Journal of Postgraduate Medicine, Education and Research, 2021
Nonalcoholic fatty liver disease (NAFLD) has emerged as the commonest liver disease in children. ... more Nonalcoholic fatty liver disease (NAFLD) has emerged as the commonest liver disease in children. The entire spectrum of NAFLD from simple steatosis to inflammation and fibrosis is seen in children. Pediatric NAFLD (pNAFLD) is most common in preadolescent and adolescent children and has the potential to cause severe liver-related as well as cardiovascular morbidity in young adulthood. The natural history of pNAFLD needs further elucidation. However, early diagnosis is imperative to prevent serious later outcomes. Lifestyle modification through diet and exercise remains the cornerstone in the management of pNAFLD. There is a great potential for the development of noninvasive biomarkers for diagnosis and novel pharmacotherapy to become available for the treatment of pNAFLD shortly.
Objective: The present study aimed to evaluate the potential benefits of ultrasonographic (USG) a... more Objective: The present study aimed to evaluate the potential benefits of ultrasonographic (USG) assessment in the diagnostic workup of children suspected of having celiac disease and its correlation with tissue transglutaminase (TTG) antibody assay. Materials and methods: A retro-prospective study, consisting 30 children with biopsy proven celiac disease diagnosis, was conducted. The tissue transglutaminase (tTG) assay was done on all the children, and further the children were subclassified based on the tTG titer values. The transabdominal sonography using convex (3-5 MHz) and linear (7-12 MHz) ultrasound probes was carried on the subjects, assessing the bowel lumen dimeter, bowel wall thickness, abnormal rugal folds count, presence of perihepatic fluid, fatty hepatic changes and mesenteric lymphadenopathy. Routine abdominal sonography was also carried out to complete the detailed evaluation of the abdomen. The sonographic bowel findings and the different classes of the tTG titers were correlated. Results: The median (IQR) age at onset of symptoms and at diagnosis was 6 (4-9) years. The median (IQR) value of blood tissue transglutaminase level (tTG) was 535 U/mL (63-7100), and most of the children belonged to class A Arch Clin Med Case Rep 2020; 4 (5): 978-989 DOI: 10.26502/acmcr.96550287 Archives of Clinical and Medical Case Reports 979 (n=18, 60%) subclassification followed by class C (n=7, 23.3%) and class B (n=5, 16.6%). On sonological evaluation, the bowel abnormalities including bowel thickening was seen in 8 (26.6%) of the children, followed by the reduced jejunal folds and transient intussusception in the same frequency (n=5, 16.6%) and, hyper-peristalsis in 4 (13.3%) children. No dilated bowel loops were seen. Extra-intestinal observations like fatty liver changes, mesenteric lymphadenopathy, and peritoneal free fluid were noted in 23 (76.6%), 6 (20%), and 4 (13.3%) of the children respectively. Conclusion: Ultrasound is the most widely used first imaging tool in the evaluation of abdominal ailments. Multiple small bowel abnormalities are found in the celiac patients, which may vary according to the study population's demographic and clinical features. More than half of the children with celiac disease showed small bowel abnormalities, and small bowel thickening, if present, may vary with the anti-tTg titer level. Multicentric and large size sample studies are required to accurately assess the association of serological antibodies titers with the bowel wall abnormality.
Introduction: Histological diagnosis is gold standard in many of the pediatric onset liver diseas... more Introduction: Histological diagnosis is gold standard in many of the pediatric onset liver diseases. The safety and efficacy of the liver biopsy is less studied in infants and children, even though it is more often performed than in adult. We retrospectively reviewed the patients who had undergone percutaneous liver biopsy (LB) to assess the safety and role in the diagnosis of liver diseases. Patients and methods: A total of 234 cases of ultrasonography (USG) guided percutaneous LBs under the age of 20 years were performed (Results: Cholestasis of infancy was the most common indication (47.4%), followed by suspected glycogen storage disorder (14.1%) and rest were patients of chronic liver diseases of various etiologies. LB confirmed the specific etiologies of cholestasis of infancy in more than 90% of cases and the procedure was safe in young infants. Overall, most common confirmed etiologies confirmed were congenital hepatic fibrosis (16.2%), glycogen storage disorder (11.5%), progressive familial intrahepatic cholestasis (16.7%) and biliary atresia (6.4%). The proportion of confirmed cases were significantly more among infants < 3 months as compared to older children (82.6% vs. 39.7%; p<0.001). Arch Clin Med Case Rep 2020; 4 (5): 932-939 Conclusion: Liver biopsy in infants and children is a safe procedure, with high yield and very low rate of serious complication (1.2%).
Introduction Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of ... more Introduction Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of care in pediatric age group. Robotic-assisted excision of CC is increasingly being practiced at centers which have access to the system. We present our experience and technique of hepaticoduodenostomy (HD). Over all initial experience, short-term outcomes and complications are also presented and discussed. Materials and Methods Patients with CC and undergoing robotic excision were retrospectively studied. Patients with active cholangitis, liver dysfunction, and perforated CC were excluded for robotic procedures. All included patients were preoperatively evaluated as per the defined protocol. They underwent excision of CC with HD. The duodenal anastomosis was done after limited mobilization and emphasis was laid on anastomosing the distal D2 part to the common hepatic duct. This prevents bile reflux into stomach. The follow-up evaluation was done for these patients. Hepatobiliary iminodia...
Eosinophilic ascites, owing to serosal involvement, is a very rare manifestation of eosinophilic ... more Eosinophilic ascites, owing to serosal involvement, is a very rare manifestation of eosinophilic gastroenteritis in children, especially when it occurs with muscular involvement in the absence of mucosal disease, which may be confirmed by endoscopic ultrasonography. An 11-year-old girl, presenting with massive eosinophilic ascites and colicky abdominal pain with peripheral eosinophilia, raised IgE levels and positive skin prick test, had such investigation which confirmed the presence of muscle layer thickening of both stomach and small bowel. She responded well to steroids and montelukast.
Chronic abdominal pain without red flag signs is usually functional abdominal pain and does not r... more Chronic abdominal pain without red flag signs is usually functional abdominal pain and does not require investigation. We encountered an eight-year-old boy who was subsequently diagnosed with a retroperitoneal ganglioneuroma. In view of the rarity of this tumour and its presentation, we are prompted to report this case.
Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe th... more Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe the clinical characteristics of an uncommon inherited cause of chylous ascites, Hennekam syndrome, treated by nutritional modification.
Journal of Postgraduate Medicine, Education and Research, 2021
Nonalcoholic fatty liver disease (NAFLD) has emerged as the commonest liver disease in children. ... more Nonalcoholic fatty liver disease (NAFLD) has emerged as the commonest liver disease in children. The entire spectrum of NAFLD from simple steatosis to inflammation and fibrosis is seen in children. Pediatric NAFLD (pNAFLD) is most common in preadolescent and adolescent children and has the potential to cause severe liver-related as well as cardiovascular morbidity in young adulthood. The natural history of pNAFLD needs further elucidation. However, early diagnosis is imperative to prevent serious later outcomes. Lifestyle modification through diet and exercise remains the cornerstone in the management of pNAFLD. There is a great potential for the development of noninvasive biomarkers for diagnosis and novel pharmacotherapy to become available for the treatment of pNAFLD shortly.
Objective: The present study aimed to evaluate the potential benefits of ultrasonographic (USG) a... more Objective: The present study aimed to evaluate the potential benefits of ultrasonographic (USG) assessment in the diagnostic workup of children suspected of having celiac disease and its correlation with tissue transglutaminase (TTG) antibody assay. Materials and methods: A retro-prospective study, consisting 30 children with biopsy proven celiac disease diagnosis, was conducted. The tissue transglutaminase (tTG) assay was done on all the children, and further the children were subclassified based on the tTG titer values. The transabdominal sonography using convex (3-5 MHz) and linear (7-12 MHz) ultrasound probes was carried on the subjects, assessing the bowel lumen dimeter, bowel wall thickness, abnormal rugal folds count, presence of perihepatic fluid, fatty hepatic changes and mesenteric lymphadenopathy. Routine abdominal sonography was also carried out to complete the detailed evaluation of the abdomen. The sonographic bowel findings and the different classes of the tTG titers were correlated. Results: The median (IQR) age at onset of symptoms and at diagnosis was 6 (4-9) years. The median (IQR) value of blood tissue transglutaminase level (tTG) was 535 U/mL (63-7100), and most of the children belonged to class A Arch Clin Med Case Rep 2020; 4 (5): 978-989 DOI: 10.26502/acmcr.96550287 Archives of Clinical and Medical Case Reports 979 (n=18, 60%) subclassification followed by class C (n=7, 23.3%) and class B (n=5, 16.6%). On sonological evaluation, the bowel abnormalities including bowel thickening was seen in 8 (26.6%) of the children, followed by the reduced jejunal folds and transient intussusception in the same frequency (n=5, 16.6%) and, hyper-peristalsis in 4 (13.3%) children. No dilated bowel loops were seen. Extra-intestinal observations like fatty liver changes, mesenteric lymphadenopathy, and peritoneal free fluid were noted in 23 (76.6%), 6 (20%), and 4 (13.3%) of the children respectively. Conclusion: Ultrasound is the most widely used first imaging tool in the evaluation of abdominal ailments. Multiple small bowel abnormalities are found in the celiac patients, which may vary according to the study population's demographic and clinical features. More than half of the children with celiac disease showed small bowel abnormalities, and small bowel thickening, if present, may vary with the anti-tTg titer level. Multicentric and large size sample studies are required to accurately assess the association of serological antibodies titers with the bowel wall abnormality.
Introduction: Histological diagnosis is gold standard in many of the pediatric onset liver diseas... more Introduction: Histological diagnosis is gold standard in many of the pediatric onset liver diseases. The safety and efficacy of the liver biopsy is less studied in infants and children, even though it is more often performed than in adult. We retrospectively reviewed the patients who had undergone percutaneous liver biopsy (LB) to assess the safety and role in the diagnosis of liver diseases. Patients and methods: A total of 234 cases of ultrasonography (USG) guided percutaneous LBs under the age of 20 years were performed (Results: Cholestasis of infancy was the most common indication (47.4%), followed by suspected glycogen storage disorder (14.1%) and rest were patients of chronic liver diseases of various etiologies. LB confirmed the specific etiologies of cholestasis of infancy in more than 90% of cases and the procedure was safe in young infants. Overall, most common confirmed etiologies confirmed were congenital hepatic fibrosis (16.2%), glycogen storage disorder (11.5%), progressive familial intrahepatic cholestasis (16.7%) and biliary atresia (6.4%). The proportion of confirmed cases were significantly more among infants < 3 months as compared to older children (82.6% vs. 39.7%; p<0.001). Arch Clin Med Case Rep 2020; 4 (5): 932-939 Conclusion: Liver biopsy in infants and children is a safe procedure, with high yield and very low rate of serious complication (1.2%).
Introduction Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of ... more Introduction Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of care in pediatric age group. Robotic-assisted excision of CC is increasingly being practiced at centers which have access to the system. We present our experience and technique of hepaticoduodenostomy (HD). Over all initial experience, short-term outcomes and complications are also presented and discussed. Materials and Methods Patients with CC and undergoing robotic excision were retrospectively studied. Patients with active cholangitis, liver dysfunction, and perforated CC were excluded for robotic procedures. All included patients were preoperatively evaluated as per the defined protocol. They underwent excision of CC with HD. The duodenal anastomosis was done after limited mobilization and emphasis was laid on anastomosing the distal D2 part to the common hepatic duct. This prevents bile reflux into stomach. The follow-up evaluation was done for these patients. Hepatobiliary iminodia...
Eosinophilic ascites, owing to serosal involvement, is a very rare manifestation of eosinophilic ... more Eosinophilic ascites, owing to serosal involvement, is a very rare manifestation of eosinophilic gastroenteritis in children, especially when it occurs with muscular involvement in the absence of mucosal disease, which may be confirmed by endoscopic ultrasonography. An 11-year-old girl, presenting with massive eosinophilic ascites and colicky abdominal pain with peripheral eosinophilia, raised IgE levels and positive skin prick test, had such investigation which confirmed the presence of muscle layer thickening of both stomach and small bowel. She responded well to steroids and montelukast.
Chronic abdominal pain without red flag signs is usually functional abdominal pain and does not r... more Chronic abdominal pain without red flag signs is usually functional abdominal pain and does not require investigation. We encountered an eight-year-old boy who was subsequently diagnosed with a retroperitoneal ganglioneuroma. In view of the rarity of this tumour and its presentation, we are prompted to report this case.
Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe th... more Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe the clinical characteristics of an uncommon inherited cause of chylous ascites, Hennekam syndrome, treated by nutritional modification.
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