPS, and a genetic defect in PGE synthesis can be excluded as the primary event in the pathogenesis in HPS.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Nüsing RM,Schaub TP,Klein T,Schweer H,Seyberth HWdoi
10.1203/00006450-199708000-00019subject
Has Abstractpub_date
1997-08-01 00:00:00pages
241-6issue
2eissn
0031-3998issn
1530-0447journal_volume
42pub_type
杂志文章abstract::Lung deposition of inhaled drugs in ventilated neonates has been studied in models of questionable relevance. With conventional nebulizers, pulmonary deposition has been limited to 1% of the total dose. The objective of this study was to assess lung delivery of aerosols in a model of neonatal ventilation using a conve...
journal_title:Pediatric research
pub_type: 杂志文章
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abstract::In 1964, H. K. A. Visser and W. S. Cost were the first to suggest a defect of the terminal aldosterone (Aldo) biosynthesis in patients with hypoaldosteronism. In the last years, the molecular basis of the terminal Aldo biosynthesis has been elucidated. Aldo biosynthesis requires 11beta-hydroxylation of 11-deoxycortico...
journal_title:Pediatric research
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journal_title:Pediatric research
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abstract::In a review of 13 documented cases of fetal alcohol syndrome (FAS), an increased incidence of life-threatening bacterial infections as well as a propensity to minor infections was observed. Five of 13 patients had had at least one episode of pneumonia, two had meningitis, and one had sepsis. A comprehensive immunologi...
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更新日期:1981-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.131
更新日期:2017-10-01 00:00:00
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journal_title:Pediatric research
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更新日期:1996-04-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
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更新日期:2001-08-01 00:00:00
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更新日期:2014-02-01 00:00:00
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journal_title:Pediatric research
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更新日期:1980-02-01 00:00:00
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更新日期:2003-09-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198405000-00012
更新日期:1984-05-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199010000-00005
更新日期:1990-10-01 00:00:00
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更新日期:1993-02-01 00:00:00
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更新日期:2007-10-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2005-05-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:1993-03-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
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更新日期:2001-09-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1975-10-01 00:00:00