case_images: PMC9978417_01
case_images: PMC9978417_02
case_images: Clinical course and serial changes of laboratory data after 1 year of age. WBC, white blood cell; CRP, C-reactive protein; SAA, serum amyloid A.
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case_images: PMC9978417_02_fped-11-1133097-g001.jpg
case_images: F1
case_images: After the resection of the mass, no clinical symptoms, including fever and swelling in her left thigh and knee, relapsed whereas leukocytosis (around 15.0 x 109/L) and mild serum CRP elevation (around 10.0 mg/L) had persisted (Figure 1). After 4 years of age, the patient irregularly complained of leg pain and headache (Figure 1). In addition to leukocytosis and serum CRP elevation, serum amyloid A level has elevated (25-231 mg/ml, normal range <8 mg/ml) (Figure 1).
case_images: Hearing threshold in the patient with Muckle-Wells syndrome. (A) Hearing threshold before canakinumab therapy, (B) serial changes of the pure tone average for the frequencies 500, 1,000 and 2,000 Hz (three-frequency pure tone average). The solid and dashed lines indicate the hearing thresholds of right and left ears, respectively.
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case_images: PMC9978417_02_fped-11-1133097-g002.jpg
case_images: F2
case_images: She was diagnosed with having bilateral sensorineural hearing loss (Figure 2A). She no longer complained of leg pain or headache and her hearing threshold gradually improved over 11 months of treatment (Figure 2B).
case_images: Monocytic cell death induced by lipopolysaccharide stimulation in the patient with muckle-wells syndrome. The figures of the upper left and right sides indicate the proportion of CD14+ cells, mainly monocytes, in peripheral blood mononuclear cells from the patient with Muckle-Wells syndrome without and with lipopolysaccharide stimulation, respectively. The figures of the lower left and right sides indicate the proportion of CD14+ cells, mainly monocytes, in peripheral blood mononuclear cells from an age- and sex- matched healthy child without and with lipopolysaccharide stimulation, respectively.
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case_images: PMC9978417_02_fped-11-1133097-g003.jpg
case_images: F3
case_images: Miami, FL, USA) and the proportion of monocytes was analyzed by flow cytometer (EC800 cell analyzer, Sony Corp., Tokyo, Japan). Monocytes (CD14+ cells) was decreased by 60.9% with LPS stimulation, which indicates a moderate cell death response compared to control (no decrease) (Figure 3).