“Oral Manifestations of Patients with Inherited Defect in Phagocyte Number or Function” a systematic review

Ziaei, Heliya; Tonkaboni, Arghavan; Shamshiri, Ahmadreza; Rezaei, Nima

doi:10.1016/j.clim.2021.108796

Cited by 2 publications

(4 citation statements)

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“…To our knowledge, this is the first case of extensive caries and periodontal disease in a patient with GATA2 deficiency. Therefore, this report expands the spectrum of the oral manifestations of this disorder, which already include oral ulcers and recurrent fungal, HSV and HPV infections, that may eventually lead to squamous cell carcinoma ( 1 , 5 , 12 , 13 ).…”

Section: Discussionmentioning

confidence: 81%

“…Despite the severity of the dental Tableau, an early surgical approach with extraction of the affected teeth with scaling and root planning of the alveolar sockets allowed to reduce the risk of life-threatening oral infections, especially throughout the prolonged phase of post-induction aplasia during HSCT. This suggests that pre-transplant work-up should regularly include a dental evaluation and eventually a preventive surgical intervention, especially in children more prone to develop oral disease, such as those affected by IEI ( 5 , 6 , 8 ).…”

Section: Discussionmentioning

confidence: 99%

“…Among this pleiotropic clinical heterogeneity, oral manifestations due to fungal or viral infections are not rare. Similarly, dental anomalies frequently occur in other IEI ( 5 - 10 ). However, dental caries have never been specifically described in GATA2 deficiency ( 1 , 3 , 5 , 6 ).…”

Section: Introductionmentioning

confidence: 98%

“…Similarly, dental anomalies frequently occur in other IEI ( 5 - 10 ). However, dental caries have never been specifically described in GATA2 deficiency ( 1 , 3 , 5 , 6 ). Herein, we report a child with GATA2 deficiency, who presented to our Dentistry unit for an evaluation in the context of AML.…”

Section: Introductionmentioning

confidence: 98%

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Extensive dental caries and periodontal disease in a child with GATA2 deficiency

Consonni,

Gambineri,

Veltroni

et al. 2023

J Clin Exp Dent

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Background GATA2 deficiency is an inborn error of immunity (IEI) characterized by infectious susceptibility and increased risk of myelodysplasia leading to acute myeloid leukaemia (AML). Oral anomalies already described in this disorder include recurrent viral and fungal infections and oral ulcers. Material and Methods We report a 9-year-old girl presenting with AML with myelodysplasia-related changes, monosomy 7 karyotype on marrow aspirate, numerous flat warts on her hands and multiple dental caries at oral cavity inspection. Dental evaluation and genetic testing (Sanger sequencing) for GATA2 were carried out considering the peculiar clinical presentation. Results Dental evaluation showed extensive caries and periodontal disease, while genetic studies revealed a known c.1009 C>T (p.Arg337X) mutation in GATA2. After multidisciplinary discussion, affected teeth were extracted before chemotherapy, in general anaesthesia, together with scaling and root planning of the alveolar sockets. Subsequently, the patient underwent hematopoietic stem cell transplantation (HSCT) from her HLA-matched GATA2 wild-type sibling, who did not bear any dental anomalies. No dento-alveolar infections were encountered during post-chemotherapy aplasia. Conclusions This case first describes the association between GATA2 deficiency and extensive dental caries with periodontal disease, highlighting the importance of an early dental evaluation and intervention in children with leukaemia. Key words: GATA2 deficiency, Inborn errors of immunity, teeth, dental decay, multidisciplinary approach.

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Section: Discussionmentioning

confidence: 81%