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Biomedical subjects

P Cochat

Publications and source records attributed to P Cochat.

At least 37 records · Page 2Linked to original sources

[Surgical aspects of kidney transplantation in children (study of 148 cases)].

OBJECTIVE: Kidney transplantation is the best treatment for end-stage renal disease. This procedure is by now routinely used and permits children survival and rehabilitation. We describe the surgical complications of kidney graft in our paediatric experience. MATERIALS AND METHODS: From April 1987 to August 2000, 175 kidney transplantation were proceeded in the department of urology and transplantation in Edouard Herriot Hospital (Lyon). Twenty-nine kidneys (17%) were from living related donors and 146 (83%) were from cadaveric donors. We evaluated 148 kidney transplantation (132 patients) in 79 male and 53 female children. Mean age was ten years (range: seven months to 18 years). Twelve allografts were from living related donors and 136 cadaveric donors. RESULTS: Twelve patients died of various medical complications. One patient was lost of sight. Surgical complications were postoperative bleeding, lymphocele, urologic and digestive troubles. The rest of our patients had uneventful evolution. CONCLUSION: A general commitment is the only way to promote organ donation and enable patients with terminal renal failure to have access to renal transplantation.

Adolescent↗

Lupus nephritis in a child with AIDS.

Concomitant acquired immunodeficiency syndrome (AIDS) and lupus nephritis is an exceptional feature in white patients. A white boy with maternofetal human immunodeficiency virus (HIV) infection had no medical follow-up until he presented at 12 years of age with a nephrotic syndrome, macrohematuria, renal failure, pancytopenia, and low CD4(+) cell count. A renal biopsy revealed severe lupus nephritis (World Health Organization class IV) with specific immune deposits in the absence of any clinical sign of systemic lupus erythematosus or specific autoantibodies at the time of diagnosis. The treatment consisted of methylprednisolone pulses followed by oral prednisone; antiretroviral triple therapy was started a few weeks later, which contributed to clinical and biologic improvement. To our knowledge, this is the first case report of lupus-like nephritis in a white child with AIDS, whose outcome might be improved significantly by a combination of steroids and antiretroviral therapy.

AIDS-Associated Nephropathy↗

Fading renal hyperfiltration in children following liver transplantation.

In a prospective longitudinal study, we investigated the renal function (RF) of 23 children before and after orthotopic liver transplantation (OLT). The aim was to assess both the outcome of pretransplant hyperfiltration and the clinical nephrotoxic effects of cyclosporin A (CsA); children with decreased RF prior to OLT were therefore excluded. The RF study of the 13 remaining patients included glomerular filtration rate (GFR) and effective renal plasma flow (RPF) measured by inulin (Cin: mL/min/1.73 m2) and para-amino hippurate (Cpah: mL/min/1.73 m2) clearances, respectively. Hyperfiltration prior to OLT was observed in six children, i.e. Cin>170 [range 172-230] and Cpah>800 [808-1,133]. A significant decrease in RF was noted as soon as 6 months after OLT: Cin (mean+/-SD)=107+/-23 vs. 158+/-46 (p<0.003); Cpah=583+/-119 vs. 791+/-243 (p<0.004). This was due to loss of hyperfiltration in the six children, as there was no significant difference in RF before and 6 months after OLT in the other seven children. With a 36-month follow-up, there was no correlation between CsA trough blood level and RF. In conclusion, following OLT, RF underwent early changes owing to loss of prior hyperfiltration in children without impaired RF before OLT. In addition, no evidence of CsA nephrotoxicity was found and RF remained stable during follow-up.

Acute Kidney Injury↗

Body composition in children after renal transplantation.

This study examines changes in body composition after renal transplantation (Tx) in 16 children who underwent dual-energy X-ray absorptiometry examination within 6 months preceding Tx; the examination was then repeated every 6 to 12 months after Tx. Body composition was analyzed with regard to whole-body bone mineral content (BMC), lean body mass (LBM), and fat body mass (FBM). Median BMC decreased from the initial value of +0.98 standard deviation scores (SDS) to -0.55 SDS during the first 3 months after Tx, and a further decrease was noted at the end of month 6 (M6; -1.34 SDS) and M12 after Tx (-1.32 SDS). Improvement was observed during the second year after Tx (P for global changes < 0.0001). LBM and FBM also changed significantly during the 2 years after Tx (P = 0.006 and P = 0.0001, respectively). LBM decreased during the first 3 months after Tx (median change, 0.71 SDS) and remained less than 0 SDS in all but 4 patients. Median LBM did not decrease to less than -1 SDS during the entire study period. Conversely, median FBM increased by a median of 3.73 SDS during the first 3 months and remained elevated during the first 12 months after Tx, with a subsequent decrease at 2 years after Tx. No significant correlation was found between cumulative doses of prednisone and BMC, FBM, or LBM at any interval if absolute values were considered. However, relative changes in FBM correlated significantly with relative changes in prednisone cumulative doses.

Absorptiometry, Photon↗

Skin diseases in children with organ transplants.

BACKGROUND: Skin diseases are frequent in organ transplant recipients, but studies concerning children are sparse. OBJECTIVE: We assessed skin diseases in children who had received organ transplants. METHODS: A total of 145 children referred to our dermatologic consultation were studied. RESULTS: Steroid-induced striae distensae and acne occurred only in adolescents; severe cyclosporine-related side effects were more frequent in younger children. The most common findings were warts (53.8%), tinea versicolor (14.5%), herpes simplex/zoster (9.6%), molluscum contagiosum (6.9%), and impetigo contagiosum and folliculitis (6.2%). Other notable disorders included a diffuse hyperpigmentation with a "dirty" appearance of the skin, pyogenic granulomas, melanocytic nevi proliferation, and skin tags. Two of 20 further adult patients who received transplants during childhood had squamous cell carcinomas. CONCLUSION: Children who have received organ transplants frequently present side effects of immunosuppressive drugs and infectious diseases. Most disorders are related to the age of the patients rather than to the length of immunosuppression, whereas others are favored by the reinforcement of immunosuppression. Skin cancers were not encountered, but the risk of carcinomas in early adulthood should be considered.

Adolescent↗

Cytomegalovirus infection may cause ureteral necrosis.

Cytomegalovirus (CMV) infection has protean presentation among immunocompromised patients, but the urinary tract is rarely involved. We report a case of extensive ureteral necrosis in a renal transplant, 12-year-old patient with typical histological feature of CMV inclusions. The role of CMV was confirmed by immunohistochemical analysis and concomitant CMV DNA detection in peripheral blood leukocytes by polymerase chain reaction analysis. CMV infection can, therefore, be regarded as a possible cause of ureteral necrosis in renal transplant recipients.

Child↗

Partial deletion of the AGXT gene (EX1_EX7del): A new genotype in hyperoxaluria type 1.

Primary hyperoxaluria type 1 (PH1) is a rare autosomal (2q37.3) recessive metabolic disease caused by a deficiency of the hepatic peroxisomal enzyme alanine:glyoxylate amino transferase. Molecular heterogeneity is important in PH1 as most of the patients (if the parents are unrelated) are compound heterozygotes for rare mutations. We describe the first large deletion in the AGXT gene, removing exons 1 to 7 (EX1_EX7del) that was responsible for one case of severe PH1. This 10 kb deletion was identified by Southern blotting of genomic DNA digested by Xba I and hybridized with different exonic probes. Both parents (from Turkey) are first cousin and carry the deletion. It is of note that the presently reported patient did not exhibit any AGT catalytic activity and even so, he progressed towards end-stage renal disease only at 19 years old.

Adult↗

Identification of 5 novel mutations in the AGXT gene.

In order to identify additional genotypes in primary hyperoxaluria type 1, we sequenced the AGXT genes of 9 patients. We report 5 new mutations. Three are splice-site mutations situated at the end of intron 4 and 8 (647-1G>A, 969-1G>C, 969-3C>G), one is a missense mutation in exon 5 (D183N), and one is a short duplication in exon 2 (349ins7). Their consequence is always a lack of enzymatic activity of the Alanine-Glyoxylate Aminotransferase (AGT); for 4 of them, we were able to deduce that they were associated to the absence of AGT protein. These mutations are rare, as they have been found on one allele in our study (except 969-3C>G present in 2 unrelated families), and have not been previously reported.

Adult↗

Bone mineral density in children after renal transplantation.

A successful kidney transplantation (Tx) offers good quality of life for children suffering from chronic renal failure. However, some metabolic abnormalities may not be corrected and may persist after Tx despite good graft function. Post-Tx bone disease seems to be a universal finding in adult Tx recipients, and is most probably related to steroids. Reports on bone mineral density (BMD) in children after renal Tx are not uniform. Recent studies suggest that BMD is normal when corrected for height. However, longitudinal studies show a transient decrease in BMD in the early post-Tx period. These controversial results raise the issue of the correct interpretation of dual-energy X-ray absorptiometry in children with stunted growth. Etiopathogenetic factors of the decreased BMD, preventive and therapeutic measures are discussed. In conclusion, the results of dual energy X-ray absorptiometry should be interpreted with caution, especially in children with disturbed growth.

Adult↗

Membranous nephropathy and orbital malignant tumor.

A 7-year-old girl with membranous nephropathy is reported who suffered 16 months later from an orbital rhabdomyosarcoma. Proteinuria disappeared at the time of the remission of the tumor. Membranous nephropathy as paraneoplastic syndrome is exceptional in children, since only two other cases associated with a solid tumor have been reported in the literature.

Child↗

Evolution of secondary hyperparathyroidism after renal transplantation.

Renal osteodystrophy is an important problem in children with chronic renal failure, leading to skeletal deformities. The most-frequent type of renal osteodystrophy is secondary hyperparathyroidism, and the main factors contributing to the pathogenesis of this condition are completely or partially corrected after successful renal transplantation. The present paper reviews data on the evolution of secondary hyperparathyroidism after transplantation. Studies in both adults and children suggest that secondary hyperparathyroidism and increased bone remodelling activity may persist months after transplantation. The severity of secondary hyperparathyroidism prior to transplantation, the duration of dialysis, and the development of nodular and/or monoclonal hyperplasia of parathyroid glands are the most-important factors that determine the phenomenon. Important issues, which still need to be answered, are the possible roles of growth factors, cytokines, VDR gene polymorphism (B/b allele), and type of immunosuppressive regimen in the skeletal abnormalities observed.

Adult↗