6 results on '"Garaix F"'
Search Results
2. Atypical severe early-onset nephrotic syndrome: Questions.
- Author
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Berthaud R, Heidet L, Oualha M, Brat R, Talmud D, Garaix F, Rabant M, Frémeaux-Bacchi V, Antignac C, Boyer O, and Dorval G
- Subjects
- Humans, Kidney, Proteinuria, Nephrotic Syndrome diagnosis
- Published
- 2022
- Full Text
- View/download PDF
3. Dilatation of the aorta in children with advanced chronic kidney disease.
- Author
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Quennelle S, Ovaert C, Cailliez M, Garaix F, Tsimaratos M, and El Louali F
- Subjects
- Aorta, Child, Chronic Disease, Dilatation, Humans, Hypertrophy, Left Ventricular diagnostic imaging, Hypertrophy, Left Ventricular epidemiology, Hypertrophy, Left Ventricular etiology, Renal Dialysis adverse effects, Risk Factors, Hypertension, Renal Insufficiency, Chronic complications, Renal Insufficiency, Chronic epidemiology, Renal Insufficiency, Chronic therapy
- Abstract
Background: The peculiarity of the cardiovascular risk profile with increased arterial vulnerability is well known in adults with chronic kidney disease (CKD). It is explained by an increased incidence of traditional cardiovascular risk factors together with other comorbidities related to the uremic condition and cardiorenal syndrome (CRS). The present study aimed to determine the cardiovascular impact of the uremic condition in a pediatric population with advanced CKD., Methods: From 2016 to 2018, 39 consecutive patients with advanced CKD who underwent echocardiographic evaluation were included. All echocardiographic examinations were performed by the same operator (FE). Demographic, clinical, biological, and echocardiographic data were collected., Results: The mean age at echocardiographic exam was 9.7 ± 4.6 years. Twenty-four (61.5%) patients were on hemodialysis; 17 (43.6%) patients were in a peritoneal dialysis program of whom 11 switched at a later stage to hemodialysis. Eight (20.5%) patients had an arteriovenous fistula (AVF). Hypertension was present in 30 (76.9%) patients while left ventricular hypertrophy (LVH) was described in 13 (33.3%) patients. Dilatation of the ascending aorta (Z-score > 2) was found in 15 (38.4%) patients and was statistically (in univariate analysis) related to gender, hypertension, the presence of an AVF, and the use of hemodialysis after peritoneal dialysis (p = 0.024, p = 0.016, p = 0.006, p = 0.009, respectively)., Conclusion: In addition to classical and predictable abnormalities related to CKD, we found a high prevalence of dilatation of the ascending aorta in children with advanced CKD. Hypertension, AVF, and hemodialysis were associated factors.
- Published
- 2021
- Full Text
- View/download PDF
4. Pharmacogenetics of post-transplant diabetes mellitus in children with renal transplantation treated with tacrolimus.
- Author
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Lancia P, Adam de Beaumais T, Elie V, Garaix F, Fila M, Nobili F, Ranchin B, Testevuide P, Ulinski T, Zhao W, Deschênes G, and Jacqz-Aigrain E
- Subjects
- ATP Binding Cassette Transporter, Subfamily B genetics, Adolescent, Child, Cytochrome P-450 CYP3A genetics, Cytochrome P-450 Enzyme System genetics, Diabetes Mellitus genetics, Female, France, Genetic Predisposition to Disease, Genotype, Humans, Immunosuppressive Agents therapeutic use, Male, PPAR alpha genetics, Pharmacogenetics, Postoperative Complications etiology, Postoperative Complications genetics, Receptors, Calcitriol genetics, Retrospective Studies, Risk Factors, Tacrolimus therapeutic use, Diabetes Mellitus etiology, Immunosuppressive Agents adverse effects, Kidney Transplantation adverse effects, Tacrolimus adverse effects
- Abstract
Background: Post-transplant diabetes mellitus (PTDM) is a major complication of immunosuppressive therapy, with many risk factors reported in adults with renal transplantation. The objective of this study was to investigate potential non-genetic and genetic risk factors of PTDM in children with renal transplantation treated with tacrolimus., Methods: A national database was screened for patients developing PTDM within 4 years following tacrolimus introduction. PTDM was defined as glucose disorder requiring anti-diabetic treatment. PTDM patients were matched to "non-PTDM" control transplanted children according to age, gender, and duration of post-transplant follow-up. Patients were genotyped for six selected genetic variants in POR*28 (rs1057868), PPARa (rs4253728), CYP3A5 (rs776746), VDR (rs2228570 and rs731236), and ABCB1 (rs1045642) genes, implicated in glucose homeostasis and tacrolimus disposition., Results: Among the 98 children with renal transplantation enrolled in this multicentre study, 18 developed PTDM. None of the clinical and biological parameters was significant between PTDM and control patients. Homozygous carriers of POR*28 or wild-type ABCB1 (rs1045642) gene variants were more frequent in PTDM than in control patients with differences close to significance (p = 0.114 and p = 0.066 respectively). A genetic score based on these variants demonstrated that POR*28/*28 and ABCB1 CC or CT genotype carriers were at a significantly higher risk of developing PTDM after renal transplantation., Conclusion: Identification of PTDM risk factors should allow clinicians to allocate the best immunosuppressant for each patient with renal transplantation, and improve care for patients who are at a higher risk.
- Published
- 2018
- Full Text
- View/download PDF
5. Rituximab fails where eculizumab restores renal function in C3nef-related DDD.
- Author
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Rousset-Rouvière C, Cailliez M, Garaix F, Bruno D, Laurent D, and Tsimaratos M
- Subjects
- Antibodies, Monoclonal, Murine-Derived therapeutic use, Child, Complement C3 Nephritic Factor analysis, Complement C3 Nephritic Factor immunology, Glomerulonephritis, Membranoproliferative immunology, Glomerulonephritis, Membranoproliferative pathology, Humans, Male, Rituximab, Antibodies, Monoclonal, Humanized therapeutic use, Glomerulonephritis, Membranoproliferative drug therapy
- Abstract
Background: Dense deposit disease (DDD), a C3 glomerulopathy (C3G), is a rare disease with unfavorable progression towards end-stage kidney disease. The pathogenesis of DDD is due to cytotoxic effects related to acquired or genetic dysregulation of the complement alternative pathway, which is at times accompanied by the production of C3 nephritic factor (C3NeF), an auto-antibody directed against the alternative C3 convertase. Available treatments include plasma exchange, CD20-targeted antibodies, and a terminal complement blockade via the anti-C5 monoclonal antibody eculizumab., Case-Diagnosis/treatment: We report here the case of an 8-year-old child with C3NeF and refractory DDD who presented with a nephritic syndrome. She tested positive for C3NeF activity; C3 was undetectable. Genetic analyses of the alternative complement pathway were normal. Methylprednisolone pulses and mycophenolate mofetil treatment resulted in complete recovery of renal function and a reduction in proteinuria. Corticosteroids were tapered and then withdrawn. Four months after corticosteroid discontinuation, hematuria and proteinuria recurred, and a renal biopsy confirmed an active DDD with a majority of extracapillary crescents. Despite an increase in immunosuppressive drugs, including methylprednisolone pulses and rituximab therapy, the patient suffered acute renal failure within 3 weeks, requiring dialysis. Eculizumab treatment resulted in a quick and impressive response. Hematuria very quickly resolved, kidney function improved, and no further dialysis was required. The patient received bimonthly eculizumab injections of 600 mg, allowing for normalization of renal function and reduction of proteinuria to <0.5 g per day. Since then, she continues to receive eculizumab., Conclusion: Complement regulation pathway-targeted therapy may be a specific and useful treatment for rapidly progressing DDD prior to the development of glomerulosclerosis. Our data provide evidence supporting the pivotal role of complement alternative pathway abnormalities in C3G with DDD.
- Published
- 2014
- Full Text
- View/download PDF
6. Crescentic glomerulonephritis is part of hyperimmunoglobulinemia D syndrome.
- Author
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Cailliez M, Garaix F, Rousset-Rouvière C, Bruno D, Daniel L, Chabrol B, Sarles J, and Tsimaratos M
- Subjects
- Glomerulonephritis physiopathology, Humans, Hypergammaglobulinemia blood, Hypergammaglobulinemia physiopathology, Syndrome, Glomerulonephritis immunology, Hypergammaglobulinemia immunology, Immunoglobulin D blood
- Published
- 2006
- Full Text
- View/download PDF
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