Orofacial clefts and risk factors in Tehran, Iran: a case control study

Non-syndromic cleft lip with or without cleft palate [CL/P] or cleft palate only [CPO] are orofacial clefts with multifactorial etiology. These include environmental factors and heterogeneous genetic background. Therefore, studies on different and homogenous populations can be useful in detecting related factors. The aim of the present study was to evaluate the risk factors in patients with non-syndromic cleft in Tehran, Iran. Data from 300 patients and 300 controls were collected between 2005 and 2010. Binary logistic regression analyses were used to calculate relative risk by odds ratio [OR] and%95 confidence interval. Low maternal age [OR=1.06, 95% Cl, 1.011-1.113], low socioeconomic status [OR=0.23, 95% Cl, 0.007-0.074], maternal systemic disease [OR=0.364; 95% Cl, 0.152-0.873] and passive smoking [OR=0.613, 95% Cl, 0.430-0.874] increased the risk for CL/P and CPO. There was a significant difference in iron and folic acid use during pregnancy when the case and control groups were compared. In assessing for orofacial cleft risk, we should consider lack of folic acid supplementation use, maternal age and systemic diseases and passive smoking as risk factors

[Alagille syndrome: a case report]

Alagille syndrome is a syndrome associated with decreased number of intrahepatic bile ducts, and various manifestations. The incidence rate is approximately 1 in 100000 births. It is an autosomal dominant disease with variable expression and is caused by mutation in JAG1 or NOTCH2 genes. To make a diagnosis of alagille syndrome, a positive liver biopsy and the presence of three of the following symptoms are usually required: liver symptoms, heart abnormalities, skeletal abnormalities, posterior embryotoxon, typical facial features. We report a 15 month old infant with failure to thrive and increased liver enzyme and cholestasis with peripheral ps and vertebral anomaly and posterior embryotoxon in eye with paucity of intrahepatic bile ducts in liver biopsy, so Alagille syndrome was diagnosed with positive liver biopsy and four major criteria. Then, he was treated with Ursodeoxycholic acid capsule and improved in liver function, growth and development. Alagille syndrome should be considered in patients with hepatic and cardiac involvement, because features in Alagille syndrome are various and some children may present with life threatening conditions whereas others may be subclinical cases

Evaluation of CD31 expression and mast cell count in dysplastic lesions and squamous cell carcinoma of the oral cavity

Squamous cell carcinoma [SCC] is the most common malignancy in the oral cavity. Angiogenesis is essential for development and progression of SCC. Recently, some studies have reported that mast cells play a role in tumor progression, via promoting angiogenesis. The aim of this study was to determine CD31 [an endothelial marker] expression and mast cell count in oral dysplastic lesions and SCC. The CD31 expression and mast cell count were investigated in paraffin-embedded specimens of 10 cases of fibroma [control group], 10 cases of epithelial dysplasia, and 20 cases of SCC. CD31 expression was examined by IHC and mast cell count was evaluated by Giemsa staining. The mean of CD31 expression did not show any significant difference between groups, but in the tumors, peritumoral stroma revealed a significantly higher CD31 expression than intratumoral stroma. A significant difference in the mast cell count was observed between the groups and between peri-and intratumoral stroma of SCCs. The mean of CD31 expression and mast cell count did not show any correlation. Pre-and posttreatment studies and double staining methods are suggested for more definitive results

Immunohistochemical detection and ultrastructure of myofibroblasts in the stroma of odontogenic cysts and ameloblastoma

Immunohistochemical phenotype, distribution and significance of proliferation of myofibroblasts [alpha SMA positive cells] with evaluation of ultrastructure, in dentigerous cyst, odontogenic keratocyst and ameloblastoma were analyzed. The study included paraffin embedded blocks of ameloblastoma [n=22], odontogenic keratocyst [n=20], and dentigerous cyst [n = 18]. The expression of alpha SMA was determined by immunohistochemically stained section. The percentage of positive cells was calculated from a minimum of 1000 cells and H-score was expressed [% positive cells x intensity of staining]. For transmission electron microscopy, fresh specimens were obtained from three patients and were fixed in 2.5% glutaraldehyde. The presence of cells with the ultrastructural characteristics of the myofibroblast was recorded. The mean number of positive cells in the three groups was significantly different. The difference between odontogenic keratocyst and dentigerous cyst and also the difference between dentigerous cyst and ameloblastoma were not statistically significant. The mean number of positive cells in the odontogenic keratocyst was significantly higher than that in ameloblastoma. In ultra-structural evaluation, myofibroblasts exhibited abundant cytoplasmic microfilaments, basal lamina-like material, subsurface caveolae, pinocytic vesicles, rough endoplasmic reticulum, and mitochondries. The high frequency of stromal myofibroblast in the odontogenic keratocyst implies that myofibroblast can contribute to aggressive nature of this cyst, but between odontogenic cysts and ameloblastoma, the presence of stromal myofibroblast has no correlation with invasiveness

[Correlation between P53, P63 expression and HPV infection with staging and grading of oral squamous cell carcinoma]

Squamous cell carcinoma is the eighth common cancer and forms 94% of oral malignancies. Nowadays, many molecular and genetic changes have been known to affect tumoral behavior which might be good for drug production and gene therapy. It has been known that mutated P53, overexpression of P63 as an oncogene and HPV infection are correlated with clinicopathological behavior of oral squamous cell carcinoma [OSCC] and it's prognosis and overall survival rate. The aim of this study is to determine the correlation between the expression of P53, P63 and HPV infection with oral squamous cell carcinoma and it's clinical staging and microscopical grading. Forty paraffin blocks with diagnosis of OSCC were collected from the pathology department, Shahid Beheshti School of Dentistry and the Department of general pathology, Taleghani hospital. Immunohistochemical staining was performed with antibodies against P53 and P63 and for detection of high risk types of HPV [HPVI6, 18] polymerized chain reaction [PCR] was used. Spearman correlation test was used for data analyzing. In this analytical cross-sectional study, we found significant correlation between P53 total score and staging [P=0.0001] and P53 total score and grading [P=0.013]. There was also significant correlation between P63 proportional score and grading [P=0.049]. HPV 16 also had significant correlation with P53 total score [P=0.003]. The mean nuclear staining of P53 was 33.04 +/- 25.66% [01=24.94-41.14] and P 63 was 39.25 +/- 19.35 [CI 33.13-45.37]. HPV frequency was 40% [HPV 16=20% and HPV18=7.5%]. There was no significant correlation between P63 and HPV infection [P>0.05]. We concluded that P63 could be a diagnostic marker for grading of OSCC and P53 and P63 expression might have some role in progression of OSCC and its clinicopathological behavior. Presence of HPV especially HPV 16 may be regarded as a risk factor in OSCC

[Fibroblastic osteosarcoma in mandible: a rare case report and review of literature]

Osteosarcoma is a malignancy of mesenchymal cells that have the ability to produce Osteoid or immature bone. Osteosarcoma of the jaw is uncommon and represents 6-8% of all osteosarcomas. This tumor occurs most often in the third and fourth decades of life, which is 10-15 years older than the mean age for Osteosarcomas of the long bones. The Maxilla and Mandible are involved with a boot equal frequency and a slight male predominance, is noted. Mandibular tumors arise more frequently in the posterior body and horizontal ramus. Maxillary lesions are discovered more commonly in the alveolar ridge, sinus floor and palate. This article is a report of fibroblastic Osteosarcoma in the mandible of a 35 years old man that has been referred to pathology Department of Shahid Beheshti dental school. The specific characteristic of this case, is its histologic features, because fibioblastic Osteosarcoma is rare in jaws and most cases are chondroblastic Osteosarcoma

Ameloblastomatous calcifying cystic odontogenic tumor: a rare histologic variant

In agreement with the new classification of the World Health Organization [WHO] 2005, calcifying odontogenic cyst [COC] or calcifying cystic odont-ogenic tumor [CCOT] is an uncommon developmental odontogenic lesion that demonstrates histopathologic diversity. Predominantly, it occurs in the anterior region of the mouth and in the second and third decades of life. Odontogenic tumors such as ameloblastoma have been reported to be associated with CCOT. In this paper, we report a case of ameloblastomatous CCOT in a boy with involvement of mandibular ramus-an extremely rare histologic variant. The microscopic examination revealed a CCOT; ghost cell within ameloblastic islands in the connective tissue wall was observed

Diagnostic value of human telomerase for detection of squamous cell carcinoma in oral smears

Human telomerase has been associated with most malignant tumors such as squamous cell carcinoma [SCC] of the oral cavity. One simple non-aggressive method for early diagnosis of oral SCC is taking smears. The aim of this study was to assess the frequency of telomerase expression in smears of oral SCC, compared with normal mucosa. Smears were obtained from 18 subjects with SCC of different sites in the oral cavity, presenting with various degrees of malignancy. Control samples were taken from the contra-lateral normal epithelium of the same subjects. The expression of hTERT, the most reliable marker of telomerase activity, was registered by extracting RNA, performing RT-PCR and then nested PCR by specific primers. Fisher's exact test was used to analyze the data. Positive expression of telomerase was detected in 8 of the 18 cases, while the normal epithelial samples demonstrated no such expression of telomerase. A significant difference in the expression of telomerase was reported between the two groups by Fisher's Exact test [P<0.003]. There is a potential for telomerase enzyme activity to act as a simple non - aggressive diagnostic tool in detection of oral SCC using smears of the lesion

Extramedullary plasmacytoma of the gingiva

Extramedullary plasmacytoma is defined as neoplastic proliferation of plasma cells in soft tissue. It accounts for up to 3% of all plasma cell tumors. Approximately, 90% of extramedullary plasmacytomas are found in the head and neck region commonly modality of treatment with or without adjuvant chemotherapy. We report a case of extramedullary plasmacytoma of the gingiva

[Evaluation of expression and intensity of cytiokeratin-10 and 19 in odontogenic keratocyst [OKC] with immunohistochemical staining and comparing it with dentigerous cyst and dental follicle]

Odontogenic keratocyst [OKC] and dentigerous cyst are two most common developmental odontogenic cysts involving jaws. OKC has a high growth potential in contrast to its minor clinical manifestation. It has also a high degree of recurrence rate. The purpose of this study was to diagnose the OKC and dentigerous cysts and also differentiate them based on their immunohistochemical criteria. To provide the materials for this research, we preliminarely reviewed the records of the patients referred to pathology lab of shahid Beheshti Dental College between March 1999 and March 2003 and 60 paraffin-embedded biopsies [20 OKC, 20 dentigerous cysts and 20 dental follicles] were selected. Then immunhistochemical staining was applied to identify cytokeratin-10 [CK-10] and cytokeratin-19 [CK-19] and their conditions, as well. The expression of CK-10, CK-19 are seen in OKC and dentigerous cyst. CK-10 was found more in OKC, along with CK-19 in dentigerous cyst. The expression of these cytokeratins was more in upper half of the epithelial lining of these cysts. One of the dental follicles expressed CK-19, but not CK-10. CK-10 is more specific for OKC as well as CK-19 for dentigerous cyst. These cytokeratins are usually seen in the upper layers of the cyst lining. If an expression of cytokeratin-19 is found in a dental follicle, it may correspond the cystic transformation of the latter one

Intraosseous mucoepidemoid carcinoma of the jaw in children: case report

This report presents an extremely rare tumor and provides information to deal with such tumors; intraosseous mucoepidermoid carcinoma. It's pathogenesis is still unknown and is rare in children. The conservative surgical procedures result to metastasis. An 11 year old girl was referred for a nonpainful swelling in maxillary region. Central mucoepidermoid carcinoma was diagnosed by clinical and paraclinical examinations. The lining of maxillary sinus was considered to be the source of the tumor. Hemimaxillectomy and adjuvant I radiotherapy was performed as treatment option and patient was reported to be in good health. Early diagnosis and radical resection with adjuvant radiotherapy offered the best chance for eradication of this tumor and that essential to prevent local recurrence and late distant methastasis

Efficacy of recombinant hepatitis B Vaccine in children

This study was carried out to determine the serum concentrations of anti-hepatitis B virus antibody [Anti-HBs] in 1-4 years old being vaccinated against hepatitis B. By drawing 2ml of venous blood from 175 children admitted for unrelated reasons to Yazd central medical laboratory and using a 3rd -generation ELISA test, the serum level of anti-HBs was quantitated. A questionnaire was used for obtaining data about age, sex, birth weight, current weight, schedule of previous hepatitis B vaccination, history of pathologic neonatal jaundice, and any serious health problem during childhood. In 170 of the cases [97.1%] the Anti-HBs concentration was above 10m IU/mL, routinely regarded as protective. No significant difference was observed concerning the various ages of subjects and their sexes, but anti-HBs levels were meaningfully lower in low-birth weight neonates [P-value<0.01], in children with history of pathologic neonatal jaundice [P-value =0.002] and in those having a previous serious disease [P-value=0.006]. The overall efficacy of vaccine is good, but in children who had had low-birth weight, pathologic neonatal jaundice, or any serious disease, careful follow-up must be carried out to ensure that protective levels of Anti-HBs are achieved after vaccination