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Periodontal status in two siblings with severe congenital neutropenia: diagnosis and mutational analysis of the cases.

BACKGROUND: Severe congenital neutropenia (SCN), also known as Kostmann syndrome, was originally reported as an autosomal recessive disease of neutrophil production. The disease is characterized by a maturation arrest of neutrophil precursors at the promyelocytic stage of differentiation and by extremely low levels of mature neutrophils in peripheral blood. METHODS: A 6-year-old male presented with a complaint of gingival swelling and bleeding, and swelling at the left side of his face. Upon clinical examination, severe inflammation of all gingival tissues was apparent, and a periapical abscess with mobility was noted on the left mandibular second molar. Medical and dental histories revealed numerous recurrent bacterial infections associated with oral and non-oral tissues. His medical history with recurrent infections led us to evaluate his 3-year-old sister to determine the status of her oral health. Inflammation of her oral tissues and recurrent bacterial infections were apparent. Their consanguineous parents were in good health. To assist in identifying possible systemic diseases underlying the inflammatory situation in the siblings, consultations were requested from the Pediatric Hematology Department at Selcuk University and Pediatric Oncology Department at Gulhane Military Medical Academy. RESULTS: Based on absolute neutrophil count (< or =200/mm(3)) and bone marrow aspiration findings consistent with early maturation arrest in myelopoiesis, the cases were diagnosed as SCN. No chromosomal abnormality was detected upon cytogenetic examination. Sequencing analysis also revealed no mutation in the neutrophil elastase or growth factor independent-1 (GFI-1) genes in these patients. Severe periodontal disease, attachment loss, and mobility for over 50% of the deciduous teeth were noted. Within 6 months, the male sibling lost all of his deciduous teeth due to periapical and periodontal infections. His sister presented with tooth mobility for all mandibular incisors. Monthly visits, including scaling, polishing, and 0.2% chlorhexidine digluconate irrigation were performed to support their oral hygiene and to avoid recurrent oral infections. We have been able to stabilize these patients' periodontal conditions during a 2-year follow-up period. CONCLUSION: This case report emphasizes the role of periodontists and pediatric dentists in the diagnosis of diseases linked with neutrophil and other systemic disorders and highlights the need to optimize the health of oral tissues with regular appointments.

Bacterial Infections↗

Dental effects of diet and coca-leaf chewing on two prehistoric cultures of northern Chile.

Two ancient cultures of northern Chile, the Chinchorro (9000-3500 BP) and the Maitas Chiribaya (850-700 BP) were examined for dental pathology in search of possible correlations between dental health, diet, and the cultural practice of coca-leaf chewing. The Chinchorro occupied the river mouth of the Azapa valley, subsisting almost exclusively on a maritime economy. The Maitas Chiribaya, descendants of migrant highlanders, had a rather well-developed agricultural subsistence base. The Chinchorro demonstrated extreme attrition rates and a correspondingly high frequency of periapical abscesses. They were essentially caries-free and enjoyed a moderate antemortem tooth loss frequency. The Maitas Chiribaya suffered light attrition; a high caries frequency, especially at the cementoenamel junction of crown and root, and a remarkably high antemortem tooth loss frequency. The cultural practice of coca-leaf chewing is implicated in the excessive posterior edentulism of the Maitas Chiribaya.

Adult↗

Brief communication: prehistoric dentistry in the American southwest: a drilled canine from Sky Aerie, Colorado.

A prehistoric Native American mandible from a Fremont site (circa AD 1025) in Colorado has a conical pit in the worn occlusal surface of the lower right canine. Natural causes for this modification are ruled out by the presence of internal striae, a finding confirmed by experimental replication. The canine was artificially drilled before the individual's death and is associated with a periapical abscess. This is one of a very few examples of prehistoric dentistry in the world, and the first from the American Southwest.

Adult↗

Differential diagnosis of rib lesions: a case study from Middle Woodland southern Ontario circa 230 A.D.

The right ribs of an adult male (#18) from the LeVesconte Burial Mound in southern Ontario show extensive osteolytic and/or osteobastic lesions, some with bone thickening, interpreted herein as evidence of a chronic actinomycotic infection. In the alveolus, the presence of periapical abscesses and periodonitis with secondary inflammation may be associated with the pathogenesis of this bacterial disease by providing the normally commensurate Actinomyces israelii with the necessary conditions for their reproduction. This presumptive diagnosis, if correct, appears to represent the first case of actinomycosis in human palaeopathology. Recent literature reviews suggest that actinomycosis was not uncommon in preantibiotic populations, and that it has a high predilection for bone. Though actinomycosis can be associated with any subsistence strategy, such as, in this case, hunting and gathering, hypothetically it is most likely to occur in agricultural populations where general disease stress and age-adjusted dental pathology are highest. It is argued that in the differential diagnosis of archaeological specimens, as much attention should be given to the documentation of the natural range of variation of lesions produced by less well-known diseases, such as actinomycosis, since the disease ecology of pre-western populations differed significantly from our own.

Actinomycosis↗

Inflammation of rat molar pulp and periodontium causes increased calcitonin gene-related peptide and axonal sprouting.

We have studied the response of nerve fibers containing calcitonin gene-related peptide immunoreactivity (CGRP-IR) to inflammation using a rate dental experimental system. Inflammation was induced by drilling tooth cusps to create pulpal exposures; the induced pulpitis and subsequent periapical lesions were studied 1-35 days later using standard CGRP immunohistochemistry and the avidin-biotin peroxidase method. The injury and resulting inflammation caused a disruption of CGRP-IR nerve fiber location and arborization that varied depending on whether the initial injury was limited to the pulp tip or extended throughout the pulp horn. At shorter survival periods (24 hr, 3 days) nerve fibers were either decreased or bundled into the center of the pulp with sprouting along the wound border. At 6 days necrosis and acute inflammation had advanced to varying degrees, and CGRP-IR fibers were extensively sprouted in the surviving pulp; the pulp also stained specifically for CGRP within 1-2 mm of the inflamed tissue at 6 days. At 35 days, we found total pulp necrosis in most teeth and the development of periapical bone loss, granulomatous tissue, and periapical abscesses. There was also an extensive increase in CGRP-IR nerve fibers in the tissues surrounding sites of severe periodontal inflammation and necrosis. In some cases, macrophage-like cells staining specifically for CGRP were near the abscesses. The results show important interactions between peptidergic nerve fibers and inflammatory cells, and are discussed in terms of the role of nerve fibers containing CGRP in neurogenic inflammation, mechanisms for intensification of CGRP immunoreactivity in affected fibers or neighboring cells, and implications for chronic inflammatory conditions, dental pain, and anesthesia.

Animals↗

[Partsch's chronic granulomatous inflammation, the cutaneous manifestation of a dental cause].

Dentogenous inflammatory diseases can lead to typical dermatological facial symptoms with formation of cutaneous sinuses. Partsch's chronic granulomatous inflammation can result from conducted inflammation of a nonvital tooth via a chronic apical inflammation. In this rare disease, the granulomatous tissue perforates the bone, channels through the overlying skin, and drains via cutaneous or oral sinuses. A frequent localization of the cutaneous sinus is the skin inferior to the body of the mandible, and it is caused by an inflammation of the lower molars. Treatment consists of identifying the responsible teeth and eliminating the focus of infection. Chronically progressive periradicular granuloma and/or radicular cysts can be present with impressive dermatological symptoms. Therefore, X-ray examinations are necessary to exclude possible dentogenic causes in cases of badly healing processes of the face or neck.

Adult↗

Occult dental infection as a cause of fever of obscure origin.

Three patients with prolonged unexplained fevers were ultimately found to have deep-seated dental infection. After initial examination failed to elicit symptoms or signs of dental infection, and extensive in-hospital evaluation was nonproductive, dental consultation with roentgenograms provided the diagnosis. All three patients underwent dental extractions with periapical or peridontal debridement; following a brief postoperative febrile period, all three responded with defervescence, without subsequent recurrence of fever. These cases emphasize the importance of periapical and peridontal infection as causes of fever of obscure origin. The pathogenesis, characteristics and bacteriology of periapical abscess are discussed.

Aged↗