Schwangerschaft nach sequenzieller Leber-Nieren-Transplantation bei Hyperoxalurie Typ I: Was ist daran anders als sonst?

Zusammenfassung

Schwangerschaft nach Nierentransplantation ist immer ein Risiko sowohl für Mutter und Kind als auch für das Nierentransplantat. Wir beschreiben den Fall einer jungen Frau mit primärer Hyperoxalurie Typ 1 und dadurch bedingtem terminalem Nierenversagen durch Nephrokalzinose. Sie hatte als 10-jähriges Kind eine Lebertransplantation durch Fremdspende erhalten zur Behebung des Enzymdefektes in der Leber sowie 2 Monate später eine Nierentransplantation durch Nierenspende der Mutter. Die Immunsuppression erfolgte durch Cyclosporin, Mycophenolatmofetil und Prednisolon. Das Lebertransplantat hatte 3 Abstoßungen, jeweils behandelt mit Steroidboli. Das Nierentransplantat zeigte eine langsame Funktionsverschlechterung über die Jahre, Entwicklung einer Proteinurie von 1 Gramm Protein pro 1 Gramm Kreatinin sowie eine mittelschwere Hypertonie. Trotzdem hatte die Frau einen persistierenden dringenden Kinderwunsch und setzte sich damit durch. Der Artikel beschreibt die speziellen Probleme dieses Falles: (1) das allgemeine Problem, Patienten mit Hyperoxalurie Typ 1 überhaupt zu transplantieren; (2) das spezielle Problem der Schwangerschaft dieser 31-jährigen Frau mit genetisch bedingter Nierenerkrankung und vorangehender Transplantation von 2 soliden Organen vor 18 Jahren; (3) die mit einem immunologisch instabilen Lebertransplantat und einem Nierentransplantat einhergehende langsam progrediente Funktionsverschlechterung und ansteigende Proteinurie. Die generellen Richtlinien für eine Schwangerschaft nach Nierentransplantation werden aufgezeigt im Vergleich zu dem hier beschriebenen Fall einer Schwangerschaft, bei der fast alle Parameter außerhalb der Richtlinien liegen bei persistierend starkem Kinderwunsch dieser Frau.

Abstract

Pregnancy in women with a transplanted kidney is always a risk for mother, child, and transplanted organ. We cared for a young woman with hyperoxaluria type 1 and end-stage renal failure by nephrocalcinosis. She underwent a sequential deceased-donor liver transplantation for treatment of the hepato-centric genetic enzyme defect, followed by a living-related kidney transplantation from her mother 2 months later. Both interventions were performed when she was a 10-year-old child. She received cyclosporine, mycophenolate, and prednisolone. The liver was rejected 3 times, which required intravenous steroids. The renal function deteriorated gradually over the years with proteinuria 1 gram protein per 1 gram creatinine and moderate hypertension. However, the womanʼs wish for pregnancy was not to be denied, and so it was. We were confronted with the following problems which will be discussed in the article: (1) the problems of transplanting patients with hyperoxaluria type 1 in general; (2) the special problem of a pregnancy in a 31-year-old woman with a genetic kidney disease, after transplantation of 2 organs as a child 18 years earlier; (3) an immunologically instable liver transplant and a renal allograft with slowly deteriorating glomerular filtration rate and increasing proteinuria. Our patient generally ignored our advice; her desires were paramount. The general guidelines for pregnancy after renal transplantation are given in comparison to this exceptional case with all parameters beyond the guidelines due to the insisting strong wish of this woman to have a child. Our experience may enable other clinicians faced with similar problems.

Schlüsselwörter

Hyperoxalurie Typ 1 - sequenzielle Leber-Nieren-Transplantation - Schwangerschaft nach Nierentransplantation - Präeklampsie

Key words

hyperoxaluria Type 1 - sequential liver-kidney transplantation - pregnancy after renal transplantation - preeclampsia

Publication History

Article published online:
16 November 2020

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