Not all pediatric intestinal polyps are alike.

BACKGROUND/AIMS: In childhood, clinical presentation of intes- tinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identify exceptions. Rare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. METHODS: Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient files were checked for clinical manifestations and histo- pathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. RESULTS: Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. CONCLUSION: Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies.

Expression of Id proteins increases in astrocytes in the hippocampus of epileptic rats.

Reactive gliosis is a prominent morphological feature of temporal lobe epilepsy. The molecular mechanisms underlying glial cell activation remain unclear. We examined expression of Id1-3 protein, a family of helix--loop--helix proteins involved in the regulation of cell proliferation and differentiation, in glial cells after electrically induced status epilepticus (SE) in the rat. In control hippocampus, Id3 was weakly expressed in astrocytes, while Id1-2 were below detection level. After SE, Id1-3 protein expression increased markedly in reactive astrocytes within 1 day and this persisted up to 3 weeks after SE. Three months after SE when rats experience spontaneous seizures, Id expression had returned to control levels. These results support a role of the Id gene family in regulating astrocyte reactivity in epileptic tissue.

GOA, a novel gene encoding a ring finger B-box coiled-coil protein, is overexpressed in astrocytoma.

Serial analysis of gene expression (SAGE) was used to identify a gene named GOA (gene overexpressed in astrocytoma), which codes for a novel Ring finger B-box coiled-coil (RBCC) protein. Northern blot hybridization showed overexpression of GOA in 9 of 10 astrocytomas. Except for kidney, in which high expression was found, expression levels in normal tissues were low and comparable to normal brain. Immunohistochemistry demonstrated presence of GOA, with prominent nuclear staining, in astrocytoma tumor cells and astrocytes of fetal brain, but virtual absence in mature astrocytes. Overexpression was not due to amplification, since amplification of GOA was only found in one of 65 astrocytomas. GOA was localized to 17q24-25, a region that is frequently gained or amplified in a number of other tumor types. GOA contains two LXXLL motifs, which are thought to be important for nuclear receptor binding. Our data suggest an important role of GOA in the process of dedifferentiation that is associated with astrocytoma tumorigenesis and possibly with that of other tumor types as well.

Postexercise protein-carbohydrate and carbohydrate supplements increase muscle glycogen in men and women.

We have previously demonstrated that women did not increase intramuscular glycogen in response to an increased percent of dietary carbohydrate (CHO) (from 60 to 75% of energy intake) (M. A. Tarnopolsky, S. A. Atkinson, S. M. Phillips, and J. D. MacDougall. J. Appl. Physiol. 78: 1360-1368, 1995). CHO and CHO-protein (Pro) supplementation postexercise can potentiate glycogen resynthesis compared with placebo (K. M. Zawadzki, B. B. Yaspelkis, and J. L. Ivy. J. Appl. Physiol. 72: 1854-1859, 1992). We studied the effect of isoenergetic CHO and CHO-Pro-Fat supplements on muscle glycogen resynthesis in the first 4 h after endurance exercise (90 min at 65% peak O2 consumption) in trained endurance athletes (men, n = 8; women, tested in midfollicular phase, n = 8). Each subject completed three sequential trials separated by 3 wk; a supplement was provided immediately and 1-h postexercise: 1) CHO (0.75 g/kg) + Pro (0.1 g/kg) + Fat (0.02 g/kg), 2) CHO (1 g/kg), and 3) placebo (Pl; artificial sweetener). Subjects were given prepackaged, isoenergetic, isonitrogenous diets, individualized to their habitual diet, for the day before and during the exercise trial. During exercise, women oxidized more lipid than did men (P < 0.05). Both of the supplement trials resulted in greater postexercise glucose and insulin compared with Pl (P < 0.01), with no gender differences. Similarly, both of these trials resulted in increased glycogen resynthesis (37.2 vs. 24. 6 mmol . kg dry muscle-1 . h-1, CHO vs. CHO-Pro-Fat, respectively) compared with Pl (7.5 mmol . kg dry muscle-1 . h-1; P < 0.001) with no gender differences. We conclude that postexercise CHO and CHO-Pro-Fat nutritional supplements can increase glycogen resynthesis to a greater extent than Pl for both men and women.

CD3 components at the surface of pro-T cells can mediate pre-T cell development in vivo.

Developmentally arrested pro-T cells (CD4-8-, IL-2R+, HSA++) of RAG-1-deficient mice appear to express low levels of CD3 molecules in the absence of T cell receptor (TcR) chains at their surface, while developmentally arrested pre-T cells of TcR alpha-deficient mice express low levels of a disulfide-linked TcR beta chain in association with CD3 molecules. Cross-linking of the CD3 modules on pro-T cells of RAG-1-/- mice in vivo, with either of two different CD3 epsilon-specific monoclonal antibodies, induces differentiation of these pro-T cells into pre-T cells (CD4+8+, IL-2R-, HSA+), concomitant with a rapid expansion of the thymic T cell compartment, up to 175-fold within 12 days. The same effects can be produced by introduction of a mutant TcR beta transgene lacking most of the variable domain (delta V-TcR beta) into the RAG-1-/- background. These experiments suggest that cross-linking of the CD3 modules on pro-T cells mimics the signaling function expected of the pre-TcR complex, which is found at the surface of pre-T cells prior to functional TcR alpha gene rearrangement. The variable domain of the TcR beta chain is apparently not essential for inducing these aspects of T cell development.

Study of intracellular deposition of the anti-leprosy drug clofazimine in mouse spleen using laser microprobe mass analysis.

Laser microprobe mass analysis (LAMMA) was used to study the composition of the brick-red crystalline material which had accumulated in the spleen of mice that had received the anti-leprosy drug Clofazimine in their diet for several months. The crystalline deposits light-microscopically resembled pure Clofazimine crystals. The presence of the drug in the crystals was indicated by LAMMA by the appearance of the chloride mass peaks in the negative mass spectra. More specific information was obtained from the positive mass spectra. A mass signal for the protonated molecule was present.

Laser microprobe mass analysis (LAMMA) of aluminum and lead in fine roots and their ectomycorrhizal mantles of Norway spruce (Picea abies (L.) Karst.).

Fine roots and ectomycorrhizal root tips were sampled in a Norway spruce (Picea abies (L.) Karst.) stand in the eastern part of the Belgian Ardennes. The cellular and partly subcellular localizations of aluminum and lead were identified by the micro-analytical laser microprobe mass analysis (LAMMA) technique. In fine roots with secondary structure, localization of aluminum was limited to the peripheral cell layers. Lead was found in the outer layers, and also in the primary phloem. Aluminum penetrated the mycorrhizal mantle, but lead was seldom detected in ectomycorrhizae.

Phosphorus, calcium and lead distribution in collagen in lead induced soft tissue calcification. An ultrastructural and X-ray microanalytical study.

Repeated intraperitoneal injections of lead acetate in rats caused a calcification of the skin of the abdomen near the site of the injections. In the lead-induced calcifications, electron dense collagen bundles could be observed. On the surface of the collagen fibrils, needle-like crystals were visible. With energy-dispersive X-ray analysis, phosphorus, calcium and lead were detected in the electron dense collagen bundles. X-ray maps of the P-K alpha, Ca-K alpha, and Pb-L alpha plus Pb-L beta lines showed an equivalent distribution along the collagen fibrils for phosphorus and calcium. The occurrence of the most electron dense areas in the STEM-image was comparable to the lead distribution. A good correlation existed between the structural and the elemental images of the same area. Although the medicinal use of preparations containing lead is no longer recommended, some are still prescribed. From our results we can conclude that they should not be applied to injured or inflamed skin.

Influence of fixation procedures on the microanalysis of lead-induced intranuclear inclusions in rat kidney.

Using Laser Microprobe Mass Analysis (LAMMA), we studied the chemical composition of lead-induced intranuclear inclusions in rat kidney tissue prepared by three different wet chemical fixation procedures for transmission electron microscopy. Fixation with glutaraldehyde-Na2S gave the same results as fixation with glutaraldehyde only: a high lead concentration could be detected. Therefore, for lead strongly bound to proteins, precipitation procedures are not essential. Post-fixation with osmium tetroxide drastically changed the composition of the inclusions: the lead concentration decreased substantially, while sodium, calcium, and barium were introduced. The osmium tetroxide fixative was found to be the source of the contamination. It also contained aluminum, and we suggest that other proteins (e.g., in neurofibrillary tangles) might be able to take up Al out of solution and that care must be exercised in interpreting the microanalytical results of osmium-fixed material. For the microanalysis of the lead inclusions, fixation with glutaraldehyde only provides a good compromise between preservation of the ultrastructure and maintenance of the element distribution.

Characterization of the spheroliths present in primary atypical bandkeratopathy using laser microprobe mass analysis.

Laser microprobe mass analysis was applied to study the chemical composition of spheroliths in the Bowman's membrane of patients suffering from primary atypical bandkeratopathy. The inclusions appear to consist mainly of calcium phosphate.

Ultrastructural localization of aluminium in liver of aluminium maltol-treated rabbits by laser microprobe mass analysis.

By means of laser microprobe mass analysis (LAMMA), we have studied the ultrastructural localization of aluminium in livers of aluminium maltol-treated rabbits. This animal model was developed to study long-term aluminium toxicity using systemic (intravenous) administration of aluminium. We could only detect aluminium in electron-dense inclusion bodies found in large, sometimes multinucleated cells. These results prove that the actual observation of aluminium deposits in liver with LAMMA gives more information than bulk analysis and can be very useful to explore mechanisms of toxicity.

Localization of lead and fluoride in cultured tooth germs by laser microprobe mass analysis.

Trace elements can influence dental health, possibly by altering tooth resistance during preeruptive development. Therefore, it was investigated whether lead and fluoride would be incorporated into the calcifying matrices or the cellular parts of tooth germs in vitro. Using laser microprobe mass analysis, the localization of lead and fluoride was studied in the different layers or tooth germs that had been cultured in a medium to which PbCl2 of NaF had been added in different concentrations. Both elements could only be detected in the dentine layer. Hence, the enamel organ in the secretory stage of tooth development excludes lead and fluoride from the enamel, even when enamel formation by the ameloblasts is visibly disturbed. Furthermore, there seemed to be a process of saturation in the accumulation of lead and fluoride in the dentine.