11 research outputs found
Yersinia pseudotuberculosis infection in Kawasaki disease and its clinical characteristics
Pyogenic spondylitis and paravertebral abscess caused by Salmonella Saintpaul in an immunocompetent 13-year-old child: a case report
Abstract Background Salmonella spondylitis is an uncommon complication of Salmonella infection in immunocompetent children. To prevent treatment failure and neurological deficits, it needs prompt diagnosis and sufficient effort to identify the causative organism. There are some options to identify the causative organism such as Computed Tomography (CT) guided biopsy or surgical debridement, however when to perform these invasive interventions remains controversial. Case presentation A 13-year-old boy presented with occasional high fever and lower back pain. He was diagnosed with spondylitis of the L4–5 vertebral bodies and paravertebral abscess. Initial blood cultures were negative, therefore empirical antibiotic treatment was started. He responded well to conservative management, and was discharged after clinical improvement. However, he was re-hospitalized 2 weeks after discharge, and surgical debridement was performed which led to the detection of Salmonella Saintpaul as the causative pathogen. It was revealed that the possible source of infection was consumption of raw poultry eggs, or contact with poultry. Definitive antibiotic therapy was started. He was discharged with good recovery after a 6-week hospitalization. Conclusions This is the very first case report of pyogenic spondylitis caused by Salmonella Saintpaul. Salmonella should be considered as a causative pathogen of pyogenic spondylitis in immunocompetent children. Identifying the causative organism is essential to prevent treatment failure, and a high index of suspicion is needed for prompt diagnosis especially when blood cultures are negative. Invasive interventions such as CT-guided biopsy should be considered even if the clinical course seems to be uncomplicated
Comprehensive stratum corneum ceramide profiling reveals reduced acylceramides in ichthyosis patient with CERS3 mutations
The stratum corneum (SC) of the epidermis acts as a skin permeability barrier, and abnormalities in SC formation lead to several skin disorders. Lipids, especially the epidermis-specific ceramide classes omega-O-acylceramides (acylceramides) and protein-bound ceramides, are essential for skin barrier formation. Ceramide synthase 3 (CERS3) is involved in the synthesis of acylceramides and protein-bound ceramides, and CERS3 mutations cause autosomal recessive congenital ichthyosis. In the present study, we measured ceramide synthase activity and performed comprehensive SC ceramide profiling in an ichthyosis patient with compound heterozygous CERS3 mutations: nonsense mutation p.Arg75* and missense mutation p.Arg229His. The activity of p.Arg75* and p.Arg229His mutant CERS3 proteins was reduced to 4% and 56%, respectively, of the wild-type protein. In the patient's SC, acylceramide levels were greatly reduced, but the levels of protein-bound ceramides remained almost unchanged. Non-acylated ceramide levels were also affected in the patient; in particular, the levels of ceramides composed of sphingosine and non-hydroxy or alpha-hydroxy fatty acid were substantially higher than in healthy controls. These results suggest that a reduction in acylceramide levels alone leads to ichthyosis. Although protein-bound ceramides are synthesized from acylceramides, levels of acylceramides and protein-bound ceramides are not necessarily correlated
A Fatal Case of Congenital Langerhans Cell Histiocytosis with Disseminated Cutaneous Lesions in a Premature Neonate
Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. Observations. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure although no apparent pulmonary or bone lesions on X-rays were noted. Skin biopsy confirmed a diagnosis of LCH due to observation of CD1a+ Langerhans cells, which lacked expression of E-cadherin and CD56. The patient died 57 hours after birth. Conclusions. Based on this case and the literature survey, the outcome of premature babies with congenital cutaneous LCH lesions is noted to be unfavorable, with the majority of such cases suffering from multisystem disease
An infant with refractory cytomegalovirus-induced thrombocytopenia
The present case underscores the importance of considering the association of severe thrombocytopenia or immune thrombocytopenia with cytomegalovirus (CMV) infection because CMV-induced thrombocytopenia occasionally requires antiviral therapy