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Issue highlights—November 2021
Anna Käll,Hugo Lagercrantz
doi : 10.1111/apa.16109
Volume 110, Issue 11 p. 2886-2887
Langerhans cell histiocytosis: A malignant myeloid neoplasm or disorder of immune regulation?
Jenee Mitchell,George Kannourakis
doi : 10.1111/apa.15995
Volume 110, Issue 11 p. 2888-2891
Clinical features and treatment of Langerhans cell histiocytosis
Carlos Rodriguez-Galindo
doi : 10.1111/apa.16014
Volume 110, Issue 11 p. 2892-2902
Langerhans cell histiocytosis (LCH) is caused by the expansion of CD1a+/CD207+ cells and is characterised by a wide spectrum of organ involvement and dysfunction, affecting all ages. While almost all organs and systems can be affected, only the involvement and dysfunction of liver, spleen, and haematopoietic system influence survival. The LCH pathogenic cells are defined by universal activation of the mitogen-activated protein kinase (MAPK) signalling pathway. The most common alteration is a somatic BRAFV600E mutation, which is present in approximately two-thirds of the cases, followed by MAP2K1 mutations. Treatment of LCH is risk-adapted; patients with single lesions may respond well to local treatment, whereas patients with multi-system disease require systemic chemotherapy. While survival for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 20%. Despite progress made in the treatment of LCH, disease reactivation rates remain above 30%, and standard second-line treatment has yet to be established. Long-term effects, including neuroendocrine dysfunction and neurodegeneration, represent a major challenge for survivors. Treatment with BRAF or MEK inhibitors results in immediate responses, but reactivations are very common after discontinuation. Their role as single agents and in combination with chemotherapy is being explored.
Genetics and pathophysiology of haemophagocytic lymphohistiocytosis
Marie Meeths,Yenan T. Bryceson
doi : 10.1111/apa.16013
Volume 110, Issue 11 p. 2903-2911
Haemophagocytic lymphohistiocytosis (HLH) represents a life-threatening hyperinflammatory syndrome. Familial studies have established autosomal and X-linked recessive causes of HLH, highlighting a pivotal role for lymphocyte cytotoxicity in the control of certain virus infections and immunoregulation. Recently, a more complex etiological framework has emerged, linking HLH predisposition to variants in genes required for metabolism or immunity to intracellular pathogens. We review genetic predisposition to HLH and discuss how molecular insights have provided fundamental knowledge of the immune system as well as detailed pathophysiological understanding of hyperinflammatory diseases, highlighting new treatment strategies.
Kostmann disease and other forms of severe congenital neutropenia
Bengt Fadeel,Daniel Garwicz,Göran Carlsson,Bengt Sandstedt,Magnus Nordenskjöld
doi : 10.1111/apa.16005
Volume 110, Issue 11 p. 2912-2920
Congenital neutropenia with autosomal recessive inheritance was first described by the Swedish paediatrician Rolf Kostmann who coined the term ‘infantile genetic agranulocytosis’. The condition is now commonly referred to as Kostmann disease. These patients display a maturation arrest of the myelopoiesis in the bone marrow and reduced neutrophil numbers and suffer from recurrent, often life-threatening infections. The molecular mechanism underlying congenital neutropenia has been intensively investigated, and mutations in genes that impinge on programmed cell death have been identified. The present review provides an overview of these studies.
Utilising recorded music to reduce stress and enhance infant neurodevelopment in neonatal intensive care units
Nicole Mikulis,Terrie E. Inder,Carmina Erdei
doi : 10.1111/apa.15977
Volume 110, Issue 11 p. 2921-2936
This paper summarises published evidence on the use of recorded music in high-risk infants to reduce stress and improve neurodevelopment, forming recommendations for proposed clinical applications in neonatal intensive care units.
Intracranial haemorrhage associated with Vitamin K deficiency in Egyptian infants
Mohsen Elalfy,Khadiga Eltonbary,Omar Elalfy,Mohsen Gadallah,Ashraf Zidan,Hesham Abdel-Hady
doi : 10.1111/apa.16011
Volume 110, Issue 11 p. 2937-2943
Intracranial haemorrhage (ICH) in infancy is a rare life-threatening event. The aim of this review is to highlight the association of ICH and potentially preventable vitamin K deficiency and to describe risk factors, presentation and outcome.
Neurodevelopmental and psychiatric comorbidities negatively affect outcome in children with unprovoked seizures—A non-systematic review
Eva Åndell Jason
doi : 10.1111/apa.16026
Volume 110, Issue 11 p. 2944-2950
Children with epilepsy have at least one comorbidity in 80% of cases. This unstructured review provides insights into the most common comorbidities, their effects on seizure prognosis and treatment. We also review the epilepsy terminology and classifications. Neurodevelopmental and psychiatric comorbidities were common in children with seizures and had a negative effect on quality of life, and seizure freedom. Children with seizures were treated with drugs used for attention deficit hyperactivity disorder (ADHD), depression or psychosis, more often than the general population but less often than prevalence rates would suggested.
Mother-Newborn Couplet Care from theory to practice to ensure zero separation for all newborns
Stina Klemming,Siri Lilliesköld,Björn Westrup
doi : 10.1111/apa.15997
Volume 110, Issue 11 p. 2951-2957
With an increasing awareness of the importance of nurturing care and within a framework of Infant- and Family-Centred Developmental Care (IFCDC), zero separation, keeping parent and infant in continuous close physical and psychological proximity to each other, is key. In modern neonatology, high technological and pharmaceutical treatments are consistently integrated with caregiving considerations. Mother-Newborn Couplet Care is a concept of care where the dyad of the ill or prematurely born infant and the mother, needing medical care of her own, are cared for together, from the birth of the baby to its discharge. Mother-Newborn Couplet Care requires systems changes in both obstetrics and paediatrics considering planning and organisation of care, equipment and design of units. Accordingly, strong leadership setting clear goals and changing the professional mindset by providing targeted education and training is crucial to ensure the warranted high quality of care of all mother-baby dyads.
The jury is still out on the existence of a placental microbiome
Despina D. Briana,Vassiliki Papaevangelou,Ariadne Malamitsi-Puchner
doi : 10.1111/apa.16048
Volume 110, Issue 11 p. 2958-2963
The human microbiome is crucial for regulating normal development, but the exact point when it is established remains unknown. A sterile placenta was traditionally considered a prerequisite for a healthy pregnancy, but studies have revealed that the placenta harbours microbial communities, even under normal conditions. However, reports have failed to provide evidence for the consistent presence of bacteria in the normal human placenta, challenging the in utero colonisation hypothesis. This mini review examines our understanding of the potential placental microbial colonisation in normal healthy pregnancies. This may impact the metabolic and immune functions of the growing foetus and have long-term consequences.
The moral and religious obligation to vaccinate children in Jewish ethics
Tsuriel Rashi
doi : 10.1111/apa.16024
Volume 110, Issue 11 p. 2964-2967
Retrospective observational study of neonatal attendances to a children's emergency department
Sarah J. Blakey,Mark D. Lyttle,Dan Magnus
doi : 10.1111/apa.16039
Volume 110, Issue 11 p. 2968-2975
Attendances to emergency departments (EDs) in the UK are increasing, particularly for younger children. Community services are under increasing pressure and parents may preferentially bring their babies to the ED, even for non-urgent problems. This study aimed to characterise the presenting features, management and disposition of neonatal attendances to a children's ED (CED).
Continuous positive airway pressure treatment may negatively affect auditory maturation in preterm infants
Jaana Antinmaa,Jaakko Salonen,Satu K. Jääskeläinen,Anne Kaljonen,Helena Lapinleimu
doi : 10.1111/apa.16029
Volume 110, Issue 11 p. 2976-2983
Nasal continuous positive airway pressure (CPAP) devices generate loud noise, which might harm auditory function and maturation. The function of auditory pathways can be examined by using brainstem auditory evoked potential (BAEP) and brainstem audiometry (BA) recordings. Our objective was to study whether CPAP treatment during the neonatal period is associated with abnormalities in BAEP and BA recordings.
Mental health monitoring in parents after very preterm birth
Eline Vriend,Aleid Leemhuis,Monique Flierman,Petra van Schie,Frans Nollet,Martine Jeukens-Visser
doi : 10.1111/apa.16064
Volume 110, Issue 11 p. 2984-2993
To evaluate parental mental health monitoring during follow-up care for very preterm (VPT) infants, describe symptoms of anxiety and depression and risk factors for mothers and fathers at 1 and 12 months of corrected age.
Extremely low birthweight neonates with phenylketonuria require special dietary management
Marketa Zemanova,Petr Chrastina,Vaclav Sebron,Dagmar Prochazkova,Helena Jahnova,Petra Sanakova,Lucie Prochazkova,Barbara Tesarova,Jiri Zeman
doi : 10.1111/apa.16035
Volume 110, Issue 11 p. 2994-2999
Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants.
Asymptomatic full-term infants born to women with chorioamnionitis may not need routine antibiotics
Nakul Kothari,Vanessa Dsouza,Umesh Mishra,Rajesh Maheshwari,Dharmesh Shah,Daphne D’Cruz,Jane Baird,Melissa Luig,Pranav Jani
doi : 10.1111/apa.16059
Volume 110, Issue 11 p. 3000-3005
While infants with early-onset sepsis require antibiotics, there is little evidence to support their routine use in asymptomatic infants exposed to maternal chorioamnionitis. We aimed to ascertain the incidence of culture-proven sepsis in full-term infants exposed to chorioamnionitis and to determine whether asymptomatic infants need routine antibiotic treatment.
The value of laboratory parameters in predicting the progression of necrotising enterocolitis
Ali Ahmed Raba,John Coleman,Katie Cunningham,Margaret M. Moran
doi : 10.1111/apa.16047
Volume 110, Issue 11 p. 3006-3008
Paediatric residents deliver similar quality simulated neonatal resuscitation using 3:1 and 15:2 ratios
Aida Carballo-Fazanes,Javier Trastoy-Quintela,Cristina Rey-Noriega,Laura Sánchez-Rodríguez,Antonio Rodríguez-Núñez
doi : 10.1111/apa.15991
Volume 110, Issue 11 p. 3009-3010
Plasma secretory phospholipase A2 as an early marker for late-onset sepsis in preterm infants—a pilot study
Julie Hibbert,Nicola J. Armstrong,Caitlyn Granland,Sherrianne Ng,Karen Simmer,Peter Richmond,David Burgner,Tobias Strunk,Andrew Currie
doi : 10.1111/apa.15969
Volume 110, Issue 11 p. 3011-3013
Preterm infants are particularly susceptible to bacterial late-onset sepsis (LOS). Diagnosis by blood culture and inflammatory markers have sub-optimal sensitivity and specificity and prolonged reporting times. There is an urgent need for more rapid, accurate adjunctive diagnostics in LOS to improve management and minimise antibiotic exposure. We measured the diagnostic performance of secretory phospholipase A2 type IIA (sPLA2-IIA) in very preterm infants (<30 weeks gestational age) with suspected LOS. Plasma sPLA2-IIA levels were elevated in infants with LOS (n = 28) compared to those without LOS (n = 21; median 30,970 vs. 2534 pg/ml, p < 0.0001). The mean area under the curve was 0.884 (95% CI: 0.771, 0.977) with a sensitivity of 0.907 (95% CI: 0.667, 1.00) and specificity of 0.804 (95% CI: 0.600, 1.00). The positive and negative predictive values were 0.833 (95% CI: 0.664, 0.927) and 0.842 (95% CI: 0.624, 0.945), respectively. This pilot study suggests that sPLA2-IIA may have clinical utility for the early diagnosis of LOS in very preterm infants, potentially informing clinical management and antibiotic stewardship.
Foetal mouth movements: Effects of nicotine
Suzanne Froggatt,Nadja Reissland,Judith Covey,Kumar Kumarendran
doi : 10.1111/apa.16042
Volume 110, Issue 11 p. 3014-3020
To assess whether foetal mouth movement frequency changes across gestation and whether there are differences between cigarette and e-cigarette exposure conditions in comparison to a non-exposed group of foetuses.
Health perception by young adults born very preterm
Camille Girard-Bock,Adrien Flahault,Élisabeth Bernard,Claude Julie Bourque,Catherine Fallaha,Anik Cloutier,Annie Janvier,Anne Monique Nuyt,Thuy Mai Luu
doi : 10.1111/apa.16056
Volume 110, Issue 11 p. 3021-3029
This study assessed the self-reported health perception and use of health care by adults born very preterm before 30 weeks of gestation.
The ability of Bayley-III scores to predict later intelligence in children born extremely preterm
Johanna Månsson,Karin Källén,Eva Eklöf,Fredrik Serenius,Ulrika Ådén,Karin Stjernqvist
doi : 10.1111/apa.16037
Volume 110, Issue 11 p. 3030-3039
To investigate the ability of the Bayley Scales of Infant and Toddler Development—Third Edition (Bayley-III), scores to predict later Wechsler Intelligence Scale for Children—Fourth Edition (WISC-IV), performances in a cohort of children born extremely preterm.
Growth assessment of children during the COVID-19 pandemic—Can we rely on parental measurements?
Ariel Tenenbaum,Noa Shefer-Averbuch,Liora Lazar,Michal Yakobovitch-Gavan,Moshe Phillip,Tal Oron
doi : 10.1111/apa.16034
Volume 110, Issue 11 p. 3040-3045
The COVID-19 pandemic prompted the rapid development of remote medical services. During lockdown periods, children's growth data were obtained from parents’ home assessments. This study aimed to assess the accuracy of home height and weight measurements and analyse their utility in clinical decision-making.
Exposure to electronic media was negatively associated with speech and language development at 18 and 24 months
Marja Asikainen,Anneli Kylliäinen,Tiina E. Mäkelä,Outi Saarenpää-Heikkilä,E. Juulia Paavonen
doi : 10.1111/apa.16021
Volume 110, Issue 11 p. 3046-3053
This study evaluated early speech and language development at 18 and 24 months, and associated factors, based on parental reports.
SARS-CoV-2 antibodies started to decline just four months after COVID-19 infection in a paediatric population
Adin Breuer,Allon Raphael,Hagay Stern,Ma'aran Odeh,Judith Fiszlinski,Nurit Algur,Sophie Magen,Orli Megged,Yechiel Schlesinger,Yuval Barak-Corren,Eyal Heiman
doi : 10.1111/apa.16031
Volume 110, Issue 11 p. 3054-3062
We evaluated the prevalence of paediatric severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections using antibody testing and characterised antibody titres by time from exposure.
Multi-inflammatory syndrome and Kawasaki disease in children during the COVID-19 pandemic: A nationwide register-based study and time series analysis
Ulla Koskela,Otto Helve,Emmi Sarvikivi,Merja Helminen,Tea Nieminen,Ville Peltola,Marjo Renko,Harri Saxén,Hanna Pasma,Tytti Pokka,Minna Honkila,Terhi Tapiainen
doi : 10.1111/apa.16051
Volume 110, Issue 11 p. 3063-3068
We investigated whether the ongoing COVID-19 pandemic was associated with the occurrence of Kawasaki disease or with multi-inflammatory syndrome in children (MIS-C).
Age- and sex-specific prevalence of serious bacterial infections in febrile infants ?60 days, in Sweden
Ioannis Orfanos,Tobias Alfvén,Maria Mossberg,Mattias Tenland,Jorge Sotoca Fernandez,Erik A. Eklund,Kristina Elfving
doi : 10.1111/apa.16043
Volume 110, Issue 11 p. 3069-3076
The aim of the study was to describe age- and sex-specific prevalence of serious bacterial infections (SBI: urinary tract infection, bacteraemia, meningitis) among febrile infants ?60 days in Sweden.
Children may need higher vancomycin doses to achieve therapeutic levels
Kristin Oskarsdottir,Asgeir Haraldsson,Thordur Thorkelsson,Thorunn Oskarsdottir,Petur Gunnarsson,Valtyr Thors
doi : 10.1111/apa.16025
Volume 110, Issue 11 p. 3077-3082
Vancomycin is frequently used in paediatric hospitals. Data suggest trough levels of 10–20 mg/L are needed to achieve bacterial killing. This study aimed to evaluate if commonly used dosing regimens are efficient in reaching these levels and if therapeutic drug monitoring (TDM) was appropriately used.
Challenges parents encounter when implementing infant safe sleep advice
Roni Cole,Jeanine Young,Lauren Kearney,John M. D. Thompson
doi : 10.1111/apa.16040
Volume 110, Issue 11 p. 3083-3093
To understand which safe sleep recommendations parents find most challenging to implement, identifying common barriers encountered; and investigate whether challenges are associated with practices employed.
Poor parental sleep did not predict future sleep problems in children aged 2–6 years
Hanni Rönnlund,Marko Elovainio,Irina Virtanen,Anna-Riita Heikkilä,Hanna Raaska,Helena Lapinleimu
doi : 10.1111/apa.15984
Volume 110, Issue 11 p. 3094-3096
Self-reported physical activity and sedentary behaviour amongst adolescents in Sweden vary depending on sex, age and parental education
Andreas Fröberg,Karin Kjellenberg,Anna Karin Lindroos,Gisela Nyberg
doi : 10.1111/apa.16077
Volume 110, Issue 11 p. 3097-3104
The aim of this study was to investigate self-reported physical activity and sedentary behaviour by sex, age and parental education in a large representative sample of Swedish adolescents.
Sudden cardiac death in childhood hypertrophic cardiomyopathy is best predicted by a combination of electrocardiogram risk-score and HCMRisk-Kids score
Ingegerd Östman-Smith,Gunnar Sjöberg,Jenny Alenius Dahlqvist,Per Larsson,Eva Fernlund
doi : 10.1111/apa.16045
Volume 110, Issue 11 p. 3105-3115
To compare risk algorithms (HCMRisk-Kids, ECG Risk-score) in hypertrophic cardiomyopathy (HCM) without syndrome association (ns-HCM) and with Noonan-like syndromes (RAS-HCM).
Adrenal androgen trajectories are established during childhood in preterm boys
Kjersti Kvernebo Sunnergren,Ann-Katrine Karlsson,Kerstin Allvin,Staffan Nilsson,Carina Ankarberg-Lindgren,Jovanna Dahlgren
doi : 10.1111/apa.16036
Volume 110, Issue 11 p. 3116-3123
We investigated longitudinal adrenal androgen concentrations and any relationship between gestational age, birth size, anthropometric parameters and adrenal androgen concentrations during childhood in boys born moderate to late preterm.
Linguistic and content validity of the Swedish version of the PedsQL™ gastrointestinal symptoms scales and symptoms module for paediatric patients
Matilda Bräutigam,Michaela Dellenmark-Blom,Vladimir Gatzinsky,Jenny Bergström,Kate Abrahamsson
doi : 10.1111/apa.16066
Volume 110, Issue 11 p. 3124-3130
To describe the process of linguistic and content validity of the Swedish version of the PedsQL™ Gastrointestinal Symptoms Scales and Symptoms Module, measuring health-related quality of life (HRQOL) in children with gastrointestinal (GI) disorders.
Attention deficit hyperactivity and autism spectrum disorders in patients with anorectal malformations
Tomas Wester,Anna Gunnarsdottir,Charlotte Skoglund,Anna Svenningsson
doi : 10.1111/apa.16100
Volume 110, Issue 11 p. 3131-3136
To assess the risk of neurodevelopmental disorders in patients with anorectal malformations compared to controls.
Virus antibodies after SARS-CoV-2 infection
Rujittika Mungmunpuntipantip,Viroj Wiwanitkit
doi : 10.1111/apa.16058
Volume 110, Issue 11 p. 3137-3137
Low-grade intraventricular haemorrhage is not as harmless as first thought when born extremely preterm
Ranveer Sanghera,Jonathan Cusack
doi : 10.1111/apa.15975
Volume 110, Issue 11 p. 3138-3139
Does fluid balance affect neurodevelopmental outcomes in hypoxic-ischaemic encephalopathy?
Sreekruthi Bachu,Gopakumar Hariharan
doi : 10.1111/apa.15992
Volume 110, Issue 11 p. 3140-3141
