Abnormal technetium labelled white cell scan in the colitis of chronic granulomatous disease

Hoare, Simon; Walsh, J E; Eastham, Edmund J.; Abinum, M A; Cant, AJ

doi:10.1136/adc.77.1.50

Cited by 14 publications

(7 citation statements)

References 5 publications

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“…10 However, they were not seen in all patients but when present the accumulations of macrophages appeared to be both much more prominent and prolific than those seen in Crohn's disease. This feature, together with their pigment content and the lack of neutrophils makes them quite distinct from those observed in Crohn's disease.…”

Section: Discussionmentioning

confidence: 78%

Colitis in chronic granulomatous disease

Schäppi

Smith

Green

et al. 2001

Archives of Disease in Childhood

124

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Background-Involvement of the gut in chronic granulomatous disease (CGD) has been previously described and colitis highlighted. However, the nature and histopathology of the colitis are unclear and have been thought to be non-specific or similar to Crohn's disease. Methods-Seven patients with CGD, suffering from gastrointestinal symptoms were prospectively studied. Results-All patients had anaemia; other symptoms were failure to thrive (5/7) and diarrhoea (5/7). Most had microcytic anaemia (5/7), increased platelets (7/7), and increased erythrocyte sedimentation rate (6/6). Endoscopically there was a friable erythematous mucosa in 6/7. The histological features present in all patients consisted of a colitis with paucity of neutrophils, increased numbers of eosinophils, eosinophilic crypt abscesses, pigmented macrophages, and nuclear debris. In some granulomas were present (2/7). Conclusions-Colitis is a common cause of gastrointestinal symptoms in CGD and is caused by a non-infective inflammatory process. The histology has specific features, which are distinctive from those seen in Crohn's disease.

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Section: Discussionmentioning

confidence: 78%

Colitis in chronic granulomatous disease

Schäppi

Smith

Green

et al. 2001

Archives of Disease in Childhood

124

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“…62 Approximately 50% of patients, however, also develop a noninfectious chronic inflammatory bowel disease strikingly similar to Crohn's disease. [63][64][65][66][67][68][69][70][71][72][73] Although 1 study suggested more frequent gastrointestinal involvement in patients with X-linked disease, 73 another found equal rates in the X-linked and autosomal recessive groups. 74 The series to date suggest that the primary clinical manifestation of CGD enteritis is abdominal pain and that up to one-third of patients demonstrate bowel obstruction at some time.…”

Section: Chronic Granulomatous Diseasementioning

confidence: 99%

Phagocyte dysfunction and inflammatory bowel disease

Rahman

Marks

Hayee

et al. 2008

Inflammatory Bowel Diseases

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Inflammatory bowel diseases are common chronic inflammatory disorders. The majority are idiopathic and can be broadly divided into Crohn's disease and ulcerative colitis. Their cause is unknown, but most hypotheses focus on a primary role for T-cell dysfunction. Conversely, there is a collection of congenital disorders of phagocyte function that result not only in immunodeficiency but also in noninfectious inflammatory bowel disease. In all cases, the latter is strikingly reminiscent of the clinical and pathological features of Crohn's disease. This coincides with recent work demonstrating that despite previous emphasis on adaptive immune dysfunction, patients with Crohn's disease actually possess an unusually weak acute innate inflammatory response. This review consolidates the literature on inflammatory bowel disease in congenital immunodeficiencies and considers the role of phagocyte dysfunction in Crohn's disease. Concepts about pathogenesis and treatment that can be carried across these disorders are also discussed.

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“…Patients may also present with pneumonia, lung abscesses, osteomyelitis and a granulomatous colitis which is clinically and histologically indistinguishable from Crohn's disease. 3 The association of colitis with CGD might be expected to increase the likelihood of neutrophilic dermatoses such as pyoderma gangrenosum, Sweet's syndrome (acute, febrile neutrophilic dermatosis) and bowel-associated pustular dermatosis. However we have found only one report of a neutrophilic dermatosis associated with CGD: 4 in that case infantile Sweet's syndrome was thought to be the presenting manifestation of CGD.…”

Section: Reportmentioning

confidence: 99%

Chronic granulomatous disease and acute neutrophilic dermatosis

Lyon

Griffiths

1999

Clinical and Experimental Dermatology

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Chronic granulomatous disease is an inherited disorder characterized by defective oxidative killing by neutrophils and other phagocytes. This results in susceptibility to persistent and life-threatening infections. We describe a 25-year-old man with chronic granulomatous disease who presented with an acute, febrile neutrophilic dermatosis. This indicates that normal neutrophil intracellular killing mechanisms are not essential in the pathogenesis of neutrophilic dermatoses.

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Abnormal technetium labelled white cell scan in the colitis of chronic granulomatous disease

Cited by 14 publications

References 5 publications

Colitis in chronic granulomatous disease

Colitis in chronic granulomatous disease

Phagocyte dysfunction and inflammatory bowel disease

Chronic granulomatous disease and acute neutrophilic dermatosis

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