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1.  Bilateral Odontogenic Keratocyst of the Mandible 
Odontogenic keratocyst (OKC) is a cyst of dental origin with an aggressive clinical behavior, having high recurrence rate. Multiple cysts are associated with bifid-rib basal cell nevus syndrome (Gorlin syndrome). We present a case of bilateral odontogenic keratocyst in a cleft lip patient.
PMCID: PMC4082547  PMID: 25018611
Keratocyst; Mandible; Maxilla; Odontogenic; Recurrence
2.  The Efficacy of ErCr:YSGG Laser Fibrotomy in Management of Moderate Oral Submucous Fibrosis: A Preliminary Study 
Oral submucous fibrosis (OSMF) is a chronic insidious disease mainly associated with fibroelastic change of the oral mucous membrane, leading to progressive trismus and oral burning sensation. The management of OSMF is empirical, depends on staging of the condition and is combination of conservative/medical/surgical interventions. Management of moderate OSMF is more challenging as conservative and medical treatments are not effective while surgical techniques involving fibrotomy and reconstruction of resultant defect are excessive. Lasers can provide an alternative and better means for surgical fibrotomy in moderate OSMF as they are minimally invasive and have the advantage of short operating time, less hemorrhage, faster healing, less morbidity, less surgical-site scarring and relapse. Laser fibrotomy in moderate OSMF have been done under general anesthesia.
Materials and Methods
A case series of 16 cases of moderate OSMF treated with Erbium Chromium Yttrium Scandium Gallium Garnet (ErCr:YSGG) laser fibrotomy under local anesthesia in combination with cessation of habits, topical steroids, lycopene and oral physiotherapy is presented.
The mean increase in mouth opening achieved at 1 year was 17.5 mm. The mean difference in the preoperative and 1 year mouth opening was found to be statistically significant. The mean difference in the preoperative and six-month Visual Analogue Scale scores for oral burning sensation and Oral Health Impact Profile-14 scores for assessment of oral health-related quality of life was statistically significant implying improvement.
ErCr:YSGG laser fibrotomy under local anesthesia is a minimally invasive, cost effective, chair-side procedure and an useful adjunct in management of moderate OSMF.
PMCID: PMC4082553  PMID: 25018602
Oral submucous fibrosis; Trismus; Laser surgery; Burning mouth; Oral health-related quality of life
4.  Neodymium:Yttrium aluminum garnet laser in the management of oral leukoplakia: A case series 
Contemporary Clinical Dentistry  2015;6(Suppl 1):S32-S35.
Oral leukoplakia is a nonscrappable whitish patch described as a potentially malignant disorder with high prevalence in India. Besides medicinal treatment, neodymium: Yttrium aluminum garnet (Nd: YAG) laser is also used for the management of oral leukoplakia. This study evaluated the role of Nd: YAG laser in the management of oral leukoplakia and also investigated postoperative complications along with long-term prognosis of the disease.
Settings and Designs:
The study is a prospective cohort study conducted for 24 months (June 2011 to May 2013) in Lucknow.
Materials and Methods:
The study comprised of 42 patients, both male and female of Indian origin and diagnosed with oral leukoplakic lesions. Patients with biopsy proven squamous cell carcinoma and medically compromised are excluded from the study. All patients has undergone ablation of lesion by pulsed Nd: YAG laser and were followed after 24 h, 72 h, 1-week and then successively for 1, 3, 6 months and then 1st and 2nd postoperative years.
Pain and slough were evaluated by Wicoxon rank test (P = 0.0001 statistically significant) which decreased from 24 h to 1-week and became nil in subsequent follow-ups. Similarly, McNemar's test (P = 0.001 statistically significant) was used for evaluation of burning sensation, paresthesia, infection and recurrences. Recurrence was noted in 2 patients but following the second application, there were no recurrences over the period of further follow-up. None of the patient suffered from an infection, paresthesia or anesthesia.
Hence, Nd: YAG laser was found to be effective in ablating leukoplakia. It is convenient, economical with minimum complications and morbidities.
PMCID: PMC4374315  PMID: 25821371
Ablation; neodymium: Yttrium aluminum garnet laser; oral leukoplakia
5.  Evaluation of exfoliative cytology in the diagnosis of oral premalignant and malignant lesions: A cytomorphometric analysis 
Dental Research Journal  2015;12(1):83-88.
Many oral squamous cell carcinomas (OSCCs) arise within regions that previously had premalignant lesion. Early diagnosis and prompt treatment of premalignant lesions offers the best hope of improving the prognosis in patients with OSCC. Exfoliative cytology is a simple and non-invasive diagnostic technique that could be used for early detection of oral premalignant and malignant lesions. This study was undertaken to evaluate the quantitative changes in nuclear area (NA), cytoplasmic area (CA) and nuclear-to-cytoplasmic ratio (NA/CA) in cytological buccal smears of oral leukoplakia with dysplasia (OLD) and OSCC patients while comparing with normal healthy mucosa.
Materials and Methods:
A quantitative study was conducted over 90 subjects including 30 cases each of OLD, OSCC and clinically normal oral mucosa. The smears obtained were stained with Papanicolaou (PAP) stain and cytomorphological assessment of the keratinocytes was carried out. The statistical tools included arithmetic mean, standard deviation, Chi-square test, analysis of variance, Tukey multiple comparison. P < 0.001 was considered as significant.
The mean NA of keratinocytes in the normal mucosa was 65.47 ± 4.77 μm2 while for OLD it was 107.97 ± 5.44 μm2 and 139.02 ± 8.10 μm2 for that of OSCC. The differences show a statistically significant increment in NA (P < 0.001). There was significant reduction (P < 0.001) in the CA of keratinocytes from OSCC when compared with those from smears of OLD and normal mucosa with the values of 1535.80 ± 79.38 μm2, 1078.51 ± 56.65 μm2 and 769.70 ± 38.77 μm2 respectively. The NA/CA ratio in the smears from normal oral mucosa, OLD and OSCC showed a mean value of 0.043 ± 0.004, 0.100 ± 0.008, 0.181 ± 0.015 respectively with a significant difference among the groups (P < 0.001).
Evaluation of nuclear and CA of keratinocytes by cytomorphometry can serve as a useful adjunct in the diagnosis and prognosis of a dysplastic lesion which may lead to OSCC.
PMCID: PMC4336977  PMID: 25709680
Cytomorphometry; dysplasia; exfoliative cytology; oral leukoplakia; oral squamous cell carcinoma
6.  A Case of Vander Woude Syndrome with Rare Phenotypic Expressions 
Van der Woude syndrome (VWS) is a rare developmental disorder with an autosomal dominant inheritance. The prevalence of VWS varies from 1:100,000 to 1:40,000 still born or live births. It has variable expressivity and generally expressed as orofacial manifestations like lower lip pits, cleft lip and/or cleft palate, hypodontia, cleft or bifid uvula, ankyloglossia and some extraoral anomalies involving hand, foot and genitalia.
Thorough family history, clinical examination and genetic counseling helps in correct diagnosis of VWS as Popliteal pterygium syndrome has overlapping clinical manifestations. Most cases of Van der Woude syndrome have been associated with mutations and genetic changes.
The current case has classical features of VWS with some rare features like undescended small testis and unreported finding of syndactyly of second and third toe adds on to the existing knowledge of VWS presentation.
PMCID: PMC4253239  PMID: 25478421
Lip pits; Popliteal pterygium syndrome; Syndactyly; Van der woude syndrome
7.  Preparation of (+)-Trans-Isoalliin and Its Isomers by Chemical Synthesis and RP-HPLC Resolution 
Naturally occurring (+)-trans-isoalliin, (RCRS)-(+)-trans-S-1-propenyl-L-cysteine sulfoxide, is a major cysteine sulfoxide in onion. The importance of producing it synthetically to support further research is very well recognized. The (+)-trans-isoalliin is prepared by chemical synthesis and reversed-phase (RP)-HPLC. First, S-2-propenyl-L-cysteine (deoxyalliin) is formed from L-cysteine and allyl bromide, which is then isomerized to S-1-propenyl-L-cysteine (deoxyisoalliin) by a base-catalyzed reaction. A mixture of cis and trans forms of deoxyisoalliin is formed and separated by RP-HPLC. Oxidation of the trans form of deoxyisoalliin by H2O2 produces a mixture of (−)- and (+)-trans-isoalliin. Finally, RP-HPLC is used successfully in separating (−)- and (+)-trans-isoalliin, and hence, (+)-trans-isoalliin is synthesized for the first time in this study. In addition, the (±) diastereomers of cis-isoalliin are also separated and purified by RP-HPLC.
PMCID: PMC3970760  PMID: 25187757
Allium cepa; 1-propenyl-L-cysteine sulfoxide; organosulfur compounds
8.  Acanthamoeba Detection in the Anterior Chamber after Therapeutic Penetrating Keratoplasty 
Acanthamoeba infection has been recognized in almost all parts of the world. With most of the literature focusing on contact lens related Acanthamoeba keratitis, ophthalmologists may hesitate to diagnose this entity in patients without lenses. We report the case of a patient with Acanthamoeba infection of the anterior chamber, unrelated to contact lens wear, diagnosed by examination of aqueous tap following penetrating therapeutic keratoplasty.
PMCID: PMC4129262  PMID: 25120983
Acanthamoeba; Aqueous tap; Corneal ulceration; Keratitis
9.  Prevalence of oral cancer and pre-cancerous lesions and the association with numerous risk factors in North India: A hospital based study 
Oral cancer is one of the most common life-threatening diseases all over the world. Developing countries face several challenges to identify and remove potential risk factors. Chewing tobacco/pan masala is considered to be the most potent risk factor for oral precancerous lesions and oral cancer.
To investigate the relative occurrence of different oral pre-cancerous lesions and oral cancer in North India and to identify the associated risk factors.
Materials and Methods:
A hospital-based study was conducted and 471 subjects were recruited in the study. The subjects comprised patients with squamous cell carcinoma (n = 85), oral submucous fibrosis (n = 240), leukoplakia (n = 32), lichen planus (n = 15), and controls (n = 99). Statistical analysis of the data was done using Chi-square and regression analysis.
A strong correlation was observed between the presence of the chewing habit in all the oral precancerous lesions and oral cancer. Duration of the habit and intensity of habit ware also strongly correlated with the risk of oral precancerous lesions and oral cancer. Other factors such as alcohol and smoking were found to be less important in concern with oral cancer and precancerous lesions.
PMCID: PMC4405954  PMID: 25937723
Leukoplakia; lichen planus; oral cancer; oral submucous fibrosis; tobacco chewing
10.  Plasma cell mucositis with gingival enlargement and severe periodontitis 
Plasma cell mucositis (PCM) is a very rare, chronic, multifocal, idiopathic, non-neoplastic plasma cell proliferative disorder of the upper aerodigestive tract. The classic clinical presentation is an intensely erythematous mucosa with surface changes described variously as papillomatous, cobblestone, nodular or velvety. It is a very rare condition <50 cases reported in literature. A 72-year-old male patient complained of sore throat, stomatodynia, dysphagia, multiple oral ulcers, enlarged swollen bleeding gums and mobile teeth. There was chronic inflammatory enlargement of the gingiva and palate with severe periodontitis. Histopathological examination revealed a hyperplastic epithelium with a dense infiltrate of mature polyclonal plasma cells in the superficial layer of the lamina propria. PCM is a diagnosis of exclusion, to be differentiated from other infective, reactive, autoimmune, allergic and neoplastic disorders with plasma cell infiltrates. Management with surgical and immunosuppressive therapy is mostly ineffective with short remissions and frequent relapses.
PMCID: PMC4095634  PMID: 25024555
Dysphagia; gingival enlargement; periodontitis; plasma cell mucositis; stomatodynia
11.  Contemporary practice in forensic odontology 
Forensic odontology plays a major role in the identification of those individuals who cannot be identified visually or by other means. The unique nature of dental anatomy and placement of custom restorations ensure accuracy when the techniques are correctly employed. It is evident that identification of victims in accidents and natural calamities is of utmost importance and is a challenging task. The teeth may also be used as weapons and under certain circumstances; they may provide information regarding the identity of the biter. Dental professionals play a major role in keeping accurate dental records and providing all necessary information so that legal authorities may recognize malpractices, negligence, fraud child abuse and also, identify an individual. In this article, we will discuss such evolvement of the subject.
PMCID: PMC4196294  PMID: 25328306
Age estimation; bite mark analysis; dental records; forensic odontology; mass disaster; sex determination
12.  Preparation of (+)-Trans-Isoalliin and Its Isomers by Chemical Synthesis and RP-HPLC Resolution 
Naturally occurring (+)-trans-isoalliin, (RCRS)-(+)-trans-S-1-propenyl-L-cysteine sulfoxide, is a major cysteine sulfoxide in onion. The importance of producing it synthetically to support further research is very well recognized. The (+)-trans-isoalliin is prepared by chemical synthesis and reversed-phase (RP)-HPLC. First, S-2-propenyl-L-cysteine (deoxyalliin) is formed from L-cysteine and allyl bromide, which is then isomerized to S-1-propenyl-L-cysteine (deoxyisoalliin) by a base-catalyzed reaction. A mixture of cis and trans forms of deoxyisoalliin is formed and separated by RP-HPLC. Oxidation of the trans form of deoxyisoalliin by H2O2 produces a mixture of (−)- and (+)-trans-isoalliin. Finally, RP-HPLC is used successfully in separating (−)- and (+)-trans-isoalliin, and hence, (+)-trans-isoalliin is synthesized for the first time in this study. In addition, the (±) diastereomers of cis-isoalliin are also separated and purified by RP-HPLC.
PMCID: PMC3970760  PMID: 25187757
Allium cepa; 1-propenyl-L-cysteine sulfoxide; organosulfur compounds
13.  Profile of Vitiligo in Kumaun Region of Uttarakhand, India 
Vitiligo is a common, acquired, pigmentary disorder characterized by loss of melanocytes resulting in white spots. This disease carries a lot of social stigma in India.
To study the clinico-epidemiological profile of vitiligo patients in Kumaun region of Uttarakhand state in India.
Materials and Methods:
The clinical presentation of vitiligo was examined and analyzed in 762 vitiligo patients attending the Dermatology outdoor of Government Medical College, Haldwani, which is a referral centre for Kumaun region of Uttarakhand state in India.
Male and female patients were found to be affected almost equally. It was observed that onset of vitiligo was most common in 0-10 years age group, as evidenced by 336 cases out of 762 cases. Acrofacial type of vitiligo (339 cases out of 762) was most commonly observed, followed by vitiligo vulgaris, focal, segmental, mucosal, mixed, and universal vitiligo. The most common site of onset was the lower limbs followed by head and neck, upper limbs, trunk, genitalia, and mucasae. Leucotrichia was observed in 33.5%, Koebner's phenomenon in 26.3%, and a positive family history in 19% of the vitiligo patients. The other common conditions associated were thyroid disorders (8.9%), diabetes (5.3%), and atopic dermatitis (4.9%).
The study indicates that acrofacial vitiligo is the most common clinical type observed in Kumaun region of Uttarakhand in India. Onset of vitiligo is most common in first decade of life.
PMCID: PMC3969695  PMID: 24700953
Clinico-epidemiological profile; Uttarakhand; vitiligo
14.  Development of a Novel BAFF Responsive Cell Line Suitable for Detecting Bioactive BAFF and Neutralizing Antibodies against BAFF-Pathway Inhibiting Therapeutics 
Cells  2014;3(1):79-91.
BAFF has a critical role in B-cell survival, maturation and function, which makes its pathway a prime therapeutic target for various autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and Sjögren’s syndrome. A cell-based assay that measures the functional activity of BAFF is required for many high throughput purposes, such as lead target screening and BAFF quantification. We report here the development of a sensitive BAFF responsive cell line via stable transfection of the BAFFR-TNFR1 hybrid receptor into monkey kidney epithelial COS-1 cells. The cellular response to BAFF can be detected by measuring the secretion of IL-8. This BAFF bioassay is not only reproducible and sensitive, but also responsive to a wide concentration range of BAFF stimulation in sera from various species. This cell line is useful in the development of sensitive bioassays to measure the levels of bioactive BAFF, inhibition of BAFF and neutralizing antibodies against any BAFF pathway-mediated therapeutic proteins.
PMCID: PMC3980743  PMID: 24709903
hybrid receptor; neutralizing antibody detection; BAFF response; serum matrix; transfection; COS-1 cell line
15.  The attitudes and perceptions of medical students towards basic science subjects during their clinical years: A cross-sectional survey 
In the conventional system of medical education, basic subjects are taught in the 1st year with least interdisciplinary interaction. The objective of this study was to explore the students’ perception about content, need and application of basic science subjects during the clinical years of their medical education.
Materials and Methods:
A total of 300 questionnaires were distributed among students randomly after taking their written consent for participation in the study. About 265 completely filled questionnaires were received back and the response was analyzed.
Students identified anatomy as the subject with overloaded syllabus (75.4%) and also with maximum clinical application with 50.1% of them considering it the most important basic subject. Students were satisfied with the practical integration of subjects to impart clinical skills, but considered problem based learning a better method of teaching. According to 37%, 43.8% and 33.2% of respondents respectively; anatomy, biochemistry and physiology curriculum should only cover the general concepts to give the working knowledge of the subject. Approximately, 65% of the respondents were able to recall the knowledge of anatomy and physiology while biochemistry was retained by 40%.
Overall, the attitudes of students toward basic science subjects were positive. The learning experience for them can be improved significantly by better clinical integration of the subjects.
PMCID: PMC3931207  PMID: 24600572
Basic science subjects; medical students; perceptions
16.  Association between Alcohol Screening Scores and Mortality in Black, Hispanic, and White Male Veterans 
AUDIT-C alcohol screening scores are associated with mortality, but whether or how associations vary across race/ethnicity is unknown.
Self-reported black (n=13,068), Hispanic (n=9,466), and white (n=182,688) male VA outpatients completed the AUDIT-C via mailed survey. Logistic regression models evaluated whether race/ethnicity modified the association between AUDIT-C scores (0, 1–4, 5–8, and 9–12) and mortality after 24 months, adjusting for demographics, smoking, and comorbidity.
Adjusted mortality rates were 0.036, 0.033, and 0.054, for black, Hispanic, and white patients with AUDIT-C scores of 1–4, respectively. Race/ethnicity modified the association between AUDIT-C scores and mortality (p=0.0022). Hispanic and white patients with scores of 0, 5–8, and 9–12 had significantly increased risk of death compared to those with scores of 1–4; Hispanic ORs: 1.93, 95% CI 1.50–2.49; 1.57, 1.07–2.30; 1.82, 1.04–3.17, respectively; white ORs: 1.34, 95% CI 1.29–1.40; 1.12, 1.03–1.21; 1.81, 1.59–2.07, respectively. Black patients with scores of 0 and 5–8 had increased risk relative to scores of 1–4 (ORs 1.28, 1.06–1.56 and 1.50, 1.13–1.99), but there was no significant increased risk for scores of 9–12 (ORs 1.27, 0.77–2.09). Post-hoc exploratory analyses suggested an interaction between smoking and AUDIT-C scores might account for some of the observed differences across race/ethnicity.
Among male VA outpatients, associations between alcohol screening scores and mortality varied significantly depending on race/ethnicity. Findings could be integrated into systems with automated risk calculators to provide demographically-tailored feedback regarding medical consequences of drinking.
PMCID: PMC3443543  PMID: 22676340
alcohol; race; ethnicity; mortality; AUDIT-C
17.  Turmeric - A new treatment option for lichen planus: A pilot study 
Turmeric is dried rhizome of the perennial herbs curcumalonga. It is called Haldi in Hindi, turmeric in English, ukon in Japanese. It has been used in Asian Medicine since the second millennium BC. It's utility is referred to in the ancient Hindu script the Ayurveda. Pathogenesis of the OLP should be taken in consideration for the treatment point of view. The Cell mediated immunity to secondary antigenic change in oral mucous membrane is thought to play a major role in its pathogenesis modified keratocyte surface antigens are the primary target for cytotoxic cellular response. Curcumin also been shown to have immune modulatory effect involving activation of host macrophages and natural killer cells and modulation of lymphocytes mediated function.
PMCID: PMC3961895  PMID: 24665176
Antioxidants; lichenplanus; turmeric
18.  Does LDL-C Estimation Using Anandaraja’s Formula Give a Better Agreement with Direct LDL-C Estimation than the Friedewald’s Formula? 
Estimation of low density lipoprotein cholesterol (LDL-C) is crucial in management of coronary artery disease patients. Though a number of homogenous assays are available for estimation of LDL-C, use of calculated LDL-C by Friedewald’s formula (FF) is common in Indian laboratories for logistic reasons. Recently Anandaraja and colleagues have derived a new formula for calculating LDL-C. This formula needs to be evaluated before it is extensively applied in diagnosis. We measured LDL-C by homogenous method (D-LDL-C) in 515 fasting samples. Friedewald’s and Anandaraja’s formulas were used for calculation of LDL-C (F-LDL-C and A-LDL-C, respectively). The mean LDL-C levels were 123.3 ± 53.2, 112.4 ± 50.2 and 109.2 ± 49.8 mg/dl for D-LDL-C, F-LDL-C and A-LDL-C, respectively. There was a statistically significant difference between the results (P > 0.001) obtained by calculation formulas compared to the measured LDL-C. There was underestimation of LDL-C by 10.8 and 14 mg/dl by Friedewald’s and Anandaraja’s formulas respectively. The Pearson’s correlation between F-LDL-C and D-LDL-C was 0.931 and that between A-LDL-C and D-LDL-C was 0.930. Bland–Altman graphs showed a definite agreement between mean and differences of the calculation formulas and direct LDL-C with 95% of values lying with in ±2 SD limits. The mean percentage difference (calculated as {(Calculated LDL-C)-(D-LDL-C)}/D-LDL-C × 100) for F-LDL-C was maximum (−11.6%) at HDL-C ≥ 60 mg/dl and TG levels of 200–300 mg/dl (−10.4%) compared to D-LDL-C. A-LDL-C results gave highest mean percentage difference at total cholesterol concentrations <100 mg/dl (−37.3%) and HDL-C < 40 mg/dl (−17.1%), respectively. The results of our study showed that FF is better in agreement with D-LDL-C than Anandaraja’s formula for estimation of LDL-C by calculation though both lead to its underestimation.
PMCID: PMC3358371  PMID: 23543806
LDL-C; Friedewald’s formula; Anandaraja’s Formula
Hypertension  2012;59(3):665-672.
Cardiotonic steroids signaling through the basolateral sodium pump (Na/KATPase) have been shown to alter renal salt handling in intact animals. As the relationship between renal salt handling and blood pressure is a key determinant of hypertension, and patients with insulin resistance are frequently hypertensive, we chose to examine whether there might be competition for resources necessary for receptor mediated endocytosis.
In LLC-PK1 cells, the Na/K-ATPase-α1 and carcinoembryonic antigen cell adhesion molecule (CEACAM1), a plasma membrane protein that promotes receptor-mediated endocytosis, co-localized in the plasma membranes and translocated to the intracellular region in response to ouabain. Either ouabain or insulin alone caused accumulation of CEACAM1 as well as IRβ, and EGFR in early endosomes, but no synergy was demonstrable. Like ouabain, insulin also caused c-Src activation. When caveolin or Na/K-ATPase-α1 expression was knocked down with siRNA, insulin but not ouabain induced CEACAM1, IRβ, and EGFR endocytosis.
To determine whether this might be relevant to salt handling in vivo, we examined salt loading in mice with null renal CEACAM2 expression (Cc2−/−). The Cc2−/− animals demonstrated greater increases in blood pressure with increases in dietary salt than control animals.
These data demonstrate that cardiotonic steroids and insulin compete for cellular endocytosis resources and suggest that under conditions where circulating insulin concentrations are high, cardiotonic steroid mediated natriuresis could be impaired.
PMCID: PMC3336087  PMID: 22311908
chronic renal insufficiency; renal proximal tubule cell; endocytosis
20.  Lemierre's Syndrome: Rare, but Life Threatening—A Case Report with Streptococcus intermedius 
Case Reports in Medicine  2012;2012:624065.
Lemierre's syndrome (LS) is a rare, but a life-threatening complication of an oropharyngeal infection. Combinations of fever, pharyngitis, dysphagia, odynophagia, or oropharyngeal swelling are common presenting symptoms. Infection of the lateral pharyngeal space may result in thrombosis of the internal jugular vein, subsequent metastatic complications (e.g., lung abscesses, septic arthritis), and significant morbidity and mortality. LS is usually caused by the gram-negative anaerobic bacillus Fusobacterium necrophorum, hence also known as necrobacillosis. We present a case of LS caused by Streptococcus intermedius, likely secondary to gingival scraping, in which the presenting complaint was neck pain. The oropharyngeal examination was normal and an initial CT of the neck was done without contrast, which likely resulted in a diagnostic delay. This syndrome can be easily missed in early phases. However, given the potential severity of LS, early recognition and expedient appropriate antimicrobial treatment are critical. S. intermedius is an unusual cause of LS, with only 2 previous cases being reported in the literature. Therefore, an awareness of the myriad presentations of this syndrome, which in turn will lead to appropriate and timely diagnostic studies, will result in improved outcome for LS.
PMCID: PMC3502875  PMID: 23197986
21.  Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B 
Aglossia congenita (AC), congenital total absence of the tongue, is a very rare midline developmental anomaly, hypothesized to be associated with vascular disruption between the fourth and eighth week of gestation. It was classified by Hall (1971) as part of oromandibular limb hypogenesis syndrome (OLHS) type I B. Most of the cases reported with OLHS are actually hypoglossia with limb abnormalities whereas isolated aglossia is an extremely rare entity. A case of isolated AC is presented in a 28-year-old Indian male. He had long narrow face, tapering chin, low set ears, and microstomia. Intraorally, he had narrow palatal vault, constricted oropharyngeal isthmus, oligodontia, and maxillo-mandibular hypoplasia. Interestingly, the patient showed a median palatal groove, which has not been reported before. He also had an unusual acquired adaptive mechanism to compensate for aglossia. This report presents the manifestations of this rare syndrome, its complications, differential diagnosis, and rehabilitation strategies.
PMCID: PMC3519220  PMID: 23248477
Adaptation; anomaly; developmental; palate; rehabilitation; tongue
22.  Parenchymal Expression of CD40 Exacerbates Adenovirus-Induced Hepatitis 
Hepatology (Baltimore, Md.)  2011;53(5):1455-1467.
The healthy adult human liver expresses low levels of MHC II and undetectable levels of immune co-stimulatory molecules. However, high levels of MHC class II, CD40 and B7 family molecules are expressed in the activated Kupffer cells and hepatocytes of patients having viral hepatitis. The precise role of these molecules in viral clearance and immune-mediated liver injury is not well understood. We hypothesize that parenchymal CD40 expression enhances T-cell recruitment and effector functions, which may facilitate viral clearance and alleviate liver injury. To test this hypothesis, we generated novel, liver-specific, conditional CD40 transgenic mice, and challenged them i.v. with recombinant replication-deficient adenovirus carrying Cre recombinase (AdCre). Wild-type mice infected with AdCre developed a relatively mild course of viral hepatitis and recovered spontaneously. CD40 expression in the liver of transgenic animals, however, resulted in CD80 and CD86 expression. Dysregulation of population dynamics and effector functions of intrahepatic lymphocytes results in severe lymphocytic infiltration, apoptosis, necroinflammation, and serum alanine transferase (ALT) elevation in a dose-dependent fashion. To our surprise, an early expansion followed by a contraction of intrahepatic lymphocytes, especially CD8+ and NK cells, accompanied by increased granzyme B and IFN-γ production, did not lead to a faster viral clearance in CD40 transgenic mice. Conclusion: Our results demonstrated that hepatic CD40 expression does not accelerate adenoviral clearance, but rather exacerbates liver injury. This study unveils a previously unknown deleterious effect of hepatic CD40 in adenovirus-induced liver inflammation.
PMCID: PMC3082591  PMID: 21360722
liver; animal models; T lymphocytes and co-stimulation
23.  Syndontia with talon cusp 
Teeth are specialized structural components of the craniofacial skeleton. Developmental defects occur either alone or in combination with other birth defects. Macrodontia of anterior teeth may occur as an isolated condition or as a result of fusion or gemination and can occur in the primary or permanent dentition. Fusion is more commonly seen in the anterior maxillary region. This case presentation reports a case of fusion of a supplemental tooth to one in the normal series in conjunction with a talon cusp. This condition is extremely rare and has been reported at fourth occasion in the literature. The etiology, prevalence, clinical features, and management of the aforementioned anomalies have been reviewed in detail. Early diagnosis of this condition is important because it may cause clinical problems, such as esthetic concerns and tooth crowding.
PMCID: PMC3424946  PMID: 22923902
Fusion; gemination; macrodontia; talon cusp
24.  Hypohidrotic ectodermal dysplasia 
Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disorder characterized by the faulty development of the ectodermal structure, resulting in most notably anhydrosis/hypohydrosis, hypotrichosis and hypodontia. The condition is usually an X-linked recessive disorder affecting predominantly males. We are here reporting a classical case of hypohidrotic ectodermal dysplasia with a review of the literature.
PMCID: PMC3481883  PMID: 23130287
Ectodermal dysplasia; genetic disorder; hypohidrotic
25.  Are we using Thyroid Function Tests Appropriately? 
Thyroid function tests are very important for the diagnosis and monitoring of patients with thyroid dysfunction. The guidelines recommend serum thyroid stimulating hormone (TSH) as the single most reliable test to diagnose all common forms of hypothyroidism and hyperthyroidism. The aim of this study was to analyze the ordering pattern for thyroid function tests by physicians and the analysis of results based on the clinical history. The mean age of the patients was 32.5 ± 6.5 years. Majority of samples (87.7% of total) were received from the departments of Medicine and Gynae. Thyroid profiles (47.5%) were ordered more frequently as compared to TSH only (46%). There was no significant difference in the percentage of normal reports for both types of tests. 77.8% of TFT and 76.6% of TSH samples had results within the reference range. The percentage of abnormal results was 13.7% in the patients who were screened for thyroid disorders. There is a need to redefine the case definition for thyroid dysfunction and order the appropriate test in a rational and cost effective manner.
PMCID: PMC3107420  PMID: 22468046
Thyroid function test; TSH; Hypothyroidism; Hyperthyroidism

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