Ultrasound of the paediatric urogenital tract

Pathology in the urinary tract is one of the most frequent queries when children are referred for an ultrasound examination. Comprehensive ultrasound examinations can answer most clinical questions of the urogenital tract with minimal patient preparation and without the use of ionising radiation. Therefore, optimised imaging protocols should be available in all radiology departments where children are examined. This review suggests a preferred imaging protocol for urogenital imaging in children and gives an overview of the different structures of the urogenital tract, the normal age-related sonographic anatomy, and gives examples of the most commonly encountered diseases of the urogenital system in children.     

Pharmacological regulators of autophagy and their link with modulators of lupus disease

Autophagy is a central regulator of cell survival. It displays both anti- and pro-death roles that are decisive in the maintenance of cell homeostasis. Initially described in several eukaryotic cellular models as being induced under nutrient stress favouring survival by energy supply, autophagy was found later to display other decisive physiological roles, especially in the immune system. Thus, it is involved in antigen presentation and lymphocyte differentiation as well as in the balance regulating survival/death and activation of lymphocytes. Autophagy therefore appears to be central in the regulation of inflammation. The observation that autophagy is deregulated in systemic lupus erythematosus is recent. This discovery revives the programme dealing with the design and development of pharmacological autophagy regulators in the therapeutic context of lupus, a debilitating autoimmune disease that affects several million people in the world. A large number of molecules that positively and negatively regulate autophagy have been described, most of them with therapeutic indications in cancer and infection. Only a few, however, are effectively potent activators or inhibitors endowed with experimentally demonstrated selective properties. In this review article, we highlight the most relevant ones and summarize what we know regarding their mechanism of action. We emphasize the link between pharmacological regulators of autophagy and inducers or inhibitors of lupus disease and discuss the fundamental and pharmacological/therapeutic interest of this functional interplay.     

Developmental defects in zebrafish for classification of EGF pathway inhibitors

One of the major challenges when testing drug candidates targeted at a specific pathway in whole animals is the discrimination between specific effects and unwanted, off-target effects. Here we used the zebrafish to define several developmental defects caused by impairment of Egf signaling, a major pathway of interest in tumor biology. We inactivated Egf signaling by genetically blocking Egf expression or using specific inhibitors of the Egf receptor function. We show that the combined occurrence of defects in cartilage formation, disturbance of blood flow in the trunk and a decrease of myelin basic protein expression represent good indicators for impairment of Egf signaling. Finally, we present a classification of known tyrosine kinase inhibitors according to their specificity for the Egf pathway.     

Autoimmune neutropenia in children: analysis of 116 cases

To cite this article: Audrain M, Martin J, Fromont P, Prié N, Thomas C, Muller J‐Y. Autoimmune neutropenia in children: analysis of 116 cases. Pediatr Allergy Immunol 2011; 22 : 494–496. Diagnosis of autoimmune neutropenia (AIN) in infants is important, because it allows the exclusion of more severe forms of neutropenia that have an increased risk for leukemia. AIN is characterized by chronic neutropenia, which spontaneously resolves within several months to a few years, and mild infections. Diagnosis is confirmed by the presence of antibodies directed against neutrophil antigens. The human neutrophil antigen (HNA) system is a polymorphic system, which includes five antigen groups with different polymorphisms. In AIN, antibodies are mostly directed against HNA‐1 (or against a specific allele of HNA‐1) and HNA‐4. Here, we present a series of 116 infants with AIN. We observed that anti‐neutrophil antibodies were present in 60% cases; directed against HNA‐1a in 73% of cases. In addition, we showed there was a bias in the HNA allele distribution in these infants because the frequency of the HNA‐1a allele was greater in comparison with controls.     

Guide to the Development of an Industrial Medical Records System

Biological parameters known to be affected in lead poisoning were measured in rats following ingestion of graded dosages of lead. Intranuclear inclusion bodies are formed in renal tubular lining cells with smaller doses of lead than produce other changes. Decreased body weight is the next most sensitive abnormality. This is followed by increased delta-aminolevulinic acid (ALA) excretion, reticulocytosis, renal edema, and aminoaciduria. Anemia only occurs at the highest lead dosage. Over a wide range of lead ingestion, urinary lead excretion remains constant, although renal lead content increases. Quantitative lead analyses of cell organelles show that lead is concentrated within the inclusion bodies. Relatively small amounts of lead are present in the cytoplasm and mitochondria. It is suggested that soft-tissue lead accumulates in the intranuclear inclusion body, thereby sparing toxic injury to cytoplasmic organelles.