Churg-Strauss Syndrome: A Patient Report in Infancy

Yüksek, Mutlu; Kunak, Benal; Ertan, Ülker; İğde, Mahir

doi:10.1177/000992280304200412

Cited by 9 publications

(7 citation statements)

References 4 publications

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“…One was a male with a fatal evolution involving the heart, 4 and the other one was a male with severe asthma and skin manifestations. 5 Interestingly, this patient also suffered from oral aphthaes, as in case 2. In the literature, a case of an affected 4-yearold was also described.…”

Section: Discussionmentioning

confidence: 75%

“…2 Furthermore, it is even more rarely described in the pediatric population, and only a few cases in that age group and one cohort study of asthmatics with two probable pediatric cases have been reported since its description in 1951. [3][4][5][6][7][8][9][10][11][12][13][14] We report on two cases of Churg-Strauss syndrome in the pediatric population. To our knowledge, these are the first two cases reported in children in Mexico.…”

Section: Introductionmentioning

confidence: 99%

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Pediatric Churg‐Strauss syndrome in Mexico

Hernández-Bautista

Espinosa‐Padilla

Yamazaki‐Nakashimada

et al. 2006

Pediatric Pulmonology

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Section: Discussionmentioning

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Pediatric Churg‐Strauss syndrome in Mexico

Hernández-Bautista

Espinosa‐Padilla

Yamazaki‐Nakashimada

et al. 2006

Pediatric Pulmonology

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“…Not less than 15 pediatric cases have been cited in literature during the past 8 years [4,6-18]. The youngest reported patients with CSS were 2 years old [13,19]. …”

Section: Epidemiologymentioning

confidence: 99%

“…They range from discrete to diffuse patchy, petechial, or purpuric lesions and can be itchy, edematous, or tender [9,13,15,51]. A 12-year-old child with CSS experienced typical cutaneous manifestations including palpable purpura, skin nodules, urticarial rash, and rare lesions, such as finger tip vesicles, infiltrated papules, and aseptic pustules [7].…”

Section: Clinical Picturementioning

confidence: 99%

Churg-Strauss Syndrome in the Pediatric Age Group

El-Gamal

2008

World Allergy Organization Journal

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The rate of reporting of childhood Churg-Strauss syndrome (CSS) has increased lately because of either increased awareness to the disease or a real increase in incidence. It is defined as one of the antineutrophil cytoplasmic antibody-associated vasculi-tides, but the antineutrophil cytoplasmic antibody positivity is less reported in pediatric cases. The cause of CSS remains unknown. Several lines of evidence suggest genetic predisposition, which may entail inherited tendency to dysregulation of the cellular immune system. With the addition of leukotriene receptor antagonists to the treatment regimen of asthma, an association to CSS was presumed. However, the nature of this relationship remains to be elucidated. In addition, some environmental factors seem to provoke transient effects that resemble the disease. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. Three clinical stages have been described in the clinical evolution of CSS: prodromal phase involving allergic rhinitis and asthma (usually without family history of atopy), a second phase that involves peripheral eosinophilia and eosinophilic tissue infiltration, and the hallmark of the final phase is systemic vasculitis. Pulmonary disease is a central feature of pediatric CSS, but other manifestations include skin lesions, testicular pain, hypertension, seizures, and nephropathy. More subtle presentations in children include cervical lymphadenopathy, acute abdominal pain, deep venous thrombosis, oral ulceration, multiple colonic ulcers, chorea, bilateral optic neuropathy, and retinal artery occlusions. Churg-Strauss syndrome patients usually respond well to corticoster-oid therapy. Several trials reported additional benefit from cyclopho-sphamide, azathioprine, and methotrexate, whereas the therapeutic effects of etanercept, plasma exchange, and intravenous immunoglo-bulin therapy are controversial. The relapse rate is approximately 25% to 30%, but corticosteroids have significantly increased survival, which now approaches greater than 75% at 5 years. However, there is limited information about survival or long-term outcome in childhood.

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“…2,3 Occurrence in young children is very rare and is limited to case reports. 4,5 Gastrointestinal (GI) involvement, which occurs in one-third of patients with CSS, indicates a poor prognosis. 6 We report a young child with fatal CSS secondary to severe GI involvement.…”

Section: Introductionmentioning

confidence: 99%