Prevalence and Precipitants of Hepatic Encephalopathy in Hospitalized Children With Chronic Liver Disease.

Objective: Hepatic encephalopathy (HE) is a marker of poor prognosis in adults with chronic liver disease (CLD). We prospectively studied the prevalence and precipitants of HE in children with CLD as there is a paucity of literature on the same. Methods: Children (1-18 years) admitted with CLD were examined daily for the presence and grading of HE (West Haven/Whittington grading). Precipitants were classified as infection, dyselectrolytemia, gastrointestinal bleeding, constipation and dehydration. Changes in grades of HE and outcome were noted. Results: One hundred and sixty children (age 120 [84-168] months) were enrolled. HE was present in 50 (31.2%) patients with a total of 61 episodes. Maximum grade of HE was grade I (n = 16), II (n = 23), III (n = 11) and IV (n = 11). Forty-two cases had single and 8 had recurrent (2-5) episodes. Median duration of HE episodes was 96 (72-192) hours. Precipitants were identified in 55/61 (90.2%) episodes with infection (45/61, 73.7%) and dyselectrolytemia (33/61, 54%) being the most common. Lower albumin and sodium, higher INR and presence of infection were significantly associated with presence of HE. Overall, HE resolved in 33 (54%) episodes, while it progressed and persisted in 28 (45.9%) episodes. Patients with HE had a poorer outcome (25/50 vs 13/110; P < 0.01) with both higher in-hospital (11/50 vs 9/110; P = 0.02) and 1-month post discharge (14/39 vs 4/101; P < 0.01) mortality than those without HE. Conclusion: One-third of admitted CLD children have HE, with identifiable precipitants in 90% of cases. Children with HE have poorer liver functions, higher rate of infections and worse outcome than those without HE.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience.

Background: This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. Methods: EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. Results: One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n = 132, 95%); ii) growth retardation (GR, n = 95, 68%); iii) isolated gastric (IGV) and ectopic varices (n = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n = 104); side-to-side splenorenal shunt (n = 4); mesocaval shunt (n = 1); inferior mesenteric vein (IMV) to left renal vein shunt (n = 2); IMV to inferior vena cava shunt (n = 3); H-graft interposition splenorenal shunt (n = 1); spleno-adrenal shunt (n = 3); makeshift shunt (n = 1). Esophagogastric devascularization (n = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Conclusions: Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

An unusual cause of obstructive jaundice in childhood: Intra-choledochal malignant neuroendocrine tumour.

The extrahepatic biliary apparatus is a rare site for neuroendocrine tumours. A 13-year-old child presented with cholestatic symptoms of jaundice and pruritus with soft hepatomegaly and mild ascites. Magnetic resonance imaging and endoscopic ultrasound revealed a mid-common bile duct mass, and dilated intrahepatic biliary system. An en-bloc resection of the extrahepatic biliary apparatus, showed malignant cells disposed in lobules in a desmoplastic stroma with intramural invasion, staining positive for cytokeratin, chromogranin, synaptophysin and negative for CD56. At 3 months post-resection, whole body positron emission tomography scan was normal with no recurrence at 24 months.

Eosinophilic gastroenteritis: Clinical characteristics and management.

There is paucity of published data related to eosinophilic gastroenteritis (EGE). We aimed to study the clinical characteristics, management, and follow-up of EGE. From March 2014 to December 2018, patients with gastrointestinal (GI) symptoms suspected to have EGE were investigated. This is a retrospective study. Complete blood count, upper GI endoscopy (UGIE), and biopsy were done. Contrast-enhanced computed tomography (CECT) abdomen was done when intestinal obstruction was suspected. Laparoscopic small bowel resection or stricturoplasty and full-thickness biopsy were obtained. EGE was diagnosed if the biopsies showed eosinophilic infiltration of one or more regions of the GI tract (> 30 eosinophils per high power field [HPF]). Patients were treated with immunosuppressive therapy and if indicated surgery. Clinical response to therapy was assessed and patients were followed up for 1 year. Forty-one patients (mean age 34.8 years, median age 32, range 25-70 years, 29 males) had EGE. Upper abdominal pain was the most common symptom. Peripheral eosinophilia was present in 82.9% patients. On UGIE, duodenal lesions were observed in 75% patients. EGE was confirmed in 37 patients by endoscopic duodenal biopsies, in 2 patients by jejunal mucosal biopsies using enteroscopy, and in 2 patients by full thickness surgical biopsies. Forty-one patients were treated with oral corticosteroids inclusive of 4 patients who underwent surgery. Of the 37 patients, 6 were lost to follow-up; 31 patients were followed up for a period of 1 year. All the patients who were treated with corticosteroids responded to initial therapy. Over a follow-up period one case had frequent relapses. EGE should be suspected in patients with upper abdominal pain. Peripheral eosinophilia occurs in the majority. Tissue diagnosis showing eosinophilic infiltration is diagnostic. Oral corticosteroid therapy is highly effective and relapse is rare.

Chronic vomiting in children: A prospective study reveals rumination syndrome is an important etiology that is underdiagnosed and untreated.

BACKGROUND: The diagnosis of rumination syndrome is frequently overlooked, and under-recognized; children are subjected to unnecessary testing and inappropriate treatment for a condition which can be diagnosed clinically and managed easily. In the first ever systematic exploration of this condition from India, we present a prospective study on children with chronic vomiting in which rumination emerged as the predominant cause. METHODS: This was a prospective study in which all consecutive children (5-18 years) presenting with chronic or recurrent vomiting of at least 2-month duration were enrolled. Clinical history was assessed by a physician-administered questionnaire. All subjects underwent standard testing followed by additional investigations as required. The ROME III criteria were used. RESULTS: Fifty children (28 boys, age 12.2 + 3 years) were enrolled. Diagnosis was rumination syndrome 30, cyclical vomiting 8, functional vomiting 6, intestinal tuberculosis 4, intestinal malrotation 1, and superior mesenteric artery syndrome 1. Children with rumination syndrome had a relapsing and remitting (12, 40%) or a chronically symptomatic course (18, 60%). These children received incorrect diagnoses (26, 87%) or no diagnosis (3, 10%) and extensive investigation before referral. Before referral, children with rumination syndrome were treated with a median of four drugs (range 1 to 9); two underwent surgery (appendectomy) for their symptoms while one child was subjected to electroconvulsive therapy. Overall, resolution after treatment was seen in 26 (87%) with a relapse in 8 (27%) children. CONCLUSION: The diagnosis of rumination syndrome is delayed and these children are often inappropriately treated. Therapy in the form of diaphragmatic breathing has a good success rate.

Solitary Rectal Ulcer Syndrome in Children: A Report of 140 Cases.

OBJECTIVES: Solitary rectal ulcer syndrome (SRUS) is said to be rare in children (largest series so far; 55 in children, 116 in adults). We analyzed our experience to look at its clinical presentations, endoscopic appearance, and treatment outcome in a large cohort of children. METHODS: Clinical and endoscopic data were collected between 2000 and 2018. Children (18 years or younger) diagnosed to have SRUS on colonoscopy and confirmed by histopathology were included. All children with SRUS were treated with behavioral modification, bulk laxative. Most with ulcer received steroid enema and some sulfasalazine or sucralfate enema. RESULTS: The median age of 140 children was 12 (interquartile range [IQR]: 10-14) years, 79% were boys. The median symptom duration was 21 (IQR: 9-36) months. Rectal bleeding was the presenting feature in 131 (93.6%); constipation in 38 (27%); and small, frequent stools in 79 (56%). Most children had features of dyssynergic defecation such as prolonged sitting in the toilet (131, 93.6%), excessive straining (138, 98.6%), a feeling of incomplete evacuation (130, 92.8%), and rectal digitation (71, 50.7%). Rectal prolapse was noted in 24 (17%) cases. Colonoscopy documented rectal ulcer in 101 (72%) [Single: 84]. Over a median follow-up of 6 (IQR: 4-18) months, 27 patients were lost to follow-up and of the remaining 113 cases, 71 (62.8%) showed clinical improvement (healing of ulcer documented in 36/82, 44%). CONCLUSIONS: The majority of cases of SRUS presented in second decade with rectal bleeding and features of dyssynergic defecation. Ulcer was noted in three fourths of cases. The outcome of medical treatment with behavioral modification and local therapy was modest.

Optic nerve sheath diameter in children with acute liver failure: A prospective observational pilot study.

BACKGROUND & AIMS: Early detection of raised intracranial pressure (ICP) improves outcome in acute liver failure (ALF). We evaluated the feasibility of bedside, ultrasound-guided measurement of optic nerve sheath diameter (ONSD) in normal and ALF children and correlation of ONSD with grade of hepatic encephalopathy (HE), international normalized ratio (INR) and blood ammonia (BA). METHODS: Forty-one ALF and 47 healthy children (5-18 years) were prospectively enrolled and 12 hourly clinical evaluation was done. Laboratory parameters including INR and BA were recorded. ONSD was measured at admission, change in HE grade and at recovery in ALF, and once in controls. RESULTS: One hundred and twenty-one ONSD measurements (ALF-74, controls-47) were taken without complications. ONSD was 4.2 (3.9-4.3), 4.4 (4.0-4.6), 5.2 (4.8-5.8) and 3.9 (3.3-4.1) mm in controls, ALF without HE, with HE and at recovery respectively. ONSD was significantly higher in ALF with HE than those without HE. ALF without HE and at recovery had ONSD similar to controls. ONSD was higher in ALF with the clinical signs of raised ICP than those without (5.4 [4.9-5.7] vs 4.6 [4.1-5.3] mm; P = .01). ONSD of 4.6 mm differentiated ALF with HE vs without HE and 5.1 mm between poor vs good non-transplant outcome with ≥80% sensitivity and specificity. ONSD positively correlated with INR (r = .53, P < .001) and BA (r = .42, P = .002). CONCLUSIONS: ONSD can be safely and easily measured in ALF children and correlates with HE grade, INR and BA. Normal ONSD in children (>4 years) is <4.5 mm and value of >5.1 mm in ALF requires urgent attention.

Prevalence, nature, and predictors of colonic changes in children with extrahepatic portal vein obstruction.

BACKGROUND AND AIMS: The medical literature on colonic changes in children with extrahepatic portal venous obstruction (EHPVO) is limited. We evaluated EHPVO children for prevalence, nature, and relation of colonic changes with disease duration, extent of splenoportal axis (SPA) thrombosis, portal hypertensive gastropathy (PHG), and esophageal varices (EVs). The correlation between histologic and endoscopic changes was studied. METHODS: Subjects were evaluated by colonoscopy with ileoscopy and biopsy sampling, clinico-laboratory profiles, and SPA imaging. Colonic changes were classified as varices (rectal/colonic) and portal hypertensive colopathy (PHC; colitis-like or vascular lesions). Morphometric analysis of colonic biopsy specimens was performed. RESULTS: Fifty-four children (median age, 12 years [range, 8-15]; hematochezia in 9 [16.6%]) were evaluated. Rectal and colonic varices were seen in 51 (94%) and 2 (3.7%) cases, respectively. Seventy-five percent of patients had PHC, and colitis-like lesions were more common than vascular lesions (36/40 vs 23/40; P = .001). Colopathy changes were pancolonic in 52.5%, left-sided in 42.5%, and right-sided in 5% of cases. Sixteen percent of patients (8/49) had ileal changes. Children with PHC had PHG more often (90% vs 57%; P = .01), more endotherapy sessions (mean 6 [range, 4-8] vs 2 [range, 1-4]; P = .03), and large EVs less often (12.5% vs 43%; P = .02) than those without colopathy. The extent of SPA thrombosis was similar in patients with and without PHC. The number of capillaries per crypt was higher in EHPVO than in control subjects. Morphometric changes had no correlation with endoscopy. CONCLUSIONS: Most EHPVO children had colonic changes, and 16% had ileopathy. "Colitis-like" changes and left-side involvement were more common. Patients with PHG and eradicated EVs had a higher risk of PHC.

Effect of Long-term Oral Steroids on Intraocular Pressure in Children With Autoimmune Hepatitis: a Prospective Cohort Study.

OBJECTIVE: Topical corticosteroids are known to cause raised intraocular pressure (IOP). However, there is a scarcity of literature regarding systemic steroids-induced raised IOP in children. The authors aimed to evaluate the IOP in children with autoimmune hepatitis (AIH) receiving oral prednisone. METHODS: In this prospective study, children (1 to 18 y) with newly diagnosed AIH who received oral prednisone (July 2016 to December 2017) were included. Comprehensive ophthalmic check-up, including visual acuity, IOP, slit-lamp, and fundus examinations were done before and at 1, 3, and 6 months of follow-up. IOP ≥20 mm Hg was considered raised IOP. Symptomatic raised IOP patients were managed with antiglaucoma medication with a tapering dose of prednisone. RESULTS: A total of 33 children (19 boys) with a median age of 11 (3 to 18) years were analyzed. Raised IOP was observed in 20 (61%) children (19 were moderate and 1 was high responder) at 1 month, 8 (24%) at 3 months, and 1 (3%) at 6 months of treatment. Patients who developed raised IOP had a more severe liver disease in terms of decompensation and low albumin and high pediatric end-stage liver disease scores at presentation. There was no correlation between IOP and starting and cumulative dose of steroids. On multivariate analysis, decompensation at presentation was significantly associated with raised IOP (P<0.001; odds ratio, 30; 95% confidence interval, 4.2-210.6). Raised IOP returned to normal with antiglaucoma medication along with prednisone tapering in all except one. CONCLUSIONS: Systemic corticosteroids in children with AIH carry a risk for IOP elevation, especially in decompensated liver disease. A periodic ophthalmic check-up is necessary for early recognition and intervention before irreversible vision loss.

Non-variceal gastrointestinal bleed in children: surgical experience with emphasis on management challenges.

PURPOSE: This exclusively surgical series on pediatric non-variceal gastrointestinal bleed (NVGIB) defines three levels of bleed site and describes etiology, bleed severity, diagnostic algorithm, and surgical management for each bleed site. Management challenges are detailed. METHODS: Patients aged ≤ 18 years treated surgically for NVGIB were analysed. RESULTS: Bleed site (n = 87) was classified as: upper gastrointestinal bleed (UGIB; n = 11); small bowel bleed (SBB: n = 52); and lower GIB (n = 24). Four etiology-based groups were identified: lesions with ectopic gastric mucosa (EGM; n = 33), tumours (n = 23), ulcers (n = 21), and vascular pathology (n = 8). Bleed severity spectrum was: acute severe bleed (n = 12); subacute overt bleed (n = 59); and occult GIB (n = 16). Preoperative diagnosis was obtained in all UGIB and LGIB lesions. Eighty-two percent of surgical SB lesions were diagnosed preoperatively on Tc99m pertechnetate scan, computed tomography enterography-angiography, and capsule endoscopy; remaining 18% were diagnosed at laparotomy with intra-operative enteroscopy (IOE). Surgical management was tailored to bleed site, severity, and etiology. Indications of IOE and approach to management challenges are detailed. CONCLUSIONS: The commonest site-specific bleed etiologies were duodenal ulcers for UGIB, EGM lesions for SBB, and tumours for LGIB. SBB presented diagnostic challenge. Diagnostic algorithm was tailored to bleed site, age-specific etiology, bleed severity, and associated abdominal/systemic symptoms. Management challenges were acute severe bleed, occult GIB, SBB, obscure GIB, and rare etiologies. IOE has a useful role in SBB management.

Aetiological spectrum, clinical differentiation and efficacy of polyethylene glycol over lactulose in children with constipation: Experience of 316 cases.

AIM: To find the aetiological spectrum, clinical features to differentiate organic from functional constipation (FC) and the efficacy of polyethylene glycol (PEG) over lactulose in the treatment of childhood constipation. METHODS: From January 2007 to December 2014, 316 consecutive children (up to 18 years) with constipation were included in this study. FC was defined as per Rome III criteria. Standard treatment protocol (disimpaction with PEG followed by laxatives) was followed in FC. Lactulose was used in the first 4 years of study and PEG in the last 4 years. Success of therapy was assessed at 3 months. RESULTS: The median age was 44 (25.00-78.00) months, and 227 (72%) were boys. The majority, 245 (77.5%), had FC, while Hirschsprung disease was the most common organic cause (39.4%). On multivariate analysis, delayed passage of meconium, growth failure, absence of retentive posturing and absent faecal impaction significantly (P < 0.05) determined an organic aetiology. In FC, successful outcome at 3 months was achieved equally with PEG and lactulose (81 vs. 76%, respectively). However, more children in lactulose group had to be switched over to PEG due to lack of efficacy (P = 0.0002). CONCLUSIONS: FC is the most common cause of constipation in children. Presence of delayed passage of meconium, growth failure and absence of retentive posturing and absent faecal impaction raise the suspicion of an organic cause. Both lactulose and PEG are equally effective. PEG has an edge over lactulose as the need for switch over was uncommon.

Spectrum of genomic variations in Indian patients with progressive familial intrahepatic cholestasis.

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is caused by variations in ATP8B1, ABCB11 or ABCB4 genes. Data on genetic variations in Indian patients with PFIC are lacking. METHODS: Coding and splice regions of the three genes were sequenced in unrelated Indian children with PFIC phenotype. The variations identified were looked for in parents, 30 healthy persons and several variation databases, and their effect was assessed in-silico. RESULTS: Among 25 children (aged 1-144 months), nine (36%) had unique major genomic variations (ATP8B1: 4, ABCB11: 3 and ABCB4: 2). Seven had homozygous variations, which were assessed as 'pathogenic' or 'likely pathogenic'. These included: (i) four amino acid substitutions (ATP8B1: c.1660G > A/p.Asp554Asn and c.2941G > A/p.Glu981Lys; ABCB11: c.548 T > C/p.Met183Thr; ABCB4: c.431G > A/p.Arg144Gln); (ii) one 3-nucleotide deletion causing an amino acid deletion (ATP8B1: c.1587_1589delCTT/p.Phe529del); (iii) one single-nucleotide deletion leading to frame-shift and premature termination (ABCB11: c.1360delG/p.Val454Ter); and (iv) a complex inversion of 4 nucleotides with a single-nucleotide insertion leading to frame-shift and premature termination (ATP8B1: c.[589_592inv;592_593insA]/p.Gly197LeufsTer10). Two variations were found in heterozygous form: (i) a splice-site variation likely to cause abnormal splicing (ABCB11: c.784 + 1G > C), and (ii) a nucleotide substitution that created a premature stop codon (ABCB4: c.475C > T/p.Arg159Ter); these were considered as variations of uncertain significance. Three of the nine variations were novel. CONCLUSIONS: Nine major genomic variations, including three novel ones, were identified in nearly one-third  of Indian children with PFIC. No variation was identified in nearly two-thirds of patients, who may have been related to variations in promoter or intronic regions of the three PFIC genes, or in other bile-salt transport genes.

Prevalence and Impact of Bacterial Infections in Children With Liver Disease-A Prospective Study.

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.

Concurrent extrahepatic autoimmune disorders: unexplored dimension of autoimmune liver disease in children.

BACKGROUND AND AIM: No comprehensive and prospective data are available for concurrent extrahepatic autoimmune disorders (CEAIDs) in children with autoimmune liver disease (AILD). The aim of this study was to evaluate CEAIDs in AILD and their effect on AILD outcome. PATIENTS AND METHODS: Enrolled AILD and CEAIDs children were diagnosed on the basis of simplified and standard diagnostic criteria, respectively. The clinicopathological profile, treatment response, and outcome were compared between AILD with CEAIDs (group A) and AILD without CEAIDs (group B). RESULTS: In 62 AILD children, CEAIDs were found in 42% (n=26) [vitiligo (42%), celiac disease (CD) (15%), potential CD (15%), autoimmune hemolytic anemia (AIHA) (15%)]. CEAIDs were asymptomatic in 75%. Single CEAID was found in 81% (21/26) and multiple CEAID was found in 19% (5/26). Significantly less biochemical remission (46.1 vs. 74.2%, P=0.03), more treatment failure (23 vs. 3.2%, P=0.04), and higher mortality (15.3 vs. 3.2%, P=0.04) were encountered in group A compared with group B. On multivariate analysis (n=57), less biochemical remission in vitiligo (P=0.04); more treatment failure in AIHA (P=0.004) and vitiligo (P=0.04); and high mortality in AIHA (P=0.02) subgroups were reported. CD treatment has good impact on AILD outcome. All cases of diabetes mellitus in AILD were steroid-induced rather than because of autoimmunity (absence of antibody against tyrosine phosphatase and glutamic acid decarboxylase and elevated C-peptide). CONCLUSION: All AILD children should be screened for CEAIDs as the majority are asymptomatic. The AILD outcome was favorable in CD, but poor in vitiligo and AIHA. We suggest the incorporation of CEAIDs in a pediatric AILD scoring system.

Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children.

BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention. PSS analysed included Paediatric end-stage liver disease (PELD), Rotterdam, BCS-Transjuglar intrahepatic Portosystemic shunt (BCS-TIPS) index, Zeitoun, Child-Pugh and Model for end-stage liver disease. RESULTS: Of 113 BCS children, 48 children underwent 53 successful primary RI. Actuarial probability of vascular patency was 87% at 1 year and 82% at 5 years follow-up. Four groups (SI: n = 40, PO: n = 7, NU: n = 13, DBI: n = 6) were analysed. Univariate analysis showed pre-intervention PELD score [PO: 11 (-1-23) vs SI: 2 (-8-25), P = .009] with a cut-off of 4 (AUC: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (NU vs DBI), multivariate analysis demonstrated that Zeitoun score predicted death independently (OR 15.4, 95% CI: 1.17-203.56, P = .04) with a cut-off of 4.3 (AUC: 0.923, 83% sensitivity and 77% specificity). CONCLUSIONS: Children with BCS have a favourable long-term outcome. Among those undergoing RI, pre-intervention PELD score determines the outcome. Survival is determined by Zeitoun score in those unintervened.

Differentiation Between Crohn Disease and Intestinal Tuberculosis in Children.

OBJECTIVES: The aim of the study was to evaluate clinical, endoscopic, radiologic, and histopathological features helpful in differentiating Crohn disease (CD) from intestinal tuberculosis (ITB) in children. METHODS: Patients diagnosed to have CD or ITB based on standard recommended criteria were enrolled. Children with inflammatory bowel disease unclassified or suspected ITB or CD with incomplete work-up or lost to follow-up were excluded. The clinical and laboratory (radiology, endoscopy, and histology) details of children were analyzed. RESULTS: Twenty cases of ITB (14 [3-17] years) and 23 of CD (11 [1-17] years) were enrolled. Presentation with chronic diarrhea (82% vs 40%; P = 0.006) and blood in stool (74% vs 10%; P = 0.001) favored CD, whereas subacute intestinal obstruction (20% vs 0%; P = 0.04) and ascites (30% vs 0%; P = 0.005) favored ITB. Presence of deep ulcers (61% vs 30%; P = 0.04), longitudinal ulcers (48% vs 15%; P = 0.02), involvement of multiple colonic segments (70% vs 35%; P = 0.02), left-sided colon (87% vs 40%; P = 0.003), extraintestinal manifestations (21.7% vs 0%; P = 0.02), and higher platelet count (3.9 vs 2.6 × 10/mm; P = 0.02) favored CD. Isolated ileocecal involvement (40% vs 8.7%; P = 0.03) was a feature of ITB. TB bacilli were demonstrated in 40% ITB cases (colon-6, ascites-1, abdominal lymph node-1). On multivariate analysis, presence of blood in stool (odds ratio: 37.5 [confidence interval: 3.85-365.72], P = 0.002) and left-sided colonic involvement (odds ratio: 16.2 [confidence interval: 1.63-161.98], P = 0.02) were independent predictors of CD. CONCLUSIONS: Microbiologic confirmation of tuberculosis is possible in 40% ITB cases. Presence of blood in stool and left-sided colonic involvement are the most important features favoring CD.

Yield of Endoscopic Ultrasound in Children and Adolescent With Acute Recurrent Pancreatitis.

OBJECTIVES: Endoscopic ultrasound (EUS) is an established tool for evaluation of adults with acute recurrent pancreatitis (ARP) whereas data in pediatrics is limited. Our study assessed the role of EUS in identifying etiology including changes of chronic pancreatitis (CP) in children and adolescents with ARP. METHODS: Children with ARP (≥2 episodes of acute pancreatitis [AP]) were prospectively evaluated with a detailed clinical proforma and EUS. Subjects with known etiology of ARP or CP on ultrasonography/computed tomography and magnetic resonance cholangiopancreatography (MRCP, Cambridge grade ≥3) were excluded. Parenchymal and ductal changes on EUS as per minimal standards terminology (MST) features were noted. RESULTS: Thirty-two children (22 boys, age 14 [8-18] years) with ARP (median of 3 [2-5] episodes of AP) were enrolled. EUS was safe and technically successful in all. Gall bladder sludge was found in 1 (3%) case and none had other pancreatobiliary structural abnormalities. EUS diagnosis of CP (≥4 features) was made in 10/32 (31%) cases. Subjects with CP on EUS had a longer disease duration than those without CP (45 [10-97] vs 22 [8-78] months; P = ns). MRCP was normal in 28 and showed pancreas divisum in 1 case. Three cases had equivocal (Cambridge II) changes at initial MRCP and 2 of them had repeat MRCP, which showed definite (Cambridge IV) CP. All these 3 cases had CP on EUS. CONCLUSIONS: EUS diagnosed CP (≥4 features) in 31% and biliary abnormality in 3% children with ARP. EUS is safe, sensitive, and useful for early diagnosis of CP in children with ARP.

Autoimmune acute liver failure and seronegative autoimmune liver disease in children: Are they different from classical disease?

OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available. RESULTS: Seventy children [AI-ALF=15 and AI-non-ALF=55 (SN-AILD=11, SP-AILD=44)] were evaluated. Age at presentation [7 (1.2-16) vs. 9 (2-17) years] percentage of female patients (67 vs. 62%), and AILD type (type II, 53 vs. 31%) were similar in AI-ALF and AI-non-ALF patients], respectively. 8/15 AI-ALF cases were treated with steroids (improved-4, liver transplant-1, and death-3) and 7/15 died before initiation of therapy. Hepatic encephalopathy (100 vs. 16.3%; P<0.001), massive hepatic necrosis (60 vs. 0%; P<0.001), and higher pediatric end-stage liver disease [n=53, 29.9 (13.1-56.9) vs. 9.8 (-10-28.7) P<0.001], model for end-stage liver disease [n=17, 38.5 (24-46) vs. 18 (6-24); P=0.005], and Child-Turcotte-Pugh [n=70, 13 (8-13) vs. 9 (5-13); P<0.001] scores were features of AI-ALF. Poorer response to immunosuppression (4/8 vs. 48/55; P=0.02) and higher mortality (11/15 vs. 4/55; P=0.0001) were seen in AI-ALF than in AI-non-ALF patients. Clinicolaboratory profile, therapeutic response, and outcome were similar in SN-AILD and SP-AILD. CONCLUSION: AI-ALF is characterized by poorer liver function, lower response to immunosuppression, and higher mortality compared with SP or SN AI-non-ALF, which are similar.