Case 21

Question 1

What are key findings in the history of a child with Henoch-Schonlein Purpura?

Answer 1

Bruising and leg pain for greater than 24 hours, Afebrile, Recent URI, no past or family history of bleeding.

Question 2

What are key findings in the physical exam of a child with Henoch-Schonlein Purpura?

Answer 2

Palpable purpura and petechiae distributed over buttocks and lower extremities, no adenopathy, mild tenderness with passive ankle flexion, possibility of palpable spleen tip

Question 3

What is on the differential diagnosis for HSP?

Answer 3

ITP, Leukemia, sepsis, systemic lupus erythematosus (SLE), coagulation disorder

Question 4

What are finding from testing for HSP?

Answer 4

Platelet count: wnl, Leukocyte and hemoglobin counts: wnl. Urinalysis: 1+ blood in urine.

Question 5

Mechanisms of petechiae and purpura:

Answer 5

Petechiae and purpura can be caused by:

• Trauma
• Plt deficiency or dysfunction (eg, immune-mediated thrombocytopenia, bone marrow infiltration or suppression, malignancy)
• Coagulation abnormalities (eg, hereditary or acquired clotting-factor deficiencies)
• Vascular fragility (eg, immune-mediated vasculitis)
• Combinations of the above (eg infection causing coagulation abnormalities, vascular fragility, platelet consumption)

Question 6

What is the definition of Henoch-Schonlein Purpura (HSP)?

Answer 6

Also known as anaphylactoid purpura, HSP is self-limited (may last a month or so), IgA-mediated, small vessel vasculitis that typically involves the skin, gastrointestinal tract, joints and kidneys.

Question 7

What are the signs and symptoms of HSP?

Answer 7

• Hallmark is non-thrombocytopenic petechiae and purpura
• Hematuria (renal involvement in about a third of cases)
• Arthritis, mainly of knees and ankles (in up to 75 percent of cases)
• Colicky abdominal pain (65 percent of cases)

Question 8

What is the epidemiology of HSP?

Answer 8

• Most common form of vasculitis in children
• Approximately 10 cases per 100,000 children annually
• Peak incidence at 4-6 years (range 2-17 years)
• Boys affected twice as often as girls

Question 9

What is the pathophysiology of HSP?

Answer 9

• Underlying mechanism unknown: likely an IgA-dominated immune response to infection or other triggers. About 2/3 of patients report a recent URI.
• Biopsy of affected organs reveals leukocytoclastic vasculitis and deposition of IgA
• About 5 percent of children progress to chronic renal failure; less than 1 percent will have end-stage renal disease

Question 10

What are the signs associated with Idiopathic thrombocytopenic purpura (ITP)?

Answer 10

• Usually asymptomatic petechiae and bruising
• In about 3 percent of cases, severe epistaxis or other mucous membrane hemorrhage may be seen; intracranial hemorrhage, while concerning, is rare (0.1-0.5 percent of cases)

Question 11

What is the epidemiology of ITP?

Answer 11

• Most common cause of isolated thrombocytopenia in otherwise healthy children
• 5 cases per 100,000 children annually
• Peak incidence at 2-5 years (range 2-10 years)
• Boys and girls affected equally

Question 12

What is the pathophysiology of ITP?

Answer 12

• Anti-platelet antibody binds to platelet surface, leading to removal and destruction of platelets by spleen and liver
• Often (about 50 percent of the time) preceded by a nonspecific viral infection

Question 13

What are the treatment options for ITP?

Answer 13

-Observation, oral corticosteroids, intravenous immunoglobulin (IVIg), and anti-D immunoglobulin (Rhogam)

Question 14

What is the epidemiology of Intussusception?

Answer 14

• Most common form of bowel obstruction in children between 6 mo and 6 yr of age
• 80 percent of cases occur in children under 2 years
• Boys affected more than girls

Question 15

What is the pathophysiology of intussusception?

Answer 15

• Occurs when a proximal segment of bowel invaginate into the adjacent distal segment causing entrapment of mesentery and vascular compression and ischemia
• Hypertrophied lymphoid tissue due to viral inflammation may be trigger of idiopathic intussusception.
• A pathological lead point (eg polyp, meckel’s diverticulum, or intestinal edema) is also occasionally seen
• Idiopathic intussusception typically involves the ileocecal junction, while intussusception as a complication of HSP is typically ileoileal

Question 16

What are symptoms of intussusception?

Answer 16

• Classic triad of presenting findings (although occurs in a minority of patients, so high level of clinical suspicion is required for diagnosis):
• -Paroxysms of severe abdominal pain with inconsolable crying
• -Passage of “currant jelly” stool containing blood and mucus
• -Palpation of a “sausage-shaped” mass in the right abdomen
• Additional signs and symptoms: Vomiting (bilious or non-bilious), lethargy, and a toxic appearance, with or without signs of pain.

Question 17

How is intussusception managed?

Answer 17

• Dx and tx of non-HSP related intussusception are accomplished with air or contrast enema. Occasionally surgical reduction is needed in these cases.
• Dx of HSP-related intussusception is by abdominal US, and tx generally is surgical

Question 18

Obtain the patient’s perspective:

Answer 18

Ask questions like:

• What do you think is going on?
• Why do you think this is happening?
• What worries you about what is happening?
• What do you think might happen next?

Question 19

Liver Exam:

Answer 19

• Place child supine, knees flexed (relaxes abdominal muscles); helpful to start in right lower quadrant and move superiorly
• Examine by percussing the upper edge and palpating the lower edge in the midclavicular line
• In normal newborns, liver edge may be palpable up to 3.5 cm below the costal margin; in normal older children, up to 2 cm below the costal margin.
• Causes of hepatomegaly:
• -Inflammation (eg viral hepatitis)
• -Infiltration (eg leukemia/lymphoma)
• -Accumulation of storage products (eg glycogen storage disease)
• -Congestion (eg congestive heart failure)
• -Obstruction (eg biliary atresia)

Question 20

Spleen exam:

Answer 20

• Place child supine with knees flexed. Lift left flank or have patient roll onto right side to help move spleen forward into a palpable position.
• Spleen tip is palpable in up to a third of neonates, 10 percent of normal children, and 2 percent of healthy adolescents
• A spleen edge palpable more than 2 cm below the left costal margin is abnormal.

Question 21

What are some possible causes of splenomegaly:

Answer 21

• Infection (EBV, CMV, bacterial sepsis, endocarditis)
• Hemolysis (sickle cell disease)
• Malignancy (leukemia, lymphoma)
• Storage diseases (Gaucher disease)
• Systemic inflammatory diseases (systemic lupus erythematous, juvenile rheumatoid arthritis)
• Congestion (related to portal HTN)

Question 22

Lymph node physical exam:

Answer 22

• Examine for size, location, distribution, texture and mobility.
• Features of abnormal nodes:
• -Larger than 2 cm
• -Palpable in areas other than the cervical, axillary, and inguinal regions
• -Tender, warm, fluctuant, erythematous (suggestive of local infection or lymphadenitis)
• -Hard, rubbery, matted together, fixed/immobile (concerning for malignancy)
• -Supraclavicular nodes are highly concerning for lymphoma
• -Diffuse adenopathy occurs with generalized infection, malignancy, storage diseases, and chronic inflammatory diseases

Question 23

What is on the differential diagnosis for HSP?

Answer 23

ITP, Leukemia, Meningococcal septicemia, Systemic lupus erythematosus (SLE), Coagulation disorder

Question 24

Henoch-Schonlein Purpura (HSP):

Answer 24

• Skin lesions may being as erythematous macule or urticarial wheals that evolve to petechiae and palpable purpura. Rash is symmetrically distributed in gravity-dependent or pressure-sensitive areas (eg, lower extremities, elbows)
• All children eventually develop skin findings. The presence and timing of the following findings varies:
• -Colicky, diffuse or periumbilical abdominal pain
• -Arthritis or arthralgias
• -Renal disease
• Laboratory findings often normal and non-specific
• A palpable spleen in a child with suspected HSP should prompt evaluation for other causes.

Question 25

Idiopathic thrombocytopenic purpura (ITP):

Answer 25

- Children with ITP usually present with asymptomatic petechiae and bruising. Some have evidence of mucosal bleeding. Other symptoms/findings are generally absent. - Lab findings in ITP include thrombocytopenia (usually with platelet counts less than 20,000) with a nil leukocyte count and Hgb. Abnormalities in either leukocyte or red cell counts require additional evaluation. - Presence of splenomegaly should prompt evaluation for other processes including leukemia or other malignancy, ion a child with thrombocytopenia.

Question 26

Leukemia:

Answer 26

Clinical manifestations are related to bone marrow infiltration and cytopenias. Presenting sings and symptoms often include: Petechiae and purpura, bone pain, fever, fatigue, malaise. Hepatosplenomegaly and lymphadenopathy are common findings.

Question 27

Meningococcal septicemia:

Answer 27

Infection severe enough to cause petechiae and purpura is likely to be accompanied by fever and ill appearance.

Question 28

Systemic lupus erytehmatosus (SLE):

Answer 28

SLE often presents with fever and malaise. SLE can cause both vasculitis and thrombocytopenia, leading to petechiae and purpura involving the lower extremities. (though they do occur, these are relatively uncommon as presenting symptoms) Joint complaints are common in SLE. Splenomegaly can also occur.

Question 29

Coagulation disorder:

Answer 29

Disorders of bleeding, such as a hemophilia or Von Willebrand's disease, can cause superficial bleeding, but usually only in response to trauma. Hemophilias can cause painful bleeding into joints. There is often a past and/or family history positive for easy bleeding/bruising.

Question 30

Platelet count:

Answer 30

To confirm type of purpura (i.e. thrombocytopenic (possibly ITP or leukemia) or non-thrombocytopenia (eg HSP))

Question 31

Leukocytes and hemoglobin:

Answer 31

Should be nml in HSP. Depression in more than one cell line warrants evaluation for possible leukemia.

Question 32

Urinalysis:

Answer 32

Hematuria or proteinuria would identify presence of renal involvement in HSP.

Question 33

Blood urea nitrogen (BUN)/Creatinine:

Answer 33

To evaluate the extent of renal disease, if present.

Question 34

How do you best manage Henoch-Schonlein Purpura?

Answer 34

- Usually resolves without treatment in 4-6 weeks - Monitor for development of renal involvement (usually weekly until rash disappears, then monthly for several months) - Recurrence rate is approx. 30 percent (may be months to years later and may start with abdominal pain rather than rash)

Question 35

How do you best manage pain with HSP?

Answer 35

- NSAIDs for joint pain - Corticosteroids - -Tx of HSP with steroids is controversial, but they may reduce complications from GI manifestations; no certain benefit has been shown in reducing renal complications

Question 36

What are complications of HSP?

Answer 36

The following complications may require hospitalization: - Severe abdominal pain - Intussusception - GI bleeding - Renal involvement