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Biomedical subjects

P Cochat

Publications and source records attributed to P Cochat.

At least 19 recordsLinked to original sources
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↗
PubMed2005

[What is new in pediatric nephrology?].

Recent advances in the knowledge of physiology, genetics, imaging, and therapeutics have lead to novel practical approaches in paediatric nephrology. Many inherited syndromes have been revisited in order to identify precise renal diseases at the molecular level. In addition, a large number of epidemiological studies and clinical trials have allowed guidelines and recommendations to be provided for chronic and end-stage renal failure, urinary tract infection, glomerular diseases, etc.

Child↗
PubMed2005

[MURCS association: a challenging diagnosis].

MURCS association includes Mullerrian duct aplasia-hypoplasia (MU), renal malformations (R) and cervicothoracic somite dysplasia (CS). This rare disease (1/50 000 females) is sporadic and of unknown aetiology. The reported case is the first one with additional esophageal atresia and ovarian mature teratoma. Esophageal atresia first led to the diagnosis of VACTERL association, which is more frequent and well known, showing that the identification of such malformative association may be challenging. The presence of mullerrian abnormality has allowed the diagnosis of MURCS association, as there is no mullerrian hypoplasia in VACTERL association. Therefore the association of isolated or combined renal and cervical malformation with VACTERL features should lead to the search for mullerrian abnormalities.

Abnormalities, Multiple↗
PubMed2005

[Kidney transplanted child and sport practices].

OBJECTIVE: Evaluation of physical capacity in kidney transplanted children for sport practice adequacy. POPULATION AND METHODS: A retrospective study was carried out in 32 children transplanted at least one year before evaluation. We studied auxology, respiratory function testing, cardiovascular parameters at rest and after exercise, and aerobic capacity performed during a triangular effort test. RESULTS: Kidney transplanted children were smaller and had a higher body mass index (IMC). Breathing parameters and cardiac ultrasound examinations were normal in all but one child. After exercise, four children had high systolic blood pressure requiring a treatment adaptation. Lower values of maximal aerobic power (Pmax) and oxygen maximal uptake (VO2max) were noticed, mainly in girls. CONCLUSION: Kidney transplanted children have a good physical exercise tolerance. Therefore they can be allowed to practise sports at school or in a club after a normal effort test (taking into account the risk of transplant trauma).

Adolescent↗
PubMed2005

Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study.

OBJECTIVE: To describe the clinical and laboratory manifestations of childhood-onset systemic lupus erythematosus (SLE) at presentation. STUDY DESIGN: This retrospective French multicenter study involved 155 patients in whom SLE developed before the age of 16 years. Mean patient age at onset was 11.5 +/- 2.5 years (range, 1.5-16 years). The female to male ratio was 4.5. RESULTS: The most common initial manifestations were hematologic (72%), cutaneous (70%), musculoskeletal (64%), renal (50%), and fever (58%). Thirty-two percent of children had atypical symptoms, mainly including abdominal involvement in 26 patients, which lead to negative laparotomy results for presumed appendicitis. Severe renal, neurologic, hematologic, abdominal, cardiac, pulmonary, thrombotic, and/or cutaneous manifestations occurred within the first month after the diagnosis in 40% of patients. The mean erythrocyte sedimentation rate was 72 +/- 29 mm/h, and the mean C-reactive protein value 22 +/- 21 mg/L. Antinuclear antibodies an, anti-double stranded DNA antibodies, and low C3 or C4 level were retrieved in 97%, 93%, and 78 % of patients, respectively. CONCLUSION: Initial manifestations of childhood-onset SLE are diverse and often severe. The diagnosis of SLE should be promptly considered in any febrile adolescent with unexplained organ involvement, especially when associated with an increased erythrocyte sedimentation rate.

Child↗
PubMed2005

[Relation between ocular manifestations and organ involvement in ten patients with Fabry disease].

PURPOSE: To describe ocular manifestations in Fabry disease and compare them to other organ involvement in this disease. PATIENTS AND METHODS: Ten patients were included in a clinical trial, all of whom had specialized investigations in ophthalmology, cardiology, nephrology and dermatology. RESULTS: All the patients presented with ocular damage, some of which were rather characteristic of this disease, such as cornea verticillata and spoke-like cataract. However, such anomalies were not responsible for any visual acuity changes in most patients. Some patients had renal or cardiovascular damage at early stages of the disease. We therefore tried to establish a correlation between the severity of such involvement and ocular damage. CONCLUSION: Many ocular manifestations are frequently observed that should suggest a diagnosis of Fabry disease. However, there is no clear relationship between the presentation of the systemic cardiac and renal manifestations of the disease and the presence of ocular abnormalities.

Adult↗
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↗
PubMed2004

[Mortality in pediatric renal transplants: 15 years' experience].

UNLABELLED: The aim of the study was to analyse retrospectively the patient survival and mortality risk factors in renal transplant children at Edouard-Herriot hospital, Lyon. PATIENTS AND METHODS: One hundred and ninety four patients 0 to less than 18 years of age received a renal transplant between April 1987 and January 2002. We collected data concerning the age at transplantation, the mean duration of dialysis prior to transplantation, the type of donor (living related: LRD, or cadaver: CAD) as well as long term follow-up of all the patients. Eleven patients died during the study period and we collected mortality and morbidity factors. RESULTS: Mean age at transplantation was 9.5 years (range: 0.6-17.9 years); 16 children were transplanted before 2 years of age. Eighty six percent received a first graft, 10% a second graft, and 3% a third graft. One third of the patients had preemptive transplantation. The graft was carried out respectively from a LRD in 18% and from CAD in 82% of the cases. Eleven patients (5.7%) died during this period; four of them were transplanted twice. Death occurred within 2.4 years after transplantation (range: 2 days-6.3 years). Five of these deceased children developed an acute rejection episode treated with methylprednisolone pulses and OKT3 monoclonal antibodies. Four had a cytomegalovirus (CMV) infection and three required repeated surgery. The causes of death were bacterial infections (4/11), CMV infection (2/11), neurological involvement (2/11), Burkitt lymphoma (1/11) and unexplained sudden death (2/11). CONCLUSION: The main causes of deaths in renal transplant children are bacterial infections. The improvement in patient survival probably will come from both better preventive approach to nosocomial infections and a less aggressive immunosuppression.

Actuarial Analysis↗
PubMed2004

Renal outcome of children exposed to cyclosporine in utero.

The number of pregnancies in immunosuppressed women has increased during the recent years and this has become a major part of the rehabilitation and quality of life of treated patients. Most of them are organ transplant recipients and large series from the literature have shown that children born to such women may present with intrauterine growth retardation, a condition which may be associated with significant reduction in nephron number and oligomeganephronia. On the other hand, experimental data in animals have demonstrated that in utero exposure to CsA may alter nephrogenesis and further alter renal function. Therefore offspring of organ transplant women treated with CsA exhibit a theoretical risk of renal impairment, due to both IUGR and fetal nephrotoxicity. However, despite the limited experience of long term studies in children, there is no evidence of any significant deleterious adverse effect of in utero exposure to CsA. However further studies based on large series are required in order to demonstrate that renal fetal effects have limited clinical consequences.

Animals↗
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↗
PubMed2003

[Childhood-onset systemic lupus erythematosus].

Systemic Lupus Erythematosus (SLE) remains a challenging autoimmune disease in term of etiology, pathogenesis and treatment. It is estimated that 10-17% of lupus patients present before the age of 16. SLE in children appears to have more severe organ involvement than in adults. The outcome of childhood SLE has improved during the last decade, but the morbidity remains high.

Adolescent↗
PubMed2003

Quality of life of children and adolescents after kidney or liver transplantation: child, parents and caregiver's point of view.

A cross-sectional study was performed to assess quality of life (QoL) after kidney or liver transplantation during childhood. Self-questionnaires explored children, adolescent and parent QoL. Seventy-five transplant children, 36 transplant adolescents, 67 mothers, 34 fathers and 67 caregivers filled out the questionnaires; they were compared with a reference population. Children reported a rather good QoL, but their extra-family involvement appeared not as satisfactory as that of the reference population children. Adolescents reported a very high QoL when completing the structured format scale; however, their responses to open-ended questions showed qualitative differences compared with those of a reference population: they expressed concern about their body or health, less pleasure than ordinary adolescents to manage by themselves, and a poor relationship with peers. Mothers indicated a deep impact of the child's illness on their own QoL, and the need for psychological support. In conclusion, a rather good QoL is a long-lasting feature of kidney and liver transplantation in children. Such an assessment is mandatory to identify remaining impairments in some selected areas, either in the recipient or family.

Adolescent↗