[Adopted children born in foreign countries: management in main French pediatric services].
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Adoption↗
Biomedical subjects
F Huet
Publications and source records attributed to F Huet.
The acquisition of competence to respond to ecdysone in Drosophila is transcript specific.
The steroid hormone ecdysone induces a precise sequence of gene activity in Drosophila melanogaster salivary glands in late third larval instar larvae. The acquisition of competence for this response does not result from a single event or pathway but requires factors that accumulate throughout the instar. Individual transcripts become competent to respond at different times and their expression is differentially affected in ecd1, dor22 and BR-C mutants. The induction of early-late transcripts, originally assumed to necessarily follow early transcripts, is partially independent of early transcript activation. Attempts to inhibit the synthesis of regulatory proteins reveal transcript-specific superinduction effects. Furthermore these inhibitors lead to the induction of betaFTZ-F1 and E93 transcripts at levels normally found in prepupal glands. These studies reveal the complexity of the processes underlying the establishment of a hormonal response.
Animals↗
[Inhaled medications: the application of nebulization in France].
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Administration, Inhalation↗
[Precocious puberty in adopted children, a risk not to be forgotten].
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Adoption↗
'Fou rire prodromique' as the heralding symptom of lenticular infarction, caused by dissection of the internal carotid artery in a 12-year-old boy.
A 12-year-old, right-handed boy experienced a pathological fit of laughter before a sudden right hemiplegia. Magnetic resonance imaging showed a left basal ganglia infarction, induced by a left internal carotid dissection. Arteriography revealed an underlying fibromuscular dysplasia. This case study demonstrates that cerebral artery dissection can occur in children and that a basal ganglia infarction may be preceded by pathological laughter called 'fou rire prodromique'. The clinical and anatomical relationship of this paroxysmal event are discussed.
Carotid Artery Diseases↗
Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology.
Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.
Age of Onset↗
Protective effect of perindoprilat in the hypoxemia-induced renal dysfunction in the newborn rabbit.
The renal effects of acute hypoxemia and the ability of perindoprilat, a potent angiotensin-converting enzyme inhibitor, to prevent these effects were assessed in 31 anesthetized and mechanically ventilated newborn (5 to 8 d of age) rabbits. Renal blood flow (RBF) and GFR were determined by the clearances of para-aminohippuric acid and inulin, respectively. Each animal acted as its own control. In eight normoxemic rabbits (group 1), the i.v. infusion of saline did not change renal and hemodynamic functions. In eight additional rabbits, acute hypoxemia (PaO2= 40 mm Hg) induced a significant decrease in mean blood pressure (-8+/-2%), RBF (-36+/-3%), and GFR (-31+/-3%) and an increase in renal vascular resistance (+50+/-12%). A third group of newborn animals (n=7) was used to determine the renal effects of perindoprilat administration (20 microg/kg) under normoxemic conditions. RBF significantly increased (+15+/-2%) and renal vascular resistance significantly decreased (-15+/-3%), whereas GFR, mean blood pressure, and filtration fraction did not change significantly. In group 4 (n=7), perindoprilat infusion completely prevented the hypoxemia-induced alterations in GFR and renal vascular resistance and partially prevented the fall in RBF. These results demonstrate that angiotensin II modulates the renal immature microcirculation and that inhibition of its formation effectively prevents the hypoxemia-induced decrease in GFR.
Angiotensin-Converting Enzyme Inhibitors↗
Long-term results of GH therapy in GH-deficient children treated before 1 year of age.
OBJECTIVES: To evaluate the long-term effects of GH therapy in early diagnosed GH-deficient patients treated before 1 year of age. STUDY DESIGN: We studied all 59 patients (33 males) recorded by Association France-Hypophyse and treated with GH (0.50+/-0.15 IU/kg (S.D.) per week) before 1 year of age. Clinical presentation and growth parameters under GH treatment were analyzed. RESULTS: Neonatal manifestations of hypopituitarism were frequent: hypoglycemia (n=50), jaundice (n=25) and micropenis (n=17/33). Although birth length was moderately reduced (-0.9+/-1.4), growth retardation at diagnosis (5.8+/-3.8 months) was severe (-3.5+/-1.9 standard deviation scores (SDS)). Fifty patients (85%) had thyrotropin and/or corticotropin deficiency. After a mean duration of GH therapy of 8.0+/-3.6 years, change in height SDS was +3.11+/-2.06 S.D., exceeding 4 SDS in 19 patients. Only 9 patients (15%) did not reach a height of -2 S.D. for chronological age and 20 patients (34%) exceeded their target height. Pretreatment height SDS was independently associated with total catch-up growth. CONCLUSION: Conventional doses of GH allow normalization of height in patients with early GH deficiency and treatment.
Age Determination by Skeleton↗
Two cases of terminal deletion of chromosome 13: clinical features, conventional and molecular cytogenetic analysis.
We report the cases of two unrelated patients with psychomotor retardation and craniofacial abnormalities, in whom cytogenetic studies have revealed a terminal deletion of chromosome 13 confirmed by fluorescence in situ hybridization (FISH). This del(13)(q33.2) is the smallest terminal deletion of the 13q reported so far. Interestingly enough, the serum level of coagulation factors VII and X, whose genes are located in 13q34, were reduced in both patients. These cases illustrate the difficulties in identifying precisely chromosome deletions and demonstrate that FISH techniques allow to obtain a more precise correlation between clinical phenotype and cytogenetic abnormalities.
Child, Preschool↗
[Catch-up growth in growth hormone deficit children treated with GH since the first year of life].
Long-term effects of growth hormone (GH) treatment were studied in 59 GH deficient (GHD) children, whose treatment was started before the age of 1 year, during the period 1978-1992, under the supervision of the France-Hypophyse Association. Mean duration of treatment was 8 +/- 3.6 years. At the last evaluation, 20 patients (34%) had a height above normal mean for age, and 50 (85%) had a height above the second lower standard deviation (SD) for age. Final height prediction performed in 29 patients was greater than target size (-0.35 +/- 1.4 SD vs -0.56 +/- 0.79 SD). These results show that early treatment of GHD infants allows normal statural growth in the majority of the cases; in addition, they underline the importance of early diagnosis of GH deficiency.
Age Factors↗
A multicentre study comparing cervicography and cytology in the detection of cervical intraepithelial neoplasia.
OBJECTIVE: To evaluate in a multicentre setting the performance of cervicography compared with cytology for the detection of cervical intraepithelial neoplasia. DESIGN: Prospective comparative multicentre study. SETTING: Three hospitals with outpatient gynaecology clinics and three cancer screening clinics. PARTICIPANTS AND METHODS: Cervical cytology and cervicography were performed on 5724 women. If one or both tests showed an abnormality suggestive of at least a low grade squamous intraepithelial lesion, a colposcopy with directed biopsy was carried out. Cervicograms were evaluated by four experienced 'senior' assessors and by ten new 'junior' assessors. RESULTS: Results were fully analysed for 5192 women (91%). A cervical biopsy was carried out on 228 women and this confirmed a true positive lesion in 116 cases (incidence rate: 2.2%). Of these, 72 cases (62.1%) were detected by cervicography and 64 (55.2%) by cytology. This difference was not statistically significant (McNemar: P=0.475). Only 20 cases of CIN (17%) were concordantly detected by both tests. Senior assessors performed significantly better with a detection capacity of 80.6% compared to a detection capacity of 56.6% for the junior assessors (chi2 test: P=0.034). CONCLUSIONS: Cervicography must be considered as a complementary test to cytology. Overall detection of CIN is improved, but this is mainly due to the detection of more low grade lesions. The lower sensitivity and specificity in high grade lesions compared with cervical cytology is the main limitation of cervicography in screening for CIN. An important finding was that the performance of cervicography was highly dependent on the assessors' experience.
Adolescent↗
Vitamin A deficiency and nocturnal vision in teenagers with cystic fibrosis.
UNLABELLED: The aim of this study was to document plasma retinol status and nocturnal vision in ten eutrophic adolescents with cystic fibrosis (CF) receiving daily retinol supplementation. Plasma retinol, alpha and beta carotenes and retinol binding protein were measured in ten clinically stable CF patients (mean age: 14.3 years; Shwachman score: 80-100). Nocturnal vision evaluation was performed with a Beyne optometer. Plasma retinol (mean 0.42 +/- 0.16 mg/l), alpha carotene and beta carotene levels were below the lower limit of normal in all but one patient. Five out of ten patients with normal standard opthalmological examination presented a poor (n = 3 patients) or a pathological (n = 2) dark adaptation test. These two patients showed a dramatic increase in nocturnal vision after 1 year of adapted retinol supplementation. CONCLUSION: Low vitamin A levels occur frequently in clinically stable, eutrophic and retinol supplemented CF adolescents. Since vitamin A deficiency is associated with poor nocturnal vision and since this pattern can be reversed by adapted retinol supplementation, we recommend monitoring plasma vitamin A levels in CF patients and evaluation of dark adaptation in retinol deficient patients.
Adaptation, Physiological↗
Prevention of hypoxemia-induced renal dysfunction by perindoprilat in the rabbit.
The role of angiotensin II, a potent postglomerular vasoconstrictor, in the hypoxemia-induced renal changes is still controversial. The ability of perindoprilat, an angiotensin converting-enzyme inhibitor, to prevent the acute renal effects of hypoxemia was assessed in 22 anesthetized-ventilated rabbits. In 8 untreated rabbits, hypoxemia induced a significant drop in mean blood pressure (MBP) (-12 +/- 2%), glomerular filtration rate (GFR) (-16 +/- 3%) and renal blood flow (RBF) (-12 +/- 3%) with a concomittant increase in renal vascular resistance (RVR) (+18 +/- 5%) and urine flow rate (+33 +/- 14%), and without any changes in filtration fraction (FF) (-4 +/- 2%). This suggests the occurrence of glomerular vasoconstriction during the hypoxemic stress. In 7 normoxemic rabbits, intravenous perindoprilat (20 microg/kg) induced an increase in urine flow rate (+17 +/- 4%) and RBF (+17 +/- 4%), and a decrease in MBP (-6 +/- 1%), RVR (-14 +/- 3%) and FF (-11 +/- 2%) without a significant change in GFR. The drop in FF and the increase in RBF suggests preferential postglomerular vasodilatation. In 7 rabbits, perindoprilat prevented the occurence of the hypoxemia-induced changes in RBF and RVR without improving MBP. FF decreased significantly (-18 +/- 2%), while the drop in GFR (-7 +/- 2%) was partially blunted and the increase in urine flow rate (+25 +/- 9%) was confirmed. These results could be explained by the inhibition of the angiotensin-mediated efferent vasoconstriction and by the inhibition of bradykinin degradation by perindoprilat. These data confirm the ability of converting-enzyme inhibitors to prevent the renal hypoperfusion induced by acute hypoxemia.
Angiotensin II↗
Effects of cisapride on QTc interval in neonates.
AIM: Prospective survey of the effects of cisapride on QTc interval in neonates given cisapride. METHODS: QTc interval was determined just before and 2.9 (0.9) days after outset of the treatment in 49 neonates treated with cisapride between 1 August 1995 and 29 February 1996. RESULTS: Cisapride significantly increased QTc interval (p = 0.0001), and this was higher when birthweight or gestational age were lower. The prolongation of QTc interval above the arbitrary value of 0.450 (n = 7) was clinically asymptomatic and was significantly more common in the infants born with a gestational age < or = 33 weeks (n = 6). CONCLUSION: The findings indicate that cisapride accumulates in less mature neonates. Further pharmacokinetic studies are needed.
Cisapride↗
[Aerosol therapy and cystic fibrosis: a national survey].
Aerosol therapy in cystic fibrosis is indicated in order to administer active agents directly into the diseased organ. The practical application of this technique often remains a question of personal experience rather than rigorous and validated schemes. In order to determine the experience of those using this technique, a questionnaire was prepared by the health care centers certified by the French association against cystic fibrosis (65 centers). There were 53 responses (82%) covering 3400 patients. Two-thirds of these patients (2250) used nebulizers. Drugs used by order of frequency were: rhDNase (52 centers), antibiotics (51), conventional fluidifying agents (17), isotonic saline solution (14). Bronchodilators were used as sprays or inhalation chambers. Antibiotics most frequently prescribed were: colimycin (51 centers, doses 500.000 to 3 MIU per aerosol), tobramycin (44 centers, 25 to 600 mg per aerosol), amikacine (7 centers, 150 mg to 1.5 g per aerosol). Indications were mainly chronic colonization with Pseudomonas aeruginosa (39/53 centers) and episodes of bronchial superinfection with Pseudomonas aeruginosa (18/53 centers). Equipment maintenance appeared as the main problem in most centers (daily or more cleaning with antiseptic solutions in 46/53 centers). The differences in indications and dosages prescribed justify further prospective randomized studies to optimize treatment.
Administration, Inhalation↗
Transformation zone location and intraepithelial neoplasia of the cervix uteri.
We examined the relationship between the frequency of premalignant lesions of the cervix and location of the transformation zone on the cervix among 8758 women as assessed using cervicography. An endo- and exocervical smear test was performed at the same time. Women with smear test classified CIN I or more were recalled and any abnormal area was biopsied under colposcopy. The transformation zone was located on the exocervix in 94% of women younger than 25 years old; as age increased, the proportion of women with a transformation zone located on the exocervix steadily decreased to reach less than 2% after 64 years old. As compared with women having a transformation zone in the endocervical canal, the age-adjusted likelihood of discovering a histologically proven dysplastic lesion was 1.8 times more frequent among women with a transformation zone located on the exocervix (95% confidence interval 1.1-2.9). This higher frequency seemed not attributable to a lower sensitivity of the smear test when the transformation zone was hidden. The results also showed that deliveries tended significantly to maintain the transformation zone on the exocervix. Parity is a known risk factor for cervix cancer, but the mechanism by which it favours malignant lesions remain unknown. Our results suggest that with increasing numbers of livebirths, the transformation zone is directly exposed for longer periods to external agents involved in dysplastic lesions.
Adult↗
[Drugs used in pediatrics. Pediatric therapy: practical utilization of drugs].
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Age Factors↗
Effects of theophylline on renal insufficiency in neonates with respiratory distress syndrome.
We report here 6 cases of critically ill newborn infants with both RDS and acute renal insufficiency, unresponsive to conventional treatment (furosemide, dopamine). Theophylline, an adenosine antagonist, has been shown to prevent hypoxemia-induced renal insufficiency in rabbits and our patients thus received compassionately a low-dose of theophylline (1 mg . kg-1 i.v.). Urinary water excretion and creatinine clearances increased significantly in 5 out of 6 neonates, thus suggesting a beneficial role of theophylline in neonatal prerenal failure.
Acute Kidney Injury↗