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Ectodermal Dysplasia (ED) is a rare genetic condition characterized by the involvement of ectoderm derivatives such as hair, nail, sweat glands, and teeth. It has many variants, but the two most common ones are hypohidrotic/anhidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia. Herein, we present a case of a 20-year-old female with hypohidrotic ectodermal dysplasia who had anodontia, hypohidrosis, and hypotrichosis, and her condition went unrecognized until she was seen for gastroenteritis at a tertiary care center. This case report will help spread education and awareness regarding such a rare and under-recognized condition. Early diagnosis and intervention help improve the quality of life.

Acrodermatitis Enteropathica is a rare hereditary condition characterized by perioral dermatitis, diarrhea, and alopecia. Etiology includes autosomal recessive inheritance and acquired causes such as protein malnutrition, malabsorption syndromes, premature births, parenteral nutrition, chronic illnesses, and alcoholism. We report a case of a 12-year old boy who presented with cutaneous manifestations involving the whole body, diarrhea, and sparse hairs. A low level of plasma zinc, and alkaline phosphatase level was revealed and he was started on zinc supplements. He significantly improved in a few days, and was discharged after counseling the parents about treatment compliance. This case highlights importance of early diagnosis, and the importance of treatment compliance in Acrodermatitis Enteropathica. It also stresses the need to take measures to ensure the continuity of health dissemination especially in remote areas.

Capacitive deionization (CDI), as a promising desalination technology, has been widely applied for water purification, heavy metal removal and water softening. In this study, the hierarchical porous carbon (HPC) with extremely large specific surface area (∼1636 m

Rutherford back scattering technique can be used to investigate ion migration in halide perovskites.

Wild and managed pollinators are the key component of biodiversity, contributing to important ecosystem services such as pollination and supporting human food security. Pollination by insects is a crucial component of the food chain that ensures the production of fruits and strongly affects the fruit quality, but the effect of insect pollination on fruit physiological and chemical parameters is largely unknown. The current study was conducted to determine the insect pollinators diversity and their relative abundance in the loquat (Eriobotrya japonica) orchard during 2017–2019. Further, the effect of insect pollinators pollination on the physiological and chemical parameters of fruit quality as compared to control pollinated flowers was investigated. The results revealed that a total of 22 species from 3 families (Apidae, Halictidae, and Syrphidae) were identified during the flowering season. The Apidae and Syrphidae were the most frequently observed families with major groups honey bees (67.89%) and hoverflies (21.57%), respectively. Moreover, results indicated that the fruit yield by the open-pollinated flowers (22.31 ± 0.34 kg/tree) was significantly higher than the control pollinated flowers (14.80 ± 0.25 kg/tree). Physiological and chemical parameters of loquat fruit differed significantly when fruits obtained from open-pollinated flowers as compared to control pollinated flowers. These results suggested that native insect pollinators play important role in the fruit quality of loquat. Hence, maintenance of appropriate habitat of native pollinators near loquat orchards is necessary to ensure good productivity and fruit quality.

Alkaline-earth metal-based MOF has many merits such as light weight, environmental friendliness and low cost, but it has rarely been used in energy storage and capacitive deionization (CDI). This paper presents a facile method for the fast synthesis of small-size magnesium formate MOF (Mg-MOF) under mild conditions by linker-free incubation in the presence of Mg(CH3COO)2⋅ 4H2O and dimethylformamide. The formic acid was in situ formed as ligand to facilitate the formation of Mg-MOF. The effect of incubation time on the morphology of MOF crystals was investigated. The small and well-defined Mg-MOF particles with the size of ~ 18 nm were formed in 1 h incubation, characterized by scanning electron microscopy and X-ray diffraction. Following carbonization, the Mg-MOF-derived mesoporous carbon was obtained. N2-sorption isotherm confirmed the mesoporous structure. Cyclic voltammetry test indicated the good electric double-layer feature of the Mg-MOF-derived carbon electrode, and the impedance results showed the excellent conductivity. As a capacitor electrode material, it delivered the specific capacitances of 102 F/g by GCD (0.2 A/g). The Mg-MOF-derived carbon electrode was applied successfully for capacitive deionization (CDI) with a desalination capacity of 8.0 mg/g in a CDI device. A supper fast desalination rate of 1.1 mg/g/min was achieved due to the abundant mesoporous structure. The work provides a cost effective and environmentally friendly way for the synthesis of MOF-based carbon materials free of transition metal, and the prospects for CDI application have been demonstrated.

The nutritional requirements of mosquitoes include both sugar (generally derived from the nectar of flowers) and blood (humans or animals). Mosquitoes express different degrees of preferences towards hosts depending on behavioral, ecological, and physiological factors. These preferences have implications for mosquito-borne disease risk. The present study is directed to reveal the effect of the human blood groups on the fecundity and fertility of the malaria vector Anopheles stephensi. In laboratory tests, mosquitoes were fed on ABO blood groups via artificial membrane feeders, and the level of attraction against different blood groups was tested by the electroantennogram and wind tunnel bioassay under control conditions. Results indicate that the female mosquitoes had a strong preference towards the blood group B, while in the case of females fed on O blood group had the highest digestibility rate. Overall, the human blood type had a significant impact on the fecundity and fertility of female An. stephensi. The highest numbers of eggs are laid, in the case of blood group B, (mean (± SD)) 216.3 (8.81) followed by the AB, 104.06 (7.67), and O, 98.01 (7.04). In the case of blood group B, females attain the highest fertility of about 92.1 (9.98). This study provides novel insight into the ABO blood type host choice of the mosquitoes that are still partially unknown and suggests encouraging personal protection for relevant individuals within communities at risk, which is a useful tool for preventing malaria where the An. stephensi is present as a dominant vector.

Dengue infection is common in 129 countries including Pakistan, with an estimated 390 million dengue virus infections per year. Asia accounts for 70% of the actual burden. Clinical signs and symptoms of dengue include a high fever (40°C/104 °F) accompanied by two of the following symptoms during the febrile phase: severe headache, pain behind the eyes, muscle and joint pains, nausea, vomiting, swollen glands and rash. Severe dengue is a potentially fatal complication, due to plasma leaking, fluid accumulation, respiratory distress, severe bleeding or organ impairment. Dengue virus does not usually cause neurological manifestations, unlike other arboviral infections. Encephalopathy is the most common neurological complication. Encephalitis is rare manifestation. We report a rare case of dengue encephalitis from Pakistan, with extensive brain lesions involving the thalamus, cerebellar vermis and frontoparietal periventricular regions. Therefore, we suggest dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in Asian countries such as Pakistan where dengue is rampant as dengue encephalitis can be fatal at times.

Background Vitamin D (Vit-D) plays a central role in calcium homeostasis and maintains skeletal integrity. Housewives in Quetta, Pakistan are at increased risk of vitamin D deficiency (VDD). They spend a greater part of their day in cleaning, washing, cooking, managing daily groceries, and other household chores. Thus, little time is left for self-care and outdoor activities. They wear hijab and have very little exposure to sunlight. In addition, their diet is deficient in Vit-D-rich food items, rendering them at high risk of VDD. Fear of getting tanned, melasma, and preference for a fair complexion further limit their sun exposure. This study evaluates the prevalence of VDD in housewives and determines its various risk factors to recommend screening guidelines for VDD. Methods A cross-sectional study was performed between November 2020 and April 2021 and recruited housewives aged >18 from the outpatient department of a tertiary care hospital in Quetta. Informed consent was obtained from all participants. VDD was defined as a serum 25(OH)-D level

Chorea is caused by a number of conditions, including genetic, metabolic derangements, infections, drugs, toxins, tumors, and disorders of the immune and inflammatory system of the body. Huntington's disease (HD) is the most common genetic cause of chorea. Systemic lupus erythematosus (SLE) is an autoimmune condition. Common symptoms include oral ulcers, joint pain, malar or discoid rashes, photosensitivity, and blood dyscrasias. It can involve the heart, lungs, kidneys, and brain. SLE can cause neuropsychiatric manifestations like psychosis, seizures, headache, confusion, and stroke. Chorea is a known symptom of SLE. HD is now recognized to involve more than one system and is associated with a number of comorbid conditions. We report the first case of hereditary choreiform disorder associated with and aggravated by SLE. This is also the first case report of probable Huntington disease from Balochistan, Pakistan. We report a 19-year-old girl with choreiform disorder and a family history of chorea. Choreiform disorder was present in her paternal grandmother and uncles. She presented with fever, cough, and aggravation of choreiform movements of upper and lower limbs for 10 days. She also complained of pain in the small joints of her hands and feet, oral ulcers, hair loss, and aggravation of choreiform movements for two and half months. Probable differential diagnoses of HD, Wilson's disease, and other types of hereditary chorea, aggravated by infections, SLE, or Covid-19, were made. Her initial lab results revealed pancytopenia, increased D-dimers and serum ferritin, positive antinuclear antibodies (ANA), and anti-double-stranded DNA (anti-dsDNA). Her C-3 and C-4 complement factors were low. The rest of the lab test results, including polymerase chain reaction (PCR) coronavirus disease ( COVID-19), blood culture, and malaria, were negative. Thus, a diagnosis of hereditary chorea associated with and aggravated by SLE was made. Hereditary choreiform disorders can be associated with and aggravated by autoimmune conditions like SLE. Thus, it is recommended to be vigilant and have a low threshold for diagnosing co-existing autoimmune conditions like SLE in patients with hereditary choreiform disorder.

Trace nickel oxide-embedded hierarchical porous carbon nanofibers (CNF-NiO) were fabricated by electrospinning polyacrylonitrile-Ni(NO3)2 (PAN-Ni) followed by stabilization, carbonization and acid treatment. The resultant CNF-NiO was characterized by scanning electron microscopy, transmission electron microscopy, X-ray photoelectron spectroscopy, thermo gravimetric analysis and nitrogen adsorption-desorption isotherm. The NiO amount and porous structure can be tuned by varying the PAN/Ni ratio and acid treatment time. The electrochemical properties of the electrospun CNF-NiO nanofibers were analyzed by cyclic voltammetry and impedance method. The specific capacity of 157.9 F/g was obtained at the PAN/Ni mass ratio of 90:3 with 3 h HCl wash. This porous CNF-NiO composite has been applied as a self-supporting cathode for capacitive deionization. The desalination amount arrived at 6.2 mg/g, which is 3 times as high as that of the electrospun pure carbon fibers. Longer wash time leads to decreased capacitance and desalination performance.

An advancement in Hyperspectral Imaging (HI) technology is creating important attraction among the researchers to develop better classification techniques. This technology is well known for its high spatial and spectral information due to which the discrimination of materials is much more accurate and efficient. The useful information is extracted in Hyperspectral Imaging technology after applying it in agriculture, biomedical, and disaster management studies. A review comparison has been carried out for air borne images using hyperspectral acquisition hardware for classification as well as segmentation purpose. Numerous approaches that have been focused for implementation namely semi-supervised technique used for hyperspectral imaging using active learning and multinomial logistic regression, Generalized Composite Kernels (GCKs) classification framework, classification of spectral-spatial based data on loopy belief propagation (LBP), multiple feature learning of HI classification, and semi-supervised GCKs with classification accuracy on AVIRIS dataset (59.97%, 92.89%, 81.45%, 75.84%, and 95.50) and segmentation accuracies using α-expansion method as (73.27%, 93.57%, 92.86%, 91.73% and 98.31), respectively.

Ascariasis is the most common helminthic infection. It is most common in children of tropical and developing countries where the transmission is by contamination of soil by human feces or use of untreated feces as fertilizer. Transmission in most endemic areas is via person to person contact. We hereby present a case report of acute pancreatitis due to ascariasis. Twenty-five-year-old male patient presented to us with chief complaint of acute epigastric pain radiating to back and associated with vomiting. Initial lab investigations revealed increased serum amylase and lipase. Probable diagnosis of acute pancreatitis was made. CT scan was done and report revealed bulky pancreas, and significant peri-pancreatic fat stranding. He was managed symptomatically with intravenous fluids, analgesics, anti-emetics and enteral nutrition. However, the cause remained undetermined as we ruled out the possible etiologies of acute pancreatitis until one day the patient vomited a 15-cm round worm. Thereafter, his condition improved dramatically. This is one of the first few case reports of ascariasis-induced pancreatitis from Pakistan and the first one from Baluchistan province of Pakistan. Thus it highlights ascariasis as possible etiology of acute pancreatitis in regions where ascariasis is geographically endemic.

Objective: Hepatitis B and C is a blood borne infection which attacks the liver, causing both acute and chronic liver disease. It is one of the major causes of end stage liver disease including cirrhosis andhepatocelluar carcinoma. Our study is about the knowledge, attitude and perception of Hazara population of Quetta Pakistan regarding hepatitis B and C. Hazaras are the third largest ethnicity in Afghanistan and a minority in Pakistan. Special focus has been given to flagellation practice during "Ashura" in the first Islamic month called "Muharram". The "Mourning of Muharram"marks the martyrdom anniversary of Imam Hussain Ibn Aliat Karbala Iraq by the forces of the second Umayyad caliph “Yazeed”. Imam Hussain was the grandson of Prophet Muhammad, peace be upon him. During flagellation the mourners have open wounds, blood splatters all around. The mourners flagellate their back and head with sharp mourning blades and knives. Thus there is a hypothetical risk of hepatitis B and C transmission from those are already infected to others. Subjects and methods: A cross sectional survey was conducted by a survey questionnaire. The survey questionnaire was generated on google forms and conducted from August 2020 to October 2020. It assessed the knowledge, attitude and perception of Hazara ethnicity of Quetta Pakistan about Hepatitis B and C. The questions were verified and taken from "World Health Organization" and "Center for Disease Control”. We adopted convenience sampling. The responses were collected by interviewers. The interviewers visited the five main hospitals serving Hazara population, local academic institutions, neighborhood homes, and roadside shops. In addition the survey was also forwarded on social media.Both English and Urdu version of the questionnaire in the same form was generated. The questionnaire asked about their knowledge of signs and symptoms, modes of transmission, vaccination status, and other general perception, attitude and practices towards hepatitis B and C. The survey also explored Hazara community’s perception, practices and attitude towards flagellation with mourning blades. The data from the google form was retrieved and transferred to excel spreadsheet for further analysis. Results: A total of 650 responses were collected. After excluding incomplete responses and responses from other ethnicities, the following results were obtained for a total of 603 responses. Among the respondents 58.2 % were aged 18-30, female and male constituted 56.88% and 43.11% of the respondents respectively. 30.67 % had graduate degrees. 27.19 % respondents were students while 25.37 % were housewives. The percentage of respondents who knew the sign and symptoms were as follows: fatigue 50.7 %,Jaundice 46.6 %, nausea and vomiting 37.8%. The percentageaware of the modes of transmission were as follows: Unscreened blood transfusion 66.8%, reused syringes 75.78%, tattoo, ear, nose piercing 55.7 %, sexual transmission 54.22.Only 44.4 % knew that hepatitis B has vaccine available where as 54.5 % had not been vaccinated.49.5 % did not know that hepatitis B and C is quite prevalent in Pakistan. 62 % expressed flagellation as a risk factor. 28.05 % of male respondents (85 out of 218 male respondents) had flagellated which is a considerable proportion of male Hazara population,23.5 % of whom had exchanged flagellation blades with each other. 15.09 % of respondents do not believe on protective effects of hepatitis vaccine to those who flagellate. A large proportion of those who had flagellated (55.3 %) declined getting screening test for hepatitis B or C. Conclusion: There is a need of spreading awareness among the Hazara population regarding hepatitis B and C, particularly regarding the flagellation practice, which should also be scientifically investigated as a possible risk factor. Vaccination and avoiding exchange of flagellation blades should be encouraged. Key words: Hepatitis B and C, flagellation with mourning blades, Muharram, Ashura, Quetta, Pakistan.

The carbon material derived from citrus peel was prepared via hydrothermal synthesis method with low amount of ZnCl2. The morphology and surface structure of the synthesized carbons were characterized by field emission scanning electron microscope (FESEM), X-ray diffraction (XRD) and N2 adsorption/desorption isotherms measurements. The electrochemical and capacitive deionization performance of carbon materials were comparatively studied by cyclic voltammetry (CV), electrochemical impedance spectroscopy (EIS) and galvanostatic charge-discharge (GCD). Compared with the materials activated with KOH or H3PO4, ZnCl2-activated carbon showed the best electrochemical double-layer characteristics, with the specific capacity of 120 F g−1. When being applied as anode in the capacitive deionization (CDI) cell, the desalination amount of 16 mg g−1 and the average salt adsorption rate (ASAR) of 0.67 mg g−1 min−1 were achieved, which is much higher than those of KOH- and H3PO4-activated materials. The desalination amount retained 80% after 35 cycles. The possible mechanism is proposed in light of X-ray photoelectron spectroscopy (XPS) and Fourier transform infrared (FTIR) analysis. This work provides a facile and environmentally friendly method for the preparation of a green and low cost biomass derived porous carbon material by fruit waste with a low amount (1% mass) of activation regents for high performance capacitive deionization.

Background A significant proportion of patients with acute myeloid leukemia (AML) will either be refractory to initial chemotherapy or will suffer refractory relapse. The role of allogeneic transplantation (SCT) in active disease is contentious. There is a growing body of literature that sequential chemotherapy, pioneered by the German FLAMSA regimen, followed by RIC SCT is a safe and efficacious modality in these patients, and there have been numerous modifications of this regimen, especially as amsacrineis not widely available. Fludarabine, cytarabineand and etoposide (VP16) (FLAV) have been reported as an effective salvage regimen. Here we report on single center outcomes of a variation of the FLAMSA regimen, substituting amsacrine for etoposide with mainly myeloablative conditioning. Methods Patients were offered this regimen if fit for allogenic HSCT and had AML which is refractory to two cycles of chemotherapy or refractory to one cycle and considered at high risk for complication with second cycles. Patients with MDS received this regimens if eligible for transplant with high or very high risk cytogenetics. All patients received cytoreductive chemotherapy consisted of fludarabine 30mg/m2/day x 4 days, Cytarabine 2g/m2/day x 4 days, etoposide 100mg/m2/day x 3days, commenced simultaneously. After 3 days of rest, conditioning chemotherapy consisted of fludarabine 30mg/m2 x 2 days and and IV busulfan 0.8mg/m2 q 6 hours; the number of busulfan doses varied between 8 - 12, depending on patient comorbidity. All patients received 2 doses of ATG at 2.5mg/m2/day on day -3 and -2. All patients received GCSF mobilized peripheral blood hematopoietic cells. Post-transplant GVHD prophylaxis consist of CsA and MMF. CsA was tapered from day+60 and stopped at day +90 in the absence of GVHD. MMF was discontinued between day +30 and day +40. Donor lymphocyte infusions were collected for planned prophylactic DLI. Results Twenty six patients received FLAV-SCT between March 2014 and July 2019. The median age was 38 (14-60); 16 (62%) female. Overall 12 (46%) pts had de novo AML, 10 (39%) pts had secondary or therapy related AML and 4 (15%) pts had MDS. Fourteen (54%) pts had adverse risk cytogenetics include 8 (31%) pts had complex or monosomal karyotype. Patients' characteristics are summarized in Table 1. All patients had active disease prior to FLAV-SCT. The median time for ANC and platelet engraftment was 14 (10 - 42) days and 17 (10 - 52) days respectively. Day 30 assessment shows CR in 16 (61%) pts and CR/CRi in 17 (65%) pts. Outcomes are summarized in Table 2. Three patients (12%) developed veno-occlusive disease. Acute GVHD grade II-IV and III-IV occurred in 9 (35%) pts and 2 (8%) pts respectively. Three (12%) patients developed chronic GVHD. Cumulative incidence of NRM at 100 days and 2 years was 8% and 12% respectively. The median OS for all pts was 5.2 months with 2 years rate of 32% (15 - 50). Among responders, the median OS and RFS were 19.2 months and 8.7 months, 2y-OS and RFS were 47% and 25%, respectively (Figure 1). Conclusion Our result demonstrates that transplant is an effective therapeutic modality in this very high risk refractory AML/MDS patients. Sequential chemotherapy (FLAV) followed by SCT with busulfan at myeloablative dose is tolerable with an acceptable toxicities and encouraging results. Figure 1 Figure 1. Disclosures Chaudhri: Novartis: Honoraria; Abbvie: Honoraria; Astra Zeneca: Honoraria. Alzahrani: King Faisal Specialist Hospital and Research Centre: Current Employment; Novartis: Consultancy, Honoraria; Bayer: Consultancy, Honoraria, Speakers Bureau; Sobi: Consultancy, Honoraria, Speakers Bureau; Pfizer: Consultancy, Honoraria, Speakers Bureau.

Background Gall stone and alcohol are the two most common causes of pancreatitis. Other etiologies include trauma, post endoscopic retrograde cholangiopancreatography (ERCP), bacterial, viral, fungal, and parasitic infections, hypertriglyceridemia, medications, and scorpion bite to name a few. Hypercalcemia is a rare cause of acute pancreatitis. Hypercalcemia can be due to primary hyperparathyroidism (PHPT) manifested by raised parathyroid hormone and serum calcium level. In addition acute pancreatitis is a rare presenting complain of PHPT. PHPT can be asymptomatic or present with symptoms of bone pain, kidney stones, nephrocalcinosis, renal failure, peptic ulcer disease, accelerated atherosclerotic heart disease, hypertension and proximal muscle weakness. Osteitis fibrosa cystica is the term for radiologically visible characteristic feature of skeletal involvement. Case presentation We report a case of 20 years old male with chief complaint of epigastric and left hypochondrium pain associated with vomiting for two months. Abdominal pain was gradual in onset, radiating to back, and progressive. He also had complains of constipation, blood tinged feces and pain during defecation. Initial lab tests were significant for increased amylase, lipase and a diagnosis of acute pancreatitis was made which was confirmed by CT scan. After ruling out the common etiologies of acute pancreatitis, further investigations for uncommon etiologies revealed increased IgG-4 values suggesting autoimmune pancreatitis. However further investigations were done and it did not meet the diagnostic criteria for autoimmune pancreatitis. Covid-19 IgG antibodies were positive, however patient did not have any other symptoms of Covid-19 and PCR Covid-19 test was negative. Although Covid-19 can cause pancreatitis, given the ongoing pandemic, he might have been asymptomatically infected with Covid-19 and developed immunity. Therefore we did not believe Covid-19 to be the cause of his pancreatitis. Thus a search for other rare etiologies was initiated which revealed serum calcium of 15 mg/dL. Serum parathyroid hormone and urinary calcium were increased several times suggesting primary hyperparathyroidism. DEXA scan revealed osteoporosis. Conclusion Hypercalcemia is one of the rare etiologies of pancreatitis. Thus it should be considered as one of the etiologies of acute pancreatitis after ruling out the more common ones. Secondly, this Case report highlights acute pancreatitis as one of the rare presenting complain of primary hyperparathyroidism which may be associated with several times increased IgG-4 values. Covid-19 has been associated with a number of presenting complains and can cause acute pancreatitis, amid the ongoing pandemic physicians should exercise caution to avoid spurious association of Covid-19 with acute pancreatitis or other presenting complains.

A new methodology, the hybrid learning system (HLS), based upon semi-supervised learning is proposed. HLS categorizes hyperspectral images into segmented regions with discriminative features using reduced training size. The technique utilizes the modified breaking ties (MBT) algorithm for active learning and unsupervised learning-based regressors, viz. multinomial logistic regression, for hyperspectral image categorization. The probabilities estimated by multinomial logistic regression for each sample helps towards improved segregation. The high dimensionality leads to a curse of dimensionality, which ultimately deteriorates the performance of remote sensing data classification, and the problem aggravates further if labeled training samples are limited. Many studies have tried to address the problem and have employed different methodologies for remote sensing data classification, such as kernelized methods, because of insensitiveness towards the utilization of large dataset information and active learning (AL) approaches (breaking ties as a representative) to choose only prominent samples for training data. The HLS methodology proposed in the current study is a combination of supervised and unsupervised training with generalized composite kernels generating posterior class probabilities for classification. In order to retrieve the best segmentation labels, we employed Markov random fields, which make use of prior labels from the output of the multinomial logistic regression. The comparison of HLS was carried out with known methodologies, using benchmark hyperspectral imaging (HI) datasets, namely “Indian Pines” and “Pavia University”. Findings of this study show that the HLS yields the overall accuracy of {99.93% and 99.98%}Indian Pines and {99.14% and 99.42%}Pavia University for classification and segmentation, respectively.

Hyperspectral imaging (HI) is getting much more attention among researchers in different fields like agriculture, defense, medical, and geographical surveys. In this work, we have proposed a novel automated system for the classification and segmentation of landscapes using hyperspectral images. The proposed semi-supervised based approach has improved the extraction of spatial characteristics of the scene that has employed an extended multi-attribute profile (EMAP) by stacking of several attributes. The unlabeled data points located near the classifier boundaries are selected on the basis of entropy related to the corresponding class labels. In the next segmentation phase, MLR probabilities are computed against the output of classifier. Finally, maximum-a-posteriori segmentation is carried out on the multilevel logistic prior labels. The simulated results have obtained classification accuracy of 95.50% by comparing predicted labels with original ones. The segmentation accuracy, after developing regions on the output of classification, is 98.31%. A performance comparison of the proposed approach with several approaches has also been carried out.

In recent years, the demands for lithium are increasing sharply with the fast development of electrical vehicles, new green energy and portable electronic devices. To meet the demands, selective recovery of lithium from brine/seawater has attracted extensive attention. LiMn2O4 (LMO) is a good redox material for lithium recovery. However, the dissolution of Mn is inevitable during the redox process which can lead to severe capacity decay and poor cycle performance. In this paper, LiNi0.5Mn1.5O4 (LNMO) nanoparticles were prepared through one-step solid-state sintering and applied as a positive electrode in the hybrid capacitive deionization cell for lithium recovery. Cyclic voltammetry (CV) scan of the LNMO electrode in LiCl aqueous solution presented clear redox peaks, illustrating the reversible intercalation/de-intercalation of Li+ into/from LNMO; while the Na+, K+, Mg2+ and Ca2+ cations had negligible intercalation. Combined with an activated carbon (AC) negative electrode, the hybrid capacitive deionization cell (LNMO||AC) was assembled to selectively adsorb lithium, arriving at the adsorption amount of ~260 µmol/g, almost three times higher than that of the symmetric AC cell (AC||AC). Even in the Mg2+ and Li+ mixed binary salt solution at a high C Mg 2 + / C Li + ratio of 30, LNMO remained high selectivity to Li+ with the separation factor of ~104 achieved. In the synthetic brine water containing various salts, the LNMO||AC cell kept excellent selectivity to Li+ with the adsorption amount still remaining ~230 µmol/g. And the cell can preserve a retention of ~86% after 100 cycles, much higher than the LMO||AC cell (~50%). Meanwhile, neither Mn2+ nor Ni2+ were found in the cycling solution of LNMO||AC cell. Nevertheless, the LMO||AC cell deteriorated gradually with the dissolution loss ratio of Mn in LMO close to 40%. Such excellent selectivity and high stability of LNMO enable it applicable for practical lithium recovery application, superior to LMO.

Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure (BMF), constitutional anomalies and high risk of developing cancer. Distinguishing FA from severe aplastic anemia (SAA) can be challenging especially in asyndromic patients. We undertook a clinical and laboratory cohort study of adolescent and young adult (AYA) patients with a diagnosis of FA treated at our institution to characterize the clinical features in our population, and conducted a prospective translational study to explore integration of a genomic and proteomic approach for improved diagnosis and molecular characterization of FA. Methods: Data on FA patients was obtained from an institutionally approved BMF database and hematopoietic stem cell transplant (HSCT) database. Further data was obtained from a register of chromosomal breakage (CB) analysis results. Index cases were identified if they were older than 14 years of age at the time of diagnosis or under the care of adult hematology with a clinical diagnosis of FA based on the presence of BMF and abnormal CB, or clinical phenotype with the presence of homozygous FA related genes. Family pedigrees were constructed based on history. In addition, patients presenting with BMF were enrolled onto an institutionally approved study investigating proteomic biomarkers and genomics of BMF syndromes. Consented peripheral blood samples and/or extracted DNA were subject to either panel based next generation sequencing (NGS) testing as part of the Saudi Genome Project or subjected to whole exome sequencing (WES) by external lab. For proteomic analysis, peripheral blood plasma (PBP) samples from 6 patients with FA, 10 SAA patients and 7 normal controls were subjected to expression proteomics using liquid chromatography tandem mass spectrometry (LC-MS/MS). Result: Patients and clinical features: 55 patients (26 M, 29 F) in 30 families were identified. While 18 patients (32%) were referred with a diagnosis/suspicion of FA, in 26 (47%) FA was diagnosed at our institution. The most frequent anomaly was short stature (14 patients, 25%), skin changes (7, 12%), urogenital abnormalities (7, 12%), dysmorphism/craniofacial abnormalities (7, 12%), hands anomalies (4, 7%); 12 (22%) had no recorded anomalies. 18 patients (33%) developed a malignancy either before or after diagnosis of FA: solid tumors in 5 (9%), AML and/or MDS in 15 (27%); 3 (5%) of these patients had both solid tumors and AML/MDS. Diagnostic Tests: 35 patients (63.6%) had a positive CB analysis with diepoxybutane (DEB) or mitomycin-C (MMC) testing; in 5 patients (9%) DEB testing was borderline and 3 (5%) had a normal CBA but had a diagnostic phenotype+/- family history and presence of a homozygous mutation in a known FA related gene. 14 patients had cytogenetic abnormalities and abnormalities involving chromosome 1 were the most frequent (50%). Mutation Analysis: Mutational analysis was available for 12 (22%) cases; homozygous mutations in FA genes were identified in 10 patients (18%) in 7 families (23% of families): FANCA (5 patients/3 families); BRIP1 (2/2); FANCP (1/1); FANCD2 (2/1). In one case, post matched sibling (HSCT) blood sample revealed a known pathogenic heterozygous c.2632G>C,p.Glu878Gln mutation in FANCA, suggesting a carrier donor. Proteomic analysis: Over 1650 unique PBP protein species were identified of which 605 were significantly differentially expressed (≥ 2 to ∞ - fold change & p < 0.001) between SAA /FA/ normal control subjects (Fig 1a). DNMT3A, Kinase Insert Domain Receptor (KDR) and TGFB-1 was found to be highly expressed in SAA versus FA, while ATM and APOB were highly expressed in FA versus SAA (Fig.1b). Treatment outcomes: 36 out of 55 patients (65%) received HSCT. Actuarial survival of HSCT (n=37) and non-HSCT (n=14) patients was 70% and 77%, respectively. Treatment details were not available on 6 (11%). CONCLUSION: We report the first characterization of AYA patients with FA in Saudi Arabia. Our report emphasizes the need for a high index of suspicion of a diagnosis of FA in BMFs. CB may be falsely negative in cases, and panel based and/or WES based NGS testing increases diagnostic accuracy; in this cohort, mutations in FANCA were the most frequent (50%). Occurrence of hematological and solid tumors is a significant risk in these AYA patients. We also report proteomic panels as potential biomarkers that distinguish FA from SAA and may provide mechanistic insights. Disclosures No relevant conflicts of interest to declare.

Background: Primary central nervous system lymphoma (PCNL) is relatively uncommon type of lymphoid malignancy and there is paucity of data on the incidence and prevalence, outcomes of these patients from Middle Eastern region. Methods: We did a retrospective chart review of over 1200 patients diagnosed with lymphoma at our centre which is a tertiary care teaching hospital. We identified 10 patients (over the age of 18 years) with PCNL over a ten year period from 2005-2015. Results: Out of 10 patients, 4 (40%) were males. Majority was over 45 years of age (70%). 8 of 10 (80%) patients had ECOG performance status of 3 or more. Histologically, 8 of 10 (80%) patients belonged to diffuse large B cell lymphoma, the rest were primary burkitt's lymphoma of the brain and primary T cell lymphoma respectively. Modalities of treatment received were Whole Brain Radiotherapy only (20%), High Dose Methotrexate (HD MTX) with High Dose Cytarabine (HD ARA C) (40%), both chemoradiation (40%), no treatment due to poor performance status (20%). Rituximab was given to all patients who received chemotherapy. Patient with Primary Burkitt's lymphoma received anthracycline containing combination chemotherapy with high dose methotrexate (RCHOPM) along with intrathecal chemotherapy. In the chemotherapy group 67% of patients achieved (4 out of 6) either partial remission (PR) or complete remission (CR) while 33% were refractory to treatment. Two patients (20%) received upfront radiotherapy only and achieved PR and CR respectively. Of the two patients who were refractory to upfront chemotherapy, one was salvaged with WBRT and is alive after 5 years, the other was lost to follow up. Of two patients who received upfront radiotherapy one was lost to follow up, the other relapsed after nearly 47 months later and was salvaged with Temozolamide and Rituximab, achieved partial remission and is well at the time of analysis. Of the evaluable 8 of 10 patients, at a median follow up of 31 months (Range1-66), 6 patients were alive and four without relapse or progression with an overall survival and progression free survival of 75% and 50% respectively. Discussion: To our knowledge this is the first case series of PCNL from Saudi Arabia or Middle East. All the evaluable patients who received treatment are alive at the time of analysis. Our series shows PCNL is extremely rare malignancy even this part of the world and DLBCL remains the commonest histological form like in west. Radiotherapy remains valuable tool and can salvage patients refractory to chemotherapy achieving long periods of remission, its utility must not be underestimated even in elderly. Outcomes of patients at our institute are comparable with other centers, although numbers are small. Further detailed studies are planned to define exact incidence, prevalence, characteristics and outcomes of these patients from this region. Disclosures No relevant conflicts of interest to declare.

Background: Secondary central nervous system (CNS) involvement with diffuse large B cell lymphoma (DLBCL) is associated with poor outcomes. We looked at characteristics and outcomes of these patients treated at King Fahad Medical City, Riyadh over eleven year period from 2005-2016. Methods: Out of 1235 patients diagnosed with lymphoma at our centre over a ten year period from 2005 to 2016 we identified 10 adult patients over the age of 18 years, who had DLBCL with secondary CNS involvement. It was a retrospective chart review. Results: Out of 10 patients, 4 (40%) were males and 6 (60%) were females.7 of 10 (70%) were over 45 years of age. All patients had stage III disease or beyond with at least one extranodal involvement at presentation. All patients had evidence of CNS involvement either radiologically or histologically or both. 9 of 10 patients (90%) had CNS involvement within one year of presentation (whilst on treatment or within a year of diagnosis) while one patient had been treated for gastric lymphoma 10 years before relapse. 2 of 10 (20%) patients presented with concurrent CNS involvement at initial presentation while 8 of 10 (80%) had CNS involvement in addition to systemic disease due to progression of disease whilst on treatment or due to relapse. 3 of 10 (30%) patients who did not have CNS involvement at presentation received CNS prophylaxis (one patient received high dose methotrexate, the three other received intrathecal chemotherapy).7 of 10 (70%) patients received anthracycline containing chemotherapy with Rituximab as upfront, the other three were unfit for intensive chemotherapy. Treatment at relapse or on progression included various modalities including High Dose Methotrexate, High Dose Cytarabine or both, Whole Brain Radiotherapy, Intrathecal Chemotherapy, Temozolamide, however all patients received Rituximab. Only one patient underwent autologous stem cell transplant but relapsed shortly afterwards. After CNS involvement, all patients (n=7) were uniformly refractory to chemotherapy/radiotherapy or both. 3 patients were unfit to receive any treatment and died within a month of diagnosis. Median survival was only 3.5 months from the time of CNS involvement (range: 1-8 months). None of the patients were alive beyond 8 months of CNS involvement. Discussion: To our knowledge this is the first case series of DLBCL with secondary CNS involvement from Middle East. This study shows that CNS involvement is rare but associated with extremely poor outcome as shown in other studies, with a median survival of only 3.5 months. None of the patients were responsive to therapy or were alive beyond 8 months of CNS involvement. It is interesting to note even 3 patients who had received some form of CNS prophylaxis also had CNS involvement, suggesting aggressive nature of disease and ineffective current form of CNS prophylaxis, even if given. Further studies are needed to define incidence and characteristics and outcomes of these patients from this region. Though numbers are small to make any specific recommendation, patients with high risk disease should be considered for intensive CNS prophylaxis upfront otherwise CNS involvement later would invariably associated with dismal outcome irrespective of any therapy. There is certainly urgent need for improvement in how we manage these high risk patients with probable inclusion of novel B cell antagonists and aggressive CNS prophylaxis upfront through well designed clinical trials. Disclosures No relevant conflicts of interest to declare.