Two new species of Simothraulopsis Demoulin, 1966 (Ephemeroptera: Leptophlebiidae) from Roraima State, northern Brazil.

Two new species of Simothraulopsis (Ephemeroptera: Leptophlebiidae) are described and illustrated, based on recent collections and specimens deposited in the Zoological Collection of Universidade Federal de Roraima (UFRR), northern Brazil. Simothraulopsis pacaraima sp. nov.

Updated records for Leptophlebiidae (Ephemeroptera) and a new species of Thraulodes Ulmer, 1920 from Tocantins State, Northern Brazil.

An annotated checklist with complementary information for leptophlebiid mayflies from Tocantins State, Brazil is provided. Additionally, we describe a new species of Thraulodes Ulmer, 1920 based on male adults. New reports are based on collections conducted between August 2017 and August 2019 in 13 localities of 10 municipalities.

Redescription of Apobaetis lakota McCafferty, 2000 (Ephemeroptera: Baetidae) and description of two new species from Brazil.

The genus Apobaetis Day is known by its small size and larval shifting-sand habitat preference (psammophilous). Three species of this genus are recorded in North America, from these, only Apobaetis lakota McCafferty needs to be redescribed because its original description is incomplete, turning difficult to distinguish it from species with similar morphology. Therefore, one of the objectives of this study is to redescribe A.

Immunofluorescence mapping, electron microscopy and genetics in the diagnosis and sub-classification of inherited epidermolysis bullosa: a single-centre retrospective comparative study of 87 cases with long-term follow-up.

Background: Epidermolysis bullosa (EB) comprises a heterogeneous group of skin fragility disorders, classified in four major types based on skin cleavage level, i.e. EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), Kindler EB, and in more than 30 subtypes defined by the combination of laboratory and clinical data, including disease course.

Cellular and gene signatures of tumor-infiltrating dendritic cells and natural-killer cells predict prognosis of neuroblastoma.

Tumor-infiltrating lymphocytes play an essential role in improving clinical outcome of neuroblastoma (NB) patients, but their relationship with other tumor-infiltrating immune cells in the T cell-inflamed tumors remains poorly investigated. Here we show that dendritic cells (DCs) and natural killer (NK) cells are positively correlated with T-cell infiltration in human NB, both at transcriptional and protein levels, and associate with a favorable prognosis. Multiplex imaging displays DC/NK/T cell conjugates in the tumor microenvironment of low-risk NB.

The Crucial Role of Surgical Treatment in BIA-ALCL Prognosis in Early- and Advanced-Stage Patients.

Background: Studies on breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) are trying to optimize medical and surgical treatments for early and advanced stages of this disease. The aim of this article is to share the experience gathered on the authors' prospectively collected 46 well-documented cases.

Methods: Italian physicians are obliged to report BIA-ALCL cases to the Italian Ministry of Health.

New species of the genus Macrogynoplax and new records of Anacroneuria (Plecoptera: Perlidae) from Roraima State, Brazil.

Adults of Plecoptera of the family Perlidae were studied from seven municipalities of Roraima State, Brazil. One new species of Macrogynoplax Enderlein, 1909 was discovered and is described. We also report seven species of Anacroneuria Klapálek 1909 that represent two new country records for Brazil, A.

A new species of Macrelmis Motschulsky, 1859 (Coleoptera: Elmidae) and new records of Elmidae from Roraima State, northern Brazil.

A new species of Macrelmis Motschulsky, 1859 (Macrelmis rodrigoi sp. nov.) is described and illustrated based on specimens found in mountainous areas of the northern Roraima State, Brazil.

Hoarse cry in a newborn with epidermolysis bullosa simplex, generalized severe.

Hoarse cry and respiratory stridor are the signs of potentially life-threatening laryngeal involvement in selected severe and frequently early lethal subtypes of inherited epidermolysis bullosa (EB). We present a newborn with generalized skin blistering and onychodystrophy who developed a hoarse cry and inspiratory stridor. Ultrastructural skin examination revealed tonofilament clumping in basal keratinocytes and genetic testing identified the de novo missense mutation p.

Diversity of EPT (Ephemeroptera, Plecoptera, Trichoptera) Along Streams Fragmented by Waterfalls in the Brazilian Savanna.

In stream ecosystems, waterfalls intercept the fluvial continuum and limit the dispersion of aquatic organisms. However, the barrier effect may be minimized or absent for insects whose life cycle is not restricted to the aquatic environment (i.e.

A new species of Camelobaetidius Demoulin, 1966 (Ephemeroptera: Baetidae), from the Colombian Orinoco River basin.

Three species of Camelobaetidius Demoulin, 1966 have been reported from Colombia until now, based on nymphs. We describe a fourth species based on nymphs from the Colombian Orinoco river basin. The new species can be recognized by: 1) labrum narrowly rounded anteriorly; 2) segment II of labial palp with a short rounded distomedial projection; 3) incisors of right mandible with eleven denticles; 4) gills absent from the bases of coxae; 5) tarsal claws with ten denticles; 6) outer margin of forefemur with a row of about 23 long, spine-like setae; 7) posterior margin of tergum IV with truncate spines, and 8) terminal filament almost as long as cerci.

A new species of Simothraulopsis Demoulin, 1966 (Ephemeroptera: Leptophlebiidae) from Tocantins state, Brazil.

There are seven genera and eight species of mayflies recorded for Tocantins state, Brazil, but only three belonging to the family Leptophlebiidae. Based on male imagoes, Simothraulopsis primus sp. nov.

Phenotype characterisation of mutation and deletion carriers with neonatal and paediatric pulmonary hypertension.

Rare variants in the T-box transcription factor 4 gene () have recently been recognised as an emerging cause of paediatric pulmonary hypertension (PH). Their pathophysiology and contribution to persistent pulmonary hypertension in neonates (PPHN) are unknown. We sought to define the spectrum of clinical manifestations and histopathology associated with variants in neonates and children with PH.

Tumor-infiltrating T cells and PD-L1 expression in childhood malignant extracranial germ-cell tumors.

Although pediatric malignant extracranial germ-cell tumors (meGCTs) are among the most chemosensitive solid tumors, a group of patients relapse and die of disease. To identify new markers predicting clinical outcome, we examined the prognostic relevance of tumor-infiltrating T lymphocytes (TILs) and the expression of PD-1 and PD-L1 in a cohort of pediatric meGCTs by immunohistochemistry. MeGCTs were variously infiltrated by T cell-subtypes according to the tumor subtype, tumor location and age at diagnosis.

New species and new record of Thraulodes Ulmer, 1920 (Ephemeroptera: Leptophlebiidae: Atalophlebiinae) from Brazil.

A new species of the mayfly genus Thraulodes is described based on specimens from the state of Roraima, Brazil. The male imago of Thraulodes rodrigoi sp. nov.

Description of the nymph of Miroculis (Miroculis) eldorado Gama-Neto Hamada, 2014 (Leptophlebiidae: Ephemeroptera) from Roraima, northern Brazil.

The genus Miroculis Edmunds, 1963 (Ephemeroptera: Leptophlebiidae) is represented by 23 species: two of them aredescribed based solely on nymphs, 10 based solely on male adults, and three based on nymphs and adults  (Domínguez et al., 2006; Gama-Neto Hamada, 2013, 2014; Raimundi et al., 2017).

New species and new records of Coryphorus Peters, 1981 (Ephemeroptera: Coryphoridae) from Brazil.

A new species Coryphorus is presented,  based on recent collections from Northeastern Brazil and specimens deposited in the Museu Regional de Entomologia da Universidade Federal de Viçosa (UFVB) and Coleção Zoológica da Universidade Federal de Roraima (UFRR). Coryphorus molinerii sp. nov.

Malignant testicular germ cell tumors in children and adolescents: The AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) protocol.

Objectives: We report the results of an Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) study on the treatment of testicular germ cell tumors (TGCT) with a pediatric PEB (pPEB) regimen (cisplatin 25 mg/m daily on days 1-4; etoposide 100 mg/m daily on days 1-4; bleomycin 15 mg/m on day 2, once per cycle).

Methods And Materials: Male patients under 18 years old with malignant TGCT were enrolled for a second national prospective protocol. All patients underwent orchiectomy at diagnosis.

gene acts as a tumor suppressor in human neuroblastoma.

Neuroblastoma is an aggressive, relapse-prone childhood tumor of the sympathetic nervous system that accounts for 15% of pediatric cancer deaths. A distal portion of human chromosome 3p is often deleted in neuroblastoma, this region may contain one or more putative tumor suppressor genes. A 2.

Mineralization of alpha-1-antitrypsin inclusion bodies in Mmalton alpha-1-antitrypsin deficiency.

Background: Alpha-1-antitrypsin (AAT) deficiency (AATD) of Z, Mmalton, Siiyama type is associated with liver storage of the mutant proteins and liver disease. The Z variant can be diagnosed on isoelectric focusing (IEF) while Mmalton and Siiyama may be missed or misdiagnosed with this technique. Therefore, molecular analysis is mandatory for their characterization.

Congenital Rhabdomyosarcoma: a different clinical presentation in two cases.

Background: Rhabdomyosarcoma (RMS), one of the most common soft tissue sarcomas of childhood, is very rare in the neonatal period (0.4-2% of cases). In order to gain a deeper understanding of this disease at such age, patient and tumor features, as well as treatment modality and outcome need to be reported.