Risk Factors And Treatment Outcome Of Children With HCV Infection.

BACKGROUND: Combination of pegylated interferon α-2a or α-2b has been considered to be the standard for treating HCV infection among children. Many new agents inhibiting HCV during various steps while replicating is under study around the world. This study was aimed to note the efficacy of sofosbuvir and ribavirin among children having HCV infection. METHODS: This was an open label experimental trail done at Department of Gastroenterology, Children Hospital and The Institute of Child Health, Multan. The study duration was from July to December 2019. A total of 89 HCV treatment naïve children aged 6-16 years of age, having HCV PCR as positive were enrolled. Sofosbuvir as 400 mg once a day along with ribavirin 10-15 mg per kg per day in the form of once or twice as divided doses were given in all the cases. After starting the treatment, along with side effects, rapid virological response (RVR) as PCR at 4 weeks, early virological response (EVR) at 12 weeks and post treatment 12 weeks HCV PCR as sustained virological response (SVR) was noted. RESULTS: Out of a total of 89 children, there were 53 (59.6%) boys and 36 (40.4%) girls. Mean age was noted to be 12.42±2.57 years. Majority of the children, 72 (80.9%) had genotype 3 while genotype 1 was noted in 11 (12.4%) and un-typable in remaining 6 (6.7%). History of blood or blood products transfusion was seen to be the commonest mode of HCV transmission, found in 41 (46.1%) children, perinatal transmission in 20 (22.5%) and history of previous surgery in 9 (10.1%). Rapid virological response was noted in 73 (83.1%) children, all 89 (100%) children achieved EVR whereas SVR was noted in 86 (96.7%). Headache was the commonest side effect, reported by 24 (27.0%) followed by nausea in 15 (16.9%). CONCLUSIONS: Regardless of the genotype, sofosbuvir and ribavirin combination therapy was noted to have excellent efficacy amongst children with HCV infection. History of blood and blood product transfusion was the commonest risk factor found.

Etiologies and outcome of lower gastrointestinal bleeding in patients presenting to a tertiary care Children's Hospital.

OBJECTIVE: To determine etiology and outcome of children with lower gastrointestinal bleeding (LGIB). METHODS: This was a prospective study conducted at the Department of Pediatric Gastroenterology and Hepatology, Children's Hospital and The Institute of Child's Heath, Multan, Pakistan, from July 2019 to March 2020. A total of 148 cases presented with bleeding per rectum and underwent colonoscopy, were included. Children of both genders and aged three month to 15 years were included. Detailed history, clinical examination, laboratory studies, colonoscopy and histopathology were done in all cases. Study information like demographics, complaints, general clinical examination, colonoscopy and histopathological findings were recorded. RESULTS: Overall, mean age was noted to be 7.20±1.83 years. Abdominal pain was reported in 41 (27.7%), diarrhea 36 (24.3%), fever 12 (8.1%) and constipation in 4 (2.7%). Pallor was noted among 68 (45.9%), weight loss 39 (26.3%) and tachycardia 31 (20.9%). Colonoscopy revealed juvenile colonic / rectal polyps, infectious colitis and solitary rectal ulcer (SRU) as the most common etiologies found among 58 (39.2%), 20 (13.5%) and 19 (12.8%) cases respectively. Juvenile polyps and non-specific colitis were the commonest histopathological findings seen in 55 (37.2%) and 20 (13.5%) cases respectively. Colonoscopic polypectomy was used to remove all juvenile polyps. CONCLUSION: LGIB is presentation of various underlying causes. Children with LGIB commonly present with abdominal pain. Juvenile polyps were the most frequent cause of LGIB among children flowed by non-specific colitis. Most of the children having LGIB were diagnosed and treated successfully, few are in remission and very few were found resistant to treatment.

Is Hepatovenocaval Syndrome a Different Entity from Budd-Chiari Syndrome in Children?

OBJECTIVE: To differentiate between clinical and demographic spectrum, and outcome in hepatovenocaval syndrome (HVCS) and Budd-Chiari syndrome (BCS). STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital, Lahore, from January 2014 to January 2017. METHODOLOGY: All children less than 18 years of age, presenting with ascites and visible veins over abdomen, flanks and back were enrolled in the study. Real time Doppler Ultrasonogram was performed in all children for documentation of intra- hepatic part of IVC obstruction along with or without hepatic venous obstruction. Children meeting inclusion criteria underwent liver profile, coagulation profile, diagnostic paracentesis for SAAG gradient, and Gadolinium enhanced multiphasic MR scan. Liver biopsy and venography was performed in selected patients. RESULTS: A total of 92 children presented with ascites, among them 58 children met our inclusion criteria. Intrahepatic IVC obliteration, i.e. HVCS, found in 67% (n=39) and hepatic venous outflow obstruction, i.e. BCS was found in 33% (n=19) children. Children with BCS were older than HVCS with mean age of 9.5 ±2.58 versus 4.12 ±0.977 years. HVCS group had 14 boys and 24 girls with a ratio of 1:1.8, while BCS had a ratio of 1:0.9 with 10 boys and 9 girls. No etiological factor was found for HVCS, while most of patients with BCS had a procoagulant disorder. Caudate lobe hypertrophy was a consistent feature in BCS, while IVC obstruction was found in HVCS persistently. Orthotopic liver transplant was needed in three cases (7.6%) of HVCS and four (20.96%) of BCS cases. Antibiotic therapy has a good role in HVCS, while anticoagulation and diuretics had good result in BCS. CONCLUSION: Hepatovenocaval syndrome (HVCS) mostly affected younger children, especially girls. BCS usually affected older age groups with pro-coagulant disorders who responded to anticoagulation and diuretic. Further studies are needed to compare both conditions.

Chanarin-Dorfman Syndrome.

Chanarin-Dorfman syndrome is a rare, genetically determined autosomal recessive disorder, characterised by the presence of lipid droplets in the cytoplasm of multiple tissues of the body, particularly in the blood leukocytes and congenital non-bullous icthyosiform erythroderma. In this paper, we report one-year child who presented with skin lesions since birth and hepatomegaly. Liver biopsy showed steatohepatitis; and peripheral blood smear confirmed Jordan`s anomaly, which is a permanent feature of Chanarin-Dorfman syndrome.

Celiac Crisis: A Rare Or Rarely Recognized Disease.

BACKGROUND: Celiac crisis is a serious life threatening complication of celiac disease characterized by profuse diarrhoea, severe dehydration and metabolic disturbances leading to neuromuscular weakness, cardiac arrhythmias and sudden death. It has been described as rare condition and not well documented in the literature. To improve awareness and facilitate diagnosis of this condition, we studied risk factors, pattern of presentation and management plans of celiac crisis. METHODS: It was a descriptive cross sectional study. Patients presenting in emergency room(ER) with profuse diarrhoea leading to severe dehydration, neuromuscular weakness, and metabolic acidosis and electrolyte abnormalities enrolled in the studies after positive serology and small bowel biopsy suggestive of celiac disease. RESULTS: Total 126 patients out of 350 fulfilled the criteria including 54 (42.8%) male and 71 (56.3%) female. The mean age at presentation was 5.25±1.18 years. Risk factors were poor social status (97.60%), consanguinity (96.77%), early weaning with gluten contained diet (93.54%), and Presenting complaints were loose motion (100%), loss of neck holding (96.77%), dehydration (96.77%), polyuria (95.96%), inability to walk (67.74%), abdominal distension (85.86%). Electrolytes imbalances were hypokalaemia (2.4±0.55), hypocalcaemia (7.29±0.66), hypomagnesaemia (1.89±0.50), hypophosphatemia (2.8±0.68), hypoalbuminemia (3.05±0.48) and metabolic acidosis (96%). One hundred & twenty patients were stabilized with GFD and correction of dehydration, acidosis and electrolyte imbalance. Six patients needed parenteral steroids ant total parenteral nutrition (TPN). Recovery time from crisis was mean 5.4±2.73 days (range 3-20 days). CONCLUSIONS: Celiac crisis is a common but under recognized problem in developing countries. Commonest presenting feature is neuromuscular paralysis and biochemical abnormality is hypokalaemia.

CLINICAL PRESENTATION, AETIOLOGY AND COMPLICATIONS OF PANCREATITIS IN CHILDREN.

BACKGROUND: Childhood Pancreatitis is an uncommon but serious condition with incidence on the rise. It manifests as acute or chronic form with epigastric pain, vomiting and elevated serum -amylase and lipase. This study was conducted with the aim to determine the clinical presentation, aetiology, and complications of pancreatitis in children. METHODS: This descriptive case series was conducted in the Department of Paediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital & the Institute of Child Health, Lahore from 1st January to 31st December 2014. Seventy-two patients up to the age of 15 years having abdominal pain, Amylase >200 IU/L and/or lipase >165 IU/L, with features of acute or chronic pancreatitis on abdominal imaging; were included in study. Data analysis was done using SPSS-20. RESULTS: Of the total 72 patients, 43 (60%) had acute pancreatitis, males were 25 (58%) and females 18 (42%) and chronic pancreatitis was diagnosed in 29 (40%), males 10 (34%) and females 19 (66%). Common clinical features were abdominal pain (100%), nausea and vomiting (79%). Common aetiologies were idiopathic (40%) while choledochal cyst 8%, hyperlipidaemia 7%, biliary tract stones/sludge 7% and abdominal trauma 6%. Complications were more frequently associated with acute pancreatitis (60%) than with chronic pancreatitis (34%). Common complications were pseudo-pancreatic cyst (36%), ascites (17%) and pleural effusion (4%). CONCLUSION: Abdominal pain, nausea and vomiting were common presenting features of childhood pancreatitis. Common aetiologies were idiopathic hyperlipidemia, biliary tract stones/sludge, choledochal cyst and abdominal trauma. Common complications were Pseudo-pancreatic cyst, ascites and pleural effusion.