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Y Pirson

Publications and source records attributed to Y Pirson.

At least 19 recordsLinked to original sources
PubMed2003

Post-renal transplant syndrome of transient lower limb joint pain: description under a tacrolimus-based immunosuppression.

The occurrence of a post-renal transplant syndrome of lower limbs joint pain has been reported extensively over the last decade. Clinical examination of the symptomatic joints is often unremarkable and magnetic resonance imaging reveals abnormalities of the bone marrow suggestive of edema and/or hemorrhage. The main striking features of this syndrome are the spontaneous resolution of the symptoms within a few weeks as well as of the marrow abnormalities. This syndrome has been attributed to cyclosporine, given in the immunosuppression regimen or to epiphyseal impactions. We here document the occurrence of this syndrome in 5 kidney graft recipients given a tacrolimus-based immunosuppression.

Adult↗
PubMed2003

Evaluation of bone mineral density after renal transplantation under a tacrolimus-based immunosuppression: a pilot study.

BACKGROUND: Progressive bone loss consistently complicates renal transplantation (TP) in patients given an immunosuppression including prednisolone. The adjunction of cyclosporine in the immunosuppressive regimen does not reverse the negative impact of renal TP on the skeleton. The post-transplant effect of tacrolimus on bone mass is still unknown. METHODS: We evaluated the evolution of bone mineral density (BMD) and various biochemical markers over the first 12 months following renal TP in 23 patients given an immunosuppression combining tacrolimus and low-dose prednisolone. BMD of lumbar spine, total hip and hip subregions was measured by dual-energy X-ray absorptiometry within the first 15 days and 1 year after TP. RESULTS: At the time of TP, the average BMD was low in both the lumbar spine and the hip. After TP, a normalization of serum creatinine and a decrease in serum phosphate and iPTH levels occurs. Serum alkaline phosphatase level significantly rose transiently within the first 6 months and decreased thereafter. At 1 year post TP, BMD remained unchanged in the lumbar and in the trochanter subregions and rose in the other sites. BMD increased by at least 2% in 8, 13, 10 and 10 out of the 23 patients in the lumbar, neck, trochanter and total hip subregions, respectively. No correlation was found between evolution in BMD and age, sex, dialysis duration, level of hyperparathyroidism, prednisolone and tacrolimus cumulative intake and prescription of calcium, vitamin D or hormone replacement therapy. CONCLUSIONS: An immunosuppression combining tacrolimus and low-dose prednisolone might avoid the usual post-TP bone loss. Further randomized double-blind studies evaluating a larger cohort of patients should be undertaken to compare the effect of cyclosporine and tacrolimus on bone mass.

Absorptiometry, Photon↗
PubMed2003

Massive amoxycillin crystalluria causing anuric acute renal failure.

The authors report the case of a 45-year-old woman admitted for pneumonia who presented anuric acute renal failure after 12 days of intravenous amoxycillin-clavulanate treatment. Acute renal failure resolved rapidly and completely after antibiotic withdrawal. Analysis of the first post-anuric urine specimen showed many crystals. Infrared spectrophotometry revealed that the crystals were composed of trihydrated amoxycillin. The possibility of intrarenal obstruction due to massive drug crystalluria should not be overlooked in the face of abrupt anuria.

Acute Kidney Injury↗
PubMed2002

Modifier effect of ENOS in autosomal dominant polycystic kidney disease.

A significant phenotypical variability is observed in autosomal dominant polycystic kidney disease (ADPKD). ADPKD is associated with altered endothelial-dependent vasodilation and decreased vascular production of nitric oxide (NO). Thus, ENOS, the gene coding for the endothelial nitric oxide synthase (eNOS), could have a modifier effect in ADPKD. In order to test this hypothesis, we genotyped 173 unrelated ADPKD patients from Belgium and the north of France for the Glu298Asp, intron 4 VNTR and T-786C polymorphisms of ENOS and looked for their influence on the age at end-stage renal disease (ESRD). In males (n = 93), the Glu298Asp polymorphism was associated with a lower age at ESRD (Glu/Asp + Asp/Asp: 49.0 +/- 1.2 years, n = 53; Glu/Glu: 53.5 +/- 1.5 years, n = 40; simple regression, P = 0.02; multiple regression, P = 0.006). This effect was confirmed in a subset of males linked to PKD1 and reaching ESRD before age 45, and by a cumulative renal survival analysis in PKD1-linked families. Further studies demonstrated that NO synthase (NOS) activity was decreased in renal artery samples from ADPKD males harbouring the Asp298 allele, in association with post-translational modifications and partial cleavage of eNOS. No significant effect of the other polymorphisms was found in males, and no polymorphism influenced the age at ESRD in females. In conclusion, the frequent Glu298Asp polymorphism of ENOS is associated with a 5 year lower mean age at ESRD in this subset of ADPKD males. This effect could be due to a decreased NOS activity and a partial cleavage of eNOS, leading to a further decrease in the vascular production of NO.

Age of Onset↗
PubMed2001

Autosomal-dominant periodic fever with AA amyloidosis: Novel mutation in tumor necrosis factor receptor 1 gene Rapid Communication.

BACKGROUND: The recent identification of genes responsible for syndromes of periodic fever with amyloidosis has opened the way to a molecular diagnosis of hereditary AA amyloidosis. METHODS: A Belgian woman presented for genetic counseling. Three first-degree relatives had a diagnosis of renal amyloidosis with a history of recurrent fever and inflammatory episodes. Medical records and pathological specimens were obtained from all physicians who had been in charge of her three relatives. Immunohistochemical staining was performed on paraffin-embedded material. A mutation search was performed in the MEFV (Mediterranean fever) and tumor necrosis factor receptor 1 (TNFR1 or TNFRSF1A) genes causing familial Mediterranean fever (FMF) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), respectively. RESULTS: The family history was consistent with autosomal-dominant transmission of periodic fever with arthralgias, abdominal pain, and eventual AA amyloidosis involving the kidneys, digestive tract, and thyroid. Recurrent amyloidosis in kidney graft was demonstrated in one patient and was suspected in the other. A novel heterozygous mutation (C55S) in TNFRSF1A was identified in the affected patient available for genetic testing but not in the asymptomatic woman requiring counseling. No mutation was detected in MEFV. CONCLUSIONS: We report a novel mutation (C55S) in TNFRSF1A, resulting in autosomal-dominant periodic fever and AA amyloidosis. This condition, known as TRAPS, should be added to the differential diagnosis of hereditary renal amyloidosis, with obvious implications for management and genetic counseling.

Adult↗
PubMed2001

Increase of physical activity level after successful renal transplantation: a 5 year follow-up study.

BACKGROUND: Physical activity (PA) level of end-stage renal disease (ESRD) patients after renal transplantation (TP) is a largely unexplored field, although it is an important component of quality of life. METHODS: Using the Baecke self-administered and the Five-City Project 7-day PA recall questionnaires, PA level was estimated in 32 consecutive ESRD patients (12 males, 20 females; mean age 45.9+/-13.1 years; mean dialysis duration 23.5+/-21.8 months) admitted for renal TP and to whom no exercise programme of any kind was proposed. PA were recorded 1, 3, 6, 12 and 60 months after TP. RESULTS: Immediate pre-TP PA level of renal transplant candidates was between 18 and 35% less than that of age-matched healthy subjects (P < 0.05), depending on gender and questionnaire. After an immediate decrease in PA level 1 month post-TP, mean PA level increased and reached a plateau 1 year after TP. This gain in PA capacity reached 30%, as compared with pre-TP values (P = 0.06 to P < 0.01). During the fifth year after TP, the mean level of PA was unchanged. A more qualitative analysis, allowed by the sub-score comparisons, showed that although the occupational status of the patients remained the same, they participated significantly more in moderate and even high intensity PA (leisure, sports, household chores) after TP. CONCLUSIONS: Most renal graft recipients are spontaneously more active after TP, an observation consistent with a better quality of life. Therefore, they should be advised precisely about how to resume more strenuous activities such as sports in order to avoid cardiac or musculoskeletal disorders in relation to their weakened pre-TP condition.

Adult↗
PubMed2001

Autosomal recessive polycystic kidney disease in adulthood.

BACKGROUND: Renal cysts arising from collecting ducts, congenital hepatic fibrosis, and recessive inheritance characterize autosomal recessive polycystic kidney disease (ARPKD). The disorder usually manifests in infancy, with a high mortality rate in the first year of life. For the patients who survive the neonatal period, the probability of being alive at 15 years of age ranges from 50 to 80%, with 56--67% of them not requiring renal replacement therapy at that stage. Some develop portal hypertension. Long-term outcome of adults escaping renal insufficiency above age 18 is largely unknown. METHOD: In consecutive patients with ARPKD and autonomous renal function at age 18, clinical course of kidney and liver disease in adulthood and status at last follow-up were evaluated. Progression of renal insufficiency was assessed by the rate of decline of creatinine clearance, according to Schwartz's formula before age 18 and Cockcroft and Gault formula thereafter. Severity of liver involvement was estimated by imaging techniques, liver function tests, and endoscopy. RESULTS: Sixteen patients from 15 families were included. ARPKD was diagnosed between 1 day and 13 years of age. From diagnosis, mean follow-up period lasted 24+/-9 years. Before age 18, nine patients (56%) were hypertensive, nine (56%) had renal failure, and four (25%) had portal hypertension. Beyond age 18, no additional patient became hypertensive, and another five developed progressive renal insufficiency; altogether, the mean yearly decline of creatinine clearance was 2.9+/-1.6 ml/min. Portal hypertension was recognized in two additional patients. Four patients experienced gastro-oesophageal bleeding, while recurrent cholangitis or cholangiocarcinoma developed in one case each. At the end of follow-up, 15/16 patients (94%) were alive at a mean age of 27 (18--55) years. Two patients had a normal renal function, 11 had chronic renal insufficiency, one was on regular dialysis, and two had functioning kidney grafts. Four patients had required a porto-systemic shunt. CONCLUSIONS: A subset of ARPKD patients with autonomous renal function at age 18 experiences slowly progressive renal insufficiency. With prolonged renal survival, complications related to portal hypertension are not rare, requiring careful surveillance and appropriate management.

Adolescent↗
PubMed2001

Outcome of cadaver kidney transplantation in 23 patients with type 2 diabetes mellitus.

BACKGROUND: Type 2 diabetes mellitus (DM) is a growing cause of end-stage renal failure worldwide. Yet, only a minority of type 2 diabetics are considered today for kidney transplantation (KT). The scarcity of data on the outcome of such patients after KT prompted us to review our experience. METHODS: Between 1 January 1983 and 30 June 1996, 23 patients with type 2 DM received a first cadaver KT at a mean age of 57+/-9 (41-73) years, after a dialysis period ranging from 5 to 72 (mean 25+/-18) months. Only nine patients had a history of coronary and/or peripheral vascular disease before KT. All were given cyclosporin- or tacrolimus-based immunosuppression. Post-KT follow-up ranged from 4 to 181 (mean 70+/-38) months. Outcome analysis focused on the impact of cardiovascular complications. RESULTS: Patient survival at 1, 5 and 8 years was 91, 83 and 76% respectively. Death was due to infection in three patients and to a cardiovascular event in two. The actuarial risk of coronary, cerebrovascular, peripheral vascular, and any cardiovascular event after KT was 14, 13, 9 and 30% at 1 year, 20, 13, 50 and 58% at 5 years, and 20, 46, 66 and 72% at 8 years respectively. Post-KT hospital readmissions averaged 10 days/patient-year and were mostly related to the management of peripheral vascular disease. CONCLUSION: KT is an excellent therapeutic option for selected patients with type 2 DM. Peripheral vascular disease is the leading cause of morbidity following KT. KT should be considered in type 2 diabetics with a low/medium cardiovascular risk.

Adult↗
PubMed2000

Identification of a locus for autosomal dominant polycystic liver disease, on chromosome 19p13.2-13.1.

Polycystic liver disease (PCLD) is characterized by the growth of fluid-filled cysts of biliary epithelial origin in the liver. Although the disease is often asymptomatic, it can, when severe, lead to complications requiring surgical therapy. PCLD is most often associated with autosomal dominant polycystic kidney disease (ADPKD); however, families with an isolated polycystic liver phenotype without kidney involvement have been described. The clinical presentation and histological features of polycystic liver disease in the presence or absence of ADPKD are indistinguishable, raising the possibility that the pathogenetic mechanisms in the diseases are interrelated. We ascertained two large families with polycystic liver disease without kidney cysts and performed a genomewide scan for genetic linkage. A causative gene, PCLD, was mapped to chromosome 19p13.2-13.1, with a maximum LOD score of 10.3. Haplotype analysis refined the PCLD interval to 12.5 cM flanked by D19S586/D19S583 and D19S593/D19S579. The discovery of genetic linkage will facilitate diagnosis and study of this underdiagnosed disease entity. Identification of PCLD will be instrumental to an understanding of the pathogenesis of cyst formation in the liver in isolated PCLD and in ADPKD.

Adult↗
PubMed2000

Late-onset renal failure in Senior-Loken syndrome.

We report on four patients, from three different families, with Senior-Loken syndrome (SLS). They were unusual in that they reached end-stage renal failure (ESRF) only during the fifth or sixth decade. SLS is an autosomal-recessive disorder defined by the association of nephronophthisis and retinal dystrophy. Affected individuals invariably progress to ESRF, usually before the age of 20 years. The diagnosis was based on typical clinical presentation and characteristic renal histology, that is, a picture of chronic interstitial nephritis with pronounced thickening and multilayering of tubular basement membranes. Deterioration of renal function was slow, leading to ESRF between the ages of 42 and 56 years. Retinal dystrophy, already symptomatic during childhood in two patients, led to severe visual impairment in all. In contrast with four cases of SLS recently reported in very young patients, the NPH1 gene (the main gene responsible for nephronophthisis) was not deleted in our two tested patients. We conclude that SLS should be considered in adults who suffer from both chronic interstitial nephropathy and retinal degeneration. Whether the SLS is a variant of nephronophthisis and whether early- and late-onset renal failure in SLS is accounted for by genetic or allelic heterogeneity remain to be determined.

Adult↗
PubMed2000

Kidney and kidney-pancreas transplantation in diabetic recipients.

Type 1 as well as Type 2 diabetic patients in end-stage renal failure and with no contraindications to kidney transplantation have a greater probability of survival with a functioning kidney graft than if they remain on dialysis. Five-year patient and pancreas graft survival rates for simultaneous kidney-pancreas transplantation are currently 81 and 67% respectively. The main benefit of this operation is to achieve insulin independence and improved quality of life. However, surgical morbidity is higher and the immunosuppressive regimen more powerful than for kidney transplantation alone. The 5-year survival rate for kidney transplantation in Type 2 patients without severe cardiovascular disease is 81%, although a high incidence of peripheral vascular complications can be expected. Renal transplantation should be considered in diabetic patients with a life expectancy of more than 5 years, no contraindications to immunosuppressive treatment, and low perioperative risk. Combined kidney-pancreas transplantation should be considered in Type 1 patients under 50 years of age with no or moderate cardiovascular complications and a thorough understanding of the risks and benefits of the procedure.

Diabetes Mellitus, Type 1↗
PubMed1999

Malakoplakia of the caecum in a kidney-transplant recipient: presentation as acute tumoral perforation and fatal outcome.

Malakoplakia is a rare pseudotumoral inflammatory disease known to affect immunocompromised subjects, mainly with a history of recurrent Escherichia coli infection. The urinary tract is the most frequent site of the disease, although all organs can be involved. In the present article, we report a case of malakoplakia of the caecum, that developed in a 52-year-old man, who had received a kidney transplant 9 years before and had a history of recurrent E. coli urinary tract infections. Malakoplakia presented as acute intestinal perforation, and, despite aggressive surgical and medical management, disease progressed toward a fatal outcome due to sepsis and multiple organ failure 9 months later. A defect in the macrophagic activity was demonstrated.

Bacteremia↗