Navigating Virtual Learning Landscapes: Perspectives of Health Sciences Students in Saudi Arabia During the COVID-19 Pandemic.

Introduction In December 2019, COVID-19 originated in Wuhan, China, triggering a global pandemic. However, the Saudi Arabian Ministry of Education ensured the safe continuation of teaching and learning activities. Amid the pandemic, health sciences students were exposed to diverse learning opportunities. Methods This study seeks to explore their experiences with online teaching. Conducted as a descriptive cross-sectional study, it involved 397 health sciences students from three universities in the Makkah province who had encountered both traditional and virtual teaching methods. Results Most participants were female (71.1%), predominantly from Jeddah city (76.5%). The highest agreement scores were observed for student comprehension during online sessions (61.1%). A significant proportion (74.4%) found paying attention during online lectures easier than traditional ones. Blackboard emerged as the preferred educational platform for online teaching. Notably, there were no significant variations in students' perceptions of online teaching based on location, gender, or specialisation. Approximately 54.7% of students preferred watching their instructors through a webcam during online lectures. Conclusion Medical educators can leverage these findings to develop standardised teaching protocols and enhance the effectiveness of online education systems. The study underscores the importance of instructors using webcams during online teaching sessions, as it allows students to visually connect with their instructors, potentially improving the learning experience.

Additively manufactured CAD-CAM complete dentures with intraoral scanning and cast digitization: A controlled clinical trial.

PURPOSE: To assess, clinically, patient satisfaction of additively manufactured complete dentures with intraoral scanning and hybrid cast digitization in comparison with conventional complete dentures. MATERIALS AND METHODS: Participants who were edentulous in both arches were recruited and received three types of complete dentures (CDs): conventionally manufactured with conventional impression (CC), additively manufactured with intraoral scanning (AMI), and additively manufactured with cast digitization (AMH). Definitive impressions of the edentulous arches were made with medium viscosity polyvinyl siloxane (Hydrorise Monophase; Zhermack, Italy) for the CC group, intraoral scanning (TRIOS 4; 3Shape, Copenhagen, Denmark) for the AMI group, and laboratory scanning of the definitive casts (Ceramill Map400 AMANNGIRRBACH, Pforzheim, Deutschland) for the AMH group. The trial dentures of the CC group were scanned for occlusion registrations of the AMI and AMH groups and were used to guide the designing process (Exocad 3.0 Galway; Exocad GmbH). The AMI and AMH dentures were additively manufactured with a vat-polymerization 3D printer (Sonic XL 4K; phrozen, Taiwan). Patient satisfaction and clinical outcome were assessed with OHIP EDENT, and 14-factor criteria, respectively. Statistical analyses were performed with paired sample t-test and one-way repeated measure ANOVA for satisfaction, Wilcoxon signed rank test for clinical outcome, and Pearson's r (r) for effect size, with α = 0.05. RESULTS: A total of 20 participants were included. Satisfaction had no statistically significant difference within or among the groups (p < 0.105). Within-group comparison between the two arches showed no statistical significance for the clinical outcome except for a significantly higher maxillary AMI score (p = 0.01, r = -0.40 with medium effect size). For among group's comparison; AMI had a significantly lower score than CC for the maxillary and mandibular arches (p = 0.01, r = -0.40, medium effect size, and p = 0.003, r = -0.47, medium effect size), and significantly lower score than the mandibular AMH (p = 0.03, r = -0.47, medium effect size), with significantly lower quality in teeth arrangement and retention domains for the AMI, and teeth arrangement for the AMH, in comparison with CC. CONCLUSIONS: Patient satisfaction with both types of additively manufactured dentures is comparable to conventional dentures. The comparable overall clinical outcomes between hybrid and conventional dentures indicate that additive manufacturing is an acceptable clinical substitute for the conventional methods. However, additively manufactured dentures made with intraoral scanning have lower clinical quality and retention than hybrid and conventional dentures, particularly for the mandibular arch. Teeth arrangement of both additively manufactured dentures is clinically inferior to the conventional denture.

Virtual patient representation with silicone guide and a 3D scanner accessory for a user-friendly facial scanning workflow: A clinical report of smile design and ceramic veneers.

Digital smile design and ceramic veneers are described with virtual patient representation. The procedure included facial scanning with a 3D scanner accessory (Structure sensor pro; Occipital Inc) mounted on a tablet computer (iPad; Apple Inc) and an innovative chairside silicone guide to replace the intraoral scan body for a straightforward and user-friendly workflow.

Trueness of intraoral scanning of edentulous arches: A comparative clinical study.

PURPOSE: To compare the accuracy of intraoral scanning (IOS) of the edentulous arch with the hybrid protocol of cast digitization (CD), and to investigate the effect of arch type and area on trueness. MATERIALS AND METHODS: Participants that were edentulous in both arches were recruited. Two impression protocols were used; the IOS as the test protocol with an IOS device (TRIOS 4; 3Shape, Denmark), and the CD as the control, including tracing compound (TRACING STICKS; Kemdent, UK) for border molding, polyvinyl siloxane (Hydrorise Monophase; Zhermack, Italy) for impression, and cast digitization with a laboratory scanner (ceramill® map400, AMANNGIRRBACH, Germany). Scanned files were exported to a 3D inspection software (Geomagic Control X; 3D Systems, NC) for trueness analysis. The CD file (reference file) for each participant was split into 2 areas; the dynamic area representing the mobile tissues at the peripheral border, and the static area representing the rest of the arch. Statistical analyses were performed with 1-sample t-test for the difference between CD and IOS protocols, paired sample t-test for the difference between the static and dynamic areas for each arch, and an independent sample t-test for the difference between the maxillary and mandibular arches for each area, with α = 0.05. Effect size was calculated with Cohen's d (d), with 0.2 as small, 0.5 as medium, and 0.8 as large. RESULTS: A total of 21 participants were included. The difference between the IOS and CD protocol was significant for all subset comparisons (p < 0.001, d: 2.5-6.2, large effect size). Dynamic areas had lower trueness in comparison with static areas (p < 0.001, d = 4.57, large effect size for the maxillary arch, p < 0.001, d = 3.96, large effect size for the mandibular arch). Mandibular arch had lower trueness in comparison with the maxillary arch (p < 0.001, d = 1.45, large effect size for the static areas, p = 0.009, d = 0.85, large effect size for the dynamic areas, p < 0.001, d = 1.71, large effect size for all areas). Color difference map showed marked positive deviation in the buccal dynamic areas of both arches, and nonmatching areas with evident overstretching. CONCLUSIONS: While the IOS of edentulous arches could be feasible for attached mucosa, providing a functional shape for the peripheral border remains a challenge, with a thinner and more outward border for the IOS in comparison with the CD protocol. The IOS of the mandibular arch is more difficult and has lower trueness in comparison with the maxillary arch.

Fabrication of poly(maleic acid)-grafted cross-linked chitosan/montmorillonite nanospheres for ultra-high adsorption of anionic acid yellow-17 and cationic brilliant green dyes in single and binary systems.

In this contribution, poly(maleic acid)-grafted cross-linked chitosan/montmorillonite composite nanospheres (PMAL-CTS/MMT) were synthesized via a facile approach for adsorption of organic dyes. The adsorption capacity of PMAL-CTS/MMT towards anionic acid yellow-17 (AY17) and cationic brilliant green (BG) was compared to PMAL-CTS, CTS/MMT, and MMT to emphasize the role of surface functional groups introduced by poly(maleic acid) and montmorillonite. Interestingly, the adsorption efficiency of PMAL-CTS/MMT nanocomposite towards both dyes in the single and binary systems was extremely high due to plenty of functional groups. The affinity of PMAL-CTS/MMT towards cationic and anionic dyes resulted from the feasible modulation of the surface charges as a function of the solution pH. The PMAL-CTS/MMT nanocomposite exhibited a maximum adsorption capacity of 518 and 1910 mg g-1 for AY17 and BG, respectively, which is higher than most of the adsorbents reported in recent literature studies. The proposed mechanism based on the characterization of PMAL-CTS/MMT after the adsorption highlighted that the adsorption is mainly controlled by electrostatic interaction, π - π interactions, and hydrogen bonding. More importantly, the PMAL-CTS/MMT nanocomposite was successfully applied to separate the AY17 and BG dyes from real-life aquatic environments. Collectively, the simple fabrication and superior adsorption performance reveal that PMAL-CTS/MMT has the potential to treat concomitant organic dyes effectively.

Additive Manufacturing of Dental Ceramics: A Systematic Review and Meta-Analysis.

PURPOSE: The purpose of this systematic review and meta-analysis was to evaluate the effect of using additive manufacturing (AM) for dental ceramic fabrication in comparison with subtractive manufacturing (SM), and to evaluate the effect of the type of AM technology on dental ceramic fabrication. MATERIALS AND METHODS: A search was conducted electronically in MEDLINE (via PubMed), EBSCOhost, Scopus, and Cochran Library databases, and also by other methods (table of contents screening, backward and forward citations, and grey literature search) up to February 12, 2022, to identify records evaluating additive manufacturing of ceramics for dental purposes in comparison with subtractive manufacturing. A minimum of 2 review authors conducted tstudy selection, quality assessment, and data extraction. Quality assessment was performed with Joanna Briggs Institute tool, and the quantitative synthesis was performed with the Comprehensive Meta-Analysis program (CMA, Biostat Inc). Hedges's g for effect size was calculated, with 0.2 as small, 0.5 as medium, and 0.8 as large. Heterogeneity was assessed with I2 and prediction interval (PI) statistics. Publication bias was investigated with funnel plots and grey literature search. Certainty of evidence was assessed with the Grading of Recommendations: Assessment, Development, and Evaluation (GRADE) tool. RESULTS: A total of 28 studies were included for the qualitative and quantitative synthesis; 11 in vitro studies on accuracy, 1 in vivo study on color, and 16 in vitro studies on physical and mechanical properties. Meta-analysis showed overall higher accuracy for SM compared with AM, with medium effect size (0.679, CI: 0.173 to 1.185, p = 0.009) and also for marginal (g = 1.05, CI: 0.344 to 1.760, p = 0.004), occlusal (g = 2.24, CI: 0.718 to 3.766, p = 0.004), and total (g = 4.544, CI: -0.234 to 9.323, p = 0.062) with large effect size; whereas AM had higher accuracy than SM with small effect size for the external (g = -0.238, CI: -1.215 to 0.739), p = 0.633), and internal (g = -0.403, CI: -1.273 to 0.467, p = 0.364) surfaces. For technology, self-glazed zirconia protocol had the smallest effect size (g = -0.049, CI: -0.878 to 0.78, p = 0.907), followed by stereolithography (g = 0.305, CI: -0.289 to 0.9, p = 0.314), and digital light processing (g = 1.819, CI: 0.662 to 2.976, p = 0.002) technologies. Flexural strength was higher for ceramics made by SM in comparison to AM with large effect size (g = -2.868, CI: -4.371 to -1.365, p < 0.001). Only 1 study reported on color, favoring ceramics made through combined AM and SM. CONCLUSIONS: Subtractive manufacturing had better overall accuracy, particularly for the marginal and occlusal areas, higher flexural strength, and more favorable hardness, fracture toughness, porosity, fatigue, and volumetric shrinkage; whereas AM had more favorable elastic modulus and wettability. Both methods had favorable biocompatibility. All studies on accuracy and mechanical properties were in vitro, with high heterogeneity and low to very low certainty of evidence. There is a lack of studies on color match and esthetics.

A Patient with a Novel RARS2 Variant Exhibiting Liver Involvement as a New Clinical Feature and Review of the Literature.

Pontocerebellar hypoplasia (PCH) is a heterogeneous neurodevelopmental disorder that is characterized by decreased brainstem and cerebellum volume. Pontocerebellar hypoplasia type 6 (PCH6) is a mitochondrial disease associated with autosomal recessive inheritance that results from mutations in the RARS2 gene. In this case report, we describe a new clinical presentation with a novel RARS2 pathogenic variant. We report here on 2 siblings who presented with neonatal lactic acidosis, microcephaly, growth retardation, persistent seizures, and cholestasis with a previously undefined RARS2 pathogenic variant. In our literature review, we evaluated the clinical features and pathogenic variants of 34 patients reported in 16 publications since the initial identification of RARS2 pathogenic variants in PCH6 in 2007. Both siblings were detected with c.1564G>A (p.Val522Ile), a novel homozygous pathogenic variant of the RARS2 gene. Imaging revealed advanced cerebral atrophy and cerebellar hypoplasia, while the basal ganglia and pons were preserved. At follow-up, the elevations in liver function test results and cholestasis had regressed while the LDH and GGT elevations persisted. Both siblings showed microcephaly on follow-up and started to suffer seizures. Severe developmental delay and nutritional problems were observed, and both died in infancy. RARS2 pathogenic variant is a mitochondrial disease that causes severe mental, motor, and developmental retardation, as well as short life expectancy. Our patients are the first cases with liver involvement in PCH6 and a novel homozygous RARS2 pathogenic variant to be reported in the literature. This additional phenotype can be considered as making a valid contribution to the literature.

Efficacy of dietary inclusion of biologically treated pruning peach trees by-products on growth performance, blood biochemicals and economic efficiency of New Zealand White rabbits.

This work examined the influences of partial replacement of clover hay with 15 or 30% of untreated pruning peach trees by-products (UPPT) or those biologically treated (BTPPT) with effective microorganisms (EM1) on growing rabbit performance. Seventy-five NZW weaned rabbits (6-weeks-old; average body weight 657 g) were assigned randomly into five groups. Five pelleted diets were used, control and other four diets (15 or 30% UPPT or BTPPT were replaced of clover hay). The biological treatments improved the chemical composition of pruning peach trees by-product, in terms of increasing crude protein and decreasing crude fiber as compared to UPPT. Final body weight, body weight gain, feed conversion ratio and performance index values were enhanced when rabbits fed either 15 or 30% BTPPT. Digestibilities of crude protein, crude fiber, nitrogen free extract, total digestible nutrients and digestible energy were increased with rabbits fed diets containing BTPPT. Carcass traits studied, moisture, crude protein (CP), ash, ether extract (EE) of rabbits meat, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) activities, total cholesterol, creatinine and urea were not different between the applied treatments. Net revenue and economic efficiency were increased with the biological treatment. Conclusively, BTPPT could be used up to 30% with positive effects on growth performance, physiological functions and economic efficiency in NZW growing rabbits.

Co-Occurring Atypical Galactosemia and Wilson Disease.

Introduction: Classic galactosemia is a disorder of the galactose metabolism and is inherited as an autosomal recessive disease. It is caused by a complete or severe deficiency of galactose-1-phosphate uridyltransferase (GALT), and in rare cases, atypical galactosemia can manifest at older ages. Wilson disease (WD) is a disorder of the copper metabolism that, like galactosemia, is inherited as an autosomal recessive disease. Hepatic, neurological, or psychiatric symptoms can be seen, independently or in combination, and symptoms vary from family to family. We present here a patient diagnosed with both WD and galactosemia. Case Presentation: A 6-year-old girl was referred to our center with elevated transaminase levels and hepatosplenomegaly. The child, birthweight of 2,200 g, was born to first-degree consanguineous parents after a full-term uneventful pregnancy and was hospitalized in the neonatal period due to indirect hyperbilirubinemia, gastrointestinal bleeding, diarrhea lasting 2 weeks, and elevated liver enzymes. Hepatosplenomegaly was evident at the time of admission, a cataract was detected, and a neuropsychiatric evaluation revealed borderline mental capacity, as well as cognitive and speech retardation. Metabolic investigations revealed no specific findings other than trace positivity of reducing substances in the urine. A liver biopsy revealed copper accumulation in hepatocytes and low ceruloplasmin levels. Although WD was suspected in the patient, this diagnosis did not explain the intellectual disability, behavioral disorder, or cataract findings. A genetic analysis revealed homozygous mutations in the ATP7B and GALT genes. The galactose-1-phosphate uridyltransferase enzyme level was found to be low, and the patient was diagnosed with coexisting WD and galactosemia. Conclusion: Coexistences of rare genetically transmitted diseases can be seen in countries where consanguineous marriages are common (Saudi Arabia, Iran, Pakistan, etc.), as in our country, Turkey.

A Novel PUS1 Mutation in 2 Siblings with MLASA Syndrome: A Review of the Literature.

ABSTRACT: Myopathy, lactic acidosis, and sideroblastic anemia (MLASA) is a rare mitochondrial disorder characterized by MLASA. Variable features of this condition include failure to thrive, and developmental delay or intellectual disability. Additional symptoms consist of cognitive impairment, skeletal and dental abnormalities, delayed motor milestones, cardiomyopathy, dysphagia, and respiratory insufficiency. MLASA has previously been associated with mutations in pseudouridylate synthase 1 (PUS1) and YARS2. PUS1 encodes the nuclear PUS1 enzyme, which is located in both the nucleus and the mitochondria. PUS1 converts uridine into pseudouridine in several cytosolic and mitochondrial transfer RNA positions and increases the efficiency of protein synthesis in both compartments.In the present report, we report on 2 Turkish sisters 4 and 11 of years with an MLASA plus phenotype. Both patients have sideroblastic anemia, lactic acidosis, failure to thrive, developmental delay, and chronic diarrhea; in addition, the older sister has strabismus and skeletal anomalies. The sequencing of the PUS1 gene revealed a novel homozygous p.Glu311* mutation. The phenotype of the older sibling is also unique because of the strabismus and skeletal anomalies, when compared with her sister and other previously reported patients with MLASA. The structural differences in the nuclear versus mitochondrial isoforms of PUS1 and modifier genes may be implicated in the variability of the clinical presentations in MLASA. CONCLUSION: This report adds to the growing number of mutations causing complex clinical manifestations of MLASA including lactic acidosis, sideroblastic anemia, chronic diarrhea, and myopathy.

The Struggle with Rheumatism through Dracunculus vulgaris Schott: In the Light of Ethnobotanical Information.

BACKGROUND: The fruits with the seeds of Dracunculus vulgaris Schott. (Araceae) are used against inflammatory diseases in Turkey. OBJECTIVE: The present study was designed to justify this folkloric usage of the plant. Therefore, the aim of this study was to investigate the anti-inflammatory activity of D. vulgaris. METHODS: Petroleum ether, ethyl acetate and methanol extracts were prepared from the fruits, successively. Carrageenan-, serotonin-, and prostaglandin E2-induced hind paw edema; acetic acid-induced capillary permeability and 12-O-tetradecanoyl-phorbol-13-acetate-induced mouse ear edema models were used to assess the anti-inflammatory activity of the extracts. The analgesic activity was observed by using p-benzoquinone-induced abdominal constriction test. RESULTS: The petroleum ether extract displayed the highest activities in all of the used test models compared with the control group. Therefore, the constituents of this extract were determined by using gas chromatography-mass spectroscopy (GC-MS). Linoleic acid was found to be the major constituent of the petroleum ether extract of D. Vulgaris. CONCLUSION: This study has provided some justification for the folkloric use of the plant.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma.

The aim of this study is to evaluate the clinical and laboratory findings of pediatric patients with non-Hodgkin lymphoma (NHL) who developed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Between 2006 and 2018, the medical records of child patients with NHL who developed SJS and TEN were reviewed retrospectively. SJS/TEN developed in 7 of 70 patients with NHL (10%). The pathologic subgroups of the patients with SJS/TEN were ALK-negative anaplastic large cell lymphoma (n: 3), Burkitt lymphoma (n: 2), lymphoblastic lymphoma (n: 1), and primary mediastinal B-cell lymphoma (n: 1). Five patients had TEN, 1 patient had SJS/TEN, and 1 patient developed only SJS. In 5 patients, both steroids and intravenous immunoglobulin were administered for treatment, and clinical improvement was achieved in 3 of these patients. Only steroid treatment was used for 1 patient, whereas for the other patient, intravenous immunoglobin was preferred. In addition, N-acetylcysteine treatment was administered for these 2 patients. Four patients with acute renal failure died, and it was found that SJS/TEN is observed more frequently in patients with NHL in which intensive treatment protocols with high-dose methotrexate are used more than with other childhood malignant diseases. Early diagnosis and administration of appropriate and supportive treatment approaches may improve the prognosis.

Novel benzo[b]xanthene derivatives: Bismuth(III) triflate-catalyzed one-pot synthesis, characterization, and acetylcholinesterase, glutathione S-transferase, and butyrylcholinesterase inhibitory properties.

In this study, 3,4-dihydro-12-aryl-1H-benzo[b]xanthene-1,6,11-(2H,12H)trione compounds were obtained through one-pot condensation of various substituted aromatic aldehydes, 2-hydroxy-1,4-naphthoquinone, and dimedone in the presence of Bi(OTf)3 as a green and reusable catalyst. The structural characterization of these novel substituted benzo[b]xanthenes was performed by spectroscopic methods, and their inhibitory actions against butyrylcholinesterase (BChE), acetylcholinesterase (AChE), and glutathione S-transferase (GST) were investigated. GST is an enzyme responsible for removing toxic molecules during Phase II reactions in the detoxification mechanism. The AChE and BChE enzymes, which are called cholinesterases, are among the enzymes that occur especially during dementia such as brain damage or Alzheimer's disease. Inhibition effects of the benzo[b]xanthene derivatives on AChE, BChE, and GST were found at the millimolar level. The best inhibitor for GST is compound 4a (31.18 ± 6.13 mM), for AChE, it is compound 4d (28.16 ± 3.46 mM), and for BChE, it is compound 4f (36.24 ± 3.19 mM). Compound 4a inhibited the dimerization of GST subunits, and compounds 4d and 4f directly inhibited the catalytic activity by interacting with the catalytic active site or a related site of the AChE and BChE enzymes, respectively.

Skeletal scintigraphy image enhancement based neutrosophic sets and salp swarm algorithm.

Recently, several schemes are proposed for enhancing the dark regions of the skeletal scintigraphy image. Nevertheless, most of them are flawed by some performance problems. This paper presents an adaptive scheme based on Salp Swarm algorithm (SSA) and a neutrosophic set (NS) under multi-criteria to enhance the dark regions of the skeletal scintigraphy image efficiently. Enhancing the dark regions is first converted into an optimization problem. The SSA algorithm is used to find the best improvement for each image separately, and then the neutrosophic algorithm is used to find similarity score to each image with adaptive weight coefficients obtained by the SSA algorithm. The proposed algorithm is applied to an Egyptian medical dataset collected from Menoufia University Hospital and it is a no-reference image. The experiments are done using 3 different resolutions 512*512, 256*256, and 128*128 and compared with Gamma Correction, the NS algorithm and the local enhance algorithm. The results demonstrate that the proposed algorithm achieves superior performance in almost criteria fitness function, entropy, eumber of edges, nNaturalness image quality Evaluator, sharpness, sharpness index, and contrast-distorted images using contrast enhancement. The results showed the idea of integration between the falsity membership of the neutrosophic set and the Salp swarm algorithm can be used to Skeletal Scintigraphy enhancement. This paper proved that it can depend on falsity membership of the neutrosophic set in the Image Enhancement field.

Extracellular laccase production and its optimization from Arthrospira maxima catalyzed decolorization of synthetic dyes.

In the present study laccase production potential of a photosynthetic, non nitrogen fixing cyanobacteria Arthrospira maxima (SAE-25780) was investigated for their probable use in synthetic dye decolorization which poses environmental pollution problem in aquatic bodies. A. maxima (SAE-25780) showed a constitutive production of laccase which increased up to 80% in the presence of inducer guaiacol. The optimal condition for laccase was 30 °C, 10 mM sucrose as a carbon source, 10 mM sodium nitrate as a nitrogen source, and 2 mM copper as metal activator. The partially purified laccase showed 84% and 49% decolorization potential for the two anthroquinonic dyes-Reactive Blue 4 and Remazol Brilliant Blue R, respectively (RBBR) within 96 h without any mediator. Therefore the laccase extracted from A. maxima (SAE-25780) can be used efficiently in bioremediation of synthetic dyes from paper, pulp and textile industries.

Congenital Hemophilia A Presenting With Hashimoto's Encephalopathy and Myocarditis: The First Reported Case.

Here, we report a case of hemophilia A with myocarditis, encephalopathy, and spontaneous intramedullary hemorrhage. A 14-month-old male infant presented with loss of consciousness, generalized tonic-clonic convulsions, and cardiac failure. The neurological examination was normal. Myocarditis was diagnosed. After administration of fresh frozen plasma, the aPTT did not return to normal. The factor VIII (FVIII) level was 10.2% the normal level, and the patient was diagnosed with hemophilia A. The cerebrospinal fluid (CSF) evaluation was unremarkable, with the exception of elevated CSF protein levels. An electroencephalogram revealed diffuse slowing of background activity. The spinal MRI revealed chronic bleeding in areas of the medulla spinalis. Tests for the antibodies associated with autoimmune encephalitis were negative. Anti-thyroglobulin (THG) (11.8 U/mL [normal, 0 to 4 U/mL]) and anti-thyroid peroxidase (TPO) (53.9 U/mL [normal, 0 to 9 U/mL]) antibodies were present in the serum. The patient received 1 g/kg intravenous immunoglobulin G (IVIG) for 2 days based on a diagnosis of myocarditis and Hashimoto's encephalopathy. After the first month of treatment his clinical findings were negative. He developed an inhibitor to FVIII 1 month after initiation of FVIII replacement therapy. Hemophilia A has various clinical presentations. Autoimmunity may lead to early inhibitor development in patients with hemophilia.

A rare cause of fatal pulmonary alveolar proteinosis: Niemann-Pick disease type C2 and a novel mutation.

Niemann-Pick disease type C (NPC) is a fatal autosomal recessive lipid storage disease associated with impaired trafficking of unesterified cholesterol and glycolipids in lysosomes and late endosomes. This disease is commonly characterized by hepatosplenomegaly and severe progressive neurological dysfunction. There are two defective genes that cause this illness. One of these genes is NPC1 gene which is the cause of illness in 95% of the patients. The other gene is the rare type NPC2 which is the cause of illness in 5% of the patients. Patients with NPC2 usually present with respiratory distress in early infancy, which is rather unusual with NPC1. This article discusses about a patient who died at an early age from pulmonary involvement and who subsequently was found to have a novel homozygous mutation of NPC2 gene.

A case of congenital afibrinogenemia complicated with thromboembolic events that required repeated amputations.

Although congenital afibrinogenemia is a rare autosomal recessive bleeding disorder, it can be more frequently encountered in countries where consanguineous marriages are common. Congenital afibrinogenemia is characterized by the undetectable low level of fibrinogen, which causes hemorrhagic diathesis. Paradoxically, arterial and venous thromboembolic complications can develop in patients with afibrinogenemia, which may cause a diagnostic problem to anyone unfamiliar with its clinical features. We report a case of congenital afibrinogenemia presenting with bilateral ischemic lesions of bilateral foot and ankle that required amputations. The patient was treated with fibrinogen concentrate, low-molecular-weight heparin, aspirin, and nifedipine. In conclusion, arterial and venous thromboembolic complications are rare, but severe complications of afibrinogenemia. The management of thromboembolic complications in patients with afibrinogenemia is a balance game. At one end of the scale, there is a bleeding disorder, and at the other end, there is a thrombosis. This fine adjustment is a job of mastery.

Nerve growth factor enhances cough via a central mechanism of action.

The mechanisms involved in enhanced cough induced by central and inhaled NGF in guinea pigs were investigated. Cough and airway function were assessed by plethysmography following inhaled or intracerebroventricular (i.c.v.) NGF treatment. Expression of TrkA and/or TRPV1 was determined in bronchi and/or brainstem by real-time PCR and immunoblotting. I.c.v. and inhaled NGF enhanced citric acid induced-cough and airway obstruction. Pretreatment (i.c.v.) with antagonists of TrkA (K252a) or TRPV1 (IRTX) significantly reduced both the NGF (i.c.v.) enhanced cough and airway obstruction whereas the NK1 antagonist (FK888) inhibited only cough. The H1 antagonist (cetirizine) did not affect either. Inhaled NGF increased phosphorylation of TrkA receptors in the bronchi but not the brainstem at 0.5h post-treatment. TrkA mRNA was elevated at 0.5h in the bronchi and at 24h in the brainstem while TRPV1 mRNA was elevated from 0.5h to 24h in brainstem and at 24h in the bronchi. Pretreatment (i.c.v.) with IRTX, but not K252a, significantly inhibited the inhaled NGF-enhanced cough. Central NGF administration enhances cough and airway obstruction by mechanisms dependent on central activation of TrkA, TRPV1 and NK1 receptors while inhaled NGF enhances cough via a mechanism dependent on central TRPV1 and not TrkA receptors. These data show that NGF, in addition to its effects on the airways, has an important central mechanism of action in the enhancement of cough. Therefore, therapeutic strategies targeting NGF signaling in both the airways and CNS may be more effective in the management of cough.

Misoprostol for midtrimester termination of pregnancy in women with 1 or more prior cesarean deliveries.

OBJECTIVE: To evaluate the safety and efficacy of vaginal misoprostol for midtrimester termination of pregnancy (TOP) in women with 1 or more prior cesarean deliveries (CDs). METHODS: A retrospective study was conducted with 279 women undergoing TOP with vaginal misoprostol between 14 and 26 weeks of gestation. Of these, 193 had no uterine scars (group 1), 60 had 1 prior CD (group 2), and 26 had 2 or more prior CDs (group 3). The primary outcome was the success rate of TOP. Secondary outcomes were time from induction to abortion, total dose of misoprostol used, and occurrence of uterine rupture. RESULTS: The success rates were 96.4% in group 1, 81.7% in group 2, and 76.9% in group 3 (P=0.001). Time from induction to abortion, total dose misoprostol, and duration of hospital stay differed significantly among the groups (P=0.001 for all variables). There were 3 cases (11.5%) of uterine rupture in group 3, for an overall rate of 1.1%. CONCLUSION: Misoprostol inserted vaginally was effective for midtrimester TOP but the safety of using misoprostol in women with 2 or more prior CDs cannot be confirmed from the present study. Misoprostol should be used carefully, particularly in women with 2 or more prior CDs.