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Journals / Süleyman Demirel Üniversitesi Sağlık Bilimleri Dergisi / 2012 / Cilt: 3 - Sayı: 1

Papillon Lefevre Syndromewith Multidiciplinar Approach: 6 years follow up

Multidisipliner yaklaşım ile Papillon Lefevre Sendromu: 6 yıllık takip

Buse SERİN
Muharrem DOĞAN
Volkan ÇİFTÇİ
Onur ÖZÇELİK
Pages
39–44
Publication Date
2012-07-31
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

The patient is still under control.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:This paper presents treatment planning and follows up of 6 year of a child patient with Papillon Lefevre syndrome.
📊
Key Findings & Data:As the result of clinical, immunological and hematological findings, the child patient was diagnosed as having PLS at 2005.
💡
Conclusion & Impact:The patient is still under control.
🔬Design:Research Article
📍Scope:2005 Verileri
⚙️Variables:Papillon Lefevre Sendromu · Juvenil

Abstract

This paper presents treatment planning and follows up of 6 year of a child patient with Papillon Lefevre syndrome. Papillon Lefevre syndrome is a rare autosomal recessive syndrome that is characterized by palmoplanar hyperkeratozis and premature loss of tooth. As the result of clinical, immunological and hematological findings, the child patient was diagnosed as having PLS at 2005. The treatment consisting of extraction of all primary teeth at general anesthesia. After an edentulous period complete denture were inserted. And when permanent teeth were erupted, the denture was modified. Treatment procedure was continued with the distal screw with removal appliance. The patient is still under control.

Özet

Papillon Lefevre sendromlu (PLS) bir çocuk hastanın 6 yıllık tedavi ve takip süreci sunulmuştur. Papillon Lefevre sendromu, palmoplantar hiperkeratoz ve dişlerin erken kaybı ile karakterize, nadir görülen otozomal resesif bir hastalıktır. 2005 yılında kliniğimize yönlendirilen çocuk hastaya klinik, immunolojik ve hematolojik bulguların sonucunda hastaya PLS teşhisi konuluştur. Tedavi olarak genel anestezi altında bütün süt dişleri çekilmiştir. Dişsiz periyodu takiben hastaya çocuk protezi uygulaması yapılmıştır. Ve daimi dişleri çıkmaya başladıkça protezi modifiye edilmiştir. Hastada distal vidalı verenli müteharik aparey ile yer açma tedavisine başlanmıştır. Hasta rutin kontrollerine devam etmektedir.

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Cite This Article

SERİN, B., DOĞAN, M., ÇİFTÇİ, V., ÖZÇELİK, O. (2012). Papillon Lefevre Syndromewith Multidiciplinar Approach: 6 years follow up. Süleyman Demirel Üniversitesi Sağlık Bilimleri Dergisi, Cilt: 3 - Sayı: 1, 39–44. https://ajindex.com/articles/702395

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References (13)

  1. Papillon MN, Lefevre B. Two cases of familial sym- metric palmoplanter keratosis (Maleda’s disease) in a brother and his sister. Alterations in both cases (Fren- ch) Bull Soc Francaise Dermatologie Syphiligraphie 1924; 31: 81–84.
  2. Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmarplantar hyperkeratosis and premature peri- odontal destruction of the teeth. J Pediatr 1964; 65: 895–908.
  3. Hart TC, Bowden DW, Ghaffar KA, et al. Subloca- lization of the Papillon–Lefevre syndrome locus on 11q14–q21. American Journal of Medical Genetics 1998; 79: 134–139.
  4. Hart TC, Walker SJ, Bowden DW. An integrated phy- sical and genetic map of the PLS locus interval on chromosome 11q14. Mammalian Genome 2000; 11: 243–246.
  5. Hart TC, Hart PS, Michalec MD, et al. Localisation of a gene for prepubertal periodontitis to chromosome 11q14 and identification of a cathepsin C gene muta- tion. Journal of Medical Genetics 2000; 37: 95–101.
  6. Dalgıc B, Bukulmez A, Sarı S. Eponym Papillon–Le- fevre syndrome Eur J Pediatr 2011; 170:689–691.
  7. Kressin S, Herforth A, Preis S, Wahn V, Lenard HG. Papillon–Lefèvre syndrome – successful treatment with a combination of retinoid and concurrent syste- matic periodontal therapy: case reports. Quintessence International 1995; 26: 795–803
  8. Lee MR, Wong LCF, Fischer GO. Papillon le–fevre syndrome treated with acitretin. Australasian journal of dermatology. 2005; 46,199–201. Haneke E.The Papillon–Lefevre syndrome: kerato- sis palmoplantaris with periodontopathy. Report of a case and review of cases in the literatüre. Hum Ge- net.1979;51:1–35. de Haar SF, Tigchelaar–Guter W, Everts V, Beertsen W. Structure of the periodontium in cathepsin C–defi- cient mice. Eur j oral sci 2006; 114: 171–173
  9. Özçelik O, Haytaç MC. Papillon–Lefevre Sendromu ile ilişkili periodontitis olgusu: 1 yıllık takip. AÜ Diş Hek. Fak. Derg. 2006; 33(1): 127–133.
  10. Wiebe CB, Hakkinen L, Putnins EE, Walsh P, Lar- java HS. Successful periodontal maintenance of a case with Papillon–Lefevre syndrome: 12–year fol- low–up and review of the literature. J Periodontol 2001;72:824–30.
  11. Hart TC, Shapira L. Papillon– Lefe`vre syndrome. Periodontol 2000 1994;6:88–100.
  12. Firatli E, Tuzun B, Efeoglu A. Papillon– Lefe`vre sy- ndrome. Analysis of neutrophil chemotaxis. J Perio- dontol 1996;67:617–20.
  13. Soskolne WA, Stabholz A, Van Dyke TE, Hart TC & Meyle J. Partial expression of the Papillon–Lefe`vre syndrome in 2 unrelated families.J Clin Periodontol 1996;23:764–9. Lyberg T. Immunological and metabolical stud