Enablers & challenges of tribal women & health system for implementation of screening of non-communicable diseases & common cancers: A mixed-methods study in Palghar district of Maharashtra, India.

Background & objectives: Non-communicable diseases (NCDs) and cancers of breast, oral cavity and cervix contribute to around 5.87 million (60%) deaths in India. Despite this, there is limited evidence on preparedness of the tribal health system in mitigating these conditions. This mixed-methods study aimed at identifying enablers and challenges using a multistakeholder approach for the screening of NCDs and common cancers in a tribal block of Maharashtra, India. Methods: This study was conducted in a tribal community of Dahanu taluka in Palghar district of Maharashtra. A total of nine focus group discussions (FGDs) among tribal women and accredited social health activists (ASHAs), 13 key informant interviews (KIIs) among auxiliary nurse midwives (ANMs) and community health officers (CHO) and facility surveys of five public health facilities were conducted. The FGDs and KIIs were conducted using guides, recorded digitally, transcribed, analyzed and triangulated to identify emerging themes. Results: The tribal women had limited knowledge about NCDs and common cancers. Paucity of health facilities, out-of-pocket expenditure, misconceptions, belief on traditional healers and inability to prioritize health were identified as major challenges. The ASHAs were recognized as a key connecting link between health system and community while provision of culturally appropriate IEC materials and adequate training were recognized as critical enablers by healthcare providers in implementing screening for NCDs and common cancers. Interpretation & conclusions: The study recommends incorporating socioculturally relevant strategies in the tribal population and strengthening health facilities in terms of infrastructure and training with involvement of ASHAs for successful implementation of the National Programme for Prevention and Control of Cancer, Diabetes, Cardiovascular Diseases and Stroke (NPCDCS) through health and wellness centres.

Study of Impact Damage in PVA-ECC Beam under Low-Velocity Impact Loading Using Piezoceramic Transducers and PVDF Thin-Film Transducers.

Compared to conventional concrete, polyvinyl alcohol fiber reinforced engineering cementitious composite (PVA-ECC) offers high-strength, ductility, formability, and excellent fatigue resistance. However, impact-induced structural damage is a major concern and has not been previously characterized in PVA-ECC structures. We investigate the damage of PVA-ECC beams under low-velocity impact loading. A series of ball-drop impact tests were performed at different drop weights and heights to simulate various impact energies. The impact results of PVA-ECC beams were compared with mortar beams. A combination of polyvinylidene fluoride (PVDF) thin-film sensors and piezoceramic-based smart aggregate were used for impact monitoring, which included impact initiation and crack evolution. Short-time Fourier transform (STFT) of the signal received by PVDF thin-film sensors was performed to identify impact events, while active-sensing approach was utilized to detect impact-induced crack evolution by the attenuation of a propagated guided wave. Wavelet packet-based energy analysis was performed to quantify failure development under repeated impact tests.

Classical Cardiovascular Manifestations of Marfan Syndrome.

Presence of multiple cardiovascular manifestations of the Marfan syndrome in the same patient is not commonly encountered. We present a 49 year-old lady with this syndrome who presented with decompensated heart failure. Evaluation revealed presence of extensive Stanford type A aortic dissection alongwith severe aortic and mitral incompetence. However, the patient declined surgery and was discharged on medical management. At a year's follow-up, she had dyspnea of NYHA class II with persistent cardiovascular findings.

Nanotechnology: Newer Approach in Insulin Therapy.

Insulin is a peptide hormone released by pancreatic beta cells. An autoimmune reaction in diabetes mellitus type 1 causes the beta cells to die, preventing insulin from being produced or released into the bloodstream; that impacts 30 million people globally and is linked to shortened lifespan due to acute and chronic repercussions. Insulin therapy aims to replicate normal pancreatic insulin secretion, which includes low levels of insulin that are always present to support basic metabolism, as well as the two-phase secretion of additional insulin in response to high blood sugar - an initial spike in secreted insulin, followed by an extended period of continued insulin secretion. This is performed by combining various insulin formulations at varying rates and lengths of time. Since the beginning of human insulin use, several advances in insulin formulations have been made to help meet these aims as much as possible, resulting in improved glycaemic control while limiting hypoglycemia. In this review, we looked at devices used by patients with type 1 diabetes, such as insulin pumps, continuous glucose monitors, and, more recently, systems that combine a pump with a monitor for algorithm-driven insulin administration automation. We intend to provide insight into supplementary therapies and nanotechnology employed in insulin therapy as a result of our review.

Correlation of Ora Test and Caries Assessment Spectrum and Treatment Index (CAST) to Evaluate Caries Activity in 5-to-8-Year-Old Children.

Objectives: Dental caries is a common chronic disease amongst children and are typically evaluated using the DMFT/deft index (decayed, missing, and filled teeth for permanent dentition/decayed, extracted, and filled teeth for primary dentition). To address the limitations associated with these indices, alternative assessment tools such as the Caries Assessment Spectrum and Treatment (CAST) index and Ora test have been developed. These methods aim to estimate caries activity within the oral cavity more accurately. The objective of our study was to evaluate and correlate caries activity in 5-to-8-year-old children using Ora test and CAST index. Materials and Methods: Thirty schoolchildren between the ages of 5 and 8 years were selected and allocated into two groups (n=15) with DMFT/deft scores of <5 (group A) and >5 (group B). Two separate blinded examiners administered the assessments by first determining CAST scoring, which was followed by Ora test. Statistical analysis was performed using Pearson correlation test and significance was set at P≤0.05. Results: The mean time for color change of Ora test, was 118.53±23.28 minutes in group A and 53.33±15.07 minutes in group B. CAST severity scores were 3.67±2.08 and 15.7±9.70 for groups A and B, respectively. Time taken for color change in Ora test and CAST scores showed a significant negative linear relationship (P=0.039). Conclusion: Based on the negative correlation between CAST scores and Ora test, it may be postulated that microbial activity is directly related to caries activity in 5-to-8-year-old children.

Comparison of pain perception between computer-controlled local anesthetic delivery and the conventional syringe for inferior alveolar nerve block in children.

BACKGROUND: Local anesthesia (LA) is commonly used for pain control in clinical dental practice. However, it is often perceived as the most painful part of the treatment and the factor leading to the avoidance of dental care. Hence, research on better means of pain management is being conducted. OBJECTIVES: The aim of the study was to evaluate and compare pain perception using the No Pain III™ computer-controlled local anesthesia delivery (CCLAD) system and the conventional syringe, for inferior alveolar nerve block (IANB) in children. MATERIAL AND METHODS: Thirty children aged 6-12 years were included in the study. Children were randomly allocated into 2 groups by the flip of a coin. Group A received LA by conventional syringe and group B received LA by No Pain III™, on the contralateral side. Physiological parameters including blood pressure (BP), heart rate (HR) and respiratory rate (RR) were assessed at baseline, during the deposition and after the deposition of LA. A subjective evaluation of pain perception was assessed using the Wong-Baker FACES Pain Rating Scale (WBS). The measured values were subjected to statistical analysis. RESULTS: A statistically significant difference was observed between group A and group B for pain perception using the WBS, systolic BP and RR. CONCLUSIONS: The use of the No Pain III™ CCLAD system resulted in reduced pain perception and better acceptance when compared to the use of the conventional syringe, for IANB in children.

Comparative Evaluation of Microleakage of Bioactive, Ormocer, and Conventional GIC Restorative Materials in Primary Molars: An In Vitro Study Microleakage of Three Restorative Materials.

This in vitro study aimed to evaluate and compare the microleakage of bioactive, ormocer, and conventional glass ionomer cement (GIC) restorative materials in primary molars. In this study, class V cavities were prepared on the buccal surface of 75 noncarious extracted primary molars. The teeth were then restored as per the groups assigned. Group A, group B, and group C used bioactive restorative materials, ormocer restorative materials, and conventional GIC restorative materials for restorations, respectively. The teeth were then thermocycled and subjected to microleakage analysis via dye penetration. The microleakage scores were compared for differences using the Kruskal-Wallis test. This was followed by multiple pairwise comparisons using the Dunn test. All testing was carried out using a 'p' value of <0.05. The percentage of samples showing microleakage score 0 depicting no dye penetration was highest for group A (56%) followed by group C (44%) and group B (12%). Statistical analysis revealed highest microleakage with group B, which was statistically significant (p < 0.05). Microleakage was evident in all the materials tested. The lowest microleakage was seen with bioactive restorative material.

Teledentistry: A Boon Amidst COVID-19 Lockdown-A Narrative Review.

The recent spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its associated coronavirus disease (COVID-19) has caused widespread public health concerns. Despite huge efforts to contain the disease spread, it is still on the rise because of the community spread pattern of this infection. In order to prevent the community spread, a nationwide lockdown was implemented, due to which many restrictions were imposed on movements of citizens within the country. Since the dental professionals were at the forefront of acquiring the infection, the majority of the dental clinics were shut for routine dental procedures. Only emergency treatment was provided to the patients. However, due to restrictions in movement, it was difficult for the patients to visit the clinics for routine check-ups. This was overcome by the advancements in technology which has a major impact on medicine. Due to increased usage of smartphones and related software applications, the clinical data exchange was facilitated between patients and clinicians which has been termed as "teledentistry." Teledentistry is a combination of telecommunications and dentistry, involving the exchange of clinical information and images for dental consultation and treatment planning. This technology served as a boon for the dentists to manage dental emergencies during the lockdown period. This narrative review discusses teledentistry and its applications in general and specialty dental practice amidst the COVID-19 lockdown.

Knowledge and awareness of school teachers regarding emergency management of dental trauma in school children of Navi Mumbai.

CONTEXT: Dental trauma is a serious oral health problem that can impair function and aesthetics, which affects the overall quality of life in children. AIMS: The aim of the present study was to identify the level of knowledge and awareness of school teachers regarding emergency management of dental trauma in school children of Navi Mumbai. The second objective was to correlate it with demographic characteristics and evaluate the need for oral health education programs for teachers. SETTINGS AND DESIGN: The present study is a cross-sectional survey and involved the completion of a self-administered anonymous questionnaire by teachers. MATERIALS AND METHODS: A total of 559 school teachers of primary and secondary sections completed the questionnaire comprising 19 questions. These included demographic data, knowledge regarding teeth most frequently affected by trauma, immediate action to be taken, awareness about storage media and the use of mouth guards. The questionnaire also assessed the willingness of teachers to participate in future training programs on dental trauma management. Data were statistically analysed using Chi-square test for any correlation. RESULTS: Among 559 school teachers who completed the questionnaire, 75.3% were females and 24.7% were males. Merely 6.6% of them agreed to have received prior training in dental trauma management. The majority of teachers (87.5%) were willing to attend training programs on dental trauma management. CONCLUSIONS: The knowledge and awareness of school teachers about dental trauma management and storage media for avulsed teeth is poor. Overall, they have a fair knowledge of dental trauma management in school children.

Comparative Evaluation of Shear Bond Strength of Bioactive Restorative Material, Zirconia Reinforced Glass Ionomer Cement and Conventional Glass Ionomer Cement to the Dentinal Surface of Primary Molars: an in vitro Study.

STATEMENT OF THE PROBLEM: The success of dental restorations depends mainly on its ability to bond to dental structures and resist the multitude of forces acting on it within the oral cavity. PURPOSE: Therefore, the aim of this study was to evaluate the shear bond strength (SBS) of three different glass ionomer based restorative materials. MATERIALS AND METHOD: In this in vitro analytical study, 30 intact primary molars were sectioned buccolingually to obtain 60 sections. These sections were embedded in auto polymerizing acrylic resin and polished to obtain a flat dentin surface. Restoration cylinders were built on the dentin surface with the help of a Teflon template called bonding jig. Each group (n= 20) was restored as group A with conventional glass ionomer cement (GIC) (GC Fuji Gold Label Type 9), group B with Bioactive restorative material (ACTIVATM KIDS BioACTIVE Restorative material), and group C with Zirconia reinforced glass ionomer cement (Zirconomer). Following restoration, SBS testing was performed using Universal Testing Machine. The data obtained were statistically analyzed using One way ANOVA test and post hoc Tukey test (p= 0.05). RESULTS: The SBS values were significantly greater in the ACTIVA KIDS group as compared to the other two groups (p< 0.05). There was no significant difference in the SBS values between group B and group C (p> 0.05). CONCLUSION: The SBS of the ACTIVA KIDS to primary teeth dentin was the highest as compared to Zirconomer and conventional GIC. Therefore ACTIVA KIDS may protect primary teeth against recurrent caries and failure of the restoration.

Comparative evaluation of anesthetic efficacy of 4% articaine infiltration versus 2% lignocaine inferior alveolar nerve block for extraction of primary mandibular molars: A prospective, split-mouth, randomized controlled trial.

CONTEXT: Control of pain during dental treatment is an essential aspect of pediatric dentistry. AIMS: This study was conducted to evaluate and compare the anesthetic efficacy of 4% articaine buccal infiltration with 2% lignocaine inferior alveolar nerve block (IANB) for primary mandibular molar extractions. SETTINGS AND DESIGN: The study was a prospective, split-mouth, randomized controlled trial. METHODS: Bilateral symmetrical carious primary mandibular molar (n = 92) extractions in 46 healthy children aged 5-10 years were included in this randomized controlled trial. Extraction was performed on one side using 4% of articaine buccal infiltration and on the contralateral side using 2% lignocaine IANB in two subsequent appointments. Pain and behavior were assessed at baseline, during injection and extraction using Wong-Baker Faces Pain Rating Scale, Modified Behavior Pain Scale (MBPS), and Frankl Behavior Rating Scale. STATISTICAL ANALYSIS USED: Values thus obtained were statistically analyzed by one-way analysis of variance test and compared using independent samples test. RESULTS: According to MBPS, the mean value of pain experienced in the form of cry during injection was reported to be more for 2% lignocaine IANB (1.76) as compared to 4% articaine buccal infiltration (1.30), which was statistically significant (P = 0.024). Comparison of behavior depicted showed no statistically significant difference between the groups. CONCLUSION: Buccal infiltration with 4% articaine can be utilized as an effective alternative to 2% lignocaine IANB for primary mandibular molar extractions.

Effectiveness of Virtual Reality Eyeglasses as a Distraction Aid to Reduce Anxiety among 6-10-year-old Children Undergoing Dental Extraction Procedure.

INTRODUCTION: Distraction is commonly used nonpharmacologic pain management technique by pedodontists to manage pain and anxiety. There are some new techniques which uses audiovideo stimulation and distract the patient by exposing him or her to three-dimensional videos. These techniques are referred to as virtual reality audiovisual systems. The aim is to evaluate the effectiveness of virtual reality eyeglasses as a distraction aid to reduce anxiety of children undergoing extraction procedure. OBJECTIVE: The aim of this study is to evaluate the effectiveness of virtual reality eyeglasses as a distraction aid to reduce anxiety of children undergoing dental extraction procedure. MATERIALS AND METHODS: Thirty children of age 6-10 years (n = 60) with bilateral carious primary molars indicated for extraction were randomly selected and divided into two groups of 30 each. The first one was group I (VR group) (n = 30) and group II (control group) (n = 30). Anxiety was measured by using Venham's picture test, pulse rate and oxygen saturation. Anxiety level between group I and group II was assessed using paired "t" test. RESULTS: The mean pulse rate values after extraction procedure in group I were 107.833 ± 1.356 and group II were 108.4 ± 0.927 respectively. The pulse rate values in intergroup comparison were found statistically significant p = 0.03. CONCLUSION: The virtual reality used as a distraction technique improves the physiologic parameters of children aged 6-10 years but does not reduce the patient's self-reported anxiety according to Venham's picture test used. HOW TO CITE THIS ARTICLE: Koticha P, Katge F, et al. Effectiveness of Virtual Reality Eyeglasses as a Distraction Aid to Reduce Anxiety among 6-10-year-old Children Undergoing Dental Extraction Procedure. Int J Clin Pediatr Dent 2019;12(4):297-302.

Validation of "left ventricular early inflow-outflow index": A novel echocardiographic method for quantification of mitral regurgitation in an Indian population with special focus on rheumatic etiology.

BACKGROUND: Quantification of mitral regurgitation (MR) has always required an "integrated approach" as there is no single gold-standard method. We investigated a new Doppler-derived parameter "left ventricular early inflow-outflow index (LVEIO)" for the quantification of MR and its likelihood to predict severe MR in correlation with already established parameters in an Indian population including a large subset of patients with rheumatic etiology. METHODS: A prospective study was performed at a major tertiary care center in western India over a 5-month period. Five hundred patients diagnosed with isolated MR including 260 (52%) patients with rheumatic etiology were included in the study after applying exclusion criteria. We analyzed MR using color flow jet, effective regurgitant orifice area (EROA), and vena contracta (VC) width. LVEIO is a simplification of the regurgitant volume (RV) method, which was calculated as "E velocity divided by LV outflow velocity integrated over the systolic ejection period left ventricular outflow tract velocity time integral" and compared with the established parameters. RESULTS: LVEIO was 4.65 ± 1.45, 6.56 ± 1.52, and 9.91 ± 3.70 among patients diagnosed with mild, moderate, and severe MR, respectively (p < 0.001). Those with LVEIO ≥8 were the most likely to have severe MR (positive likelihood ratio: 10.42). LVEIO had specificity of 93.25% for diagnosis of severe MR with positive predictive value of 86.36%. There was positive correlation observed between LVEIO and VC width (r = 0.591), RV (r = 0.410), and EROA (r = 0.778) (all p < 0.001) in the Pearson correlation test. The specificity of LVEIO remained consistent in diagnosing severe MR in patients with rheumatic etiology. CONCLUSION: LVEIO is a simple yet specific Doppler echocardiographic parameter for estimation of severity of MR including that of rheumatic etiology.

Comparative Analysis of 2 Calcium Silicate-based Cements (Biodentine and Mineral Trioxide Aggregate) as Direct Pulp-capping Agent in Young Permanent Molars: A Split Mouth Study.

INTRODUCTION: The purpose of this study was to compare Biodentine and mineral trioxide aggregate (MTA) for direct pulp capping in young permanent molars by clinical and radiographic evaluation in 7- to 9-year-old children. METHODS: In 50 patients, 29 patients with bilateral asymptomatic first permanent molars with carious involvement were selected. According to split mouth design, these patients were then divided into 2 groups, Biodentine group (right side) and MTA group (left side). The pulp-capping procedure was performed by using Biodentine and MTA in 58 asymptomatic bilateral permanent molars with pulp exposure. At each recall (baseline, 6 and 12 months), treatment outcome was assessed clinically through pulpal sensitivity tests as well as radiographically to evaluate dentin bridge formation. RESULTS: The study reported 100% success rate with both Biodentine and MTA at baseline and 6- and 12-month follow-up on the basis of clinical and radiographic parameters. These findings were statistically non-significant (P < .05) between both groups (Biodentine and MTA). Radiographically, dentin bridge formation was not evident with both groups at baseline, but it was evident after 6- and 12-month follow-up. These findings were statistically non-significant (P < .05) in both Biodentine and MTA groups. CONCLUSIONS: This study reported 100% success rate with both MTA and Biodentine when used as direct pulp-capping agent in first permanent molars in 7- to 9-year-old children. The major limitations of the study were smaller sample size and short follow-up period.

Appropriateness of elective percutaneous coronary intervention and impact of government health insurance scheme - A tertiary centre experience from Western India.

BACKGROUND: There is a dearth of data regarding the appropriateness of elective percutaneous coronary intervention (PCI) in a limited-resource country such as India. In an attempt to rationalise the use of PCI, Appropriate Use Criteria (AUC) were developed for cardiovascular care in the USA. In the Indian context, considering the high prevalence of coronary artery disease, the dramatic rise in the number of revascularization procedures and an increasing role of government/private reimbursements, application of AUC could potentially guide policy to optimize the utilization of resources and the benefit-risk ratio for individual patients. OBJECTIVES: The study sought to determine the overall and year-wise trends in the appropriateness of elective PCI using the AUC and also understand the impact of the government health insurance scheme (GHIS). MATERIAL AND METHODS: The inpatient records of all patients undergoing elective PCI, at a single large tertiary care centre in Western India, from January 2009 to December 2014 were retrospectively analysed (n=972, 759 males, 213 females) by a neutral observer. The AUC scores and subsequent ranking were calculated using the dedicated web-based software and each PCIwas ranked as either 'appropriate', 'uncertain' or 'inappropriate'. Elective PCI performed within a month after the index acute coronary syndrome (ACS) was considered as 'ACS' while applying the AUC. All other indications were considered as 'non-ACS'. Nearly 95% of elective PCI performed after July 2012 were covered under theGHIS and therefore the period January 2009-June 2012 was compared with the July 2012- December 2014 to assess the impact of this scheme. RESULTS: A total of 894 elective PCI (379 and 515 PCI in the ACS setting and non-ACS setting respectively) performed on 857 patients were analysed. The elective PCI performed in the pre-GHIS and GHIS period were 458 and 436 respectively. As per AUC, 352 (39.6 ± 4.4 %) of the overall elective PCI were ranked as 'appropriate', while 487 (55.3 ± 4.1 %) cases as 'uncertain' and 55 (5.1 ± 0.6 %) cases as 'inappropriate'. An overall year-wise temporal trend in the proportion of cases in any of the AUC rankings did not show any significant trends(p > 0.05). However, 80.4 ± 7.3 % of elective PCI in the ACS setting were categorised as 'appropriate' and 82.6 ± 6.9 % of elective PCI in non-ACS setting were ranked as 'uncertain'. With state-wide implementation of the GHIS, the total number of elective PCI increased by 50% (436 in the 3½ year pre-GHIS study period as against 458 in the 2½ year GHIS study period). The introduction of GHIS led to a marginal increase (p > 0.05) in the average annual number of elective PCI in non-ACS setting as opposed to a 120% rise in the number of elective PCI done in the ACS setting (p < 0.001) and the delay in performing PCI after coronary angiogram reduced from 55.8 ± 43.6 days to 33 ± 22.9 days (p < 0.01). Also, the ratio of men: women undergoing elective PCI rationalised from 5.4:1 to 2.7:1 (p < 0.001). With the introduction of the GHIS, the share of 'inappropriate' elective PCI in the ACS setting increased from 1.34 % to 4.81 % (p =0.065). However, there was also a fall in 'appropriate' elective PCI in the non-ACS setting from 15.0 ± 3.2% to 7 ± 1.6% (p < 0.001). CONCLUSION: On applying the 2012 updated AUC, about 5 % of overall elective PCI were deemed as 'inappropriate'. About four in every five elective PCI in the non-ACS setting were of 'uncertain' appropriateness. The implementation of the GHIS not only significantly reduced the gender bias and delay in seeking interventional coronary care but also led to a significant rise in the proportion of PCI performed in the ACS setting. However, there was also a rise in 'inappropriate' PCI in the ACS setting and a significant fall in 'appropriate' PCI in the non-ACS setting after introduction of the GHIS..

Sealing ability of mineral trioxide aggregate Plus™ and Biodentine™ for repair of furcal perforation in primary molars: An in vitro study.

BACKGROUND: One of the unfavorable outcomes of endodontic treatment in primary molars is furcal perforation. During treatment, bacterial infection at the site of perforation should be prevented for better prognosis. AIM: This study aims to compare sealing ability of mineral trioxide aggregate (MTA) Plus™ and Biodentine™ for the repair of furcal perforation in primary molars using spectrophotometry. MATERIALS AND METHODS: Access opening was done for all ninety extracted teeth. Perforation was made in furcation area in all the teeth. The sample size consisted of ninety extracted teeth. They were divided into four groups, Group 1 (n = 30) in which perforations were repaired with MTA Plus™, Group 2 (n = 30) in which perforations were repaired with Biodentine™. The other two groups were considered as control groups, Group 3 (n = 15) in which perforations were left unsealed (positive control) and Group 4 (n = 15) without perforations (negative control). Dye extraction method was used to compare the sealing ability of MTA Plus™ and Biodentine™. Statistical analysis was done using ANOVA test to compare the mean between the different groups. Intergroup comparison was performed using post hoc Scheffe test. RESULTS: The highest dye absorbance was seen in the positive control group with a mean value of 0.080 ± 0.033. The mean value of MTA Plus™ was 0.031 ± 0.026 and Biodentine™ was 0.024 ± 0.031. CONCLUSION: The mean value of dye absorption of MTA Plus™ was greater than Biodentine™ but it was statistically insignificant.

Brothers with constrictive pericarditis - A novel mutation in a rare disease.

Familial constrictive pericarditis is extremely rare. We report a case of two brothers both suffering constrictive pericarditis along with having multiple painless joint deformities. Genetic workup confirmed the clinical diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome CACP syndrome and also revealed a rare mutation in the causative gene.

Langer-Giedion Syndrome: a Rare Case Report.

Langer-Giedion syndrome is a very uncommon autosomal dominant genetic disorder caused by the deletion of chromosomal material. It is characterized by multiple bony exostosis, short stature, mental retardation, and typical facial features. The characteristic appearance of individuals includes sparse scalp hair, rounded nose, prominent philtral area and thin upper lip. Some cases with this condition have loose skin in childhood which typically resolves with age. Oral and dental manifestations include micrognathia, retrognathia, hypodontia, and malocclusion based on cephalometric analysis. This report presents a case of Langer-Giedion syndrome in a 10-year-old child.

Echocardiographic parameters in clinical responders to surgical pericardiectomy - A single center experience with chronic constrictive pericarditis.

BACKGROUND: Chronic constrictive pericarditis (CCP) is the end result of chronic inflammation of the pericardium. Developing countries continue to face a significant burden of CCP secondary to tuberculous pericarditis. Surgical pericardiectomy offers potential cure. However, there is paucity of echocardiography data in post-pericardiectomy patients vis-a-vis their clinical status. We studied the changes in multiple echocardiographic parameters in these patients before and after pericardiectomy. METHODS: Twenty-three patients (14 men, 9 women) who underwent pericardiectomy for CCP in the last 5 years (from January 2009 to December 2014) were subjected to detailed clinical and echocardiographic evaluation during the study period (between June 2013 and December 2014). Patients with residual symptoms of NYHA class II and below were considered as 'responders'. The data thus obtained were compared to the pre-operative parameters. RESULTS: After pericardiectomy, the incidence of vena caval congestion decreased from 100% to 15% (p<0.001). There was significant reduction in the mean left atrial size from 39.33±10.52mm to 34.45±10.08mm (p<0.001) and also the ratio of left atrium to aortic annulus from 1.93 to 1.69 (p<0.001) among 'responders' to pericardiectomy. Septal bounce was observed to persist in 5 (25%) patients after pericardiectomy. There was significant respiratory variation of 39.23±15.11% in the mitral E velocity before pericardiectomy. After pericardiectomy, this variation reduced to 14.43±7.76% (p<0.001). There was also significant reduction in the respiratory variation in tricuspid E velocities from 31.33±18.81% to 17.35±16.26% (p<0.001). After pericardiectomy, the mean ratio of mitral annular velocities, medial e': lateral e', reduced from 1.08 to 0.87 (p<0.03). The phenomenon of 'annulus reversus' was found to persist in 6 'responders', thereby reflecting a 50% reduction in its incidence after pericardiectomy (p<0.001). The ratio of mitral E to medial e' (E/e') increased from 4.21±1.35 before pericardiectomy to 6.91±2.62 after pericardiectomy (p=0.001). CONCLUSION: Among clinical responders to surgical pericardiectomy, echocardiographic assessment revealed a significant reduction in vena caval congestion, LA size, ratio of LA to aortic annulus, septal bounce, respiratory variation in mitral and tricuspid E velocities, mitral annular medial e' and the phenomenon of annulus reversus. Also, there was a significant rise in minimum tricuspid and mitral E velocities and the E/e' ratio.