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Biomedical subjects

M F Gagnadoux

Publications and source records attributed to M F Gagnadoux.

At least 37 records · Page 2Linked to original sources
PubMed1998

Renal transplantation in children with augmentation cystoplasty: long-term results.

PURPOSE: We assessed the long-term results of renal transplantation in children with augmentation cystoplasty. MATERIALS AND METHODS: We retrospectively reviewed the complications and followup in 14 pediatric renal transplant recipients with augmentation cystoplasty. The etiology of bladder dysfunction included posterior urethral valves in 10 cases, neurogenic bladder in 3 and vesicoureteral reflux in 1. All transplants were cadaver donor kidneys. Mean patient age at transplantation was 12.1 years (range 5 to 18). Augmentation cystoplasty was performed before and after transplantation in 10 and 4 cases, respectively. Detubularized ileum was used in 5 cases, tubular ileum in 4, tubular sigmoid in 4 and stomach in 1. RESULTS: Of the 14 transplanted kidneys 10 (71%) were functioning at a mean followup of 80 months (range 12 to 151). Serum creatinine was less than 1.4 mg./dl. in 9 patients. Four grafts were lost to chronic rejection. The 5 and 10-year graft survival rates were 84 and 73%, respectively. Two patients with a functioning kidney died of causes unrelated to augmentation cystoplasty. Complications included symptomatic urinary infections in 4 patients, hyperchloremic metabolic acidosis in 2, nephrolithiasis in the allograft in 2 and the hematuria-dysuria syndrome in 1. All patients were continent. CONCLUSIONS: Augmentation cystoplasty is a safe and effective method of restoring lower urinary tract function in the pediatric renal transplant population with a small noncompliant bladder.

Adolescent↗
PubMed1998

Crystalluria: a clinically useful investigation in children with primary hyperoxaluria post-transplantation.

Primary hyperoxaluria type I (PH I) is a congenital error of metabolism that can be manifested by an increased oxalate production, and ultimately result in kidney failure. After a combined liver/kidney transplantation, children with PH I have persistent excretion of oxalate that causes crystal formation in the urinary tract, and could result in systemic oxalosis and eventual graft failure. We speculated that crystalluria may be predictive of this nephrolithogenic tendency and thus investigated the effect of an intensive therapeutic strategy to prevent crystal formation in 13 children at our hospital. Oxalate crystal volume (OCV) measurements were performed at regular intervals for 36 months, and compared with urine supersaturation measurements. We found that crystalluria with the OCV measurement is non-invasive, easily performed, and gives feedback on the efficacy of PH I therapy within one hour. Further study is needed to determine whether this method is a better predictor of nephrocalcinosis than is supersaturation alone.

Adolescent↗
PubMed1997

[Cystic kidney diseases].

Among all inherited cystic kidney diseases, the commonest are polycystic kidney diseases, which include 2 diseases characterized by their pathological characteristics and their mode of inheritance, namely autosomal dominant or recessive. Autosomal dominant polycystic kidney disease is usually diagnosed in adulthood and is related at least to 2 different genes; PKD1 gene on chromosome 16 accounts for 85% of cases. This frequent disease (1 in 1,000 people) leads to end-stage renal failure in most patients at a mean age of 55 years. Renal ultrasonography allows its detection at an early stage, during childhood or adolescence, and even in utero in some cases. Autosomal recessive polycystic kidney disease, related to a single gene mapped to chromosome 6, is a rare disease, usually diagnosed during infancy because of enlarged kidneys and hypertension. The early occurrence of advanced renal failure is uncommon and only 1/3 of patients require renal replacement therapy during childhood. The term "polycystic kidney disease" should be limited to these 2 diseases; however there are many other inherited conditions including renal cysts like tuberous sclerosis or Hippel-Lindau's disease in adults, and several malformative syndromes in children.

Humans↗
PubMed1997

A controlled study of deflazacort in the treatment of idiopathic nephrotic syndrome.

Forty patients with steroid-dependent idiopathic nephrotic syndrome (INS), a mean follow-up of 5.5 years, and a mean number of relapses of ten were blindly assigned to either deflazacort (DFZ) (n = 20) or prednisone (PDN) (n = 20) according to a ratio of equivalence of DFZ/ PDN = 0.8. This treatment was given for 1 year. The number of relapses was significantly lower in patients receiving DFZ. After 1 year, 12 remained in remission with DFZ compared with 2 with PDN. Growth velocity was not different in the two groups. Bone mineral content, assessed by quantitative computed tomography of L1 L2 vertebrae, decreased after 1 year by 6% in the DFZ group versus 12% in the PDN group (NS). The mean body weight increase of +3.9 +/- 4.1 kg in the PDN group was higher than that of the DFZ group, +1.7 +/- 2.8 kg (P = 0.06). Cushingoid symptoms tended to be less after 12 months in the DFZ group. In conclusion, this study shows that DFZ was more effective than PDN in limiting relapses in steroid-dependent INS, and that cushingoid symptoms, weight gain, and decrease in bone mineral content tended to be less marked with this drug than with PDN.

Adolescent↗
PubMed1997

Posterior urethral valves: long-term renal function consequences after transplantation.

PURPOSE: We assessed the long-term efficacy of renal transplantation in children with posterior urethral valves. MATERIALS AND METHODS: We retrospectively compared the outcomes of renal transplantation in 66 children with posterior urethral valves and 116 with malformation uropathies (controls). RESULTS: Graft survival in the posterior urethral valves and control groups was 69 and 72% at 5 years, and 54 and 50% at 10 years, respectively (not statistically significant). A statistically significant increase in serum creatinine was noted at 10 years in children with posterior urethral valves but not in controls (p < 0.05). CONCLUSIONS: Renal transplantation in children with posterior urethral valves is not associated with a high rate of failure. However, long-term deterioration of graft function is likely related to lower urinary tract dysfunction.

Child↗
PubMed1997

Long-term results of renal transplantation in children with the prune-belly syndrome.

PURPOSE: We assessed the long-term efficacy of renal transplantation in children with the prune-belly syndrome. MATERIALS AND METHODS: We retrospectively compared the outcomes of renal transplantation in 9 children with the prune-belly syndrome and 100 with malformative uropathy. RESULTS: Graft survival in the prune-belly syndrome and control groups was 50 and 72% at 5 years, and 50 and 47% at 10 years, respectively (not statistically significant). No statistically significant increase in serum creatinine was noted at 10 years in children with the prune-belly syndrome. Two patients with the prune-belly syndrome underwent internal urethrotomy after transplantation. All patients voided well and did not require intermittent catheterization. CONCLUSIONS: Renal transplantation in children with the prune-belly syndrome is not associated with a high rate of failure. However, these patients must be followed with regular urological evaluation since voiding efficiency may deteriorate.

Child↗
PubMed1997

Varicella and zoster in children after kidney transplantation: long-term results of vaccination.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: Naive VZ patients with a kidney graft are at risk to develop severe varicella and this may be effectively prevented by available immunization.

Adrenal Cortex Hormones↗
PubMed1997

Cyclosporine in the therapy of steroid-resistant idiopathic nephrotic syndrome.

Steroid-resistant nephrotic syndrome with either minimal changes or focal and segmental glomerular sclerosis on initial biopsy is a severe condition as more than 50% of patients with the disease progress to end-stage renal failure within 10 years. We recently identified a distinct form of idiopathic nephrotic syndrome with an autosomal recessive mode of inheritance and were able to map the gene on the long arm of chromosome 1. The absence of recurrence of the disease after renal transplantation suggest a primary defect in a glomerular basement membrane protein. Several reports suggest that cyclosporine is effective in a proportion of patients with steroid-resistant idiopathic nephrotic syndrome, particularly when used in combination with corticosteroids. There are also data suggesting that high doses of cyclosporine may be necessary in patients with severe hypercholesterolemia. However, cyclosporine treatment should be carefully monitored in view of the high risk of nephrotoxicity, as shown by the results of repeat renal biopsies. The beneficial role of cyclosporine in recurrent steroid-resistant nephrotic syndrome is still debated. Preliminary observations suggest that the early use of intravenous cyclosporine may be effective in these patients.

Cyclosporine↗
PubMed1996

Membranous nephropathy following perinatal transmission of hepatitis B virus infection--long-term follow-up study.

In children with hepatitis B-associated membranous glomerulonephritis, the time of onset of the infection and the duration of the carrier state before diagnosis of the renal disease are always unknown. Moreover, follow-up is usually short. We report the unique observation of a French girl who was infected with hepatitis B virus by her mother who had acute hepatitis during the immediate postpartum period; the girl developed proteinuria at 6 years of age. The onset of the infection in the perinatal period, the mild liver abnormalities, and the absence of nephrotic syndrome did not justify any treatment. Spontaneous seroconversion to anti-HBe antibody positive occurred at 12 years of age. Proteinuria gradually diminished and was absent at 18 years. However, HBs antigenemia persists.

Adult↗
PubMed1996

Long-term (15-25 years) outcome of childhood hemolytic-uremic syndrome.

The short-term prognosis of the "typical", "post-enteropathic" form of infantile HUS is usually good, with a complete recovery of renal function. However, the extent of the renal damage observed on some biopsies may raise concern for the long-term prognosis. Therefore, we studied the outcome of 29 patients affected with classical HUS in infancy or early childhood and followed-up for 15-28 years (m = 18 yrs). Initial renal symptoms ranged from a moderate renal failure with normal diuresis to a 12-day anuria: 21 children had to be treated by peritoneal dialysis. Twenty-five patients underwent a renal biopsy shortly after recovery: lesions of glomerular thrombotic microangiopathy (TMA) were found in 14 patients, and patchy cortical necrosis was diagnosed in the other 11. At latest examination 10 patients had no renal abnormality, 12 had residual renal symptoms (hypertension in 7, proteinuria in 4 and midly reduced GFR in 1), 3 were in chronic renal failure (CRF), and 4 had reached end-stage renal failure (ESRF) 16-24 years after onset; 2 of these latter 4 had a normal GFR at 10-year examination. The long-term evolution was not correlated with the initial clinical severity but appeared well correlated with the extent of the histological damage: 10 of the 11 patients with cortical necrosis have either ESRF (4), CRF (3) or renal sequelae (3), and 4 of the 5 patients with TMA involving more than 50% of glomeruli present with moderate sequelae, whereas the 9 patients with TMA involving less than 50% of glomeruli are symptom-free or have mild sequelae. Thus, the risk of renal failure 20 years after a seemingly cured childhood HUS is not negligible, and renal histology is the best indicator of long-term prognosis.

Age of Onset↗