Characterization of an in vivo hormonally regulated phosphodiesterase 3 (PDE3) associated with a liver Golgi-endosomal fraction.

The biochemical properties of an in vivo hormonally regulated low Km cAMP phosphodiesterase (PDE) activity associated with a liver Golgi-endosomal (GE) fraction have been characterized. DEAE-Sephacel chromatography of a GE fraction solubilized by a lysosomal extract resulted in the sequential elution of three peaks of activity (numbered I, II, and III), while ion-exchange HPLC resolved five peaks of activity (numbered 1, 2, 3, 4, and 5). Based on the sensitivity of the eluted activity to cGMP and selected phosphodiesterase inhibitors, two phosphodiesterase isoforms were resolved: a cGMP-stimulated and EHNA-inhibited PDE2, eluted in DEAE-Sephacel peak I and HPLC peak 2 and a cGMP-, a cilostamide-, and ICI 118233-inhibited PDE3, eluted in DEAE-Sephacel peak III and HPLC peaks 3, 4, and 5.

Postal survey of cuffed or uncuffed tracheal tubes used for paediatric tracheal intubation.

A postal survey of the use of cuffed or uncuffed tracheal tubes for tracheal intubation in children and infants was performed to investigate the criteria used for deciding the choice of tube and the manner of inflating the cuff in the case of use of a cuffed tracheal tube (CTT). From 200 questionnaires despatched, replies were received from 130 paediatric anaesthesiologists (response rate 65%). In paediatric practice, the CTT was routinely used by 25% of respondents for more than 80% of their patients, while more than 37% of respondents use them in less than 20% of the cases.

Fontan completion: intracardiac tunnel or extracardiac conduit?

The concept of Fontan circulation was first clinically introduced in 1971. Since that time, many technical modifications have been advocated. Among them, the concept of total cavopulmonary connection, introduced in the late 80's, has experienced widespread use over the past 10 years.

The contribution of prenatal diagnosis to the understanding of malformative intracranial cysts: state of the art.

This review evaluates the contribution of prenatal diagnosis to the understanding of intracranial cysts. We describe the outcome of 54 fetuses in which prenatal investigations indicated the presence of such lesions. The cysts were diagnosed between 20 and 30 weeks of gestation.

[Prevention of renal carcinoma: the nutri-genetic approach].

The development of renal cell carcinoma (RCC) has been associated with both genetic and environmental factors, with somatic and germline mutations in the von Hippel-Lindau (VHL) tumor suppressor gene and with tobacco smoking, obesity, long term exposure to some nutrients, pollutants, and industrial solvents such as trichloroethylene. Intra and interfamilial variability of expression of germline mutations in the VHL gene and variable susceptibility to carcinogens in the sporadic forms strongly suggest the involvement of conditional modifier genes. In order to identify sub groups of individuals at increased risk because of susceptibility genotypes, we have collected a series of 460 patients who developed an RCC and 79 families with the von Hippel Lindau disease.

[Genetic tests: predict or curse].

Genetic testing is aimed to the goal of i) confirming the diagnosis of a genetic disease in an affected individual and ii) of determining the status of relatives and the genetic risk to the progeny. Genetic testing also allows to determine whether an at risk individual is the carrier of the disease gene prior to symptoms. Genetic tests should be carried out for the benefit of the patients only.

Perioperative blood salvage during surgical correction of craniosynostosis in infants.

Surgical correction of craniosynostosis in infants is a very haemorrhagic procedure. The aim of this study was to determine whether the perioperative use of the continuous autotransfusion system (CATS) would reduce homologous transfusion during repair of craniosynostosis. Two groups of patients were studied according to the availability of the CATS in our hospital.

[Component analysis of urinary calculi in the etiologic diagnosis of urolithiasis in the child].

The potential risk of recurrence and degradation of renal function justifies the etiological investigation of all lithiasis-associated pathologies. Therefore calculus analysis of the crystalline phases and morphological characteristics is an important factor in the etiological diagnosis of the disease. Microscopic examination and infrared spectroscopy of calculi from 727 children showed that calcium oxalate was the main component in 36.

[Towards the integration of the digital medical image folder within the computerized patient folder: PACS and image networks].

Medical Images are components of the so-called "Medical Imaging Folder". This folder is a subset of the so-called "Medical Folder", part of the "Patient Folder. The G8 promotes the concept of a "Global Information Society for Health.

[Effect of a lung contusion on the prognosis of severe head injury in the child].

Objectives: To assess the effects of a pulmonary contusion (PC) on the outcome of a severe head trauma (SHT) in children less than 15-year-old.

Study Design: Retrospective study.

Patients: The study included 30 severely head injured children with a Glasgow Coma Scale score (GCS) < or = 8, associated with a PC (PC+) diagnosed on a thoracic CT-scan and 30 severely head injured children without PC (PC-).

[Scleroderma in children: a retrospective study of 70 cases].

Background: Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic significance, biological features, and co-morbidities and to assess the pertinence of therapeutic options.

Patients And Methods: The files of 70 children with primary scleroderma seen from 1980 to 1997 were retrospectively reviewed.

Intestinal intussusception survey about diagnostic and nonsurgical therapeutic procedures.

Objective: To provide an overview of the diagnostic and therapeutic procedures performed by European paediatric radiologists in the management of intussusception.

Materials And Methods: A postal survey was sent to the European members of ESPR. Items surveyed included diagnostic imaging procedures (plain films, US, contrast enema [CE]), contrast medium used (barium, iodine, air, saline solution), and imaging technique used for monitoring during reduction (films, fluoroscopy, US).

[Syringomyelia in children].

From 1985 to 1997, 442 children were treated for syringomyelia. One hundred and eighty eight had syringomyelia in association with Chiari I malformation. In 65% of the case scoliosis was the initial symptom.

Juvenile rheumatoid arthritis and lymphoedema: lymphangiographic aspects.

We report a 5 1/2-year-old boy with juvenile rheumatoid arthritis (JRA) and lower-limb lymphoedema. US, MRI and lymphangiography were performed. Based on the lymphangiographic study, we propose a pathogenesis based on obstruction of normal superficial lymphatic vessels in the affected limb.

Activation of a cGMP-stimulated cAMP phosphodiesterase by protein kinase C in a liver Golgi-endosomal fraction.

The ability of Ca2+/phospholipid-dependent protein kinase (protein kinase C, PKC) to stimulate cAMP phosphodiesterase (PDE) activity in a liver Golgi-endosomal (GE) fraction was examined in vivo and in a cell-free system. Injection into rats of 4 beta-phorbol 12-myristate 13-acetate, a known activator of PKC, caused a rapid and marked increase in PKC activity (+325% at 10 min) in the GE fraction, along with an increase in the abundance of the PKC alpha-isoform as seen on Western immunoblots. Concurrently, 4 beta-phorbol 12-myristate 13-acetate treatment caused a time-dependent increase in cAMP PDE activity in the GE fraction (96% at 30 min).

Diagnostic value of nasal provocation challenge with allergens in children.

A total of 106 children suffering from perennial rhinitis and/or asthma, and all allergic to Dermatophagoides pteronyssinus (DP), underwent nasal provocation challenge (NPC) with DP to determine the best method of diagnosis. Posterior rhinomanometry was uninterpretable in 17 patients and gave negative results in 31. Clinical scores for sneezing and rhinorrhea were more effective but did not diagnose the disorder in 11 children.

Spatio-temporal expression of FGFR 1, 2 and 3 genes during human embryo-fetal ossification.

Mutations in FGFR 1-3 genes account for various human craniosynostosis syndromes, while dwarfism syndromes have been ascribed exclusively to FGFR 3 mutations. However, the exact role of FGFR 1-3 genes in human skeletal development is not understood. Here we describe the expression pattern of FGFR 1-3 genes during human embryonic and fetal endochondral and membranous ossification.