CLIPP 11

Percent of KD patients who experience hydrops of the gallbladder

10%

Percent of KD patients who experience arthritis

30%

Can you use antibiotics in treatment of KD?

Antibiotics have not been shown to have any effect on the clinical course.

What viruses cause enteroviral infection?

Coxsackievirus, echovirus, enterovirus

Rash that is red and flat

Erythematous macular rash

Scarlet fever is caused by what infection?

Group A Strep

Roseola is caused by what virus?

HHV-6

Flat, discolored spot

Macule

- Children with these infections usually appear well with minimal if any constitutional signs and symptoms. - The overlying skin may be erythematous initially, but left untreated often becomes violaceous as the nodes enlarge. - Nodes may rupture through the skin, resulting in a draining sinus tract. - Treatment is surgical excision, as incision and drainage can also result in a sinus tract.

Mycobacterial infection

Typical rash of roseola/erythema infectiosum

Slapped cheek

Can you use steroids in the treatment of KD?

The role of steroids is unclear. For years, steroids were felt to have no role, or even to be detrimental. Newer studies suggest that they may have a role in the acute management, and additional information is needed. Other immunomodulators targeting more specific arms of the inflammatory cascade are being studied (see references below for more information); no treatment has proven superior to IVIG and aspirin, which thus remains the standard of care.

___________ is the most common cause of mycobacterial lymphadenitis in children over 12 years of age.

Tuberculosis

All patients with KD should receive a baseline echocardiogram during the _____ phase, both to look for the presence of aneurysms and to provide a baseline for future comparison.

acute

If coronary artery aneurysms develop, they usually do so during the ______ phase of illness and almost always by four weeks of onset of the acute illness. A followup visit at one to two weeks is recommended.

subacute

The greatest risk for the development of CA aneurysms in KD is in the ________, and aneurysms almost always develop within four weeks of the onset of the disease. Aneurysms that form during this time are at risk for thrombosis due to the concurrent thrombocytosis.

subacute phase of illness (beyond 10 days)

Non-Infectious Causes of Generalized Lymphadenopathy

- Lymphomas - Leukemia - Histiocytosis - Metastatic neuroblastoma - Rhabdomyosarcoma

There are three distinct phases of illness in KD:

1. Acute phase: onset through ~10 days. Fever and clinical findings are present, with serologic evidence of systemic inflammation (elevated acute phase reactants). 2. Subacute phase: 10 days through ~3 weeks. Fever resolves and clinical findings largely subside (often with peeling of hands and feet). Serologic evidence of inflammation continues. 3. Convalescent phase: 3 weeks through 6-8 weeks. All clinical findings have resolved. Continued serologic evidence of inflammation. Beyond 6-8 weeks, serologic evidence for inflammation has resolved. KD causes a panvasculitis, impacting any blood vessels, although there is a predilection for small and medium-sized vessels (especially the coronary arteries for unclear reasons). This vasculitis can lead to aneurysmal dilation, particularly during the subacute phase of illness.

In addition to high fever for at least five days, four of the following five criteria are needed for a diagnosis of Kawasaki disease:

1. Changes in oral mucosa (i.e.: "strawberry tongue") 2. Extremity changes (redness/swelling) 3. Unilateral cervical lymphadenopathy 4. Rash 5, Conjunctivitis (bilateral, nonpurulent) The one least likely to be present is cervical adenopathy. The final criterion is that there is *no other apparent cause for the presentation* (i.e., a child presenting with an obvious site of infection, even if meeting all criteria for Kawasaki, cannot be given the diagnosis). Though irritability is very common, it is not part of the diagnostic criteria.

Percent of KD patients who experience coronary artery aneurism:

20-25%

The use of IVIG in Kawasaki disease has decreased the incidence of coronary artery aneurysms from ____ to ____.

20-25% to 2-4%

Percent of KD patients who experience liver dysfunction (as evidenced by elevated transaminases and a decreased albumin)

40%

Percent of KD patients who experience aseptic meningitis or other CNS manifestations:

90% of patients develop neurologic manifestations, which can range from irritability to aseptic meningitis

UA findings typically found in Kawasaki

A sterile pyuria, secondary to a sterile urethritis, is associated with Kawasaki disease. A clean-catch urine is likely to show white cells, whereas a catheterized urine may not (because the white cells come from the urethra).

Low grade fever followed by a rash, which starts as a facial erythema to the face ("slapped cheek" appearance), which can spread to the trunk and extremities and appears lacy Can lead to pain and swelling of the extremities, as well as development of aplastic anemia

Also called 5th disease Viral infection (parvovirus B19) Erythema Infectiosum

KD "outbreaks" follow seasonal patterns, and children of _______ descent have a higher incidence of KD (suggesting both infectious and genetic influences).

Asian

Does aspirin administration affect the likelyhood of aneurisms in KD?

Aspirin shortens the febrile course of the illness but has no effect on the development of aneurysms.

White spots on the buccal mucosa are also known as _________, which are pathognomonic for measles. The fever and conjunctivitis could be measles, but there is no cough or coryza. In addition, the rash typically starts at the head and moves downward, rather than starting on the hands and feet.

Koplik spots

LFT findings typically found in Kawasaki

Liver enzymes (transaminases) can be elevated in a number of conditions on the differential, including both Kawasaki disease and Stevens-Johnson syndrome. Serum albumin level is frequently low in Kawasaki disease.

__________ is important to keep in mind for any patient with fever and rash. The time course is typically rapid, so it would be less likely in a child with multiple days of fever and rash.

Meningococcemia

Can you use ibuprofen in treatment of KD?

Other antipyretics than aspirin are usually not effective for fever control. Furthermore, use of ibuprofen has been shown to antagonize the irreversible platelet inhibition induced by aspirin and therefore should be avoided in children with coronary aneurysms taking aspirin for its antiplatelet effects.

Small, well defined solid palpable bump

Papule

Rash in RMSF?

Petechial rash

Small, raised, differentiated patch or area on body surface

Plaque

Smell, well defined bump containing purulent material

Pustule

Children taking aspirin are at risk for _______ (a potentially fatal illness that causes multi-organ damage) if infected with certain viruses, including influenza and varicella. Parents should avoid exposure to anyone with known flu or chicken pox, and children should receive the intramuscular flu vaccine.

Reye syndrome

Rocky Mountain Spotted Fever (RMSF) is a tick-borne disease caused by _______. This tick is commonly found in southeastern parts of the U.S., and patients will often come from or have a history of travel to that region. The disease is characterized by headache, fever, myalgia, and a centrally progressing petechial rash originating on the wrists and ankles.

Rickettsia rickettsii

Headache would be present in ___________. This seems reasonable, given the fever and rash that began on the palms and soles. However, the other findings suggest Kawasaki disease, so this is not the best answer.

Rocky Mountain Spotted Fever

- "Exanthem subitum:" erythematous macules start on trunk and spreads to arms and neck (less commonly face and legs) - Rash is typically preceded by 3-4 days of high fevers, which end as the rash appears - Usually occurs in children under age 2 years

Roseola HHV-6

What is desquamation?

Shedding of the outer layer of skin surface

- Severe, pruritic rash (erythema multiforme) - Fever - Mucosal changes (e.g., stomatitis) - Conjunctivitis

Stevens-Johnson syndrome

A mucocutaneous disorder caused by a hypersensitivity reaction to medications, infections, or other illnesses

Stevens-Johnson syndrome

Scarlet fever is caused by erythrogenic toxin produced by ___________. Symptoms can include sore throat, fever, "strawberry tongue" and a blanching, erythematous rash with desquamation of the affected areas about six to seven days later as the rash begins to disappear.

Streptococcus pyogenes

Which of these vitals are abnormal for a 4 year old? Temperature: 39.7 C (103.5 F) oral Heart rate: 150 beats/minute Respiratory rate: 24 breaths/minute Blood pressure: 95/60 mm Hg Weight: 19 kg Height: 108 cm

T: high, over 38 HR: 65 to 135 is normal

Lymphadenopathy with Kawasaki disease is most often *(bilateral/unilateral)*, with lymph nodes often larger than ____ cm in diameter, although less commonly diffuse lymphadenopathy can occur. Enlarged lymph nodes are nonfluctuant.

Unilateral, larger than 1.5cm

Small, well defined fluid containing bump

Vesicle

Is measles viral or bacterial?

Viral

Gold standard treatment for KD?

aspirin and IVIG

Kawasaki disease (KD) is an acute inflammatory panvasculitis of unknown etiology. It is thought that the disease results from an...

autoimmune response to a not yet identified infectious trigger

Of these potential complications associated with KD, the risk with the greatest morbidity is the vasculitis that can lead to the development of _______. The main purpose of the treatment of Kawasaki disease is to minimize this risk.

coronary aneurysms

Tonsillar exudates would be present in ______ or ______. Given the prolonged fever, rash, lymph node involvement, and conjunctivitis, the disease process is more widespread than a simple tonsillitis. You should recognize this constellation of symptoms as Kawasaki disease.

strep pharyngitis or tonsillitis

Typical presentation of adenovirus infection

- May cause upper respiratory tract infection, pharyngitis, conjunctivitis, tonsillitis, or otitis media - Potential for more severe infections in immunocompromised hosts

Infectious Causes of Generalized Lymphadenopathy

- Measles (presents with a marked generalized lymphadenopathy and splenomegaly that may last several weeks) - Infectious mononucleosis-caused by either - - Epstein-Barr virus or cytomegalovirus - Human immunodeficiency virus (HIV) - Histoplasmosis - Toxoplasmosis - Mycobacteria (can also present as localized lymphadenopathy)

Systemic onset juvenile idiopathic arthritis, also known as Still's disease, is a subset of JIA describing patients with intermittent rash, fever and arthritis. While our patient does present with rash and fever, as well as refusal to walk (potentially a sign of arthritis), systemic onset JIA tends to present with a history of spiking fevers and __________, and disappearing as the fever fades.

"salmon" rash occurring when the child is febrile

CBC findings typically found in Kawasaki

*WBC*: The white blood count is usually elevated, with a predominance of neutrophils. *Hgb/Hct*: A normochromic, normocytic anemia is common. *Platelets*: Thrombocytosis is a common feature of Kawasaki disease - usually starting in the second week of the illness.

possible causes of unilateral cervical lymphadenopathy

- Bacterial cervical adenitis - cat scratch disease - reactive node from an oral inflammatory or infectious process - Kawasaki disease - mycobacterial infection

Acute phase reactants findings typically found in Kawasaki

- Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) lack specificity, which limits their utility for diagnostic purposes, although they can lend support for or against some diagnoses. They frequently play a more useful role in following a disease process. - In this case, a negative ESR would argue strongly against Kawasaki disease. The persistence of an elevated ESR after the fever has subsided can help to distinguish Kawasaki disease from other infectious rash/fever illnesses.

KD is a disease of childhood, with the typical age of patients _____ months old; 80% of KD patients are under the age of 5 years.

15-18

In a patient with KD, if you do an ECHO in the acute phase and find normal coronary arteries, you do a repeat ECHO in ___ AND ___ weeks.

2 AND 6 weeks

- Typically seen in children ages one to five years with a history of a recent upper respiratory tract infection. - Staphylococcus aureus and Streptococcus pyogenes are the organisms most commonly identified. - Patients may have high fevers and a toxic appearance. - Overlying cellulitis and development of fluctuance are common.

Bacterial cervical adenitis

- Fever - Chills, malaise - Rash (often petechial) - May lead to shock and DIC (often rapidly progressing)

Bacterial meningoccemia

Based on your understanding of the pathophysiology of Kawasaki disease, which studies should be performed to screen for complications related to the disease? Choose the single best answer. A. Liver ultrasound B. Lumbar puncture C. Radiographs of hands and feet D. Renal ultrasound E. Echocardiogram

A. Liver ultrasound B. Lumbar puncture C. Radiographs of hands and feet D. Renal ultrasound *E. Echocardiogram*

Based on your understanding of the pathophysiology and complications of Kawasaki disease, which of the following therapies would be appropriate in the acute management of Jason's illness? Select all that apply. A. Acetaminophen B. Aspirin C. Ceftriaxone D. Ibuprofen E. Intravenous immune globulin (IVIG)

A. Acetaminophen B. *Aspirin* C. Ceftriaxone D. Ibuprofen E. *Intravenous immune globulin (IVIG)*

An erythematous tongue with prominent papillae has the marbled appearance of the flesh of a strawberry, and is thus often referred to as a "strawberry tongue." Which of the following conditions have been associated with a finding of a "strawberry tongue?" Select all that apply. A. Adenovirus B. Enterovirus C. Group A streptococcal pharyngitis D. Infectious mononucleosis E. Kawasaki disease F. Toxic shock syndrome

A. Adenovirus B. Enterovirus C. *Group A streptococcal pharyngitis* D. Infectious mononucleosis E. *Kawasaki disease* F. *Toxic shock syndrome* Though this finding can be seen in infectious mononucleosis, it is usually in the presence of a concomitant streptococcal pharyngitis.

A 5-year-old female, previously healthy, presents with an erythematous, vesicular rash on the palms and soles and a high fever for several days. Upon examination, she is also found to have ulcers in her mouth. A few days later, the fever and rash resolve. What is the most likely pathogen? A. Herpes simplex virus 1 (HSV-1) B. HIV C. Enterovirus D. Human herpesvirus 6 (HHV-6) E. Group A strep

A. Herpes simplex virus 1 (HSV-1) B. HIV C. *Enterovirus* D. Human herpesvirus 6 (HHV-6) E. Group A strep This presentation is consistent with infection by cocksackie A, an enterovirus. Following an incubation period of three to five days, patients have fever, tender vesicles on their hands and feet, and oral ulcers. Sometimes the rash also occurs on the buttocks and the genitals. The infection resolves spontaneously within three days, and is spread from person to person via saliva, fluid from the vesicles, stool, or nasal discharge.

A 3-year old girl comes to the clinic with a chief complaint of fever (104F) for over a week. Her mom reports that she has been fussy and inconsolable since she became febrile. She has a red tongue, with large papillae, conjunctivitis, a palmar rash, unilateral cervical adenopathy, as well as swollen feet. Given the most likely diagnosis, what is the most important follow-up for this patient over the next few weeks? A. Ophthalmology follow-up to determine extent of eye damage and determine need for corticosteroids B. Physical therapy follow-up to help prevent long-term joint deformities and ensure long-term functionality C. Cardiology follow-up to rule out presence of rheumatic fever D. Echocardiogram to look for coronary artery aneurysm E. Neurology follow-up to evaluate partial paralysis of lower extremities

A. Ophthalmology follow-up to determine extent of eye damage and determine need for corticosteroids B. Physical therapy follow-up to help prevent long-term joint deformities and ensure long-term functionality C. Cardiology follow-up to rule out presence of rheumatic fever *D. Echocardiogram to look for coronary artery aneurysm* E. Neurology follow-up to evaluate partial paralysis of lower extremities D. Choice D is correct because children with Kawasaki disease are at high risk for coronary artery aneurysm formation and should receive an echocardiogram within four weeks of the onset of their illness. Use of IVIG for the treatment of Kawasaki disease has decreased the risk of coronary artery aneurysms significantly. Kawasaki disease is diagnosed when there is a fever plus four of the following: changes in oral mucosa (e.g., strawberry tongue), extremity swelling or redness, unilateral cervical adenopathy, conjunctivitis, and rash. Infectious and rheumatologic causes must be excluded in order to make the diagnosis of Kawasaki disease.

A 3-year-old male presents with fever to 103 F for the past week, injected eyes, and a refusal to walk for the past two days. On physical exam, you note conjunctival injection without pus or exudates bilaterally, prominent papillae of his tongue with redness as well as redness of his hands, and feet. He also has a new non-diffuse maculopapular rash on his torso that gets worse with fever. On examination of the swollen extremities, you are unable to elicit any tenderness or effusions in any joints. Which of the following is the most likely diagnosis? A. Rocky Mountain Spotted Fever (RMSF) B. Bone or joint infection C. Kawasaki disease (KD) D. Scarlet fever E. Systemic onset juvenile idiopathic arthritis

A. Rocky Mountain Spotted Fever (RMSF) B. Bone or joint infection *C. Kawasaki disease (KD)* D. Scarlet fever E. Systemic onset juvenile idiopathic arthritis Kawasaki disease (KD) is one of the most common vasculitides of childhood. For diagnosis, in addition to fever of > 5 days, patient must meet four of the following criteria: rash, conjunctivitis, unilateral cervical lymphadenopathy, changes in oral mucosa, or extremity changes (redness/swelling). Our patient does not have lymphadenopathy, but often this is the least common finding in KD. If children have fever with fewer than four of the five clinical findings, they can have "incomplete KD" if they meet certain laboratory criteria.

A 5-year-old male comes to the clinic with a chief complaint of four days of progressively worsening fever and that has been minimally responsive to acetaminophen. The patient complains of sore throat and decreased appetite. His sister had a positive rapid strep test and is now being treated with amoxicillin. Your concern is for Group A strep. What is the next best step in management? A. Start antibiotic treatment B. Send blood cultures C. Advise parents to give patient acetaminophen with return precautions D. Rapid strep test with back-up culture if negative E. Chest x-ray

A. Start antibiotic treatment B. Send blood cultures C. Advise parents to give patient acetaminophen with return precautions *D. Rapid strep test with back-up culture if negative* E. Chest x-ray A. Choice A is incorrect. Although you may empirically treat this child for infection with Group A strep, a test to diagnose infection should be done prior to initiation of antibiotics. B. Choice B is incorrect. There is no indication for a blood culture at this time. C. Choice C is incorrect. As the patient has a history of being exposed to a sick contact with Group A strep, being sent home with acetaminophen is also not sufficient because the patient has already been treated at home with acetaminophen with no improvement. D. Choice D would provide confirmation of your clinical suspicion and allow for correct diagnosis prior to empiric antibiotic treatment. E. Choice E is incorrect because there is no indication of respiratory symptoms.

A 2-year-old girl presents to the urgent care clinic with a 7-day history of high fever to 38.5 C, a maculopapular rash that began on the palms and soles of her feet, red eyes without discharge, and unilateral cervical adenopathy. What other symptom/sign might you discover on further history and exam? A. Tonsillar exudates B. Headache C. Erythematous and edematous feet D. White spots on buccal mucosa E. Dysuria

A. Tonsillar exudates B. Headache C. *Erythematous and edematous feet* D. White spots on buccal mucosa E. Dysuria C. The constellation of symptoms described suggests Kawasaki disease. The other two classic signs not mentioned are erythematous tongue ("strawberry tongue"), and erythema/edema of the extremities, which is the best answer here.

Four year old w/ 4 days of fever and irritability and rash and sore throat. Little sister has Group A Strep. A. Given an intramuscular injection of penicillin. B. Started oral amoxicillin and discontinued it if the throat culture is negative. C. Started oral penicillin and discontinued it if the throat culture is negative. D. Waited for result of the throat culture and started oral amoxicillin if positive for Group A Strep. E. Waited for result of the throat culture and given an injection of penicillin if positive for Group A Strep.

ANY would be acceptable

Reactive cervical adenitis occurs in response to _____ or ____.

An oral infectious or inflammatory process

- Infections caused by the bacterium Bartonella henselae can be asymptomatic or symptomatic. - The infection usually is introduced by a scratch from a cat or kitten, with subsequent infection of the node or nodes draining that site. - The site most commonly involved is the axilla, followed by cervical, submandibular, and inguinal areas. - Usually a self-limited disease, with regression of the lymph node in four to six weeks.

Cat scratch disease

HIV infection can increase the risk of oral lesions secondary to infections by _____ or _______, but again would be unlikely to cause lesions on the hands and feet. Furthermore, at this patient's age (5 years), she is unlikely to be HIV-positive unless it was vertically transmitted from her mother.

HSV-1 or Candida albicans

A single dose of IVIG at a dose of 2 g/kg administered over ten to twelve hours has been shown to be more effective in reducing the risk of coronary artery aneurysms than ______.

multiple lower doses

How much aspirin do you give in treatment of KD?

High-dose aspirin (80-100 mg/kg/day, divided into four doses) is administered for its anti-inflammatory properties. Following defervescence, low-dose aspirin (3-5 mg/kg/day given in a single dose) is administered for its anti-platelet effects. Aspirin is discontinued altogether after a total of six to eight weeks if no coronary artery changes are seen in followup echocardiograms. If there are coronary artery abnormalities, low dose aspirin is continued indefinitely as an anti-platelet agent.

Mycobacterial infections can cause diffuse lymphadenopathy or isolated lymphadenitis. Lymphadenitis is the most common manifestation of ________ in children, with a peak age of presentation of 2 to 4 years.

nontuberculous mycobacteria

- After a prodrome of fever (over 38.3 C, or 101 F), cough, coryza, and conjunctivitis, this maculopapular rash starts on the neck, behind the ears, and along the hairline. - The rash spreads downward, reaching the feet in two or three days. - The initial rash appears on the buccal mucosa as red lesions with bluish white spots in the center (known as Koplik spots). These have frequently disappeared by the time the patient presents to medical attention. Immunization is very effective in preventing this infection.

Measles

- Fever - Headache - Rash (typically starts on ankles and wrists and progresses centrally and to palms and soles; may be macular or papular at first, quickly becoming petechial; in 5% of cases, there may be no rash) - Myalgias

RMSF

Tick-borne bacterial infection (Rickettsia rickettsii) common in NC

RMSF

- Fever - A diffuse, erythematous, finely papular rash (described as having a "sandpaper" texture) is pathognomonic - Rash often begins at neck, axillae, and groin and then spreads over trunk and extremities, typically resolving within four or five days

Scarlet fever

What type of rash is typical in Varicella?

Vesicular rash at various stages of development

- Rash starts on trunk and spreads to extremities and head - Each lesion starts as an erythematous macule, then forms a papule followed by a vesicle before crusting over - Lesions at various stages of development are seen in the same area of the body - Immunization is effective in preventing this infection

Viral infection (varicella-zoster virus)

- Fever lasting 3-5 days - Nonspecific rash (which may include the palms and soles) - May also cause conjunctivitis, oral ulcers, diarrhea, aseptic meningitis

enteroviral infection Coxsackievirus, echovirus, enterovirus

HSV-1 causes _______ and can sometimes be accompanied by fever and malaise, but lesions on the hands and feet would be uncommon.

gingivostomatitis

Erythema Infectiosum is caused by what virus?

parvovirus B19

Although children with Kawasaki disease can have ______, it is not associated with dysuria, a symptom of a UTI, which would be highly unlikely given the other signs.

pyuria

Once the febrile phase of Kawasaki disease resolves, children are typically prescribed a low dose of aspirin to be taken for ______ weeks. Since aspirin can cause gastrointestinal bleeding and other problems, parents should watch for warning signs, such as a stomachache or blood in the stool.

six to eight