Long-term follow-up in pediatric intensive care-a narrative review.

Pediatric intensive care is a rapidly developing medical specialty and with evolving understanding of pediatric pathophysiology and advances in technology, most children in the developed world are now surviving to intensive care and hospital discharge. As mortality rates for children with critical illness continue to improve, increasing PICU survivorship is resulting in significant long-term consequences of intensive care in these vulnerable patients. Although impairments in physical, psychosocial and cognitive function are well documented in the literature and the importance of establishing follow-up programs is acknowledged, no standardized or evidence-based approach to long-term follow-up in the PICU exists. This narrative review explores pediatric post-intensive care syndrome and summarizes the multifactorial deficits and morbidity that can occur in these patients following recovery from critical illness and subsequent discharge from hospital. Current practices around long-term follow-up are explored with discussion focusing on gaps in research and understanding with suggested ways forward and future directions.

Neurodevelopmental outcomes in neonatal extracorporeal membrane oxygenation survivors: An institutional perspective.

AIM: To describe the neurodevelopmental outcomes following extracorporeal membrane oxygenation (ECMO) in early infancy. METHODS: Thirty-seven patients who had survived following ECMO support from 2008 to 2018 had their neurodevelopmental outcomes assessed and analysed using the Bayley Scales of Infant and Toddler Development. Developmental outcome was defined as impairment in any of the developmental domains of motor function, cognition and language with mild impairment being 1-2 standard deviations below the test mean, moderate being 2-3 standard deviations below and severe being greater than 3 standard deviations below. RESULTS: Of these 37 patients, the median age at admission to Paediatric Intensive Care Unit was 0.4 months (interquartile range 2.8 months) with all of the study patients having an underlying diagnosis of congenital cardiac disease and 37/40 (92.5%) ECMO runs occurring in the immediate post-operative period. Of the 29 patients who had had follow-up at 12 months of age or older, 3 (10.3%) had severe impairment, 4 (13.8%) had moderate impairment, 12 (41.3%) had mild impairment and 10 (34.5%) had no impairment. Gross motor function was most significantly impacted in 18/29 (62.1%) patients, of which 7/18 (38.9%) had severe impairment. This was followed by impairment of receptive language in 8/29 (27.6%) patients and expressive language in 6/29 (20.1%) patients. CONCLUSIONS: One in four infants undergoing ECMO treatment in early infancy has moderate to severe neurodevelopmental impairment. Gross motor and language are the most affected developmental domains.

A familial case of Kikuchi-Fujimoto disease in dizygotic twins.

BACKGROUND: Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970's. Despite its rarity in the pediatric population, it is an important differential in persistent lymphadenopathy. Familial cases of KFD in the literature are rare. Here we describe the first reported case of KFD in non-identical twin sisters. CASE PRESENTATION: Twin 1 presented with a 3-week history of worsening right-sided cervical lymphadenopathy, daily fevers, significant lethargy, weight loss and arthralgia of her knees and ankles at the age of 12 years in 2015. She had had an unremarkable medical history. A biopsy of her lymph nodes showed histiocytic necrosis consistent with KFD. Twin 2 presented with a three-week history of lethargy, fatigue, weight loss and left-sided posterior cervical chain lymphadenopathy at 16 years of age in 2018. She had a history of frequently relapsing nephrotic syndrome and celiac disease. A biopsy of her lymph nodes was undertaken and showed histiocytic necrosis consistent with KFD. CONCLUSIONS: KFD is a rare but self-limiting pathological process of necrotizing histiocytic lymphadenitis. Although further research is needed, there is an increasing amount of evidence which suggests a multifactorial pathological basis of disease. The two cases we document here are the first reported cases of familial KFD in dizygotic HLA-identical twins which reinforces the likely HLA-linkage in the etiology of KFD.

Procalcitonin and C-reactive protein as biomarkers for neonatal bacterial infection.

AIM: Neonates are predisposed to bacterial infection which are an important cause of early childhood morbidity and mortality globally. It has been proposed that procalcitonin has significant utility as a diagnostic marker for bacterial infection in febrile neonates when compared to C-reactive protein (CRP). The aim of this study is to conduct a literature search to find the best available evidence to answer the clinical question of the utility of procalcitonin when compared to CRP as a predictor of bacterial infection in febrile neonates. METHODS: Medline/PubMed was searched using the terms 'procalcitonin', 'C-reactive protein', 'bacterial infection' and 'neonatal sepsis'. Three systematic reviews relevant to the clinical question were identified and the key article selected for critical appraisal was the systematic review by Yu et al. (2010). RESULTS: The appraised literature concludes that procalcitonin has moderate accuracy in diagnosing neonatal sepsis, but suggests it should be considered only within the context of other clinical parameters and other relevant investigations. The studies included in the systematic review were of variable quality, showed considerable heterogeneity in their methods and evidence of possible publication bias. CONCLUSION: Further research is required before definitive recommendations can be made about the utility of procalcitonin compared with CRP as a diagnostic marker for neonatal sepsis and bacterial infection in clinical practice.

The dual role of the novel Wnt receptor tyrosine kinase, ROR2, in human carcinogenesis.

The Wnt signaling pathway is involved in the development and progression of many human cancers, yet attempts to target the pathway therapeutically have been disappointing to date. The recent discovery that the ROR2 receptor tyrosine kinase (RTK) is a novel Wnt receptor provides the potential to target the non-canonical Wnt pathway for cancer treatments. As a member of the RTK superfamily of surface receptors ROR2 appears to possess dual roles as a tumor suppressor or activator depending on tumor type. This review will explore the dual role of ROR2 in tumorigenesis and provide an up to date analysis of current literature in this rapidly expanding field.