What are key findings in the history of a child with Henoch-Schonlein Purpura?
Bruising and leg pain for greater than 24 hours, Afebrile, Recent URI, no past or family history of bleeding.
What are key findings in the physical exam of a child with Henoch-Schonlein Purpura?
Palpable purpura and petechiae distributed over buttocks and lower extremities, no adenopathy, mild tenderness with passive ankle flexion, possibility of palpable spleen tip
What is on the differential diagnosis for HSP?
ITP, Leukemia, sepsis, systemic lupus erythematosus (SLE), coagulation disorder
What are finding from testing for HSP?
Platelet count: wnl, Leukocyte and hemoglobin counts: wnl. Urinalysis: 1+ blood in urine.
Mechanisms of petechiae and purpura:
Petechiae and purpura can be caused by:
- 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)
What is the definition of Henoch-Schonlein Purpura (HSP)?
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.
What are the signs and symptoms of HSP?
- 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)
What is the epidemiology of HSP?
- 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
What is the pathophysiology of HSP?
- 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
What are the signs associated with Idiopathic thrombocytopenic purpura (ITP)?
- 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)
What is the epidemiology of ITP?
- 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
What is the pathophysiology of ITP?
- 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
What are the treatment options for ITP?
-Observation, oral corticosteroids, intravenous immunoglobulin (IVIg), and anti-D immunoglobulin (Rhogam)
What is the epidemiology of Intussusception?
- 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
What is the pathophysiology of intussusception?
- 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
What are symptoms of intussusception?
- 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.
How is intussusception managed?
- 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
Obtain the patient’s perspective:
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?
- 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)
- 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.
What are some possible causes of splenomegaly:
- 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)
Lymph node physical exam:
- 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
What is on the differential diagnosis for HSP?
ITP, Leukemia, Meningococcal septicemia, Systemic lupus erythematosus (SLE), Coagulation disorder
Henoch-Schonlein Purpura (HSP):
- 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.
Idiopathic thrombocytopenic purpura (ITP):
- 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.
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.
Infection severe enough to cause petechiae and purpura is likely to be accompanied by fever and ill appearance.
Systemic lupus erytehmatosus (SLE):
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.
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.
To confirm type of purpura (i.e. thrombocytopenic (possibly ITP or leukemia) or non-thrombocytopenia (eg HSP))
Leukocytes and hemoglobin:
Should be nml in HSP. Depression in more than one cell line warrants evaluation for possible leukemia.
Hematuria or proteinuria would identify presence of renal involvement in HSP.
Blood urea nitrogen (BUN)/Creatinine:
To evaluate the extent of renal disease, if present.
How do you best manage Henoch-Schonlein Purpura?
- 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)
How do you best manage pain with HSP?
- NSAIDs for joint pain
- -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
What are complications of HSP?
The following complications may require hospitalization:
- Severe abdominal pain
- GI bleeding
- Renal involvement