Published online August 7, 2006
PEDIATRICS Vol. 118 No. 3 September 2006, pp. e914-e920 (doi:10.1542/peds.2006-0113)
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EXPERIENCE & REASON

Churg-Strauss Syndrome in Children: A Clinical and Pathologic Review

Debra Boyer, MDa, Sara O. Vargas, MDb, Dubhfeasa Slattery, MDa, Yadira M. Rivera-Sanchez, MDa and Andrew A. Colin, MDa

a Departments of Medicine (Division of Respiratory Diseases)
b Pathology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts

ABSTRACT

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.

Key Words: Churg-Strauss syndrome • pediatric • eosinophilia • pulmonary

Abbreviations: CSS, Churg-Strauss syndrome • CT, computed tomography • WBC, white blood cell • Ig, immunoglobulin • ANCA, antineutrophil cytoplasmic antibodies • p-ANCA, antineutrophil cytoplasmic antibodies with a perinuclear pattern • c-ANCA, antineutrophil cytoplasmic antibodies with a cytoplasmic pattern • ACR, American College of Rheumatology

Churg-Strauss syndrome (CSS) is an uncommon entity that is rarely reported in children.1 The clinical presentation is variable and depends on the stage of the illness and previous use of corticosteroids. Data on the pathologic features of CSS in childhood are sparse. Here we present the cases of 2 patients: a young woman with pleuritic chest pain and a peripheral pulmonary nodule and a younger child with respiratory symptoms, fatigue, and weight loss. The diagnosis of CSS was suggested both by a history of asthma and peripheral eosinophilia and, in the first patient, by an eosinophilic pleural effusion. The diagnosis was substantiated by lung biopsies. The findings illustrate the multisystem and severe involvement that may affect this age group. Furthermore, these cases highlight that eosinophilia, whether it be noted in pleural fluid, bronchoalveolar lavage, serum, or a tissue specimen, must heighten the physician's concern for CSS.

CASE REPORTS

Patient1.
K.T. was a 16-year-old athletic white girl with a past history of moderate asthma that had not required medication for years. In the remote past, she had been treated for sinusitis. Six months before presentation, she developed left chest pain of unknown etiology that resolved after treatment with clarithromycin and corticosteroids.

Having been asymptomatic for 6 months, she presented with a 2-week history of fever (101°F), mild cough, and right-sided pleuritic chest pain. Transient improvement was provided by a short course of oral corticosteroids, but her pain recurred after discontinuation. Relevant negatives included no shortness of breath, hemoptysis, cigarette smoking, or drug use.

A chest radiograph was normal, as were previous films. Because of the persistence and focality of the pain, chest computed tomography (CT) was performed. CT of the chest revealed a wedge-shaped pleural-based right middle lobe infiltrate associated with a small pleural effusion and a small lingular scar (Fig 1). Oral corticosteroids again provided significant pain relief. With the concern for a pulmonary embolism, a Xenon perfusion scan and lower-extremity Doppler ultrasound were performed, the results of which were normal.


Figure 1
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  		FIGURE 1 Patient 1. CT of the chest: wedge-shaped, pleural based infiltrate of right middle lobe. A small pleural effusion is noted.

 
Flexible bronchoscopy revealed an extremely narrowed orifice (<3.0 mm), initially presumed to be the orifice to the right middle lobe but later shown to be a cardiac bronchus. There were no significant secretions. A bronchoalveolar lavage returned a scant amount of fluid containing 70% macrophages, 25% columnar cells, and 5% neutrophils. All cultures and serologic studies were negative for multiple infectious etiologies including Mycoplasma, Coccidioides, Blastomyces, Histoplasma, Aspergillus, and parasitic diseases.

Additional laboratory studies revealed a white blood cell (WBC) count of 8930/mm3 with 67% neutrophils, 18% lymphocytes, 7% eosinophils, and 3% monocytes, negative antinuclear antibody, an angiotensin-converting enzyme level of 25 IU/L (reference range: 14–78 IU/L), an immunoglobulin E (IgE) level of 93 U/mL (reference range: 0–200 U/mL), a rheumatoid factor level of 11.8 IU/mL (reference range: <10.0 IU/mL), and an erythrocyte sedimentation rate of 48 (reference range: 0–20). A test for antineutrophil cytoplasmic antibodies with a perinuclear pattern (p-ANCA) was negative, and although a test for ANCA with a cytoplasmic pattern (c-ANCA) was negative, her proteinase-3 autoantibody level was positive at 23.2 IU/mL (reference range: <3.5 IU/mL). A previous complete blood count (dating from before this hospitalization) revealed a peripheral WBC count of 7300/mm3 with 15% eosinophils. The possibility of a postobstructive pneumonia was considered, and she was discharged with a 2-week course of amoxicillin/clavulanic acid.

Her pleuritic chest pain recurred bilaterally and was associated with a larger right middle-lobe infiltrate and associated effusion on repeat CT. CT-guided needle aspiration of pleural fluid revealed negative cultures, pH of 8.0, WBC count of 7545/mm3 with 57% lymphocytes, 8% monocytes, 17% neutrophils, and 17% eosinophils, and 100000 red blood cells. A repeat flexible bronchoscopy was unchanged from the previous one.

Six weeks after the onset of her symptoms, a thoracoscopic lung biopsy removed the right middle lobe mass in its entirety. An intraoperative flexible bronchoscopy with bronchial biopsy was performed. Gross examination of the wedge biopsy specimen showed a 4-cm pale, poorly circumscribed, slightly firm nodule. The overlying pleura showed a fibrinous exudate. Microscopic examination showed a necroinflammatory process, characterized by extensive geographic necrosis with surrounding palisading histiocytes and abundant giant cells. Vasculitis consisting of an endothelialitis rich in eosinophils was present. Fibrinoid necrosis and granulomatous inflammation of vessel walls was not identified, nor was capillaritis. There was a striking profusion of extravascular microgranulomas, filled exclusively with eosinophils and granular eosinophilic debris (Fig 2). Sarcoidal-type granulomas were absent, and results of special stains for microorganisms were negative. A diagnosis of CSS was suggested. Histologic evaluation of the bronchial biopsy showed features of asthma and chronic bronchitis, including frequent eosinophils, mucous gland hyperplasia, and a thickened basement membrane.


Figure 2
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  		FIGURE 2 Patient 1. A, Microabscesses filled solely with eosinophils and eosinophilic debris and surrounded by palisading histiocytes (original magnification: 40 x). B, Collections of epithelioid histiocytes with admixed giant cells (original magnification: x40). C, Pulmonary artery showing endothelialitis with intramural eosinophils (original magnification: 20 x).

 
The patient was treated with high-dose oral corticosteroids, initially at a dose of 2 mg/kg per day, resulting in a 5-year abatement of her pleuritic pain. Over the first few years of her treatment, she developed joint involvement and was forced to abandon all athletic activities. Treatment complications also included weight gain, fatigue, and mild cataracts. Attempts to wean her from corticosteroids resulted in severe skin rashes, but she eventually tolerated maintenance treatment on low doses of methotrexate (10 mg every 3 weeks). One year after her diagnosis, she experienced episodes of loss of consciousness of unknown etiology despite extensive neurologic workup. Antiinflammatory and anticonvulsant therapies failed to improve these episodes, which subsequently resolved spontaneously. Electrocardiographic, echocardiographic, and cardiac evaluation were normal.

Patient2.
V.G. was a 6-year-old girl with a 2-year history of well-controlled asthma and allergic rhinitis. She presented with a 3-week history of productive cough that was worse at night and on exertion. Of note, she had reduced energy and a 2.3-kg weight loss, as well as increased wheezing and dyspnea. She was admitted because of the exacerbation of her symptoms and a chest radiograph, which revealed a left upper lobe multicystic cavitary lesion (Fig 3A).


Figure 3
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  		FIGURE 3 Patient 2. A, Chest radiograph: cystic lesion in left upper lobe. B, CT of the chest: a thick-walled multiloculated cavitary lesion in the apical segment of the left upper lobe with associated bronchiectasis.

 
Her asthma therapy included fluticasone 110 µg twice daily for 2 years with the addition of montelukast 4 mg daily for 1 year. Relevant history included contact with an uncle's friend who had a diagnosis of tuberculous disease.

On admission, her vital signs were normal, and her weight was between the 5th and 10th percentiles for age. Examination revealed pallor but was otherwise normal.

Initial laboratory data included a WBC count of 10800/mm3 with 5% eosinophils and 79% neutrophils. Her erythrocyte sedimentation rate was 67 mm/hour. A sweat test ruled out cystic fibrosis, and testing to purified protein derivative was negative. Allergy skin testing was positive for Aspergillus, molds, and cat dander. Results of Mycoplasma titers and immunologic workup including Ig levels (IgG, IgM, IgA, IgE), pneumococcal and tetanus titers, HIV, angiotensin-converting enzyme, and complement levels were all normal. Tests for c-ANCA and p-ANCA were negative, and her antinuclear antibody level was 1:160. CT of the chest confirmed a thick-walled multiloculated cavitary lesion in the apical segment of the left upper lobe and a nodular density in the left lower lobe (Fig 3B). Both lesions had associated bronchiectasis. Flexible bronchoscopy demonstrated purulent secretions at the right upper and left upper lobes. Bronchoalveolar lavage cytology studies revealed a predominance of inflammatory cells with 45% neutrophils and 20% eosinophils. Cultures and/or stains for virus, fungus, and acid-fast bacilli were all negative. Bronchoalveolar lavage cultures grew Moraxella catarrhalis. She was treated with ampicillin-sulbactam for a period of 3 weeks.

Two weeks after discharge her respiratory symptoms deteriorated, but a repeat chest radiograph remained unchanged. On readmission, peripheral eosinophilia had increased to 19%. Of note, 1 month later her eosinophil count had increased to 28% with an absolute eosinophil count of 3130/mm3. The left upper lobe cavitation had expanded on repeat CT scan.

Thoracoscopic lung biopsy from the left upper lobe including the cavitary lesion was undertaken. Histologic examination showed eosinophilic pneumonia with eosinophilic abscesses and vasocentric collections of eosinophils. Mural eosinophilic infiltration without necrosis was noted in multiple small arteries (Fig 4). In the context of her presentation and extensive negative cultures and serologic assays for infectious organisms, these changes were most consistent with CSS. A bone marrow aspirate performed at the time of the biopsy revealed increased early eosinophilic forms and no malignancy. Results of cardiac evaluation including an electrocardiogram and echocardiogram were normal.


Figure 4
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  		FIGURE 4 Patient 2. A, Perivascular collections of eosinophils with nonnecrotizing "vasculitis" limited to intramural eosinophils (original magnification: x20). B, Organizing eosinophilic pneumonia (original magnification: x20).

 
Corticosteroids were started at 2 mg/kg per day. A peripheral eosinophil count at the time of discharge was 0%, and the sedimentation rate decreased from 44 to 6 mm/hour.

During the 3 years since her diagnosis, attempts to wean her off of corticosteroids resulted in serious exacerbations of her pulmonary and sinus disease and increase of inflammatory markers. Ultimately, azathioprine (500 mg twice a day) and monthly infliximab (5 mg/kg per dose) were initiated as potential steroid-sparing agents. Subsequently, her corticosteroids have been weaned to a dose of 0.2 mg/kg per day, with ongoing efforts to discontinue this therapy.

DISCUSSION

We have described 2 pediatric patients with CSS characterized predominantly by pulmonary involvement. Both had history of asthma and sinusitis, peripheral eosinophilia, nonfixed pulmonary infiltrates, and biopsy-proven eosinophilic abscesses. Secondary causes of eosinophilic syndromes were ruled out. Detailed clinicopathologic analysis of these 2 patients helped to elucidate the features of pediatric CSS, which similar to adult CSS, has a poorly understood pathogenesis.

The syndrome of "allergic granulomatosis and angiitis," characterized by severe asthma, pulmonary infiltrates, eosinophilia, cardiac failure, renal disease, and peripheral neuropathy, was described by Churg and Strauss2 in 1951 and named after the authors. The original report was based on autopsy data and described diffuse vasculitis and extravascular granulomas with eosinophilic cores. This vasculitis was likened to that of periarteritis nodosa and was often associated with granulomas. Frequently, these changes were accompanied by histologic features of bronchial asthma.3,4 CSS is a rare entity, with a mean age of onset of 38 to 45 years.5,6

Reports of CSS occurring in children are limited and generally consist of single case reports. The findings in our patients demonstrate that CSS in children may present with features typical of adult CSS. Nevertheless, it is conceivable that patients may go undiagnosed because of the lack of specificity, low index of suspicion, and invasiveness often involved with pathologic confirmation. Specific clinical criteria developed by the American College of Rheumatology (ACR) for the classification of CSS include history of asthma, eosinophilia >10%, mononeuropathy/polyneuropathy, nonfixed pulmonary infiltrates, paranasal sinus abnormality, and a biopsy with extravascular eosinophils. The presence of ≥4 of the 6 criteria are thought to have a diagnostic sensitivity of 85% and specificity of 99.7%.6 Both patients presented herein met 5 of the 6 ACR diagnostic criteria, namely, history of asthma, history of sinusitis, eosinophilia >10%, nonfixed pulmonary infiltrates, and a lung biopsy revealing extravascular eosinophils.

Three clinical stages have been described in the clinical evolution of CSS. The prodromal phase involves allergic rhinitis and asthma that can be protracted for many years. The second includes peripheral eosinophilia and eosinophilic tissue infiltration. The hallmark of the final phase is a systemic vasculitis, which, if left untreated, can be fatal.5,7 The predominant causes of death include heart failure, renal failure, cerebral failure, and gastrointestinal perforation/hemorrhage.5 Cardiovascular disease occurs in approximately half of the patients with CSS irrespective of age. Specific cardiac involvement in CSS includes pericarditis and, less commonly, tamponade and myocarditis.8 Congestive heart failure/myocardial infarction accounted for 48% of all deaths in one series.5 All patients with CSS should be screened for signs of cardiac involvement.

The incidence of CSS in adults is estimated at 2.4 per million per year.7 CSS remains rare also among asthma patients. Because of its rarity in children, the incidence of pediatric disease is unknown. A review of CSS in childhood from 1999 identified 10 published cases of children with an average age of 10.2 years and an age range of 4 to 16 years.1 However, an increasing number of reports have emerged in the last 5 years, suggesting either an increasing incidence or, more likely, higher awareness of this entity in the pediatric community. Interestingly, review of the recent literature reveals worldwide reports with a significant number arising from Japan,9–11 raising the question of a possible geographical or ethnic predominance similar to that described for Kawasaki disease.

Of the diagnostic laboratory criteria, only ANCA seems to be significantly different between children and adults. Neither of our 2 patients were positive for ANCA, nor did 9 of the 10 children in the pediatric series. In contrast, more than half of the adults with CSS were positive for ANCA.1,7 In addition to clinical features seen in adults, reported symptoms in children included testicular pain, hypertension, chorea, and seizures.12,13 Renal involvement, although rare in adults with CSS, was seen in 40% of the children in the early review1 but was less frequent in the more-recent reports. The youngest reported patients with CSS were 2 years old. The first was a boy with severe asthma, eosinophilia, diffuse pulmonary infiltrates, and evidence of extravascular eosinophilia. This child died, and autopsy revealed severe myocarditis with eosinophilic infiltration.14 More recently, a boy was reported from Turkey with swelling of his legs and feet, purple itchy lesions, and oral aphthae.15 He subsequently developed hepatomegaly, myalgia, and respiratory distress. The diagnosis was established by skin biopsy. The patient responded to corticosteroid therapy, but the symptoms recurred when therapy was withdrawn.

An increasing number of pediatric patients are reported with various combinations of asthma, polyarthralgia, myalgia, and polyneuropathies presenting as dysesthesia, muscle weakness, and gait disturbances.10,11,16,17 Some of these patients present in combination with lung or cardiac manifestations. The reported range of pulmonary manifestations in childhood is widening. Most frequent are reports of diffuse pulmonary infiltrates, often with high fever and severe systemic manifestations that respond to corticosteroid therapy.9,11,16,18,19 Less common are reports of other pulmonary presentations such as chest pain,9 hemoptysis,17 and pulmonary infarction.9 Severe cardiomyopathies as the presenting feature, often in combination with manifestation of other system involvement, have been reported, albeit less frequently,16 occasionally with pericardial effusion,17 and can be fatal.14 Skin manifestations are a common early presentation of CSS in childhood. They range from discrete to diffuse, patchy, petechial or purpuric lesions and can be itchy, edematous, or tender.9,11,15,16

A number of recent reports reveal more subtle presentations of CSS, including acute abdominal pain,18 sudden painless loss of vision with bilateral optic neuropathy, vasculitis, retinal artery occlusions,20 deep venous thrombosis,9 and discrete cervical lymphadenopathy.21

Detailed pathologic findings in childhood CSS are few, and it is not known whether features differ from the adult form of the disease. Although Churg and Strauss included 1 pediatric patient in their original article from 1951, individual details about the pathologic features in this patient are not separately described.2 Specific pulmonary histologic findings in pediatric CSS have been described only once, briefly, in a report describing "granulomas and angiitis with focal necrosis and eosinophilic infiltration, findings consistent with CSS" in a 14-year-old boy.22 Lung biopsies from both our patients showed aggregates of intra-alveolar eosinophils ("eosinophilic abscesses"), constituting eosinophilic pneumonia. Our first patient's biopsy revealed extravascular microgranulomas filled with eosinophils and their breakdown products. These were described in the original report of CSS, and although not consistently found in adult CSS, they are thought to be specific for the disease.2 In both patients, vasculitis was limited to an endothelialitis rich in eosinophils, similar to that seen in robust cases of eosinophilic pneumonia. In adult forms of CSS, capillaritis and granulomatous vasculitis are often present. Although the 2 patients presented here contribute significantly to existing knowledge of the pathologic features of childhood CSS, we think that the number of reported childhood cases remains too small for adequate comparisons between the histologic features of adult and childhood disease.

The cornerstone of treatment for CSS remains corticosteroid therapy, often started in doses of 1 mg/kg per day. Corticosteroids have significantly increased survival, which now approaches >75% at 5 years.7 The longest reported survival is 37 years.23 Obviously, there is limited information about survival or long-term outcomes in childhood. In patients with severe or unremitting disease, as is the case with both our young patients, combination therapy with cyclophosphamide, azathioprine, infliximab, or methotrexate may be required. Recent literature has supported the use of these various immunosuppressive medications to both treat refractory CSS symptoms and minimize the development of relapses.24,25

Cardiac and renal involvement significantly decreases the survival in affected patients. Proteinuria >1 g/day, renal insufficiency, cardiomyopathy, and gastrointestinal tract or central nervous system involvement are all predictors of worse prognosis. A large prospective study suggests that as the number of systems involved increases, a higher mortality is observed.26

Both patients we report had a prodrome of asthma and sinusitis. The course of our first patient was typical for CSS, with the asthma in remission for years before the diagnosis of the pulmonary vasculitis. In her case, the eosinophilic pleural effusion was the first clue to the diagnosis. Eosinophils in a pleural effusion are nonspecific and can be found in various conditions including trauma, pneumothorax from any cause, malignancy, congestive heart failure, infections, and rheumatologic conditions such as CSS.27,28 Thus, although rare, CSS should be considered in the differential diagnosis of an eosinophilic effusion.29

Although both patients had pulmonary vasculitis, they initially had no evidence of other systemic vasculitic disease. Thus, their diagnoses were apparently made before the widespread vasculitic stage. The first patient had received short courses of corticosteroids before her diagnosis for presumed asthma; however, the doses were likely insufficient to suppress the development of a systemic vasculitis. One year after the initial diagnosis, soon after discontinuation of therapy, she did develop skin involvement with a vasculitic rash and, subsequently, a much more significant and poorly defined neurologic involvement. In adults there have been reports of CSS similar to our patients with asthma, eosinophilic pulmonary infiltrates, and pulmonary vasculitis, without progression to systemic vasculitis.5 It is notable that patients with asthma treated with steroids may present with CSS after steroid taper, suggesting that there is suppression of the full clinical picture with prolonged steroid therapy.3 Such cases were attributed to montelukast and other leukotriene modifiers,10 but recent literature suggests that improved asthma control by the addition of a leukotriene modifier allows tapering of the steroid dose and consequently the unmasking of an underlying systemic inflammatory process.30–33 Our older patient did not receive montelukast, but the younger patient did. Her case was reported previously in a short communication34 that emphasized her being the youngest patient reported to date with CSS after a prolonged course of montelukast.

Asthma and eosinophilia are common in children, and treatment with corticosteroids is frequent. Although rare, CSS must be considered when the constellation of asthma, peripheral eosinophilia, and symptoms that may be compatible with a systemic vasculitis are suspected. Specifically, evidence of neuropathy or cardiomyopathy in conjunction with a previous history of asthma and sinusitis should alert the practitioner to this diagnosis even in children and young adults. Corticosteroid use may limit a clinician's ability to diagnose CSS by modifying the typical findings.

The cause of CSS remains unknown. Of great importance, recent studies have demonstrated primary genetic causes of hypereosinophilia.35–40 There is significant clinical overlap between such syndromes and CSS. It is not known what subset of patients with CSS may have such an underlying genetic mechanism of disease. It is possible that primary genetic disorders may tend especially to underlie disease with early (childhood) presentations, and such investigation, although beyond the scope of this report, remains among the most promising ways to advance our knowledge about the pathogenesis and possible improved modes of treatment of childhood CSS.

Obviously, a high index of suspicion is key to the diagnosis of CSS in childhood. Some of the reported cases received diagnoses on the basis of their clinical and laboratory parameters and compatibility with the ACR criteria6 but were made without histologic confirmation.10,20 The diagnosis is frequently established by skin biopsy.9,15,17,18 For lung involvement, the older literature reveals open or thoracoscopic lung biopsy as the common mode of obtaining the diagnostic sample.1,16 Increasingly, however, the diagnosis is aided by transbronchial lung biopsy11,19,22 and, infrequently, sinus biopsy.19 Our opinion is that the seriousness of this disease and its impact on longevity requires tissue diagnosis and should not rely solely on the ACR criteria. The latter could potentially lead to "overdiagnosis" of borderline cases. In our own experience, transbronchial biopsies in young patients yield a minute amount of tissue; therefore, we suggest that such samples be used to confirm a diagnosis when appropriate but should not be used to rule the diagnosis out before a larger tissue sample can be analyzed.

The diagnosis of CSS in childhood remains challenging. However, a strong clinical suspicion coupled with pathologic confirmation can expedite the diagnosis. With recent advances in immunomodulatory therapy, early treatment may significantly improve the outcome of this disease.

ACKNOWLEDGMENTS

We thank Peter Weller, MD, from Beth Israel-Deaconess Medical Center, Peter Merkel, MD, from Boston University Medical Center, and Michael Wechsler, MD, from Brigham and Women's Hospital (all in Boston, MA) for assistance with the diagnosis and management of the first patient.

FOOTNOTES

Accepted Mar 20, 2006.

Address correspondence to Debra Boyer, MD, Division of Respiratory Diseases, Children's Hospital Boston, 300 Longwood Ave, Hunnewell 2, Boston, MA 02155. E-mail: debra.boyer{at}childrens.harvard.edu

The authors have indicated they have no financial relationships relevant to this article to disclose.

Dr Slattery's current address is Children's University Hospital, Temple St, Dublin 1, Ireland.

Dr Rivera-Sanchez's current address is Pediatric Pulmonary Section, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland OH 44195.

Dr Colin's current address is Division of Pediatric Pulmonology, Miller School of Medicine, University of Miami, 1580 NW 10th Ave, Miami, FL 33136

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PEDIATRICS (ISSN 1098-4275). ©2006 by the American Academy of Pediatrics

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Monteleukast ?
Jayendra R Gohil
Pediatrics Online, 18 Aug 2006 [Full text]
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