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Biomedical subjects

Y Pirson

Publications and source records attributed to Y Pirson.

At least 73 records · Page 4Linked to original sources
PubMed1994

Early diagnosis of CMV infection by detection of pp65 antigen in 91 renal transplant recipients.

We evaluated how accurately a CMU antigenemia test correlated with classical CMU infection markers. We studied 91 kidney transplant recipients from February 1991 to June 1992. Antigenemia (pp65 antigen) was positive in 100% of cases of primary infection and in 70% of cases of reactivation and/or reinfection. Furthermore, antigenemia detected more infections (71%) than viremia (16%). The antigenemia test proved to be highly specific: it remained consistently negative in 22 seronegative patients, as well as in 19 of 20 seropositive recipients without recurrent infection. pp65 Antigen in the polymorphonuclear leukocytes was detected earlier than, or simultaneously with, virus culture in 78% of cases and became positive before serologic tests of primary and secondary infection in nearly 90% of cases. Most importantly, the antigenemia test detected all of the symptomatic cases.

Adolescent↗
PubMed1994

Intracranial aneurysms in autosomal dominant polycystic kidney disease.

Rupture of intracranial aneurysm (ICA) is a rare but severe manifestation of autosomal dominant polycystic kidney disease (ADPKD). In order to assess its natural history, to determine the prevalence of familial aggregation and to document linkage to PKD1 locus, we conducted a retrospective study on 77 ADPKD patients from 64 families presenting with ruptured (N = 71) or unruptured (N = 6) aneurysm. Information was collected on kidney disease, intracranial aneurysm and family history. Linkage to PKD1 locus was examined by five probes to obtain informative flanking markers. Within one year prior to rupture, blood pressure was normal in 29% of the patients. At the time of rupture, mean age was 39.5 years (range 15 to 69), renal function was normal in half of the patients and 11% were on renal replacement therapy. The ruptured aneurysm was usually located on the middle cerebral artery. Additional intact aneurysms (1 to 6) were detected in 31% of the patients. Surgical or endovascular treatment was performed in 54 (76%) patients whereas 17 (24%) had medical management only. Rupture of ICA was fatal in seven (10%) patients. On long-term follow-up 27 (38%) were left with severe disablement. Five patients bled from another aneurysm 2 days to 14 years after initial rupture. Only two of six patients with unruptured aneurysm alone were treated on a prophylactic basis. No clinical marker associated with aneurysm was found. A family history of aneurysm rupture was demonstrated in 10 (18%) kindreds. Linkage to the PKD1 locus was established in two of three tested families.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Outcome of HCV infection after renal transplantation.

The fate and significance of hepatitis C virus (HCV) infection after renal transplantation (TP) remain debated. We therefore evaluated the incidence and outcome of HCV infection in 120 kidney graft recipients both at the time of TP and 54 +/- 28 (13 to 123) months later using ELISA-II and RIBA-II immunoblot. Furthermore, the presence of anti-HCV antibodies at follow-up was correlated with HCV viremia, as detected by the nested polymerase chain reaction (PCR), and with chronically abnormal ALT levels. At the time of TP, 17 patients (14.2%) had anti-HCV antibodies. Compared to anti-HCV (-) patients, anti-HCV (+) patients had a longer duration of pre-TP dialysis (P < 0.001) and had received more pre-TP blood transfusions (P < 0.01). After an average follow-up of 48.5 +/- 24.7 (21 to 97) months, all these patients remained anti-HCV (+) but only 10 were still RIBA "reactive" due to the loss of reactivity against the 5-1-1, C100 and C33 (but not the C22) antigens. Five initially anti-HCV (-) patients had become (+) at follow-up. Among the 22 patients anti-HCV (+) at follow-up, 20 had HCV-RNA detectable by PCR but only 10 had elevated ALT. Ten out of 13 HBsAg (-) patients with elevated ALT were anti-HCV (+). Our study indicates that disappearance of ELISA-II anti-HCV antibodies is rare in kidney recipients and that HCV infection may also occur after TP. Anti-HCV antibodies are likely to reflect a persisting infection as suggested by the frequent detection of HCV-RNA.(ABSTRACT TRUNCATED AT 250 WORDS)

Alanine Transaminase↗
PubMed1994

Identification of mutations in the alpha 3(IV) and alpha 4(IV) collagen genes in autosomal recessive Alport syndrome.

Alport syndrome (AS) is an hereditary disease of basement membranes characterized by progressive renal failure and deafness. Changes in the glomerular basement membrane (GBM) in AS suggest that the type IV collagen matrix, the major structural component of GBM, is disrupted. We recently isolated the genes for two type IV collagens, alpha 3(IV) and alpha 4(IV), that are encoded head-to-head on human chromosome 2. These chains are abundant in normal GBM but are sometimes absent in AS. We screened for mutations in families in which consanguinity suggested autosomal recessive inheritance. Homozygous mutations were found in alpha 3(IV) in two families and in alpha 4(IV) in two others, demonstrating that these chains are important in the structural integrity of the GBM and that there is an autosomal form of AS in addition to the previously-defined X-linked form.

Adolescent↗
PubMed1994

Evaluation in patients with Alport syndrome of knowledge of the disease and attitudes toward prenatal diagnosis.

Cloning of the COL4A5 gene has now made possible prenatal testing for Alport syndrome with X-linked dominant inheritance. We interviewed 27 females and 24 males with Alport syndrome to evaluate their knowledge of the disease and its transmission, and their attitudes to prenatal testing. Twenty-two males and 8 females were on renal replacement therapy. In all cases transmission was compatible with X-linked disease. Only 59% of the interviewees (74% of women, 42% of men) knew that gender was the major determinant in progression of the disease. Knowledge of the mode of inheritance was adequate in only 25%, in both sexes. Seventy percent of the participants (78% of women, 63% of men) would use prenatal testing. Of the women in favor of prenatal diagnosis, 67% and 39% would terminate pregnancy in the case of an affected male or female fetus, respectively. Of the men in favor of prenatal diagnosis, 53% would consider termination of an affected fetus. In summary, a majority would use prenatal testing, but only one or two thirds of them wished to use selective abortion. As in other inherited disorders, there is a discrepancy between the demand for prenatal diagnosis and the decision to terminate pregnancy. Most of the participants who would terminate a pregnancy had, however, little knowledge of the clinical and genetic aspects of Alport syndrome on which to base such a decision. An important aspect of genetic counselling is to assist consultants in reaching a decision regarding future reproductive behaviour which is appropriate to their situation. This study underlines the need to improve education and counselling to assure appropriate use of prenatal testing.

Abortion, Eugenic↗
PubMed1993

Epiphyseal impaction as a cause of severe osteoarticular pain of lower limbs after renal transplantation.

A syndrome of severe osteoarticular pain of lower limbs occurring early after renal transplantation (TP) has been recently identified. We describe its prevalence, clinical presentation and outcome. Symptomatic patients have been studied with conventional X-rays, magnetic resonance (MR) imaging, and 99mTechnetium scintigrams of the painful areas. Among 86 patients transplanted over a two-year period, nine (4 men, 5 women; mean age of 40.4 years; range 32 to 59) developed unexplained severe spontaneous osteoarticular pain of lower limbs 19 to 105 (mean 58) days after TP. Pain affected hip(s), knee(s), and/or ankle(s). Clinical examination was usually unremarkable. Favorable outcome was the rule; mean duration of pain was 86 (range 19 to 175) days. Radiographs were abnormal (joint swelling, patchy osteoporosis and/or periosteal reactions) in 41%, MR (epiphyseal fatty marrow replacement by edema and/or hemorrhages) in 83%, and bone scans (one to several epiphyseal foci of increased uptake) in 81% of the symptomatic examined areas. Among joints re-examined 9 to 12 months after resolution of the symptoms, X-rays showed periosteal reactions in 31%, and the bone scans disclosed persistent increased uptake in 53% of the joints, whereas epiphyseal MR abnormalities had completely disappeared in 86%. There was no difference in dialysis duration, post-TP weight gain, evidence of hyperparathyroidism, and steroid and cyclosporine doses between symptomatic and an appropriately selected group of asymptomatic patients. By contrast, serum alkaline phosphatase levels were transiently higher (at the onset of symptoms) in the symptomatic group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1992

Kidney transplantation in patients with Wilms' tumor.

We report our experience of renal transplantation in three patients treated for Wilms' tumor (with lung metastasis in two of them), and review 26 previously reported cases in order to define the current indications of transplantation in this setting. Our patients, aged 5-12 years, were transplanted 13-95 months after completion of Wilms' tumor treatment. All three are alive and tumor-free, two with a functioning graft 20 and 97 months after transplantation. Two findings emerge from the review of the literature. First, posttransplant mortality is influenced by the delay between completion of tumor treatment and transplantation. Mortality reaches 79% when that delay is less than one year but falls to 27% when that delay exceeds one year. Second, the prognostic value of pretransplant metastasis depends on its location. All four patients with pretransplant abdominal metastasis died with active metastatic disease. By contrast, of three patients treated before transplantation for metastasis confined to the lung, two are alive and tumor free. We conclude that renal transplantation should be offered to patients successfully treated for Wilms' tumor for at least one year, even if the disease has been complicated by pulmonary metastasis. Several long-term survivors attest that the disease can be cured even under maintenance immunosuppression.

Child, Preschool↗
PubMed1992

No association between Alport's syndrome and antithyroid antibodies.

The presence of circulating antithyroid antibodies in a small number of patients with Alport's syndrome has led some to suggest that the two abnormalities are associated. If confirmed, such an association could have important diagnostic as well as pathophysiological implications. Using sensitive methods, we assessed the prevalence of antithyroid antibodies in 40 patients with Alport's syndrome from 28 families. Antithyroglobulin antibodies were absent in all patients. Antimicrosomal and antithyroperoxidase antibodies were absent in all but one patient. Three patients with Alport's syndrome from the latter family were negative for antithyroid antibodies. In this large number of patients we found no association between Alport's syndrome and antithyroid antibodies. Screening for the presence of such antibodies in patients with Alport's syndrome is thus not warranted. The previously reported coexistence of the two disorders might be fortuitous.

Adult↗
PubMed1992

[Aseptic necrosis of the femoral head following renal transplantation: assessment of a 25-year experience].

From June 1963 to December 1988 aseptic necrosis of the femoral head has been treated surgically in 84 renal transplant recipients (150 surgical procedures). The long-term results of drilling of the neck and head of the femur (16), cup arthroplasty (32), cemented cup (1) and hemiarthroplasty (8) were unsatisfactory, as 23 of these 57 hips underwent a secondary procedure. Total hip arthroplasty progressively became the standard procedure for treatment of hip disease in transplanted patients. Since 1971, 63 renal transplant recipients underwent 92 cemented total hip replacement (THR) as a primary (73), secondary (16) or third (3) surgical procedure for severely symptomatic femoral head necrosis. Hospital stay averaged 22 days, and follow-up averaged 53 months. Two deaths related to the surgical procedure occurred in the first 4 years of our experience (one major local sepsis, one pulmonary infection). Other postoperative complications were urinary tract infection (12), pulmonary infection 1, transient sciatic nerve irritation (3), wound hematoma (6), reversible deterioration of renal function (3) and rejection of the graft (2). Thromboembolic complications did not occur. All operated hips showed a marked symptomatic improvement. Loosening of one or both components was definite in one, probable in two and possible in three of the 33 hips followed up more than 5 years. Other late complications included dislocation (6), painful class III heterotopic ossification (4), recurrence of previous sepsis (1) and late hematogenous sepsis. Late hip revision was required in 5 cases (recurrent dislocation, 1, ossification, 2, sepsis, 2). Two renal complications (one graft infarction and one reversible acute tubular necrosis) occurred after these revisions. The functional results of THR compare favourably with the results of other surgical procedures used in our early experience. We conclude that THR has become the treatment of choice for symptomatic established osteonecrosis of the femoral head in renal transplant patients. A relatively high rate of early and late complications is nevertheless to be expected.

Adolescent↗
PubMed1991

Fundus changes in membranoproliferative glomerulonephritis type II. A fluorescein angiographic study of 23 patients.

A total of 23 patients aged between 11 and 64 years who had biopsy-proven membranoproliferative glomerulonephritis type II (dense deposit disease) were studied using fluorescein angiography of the retina. With the exception of two adolescents, all patients exhibited small subretinal nodules that were similar to basal laminar drusen. Subjects with a long history of renal disease displayed more numerous and larger nodules as well as atrophic changes. Four subjects presented with subretinal neovascular membranes.

Adolescent↗