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is recommended to closely consult with in which genes are mutated, there are many doi:10.1002/humu.23968.
the hematologist before proceeding with similarities between the two. For example, 6. Huizing M, Helip-Wooley A, Westbroek W,
this procedure. Further, extractions can both HPS and CHS present with the dys- et al. Disorders of Lysosome-Related Organelle
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be completed safely in patients with HPS, function of lysosomes and LROs, albinism, Biogenesis: Clinical and Molecular Genetics.
provided the clinicians have the necessary bleeding diathesis and altered immune re- Annual Review of Genomics and Human Genet-
ics. 2008;9(1):359-386. doi:10.1146/annurev.ge-
preprocedural blood analysis, medicaments, sponse. 4, 25 Literature demonstrates a high nom.9.081307.164303.
and hemostatic measures available. prevalence of periodontitis in CHS patients, 7. Marks MS, Heijnen HF, Raposo G. Lyso-
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especially in children. 26-28 Therefore, we ex- some-related organelles: unusual compartments
Infection and Pre-Cancerous Screening pected the presence of severe periodontitis become mainstream. Current Opinion in Cell
HPS impacts the function of cytotoxic in our patient. Contrary to our expectation, Biology. 2013;25(4):495-505. doi:10.1016/j.
T-lymphocytes, making patients more sus- the periodontal condition was mild and ceb.2013.04.008.
ceptible to infections. Given that these pa- consistent with the presence of local oral 8. Miller PD. A classification of marginal tissue
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tients could be at increased susceptibility factors (i.e. plaque, calculus). It is possible recession. The International Journal of Peri-
to infection as well as prolonged bleeding, that the mild presentation of the patient’s odontics & Restorative Dentistry. 1985;5(2):8-
providers should carefully plan treatment HPS accounts for the lack of severe peri- 13.
modifications, especially for invasive treat- odontal disease. Due to the patient’s young 9. Turner KA, Missirlian DM. Restoration of
the extremely worn dentition. The Journal
ments. Additionally, practitioners should age, we cannot preclude increased risk for of Prosthetic Dentistry. 1984;52(4):467-474.
consider the use of antibiotic coverage, de- periodontal disease later in life. Therefore, doi:10.1016/0022-3913(84)90326-3.
pending on the invasiveness of the proce- close monitoring of his periodontal health is 10. Feliciano NZ, Rivera E, Agrait E, et al. Her-

dure and the immune status of the patient. highly important. Although epidemiological mansky-Pudlak Syndrome: Dental Management
When there are concerns of infections and studies are desirable to describe oral condi- Considerations. Journal of Dentistry for Chil-
delayed wound healing, providers should re- tions in HPS, it is important to report case dren. 2006;73(1):51-56.
quest a T-cell test to determine the patient’s studies in patients with HPS as well. 11. Kouklakis G, Efremidou EI, Papageorgiou
T-cell count, or absolute neutrophil count MS, et al. Complicated Crohn’s-like colitis, as-
(ANC) to determine if the patient presents CONCLUSIONS sociated with Hermansky-Pudlak syndrome,
with neutropenia, as neutropenia may also Epidemiological studies investigating oral treated with Infliximab: a case report and brief
review of the literature. Journal of Medical Case
occur in these patients. 5, 22 If the ANC value health/conditions in HPS are lacking. We Reports. 2007;1(1). doi:10.1186/1752-1947-1-
is below 500 cells/µL, antibiotic coverage present a HPS case report describing oral 176.
is indicated. An important function of cyto- health/conditions. It is possible that the oral 12. Schinella RA, Greco MA, Cobert BL, et
toxic T-cells is the extra-cellular destruction conditions depend on the severity of the al. Hermansky-Pudlak Syndrome with Granu-
of virally infected or cancerous target cells. HPS. Therefore, a thorough understanding lomatous Colitis. Annals of Internal Medicine.
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This, combined with the aforementioned al- of Hermansky-Pudlak Syndrome is key 1980;92(1):20. doi:10.7326/0003-4819-92-1-20.
binism, could leave these patients more sus- for the appropriate management of these 13. Erzin Y, Cosgun S, Dobrucali A, et al. Com-
ceptible to malignant or premalignant con- patients, especially if they present with se- plicated granulomatous colitis in a patient with
ditions, like actinic cheilitis, squamous cell vere disease. A thorough and detailed med- Hermansky-Pudlak syndrome, successfully
carcinoma, and basal cell carcinoma. As ical history is crucial. Further research is treated with infliximab. Acta Gastro-Enterolog-
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ica Belgica. 2006;69(2):213-216.
such, providers should thoroughly screen required to understand this condition in its 14. Katsanos KH, Roda G, Brygo A, et al. Oral
for these conditions. entirety. Cancer and Oral Precancerous Lesions in Inflam-
matory Bowel Diseases: A Systematic Review.
Renal Complications ACKNOWLEDGEMENTS Journal of Crohn’s and Colitis. 2015;9(11):1043-
Patients with HPS are frequently diagnosed The authors would like to thank Dr. Analia 1052. doi:10.1093/ecco-jcc/jjv122.
with kidney complications, such as renal Veitz-Keenan for her contributions in re- 15. Lauritano D, Boccalari E, Di Stasio D, et
failure secondary to interstitial ceroid depo- viewing the manuscript. al. Prevalence of Oral Lesions and Correlation
sition. In these patients, the use of non-ste- with Intestinal Symptoms of Inflammatory Bow-
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roidal anti-inflammatory drugs (NSAIDs) is REFERENCES el Disease: A Systematic Review. Diagnostics.
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presses complaints of impaired breathing, 3. De Jesus Rojas W, Young LR. Herman- lecular Medicine. 2002;2(5):451-467.
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patient should also be seated in an upright and Critical Care Medicine. 2020;41(2):238- 18. Russeau AP, Vall H, Manna B. Bleeding
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Research investigating the association be- 24 [updated 2017 Oct 26]. In: Adam MP, Ar- of patients with inherited bleeding disor-
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this disease. Chédiak-Higashi Syndrome Washington, Seattle; 1993–2020. 20. Balshi TJ, Hernandez R, Shapiro SA. Osse-
(CHS) is another genetic disorder present- 5.Huizing M, Malicdan MCV, Wang JA, et al. ointegrated Implants in a Patient With Herman-
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