May 26, 2021 | Volume 12 | Issue 21
Posterior Reversible Encephalopathy Syndrome (PRES) in childhood hematological/
oncological diseases
“PRES is a novel clincoradiological syndrome characterized by convulsions, headache, altered mentality and impaired vision usually accompanied by hypertension.” Typical neuroimaging shows vasogenic edema mainly involving the posterior brain. While PRES is frequently associated with hematological/oncological conditions and related therapies, it can occur with renal insufficiency, collagen vascular disease, immunodeficiency syndromes and others.

A study of 58 children (mean age 8.95 years) describes the characteristics of PRES and indicates that most (80.4%) have a primary diagnosis of acute leukemia, have been recently exposed to chemotherapy (71.4%) and/or steroids (85.7%) with a majority (83.9%) having hypertension. Multiple electrolyte abnormalities are identified and magnetic resonance imaging (MRI) of the brain shows involvement of the occipital (58%) parietal (51%) and frontal lobe (45%) respectively.
Neonatal cholestasis (NC): Dubin-Johnson Syndrome (DJS)
Cholestasis is defined as an impairment in the excretion of bile which can be caused by defects in intrahepatic production of bile, transmembrane transport of bile or mechanical obstruction to bile flow.” An elevated conjugated (Direct) hyperbilirubinemia is the predominant characteristic in most of the multiple causes of neonatal/young infant cholestasis (present longer than 2 weeks after birth and within the first months of life) and which includes obstructive, infectious, genetic, metabolic, autoimmune and toxic causes.

Dubin-Johnson Syndrome is a rare, benign usually asymptomatic autosomal recessive disorder (mostly seen during puberty or later in Middle Eastern, Jewish and Japanese people) of the ABCC2 gene which encodes for a protein called “multidrug resistant protein 2 (MRP2)”, the deficiency of which results in an excretion disorder of conjugated bilirubin in the liver cell.

A study compared 4 DJS patients to 26 patients with biliary atresia (BA) to identify specific characteristics of DJS and to highlight diagnostic tools available.

Infants with DJS neonatal cholestasis have lower values of aspartate aminotransferase (AST) alanine aminotransferase (ALT) and gamma-glutamyl transferase (GGT) vs. those with biliary atresia. DJS though rare should be considered in every infant with NC and an atypical enzyme pattern.


See related video HERE & below:
Evaluation of topical cortisteroid use in pregnancy and the risk of newborns being small for gestational age (SGA)
A study of 60,497 paired and matched corticosteroid exposed to unexposed pregnancies, investigated the association between topical steroid use during pregnancy and the risk of offspring being SGA and low birth weight.

There appears to be no association between topical corticosteroid use in pregnancy (even large amounts of potent ones) and an increased risk of SGA or low birth weight in offspring.
Association of positional Plagiocephaly and neurodevelopment
“It is relatively common for infants and children to present to primary care services with abnormal skull shapes.” Mostly the abnormal head shape (Plagiocephaly) is positional and benign (some deformities however are caused by craniostenosis which should be diagnosed early and surgically treated). Previous studies evaluated neurodevelopment in children seen at Subspecialty clinics (with potential confounding factors of selection and referral biases). This retrospective study of children (2,315 diagnosed with Plagiocephaly at <12 months of age) from 45 Primary Care sites examined whether the association between Plagiocephaly and developmental delay could be confirmed.

Infants <1 year of a age diagnosed with Plagiocephaly from Primary Care settings have an increased risk (1.50 odds ratio) of developmental delay after adjusting for multiple covariates.


See related video HERE & below:
Plagiocephaly and its treatment | Boston Children’s Hospital
Efficacy and safety of breast milk eye drops in infants with eye discharge
“To prevent neonatal conjunctivitis (Ophthalmia Neonatorum) State Law requires most hospitals to put drops or ointment in a newborn’s eye.” Previously silver nitrate was used, now hospitals use an antibiotic, often erythromycin.

“Breast Milk (BM) contains various protective components like Immunoglobulins, lactoferrin, lysozyme, oligosaccharides and immune cell subsets.”

A randomized control study of 2 groups each of 119 breastfed infants <180 days of age with eye discharge who received either eye drops of breast milk or sodium azulene sulfonate hydrate (OS – 0.02%; a water-soluble anti-inflammatory/anti-allergic natural product eye drop used in Japan) for 7 days, examined the effectiveness of BM as an eye drop for infants with eye discharge.

As 76.8% and 75.8% of infants respectively (BM vs. OS) improved, breast milk could be considered as a first-line treatment for an eye discharge in infants <6 months of age.
Ferric carboxymaltose (FCM) across all ages in pediatric gastroenterology for correcting iron deficiency anemia (IDA)
Iron deficiency anemia is common worldwide; the most common causes include insufficient intake together with rapid growth, low birth weight and gastrointestinal loss related to excessive intake of cow’s milk/blood loss. Management includes investigation and elimination of the cause, replacement of deficiency, improvement of nutrition and family/child education.

Ferric carboxymaltose (Ferinject®) is a novel iron complex that consists of ferric hydroxide stabilized by a carbohydrate shell which when given intravenously (to adults) rapidly improves hemoglobin levels given to patients for whom oral iron preparations are ineffective or cannot be administered.

A retrospective study of 61 children form a pediatric gastroenterology clinic (<18 years of age; 42% < 14 years old; 11.5% <5 years of age) 28% of whom were switched from an oral iron supplement to FCM, assessed FCM efficacy (19mg/kg) safely and side-effects (to 1 year post infusion).

In children with IDF across a wide range of gastroenterology indications (and for all ages) FCM corrects IDA (94%) and increases hemoglobin levels from 10.8 to 12g/dl at 1 month post infusion. 3% of children have side effects which may be avoided by careful monitoring during drug infusion.


See related video HERE, HERE & below:
USDA infant feeding guidelines: 2020-2025
An extensive publication outlines the latest US Department of Agriculture (USDA) feeding guidelines for infants/toddlers. In very brief summary:

1.) Babies should be breastfeed until 4-6 months of age (plus vitamin D supplements – if breastfeeding is not possible infants should be fed iron fortified infant formula)

2). Potentially allergenic foods should be fed early and often

3.) Diet diversity is critical

4.) “Make every bite count”

5.) Pay attention to beverages: No beverages outside human milk/formula and water (no cow’s milk in first year of life. No fruit juices)
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Dear Reader:

I am sure many of you, like I, are feeling great relief at the improving outcomes following the various public health measures and SARS-CoV-2 immunization programs being undertaken in the USA to date. It seems that while there are many unanswered questions remaining, and firm answers not yet available on many interesting aspects, at present there is significant optimism that the mRNA vaccines will provide not only substantial (but not total) protection against the worst results of COVID-19 but will also minimize spread while appearing to be effective against the variants circulating.

Nevertheless I would urge all to remember that there are places and situations that place us at increased risk for infection, like hospitals, nursing homes, prisons and homeless shelters or while traveling. Some immunocompromised people will remain vulnerable to COVID-19. Continue to take care, wear a mask when appropriate, in addition wash hands or use a sanitizer when in risky situations, continue to cough or sneeze into ones elbow and be aware and cautious in balancing risks and benefits.
 
Best of luck.

Jack Wolfsdorf, MD, Editor
Staff NCH
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