A Prospective Study on Children with Initial Diagnosis of Transient Hypogammaglobulinemia of Infancy: Results from the Italian Primary Immunodeficiency Network

Moschese, Viviana; Graziani, S; Avanzini, Maria Antonietta; Carsetti, Rita; Marconi, Massimo; Rocca, Marianna La; Chini, L; Pignata, Claudio; Soresina, Annarosa; Consolini, Rita; Bossi, Grazia; Trizzino, Antonino; Martino, Silvana; Cardinale, Fabio; Bertolini, Patrizia; Marseglia, Gian Luigi; Zecca, Marco; Cesare, Silvia Di; Quinti, Isabella; Rondelli, Roberto; Pietrogrande, Maria Cristina; Rossi, Paolo; Plebani, Alessandro

doi:10.1177/039463200802100211

Cited by 64 publications

(91 citation statements)

References 25 publications

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“…In agreement with the literature (13,(15)(16), the main clinical feature in patients with THI were respiratory infections; however, all children had a benign clinical course. Five patients underwent IVIG because they had severe clinical symptoms in winter, a period of high morbidity.…”

Section: Discussionsupporting

confidence: 91%

Evolution of Hypogammaglobulinemia in Premature and Full-Term Infants

Ricci

Piccinno

Giannetti

et al. 2011

Int J Immunopathol Pharmacol

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There are few data in the literature reporting the evolution of hypogammaglobulinemia in premature and full-term infants during the first years of life. The aim of this study was to assess the clinical and immunological evolution of premature and full-term infants with hypogammaglobulinemia. We included 24 children (11 premature and 13 full-term infants), aged 0-36 months, with hypogammaglobulinemia. Fifteen (62.5%) children had an isolated reduction in IgG, 7 (29.2%) had a decrease in both IgG and IgA and 2 (8.3%) a reduction in IgG and IgM. Normalization of IgG serum levels occurred in the premature infants at a mean age of 7.2 months. FUll-term infants were divided into 3 groups based on age at normalization of IgG serum level: A) hypogammaglobulinemia with normalization within 12 months of life; B) with normalization within 36 months of life; C) normalization after 36 months. All the premature infants with hypogammaglobulinemia recovered, even though in the lower limits for age in the first years, while transient hypogammaglobulinemia observed in full-term infants has a different age of recovery. Immunoglobulins(Igs) G levels are physiologically lower in the first months of life, a condition that increases in premature infants in direct proportion to gestational age (1-2). In some infants, occasionally, adequate Ig synthesis may be abnormally delayed until 36 months of age, resulting in a prolonged reduction of Ig levels; this process is referred to as transient hypogammaglobulinemia of infancy (THI) (3).At present there are no clinical features or diagnostic tests that differentiate self-limiting hypogammaglobulinemia from permanent immune defects in young children. A definitive diagnosis of THI is only possible a posteriori, after normalization of IgG levels and the exclusion of other causes of hypogammaglobulinemia.The aim ofthis study is to appraise the clinical and immunological features of premature and full-term infants with a diagnosis of hypogammaglob ulinemi a and their subsequent evolution. MATERIALS AND METHODSThe study included 24 children (aged 0-36 months), consecutively referred to the Department of Pediatrics, Pediatrics Allergology and Immunology Center of Bologna University between January I, 2000 and December 31, 2008, with a diagnosis of hypogammaglobulinemia associated with other clinical symptoms. Hypogammaglobulinemia was considered if the infants showed values of one or more Ig isotypes below the "lower limit" «2 DS) of our pre-determined set of reference ranges per age (4).

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Section: Discussionsupporting

confidence: 91%

Evolution of Hypogammaglobulinemia in Premature and Full-Term Infants

Ricci

Piccinno

Giannetti

et al. 2011

Int J Immunopathol Pharmacol

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“…It is important to discriminate between infants who are likely to have early resolution of hypogammaglobulinemia and infants who are likely to have persistence because there is evidence that infants with prolonged unresolved hypogammaglobulinemia are more likely to have recurrent infection and autoimmune problems. [7] This study confirms an easily understood hypothesis: infants with mild or moderate decreases in initial aggregate immunoglobulin levels or IgG levels alone are more likely to outgrow their immunologic deficiency and to do so sooner when compared to infants with greater decreases in immunoglobulin levels. Not surprisingly, infants with higher initial aggregate immunoglobulin levels have a greater probability of prompt resolution.…”

Section: Discussionsupporting

confidence: 83%

“…[6] Without question, some Infants with hypogammaglobulinemia will not have resolution of their deficiency and those infants with persistent hypogammaglobulinemia may be at increased risk for recurrent infection and autoimmune disease. [7] At the time of initial recognition of hypogammaglobulinemia in an infant, since this may be physiologic, immunologists are often finding with clinically relevant questions regarding the likely duration of hypogammaglobulinemia. Previous studies have shown that males are more likely than females to have resolution of hypogammaglobulinemia during infancy, [8] however, other than this observation, there has been little information that would allow an informed prediction about the probable time to resolution to hypogammaglobulinemia.…”

Section: Introductionmentioning

confidence: 99%

Phenotypic Parameters Predict Time to Normalization in Infants with Hypogammaglobulinemia

Winkle

McGeady

2013

J Clin Immunol

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“…Bu olgularda, serum IgG düzeyi yaşa göre ortalamanın 2 standart sapma değerinin altındadır. Daha sonra Ig düzeyleri yükselir ve genelde yaşamın 30.-40. aylarında normal değere ulaşır (3,7) . Bazı olgularda bu süre 6 yaşına kadar da uzayabilir (6) .…”

Section: Introductionunclassified

Five years of experience in transient hypogammaglobulinemia of infancy

Karaman¹,

Gülez²,

Erdem³

et al. 2016

BUCH

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ÖZAmaç: Süt çocuğunun geçici hipogamaglobulinemisi (SÇGH), immunoglobulin sentezindeki gecikmeden kaynaklanan, yaşla birlikte tamamen düzelen geçici primer immün yetmezliktir. Bu çalışmada, hipogamaglobulinemi ön tanısı ile izlenen ve SÇGH tanısı alan olgular retrospektif olarak değerlendirildi. 'sı ( %3,1) her ikisini almıştı. Olgular düzelme yaş gruplarına göre gruplandırıldığında, enfeksiyon sıklığı, klinik prezentasyon, aşı yanıtı, İV gamaglobülin replasmanı ve antibiyotik profilaksisi açısından gruplar arasında istatistiksel anlamlı fark saptanmadı (p>0.05). Sonuç: SÇGH çoğunlukla üç yaş civarında kendiliğinden düzelir. Düzelme yaşı ile klinik prezantasyon, aşı yanıtları, IV gamaglobülin replasmanı ve antibiyotik profilaksisi arasında ilişkisi yoktur. Doğru tanı için olguların düzenli izlenmesi gerekir.Anahtar kelimeler: Geçici hipogamaglobulinemi, intravenöz immünoglobülin, sütçocuğu ABSTRACT Objective: Transient hypogammaglobulinemia of infancy (THI) is a transient primary immune deficiency stemming from delay in immunoglobulin synthesis which spontaneously, and completely resolves with age. In this study, cases which were followed with the initial diagnosis of hypogammaglobulinemia and received the diagnosis of THI were retrospectively evaluated. Methods: A total of 193 patients, who received the diagnosis of THI in the Department of Immunology of Dr. Behçet Uz Children's Hospital, and Pediatric Surgery between January 2011 and December 2015, were included in the study. Clinical, laboratory and demographic data, prophylactic treatments they received, their follow-up periods, and age of onset of disease resolution were retrospectively analyzed. Results: In the study, 70.5% of 193 cases were male. The age of onset of symptoms was 11.7±7.0 month, the age of hypogammaglobulinemia recovery was 30.6±10.5 month. The cases were most frequently admitted (40.9%) for the recurrent upper respiratory tract infection. Only one case had immunodeficiency in the family history. At the admission, the immunoglobulin levels were as follows; IgG: 420.2±110.6 mg/dL, IgA: 29.8±23.3 mg/dL, IgM: 71±3.1 mg/dL. In vaccine responses; anti tetanus 75.9% (60/79), anti HBs 91.6% (141/154) and anti hemophilus influenza type B was 86.4% (51/59) positive. Twenty five (13%) of the patients received only antibiotic prophylaxis, 19 of them (9.8%) received only IV gamma globulin replacement, and 6 of them ( 3.1%) received both. There was no statistically significant difference between age of recovery and infection frequency, clinical presentation, vaccination response, IV gamma globulin replacement and antibiotic prophylaxis (p>0.05). Conclusion: THI is usually recovers spontaneously around 3 years of age. There is no relationship between age of recovery and clinical presentation, vaccine responses, IV gamma globulin replacement and antibiotic prophylaxis. The cases must be monitored regularly for correct diagnosis.

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A Prospective Study on Children with Initial Diagnosis of Transient Hypogammaglobulinemia of Infancy: Results from the Italian Primary Immunodeficiency Network

Cited by 64 publications

References 25 publications

Evolution of Hypogammaglobulinemia in Premature and Full-Term Infants

Evolution of Hypogammaglobulinemia in Premature and Full-Term Infants

Phenotypic Parameters Predict Time to Normalization in Infants with Hypogammaglobulinemia

Five years of experience in transient hypogammaglobulinemia of infancy

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