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Kongenitale alveolar-kapilläre Dysplasie

Seltene Ursache der persistierenden pulmonalen Hypertension beim Neugeborenen

Congenital alveolar-capillary dysplasia

A rare cause of persistent pulmonary hypertension in neonates

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Monatsschrift Kinderheilkunde Zeitschrift für Kinder- und Jugendmedizin Aims and scope Submit manuscript

Zusammenfassung

Hintergrund

Die kongenitale alveolar-kapilläre Dysplasie (CACD) ist eine seltene Ursache der persistierenden pulmonalen Hypertension (PPHN) des Neugeborenen. Die Diagnose dieser idiopathischen PPHN ist zumeist eine Ausschlussdiagnose.

Fallbericht

Wir berichten über einen ungewöhnlichen klinischen Verlauf bei einem männlichen Säugling mit PPHN, welche ursächlich durch eine herdförmige CACD bedingt war.

Nach unauffälligem postnatalen Verlauf dekompensierte das Neugeborene im Alter von 10 Wochen. Der Zustand besserte sich nach zweiwöchiger intensivmedizinischer, medikamentöser antipulmonal-hypertensiver und herzunterstützender Behandlung vorübergehend. Wenige Tage später verstarb der Patient an Herz-Kreislauf-Versagen. Die endgültige Diagnosestellung erfolgte nach Obduktion und histopathologischer Untersuchung des Lungengewebes anhand der typischen Zeichen für eine CACD.

Schlussfolgerung

Bei jedem Neugeborenen mit PPHN ohne erkennbare sekundäre Störung ist die CACD differentialdiagnostisch in Betracht zu ziehen. Eine definitive Diagnosestellung der CACD ist nur durch eine offene Lungenbiopsie möglich.

Abstract

Background

Congenital alveolar capillary dysplasia (CACD) is a rare cause of persistent pulmonary hypertension in neonates (PPHN). This idiopathic PPHN is mostly diagnosed by exclusion.

Case report

We report an unusual clinical course in a male newborn with PPHN caused by patchy CACD.

Following an uneventful postnatal period the infant decompensated at the age of 10 weeks. His condition improved temporarily after 2 weeks of intensive medical care including medication to combat the pulmonary hypertension and support the heart. A few days later the patient died of heart failure. The final diagnosis was established by autopsy and histopathological examination of the lung tissue, which exhibited typical signs of CACD.

Conclusions

CACD should be considered in the differential diagnosis in all neonates with refractory PPHN when there is no recognizable secondary disturbance. Only an open lung biopsy can yield a definitive diagnosis of CACD.

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Authors and Affiliations

  1. Fehlbildungsmonitoring Sachsen-Anhalt, Medizinische Fakultät, Otto-von-Guericke-Universität Magdeburg, Leipziger Straße 44, 39120, Magdeburg, Deutschland

    J. Hoyer-Schuschke & S. Pötzsch

  2. Klinik für Allgemeine Pädiatrie und Neonatologie, Otto-von-Guericke-Universität Magdeburg, Magdeburg, Deutschland

    R. Böttger & M. Gleißner

  3. Institut für Pathologie, Otto-von-Guericke-Universität Magdeburg, Magdeburg, Deutschland

    K. Herrmann

  4. Klinik für Radiologie und Nuklearmedizin, Abt. Kinderradiologie, Otto-von-Guericke-Universität Magdeburg, Magdeburg, Deutschland

    L. von Rohden

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  1. J. Hoyer-Schuschke
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  2. S. Pötzsch
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  3. R. Böttger
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  4. K. Herrmann
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  5. L. von Rohden
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  6. M. Gleißner
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Correspondence to J. Hoyer-Schuschke.

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Hoyer-Schuschke, J., Pötzsch, S., Böttger, R. et al. Kongenitale alveolar-kapilläre Dysplasie. Monatsschr Kinderheilkd 156, 57–62 (2008). https://doi.org/10.1007/s00112-007-1585-7

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  • DOI: https://doi.org/10.1007/s00112-007-1585-7

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