Early diagnosis effects the prognosis in children with atypical wheeze

WOS: 000580388500007PubMed: 33061752Aim: Recurrent wheezing is a common problem in preschool children. It is classified into two groups because there can be many reasons for wheeze: typical and atypical. the aim of this study was to identify the general features of atypical wheezy children. Material and Methods: Three hundred two children who presented to our clinic between 2000 and 2015 for three or more wheezing attacks and were diagnosed as having an underlying disease such as bronchiectasis, foreign body aspiration, recurrent aspiration pneumonia, cystic fibrosis, bronchopulmonary dysplasia, congenital anomalies, and tuberculosis, were included in the study. Results: in this study, 127 (42.1%) girls and 175 (57.9%) boys were evaluated. the diagnostic distribution of the patients was as follows: bronchopulmonary dysplasia (21.9%), bronchiolitis obliterans (16.6%), bronchiectasis (14.5%), bronchiolitis obliterans + primary immunodeficiency (12.3%), cystic fibrosis (10.3%), bronchiectasis + primary immunodeficiency (7.9%), recurrent aspiration pneumonia (3.6%) and foreign body aspiration (3.3%), and other diseases (9.6%). Mosaic oligemia, bronchiectasis, atelectasis, bronchiolectasis, and small airway disease were the most distinct findings on high-resolution lung tomography. When the patients were evaluated clinically, radiologically, and according to pulmonary functions after an average period of 40 months, it was seen that 9.2% deteriorated, 33.9% regressed, and 56.7% remained stable. Presentation to hospital after the first attack occurred earlier in patients with bronchopulmonary dysplasia, bronchiolitis obliterans and bronchiolitis obliterans + primary immunodeficiency compared with patients with bronchiectasis, bronchiectasis + primary immunodeficiency, and cystic fibrosis. When presentation time and outcomes were evaluated, it was found that 63.4% of patients who presented to hospital early (0-6 months) and 7.5% of patients who presented late (after 5 years) had regression. Conclusion: Recurrent wheezy children must be promptly evaluated for an underlying disease. Early diagnosis and treatment influence the prognosis

Could Age and Oral Challenge Outcomes Identify High-Risk Patients During Cow's Milk Oral Immunotherapy?

Objective: Severe immunglobuline E (IgE)-mediated reactions during oral immunotherapy (OIT) are major obstacles to treatment. The present study aimed to evaluate and identify clinical and laboratory biomarkers of adverse events during OIT among children with cow's milk (CM) allergy.Study Design: Eighty-six children older than 36 months who had undergone OIT with milk were enrolled. Clinical data, oral food challenge (OFC) test results, and laboratory data were recorded retrospectively.Results: The median duration of the build-up phase of OIT was 19 weeks (min 10-max 40) and the duration of the maintenance phase was 86.5 (min 1-max 132) months. A total of 11,767 CM doses were administered during the build-up phase and adverse reactions were seen in 62 (73.8%) patients with reactions registered for 157 doses among 11,767 (1/75 doses). The number of reactions during the maintenance phase was 41 (47.6%) in 24 (27.9%) patients. There was a significant reduction in the number of reactions (P = 0.000) between the build-up phase and maintenance phase. Adverse reactions and anaphylaxis were higher for patients who had cough during OFC (P = 0.003, P = 0.002, respectively) during the build-up phase and also during the maintenance phase too (P = 0.000). Evaluation for all reactions and anaphylaxis (during build-up and maintenance) with Kaplan-Meier and Cox regression analysis showed class IV-VI of CM-specific immunoglobulin E (sIgE), casein-sIgE and cough during OFC were significantly associated with increased probability of reaction and anaphylaxis. Younger age at onset of OIT was associated with risk reduction (0.017).Conclusion: Laboratory data and reactions during the OFC (especially cough) can help to identify high-risk patients during OIT

Successful Desensitization Protocol in an Infant Following Anaphylaxis Secondary to Recombinant Factor VIIa

WOS: 000571400900012Background:Recombinant factor VIIa (rFVIIa) is a highly purified recombinant protein. It is approved for the treatment and prevention of bleeding episodes associated with congenital factor VII deficiency, congenital hemophilia with inhibitors, and Glanzmann's thrombasthenia. the most commonly reported adverse events are thrombolytic in nature. in this report, we present a successful desensitization protocol administered to an infant with a history of anaphylaxis to rFVIIa. Case:A male infant with a history of gingival bleeding at the age of 6 months was diagnosed with factor VII deficiency with a factor VII level of 1%. His sister also had diagnosis of factor VII deficiency. Our patient was hospitalized at 10 months of age with generalized petechiae and bloody stools. Twenty minutes after administration of rFVIIa, he developed anaphylaxis that responded to epinephrine and supportive care. Subsequently he was evaluated at the allergy clinic, where a skin prick test with rFVIIa was negative. However, the intradermal skin test, applied with 1/1,000 (1 mu g/1 mL, 0.1 mL) dilution of rFVIIa, showed induration of 8 mm (positive reaction). Because there is no alternative treatment for factor VII deficiency, we developed a successful 13-step desensitization protocol with rFVIIa (NovoSeven(R)). Desensitization was performed an additional 2 times using the same protocol, one of which was for a head injury and the other for a swollen knee since the period between the doses was similar to 3 months. Conclusion:Allergic reactions, such as anaphylaxis can occur without prior exposure. This can be due to the high molecular weight and structural property of the biological agent. in this report, we present an effective desensitization protocol for an infant with a history of anaphylaxis to rFVIIa. Desensitization protocols in this age group should be carried out in a medical facility and with specialized staff and equipment prepared to care for anaphylaxis

Effects of Cow's Milk Components, Goat's Milk and Sheep's Milk Sensitivities on Clinical Findings, and Tolerance Development in Cow's Milk Allergy

Objective: Cow's milk (CM) contains some proteins capable of causing an allergic reaction in a sensitized individual and one of the most common causes of food allergy in childhood. Most of the patients will develop tolerance by the age of 3. In this study, we aimed to evaluate sensitivity to CM allergen components as well as goat's milk (GM) and sheep's milk (SM) cross reactions in cow's milk allergic (CMA) patients and to figure out the risk factors for tolerance non-development. Methods: This is a retrospective cross-sectional study including 66 patients for IgE-mediated CMA with mean age of 38 months. We evaluated the patients in two groups: Group 1 (n=50): Patients who have no tolerance in oral food challenge test; Group 2 (n= 16): Patients who were found tolerant to CM after elimination diet. CM-sIgE, alpha-lactalbumin (ALA)-sIgE, beta-Lactoglobulin (BLG)-sIgE, casein (CAS)-sIgE, GM-sIgE, and SM-sIgE, skin prick tests with CM and GM, and eosinophils in peripheral blood were all compared between two groups. Results: In the whole group, GM-sIgE and SM-sIgE were positive in 84.8% and ALA-sIgE, BLG-sIgE, and CAS-sIgE were positive in, respectively, 69.7%, 62.7%, and 77.3% of the patients. Two groups were similar in terms of age at onset and diagnosis, gender, median elimination period, total IgE levels, CM-sIgE, and eosinophilia (p>0.05). Mean wheal diameters of CM and GM in SPT (p<0.001), GM-sIgE (p=0.03), and SM-sIgE (p=0.01) were significantly higher in Group 1. There was a positive correlation between CM-sIgE and total IgE (p=0.001), eosinophilia percentage (p=0.04), CM wheal diameter in SPT (p=0.001), CAS-sIgE (p<0.001), GM-sIgE (p<0.001), and SM-sIgE (p<0.001) in Group 1. Patients with respiratory symptoms and history of anaphylaxis had higher CM-SPT, CM-sIgE, CAS-sIgE, GM-sIgE, and SM-sIgE (p<0.05) levels. Gastrointestinal and skin symptoms showed no relation with laboratory findings. Tolerance was not developed in any patient with a history of anaphylaxis. Conclusions: As with CM-sIgE levels and high induration diameters in SPT, high CAS-sIgE, SM-sIgE, and GM-sIgE levels are also risk factors for persistence of CMA; anaphylaxis, as a first reaction, may also be a risk factor. High CM-sIgE, CAS-sIgE, SM-sIgE, and GM-sIgE levels are associated with respiratory symptoms

Gain of function Mutations in STAT1: A recently defined cause for chronic mucocutaneous candidiasis disease mimicking combined immunodeficiencies

Chronic Mucocutaneous Candidiasis (CMC) is the chronic, recurrent, noninvasive Candida infections of the skin, mucous membranes, and nails. A 26-month-old girl was admitted with the complaints of recurrent oral Candidiasis, diarrhea, and respiratory infections. Candida albicans grew in oral mucosa swab. CMV and EBV DNA titers were elevated. She had hypergammaglobulinemia; IgE level, percentages of lymphocyte subgroups, and in vitro T-cell proliferation responses were normal. She had parenchymal nodules within the lungs and a calcific nodule in the liver. Chronic-recurrent infections with different pathogens leading to significant morbidity suggested combined immunodeficiency, CMC, or Mendelian susceptibility to mycobacterial diseases. Genetic analysis revealed a predefined heterozygous gain-of-function mutation (GOF) (c.1154 C>T, p.Thr385Met) in the gene coding STAT1 molecule. Hematopoietic stem cell transplantation (HSCT) was planned because of severe recurring infections. Patients with STAT1GOFmutations may exhibit diverse phenotypes including infectious and noninfectious findings. HSCT should be considered as an early treatment option before permanent organ damage leading tomorbidity andmortality develops. This case is presented to prompt clinicians to consider STAT1GOFmutations in the differential diagnosis of patientswith chronic Candidiasis and recurrent infections with multiple organisms, since these mutations are responsible for nearly half of CMC cases reporte

Gain-of-Function Mutations in STAT1

Chronic Mucocutaneous Candidiasis (CMC) is the chronic, recurrent, noninvasive Candida infections of the skin, mucous membranes, and nails. A 26-month-old girl was admitted with the complaints of recurrent oral Candidiasis, diarrhea, and respiratory infections. Candida albicans grew in oral mucosa swab. CMV and EBV DNA titers were elevated. She had hypergammaglobulinemia; IgE level, percentages of lymphocyte subgroups, and in vitro T-cell proliferation responses were normal. She had parenchymal nodules within the lungs and a calcific nodule in the liver. Chronic-recurrent infections with different pathogens leading to significant morbidity suggested combined immunodeficiency, CMC, or Mendelian susceptibility to mycobacterial diseases. Genetic analysis revealed a predefined heterozygous gain-of-function mutation (GOF) (c.1154 C>T, p.Thr385Met) in the gene coding STAT1 molecule. Hematopoietic stem cell transplantation (HSCT) was planned because of severe recurring infections. Patients with STAT1 GOF mutations may exhibit diverse phenotypes including infectious and noninfectious findings. HSCT should be considered as an early treatment option before permanent organ damage leading to morbidity and mortality develops. This case is presented to prompt clinicians to consider STAT1 GOF mutations in the differential diagnosis of patients with chronic Candidiasis and recurrent infections with multiple organisms, since these mutations are responsible for nearly half of CMC cases reported