We report a 6-year-old girl with atopic dermatitis who developed eczema coxsackium caused by coxsackievirus A6 (CVA6) . At the time of her initial diagnosis, based on her medical history and clinical ...symptoms, we considered her to have Kaposi's varicella-like rash (eczema herpeticum) caused by herpes simplex virus (HSV) and started her on acyclovir, but later found that her serum HSV antibody titer was negative and CVA6 antibody titer was positive on admission. Although eczema herpeticum often presents with corneal and periocular symptoms, no periocular skin rash was seen in this case. If the periocular rash is not severe, a viral infection other than HSV may be suspected, and searching for the causative virus, including CVA6, may help in the diagnosis.
X-linked lymphoproliferative disease type 1 (XLP1) is a rare monogenic immune dysregulation disorder caused by a deficiency of a signaling lymphocyte activation molecule-associated protein (SAP). ...While many patients with XLP1 present with fatal hemophagocytic lymphohistiocytosis upon Epstein Barr virus (EBV) infection, a small fraction present with limbic encephalitis in the absence of EBV infection. It is poorly understood why SAP deficiency may cause limbic encephalitis in XLP1.
A 12-year-old boy presented with seizures, changes in personality, memory loss, and cognitive deficits during treatment for interstitial pneumonia. A diagnosis of limbic encephalitis was made. Despite treatment against CD8+ T cell-mediated autoimmunity with intravenous methylprednisolone, dexamethasone, intravenous immunoglobulin, plasma exchange, cyclosporine, weekly etoposide, mycophenolate mofetil, and adalimumab, encephalitis progressed until the patient died after one month of treatment intitiation. Post-mortem genetic testing revealed a de novo SH2D1A truncating mutation. Tests for EBV infection were negative. Initial spinal fluid revealed markedly elevated protein levels, mild pleocytosis, and elevation of two chemokines (C-X-C motif chemokine ligand CXCL 10 and CXCL 13). Moreover, initial spinal fluid was tested positive for anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) autoantibody.
In XLP1-associated limbic encephalitis, anti-AMPAR autoantibody production by the dysregulated immune system due to SAP deficiency might be a pathogenic mechanism of central nervous system manifestations. In addition to the standard treatment for XLP1, targeted treatment against B-cell-mediated immunity might be indicated for patients with XLP1-associated limbic encephalitis.
The evidence that gene mutations in the polarity determinant Crumbs homologs-2 (CRB2) cause congenital nephrotic syndrome suggests the functional importance of this gene product in podocyte ...development. Because another isoform, CRB3, was reported to repress the mechanistic/mammalian target of the rapamycin complex 1 (mTORC1) pathway, we examined the role of CRB2 function in developing podocytes in relation to mTORC1. In HEK-293 and MDCK cells constitutively expressing CRB2, we found that the protein localized to the apicolateral side of the cell plasma membrane and that this plasma membrane assembly required N-glycosylation. Confocal microscopy of the neonate mouse kidney revealed that both the tyrosine-phosphorylated form and non-phosphorylated form of CRB2 commence at the S-shaped body stage at the apicolateral side of podocyte precursor cells and move to foot processes in a capillary tuft pattern. The pattern of phosphorylated mTOR in developing podocytes was similar to that of CRB2 tyrosine phosphorylation. Additionally, the lack of a tyrosine phosphorylation site on CRB2 led to the reduced sensitivity of mTORC1 activation in response to energy starvation. CRB2 may play an important role in the mechanistic pathway of developing podocytes through tyrosine phosphorylation by associating with mTORC1 activation.
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Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy in infants characterised by repetitive vomiting and diarrhoea occurring within one to four hours of ingesting ...the offending food.
Diagnosis of FPIES is difficult because patients present with non-specific symptoms such as vomiting and diarrhoea, and useful diagnostic biomarkers are lacking.
We report on three infants who had FPIES with a transient increase in serum thymus and activation regulated chemokine (TARC) levels one day after ingesting the offending food. Serum TARC levels were quantified by a chemiluminescent enzyme immunoassay using an HISCL
system (Sysmex, Hyogo, Japan). In two of these cases, no increase in the serum TARC level was observed following the oral food challenge (OFC), which yielded a negative result. We suggest that a transient elevation in serum TARC levels one day after ingesting an offending food may be a useful diagnostic marker in some cases of FPIES. This article is protected by copyright. All rights reserved.
Making Sense of Japanese Grammar explains in a lively and highly informative manner basic principles that underlie a wide range of phenomena in Japanese. Students--irrespective of proficiency level ...and linguistic training--will find clarification on matters of grammar that often seem idiosyncratic and Japanese-specific, such as avoiding the use of certain pronouns, employing the same word order for questions, hidden subjects, polite and direct forms. Organized for easy access and readability, Making Sense of Japanese Grammar consists of short units, each focused on explaining a distinct problem and illustrated with a wealth of examples. To further enhance their usefulness, the units are cross-referenced and contain brief comprehension exercises to test and apply newly acquired knowledge. A glossary and keys to the exercises are at the back of the book. This volume may be used as a supplementary classroom reading or a helpful reference for students of all levels. Both students and instructors, even those trained in linguistics, will find its accessible explanations of grammatical concepts helpful. Grounded in sound scholarship and extensive teaching experience, Making Sense of Japanese Grammar brings a fresh and liberating perspective to the study of Japanese.
An oral food challenge test (OFC) is recommended in treating children with food allergies.We studied how to safely consume allergic foods at home.Eight children were hospitalized between January 2017 ...and February 2019 following an anaphylactic shock. The foods that they consumed were in lesser quantity than those that were approved by taking the previous OFC.It was observed that anaphylaxis occurred in 7 children due to the consumption of cow's milk and in one child who had eaten an egg.The triggering factors were considered to be lack of continuous intake, insolubilization by flour to make it hypoallergenic, physical condition at the time of intake and exercise after intake. Half of the children did not like the indicated foods and did not consume them as directed. Regular follow-up is necessary to ensure continued safe intake.
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