[Transition from childhood to adulthood and lumbosacral dysraphism].

The diagnosis of lumbosacral dysraphism is now made during the first year of life. Although problems due to filum lipomas are solved when the patient becomes an adult, the lipomas of the lumbar spinal cord can induce late complications. It is therefore necessary to organize a neurological, urological, and obstetrical follow-up to: screen for late retethering and/or deterioration ; preserve kidney function and monitor the consequences of the different procedures performed during childhood ; support pregnancies (delivery, prenatal screening for Currarino syndromes).

[Passage from childhood to adulthood and craniovertebral malformations].

Craniovertebral malformations are infrequent in children. Their causes are numerous (Chiari, congenital bone diseases, metabolic diseases, and genetic anomalies). When symptomatic (seldom), these malformations require surgical decompression and fixation.

[Characteristics of encrustation of ureteric stents in patients with urinary stones].

Introduction: The goal of this prospective study was to characterize ureteral stents encrustation in stone formers.

Material And Methods: We report the results of a study based on 658 double-J stents (412 men and 246 women) collected from patients with in situ urinary calculi. The mean age was 48.

Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders in 4 immunosuppressed children.

Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders are rare. We describe 4 cases in children: two with acquired immunodeficiencies (HIV infection, heart transplantation) and two with congenital immunodeficiencies (ataxia-telangiectasia and an undetermined disease affecting the T lymphocytes). Two of the lymphoproliferative disorders were T-cell types and two were B-cell types.

[Anhidrotic ectodermal dysplasia: "congenital ameibomia"].

Anhidrotic ectodermal dysplasia is a congenital, generally X-linked dermatosis that associates facial dysmorphy, short stature, and severe blepharitis. The anomalies of the skin are epidermic abnormalities; reduction of the glands of the derm, particularly the sweat glands, explaining the hypohidrosis; onychodysplasia; trichodysplasia; and abnormal dentition. The ophthalmologic manifestations are palpebral anomalies with a reduction in or an absence of Meibomian glands, dysfunction of the Moll and Zeis glands, leading to chronic squamous blepharitis and lacrimal punctal atresia.

Experimental ischemic cardiomyopathy: insights into remodeling, physiological adaptation, and humoral response.

Ligation of the left anterior descending coronary artery (LAD) is used to induce experimental myocardial infarction (MI). Most previous studies have focused on the early postoperative period, while data on mid-term follow-up are scanty. This study examined the mid-term effects of LAD ligation in 95 MI rats and 28 controls.

Prenatal diagnosis of some metabolic diseases using early amniotic fluid samples: report of a 15 years, experience.

Background: In the present study, we report the results of 132 prenatal diagnoses performed on chorionic villi and cell-free amniotic fluid obtained simultaneously at 12-13 weeks of gestation. In addition, we report the result of 59 prenatal diagnoses performed at 12-13th week using amniotic fluid only.

Methods And Results: A total of one fetal loss (1/191) was observed when a sample of amniotic fluid was obtained at around 12-13 weeks, whereas three losses (3/82) were observed after midtrimester amniocentesis.

Orthotopic heart transplantation for congenital heart defects: situs inversus.

Although rare, visceroatrial situs inversus is not exceptional in patients with complex congenital heart defects. Achieving orthotopic heart transplantation using a graft harvested in a donor with situs solitus is surgically demanding. Technical problems can, however, be overcome by adequate harvesting of donor heart and by the use of innovative reimplantation techniques.

Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return.

Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium.

Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series.

Background: A prospective study on childhood craniopharyngiomas (CPs) was conducted from 1994 to 1998 to appreciate the pre- and postoperative clinical, endocrine, mental, and intellectual status of the patients and to determine the incidence and severity of the postoperative hypothalamic syndrome.

Methods: The series included 14 consecutive CPs. Twelve were retrochiasmatic and intraventricular, and two were partly prechiasmatic and extraventricular.

[Hereditary cholestasis, an unusual etiology of pruritus in the infant].

Introduction: Pruritus in the infant is predominantly related to common dermatosis. General causes remain exceptional. We report two cases of pruritus in infants revealing anicteric cholestasis.

Association of a fetal quadrigeminal cyst with a possibly congenital thalamic astrocytoma.

Case Report: This article reports on the exceptional association of a fetal quadrigeminal malformative cyst with a possibly congenital pilocytic astrocytoma of the pulvinar. Both the cyst and the tumor required treatment due to their postnatal growth.

Discussion: Treatment modalities and difficulties are emphasized, as well as the difficulties of prenatal counseling.

Skin markers of occult spinal dysraphism in children: a review of 54 cases.

Objectives: To verify the diagnostic value of lumbosacral midline cutaneous lesions in asymptomatic children to detect occult spinal dysraphism (OSD) and to propose a practical approach for clinical investigations with respect to the type of cutaneous lesions observed.

Design: Retrospective study of 54 children referred to the Department of Pediatric Dermatology between 1990 and 1999 for congenital midline lumbosacral cutaneous lesions.

Setting: The private or institutional practices of participating dermatologists and pediatricians.

Conservative management of asymptomatic spinal lipomas of the conus.

Objective: The natural history of spinal lipomas of the conus (SLCs) has not been well studied. Because of disappointing long-term results with early surgical treatment of asymptomatic children with SLCs, we have followed a protocol of conservative management for these patients. The results are presented in this report.

[Contrast-enhanced ultrasonography: renal applications].

Color Doppler US of the urinary tract is still facing a few limitations including, for B-mode imaging, the detection of small lesions, and, for color Doppler, the detection of low flow. Ultrasound contrast agents (USCA) improve these two limitations and allow the development of new functional applications for renal blood flow quantification. This improvement results from an increased acoustic response obtained from the microbubbles, as well as from the development of pulse sequencing and signal processing that led to the concept of specific ultrasound sequences.