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Biomedical subjects

P Bareille

Publications and source records attributed to P Bareille.

15 recordsLinked to original sources

Growth hormone and segmental growth in survivors of head and neck embryonal rhabdomyosarcoma.

AIMS: To assess the impact of treatment for embryonal rhabdomyosarcoma on spinal growth and limb length and examine the response of these parameters to growth hormone (GH) treatment. METHODS: We conducted a retrospective case note review of 17 survivors of head and neck rhabdomyosarcoma followed up at a single institution. All children had been treated with chemotherapy and local radiotherapy. Growth velocity, height, sitting height, and subischial limb length SDS scores were analysed. RESULTS: Growth failure secondary to isolated GH deficiency (GHD) developed in 7/17 patients. GHD occurred at a median (range) of 3.4 (1.3-9.9) years after radiotherapy tumour doses of 46 (40-50) Gy. Growth velocity, height, and subischial limb length SDS were significantly reduced in the GHD group and improved with GH therapy. CONCLUSIONS: GH treatment resulted in a significant improvement in sitting height SDS. We discuss the unexpected improvement in spinal growth in survivors with GHD.

Body Height↗

Growth and endocrine disorders in multisystem Langerhans' cell histiocytosis.

INTRODUCTION: Langerhans' cell histiocytosis is a rare disorder, with diabetes insipidus occurring in up to half of patients. Causes of growth failure include the illness itself, treatments used and growth hormone insufficiency. PATIENTS AND METHODS: We identified all patients with an endocrinopathy secondary to Langerhans' cell histiocytosis (LCH). Growth data were analysed from all patients with multisystem involvement. RESULTS: Of 144 patients with multisystem LCH, 50 had an endocrinopathy, 49 of whom had diabetes insipidus. Growth hormone insufficiency (GHI) was present in 21 patients, seven of whom had other anterior pituitary deficiencies as well (gonadotrophin deficiency + GHI n = 2, gonadotrophin deficiency + TSH deficiency + GHI n = 2, panhypopituitarism n = 3). GH insufficiency, the development of which appeared to be independent of pituitary radiation, occurred at a median age of 8.3 years (4.7-18 years) and at a median interval of 3.5 years (0-11.8 years) after diagnosis of LCH. The median height SDS at diagnosis of growth hormone insufficiency was -2.9. Thirteen of the patients with growth hormone insufficiency attained final height with a median height SDS of -1.2. The final height SDS of 15 patients without GH insufficiency was closer to target height SDS, but not statistically different from that of the GH insufficient group. CONCLUSIONS: GH therapy significantly improves growth in GH insufficient patients with Langerhans' cell histiocytosis. Early institution of GH therapy may further improve height outcome. However, most children with Langerhans' cell histiocytosis regardless of endocrine function, failed to reach target height.

Adolescent↗

Lipid profile, glucose tolerance and insulin sensitivity after more than four years of growth hormone therapy in non-growth hormone deficient adolescents.

OBJECTIVE: To study the effects of long-term (> 4 years) growth hormone (GH) therapy on insulin sensitivity, glucose tolerance and lipid profile in non-GH deficient adolescents at completion of their growth. SUBJECTS: Thirty non-GH deficient (15 'idiopathic' short stature, 8 intrauterine growth retardation, 7 partial GH deficiency in childhood but normal on retesting) were recruited, median (range) age 16.9 years (15-20.3) prior to ceasing their GH therapy. Their median (range) duration of GH treatment was 7.9 years (4-11). Insulin sensitivity was also recorded in 10 normal controls with a median (range) age of 20.5 years (18.4-22.3). METHODS: Insulin sensitivity was assessed by a short insulin tolerance test in 18 patients on GH therapy and controls. It was repeated in 14 patients six months after stopping their GH therapy. A 3-h standard oral glucose tolerance test (OGTT) was performed in 19 patients on GH therapy, and repeated after 6 months off GH in 10 patients. Fasting lipids were also measured. RESULTS: Insulin sensitivity index was significantly lower in the patients on GH therapy than in the controls, (median (range)) 3.7%/min (1.2-5.3) and 5.3%/min (3.8-6.2), respectively. Six months after termination of GH therapy, insulin sensitivity increased significantly from 3.6%/min (1.2-5) to 4. 8%/min (2.8-5.6). Fasting plasma insulin decreased significantly off GH therapy from 10.1 to 3.6 mU/l. The area under the insulin curve during the OGTT was also significantly higher on GH therapy. Apart from one patient with impaired glucose tolerance on GH treatment, plasma glucose concentrations remained within the normal range. No lipid abnormalities were recorded. CONCLUSIONS: These data suggest that long-term GH therapy may cause insulin resistance in non GH deficient adolescents, but usually with neither impaired glucose tolerance nor hyperlipidaemia.

Adolescent↗

Multiple neonatal endocrinopathies in McCune-Albright syndrome.

Two cases of McCune-Albright syndrome (MAS) are reported who presented in the neonatal period with profound failure to thrive, cardio-respiratory distress, precocious puberty and Cushing's syndrome for which both underwent bilateral adrenalectomy. Both girls had also bilateral nephrocalcinosis; in one case that may have been attributed to Cushing's syndrome, but in the second case the cause remained obscure with no obvious abnormality of calcium metabolism. The first girl had hydrocephalus which is uncommon in this condition and the second girl still failed to thrive at the age of 6 years, despite adequate caloric intake and hormonal manipulation. A constellation of other abnormal features are described. These cases illustrate the complexity of MAS which can become a life-threatening or a debilitating disorder.

Age of Onset↗

Growth hormone treatment in growth hormone-sufficient and -insufficient children with intrauterine growth retardation/Russell-Silver syndrome.

Fifty-eight short prepubertal children with IUGR received GH treatment (mean dose: 28 IU/m2/week) for a mean (SEM) period of time of 3.4 (0.13) years (range 1-4 years). They were subdivided according to their GH response to a pharmacological test. Twenty-six were GH insufficient (GHI) (group 1) and 32 were non-GHI (group 2). At the commencement of GH therapy mean chronological age was 6.1 (0.4) years in both groups, mean height SDS (SEM) was -3.5 (0.2) in group 1 and -3.6 (0.2) in group 2, mean growth velocity (GV) SDS (SEM) was -1.9 (0.3) in group 1 and -0.3 (0.2) in group 2. GH therapy induced significant growth acceleration throughout the follow-up period without any significant differences between the two groups. GV SDS (SEM) increased to +3.0 (0.5) in group 1 and to +3.7 (0.4) in group 2 (p < 0.05 compared to baseline) during the first year of therapy. Subsequently, the growth-promoting effects of GH therapy diminished with time but GV remained significantly higher than baseline. This growth enhancement produced a significant rise in height SDS (SEM) reaching - 1.4 (0.2) in group 1 and - 1.7 (0.2) in group 2 after 4 years. In conclusion, our data did not show any significant differences in the growth response to GH therapy between GH-sufficient and -insufficient IUGR children who were only distinguishable by their GH secretion. This indicates that the decision to treat a short IUGR child with GH therapy should not be based upon the GH response to a provocative test.

Body Height↗

Final height outcome in girls with Turner syndrome treated with a combination of low dose oestrogen and oxandrolone.

Final stature in girls with Turner syndrome treated with combination of low dose oestrogen and oxandrolone. Nineteen prepubertal girls with Turner syndrome (mean age 10.9 years, range, 8.9-14.2 years) were randomly assigned to receive either oxandrolone (0.05 mg/kg/day) or ethinyloestradiol (40 ng/kg/day) for 1 year. Subsequently the alternate therapy was added and the combination given until attainment of final height. Ethinyloestradiol was gradually increased at the age of 12.5 years in order to induce secondary sexual characteristics. The duration of treatment was a mean of 5.2 years (range, 3.7 years) when the 1st year of monotherapy was included. Therapy produced a sustained acceleration in growth rate for a duration of 4 years and eventually has resulted in an increment of mean adult height of 3 cm relative to pre-treatment projected height with mean values of 146.5 cm versus 143.5 cm respectively. The moderate side-effects observed did not cause any of the girls to discontinue treatment. Nevertheless, amelioration of adult height appears to be modest, notably in comparison to published data of growth hormone treatment and 4 girls had a decrease in final height prediction. CONCLUSION Combination of low dose of oxandrolone and oestrogen may have a moderate but positive impact on final height in girls with Turner syndrome. However, some girls do worse than predicted in term of final height using this regimen. Oestrogen therapy started at low dose around the age of 10 years and increased gradually at approximately 12.5 years to induce secondary sexual characteristics does not have a deleterious effect on adult height in Turner syndrome. In summary, low dose oxandrolone-oestrogen treatment was found to accelerate the tempo of growth in girls with Turner syndrome, but did not appear to have a consistent effect on final height.

Adolescent↗

Growth hormone treatment without a needle using the Preci-Jet 50 transjector.

A new delivery system (Preci-Jet 50) which administers growth hormone through the skin using high pressure and without a needle was evaluated. This device was inconvenient and painful compared with a pen injection system. The conclusion is that the Preci-Jet is not the panacea for solving the problem of compliance with subcutaneous growth hormone injections.

Administration, Cutaneous↗

[Constant-flow drug administration in anesthesia].

The authors describe a technique of constant flow perfusion of several anaesthetic drugs, using a simple formula to adjust the perfusion rate to the weight of the patients. Calculation of dilutions is easy: the drug concentration is equal to ten times the rate, per hour and per kg, in practical cases. The flow rate is then a tenth of the patient's weight. This method is easy to apply to several drugs, of which pharmacokinetic parameters justify this administration mode: alfentanil, etomidate, vecuronium bromide... but clinical and instrumental observations should be taken into account for the adjustment of the flow.

Alfentanil↗

[Interaction between electrocautery and a pacemaker].

Following a new case of inhibition of a sentinel pacemaker by the cutting current of an electrocoagulator during endoscopic urologic surgery, the mechanism of this complication is recalled. The non-selectivity of the pacemaker detector circuit is responsible for interpreting the electrical disturbances due to the electrocoagulator as cardiac activity. The problems seen with other types of stimulators are discussed, especially programmable stimulation where the use of a magnet can lead to variations in the stimulator frequency. The stimulating wire can also be responsible for accidents, such as myocardial burns, and rhythm disturbances. The safety rules for the use of the electrocoagulator in patients with a non programmable sentinel pacemaker.

Aged↗

[Use of vecuronium bromide administered at a constant rate during renal transplantation].

The authors describe a technique of vecuronium bromide perfusion at constant flow used to obtain curarisation during 50 renal transplantations. The muscular effects of the drug were monitored through an electromyographical recorder, so as to adjust the perfusion rate. The dose of vecuronium bromide was 47.7 +/- 0.74 micrograms X kg-1 X h-1 (mean +/- SEM), this being lower than the dose recommended by D'Hollander (60 micrograms X kg-1 X h-1) for patients with normal renal function. Furthermore, no recurarisation was observed. Therefore, vecuronium can be considered as a satisfactory muscle relaxant in patients with renal failure, but neuromuscular monitoring appears to be a most important safety factor.

Adult↗

[Induced normovolemic hemodilution. Calculation of the amount of blood to be withdrawn].

The authors study the variation of the hematocrit during a provoked normovolemic hemodilution. To get a predetermined hematocrit rate they use a simple and original biophysical model which gives the exact blood volume to be extracted. Then they use the same method to evaluate the hematocrit of the extracted blood after its conditionnement in packs.

Hematocrit↗