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Biomedical subjects

M Craen

Publications and source records attributed to M Craen.

51 records · Page 3Linked to original sources
PubMed1988

Factors contributing to the impairment of growth in children with acute lymphoblastic leukemia.

Growth was studied in 88 long-term survivors of acute lymphoblastic leukemia who had been treated with three different regimens of therapy. The following time periods were evaluated: (1) during therapy; (2) between the end of therapy and the onset of puberty, and (3) between the onset of puberty and the most recent observation. We found: (1) a reduction of height SDS during therapy, related to the irradiation dose used; no significant effect of the duration of the therapy could be established; (2) a normal growth rate during the second time period studied for the total group, but a further decrease in height SDS for those found to be growth hormone deficient after therapy (47%), and (3) a further decrease in height SDS during puberty. The timing of puberty in the female patients was normal. We conclude that in patients treated for acute lymphoblastic leukemia, growth impairment has several components, different in timing and mechanism.

Adolescent↗
PubMed1987

Treatment of central precocious puberty with an intranasal analogue of GnRH (Buserelin).

One boy and 13 girls with central precocious puberty were treated for 1 year using Buserelin, a GnRH analogue, given intranasally (0.3 mg, four times a day). After 1, 3 and 12 months of therapy, the gonadotropin responses to GnRH were abolished in all the patients whereas mean basal serum concentrations of luteinizing hormone (LH) remained similar to those of pubertal controls. During Buserelin treatment, genital development in the boy and breast development in the girls showed no further progress or some regression. In the boy, serum testosterone levels returned to prepubertal values. In the girls, serum oestradiol levels were variable and, in four of them, vaginal smears showed the persistence of a slight oestrogenic effect during therapy. Pelvic ultrasonography did not show any significant variation in ovarian and uterine lengths. Among the 14 patients, 3 had some progression of pubic hair development, irrespective of serum dehydroepiandrosterone sulphate (DHEAS) levels. In eight patients previously treated with cyproterone, elevated prolactin levels were observed before and during the first month of Buserelin administration. During treatment, mean height velocity was markedly reduced from 11.6 to 6.1 cm/year and mean bone age velocity (+/- 1SD) was 0.85 +/- 0.38 year/year. After 1 year of treatment, the differences in predicted adult height ranged between -0.74 and + 1.04 SDS (standard deviation score). These differences were inversely related (r = -0.72) to the prognosis of adult height calculated before treatment. We conclude that, in central precocious puberty, intranasal administration of Buserelin 1.2 mg/day, may arrest sexual development and reduce height velocity and bone maturation. Improvement of adult height prognosis may occur, especially when it was markedly impaired before treatment.

Administration, Intranasal↗
PubMed1987

Growth hormone deficiency: a hidden obstetrical trauma?

Fetal presentation, mode of delivery and onset of labour were reviewed in 177 patients with documented growth hormone deficiency. Non-cephalic presentations were about ten times more frequent in this group of patients than in a control population. All children with breech position were delivered vaginally and spontaneously, suggesting a pituitary insult during vaginal delivery. 'True idiopathic' isolated growth hormone deficiency was frequently found in association with induction of labour. The data indicate that even a mild birth trauma may result in growth hormone deficiency.

Breech Presentation↗
PubMed1987

Comparative study of the lipolytic potencies of pituitary-derived and biosynthetic human growth hormone in hypopituitary children.

Whether stimulation of lipolysis is an intrinsic property of the human growth hormone (hGH) molecule or is due to contaminants of pituitary origin is controversial. We compared the rises in plasma FFA levels 4 h after an im injection of 0.2 U/kg of either pituitary hGH (n = 5) or biosynthetic methionyl hGH (n = 32) to hypopituitary patients. In each patient, plasma FFA levels also were measured during a similar period of fasting alone, without hGH injection. Plasma FFA levels rose between 0800 and 1230 h in both subgroups of patients during fasting alone. Injection of either pituitary or biosynthetic methionyl hGH led to a greater increase in plasma FFA than that induced by fasting alone, and the percent increases over baseline plasma FFA levels induced by either pituitary or synthetic hGH were similar. Triceps skinfold thickness before and after 3 months of treatment with biosynthetic hGH in 20 patients diminished by a mean of 2.5 mm, a decrease similar to that reported with pituitary hGH. We conclude that the acute and chronic lipolytic effect of hGH in man is an intrinsic property of the hGH molecule.

Adolescent↗
PubMed1987

Evaluation and significance of the degree of pituitary-gonadal inhibition during intranasal administration of buserelin.

In 12 patients (11 girls, 1 boy) with central precocious puberty and 4 patients (3 girls, 1 boy) with idiopathic short stature treated for 1 year with a GnRH superagonist, buserelin (0.3 mg intranasally, 4 times a day), a variable degree of inhibition of sex steroid secretion and pubertal development was observed. Regression of breast or genital development required a daily dosage of buserelin greater than or equal to 34 microgram/kg. After 3, 6, 9 and 12 months of treatment, the serum oestradiol level in the girls was positively related (r = 0.69) to basal serum LH measured at the same time and to change in breast development during the previous 3 months. In contrast, LH response to GnRH was very low in all the patients and not related to the degree of oestradiol inhibition. Height velocity and bone age velocity during the year of treatment showed no significant correlation with mean oestradiol level. Bone age velocity during treatment was inversely related to bone age at onset of buserelin. These data show that 1) the pituitary gonadal suppression during intranasal administration of buserelin is variable and dose-dependent; 2) gonadotropin response to GnRH is not a sensitive indicator of incomplete pituitary suppression during buserelin treatment; and 3) bone age velocity during treatment is more reduced the more advanced bone age is at onset of treatment.

Administration, Intranasal↗
PubMed1985

Effect of growth hormone-releasing factor on plasma growth hormone, prolactin and somatomedin C in hypopituitary and short normal children.

We studied the effect of a single intravenous bolus of 0.5 microgram/kg of growth hormone-releasing factor (GRF) on plasma GH, prolactin (PRL) and somatomedin C (SMC) in 12 short normal children and 24 patients with severe GH deficiency (GHD), i.e. GH less than 5 ng/ml after insulin and glucagon tolerance tests. GRF elicited an increase in plasma GH in both short normal and GHD children. The mean GH peak was lower in the GHD than in the short normal children (8.2 +/- 2.5 vs. 39.2 +/- 5.1 ng/ml, p less than 0.001). In the GHD patients (but not in the short normals) there was a negative correlation between bone age and peak GH after GRF (r = -0.58, p less than 0.005); GH peaks within the normal range were seen in 5 out of 8 GHD children with a bone age less than 5 years. In the short normal children, GRF had no effect on plasma PRL, which decreased continuously between 8.30 and 11 a.m. (from 206 +/- 22 to 86 +/- 10 microU/ml, p less than 0.005), a reflection of its circadian rhythm. In the majority of the GHD patients, PRL levels were higher than in the short normal children but had the same circadian rhythm, except that a slight increase in PRL was observed 15 min after GRF; this increase in PRL was seen both in children with isolated GHD and in those with multiple hormone deficiencies; it did occur in some GHD patients who had no GH response to GRF. Serum SMC did not change 24 h after GRF in the short normal children. We conclude that: (1) in short normal children: (a) the mean GH response to a single intravenous bolus of 0.5 microgram/kg of GRF is similar to that reported in young adults and (b) GRF has no effect on PRL secretion; (2) in GHD patients: (a) normal GH responses to GRF are seen in patients with a bone age less than 5 years and establish the integrity of the somatotrophs in those cases; (b) the GH responsiveness to GRF decreases with age, which probably reflects the duration of endogenous GRF deficiency, and (c) although the PRL response to GRF is heterogeneous, it does in some patients provide additional evidence of responsive pituitary tissue.

Adolescent↗
PubMed1982

Hypopituitarism and idiopathic delayed puberty: a longitudinal study in an attempt to diagnose gonadotropin deficiency before puberty.

Fifty-five hypopituitary patients (43 boys and 12 girls) treated with human GH were studied longitudinally before and during puberty, occurring either spontaneously or induced with testosterone enanthate (100 mg/month, im) in boys and ethinylestradiol (10 micrograms/day, orally) in girls. In addition, 53 boys with idiopathic delayed puberty (IDP) were studied. Gonadotropin integrated responses (IRs) during 90 min after the iv injection of 25 micrograms/m2 LRH, bone ages (BA), and plasma levels of dehydroepiandrosterone sulfate and testosterone were determined at least yearly. Prepubertal hypopituitary patients with gonadotropin deficiency were characterized by: 1) a lowered FSH IR to LRH in most boys and in all girls; 2) a low LH IR for BA; 3) adrenarche either absent or delayed BA; 4) height age close to BA; and 5) the presence of several pituitary deficiencies. In contrast, most prepubertal hypopituitary patients without gonadotropin deficiency showed: 1) a normal FSH IR to LRH; 2) a normal or intermediate (greater than or equal to 75 mIU/ml . 90 min) LH IR for BA; 3) a normal adrenarche for BA; 4) a height age below BA; and 5) isolated GH or GH plus TSH deficiencies. A significant linear correlation was found between LH IR and the logarithm of plasma testosterone. The slopes and levels were similar in controls and hypopituitary boy without gonadotropin deficiency. In IDP, the level was significantly higher. All data obtained in these patients show that the increase in plasma testosterone and the clinical onset of puberty are delayed for the observed pubertal pattern of LH responsiveness. It is concluded that the study of several clinical and biological features, especially the gonadotropin IR to LRH, are of predictive value for the diagnosis of normal or deficient gonadotropic function in prepubertal patients with IDP and hypopituitarism.

Adolescent↗
PubMed1979

Neuropsychological study in treated thyroid dysgenesis.

Neuropsychological assessment was carried out in 57 patients aged 3.0 to 17.5 years (mean 8.5) with thyroid dysgenesis under adequate long-term therapy. Starting age of hypothyroidism as estimated by bone age at diagnosis was prenatal in 32 cases, close to birth in 13 cases and postnatal of 1-12 months in 12 cases. Hypothyroidism of prenatal onset results in severe neuropsychological disorders and mental retardation if not treated early. Only some signs of "minimal brain dysfunction" which compensate with advancing age and normal IQ are found in these children if therapy is started before one month of age. Hypothyroidism starting at birth does not result in mental retardation but neuropsychological disorders are found. They are more frequent if treatment is started after 6 months of age. Children who become hypothyroid between 1 and 12 months of age are usually not mentally retarded and show minor neuropsychological disorders.

Adolescent↗
PubMed1976

A new chromosome deletion syndrome. Report of a patient with a 46,XY,8p- chromosome constitution.

A mentally retarded boy with narrow cranium, high forehead, epicanthic folds, flat nasal bridge, low-set but normal ears, retrognathy, short neck and broad chest with wide-set nipples is described. The routine chromosome and banding analyses revealed a 46,XY,8p- chromosome constitution. The parents had normal chromosomes. On the basis of measurements on reflectodensitometer tracings and of measurements of the individual G-bands directly on photographic prints of the normal and abnormal chromosome 8, an intercalary deletion of band p22 seemed most probable. The clinical features of the patient are compared with the 8p- case of Lubs & Lubs (1973) as well as with four other cases of short arm deletion of an unidentified C-autosome. The clinical features of one of these cases were very similar to those of the boy described. An attempt was made to delineate the clinical characteristics of this new chromosomal deletion syndrome.

Child↗
PubMed1975

Short arm deletion of chromosome 12: report of two new cases.

Two boys (W.M. and C.P.) are described, in each of whom a short-arm deleted C chromosome was apparently present. The clinical findings on W.M. are stenosis of the sagittal sutura associated with atrophy of the nervus opticus and mental retardation, and on C.P. osteogenesis imperfecta. An analysis of the G- and Q-banding patterns revealed in each patient a 12p-- chromosome. The deletion involved most of band p12 as shown by length measurements on G-banded chromosomes. Both cases were compared to proven and presumed cases of 12p-- from literature, but no common clinical phenotype could be demonstrated.

Chromosome Aberrations↗
PubMed1975

Unexpected findings with the new chromosome banding techniques in a patient formerly diagnosed as having G-deletion syndrome II.

A girl is described, who, upon her first admission to our pediatric department in 1965, was supposed to have G-deletion syndrome II on the base of the chromosome findings in routine orcein-stained preparations and her clinical aspect. When, however, in 1972 a Q- and G-banding analysis was performed, the patient seemed to be a t(14q+; 22q-) translocation carrier. These findings are discussed in relation to the known heterogeneity of the clinical picture of patients previously reported as having a G-deletion syndrome and in whom no banding studies were done. Finally, the need for more extensive studies with the different techniques is stressed, particularly, in cases of Gq- chromosomes, of which the familial occurrence is suggestive of a hidden reciprocal translocation.

Child↗