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Biomedical subjects

G Picon

Publications and source records attributed to G Picon.

At least 19 recordsLinked to original sources

Kidney function in cyclosporine-treated paediatric pulmonary transplant recipients.

BACKGROUND: Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients. METHODS: We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula. RESULTS: The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure. CONCLUSIONS: Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

Adolescent↗

Chronic renal failure and portal hypertension--is portosystemic shunt indicated?

We report two girls with histories of recessive polycystic kidney disease. Both were on maintenance hemodialysis. They had undergone surgical distal portocaval shunt because of portal hypertension. Later, bilateral nephrectomy was performed, and they presented with hepatic encephalopathy (HE) and evolution towards irreversible hepatic coma and death. Portosystemic shunt is the treatment of choice of portal hypertension. The kidney plays a pivotal role in ammonia disposal during portosystemic shunt. Thus, we stress the risk of HE after portosystemic shunt followed by bilateral nephrectomy in patients with end-stage renal failure and suggest that combined liver-kidney transplantation should be considered.

Child↗

Crescentic glomerulonephritis in hyper IgD syndrome.

The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. Kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS.

Child↗

[Iterative fractures in type I primary hyperoxaluria. Report of 2 cases].

PURPOSE OF THE STUDY: Type I primary hyperoxaluria is a rare autosomal recessive disease linked to a deficit in an hepatic enzyme. The purpose of this study was to analyze orthopedics problems caused by type I primary hyperoxaluria before and after liver and kidney transplantation. MATERIAL AND METHODS: Two cases of children carrying this type I primary hyperoxaluria followed up after liver kidney transplantation are presented and compared to last publications. RESULTS: Combined transplantation progressively corrected osseous lesions and aspect of the stroma. However it did not provide protection against fractures particularly for femoral neck fractures. DISCUSSION: In type I hyperoxaluria overproduction of calcium oxalate causes its accumulation in the whole organism and particularly in bone. Osseous fragility favors pathological fractures. Only combined liverkidney transplantation can save and cure these children. Frequency of this fracture after transplantation indicates preventive plating at first pain, possibly at the same time as transplantation. Kidney transplant failure puts the patient in a "congealed" clinical state where the bone is very rich in oxalate and where the hemodialysis does not eliminate oxalate salts. CONCLUSION: Type I primary hyperoxaluria is a very rare disease. Fractures are very common even after liver and kidney transplantation and especialy femoral neck fractures. We think that preventive plating must be done at first pain. We do not have any explanation for bony weakness after liver-kidney transplantation.

Child↗

[Drash syndrome].

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

Cadaver↗

[The role of dialysis in the treatment of terminal renal insufficiency in children].

In order to analyze the place of dialysis in a hemodialysis/transplantation program, the duration of each treatment modality, mortality rate and quality of inclusion in the social network were studied. Complications which arose during hemodialysis were evaluated by comparing the 1970's and the 1980's. Sixty children with terminal renal failure, aged 3 to 15 years, were entered in a hemodialysis/transplantation program between May 1971 and December 1988. Patients were followed up until December 1989. Among the 47 (78%) survivors at the end of the follow-up period, 25 had a functioning renal transplant and 22 were undergoing dialysis. Among the 13 deaths, 7 occurred during renal transplantation or immediately after loss of the transplant and 6 occurred under dialysis. Mean duration of treatment, including both dialysis and transplantation, was 7 years 11 months. Mean time spent under dialysis was 4 years 9 months. Time spent with a functioning transplant was 3 years 10 months for the 46 transplant recipients. Mean time spent on the transplant waiting list fell from 3 years 6 months before 1980 to 2 years after 1980. Virtually no cases of renal osteodystrophy, acute arterial hypertension or hepatitis B were seen after 1980 as a result of the use of higher-potency vitamin D derivatives, recent antihypertensive drugs including ACE inhibitors, and the Hevac B vaccine. Similarly, safety and patient comfort during dialysis improved substantially, as well as the quality of rehabilitation. Growth remained a significant problem although improvements can be expected to occur in the near future. Hemodialysis is an indispensable complement to transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Discordant evolution in monozygotic twins with familial nephropathy].

Two monozygotic twin sisters presented a familial nephropathy, discovered at 5 years of age. The clinical course was quite different for each patient: end-stage renal failure occurred at 5.5 years of age for one and at 14 years for the other. This difference raises the hypothesis of the role of environmental factors on the clinical expression of an inherited disease.

Adolescent↗

[Renal transplantation in the child. Medical and surgical aspects (author's transl)].

The authors report the experience of the Children's Hospital of Marseille about ten renal transplantations in nine children. Indications, immunosuppressive treatment, surgical techniques employed are detailed. The results study show two mains complications : rejection and arterial hypertension. A better recipient selection, as in adults, would increase good results.

Acute Kidney Injury↗

[PAH clearance measurement without urine samples in the newborn infant with respiratory distress].

PAH clearance was carried out in 12 newborns, hospitalized in the infantile resuscitation unit for respiratory distress. 6 of these children weighed less than 2.5 kg, 4 had hyaline membrane disease, 6 had either amniotic abnormalities or transitory tachypnea, 2 were surgical patients: one right diaphragmatic hernia, one post-operative respiratory complication after intervention for neonatal occlusion. In 9 cases the newborn was under controled artificial ventilation associated with PEEP at 5 to 7 cm of water. In all of the cases, the hemodynamic, metabolic and blood gas conditions were normal. A control series of 11 newnorn was carried out in a pediatric unit, the clearance was done without urine samples, the rough value of the figures found varied from 5.5 ml per minute to 30 ml per minute in the respiratory distress series and 16 to 62 ml per minute in the control series. The analysis of these results in rendered difficult by the juxtaposition of several factors: Choice of a reference criterion: body surface area, PAH space, patient's weight theoretical weight of the kidneys. The factor of prematurity. The problem of the date of the investigation in comparison with the date of birth.

Aminohippuric Acids↗