Porto-Sinusoidal Vascular Disease
Conditions
Brief summary
To look for the factors of exposure present prior to the onset of Porto-Sinusoidal Vascular Disease (PSVD) and which can lead to the occurrence of PSVD after renal transplantation, and therefore to consider the identification of risk factors, in order to prevent or detect early signs of portal hypertention in predisposed patients and improve their prognosis.
Detailed description
Porto-Sinusoidal Vascular Disease (PSVD) is a heterogeneous clinico-pathological entity characterized by alterations in small liver vessels. The most common is known as nodular regenerative hyperplasia (NRH). Patients with PSVD especially NRH can developed portal hypertension. An acute portal thrombosis occurs in 1/3 of patients at five years. In case of portal hypertension, 10 years survival decreases down to 56 to 82%. De novo NRH has been described after liver transplantation (1 to 1.4% in adults, 8.2% in kids), and also in small series of kidney, cardiac and bone marrow transplantation. Prevalence is estimated to be 0.5% after kidney transplantation. Several risks factors have been related to PSVD (infections, Immune-mediated disorders, haematological diseases, prothrombotic disorders, drugs such as azathioprine, genetic diseases). In transplantation, mechanical vascular factors, drugs, virus, immunogenetic and immunologic factors have been suggested, but none of them are well established. The study aims to determined risk factors which can lead to PSVD in kidney transplant recipients, in order to prevent or to early detect portal hypertension and improve the prognosis of these patients. A case-control multicentric retrospective study will be done to compare adult kidney transplant recipients with PSVD with or without portal hypertension, to adult kidney transplant recipients without evidence for PSVD (matched according to the date of kidney transplantation of the case ± 2 years), and to non-transplanted adults with PSVD. Kidney transplant cases and controls will be recruited from French hepatogastroenterology and nephrology units. PSVD non transplanted controls will be recruited from the Beaujon Hospital database dedicated to liver vascular diseases.
Interventions
None listed
Sponsors
University Hospital, Caen
Study design
Observational model
CASE_CONTROL
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* PSVD (between january 2000 and december 2021) after kidney transplantation or * Kidney transplantation without PSVD or * PSVD without kidney transplantation
Exclusion criteria
* liver cirrhosis * transplanted from an other organ * thrombocytopenia and/or abnormal liver tests and/or hepatic dysmorphism and/or portal hypertension signs
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To highlight risk factors significantly related to the occurrence of PSVD in renal transplant recipients. | 2 years | Blood samples histrory results analyse to identify usual prothrombic factors associated (or not) to PSVD (e.g;, myeloproliferative syndrome, Factor V Leiden mutation, blood group, ...). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Clinical profiles. | 2 years | Natural history of PSVD in renal transplant recipients included in the study and in control group (medical history, complications of portal hypertension as esophageal varices, hepatic encephalopathy, TIPS procedure, ascites..., imaging results...) by studying medical record. |
| Biological, and evolutionary profiles. | 2 years | Evolution of liver blood results : ASAT, ALAT, GGT (same units of measure) |
| Evolution of liver function. | 2 years | Aggregation of results (Bilirubin, Albumin, prothrombin rate) into Child Pugh Score. |
Countries
France
Outcome results
None listed