Clinical spectrum & changing presentation of celiac disease in Indian children.

Background & objectives: Celiac disease (CD) is a genetic immune mediated disorder characterised by gluten intolerance. This single centre study, from north India was aimed to assess the clinical, serological and histological profile of CD in a large cohort of children and the changing trends in its presentation. Methods: A review of clinical details of CD children diagnosed between 2000 and 2019 and currently on follow up was performed. Information on demography, symptoms, associated conditions, serology, biopsy findings and gluten-free diet were analyzed. Results: The mean age (±standard deviation) of 891 children included in the study, at onset and at diagnosis was 4.0±2.7 and 6.2±3.1 yr, respectively. Growth faltering, abdominal pain, abdominal distension and diarrhoea were presenting symptoms in 70, 64.2, 61.2 and 58.2 per cent, respectively. A positive family history of CD was present in 14 per cent and autoimmune conditions in 12.3 per cent of children. Thyroid disorders were seen in 8.5 per cent of children and Type 1 diabetes mellitus (T1DM) in 5.7 per cent. The duration of breastfeeding had a weak positive correlation with age at onset and diagnosis of CD (P<0.001). Non-classical CD was significantly more common in children aged >10 yr and in those presenting after 2010 (P<0.01). T1DM and hypothyroidism occurred more frequently in non-compliant children. Interpretation & conclusions: This was the largest single centre study, pertaining to the presentation and follow up of CD in children. Infants and young children were more likely to present with classical symptoms of diarrhoea, abdominal distension and growth failure while older children presented with non-classical CD. There was a trend towards non-classical forms of CD in recent years.

Knowledge, Awareness and Prevalence of Hepatitis B Among Urban Slum Dwellers and Residents of Social Welfare Home: A Cross sectional Study From Eastern India.

Background/aims: In view of limited data on the knowledge and awareness of hepatitis B virus (HBV) and the available preventive strategies at the community level, it was aimed to analyse the knowledge and awareness of HBV in the community. Methods: A cross-sectional questionnaire-based survey was conducted among residents of an urban slum and a social welfare home in Bhubaneswar, Odisha, from October 2019 to April 2021. The prevalence of HBV infection was also measured by testing the serum positivity for hepatitis B surface antigen using rapid point-of-care test kits. The statistical analysis was done by using the software SPSS version 20. Results: A total of 370 individuals (mean age 38.7 ± 14.9 years, males: 55.1%) were assessed. Although 18.1% (67) had good knowledge, only 16.7% (62) had good awareness about HBV. Approximately 14.8% (55) knew that a vaccine is available in the country for HBV, and 6.2% (23) identified themselves as being vaccinated. Educational status was a significant independent predictor of knowledge and awareness such that people with education level of matriculation and above had odds of 11.05 (95% confidence interval: 5.3-22.7) and 14.7 (95% confidence interval: 6.5-33.1) for having good knowledge and awareness regarding HBV, respectively. A total of 10 participants tested positive for hepatitis B surface antigen contributing to a point prevalence rate of 2.7%. The proportion of individuals with an education status of matriculation and above was higher in the slum area when compared with the welfare home (67% vs 33%; P < 0.001), the knowledge (71.6% vs 28.4%; P < 0.001) and so was the awareness (71% vs 29%; P < 0.001) about HBV as well. Conclusion: The relatively low figures of knowledge and awareness identified in our study undermine the need for intensification of health education and promotion activities regarding the prevalence of hepatitis B infection on a large scale at the community level.

Treatment of hepatitis C in children and adolescents: how far have we reached?

BACKGROUND: Hepatitis C virus (HCV) infection is a global public health problem and also generates a significant case load in children and adolescents. With the introduction of directly acting antivirals (DAA), the treatment and care of HCV-infected patients have progressed significantly. The available treatment options in children are limited, and this review aims to provide an overview of treatment of HCV infection in children and adolescents with the current available DAA regimens. DATA SOURCES: This comprehensive review was undertaken after searching the PubMed/Medline and Embase databases for the available up-to-date literature on pediatric HCV infection and treatment using hepatitis C virus infection/HCV, directly acting antivirals/DAA, natural history, treatment, pediatrics, children, and adolescents as keywords. RESULTS: Combination therapies with highly effective DAA regimes, such as sofosbuvir/ledipasvir, sofosbuvir/velpatasvir, glecaprevir/pibrentasvir, sofosbuvir/daclatasvir, sofosbuvir/ribavirin and others, are available for use in children. Most of the DAA regimens have either received or are pending to receive regulatory approval by different medical/drug agencies for use in children and adolescents. Pan-genotypic regimens are also available in children and adolescents, and these regimens can be used while skipping genotype testing. CONCLUSION: The literature on different DAA regimens for use in children shows that these regimens have higher cure rates with minimal side effects and shorter duration of therapy.

Morphological and functional recovery following acute and acute recurrent pancreatitis in children: A prospective sequential 2-point evaluation.

BACKGROUND: The functional and morphological recovery following an episode of acute pancreatitis (AP) in children still remains ill understood as research exploring this is limited. We aimed to characterize the morphological and functional changes in pancreas following AP and ARP (acute recurrent pancreatitis) in children. METHODS: Children with AP were followed prospectively and assessed at two time points at least 3 months apart, with the first assessment at least 3 months after the AP episode. Exocrine and endocrine functions were measured using fecal elastase and fasting blood sugar/HbA1c levels respectively. Morphological assessment was done using endoscopic ultrasound (EUS) and magnetic resonance imaging and cholangiopancreatography (MRI/MRCP). RESULTS: Seventy-three children (boys:59%; mean age:8.4 ± 3.2years) were studied and 21 of them (29%) progressed to ARP. Altered glucose homeostasis was seen in 19 (26%) at first and 16 (22%) at second assessment and it was significantly more in ARP group than the AP group at first (42.8%vs19.2%; p = 0.03) as well as second assessment (38.1%vs15.3%; p = 0.03). Twenty-one children (28.7%) at first and 24 (32.8%) at second assessment developed biochemical exocrine pancreatic insufficiency. EUS detected indeterminate and suggestive changes of chronic pancreatitis in 21% at first (n = 38) and 27.6% at second assessment (n = 58). On MRCP, main pancreatic duct and side branch dilatation were seen in 15 (20.5%) and 2 (2.7%) children respectively. CONCLUSIONS: More than one-quarter of children have evidence of altered glucose homeostasis and biochemical exocrine pancreatic insufficiency following an episode of AP. Similarly, morphological features of chronicity seen in some of the children suggest that a fraction of subjects may develop chronic pancreatitis on longer follow-up.

Portal Cavernoma Cholangiopathy in Children: An Evaluation Using Magnetic Resonance Cholangiography and Endoscopic Ultrasound.

BACKGROUND: Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra- and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extrahepatic portal venous obstruction (EHPVO) using endoscopic ultrasound (EUS) and magnetic resonance cholangiography/portovenography (MRC/MRPV). METHODS: A total of 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity. RESULTS: All 53 children (100%) had PCC changes on MRC/EUS, but none were symptomatic. Extrahepatic ducts (EHDs) and intrahepatic ducts were involved in majority (85%), and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intrahepatic ducts, whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the EHDs. Increased CBD angulation predisposed to CBD strictures (P = 0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic, and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade. CONCLUSIONS: PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC.

Gallbladder motility in children with celiac disease before and after gluten-free diet.

BACKGROUND: Gallbladder (GB) hypomotility has been reported in adults with celiac disease (CD), but there is no literature on GB dysfunction in children with CD. We aimed to study GB motility in children with CD, before and after a gluten-free diet (GFD), using ultrasonography (USG) and technetium-99 labeled mebrofenin hepatobiliary scintigraphy (HBS). METHODS: Children with newly diagnosed CD were enrolled and evaluated for GB ejection fraction (GBEF) using HBS and USG. Those with reduced GBEF on initial HBS were again evaluated after 6 months of strict GFD and the results were compared. RESULTS: Of the 50 children with CD (mean age 9±2.1 years, 54% boys), 16% (n=8) had a low GBEF at baseline (19±13%). These children had a significantly greater delay in diagnosis compared to those with normal GBEF (6.5±2.0 vs. 2.3±1.2 years, P<0.001). A significant improvement in GBEF was noted on HBS post GFD (74±12%1 vs. 9±13%, P<0.001). GBEF also improved significantly as assessed by USG parameters after GFD (P<0.001). The fasting GB volume decreased (5.7±1.4 vs. 10.2±2.6, P=0.002), with a significant improvement in percentage postprandial GB volume change (52±10.5% vs. 24±16.5%, P=0.007) compared to baseline pre-GFD values. Orocecal transit time was also increased in children with reduced GBEF. CONCLUSIONS: GB function is impaired in at least 16% of children with CD at diagnosis and is reversible with GFD. GB dysfunction is significantly associated with a delayed diagnosis and may be a part of general gastrointestinal dysmotility.

Liver Abscess in Children-experience From a Single Tertiary Care Center of North India: Etiology, Clinical Profile and Predictors of Complications.

BACKGROUND: Liver abscess (LA), a common problem in children in the tropics, is believed to be mostly pyogenic (PLA), sometimes amebic (ALA). We aimed to analyze the clinical profile, etiology, risk factors for complications, management and outcomes of LA in children. METHODS: The details of 81 children with LA managed in a tertiary set up over a period of 3 years were analyzed. A comparison of different parameters was performed with respect to etiology and complications. RESULTS: ALA, PLA and mixed infection LA were diagnosed in 40 (49.4%), 32 (39.5%) and 9 (11.1%) children. The triad of fever, hepatomegaly and right upper quadrant tenderness was seen in 65 (80.2%). Coagulopathy was observed in 60 (77%) and jaundice in 12 (14.8%). Majority (71.6%) had a single LA in the right lobe (69%). Conservative, percutaneous needle aspiration, percutaneous catheter drainage and surgical drainage were done in 11.1%, 3.7%, 82.7% and 2.5%, respectively. Forty-three (53.1%) had complicated LA with rupture in 55.8% and vascular thrombosis in 16.2%. Children with complicated LA had higher alanine transaminase, prolonged prothrombin time/international normalized ratio, low serum protein and albumin levels (P < 0.05). Median duration of follow-up was 2 months and mean time to resolution of LA was 48.5 ± 18 days. CONCLUSIONS: ALA is the commonest cause of pediatric LA in endemic regions and is difficult to differentiate from PLA clinically. Percutaneous catheter drainage is safe and effective modality for the management of LA in children. A higher alanine transaminase, prolonged prothrombin time/international normalized ratio and low serum albumin levels (<3 g/dL) at presentation identify complicated LA.

Necrotizing Enterocolitis Following Pediatric Living Donor Liver Transplant.

Necrotizing enterocolitis is one of the most common and worrying diseases in neonates, commonly shown in premature neonates, and is associated with significant mortality and morbidity. Necrotizing enterocolitis is characterized by intestinal mucosal injury that can progress to transmural bowel necrosis, and radiologically it can present with either pneumatosis intestinalis or portal venous gas. It is postulated to develop in an immunocompromised host in the setting of bacterial colonization, usually after administration of non-breast milk feed. Cow's milk allergy association with necrotizing enterocolitis has not been well determined, and the pathophysiology is still not clear. Necrotizing enterocolitis is very rare following living donor liver transplant. In our case, a 6-year-old boy who was doing well in the postoperative period had sudden worsening of general condition after he was started on milk feed. On evaluation and reexploration, he was diagnosed with necrotizing enterocolitis and later succumbed to death.

Pulmonary thromboembolism: A rare complication of amoebic liver abscess in a child.

Amoebic liver abscess is common in children in developing countries due to lack of hygiene and sanitary conditions. Inferior vena cava thrombosis is a rare complication of this disease, with only a few cases reported in the literature, where this thrombus led to pulmonary thromboembolism. We report the case of a 7-year-old child with amoebic liver abscess who developed pulmonary thromboembolism and was promptly diagnosed and managed.

Pancreatic ascites and Pleural Effusion in Children: Clinical Profile, Management and Outcomes.

BACKGROUND: Pancreatic ascites (PA) and pleural effusion (PPE) are rarely encountered in children. They develop due to disruption of the pancreatic duct (PD) or leakage from an associated pancreatic fluid collection (PFC). The literature on childhood PA/PPE and its management is scarce. METHODS: A retrospective review of children with PA/PPE diagnosed and managed at our center over the last 4 years was performed. The clinical, biochemical, radiological and management profiles were analyzed. Conservative management included nil per oral, octreotide and drainage using either percutaneous catheter or repeated paracentesis. Endotherapy included endoscopic retrograde cholangiopancreatography (ERCP) and transpapillary stenting. RESULTS: Of the 214 children with pancreatitis, 15 (7%) had PA/PPE. Median age was 9 years with a third under 2 years. Median ascitic fluid amylase was 8840 U/L and all had elevated protein (>2.5 g/dl) and low serum ascites-albumin gradient ascites (<1.1). While PA/PPE was the first manifestation of underlying chronic pancreatitis (CP) in 10 children (67%), trauma was seen in 4 (26%) and hypertriglyceridemia in 1 (7%). On imaging, PD disruption could be identified in 10 (67%) children. ERCP and stenting was done in 10 children. Conservative management alone (n = 4) and endotherapy (n = 10) was successful in 93% with only one requiring surgery. The younger children (n = 4), were managed conservatively and only 1 of them required surgery. Resolution of PA/PPE was achieved in all with no recurrences. CONCLUSIONS: Conservative management and ERCP plus transpapillary stenting results in resolution of majority of pediatric PA/PPE. Children presenting with PA/PPE needs to be evaluated for CP.

Abdominal tuberculosis in children: A real-world experience of 218 cases from an endemic region.

BACKGROUND AND AIM: Abdominal tuberculosis (ATB) in children poses a diagnostic challenge because of its nonspecific clinical features, which often delay the diagnosis. Our aim was to present our real-world experience and provide an insight into the presentation, pattern of distribution, and diagnosis of the disease. METHODS: A retrospective review was conducted of case records of all children ≤12 years of age diagnosed with ATB from January 2007 to January 2018. Clinical details and investigations were recorded and analyzed. RESULTS: A total of 218 children (110 boys), with a median age of 10 (0.25-12) years, were included. There was a median delay of 4 (0.5-36) months in establishing the diagnosis. Abdominal pain, fever, and loss of weight were the most common presenting features, with the triad of symptoms present in 54%. Multiple intra-abdominal sites were involved in 118 (54%) patients, with a combination of the gastrointestinal tract (I) and abdominal lymph nodes (L) being the most common (53/118). Among children with single-site involvement (n = 100), the most commonly involved was L in 39 (39%), followed by I in 35(35%). Loss of weight was more common in children with involvement of multiple sites (85/118 vs 60/100, P = 0.03). Overall, a confirmed diagnosis was possible in 94 participants (43.1%). Suggestive imaging had the highest diagnostic yield of 85%. Nine (4.1%) patients needed surgical management. CONCLUSION: A triad of abdominal pain, fever, and weight loss is suggestive of ATB. Multiple intra-abdominal sites are frequently involved. Microbiological confirmation is possible in only one-third of the cases.

Paediatric acute pancreatitis: Clinical profile and natural history of collections.

BACKGROUND & AIMS: Acute Pancreatitis (AP) tends to have a benign course in children. However there is a paucity of information with respect to severity of AP in children, the categorization of collections viz., walled off necrosis (WON)& pseudocyst and their natural history & outcomes. METHODS: A retrospective medical record review of 187 children with pancreatitis diagnosed and managed at our centre was performed. RESULTS: 101 children (59% boys, Median age 9yrs) had AP of which 37.6%, 60.4% and 2% had mild, moderately severe and severe AP. 61.4%(62) had PFC at diagnosis; 34%(21) acute pancreatic fluid collections (APFC) and 66%(41) acute necrotic collections (ANC). 52.3%(11of21) of APFC evolved into pseudocysts & 68.2%(28of41) of ANC into WON's. Drainage was required in 31%(12of39) of persisting collections, more frequently in children with traumatic AP. Percutaneous catheter drainage (PCD) was done in 6 children and endoscopic ultrasound (EUS) guided cystogastrostomy with placement of plastic or self expanding metal stents (SEMS) in 6 children. CONCLUSIONS: Moderately severe AP is common in hospitalized children with AP with PFC developing in 61.4%, majority being APFC. 48% of APFC and 32% of ANC will resolve and the rest evolve into pseudocyst or WON. Spontaneous resolution is more likely in children with non -traumatic AP having pseudocysts rather than WON's.

Ascites: an unusual presentation of eosinophilic gastroenteritis in a child.

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.

Bilateral renal cortical necrosis in a child with acute pancreatitis.

Bilateral renal cortical necrosis (RCN) as a cause of acute kidney injury is very rare in the pediatric population. Progression to end-stage renal disease is seen virtually in every patient with RCN. There are many causes for the occurrence of cortical necrosis in children, with severe pancreatitis being a rarity. In this report, we describe a child with severe acute pancreatitis complicated by bilateral RCN.

Hennekam syndrome: an uncommon cause of chylous ascites and intestinal lymphangiectasia in the tropics.

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.