Fatal unexpected death due to X-linked lymphoproliferative disease

X-linked lymphoproliferative disease (XLP) is a rare immunodeficiency disease characterized by severe immune disorder and extreme vulnerability to Epstein-Barr virus (EBV) infections. Here we report a 14-month-old Chinese boy presenting with fulminant infectious mononucleosis (FIM) following EBV infection, and died of hepatic failure within one week of disease progression. Postmortem examination revealed icterus, ascites, extensive enlarged mesenteric lymph nodes and hepatosplenomegaly. Histopathological examination showed diffuse proliferation of cytotoxic T lymphoid cells and hemophagocytosis in multiple organs. The family history revealed his brother had died under similar circumstances at 5 five years of age. The cause of death of the boy was ascribed to XLP. To the best of our knowledge, there is few autopsy-confirmed XLP case in the forensic practice. The complicated manifestations and systemic pathological changes should be well recognized by clinicians and forensic pathologists.     

Comments on: Perioperative von Willebrand factor dynamics are associated with liver regeneration and predict outcome after liver resection

正Recentlythearticle"PerioperativevonWillebrandfactordynamics are associated with liver regeneration and predict outcome afterliver resection" was published in Hepatology[1].Prof.Starlinger et al. aimed to assess the association of von Willebrand factor (vWF) levels and clinical outcome in patients with liver cancers post-liverresection(LR).Basedonthemechanismthatplatelets accumulation in the liver may promote liver regeneration after partial LR in mice, they found the vWF-dependent pattern of platelets accumulationduringliverregenerationinpatientsaftersurgery.     

A Case Report: Organs From a Donor With Highly Virulent Zoonotic Outbreak Strain of Streptococcus agalactiae Serotype III,Multilocus Sequence Type 283 Infective Endocarditis Did Not Result in Transmission With Adequate Prophylactic Antibiotic Cover

In 2015, an outbreak involving a highly virulent zoonotic outbreak strain of Streptococcus agalactiae serotype III, multilocus sequence type 283 occurred in Singapore with increased neurologic complications, septic arthritis, and spinal infections in healthier patients. We report a case of a successful dual kidney transplant from a deceased donor with infective endocarditis and disseminated infection with the same strain of S agalactiae and we review the current literature.     

Alfavirus tropicales artritogénicos

Tropical alphaviruses have special tropism for bone and joint tissue. Patients can develop chronic rheumatic disorders similar to rheumatoid arthritis and ankylosing spondylitis. The prototype is Chikungunya virus, although other lesser known viruses in our environment such as Sindbis, Ross River, Mayaro, O’nyong nyong and Barmah Forest viruses have the potential to be sped through vectors and cause chronic rheumatic disease.International population movements have increased the numbers of patients diagnosed with these tropical viruses in areas in which they are not endemic. Since they can leave persistent symptoms and affect the quality of life of the patients, it is important that we be aware of them. Changes in ecosystems have favored the expansion of competent mosquitoes, making fears of local transmission in southern Europe a reality.The objective of this review is to provide a clinical approach to the different arthritogenic tropical alphaviruses, especially those in which chronic rheumatic disease is more frequent.     

Identification of the critical attribute(s) of EBV gp350 antigen required for elicitation of a neutralizing antibody response in vivo

Vaccine prophylaxis with EBV glycoprotein 350 (gp350) subunit plus adjuvant has been demonstrated clinically to protect individuals against infectious mononucleosis (IM), but the specifications of the antigen required to elicit this protection has remained largely theoretical. Previous studies have shown that antibodies to gp350 comprise the principle component of EBV-neutralizing sera. Further, a murine monoclonal antibody against gp350 (clone 72A1) is able to prevent infection by the virus both in vitro and in vivo. In the present study, we identify the 72A1 epitope on recombinant gp350 antigen as the site required for binding to CD21 on human B cells. We also identify the need for conformational-dependence of the antigen to generate EBV-neutralizing antibodies in vivo. Further, we have characterized the glycosylation status and antigenicity profiles of both native and denatured CHO-produced soluble gp350 as well as non-glycosylated protein produced in Escherichia coli. Collectively our in vitro and in vivo data demonstrate the requirement for a conformationally accessible 72A1 epitope on gp350 to elicit EBV-neutralizing responses, and establish this as a critical attribute of this vaccine antigen. These data provide direction for commercial vaccine development, as the absence of this epitope on either E. coli-expressed or denatured gp350, may limit production and purification options for the antigen.