Identification of Variants and Mutational Analyses of Cardiac Myosin-binding Protein C (MYBPC3) Gene of Adult Bangladeshi Patients with Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic hereditary cardiomyopathy characterized by sudden cardiac death. Mutations in the MYBPC3 gene are often the most prevalent genetic abnormality in HCM with a prevalence ranging from 20.0 to 42.0%. The mutation spectrum is available for different countries, but such studies are lacking in the Asian population including Bangladeshi patients. A cross-sectional descriptive study was conducted for mutation analysis of the whole MYBPC3 gene on a cohort of 75 HCM Bengali Bangladeshi probands through Next Generation Sequencing at the Genetic Research Lab of Bangabandhu Sheikh Mujib Medical University from 2016 to 2019. The structural and functional impact of the mutations was further analyzed by in silico process. We analyzed the data and found 103 variants in 102 locations in the MYBPC3 gene. Variants were identified in both the coding region and the non-coding region. We found one possibly novel variant in the MYBPC3 gene. The findings of this research will help to develop a genetic database of HCM which will help in the early diagnosis and proper management of HCM patients in Bangladesh. One pathogenic splice donor variant (47356592 C >T) was found in the intronic region. Among the variants in the coding region, one missense mutation was pathogenic (NP₋000247.2: p.Asp770Asn) which was found in seven patients and another one is of conflicting interpretations of pathogenicity (NP₋000247.2: p.Ser217Gly) which was found in two patients. We have identified one in-frame deletion (NP₋000247.2: p.Ala433del) that is possible a novel variant responsible for the development of HCM.

Formulation of Genetic Counseling Format for Adult Bangladeshi Patients with Acute Myeloid Leukemia.

With the advancement of medical genetics, particular emphasis is given on the genetic counseling worldwide. In Bangladesh, genetic counseling services are not yet developed. Acute myeloid leukemia (AML) is a malignant disease of the myeloid cells of bone marrow. Like other malignant diseases, it may result from a mutation in the DNA. A genetic counseling format will educate the AML patients and provide appropriate medical and emotional support. The aim of this descriptive cross-sectional study was to develop a genetic counseling format for adult Bangladeshi patients with AML. Taking this into account, a draft format was prepared by reviewing relevant documents available online which was later analyzed by an expert panel through a group discussion and thus a proposed format was developed. To make the format effective in the perspective of Bangladeshi population, the proposed format was applied in counseling, and thus a final format was developed in the English language. This format will educate the counselors, clinicians, and patients about the utility and importance of the genetic counseling and genetic tests. Also, the patients feel comfort regarding the whole counseling process and going for postcounseling treatments and advice. Though it is written in English, it may be translated into mother tongue for better communication during counseling.

Topical Tacrolimus versus Hydrocortisone on Atopic Dermatitis in Paediatric Patients: A Randomized Controlled Trial.

Atopic dermatitis (AD) is a chronic, inflammatory skin disease in early childhood. Atopic dermatitis is familial disease, often coexists with other atopic diseases with multiple risk factors associated with atopic eczema. The disease is more frequent in urban areas compared with rural areas. Changes in nutrition and a decrease in infant breast-feeding and respiratory allergies are contributory factors for the condition. A Randomized Controlled Trial (RCT) was carried to compare the efficacy and safety of Tacrolimus ointment with a topical corticosteroid reference therapy. A total 60 patients aged between 2 to 10 years, having atopic dermatitis for at least one year and comply Hanifin-Rajka criteria were selected using random number table and allocated into study and control groups through randomization. Study group was treated with topical Tacrolimus 0.03% twice daily for three weeks, while the control group was treated with 1% Hydrocortisone acetate for the same period. Both groups had a washed out phase for 2 weeks with a follow up period of 6 weeks. Eczema Area and Severity lndex (EASI) was assessed at baseline and three weeks after treatment. Efficacy was evaluated at each visit by six clinical signs of atopic dermatitis through measurement of the affected surface area and the EASI score in each of four body regions. Before intervention, in study group mean EASI score was 11.29 with a SD of 2.14, while in control group it was 11.05 with a SD of 2.46. Difference was statistically insignificant (p>0.05). At the end of the treatment, in study group mean EASI score was 4.86 with a SD of 1.01, while in control group it was 7.97 with a SD of 1.80. Statistically high significant difference was observed between EASI scores of two groups before and after the treatment (p<0.001). After getting treatment with Tacrolimus, median reduction of EASI score was 56.07 in study group, while getting treatment with Hydrocortisone, median reduction of EASI score was 27.16. Difference was highly significant (p<0.001). It is evidenced that Tacrolimus ointment (0.03%) acts as an effective as well as safe non-steroidal topical therapy for the treatment of dermatitis in paediatric patients.

Antibacterial Effect of Curcuma longa (Turmeric) Against Staphylococcus aureus and Escherichia coli.

This observational study was conducted during the period from July 2010 to June 2011 in the Department of Pharmacology in the collaboration of Department of Microbiology, Mymensingh Medical College, Mymensingh to determine the profile of antibacterial effect of Crude Turmeric paste aqueous turmeric extract, and standard antibiotic Amikacin against Staphylococcus aureus and Escherichia coli. Three separate experiments were done e.g. (Expt- I) Inhibitory effect of Crude Turmeric paste incorporated into nutrient agar (NA) media, (Expt- II) Minimum inhibitory concentration of (a) Aqueous Turmeric extract and (b) Amikacin by broth dilution technique and (Expt-III) their subculture study in nutrient agar (NA) media for confirmation of respective results of previous experiments. Inhibitory effects were observed against the growth of Staph Aureus and Esch coli at 10% and 30% respectively of Crude Turmeric paste incorporated into NA media. The broth dilution technique was followed to determine the MIC of Aqueous Turmeric extract and Amikacin. The MIC of Aqueous Turmeric extract was 800 µg/ml against Staph aureus and that against Esch coli was 2000 µg/ml and the MIC of Amikacin was 10 µg/ml for both the bacteria. The MIC of Amikacin was the lowest in comparison to MIC of Aqueous Turmeric extract for complete inhibition of growth of Staph aureus and Esch coli. The subculture study showed similar results with that of previous experiments in terms of inhibitory effects of Crude Turmeric paste and MIC of Aqueous Turmeric extract and Amikacin against all of the organisms studied.

Recent Trends in the Presentation of Neuroanatomy in Contemporary Neuroanatomy Books as Revealed in Their 'Preface's: A Review.

Many changes have been made in the field of Neuroanatomy teaching and assessment. One important way to know the changes in other country is by analyzing the Neuroanatomy text books and we can compare their reflections in our curriculum by analyzing the assessment system. To analyze the 'Preface's of contemporary Neuroanatomy text books, qualitatively, for noting the approaches taken and means applied in dealing with Neuroanatomy in the text books. This review was done in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from July 2008 and June 2009. The 'Preface's of ten contemporary text books dealing with Neuroanatomy, published since 1993, was analyzed. Among them, the two that are most commonly recommended to the medical postgraduates in Bangladesh were selected for analyzing their presentation of text and illustrations. The 'Preface's of ten books were analyzed qualitatively to identify the information and explanations provided and comments made by their author(s)/editor(s) on the approaches taken and the means applied in the books in selecting and presenting topics, text and illustrations. The observations were organized into specific 'theme's. Twelve 'theme's were identified from the analyses of the 'Preface's of ten contemporary Neuroanatomy text books. These include special emphasis in the books on incorporation of new information, on practical application of Neuroanatomical facts. Addition and improvement regarding illustrations are also highlighted. By incorporating the findings of the present study with the present-day ideas and trends in Neuroanatomy in the developed world as evident from the available literature, suggestions could be formulated on improving the methods of teaching and assessment of Neuroanatomy in Bangladesh.

Intermittent prophylaxis of recurrent febrile seizures with clobazam versus diazepam.

Febrile seizures are the most common type of seizure among children that can be prevented by using prophylactic drugs like Clobazam and Diazepam. The present prospective study was conducted in the Department of Pediatrics, Mymensingh Medical College Hospital and Community Based Medical College Hospital, Bangladesh over a period of 1 year from July 2012 to June 2013 to compare the effectiveness of intermittent Clobazam versus Diazepam therapy in preventing the recurrence of febrile seizures and assessed adverse effects of each drug. A total of 65 patients (32 children administered Clobazam and rest 33 children received Diazepam) of simple and complex febrile seizures aged 6 months to 5 years of both sexes were the study population. Data were collected by interview of the patients, clinical examination and laboratory investigations using the research instrument. Data were analyzed by using Chi-square (χ2) Test, Student's 't' Test and Fisher's Exact Test. For all analytical tests, the level of significance was set at 0.05 and p<0.05 was considered significant. The proportion of patients was higher between age 12-36 months and male was predominant in the both Clobazam and Diazepam groups. Over 31% of patients in Clobazam group who experienced episode of fever within 3 months, 40.6% within 6 months and 9.4% within 9 months compared to 36.4% in Diazepam group within 3 months, 45.5% within 6 months & 12.1% within 9 months after discharge from the hospital. Three (9.4%) patients in Clobazam group and 7(21.3%) in Diazepam group who experienced febrile convulsion during the follow up period. From the data adverse effects within 3 and 6 months experienced by the patient's drowsiness, sedation and ataxia were higher in Diazepam group than those in Clobazam group. However, within 9 months lethargy and irritability were somewhat higher in Clobazam group than those in Diazepam group. The mean duration of hospitalization was significantly higher in Diazepam group compared to Clobazam group (6.0±1.0 vs. 4.6±0.08 days, P<0.001). Seven (21.2%) out of 33 children with febrile seizures in Diazepam group had a history of recurrent seizures, whereas 3(9.4%) of 32 children in the Clobazam group. The risks of recurrent febrile seizure in the Diazepam group was 2.6 times greater compared to those in the Clobazam group (P=0.186). The result indicates that Clobazam is safe, efficacious, requires less frequent dosing and has less adverse effects such as drowsiness, sedation, ataxia and irritability as compared to Diazepam. So, Clobazam may be an alternative to Diazepam given intermittently for prevention of recurrent febrile seizures.

Cord blood stem cells - a dream for future medicine.

Umbilical cord blood collected at birth is a rich source of stem cells that can be used to treat diseases of the blood and immune system. Cord blood stem cells which have infinite medical potential are currently used in the treatment of several life-threatening diseases, and play an important role in the treatment of blood and immune system related genetic diseases, cancers, and blood disorders. Transplant recipients are less likely to reject a cord blood stem cell and do not require a perfect match as bone marrow transplants do. Transplantation of umbilical cord blood stem cells represents a major advantage in providing a new source of stem cells to patients in need. Private storage of cord blood stem cells clearly has value, in that it provides future patients and families with potential therapeutic options for transplant. Many recent studies showed that human umbilical cord blood stem cells have the potential to generate cells with neuronal characteristics. Therefore, the umbilical cord blood stem cells can be viewed as the stem cells source of choice for clinical and non-clinical research applications.

Diameter of the lymphoid follicles in the vermiform appendix of Bangladeshi cadaver.

Appendicitis is the most common clinical condition of the appendix. Many cases of acute appendicitis result from obstruction of the lumen of the appendix by lymphoid hyperplasia. The vermiform appendix is a worm-like, closed-ended, narrow, small tubular structure, projecting from posteromedial wall of cecum. It is an integral part of the Gut Associated Lymphoid Tissue (GALT) system. The lymphoid follicles of the vermiform appendix vary in respect to their number, diameter and location in different ages. This study was done to measure and establish the normal diameter with age related variation of the lymphoid follicles of vermiform appendix in Bangladeshi population. This descriptive cross sectional study was carried out in the Department of Anatomy, Sir Salimullah Medical College, Dhaka, from January 2008 to June 2009. Sixty (60) postmortem vermiform appendices of different age groups of Bangladeshi cadavers. For studying the diameter of lymphoid follicles in the vermiform appendix in relation to age, the collected samples were divided into five groups namely Group-A between age ranges 0-20 years, Group-B between age ranges 21-30 years, Group-C between age ranges 31-40 years, Group-D 41-50 years and Group-E age above 50 years. From each age group, six (6) fresh samples were selected for histological study. From each vermiform appendix three slides were prepared each from its base, middle part and near the tip and were stained with routine H & E stain. The normal diameter of the follicles of the vermiform appendix ranged from 0.40 mm to 0.66 mm. The highest average group diameter was 0.62±1.10mm in Group-A and the lowest value was 0.45±2.73mm in Group-E. It was obvious that the diameter decreased gradually with advancing age. The average normal diameter of the lymphoid follicles per histological section of vermiform appendix reduced with advancing age and showed a significant negative correlation with age (p<0.001). In the present study, the normal diameter of the lymphoid follicles of the vermiform appendix reduced significantly with advancing age.

Microscopic impacts of gestational diabetes mellitus on the umbilical cord.

This observational study was done among the different types of diabetes, gestational diabetes mellitus (GDM). In the present study we looked for the microscopic impacts of GDM on the umbilical cord. This study was carried out in the department of Anatomy, Chittagong Medical College from May 2006 to April 2007. Total fifty umbilical cords with placenta were collected from selected subjects within 36 to 40th weeks of gestation and divided them into two groups. Twenty five control group (specimen collected from normal mothers) and 25 GDM group (specimen collected from mothers developing diabetes during pregnancy state). Tissues from a particular area of the umbilical cord were processed for histological study by routine procedure. All the slides were examined under light microscope. In GDM group light microscopy showed rupture and erosion of the endothelium and complete rupture of the umbilical vessels resulting in extravasation of blood within Wharton's jelly. The umbilical vein was grossly dilated and thin walled. Smooth muscles of the umbilical vessel showed disruption and degeneration of its fibers. Wharton's jelly showed alteration in the pattern of distribution and degeneration of its fibers with honeycomb like empty spaces among them. It is suggested that GDM might have some harmful effects on the umbilical vessels and connective tissue component of the Wharton's jelly.

Stature estimation from craniofacial anthropometry in Bangladeshi Garo adult females.

Estimation of stature is an important tool in forensic examination especially in unknown, highly decomposed, fragmentary and mutilated human remains. When the evidences are skeletal remains; forensic anthropology has put forward means to estimate the stature from the skeletal and even from fragmentary bones. Sometimes, craniofacial remains are brought in for forensic and postmortem examination. In such a situation, estimation of stature becomes equally important along with other parameters like age, sex, race, etc. Today, anthropometry plays an important role in industrial design, clothing design, ergonomics and architecture where statistical data about the distribution of body dimensions in the population are used to optimize products. It is well established that a single standard of craniofacial aesthetics is not appropriate for application to diverse racial and ethnic groups. Bangladesh is a country not only for the Bengalis; the country harbours many cultures and people of different races because of the colonial rules of the past regimes. Like other ethnic groups, the Garos (study subjects) have their own set of language, social structure, cultures and economic activities and religious values. In the above context, the present study was attempted to establish ethnic specific anthropometric data for the Bangladeshi Garo adult females. The study also attempted to find out the correlation of the craniofacial dimensions with stature and to determine multiplication factors. The study was an observational, cross-sectional and primarily descriptive in nature with some analytical components. The study was carried out with a total number of one hundred Garo adult females, aged between 25-45 years. Craniofacial dimension such as head circumference, head length, facial height from 'nasion' to 'gnathion', bizygomatic breadth and stature were measured using a measuring tape, spreading caliper, steel plate and steel tape and sliding caliper. The data were then statistically analyzed by computation to find out its normatic value. Study was also aimed to observe the possible correlation of them with the stature. Multiplication factors were estimated for estimating stature and comparison were made between 'estimated' values with the 'measured' values by using 't' test. The mean±SD of the head length, head circumference, facial height from 'nasion' to 'gnathion', bizygomatic breadth were 17.49±0.58cm, 51.56±1.49cm, 10.65±0.40cm, 13.47±0.52cm respectively and The mean±SD of the stature was 152.79±5.62cm. Head circumference showed significant positive correlation with stature (r=0.278 and p= 0.005) but head length (r= -0.029 and p=0.774), bizygomatic breadth (r= 0.143 & p=0.156) and facial height from 'nasion' to 'gnathion' (r=00.023 and p=0.818) did not reach statistically significant level with stature. No significant difference was found between the 'measured' and 'estimated' stature for head circumference, head length, bizygomatic breadth and facial height from 'nasion' to 'gnathion'.

Achieving Millennium Development Goals 4 and 5 in Bangladesh.

Bangladesh has made commendable progress in achieving Millennium Development Goals (MDGs) 4 and 5. Since 1990, there has been a remarkable reduction in maternal and child mortality, with an estimated 57% reduction in child mortality and 66% in maternal mortality. This review highlights that, whereas Bangladesh is on track for achieving MDG 4 and 5A, progress in universal access to reproductive health (5B) is not yet at the required pace to achieve the targets set for 2015. In addition, Bangladesh needs to further enhance activities to improve newborn health and promote skilled attendance at birth.

Effect of fenugreek seeds on the fasting blood glucose level in the streptozotocin induced diabetic rats.

In this experiment defatted Trigonella foenumgraecum (fenugreek seeds/methi seeds) has used as the antidiabetogenic herbal medicine. The experiment was carried out in Bangabandhu Sheikh Mujib Medical University and BIRDEM from 1996 to 1998 on a total of 58 Long Evans rats of either sex. They were 50-60 days young rats with average body weight 72-174 gm. Among the total, 10 rats were treated with only vehicle called as non-diabetic control rats, 48 rats were treated with Streptozotocin (STZ) at a dose of 90 mg in 1 ml of citrate buffer solution per kg body weight, among which 20 were diabetics. Ten (1 died, 1 escaped) diabetic rats were again treated with fenugreek called as Fenugreek-treated diabetic rats and the rest 10 diabetic rats were called as diabetic control rats. The change in the mean fasting blood glucose (FBG) level in different groups of rat from day 5 from streptozotocin injection were higher in diabetic control group and in fenugreek-treated diabetic group than in non diabetic control group. The FBG level on day 13 the mean in non-diabetic control group was 5.21 mmol/L. In diabetic control group and in fenugreek-treated diabetic group the mean FBG level were 24.33 mmol/L and 9.89 mmol/L respectively. So, from this experiment it may be concluded that fenugreek decreases the FBG level considerably by improving diabetes mellitus.

Use of Wigglesworth classification for the assessment of perinatal mortality in Bangladesh--a preliminary study.

The Wigglesworth pathophysiological classification was used to analyse perinatal deaths occurring in 5 health centres in Bangladesh. The aims were to assess the feasibility of this classification, to determine the causes of perinatal deaths and thereby to identify the areas in need of intervention. A total of 8058 births were recorded at 5 centres during the period of 11 months from mid-January to mid-December 2001. There were 1069 deaths in the perinatal period. Stillbirths were slightly more frequent (53.5%) than early neonatal deaths (46.5%). Among the stillbirths, fresh stillbirths predominated over normally formed macerated ones at all centers except BIRDEM, where the majority (52.5%) was macerated. The majority (71.6%) of perinatal deaths were in the groups comprising asphyxial conditions (46.8%), conditions associated with immaturity (13.3%), and normally formed macerated stillbirths (NFMSB, 11.5%). In the group, 'other specific conditions' which was responsible for 9.3% of perinatal deaths, all but one case was attributed to sepsis. When the cases were subdivided by birth groups, asphyxia predominated in all but the <1000g group, in whom immaturity was responsible. Conditions associated with immaturity were second highest in number. The majority of the perinatal deaths (83.4%) was in babies less than 2500g. The study has shown that the Wigglesworth classification can be used in different types of health facilities in Bangladesh by doctors, nurses and midwives. The areas which need intervention are antepartum care, obstetric and newborn care practices, and environmental factors responsible for the high prevalence of prematurity and low birth weight.

Nutritional status and birth outcomes of the diabetic and non-diabetic pregnant women.

This cross sectional study compares the nutritional status and birth outcomes of 357 diabetic and non-diabetic pregnant women (203 DM and 154 NDM as control). Uncomplicated diabetic and non-diabetic pregnant women of singleton pregnancies with age range of 19-35 years were enrolled at term in BIRDEM hospital. Maternal anthropometry and neonatal anthropometric measurements were taken following standard techniques. Educational level was significantly different between the groups. The diabetic mothers were found significantly less educated (p<0.0001) compared to non-diabetic mothers. Highly significant differences were observed between the groups on mean maternal age, weight at term, height, body mass index (BMI), mid arm circumference (MAC), and hemoglobin concentration (p values for all: <0.001) with higher values for the DM group. Most of the DM pregnant mothers were either overweight (BMI: 26.0-29.0) or obese (BMI: >29.0), on the other hand most of the NDM pregnant mothers were within normal range (BMI: 19.8-26.0). DM pregnant mothers were found more anemic (45.8% vs. 23.4%; p<0.001). Mean birth weight of the infants of DM & NDM groups were 3100g +/- .500g and 2850g +/- 360g respectively. The mean chest circumference of the infants of DM mothers was found significantly higher for diabetic group (p<0.01). DM mothers delivered most of the preterm babies (16.3% vs. 5.8%; p<0.002) and macrosomy babies were found only in this group (5.9%). Significant correlation was observed between birth weight and maternal MAC (p<0.001) in both the groups. Head circumference was found significantly correlated with maternal MAC and age in the DM group only. Diabetic pregnant women were significantly different compared to the non-diabetic group by nutritional status. The DM group experienced more anemia and preterm deliveries and macrosomic babies were born only in them.