Ambras Syndrome: First Reported Case in Bangladesh and its Oral Rehabilitation.

People with rare hypertrichosis syndromes became crowd-drawing money-making phenomena in many 19th century sideshow acts. These individuals have been referred to as dog-men, hair-men, and werewolves. In 1993, Baumister et al. described congenital hypertrichosis lanuginose or Ambras syndrome: a distinct form of congenital hypertrichosis characterized by excessive hair growth over the body and face associated with facial and occasional dental anomalies. Much is not known about this syndrome since fewer than 50 cases have been documented worldwide. In this case report, a nine year old girl presented with excessive hair growth throughout her body that was denser along her midline. Furthermore, her face displayed the typical dysmorphic features characteristic of Ambras syndrome: a round tip nose, thickened nasal cartilage, antiverted nares, prominent philtrum with deep groove, and a trapezoid mouth. Oral examination revealed normal oral mucosa with completely missing and unerupted decidious and permanent teeth. Panoramic radiographs confirmed unerupted deciduous teeth. Previous case reports have mentioned the presence of occasional dental anomalies such as retarded first and second dentition and absence of some teeth. However, this is the first reported case of Ambras syndrome presenting with complete anodontia. Prior cytogenetic studies performed on persons with Ambras syndrome have implicated a balanced pericentric inversion of chromosome 8. However, it is likely that dental anomalies are likely a result of a different genetic rearrangement. Further studies are needed to explore the cause of this rare phenotype of Ambras syndrome with complete unerupted dentition.

Clinical experience with BIAsp 30: Results from the Bangladesh cohort of the global A1chieve study.

The aim of A1chieve was to remedy the deficit of data on the efficacy and safety of insulin analogues in routine clinical care in less well-resourced developed countries. To present results from the Bangladesh cohort of the A1chieve study receiving BIAsp 30 ± oral anti diabetic drugs. A1chieve was a 6-month, observational study of 66,726 people with type 2 diabetes, started on insulin detemir, insulin aspart or biphasic insulin aspart (BIAsp 30) in 28 countries across four continents. A total of 1,093 subjects were recruited from 49 sites in Bangladesh and 580 subjects initiated on BIAsp 30 were studied. In the entire cohort, treatment with BIAsp 30 for 24 weeks significantly reduced mean HbA(1c) (2.8%, p < 0.001), fasting plasma glucose (4.0 mmol/L, p < 0.001) and post prandial plasma glucose (6.6 mmol/L, p < 0.001) levels from baseline. The rate of overall hypoglycaemic events in the entire cohort also reduced significantly at 24 weeks (1.86 to 0.02 events/person year, p < 0.0001). BIAsp 30 can be considered as a safe and effective option for initiating as well as intensifying insulin therapy for type 2 diabetes.

Clinical and Liver Enzymes among the Patients with Metabolic Syndrome with or without Non Alcoholic Fatty Liver Disease attending a Tertiary Care Hospital.

Metabolic syndrome is characterized by central obesity, dyslipidemia, raised blood pressure and impaired blood sugar levels. Patients with metabolic syndrome are at increased risk of type 2 diabetes and atherosclerotic cardiovascular disease. This cross-sectional observational study was carried out from January 2019 to December 2019 at the inpatient and outpatient department of BIRDEM General Hospital, Dhaka, Bangladesh. Adult subjects aged ≥18 years with metabolic syndrome (IDF criteria, 2006) were included and purposive sampling was done. A total of 242 participants were included and the mean age was 40.2±14.1 years ranging from 18-70 years. Among them, 140(57.85%) were female and 102(42.15%) were male. Out of 242 participants, 170(70.25%) subjects had Metabolic Syndrome (MetS) with Non-Alcoholic Fatty Liver (NAFLD) and 72(29.75%) subjects had metabolic syndrome without NAFLD. In the male participants, the mean waist-hip ratio (WHR) of MetS with NAFLD and MetS without NAFLD was 1.01±0.07 vs. 0.96±0.08 respectively (p-value 0.003). In female subjects, the mean waist-hip ratio (WHR) of MetS with NAFLD and MetS without NAFLD group was 0.90±0.10 vs. 0.86±0.08 respectively (p-value 0.026). MetS with NAFLD subjects were more hypertensive than MetS without NAFLD subjects (61.2% vs. 42.7%). In the MetS with NAFLD group (n=170), 11.8% was normoglycemic, 43.5% was prediabetic and 44.7% was diabetic. In the MetS without NAFLD group (n=72), 19.5% was normoglycemic, 50% was prediabetic and 30.5% was diabetic. SGPT value was significantly raised in MetS with NAFLD subjects (56.4%) than MetS without NAFLD (38.9%) subjects (p-value 0.038). SGOT value was significantly raised in MetS with NAFLD subjects (58.8%) than MetS without NAFLD subjects (41.7%); (p-value 0.005). Mean Total Cholesterol and Triglyceride were significantly raised in MetS with NAFLD subjects than MetS without NAFLD subjects (p-value 0.01). In Subjects with grade I fatty liver, mean SGPT and SGOT were 42.27±22.31 vs. 39.59±16.93 respectively. In Subjects with grade II fatty liver, mean SGPT and SGOT were 62.13±32.42 vs. 52.45±28.56 respectively. In grade III fatty liver, mean SGPT and SGOT were 51.50±32.19 vs. 41.00±17.52 respectively (p value <0.001). More than two-third of participants with metabolic syndrome had non-alcoholic fatty liver disease (NAFLD) and a significant elevation of liver enzymes than metabolic syndrome without NAFLD participants. About 85.0% of metabolic syndrome participants had glucose intolerance in the form of prediabetes and diabetes.

Prevalence of diabetes among hypothyroid subjects.

Thyroid dysfunction affects a significant portion of the general population. Most studies have found a higher prevalence of hypothyroidism in women, increasing with age. Prevalence of hyperthyroidism has been found to range 0.5-3.0%. Type 2 diabetes is a major public health problem affecting approximately 4.8 percent of the Bangladeshi population. The study was designed to see the prevalence of type 2 diabetes in hypothyroid patients of any etiology. Four hundred forty two consecutive cases of diagnosed hypothyroidism were recruited in the study. All diagnosed hypothyroid subjects (sub-clinical or overt) reported in one year not known to be diabetic previously, underwent standard OGTT (FPG and 2-hour post 75 gram glucose) before initiation of thyroid hormone replacement. Newly detected diabetes among the subjects diagnosed as hypothyroidism is significantly higher 4.8 vs. 7.01% (p<0.01) and the prevalence of pre-diabetic state Impaired Glucose Tolerance (IGT) is also higher (11% vs. 12.6%) among hypothyroid subjects. Pure IFG (FPG ≥6.1 mmol/l but <7.0 mmol/l, and 2-PG <7.8 mmol/l) was found in 5.2% according to WHO criteria and the prevalence is higher (6.8%) with ADA criteria (considering normal FBG <5.6mmol/l). Diabetes was found in 7.01% among hypothyroid subjects which is higher than the existing known prevalence (4.8%). IGT was also found higher among hypothyroid subjects than population prevalence (8.6%). Further larger sample study is essential to establish the findings.

Androgen versus erythropoietin for the treatment of anaemia of pre-dialysis chronic kidney disease.

Chronic kidney disease is a microvascular complication of diabetes mellitus (DM). Anemia is an important clinical manifestation to treat chronic kidney disease. Many subjects with poor socio-economic status having chronic kidney disease (CKD) and anaemia in a developing country can not afford the treatment with erythropoietin. This study has designed to see the efficacy of Nandrolone, a cheaper alternative; in comparison with recombinant human erythropoietin for management of anemia of pre-dialysis diabetic chronic kidney disease. Sixty adult diabetic patients with anaemia of chronic kidney disease on conservative treatment [Not on Hemodialysis (HD)] were enrolled. Patients were divided into two groups (Group 1 and Group 2) of 30 patients each. Group 1 patients received nandrolone deaconate 50 mg deep intramuscular and Group 2 recombinant human erythropoietin 100 IU per kilogram of body weight subcutaneously once weekly. Patients of both group received oral supplements in order to maintain body iron stores. All the relevant haematological and renal parameters were evaluated at the end of 3rd & 6th months. There was a statistically significant rise in haemoglobin concentration, packed cell volume, in both groups. The rise in haemoglobin concentration, in Group 2 was more marked followed by Group 1, at the end of 3rd, and 6th months. Nandrolone, though not equally effective, may be considered as a valid alternative therapy for the treatment of anemia of pre-dialysis diabetic chronic kidney disease to that of erythropoietin.

Bangladesh Endocrine Society (BES) Position Statement for Management of Diabetes and Other Endocrine Diseases in Patients with COVID-19.

BACKGROUND: The year 2020 witnessed a largely unprecedented pandemic of coronavirus disease (COVID-19), caused by SARS COV-2. Many people with COVID-19 have comorbidities, including diabetes, hypertension and cardiovascular diseases, which are significantly associated with worse outcomes. Moreover, COVID-19 itself is allied with deteriorating hyperglycemia. Therefore, Bangladesh Endocrine Society has formulated some practical recommendations for management of diabetes and other endocrine diseases in patients with COVID-19 for use in both primary and specialist care settings. OBJECTIVE: The objective of the article is to develop a guideline to protect the vulnerable group with utmost preference - the elderly and those with comorbid conditions. Therefore, to ensure the adequate protective measures and timely treatment for COVID-19 patients with diabetes, other endocrine diseases or any other comorbidities. CONSIDERING AND MONITORING ISSUES: The risk of a fatal outcome from COVID-19 may be up to 50% higher in patients with diabetes than in non-diabetics.Patients with diabetes and COVID had CFR 7.3-9.2%, compared with 0.9-1.4% in patients without comorbidities.Diabetic ketoacidosis may be one of the causes of mortality in COVID-19.There is wide fluctuation of blood glucose in these patients, probably due to irregular diet, reduced exercise, increased glucocorticoids secretion, and use of glucocorticoids. HbA1c should be <7.0% for the majority of the patients, this target may be relaxed in appropriate clinical settings.More emphasis should be given on day-to-day blood glucose levels. Hypoglycemia (<3.9 mmol/l) must be avoided.Frequent monitoring of blood glucose is needed in critically ill patients. CONCLUSION: The fight against COVID-19 has been proven to be a challenging one. Therefore, all healthcare personnel should make the best use of updated knowledge and skills to ensure adequate protective measures and timely treatment for COVID-19 patients with diabetes, other endocrine diseases or any other comorbidities.

The Incidence of Hypoglycemia among Insulin-Treated Patients with Type 1 or Type 2 Diabetes: Bangladeshi Cohort of International Operations-Hypoglycemia Assessment Tool Study.

OBJECTIVES: The objective of this study was to assess the incidence of hypoglycemia in patients with type 1 diabetes mellitus (T1DM) or type 2 diabetes mellitus (T2DM) in Bangladeshi cohort of the International Operations-Hypoglycemia Assessment Tool study. MATERIALS AND METHODS: Patients diagnosed with either T1DM or T2DM, aged ≥18 years, treated with insulin (any regimen) for >12 months, and completed self-assessment questionnaires (SAQs) to record demography, treatment information, and hypoglycemia during the 6-month retrospective and 4-week prospective periods (a total of 7 months) were enrolled in the study. RESULTS: A total of 1179 patients were enrolled and completed the SAQ1 (T1DM, n = 25; T2DM, n = 1154). Almost all patients (T1DM: 100.0% [95% confidence interval (CI): 86.3%, 100.0%] and T2DM: 97.0% [95% CI: 95.9%, 97.9%]) experienced at least 1 hypoglycemic event prospectively. The estimated rates of any and severe hypoglycemia were 26.6 (95% CI: 19.8, 35.0) and 14.1 (95% CI: 9.3, 20.4) events per patient-per year (PPY), respectively, for patients with T1DM and 18.3 (95% CI: 17.4, 19.2) and 12.1 (95% CI: 11.4, 12.9) events PPY, respectively, for patients with T2DM during the prospective period. At baseline, mean glycated hemoglobin (HbA1c) (±standard deviation) was 8.1 (±1.8%) for T1DM and 8.8 (±1.8%) for T2DM. Hypoglycemic rate was independent of HbA1c levels and types of insulin. CONCLUSIONS: This is the first patient dataset of self-reported hypoglycemia in Bangladesh; results confirm that hypoglycemia is underreported.

Neck Circumference as a Marker of Overweight and Obesity and Cutoff Values for Bangladeshi Adults.

OBJECTIVE: There are several methods of assessing overweight and obesity. Several studies conducted in different populations indicate that neck circumference (NC) can be used as a simple measure of overweight and obesity. This study was conducted to evaluate NC as a marker of overweight and obesity and to determine respective cutoff values for Bangladeshi male and female participants. RESEARCH DESIGN/MATERIALS AND METHODS: This cross-sectional observational study was conducted with during July 2013-June 2014 among randomly selected 871 Bangladeshi participants (male = 496 [56.9%], female = 375 [43.1%], aged >18 years) who visited Outpatient Department of United Hospital, Bangladesh Institute of Research and Rehabilitation in Diabetes, Endocrine and Metabolic disorders, primary health-care centers located in Dhaka, Savar, Gazipur. NC of participants was taken in centimeter to the nearest 1 mm, using plastic tape measure. Main outcome included NC, waist circumferences (WC), body mass index (BMI), and waist: hip ratio (WHR). RESULTS: Pearson's correlation coefficients indicated a significant association between NC and height (men, r = 0.33; women, r = 0.28; P < 0.0001), weight (men, r = 0.61; women, r = 0.55; P < 0.0001), BMI (men, r = 0.51; women, r = 0.41; P < 0.0001), WC (men, r = 0.61; women, r = 0.46; P < 0.0001), hip circumference (men, r = 0.61; women, r = 0.44; P < 0.0001), WHR (men, r = 0.22; women, r = 0.18; P < 0.0001). Receiver operating characteristic curve analysis showed that NC ≥34.75 cm in men (area under curve [AUC]: 0.77; P < 0.001) and ≥31.75 cm in women (AUC: 0.62; P < 0.001) were the best cutoff value for BMI ≥23 (overweight). NC ≥35.25 cm in men (AUC: 0.82; P < 0.001) and NC ≥34.25 cm in women (AUC: 0.76; P < 0.001) were the best cutoff value for BMI ≥27.5 (obesity). NC ≥35.25 cm in male (AUC: 0.83; P < 0.001) and NC ≥31.25 cm in women (AUC: 0.65; P < 0.001) were the best cutoff value for WC >90 cm in men and > 80 cm in women, respectively. NC ≥34.45 cm in male (AUC: 0.59; P = 0.001) and NC ≥31.25 cm in women (AUC: 0.66; P = 0.008) were the best cutoff value for WHR >0.9 in men and >0.8 in women, respectively. CONCLUSION: NC measurement is a simple, convenient, inexpensive screening measure to identify overweight and obese participants. Men with NC ≥34.75 cm and women with NC ≥31.75 cm are to be considered overweight while men with NC ≥35.25 cm and women with NC ≥34.25 cm are to be considered obese. NC ≥35.25 cm in male and NC ≥31.25 cm in women were the best cutoff value for abdominal obesity.

Safety and effectiveness of biphasic insulin aspart 30 in a Bangladeshi subgroup of type 2 diabetic patients switched from biphasic human insulin 30: a sub-analysis of the A1chieve study.

AIM: To determine the safety and effectiveness of biphasic insulin aspart 30 (BIAsp 30) therapy in Bangladeshi type 2 diabetes (T2D) patients switched from biphasic human insulin (BHI) as a sub-analysis of the A1chieve study. METHODS: Bangladeshi patients switched from BHI to BIAsp 30 at the discretion of their physicians were included. The primary outcome was the incidence of serious adverse drug reactions (SADRs), including major hypoglycaemia. Secondary outcomes comprised changes from baseline to Week 24 in the number of hypoglycaemic events, glycated haemoglobin A1c (HbA1c), fasting plasma glucose (FPG), postprandial plasma glucose (PPPG), systolic blood pressure and body weight. Quality of life (QoL) was evaluated at baseline and Week 24 using the EQ-5D questionnaire. RESULTS: A total of 82 patients (mean age ± SD: 52.3 ± 12.2 years; body mass index: 25.6 ± 3.3 kg/m(2)) with a mean diabetes duration of 9.5 ± 5.5 years and mean duration on insulin of 2.5 ± 2.4 years were included. The mean BIAsp 30 dose was 0.49 ± 0.20 U/kg at baseline and 0.47 ± 0.17 U/kg at Week 24. No SADRs were reported. No events of hypoglycaemia (overall, major, minor or nocturnal) were reported at Week 24. Mean HbA1c, FPG and PPPG levels improved by -2.5 ± 1.3%, -65.0 ± 31.8 mg/dL and -119.3 ± 48.7 mg/dL, respectively, over 24 weeks. QoL also improved (mean change from baseline: +28.5 ± 12.9 points). CONCLUSION: Switching from BHI to BIAsp 30 therapy improved blood glucose control and was well-tolerated in this Bangladeshi subgroup.

Presence and association of sub clinical hypothyroidism in subjects with metabolic syndrome.

AIMS: To determine the prevalence of subclinical hypothyroidism (SCH) among subjects with metabolic syndrome and to find out the relationship of subclinical hypothyroidism with different components of metabolic syndrome. MATERIALS AND METHODS: The study was conducted in the Department of Endocrinology, Bangladesh Institute of Research and Rehabilitation in Diabetes, Endocrine and Metabolic Disorders (BIRDEM), Dhaka during the period of April 2008-March 2009. One hundred and seventy three subjects with metabolic syndrome (according to IDF criteria) aged 20-69 years were included in the study. After primary selection, FPG and lipid profile were done. Those who had FPG≥100mg/dl or dislipidemia were selected for routine investigations such as SGPT, S. creatinine, TC, DC, ESR, HB%, ECG, and Ultrasonography of whole abdomen to exclude liver disease, renal disease, acute illness and cardiac disease respectively. Patients having normal investigations were finally selected for serum level of FT4 and TSH. RESULTS: A total of 173 subjects (105 male, and 68 female,) with metabolic syndrome were studied. Among them 14.3% (n=15) of male and 19.1% (n=13) of female had SCH. SCH was found more in obese subjects (BMI≥25kg/m(2) vs. BMI<25kg/m(2)). There was no significant difference among different parameters of metabolic syndrome in subjects with or without SCH. Although SCH was more prevalent in those who had hypertrigyceridemia and hypertension, there was no association between presence of fatty liver and SCH. CONCLUSIONS: Among the study subjects 14.3% male and 19.1% female had SCH. SCH is more prevalent in 41-60 years age group. No significant association was found among different parameters of MetS with SCH, however, when they constitute metabolic syndrome; there was a significant association between MetS and SCH.