Chediak Higashi syndrome is a immunodeficiency disorder seen in children ,

1. z CHÉDIAK HIGASHI SYNDROME
PREPARED BY - @thedentist77

2. z
INTRODUCTION
 Also called Béguez Cesar Syndrome, Chediak Steinbrick
Higashi Syndrome
 It is an autosomal recessive immunodeficiency disorder
characterized by abnormal intracellular proteins transport

3. z
CLINICAL FEATURES
 All races are affected, usually seen in children bellow 5 years
of age
 Immunodeficiency, partial occulocutanous albinism
 Recurrent infections with neutropenia
 Often Fatal in children at terminal phage characterized by
lymphohistocytic lymphoma in multiple organ, in 80% patients
this is precipitated by Epstien Barr Virus Infection
 Anemia may also be present

4. z
Clinical features continued ....
 Secondary infection due to a normal functioning of
Polymorphonuclear leukocytes occur in skin, lungs, respiratory tract
caused by Staphylococcus aureus ,Streptococcus pyogens and
some pneumococcus sps.
 Few patients may survive till the age of adulthood with neurologic
disfunction

5. z
ORAL MANIFESTATIONS
 Ulceration of oral mucosa
 Severe gingivitis, glossitis
 Periodontal breakdown

6. z
Laboratory Findings
 Gaint granules in peripheral circulating leukocytes, marrow
precursors and other cell is the hallmark of this syndrome
 Pancytopenia
 Bacteria with the abnormal granules are found in periodontal
polymorphonuclear leukocytes

7. z
Treatment
 No specific treatment patients may die before 10years of their
age.

8. z
References
 Shafer’s Textbook of Oral Pathology, R. Rajendran and Shiva
Patha Sundharam, 7th ed
 Textbook of Oral Pathology, Anil Govindrao Ghom And
Subhangi Mhaske, 2nd ed