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B Ranchin

Publications and source records attributed to B Ranchin.

15 recordsLinked to original sources
PubMed2006

Abdominal manifestations in childhood-onset systemic lupus erythematosus.

BACKGROUND: Childhood-onset lupus erythematosus is a rare disorder of unknown origin. OBJECTIVES: To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow-up, and discuss the specific causes of these manifestations. METHODS: Medical records of 201 patients with childhood-onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations. RESULTS: Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5-16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment-induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were <40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients. CONCLUSION: Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded.

Abdomen, Acute↗
PubMed2006

Rituximab therapy for childhood-onset systemic lupus erythematosus.

OBJECTIVE: To describe the safety and efficacy of rituximab in the treatment of childhood-onset systemic lupus erythematosus (SLE). STUDY DESIGN: We conducted a French multicenter retrospective study of childhood-onset SLE treated with rituximab. RESULTS: Eleven girls with severe SLE, including 8 girls with class IV or V lupus nephritis, 2 girls with severe autoimmune cytopenia, and 1 girl with antiprothrombin antibody with severe hemorrhage, were treated with rituximab. The mean age at onset of rituximab treatment was 13.9 years. Patients received 2 to 12 intravenous infusions of rituximab (350-450 mg/m2/infusion), with corticosteroids. Six patients also received different standard immunosuppressive agents, including Cyclophosphamide (2 patients). Remission was achieved in 6 of 8 patients with lupus nephritis and in the 2 patients with autoimmune cytopenia. Steroid therapy was tapered in 5 patients who responded to treatment, and low-dose prednisone treatment was maintained in 1 patient. The mean follow-up period was 13.2 months (range, 6-26 months), and remission lasted in all who patients who responded to treatment, except 1 patient who was successfully retreated with a second course of rituximab. Anti-double-stranded DNA antibody levels decreased in 6 of 11 patients, and anticardiolipin antibody levels decreased in 3 of 4 patients. Severe adverse events developed in 5 patients. Effective depletion of peripheral blood B cells was observed in 7 of 8 patients who were examined, and this paralleled the remission. CONCLUSION: Rituximab may be an effective co-therapy; however, further investigations are required because severe adverse events occurred in 45% of the patients in this study.

Adolescent↗
PubMed2004

[Disseminated lupus erythematosus in children: guidelines about investigations during the initial evaluation and follow-up].

Childhood-onset systemic lupus erythematosus (SLE) is often severe and has a serious long-term morbidity. Pediatric guidelines about its management do not exist. The French study group of childhood-onset SLE proposes recommendations about the investigation which are needed at diagnosis and during follow-up of SLE, in order to adjust the treatment according to the severity of the disease and to avoid unnecessary investigations.

Aftercare↗
PubMed2003

Acute renal failure in a patient with phosphofructokinase deficiency.

A 16-year-old Caucasian girl was admitted to hospital with acute renal failure and hemolytic anemia due to rhabdomyolysis following a 3-km walk. (31)P-magnetic resonance spectroscopy provided characteristic spectra of type VII glycogen storage disease (phosphofructokinase deficiency).

Acute Kidney Injury↗
PubMed2002

[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↗
PubMed2002

[Neurologic signs revealing a Behçet's disease: two pediatric case reports].

UNLABELLED: Behçet's disease can be revealed by neurologic signs. CASE REPORTS: We report two pediatric cases of Behçet's disease which initially presented with cerebral venous thrombosis. Glucocorticoïds, associated with anticoagulant treatment allowed a rapid recovery. One of the children presented three years later a thrombotic recurrence. CONCLUSION: A cerebral venous thrombosis may reveal Behçet's disease.

Adolescent↗
PubMed2000

Vesicoureteral reflux after kidney transplantation in children.

BACKGROUND: The prevalence and significance of vesicoureteral reflux (VUR) after kidney transplantation in adults varies between authors and there have been few reports in children. METHODS: We conducted a retrospective study in a single-centre paediatric cohort. Fifty-five of the 84 children who underwent kidney transplantation over a 5-year period were checked with routine cystography after a median of 8 months post-transplantation. Graft function and urinary-tract infections were assessed during the first 6 years after transplantation. RESULTS: VUR into the graft was present in 58% of the patients. Graft function and incidence of urinary-tract infections were similar in the two groups, independent of VUR. After having excluded infections attributed to the presence of a catheter, actuarial survival rates without pyelonephritis and without pyelonephritis following a first lower urinary-tract infection were worse in patients with VUR (P:=0.017 and P:=0.0039 respectively). None of the eight patients with VUR treated with antibiotic prophylaxis after a first acute pyelonephritis (APN) episode presented subsequent APN after 4.4+/-3.3 years on therapy. CONCLUSIONS: VUR to the graft occurred in more than half paediatric renal transplant recipients. This condition was associated with an increased risk of APN. Long-term antibiotic prophylaxis seems to be able to prevent APN in transplanted children with VUR.

Adolescent↗
PubMed1996

Management of recurrent nephrotic syndrome after kidney transplantation in children.

Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosis and its recurrence after transplantation (Tx) are mainly seen in children. The average recurrence rate is 30% and the graft loss is half this; the risk of recurrent NS in subsequent Tx is 50 to 80% according to the fate of the primary allograft. The immediate appearance of proteinuria after Tx suggests that circulating factor(s) might be present which alter the glomerular permeability. Several therapeutic schedules have been proposed and give conflicting results. However, from the current literature, a 3-step management should reasonably be settled: 1) preventive measures in patients at risk include bilateral nephrectomy prior to Tx and introduction of intravenous cyclosporine A (target CyA whole blood level 200 to 250 ng/ml) as early as possible in association with prednisone and azathioprine (+/-anti-thymocyte globulin), 2) in recurrent patients who were not under such a CyA preventive regime, high dose intravenous CyA should be started as soon as possible (target CyA whole blood level 250-350 ng/ml), 3) in children who fail to respond to the above therapeutic proposals, a combination of plasmapheresis followed by substitutive immunoglobulins in association with methylprednisolone pulses and cyclophosphamide instead of azathioprine for 2 months should be proposed early.

Child↗
PubMed1996

[Value of cyclosporine in the treatment of the recurrence of nephrosis after renal transplantation].

From May 1992 to December 1994, 14 children with end stage renal failure secondary to steroid-resistant idiopathic nephrotic syndrome received a cadaver kidney graft. Immediate recurrence of the nephrotic syndrome occurred in four patients. In the four patients, a complete remission was observed shortly after the start of intravenous cyclosporin; two children received in addition methylprednisolone pulses for secondary rejection. Two children were still protein-free 9 and 15 months after transplantation. In the third patient, proteinuria relapsed after 9 months of complete remission and persisted in spite of the reintroduction of intravenous cyclosporin. The fourth graft was lost at 3.5 month from irreversible rejection. The creatinine clearance of the 3 functioning grafts was respectively: 73, 76 and 92 ml/mn/1.73 m2 16, 10 and 15 months after transplantation. Intravenous cyclosporin started shortly after the recurrence, maintaining blood levels between 200 and 300 ng/ml, may induce a remission in recurrent nephrotic syndrome after renal transplantation in childhood.

Child, Preschool↗
PubMed1994

[Exophthalmos in children caused by vascular orbital neoformation].

BACKGROUND--Exophthalmos or proptosis may be a result of several causes; when unilateral, it leads to search a variety of orbital tumors. CASE 1--A 2 month-old baby presented with a right exophthalmos which developed within a few days. Computed tomography showed infiltration of the orbit by a mass. Surgical biopsy showed capillary hemangioma. The patient was given prednisone 2 mg/kg/day for 2 months; exophthalmos disappeared within 10 days and the child was normal 7 years later. CASE 2--A 13 year-old boy was examined because he had developed left exophthalmos within 2-3 weeks. Ultrasonography and computed tomography showed an orbital tumor compressing the eye and the optic nerve. This tumor was excised and histological examination showed that it was a thrombotic arterial aneurysm. CONCLUSIONS--Vascular lesions can be responsible for a rapidly developing orbital tumor. Their diagnosis can be difficult and must be made in order to avoid aggressive surgery.

Adolescent↗