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Triple A [Allgrove] syndrome, an autosomal recessive disease is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure with progressive neurological syndrome including central, peripheral and autonomic nervous system impairment, and mild mental retardation. The triple A syndrome gene, designated AAAS, localized on chromosome 12q 13 encodes for a 546 amino acid protein called ALADIN [Alacrimia-Achlasia-Adrenal Insufficiency and Neurologic disorder]. This report relates to two sisters, aged 8 and 12 years, who had vomiting, muscle weakness, alacrimia, excessive fatigue and dysphagia. Abdominal sonography, esophago-gastroduodenoscopy, barium swallow, esophageal manometry, CT scan abdomen and brain, biochemical profiles, as well as neurologic and ophthalmic evaluations were consistent with Allgrove's syndrome. Management consisted of pneumatic balloon dilatation for achalasia and initiation of cortisone therapy with successful resolution of dysphagia and other symptoms
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Objective: Achalasia Cardia is treated by Pneumatic balloon dilatation, Heller's Myotomy and recently, by Peroral Esophagaeal Myotomy. This study reports the efficacy of pneumatic balloon dilatation as a non-surgical motility in achieving relief of dysphagia, clinical improvement and recurrence. Long-term complications were reported
Methods: Eight hundred ninety two adult achalasia patients of both genders were treated from January 1988 till December 2011, with pneumatic balloon [Rigiflex Microvasive] dilatation, under fluoroscopy Barium swallow was obtained prior to and five minutes after dilatation to evaluate for efficacy of dilatation as well as for complications. Patients not responding to 30 mm balloon had repeat dilatation with 35 mm balloon after 8 weeks. All patients were enrolled in regular follow up at one, six months and yearly intervals up to a period of five years. Recurrence was defined as an increase in symptom score at 8 weeks greater than 50% of their baseline value. These patients were treated with 35 mm balloon or referred for surgical intervention
Results: Of 892 patients, follow up was obtained in 50% for 5 years, 9.2% for 4-years, 9.3% for 3-years, 10% for 2-years and 21.5% for 1-year of patients. One patient died after repeat dilatation. Eighty-eight patients were excluded from this analysis [20 died due to non-procedure related causes and another 68 were lost during follow up]. Statistically significant improvement was noted in reduction in height and width of barium column and symptom score coupled with weight gain during follow up. Forty-eight patients were subjected to repeat dilatation with 35 mm balloon, two of these developed post-procedure perforations with one mortality. Three non-responsive patients required surgical laparoscopic myotomy. No carcinoma of esophagus was reported during follow up. One patient post dilatation, developed esophageal bezoar. A single pneumatic dilatation achieved a remission rate of 93% at four years, 90% at three years, 95% at two years and 92% at one year post dilatation
Conclusion: Achalasia of esophagus can be effectively and safely treated with balloon dilatation to achieve adequate short and long-term symptomatic relief with a low complication rate
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Cardia , Dilatation , Deglutition Disorders , Recurrence , Esophageal Sphincter, LowerABSTRACT
Almost a 3[rd] of patients with liver cirrhosis and ascites may develop spontaneous bacterial peritonitis [SBP]. SBP carries a high morbidity and mortality. Hence identifying predisposing factors that may cause a patient with ascites to develop SBP are of great interest. The Model for End-Stage Liver Disease [MELD] score is a measure of mortality risk in patients with end-stage liver disease7. Development of ascites and encephalopathy, two complications of end-stage liver disease that are not used in the MELD score calculation, have generally correlated with higher MELD scores
Aims and methods: The aim of this study was to determine the frequency of higher MELD score in patients of SBP with liver cirrhosis. Eighty-five patients fulfilling the inclusion criteria were selected. An informed consent was taken from all patients. Demographic data including age, sex etc. was obtained. All patients had MELD scoring on the basis of serum creatinine, serum bilirubin and INR. MELD scores were calculated according to the method used by the United Network of Organ Sharing [UNOS]
Results: A total of 85 patients were included in the study. It included 52 [61%] male patients and 33 [39%] female patients. Mean age was 48.88 years [SD +/-8.82], Serology for viral markers showed that 68[80 %] patients were positive for hepatitis C antibodies and 8[9.4%] patients were positive for hepatitis B while 3 [3.5%] patients were positive for both hepatitis B and C. In the remaining 6 patients, 2 [2.35%] patients had alcoholic cirrhosis and in 4 [4.7%] patients, etiology of liver disease could not be ascertained. MELD scores in these patients ranged from 9 to 36. Eight patients [9.41%] with SBP had a MELD score of below 15 while 15 [17.64%] patients had scores between 16 and 20. 32 [37.64%] patients had MELD scores between 21 and 25. 20 [23.52%] patients had scores between 26 and 30 while 10 [11.76%] patients had a MELD score higher than 30. Conclusion: A high MELD score [>16] is noted in a significant number of patients with spontaneous bacterial peritonitis [over 90%]
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Clinical bed side teaching to learn the art and science of reaching an appropriate diagnosis is the most important part of training of medical students. Transition from basic science teaching to the complex world of clinical problem solving dealing with real patients is a difficult task to which some adapt quickly while for others it is a challenging proposition. Clinical faculty needs to focus on facilitating this transition helping medical students adjust and adapt to their new roles as future doctors
Study design: Cross sectional non comparative study
Place and duration of study: Gastroenterology Department, Shaikh Zayed Postgraduate Mediical Institute, Lahore from March to May 2013
Methodology: A total of twenty seven 3[rd] year MBBS students already experienced in history taking and clinical examination techniques were included. Facilitator adopted a constructivist approach with schema activation recalling applied aspects of anatomy, physiology and biochemistry relevant to diagnostic approach to 6 patient scenarios of which one is presented. A 7 item questionnaire recorded responses of students on a 5-point Likert scale
Results: Seventy seven percent medical students reported an improved ability to appreciate the relevance of knowledge gained in basic sciences to bed side teaching. Role of teacher was declared positive by 83%, 85% had improved their ability to correlate basic sciences to clinical inferences. Seventy five percent appreciated that mistakes identified helped them in improving performance with 92% confident of applying clinical reasoning skills obtained in solving future clinical problems
Conclusion: Clinical teaching should be based on the principles of adult learning employing a constructivist approach. Linking previous knowledge of basic sciences to the information obtained from bed side examination is the key to help medical students learn the art and science of medicine and become lifelong self-directed learners
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Hepatic encephalopathy [HE] is one of the common complications of liver cirrhosis. Lactulose is conventionally used for the treatment of hepatic encephalopathy
Aims and methods: The aim of this study was to evaluate the combined efficacy of lactulose plus rifaximin versus lactulose alone in the management of hepatic encephalopathy. 80 patients with HE were given lactulose alone and another 80 were given a combination of lactulose and rifaximin. They were monitored for 7 days for response i.e., reversal of HE
Results: Initially 160 patients with HE, who met inclusion and exclusion criteria, were enrolled in this study [out of 183 screened]. 80 patients were randomized in lactulose plus rifaximin group [group A] while the other 80 were placed in lactulose alone group [group B]
Patients were given respective treatment and were monitored for improvement of HE by using West Haven [WH] criteria. Patients were assessed daily. Patients were monitored till recovery or death for 7 days, whichever occurred first. 62 [77.5%] patients in the rifaximin plus lactulose group showed reversal while 43[53.75%] patients in lactulose only group showed reversal of HE. Mortality was also less in the rifaximin plus lactulose group compared to lactulose alone group 17[21.25%] vs. 33 [41.25%] respectively [P<0.05]
Conclusion: Rifaximin and lactulose have different mechanisms of action and act synergistically in combination and show better response in treating HE. This can result in significant decrease in morbidity and mortality
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Background: Educational developers argue that the way students learn is not just based on individual characteristics, rather it is a dynamic process including personal experiences, teaching methods, assessment protocols and subject matter. We analyzed the reliability of a standard research instrument in elucidating different learner types amongst medical students
Methods: Second year MBBS students from two medical colleges in the Punjab were asked to complete the Approaches and Study Skills Inventory [ASSIST] instrument comprising of four sections. The second section consists of 52 items that assess study approaches in three different scales: Deep, Strategic and Surface Apathetic - against a Likert-type scale. Cronbach's alpha estimation was used to check the reliability of the scales and subscales
Results: The alpha values for the main scales range from 0.57 for the Surface Apathetic Approach, and 0.75 each for Deep and Strategic Approaches, indicating low and high levels of internal consistency respectively. The results for the subscales for our sample ranged from 0.51 to 0.67
Conclusions: Our alpha scores are in congruence with various international research findings, except that of Surface Apathetic Approach score. Further research is required on this variation. Furthermore, this inventory may be used for classifying students on entry into the MBBS program in order to 'adjust' teaching methods and techniques. Further follow up analysis of the same student body is expected to yield valuable research data about medical student learning practices and how they change during their formative undergraduate years
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Background: Obesity acts as a risk factor for many diseases like diabetes mellitus, hypertension, and ischemic heart disease. Recently, it has been found that obesity is also associated with periodontal disease especially in adults and urban population
Objective: The objective of the present study was to assess association between obesity and alveolar bone loss caused by periodontal diseases in urban Pakistani population in 30-40 years age group
Subjects and Methods: 100 patients of established periodontal disease were selected based on Community Periodontal Index [CPI]. Patients were divided into obese and non-obese groups of 50 each. The criterion for diagnosis obesity was based on body mass index [BMI]. The selection of all 100 subjects was done according to exclusion and inclusion criteria which possibly excluded all other factors which might enhance alveolar bone loss except obesity. Then panoramic radiographs of all subjects were taken and alveolar bone loss was measured. Then to rule out the effect of age on alveolar bone loss an age-related alveolar bone score was calculated for all subjects of both; obese and non-obese group
Results: In this study there were 43 males and 57 females with the mean age of 35.10+2.24 and mean alveolar bone score 64.51+8.21. Obese patients had higher Alveolar Bone loss sore than non-obese individuals with an odds ratio of 4.33 [2.43 - 6.57]
Conclusion: In middle aged patients of periodontal disease belonging to urban Pakistani population obesity as assessed by body mass index [BMI] is associated with increased alveolar bone loss
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Objective: to determine the frequency of paraneoplastic manifestations in patients with hepatocellular carcinoma [HCC] presenting at tertiary care center
Design: descriptive study
Place of study: department of Gastroenterology - Hepatology, Shaikh Zayed Federal Post Graduate Medical Institute, Lahore
Patients and Methods: patients of hepatocellular carcinoma were interviewed for symptoms suggestive of paraneoplastic manifestations of HCC. The patients were subsequently examined and investigated. The frequency of paraneoplastic features was determined using SPSS 13.0
Results: of the total 100 patients included, musculoskeletal involvement with joint pain [11%] and muscle tenderness [4%] was noted. Skin lesions were noted in 2% of patients. On investigation 1% patient had erythrocytosis, 3% had thrombocytosis and eosinophilia was noted in 10% of patients. Calcium and cholesterol level were raised in 7% of patients each
Conclusions: paraneoplastic features are seen in significant number of patients with hepato cellular carcinoma
ABSTRACT
About 5 million people are infected with hepatitis B virus and about 10 million people harbor the hepatitis C virus in Pakistan. Management of chronic hepatitis in Pakistan carries substantial social impact like fear of tolerability of the drug due to its side effects and the most commonly its affordability. Interferon in combination with Ribavirin is used for the treatment of chronic hepatitis C patients. There are about 60 preparations are available in Pakistan imported from different countries with claim of bioavailability more than 90% . Bioavailability of interferon has not been studied yet in Pakistan. This study was designed to find out the first dose bioavailability of the three formulations of interferon alpha 2b which are commonly prescribed by using limited sampling strategy to help the physician to select the drug with maximum bioavailability
Aim and objectives: the study was conducted to see the bioavailability of interferon in three formulations of interferon alpha 2b in patients with chronic hepatitis C patients
Methods: this was a Quasi - experimental study including sixty patients of either gender. These patients were divided into three groups at random after giving them first dose of three million units of interferon alpha 2b subcutaneously. Group 1: Uniferon [Getz Pharma Brand] Group 2: Ceron-alfa [Biocare Pharma] Group 3: Anferon [CCL Pharmaceuticals] Blood samples were collected at 00, 08, 20 hours according to limiting sampling strategy
Results: the bioavailability was found to be 70% , 60% and 55% in group 1, 2, and 3 respectively. The difference was statistically significant [p < 0.5] based on ANOVA and t-test. Almost all patients reported mild interferon side effects [flu-like symptoms, headache]
Conclusions: different formulations have variable bioavailability and there is a strong reason to choose the best drug with maximum bioavailability to give the patient maximum benefit
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Hepatitis C virus infection is one of the major causes of chronic liver diseases throughout the world. In Pakistan, 10 million people are presumed to be infected with this disease. Hepatitis C virus has been characterized by having a higher rate of spontaneous mutation that leads to a marked degree of heterogeneity among its genotypes. HCV genotype plays an important role in the management of chronic hepatitis C. Knowing the genotype helps to decide about the duration, as well as to predict the response to treatment. But it is an expensive test, and not affordable for majority of patients. HCV RNA by PCR testing is advisable at various stages during the therapy. Early viral response [EVR] is to check the HCV RNA by PCR at 12 weeks, End of treatment response [ETR] is to do the testing at the end of treatment, while Sustained viral Response [SVR] means HCV RNA by PCR testing six months after completion of therapy. All these tests i.e. HCV genotype, EVR, ETR and SVR were checked in these patients
Aims and Objectives: the primary objective is to evaluate the proper utilization of Government funded program for the treatment of hepatitis C. Also it was aimed to determine the type of HCV genotypes as well as subtype in chronic hepatitis C patients, to check EVR, ETR and SVR in these patients, and to evaluate the cost effectiveness of these tests. A total of 1000 patients of hepatitis C virus were recruited
Results: out of 1000 patients, 506 [50.6%] were males, while 494 [49.4%] were females. The age ranged from 16 to 67 years with a predominantly larger proportion of younger patients. HCV genotype was checked in 295 patients Genotypes 1, 2, 3, mixed and untypeable were found in these patients. The predominant genotype was 3 [84%] and its subtype 3a [71%]. Early Viral Response [EVR] was checked in 142 [14.2%] patients; it was achieved in 97 patients. End of treatment response [ETR] was checked in 609 [60.9%] patients, it was achieved in 405 [66.50]. HCV RNA reports to confirm the SVR were available for only 60 [6%] of patients. Out of these 60, SVR was achieved in 46 [76.66%]
Conclusion: genotypes 2 and 3 were detected in almost 90% , while other types collectively detected in rest 10% of screened patients. Although 61% patients reported with HCV RNA reports at the end of treatment, but only 6% patients opted for follow up HCV RNA by PCR testing to look for SVR. Considering the huge cost of treatment of from government funds, measures should be adopted to have a structured program for proper evaluation of these patients before, during and after the treatment. Because of its financial implications, genotype testing is not advisable for naïve patients of chronic hepatitis C
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Hepatitis B and C have become major public health problems worldwide. Estimates of prevalence of these diseases in Pakistan range from 5-15% . This places a huge financial burden on the available health resources. Currently available drugs for the treatment of hepatitis are very expensive and achieve eradication of the disease in around 50% of patients. The best strategy for controlling the menace of hepatitis and its complications lies in prevention. Any such plan should focus on public education. Considerable myths and misconceptions abound in the general public about hepatitis especially about the mode of spread and preventive measures
Aims and Objectives: the present study was undertaken to evaluate the knowledge, attitudes and practices regarding hepatitis in a group of pregnant females attending an ante natal clinic
Results: results of this study have demonstrated that the knowledge about hepatitis is not adequate. Almost half of the subjects believed that hepatitis B and C could spread through food and water. Forty percent of the respondents never insisted on sterilization of dental or barber instruments even when they knew that these were important sources of spread of hepatitis. 55% of the respondents were of the opinion that hepatitis B and C was an absolute contraindication to breast feeding. 77% knew that hepatitis B could be prevented by vaccination but only 67% were actually vaccinated. Almost 50% of the respondents believed that vaccination against hepatitis B also prevented hepatitis C. Hepatitis was considered a social stigma by 40% of the respondents
Conclusions: over all the results of this study suggest that more attention needs to be given to providing correct health education to the public. Misconceptions and myths about hepatitis can only be dispelled by adopting a rational health education strategy
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Extraskeletal or soft-tissue osteosarcoma is rare, occurring approximately as 1.2% of all soft-tissue sarcomas and 4% of all osteosarcomas. The peak occurs in patients, which are in the 6th decade of life. The males are slightly more frequently affected. Symptoms often include a slowly growing painful mass, with a history of trauma [1 2% of cases]. Common sites of involvement are the deep soft tissues of the thigh [47% of cases], upper extremity [20%], and retropcritoneum [17%]. The identification of neoplastic osteoid matrix formation and aggressive characteristics are necessary for histological diagnosis of osteosarcoma. We are going to discuss 06 cases of extraskeletal osteosarcomas including primary renal, primary hepatic osteosarcoma, one arising in a cystosarcoma phylloides and 03 cases involving the thigh regions
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Objective: Objective of study was to observe differences of tooth loss prevalence in males and females with cardiac diseases
Introduction: Poor oral health, tooth loss and periodontal diseases have been reported to be associated with coronary heart diseases. Studies report gender differences in prevalence of cardiac diseases and tooth loss. This paper presents gender differences of tooth loss in cardiac patients of a cardiac hospital of Lahore, Pakistan
Methodology: Age matched Cardiac males and females attending OPD of the Punjab Institute of Cardiology [PIC], Lahore were included in the study. Personal and health-related information were questioned and noted. Oral examination was performed for recording of missing teeth
Results: 1200 CHD study subjects with age-range of 30 to 80 years were enrolled for study. 1045 age matched, 766 [73.30%] males and 279 [26.70%] females were examined for tooth loss. 852 [81.53%] genders had at least one tooth missing. 599 [78.19] males and 253 [90.68%] females were observed with mean tooth loss of 7.5 [SD 8.720] and 11.15 [SD 10.375] respectively. Statistical association among them was also significant [P= 0.000] with OR of 2.339. More subjects [76.29%] showed a loss of 1-15 teeth that was also significant among males and females. Tooth loss was significant in age groups of 41-50 years [P=0.001] and 51-60 years [P=0.000]
Conclusion: Gender differences of tooth loss prevalence among CHD subjects were significant with more risk for males as compared to females
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Objective: To study the demographic, endoscopic and histological features of patients with carcinoma stomach presenting in the Endoscopy suite at the Shaikh Zayed Hospital
Study Design: Cross sectional type of descriptive study
Place of study: Study was carried out at Department of Gastroenterology and Hepatology Shaikh Zayed Post-Graduate Medical Institute Lahore from November 2005 to March 2006
Method and Material: All patients diagnosed to have growth, ulcer or infiltrating lesion in stomach on upper gastrointestinal endoscopy were included from the record of the last eighteen years. Patients were divided in two groups depending on the time of endoscopy, each group comprising of endoscopies performed over nine years time. Both groups were compared using SPSS 11.1
Results: Total number of patients included was 267, male to female ratio was 1.42: 1 [158/109]. Mean age of patients was 52.11 [range 17-85 years] with 39% patients below 45 years of age. Predominant gross appearance was polypoidal, seen in 179 [67%] patients while in 151[56%] patients tumor was located in body of stomach, while 7 1[27%] patients had tumor in antrum and 45[17%] in fundus. Time based analysis oftwo groups of patients revealed no significant change in location, gross appearance or histological diagnosis over 18 years in patients presenting at the Shaikh Zayed Hospital
Conclusion: Gastric carcinoma involves younger age group in our population and is mostly located· in proximal two third of stomach. Features of gastric carcinoma have not shown much change over last 18 years in our population
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Chronic viral hepatitis secondary to hepatitis B and C infections with its sequale of cirrhosis and hepatocellular carcinoma has emerged as a modern day epidemic worldwide with huge sections of population being afflicted by these diseases both in underdeveloped and developed countries. More than 10 million people in Pakistan are estimated to suffer from chronic hepatitis B and C. Treatment of chronic viral hepatitis is expensive and effective in only 50% of patients. Urgent measures are therefore needed to curtail the spread of viral hepatitis by focusing on the possible routes of spread including but not limited to unsafe use of therapeutic injections, blood transfusion, surgical interventions, shaving from barbers, tattooing, mother to child transmission and unsafe sexual practices. Barbers and unqualified dental surgeons without adequate sterilization facilities could play an important role in the spread and transmission of hepatitis B and C. A pilot KAP [Knowledge, Attitudes and Practices] Survey of barbers and street dentists from an urban and a rural location in Punjab was carried out in November.2006 to determine the state of knowledge, attitudes and practices of the above two groups regarding risk of transmission of HBV and HCV as well as to evaluate differences in these parameters between the urban and rural population. This study has revealed that while knowledgeable about the transmission of hepatitis, attitudes and practices regarding prevention of transmission by the two groups of professionals were very unsatisfactory. Additionally on all the domains measured in this pilot study, urban professionals did better than their rural counterparts
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Background: in Pakistan cirrhosis has reached alarming proportions due to spread of HCV and HBV viruses. Some of the oral manifestations of cirrhosis have been found to exhibit geographically variability. There is no research available on the oral health of these patients in Pakistan and their specific oral problems are not known
Objectives: this study examined a cohort of cirrhotic patients admitted at Shaikh Zayed hospital Lahore to assess their oral health status and needs
Materials and methods: thirty consecutive patients admitted with diagnosis of cirrhosis were examined for oral health status. Extra oral examination included any visible lesions or abnormalities on the face, head or neck region, lymph node enlargement and TMJ dysfunction. Intra oral examination included measurement of caries [DMFT] and periodontal disease level [CPITN], oral mucosal lesions, prosthetic status and prosthetic needs of the patients. All findings were recorded on the standard WHO oral health survey form
Results: the oral health of this cohort was poor compared to the general population. Xerostomia [20%], papillary atrophy [1 00%], oral Melanosis [90%], petechieal hemaorrhages [26%] and glossitis [53%] were found prevalent in the sample. Two females had oral lichen planus and one of the patients had oral ulceration. DMFT score [Mean=5.7] was not different from national average [Mean=8.02]. Periodontal disease was more severe than general population. Less than 21% of the sample wore prostheses and over 50% needed one. TMJ dysfunction was common but fewer patient complained of the condition that the number of patients with clinical signs
Conclusions: oral health of cirrhotic population is worse than general population and they have significant oral health needs. Further research is required to investigate this relationship. Effective oral health educatior of patients' and physicians' is required to reduce the burden of oral disease thus mproving quality of life for these patients
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Ratio of platelet count and spleen diameter has recently been proposed as predictor of presence of esophageal varices in patients with cirrhosis. This study was carried out to evaluate predictive value of this ratio for presence of varices. One hundred and one patients with established cirrhosis and no history of variceal bleed were included. Data on physical examination, hematological, biochemical, abdominal ultrasound examination and esophagogastroduodenoscopy [EGD] was recorded for all patients. Presence of varices on EGD was correlated with platelet count/spleen diameter ratio. Esophageal varices were seen 1 in 65 patients while 36 patients had no varices. High grade varices were seen in 15 patients and 50 patients had low grade varices. Value of platelet count/spleen diameter ratio was not significantly different among patients with and without varices. Ratio of platelet count/spleen diameter can not be used to predict the presence of esophageal varices