eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Bruton Agammaglobulinemia: Treatment & Medication

Author: Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Contributor Information and Disclosures

Updated: Sep 3, 2008

Treatment

Medical Care

Until gene therapy becomes developed,²⁰ the mainstay therapy for Bruton agammaglobulinemia, formally termed X-linked agammaglobulinemia (XLA), and other primary antibody deficiencies is IVIG administration, which has supplanted IMIG injections in most instances.²¹ SCIG administration is also possible and offers the advantage of providing IgG levels that are relatively constant compared with the peaks and troughs observed with monthly intravenous therapy.

Numerous studies have shown that IVIG and SCIG given in equal doses provide equal infection prevention in patients with primary antibody deficiency syndromes.²² A major advantage is that SCIG can be administered at home. However, subcutaneous administration causes frequent local discomfort in various sites in the abdomen, thighs, upper arms, and/or lateral hips. In addition, whether home health care is appropriate for each patient must be evaluated. Not only is compliance an issue, but the lack of close medical observation is also a concern because these patients no longer need to come to the hospital for monthly infusions.

FFP has been used in the past but has the obvious disadvantage of the potential transmission of infectious agents, both known and unknown, despite extensive screening in blood banks.
- IVIG doses are usually 400-600 mg/kg/mo or more. The administration interval is usually every 3-4 weeks, based on the average IgG half-life of 21-28 days. The dose and interval are chosen based on the clinical response. Maintaining a trough serum IgG level of approximately 500-800 mg/dL is necessary.
- Clinical situations in which higher IVIG doses are given include, but are not limited to, chronic pulmonary infection and chronic enteroviral infection. Therefore, patients with bronchiectasis may need higher doses (eg, 600 mg/kg).
- For SCIG administration, 14 days of 200 mg/kg body weight resulted in serum IgG levels of more than 7 g/L and was tolerated well in adult patients with XLA and CVID.^23,24
Antibiotics are frequently required to manage the infectious complications of antibody deficiencies. Obtain appropriate cultures to identify causative microorganisms and to establish sensitivities; these results allow for optimal antibiotic therapy.
- Because most infections are sinopulmonary and involve encapsulated bacterial agents, first-line oral antibiotics include amoxicillin, amoxicillin/clavulanate, and cefuroxime axetil. Intravenous ceftriaxone may be required for chronic pulmonary infection, acute severe pneumonia, or sepsis.
- As with other patient populations, the risk for penicillin-resistance among S pneumoniae is an increasing concern; ceftriaxone, cefotaxime, and vancomycin are used to treat penicillin-resistant organisms.
- Less frequent, but significant, infectious agents include Mycoplasma and Ureaplasma species; these organisms are best treated with clarithromycin, which is generally better tolerated than erythromycin in terms of adverse GI effects. Clarithromycin is more effective than azithromycin.
- Antibiotic therapy for antibody deficiencies is in the high end of the dose range for immunocompetent individuals, and the duration is the same or longer. Some clinicians advocate rotating the use of antibiotics in select patients with bronchiectasis and frequent exacerbations.
- Opportunistic organisms are uncommon in XLA, but the risk of infection is increased, particularly in the presence of chronic debilitating pulmonary disease or (more rarely) chronic colitis. Pneumocystis carinii and B cepacia can be etiologic agents in these settings. Trimethoprim-sulfamethoxazole is the first-line drug for both.
- Recently released antibiotics such as linezolid for penicillin-resistant pneumococci are presumably effective, although results in primary immunodeficiency diseases are not yet published.
Many infections require interventions in addition to antibiotics.
- Recurrent or chronic pulmonary infections require annual PFTs. Children older than 5 years should be able to undergo these tests.
- Bronchodilators, inhaled corticosteroids, and leukotriene modifiers are integral in the therapy of many patients.
- Sinusitis is typically chronic in older patients and requires therapy with nasal steroids, saline sprays, and surgical intervention in some cases.
- Chronic eczema is treated with moisturizing creams and topical steroids, as in immunocompetent patients. Uncontrolled atopic dermatitis is associated with a greater risk for superinfection than that of topical steroid use.
- Nutritional intervention or supplementation and the use of multivitamin and mineral preparations are usually unnecessary in XLA, although some patients with autoimmune colitis occasionally require such therapy. Determining the etiology of the diarrhea (often infectious) is more important.
- Liver function tests are recommended annually because autoimmune hepatitis and hepatitis C may progress subclinically.

Surgical Care

Patients with chronic sinusitis who may benefit from surgical drainage procedures usually require a consultation with an otolaryngologist, as do children with recurrent otitis media who may improve with the placement of tympanostomy tubes.
Surgical interventions for pulmonary infections include diagnostic and therapeutic thoracentesis, lung biopsy, and care for lung abscesses and bronchopleural fistulas.
GI disorders usually do not require surgical intervention and are managed by a gastroenterologist.

Consultations

A pulmonologist, allergist/immunologist, infectious disease specialist, gastroenterologist, and/or hematologist may be consulted to manage specific complications.

Pulmonologists are particularly valuable in evaluating radiological findings, assisting with bronchodilator therapy, and interpreting detailed PFT results.
Allergy/immunology specialists are trained in the diagnosis and management of primary immunodeficiency disorders and are particularly valuable in diagnosing XLA and guiding IVIG therapy.
Infectious disease specialists are often consulted to determine the infectious etiologies, and they can recommend first-line antibiotics.
Gastroenterologists are essential in the diagnosis and management of inflammatory bowel disease.
Hematologists and clinical immunologists must collaborate to treat autoimmune cytopenias because immunosuppressive therapies for these hematologic disorders further compromise immune function in patients with XLA.

Diet

Most children and adults with XLA should maintain a normal and nutritious diet.

Patients with inflammatory bowel disease may require a low-fat diet and vitamin supplementation.
Nutritional supplementation with products such as PediaSure, Ensure, or Vivonex is necessary for some patients with persistent malabsorption and malnutrition.

Activity

Encourage patients with XLA to exercise actively, attend school, and maintain employment. Discourage patients from smoking, exposing themselves to smoke, and using illegal drugs. Instruct them to avoid unnecessary exposure to infectious agents. However, patients may generally benefit from outdoor activities. Considering the relatively good prognosis of XLA, the physician should encourage patients with this immunodeficiency disease to have a positive mental attitude.

Medication

The overall consensus among clinical immunologists regarding replacement therapy with IVIG in patients with primary immune deficiencies is that an IVIG dose of 400-600 mg/kg/mo or a dose that maintains trough serum IgG levels greater than 500 mg/dL is desirable. The number and severity of infectious complications is inversely correlated with the dose of IVIG administered. A recent consensus statement suggests that maintaining trough IgG levels greater than 800 mg/dL prevents serious bacterial illness and enteroviral meningoencephalitis.²⁵

Patients with Bruton agammaglobulinemia, formally termed X-linked agammaglobulinemia (XLA), and meningoencephalitis require higher doses (1 g/kg) and, perhaps, intrathecal therapy. SCIG administration is also possible. The recommended dose is 100-200 mg/kg SC every week. The initial weekly SC dose can be calculated by multiplying the previous IVIG dose by 1.37 and then dividing that dose into weekly doses, based on the patient's previous IVIG treatment interval. For example, if IVIG dosage is 200 mg/kg every 3 weeks, multiply 200 mg/kg by 1.37 and then divide by 3 to get a calculated dose of 91 mg/kg SC every week. The calculated SCIG dose provides systemic exposure similar to that of the previous IVIG dose. SCIG dose should be initiated 1 week after the last IVIG dose. For SCIG administration, do not exceed 15 mL (3200 mg) per injection site, and the administration rate is not to exceed 20 mL/h per injection site.

Preinfusion (trough) serum IgG levels are measured every 3 months until a steady state is achieved and then every 6 months if the patient is stable. These measurements may be helpful in adjusting the dose of IVIG or SCIG to achieve adequate serum levels. For persons in whom the catabolism of infused IgG is high, more frequent (eg, every 2-3 wk) IV infusions of smaller doses may maintain the serum level within the reference range. The rate of elimination of IgG may be higher during a period of active infection. Therefore, serum IgG levels may need to be measured more frequently, doses may need to be increased, or shorter intervals may be required.

For replacement therapy in patients with primary immune deficiency, all brands of IVIG are probably equivalent, although viral inactivation processes (eg, solvent detergent vs pasteurization and liquid vs lyophilized) differ. The choice of brand may depend on the hospital or home care formulary and on local availability and cost. In addition, whether home SCIG administration is appropriate must be determined. In patients who have IV access problems or who develop adverse effects with IVIG administration (eg, headache, myalgias), SCIG is an alternative. Questions regarding compliance need to be answered. The requirement of weekly infusions and local reactions at the site of infusions are disadvantages.

In addition, contraindications include patients with thrombocytopenia or other bleeding disorders and patients who are receiving anticoagulant therapy. SCIG was shown to be equal in efficacy to the same dose administered IV. The dose, manufacturer, and lot number should be recorded for each infusion to facilitate review for adverse events or other consequences. Recording of all adverse effects that occur during the infusion is crucial.

Periodic liver and renal function testing, approximately 3-4 times yearly, is also recommended. The US Food and Drug Administration (FDA) advises that, in patients at risk for renal failure, the recommended doses should not be exceeded and that infusion rates and concentrations should be at the practicable minimum levels. Examples of patients at risk for renal failure include patients older than 65 years; patients who use nephrotoxic drugs; and patients with preexisting renal insufficiency, diabetes mellitus, volume depletion, sepsis, or paraproteinemia.

The initial treatment should be administered under the close supervision of experienced personnel. The risk of adverse reactions in the initial treatments is high, especially in patients with infections and in those in whom immune complexes form. In patients with active infection, infusion rates may need to be slower, and the dose may need to be halved (ie, to 200-300 mg/kg). The remaining half should be administered the next day to achieve a full dose. Treatment should not be discontinued. After normal serum IgG levels are achieved, adverse reactions are uncommon unless patients have active infections.

With the new generation of IVIG products, adverse effects are reduced. Adverse effects include tachycardia, chest tightness, back pain, arthralgia, myalgia, hypertension or hypotension, headache, pruritus, rash, and low-grade fever. More serious reactions include dyspnea, nausea, vomiting, circulatory collapse, and loss of consciousness. Patients with more profound immunodeficiency and patients with active infections have more severe reactions.

The activation of complement due to IgG aggregates in the IVIG and the formation of immune complexes are thought to be related to the adverse reactions. The formation of oligomeric or polymeric IgG complexes that interact with crystallizable fragment (Fc) receptors and that trigger the release of inflammatory mediators is a cause. Most adverse reactions are rate related. Slowing the infusion rate or discontinuing therapy until symptoms subside may diminish the reaction. Pretreatment with ibuprofen (5-10 mg/kg every 6-8 h), acetaminophen (15 mg/kg/dose; not to exceed 1000 mg/dose or 2.6 g/24 h if age <12 y), diphenhydramine (1 mg/kg/dose; not to exceed 50 mg/dose), and/or hydrocortisone (6 mg/kg/dose; not to exceed 100 mg/dose) 1 hour before the infusion may prevent adverse reactions. In some patients with a history of severe adverse effects, therapy with analgesics and antihistamines may be repeated.

Acute renal failure is a rare but significant complication of IVIG treatment. Reports suggest that IVIG products with sucrose as a stabilizer may be associated with a greater risk for this renal complication. Acute tubular necrosis, vacuolar degeneration, and osmotic nephrosis suggest osmotic injury to the proximal renal tubules. The infusion rate for sucrose-containing IVIG should not exceed 3 mg/kg/min based on the amount of sucrose. Risk factors for this adverse reaction include preexisting renal insufficiency, diabetes mellitus, dehydration, age older than 65 years, sepsis, paraproteinemia, and concomitant use of nephrotoxic agents. For patients at increased risk, monitoring the BUN and creatinine levels before starting the treatment and prior to each infusion is necessary. If the patient's renal function deteriorates, the treatment should be discontinued.

IgE antibodies to IgA have rarely been reported to cause severe transfusion reactions in patients with IgA deficiency. A few cases of true anaphylaxis have been reported in patients with selective IgA deficiency and CVID who developed IgE antibodies to IgA after treatment with Ig. However, this is rare in actual experience. In addition, this is not a problem in patients with XLA or in patients with SCID. Caution should be exercised in patients with IgA deficiency (<7 mg/dL) who need IVIG. (IgA levels can be low in patients with selective IgA deficiency, in patients with CVID, and in some patients with IgG-subclass deficiencies.) IVIG preparations with low concentrations of contaminating IgA are advised in these situations (see the Table below).

Table 1. Immune Globulin, Intravenous

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Table

Brand(Manufacturer)	Manufacturing Process	pH	Additives*	Parenteral Form and Final Concentrations	IgA Content mcg/mL
Carimune NF (ZLB Behring)	Kistler-Nitschmann fractionation, pH 4 incubation, nanofiltration	6.4-6.8	6% solution: 10% sucrose, <20 mg NaCl/g protein	Lyophilized powder 3, 6, 9, 12%	Trace
Flebogamma (Grifols USA)	Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization	5.1-6	Sucrose free, contains 5% D-sorbitol	Liquid 5%	<50
Gammagard Liquid 10% (Baxter Bioscience)	Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation	4.6-5.1	0.25 M glycine	Ready-for-use liquid 10%	37
Gammar-P IV (ZLB Behring)	Cohn-Oncley fraction II/III, ultrafiltration, pasteurization	6.4-7.2	5% solution: 5% sucrose, 3% albumin, 0.5% NaCl	Lyophilized powder 5%	<20
Gamunex (Talecris Biotherapeutics)	Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation	4-4.5	Contains no sugar, contains glycine	Liquid 10%	46
Iveegam EN (Baxter Bioscience)	Cohn-Oncley fraction II/III, ultrafiltration, pasteurization	6.4-7.2	5% solution: 5% glucose, 0.3% NaCl	Lyophilized powder 5%	<10
Polygam S/D Gammagard S/D (Baxter Bioscience for the American Red Cross)	Cohn-Oncley cold ethanol fractionation followed by ultracentrafiltration and ion exchange chromatography, solvent detergent treated	6.4-7.2	5% solution: 0.3% albumin, 2.25% glycine, 2% glucose	Lyophilized powder 5%, 10%	<1.6 (5% solution)
Octagam (Octapharma USA)	Cohn-Oncley fraction II/III, ultrafiltration, low pH incubation, S/D treatment pasteurization	5.1-6	10% maltose	Liquid 5%	200
Panglobulin (Swiss Red Cross for the American Red Cross)	Kistler-Nitschmann fractionation, pH 4 incubation, trace pepsin, nanofiltration	6.6	Per gram of IgG: 1.67 g sucrose,<20 mg NaCl	Lyophilized powder 3, 6, 9, 12%	720
Privigen (CSL Behring)	pH 4 incubation, octanoic acid fractionation, depth filtration, and virus filtration	4.6-5	10% solution; Preservative-free and sucrose- and maltose-free	Ready-to-use solution 10%	25

Brand(Manufacturer)	Manufacturing Process	pH	Additives*	Parenteral Form and Final Concentrations	IgA Content mcg/mL
Carimune NF (ZLB Behring)	Kistler-Nitschmann fractionation, pH 4 incubation, nanofiltration	6.4-6.8	6% solution: 10% sucrose, <20 mg NaCl/g protein	Lyophilized powder 3, 6, 9, 12%	Trace
Flebogamma (Grifols USA)	Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization	5.1-6	Sucrose free, contains 5% D-sorbitol	Liquid 5%	<50
Gammagard Liquid 10% (Baxter Bioscience)	Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation	4.6-5.1	0.25 M glycine	Ready-for-use liquid 10%	37
Gammar-P IV (ZLB Behring)	Cohn-Oncley fraction II/III, ultrafiltration, pasteurization	6.4-7.2	5% solution: 5% sucrose, 3% albumin, 0.5% NaCl	Lyophilized powder 5%	<20
Gamunex (Talecris Biotherapeutics)	Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation	4-4.5	Contains no sugar, contains glycine	Liquid 10%	46
Iveegam EN (Baxter Bioscience)	Cohn-Oncley fraction II/III, ultrafiltration, pasteurization	6.4-7.2	5% solution: 5% glucose, 0.3% NaCl	Lyophilized powder 5%	<10
Polygam S/D Gammagard S/D (Baxter Bioscience for the American Red Cross)	Cohn-Oncley cold ethanol fractionation followed by ultracentrafiltration and ion exchange chromatography, solvent detergent treated	6.4-7.2	5% solution: 0.3% albumin, 2.25% glycine, 2% glucose	Lyophilized powder 5%, 10%	<1.6 (5% solution)
Octagam (Octapharma USA)	Cohn-Oncley fraction II/III, ultrafiltration, low pH incubation, S/D treatment pasteurization	5.1-6	10% maltose	Liquid 5%	200
Panglobulin (Swiss Red Cross for the American Red Cross)	Kistler-Nitschmann fractionation, pH 4 incubation, trace pepsin, nanofiltration	6.6	Per gram of IgG: 1.67 g sucrose,<20 mg NaCl	Lyophilized powder 3, 6, 9, 12%	720
Privigen (CSL Behring)	pH 4 incubation, octanoic acid fractionation, depth filtration, and virus filtration	4.6-5	10% solution; Preservative-free and sucrose- and maltose-free	Ready-to-use solution 10%	25

*IVIG products containing sucrose are more often associated with renal dysfunction, acute renal failure, and osmotic nephrosis, particularly with preexisting risk factors (eg, history of renal insufficiency, diabetes mellitus, age >65 y, dehydration, sepsis, paraproteinemia, nephrotoxic drugs).

Contents of table are adapted from the following sources:

Manufacturers' literature.
Siegel J. The Product: All intravenous immunoglobulins are not equivalent. Pharmacotherapy. 2005; 25(11 Pt 2):78S-84S.
Shah S. Pharmacy consideration for the use of IGIV therapy. Am J Health-Syst Pharm. 2005; 62(Suppl 3):S5-11.

Table 2. Immune Globulin, Subcutaneous

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Table

Brand(Manufacturer)	Manufacturing Process	pH	Additives	Parenteral Form and Final Concentrations	IgA Content mcg/mL
Vivaglobin (ZLB Behring)	Cold ethanol fractionation, pasteurization	6.4-7.2	2.25% glycine, 0.3% NaCl	Liquid 16% (160 mg/mL)	<50 mcg/mL

Brand(Manufacturer)	Manufacturing Process	pH	Additives	Parenteral Form and Final Concentrations	IgA Content mcg/mL
Vivaglobin (ZLB Behring)	Cold ethanol fractionation, pasteurization	6.4-7.2	2.25% glycine, 0.3% NaCl	Liquid 16% (160 mg/mL)	<50 mcg/mL

Antibiotics

Precautions

Caution in renal failure and neutropenia; red man syndrome caused by too-rapid IV infusion (dose given over a few min) but rare when dose given as 2-h administration or as PO or IP; red man syndrome is not an allergic reaction

Bronchodilators

Inhaler bronchodilator therapy is the mainstay of pulmonary care in most patients with XLA. A combination of a beta2-agonist (eg, albuterol, salmeterol) with a high-potency steroid (eg, budesonide, fluticasone) is conventional care.

Inhalers are used to relieve bronchoconstriction and decrease the inflammatory response in the respiratory tree. Both pulmonary and nasal inhalers may be needed. Inhaler use is hampered in young children and in others who cannot understand the technique of administration and in older individuals who are unable to achieve forceful inhalation. Adding a spacer is customary to improve coordination in children. If patients cannot reliably use a metered-dose inhaler, a nebulizer may be an option. Steroid inhalation is followed by rinsing the mouth to prevent thrush.

Albuterol (Proventil, Ventolin)

Relaxes bronchial smooth muscle by action on beta2-receptors with little effect on cardiac muscle contractility. Is also available as a solution for nebulization. MDI delivers 90 mcg/actuation.

Adult

2 inhalations q4-6h; not to exceed 12 inhalations/d

Pediatric

MDI:
<12 years: 1-2 inhalations qid with tube spacer
>12 years: Administer as in adults
Nebulizer:
<5 years: Dilute 0.25-0.5 mL (1.25-2.5 mg) of 0.5% inhalation solution in 1-2.5 mL 0.9% NaCl and administer via nebulizers q4-6h
>5 years: Administer as in adults

Beta-adrenergic blockers antagonize effects; inhaled ipratropium may increase duration of bronchodilatation due to albuterol; cardiovascular effects may increase with MAOIs, inhaled anesthetics, tricyclic antidepressants, and sympathomimetic agents

These agents are used to prevent and decrease inflammatory reaction within airway.

Beclomethasone (Qvar)

Adult

2 inhalations (160 mcg) qd/bid for Qvar (80 mcg/actuation)

Pediatric

<6 years: Not established
6-12 years: 2 inhalations (80 mcg) qd/bid for Qvar (40 mcg/actuation)

Coadministration with ketoconazole may increase plasma levels but do not appear to be clinically significant

Documented hypersensitivity; bronchospasm; status asthmaticus; other types of acute episodes of asthma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Suppression of hypothalamic-pituitary-adrenocortical (HPA) axis, linear growth, or Cushing syndrome may occur; caution with untreated systemic infections, ocular herpes simplex infection, or respiratory tuberculosis; patient should rinse mouth after use to reduce likelihood of PO candidiasis; use with spacer

Fluticasone (Flovent HFA, Flovent Diskus)

Has extremely potent vasoconstrictive and anti-inflammatory activity. Has weak HPA-axis inhibitory potency when applied topically. Some patients may require higher doses. Various inhalant devices deliver different dosages per actuation. Flovent HFA delivers 44 mcg, 110 mcg, and 220 mcg per actuation, whereas Flovent Diskus is specially designed with blister pack containing 50 mcg as a powder for inhalation.

Adult

HFA: 2-6 inhalations/d (44 mcg/actuation) or 2 inhalations/d (110 mcg/actuation)
Diskus: 100 mcg inhaled bid initially; may increase dose depending if patient is dependent on PO or other inhaled corticosteroids; not to exceed 1000 mcg bid

Pediatric

HFA: 2-4 inhalations/d (44 mcg/actuation)
Diskus:
<4 years: Not established
4-11 years: 50-100 mcg inhaled bid
Adolescents: Administer as in adults

Drugs metabolized by CYP3A4 isoenzyme (eg, ketoconazole) may increase concentrations

Documented hypersensitivity; viral, fungal, and bacterial skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Suppression of HPA axis, linear growth, or Cushing syndrome may occur; caution with untreated systemic infections, ocular herpes simplex, or respiratory tuberculosis; patient should rinse mouth after use to reduce likelihood of PO candidiasis; use with spacer

Flunisolide (AeroBid, AeroSpan)

Has extremely potent vasoconstrictive and anti-inflammatory activity. Has weak HPA-axis inhibitory potency when applied topically. Some patients may require higher doses. AeroBid (flunisolide CFC) delivers about 250 mcg/actuation. AeroSpan (flunisolide HFA) delivers about 80 mcg/actuation.

Adult

AeroSpan (HFA): 2 inhalations (160 mcg/2 actuations) bid; not to exceed 4 inhalations bid
AeroBid (CFC): 2 inhalations (500 mcg/2 actuations) bid; not to exceed 4 inhalations bid (2 mg/d)

Pediatric

<6 years: Not established
HFA product:
6-11 years: 1 inhalation (80 mcg) bid; not to exceed 2 inhalations bid
>12 years: Administer as in adults
CFC product:
6-16 years: 2 inhalations (500 mcg/2 actuations) bid; not to exceed 1 mg/d

None reported

Documented hypersensitivity; viral, fungal, and bacterial skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Budesonide (Pulmicort Turbuhaler, Pulmicort Respules)

Inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease number and activity of inflammatory cells, decreasing airway hyperresponsiveness. Available in various inhaled products. Pulmicort Turbuhaler delivers a powder that is inhaled (200 mcg/actuation). Pulmicort Flexhaler delivers a powder for inhalation as either 90 mcg or 180 mcg per dose. Pulmicort Respules is an inhalation susp administered via nebulization (available in 2 strengths: 0.25 mg/2 mL, 0.5 mg/2 mL).

Adult

1-2 inhalation qd/bid (200 mcg/actuation)

Pediatric

Turbuhaler:
<6 years: Not established
6-18 years: 1-2 inhalations qd
<6 years: Not established
6-18 years: Administer as in adults
1 vial Pulmicort Respules (0.5 mg or 0.25 mg): Administer via nebulization qd or bid

None reported

Documented hypersensitivity; viral, fungal, and bacterial skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause HPA axis suppression, decreased linear growth, or Cushing syndrome; caution with untreated systemic infections, ocular herpes simplex, or respiratory tuberculosis; patient should rinse mouth after use to reduce likelihood of PO candidiasis; use with spacer; not for acute asthma

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References

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Keywords

Bruton agammaglobulinemia, Bruton's agammaglobulinemia, severe combined immunodeficiency, X-linked agammaglobulinemia, XLA, Bruton type agammaglobulinemia, X-linked hypogammaglobulinemia, X-linked infantile hypogammaglobulinemia, Bruton disease, Bruton's disease, congenital agammaglobulinemia, Glanzmann-Riniker syndrome, primary agammaglobulinemia, pneumonia, Streptococcus pneumoniae, Haemophilus influenzae, meningoencephalitis, enterovirus, bronchiectasis, inflammatory bowel disease, malnutrition, meningitis, osteomyelitis, sepsis, gastroenteritis, diarrhea, otitis media, sinusitis, Mycoplasma, Ureaplasma, Giardia, Campylobacter, bacteremia, reactive arthritis, poliomyelitis

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Contributor Information and Disclosures

Author

Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Terry Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

James M Oleske, MD, MPH, François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, New Jersey Medical School
James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society
Disclosure: "no financial interest" None None

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

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