Md . Benzamin | Bangabandhu Sheikh Mujib Medical University (original) (raw)
Papers by Md . Benzamin
Gastroenterology & Endoscopy, 2024
Background: Antibiotics are often overused in the management of diarrhea, despite few indications... more Background: Antibiotics are often overused in the management of diarrhea, despite few indications. Lack of confidence regarding the causative organism and limited access to diagnostic facilities contribute to the misuse of antibiotics in acute watery diarrhea (AWD). Objective: The present study aims to assess the variation in diarrhea management before and after implementing the 'Diarrhea Stool Card'.
Kathmandu University Medical Journal, Oct 24, 2022
KYAMC Journal, 2021
Wilson disease is an autosomal recessive, copper storage disease, caused by a mutation in the ATP... more Wilson disease is an autosomal recessive, copper storage disease, caused by a mutation in the ATP7B gene. Due to mutation in ATP7B is decreased secretion of ceruloplasmin into blood and decrease in excretion of copper into bile. Excess copper accumulate to toxic levels,mainly in the liver and secondarily in other organs. Children clinically become symptomatic after the age of 5 years. Clinical features ranges from asymptomatic raised transaminases to variable degree of liver disease, neurological symptoms and according involvement of other oragns. Diagnosis of Wilson disease is challenging. Modified Leip-zig score is useful for diagnosis. Treatment can be done with zinc and other chelators. KYAMC Journal Vol. 11, No.-4, January 2021, Page 212-217
The most devastating pandemic of this era coronavirus disease-2019 (COVID-19) is caused by a nove... more 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 CO...
Pediatrics …, 1997
Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in child... more Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5±3.8 years) with this diagnosis, to define the ...
Pakistan pediatric journal, 2019
Objective: Evaluation of etiological spectrum and outcome of children with acute hepatitis in a t... more Objective: Evaluation of etiological spectrum and outcome of children with acute hepatitis in a tertiary care hospital. Study Design: Retrospective observational study. Place and Duration of Study: This study was conducted at the Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from January 2015 to January 2018. Material and Methods: This study included 161 admitted children; age between 5 months to 16 years who were diagnosed as acute hepatitis. Results: Mean age (years) of children was 8.14±3.11, range 0.5-16 years. About 91.3% were acute viral hepatitis. Most common etiologies were hepatitis A virus (HAV) (70.9%), then hepatitis E virus (HEV) (8%), HAV with HEV co-infection (3.7%), Salmonella hepatitis (6.3%), enteric fever with HAV co-infection (3.2%), drug induced hepatitis (anti-TB) (2.4%) and others. Anicteric hepatitis was 7.5%. Conclusion: Acute hepatitis is a common problem in children, HAV and HEV is the most common etiology. Dual infections, like HAV with HEV co-infection and enteric fever with co-infection are not uncommon. KeyWords: Children, Viral Hepatitis, Salmonella Hepatitis, Anicteric Hepatitis
Definition: Portal hypertension (PHT) is a condition that occurs due to the formation of portal-s... more Definition: Portal hypertension (PHT) is a condition that occurs due to the formation of portal-systemic collaterals which shunt a portion of the portal blood flow to the systemic circulation , bypassing the liver. It can arise from disorders with blood flow at any level within the portal system. 1 Extra hepatic portal venous obstruction (EHPVO) is an important cause of portal hypertension which constitutes 68-76% of portal hypertension in children from developing countries. The relation of haematemesis, splenomegaly and portal hypertension was first recognized by Banti almost a century ago. Majority of cases are due to primary thrombosis of the portal vein. 2 Kobrich coined the term cavernoma to describe spongy appearance of portal vein (PV). 3 As per the Asia Pacific association for study of Liver (APASL) consensus (2006), EHPVO is defined as ''a vascular disorder of liver, characterized by obstruction of the extrahepatic PV with or without involvement of intrahepatic PV radicles or splenic or superior mesenteric veins''. The recent Baveno VI consensus definition adds recent thrombus in the PV in the definition along with the presence of cavernoma, however, excludes cirrhosis and other liver condition like Idiopathic portal hypertension. 5 Abstract Portal hypertension in children is not uncommon and extra hepatic portal venous obstruction (EHPVO) constitute about 75% of portal hypertension. Several risk factors predispose to development of EHPVO such as neonatal sepsis, umbilical catheterization, severe dehydration, abdominal trauma or surgery etc. Common presentations are haematemesis and splenomegaly. Acute variceal bleeding is a medical emergency. Liver function is normal in extrahepatic portal hypertension unless there is portal bilopathy. High index of suspicion is the key of early diagnosis. Esophagogastrodudenoscopy is diagnostic for portal hypertension and doppler ultrasonography of portal vein confirm extra hepatic portal venous obstruction (EHPVO) in presence of thrombus and/or cavernoma. Adequate management including endotherapy, pharmacotherapy and/or surgery is an important key for better outcome.
Congenital cytomegalovirus (CMV) infection is the most common congenital infection worldwide and ... more Congenital cytomegalovirus (CMV) infection is the most common congenital infection worldwide and most
individuals are eventually exposed to this agent. In developing countries the seroprevalence in women of
reproductive age approximates 100%. Cytomegalovirus (CMV) infection has great importance to obstetriciangynecologists
and pediatricians. Despite the heavy disease burden, CMV infection is severely under-diagnosed
because the majority (approximately 80%) of affected mothers are asymptomatic. The clinical manifestations of
congenital CMV infection vary widely, from asymptomatic infection to potentially life-threatening disseminated
disease. Prenatal diagnosis of fetal CMV infection can be made by testing amniotic fluid for cytomegalovirus
by amniocentesis. Diagnosis of congenital cytomegalovirus infection in neonates should include real-time
PCR of saliva, urine, or both, as soon as possible after birth. Antiviral therapy is not routinely recommended
for congenital cytomegalovirus infection. Neonates with life-threatening infection and moderately to severely
symptomatic congenital cytomegalovirus disease, CNS involvement is considered for immediate treatment that
should be initiated within first month of life.
Asian Journal of Research and Reports in Gastroenterology, 2019
Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterol... more Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterology and hepatology department. Outcome is different according to aetiology. Objective: To observe the aetiology, outcome and prognostic factors of pediatric acute liver failure. Methods: Consecutive 62 children aged 2 to 16 years of age who were diagnosed as acute liver failure from November 2015 to April 2018 were included in this study. All the clinical profiles, laboratory data and outcome were recorded in a preformed data sheet. Data were analysed by SPSS for Windows version 20. Original Research Article Yasmin et al.; AJRRGA, 2(1): 1-8, 2019; Article no.AJRRGA.48550 2 Results: Mean age was 8.5 years. Thirty-nine (62.9%) patients were between 5-10 years of age. Male were 53%. We made a diagnosis of 39 (63%) patients as Wilson disease alone; Another 3 Wilson disease acute liver failure patients had concomitant with HAV, HEV or HSV in each one. HAV only was responsible for 17 patients and HEV for 1. One patient was Haemophagocytic lymphohistiocytosis and etiology could not be identified in 1 patient. The overall death in study population was 48% (30). Twenty-four (57%) of 42 acute liver failure patients due to Wilson disease had died. Five (29%) of 17 patients due to HAV infection and 1 patient with HLH died. Ascites, high total bilirubin, high INR and etiology like Wilson disease were the worse prognostic factors for outcome of acute liver failure in children. Conclusion: Wilson disease was the most common aetiology of acute liver failure in children in this study. Early diagnosis is essential as outcome was worse. Majority of viral etiology improved with supportive care.
Paediatric Nephrology Journal Of Bangladesh, 2018
Wilson disease is an autosomal-recessive disorder of chronic copper toxicosis due to mutation in ... more Wilson disease is an autosomal-recessive disorder of chronic copper toxicosis due to mutation in theATP7B gene which causes impaired biliary copper excretion resulting in hepatic copper accumulation & toxicity and subsequent multisystem disease involving the liver, brain, cornea, skeleton and rarely the heart. The disease typically begins with an asymptomatic period with subclinical hepatitis and progresses to liver cirrhosis and neuropsychiatric symptoms. It may present with some unusual features which sometimes confuses clinicians and makes a diagnostic dilemma. Here we present an 15 years old boy presenting with Bowing of leg and gynaecomastia diagnosed as Wilson disease.
Bangabandhu Sheikh Mujib Medical University Journal, 2018
Langerhans cell histiocytosis is a rare disease of Langerhans cell where multiple systems like sk... more Langerhans cell histiocytosis is a rare disease of Langerhans cell where
multiple systems like skin, bones, lymph nodes,
pituitary gland, bone marrow, liver, spleen, lungs
etc. can be affected. Langerhans cell histiocytosis
was previously called histiocytosis X, which included
three entities named eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease.These are, now, replaced by singlesystem
single site, single-system multisite, and multisystem disease.
Bangabandhu Sheikh Mujib Medical University Journal(BSMMUj), 2018
Juvenile polyposis syndrome is a rare hamartomatous polyposis disorder with significant gastroint... more Juvenile polyposis syndrome is a rare hamartomatous
polyposis disorder with significant gastrointestinal
malignant potential due to mutations in
BMPR1A and SMAD4.3 Juvenile polyposis syndrome
patient presented with rectal bleeding,
prolapsed rectal polyp, pallor, abdominal pain due
to intussusception and diarrhea. Associated other
morphological abnormalities are growth failure,
alopecia, cleft lip or palate, macrocephaly, hypertelorism,
polydactyly, digital clubbing, hydrocepha-lus, mental retardation, congenital cardiac anomalies, pulmonary arteriovenous malformations, pulmonary stenosis, telangiectasias, Meckel’s diverticulum,
intestinal malrotation, bifid uterus and vagina (female) and cryptorchidism (male).
Bangladesh Medical Journal, 2016
Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads ... more Moyamoya disease is a chronic, progressive occlusion of the
circle of Willis arteries that leads to the development of
characteristic collateral vessels evidence by imaging,
particularly cerebral angiography.1 e disorder initially
involves the intracranial portion of the internal carotid arteries
and progresses to involve the middle, anterior and posterior
cerebral arteries. Collateral vascular network developed due to
compensatory dilatation of lenticulostriate and thalamostriate
arteries
Paediatric Nephrology Journal Of Bangladesh, 2019
Background Childhood gastrointestinal diseases are common and increasing in Bangladesh. Wide vari... more Background
Childhood gastrointestinal diseases are common and increasing in Bangladesh. Wide variety of diseases present with gastrointestinal symptoms specially hepatomegaly and/ or splenomegaly. It is essential to monitor the nature of diseases according to regional area.
Aim and objective
To identify the pattern and etiology of diseases in children presented hepatomegaly with or without splenomegaly at Pediatric Gastroenterology department in Bangabandhu Sheikh Mujib Medical University (BSMMU).
Methods
We reviewed retrospectively the data of all children presented with hepatomegaly and/ or splenomegaly between 1st July 2017 to 30th June 2018 in the Pediatric Gastroenterology department, Bangabandhu Sheikh Mujib Medical University (BSMMU).
Result
A total 390 patients were studied, 26 patients were excluded due to incomplete data, rest 364; among them 222 were males and 142 females. About 39.5% (143) had hepatosplenomegaly and 40%(146) had isolated hepatomegaly. Twenty eight percent (103) diagnosed as neonatal cholestasis,15.5%(56) chronic liver disease (CLD) other than biliary cirrhosis,14% (51) extra hepatic portal hypertension, 12% (43) acute hepatitis, 6% (22) storage disease, 6%(22) diagnosed as malignant disease, rest 18%(66) were some common and some rare diseases/syndrome, 3% patients had double pathology at diagnosis.
Conclusion
It is observed that neonatal cholestasis was most common followed by chronic liver disease (CLD) and extrahepatic portal hypertension. Double pathology in neonatal cholestasis were common. Malignancies were not uncommon.
Scholars Journal of Applied Medical Sciences (SJAMS), 2018
Bardet-Biedl syndrome (BBS) is a rare ciliopathic autosomal recessive genetic disorder. It presen... more Bardet-Biedl syndrome (BBS) is a rare ciliopathic autosomal recessive genetic disorder. It presents with varied clinical manifestations like retinitis pigmentosa, polydactyly, central obesity, mental retardation and renal dysfunction. Other rare manifestations include diabetes mellitus, heart disease, hepatic fibrosis and neurological manifestations. Most of these symptoms may not be present at birth but appear and progressively worsen during the first and second decades of life. We, here, have presented a 4-year-old girl with features of Bardet-Biedl syndrome.
Scholars Journal of Applied Medical Sciences, 2019
Background: Chronic liver disease (CLD) is not uncommon in Bangladesh. Ascites is common feature ... more Background: Chronic liver disease (CLD) is not uncommon in Bangladesh. Ascites is common feature of CLD patients. Ascites is a culture media for bacterial infection. Spontaneous bacterial peritonitis is a frequent complication of ascites in children with chronic liver disease. The rapid and effective diagnosis of peritonitis will reduce mortality. Aim: The aim of this study is to see the variants of ascitic fluid infection in children with chronic liver disease. Methods: It is a cross sectional observational study. This study was conducted at the department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh without interrupting standard care practiced in the department. The study was done over a period of one & half year, from January 2016 through July 2017. During this period consecutive children CLD with ascites were included in this study. Sample was collected purposively who was fulfilling inclusion criteria. The details history, physical examination findings and investigation reports were recorded in a predesigned standard data sheet. History was obtained directly from the parents, which include jaundice, abdominal pain, fever, diarrhea, family history of liver diseases or other relevant medical histories. Investigations were done for diagnosis of chronic liver disease & identify the cause. Ascitic fluid study especially physical appearance, cytology, total protein, LDH, Gram stain & culture were done in all case. Statistical analysis was done using Statistical Package for Social Science 20.0 (SPSS; Chicago, Illinois) for Windows XP. Results: A total of 30 children were selected according to selection criteria. After ascitic fluid study, all patients were divided into two groups: Group I included five patients (16.67%) with culture negative neutrocytic ascites (CNNA) in which the neutrophil count ≥ 250/mm3 and culture was negative indicate infected group. Group II, twenty five (83.33%) patients in which the neutrophil count < 250/mm3 and negative culture indicate non infected group. None of our patients had spontaneous bacterial peritonitis (SBP) or bacterascites. Presence of fever, history of abdominal pain and tenderness significantly higher in CNNA group (p<0.05). Conclusions: Culture negative neutrocytic ascites (CNNA) was the only variety of ascitic fluid infection in this study. Infected cases may be asymptomatic. Clinical features of ascitic fluid infection are needed to differentiate the infected and non-infected cases. Ascitic fluid study is essential to identify infection. Culture of ascitic fluid is not always diagnostic of infection.
Portal hypertension not uncommon in children. Extra hepatic portal venous obstruction (EHPVO) con... more Portal hypertension not uncommon in children. Extra hepatic portal venous obstruction (EHPVO) constitutes about 75% of portal hypertension. Several risk factor predispose to development of EHPVO such as neonatal sepsis, umbilical catheterization, severe dehydration, abdominal trauma or surgery etc. But risk factors are usually detected in less than half of patients. Patient commonly present with variceal bleeding with splenomegaly. Here we are presenting a case with some atypical features.
Gastroenterology & Endoscopy, 2024
Background: Antibiotics are often overused in the management of diarrhea, despite few indications... more Background: Antibiotics are often overused in the management of diarrhea, despite few indications. Lack of confidence regarding the causative organism and limited access to diagnostic facilities contribute to the misuse of antibiotics in acute watery diarrhea (AWD). Objective: The present study aims to assess the variation in diarrhea management before and after implementing the 'Diarrhea Stool Card'.
Kathmandu University Medical Journal, Oct 24, 2022
KYAMC Journal, 2021
Wilson disease is an autosomal recessive, copper storage disease, caused by a mutation in the ATP... more Wilson disease is an autosomal recessive, copper storage disease, caused by a mutation in the ATP7B gene. Due to mutation in ATP7B is decreased secretion of ceruloplasmin into blood and decrease in excretion of copper into bile. Excess copper accumulate to toxic levels,mainly in the liver and secondarily in other organs. Children clinically become symptomatic after the age of 5 years. Clinical features ranges from asymptomatic raised transaminases to variable degree of liver disease, neurological symptoms and according involvement of other oragns. Diagnosis of Wilson disease is challenging. Modified Leip-zig score is useful for diagnosis. Treatment can be done with zinc and other chelators. KYAMC Journal Vol. 11, No.-4, January 2021, Page 212-217
The most devastating pandemic of this era coronavirus disease-2019 (COVID-19) is caused by a nove... more 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 CO...
Pediatrics …, 1997
Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in child... more Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5±3.8 years) with this diagnosis, to define the ...
Pakistan pediatric journal, 2019
Objective: Evaluation of etiological spectrum and outcome of children with acute hepatitis in a t... more Objective: Evaluation of etiological spectrum and outcome of children with acute hepatitis in a tertiary care hospital. Study Design: Retrospective observational study. Place and Duration of Study: This study was conducted at the Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from January 2015 to January 2018. Material and Methods: This study included 161 admitted children; age between 5 months to 16 years who were diagnosed as acute hepatitis. Results: Mean age (years) of children was 8.14±3.11, range 0.5-16 years. About 91.3% were acute viral hepatitis. Most common etiologies were hepatitis A virus (HAV) (70.9%), then hepatitis E virus (HEV) (8%), HAV with HEV co-infection (3.7%), Salmonella hepatitis (6.3%), enteric fever with HAV co-infection (3.2%), drug induced hepatitis (anti-TB) (2.4%) and others. Anicteric hepatitis was 7.5%. Conclusion: Acute hepatitis is a common problem in children, HAV and HEV is the most common etiology. Dual infections, like HAV with HEV co-infection and enteric fever with co-infection are not uncommon. KeyWords: Children, Viral Hepatitis, Salmonella Hepatitis, Anicteric Hepatitis
Definition: Portal hypertension (PHT) is a condition that occurs due to the formation of portal-s... more Definition: Portal hypertension (PHT) is a condition that occurs due to the formation of portal-systemic collaterals which shunt a portion of the portal blood flow to the systemic circulation , bypassing the liver. It can arise from disorders with blood flow at any level within the portal system. 1 Extra hepatic portal venous obstruction (EHPVO) is an important cause of portal hypertension which constitutes 68-76% of portal hypertension in children from developing countries. The relation of haematemesis, splenomegaly and portal hypertension was first recognized by Banti almost a century ago. Majority of cases are due to primary thrombosis of the portal vein. 2 Kobrich coined the term cavernoma to describe spongy appearance of portal vein (PV). 3 As per the Asia Pacific association for study of Liver (APASL) consensus (2006), EHPVO is defined as ''a vascular disorder of liver, characterized by obstruction of the extrahepatic PV with or without involvement of intrahepatic PV radicles or splenic or superior mesenteric veins''. The recent Baveno VI consensus definition adds recent thrombus in the PV in the definition along with the presence of cavernoma, however, excludes cirrhosis and other liver condition like Idiopathic portal hypertension. 5 Abstract Portal hypertension in children is not uncommon and extra hepatic portal venous obstruction (EHPVO) constitute about 75% of portal hypertension. Several risk factors predispose to development of EHPVO such as neonatal sepsis, umbilical catheterization, severe dehydration, abdominal trauma or surgery etc. Common presentations are haematemesis and splenomegaly. Acute variceal bleeding is a medical emergency. Liver function is normal in extrahepatic portal hypertension unless there is portal bilopathy. High index of suspicion is the key of early diagnosis. Esophagogastrodudenoscopy is diagnostic for portal hypertension and doppler ultrasonography of portal vein confirm extra hepatic portal venous obstruction (EHPVO) in presence of thrombus and/or cavernoma. Adequate management including endotherapy, pharmacotherapy and/or surgery is an important key for better outcome.
Congenital cytomegalovirus (CMV) infection is the most common congenital infection worldwide and ... more Congenital cytomegalovirus (CMV) infection is the most common congenital infection worldwide and most
individuals are eventually exposed to this agent. In developing countries the seroprevalence in women of
reproductive age approximates 100%. Cytomegalovirus (CMV) infection has great importance to obstetriciangynecologists
and pediatricians. Despite the heavy disease burden, CMV infection is severely under-diagnosed
because the majority (approximately 80%) of affected mothers are asymptomatic. The clinical manifestations of
congenital CMV infection vary widely, from asymptomatic infection to potentially life-threatening disseminated
disease. Prenatal diagnosis of fetal CMV infection can be made by testing amniotic fluid for cytomegalovirus
by amniocentesis. Diagnosis of congenital cytomegalovirus infection in neonates should include real-time
PCR of saliva, urine, or both, as soon as possible after birth. Antiviral therapy is not routinely recommended
for congenital cytomegalovirus infection. Neonates with life-threatening infection and moderately to severely
symptomatic congenital cytomegalovirus disease, CNS involvement is considered for immediate treatment that
should be initiated within first month of life.
Asian Journal of Research and Reports in Gastroenterology, 2019
Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterol... more Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterology and hepatology department. Outcome is different according to aetiology. Objective: To observe the aetiology, outcome and prognostic factors of pediatric acute liver failure. Methods: Consecutive 62 children aged 2 to 16 years of age who were diagnosed as acute liver failure from November 2015 to April 2018 were included in this study. All the clinical profiles, laboratory data and outcome were recorded in a preformed data sheet. Data were analysed by SPSS for Windows version 20. Original Research Article Yasmin et al.; AJRRGA, 2(1): 1-8, 2019; Article no.AJRRGA.48550 2 Results: Mean age was 8.5 years. Thirty-nine (62.9%) patients were between 5-10 years of age. Male were 53%. We made a diagnosis of 39 (63%) patients as Wilson disease alone; Another 3 Wilson disease acute liver failure patients had concomitant with HAV, HEV or HSV in each one. HAV only was responsible for 17 patients and HEV for 1. One patient was Haemophagocytic lymphohistiocytosis and etiology could not be identified in 1 patient. The overall death in study population was 48% (30). Twenty-four (57%) of 42 acute liver failure patients due to Wilson disease had died. Five (29%) of 17 patients due to HAV infection and 1 patient with HLH died. Ascites, high total bilirubin, high INR and etiology like Wilson disease were the worse prognostic factors for outcome of acute liver failure in children. Conclusion: Wilson disease was the most common aetiology of acute liver failure in children in this study. Early diagnosis is essential as outcome was worse. Majority of viral etiology improved with supportive care.
Paediatric Nephrology Journal Of Bangladesh, 2018
Wilson disease is an autosomal-recessive disorder of chronic copper toxicosis due to mutation in ... more Wilson disease is an autosomal-recessive disorder of chronic copper toxicosis due to mutation in theATP7B gene which causes impaired biliary copper excretion resulting in hepatic copper accumulation & toxicity and subsequent multisystem disease involving the liver, brain, cornea, skeleton and rarely the heart. The disease typically begins with an asymptomatic period with subclinical hepatitis and progresses to liver cirrhosis and neuropsychiatric symptoms. It may present with some unusual features which sometimes confuses clinicians and makes a diagnostic dilemma. Here we present an 15 years old boy presenting with Bowing of leg and gynaecomastia diagnosed as Wilson disease.
Bangabandhu Sheikh Mujib Medical University Journal, 2018
Langerhans cell histiocytosis is a rare disease of Langerhans cell where multiple systems like sk... more Langerhans cell histiocytosis is a rare disease of Langerhans cell where
multiple systems like skin, bones, lymph nodes,
pituitary gland, bone marrow, liver, spleen, lungs
etc. can be affected. Langerhans cell histiocytosis
was previously called histiocytosis X, which included
three entities named eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease.These are, now, replaced by singlesystem
single site, single-system multisite, and multisystem disease.
Bangabandhu Sheikh Mujib Medical University Journal(BSMMUj), 2018
Juvenile polyposis syndrome is a rare hamartomatous polyposis disorder with significant gastroint... more Juvenile polyposis syndrome is a rare hamartomatous
polyposis disorder with significant gastrointestinal
malignant potential due to mutations in
BMPR1A and SMAD4.3 Juvenile polyposis syndrome
patient presented with rectal bleeding,
prolapsed rectal polyp, pallor, abdominal pain due
to intussusception and diarrhea. Associated other
morphological abnormalities are growth failure,
alopecia, cleft lip or palate, macrocephaly, hypertelorism,
polydactyly, digital clubbing, hydrocepha-lus, mental retardation, congenital cardiac anomalies, pulmonary arteriovenous malformations, pulmonary stenosis, telangiectasias, Meckel’s diverticulum,
intestinal malrotation, bifid uterus and vagina (female) and cryptorchidism (male).
Bangladesh Medical Journal, 2016
Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads ... more Moyamoya disease is a chronic, progressive occlusion of the
circle of Willis arteries that leads to the development of
characteristic collateral vessels evidence by imaging,
particularly cerebral angiography.1 e disorder initially
involves the intracranial portion of the internal carotid arteries
and progresses to involve the middle, anterior and posterior
cerebral arteries. Collateral vascular network developed due to
compensatory dilatation of lenticulostriate and thalamostriate
arteries
Paediatric Nephrology Journal Of Bangladesh, 2019
Background Childhood gastrointestinal diseases are common and increasing in Bangladesh. Wide vari... more Background
Childhood gastrointestinal diseases are common and increasing in Bangladesh. Wide variety of diseases present with gastrointestinal symptoms specially hepatomegaly and/ or splenomegaly. It is essential to monitor the nature of diseases according to regional area.
Aim and objective
To identify the pattern and etiology of diseases in children presented hepatomegaly with or without splenomegaly at Pediatric Gastroenterology department in Bangabandhu Sheikh Mujib Medical University (BSMMU).
Methods
We reviewed retrospectively the data of all children presented with hepatomegaly and/ or splenomegaly between 1st July 2017 to 30th June 2018 in the Pediatric Gastroenterology department, Bangabandhu Sheikh Mujib Medical University (BSMMU).
Result
A total 390 patients were studied, 26 patients were excluded due to incomplete data, rest 364; among them 222 were males and 142 females. About 39.5% (143) had hepatosplenomegaly and 40%(146) had isolated hepatomegaly. Twenty eight percent (103) diagnosed as neonatal cholestasis,15.5%(56) chronic liver disease (CLD) other than biliary cirrhosis,14% (51) extra hepatic portal hypertension, 12% (43) acute hepatitis, 6% (22) storage disease, 6%(22) diagnosed as malignant disease, rest 18%(66) were some common and some rare diseases/syndrome, 3% patients had double pathology at diagnosis.
Conclusion
It is observed that neonatal cholestasis was most common followed by chronic liver disease (CLD) and extrahepatic portal hypertension. Double pathology in neonatal cholestasis were common. Malignancies were not uncommon.
Scholars Journal of Applied Medical Sciences (SJAMS), 2018
Bardet-Biedl syndrome (BBS) is a rare ciliopathic autosomal recessive genetic disorder. It presen... more Bardet-Biedl syndrome (BBS) is a rare ciliopathic autosomal recessive genetic disorder. It presents with varied clinical manifestations like retinitis pigmentosa, polydactyly, central obesity, mental retardation and renal dysfunction. Other rare manifestations include diabetes mellitus, heart disease, hepatic fibrosis and neurological manifestations. Most of these symptoms may not be present at birth but appear and progressively worsen during the first and second decades of life. We, here, have presented a 4-year-old girl with features of Bardet-Biedl syndrome.
Scholars Journal of Applied Medical Sciences, 2019
Background: Chronic liver disease (CLD) is not uncommon in Bangladesh. Ascites is common feature ... more Background: Chronic liver disease (CLD) is not uncommon in Bangladesh. Ascites is common feature of CLD patients. Ascites is a culture media for bacterial infection. Spontaneous bacterial peritonitis is a frequent complication of ascites in children with chronic liver disease. The rapid and effective diagnosis of peritonitis will reduce mortality. Aim: The aim of this study is to see the variants of ascitic fluid infection in children with chronic liver disease. Methods: It is a cross sectional observational study. This study was conducted at the department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh without interrupting standard care practiced in the department. The study was done over a period of one & half year, from January 2016 through July 2017. During this period consecutive children CLD with ascites were included in this study. Sample was collected purposively who was fulfilling inclusion criteria. The details history, physical examination findings and investigation reports were recorded in a predesigned standard data sheet. History was obtained directly from the parents, which include jaundice, abdominal pain, fever, diarrhea, family history of liver diseases or other relevant medical histories. Investigations were done for diagnosis of chronic liver disease & identify the cause. Ascitic fluid study especially physical appearance, cytology, total protein, LDH, Gram stain & culture were done in all case. Statistical analysis was done using Statistical Package for Social Science 20.0 (SPSS; Chicago, Illinois) for Windows XP. Results: A total of 30 children were selected according to selection criteria. After ascitic fluid study, all patients were divided into two groups: Group I included five patients (16.67%) with culture negative neutrocytic ascites (CNNA) in which the neutrophil count ≥ 250/mm3 and culture was negative indicate infected group. Group II, twenty five (83.33%) patients in which the neutrophil count < 250/mm3 and negative culture indicate non infected group. None of our patients had spontaneous bacterial peritonitis (SBP) or bacterascites. Presence of fever, history of abdominal pain and tenderness significantly higher in CNNA group (p<0.05). Conclusions: Culture negative neutrocytic ascites (CNNA) was the only variety of ascitic fluid infection in this study. Infected cases may be asymptomatic. Clinical features of ascitic fluid infection are needed to differentiate the infected and non-infected cases. Ascitic fluid study is essential to identify infection. Culture of ascitic fluid is not always diagnostic of infection.
Portal hypertension not uncommon in children. Extra hepatic portal venous obstruction (EHPVO) con... more Portal hypertension not uncommon in children. Extra hepatic portal venous obstruction (EHPVO) constitutes about 75% of portal hypertension. Several risk factor predispose to development of EHPVO such as neonatal sepsis, umbilical catheterization, severe dehydration, abdominal trauma or surgery etc. But risk factors are usually detected in less than half of patients. Patient commonly present with variceal bleeding with splenomegaly. Here we are presenting a case with some atypical features.