Periodontal Status in Two Siblings With Severe Congenital Neutropenia: Diagnosis and Mutational Analysis of the Cases

Hakkı, Sema S.; Aprikyan, Andrew A.G.; Yıldırım, Sibel; Aydınbelge, Mustafa; Gökalp, Alparslan; Uçar, Canan; Guran, Sefik; Köseoğlu, Vedat; Ataoğlu, Tamer; Somerman, Martha J.

doi:10.1902/jop.2005.76.5.837

Cited by 31 publications

(15 citation statements)

References 33 publications

(29 reference statements)

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“…Existen, aproximadamente 1-2 casos por millón y afecta principalmente a niños sin predilección por ningún sexo 23 . Debido al gran descenso de neutrófilos circulantes, las defensas de estos pacientes están muy disminuidas por lo que están expuestos a diferentes infecciones como celulitis, estomatitis, meningitis, neumonía y sepsis.…”

Section: Neutropenia Severa Congé-nita (Nsc)unclassified

Alteraciones gingivales no relacionadas con placa

Palacios-Sánchez¹,

Cerero-Lapiedra

Campo‐Trapero

et al. 2006

RCOE

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Resumen: El depósito de placa bacteriana constituye el principal factor etioló-gico de las alteraciones gingivales; sin embargo se debe tener en cuenta que las lesiones gingivales pueden ser la manifestación de múltiples procesos en los cuales la placa no constituye el factor principal. Debido a la importancia que pueden tener algunos de estos procesos y a que las lesiones gingivales pueden ser un signo temprano que pueden ayudar a establecer un diagnóstico precoz, se ha propuesto hacer una revisión de los mismos destacando las alteraciones gingivales que aparecen relacionadas con enfermedades mucocutáneas, con niveles hormonales, con los tratamientos farmacológicos, con las infecciones víricas, con las discrasias sanguíneas y, por último, las alteraciones gingivales de origen genético o fibromatosis gingival hereditaria. Palabras clave: Encía, Gingivitis descamativa crónica, Hiperplasia gingival.Abstract: Dental plaque remains the main aetiological factor of gingival diseases; nevertheless, a number of gingival lesions could be the expression of other, non-plaque related entities. The aim of this paper is to perform a review and underscore the importance of some of these diseases, since they can be an early and useful sign in order to establish an accurate diagnosis. The main entities are: gingival lesions related to mucocutaneous diseases, gingival lesions related to hormonal levels, gingival alterations produced by pharmacological treatments, gingival lesions produced by viral infections, gingival alterations related to haematological disorders and, finally, gingival alterations of genetic origin such as gingival fibromatosis.

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Section: Neutropenia Severa Congé-nita (Nsc)unclassified

Alteraciones gingivales no relacionadas con placa

Palacios-Sánchez¹,

Cerero-Lapiedra

Campo‐Trapero

et al. 2006

RCOE

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“…Patients with the congenital disease Kostmann's syndrome, characterized by neutropenia and frequent and severe infections, have either no or extremely low levels of LL‐37 in plasma and saliva . Interestingly, these patients also suffer from gingivitis and periodontitis, suggesting that LL‐37 is relevant for the in‐vivo situation, although it should be considered that the low plasma and salivary concentrations of LL‐37 are secondary to the lack of neutrophils .…”

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confidence: 99%

Antimicrobial peptide LL‐37 and its pro‐form, hCAP18, in desquamated epithelial cells of human whole saliva

Aidoukovitch

Dahl

Fält

et al. 2019

European J Oral Sciences

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The antimicrobial peptide LL‐37 is active against oral bacteria and has been demonstrated to be present in human saliva, but its distribution in different fractions of saliva is not known. LL‐37 is formed from its intracellular pro‐form, hCAP18, in an extracellular enzymatic reaction catalyzed by proteinase 3 and kallikrein 5. Here, we prepared cell‐containing and cell‐free fractions of unstimulated human whole saliva by centrifugation after depolymerization of mucins with dithiothreitol, and measured the levels of hCAP18/LL‐37 in these fractions using ELISA. Cellular expression of hCAP18/LL‐37 was determined by western blotting and immunocytochemistry. The ELISA analyses demonstrated that both cells and cell‐free saliva contained hCAP18/LL‐37. Western blot analysis of cell‐pellet homogenates showed a strong band corresponding to hCAP18 at the correct molecular weight and a weak band corresponding to LL‐37. Phase‐contrast and light microscopy revealed that the cells consisted of desquamated epithelial cells. These cells expressed cytoplasmic immunoreactivity for hCAP18/LL‐37. The peripheral part of the cytoplasm, corresponding to the plasma membrane, was particularly rich in hCAP18/LL‐37 immunoreactivity. No immunoreactivity was observed after omission of the primary antibody. We conclude that desquamated epithelial cells of human whole saliva contain antimicrobial hCAP18/LL‐37, suggesting that these cells may take part in the innate immune system by harboring and releasing these peptides.

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“…Le parodonte est rapidement détruit et l'évolution est en corrélation avec l'attaque bactérienne due à l'insuffisance de peptides antibactériens dans le sang et la salive. Une atteinte sévère parodontale, une perte d'attache et une mobilité de plus de 50 % des dents lactéales sont rapportées par différents auteurs [8].…”

Section: Syndrome De Kostmann Et Maladie Parodontaleunclassified

“…La mise en place de cette antibioprophylaxie contribue au maintien de l'état parodontal. L'action chimique s'additionne à l'action physique [8]. Des bains de bouche à la chlorexidine à 0,2 % deux fois par jour associés à un brossage avec du bicarbonate de soude associé à de l'eau oxygénée sont indispensables pour maintenir la santé bucco-dentaire de ces enfants.…”

Section: Prise En Charge Parodontale Du Syndrome De Kostmannunclassified

“…Des bains de bouche à la chlorexidine à 0,2 % deux fois par jour associés à un brossage avec du bicarbonate de soude associé à de l'eau oxygénée sont indispensables pour maintenir la santé bucco-dentaire de ces enfants. Ainsi, Hakki et al ont réussi, au bout de deux ans de suivi régulier, à stabiliser l'état parodontal de leurs patients [8].…”

Section: Prise En Charge Parodontale Du Syndrome De Kostmannunclassified

See 1 more Smart Citation

Syndrome de Kostmann et cavité buccale : présentation d’un cas et revue de la littérature

Boisramé

Lelièvre

Moine

et al. 2010

Med Buccale Chir Buccale

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Periodontal Status in Two Siblings With Severe Congenital Neutropenia: Diagnosis and Mutational Analysis of the Cases

Abstract: 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.

Cited by 31 publications

References 33 publications

Alteraciones gingivales no relacionadas con placa

Alteraciones gingivales no relacionadas con placa

Antimicrobial peptide LL‐37 and its pro‐form, hCAP18, in desquamated epithelial cells of human whole saliva

Syndrome de Kostmann et cavité buccale : présentation d’un cas et revue de la littérature

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