Freeman-Sheldon syndrome (FSS) is an extremely rare syndrome with only about 100 cases reported . It was first described by Freeman and Sheldon in 1938 . It is characterized by oropharyngeal abnormalities, contractures of the hands and feet and a characteristic "whistling" facies. We report a male neonate of Freeman Sheldon Syndrome presenting with small mouth (microstomia) and pursed lips, prominent nasolabial folds and bilateral distal arthrogryposis. A brief review of literature has also been added.
Retraction Notice: This paper has been retracted from the journal after receipt of written complains. This journal is determined to promote integrity in research publication. This retraction is in spirit of the same. After formal procedures editor(s) and publisher have retracted this paper on 30th August-2017. Related policy is available here: http://goo.gl/lI77Nn
Introduction: Assessment of expertise in regional anesthesia techniques is traditionally based upon quota fulfillment of procedures during training. Validation of practitioner proficiency in performing procedures in surgical specialties has moved from simple measurement of technical skills to evaluation of global patient outcomes. Complete absence of pain as a result of nerve blockade is the most important clinical endpoint but patient, technical and procedural factors influence results. The purpose of this study was to measure the postoperative pain scores and associated analgesic medication requirements for patients administered sciatic nerve blockade by nurse anesthetists and determine patient or procedural factors that influenced this outcome.
Methods: Either nerve stimulator or ultrasound guided sciatic nerve blockade was administered by nurse anesthetists under the supervision of regional anesthesia faculty. Patient demographic data that was collected included gender, body mass index, surgical procedure, and pre-existing chronic pain with associated opioid use. Patient self-reported pain scores and opioid analgesic dosages in the preoperative, intraoperative, immediate postoperative and 24 hour post procedure intervals were recorded.
Results: 22 nurse anesthetists administered sciatic nerve blockade to 48 patients during a 36 month interval. Transition from a nerve stimulator to ultrasound guided sciatic nerve block technique resulted in lower mean pain scores. Patients reporting chronic opioid use were observed to have elevated perioperative opioid analgesic requirements and pain scores compared to opioid naïve patients.
Conclusion: Effective analgesia is a prime measure for assessing expertise in regional anesthesia and continuous evaluation of this outcome in everyday practice is proposed.
Cardiovascular disease is a major health concern globally. Genetic testing is an attractive tool for cardiovascular disease prediction because it is a low-cost, high-fidelity technology with multiplex capability. Developments in genomic discovery have yielded valuable new candidates in the quest for better biomarkers and novel therapeutic targets. This brief review focuses on recent trends in biomarkers of cardiovascular disease. DNA microarrays, single nucleotide polymorphism chips, linkage analysis, genome-wide association studies, and other strategies have increased our knowledge of metabolic diseases of the heart. This review also examines the potential applications and challenges of using genetic information for predicting cardiovascular disease.
Introduction: Inhaled & systemic steroids are one of the well-documented risks factors for Candida esophagitis. However, the role of gastric acid suppression remains controversial.
Methods: We conducted a retrospective case-control study of 420 patients consisting of 84 cases of Candida esophagitis and 336 matched controls. Our cohort was gathered from subjects evaluated from 2001 to 2012. The diagnosis of Candida esophagitis was based on endoscopic and/or histological criteria.
Results: On univariate analysis, proton pump inhibitors were associated with higher risk (OR = 2.14; 95 % CI: 1.30 to 3.54); steroid use also increased the risk (OR = 3.55; 95% CI: 2.10 to 6.00). Furthermore, concurrent use of proton pump inhibitors & steroids substantially raised this risk (OR = 13.8; 95% CI 5.07 to 37.5), suggesting a synergistic effect. When adjusted for covariates (cancer, chemotherapy/radiation, antibiotic use, hypothyroidism, anemia, chronic liver disease & diabetes), anemia decreased the odds ratio for proton pump inhibitors to 1.67 (95% CI 1.02 to 2.75) and steroids to 1.69 (95% CI to 1.03 to 2.87). Hypothyroidism also substantially reduced the observed risk associated with steroid use. However, neither anemia nor hypothyroidism reduced the odds ratio for combined use of steroids and proton pump inhibitors.
Conclusions: Our data suggests that patients who have been treated with steroids or proton pump inhibitors are at an increased risk for developing Candida esophagitis. Our data also suggests that steroids and proton pump inhibitors act synergistically to greatly increase the likelihood of Candida esophagitis.
Background: Some of the short term morbidities in caesarean section include hemorrhage, post operative fever and endometritis and caesarean is the most common major operation performed worldwide on women, still there is no universally accepted procedure for management of placental delivery after caesarean section, whether to clear the uterine cavity or not for the residual membranes and whether to close the uterine incision by exteriorization or intraperitoneally. Taking all this into consideration this study was aimed at looking for the best method of practice in one of the most common operations in Obstetrics in this era.
Methods: After extraction of the baby the placenta was removed either manually or delivered spontaneously and to wipe the uterine cavity with a mop was left to the decision of the operating surgeon and closure of the uterine incision (intraperitoneal or exteriorization) was done according to the convenience of the surgeon. The following outcomes were studied based upon the above operative techniques- intra operative blood loss & post operative endometritis.
Results: The average blood loss in spontaneous delivery of placenta was 319.30±104.1 ml and in manual removal of placenta it was 346.63±111.7 ml. Uterine incision was closed extraperitoneally in 61 cases with an average blood loss of 291.03±98.9 ml and in 42 cases of intraperitoneal uterine closure the average blood loss was 396.79±91.1 ml with a significant p value of <0.0001.
Conclusion: Spontaneous delivery of placenta along with closing the uterine incision by exteriorizing the uterus has minimal blood loss. By knowing this, caesarean sections, one of the most common operations in obstetric practice can be made as safe as possible.
Aim: Iron deficiency is one of the most common nutritional disorders in the world, and blood donation may cause iron depletion. This study assessed the incidence and stages of iron deficiency in male subjects donating blood in a bleeding bay in Calabar, Nigeria.
Methodology: One hundred and eighty-four male blood donors attending University of Calabar Teaching Hospital (UCTH) bleeding bay were used for the study. The donors were divided into 5 groups namely; control [n = 35; (19.0%)], first time donors [n = 32; (17.4%)], second time donors [n = 35; (19.0%)], third time donors [n = 41; (22.3%)] and forth time donors [n = 41; (22.3%)]. Blood samples were taken from all the donors and their iron–related parameters namely, haemoglobin concentration (Hb), transferrin saturation (TS), serum ferritin level (SF) and serum transferrin receptor level (STfR) was determined as indicators of iron stores.
Results: The prevalence of anaemia 49 (26.6%), iron depletion 60 (32.6%), iron deficiency without anaemia 106 (57.6%) and iron deficiency anaemia 60 (32.6%) was significantly (p<0.05) increased in 184 male blood donors. The percentage value of the various iron deficiency stages was observed to be higher in 2nd, 3rd and 4th timers when compared with control and first time groups. The higher prevalence of iron deficiency may be caused by more frequent and larger volumes of blood donated by 2nd, 3rd and 4th timers when compared to control and first time groups.
Conclusion: It is concluded that, to avoid the differences in the prevalence among these donors, regular supervision of their haematocrit levels and introducing haemoglobin estimation (using autoanalyser) and serum ferritin be made for them before donating or else, 2nd timers and those above second should not be allowed to donate blood in a year. Moreover, adequate iron supplement should be given in order to prevent the development of iron deficiency.
Air pollution is a worldwide problem to millions of people exposed to concentrations of air pollutants above safety standards, including fine particulate matter (PM2.5). In Haifa, Israel, the apparent link between pollution and cancer development is a topic of concern. The study focused on evaluating the metal exposure of children and adults residing in the Haifa area. A high toxic burden to combustion metals such as nickel and potential carcinogens such as mercury was determined.
Samples of hair were collected from people living in and around Haifa between 2007 and 2015. Selected patients were separated into three groups, adult males and females, and children with a median age of 6.6 years. Multiple metal exposure was determined in all groups, with the greatest burden found in children.
Introduction: The relationship of vitamin D and asthma is known but the association between vitamin D and time required to recover from acute asthma exacerbation has not been studied.
Aims: To find an association between serum vitamin D levels and time required to recover from an asthma exacerbation (recovery days) in asthmatic children from age 5 to 15 years. And to find association of other risk factors of asthma on recovery days.
Study Design: It was a cross sectional study.
Place and Duration of Study: Department of Paediatrics, Qatar Hospital Orangi Town and Abbassi Shaheed Hospital, Karachi, between February 2012 to June 2014.
Methodology: We included 99 asthmatic children (60 male and 39 females; age range 5 to 15 years) at the time of an acute asthma exacerbation. Spirometry was done using Vitalograph (alpha) (UK). FEV1/FEVC ratio <80 % was used to confirm acute asthma exacerbation. Serum vitamin D levels were measured by chemiluminescence method using Abbott's ARCHITECT clinical chemistry analyzer. Children were followed up for confirming recovery by spirometry. FEV1, FVC and FEV1/FVC ratio within the reference ranges (The Thoracic Society of Australia and New Zealand and Polgar) were taken as normal.
Results: Applying Poisson regression model it was found out that that there was no association between vitamin D levels and time to recover from asthma (P =.398). 28.6% (n=2) children showed early recovery when recovery days were associated with exposure to home environment (P =0.000).
Conclusions: Despite the fact that 95% of the subjects with asthma had either vitamin D deficiency or insufficiency but there was no significant association between vitamin D levels and the time required to recover from an asthma exacerbation. Further studies are needed to establish the role of vitamin D in asthma.
Background: Stroke is a major cause of mortality and morbidity worldwide and primary prevention, through adequate knowledge of its risk factors and attendant risk factor control/modification, remains the best solution. This study assessed knowledge of stroke risk factors among individuals diagnosed with two of the most common modifiable risk factors of stroke namely hypertension and diabetes.
Methods: A consecutive sample of 200 clients diagnosed with hypertension and/or diabetes was recruited into this cross-sectional survey from a university teaching hospital in northern Nigeria between January and March 2011. Clinical, socio-demographic, and knowledge of stroke risk factors data were obtained through face-to-face interview using researcher-developed questionnaire. Descriptive and inferential statistics were utilized to identify level of knowledge and its associations with socio-demographic and clinical characteristics of the participants.
Results: Mean (SD) age of the participants was 49.69 (16.27) years, with more female participants (55.5%) and those diagnosed with hypertension (40.5%). Majority (85%) of the participants knew at least one risk factor and the most commonly known risk factor was hypertension (80%) while family history of stroke (0.5%) and transient ischaemic attack (0.5%) were the least known risk factors. Only diagnosis (P<0.01) and level of educational attainment (P<0.05) had significant associations with knowledge of stroke risk factors with those diagnosed with diabetes (96.3%) and those who had tertiary education (93.1%) being more knowledgeable. Regarding specific risk factors, 64.3% of those with hypertension and 56.8% of those with diabetes respectively identified their conditions as risk factors of stroke.
Conclusion: Although a substantial majority of participants in the study knew at least one stroke risk factor, knowledge of some specific risk factors was poor while a little above half of the participants identified their conditions as risk factors for stroke. Effective stroke risk factor awareness and educational strategies are therefore required.
Aims: To find out an association between Human Papilloma Virus and IL 6 gene polymorphism in Oral Squamous Cell Carcinoma patients.
Study Design: Cross-sectional study.
Place and Duration of Study: Ziauddin Hospital (Dental OPD), Karachi, Pakistan. In between the period of January 2014 to May 2015.
Methodology: This cross-sectional study consisted of a total of 140 oral squamous cell carcinoma patients of 18 years and above (104 males and 36 females). Detailed questionnaire followed by sample collection from each patient was done. These samples were analyzed by polymerase chain reaction for human papilloma virus and IL 6 gene polymorphism was analyzed through restriction fragment length polymorphism.
Results: Mean age of the patients was 43.5±11.84 years (range 31-40 years). Most of the patients (45; 32.1%) belonged to the Urdu speaking ethnic group. Pan (87; 62.1%) and Gutka (82; 58.6%) were used by most of the patients. (17;12.1%) patients had history of systemic disease (e.g hypertension, diabetes). And (3021.4%) patients had a positive family history of OSCC. The most common site of OSCC was buccal mucosa (86; 61.4%) in these patients. Majority of the patients (77; 55%) had histologically moderately differentiated OSCC, and more than half of these patients (78; 55.7%) had Group B (stage III & IV) of OSCC. (12;8.6%) out of 140 samples tested positive for human papilloma virus gene and the following pattern was observed for IL 6 gene polymorphism, GG: (46.4%), GC (39.3%), CC (14.3%). A positive association was observed for Group B (stages III & IV) of oral squamous cell carcinoma with IL 6 genotypes: GC heterozygote (OR=3.819, 95% CI=1.782-8.183, P=0.001) and CC homozygote (OR=6.833, 95% CI=2.046-22.822, P=0.002), and also a strong positive association was found between human papilloma virus and CC homozygote genotype (OR=21.333, 95% CI=2.318-196.311, P=0.007).
Conclusion: Human papilloma virus association with IL 6 gene polymorphism in oral squamous cell carcinoma patients suggests rapid and aggressive progress of oral carcinogenesis.