Chediak-Higashi Syndrome and Premature Exfoliation of Primary Teeth

Abstract

The Chediak-Higashi syndrome (CHS) is a rare hereditary fatal disease, if not treated. These changes are associated with various diseases and syndromes that mainly cause periodontal disease and thus the premature loss of teeth. This paper describes the monitoring of premature loss of primary teeth that began when the child was 5 years old. On presentation his teeth were mobile and there was a history of gingival bleeding. Panoramic radiography revealed generalized and severe bone loss, and the teeth showed no bony support enough for their stability. Blood test was ordered to assess the overall health of the child and giant cells with cytoplasmic granules were found, confirming the diagnosis of CHS. The management of periodontal disease focused on the control of infection and bacterial plaque by mechanical and chemical methods.

Congenital Adrenal Hyperplasia: A Case Report With Premature Teeth Exfoliation and Bone Resorption

“…24 Moreover, none of the clinical features of Chediak-Higashi syndrome, such as albinism, strabismus, and nystagmus, infections of the skin and respiratory tract, and characteristic alterations in blood cell values and cell morphology, were found in the present patient. 25 Patients with Singleton-Merten syndrome usually present with skeletal and joint defects, glaucoma, and calcification of the aortic arch, whereas Hajdu-Cheney syndrome is characterized by joint problems and multiple osteolytic lesions. 18 None of these defects were apparent in this patient.…”

Innovative Formula for Management of Dermatological Disorders Associated with Chediak Higashi

“…It mainly affects granulocytes as white blood cells (neutrophils, macrophages, lymphocytes and natural killer cells), melanocytes, neurons and platelets. The excretory functions and healing power of the cellular membrane are mainly affected with different degrees [1,2].…”

“…On the histopathological level, intracellular giant cytoplasmic organelles could be detected in relation to the affected cells. Furthermore, aggregation, clumping and coalescence of the latter granules could be detectedcausing precipitations at the peripheral blood vessels or intracellularly [1,2]. While, on the clinical level, the affected patients suffer from various manifestations.…”

“…Death usually occurs due to the recurrent infections, prolonged uncontrolled bleeding and lymphohistocytic infiltration of all organs [1].…”

“…Melanocytes are large dendritic cells which produce melanin and transfer melanosomes to the surrounding keratinocytes. They have several immunological and protecting functions [1,3].…”