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Biomedical subjects

P Barbet

Publications and source records attributed to P Barbet.

At least 19 recordsLinked to original sources
PubMed2006

Fetal lung growth in congenital diaphragmatic hernia.

BACKGROUND: In spite of significant therapeutic progress, the prognosis of congenital diaphragmatic hernia (CDH) remains pejorative in those forms in which the liver is herniated into the chest. The severity of this malformation relies on the pulmonary hypoplasia due to lung compression by the herniated viscera in the thoracic cavity, particularly the liver. This impaired growth concerns the whole pulmonary tissue, i.e. both the vessels and the alveoli. For the clinician, it is mandatory to know the evolution pattern of the lesions, to define the best time to treat them. AIM AND METHOD: The aim of this work was to study the pulmonary lesions along the gestation in fetuses affected byCDH. This morphological study was carried out on 134 human fetuses aged from 22 to 40 weeks of gestation. Anatomical and histological analysis focused on lung weight, alveolar count and wall thickness of the distal vessels. RESULTS: The results indicate that the pulmonary lesions worsen as the pregnancy continues, particularly beyond 30 weeks of gestation. CONCLUSION: Such an anatomical study should bring to the clinicians useful data to enhance the management of the patients.

Female↗
PubMed2004

Pelvic development in the rabbit embryo: implications in the organogenesis of bladder exstrophy.

Current theses of the development of bladder exstrophy and its variants rely on defective evolution of the urinary tract and cloacal membrane. They do not account satisfactorily for the clinical features reported in children affected by exstrophy, especially the pelvic bone anomaly. We herein describe the normal development of the pelvic ring in the rabbit embryo and its chronological relationship with the lower urinary tract organogenesis. Our results suggest that these events are intricated and allow us to propose a novel mechanism to explain exstrophies.

Animals↗
PubMed2004

Uncommon ureteric ectopias: embryological implications.

We herein report two rare ureteric ectopias, one in the uterus, the second in the rectum. These kinds of ectopias are not easily explained with regard to the classic embryological theories of the too-cranial or too-caudal origin of the ureteric bud on the mesonephric duct. We thus set out new explanations consistent with embryological studies on the narrow contact between the developing paramesonephric and mesonephric ducts, and on the so-called cloacal septation.

Choristoma↗
PubMed2003

Fetal bony pelvis in the bladder exstrophy complex: normal potential for growth?

OBJECTIVES: To provide the first look at the bony histologic features of fetuses with the exstrophy complex, specifically evaluating the endochondral ossification, stage of development, and microscopic potential for normal growth. METHODS: Three fetuses between 28 and 30 weeks of gestation, one with classic bladder exstrophy, one with cloacal exstrophy, and one control, were obtained from France. The bony pelves were dissected and preserved in formalin, and multiple representative sections were sliced from all pelvic areas: pubis, ischium, ilium, and sacrum. These slices were sequentially processed as slides, stained with hematoxylin-eosin, and evaluated microscopically for histologic changes, developmental stage, and degree of endochondral ossification. RESULTS: All slides from the three specimens showed cartilage analogue with endochondral ossification. Histologically the exstrophy specimens were identical to the control and appeared completely normal; bone development was occurring at an expected rate with the potential for continued normal growth. CONCLUSIONS: These new findings illustrate that fetal bone in the exstrophy complex displays normal microscopic growth patterns and unhindered endochondral ossification at 28 weeks of gestation, well beyond the embryologic period. With no evident microscopic bony defect, the gross bony anomalies in exstrophy should be surgically correctable, leading us to conclude that early reapproximation of the physiologic shape of the pelvis could lead to more normal gross bone growth, decreased shortage of bone, and a more appropriate distribution of the mechanical and developmental forces on a closed, normally functioning pelvic ring.

Abortion, Legal↗
PubMed2001

Cyclopia: a radiological and anatomical craniofacial post mortem study.

Cyclopia is a rare foetal malformation characterized by a single palpebral fissure and a proboscis associated with severe brain malformation. Approximately 1.05 in 100,000 births including stillbirths are identified as cyclopian. Cyclopia is not compatible with life. The authors present an anatomical and histological study of the fronto-orbito-maxillary region carried out after 3-D CT reconstruction in a 21 week-old foetus with cyclopia. Anatomical and histological observations suggest that the integrity of the trigeminal nerves is very important for the normal development of the embryological structures of the face. Fusion of the facial processes in the midline takes place even if central prosencephalic structures are absent. For this reason the face in cyclopia, in both its positive and negative aspects, constitutes a model for the study of the normal development of this region.

Brain↗
PubMed1998

Anatomic study of the umbilical vein and ductus venosus in human fetuses: ultrasound application in prenatal examination of left congenital diaphragmatic hernia.

For clinicians it is very difficult to evaluate the prognosis of a left congenital diaphragmatic hernia (CDH) at prenatal ultrasound examination. Surgical studies show that the presence of a large part of the liver in the chest is a criterion of poor prognosis. However, ultra-sonography encounters some difficulties in determining the precise position of the liver in the thoracic cavity. The aim of this anatomic study was to define the relationship between the position of the liver and the path of the ductus venosus and of the umbilical v., which are easily recognizable at prenatal sonography. Twenty dead fetuses were used for the study (12 with a left CDH and 8 without). All fetuses underwent radiographic assessment, anatomic dissection and cross-sectional study. The angle between the umbilical v. and the ductus venosus in different planes was measured. The more the liver was in the thorax, the greater was the angle between the ductus venosus and the sagittal plane, and the less the angle between the ductus venosus and the umbilical v. These angles can be easily measured by prenatal ultrasound examination of the fetus. Our findings suggest that it is now possible to offer the clinician a new and reliable way to determine the prognosis of a left CDH before birth.

Autopsy↗
PubMed1998

The 13carbon urea breath test for the noninvasive detection of Helicobacter pylori in children: comparison with culture and determination of minimum analysis requirements.

BACKGROUND: The purpose of the study was to determine the accuracy of the labelled 13carbon urea breath test for the diagnosis of Helicobacter pylori in children and to simplify the 13carbon urea breath test in identifying the most discriminating sampling time. METHODS: H. pylori was searched for in 100 children aged 10.5+/-4.5 years by histology, bacteriological counts, and culture on antral biopsies together with serology and 13carbon urea breath test. Breath samples were obtained before ingestion (T0) of 75 mg urea-13C and every 10 minutes after until T60. 13CO2 excess ratio was measured by isotope ratio mass spectrometry, and values expressed as delta per mil over baseline enrichment (delta 13CO2). The arithmetic mean (Mdelta 13CO2) of T20 to T60 values was calculated and the test considered positive with Mdelta 3CO2 higher than Mdelta 13CO2 + 3 SD as determined in noninfected children. RESULTS: Mdelta 13CO2 of noninfected children as assessed by culture was 1.4+/-0.6 per mil, determining a positive cut-off value of 3.44 per mil. Mdelta 13CO2 was correlated in 11 children with biopsy bacteriological counts. Both culture and 13carbon urea breath test were positive in 38 of 100 children, without any discordance. Plotting 13carbon urea breath test results at each sampling time versus Mdelta 13CO2 showed weaker correlations at T20, T30, T50, and T60, than at T40. The two-sample method at T0 and T30, T40, T50, had high sensitivity and specificity. Single-sample analysis obtained at T40 gave a comparable sensitivity and a slightly reduced specificity. CONCLUSION: 13carbon urea breath test is sensitive and specific in children. Two samples collected at T0 and T40 provide the most discriminating procedure.

Adolescent↗
PubMed1996

Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency.

A patient with cryptogenic multifocal ulcerous stenosing enteritis characterized by repeated bouts of intestinal obstruction, ulcerative stenosis of the small bowel relapsing after surgical resection, and steroid sensitivity is described. Fourteen strictures of the jejunum were found at laparotomy. Despite resection, abdominal pain persisted. Steroid therapy was effective but led to dependence. In our patient, cryptogenic multifocal ulcerous stenosing enteritis was associated with fever, asthma, Raynaud's phenomenon, sicca syndrome, heterozygous type I C2 deficiency (28-base pair gene deletion), stenosis, and aneurysms in selective mesenteric angiography. It is hypothesized that cryptogenic multifocal ulcerous stenosing enteritis might be related to a particular form of polyarteritis nodosa with mainly intestinal expression or to a yet unclassified independent vasculitis.

Adult↗
PubMed1996

Testicular dysgenesis does not affect expression of anti-müllerian hormone by Sertoli cells in premeiotic seminiferous tubules.

Anti-Müllerian hormone (AMH) immunoreactivity was studied on paraffin sections obtained from archival testicular biopsies of 29 children with intersex disorders and of 22 controls. Strong AMH immunoreactivity was observed in Sertoli cell cytoplasm from 8 fetal weeks until puberty. During pubertal maturation, in both normal and intersex patients, AMH expression was present in premeiotic seminiferous tubules, but was no longer detected in neighboring tubules with meiotic development. AMH immunostaining was abolished in the testis of one patient with persistent Müllerian ducts due to a mutation of the AMH gene, but was conserved in the testes of two patients with mutations of the AMH receptor gene. Testicular dysgenesis usually results in sexual ambiguity, with low testosterone and AMH serum levels and persistence of Müllerian derivatives. AMH immunoreactivity was conserved in premeiotic seminiferous tubules of dysgenetic testes, and also in sex-cord cells of a gonadoblastoma. In patients with asymmetric gonadal differentiation, the streak gonad was AMH-negative. In conclusion, secretion of AMH is a constitutive feature of the immature Sertoli cell and its expression is altered only by mutations of the AMH gene, but not by gonadal dysgenesis. The degree of regression of Müllerian ducts and serum AMH levels reflect the number, not the functional value, of Sertoli cells present in the immature testis.

Adolescent↗
PubMed1993

Anti-müllerian hormone and testosterone serum levels are inversely during normal and precocious pubertal development.

Anti-Müllerian hormone (AMH), also called Müllerian inhibiting substance or factor, is produced by Sertoli cells from fetal life until puberty. In the present study, AMH, testosterone (T), LH, and FSH were measured by immunochemical methods in the serum of 50 boys with normal or delayed pubertal development, 4 patients with suspected androgen insensitivity, and 11 patients with either central (CPP) or gonadotropin-independent (GIPP) precocious puberty to investigate the hormonal regulatory mechanisms of AMH secretion at puberty. An inverse relationship between AMH and T levels was demonstrated. In boys with normal or delayed puberty with T concentrations below 6.7 nmol/L, AMH values were elevated (mean +/- SEM, 22.4 +/- 3.1 micrograms/L) and widely dispersed. In subjects with T levels over 6.7 nmol/L, AMH levels were uniformly low (3.4 +/- 0.5 micrograms/L), except in patients with suspected androgen insensitivity. No significant relationship was found between AMH and gonadotropin levels. Similar results were obtained in patients with either CPP or GIPP. Longitudinal studies were performed on four boys with CPP and two with GIPP before and after treatment. At the time of diagnosis, the T concentration was high, and AMH levels were usually low in CPP and GIPP patients alike. When appropriate treatment was initiated, the T concentration was normalized within 2-4 weeks, but restoration of prepubertal AMH levels required several months. Mature Sertoli cells were observed in testicular biopsies performed in three patients with untreated GIPP. Our results suggest that gonadotropins are not directly implicated in repression of AMH synthesis at puberty, but, rather, that the decrease in AMH production is the consequence of an androgen-mediated, long term, reversible chain of events leading to morphological and functional maturation of the Sertoli cells. Thus, the fall in serum AMH levels appears to be an excellent marker of Sertoli cell pubertal development.

Adolescent↗
PubMed1993

Systemic lupus erythematosus and chronic intestinal pseudoobstruction.

We describe the first case of a patient with systemic lupus erythematosus in which clinical and radiological features, small bowel manometry and histological studies were consistent with the diagnosis of chronic intestinal pseudoobstruction. Our patient developed a fatal form of lupus with cerebral angiitis and tubulointerstitial lupus nephritis, with no sign of progressive systemic sclerosis or dermatopolymyositis. Postmortem examination confirmed the presence of an extensive fibrotic process in the muscularis layers, whereas the muscularis mucosae and the innervation of the digestive wall were spared.

Adult↗
PubMed1990

[Benign hypertrophic gastritis associated with cytomegalovirus infection].

A new case of benign hypertrophic gastritis associated with cytomegalovirus infection in a 7 year-old boy is reported. The main symptoms were complete digestive intolerance and protein loss, resulting in major hypoalbuminemia and anasarca. The alpha-1-antitrypsin clearance was very increased. Diagnosis was established by upper gastrointestinal x-rays which displayed important thickening of the gastric rugae, and by endoscopic examination with biopsy. A cytomegalovirus infection attested by seroconversion and viruria probably played a role in its origin. Symptomatic treatment and albumin infusions led to recovery within one month. In the last 25 years, 31 cases of benign hypertrophic gastritis have been reported. Vomiting is the main symptom with abdominal pain and G-I bleeding. The protein loss is constant, often severe. In most cases, the disease recedes within a few weeks, unlike the adults' Ménétrier disease which proves to be chronic and severe. The cause remains unknown. In 11 cases, a cytomegalovirus infection was reported and, so, is probably not fortuitous.

Child↗
PubMed1990

[Perinatal hemochromatosis].

Perinatal hemochromatosis (PHC) is a rare disorder presumably autosomal recessive, responsible for foetal death or severe liver failure during the neonatal period. It is fatal in nearly all cases. Diagnosis relies on histologic examination, generally post mortem, which shows numerous iron deposits in the liver as well as other organs (pancreas, heart, thyroid...). The study of iron and of its ligands may have a diagnostic interest. The authors report 2 cases of PHC in one family, of whom one concerned child presented with biological signs evoking PHC on foetal blood at 31 weeks of pregnancy.

Female↗