A Turkish family with xerostomiaBahadır Ceylan1, Aydın Mazlum2, Aslı Çurgunlu3, Cengiz Çelikyurt4, Şule Ceylan5
1SSK Istanbul Hospital of Infectious Diseases, Istanbul
2Safa Hospital Internal Medicine Clinic, Istanbul
3Faculty of Medicine, Internal Medicine Clinic, Istanbul
4Safa Hospital ENT Clinic, Istanbul
5Department of Nuclear Medicine, Marmara University, Istanbul
Xerostomia is an important symptom which disrupts the quality of life very seriously, and may be the major symptom of Sjogrens syndrom (SS). The etiology for SS remains unknown where multifactorial influences contribute to the pathogenesis of subsequent development of the disease.Keywords: Xerostomia
In this letter we report a Turkish family with xerostomia whose genetic susceptibility to SS followed a mendelian dominant trait. This family contain one grandfather (age: 70), 5
children (age: 20-36) and 6 grandchidren (age: 5-9). Grandfather, four of five chidren and two of six grandchidren had xerostomia. Seven member of the family reported the following: Inability to eat dry food, putting a glass of water on their bed stand to drink at night, difficulty speaking for long periods of time, higher incidense of dental caries and periodontal disease. All cases stated that his complaints of dry mouth was
started at the age of 3 after a common cold. Physical examination revealed dental caries and periodontal disease. The routine laboratory tests were within normal limits and RF,
ANA, SS-A (anti-RO), SS-B (anti-LA) was negative. In all patients we found those an unstimulated whole mouth salive flow rate of < 0.1 ml/min, Rose Bengal test was positive and Schirmer test was negative. Glandulary stricture and inflamatory cells wasnt seen in labial biopsy. Our patient had two of Fox criteria.
It seems to be a genetic predisposition for the SS in some cases. Immunogenetic studies have demonstrated that HLA-B8, - DR-3, and DRw52 are prevalent in primary Sjogrens syndrome patients as compared with the normal control population.
On the other hand, family studies indicated the presence of an additional autosomal dominant gene(s), not linked to HLA, in predisposition to SS. Possesion of the necessary genetic factors doesnt invariably lead to clinical disease. The cumulative effect of interactive genetic (MHC and non MHC) and nongenetic influences (virus infection) may dictate protective and/or susceptibility factors in disease expression. In conclusion, xerostomia may show familial occurens and other family members are asked for this symptom.
Bir ailede kserostomili 7 olgunun irdelenmesiBahadır Ceylan1, Aydın Mazlum2, Aslı Çurgunlu3, Cengiz Çelikyurt4, Şule Ceylan5
1SSK İstanbul Eğitim Hastanesi İnfeksiyon Hastalıkları Kliniği, İstanbul
2Safa Hastanesi İç Hastalıkları Kliniği, İstanbul
3Cerrahpaşa Tıp Fakültesi, İç Hastalıkları Kliniği, İstanbul
4Safa Hastanesi Kulak Burun Boğaz Kliniği, İstanbul
5Marmara Üniversitesi Nükleer Tıp Anabilim Dalı, İstanbul
Anahtar Kelimeler: Kserostomi
Bahadır Ceylan, Aydın Mazlum, Aslı Çurgunlu, Cengiz Çelikyurt, Şule Ceylan. A Turkish family with xerostomia. Medeniyet Med J. 2004; 19(2): 109-110
Corresponding Author: Bahadır Ceylan, Türkiye