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Byline: J. P. Benamenyo (1), D. Droz (2), P. Niaudet (1) Keywords: Keywords Kidney transplantation; Routine biopsy; Pathology; Immunosuppression Abstract: In our institution, systematic renal graft biopsies are performed 1 year after transplantation before deciding to switch to alternate-day steroid therapy, which has been shown to be beneficial for statural growth. We analyzed the results of systematic graft biopsies in 145 children with a creatinine clearance aY=45 ml/min per 1.73 m.sup.2. Biopsies were classified according to Banff diagnostic categories. Normal parenchyma was observed in 19 cases (13%), non-specific lesions in 42 cases (29%), chronic allograft nephropathy grade 1--3 in 68 cases (49%), and acute rejection in 8 cases (5%). Clinicopathological correlations indicated that patients with chronic allograft nephropathy had received kidneys from older donors, with longer cold ischemia time and with a higher incidence of delayed graft function. There was a strong correlation between the donor age and the presence of vascular lesions. There was also a good correlation between the severity of histological lesions and the occurrence of acute rejection episodes during the 1st year after transplantation. Renal function remained stable for up to 10 years in patients with normal parenchyma or non-specific lesions, while serum creatinine levels increased after the 2nd year in patients with chronic allograft nephropathy. Author Affiliation: (1) Service de Nephrologie Pediatrique, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France. Niaudet@necker.fr, FR (2) Department of Renal Pathology, Necker-Enfants Malades Hospital, Paris, France, FR Article note: Received: 16 July 2001 / Revised: 27 August 2001 / Accepted: 28 August 2001

Byline: M. F. Gagnadoux (1), F. Lacaille (2), P. Niaudet (1), Y. Revillon (3), P. Jouvet (4), D. Jan (3), G. Guest (1), M. Charbit (1), M. Broyer (1) Keywords: Keywords Oxalosis; Renal transplant; Hepatorenal transplant 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 &lt 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 (aY=3 l/m.sup.2 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 m.sup.2 (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. Author Affiliation: (1) Department of Pediatric Nephrology, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France. marie-france.gagnadoux@nck.ap-hop-paris.fr, FR (2) Department of Hepatology, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France, FR (3) Department of Surgery, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France, FR (4) Department of Intensive Care, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France, FR Article note: Received: 7 June 2001 / Revised: 7 August 2001 / Accepted: 7 August 2001

Byline: R. Salomon (1), M. Charbit (1), M. F. Gagnadoux (1), P. Niaudet (1), P. Gao (2), T. Cantor (2), J. -C. Souberbielle (3) Keywords: Keywords Parathyroid hormone; Immunoassay; Haemodialysis; Renal osteodystrophy; Ionized calcium Abstract: The measurement of serum intact parathyroid hormone (PTH) is routinely made in haemodialysed (HD) patients to diagnose and monitor secondary hyperparathyroidism. We measured pre- and post-dialysis serum ionized calcium (Ca.sup.2+) and PTH in 12 HD children (7 boys) aged 13.8+-3.6 years. A group of 27 normal short-statured children served as controls. Serum PTH was assessed by a new assay (CAP) recognizing only the (1--84) molecule and an older one (Allegro) recognizing both the 1--84 and a non-(1--84) PTH equally. The concentrations obtained with the CAP assay were lower than those obtained with the Allegro assay both in controls and in HD patients. They were still lower in HD patients when expressed as multiples of the median of the control group. The Allegro/CAP ratio, was highly variable from one subject to another and was lower (P&lt 0.0001) in controls (1.46+-0.26) than in HD patients, both before (3.06+-1.60) and after dialysis (2.94+-0.65). During dialysis, Ca.sup.2+ increased significantly (P&lt 0.0001) and PTH decreased significantly (P&lt 0.0001) with both the CAP and the Allegro assays, but was more often normal or low with the CAP than with the Allegro assay. Although the two assays correlate well, they may provide different clinical information in some HD children which could lead to different therapeutic decisions. Author Affiliation: (1) Service de Nephrologie Pediatrique, Hopital Necker-Enfants Malades, Assistance Publique-Hopitaux de Paris (AP-HP) 149 rue de Sevres, 75743 Paris Cedex 15, France, FR (2) Scantibodies Laboratory, Inc., Santee, California, USA, US (3) Laboratoire d'Explorations Fonctionnelles, Hopital Necker-Enfants Malades (AP-HP), 149 rue de Sevres, 75743 Paris Cedex 15, France, FR Article note: Received: 13 March 2001 / Revised: 12 July 2001 / Accepted: 12 July 2001

Byline: J.-J. Robert (1), M.-J. Tete (2), G. Guest (2), M.-F. Gagnadoux (2), P. Niaudet (2), M. Broyer (2) Keywords: Key wordsaInfantile cystinosis; Renal transplantation; Diabetes mellitus; Impaired glucose tolerance 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. Author Affiliation: (1) Service de Diabete de l'Enfant et de l'Adolescent, Federation de Pediatrie Medicale, Hopital Necker -- Enfants Malades, 149, rue de Sevres, F-75015 Paris, France Tel./Fax: +33-1-44494833, FR (2) Service de Nephrologie Pediatrique, Federation de Pediatrie Medicale, Hopital Necker -- Enfants Malades, Paris, France, FR Article note: Received: 28 July 1998 / Revised: 22 September 1998 / Accepted: 23 September 1998

Byline: P. Niaudet (1) Author Affiliation: (1) Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France, FR

Byline: P. Niaudet (1), Olivier Reigneau (2), Henri Humbert (2) Keywords: KeywordsaNephrotic syndrome; Pharmacokinetic; Cyclosporine Abstract: Seven children with steroid-dependent nephrotic syndrome who were on stable remission under Sandimmun therapy were switched to Neoral at the same dosage. During the 4-month follow-up period, two patients relapsed, due to poor compliance in one of them. Serum creatinine remained stable in all patients. Pharmacokinetic profiles were performed at day 0 while on Sandimmun and 4 weeks after conversion to Neoral. Following conversion to Neoral, the peak concentration occurred earlier (2+-1.4 h vs 3.9+-2.4 h), and the maximum concentration (677+-386 ng/ml vs 488+-265 ng/ml) and the area under the curve (3082+-1536 ng/ml/h vs 2201+- 889 ng/ml/h) were higher. We conclude that Neoral results in an increased bioavailability of cyclosporine (CsA) as compared to Sandimmun in patients with steroid-dependent nephrotic syndrome in remission. Author Affiliation: (1) Service de Nephrologie Pediatrique, Hopital Necker-Enfants Malades, 149, rue de Sevres, 75743 Paris, Ceclex 15, France e-mail: niaudet@necker.fr Tel.: +33-1-4449-4463, Fax: +33-1-4449-4369, FR (2) Novartis France, Rueil Malmaison, France, FR Article note: Received: 28 April 2000 / Revised: 5 October 2000 / Accepted: 5 October 2000