Very Early Onset Inflammatory Bowel Disease: Diagnostic and Therapeutic Challenges for Pediatric Gastroenterologists.

Very early onset inflammatory bowel disease (VEO-IBD) is called when age of onset of IBD occurs below 6 years. Though it is rare, it has been increasing over last decade with decreasing age of onset. VEO-IBD is different compared with pediatric and adult-onset IBD in many aspects, including the disease type, location of the lesion, disease behavior and genetic susceptibility. These children with VEO-IBD are usually present with more severe disease than older children and adults. VEO-IBD is associated with monogenic defect. The thought of a monogenic cause of VEO-IBD was first confirmed by the detection of mutations of interleukin 10 (IL-10) receptor genes that cause impaired IL-10 signaling. Monogenic IBD possesses significant concern because it usually presents with refractory to conventional IBD treatment or fistulous Crohn's disease, so early treatment with biologics or an alternative approach such as hematopoietic stem cell transplantation (HSCT) might be looked-for. Before establishing IBD, we must think of more common diseases of this age group. Infection and Cow's milk protein allergy (CMPA) are two common conditions and it can cause severe colitis. Confirmation of chronic intestinal inflammation by endoscopies is of greatest significance for the diagnosis of IBD. There should be no age limit for performing endoscopies. Severe disease should be treated with biologic agents and surgery. Identification of genes associated with IBD leads to better understanding of its pathogenesis, which could help to provide more targeted interventions. We discuss the topic here to create awareness among Pediatricians so that the patients can be benefited.

Study on Body Mass Index and Fasting Serum Glucose in Postmenopausal Women.

Menopause is a normal physiological period that is characterized by the completely stoppage of endometrial cycles in women between the age of 45-55 years due to lack of ovarian follicular function. Several postmenopausal syndromes such as hot flushes, night sweats, vaginal dryness, depression, irritability, headache, and sleep disturbance can occur more frequently in this period which hampers the standard of life. This study was done to assess body mass index and fasting serum glucose changes in postmenopausal women in contrast with reproductive women. This cross-sectional analytical type of study was conducted in the Department of Physiology, Mymensingh Medical College, Bangladesh from January 2021 to December 2021. Total 140 women subject's age ranged between 25-65 years were involved in this study. Among them 25-45 years aged 70 (Seventy) reproductive women were involved as control group (Group I) and 45-65 years aged 70 (Seventy) postmenopausal women were taken as study group (Group II). By Anthropometric measurements, Body Mass Index (BMI) such as height and weight were taken in meter and kilogram respectively and fasting serum glucose was measured by GOD-PAP method. Results were expressed as mean (±SD) and by unpaired Student's 't' test, statistical significance of difference among the group was calculated. The mean±SD of BMI of Group I and Group II were 23.05±4.43kg/m² and 29.01±3.12kg/m² respectively. The mean±SD of body mass index is notably increase in study group in contrast with control group. The mean±SD of fasting serum glucose of control Group I and study Group II were 4.77±2.04mmol/l and 6.11±1.61mmol/l respectively. In study Group II fasting serum glucose was increased. Due to increased fasting serum glucose caused by lower level of female sex hormones specially estrogen hormone increased chance of cardiovascular diseases in postmenopausal women. Assessment of these parameters are important for early detection and prevention of complication related to high BMI and fasting serum glucose level for leading a better life.

Status of Body Mass Index and Blood Pressure in Post-Menopausal Women.

Menopause, in between the age of 45-55 years every woman faces it, that means completely stoppage of menstruation because the decrease level of estrogen from the normal level. In this period the quality life is disturbed due to hormonal imbalance specially estrogen. This study was done to evaluate the changes of body mass index and blood pressure in post-menopausal women in comparison to reproductive women. This cross-sectional analytical type of study was done in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh and the period is from January 2021 to December 2021. Total 140 women subjects age ranged between 25-65 years were involved in this study. Among them 70 post-menopausal women (45-65 years) were involved as study group (Group II) and 70 (seventy) reproductive women (25-45 years) were taken as control group (Group I). By anthropometric measurements, Body Mass Index (BMI) such as height and weight were taken in meter and kilogram respectively and systolic and diastolic blood pressure by aneroid sphygmomanometer (ALPK2, Japan). Findings were expressed as mean±SD and by unpaired student's 't' test, analytical significance of difference among the group was calculated. The mean±SD of BMI of Group I and Group II were 23.05±4.43kg/m² and 29.01±3.12kg/m² respectively. The mean±SD of body mass index is significantly higher in study group in comparison with control group. The average (±SD) of systolic blood pressure of control group I and study group II were 118.29±10.00 mm of Hg & 134.00±11.91 mm of Hg respectively. The mean±SD of systolic blood pressure was significantly higher in study group in comparison with control group. The mean±SD of diastolic blood pressure of control group I and study Group II were 79.21±6.46 mm of Hg and 89.00±6.23 mm of Hg respectively. The mean±SD of diastolic blood pressure was significantly higher in study group in comparison with control group. Post-menopausal women with high systolic and diastolic pressure have a chance of development of cardiovascular diseases, stroke. So assessment of these parameters are important for early detection and prevention of complication related to high BMI and Blood Pressure for leading a healthy life.

Evaluation of Fecal Pancreatic Elastase-1 as a Measure of Pancreatic Exocrine Function in Children with Pancreatitis.

Pancreatic exocrine insufficiency occurs as a clinical consequence of chronic pancreatitis leading to fat maldigestion, malabsorption and malnutrition. Fecal elastase-1 is a laboratory-based test used for the diagnosis or exclusion of pancreatic exocrine insufficiency. The aim of the study was to observe the value of fecal elastase-1 in children with pancreatitis as an indicator of pancreatic exocrine insufficiency. This was a cross-sectional descriptive study conducted from January 2017 through June 2018. Thirty children with pain abdomen as control and 36 patients with pancreatitis as cases were included. An ELISA technique which recognizes human pancreatic elastase-1 from spot stool sample was employed for the test. Fecal elastase-1 activity in spot stool samples in acute pancreatitis (AP) ranged from 198.2-500µg/g with a mean of 342.1±136.4µg/g, acute recurrent pancreatitis (ARP) ranged from 15-500µg/g with a mean of 332.8±194.5µg/g and chronic pancreatitis (CP) ranged from 15-492.8µg/g with a mean of 222.2±197.1µg/g was obtained. In controls, fecal elastase-1 ranged from 28.4-500µg/g with a mean of 398.8±114.9µg/g. Disease severity was classified as mild to moderate pancreatic insufficiency (fecal elastase-1 100 to 200µg/g stool) was found in AP (14.3%) and CP (6.7%) cases. The severe pancreatic insufficiency (fecal elastase-1<100µg/g stool) was observed in ARP (28.6%) and CP (46.7%) cases. Malnutrition was observed in severe pancreatic insufficiency cases. This study result showed that fecal elastase-1 can be used as a measure of pancreatic exocrine function in children with pancreatitis.

Evaluate the Changes of Body Mass Index and Blood Pressure in Combined Oral Contraceptive Pill Users.

The oral contraceptive pill, 'the pill', was introduced in the starting of the 1960s. Presently, worldwide about 100 million women are current users of combined hormonal contraceptives (COC) most frequently used in the western world. The most frequently used agents are a combination of drugs containing both the estrogen and progesterone. This combination is considered to be highly efficacious, generally considered 99.9% and a use effectiveness of 97.0% to 98.0%. This study was done to evaluate the changes of Body Mass Index (BMI) and Blood pressure ( BP) in combined oral contraceptive pill users in comparison to non- users. This cross sectional study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from July 2021 to June 2022. Total number of 120 reproductive aged women, age ranged from 15-55 years was included in this study. Among them, sixty (60) combined oral contraceptive pill users subjects were taken as study group (Group II) and sixty (60) age matched oral pill non users subjects were taken as control group (Group I). Data were expressed as mean±SD and statistical significance of difference among the group was calculated by unpaired students' 't' test and Pearson's correlation coefficient test. The mean±SD of BMI of control group (Group I) and study group (Group II) were 22.50±1.50 kg/m² and 27.64±1.28 kg/m² respectively. In study group (Group II) BMI was increased. Result is statistically highly significant (p<0.001). The mean±SD of systolic blood pressure of control group (Group I) and study group (Group II) were 111.17±8.51 mm of Hg and 127.50±6.14 mm of Hg respectively. In this study the mean±SD of diastolic blood pressure of control group (Group I and study group (Group II) were 75.58±5.05mm of Hg and 87.00±4.89 mm of Hg respectively. In study group (Group II) diastolic blood pressure was increased. Result is statistically highly significant (p<0.001). So the assessment of this parameter is important for early detection and prevention of complication related to combined oral contraceptive pill use for leading a healthy life.

Study on Body Mass Index, Serum Total Cholesterol and Serum Triglycerides in Adult Male Hypertensive Patients.

Hypertension or elevated blood pressure is a serious medical condition that significantly increases the risks of heart, brain, kidney and other diseases. An estimated 1.28 billion adults aged 30-79 years worldwide have hypertension, most (two-thirds) living in low- and middle-income countries. An estimated 46.0% of adults with hypertension are unaware that they have the condition. In Bangladesh, 21.3 million of the adult population has hypertension and that is 21.0% of total population. About half of them (48.7%) with hypertension are diagnosed and 34.9% are under treatment. One of the global targets for non communicable diseases is to reduce the prevalence of hypertension by 33.0% between 2010 and 2030. This study was done to evaluate the effects of hypertension and to find out the major complications occurring due to hypertension. This analytical type of cross sectional study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from January 2021 to December 2021. A total number of 120 male subjects, age ranged from 20-59 years was included in this study. Among them, sixty (60) hypertensive subjects were taken as study group (Group II) and sixty (60) ages matched normotensive adult male subjects were taken as control group (Group I). The results were calculated and analyzed by using Statistical package for social science (SPSS). Anthropometric measurements such as height and weight were taken in meter and kilogram respectively. Systolic and diastolic blood pressure were measured by Aneroid Sphygmomanometer (ALPK2, Japan), laboratory analysis of fasting serum total cholesterol by CHOD-POD method, fasting serum triglycerides by GPO-POD method. In this study we found that in study group Mean±SD of body mass index (29.36±2.60, p<0.001), fasting serum total cholesterol (232.05±11.18, p<0.001) and fasting serum triglycerides (180.67±19.44, p<0.001) which were significantly increased with comparison to control group. It's concluded from this study we recommended that routine estimation of these parameters is important for prevention of complication related to hypertension for leading a healthy life.

A Comparative Study of Body Mass Index and Blood Pressure between Sedentary and Non-Sedentary Workers.

Sedentary lifestyle are spreading worldwide because of lack of available spaces for exercise, increased occupational sedentary behaviors such as office work and the increased prevalence of television and video devices. Increased incidence of sedentary lifestyle and obesity in developed and developing countries is one of the major risk factors for the development of cardiovascular diseases. This study was undertaken to analyze the differences in anthropometric and cardiovascular parameters in sedentary and non-sedentary male subjects. This analytical type of cross sectional study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from July 2021 to June 2022. This study consisted 50 male employees of 25-60 years who worked in an office were study group (Group II) and control group Group-I) consisted of 50 male age matched physical laborers. BMI was calculated as weight in kilogram divided by the height in meter square i.e. kg/m². Blood Pressure was measured by indirect auscultatory method with an aneroid sphygmomanometer. Statistical analysis was done by using Student's un-paired 't' test. Values expressed as mean ± standard deviation. P value was taken as significant at 5 percent confidence level. The mean±SD BMI in kg/m² of Group I and Group II were 21.90±2.22 and 29.32±3.13 respectively. The mean±SD systolic blood pressure of Group I and Group II were 112.20±6.79 and 145.10±8.11 and mean±SD diastolic blood pressure of Group I and Group II were 72.80±5.73 and 91.00±5.05 respectively. The BMI and blood pressure were increased in sedentary group compared to non-sedentary group. From the study, it appears that the risk for cardiovascular disease is increased in sedentary workers.

Malignant Infantile Osteopetrosis with Bone Marrow Involvement.

Osteopetrosis (Marble bone disease) is a very rare congenital genetic disease of skeleton, resulting from defective bone resorption, due to functionally defective osteoclast, leading to accumulation of excessive bone mass. Malignant infantile osteopetrosis (MIO) is one of the varieties of osteopetrosis, which is fatal and is diagnosed in early infancy. Malignant infantile osteopetrosis is present with abnormal bone remodeling, hematological abnormities, features of extramedullary hematopoiesis. Radiology is the key of diagnosis. In this case, we present a 5-monthold male infant diagnosed as malignant infantile osteopetrosis, who presented with bronchopneumonia, anemia, thrombocytopenia, hepatosplenomegaly, failure to thrive (FTT).

Solitary Rectal Ulcer Syndrome: A Rare and Often Misdiagnosed Cause of Rectal Bleeding in Children.

Solitary rectal ulcer syndrome (SRUS) is an uncommon benign rectal disorder. Typically, young adults are affected and it is rare in children. Straining during defecation, self-induced trauma and paradoxical contraction of puborectalis muscle are the major contributing factors of this condition. Clinical features of SRUS are rectal bleeding, mucorrhoea, excessive straining during defecation, tenesmus, feeling of incomplete defecation and constipation. A complete and thorough history is most important for diagnosis of SRUS. Rectal bleeding may be misinterpreted as originating from an anal fissure caused by constipation or as other causes of rectal bleeding such as a juvenile polyp. The best and most accurate diagnostic method of SRUS is rectal biopsy. The major histological feature of SRUS is fibromuscular obliteration of the lamina propria. Avoiding straining, regular toilet habit, use of bulk laxatives, steroid and sucralfate enemas are the mainstay of treatment. Biofeedback mechanism is another treatment option. Because the clinical presentation varies, the diagnosis requires a high index of suspicion for both the clinician and the pathologist.

Relationship between Body Mass Index and Serum Alanine Transaminase Concentration in Obese Female.

The serum Alanine Transaminase (ALT) activity has been regarded as a reliable and sensitive marker of liver disease. In the context of obesity ALT may also be a good indicator of overall health. Obesity has been reported as a risk factor associated with elevation of ALT, which is a surrogate marker of Non-alcoholic fatty liver disease (NAFLD). Elevated ALT may correlate with the severity of NAFLD in obese female. This study was done to evaluate the changes of serum ALT in obese female age ranged 30-60 years in comparison to normal healthy female of same age. At the same time we can know the relationship between body mass index and serum ALT concentration in obese female. This analytical cross-sectional study was carried out in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from January 2020 to December 2020. A total number of 100 female subjects were included in this study. Among them fifty (50) normal healthy female were taken as control group (Group I) and fifty (50) obese female were taken as study group (Group II). The level of serum ALT was determined by Ultra violet (UV) method. Data were expressed as mean±SD and statistical significance of difference among the group was calculated by unpaired Student's 't' test. Pearson's correlation coefficient test was done to find the correlation of serum ALT with BMI by using SPSS (version 21.0). During interpretation of results, p values of <0.001 were considered as statistically highly significant. In this study, serum level of ALT was significantly higher (p<0.001) in obese female compared to those of healthy control female. In addition, there is a positive correlation of serum ALT with BMI. From the results of the present study, it can be concluded that, elevated ALT was significantly associated with high BMI as well as with other feature of NAFLD.

Study on Body Mass Index and Serum Calcium Level in Postmenopausal Women.

Menopause is one of the natural parts of women's life when menstrual cycle stops due to lack of estrogen, which occurs in middle age. Various health problems occur in this period which hampers quality of life. This study was done to evaluate the changes of body mass index and serum calcium level in post-menopausal women in comparison to reproductive women. This cross-sectional analytical type of study was done in the Department of Physiology, Mymensingh Medical College, Mymensingh, Bangladesh from January 2021 to December 2021. A total number of 140 female subjects age ranged from 25-65 years were included in this study. Among them 70 (seventy) postmenopausal woman (45-65 years) were taken as study group (Group II) and 70 (seventy) reproductive women (25-45 years) were taken as control group (Group I). Body mass index was measured by Anthropometric measurements such as height and weight taken in meter and kilogram respectively and Serum calcium level was measured by colorimetric method with O-Cresolftalein-complexone (OCC). Data were expressed as mean (±SD) and statistical significance of difference among the group was calculated by unpaired Student's 't' test. The mean±SD of BMI of Group I and Group II were 23.05±4.43 kg/m² and 29.01±3.12 kg/m² respectively. The mean±SD of body mass index is significantly higher in study group in comparison with control group. Serum calcium of Group I and Group II were 10.95±0.77 mg/dl and 7.99±0.55 mg/dl respectively. The mean±SD of serum calcium level was significantly lower in study group in comparison with control group. Post-menopausal women with low serum calcium level have a chance of development of osteoporosis. So, assessment of this parameter is important for early detection and prevention of complication related to low serum calcium level for leading a sound life.

Efficacy of Different Diagnostic Test for Identifying Wilson's Disease.

Wilson disease is an autosomal recessive disorder in which copper pathologically accumulates primarily within the liver, brain and other tissues. It can presents clinically as liver disease, as a progressive neurological disorder or as psychiatric illness. The wide array of clinical manifestations of WD can lead to misdiagnosis with subsequent greater risk of irreversible damage to liver and brain. Many tests can be used to investigate patients of Wilson disease, including serum free copper, 24 hours urine copper estimation, hepatic copper estimation and genetic mutation testing. But there is no single ideal diagnostic test that can exclude or confirm the disease with certainty. The aim of the study was to find out the efficacy of different diagnostic test for the diagnosis of Wilson disease. This cross-sectional analytical study was conducted at department of Paediatric Gastroenterology and Nutrition of Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from January 2016 through January 2018. A total of 56 cases of Wilson disease and 39 patients with a liver disease other than WD were studied. Wilson disease was diagnosed by Leipzig score. Along with other physical findings and laboratory investigations slit lamp eye examination for KF ring, serum ceruloplasmin and 24 hour urinary copper excretion were done. The mean age of WD patients was 9.69±2.37 years, male female ratio was 1:1. Serum ceruloplasmin level was significantly lower in WD patient (p<0.001). Median of 24 hour urinary copper in WD was 702.75µg/ 24 hr (range119-11210µg/24 hour) and in non WD group it was 77.41µg/24 hour (range 20.0-478µg/24 hour) and the difference between them is statistically significant (p=0.001). The sensitivity of KF ring was 82.1% and specificity was 100%. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of serum ceruloplasmin were 98.2%, 92.3%, 94.8%, 97.2% and 95.7% respectively. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 24 hour urinary copper were 100%, 63%, 80% and 85.1% respectively. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of KF ring, serum ceruloplasmin and basal 24 hour urinary copper excretion when combined together came out to be 70.4%, 100%, 100%, 59.3% and 79.3% respectively. This study result showed that serum ceruloplasmin and 24 hour urinary copper can be used as a screening test for the diagnosis of Wilson disease.

COVID 19 in Children: Gastrointestinal, Hepatobiliary and Pancreatic Manifestation.

The most devastating pandemic of this era coronavirus disease-2019 (COVID-19) is caused by a novel virus named severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). Although it is primarily a respiratory pathogen, it can also result in several extra-pulmonary manifestations includes gastrointestinal symptoms, hepatocellular injury. Angiotensin-converting enzyme-2 (ACE-2) receptor and transmembrane serine protease 2 (TMPRSS2), the entry receptor for the causative coronavirus SARS-CoV-2 is co-express in the gastrointestinal tract, hepatocyte, and cholangiocytes similar to the respiratory mucosa. The presence of these receptors facilitates the entry into the tissue and causes direct viral tissue damage, which is a proposed mechanism of injury. Diarrhoea, nausea, vomiting, abdominal discomfort are common gastrointestinal manifestations, whereas derangement of liver function tests is the most hepatic manifestation in COVID-19. In this article, we reviewed on SARS-CoV-2 disease COVID-19 regarding gastrointestinal, hepatic, and pancreatic manifestation, the mechanisms by which the virus may inflict damage, and their management perspective.

Abdominal Tuberculosis in Children: Is It Really Uncommon?

Now a day's tuberculosis has become a global emergency especially in children and abdomen is the sixth commonest extra-pulmonary site of involvement. Diagnosis of abdominal tuberculosis (TB) in children is still challenging. Non specific constitutional symptoms and long lasting abdominal manifestations cause unnecessary delay in diagnosis in children. Abdominal TB can be of various types like peritoneal TB, gastrointestinal TB, tubercular lymphadenopathy and visceral TB. Diagnosis can be confirmed by histopathology, culture or PCR and imaging technique also play an important role in diagnosis. Morbidity and mortality can be reduced in significant number by early recognition and effective aggressive treatment. In TB endemic areas, it could be strongly considered in children with vague complaints like chronic abdominal pain, low grade fever and weight loss. Response to anti-tubercular therapy may indirectly help the physicians to come to a diagnosis.

Role of Liver Biopsy in the Diagnosis of Neonatal Cholestasis due to Biliary Atresia.

Biliary atresia is the commonest cause of cholestatic jaundice. Early diagnosis & surgical correction helps long time survival & prevent development of cirrhosis. This study was conducted to find out the role of liver biopsy in the diagnosis of biliary atresia having positive hepatobiliary scintigraphy as there is chance of false positivity. This cross sectional study was carried out in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh at Paediatric Gastroenterology & Nutrition Department from January 2014 to June 2015. All admitted patients with the diagnosis of neonatal cholestasis were evaluated clinically and by scintigraphy for biliary atresia. Having positive hepatobiliary scintigraphy but highly suggestive of biliary atresia were enrolled for this study. A total of 108 cases were initially selected. Among them 33 patients showed no excretion of tracer during hepatobiliary scintigraphy. Liver biopsy was done in these 33 cases. All cases (100%) had history of passage of pale or acholic stool. Fractionated serum bilirubin of >2mg/dl was found all of the cases. Histological scoring system reveals typical features of biliary atresia in 27(81.8%). Two had no conclusive histology of biliary atresia, four had features of neonatal hepatitis (12.1%). In this study, percutaneous liver biopsy with histological analysis by scoring system was found useful for the correct diagnosis of biliary atresia.

Irritable Bowel Syndrome: Is it Rare in Children?

Irritable bowel syndrome (IBS) is one of the most common and best studied disorders among the group of functional gastrointestinal disorders. It is a functional bowel disorder in which abdominal pain or discomfort is associated with defecation or a change in bowel habit. Visceral hypersensitivity and increased GIT motility are the main patho-physiological mechanism for developing IBS. IBS present with diarrhea and constipation or both. Investigation is least needed for diagnosis of IBS rather done to exclude differential diagnosis. Diagnosis is done on the basis of Rome-III criteria. Proper counseling, dietary management, anti-spasmotic and antidepressant are the mainstay of treatment.

Clinical and Laboratory Predictors of Esophageal Varices in Children with Chronic Liver Disease.

Esophageal varices are a serious consequence of portal hypertension in patients with chronic liver diseases. Several studies have evaluated possible noninvasive markers of esophageal varices to reduce the number of unnecessary endoscopies in patients with chronic liver disease. Aim of this study was to identify clinical and laboratory predictors of esophageal varices in children with chronic liver disease. This cross sectional observational study was done at Pediatric Gastroenterology and Nutrition Department of Bangabandhu Sheikh Mujib Medical University, Dhaka, over a period of 6 months. Fifty (50) consecutive cases of chronic liver disease patient under 15 years of age were evaluated clinically, biochemically and endoscopically. Esophageal varices were classified into 4 grades according to Conn's grading system. Based on endoscopic findings (presence/absence of esophageal varices) all the patients were categorized into two groups; Group I: Chronic liver disease with esophageal varices and Group II: Chronic liver disease without esophageal varices. Most (51.5%) of the patients in Group I belong to age 10-12 years and majority 35.3% of Group II patients belong to ≤2 years. More than half of the (51.5%) patients had grade III varices followed by 24.2% grade II, 21.2% grade I and only 3.0% had grade IV. All patients had splenomegaly in Group I and 70.6% of Group II, which was significantly (p<0.05) higher in Group I. Jaundice, spider angioma, hepatomegaly and ascites werealmost alike between two groups. Platelet count <1,50,000/mm³ in 72.73% patients of Group I and 41.18% in Group II, which was significantly (p<0.05) higher in Group I. Similarly, Serum albumin <3.5mg/dl in 78.79% patients of Group I and 47.6% of Group II, was also significantly (p<0.05) higher in Group I. Serum bilirubin, serum alanine aminotransferase and International normalized ratio (INR) were almost similar between two groups. Wilson's disease was found in 42.4% of chronic liver disease with esophageal varices and 35.3% of Chronic liver disease without esophageal varices. Hepatitis B virus infections were 6.1% and 5.9% in Group I and Group II respectively. Unknown cause was found 42.4% and 17.6% in Group I and Group II respectively. Splenomegaly, Platelet count <1,50,000/mm³ and serum albumin <3.5mg/dl are important predictors of esophageal varices. These three parameters can be used independently to predict esophageal varices in children with chronic liver disease.

Childhood Wilson Disease: Bangladesh Perspective.

Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013. One hundred consecutive children of WD between 3 to 18 years of age were evaluated for clinical & biochemical profile. Mean age of studied children was 8.5±1.5 years. Male female ratio was 2:1. Ninety one percent patients were Muslim and nine percent Hindu. Consanguinity of marriage was found in 30% cases. Seven parents were first degree cousin. Family history of chronic liver disease was present in 15% of patients. Most (53%) cases of the hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Among 100 patients of WD, 69 children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic & neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of chronic liver disease were found in 18% patients. Commonest stigmata was thenar and hypothenar wasting (n=8). Keiser- Fleischser ring (K-F ring) was found in 76% of the total patients. K-F ring was present in 84% ( 58 out of 69) of the hepatic only Wilsonian patients and in 90% (18 out of 20) of all neurologic Wilsonian patients. Asymptomatic and psychiatric patient had no K-F ring. About 26% of the WD patients had Coombs negative hemolytic anemia in PBF. Most of the WD patients had altered liver function. Elevated serum transaminase was found in 85% of all cases, prolonged prothrombin time in 59% cases & low serum albumin in 53% cases. Seventy three percent patients had low serum ceruloplasmin, basal urinary copper of >100µgm/day was found in 81% cases and urinary copper following penicillamine challenge of >1200µgm/day was found in 92% cases. In 28 cases with hepatic presentation esophageal varices were identified by upper gastrointestinal endoscopy. WD patient with hepatic presentations were given zinc sulphate along with penicillamine. All patients with neurological manifestation as well as asymptomatic cases were maintained on zinc therapy. WD is a treatable metabolic cause of liver disease. Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.

Aetiology and Outcome of Acute Liver Failure in Children: Experience at a Tertiary Care Hospital of Bangladesh.

Acute liver failure (ALF) is a rapidly progressive, potentially fatal syndrome resulting from rapid death or injury to a large proportion of hepatocytes, caused by a variety of insult, leaving insufficient hepatic paranchymal mass to sustain liver function. The aetiology of ALF varies according to the age of patient and development of the country. The outcome of ALF also varies according to aetiology: survival is better in paracetamol poisoning whereas it is poor in metabolic diseases. The present study was undertaken to observe the underlying aetiology and outcome of ALF in children under 18 years of age admitted at the department of Paediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. It was a retrospective review of medical records from November 2011 through October 2014. During this period a total of 35 patients were diagnosed to have ALF. Aetiology was established in 25(71.4%) cases, whereas in 10(28.6%) cases, no identifiable cause was found. Viral hepatitis was the underlying cause in 12(34.3%) cases. After treatment 15(43%) ALF patients survived, 8(23%) left hospital with risk bond (DORB), and 12(34%) patients died. The study showed that among the 12 death patients, 5(41.7%) had viral hepatitis, 3(25%) Wilson's disease, and in 4(33.3%) no cause could be identified. Viral hepatitis and Wilson disease were found to be two common causes of ALF in this study. Future studies with larger sample size are required to know the actual causes of acute liver failure in Bangladeshi children.

Chronic Hepatitis B in Children - A Review.

Hepatitis B Virus (HBV) infection is prevalent worldwide. The prevalence is lowest (0.2-0.5%) in countries having high standard of living. About 10 million people are chronically infected with HBV in Bangladesh and it is possible that most infections occur during childhood. Overall prevalence is about 3% in Bangladesh. Perinatal transmission is more common in hyper-endemic areas of South East Asia. Chronic hepatitis B infection evolves through five phases. Most of the children belong to immune tolerant phase. About 57% patients of chronic hepatitis B are asymptomatic. Treatment of chronic hepatitis B is difficult. Decision regarding when, whom and how to treat in children is complex. Moreover, there are only limited drugs that can be used in treating chronic hepatitis B in children. Goal of therapy are to reduce viral replication, to minimize liver injury, to reduce consequence of liver injury like cirrhosis, hepatocellular carcinoma and to reduce infectivity of HBV. Treatment should be considered in chronic hepatitis B if patient have persistently elevated ALT of more than twice normal and evidence of viral replication. There are some special circumstances where treatment of chronic hepatitis B can be given in absence of standard criteria. These conditions are cirrhosis, chemotherapy, immunosuppression, presence of co-infection (HBV-HIV), family history of HCC and pregnant women with high viral load. Sero-conversion occurs in about 17-32% cases if treated with oral nucleot(s)ide analogue and in about 58% cases if treated with interferon. These expensive drugs with limited treatment success are not suitable for the people of Bangladesh. Therefore, risk factors identification and prevention of HBV infection is the logical and rational approach for a country like Bangladesh. Vaccination against HBV play central role in preventing infection. HBV vaccine has been incorporated in EPI schedule since 2004 in Bangladesh. Immunoprophylaxis of babies of HBsAg positive mother and post exposure prophylaxis are effective ways of prevention.