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. MethodsWe 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. FindingsWe 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•4...
Adolescence is a period in which eating disorders and juvenile systemic lupus erythematosus are typically diagnosed. The coexistence of both disorders prompts the search for a common aetiology. In this paper, we present a case of a 16-year-old girl with life-threatening anorexia nervosa followed by clinical and immunological manifestations of systemic lupus erythematosus. The severity of the symptoms of anorexia nervosa resulted in significant delay in proper diagnosis of the concomitant systemic disease which had already been active. The administration of immunosuppressive treatment resulted in decreased lupus activity and resolution of the symptoms of anorexia nervosa.Being affected by one severe and chronic disease does not preclude the coexistence of another disease of different aetiology. However, such coexistence may suggest a common pathophysiology. Many authors have indicated a possible link between anorexia nervosa and many autoimmune disorders. Currently, modern genetic techniques have confirmed a significant correlation between these disorders. This issue needs further investigation and may be helpful in arriving at the final diagnosis in similar cases.
Objectives: Perinatal medicine is a relatively new, dynamically developing branch of medicine. Its main purpose is taking care of a woman in the pre-conception period, pregnancy and delivery, as well as taking care of a newborn baby. The main aim of the study was to assess the state of knowledge and opinion on hospice perinatal care of professionally active nurses and midwives. Material and methods: An original and anonymous questionnaire containing 30 questions was used for the study. 572 nurses and midwives from the Silesian Voivodeship took part in the study. The obtained data were analyzed. Results: Only 31.6% of respondents defined the level of their knowledge of pregnancy and neonatal care as high. 12.8% of respondents were able to indicate the definition of perinatal care and accurately determine its goals. The women participating in the study were in favor of enclosing the information about not attempting resuscitation (DNAR) in medical record of children with incurable disease diagnosed in fetal life (99.3%). Conclusions: The study showed deficits in practical and theoretical knowledge of nurses and midwives in the area of hospice perinatal care. Lack of proper preparation is also one of the most frequently mentioned difficulties in taking care of a child and family with poor prognosis.
The neurogenic bladder is a polyaetiological disease syndrome. It results from changes in the central and peripheral nervous system and, so far, has been mainly regarded at the macroscopic level. An increasing number of recent studies have pointed, however, to the role of intact bladder innervation also at the cellular level. At present, urothelium is considered not only as a tight barrier but also as a complex structure controlling the activity of the urinary bladder due to multiple connections with the nervous, endocrine, and immune systems. As a result of denervation, the biological processes taking place in urothelium are disturbed. Understanding of such signalling pathways could contribute significantly to effective pharmacological treatment of neurogenic bladder dysfunction. Moreover, it may contribute to the creation of a bio-organ that could potentially replace a dysfunctional urinary bladder in such patients.
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