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Y Pirson

Publications and source records attributed to Y Pirson.

At least 37 records · Page 2Linked to original sources
PubMed1999

Benign monoclonal gammopathy turning to AL amyloidosis after kidney transplantation.

The fate of preexisting benign monoclonal gammopathy after organ transplantation is largely unknown. We report the case of a 47-year-old male kidney graft recipient with a pretransplantation IgG kappa monoclonal gammopathy who developed, 10 years after transplantation, de novo augloid light chain (AL) amyloidosis involving skin and kidney graft. The potential role of heavy immunosuppressive treatment in the development of this complication is discussed. The possible occurrence of AL amyloidosis should be kept in mind when a patient with benign monoclonal gammopathy is evaluated for organ transplantation, as well as when a transplanted patient with pre-existing monoclonal gammopathy develops new onset of proteinuria.

Amyloidosis↗
PubMed1998

Recurrent fulminant anti-glomerular basement membrane nephritis at a 7-year interval.

True recurrence of anti-glomerular basement membrane (anti-GBM) nephritis is very rare, both in native kidneys and after renal transplantation. We report the recurrence of fulminant anti-GBM nephritis in a kidney graft recipient after the spontaneous withdrawal of immunosuppressive treatment more than 5 years after renal transplantation. The initial episode of anti-GBM nephritis had destroyed the native kidneys 7 years earlier. Circulating anti-GBM antibodies had disappeared for 14 months at the time of transplantation and reappeared with recurrence. This observation challenges the concept of anti-GBM nephritis as a single-shot illness and emphasises the need to consider the possibility of recurrence, even in the long term, among patients who underwent transplantation for anti-GBM nephritis.

Adult↗
PubMed1998

Combined heart-kidney transplantation: report on six cases.

BACKGROUND: Combined heart-kidney transplantation has become a new therapeutic solution for patients with coexisting, irreversible heart and kidney failure. Though this combined approach has several theoretical advantages over sequential transplantation, it has yet to be established that it does not jeopardize patient and graft outcomes. We here report our experience with six cases of combined heart-kidney transplantation from single donors and review the literature in order to clarify this issue. METHODS: Four patients were kidney-transplant candidates with severe heart failure and two were heart-transplant candidates with independent chronic renal failure. Donors were selected on the basis of weight and size matching, ABO compatibility, and negative T-cell cross-match. RESULTS: The heart was always grafted first. The surgical procedure was uneventful in all cases. Heart and kidney function recovered quickly in all patients. Two patients died, one at day 45 from heart subacute rejection and the other one at day 157 from cerebral haemorrhage. The four remaining patients are alive 23-84 months after transplantation (2-year survival rate: 67%) and have well-functioning kidneys (creatinine clearance 31-83 ml/min) and hearts (left ventricular ejection fraction 53-83%). Remarkably, four of six patients had no acute rejection episode of either organ. These patient and graft outcomes are in agreement with previous reports and compare favourably with the results of isolated heart and kidney transplantation. CONCLUSIONS: Combined heart-kidney transplantation from the same donor should be proposed to patients who would qualify for transplantation of each organ within a few years.

Adolescent↗
PubMed1998

Genetic studies into inherited and sporadic hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) in adults carries a high morbidity and mortality, and its cause remains unknown despite many theories. Although familial HUS is rare, it affords a unique opportunity to elucidate underlying mechanisms that may have relevance to acquired HUS. We have undertaken a genetic linkage study based on a candidate gene approach. A common area bounded by the markers D1S212 and D1S306, a distance of 26 cM located at 1q32 segregated with the disease (Z max 3.94). We demonstrate that the gene for factor H lies within the region. Subsequent mutation analysis of the factor H gene has revealed two mutations in patients with HUS. In an individual with the sporadic/relapsing form of the disease we have found a mutation comprising a deletion, subsequent frame shift and premature stop codon leading to half normal levels of serum factor H. In one of the three families there is a point mutation in exon 20 causing an arginine to glycine change, which is likely to alter structure and hence function of the factor H protein. Factor H is a major plasma protein that plays a critical regulatory role in the alternative pathway of complement activation. In light of these findings and previous reports of HUS in patients with factor H deficiency, we postulate that abnormalities of factor H may be involved in the etiology of HUS.

Adult↗
PubMed1998

[Physiopathology of diabetic nephropathy: what we learn from transplantation].

The contribution of clinical transplantation to the understanding of the pathophysiology of diabetic nephropathy is reviewed. Isolated kidney transplantation into diabetic recipients provides the opportunity to observe early lesions of recurrence, consisting in mesangium expansion and glomerular basement membrane thickening; in these patients, tight glycaemic control can significantly slow down the progression of recurrence. Simultaneous kidney-pancreas transplantation effectively prevents recurrence. Isolated pancreas transplantation in the presence of established diabetic nephropathy can prevent its progression and seems even able to induce its regression in the long-term provided transplantation takes place at an early stage of diabetic nephropathy. In summary, clinical transplantation confirms that the quality of glycaemic control is the main determinant of both the development and progression of diabetic nephropathy. It remains a unique opportunity to study its reversibility.

Animals↗
PubMed1998

Recurrence of membranous nephropathy after renal transplantation: probability, outcome and risk factors.

Recurrence of membranous nephropathy after renal transplantation has been reported either anecdotally or in a few series. In the present study, the potential risk factors as well as hitherto unreported actuarial risk for recurrence and graft loss due to recurrence were evaluated by combining data of a series of transplanted patients with MN at Louvain Medical School (n = 12) and at Lyon (n = 18; previously reported by Couchoud et al. 1995) giving a total of 30 patients. No risk factor for recurrence was identified as there was no statistical difference between the patients with and without recurrence for duration of MN in native kidneys or of pretransplant hemodialysis, presence of HLA-DR3, graft origin and use of cyclosporin. Actuarial risk for recurrence reached 29% at 3 years, plateauing up to 10 years. The outcome of recurrence was poor since the actual risk for graft loss among patients with recurrent MN was 38 and 52% at 5 and 10 years, respectively.

Actuarial Analysis↗
PubMed1997

[Renal fibromuscular dysplasia and cerebral aneurysm in a hypertensive patient with a familial history of cerebral vascular complications].

BACKGROUND: The possibility of an association between two vascular anomalies, renal artery dysplasia and cerebral artery aneurysm, merits recognition. CASE REPORT: We report the case of a 63 year old woman who was found to have fibromuscular dysplasia affecting the right renal artery while being investigated for systemic hypertension. Given a family history of cerebrovascular accident occurring before the age of 50, a cerebral angiogram was performed which demonstrated a saccular aneurysm of the middle cerebral artery measuring 6 mm in diameter. DISCUSSION: The association of these two anomalies could result from a familial arterial fibromuscular dysplasia. There are practical implications, notably the risk of aneurysm rupture and the role of hypertension.

Cerebrovascular Disorders↗
PubMed1997

[Combined kidney-pancreas transplantation: what are the costs and benefits, who are the recipients?].

As the requirement for immunosuppressive therapy after pancreas transplantation for insulin-dependent diabetes, which has compromised its use in non-uremic patients, cannot be avoided in uremic patients undergoing kidney transplantation, the benefits of combined kidney-pancreas transplantation might be sufficient to counterbalance the risks of the procedure. The International Pancreas Transplant Registry has collected data on over 7000 combined transplantations, allowing evaluation of the risks involved, assessment of patient benefit and identification of indications. Compared with renal graft alone, survival after combined transplantation is equivalent in patients under 45 years of age, but decrease in older patients and those with a history of heart failure. Actuarial survival of the pancreas graft, defined as absence of insulinotherapy, is currently 78% at 1 year and 65% at 5 years. Although combined transplantation may not reach early expectations regarding its affect on the progression of diasets complications patients quality of life is greatly improved after successful grafting as the daily constraints of regular meals, insulin injections and glycemia controls disappear at the same time as the burden of dialysis treatment. Combining a pancreas graft with a kidney graft inevitably increases morbidity during the post-operative months, but experience has shown that many young patients are very willing to pay the price in order to benefit from a combined graft. We currently propose combined transplantation in patients under 45 who are free of severe cardiovascular disease and accept to reconsider candidates after myocardial revascularization. The recent introduction of new immunosuppressive drugs such as tacrolimus and mycophenolate offer hope for further improvement in success rates. Despite currently disappointing clinical results, pancreatic islet-cell transplantation provides excitivy perspectives.

Adult↗
PubMed1997

Recurrent corneal erosion associated with Alport's syndrome. Rapid communication.

Ocular defects associated with Alport syndrome (AS) include anterior lenticonus and retinal flecks. We report on recurrent corneal erosion (RCE) as another ocular manifestation of the disease. Three brothers with AS reported a history of spontaneous attacks of RCE (2 episodes over 1 to 3 years in 2 of them and about 60 episodes in one brother over the last 10 years) characterized by acute ocular pain, lacrimation and photophobia lasting two to five days. The absence of RCE in the two other non-affected brothers from the same kindred suggested an association between AS and RCE, and prompted us to assess its prevalence. Forty-one patients with AS and renal failure and 67 control transplanted patients (with another original nephropathy) were evaluated. Seven AS patients had a history of RCE (first manifested between the ages of 12 and 21) versus only one control patient (P = 0.003). In conclusion, a history of RCE is found in about 20% of patients with AS and renal failure. RCE is likely to result from an inherent structural weakness of the corneal epithelial basement membrane (containing type IV collagen). A history of RCE should be sought when evaluating a patient for AS. Ophthalmologists should also be aware of this association, when confronted with a patient suffering from non-traumatic RCE.

Adolescent↗
PubMed1997

Mutation detection in the repeated part of the PKD1 gene.

The principle cause of one of the most prevalent genetic disorders, autosomal dominant polycystic kidney disease, involves mutations in the PKD1 gene. However, since its identification in 1994, only 27 mutations have been published. Detection of mutations has been complicated because the greater part of the gene lies within a genomic region that is reiterated several times at another locus on chromosome 16. Amplification of DNA fragments in the repeated part of the PKD1 gene will lead to coamplification of highly homologous fragments derived from this other locus. These additional fragments severely hamper point-mutation detection. None of the point mutations published to date are located in the repeated part of the PKD1 gene. However, we have reduced the problems posed by the strong homology, by using the protein-truncation test, and we have identified eight novel mutations, seven of which are located in the repeated part of the PKD1 gene.

Chromosomes, Human, Pair 16↗