ABSTRACT
Chronic recurrent multifocal osteomyelitis [CRMO] is a rare, systemic, aseptic, inflammatory disorder that involves different sites. Pathogenesis of chronic recurrent multifocal osteomyelitis is currently unknown. To our knowledge, there are no reports of CRMO from Saudi Arabia. We describe the clinical and laboratory features and treatment of a cohort of children with CRMO. Retrospective, patients referred to pediatric rheumatology clinic at a tertiary care center in Riyadh, Saudi Arabia. The diagnosis of CRMO was based on evidence of recurrent osteomyelitis with radiographic evidence of chronic osteomyelitis involving at least two sites in the absence of infectious cause in a child less than 14 years old. Ten patients [9 female, 1 male] with CRMO; 2 patients presented in infancy. The referral diagnosis was inaccurate in all patients. All of them presented with pain and 8 of them had associated swelling and were found to have multifocal lesions. Imaging studies showed findings consistent with chronic osteomyelitis. Histopathological and microbiological examination confirmed the diagnosis in 9 patients. Cyclic pamidronate infusions induced good improvement in 6 patients. This report indicates that CRMO may be overlooked in our community. Early diagnosis and treatment are required to avoid potential complications.
ABSTRACT
Published data from Saudi Arabia regarding autoinflammatory diseases are scarce. In this study, we describe the clinical and laboratory features of autoinflammatory diseases in Saudi children. Restrospective, hospital-based study conducted from January 2010 until June 2010.Patients with autoinflammatory disease treated at the Pediatric Rheumatology Clinic at King Faisal Specialist Hospital and Research Center, Riyadh, over the past 10 years were included. Autoinflammatory diseases included the following: familial Mediterranean fever [FMF]; chronic recurrent multifocal osteomyelitis [CRMO]; early-onset sarcoidosis [EOS]; periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome [PFAPA]; chronic infantile neurologic cutaneous and articular syndrome [CINCA]; and Muckle-Wells syndrome [MWS]. Demographic characteristics, diagnosis, age at onset, disease duration, follow-up duration, clinical and laboratory variables, and outcome data were compiled. Gathered laboratory data were part of patients usual medical care. Thirty-four patients [females, 53%] with autoinflammatory diseases were included [mean age, 151 months]. Mean disease duration was 118 months; mean age at onset was 32 months; consanguinity was present in 40%. Patients were diagnosed as follows: FMF, 50%; CRMO, 23.5%; CINCA, 8.8%; EOS, 8.8%; MWS, 6%; and PFAPA, 2.9%. The referral diagnosis was inaccurate in all patients except for FMF patients. Gene study was informative in 9 of 14 FMF patients who had molecular analyses. None of our cohort had amyloidosis. All CRMO patients had a favorable response to treatment except 1 patient, who had refractory, progressive disease. All patients with EOS had multiorgan involvement, including uveitis. All CINCA patients had a favorable response to anakinra. Our report shows that autoinflammatory diseases other than FMF may be overlooked. Increased awareness among pediatricians about these conditions will help to provide better health care to patients in the form of early diagnosis and management
Subject(s)
Humans , Male , Female , Familial Mediterranean Fever/epidemiology , Child , Hereditary Autoinflammatory Diseases/diagnosisABSTRACT
Stem cells from umbilical cord blood [CB] have increasingly become a viable alternate source of progenitor cells for hematopoietic cell transplantation [HSCT] Cytomegalovirus [CMV] is thought to contribute significantly to HSCT morbidity and mortality. Retrospective case-control study in patients at tertiary care center. We determined the incidence, risk factors and outcomes for CMV infection and disease after unrelated cord blood transplantation [UCBT] in children. Between 2003 and 2007, 73 pediatric patients underwent UCBT and 68% of recipients were CMV seropositive. The overall incidence of CMV infection, early and late CMV infection was 58.9% [43/73], 62.8% [27/43], and 37.4% [1 6/43], respectively. In patients with early CMV infection, 6 of 27 [22%] patients progressed to develop CMV end-organ disease including pneumonitis and retinitis. High levels CMV antigenemia >70 infected cells by pp65 antigenemia assay + PMNs, P-.237 were associated with a higher risk of progression to CMV disease. The development of CMV infections was higher in CMV-seropositive recipients [P<.001] and in those who developed graft-versus-host-diseases [GVHD] [P<.001]. Other risk factors for CMV infection include the use of high-dose corticosteroids [P<.001] and older age of the recipient at the time of transplant [P<.002]. Late CMV infection was strongly associated with a previous history of early CMV infection [P<.001]. CMV infection is a significant complication in UCBT recipients in pediatric patients and is associated with an increase in transplant-related morbidity and mortality. Risk factors for CMV infections after UCBT include GVHD, use of corticosteroids, underlying diseases [hematologic malignancies] and older age. Late CMV infection was strongly associated with a previous history of CMV infection