Manish Butte

Epidemiology, Comorbidities, and Outcomes of Posterior Reversible Encephalopathy Syndrome in Children in the United States.

Pediatr Neurol. 2019 Jul 22;:

Authors: Thavamani A, Umapathi KK, Puliyel M, Super D, Allareddy V, Ghori A

Abstract
BACKGROUND: Posterior reversible encephalopathy syndrome is an increasingly recognized entity with certain identified predisposing factors in children. However, the actual incidence, comorbidities, outcomes, and hospitalization charges among children (aged < 20 years) in the United States are largely unknown.
METHODS: We analyzed the Kids’ Inpatient Database for incidence of posterior reversible encephalopathy syndrome-related hospitalizations, associated diagnoses, in-hospital outcomes, and charges for children in the United States in 2016. We report demographics, risk factors, discharge status, mortality, length of stay, and hospitalization charges.
RESULTS: In 2016, 825 pediatric hospitalizations related to posterior reversible encephalopathy syndrome were captured in the Kids’ Inpatient Database. Hospital discharges including solid organ transplant, bone marrow transplant, hypertension, renal disorder, primary immunodeficiency, malignancy, sepsis, severe sepsis, systemic connective tissue disorder, blood transfusion, hypomagnesemia, and sickle cell anemia were queried for presence of posterior reversible encephalopathy syndrome. The majority of patients were discharged home. We found that posterior reversible encephalopathy syndrome-related hospitalizations were significantly associated with increased length of stay and hospitalization charges in 2016 (P < 0.001). A mortality rate of 3.2% was found in posterior reversible encephalopathy syndrome-related hospitalizations when compared with 0.4% in non-posterior reversible encephalopathy syndrome hospitalizations (P < 0.001).
CONCLUSION: Incidence of posterior reversible encephalopathy syndrome-related hospitalizations is 0.04%. Hypertension and presence of renal disorder are the most significant risk factors found to be associated with posterior reversible encephalopathy syndrome. Presence of posterior reversible encephalopathy syndrome was associated with a significant increase in hospitalization charges and increased length of stay.

PMID: 31481327 [PubMed – as supplied by publisher]

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A Novel Homozygous Mutation With Different Clinical Presentations in 2 IRAK-4-Deficient Siblings: First Case With Recurrent Salmonellosis and Non-Hodgkin Lymphoma.

J Investig Allergol Clin Immunol. 2018 Aug;28(4):271-273

Authors: Gokturk B, Casanova JL, Picard C, Cagdas Ayvaz D, Erman B, Tezcan I, Ozdemir H, Ozel A, Reisli I

PMID: 30073964 [PubMed – indexed for MEDLINE]

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Granuloma-like lesion at subcutaneous immunoglobulin site in a common variable immunodeficiency patient.

Immunotherapy. 2019 Sep 03;:

Authors: Gupta A, Wang B, Gupta S

Abstract
Immunoglobulin therapy is the main stay in the treatment of primary antibody deficiencies. Granulomatous lesions are common complication in patients with common variable immunodeficiency (CVID). We present the first case of cutaneous granuloma-like lesion at site of subcutaneous immunoglobulin injections in a patient with CVID. These lesions resolve overtime following switching treatment to intravenous immunoglobulin. Unlike granulomas associated with CVID, granulomatous lesion in this patient did not require any specific therapy, and resolved over a period of 4 weeks following switching subcutaneous immunoglobulin to intravenous immunoglobulin.

PMID: 31478429 [PubMed – as supplied by publisher]

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CTLA-4 Expression in CD4+ T Cells From Patients With LRBA Deficiency and Common Variable Immunodeficiency With No Known Monogenic Disease.

J Investig Allergol Clin Immunol. 2018 Dec;28(6):422-424

Authors: Azizi G, Jamee M, Yazdani R, Bagheri Y, Fayyaz F, Jadidi-Niaragh F, Abolhassani H, Aghamohammadi A

PMID: 30530390 [PubMed – indexed for MEDLINE]

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Disseminated Bacille Calmette-Guérin infection at a glance: a mini review of the literature.

Adv Respir Med. 2019;87(4):239-242

Authors: Hassanzad M, Valinejadi A, Darougar S, Hashemitari SK, Velayati AA

Abstract
INTRODUCTION: Immunodeficient children are at a high risk of disseminated Bacillus Calmette-Guérin [BCG] infection. We assessed the literature on clinical manifestations of BCGosis in children with specific primary immunodeficiencies.
MATERIAL AND METHODS: We conducted a systematic review of clinical practice articles by searching Medline, PubMed, Embase, Scopus, Web of Science and Google Scholar from their inception to date.
RESULTS: Thirty-seven articles were included regarding BCG vaccination and its dissemination in children with primary immunodeficiencies. Articles on dissemination after intravesicular BCG were excluded from the study.
CONCLUSIONS: Since disseminated BCG vaccination may be the first manifestation of a primary immunodeficiency disease, a comprehensive search for immunological defects in children developing these problems after BCG vaccination seems rational.

PMID: 31476012 [PubMed – in process]

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Tumor infiltrating M2 macrophages could predict biochemical recurrence of localized prostate cancer after radical prostatectomy.

Exp Cell Res. 2019 Aug 29;:111588

Authors: Zhang Q, Xia J, Wang Y, Zhang J, Ji C, Cong R, Wang Y, Song N

Abstract
Given the critical role of the tumor microenvironment in PCa progression, we aimed to assess the prognostic effect of tumor infiltrating M2 macrophages (TIMMs) on biochemical recurrence (BCR) in patients with localized prostate cancer (PCa) after radical prostatectomy. A total of 127 localized PCa patients from GSE116918, 268 patients from TCGA database and 77 patients from GSE70770 were enrolled in our study. TIMMs were evaluated by the CIBERSORT method. Patients with high TIMMs had a significantly poorer recurrence free survival (RFS) (P = 0.017, P = 0.0063 and P = 0.001) in the three sets. In the multivariate analysis, the presence of high TIMMs (HR = 3.026, P = 0.023; HR = 2.679, P = 0.017; HR = 2.648, P = 0.005) was identified as an independent prognostic factor for RFS in the three sets. Harrell’s Concordance index (C-index) increased in all three sets after combining TIMMs with traditional risk factors (PSA, clinical stage(T) and Gleason score). Gene Set Enrichment Analysis showed that T cell receptor signaling pathway, B cell receptor signaling pathway and primary immunodeficiency were significantly enriched in the low TIMMs group. TIMMs could serve as an independent prognostic factor for BCR in localized PCa patients after RP. Incorporation of TIMMs into traditional risk classification might further stratify patients with different prognosis.

PMID: 31473209 [PubMed – as supplied by publisher]

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Practical Approach to Genetic Testing for Primary Immunodeficiencies.

Ann Allergy Asthma Immunol. 2019 Aug 28;:

Authors: Chinen J, Lawrence M, Dorsey M, Kobrynski LJ

PMID: 31472268 [PubMed – as supplied by publisher]

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Familial hypogammaglobulinemia with high RTE and naïve T lymphocytes.

Inflamm Res. 2019 Aug 29;:

Authors: Piscianz E, Conversano E, Bianco AM, Faletra F, Tommasini A, Valencic E

Abstract
Most of primary immunodeficiencies with hypogammaglobulinemia are associated with reduced memory B cells. T cell development may be interesting as well, but increased recent thymic emigrants are rarely reported in these patients. We report the case of a family (mother and her two sons) diagnosed with common variable immunodeficiency 10 due to a mutation in the NFKB2 gene. Laboratory findings showed that all three patients presented hypogammaglobulinemia, reduced memory B cells and elevated naïve T lymphocytes and recent thymic emigrants. This feature, in the absence of glucocorticoid deficiency, may suggest a primary thymic dysfunction. Interestingly, the mother presented the worst immune phenotype, as regards both antibody production and NK function, indicating that immune function may deteriorate in the course of time. We conclude that close monitoring of immune functions may widen the knowledge on the CVID10 and improve the patients’ care.

PMID: 31468084 [PubMed – as supplied by publisher]

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Coxsackievirus Type B3 Is a Potent Oncolytic Virus against KRAS-Mutant Lung Adenocarcinoma.

Mol Ther Oncolytics. 2019 Sep 27;14:266-278

Authors: Deng H, Liu H, de Silva T, Xue Y, Mohamud Y, Ng CS, Qu J, Zhang J, Jia WWG, Lockwood WW, Luo H

Abstract
KRAS mutant (KRAS mut ) lung adenocarcinoma is a refractory cancer without available targeted therapy. The current study explored the possibility to develop coxsackievirus type B3 (CVB3) as an oncolytic agent for the treatment of KRAS mut lung adenocarcinoma. In cultured cells, we discovered that CVB3 selectively infects and lyses KRAS mut lung adenocarcinoma cells (A549, H2030, and H23), while sparing normal lung epithelial cells (primary, BEAS2B, HPL1D, and 1HAEo) and EGFR mut lung adenocarcinoma cells (HCC4006, PC9, H3255, and H1975). Using stable cells expressing a single driver mutation of either KRAS G12V or EGFR L858R in normal lung epithelial cells (HPL1D), we further showed that CVB3 specifically kills HPL1D-KRAS G12V cells with minimal harm to HPL1D-EGFR L858R and control cells. Mechanistically, we demonstrated that aberrant activation of extracellular signal-regulated kinase 1/2 (ERK1/2) and compromised type I interferon immune response in KRAS mut lung adenocarcinoma cells serve as key factors contributing to the sensitivity to CVB3-induced cytotoxicity. Lastly, we conducted in vivo xenograft studies using two immunocompromised mouse models. Our results revealed that intratumoral injection of CVB3 results in a marked tumor regression of KRAS mut lung adenocarcinoma in both non-obese diabetic (NOD) severe combined immunodeficiency (SCID) gamma (NSG) and NOD-SCID xenograft models. Together, our findings suggest that CVB3 is an excellent candidate to be further developed as a targeted therapy for KRAS mut lung adenocarcinoma.

PMID: 31463367 [PubMed]

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