Evaluation of the growth and osteogenic differentiation of ASCs cultured with PL and seeded on PLGA scaffolds.

Scaffold serves as an important component of tissue engineering, which facilitates cell attachment, proliferation and differentiation of cultured cells. In this study we aimed to use platelet lysates as a substitute for FBS in culturing and proliferation of human adipose tissue-derived stromal cells (ASCs), which constitute a promising source for cell therapy. We characterized ASCs in the presence of PL, and then we seeded them onto poly(lactic-co-glycolic acid) (PLGA) scaffolds, osteogenic media was used to induce their proliferation and osteogenic differentiation. Gene expression analysis revealed higher expression of osteogenic related genes, immunohistochemical staining showed proper cell attachment, growth and collagen matrix formation with the ability to induce vascularization. In conclusion, expansion of ASCs in PL-supplemented medium could promote cell proliferation and osteogenic differentiation of cells seeded on PLGA scaffolds, therefore it could be considered as a suitable and effective substitute for FBS to be used in clinical applications.

Mast Cell Numbers in Primary Eosinophilic Colitis are Significantly Higher than in Secondary Tissue Eosinophilia and Normal Control: a Possible Link to Pathogenesis.

BACKGROUND: Primary Eosinophilic Colitis (PEC) is one of the rare eosinophilic gastrointestinal diseases with a poorly understood pathogenesis. Eosinophilic esophagitis (EE) is the most common and best-understood disease in this category. Activated mast cells (MCs) have a role to play in the tissue damage in EE. It is not known if PEC shares this mechanism. OBJECTIVE: This cross-sectional study aimed to investigate the number of MCs in PEC and to compare them with cases of secondary colonic tissue eosinophilia (TE) and normal colon. METHODS: The study included 19 PEC cases, 47 cases of secondary tissue eosinophilia and 50 normal colon tissues. Histopathological slides of all cases were reviewed to confirm the diagnosis and count the number of eosinophils. Glass slides for all cases were stained for C-kit (CD117) to highlight and count the MCs. RESULTS: The mean number of the MCs in normal controls was 9.7 MCs per HPF (SD= 4.6). The mean number of MCs in the PEC cases was 26.5 (SD=7.1) which was significantly higher than the normal counts (p-value <0.000). The mean number of MCs in the secondary TE group was 18.0 (SD=7.1), which was significantly higher than normal controls; p-value <0.000. Comparing MC counts in PEC and secondary TE also revealed a significant difference with a p-value of < 0.000. CONCLUSION: MCs in PEC are significantly higher than those in secondary TE and normal controls. This suggests the role of the MCs in the pathogenesis of Primary Eosinophilic Colitis.

Arg399Gln XRCC1 Polymorphism and Risk of Squamous Cell Carcinoma of the Head and Neck in Jordanian Patients.

BACKGROUND AND OBJECTIVE: X-ray repair cross-complementing group1 (XRCC1) is a key protein in base excision repair and closely associated with the coordination of the base excision repair pathway. Many studies have focused on XRCC1 SNPs and have shown an associated between these SNPs and the risk of several types of cancers, including head and neck cancer. There are many single nucleotide polymorphisms XRCC1 gene (SNPs) and the most common SNP that result in amino acid substitutions is exon 10 (Arg399Gln). This study aimed to investigate the association between Arg399Gln SNP and the risk of squamous cell carcinoma of the head and neck. MATERIAL AND METHOD: Ninety nine patients with squamous cell carcinomas of the head and neck and 89 healthy adult controls were enrolled in this study. The Arg399Gln in XRCC1 allele was genotyped using polymerase chain reaction-restriction fragment length polymorphism method. RESULTS: In the single-locus analyses, Arg399Gln SNP showed a significant association with head and neck cancer risk (p value = 0.016 and odd ratio of 1.8). On the genotype level, we applied three analysis models, namely co-dominant, dominant, and recessive genotypes. Arg/Arg homozygous major genotype was significantly (p value <0.05) associated with head and neck squamous cell carcinoma incidence with odd ratio of 2.23 and 2.24 for the co-dominant and recessive models, respectively. CONCLUSION: The findings indicated that Arg399Gln allele was associated with squamous cell carcinoma of the head and neck among Jordanian patients. This allele might be used as a genetic biomarker of squamous cell carcinoma of the head and neck.

KRAS and NRAS mutational gene profile of metastatic colorectal cancer patients in Jordan.

BACKGROUND: A constitutively active RAS protein in the absence of stimulation of the epidermal growth factor receptor (EGFR) is the result of mutations in KRAS and NRAS genes. Mutations in the KRAS exon 2 and outside exon 2 have been found to predict the resistance to anti-EGFR monoclonal therapy. A substantial proportion of metastatic colorectal cancer cases (mCRC) exhibit RAS mutations outside KRAS exon 2, particularly in KRAS exon 3 and 4 and NRAS exons 2 and 3. No data about RAS mutations outside KRAS exon 2 are available for Jordanian patients with mCRC. We aim to study the molecular spectrum, frequency, and distribution pattern of KRAS and NRAS mutations in Jordanian patients with mCRC. METHODS: A cohort of 190 Jordanian metastatic colorectal cancer patients were enrolled in the trial. We detected mutations in exon 2 of the KRAS and NRAS gene as well as mutations outside of exon 2 using the StripAssay technique. The KRAS StripAssay covered 29 mutations and 22 NRAS mutations. RESULTS: Mutations were observed in 92 (48.42%) cases, and KRAS exon 2 mutations accounted for 76 cases (83.69%). KRAS G12D was the most common mutation, occurring in 18 cases, followed by KRAS G12A in 16 cases, and G12T in 13 cases. Mutations outside of KRAS exon 2 represented 16.3% of the mutated cases. Among those, 6 cases (6.48%) carried mutations in NRAS exon 2 and 3, and 10 cases (10.87%) in KRAS exon 3 and 4. CONCLUSION: The frequency of NRAS and KRAS mutations outside of exon 2 appears to be higher in Jordanian patients in comparison with patients from western countries. KRAS mutations outside of exon 2 should be tested routinely to identify patients who should not be treated with anti-EGFR antibodies.

Is the Diagnosis of Celiac Disease Possible Without Intestinal Biopsy?

BACKGROUND: Coeliac disease is defined as a state of immune-mediated hyper-responsiveness to dietary gluten from wheat, barley, or rye in genetically predisposed individuals that results in tissue damage. The diagnosis is made by microscopic examination of a small intestinal biopsy, although serological testing for antibodies against tissue transglutaminase and deamidated gliadin peptide can be of great advantage. It has been suggested that duodenal biopsy can be avoided in patients with high levels of the tissue transglutaminase antibody, since a relationship has been found to be present between tissue transglutaminase antibody titres and coeliac disease. AIMS: To study the correlation between tissue transglutaminase titre and small intestinal biopsy findings in patients with coeliac disease. STUDY DESIGN: Diagnostic accuracy study. METHODS: Ninety-five cases of patients diagnosed with coeliac disease and with positive serum tissue transglutaminase titres were retrieved from the Jordan University Hospital archives between December 2014 and December 2015. All the cases were classified according to the Marsh classification. RESULTS: Ninety-five cases with a positive titre for the antibody were included in this study, 73 (76.8%) of them were females and 22 cases (23.2%) were males. The age of the patients ranged between 4 and 75 years with a mean age ± standard deviation of 32.3±14.7. The sensitivity was the highest in Marsh IIIC and lowest in Marsh IIIA (95% versus 68% respectively). The specificity was moderate (76%) for all subtypes of Marsh III. CONCLUSION: This study showed a positive correlation between the tissue transglutaminase titre and the degree of duodenal damage (Marsh IIIC) in patients with coeliac disease. In the presence of high tissue transglutaminase levels, duodenal biopsy might not be always necessary for diagnosis, particularly in symptomatic patients.

Leydig cell tumor in grey zone: A case report.

INTRODUCTION: Leydig cell tumor constitutes only about 1-3% of testicular neoplasms. There is apparently increased incidence in the last few years; one possible explanation for this phenomenon is the widespread use of ultrasound technology and the subsequent increased early detection of smaller lesions that have not been found in historical series. CASE PRESENTATION: We report a case of Leydig cell tumor of testis in a patient presenting with painless long standing slowly growing left scrotal mass who found to have intrapulmonary nodule and multiple enlarged retroperitoneal lymph nodes on staging work up. The mass was managed by radical orchiectomy. Pathological diagnosis was Leydig cell tumor. DISCUSSION: Orchiectomy is the accepted mode of treatment but follow-up every 3-6 months with physical examination, hormone assays, scrotal and abdominal ultrasonography, chest radiography, and CT scans is essential in such a case with a potential for malignant behavior. CONCLUSION: Inguinal orchiectomy is the therapeutic decision of choice and long-term follow-up is necessary to exclude recurrence or metastasis. Cases which fall in the grey zone like ours need to be followed up carefully for metastasis instead of rushing into an early retroperitoneal lymph node dissection, with its potential risks and complications.

New trends in the clinicopathological features of differentiated thyroid cancer in Central Jordan.

OBJECTIVE: To investigate the current trends in presentation and distribution of differentiated thyroid cancer (DTC) at the largest referral hospital for endocrine cancers in Central Jordan. METHODS: We analyzed the clinical features, management and outcome of 110 patients diagnosed with thyroid carcinoma at Jordan University Hospital, Amman, between 1996 and 2001. RESULTS: Papillary carcinoma was diagnosed in 87 patients (80%), follicular carcinoma in 3 patients (2.7%), Hurthle cell carcinoma in 8 patients (7.3%), medullary carcinoma in 5 (4.5%), and anaplastic carcinoma in 4 patients (3.6%), metastatic cancer in 2 patients and lymphoma in one patient. Time course analysis showed an increasing trend in surgery for thyroid cancer from 28 cases in 1986-1991 to 48 in 1996-2001. As time advanced, the incidence of locally invasive disease and lymph node involvement markedly increased over the last 5 years of the study (from 28-62%). All patients with follicular carcinoma were diagnosed in the period 1986-1994. After thyroidectomy and a follow up period of 2-15 years, 10 patients died of their disease, 4 of these died within one year from anaplastic thyroid carcinoma. CONCLUSION: The dramatic decline in the incidence of follicular thyroid carcinoma combined with the increase in the advanced forms of thyroid cancer in Central Jordan may suggest a possible environmental factor in thyroid carcinogenesis in this region. We suggest a larger scale studies and steps to investigate the etiologic factors for thyroid carcinogenesis in Central Jordan.

Frequency of epidermal growth factor receptor mutations in Jordanian lung adenocarcinoma patients at diagnosis.

BACKGROUND: Somatic mutations of the epidermal growth factor receptor (EGFR) gene have been associated with tumor response to tyrosine kinase inhibitors (TKIs) and favorable outcome in patients with non-small-cell lung cancer (NSCLC). The activating mutations that confer sensitivity to EGFR TKIs are present in the TK domain of the EGFR gene. This study aims to report on the prevalence of EGFR mutations in NSCLC and non-squamous lung cancer patients at diagnosis, using genomic deoxyribonucleic acid (DNA) obtained from paraffin-embedded tissue samples. MATERIALS AND METHODS: We collected formalin.fixed, paraffin.embedded. (FFPE) tissue samples from 166. cases of lungadenocarcinomas referring to Jordan University Hospital and King Hussein Cancer Center between 2007 and first half of 2013. None of the patients met the definition of never smoker defined as those who smoked less than 100 cigarettes in their lifetime. We evaluated EGFR mutations by nested polymerase chain reaction. (PCR) followed by direct sequencing of the EGFR kinase domain from exon 18 to 21. RESULTS: Six different point mutations were detected in 24 patients (14.46%) of the study population. The resultant mutations were as follows: Ten patients have deletion in exon 19, sevenpatients have L858R, two patients have L861P, and one of each of the following: A735T, D770_N771 insY, L858P, L861Q, and G917C. CONCLUSION: The present study revealed that the EGFR mutations rate in Jordanian patients with adenocarcinoma of the lung was higher than in African-American, and some white Caucasian patients, and was lower than in patients in East Asia, and other countries of South Asia.

Simultaneous medullary and papillary thyroid carcinoma with lymph node metastasis in the same patient: case report and review of the literature.

We report a rare case of simultaneous medullary thyroid carcinoma on the left thyroid lobe with lymph node metastasis and papillary thyroid cancer on the right thyroid lobe. The 55-year-old woman was diagnosed with medullary thyroid carcinoma after left hemithyroidectomy for goitre. Completion thyroidectomy, central neck dissection and left modified neck dissection revealed the presence of papillary carcinoma on the right side. The extreme rarity and interesting pathological features are discussed and we raise the question of whether the finding of another thyroid cancer on the opposite side was coincidental or from possible activation of a common tumorigenic pathway for both follicular and parafollicular thyroid cells.

Cyclooxygenase-2 (COX2) gene polymorphisms and the risk of sporadic colorectal cancer and polyps among Jordanian population.

BACKGROUND/AIMS: Cyclooxygenase-2 (COX2) is a crucial enzyme involved in the metabolism of Prostaglandins and it has been implicated in several processes. This study was done to investigate the associations of polymorphisms in COX2 gene with the risk of colorectal cancer or polyps development among Jordanian population and to correlate with other ethnicities. MATERIALS AND METHODS: One hundred and thirty five cases (135) of colorectal carcinoma were studied for COX2 -A1195G polymorphisms employing PCR-RFLP technique, in addition to 104 cases of adenomatous polyps and 115 matched controls taken from the general population. RESULTS: Sixty eight colorectal cancer patients were males and 67 of patients were females with a median age of (58.0±13.9 year). Sixty six (66) of polyp cases were males and 38 were females with a median age of (58.1±14.16). The A-1195G AA carriers were 3.1 times less likely to develop CRC (95% CI: 1.8-5.3, p<0.0001), and 1.8 times less likely to develop polyps (95% CI: 0.99-3.2, p=0.056).The A-1195G AG carriers were at higher risk to develop cancer in a dose dependent manner. The AG carriers were 2.9 time more likely to develop CRC and two times more likely to develop polyps when compared to controls. The A allele was more predominant in controls than in polyps or CRC cases. Carriers of the A allele were 1.6 times less likely to develop polyps and 2.6 times less likely to develop CRC. CONCLUSION: The presence of the COX-2 -1195AA genotype may protect against risk of developing colorectal cancer.

Allele and Genotype Frequencies of the Polymorphic Methylenetetrahydrofolate Reductase and Lung Cancer in ther Jordanian Population: a Case Control Study.

BACKGROUND: Methylenetetrahydrofolate reductase (MTHFR) is involved in amino acid synthesis and DNA function. Two common polymorphisms are reported, C677T and A1298C, that are implicated in a number of human diseases, including cancer. OBJECTIVE: The association between MTHFR C677T and A1298C genotype and haplotype frequencies in risk for lung cancer (LC) was investigated in the Jordanian population. MATERIALS AND METHODS: A total of 98 LC cases were studied for MTHFR C677T and A1298C polymorphisms, compared to 89 controls taken from the general population, employing the PCR-RFLP technique. RESULTS: The frequency of the genotypes of MTHFR C677T among Jordanians was: CC, 59.6%, CT, 33%; and TT, 7.4% among LC cases and 49.4%, 40.2% and 10.3% among controls. No significant association was detected between genetic polymorphism at this site and LC. At MTHFR A12987C, the genotype distribution was AA, 29.5%; AC, 45.3%, and CC 25.3% among LC cases and 36.8%, 50.6% and 12.6% among controls. Carriers of the CC genotype were more likely to have LC (OR=2.5; 95%CI: 1.04-6; p=0.039) as compared to AA carriers. Smokers and males with the CC genotype were 9.9 and 6.7 times more likely to have LC, respectively (ORsmokers=9.9; 95%CI: 1.2-84.5, p=0.018; ORmen=6.6; 95%CI: 1.7-26.2, p=0.005). Haplotype analysis of MTHFR polymorphism at the two loci showed differential distribution of the CC haplotype (677C-1298C) between cases and controls. The CC haplotype was associated with an increased risk for lung cancer (OR=1.6; 95% CI: 1.03-2.4, p=0.037). CONCLUSIONS: The genetic polymorphism of MTHFR at 1298 and the CC haplotype (risk is apparently lower with the C allele at position 677) may modulate the risk for LC development among the Jordanian population. Risk associated with the 1298C allele is increased in smokers and in males. The results indicate that a critical gene involved in folate metabolism plays a modifying role in lung cancer risk, at least in the Jordanian population.

Is Hashimoto's thyroiditis a risk factor for medullary thyroid carcinoma? Our experience and a literature review.

The etiology of medullary thyroid carcinoma remains unknown. The aim of this study was to determine whether there is a significant association between medullary thyroid carcinoma and Hashimoto's thyroiditis in the histopathologic material of thyroidectomized patients. Retrospective cross-sectional study. In this study, we reviewed the medical records of all patients who underwent total thyroidectomy for different thyroid-related complaints between January 2000 and January 2012 at Jordan University Hospital-Amman, Jordan. To highlight relevant previously published studies addressing this topic, a literature search was conducted for English language studies reporting "medullary thyroid carcinoma" or "C-cell hyperplasia" in patients with Hashimoto's thyroiditis. Of the 863 patients with a mean age of 47.2 ± 12.3 years who underwent total thyroidectomy during the study period, 78 (9.04 %) were diagnosed with Hashimoto's thyroiditis, and 15 (1.74 %) had medullary thyroid carcinoma, 3 (20 %) of whom had coexistent Hashimoto's thyroiditis. A total of 683 (79.1 %) patients had benign thyroid disease, 67 (9.8 %) of whom had Hashimoto's thyroiditis. The difference between these rates was not statistically significant (p = 0.19). When examined by gender, 9 females had medullary thyroid carcinoma, 3 (33.3 %) of whom had coexistent Hashimoto's thyroiditis; by contrast, of 560 females with benign thyroid disease, 62 (11.1 %) had Hashimoto's thyroiditis (p = 0.04). Although this study population represents a small and single-institution experience, our results suggest that there might be an association between Hashimoto's thyroiditis and medullary thyroid carcinoma only in female patients who undergo total thyroidectomy.

Pulmonary Artery Sarcoma: A Rare Entity.

Pulmonary artery sarcomas (PAS) are extremely rare sarcomas of uncertain histogenesis that often mimic pulmonary thromboemboli. This is a report of a 60-year-old female patient who presented with recurrent chest pain and cough. The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy. Follow-up imaging studies revealed a mass in the left hilar region extending into the pulmonary trunk and branches of the left pulmonary artery. The tru-cut biopsy revealed an undifferentiated sarcoma. The patient died 10 months after her initial presentation.

Gastric perforation secondary to metastasis from ovarian cancer: Case report.

INTRODUCTION: Metastasis from ovarian cancer occurs frequently through the peritoneal cavity in the form of peritoneal carcinomatosis; isolated gastric metastasis is rarely reported in literature. PRESENTATION OF CASE: We present a case of 43-year-old infertile lady, who developed a picture of acute abdomen four days post total abdominal hysterectomy and salpingoopherectomy for ovarian cancer. Further contrast-enhanced CT scan demonstrated massive free gas and fluid in the abdomen. She underwent antrectomy with truncal vagotomy due to 3cm×4cm prepyloric gastric ulcer. Final pathology proved the presence of metastatic serous cystadenocarcinoma of ovarian origin. DISCUSSION: Our patient had a gastric perforation secondary to ovarian metastasis. Being isolated, the absence of ascites and the transmural nature of the gastric metastasis suggest haematogenous spread .To the best of our knowledge perforated gastric metastasis secondary to ovarian cancer was not reported in literature before. CONCLUSION: Gastric metastasis should be kept in mind in patients with a well-known ovarian cancer who present with gastric lesions, ulcers, bleeding or perforation.

Synthesis and evaluation of mutual azo prodrug of 5-aminosalicylic acid linked to 2-phenylbenzoxazole-2-yl-5-acetic acid in ulcerative colitis.

In this study, the syntheses of 4-aminophenylbenzoxazol-2-yl-5-acetic acid, (an analogue of a known nonsteroidal anti-inflammatory drug [NSAID]) and 5-[4-(benzoxazol-2-yl-5-acetic acid)phenylazo]-2-hydroxybenzoic acid (a novel mutual azo prodrug of 5-aminosalicylic acid [5-ASA]) are reported. The structures of the synthesized compounds were confirmed using infrared (IR), hydrogen-1 nuclear magnetic resonance (1H NMR), and mass spectrometry (MS) spectroscopy. Incubation of the azo compound with rat cecal contents demonstrated the susceptibility of the prepared azo prodrug to bacterial azoreductase enzyme. The azo compound and the 4-aminophenylbenzoxazol-2-yl-5-acetic acid were evaluated for inflammatory bowel diseases, in trinitrobenzenesulfonic acid (TNB)-induced colitis in rats. The synthesized diazo compound and the 4-aminophenylbenzoxazol-2-yl-5-acetic acid were found to be as effective as 5-aminosalicylic acid for ulcerative colitis. The results of this work suggest that the 4-aminophenylbenzoxazol-2-yl-5-acetic acid may represent a new lead for treatment of ulcerative colitis.

Distribution of breast cancer subtypes among Jordanian women and correlation with histopathological grade: molecular subclassification study.

OBJECTIVE: To evaluate the hormone receptor status and human epidermal growth factor receptor 2 (HER2)/neu gene expression among Jordanian women with breast cancer. To classify our patients into molecular subtypes and to correlate the results with age of the patients and tumour grade. DESIGN: Evaluation of estrogen receptor (ER), PR and HER2/neu was done by standard immunohistochemical technique and subclassification into molecular subtypes. SETTING: Jordan University Hospital, Amman, Jordan. PARTICIPANTS: One hundred and ninety-three cases of breast cancer diagnosed at Jordan University Hospital. MAIN OUTCOME MEASURES: Molecular subtypes of breast cancer, age and tumour grade. RESULTS: ALL THE CASES WERE DIVIDED INTO TWO GROUPS: the young age group less or equal 50 years of age and the older age group more than 50 years of age. The cases were subclassified into luminal A, luminal B, basal cell like (BCL) and Her2/neu+. In older age group, the most common subtype was luminal A (72%). In this age group, most of the cases (48%) were of grade II. In younger age group, 47% of the cases were of luminal A subclass. In this age group, 42% were of grade I. CONCLUSIONS: Molecular subtyping of breast cancer is an essential predicting factor of tumour response to hormonal therapy. This fact puts increased stress on the urgent need for the development of reliable and reproducible classification systems.

Regression of endometrial implants treated with vitamin D3 in a rat model of endometriosis.

Endometriosis is one of the most frequent gynecological diseases. In addition to their side effects, available medical therapies may decrease fertility. Current understanding of endometriosis focuses on the role of the immune system in its pathophysiology. Recent research shed light on the immunomodulatory effect of vitamin D3. Thus, this study was designed to study the effect of vitamin D3 on regression of endometriotic implants in a rat surgical model. Vitamin D3 reduced cyst cross sectional area by 48.8%. Histologically, vitamin D treatment produced fibrosis as well as apoptosis in the stroma. The results of the present study suggest that vitamin D3 administration may have a beneficial effect in treating endometriosis.

Allele and genotype frequencies of the polymorphic methylenetetrahydrofolate reductase and colorectal cancer among Jordanian population.

BACKGROUND: Methylenetetrahydrofolate reductase (MTHFR) is involved in DNA synthesis and repair. We here aimed to investigate two common polymorphisms, C677T and A1298C, with genotype and haplotype frequencies in colorectal cancer (CRC) cases among Jordanian. MATERIALS AND METHODS: 131 CRC cases were studied for MTHFR C677T and A1298C polymorphisms, compared to 117 controls taken from the general population, employing the PCR-RFLP technique. RESULTS: We found the frequency of the three different genotypes of MTHFR C677T among Jordanians to be CC: 61.7%, CT: 35.2%, and TT 3.1% among CRC cases and 50.9%, 38.8% and 10.3% among controls. Carriers of the TT genotype were less likely to have CRC (OR=0.25; 95%CI: 0.076-0.811; p=0.021) as compared to those with the CC genotype. Genotype analysis of MTHFR A12987C revealed AA: 38.9%, AC: 45%, and CC 16% among CRC cases and 37.4%, 50.4% and 12.2% among controls. There was no significant association between genetic polymorphism at this site and CRC. Haplotype analysis of MTHFR polymorphism at the two loci showed differential distribution of the TA haplotype (677T-1298A) between cases and controls. The TA haplotype was associated with a decreased risk for colorectal cancer (OR=0.6; 95% CI: 0.4-0.9, p=0.03). CONCLUSIONS: The genetic polymorphism of MTHFR at 677 and the TA haplotype may modulate the risk for CRC development among the Jordanian population. Our findings may reflect an importance of genes involved in folate metabolism in cancer risk.

Orofacial findings in chronic granulomatous disease: report of twelve patients and review of the literature.

BACKGROUND: Chronic granulomatous disease is an extremely rare primary immunodeficiency syndrome that can be associated with various oral complications. This can affect high number of patients. However, data on oral complications is sparse. Here we will review the literature and describe the orofacial findings in 12 patients. FINDINGS: The age range was 5-31 years. Oral findings were variable, and reflected a low level of oral hygiene. They included periodontitis, rampant caries, gingivitis, aphthous-like ulcers, and geographic tongue. One patient had white patches on the buccal mucosa similar to lichen planus. Another patient had a nodular dorsum of the tongue associated with fissured and geographic tongue. Biopsies from the latter two lesions revealed chronic non-specific mucositis. Panoramic radiographs showed extensive periodontitis in one patient and periapical lesions in another patient. CONCLUSION: Patients with chronic granulomatous disease may develop oral lesions reflecting susceptibility to infections and inflammation. It is also possible that social and genetic factors may influence the development of this complication. Therefore, oral hygiene must be kept at an optimum level to prevent infections that can be difficult to manage.

Intramuscular myxoma of the hypothenar muscles.

Intramuscular myxomas of the hand are rare entities. Primarily found in the myocardium, these lesions also affect the bone and soft tissues in other parts of the body. This article describes a case of hypothenar muscles myxoma treated with local surgical excision after frozen section biopsy with tumor-free margins. Radiographic images of the axial and appendicular skeleton were negative for fibrous dysplasia, and endocrine studies were within normal limits. The 8-year follow-up period has been uneventful, with no complications. The patient is currently recurrence free, with normal intrinsic hand function.