Diagnosis and phenotypic assessment of Pakistani Haemophilia B carriers

Objectives: 1: To assess the diagnostic utility of three polymorphisms [DdeI, XmnI and TaqI] and direct sequencing in haemophilia B [HB] carrier detection in Pakistani families. 2: To compare phenotypes of HB carriers with those of healthy females

Methods: The study was conducted from March 2014 till February 2016 at Khyber Medical University Peshawar and National Institute of Blood Diseases, Karachi. Individuals from HB families of Khyber Pakhtunkhwa [KP] and Federally Administered Tribal Areas [FATA] with known F9 mutation in the proband were enrolled into the study. FIX activity [FIX:C] levels were determined in all the participants. Bleeding scores [BS] and complete blood counts were performed in the female participants. Linkage analysis followed by targeted Sanger sequencing was carried out in all the study participants. Heterozygosity rate was determined for each polymorphism. Healthy females and the carrier groups were compared for bleeding phenotypes

Results: A total of 30 males and 48 females from 13 HB families were studied. The polymorphisms had a low heterozygosity rate. Direct sequencing determined the carrier status in all cases. The mean FIX:C was reduced whereas BS was raised in the carriers when compared with healthy females. A significant raise in white blood cells [WBCs] count was observed in the carriers

Conclusion: The three polymorphisms have a low heterozygosity rate in HB families from KP and FATA. Sanger sequencing is conclusive in determining carrier status in all the cases. FIX:C is low and BS is raised in the HB carriers in comparison to that of normal females. The mean WBCs count is significantly higher in the HB carriers than the normal females

Knowledge, attitude and practices regarding counterfeit drugs among nurses of Madina teaching hospital Faisalabad

Objective: To assess the knowledge, attitude and practices among nurses regarding counterfeit drugs.

Methodology: Cross sectional study. The study was conducted in Madina Teaching Hospital which is attached to the University Medical and Dental College Faisalabad. Total number of nurses was 60 and all were included in the study. Only nurses were chosen because of their direct involvement in administering the drug/injection during duty hours and also they are the first to notice any adverse effects of drugs. Data was collected through pretested restrictive form of questionnaire. Written permission from ethical review committee and informed consent from the participants was taken before conducting the survey. All questionnaires were edited before entering in the computer. Once the data had been cleaned as thoroughly as possible, the distribution of each variable was re-examined as a final check. Non-sampling errors like selection bias, non-coverage, non-contact and non-response were tried to minimize

Results: Out of the 59 nurses who responded from a total of 60, 57 [96.6%] had heard of incidences regarding counterfeit drugs [see table I]. 59 [100%] considered them substandard in quality, 33 [55.9%] thought they were expired drugs, while 9 [15.3%] said that they were made from herbs [see table II]. However a majority of 94.9% understood that they were harmful drugs. 100% nurses said they examined the packaging meticulously and checked the expiry date before administrating the drug [see table III]. 98.3% sent any such drugs for laboratory testing [see table IV]. 100% nurses immediately stopped the use of such drug [see table V]. 34 out of the 59 nurses [57.6%] had seen adverse drug reaction in multiple patients. 89.8% said they kept records of such drugs and all of the participants [100%] reported such incidents to the authorities [see table VI]

Conclusion: Majority of the nurses were aware of the counterfeit drugs problem and its effects on the patients. Over all, most of them understood that counterfeit drugs were harmful to the health of the patients and this study further helped to clear their concepts

Outcome of the surgical treatment of bullous lung disease: a prospective study

This study aimed at evaluating the outcome of surgery for bullous lung disease by comparing the preoperative and postoperative subjective dyspnea score, pulmonary function and clinical features. This prospective study was conducted from May 2009 to October 2011, on 54 patients operated for bullous lung disease. Follow-up at 3-6 months consisted of taking a comprehensive history, physical examination, radiological work-up, and evaluation of changes in subjective dyspnea score, arterial blood gas analysis [ABG], and pulmonary function test [PFT]. After comparison with preoperative values, the student's paired t-test was used to calculate the statistical significance. With approximately 21.6 cases per year, the most common underlying lung pathology was primary bullous lung disease, followed by COPD. The most common presenting complaint was spontaneous pneumothorax in tall young adults in their fourth decade of life with a history of smoking. Bullectomy, with or without decortication, was done for all cases. Improvement in mean PaO2 [arterial partial pressure of oxygen], SaO2 [arterial oxygen saturation] and PaCO2 [arterial partial pressure of carbon dioxide] was seen in most cases but was statistically insignificant. Improvement in mean FEV1 [forced expiratory volume in 1st second], FVC [forced vital capacity] and FEV1 / FVC was statistically significant, with FEV1 being the most reliable indicator of postoperative progress. Improvement in subjective dyspnea score was statistically significant and showed an inverse correlation with FEV1. Those with diffuse pulmonary parenchymal involvement had poorer baseline values and less significant postoperative improvement. Complications occurred more commonly in those with diffuse disease. Mortality was seen exclusively in those with diffuse disease. We conclude that surgery is required for bullous lung disease more frequently in our community since we have a high number of young patients with primary bullous lung disease and localized parenchymal involvement and these patients have a good surgical outcome. Potentially fatal complications like pneumothorax and recurrent infections can therefore be prevented in them. Those with underlying diffuse disease and severely decreased FEV1 [especially below 1 L] also benefit from surgery but require careful patient selection

Infiltrating syringomatous adenoma of nipple

Infiltrating syringomatous adenoma of the nipple is a rare, benign lesion with distinct clinical and histological features. Origin from ducts of dermal sweat glands has been postulated. Important differential diagnosis include nipple adenoma, tubular carcinoma and adenosquamous carcinoma. Appropriate local management includes accurate diagnosis and complete excision to avoid local recurrences. This report describes the condition in a 39-year-old parous lady

Measuring the blood pressure, do we really need sleeves rolled up?

Technical issues and techniques are very important while measuring the Blood Pressure. It has been recommended that blood pressure should be measured on bare arm. In Pakistani society especially in females, this procedure becomes difficult to practice due to social and cultural issues. This study is carried out to check that measuring the Blood Pressure with cuff placed over the sleeve or without sleeve [bare arm] makes any difference

Method: two hundred subjects admitted in Civil Hospital Karachi, attending the OPD of CHK and their attendants were examined under standard conditions and Blood Pressure was measured by mercury sphygmomanometer. Three readings were recorded first with cuff placed over sleeves and with cuff placed on bare arm. Mean of systolic and diastolic blood pressure with and without sleeve were analysis on SPSS Version 11.5

Result: difference in means of systolic blood pressure between clothed and unclothed arm was 0.94 mmHg with a standard deviation of 4.32 and difference of means of diastolic blood pressure for the same was 0.58 mmHg with a standard deviation of 3.80. This was clinically insignificant

Conclusion: the difference found in blood pressure with and without sleeve was not significant clinically

Frequency of impaired glucose tolerance in hypertensive patients

Objective: To determine the frequency of impaired glucose tolerance [IGT] in hypertensive patients

Design: Descriptive cross-sectional study

Place: Civil Hospital Karachi

Sample Size: Eighty patients

Duration: July 2001 to July 2002

Patients and Methods: All the hypertensive patients above 30 years of age of both sexes were included in this study. A detailed relevant history, physical examination including BP was carried out and all the base line investigations and Glucose Tolerance Test [GTT] were performed. Data of each patient were entered on a separate perfoma

Results: Eighty hypertensive patients were studied, out of these 46 [57.5%] were females and 34 [42.5%] were males. The ages of patients were between 32-80 years, mean age + SD was 55.725 + 13.364. The maximum patients were in 41-60 years of age [56.25%]. Results of this study showing that the frequency of IGT was 46.2%, prevalence of impaired fasting glucose [IFG] was 8.7% and prevalence of diabetes was 5% in these hypertensive patients

Conclusion: It is concluded hat impaired glucose tolerance is related to hypertension and other clinical and metabolic abnormalities such as obesity, smoking, hyperlipidemia and family history of DM and positive history of intake of diabetogenic antihypertensive drugs such as diuretics and beta-blockers. Hypertensive patients should be screened for glucose tolerance by OGTT on mass level for the prevention of diabetes. It is an easy and cost effective approach

Vaccination against hepatitis B among doctors at a teaching hospital in Lahore

Hepatitis B is still a major cause of morbidity and mortality, but the disease is preventable, specially through awareness of its spread, screening and vaccination. Setting: Study was done at Sir Ganga Ram Hospital/Fatima Jinnah Medical College to examine the vaccination status against Hepatitis B among the doctors from the Departments of Medicine, Surgery, Gynae and Pathology. Material and In this descriptive study, a total of 110 doctors participated. It took into account different aspects like their vaccination status, status of the booster dose, whether they checked their immunologic status before or after vaccination and reason for not being vaccinated. A total of 71.8% of doctors working in the institution were vaccinated. The results showed that the status was better in the female doctors. A total of 81.5% consultants got themselves vaccinated followed by 74.3% house officers and only 63.6% medical officers who were probably most involved with the patients. Regarding the post-vaccination status 87.5% consultants, 7.1% medical officers and none i.e. 0% house officer had their post vaccine antibody titre checked. Among the house officers 66.6% received their booster dose [where applicable] followed by consultants and medical officers. Lack of awareness was mentioned as the biggest reason for not being vaccinated. Awareness through health education regarding Hepatitis B and bringing down the cost through other methods of vaccination is important in this country where the disease is very common