June 30, 2021 | Volume 12 | Issue 26
A review of the latest research on Kingella Kingae (KK): The importance of molecular testing
KK is a gram negative beta-hemolytic encapsulated coccobacillus belonging to the Neisseriaceae family, frequently colonizing the oropharynx of healthy infants between 6 and 48 months of age. The pathogen is transmitted by close contact between young children (particularly in daycare centers) and is associated with invasive blood borne disease which most frequently involves bone and joints (but may also infect soft tissues, e.g., heart (endocarditis) meninges and eyes).

KK is fastidious, slow growing and notoriously difficult to culture from synovial fluid and bone aspirate. Molecular tests (nucleic acid amplification – NAA; polymerase chain reaction - PCR etc.) significantly improve detection. KK is generally susceptible to Beta Lactam antibiotics (a review worth reading. ED).
Neurobehavior of very preterm infants (VPI) at term equivalent age and neurodevelopment at 4 years of age
From a study of 48 infants born <32 weeks gestation who had neurobehavior assessed at term equivalent and neurodevelopment outcomes measures at 4 year of age it appears that:

  1. 31-48% have at least 1 below average score on questionnaires.
  2. Neonates with poorer orientation, self-regulation, hypertonia and sub-optimal reflexes have lower fine motor scores at 5 years.
  3. Neonates with sub-optimal reflexes and lethargy have lower gross motor scores at 4 years.
  4. Neonates with poorer orientation and tolerance of handling have increased tactile sensitivity at 4 years of age.
  5. Neonates with low arousal are less responsive; and hypotonia as a neonate affects auditory filtering at 4 years of age.

“Early neurobehavior is related to neurodevelopmental outcome in early childhood.”


See related video below:
Terrie Inder, MD, MBChB, Chair of the Department of Pediatric Newborn Medicine at Brigham and Women’s Hospital
Severe aplastic anemia (SAA) in children; Impact of histopathology profile and treatment on very long-term outcomes
Aplastic anemia is a type of bone marrow failure that frequently involves all 3 blood cell types (red and white cells and platelets – pancytopenia) resulting in anemia, and inability to fight infections and unexplained, easy bruising, nose bleeds, etc. In most cases the cause is unknown however some are of genetic origin, others secondary to a viral infection, autoimmune disorder, malignancy, radiation or certain toxic chemicals and some antibiotics etc. Diagnosis involves not only a complete medical history and examination but also blood tests (hemoglobin, complete blood count peripheral smear) and bone marrow aspiration/biopsy.

A retrospective study of 57 consecutive children with SAA examined response rates, survivorship, histopathology and treatment (androgens, immunosuppression and hematopoietic cell transplantation – HCT).

Overall survival of SAA (from what was once a fatal disease) has improved on immunosuppression or HCT from 35.3% to 77%.
Atypical general movements in the general population
General movements (GMs) are distinct spontaneous movement patterns (normally short complex movements) involving all parts of the body in the fetus and after birth to 20 weeks of corrected age. Assessment of the quality of GMs evaluates early neurological function and identifies infants as high risk for developmental problems e.g., cerebral palsy.

Normal fidgety movements (FMs) are typically tiny, elliptical in form, smooth and of small amplitude and moderate speed in all directions of the neck, trunk and limbs in the calm, alert awake infant. Virtually all infants develop normally if FMs are present and normal. (Abnormal fidgety movements are those whose amplitude, speed and jerkiness are greatly exaggerated and occur after 20 weeks of life. “Absent FMs” are never normal from 9-20 weeks post term). 

The predictive value of GMs is particularly powerful when it is preform at 4-20 weeks corrected age during the so-called age dependent “fidgety phase.”

A representative sample of 300 Dutch infants (gestational age 39.4 weeks corrected age) assessed GMs at 8-16 weeks to determine the prevalence of atypical general movements in the general population and the presence/absence of fidgety movements.

“In the general population, the prevalence of definitely abnormal general movement complexity and absent fidgety movements is 3%.” Abnormality appears associated with maternal smoking and prematurity (marginally). General movement complexity and fidgety movements appears to be based on different neurobiological mechanisms – fidgety movement abnormalities/absence being more associated with preterm very preterm birth.

(>50% of infants with a combination of abnormal general movement complexity and no fidgety movements are later diagnosed with Cerebral Palsy).


See related video HERE, HERE & HERE.
Management and course of eosinophilic esophagitis (EoE)
EoE is a chronic allergic inflammatory esophageal disease that affects children of all ethnicities and family income level. Clinical presentation frequently includes (all or some of) nausea, vomiting, abdominal pain, “heart burn,” difficulty swallowing and gaging (infants may not want to breast/bottle feed, spit up and arch their backs in pain).

A retrospective study of 102 children diagnosed with EoE evaluated their response (clinical, endoscopic and histological) to 3 treatment options i.e., proton pump inhibitors (PPIs), an elimination diet and topical steroids.

55%, 75% and 87.5% of children with EoE improve clinically on PPIs, diet and budesonide respectively. While there is some correlation between symptoms and endoscopic and histological appearances clinical response cannot be relied upon to define endoscopic/histologic appearance. Topical steroids appear to be the most effective treatment. Repeated endoscopies and biopsies are mandated.

Vitamin D supplementation as an adjunct to standard treatment of asthma in children
Vitamin D (1.25 (OH) 2D) is involved in metabolic processes that affect bone growth and mineralization through regulation of calcium and phosphorous metabolism. In addition, 5 biological systems have vitamin D receptors (immune system, pancreas, cardiovascular, muscle and brain) which locally generate vitamin D. The lung’s airway epithelium, alveolar macrophages and other immune responses are affected by vitamin D. Asthma is an immunological based allergic disease which has increased in prevalence over the same time period that vitamin D insufficiency has increased.

A placebo-controlled, blinded, randomized controlled trial of 60 children (ages 6-11 years) with moderate persistent asthma who received either 2,000IU/day vitamin D (or placebo) assessed its effect on asthma control/lung function, systemic steroid use, Emergency Department usage, post-intervention vitamin D levels and adverse outcomes.

Vitamin D supplementation does not improve asthma control (or any of the secondary outcomes) in children.


See related video HERE and below:
Vitamin D supplementation during pregnancy and the prevention of childhood asthma
Reproductive health counseling and contraceptive use in adolescents with Cystic Fibrosis (CF)
Children born between 2015 and 2019 with a diagnosis of CF (a genetic, progressive disease) are predicted to live to 46 years; with those born in 2019, 50% may survive to 48 years or longer and this data doesn’t reflect the potential benefit of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy or the ongoing improvement in specialized CF care being undertaken. In CF diagnosed women of child bearing age this disease poses reproductive health concerns like high-risk pregnancies and medication interactions leading to contraceptive failure.

A retrospective chart review of 16 non-sexually active and 17 sexually active females 10-21 years old diagnosed with CF evaluated reproductive health counseling and contraceptive use.

<50% of young CF women are counseled about pregnancy and contraception and most choose high failure methods (e.g., condoms). Adolescents/young adult females with CF should be routinely screened for sexual activity, counseled on the effects of CF on pregnancy and offered long-acting reversible contraception.
  • Are mRNA COVID-19 vaccines safe for pregnant women?
  • Can you describe the potential adverse consequences associated with long-term use of oral corticosteroids?
  • What is the incidence rate of clavicular fractures in children?

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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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