Pycnodysostosis with special emphasis on dentofacial characteristics

Abstract Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients

Cephalometric evaluation of the effects of the twin block appliance in subjects with class II, division 1 malocclusion amongst different cervical vertebral maturation stages.

Objectives: To evaluate the cephalometric changes in skeletal, dentoalveolar and soft tissue variables induced by Clark’s Twin Block (CTB) in Class II, Division 1 malocclusion patients and to compare these changes in different cervical vertebral maturation stages. Methods: Pre- and post-treatment/observation lateral cephalograms of 53 Class II, Division 1 malocclusion patients and 60 controls were compared to evaluate skeletal, dentoalveolar and soft tissue changes. Skeletal maturity was assessed according to cervical vertebral maturation stages. Pre- and post-treatment/observation mean changes and differences (T2 -T1 ) were compared by means of Wilcoxon sign rank and Mann-Whitney U-tests, respectively. Intergroup comparisons between different cervical stages were performed by means of KruskalWallis test and Mann-Whitney U-test (p ≤ 0.05). Results: When compared with controls, there was a significant reduction in ANB angle (p \u3c 0.001), which was due to a change in SNB angle in CS-2 and CS-3 (p \u3c 0.001), and in SNA (p \u3c 0.001) and SNB (p = 0.016) angles in the CS-4 group. There was significant increase in the GoGn-SN angle in CS-2 (p = 0.007) and CS-4 (p = 0.024), and increase in Co-Gn and Go-Gn amongst all cervical stages (p \u3c 0.05). There was significant decrease in U1-SN and increase in IMPA amongst all cervical stages (p \u3c 0.05). There was significant retraction of the upper lip in CS-3 (p = 0.001), protrusion of the lower lip in CS-2 (p = 0.005), increase in nasolabial angle in CS-4 (p = 0.006) and Z-angle in CS-3 (p = 0.016), reduction in H-angle in CS-2 (p = 0.013) and CS-3 (p = 0.002) groups. When pre- and post-treatment mean differences were compared between different cervical stages, significant differences were found for SNA, SNB and UI-SN angles and overjet.Conclusions: The Twin-Block along with the normal craniofacial growth improves facial esthetics in Class II, Division 1 malocclusion by changes in underlying skeletal and dentoalveolar structures. The favorable mandibular growth occurs during any of the cervical vertebral maturation stages, with more pronounced effect during CS-3 stage

Understanding experiences of mental health help-seeking in Arab populations around the world: a systematic review and narrative synthesis

BACKGROUND: Racial and ethnic disparities in mental health service utilisation and access is well established. Mental illness is common among Arab populations globally, but most individuals display negative attitudes towards mental health and do not seek professional help. The aim of this systematic review was to determine 1) help-seeking behaviours 2) help-seeking attitudes and 3) help-seeking barriers and facilitators, related to mental health services among Arab adults. METHOD: A pre-defined search strategy and eligibility criteria allowed for database searching using terms related to: mental health, Arabs, help-seeking, as well as experiences and behaviours. Seventy-four articles were included and analysed through narrative synthesis. Results were reported using the PRISMA guidelines. The review protocol was registered prospectively on PROSPERO (CRD42022319889). RESULTS: Arabs across the world have negative attitudes towards formal help-seeking and are reluctant to seek help, despite the presence of psychological distress. There is little information on factors that influence help-seeking behaviours and rates of service use. Preference for informal help sources such as family and friends were expressed and considered more acceptable. Low mental health literacy, stigma, gender, age, education, religion, acculturation, and immigrant status were the most common factors influencing help-seeking attitudes. Barriers to help-seeking included stigma, privacy and confidentiality, trust, mental health literacy, language, logistics, and culture related barriers. Increasing societal and family awareness, external support and encouragement, shared culture between the client and therapist, quality of doctor patient relationship, and feelings of connectedness with the host country among refugees were mentioned facilitators. Mixed findings for the role of religion, and family and community, in relation to facilitating or hindering help-seeking were reported. CONCLUSIONS: There is an increased likelihood and preference to seek informal sources of psychological support among Arabs. Contextual and cultural factors impeding help-seeking for Arabs are common across the world. Future research should address actual utilisation rates of services to better understand factors that influence help-seeking behaviours and facilitators to help-seeking. Increasing mental health literacy and developing anti stigma campaigns is necessary. Developing culturally informed interventions should inform future efforts to promote help-seeking among this population

Stridor: a rare complication of magnesium sulfate therapy in a pregnant patient

Magnesium sulfate is frequently used in severe preeclampsia and eclampsia for the prevention and reoccurrence of seizure activity. Their adverse effects of magnesium sulfate are minor but it cause respiratory depression and tetany. We report a case of stridor due to laryngospasm as result of hypocalcemia in a pregnant patient on magnesium sulfate therapy.A 30 year old gravida5 para 4 had severe preeclampsia started on magnesium sulfate therapy and to control her hypertension she was on labetalol and nifidipine. One hour after the lower segment cesarean section, she developed severe laryngospasm and stridor, no upper airway secretion but found to have hypocalcemia. She responded to immediate intravenous calcium chloride with dramatic clinical improvement. Patients on magnesium sulfate can have life threatening hypocalcemia and stridor. The calcium channel blockers may augment the hypocalcaemic effect of magnesium sulfate

Concordance between clinical and pathological response assessment after neo-adjuvant chemotherapy in patients with invasive lobular carcinoma

Background: Neo-adjuvant chemotherapy (NAC) is frequently administered in breast carcinoma patients. The clinical response to NAC guides further treatment. The pathological response is not only an independent prognostic factor, but it also guides further treatment and prognosis.Objectives: The aim of our study was to find the degree of concordance between clinical and pathological response assessments after NAC in Invasive lobular Carcinoma (ILC) cases by using World Health Organization (WHO) criteria and different pathological systems, respectively. We also tried to identify any useful parameter of clinical assessment that could better correlate with pathologic assessment and provide a better estimation of residual tumor.Methods: This retrospective study was conducted on 26 ILC tumors diagnosed in 24 patients who were treated with NAC followed by surgical resection between January 2009 and December 2020. Medical records and microscopy glass slides were reviewed for clinical and pathological response assessments, respectively.Results: The pre-treatment tumor area ranged from 1.8-255 cm2 and the mean±SD was 52.2±66.8 cm2. After NAC, complete clinical response was observed in four (15.3%) cases. The clinically assessed mean tumor area significantly reduced from 52.2±66.8 cm2 to 17.2±22.6 cm2 (p-value2) didn’t differ significantly from the clinically assessed mean tumor area (17.2±22.6 cm2) (p-value=0.114). Pathologically, the majority of the cases showed partial response, and a complete pathological response was achieved in only two (7.7%) cases. The concordance rates between clinical assessment by the WHO method and pathological assessment of the breast using the Sataloff method, Miller-Payne (MP) system, Residual Cancer Burden system, and Chevallier method were 26.7%, 15.8%, 9%, and 3.5%, respectively, with insignificant p-values. Percentage reduction in clinical size and percentage reduction in tumor cellularity differed significantly (p-value=0.038).Conclusion: Clinical response assessment provides a less accurate estimation of residual disease, as it shows poor concordance with pathological assessment using different assessment systems/methods

Amplitude Scintillation Forecasting Using Bagged Trees

Electron density irregularities present within the ionosphere induce significant fluctuations in global navigation satellite system (GNSS) signals. Fluctuations in signal power are referred to as amplitude scintillation and can be monitored through the S4 index. Forecasting the severity of amplitude scintillation based on historical S4 index data is beneficial when real-time data is unavailable. In this work, we study the possibility of using historical data from a single GPS scintillation monitoring receiver to train a machine learning (ML) model to forecast the severity of amplitude scintillation, either weak, moderate, or severe, with respect to temporal and spatial parameters. Six different ML models were evaluated and the bagged trees model was the most accurate among them, achieving a forecasting accuracy of $81\%$ using a balanced dataset, and $97\%$ using an imbalanced dataset.Comment: This paper was presented at IGARSS 2022, Kuala Lumpur, Malaysia. doi: 10.1109/IGARSS46834.2022.988338

Comparison of Outcome of one Versus two Drains Insertion for Seroma Formation Following Modified Radical Mastectomy in Breast Carcinoma

Objective: Comparative outcome of one versus two drains insertion for in the term of seroma formation following modified radical mastectomy in breast carcinoma. Methodology: This Prospective Interventional trial was conducted at Department of General Surgery, Liaquat University Hospital Hyderabad from February 2018 to January 2019.  Females with breast carcinoma admitted for modified radical mastectomy were included. Patients were divided into two groups.  Groups I underwent one drain placement and group II underwent two drains placement. All patients were observed to measure and record the volume of the fluid. Patients were discharged from Hospital in stable condition and after removal of drains, and followed up weekly for one month. Data was recorded on self-made proforma and analyzed by using SPSS-20. Results: Total of 80 patients were selected, 38 in group A and 42 in group B. Mean age of patients of group A was 49.08 ± 9.89 years and group B was 51.40 ± 13.59 years. , Excised Mass weight was lesser in group A as compared to group B. Mean volume of drain discharge was significantly higher in Group B 323.43 ± 158.88 ml, while it was in group A 230.29± 200.98, findings were statistically significant 0.013. Seroma formation was statistically insignificant among both groups as 8(21.1%) in group A and   10(23.8%) in group B, p-value 0.768. Conclusion: One-drain and two-drain insertion are equally effective to reduce the seroma formation after modified radical mastectomy; however, one drain insertion leads to more patient compliance and comfort with probably less morbidity and cost

New roles for the major human 3'-5' exonuclease TREX1 in human disease

Aicardi-Goutières syndrome (AGS), Systemic Lupus Erythematosus (SLE), Familial Chilblain Lupus (FCL) and Retinal Vasculopathy and Cerebral Leukodystrophy (RVCL) {a new term encompassing three independently described conditions with a common etiology—Cerebroretinal Vasculopathy (CRV), Hereditary Vascular Retinopathy (HVR) and Hereditary Endotheliopathy, Retinopathy and Nephropathy (HERNS)}—have previously been regarded as distinct entities. However, recent genetic analysis has demonstrated that each of these diseases maps to chromosome 3p21 and can be caused by mutations in TREX1, the major human 3'–5' exonuclease. In this review, we discuss the putative functions of TREX1 in relationship to the clinical, genetic and functional characteristics of each of these conditions

Performance Enhancement of NEMO based VANET using localization Router (LR) to reduce handoff delays

Vehicular Ad hoc networks (VANETs) combined with vehicle-to-vehicle and vehicle-to-infrastructure communications can be considered as the most suitable technology to enable ITS (Intelligent Transport System) application bestowed upon travellers with mobility, safety and productivity with human comfort. As a delay sensitive ITS application, handoff delays and packet losses are critical parameters for maintaining seamless connectivity in VANET solution. During handoff, when mobile node (vehicle) is acquiring new CoA (care of address), packets directed towards that node are lost; because it’s old identity is no more valid. So in high speed dynamic vehicular environment the number of frequent handoffs would produce delay beyond the normal limit. Therefore, it is very important to resolve the issues of handoff delay and packet losses in VANET environment. As a solution, a domain based RHD-NV (Reducing Handoff Delay in NEMO based VANET) scheme is proposed in this paper. Number of vehicles moving towards the road constructs a domain where network mobility NEMO-BS protocol is applied. A vehicle is selected as MR (master router) and connected to the RSU (road side unit) to the internet and other vehicles in the domain work as LRs (localization router) and communicate through MR. Simulation tests performed in NS3 (network simulator) and MATLAB SIMULINK demonstrate that using LRs (localization router) in the domain, the number of handoffs and handoff delay are significantly reduced