Chest Pain - Case 14-1: 17-Year-Old Boy
I. History of Present Illness
A 17-year-old boy was in good health until 3 days before his admission. At that
time, he fell playing basketball and noted some pain in his right thigh. He
also began to complain of shortness of breath and chest discomfort when lying
flat. He denied fever, rash, joint pains, and cough.
II. Past Medical History
Bilateral inguinal hernia repairs were performed in infancy, but he had had no
other hospitalizations. He was not taking any medications. A paternal uncle
required renal transplantation at 43 years of age for an unknown diagnosis. A
maternal grandmother had systemic lupus erythematosus (SLE).
III. Physical Examination
T, 37.2°C; RR, 20/min; HR, 92 bpm; BP 151/66 mm Hg; SpO2, 100% in room air
Weight, 50th percentile; height, 75th percentile
Initial examination revealed a teenage boy who was awake and alert and in no
respiratory distress. His chest examination demonstrated decreased breath
sounds at the right base. No wheezes or rales were noted. His cardiac
examination was significant for slightly diminished heart sounds but no murmurs
or rubs. His right thigh was swollen, with a circumference 6 cm greater than
the left thigh. He also had swelling of his right calf, which was 2 cm greater
in circumference than the left calf. Flexion of the right knee was limited, and
there was mild calf pain with dorsiflexion of the right foot. The remainder of
his physical examination was normal.
IV. Diagnostic Studies
Laboratory analysis revealed a peripheral blood count with 6,000 white blood
cells (WBCs)/mm
3, including 79% segmented neutrophils and 14% lymphocytes. The hemoglobin was
12.9 g/dL, and the platelet count was 156,000/mm
3. The erythrocyte sedimentation rate was elevated at 101 mm/hour. Prothrombin
and partial thromboplastin times were 13.6 and 31.9 seconds, respectively.
Urinalysis revealed large blood and 3+ protein. A Doppler ultrasound study of
the right lower extremity revealed a thrombus extending from the superficial
femoral vein to the calf vein.
V. Course of Illness
The patient was admitted and treated with intravenous heparin at 20 units/kg per
hour for his deep vein thrombosis (DVT) and with furosemide for his
hypertension. A chest roentgenogram in conjunction with further laboratory work
suggested an underlying condition that predisposed to this presentation (Fig.
14-1).
Discussion: Case 14-1
I. Differential Diagnosis
As mentioned previously, chest pain in children and adolescents is rarely
life-threatening. The majority of cases of chest pain in these age groups are
classified as idiopathic. Among adolescents, the most common nonidiopathic
causes are psychogenic origin, cough, asthma, pneumonia, and musculoskeletal
pain. Less common causes include trauma, drug use or abuse, gastroesophageal
reflux, and pneumothorax. Cardiac causes are exceedingly uncommon but should be
considered in certain clinical situations, such as a patient with syncope and
exertional or positional symptoms.
This patient had many physical and laboratory findings that warranted further
evaluation. The two most worrisome findings were his chest pain when supine and
his DVT. Shortness of breath and chest pain that worsen with supine lying
suggest possible pericardial disease. The development of DVT in an otherwise
healthy adolescent is extremely uncommon. In this situation, one should suspect
underlying hypercoagulation disorders. Finally, this DVT in conjunction with
shortness of breath and chest pain suggested pulmonary embolus as a possible
diagnosis.
II. Diagnosis
The chest roentgenogram revealed blunting of the right costophrenic angle,
suggesting a small right pleural effusion, and cardiomegaly (Fig. 14-1). An
echocardiogram demonstrated a small to moderate-sized pericardial effusion that
accounted for the finding of cardiomegaly on the chest radiograph. A
ventilation-perfusion (VQ) scan suggested a low probability of pulmonary
embolus.
As his hospitalization progressed, his hemoglobin dropped acutely to 10.3 g/dL
and Coombs positive warm antibodies were demonstrated. A 24-hour urine
collection demonstrated 8.5g protein per day. The antinuclear antibody (ANA)
titer was elevated at 1:1,280, and complement C3 and C4 concentrations were
decreased. Autoantibody studies were positive including anti-Smith, anti-RNP,
anti-SSA, anti-SSB, anti-SCL 70 and anti-JO 30. As part of his hypercoagulation
workup, he was found to have anti-cardiolipin antibodies and anti-phospholipid
antibodies.
These laboratory values along with his clinical picture suggested the underlying
diagnosis of SLE.
He was treated with prednisone for his nephritis. After a period of time, his
anticoagulation regimen was changed to low-molecular-weight heparin, and he was
discharged home on the 10th day of hospitalization.
III. Incidence and epidemiology
SLE is a multisystemic autoimmune disorder that can manifest in children and
adolescents. Determining the incidence of SLE in children is difficult with
minimal data. However, national registries in Canada and Finland have suggested
a mean annual incidence of 0.36 per 100,000 and 0.37 per 100,000 population,
respectively. Studies in the United States have suggested an annual incidence
of 0.53 to 0.60 per 100,000 population.
SLE rarely develops before the age of 5 years and most often has its onset
during adolescence. Girls are more commonly affected than boys, with a ratio of
approximately 5:1. There is a suggestion of a higher incidence in
African-Americans, followed by Hispanic children and adolescents.
IV. Clinical Presentation
SLE has a quite variable presentation, and children often have more severe
presentations than adults do. The most common presenting signs and symptoms are
fever, arthralgias or arthritis, rashes, lymphadenopathy, hepatosplenomegaly,
malaise, and weight loss. However, almost all organ systems have the potential
for involvement.
Constitutional symptoms are common at diagnosis and with disease flares.
Cutaneous findings may include the classic butterfly rash, discoid rash, or
even mucosal ulcerations. Arthralgias and arthritis, as well as aseptic
necrosis of the femoral head, may occur. Classic cardiac findings can include
pericarditis, pericardial effusions, myocarditis, and Libman-Sacks
endocarditis. Pulmonary manifestations occur in approximately 50% of patients.
Both pleural and parenchymal involvement can occur, with pleuritis and
pneumonitis most often seen. Neurologic findings include seizures, psychosis,
cerebrovascular accidents, peripheral neuropathies, and pseudotumor cerebri.
Ocular findings includes papilledema and retinopathy. From a hematologic
standpoint, patients with SLE are at a higher risk for development of the
anti-phospholipid syndrome, placing them at high risk for thromboembolic
events. Finally, renal disease is also common, with the development of
glomerulonephritis, nephrotic syndrome, and hypertension. These renal
manifestations are probably the major prognostic factor in patients with SLE.
V. Diagnostic Approach
With such a variable presentation, attempts have been made to provide criteria
for the diagnosis of SLE. The most recent revision by the American College of
Rheumatology (1997) included the following criteria for SLE:
• Malar (butterfly) rash
• Discoid-lupus rash
• Photosensitivity
• Oral or nasal mucocutaneous ulcerations
• Nonerosive arthritis
• Nephritis
• Proteinuria >0.5 g/24 hr
• Cellular casts
• Encephalopathy
• Seizures
• Psychosis
• Pleuritis or pericarditis
• Cytopenia
• Positive immunoserology
• Antibodies to dsDNA
• Antibodies to Sm nuclear antigen
• Positive finding of antiphospholipid antibodies based on:
(a) Immunoglobulin G (IgG) or IgM anti-cardiolipin antibodies, or
(b) Lupus anticoagulant, or
(c) False-positive serologic test for syphilis for at least 6 months, confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test
• Positive ANA test
In general, patients with a minimum of 4 of the 11 are diagnosed with SLE. In
childhood, these criteria have a sensitivity of 96% and a specificity of 100%
for diagnosis of SLE.
Acute phase reactants. Most acute phase reactants, including erythrocyte sedimentation rate and serum
ferritin levels, are elevated in lupus exacerbations. There is also a
hypergammaglobulinemia.
Hematologic studies. Approximately 50% of children with SLE have anemia of chronic disease. Other
findings include an acute hemolytic anemia, leukopenia, and thrombocytopenia.
As mentioned previously, a high proportion of SLE patients have a
hypercoagulable state, with the presence of anti-phospholipid antibodies.
Autoantibodies. The majority of SLE patients have detectable ANAs. The ANAs that can be seen in
SLE include anti-dsDNA, anti-DNP, anti-Ro (SS/A), anti-La (SS/B), anti-Sm and
anti-histone antibodies. Various other autoantibodies include anti-erythrocyte,
anti-lymphocytotoxic, anti-tissue-specific, and anti-phospholipid antibodies,
as well as rheumatoid factors. In terms of diagnosis, antibodies against dsDNA
are considered pathognomonic of SLE.
Complement levels. Decreased complement levels are particular indicators of active disease in SLE.
One can measure either complement components C3 and C4 or total hemolytic
complement, as measured by the ability of a test sample to hemolyze 50% of
antibody-coated erythrocytes (CH
50).
Urinalysis. The most common abnormality on urinalysis in SLE is proteinuria. Hematuria and
red blood cell casts also occur. Further tests to evaluate for lupus nephritis
include creatinine clearance, glomerular filtration rate studies, renal
ultrasonography, and biopsy.
VI. Treatment
There is no standard protocol to treat patients with SLE, because each child has
a unique presentation. The primary goal is to prevent exacerbations, rather
than to treat each flare episodically. Certain recommendations are universal,
including the need to avoid exposure to excessive sunlight.
A variety of pharmacologic agents are available to treat symptoms of SLE. NSAIDs
are typically used for the treatment of musculoskeletal complaints. Patients
with anti-cardiolipin antibodies often receive low-dose aspirin to decrease the
risk of thromboembolism. Hydroxychloroquine can be very effective in
conjunction with glucocorticoids to minimize disease exacerbations. However,
these agents may not always be effective in controlling the disease, and other
immunosuppressive agents, such as azathioprine, cyclophosphamide, and
methotrexate, may be needed.
VII. References
1. Lawrence EC. Systemic lupus erythematosus and the lung. In: Lahita RG, ed. Systemic lupus erythematosus. New York: John Wiley and Sons, 1987:691–708.
2. Petty RE, Cassidy JT. Systemic lupus erythematosus. In: Cassidy JT, Petty
RE, eds.
Textbook of pediatric rheumatology, 4th ed. Philadelphia: WB Saunders, 2001:396–438.
3. Tucker LB. Caring for the adolescent with systemic lupus erythematosus. Adolesc Med 1998;9:59–67.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
Other Book Chapters Related to Chest pain
Read excerpts from these other book chapters related to Chest pain:
Medical Books Excerpts
- FLANK PAIN
- "Algorithmic Diagnosis of Symptoms and Signs" (2003)
- [ read ]
- CHEST PAIN
- "Algorithmic Diagnosis of Symptoms and Signs" (2003)
- [ read ]
- Flank pain
- "Handbook of Signs & Symptoms (Third Edition)" (2006)
- [ read ]
- Chest pain
- "Handbook of Signs & Symptoms (Third Edition)" (2006)
- [ read ]
- Chest Pain
- "A Pocket Manual of Differential Diagnosis" (1999)
- [ read ]
- Pneumonia
- "Professional Guide to Diseases (Eighth Edition)" (2005)
- [ read ]
- Flank pain
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
- [ read ]
- Chest pain
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
- [ read ]
- Flank pain
- "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
- [ read ]
- Chest pain
- "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
- [ read ]
- Flank pain
- "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
- [ read ]
- Chest pain
- "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
- [ read ]
- Chest Pain
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
- [ read ]
- Chest Pain
- "Pediatric Complaints and Diagnostic Dilemmas" (2003)
- [ read ]
Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
More About Causes of Chest pain
» Next page: Chest Pain - Case 14-2: 15-Year-Old Boy (Pediatric Complaints and Diagnostic Dilemmas)
Rate This Website
What do you think about the features of this website?
Take our user survey and have your say:
Website User Survey
Medical Tools & Articles:
Next articles:
Tools & Services:
Medical Articles:
Forums & Message Boards
- Ask or answer a question at the Boards:
