Search results for: Enamel renal syndrome
Commenced in January 2007
Frequency: Monthly
Edition: International
Paper Count: 1069

Search results for: Enamel renal syndrome

829 Isolation and Characterization White Spot Syndrome Protein Envelope Protein 19 from Black Tiger Shrimp (Penaeus monodon)

Authors: Andi Aliah Hidayani, Asmi Citra Malina A. R. Tassakka, Andi Parenrengi

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Vanname Shrimp is one of the high yielding varieties that are more resistant to virus attacks. However, now this shrimp more death due to virus attack such as white spot disease caused by white spot syndrome virus (WSSV). Various efforts have done to prevent the disease, like immunostimulatory, probiotics, and vaccine. White spot syndrome virus (WSSV) envelope protein VP19 gene is important because of its involvement in the system infection of shrimp. This study aimed to isolate and characterize an envelope protein VP19 – encoding gene of WSSV using WSSV infected Vanname Shrimp sample from some areas in South Sulawesi (Pangkep, Barru and Pinrang). The genomic of DNA were isolated from shrimp muscle using DTAB-CTAB method. Isolation of gene encoding envelope protein VP19 WSSV ws successfully performed with the results of the length of DNA fragment was 387 bp. The results of homology analysis using BLASTn homology suggested that these isolates genes from Barru, Pangkep and Pinrang have closest relationship with isolates from Mexican.

Keywords: vanname, shrimp, WSSV, viral protein 19

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828 Association of Genetic Variants of Apolipoprotein A5 Gene with the Metabolic Syndrome in the Pakistani Population

Authors: Muhammad Fiaz, Muhammad Saqlain, Bernard M. Y. Cheung, S. M. Saqlan Naqvi, Ghazala Kaukab Raja

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Background: Association of C allele of rs662799 SNP of APOA5 gene with metabolic syndrome (MetS) has been reported in different populations around the world. A case control study was conducted to explore the relationship of rs662799 variants (T/C) with the MetS and the associated risk phenotypes in a population of Pakistani origin. Methods: MetS was defined according to the IDF criteria. Blood samples were collected from the Pakistan Institute of Medical Sciences, Islamabad, Pakistan for biochemical profiling and DNA extraction. Genotyping of rs662799 was performed using mass ARRAY, iPEX Gold technology. A total of 712 unrelated case and control subjects were genotyped. Data were analyzed using Plink software and SPSS 16.0. Results: The risk allele C of rs662799 showed highly significant association with MetS (OR=1.5, Ρ=0.002). Among risk phenotypes, dyslipidemia, and obesity showed strong association with SNP (OR=1.49, p=0.03; OR =1.46, p=0.01) respectively in models adjusted for age and gender. Conclusion: The rs662799C allele is a significant risk marker for MetS in the local Pakistani population studied. The effect of the SNP is more on dyslipidemia than the other components of the MetS.

Keywords: metabolic syndrome, APOA5, rs662799, dyslipidemia, obesity

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827 Molecular Characterization of White Spot Syndrome Virus in Some Cultured Penaeid Shrimps of Coastal Regions in Bangladesh

Authors: Md. Baki Billah, Suraiya Parveen, Shuvra Kanti Dey

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Bangladesh is earning a lot of foreign currency by exporting shrimp, but this industry is facing a tremendous problem due to the infection of white spot syndrome virus (WSSV). This study was undermined to develop rapid detection method of WSSV. A total of shrimp samples 240 collected from the 12 shrimp farms of different coastal regions (Satkhira, Khulna, and Bagerhat) were analyzed by conventional PCR using VP28 and VP664 gene-specific primers. In satkhira, Bagerhat and Khulna 39, 41 and 29 samples were found WSSV positive respectively. Real-time PCR using 71-bp amplicon for VP664 gene correlated well with conventional PCR data. The prevalence rates of WSSV among the collected 240 samples were Satkhira 38%, Khulna 47% and Bagerhat 50%. Molecular analysis of the VP28 gene sequences of WSSV revealed that Bangladeshi strains phylogenetically affiliated to the strains belong to India. This work concluded that WSSV infections are widely distributed in the coastal regions cultured shrimp in Bangladesh. Physico-chemical parameters were within the range of fish culture.

Keywords: coastal regions of Bangladesh, PCR, shrimp, white spot syndrome virus

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826 Dialysis Access Surgery for Patients in Renal Failure: A 10-Year Institutional Experience

Authors: Daniel Thompson, Muhammad Peerbux, Sophie Cerutti, Hansraj Bookun

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Introduction: Dialysis access is a key component of the care of patients with end stage renal failure. In our institution, a combined service of vascular surgeons and nephrologists are responsible for the creation and maintenance of arteriovenous fisultas (AVF), tenckhoff cathethers and Hickman/permcath lines. This poster investigates the last 10 years of dialysis access surgery conducted at St. Vincent’s Hospital Melbourne. Method: A cross-sectional retrospective analysis was conducted of patients of St. Vincent’s Hospital Melbourne (Victoria, Australia) utilising data collection from the Australasian Vascular Audit (Australian and New Zealand Society for Vascular Surgery). Descriptive demographic analysis was carried out as well as operation type, length of hospital stays, postoperative deaths and need for reoperation. Results: 2085 patients with renal failure were operated on between the years of 2011 and 2020. 1315 were male (63.1%) and 770 were female (36.9%). The mean age was 58 (SD 13.8). 92% of patients scored three or greater on the American Society of Anesthiologiests classification system. Almost half had a history of ischaemic heart disease (48.4%), more than half had a history of diabetes (64%), and a majority had hypertension (88.4%). 1784 patients had a creatinine over 150mmol/L (85.6%), the rest were on dialysis (14.4%). The most common access procedure was AVF creation, with 474 autologous AVFs and 64 prosthetic AVFs. There were 263 Tenckhoff insertions. We performed 160 cadeveric renal transplants. The most common location for AVF formation was brachiocephalic (43.88%) followed by radiocephalic (36.7%) and brachiobasilic (16.67%). Fistulas that required re-intervention were most commonly angioplastied (n=163), followed by thrombectomy (n=136). There were 107 local fistula repairs. Average length of stay was 7.6 days, (SD 12). There were 106 unplanned returns to theatre, most commonly for fistula creation, insertion of tenckhoff or permacath removal (71.7%). There were 8 deaths in the immediately postoperative period. Discussion: Access to dialysis is vital for patients with end stage kidney disease, and requires a multidisciplinary approach from both nephrologists, vascular surgeons, and allied health practitioners. Our service provides a variety of dialysis access methods, predominately fistula creation and tenckhoff insertion. Patients with renal failure are heavily comorbid, and prolonged hospital admission following surgery is a source of significant healthcare expenditure. AVFs require careful monitoring and maintenance for ongoing utility, and our data reflects a multitude of operations required to maintain usable access. The requirement for dialysis is growing worldwide and our data demonstrates a local experience in access, with preferred methods, common complications and the associated surgical interventions.

Keywords: dialysis, fistula, nephrology, vascular surgery

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825 Protective Role of Peroxiredoxin V against Ischemia/Reperfusion-Induced Acute Kidney Injury in Mice

Authors: Eun Gyeong Lee, Ji Young Park, Hyun Ae Woo

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Reactive oxygen species (ROS) production is involved in ischemia/reperfusion (I/R) injury in kidney of mice. Oxidative stress develops from an imbalance between ROS production and reduced antioxidant defenses. Many enzymatic and nonenzymatic antioxidant systems including peroxiredoxins (Prxs) are present in kidney to maintain an appropriate level of ROS and prevent oxidative damage. Prxs are a family of peroxidases that reduce peroxides, with a conserved cysteine residue serving as the site of oxidation by peroxides. In this study, we examined the protective role of Prx V against I/R-induced acute kidney injury (AKI) using Prx V wild type (WT) and knockout (KO) mice. We compared the response of Prx V WT and KO mice in mice model of I/R injury. Renal structure, functions, oxidative stress markers, protein levels of oxidative damage marker were worse in Prx V KO mice. Ablation of Prx V enhanced susceptibility to I/R-induced oxidative stress. Prx V KO mice were seen to have more severe renal damage than Prx V WT mice in mice model of I/R injury. Our results demonstrate that Prx V is protective against I/R-induced AKI.

Keywords: peroxiredoxin, ischemia/reperfusion, kidney, oxidative stress

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824 Association of Vitamin D Levels in Obese and Non-Obese Patients with Polycystic Ovarian Syndrome in East Indian Populations

Authors: Dipanshu Sur, Ratnabali Chakravorty

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Introduction: Polycystic ovary syndrome (PCOS) is the most common metabolic abnormality such as changes in lipid profile, diabetes, hypertension and metabolic syndrome occurring in women. Hypovitaminsis D was found to be associated with the development of obesity and insulin resistance in women with PCOS. Aim: To evaluate the association of vitamin D levels in obese and non-obese patients with PCOS in an East Indian populations. Methods: A case control study was conducted. It enrolled 100 cases of PCOS based on Rotterdam criteria and 100 ovulatory normal cases matched for their age and BMI. Vitamin D levels were compared in the obese and non-obese PCOS groups and also with the controls. Results: The mean age of subjects was 29.48 ± 3.29 years in the PCOS group and 26.24 ± 2.56 years in the control group. Hypovitaminosis D was present in 75 out of 100 PCOS women (75.0%) and 25 women (25.0%) showed sufficient 25OHD levels ≥30 ng/ml. Women with PCOS had significantly lower total serum calcium (8.4 ± 0.25 mg/dl versus 9.8 ± 0.17 mg/dl in controls), and 25 OHD (21.2 ± 2.56 ng/ml versus 32.6 ± 2.23 ng/ml in control group) than ovulatory normal women. This difference remained significant for both groups after adjustment for BMI. Obese women in both groups had significantly lower concentration of calcium and 25OHD than normal weight patients in this study. Conclusion: Our study shows majority of the patients and controls had vitamin D deficiency and there was significant difference in the vitamin D levels in PCOS group and controls as well as obese and non-obese groups. This may reflect the vitamin D deficiency status of the community. Vitamin D deficiency should demands immediate attention as it is a severe problem among the East Indian population.

Keywords: vitamin D deficiency, polycystic ovary syndrome, obese, hypovitaminsis D

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823 Effect of Antioxidant-Rich Nutraceutical on Serum Glucose, Lipid Profile and Oxidative Stress Markers of Salt-Induced Metabolic Syndrome in Rats

Authors: Nura Lawal, Lawal Suleiman Bilbis, Rabiu Aliyu Umar, Anas A. Sabir

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Metabolic syndrome (MS) a high-risk condition involving obesity, dyslipidemia, hypertension, and diabetes mellitus is prevalent in Nigeria. The study aims to formulate an antioxidant-rich nutraceutical from locally available foodstuff (onion, garlic, ginger, tomato, lemon, palm oil, watermelon seeds) and investigate their effects on blood pressure, body weight, serum glucose, lipid profile, insulin and oxidative stress markers in salt-induced rats. The rats were placed on 8% salt diet for 6 weeks and then supplementation and treatment with nutraceutical and nifedipine in the presence of salt diet for additional 4 weeks. Feeding rats with salt diet for 6 weeks increased blood pressure and body weight of the salt-loaded rats relative to control. Significant (P < 0.001) increase in serum blood glucose and lipid profile, and the decrease in high-density lipoprotein-cholesterol (HDL-C) was observed in salt-loaded rats as compared with control. Both supplementation and treatment (nifedipine) lowered the blood pressure but the only supplementation lowered the body weight. Supplementation with nutraceutical resulted in significant (P < 0.001) decrease in the serum blood glucose, lipid profile, malonyldialdehyde (MDA), insulin levels, insulin resistance, and increased HDL-C and antioxidant indices. The percentage protection against atherogenesis was 76.5±2.13%. There is strong positive correlation between blood pressure, body weight and serum blood glucose, lipid profile, markers of oxidative stress and strong negative correlation with HDL-C and antioxidant status. The results suggest that the nutraceuticals are useful in reversing most of the component of metabolic syndrome and might be beneficial in the treatment of patients with metabolic syndrome.

Keywords: metabolic syndrome, hypertension, diabetes mallitus, obesity

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822 Rice Tablet Poisoning in Iran

Authors: Somayeh Khanjani, Samaneh Nabavi, Shirin Jalili

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Aluminum phosphide (ALP) is an inorganic phosphide used to control insects and is a highly effective insecticide and rodenticide used frequently to protect stored grain. Acute poisoning with this compound is common in some countries including India and Iran, and is a serious health problem. In Iran it was known as "rice tablet", for its use to preserve rice. Two kinds of rice tablets one being herbal while other containing 3g aluminum phosphide (AlP) are available for use in Iranian households to protect stored food grains from pests and rodents. The toxicity of Aluminum phosphide is attributed to the liberation of phosphine gas in contact with water or weak acid and is the major cause of poisoning and deaths. Rice tablet (Aluminum Phosphid) poisoning may be associated with serious and sometimes incurable complications. In 61.3% of patients were shown uniform ingestion. Vomiting was the most common symptoms reported by 96.4% patients. Agitation was reported in 36.9% and felling of thirsty in 27.9 %. Although many complications such as Hypotension, Adult Respiratory Distress Syndrome (ARDS), Acute Renal Failure (ARF) AND Multi Organ Failure (MOF) were the common complications observed in these patients, but the most lethal complication was Cardiac Arrhythmias occurred in 36.9% of cases. Abdominal pain in 31.4% of the patients, nausea in 79.4% of the patients and 41.1% of the patients showed metabolic acidosis. Suicidal intention was the most common cause of poisoning leading to deaths in 18.6% of the patients. Aluminum phosphide can cause either elevation, decrease or no change in electrolytes, bicarbonate and blood glucose level. The possible mechanism for changes in blood glucose levels are complex and depend on the balance of factors which increase its concentration and those which reduce it. AlP poisoning has been postulated to stimulate cortisol which leads to increasing blood level of cortisol, also it may cause stimulation of glucagon, and Adrenaline secretion; in addition, it can inhibit insulin synthesis which may lead to hyperglycemia. Another suggested mechanism of hyperglycemia is rennin activity in some cases, an increase in magnesium level of plasma and that of tissues, and high phosphate level. Although hyperglycemia is most frequent in this poisoning and also is known as a marker of poor prognostic, hypoglycemia in aluminum phosphide poisoning is a rare finding which may be so dangerous. Patients showed sever hypotension and sever acidosis in addition to sever hypoglycemia. The presenting features of AlP intoxication are rapid onset of shock, severe metabolic acidosis, cardiac dysrhythmias and adult respiratory distress syndrome (ARDS).

Keywords: aluminum phosphide (ALP), rice tablet, poisoning, phosphine gas

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821 Lower Limb Oedema in Beckwith-Wiedemann Syndrome

Authors: Mihai-Ionut Firescu, Mark A. P. Carson

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We present a case of inferior vena cava agenesis (IVCA) associated with bilateral deep venous thrombosis (DVT) in a patient with Beckwith-Wiedemann syndrome (BWS). In adult patients with BWS presenting with bilateral lower limb oedema, specific aetiological factors should be considered. These include cardiomyopathy and intraabdominal tumours. Congenital malformations of the IVC, through causing relative venous stasis, can lead to lower limb oedema either directly or indirectly by favouring lower limb venous thromboembolism; however, they are yet to be reported as an associated feature of BWS. Given its life-threatening potential, the prompt initiation of treatment for bilateral DVT is paramount. In BWS patients, however, this can prove more complicated. Due to overgrowth, the above-average birth weight can continue throughout childhood. In this case, the patient’s weight reached 170 kg, impacting on anticoagulation choice, as direct oral anticoagulants have a limited evidence base in patients with a body mass above 120 kg. Furthermore, the presence of IVCA leads to a long-term increased venous thrombosis risk. Therefore, patients with IVCA and bilateral DVT warrant specialist consideration and may benefit from multidisciplinary team management, with hematology and vascular surgery input. Conclusion: Here, we showcased a rare cause for bilateral lower limb oedema, respectively bilateral deep venous thrombosis complicating IVCA in a patient with Beckwith-Wiedemann syndrome. The importance of this case lies in its novelty, as the association between IVC agenesis and BWS has not yet been described. Furthermore, the treatment of DVT in such situations requires special consideration, taking into account the patient’s weight and the presence of a significant, predisposing vascular abnormality.

Keywords: Beckwith-Wiedemann syndrome, bilateral deep venous thrombosis, inferior vena cava agenesis, venous thromboembolism

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820 Application of Latent Class Analysis and Self-Organizing Maps for the Prediction of Treatment Outcomes for Chronic Fatigue Syndrome

Authors: Ben Clapperton, Daniel Stahl, Kimberley Goldsmith, Trudie Chalder

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Chronic fatigue syndrome (CFS) is a condition characterised by chronic disabling fatigue and other symptoms that currently can't be explained by any underlying medical condition. Although clinical trials support the effectiveness of cognitive behaviour therapy (CBT), the success rate for individual patients is modest. Patients vary in their response and little is known which factors predict or moderate treatment outcomes. The aim of the project is to develop a prediction model from baseline characteristics of patients, such as demographics, clinical and psychological variables, which may predict likely treatment outcome and provide guidance for clinical decision making and help clinicians to recommend the best treatment. The project is aimed at identifying subgroups of patients with similar baseline characteristics that are predictive of treatment effects using modern cluster analyses and data mining machine learning algorithms. The characteristics of these groups will then be used to inform the types of individuals who benefit from a specific treatment. In addition, results will provide a better understanding of for whom the treatment works. The suitability of different clustering methods to identify subgroups and their response to different treatments of CFS patients is compared.

Keywords: chronic fatigue syndrome, latent class analysis, prediction modelling, self-organizing maps

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819 Effect of Lifestyle Modification for Two Years on Obesity and Metabolic Syndrome Components in Elementary Students: A Community-Based Trial

Authors: Bita Rabbani, Hossein Chiti, Faranak Sharifi, Saeedeh Mazloomzadeh

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Background: Lifestyle modifications, especially improving nutritional patterns and increasing physical activity, are the most important factors in preventing obesity and metabolic syndrome in children and adolescents. For this purpose, the following interventional study was designed to investigate the effects of educational programs for students, as well as changes in diet and physical activity, on obesity and components of the metabolic syndrome. Methods: This study is part of an interventional research project (elementary school) conducted on all students of Sama schools in Zanjan and Abhar in three levels of elementary, middle, and high school, including 1000 individuals in Zanjan (intervention group) and 1000 individuals (control group) in Abhar in 2011. Interventions were based on educating students, teachers, and parents, changes in food services, and physical activity. We primarily measured anthropometric indices, fasting blood sugar, lipid profiles, and blood pressure and completed standard nutrition and physical activity questionnaires. Also, blood insulin levels were randomly measured in a number of students. Data analysis was done by SPSS software version 16.0. Results: Overall, 589 individuals (252 male, 337 female) entered the case group, and 803 individuals (344 male, 459 female) entered the control group. After two years of intervention, mean waist circumference (63.8 ± 10.9) and diastolic BP (63.8 ± 10.4) were significantly lower; however, mean systolic BP (10.1.0 ± 12.5), food score (25.0 ± 5.0) and drinking score (12.1 ± 2.3) were higher in the intervention group (p<0.001). Comparing components of metabolic syndrome between the second year and at time of recruitment within the intervention group showed that although number of overweight/obese individuals, individuals with hypertriglyceridemia and high LDL increased, abdominal obesity, high BP, hyperglycemia, and insulin resistance decreased (p<0.001). On the other hand, in the control group, number of individuals with high BP increased significantly. Conclusion: The prevalence of abdominal obesity and hypertension, which are two major components of metabolic syndrome, are much higher in our study than in other regions of country. However, interventions for modification of diet and increase in physical activity are effective in lowering their prevalence.

Keywords: metabolic syndrome, obesity, life style, nutrition, hypertension

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818 Somatosensory-Evoked Blink Reflex in Peripheral Facial Palsy

Authors: Sarah Sayed El- Tawab, Emmanuel Kamal Azix Saba

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Objectives: Somatosensory blink reflex (SBR) is an eye blink response obtained from electrical stimulation of peripheral nerves or skin area of the body. It has been studied in various neurological diseases as well as among healthy subjects in different population. We designed this study to detect SBR positivity in patients with facial palsy and patients with post facial syndrome, to relate the facial palsy severity and the presence of SBR, and to associate between trigeminal BR changes and SBR positivity in peripheral facial palsy patients. Methods: 50 patients with peripheral facial palsy and post-facial syndrome 31 age and gender matched healthy volunteers were enrolled to this study. Facial motor conduction studies, trigeminal BR, and SBR were studied in all. Results: SBR was elicited in 67.7% of normal subjects, in 68% of PFS group, and in 32% of PFP group. On the non-paralytic side SBR was found in 28% by paralyzed side stimulation and in 24% by healthy side stimulation among PFP patients. For PFS group SBR was found on the non- paralytic side in 48%. Bilateral SBR elicitability was higher than its unilateral elicitability. Conclusion: Increased brainstem interneurons excitability is not essential to generate SBR. The hypothetical sensory-motor gating mechanism is responsible for SBR generation.

Keywords: somatosensory evoked blink reflex, post facial syndrome, blink reflex, enchanced gain

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817 Kinetics of Acetaminophen Based Oscillatory Chemical Reaction with and without Ferroin as Catalyst: An Inorganic Prototype Model for Paracetamol-Ethanol Syndrome

Authors: Nadeem Bashir, Ghulam Mustafa Peerzada

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The present study pertains to the nonlinear behavior of acetaminophen based uncatalyzed Belousov-Zhabotinsky (BZ) oscillator and its dynamics in the presence of Ferroin as the catalyst. The role of free metal ions as catalysts was examined and the results compared with corresponding complexed catalysts. Free metal ions were found to be sluggish with respect to the evolution of the oscillatory regime as compared to complexed ones. Effect of change of the ligand moiety of the catalyst complex on the oscillatory parameters was monitored. Since ethanol potentiates the hepatotoxicity caused by acetaminophen in-vivo, it is thought to understand this interaction by virtue of causing perturbation of the acetaminophen based oscillator with different concentrations of the ethanol with and without ferroin as the catalyst. Another dimension to the ethanol effect was added by perturbation of the system with ethanol at different stages of the reaction so as to get an idea whether it is acetaminophen or some reactive intermediate generated in the reaction system which reacts with ethanol. Further, the ferroin-catalyzed oscillator is taken as a prototype inorganic model of the acetaminophen-ethanol syndrome, as ferroin and HOBr were inorganic replacements to Cyt P450 and NADPH in the alcohol metabolism.

Keywords: Belousov-Zhabotinsky reaction, ferroin, Paracetamol-Ethanol syndrome, kinetics

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816 Intracranial Hypertension without CVST in Apla Syndrome: An Unique Association

Authors: Camelia Porey, Binaya Kumar Jaiswal

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BACKGROUND: Antiphospholipid antibody (APLA) syndrome is an autoimmune disorder predisposing to thrombotic complications affecting CNS either by arterial vasooclusion or venous thrombosis. Cerebral venous sinus thrombosis (CVST) secondarily causes raised intracranial pressure (ICP). However, intracranial hypertension without evidence of CVST is a rare entity. Here we present two cases of elevated ICP with absence of identifiable CVST. CASE SUMMARY: Case 1, 28-year female had a 2 months history of holocranial headache followed by bilateral painless vision loss reaching lack of light perception over 20 days. CSF opening pressure was elevated. Fundoscopy showed bilateral grade 4 papilledema. MRI revealed a partially empty sella with bilateral optic nerve tortuosity. Idiopathic intracranial hypertension (IIH) was diagnosed. With acetazolamide, there was complete resolution of the clinical and radiological abnormalities. 5 months later she presented with acute onset right-sided hemiparesis. MRI was suggestive of acute left MCA infarct.MR venogram was normal. APLA came positive with high titres of Anticardiolipin and Beta 2 glycoprotein both IgG and IgM. Case 2, 23-year female, presented with headache and diplopia of 2 months duration. CSF pressure was elevated and Grade 3 papilledema was seen. MRI showed bilateral optic nerve hyperintensities with nerve head protrusion with normal MRV. APLA profile showed elevated beta 2 glycoprotein IgG and IgA. CONCLUSION: This is an important non thrombotic complication of APLA syndrome and requires further large-scale study for insight into the pathogenesis and early recognition to avoid future complications.

Keywords: APLA syndrome, idiopathic intracranial hypertension, MR venogram, papilledema

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815 Autoimmune Diseases Associated with Primary Biliary Cirrhosis: A Retrospective Study of 51 Patients

Authors: Soumaya Mrabet, Imen Akkari, Amira Atig, Elhem Ben Jazia

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Introduction: Primary biliary cirrhosis (PBC) is a cholestatic cholangitis of unknown etiology. It is frequently associated with autoimmune diseases, which explains their systematic screening. The aim of our study was to determine the prevalence and the type of autoimmune disorders associated with PBC and to assess their impact on the prognosis of the disease. Material and methods: It is a retrospective study over a period of 16 years (2000-2015) including all patients followed for PBC. In all these patients we have systematically researched: dysthyroidism (thyroid balance, antithyroid autoantibodies), type 1 diabetes, dry syndrome (ophthalmologic examination, Schirmer test and lip biopsy in case of Presence of suggestive clinical signs), celiac disease(celiac disease serology and duodenal biopsies) and dermatological involvement (clinical examination). Results: Fifty-one patients (50 women and one men) followed for PBC were collected. The Mean age was 54 years (37-77 years). Among these patients, 30 patients(58.8%) had at least one autoimmune disease associated with PBC. The discovery of these autoimmune diseases preceded the diagnosis of PBC in 8 cases (26.6%) and was concomitant, through systematic screening, in the remaining cases. Autoimmune hepatitis was found in 12 patients (40%), defining thus an overlap syndrome. Other diseases were Hashimoto's thyroiditis (n = 10), dry syndrome (n = 7), Gougerot Sjogren syndrome (n=6), celiac disease (n = 3), insulin-dependent diabetes (n = 1), scleroderma (n = 1), rheumatoid arthritis (n = 1), Biermer Anemia (n=1) and Systemic erythematosus lupus (n=1). The two groups of patients with PBC with or without associated autoimmune disorders were comparable for bilirubin levels, Child-Pugh score, and response to treatment. Conclusion: In our series, the prevalence of autoimmune diseases in PBC was 58.8%. These diseases were dominated by autoimmune hepatitis and Hashimoto's thyroiditis. Even if their association does not seem to alter the prognosis, screening should be systematic in order to institute an early and adequate management.

Keywords: autoimmune diseases, autoimmune hepatitis, primary biliary cirrhosis, prognosis

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814 Computer-Aided Diagnosis of Polycystic Kidney Disease Using ANN

Authors: G. Anjan Babu, G. Sumana, M. Rajasekhar

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Many inherited diseases and non-hereditary disorders are common in the development of renal cystic diseases. Polycystic kidney disease (PKD) is a disorder developed within the kidneys in which grouping of cysts filled with water like fluid. PKD is responsible for 5-10% of end-stage renal failure treated by dialysis or transplantation. New experimental models, application of molecular biology techniques have provided new insights into the pathogenesis of PKD. Researchers are showing keen interest for developing an automated system by applying computer aided techniques for the diagnosis of diseases. In this paper a multi-layered feed forward neural network with one hidden layer is constructed, trained and tested by applying back propagation learning rule for the diagnosis of PKD based on physical symptoms and test results of urinanalysis collected from the individual patients. The data collected from 50 patients are used to train and test the network. Among these samples, 75% of the data used for training and remaining 25% of the data are used for testing purpose. Furthermore, this trained network is used to implement for new samples. The output results in normality and abnormality of the patient.

Keywords: dialysis, hereditary, transplantation, polycystic, pathogenesis

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813 A Systematic Review on Measuring the Physical Activity Level and Pattern in Persons with Chronic Fatigue Syndrome

Authors: Kuni Vergauwen, Ivan P. J. Huijnen, Astrid Depuydt, Jasmine Van Regenmortel, Mira Meeus

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A lower activity level and imbalanced activity pattern are frequently observed in persons with chronic fatigue syndrome (CFS) / myalgic encephalomyelitis (ME) due to debilitating fatigue and post-exertional malaise (PEM). Identification of measurement instruments to evaluate the activity level and pattern is therefore important. The objective is to identify measurement instruments suited to evaluate the activity level and/or pattern in patients with CFS/ME and review their psychometric properties. A systematic literature search was performed in the electronic databases PubMed and Web of Science until 12 October 2016. Articles including relevant measurement instruments were identified and included for further analysis. The psychometric properties of relevant measurement instruments were extracted from the included articles and rated based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. The review was performed and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 49 articles and 15 unique measurement instruments were found, but only three instruments were evaluated in patients with CFS/ME: the Chronic Fatigue Syndrome-Activity Questionnaire (CFS-AQ), Activity Pattern Interview (API) and International Physical Activity Questionnaire-Short Form (IPAQ-SF), three self-report instruments measuring the physical activity level. The IPAQ-SF, CFS-AQ and API are all equally capable of evaluating the physical activity level, but none of the three measurement instruments are optimal to use. No studies about the psychometric properties of activity monitors in patients with CFS/ME were found, although they are often used as the gold standard to measure the physical activity pattern. More research is needed to evaluate the psychometric properties of existing instruments, including the use of activity monitors.

Keywords: chronic fatigue syndrome, data collection, physical activity, psychometrics

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812 Cardiopulmonary Disease in Bipolar Disorder Patient with History of SJS: Evidence Based Case Report

Authors: Zuhrotun Ulya, Muchammad Syamsulhadi, Debree Septiawan

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Patients with bipolar disorder are three times more likely to suffer cardiovascular disorders than the general population, which will influence their level of morbidity and rate of mortality. Bipolar disorder also affects the pulmonary system. The choice of long term-monotherapy and other combinative therapies have clinical impacts on patients. This study investigates the case of a woman who has been suffering from bipolar disorder for 16 years, and who has a history of Steven Johnson Syndrome. At present she is suffering also from cardiovascular and pulmonary disorder. An analysis of the results of this study suggests that there is a relationship between cardiovascular disorder, drug therapies, Steven Johnson Syndrome and mood stabilizer obtained from the PubMed, Cochrane, Medline, and ProQuest (publications between 2005 and 2015). Combination therapy with mood stabilizer is recommended for patients who do not have side effect histories from these drugs. The replacement drugs and combinations may be applied, especially for those with bipolar disorders, and the combination between atypical antipsychotic groups and mood stabilizers is often made. Clinicians, however, should be careful with the patients’ physical and metabolic changes, especially those who have experienced long-term therapy and who showed a history of Steven Johnson Syndrome (for which clinicians probably prescribed one type of medicine).

Keywords: cardiopulmonary disease, bipolar disorder, SJS, therapy

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811 Validating Chronic Kidney Disease-Specific Risk Factors for Cardiovascular Events Using National Data: A Retrospective Cohort Study of the Nationwide Inpatient Sample

Authors: Fidelis E. Uwumiro, Chimaobi O. Nwevo, Favour O. Osemwota, Victory O. Okpujie, Emeka S. Obi, Omamuyovbi F. Nwoagbe, Ejiroghene Tejere, Joycelyn Adjei-Mensah, Christopher N. Ekeh, Charles T. Ogbodo

Abstract:

Several risk factors associated with cardiovascular events have been identified as specific to Chronic Kidney Disease (CKD). This study endeavors to validate these CKD-specific risk factors using up-to-date national-level data, thereby highlighting the crucial significance of confirming the validity and generalizability of findings obtained from previous studies conducted on smaller patient populations. The study utilized the nationwide inpatient sample database to identify adult hospitalizations for CKD from 2016 to 2020, employing validated ICD-10-CM/PCS codes. A comprehensive literature review was conducted to identify both traditional and CKD-specific risk factors associated with cardiovascular events. Risk factors and cardiovascular events were defined using a combination of ICD-10-CM/PCS codes and statistical commands. Only risk factors with specific ICD-10 codes and hospitalizations with complete data were included in the study. Cardiovascular events of interest included cardiac arrhythmias, sudden cardiac death, acute heart failure, and acute coronary syndromes. Univariate and multivariate regression models were employed to evaluate the association between chronic kidney disease-specific risk factors and cardiovascular events while adjusting for the impact of traditional CV risk factors such as old age, hypertension, diabetes, hypercholesterolemia, inactivity, and smoking. A total of 690,375 hospitalizations for CKD were included in the analysis. The study population was predominantly male (375,564, 54.4%) and primarily received care at urban teaching hospitals (512,258, 74.2%). The mean age of the study population was 61 years (SD 0.1), and 86.7% (598,555) had a CCI of 3 or more. At least one traditional risk factor for CV events was present in 84.1% of all hospitalizations (580,605), while 65.4% (451,505) included at least one CKD-specific risk factor for CV events. The incidence of CV events in the study was as follows: acute coronary syndromes (41,422; 6%), sudden cardiac death (13,807; 2%), heart failure (404,560; 58.6%), and cardiac arrhythmias (124,267; 18%). 91.7% (113,912) of all cardiac arrhythmias were atrial fibrillations. Significant odds of cardiovascular events on multivariate analyses included: malnutrition (aOR: 1.09; 95% CI: 1.06–1.13; p<0.001), post-dialytic hypotension (aOR: 1.34; 95% CI: 1.26–1.42; p<0.001), thrombophilia (aOR: 1.46; 95% CI: 1.29–1.65; p<0.001), sleep disorder (aOR: 1.17; 95% CI: 1.09–1.25; p<0.001), and post-renal transplant immunosuppressive therapy (aOR: 1.39; 95% CI: 1.26–1.53; p<0.001). The study validated malnutrition, post-dialytic hypotension, thrombophilia, sleep disorders, and post-renal transplant immunosuppressive therapy, highlighting their association with increased risk for cardiovascular events in CKD patients. No significant association was observed between uremic syndrome, hyperhomocysteinemia, hyperuricemia, hypertriglyceridemia, leptin levels, carnitine deficiency, anemia, and the odds of experiencing cardiovascular events.

Keywords: cardiovascular events, cardiovascular risk factors in CKD, chronic kidney disease, nationwide inpatient sample

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810 Clinical Phenotypic Characterization of the SLC26A4 Mutation in Pendred Syndrome/Nonsyndromic Enlarged Vestibular Aqueduct

Authors: Rong Wang

Abstract:

Objective: The aim is to summarize the Solute Carrier Family 26 Member 4 (SLC26A4) mutations and clinical phenotypic characteristics of patients with Pendred syndrome/nonsyndromic enlarged vestibular aqueduct (PS/NSEVA). Design: A retrospective cohort study for the Chinese population was performed to analyze the hearing test results of 406 patients with PS/NSEVA who had a SLC26A4 mutation and the relationship between inner ear imaging and audiology. Results: There was a significant difference in the mean hearing threshold in patients with biallelic mutations (M2), monoallelic mutations (M1), and nonallelic mutations (M0) and between patients with isolated vestibular aqueduct enlargement (IEVA) and patients with IEVA combined with Mondini malformation. There was no significant difference between patients with different gene mutation types or different sexes or between the width of the vestibular aqueduct (VA) and the mean hearing threshold. The degree of hearing loss was linearly correlated with age. Conclusions: We propose that the presence or absence of SLC26A4 mutation, whether combined with Mondini malformation and patient age, are essential factors affecting the degree of hearing loss in the Chinese population. However, the number and type of mutations, degree of VA expansion, and sex of the patients did not affect the clinical audiological phenotype.

Keywords: hearing loss, Pendred syndrome/nonsyndromic vestibular enlargement of aqueduct, radiologic, SLC26A4

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809 Elevated Creatinine Clearance and Normal Glomerular Filtration Rate in Patients with Systemic Lupus erythematosus

Authors: Stoyanka Vladeva, Elena Kirilova, Nikola Kirilov

Abstract:

Background: The creatinine clearance is a widely used value to estimate the GFR. Increased creatinine clearance is often called hyperfiltration and is usually seen during pregnancy, patients with diabetes mellitus preceding the diabetic nephropathy. It may also occur with large dietary protein intake or with plasma volume expansion. Renal injury in lupus nephritis is known to affect the glomerular, tubulointerstitial, and vascular compartment. However high creatinine clearance has not been found in patients with SLE, Target: Follow-up of creatinine clearance values in patients with systemic lupus erythematosus without history of kidney injury. Material and methods: We observed the creatinine, creatinine clearance, GFR and dipstick protein values of 7 women (with a mean age of 42.71 years) with systemic lupus erythematosus. Patients with active lupus have been monthly tested in the period of 13 months. Creatinine clearance has been estimated by Cockcroft-Gault Equation formula in ml/sec. GFR has been estimated by MDRD formula (The Modification of Diet in renal Disease) in ml/min/1.73 m2. Proteinuria has been defined as present when dipstick protein > 1+.Results: In all patients without history of kidney injury we found elevated creatinine clearance levels, but GFRremained within the reference range. Two of the patients were in remission while the other five patients had clinically and immunologically active Lupus. Three of the patients had a permanent presence of high creatinine clearance levels and proteinuria. Two of the patients had periodically elevated creatinine clearance without proteinuria. These results show that kidney disturbances may be caused by the vascular changes typical for SLE. Glomerular hyperfiltration can be result of focal segmental glomerulosclerosis caused by a reduction in renal mass. Probably lupus nephropathy is preceded not only by glomerular vascular changes, but also by tubular vascular changes. Using only the GFR is not a sufficient method to detect these primary functional disturbances. Conclusion: For early detection of kidney injury in patients with SLE we determined that the follow up of creatinine clearance values could be helpful.

Keywords: systemic Lupus erythematosus, kidney injury, elevated creatinine clearance level, normal glomerular filtration rate

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808 Endoscopic Versus Open Treatment of Carpal Tunnel Syndrome: Postoperative Complications in Patients with Diabetes Mellitus

Authors: Arman Kishan, Mark Haft, Steve Li, Duc Nguyen, Dawn Laporte

Abstract:

Objective: Patients with Type 2 diabetes (T2DM) often face higher postoperative complication rates. Limited data exist on outcomes in T2DM patients undergoing carpal tunnel release (CTR). This study aims to compare complication rates between endoscopic CTR (ECTR) and open CTR (OCTR) in patients with T2DM. Methods: This was a retrospective cohort study using the TriNetX database of 56741 patients with T2DM undergoing ECTR (N= 14,949) or OCTR (N= 41,792). Demographic data, medical comorbidities, and complication rates were analyzed. We used multivariable analysis to identify differences in postoperative complication rates between the two treatment methods in patients with T2DM. Results: Patients with T2DM undergoing ECTR had a significantly lower incidence of 90-day wound infection (p < 0.001), 90-day wound dehiscence (p < 0.001), and nerve injury (p < 0.001) when compared to patients who underwent OCTR. After matching, there was a significantly higher number of T2DM patients undergoing ECTR who had peripheral vascular disease (p = 0.045) and hypertension (p = 0.020) when compared to the OCTR group. These patients also had a lower incidence of fluid and electrolyte disorders (p = 0.002) and chronic blood loss anemia (p = 0.025). Conclusion: ECTR presents a superior choice for T2DM patients undergoing CTR, yielding significantly lower rates of wound infection, wound dehiscence, and nerve injury within 90 days post-surgery—reducing the risk by 31%, 48%, and 59%, respectively. These findings support the adoption of ECTR as the preferred method in this patient population, potentially leading to improved postoperative outcomes.

Keywords: endoscopic treatment of carpal tunnel syndrome, open treatment of carpal tunnel syndrome, carpal tunnel syndrome, postoperative complications in patients with diabetes mellitus

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807 Toxicity of Cymbopogon proximus (Maharaib) Oil Extract to Newzealand Rabbits

Authors: A. B. Amna, M. A. E. Samia, A. K. Hassan

Abstract:

The clinical, pathological, hematological and biological changes in Newzealand rabbits groups given daily oral doses of 0.1,0.25 and 0.5 ml/kg body weight/day of Cpmbopogon proximus oil extract were investigated in an experiment durated for 21 days. Other than the dose co-related mortality rates, the clinical signs were observed daily after dosing to be low appetite and nervous signs including restlessness and increased consciousness. Pulmonary excretion of the oil extract led to bloody spots on the lungs, lymphocyte infiltration, congestion and edema. Renal glumeruli manifested lymphocyte infiltration in addition to shrinkages and easinophilic material in the medulla, if considered with the corticomedullary generalized necrosis and the significant changes in urea, they can explain the renal dysfunction. Hepatic malfunction was manifested by significant changes in serum alkaline phosphatase and aspartate transferases accompanied by the congested, fatty changed livers. The direct physical effect of the extracted oil was detected by the catarrhal inflammation of the intestines.There was no significant haematological change except for the slight changes in RBCs and MCVs in rabbits given the highest dose. Future work for Cpmbopogon proximus oil extract was forwarded and practical implications of the result were highlighted.

Keywords: toxicity, cymbopogon proximus (maharaib), oil extract, Newzealand rabbits

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806 Nutritional Management of Polycystic Ovary Syndrome Using a Mediterranean Diet

Authors: Mohamed Radwan

Abstract:

Introduction: Polycystic ovary syndrome is becoming very common among girls from ages 14 to 21 years old, alongside women in their childbearing period. A combination of menstrual irregularities, overweight or obesity, insulin resistance (type 2 diabetes), and symptoms of virilization are characteristics of these cases. Background: A multidisciplinary team should be involved in managing these cases and may consist of A gynecologist, endocrinologist, nutritionist, and psychologist. The role of nutritionists is crucial in these cases in weight reduction and nutritional management. It is mandatory to present some questions that may help in expanding further our understanding of the nutritional management of this syndrome, such as it is not an easy process to reduce the weight of PCO patients (2) we need to know what suitable nutritional plans for these cases are. Methodology: It is not successful to use one diet plan for all patients or a specific plan, but we need to give tailored plans for each patient. We will summarize the medical, nutritional therapy and weight management in polycystic ovary patients and highlight the best eating plan and dietary composition in the treatment of these women. We will also discuss the role of dieticians in treating polycystic ovary cases and overcoming the challenges these women face. Conclusion: All diet plans would decrease the weight of Polycystic ovary cases as low calory diet, low glycemic index diet, high protein diet, as well as Dash diet, but the most suitable diet plan to improve hormones and lead to spontaneous pregnancy is the Mediterranean diet.

Keywords: obesity, PCO mediteranian diet, dash diet, high protein diet

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805 Common Sports Medicine Injuries in Primary Health Care

Authors: Thuraya Ahmed Hamood Al Shidhani

Abstract:

Sports Medicine injuries are very common in primary health care. It is not necessary related to direct trauma, but it could be because of repetitive stress and overuse injuries. Knowledge of Primary Health care providers about the common sports medicine injuries and when to refer to a specialist is essential. Common sports injuries are muscle strain, joint sprain, bone bruise, Patellofemoral pain syndrome, Anterior cruciate ligament injuries, meniscal injuries, ankle ligaments injuries, concussion, Rotator cuff tendinosis/impingement syndrome, lateral and medial epicondylitis and fractures. Systematic approach is very useful in evaluation of sports injuries. RICE is important in initial management. Physiotherapy is essential for rehabilitation. Definitive Management is dependent on patient’s condition and function.

Keywords: common, sports medicine injuries, primary health care, injuries

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804 Hospital Workers’ Psychological Resilience after 2015 Middle East Respiratory Syndrome Outbreak

Authors: Myoungsoon You, Heejung Son

Abstract:

During a pandemic, hospital workers should protect not only their vulnerable patients but also themselves from the consequences of rapidly spreading infection. However, the evidence on the psychological impact of an outbreak on hospital workers is limited. In this study, we aim to assess hospital workers’ psychological well-being and function at the workplace after an outbreak, by focusing on ‘psychological resilience’. Specifically, the effects of risk appraisal, emotional experience, and coping ability on resilience indicated by the likelihood of post-traumatic syndrome disorder and willingness to work were investigated. Such role and position of each factor were analyzed using a path model, and the result was compared between the healthcare worker and non-healthcare worker groups. In the investigation, 280 hospital workers who experienced the 2015 Middle East Respiratory Syndrome outbreak in South Korea have participated. The result presented, in both groups, the role of the appraisal of risk and coping ability appeared consistent with a previous research, that was, the former interrupted resilience while the latter facilitated it. In addition, the role of emotional experience was highlighted as, in both groups, emotional disruption not only directly associated with low resilience but mediated the effect of perceived risk on resilience. The differences between the groups were also identified, which were, the role of emotional experience and coping ability was more prominent in the non-HCW group in explaining resilience. From the results, implications on how to support hospital personnel during an outbreak in a way to facilitate their resilience after the outbreak were drawn.

Keywords: hospital workers, emotions, infectious disease outbreak, psychological resilience

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803 Revisiting the Surgical Approaches to Decompression in Quadrangular Space Syndrome: A Cadaveric Study

Authors: Sundip Charmode, Simmi Mehra, Sudhir Kushwaha, Shalom Philip, Pratik Amrutiya, Ranjna Jangal

Abstract:

Introduction: Quadrangular space syndrome involves compression of the axillary nerve and posterior circumflex humeral artery and its management in few cases, requires surgical decompression. The current study reviews the surgical approaches used in the decompression of neurovascular structures and presents our reflections and recommendations. Methods: Four human cadavers, in the Department of Anatomy were used for dissection of the Axillae and the Scapular region by the senior residents of the Department of Anatomy and Department of Orthopedics, who dissected quadrangular space in the eight upper limbs, using anterior and posterior surgical approaches. Observations: Posterior approach to identify the quadrangular space and secure its contents was recognized as the easier and much quicker method by both the Anatomy and Orthopedic residents, but it may result in increased postoperative morbidity. Whereas the anterior (Delto-pectoral) approach involves more skill but reduces postoperative morbidity. Conclusions: Anterior (Delto-pectoral) approach with suggested modifications can prove as an effective method in surgical decompression of quadrangular space syndrome. The authors suggest more cadaveric studies to facilitate anatomists and surgeons with the opportunities to practice and evaluate older and newer surgical approaches.

Keywords: surgical approach, anatomical approach, decompression, axillary nerve, quadrangular space

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802 A Rare Entity: Case Report on Anaesthetic Management in Robinow Syndrome

Authors: Vidhi Chandra, Arshpreet Singh Grewal

Abstract:

A five-year-old male child born from non-consanguineous marriage, who presented with complaints of growth retardation and no appreciable increase in the penile size since birth and he was posted for de-gloving of penis with dissection of corpora under anaesthesia. After thorough preoperative evaluation it was revealed that patient had peculiar facial dysmorphism that of Robinow Syndrome, high arched palate, Mallampati grade III, mesomelic limbs, scoliotic spine and short stature. All routine investigation were within normal limit, electrocardiography (ECG) and 2D-Echocardiography (ECHO) were normal. In antero-posterior roentgenogram chest showed butterfly and hemivertebrae at multiple levels. The patient was considered to be ASA II. On the day of surgery after ensuring fasting of 6 hours, patient was taken in operation theatre, all standard ASA monitoring was done with ECG, non-invasive blood pressure, peripheral oxygen saturation (SpO2) and body temperature. The patient was pre-oxygenated with 100% oxygen with anatomical face mask. General anaesthesia was induced with Sevoflurane 1-8%, and airway was secured with an appropriate size supraglottic airway and anaesthesia was maintained with nitrous oxide and oxygen in 1:1 ratio along with sevoflurane 2%. An ultrasound guided caudal block was given owing to the skeletal deformities making it difficult even under USG guidance. Post operatively patient was given supportive care with proper hydration, antibiotics, anti-inflammatory and analgesics. He was discharged the next day and followed up weekly for a month. DISCUSSION Robinow syndrome is genetically inherited as autosomal dominant, autosomal recessive or heterogenous disorder involving tyrosine kinase ROR2 gene located on chromosome 9. It has low incidence with no preponderance for any gender. Though intelligence is normal but developmental delay and mental retardation occurs in 20%cases

Keywords: Robinow Syndrome, dwarfism, paediatric, anaesthesia

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801 Central Palmar Necrosis Following Steroid Injections for the Treatment of Carpal Tunnel Syndrome: A Case Report

Authors: M. Ridwanul Hassan, Samuel George

Abstract:

Aims: Steroid injections are commonly used as a diagnostic tool or an alternative to surgical management of carpal tunnel syndrome (CTS) and are generally safe. Ischaemia is a rare complication with very few cases reported in the literature. Methods: We report a case of a 50-year-old female that presented with a necrotic wound to her left palm one month after a steroid injection into the carpal tunnel. She had a 2-year history of CTS in her left hand that was treated with six previous steroid injections in primary care during this period. The wound evolved from a blister to a necrotic ulcer which led to a painful, hollow defect in the centre of her palm. She did not report any history of trauma, nor did she have any co-morbidities. Clinical photographs were taken. Results: On examination, she had a 0.5 cmx1 cm defect in the palm of her left hand down to aponeurosis. There was purulent discharge in the wound with surrounding erythema but no spreading cellulitis. She had full function of her fingers but was very tender on movements and at rest. She was admitted for intravenous antibiotics and underwent a debridement, washout, and carpal tunnel release the next day. The defect was packed to heal by secondary intention and has now fully healed one month following her operation. Conclusions: This is an extremely rare complication of steroid injections to the carpal tunnel and may have been avoided by earlier referral for surgery rather than treatment using multiple steroid injections.

Keywords: hand surgery, complication, rare, carpal tunnel syndrome

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800 Multi-Disciplinary Rehabilitation in Osmotic Demyelination Syndrome: A Case Report

Authors: Wei Qu, Cassandra Agius, Nikki Varvazovsky, Angela Meade

Abstract:

The goals of the case study are to address the importance of early diagnosis of osmotic demyelination syndrome (ODS) and to analyse the types, duration, and intensities of the rehabilitation program to promote neurological and functional recovery. It can be associated with biphasic course of disease and severe neurological and neuropsychiatric symptoms. Although a few treatment modalities, such as plasmapheresis, immunoglobulin therapy, steroid, and thyrotrophin-releasing hormone, have been suggested, there is no effective treatment for ODS. The overall prognosis of established ODS is generally poor. A high proportion of patients have a severe permanent disability, which has led to social, economic, and emotional burdens to carers and societies. In this case, a 69-year-old retired pensioner with chronic alcoholism was admitted to the hospital with a reduced level of consciousness and tonic-clonic seizure. He had severe hyponatraemia (serum sodium 118 mmol/L) and hypokalemia (serum potassium 2.8 mmol/L). He was treated with anticonvulsants, 150ml 3% hypertonic saline over one hour, and 40 mmol potassium chloride over one hour, and his sodium was increased by 11 mmol/L in the first 24 hours. However, he had worsened neurological symptoms with quadriplegia, dysphagia, anarthria, and confusion, and the radiological features suggested the diagnosis of ODS. He had minimal neurological recovery during the first four weeks of hospital admission. He was treated with seven weeks of a multi-disciplinary intensive rehabilitation program. On discharge, he had made a significant cognitive and functional recovery and could mobilize independently without a walking aid. In conclusion, ODS can still occur despite correcting sodium following the current clinical guidelines. Patients with severe neurological deficits in the context of osmotic demyelination syndrome would benefit from intensive rehabilitation to facilitate their functional improvement and to promote their quality of life.

Keywords: osmotic demyelination syndrome, hyponatremia, central pontine and extrapontine myelinolysis, rehabilitation

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