21: 6-year-old male with bruising

Question 1

Petechiae and purpura can be caused by:

Answer 1

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

Question 2

Differential diagnosis for bruising and leg pain: Coagulation disorder

Answer 2

• A coagulation disorder may present with petechiae or superficial bruising, but more often presents with easy bruising in deep tissues or hemarthrosis.
• Bleeding disorders (hemophilias, von Willebrand’s disease) are characterized by easy bruising in response to minor trauma. Spontaneous superficial bruising is less common.
• Hemophilias may present with painful bleeding into joints (hemarthrosis).
• Children with coagulation disorders often have a positive family history and/or personal history of bleeding (e.g., after trauma, immunizations, circumcision, dental work).

Question 3

Differential diagnosis for bruising and leg pain: Henoch-Schönlein purpura (HSP)

Answer 3

• -HSP is a self-limited, IgA-mediated, small vessel vasculitis that typically involves the skin, GI tract, joints, and kidneys.
• -Often presents in an otherwise well appearing child with bruising and leg pain (due to arthritis).
• -In roughly 50% of cases, a URI precedes the diagnosis of HSP.

Question 4

Differential diagnosis for bruising and leg pain: Idiopathic thrombocytopenic purpura (ITP)

Answer 4

• Often presents w/ asymptomatic petechiae.

- A nonspecific URI precedes ITP more than 50% of the time.

Question 5

Differential diagnosis for bruising and leg pain: Leukemia

Answer 5

• Usually presents with constitutional symptoms such as fever, malaise and weight loss.
• Bone pain is also a common presentation of leukemia in children. The pain results from infiltration of the bone marrow by malignant cell.
• Petechiae can be caused by thrombocytopenia due to bone marrow replacement by malignant cells.

Question 6

Differential diagnosis for bruising and leg pain: Viral Infection

Answer 6

• Some viruses, such as enteroviruses, may present with a petechial rash.
• Children usually have a low-grade fever.
• Other constitutional complaints may be present or absent.
• Prominent coughing and/or vomiting can also cause petechiae, generally above the nipple line.

Question 7

Differential diagnosis for bruising and leg pain: Bacterial endocarditis

Answer 7

• Typical presenting complaints are fever, fatigue, and weight loss.
• A petechial rash is commonly seen.
• Bruising is not characteristic.
• Fever is usually present, but may be low-grade.

Question 8

Differential diagnosis for bruising and leg pain: Meningococcal septiciemia

Answer 8

• -Children with meningococcal septiciemia may present with petechiae and purpura, which can be confused with bruising.
• -While the early stages of meningococcemia may have only mild symptoms, by the time the hemorrhagic rash appears, patients are usually very ill appearing and require emergent care.

Question 9

Differential diagnosis for bruising and leg pain: Rocky Mountain spotted fever (RMSF)

Answer 9

• The rash of RMSF is often petechial, and starts on the extremities before moving centrally.
• Fever is a hallmark of RMSF.

Question 10

Differential diagnosis for bruising and leg pain: SLE

Answer 10

• More common in older children and in girls.

- SLE often presents with constitutional symptoms such as fever and malaise.

Question 11

Hepatomegaly

Answer 11

Hepatomegaly may occur as a result of inflammation (e.g., viral hepatitis), infiltration (e.g., leukemia/lymphoma), accumulation of storage products (e.g., glycogen-storage disease), congestion (e.g., congestive heart failure) or obstruction (e.g., biliary atresia).

Question 12

Henoch-Schönlein Purpura (HSP)

Answer 12

Signs and Symptoms

• The hallmark of HSP is non-thrombocytopenic purpura.
• One-third of children with HSP have renal involvement, the most common manifestation of which is hematuria. Renal involvement is less common in children under two years of age, occurring in about 25% in that age group.
• Arthritis or arthralgia, mainly of the knees and ankles, is seen in about 75% of children with HSP.
• Colicky abdominal pain is present in 65% of patients. About 50% of children with HSP may develop intestinal bleeding, with guiaic positive stool..
• Two-thirds of patients report a recent upper respiratory tract infection.

Question 13

HSP Epidemiology

Answer 13

HSP is the most commonly diagnosed form of vasculitis in children (about 50% of cases)

Question 14

Instructions for Parents HSP

Answer 14

• Acetaminophen may be given for pain.
• Watch for stomach pain or vomiting.
• Reasons to seek urgent medical attention:
• –If the stomach pain is sudden or severe,
• –Blood in the stool,
• –Vomiting with inability to keep fluids down,
• –Child appears “puffy” in face, hands, or feet (a sign of renal malfunction), or
• –Any other symptoms or changes that worry the parent.

Question 15

Pathophysiology Intussusception

Answer 15

• Intussusception occurs when a proximal segment of bowel invaginates or telescopes into the distal segment adjacent. The accompanying mesentery becomes entrapped, causing vascular compression and eventual ischemia.
• Most cases of intussusception do not involve a discrete, identifiable lead point. Hypertrophied intestinal lymphoid tissue (e.g., in response to a concurrent viral infection) has been suggested as a possible trigger.
• Occasionally, a pathological lead point (e.g., polyp, Meckel’s diverticulum) starts the telescoping process. Intussusception in HSP likely begins at points of intestinal edema or submucosal hemorrhage.
• Idiopathic intussusception (in which there is no identifiable lead point) occurs in otherwise healthy infants and toddlers. The majority of cases involve the ileocecal junction.
• Intussusception in HSP is usually ileo-ileal.

Question 16

CP Intussusception

Answer 16

• -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

Question 17

Diagnosis and Treatment Intussusception

Answer 17

• -The standard approach to diagnosis and treatment of idiopathic intussusception is to perform an air or barium enema.
• -Ultrasound has increasing utility in the initial evaluation.
• -Once the intussusception is visualized, the telescoped segment of bowel is reduced by air or hydrostatic pressure. Occasionally, the procedure is unsuccessful and patients require surgical reduction.
• -Because intussusception occurring in HSP is usually ileo-ileal, not ileo-colic, it will not be reduced by air or barium enema. Diagnosis requires abdominal ultrasound, and treatment is generally surgical.

Question 18

The findings selected are possible signs of early or impending respiratory or circulatory failure and require emergent attention:

Answer 18

• AMS
• respiratory distress/respiratory depression
• mottle skin/cyanosis

Question 19

Lymph nodes should be examined for

Answer 19

size, location, distribution, texture, and mobility

Question 20

Causes of Splenomegaly

Answer 20

Infection (most frequent cause in children)
Epstein-Barr virus Cytomegalovirus Bacterial sepsis Endocarditis

Hemolysis
Sickle cell disease

Malignancy
Leukemia Lymphoma

Storage diseases
Gaucher disease

Systemic inflammatory diseases
Systemic lupus erythematosus Juvenile idiopathic arthritis

Congestion
A complication of portal hypertension

Question 21

Laboratory Evaluation of Suspected HSP

Answer 21

• platelet count
• Urinalysis
• BUN, Cr

Question 22

HSP rash

Answer 22

• The rash of HSP is somewhat unique in its tendency to involve primarily the lower extremities.
• HSP often causes a periarticular vasculitis presenting as arthritis or arthralgia.
• HSP is not associated with splenomegaly. A palpable spleen in a child with suspected HSP should prompt evaluation for other causes.

Question 23

ITP rash

Answer 23

ITP is not associated with splenomegaly. If discovered in a child with thrombocytopenia, splenomegaly should prompt evaluation for other processes, including leukemia or other malignancy.

Question 24

Leukemia rash

Answer 24

• Bone pain secondary to bone marrow expansion may cause pain localized to the joints.
• Splenomegaly and lymphadenopathy are common findings in leukemia

Question 25

Acute lymphoblastic leukemia

Answer 25

child presents with evidence of anemia (fatigue, tachycardia, pallor) and thrombocytopenia (unexplained bruising). Failure of two or more hematologic cell lines should always raise suspicion for malignant invasion of the marrow. Furthermore, the child’s chief complaint, fatigue, is the most common presenting symptom of acute leukemia. Finally, the incidence of ALL peaks at age 4 years

Question 26

HSP

Answer 26

exact etiology of HSP is unknown, but it is believed to involve an IgA-mediated immune response to infection or other triggers. The incidence is 10 cases per 100,000 children with a peak at ages 4 to 6 years (range of 2 to 17 years). HSP is characterized by a rash consisting of petechiae and palpable purpura. Other findings include a colicky diffuse or periumbilical abdominal pain, arthritis or arthralgia, and renal disease. Given the incidence of renal disease, it is important to check the urine for signs of hematuria or proteinuria; sudden changes in blood pressure can potentially suggest a change in renal function. With abnormal findings, serum BUN and creatinine must be checked.

a small vessel vasculitis. The exact mechanism of HSP is unknown; however, it is thought to be an IgA-mediated immune response affecting small vessels (skin, GI tract, joints, kidneys). Approximately 50% of cases follow viral or bacterial URIs. Biopsy of affected organs shows leukocytoclastic vasculitis with IgA deposition.

Question 27

Decreased platelets

Answer 27

often the delineating finding between HSP and ITP

Question 28

HSP tx

Answer 28

Most cases of HSP resolve within approximately one month and do not require treatment. However, symptomatic treatment for joint pain, initially with NSAIDs, may be indicated.