Search

BACKGROUND: Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on histopathological evaluation of lung biopsy or autopsy tissue or genetic testing of FOXF1 on chromosome 16q24.1. Here, we describe the first two Polish patients with ACD confirmed by histopathological and genetic examination. CASE PRESENTATION: The patients were term neonates with high Apgar scores in the first minutes of life. They both were diagnosed prenatally with heart defects. Additionally, the first patient presented with omphalocele. The neonate slightly deteriorated around 12th hour of life, but underwent surgical repair of omphalocele followed by mechanical ventilation. Due to further deterioration, therapy included inhaled nitric oxide (iNO), inotropes and surfactant administration. The second patient was treated with prostaglandin E1 since birth due to suspicion of aortic coarctation (CoA). After ruling out CoA in the 3rd day of life, infusion of prostaglandin E1 was discountinued and immediately patient's condition worsened. Subsequent treatment included re-administration of prostaglandin E1, iNO and mechanical ventilation. Both patients presented with transient improvement after application of iNO, but died despite maximized therapy. They were histopathologically diagnosed post-mortem with ACD. Array comparative genomic hybridization in patient one and patient two revealed copy-number variant (CNV) deletions, respectively, ~ 1.45 Mb in size involving FOXF1 and an ~ 0.7 Mb in size involving FOXF1 enhancer and leaving FOXF1 intact. CONCLUSIONS: Both patients presented with a distinct course of ACD, extra-pulmonary manifestations and response to medications. Surgery and ceasing of prostaglandin E1 infusion should be considered as potential causes of this variability. We further highlight the necessity of thorough genetic testing and histopathological examination and propose immunostaining for CD31 and CD34 to facilitate the diagnostic process for better management of infants with ACD.

Supplemental material, sj-pdf-1-pec-10.1177_03010066211034368 for Perceptual Awareness of Optic Flows Paced Optimally and Non-optimally to Walking Speed by Paweł Motyka, Zuzanna Kozłowska and Piotr Litwin in Perception

Dwu- oraz wielojęzyczność tekstu literackiego poddają się analizie jako obliczony na określony efekt chwyt. Przekroczenie horyzontu jednojęzyczności jest zarazem wkroczeniem w przestrzeń wielokulturową. Negacja monolingwizmu komplikuje relacje tożsamości i przynależności, rekalibruje związki obcego z własnym, znanego z nieznanym, toż-samego i innego, wreszcie: staje się pytaniem o możliwość przekładu słowa, języka, doświadczenia. Autobiografizm, w swoich miriadowych formach i przejawach, wymaga od badacza prześledzenia i określenia relacji pomiędzy przeżytym a powiedzianym. W przypadku tekstów łamiących dogmat jednojęzyczności nasileniu - swoistemu podwojeniu - i komplikacji ulec mogą napięcia pomiędzy doświadczeniem a jego językową transpozycją. Rozpięta pomiędzy Nowym Wiśniczem, Krakowem a Paryżem twórczość Jana Brzękowskiego realizuje się w paradygmacie wielojęzyczności faktycznej. Autor tomiku Na katodzie uczestniczy w literaturze podwójnie - jako pisarz polsko- oraz francuskojęzyczny. W twórczości Brzękowskiego wyczuwalne są przede wszystkim polsko-romańskie napięcia językowe. Jego polskojęzyczna poezja, zarażona mniej lub bardziej wyraźnie żywiołem francuszczyzny, przypomina niekiedy cytat z "obcej" kultury, będąc nierzadko całkiem dosłownym cytatem z obcego języka. Eksperymentalne zestawienie utworu poetyckiego Montparnasse z fragmentem kroniki kulturalnej W Krakowie i w Paryżu... (rozdział Seuphor) ujawniło mechanizm wzajemnego naświetlania się przeżytego-pamiętanego oraz poetyckiego w wielojęzycznych przestrzeniach twórczości autora. Wspomnienie dopełnia poezję, staje się jej komentarzem, przypisem, odautorską notą. Porównanie sytuuje oba teksty poza - fantazmatycznym w istocie - podziałem na fikcję i autobiografię, życie i opowieść. W poezji Brzękowskiego fantastyka graniczy z pretensją do autentyzmu, realność zasmakowanego, zasłyszanego - z metarealizmem. To właśnie mechanizm parafra-zowania własnej biografii uruchamia "chwyt wielojęzyczności", służący ekspresji oraz kreacji plurilingwalnej i wielokulturowej podmiotowości oraz hybrydycznego modelu świata na wzór biograficznie motywowanego doświadczenia emigracyjnego pogranicza, przebiegającego zawsze w poprzek kultur, języków, literatur, biografii. Bi- and multilingualism of literary text can be regarded and analyzed as an artistic device, employed purposely as a means of producing certain literary effects. Where monolingualism is transgressed, multiculturalism emerges. Along rise the ambiguities and complexities of linguistic and cultural identities (the Derridian "disorders"), thus recalibrating the relation of self and other, of one's own and foreign, known and unknown, eventually calling into question a mere translatability of a word, of a language and finally: of one's experience. Autobiographism, in its myriad forms and manifestations, requires on the part of researcher a determination of interdependence between experience and its verbal account. In the case of literary works that rebel against the monolingualism dogma the tension between experience and its textual transposition becomes inevitably intensified, "doubled", tangled. The Polish-Romance linguistic tensions are by far the most conspicuous in Jan Brzękowski's multilingual literary works. The writer's artistic path split indeed into two separate literary careers: a French and a Polish one. Brzękowski's Polish poetry, often contaminated with French expressions, hints, and wordplays, resembles an extract of foreign culture - and is in fact quite often encrusted with macaronic French quotes. In the article, the experimental comparative juxtaposition of Jan Brzękowski's poem Montparnasse with a passage of the writer's autobiographical prose W Krakowie i w Paryżu... (chapter Seuphor) revealed the mecanism of mutual elucidation of remembered experience and its poetic rendering in the multilingual area of the writer's works. The memoirs complete the poetry as its circumstantial and factual commentary, as the author's explanatory note to the exotic content of his surrealism-inspired poetry. The comparison reveals a phantasmic and arbitrary nature of the widely presumed distinction between fiction and non-fiction, a tale and an autobiography, life and literature. In Jan Brzękowski's poetry, fiction borders pretention to authenticity; the biographical facts and memoirs intertwine with the exoticism of metarealism. It's the mechanism of paraphrasing one's own biography that activates the "plurilinguism device", thus serving the expression and creation of a polyglot and multicultural subject and of a hybrid model of the world - thereby corresponding with biographically motivated experience of emigrant liminality, positioned invariably at the intersection of cultures, languages, literatures and biographies.

Background Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on histopathological evaluation of lung biopsy or autopsy tissue or genetic testing of FOXF1 on chromosome 16q24.1. Here, we describe the first two Polish patients with ACD confirmed by histopathological and genetic examination. Case presentation The patients were term neonates with high Apgar scores in the first minutes of life. They both were diagnosed prenatally with heart defects. Additionally, the first patient presented with omphalocele. The neonate slightly deteriorated around 12th hour of life, but underwent surgical repair of omphalocele followed by mechanical ventilation. Due to further deterioration, therapy included inhaled nitric oxide (iNO), inotropes and surfactant administration. The second patient was treated with prostaglandin E1 since birth due to suspicion of aortic coarctation (CoA). After ruling out CoA in the 3rd day of life, infusion of prostaglandin E1 was discountinued and immediately patient’s condition worsened. Subsequent treatment included re-administration of prostaglandin E1, iNO and mechanical ventilation. Both patients presented with transient improvement after application of iNO, but died despite maximized therapy. They were histopathologically diagnosed post-mortem with ACD. Array comparative genomic hybridization in patient one and patient two revealed copy-number variant (CNV) deletions, respectively, ~ 1.45 Mb in size involving FOXF1 and an ~ 0.7 Mb in size involving FOXF1 enhancer and leaving FOXF1 intact. Conclusions Both patients presented with a distinct course of ACD, extra-pulmonary manifestations and response to medications. Surgery and ceasing of prostaglandin E1 infusion should be considered as potential causes of this variability. We further highlight the necessity of thorough genetic testing and histopathological examination and propose immunostaining for CD31 and CD34 to facilitate the diagnostic process for better management of infants with ACD.