Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases
Clinical Rheumatology,  Clinical Article

Lin CI et al. – The results showed that Macrophage activation syndrome (MAS) could be fatal and complicate various pediatric autoimmune diseases. It generally has a good response to corticosteroids and intravenous immunoglobulin (IVIG). Prompt recognition and timely treatment can result in good outcomes.

Methods
  • The authors retrospectively reviewed the hospital’s medical records of 102 HLH/MAS patients from the past 20 years.
  • Demographics, clinical data, treatment, and outcomes were analyzed.
  • Among 102 patients, eight patients with underlying juvenile systemic lupus erythematous (two patients), mixed connective tissue disease (one patient), primary anti–phospholipid syndrome (one patient), and systemic type juvenile rheumatoid arthritis (sJRA; four patients) with 13 episodes of MAS were studied.

Results
  • Clinical manifestations of MAS included fever (100 %), hepatosplenomegaly (77 %), lymphadenopathy (38 %), skin rash (62 %), and neurological involvement (31 %).
  • Laboratory features included leukopenia (54 %), anemia (46 %), thrombocytopenia (77 %), jaundice (27 %), hypofibrinogenemia (40 %), decreased erythrocyte sedimentation rate (67 %), and elevated liver enzymes (77 %), lactate dehydrogenase (100 %), ferritin (88 %), triglycerides (91 %), C–reactive protein (85 %), plasma D–dimer (50 %), and hemophagocytosis in bone marrow (83 %).
  • The Epstein–Barr virus and adenovirus infection triggered MAS in two patients with sJRA.
  • Methylprednisolone pulse therapy was effective in two out of three patients, and high–dose intravenous immunoglobulin (IVIG) was effective in two out of six patients.
  • Patients with sJRA responded well to corticosteroids and cyclosporine.
  • Complications included opportunistic infection with Pneumocystis jiroveci, multiple organ failure, and intensive care unit myopathy.
  • The mortality rate was one out of eight (12.5 %).

Please login or register to follow this author.
Are you sure you want to Unfollow this Author?
► Click here to access PubMed, Publisher and related articles...
<< Previous Article | Next Article >>

Your Unread Messages in Nurse Practitioner

See All >> Messages include industry-sponsored communications and special communications from MDLinx

Most Popular Nurse Practitioner Articles

1 Vitamin D and sleep disorders Full Text Nature and Science of Sleep, August 13, 2014    Free full text    Clinical Article

2 Prazosin in the treatment of PTSD Journal of Psychiatric Practice, July 24, 2014    Review Article

3 Vitamin D and the risk of dementia and Alzheimer's disease Neurology®, August 27, 2014    Clinical Article

4 Nut consumption and the risk of coronary artery disease: A dose–response meta-analysis of 13 prospective studies Thrombosis Research, August 14, 2014    Clinical Article

5 Analysis of vitamin D status in major depression Journal of Psychiatric Practice, September 23, 2014    Clinical Article

6 Blood pressure in relation to coffee and caffeine consumption Current Hypertension Reports, August 8, 2014    Review Article

7 Dark chocolate acutely improves walking autonomy in patients with peripheral artery disease Full Text Journal of the American Heart Association, September 22, 2014    Free full text    Clinical Article

8 Nut consumption for vascular health and cognitive function Nutrition Research Reviews, July 11, 2014    Review Article

9 Carbohydrate beverages attenuate bone resorption markers in elite runners Metabolism, September 25, 2014    Clinical Article

10 A systematic review investigating interventions that can help reduce consumption of sugar-sweetened beverages in children leading to changes in body fatness Journal of Human Nutrition and Dietetics, September 26, 2014    Review Article

11 Nutrition for the prevention and treatment of chronic kidney disease in diabetes Canadian Journal of Diabetes, September 26, 2014    Review Article

12 Impact of dairy consumption on essential hypertension: A clinical study Full Text Nutrition Journal, August 29, 2014    Free full text    Clinical Article

13 Long-term high-dose vitamin D3 supplementation and blood pressure in healthy adults Hypertension, September 26, 2014    Clinical Article

14 Nutritional aspects of bone health Best Practice & Research Clinical Endocrinology & Metabolism, September 26, 2014    Clinical Article

15 Mediterranean diet and incidence of rheumatoid arthritis in women Arthritis Care & Research , September 24, 2014    Clinical Article

16 Validation of encephalapp, smartphone-based stroop test, for the diagnosis of covert hepatic encephalopathy Clinical Gastroenterology and Hepatology , September 26, 2014    Clinical Article

17 Meta-analysis of black tea consumption and breast cancer risk: update 2013 Nutrition and Cancer, August 6, 2014    Evidence Based Medicine

18 Coffee consumption and mortality from all causes, cardiovascular disease, and cancer: a dose-response meta-analysis American Journal of Epidemiology, August 26, 2014    Evidence Based Medicine    Review Article

19 Additional benefit of yoga to standard lifestyle modification on blood pressure in prehypertensive subjects: A randomized controlled study Hypertension Research, September 5, 2014    Clinical Article

20 A comparison of the education and work experiences of immigrant and the United States of America-trained nurses International Nursing Review, September 4, 2014    Clinical Article

Indexed Journals in Nurse Practitioner: Journal of the American Academy of Nurse Practitioners, Holistic Nursing Practicemore

Register now to view all the MDLinx contents (FREE)!

  • Stay current on the latest literature, research and clinical news
  • Get special communications and offers from MDLinx and our sponsors
  • Receive invitations to paid market research
View Samples and Register

Connect with us, stay current.

Receive the latest mecial news
updates for free via email

Sign up!

Subscribe to our free RSS feeds:

Get the latest news in your specialty automatically added to your newsreader or your personal My Yahoo!, Google, My MSN or My AOL page. Learn More

Close