A 6 month-old male presents to the emergency room with progressively increasing work of breathing. A chest x-ray obtained shows the following:
Case courtesy of Dr Jeremy Jones, Radiopaedia.org. From the case rID: 24086
What are you concerned about?
This X-ray demonstrates cardiomegaly with prominent lung markings, which is suggestive of heart failure as the etiology of this infant’s respiratory distress.
Heart Failure in Children: The Basics
- In the US, the most common cause of pediatric heart failure is structural congenital heart disease. In children with structurally normal hearts, cardiomyopathy is the most common cause of heart failure. [Hsu et al, 2009]
- Heart failure presents when the metabolic demands of the body exceed cardiac output.
- Clinical Presentation is Diverse & Age related. [Madriago et al, 2010]
- Neonates: Feeding Difficulties and ultimately Failure to Thrive
- Older Children: Fatigue, GI symptoms, and exercise intolerance
- Physical Exam:
- “Pulmonary”: Tachypnea, Respiratory Distress (retractions, grunting), crackles on auscultation
- “Cardiac”: Tachycardia, gallop rhythm
- “GI”: hepatomegaly
Faculty Reviewer: Kristin Lombardi, MD
Radiology Reviewer: Laura Sternick, MD
Hsu DT et al. “Heart Failure in Children, Part I: History, etiology, and pathophysiology.” Circ Heart Fail. 2009;2:63-70
Madriago E et al. “Heart Failure in Infants and Children.” Pediatrics in Review. 2010;31(1)4-12.
Rosalie is a 2 year-old girl who comes in for her regular checkup. At the end of the visit, when you hand a lab slip to check hemoglobin and lead levels, her mom asks, “Why should we check her blood again? We did that last year and everything was fine. Last time, they had to try 3 different times to get blood, and I really don’t want her to get stuck again.”
What do you say?
Why should we care about elevated blood lead levels (BLLs)?
- NO BLOOD LEAD LEVEL IS CONSIDERED SAFE
Toxic Effects to the Following Organ Systems [Papanikolaou NC et al, 2005]:
- Developmental Delay: Decline in IQ, reduced academic performance, hyperactivity, increased aggression, etc (can been seen at levels ≥35μg/dL).
- Encephalopathy (BLL usually > 100μg/dL)
- Abdominal Pain
- Hematologic (effects observed at levels as low as 10 μg/dL)
- Cardiac, Renal
- According to the CDC, in 2014 4% of children tested in the United States had elevated BLL (>5 μg/dL)
- In Rhode Island, slightly more children (5%), had elevated BLLs
- Unfortunately, of the >24 million children <72 months, only 10% were actually screened.
- In Rhode Island, 39% of children <72 months were screened
Elevated Lead levels are more likely to affect children who are: [MMWR, 2013]
- Black, non-Hispanic
- Live in houses built before 1950
- Receive Medicaid
- Low socioeconomic status
Where is this lead located?
- Lead Paint (used in older houses; imported hand painted dishes, toys)
- Contaminated Soil (usually where leaded gasoline deposited)
- Water (Lead pipes); well water
- Natural Remedies (certain health tonics, digestive aids, or colic remedies)
When should you check Rosalie’s blood lead level?
- According to the CDC:
- Universal screening of all children, at 1 and 2 years of age (of note, ALL children on Medicaid MUST be screened)
- In Rhode Island it is mandatory to screen all children who are under 36 months at least twice (www.health.ri.gov). This is usually done at 12 and 24 months
- Children should be screened annually through 6 years of age
- After 2 negative blood levels, use risk assessment and screening tools to guide workup.
Her lead level returns elevated, what should you do?
- Consult with your State’s Lead Prevention Program Recommendation. Table below is from Rhode Island Department of Health
|Venous Blood Level
||Recommended actions for Primary Care Providers
|< 5 μg/dl
||No confirmation needed
||Provide Lead education and continue to assess for lead exposure
|5- 14 μg/dl
||Retest within 3 months
||· Explain lead level to parents
· Assess Nutrition
· Test Siblings < 6yo
· Provide Lead Education
||Retest within 3 months
||· Same recommendations as above
· Dept. of Health will refer to lead center
|20- 44 μg/dl
||Retest within 1 week
||· Refer for evaluation and treatment to local lead clinics
|≥ 45 μg/dl
||· As above, but consider hospitalization
Adapted from Rhode Island Department of Health Lead Screening and Referral Guidelines. Updated September 2013.
Rosalie’s lead level is 56, which is confirmed on repeat testing. What is the next step?
Moderate Intoxication (45-69mcg/L)
- Once level is confirmed, chelation should be started as follows:
- DMSA (Succimer)- this is the oral version of dimercaprol
- CaNa2EDTA- for children who cannot tolerate DMSA
- D-penicillamine (third line agent for children who cannot take 1 or 2 due to adverse affect).
Severe Intoxication (70mcg/L or encephalopathy)
- Repeat labs (especially serum lead); if confirmed
- Dimercaprol (BAL)
- Calcium disodium edetate (CaNa2EDTA)
- Disposition dependent on availability of follow-up, ensuring lead free household
- No blood lead level is considered safe
- Lead intoxication remains an important cause of preventable developmental delay
- All children should be screened at 12 and 24 months of age
- Know your state’s guidelines on Lead screening and Referral Guidelines!
Faculty Reviewers: Shuba Kamath, MD; Chandan Lakhiani, MD
Lead Poisoning in Popular Culture
“Blood Lead Levels in Children Aged 1–5 Years — United States, 1999–2010.” Morbidity and Mortality Weekly Report. 2013;62(13);245-248
Chandran L et al. “Lead Poisoning: Basics and New Developments.” Pediatrics in Review. 2010;31(10)399- 406.
Dietrich KN et al. “Effect of Chelation Therapy on the Neuropsychological and Behavioral Development of Lead-Exposed Children After School Entry.” Pediatrics. 2004;114(1)19-26.
Lowry JA et al. “Childhood lead poisoning: Management.” UpToDate. http://www.uptodate.com/contents/childhood-lead-poisoning-management. Accessed July 10, 2016.
Papanikolaou NC et al. “Lead toxicity update. A brief review.” Med Sci Monit.. 2005;11(10)RA329-336.
Schmidt, Silke and Dee Hall. “Failure at the faucet: Lead in drinking water poses danger for children, pregnant women.” http://wisconsinwatch.org/2016/01/140477/
“Lead Screening and Referral Guidelines.” Rhode Island Department of Health. September 2013
As you are starting your morning ED shift, your first patient is a 2-year-old boy, previously healthy, who is coming in with 5 days of fevers at home. Fevers have occurred daily and have been as high as 39.5ºC. His mother states that during this time he has been more fussy than usual and has been not been eating or drinking very well. Vital signs demonstrate a temperature of 38.9ºC, HR of 150, RR is 20. His BP is 110/60. On exam, he is fussy and appears ill. His conjunctiva are injected and you appreciate limbic sparing. Oropharyngeal exam is shown below. You also note a diffuse macular rash. What are you most concerned about?
Kole A, Chandakole D. N Engl J Med 2015;373:467-467.
Answer: The picture above shows a strawberry tongue with fissured lips, which in conjunction with 5 days of fever, rash and bilateral conjunctival injection is concerning for Kawasaki Disease (KD). KD is an acute vasculitis of still unknown etiology affecting primarily infants and children. KD affects coronary arteries, and without IVIG, 20% of children will go on to have coronary artery aneurysms (Newburger et al). Diagnosis of KD is primarily clinical, and requires 5 or more days of fever in conjunction with 4/5 clinical criteria (see below). For those children with persistent fever, but who lack all 4 criteria (child mentioned in case has 3), diagnosis utilizes lab and ECHO findings (listed below). In children with classic Kawasaki, as well as those with incomplete disease, the mainstay of acute management is IVIG paired with high-dose aspirin. For more information, please review the attached references.
Diagnostic Criteria of Kawasaki Disease (requires at least 5 days of fever)
- Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Desquamation of fingertips
- Polymorphous exanthema
- Bilateral, painless bulbar conjunctival injection without exudate
- Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
- Cervical lymphadenopathy (≥1.5 cm in diameter), usually unilateral
Diagnostic Criteria for children who have at least 5 days of fever and only 2-3 findings mentioned above:
- Obtain Labs: CRP/ESR, CBC, LFTs, and UA
- If CRP < 3 mg/dl AND ESR <40
- If fever continues, re-evaluate
- If fever defervesces, no follow-up required (EXCEPTION: in children who develop desquamation, an ECHO should be obtained).
- If CRP ≥ 3 mg/dl and/or ESR ≥40, obtain ECHO
- If > 3 supplementary lab findings (See Below), treat with IVIG and high dose ASA
- If < 3 supplementary Findings
- ECHO positive (see below for criteria) –> TREAT
- ECHO Negative
- If fever resolves, Kawasaki is unlikely
- If fever persists, obtain 2nd ECHO, consult specialist
Important Lab or Imaging Findings
- Supplementary Lab Findings (Need 3 or more)
- LFTs: Albumin ≤ 3 g/dL or elevated ALT (> 50 units/L)
- CBC: WBC ≥ 15,000 or PLT≥ 450,000 after 7 days, or anemia for age [normochromic, normocytic]
- UA: ≥10WBC per HPF
- ECHO findings
- Any of the Following
- z score of LAD or RCA ≥2.5
- coronary arteries meet Japanese Ministry of Health criteria for aneurysms
- ≥3 other suggestive features exist:
- perivascular brightness
- lack of tapering
- decreased LV function
- mitral regurgitation
- pericardial effusion,
- zscores in LAD or RCA of 2–2.5
From: AHA Scientific Statement on Kawasaki Disease 2004.
Faculty Reviewer: Erica Chung, MD
- “Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease.” Circulation. 2004; 110(17)2747-2771
- Son MB and Newburger JW. “Kawasaki Disease.” Pediatrics in Review. 2013; 24(4)151-161
- Newburger JW et al. “Kawasaki Disease.” Journal of the American College of Cardiology. 2016;67(14)1738-49.
A 4 year-old boy presents to the emergency room with a chief complaint of worsening limp. His parents tell you that yesterday he complained of pain in his left leg, and today he refuses to bear weight. On exam, he is febrile to 39º Celsius, tachycardic and appears ill. Exam is significant for exquisite tenderness over the left distal femur. There is no redness or swelling of the left knee. His labs are significant for WBC of 14, CRP of 150, ESR of 90. Blood cultures are drawn. X-ray of the femur reveals non-specific swelling around the femur, but no fracture. MRI shows:
Image: Case courtesy of Dr Mohammad A. ElBeialy, Radiopaedia.org. From the case rID: 38589