Case report: Single-stage facial reanimation with bilateral lengthening temporalis myoplasties for immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome related developmental facial palsy.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare primary immunodeficiency, typically associated with clinical features of intractable diarrhoea, type 1 diabetes mellitus and eczema. We present a case of IPEX syndrome referred to our regional facial palsy service for smile restoration surgery. The patient presented with dissatisfaction of facial appearance, including mask-like facies and no functional smile. Pre-operative electromyography confirmed normal temporalis muscle activation. Consequently, the patient was offered single-stage bilateral lengthening temporalis myoplasties. The patient reported improved satisfaction with facial appearance. Surgery resulted in good early resting and voluntary symmetry. Oral commissures were elevated at rest improving oral incompetence. This is the first description of facial animation surgery in the context of IPEX syndrome. With careful consideration and patient selection, successful surgical restoration of resting symmetry and dynamic commissural smile can be achieved in this complex cohort of patients.

Case report: postpartum cerebral venous thrombosis misdiagnosed as postdural puncture headache.

BACKGROUND: Cerebral venous thrombosis can be a fatal complication of the postpartum period. Pregnancy is known to be a risk factor for thromboembolism in itself. CASE PRESENTATION: A normal spontaneous vaginal delivery was planned for a 20-year-old primigravida patient with patient-controlled epidural analgesia. Next morning, the patient complained of an occipital headache. An epidural blood patch was performed for diagnostic and therapeutic purpose with 10 ml of autologous blood. That night, she had an episode of seizures. Endotracheal intubation was done to secure the airway. She was transferred to an intensive care unit. Brain CT angiography and MRI showed superior sagittal sinus thrombosis with acute infarct and mild subarachnoid haemorrhage. For cerebral venous thrombosis treatment, heparin was injected and for intracranial pressure control, a hypertonic solution was injected. Despite this medical treatment, intracranial pressure continued to rise. The next day, her mental state changed to stupor. Emergency decompressive craniectomy was performed. Her mental state improved rapidly after surgery. A week later, she was transferred to a general ward. Her health recovered and she was discharged. CONCLUSIONS: We experienced postpartum cerebral venous thrombosis misdiagnosed as postdural puncture headache. We hope that this case report would be helpful in situation which a postpartum young woman complains severe headache in spite of management for headache including autologous epidural blood patch.

Characterization of an H3N2 triple reassortant influenza virus with a mutation at the receptor binding domain (D190A) that occurred upon virus transmission from turkeys to pigs.

The hemagglutinin (HA) protein of influenza virus mediates essential viral functions including the binding to host receptor and virus entry. It also has the antigenic sites required for virus neutralization by host antibodies. Here, we characterized an H3N2 triple reassortant (TR) influenza virus (A/turkey/Ohio/313053/04) with a mutation at the receptor binding domain (Asp190Ala) that occurred upon virus transmission from turkeys to pigs in an experimental infection study. The mutant virus replicated less efficiently than the parental virus in human, pig and turkey primary tracheal/bronchial epithelial cells, with more than 3-log10 difference in virus titer at 72 hours post infection. In addition, the mutant virus demonstrated lower binding efficiency to plasma membrane preparations from all three cell types compared to the parental virus. Antisera raised against the parental virus reacted equally to both homologous and heterlogous viruses, however, antisera raised against the mutant virus showed 4-8 folds lower reactivity to the parental virus.

Species and age related differences in the type and distribution of influenza virus receptors in different tissues of chickens, ducks and turkeys.

We undertook one of the most detailed studies on the distribution of alpha2,3 sialic acid (SA)-galactose (gal) (avian type) and alpha2,6SA-gal (human type) receptors on different tissues of chickens, ducks and turkeys of varying age groups. On the tracheal epithelium, all 3 bird species expressed strong positive staining (80-90%) for alpha2,3SA-gal receptors in the 3 different age groups. In addition, a lesser amount of alpha2,6SA-gal receptors (30-90%) were observed with slight differences in distribution with age and species. The epithelium of the small and large intestine of turkeys and ducks showed negligible staining for alpha2,6SA-gal receptors whereas the large intestine consistently showed 40-70% positive staining for alpha2,3SA-gal receptors. In contrast, a greater amount of staining for alpha2,3SA-gal (50-80%) and alpha2,6SA-gal (20-50%) receptors were observed along the epithelium of small and large intestine of chickens. Kidney and esophagus sections from the 3 bird species also expressed both avian and human type receptors. In other tissues examined, brain, breast muscles, bursa, spleen, cecal tonsils and oviduct, human type receptors were absent. Though different viral and receptor components may play roles in successful viral replication and transmission, understanding the receptor types and distribution in different tissues of domestic birds might be good initial tool to understand host factors that promote successful influenza viral infection.

Prevention of macrophage-related inflammatory diseases by allergina.

The oriental herbal combination allergina has been shown to inhibit allergic inflammation. In the present study, we demonstrate that the oral administration of allergina markedly inhibits the progression of inflammatory diseases, such as graft-versus-host diseases (in the allogeneic bone marrow transplantation and the parent-into-F1 transplantation models), collagen-induced arthritis and sheep red blood cell-induced delayed type hypersensitivity. The immunosuppressive activity of allergina in vivo appears to be associated, at least in part, with the inhibition of tumor necrosis factor-alpha production. In conclusion, our results suggest that allergina could be useful as a immunosuppressive agent for the treatment of macrophage-related inflammatory disease.

An application of linear programming discriminant analysis to classifying and predicting the symptomatic status of HIV/AIDS patients.

This study presents an application of linear programming discriminant analysis (LPDA) to classify and to predict the symptomatic status of HIV/AIDS patients. We applied LPDA as well as several traditional discriminant analysis methods to the AIDS Cost and Services Utilization Survey data set in order to demonstrate the use of LPDA to classify the symptomatic status of HIV/AIDS patients. The potential benefit of LPDA in terms of the classification accuracy was also analyzed.