Infantile or Malignant osteopetrosis is a rare congenital disorder of bone resorption. It is caused by failure of osteoclasts to reabsorb immature bone. Severe infantile or malignant osteopetrosis present at birth or develops within the first few months of life. We are reporting here a case of two siblings with malignant osteopetrosis. Prominent clinical features included marked pallor, noisy respiration, progressive blindness and developmental delay.
Infantile; Malignant; Osteopetrosis
To compare Reading Center (RC) cup-to-disc ratio (CDR) assessment from stereoscopic photographs with clinician estimation in a uveitis clinical trial.
Clinical estimation of CDR was performed by ophthalmologists via dilated biomicroscopy. Photographic evaluation was performed at an independent RC by masked, certified evaluators. Quality control was performed by repeat grading of 77 randomly selected images.
Among 481 eyes with uveitis, 353 eyes had clinical and photographic grades for CDR. Agreement between clinical and RC grading was fair, with exact agreement in 29%. Agreement within 0.1 and 0.2 CDR were 70% and 93%, respectively (wkappa=0.34). Inter-grader reproducibility at the RC was better (wkappa=0.59, ICC 0.74).
Morphologic assessment of cup to disc ratio is an important outcome and safety measure for determining glaucomatous damage in clinical trials. Masked RC measurements are more likely to be accurate than biomicroscopic grading in identifying meaningful anatomical change associated with glaucoma.
clinical trial; glaucoma; optic nerve head; uveitis
Skin thickness of type-2 diabetic insulin naïve adult patients.
We have limited data on skin and subcutaneous tissue thickness of Indian type-2 diabetic population. Objective of this study was to assess skin and subcutaneous tissue thickness in insulin naïve type-2 diabetic patients as this information may be useful for insulin injection technique.
To assess the skin and subcutaneous tissue thickness at insulin injection sites in insulin naïve, type-2 diabetic adult population across different body mass index (BMI).
Settings and Design:
Observational study carried out at our institute.
Materials and Methods:
One hundred and one insulin naïve type-2 diabetic subjects underwent skin thickness measurement using ultrasound at insulin administration sites. Skin and subcutaneous tissue thickness were measured and prints taken. Though, the sample size to be taken for the study was not calculated, the results obtained clearly show that the power of the study was 80%.
At arm and thigh, the mean skin thickness was more in males as compared to females in the BMI range <23 kg/m2 (P < 0.05). At abdomen, skin thickness was more in males in the BMI range 19-23 kg/m2 (P < 0.05). Across all the BMIs, mean skin plus subcutaneous thickness at arm was more in females (P < 0.05) except for BMI >25 kg/m2 where thickness in males was comparable. At thigh, the skin plus subcutaneous tissue thickness was more in females (P < 0.05), across all BMI ranges. At abdomen, thickness was more in females for the BMI ranges 17-19 kg/m2 and 23-25 kg/m2, while it was comparable across all other BMI ranges (P > 0.05).
Skin and subcutaneous tissue thickness can be estimated by BMI. In general it is higher in females.
Insulin naïve diabetic adult population; skin thickness; ultrasonographic evaluation of skin thickness
The identification of a new class of potent and selective ROCK-II inhibitors is presented. Compound 5 (SR-3677) had an IC50 of ~3 nM in enzyme and cell based assays and had an off-target hit rate of 1.4% against 353 kinases, and inhibited only 3 out of 70 nonkinase enzymes and receptors. Pharmacology studies showed that 5 was efficacious in both, increasing ex vivo aqueous humor outflow in porcine eyes and inhibiting myosin light chain phosphorylation.
India, with one of the largest and most diverse populations of people living with diabetes, experiences significant barriers in successful diabetes care. Limitations in appropriate and timely use of insulin impede the achievement of good glycemic control. The current article aims to identify solutions to barriers in the effective use of insulin therapy viz. its efficacy and safety, impact on convenience and life-style and lack of awareness and education. Therapeutic modalities, which avoid placing an undue burden on patients’ life-style, must be built. These should incorporate patient-centric paradigms of diabetes care, team-based approach for life-style modification and monitoring of patients’ adherence to therapy. To address the issues in efficacy and safety, long-acting, flat profile basal insulin, which mimics physiological insulin and show fewer hypoglycemic events is needed. In addition, therapy must be linked to monitoring of blood glucose to enable effective use of insulin therapy. In conjunction, wide-ranging efforts must be made to remove negative perception of insulin therapy in the community. Patient- and physician – targeted programs to enhance awareness in various aspects of diabetes care must be initiated across all levels of health-care ensuring uniformity of information. To successfully address the challenges in facing diabetes care, partnerships between various stakeholders in the care process must be explored.
Delivery of health-care; diabetes mellitus; health-care disparities; insulin; life-style; medication adherence; patient compliance; patient-centered care; physician-patient relations; safety
Background & objectives:
The resurgence of chikungunya virus (CHIKV) in the Indian Ocean Islands and India has drawn worldwide attention due to its explosive nature, high morbidity and complex clinico-pathological manifestations. The early confirmatory diagnosis of CHIKV is essential for management as well as control of unprecedented epidemics. The present study describes the development and evaluation of a highly sensitive and specific E1 structural gene specific biotinylated DNA probe for detection of chikungunya virus in clinical samples using a dot blot format.
The complementary DNA (cDNA) of CHIKV was spotted on to nylon membrane. The membrane was subjected to prehybridization and hybridization and developed using a colour development solution containing DAB chromogen.
The CHIKV E1 specific DNA probe was highly sensitive detecting picogram levels of target nucleic acid. The comparative evaluation with SYBR Green I based real-time RT-PCR revealed 99 per cent accordance with a sensitivity and specificity of 99 and 98 per cent, respectively. The specificity of this assay was further confirmed through cross-reaction studies with confirmed dengue and Japanese encephalitis (JE) patient serum samples along with infected culture supernatant of Ross River and Saint Louis encephalitis and plasmid DNA of O’Nyong Nyong, Semlinki forest and Sindbis viruses.
Interpretation & conclusion:
The DNA probe reported in this study may be useful for specific, sensitive and confirmatory clinical diagnosis of chikungunya infection in acute phase human patient serum and CSF samples. This assay can also be used in the laboratory for quantification of viral antigen in cell culture supernatant for research purpose.
Biotinylated DNA probe; chikungunya; clinical diagnosis; E1 structural gene
Measuring the three-dimensional motion of muscular tissues, e.g., the heart or the tongue, using magnetic resonance (MR) tagging is typically carried out by interpolating the two-dimensional motion information measured on orthogonal stacks of images. The incompressibility of muscle tissue is an important constraint on the reconstructed motion field and can significantly help to counter the sparsity and incompleteness of the available motion information. Previous methods utilizing this fact produced incompressible motions with limited accuracy. In this paper, we present an incompressible deformation estimation algorithm (IDEA) that reconstructs a dense representation of the three-dimensional displacement field from tagged MR images and the estimated motion field is incompressible to high precision. At each imaged time frame, the tagged images are first processed to determine components of the displacement vector at each pixel relative to the reference time. IDEA then applies a smoothing, divergence-free, vector spline to interpolate velocity fields at intermediate discrete times such that the collection of velocity fields integrate over time to match the observed displacement components. Through this process, IDEA yields a dense estimate of a three-dimensional displacement field that matches our observations and also corresponds to an incompressible motion. The method was validated with both numerical simulation and in vivo human experiments on the heart and the tongue.
Incompressible motion; tagged MRI; divergence-free vector spline; HARP
Onion (Allium cepa), a very commonly used vegetable, ranks third in the world production of major vegetables. Apart from imparting a delicious taste and flavour due to its pungency in many culinary preparations, it serves several medicinal purposes also. Processing and preservation of onion by suitable means is a major thrust area since a long time. The various kinds of treatments followed for dehydration of onion such as convective air drying, solar drying, fluidized bed drying, vacuum microwave drying, infrared drying and osmotic drying are reviewed here. These techniques are mainly used for preservation and value addition of onion. Several researchers have tried for decades to model the drying kinetics and quality parameters, which are also compiled here briefly.
Onion; Dehydration; Nutritional value; Mathematical modeling
Correct occlusal plane orientation is a prerequisite in Prosthodontic reconstructive treatment therapy as it helps in achieving esthetics and phonetics anteriorly and forms a milling surface posteriorly where tongue and buccinator muscle position the food bolus during mastication. Activity of Muscles during clenching will be least, when the occlusal plane is made parallel to plane of lost natural teeth. Conventionally the ala-tragus line (Camper’s plane) is used as a guide for assessment of the occlusal plane and fox plane is used to check the parallelism between the two. Occlusal plane orientor is a new device developed to mark occlusal plane parallel to ala-tragus line on maxillary occlusion rim, thereby saving clinician’s time and increasing efficiency. Additionally, it is a useful tool for occlusal plane orientation in patients with facial deformity, absence of an eye or an ear.
Occlusal plane orientor; Occlusal plane; Occlusion rim
Introduction: Dengue is an acute viral infection which presents as uneventful pyrexia to a fatal complication. This infection is increasingly being recognized as the world’s major emerging tropical disease and an important public health problem.
This article highlights the clinical manifestations of Dengue virus infection and the various molecular tests that were used for its laboratory diagnosis.
Methods: Serum samples from 713 suspected cases of Dengue were collected between August and December 2007. The clinical profiles of 123 hospitalized patients were analyzed. Serology, RT- PCR, virus isolation and sequencing were done.
Results: The most common clinical symptoms were fever, thrombocytopenia, rash and elevated liver enzymes. The demonstration of the Dengue RNA in 5.16% samples, the detection of Dengue specific IgM antibodies in 18% samples and the isolation of the DENV-4 and the DENV-3 viruses from the clinical samples confirmed this Dengue outbreak. A co -infection with Chikungunya was observed in 2.06% of the cases. The phylogenetic analysis revealed that the Indian Dengue-4 isolates from this outbreak belonged to the genotype I. This study clearly indicated the sudden dominance of DENV-4 in an Indian Dengue outbreak.
Conclusion: The surveillance of the Dengue viruses needs to be closely monitored for the emergence of newer serotype(s) in hitherto unknown areas.
Dengue fever; Dengue-3; Dengue-4; Chikungunya; Phylogenetic analysis
The limb-body wall complex defects a form of amniotic band syndrome which consists of a polymal formation with a thoracic and /or an abdominal-schisis, eventration of the internal organ and anomalies of the extremities. We are presenting a case of a limb-body wall complex defect with the phenotype of a placenta-abdominal attachment, anomalies of the abdominal wall defect, absence of the right lower limb and genitourinary defects.
Amniotic band syndrome; Limb body wall complex
Background & objectives:
Disability-adjusted life years (DALYs) have been accepted as a useful method to estimate the burden of disease, and can be adapted to determine the number of productive years lost due to the disability. DALY has been reported for many studies but not for leprosy. Hence this study was carried out in three States of India. In view of the fact that in this study, productive working years are used, the term is modified as DAWLY.
A representative random sample of 150 leprosy affected persons, 50 from each States of Uttar Pradesh, West Bengal and Chhattisgarh, was chosen, and data were collected on detailed work-life history, occupation, time when leprosy was discovered, reported and treatment started, break of job/loss of income due to leprosy. The loss of wages and durations were used to compute the life-years lost due to leprosy, and summarized over the average total duration of 42 years of productive work-life from 18 to 60 years. The percentage losses were determined and differences tested for statistical significance.
The overall mean (± SE) disability adjusted working life years was 28.6 (±0.67), a reduction of 13.4 yr from the ideal productive working life period of 42 yr. The youngest patients with disability had a reduction of 41.4 per cent, as compared to the oldest patients. There was a significant increase in loss based on year for those whose disability started earlier (P=0.0024).
Interpretation & conclusions:
On an average, 30 per cent of the leprosy affected person's work life is lost due to disability.
DALY; DAWLY; India; leprosy; working life
Diffusion Kurtosis Imaging (DKI) provides quantifiable information on the non-Gaussian behavior of water diffusion in biological tissue. Changes in water diffusion tensor imaging (DTI) parameters and DKI parameters in several white and grey matter regions were investigated in a mild controlled cortical impact (CCI) injury rat model at both the acute (2 hours) and the sub-acute (7 days) stages following injury. Mixed model ANOVA analysis revealed significant changes in temporal patterns of both DTI and DKI parameters in the cortex, hippocampus, external capsule and corpus callosum. Post-hoc tests indicated acute changes in mean diffusivity (MD) in the bilateral cortex and hippocampus (p < 0.0005) and fractional anisotropy (FA) in ipsilateral cortex (p < 0.0005), hippocampus (p = 0.014), corpus callosum (p = 0.031) and contralateral external capsule (p = 0.011). These changes returned to baseline by the sub-acute stage. However, mean kurtosis (MK) was significantly elevated at the sub-acute stages in all ipsilateral regions and scaled inversely with the distance from the impacted site (cortex and corpus callosum: p < 0.0005; external capsule: p = 0.003; hippocampus: p = 0.011). Further, at the sub-acute stage increased MK was also observed in the contralateral regions compared to baseline (cortex: p = 0.032; hippocampus: p = 0.039) while no change was observed with MD and FA. An increase in mean kurtosis was associated with increased reactive astrogliosis from immunohistochemistry analysis. Our results suggest that DKI is sensitive to microstructural changes associated with reactive astrogliosis which may be missed by standard DTI parameters alone. Monitoring changes in MK allows the investigation of molecular and morphological changes in vivo due to reactive astrogliosis and may complement information available from standard DTI parameters. To date the use of diffusion tensor imaging has been limited to study changes in white matter integrity following traumatic insults. Given the sensitivity of DKI to detect microstructural changes even in the gray matter in vivo, allows the extension of the technique to understand patho-morphological changes in the whole brain following a traumatic insult.
Magnetic Resonance Imaging; diffusion tensor imaging; diffusion kurtosis imaging; traumatic brain injury; astrogliosis; rat brain
Newer management options are needed for leprosy control even at present, as it is predicted that new cases of leprosy will continue to appear for many more years in future. This article detail newer methods of clinical grading of peripheral nerve involvement (thickening, tenderness and nerve pain which are subjective in nature) and the advances made in the use of Ultrasonography and Colour Doppler as an objective imaging tool for nerves in leprosy. It also briefly discusses the newer drugs and alternative regimens as therapeutic management options which hold promise for leprosy in future.
Clinical grading of nerve involvement; leprosy-new management options; newer drugs and regimens; ultrasonography and colour doppler imaging of nerves
Hypertrophic obstructive cardiomyopathy (HOCM) is a challenge to anesthesiologists due to the complex pathophysiology involved and various perioperative complications associated with it. We present a 50-year-old man, a known case of HOCM, who successfully underwent emergency haemostasis, and debridement of the traumatically amputated right upper limb and the contused lacerated wound on the left forearm under bilateral brachial plexus blocks. His co-morbidities included hypertension (in hypertensive crisis) and diabetes mellitus. He was full stomach and also had an anticipated difficult airway. The management included invasive pressure monitoring and labetalol infusion for emergent control of blood pressure. The regional anaesthesia technique required careful consideration to the dosage of local anaesthetics and staggered performance of brachial plexus blocks on each of the upper limbs to avoid local anaesthetic toxicity. Even though bilateral brachial plexus blocks are rarely indicated, it seemed to be the most appropriate anaesthetic technique in our patient. With careful consideration of the local anaesthetic toxicity and meticulous technique, bilateral brachial plexus blocks can be successfully performed in those patients where general anaesthesia is deemed to be associated with higher risk.
Anaesthetic; brachial plexus; cardiomyopathy; hypertension; hypertrophic obstructive; local; malignant; nerve block
Fetus exists in a complex, dynamic, and yet intriguing symbiosis with its mother as far as fuel metabolism is concerned. Though the dependence on maternal fuel is nearly complete to cater for its high requirement, the fetus is capable of some metabolism of its own. The first half of gestation is a period of maternal anabolism and storage whereas the second half results in exponential fetal growth where maternal stores are mobilized. Glucose is the primary substrate for energy production in the fetus though capable of utilizing alternate sources like lactate, ketoacids, amino acids, fatty acids, and glycogen as fuel under special circumstances. Key transporters like glucose transporters (GLUT) are responsible for preferential transfers, which are in turn regulated by complex interaction of maternal and fetal hormones. Amino acids are preferentially utilized for growth and essential fatty acids for development of brain and retina. Insulin, insulin like growth factors, glucagon, catecholamines, and letpin are the hormones implicated in this fascinating process. Hormonal regulation of metabolic substrate utilization and anabolism in the fetus is secondary to the supply of nutrient substrates. The knowledge of fuel homeostasis is crucial for a clinician caring for pregnant women and neonates to manage disorders of metabolism (diabetes), growth (intrauterine growth restriction), and transitional adaptation (hypoglycemia).
Fetus; fuel; glucose; metabolism
Emerging evidence indicates that many aspects of alcohol and drug dependence involve changes in glutamate transmission. A number of studies have reported that drugs of abuse, including alcohol and cocaine, alter glutamate transport. Extracellular glutamate is regulated by a number of glutamate transporters in various brain regions. Of these transporters, glutamate transporter (GLT1) is a key player in the removal of most of the extracellular glutamate. Similar to neurodegenerative disease models, in which there is dysfunction of the glutamatergic excitatory system, the role of GLT1 has been tested in drug dependence models that show dysfunction of glutamate transmission. We and others have recently found that ceftriaxone, an FDA-approved drug known to elevate GLT1 expression, attenuates cue-induced cocaine relapse. Moreover, we recently found that alcohol-preferring rats treated with ceftriaxone showed a significant dose-dependent reduction in alcohol consumption. We also demonstrated that ceftriaxone-induced upregulation of GLT1 expression was associated with increases in glutamate uptake in Huntington’s disease mouse model. Importantly, ceftriaxone is currently in clinical trials for the treatment of amyotrophic lateral sclerosis. This review provides information about the potential therapeutic role of GLT1 for the treatment of alcohol abuse and dependence.
GLT1; EAAT2; glutamate; alcohol dependence; alcohol addiction; cocaine; GLAST; EAAT1; glutamate transporters; alcohol-preferring rats; glutamate uptake; cystine-glutamate exchanger; basal extracellular glutamate; nucleus accumbens; prefrontal cortex
High-force lengthening contractions are associated with muscle damage and pain, and the muscle–tendon junction is commonly cited as the primary area where myofiber damage occurs. We induced injury in the rat tibialis anterior muscle and acquired magnetic resonance imaging (MRI) images postinjury. We also assayed membrane damage and quantified the number of centrally nucleated myofibers throughout the injured muscles. Results suggest that myofiber injury occurs primarily in the middle portion of the muscle, with interstitial edema in the middle and distal portions.
eccentric injury; MRI; sarcolemma
The IMPROVE Control Training program was designed by Indian Academy of Diabetes (IAD), and a non-intervention study was conducted to evaluate the effectiveness of this standardized healthcare professionals (HCPs) training program on achieving treatment goals in patients with diabetes mellitus and its impact on standard of care.
Materials and Methods:
This multi-center, parallel group, open-label, non-randomized, non-intervention study included patients with type 2 diabetes who had an HbA1c >9 at time of diagnosis or an HbA1c >7% even after 6 months of initiation of therapy with anti-diabetic agents (Oral anti diabetic agents (OADs) and/or insulin). The data recorded at baseline included demographic characteristics, medical history, and the treatment regimens.
The study included 20,493 patients with diabetes, of which 13,295 (64.9%) were men. The mean [standard deviation (SD)] duration of diabetes was 6.4 (4.2) years and 6608 (32.2%) reported complications of diabetes. Poor glycemic control [HbA1c = 9.4 (1.3), FPG (mg/dl) = 181.2 (45.7); mean (SD)] was observed. The postprandial glucose was also high [post-breakfast, lunch, and dinner values in mg/dl were 263.6 (68.5), 278.1 (69.6), and 250.2 (63.7), respectively] in these patients. Failure of OADs was the most common reason cited for initiation of insulin. Premixed insulin was rated the regimen of choice for initiating therapy by the physicians (62.2% vs. 34.5% who preferred basal insulin).
The baseline results confirm the poor glycemic control and the delayed initiation and/or inadequacy of treatment in subjects with type 2 diabetes. These results also highlight the need for early and optimal insulin-based therapy.
baseline characteristics; oral anti diabetic agents failure; insulin initiation; IMPROVE
In the title complex, [Cu(C13H11N2O)Cl(C6H8N2)], the CuII atom adopts a distorted tetrahedral geometry being coordinated by the phenolic O atom and the azomethine N atom of the Schiff base ligand N-salicylidene 2-aminopyridine, and by the 2-aminopyridine N atom and a Cl atom. The pyridyl N atom of the Schiff base and the imino N atom of the 4-methyl-pyridine-2-ylimino ligand are not involved in the coordination. There is an intramolecular N—H⋯N hydrogen bond involving the pyridine N atom and the amino group of the 2-aminopyridine ligand. In the crystal, molecules are linked via N—H⋯Cl hydrogen bonds, forming chains propagating along .
A neonate with unilateral complete duplex system with congenital giant megaureter of the upper moiety presenting as abdominal lump is reported. A left upper moiety nephroureterectomy was performed. Such an anomaly with this presentation has not been reported in neonates.
Duplex kidney; hydronephrosis; megaureter
To assess the in-vitro antibacterial activity and anti-inflammatory activity of orally administered different extracts (Hydro-alcoholic, methanolic, ethyl acetate and hexane) of Rauvolfia tetraphylla (R. tetraphylla) root bark in Carrageenan induced acute inflammation in rats.
In-vitro antibacterial activity was evaluated for extracts against four Gram positive and four Gram negative bacteria by using cylinder plate assay. Hydro-alcoholic extract (70% v/v ethanol) at 200, 400 and 800 mg/kg doses and methanolic, ethyl acetate and hexane extracts at doses 100, 200 and 400 mg/kg were tested for anti-inflammatory activity in Carrageenan induced rat paw oedema model and paw thickness was measured every one hour up to 6 hrs.
All extracts of R. tetraphylla root bark showed good zone of inhibition against tested bacterial strains. In Carrageenan induced inflammation model, hydro-alcoholic and methanolic extract of R. tetraphylla root bark at three different doses produced significant (P<0.001) reduction when compared to vehicle treated control group and hexane, ethyl acetate extracts.
In the present study extracts of R. tetraphylla root bark shows good in-vitro antibacterial activity and in-vivo anti-inflammatory activity in rats.
Rauvolfia tetraphylla; Root bark; In-vitro antibacterial activity; Inflammation; Anti-inflammatory activity
The use of magnetic resonance (MR) imaging in conjunction with an endorectal coil is currently the clinical standard for the diagnosis of prostate cancer because of the increased sensitivity and specificity of this approach. However, imaging in this manner provides images and spectra of the prostate in the deformed state because of the insertion of the endorectal coil. Such deformation may lead to uncertainties in the localization of prostate cancer during therapy. We propose a novel 3-D elastic registration procedure that is based on the minimization of a physically motivated strain energy function that requires the identification of similar features (points, curves, or surfaces) in the source and target images. The Gauss–Seidel method was used in the numerical implementation of the registration algorithm. The registration procedure was validated on synthetic digital images, MR images from prostate phantom, and MR images obtained on patients. The registration error, assessed by averaging the displacement of a fiducial landmark in the target to its corresponding point in the registered image, was 0.2 ± 0.1 pixels on synthetic images. On the prostate phantom and patient data, the registration errors were 1.0 ± 0.6 pixels (0.6 ± 0.4 mm) and 1.8 ± 0.7 pixels (1.1 ± 0.4 mm), respectively. Registration also improved image similarity (normalized cross-correlation) from 0.72 ± 0.10 to 0.96 ± 0.03 on patient data. Registration results on digital images, phantom, and prostate data in vivo demonstrate that the registration procedure can be used to significantly improve both the accuracy of localized therapies such as brachytherapy or external beam therapy and can be valuable in the longitudinal follow-up of patients after therapy.
Elastic registration; strain energy minimization; magnetic resonance imaging; prostate; endorectal coil
In the title compound, [Cu(C4H4O4)(C10H8N2)(H2O)]·2H2O, the CuII ion displays a slightly distorted square-pyramidal coordination. The water molecule at the apical position shows a long bond [Cu—O = 2.276 (2) Å]. The basal plane is formed by two N atoms of the 2,2′-bipyridine ligand and two carboxylate O atoms from a malonate group. The five-membered chelate ring is almost planar [maximum deviation = −0.006 (2) Å], while the six-membered chelate ring defined by the malonate ligand adopts a distorted boat conformation. In the crystal, CuII complex molecules and lattice water molecules are connected by O—H⋯O and C—H⋯O hydrogen bonds. The crystal packing is further stabilized by π–π interactions [centroid–centroid distances = 3.563 (2)–3.828 (2) Å].