Decline of antibodies in XLA infant: when to start IVIG

Okocha, I.; Hanson, C. G.; Chinen, Javier; Shearer, William T.

doi:10.1111/j.1398-9995.2010.02481.x

Cited by 6 publications

(7 citation statements)

References 5 publications

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“…The observed variability of postnatal decline of maternal antibodies has been attributed to different factors, including the catabolism of antibodies and the expansion of the volume occupied by the plasma and extravascular proteins during the growth of infants, which would vary considerably from child to child (5). In our patient, as well as in the XLA patient reported by Okocha et al (3), the level of total IgG remained in the normal range for two months of age, although the level of specific antibodies of some preventable diseases dropped below protective levels, with the consequent risk of infection for the patient. The protective antibody concentrations are well established for a number of vaccine-preventable diseases, including tetanus, diphtheria, and pneumococcus (6).…”

Section: Clues To Management Of Neonatally Diagnosed Btk Deficiencysupporting

confidence: 46%

Clues to management of neonatally diagnosed BTK deficiency

Deyà‐Martínez

Esteve‐Solé

Giner

et al. 2016

Pediatric Allergy Immunology

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Section: Clues To Management Of Neonatally Diagnosed Btk Deficiencysupporting

confidence: 46%

Clues to management of neonatally diagnosed BTK deficiency

Deyà‐Martínez

Esteve‐Solé

Giner

et al. 2016

Pediatric Allergy Immunology

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“…Prognosis for survival is relatively favorable owing to immunoglobulin replacement therapy (intravenous immunoglobulin therapy, or IVIG) [3]. …”

Section: Introductionmentioning

confidence: 99%

Tubulointerstitial nephritis complicating IVIG therapy for X-linked agammaglobulinemia

et al. 2014

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BackgroundPatients with X-linked agammaglobulinemia (XLA) develop immune-complex induced diseases such as nephropathy only rarely, presumably because their immunoglobulin (Ig) G concentration is low. We encountered a patient with XLA who developed tubulointerstitial nephritis during treatment with intravenous immunoglobulin (IVIG).Case presentationA 20-year-old man was diagnosed with XLA 3 months after birth and subsequently received periodic γ-globulin replacement therapy. Renal dysfunction developed at 19 years of age in association with high urinary β2-microglobulin (MG) concentrations. A renal biopsy specimen showed dense CD3-positive lymphocytic infiltration in the tubulointerstitium and tubular atrophy, while no IgG4-bearing cell infiltration was found. Fibrosclerosis and crescent formation were evident in some glomeruli. Fluorescent antibody staining demonstrated deposition of IgG and complement component C3 in tubular basement membranes. After pulse steroid therapy was initiated, urinary β2-MG and serum creatinine concentrations improved.ConclusionNeither drug reactions nor collagen disease were likely causes of tubular interstitial disorder in this patient. Although BK virus was ruled out, IgG in the γ-globulin preparation might have reacted with a pathogen present in the patient to form low-molecular-weight immune complexes that were deposited in the tubular basement membrane.

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“…With the on-going consumption of maternal antibodies the clinical manifestation with the recurrent infections becomes more evident. Recently it has been shown, that in addition to serum IgG levels, measurement of specific post-vaccination antibody levels may prove helpful in deciding when to begin IgG replacement in XLA infants 6 . Typical infectious complications are caused by pathogenic encapsulated bacteria (Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis), but typically these patients also suffer from giardiasis (lambliasis) and meningoencephalitis caused by echoviruses.…”

Section: Discussionmentioning

confidence: 99%

X-linked agammaglobulinemia caused by new mutation in BTK gene: A case report

Havlíčeková¹,

Jeseňák²,

Freiberger³

et al. 2014

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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Aim. Primary immunodeficiencies (PID) are becoming a recognized public health problem worldwide. The most important subgroup of these disorders are the antibody deficiencies. X-linked agammaglobulinaemia was the first described entity of this group and is characterised by early onset of recurrent bacterial infections, profound deficiency of all immunoglobulin isotypes and markedly reduced number of peripheral B-lymphocytes. Case report. We report the case of a 10-year old boy with X-linked agammaglobulinaemia caused by a previously non-described mutation in BTK gene with typical clinical presentation but delayed diagnosis. Following diagnosis, substitution therapy with intravenous immunoglobulins was started and the clinical status of the patient improved. Conclusion. We reported a case of X-linked agammaglobulinaemia with delayed diagnosis despite the typical anamnestic signs for primary humoral immunodeficiency. The disease was caused by a previously non-reported mutation in the BTK gene. Measurement of serum immunoglobulins should be performed in all children with recurrent, complicated respiratory infections as a screening test for humoral immunodeficiencies.

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Decline of antibodies in XLA infant: when to start IVIG

Abstract: Summary Statement Maternal antibody levels in XLA neonates may predict when to start replacement IgG.

Cited by 6 publications

References 5 publications

Clues to management of neonatally diagnosed BTK deficiency

Clues to management of neonatally diagnosed BTK deficiency

Tubulointerstitial nephritis complicating IVIG therapy for X-linked agammaglobulinemia

X-linked agammaglobulinemia caused by new mutation in BTK gene: A case report

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