Abstract:
AIMS:To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS). METHODS:Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000. RESULTS:Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 x 10(9)/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died. CONCLUSION:MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Sawhney S,Woo P,Murray KJdoi
10.1136/adc.85.5.421subject
Has Abstractpub_date
2001-11-01 00:00:00pages
421-6issue
5eissn
0003-9888issn
1468-2044journal_volume
85pub_type
杂志文章abstract::The urinary citrate/creatinine ratio was evaluated in 25 children with idiopathic calcium urolithiasis and 24 controls. The mean (SD) urinary citrate/creatinine ratio in controls and patients was 0.510 (0.205) and 0.181 (0.076), respectively, a statistically significant difference. In neither group was there a relatio...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.74.4.350
更新日期:1996-04-01 00:00:00
abstract::Attention deficit-hyperactivity disorder (ADHD) remains a controversial disorder, despite it now being a well validated clinical diagnosis. Ethical and legal issues are important in determining how doctors should behave in offering a diagnosis or treatment that may generate strong and unpredictable reactions from chil...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2004.064576
更新日期:2006-02-01 00:00:00
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.77.5.396
更新日期:1997-11-01 00:00:00
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.74.3.188
更新日期:1996-03-01 00:00:00
abstract::A previously described difference in newborn blood phenylalanine concentrations between those living in urban and non-urban areas in the south west of England has been confirmed and shown to be independent of the type of feed. Several factors including the place of abode, type of feed, birthweight, and the accuracy of...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.58.4.271
更新日期:1983-04-01 00:00:00
abstract:BACKGROUND:A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases. METHODS:We describe the long-term clinical course of 119 children diagnosed with these lesions ...
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.74.4.309
更新日期:1996-04-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.1.96
更新日期:1989-01-01 00:00:00
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.2010.205211
更新日期:2012-04-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.4.308
更新日期:1978-04-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.5.734
更新日期:1989-05-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.54.1.62
更新日期:1979-01-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.8.774
更新日期:1985-08-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
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更新日期:2018-09-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章,随机对照试验
doi:10.1136/adc.2007.132571
更新日期:2009-10-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.71.1.40
更新日期:1994-07-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.67.3.332
更新日期:1992-03-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:
更新日期:1982-01-01 00:00:00
abstract::The association between measurements of lateral ventricle area (determined by serial ultrasound scans) and outcome was studied in 70 preterm neonates of 33 weeks' gestation or less. The study group was subdivided into four groups according to cranial ultrasonographic findings at 2 weeks postnatal age: group A (n = 15)...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.10_spec_no.1033
更新日期:1990-10-01 00:00:00
abstract:OBJECTIVES:To assess the efficacy and safety of polyethylene glycol 3350 plus electrolytes (PEG+E) for the treatment of chronic constipation in children. DESIGN:Randomised, double blind, placebo controlled crossover trial, with two 2-week treatment periods separated by a 2-week placebo washout. SETTING:Six UK paediat...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究,随机对照试验
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更新日期:2007-11-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.50.4.311
更新日期:1975-04-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.63.7.821
更新日期:1988-07-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.8.735
更新日期:1984-08-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.7.1058
更新日期:1989-07-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.71.4.381
更新日期:1994-10-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
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更新日期:1988-07-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.3.234
更新日期:1994-03-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.73.2.100
更新日期:1995-08-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章
doi:10.1136/adc.2010.190330
更新日期:2010-12-01 00:00:00
abstract::We have studied 50 children with one parent with asthma at a mean age of 6.4 years by symptom questionnaire and performed allergy skin testing and measurement of bronchial responsiveness to methacholine in both parent and child in 29-32 cases. Ninety eight per cent of the parents were receiving medication for asthma. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.62.1.66
更新日期:1987-01-01 00:00:00