'We need to share our stories': the lives of Pakistanis with intellectual disability and their guardians.

INTRODUCTION: The experiences of Pakistanis with intellectual disabilities (IDs) and their family members have been underexplored empirically. METHOD: The present study sought to address this gap by understanding the lives of five Special Olympics Pakistan athletes and their guardians through PhotoVoice. FINDINGS: Through thematic analysis, we present the primary theme concerning Pakistan's cultural context that provides an empirical exploration of cultural beliefs about intellectual disability, cultural expectations and support received by people with intellectual disabilities and their guardians. DISCUSSION: We discuss implications for research and practice.

Transfusion for sickle cell disease in pregnancy: a single-centre survey.

INTRODUCTION: Sickle cell disease in pregnancy carries a high risk of maternal and fetal adverse outcomes. The use of prophylactic transfusions to reduce the risk of sickle complications is controversial. Current UK standards do not recommend the routine use of transfusion for sickle pregnancy. We examined transfusion episodes during sickle pregnancies in a single centre over an 11-year period. METHODS: We conducted a retrospective observational study of all pregnancies in patients with sickle cell disease who attended the joint obstetric/haematology clinic over an 11-year period. All pregnancies were managed according to a local protocol, which did not recommend routine transfusion. RESULTS: A total of 38 pregnancies (HbSS 22, HbSC 13, Hb S/beta thalassaemia 3) were included, with a mean age at booking of 29 years. A total of 61% of pregnancies required on-demand or emergency transfusion during the course of pregnancy or post-partum. Women requiring a transfusion during pregnancy had a higher mean number of hospital admissions in the previous year (1·11 vs 0·15, P = 0·057), a significantly lower mean steady-state haemoglobin (85·0 vs 99·6 g L-1 , P = 0·003) and a significantly lower mean haemoglobin at the pregnancy booking visit. (86·1 vs 99·5 g L-1 , P = 0·02). CONCLUSION: In sickle pregnancies assigned to standard management in a single centre, a high proportion of women required on-demand transfusion. Possible pre-pregnancy factors predictive of a need for transfusion include lower baseline haemoglobin and number of hospital admissions in the previous 12 months.

Serum Magnesium Level and It's Relation in Predicting Adverse In-Hospital Outcome in Patients with First Attack of Myocardial Infarction.

Acute myocardial infarction (AMI) patients characterize a large proportion of admissions in coronary care unit and their management and risk stratification is of immense importance. Hypomagnesemia is a long-term risk factor for incident of both myocardial infarction and arrhythmia. We assessed whether serum magnesium levels at admission is associated with arrhythmias and in-hospital mortality in patients with acute myocardial infarction (AMI). The aim of the study was to evaluate the prognostic implications of serum magnesium level in patients with acute myocardial infarction. This cross-sectional observational study was conducted in the department of cardiology in Mymensingh Medical College Hospital from October 2017 to March 2019. Total 259 acute myocardial infarction patients were included considering inclusion and exclusion criteria. The sample population was divided into two groups: Group-I: Patients with acute myocardial infarction with serum magnesium ≥1.82mg/dl. Group-II: Patients with acute myocardial infarction with serum magnesium <1.82mg/dl. Serum magnesium level was measured on admission, and the incidence of in-hospital major cardiac events was assessed. In this study mean serum magnesium level of Group-I, Group-II were 2.21±0.14mg/dl, 1.60±0.15mg/dl respectively. It was statistically significant (p<0.05). In-hospital outcomes of the study group revealed that low risk group patients were uneventful outcome during hospitalization period, they had no any complication. In Group-I patient, 9(4.8%) were developed arrhythmias, 26(13.9%) were developed heart failure, 9(4.8%) were developed cardiogenic shock and 3(1.6%) were died and in Group-II patient, 44(61.10%) developed arrhythmias, 9(12.50%) were developed heart failure, 7(9.7%) were developed cardiogenic shock and 12(16.7%) were died out of them which was statistically significant (p<0.05). Mean duration of hospital stay of the study population according serum magnesium level was in Group-I, 4.27±0.68 days, in Group-II, 5.84±1.05 days which was statistically significant (p<0.05). In conclusion patient with serum magnesium level less than 1.82mg/dl increased the risk of in-hospital arrhythmia and death.

Role of High Neutrophil Lymphocyte Ratio as an Independent Predictor of Adverse In-Hospital Outcomes in Patients with First Attack of ST-Elevation Myocardial Infarction Thrombolysed with Streptokinase.

Atherosclerosis is the pathognomic sign of ischaemic heart disease. Inflammation of the coronary artery contributes to the development of atherosclerosis. Neutrophil-to-lymphocyte ratio (NLR) has been reported to predict the risk of CAD and associated events in patients with ST-Segment elevation myocardial infarction (STEMI). This study was done to investigate the role of neutrophil-to-lymphocyte ratio (NLR) in predicting in-hospital adverse cardiac events in patients with STEMI thrombolysed with streptokinase (STK). This cross sectional descriptive type of study was conducted in the Department of Cardiology, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from August, 2017 to October, 2018. The STEMI patients, thrombolysed with STK had blood samples at admission, analyzed for complete blood counts and NLR calculated. They were grouped into two, low and high NLR, taking 4.50 as cut-off value. Chi square test was used to compare rate of adverse events and death in hospital stay. Logistic regression analysis was used to estimate predictive ability of NLR for in-hospital cardiac events. A total of 87 (39.90%) patients had complications. Patients in high NLR group had higher rate of complications (48.3% vs. 22.5%, p<0.001) in hospital than those in low NLR group. Arrhythmias (21.1% vs. 9.9%, p<0.041), heart failure (27.9% vs. 14.1%, p=0.024), cardiogenic shock (16.3% vs. 4.2%, p<0.011), death (6.8% vs. 2.8%, p=0.227), re-infarction /post MI angina (4.1% vs. 0.0% p=0.084) occurred more in high NLR group. Mean NLR was significantly different between Group I and Group II (3.11±0.84 vs. 10.20±6.08, p<0.0001). Multivariate regression analysis showed NLR an independent predictor of in-hospital adverse cardiac events (p<0.0001). High on admission NLR is an independent predictor for in-hospital adverse cardiac events in patients with STEMI thrombolysed with streptokinase.

Status of Plasma Vitamin-D Level in Predicting Adverse In-Hospital Outcome in Patients with First Attack of Acute Myocardial Infarction.

Acute myocardial infarction has many risk factors and etiologies. Different factors are responsible for adverse in-hospital outcome after acute MI. Status of plasma vitamin D level has been found to be a good predictor of future adverse cardiovascular outcomes in patients with acute MI. Plasma vitamin D level has been considered as a potential marker for identifying individuals under risk of CAD and associated events. This study was done to investigate the role of plasma vitamin D level in predicting in-hospital adverse cardiac events in patients with acute MI. This cross sectional descriptive type of study was conducted in the cardiology department of Mymensingh Medical College Hospital, Mymensingh, Bangladesh from October 2017 to March 2019. Total 257 patients of first attack of acute MI were included considering inclusion and exclusion criteria. Fasting blood samples were analyzed for plasma vitamin D level. Sample population were grouped at first into two, normal and low vitamin D level, taking 30ng/ml as cut-off value, low vitamin D level is further subdivided into insufficiency (21-29ng/ml), deficiency (10-20ng/ml) and severe deficiency (<10ng/ml). Adverse in-hospital cardiac outcomes were observed. In-hospital adverse outcomes occurred in 42.9% patients having normal vitamin D level (>30ng/ml), 66.2% of patients having vitamin D insufficiency (21-29ng/ml), 78.2% of patients having vitamin D deficiency (10-20ng/ml) and 94.4% patients having severe vitamin D deficiency (<10ng/ml), which was statistically significant (p<0.05). Heart failure (30.3%, 47.7%, 63.6% and 77.8%, p<0.05), cardiogenic shock (12.6%, 27.7%, 34.5% and 33.3%, p<0.05), Arrhythmias (14.3%, 21.5%, 23.6% and 22.2%, p>0.05), death (2.5%, 0%, 3.6% and 11.1%, p>0.05) occurred more in low vitamin D groups. Mean vitamin D level was significantly different between Group I and Group II (42.59±10.08 vs. 18.64±6.54, p<0.0001). Multivariate regression analysis showed vitamin D is an independent predictor of in-hospital adverse cardiac events (p=0.001). Age (p=0.001) and obesity (p=0.048) were also other predictors of in-hospital adverse cardiac events. Low plasma vitamin D level is an important predictor for in-hospital adverse cardiac events in patients hospitalized with first attack of acute MI.

Relationship of Plasma Vitamin-D Level with Left Ventricular Ejection Fraction in Patients with First Attack of Acute Myocardial Infarction.

It has been widely reported that vitamin D deficiency is associated with Coronary heart disease (CHD), especially acute Myocardial infarction (MI). Many factors are responsible for reduced Left ventricular ejection fraction (LVEF) and acute Left ventricular fraction (LVF) after acute MI. This cross sectional descriptive type of study was conducted in the Cardiology department of Mymensingh Medical College Hospital from October 2017 to March 2019 to investigate the relationship of plasma vitamin D with LVEF in patients with first attack of acute MI. Total 185 patients of first attack of acute MI were included considering inclusion and exclusion criteria. Fasting blood samples were analyzed for plasma vitamin D level. Sample population were grouped at first into two, normal and low vitamin D level, taking 30ng/ml as cut-off value, low vitamin D level is further subdivided into insufficiency (21-29ng/ml), deficiency (10-20ng/ml) and severe deficiency (<10ng/ml). LVEF among the patients was observed. LVEF was found 49.88±8.58% patients having normal vitamin D level (>30ng/ml), 47.60±8.24% of patients having vitamin D insufficiency (21-29ng/ml), 44.38±8.12% of patients having vitamin D deficiency (10-20ng/ml) and 40.61±8.64% patients having severe vitamin D deficiency (<10ng/ml), which was statistically significant (p<0.05). So, low plasma vitamin D level is associated with reduced LVEF in patients hospitalized with first attack of acute MI.

Frequency and Risk Factors of Erectile Dysfunction among Bangladeshi Adult Men with Type 2 Diabetes Mellitus.

Various forms of sexual dysfunction occur in men with diabetes mellitus (DM) including disorders of libido, ejaculatory problems, and erectile dysfunction (ED). This cross sectional study was conducted in a tertiary hospital of Bangladesh from December 2017 to May 2018 to find out the frequency and risk factors of ED in subjects with type 2 DM (T2DM). One hundred fifty (150) consecutive male patients with T2DM attending the Endocrinology outpatient department (OPD) of the hospital during the study period were evaluated for the presence of ED by using the International Index of Erectile Function-5 (IIEF-5) questionnaire; their socio-demographic, anthropometric, and clinical data were also recorded. Glycemic status was assessed by measurement of fasting plasma glucose (FPG) and HbA1c. Morning serum testosterone was measured in all. Among 150 subjects 68(45.3%) had ED; ED was mild in 14.7%, mild to moderate in 18.0%, moderate in 6.0% whereas severe ED was present in 6.7% of the subjects. The subjects with ED had higher mean age, longer duration of DM, higher body mass index (BMI), higher HbA1c, higher FPG, higher serum creatinine, and lower serum testosterone level than those without ED. Study subjects in the higher age group and higher duration of DM had higher frequencies of ED. IIEF-5 score showed significant negative correlation with age, duration of DM, HbA1c, fasting plasma glucose, serum creatinine and significant positive correlation with serum testosterone. In logistic regression analysis, duration of DM and serum testosterone were found be independent predictors of ED. Frequency of ED among Bangladeshi type 2 diabetic males is high; duration of DM and serum testosterone are independent predictors of ED in them.

Prevalence of Serological Markers of Viruses in Patients of Acute Hepatitis.

Infections due to hepatitis A (HAV), hepatitis B (HBV), hepatitis C (HCV) and hepatitis E (HEV) viruses are the major causes of hepatitis and are associated with significant morbidity and mortality in developing countries like Bangladesh. The aim of this study was to assess the distribution pattern of serological markers in patients of acute viral hepatitis. This was a hospital based observational cross sectional study among purposively selected 107 patients admitted with acute viral hepatitis in the Department of Medicine, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from April 2017 to September 2017. Data were collected by face-to-face interview of the patients, clinical assessment and investigations of biochemical and serological parameters using a structured questionnaire. Descriptive analysis was done using the analytic software SPSS version 21.0. The mean age of the patients was 33.35±12.97 years. Majority was male (68.2%), Muslim (87.9%), married (72.9%) and came from urban area (63.6%) with different level of educational qualifications. The prevalence of viral hepatitis is higher in male (68.22%) than female (31.78%). The common clinical presentations were dark coloured urine (100.0%), yellow colouration of the sclera (100.0%), anorexia (90.6%), nausea/vomiting (79.4%) and abdominal pain (68.2%). Of the 107 patients, 51.40% (n=55) had acute viral E hepatitis, 36.40% (n=39) had acute viral B hepatitis, 12.15% (n=13) had acute viral A hepatitis. Mixed infection with both hepatitis E and A viruses was 1.87% (n=2). HEV and HBV are common in relatively older age while HAV is common in relatively younger age to cause acute viral hepatitis. The study revealed a high prevalence of HEV followed by HBV and HAV in the Bangladeshi population suspected of having suffered from acute viral hepatitis.

The Pattern of Serum Calcium, Phosphate and Parathyroid Hormone Levels in Pre-Diagnosed Chronic Kidney Disease Patients.

Chronic kidney disease (CKD) is an international health problem affecting 5-10% of the world population. Patients with chronic kidney disease were known to have higher prevalence of changes in serum calcium, phosphate and parathyroid hormone in Western countries, but data on the impact of CKD on serum calcium, phosphate and parathyroid hormone in our country are scarce. This cross-sectional type of descriptive study was conducted in the Department of Medicine, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from March 2016 to September 2016. The aim of this study is to estimate serum calcium, phosphate and parathyroid hormone levels in chronic kidney disease (CKD) patients for early recognition of abnormalities, understanding of their patho-physiological consequences & planning management strategies to prevent their progression, thus reducing the morbidity & mortality. This study shows Hypocalcemia, hyperphosphataemia and elevated serum PTH levels are noted in later CKD stages & worsen with disease progression.

Prevalence of GERD in Type II Diabetes Mellitus Patients Admitted in A Tertiary Care Hospital of Bangladesh.

Gastroesophageal reflux disease (GERD) is defined as symptoms or complications associated with regurgitation from the stomach and/or the duodenum to the esophagus. Patients with type II diabetes mellitus (DM) were known to have higher prevalence of GERD in the Western countries, but data on the impact of GERD on DM patients in our country are scarce. The aim of this cross-sectional study was to evaluate the presence of GERD in type II DM patients admitted at the Department of Medicine, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from April 2015 to September 2015. A total of 137 type II DM cases were purposively collected. Reflux Disease Questionnaire (RDQ) was used to check the presence of GERD. Patient's characteristics, laboratory data, face-to-face interview data were analyzed. Out of 137 type 2 DM patients 108 were suffering from GERD giving a prevalence rate of 78.8% which is quite high. Pathophysiological factors like age, sex, duration of DM, weight, waist circumference (WC) had no significant difference between GERD-DM and non-GERD-type II DM patients. Only hypertension and frequent eating fatty food were found to have significant differences between the two groups. From the study findings it could be said that the higher rate of GERD in patients with type II DM may be associated with lifestyle factor and some pathophysiological factors like hypertension. Psychiatric factors may also play role in contributing GERD. Further in-depth and large scale studies are necessary in our country in this regard.

pH Dependence of the inhibition of yeast glyoxalase I by porphyrins.

A number of porphyrin derivatives have been found to inhibit yeast glyoxalase I (EC 4.4.1.5) at 25 degrees C, including haemin, protoporphyrin IX, coproporphyrin III, haematoporphyrin, deuteroporphyrin as well as meso-(tetrasubstituted) porphines. Bilirubin and chlorophyllin were also inhibitory, but not cobalamin, adipic, pimelic or suberic acids. Whilst the Ki value for linear competitive inhibition by meso-tetra(4-methylpyridyl)porphine was pH-dependent, analogous Ki values for meso-tetra(4-carboxyphenyl)- and meso-tetra(4-sulphonatophenyl)porphines followed the Henderson-Hasselbalch equation with pKapp values of 7.10 and 6.50, respectively. Protoporphyrin showed similar behaviour (pKapp 7.06) with a deviation at lower pH. The haemin pH profile for Ki showed a maximum at approx. pH 6.5. The redox reaction between haemin and glutathione did not interfere in the inhibition studies. The Ki value for S-(p-bromobenzyl)glutathione was pH-independent. A detailed analysis of porphyrin binding modes was undertaken.

Carboxypeptidase Y from Saccharomyces cerevisiae. Conformational differences reflected in kinetic behaviour in water and deuterium oxide.

The glycoenzyme carboxypeptidase Y (peptidyl--amino-acid hydrolase, EC 3.4.16.1), from baker's yeast (British Fermentation Products Strain, Ng 72), of molecular weight 60 000, had a protein portion closely similar to those in the literature for carboxypeptidase Y isolations from other yeast sources, but was 25.3 wt% carbohydrate (mannose 83.3% by wt., glucosamine 10.3% by wt. with traces of galactose and galactosamine). Circular dichroic spectra indicated that the enzyme lost its beta-structure as the pH was lowered from 8.08 to 4.16. At p2H 8.22 in 2H2O media the conformation of this enzyme was different from that observed at pH 8.08. A tyrosine residue appeared to be perturbed by lowering the pH of the medium. Carboxypeptidase Y was rapidly, and essentially irreversibly, inactivated at low p2H. The pH profile of kcat for the carboxypeptidase Y-catalysed hydrolysis of 4-nitrophenyltrimethylacetate showed two inflections at 45 degrees C: one at pKapp approximately 3.7 insensitive to temperature variation (ascribed to a carboxyl group), and one of pKapp approximately 5.7 markedly temperature-dependent and possibly caused by a histidine residue.

Predicting high blood pressure in pregnancy: a multivariate approach.

OBJECTIVE: To identify predictors of pregnancy-induced hypertension and pre-eclampsia in 212 nulliparous women before 20 weeks' gestation and at approximately 28 weeks' gestation. STUDY DESIGN: A randomized, prospective study in a teaching hospital. We performed standardized measurements of systolic and diastolic arterial blood pressures, body mass index, urinary calcium:creatinine ratio and components of the renin-angiotensin system, including platelet angiotensin II binding site density. Attending clinicians were blinded to the results. Outcome was assessed by one observer at the end of pregnancy. Discriminant function analysis was used to identify significant predictors. RESULTS: Fifty-five women had transient, presumed 'white-coat', systolic hypertension at the time of first pregnancy visit; they were twice as likely to develop pregnancy-induced hypertension and pre-eclampsia and five times more likely to deliver prematurely. Body mass index, platelet angiotensin II binding site density and urinary calcium:creatinine ratio measured before 20 weeks gestation were also significant predictors. At 28 weeks of pregnancy, measurements of the blood pressure were significant predictors (reflecting the near clinical expression of the disease), together with the plasma angiotensinogen concentration. CONCLUSIONS: A single systolic blood pressure reading of 140 mmHg or more before 20 weeks' gestation indicates a higher than normal risk of pregnancy-induced hypertension and pre-eclampsia and premature delivery. Discriminator biochemical variables were also identified at this time, which might allow the more rational use of prophylactic measures.

Assessing the status of intrauterine insemination before forming a medically assisted conception unit.

A survey in 1996 of our female patients suggest that the three commonest causes of infertility were endometriosis, anovulation and idiopathic which comprises of about 70% of all the patients. In the male patients, sperm morphology evaluation by critical criteria showed that abnormal morphology was present in 71% while 87% of all the semen analysis were abnormal. The objective of this study was to assess the status of IUI before proceeding to formulate patient protocols for IVF in a tertiary infertility referral unit. A retrospective study of patients data was done from Jan 1995 to Dec 1996. Ovarian stimulation by clomiphene for anovulatory and idiopathic patients was performed on couples with at least one patent fallopian tube. Ovulation induction was by an intramuscular injection of 5000 i.u of HCG after follicular maturation. IUI was performed approximately 36-40 hours later. A total of 74 couples received 114 treatment cycles producing a total of 9 conceptions. The conception rate of IUI was therefore 7.89% per cycle and 12.16% per couple with the cumulative pregnancy rate of 28.21%. Associated success features of IUI found in this study were the age of the woman and the semen parameters of the husband.

Evaluating the use of metabolite measurement in children receiving treatment with a thiopurine.

BACKGROUND: Clinical response to thiopurine medication is related to the concentration of its metabolites. Proxy measures are traditionally used to assess dose adequacy. We present our experience of using tioguanine (previously known/formerly referred to as thioguanine) metabolite measurements in paediatric patients and evaluate their effect on clinical practice. AIMS: To report our experience of using tioguanine metabolite measurements in paediatric patients and to evaluate their effects on clinical practice. METHODS: The 6-tioguanine nucleotide (6-TGN) and 6-methylmercaptopurine (6-MMP) were measured in children prescribed thiopurine medication for at least 3 months. Data were collected on thiopurine methyl transferase (TPMT) genotype, drug dose, laboratory indices and management changes. Therapeutic 6-TGN levels were defined as 235-400 pmol/8 × 10(8) RBCs. Seventy individuals (30 males) with a median age of 15 years. Underlying diagnoses were 'IBD' (68/70) and two cases of eosinophilic colitis. Sixty-three were treated with azathioprine and seven with mercaptopurine. A total of 103 separate measurements were made. RESULTS: On initial measurement, 68% of patients had 6-TGN levels outside therapeutic levels despite standard thiopurine dosing. Initial 6-TGN levels were significantly higher in patients with TPMT mutations. Toxicity occurred in seven cases. The 6-TGN levels were significantly higher in those with signs of marrow toxicity. The 6-TGN level correlated with WBC, leukocyte count, mean corpuscular volume (MCV) and ΔMCV; however, the ability of each of these to predict therapeutic 6-TGN levels was poor. After initial measurement, management was changed in 25/70 cases (36%). CONCLUSIONS: 6-TGN levels were therapeutic in a minority of those patients who were tested. Proxy measures perform poorly in predicting therapeutic 6-TGN levels. Measuring thiopurine metabolites is useful for dosage adjustment in children, and for the detection of potential toxicity.