UMEM Educational Pearls - Pediatrics

Teaching has circulated that a temperature of 40 degrees Celsius or above (hyperpyrexia), was associated with a greater incidence of serious bacterial infection.  However, this teaching originated in a time prior to the availability of childhood vaccinations. In fact, the largest retrospective study to support this used data from 1966-1974.  

2565 WELL APPEARING patients between the ages of 61 days and their 18th birthday who presented to a single tertiary care pediatric emergency department with the chief complaint of fever were included.  The prevalence of serious bacterial infection was compared to the presence of hyperpyrexia, age, chronic conditions, gender and vaccination status.

Serious bacterial infections (SBIs) included: deep space infections, appendicitis, pneumonia, mastoiditis, lymphadenitis, acute bacterial rhinosinusitis, urinary tract infection, pyelonephritis, cholecystitis, tubo-ovarian abscess, septic arthritis, osteomyelitis, bacteremia or bacterial meningitis.

There was NO statistically significant association between hyperpyrexia and SBIs. Older age and make sex were associated with a higher risk of SBIs.

Bottom line: In well appearing children 61 days and older, having a temperature >/= to 40 degrees was not associated with serious bacterial infections.

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Category: Pediatrics

Title: Never miss a supracondylar fracture!

Keywords: pediatrics, orthopedics, fracture (PubMed Search)

Posted: 6/6/2024 by Kathleen Stephanos, MD (Emailed: 6/10/2024) (Updated: 6/10/2024)
Click here to contact Kathleen Stephanos, MD

The supracondylar fracture is one of the most common pediatric fractures. It typically occurs due to a FOOSH injury and is a result of fracture through an area of high growth (and therefore weaker bone structure) in the pediatric distal humerus. Appearance on x-ray depends on the degree of displacement, however in cases without obvious displacement, providers must look for more subtle signs on x-ray. For example, a “sail sign” of the anterior fat pad and appearance of a posterior fat pad indicate a joint effusion and are suggestive of a fracture. However, there are often still equivocal x-rays in children with notable tenderness on exam, and failure to appropriately immobilize these fractures can result in pain and higher risk of injury resulting in displacement. 

In 2021, Varga et al, looked into the ability to assess for supracondylar fractures with ultrasound. This prospective study evaluated 5 locations in the pediatric elbow for signs of fracture. Ultimately, this was able to identify more fractures than x-ray alone, and was a useful tool for equivocal cases. This is not an isolated study, but one of the most comprehensive, looking into ultrasound as a tool for supracondylar fracture identification.

It may be time to grab that ultrasound probe to look for fractures in pediatric patients with pain but an unclear elbow x-ray.

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Category: Pediatrics

Title: Skeeter Syndrome

Posted: 5/31/2024 by Rachel Wiltjer, DO (Updated: 6/21/2024)
Click here to contact Rachel Wiltjer, DO

Histamine is present in mosquito saliva contributing to itch with bites, however, certain populations – including children – can experience an exaggerated reaction. Skeeter syndrome is a large, localized inflammatory reaction secondary to a mosquito bite that presents with warmth, swelling, and itching. There can occasionally be associated lymphadenopathy and fever as well. The rapid onset is what best differentiates it from cellulitis. Treatment is primarily symptomatic in nature, focused on relieving itch, with antihistamines and topical therapy. There may also be a role for prophylactic antihistamine usage at times when mosquito bites will be unavoidable in a patient known to have developed Skeeter syndrome previously. Other patients who may be affected include those with immunologic and autoimmune phenomena, those with underlying atopy, outdoor workers with frequent exposure, and those with new exposure to indigenous mosquitos. 

Take Home: Consider insect (mosquito bite) when evaluating for cellulitis/infection in pediatric patients, with impressive skin findings but otherwise unremarkable exams. They present with rapid onset and itching instead of pain. This can be especially prominent in periorbital and auricular presentations.

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Category: Pediatrics

Title: Seasonality of pediatric visits for suicidality

Keywords: psych, pediatric mental health, suicide (PubMed Search)

Posted: 5/17/2024 by Jennifer Guyther, MD (Updated: 6/21/2024)
Click here to contact Jennifer Guyther, MD

This was a cross sectional study examining insurance data to determine if there is seasonality related to ED visits and psychiatric hospitalizations related to suicidality.

Suicidality includes both suicidal ideation and suicide attempts (but not suicide).  One survey showed that up to 12% of US adolescents reported serious thoughts of suicide.  This study included 73,123 patients where 19.4% were direct inpatient admissions and 80.6% were ED visits, 44% of whom were subsequently admitted.

Metrics for suicidality in 10-18 year olds peaked in April and October with a nadir in June.  Incidentally, in the Spring of 2020 when US schools closed due to COVID, there was a decrease in both ED visits and hospitalizations with April and May having the lowest rates across the study period.

School is believed to increase stress with risk factors such as bullying and peer pressure, academic and extracurricular stressors and poor sleep hygiene.

Bottom line: There has been an increase in adolescent suicidality over the recent years, many of whom present to the ED for evaluation.  More mental health resources are needed, especially during the school year.

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Intranasal medications are an increasingly popular option for pediatric patients, particularly for analgesia and anxiolysis, with an increasing number of medications being used via the intranasal route of administration. 

Fentanyl has been shown in prior studies to be a safe and effective pain management strategy for children, but is likely under utilized. In sickle cell patients, studies have shown that time to analgesia may improve outcomes including hospitalization. 

In 2023, Rees et al. showed that in the sickle cell patient population IN fentanyl can be a very effective tool for patient's experiencing a Vaso-occlusive episode (VOE). This study looked at 400 children with a mean age of 14.6 years. Of these 19% received IN fentanyl.

Ultimately, the IN fentanyl patient population had a shorter time to initial administration of analgesia and a lower chance of admission to the hospital. 

Notably, this was not a randomized study, so there is limitations in assessment of the causality of the lower discharge rates. However this is a tool that could likely be used more regularly in the pediatric sickle cell patient population to allow for more rapid pain management in the emergency department.

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This was a multicenter, randomized double blind, placebo controlled, non inferiority trial looking at children aged 2 months to 10 years with a diagnosis of urinary tract infection to see if the antibiotic course could be shortened from 10 days to 5 days in those patients who have clinically improved by day 5.

Children were prescribed amoxicillin/clavulanic acid, cefixime, cefdinir, cephalexin or trimethoprim-sulfamethoxazole and on day 6, after an in person visit were switched to placebo or continued the same antibiotic course.

A urine sample was collected on days 11-14 and treatment failure was defined as symptomatic urinary tract infection at or before this visit, asymptomatic bacteriuria, positive urine culture or gastrointestinal colonization with resistant organisms.

693 children were randomized in this trial.  Children who received 5 days of antibiotics were more likely to have asymptomatic bacteriuria or a positive urine culture on days 11-14 (0.6 vs 4.2%).  28 children would need to be treated with a 10 day course to prevent one treatment failure with the 5 day course.

Bottom line: 10 days is still the ideal duration of treatment for a urinary tract infection, but the rate of failure of a 5 day course was low and the clinical significance of asymptomatic bacteriuria or a positive urine culture in an otherwise asymptomatic child is unknown.

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Category: Pediatrics

Title: Are YOU Ready? Check your Pediatric Readiness Score.

Keywords: Pediatrics, preparedness (PubMed Search)

Posted: 4/5/2024 by Kathleen Stephanos, MD (Emailed: 4/12/2024) (Updated: 4/12/2024)
Click here to contact Kathleen Stephanos, MD

In early 2023 Newgard et al published an article in JAMA which looked at pediatric readiness in ED's across the county. This study showed that there was a significant increase in pediatric mortality in patients who presented to EDs with lower readiness scores (<87 out of 100) when compared to those with higher readiness scores. And this translated to not just the time in the ED, but up to a year after they are seen in an ill-prepared ED. This number equated to an estimated total of 1,500 preventable deaths in children in the US each year. 

Notably this does NOT look at what designation your hospital has for pediatrics (so being a level 1 pediatric trauma center does not automatically give you any points). This is based on having the physical materials needed for each age group, plans in place for specific patient age groups and evaluations (lower radiation doses for children in CT, using an US before CT for appendicitis evaluation, etc), and a person/people in place to review cases and ensure everyone is up to date on pediatric related training. 

Want to check YOUR score? Go to https://www.pedsready.org/

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Category: Pediatrics

Title: Hot off the Press! A Pediatric Sepsis Update

Keywords: pediatrics, sepsis, SIRS, septic shock (PubMed Search)

Posted: 4/5/2024 by Kelsey Johnson, DO (Updated: 6/21/2024)
Click here to contact Kelsey Johnson, DO

Sepsis remains a leading cause of mortality in the pediatric population, and we have long been overdue for an update in recognizing and risk stratifying our pediatric patients. 

Until now, pediatric sepsis screening guidelines have followed a framework modeled after the adult screening criteria from 2001 (Sepsis-2): Systemic Inflammatory Response Syndrome (SIRS). While attempts were made in 2005 to adjust these criteria to the pediatric population, data has shown poor specificity correlating to PICU admissions, need for critical interventions, end organ damage, and mortality. 

As a reminder: Pediatric SIRS criteria: Temp >38.5 C or <36 C, tachycardia (or bradycardia if <1yr old), Tachypnea, leukocytosis/ leukopenia or >10% bandemia

In 2016, Sepsis-3 revised the criteria for sepsis and septic shock for the adult cohort by placing the focus on the presence of life-threatening organ dysfunction, which led to adopting criteria such as the SOFA and qSOFA scores to predict mortality risk. 

Enter: The Phoenix Sepsis Score

An international, multidisciplinary task force convened by the Society of Critical Care Medicine in 2019 used evidence from an international survey, systematic review and meta-analysis to develop and validate a scoring tool intended to identify life-threatening organ dysfunction in children. Scoring tool was derived and validated retrospectively using an international, multi-center electronic medical record review of 3 million pediatric hospital encounters (excluding pre-term infants born <37 weeks and birth hospitalizations). 

A Phoenix score of at least 2 carried a 7.1% risk of in-hospital mortality (in higher resource settings such as the USA). 

Children with sepsis and organ dysfunction remote from the primary site of infection (eg respiratory failure in a child with meningitis) carried the highest mortality burden (8%) in this study. 

Sepsis is identified in children with suspected infection and an overall Phoenix score of 2 or more points, as this indicates potentially life-threatening organ dysfunction and thus carries higher mortality risk. Septic shock is identified by a score of 1 or more point(s) in cardiovascular dysfunction. 

Lastly, it is important to recognize that this scoring criteria is NOT a sepsis screening tool, but rather should be applied when there is clinical suspicion for significant infection in the unwell child, or meeting sepsis screening criteria per your institutional criteria. Screening pediatric patients remains a clinical challenge, and there is not yet a reliable tool to apply to this population. 

Bottom Line: Sepsis remains a significant cause of pediatric morbidity and mortality. The Phoenix Sepsis Score should be applied to the unwell child with suspected infection to identify sepsis and septic shock, thereby providing risk stratification and improving clinical care. Sepsis is defined by a Phoenix score of 2 or more and septic shock by a score of 1 or more in the cardiovascular category.

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Standard practice regarding various pediatric fractures has started to shift over the last several years, often to less restrictive means of treatment. Torus (buckle) fractures of the distal radius are one of the most common pediatric fractures and tend to heal very well with minimal intervention. 

The FORCE study (FOrearm fracture Recovery in Children Evaluation), a multicenter study out of the UK, was conducted to compare rigid immobilization (splinting) to a soft bandage used as needed per family discretion for treatment of these fractures. There was no different in outcomes of self-reported pain, function, quality of life, complications, or school absences. UK orthopedic guidelines have been updated to reflect a recommendation against rigid immobilization as well as against any need for specialist follow-up. American guidelines are slower to follow suit, but in recent years have transitioned to an approach of a removable brace. 

Take Home: Pediatric torus fractures of the distal radius likely do not require immobilization and can be managed with self-limited activity instead. Practice in the US is in flux, but it is reasonable to manage with a removable brace or soft dressing as well as pediatrician follow up.

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The gold standard for confirming ETT position is a chest xray, but this can often be delayed while the patient is stabilized. Many physicians will estimate ETT insertion depth to be 3x the ETT size, but this is based on selection of the correct tube.  There are several other published formulas, including the PALS guidelines [age in years/2 + 12] which applies to children older than one year.  In 1982, there was an article published that cited the formulas of [Height (cm) x 0.1 +5] or [Weight(kg)/5 + 12].

This was a retrospective study where the ideal position of cuffed ETT (from the front teeth) was determined by looking at post intubation xrays of 167 patients between 28 days and 18 years.  The individual optimal ETT insertion depth was plotted against age, weight and height for all children.  This study showed that there is not a fully linear relationship between age, height or weight which is a flaw of all of these formulas.  Calculations using the patients’ weight performed the worst.  Age based and height formulas performed the best.

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Category: Pediatrics

Title: Pearl on Tessalon Perles: One pill can kill

Posted: 2/29/2024 by To-Lam Nguyen, MD (Emailed: 3/1/2024) (Updated: 3/1/2024)
Click here to contact To-Lam Nguyen, MD

You've heard of one kill pills such as calcium channel blockers, beta blockers, sulfonylureas, anti-malarials, but less commonly known is benzonatate, or tessalon perles.

Tessalon perles are not recommended for children under the age of 10. 1-2 capsules of benzonatate in children <2 years old have been reported to cause serious side effects including restlessness, tremors, convulsions, coma and even cardiac arrest rapidly after ingestion (within 15-20 minutes and death within a couple of hours). It is attractive to young children as it somewhat resembles a jelly bean. 

Pearls on Tessalon Perles:

  • Always ask your patients about children in their household and strongly consider alternative to tessalon perles if children <10 years in home
  • If you do decide to prescribe, prescribe only the amount of tessalon perles that a patient needs for cough relief
  • Inform your patients about this high risk of accidental ingestion by children
  • Keep tessalon perles in child-resistant container and keep it out of reach of children AT ALL TIMES
  • Call the poison center (800-222-1222) immediately if accidental ingestion by child

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Category: Pediatrics

Title: What is BACM?

Keywords: myositis, acute kidney injury, problems walking, calf pain (PubMed Search)

Posted: 2/14/2024 by Jennifer Guyther, MD (Emailed: 2/16/2024) (Updated: 2/16/2024)
Click here to contact Jennifer Guyther, MD

BACM stands for benign acute childhood myositis which is typically a benign, viral induced self limiting illness.  This was a retrospective study looking at 65 patients in Italy to further characterize the characteristics of the disease.

In this study, the median patient age was 6 years with a male predominance.  The incidence of BACM peaked in winter with a second peak in the fall.  Patients presented with prodromal symptoms including fever, cough, coryza, sore throat and vomiting.  The exam showed difficulty walking and myalgias with reproducible calf tenderness and preserved reflexes.  Influenza B and A, COVID and other viral pathogens have been detected in these patients.

Lab work may show an elevated creatinine kinase, AST and potassium.  WBC and CRP may also be elevated.  The median CK value was 943 U/L and on average normalized within one week.  Other studies have shown median CK values in the 3300s. Treatment includes hydration to promote CK clearance and prevent complications including acute kidney injury related to rhabdomyolysis.  Recurrent myositis or CK values > 5000 U/L should have screening tests for muscular dystrophy and metabolic disorders.

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It is an often asked question: should I consider the corrected or the chronologic age when determining the need for fever evaluation in a pediatric patient? The 2021 AAP guidelines for the well appearing febrile neonate are widely accepted and apply to neonates under 60 days. These highly practical guidelines are, unfortunately, not applicable to pre-term neonates. The question often becomes what age to use for a pre-term neonate- the age they actually are, or the age they would be if they had completed a full term gestation. 

Hadhud et al attempted to clarify the age utilized in a retrospective review. This looked at febrile 448 pre-term neonates evaluated for fevers. It found that those patients with both a corrected and chronologic age over 3 months had a 2.6% rate of serious bacterial infections or SBI (UTI, bacteremia or meningitis), those with a corrected age under 3 months but a chronologic age over 3 months had a 16.7% rate of SBIs, and those with both a corrected and chronologic age of under 3 months had a 33.3% rate of SBI. 

Overall, these rates of infection are higher than the typically reported in febrile neonates, supporting that pre-term neonates have a much higher risk of infections overall. Ultimately, pre-term neonates should be carefully assessed and a more thorough evaluation is typically warranted in this patient population even if they have reached the generally accepted 60 day marker by chronologic age- use the corrected age.

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Category: Pediatrics

Title: Pediatric bounce backs

Keywords: Bouncebacks, high risk discharges, gastroenteritis, death (PubMed Search)

Posted: 1/19/2024 by Jennifer Guyther, MD (Updated: 6/21/2024)
Click here to contact Jennifer Guyther, MD

Revisits back to the ED within 3 days of the initial visit represent a standard quality measure.  A critical ED revisit was defined as an ICU admission or death within 3 days of ED discharge.  This study looked at 16.3 million children who were discharged from various EDs over a 4 year period and found that 0.1% (18,704 patients) had a critical revisit and 0.00001% (180 patients) died.  

The most common diagnosis at the initial visit of those patients coming back with a critical revisit included: Upper respiratory infections, gastroenteritis/nausea/vomiting and asthma.

The most common critical revisit diagnosis were: asthma, pneumonia, cellulitis, bronchiolitis, upper respiratory infections, respiratory failure, seizure, gastroenteritis/nausea/vomiting, appendectomy and sickle cell crisis. Among the patients who died, 48.9% were younger than 4 years. Patients with complex medical problems and patients seen at a high volume center were more likely to have a critical ED visit.

Bottom line: These ED revisits may not have been related to missed diagnosis (with the exception of appendicitis), but rather due to the natural progression of certain disease processes.  Patients with these diagnoses may benefit from careful reassessment, targeted patient education, more specific return precautions and closer outpatient follow up.

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Urinary tract infection (UTI) is the leading cause of fever without a source in infants younger than 3 months.  This data was collected from patients who presented to the emergency department with fever without a source over a 16 year period.  Out of 2850 patients, 20.8% were diagnosed with a UTI, the majority of which grew E coli.  Of those patients who were diagnosed with UTI, these patients were more likely to have a history of renal/GU problems, have a fever of at least 39C (38% vs 29%) or poor feeding (13% vs 8.7%).  However, 48% had none of these risk factors.  Also 6.1% of patients with a febrile UTI had another invasive bacterial infection.  These patients were more likely to be < 1 month, be "irritable" per parents and have an elevated procalcitonin and CRP.  

Bottom line:  A lack of risk factors can not exclude a UTI in febrile infants < 3 months.  A diagnosis of UTI also does not definitively exclude an additional invasive bacterial infection in a subset of these children.

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Since Christmas is coming up, let's talk about Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency, also known as Christmas disease)

Deficiencies in Factors VIII and IX are the most common severe inherited bleeding disorders.

Pathophysiology: 

  • Factors VIII and IX are required for activation of factor X.
  • In patients with Hemophilia A (factor VIII deficiency) or Hemophilia B (factor IX deficiency, also known as Christmas disease), after an injury, clot formation is delayed. 
  • Inadequate thrombin generation leads to failure to form a tightly crosslinked fibrin clot to support platelet plug, which leads to easy bleeding.
  • Clot that is formed may be friable and rebleeding occurs during physiologic lysis of clots or with minimal new trauma

Clinical Manifestations:

  • 2% of neonates with hemophilia have intracranial hemorrhages
  • 30% of male infants with hemophilia bleed with circumcision
  • Continued bleeding from umbilical stump in neonate
  • In absence of positive family history (hemophilia has high rate of spontaneous mutation), hemophilia may go undiagnosed in a newborn
  • Easy bruising, intramuscular hematomas, and hemarthroses (hallmark for hemophilic bleeding) begin when child begins to cruise
  • Bleeding from minor traumatic lacerations of the mouth (e.g. torn frenulum) can persist for hours or days
  • Iliopsoas hemorrhage: patient may lose large volumes of bleed into the muscle, leading to hypovolemic shock, with only a vague complaint of area of referred pain in the groin. Hip is held in a flexed, internally rotated position, due to irritation of the iliopsoas.
    • Confirmed on CT or US
    • Clinically unable to extend hip
  • Hemarthrosis rare in patients with acquired hemophilia

Lab findings and diagnosis

  • Reduced levels of factor VIII or factor IX will cause higher PTT 
  • PTT is usually 2-3x upper limit of normal in patients with severe hemophilia.
  • Platelet count, bleeding time, prothrombin time, and thrombin time are all normal
  • If PTT is not corrected after administration of factor VIII or IX, an inhibitor may be present.
    • 25-35% of patients with hemophilia who received infusions of factor VIII or factor IX, a factor specific antibody may develop (inhibitor)

Genetics

  • Hemophilia occurs in 1:5000 males, with 85% having factor VIII deficiency and 10-15% having factor IX deficiency
  • No apparent racial predilection, appearing in all ethnic groups

 

Classification

  • Severe hemophilia: <1% activity of specific clotting factor and bleeding is often spontaneous
  • Moderate hemophilia: 1-5% activity and require mild

trauma to induce bleeding

  • Mild hemophilia: >5% activity and can go many years before diagnosis and usually require significant trauma to induce bleeding.

Treatment

  • Ask patient or family if they brought their dosing information with them or their factor replacement with them. In many cases, they have it!
  • For life-threatening or major hemorrhages, dose should aim to achieve levels of 100% activity
    • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
    • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
    • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
    • If you don’t have factor-specific products:
      • Hemophilia A
        • can give 1U cryoprecipitate (~80U of factor VIII) or try PCC (as it contains factors II, VII, IX, and X)
        • activated PCC (FEIBA) 75-100U/kg
      • Hemophilia B
        • FFP NO LONGER RECOMMENDED (volume of FFP required has high risk of volume overload)
        • Cryoprecipitate does NOT contain factor IX, so will not work.
  • For acute bleeding in patients with mild hemophilia A:
    • Can give DDAVP: increases factor VIII by 3-5x by encouraging release of endogenous factor VIII. Recommended dose: 0.3mcg/kg/dose IV
  • For mild bleeding:
    • TXA (clot stabilizer)
    • Desmopressin
    • Aminocaproic acid
  • If patient has inhibitors:
    • Hemophilia A: 
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    • Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Summary:

  • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
  • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
  • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
  • Treatment if patient has no inhibitors:
    • Hemophilia A: 
      • Severe bleed: Give full dose factor XIII (50U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor XIII replacement (25U/kg), TXA, DDAVP, aminocaproic acid
    • Hemophilia B: 
      • Severe bleed: Give full dose factor IX (100U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor IV replacement (50U/kg), TXA, aminocaproic acid
  • Treatment if patient has inhibitors:
    • Hemophilia A: 
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    •  Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Show References



Since Christmas is coming up, let's talk about Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency, also known as Christmas disease)

Deficiencies in Factors VIII and IX are the most common severe inherited bleeding disorders.

Pathophysiology:

  • Factors VIII and IX are required for activation of factor X.
  • In patients with Hemophilia A (factor VIII deficiency) or Hemophilia B (factor IX deficiency, also known as Christmas disease), after an injury, clot formation is delayed.
  • Inadequate thrombin generation leads to failure to form a tightly crosslinked fibrin clot to support platelet plug, which leads to easy bleeding.
  • Clot that is formed may be friable and rebleeding occurs during physiologic lysis of clots or with minimal new trauma

Clinical Manifestations:

  • 2% of neonates with hemophilia have intracranial hemorrhages
  • 30% of male infants with hemophilia bleed with circumcision
  • Continued bleeding from umbilical stump in neonate
  • In absence of positive family history (hemophilia has high rate of spontaneous mutation), hemophilia may go undiagnosed in a newborn
  • Easy bruising, intramuscular hematomas, and hemarthroses (hallmark for hemophilic bleeding) begin when child begins to cruise
  • Bleeding from minor traumatic lacerations of the mouth (e.g. torn frenulum) can persist for hours or days
  • Iliopsoas hemorrhage: patient may lose large volumes of bleed into the muscle, leading to hypovolemic shock, with only a vague complaint of area of referred pain in the groin. Hip is held in a flexed, internally rotated position, due to irritation of the iliopsoas.
    • Confirmed on CT or US
    • Clinically unable to extend hip
  • Hemarthrosis rare in patients with acquired hemophilia

Lab findings and diagnosis

  • Reduced levels of factor VIII or factor IX will cause higher PTT
  • PTT is usually 2-3x upper limit of normal in patients with severe hemophilia.
  • Platelet count, bleeding time, prothrombin time, and thrombin time are all normal
  • If PTT is not corrected after administration of factor VIII or IX, an inhibitor may be present.
    • 25-35% of patients with hemophilia who received infusions of factor VIII or factor IX, a factor specific antibody may develop (inhibitor)

Genetics

  • Hemophilia occurs in 1:5000 males, with 85% having factor VIII deficiency and 10-15% having factor IX deficiency
  • No apparent racial predilection, appearing in all ethnic groups

 

Classification

  • Severe hemophilia: <1% activity of specific clotting factor and bleeding is often spontaneous
  • Moderate hemophilia: 1-5% activity and require mild

trauma to induce bleeding

  • Mild hemophilia: >5% activity and can go many years before diagnosis and usually require significant trauma to induce bleeding.

Treatment

  • Ask patient or family if they brought their dosing information with them or their factor replacement with them. In many cases, they have it!
  • For life-threatening or major hemorrhages, dose should aim to achieve levels of 100% activity
    • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
    • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
    • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
    • If you don’t have factor-specific products:
      • Hemophilia A
        • can give 1U cryoprecipitate (~80U of factor VIII) or try PCC (as it contains factors II, VII, IX, and X)
        • activated PCC (FEIBA) 75-100U/kg
      • Hemophilia B
        • FFP NO LONGER RECOMMENDED (volume of FFP required has high risk of volume overload)
        • Cryoprecipitate does NOT contain factor IX, so will not work.
  • For acute bleeding in patients with mild hemophilia A:
    • Can give DDAVP: increases factor VIII by 3-5x by encouraging release of endogenous factor VIII. Recommended dose: 0.3mcg/kg/dose IV
  • For mild bleeding:
    • TXA (clot stabilizer)
    • Desmopressin
    • Aminocaproic acid
  • If patient has inhibitors:
    • Hemophilia A:
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    • Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

Summary:

  • Aim for 50% correction in moderate bleeds and 100% correction in severe bleeds
  • Hemophilia A: 50U/kg recombinant Factor VIII (each U/kg of factor VIII in hemophilia A increases factor by 2%)
  • Hemophilia B: 100U/kg recombinant Factor IX (each U/kg of factor VIII in hemophilia A increases factor by 1%)
  • Treatment if patient has no inhibitors:
    • Hemophilia A:
      • Severe bleed: Give full dose factor XIII (50U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor XIII replacement (25U/kg), TXA, DDAVP, aminocaproic acid
    • Hemophilia B:
      • Severe bleed: Give full dose factor IX (100U/kg), even if the patient is on prophylaxis
      • Mild bleeds: factor IV replacement (50U/kg), TXA, aminocaproic acid
  • Treatment if patient has inhibitors:
    • Hemophilia A:
      • Activated PCC (75-100U/kg) (do NOT give if on patient is on emicizumab (Hemlibra) due to risk of thrombosis)
      • Recombinant factor VII 90mcg/kg
    •  Hemophilia B:
      • Recombinant factor VII 90mcg/kg

 

 

Show References



Category: Pediatrics

Title: Pediatric Lung Ultrasound

Keywords: POCUS, Pediatrics, Lung Ultrasound, Bronchiolitis (PubMed Search)

Posted: 11/20/2023 by Alexis Salerno, MD
Click here to contact Alexis Salerno, MD

Acute bronchiolitis (AB) is a common cause of respiratory tract infections in infants. A recent study looked at the application of Point-of-Care Lung Ultrasound (LUS) in infants <12 months who presented with symptoms of AB. 

They scored infant lungs using a cumulative 12-zone system. With the below scale: 

0 - A lines with <3 B lines per lung segment. 

1 - ≥3 B lines per lung segment, but not consolidated. 

2 - consolidated B lines, but no subpleural consolidation. 

3 - subpleural consolidation with any findings scoring 1 or 2. 

 

They found that infants with higher LUS scores had increased rates of hospitalization and length of stay.  

Here are some tips for ultrasounding a pediatric patient: 

 

  • Attempt to warm the gel
  • Have the parent/relative hold the patient while scanning
  • For those old enough, allow the child to investigate the ultrasound probe prior to placing the probe on the child.
  • Destract the child while performing scanning

 

Show References



Category: Pediatrics

Title: Does the timing of patient transfer impact mortality in the pediatric trauma patient?

Keywords: pediatric trauma, transport, time to destination (PubMed Search)

Posted: 11/17/2023 by Jennifer Guyther, MD (Updated: 6/21/2024)
Click here to contact Jennifer Guyther, MD

Pediatric patients treated at pediatric specific trauma centers have improved mortality.  However, it is estimated that only 57% of patients live within 30 miles of a pediatric trauma center.  This means that many children will need to be stabilized at an adult trauma center or community hospital prior to transfer.  This study showed that > 25% of injured children were transferred to a pediatric trauma center following stabilization at another hospital.
 
The American College of Surgeons has previously recommended that the optimal interfacility transfer time for trauma patients is 60 minutes.
 
Data for this study was extracted from a database fed by over 800 trauma hospitals.  Every minute increase in the interfacility transfer time is associated with a 2% increase in risk adjusted odds of mortality among severely injured pediatric trauma patients.
 
Bottom line: When faced with a moderate to severely injured pediatric trauma patient, the availability and time to transport should be taken into account. If the time is > 60 minutes, then mode of transport and destination (if others are available), should be considered.

Show References



Category: Pediatrics

Title: Neonate Resus Review

Keywords: Neonate, Newborn, resuscitation, NRP (PubMed Search)

Posted: 11/3/2023 by Kelsey Johnson, DO (Updated: 6/21/2024)
Click here to contact Kelsey Johnson, DO

Term? Tone? Tantrum?

Immediately after delivery, your initial neonatal assessment should evaluate for:

-       Appearance of full or late pre-term gestation (>34 weeks)

-       Appropriate tone (flexed extremities, not floppy)

-       Good cry and respiratory effort

 

Newborns meeting this criteria should not require resuscitation. They can be placed skin to skin on mother and allowed to breastfeed. Delayed cord clamping for 60 seconds is recommended, as data shows improved neurodevelopmental outcomes and iron stores in first year of life.

 

Neonates not meeting these criteria should be brought to the warmer for resuscitation, with the focus being on:

-       Warm - via radiant warmer. Maintain temps 36.5 C – 37.5 C

-       Dry - Neonates have thin skin and lose heat readily from evaporative loses

-       Stim - tactile stimulation on the head, midline of the back and extremities to provoke a cry and encourage respiratory effort

 

Avoid routinely bulb-suctioning unless there is significant obstructing mucous, as this can increase vagal tone and result in bradycardia. If bulb suctioning is used, first suction the mouth before the nose.

 

Majority of resuscitations do not require additional support, however if heart rate is <100 or there is poor respiratory effort, the physician should initiate PPV.

 

PPV settings:   PIP 20              PEEP 5              FiO2 21%         Rate of 60 breaths per minute

 

Improvement in the neonate’s HR is the primary indicator of effective PPV!

If HR poorly responding (remains <100), ensure appropriate mask size, reposition, suction, and increase PIP (max 35) and FiO2.

 

If HR drops below 60, intubate with uncuffed ETT

-       Prioritize adequate ventilation as this is the highest priority in neonatal resuscitation

-       Initiate compressions at rate of 120/min.

-       Epi dosing is 0.01-0.03 mg/kg q3-5 min

-       ETT size estimation by gestational age:

        25 weeks = 2.5, 30 weeks = 3.0, 35 weeks = 3.5, 40 weeks = 4.0