scholarly journals Partial and Transient Clinical Response to Omalizumab in IL-21-Induced Low STAT3-Phosphorylation on Hyper-IgE Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Cesar Daniel Alonso-Bello ◽  
María del Carmen Jiménez-Martínez ◽  
María Eugenia Vargas-Camaño ◽  
Sagrario Hierro-Orozco ◽  
Mario Alberto Ynga-Durand ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
High Serum ◽  
Recurrent Infections ◽  
Dominant Form ◽  
Cutaneous Manifestations ◽  
Serum Ige ◽  
Hyper Ige Syndrome ◽  
Stat3 Phosphorylation ◽  
Eczematous Dermatitis ◽  
Pulmonary Abnormalities

Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by elevated levels of immunoglobulin E (IgE), eczematous dermatitis, cold abscesses, and recurrent infections of the lung and skin caused by Staphylococcus aureus. The dominant form is characterized by nonimmunologic features including skeletal, connective tissue, and pulmonary abnormalities in addition to recurrent infections and eczema. Omalizumab is a humanized recombinant monoclonal antibody against IgE. Several studies reported clinical improvement with omalizumab in patients with severe atopic eczema with high serum IgE level. We present the case of a 37-year-old male with HIES and cutaneous manifestations, treated with humanized recombinant monoclonal antibodies efalizumab and omalizumab. After therapy for 4 years, we observed diminished eczema and serum IgE levels.

Hyper-IgE Syndrome in an Infant

2016 ◽  
Vol 20 (4) ◽  
pp. 340-342 ◽  
Author(s):  
Mariah Giberson ◽  
Laura Finlayson
Keyword(s):  
Clinical Presentation ◽  
Neonatal Period ◽  
Immunoglobulin E ◽  
Current Theory ◽  
Primary Immunodeficiency Disorder ◽  
Multisystem Disorder ◽  
Cutaneous Manifestations ◽  
Multiple Systems ◽  
Hyper Ige Syndrome ◽  
Immunodeficiency Disorder

Background: Hyper–immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder that affects multiple systems. One of the early findings is a papulopustular rash, which has a wide differential diagnosis. Method: The authors report the case of a male newborn diagnosed with HIES. He presented with papulopustular dermatitis on the scalp. The authors also present a review of current theory on the pathophysiology, clinical presentation, and management of HIES. Conclusion: Although HIES is a multisystem disorder, many of the manifestations of HIES may present after the neonatal period. The cutaneous manifestations of HIES are usually present shortly after birth, and the presentation of pustules in a newborn may be one of the reasons a dermatologist would be asked to assess a patient in the neonatal period.

scholarly journals Hyper-IgE Syndrome with STAT3 Mutation: A Case Report in Mainland China

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Lixin Xie ◽  
Xiaoxiang Hu ◽  
Yang Li ◽  
Weihua Zhang ◽  
Liang'an Chen
Keyword(s):  
Autosomal Dominant ◽  
Immunoglobulin E ◽  
Mainland China ◽  
High Serum ◽  
Deciduous Teeth ◽  
Autosomal Dominant Trait ◽  
Hyper Ige Syndrome ◽  
Stat3 Mutation ◽  
Transcription 3 ◽  
Human Signal

Hyper-immunoglobulin E syndromes (HIES) including compound primary immunodeficiency and nonimmunological abnormalities are characterized by extremely high serum IgE levels, eosinophilia, eczema, susceptibility to infections, distinctive facial appearance, retention of deciduous teeth, cyst-forming pneumonias, and skeletal abnormalities. Itis reported that some cases of familial HIES are relative to autosomal dominant or recessive inheritance, but most cases are sporadic, and result from mutations in the human signal transducer and activator of transcription 3 (STAT3) gene. In this paper, we firstly report a young man diagnosed of Hyper-IgE syndrome with STAT3 mutation in Mainland China, and investigate the autosomal dominant trait of his family members.

scholarly journals Immunoglobulin E levels and pregnancy-induced hypertension: Japan Environment and Children’s Study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hyo Kyozuka ◽  
◽  
Tsuyoshi Murata ◽  
Toma Fukuda ◽  
Yuta Endo ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Late Onset ◽  
First Trimester ◽  
High Serum ◽  
Pregnancy Induced Hypertension ◽  
Serum Ige ◽  
Japanese Cohort ◽  
Increased Risk ◽  
Induced Hypertension ◽  
Hypertensive Disorders In Pregnancy

AbstractHigh serum immunoglobulin E (IgE) levels are associated with cardiovascular events. We aimed to evaluate the association between total IgE levels during the first trimester of pregnancy and pregnancy-induced hypertension (PIH) development in a large Japanese cohort. We analysed data pertaining to singleton primipara pregnancies recorded in the Japan Environment and Children’s Study involving births from 2011 to 2014. Serum IgE levels were determined using the immunonephelometric technique. High serum IgE was defined as level ≥ 170 IU/ml. Hypertensive disorders in pregnancy (HDP) were categorized into early onset (Eo) PIH (developed < 34 weeks) or late onset (Lo) PIH (developed ≧ 34 weeks). A multiple logistic regression model was used to estimate the risk of high serum IgE levels on PIH, Eo-PIH, and Lo-PIH. Overall, 32,518 participants were enrolled. The prevalence of total, Eo-, and Lo-PIH was 3.2%, 0.6%, and 2.3%, respectively. Patients with high serum IgE levels had an increased risk of Lo-HDP (adjusted odds ratio [aOR]:1.19, 95% confidence interval 1.01–1.40). No correlation was found with either PIH (total) or Eo-PIH. High serum IgE levels during the first trimester were associated with the risk of Lo-PIH. Our results could influence and shape further research regarding the pathogenesis of Lo hypertension.

scholarly journals Hyper IgE syndrome (hies; job syndrome): A case report

2015 ◽  
Vol 05 (04) ◽  
pp. 099-101
Author(s):  
Priyanka Ameta ◽  
Anuj Dhyani ◽  
Vignesh Hebri Nayak ◽  
Suresh Goyal
Keyword(s):  
Immunoglobulin E ◽  
Serum Levels ◽  
High Serum ◽  
Facial Appearance ◽  
Oral Manifestations ◽  
Pulmonary Infections ◽  
Primary Immunodeficiency Disorder ◽  
Hyper Ige Syndrome ◽  
Immunodeficiency Disorder ◽  
Job Syndrome

AbstractThe hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder characterized by high serum levels of immunoglobulin E (IgE), recurrent cutaneous and pulmonary infections, chronic dermatitis and a variety of connective tissue and skeletal abnormalities. These patients share characteristic facial appearance and many oral manifestations. We report a case of hyper IgE syndrome (HIES) also known as “Job syndrome“.

Recurrent Skin Ulcers with Facial Dysmorphism and Sinopulmonary Infections: Thinking Beyond Hyper-IgE Syndrome

2022 ◽  
Author(s):  
Aakash Chandran Chidambaram ◽  
Kiruthiga Sugumar ◽  
Selvamanojkumar Sundaravel ◽  
Jaikumar Govindaswamy Ramamoorthy ◽  
Siddardha Bathula ◽  
...  
Keyword(s):  
Primary Teeth ◽  
Immunoglobulin E ◽  
Case Reports ◽  
Facial Dysmorphism ◽  
Recurrent Infections ◽  
Prolidase Deficiency ◽  
Hyper Ige Syndrome ◽  
Flow Cytometric ◽  
Skin Ulcers ◽  
Rare Inborn Error

AbstractProlidase deficiency (PD) is a rare inborn error of metabolism causing ulcers and other skin disorders, splenomegaly, developmental delay, and recurrent infections. Most of the literature is constituted of isolated case reports. It occurs due to the mutations in the prolidase gene (PEPD) that result in loss of prolidase activity. We reported here a child who had presented with features compatible with hyper-immunoglobulin E syndrome (HIES) like recurrent skin ulcers, recurrent infections, facial dysmorphism, retained primary teeth, and elevated levels of immunoglobulin E levels but with normal flow cytometric assays, which was later diagnosed as PD.

scholarly journals Two Sisters with Idiopathic Pulmonary Hemosiderosis

2007 ◽  
Vol 14 (8) ◽  
pp. 490-493 ◽  
Author(s):  
Mehmet Gencer ◽  
Erkan Ceylan ◽  
Muharrem Bitiren ◽  
Ahmet Koc
Keyword(s):  
Chest Radiography ◽  
Immunoglobulin E ◽  
High Serum ◽  
Hemoglobin Level ◽  
Unknown Etiology ◽  
Total Serum ◽  
Diffuse Infiltration ◽  
Serum Ige ◽  
Pulmonary Hemosiderosis ◽  
Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE) level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%. Her chest radiography revealed diffuse infiltration. She died due to respiratory failure. The second patient was 18 years of age. She had also suffered from dry cough and gradually increasing dyspnea for two years. She had partial pneumothorax in the right lung and diffuse infiltration in other pulmonary fields on chest radiography. Her hemoglobin level was 99 g/L, total serum IgE level was 1200 U/mL and eosinophilia was 8%. IPH was diagnosed by open lung biopsy. All these findings suggested that familial or allergic factors, as well as immunological factors, might have contributed to the etiology of IPH.

scholarly journals Study of serum IgE levels in childhood asthma in Barabanki region, India

2018 ◽  
Vol 5 (5) ◽  
pp. 1755
Author(s):  
Ekansh Rathoria ◽  
Utkarsh Bansal ◽  
Abhishek Gupta ◽  
Nyay Bhai Gupta ◽  
Ravindra Ahuja ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Elevated Serum ◽  
Allergic Diseases ◽  
Normal Subjects ◽  
High Serum ◽  
Serum Immunoglobulin ◽  
Age Group ◽  
Mean Values ◽  
Serum Ige ◽  
Asthmatic Patients

Background: Elevated Serum Immunoglobulin E (IgE) levels are characteristic of most of the allergic diseases including asthma. Most of the asthma patients are allergic to inhaled antigens and chemical antigens, which lead to their sensitization and induce a state of hypersensitivity that is IgE mediated hypersensitivity reaction. The aim of this study was to estimate and compare serum IgE levels in childhood asthmatics and in normal subjects and to obtain the relationship between serum IgE levels and severity of asthma.Methods: A stratified sample of 58 patients within the age group of 5-15 years including 36 male and 22 female asthmatic patients and 58 healthy controls within the same age group were included in this study and classified according to GINA classification 2016. Serum IgE levels were estimated by using ELISA kit.Results: Mean IgE levels ranged from 163.82 IU/mL in normal subjects to 881.81 IU/mL in asthmatics. The mean values of Serum IgE levels in mild, moderate and severe asthmatic children were 625.25 IU/mL, 871.77 IU/mL, 1225.05 IU/mL respectively.Conclusions: High Serum Immunoglobulin E levels were found in childhood asthmatics as compared to normal subjects. Serum IgE levels were found to increase as the severity of asthma increased. The variability in each grade of asthma was very large so we could not find any statistically significant correlation.

scholarly journals Immunoglobulin E Levels and Hypertensive Disorders of Pregnancy: Japan Environment and Children’s Study

2020 ◽  
Author(s):  
Hyo Kyozuka ◽  
Tuyoshi Murata ◽  
Toma Fukuda ◽  
Yuta Endo ◽  
Akiko Yamaguchi ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Early Onset ◽  
Immunoglobulin E ◽  
Late Onset ◽  
First Trimester ◽  
High Serum ◽  
Hypertensive Disorders Of Pregnancy ◽  
Serum Ige ◽  
Hypertensive Disorders ◽  
Increased Risk

Abstract High serum immunoglobulin E (IgE) levels are associated with cardiovascular events. We aimed to evaluate the association between total IgE levels during the first trimester of pregnancy and hypertensive disorders of pregnancy (HDP) development in a large Japanese cohort. We analysed data pertaining to singleton primipara pregnancies recorded in the Japan Environment and Children’s Study involving births in 2011–2014. First trimester’s serum IgE levels were determined using the immunonephelometric technique. High serum IgE was defined as IgE levels ≥170 IU/ml. A multiple logistic regression model was used to estimate the risk of high serum IgE levels on HDP, comprising early-onset and late-onset hypertension. A total of 32,518 participants were enrolled. The prevalence of total, early-onset, and late-onset HDP was 3.2%, 0.6%, and 2.3%, respectively. Patients with high serum IgE levels had an increased risk of late-onset hypertension (adjusted odds ratio [aOR]: 1.19, 95% confidence interval: 1.01–1.40). No correlation was found with either HDP (total) or early-onset hypertension (aOR: 1.15 and 0.85, 95% confidence interval: 0.99–1.32 and 0.60–1.21, respectively). High serum IgE levels during the first trimester are associated with late-onset hypertension. Our results could influence and shape further research regarding the pathogenesis of late-onset hypertension.

Hyper Immunoglobulin E Syndrome (HIES): Report of a New Case Treated with Omalizumab and Dexametasone

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4934-4934
Author(s):  
Luis Fernando Cortázar-Benítez ◽  
R.M. Rojas Sotelo ◽  
Pablo Vargas-Viveros ◽  
Rafael Hurtado-Monroy ◽  
Catalina Romo Aguirre ◽  
...  
Keyword(s):  
Immunoglobulin E ◽  
Conflicts Of Interest ◽  
Upper Airway ◽  
Severe Case ◽  
Skin Lesions ◽  
High Serum ◽  
Response To Treatment ◽  
Rapid Improvement ◽  
Serum Ige ◽  
Airway Infections

Abstract Abstract 4934 The classical triad of eczema, recurrent skin and lung infections and high serum IgE, other connective tissue, cardiac and brain abnormalities are the accepted diagnostic criteria for HIES. The treatment of HIES is not established due to the scarce number of published cases, however is limited to skin local measures and supportive care. Herein we inform a severe case with response to Omalizumab, a monoclonal anti IgE antibody in combination with Dexametasone. A 36 year old male with family history of asthma and atopic dermatitis (AD). Since child, the patient had allergies and AD. At 8 years he presented asthma and recurrent upper airway infections at 2 to 3 times per year and 7 dental pieces removal. In the last 3 years he noted important increase of AD with very intense and disabling pruritus without response to treatment and progression to generalized erithrodermia, nodes in legs and arms, and skin trasudate, axilar and inguinal lymph nodes (6×4 cm). Laboratory positive test were eosinophils 6%, serum IgE 29,280 IU/mL. Skin biopsy showed psoriasis-like dermatitis, micro abscesses and dermatopathic lymphadenopathy. We started treatment with trimetoprim/Sulfamethoxazole 80/400 mg twice a day, oral Dexametasone 20 mg weekly and Omalizumab 200 mg subcutaneously every two weeks. After 15 days of treatment there was a rapid improvement of skin derangement and IgE levels dropped to 2000 IU/mL after 16 weeks of treatment. Patient continues on treatment with no side effects and pruritus disappears. Omalizumab is a monoclonal antibody against IgE recently introduced to the treatment of HIES in a few patients reported since 2008 with excellent response, along with intensive care of skin lesions, prompt antibiotic and antimycotic treatment for infections are the mainstay of HIES management. Disclosures: No relevant conflicts of interest to declare.

Serum immunoglobulin E reactivity to cross-reacting panallergen components in north-eastern Poland patients pollen sensitized

2020 ◽  
Vol 41 (3) ◽  
pp. 183-191
Author(s):  
Krzysztof Kowal ◽  
Agnieszka Pampuch ◽  
Ewa Sacharzewska ◽  
Ewa Swiebocka ◽  
Zenon Siergiejko ◽  
...  
Keyword(s):  
Allergic Rhinitis ◽  
Allergen Immunotherapy ◽  
Immunoglobulin E ◽  
Pollen Allergy ◽  
Serum Ige ◽  
Ige Reactivity ◽  
Allergen Components ◽  
North Eastern ◽  
Allergen Sources ◽  
Seasonal Pollen

Background: The presence of immunoglobulin E (IgE), which cross-reacts with allergen components, such as profilins, polcalcins, and cross-reacting carbohydrate determinants (CCD), creates a problem when selecting patients for allergen immunotherapy by using conventional methods. The aim of this study was to evaluate the prevalence of sensitization to profilins, polcalcins, and CCDs in patients with seasonal pollen allergic rhinitis. Methods: The study was performed on a group of 112 patients with seasonal pollen allergic rhinitis, ages 14 to 55 years, with sensitization to at least one seasonal allergen (IgE > 0.7 kUA/L). The presence of IgE sensitization to recombinant (r) Bet v 2, rPhl p 12, rBet v 4, rPhl p 7, and CCDs, in addition to rBet v 1, rPhl p 1, rPhl p 5, was evaluated by using a multiparameter immunoblot. Results: Among the studied patients, 64.3, 80.4, and 41.1% were sensitized to birch, timothy grass, and mugwort pollen, respectively. Sensitization to profilins rBet v 2/Phl p 12 was demonstrated in 28.6%, to polcalcins Bet v 4/Phl p 7 in 8.9%, and to CCDs in 25%. In 29.3%, serum IgE reactivity to any of the cross-reactive components could be demonstrated. Serum IgE reactivity to rBet v 2 was always accompanied by IgE reactivity to rPhl p 12, and IgE reactivity to rBet v 4 was always accompanied by IgE reactivity to rPhl p 7. Among the patients with pollinosis co-sensitized to at least two allergen sources according to extract-based diagnosis, possible false-positive results due to sensitization to cross-reactive components were detected in 17.9%. Conclusion: Evaluation of sensitization to cross-reacting components may be useful in evaluation of patients with pollen allergy who are being assessed for allergen immunotherapy to optimize the constitution of their immunotherapy vaccines.

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