Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=27826329
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 229.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 263.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\27826329
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Korean+J+Pediatr
2016 ; 59
(10
): 421-424
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Recurrent macrophage activation syndrome since toddler age in an adolescent boy
with HLA B27 positive juvenile ankylosing spondylitis
#MMPMID27826329
Park JH
; Seo YM
; Han SB
; Kim KH
; Rhim JW
; Chung NG
; Kim MS
; Kang JH
; Jeong DC
Korean J Pediatr
2016[Oct]; 59
(10
): 421-424
PMID27826329
show ga
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case
of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing
spondylitis (AS), who experienced episodes of recurrent MAS since he was a
toddler. A 16-year-old boy was admitted because of remittent fever with
pancytopenia and splenomegaly after surgical intervention for an intractable
perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis
(HLH) 4 different times, which was well controlled with intravenous
immunoglobulin and steroids since the age of 3. We were unable to identify the
cause for the HLH. He remained symptom-free until the development of back pain
and right ankle joint pain with swelling at 15 years of age. He was diagnosed
with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom
aggravation despite taking naproxen and methotrexate, and the symptoms improved
with etanercept. On admission, his laboratory data showed leukopenia with high
ferritin and triglyceride levels. Bone marrow biopsy examination showed
histiocytic hyperplasia with hemophagocytosis. There was no evidence of
infection. He received naproxen alone, and his symptoms and laboratory data
improved without any other immunomodulatory medications. Genetic study revealed
no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune
disease should have been considered as the cause of recurrent MAS in the young
patient once primary HLH was excluded.