Medhini Madi

Goldenhar syndrome: Report of two cases with review of literature

Goldenhar syndrome consists of a varied group of malformations that can involve multiple systems of the body. It is believed to be a variant of hemifacial microsomia with ocular and vertebral involvement. Characteristic findings, such as hypoplasia of one half of the face, epibulbar dermoids, ear tags, and spinal cord defects, warrant the name occulo-auriculo-vertebral dysplasia. The syndrome occurs due to imbalance in cells during the blastogenesis period of embryo formation. It is found to involve the derivatives of first and second branchial arches. The condition is apparent at birth, but the phenotype can vary greatly in its severity depending on the activation and expression of the defective gene. Reported here are detailed clinical and radiographic features of two sporadic cases of Goldenhar syndrome in young males. This work mainly highlights the various theories of etiopathogenesis as well as step-wise management protocol for patients diagnosed with the syndrome.

Status of thiocyanate levels in the serum and saliva of non-smokers, ex-smokers and smokers

Background Use of tobacco is often implicated in the development of oral diseases. Questionable accuracy of the traditional questionnaires to assess cigarette exposure necessitates the use of biomarkers like thiocyanate which provide a definitive quantitative measure. Objective To assess the rise in the level of thiocyanate for measurement of smoking behaviour in adults. Materials and methods Serum and salivary thiocyanate levels were estimated in 20 non-smokers, 20 ex-smokers and 40 smokers. Smokers were divided into two groups based on the presence or absence of oral mucosal lesions. Results The mean serum and salivary thiocyanate levels were increased significantly in smokers when compared to non-smokers and ex-smokers. The levels were not significantly different between ex-smokers and non-smokers and between smokers with tobacco related oral mucosal lesions and those without. Statistically significant correlation was seen between the serum and salivary levels of thiocyanate. Conclusion This study highlights the high level of thiocyanate in the serum and saliva of smokers when compared to non-smokers and ex-smokers. Significant increase in thiocyanate level was also seen in saliva. Hence it can be stated that saliva can be used as a reliable, non-invasive tool to assess smoking behaviour in the population and its changes over time.

Status of serum and salivary ascorbic acid in oral potentially malignant disorders and oral cancer

Background and Aims: The aim of this study was to estimate the detoxification status of serum and saliva by assessing the serum and salivary Vitamin C in oral potentially malignant disorders and oral cancer. Subjects and Methods: A total of 90 subjects, 30 subjects with oral potentially malignant disorders, 30 subjects with oral cancer, and 30 healthy subjects (controls) were included in the study. Serum and saliva were collected and levels of Vitamin C were assessed. Data obtained was analyzed using ANOVA test for the comparison between the groups. Post hoc Tukeys analysis was used for the comparison of the two study groups to the control group. Correlation between the groups was done using Pearsons correlation coefficient test. Results: The mean serum and salivary Vitamin C levels were decreased significantly in potentially malignant disorders and oral cancer when compared to healthy subjects. Conclusions: As significant reduction of Vitamin C is seen in saliva, it can be stated that saliva can be used as a reliable, noninvasive biomarker in diagnosis and management of potentially malignant disorders and oral cancer.

Sialic acid as a biomarker of oral potentially malignant disorders and oral cancer

Background: Carcinogenesis is a multistep process where a healthy cell has initially a precancerous stage and finally an early cancerous stage. The process of carcinogenesis can be divided into three stages of initiation, promotion, and progression. In this process, there is increased turnover, secretion, and/or shedding from malignant cells. Glycoproteins like sialic acid are expressed on the cell surface. In oral potentially malignant disorders (OPMDs) and oral cancer (OC), the sialic acid level is seen to increase due to high cell turnover and shedding of malignant cells which, in turn, results in the release of glycoproteins like sialic acid into circulation. Glycoproteins also form an important constituent of salivary mucins and hence due to the same mechanism, an increase in sialic acid level is also seen in saliva. Objective: The aim is to estimate serum and salivary sialic acid levels in healthy controls, patients with OPMDs and patients with OC. Materials and Methods: In this observational cross-sectional study, serum and salivary sialic acid levels were estimated in thirty healthy controls, thirty patients with OPMDs and thirty patients with OC. Results: Serum and salivary sialic acid levels obtained were subjected to statistical analysis. Post hoc Tukey test was used to compare the serum and salivary sialic acid levels of the two study groups to the control group. ANOVA test was used for the comparison of sialic acid levels between the groups. Pearsons correlation coefficient was used to assess correlation (P < 0.05 was considered statistically significant). The mean serum and salivary sialic acid levels were increased significantly in subjects with OPMDs and OC when compared to healthy controls. Conclusion: This study highlights the high expression of sialic acid on outer cell membranes, due to the significant increase in subjects with OPMDs and OC when compared to healthy controls. A significant increase in sialic acid level is also seen in saliva. Hence, it can be stated that saliva can be used as a reliable, noninvasive tool in diagnosis and management of OPMDs and OC.

Sıradışı bir yerde meydana gelen keratokistik odontojenik tümör

Keratokistik odontenik tumor, daha once Odontojenik Keratosist olarak bilinen, cenenin iyi huylu bir intraosseoz tumorudur. Maksiller tutulum literaturde az sayida olgu ile belgelenmis olup, cok sik karsilasilmayan bir durumdur. Bu yazida anterior maksillada keratokistik odontojenik tumorlu 35 yasinda bir erkek hasta sunulmustur.

Bir fibro-kemik lezyonunu taklit eden desmoplastik ameloblastoma

Desmoplastik ameloblastoma farkli klinik, radyografik ve histopatolojik ozellikler gosterir. Kapsamli stroma kolonizasyonu veya desmoplaziyi iceren olagandisi histomorfoloji ile karakterizedir. Tumorun karisik radyoopak-radyolusent gorunumu, taniyi karmasik hale getirir ve tedavi modalitesinde tedavide gecikmeye veya aggresivite eksikligine neden olur. Bu maksillerde ortaya cikma egilimi ve infiltratif dogasi ile birlikte desmoplastik ameloblastoma yanlis teshis icin tehlikeli bir lezyon olusturur. Burada fibro-osseoz lezyonun klinik ve radyografik ozelliklerini gosteren, bu nedenle histopatolojik dogrulama gerektiren bir desmoplastik ameloblastom olgusunu sunuyoruz. Hint alt kitasinda bildirilen vakalarin azligi ile bu alisilmadik lezyonla ilgili bilgi birikimimizi iyilestirmeyi ve erken ve dogru teshisinin onemini ortaya koymayi umuyoruz.

Siblings with pierre robin sequence

Pierre Robin anomalad is a syndrome characterized by cleft of the palate, micrognathia of the mandible, and glossoptosis. This syndrome can occur as an individual presentation, or it may be associated with a wide variety of syndromes and anomalies. Such patients are burdened with several problems, some obvious and some less so. The most obvious problems such as the psychological difficulties the patients will have to face due to the appearance and associated problems such as feeding and speech difficulties and serious consequences such as airway obstruction. Here, we report two cases of Pierre Robin sequence seen in the same family in two children of the same parents.