DOI:10.2214/AJR.07.3482
AJR 2008; 191:1570-1575
© American Roentgen Ray Society
Chest Radiographic and CT Manifestations of Chronic Granulomatous Disease in Adults
Myrna C. B. Godoy1,2,
Patrick M. Vos1,
Peter L. Cooperberg1,
Carmen P. Lydell1,
Peter Phillips3 and
Nestor L. Müller4
1 Department of Radiology, St. Paul's Hospital, University of British Columbia,
Vancouver, BC, Canada.
2 Present address: Department of Radiology, New York University School of
Medicine, 560 First Ave., IRM 236, New York, NY 10016.
3 Division of Infectious Diseases, St. Paul's Hospital, University of British
Columbia, Vancouver, BC, Canada.
4 Department of Radiology, Vancouver General Hospital, University of British
Columbia, Vancouver, BC, Canada.
Received December 2, 2007;
accepted after revision June 3, 2008.
Address correspondence to M. C. B. Godoy
(migbarco{at}gmail.com).
Abstract
OBJECTIVE. The purpose of this study was to describe the thoracic
radiologic findings of chronic granulomatous disease in adults.
MATERIALS AND METHODS. We retrospectively analyzed the chest
radiographic and CT findings in four adults with chronic granulomatous disease
during five episodes of lower respiratory tract infection.
RESULTS. Chest radiographic findings included areas of consolidation
(60%), diffuse reticulonodular opacities (40%), pleural effusion (20%), and
pulmonary artery enlargement (20%). CT findings included areas of
consolidation (60%), pulmonary nodules in a random distribution (60%),
centrilobular nodules (60%), tree-in-bud opacities (40%), areas of scarring
and traction bronchiectasis (100%), emphysematous changes (75%), areas of
decreased attenuation and vascularity associated with air trapping on
expiratory CT (50%), mediastinal and/or hilar lymphadenopathy (60%), pulmonary
artery enlargement (50%), and pleural effusion (20%). Areas of consolidation
and nodules were the most prominent findings and at histologic examination
were found to be associated with infection or granulomatous inflammation.
CONCLUSION. The pulmonary radiologic findings of chronic
granulomatous disease include consolidation, nodules, areas of scarring,
traction bronchiectasis, emphysema, air trapping, mediastinal and hilar
lymphadenopathy, pulmonary artery enlargement, and pleural effusion.
Keywords: chest imaging • chronic granulomatous disease • primary immunodeficiency disorders • pulmonary imaging • pulmonary infection
Introduction
Chronic granulomatous disease (CGD) is an inherited disorder of the immune
system caused by a defect in the gene that encodes reduced nicotinamide
adenine dinucleotide phosphate (NADPH) oxidase. The result is deficient
production of the oxygen radicals needed by phagocytes for intracellular
killing of microorganisms. The incidence of CGD in the United States is
between one case in 200,000 and one case in 250,000 live births
[1–3].
Although different molecular defects can cause CGD, they all affect one of the
components of the NADPH oxidase system of the phagocytic cell
[1,
2]. An X-linked recessive
defect is the most common form of the disease. Consequently the disorder is
much more common in men and boys, who were 86% of 368 patients in the U.S.
national registry in 2000 [2].
Other forms of the disease are caused by autosomal recessive defects in other
major components of the oxidase
[2,
4].
The characteristic manifestation of CGD is recurrent bacterial and fungal
infections. Pulmonary infection is the most frequent manifestation, affecting
nearly 80% of patients at some time
[2]. The most common organisms
causing pneumonia are Aspergillus species, Staphylococcus
species, Burkholderia cepacia, Nocardia species, and
Serratia species [2,
3]. Other common infections
include subcutaneous and visceral abscess formation, suppurative adenitis,
osteomyelitis, bacteremia, fungemia, and cellulitis or impetigo
[1–3].
The development of exuberant inflammatory responses with granuloma
formation is another manifestation of CGD and usually involves the lungs,
liver, urinary tract, lymph nodes, skin, spleen, bone, and gastrointestinal
tract [1,
5,
6]. A variety of inflammatory
and rheumatic diseases, such as obstructive lesions of the upper
gastrointestinal and urinary tracts, inflammatory bowel disease, pneumonitis,
and lupus-like syndrome have been described in patients with CGD
[1–3].
The cause of this abnormal inflammatory response and granuloma formation is
unclear
[1–3].
In some cases, the condition arises from chronic inflammatory stimulation
caused by incompletely resolved or recurrent infection
[1,
6]. There is evidence, however,
that exuberant inflammation can occur independently of infection, possibly
owing to an inability to inactivate proinflammatory substances
[1].
As described by Winkelstein et al.
[2], early diagnosis,
prophylactic administration of antimicrobial agents and interferon 
,
and aggressive management of infections have converted CGD from a fatal
granulomatous disease of childhood to a chronic disease that affects children
and adults because a larger number of patients are surviving to adulthood
[2,
7]. Currently, approximately
50% of patients with CGD survive through the third or fourth decade of life
[6,
8]. Furthermore, although CGD
most commonly manifests itself in the first years of life, in approximately
10% of patients the diagnosis is not made until the second decade of life or,
on rare occasions (4%), even later
[2,
4,
9]. Knowing the radiologic
manifestations of this disease in adults is relevant. There is little
information in the literature regarding the radiologic manifestations of CGD
in this population. The aim of this study was to analyze the radiographic and
CT findings of CGD in adults.
Materials and Methods
We retrospectively reviewed the chest radiographic and CT findings of four
adults with longstanding X-linked CGD diagnosed in infancy or childhood. The
patients were treated at our institution from 1999 to 2006 during five
episodes of lower respiratory tract infection and during follow-up after
treatment. All four patients were men (mean age, 24 years; range, 20–29
years). All patients were nonsmokers. Informed consent was not required for
our retrospective study, which involved only review of previously obtained
data. Patient confidentiality was protected.
The diagnosis of acute lung infection was based on the clinical symptoms
(shortness of breath, cough, chest pain, and fever) in association with new
abnormalities on chest radiographs and resolution of the symptoms with
long-term broad-spectrum antibiotic therapy (n = 4) or progression to
septic shock and death (n = 1). Two patients underwent bronchoscopy
and percutaneous transthoracic core biopsy, the findings of which were
negative for microorganisms and showed granulomatous inflammation of the
lungs. One patient underwent transbronchial biopsy. Both fluorescent and
Ziehl-Nielsen stains showed rare organisms consistent with mycobacteria, but
results of all mycobacterial cultures were negative. One patient underwent
open lung biopsy, which was positive for B. cepacia infection. In
this case, blood culture was also positive.
A total of 10 chest radiographs obtained during acute infection (n
= 5) and the follow-up period (n = 5) were analyzed. A total of 12 CT
scans were analyzed, five obtained during acute infection and seven during
follow-up. All patients underwent at least one follow-up CT examination a mean
of 8.8 months (range, 1–20 months) after acute infection. The mean
interval between chest radiography and CT was 3.6 days (range, 0–14
days).
The CT scans were obtained with a single-detector scanner (HiSpeed, GE
Healthcare) or a 4-MDCT scanner (LightSpeed Plus, GE Healthcare). The images
were acquired at end-inspiration from the apex of the lung to the diaphragm at
120 kV and 210 mA. The CT scans obtained during acute infection had
slice-thickness reconstruction of 5 mm in four cases and 1.25 mm in one case.
The follow-up scans had a slice-thickness reconstruction of 5 mm in four cases
and 1.25 mm in three cases. Two patients underwent expiratory CT, one during
acute infection and the other during follow-up. Only one CT examination was
performed with IV contrast enhancement.
Two experienced chest radiologists in con sensus recorded the chest
radiographic findings as presence or absence of consolidation, nodules,
pleural effusion, and mediastinal or hilar lymphadenopathy. The radiologic
findings were classified as mild, moderate, or severe on the basis of visual
assessment of extent and severity to characterize progression, stability, or
regression of disease. The observers in consensus recorded the CT findings as
presence or absence of consolidation, ground-glass opacity, nodules,
tree-inbud opaci ties, interlobular septal thickening, irregular linear
opacities (reticulation), bandlike opacities, bronch iectasis, traction
bronchiectasis, paraseptal and centrilobular emphysema, areas of decreased
attenuation and vascularity, air trapping, pleural effusion, mediastinal or
hilar lymphadenopathy, and splenomegaly.
Bandlike opacity was defined as an elongated opacity, usually extending to
the pleura, which could be thickened and retracted at the site of contact.
Bronchiectasis was defined as irreversible dilatation of a bronchus. Traction
bronchiectasis was defined as bronchial dilatation in association with
juxtabronchial opacification interpreted as representing retractile pulmonary
fibrosis. The findings were defined and assessed according to the Fleischner
Society nomenclature [10].
These findings were classified as mild when focal or localized, moderate when
involving two or more lobes, and severe when involving all pulmonary lobes.
Mediastinal or hilar lymphadenopathy was considered present when the
short-axis diameter of the lymph node was greater than 10 mm. When present,
lymphadenopathy was classified as mild when the nodal diameter was less than
20 mm, moderate when the diameter was between 20 and 30 mm, and severe when
the diameter was greater than 30 mm. Pulmonary artery enlargement was
considered present when the transverse diameter of the main pulmonary artery
was greater than 29 mm or when it was greater than the transverse diameter of
the adjacent ascending aorta. Splenomegaly was considered present when the
splenic length was greater than 10 cm
[11,
12].
Results
All patients had abnormal chest radiographic and CT findings during acute
infection and at follow-up (Tables
1 and
2). Chest radiographs showed
areas of consolidation, involving mainly the lower lobes, in three patients
(Figs. 1A,
1B,
1C,
1D,
1E,
1F and
2A,
2B,
2C,
2D,
2E). Diffuse bilateral
reticulonodular opacities were found in two cases (Fig.
3A,
3B,
3C,
3D). Unilateral pleural
effusion and pulmonary artery enlargement were found in one patient (Fig.
3A,
3B,
3C,
3D).
View larger version (126K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B —26-year-old man with X-linked chronic granulomatous disease
presenting with nonproductive cough and pleuritic chest pain. High-resolution
CT scan shows scarring, traction bronchiectasis, areas of decreased
attenuation and vascularity, and emphysema in the upper lobes.
|
|
View larger version (128K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C —26-year-old man with X-linked chronic granulomatous disease
presenting with nonproductive cough and pleuritic chest pain. High-resolution
CT scan shows area of consolidation in the right middle lobe with adjacent
centrilobular nodules, focal interlobular septal thickening, and ground-glass
opacity. A pulmonary nodule is seen in the superior segment of right lower
lobe.
|
|
View larger version (126K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1E —26-year-old man with X-linked chronic granulomatous disease
presenting with nonproductive cough and pleuritic chest pain. Follow-up
high-resolution CT scan, performed 2 months after A–D, after
antibiotic therapy, shows minimal change in the extent of consolidation in the
right middle lobe. Areas of decreased attenuation and vascularity are again
noted bilaterally.
|
|
View larger version (132K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1F —26-year-old man with X-linked chronic granulomatous disease
presenting with nonproductive cough and pleuritic chest pain. CT scan (5-mm
slice thickness) obtained 10 months after E shows decrease in extent of
the consolidation in the right middle lobe and decrease in the pulmonary
nodule in the right lower lobe.
|
|
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2B —22-year-old man with history of chronic granulomatous disease
presenting with fever and productive cough for 10 days. CT scan (5-mm slice
thickness) shows consolidation with surrounding ground-glass opacities in the
left lower lobe.
|
|
View larger version (132K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2C —22-year-old man with history of chronic granulomatous disease
presenting with fever and productive cough for 10 days. Follow-up CT scan
(5-mm slice thickness) performed 1 month after A and B shows
progression of the consolidation in the left upper and lower lobes associated
with small pulmonary nodules and small left pleural effusion.
|
|
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2D —22-year-old man with history of chronic granulomatous disease
presenting with fever and productive cough for 10 days. Follow-up CT scan
(5-mm slice thickness) performed 1 month after A and B shows
progression of the consolidation in the left upper and lower lobes associated
with small pulmonary nodules and small left pleural effusion.
|
|
View larger version (158K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2E —22-year-old man with history of chronic granulomatous disease
presenting with fever and productive cough for 10 days. Chest radiograph
performed 7 days after C and D shows extensive consolidation in
the mid and lower left lung zones. Blood culture and open lung biopsy were
positive for Burkholderia cepacia.
|
|
View larger version (143K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3B —29-year-old man with chronic granulomatous disease presenting
with chest pain and fever. CT scan (5-mm slice thickness) at level of inferior
pulmonary veins shows areas of scarring, traction bronchiectasis, emphysema,
areas of decreased attenuation and vascularity, and bilateral ill-defined
pulmonary nodules measuring up to 7 mm in diameter.
|
|
View larger version (155K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3C —29-year-old man with chronic granulomatous disease presenting
with chest pain and fever. Follow-up chest CT scan (5-mm slice thickness)
performed 10 months after B shows resolution of the small pulmonary
nodules with persistence of fibrotic changes.
|
|
View larger version (139K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3D —29-year-old man with chronic granulomatous disease presenting
with chest pain and fever. High-resolution CT scan at the level of the upper
lobes, performed 7 months after C, shows bilateral scarring with
traction bronchiectasis associated with reticulation, emphysema, and areas of
decreased attenuation and vascularity.
|
|
Areas of scarring, bandlike opacities, and traction bronchiectasis were
seen in all patients at CT during acute infection and follow-up. The scarring
was focal in one case, moderate in two cases, and severe in two cases,
including one case associated with diffuse and bilateral reticulation and
centrilobular emphysema (Fig.
3A,
3B,
3C,
3D). Another two patients also
had centrilobular emphysema, but it was not associated with fibrotic changes
(Fig. 1A,
1B,
1C,
1D,
1E,
1F). The areas of scarring and
emphysema predominated in the upper lobes in all patients. Two patients had
areas of decreased attenuation and vascularity, both in the acute phase and at
follow-up, associated with air trapping on expiratory CT (Fig.
1A,
1B,
1C,
1D,
1E,
1F). This finding was diffuse
but had upper-lobe predominance in both patients. The other two patients did
not undergo expiratory CT, therefore air trapping could not be excluded.
Areas of consolidation involving mainly the lower lobes were seen in the
acute phase in three of five episodes of infection (Figs.
1A,
1B,
1C,
1D,
1E,
1F and
2A,
2B,
2C,
2D,
2E). The follow-up CT scans
(mean, 8.8 months after treatment) showed persistence of this finding with a
slow decrease in size in two cases (Fig.
1A,
1B,
1C,
1D,
1E,
1F). In both of these cases,
extensive additional investigations, including bronchoscopy, core biopsy, and
open lung biopsy, did not reveal the causative organisms and showed
granulomatous inflammation. In the other case (Fig.
2A,
2B,
2C,
2D,
2E), follow-up CT showed an
increase in size of the consolidation and progression from the left lower lobe
to the left upper lobe. Despite broad-spectrum antibiotic treatment, including
coverage for B. cepacia, the consolidation progressed and unilateral
pleural effusion developed. Microbiologic specimens, including percutaneous
needle aspirate, were nondiagnostic. Surgical lung biopsy yielded B.
cepacia, which later was also recovered from a blood culture. Respiratory
failure occurred, and the patient died of septic shock.
Ground-glass opacities were found in two patients in the acute phase and at
follow-up. When present at CT, ground-glass opacities predominated in the
lower lobes and were always associated with consolidation (Figs.
1A,
1B,
1C,
1D,
1E,
1F and
2A,
2B,
2C,
2D,
2E). Randomly distributed
pulmonary nodules measuring up to 1.4 cm in diameter were found during acute
infection in two cases. Follow-up CT showed persistence of this finding in one
case. Randomly distributed pulmonary nodules measuring up to 7 mm were found
in one case (Fig. 3A,
3B,
3C,
3D). In this patient,
bronchoalveolar lavage was nondiagnostic. At transbronchial biopsy, however,
fluorescent and Ziehl-Neelsen stains both showed rare organisms consistent
with mycobacteria. Results of all mycobacterial cultures remained negative.
The patient's clinical condition improved with empiric therapy for
tuberculosis and Mycobacterium avium complex infection. Follow-up CT
showed a decrease in size and number of the nodules.
Focal centrilobular micronodules were found in two cases during the acute
phase and in three cases at follow-up (Fig.
2A,
2B,
2C,
2D,
2E). These nodules were
associated with tree-in-bud opacities in one patient in the acute phase and in
two patients at follow-up. When present, centrilobular micronodules and
tree-in-bud opacities were always associated with consolidation and had
lower-lobe predominance.
Focal interlobular septal thickening was found both in the acute phase and
at followup in the two patients who underwent high-resolution CT during three
episodes of infection (Fig. 1A,
1B,
1C,
1D,
1E,
1F). CT showed mediastinal
lymph adenopathy in three patients during acute infection and at follow-up,
and hilar lymphadenopathy in two patients during the acute phase and in three
at follow-up. Small unilateral pleural effusion was found in only one patient
at follow-up CT (Fig. 2A,
2B,
2C,
2D,
2E) and was associated with
lung consolidation and nodules. CT showed pulmonary artery enlargement in two
patients, and pulmonary artery hypertension was confirmed at echocardiography.
All patients had splenomegaly.
CT was superior to chest radiography in the detection of pulmonary nodules,
areas of decreased attenuation and vascularity, air trapping, emphysema,
traction bronchiectasis, and pulmonary artery enlargement.
Discussion
To our knowledge, the first case of CGD was described in 1957 by Berendes
et al. [7]. The patient was a
12-month-old boy with suppurative lymphadenitis, pulmonary opacities,
hepatosplenomegaly, and eczematoid dermatitis. CGD later was recognized as a
rare inherited immunodeficiency characterized by NADPH oxidase deficiency,
lack of generation of superoxide and related toxic oxygen metabolites, and
consequently, inability of phagocytes to effectively kill ingested organisms,
especially catalase-positive organisms, causing recurrent pyogenic infections,
especially pneumonia [2,
3,
5,
13]. Few publications in the
literature describe the radiologic findings of CGD of the lungs, and most of
them focus on the pediatric population. Our study was limited by the small
number of patients, but the rarity of the disease, specifically in the adult
population, limits the study of larger series. Previous authors have described
findings of chronic or recurrent pneumonia, including abscess formation
[14], empyema
[13], osteomyelitis involving
the ribs and vertebral bodies
[13,
14], chest wall invasion
[13–18],
and hilar and mediastinal lymphadenopathy
[13,
14].
In our patients, the diagnosis of pneumonia was made by correlation of
clinical and radiologic findings. The infections in two patients had a
markedly protracted course complicated by granulomatous inflammation, chest
radiography and CT showing areas of consolidation and pulmonary nodules. Both
patients' conditions improved slowly after broad-spectrum antibiotic therapy.
The size of the areas of consolidation decreased slowly during long-term
follow-up. Khanna et al. [13]
described that lung infections in children with CGD also tended to follow a
protracted course and could be complicated by granulomatous inflammation.
None of our adult patients had lung abscesses, empyema, or chest wall
invasion during the evaluation, although these complications are described in
as many as 20% of children with CGD
[13]. One of our patients had
a history of pulmonary Scedosporium apiospermum (formerly
Pseudallescheria boydii) infection during childhood that extended to
the thoracic spine and necessitated surgical intervention
[19]. Pulmonary scarring and
fibrotic changes have been described in children with CGD
[13,
20]. All of our patients had
these changes. These findings were extensive, however, in only one case,
compromising all lung lobes and being associated with extensive emphysematous
changes, although the patient was a nonsmoker. Two other patients also had
small amount of centrilobular emphysema, although they also were
nonsmokers.
Areas of decreased attenuation and vascularity were present in two of four
patients. Air trapping was present in both patients who underwent expiratory
CT. These changes and the areas of emphysema were presumed to be related to
recurrent infection because all patients were nonsmokers. These findings were
previously rarely mentioned in patients with CGD
[13].
Despite numerous previous infections, bronchiectasis was not a significant
finding, and although it was found in two patients, this finding was focal and
mild. Persistent hilar or mediastinal lymphadenopathy was a common finding in
our series (60%) as previously reported
[13,
14]. Two patients had
radiologic signs of pulmonary hypertension, one presumed to be secondary to
extensive pulmonary fibrosis. The presence of hepatosplenomegaly has been
described in 90% of patients with CGD
[13]. Our findings are
consistent with that because all patients had splenomegaly.
In patients with CGD, each infection is potentially life-threatening, the
greatest mortality being related to respiratory tract infection. In the study
by Winkelstein et al. [2],
infection caused by Aspergillus was the most common cause of death,
accounting for more than one third of all deaths. Infections caused by
Burkholderia also were a relatively common cause of death, death due
to Pseudomonas and Candida infections occurring less
commonly. Fatal infections with Burkholderia or Pseudomonas
species often involve concurrent pneumonia and sepsis, as in one of our
patients with Burkholderia infection. The 5-year survival rate among
patients with CGD has been estimated at 76–88%
[2]. Survival rates have
improved markedly from the original studies on CGD. The improvement is thought
to be a result of the combination of earlier diagnosis, prophylaxis of
infection, immunoprophylaxis with interferon , and early aggressive
management of infections
[2].
Despite progress, recurrent infections account for most of the morbidity
and mortality among patients with CGD. Infections are often a diagnostic and
therapeutic challenge because patients may present with relatively mild
clinical symptoms and signs, and the causative organisms can be difficult to
isolate. Invasive procedures (e.g., lung biopsy) are seldom needed in the
management of pneumonia in immunocompromised patients. In contrast, early lung
biopsy should be considered for patients with CGD and pneumonia, particularly
if there has not been a convincing response to empiric therapy directed at the
usual causative organisms. Percutaneous fine-needle aspiration before
antibiotic therapy has been recommended
[1], but relative yield and the
timing of various invasive techniques (e.g., fine-needle aspiration,
transbronchial, and open lung biopsy) in this setting have not been evaluated,
to our knowledge. Unfortunately, bronchoscopy appears to be usually unhelpful
in the diagnosis of CGD-associated pneumonia. Radiologists play an important
role in the diagnosis of the complications of CGD and must be aware of most
common radiologic findings in the adult population.
In conclusion, CGD is an inherited disorder of the immune system that
results from deficient phagocytic function and leads to recurrent bacterial
and fungal infections associated with granulomatous inflammation. Advances in
the treatment of patients with CGD have resulted in improved life expectancy,
with more patients reaching adult life than did in the past. The most
prominent radiologic findings of CGD in adults include air-space consolidation
and pulmonary nodules. These findings may be the result of acute infection or
chronic granulomatous inflammation. Other common findings in these patients
include areas of scarring, emphysematous changes, areas of decreased
attenuation and vascularity, air trapping, and mild traction
bronchiectasis.
References
- Segal BH, Leto TL, Gallin JI, Malech HL, Holland SM. Genetic,
biochemical, and clinical features of chronic granulomatous disease.
Medicine (Baltimore) 2000;79
: 170–200[CrossRef][Medline]
- Winkelstein JA, Marino MC, Johnston RB Jr, et al. Chronic
granulomatous disease: report on a national registry of 368 patients.
Medicine (Baltimore) 2000;79
: 155–169[CrossRef][Medline]
- Johnston RB Jr. Clinical aspects of chronic granulomatous disease.
Curr Opin Hematol 2001;8
: 17–22[CrossRef][Medline]
- Liese JG, Jendrossek V, Jansson A, et al. Chronic granulomatous
disease in adults. Lancet 1996;347
: 220–223[CrossRef][Medline]
- Ma JS, Chen PY, Fu LS, et al. Chronic granulomatous disease: a case
report. J Microbiol Immunol Infect 2000;33
: 118–122[Medline]
- Liese J, Kloos S, Jendrossek V, et al. Long-term follow-up and
outcome of 39 patients with chronic granulomatous disease. J
Pediatr 2000; 137:687
–693[CrossRef][Medline]
- Berendes H, Bridges RA, Good RA. A fatal granulomatosus of
childhood: the clinical study of a new syndrome. Minn
Med 1957; 40:309
–312[Medline]
- Finn A, Hadzic N, Morgan G, Strobel S, Levinsky RJ. Prognosis of
chronic granulomatous disease. Arch Dis Child1990; 65:942
–945[Abstract/Free Full Text]
- Lun A, Roesler J, Renz H. Unusual late onset of X-linked chronic
granulomatous disease in an adult woman after unsuspicious childhood.
Clin Chem 2002;48
: 780–781[Free Full Text]
- Austin JH, Muller NL, Friedman PJ, et al. Glossary of terms for CT
of the lungs: recommendations of the Nomenclature Committee of the Fleischner
Society. Radiology 1996;200
: 327–331[Free Full Text]
- Bezerra AS, D'Ippolito G, Faintuch S, Szejnfeld J, Ahmed M.
Determination of splenomegaly by CT: is there a place for a single
measurement? AJR 2005;184
:1510
–1513[Abstract/Free Full Text]
- Lamb PM, Lund A, Kanagasabay RR, Martin A, Webb JA, Reznek RH.
Spleen size: how well do linear ultrasound measurements correlate with
three-dimensional CT volume assessments? Br J Radiol2002; 75:573
–577[Abstract/Free Full Text]
- Khanna G, Kao SC, Kirby P, Sato Y. Imaging of chronic granulomatous
disease in children. RadioGraphics 2005;25
:1183
–1195[Abstract/Free Full Text]
- Yin EZ, Frush DP, Donnelly LF, Buckley RH. Primary immunodeficiency
disorders in pediatric patients: clinical features and imaging findings.
AJR 2001; 176:1541
–1552[Free Full Text]
- Wilhelm L, McLeary MS, Janner D. MR diagnosis of pulmonary and
chest wall aspergillosis as an initial presentation of chronic granulomatous
disease in a 7-month-old male. Pediatr Radiol2000; 30:719
–720[CrossRef][Medline]
- Gaisie G, Bowen A, Quattromani FL, Oh KS. Chest wall invasion by
aspergillus in chronic granulomatous disease of childhood. Pediatr
Radiol 1981; 11:203
–206[CrossRef][Medline]
- Altman AR. Thoracic wall invasion secondary to pulmonary
aspergillosis: a complication of chronic granulomatous disease of childhood.
AJR 1977; 129:140
–142[Medline]
- Kawashima A, Kuhlman JE, Fishman EK, et al. Pulmonary
Aspergillus chest wall involvement in chronic granulomatous disease:
CT and MRI findings. Skeletal Radiol1991; 20:487
–493[Medline]
- Phillips P, Forbes JC, Speert DP. Disseminated infection with
Pseudallescheria boydii in a patient with chronic granulomatous
disease: response to gamma-interferon plus antifungal chemotherapy.
Pediatr Infect Dis J 1991;10
: 536–539[Medline]
- Pogrebniak HW, Gallin JI, Malech HL, et al. Surgical management of
pulmonary infections in chronic granulomatous disease of childhood.
Ann Thorac Surg 1993;55
: 844–849[Abstract]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Top
Abstract
Introduction
