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Tijdschrift voor kindergeneeskunde

, Volume 70, Issue 2, pp 226–231 | Cite as

Primaire pulmonale hypertensie op de kinderleeftijd

Presentatie, diagnostiek en nieuwe behandelingsmogelijkheden
  • P. P. Roeleveld
  • M. Jongejan
  • R. M. F. Berger
Artikelen

Samenvatting

Primaire pulmonale hypertensie is een zeldzame en levensbedreigende longvaataandoening met onbekende pathogenese, die zich op de kinderleeftijd op verschillende manieren kan presenteren. Er bestaat geen causale behandeling en tot voor kort was alleen ondersteunende therapie mogelijk. De laatste jaren zijn er echter belangrijke nieuwe ontwikkelingen op het gebied van de behandeling. Hierbij is vooral de chronische continue intraveneuze therapie met epoprostenol, een prostacyclineanaloog, bij geselecteerde patiënten hoopgevend. Dit leidt tot langere overlevingsduur, maar heeft grote invloed op het dagelijks leven van de patiënt en vereist intensieve begeleiding. In dit artikel worden presentatievormen, differentiaaldiagnose en de verschillende behandelingsmogelijkheden besproken aan de hand van een ziektegeschiedenis.

Summary

Primary pulmonary hypertension is a rare and life threatening pulmonary vascular disease with unknown pathogenesis, which may present in various ways in the pediatric population. There is no causal treatment and until recently the only possible strategy was supportive care. In recent years, however, there have been important new developments in the area of treatment modalities. Especially chronic and continuous intravenous therapy with epoprostenol, a prostacyclin analogue, in selected patients gives rise to hope. It leads to a better survival rate, but also has a great impact on patient's daily life and demands intensive guidance. In this article presenting symptoms, differential diagnosis and current treatment modalities are discussed, illustrated by a case report.

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Copyright information

© Bohn Stafleu van Loghum 2002

Authors and Affiliations

  • P. P. Roeleveld
    • 1
  • M. Jongejan
  • R. M. F. Berger
  1. 1.

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