Lhermitte-Duclos Disease in Pediatric Population: Report of 2 Cases.

INTRODUCTION: Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is an uncommon disorder in children, characterized by being a slow-growing lesion of the posterior fossa, which mainly affects the granular cell layer of the cerebellar parenchyma and may be associated with other multiple hereditary hamartomas and neoplasms. CASE PRESENTATION: We report 2 cases of LDD in pediatric patients and describe clinical symptoms and radiological and histopathological characteristics. In addition, we analyzed the relation to Cowden Syndrome based on the International Cowden Syndrome Consortium Operational Criteria and the most updated guidelines by the National Comprehensive Cancer Network (NCCN Guidelines Version 1.2020). CONCLUSION: LDD is a very rare disease in childhood but should be considered in the differential diagnosis of posterior fossa lesions. LDD can mimic low-grade glial tumors or infectious diseases. Patients develop late clinical manifestations due to the slow-growing pattern, and conservative treatment with outpatient follow-up may be an option in asymptomatic children.

Recognition of the genus Thaumatophyllum Schott - formerly Philodendron subg. Meconostigma (Araceae) - based on molecular and morphological evidence.

Philodendron subgenus Meconostigma has been a well-circumscribed group since 1829. Members of this group are easily distinguished by diagnostic morphological characters as well as by a distinct ecology and geographical distribution. Based on molecular, morphological and cytological evidence, we propose the recognition of P. subg. Meconostigma as a distinct genus, Thaumatophyllum Schott. We also present the necessary new combinations, an emended key and some nomenclatural and taxonomic corrections regarding 21 names of Thaumatophyllum.

Anthelmintic, Antibacterial and Cytotoxicity Activity of Imidazole Alkaloids from Pilocarpus microphyllus Leaves.

Pilocarpus microphyllus Stapf ex Wardlew (Rutaceae), popularly known as jaborandi, is a plant native to the northern and northeastern macroregions of Brazil. Several alkaloids from this species have been isolated. There are few reports of antibacterial and anthelmintic activities for these compounds. In this work, we report the antibacterial and anthelmintic activity of five alkaloids found in P. microphyllus leaves, namely, pilosine, epiisopilosine, isopilosine, epiisopiloturine and macaubine. Of these, only anthelmintic activity of one of the compounds has been previously reported. Nuclear magnetic resonance, HPLC and mass spectrometry were combined and used to identify and confirm the structure of the five compounds. As regards the anthelmintic activity, the alkaloids were studied using in vitro assays to evaluate survival time and damaged teguments for Schistosoma mansoni adult worms. We found epiisopilosine to have anthelmintic activity at very low concentrations (3.125 µg mL-1 ); at this concentration, it prevented mating, oviposition, reducing motor activity and altered the tegument of these worms. In contrast, none of the alkaloids showed antibacterial activity. Additionally, alkaloids displayed no cytotoxic effect on vero cells. The potent anthelmintic activity of epiisopilosine indicates the potential of this natural compound as an antiparasitic agent. Copyright © 2017 John Wiley & Sons, Ltd.

Floral evolution of Philodendron subgenus Meconostigma (Araceae).

Elucidating the evolutionary patterns of flower and inflorescence structure is pivotal to understanding the phylogenetic relationships of Angiosperms as a whole. The inflorescence morphology and anatomy of Philodendron subgenus Meconostigma, belonging to the monocot family Araceae, has been widely studied but the evolutionary relationships of subgenus Meconostigma and the evolution of its flower characters have hitherto remained unclear. This study examines gynoecium evolution in subgenus Meconostigma in the context of an estimated molecular phylogeny for all extant species of subgenus Meconostigma and analysis of ancestral character reconstructions of some gynoecial structures. The phylogenetic reconstructions of all extant Meconostigma species were conducted under a maximum likelihood approach based on the sequences of two chloroplast (trnk and matK) and two nuclear (ETS and 18S) markers. This topology was used to reconstruct the ancestral states of seven floral characters and to elucidate their evolutionary pattern in the Meconostigma lineage. Our phylogeny shows that Meconostigma is composed of two major clades, one comprising two Amazonian species and the other all the species from the Atlantic Forest and Cerrado biomes with one Amazonian species. The common ancestor of the species of subgenus Meconostigma probably possessed short stylar lobes, long stylar canals, a stylar body, a vascular plexus in the gynoecium and druses in the stylar parenchyma but it is uncertain whether raphide inclusions were present in the parenchyma. The ancestral lineage also probably possessed up to 10 ovary locules. The evolution of these characters seems to have occurred independently in some lineages. We propose that the morphological and anatomical diversity observed in the gynoecial structures of subgenus Meconostigma is the result of an ongoing process of fusion of floral structures leading to a reduction of energy wastage and increase in stigmatic surface.

Genetic relationships and variation in reproductive strategies in four closely related bromeliads adapted to neotropical 'inselbergs': Alcantarea glaziouana, A. regina, A. geniculata and A. imperialis (Bromeliaceae).

BACKGROUND AND AIMS: Bromeliads (Bromeliaceae) adapted to rock outcrops or 'inselbergs' in neotropical rain forests have been identified as suitable plant models for studying population divergence and speciation during continental plant radiations. Little is known about genetic relationships and variation in reproductive strategies within and among inselberg-adapted species, yet knowledge of these parameters is important for understanding divergence processes and for conservation planning. METHODS: Nuclear microsatellites were used to assess the role of clonal reproduction, estimate genetic diversity and explore genetic relationships and variation in reproductive strategies for a total of 15 populations of four closely related Alcantarea inselberg species in south-eastern Brazil: A. glaziouana, A. regina, A. geniculata and A. imperialis. KEY RESULTS: Clonal propagation is frequent in coastal populations of A. glaziouana and A. regina, but absent in the high-altitude species A. geniculata and A. imperialis. Considerable variation in clonal diversity, gene diversity (H(e)), allelic richness, and Wright's inbreeding coefficient (F(IS)) exists within and between species of Alcantarea. A Bayesian analysis of coastal inselberg species indicated pronounced genetic structure. A neighbor-joining analysis grouped populations of each species together with moderate bootstrap support, except for the high altitude species A. imperialis. CONCLUSIONS: The coastal inselberg species A. glaziouana and A. regina tend to propagate asexually via vegetative clonal growth, and both reproductive strategies and breeding systems vary greatly between populations and species of Alcantarea. The microsatellite data indicate a history of hybridization and reticulation involving the high-altitude species A. geniculata and A. imperialis in areas of co-occurrence. The results highlight the need to understand similarities and differences in reproductive strategies both within and between related species for conservation planning and as a basis for understanding evolutionary processes in tropical radiations.

Phylogenetic relationships of aroids and duckweeds (Araceae) inferred from coding and noncoding plastid DNA.

Familial, subfamilial, and tribal monophyly and relationships of aroids and duckweeds were assessed by parsimony and Bayesian phylogenetic analyses of five regions of coding (rbcL, matK) and noncoding plastid DNA (partial trnK intron, trnL intron, trnL-trnF spacer) for exemplars of nearly all aroid and duckweed genera. Our analyses confirm the position of Lemna and its allies (formerly Lemnaceae) within Araceae as the well-supported sister group of all aroids except Gymnostachydoideae and Orontioideae. The last two subfamilies form the sister clade of the rest of the family. Monophyly of subfamilies Orontioideae, Pothoideae, Monsteroideae, and Lasioideae is supported, but Aroideae are paraphyletic if Calla is maintained in its own subfamily (Calloideae). Our results suggest expansion of the recently proposed subfamily Zamioculcadoideae (Zamioculcas, Gonatopus) to include Stylochaeton and identify problems in the current delimitation of tribes Anadendreae, Heteropsideae, and Monstereae (Monsteroideae), Caladieae/Zomicarpeae, and Colocasieae (Aroideae). Canalization of traits of the spathe and spadix considered typical of Araceae evolved after the split of Gymnostachydoideae, Orontioideae, and Lemnoideae. An association with aquatic habitats is a plesiomorphic attribute in Araceae, occurring in the helophytic Orontioideae and free-floating Lemnoideae, but evolving independently in various derived aroid lineages including free-floating Pistia (Aroideae).

Pielonefritis enfisematosa: reporte de dos casos y revisión de la literatura / Emphysematous pyelonephitis: report of two cases and review

Presentamos dos pacientes mujeres diabéticas con Pielonefritis enfisematosa bilateral atendidas en nuestro hospital. Ambas tenían más de 50 años de edad. El diagnóstico se realizó por la presencia de gas a nivel de amos riñones, en la tomografía axial computarizada (TAC) abdominal. Una de las pacientes fue sometida a nefrectomía bilateral y la otra a nefrectomía derecha. Ambas pacientes tuvieron mala evolución pese al tratamiento antibiótico y quirúrgico. Debido a su alta mortalidad hacemos énfasis en el diagnóstico y tratamiento precoz.

Combined genotypic-phenotypic phylogeny of the tribe Spathicarpeae (Araceae) with reference to independent events of invasion to Andean regions.

The Neotropical tribe Spathicarpeae is noteworthy within the family Araceae for having members occurring in very dry and/or very high (above 3000m) areas in South America. Considering the small size of previously included genera in the tribe (10 geophytic genera, none with more than 15 described species), generic circumscription has been considered troublesome. To address these issues, we sequenced two cpDNA markers (matK and trnL-F) and reconstructed the phylogeny of the tribe using two approaches: Maximum parsimony and Bayesian Inference. We also evaluated the effect of adding phenotypic characters in a "total evidence" analysis. Our results confirm the previous suggestion that the two non-geophytic genera Dieffenbachia and Bognera should be included in Spathicarpeae, but the precise placement of Bognera within the tribe is still unresolved. Our results also support the segregation of Asterostigma pavonii and Asterostigma integrifolium, recently described as new monospecific genera (Incarum and Croatiella, respectively). The reconstructed phylogenies suggest that the invasion of the Andean range by the Spathicarpeae occurred in at least two independent events. The inclusion of phenotypic characters in a combined analysis has improved both internal resolution and the number of strongly supported clades.

Hiperoxaluria primaria con pancitopenia: a propósito de un caso / Primary hyperoxaluria and pancitopenia: A case report

Se presenta el caso de un varón, de 15 años de edad, con diagnóstico de litiasis renal desde la infancia que evolucionó a Insuficiencia Renal Crónica, requiriendo hemodiálisis. Seis meses antes del reporte se agregan dolores articulares, con signos flogósicos en rodilla derecha, compromiso progresivo del estado general, pérdida de peso y anemia. Al examen físico se encuentra a un paciente emaciado, pálido, con múltiples adenopatías cervicales, y presencia de hepato-esplenomegalia. Se realizan exámenes evidenciándose: pancitopenia, con mayor compromiso de la serie roja (hemoglobina 7,3mg/dL, leucocitos 2600/uL, y plaquetas 123 000/uL). Los valores de transaminasas y bilirrubinas fueron normales así como los dosajes de Fierro, Transferrina, Acido Fólico y Vitamina B12. La ecografía abdominal revela riñones calcificados y atróficos y los Rx de abdómen demuestran nefrocalcinosis y litiasis renal. La biopsia de medula ósea evidenció un extenso depósito de cristales de oxalato de calcio, dispuestos en forma radiada, con casi completa obliteración de la medula ósea con un número variable de células multinucleadas y fibrosis moderada. El aspirado de medula ósea no mostró cristales de oxalato de calcio. este reporte hace una revisión sobre hiperoxaluria primaria, y resalta la importancia de reconocer la enfermedad como causa de falla renal en un paciente con historia clínica de litiasis renal y nefrocalcinosis.