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X-Linked Agammaglobulinemia

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X-Linked Agammaglobulinemia

X-Linked Agammaglobulinemia

X-Linked Agammaglobulinemia are Bruton’s Disease and Congenital Agammaglobulinemia. XLA  generates X-linked recessive patterns. 

X-Linked Agammaglobulinemia is an inherited immunodeficiency disease, firstly came into existence in 1952 by Dr. Ogden Bruton. When the capacity of immunoglobulins, especially protein, B-cells and other antibodies to fight against certain bacteria decreases, XLA disease can happen. Bacteria such as streptococcus and staphylococcus are highly responsible for infection X-linked Agammaglobulinemia.  

Symptoms

Symptoms start appearing when the mother’s antibodies protection level becomes low. So when infant fail to generate the new antibodies in the body, infection enters and spreads in body, along with the below mentioned symptoms:

  • Skin Infection
  • Giardia related Diarrhea
  • Severe Arthritis
  • Hepatitis
  • Tonsils problems
  • Absence of lymph nodes
  • Pink Eye
  • Infection in bloodstream
  • Meningitis
  • Polio

All these Bruton’s Agammaglobulinemia Symptoms can lead to unpredictable and uncontrollable diseases, urgent doctor’s advice is a wise thing to do. 

Causes

40-50% of people get Bruton’s Agammaglobulinemia from their parents or other family members. Rest 40-50% occurs due to BTK gene’s presence and also because of instability in B-lymphocytes number.

More about Treatment

People associated with XLA disease mostly have problems like pneumonia, otitis, bronchitis, severe diarrhoea and sinus infections. Sometimes symptoms may be seen at a very early age of 1-2 years and sometimes it can be seen in adulthood also. In most of the cases XLA disease targets males rather than females about 85-90% in males and 10-15% in females.One of the reason in XLA disease formation, is mutation in BTK ( Bruton’s Tyrosine Kinase)gene which tend to destroy the B-cells and eventually generate X-linked recessive pattern. 

If an infant’s family members have Congenital Agammaglobulinemia then some traces can be found in the blood of the baby. In some people symptoms aren’t visible but still they can pass XLA to the next generation.

X-LINKED AGAMMAGLOBULINEMIA TREATMENTS

Though there is no permanent cure of Congenital Agammaglobulinemia , treatment can give improvement in life. There are numerous treatments comprising all kinds of medicines suitable from small toddler to adults to skyrocket the immune system and safe-guard patients from troublesome infections. Take a closer look at some :

  • To add and neutralize the numbers of antibodies, one of the reliable options is Immunoglobulin Replacements. 
  • Antibiotics - works best to reduce the symptoms in the body, especially taken daily or alternate days, can slowly vanish the infection.
  • Infusions of Gamma Globulin once or twice in a month is very beneficial because it is rich in antibodies.
  • For small children and infants injection is a safe option and for adults either is fine. 
  • There are some minor side effects of gamma globulin such as headache, nausea, uneasiness, back pain, etc.
  • Live viral vaccines are not commendable for children as it may increase and add new serious diseases.

Continuously stay in touch with the doctor or frequent visits to know more about the disease and discuss the possible ways to maintain healthy life and follow the lead of the doctor to diminish the exposure of infections. 

AGAMMAGLOBULINEMIA DIAGNOSIS

Diagnosis is tedious for uncommon and inherited diseases like XLA. Medical experts first monitor the family history, patients' symptoms, since how long the disease is present inside the body of the patient and on the basis of such factors diagnosis can be done.

Doctors start from screening tests, to observe the level of B-cells in the body. GTR elaborates the genetic tests and gives relevant information about the gene arrangement.

Other options are serum immunoglobulins tests, advanced single strand DNA tests, serum/gel infused electrophoresis. For females and males separate testing is done on the basis of gender.

FAQ

1. WHAT ARE THE RISK FACTORS ASSOCIATED WITH BRUTON’S AGAMMAGLOBULINEMIA ?

Lung Cancer, Rheumatoid arthritis, muscle inflammation and harms the central nervous system all these happens if the treatment is inappropriate and in absence of regular medications life-threatening disease enters.

2. WHAT IS THE ROLE OF LAB TESTS IN CONGENITAL AGAMMAGLOBULINEMIA?

Lab tests are very essential to detect specific symptoms at actual level and to get fast results molecular genetic test is in demand and the best about MGT test is it gives the result before the birth of the child. Fluorocytometry analysis is also very helpful. 

3. GENERALLY AT WHAT AGE XLA IS PRESENT ?

Typically it happens at an early stage of life, when the child is 6 months or 1 year old. Very few cases are reported where people in their adulthood, XLA is present.

4. IS THERE ANY NEED OF SURGERY FOR XLA disease ?

Wide choices of surgery for such disease is not there but some are excellent with tons of pros such as hematopoietic stem cell transplantation and tools like tympanostomy tubes and surgical drainage are reliable surgeries.

5. HOW COMMON IS X LINKED AGAMMAGLOBULINEMIA ?

It is a very rare case, 1-2 people are affected with Bruton’s disease out of 300,000. Also 60% cases are genetic and caused from family only and 30% happens due to BTK gene.

6. FROM WHICH SPECIALIST SHOULD A PERSON WITH XLA DISEASE SHOULD TAKE ADVICE ?

There are many diseases involved with XLA and so are the symptoms and each doctor can give you new and relevant information. So in my opinion, Hematologists, Pulmonologists, Osteologists, Gastroenterologists and dermatologists should be consulted.

7. WHAT  SHOULD BE THE DIET OF X LINKED AGAMMAGLOBULINEMIA PERSON ?

Person should eat balanced food which includes enough proteins, carbs, vitamins and fats. To cope up with a nutrient rich diet you can add supplements also under the doctor’s guidance. But fat content should be minimum.

8. WHAT THINGS A PATIENT WITH BRUTON’S DISEASE SHOULD AVOID ?

People should stay around the clean air. Also alcohol consumption must be minimized and smoking and drugs should be completely avoided.

Top Doctors For X-Linked Agammaglobulinemia Treatment

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Dr. Mahesh Janarthanan Pediatrics | Gender: Male | Experience: 6 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. Vidya Krishna Pediatrics | Gender: Female | Experience: 11 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. P. Venkatraman Pediatrics | Gender: Male | Experience: 30 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. S. Elayaraja Pediatrics | Gender: Male | Experience: 10 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. P. N. Vinoth Pediatrics | Gender: Male | Experience: 7 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. Saji James Pediatrics | Gender: Male | Experience: 17 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. P. S. Rajakumar Pediatrics | Gender: Male | Experience: 10 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. Latha Ravichandran Pediatrics | Gender: Female | Experience: 23 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. S. Shuba Pediatrics | Gender: Female | Experience: 18 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. P. Anitha Pediatrics | Gender: Female | Experience: 5 years | Hospital: Sri Ramachandra Medical Centre (SRMC)
Dr. Abhishek Kulkarni Pediatrics | Gender: Male | Experience: 10 years | Hospital: Narayana Hospital
Mr. C. R. Satish Kumar Pediatrics | Gender: Male | Experience: 10 years | Hospital: Manipal Hospitals, Bengaluru
Dr. A. Sivagnanam Pediatrics | Gender: Female | Experience: 10 years | Hospital: Manipal Hospitals, Bengaluru
Dr. Anuradha Vinod Pediatrics | Gender: Female | Experience: 10 years | Hospital: Manipal Hospitals, Bengaluru
Dr. Gursev Sandlas Pediatrics | Gender: Male | Experience: 10 years | Hospital: Kokilaben Dhirubhai Ambani Hospital
Dr. Hariram Pediatrics | Gender: Male | Experience: 30 years | Hospital: Fortis Hospital, Bengaluru
Dr. Deepa Sharma Pediatrics | Gender: Female | Experience: 20 years | Hospital: Apollo Indraprastha
Dr. Anupam Sibal Pediatrics | Gender: Male | Experience: 23 years | Hospital: Apollo Indraprastha
Dr. Gülen Usanmaz Pediatrics | Gender: Female | Experience: 10 years | Hospital: Hisar Intercontinental
Dr. Gülden Kafali Pediatrics | Gender: Female | Experience: 20 years | Hospital: Hisar Intercontinental
Dr. Nuh Duman Pediatrics | Gender: Male | Experience: 33 years | Hospital: Hisar Intercontinental
Dr. Yunus Nas Pediatrics | Gender: Male | Experience: 28 years | Hospital: Hisar Intercontinental
Dr. Mahi Kasap Pediatrics | Gender: Female | Experience: 10 years | Hospital: Hisar Intercontinental
Dr. Anurag Krishna Pediatrics | Gender: Male | Experience: 30 years | Hospital: Max Super Speciality Hospital, Saket
Dr. Mufique Gajdhar Pediatrics | Gender: Male | Experience: 10 years | Hospital: Thumbay Hospital, Ajman
Dr. Amira Omer Abdelrahman Pediatrics | Gender: Female | Experience: 13 years | Hospital: Thumbay Hospital, Dubai
Dr. Mamata Bothra Pediatrics | Gender: Female | Experience: 11 years | Hospital: Thumbay Hospital, Dubai
Dr. Fozi Abozaid Dakilah Pediatrics | Gender: Male | Experience: 20 years | Hospital: Thumbay Hospital, Dubai
Dr. Ahmar Shamim Pediatrics | Gender: Male | Experience: 5 years | Hospital: Thumbay Hospital, Dubai
Dr. Anil Kumar Shetty Pediatrics | Gender: Male | Experience: 16 years | Hospital: RAK Hospital
Dr. Tawfiq Nasir Fawazi Pediatrics | Gender: Male | Experience: 4 years | Hospital: RAK Hospital
Dr. Vishal Rajmal Mehta Pediatrics | Gender: Male | Experience: 15 years | Hospital: RAK Hospital
Dr. Rashmi Anandani Pediatrics | Gender: Female | Experience: 8 years | Hospital: RAK Hospital
Dr. Feras Al Hamed Pediatrics | Gender: Male | Experience: 8 years | Hospital: RAK Hospital
Dr. Ahmed Said Ahmed Ateek Pediatrics | Gender: Male | Experience: 40 years | Hospital: RAK Hospital
Dr. Deve Dass Pediatrics | Gender: Male | Experience: 7 years | Hospital: RAK Hospital
Dr. Waseem Ahmed Pediatrics | Gender: Male | Experience: 24 years | Hospital: RAK Hospital
Dr. Sameer Sajwani Pediatrics | Gender: Male | Experience: 25 years | Hospital: Saudi German Hospital
Dr. Mirvat Osman Pediatrics | Gender: Female | Experience: 15 years | Hospital: Saudi German Hospital
Dr. Saba Junaid Pediatrics | Gender: Female | Experience: 9 years | Hospital: Saudi German Hospital
Dr. Habib Mansour Sammouh Pediatrics | Gender: Male | Experience: 20 years | Hospital: Saudi German Hospital
Dr. Haitham Elsayed Pediatrics | Gender: Male | Experience: 10 years | Hospital: Saudi German Hospital
Dr. Eman Alayadi Salem Pediatrics | Gender: Female | Experience: 15 years | Hospital: Saudi German Hospital
Dr. Younis Mohd. Khalid Pediatrics | Gender: Male | Experience: 25 years | Hospital: Saudi German Hospital
Dr. Kamalesh Pal Pediatrics | Gender: Male | Experience: 17 years | Hospital: Saudi German Hospital
Dr. Hala Kamal El Din Ibrahim Pediatrics | Gender: Female | Experience: 25 years | Hospital: Saudi German Hospital
Dr. Mohammed Wajid Chaudhary Pediatrics | Gender: Male | Experience: 19 years | Hospital: NMC Royal AbuDhabi
Dr. Mohamed Laban Pediatrics | Gender: Male | Experience: 12 years | Hospital: NMC Royal AbuDhabi
Dr. Sweta Swaminath Pediatrics | Gender: Female | Experience: 10 years | Hospital: NMC Royal AbuDhabi
Dr. Smita Malhotra Pediatrics | Gender: Female | Experience: 7 years | Hospital: Apollo Indraprastha
Dr. Sevil Bilir Göksügür Pediatrics | Gender: Female | Experience: 10 years | Hospital: Hisar Intercontinental
Dr. Amita Mahajan Pediatrics | Gender: Female | Experience: 25 years | Hospital: Apollo Indraprastha

Top Hospitals For X-Linked Agammaglobulinemia Treatment

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Thumbay Hospital, Dubai With our Hospitals and Medical Centre in Ajman, Fujairah, Sharjah, Dubai and Hyderabad (India), Thum
Maharaja Agrasen Hospital, Bengaluru The Maharaja Agrasen Hospital is located in the heart of Bangalore and is known of its excellence in
Columbia Asia Hospital, Bengaluru Columbia Asia is an international healthcare group operating a chain of modern hospitals across Indi
Gleaneagles Global Hospital, Mumbai Gleneagles Global Hospital, Mumbai offers end-to-end clinical, surgical and diagnostic services. The
Primus Super Speciality Hospital, Delhi Primus Super Speciality Hospital is a state of the art multi specialty hospital,conveniently located
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