[Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries].

Introduction: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants.

Methods: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries.

[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.

[EBV-associated cutaneous plasmocytoma in a renal transplant patient].

Introduction: Postransplant lymphoproliferative disorders are well known complications of solid organ transplant, usually associated with Epstein-Barr virus (EBV).

Observation: A 25 year old renal transplant patient presented with two subcutaneous nodules on the lower limb that appeared 3 years after a second renal transplantation. Biopsy of one nodule showed an EBV associated plasmocytoma located in the subcutaneous tissue.

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.

[Clinical value of crystalluria study].

Crystalluria is a marker of urine supersaturation present in both normal and pathological conditions. Indeed, nature and characteristics of the spontaneous crystalluria are of clinical interest for detecting and following biological disorders involved in renal diseases. Method.

Surgical reconstruction of occluded pulmonary arteries in patients with congenital heart disease: effects on pulmonary artery growth.

Background: This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease.

Methods And Results: Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively.

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.

Prenatal diagnosis of carnitine palmitoyltransferase 2 deficiency in chorionic villi: a novel approach.

Carnitine palmitoyltransferase 2 (CPT2) deficiency, the most common autosomal recessive inherited disease of the mitochondrial long-chain fatty acid (LCFA) beta-oxidation, may result in three distinct clinical phenotypes, namely, a mild adult muscular form, a severe infantile hepatocardiomuscular disease, and a neonatal form, which includes dysmorphic features in addition to hepatocardiomuscular symptoms. Both the latter forms are life-threatening diseases, and prenatal diagnosis (PND) can be offered to couples at a one-fourth risk of having an affected child. PND of CPT2 deficiency hitherto relied mostly on mutation detection from fresh chorionic villi (10 weeks' gestation), since CPT2 activity could be assayed on cultured amniocytes only (16-17 weeks' gestation).

Brain vascular malformations in the fetus: diagnosis and prognosis.

Unlabelled: DIAGNOSIS OF BRAIN VASCULAR MALFORMATIONS IN THE FETUS: Vein of Galen aneurysms can be diagnosed in utero. Other malformations are not seen. Ultrasound makes the diagnosis by showing a "cystic" structure in the posterior fossa.

Fetal CT.

Introduction: Fetal CT is helpful in the diagnosis of bony anomalies, particularly with 3D reconstructions.

Discussion: Because of the potential risks of irradiation, CT should not be performed before 32 weeks' gestation and should be restricted to a carefully selected group of patients.

Prognostic factors for the survival of patients with papillary renal cell carcinoma: meaning of histological typing and multifocality.

Purpose: We identified prognostic factors of papillary renal cell carcinoma (PRCC) types 1 (PRCC1) and 2 (PRCC2).

Materials And Methods: Between 1985 and 1998, 759 patients underwent surgery for renal cell carcinoma, of whom 88 (11.6%), including 69 males and 19 females with a mean age of 61.

Malformative intracranial cysts: diagnosis and outcome.

Introduction: Prenatal investigations make it possible to follow up malformative intracranial cysts from their detection in utero through the postnatal period. By including those that will remain silent postnatally, precious information can be provided about their real natural history.

Diagnosis: Contrary to common belief, the vast majority of these lesions, if not associated with other fetal anomalies, are benign in nature, remain clinically silent, do not evolve or even frequently regress spontaneously.

[Early complications of stenting in children and young adults with congenital heart disease].

We report the early complications of stent implantation. Since 1/1/97 to 1/12/02, we implanted 91 stents in 76 patients. Median age and weight were 15 years (range 0.

[Experience of implantation of prefitted Palmaz Corinthian stents].

We report our recent experience with the Palmaz Corinthian stent in children. Since 1/10/01 to 1/11/02 we implanted 18 Palmaz Corinthian stents in 12 children. Median age and weight of patients were 5.