The 2021 Eurpean Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type i interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of \u27points to consider\u27 to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, \u27points to consider\u27 to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes

The 2021 EULAR and ACR points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes

The 2021 EULAR and ACR points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes

Utilization of mechanical power and associations with clinical outcomes in brain injured patients: a secondary analysis of the extubation strategies in neuro-intensive care unit patients and associations with outcome (ENIO) trial

Background: There is insufficient evidence to guide ventilatory targets in acute brain injury (ABI). Recent studies have shown associations between mechanical power (MP) and mortality in critical care populations. We aimed to describe MP in ventilated patients with ABI, and evaluate associations between MP and clinical outcomes. Methods: In this preplanned, secondary analysis of a prospective, multi-center, observational cohort study (ENIO, NCT03400904), we included adult patients with ABI (Glasgow Coma Scale ≤ 12 before intubation) who required mechanical ventilation (MV) ≥ 24 h. Using multivariable log binomial regressions, we separately assessed associations between MP on hospital day (HD)1, HD3, HD7 and clinical outcomes: hospital mortality, need for reintubation, tracheostomy placement, and development of acute respiratory distress syndrome (ARDS). Results: We included 1217 patients (mean age 51.2 years [SD 18.1], 66% male, mean body mass index [BMI] 26.3 [SD 5.18]) hospitalized at 62 intensive care units in 18 countries. Hospital mortality was 11% (n = 139), 44% (n = 536) were extubated by HD7 of which 20% (107/536) required reintubation, 28% (n = 340) underwent tracheostomy placement, and 9% (n = 114) developed ARDS. The median MP on HD1, HD3, and HD7 was 11.9 J/min [IQR 9.2-15.1], 13 J/min [IQR 10-17], and 14 J/min [IQR 11-20], respectively. MP was overall higher in patients with ARDS, especially those with higher ARDS severity. After controlling for same-day pressure of arterial oxygen/fraction of inspired oxygen (P/F ratio), BMI, and neurological severity, MP at HD1, HD3, and HD7 was independently associated with hospital mortality, reintubation and tracheostomy placement. The adjusted relative risk (aRR) was greater at higher MP, and strongest for: mortality on HD1 (compared to the HD1 median MP 11.9 J/min, aRR at 17 J/min was 1.22, 95% CI 1.14-1.30) and HD3 (1.38, 95% CI 1.23-1.53), reintubation on HD1 (1.64; 95% CI 1.57-1.72), and tracheostomy on HD7 (1.53; 95%CI 1.18-1.99). MP was associated with the development of moderate-severe ARDS on HD1 (2.07; 95% CI 1.56-2.78) and HD3 (1.76; 95% CI 1.41-2.22). Conclusions: Exposure to high MP during the first week of MV is associated with poor clinical outcomes in ABI, independent of P/F ratio and neurological severity. Potential benefits of optimizing ventilator settings to limit MP warrant further investigation

Proceedings of the 24th Paediatric Rheumatology European Society Congress: Part three

From Springer Nature via Jisc Publications Router.Publication status: PublishedHistory: collection 2017-09, epub 2017-09-0

Urban analysis of the Našice city square

Izgled glavnog našičkog trga mijenjao se nekoliko puta kroz povijest. Važnu ulogu u razvoju grada imali su franjevci, obitelj Aba te kasnije obitelj Pejačević. Franjevci su u središtu mjesta izgradili samostan i crkvu koji se ondje nalaze i danas, na zapadnom rubu trga. Tijekom vladavine obitelji Aba naseljavali su se ljudi iz raznih krajeva te se razvijala trgovina i obrt. Na istočnoj strani današnjeg trga tada se nalazila crkva Presvetog Trojstva i groblje na brežuljku. Dolaskom obitelji Pejačević u 19. st. Počinje značajniji razvoj grada. Uklanjanjem ostataka crkve Presvetog Trojstva i groblja otvorio se prostor na kojemu se grade stambene i javne zgrade te se tako počinje oblikovati središnji trg. Prostrani trg u središtu Našica izrazito je izduženog oblika u smjeru istok – zapad. Omeđen je važnim zgradama poput franjevačke crkve sv. Antuna Padovanskog na zapadnom dijelu, zgradom starog hotela te Dvorca Pejačević na sjevernom dijelu, kućom obitelji Goldfinger te mnogim stambenim i poslovnim zgradama uz južni rub trga. Krajem 20. st. Trg je rekonstruiran, izgrađena je fontana na istočnom dijelu, a zapadni dio služio je kao parkiralište. Današnji izgled glavni našički trg dobiva 2020. godine. U istočnom dijelu napravljen je vodoskok i paviljon, a zapadni dio trga postao je pješačka zona.The appereance of main square in Nasice has changed few times through years. Important role in city development had Franciscans, family Aba and later family Pejacevic. Franciscans built monastery and church in the city centre where they can be found still today, at west side of square. While family Aba was on reign, people started to move from other countries as trading and crafting were increasing. On the east part of todays main square there were church of Saint Trinity and cemetery on hill. In 19th century family Pejacevic came on reign and city started to develop significantly. By removing leftovers of the church and cemetery, big space was made where they built residential and public buildings which constructed shape of main square. Spacious square in city centre in Nasice is markedly elongate shaped in direction east-west. It is bounded with important buildings such as Church of Antun Padovanski on west side , old city hotel and Pejacevic Castle on northern side, family Goldfinger house and many residential and business buildings along southern side of square. At the end of 20t century square was reconstructed, fountain was built on eastern side, as western part was used as parking lot. Todays appereance main square in Nasice got in 2020. On eastern side new fountain and pavilion were built , while western part became pedestrian zone

Urban analysis of the Našice city square

Izgled glavnog našičkog trga mijenjao se nekoliko puta kroz povijest. Važnu ulogu u razvoju grada imali su franjevci, obitelj Aba te kasnije obitelj Pejačević. Franjevci su u središtu mjesta izgradili samostan i crkvu koji se ondje nalaze i danas, na zapadnom rubu trga. Tijekom vladavine obitelji Aba naseljavali su se ljudi iz raznih krajeva te se razvijala trgovina i obrt. Na istočnoj strani današnjeg trga tada se nalazila crkva Presvetog Trojstva i groblje na brežuljku. Dolaskom obitelji Pejačević u 19. st. Počinje značajniji razvoj grada. Uklanjanjem ostataka crkve Presvetog Trojstva i groblja otvorio se prostor na kojemu se grade stambene i javne zgrade te se tako počinje oblikovati središnji trg. Prostrani trg u središtu Našica izrazito je izduženog oblika u smjeru istok – zapad. Omeđen je važnim zgradama poput franjevačke crkve sv. Antuna Padovanskog na zapadnom dijelu, zgradom starog hotela te Dvorca Pejačević na sjevernom dijelu, kućom obitelji Goldfinger te mnogim stambenim i poslovnim zgradama uz južni rub trga. Krajem 20. st. Trg je rekonstruiran, izgrađena je fontana na istočnom dijelu, a zapadni dio služio je kao parkiralište. Današnji izgled glavni našički trg dobiva 2020. godine. U istočnom dijelu napravljen je vodoskok i paviljon, a zapadni dio trga postao je pješačka zona.The appereance of main square in Nasice has changed few times through years. Important role in city development had Franciscans, family Aba and later family Pejacevic. Franciscans built monastery and church in the city centre where they can be found still today, at west side of square. While family Aba was on reign, people started to move from other countries as trading and crafting were increasing. On the east part of todays main square there were church of Saint Trinity and cemetery on hill. In 19th century family Pejacevic came on reign and city started to develop significantly. By removing leftovers of the church and cemetery, big space was made where they built residential and public buildings which constructed shape of main square. Spacious square in city centre in Nasice is markedly elongate shaped in direction east-west. It is bounded with important buildings such as Church of Antun Padovanski on west side , old city hotel and Pejacevic Castle on northern side, family Goldfinger house and many residential and business buildings along southern side of square. At the end of 20t century square was reconstructed, fountain was built on eastern side, as western part was used as parking lot. Todays appereance main square in Nasice got in 2020. On eastern side new fountain and pavilion were built , while western part became pedestrian zone

Urban analysis of the Našice city square

Izgled glavnog našičkog trga mijenjao se nekoliko puta kroz povijest. Važnu ulogu u razvoju grada imali su franjevci, obitelj Aba te kasnije obitelj Pejačević. Franjevci su u središtu mjesta izgradili samostan i crkvu koji se ondje nalaze i danas, na zapadnom rubu trga. Tijekom vladavine obitelji Aba naseljavali su se ljudi iz raznih krajeva te se razvijala trgovina i obrt. Na istočnoj strani današnjeg trga tada se nalazila crkva Presvetog Trojstva i groblje na brežuljku. Dolaskom obitelji Pejačević u 19. st. Počinje značajniji razvoj grada. Uklanjanjem ostataka crkve Presvetog Trojstva i groblja otvorio se prostor na kojemu se grade stambene i javne zgrade te se tako počinje oblikovati središnji trg. Prostrani trg u središtu Našica izrazito je izduženog oblika u smjeru istok – zapad. Omeđen je važnim zgradama poput franjevačke crkve sv. Antuna Padovanskog na zapadnom dijelu, zgradom starog hotela te Dvorca Pejačević na sjevernom dijelu, kućom obitelji Goldfinger te mnogim stambenim i poslovnim zgradama uz južni rub trga. Krajem 20. st. Trg je rekonstruiran, izgrađena je fontana na istočnom dijelu, a zapadni dio služio je kao parkiralište. Današnji izgled glavni našički trg dobiva 2020. godine. U istočnom dijelu napravljen je vodoskok i paviljon, a zapadni dio trga postao je pješačka zona.The appereance of main square in Nasice has changed few times through years. Important role in city development had Franciscans, family Aba and later family Pejacevic. Franciscans built monastery and church in the city centre where they can be found still today, at west side of square. While family Aba was on reign, people started to move from other countries as trading and crafting were increasing. On the east part of todays main square there were church of Saint Trinity and cemetery on hill. In 19th century family Pejacevic came on reign and city started to develop significantly. By removing leftovers of the church and cemetery, big space was made where they built residential and public buildings which constructed shape of main square. Spacious square in city centre in Nasice is markedly elongate shaped in direction east-west. It is bounded with important buildings such as Church of Antun Padovanski on west side , old city hotel and Pejacevic Castle on northern side, family Goldfinger house and many residential and business buildings along southern side of square. At the end of 20t century square was reconstructed, fountain was built on eastern side, as western part was used as parking lot. Todays appereance main square in Nasice got in 2020. On eastern side new fountain and pavilion were built , while western part became pedestrian zone

The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: CANDLE/PRAAS, SAVI, and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)–associated vasculopathy with onset in infancy (SAVI), and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of “points to consider” to improve diagnosis, treatment, and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates, and an allied health care professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires, and consensus methodology, “points to consider” to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment, and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI, and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment, and management of patients with CANDLE/PRAAS, SAVI, and AGS and aim to standardize and improve care, quality of life, and disease outcomes