Impact of IVIG vs. SCIG on IgG trough level and infection incidence in primary immunodeficiency diseases: A systematic review and meta-analysis of clinical studies.

Background: Monthly intravenous immunoglobulin (IVIG) and weekly subcutaneous immunoglobulin (SCIG) have been regarded as therapeutically equivalent treatments for primary immunodeficiency diseases (PIDD). Immunoglobulin G (IgG) trough level is used as a monitoring measure for infection prevention. Objective: A systematic review and meta-analysis were performed to elucidate the relationship between IgG dosing, trough IgG levels with overall infection incidence in patients with PIDD receiving IVIG and SCIG therapy. Methods: Medline, EMBASE, Cochrane, Central, and Scopus were searched for studies published from Jan 2010-June 2018, fulfilling the inclusion criteria. DerSimonian and Laird random-effects method were used to pool the difference of IgG trough levels. Random-effect meta-regression was used to evaluate infection incidence per 100 mg/dl IgG trough increase though IVIG and SCIG. Results: Out of 24 observational studies included, 11 compared IgG trough levels among SCIG and IVIG (mean difference: 73.4 mg/dl, 95% CI: 31.67-119.19 mg/dl, I2 = 45%, p = 0.05), favoring weekly SCIG. For every 100 mg/dl increase in the trough, a linear trend of decreased incidence rates of infection was identified in SCIG patients (p = 0.03), but no similar trend was identified in trough levels vs. infection rates for patients receiving IVIG (p = 0.67). Conclusion: In our study, weekly SCIG attained a higher trough level in comparison to monthly IVIG. Higher SCIG troughs were associated with lower infection rates, while IVIG troughs demonstrated no relationship

Barbarians at the British Museum: Anglo-Saxon Art, Race and Religion

A critical historiographical overview of art historical approaches to early medieval material culture, with a focus on the British Museum collections and their connections to religion

Autoimmune Lymphoproliferative Syndrome: A Rare Cause of Disappearing HDL Syndrome

The term disappearing HDL syndrome refers to development of severe high density lipoprotein cholesterol (HDL-C) deficiency in noncritically ill patients with previously normal HDL-C and triglyceride levels. Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of the immune system due to an inability to regulate lymphocyte homeostasis resulting in lymphadenopathy and hepatosplenomegaly. We describe a 17-year-old boy who was evaluated in the lipid clinic for history of undetectable or low HDL-C and low density lipoprotein cholesterol (LDL-C) levels. Past medical history was significant for ALPS IA diagnosed at 10 years of age when he presented with bilateral cervical adenopathy. He was known to have a missense mutation in one allele of the FAS protein extracellular domain consistent with ALPS type 1A. HDL-C and LDL-C levels had been undetectable on multiple occasions, though lipids had not been measured prior to the diagnosis of ALPS. He had been receiving sirolimus for immunosuppression. The HDL-C and LDL-C levels correlated with disease activity and improved to normal levels during times when the activity of ALPS was controlled. This case highlights the importance of considering ALPS as a cause of low HDL-C and LDL-C levels in a child with evidence of lymphoproliferation

Case Report Autoimmune Lymphoproliferative Syndrome: A Rare Cause of Disappearing HDL Syndrome

The term disappearing HDL syndrome refers to development of severe high density lipoprotein cholesterol (HDL-C) deficiency in noncritically ill patients with previously normal HDL-C and triglyceride levels. Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of the immune system due to an inability to regulate lymphocyte homeostasis resulting in lymphadenopathy and hepatosplenomegaly. We describe a 17-year-old boy who was evaluated in the lipid clinic for history of undetectable or low HDL-C and low density lipoprotein cholesterol (LDL-C) levels. Past medical history was significant for ALPS IA diagnosed at 10 years of age when he presented with bilateral cervical adenopathy. He was known to have a missense mutation in one allele of the FAS protein extracellular domain consistent with ALPS type 1A. HDL-C and LDL-C levels had been undetectable on multiple occasions, though lipids had not been measured prior to the diagnosis of ALPS. He had been receiving sirolimus for immunosuppression. The HDL-C and LDL-C levels correlated with disease activity and improved to normal levels during times when the activity of ALPS was controlled. This case highlights the importance of considering ALPS as a cause of low HDL-C and LDL-C levels in a child with evidence of lymphoproliferation

Impact of IVIG vs. SCIG on IgG trough level and infection rates in Primary Immunodeficiency diseases: A systematic review and meta-analysis of 1,426 patients

Rationale: IVIG (Intravenous Immunoglobulin) and SCIG (Subcutaneous Immunoglobulin) have been regarded as therapeutically equivalent treatments for primary immunodeficiency diseases (PIDDs). IgG trough level is used as a monitoring measure for infection prevention. A systematic review and meta-analysis was performed in patients with PIDD and relationship between IgG dosing, trough IgG levels with overall infection incidence were evaluated. Methods: Medline, EMBASE, Cochrane, Central and Scopus were searched for studies published from Jan 2000- June 2018, fulfilling the inclusion criteria. DerSimonian and Laird random-effect method was used to pool difference of IgG trough levels. Random-effect meta-regression was used to evaluate infection incidence per 100 mg/dl IgG trough increase through IVIG and SCIG. Results: Out of 24 observational studies included (1,426 patients), 11 compared IgG trough levels among SCIG and IVIG (mean difference: 73.4 mg/dl, 95% CI: 31.67-119.19 mg/dl, I2= 45%, p=0.05), favoring SCIG. For every 100 mg/dl increase in trough, a linear trend of decreased incidence rates of infection was identified in SCIG patients (p=0.03), but no similar trend was identified in trough levels vs. infection rates for patients receiving IVIG (p=0.67). Conclusions: SCIG is associated with a higher IgG trough level in comparison to IVIG. Higher SCIG troughs were associated with lower infection rates, while IVIG troughs demonstrated no relationship

Atypical Omenn Syndrome due to Adenosine Deaminase Deficiency

We present here a novel case of an atypical Omenn syndrome (OS) phenotype due to mutations in the ADA gene encoding adenosine deaminase. This case is noteworthy for a significant increase in circulating CD56brightCD16- cytokine-producing NK cells after treatment with steroids for skin rash