An 8-Year-Old Boy with Hypereosinophilic Syndrome
Hypereosinophilic syndrome (HES) is a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia and tissue eosinophilia, resulting in a wide variety of clinical manifestations. We present the case of an 8-year-old boy with HES. He complained of r...
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| Veröffentlicht in: | International archives of allergy and immunology 2011-06, Vol.155 (Suppl 1), p.117-122 |
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| creator | Hosoki, Koa Nagao, Mizuho Iguchi, Kosei Ihara, Toshiaki Yamada, Yoshiyuki Higashigawa, Masamune Kephart, Gail M. Kita, Hirohito Fujisawa, Takao |
| description | Hypereosinophilic syndrome (HES) is a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia and tissue eosinophilia, resulting in a wide variety of clinical manifestations. We present the case of an 8-year-old boy with HES. He complained of recurrent abdominal pain, general fatigue, and diarrhea. Laboratory data showed marked eosinophilia, elevated total IgE with positive specific IgE antibodies to common inhalant and food allergens, and elevated serum CCL17/TARC. A chest CT scan revealed central bronchiectasis, bronchial wall thickening, a mosaic attenuation pattern, and multiple small nodules in lung parenchyma; abdominal CT showed a thickened bladder wall. Gastrointestinal endoscopy revealed scarring in the gastric mucosa and mucosal erosion in the duodenum. Immunohistochemical examination demonstrated numerous eosinophil infiltrations with extensive extracellular eosinophil major basic protein deposition in the gastric mucosa. Only high-dose oral steroid was effective and cyclosporine appeared to have a steroid-sparing effect. HES is extraordinary rare in children and the long-term prognosis in pediatric HES is not well known. Comprehensive diagnostic procedures are vital for the early detection and management of complications in pediatric HES. |
| doi_str_mv | 10.1159/000327495 |
| format | Article |
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We present the case of an 8-year-old boy with HES. He complained of recurrent abdominal pain, general fatigue, and diarrhea. Laboratory data showed marked eosinophilia, elevated total IgE with positive specific IgE antibodies to common inhalant and food allergens, and elevated serum CCL17/TARC. A chest CT scan revealed central bronchiectasis, bronchial wall thickening, a mosaic attenuation pattern, and multiple small nodules in lung parenchyma; abdominal CT showed a thickened bladder wall. Gastrointestinal endoscopy revealed scarring in the gastric mucosa and mucosal erosion in the duodenum. Immunohistochemical examination demonstrated numerous eosinophil infiltrations with extensive extracellular eosinophil major basic protein deposition in the gastric mucosa. Only high-dose oral steroid was effective and cyclosporine appeared to have a steroid-sparing effect. HES is extraordinary rare in children and the long-term prognosis in pediatric HES is not well known. Comprehensive diagnostic procedures are vital for the early detection and management of complications in pediatric HES.</description><identifier>ISSN: 1018-2438</identifier><identifier>ISBN: 3805597657</identifier><identifier>ISBN: 9783805597654</identifier><identifier>EISSN: 1423-0097</identifier><identifier>EISBN: 3805597665</identifier><identifier>EISBN: 9783805597661</identifier><identifier>DOI: 10.1159/000327495</identifier><identifier>PMID: 21646806</identifier><language>eng</language><publisher>Basel, Switzerland: Karger</publisher><subject>Biological and medical sciences ; Blood diseases ; Bone Marrow - pathology ; Case management ; Cell Count ; Child ; Children & youth ; Cyclosporine - therapeutic use ; Cytokines - blood ; Eosinophils - pathology ; Fundamental and applied biological sciences. Psychology ; Fundamental immunology ; Gastrointestinal Tract - pathology ; Helicobacter Infections - complications ; Helicobacter Infections - drug therapy ; Hematologic and hematopoietic diseases ; Humans ; Hypereosinophilic Syndrome - blood ; Hypereosinophilic Syndrome - complications ; Hypereosinophilic Syndrome - diagnosis ; Hypereosinophilic Syndrome - drug therapy ; Hypereosinophilic Syndrome - pathology ; Immunopathology ; Lung - diagnostic imaging ; Male ; Medical diagnosis ; Medical sciences ; Novel Insights from Clinical Practice ; Other diseases. Hematologic involvement in other diseases ; Prednisolone - therapeutic use ; Radiography ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Steroids ; Urinary Bladder - diagnostic imaging</subject><ispartof>International archives of allergy and immunology, 2011-06, Vol.155 (Suppl 1), p.117-122</ispartof><rights>2011 S. Karger AG, Basel</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2011 S. Karger AG, Basel.</rights><rights>Copyright (c) 2011 S. Karger AG, Basel</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c394t-471a44b78fe07cd15f46ee8563ae89f63fabdd37031ec0c53c932e7804e045333</citedby><cites>FETCH-LOGICAL-c394t-471a44b78fe07cd15f46ee8563ae89f63fabdd37031ec0c53c932e7804e045333</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>309,310,314,776,780,785,786,2423,23909,23910,25118,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=24310989$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21646806$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hosoki, Koa</creatorcontrib><creatorcontrib>Nagao, Mizuho</creatorcontrib><creatorcontrib>Iguchi, Kosei</creatorcontrib><creatorcontrib>Ihara, Toshiaki</creatorcontrib><creatorcontrib>Yamada, Yoshiyuki</creatorcontrib><creatorcontrib>Higashigawa, Masamune</creatorcontrib><creatorcontrib>Kephart, Gail M.</creatorcontrib><creatorcontrib>Kita, Hirohito</creatorcontrib><creatorcontrib>Fujisawa, Takao</creatorcontrib><title>An 8-Year-Old Boy with Hypereosinophilic Syndrome</title><title>International archives of allergy and immunology</title><addtitle>Int Arch Allergy Immunol</addtitle><description>Hypereosinophilic syndrome (HES) is a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia and tissue eosinophilia, resulting in a wide variety of clinical manifestations. We present the case of an 8-year-old boy with HES. He complained of recurrent abdominal pain, general fatigue, and diarrhea. Laboratory data showed marked eosinophilia, elevated total IgE with positive specific IgE antibodies to common inhalant and food allergens, and elevated serum CCL17/TARC. A chest CT scan revealed central bronchiectasis, bronchial wall thickening, a mosaic attenuation pattern, and multiple small nodules in lung parenchyma; abdominal CT showed a thickened bladder wall. Gastrointestinal endoscopy revealed scarring in the gastric mucosa and mucosal erosion in the duodenum. Immunohistochemical examination demonstrated numerous eosinophil infiltrations with extensive extracellular eosinophil major basic protein deposition in the gastric mucosa. Only high-dose oral steroid was effective and cyclosporine appeared to have a steroid-sparing effect. HES is extraordinary rare in children and the long-term prognosis in pediatric HES is not well known. Comprehensive diagnostic procedures are vital for the early detection and management of complications in pediatric HES.</description><subject>Biological and medical sciences</subject><subject>Blood diseases</subject><subject>Bone Marrow - pathology</subject><subject>Case management</subject><subject>Cell Count</subject><subject>Child</subject><subject>Children & youth</subject><subject>Cyclosporine - therapeutic use</subject><subject>Cytokines - blood</subject><subject>Eosinophils - pathology</subject><subject>Fundamental and applied biological sciences. 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We present the case of an 8-year-old boy with HES. He complained of recurrent abdominal pain, general fatigue, and diarrhea. Laboratory data showed marked eosinophilia, elevated total IgE with positive specific IgE antibodies to common inhalant and food allergens, and elevated serum CCL17/TARC. A chest CT scan revealed central bronchiectasis, bronchial wall thickening, a mosaic attenuation pattern, and multiple small nodules in lung parenchyma; abdominal CT showed a thickened bladder wall. Gastrointestinal endoscopy revealed scarring in the gastric mucosa and mucosal erosion in the duodenum. Immunohistochemical examination demonstrated numerous eosinophil infiltrations with extensive extracellular eosinophil major basic protein deposition in the gastric mucosa. Only high-dose oral steroid was effective and cyclosporine appeared to have a steroid-sparing effect. HES is extraordinary rare in children and the long-term prognosis in pediatric HES is not well known. Comprehensive diagnostic procedures are vital for the early detection and management of complications in pediatric HES.</abstract><cop>Basel, Switzerland</cop><pub>Karger</pub><pmid>21646806</pmid><doi>10.1159/000327495</doi><tpages>6</tpages></addata></record> |
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| ispartof | International archives of allergy and immunology, 2011-06, Vol.155 (Suppl 1), p.117-122 |
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| source | Karger Journals; MEDLINE; Alma/SFX Local Collection |
| subjects | Biological and medical sciences Blood diseases Bone Marrow - pathology Case management Cell Count Child Children & youth Cyclosporine - therapeutic use Cytokines - blood Eosinophils - pathology Fundamental and applied biological sciences. Psychology Fundamental immunology Gastrointestinal Tract - pathology Helicobacter Infections - complications Helicobacter Infections - drug therapy Hematologic and hematopoietic diseases Humans Hypereosinophilic Syndrome - blood Hypereosinophilic Syndrome - complications Hypereosinophilic Syndrome - diagnosis Hypereosinophilic Syndrome - drug therapy Hypereosinophilic Syndrome - pathology Immunopathology Lung - diagnostic imaging Male Medical diagnosis Medical sciences Novel Insights from Clinical Practice Other diseases. Hematologic involvement in other diseases Prednisolone - therapeutic use Radiography Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Steroids Urinary Bladder - diagnostic imaging |
| title | An 8-Year-Old Boy with Hypereosinophilic Syndrome |
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