Unusual presentation of adamantinoma with synchronous involvement of entire-lengths of the tibia and fibula in an elderly man: A case report.

INTRODUCTION AND IMPORTANCE: Adamantinoma is a rare primary low-grade malignant bone tumor with a median age of 20 to 30 years with a specific predilection to the lower 2/3rd shaft of the tibia. We present an unusual presentation of a giant adamantinoma with synchronous involvement of almost entire lengths of the tibia and fibula and extensive to the skin in a geriatric man. CASE PRESENTATION: An elderly male patient in their late 50s presented to us with a grossly deformed left leg with a fungating mass over the left leg for 5 years. X-rays showed a lytic sclerotic lesion with a honeycomb appearance involving the entire length of the tibia and fibula. Magnetic Resonance Imaging showed a heterogeneous altered signal intensity (T1 isointense and T2 heterogeneous hyper-intense lesion) large lobulated lesion involving the entire length of the leg with lytic destruction of the entire tibia and fibula and associated remodeling. The histopathological examination revealed an Invasive tumor composed of both epithelial and mesenchymal elements. On immunohistochemistry, tumor cells were positive for D240 and negative for CD31. After confirming the diagnosis of adamantinoma of tibia and fibula radical resection of the tumor was planned and performed in the form of above-knee amputation. The patient was disease-free at 18 months of the latest follow-up and walking with the above knee prosthesis comfortably without any assistance. CLINICAL DISCUSSION: Two morphological patterns of adamantinoma on MRI have been described, a solitary lobulated focus and a pattern of multiple small nodules in one or more foci. Our case has demonstrated the second type of morphology. Histologically, this case presented with "classical basaloid type epithelial cells embedded in osteofibrous dysplasia-like stroma." CONCLUSION: The diagnosis of adamantinoma was based on the clinical-radiological findings and histo-morphology, and should be confirmed by immunohistochemistry for demonstrating epithelial cells. Ultra-structural analysis and Cytogenetic studies may be required in the cases of unusual presentation of these tumors. Wide local resection is the preferred treatment.

Comparison of efficacy and safety of 30% hydrogen peroxide with 50% trichloroacetic acid in seborrheic keratosis: a randomized controlled study.

BACKGROUND: Seborrheic keratosis is a benign skin tumour. None of the common therapies is satisfactory. This study compared the efficacy of 30% hydrogen peroxide versus 50% trichloroacetic acid in treatment of seborrheic keratoses. METHODS: This unblinded, comparative interventional study was conducted on 85 patients of seborrheic keratosis. Lesions were graded using physician's lesion assessment (PLA) scale (0, clear; 1, nearly clear; 2≤1 mm thick; 3≥1 mm thick). Patients were randomly assigned into two groups: A and B. Five target seborrheic keratosis lesions with PLA score of three were selected in each patient. Group A was treated with 30% hydrogen peroxide and group B with 50% trichloroacetic acid. Both groups received treatment every week up to 5 weeks or till complete resolution of lesions. Clinical photographs of all patients were taken every 2 weeks. RESULTS: Both the groups were comparable in terms of age and sex. At the end of 6 weeks, lesions were cleared completely in 41.8% of patients in group A and in 23.8% in group B. Mean PLA score decreased significantly in group A from 3 to 1.79 (40.3%) at 2 weeks and to 0.63 (79%) at 6 weeks (P<0.001). The decrease in PLA score in group B was 2.41 (19.7%) and 1.13 (62.3%) at 2, and 6 weeks respectively (P<0.001). Hydrogen peroxide produced greater clearance of seborrheic keratosis (P=0.017) than trichloroacetic acid. Also, no major adverse effects were observed in the two groups. CONCLUSIONS: Application of 30% hydrogen peroxide appears to be more efficacious as compared to trichloroacetic acid (50%) for treatment of seborrheic keratosis.

Identification and characterization of 30 novel pathogenic variations in 69 unrelated Indian patients with Mucolipidosis Type II and Type III.

Mucolipidosis (ML) (OMIM 607840 & 607838) is a rare autosomal recessive inherited disorder that occurs due to the deficiency of golgi enzyme uridine diphosphate (UDP)- N-acetylglucosamine-1-phosphotransferase (GlcNAc-phosphotransferase) responsible for tagging mannose-6-phosphate for proper trafficking of lysosomal enzymes to lysosomes. Variants in GlcNAc-phosphotransferase (GNPTAB (α, ß subunits) and GNPTG (γ subunits) are known to result in impaired targeting of lysosomal enzymes leading to Mucolipidosis (ML) Type II or Type III. We analyzed 69 Indian families of MLII/III for clinical features and molecular spectrum and performed in silico analysis for novel variants. We identified 38 pathogenic variants in GNPTAB and 5 pathogenic variants in GNPTG genes including missense, frame shift, deletion, duplication and splice site variations. A total of 26 novel variants were identified in GNPTAB and 4 in GNPTG gene. In silico studies using mutation prediction software like SIFT, Polyphen2 and protein structure analysis further confirmed the pathogenic nature of the novel sequence variants detected in our study. Except for a common variant c.3503_3504delTC in early onset MLII, we could not establish any other significant genotype and phenotype correlation. This is one of the largest studies reported till date on Mucolipidosis II/III in order to identify mutation spectrum and any recurrent mutations specific to the Indian ethnic population. The mutational spectrum information in Indian patients will be useful in better genetic counselling, carrier detection and prenatal diagnosis for patients with ML II/III.

The Impact of Onychopathies on Quality of Life: A Hospital-based, Cross-sectional Study.

BACKGROUND: Onychopathies or nail disorders are associated with social stigma and causes limitation of daily activities by hampering the function of both fingers and toes. AIM: To evaluate the impact of onychopathies on quality of life (QoL) and compare the severity of impact on QoL in various nail disorders. MATERIALS AND METHODS: A hospital-based cross-sectional study consisting of 540 patients with onychopathies was conducted in the dermatology outpatient department. Patients were requested to complete a nail-specific QoL questionnaire consisting of 24 and 16 questions, respectively, for fingernails (group F) and toenails (group T) with five possible responses to each question. A score of 1-5 was given to each response. Statistical analysis was done to compare the impact of QoL on the different types of onychopathies. RESULTS: We found that onychopathies have a significant impact on QoL. QoL was significantly more affected when multiple nails were involved (P = 0.020 for group F and P = 0.001 for group T). QoL impact was statistically more significant in women (P = 0.038 for group F and P < 0.001 for group T) and in younger people aged <20 years in group F and 20-39 years in group T (P < 0.001 for both groups F and T). Patients with onychomycosis, structural nail defects, and psoriasis had a more significant impact than other diseases (P < 0.001 for both groups F and T). CONCLUSION: Onychopathies have a significant adverse effect on QoL because of their serious physical, psychological and social impact. Hence, clinicians should treat the nail disorders with utmost seriousness.

New vision for improving oral hygiene status of visually impaired students aged from 9 to 17 years.

BACKGROUND: Visually impaired patients are at a higher risk of developing periodontal disease because of greater difficulty in attaining good oral hygiene. This study aims to assess and compare the oral hygiene status of visually impaired students before and after oral health education interventions using special customized methods. METHODS: The present study was a randomized control trial of 180 visually impaired students divided into three groups. Each group includes 60 students selected randomly from blind school. Oral health education was given using Braille in Group 1, Audio Tactile performance (ATP) technique in Group 2, and a combination of Braille and ATP technique in Group 3. Plaque index (PI) scores and gingival index (GI) scores were calculated and evaluated at baseline and after 3 months. Intergroup comparison and intragroup comparison of PI and GI at baseline and 3 months was by using one way ANOVA and Paired t test, respectively. RESULTS: There was a highly significant difference seen for the intergroup comparison of post PI (P < 0.01) and post GI (P < 0.01) with least mean in Group 3. There was a statistically highly significant difference seen for the intra group comparison of pre and post PI and GI (P < 0.01) with lesser means in post as compared to pre in all three groups. CONCLUSIONS: Visually impaired children could maintain an acceptable level of oral hygiene when taught using combination of Braille and ATP technique.

Effects and association of pro-oxidants with magnesium in patients with diabetic nephropathy.

Diabetic nephropathy (DN) is the most common microvascular complication observed in patients with type-2 diabetes mellitus. Furthermore, magnesium (Mg) deficiency is a common problem in diabetic patients. In this study, we estimated the levels of Mg, which is an important trace element and pro-oxidant marker, and then evaluated the association between serum Mg and pro-oxidants in patients with DN. In the present study, 200 patients were enrolled and were divided into two groups. The control and DN groups consisted of 100 healthy individuals and 100 patients with DN, respectively. Serum Mg, total anti-oxidant capacity (TAC), and superoxide dismutase (SOD) levels were estimated using the Calmagite, Koracevic, and Marklund and Marklund methods, respectively. Glutathione (GSH) and malondialdehyde (MDA) levels were estimated using the Tietze F and Jean CD method, respectively. Mg levels were found to be significantly decreased in the DN group in comparison to the control group. Anti-oxidant markers were statistically significantly reduced (P <0.001), whereas MDA levels were statistically significantly elevated (P <0.001) in the DN group compared to the control group. There was a significant positive association of Mg with TAC, SOD, and GSH. A statistically significant negative association of Mg with MDA (r = -0.302, P <0.001, n = 100) was also observed. An apparent relationship was observed between hypomagnesemia and oxidative stress in patients with DN. Lower levels of Mg and oxidative stress were also strongly linked.

Association of Dyslipidemia, Increased Insulin Resistance, and Serum CA 15-3 with Increased Risk of Breast Cancer in Urban Areas of North and Central India.

OBJECTIVE: This study aims to determine the association of dyslipidemia and increased insulin resistance (IR) with increased breast cancer (BC) risk. MATERIALS AND METHODS: The study group comprised 110 premenopausal and 143 postmenopausal, untreated female BC patients in the age range of 29-72 years. Control group consisted of 117 premenopausal and 141 postmenopausal healthy females in the age range of 23-75. Approximately 8-ml blood samples were drawn to measure various biochemical parameters. Serum glucose, total cholesterol, triglyceride (TG), and high-density lipoprotein-cholesterol were measured. Very low-density lipoprotein-cholesterol (VLDL-C) and LDL-C were calculated using Friedewald's formula. Serum insulin and serum CA 15-3 were estimated by immune enzymatic assay. IR was assessed using homeostasis model assessment IR index (HOMA-IR). RESULTS: Clinical variables in the case and control groups were compared using the unpaired Student's t-test. The crude and adjusted odds ratios (ORs) and 95% confidence intervals (CIs) were calculated by binary logistic regression analysis. Pearson's correlation analysis was used to determine the association between CA 15-3 and variables of interest. Total cholesterol, TG, LDL, VLDL, serum glucose, serum insulin, HOMA-IR, and serum CA 15-3 were significantly higher (P < 0.001) in BC patients compared to those in controls. Significant adjusted ORs with 95% CI were found to be fasting glucose, total cholesterol, and TGs. We also found a significant positive correlation between total cholesterol, TG, LDL, serum glucose, serum insulin, HOMA-IR, and serum CA 15-3. CONCLUSION: This study confirms the association between dyslipidemia, IR, and increased BC risk.

Analysis of the Clinical Profile in Patients with Plasmodium falciparum Malaria and Its Association with Parasite Density.

BACKGROUND: Malaria remains a major health hazard in the modern world, particularly in developing countries. In Plasmodium falciparum malaria, there is a direct correlation between asexual erythrocytic stage parasite density and disease severity. Accordingly, the correlations between parasite density and various clinical presentations, severity, and outcome were examined in falciparum malaria in India. MATERIALS AND METHODS: The study was conducted in a tertiary health-care center in North India. Of 100 cases of falciparum malaria, 65 patients were male and 35 were female. A total of 54 patients were in the uncomplicated group and 46 patients were in the complicated malaria group. RESULTS: Fever, anemia, icterus, splenomegaly, hepatomegaly, and hepatosplenomegaly were common clinical findings. All clinical findings were significantly more common in the complicated malaria group and patients with a high parasite density than in the uncomplicated group and those with a low parasite density. All patients in the uncomplicated malaria group had a parasite density of <5% while most patients in the complicated malaria group had a parasite density of >5%, and the difference between groups was statistically significant. The incidence of cerebral malaria was significantly higher in cases with a high parasite density; 58.33% mortality was observed in these cases. Cerebral malaria and hyperbilirubinemia was the most frequently encountered combination of complications. CONCLUSIONS: In P. falciparum malaria, parasite density was associated with complications and poor clinical outcomes. These results may inform treatment decisions and suggest that a threshold parasite density of 5% is informative.

Study of the Stability of Various Biochemical Analytes in Samples Stored at Different Predefined Storage Conditions at an Accredited Laboratory of India.

BACKGROUND: Storage of serum and other blood products is often necessary in laboratories because of technical issues or to preserve samples for subsequent research purposes. The aim of this study was to determine whether the stability of biochemical analytes is affected by storage conditions. MATERIALS AND METHODS: A total of 17 biochemical analytes in the sera of ten patients were examined following storage. Subsequent to determining the baseline measurements, the serum of each patient was aliquoted and stored at -20°C for 7, 15, and 30 days and then analyzed for stability. The results were compared with the initial analysis measurements obtained from fresh samples. Mean changes compared to baseline (T0) concentrations were evaluated both statistically and clinically. RESULTS: Our results show that sodium, potassium, urea, creatinine, uric acid, total calcium, phosphorus, direct bilirubin, total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, total protein, albumin, cholesterol, and triglyceride levels were stable under all conditions. Serum amylase was the only analyte demonstrating instability following prolonged storage; amylase levels changed significantly (both statistically and clinically) at 7, 15, and 30 days (P < 0.05). CONCLUSION: Most common biochemical analytes, except for amylase, showed adequate stability in serum following 30 days of storage at -20-C. Serum amylase analysis should be conducted on the same day that the sample is received in the laboratory.

Skin Hyperpigmentation in Indian Population: Insights and Best Practice.

Skin pigmentation is one of the most strikingly variable phenotypes in humans, therefore making cutaneous pigmentation disorders frequent symptoms manifesting in a multitude of forms. The most common among them include lentigines, postinflammatory hyperpigmentation, dark eye circles, and melasma. Variability of skin tones throughout the world is well-documented, some skin tones being reported as more susceptible to pigmentation disorders than others, especially in Asia and India. Furthermore, exposure to ultraviolet radiation is known to trigger or exacerbate pigmentation disorders. Preventive strategies for photoprotection and treatment modalities including topical and other medical approaches have been adopted by dermatologists to mitigate these disorders. This review article outlines the current knowledge on pigmentation disorders including pathophysiology, molecular profiling, and therapeutic options with a special focus on the Indian population.

Implication of HLA-G 5' upstream regulatory region polymorphisms in idiopathic recurrent spontaneous abortions.

The effect of HLA-G 5'-upstream regulatory region (URR) single nucleotide polymorphisms (SNP) in idiopathic recurrent spontaneous abortion (RSA) was evaluated. Parental genotype combination analysis and HLA-G expression at transcriptional level was evaluated for 5'URR SNP, which have shown increased risk for idiopathic RSA. If a fetus were aneuploid, attributing causation to a HLA-G 5'-URR SNP would be illogical; therefore couples with abnormal parental karyotypes and also those with abortus material that revealed chromosomal abnormalities were excluded. One hundred women who had experienced idiopathic RSA, along with their respective male partners and 100 pairs of control couples, were studied. HLA-G 5'-URR SNP were evaluated through sequencing. Quantitative polymerase chain reaction was used for HLA-G expression analysis. An increased risk for idiopathic RSA cases among women carriers of mutant genotypes of -1179G>A(rs1233335), -725C>G/T(rs915670) and -486A>C(rs114252012) SNP. The parental genotype combination analysis revealed a 3.5-fold increased risk for -1179G>A and 4.3-fold increased risk for -725C>G/T SNP among carriers of mutant parental genotypes in couples who have experienced idiopathic RSA. Down-regulation in HLA-G expression was seen at transcriptional level for -1179G>A and -725C>G/T SNPs in cases of idiopathic RSA. Transmission of a mutant allele from single carrier parents may, therefore, affect pregnancy outcome.

A quick method to screen high and low yielding wheat cultivars exposed to high temperature.

In tropical countries, high temperature stress is the major abiotic stress, which controls the productivity and yield of crop plants. Two high yielding and low yielding genotypes of durum wheat were selected for detailed analysis of their photochemical efficiencies. In low yielding genotypes (Malvi local and Sawer local), the whole primary photochemical reactions are affected before and after heat stress. The results show that low yielding genotypes show less efficiency in the usage of the available excitation energy. This is a case study to establish use of chlorophyll a fluorescence measurement as an effective tool to screen plants for their stress tolerance. The study is important for stress physiology and may be useful for assessment of stress tolerant plants.

Study of incidence and prevalence of musculoskeletal anomalies in a tertiary care hospital of eastern India.

INTRODUCTION: Congenital anomalies or malformations are anatomical. Structural or functional defects present at birth leads to physical and mental disabilities. With the advent of newer drugs, infectious diseases have taken the backseat and congenital defects have emerged as an important cause of neonatal morbidity and mortality. In India, anomalies of musculoskeletal system have been most commonly reported. AIMS AND OBJECTIVES: With this in view, the present study was carried out to find the incidence and types of musculoskeletal defects in a tertiary care hospital in Eastern India. Various maternal factors were also correlated and analyzed. The purpose of this communication is to report these defects so as to help doctors and parents to prevent unexpected fetal loss and better parental counseling. MATERIALS AND METHODS: This study was done in the Department of Obstetrics and Gynaecology in association with Department of Paediatrics of a tertiary care hospital in Odisha from for a period of 1 year. The newborns were examined within 1(st) three days of delivery for congenital malformations. The study group included all live borns along with still borns after 28 weeks of gestation or those dead babies whose weight was about 1kg. A thorough physical examination from head to toe was done to look for musculoskeletal defects within 24hrs of delivery. OBSERVATION AND RESULTS: Out of total 7268 babies delivered, 116 babies were found to have anomalies. Thirty two of these had musculoskeletal defects. Talipes equinovarus was the commonest anomaly observed followed by polydactyly. The ratio of male babies with musculoskeletal defects to female babies was found to be 1.28:1.The malformed babies were mainly born to term mothers (77.6%), who were mostly unbooked (62.5%) and belonged to lower or middle class. But no significant relationship could be established between these factors and defects. Most mothers were in the age group of 20-35 years and there was no history of drug addiction, smoking, trauma or irradiation that could be related to the occurrence of congenital malformations. CONCLUSION: It is concluded that better maternal care and improved standards of living have very little effect on the overall frequency of congenital malformations. For the better future of neonates an early recognition of correctable lesions is essential, which calls for a systematic approach to the study of musculoskeletal defects.

Photosynthesis: response to high temperature stress.

Global warming has led to increased temperature of the earth which is a major abiotic stress posing a serious threat to the plants. Photosynthesis is amongst the plant cell functions that is highly sensitive to high temperature stress and is often inhibited before other cell functions are impaired. The primary sites of targets of high temperature stress are Photosystem II (PSII), ribulose-1,5-bisphosphate carboxylase/oxygenase (Rubisco) while Cytochrome b559 (Cytb559) and plastoquinone (PQ) are also affected. As compared to PSII, PSI is stable at higher temperatures. ROS production, generation of heat shock proteins, production of secondary metabolites are some of the consequences of high temperature stress. In this review we have summarized the physiological, biochemical and molecular aspects of high temperature stress on the process of photosynthesis, as well as the tolerance and adaptive mechanisms involved.

Success of initial and repeated medial branch neurotomy for zygapophysial joint pain: a systematic review.

OBJECTIVES: To review the duration of pain relief after initial and repeated radiofrequency neurotomy (RFN) for cervical and lumbar zygapophysial joint pain. METHODS: We searched PubMed to identify all articles that met review criteria for in-depth analysis, synthesis, and review. RESULTS: Data from 16 articles are reported in this review, including 8 cervical studies, 7 lumbar studies, and 1 study of both cervical and lumbar treatment. Overall, methodology and design quality of cervical studies exceeded that of lumbar studies. For initial cervical RFN, average range duration of >50% pain relief was 7.3-8.6 months. Repeated cervical RFN was successful 67%-95% of the time when the first RFN procedure was successful. When the first RFN procedure was unsuccessful, repeated RFN was successful 0%-67% of the time. The average range duration of pain relief after successful repeated RFN was 6.0-12.7 months. For initial lumbar RFN, the average duration of >50% pain relief was 9.0 months. Repeated lumbar RFN was successful 33%-85% of the time when the first RFN procedure was successful. The average duration of pain relief after successful repeated lumbar RFN was 11.6 months. CONCLUSIONS: The results of this review indicate that pain relief after initial RFN generally ends after 7-9 months and that repeating RFN is likely to provide additional pain relief if initial RFN was successful. Results are similar between cervical and lumbar spine studies.

Computational analysis of fluorescence induction curves in intact spinach leaves treated at different pH.

Effects of change in pH have been investigated on spinach leaf discs by measuring fluorescence induction kinetics using plant efficiency analyzer (PEA). On the basis of computational analysis of the results, we have reported that acidic pH causes a significant inhibition of the donor and the acceptor side of PS II. Energy flux models have been presented using the software Biolyzer HP 3. Effects of pH were investigated on the antenna size heterogeneity of PS II and a relative change in the proportions of α, ß, and γ centers was observed.

Intramolecular scrambling of aryl groups in organopalladium complexes [ArPd(PPh3)2]+: from solution to the gas phase, back again, and in-between.

Gas-phase experiments are used to probe the intramolecular scrambling of aryl groups between palladium and phosphorus in organopalladium complexes, [ArPd(PPh(3))(2)](+), generated by means of electrospray ionization (ESI). To this aim, ESI mass spectrometry, including tandem mass spectrometric experiments, were carried out on deuterated, non-deuterated, and substituted [ArPd(PPh(3))(2)](+) complexes. The fragment ions obtained from the deuterated parent ions clearly show the occurrence of intramolecular scrambling between the aryl group bound to palladium and the phenyl groups of the phosphine in the gas phase. Fragmentation pathways, supported by a statistical model, are proposed to explain these migrations and the implications for the condensed-phase chemistry are probed experimentally by using ESI mass spectrometry.

Silylmethyl-substituted cyclopropyl and other strained ring systems: cycloaddition with dipolarophiles.

Small ring compounds represent a class of versatile building blocks in organic synthesis. Three- and four-membered ring carbo- and heterocycles are regarded as unique functional groups. Lewis acid-assisted cycloaddition of cyclopropanes, aziridines and azetidines substituted by vicinal electron-donor and electron-acceptor groups takes place in a regio- and stereocontrolled fashion. Trialkylsilylmethyl is an interesting donor substituent. In this feature article, we provide an overview of the cycloaddition of different dipolarophiles to silylmethyl-substituted small ring compounds and discuss their possible applications in synthesis.