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Pediatric liver disease.

Pediatric hepatology has advanced greatly over the past few years. Many more children with severe liver disease are now surviving into adulthood. There is a number of conditions not seen in adult practice that have been the focus of research efforts, and many of these efforts have borne fruit in the past year. Disorders characterized by intrahepatic cholestasis in particular have now been substantially unravelled, and this work has elucidated a great deal about hepatic physiology. Liver-directed gene therapy is on the threshold of human application. This research has been facilitated by excellent animal models and the advent of isolated hepatocyte transplantation. Relatively common conditions such as biliary atresia, however, remain largely unexplained and the viral hepatitides have no effective form of treatment. It remains a hope that our increase in knowledge in the fields of genetics and immunology will translate into advances in therapy.

Journal Article↗

PET in pediatric diseases.

FDG-PET is being increasingly applied to pediatric conditions, particularly in oncology. PET and PET/CT scanning in children are not currently supported by Centers for Medicare and Medicaid Services unless the disease condition coincides with a reimbursed adult condition. The recent merger of the Children's Cancer Group and the Pediatric Oncology Group to form the Children's Oncology Group creates an opportunity to examine the use of FDG-PET in the management of childhood tumors in multi-institutional, cooperative efforts. The interest in incorporating PET imaging technology in pediatric medicine has been evidenced by several recent review articles summarizing the ongoing progress in this area. Future data will show that FDG-PET provides useful diagnostic information and can play a pivotal role in the clinical management and care of children with disease.

Child↗

Association between the biological characteristics of HIV-1, vertical transmission of infection and clinical progression of pediatric disease.

The design of effective prophylactic measures to prevent the vertical transmission of HIV-1 and of therapies to alter the natural progression of pediatric HIV disease requires a thorough understanding of basic pathogenetic principles. Maternal viral load, the biological behavior of HIV, such as replicative capacity in different types of cells, monocyte/macrophage tropism, and the capacity of the infant's cells to support infection have all been assessed for their contribution to the risk of mother-to-child transmission. Similarly, the effects of viral load and phenotype (e.g. replicative capacity, cell tropism, syncytium-inducing capacity and the use of chemokine co-receptors) have all been investigated as parameters associated with variations in the expression of clinical disease in children. Some of the extant data are conflicting, but general principles regarding pathogenesis are beginning to emerge.

Journal Article↗

[Disorders in sleep organization in pediatric diseases].

Interactions between sleep and general pathology in pediatrics are numerous and little known. Sleep organization evolves with age and stages of development. The most important physiological sleep changes occur during the first year of life. The main symptom of sleep disorders is excessive daytime sleepiness associated with dyssomnia, behavioral and learning disorders. Sleep disorders in childhood are often difficult to evidence, sleep investigations being still heavy and expensive.

Age Factors↗

Pathologic significance of nephromegaly in pediatric disease.

Ultrasonic renal volume and length measurements are easily performed in pediatric patients. Increases in volume over the normal level provide a potentially attractive method of assessing the severity and progression or regression of renal diseases. However, an analysis of data on 59 children with clinical renal disease and nephromegaly did not demonstrate any correlation with severity of the disease. We conclude that increases in renal length and volume in pediatric patients may indicate the presence of disease but not its severity.

Adolescent↗

Autologous versus allogeneic BMT in AML: the European experience. Report of the EBMT--Pediatric Diseases Working Party.

The Austrian-German-Italian (AGI) Pediatric Bone Marrow Transplantation Registry includes now data of 520 patients grafted for acute myeloid leukemia (AML) by autologous and allogeneic stem cells. In first complete remission (CR1) 145 patients with allografts had a possibility of event free survival (pEFS) of 0.57 and 140 patients with autografts a pEFS of 0.46. In second complete remission (CR2) autografted patients (n = 70) had a pEFS of 0.36 and allogeneic patients (n = 41) of 0.34. Patients with no CR went worse (allogeneic pEFS 0.15 n = 37; autologous pEFS 0.17 n = 6). Therefore, from the European data the following conclusions can be drawn: neither in CR1 nor in CR2 any statistical advantage for a particular transplantation type can be found. To assess the value of transplantation with family mismatch donors or matched unrelated donors (MUD) basing on these data pool is not possible. The following decisions should be reached: 1) BMT for AML with mismatched or unrelated donors should be done only within cooperative European trials. 2) Generally accepted definitions of indications for BMT in AML are needed. 3) Study protocols concerning conditioning are necessary.

Bone Marrow Transplantation↗

[A working classification and nomenclature of rheumatic diseases (pediatric aspects)].

The authors provide the working classification of rheumatic diseases prepared by a large group of scientists under the aegis of the All-Union Society of Rheumatologists and under the guidance of V. A. Nasonova, Academician of the USSR AMS. In preparing the final variant of the classification use was made of the experience gained by therapists in cooperation with pediatricians.

Adolescent↗