scholarly journals Innate Mechanisms in Selective IgA Deficiency

2021 ◽  
Vol 12 ◽  
Author(s):  
Jingyan Zhang ◽  
Dèlenn van Oostrom ◽  
JianXi Li ◽  
Huub F. J. Savelkoul
Keyword(s):  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Serum Levels ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Immune Defense ◽  
Selective Iga Deficiency ◽  
Young Infants ◽  
Novel Therapeutic Strategies

Selective IgA deficiency (SIgAD), characterized by a serum IgA level below 0.07 mg/ml, while displaying normal serum levels of IgM and IgG antibodies, is the most frequently occurring primary immunodeficiency that reveals itself after the first four years after birth. These individuals with SIgAD are for the majority healthy and even when they are identified they are usually not investigated further or followed up. However, recent studies show that newborns and young infants already display clinical manifestations of this condition due to aberrancies in their immune defense. Interestingly, there is a huge heterogeneity in the clinical symptoms of the affected individuals. More than 50% of the affected individuals do not have clinical symptoms, while the individuals that do show clinical symptoms can suffer from mild to severe infections, allergies and autoimmune diseases. However, the reason for this heterogeneity in the manifestation of clinical symptoms of the individuals with SIgAD is unknown. Therefore, this review focusses on the characteristics of innate immune system driving T-cell independent IgA production and providing a mechanism underlying the development of SIgAD. Thereby, we focus on some important genes, including TNFRSF13B (encoding TACI), associated with SIgAD and the involvement of epigenetics, which will cover the methylation degree of TNFRSF13B, and environmental factors, including the gut microbiota, in the development of SIgAD. Currently, no specific treatment for SIgAD exists and novel therapeutic strategies could be developed based on the discussed information.

scholarly journals Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

Medicina ◽  
2022 ◽  
Vol 58 (1) ◽  
pp. 129
Author(s):  
Bianca Laura Cinicola ◽  
Federica Pulvirenti ◽  
Martina Capponi ◽  
Marta Bonetti ◽  
Giulia Brindisi ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Atopic Disease ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Secretory Iga ◽  
Primary Immune Deficiency ◽  
Recurrent Infections ◽  
Selective Iga Deficiency ◽  
The Relationship ◽  
Allergic Disorders

Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). It is classified as a humoral PID characterized by isolated deficiency of IgA (less than 7 mg/dL but normal serum IgG and IgM) in subjects greater than 4 years of age. Intrinsic defects in the maturation of B cells and a perturbation of Th cells and/or cytokine signals have been hypothesized to contribute to SIgAD pathogenesis. The genetic basis of IgA deficiency remains to be clarified. Patients with SIgAD can be either asymptomatic or symptomatic with clinical manifestations including allergy, autoimmunity and recurrent infections mainly of the respiratory and gastrointestinal tract. Studies analyzing allergy on SIgAD patients showed prevalence up to 84%, supporting in most cases the relationship between sIgAD and allergic disease. However, the prevalence of allergic disorders may be influenced by various factors. Thus, the question of whether allergy is more common in SIgAD patients compared to healthy subjects remains to be defined. Different hypotheses support an increased susceptibility to allergy in subjects with SIgAD. Recurrent infections due to loss of secretory IgA might have a role in the pathogenesis of allergy, and vice versa. Perturbation of microbiota also plays a role. The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy and SIgAD.

scholarly journals Secrets, puzzles and mysteries of macroamylasemia

2020 ◽  
Vol 46 (1) ◽  
pp. 12-22
Author(s):  
N. B. Gubergrits ◽  
N. V. Byelyayeva ◽  
G. M. Lukashevich ◽  
T. L. Mozhyna
Keyword(s):  
Serum Amylase ◽  
Amylase Activity ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Normal Serum ◽  
Amylase Level ◽  
Clearance Ratio ◽  
Urine Amylase ◽  
Classical Constant

Physiological features of amylase synthesis and excretion are considered in the article, presence of other sources of amylase synthesis different from pancreas and salivary glands is emphasized. Definitions of hyperenzymemia and macroamylasemia (MAE) are given. MAE is a state characterized by presence of circulating complexes of normal serum amylase with protein or carbohydrates in blood. There are 3 types of MAE: first — classical (constant hyperamylasemia, decreased amylase level in urine, high blood concentration of macroamylase complexes); second — hyperamylasemia with slightly decreased amylase activity in urine, macroamylase/normal amylase ratio is less than in the first type; third — normal blood and urine amylase activity, low macroamylase/normal amylase ratio. Pathogenesis is explained by connection of blood amylase and acute phase protein in different inflammatory, infectious diseases, malabsorption. MAE clinical manifestations could be absent, sometimes abdominal pain is possible. Hyperamylasemia and reduced urine amylase activity are typical. MAE diagnostics means determination of macroamylase complexes in blood (chromatography, calculation of the clearance ratio of amylase and creatinine). The article presents clinical cases describing extra-pancreatic MAE in women with malignant ovarian lesions. The question of expediency of thorough diagnostic examination in asymptomatic MAE is raised, which may turn out to be a symptom of cancer. The lack of specific treatment for MAE is emphasized.

scholarly journals Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Paula F. Aarestrup ◽  
Matheus F. Aarestrup ◽  
Beatriz J. V. Aarestrup ◽  
Fernando M. Aarestrup
Keyword(s):  
Sublingual Immunotherapy ◽  
Respiratory Infections ◽  
Clinical Manifestations ◽  
Iga Deficiency ◽  
Whole Cell ◽  
Bacterial Extract ◽  
Selective Iga Deficiency ◽  
Concurrent Use ◽  
Airway Infections ◽  
Der P1

Selective IgA deficiency is the most common type of primary immunodeficiency, but there is not yet a specific effective treatment. The most prevalent clinical manifestations are infectious diseases of the respiratory system. We report herein the case of an 11-year-old female with selective IgA deficiency and recurring episodes of respiratory infections associated with rhinitis and asthma. We evaluated the efficacy of sublingual immunotherapy combined with inactivated whole-cell bacterial extract and Der p1-specific immunotherapy. After 18 months of clinical follow-up, we observed a significant reduction in the number of episodes of respiratory infections associated with control of atopic diseases. We also observed a 3-fold increase in serum IgA levels compared to treatment initiation. This case demonstrates the potential utility of the concurrent use of sublingual immunotherapy with inactivated whole-cell bacterial extract and Der p1 for successful control of allergy and infection in partial selective IgA deficiency.

scholarly journals Symptomatic Secondary Selective IgM Immunodeficiency in Adult Man with Undiagnosed Celiac Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Eli Magen ◽  
Viktor Feldman ◽  
Mishal Joseph ◽  
Hadari Israel
Keyword(s):  
Celiac Disease ◽  
Adult Patient ◽  
Genetic Background ◽  
Clinical Symptoms ◽  
Iga Deficiency ◽  
Gluten Free Diet ◽  
Gluten Free ◽  
Selective Iga Deficiency ◽  
Igm Deficiency ◽  
Secondary Condition

Selective IgM immunodeficiency (SIgMID) is a heterogeneous disorder with no known genetic background and may occur as a primary or a secondary condition. Celiac disease has been reported in association with several humeral immunodeficiencies, including isolated severe selective IgA deficiency, panhypogammaglobulinemia, and isolated combined IgA and IgM deficiency. There are only few reported cases of pediatric and adult patients with SIgMID and celiac disease. In this paper, we describe an adult patient with a symptomatic secondary SIgMID associated with undiagnosed celiac disease, with a resolution of clinical symptoms of immunodeficiency and serum IgM normalization following a gluten-free diet.

Clinical Manifestations in Selective IgA Deficiency in Childhood; A Follow-up Report

1991 ◽  
Vol 80 (8-9) ◽  
pp. 798-804 ◽  
Author(s):  
P. C. J. DE LAAT ◽  
C. M. R. WEEMAES ◽  
R. GONERA ◽  
P. J. J. VAN MUNSTER ◽  
J. A. J. M. BAKKEREN ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Iga Deficiency ◽  
Selective Iga Deficiency

Primary immunodeficiency

2013 ◽  
pp. 1-80
Author(s):  
Gavin P Spickett
Keyword(s):  
B Lymphocyte ◽  
Iga Deficiency ◽  
Normal Serum ◽  
Antibody Deficiency ◽  
Igg Subclass Deficiency ◽  
Selective Iga Deficiency ◽  
Subclass Deficiency ◽  
Bruton’S Disease ◽  
Common Variable

Introduction Classification of immunodeficiency Clinical features of immunodeficiency Investigation of immunodeficiency Laboratory investigation Major B-lymphocyte disorders Rare antibody deficiency syndromes X-linked agammaglobulinaemia (Bruton’s disease) Common variable immunodeficiency (CVID) CVID 2: complications and treatment Selective IgA deficiency IgG subclass deficiency Specific antibody deficiency with normal serum immunoglobulins...

SERUM LIPID PATTERN IN CHICKENS OF THE OBESE STRAIN

1972 ◽  
Vol 55 (3) ◽  
pp. 609-615 ◽  
Author(s):  
BARBARA RUDAS ◽  
G. WICK ◽  
R. K. COLE
Keyword(s):  
Blood Sugar ◽  
Serum Lipid ◽  
Clinical Symptoms ◽  
Serum Levels ◽  
Normal Serum ◽  
Total Lipids ◽  
Glucose Levels ◽  
Prolonged Bleeding Time ◽  
Normal Limits ◽  
Total Glycerol

SUMMARY Blood sugar, the serum concentration of total lipids, total glycerol, cholesterol and phosphatides were determined in chickens from the Obese strain (OS) of White Leghorns. Over 90% of these chickens are afflicted with a spontaneously occurring autoimmune thyroiditis and show clinical symptoms of hypothyroidism. Blood glucose levels were increased above normal limits in OS chickens up to 5 weeks of age. Significantly increased serum lipid concentrations were found in the OS chickens at the ages of 1, 5, 8 and 15 weeks, while 3-week-old OS chickens showed almost normal serum lipid levels. A single intravenous injection of heparin in a dose which prolonged bleeding time, had no 'clearing effect' on the serum of OS birds. Insulin given subcutaneously for 4 days had no effect on blood sugar but decreased the serum phosphatides and total lipid concentrations. The short-term administration of high doses of thyroxine significantly lowered the serum levels of total lipids, cholesterol, phosphatides and total glycerol.

scholarly journals Monocrotophos poisoning through contaminated millet flour

2012 ◽  
Vol 63 (3) ◽  
pp. 377-383 ◽  
Author(s):  
Ashwin B. Patel ◽  
Aruna Dewan ◽  
Bharat C. Kaji
Keyword(s):  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Food Poisoning ◽  
Specific Treatment ◽  
Plasma Cholinesterase ◽  
Food Sources ◽  
Serum Cholinesterase ◽  
History Of ◽  
Cholinesterase Activities

Several episodes of mass poisoning by organophosphates (OPs) have been reported from the developing countries. The diagnosis of OP-poisoning is mainly based on the characteristic clinical features and history of exposure to a known OP compound. Estimation of serum and red blood cell (RBC) cholinesterase activities are helpful in confi rming the diagnosis. However, there is controversy regarding a defi nite relationship between serum cholinesterase activity and the severity of clinical manifestations and prognosis. This report describes an episode of mass monocrotophos poisoning that occurred due to accidental ingestion of monocrotophos-contaminated millet (so called bavta) fl our involving eight severely poisoned persons. Clinical presentation included severe abdominal pain, diarrhoea, vomiting, pupil narrowing, and diffi culty breathing. On hospital admission, plasma cholinesterase (PChE) and especially RBC acetylcholinesterase (AChE) activities correlated well with clinical symptoms presented by the patients. This case study highlights the need for clinicians to be aware of OP-pesticide poisoning from food sources and the need to look for depressed PChE and AChE activities that may point to OP exposure, so that OP-poisoning can be identifi ed immediately and patients can receive specific treatment, rather than general treatment for food poisoning.

scholarly journals Evaluation of infection with N protein-specific Immunoglobulin M and G in naturally occurring distemper in dogs

2020 ◽  
Vol 65 (No. 4) ◽  
pp. 168-173
Author(s):  
HS Saltik ◽  
M Kale
Keyword(s):  
Canine Distemper Virus ◽  
Indirect Elisa ◽  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Immunoglobulin M ◽  
High Morbidity ◽  
Canine Distemper ◽  
Cutaneous Lesions ◽  
N Protein

In dogs, canine distemper has a worldwide distribution with high morbidity/mortality, despite the widespread usage of vaccines and has no specific treatment. In susceptible animals with the canine distemper virus, respiratory, gastrointestinal and nervous system disorders, immunosuppression and cutaneous lesions can also be seen. Especially puppies and unvaccinated dogs are prone to get the viral infection. IgM and IgG antibodies constitute the major component of the natural antibodies produced during the primary and secondary antibody response that have long been recognised to inhibit viral infections. In the present study, the presence of the viral N protein-specific IgM and IgG was investigated by indirect ELISA in naturally infected dogs. Moreover, the rate of outbreaks in naturally infected dogs was shown by the detection of new and re-infections. In the Western Mediterranean region, blood serum samples were collected from 50 unvaccinated dogs for the mentioned infection between 2015 and 2017. At 0–12 months, in the dogs with clinical symptoms, the indirect ELISA detected 4% acute, 54% early convalescent, 40% late convalescent and 2% no infections phases. The clinical manifestations were studied in four main groups follow as: respiratory, gastrointestinal, nervous and cutaneous symptoms. The evaluation showed that the canine distemper virus N protein-specific antibodies detection by the indirect ELISA is quick and safe in naturally infected dogs. In conclusion, the method is very useful for the pre-diagnosis of the disease when evaluated together with the clinical symptoms. It helps to distinguish acute and convalescent (early/late) phases. Distinguishing these phases of infection is important for monitoring the spread of the outbreaks and identifying the risk of severe forms of canine distemper.

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