X linked agammaglobulinemia #Bruton Agammaglobulinemia #Ayurveda in Agammaglobulinemia #Ayurvedic management

1. Short Note on Bruton
Agammaglobulinemia
Presented By : Dr. Vijay Kumar Pathak

2. • Bruton agammaglobulinemia is X-linked inherited immunodeficiency
disease.
• It is caused by mutations in the gene coding for Bruton tyrosine kinase
(BTK)
• The disease was first elucidated by Bruton in 1952, for whom the gene is
named.
• Also known as X-linked agammaglobulinemia (XLA).
mature B cells
Pre–B
cells
BTK

3. INHERITANCE

4. PATHOPHYSIOLOGY
mature B cells
Pre–B
cells
BTK
antibody-
producing
plasma
cells
Poorly
developed
Lymphoid
organ
Spleen, the tonsils, the
adenoids, the peyer
patches in the
intestines, and the
peripheral lymph nodes
may all be reduced in
size or absent

5. XLA is an inherited disease that occurs in approximately 1 in 250,000 males.
Female carriers have no clinical manifestations.
Infections begin once transferred maternal immunoglobulin G (IgG) antibodies have
been catabolized, typically at about 6 months of age.
Baby has small or absent tonsils and lymph nodes with recurrent or severe bacterial
infections.
Children typically clinically manifest the disease at age 3-9 months with pneumonia,
otitis media, cellulitis, meningitis, osteomyelitis, diarrhea, or sepsis.
Rare cases of adults in their second decade have been diagnosed with a milder form

6. 1. Immunoglobulins (IgG, IgM and IgA) are markedly reduced or absent.
2. Abnormally low or absent numbers of mature B lymphocytes,
3. Absence of BTK ribonucleic acid (RNA) or protein.
4. Rarely, the diagnosis is made in adults in their second decade of life. This is
thought to be due to a mutation in the protein, rather than a complete absence.

7. Medical Care
No curative therapy exists for X-linked agammaglobulinemia (XLA), or Bruton
agammaglobulinemia.
Treatment for XLA is IVIG. Typical doses are 400-600 mg/kg given every 3-4 weeks.
Doses and intervals can be adjusted based on individual clinical responses.
Therapy should begin at age 10-12 weeks.
Antibiotics, such as amoxicillin and amoxicillin/clavulanate, are administered for
common sinopulmonary infections.
Nutritional supplementation with multivitamins is recommended.

8. AYURVEDA VIEW
Inappropriate Ritu (Fertile period), Kshetra (Uterus), Ambu (Ahara rasa nutrients) and Bija (sperm
and ovum), Dauhrida Avamanana (negligence of urges during Dauhrida stage of pregnant
women), Garbhopaghatkarbhava (Dont’s in Antenatal period), incompatible Garbha
Vriddhikarabhava (embryonic growth factors) and improper following of Garbhini Paricharya
Beejdushti (Abnormality in Gene / Mutation)
Khavagunya (Organs predisposed to disease)
Dosha Sthan sanshraya (Poorly developed Lymphoid organ)
Vyadhi (Bruton agammaglobulinemia)

9. AYURVEDIC MANAGEMENT
Line of treatment might be
• Oja vardhan
• Agni deepan
• Ayurvedic immune inhancing drugs example swarnaprashan
• Vaat anuloman
• Based upon clinical sign and symptoms management is done

10. THANK YOU