L’essentiel pour une gestion sans stress des patients porteurs d’une polykystose hépato-rénale en dialyse péritonéale.

Cas clinique et revue.

Résumé

Résumé

La polykystose hépatorénale autosomique dominante (PKR) est une maladie rénale chronique fréquente. La dialyse péritonéale (DP) concerne moins de 7% de ces patients. La sous-utilisation de la DP est due à la crainte d’un échec technique en raison de volumineux organes intra-péritonéaux.
Pour illustrer la faisabilité de la DP chez les patients PKR malgré les organomégalies. nous rapportons le cas d’une patiente de 70 ans atteinte de polykystose hépatorénale, traitée par DP après une longue histoire de transplantation rénale et d’hémodialyse. L’évolution de la patiente en DP a été satisfaisante en termes d’adéquation et d’équilibre hydrosodé.
Nous faisons ensuite une revue de la littérature sur les spécificités de la prise en charge des patients PKR en DP. La survie des patients atteints de polykystose est identique en DP et en hémodialyse. Il n’y a pas de surrisque d’échec technique ni de péritonites chez les patients polykystiques en DP. Cependant, il y a un peu plus de hernies symptomatiques chez les patients polykystiques, sans impact sur la survie technique. La mesure de la pression intra-péritonéale (PIP) est une aide à la prescription, permettant d’adapter le volume de dialysat pour les échanges. En cas de nécessité de réduction néphronique, l’embolisation artérielle rénale semble être la technique à privilégier. Elle est associée à une meilleure survie technique, à une réduction des transferts temporaires ou permanents en hémodialyse et à une réduction du temps d’hospitalisation.
En conclusion, la dialyse péritonéale est une option viable pour les patients atteints de polykystose hépatorénale malgré les organomégalies. Une orientation précoce en DP pourrait préserver le capital vasculaire des patients. Les professionnels de santé doivent être informés sur la survie, l’échec technique, les péritonites, les hernies symptomatiques et l’utilisation de la presssion intrapéritonéale (PIP) pour optimiser la prise en charge des patients polykystiques en DP.

INTRODUCTION

Autosomal dominant polycystic kidney disease is a common chronic kidney disease affecting 1/800 births and accounting for 6-10% of chronic end-stage renal disease cases each year[1]. The main therapies proposed for these patients are transplantation (59%)[2]and hemodialysis (43%). Peritoneal dialysis (PD) is used in less than 7% of polycystic patients[2]. This underuse of PD in polycystic patients is related to an unfounded fear of potential technical failure associated with the presence of large intraperitoneal organs.

In the first part of this article, we report the clinical case of a 70-year-old polycystic patient treated with PD after a long history of renal transplantation and hemodialysis. In the second part, some specific points on the management of polycystic patients with PD will be briefly discussed. The aim of this article is to address the specificities related to the management of polycystic patients on PD in order to facilitate the use of PD in the early stages of their care.

CLINICAL CASE

In 2020, our center was faced with initiation of PD in a 70-year-old female with renal disease secondary to hepatorenal polycystic disease. The patient started hemodialysis in 2006. The period of hemodialysis was marked by multiple vascular access complications and the performance of a right nephrectomy in 2007 as part of the pre-transplant preparation. Renal transplantation was performed in 2009. In 2019, renal function deteriorated due to allograft nephropathy, so a return to hemodialysis was decided, and a two-channel central catheter was placed because of the impossibility of creating an arteriovenous fistula (AVF). The year 2019 was complicated by multiple episodes of E. faecium bacteremia, which finally led to a superior vena cava thrombosis in a septic context.

Given the inability to obtain vascular access, the patient was offered PD. The catheter was placed laparoscopically, and a hernia repair of the linea alba was performed simultaneously. At the time of PD, the patient’s weight was 53 kg for 1.66 m, and the residual diuresis was 1500 ml/24 h. The abdominal computed tomography (CT) scan of the patient at that time is shown inFigure 1..

Figure 1.Abdominal CT scan shows the large hepatomegaly, the residual native kidney on the left, and the dysfunctional renal graft in the right iliac fossa

The patient started continuous ambulatory peritoneal dialysis (CAPD) for 4 months with the following regimen: an infused volume of 1500 ml and 4 exchanges of Iso, Nutrineal, Iso, and Icodextrine. Then, in APD with a total volume of 7500 (Iso + Nutrineal), an infused volume of 1800 ml/exchange, and a long-day dwell with 1000 ml of Icodextrin. The evolution of the patient’s intraperitoneal pressure (IPP) is shown in Table I.

The patient’s progress on PD was satisfactory in terms of adequacy for PD and hydrosodic balance (Table II).

IPP measurement

Table I.Intra-peritoneal Pressure (IPP) measurements

Dates	Infused volume (ml)	IPP (cm of water)
2021	1500	11.5
2023	1500	16
2023	1000	12.5

Table II. Patient’s adequacy parameters

Table II.Patient’s adequacy parameters

	Technique	Creatinine clearance/1.173 m	Kt/v	UF + Diuresis	Estimated RRF	Alb	nPCR
Feb 2021	CAPD	177.82	3.43	1300	13.26	26.6	1.18
Jun 2021	CAPD	146.85	2.3	1400	10.45	27.5	0.84
Dec 2021	APD	147.35	3.05	1450	10.77	29	1.16
April 2022	APD	153.56	3	1550	11.45	27.5	1.20
Aug 2022	APD	123.59	2.55	1000	9.49	27.6	1.06
Nov 2022	APD	112.47	2.06	1100	7.96	29.2	0.90

Conclusion

This clinical case illustrates the feasibility of PD in polycystic patients despite organomegaly. For this patient, an early referral to

PD in 2006 could have preserved her vascular capital.

WHAT YOU NEED TO KNOW ABOUT POLYCYSTIC KIDNEY DISEASE AND PERITONEAL DIALYSIS

The survival of patients with polycystic disease is identical in peritoneal dialysis and in hemodialysis

Several studies have examined the survival of patients with polycystic kidney disease treated with peritoneal dialysis (PD). Some studies have used patients treated with PD for nephropathy other than polycystic kidney disease (diabetes, vascular disease) as a control group. These studies show better survival in the polycystic kidney disease group than in the control group[3][4].

In other studies, the control group is a group of polycystic patients treated with hemodialysis. Again, there was no difference in survival between polycystic patients treated with hemodialysis and those treated with PD[4].

There is no increased risk of technical failure in patients with polycystic disease

A meta-analysis including 9 international studies and more than 7000 patients found no statistically significant difference between the polycystic group and the control group consisting of non-polycystic patients treated with PD. PD is therefore not associated with an increased risk of technical failure in the polycystic population[5].

There is no more peritonitis in polycystic patients

In the same meta-analysis, the frequency of episodes of peritoneal infection during PD was compared between the group of polycystic patients and the control group, which consisted of patients with other kidney diseases. No statistically significant difference was found between the two groups[5].

There is slightly more symptomatic hernia in polycystic patients

In a meta-analysis by Dupont et al, an excess risk of hernia was found in the polycystic disease group with an odds ratio of 2.28 (1.28 - 4.12). However, this excess risk did not affect the technical survival of this population[5].

IPP measurement is a prescription aid

One of the problems of polycystic disease is the large

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