High- vs Low-Dose Immunoglobulin Therapy in the Long-term Treatment of X-linked Agammaglobulinemia

Liese, Johannes G.; Wintergerst, Uwe; Tympner, K. D.; Belohradsky, Bernd H.

doi:10.1001/archpedi.1992.02160150075025

Cited by 56 publications

(52 citation statements)

References 17 publications

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“…This parallels the observations, albeit from a small number of patients, that Btk-deficient people suffer adverse effects after human adenovirus infection (25,27,28). B-cell-and Btk-deficient mice were highly susceptible to MAV-1 and succumbed with high viral loads and disseminated infection ( Fig.…”

Section: Discussionsupporting

confidence: 85%

Fatal Disseminated Mouse Adenovirus Type 1 Infection in Mice Lacking B Cells or Bruton's Tyrosine Kinase

Moore

McKissic

Brown

et al. 2004

J Virol

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Human adenoviruses are associated with self-limiting respiratory, conjunctival, and gastrointestinal disease. In immunocompromised people, human adenovirus infection can result in pneumonia, hepatitis, encephalitis, pancreatitis, gastroenteritis, or disseminated disease involving multiple organs (8, 43). Disseminated human adenovirus infection usually results in death. The incidence of disseminated human adenovirus disease is increasing with the increased number of immunocompromised children, and pediatric bone marrow transplant recipients are most at risk (5,12,13,16).Due to species specificity, human adenovirus pathogenesis is poorly understood. Study of mouse adenovirus type 1 (MAV-1) permits the analysis of a replicating adenovirus in vivo. The outcome of infection depends on the virus dose and mouse strain (15,26,33,40). In outbred and C57BL/6 (B6) mouse strains, MAV-1 infects cells of the monocyte-macrophage lineage and endothelial cells (10,20,33). The highest levels of virus are found in the spleen and brain (20,26,39). MAV-1-specific cytotoxic T cells peak at 10 days postinfection (d.p.i.) and then decline (18). T cells cause acute immunopathology and are required for survival 9 to 16 weeks postinfection in MAV-1-induced encephalomyelitis (33). Inbred mouse strains susceptible and resistant to MAV-1 are available, and sublethal irradiation of resistant mice renders them susceptible (40). Mice with a severe combined immunodeficiency (SCID) mutation are susceptible to MAV-1 (10, 35).Here we report findings that survival of acute MAV-1 infection is B-cell dependent and T-cell independent. We postulated that Bruton's tyrosine kinase (Btk) plays a role in protection from MAV-1. Loss of Btk in mice results in the X-linked immunodeficiency (Xid) phenotype (22). Btk Ϫ/Ϫ mice have reductions in serum immunoglobulin (natural antibody), conventional B cells, and peritoneal B-1 cells relative to control mice (22). Here we demonstrate that Btk is required for survival of MAV-1 infection. We present data indicating that early T-cell-independent antiviral immunoglobulin M (IgM) plays a pivotal role in protection against disseminated MAV-1 infection. MATERIALS AND METHODSVirus and mice. Wild-type MAV-1 was propagated and titrated in 3T6 cells (9). The mice used are indicated in Table 1 (11,14,23,24,(29)(30)(31). A ␤ bϪ/Ϫ and Jh mice were purchased from Taconic. C57BL/6NCr (B6) and BALB/CAnNCr mice were purchased from the National Cancer Institute. All other mice were purchased from Jackson Laboratory. The 50% lethal dose (LD 50 ) was determined as described previously (37). Sera were heat inactivated at 57°C for 45 min before passive transfer.Quantitation of virus. Organ homogenates were prepared as described previously (40) or in phosphate-buffered saline (PBS) with 1-mm glass beads (BioSpec Products, Bartlesville, Okla.) in 2 ml/well 96-well plates (Axygen, Union City, Calif.) with a Mini Beadbeater (BioSpec) and the manufacturer's protocol. Virus was titrated by plaque assay as described previously (9). The means of...

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Section: Discussionsupporting

confidence: 85%

Fatal Disseminated Mouse Adenovirus Type 1 Infection in Mice Lacking B Cells or Bruton's Tyrosine Kinase

Moore

McKissic

Brown

et al. 2004

J Virol

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“…The most common frequency of IV Ig dosing was every 4 weeks, consistent with the survey by Yong et al, which reported that 87 % of surveyed allergist/immunologists initially prescribed this frequency [18]. This study also showed that subjects with agammaglobulinemia were also were given more IV and SC Ig (566.7 mg/kg/mo and 615.4 mg/kg/mo) than subjects with CVID (534.4 mg/kg/ mo and 345.7 mg/kg/mo), in accordance with other work which showed that these subjects may need higher doses [4,15].…”

Section: Discussionsupporting

confidence: 84%

“…The prescribed doses, calculated with the number of grams of Ig ordered, the patient's weight, and the frequency of administration, were generally in accordance with the recommended dose of 400 to 600 mg/kg/month [8,[14][15][16][17]. The most common frequency of IV Ig dosing was every 4 weeks, consistent with the survey by Yong et al, which reported that 87 % of surveyed allergist/immunologists initially prescribed this frequency [18].…”

Section: Discussionsupporting

confidence: 61%

Home Care Use of Intravenous and Subcutaneous Immunoglobulin for Primary Immunodeficiency in the United States

Huang

Cunningham‐Rundles

2012

Journal of Allergy and Clinical Immunology

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Purpose-Utilization reports on immunoglobulin (Ig) use for immunodeficiency in the United States (U.S.) have focused on prescribing practices in hospitals. There have been no large-scale reports on Ig use for immune deficiency in the home. We investigated the use of Ig in 3,187 subjects diagnosed with primary immunodeficiency.Methods-Cross-sectional data on 4,580 subjects in the U.S. receiving Ig in 2011 was obtained from a major home care provider. Demographics, route, dose, and frequency of Ig use by subjects with ICD-9 coded primary immunodeficiencies were analyzed.Results-Of 4,580 subjects, 3,187 had ICD-9 codes suggesting primary immunodeficiencies; 1,939 (60.8 %) were females and 1,248 (39.2 %) were males, with age ranging from 0 to 95 years. The predominant diagnoses were: common variable immunodeficiency (279.06; n =1,764; 55.3 %), hypogammaglobulinemia (279.00; n=635; 19.9 %), unspecified immunity deficiency (279.3; n=286; 9 %), other selective Ig deficiencies (279.03; n=171; 5.4 %), and agammaglobulinemia (279.04; n=127; 4 %). 54 % of subjects received Ig by the subcutaneous (SC) route, and 46 % by intravenous (IV) route, with more SC use by older subjects. The mean dose prescribed was 483 mg/kg/month, but less Ig was ordered for subjects on SCIg (409 mg/kg/month), as compared to subjects on IVIg (568 mg/kg/month). A highly significant inverse correlation between increasing age and dosage of Ig ordered was found (P=<.0001).Conclusion-Analysis of home care use of Ig in primary immune deficiency revealed that the SC route was prescribed more than the IV route, especially for older patients. By either method of administration, less immunoglobulin was prescribed for older subjects.

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“…IgG (400 mg/kg for 3 to 4 weeks or more) became possible because of more-tolerated formulations such as low-pH preparations and preparations containing stabilizing additives (10,75,78). Levels of IgG in the serum of patients treated in this manner can be maintained in the lower normal range (38,75). High doses of IgG can also be administered subcutaneously with infusion pumps (22).…”

Section: The Scripps Research Institute La Jolla California 92037mentioning

confidence: 99%

“…While CVID patient observations can be difficult to interpret because accompanying T-cell defects can also be present, these tend to appear late in life (13). Encephalitides caused by other viral agents reported in patients with antibody deficiencies include those due to infections by adenoviruses (33,35,38) and measles virus (25,27).…”

Section: The Scripps Research Institute La Jolla California 92037mentioning

confidence: 99%

Role of Antibodies in Controlling Viral Disease: Lessons from Experiments of Nature and Gene Knockouts

Sanna¹,

Burton

2000

J Virol

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High- vs Low-Dose Immunoglobulin Therapy in the Long-term Treatment of X-linked Agammaglobulinemia

Cited by 56 publications

References 17 publications

Fatal Disseminated Mouse Adenovirus Type 1 Infection in Mice Lacking B Cells or Bruton's Tyrosine Kinase

Fatal Disseminated Mouse Adenovirus Type 1 Infection in Mice Lacking B Cells or Bruton's Tyrosine Kinase

Home Care Use of Intravenous and Subcutaneous Immunoglobulin for Primary Immunodeficiency in the United States

Role of Antibodies in Controlling Viral Disease: Lessons from Experiments of Nature and Gene Knockouts

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