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Biomedical subjects

Y Pirson

Publications and source records attributed to Y Pirson.

At least 19 recordsLinked to original sources
PubMed1996

Isolated polycystic liver disease as a distinct genetic disease, unlinked to polycystic kidney disease 1 and polycystic kidney disease 2.

Polycystic liver disease (PLD) is proven to occur either sporadically or in association with autosomal dominant polycystic kidney disease (ADPKD), whereas the existence of an isolated (i.e., without any kidney cyst) familial form is disputed. We describe a family with definitely isolated PLD transmitted through three generations and exclude the linkage of the disease to the genetic markers of PKD1 and PKD2, the two main loci responsible for ADPKD. These findings strongly support the existence of PLD as a genetic disease distinct from the known forms of ADPKD.

Cysts↗
PubMed1996

Hepatitis C infection in renal transplant patients: new insights and unanswered questions.

The prevalence of HCV infection in a population of renal transplantation patients is mostly dependent on that preexisting before transplantation. It also has been demonstrated that HCV infection can be transmitted by the renal graft. Although grafting an HCV+ kidney does not affect survival 5 years after surgery, the risk incurred by recipients on the longer term is unclear. A suggestion has been made to reserve HCV+ kidneys for recipients who are themselves HCV+. However, it has been established that a given HCV strain has little chance of inducing immunity to a different HCV strain. This is why the use of HCV+ kidneys no longer meets consensus. It could be considered to match the recipient and the graft with regard to the HCV strain when genotype identification is routinely available, quick and reliable. Immunosuppressive therapy enhances viral replication. Its long-term effect on the course of HCV disease is unclear. In particular, no studies have compared the long-term outcome of HCV+ patients treated by haemodialysis and transplantation. The data available on the 10-year outcome of HCV+ grafted patients are nonetheless reassuring. At least they allow considering renal transplantation to non-cirrhotic HCV+ patients. Several issues related to the interaction between HCV and immunosuppressive therapy remain to be clarified. Does the viral strain play a role in the course of infection under immunosuppressive treatment? Does immunosuppressive treatment promote strain mutagenesis? Does HCV infection require modulation of the immunosuppressive treatment?

Antibodies, Viral↗
PubMed1996

Complications of autosomal dominant polycystic kidney disease in 50 haemodialysed patients. A case-control study. The U.C.L. Collaborative Group.

BACKGROUND: The impact of renal and extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD) during chronic haemodialysis (HD) has never been assessed in a paired case-control study. METHODS: Comparison of the course of 50 ADPKD patients with 50 matched control (C) patients who started chronic HD at the same time. RESULTS: Follow-up averaged 48 and 39 months in the ADPKD and C groups respectively. Actuarial survival was similar in both groups. Prevalence of renal pain (36 vs 2%, P=0.0001), haematuria (36 vs 16%, P<0.03) and renal infection (16 vs 2%, P<0.04) was higher in the ADPKD than in the C group. Nephrectomy during HD was performed in six ADPKD (in 4 cases in preparation for transplantation) and in one control patient. Number of patients with coronary and heart valve complications was similar in both groups. Stroke occurred in three patients from both groups. Only two ADPKD patients experienced a single episode of pain related to liver cyst. Prevalence of severe infection was similar in the ADPKD group (36%) and the C group (28%). Number and duration of hospitalizations were similar in both groups. CONCLUSIONS: The overall outcome of ADPKD patients on maintenance HD is similar to that of HD patients with other primary renal diseases. Complications related to cystic kidneys are frequent but rarely severe. Extrarenal manifestations of ADPKD have a limited clinical impact in this short-term study.

Adult↗
PubMed1995

Autosomal recessive Alport syndrome: immunohistochemical study of type IV collagen chain distribution.

Alport syndrome (AS) is an hereditary disease of basement membrane collagen. It is mainly transmitted as a dominant X-linked trait and caused by mutations in the COL4A5 gene encoding the alpha 5 chain of type IV collagen. However, autosomal recessive AS due to mutations in the COL4A3 or COL4A4 genes could represent up to 15% of AS. Using the immunofluorescence technique, we analyzed the distribution of the different chains of type IV collagen in renal (12 specimens) and skin (4 specimens) basement membranes of 12 AS patients belonging to 11 unrelated kindreds in which autosomal recessive inheritance had been demonstrated (3 kindreds) or was suggested by clinical and genealogic data (8 kindreds). The renal and skin distribution was normal in one patient with COL4A4 mutations. A peculiar pattern of distribution of the alpha 3-alpha 5(IV) chains was observed in the other patients. It was characterized the co-absence of the alpha 3(IV), alpha 4(IV) and alpha 5(IV) chains in the glomerular basement membrane, and the presence of the alpha 5(IV) chain in a series of extraglomerular basement membranes including capsular, collecting ducts and epidermal basement membranes, a combination never observed in X-linked AS. This immunohistochemical pattern is correlated with the specific distribution of the alpha 3-alpha 5 chains of type IV collagen chains within extraglomerular basement membranes. It could be a useful marker for the identification of autosomal recessive AS.

Adolescent↗
PubMed1995

[Pulmonary-renal syndrome: diagnostic and therapeutic strategy].

The pulmonary-renal syndrome is defined by the association of alveolar hemorrhage and rapidly progressive glomerulonephritis. Goodpasture syndrome and necrotizing vasculitides are the most frequent causes. New serologic markers are currently more rapidly available (anti-glomerular basement membrane--anti-GBM--and antineutrophil cytoplasmic antibodies -ANCA), allowing clinicians to identify and distinguish these 2 entities, and to hasten the initiation of a pulse therapy, now well standardized, which improves the outcome of patients. However, these 2 serologic markers have limitations so that clinical assessment and renal biopsy remains essential in the diagnosis of pulmonary-renal syndrome. The authors propose an algorithmic approach for those confronted with such a condition.

Anti-Glomerular Basement Membrane Disease↗
PubMed1995

Implications of chronic hepatitis B or hepatitis C infection for renal transplant candidates.

Hepatic cirrhosis and clinically active hepatitis due to HBV or HCV infection clearly contra-indicate kidney transplantation. More controversial is the attitude to be adopted towards candidates with clinically quiescent chronic HBV or HCV infection. The presence of the HBs antigen does not adversely affect survival or increase morbidity on maintenance haemodialysis, at least during the first decade. After transplantation, by contrast, the long-term outcome of HBV infection is undoubtedly worse than on haemodialysis: more patients develop chronic hepatitis and eventually die from liver disease. The risk of fatal liver disease after transplantation is greater in patients with markers of active viral replication before transplant and in those with severe histological liver lesions. Pretransplant candidates should be warned of this significant risk factor. Comparison of survival of HCV-infected patients on haemodialysis and after transplantation is not yet possible. The outcome of HCV infection after transplantation appears less severe than that of HBV infection: the survival of anti-HCV-positive patients is similar to that of anti-HCV-negative patients, at least during the first decade after transplantation. Liver biochemical abnormalities, serological markers and detection of HCV RNA are of little value to identify patients at greater risk of poor outcome after transplantation. Only liver biopsy might help identify such patients. Both efficacy and risks of antiviral therapies are yet to be properly assessed during haemodialysis. Preliminary evidence suggests that interferon therapy given after transplantation entails an unacceptable rate of deterioration in graft function.(ABSTRACT TRUNCATED AT 250 WORDS)

Attitude of Health Personnel↗
PubMed1994

Course of Henoch-Schönlein nephritis after renal transplantation. Report on ten patients and review of the literature.

The frequency and the risk factors for clinical recurrence of Henoch-Schönlein nephritis following renal transplantation (TP) remain largely unknown. We report on 14 transplants performed at our center in 10 patients, detail the evolution of 2 of them with clinical recurrence, and review 64 other transplants reported in the literature. In our series, all patients are currently alive. Seven grafts are well-functioning 22-295 (mean, 97) months after TP without any sign of clinical recurrence. Five grafts were lost from rejection. Clinical recurrence occurred in 2 patients who were on cyclosporine/azathioprine/prednisone therapy. Pooling our series with that of Hasegawa et al., the actuarial risk for renal recurrence and for graft loss due to recurrence was 35 and 11% at 5 years after TP, respectively. In our series, duration of original disease was 2 and 28 months in the 2 patients with recurrence versus 31-144 months in the others without recurrence. In the literature, this duration was < or = 36 months in all 7 patients with recurrence. Recurrence occurred despite a > 12-month delay between disappearance of purpura and TP in our 2 patients and in 3 of 6 previously reported recurrences. We conclude that Henoch-Schönlein purpura nephritis frequently recurs after TP. Recurrence (1) seems to be associated with a shorter duration of the original disease, (2) can occur despite a delay of more than 1 year (as commonly advised) between disappearance of purpura and TP, and (3) is not prevented by a triple immunosuppressive regimen that includes cyclosporine.

Adolescent↗