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Biomedical subjects

B Leheup

Publications and source records attributed to B Leheup.

51 records · Page 3Linked to original sources
PubMed1988

1-year treatment with recombinant somatropin in prepubertal and pubertal growth hormone deficient patients: results from a French multicentre trial.

In this French study with recombinant somatropin, the stimulation of growth in 32 prepubertal (age 10.0 +/- 3.5 years; mean +/- SD) and 19 pubertal (age 14 +/- 1.5 years) GH deficient children was compared; the stimulation of growth was similarly good in the two groups. The height velocity SD scores increased from -2.5 +/- 1.7 and -0.9 +/- 1.5 to 2.2 +/- 1.9 and 1.6 +/- 1.6 in prepubertal and pubertal children, respectively. Expressed as cm/year, these correspond to increases from 3.2 +/- 1.3 cm/year and 4.1 +/- 1.2 cm/year to 8.1 +/- 1.5 cm/year and 8.6 +/- 1.9 cm/year in the prepubertal and pubertal patients, respectively. Safety and tolerance were good and the immunogenicity of Genotonorm was low.

Adolescent↗
PubMed1988

[The current practice of prevention of rachitis in France. A survey of 600 physicians].

French legislation prohibiting vitamin D food supplementation, rachitism prophylaxis is based on specific infantile calciferol administration, according to an old ministerial recommendation published in 1963. Out of 1000 questioned physicians, 600 completed the present inquiry. Ninety-nine per cent of them indicated that they systematically prescribe vitamin D for infants, but 18% do not propose it to breast-fed babies; 36% begin prophylactic administration only after 2 weeks and 5% after one month of age; 16% stop prevention between 12 and 18 months, 72% between 2 and 6 years and 12% after 8 years. Expressed on a daily basis, vitamin D dosage is usually rather high, generally slightly higher than the officially recommended intake (1100 to 1400 versus 1000 IU/24 h) and can reach excessive levels, especially in coloured people for which the already large proposed dose (2500 IU/24 h) is exceeded by 13% of inquired physicians. Daily rhythm of supplementation is the most frequent (94% of physicians) before 2 years, semestral or annual afterwards. The mode of administration, diluted in milk (40%), water (27%) or fruit juice (14%) depends on the physicians. In free commentaries, none of them speaks about maternal supplementation during pregnancy. Very few propose special dosage for premature babies. These defects in rachitism prophylaxis are partly due to the obsolescence of official recommendations which postpone the beginning of vitamin D prevention until the age of 6 weeks, do not mention maternal supplementation during pregnancy and do not specify the necessity for vitamin D intake in breast fed infants.(ABSTRACT TRUNCATED AT 250 WORDS)

France↗
PubMed1987

Influence of testicular secretions on differentiation in the rat epididymis: ultrastructural studies after castration, efferent duct ligation and cryptorchidism.

The differentiation of the rat epididymis was studied in prepubertal castrated, ligated or cryptorchid rats, in order to assess the influences of blood-borne and luminal androgens. The principal cells showed partial differentiation: decrease in cell height, decreased numbers of cytoplasmic organelles implicated in the elaboration phenomena (Golgi apparatus, smooth endoplasmic reticulum), whereas the organelles implicated in the absorptive function remained relatively intact. The lamina densa of the basement membrane underlying the epithelium was irregular, thicker than normal and followed the irregular outline of the basal parts of the epithelial cells. These changes were evident in castrated rats, to a lesser degree in ligated and cryptorchid rats, and were more prominent in the initial part of the duct. On the other hand, the narrow cells and the clear cells followed a normal differentiation pattern in the experimental rats, suggesting that a differential androgen dependence exists among the various type of epididymal cells.

Androgens↗
PubMed1986

The physical, psychological, educational and professional conditions of young adults given growth hormone for childhood growth hormone deficit.

Twenty five of the 75 patients having been given human growth hormone in the Pediatric Nancy Endocrinological Division have reach final adult height. All have been treated the same way, 10 boys and 4 girls were diagnosed as isolated deficit, 7 boys and 4 girls as combined deficit. The physical, sexual, radiological, intellectual, professional and psychological characteristics have been defined either during the treatment follow-up or at a final interview. All results have been compared to the familial conditions if possible. Final adult statures are in the low range of the normal (-2 DS). The sexual development, normal for patients with isolated deficit, has not been achieved completely by regular protocol for patients with combined deficit. The intellectual and professional achievements are rather low but this has to be matched with below the normal familial conditions. Psychological determination is quite satisfactory but the personality is dominated by shyness and lack of responsibility. It is likely that an earlier onset of treatment and a better psychological guidance may lead to a better final results judged both on physical grounds but also on psychological and professional conditions.

Adolescent↗
PubMed1986

[Value of mexiletine in the treatment of Thomsen-Becker myotonia].

The case of a 1 month-old boy with a severe form of Thomsen-Becker myotonia is reported. Resistance to regular antimyotonic treatment over a 3 month-period of time led to use mexiletine, an antiarrhythmic drug. This treatment was responsible for a dramatic clinical and electromyographic improvement. No adverse effect has been observed over a 16 month-period of continuous treatment.

Drug Resistance↗
PubMed1985

[Normal growth].

Explore the source record for details and available documents.

Adolescent↗
PubMed1985

[Genetic counseling and the caudal regression syndrome].

It has been published about 500 cases of caudal regression (sacral agenesis) of which 12 are undoubtly familial. In most of the non familial cases an aetiology is not demonstrable except the cases related to maternal diabetes and/or insulin treatment. A genetic control of the caudal regression is implicit in the familial transmission. Three sporadic new cases are reported and, at the occasion of the genetic counselling we analyse the 8 well reported genealogies. Among 133 subjects, 72 show some evidences of caudal regression. This is compatible with a pattern of autosomic dominant transmission. The analogy with the caudal regression anomaly of the mouse, in which the role of genes located closely to the histocompatibility system is demonstrated, evokes such a relation in the human with the major histocompatibility system. If true, this may be used as a genetic marker, especially for early antenatal diagnosis.

Congenital Abnormalities↗
PubMed1984

Recurrence of symptomatic patent ductus arteriosus in extremely premature infants, treated with indomethacin.

The administration of a single intravenous injection of indomethacin was followed by a major constrictive effect on the ductus in 36 of 42 very-low-birth-weight (less than or equal to 1000 gm) infants with symptomatic patent ductus arteriosus (PDA). In 26 of the 36 responders, the effect was sustained; symptomatic PDA recurred in the remaining 10. Infants who experienced a recurrence of symptomatic PDA had lower birth weights and had received indomethacin at an earlier postnatal age than did infants with a sustained effect. These results may be explained by differences in the production and clearance of prostaglandins or in the sensitivity of the ductus to prostaglandin effects between infants with a recurrence and infants with sustained constriction of PDA.

Ductus Arteriosus, Patent↗
PubMedDate not supplied

Manifestations and treatment of Schimke immuno-osseous dysplasia: 14 new cases and a review of the literature.

UNLABELLED: Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive spondylo-epiphyseal dysplasia. The characteristic features of SIOD include 1) short stature with hyperpigmented macules and an unusual facies, 2) proteinuria with progressive renal failure, 3) lymphopenia with recurrent infections, and 4) cerebral ischaemia. Although 25 patients have been reported with this disorder, the clinical course and phenotype of SIOD are not well characterized. This report summarizes the clinical findings, course and treatment of reported patients and includes 14 additional patients with SIOD. We emphasize the high incidence of cerebral ischaemia and ocular abnormalities, define the high incidence of thyroid dysfunction and blood cytopenia, and confirm the absence of effective and durable medical therapies. CONCLUSION: Schimke immuno-osseous dysplasia is a multi-system autosomal recessive disorder with variable expression that affects the skeletal, renal, immune, vascular, and haematopoietic systems. Medical therapy is limited especially for more severely affected individuals.

Adolescent↗
PubMedDate not supplied

Meconium ileus and intestinal atresia in fetuses and neonates.

A collaborative study was performed to determine the different types and mechanisms of intestinal abnormalities during gestation. Cases had to fulfill one or more of the following three criteria: (1) meconium ileus, (2) intestinal stenosis or atresia, and (3) meconium peritonitis. Esophageal atresia, anorectal atresia, and abdominal wall defects were excluded. One hundred two cases were reviewed from the autopsies of 42 induced abortions, 22 stillborns, and the surgical findings in 38 neonates. Meconium ileus was detected mainly during the second trimester (28/38), and was associated with cystic fibrosis (15), fetal blood deglutition (4), infection (6), or multiple-abnormalities (10), in which three chromosomal aberrations were found. Intestinal stenosis or atresia was more commonly detected during the third trimester of gestation (46/56). Sixteen of the 30 duodenal malformations were associated with trisomy 21, whereas in the 26 small intestinal atresias, signs of distress or ischemia were most frequently detected. Only 8 of 25 meconium peritonitis cases were isolated. A total of 20 cystic fibrosis cases could be proved. In this series, functional abnormalities were observed predominantly in the second trimester and associated mainly with cystic fibrosis or amniotic fluid abnormalities. Anatomic lesions were commonly detected later on and associated with ischemic conditions, chromosomal aberrations, and even cystic fibrosis.

Constriction, Pathologic↗