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1.
Support Care Cancer ; 30(6): 5187-5200, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35257229

RESUMO

INTRODUCTION: In secondary immunodeficiency, immunoglobulin replacement therapy (IgRT) is recommended by guidelines (GL) for patients with IgG level < 4 g/l and more than 3 infections or a severe infection. IgRT may be appropriate if IgG level < 4 g/l and/or 1-3 less severe infections (≤ grade 2). METHODS: This was a retrospective sample analysis representative for practices and hospitals in Germany. The treatments and infection data were collected from patients with chronic lymphocytic leukemia (CLL) and multiple myeloma (MM). GL adherence (GLAD) was analyzed. RESULTS: Data from 1086 patients (CLL 490, MM 596) were collected from 86 centers. Of all patients, 34.8% developed IgG deficiency during therapy (CLL 35.5%; MM 34.2%). IgRT was given in 23.5% of CLL and 14.4% of MM patients. GLAD in hypogammaglobulinemia and indication to IgRT was 23.3% of 86 CLL and 22.1% of 77 MM patients. Without GLAD, the hazard ratio (HR) for any infection was 4.49 (95% CI 3.72-5.42; p < 0.001) and for severe infections (grade ≥ 3) 10.64 (95% CI 7.54-15.00; p < 0.001). Significant independent risk factors for infections were a higher Charlson Comorbidity Index, IgG deficiency, and 3rd + line treatment, as well as therapy with BTK inhibitors or chemotherapy in CLL. Multivariable analysis showed a significantly lower risk of severe infections after start of IgRT with a HR of 0.47 (95% CI 0.28-0.77; p = 0.003). CONCLUSIONS: Guideline adherence correlated with fewer and less severe infections but was low in patients with indication to IgRT. Risk factors for infection can be identified. Risk of severe infections was significantly lower in patients with IgRT.


Assuntos
Deficiência de IgG , Síndromes de Imunodeficiência , Leucemia Linfocítica Crônica de Células B , Mieloma Múltiplo , Fidelidade a Diretrizes , Humanos , Deficiência de IgG/complicações , Imunoglobulina G/uso terapêutico , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/tratamento farmacológico , Mieloma Múltiplo/complicações , Mieloma Múltiplo/terapia , Estudos Retrospectivos
2.
Pediatr Diabetes ; 22(5): 707-716, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33840156

RESUMO

BACKGROUND: Type 1 diabetes (T1D) may coexist with primary immunodeficiencies, indicating a shared genetic background. OBJECTIVE: To evaluate the prevalence and clinical characteristics of immunoglobulin deficiency (IgD) among children with T1D. METHODS: Serum samples and medical history questionnaires were obtained during routine visits from T1D patients aged 4-18 years. IgG, IgA, IgM, and IgE were measured by nephelometry and enzyme-linked immunosorbent assay (ELISA). IgG and IgM deficiency (IgGD, IgMD) were defined as IgG/IgM >2 standard deviations (SD) below age-adjusted mean. IgE deficiency was defined as IgE <2 kIU/L. IgA deficiency (IgAD) was defined as IgA >2 SD below age-adjusted mean irrespective of other immunoglobulin classes (absolute if <0.07 g/L, partial otherwise) and as selective IgAD when IgA >2 SD below age-adjusted mean with normal IgG and IgM (absolute if <0.07 g/L, partial otherwise). RESULTS: Among 395 patients (53.4% boys) with the median age of 11.2 (8.4-13.7) and diabetes duration 3.6 (1.1-6.0) years, 90 (22.8%) were found to have hypogammaglobulinemia. The IgGD and IgAD were the most common each in 40/395 (10.1%). Complex IgD was found in seven patients. Increased odds of infection-related hospitalization (compared to children without any IgD) was related to having any kind of IgD and IgAD; OR (95%CI) = 2.1 (1.2-3.7) and 3.7 (1.8-7.5), respectively. Furthermore, IgAD was associated with having a first-degree relative with T1D OR (95%CI) = 3.3 (1.4-7.6) and suffering from non-autoimmune comorbidities 3.3 (1.4-7.6), especially neurological disorders 3.5 (1.2-10.5). CONCLUSIONS: IgDs frequently coexist with T1D and may be associated with several autoimmune and nonimmune related disorders suggesting their common genetic background.


Assuntos
Diabetes Mellitus Tipo 1 , Síndromes de Imunodeficiência , Adolescente , Idade de Início , Criança , Estudos de Coortes , Diabetes Mellitus Tipo 1/classificação , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/epidemiologia , Diabetes Mellitus Tipo 1/patologia , Feminino , Humanos , Deficiência de IgG/complicações , Deficiência de IgG/epidemiologia , Deficiência de IgG/patologia , Imunoglobulina A/análise , Imunoglobulina A/sangue , Imunoglobulina G/análise , Imunoglobulina G/sangue , Síndromes de Imunodeficiência/classificação , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/patologia , Masculino , Fenótipo , Polônia/epidemiologia , Prevalência
3.
Adv Exp Med Biol ; 1289: 63-70, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-32519307

RESUMO

Recurrent respiratory tract infections (RTI) are one of the most common diseases in childhood. Frequent infections adversely affect the development of a child and may lead to suspicion of immunodeficiency. An additional allergy component is thought conducive to infection occurrence. In this study, we retrospectively assessed medical records of 524 children hospitalized with RTI. Patients were divided into two groups: RTI-alone (n = 394) and RTI with a history of allergy (n = 130). Overall, we found that a great majority of children with RTI had the immunoglobulin G within the normal limit, irrespective of allergy. A variable IgG deficiency, most often affecting IgG1, IgG3, and IgG4 subclass, was present in less than one-third of children. Proportions of specific IgG subclass deficiency, varying from about 10% to 40%, were similar in both RTI-alone and RTI-allergy groups. The only significant effect was a modestly smaller proportion of children with IgG4 deficiency in the RTI-allergy group when compared with the RTI-alone group. We also found that IgG deficiencies were age-dependent as their number significantly increased with children's age, irrespective of allergy. The results demonstrate a lack of distinct abnormalities in the immunoglobulin G profile which would be characteristic to a clinical history of allergy accompanying recurrent RTI in children. Thus, we conclude that the assessment of IgGs could hardly be of help in the differential diagnostics of the allergic background of RTI.


Assuntos
Hipersensibilidade , Deficiência de IgG , Infecções Respiratórias , Criança , Humanos , Hipersensibilidade/diagnóstico , Hipersensibilidade/epidemiologia , Deficiência de IgG/complicações , Deficiência de IgG/diagnóstico , Deficiência de IgG/epidemiologia , Imunoglobulina G , Recidiva , Infecções Respiratórias/epidemiologia , Estudos Retrospectivos
5.
Transpl Infect Dis ; 21(3): e13086, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30929295

RESUMO

BACKGROUND: Immunoglobulin (Ig) G2 subclass deficiency is known to be associated with recurrent bacterial respiratory infections caused by capsulated bacteria and is found mostly in pediatric patients. However, its impact after allogeneic hematopoietic stem cell transplantation (HSCT) has not been fully assessed. METHODS: We retrospectively evaluated the relationship between IgG2 subclass levels and bacterial pneumonia in 74 adult patients who survived longer than 2 years after allogeneic HSCT. RESULTS: During the evaluation period, nine patients developed bacterial pneumonia. The median IgG2 level was significantly lower in patients with an infectious episode than in those without (143 mg/dL vs 287 mg/dL; P < 0.01). In multivariate analysis, a history of rituximab therapy and cord blood as a stem cell source were significantly associated with decreased levels of both IgG2 and IgG2/IgG ratios (P < 0.05). CONCLUSIONS: Suboptimal serum IgG2 levels could increase susceptibility to late-onset bacterial pneumonia after allogeneic HSCT. IgG2 levels should be considered carefully, especially in patients receiving cord blood transplantation and/or rituximab treatment.


Assuntos
Anticorpos Antibacterianos/sangue , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Imunoglobulina G/sangue , Pneumonia Bacteriana/imunologia , Adulto , Idoso , Feminino , Doença Enxerto-Hospedeiro , Humanos , Deficiência de IgG/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rituximab/administração & dosagem , Transplante Homólogo/efeitos adversos , Adulto Jovem
6.
J Asthma ; 56(1): 79-83, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29420096

RESUMO

We report on five adult cases of the rare association of asthma with humoral immunodeficiency (huID). All patients had uncontrolled asthma related to recurrent respiratory infections. Asthma was diagnosed according to the Global Initiative for Asthma (GINA) guidelines, and bronchiectasis was ruled out by a CT chest scan. Two men (aged 28 and 60) presented with pollen allergies, chronic rhinosinusitis, and IgG deficiency (7.8 and 7.6 g/L, respectively). Both patients underwent surgery for nasal polyposis but relapsed with acute sinusitis and severe asthma exacerbations requiring treatment with oral corticosteroids and antibiotics. The immunoglobulin replacement therapy (IRT) partially relieved the asthma by reducing the number of exacerbations. A 55-year-old woman presented with nonallergic, corticosteroid-dependent asthma (20 mg/day prednisone) and IgG deficiency (5.72 g/L). IRT improved asthma control (fall in the Asthma Control Questionnaire (ACQ)-7 score from 3.5 to 1.7) and enabled withdrawal of the corticosteroids. In a 47-year-old woman with an IgG2 subclass deficiency (1.9 g/L) and asthma, IRT increased the degree of asthma control (fall in the ACQ-7 score from 2.8 to 1.1). A 75-year-old woman presented with corticosteroid-dependent asthma (40 mg/day prednisone), IgM and IgG deficiencies (0.28 g/L and 5.36 g/L, respectively), and recurrent respiratory, skin and urinary infections. Again, IRT improved asthma control (fall in the ACQ-7 score from 2.5 to 1.2), reduced the number of hospitalizations for asthma exacerbations, and enabled a 10-mg reduction in the daily dose of prednisone. These observations suggest that IRT may improve disease control in some patients with asthma and associated huID.


Assuntos
Asma/complicações , Asma/tratamento farmacológico , Deficiência de IgG/complicações , Deficiência de IgG/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Adulto , Idoso , Asma/fisiopatologia , Feminino , Humanos , Síndromes de Imunodeficiência/complicações , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença
7.
Adv Exp Med Biol ; 1108: 99-106, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30182338

RESUMO

Respiratory tract infections in children are one of the most common causes for medical consultations. When the infections are of recurring nature, they are a major reason for the diagnostics for primary immunodeficiency that is in about 65% of cases underlain by disorders of humoral immunity. This study seeks to retrospectively evaluate the history of recurrent respiratory tract infections in children with humoral disorders and the associations among deficiencies in the immune system components. We evaluated 394 children aged 3 months to 18 years. We found 49.5% (195 cases) of children with IgG deficiencies, all of whom had normal IgE levels. There were 8.4% (33 cases) of IgA deficiency, 7.4% (29 cases) of IgM insufficiency, and 4.1% (16 cases) of CD19+ cells deficiency. The elevated level of CD19+ cells was found in 27.7% (109 out of the 394 children). Immunoglobulin deficiencies often coexisted with a deficiency in another immunoglobulin class above outlined. There was an interdependence between IgA abnormality and IgG, IgG3, and IgG4 abnormalities as well as between IgM abnormality and IgG and IgG1 abnormalities. We conclude that respiratory tract infections in children are often underlain by a convergence of IgG with both IgA and IgM abnormal states. The physiopathological meaning of this convergence for the infection course and resulting functional respiratory changes remains elusive.


Assuntos
Deficiência de IgG/complicações , Imunidade Humoral , Infecções Respiratórias/complicações , Adolescente , Criança , Pré-Escolar , Humanos , Deficiência de IgG/imunologia , Imunoglobulina G , Lactente , Recidiva , Infecções Respiratórias/imunologia , Estudos Retrospectivos
8.
Harefuah ; 156(11): 705-709, 2017 Nov.
Artigo em Hebraico | MEDLINE | ID: mdl-29198088

RESUMO

INTRODUCTION: Bronchiectasis is characterized by an abnormal dilatation of the bronchi leading to a chronic inflammatory process, airway blockage and impaired clearance of secretions. The damage to the airways is usually progressive and is the result of several pathogenic processes. In the past, healing of infections (especially pulmonary tuberculosis) was the main cause of airway dilatation and progression of chronic inflammation. Today, congenital illnesses, anatomical defects and immune deficiency play an important role in the pathogenesis of bronchiectasis formation. The immunoglobulin repertoire is vital for effective host protection against a wide variety of pathogens. Primary antibody deficiency diseases are defects of the humoral arm of the immune system and involve an absence/reduced levels of one or more immunoglobulin classes/subclasses or defects of specific antibody formation. Immunoglobulin G (IGG) subclass deficiency can occur in a healthy person and could be without clinical significance. However, in recent years there is emerging evidence that in patients with recurrent infections, early diagnosis of antibody deficiency affects the prognosis and prevention of ongoing lung damage. The use of IVIG has contributed significantly to the survival rate in primary antibody deficiencies. There is limited literature on the treatment of IVIG for patients with IGG subclass deficiency. However, all studies presented so far demonstrated that immunoglobulin therapy reduced the rate of bacterial infections, days of antibiotic usage, hospital admissions and significantly increased patients' quality of life. Therefore, in the appropriate clinical setting, ie: a patient with bronchiectasis and recurrent infections, it is justified to test whether there are humoral immune defects such as IGG subclass deficiency. In a patient with proven deficiency, we should recommend to start IVIG treatment until clinical benefit is achieved.


Assuntos
Bronquiectasia/imunologia , Bronquiectasia/terapia , Deficiência de IgG/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Infecções Bacterianas , Bronquiectasia/etiologia , Humanos , Imunoglobulina G , Qualidade de Vida
9.
J Korean Med Sci ; 31(10): 1560-5, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27550483

RESUMO

Immunoglobulin G subclass deficiency (IgGSCD) is a relatively common primary immunodeficiency disease (PI) in adults. The biological significance of IgGSCD in patients with chronic airway diseases is controversial. We conducted a retrospective study to characterize the clinical features of IgGSCD in this population. This study examined the medical charts from 59 adult patients with IgGSCD who had bronchial asthma or chronic obstructive pulmonary disease (COPD) from January 2007 to December 2012. Subjects were classified according to the 10 warning signs developed by the Jeffrey Modell Foundation (JMF) and divided into two patient groups: group I (n = 17) met ≥ two JMF criteria, whereas group II (n = 42) met none. IgG3 deficiency was the most common subclass deficiency (88.1%), followed by IgG4 (15.3%). The most common infectious complication was pneumonia, followed by recurrent bronchitis, and rhinosinusitis. The numbers of infections, hospitalizations, and exacerbations of asthma or COPD per year were significantly higher in group I than in group II (P < 0.001, P = 0.012, and P < 0.001, respectively). The follow-up mean forced expiratory volume (FEV1) level in group I was significantly lower than it was at baseline despite treatment of asthma or COPD (P = 0.036). In conclusion, IgGSCD is an important PI in the subset of patients with chronic airway diseases who had recurrent upper and lower respiratory infections as they presented with exacerbation-prone phenotypes, decline in lung function, and subsequently poor prognosis.


Assuntos
Asma/diagnóstico , Deficiência de IgG/diagnóstico , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Adulto , Idoso , Asma/complicações , Feminino , Volume Expiratório Forçado , Humanos , Deficiência de IgG/complicações , Imunoglobulina G/classificação , Klebsiella/isolamento & purificação , Masculino , Pessoa de Meia-Idade , Moraxella catarrhalis/isolamento & purificação , Pseudomonas aeruginosa/isolamento & purificação , Doença Pulmonar Obstrutiva Crônica/complicações , Testes de Função Respiratória , Infecções Respiratórias/complicações , Infecções Respiratórias/microbiologia , Estudos Retrospectivos
10.
Can J Anaesth ; 61(5): 441-5, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24585233

RESUMO

PURPOSE: When exposed in the perioperative period to blood components containing immunoglobulin (Ig)A IgA-sensitized IgA-deficient patients are at an increased risk of transfusion-associated anaphylaxis. We present the case of an IgA-deficient patient whose candidacy for double-lung transplantation was under review in the preoperative period. CLINICAL FEATURES: A 49-yr-old patient with end-stage chronic obstructive lung disease secondary to deficiencies in IgA and IgG subclasses was being assessed for double-lung transplantation. Early recognition of the ramifications of perioperative transfusion prompted consultation with the transfusion medicine service. This in turn facilitated specialized laboratory testing and the coordinated provision of appropriate blood products for the unpredictable date of transplantation. The theoretical systemic risks of a non-IgA-deficient graft on the sensitized IgA-deficient host were considered. To affirm the patient's candidacy for transplantation, he was ultimately challenged preoperatively with IgA-containing products in a controlled intensive-care setting. CONCLUSION: Through a multidisciplinary approach [corrected], a successful transplantation outcome was achieved in an IgA-deficient patient undergoing major surgery. Strategies to mitigate risk include the procurement and transfusion of IgA-deficient components, which may be challenging or untenable in emergent perioperative settings.


Assuntos
Deficiência de IgA/complicações , Transplante de Pulmão/métodos , Assistência Perioperatória/métodos , Doença Pulmonar Obstrutiva Crônica/cirurgia , Humanos , Deficiência de IgG/complicações , Imunoglobulina A/administração & dosagem , Comunicação Interdisciplinar , Masculino , Pessoa de Meia-Idade , Doença Pulmonar Obstrutiva Crônica/etiologia , Resultado do Tratamento
12.
J Pak Med Assoc ; 64(8): 963-5, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25252530

RESUMO

Recurrent meningitis is an uncommon life-threatening condition. Here, the case of a 6-year-old boy is reported who had two episodes of meningitis with an IgG3 subclass deficiency. The boy had aseptic meningitis at the age of 3 years, followed by bacterial meningitis at the age of 4 years. Primary immunoglobulin deficiencies are a group of disorders associated with an increased incidence and/or severity of infection. Recurrent infections, sinusitis, bronchitis, and pneumonia are the most frequently observed illnesses in patients with IgG subclass deficiencies, of which an IgG3 subclass deficiency is the most common, especially in adults. Although cases of recurrent viral or bacterial meningitis have been reported, herein a patient is presented with recurrence of aseptic and bacterial meningitis 1 year after the initial episode. Some researchers recommend that all children with episodes of recurrent meningitis should be screened for primary immunoglobulin or complement deficiencies.


Assuntos
Deficiência de IgG/complicações , Meningite/etiologia , Antibacterianos/uso terapêutico , Criança , Diagnóstico Diferencial , Humanos , Masculino , Recidiva
13.
Rev Laryngol Otol Rhinol (Bord) ; 135(3): 151-5, 2014.
Artigo em Francês | MEDLINE | ID: mdl-26521359

RESUMO

PURPOSE: To study the effect of the introduction of a substitution by intravenous Immunoglobulins (Ig IV) at patients with immunoglobulins G (IgG) subclasses deficiency and nasal polyposis. MATERIAL AND METHODS: Prospective study concerning five patients with IgG subclasses deficiency and nasal polyposis treated by Ig IV. Rhinologic, otologic and pulmonary symptoms, exacerbations of nasal polyposis, chronic otitis and asthma as well as the number of antibiotics and corticoids treatments were counted during the Ig IV substitution. OBJECTIVES: To study the association between IgIV substitution and the number of exacerbations of nasal polyposis, chronic otitis, asthma and the number of antibiotics and corticoids treatments in patients with IgG subclasses deficiency and nasal polyposis. RESULTS: Five patients with a IgG subclass deficiency and nasal polyposis were substituted. The number of antibiotics and corticoids cures increased at one patient and remained stable at four others. The number of sinus, ear and lung infections as well as the global rhinologic score of symptoms and the endoscopic stage of the nasal polyposis remained stable. In the absence of efficiency of the treatment, this one was interrupted at the end of 6 months for patients n° 1 and n° 3, 24 months for patient n° 4 and 42 months for patient n° 5. CONCLUSION: The current study failed to highlight clinical improvement in patients wih IgG subclasses deficiency and nasal polyposis treated by Ig IV. A previous study had not allowed to find a link between IgG subclasses deficiency and severity of nasal polyposis, what seems to be confirmed by the absence of improvement brought during the substitution of this deficit in the current study.


Assuntos
Deficiência de IgG/complicações , Deficiência de IgG/tratamento farmacológico , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Pólipos Nasais/complicações , Sinusite/complicações , Feminino , Humanos , Deficiência de IgG/sangue , Imunoglobulina G/classificação , Masculino , Pessoa de Meia-Idade , Pólipos Nasais/terapia , Estudos Prospectivos , Fatores de Risco , Sinusite/terapia , Falha de Tratamento , Resultado do Tratamento
14.
Pediatr Neonatol ; 64(1): 38-45, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36089538

RESUMO

BACKGROUND: IgG subclass deficiency is a laboratory diagnosis and becomes important with recurrent infections. This study aimed to examine the demographic, clinical, and laboratory results of pediatric cases with IgG subclass deficiency and to improve the understanding of the clinical significance of IgG subclass deficiency. METHODS: In this study, the clinical and laboratory features of 111 pediatric patients, with at least one whose serum IgG subclasses was measured as lower than 2 standard deviation of healthy aged-matched control values, were evaluated. The clinical and laboratory features of the cases with isolated IgG subclass deficiency (Group 1) and those with low serum levels of any of IgG, IgA, and IgM in addition to the IgG subclass deficiency (Group 2) were compared. RESULTS: A total of 55 (49.54%) and 56 (50.45%) patients were included in Groups 1 and 2, respectively. Among our studied cases, 20 (18.1%) had a history of hospitalization in the neonatal period, 61 (54.95%) had at least one hospitalization due to infection, and 55 (49.54%) had a history of recurrent infection. The frequencies of these three conditions were statistically significantly higher in Group 2 (p < 0.05). The frequencies of infections in the last year in Groups 1 and 2 were 4.4 ± 1.2 and 5.4 ± 1.9, respectively (p < 0.05). As a result of recurrent infections, 43.24% (n = 48) of our patients received antibiotic prophylaxis, and 21.62% (n = 24) had immunoglobulin replacement therapy. Furthermore, the numbers of patients who needed these treatments were higher in Group 2 (p < 0.05). CONCLUSION: In cases with IgG subclass deficiencies, concomitant main-group immunoglobulin deficiencies may increase the number and severity of infections, leading to hospitalizations, antibiotic prophylaxis, and immunoglobulin therapy. More attention should be paid to cases of immunoglobulin main-group deficiencies in the follow-up of these cases.


Assuntos
Deficiência de IgG , Reinfecção , Recém-Nascido , Criança , Humanos , Idoso , Reinfecção/complicações , Deficiência de IgG/diagnóstico , Deficiência de IgG/complicações , Imunoglobulina G , Antibioticoprofilaxia
16.
Pediatr Nephrol ; 27(3): 489-92, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22207346

RESUMO

BACKGROUND: There have been few reports on children who developed common variable immunodeficiency (CVID) in association with immunoglobulin A (IgA) and IgG2 deficiencies and systemic lupus erythematosus (SLE). CASE-DIAGNOSIS/TREATMENT: Our patient experienced nephrotic syndrome and acute respiratory distress syndrome (ARDS) caused by influenza A/H1N1 virus infection at 5 years of age. A diagnosis of IgA and IgG2 deficiency and SLE was made on the basis of severe proteinuria, hematuria, hypocomplementemia, high anti-DNA antibody and antinuclear antibody (ANA) titers, and malar rash. However, these clinical signs and symptoms and laboratory features disappeared after the administration of methylprednisolone pulse therapy and prednisolone. For the 5 years following the initial treatment for SLE, the patient experienced a number of infections and had a low serum total IgG level; she was eventually diagnosed with CVID. The administration of intravenous immunoglobulin (IVIG) was required to prevent subsequent infections, and no relapse of SLE was observed. CONCLUSION: We report the development of CVID in an IgA- and IgG2-deficient patient with SLE on the basis of multiple episodes of infection. To prevent the development of CVID in IgA- and IgG2-deficient patients with SLE, it is important to prevent immune dysregulation by the avoidance of infections through the use of IVIG therapy.


Assuntos
Imunodeficiência de Variável Comum/etiologia , Deficiência de IgA/complicações , Deficiência de IgG/complicações , Lúpus Eritematoso Sistêmico/complicações , Pré-Escolar , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lúpus Eritematoso Sistêmico/imunologia , Masculino
17.
Pediatr Int ; 54(6): 758-61, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22726275

RESUMO

BACKGROUND: The severity of the 2009 pandemic H1N1 influenza (H1N1 pdm 09) in immune deficient children is unknown. The aim of the present study was to investigate this in a case of complete IgG3 deficiency complicated by pneumonia and asthma attack. METHODS: The clinical parameters of the IgG3 deficiency patient were compared with those of four control patients using 95% confidence intervals. These control patients were selected from 71 patients admitted due to pneumonia or bronchitis caused by H1N1 pdm 09, and were chosen according to age, absence of pretreatment with oseltamivir before admission, presence of a past history of asthma, use of antibiotics, and combination of inhalation of a beta2 agonist and treatment with i.v. methylprednisolone for asthma attack. RESULTS: The IgG3 deficiency patient had significantly longer duration of admission and period of oseltamivir, with a significantly decreased pulse oxygen saturation and increased maximum serum C-reactive protein, creatine kinase and urinary excretion of ß2-microglobulin/creatinine, compared with the controls (P < 0.05). CONCLUSIONS: Complete IgG3 deficiency is possibly associated with severity of the clinical course of pneumonia and asthma attack in children suffering from H1N1 pdm 09.


Assuntos
Asma/etiologia , Deficiência de IgG/complicações , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/epidemiologia , Pandemias , Pneumonia Viral/etiologia , Adolescente , Asma/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Deficiência de IgG/epidemiologia , Deficiência de IgG/metabolismo , Incidência , Influenza Humana/complicações , Influenza Humana/diagnóstico , Japão/epidemiologia , Masculino , Pneumonia Viral/epidemiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências
18.
Eur J Pediatr ; 170(6): 693-702, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21544519

RESUMO

Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies and are characterized by a defect in the production of normal amounts of antigen-specific antibodies. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemias, in which the antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent respiratory tract infections, PADs are associated with a wide range of other clinical complications. This review will describe the pathophysiology, diagnosis, and treatment of the different PADs.


Assuntos
Agamaglobulinemia , Anticorpos/sangue , Deficiência de IgA , Deficiência de IgG , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Agamaglobulinemia/fisiopatologia , Agamaglobulinemia/terapia , Criança , Diagnóstico Diferencial , Humanos , Deficiência de IgA/complicações , Deficiência de IgA/diagnóstico , Deficiência de IgA/fisiopatologia , Deficiência de IgA/terapia , Deficiência de IgG/complicações , Deficiência de IgG/diagnóstico , Deficiência de IgG/fisiopatologia , Deficiência de IgG/terapia , Infecções Respiratórias/imunologia , Resultado do Tratamento
19.
Asian Pac J Allergy Immunol ; 29(4): 332-7, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22299313

RESUMO

BACKGROUND: Patients with Immunoglobulin G (IgG) subclass deficiency may suffer from recurrent infections, mainly sino-pulmonary infection. OBJECTIVE: To determine the epidemiology of IgG subclass deficiency in Thai children at a tertiary care hospital and to compare the differences between children who were diagnosed with IgG subclass deficiency by using low level criteria [less than 2 standard deviation (SD) of normal levels for age] and by using low percentage criteria (proportion of each IgG subclasses/total IgG). METHODS: The study was a descriptive study of 55 children up to 15 years old with recurrent infections diagnosed as having IgG subclass deficiency but no acquired or other primary immune deficiencies except for IgA and/or IgM deficiency. RESULT: Isolated IgG3 subclass deficiency was the most common IgG subclass deficiency (56.4%). IgG3 subclass deficiency, either isolated or combined with other IgG subclass deficiency, was found in 85.5% of the cases. The common age of onset was between birth and five years of age. The most common presenting symptom was recurrent sinusitis (83.6%). Majority of the cases (89.3%) were diagnosed by low percentage criteria while 12.7% were diagnosed by low level criteria. All cases with low levels of IgG subclass antibodies also had low percentages. There were no statistically significant differences in the clinical manifestations and management methods between the children who were diagnosed by low level and low percentage. CONCLUSION: IgG3 subclass deficiency was the most common IgG subclass deficiency in Thai children. The most common presenting symptom was recurrent sinusitis. Although the diagnosis could be made in the patients with recurrent upper respiratory infection by using low level criteria, but the diagnosis should be considered when the low percentage criteria are met.


Assuntos
Deficiência de IgG/epidemiologia , Deficiência de IgG/imunologia , Adolescente , Idade de Início , Criança , Pré-Escolar , Feminino , Humanos , Deficiência de IgG/complicações , Lactente , Recém-Nascido , Infecções/imunologia , Masculino , Tailândia/epidemiologia
20.
Asian Pac J Allergy Immunol ; 29(1): 73-7, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21560491

RESUMO

BACKGROUND: Frequent upper respiratory illness (URI) is a common problem in preschool children. Allergic rhinitis and immunoglobulin (Ig) deficiency are usually suspected as underlying etiologies. OBJECTIVE: To determine the prevalence of allergic rhinitis and Ig and IgG subclass deficiency in preschool children with frequent URI. METHODS: Two thousand eight hundred and seventy-six questionnaires were distributed to the parents of children aged 3-6 years in 24 kindergartens. Firstly, they determined the frequency of URI in the previous year and secondly the prevalence of rhinitis according to the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. The skin prick test (SPT) was performed and serum Ig and IgG subclasses were measured in children with frequent URI (> or = 10 episodes per year). Allergic rhinitis was diagnosed when the child had had rhinitis in the previous 12 months and positive SPT for at least 1 aeroallergen. RESULTS: Two thousand three hundred and one questionnaires (80.01%) were returned. Ninety-four out of 219 children with frequent URI participated in the study. The prevalence of allergic rhinitis in the participants was 42.55%. Exclusive breastfeeding for at least 6 months had a protective effect, while paternal history of rhinitis was a risk factor. All participants had normal serum IgG, IgA, IgM and IgG subclass levels for age. CONCLUSION: The prevalence of allergic rhinitis in preschool children with frequent URI in our study was 42.55%. Allergic rhinitis should be considered if they have a family history of allergic rhinitis. Immunoglobulin deficiency was not found in our study.


Assuntos
Deficiência de IgG/complicações , Infecções Respiratórias/complicações , Infecções Respiratórias/epidemiologia , Rinite Alérgica Perene/complicações , Rinite Alérgica Perene/epidemiologia , Agamaglobulinemia/complicações , Agamaglobulinemia/epidemiologia , Agamaglobulinemia/imunologia , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Deficiência de IgG/epidemiologia , Deficiência de IgG/imunologia , Imunoglobulinas/sangue , Masculino , Prevalência , Infecções Respiratórias/imunologia , Rinite Alérgica Perene/imunologia , Fatores de Risco , Testes Cutâneos
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