infants

January 10 2023

Apnea

PME-Admin article, medical BPAP machines, central apnea, children, CPAP machines, infants, neonatal apnea, pediatric, pediatrics, respiratory

Apnea
Betsy S. Kastak DNP, RN C-PNP + Santa J. Bartholomew MD

Apnea is defined as a pause in the regular breathing pattern during sleep. The causes are many; however, the results are similar in that the interruption of breathing burdens the individual’s respiratory, cardiovascular, and neurological systems. This burden is cumulative over time.

kinds of apnea

There are three general kinds of apnea:

Obstructive Sleep Apnea is breathing blocked by an “obstruction” of the upper airway while chest and abdominal muscles keep attempting to breathe.
- shallow breathing, snoring with big gasps
“Central” Sleep Apnea
- breathing takes a pause, the brain “skips a beat”
Hypopnea
- reduction in breathing of about 50%, shallow breath, due to a partially obstructed airway
- small change in oxygen level
- shallow breath
- Rarely requires intervention

Apnea in Children

Pediatric obstructive sleep apnea syndrome (OSAS) is a sleep disorder in which a child’s breathing is partially or entirely blocked repeatedly during sleep. The condition is due to narrowing or blockage of the upper airway. The prevalence of OSAS ranged from 0% to 5.7%, with obesity being an independent risk factor. OSAS can be associated with cardiovascular issues, growth, neurobehavioral abnormalities, and possibly inflammatory changes in the body.

Children with OSAS are most likely to demonstrate behavioral problems unlike their adult counterparts how usually show daytime sleepiness. No matter how OSAS is revealed in adults and children it may residual complications if not treated.

The most common underlying condition in children is the enlargement of the adenoids and tonsils. Therefore, the primary treatment is typically adenotonsillectomy (AT). Usually, this results in significant improvements in behavior and attention and can also improve cognitive abilities. Although OSAS will often improve postoperatively, children may have residual problems, especially if they are in the “obese” category on the BMI scale.

Following an adenotonsillectomy (AT), if residual obstruction remains, is one common therapy used to treat the issue:

CPAP (continuous positive airway pressure): a machine that delivers a steady stream of air. The airstream pushes against any obstructions, maintaining an open airway for breathing. CPAP machines are used to treat OSAS.
- Though respiratory therapy may be effective in treating OSAS, adherence is a significant barrier for children. For this reason, CPAP is not recommended as first-line therapy for OSAS when AT is an option.

Children with Down syndrome are most vulnerable to obstructive sleep apnea because of their unique craniofacial anatomy and hypotonia (poor muscle tone).

Smaller mid-face: typically, people with Down syndrome have a smaller upper jaw, and/or nose.
- A smaller jaw makes the tongue seem larger and this can cause an obstruction.
Decreased pharyngeal muscle tone: pharyngeal muscles are the muscles that support breathing, and those muscles can be weaker in people with DS.

Timely recognition and treatment of OSA in this population of children is an important to achieve optimal cognitive function and quality of life. Although guidelines recommend routine OSA screening in young children with Down syndrome, it can be difficult to perform, and the results are often uncertain.

The National Down Syndrome Society (NDSS) promotes awareness of OSA for individuals with Down Syndrome. Their website states: “There is a 50-100% incidence of obstructive sleep apnea in individuals with Down syndrome, with almost 60% of children with Down syndrome having abnormal sleep studies by age 3.5 -4 years.”

In 2011, the American Academy of Pediatrics recommended that all children with Down syndrome have polysomnography, or a sleep study, by four years of age. The sleep study consists of a night of observed sleep during which professionals measure brain waves, the oxygen level in the blood, heart rate, breathing, and eye and leg movement. Children who cannot have a sleep study PSG due to lack of access or inability to tolerate the conditions may have a home sleep study or pulse oximetry as an initial screening.

CPAP and BiPAP machines are typically life-long treatments. Although many individuals with Down syndrome adjust to using the CPAP during sleep, some cannot tolerate wearing the facial mask and require alternative therapies.

Research suggests that OSAS is also a risk factor other diseases:

Reactive airways with bronchoconstriction
- Research confirms that asthmatic patients are more prone to develop OSAS symptoms than people who do not have asthma
- Nasal obstruction due to allergies is one of the characteristics of asthmatics that increase OSAS symptoms and increase the collapse of the upper airways
Gastroesophageal reflux
Generalized inflammation of airways
OSAS-induced cardiac dysfunction have been proposed as factors that worsen asthma control.

Neonatal apnea is a condition that affects both term and pre-term infants for various reasons, though it is more common in preterm infants. Unlike periodic neonatal breathing that combines a regular respiratory pattern and respiratory pauses, neonatal apnea and apnea of prematurity are defined as the absence of breathing for more than 20 seconds.

Clinical manifestations of neonatal apnea require careful management, including invasive or noninvasive treatment, ventilation, monitoring, and pharmacology intervention to prevent acute and long-term complications. When a neonate, preterm or not, has a classic apneic event, they will cease breathing for 15-20 seconds and have no respiratory effort. This may be accompanied by bradycardia and some oxygen desaturation; the baby will also bluish tint to the skin.

This is one of the more common conditions diagnosed in the neonatal intensive care unit. Apnea is caused by immaturity of the neurological system and weakness of the muscles that keep the airway open. At times, additional stresses, specifically in a premature baby — including infection, heart or lung problems, low blood count, low oxygen levels, temperature problems, feeding problems, and overstimulation, may cause or enhance incidents of apnea. Recurrent apnea can lead to respiratory failure, pulmonary hemorrhage, abnormal heart and lung function, intracranial hemorrhage, abnormal nervous system development, and even sudden death.

Caffeine citrate has been used for treating apnea since 1970 due to it’s excellent bioavailability and paucity of side effects. Varying doses of caffeine citrate for apnea have been proposed. The safe and effective amount of low and high caffeine citrate maintenance doses for apnea treatment of premature infants has been determined by analysis of multiple clinical studies. Higher-maintenance doses of caffeine citrate appear more effective than low-maintenance doses for the treatment of apnea despite a high incidence of tachycardia.

Central sleep apnea (CSA) is less common than neonatal or obstructive sleep apnea. It is a pause in breathing during sleep, usually without snoring or gasping. Everyone experiences central apneas occasionally. However, if it occurs too frequently or for long periods, it can cause a decrease in the oxygen levels in the body, disrupt sleep and impact neurologic development in children.

Central sleep apnea is caused by increased sensitivity to the partial pressure of CO2 in the system that regulates the respiratory drive. CSA commonly presents as cycles of deep, rapid, crescendo decrescendo breathing (hyperpnea), followed by slower, shallower breathing (hypopnea) or no breathing without respiratory effort from the diaphragm (apnea), known as Cheyne-Stokes breathing.

The cause of CSA in children is either idiopathic or due to a profound neurological insult at birth or after. CSA can lead to systemic and pulmonary hypertension, arrhythmias, and cardiovascular collapse is untreated.

Characteristics of central sleep apnea include:

Observed episodes of pauses in breathing or abnormal breathing patterns during sleep
Abrupt awakenings followed by shortness of breath
Frequent awakenings in general
Headaches in the morning
Difficulty focusing and concentrating
Daytime sleepiness (if sleep is disturbed)

BiPAP machines offer three air-pressure settings which include spontaneous switching that senses breathing patterns and adjusts accordingly, timed switching which is better for those who need a regular rate and spontaneous timed switching that is a mix of both. BiPAP is better for those with central sleep apnea since they assist with air trapping and can provide a backup respiratory rate when the patients has to long a period of apnea.

RESOURCES

Abu-Shaweesh JM, Martin RJ. (2005) Apnea of prematurity: past, present, and future. J Arab Neonatal Forum; 2:63-73.
Chen, J; Chen, X (2008) BioMed Research International, (8)1-11.
Deakins, K (2011) Management of Neonatal Apnea, AARC Times, 35(11): 5-8.
Hill CM, Evans HJ, Elphick H, et al. (2016) Sleep Med (27)28: 99–106
Javaheri S, Dempsey JA. (2013) Central sleep apnea. Comprehensive Physiology. (3): 141‐163.
Alkhalil, M, Schulman, E, Getsy,J, 2009 Obstructive Sleep Apnea Syndrome (OSAS) and Asthma: What are the links? J Clin Sleep Med. 5(1): 71–78.
Sleep & Down Syndrome | National Down Syndrome Society (NDSS)

Disclaimer: This web site provides general information about pediatric health and related subjects. The information and other content provided on this website or in any linked materials are not intended and should not be considered, or used as a substitute for, medical advice, diagnosis or treatment. Never disregard professional medical advice or delay in seeking it because of something that you have read on this website or in any linked materials.

January 3 2023

Monkeypox

PME-Admin article, medical children, infants, monkeypox, pediatric, pediatrics

Monkeypox
Cynthia D. Read, CPNP-PC, LNC & Santa J. Bartholomew MD

The World Health Organization (WHO) has recently recommended “mpox” to be the new preferred term/synonym for Monkeypox. Both the Centers for Diseases control (CDC) and Health and Human Services (HHS) have adopted “mpox” as the new term used for Monkeypox.

Monkeypox is a virus belonging to the Orthopoxvirus genus in the Poxviridae family. Monkeypox primarily occurs in west and central Africa, often close to tropical rainforests. The first outbreak of Monkeypox outside of Africa occurred in 2003 in the United States. Since that time, Monkeypox has continued to be reported in several areas of the world including the United States.

A 7-year-old Zairian girl with monkeypox in the acute stage, day 7 of rash, and monkeypox in a 3-year-old Zairian boy with rash in the scabbing stage, DRC, 1970-1977. WHO / Mark V. Szczeniowski.

Signs and Symptoms of Monkeypox

Symptoms of Monkeypox are similar to those of smallpox, but often less severe. The time from infection to the onset of symptoms (Incubation period) can range from 5 to 21 days, but is usually 6 to 13 days.

Two Phases of Infection

The Invasion Period: Lasts 0-5 days. Characterized by intense headaches, fever, swelling of the lymph nodes, muscle aches, back pain, extreme fatigue. Swelling of the lymph nodes is a primary feature of monkey pox not generally seen with other similar illnesses such as chickenpox, smallpox, and measles.
The Skin Eruption Period: Usually appears 1-3 days following fever. The rash is usually seen more on the face, arms and legs rather than the trunk. Affects the face (95% of cases), palms of hands and soles of feet (75% of cases), oral mucous membranes (70% of cases), genitalia (30% of cases), conjunctivae (20% of cases) and cornea. The rash starts as flat lesions progressing to raised, firm lesions. These lesions then become filled with a yellowish fluid which then dry up and fall off. The number of lesions may vary from a few to thousands.

Monkeypox is a self-limiting disease. Symptoms generally lasts 2-4 weeks. Severe cases are more common in children. Severity of the illness is related to the extent of the exposure to the virus.

Complications of Monkeypox

Secondary infections, pneumonia, overwhelming infection/sepsis, encephalitis, infection of the cornea leading to vision loss.

Gaffney, B. (2022, August 5). What You Need to Know about Monkeypox. Baton Rouge Clinic. https://batonrougeclinic.com/what-you-need-to-know-about-monkeypox/.

Cleveland Clinic. (2022, June 17). Monkeypox: Causes, Symptoms, Treatment and Prevention. Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/22371-monkeypox.

How is Monkeypox Transmitted

Transmission may occur from the mother to fetus via the placenta. This can lead to congenital monkeypox. Transmission may also occur due to close contact during and after birth. The risk of children and adolescents being infected with the monkeypox virus is low. Infants, children and adolescents may be at an increased risk of contracting Monkeypox if they suffer from eczema, other skin conditions, or immunocompromising illnesses.

Various animals have been identified as being susceptible to the monkeypox virus and therefore, becoming hosts for the disease. Animals included are rope squirrels, tree squirrels, Gambian pouched rats, dormice, non-human primates and several other species.

Transmission from animal-to-human may occur through direct contact with blood, body fluids, or skin or mucosal lesions of infected animals. Possible risk factors include eating meat that has not been cooked adequately and other animal products of infected animals.

Human-to-human transmission may also occur. This can result from close contact with respiratory secretions, lesions of the skin of an infected person, or touching recently contaminated objects. Transmission via respiratory droplets requires a prolonged length of time having face-to-face contact. This places health care workers, family members and other close contacts of active cases at an increased risk.

Diagnosis and Treatment

It is important to consider other similar illness when diagnosing:

Other rash illnesses (chickenpox, measles, bacterial skin infections, scabies, syphilis, allergies from medications)

Diagnosis by PCR is the preferred laboratory test. Fluid from the vesicles/pustules and dry crusts. Biopsy may also be taken. PCR blood test often inconclusive. Previous vaccination with smallpox vaccine or monkeypox vaccine may result in false positive results.

Treatment includes

Alleviating symptoms, managing complications and preventing long-term sequelae.
Fluids and food are important to maintain good nutritional status.
Secondary bacterial infections will also need to be treated.
An antiviral (tecovirimat) was developed for smallpox and licensed by the European Medicines Agency (EMA) for monkeypox in 2022. This was based on data from animal and human studies. Not yet widely available

Prognosis

In the past, fatality ranged 0-11% (higher among young children). More recently, 3-6%.

Vaccination

Smallpox was eradicated in 1980 and there was subsequent cessation of the smallpox vaccine. Being vaccinated against smallpox has been shown to be approximately 85% effective in preventing monkeypox. Due to no longer manufacturing the smallpox vaccine, persons under 40 to 50 years of age may be more susceptible to monkeypox.

A newer vaccine was approved in 2019 for the prevention of monkeypox. This is a two dose vaccine, but availability is limited.

Hernandez, A. (2022). Monkeypox. Elsevier. https://www.osmosis.org/answers/monkeypox.

Prevention

Be aware of risk factors
Educating about measures to reduce exposure to the virus is the key to prevention
Possibility of more widely available vaccines in the future

RESOURCES

Monkeypox. (n.d.). https://www.aap.org
Mpox. (2022, December 8). https://www.cdc.gov
Monkeypox. (2022, May 19). https://www.who.int

December 6 2022

Extubation Failure

PME-Admin article, medical breathing issues, children, extubation, infants, pediatric, pediatrics, ventilator

Extubation Failure
Santa J. Bartholomew MD

Mechanical ventilation is often required for critically ill patients in the ICU. As the acute phase of illness resolves, the goal is to wean the ventilator, allowing the patient to breathe more on their own (spontaneous breathing trials), and to successfully extubate, or remove the artificial airway. Patients may tolerate weaning the ventilator, but fail extubation. Extubation failure is defined as the inability to sustain spontaneous breathing after the removal of the artificial airway (an endotracheal tube or tracheostomy tube) and require reintubation within a specified time period, usually defined as 24-72 hours or up to 7 days.

Causes of Extubation Failure

Excessive secretions affecting airway patency
Respiratory Failure: Most common cause. Manifest as increased work of breathing, hypoxia (insufficient oxygen), hypercapnea (excessive carbon dioxide in the blood), use of accessory muscles for breathing, respiratory acidosis
Upper airway edema affecting patency of airway
Neurological impairment: May be secondary to primary illness, encephalopathy, or residual effects of sedation
Neuromuscular Diseases (Guillain-Barre syndrome, Duchenne Muscular Dystrophy)
Acute heart failure
Poor nutrition
Other risk factors: Severity of illness on admission to ICU, prolonged intubation, continuous sedation

Photo + caption source: Fliesler, N. (2020, May 11). Children with COVID-19 in the PICU: A snapshot. Boston Children’s Answers. https://answers.childrenshospital.org/covid-19-picu

It is essential for the Health Care Provider to identify patients at a higher risk for extubation failure/need for re-intubation, as extubation failure can have detrimental consequences. It is also imperative that all appropriate equipment and personnel are on hand for difficult extubation.

Consequences of Extubation Failure

The incidence of extubation failure has been noted to be between 6% and 47%. Nosocomial pneumonia is significantly increased in patients failing extubation (47% versus 10%). Additional consequences of extubation failure are:

Prolonged ICU stay and hospitalization
Prolonged mechanical ventilation
Need for tracheostomy
Increased medical cost
Increased morbidity and mortality

Mortality is increased with increasing duration of time between extubation and reintubation. This is due to deterioration in the patients’ condition leading to organ dysfunction and failure.

Predictors of Extubation Failure

There are many risk factors for extubation failure. It is vital for the Healthcare Provider to do a thorough assessment of extubation readiness. Parameters that are used to predict failure of extubation are often categorized into parameters assessing respiratory mechanics, airway patency and protection of airway, and cardiovascular reserve. Successful extubation is dependent on adequate cough strength, minimal secretions, and mental alertness or the neurological ability to maintain adequate spontaneous breathing.

Successful spontaneous breathing trials: These trials allow the patient to breath more on their own while the artificial airway remains in place. This allows the Healthcare provider to better assess respiratory muscle reserve/weakness and work of breathing
Adequate cough strength: Important for patient to be able to clear airway of secretions. This may be affected due to weak respiratory muscles and laryngeal dysfunction
Secretions: Excessive secretions or the patient’s inability to handle their secretions
Neurological Dysfunction: Study results vary on the impact of neurological impairment on extubation failure. Most recently, a GCS ≥10 is prerequisite for successful extubation
Cuff leak: Laryngeal edema and airway obstruction following extubation may lead to extubation failure. When the cuff on the artificial airway is deflated, the amount of leak noted depends on the degree of laryngeal and airway edema. Glucocorticoids (steroids) are often administered prior to extubation to reduce airway edema
Vital Signs and Blood Gases: Spontaneous breathing requires the heart to work harder. Patients with poor heart function or following cardiac surgery may be at an increased risk for failing extubation. An increase in heart rate and respiratory rate during spontaneous breathing trials may be an indicator of extubation failure. Other findings may include an increase in lactic acid, an increase in PaCO2 (carbon dioxide) in the blood, a decrease in the blood pH (indicating acidosis), or decreased oxygen content in the blood.

Predictors of extubation failure may not apply to neurologically injured patients as the ability to protect their airway and clear secretions may be impaired independently of their ability to ventilate.

*Photo + caption source: Mark, N. (n.d.). WEANING FROM THE VENTILATOR. Retrieved November 30, 2022, from https://static1.squarespace.com/static/5e6d5df1ff954d5b7b139463/t/5e7a8442e1294b32cd7106b1/1585087555516/ICU_one_pagers_vent_weaning.pdf

Photo + caption source: Kurachek, S. (2003). Extubation failure in pediatric intensive care: A multiple-center study of risk factors and outcomes. Critical Care Medicine, 31(11). https://doi.org/10.1097/01.CCM.0000094228.90557.85

Management of Failed Extubation

Extubation failure is a common occurrence in the ICU. Even with successful spontaneous breathing trials, patients remain at risk for extubation failure following removal of the artificial airway due to other factors and circumstances.

Nasal Intermittent Mandatory Ventilation (NIMV)/Intermittent Mandatory Ventilation (IMV): Use of NIMV/IMV has been shown to provide the additional respiratory support needed to avert/prevent reintubation in some patients.
High-Flow Nasal Cannula: Treatment for hypoxemic acute respiratory failure. Delivers a high flow/fraction of inspired oxygen (FiO2) and generates a low level of positive pressure. This provides support for patients with weak respiratory muscles and improves the work of breathing and respiratory rate.
Oxygen alone should not be used to correct hypoxemia that is caused by hypoventilation (ineffective breathing and ventilation). In doing so, the respiratory drive can be suppressed and precipitate acute respiratory failure
Steroids: Edema of the upper airway is difficult to assess before extubation. Upper airway edema is a risk factor for reintubation. Evaluating for a cuff leak prior to extubation predicts stridor but not the need for reintubation. In a double blinded trial, four (20 mg) doses of prophylactic use of methylprednisolone initiated 12 hours before extubation at 4 hour intervals versus placebo, resulted in reduction (22% to 3%) of laryngeal edema and reduction in reintubation (8% to 54%) due to laryngeal edema.

Evidence is suggestive that prophylactic administration of glucocorticoids and early institution of non-invasive ventilation may prevent reintubation in some patients. If necessary, early reintubation and reinstitution of ventilation should be instituted.

Photo + caption source: Frat, J.-P. (2017). High-Flow nasal oxygen therapy and noninvasive ventilation in the management of acute hypoxemic respiratory failure. Annals of Translational Medicine, 5(14).

RESOURCES

Agarwal, A. (2017). Respiratory Care of Patients with Neuromuscular Diseases.
Coplin, W. (2000). Implications of Extubation Delay in Brain-Injured Patients Meeting Standard Weaning Criteria. American Journal of Respiratory Critical Care Medicine, 161, 1530–1536
Frat, J.-P. (2017). High-Flow nasal oxygen therapy and noninvasive ventilation in the management of acute hypoxemic respiratory failure. Annals of Translational Medicine, 5(14).
Kulkarni, A. (2008). Extubation failure in intensive care unit: predictors and management. Indian Journal of Critical Care Medicine, 12(1), 1–9. https://doi.org/10.4103/0972-5229.40942
Kurachek, S. (2003). Extubation failure in pediatric intensive care: A multiple-center study of risk factors and outcomes. Critical Care Medicine, 31(11). https://doi.org/10.1097/01.CCM.0000094228.90557.85

November 22 2022

Headaches in Children

PME-Admin article, medical brain tumor, children, headaches, herpes simplex virus, infants, pediatric, pediatrics

Headaches in Children
Santa J. Bartholomew MD

Headache known as pain above the orbitomeatal line is one of the most common complaints in children and young people. The prevalence of headaches increases with age and greatly impacts school attendance for the child and work attendance for parents. The primary concern for caregivers always being brain tumor. Headaches are estimated to occur in 75% of adolescents and 25% of younger children with migraine being the most common reason for a child to see a neurologist.

Thorough headache history and focused neurological examination are critical in evaluating headaches and distinguishing between primary headaches such as migraine, tension and chronic daily headache and secondary headaches such as those that occur with brain tumors, other causes of intracranial hypertension such as pseudotumor cerebri, meningitis, hydrocephalus from any cause. Acute febrile illnesses can also cause headache and essential hypertension. Classification of headache disorders have been established by the International Headache Society in 2013 and following these guidelines can help practitioners arrive at appropriate treatment plans for the particular headache the child is suffering from.

GETTING A HEADACHE HISTORY

History needs to be gotten from parent AND child. Of course, questions need to be developmentally appropriate.
Essential information in headache history
- When did headaches first begin?
- Location of the pain.
- Quality of the pain.
- Severity of the pain, has the pain remained the same or getting worse.
- Are there other symptoms such as focal weakness, sensory or visual disturbances, vomiting, light intolerance, double vision?
- Are headaches chronic or episodic? Frequency and duration of headache?
- How long have headaches been occurring?
  - If headaches are long-standing has there been a change in the quality or position of the headache?
- Can you tell the headache is coming on?
- Has the headache every woken you up at night?
- Have you ever had a seizure with the headache?
- Are there activities or foods or medications that make the headaches worse?

The Orbitomeatal Line

Focused Neurologic Examination

Although there is consensus on how to achieve historical data about history of headache through the International Headache society and the use of tools such as the Pediatric Migraine Disability Assessment (PesMIDAS) there is little consensus on the evaluation of these complaints.

Most agree that at the least an examination should include:

Assessment of the immunocompetence of the child
Fever
Careful cranial nerve examination: nuchal rigidity, swelling of scalp, sinus tenderness, TMJ tenderness, posterior neck pain (sometimes seen in Chiari malformation), thyromegaly, muscular tightness.
Palpation of head and neck:
Assessment of gait and reflexes
Careful examination of spine
Careful examination of skin looking for signs of neurocutaneous disorders such as tuberous sclerosis. Neurofibromatosis.
Assessment of the retina with full visualization of the optic nerve
Auscultation of eyes, neck and head for bruits
Assessment of oropharynx looking for tooth decay or tonsillar abscess

Diagnostic studies:

Neuroimaging: may be considered when headache is subacute but rapidly progressive in severity, new onset of HA in immunocompromised child, presence of systemic findings like nuchal rigidity or fever, abnormal neuro examination in a new onset headache, headaches awakening a child from sleep.
- Neuroimaging should also be considered in all children WITHOUT a family history of primary headache disorders.
EEG: not recommended in routine evaluation of headache in children as diagnostic yield is usually low.
Lumbar puncture: not routinely recommended unless clinical presentation suggests infectious etiology to headache.
Lab testing: often unnecessary in assessment of primary headache disorder unless there is suspicion of an underlying cause such as hyperthyroidism, severe anemia.

Primary Headaches

Migraines:
- Intermittent attacks of headaches characterized by recurrent episodes of moderate to severe head pain lasting 2 to 72 hours.
- Usually, bifrontal or bitemporal
- Pain is usually focal, throbbing and worsens with activity.
- Can be accompanied by nausea, light sensitivity, and sound sensitivity
- 10% of children have an aura
- Treatment occurs in tiers:
  - I: Analgesics such as Tylenol, Ibuprofen, Naproxen are the initial treatment for treatment naïve children
  - II: Triptans: for children at least 5 years old and who have moderate to severe attacks. These are dosed by age, weight and ability to swallow a pill
  - III: Multiple drug therapy: For children>5 years old who have refractory to monotherapy migraines. Suggestion is a tier I and II drug in combination.
- Chronic Migraine
  - Migraine that occurs 15 or more days a month with at least 8 of the headaches having migraine features.
  - More common in females than males.
  - Overall prevalence in middles school to high school aged children is 1.5%
  - Avoidance of overuse of medication is important step in prevention of chronicity.
- Tension Headaches
  - Characterized by diffuse location, non-throbbing, mild to moderate in severity and does not worsen with activity.
  - Typically last 30 min to 7 days and may be associated with light or sound sensitivity but symptoms do not include an aura, nausea or vomiting
  - Acute treatment is with tier I drugs although gabapentin and Topamax have been used as medication alternatives.
  - Prevention is accomplished with lifestyle modification: healthy habits such as not skipping meals, limiting caffeine consumption, good sleep hygiene.

Cluster Headaches: constitute the most common trigeminal nerve headache. It is usually along the trigeminal nerve location.

Pain is usually severe but lasts < than 3 hours. But multiple episodes of headache can occur during that time.

- They are often one-sided and in addition to headache the sufferer may have watery eyes, injection of the conjunctivae, runny nose or congestion, facial and forehead sweating, eyelid edema or droopy eyelid and changes in pupil response.
- Acute therapy includes: oxygen in an ED or urgent care setting or SQ sumatriptan or in patients who cannot administer an injection then IN sumatriptan.
- Patients who do not respond to these simple therapies may be treated with intranasal lidocaine of oral ergotamine’s
- Prevention should be initiated immediately for cluster headaches, and this includes preventative therapy with Verapamil or in active clusters glucocorticoids may help.
- Lithium and Topamax have also been used in combination with other drugs for refractory headaches.

Secondary Headaches

Headaches caused by an underlying condition.

Acute febrile illness
- Rhinosinusitis
- Meningitis: acute or chronic
- Encephalitis
Post-Traumatic headaches
- Intracranial hemorrhage from trauma: epidural, subarachnoid or subdural hematoma
- Concussions
Side effect of medications
Headache medication overuse
Severe and acute systemic hypertension (very high blood pressure)
Brain tumor
Idiopathic Intracranial hypertension: pseudotumor cerebri
Hydrocephalus

In these cases head history usually sorts these patients from primary headache sufferers and physical examination provides clues to etiology of headache for example changes n mental status or gait disturbances, abnormal fundoscopic examination.

Diagnostics: imaging often is needed and helps identify the cause of the headache and when cause is identified then treatment can be determined.

RESOURCES

Popova V, Berk T Pedatric Migraine- An Updated Review. US Neurology.2019:15(2):68-73
Klein J, Koch T. Headache in Children. Pediatrics in Review 2020:41(4):159-169
Al Khalili Y, Chopra P. Migraine Headache in children Stat Pearls Jan 2022
Bonthisu D, Hershey A. Headache in Children: Approach to Evaluation and General Management Strategies. UpToDate Feb 2021
May A. Cluster Headache: Treatment and Prognosis. UpToDate Feb 2022
May A. Culster Headache: Epidemiology, Clinical Features and Diagnosis UpToDate Jan 2022
Wald E. Acute Bacterial Rhinosinusitis in Children: Clinical Features and Diagnosis UpToDate Nov 2022
Kacperski J, Kabbouche M, O’Brien H., Weberding J. The Optimal Manamgent for Heaches in Children and Adolescents. Ther Adv Neuro Dis 2016:9(1):53-68.

Pediatric Medical Experts Encephalitis Headaches in Children

October 18 2022

Pediatric Encephalitis

PME-Admin article, medical children, encephalitis, infants, pediatric, pediatrics

Pediatric Encephalitis
Santa J. Bartholomew MD & Cynthia D. Read, LNC

Pediatric Encephalitis is an altered mental status for more than 24 hours that is accompanied by two or more symptoms/findings that are concerning for inflammation of the brain parenchyma. The brain parenchyma is the tissue responsible for specific functions of the brain. The incidence of pediatric encephalitis is unclear, due to multiple etiologies and inconsistent definitions of encephalitis. Recent reports do indicate that cases of pediatric encephalitis may be increasing due to increased recognition of autoimmune etiologies and use of magnetic resonance imaging (MRI) to describe abnormalities in the brain parenchyma. Up to 30% of cases of pediatric encephalitis have an undiagnosed etiology.

How does Encephalitis happen?

Encephalitis is inflammation of the brain. This can be caused by an infection or an autoimmune cause. The inflammation is the body’s immune system reacting to the infection or invasion. The inflammation causes the brain’s tissues to become swollen. The infection can get into the cerebral spinal fluid (CSF).

In the past, infectious agents accounted for most of the etiologies for encephalitis. Before the use of vaccinations, measles, mumps, varicella and polio were among the top causes for encephalitis. With the introduction of vaccines, other viral etiologies such as herpes simplex virus (HSV) and enterovirus (EV) have risen to the top of etiologies for infectious encephalitis in pediatrics.

In autoimmune encephalitis, the body’s immune system attacks the brain causing inflammation. The number of cases of autoimmune encephalitis are beginning to outnumber those of viral encephalitis, with the incidence of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis exceeding the rate for a single viral etiology for pediatric encephalitis.

TABLE 1: Flowchart showing categorization of childhood (age ≤14 years) suspected encephalitis cases identified by the Australian Childhood Encephalitis study, 2013-2016.

Pediatric Medical Experts Encephalitis in Children from Mosquito Bites

Pediatric Medical Experts Encephalitis in Children from Tick Bites

Causes

Herpes Simplex Virus: Most common cause of pediatric encephalitis. Estimated incidence 3 to 30 cases per 100,000 live births. Serious morbidity and mortality. Transmitted intrauterine, perinatal, and postnatal
Enterovirus: Among top causes of pediatric encephalitis
West Nile Virus: Most common arbovirus in North America (virus spread to people by the bite of an infected arthropod/insect such as mosquitoes and ticks). <4% of cases involve children <18 years old. Transmitted by culex mosquito and therefore, mostly seen in summer
La Crosse Virus: Second most common arbovirus in North America. 88% of all encephalitis due to La Crosse virus occurs in children <18 years of age. Most cases in Appalachian and Midwestern region of US. Usually seen in late spring through early fall. Transmitted via infected Aedes mosquito
Zika Virus: Can cause microcephaly (small head and abnormal brain development) in infants. Transmission via infected Aedes mosquito. Seen in summer and early fall.
Bacteria: Streptococcus pneumoniae, haemophilus influenzae serotype b, Neisseria meningitidis, Listeria monocytogenes, mycoplasma
Autoimmune diseases

Related Reading – Tick Diseases

SYMPTOMS

Symptoms may be similar for infectious and autoimmune encephalitis. A consensus statement provided by the International Encephalitis Consortium outlines diagnostic criteria for possible and confirmed encephalitis. Per this consensus statement, a requirement for encephalitis is altered mental status for >24 hours without an alternative diagnosis, and 2 or more of the additional clinical features mentioned below:

Fever: >100°F (>38°C) within 72 hours
Seizures: New onset or different than pre-existing seizure history
New focal neurologic findings
Cerebrospinal fluid (CSF) pleocytosis (white blood cell count > 5/µL
Abnormal neuroimaging findings consistent with encephalitis and/or abnormal electroencephalogram (EEG) reading consistent with encephalitis

NEUROLOGIC DISFUNCTION ENCEPHALITIS

Messacar, K. (2017). Encephalitis in US Children. Infectious Disease Clinics, 32(1).

MRI Patterns in Patients with Viral Encephalitis. Read the full review on acute viral encephalitis

Encephalitis may initially present with flu like symptoms. History of the illness and other physical exam findings are pertinent.

Clinical features

Fever (67%-80%)- More common in Viral Encephalitis
Seizure (33%-78%)
Headache (43%)
Weakness or pyramidal signs (36%-78%)
Agitation (34%)
Decreased consciousness (47%)
Autoimmune Encephalitis: More chronic findings with delayed diagnosis up to several weeks

Image: MRI Patterns in Patients with Acute Viral Encephalitis
Source: New England Journal of Medicine

HOW DO WE DIAGNOSE ENCEPHALITIS?

Level of suspicion based on history, signs and symptoms, epidemiologic risk factors, recent travel, recreational, occupational exposures, insect or animal contact, seasonality, mood/behavior or personality change resembling acute psychosis
Herpes Simplex Virus: HSV is the most common cause of pediatric encephalitis and should be suspected in any child with concern of encephalitis. Ophthalmologic exam and conjunctival HSV culture to evaluate for eye involvement
Lab Studies: CBC with differential, CMP, HSV serum PCR, Enterovirus serum PCR
Lumbar Puncture/CSF Studies: Cell count, Protein, Glucose, Gram-stain and bacterial culture, HSV PCR, Enterovirus PCR. If patient is not improving, Autoimmune panel, Meningoencephalitis multiplex PCR panel, CSF immunoglobulin M to look for specific arboviruses such as West Nile virus and La Crosse virus, Extra CSF to freeze for additional testing (Obtain opening pressure with lumbar puncture. If there is concern for increased intracranial pressure, always obtain a head CT prior to performing a lumbar puncture
NeuroImaging:
1. MRI with and without contrast. First-line imaging test for pediatric patients with suspected intracranial infection.
2. Dedicated Epilepsy Protocol MRI with and without IV contrast for suspicion of autoimmune encephalitis
3. Computed Tomography (CT): Head CT without IV contrast is first line imaging in pediatric patients presenting with acute mental status change as this can quickly and accurately exclude neurosurgical emergencies
4. Abdominal/Pelvic Ultrasound: Anti-NMDAR encephalitis has ha an association with ovarian teratomas. US may allow resection of the tumor to alleviate the encephalitis
5. EEG: Controversial. Most patients with encephalitis will have an abnormal EEG. Recommend consultation with Pediatric Neurology

“Encephalitis in Previously Healthy Children.” Pediatrics in Review, vol. 42, no. 2, Feb 2021, pp. 68–77.

TREATMENT

HSV is the leading cause of severe encephalitis and is associated with poor outcomes. Acyclovir should be initiated on all patients with suspected encephalitis until HSV is ruled out
Empirical antibacterial antibiotics, while awaiting culture results, focused primarily on coverage for Streptococcus pneumoniae, haemophilus influenzae serotype b, Neisseria meningitidis, Listeria monocytogenes, Mycoplasma
Corticosteroids – more commonly used for viral encephalitis such as varicella zoster and HSV if concern for swelling in the brain
Immunomodulators (help to “calm” the immune system) – Corticosteroids, IVIG (decreases the overall burden of the offending autoantibody), Plasma exchange (potentially removes the disease causing antibodies), Rituximab
Always evaluate for and treat any underlying disease process

PROGNOSIS

Highly variable. Dependent on etiologic agent/cause
Meta-analysis examining outcomes in patients with infectious encephalitis: 42% incomplete recovery, 6.7% severe sequelae, 17.5% decrease in intelligence quotient
HSV encephalitis: Up to 64% experience late effects such as developmental delay, neuropsychological dysfunction, and focal motor deficits
Autoimmune encephalitis: ~ 1/3 demonstrate full recovery, 1/3 partial recovery, 1/3 with limited recovery and severe deficits
Use of immunotherapy in autoimmune encephalitis correlates with improved outcomes

RESOURCES

“Encephalitis in Previously Healthy Children.” Pediatrics in Review, vol. 42, no. 2, Feb 2021, pp.68-77.
Messacar, K. (2017). Encephalitis in US Children. Infectious Disease Clinics, 32(1).
Up-to Date: 2021: Neonatal herpes simplex virus infection: Clinical features and diagnosis

October 11 2022

Abdominal Pain Part V: Acute Pancreatitis

PME-Admin article, medical abdominal pain, acute pancreatitis, children, infants, pancreatitis, pediatric, pediatrics

Abdominal Pain Part V: Acute Pancreatitis
Santa J. Bartholomew MD & Cynthia D. Read, LNC

Acute pancreatitis is one of the leading gastrointestinal causes of hospitalizations in the United States, and is increasingly being diagnosed in children within the past two decades.

Characterized by an acute inflammation of the pancreas, older children often present with a sudden onset of persistent, severe mid-abdominal pain that may radiate to the back. Younger children and infants may present with more generalized abdominal discomfort, irritability and vomiting.

The Pancreas

The pancreas is in the abdomen just behind the stomach. The pancreas makes enzymes during digestion that break down sugars, fats and starches. Hormones produced in the pancreas help the digestive system.

Classification

Pancreatitis in children may be mild, moderate, or severe. More severe episodes of pancreatitis typically have more complications. Acute pancreatitis may progress to acute recurring pancreatitis (ARP) or chronic pancreatitis (CP).

TABLE 1: DEFINITIONS OF PANCREATITIS IN CHILDREN (INSPPIRE Criteria)

Etiology

Genetic: <10% in acute pancreatitis, >50% in reoccurring pancreatitis, ~75% in chronic pancreatitis
Anomalies of the pancreas: ~ 5-20% of children with acute pancreatitis
Gallstones: most common cause in children. 3-30% of cases
Medications: contribute up to one-fourth of cases
Trauma: accidents and non-accidental trauma from child abuse
Metabolic Disorders: high calcium levels most common in children. Inborn errors of metabolism
Infection: mumps, herpes, salmonella
Other illness: Lupus, Kawasaki disease, inflammatory bowel disease

Diagnosis

Acute pancreatitis is diagnosed based on the patient’s history, physical exam, and supported by laboratory tests and imaging as indicated. Physical findings on exam will vary based on the severity of disease.

Diagnosis requires two of the following three criteria:

Sudden onset of severe mid-abdominal pain that does not go away. This pain may radiate to the back.
Increase in enzymes made by the pancreas more than three times their normal level
Findings specific to pancreatitis on an ultrasound of the stomach, computed tomography (CT) of the stomach or magnetic resonance imaging (MRI) of the stomach

Classic Examination Findings

Tenderness to mid-abdomen, abdominal distention, yellowing of the eyes, fever
3% of patients with acute pancreatitis may have a bluish discoloration around their umbilicus (Cullen’s sign) or a bluish discoloration along their flank (Grey Turner’s Sign)
Some patients may experience:
- Shortness of breath because the diaphragm just above the pancreas gets inflamed and this may progress to acute respiratory failure.
- 5% of children have hemodynamic instability and end organ failure such as renal failure and shock.

DIFFERENTIAL DIAGNOSIS OF PANCREATITIS

Peptic Ulcer Disease (PUD)
Gallstones
Cholecystitis
Perforated viscus
Intestinal obstruction
Mesenteric Ischemia
Hepatitis

Acute pancreatitis is diagnosed based on the patient’s history, physical exam, and supported by laboratory tests and imaging as indicated.

LABORATORY SIGNS

Elevated amylase and lipase: these are enzymes secreted by the pancreas.
- Lipase: elevations are highly sensitive for pancreatitis (85-100%) and rise within 4-6 hours of the onset of symptoms and generally return to normal within 8 to 14 days
- Amylase: elevations with 6 to 12 hours but usually returns to normal within 3-5 days. Serum amylase > three times upper limit of normal has 67 to 83% sensitivity for acute pancreatitis.
- The “gold standard” for diagnosis of acute pancreatitis: elevated lipase and amylase
Markers of immune activation:
- Increase C-reactive protein
- Increase procalcitonin.
- Increase TNF
- Elevated white count
Signs of third spacing
- Elevated hematocrit from hemoconcentration
- Elevated BUN and creatinine
Hyperglycemia/hypoglycemia: with broad swings in insulin levels- insulin also being a hormone secreted by pancreas.

IMAGING

X-ray of abdomen may show distention of bowel/intestines
Abdominal Ultrasound- First choice of imaging in pediatrics
Abdominal CT- is not needed to diagnose pancreatitis
Magnetic resonance cholangiopancreatography (MRCP) – a special kind of MRI used to look at the pancreas and the organs around the pancreas. Is quickly becoming study of choice in pediatrics

Ultrasound of a Human Pancreas

MANAGEMENT AND TREATMENT

Supportive care with fluids, pain control, and nutrition.
Change in therapy in recent years has been towards feeding through the acute phase of pancreatitis.
If unable to take fluids and medications by mouth, may require hospitalization for IV fluids and nutrition through a feeding tube or an IV.
Treat any underlying diseases or predisposing factors for pancreatitis.
If there is an obstruction to flow of secretions from pancreas, then ERCP (endoscopic retrograde cholangiopancreatography) may be needed. While it is not as commonly used in children as in adults has become an invaluable tool in helping treat obstruction.
Islet auto-transplantation (TPIAT) has become the surgery of choice for recurring pancreatitis and chronic pancreatitis. This entails all or part of the pancreas is removed, and special insulin producing cells being placed in the liver as an alternative.

Nguyen,T. (n.d.). Pancreatitis [Review of Pancreatitis]. 41(10).

COMPLICATIONS AND PROGNOSIS

Most children presenting with acute pancreatitis will have mild pancreatitis. Complications that may occur from pancreatitis range from mild to more severe including:

Infection
Fluid collection around the pancreas
Pancreatic Pseudocyst: encapsulated collection of necrotic tissue, secretions or blood
Pancreatic Necrosis: an area of tissue in the pancreas dies due to too little blood supply

The mortality rate for acute pancreatitis in children is <5%. In ~ 15-35%, their pancreatitis will reoccur. Children with acute reoccurring pancreatitis and chronic pancreatitis tend to have more genetic and/or anatomical risk factors compared to adults who tend to have more environmental risk factors. In adolescents, autoimmune and environmental risk factors begin to emerge.

Pancreatic Pseudocyst

Pancreatic Necrosis

RESOURCES

Crockett, S. (2018). American Gastroenterological Association Institute Guideline on Initial Management of Acute Pancreatitis [Review of American Gastroenterological Association Institute Guideline on Initial Management of Acute Pancreatitis]. Gastroenter
Leppaniemi,A. (2019). 2019 WSES guidelines for the management of severe acute pancreatitis [Review of 2019 WSES guidelines for the management of severe acute pancreatitis]. World Journal of Emergency Surgery, 14(27).
Nguyen,T. (n.d.). Pancreatitis [Review of Pancreatitis]. 41(10).
Pancreatitis in Children: Short Title: Pediatric Pancreatitis. Aliye Uc, MD
Up-to Date: 2022: Clinical manifestations and diagnosis of acute pancreatitis
Up-to Date: 2022: Etiology of acute pancreatitis
Up-to Date: 2022: Management of acute pancreatitis

October 4 2022

Abdominal Pain Part IV: Inflammatory Bowel Disease and Ulcerative Colitis

PME-Admin article, medical abdominal pain, celiac disease, children, crohn's disease, infants, inflammatory bowel disease, irritable bowel syndrome, pediatric, pediatrics, ulcerative cholitis

Abdominal Pain Part IV: Inflammatory Bowel Disease and Ulcerative Colitis
Santa J. Bartholomew M.D.

The category of inflammatory bowel disease (IBD) is divided into two specific categories: Crohn’s disease and Ulcerative colitis, both of which are diagnosed in late childhood or early adulthood. IBD is one of the major chronic gastrointestinal diseases among children and adolescents in the westernized world.

Both are characterized by a chronic and debilitating condition defined by a relapsing episode of GI inflammation. In Ulcerative colitis (UC), this includes the superficial lining of the bowel starting at the rectum, and it is limited to the colon. In Crohn’s disease (CD), the inflammation can affect any part of the GI tract from mouth to colon, and it is transmural (across the entire bowel wall).
Pediatric patients often have more severe disease of both UC and CD than their adult counterparts.
In children, there is a male predominance
25% of IBD occurs in childhood, the rest in early adulthood, with the worst of the disease peaking in the second and third decades of life.
- Incidence: Crohn’s Disease: 40/100,000 and Ulcerative Colitis
- Approximately 1.4 million people suffer from IBD in the USA
- A subset of children acquire IBD at a very early age and this is thought to be a different type of IBD than what is seen in older children.

What is the cause of IBD?

The cause of IBD is unknown, but there are several theories:

Dysregulation of the hosts immune system to the normal bacteria that live in the bowel
Genetic component
Alterations in the bacterial biome of the gut
Environmental exposures- since IBD is a Western world disease, it seems reasonable that our diet and food hygiene may play a role in the development of IBD.

Symptoms of IBD

Presenting symptoms: can be HIGHLY VARIABLE.

Abdominal pain: varying quality and location.
Nausea and vomiting
Rectal bleeding
Diarrhea
Weight loss
Growth delay

Pediatric patients often have more severe disease of both UC and CD than their adult counterparts.

Crohn's Disease

Ulcerative Cholitis

Inflammatory Bowel Disease

Diagnosis

Differential Diagnosis Considerations with Inflammatory Bowel Disease

MORE LIKELY TO PRESENT WITH CHRONIC COMPLAINTS

Growth failure
Irritable Bowel syndrome
Lactose intolerance
Ovarian cyst
Celiac disease
Acid-peptic disease
Anorexia nervosa
Excess laxative intake
Lymphoma
Constipation
Sorbitol Ingestion

Peds in Review:37(8) Aug 2018

MORE LIKELY TO PRESENT MORE ACUTELY

Appendicitis
Mesenteric adenitis
Fissure-in-ano
Hemorrhoids
Colonic polyps
Meckel diverticulum
Acute infections
- Clostridium dificile,
- Salmonella/Shingella/Compylobacter species
- Yersinia species
- Giargia species
- Entamoeba histolytica
Parasitic infections

Laboratory Data: 20% of children have COMPLETELY NORMAL LABS

There is no test specific for IBD, but in most of these children, inflammatory markers like CPR, and ESR are elevated
Albumin can be low because of poor nutritional health
Chronic inflammation of the bowel can lead to malabsorption, so vitamin and Zinc levels may be low
There can be other organs affected, such as the liver, and then one would see elevated transaminases

Other organs affected:

COMORBID SIGNS AND SYMPTOMS OF INFLAMMATORY BOWEL DISEASE BY ORGAN SYSTEM

Peds in Review:37(8) Aug 2018

Diagnosis:

Children suspected of IBD need a referral to a GI specialist
Endoscopic examination and biopsies help confirm the diagnosis and determine UC from CD
One-third of patients cannot be classified into either category as there is inflammation of the ileum.

During an upper endoscopy, a thin flexible tube with a camera on the end is inserted into the mouth and down the esophagus to look at the esophagus, stomach, and duodenum.

Treatment for IBD

Treatment involves controlling active symptoms and trying to prevent long-term complications. Treatment, however, is complicated as many of the therapies can impact the growth of the child.

STEROIDS: help induce remission in both UC and CD, but fewer than a third of patients achieve true healing of the bowel mucosa so it can go back to normal function
ENTERAL NUTRITION: giving 100% of the nutritional needs of the child as a liquid diet is also used to induce remission and help heal the mucosa
AMINOSALICYLATES: are topical anti-inflammatory drugs that help decrease inflammation in the bowel, such as Sulfasalazine
IMMUNOMODULATORS: Also assist in attaining and maintaining remission but come with significant side effects such as myelosuppression, chemical hepatitis, pancreatitis, and in a small number of cases lymphoma
ANTI-TNF THERAPY: biologics such as Infliximab, Adalimumab, and Golimumaub that are used to induce remission in children resistant to steroids or who are steroid dependent despite using an immunomodulator. The can, however, cause a psoriasis-like rash, and increased risk of infection, particularly fungal, viral, and mycobacterial infections
SURGERY: total colectomy with an ileal pouch is used in children refractory to medical therapy

Long-term problems:

Growth failure in 40% with CD and 10% with UC
Vitamin deficiencies: Iron, Folate, Vitamin B12, and Vitamin D
Colon cancer
Depression and anxiety disorders

RESOURCES

Rosen, et.al Inflammatory Bowel Disease in Children and Adolescents JAMA Pediatr 2015 Nov; 169(11):1053-1060.
Sauer Cary, Kugathasan Subra. Pediatric Inflammatory Bowel Disease: Highlighting Pediatric Differences in IBD. Gastroenterol Clin N Am 38(2009)611-628.
Shapiro, Jason, Subedi Shova, LeLeiko Neal. Inflammatory Bowel Disease. Peds in Rev 37(8)337-347.
Levine A.,et.al. ESPGHAN Revised Porto Criteria for the Diagnosis of Inflammatory Bowel Disease in Children and Adolescents. JPGN 58(6):795-806.

April 5 2022

Acute Rheumatic Fever

PME-Admin article, medical acute rheumatic fever, ARF, children, Group A Strep, infants, infections, pediatrics

Acute Rheumatic Fever (ARF) — Santa J. Bartholomew M.D.

The incidence of ARF is 8-51/100,000 people worldwide. It most commonly affects children ages 5-15 years after having a Group A Streptococcal infection (Strep throat). Overcrowding and poverty are directly proportional to the incidence of ARF as strep can sweep through a community and untreated can lead to acute rheumatic fever. The incidence of untreated Group A strep leading to Acute Rheumatic Fever is approximately 3% and recurrence is 50% in children who have had ARF. Timely treatment of strep throat can prevent acute rheumatic fever and is the most effective way to prevent all rheumatic heart disease (RHD).

PATHOPHYSIOLOGY

Group A Strep (GAS) is also known as streptococcus pyogenes is a gram-positive bacterium with more than 100 different serotypes but only serotype B causes disease. A protein on the outside of the cell called Protein M helps the bacterium cling to the skin of the host (typically a child) and cause illness. This protein causes a large inflammatory response and the clinical symptoms that the children experience. The immune response causes the child’s body to make proteins that can attack the muscle of the heart. This usually occurs 10 days to 5 weeks after the Group A strep pharyngitis.

The immune response causes the child’s body to make proteins that can attack the muscle of the heart.

CLINICAL DIAGNOSIS

GAS pharyngitis (strep throat) rarely affects children younger than 3 years old. Typically, children experience a sore throat, fever and headache. Some children may have nausea, vomiting and abdominal pain. On physical examination the physician may find exudate in the throat and on the tonsils but not always, redness or erythema. There are often swollen lymph nodes in the neck and sometimes petechiae on the soft portion of the palate. The uvula at the back of throat may be red and swollen. There maybe a fine red, sandpaper like rash over the child’s skin. It is also helpful to know if the child was near another recently diagnosed with strep throat.

Evidence of GAS infection can be obtained by drawing blood for titers in the blood to group A strep, in the acute setting throat swabs can help make the diagnosis of strep throat and thus quick treatment initiated avoiding ARF.

DIAGNOSIS OF ARF

Acute rheumatic fever is diagnosed using modified Jones criteria that were fist published in 1944 and have undergone multiple revisions through the years the last time in 2015 and are still used today.

The initial diagnosis of ARF is positive if there are 2 major criteria or 1 major and 2 minor criteria present along with evidence of persistent streptococcal infection. Further the presence of Sydenham Chorea is by itself enough to diagnose acute rheumatic fever. Sydenham chorea is a movement disorder in which the child has purposeless involuntary movements that disappear when the child is asleep. Face and hands are mostly involved and can only be suppressed very briefly. Children have deterioration in their handwriting, they can have slurred speech and emotional lability.

The Modified Jones Criteria (2015)

LR Populations

Major Criteria	Minor Criteria
Carditis-Clinical and/or subclinical	Polyarthalgia
Arthritis Polyarthritis only	Fever (≥ 38.5ºC)
Chorea	ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dL
Subcutaneous nodules	Prolonged PR interval after accounting for age variability
Erythema marginatum

Moderate Risk to HR Polulations

Major Criteria	Minor Criteria
Carditis-Clinical and/or subclinical	Monocarthralgia
Arthritis Monoarthritis or polyarthritis Polyarthralgia	Fever (≥ 38ºC)
Chorea	ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dL
Subcutaneous nodules	Prolonged PR interval after accounting for age variability
Erythema marginatum

CRP, C-reactive protein: ESR, erythrocyte sedimentation rate. For all patient populations with evidence of preceding Group A streptococcal infection. Diagnosis of initial ARF: 2 major manifestations or 1 major plus 2 minor manifestations.

TREATMENT

Primary treatment

prevention of the primary streptococcal infection to avoid ARF completely with the use of oral penicillin or IM doses of penicillin G, or like antibiotics. Prevention of repeated GAS infections is the most efficient way to prevent progression of ARF to rheumatic fever.

Secondary treatment

supportive and is not based on robust clinical trials, the mainstay is anti-inflammatory agents that will help with joint and cardiac inflammation but to date there is little evidence that these change outcome.

ARTHRITIS
- Ambulatory restriction for arthritis for 6 weeks
- High dose aspirin for typically 1-4 weeks
- On occasion corticosteroids are used for persistent inflammation
ACUTE CARDITIS
- Restricted activity for 4-6 weeks
- Aspirin
- Prednisone and/or Immunoglobulin in severe cases
- Treatment of heart failure associated with carditis
- Surgical procedures for valve problems associated with severe RHD.
SYDENHAM CHOREA
- Usually resolves spontaneously in 4-5 weeks
- Dopamine antagonists have been used with some success.
- Carbamazepine and Valproate have been used for prolonged movement problems.

ARF and RHD continue to be a major health problem in the United States and around the world. The most common clinical features of ARF are arthritis, chorea and carditis. ARF can be controlled by a combination of prevention, prompt recognition and treatment of Strep infections and improving access to health care.

March 22 2022

Foreign Body Ingestions

PME-Admin article, medical children, foreign body ingestion, infants, pediatrics

Foreign Body Ingestion: What Needs to Come Out! — Santa J. Bartholomew M.D.

Toddlers and young children often put things in their mouth and are also unable to tell care providers, both family and healthcare, what they put in their mouth, so it can be a challenge to decide what needs to be removed quickly and what can move through the gastrointestinal tract. Unfortunately, even benign foreign bodies that may not cause harm to the child often get caught at the proximal esophagus or mid-esophagus ( near the aortic arch) and cannot pass into the stomach.

Even benign foreign bodies that may not cause harm to the child often get caught at the proximal esophagus or mid-esophagus and cannot pass into the stomach.

THE MOST COMMON FOREIGN BODIES + CARE

COINS — The most commonly ingested objects in children in the United States. In about 30% of children these pass spontaneously- one the coin passes to the stomach it is very likely to clear without issue. Since Canadian quarters are larger than US coins, they more often need to be removed endoscopically particularly in children under 5 years old.

Children may be asymptomatic or may present with drooling, pain on swallowing or respiratory distress.
Evaluation should include chest, abdomen x-rays both AP and lateral to determine coin from batteries.
Treatment: Coins lodged in the esophagus should be removed within 24 hours to minimize risk of esophageal erosion or injury. Coins that have already entered the stomach can be observed without intervention unless there is unremitting abdominal pain.

BUTTON BATTERIES — The most common batteries found in a household they are found in watches, toothbrushes, toys and musical greeting cards. The ingestion of a button battery is a medical emergency.

Once the battery contacts the esophageal mucosa it makes hydrogen radicals which result in chemical damage to the esophagus. Second, the electrical current that occurs when both sides of the batteries touch the wall of the esophagus can also damage the esophagus. In animal models these injuries can occur within 15 minutes of ingestion.

The change in batteries for toys and watches from 3V to 20mm lithium button batteries have led to a significant increase in ingestions.
Children typically present with drooling, fussiness, fever, refusal to feed and respiratory distress/stridor.
Plain x-rays should be obtained in all suspected ingestions- clues on x-ray to a button battery may be a halo sign or double shadow.
Esophageal battery ingestions should be removed within 2 hours regardless of presence of symptoms or not.
If the battery has left the esophagus it can be observed for 72 hours and the use of Carafate or honey to neutralize the pH is now recommended.
Button battery ingestions have many morbidities even when handled appropriately from both the pH and the electric charge such as tracheoesophageal necrosis and subsequent stricture, vocal cord injury, aorto-enteric fistula, mediastinitis.

SHARP OBJECTS — The frequency of ingestion of objects like pins, knives, glass, bones, sharp plastic can vary by cultural but is also part of a rare childhood disease, PICU and done by dare in teenagers and are not very common. Objects that are larger than 25 mm most likely will not pass from stomach into the bowel and will need to be removed. Objects that become lodged in the esophagus hold the highest risk for perforation and its consequent side effects. These require surgical removal with surgery and/or ENT.

X-rays should be performed to assess the location of the object but remembering that not every object can be seen on an x-ray such as wood, some plastic, bone and glass. Complications of ingestions such as these are perforation of the esophagus with mediastinitis, abscess in the mediastinum, organ penetration, rupture of the aorta, bowel perforation, bowel obstruction or bowel perforation and death.

Although it seems counterintuitive many times these ingestions can remain asymptomatic for long periods of time making care for them more difficult when recognized.

MAGNETS — Frequency of ingestion of magnets has increased in the last 10 years. These ingestions typically include more than two magnets and although one magnet may be able to pass the GI tract with minimal issue, multiple magnets are more difficult to pass or magnets attached to other foreign bodies. The neodymium magnet found in toys is 5 x more attractive than standard magnets. These are used in toys, body piercings, facial piercings and appear on x ray like small ball bearings.

Physical examination should concentrate on if the child has symptoms of bowel obstruction since the batteries can stick together causing a large mass. Emergency endoscopy is the treatment of choice.

In single small magnet ingestion observation and laxative therapy can be utilized. Endoscopy is only indicated when magnet is large or of unusual shape or pointed that may not pass the GI tract easily. Occasionally, surgical laparotomy may be needed if magnet is in the terminal ileum to prevent perforation.

pme foreign body ingestion of absorptive objects

ABSORPTIVE OBJECTS — such as toys that grow in water, water beads, diapers, feminine pads become very dangerous when ingested because they rapid expand resulting in acute bowel obstruction.

These items are typically not seen on regular x-rays so diagnosis may be delayed. Contrast studies may be performed to confirm diagnosis but SHOULD NOT delay definitive therapy.

Treatment is an Emergency Endoscopy.

March 8 2022

Kawasaki Disease in Children

PME-Admin article, medical children, infants, kawasaki disease, pediatrics

Kawasaki Disease in Children — Santa J. Bartholomew M.D.

Kawasaki Disease is an acute, self-limited disease of the blood vessels including the coronary arteries of the heart occurring in infants and young children. It is usually manifested by high fever, inflammation of the mouth, tongue, and eyes (causing peeling and cracked lips, swollen and red tongue and red, swollen but not crusty eyes) and swollen lymph nodes in the neck. The inflammation of this disease also targets the coronary arteries of the heart and other cardiovascular structures. There are diagnostic criteria that assist health care providers in accurately diagnosing Kawasaki disease that have been published by the American Heart Association (AHA) as seen below.

CLASSIC KAWASAKI DIAGNOSIS

Classic KD is diagnosed in the presence of fever for at least 5 d (the day of fever onset is taken to be the first day of fever) together with at least 4 of the 5 following principal clinical features. In the presence of ≥4 principal clinical features, particularly when redness and swelling of the hands and feet are present, the diagnosis of KD can be made with 4 d of fever, although experienced clinicians who have treated many patients with KD may establish the diagnosis with 3 d of fever in rare cases.

Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa
Bilateral bulbar conjunctival injection without exudate
Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like
Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase
Cervical lymphadenopathy (≥1.5 cm diameter), usually unilateral

Laboratory tests typically reveal normal or elevated white blood cell count with neutrophil predominance and elevated acute phase reactants such as C-reactive protein and erythrocyte sedimentation rate during the acute phase. Low serum sodium and albumin levels, elevated serum liver enzymes, and sterile pyuria can be present. In the second week after fever onset, thrombocytosis is common.

There are also children who may have a “non-classic” presentation and the diagnosis of “Incomplete Kawasaki Disease” has been described in a similar algorithm from the AHA.

Once diagnosis is made echocardiogram is the mainstay of cardiac evaluation in the acute phase looking for coronary artery aneurysms which will affect the morbidity and mortality of the child affected.

There is no one lab test that makes the diagnosis of KD. The mortality and morbidity associated with this disease depends on how quickly the clinical signs are recognized and diagnosis made.

TREATMENT

The goal of therapy is to abort the inflammatory response and possibly stop thrombosis associated with it. IVIG is the most efficient method to accomplish this and helps diminish the incidence of giant coronary artery aneurysms. IN children with aneurysm at diagnosis antiplatelet therapy is also reasonable.

In children with coronary artery aneurysms the goal of long term therapy is to avoid thrombosis of arteries and ischemic heart disease but the ultimate goal is to recognize this disease early and abort the inflammatory response with IVIG.

Chart of Evaluation of Suspected Incomplete Kawasaki Disease

1 2

infants

ApneaBetsy S. Kastak DNP, RN C-PNP + Santa J. Bartholomew MD

kinds of apnea

Apnea in Children

RESOURCES

MonkeypoxCynthia D. Read, CPNP-PC, LNC & Santa J. Bartholomew MD

Signs and Symptoms of Monkeypox

Two Phases of Infection

Complications of Monkeypox

How is Monkeypox Transmitted

Diagnosis and Treatment

Treatment includes

Prognosis

Vaccination

Prevention

RESOURCES

Extubation FailureSanta J. Bartholomew MD

Causes of Extubation Failure

Consequences of Extubation Failure

Predictors of Extubation Failure

Management of Failed Extubation

RESOURCES

Headaches in ChildrenSanta J. Bartholomew MD

GETTING A HEADACHE HISTORY

Focused Neurologic Examination

Primary Headaches

Secondary Headaches

RESOURCES

Pediatric EncephalitisSanta J. Bartholomew MD & Cynthia D. Read, LNC

How does Encephalitis happen?

TABLE 1: Flowchart showing categorization of childhood (age ≤14 years) suspected encephalitis cases identified by the Australian Childhood Encephalitis study, 2013-2016.

Causes

SYMPTOMS

NEUROLOGIC DISFUNCTION ENCEPHALITIS

Clinical features

HOW DO WE DIAGNOSE ENCEPHALITIS?

TREATMENT

PROGNOSIS

RESOURCES

Abdominal Pain Part V: Acute PancreatitisSanta J. Bartholomew MD & Cynthia D. Read, LNC

The Pancreas

Classification

TABLE 1: DEFINITIONS OF PANCREATITIS IN CHILDREN (INSPPIRE Criteria)

Etiology

Diagnosis

Classic Examination Findings

DIFFERENTIAL DIAGNOSIS OF PANCREATITIS

LABORATORY SIGNS

IMAGING

Ultrasound of a Human Pancreas

MANAGEMENT AND TREATMENT

Nguyen,T. (n.d.). Pancreatitis [Review of Pancreatitis]. 41(10).

COMPLICATIONS AND PROGNOSIS

Pancreatic Pseudocyst

Pancreatic Necrosis

RESOURCES

Abdominal Pain Part IV: Inflammatory Bowel Disease and Ulcerative ColitisSanta J. Bartholomew M.D.

What is the cause of IBD?

Symptoms of IBD

Crohn's Disease

Ulcerative Cholitis

Inflammatory Bowel Disease

Diagnosis

Differential Diagnosis Considerations with Inflammatory Bowel Disease

MORE LIKELY TO PRESENT WITH CHRONIC COMPLAINTS

MORE LIKELY TO PRESENT MORE ACUTELY

COMORBID SIGNS AND SYMPTOMS OF INFLAMMATORY BOWEL DISEASE BY ORGAN SYSTEM

Treatment for IBD

RESOURCES

PATHOPHYSIOLOGY

CLINICAL DIAGNOSIS

DIAGNOSIS OF ARF

The Modified Jones Criteria (2015)

LR Populations

Moderate Risk to HR Polulations

TREATMENT

Primary treatment

Secondary treatment

THE MOST COMMON FOREIGN BODIES + CARE

CLASSIC KAWASAKI DIAGNOSIS

Apnea
Betsy S. Kastak DNP, RN C-PNP + Santa J. Bartholomew MD

Monkeypox
Cynthia D. Read, CPNP-PC, LNC & Santa J. Bartholomew MD

Extubation Failure
Santa J. Bartholomew MD

Headaches in Children
Santa J. Bartholomew MD

Pediatric Encephalitis
Santa J. Bartholomew MD & Cynthia D. Read, LNC

Abdominal Pain Part V: Acute Pancreatitis
Santa J. Bartholomew MD & Cynthia D. Read, LNC

Abdominal Pain Part IV: Inflammatory Bowel Disease and Ulcerative Colitis
Santa J. Bartholomew M.D.