A novel dysfunctional LHX4 mutation with high phenotypical variability in patients with hypopituitarism.

CONTEXT: LHX4 is a LIM homeodomain transcription factor involved in pituitary ontogenesis. Only a few heterozygous LHX4 mutations have been reported to be responsible for congenital pituitary hormone deficiency. SUBJECTS AND METHODS: A total of 136 patients with congenital hypopituitarism associated with malformations of brain structures, pituitary stalk, or posterior pituitary gland was screened for LHX4 mutations. RESULTS: Three novel allelic variants that cause predicted changes in the protein sequence of LHX4 (2.3%) were found (p.Thr99fs, p.Thr90Met, and p.Gly370Ser). On the basis of functional studies, p.Thr99fs mutation was responsible for the patients' phenotype, whereas p.Thr90Met and p.Gly370Ser were likely polymorphisms. Patients bearing the heterozygous p.Thr99fs mutation had variable phenotypes: two brothers presented somato-lactotroph and thyrotroph deficiencies, with pituitary hypoplasia and poorly developed sella turcica; the youngest brother (propositus) also had corpus callosum hypoplasia and ectopic neurohypophysis; their father only had somatotroph deficiency and delayed puberty with pituitary hyperplasia. Functional studies showed that the mutation induced a complete loss of transcriptional activity on POU1F1 promoter and a lack of DNA binding. Cotransfection of p.Thr99fs mutant and wild-type LHX4 failed to evidence any dominant negative effect, suggesting a mechanism of haploinsufficiency. We also identified prolactin and GH promoters as potential target genes of LHX4 and found that the p.Thr99fs mutant was also unable to transactivate these promoters. CONCLUSIONS: The present report describes three new exonic LHX4 allelic variants with at least one being responsible for congenital hypopituitarism. It also extends the phenotypical heterogeneity associated with LHX4 mutations, which includes variable anterior pituitary hormone deficits, as well as pituitary and extrapituitary abnormalities.

[Oxytocin and maternal stress during the post-partum period]. / Ocytocine et stress de la mère au cours de la lactation en post-partum. L'ocytocine est-elle impliquée dans la confiance en soi de la mère allaitante? Revue de la littérature.

Oxytocin is required for lactation by promoting milk expulsion. Oxytocin has also been reported to exert a positive role in social attachment. The postpartum period has been shown to be crucial for maternal behavior initiation, and required self-trust reinforcement. However, this period is also remarkable for the high risk exposure of either psychic or physical stress. A negative impact on young mother is suspected, both in the short, medium or long term, which can even be deleterious for child-mother relationships. During lactation in female rats and sheep, oxytocin production has been proved to decrease stress-induced hormonal changes and later consequences. In human beings, only the first hour after breast-feeding seems to protect against physical or psychic stress. Oxytocin improves the stress-induced response by reducing the ACTH and cortisol secretion thus representing a potential therapeutic pathway in post-partum pathologies such as depression. Thus, this review of recent literature about oxytocin and stress during post-partum period, leads to the assumption that oxytocin, at the moment of installation of breastfeeding, acts not only on the physiological condition, but also on the psychic condition of the mother.

A randomized, double-blind comparison between parecoxib sodium and propacetamol for parenteral postoperative analgesia after inguinal hernia repair in adult patients.

The newly injectable cyclooxygenase-2 selective nonsteroidal antiinflammatory drug, parecoxib, has never been compared with propacetamol, a parenteral formulation of acetaminophen. In this prospective, randomized, double-blind, double-dummy study, we randomly assigned 182 patients scheduled for initial inguinal hernia repair under general anesthesia to receive a single injection of 40 mg parecoxib or 2 injections of 2 g propacetamol within the first 12 h after surgery. The study variables were morphine consumption, pain at rest and while coughing, and patient satisfaction throughout the first 12 h postoperatively. For statistical analysis, we used the Student's t-test, chi(2), and covariance analysis. Total morphine consumption did not differ between the two groups. Pain was less intense in the parecoxib group at rest (P = 0.035) but did not differ for pain while coughing. The incidence of side effects was similar. Significantly more patients in the parecoxib group rated their pain management as good or excellent (87% versus 70% in the propacetamol group, P = 0.001). Within the first 12 h after inguinal hernia repair in adult patients, a single injection of parecoxib 40 mg compares favorably with 2 injections of propacetamol 2 g.

[Pediatric activity in a rural hospital in Tokombéré (Cameroon). Description and qualitative approach during a one year period]. / Activité pédiatrique d'un hôpital rural en zone sahélienne à Tokombéré (Cameroun). Description et approche qualitative sur une période de 1 an.

AIM: To describe pediatric care in a North Cameroon rural hospital, the Health Promotion Centre of Tokombéré, and its coherence in a global project of development. POPULATION AND METHODS: This prospective and descriptive study concerned all the children admitted from October 1993 to October 1994. Analysis of data from a questionnaire has been conducted with EPI-INFO. RESULTS: Six hundred and thirty-one children were admitted. The average age was 3.9 years, the sex ratio was 1.45; the percentage of children coming from the Tokombéré health area was 75%. The predominant pathology involved infections, essentially respiratory and digestive. There were 13% of deaths, two out of three occurring before the 48th hour following admission: diarrhea, malaria and lung infections were the main causes of death. The laboratory tests contributed very little to therapeutic decisions, which were based more on symptomatology and its evolution, than on diagnostic certainties. Drug associations and therapeutic excess were within acceptable limits, but possibly improvable. For one-third of the diseases, simple care at home was possible, yet only performed in 50% of cases. CONCLUSION: This study points out some deficiencies in management of hospitalized children and suggests improvements without more cost: reorganization of the laboratory, emergency therapeutic protocols, and activities in the villages.

[Force-feeding of infants in North Cameroon. A harmful tradition-- preventive approach]. / Le gavage traditionnel des nourrissons au nord Cameroun. Nocivité d'une coutume--Approche préventive.

Repeated infant force-feeding is a widespread custom among mothers in the district of Tokombéré in North Cameroon. It is started in neonates and maintained until approximately one year of age, using mostly hot water. The authors describes the complications of this practice, the most severe one being aspiration pneumonia which can cause death. The traditional and cultural aspects of infant force-feeding in this area are discussed. The author believes that these aspects have to be taken into account in any preventive action directed towards the renunciation of this harmful practice.

[Traditional force-feeding of infants]. / Le gavage traditionnel des nourrissons.

PIP: Between July 1992 and June 1993, 896 cases of bronchopneumonia were seen at the hospital in rural Tokombere, Cameroon. 100 (11%) were hospitalized. The practice of gorging was responsible for bronchopneumonia in 28 of the hospitalized cases, all under 1 year old. Ten infants died. Interviews with 1000 young and old village women were conducted to examine the traditional practice of gorging infants. Mothers gorged their infants 3-8 times/day and 2-5 times/night, while being careful not to flood the airways. They gorged them with hot water mixed with butter, soda, or red millet water or hot water mixed at the bottom of a pot serving grilled millet. The mothers claimed to gorge their infants because tradition, ancestors, and God require it and their parents, mothers-in-law, or peers told them about it. They believed that hot water gorging promotes a long life, survival, adequate child growth, strength and intelligence, and fast walking; fights against illness and death; prevents drowning; and heals the umbilical stump. Other supposed powers of hot water gorging were facilitates sucking and digestion, complements lactation, prevents hunger, warms the entire body of the infant, kills pathogens, aids respiration, cures the cold, cleans the bronchia, calms the infant, and causes restful sleep. Some mothers even filled the infant's nostrils with hot water or held the infant's nose. Mothers know the risks of hot water gorging and have often experienced the death of one of their infants. Some continue to gorge their infants hospitalized for grave respiratory illness, even when they conceded explanations, advice, and treatments. Many women have abandoned this practice, however. One is now advising mothers to give small amounts of tepid water with a spoon or a goblet without touching the nose or forcing it on the infant. Health workers should try to avoid condemning hot water gorging because condemnation is not the best pedagogical approach.^ieng