This study demonstrated that partial dominant IFN-gammaR1 deficiency was the most common in Japanese patients who showed predisposition to curable BCG osteomyelitis.
Multifocal Recurrent Osteomyelitis and Hemophagocytic Lymphohistiocytosis in a Boy with Partial Dominant IFN-γR1 Deficiency: Case Report and Review of the Literature.
Interestingly, both patients displayed multifocal osteomyelitis, which is often seen in patients with Mendelian susceptibility to mycobacterial diseases with autosomal dominant partial IFN-γR1 deficiency.
A maternal history of multifocal Mycobacterium kansasii osteomyelitis and cutaneous M avium complex led to genetic confirmation of IFN-γ-R1818del4 deletion (a 4 base pair deletion at nucleotide position 818) in both family members.
Here we report the case of a one-year-old girl with dominant partial IFNγR1 deficiency who suffered from lymphadenitis and multiple sites of osteomyelitis due to BCG infection.
Mutations in Pstpip2, LPIN2 and IL1RN have been identified in monogenic autoinflammatory bone disorders that have allowed more detailed dissection of the immunologic defects that can produce sterile osteomyelitis.
Interleukin-1 receptor antagonist (IL-1Ra) deficiency is a rare autoinflammatory disease involving neonatal onset of pustulosis, periostitis, and sterile osteomyelitis.
Past discoveries identified several single gene defects (LPIN2, Pstpip2 and IL1RN) that cause IL-1-mediated sterile multifocal osteomyelitis.Recently Lorden et al.
Results - The IL1RN*2/*2 genotype was associated (OR: 7; p < 0.001) with a higher risk of osteomyelitis and was also significantly associated with Staphylococcus aureus infection.
Older patients with a higher admission CRP value warrant an immediate magnetic resonance imaging, as they are likely to have osteomyelitis, which was associated with worse outcomes when compared with patients with isolated septic arthritis.
This study sought to evaluate the effectiveness of the inflammatory markers, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), in monitoring treatment of osteomyelitis in the diabetic foot.
Risk factors for return to the operating room include elevated initial erythrocyte sedimentation rate and C-reactive protein, infection with MRSA, and presence of osteomyelitis.
(2) Can a diagnostic algorithm be derived to guide interpretation of ESR and CRP to improve recognition of osteomyelitis in the setting of diabetic foot infection?
An erythrocyte sedimentation rate >36 mm/hour and C-reactive protein levels >60 mg/L were found in children with osteomyelitis or septic arthritis (p = 0.043 and <0.001, respectively).
Patients with osteomyelitis caused by pvl isolates had significantly higher mean values for erythrocyte sedimentation rate at admission (P = 0.005) and erythrocyte sedimentation rate maximum value (P = 0.002), maximum C-reactive protein (P = 0.04), and absolute neutrophil count at presentation (P = 0.04) compared with patients whose isolates were pvl(-).
Male gender (odds ratio [OR]: 1.31), smoking (OR: 1.38), history of amputation (OR: 1.47), history of osteomyelitis (OR: 1.94), peripheral arterial disease (OR: 2.35), retinopathy (OR: 1.32), International Working Group on the Diabetic Foot (IWGDF) grades 3 and 4 (OR: 1.7 and 2.5), Wagner grades 4 and 5 (OR: 4.3 and 6.4), gangrene/necrosis (OR: 9.9), osteomyelitis (OR: 4.5), neuroischaemic DFI (OR: 3.06), severe infection (OR: 3.12), length of hospitalization (standardized mean difference [SMD]: 0.7), leukocytosis (OR: 1.76), mean erythrocyte sedimentation rate (ESR) (SMD: 0.5), mean C-reactive protein (CRP) (SMD: 0.8), tissue culture positivity (OR: 1.61), and isolation of Gram-negative bacteria from tissue culture (OR: 1.5) were found as predictors of amputation in DFI.
Children with K. kingae SA were less likely to be younger than 3 months (0 vs. 42.3%; P < 0.001), had less anemia (21.4 vs. 50%; P = 0.010), lower C-reactive protein (3.8 vs. 8.9 mg/dL; P = 0.039), less associated osteomyelitis (0 vs. 26.9%; P = 0.033), shorter intravenous therapy (6 vs. 15 days; P < 0.001), and had a nonsignificant lower rate of sequelae (0 vs. 30%; P = 0.15) than children with SA caused by other bacteria.
Taken together, this study revealed that PGN-sa promotes osteoclast formation via TLR2-mediated activation of the NF-κB/NFATc1 signaling pathway, revealing a potential effect of PGN-sa on osteomyelitis.
Polymorphisms in TLR2 (Arg753Gln) and TLR4 (Asp299Gly, Thr399Ile) genes are associated with bacterial infections, we therefore studied these polymorphisms in osteomyelitis patients.