Bangabandhu Sheikh Mujib Medical University
Dhaka, Dhaka | Bangladesh
Main Specialties: Pediatric Gastroenterology
Additional Specialties: Pediatric Gastroenterology, Hepatology and Nutrition
Primary Affiliation: Bangabandhu Sheikh Mujib Medical University - Dhaka, Dhaka , Bangladesh
Mediscope-The Journal of GMC
AbstractPortal 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 portalhypertension 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.
Indian J Gastroenterol 2019 08 19;38(4):310-316. Epub 2019 Aug 19.
Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, 1000, Bangladesh.
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J Enam Med Col 2019; 9(2): 116–126
Journal of Enam Medical College
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 severelysymptomatic congenital cytomegalovirus disease, CNS involvement is considered for immediate treatment that should be initiated within first month of life.
Yasmin et al.; AJRRGA, 2(1): 1-8, 2019; Article no.AJRRGA.48550
Asian Journal of Research and Reports in Gastroenterology
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.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 studypopulation 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.
BSMMU J 2019; 12: 113-117
Bangabandhu Sheikh Mujib Medical University Journal
Dr. Hazera Akter: A 6-month-old boy of nonconsanguineous parents admitted to the Department of Pediatric Gastroenterology with the complaints of progressive jaundice, dark urine and generalized pruritus for one month (Figure 1). The boy was well up to five months of age. Then he developed jaundice which was progressive in nature with intermittent pale colored stool along with dark urine. His mother also complaints for generalized pruritus which was severe in intensity (disturbing sleep and daily activities) without any diurnal variations. There was no history of sib death or family history of a similar type of illness. Physical examination revealed he was moderately icteric, scratch mark present over the whole body and mild (2 cm) firm hepatomegaly present with normal physical development. The laboratory data included normal complete blood counts except leucocytosis mostly lymphocyte. Serum total bilirubin was 8.8 mg/dL(conjugated bilirubin 5.1 mg/dL), alanine transaminase 26 U/L and gamma-glutamyl transferase 22 U/L, prothrombin time, activated partial thromboplastin time and international normalized ratio were within normal limit. Serum cholesterol level was 133 mg/dL. Anti-HAV IgM was negative. Urine R/E was normal and urinary reducing substances were nil. Renal functions, X-ray spines and echocardiography were normal. Ultrasonography of the abdomen showed mild hepatomegaly. Slitlamp examination of eyes showed no abnormality. Liver biopsy was done and histopathological examination revealed the paucity of interlobular bile ducts (out of eight portal tracts examined, two had bile duct) (Figure 2A and B). Bile duct to portal tract ratio was 0.25. The hepatocytes were mildly swollen and occasionalsinusoid contained bile plugs. Based on history, physical findings and laboratory investigations with liver biopsy our diagnosis was nonsyndromic interlobular bileduct paucity. Provisional Diagnosis Nonsyndromic bile duct paucity.
Kamal Hossen et al., Sch J App Med Sci, January, 2019; 7 (1): 410-418
Scholars Journal of Applied Medical Sciences
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.
Paediatr Indones, Vol. 59, No. 1, January 2019
Background: The prevalence of childhood overweight andobesity has increased over the last two decades due to high caloric intake and decreased physical activity which occurred in conjunction with increasing prevalence of hypertension. Microalbuminuria is an early sign of damage to the kidney and cardiovascular system. Hypertensive, overweight childrenhave an increased chance of microalbuminuria.Objectives: To assess the prevalence of overweight, hypertension, and microalbuminuria in urban and rural school going children and contributing risk factors and associations.Methods: This cross-sectional study was done in schoolchildren aged 6 to 16 years, from urban and rural areas, in Bangladesh, from September 2015 untill August 2016. Subjects’ weights, heights, and blood pressures (BP) were measured. Overweight and hypertension (HTN) statuses were determined with age-appropriate standardized charts. Subjects were divided into overweight and normoweight groups to evaluate risk factors for overweight such as family history (F/H) of obesity, F/H of HTN, daily physical outdoor activities, and monthly family income by comparative analysis. All overweight children were divided into hypertensive and normotensive groups in order to compare their fasting lipid profiles, urine microalbumin, serum creatinine, and random blood sugar.Results: A total of 976 schoolchildren from urban (471, 48.3%) and rural (505, 51.7%) areas were included. Overweightwas observed in 22.3% of the urban group and in 8.1% of the rural group. Hypertension was observed in 24.7% of overweight children and in 2.5% of normal weight children. Contributing risk factors for overweight were less physical outdoor activities, F/H of obesity, F/H of HTN, and higher family income. Microalbuminuria and random blood sugar were significantly increased in the overweight with hypertensiongroup compared to the normotensive group.Conclusion: Overweight is a health problem, noted especially in urban areas. Hypertension is also significantly increased in overweight children. Factors like F/H of hypertension, obesity, sedentary lifestyle, and higher socioeconomic status are significantly associated with overweight. Microalbuminuria and increased random blood sugar are also significantly higher observed in hypertensive overweight children compared to normotensive overweight children.
BSMMU J 2018; 11: 304-309
Bangabandhu Sheikh Mujib Medical University Journal
Dr. Rezina Parveen: A 17-month-old girl, only issue of non-consanguineous parents, hailing from Sirajgonj, Bangladesh immunized as perEPI schedule got admitted to the inpatient department with the history of abdominal distension, jaundice, not growing well along with scaly skin lesion over scalp, chest, palm and sole for 9 months. She was reasonably well 9 months back. Then she developed abdominal distention which was increasing day-by-dayassociated with gradually deepening jaundice. There was also a history of irregular fever and cough for 1 month. Fever was high-grade irregularin nature, subsided by taking antipyretics in conjunction with non-productive cough. There was no history of contact with the tuberculosis patient, any surgery or dental procedure, family history of liver disease, but the history of one unit blood transfusion. On physical examinations, she was dyspnic, febrile, mildly pale and icteric (Figure 1). The respiratory rate was 48 breaths/min, SPO2 95% without O2. The blood pressure was within normal limit. The digital clubbing, multiple red papular non-itchy skin lesion on both palm and sole and diffuse scaly lesion on the scalp, and BCG mark were present. There was no lymphadinopathy. She was severely wasted, stunted and underweight. On systemic examination, the abdominal distention with huge hepatosplenomegaly and ascites were found. The wheeze and crackles were present on both lung fields. The laboratory data (Table I) showed anemia (hemoglobin: 8.6 g/dL), markedly raised erythrocyte sedimentation rate (120 mm in 1st hour), grossly impaired liver function (serum alanine aminotransferase: 238 U/L, total bilirubin: 9.2 mg/dL, serum albumin 27 g/L). The X-ray chest showed consolidation (Figure 2). The Mantoux test was 2 mm. The abdominal ultrasonography showed heterogenous echotexture of the liver with multiple nodular lesion.Dr. Sayma Rahman Munmun: After evaluating the patient’s presenting features, physical findings and the laboratory test results she was considered forfurther investigation because the skin lesions remained unexplained. For this, the skin biopsy was done and the tissue was sent for histopathology.Dr. Md. Shafikul Alam Tanim: The histopathological examination of the biopsy specimen taken from the scalp following anesthesia revealed mixed infiltrateof Langerhans cells, lymphocytes and occasional eosinophils in the upper dermis. However, a variable number of neutrophils and plasma cells were also found in many other cases. Immunohistochemical analysis was performed with anti- CD1a, S100 and CD68 primary antibodies. Langerhans cells organized in groups and singly showed brown immunohistochemical stain for CD1a and S100 protein, but not for CD68 protein. In immunohistochemical examination, CD1a and S100 positivity confirmed the diagnosis of Langerhans cell histiocytosis (Figure 3). Dr. Munmun: After getting the histopathology report, we did the skeletal survey to see the bone involvement. We also did the bone marrow puncture which was normal and planned for the liver biopsy but could not do that as because theparents did not give consent. Dr. Manmohan Bir Shrestha: Patient’s X-ray of the right leg revealed well-defined round intramedullary non-expansile lucent lesion with marginal sclerosis in the metadiaphyseal region of the proximal shaft of tibia. Radiological differentials for this type of lesion includes benign bone neoplasms (i.e. simple bone cyst, enchondroma, chondroblastoma), Brodie’s abscess, Langerhans cell histiocytosis, brown tumor of hyperparathyroidism andmetastasis. In Langerhans cell histiocytosis, the common locations include the skull and long bones. In the case of long bones, Langerhans cell histiocytosis usually involves diaphysis or metadiaphysis. Patient’s chest X-ray anteroposterior view revealed almost round dense homogenous opacity in the right mid zone at suprahilar region, possibly consolidation. Dr. Munmun: Radiological findings of the patient histiocytosis. After considering the history, physical examination, biopsy and immunohistochemistryfindings we reached a diagnosis of Langerhans cell histiocytosis. was also in favor of the Langerhans cell
Md. Benzamin et al., Sch. J. Med. Case Rep., Nov, 2018; 6(11): 980-985
Scholars Journal of Medical Case Reports (SJMCR)
Abstract: 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.
Rukunuzzaman M., Benzamin M., Sultana K. (2018) Management of Chronic HBV Infection in Children. In: Ozaras R., Tahan V. (eds) Viral Hepatitis: Chronic Hepatitis B. Springer, Cham
Hepatitis B virus (HBV) infection is prevalent worldwide. About ten 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. Chronic hepatitis B infection evolves through five phases. Most of the children belong to the immune-tolerant phase. Treatment of chronic hepatitis B is difficult. The goals of therapy are to reduce viral replication, to minimize liver injury, to reduce consequence of liver injury like cirrhosis and hepatocellular carcinoma, and to reduce infectivity of HBV. Treatment should be considered in chronic hepatitis B if patients have persistently elevated ALT of more than twice the normal range and evidence of viral replication. Seroconversion occurs in about 17–32% of cases if treated with oral nucleot(s)ide analogue and in about 58% of cases if treated with interferon. Risk factor identification and prevention of HBV infection is the logical and rational approach
Suraiya Begum et al., Sch. J. App. Med. Sci., Jul 2018; 6(7): 2898-2901
Scholars Journal of Applied Medical Sciences (SJAMS)
Abstract: Bardet-Biedl syndrome (BBS) is a rare ciliopathic autosomal recessive genetic disorder. It presents with varied clinical manifestations like retinitispigmentosa, 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 butappear 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.
BSMMU J 2018; 11: 64-69
Bangabandhu Sheikh Mujib Medical University Journal
Case PresentationDr. Rafia Rashid: A 15 year old girl of non-comsanguineous parents presented at the outpatient department with the history of intermittent rectal bleeding for the last 6 years. Rectal bleeding was painless and there was the passage of a small amount of bright red blood drop by drop after defecation. She also complained of something coming out through the anus during defecation for last one year. Mother also noticed that her child had learning difficulties and not growing well like other peers. Her menarche had not started yet. Her elder two sisters and younger two brothers are healthy. She had no family history of rectal bleeding or colorectal cancer. She had no history of fever, abdominal pain, alteration of bowel habit, oral ulceration, weight loss and any joint manifestations. On examination, she was afebrile, mildly pale and vitally stable. Digital clubbing was present and skin finding was normal (Figure 1). Her height was 129 cm (severely stunted), mid-parental height 158 cm, target height 148-168 cm and weight 25 kg (underweight). On rectal examination, there were a few polypoid masses in the rectum.Examination of the cardiovascular, neurological and other systems revealed no abnormality. Complete blood count with erythrocyte sedimentation rate, C-reactive protein, serum albumin, prothrombin time and activated partial thromboplastin time reports all were normal except mild anemia (Table I). Stool routineexamination showed no feature of colitis. After evaluating the clinical data, physical findings and investigations results, the case was provisionally diagnosed as Juvenile colorectal polyp.Differential Diagnosis: Dr. Md. Benzamin: Patient presented with fresh bright red rectal bleeding for long duration and something coming out through anal verge after defecation. Patients also had severe growth failure and learning difficulties. Though history was quite long but the patient did not need blood transfusion. The child was mildly pale. Digital clubbing and severe growth retardation were present. The important clue to the diagnosis of juvenile colorectal polyp is characteristic rectal bleeding. After evaluating the other features, I thought about another differential diagnosis of Inflammatory bowel disease.Dr. Afsana Yasmin: After evaluating the patient’s presenting features, physical findings and the laboratory tests results, colonoscopy was done. Colonoscopy showed multiple polyps, about 30 mostly in the rectum and sigmoid colon and a few were in the descending and transverse colon. Most of the polypswere sessile and a few were pedunculated (Figure 1). Dr. Bishnu Pada Dey: Histopathology of the polyp tissues showed hamartomatous polyp with no dysplasia or malignant cells (Figure 2). Dr. Yasmin: We also did esophagogastroduodenoscopy which showed multiple sessile polyps in the stomach, mostly in the antrumwith the escape of esophagus and duodenum (Figure 1). We also did ultrasonography of pelvic organs which showed infantile uterus.Her luteinizing hormone was low and the follicle stimulating hormone was within normal range. Psychological assessment was done by the WISC-R which revealed mild mental retardation. According to the history, physical examination, colonoscopyfindings and histopathology of polyp, it was the case of gastrointestinal hamartomatous polyposis syndrome. There are four types of hamartomatous polyposis syndrome including juvenile polyposis syndrome, Bannayan Riley Ruvalcaba syndrome, Cowden syndrome and Peutz-Jegher syndrome. The clinical manifestations of all polyposis syndromes share same characteristics of intestinal juvenile polyps.Juvenile polyposis syndrome: 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 dueto 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, pulmonarystenosis, telangiectasias, Meckel’s diverticulum, intestinal malrotation, bifid uterus and vagina (female) and cryptorchidism (male).4 If extraintestinal features consistent with Cowden syndrome or Bannayan Riley Ruvalcaba syndrome are absent, then the diagnosis of juvenile polyposis syndrome is made when the following clinical criteria are present: a) more than five juvenile polyps of the colon or rectum, or; b) juvenile polyps in other parts of the gastrointestinal tract, or; c) any number of juvenile polyps and a positive family history.5 In the presenting case, presentations are rectal bleeding with associated abnormalities: growth failure, digital clubbing, mental retardation and infantile uterus, the presence of more than five juvenile polyps in the colorectum and also in the stomach. So, according to the JASS criteria, it was a case of juvenile polyposis syndrome if extraintestinal features of Cowden syndrome and Bannayan RileyRuvalcaba syndrome were excluded.Dr. Yasmin’s final Diagnosis: Juvenile polyposis syndrome
Bangladesh Med J. 2016 May; 45 (2)
Bangladesh Medical Journal
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. The 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 thalamostriatearteries .Angiographic study of these collateral vascular network give rise to characteristics appearance such as a puff of cigarette smoke. e Japanese term “MOYAMOYA” means “some thing hazy like a pu of cigarette smoke.Case report-1A three years old immunized girl of non consanguineous parents came to paediatric neurology of Bangabandhu Sheikh Mujib Medical University, Dhaka with left sided hemiperesis for 1 year. Mother stated that she developed seizure followed by left sided hemiplegia to extent that she unable to stand or walk. After this episode she gradually improved in standing & walking but weakness persist that made her walking dicult. Her perinatal and family historywas normal. Milestone of developement was age appropriate prior to this illness. ere was no history of fever, unconsiousness, vomiting, or aphasia along with this illness and no history of radiation exposure or head trauma mentioned before this. On examination she had no facial dysmorphism, vitals were within normal limit, skin survey-normal, no chest murmur. Neurological ndings revealed that she had hemiplegic gait, normal tone, power 4/5 on left side (both upper & lower limb) along with hyperreexia,exaggrarated ankle clonus & babinski sign positive. Cranial nerve & sensory function, cerebeller function were intact.Among the investigation, complete blood count, chest radiograph, ECG & EEG were normal. MRI of brain are image showed bilateral chronic cerebral ischemic changes, more remarkable on right side. (Figure-1) MRA showed gross narrowing of anterior cerebral, middle cerebral & posterior cerebral artery with extensive collaterals that appears as puff of smoke appearence suggestive of MOYAMOYA disease. (Figure-2 & 3) is patient was treated medically by nidipine as vasodilator and ecospirin and patient become gradually ambulant. Then we planned to discharge the patient after neurosurgical consultation.Case report- 2A 5 years old immunized boy of consanguineous mating parents came from rural area of Bangladesh presented with left sided hemiperesis for last 5 days, was not associated with fever, unconsiousness or aphasia. He had history of repeated attack of frontal , throbbing headache, associated with vomiting. He had an episode of chicken pox 1 month back. His perinatal history and milestone of development was normal. Examination ndings reveals that he had no facial dysmorphism, vitals were within normal limit ,multiple post vericella healed skin lesion found in whole body, no signs of meningeal irritationpresent there. Neurological ndings –hypotonia, grade 3/5 power on left side (both upper & lower limb) with areexia, bilateral planter exor, hemiplegic gait werepresent. Cranial nerve & sensory function, cerebeller functions were normal.lood glucose, Lipid prole, serum lactate, Blood ammonia,renal function tests, liver function tests, CXR & ECG were within normal limit. CT scan of brain shows-small infract in right parital region. MRI of brain T2 and air image multiple ow voids in the area of circle of willis and bilateral basal ganglia.(Figure-4&5) MR angiogram showed bussy change in the both parietal region. (Figure-6&7) He was diagnosed as moyamoya disease.e patient was treated by methylprednisolone for 3 days followed by oral steroid along with aspirin. Patient was gradually improved & discharged with advice for regular follow up.