Publications (18)85.12 Total impact
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Article: Variable outcome of growth hormone administration in respiratory chain deficiency.
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ABSTRACT: Genetic defects of oxidative phosphorylation (OXPHOS) are known to account for a variety of neuromuscular and non-neuromuscular symptoms in childhood, including growth hormone (GH) deficiency. However GH administration for GH deficiency is controversial in OXPHOS deficiencies as GH is a mitosis-stimulator which may increase energy demand for cell proliferation. Here, we report the observation of four unrelated children with OXPHOS deficiency or bearing a mitochondrial DNA rearrangement and growth retardation, who required GH therapy. The first patient had no GH deficiency while the other three had low GH response to test stimulations. The condition of the first two patients quickly deteriorated under GH administration, GH was then stopped and subsequent clinical improvement was noted. In the other two patients, no adverse event was noted but various additional organs were involved following GH administration. In all patients, no benefit was observed concerning growth response as growth speed remained unchanged. These observations question the use of GH as a treatment of growth retardation for patients with OXPHOS deficiency.Molecular Genetics and Metabolism 03/2008; 93(2):195-9. · 3.19 Impact Factor -
Article: Evaluation of gonadal function in 107 intersex patients by means of serum antimüllerian hormone measurement.
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ABSTRACT: Fetal male sexual differentiation is driven by two testicular hormones: testosterone (synthesized by interstitial Leydig cells) and antimüllerian hormone (AMH; produced by Sertoli cells present in the seminiferous tubules). Intersex states result either from gonadal dysgenesis, in which both Leydig and Sertoli cell populations are affected, or from impaired secretion or action of either testosterone or AMH. Until now, only Leydig cell function has been assessed in children with ambiguous genitalia, by means of testosterone assay. To determine whether serum AMH would help in the diagnosis of intersex conditions, we assayed serum AMH levels in 107 patients with ambiguous genitalia of various etiologies. In XY patients, AMH was low when the intersex condition was caused by abnormal testicular determination (including pure and partial gonadal dysgenesis) but was normal or elevated in patients with impaired testosterone secretion, whereas serum testosterone was low in both groups. AMH was also elevated during the first year of life and at puberty in intersex states caused by androgen insensitivity. In 46,XX patients with a normal male phenotype or ambiguous genitalia, in whom the diagnosis of female pseudohermaphroditism had been excluded, serum AMH levels higher than 75 pmol/L were indicative of the presence of testicular tissue and correlated with the mass of functional testicular parenchyma. In conclusion, serum AMH determination is a powerful tool to assess Sertoli cell function in children with intersex states, and it helps to distinguish between defects of male sexual differentiation caused by abnormal testicular determination and those resulting from isolated impairment of testosterone secretion or action.Journal of Clinical Endocrinology & Metabolism 03/1999; 84(2):627-31. · 6.50 Impact Factor -
Article: [Modalities of transition of diabetic adolescents from pediatrics to the adult care in the Paris-Ile-de-France region: an appeal to cooperative work for improving quality of care. Paris-Ile-de-France Section of DESG (Diabetes Education Study Group) in French language].
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ABSTRACT: The purpose of this study was to evaluate the conditions in which diabetic adolescents are transferred from pediatric to adult health care, and to record the opinions of the physicians about this issue. A questionnaire-based study was performed among all the pediatricians in the hospital setting and all the diabetologists from the Paris-Ile-de-France area. Questionnaires from 50 pediatricians and 51 diabetologists were completed (response rate: 68%). 1) Not enough information was transmitted: a quarter of the diabetologists were visiting for the first time without any information on the adolescent, and only half the pediatricians received feedback information from the internists after the first visit. And yet, when considered, it was important to be kept informed after the first visit and the following ones. 2) Medical relationships were poor: more than three out of four pediatricians and diabetologists had none or very few professional meetings, and two thirds of them were not aware of the way the others were working. 3) Eighty percent of pediatricians and diabetologists considered that the transfer of diabetic adolescents had to be organised in order to keep the coherence of medical follow-up, to minimise the psychological effects of the transition, and to avoid a complete break in the patient follow-up. 4) The expectations of the pediatricians were: the validation of their previous follow-up through the feedback information from diabetologists and the continuity of the medical follow-up; those of the diabetologists were: to gain the patient's confidence and to master the patient's previous history, in order to provide a better follow-up. 5) According to the opinion of both pediatricians and diabetologists, the main errors to avoid were, by the paediatricians, to miss the time and the preparation of the transfer and, by the diabetologists, to denigrate the previous pediatric management and to change the insulin regimen immediately. This study demonstrates a lack of communication between physicians of pediatric and adult health care centres. But it also underlines their recognition of the importance of the transition's stakes and their common motivations in order to improve it.Archives de Pédiatrie 01/1999; 5(12):1327-33. · 0.30 Impact Factor -
Article: Effects of growth hormone in short children after renal transplantation. French Society of Pediatric Nephrology.
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ABSTRACT: 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.Pediatric Nephrology 09/1998; 12(6):437-46. · 2.52 Impact Factor -
Article: Recombinant human growth hormone treatment of children on hemodialysis. French Society of Pediatric Nephrology.
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ABSTRACT: 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.Pediatric Nephrology 05/1998; 12(4):304-10. · 2.52 Impact Factor -
Article: Effects of growth hormone in short children after renal transplantation
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ABSTRACT: 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) 30U/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: 7.7cm with a gain of +0.3 standard deviation score in the treated group versus 4.6cm 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: 5.9cm at 2 years, 5.5at 3 years, and 5.2cm 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.Pediatric Nephrology 01/1998; 12(6):437-446. · 2.52 Impact Factor -
Article: A 5-year prospective study of growth hormone (GH)-deficient children treated with GH before the age of 3 years. French Serono Study Group.
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ABSTRACT: The aim of the study was to assess the efficacy of GH therapy in GH-deficient children treated before the age of 3 yr. A noncomparative multicenter prospective study included 49 children (22 girls and 27 boys) with isolated GH deficiency (n = 19) or multiple pituitary hormone deficiency (n = 30) treated with daily s.c. injections (0.6 U/kg.week) for 3-5 yr. They were divided into two groups according to their height SD score for chronological age (CA) at the initiation of therapy: group A consisted of 8 patients presenting an initial height within the normal range (< 2 SD below the mean) followed for 2-5 yr, and group B consisted of 25 children followed for 5 yr among 41 patients with initial growth retardation. In group A, the mean height SD score increased from -1.1 +/- 0.6 to 0.35 +/- 1.0 SD (P < 0.001) in the first year and remained in the normal range throughout the following 4 yr. In group B after 4 yr of treatment, the mean height SD score for age had increased from -3.6 +/- 1.0 SD (time zero) to -0.9 +/- 1.2 SD. During the fourth year of therapy, the mean height gain of 0.2 +/- 0.2 SD was significant (P < 0.001). After 5 yr of treatment, a plateau was reached with a corresponding height SD score (CA) of -0.8 +/- 1.2 SD (95% confidence interval between -1.3 and -0.2 SD). This value remained significantly below normal for age (P < 0.001), indicating that catch-up growth was incomplete. Only four patients (16%) remained below -2SD for CA. The 5-yr height gain was negatively correlated with the height SD score at the start of treatment (r = -0.6; P < 0.005) and the first year height gain was the most predictive parameter. There was no significant influence of intrauterine growth retardation, body mass index and age at the start of treatment, or parental target height. Bone maturation was significantly retarded over CA by a mean value of 1.1 +/- 0.9 yr (P < 0.0001), with a mean bone age/CA ratio of 0.8 +/- 0.2 after a mean treatment duration of 5.1 +/- 1.1 yr. In conclusion, the rapid and almost complete return to normal height obtained in this study supports the need for GH treatment in early diagnosed GH-deficient children. The present dosage may be considered the minimum to obtain satisfactory catch-up growth ensuring a favorable outcome for these children. In addition, it allowed growth at a rate normal for age in patients diagnosed before growth retardation.Journal of Clinical Endocrinology & Metabolism 02/1997; 82(2):452-6. · 6.50 Impact Factor -
Article: Eating and emotional disorders in adolescent obese girls with insulin-dependent diabetes mellitus.
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ABSTRACT: To study eating and emotional disorders in adolescent insulin-dependent diabetic (IDDM) girls. 98 adolescent girls, aged 13-19 years, were studied: 15 obese and 37 non-obese IDDM girls, 22 obese non-diabetic and 24 non-obese girls, DSM-III-R eating disorders (anorexia nervosa, bulimia nervosa, eating disorders NOS) and eating habits (snacking, sweet compulsions) were evaluated by a semi-structured diagnostic interview (Kiddie-SADS-E and Eating Habits Interview). Emotional disorders were assessed using self-questionnaires (State-Trait Anxiety Inventory for Children, Beck Depression Inventory, Coopersmith Self-Esteem Inventory). Psychological characteristics were correlated with BMI and, for IDDM girls, with HbA1C. IDDM and non-diabetic obese girls showed high rates of eating disorders NOS (sub-clinical bulimia: 60 and 41%, respectively) and they had more extra-snacks than non-obese girls, suggesting that obesity was the main risk factor for additional eating disorders. However, non-obese IDDM girls had more eating disorders NOS (sub-clinical bulimia: 27%) than did the normal girls (4%). Three IDDM girls had typical bulimia nervosa, while none of the non-diabetic did. The risk of depression was increased by both IDDM and obesity (16 and 18% dysthymia, respectively; 8% in normal girls); both factors cumulated in obese IDDM girls (47% dysthymia). Obesity was linked to marked changes in self-esteem scores and mild effects on anxiety. IDDM had little effect on anxiety and none on self-esteem; it even seemed to preserve the self-esteem of obese girls. Patients with bulimia nervosa had poorer metabolic control than other girls with IDDM. There was no correlation between HbA1C and eating or emotional disorders. Adolescent IDDM girls are at increased risk of eating and emotional disorders. Obesity appears to be an important factor for psychiatric complications; more obese IDDM girls suffered from eating disorders NOS sub-clinical bulimia), dysthymia, anxiety disorders, depression and low self-esteem (Family Satisfaction SEI sub-score) than did non-obese IDDM girls.European Child & Adolescent Psychiatry 11/1995; 4(4):270-9. · 2.82 Impact Factor -
Article: [Treatment with recombinant human growth hormone (rhGH) in children with chronic kidney failure or renal transplantation].
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ABSTRACT: Administration of recombinant human growth hormone (rhGH) to children with chronic renal failure (CRF), on conservative treatment or kidney transplanted, may induce acceleration of growth. We report our experience of the first 3 years of treatment in such children. Eight children with CRF on conservative treatment and six kidney transplanted children were included in a European multicentric trial. All children were given rhGH, 30 Ul/m2 body surface area/week, as daily subcutaneous injections, for 12-36 months. The mean growth velocity in children with CRF increased from 3.8 +/- 0.4 cm/yr before treatment to 9.0 +/- 0.4 (P < 0.001), 6.5 +/- 0.3 (P < 0.002) and 5.4 +/- 0.5 cm/yr, after 12, 24 and 36 months of treatment, respectively. The height gain after 2 years of treatment was 1.2 SD (P < 0.001) with bone age advancement not greater than the increase in chronological age. There was a significant decrease in the inulin clearance after 1 year of treatment. In transplanted children, the mean height gain was less important, increasing from 3.2 +/- 0.4 cm/yr before treatment to 6.2 +/- 0.6 cm/yr after 12 months of treatment (P < 0.001). There was no significant decrease in the mean inulin clearance, but two patients experienced rejection crisis. A short-term rhGH treatment may improve growth velocity of CRF or transplanted children. The possible role of GH on decrease in glomerular filtration in CRF and on incidence of acute kidney rejection after transplantation remains to be evaluated in a large cohort of patients.Archives de Pédiatrie 09/1994; 1(8):716-22. · 0.30 Impact Factor -
Article: Recombinant growth hormone in children after renal transplantation. Société Française de Néphrologie Pédiatrique.
The Lancet 03/1994; 343(8896):539-40. · 38.28 Impact Factor -
Article: [Laurence-Moon (Bardet-Biedl) syndrome with growth hormone deficiency].
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ABSTRACT: The features of Laurence-Moon syndrome vary widely from mental retardation, hypogenitalism to retinopathy; the dominant one is progressive neurological involvement, ataxia and spastic paraplegia. Like Bardet-Biedl syndrome, its inheritance is autosomal recessive. This paper describes the endocrine investigation of a new family of Laurence-Moon syndrome subjects. The 5 patients (3 girls, 2 boys) in this family of 12 sibs, all suffered from retinopathy, mental retardation and first metacarpal hypoplasia with proximal placement of thumb. All five had had spastic paraplegia since the age of 5-6 years, growth retardation and hypogonadism. Two had had seizures; only one was obese. Four patients (2 girls, 2 boys, aged 19, 15, 18 and 19 years) had low basal plasma FSH and LH levels. They had no FSH-LH response to gonadotropin-releasing hormone; their plasma testosterone and oestradiol concentrations were very low. The growth hormone peak in response to arginine-insulin were low in these 4 patients (1.5 ng/ml; 3.8 ng/ml; 5 ng/ml; 4.8 ng/ml). One boy and one girl were given sex steroids for a few months, with good effect on their sexual characters. Their growth hormone levels remained low (2.9 ng/ml, 6.5 ng/ml). One boy was given FSH and LH analogues with good effects on sexual characters and testicle growth. He was also given growth hormone, and had a growth spurt of 5.5 cm after 9 months treatment. The hypogonadism of Laurence-Moon patients seems to be of central origin. It is associated with growth hormone deficiency.Archives françaises de pédiatrie 01/1994; 50(10):859-62. -
Article: [Eating disorders in an adolescent population with insulin-dependent diabetes].
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ABSTRACT: The existence of a relationship between Insulin-Dependent Diabetes and eating disorders has recently been observed, but its prevalence and impact on somatic functioning remain poorly understood. These dimensions were evaluated in a population of 52 insulin-dependent diabetic adolescent girls and compared with evaluations of matched subjects from the general population. Results showed that the occurrence of anorexia nervosa is rare, the occurrence of unspecified eating disorders is frequent (35%) and the occurrence of bulimia nervosa is nearly six percent. Poor metabolic control as reflected in blood levels of glycosylated hemoglobin (HBA1C) was found in bulimic subjects and a tendency to be overweight was found in subjects with an unspecified eating disorder. Since such disorders frequently involve dietary restrictions, the role of a restrictive pattern in the occurrence of eating disorders is raised.Canadian journal of psychiatry. Revue canadienne de psychiatrie 12/1993; 38(9):606-10. · 2.42 Impact Factor -
Article: [Sugar diabetes after renal transplantation in children].
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ABSTRACT: Posttransplant diabetes mellitus is ascribed to the use of corticosteroids. Because use of cyclosporine has been associated with increased rates of posttransplant diabetes mellitus, risk factors for this condition have been studied in adults and found to include older age, excessive body weight, and a family history for non-insulin-dependent diabetes mellitus. Only about 1% of children develop diabetes mellitus after transplant surgery. A study of pediatric transplant recipients with diabetes mellitus and of pediatric renal transplant recipients suggested that posttransplant diabetes mellitus may be more common in children with risk factors and may reveal types of diabetes which are infrequent in childhood, e.g., non-insulin-dependent diabetes mellitus which would have gone undiagnosed until adulthood in the absence of corticosteroid therapy. In contrast, corticosteroids apparently had little influence on glucose tolerance in subjects free of risk factors. The effect of corticosteroids seemed to be somewhat less marked than that of renal function impairment.Annales de pédiatrie 03/1993; 40(2):112-8. -
Article: Effects of nutrient intake on growth, insulin-like growth factors, and their binding proteins in a Laron-type dwarf.
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ABSTRACT: In Laron-type dwarfism, a basal growth rate independent of GH and insulin-like growth factor-I (IGF-I) is maintained. This represents a unique model to further assess the relationship between growth and nutritional status. In a child aged 3 yr, 7 months with severe anorexia, growth was followed in relation to his caloric intake. While receiving 496 Cal/day with 11.6 g/day protein, he grew at a rate of 2 cm/yr (period I). The mean plasma IGF-I level was below 0.07 U/mL, insulin was 3.8 +/- 0.2 microU/mL, and blood glucose was 2.9 +/- 0.3 mM/L. During moderate hyperalimentation with 1280 Cal/day and 38.3 g/day protein (period II) for 7 months, growth rate increased to 9 cm/yr with no significant change in plasma IGF-I and persistence of relative hypoinsulinemia (low response to oral glucose tolerance test). IGF-binding proteins, analyzed by Western ligand blotting, showed that 41.5- and 38.5-kilodalton forms, which were initially low, increased to form a pattern similar to that observed in hypopituitarism. These results suggest that catch-up growth did not require normal circulating GH and/or IGF-I activity. Therefore, nutrition contributes to catch-up growth and achievement of potential statural growth by a distinct cellular effect.Journal of Clinical Endocrinology & Metabolism 02/1993; 76(1):248-50. · 6.50 Impact Factor -
Article: Plasma growth hormone-binding activity is low in uraemic children.
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ABSTRACT: Plasma growth hormone-binding protein (GH-BP) activity was evaluated in two groups of prepubertal children with chronic renal failure (CRF) who had been treated with recombinant human GH (rhGH). Group 1 consisted of eight children (mean chronological age 10.8 years) with advanced renal failure; group 2 consisted of nine children (mean chronological age 6 years) presenting with end-stage renal disease, who were on dialysis. Before treatment the specific binding of (125I)hGH to high-affinity GH-BP was low in the two groups (group 1, 17.3 +/- 1.6% of radioactivity; group 2, 14.2 +/- 1.4%) compared with the mean value obtained in normal prepubertal children (24.8 +/- 1.7%). No significant changes in GH-BP activity were found during the 1st year of GH therapy, although growth velocity and plasma levels of insulin-like growth factor-I increased significantly in both groups. The low GH-binding activity found in children with CRF supports the state of GH resistance. The reason for the absence of a GH-BP response to GH therapy has to be clarified.Pediatric Nephrology 08/1991; 5(4):545-7. · 2.52 Impact Factor -
Article: Normal growth hormone (GH) response to GH-releasing hormone in children with thalassemia major before puberty: a possible age-related effect.
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ABSTRACT: The plasma GH response to a single iv bolus dose of 2 micrograms/kg BW synthetic GHRH-(1-44)NH2 was evaluated in 13 prepubertal children with thalassemia major (mean age, 7.6 +/- 0.8 yr) with growth retardation and in 15 prepubertal children with nonendocrine short stature. All of the patients showed a significant increase in plasma GH concentration, with a mean peak of 31.4 +/- 4.5 micrograms/L at 15 min (P less than 0.001 vs. basal values; range, 18.4-65 micrograms/L) after GHRH, which was not different from that of the control group of idiopathic short stature children (40.1 +/- 3.4 micrograms/L; range, 21-65.4 micrograms/L). All but 1 of the thalassemic patients had a normal GH response to the arginine-insulin stimulation test. The mean plasma insulin-like growth factor-I level was low (0.12 +/- 0.05 U x 10(3)/L; range, less than 0.02-0.61 U x 10(3)/L). Analysis of these results as well as previously reported data indicating that older thalassemic patients have an impaired GH response indicates that there may be an age-related pituitary and/or hypothalamic dysfunction in thalassemic children. This study also confirms that the insulin-like growth factor-I decrease occurs before any alteration in GH secretion. These changes might play a role in the early growth retardation that occurs in these patients.Journal of Clinical Endocrinology & Metabolism 09/1989; 69(2):453-6. · 6.50 Impact Factor -
Article: [Central diabetes insipidus in the very young child. Treatment with oral desmopressin].
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ABSTRACT: Neonatal diabetes insipidus (DI) is extremely rare. An efficacious substitutive treatment can be particularly difficult to ensure. We have treated two children with central DI, revealed during the neonatal period, successively with an intranasal preparation of desmopressin at one month of age and with an oral preparation at one and 3 years respectively. We conclude that oral treatment with desmopressin is possible, secure and effective in DI presenting in the first months of life. This new form of administration seems even better for younger children when the difficulties of intranasal administration can be responsible for severe complications. As with the intranasal form, the doses given orally are very variable and individual.Archives françaises de pédiatrie 01/1989; 45(10):787-90. -
Article: Growth hormone response to growth hormone-releasing hormone (hp GHRH1-44) as an index of growth hormone secretory dysfunction after prophylactic cranial irradiation for acute lymphoblastic leukemia (24 grays).
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ABSTRACT: The growth hormone response to growth hormone releasing hormone hp GHRH1-44 (2 micrograms/kg i.v.) was studied in 19 prepubertal children who had been irradiated with 24 Gy for acute lymphoblastic leukemia (ALL) or lymphosarcoma (LS) at a mean chronological age of 4 10/12 years (limits 10/12 to 9 years). They were evaluated after a mean time interval of 4 8/12 +/- 3/12 years and compared to 14 prepubertal children with constitutional short stature (CSS). The individual responses to GHRH were decreased in all but three of the irradiated children. The mean GH response was 16.7 +/- 2.5 ng/ml as compared to 52.6 +/- 8.5 ng/ml in the control group (p less than 0.001). The GH response to GHRH was not correlated with the GH response to arginine-insulin tolerance test (AITT). A decreased response to GHRH with values between 12.5 and 19.4 ng/ml was observed in four cases with normal growth rates and normal GH responses to AITT. These results suggest that an impaired GH response to GHRH is a frequent finding after cranial irradiation for ALL or LS and may be the only sign of GH secretory dysfunction. It is probably indicative of early hypothalamic impairment of GH secretion.Acta paediatrica 10/1988; 77(5):681-7. · 1.77 Impact Factor
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1999
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Hôpital de Poissy Saint Germain en Laye
Saint-Germain-en-Laye, Ile-de-France, France
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