Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper reviews the literature on prepubertal periodontitis (PP) according to the definition and the classification of PP by Page. The generalized form of PP (G-PP) defined by Page was identified as the oral manifestation of a systemic disease called leukocyte adhesion deficiency (LAD) and thus the pathogenesis is known at the molecular level. On the other hand, the localized form of PP (L-PP) is a disease defined by clinical criteria and its pathogenesis is not known at the molecular level. The reported prevalence of L-PP differs widely in reports, according to the methodology and population studied. However, it seems to be at least 0.84% or greater. Potential periodontal pathogens that have been associated with L-PP include the following bacteria: Actinobacillus actinomycetemcomitans (A.a.), Bacteroides intermedius, Bacteroides gingivalis, Capnocytophaga sputigena, and Eikenella corrodens. Suggested contributing factors have included PMN or monocyte chemotactic defects and cementum defects. There is no unique pattern of bone loss in L-PP; however, there may be a type of periodontitis that affects all deciduous teeth, in the absence of LAD. L-PP may lead to localized juvenile periodontitis (LJP) or generalized juvenile periodontitis (GJP). Histiocytosis X, hypophosphatasia and mild forms of blood dyscrasias may have minimal or no clinical signs and symptoms except for alveolar bone loss. Therefore diseases known to be associated with alveolar bone loss must be definitively excluded in prepubertal children until the pathogenesis of L-PP at the cellular and molecular levels is understood and L-PP can be definitively diagnosed either as a distinct disease entity or entities.
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PMID:Prepubertal periodontitis: a review of diagnostic criteria, pathogenesis, and differential diagnosis. 213 70

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.
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PMID:Oral manifestion of Langerhans cell histiocytosis mimicking inflammation. 2499 57

Premature exfoliation of primary teeth is an important diagnostic event warranting urgent investigation. The majority of conditions presenting with early loss of teeth are serious and in some cases could be fatal. The most common causes of premature tooth loss are Papillion-Lefevre syndrome, Chediak-Higashi syndrome, hypophosphatasia, neutropenia, leukemia and in some cases Langerhans cell histiocytosis (LCH). LCH is a disorder of unknown cause, characterized by abnormal proliferation of histiocytes. The disease has a predilection for children, although LCH may occur in adults. Owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed or missed and in which many questions remain unanswered, ranging from etiology and pathogenesis to therapy. The purpose of the review is, therefore, to raise awareness of the disease and to highlight the clinical findings that should make the odontologist or primary caregiver suspect the diagnosis.
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PMID:Premature loss of primary teeth with gingival erythema: An alert to dentist. 2660 20