Pediatric Primary Care (125 page)

•  Results in intravascular thrombi and depletion of platelets and coagulation factors.

c.  Idiopathic thrombocytopenic purpura.

•  Characterized by thrombocytopenia (platelet count less than 150,000/mcL) in the absence of other causes.

•  Thought to be secondary to an autoimmune phenomenon.

d.  Henoch-Schönlein purpura.

•  Results from autoimmune reaction where the body attacks its own tissues.

•  Small, bluish purple spots on feet, legs, arms, and buttocks.

•  Usually develops after respiratory infection but can occur after immunization, insect bite, or allergic reaction to drugs or food.

•  Rate at which disease develops and its duration vary.

D.  Clinical manifestations.

1.  Excessive bruising or bleeding.

2.  Bleeding history.

a.  Location, duration, frequency, precipitating factors.

b.  What does it take to stop the bleeding?

c.  Reactions to injections, lacerations, toothbrushing, menstruation (as applicable).

d.  Medications taken that may increase tendency to bleed.

e.  Family history of unexpected or severe bleeding (surgery, childbirth, dental procedures).

f.  Consideration of abuse.

E.  Physical findings—dependent upon site of bleeding and age of the child.

1.  Epistaxis—unilateral or bilateral—bleeding longer than 15 minutes.

2.  Menorrhagia—menses longer than 7 days, double pads, lightheadedness.

3.  Ecchymosis—where, clusters or not, especially in areas that are anatomically protected.

4.  Skin and mucous membrane bleeding—generalized petechial rash, subcutaneous hemorrhagic nodules, submucosal hemorrhages of the mouth.

5.  Spontaneous hemarthrosis—joints affected, amount of swelling/pain, limited range of motion.

6.  Intracranial bleeding in absence of elicited history of trauma—headache, nausea.

7.  Hepatosplenomegaly.

8.  Hematuria.

9.  Gastrointestional cramping/pain.

F.  Diagnostic tests.

1.  Initial lab screening.

a.  CBC with differential and platelets.

b.  Prothrombin time (PT).

c.  Activated partial thromboplastin time (aPTT; see
Table 32-3
).

2.  Secondary lab screening.

a.  PT/aPTT mixing studies.

b.  von Willebrand panel.

c.  von Willebrand antigen.

d.  Factor VIII activity.

e.  Platelet function testing.

f.  Bleeding time.

g.  PFA-100.

h.  Platelet aggregation studies.

G.  Differential diagnosis.

1.  Child abuse.

2.  Leukemia.

3.  Hemophilia.

4.  von Willebrand's disease.

5.  Idiopathic thrombocytopenia purpura.

6.  Thrombocytopenia.

7.  Henoch-Schönlein purpura.

Table 32-3
Common Causes of Prolonged PT/aPTT

PT= prothrombin time

aPTT = activated partial thromboplastin time

DIC = disseminated intravascular coagulopathy

vWD = von Willebrand disease

HMWK = high-molecular-weight kininogen

PK = prekallikrein

Source:
Savage, W., & Takemoto, C. (2009). Bleeding and bruising.
Contemporary Pediatrics
, 26(6), 66.

8.  Acquired hemophilia—presence of inhibitory antibodies.

9.  Scurvy.

10.  Fabry disease.

11.  Ehlers-Danlos syndrome.

12.  Deficiency of other coagulation factors (V, VII, X, XI, or fibrinogen).

H.  Treatment.

1.  Dependent upon cause of bleeding or bruising and the site of the bleeding.

2.  Referral to hematologist if deficient factors or platelet deficiency—chronic illness that needs to have specialist involved and be enrolled in a hemophilia treatment center.

a.  Treatment is prevention of bleeding by replacement therapy.

b.  Long-term management of joint and muscle damage.

c.  Management of complications from treatment.

3.  Corticosteroids if Henoch-Schönlein purpura.

4.  Child protective services if suspected child abuse.

I.  Follow up.

1.  Well child checks and immunizations as recommended.

2.  Good communication between specialist and primary care.

3.  Action plan for family—see Education section that follows.

4.  Regular dental care.

5.  Support groups.

J.  Complications.

1.  School absence.

2.  Joint deformities.

3.  Intracranial hemorrhage.

4.  Complications from therapies like corticosteroids.

K.  Education.

1.  Importance of finding and knowing correct diagnosis.

a.  Untreated bleeding disorder leads to dangerous bleeding after childbirth, miscarriage, dental work, minor surgery, and injury.

2.  Epistaxis.

a.  Know procedure to control nosebleeds.

b.  Know when to seek medical help.

c.  Vaseline around and in the nares at bedtime.

d.  Humidifier in bedroom to provide moisture.

3.  Safety precautions with bleeding disorders to minimize long-term permanent damage to joints and muscles and the brain.

4.  Importance of keeping regular checkups with primary care provider and the hematology provider if congenital/or acquired bleeding disorder.

5.  Avoiding platelet-impairing medications such as aspirin and ibuprofen.

6.  Limit alcohol as excessive intake, which can adversely affect blood clotting.

7.  Exercise regularly.

8.  Medic alert bracelet—if hemophilia, von Willebrand, platelet dysfunction.

BIBLIOGRAPHY

Abelsohn AR, Sanborn M. Lead and children: Clinical management for family physicians.
Canadian Family Physician.
2010;56:531-535.

Berkowitz CD.
Berkowitz's pediatrics: A Primary care approach.
3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2008.

Burns CE, Brady MA, Dunn AM, et al., eds.
Pediatric primary care
, 4th ed. Philadelphia, PA: Elsevier Health Sciences; 2008.