The sustainable use of diverse plants accustomed by different ethnic groups in Sibi District, Balochistan, Pakistan.

The present study was conducted to analyze the utilization of medicinal plants (traditional as well as cultivated) and there recipes accustomed by different ethnic groups of Sibi District (SD), Balochistan, Pakistan. The study was carried out between 2018 and 2021 by using semi-structured and open-ended questionnaire.. The randomly selected methods applied for this study were mainly based on household surveys walk through and interview with indigenous communityage 40 to 80, a total of 75 plants, belonging to 63 genera and distributed among 33 plant families were recorded. The dominant Plant families were the Fabaceae (12%) of all studied taxa, followed by the Amaranthaceae (7%), Asteraceae (6%), Cucurbitaceae, Solanaceae, Poaceae (4% each), Rhamnaceae and Zygophyllaceae (3%). Thirty traditional Food Recipes (TFR) and Traditional Medicinal Recipes (TMR) were novel being first time reported from SD., which are utilized by the local communities in their daily routine. These ethnic TFR and TMR have a tremendous role in preservation and sustainable use of traditional food habits and culture. It was also documented that along with cultivated, the wild edible and medicinal plant preparations play a significant role in in the economic potential and primary health care system of the local communities. The study recommends the specific measures, such as small industries, improved export means, tourism and educational activities, to protect the traditional knowledge and biocultural heritage of the region before its erosion.

Quantitative brain morphometry identifies cerebellar, cortical, and subcortical gray and white matter atrophy in late-onset Tay-Sachs disease.

Cerebellar atrophy is a characteristic sign of late-onset Tay-Sachs disease (LOTS). Other structural neuroimaging abnormalities are inconsistently reported. Our study aimed to perform a detailed whole-brain analysis and quantitatively characterize morphometric changes in LOTS patients. Fourteen patients (8 M/6F) with LOTS from three centers were included in this retrospective study. For morphometric brain analyses, we used deformation-based morphometry, voxel-based morphometry, surface-based morphometry, and spatially unbiased cerebellar atlas template. The quantitative whole-brain morphometric analysis confirmed the finding of profound pontocerebellar atrophy with most affected cerebellar lobules V and VI in LOTS patients. Additionally, the atrophy of structures mainly involved in motor control, including bilateral ventral and lateral thalamic nuclei, primary motor and sensory cortex, supplementary motor area, and white matter regions containing corticospinal tract, was present. The atrophy of the right amygdala, hippocampus, and regions of occipital, parietal and temporal white matter was also observed in LOTS patients in contrast with controls (p < 0.05, FWE corrected). Patients with dysarthria and those initially presenting with ataxia had more severe cerebellar atrophy. Our results show predominant impairment of cerebellar regions responsible for speech and hand motor function in LOTS patients. Widespread morphological changes of motor cortical and subcortical regions and tracts in white matter indicate abnormalities in central motor circuits likely coresponsible for impaired speech and motor function.

Comparison of Efficacy of Lidocaine and Articaine as Inferior Alveolar Nerve Blocking Agents in Patients with Symptomatic Irreversible Pulpitis: Randomized Controlled Trial.

Background and Objectives: Lidocaine Hydrochloride has been the standard choice for local anesthesia in dentistry and Articaine's unique structure and growing popularity make it a viable alternative. Due to contradictory results in prior research and a scarcity of trials conducted in the Pakistani population, this study aims to compare the anesthetic efficacy of Lidocaine with Articaine for inferior alveolar nerve blocks in patients with symptomatic irreversible pulpitis. Materials and Methods: This double-blinded, randomized controlled trial included 152 patients who were selected by consecutive non-probability sampling. The participants included patients who presented with symptomatic irreversible pulpitis in mandibular posterior teeth (molars and premolars) and depicted normal apical tissue radiographically. The patients were equally and randomly divided into two groups. The control group received 2% Lidocaine Hydrochloride injections, and the experiment group received 4% Articaine Hydrochloride injections. Participants scored their pain on the HP-VAS both before and after the administration of anesthesia. A value of 54 mm or less on the scale indicated effective anesthesia. The data obtained were analyzed using SPSS. Chi-square test was applied to analyze data for statistical significance. Results: There was no statistically significant difference in the efficacy of the two anesthetic agents. During access cavity preparation, Lidocaine demonstrated a success rate of 93%, whereas Articaine exhibited a slightly higher success rate of 97%. During initial instrumentation, the success rates for Lidocaine and Articaine were 72% and 71%, respectively. This suggests that both Lidocaine and Articaine were effective in achieving anesthesia during the dental procedure in patients with symptomatic irreversible pulpitis, with Articaine showing a slightly better success rate, although the difference was not statistically significant. Conclusions: The anesthetic efficacy of Articaine is similar to that of lidocaine in subjects with symptomatic irreversible pulpitis. Hence, Articaine can serve as an alternative to Lidocaine for local anesthesia administration in dentistry.

Suicidal behaviour in psychodermatology patients: Identifying characteristics and a new model for referral.

It is well known that skin disease is associated with significant psychosocial morbidity, and that patients with skin disease can present with higher rates of suicidality than the general population. Clinicians often report numerous barriers to detecting and managing suicidality in busy outpatient settings. We aimed to establish the degree of suicidality within our psychodermatology patients and establish key characteristics that may serve as additional risk factors for suicidality. We conducted a retrospective review of clinical letters, patient notes, and a clinical database, for all 69 patients that attended our psychodermatology clinic since it was founded. Two practitioners independently recorded patient baseline demographics, presenting dermatological condition, comorbidities, Dermatology Life Quality Index scores and self-reported suicidal behaviour for each patient. From this we calculated how many patients displayed signs of active suicidality, and identified common themes and characteristics within this patient group. We went onto develop a flow diagram to guide professionals when faced with an actively suicidal patient in clinic.

Self-efficacy and Online Learning Readiness of Undergraduate Dental Students in Pakistan During COVID-19 Pandemic.

OBJECTIVE: To investigate the correlation between general self-efficacy and online learning readiness among Pakistani undergraduate dental students. STUDY DESIGN: Cross-sectional study. Place and Duration of the Study: Riphah International University, Islamabad, between September 2020 and March 2021. METHODOLOGY: Using cluster sampling, three dental colleges of Islamabad and Rawalpindi were selected. Electronic questionnaires were sent to 750 students. The questionnaire was comprised of two instruments, General Self-Efficacy Scale (GSES), and Online Learning Readiness Scale (OLRS). GSES had 10-items with response measurement on four-point Likert scale, while the OLRS had 18 items in five different domains measured by five-point Likert scale. Data were analysed using SPSS-23. Correlation between dependent variables was calculated using linear regression analysis. RESULTS: The response rate was 59.3% with a predominant response from female students (82.2%) compared with male students (17.8%). The majority of responses were recorded from third year students (27.9%). The year four students' scores for GSE, computer and internet self-efficacy, and self-directed learning differed significantly from other students. The overall mean GSE score was 29.37+4.57. Regression indicated a low degree of correlation between GSE and the five domains of OLRS with r2 values ranging from 0.12 to 0.32. CONCLUSION: A weak to moderate correlation was found between self-efficacy and the five dimensions of online learning readiness among Pakistani undergraduate dental students as depicted by the r-values. KEY WORDS: Cross-sectional studies, Dental education, Dental students, Dentistry, Efficacy, Online learning, Questionnaire.

Disparities in late and lost: Pediatricians' role in following Pompe disease identified by newborn screening.

BACKGROUND AND OBJECTIVES: Pompe disease (PD) results from a deficiency of lysosomal acid α-glucosidase that leads to glycogen accumulation in lysosomes in multiple tissues. There are two phenotypes: infantile-onset Pompe disease (IOPD) and late-onset Pompe disease (LOPD). The objective was to evaluate the diagnostic and follow-up outcomes of children identified with PD through newborn screening (NBS) in the state of Minnesota over a 4-year period. METHODS: This study is a retrospective analysis of infants born in Minnesota between August 1, 2017, and July 31, 2021, by the Minnesota Department of Health NBS Program for Pompe disease. Newborn screening and clinical diagnostic data are summarized for all newborns with positive newborn screens for Pompe disease. RESULTS: Children with IOPD had abnormal biomarkers necessitating immediate initiation of treatment. Children with LOPD are asymptomatic to date (1.25-4.58 years) with normal biomarkers including creatine kinase, urine glucotetrasaccharides, liver function tests, and echocardiogram. The estimated birth prevalence of PD is 1:15,160. The positive predictive value for PD was 81% with a false positive rate of 1.9 per 10 positive screens. 32% of the children with LOPD were lost to follow up among which 66% were from minority ethnic groups. CONCLUSION: This emphasizes the disparity in access to health care among specific demographics, as well as the importance of a primary care provider's early involvement in educating these families. To accomplish this, and ensure equality in follow-up care, the Minnesota Pompe Disease Consortium has been formed.

Renal mycetoma presenting with features suggesting renal malignancy: A case report.

INTRODUCTION AND IMPORTANCE: Neoplasms of the Kidney are rarely caused by actinomycetoma or other mycetoma species. Actinomycetoma is a neglected tropical disease which is not uncommon in Sudan. Usually, it is presented as skin and subcutaneous tissue lesions or mass and can affect the bone and other soft tissue. Sites of the lesion are found in lower limbs, upper limbs, head and neck and torso. CASE PRESENTATION: A 55-year-old female presented incidental left renal mass on ultrasound examination from the internal medical department. It is presented as a renal mass mimicking renal cell carcinoma with coexistence with another actinomycetoma brain mass. The histopathology report after nephrectomy confirmed the diagnosis. Patients commenced on anti-actinomycetoma treatment after nephrectomy. CLINICAL DISCUSSION: This is the first reported case in our facility which was diagnosed as a renal actinomycetoma. It was treated by surgical excision and received antibacterial treatments. CONCLUSION: This case demonstrates that renal actinomycetoma can occur in an endemic area even without cutaneous or subcutaneous lesions.

High-grade desmoplastic infantile astrocytoma in a 1-year-old child with Down's syndrome: a case report.

BACKGROUND: Down's syndrome is the most common chromosomal abnormality in humans. It has been associated with central nervous system tumors such as primary acute lymphoblastic leukemia and germinomas, but desmoplastic infantile astrocytoma has not yet been reported with Down's syndrome. Desmoplastic infantile astrocytoma is a rare intracranial tumor that mostly occurs in the first 2 years of life. It usually presents as a large, aggressive tumor with both solid and cystic components. Genetically, it has been linked to the BRAF V600E mutation. Despite the rapid growth pattern, it usually has a favorable prognosis after neurosurgical excision. The presence of this extremely rare, genetically linked tumor, and its combination with Down's syndrome, the most common human genetic defect, makes this a very novel clinical presentation. It also raises a very research-worthy question of an undiscovered link between these two genetic disorders. CASE PRESENTATION: In this case, we report a 1-year-old Pakistani origin male child with Down's syndrome, who presented with progressive macrocephaly and developmental regression over the last 2 months. He was unable to sit by himself, and had lost his handgrip bilaterally. Down's Syndrome was diagnosed soon after birth, based on typical facial features and presence of palmar crease, and later confirmed karyotypically for Trisomy 21. Upon presentation, initial blood tests did not show any abnormality. Magnetic resonance imaging of the brain was done, and showed a mixed intensity cystic mass with solid dural component posteriorly in the right parieto temporo occipital region. Craniotomy was performed, and about 85% of the tumor mass was excised. Histological examination and immunochemistry confirmed the suspected radiological diagnosis of desmoplastic infantile astrocytoma. After surgical excision, our patient gradually reacquired his previously regressed developmental milestones. Unfortunately, the remaining mass, which could not be excised due to its attachment to the highly vascular dura mater, showed regrowth on repeat brain magnetic resonance imaging. As his parents did not consent to further surgery, chemotherapy was offered as the next treatment option to prevent tumor regrowth. CONCLUSIONS: This case report highlights the need for more case data and research to understand desmoplastic infantile astrocytoma, and their genetic correlation with Down's syndrome. From a clinical standpoint, since desmoplastic infantile astrocytoma has a good postresection prognosis in a majority of early-diagnosed clinical cases, pediatricians, radiologists, and pathologists should consider desmoplastic infantile astrocytoma in their initial differential diagnosis in Down's syndrome patients with macrocephaly and developmental regression during the first 2 years of life.

Early Neonatal Cardiac Phenotype in Hurler Syndrome: Case Report and Literature Review.

Mucopolysaccharidosis type I (MPS I) is a rare inherited lysosomal disorder caused by deficiency of the α-L-iduronidase enzyme, resulting in the progressive accumulation of glycosaminoglycans (GAGs), which interfere with the normal function of multiple tissues and organs. The clinical phenotype includes characteristic facial features, hepatosplenomegaly, dysostosis multiplex, umbilical and inguinal hernias, progressive cognitive deficits with corresponding hydrocephalus, and neuropathology. Untreated children do not survive into the second decade. The common cardiac phenotype seen in MPS I and other MPS types includes valve thickening and dysfunction, conduction abnormalities, coronary artery disease, and cardiomyopathy-usually seen later in the disease course. A 15-month-old ex-35-weeker who presented with cardiomyopathy and left ventricular failure at the age of three weeks is presented here. Early evaluation and diagnosis with the help of newborn screening (NBS), followed by treatment with enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT), resulted in improvement of his cardiopulmonary status. In MPS I, an early cardiac phenotype is uncommon. Based on the evidence from the literature review for early neonatal cardiac phenotype, we propose that all infants with abnormal newborn screening for MPS I should receive cardiac screening with echocardiogram and NT-proB-type natriuretic peptide (BNP) during the initial evaluation.

British Association of Dermatologists guidelines for the management of adults with delusional infestation 2022.

The overall objective of the guideline is to provide up-to-date, evidence-based recommendations for the management of delusional infestation (DI) in adults. Linked Comment: I. Coulson. Br J Dermatol 2022; 187:457.

Quantifying medical manifestations in Hurler syndrome with the infant physical symptom score: associations with long-term physical and adaptive outcomes.

BACKGROUND: A physical symptom score (PSS) for the mucopolysaccharidosis (MPS) disorders has been developed to quantitate the somatic burden of disease across multiple organ systems. Studies have demonstrated the sensitivity and its relationship to age, IQ and adaptive functioning of the PSS in older children. With the onset of newborn screening, there is an increased need to characterize the somatic symptoms in the earliest stages of life, especially for young children under 36 months of age. Consequently, a new scale, Infant Physical Symptom Score (IPSS), was developed to score physical symptoms in infants and toddlers. OBJECTIVE: Part I. To create a measure to quantify somatic burden in patients with MPS disorders under 36 months of age. The IPSS assess outcomes and changes in somatic disease in individuals with MPS disorders diagnosed very early in life. Part II. To determine the relationship between IPSS and other measures to evaluate its validity and utility, a) we evaluated the relationship between the IPSS and PSS in the same patients with MPS I over time to determine if the two scales are measuring the same concepts, and b) we evaluated the association between IPSS and a functional adaptive measure over time with a focus on the age at first treatment (under 36 months) to determine if the IPSS has predictive value. METHODS: Part I. The Infant Physical Symptom Score (IPSS) for the infant population in MPS disorders was established using data from 39 patients enrolled in the Lysosomal Disease Network longitudinal MPS I study (U54NS065768). All of these patients had Hurler syndrome (MPS IH) and underwent hematopoietic stem cell transplant (HSCT) at the University of Minnesota. Items for the IPSS were selected by reviewing CRFs prepared for the MPS I longitudinal study and examining medical records of these patients prior to HSCT based on the knowledge gained from the development of the PSS. Part II. Of those 39 patients, a subset of 19 were all seen 9 to 12 years post HSCT. Having retrospectively calculated their IPSS prior to HSCT, we categorized them by age at HSCT, and examined their most recent PSS along with Composite and Daily Living Skills scores on the Vineland Adaptive Behavior Scales - Second Edition (VABS-II). RESULTS AND CONCLUSION: The total score on the IPSS collected prior to transplant differed by patient's age at transplant, as expected in this progressive condition. Those transplanted at ≤12 months of age had a mean score of 7.4, which was significantly lower, suggesting less somatic disease burden, compared to those transplanted at >12 to ≤24 months (mean 11.8) and > 24 to ≤36 months (mean 13.6). Higher IPSS reflects more evidence of somatic disease burden and lower IPSS reflects less evidence of disease burden. Nine to 12 years later, the severity level as measured by the PSS was comparable to severity on the IPSS suggesting that the two scales are measuring similar concepts. Retrospectively calculated pre-transplant IPSS were negatively associated with higher VABS-II Composite scores 9-12 years later (p value-0.015) and to a lesser extent Daily Living Skills scores (p value-0.081). We conclude that the IPSS appears to be a useful approach to quantifying the somatic disease burden of MPS IH patients under 36 months of age.

Quantitative brain MRI morphology in severe and attenuated forms of mucopolysaccharidosis type I.

OBJECTIVE: To assess our hypothesis that brain macrostructure is different in individuals with mucopolysaccharidosis type I (MPS I) and healthy controls (HC), we conducted a comprehensive multicenter study using a uniform quantitative magnetic resonance imaging (qMRI) protocol, with analyses that account for the effects of disease phenotype, age, and cognition. METHODS: Brain MRIs in 23 individuals with attenuated (MPS IA) and 38 with severe MPS I (MPS IH), aged 4-25 years, enrolled under the study protocol NCT01870375, were compared to 98 healthy controls. RESULTS: Cortical and subcortical gray matter, white matter, corpus callosum, ventricular and choroid plexus volumes in MPS I significantly differed from HC. Thicker cortex, lower white matter and corpus callosum volumes were already present at the youngest MPS I participants aged 4-5 years. Age-related differences were observed in both MPS I groups, but most markedly in MPS IH, particularly in cortical gray matter metrics. IQ scores were inversely associated with ventricular volume in both MPS I groups and were positively associated with cortical thickness only in MPS IA. CONCLUSIONS: Quantitatively-derived MRI measures distinguished MPS I participants from HC as well as severe from attenuated forms. Age-related neurodevelopmental trajectories in both MPS I forms differed from HC. The extent to which brain structure is altered by disease, potentially spared by treatment, and how it relates to neurocognitive dysfunction needs further exploration.

Intentional re-implantation in a left mandibular second molar with broken file in apical third of mesiobuccal root: A case report.

Intentional re-implantation is done in cases of endodontic failures where conventional treatment options either fail or cannot be carried out due to any reason. It involves extraction of the offending tooth, extra oral apicectomy, followed by reinsertion of the tooth into its anatomical location. The following case report presents a situation where an endodontic instrument was separated in the mesiobuccal root of the left mandibular second molar during instrumentation that could not be retrieved. The decision of intentional re-implantation was made after detailed discussion with the patient, weighing pros and cons of each available treatment option. Fortunately, a favourable outcome was observed over a span of one year and the patient is still on follow-up for evaluation of long-term prognosis.

19th Congress of the European Society for Dermatology and Psychiatry (ESDaP) and 2nd Brain Skin Colloquium Conference (BSC), June 11-13, 2021, London.

The Congress of the European Society for Dermatology and Psychiatry (ESDaP), held in conjunction with the 2nd Brain Skin Colloquium (BSC) Conference, hosted over 60 speakers delivering 47 oral presentations, 41 poster presentations and 5 keynote talks via 2 simultaneous livestream platforms. The 2-day conference, held biennially, was due to be hosted in London, but was converted to a virtual format due to the Covid-19 pandemic. This report presents a synopsis of the conference.

Undergraduate Dental Students' And Instructors' Perceptions About The Quality Of Clinical Feedback.

BACKGROUND: Feedback is an essential component of the clinical skills development of dental students. The clinical training is a complex integration of knowledge, psychomotor skills, behaviour and clinical communication skills under stress for both instructor and student. This study aimed to investigate the perceptions of undergraduate dental students and their instructors on the quality of clinical feedback. METHODS: The current cross-sectional observational study was conducted at the Department of Operative Dentistry and Endodontics, Riphah International University, Islamabad from August to December 2019. A self-administered questionnaire was used to collect responses from final year undergraduate dental students and faculty. The questionnaire comprised of 13 items, subdivided into six categories, targeting the various aspects of clinical feedback. SPSS 23 was utilized to analyse the data. Descriptive statistics were tabulated and data was analysed using the Mann-Whitney test (p<0.05) for differences between the two cohorts. RESULTS: The mean age of the 64 students who responded was 24±0.8 years. Students' male to female ratio was 1:1.56, while for the 18 instructors the ratio was 1:1. Good agreement between the students and instructors was shown for items related to personal dignity and empathy. Moderate agreement was found for items covering the domains of time, understanding, comfort level, professionalism, and self-assessment. The disagreement was observed for the items associated with conflicting feedback, conflict satisfaction, and effective communication. CONCLUSIONS: Incongruousness existed between the dental students and instructors, about the quality of clinical feedback provided to the dental students. Communication between the instructors and learners must be promoted to increase the level of understanding of the feedback given by the clinical instructors.

Biochemical profile and bioactive potential of thirteen wild folk medicinal plants from Balochistan, Pakistan.

The recent focus is on the analysis of biological activities of extracts from thirteen folk medicinal plants from arid and semi-arid zones of Balochistan, Pakistan. Only a small proportion of them have been scientifically analyzed. Therefore the present investigation explores the biochemical and bioactive potential of different plant parts. Superoxide dismutase was detected maximum in Fagonia indica, (184.7±5.17 units/g), ascorbate peroxidase in Tribulus pentandrus (947.5±12.5 units/g), catalase and peroxidase were higher in Peganum harmala (555.0±5.0 and 2597.8±0.4 units/g, respectively). Maximum esterase and α-amylase activity was found in Zygophyllum fabago (14.3±0.44 and 140±18.8 mg/g, respectively). Flavonoid content was high in T. pentandrus (666.1±49 µg/ml). The highest total phenolic content and tannin was revealed in F. olivieri (72125±425 and 37050±1900 µM/g, respectively). The highest value of ascorbic acid was depicted in F. bruguieri (F.b.N) (448±1.5 µg/g). Total soluble proteins and reducing sugars were detected higher in P. harmala (372.3±54 and 5.9±0.1 mg/g, respectively). The maximum total antioxidant capacity was depicted in Tetraena simplex (16.9±0.01 µM/g). The highest value of lycopene and total carotenoids exhibited in T. terrestris (7.44±0.2 and 35.5±0.0 mg/g, respectively). Chlorophyll contents were found maximum in T. pentandrus var. pterophorus (549.1±9.9, 154.3±10, and 703.4±20.2 ug/g, respectively). All taxa exhibited anti-inflammatory activity and anti-diabetic potential. Z. eurypterum seeds exhibited the highest anti-inflammatory potential (96%), along with other taxa indicated (96-76%) activity when compared with the standard drug diclofenac sodium (79%). Seeds of T. pentandrus (85%) exhibited the highest anti-diabetic activity. The other taxa also exhibited inhibitory activity of α-amylase ranging from (85-69%) compared with Metformin (67%) standard drug. Phytochemical screening revealed that selected taxa proved to be the potential source of natural antioxidants and could further be explored for in-vivo studies and utilized in pharmaceutical industries as potent therapeutic agents validating their ethno-pharmacological uses.