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Biomedical subjects

E Bérard

Publications and source records attributed to E Bérard.

At least 19 recordsLinked to original sources
PubMed2006

[Information guides of pediatric patients and their families: recommandations of the Commission d'Ethique de la Société Française de Pédiatrie].

Giving informations to the patient and it's parents, is a deontologic and legal obligation. Modalities of information has been given in a French law (4th mars 2002), making complete former texts. If orally delivered information remains the basic rule, the use of patient's information guides could be useful. After recall of legislative obligations, we propose recommendations for redaction and methodology for validation of such information guides, by specialised working groups of the Société française de pédiatrie, using a Delphi method.

Age Factors↗
PubMed2000

[Pigmentosum retinis and tubulo-interstitial nephronophtisis in Sensenbrenner syndrome: a case report].

PURPOSE: Sensenbrenner syndrome or cranio-ectodermal dysplasia is an extremely rare autosomal recessive condition (12 cases reported in literature). Our observation shows the possibility of both ocular and renal involvement associated with cranio-ectodermal abnormalities. PATIENTS: and method:We report the case of a girl who presented a typical cranio-ectodermal syndrome with dolicocephaly, short thorax, short limbs, short fingers and teeth abnormalities. At five years, she was found to have pigmentosum retinitis with amblyopy and moderate hyperopia. A chronic renal failure with uncontrollable hypertension underwent a cadaveric-donor transplantation at the age of six years. RESULTS: Two years later, the pigmentosum retinitis was stable. The kidney histology revealed a tubulo-interstitial nephronophtisis. The molecular analysis of the NPH 1 locus, which was associated with nephronophtisis, was negative. DISCUSSION: Our observation and two recent publications have in common ocular and renal abnormalities associated with cranio-ectodermal dysplasia. The underlying genetic defect would involve not only morphogenesis but also development and maturation of organs as eye and kidney. Sensenbrenner syndrome would thus be similar to certain disorders affecting the eye, kidney, skeleton and ectodermal structures such as the EEM, Senior-Loken, Mainzer-Saldino, and Jeune syndromes. CONCLUSION: The retinal dystrophy falls within the spectrum of clinical and genetic forms of pigmentosum retinitis. Our observation would confirm possible links between Sensenbrenner syndrome and oculorenal syndromes.

Abnormalities, Multiple↗
PubMed1999

[Antiretroviral therapy and prevention of maternal-fetal transmission of HIV-1. Current and future strategies].

Azidothymidine is effective and recommended for the prophylaxis of vertical HIV transmission. Data regarding this treatment have been collected over the last decade, leading to it being widely prescribed despite the lack of information concerning its long term toxicity. Antiretroviral drug combinations administered during pregnancy appear to ensure a better protection of both mothers and their offspring. However, data available on the adverse effects of these therapies during pregnancy are scarce and mainly obtained from in vitro or animal models. Therefore there is a need for multicentric trial including long-term follow-up of exposed patients.

Animals↗
PubMed1998

Renovascular hypertension and vascular anomalies in Alagille syndrome.

Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, peripheral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubulointerstitial nephritis, renal tubular acidosis, and mesangiolipidosis have been noted in AS. The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least four of the five major features of AS who developed secondary hypertension of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis and various other abnormalities of the main arteries in all five patients: aorta (3 cases), celiac artery (4 cases), superior mesenteric artery (1 case), subclavian artery (1 case). Our findings underscore the value of arterial blood pressure monitoring in patients with AS. If hypertension occurs, a renovascular origin should be sought. The diffuse vascular abnormalities which appeared to be a feature of AS in these patients should prompt larger studies of vascular abnormalities in AS.

Acidosis↗
PubMed1998

Recombinant human growth hormone treatment of children on hemodialysis. French Society of Pediatric Nephrology.

Forty-two children, aged 2-21.5 years on hemodialysis with a height below -2.0 standard deviation score (SDS) for age, were selected to receive recombinant human growth hormone (rhGH) therapy at 17 French centers. Of the 42 children, 36 were prepubertal and 8 were in early puberty (testicular volume between 4 and 8 ml for boys, breast development B2 or B3 in girls). All received 1 IU/kg per week by daily subcutaneous injection for 1-5 years. The year before rhGH therapy served as a control period. During the 1st year of treatment, mean growth velocity increased from 3.5 to 7.0 cm/year (P < 0.0001) and was always over 2.5 cm/year. This velocity allowed a catch-up growth of +0.5 height SDS. Neither weight nor the body mass index varied compared with the pretreatment year. No change was observed in urea, creatinine, or glucose tolerance. The mean increment in bone age was 0.9 years. The mean growth velocity decreased over subsequent years (P < 0.0001), but remained higher than the prestudy velocity. A significant negative correlation was observed during the 1st year between the increase in growth velocity and the prestudy velocity (P < 0.0001), with the least gain in patients who had the best spontaneous velocity. Pubertal status had no influence on response to rhGH. No significant side effects were observed during the 103 treatment-years. Five patients developed secondary hyperparathyroidism and 1 suffered from acute pancreatitis, but the relationship with rhGH therapy remains uncertain. rhGH therapy appears indicated for children on hemodialysis, even though the potential benefits appear somewhat lower for those with a spontaneous growth velocity over 6 cm/year.

Adolescent↗
PubMed1998

Effects of growth hormone in short children after renal transplantation. French Society of Pediatric Nephrology.

From 1991 to 1993, 90 children having received a kidney graft with a post-transplantation period of at least 12 months were included in a prospective study carried out in 18 French pediatric centers. After informed consent and randomization, children received recombinant human growth hormone (rhGH) (Genotonorm, Pharmacia peptide hormones) 30 U/m2 per week, either immediately on enrollment, for the treated group, or after 1 year of follow-up for the group serving as a control. After 1 year both groups were treated and we analyzed data during the subsequent years. Eighty-five children completed the 1-year study. Growth velocity was significantly increased by rhGH: 7.7 cm with a gain of +0.3 standard deviation score in the treated group versus 4.6 cm in the control group (P<0.0001) during the 1st year. Four factors predicted response to therapy: growth velocity prior to GH therapy, glomerular filtration rate (GFR) at the start, mode of corticosteroid administration, and degree of insulin resistance. After 1 year we observed a moderate, significant decrease in GFR in both groups. Biopsy-proven acute rejection episodes were not significantly more frequent during the 1st year in the group of patients who received rhGH: 9 in 44 versus 4 in 46 patients. The patients who rejected did not differ in terms of age, renal function at the start, and type of immunosuppression, but history of rejection before GH treatment was discriminatory: 6 of 17 children with two or more episodes had a new rejection versus 1 of 22 who had no or only one episode (P=0.01). Glucose tolerance was not modified after 1 year of GH therapy. During the subsequent years of treatment a decrease in growth velocity was noted: 5.9 cm at 2 years, 5.5 at 3 years, and 5.2 cm at 4 years. In conclusion, GH is efficient for improving growth velocity in short transplanted children, inducing clear-cut but limited catch-up growth. The risk of rejection was shown only in patients with a prior history of more than one rejection episode.

Adolescent↗
PubMed1997

[Metabolism and regulation of nitric oxide: a hard-to-control mediator].

Although nitric oxide (NO) is implicated in numerous regulatory mechanisms, its therapeutic use remains problematic. Synthesis of this mediator of low specificity with multiple effects involves two types of enzymes (constitutive and inducible). The complexity of the corresponding regulatory mechanisms precludes control for therapeutic use. As NO interacts with numerous metabolic pathways and can also be stored, interpretation of experimental results is difficult, which hinders development of therapeutic trials. In addition, NO is a free radical and thus participates in the free radical cascade. Another difficulty in use of NO is its role in equilibrium implicating still poorly understood mediators (such as endothelin at a vascular level). The complexity of NO pathways explains why therapeutic trials of NO to date have proven unsatisfactory except for treatment of arterial pulmonary hypertension.

Animals↗
PubMed1996

Is the response to rhGH in haemodialysis patients less effective than in patients with chronic renal failure? Société de Néphrologie Pédiatrique.

A total of 82 prepubertal children with chronic renal insufficiency and with (HD, n = 28) or without (CRI, n = 54) haemodialysis were treated with rhGH, 1 i.u./kg/week. The absolute response (cm/year) was better in CRI children, but the growth velocity gain was similar in the two groups. In addition, the decrease in growth velocity during the second year on rhGH was more marked in CRI patients.

Analysis of Variance↗
PubMed1995

Nephrocalcinosis and prematurity: importance of urate and oxalate excretion.

Nephrocalcinosis was described in preterm infants by several authors who tried to determine its association with hypercalciuria and furosemide therapy. We evaluated these potential mechanisms along with other lithogenic factors not previously studied in 10 premature babies. Hypercalciuria was an inconsistent finding like in other reports; elevated uric acid excretion and hyperoxaluria were observed in 5 and 6 cases, respectively. The aminocid excretion was normal in all infants. Our data suggest that in addition to hypercalciuria, other lithogenic factors may play a role in the pathophysiology of nephrocalcinosis of premature infants.

Glycosuria↗