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Khaliq T.,Pakistan Institute of Medical science PIMS
Journal of the College of Physicians and Surgeons Pakistan

Objective: To estimate the reliability of an objectively structured assessment of technical skills (OSATS) in patients for Endotracheal Intubation (ETI), using task-specific checklists during administration of general anaesthesia. Study Design: Co-relational reliability study. Place and Duration of Study: Department of Anaesthesia and Medical Education, Pakistan Institute of Medical Sciences, Islamabad, from September to November 2010. Methodology: Ten first year residents of the department of anaesthesia who had successfully completed a one-day course of ETI on mannequins and had performed 25 ETI in actual patients under supervision, were asked to perform ETI on anaesthetized patients in the operation theater after consent. The procedure was directly observed by two expert observers (ER1 and ER2) and one inexperienced observer (novice, NR) using a validated task oriented checklist. Each trainee repeated the procedure on another patient and was reassessed using the same checklist. Inter-rater for test and retest, and expert to novice reliabilities along with internal consistency were calculated. Results: The inter-rater reliability was 0.92 in test and was 0.98 in retest (Ebel's calculation). Spearman Rank Correlation Coefficient (SRCC) for ER 1 and NR was 0.76 in test and 0.89 in retest. Cronbach's alpha for internal consistency was 0.93 for first test and 0.97 for retest. Conclusion: The OSATS for ETI in real patients demonstrated excellent inter-rater test reliability, inter-rater retest reliability, expert-novice reliability and internal consistency. This instrument, therefore, proves promising as a tool for competency-based evaluations. Source

Saaiq M.,Pakistan Institute of Medical science PIMS
Journal of Burn Care and Research

To describe the epidemiologic profile of children sustaining electrical burn injuries and assess the outcome in terms of need for amputations, hospitalization, and the associated in-hospital mortality. This case series study was performed over a 5-year period (January 2008 to December 2012) at the Department of Plastic surgery and Burn Care Centre, Pakistan Institute of Medical Sciences (PIMS), Islamabad. All children of either gender up to 15 years of age were included. They were admitted for indoor management with standard burn care protocols. The demographic profile of the child, injury characteristics, and outcome were recorded and the data analyzed statistically. Of 85 children who presented with electrical burn injuries during the study period, 89.41% (n = 76) were males and 10.58% (n = 9) were females. The age ranged from 1 to 15 years with a mean age of 10.47 ± 4.09 years. High-voltage burns were the commonest type (n = 61; 71.76%). There were 35 major limb amputations. The mean hospital stay was 6.78 ± 5.01 days (range 1-25 days). The mortality rate was 2.35%. Male children aged 10 to 15 years are the most frequent victims of electric burn injuries, and high-voltage injuries constitute the commonest form of these injuries. There is associated considerable morbidity due to inevitable major amputations of the limbs among otherwise healthy children. There is dire need for primary prevention. Copyright © 2014 by the American Burn Association 1559-047X/2014. Source

Khan T.N.,National Institute for Biotechnology and Genetic Engineering NIBGE | Klar J.,Uppsala University | Tariq M.,National Institute for Biotechnology and Genetic Engineering NIBGE | Anjum Baig S.,Pakistan Institute of Medical science PIMS | And 4 more authors.
European Journal of Human Genetics

Hereditary spastic paraplegias (HSPs) comprise a heterogeneous group of disorders characterized by progressive spasticity and weakness of the lower limbs. Autosomal dominant and 'pure' forms of HSP account for ∼80% of cases in Western societies of whom 10% carry atlastin-1 (ATL1) gene mutations. We report on a large consanguineous family segregating six members with early onset HSP. The pedigree was compatible with both autosomal dominant and autosomal recessive inheritance. Whole-exome sequencing and segregation analysis revealed a homozygous novel missense variant c.353G>A, p.(Arg118Gln) in ATL1 in all six affected family members. Seven heterozygous carriers, five females and two males, showed no clinical signs of HSP with the exception of sub-clinically reduced vibration sensation in one adult female. Our combined findings show that homozygosity for the ATL1 missense variant remains the only plausible cause of HSP, whereas heterozygous carriers are asymptomatic. This apparent autosomal recessive inheritance adds to the clinical complexity of spastic paraplegia 3A and calls for caution using directed genetic screening in HSP. © 2014 Macmillan Publishers Limited All rights reserved. Source

Iqbal T.,Pakistan Institute of Medical science PIMS | Saaiq M.,Pakistan Institute of Medical science PIMS
Journal of the College of Physicians and Surgeons Pakistan

Objective: To determine the epidemiologic pattern and outcome of childhood burns by finding the presenting features of the burn victims, causes / circumstances of burn injury, involvement of body area, need for hospitalization, duration of hospital stay and mortality. Study Design: Case series. Place and Duration of Study: The Burns Care Centre (BCC), Pakistan Institute of Medical Sciences (PIMS), Islamabad, from January 2008 to June 2010. Methodology: All children of either gender with burn injuries who were managed at the centre after primarily presenting to the study centre were included. Children over 15 years were excluded. Lund and Browder chart was used to calculate the total body surface area (TBSA) burnt. Children with extensive and critical burns, high voltage electric burns and those needing any surgical interventions were admitted for indoor management. Data were recorded on a proforma. Results: A total of 1725 children were included in the study. Out of those, 66.84% (n=1153) were males and 33.15% (n=572) were females. The mean age was 5.04 ± 2.78 years. Majority (67.47%) of children were aged 3-6 years. Scalds were the commonest burns (70.31%). Household environment was the commonest site of acquisition of burns (91.47%). Winter was the most frequent season of sustaining burn injury (63%). The commonest anatomic regions affected were hands / upper limbs (65.68%). Overall the affected mean TBSA was 9.37±9.61 %, while for the hospitalized children it was 27.07 ± 10.84%. Two hundred and ninety seven children (17.21%) were admitted. The mean hospital stay was 15.59±5.61 days. The mortality rate was 9.09% for the hospitalized children and 1.56% for the entire study sample. Conclusion: Male gender, age of 3-6 years and winter season were found to have an increased frequency of childhood burns. Scalds were the commonest type of injuries, and hands/upper limbs were the most frequently affected body parts. There is a need to revisit the health care system and institute focused burn prevention strategies consistent with our local circumstances. Source

Butt Z.,Pakistan Institute of Medical science PIMS | Hyder Q.,Pakistan Institute of Medical science PIMS
Journal of the Pakistan Medical Association

Objective: To determine the role of risk factors, which promote cholelithasis in Hepatic Cirrhosis (HC). Methods: A prospective study was conducted on indoor cases with advanced HC. Outpatients with compensated Chronic Liver Disease were used as control. The subjects with history of cholecystectomy and diabetes mellitus were excluded from the study. Conventional ultrasound was used for the detection of gallstones, ascites and portal hypertension. Sonography also furnished pertinent information about the portal vein diameter, size of the spleen, gallbladder wall thickness and echogenecity of the liver. Results: The number of registered cases was 206: (age: 30-85 years): 121 (58.7%) males and 85 (41.3%) females. Hepatitis C (HCV) was the cause of HC in 187 (90.88%) cases. Of 50 (24.30%) patients with detectable gallstones, 27(54.00%) were males. We observed correlation of several risk factors with cholelithasis in our patients (n=50): advanced age: mean 57.3 ± 9.7 years (100%); prolonged duration of HC: 3.5 years (100%); Child -Pughs' class C: 34 (68%); increased thickness of gallbladder wall: 45 (90%); gross ascites: 39 (78%); splenic enlargement: 17.3cm (100%); increased portal vein diameter: 13.4mm (100%). The results were statistically significant when compared with the control group (p=<0,001). Conclusion: Gallstones tend to occur more frequently in patients with decompensated CLD due to interaction of several risk factors in these patients (JPMA 60:641; 2010). Source

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