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Updates in Pediatrics
Editor: Jack Wolfsdorf, MD, FAAP
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November 3, 2021 | Volume 12 | Issue 44 |
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Lung function in children with Cystic Fibrosis (CF) |
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CF is a progressive autosomal recessive inherited disorder that causes severe damage primarily to the lungs and gastrointestinal systems (but also affects other organs). Children with CF have mutations in the cystic fibrosis trans-membrane conductant regulator (CFTR) gene which interferes with chloride and water movement to the surface of cells.
The CF gene abnormality results in less water in secreted mucus causing clogging of the lungs, pancreas, male reproductive system, etc. Clinical/severity of presentation varies however most children have recurrent pulmonary infection needing life-long therapy. Many live into their 30s or 40s; death occurring primarily due to respiratory failure.
Two Scandinavian studies in 5 specialized CF centers done 10 years apart on 629 and 738 CF patients respectively with pancreatic insufficiency only, which examined over time demographic data, lung function and infectious status indicates that mean age increases by 2.9 years (to 20.6 years), chronic P. aeruginosa infection falls from 44.7% to 36.3% and lung function remains unchanged (Mean FEV1 in children normal: >80%).
“Maintaining lung function in children with CF is possible with aggressive treatment”.
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Read full article at Acta Paediatrica
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Laxative and cathartic use during breastfeeding
“Constipation is not uncommon in nursing mothers especially during the immediate post-partum period…”
A short article outlines the actions, benefits and side-effects of Docusate, Magnesium hydroxide (Milk of magnesia), Hydrocolloid (and others) in the lactating mother and breast feeding infant.
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Co-occurrence of neurodevelopmental disorders (NND) in pediatric Sickle Cell Disease (SCD) |
Sickle cell disease is an inherited disease of hemoglobin/red cells that causes the cells to be stiff, half-moon-shaped and to clump together. This results in hemolysis, chronic anemia and vaso-occlusive disease with resultant pain (“Painful or Sickle crisis”) and organ involvement including acute chest syndrome, frequent infections, delayed growth, blindness, brain problems (ischemic/hemorrhagic stroke) and cognitive impairment.
A retrospective study of 276 children with sickle cell disease, <18 years of age (55 with neurodevelopmental disorders) determined the co-occurrence associated characteristics and risk factors for NDD.
Children with SCD and neurodevelopmental disorders are more likely to have a history of multiple SCD related complications and used disease-modifying therapies.
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Upper gastrointestinal (GI) endoscopy with biopsy in pediatric feeding disorders |
A study to evaluate upper GI pathology among children seen for persistent feeding problems (and compared to a matched unaffected control group) indicates that while 57% on esophagogastroduodenoscopy (EGD) biopsy have abnormal endoscopic findings (higher than most studies! – 15% eosinophilic esophagitis vs. 8% control), overall there is a similar prevalence of abnormality between affected and control groups.
As eosinophilic esophagitis is not uncommonly found on biopsy/microscopy in children with unresolved feeding disorders, EGD and biopsy may be warranted.
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Links between prenatal adversities and adolescent psychiatric risk |
“Adverse prenatal and postnatal exposures may have long-lasting effects on health and development”. It remains unclear whether being exposed to a greater number of prenatal adversities affects mental health to a greater extent.
Data from the 2014 Ontario Child Health Study (OCHS) assessed the association between 7 prenatal risk factors (maternal hypertension, diabetes mellitus, bleeding during pregnancy, influenza, urinary tract infection, thyroid disease and depression/anxiety) and adolescent (12-17 years olds) psychiatric morbidity (using the Mini-International Neuropsychiatric Interview for Children and Adolescents).
Each additional prenatal adverse exposure significantly increases the odds of attention/deficit/hyperactivity disorder, generalized anxiety disorders, and social anxiety disorders (after adjusting for confounding variables) in adolescents.
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Transmission of SARS-CoV-2 after screening and mitigation measures for primary school children |
A prospective cohort study 63 children and 118 adult volunteers examined the possible role of children in SARS-CoV-2 transmission. Participants were tested for SARS-CoV-2 infection once per week (using verse transcription–polymerase chain reaction (RT-PCR); 24.9% tested positive (13 children and 32 adults). Despite the implementation of several mitigation measures the incidence of COVID-19 among children (46.2% asymptomatic) was comparable to that observed among teachers and parents (12.9% asymptomatic).
“Children may play a larger role in the transmission of SARS-CoV-2 than previously assumed”.
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Hyperinflammation: On the pathogenesis and treatment of macrophage activation syndrome (MAS) |
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The immune system is the body’s defense system to fight virus-infected cells, malignant cells and to prevent exaggerated immune responses. One of the cells involved is the macrophage which not only destroys/ingests viruses/bacteria (hemophagocytosis) but also controls other cells in the immune system; T lymphocytes and cytokines also play a significant role. In children with macrophage activation syndrome (MAS), a subtype of Hemophagocytic lymphohistiocytosis (which may be Primary/genetic/familial or Secondary to a systemic disease like autoimmune disease, malignancy or infections like EB virus), the immune system response is uncontrolled leading to a hyper-inflammatory cytokine storm which can affect any organ system.
Clinically, “MAS is characterized by the rapid onset of overwhelming inflammation with unremitting fever, hepatosplenomegaly, liver dysfunction, lymphadenopathy, encephalopathy, purpura and bleeding” which can go on to life-threatening multiorgan failure. Early recognition is crucial and there no specific diagnostic test to differentiate MAS from any other systemic inflammatory disorder. Treatments are aimed at pacifying the inflammatory storm.
An interesting article outlines MAS pathogenesis, clinical and laboratory findings and therapeutic options (Well worth taking the time to read this complex article, ED).
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