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Iron deficiency anaemia is a known complication of a large hiatal hernia in adults. It occurs as a result of erosions on the gastric mucosa secondary to traction at the hiatus during respiration and/or gastric acid-related injuries to the mucosa. Even though anaemia occurs as a result of chronic gastrointestinal blood loss, testing for faecal occult blood is often negative and upper gastrointestinal endoscopy normal as the bleeding is intermittent. In children, a hiatus hernia as a rare cause of iron deficiency anaemia and has been described only in case reports. Here, we describe a 5-year-old boy who presented with severe transfusion-dependent iron deficiency anaemia caused by a paraoesophageal hernia. Surgical repair of the hiatus hernia led to complete resolution of anaemia. One should consider a hiatus hernia as a diagnostic possibility when evaluating a child with refractory iron deficiency anaemia.

WHO defines malnutrition as severe if the z-scores are less than − 3 Standard deviation (SD), moderate if between − 2 and − 3 SD and mild if between − 2 SD to – 1 SD. This study was aimed to assess nutritional aspects of Indian children suffering from EB and to evaluate the effect of severity of EB on the severity of malnutrition. In this study, pediatric EB patients were evaluated prospectively for baseline nutritional status using anthropometric parameters and WHO growth charts, and its correlation with disease severity using instrument for Scoring Clinical Outcomes for Research of Epidermolysis Bullosa-iscorEB. In second phase, an individualized diet chart was given to meet the energy, protein and micronutrients needs and its effects were observed after 6 months. The median age of participants was 3 years (IQR-9). Of 57 patients, malnutrition was seen in 40.35% patients (22.81%-moderate and 17.54%-severe), and significantly correlated with iscorEB (r = 0.45, p

Biliary atresia (BA) is a progressive destructive cholangiopathy of unknown etiology that presents in early infancy. It has a worldwide frequency of 1:8,000–1:15,000 and is common in Asia than in the west. Based on the level at which the lumen of the extrahepatic duct is obliterated, BA is classified into three types. Type III is the commonest (∼85%) type and has the most proximal level of obstruction in the porta hepatis, while type II in which the atresia is at the level of the common hepatic duct, is the least common (∼2.5%) and has been rarely reported. Here, we report the imaging features of an infant with type IIB biliary atresia.

Corrosive ingestion causes significant morbidity in children with no standard guidelines regarding management. This survey aimed to understand practices adopted by gastroenterologists, identify lacunae in evaluation and management and suggest a practical algorithm. Indian gastroenterologists participated in an online survey (65 questions) on managing corrosive ingestion. When ≥ 50% of respondents agreed on a management option, it was considered as ‘agreement’. Ninety-eight gastroenterologists (72 pediatric) who had managed a total of ~ 2600 corrosive ingestions in the last 5 years responded. The commonest age group affected was 2–5 years (61%). Majority of ingestion was accidental (89%) with 80% due to improper corrosive storage. Ingestion of alkali and acid was equally common (alkali 41%, acid 39%, unknown 20%). History of inducing-vomiting after ingestion by community physicians was present in 57%. There was an agreement on 77% of questions. The respondents agreed on endoscopy (70%) and chest X-ray (67%) in all, irrespective of symptoms. Endoscopy was considered safe on days 1–5 after ingestion (91%) and relatively contraindicated thereafter. The consensus was to use acid suppression, always (59%); steroids, never (68%) and antibiotics, if indicated (59%). Feeding was based on endoscopic findings: oral in mild injuries and nasogastric (NG) in others. Eighty percent placed a NG tube under endoscopic guidance. Stricture dilatation was considered safe after 4 weeks of ingestion. Agreement on duration of acid suppression and stricture management (dilatation protocol and refractory strictures) was lacking. Corrosive ingestion mostly affects 2–5-year olds and is accidental in majority. It can be potentially prevented by proper storage and labelling of corrosives. An algorithm for management is proposed.

Summary Background Vitamin B12 deficiency has been associated with a very wide spectrum of neurologic manifestations and the majority of cases occur in infants, pregnant women, and elderly people. In children, common neurological complications include neuropathy, neuropsychiatric features, infantile tremor syndrome, developmental delay, cognitive decline, spastic paraparesis due to subacute combined degeneration of cord, seizures, encephalopathy, extrapyramidal features, and neuropathy. Vitamin B12 is known to cause sensory ataxia, along with impaired position and vibration sense, as well as variable spasticity, as a part of subacute combined degeneration of the spinal cord. However, only a few cases of isolated cerebellar ataxia caused by vitamin B12 deficiency have been reported in published literature. Methods and results We are reporting a case of isolated cerebellar ataxia progressing over months in a 13-year-old boy. He also had associated knuckle hyperpigmentation, megaloblastic anemia and his magnetic resonance imaging of the brain was normal. Complete blood count showed hemoglobin of 8.6 gm/dl and peripheral smear showed macrovalocytes and few hypersegmented neutrophils. Serum vitamin B12 level was low (134 pg/mL). He was started on daily intramuscular vitamin B12 supplementation and he showed a favorable response after the first week. Conclusions Clinicians need to consider vitamin B12 deficiency as one of the rare etiological possibilities in children presenting with isolated subacute onset/chronic ataxia, as supplementation of this vitamin is likely to cause a complete reversal of ataxia in such children.

Background Pulmonary embolism (PE) is a life-threatening event with a mortality of ~10%. It is relatively uncommon in children and literature regarding the condition is sparse. In adults, the classical clinical presentation is with pleuritic chest pain, hemoptysis, and dyspnea, whereas in children, the presentation is often nonspecific. Materials and methods Clinical features, risk factors, and outcome of children with PE presenting to our unit between December, 19 and March, 2020 were recorded. Results Four children [mean age: 10 (6–16) years, 3 females], all presenting with tachycardia and dyspnea were diagnosed with PE. Different risk factors such as deep vein thrombosis, nephrotic syndrome, softtissue infection, and infective endocarditis (IE) were identified in all patients. One child died while others responded to anticoagulation. Conclusion We aim to highlight the importance of timely recognition of PE in children with known risk factors for the same. Early recognition and timely treatment of PE are critical to save lives. How to cite this article Agrawal S, Shrivastava Y, Bolia R, Panda PK, Sharawat IK, Bhat NK. Pulmonary Embolism in Children: A Case Series. Indian J Crit Care Med 2020;24(12):1272–1275.

BACKGROUND Paediatric inflammatory bowel disease (PIBD) is increasing across the world. However, information from India is sparse. This multicentre study evaluated the demographics, clinical phenotype and outcome of PIBD from India. METHODS Data of children (≤18 years) with PIBD were collected using a proforma containing details of demographics, clinical profile, extraintestinal manifestations (EIM), investigations, disease extent and treatment. RESULTS Three hundred twenty-five children [Crohn's disease: 65.2%, ulcerative colitis: 28.0%, IBD unclassified (IBDU): 6.7%, median age at diagnosis: 11 (interquartile range 6.3) years] were enrolled. 6.9% children had family history of IBD. Pancolitis (E4) was predominant in ulcerative colitis (57.8%) and ileocolonic (L3, 55.7%) in Crohn's disease. Perianal disease was present in 10.9% and growth failure in 20.9% of Crohn's disease cases. Steroids were the initial therapy in 84.2%, 5-amino salicylic acid in 67.3% and exclusive enteral nutrition (EEN) in 1.3% cases. Overall, immunomodulators and biologics were given to 84.3 and 17.9% cases, respectively, and 2.9% cases underwent surgery. Very early onset IBD (VEOIBD) was seen in 60 (19.2%) children. IBDU was commoner in the VEOIBD than the older-PIBD (18/60 vs 4/253; P < 0.001). VEOIBD-Crohn's disease patients more often had isolated colonic disease than the older Crohn's disease (45.4% vs 11.8%; P < 0.001). Prevalence of perianal disease, EIM, therapeutic requirements and outcome were not different between VEOIBD and older-PIBD. CONCLUSION Disease location and phenotype of PIBD in Indian children is similar to the children from the west. However, the therapeutic options of EEN, biologics and surgery are underutilized. VEOIBD accounted for 19.2% of PIBD.

Dengue fever continues to be an important cause of morbidity and mortality in tropical and subtropical countries. A wide range of neurological manifestations including dengue encephalopathy, Guillain-Barre syndrome, acute disseminated encephalomyelitis, transverse myelitis, cranial nerve palsies, and myositis have been reported following dengue infection. But parkinsonism secondary to dengue virus infection is uncommon, with only three published case reports in adults and one in children. We describe a 13-year-old pre-morbidly normal boy, who presented with bradykinesia, bradyphonia, mask-like facies, and cogwheel rigidity while recovering from uncomplicated DF. He responded favorably to levodopa/carbidopa supplementation and had resolution of symptoms over the next 2 weeks. We also did a comparative review of all published cases of dengue-induced parkinsonism. Post-dengue, parkinsonism is uncommon, and treating clinicians should be aware of this uncommon but treatable neurological complication of a common arboviral infection.

OBJECTIVES: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of "normal GGT" BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. METHODS: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (

Primary neuroleptospirosis although rare but has been reported in the literature in the form of case reports and case series. However, there are no reports of autoimmune encephalitis triggered by leptospirosis in the literature, although four cases of acute disseminated encephalomyelitis, which is also considered to have autoimmune etiology have been reported. We are reporting an adolescent girl, who developed anti-N-methyl-d-aspartate receptor encephalitis after the resolution of systemic symptoms of leptospirosis. Her symptoms including neuropsychiatric and extrapyramidal features and sleep disturbances resolved completely after immunotherapy. As recently autoimmune encephalitis triggered by various infections are getting reported more frequently around the world, the clinicians need to consider this clinical possibility, even in patients with leptospirosis, who develop neurological symptoms while systemic clinical features are subsiding. Early recognition and timely administration of immunotherapy have the potential to completely reverse the neurological symptoms.

Objectives and study: The utility of measuring thiopurine metabolites (TM) to individualize therapy in autoimmune hepatitis (AIH) has not been defined, and the evidence regarding its use in clinical practice is heterogeneous. This systematic review and meta-analysis aimed to compare the mean concentration of TM between patients in biochemical remission and those not in remission. Methods A systematic literature search was conducted using PubMed, Scopus, the Cochrane Library, and Google Scholar for keywords related to TM and AIH. Two reviewers independently searched and selected studies comparing the levels of 6-methyl mercaptopurine (6-MMP) and 6-thioguanine nucleotide (6-TGN) and their ratio in cases of AIH in remission and otherwise. Meta-analysis was performed by calculating the weighted mean difference using the inverse variance heterogeneity model. Results A total of 1066 records were identified through systematic search, of which 7 (n = 3 pediatric, n = 4 adults) were considered for inclusion and 442 TM measurements (n = 128 in children) were analyzed. Mean 6-TGN levels were significantly higher among patients in remission than in those who were not, with a pooled weighted mean difference (WMD) of 15.67 [95% confidence interval (CI), 6.68-24.66] pmol/8 × 108 red blood cells (RBC). The difference was higher in the pediatric age group [WMD 56.11, 95% CI: 13.60-98.62] than in adults [WMD 13.77, 95% CI: 4.58-22.97]. There was no significant difference in the 6-MMP levels (WMD -431.7, 95% CI:-1237.4-373.9 pmol/8 × 108 RBC, I2 = 82%, n = 3 studies) or 6-MMP/6-TGN ratio among the patients who were in biochemical remission and those who were not (WMD -0.97, 95% CI: -5.77-3.84, I2 = 82%, n = 3 studies]. Conclusions This meta-analysis suggests a link between 6-TGN levels and biochemical remission in AIH. Further high-quality studies are required to determine the therapeutic cut-off of 6-TGN.

Background In the last two decades, with the advent of whole-exome and whole-genome sequencing, supplemented with linkage analysis, more than 150 genes responsible for X-linked intellectual disability have been identified. Some genes like NEXMIF remain an enigmatic entity, as often the carrier females show wide phenotypic diversity ranging from completely asymptomatic to severe intellectual disability and drug-resistant epilepsy. Methods We report three patients with pathogenic NEXMIF variants from an Indian family. All of them had language predominant developmental delay and later progressed to moderate intellectual disability with autistic features. We also reviewed the previously published reports of patients with pathogenic NEXMIF variants. Results Together with the presented cases, 45 cases (24 symptomatic females) were identified from 15 relevant research items for analysis. Males have demonstrated a more severe intellectual disability and increasingly delayed walking age, autistic features, central hypotonia, and gastroesophageal reflux. In contrast, females have shown a predominant presentation with drug-resistant epilepsy and mild to moderate intellectual impairment. Notably, the affected females demonstrate a higher incidence of myoclonic, absence, and atonic seizures. The majority of the variants reported are nonsense or frameshift mutations, causing loss of function of the NEXMIF gene, while a considerable proportion possesses chromosomal translocations, microdeletions, and duplications. Conclusions NEXMIF gene mutations should be suspected in all cases of X-linked ID and autism cases in males or even in refractory epilepsy cases in females.

Introduction and aim. HAVCR1 protein is the cellular receptor for hepatitis A virus (HAV). Genetic polymorphism in this gene may alter the outcome of HAV infection. In a previous study, a 6-amino acid insertion (157insMTTTVP) in HAVCR1 gene was associated with more severe disease. We decided to investigate this association further. Material and methods. We sequenced exon 4 of the HAVCR1 gene in patients with clinical hepatitis A attending our institution, and a group of healthy controls in a disease-endemic setting in India. Frequencies of different haplotypes of a genomic region with two overlapping insertion-deletion polymorphisms (indels; rs141023871 and rs139041445) were compared between patients and controls, as well as between patients with and without a severe form of disease (liver failure). Results. The gene had three haplotypes in the region of interest - a short form, an intermediate-form with a 5-amino acid 157insMTTVP insertion and a long-form with a 6-amino acid 157insMTTTVP insertion. The allele frequency (29/150 [19%] vs. 43/146 [29%]; p = ns) and haplotype frequency (29/75 [39%] vs. 39/73 [53%]; p = ns) of the 157insMTTTVP variant were similar in hepatitis A patients and healthy controls (30%). Further, the allele frequency (12/58 [21%] vs. 17/92 [18%]; p = ns) and haplotype frequency (12/29 [41%] vs.17/46 [37%]; p = ns) of the longest variant were also similar in patients with severe and mild disease. Discussion. In the study population, the 157insMTTTVP variant of HAVCR1 gene was not associated with more severe outcome of HAV infection. Further studies in other populations around the world are needed to assess the relation of this genetic variation with disease outcome.

Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner. The aim of this review is to provide evidence-based information and clinical algorithms to guide the nutritional and gastrointestinal management of patients with CF.

Objectives A significant proportion of children with Crohn disease develop a secondary loss of response (LOR) to infliximab. Our aim was to study the impact of initial treatment strategies on secondary LOR. Methods We reviewed the medical records of children with Crohn disease who received scheduled maintenance infliximab therapy for at least 12 months. We compared children who developed LOR with those who did not; with regards to their clinical and laboratory parameters, disease phenotype, and treatment strategy before developing LOR. Results A total of 73 children (median age at diagnosis 11 (2-16) years, 41 boys) who had received a median duration of 33 (13-110) months of infliximab therapy were included in the final analysis. LOR was seen in 25(34.2%). Demographic variables, disease phenotype (age, disease location, and behavior), inflammatory parameters, and pediatric Crohn disease activity index at induction with infliximab were similar between both groups. Children with LOR had a significantly greater number of flares of the disease when compared to those who did not have LOR (4 [1-8] vs 2 [1-5] P = 0.03). The choice of the concomitant immunomodulator-methotrexate (11/29 [37.9%]) versus azathioprine (11/36 [30.5%]) (P = 0.6) did not affect LOR rates. The median time-lag between diagnosis and induction with infliximab was significantly longer in children with LOR as compared to those who did not have an LOR (28 [4-90] months vs 12.5 [1-121] months, P = 0.004). Conclusion Early use of infliximab in pediatric Crohn disease is associated with a decrease in secondary LOR. The type of concomitant immunomodulator used does not make a difference to LOR rates.

Early identification of children with decompensated chronic liver disease (DCLD) at risk of short-term mortality helps improve outcome. We aimed to evaluate the predictors of outcome and role of Child-Pugh, pediatric end-stage liver disease (PELD) and pediatric chronic liver failure sequential organ failure assessment (pCLIF-SOFA) score for prognosticating 28-day mortality in children with DCLD.DCLD children were prospectively evaluated with a clinico-laboratory proforma and followed for 28 days to determine outcome. Child-Pugh, PELD and pCLIF-SOFA were calculated at admission. Univariate and multivariate analysis was performed to identify the best predictors of outcome.A total of 110 children (74 boys, 96 [4-204] months) were enrolled and 37 (33.6%) died at 28 days. Significant risk factors for mortality were a higher international normalized ratio (hazard ratio [HR] 1.17; 95% CI 1.04-1.31; p 0.001) and bilirubin (HR 1.04; 95% CI 1.01-1.08; p 0.001), lower albumin (HR 0.46; 95% CI 0.27-0.77; p = 0.03) and sodium (HR 0.93; 95% CI 0.89-0.98; p = 0.01), absence of treatable etiology (HR 2.00; 95% CI 1.40-2.87; p = 0.001) and presence of organ failure (HR 3.22; 95% CI 1.98-10.58; p 0.001). Organ failure and serum sodium were independent predictors of poor outcome on multivariate analysis. pCLIF-SOFA (16 [9-22] vs. 9 [5-15]), Child-Pugh (11 [9-15] vs. 10 [8-14]) and PELD (22.2 [7.5-45.3] vs. 15.3 [4.5-23.9]) scores were significantly higher in non-survivors. The area under the curve was 0.977 for pCLIF-SOFA, 0.815 for Child-Pugh score, and 0.741 for PELD score. A pCLIF-SOFA score of ≥11 identified 28-day mortality with a sensitivity and specificity of 94.9% and 91.5%, respectively.Thirty-four percent of children with DCLD have a poor short-term outcome. Organ failure and low serum sodium are independent predictors of outcome. pCLIF-SOFA performs better than Child-Pugh and PELD in prognostication of 28-day mortality. Our study supports the use of scores based on organ failure in prognosticating children with DCLD.The ability to predict the course of a disease is an important part of the assessment, enabling timely interventions that improve outcomes. We evaluated the outcome (death vs. survival) and compared three different scoring systems for their ability to predict mortality within 28 days in children with decompensated chronic liver disease (DCLD). One-third of children with DCLD died within 28 days and the pediatric chronic liver failure sequential organ failure assessment score, which considers the main organ systems of the body (lungs, liver, brain, kidney, blood and cardiac) fared better for identification of children with a poor outcome than the Child-Pugh and pediatric end-stage liver disease score which comprise of only liver-related parameters. Our study supports the use of scores based on organ failure in prognosticating children with DCLD.

Background and aims Risk of infections is increased in patients with Acute Liver Failure (ALF) and Decompensated Chronic Liver Disease (DCLD). We evaluated the frequency, site, type and risk-factors for bacterial infections in children with ALF and DCLD and its effect on outcome. Methods ALF or DCLD children were enrolled prospectively. Clinical and laboratory details were recorded. Cultures (blood, urine and ascites) and chest X-ray were done at admission followed by weekly surveillance cultures. Results 173 patients, 68 ALF and 105 DCLD were enrolled. Infections were more common in DCLD than ALF (60/105 [57.1%] vs. 27/68 [39.7%]; P = 0.02). Ascitic fluid infection, pneumonia, urinary tract infection and bacteremia were seen in 19%, 17.9%, 13.2% and 12.1% patients respectively. Healthcare-Associated (HCA) infections were most frequent (39/87, 44.8%), followed by Nosocomial (NC, 32%) and Community-Acquired (CA, 23%). Nearly 3/4th of bacterial isolates were resistant to cephalosporins and quinolones, 23% being Multiresistant Bacteria (MRB). DCLD patients with infection had higher Child–Pugh Score (10 [6–14] vs. 7 [6–14]; OR 3.2 [1.77–5.10]: P = 0.007), need for ICU care (26/60 vs. 3/45; OR 10.70 [2.98–38.42]: P = 0.01), in-hospital mortality (24/60 vs. 8/45;OR 3.08 [1.22–7.75]: P = 0.04) and mortality at 3 month follow-up (32/60 vs. 9/45; OR 4.57 [1.87–11.12]: P = 0.00). Infection did not affect the outcome in ALF. Conclusion Infections develop in 40% ALF and 57% DCLD children. HCA and NC infections account for 77% of infections. Most culture isolates are resistant to cephalosporins and fluoroquinolones and 23% have MRB. Risk of infections is higher in DCLD patients with advanced liver disease.

An "Upset stomach" is a common problem encountered by physicians involved in the care of children. It includes, diarrhea, vomiting or abdominal pain. Though often mild and self-limiting, at times there are uncommon life-threatening conditions that require urgent care. It is important for primary physicians to recognize "alarm signs" and make a referral to the specialist at the right time. At the same time it is important to recognize what is normal and re-assure the caregivers. A detailed history and a proper systemic examination are important at presentation, looking for any "red flags". Investigations are seldom needed and the treatment is symptomatic. In acute diarrhea, adequate hydration is the cornerstone of management, which includes recognizing the degree of dehydration and treating with oral rehydration solution and Zinc. Dysentery i.e., bloody diarrhea needs treatment with antibiotics. In children with predominant vomiting and abdominal pain, it is vital to make sure one is not dealing with a "surgical "cause. If the child is not settling, a repeated examination is useful to look for the persistence or evolution of abdominal signs. Vomiting does not localize the problem to the gastro- intestinal (GI) tract and a complete systemic examination is needed to rule out an extra GI cause.

Constipation is a common problem in childhood. The most common type of constipation is functional, accounting for 90-95% of all cases. The aim of this review is to provide clinical scenarios with treatment using evidence-based information, and management strategies and a clinical algorithm to guide the management of constipation in children. Recent guidelines and online information sites are detailed. Clinical red flags and organic causes of constipation are included. Four clinical scenarios are presented: case (1) 4-month-old child with constipation since birth and likely Hirschsprung disease; case (2) 6-month-old infant with infant dyschezia; case (3) 4-year old with functional constipation; and; case (4) 9-year old with treatment resistant constipation. Children with functional constipation need a thorough history and physical exam to rule out the presence of any 'red flags' but do not require laboratory investigations. Management includes education and demystification, disimpaction followed by maintenance therapy with oral laxatives, dietary counselling and toilet training. Treatment options differ between infants and children. Disimpaction and maintenance regimens for common laxatives are presented. On treatment failure or on suspicion of organic disease the patient should be referred for further evaluation. The radionuclide intestinal transit study (scintigraphy) is a useful modality for evaluation and planning of management in treatment-resistant children. Treatment options for treatment-resistant patients are presented. High-level evidence (meta-analyses) for pharmalogical and non-pharmalogical treatment modalities are reviewed and an algorithm for assessment and treatment are presented.

South and East Asia is home to around 50% of the world’s population. The pattern of liver diseases in terms of epidemiology, clinical manifestations and outcomes in this region is different from the Western world. Increased infections, malnutrition, poor sanitation, difference in genetic make-up and inadequate access to healthcare facilities are the reasons for this difference.

BackgroundIn recent years, treatment strategies for ulcerative colitis have evolved with an early step-up approach, the availability of biologicals, and therapeutic drug monitoring.We carried out this study to evaluate the effect of these changes on disease outcomes.MethodsIn this retrospective review, 2 time periods were defined: Group 1 (2005–2010) and Group 2 (2011–2016). Baseline demographic, endoscopic parameters, and medication use were compared. Overall colectomy rate, number of disease flares per year, and number of hospital admissions per year were compared between the 2 groups.ResultsGroup 1 had 71 children, and in children in Group 2. The use of 5-ASA increased in Group 2 (Group 2, 99.2% vs. Group 1, 84.5%, P = 0.0007). In addition, infliximab and thiopurines were introduced earlier in the disease course.The 2-year cumulative probability of colectomy decreased from 14% to 3% (P = 0.02) between the 2 periods. No change in median number of flares per year [Group 1, 0.41 (IQR 0.6) vs. Group 2, 0.62 (IQR 0.91), P = 0.28] or median number of hospital admissions per year [Group 1, 0.30 (IQR 0.77) vs. Group 2, 0.21 (IQR 0.75), P = 0.52] was seen.Thereafter, we proceeded to identify the changes in treatment strategies that were responsible for the reduction in colectomy and we found that the use of infliximab OR 3.7 (95% CI 1.1–11.7), P = 0.02, was independently associated with it.ConclusionsA reduction in 2-year colectomy rates has been observed in patients with pediatric ulcerative colitis since biologics have become available for its treatment. The numbers of disease-flares rates and hospital admissions remain unchanged.

BACKGROUND Azathioprine (AZA) is the mainstay of maintenance therapy in pediatric autoimmune hepatitis (AIH). The use of thiopurines metabolites to individualize therapy and avoid toxicity has not, however, been clearly defined. METHODS Retrospective analysis of children ≤18 years diagnosed with AIH between January 2001 and 2016. Standard definitions were used for treatment response and disease flare. Thiopurine metabolite levels were correlated with the corresponding liver function test. RESULTS A total of 56 children (32 girls) were diagnosed with AIH at a median age of 11 years (interquartile range [IQR] 9). No difference in 6-thioguanine-nucleotide (6-TG) levels (271[IQR 251] pmol/8 × 10 red blood cell vs 224 [IQR 147] pmol/8 × 10 red blood cell, P = 0.06) was observed in children in remission when compared with those who were not in remission. No correlation was observed between the 6-TG and alanine aminotransferase levels (r = -0.179, P = 0.109) or between 6-methyl-mercaptopurine (6-MMP) and alanine aminotransferase levels (r = 0.139, P = 0.213). The 6-MMP/6-TG ratio was significantly lower in patients who were in remission (2[7] vs 5 (10), P = 0.04). Using a quartile analysis, we found that having a ratio of

We assessed cardiac function (CF) in celiac disease (CD) patients and the effect of gluten-free diet (GFD) on CF.Prospective evaluation of CF using conventional and tissue doppler echocardiography in 50 CD patients (age 4.2 ± 1.1 years) at diagnosis and after a year of GFD (group 1), 100 CD children (group 2; 47 compliant and 53 non-compliant) in follow-up and 25 healthy controls.Untreated CD (n = 50) children had larger left ventricle end diastolic dimension (35.33 ± 0.87 vs. 32.90 ± 0.91 mm; p = .04), reduced (55%) left ventricular ejection fraction (20% vs. 0%; p = .01) and a higher (0.6) myocardial performance index (MPI, 66% vs. 0%; p ≤ .01) as compared to controls. Re-evaluation after one year with good dietary compliance showed changes in isovolumic relaxation time (72.5 ± 4.2 vs. 50.62 ± 2.69; p = .0001) and deceleration time (121.05 ± 10.1 vs. 99.87 ± 8.5; p = .02), reflecting improved cardiac diastolic function. GFD compliant patients had lower MPI than non-compliant (0.60 ± .03 vs. 0.66 ± .08; p = .04), reflecting improvement in load-independent echocardiographic parameters.Subclinical cardiac dysfunction is common in CD children at diagnosis. Improvement in echocardiographic parameters occurs with GFD and non-compliant children continue to have persistent cardiac dysfunction.

Recent years have witnessed an increase in acute pancreatitis (AP) in children; however, the natural history of acute fluid collection (AFC) and pseudocyst is largely unknown. We evaluated the frequency, clinical characteristics, and natural history of pseudocysts in children with AP.Children with AP admitted at Sanjay Gandhi Postgraduate Institute of Medical Sciences from 2001 to 2011 were enrolled and studied until complete resolution. Subjects with inadequate follow-up, recurrent AP, and chronic pancreatitis were excluded.Of the 58 children (43 boys, median age 14 [1-18] years) with AP, 34 (58.6%) and 22 (38%) developed AFC and pseudocyst, respectively. No difference in age (12 [4-18] vs 13 [1-16] years), etiology (idiopathic 64% vs 47% and traumatic 27.2% vs 22.2%), and systemic complications (pulmonary [18% vs 11%], renal [22.7% vs 11%], and shock [13.6% vs 10%]) was observed between children with and without pseudocyst. A total of 11 of the 22 subjects with pseudocyst underwent drainage, the commonest symptom requiring drainage being gastric outlet obstruction [n = 5] and infection [n = 2]. The 11 of the 22 children with AP and pseudocyst (size 6.4 [3-14.4] cm) showed spontaneous resolution (disappearance [n = 9] and significant reduction in size [n = 2]) during 110 (25-425) days. Symptomatic pseudocysts requiring drainage were more often secondary to traumatic AP (6/6 vs 2/14 [idiopathic], P = 0.0007) than asymptomatic pseudocysts resolving spontaneously. Overall, only 26.4% (9/34) children with AFC required drainage because of symptomatic pseudocyst.Among children with AP, 58.6% developed AFC and 38% developed pseudocysts. Only patients with symptomatic pseudocyst need drainage, and asymptomatic pseudocyst can be safely observed irrespective of size and duration of collection.

Pediatric literature on spontaneous bacterial peritonitis (SBP) is limited. We evaluated the prevalence, subtypes, clinical profile, and effect on outcome of ascitic fluid infection (AFI) in children with liver disease. Children with liver disease-related ascites and subjected to paracentesis were classified as no-AFI and AFI (SBP, culture-negative neutrocytic ascites [CNNA], and monomicrobial non-neutrocytic bacterascites). Clinical and laboratory parameters, in-hospital mortality, and outcome in follow-up were noted. Two hundred sixty-two children (163 boys; age 84 [1–240] months, chronic liver disease [CLD, n = 173], non-CLD [n = 89]) were enrolled. A total of 28.6% (n = 75) had SBP/CNNA, more common in CLD than non-CLD (55/173 [31.7%] vs 20/89 [22.4%]; P = 0.1). A total of 50.6% SBP/CNNA cases were symptomatic for AFI. Gram-negative bacilli were isolated from 70% SBP cases. Twenty-five percent (18/72) CLD children with AFI had a poor hospital outcome, with INR, Child-Pugh score and gastrointestinal bleeding predicting outcome on multivariate analysis. Patients with CLD with SBP had higher in-hospital mortality (10/20 vs 5/35; P = 0.01) than those with CNNA, but similar Child-Pugh score (12[7–15] vs 11[7–14]; P = 0.1), recurrence of AFI (3/9 vs 6/24; P = 0.6) and mortality in follow-up (22.2% vs 25%; P = 0.1). Patients with CLD with SBP/CNNA had higher mortality over 1 year follow-up than no-AFI (24.2% [8/33] vs 12.2% [7/57]; P = 0.1) but the difference was not significant. A total of 28.6% children with liver disease-related ascites have SBP/CNNA; 50% are symptomatic. Patients with CLD with SBP/CNNA have a mortality of 24% over 1year follow-up. CLD with SBP is similar to CNNA except for higher in-hospital mortality.

Early and accurate identification of infection in patients with liver disease is challenging. The present study evaluated the role of procalcitonin (PCT) and C-reactive protein (CRP) as biomarkers of bacterial infection in children with liver disease.Demographic and clinical data of consecutive children admitted with acute liver failure (ALF) or decompensated chronic liver disease (DCLD) were collected. PCT and CRP were measured within 24 hours of admission. Blood and urine culture, chest x-ray, and ascitic fluid analysis were done.One hundred sixty-four children (113 boys, age 76 [0.5-204] months, ALF 69, DCLD 95) were enrolled. Seventy-seven (47%) had infection. Most common site was ascitic fluid (n = 35), followed by urinary tract (n = 26), pneumonia (n = 22), and blood stream infection (n = 16). Twenty-one children had multiple-site infections, 18 had severe sepsis, and 36 had systemic inflammatory response syndrome. PCT and CRP correlated with infection severity, higher in severe sepsis (8.3 [3.5-38] ng/mL and 4.1 [0.3-13.8] mg/dL) than only infection (0.89 [0.1-8] ng/mL and 1.7 [0.32-24] mg/dL) and no infection (0.3 [0.1-6.75] ng/mL and 0.3 [0.1-4.16 mg/dL]). Systemic inflammatory response syndrome was more common in patients with infection (31/77 vs 5/87, P = 0.00). PCT (0.5 ng/mL) and CRP (0.6 mg/dL) performed better in DCLD (AUC of 0.90 and 0.83) compared with patients with ALF (AUC of 0.73 and 0.69).PCT and CRP are reliable markers of infection and correlate with infection severity in children with liver disease. Their diagnostic accuracy is better in DCLD than ALF cases.

We report the first two pediatric patients with CF who underwent successful combined liver-pancreas transplantation in Australia and New Zealand for CF liver disease and CF-related diabetes mellitus.

Alagille syndrome (AGS) is an autosomal dominant disorder of chronic cholestasis characterized by paucity of interlobular bile ducts. The condition has been described only as isolated case reports in India. We describe clinical profile and outcome of nine subjects (six infants and three older children) with AGS. Cholestasis and characteristic facies were present in all, followed by congenital heart disease, vertebral anomalies, and posterior embryotoxon in seven, five, and four cases, respectively. Pruritus was the commonest symptom which was refractory to medical treatment in one third of cases. Two cases developed decompensated liver disease on follow up. High index of suspicion for this multisystemic condition is essential for correct diagnosis and management.

We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation.

Humoral immunodeficiency disorders present in children after 6 months of age with recurrent respiratory and gastrointestinal infections. These may be due to the absence of B cells causing panhypogammaglobulinaemia, or due to selective deficiencies in immunoglobulin subfractions. We present the case of a child with selective deficiency of IgM and IgG4 resulting in chronic diarrhoea and recurrent lower respiratory infections. With appropriate treatment of infections, the child had good symptom relief. Such an unusual combination of antibody deficiency has not been previously described in children. This case serves to illustrate the need for awareness to institute timely therapy for infection along with appropriate prophylactic measures like vaccination for these children.