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Biomedical subjects

M Bost

Publications and source records attributed to M Bost.

At least 19 recordsLinked to original sources
PubMed2006

Bilateral anorchia in infancy: occurence of micropenis and the effect of testosterone treatment.

OBJECTIVE: To analyze the clinical and histological findings in boys with bilateral anorchia and the response to testosterone treatment on penis length. STUDY DESIGN: Patients were divided into two groups according to the absence (group A, n = 29) or the presence (group B, n = 26) of palpable intrascrotal or inguinal mass at first clinical examination. RESULTS: A micropenis was found in 46% of patients (n = 24) with a similar proportion in both groups. Testosterone treatment induced a mean penis length gain of 1.9 +/- 1.3 SDS (standard deviation score). However, micropenis persisted in six patients. Histological examination (n = 18) confirmed the absence of any testicular structure with deferent ducts being present unilaterally or bilaterally in all but three patients. In these three patients, a hemorrhagic testis, probably as a result of a mechanical torsion, was found. CONCLUSIONS: The presence of isolated micropenis in almost half of patients with bilateral anorchia strongly suggests that the testicular damage frequently occurs during the second half of gestation after male sexual differentiation. In most cases, testosterone treatment stimulates the penile growth. Although the pathogenesis of bilateral anorchia may be heterogeneous, our study suggests that gonads may have been functionally abnormal before they disappeared, and suggests that some patients have an intrinsic endocrine disorder.

Androgens↗
PubMed2005

Structural and functional abnormalities of large arteries in the Turner syndrome.

OBJECTIVE: To analyse the structural and functional abnormalities in the large arteries in women with the Turner syndrome. METHODS: Aortic stiffness (assessed by means of the carotid femoral pulse wave velocity), level of amplification of the carotid pressure wave (by applanation tonometry), and carotid remodelling (by high resolution ultrasound) were studied in women with the Turner syndrome. Clinical and ambulatory blood pressures were taken into account in the analysis. Thus, 24 patients with the Turner syndrome and 25 healthy female subjects matched for age were studied. RESULTS: Women with the Turner syndrome had a higher augmentation index than the controls (Turner, mean (SD) 0.04 (0.14) v controls, -0.14 (0.13), p < 0.001) but a lower peripheral pulse pressure (39 (8) mm Hg v 47 (11) mm Hg, p = 0.010 in the clinic; 44 (5) mm Hg v 47 (6) mm Hg, p = 0.036 during the 24 hour ambulatory recording). The luminal diameter of the common carotid artery and the carotid-femoral pulse wave velocity were similar in the two groups, whereas carotid intima-media thickness tended to be higher in women with the Turner syndrome (0.53 (0.06) mm v 0.50 (0.05) mm, p = 0.06). After correction for body surface area, carotid intima-media thickness and pulse wave velocity were higher in women with the Turner syndrome. CONCLUSIONS: Vascular abnormalities observed in the Turner syndrome are implicated in the origin of the cardiovascular complications that occur in this syndrome. These abnormalities are morphological but also functional. An increase in the augmentation index can be explained in part by the short height of these patients.

Adolescent↗
PubMed2004

[Environment and asthma in children: controversies].

The increase in asthma prevalence over the past 20 years could be due to modification of exposure to environmental factors (environmental theory) or to the lost of protective factors (hygienist theory). Among environmental factors this paper reviewed the controversial role of exposure to house dust mite (HDM). If exposure to HDM is deleterious in asthmatics known to be sensitised to this allergen, the effect of HDM exposure on asthma incidence has been challenged recently, based on longitudinal studies showing no correlation between level of exposure to HDM and asthma incidence. Exposure to animal dander may have protective effects. This review has shown that, even if some studies have shown potential protective effect of early exposure, the protection seems to be better in families with a low risk of atopy; almost all studies tend to show a deleterious effect of current exposure; exposure to dog dander may be more protective than cat. These studies cannot give definite conclusions to change current advices of early eviction of HDM and animal dander, especially in families with a high risk of atopy. The deleterious effect of passive smoking on asthma prevalence and severity is undisputable and our efforts should concentrate on obtaining effective eviction of passive smoking.

Air Pollution, Indoor↗
PubMed2002

[Acute organophosphate intoxication after using a anti-lice insecticide shampoo].

CASE REPORT: Two children were admitted for poisoning by organophosphate pesticides applied as hair rinses against lice. These chemical agents inhibit the acetylcholinesterase enzyme at various sites. The resultant accumulation of the transmitter acetylcholine causes abnormal signs and symptoms. The diagnosis is based on a reduction in the blood cholinesterase activity. The specific treatment comprises the administration of atropine and pralidoxime. CONCLUSION: Accidental poisoning by organophosphate insecticides may occur, due to the misuse of such substances as shampoo against lice. An accurate information for users is necessary.

Abdominal Pain↗
PubMed2002

[Differential diagnosis of asthma in infants and young children].

Although when an infant or a young child presents with persistent or recurrent wheezing, the probability of asthma is high, one must remain very careful in order not to miss an other etiology. Except for the chest X ray with inspiratory and expiratory studies that is mandatory, the other evaluations should be based on a careful interview and clinical examination. Fiberoptic bronchoscopy may be indicated each time there is a doubt on foreign body aspiration or tracheobronchomalacia.

Asthma↗
PubMed2002

[Prevention of respiratory syncytial virus infection by SYNAGIS (palivizumab)].

Infection with respiratory syncytial virus is frequent but most often benign. The serious forms of the illness, which make necessary hospitalisation or care in an intensive Care Unit, appear in infants of less than 6 weeks and especially in those with underlying pathologies, prematurity, congenital cardiopathies or chronic respiratory illnesses. Palivizumab (SYNAGIS) is mouse humanized monoclonal antibody which is used for prevention by monthly injections before and during the epidemic period. In a pivotal study performed on 1502 infants aged less than 6 months and former prematures of less than 36 weeks gestational age (GA) or aged less than 2 years and preventing a bronchopulmonary dysplasia, 1002 infants received 5 monthly injections, compared with 500 infants treated with placebo. There was a significant reduction of 55% risk of hospitalisation with VRS infections in the treated group, but no significant reduction in the number of stays in intensive care or deaths. The recommendation in France now is to use SYNAGIS in children aged less than 6 months, born with or GA of less then 32 weeks or aged less than 2 years and presenting a bronchopulmonary dysplasia. Questions remain on the cost-benefit ratio of this treatment and the favourable effects of this treatment in children who carry other chronic pulmonary or cardiac pathologies.

Animals↗
PubMed2001

[Chronic recurrent multifocal osteomyelitis in children: report of 17 cases].

UNLABELLED: Chronic recurrent multifocal osteitis (OCRM) is a rare condition in children, of unknown aetiology, which may be misdiagnosed as osteomyelitis, arthritis or tumour. PATIENTS AND METHODS: We present a retrospective multicentric study of 17 patients (five boys and 12 girls) with an average follow-up of 7.5 years (six months-25 years). RESULTS: A spectrum of presenting features is possible, ranging from bone lesions alone to lesions combined with arthritis, palmoplantar pustulosis or psoriasis. The diagnosis was delayed from two weeks to five years. Roentgenographic evaluation was often normal at the beginning of the disease or showed nonspecific bone reactions. Radioisotope bone scans assisted in establishing the diagnosis and in identifying lesions that were initially clinically silent. Bone biopsies were performed in seven cases. Histopathological examination showed only mild inflammatory nonspecific changes. Microbiological cultures were always negative. Treatments were different according to the evolution of the disease and the hospital. There was no response to antibiotics in seven patients. The response to nonsteroidal anti-inflammatory agents and steroids was moderate and often transient. Salazopyrine and pamidronate treatment used in two patients allowed a durable remission. We lost sight of four patients, pain persisted in three in spite of treatment, it disappeared in two with treatment, mild pain persisted in five without treatment and remission occurred in three without treatment. CONCLUSION: This study clarifies the clinical and radiologic features of chronic recurrent multifocal osteomyelitis. The recognition of this rare entity is often delayed and difficulties in patient management sometimes emerge from its usual protracted course.

Adolescent↗
PubMed2001

Formation of isoprostanes in children with type IIa hypercholesterolemia.

F2-isoprostanes are stable lipid peroxidation products of arachidonic acid and their quantification provides a novel approach to the assessment of oxidative stress in vivo. F2-isoprostanes are present in increased amounts in adult hypercholesterolemia, but no data exist concerning children. We investigated urinary isoprostaglandin F2, type III production as an index of lipid peroxidation in 15 children presenting with type IIa hypercholesterolemia (serum total cholesterol, 290 [SD +/- 70] mg/dl; low-density lipoprotein cholesterol, 210 [SD +/- 90] mg/dl) compared with 15 sex- and age-paired control children (serum total cholesterol, 160 [SD +/- 20] mg/dl). Urinary levels of isoprostaglandin F2alpha type III were measured by gas chromatography mass spectrometry. Urinary concentrations did not differ significantly in hypercholesterolemic children compared with control children (84.7 [SD +/- 37] vs. 96 [SD +/- 35] pmol/mmol creatinine, respectively). No significant correlation was found with total cholesterol, low-density-lipoprotein and high-density-lipoprotein cholesterol, and apolipoprotein B and A1 serum levels. F2-isoprostane urinary levels in children with type IIa hypercholesterolemia do not differ from those of age- and sex-matched control children and are not correlated to blood lipid parameters, suggesting that hypercholesterolemia is not associated with increased lipid peroxidation in childhood.

Adolescent↗
PubMed2001

Acromicric dysplasia: long term outcome and evidence of autosomal dominant inheritance.

Acromicric dysplasia is a rare bone dysplasia characterised by short stature, short hands and feet, normal intelligence, mild facial dysmorphism, and characteristic x ray abnormalities of the hands. Only a very small number of children with this condition have been reported so far. Here we report on a series of 22 patients including 10 boys and 12 girls with acromicric dysplasia. Length was normal at birth and height fell progressively off the centiles postnatally. The mean adult height was 130 cm (133 cm in males, 129 cm in females). The hands, feet, and limbs were short and OFC was normal. Intelligence was normal and mild dysmorphic features were noted. Other occasional features included well developed muscles, a hoarse voice, generalised joint limitation in some patients, frequent ear, tracheal, and respiratory complication, and spine abnormalities. Long term follow up showed that facial dysmorphism was less obvious in adults and that carpal tunnel syndrome was frequent in older patients. Apart from short metacarpals and phalanges, internal notch of the second metacarpal, external notch of the fifth metacarpal, and internal notch of the femoral heads, there were no major x ray abnormalities. No major complications, such as cardiac disease or major orthopaedic problems, occurred in the course of the disease. The condition appeared to be sporadic in 16 cases but the observation of vertical transmission in three families was consistent with an autosomal dominant mode of inheritance.

Abnormalities, Multiple↗
PubMed2000

Pleiotropic syndrome of dehydrated hereditary stomatocytosis, pseudohyperkalemia, and perinatal edema maps to 16q23-q24.

Dehydrated hereditary stomatocytosis (DHS) is a rare genetic disorder of red cell permeability to cations, leading to a well-compensated hemolytic anemia. DHS was shown previously to be associated in some families with a particular form of perinatal edema, which resolves in the weeks following birth and, in addition, with pseudohyperkalemia in one kindred. The latter condition was hitherto regarded as the separate entity, "familial pseudohyperkalemia." DHS and familial pseudohyperkalemia are thought to stem from the same gene, mapping to 16q23-q24. This study screened 8 French and 2 American families with DHS. DHS appeared to be part of a pleiotropic syndrome in some families: DHS + perinatal edema, DHS + pseudohyperkalemia, or DHS + perinatal edema + pseudohyperkalemia. If adequately attended to, the perinatal edema resolved spontaneously after birth. Logistic regression showed that increased mean corpuscular volume and mean corpuscular hemoglobin concentration were the parameters best related to DHS. In patients in whom cation fluxes were investigated, the temperature dependence of the monovalent cation leak exhibited comparable curves. Specific recombination events consistently suggested that the responsible gene lies between markers D16S402 and D16S3037 (16q23-q24). The 95% confidence limits (Z(max) >/= 3.02) spanned almost the complete 9-cM interval between these 2 markers.

Adolescent↗
PubMed2000

[Management of hemoptysis in children].

Hemoptysis in children are infrequent and often self-limiting. They are a manifestation of the broader spectrum of pulmonary haemorrhage. Diffuse pulmonary haemorrhages are often associated with diseases of other organs (cardiopathies, systemic diseases). Focal haemorrhages have multiple aetiologies, dominated by bronchopulmonary infections and cystic fibrosis. Fiberoptic bronchoscopy allows one to localise the bleeding, look for local causes and diagnose pulmonary hemosiderosis by BAL. For local lesions and if the medical management fails, bronchial arteriography is indicated to perform the embolisation of the bleeding vessels.

Age Factors↗
PubMed2000

Detection of human and animal rotavirus sequences in drinking water.

Reverse transcription-PCR analysis of drinking water in the homes of 56 children suffering from rotaviral gastroenteritis has shown the presence of the rotavirus genome in four samples. These strains were different from human rotaviruses detected in the children's feces, as determined by sequencing of the VP7-amplified fragments-three of them of animal origin (porcine or bovine) and one of human origin.

Amino Acid Sequence↗
PubMed2000

[Heart malformations and vascular complications associated with Turner's syndrome. Prospective study of 26 patients].

Turner's syndrome is associated with congenital heart disease in a third of cases. Several reports of aortic dilatation and of death by dissection or rupture of the aorta have been published. The authors undertook a prospective study to assess the incidence of cardiac malformations and aortic dilatation in genetically confirmed Turner's syndrome. Twenty-six out of 34 patients recalled (76%), aged 7 to 30 years (average 17 +/- 6 years) accepted their inclusion in this study and underwent clinical examination, ECG, chest X-ray and echocardiography. Thirteen patients had a monosomy 45X and 13 a mosaic or structural abnormality. Six had a history of cardiovascular disease (operated coarctation: 2 cases, kinking: 2 cases, Hypertension: 2 cases). Eight patients (30%) had one or several anatomical cardiovascular abnormalities: bicuspid aortic valve (19.2%), abnormalities of the aortic isthmus (kinking or coarctation) (15.4%), aortic regurgitation (7.7%), mitral stenosis (3.8%), partial anomalous venous drainage (3.8%), patent ductus arteriosus (3.8%) and left superior vena cava (11.5%). Systematic evaluation of the aorta resulted in the diagnosis of dilatation of the ascending aorta in 1 case and dilatation of the sinus of Valsalva in 2 other cases. The authors conclude that echocardiographic evaluation is essential after the diagnosis of Turner's syndrome. It should be repeated regularly to detect dilatation of the aorta which carries the risk of serious complications, such as rupture or dissection of the aorta.

Adolescent↗
PubMed1999

Myocardial infarction and Turner's syndrome.

This paper reports the case of a patient with Turner's syndrome, who developed a myocardial infarction at the age of 36. Turner's syndrome, associated with gonadal insufficiency, increases atherosclerotic cardiovascular risks that must be assessed during the patient's follow-up.

Adult↗