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Clinical Genetics 08/2011; 80(2):199-201. · 3.13 Impact Factor
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ABSTRACT: To assess patient survival in pediatric renal transplantation, we retrospectively reviewed 573 transplants in 553 patients, registered from 1995 to 2005.
Mean age at transplantation was 9.9 years. Patient survival at 1, 5 and 10 years was respectively 99%, 97% and 96%. Death occurred at a median time of 2.6 years after transplantation. Long-term patient survival was significantly lower in recipients younger than 5 years old. Seventeen patients (3.1%) died. Two deaths occurred while under maintenance dialysis. Among the remaining patients, the two main causes of death were infections (33%) and malignancies (27%). Interestingly, initial disease-related complications were a major cause of death (34%).
A low mortality rate was observed, with the majority of deaths due to malignancies and infections, and with a notable participation of complications related to the initial disease. No impact of cardiovascular disease was noted with the given follow-up period. Improvements in managing immunosuppression may contribute to reducing mortality in pediatric renal transplantation.
Pediatric Transplantation 10/2009; 13(6):725-30. · 1.48 Impact Factor
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M A Cosson,
G Touati,
F Lacaille,
V Valayannnopoulos,
C Guyot, G Guest,
V Verkarre,
D Chrétien,
D Rabier,
A Munnich,
J F Benoist,
Y de Keyzer,
P Niaudet,
P de Lonlay
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ABSTRACT: A boy who was diagnosed with methylmalonic aciduria (MMA) at the age of 10 days developed persistent hepatomegaly and raised transaminases from the age of 4 years. He was subsequently diagnosed with Leigh syndrome and required a kidney transplantation for end-stage renal failure. A massive hepatoblastoma led to his death by the age of 11 years. Methylmalonyl-CoA mutase activity was undetectable on both cultured skin fibroblasts and kidney biopsy and multiple respiratory chain deficiency was demonstrated in the kidney. Mitochondrial dysfunction and/or post-transplant immunosuppressive therapy should be considered as a possible cause of liver cancer in this patient.
Molecular Genetics and Metabolism 09/2008; 95(1-2):107-9. · 3.19 Impact Factor
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ABSTRACT: From 1990 to 2000, we performed eight liver-kidney transplants in eight children, aged 1-16 years, with end-stage renal failure (ESRF) due to primary hyperoxaluria (PH1). The duration of dialysis before transplantation ranged from 2 to 42 months (mean 14 months) and was <1 year in four patients. Only the first patient underwent postoperative hemodialysis; in the other five, we chose to induce maximal diuresis from the first hours with intravenous and intragastric hyperhydration (> or =3 l/m2 per day). High water intake with nocturnal tube hydration was maintained for 6 months to 5 years, as long as oxaluria exceeded 0.5 mmol/day. A quadruple sequential immunosuppressive regimen was used. Two patients died during liver graft surgery. The other six patients are alive and well, with a mean follow-up of 7.4 years (range 5-11 years). Patient and graft survival is 75% at 5 years. At latest follow-up, liver tests were normal in all six patients; creatinine clearance ranged from 55 to 95 ml/min per 1.73 m2 (mean=74). Oxaluria was lower than 0.4 mmol/day in all patients (mean=0.22). The six patients underwent 15 renal biopsies, 1-11 years after transplantation. Chronic transplant nephropathy was present in four patients and mild cyclosporin nephrotoxicity in another. No oxalate crystals were seen and repeat ultrasonography has been consistently normal in all patients. The three patients with bone oxalosis showed progressive complete healing of bone lesions. All six children or adolescents now live a normal life. From this series, we conclude that early combined liver-kidney transplantation is the treatment of choice for children with ESRF due to primary hyperoxaluria.
Pediatric Nephrology 12/2001; 16(12):946-50. · 2.52 Impact Factor
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Archives de Pédiatrie 06/2001; 8 Suppl 2:215s-217s. · 0.30 Impact Factor
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ABSTRACT: There is a tendency toward less favorable long-term graft function in patients with posterior urethral valves than in controls. We studied the role of the bladder in boys who underwent transplantation by simultaneously evaluating renal graft and voiding function.
Between 1972 and 1994, 66 boys with posterior urethral valves underwent kidney transplantation. Of these boys 44 with a mean age of 9.7 years who retained a functional renal graft did not undergo any surgery on the lower urinary tract except for the initial treatment of posterior urethral valves. Long-term evaluation included a voiding questionnaire, radiological assessment and serum creatinine measurement.
Average followup was 9.01 years (range 2.4 to 19.6). There was no voiding dysfunction symptomatology in 23 boys, while 3 (14.2%) and 8 (38.1%) of the remaining 21 had daytime and nighttime frequency, respectively. Dysuria and incontinence were present in 11 (52.4%) and 12 (57.1%) patients, respectively. Urodynamics in 11 cases revealed a mean bladder compliance plus or minus standard deviation of 11.3+/-2.8 ml./cm. water. In boys with a voiding disorder mean serum creatinine increased after 5 years of followup. At 10 years after kidney transplantation mean serum creatinine was 140.3+/-36.0 and 285.7+/-36.2 micromol./l. in asymptomatic boys and those with a voiding disorder, respectively (p<0.01).
Valve bladder has a role in the deterioration of renal transplants in boys with posterior urethral valves. In those with a voiding disorder closer followup is needed, including urodynamic and radiological studies. Bladder dysfunction, such as hypocompliance and/or hyperreflexia, requires medical or surgical treatment.
The Journal of Urology 04/2000; 163(4):1282-5. · 3.75 Impact Factor
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ABSTRACT: The cyclosporine microemulsion formulation Neoral, which allows a better absorption and a more regular pharmacokinetic profile, is proposed for replacing the original formulation, Sandimmun. The present study reports the results of conversion from Sandimmun to Neoral in children with a kidney graft, a population for which information remains limited.
Twenty children, 2.5 to 10.5 years of age, who had a kidney graft with a stable renal function for between six months to five years (m = 2.6) were the subjects of this study. The patients were switched from Sandimmun to Neoral at the same dose, adjusted afterwards on a cyclosporine trough level.
After six months, the mean dose decreased from 9.1 mg/kg/d to 8.4 mg/kg/d, i.e., 12.5%. After one year, the mean dose was 7 mg mg/kg/d, i.e., 28%. Of the 65% of patients who had a decreased dose, most of them had the highest dose of Sandimmun at the start. Mean serum creatininemia levels slightly increased from 85.6 to 89.5 mumol/L after six months (P = 0.03). None of the patients had a rejection crisis during the first six months under Neoral. Blood pressure did not change significantly, hirsutism improved in two cases but increased or appeared in two cases as well. Gingival hypertrophy increased or appeared in four cases.
A decrease in the dose was decided on either to maintain the trough CsA blood level in the desired range or because of the appearance of a symptom suggesting a side effect of cyclosporine, especially the increase of creatinemia. The trough level did not appear to be the best index for adapting the dose.
In stable pediatric kidney transplant recipients, the switch from Sandimmun to Neoral provided a reduction in drug dosage in 65% of cases without an increase in adverse events.
Archives de Pédiatrie 11/1999; 6(10):1066-9. · 0.30 Impact Factor
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ABSTRACT: Diabetes mellitus is a frequent long-term complication of infantile nephropathic cystinosis. We studied 44 cystinotic patients, aged 22.1+/-5.4 years, transplanted at a mean age of 11.3+/-2.5 years; 25% were treated with insulin at 20 years of age or 10 years after transplantation, and over half required insulin at latest follow-up. In comparison, diabetes mellitus occurred in only 1% of non-cystinotic transplanted patients. Sequential oral glucose tolerance tests (OGTTs) in these patients showed the progressive deterioration of glucose metabolism. All but 2 patients had an abnormal response at latest follow-up. The high doses of corticosteroid given after transplantation or during rejection episodes were responsible for transient insulin dependency. However, the development of impaired glucose tolerance and diabetes mellitus depended mainly on the cystinotic process, which developed slowly with time. The deterioration of glucose tolerance was correlated with a decreased early phase of insulin secretion, estimated from the plasma insulin level at 30 min of the OGTT, while there was no evidence of insulin resistance. The occurrence of diabetes mellitus correlated with a worsening of the vital prognosis.
Pediatric Nephrology 09/1999; 13(6):524-9. · 2.52 Impact Factor
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Transplantation Proceedings 10/1998; 30(6):2815. · 1.00 Impact Factor
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ABSTRACT: From 1991 to 1993, 90 children having received a kidney graft with a post-transplantation period of at least 12 months were included in a prospective study carried out in 18 French pediatric centers. After informed consent and randomization, children received recombinant human growth hormone (rhGH) (Genotonorm, Pharmacia peptide hormones) 30 U/m2 per week, either immediately on enrollment, for the treated group, or after 1 year of follow-up for the group serving as a control. After 1 year both groups were treated and we analyzed data during the subsequent years. Eighty-five children completed the 1-year study. Growth velocity was significantly increased by rhGH: 7.7 cm with a gain of +0.3 standard deviation score in the treated group versus 4.6 cm in the control group (P<0.0001) during the 1st year. Four factors predicted response to therapy: growth velocity prior to GH therapy, glomerular filtration rate (GFR) at the start, mode of corticosteroid administration, and degree of insulin resistance. After 1 year we observed a moderate, significant decrease in GFR in both groups. Biopsy-proven acute rejection episodes were not significantly more frequent during the 1st year in the group of patients who received rhGH: 9 in 44 versus 4 in 46 patients. The patients who rejected did not differ in terms of age, renal function at the start, and type of immunosuppression, but history of rejection before GH treatment was discriminatory: 6 of 17 children with two or more episodes had a new rejection versus 1 of 22 who had no or only one episode (P=0.01). Glucose tolerance was not modified after 1 year of GH therapy. During the subsequent years of treatment a decrease in growth velocity was noted: 5.9 cm at 2 years, 5.5 at 3 years, and 5.2 cm at 4 years. In conclusion, GH is efficient for improving growth velocity in short transplanted children, inducing clear-cut but limited catch-up growth. The risk of rejection was shown only in patients with a prior history of more than one rejection episode.
Pediatric Nephrology 09/1998; 12(6):437-46. · 2.52 Impact Factor
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ABSTRACT: From 1991 to 1993, 90 children having received a kidney graft with a post-transplantation period of at least 12 months were
included in a prospective study carried out in 18 French pediatric centers. After informed consent and randomization, children
received recombinant human growth hormone (rhGH) (Genotonorm, Pharmacia peptide hormones) 30U/m2 per week, either immediately on enrollment, for the treated group, or after 1 year of follow-up for the group serving as
a control. After 1 year both groups were treated and we analyzed data during the subsequent years. Eighty-five children completed
the 1-year study. Growth velocity was significantly increased by rhGH: 7.7cm with a gain of +0.3 standard deviation score
in the treated group versus 4.6cm in the control group (P<0.0001) during the 1st year. Four factors predicted response to therapy: growth velocity prior to GH therapy, glomerular
filtration rate (GFR) at the start, mode of corticosteroid administration, and degree of insulin resistance. After 1 year
we observed a moderate, significant decrease in GFR in both groups. Biopsy-proven acute rejection episodes were not significantly
more frequent during the 1st year in the group of patients who received rhGH: 9 in 44 versus 4 in 46 patients. The patients
who rejected did not differ in terms of age, renal function at the start, and type of immunosuppression, but history of rejection
before GH treatment was discriminatory: 6 of 17 children with two or more episodes had a new rejection versus 1 of 22 who
had no or only one episode (P=0.01). Glucose tolerance was not modified after 1 year of GH therapy. During the subsequent years of treatment a decrease
in growth velocity was noted: 5.9cm at 2 years, 5.5at 3 years, and 5.2cm at 4 years. In conclusion, GH is efficient for
improving growth velocity in short transplanted children, inducing clear-cut but limited catch-up growth. The risk of rejection
was shown only in patients with a prior history of more than one rejection episode.
Pediatric Nephrology 01/1998; 12(6):437-446. · 2.52 Impact Factor
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La Presse Médicale 10/1997; 26(27):1303-8. · 0.67 Impact Factor
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Transplantation Proceedings 09/1997; 29(5):2479. · 1.00 Impact Factor
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ABSTRACT: To determine the long-term prevalence of varicella infection and herpes zoster after kidney transplantation and to assess the effectiveness of varicella immunization with the Oka attenuated strain.
This study involved 704 children and adolescents who received a kidney graft in our institution from 1973 to 1994 and had routinely been given varicella vaccine beginning in 1980 in preparation for transplantation.
After vaccination 62% of these patients still had varicella/zoster (VZ) antibodies at 1 year and 42% after 10 years. After transplantation the incidence of varicella was significantly lower, 26/212 (12%), in patients who received immunization than in those who did not and had no history of varicella, 22/49 (45%). The disease was also significantly less severe in the vaccinated patients (three deaths among naive patients vs none among vacciness). In the vaccinees, varicella infection was observed only in those who did not develop or lost VZ antibodies; in addition, 21 patients of this subgroup had an asymptomatic seroconversion. Four of the 415 patients with a history of varicella had another episode of benign varicella after grafting. Herpes zoster was observed in 76 of the 704 patients included in the study. The prevalence differed according to VZ status at the time of grafting: 13% in patients with a history of varicella, 7% in the vacciness, and 38% in the naive patients at grafting who developed varicella. Three rejection episodes occurred in association with a varicella episode and four with a zoster episode, but graft function was only transiently impaired, and as a whole varicella or zoster did not significantly affect graft function or survival.
Naive VZ patients with a kidney graft are at risk to develop severe varicella and this may be effectively prevented by available immunization.
PEDIATRICS 02/1997; 99(1):35-9. · 4.47 Impact Factor
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Archives de Pédiatrie 02/1996; 3 Suppl 1:138s-140s. · 0.30 Impact Factor
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Archives de Pédiatrie 02/1996; 3 Suppl 1:344s-345s. · 0.30 Impact Factor
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Archives de Pédiatrie 02/1996; 3 Suppl 1:135s-137s. · 0.30 Impact Factor
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ABSTRACT: Of the 26 cystinotic patients over 19 years of age followed in our institution, 7 developed CNS complications at a mean age of 23 years. Two forms were observed. The first, associating cerebellar and pyramidal signs, mental deterioration and finally pseudo-bulbar palsy, may be called cystinosis encephalopathy. The other form resembled a stroke-like episode with coma and hemiplegia or milder symptoms. Hydrocephalus was rare and not associated with clinical symptoms in this series. Cysteamine was administered for longer than 6 months to 4 of the patients with encephalopathy. Two had an almost complete disappearance of their symptoms including the gross abnormalities of MR imaging in one; one improved partially and remained stable, and one continued to deteriorate but was suspected of non-compliance. These results suggest that cysteamine may be an effective treatment of cystinosis encephalopathy and encourage prescription of this drug in cystinosis in order to prevent this complication.
Journal of Inherited Metabolic Disease 02/1996; 19(1):65-75. · 3.58 Impact Factor
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Transplantation Proceedings 11/1994; 26(5):2791-7. · 1.00 Impact Factor
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ABSTRACT: Administration of recombinant human growth hormone (rhGH) to children with chronic renal failure (CRF), on conservative treatment or kidney transplanted, may induce acceleration of growth. We report our experience of the first 3 years of treatment in such children.
Eight children with CRF on conservative treatment and six kidney transplanted children were included in a European multicentric trial. All children were given rhGH, 30 Ul/m2 body surface area/week, as daily subcutaneous injections, for 12-36 months.
The mean growth velocity in children with CRF increased from 3.8 +/- 0.4 cm/yr before treatment to 9.0 +/- 0.4 (P < 0.001), 6.5 +/- 0.3 (P < 0.002) and 5.4 +/- 0.5 cm/yr, after 12, 24 and 36 months of treatment, respectively. The height gain after 2 years of treatment was 1.2 SD (P < 0.001) with bone age advancement not greater than the increase in chronological age. There was a significant decrease in the inulin clearance after 1 year of treatment. In transplanted children, the mean height gain was less important, increasing from 3.2 +/- 0.4 cm/yr before treatment to 6.2 +/- 0.6 cm/yr after 12 months of treatment (P < 0.001). There was no significant decrease in the mean inulin clearance, but two patients experienced rejection crisis.
A short-term rhGH treatment may improve growth velocity of CRF or transplanted children. The possible role of GH on decrease in glomerular filtration in CRF and on incidence of acute kidney rejection after transplantation remains to be evaluated in a large cohort of patients.
Archives de Pédiatrie 09/1994; 1(8):716-22. · 0.30 Impact Factor
