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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Prevalence, Clinical Profile, and Outcome of Ascitic Fluid Infection in Children With Liver Disease.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Role of Procalcitonin and C-Reactive Protein As Biomarkers of Infection in Children With Liver Disease.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Assessment of gastric emptying in children: Establishment of control values utilizing a standardized vegetarian meal.

BACKGROUND AND AIM: Disorders of gastric emptying constitute an important group of conditions in children. The diagnostic gold standard is scintigraphy, and recommendations for standardization have been published with adult normative data. Pediatric literature lacks standardized age specific normative values. Our aim was to establish normal values of solid phase gastric emptying utilizing scintigraphy in children (5-18 years) using the recommended imaging protocol and standardized meal. METHODS: Gastric emptying was assessed by scintigraphy of a (99m) Tc-labeled vegetarian meal. Image acquisition was dynamic for first hour and static at 2, 3, and 4 h. Results were reported as percent intragastric retention; lag phase defined as time to 5% emptying. RESULTS: Thirty (17 boys) healthy children, mean ages of 9.5 ± 3.1 (range 5 to 16) years, were enrolled. Median values (5th and 95th percentiles) for percent gastric retention at 30 min, 1, 2, 3 and 4 h were 90% (76 and 100%), 77% (56 and 96%), 33% (10 and 71%), 16% (3 and 44%), and 7% (0.5 and 22%). The median (5th and 95th percentiles) lag phase was 18 min (6 and 60 min). Lag phase had no correlation with gastric retention at 4 h. CONCLUSIONS: This study provides valuable normative ranges for solid phase gastric emptying in children. Using adult normative data in children may be inappropriate.

Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension.

BACKGROUND & AIMS: Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome. METHODS: Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia/orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra- and intergroups with and without hepatopulmonary syndrome. RESULT: Of 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) (P = 0.01). Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, P < 0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, P = 0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both paediatric end-stage liver disease score and Child-Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%). CONCLUSIONS: Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome.

Duodenal morphometry and small bowel permeability in children with portal hypertension.

OBJECTIVE: We prospectively studied children with portal hypertension (PHT) for portal hypertensive duodenopathy (PHTD) and small bowel intestinal permeability (SIP) with the objectives of defining histopathological parameters for PHTD and to find out whether any association existed among structural changes, SIP, and nutritional status. METHOD: SIP was assessed by using lactulose and mannitol sugar probes in 31 children with PHT (cirrhosis n = 15 and extrahepatic portal venous obstruction n = 16) and 15 healthy children as controls. Morphometric assessment from duodenal biopsies was done in children with PHT. SIP and morphometric parameters were correlated with nutritional status and dietary intake. RESULTS: Among children with PHT, 48% had PHTD defined as presence of villous atrophy (villous to crypt ratio < 2.5:1), dilated capillaries (capillary diameter > 16.8 µm, capillary area > 151 µm, capillary perimeter > 56 µm), and thickened muscularis mucosae (>22.2 µm). Lactulose excretion alone was increased in children with PHT as compared with healthy children (median %: 0.03, 0.02, and 0.01 for cirrhosis, extrahepatic portal venous obstruction, and controls, respectively [P < 0.01]) signifying increased paracellular permeability in PHT. Children with PHT had significantly lower z scores for height, weight, and triceps skin-fold thickness (<-2SD), whereas no differences were found in dietary intake between patients and controls. Increased SIP, nutritional compromise, and PHTD in our patients had no correlation. CONCLUSIONS: PHT is often associated with duodenopathy. SIP does occur as a result of increased paracellular permeability. Factors of increased SIP, undernutrition, and PHTD do not have correlation in childhood PHT.

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.

Refeeding syndrome in children in developing countries who have celiac disease.

OBJECTIVES: The clinical presentations of celiac crisis and refeeding syndrome in celiac disease are almost similar, but information about refeeding syndrome is scarce. We are reporting for the first time 5 cases of refeeding syndrome in children with celiac disease that could have otherwise been labeled as celiac crisis. METHODS: From January to December 2010, a chart review of hospital records of all celiac disease cases was performed, and refeeding syndrome was ascribed in those celiac patients who deteriorated clinically after initiation of a gluten-free diet and had biochemical parameters suggestive of refeeding syndrome such as hypophosphatemia, hypokalemia, hypocalcemia, and hypoalbuminemia. RESULTS: Of the total 35 celiac disease patients, 5 (median age 6.5 [range 2.2-10] years, 3 boys) were identified as having refeeding syndrome. All 5 children were severely malnourished (body mass index <14 kg/m) and all of them had anemia, hypophosphatemia, hypokalemia, hypoalbuminemia, and hypocalcemia, meaning that they had the perfect setting for developing refeeding syndrome. At the same time, their clinical features fulfilled the criteria for celiac crisis except that their symptoms have worsened after the introduction of a gluten-free diet. Nevertheless, instead of using steroids, they were managed as refeeding syndrome in terms of correction of electrolytes and gradual feeding, and that led to a successful outcome in all of them. CONCLUSIONS: Severely malnourished patients with celiac disease are at risk of developing potentially life-threatening refeeding syndrome, which may mimic celiac crisis, especially in developing countries. Early recognition and appropriate treatment are the keys to a successful outcome.