Germinogenic tumors of gonadal and extragonadal sites in children

Literature data are presented on histogenesis, classification, morphology, tumor markers and prognosis of germinogenic childhood tumors of gonadal and extragonadal origin.     

CD40 is a prognostic marker in primary cutaneous malignant melanoma.

CD40 is a receptor at the surface of B lymphocytes with important functions in the immune response. CD40 has also been found on a variety of carcinoma and melanoma cell lines where it has been suggested to serve as a possible receptor for mitogenic signals. We studied the expression and distribution of CD40 in paraffin sections of 71 uniformly treated malignant melanomas (MMs) with a long clinical follow-up using well known monoclonal antibodies. For comparison, 71 benign nevi were also studied. Common acquired nevi occasionally expressed CD40 in nests or single cells at the dermo-epidermal junction; no immunoreactivity was observed in the dermal part of acquired nevi, and all Spitz' nevi were entirely negative. One-third of large congenital nevi expressed CD40 in small clusters of heavily pigmented, epithelioid cells, corresponding to so-called proliferative nodules. In 41 of 71 MMs, CD40 was expressed in single or clustered neoplastic melanocytes; 9 cases showed CD40 expression only in the radial growth phase, and in 32 cases, the vertical growth phase showed CD40 expression. The same staining pattern was obtained with other anti-CD40 monoclonal antibodies, directed to different epitopes of the CD40 molecule. In 29 of 32 MMs showing CD40 in the vertical growth phase, expression of the CD40 ligand (CD40L) was studied; in 13 of these 29, CD40L was found in the same tumor areas that expressed CD40. Analysis of 28 metastases from 24 MM patients showed in the majority of cases a similar, scattered or nodular staining pattern as observed in the primary tumor. Patients expressing CD40 in the vertical growth phase of their MM did not differ significantly from CD40-negative patients with respect to any of the known prognostic parameters but showed a significantly shorter tumor-free survival. Patients with CD40+ CD40L+ MM tended to have a shorter tumor-free survival than those lacking CD40L. We conclude that CD40 represents a novel prognostic parameter in primary cutaneous MM. The co-localization of CD40 and CD40L suggests an autocrine growth loop in the vertical growth phase of MM.     

Congenital hepatoblastoma with a rhabdomyoblastic component

An observation of congenital hepatoblastoma of a mixed type in a baby dying on the 50th day of life is described. The boy was born with a metastasis of the tumour into the left superciliary area. The process of hepatoblastoma metastasising had begun in the intrauterine period. The main and metastatic nodes of the tumour consisted of epithelial and mesenchymal components. The multicomponent structure of the tumour attests to its origin from a pluripotent blastoma like Wilms tumour. In addition to the hepatoblastoma with disseminated metastases the boy had malformations in the liver and kidneys. The combination of hepatoblastoma with congenital malformations appears to be due to the effect on the fetus of some harmful factors in the first trimester of pregnancy (toxicosis, influenza).     

Intimal hyperplasia after long-term venous catheterization

OBJECTIVE: Intimal hyperplasia is a well-known consequence of arterial injury and arterialization in vein grafts. However, the subacute and chronic vein wall changes which occur after catheterization have not been well studied. In this animal study, intimal hyperplasia in the vein wall after catheterization was examined. METHODS: A silicon catheter was placed in the anterior caval vein of 54 rats. After in situ fixation at scheduled intervals (1 day to 6 months), the pathologic changes in the vein wall were studied on semi-serial histology sections by means of light microscopy. RESULTS: Three forms of intimal hyperplasia could be observed: plaque-like, papillary-like and incorporation of the mural part of the sleeve into the underlying vein wall. Although the appearance of each was different, their composition was identical. All were mainly composed of alpha-actin-positive cells and collagen localized above the internal elastin layer, and covered by endothelium if facing the lumen. The plaque-like and papillary-like forms were mainly localized in the anterior vena cava, while sleeve incorporation mainly occurred in the jugular vein. Plaque-like and papillary-like intimal hyperplasia could be seen together on the same slide, but these two forms were never seen together with sleeve incorporation. CONCLUSION: Intimal hyperplasia occurs after venous catheterization and is probably caused by chronic injury to the vein wall due to knocking and rubbing movements of the catheter against the wall.     

Sequencing of the coding exons of the LRP1 and LDLR genes on individual DNA samples reveals novel mutations in both genes

Five coding polymorphisms in de LRP1 gene, i.e. A217V, A775P, D2080N, D2632E and G4379S were discovered by sequencing its 89 exons in three test-groups of 22 healthy individuals, 29 Alzheimer patients and 18 individuals with different clinical and molecularly uncharacterized lipid metabolism problems. No genetic defect was evident in the LRP1 gene of any of the Alzheimer's disease (AD) patients, further excluding LRP1 as a major genetic problem in AD. Lipoprotein receptor related protein (LRP) A217V (exon 6) was clearly present in all groups as a polymorphism, while D2632E was observed only once in a healthy volunteer. On the other hand, LRP1 alleles A775P, D2080N, and G4379 were encountered only in patients with FH or with undefined problems of lipid metabolism. This finding forced one to also analyze the LDL receptor (LDLR) gene, for which a method was devised to sequence the entire region comprising LDLR exons 2-18. The resulting sequence contig of 33567 nucleotides yielded finally an exact physical map that corrects published and listed LDLR gene maps in many positions. In addition, next to known mutations in LDLR that cause FH, four novel LDLR defects were defined, i.e. del e7-10, exon 9 mutation N407T, a 20 bp insertion in exon 4, and a double mutation C292W/K290R in exon 6. No evidence for pathology connected to the LRP1 'mutations' was obtained by subsequent screening for the five LRP1 variants in larger groups of 110 FH patients and 118 patients with molecularly undefined, clinical problems of cholesterol and/or lipid metabolism. In three individuals with a mutant LDLR gene a variant LRP1 allele was also present, but without direct, obvious clinical compound effects, indicating that the variant LRP1 alleles must, for the present, be considered polymorphisms.     

Electrospinning of polyhydroxyalkanoate fibrous scaffolds: effects on electrospinning parameters on structure and properties

In this study, electrospinning was used to prepare ultrafine fibers from PHAs with different chemical compositions: P(3HB) and copolymers: P(3HB-co-4HB), P(3HB-co-3HV), and P(3HB-co-3HHx). The main process parameters that influence ultrafine fiber diameter and properties (polymer concentration, solution feeding rate, working distance, and applied voltage) have been investigated and their effects evaluated. The study revealed electrospinning parameters for the production of high-quality ultrafine fibers and determined which parameters should be varied to tailor the properties of the products. This study is the first to compare biological and physical-mechanical parameters of PHAs with different chemical compositions as dependent upon the fractions of monomers constituting the polymers and ultrafine fiber orientation. Mechanical strength of aligned ultrafine fibers prepared from different PHAs is higher than that of randomly oriented ones; no significant effect of ultrafine fiber orientation on surface properties has been found. None of the fibrous scaffolds produced by electrospinning from PHAs had any adverse effects on attachment, growth, and viability of NIH 3T3 mouse fibroblast cells, and all of them were found to be suitable for tissue engineering applications.     

Water-Soluble Copper Ink for the Inkjet Fabrication of Flexible Electronic Components

The aim of this work is preparation and investigation of copper conductive paths by printing with a different type of functional ink. The solutions based on copper-containing complex compounds were used as inks instead of dispersions of metal nanoparticles. Thermal characteristics of synthesized precursors were studied by thermogravimetry in an argon atmosphere. Based on the comparison of decomposition temperature, the dimethylamine complex of copper formate was found to be more suitable precursor for the formation of copper layers. Structure and performance of this compound was studied in detail by X-ray diffraction, test of wettability, printing on flexible substrate, and electrical measurements.     

Stillbirths and neonatal deaths in appropriate, small and large birthweight for gestational age fetuses

AIMS: To compare the risk of stillbirth and neonatal death in small-for-gestational-age (SGA), appropriate-for-gestational-age (AGA) and large-for-gestational-age (LGA) fetuses and neonates. DESIGN: Retrospective analysis of 662 043 births and outcomes recorded in the Victorian Perinatal Data Collection Unit (1992-2002). INCLUSION CRITERIA: Births in Victoria in 1992-2002. EXCLUSION CRITERIA: Multiple pregnancy and congenital birth defects. MAIN OUTCOME MEASURES: Births, stillbirths and neonatal deaths at each week of gestation after 23 weeks were stratified by birthweight into appropriate, small and large for gestational age. Stillbirth risk per 1000 ongoing pregnancies and neonatal death rate per 1000 live births were calculated. RESULTS: For the AGA group, the overall stillbirth risk was 2.88 per 1000 and neonatal death rate was 1.35 per 1000. In the LGA group, these were 2.62 and 1.83 per 1000, respectively. The slight increase in neonatal death rate among LGA fetuses was confined to those delivered after 28 weeks gestation. In the SGA group, the stillbirth risk and neonatal death rate were 15.1 and 3.99 per 1000, respectively. CONCLUSION: The risk of stillbirth per week of gestational age and neonatal death rates do not differ significantly between AGA and LGA fetuses and neonates. The SGA fetus is at significantly greater risk of both stillbirth and neonatal death, particularly with advancing gestational age.