DENOSUMAB TREATMENT IN A SPORADIC CASE OF CHERUBISM: A RARE PEDIATRIC FIBRO-OSSEOUS DISORDER – A SYSTEMATIC REVIEW
Main Article Content
Keywords
bone regeneration; pediatrics; bone lesions; genetics; giant cell lesions of the jaws; skeletal pathology.
Abstract
Background
Fibrous connective tissue and osseous components replace normal bone in a variety of histologically benign conditions known as fibro-osseous lesions. The jaw may develop a number of conditions, such as fibrous dysplasia, ossifying fibroma, and cemento-osseous dysplasias. Cherubism is a rare, autosomal dominant, non-neoplastic fibro-osseous hereditary condition caused by genetic mutations that is characterized by symmetrical expansion of the mandible and/or maxilla. It typically affects children between the ages of two and five, giving them a cherubic appearance and an upward turning of the eyes. After puberty, these lesions spontaneously recede, having progressively grown over the following few years. The diagnosis is strongly suggested by bilateral, somewhat symmetric jaw involvement restricted to the maxilla and mandible, although the histopathologic features of cherubism are not pathognomonic. Patients with cherubism who had calcitonin treatment experienced a range of outcomes, from no improvement to a noticeable improvement. If we believe that calcitonin has some effectiveness, the disparity could be explained by variations in the length of treatment, the dosage, the patient's age, and most likely the severity of cherubism. Jaw-bone sclerosis was a remarkable side effect of denosumab treatment, regardless of the dosage, duration, age, and likely severity of cherubism. Bisphosphonates have been used for decades to try to change bone turnover because they can reduce pain and stop more bone involvement or damage in high-turnover disorders such as Paget disease. Bisphosphonates have not, however, been demonstrated to be useful in reducing disease activity, lesion size, or correcting abnormalities in many RBDs. Although denosumab's effects are reversible upon stopping treatment, it may provide a quicker and more efficient reduction of bone turnover.
Material and Methods: Major databases such as Medline were explored detailed literature search in resulting in a systematic review pertaining to denosumab treatment in a sporadic case of cherubism: a rare pediatric fibro-osseous disorder.
Results: Five original research scientific articles dated between 2020 – 2024 pertaining to mentioned topic were highlighted.
Conclusions:
Both radiological and clinical improvements were noted after treatment. Nonetheless, there were clinically substantial calcium homeostasis disruptions that needed to be treated medically. This example emphasizes the significance of customized dosage, organized biochemical surveillance, and long-term follow-up while highlighting the possible effectiveness of denosumab in specific juvenile patients with severe cherubism. Detailed information regarding the denosumab treatment in a sporadic case of cherubism: a rare pediatric fibro-osseous disorder is discussed in this systematic review.
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Dr.N.Inbanesan
Reader in Department of OMFS Karpaga vinayaga Institute of Dental sciences, Tamilnadu, India,
