Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

KLASIK TIP HODGKIN LENFOMA İLE TAKIPLI HASTADA YENI GELIŞEN LENFADENOPATI: CMV LENFADENITI OLGU SUNUMU

Giriş: Sitomegalovirüs (CMV) herpesvirüs ailesinden bir virüs olup, tükrük/solunum yolu, kan ve cinsel yolla bulaşır. Primer enfeksiyonda asemptomatik ya da ateş, servikal lenfadenopati gibi grip benzeri semptomlarla seyrederken immün sistemi baskılanmış hastalarda gastrointestinal ve nörolojik semptomlara neden olan yaygın enfeksiyon görülebilir. Olgu: 2019 yılında Hodgkin lenfoma, klasik tip, nodüler skleroz tanısı ile tedavi alan 27 yaşında kadın hastanın takiplerinde PET/BT’de sağ submandibuler bölgede 9x7 mm ölçülerinde hipermetabolik lenf nodu saptandı (SUD max 13,5). Takibinde yapılan USG incelemesinde gangliyon boyutunda artış görülmesi nedeniyle eksizyonel biyopsi yapıldı. 1,8 cm çapındaki lenf gangliyonunun kesitinde kapsül ince ve düzenli, sinüsler açık olarak izlendi. Bir kısmı florid tarzda olmak üzere lenfoid folikül hiperplazisi görüldü. Fokal alanda sentrosit benzeri hücrelerle karışık halde iri-atipik hücrelerin varlığı dikkati çekti. Klinik öykü nedeniyle Hodgkin lenfomanın fokal tutulumu açısından yapılan antijenik incelemelerde bu yönde bulgu saptanmadı. Seri kesitlerde fokal olarak damar endotelinde viral sitopatik etkiyi düşündüren intranükleer inklüzyon dikkati çekti. CMV immünhistokimyasal incelemesinde damar endotelinde ve interfoliküler alanda tanımlanan iri atipik hücrelerde pozitif reaksiyon izlendi. Bulgular CMV lenfadeniti olarak değerlendirildi. Sonuç: CMV lenfadenitinde foliküler ve parakortikal hiperplazi, monositoid hücre hiperplazisi ile karakterli mikst tipte reaktif değişiklikler görülebilir. Virüs lenfosit, monosit ve endotel hücrelerini enfekte eder ve bir kısmında daha belirgin olmak üzere nükleer inklüzyon oluşturur. Belirgin nükleer inklüzyon içeren iri atipik hücreler özellikle Hodgkin lenfoma öyküsü olan hastalarda nüks açısından kuşku yaratabilir. Ayırıcı tanıda eozinofil ve histiyositlerden zengin zeminde CD30 veCD15 pozitifliği Hodgkin lenfoma lehine değerlendirilirken, CMV immünhistokimyası ile enfekte hücrelerin gösterilmesi CMV lenfadeniti açısından tanı koydurucudur

The clinical features, treatment and prognosis of neutropenic fever and Coronavirus disease 2019 results of the multicentre teos study

Abstract This multicentre (22 centres in Turkey) retrospective cohort study aimed to assess the clinical outcomes of patients with neutropenic fever and SARS-CoV-2 positivity. Study period was 15 March 2020–15 August 2021. A total of 170 cases (58 female, aged 59 ± 15.5 years) that fulfilled the inclusion criteria were included in the study. One-month mortality rate (OMM) was 44.8%. The logistic regression analysis showed the following significant variables for the mentioned dependent variables: (i) achieving PCR negativity: receiving a maximum of 5 days of favipiravir (p = 0.005, OR 5.166, 95% CI 1.639–16.280); (ii) need for ICU: receiving glycopeptide therapy at any time during the COVID-19/FEN episode (p = 0.001, OR 6.566, 95% CI 2.137–20.172), the need for mechanical ventilation (p < 0.001, OR 62.042, 95% CI 9.528–404.011); (iii) need for mechanical ventilation: failure to recover from neutropenia (p < 0.001, OR 17.869, 95% CI 3.592–88.907), receiving tocilizumab therapy (p = 0.028, OR 32.227, 95% CI 1.469–707.053), septic shock (p = 0.001, OR 15.4 96% CI 3.164–75.897), and the need for ICU (p < 0.001, OR 91.818, 95% CI 15.360–548.873), (iv) OMM: [mechanical ventilation (p = 0.001, OR 19.041, 95% CI 3.229–112.286) and septic shock (p = 0.010, OR 5.589,95% CI 1.509–20.700)]. Although it includes a relatively limited number of patients, our findings suggest that COVID-19 and FEN are associated with significant mortality and morbidity

Castleman Disease: A Multicenter Case Series from Turkey.

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions