At birth, most Alaminoglupulin serum is of the type (G), which arises from the cross (IgG) parental cross the placenta during the last third of pregnancy.
As a result, the level of the (IgG) serum at birth equal or slightly higher than the level (IgG) serum of the mother.
Premature infants have levels of the (IgG) less than normal children.
In 30 premature infants born 32 weeks of gestational age have the focus (IgG) secret about 400 mg / dL.
The level of (IgG) in preterm infants are low compared with children with gestational age and fully reflects the possibility of a placental defect in the transition.
After birth, the land levels (IgG) derived from the mother rapidly during the 3 months to a level of approximately 400 mg / dl, in addition to the production of the infant (IgG) is not fully developed.
This series of events represent the so-called low natural gamma Glupulin blood in infants, and spoke of this phenomenon more clearly in preterm infants.
Under the ranks of IgG:
Falls under the IgG class of immunoglobulins four rows under the clear and distinct, which differ in the structure and function.
IgG1 -: complementary prove better than IgG2 and IgG3 while IgG4 does not link it c1q and therefore does not prove never completed.
IgG3 - and IgG1 Fc receptors linked Bolfah higher than IgG2 or IgG4
The row under the IgG2 It contains primarily antibodies directed against antigens of the type of saccharide Many and this presumably self-evident, that the low levels of IgG2 predispose to infections with bacteria capsulatus Balbola screed.
IgA, IgM, IgD, and IgE
Under natural conditions do not reflect these immunoglobulins the placenta, so its presence and high levels in cord blood suggests the presence of infection within the uterus.
Natural changes in the levels of IgM and IgA serum after birth are as follows:
• increase the level of IgM and quickly during the first month after birth is likely to be a response to the induction of Alontegina massive new environmental conditions, then increase gradually to reach about 60% of the adult and IgM during the first year of life.
• As for the IgA rises more slowly than its predecessor until it reaches about 20% of IgA during the first year of life.
Definition:
Lack of known gamma Globylwin blood transit in infants THI definition model as a heightened case of lack of blood fibrinogen gamma Glupulin observed naturally in infants in the three months to the first half of their lives.
Although access to this definition did not unite the diagnostic criteria well.
Recommendation / suggestion:
Must support the definition of THI on the presence of IgG levels below the average by at least 2 SD, using the standards associated with age, as standards fixed, and is accompanied by low levels of other types of Ig or non-drop.
This situation continues for more than six months of age, and occur in the absence of other immune deficiencies.
Laboratory and clinical aspects:
THI was observed mainly at the two different groups of infants fatigues identifying them from each other mainly why or how to guess immunodeficiency:
1 - is symptomatic Asymptomatic:
Formed the first group of relatives of patients with immune deficiency diseases are known.
Appeared to them in general they are in good health, the person of the disease because the immune Alglupulinat concentrations in serum have examined, and nothing more.
As the days pass Alglupulinat immune levels returned to normal and these patients remained clinically asymptomatic.
Remains important to detect this case is unknown.
2 - Infections due or unusual:
Formed the second group of infants referred to the measure Alglupulinat immune to infections due because they have started early in their lives.
And varies within individual infections, and have a kind of upper respiratory infections, recurrent URIs and inflammation of the middle ear and bronchitis to life-threatening infections Ktgerthm blood and bacterial meningitis.
Alaminioglupulin levels:
Hematological abnormalities: Hematological abnormalities are rarely described, with notes in some cases with neutropenia transit.
Most studies have found that the lymphocytes in the minority and cellular immunity intact.
Response to immunization:
Patients with THI usually have antibodies to normal or near normal response to immunization Bdhuvanat tetanus and diphtheria, and sometimes before they become Alaminioglupulin normal levels.
They also have the values of blood agglutinin isohemagglutinin isoforms in the natural for their age.
Rating:
As in other syndromes HIV primary, a patient who presents unusually, from infections opportunism and \ or recurrent infections requires us to test appropriate to assess the presence or absence of defects the quality of the immune system (humoral, cellular, macrophagic, or complementary) .
Evaluation of patients suspected of having THI consists of evaluation every 6 months to 12 months of the following:
Overall health status.
Levels of serum antibodies specific to previous vaccines.
In patients aged over two and a vaccine Almmnon 23-valent pneumococcal capsule is derived from the numerous screed, take the levels of antibodies measured in months 1, 6 and 12 after vaccination.
Differential Diagnosis
The THI is a disorder of a specific definition of self-
We can not put the diagnosis is certain only after the patient recovered clinically and the conduct of all laboratory tests.
And return to this point should be doing our very best to distinguish the THI disorders other primary immune deficiency, which can manifest similarly, such as:
The gamma Glupulin blood-related physical or recessive Babbga x XLA))
Common Variable Immunodeficiency (CVID)
and even a few cases of severe immune deficiency common
In practice few patients in the beginning I think they are infected with THI, but in the end with the passage of time they have developed symptoms and signs were more indicative to other disorders (?)
The gamma Glupulin blood:
Usually, we can easily distinguish it from the THI gamma Glupulin blood, which is characterized by:
a severe shortage in all the isotopes of Ig
inability to produce antibodies
the absence of normal peripheral lymphoid tissue
absence or severe reduction in the number of circulating B lymphocytes in the blood
severe purulent infections begin between the first and second year of life
Common Variable Immunodeficiency
the CVID is the most diagnosed disorders among those who suffer from primary immune deficiency
pretending to be usually between the second to third decade of life
Ig levels are low at first but does not normally be absent.
Most patients suffer from frequent sinus infections pulmonary
The most important factor to distinguish a preferred CVID of THI and the development of differential diagnosis is the inability of patients with CVID mind to produce protective levels of antibody quality, in particular IgG
About 60% of patients with CVID are also people with varying degrees of imbalance in the cellular immune
Compared to patients with THI mind to have a healthy cellular immunity and production of natural antibodies.
Conclusion
The diagnosis of lack of blood gamma Glupulin transit when the infant is diagnosed with THI excluded must not decide until long after the tests are comprehensive variables of clinical and laboratory diagnosis is sequential and retrospective recall by the past.
not be long do I have to follow the patient with THI and clear, the process takes the body back to normal up to 10 years.
supposed to generally be less than the number of infections suffered by the patient as progress in age, and levels return Alglupulinat immune College and sub-categories for Ig in the serum of normal blood in the end.
Patients also finally be able to keep the quality of natural antibodies for a period of time than a year.
The result
Although THI has been known for a long time, but only few studies have revealed a long-term consequences to him.
definition of THI alone shows that the disease is transient, and healing occurs automatically after 9-15 months of age, and IgG levels return normal after 2-4 years of age.
Treatment
Prevention antibiotic should be taken into account mainly in patients who suffer from repeated ear infections and respiratory tract.
Preferred option is a single daily dose of the Tree Mitobram Slwamytoxasul (by 5 mg / kg of the components of the Tri Mitobram) or amoxicillin by 10 mg / kg twice daily.
Treatment based on the transfer of immunoglobulins (antibodies) remain controversial in the treatment of these patients.
There are a variety of points that must be taken into account before any decision can be taken:
• treatment by the method of transfer of antibodies is a way for expensive treatment.
• the possibility of transmission of infectious agents remains stalled on the coincidence problem not solved, although significant improvement has been achieved through endoscopy and cleansing.
• adverse reactions that are sometimes life-threatening common in patients receiving products from the mines Glupulin.
• It should be noted that the patients lack the (IgA) can suffer from anaphylactic reactions. Also, the antibodies are given a negative (passive immunization) can delay the start of installation of antibodies in the patient.
• Most patients (lack of blood gamma Glupulin transit) suffer from recurrent infections weak virulence affecting mainly the respiratory system.
• During the treatment period there is no need to vaccinate these patients immunized negative.
• In addition, patients should not be vaccinated for three months after the cessation of the transfer of antibodies in order to avoid overlap.
• During the treatment period: Most of the recorded cases have been addressed Boddad immune for a short period ranging from a single shot to over 18 months.
• After cutting treatment must follow patients on a regular basis until completed and proven clinical examination and laboratory for a significant period of time before the announcement of a full recovery.
0 comments:
Enregistrer un commentaire